492 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 200. 201. 202. Dutta Gynae 4/331; Confusion ‘A case of postmenopausal bleeding is considered to be endometrial cancer unless proved otherwise’. Note here that Dutta does not mention specific Indian context and hence the above statement should indicate a worldwide context. Answer is A (HPV 16): COGDT 10°/843 Increased risk of carcinoma cervix is seen with several Human Palilloma Virus (HP) infections including HPV 16, 18, 31 and 33. Over all the most common agent associated with ca cervix is HPV 16. HPV and Cervieal Cancer (COGDT) ‘+ Human Papilloma Virus (HPV) is central to the development of cervical cancer + HPV DNA is found in 99.7% of all cervical cancers ‘+ HPV I6is the most prevalent HPV type in squamous cell cancer cervix. + _HPY 181s the most prevalent HPV type in adenocarcinoma cervix. The most common histological type of cancer is squamous cell (70-75%) Thus, overall [HPV is the most prevalent HPY type in cancer cervix EEG oa Review + Early intercourse® (coitus before the age of 18 years) + Multiple sexual parmers® +« Delivery of frst baby before the age of 20 years * Maltiparin® ‘* Sexually transmitted infections? HIV, HSV-2, HPY (16, 18, 31, 33), Condylomata + History of preinvasive lesions + Combined oral contraceptives and progesterone (use over long period) + Others = Smoking, Alcohol, Drug abuse = Low socio economic status = Poor personal Hygiene = Immunocompromised individuals Answer is A (Benign Cystic Teratoma): Novaks 13/426; Repeat Al 2006 “A benign cystic teratoma is the most common neoplasm to undergo torsion’- Novak's Gynaecology 13"/426 Different texts (Shaw's, Dutta) give confusing and contradictory statements, making the correct answer debatable. However in view of the clearly outlined text from Novak's as stated above, the single best answer is dermoid or Benign cystic teratoma. (Benign cystic teratoma is synonymous with dermoid) Answer is B (Ca Breast): Shaw 13/227; Repeat AI 1999; KDT 5/289; Dutta 6/545 Oral contraceptives are beneficial in benign breast diseases but not in carcinoma of the breast. Oral contraceptive pills and breast cancer + The role of OCP in causing breast cancer is controversial. Breast cancer has been reported in nulliparous women before the age of 24 years who have taken OCP's for over a period of four years. ‘+ Combined OCP should not be offered to women with risk factor for breast cancer (Risk is increased in predisposed individuals).203. 204. /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS -2007 #493. | Protective/Beneficial role in OCP: Review © Uterine cancer? ‘* Autoimmune disorders of thyroid Ovarian cancer? + Pelvic inflammatory disease? (decreased incidence) + Colorectal cancer® + Ectopic pregnancy® (decreased incidence) ‘© Functional ovarian cysts. « Decreased incidence of Anemia '* Benign breast diseases? _ Usefiul in Menorrhagia / Polymenorrhea® * Fibroid uterus® + Acne® / Hirsuitism® ‘* Rheumatoid arthritis? + Endometriosis® Answer is A (Multiload Cu ~ 375 is a third generation IUD): Park 19/394 ; Repeat AI /2004 Muttiload Cu 375 is a second generation IUD and not a third generation IUD. Mutltiload Cu 375 is ition IUD and not a third generation IUD. Lippes Early devices: « Progestasert * Copper T « Levonova + Copper T-200 Never devices: «Variants of Copper T devices = Tev2206 = Teu-380A * Nova-T + Multload devices = ML~Cu-250 + ML~Cu~375 + The pregnaney rates of both Lij and CuT 200 per 100 women after 12 months of : 3/100 women. ‘Device : "Pregnancy rate Expulsion rate Firs generation ‘© Lippes loop (©) 30 19.1 Second generation + CuT-200 30 18 # NovaT 07 58 © Multiload 250, 0s 22 * Multilaod o1 at ‘Third generation ' Progestasert 18 34 + TUCD can be used for Post coital contraception (Emergency contraception) within § ‘Effective as post coital contraceptive if inserted within 3-5 days of unprotected sex’ - Park ‘Introduction of IUCD within a maximum of 5 days can prevent conception following an accidental unprotected exposure. This is probably through increased tubal peristalsis’ - Dutta ‘+ Effective life of Third generation 1UD, + Progestosert_ Releases 65 j1g of hormone daily «+ Effective life of I year + Levonova _« Releases 20 ig of levonorgesterol daily + Effective life of 5 years — (COGDT) Answer is A (Cu 380 A): Shaw 13/222: Novak 14"/263; COGDT 10"/589; Park 19/394 Copper T380A known as Paraguard has a life span of 10 years — Shaw 13/222494» AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 206. 208. ‘Most Copper IUCD's have an effective life of about 3 vears — Shaw But Specific IUCDs may have different life spans ‘© Progestasert : J year? * Nova T/Multiload 375 Syears? # Levonova (LNG - 20) S years® (approved effectiveness (COGDT) : 5 years) (Mirena/Levonorgesterol Releasing) (These IUCDs may however be effective upto 10 years) (Park) = Cur 380 (4) 10 years (Paroguard) Answer is B (Clomiphene citrate): Shaws 13/215, 304; Dutta Gynae 4/229; KDT 5/278 Clomiphene citrate is an antioestrogen used for induction of ovulation. Shaw/Dutta. ~ Clomiphene citrate = Human Menopausal gonadotrophin (hMG) - FSH and Recombinant FSH ‘Clomiphene? + HCG and Recombinant hCG Tamoxifen (SERM)? GnRH and GnRH analogues Clomiphene Clomiphene citrate is an antioestrogen and acts as a pure oestrogen antagonist in all human tissues (weak agonistic action has also been elicited). ‘+ Itblocks oestrogen receptors in the hypothalamus ‘+ This blocks the negative feedback of oestrogen by the ovaries ‘+ This results in uninhibited GnRH secretion and GnRH stimulates FSH and LH secretion by the pituitary This enhances ovulation. ‘= Anovulatory infertility : Clomiphene is the drug of choice. ® '* Polycystic ovarian disease with infertility. ° To aid in vitro fertilization. ° Answer is A (Isthmus): Telinde's operative gynaecology 9/543 Most common methods of tubal ligation involve ligation and division of the isthmic portion of the tube. Ligation and Tubectomy in the process of tubal sterelization is most commonly preferred in the proximal isthmic ‘portion of the fallopian tube- Telinde's operative Gynaecology Interstitial Isthmus Ampullary Division ofthe Isthmic \ Portion of tube‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 © 495 207, Answer is A (TESA): Shaw 13/205; Novak 14/1201; COGDT 10°/923; Dutta Gynae 4/234 TESA or Testicular Sperm Aspiration is a method to retrieve sperms from the testes. + MESA involves aspiration of sperm from the epidydimis and not from the testes. This isthe latest technology employed in cases of male infertility to retrieve sperms for assisted reproduction. ‘Sperms may be retrieved from the testes or epidydimis by a variety of techniques such as- TESA Testicular Sperm Aspiration TESE —: Testicular Sperm Extraction PESA —: Percutaneous Epidydimal Sperm Aspiration MESA: Microsurgical Epidydimis Sperm Aspiration The most important indication for these procedures is Post Testicular Azozoospermia® - Congenital absence of vasdeferens / ejaculatory duct ~ Blockage / obstruction of vasdeferens / ejaculatory ducts = Retrograde ejaculation + GIFT stands for Gamete Intra Fallopian Transfer This is a method of Assisted reproduction where in both the sperm and unfertilized oocyte are transferred into the Fallopian tubes. + ZAET stands for Zygote Intra Fallopian Transfer This is an alternative method of assisted reproduction where in the zygote (following one day of invitro fertilization) is placed into the fallopian tubes. _AIPGMEE 2007 - EXPLANATIONS 208. Answer is D (Immediate Postpartum): Dutta 6/53 Maximum cardiac output is seen in the immediate post partum period when cardiac output increases to about 70% ‘more than Prelabour values. Cardiac Output During pregnancy During Labour Tmmediate Postpartum ‘Cardiac output during pregnancy is Cardiac output inereases further ~ Cardiac output increases further to about increased by about 40-50% 10 about 50% more than the 70% more than pre labour values. (This peak is reached at 30-34 weeks prelabour values. > Maximum cardiac output is seen in the after which cardiac output remains static immediate post partum period®. till term) + Maximum stress on a compromised heart is seen in the immediate postpartum period. *Cardiac ouipua returns to prelabour values in one hour following delivery and tothe prepregnant level by another 4 weeks ime. 209. Answer is A (Down’s Syndrome) : Callen's Ultrasonography in Obstetrics and Gynaecology 5" / 39 Although most chromosomal abnormalities including triploidy, trisomies 13, 18, 22 and tetrasomy 12 p are ‘associated with nuchal transluscency, the most common aneuploidy seen with an abnormal nuchal thickness is trisomy 21 - Callen’s Ultrasonography What is Nuchal Transluscency? ‘The thickening of the nuchal soft tissues in the first trimester has been termed as “Nuchal transluscency” “Excess soft tissue in the posterior neck in the second trimester is referred to as muchal fold | Thickened nuchal transluscency has many causes but itis most charachteristic for Trisomy 21 or Down's Syndrome496 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 210. 2u1. 212. ses of Increased / Thickened Nuchal Translucency _ “Aneuploidies ‘Other Syndrome. ‘Trisomy 21 : Down’s syndrome Tumer's syndrome Trisomy 18 : Edward syndrome ‘Noonan syndrome ‘Trisomy 13 : Patau syndrome ‘Cornelia de Lange syndrome Trisomy 22 Jouber syndrome ‘Tetrasomy 12 p “Apert syndrome Triploidy Fryn's syndrome The most common Aneuploidy seen [Skeletal dysplasias) with an abnormal nuchal thickness a ‘chondrogenesis is trisomy 21 of Down’s syndrome Ectrodactyly ~ ectodermal dysplasia Multiple pterygium syndrome Robert syndrome Answer is A (RDS): Dutta 6°/316; Williams 22/653 Antenatal administration of glucocorticoids is indicated to reduce incidence of Respiratory distress syndrome. Antenatal administration of glucocorticoids reduce mortality, respiratory distress and intraventricular ‘haemorrhage in preterm infants. — Wiliams To reduce incidence of Respiratory Distress Syndrome (RDS)® To reduce incidence of Intraventricular Haemorrhage (IVH)® [Timing of Glucocorticoid administration a ‘Material administration is advocated when pregnancy is less than 34 weeks. ® Administration is beneficial when delivery is delayed beyond 48 hours of the first dose. Benefit persists as long as 18 days. ‘Choice of steroids: a Betamethasone and Dexamethasone may be used. Betamethasone is the steroid of choice.® Answer is D (Interspinous diameter): Dutta 6"/522 Bishops pre induction cervical scoring system does not include interspinous diameter ~ Dutta /COGDT Bishops Score: (COGDT 10°/209) Bishops score is used to evaluate the cervical status and is important in predicting suecess of induction. EE: Relate eT 1 2 “12 34 40-50 60-70 Ay2 0 Medium: Soft * | Position of cervix Middle Anterior Total score= 13 Favourable score= 6-13 Unfavourable score = 0-5 Answer is C (U) : Callen’s Ultrasonography in Obstetrics and Gynaecology 4%/ 583 ‘U’ shaped cervix on Transvaginal Sonography suggests complete effacement of cervix, and may be a strong indicator of preterm labour if present in a preterm infant.213. AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 © 497 ‘Sonography of the Cervix in Pregnancy * Cervical effacement begins with the dilatation of the Internal Os and is visualized Sonographically as funneling, as the amniotic a sac protrudes into the cervical canal. Closed Internal Os ‘© The letters T, Y, V and U illustrate the correlation between the length of the cervix and changes of the internal cervical Os, graphically. Né Paral Ejeet + The process of complete effacement may be described as *U” G shaped or "V’ shaped depending on the descent of fetal head. ‘Complete Effacement with limited descent of fetal head © ‘Complete Effacement with further descent of fetal head (Strongest indicator of labour) Answer is A (Reversal of diastolic flow): Williams 22/383, 401 Reversed end diastolic flow suggests severe fetal circulatory compromise and carries worst prognosis. Umbilical Artery Doppler Vi ‘+ Umblical artery Doppler can be used to determine blood flow across the umbilical artery. ‘+ This is specially important in pregnancies complicated by fetal growth restriction ((UGR).. Normal Forward Flow ‘The umbilical artery ‘Absent or Reversed flow signifies increasing impedance to umbilical artery blood normally has a forward flow. flow throughout the * This is seen in extreme cases of fetal growth restriction (IUGR) and result from cardiac cycle poorly vascularized placental villi ‘* Reversed end diastolic flow carries a worse prognosis (mortality 33%) in comparison to absent end diastolic flow (mortality = 10%). Most commonly used index for Umblical artery Doppler velocimetry is the Umbilical Artery Systole Diastole (SID) ratio: The umbilical artery normally has forward flow throughout the cardiac cycle and the amount of flow during diastole increases as gestation advances. Systolic flow _ As the amount of diastolic flow increases as gestation advances the S/D ratio Se EY = S/D Ratio Diastolic flow tends to decrease Umbilical Artery Doppler is considered abnormal if $/D Ratio falls above the 95" percentile for gestational Age. Reduced, ABsent or Reversed diastolic flow will increase the S/D ratio with advancing age and indicate abnormal ‘SP Ratio.498 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 214. 215. Answer is D (PIH): Dutta 6/380, 397, 582 ‘Severe preacclampsia or PIH is a contraindicated to External Cephalie Version External Cephalic Versiot ‘+ Extemal Cephal cephalic presentation, version isa procedure to tur the fetal presenting part from a non cephalic presentation to a ‘+ Because cephalic version is performed by manipulating the fetus through the abdominal wall, the maneuvre is known as external cephalic version. 1. Breech presentation: ‘+ Antepartum haemorrhage (placenta praevia or abruption)- risk of placental Successful version is likely separation. in case of complete breech _« Fetal causes: congenital abnormalities (major), dead fetus, hyperextension (eexed breech) that is of the head, fetal compromise (IUGR). unengaged. ‘+ Multiple pregnancy 27 i lie Extemal version is much . © Kni fthe uterus Lapeer paras 1own congenital malformation ofthe u ee a + Contracted pelvis ‘+ Ruptured membranes — with damage of liquor + Previous caesarean delivery- risk of scar rupture * Obstetrics complications: Severe pre-eclamp: primigravida, Bad obstetric History (BOH). sia, Obesity, Elderly Answer is A (Gestational Diabetes): Williams 22/1177; Dutta 6/287; Repeat Al 20¢ her References ‘Caudal Regression syndrome is about 200 times more frequent in patients with diabetes, than in normal healthy ‘women (general population). It is the most specific fetal malformation in maternal diabetes. | Fetal Macrosomia ‘Congenital Malformations [Neonatal Complications) Fetal Macrosomia or «Nervous system» Heart anomalies» Renal anomalies - Hypoglycemia’ large Body® is most = Caudal regression - VSD. ~ Agenesis + Hypocalcemia® ‘common Fetal ~ Sacral agenesis - ASD = Cystic kidney - Hypomagnesemia® ‘association® seen with - Spina bifida, - TGA = Duplex ureter - Hyperbilirubinemia® ‘maternal diabetes. = Hyérocephaly = Coarctation __» Single umbilical - Polycythemia® (Fetal macrosomia is ~ Microcephaly + Gastrointestinal artery = Respiratory alistress rnot considered a = Other central ~ Anal/ectal syndrome congenital nervous system atresia malformation) defect ~ Duodenal ~ Anencephal Aesia Fetal complications® | Most common fetal asso ‘Most common congenital fetal “Most specific congenital fetal with maternal diabetes malformation with maternal diabetes are:__malformation is: ‘Fetal Macrosomia® ‘Central Nervous System and Cardiac ‘Caudal regression syndrome® Malformation (Caudal Regression syndrome is not the most common congenital malformation but it is the most specific congenital ‘malformation associated with ‘maternal diabetes.[AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 © 499 216. Answer is A (Gestational Diabetes): Williams 22/905; Dutta 6"/287; Repeat AI 2005 Fetal macrosomia is the most common fetal association with maternal diabetes. |__ Reta! Macrosomila: Risk Factors | = ‘Maternal Diabetes, Other risk factors This is the most common risk factor for 1. Large size of parents: especially the mother whois development of fetal macrosomia ‘obese (Maternal obesity)? 2. Multiparity 3. Prolonged gestation (Prolonged pregnancy) ® 4. Increased maternal age 5. Male fetus 6. Previous infant weighing more than 4000 g@ 7. Rave and Ethnicity These factors are additive. Among women who are simultaneously Diabetic, Obese and Post term, the incidence of ‘fetal macrosomia ranges from 5 t0 15 percent. 217, Answer is B (Polyhydramnios): Dutta 6/290; Williams 22"/1195; COGDT 10/388: Polyhydramnios has not been mentioned in relation to Hypothyroidism in pregnancy. {Bre ot Hypa on Peiney Abortion (spontaneous abortion) + Abruptio placentae «sill birth + Prematurity /Premature delivery/Low Birth weight + Preeclampsia (Pregnancy Induced Hypertension) + Postpartum Hemorrhage ‘+ Subnormal intelligence in infant (Low 1Q in infant) © Anemia 218. Answer is B (Phenobarbitone): CMDT 2007/799 All antiepileptic drugs eross the placenta, equilibrate rapidly in cord blood and may have teratogenic effects. Phenobarbitone is the single best answer of choice as itis considered to be the drug of choice for epilepsy in pregnant women. + Phenobarbitone is considered the drug of choice for epilepsy in pregnant women — CMDT * Phenytoin is Teratogenic and is known to produce Fetal Hydantoin syndrome (KDT 5/373) Fetal Hydantoin Syndrome: + Facial dysmorphism ‘+ Hypoplastic phalanges + Cleft palate + Cleft tip + Microcephaly + Cardiac defects + Nail dysplasia + Carbamezapine and Valproate are teratogenic and increased incidence of foetal malformations have been reported with both ~ KDT 5/374, 375 ‘Syndrome similar to fetal hydantoin syndrome has also been described with carbamezepine and valproate. Harrison 16th/2371 Increased incidence of neural tube defects are associated with carbamazepine & valproate. —Harrison 16%/2371500» AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 219. Answer is A (Immediate Removal): Dutta 6°/310; COGDT 10°/426 Ovarian cyst during puerperium (postpartum) should be removed as early as possible - Dutta 6"/310 Management of ovarian cysts requiring surgery during pregnancy Diagnosis made in ‘Diagnosis madein [Di Diagnosis made in post First trimester. _Second trimester trimester _ partum (Puerperium) Perform elective surgery in second Perform elective Follow up until the Perform elective trimester® unless complications surgery as soonas time of delivery or surgery as soon as, develop. Best time for elective possible? postpartum possible. surgery (14-18 weeks) (ind trimester) Benign cystic teratoma (Dermoid) : 21% Serous cystadenoma : 21% Cystie corpus futeum zl 18% 220, Answer is C (IlIrd trimester): Dutta 6%/292 The risk of vertical transmission is maximum in third trimester. The risk of transmission of hepatitis B to fetus ranges from 10% in first trimester to as high as 90% in third trimester -Dutta PAEDIATRICS 221, Answer is B (5 months): Nelson 18/49; Ghai 8"/52 ‘Mouthing is seen at 4 months (16 weeks) while stranger anxiety develops at about 7 months. The child has started mouthing (> 4 months) but has not developed stranger anxiety (<7 months) and hence his ‘most appropriate age should be 5 months. Mouthing “4months | Reaches and grasps objects & brings them to mouth (mouthing) (6 weeks) Stranger anxiety (Ghai 6"/47; Nelson 17" / 82) 7-9 months | Most infants exhibit siranger anxiety beginning af 79 mons (Nelson) 7-8 months | Infant shows anxiety on meeting strangers as he becomes 7-8 months (Ghai) _ | old. 222, Answer is C (Mirror play): Ghai 8" / 49; Nelson 18/45, 49 Mirror play is the earliest milestone to develop amongst the options provided. Different text books give different ages for key developmental milestons f confusio ‘This answer is based on developmental milestones as tabulated is Ghai's text book. Mirror Play > At age of 6 months (Infant enjoys watching his own image in mirror) Crawling: At age of 8 months (Crawls ie. without lifing his abdomen off the bed) Creeping > Atage of 10 months (Creeps ie keeping his abdomen off the ground) Pincer grasp At age of 9 months223, 224. /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 © 501 Answer is A (Identify colours): Ghai 8/50, 54; Nelson 18°45, 49 Ghai 6°/46,47; UNICEF Guidelines on Child development and Early learning; Nelson 17/39 A six year old child with an IQ of $0 has a mental age of 3 years. By the age of three years a child should begin to identify colours. ‘What is the child Mental Age? Tee 00 =| Mewal Ace = 12 Chronological AGE ‘Chronological Age 100 50x6 Mental Ag =3, ental Age = US 3 years What developmental Milestones should be achieved by 3 vears, ‘+ Child should begin to Identify color by age of three vears “By the age of three years a child should be able to identify colours’-UNICEF Guidelines on cid developmen & ary learning Nelson mentions that a child at $ year of age should be able 1o name 4 colours, but it does not mention at what cage a child begins to identify two colors and begins to match similar colours at play. This should be achieved at three years according to UNICEF child development guidelines. ‘+ Achild of 3 vears can ircle but not a triangle. He can a triangle at age of 5 years Horizontal or vertical line > Dyears Circle 2 3 years Cross (plus sign) 2 4 years Rectangle 2 4 Years Tilted cross (multiplication sign): 5 years Triangle 2 Syears ‘+ A.child of three vears can ride a tricycle and not a bicycle ~ Nelson ‘A child of three vears can speak a nti n ‘a sentence as mentioned in the option. T year Jargon (child can use 2 words of his own jargon for objects) 2 year : speaks a sentence putting 3 words together (subject, verb, object) 3 year: speaks a sentence with 6 syllables 4 year : can tell a story (coherent account of recent experience and events) Answer is A (3 months): CPDT 18" /7; Ghai 6" /7 ‘Moro’s Reflex disappears by age of 3 months - CPDT 18/7 Moro’s Reflex disappears after age of 12 weeks (3 months) — Ghai 6/146 (Different books different times at which Moro's Reflex disappears and based on the textbooks mentioned above) Moi lex (Startle Reflex Maneuvre Reflex activity Positive Response) |)” Timing of Reflex 7! ‘* The supine infant hands are ‘* There is sudden abduction at the» Moro’s Reflex appears at 28 ~ grasped and shoulders lifted afew shoulder and extension at the elbow 32 weeks® (Becomes fully centimeters lifting the back of the «This is followed by adduction of developed at 37 weeks) head slightly from the bed the arm and flexion at elbow + Moro’s Reflex disappears at by + The hands are then suddenly (he hands show a prominent age of 3 months — CPDT released allowing the head to fall spreading or extension of the (Moro’s Reflex disappears afier momentarily backwards fingers) the age of 12 weeks (3 months) = Ghai Significance ‘© An absent or exaggerated response in a new born is ominous suggesting significant dysfunction of the CNS ® + An asymmetrical response may signify a fracture clavicle, brachial plexus injury or hemiparesis, ®502 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 225, Answer is C (Symmetric Tonic Neck Reflexes): Programs in Pediatrics, University of Washington School of Medicine; Nelson 18"/ 2439,1AP Pediatrics 2/31 ; Ghai 8" /142; Foundations of Orientation and Mobility By Richard L. Welsh, Bruce B. Blasch, William R. Wiener 1"/ 176,177,178 ‘Moro’s reflex, Crossed Extensor Reflex and Asymmetric Tonic Neck Reflex develop in intrauterine life and are resent at birth. Symmetric Tonic Neck Reflex appears later and is not present at birth. + Tonic Neck Reflexes: ATNR Vs STNR Are the Asymmetric Tonic Neck Reflex thing: While most standard textbooks ( Nelson, Ghai, IAP, CPDT) just mention ‘Tonic Neck Reflexes’ they do not differentiate them into the Asymmetric and Symmetric types. and the Symmetric Tonic Neck the same The Tonic Neck Sf = ATNR and STNR The ATNR commonly called the fencing reflex appears earlier than the STNR or the cat's reflex. (Asymmetric Tonic Neck Reflex (ATNR) | ['Symmetric Tonic Neck Reflex (STNR) 7) ‘Tum head to one side : Extension of the Arms and Extension of legs causes flexion of the arms and the 226. ‘Legs occurs on the side of the body corresponding to the direction of the face while flexion ocers in the contralateral extremity (This is often referred to as a fencer's stance.) ATNR is present at Birth Appears by 35 wks gestation, is fully developed at 1 ‘month & lasts 6-7 months head. Flexion of legs causes extension of the arms and the head (This is often referred to as the cat's reflex) SINR is not present at Birth STNR is present between 2 months to 12 months of age ‘+ Crossed Extensor Reflex (Ghai 6" / 146) When the foot is stroked while the leg is kept extended at the knee, there is rapid flexion, adduction and then extension of the opposite leg. This reflex is present at birth. It appears at around 32 weeks of gestation and is completely developed (adduction component) at around 36 weeks of gestation ‘© Moro’s Reflex : (Elaborated in the previous question) Moro’s reflex is present at birth. Timing of Selected Primitive Reflexes + Palmar Grasp ‘+ Symmetric Tonic Neck Reflex + Rooting / suckling + Landau Reflex + Moro’s Reflex «Neck Rightening Reflex + Asymmetric Tonic Neck Reflex . Crossed extensor Reflex Answer is A (Constitutional): Ghai 8 /35-36; CPDT 18*/938 ‘Short stature may result from normal variants of growth (Familial Short Stature and Constitutional Growth Delay) or from Pathological Conditions. Physiological short stature resulting from normal variants of growth is more common than pathological short stature. Amongst the options provided constitutional delay is the only example of physiological short stature and is hence the single option of choice.227. 228. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 © 503 Short Stature ‘© Familial short stature -————_—— (Proportionate) « Contectaat ite Proportionate Disproportionate =a etn eT a se « Intrauterine growth retardation ‘* Achonodroplasia 2 } Intrauterine infections «+ Hypochondroplasia 6 ‘© Genetic disorders (chromosomal and» Chondrodysplasia punctata & ‘metabolic disorders) + Chondroectodermal dysplasia % Postnatal causes « Diastrophic dysplasia & «Nutritional dwarfism + Metaphyseal chondrodysplasia. i * Chronic visceral disease (renal , + Deformities due to rickets and 5 cardiopulmonary, malibsontion, chronic» Oncogeness imperfect, s infections and anemias Giine Se Exctae diodes Witton wk ___ ig ‘* Psychosocial short stature (emotional ‘+ Spondylo ~ epiphyseal dysplasia 3 deprivation) (congenital and tardavarieties), = + Mucopolysaccharidosis, et + Mucolipidosis, Caries Spine and Hemivertebrae. — Answer is B (Height for age): Ghai 7°/62; Park 19/434 Defecit in height for age (Stunting) signifies accumulated consequence of retarded growth over some years and points towards a chronic, course of malnutrition (long term nutritional status indicator) ~ Ghai 6" / 101 Deficit in weight for height usually signifies acute onset malnutrition, while Weight for age & Midarm circumfrence ‘measurements do not indicate whither malnutrition is acute of chronic © Weight for Height and Height for Age masurement Measurement of Malnutrition (Currently accepted nomenclature) Deficit in weight for Height Deficit in Height for Age (Wasting) (Stunting) Indicates deficit in tissue and fat mass and may result Indicates accurmulated consequence of retarded growth failure to gain weight or from actual weight loss over some years Ic usually signifies Acute onset malnutrition, It usually signifies a chronic course of malnutrition + Mid Arm Circumference: This is an age independent anthropometric measurement? Tis a useful one time indicator to asses nutritional status where exact age is not known. ‘Normal Mid arm cireumferance between ages 1-5 years > 13.5 em + Weight for Age: Malnutrition reflects on the weight ofthe child rather quickly and a single weight for age ‘measurement does not give an indication whether, malnutrition is of an acute onset or chronic. Answer is A (Leukemias): Nelson 17" / 1694 The leukaemias are the most common malignant neoplasms in childhood accounting for 41% of all malignancies ‘that occur in children younger than 15 years of age - Nelson 17*/ 1694 ALL is the most common childhood Leukaemia accounting for 71% of cases of childhood leukaemia.‘504 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 | 29, Answer is D (Tut syndrome): Ghai 8/605; Harrisons 16" /631 Turner's syndrome has not been mentioned in association with increased incidence of Acute Myeloid Leukaemia, = Heredity associations with Acute Mycloid Leukaemia (AML) L ‘Syndromes with Somatic Cell Aneuploidy ‘Down's syndrome (chromosome 21 Trisomy) Tnherited diseases with Excessive Chromatin Fragility Fanconi's anemia’ ‘Patau syndrome (chromosome 13 Trisomy) ® Bloom syndrome® Klinefelter syndrome (XXY and variants) ° Ataxia Telengiectasia® Kostmann syndrome & Answer is C (Pre Bull ALL): Devita 6/2240, 2237; Nelson 17%/1695; Ghai 8"/600; Harrison 16" ; Wintrobe's TIM2145, 2142 Hyperdiploidy, Female sex and translocation t (2, 21) are associated with good prognosis. Pre B cell ALL may be associated with a poor prognosis and is the single answer of exclusion. nslocation 2,21 with good pri White blood cell count <10 x 10%, > 2000 x 10°/L Age 3-7 yrs <1,>10yr Gender Female Male Ethnicity White Black Node, Liver, spleen enlargement Absent Massive Testicular enlargement Absent Present Central nervous system leukemia Absent Overt (blasts + pleocytosis) FAB morphological features LI RL Hyperdiploidy Hypodiploidy < 45 Trisomies 4, 10 and/or 17 4(9; 22) [ber abt] (12,21) (tel aml 1) (45 11) [mil af) 28d i <104 > Wasnt cea associated with an unfavourable prognosis (although it ma Pre B cell ALL has been associated with a poorer outcome compared to Early Pre B cell ALL. ~ Wintrobe's Immunophenotype and Prognosis > [Bir Beara eB EAALE TY ae pe alt RIALS Early Pre B cell All Pre Bell ALL account for Mature B cell ALL in Toell ALL accounts for comprises about 2/3 cases 20% of childhood ALL and uncommon in children and 15% of ALL in children of childhood ALL and is have been associated with a accounts for 1-2% of T cell ALL was associated with a ‘poorer outcome compared childhood ALL. (previously) associated favourable prognosis. to Early Pre B cell ALL. Itis characterized by L3 with a poor prognosis but FAB morphology and has a recent use of more “Most Early Pre B cell ALL ‘poor prognosis. intensive therapeutic are CALLA positive. regimen has resulted in CALLA expression does survival approaching that not appear to have of Non T cell ALL. independent prognosis significance.231, 232, 233, ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 * 505 Answer is C (Fever): Ghai 8°/435, Nelson 18"/1141; Harrison 16" / 1978 Fever is a Minor Jones Criteria for diagnosis of Rheumatic fever, The Jones Criteri Jones criteria were first proposed in 1944 The most recent modification of the Jones criteria was published in 1992 and these were termed updated Jones criteria, Updated Jones Criteria for Rheumatic fever + Carditis ‘+ Fever (clinical) ‘© Migratory polyartbrtis «© Arthralgia (clinical) + Sydenham’s chorea ‘* Elevated acute phase reactions (LAB) + Subcutaneous nodules + Prolonged PR internal (LAB / ECG) + Erythema marginatum (Essential Criteria ‘Supporting evidence of recent group A streptococcal + Positive throat culture or rapid antigen detection test and / or » Elevated or increasing streptococcal antibody test. ajor criteria And Two minor criteria Essential criteria are required, Answer is A (ASD): Dorland's Medical Dictionary 28/1253, 1746; Stedman's Medical Dictionary 28/1452 Pentalogy of Fallot encompasses the four defects included in Tetralogy of Fallot together with patent foramen ‘ovale or atrial septal defect. + Tetralogy of Fallot: Ghai 6% T of Fallot © Ventricular septal defect # Pulmonic stenosis * Overriding or Dextroposed Aorta Right Ventricular Hypertrophy + Pentalogy of Fallot (Dorlana's 26/1253; Stedman's 28/1452) ‘Association of four defects occurring in Tetralogy of Fallot with either ASD or Patent Foramen Ovale. “Airial Septal Defect (ASD) Pantalogy ofFallot | = | Tetralogy of Fallot | + OR Patent Foramen Ovale ‘+ Trilogy of Fallot: Dorland's 28/1746; Stedman's 28/2033 ‘Applied to the combination of Pulmonic Stenosis, ASD and Right Ventricular Hypertrophy Trilogy of Fallot ‘© Pulmonic Stenosis * Atrial Septal Defect (ASD) Right Ventricular Hypertrophy Answer is D (Lowe’s Syndrome): Nelson 17" / 1572, 1760; Ghai 7%/423 Cardiomyopathy is not a feature of Lowe’s syndrome or OculoCerebroRenal Syndrome. Cardiomyopathy is a common association with glycogen storage disease like Pompe's disease, Muscular dystrophies like Duchene's and Freidriech’s ataxia. (Refer table 43 1~ 1 Nelson 17 / 1573)506 + 238. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 + Lowe's syndrome: Oculocerebrorenal syndrome of Lowe (No cardiomyo, This is a rare X lined disorder characterized by = Congenital cataracts (Oculo) ~ Mental Retardation (Cerebro) Cardiomyopathy is not associated with Lowe's syndrome —=Fanconi's syndrome (Renal) _ * Cardiomyopathy is common in Fredriech’s ataxia (Nelson 17" / 1573) “Heart disease is common in patients with Freidriech’s ataxia which chiefly affects left ventricle and results in dilated or restrictive cardiomyopathy’ - Nelson 17"/1573 + Cardiomyopathy is common in Duchenne’s Muscular Dystrophy (DMD) (Nelson 17" / 2060) ‘Cardiomyopathy is a constant feature with Duchenne’s and Beckers’ muscular dystrophy’ ~ Nelson * Cardomyopathy is common in Pompe’s disease - CPDT 18" /506 “Pompe’s disease is Glycogen storage disease (GSP Ha) that primarily involves the heart. There is marked deposition of glycogen within the heart (Hypertrophic cardiomyopathy) - CPDT 3Answer is A (Inhaled Short acting beta 2 agonists): Ghai 8” / 391 Inhaled short acting beta 2 agonists like salbutomal are the intial drugs of choice for management of asthma exacerbation ‘Successful management of Asthma requires grading of severity of disease however the question does not give us any information on the severity or grade of Asthma. Amongst the options provided the drugs of choice to provide immediate symptomatic relief are Short acting Beta — 2 agonists and hence the answer of choice. Management of Asthma Immediate Symptomatic Relief Long Term Rei Delayed Symptomatic Relief ‘Short Acting beta 2 agonists = Sabuamote + Long acting Beta 2 agonists + Terbutaline = Salmetrol Adrenaline = Formoterol ‘+ Theophylline / Aminophylline # Steroids * Inhalation rout is preferred because of quick onset of action and least side + Beclomethasane ‘effects. ~ Budisonide = Fluticasone © Mast cell stabilizers = Sodium cromoglycates = Nedocromyl sodium = Ketotifen + Leukotriene modifiers = Monteleukast = Zafarilukast = Zileuton Answer is A (Zafirlukast): KDT 5/199, 205; Katzung 10°/325 Zafirlukast and Montelukast are Leukotriene Receptor Antagonists used in Bronchial Asthma, Zileuton is a ipoxygenase inhibitor, and Nedocromil and Ketotifen are mast cell stabilizers. Leukotriene Antagonists Mast Cell Stabilizers Leukotriene Receptor Antagonists" Lipoxygenase * Sodium Cromoglyeate (LTD4 Receptor Antagonist) Inhibitors + Nedocromil © Montelukast (5-LOX Inhibitor)» Ketotifen + Za firlukast ilewon236. 237. /AIPGME EXAMINATION ANSWERS AND EXPLANATIONS 2007 * 507 Answer is B (Laryngotracheobronchitis): Ghai 8/3 felson 18"/1763; Dhingra Prodromal Symptoms of Upper Respiratory tract infection preeding sings of respiratory tract obstruction (stridor) is characteristic of Laryngotracheo bronchitis or croup, Although Laryngotracheobronchitis is not relieved on lying down it appears to be single best answer of exclusion. ‘+ Laryngotracheobronchitis present with prodromal URTI Symptoms prior to the onset of stridor ‘Most patients with Laryngotracheobronchitis have an upper respiratory tract infection for 1 10 3 days before signs and symptoms of upper airway obstruction (barking cough, hoarseness and inspiratory stridor) dvelop’. = Nelson 5 "The child may prefer to sit up in bed or be held upright’ ‘+ Acute Epiglottitis is an unlikely diagnosis here because it has an acute onset and fulminant course with no prodromal symptoms of URTI and the is not relieved by lying down. ‘The child may assume the tripod position sitting upright and leaning forward with chin up and mouth open while | bracing on the arms'~ Nelson | + Foreign body aspiration is an unlikely diagnosis hers as itis usually not associated with any prodromal ‘symptoms of URTI. Also sudden choking and cough are the prominent manifestation along with sudden onset stridor and these have not been mentioned in the question. + Retropharyngeal Abscess is unlikely as it usually presents with nonspecific symptoms including fever, irritability, decreased oral intake drooling, Neck stiffness and refusal to move the neck. Siridor and respiratory distress may be associated. Although prodromal symptoms of URTI may be present there are not characteristic as in the case of Laryngotracheobronchiti. Also symptoms of Retropharyngeal Abscess have not been mentioned to improve on lying down and are likely to _get worse in the supine position. None of the conditions mentioned as options are likely to improve on lying down. The presence of prodromal ‘symptoms of URTI preceding the onset of stridor suggests Laryngo tracheobronchitis as the single best answer of exclusion. Answer is A (Vein of Galen Malformation): Volpe’s Neurology of the Newborn 3/802 ‘Diagnosis of Vein of Galen Malformation should be suspected in any newborn presenting with unexplained congestive heart failure and Hydrocephalus’ ~ Neurology ofthe Newborn The Newborn in question is presenting with congestive heart failure and hydrocephalus (bulging anterior ‘Sontanelle and dilated lateral ventricles) thus suggesting a diagnosis of Vein of Galen Malformation. Presence of a ‘hypoechoic mass in the region of the vein of Galen (midline) confirms the diagnosis. (Dy Mat is the Vein of Galen. i Vein of Galen (Great Cerebral Vein) is formed by the confluence of the two internal cerebral veins and receives the entire deep venous drainage of the cerebrum. ‘Vein of Galen then joins the inferior saggital sinus and empties the venous drainage into the straight sinus. ‘Vein of Galen Malformation is characterized by Aneurysmal dilatation & Arteriovenous malformation of the vein of Gale. This malformation most commonly presents in the Neonatal period (40% - 50% of all Paediatric cases),508» AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 239. (tier Diletation 7 4-V Malformation ‘Marked Continuous Cranial Bruit ‘© High output cardiac failure + Hydrocephalus hed '* Bounding Carotid Pulse Increased Intra Cranial Pressure eae This is believed tobe due tothe This is secondary to the mass effect Intraventricular haemorrhage from ‘marked decrease in of the malformation resulting in rupture. cerebrovascular resistance and ‘obstruction of the CSF pathways. © Couiral bekterayrom incesasod venous coturn to the Intracranial ‘Steal’ phenomenon and CHF, '* Thrombosis of vein of Galen Diagnosis of vein of Galen Malformation should be suspected in any new born presenting with unexplained Congestive Heart Failure and Hydrocephalus or Intraventricular haemorrhage — Neurology of Newborn. Intracranial ‘Steal’ phenomenon refers to intracranial Ischaemia resulting from absence or even reversal of cerebral blood flow during diastole due to decreased cerebrovascular resistance and congestive heart failure. . Ultrasono; the of choice ‘The malformation presents as a large, echoluscent (hypoechoic) mass in the midline (Region of vein of Galen) © CT, MRI and Angiography may be used to define the lesion better. Answer is A (Streptococcus Pneumoniae): Nelson 17” / 2038 Bacterial meningitis in children 2 months ~ 12 years of age is now most commonly caused by S. pneumoniae and N- meningitidis - Nelson Causes of Acute bacterial Meningitis in children First two months of life Group B & D streptococci Gram negative enric bacilli Listera monocytogenes Occasionally H.influencae S. pneumoniae N,meningitides sia Hinfluencaypeb ‘Most common cause of bacterial meningitis between 2 months 10 12 years of age now is. pneumoniae followed by N, meningitides = Subsequent o implementation of universal immunization against H. influenzae type B, beginning at about 2 months of age the incidence of meningitis has dropped precipitously. = Subsequent to recommendation of universal use of pneumococcal vaccine beginning at 2 months of age, the incidence of meningitis caused by the pathogen is decreasing, Answer is B (Frequency of Movement): VOLPE's Neurology of the Newborn 3/181 Jitteriness can be distinguished from seizures by the type of movement but not by the frequency of movement. Jitteriness is a movement disorder of newborns that is characterized by episodic tremors (occasionally clonus) Jitteriness is not a type of seizures but is often confused with convulsions (seizure) Jitteriness is characteristically a disorder of newborns (Rarely if ever seen at a later age)240. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 * 509 ee oe nga Fern fom setae Jtteriness can be readily distinguished from seizures at the bed side ‘with help of following 5 points: Seizure Jiaeriness is exquisitely stimulus sensitive while seizures are not Abnormality of gaze The dominant movement in Jitteriness is tremor while the dominant _ of eye movement ‘movement in seizures is clonic jerking ‘Movernents + ‘= Jiteriness movements (tremor) can be stopped by passive flexion of _ exquisitely stimulus ‘the affected limb while convulsive movements will not cease with sensitive this maneure Predominant Tremor Conic ‘© Jiteriness is not accompanied by autonomic changes while seizures movernent jerking ‘are ofien accompanied by such changes (eg. Tachycardia, Increase Movements cease + in B.P., Apnoea, cutaneous vasomotor phenomena, Pupillary with passive flexion change, Salivation or Drooling) Autonomic changes 0 ‘+ Jtteriness is not accompanied by Ocular phenomena which seizures are usually accompanied by Ocular phenomenon (eg. Gaze fixation or Eye deviation) [What are the causes for Jtteriness “The most consistently defined causes for Jtteriness are ‘© Hypoxic ~ Ischaemic Encephalopathy © Hypocaleemia © Hypoglycemia © Drug withdrawal Answer is A (Phenylketonula): Nelson 18 / 529; Ghai 8" / 652; Lippincott's 3"! /267 The patient is presenting with manifestations of classic phenylketonuria, A positive Ferric chloride test (Guthria test) also indicates the diagnosis of phenylketonuria. Phenylketonuria © Phenylketomuria isthe most common clinically encountered inborn error of amino acid metabolism®™ + Itisan Autosomal Recessive disorder® 2 _Itis caused by deficiency of Phenlyalanine Hydroxvlase®, the enzyme that converts phenylalanine to tyrosine. Phenylalanine Hydroxylase ‘Tyrokinase Phenylalanene ——fJ——+ Tyrosine ——————+ Melanin Phenyiketonuria Phenylalanine cannot be converted into Tyrosine due to deficiency of Phenylalanine Hydroxylase® Deficient Tyrosine Tyrosine High levels of (Hyper phenylalaninemia) Tprosinase ‘competitively inhibit phenylalanine Phenylalanine > 20mg / dl/24 hrs on 2 occasion Tyrosinae, (First step in Elevated Phenylalanine in _Altemate mechanism of Melanine {formation of melanin train ss interferes wth phenylalanine catabolism ‘from prosine) transport of other {important amino acid to Deficient Melania brain (tyrosine, Patients with PKU show tryptophan) and thereby Phenyl pyruvate deficiency of pigmentation causes CNS abnormalities Phenylacetate + Fairskin CNS URINE * Fair hair/ Blonde ‘Mental Retardation®™ * Give urine + Blue eyes Microcephaly® charachteristic musty Seizures! ‘mousy’ odow® * Brain isthe main organ affected by Hyper Hyperactivity? «Detected by gutheric or phenylalanersia® Tremors {ferric chloride test * Phenylalanine metabolites are not directly toxic tothe Growth Retardation ® (Positive ferric brain? chloride test)®510» AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 241. 242. Answer is A (Alkaptonuria): Nelson 18" / 534; Ghai 8" /653; Lippincotts 3/272 The classical sign of Alkaptonuria is darkening of urine to almost a black colour on stan ino acid metabolism ‘Alkaptonuria isa rare in born error of Itis an Autosomal Recessive disorder® Itis caused by deficiency of homogentsic acid oxidase. ® (An enzyme involved in degrative pathway of Tyrasine) Homogentesi aid oxidase Tyrosine ————+ P-Hydroxy phenyl + Homogentesic ————+ Maleyl Aceto Pyruvicacid PHPPA oxidase] acid Acetic Acid Defect in Homogentesic acid a leads to accumulation of Homgentesic acid [Three Characteristic Manifestations _ | [ Oehronoais® “Area? omogentesic Adidurig™ ——] * Darkening of tissue due to slow «Pigment deposit iat the * Patients urine contains elevated accumulation of the blac polymer of antculrcatage resulting in levels of Homogentesic acid homogentesic acid in catlage and other __deyeneratve arthritis hich i oxidized to dark mesenchymal tissues Large joints ike Spine ip pigment on standing (specially [= Dark Black spots in scerd®—] |__ Knee are usualy imolved. iturine pHi alkaline) + Diffuse black pigmentation ofthe + Urn shows Blak reaction with conpmctive, comes and wor conage® Fehlings or Benedict reagent Clinical Manifestations of Alkaptonuria (Arthritis, onchronosis) usually present in mid Adult lif. The only sign of this disorder in children is darlunesy of urine to almost biack colour on standing? Answer is A (VDRL for Mother and Baby): COGDT 10" /667; CPDT 18" /56 Prematurity, bullous lesions on the skin and Periostitis on X-Ray suggest a diagnosis of congenital syphilis. VDRL for Mother and Baby is the single best investigation for establishing the diagnosis of congenital syphilis ‘amongst the optioons provided. Congenital syphilis and Early manifestations (First two years of life ‘Manifestations of congenital syphillis have traditionally been derided into early and late stages. 1 ‘Early Manifestation Tate Manifestation fi ‘Result from transplacental spirochactemia ‘+ Result from chronic inflammation of Bone, Teeth Appear during first two years of life and the CNS ‘Appear gradually during first two decades ‘+ The child in question is presenting with Early sings of congenital syphilis Early Manifestations of Congenital Syphilis ‘© CNS imolvement? Renal involvement * Gastrointestinal Jaundice? [Coombs Neganve®] Ovteochondritis @ Mucocutaneous rash with Toombs Negative " vanstows macalopapular —_iawolvement Elevated liver enzymes |ioomolytic omemia | Periosttis! seythematous maclopapul Sy © Exeinvotement Osteochondits is ZLsquammationinvobing = © Non immune Hydrops painfull and ofen. ands and feet are common,® # Testicular masses results in refusal to move the involved Mucus patches, Rhinitis extremity (snufMes) and condylomata are (Pscudoparaysis of Why characteristic Parroi) DRL for mother and baby is the best investigation for detecting is t the options provided243. 244, 245, [AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 #511 Answer is A (EDTA): KDT 5%/812-815; Nelson 18/2916 The child in question is presenting with signs and symptoms of Plumbism as suggested by blue line on gums (Bertonian line), Gastrointestinal manifestations like constipation, and symptoms of Lead encephalopathy (seizures, drowsiness) Choice of chelation agent is based on Blood lead levels, which have not been provided in the question. DMSA is the chelating agent of choice for Blood lead levels (BLL) between 45 and 70 pil and EDTA is the agent of choice ‘for BLL > 70 wl. According to Nelsons, changes in mentation, lethargy and seizures suggest blood lead levels of > 70 wl. As the patient in question is presenting with lethargy, changes in mention & drowsiness, he is likely to ‘have BLL > 70 wal and hence EDTA is the single most important agent for treatment. Chelation therapy for Plumbism ‘Guidelines for selecting children for chelation depend on Blood lead levels (BLL) ‘+ Chelation therapy is indicated for children with venous BLL > 45 idl Children with BLL between Children with 45 and 70wal BLL > 70 wal DMSA (Succimer, Chemet) is Calcium EDTA ithe most effective agent) | Veadache, change the drug of choice in mentaleon, DMSA (succimer) is an orally EDTA isa parentally administered chelating lethargy, seizures administrable agent. /t is now the agent. It is the recommended agent of choice for | CO™# suggest BLL recommended agent of choice for ead encephalopathy and BLL > 70wdl >10 wl -Nelson chelation therapy of plumbism except in cases of markedly > raised BLL or lead encephalopathy. No evidence of Evidence of Encephalopathy Encephalopathy Consider ‘Combine BAL supplementation of with EDTA ‘MSA or BAL along eprEnaL with EDTA EDTA BAL/DMSA Answer is D (Partial Trisomy 22) : Dorland’s 28/1626 Cat's eye syndrome is associated with partial trisomy 22 i. the presence of a partial additional copy of chromosome 22- Dorlands [GaisiBje Syndrome: Partial iisomy of chromosome 22) + Coloboma of iris (resembling vertical pupils of a cat) ‘+ Downslanting palpebral fissures ‘+ Hypertelorism © Anal Atresia ‘+ Congenital Heart Disease ‘+ Renal Malformations Skeletal Abnormaliti Answer is A (Fabry’s disease): Harrisons 15" / 391; 14" / 380; Nelson 17%/ 2207 (Not elaborated in Harrison 16) Fabry’s disease is a inherited as an X linked disorder. It does not show Autosomal dominant Inheritence. Marfan’s syndrome, Osteogenesis imperfecta are autosomal dominant. Ehlers Danlos syndrome is a group of genetically hetrogenous disorders that usually show autosomal dominant inheritance. However, autosomal recessive, and X-linked inheritance may also be seen.- EXPLANATIONS. 512 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 ‘Autosomal dominant disorders: "Aiea csi ard ¢ He + Familia Hypercholesterolemia Deafness Hemophilia A ? (recessive) = Hereditary nonpolyposis colon cancer = Albinism @ = G6PD deficiency 2 (recessive) ~ Polyposis of the colon = Wilson's disease ® ~ Ducchene/Becker muscular = BRCA 1 and BRCA 2 breast cancer =~ Hemochromatosis ° dystrophy ° (recessive) = Marfan’s syndrome ® = Sickle cell anemia? = Fabry’s disease? = Hereditary spherocytosis ® = Bthalassemia® = Ocular albinism = Adult polycystic kidney disease = Qpstic fibrosis ® + Testicular feminization syndrome + Huntington's chorea ® Hereditary emphysema (a, ~ Chronic granulomatous disease = Acute intermittent porphyria ® antitrypsin deficiency) = Hypophosphatemic rickets 2 = Osteogenesis imperfecta tarda ® + Homocystinuria 2 (dominant) = von Willebrand's disease - Freidrich's ataxia + Fragile-X syndrome © (recessive) = Mvotonie dystrophy ® = Phenylketonuria® ~ Color-blindness @ = Neurofibromatosis ° = Fanconi’s Syndrome — = Tuberous sclerosis ® (H 14%e P-2201] = _ Otospongiosis °’ otosclerosis > Gaucher's Disease — + Achondroplasia [H 14%e P-2174) Ehlers Danlos syndrome: This is a group of genetically heterogenous connective tissue disorders that may show Autosomal Dominant, Autosomal Recessive or X linked inheritance (Usually Autosomal Dominant) - Nelson 17°/2207 Ithas been classified into 10 types Type I, I, Il, & VU are Autosomal Dominant disorders. Type IV and type VI show botk Autosomal Dominant & Autosomal Recessive types. Type VI & X are Autosomal recessive. Type V & IX are X-linked. Answer is D (Uniparental): Nelson 174/389 In uniparchtal disomy child may have an autosomal recessive disorder when only one parent is actually a carrier of that recessive disorder — Nelson 17/389 What is Uniparental Disomy? Uniparental Disomy (UPD) is the term used when both chromosomes of a pair of chromosome in a person with normal number of chromosomes have been inherited from only one parent (Normally one chromosome of a pair is inherited from each parent) «The two chromosomes inherited from the same parent may be identical (Uniparental Isodisomy) or different (Uniparental Hetrodisomy) + In Uniparental Isodisomy both chromosomes in the pair are identical, consequently the genes on both chromosomes are also identical ma iparental Disomy nfluence inheritance of Autosomal R + Let us assume a parent is a carrier of an Autosomal Recessive disorder + Ifthe off spring has uniparental disomy with Isodisomy for a chromosome that carries an abnormal gene, the abnormal gene will be present in two copies in the offspring and the resultant phenotype in the offspring will be that of an Autosomal Recessive Disorder. ‘+ Inother words the off spring will manifest the Autosomal Recessive disorder when only one parent is actually a carrier of the Autosomal Recessive disorder. Autosomal Recessive disorder (One parent in carrier and other parent is normal ECO _ Normal Autosomal Recessive Uni isodisomy for cl {inheritance i (A*) in one. ‘No offspring will be affected as none C1 will be A* A Offspring with Isodisomy will be | { Other offipring will not affected be affected‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 ¢ 513 Examples of Autosomal Reecessive disorders that have ocurred due to Unigarental disomy (UPD) - Nelson ‘Spinal muscular atrophy Opstic fibrosis Cartilage Hair Hypoplasia Alpha and Beta thalassemias Bloom syndrome 247, Answer is C (Mitochondrial Inheritance): Nelson 17"/380 ; Harrison 16/374, 375, Refer Q 41/AI-2006 The Pedigree chart shows that the disease is inherited only from an affected mother. Both male and female children from affected mother inherit the disease. The disease is not inherited to any child from an affected father. Such inheritance is classical of Mitochondrial inheritance. Inherited Mitochondrial diseases are transmitted in a matrilineal fashion. All children from an affected mother will inherit the disease, But it will not be transmitied from an affected father to his children — Harrison 16 /374 Mitochondrial Inheritance: Mitochondrial inheritance Results from DNA present in mitochondria. Classical Pedigree chart for Mitochondrial inheritance as © elicited in Harrison * Mitochondrial DNA is the only non-chromosomal DNA in human celts? DNA in humans may be found either in the Nucleus (Nuclear DNA) or in the Mitochondria (Organelle DNA) ‘© Mitochondrial DNA,is always maternally inherited. ° Mitochondrial and nuclear DNA are located in different places in the cell. During fertilization, the sperm and egg én cell nuclei fuse to form an embryo. The egg cell is very large compared to the sperm, so although the cells! nuclei fuse, the rest ofthe cell mass in the embryo comes from the egg only. Nuclear DNA is therefore co- inherited but the mitochondrial DNA, which i located outside of the nucleus, is always maternally inherited because all mitochondria in a foetus and later adult are derived from the mitochondria in the mother's egg. All children from affected mother will inherit the disease but it will not be transmitted from an affected father to his children.~ Harrison's 16"/374 m All children from an affected mother will inherit the disease, children from an affected father will not inherit the disease. ORTHOPAEDICS 248. Answer is D (Knee): Maheshwari 5"/147 Tube cast is synonymous with cylinder cast and is used for fractures around the knee such as undisplaced fracture patella Plaster casts and their uses: Namie of cast Use e Minerva cast Cervical spine disease Risser’s cast Scoliosis Tum-buckle cast Scoliosis Shoulder spica* Shoulder immobilization Usslab Fracture ofthe humerus (Hanging cast Fracture ofthe humerus Colles cast Cole's fracture (Hip spica Fracture ofthe femur Cylinder cast (Tube cast) Fracture of the patella (Fracture around the knee) ((Patellar-tendon bearing east() Fracture of the tibia "A spica is cast where a limb and apart of the trunk are included, hip spca, shoulder spiea514 © _AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 249. 250. 251. Answer is B (Infrared Therapy): Maheshwari 5°81 Infrared therapy provides surface heat and not deep heat ~ Maheshwari Heat therapy ‘Surface Heat Deep Heat | ‘Only superficial structures Le. the skin and Deeper ssa aed subcutaneous tissues are heated Short wave diathermy + Hot water bottle * eat generated by high frequency ‘© Warm bath Alternating current) « Hot soaks or compresses * Ultrasonic therapy + Infrared lamp + Microwaves «Wax both Answer is A (Ulnar nerve): Maheshwari 5"/64 Claw hand is classically associated with Utnar nerve palsy. Claw Hand (Main — en ~ griffe): + Claw Hand isa deformity of the hand characterized by: at ‘and flexion at the int * Claw hand is caused by paralysis of Lumbricals? ‘Lumbricals cause flexion at metacarpophalangeal joint and extension at interphalangeal joints + Lumbricals are supplied by both ulnar nerve and median nerve Lateral two lumbricals (I" and 2") : Supplied by Median nerve™ Medial two lumbricals (3 and 4%) : supplied by ulnar nerve? Paralysis of ulnar nerve therefore leads to paralysis of medial two lumbricals and produces ulnar claw hand. Paralysis of both median nerve and ulnar nerve produces Complete or Total claw hand ‘* Partial Claw Hand or Complete / Total Claw Hand Ulnar Claw Hand ® Note that Median nerve palsy alone also produces clawing of the 1" and 2™ fingers but this has not been classically described as Claw hand. © Ulnar claw hand shows the UInar paradox? ‘Lower is the lesion in the ulnar nerve greater is the clawing. “Higher is the lesion in the ulnar nerve lesser is the clawing. Ulnar nerve also supplies medial half ofthe flexor digitorum profundus which are long flexors of the medial two fingers. In low ulnar nerve lesions the long flexors of the fingers are spared and hence there is more marked ‘unopposed flexion at the inter phalangeal joints causing more severe clawing. Finger flexors are paralysed in high ulnar nerve lesions & hence the flexion deformity at fingers (in claw hand) is less marked. Ans is B (Lateral cutaneous nerve of thigh): Apley 8 / 252 Meralgia Paraesthetica results from compression neuropathy of the lateral cutaneous nerve of thigh. al © The Lateral cutaneous nerve of thigh can be compressed as it runs through the inguinal ligament , just medial to the Anterior superior liae spine (A $ 1) ‘+ The patient complains of numbness , tingling or burning discomfort over the anterolateral aspect of thigh ~ Meralgia Paraesthetica. + If the symptoms are trouble some the nerve may have to be released.‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~2007 © 515 252. Answer is A (Femoral Nerve): Apley 8 / 246 ~ 252 Femoral Nerve has not been mentioned to be involved in Entrapment syndromes Entrapment syndromes result from entrapment and compression of nerves wherever peripheral nerves traverse fibroosscous tunnels. aaa ‘Less common Site + Carpaltunnel : Median Nerve + Tarsal tunnel Posterior tibial Nerve (Carpal tunnel syndrome) (Tarsal tunnel syndrome) | ‘+ Cubital tunnel: Ulnar Nerve Inguinal ligament : Lateral Cutaneous Nerve of (Cubital tunnel syndrome) Thigh f : (Meralgia paraesthetica) Other sites of Compression of Ulnar & Median nerve = + Supra scapular: Suprascapular Nerve Notch * [+ Median nerve ‘© Structures of elbow: + Neck ofFibula : Common Peroneal Nerve & (Pronator syndrome), or Ligament of struthers | * Fascial tunnel of : Superficial peroneal Nerve J» Anterior interosseus nerve | - Bicipital Aponeursis a Y eases ouccaarps + Thoracic outlet: Roots of Branchial plexus | 5 + Arcade of Frohse : Posterior Interosseus Nerve | Je _Ulnar Necve, 2 Guyon’s canal (Proximal edge (Posterior Interroscous. | ‘of supinator) Syndrome) Compression neuropathy may be seen from compression of digital nerves in the foot (eg Morton's metatarsalgia) 253. Answer is A (Calcaneum): Apley’s 8 / 750, 751; Rockwood and Green 5" / 2135; John Ebnezer 2™ 146 Bohler’s Tuber Joint Angle and Crucial Angle of Gissane are measured for Intra Articular Fractures of Calcaneum Radiological angles on Lateral view of Caleaneum Bobler’s Angle / (Tuber Joint Angle) (Crucial Angle of Gissane cacareus Lateral ress Lutonipocses of Gissane + This angle is formed between two lines + This angle is formed between First line is drawn between the highest part of the ‘Downward portion of the posterior facet and the anterior process connecting to the highest part of Upward portion as depicted in the figure the posterior facet or articular surface (This angle wedges the lateral process of Talus) 2 ‘Second line is drawn from same point on the posterior facet connecting to the most superior point on tuberosity of calcaneus # Normal Bobler’s (Tuber’s) angle s 24° - 40° Normal angle of Gissane is approx 100°C © Loss of this angle® (Reduction / Reversal) + An Increase in the angle® of Gissane ¢indicates Indicates intra articular fracture of calcaneum® intraarticular fracture of calcaneum® (by axial compressive force)516 + 254, 256. 257. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 Answer is A (Flexion): Maheshwari 5"/270 Flexion injury is the commonest spinal injury — Maheshwari Answer is A (Compression Violence): Maheshwari 5"/272; Rockwood & Green 5"/1413 Burst Fractures involve compressive failure of vertebral body. Burst Fracture: “Anterior column: Consists of Burst fractures involve compressive failure of the x the anterior longitudinal vertebral body. ligament, anterior part of the + There is failure ofboth the anterior column and middle vertebral body and the anterior cohamn' portion ofthe annulus fibrosis. © Posterior column remains intact? with a burst fracture + Axial compression or vertical compression force is the most important responsible force? t may be seen with ‘Axial compression alone Axial compression + Flexion ‘Axial compression + Rotation Posterior Column: Consist of Axial compression + Lateral flexion oy the bony and ligamentous ‘+ Afall from height landing on one's feet is a typical ma sposterior elements 3 “Middle column: Consists of the posterior longitudinal ligament, posterior part of the vertebral body, and posterior portion of the annulus. ‘mechanism of this fracture® Answer is A (Osteoclastoma): Maheshwari 5"/237;Campbell’s orthopaedics 1"/793-854; Apley's; Chapman's Radiology Osteoclastoma is a tumour located at the epiphysis - Maheshwari 3” /216 Osteosarcoma and chondromyxoid fibroma are metaphyseal tumours while Ewing’s sarcoma is diaphyseal Common Sites For Common Tumours ee | Epiphysis ‘Metaphysis Diaphysis ‘© Chondroblastoma ? © Osteochondroma® ‘+ Ewing's sarcoma ® + Osteoclastoma (Giant cell) '* Osteoblastoma® * Multiple myeloma? ‘© Simple bone cyst® '* Eosinophilic granuloma? '» Aneurysmal bone cyst 2 (Langerhans Cell Histiocytosis) ® + Osteosarcoma ® * Osteoid Osteoma® ‘* Chondromyxoid fibroma® ‘* Adamantinoma ® + Fibrous cartical defect ° + Enchondroma (Campbell's) + Non ossifving fibroma ® * Enchondroma (Apley's) © ous Cortical Defeet and Nonossi The commonest sites of Fibroma, Fibrous cortical defect and Non ossifving fibromas are metaphysis of long ‘bones, but these may be seen in the diaphysis and hence are best termed as metaphysio diaphyseal tumours. + Enchondroma: The commonest site for enchondroma has been mmentioned as Diaphysis’ in Campbell's Orthopaedics while Apley’s textbook mentions ‘metaphysis’ as the commonest location. (Metaphysio diaphyseal) ‘© Chondromyxoid Fibroma, Answer is A (Osteosarcoma): Apley's 8/185; Maheshwari 5"/239; Green span orthopaedic Radiology Codman’s triangle and Sunray appearance are characteristic radiological findings in osteosarcoma,258, ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS = 2007 ° 517 Differential Diagn tions provided in question ‘Features {Chowdrabloastomia ‘Age 10-25 years® 5~25 years® Location Metaphysis ® Epiphysis® ‘Commonest site Distal Femur? Distal femur? Femur (diaphysis) Numerus / Tibia / Femur Others : Other Others Others Proximal Tibia® Proximal tibia? Tibia, Fibula Smill bones of hands and guy Proximal humerus Flat bones (iliumete) feet (tolus / calcaneum) Distal radius? Radiological Codman’s ee ‘Soap bubble® Onion peel Sclerotic border with o ‘Characteristics Sunray appearance® Appearance” Appearance” Scattered calcification® 6 (Sunburst appearance) | & Answer is B (Bone marrow Biopsy): Apleys 8/185; Compbell's 10" A biopsy from the lesion is essential for grading of the tumour but a biopsy from bone marrow is not indicated Bone scan, CT chest and MRI femur are important staging investigations for osteosarcoma. Lesion rssh of keeoma “ray from lesion ‘Codman’s triangle® ‘Sunray appearance / Hair on end appearance? (New bone formation & Periosteal Reaction) ‘In most cases the diagnosis can be made with confidence on X— Ray Appearance ~ Apley _ a — | — eee Grading of Tumour == Bone Marrow Biopsy eta 9 oe Be ees, MRIs the best testto Bone sean should Be ‘Should be done to search for measure the extent of obtained to look for Pulmonary metastasis (Lang are lesion both within the skeletal metastasis ‘the most comon sites of bone and inthe soft issue metastasis”) ‘These tests should be done before Biopsy from the Lesion Answer is A (Osteosarcoma ): Annals of Surgery : Pulsating Tumors Of Bone ; Pulsating, Benign Giant Cell Tumors Of Bone ; Pulsating Tumors Of The Sternum; Other references None of the standard textbooks mention 'Pulsating tumors of Bone’, but a review of literature outlines Telengiectatic osteosarcoma as a pulsatile tumor of bone Telengiectatic osteosarcoma is an aggressive lytic form of osteosarcoma that occurs as a soft cystic tumor containing aneurysmally dilated blood filled spaces. Telengiectatic osteosarcoma may present as a pulsatile tumor ofbone. Chondrosarcoma, Ewing's sarcoma and Eosinophilic Granuloma have not been mentioned as pulsating tumors in literature Osteosarcoma is the single best answer of choice amongst the options provided ‘Tumors that may present as pulsating lesions (Review from Literature Primary Tumors Secondaries /Metastatic Tumors + Telengiectatic Osteogenic Sarcoma “Meatastasis from Renal Cell carcinoma + Angioendothelioma / Angiosarcoma of bone ‘Meatastasis from Thyroid carcinoma # Aneurysmal bone cyst * Giant Cell Tumor (Rarely)‘518 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 260. 261. “Angioendothelioma of bone is a rare slow growing pulsating tumor of bone. Other pulsatile osteolytic ‘tumours may occur in bone, such as Telangiectatic “Hypernephroma (Renal Cell Carcinoma) and Carcinoma of Thyroid are unique in their tendency to produce slow growing, pulsating and often single Osteogenic Sarcoma and Giant-Cell Tumour. Telangiectatic Osteogenic Sarcoma usually affects children and is characterised by exceedingly rapid growth and early pulmonary metastases. Giant-cell tumour of bone, when vascular and pulsatile, may cause great difficulty in differential diagnosis, its -growth being slow like that of angio-endothelioma ~ ‘Pulsating Angio-Endothelioma Of The Innominate Bone’; Goruion Gordon-Taylor And Philip \Vile5, London, England ‘metastasis’ - Pulsating Tumors Of The Sternum ; Annals Of Surgery Answer is A (Alkaline phosphatase): Harrisons 16/2273 Alkaline phosphatase is an important marker for new bone formation. Hydroxyproline, Acid phosphatase and Telopeptides are markers of bone resorption. “Markers of Bone Formation ‘+ Serum Bone specific Alkaline phosphatase * Serum Osteocalcin® + Serum propeptide or type I Procollagen | Markers of Bone Resorption i * Urine and serum N - telopeptide + Urine and serum C- telopeptide + Urine total free deoxypyriinoline + Urine Hydroxyproline + Scrum tartarate resistant Acid phosphatase + Serum Bone Sialoprotein Urine hydroxylysine glycosides Answer is B (serum Alkaline Phosphatase) : Wheeless’ Textbook of Orthopaedics; Garland D.E. A clinical perspective on common forms of acquired heterotopic ossification CORR No Feb, 1991; Other Journal articles Heterotopic ossification is characterised by periarticular deposition of ectopic new bone. Serum alkaline ‘phosphatase is an important marker of osteoblastic activity and new bone formation and is the single best investigation of choice amongst the options provided. “Although non- specific, serum alkaline phosphatase may be the earliest, least expensive test for early detection of Heterotopic Ossification. If fractures are not present this is an excellent presumptive test’- Garland D.E.A clinical perspective on common forms of acquired heterotopic ossification CORR No 263 Feb, 1991 ‘Serum Calcium and Serum Alkaline phosphatase Heterotopic Ossification is the formation of mature lamellar bone in periarticular soft tissue and often occur in foci of Dystrophic calcification. Laboratory findings (including Serum calcium and Serum Alkaline Phosphatase) are non specific and may not aid in diagnosis. However amongst the available laboratory investigations, Serum alkaline Phosphatase isthe single most important investigation as a marker for osteoblastic bone formation in foci of heterotopic ossification. Serum calcium levels forms an important investigation in cases of metastatic caleification but notin dystrophic calcification ‘Most cases (95 10 98 percent) of soft tissue calcification represent dystrophic calcification and only few cases (1 10 2 percent) represent metastatic calcification. And hence serum calcium levels are not the investigation of choice for soft tissue caleification. Serum Acid Phosphatase and Serum Phosphi These investigations have not been mentioned as first line investigations for either heterotopic ossification nor for ‘metastatic or dystrophic calcification.262. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 © 519 Answer is A (Quadriceps only): Turek's orthopaedics 2™/1368 ‘Muscle atrophy particularly involves the quadriceps muscle - Turek All periarticular muscles around the knee joint may undergo wasting in advanced osteorthritis knee. However muscle atrophy particularly involves the quadriceps which is the earliest muscle to undergo wasting. In patients with osteoarthritis knee, quadriceps strengthening exercises form an important management component to protect the articular cartilage from stress. OPHTHALMOLOGY 263. 264. Answer is B (Vitreous Humor): Harper 27" / 553, 554 Hyaluronic acid is present in bacteria and is widely distributed among various animals and tissues including synovial fluid, the vitreous humor, cartilage and loose connective tissues ~ Harper Location of lycosaminoglycans: ‘[Glycosaminoglyean em Hyaluronic acid Synovial fluid Vitreous humor Cartilage Loose connective tissue Cartilage Bone Cornea Corea (KS 1) Loose connective tissue (KS I) Mast cells Aortic wall Skin fibroblasts Chondroitin Sulphate | Keratan sulphate (KS) Heparin Heparan sulphate Answer is (Querry); Adler's physiology of the eye ; Khurana ‘s physiology of the eye ; ‘Transport of vitamin C in the lens’, Curr Eye Res. 1987 Jul:6(7):885-96 ;. ‘Ascorbic acid and the Eye’, Am J Clin Nutr 1991;54:1198S-12025, The precise mechanism for transport of Ascorbic Acid into the lens has not been determined and is still under extensive research. . The transport of Ascorbic acid into the lens is believed to be linked to the transport of Glucose ,and the most accepted mechanism for Ascorbic acid transport into the lens is thought to be facilitated diffusion of the oxidized form of Ascorbic acid (Dehydroascorbate) by way of the glucose transporter ‘GLUT’. This however has not been provided in the options and hence we do not have any single best answer of choice . (Note : the options provided are memory based and might not be accurate or correct) Most Accepted Mechanism for transport of Ascorbic acid into the lens ‘Transport of Ascorbic Acid / Dehydroascorbate into the lens is linked to Glucose Transporter (GLUT) (Facilitated diffusion) Dehydroascorbate , the oxidized form of form of Ascorbic acid can enter the lens by way of the glucose transporter where itcan be reduced to ascorbic acid by glutathione dependent process. The Prevailing evidence is thatthe lens does not contain a sodium dependent ascorbate transporter.- Adler's Physiology of the eye 10/131 Dehydroascorbate enters by carrier-mediated, facilitated diffusion;, Transport is not energy or sodium dependent but the uptake is inhibited by cytochalasin B, a compound known to inhibit uptake of D-glucose. This and results of other inhibitor studies indicate that transporters of dehydroascorbic acid are related to glucose transporters (GLUT )- ‘Ascorbic acid and the Eye", Am J Clin Nutr 1991;54:11 98S-1202S. ‘The lenticular transporter for DAS (Dehydroascorbate) evidently is interrelated with that for hexases, as determined by kinetic studies and inhibition by analogues: Transport of vitamin C in the lens ;Curr Eye Res.1987 Jul;6(7):885-96‘520 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 268. Sodium Dependent Transport of Ascorbic acid into the lens : New Evidence under research Could Na+ K ATPase be the correct option ? ' f the Eye clearh transport of Ascorbic acid into the lens is N at Dependent mechanism: The prevailing evidence is that the lens does not contain a sodium dependent ascorbate transporter. Adler's physiology of the Eye 10"/131 BUT (Confusion) ium Dependent Ts Sf mM trated i s Recently, two specific transporters for vitamin C (Sodium Dependent Vitamin C Transporters (SVCT) have been cloned in the rat and the human and a very recent study suggest that Na+-dependent transport of AA in normal lens epithelium may be mediated by SVCT. Vitamin C transport in human lens epithelial cells: evidence for the presence of SVCT2. Exp Eve Res. 2001 Aug;73(2):159-65. Kannan R, Stolz A, JiQ, Prasad PD, Ganapathy V ic acid into the lens is a very controversial topic and still under extensive research, None of the ive sufficient information to answer this question correctly, Thus If Facilitated diffusion of Dehydroascorbate with Glucose Transporters GLUT is provided amongst the options it ‘should be picked up as the single best answer of choice. Ifthe question does not provide that optic icking Sodit snt ATPase as the ‘answer, based upon the recent publication that gives evidence of the presen F sndent Vitamin ‘Transporter (SVCT) in normal lens epithelium. Answer is A (Vit A): Adler's Physiology 10"/130; Parson 20/248; Harper 27/119, 612 Glutathione, Catalase, Superoxide dismutase and Ascorbic acid (vitamin C) have been specially mentioned as true radical scavengers in relation to lens. Vitamin E (tocopherol) and B carotene are present in low levels in aqueous humor and may play a role in antioxidant mechanism in the lens. Provitamin A or B carotene is an antioxidant and not Vit A or Retnoic acid. Vitamin-A is the single best answer of exclusion, amongst the options provided. Protection against oxidative Damage: (Adler's Physiology, Yanoff) © Glutathione peroxidase is found in significant amounts in both epithelial cells and fibres of the lens Glutathione provides most of the protection against oxidative damage in the lens © Catalase is present in abundant levels in the epithelial cells but is found in very low levels in the fibres Catalase is affective against high levels of peroxide + — Superoxide dismutase is present in lens and acts by dismutation of superoxide anion * Ascorbic Acid (Vitamin C) plays the major role in the antioxidant system in the lens + Vit (Alpha tocopherol) and Provitamin A (B carotene) are present in low levels in the aqueous humor. They may have some antioxidant role in the lens but itis less important than that of Acorbic acid. Enzymes Vitamins ‘* Glutathione ‘+ Vitamin C (Ascorbic Acid plays the major role) Peroxidase © Catalase ' ¢ Vitamin E (Low levels of antioxidant @ tocopherol (Vit C) + Superoxide dismutase | | 6 fscarotene | and carotene (Provitanin A) ae present in ‘2oqueous humor and these may play some role as antioxidants in the lens.266. 267. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 * 521 Answer is B (Disruption of Neurofilament) : Parsons 20" /330; Khurana 4/298 Disruption of Neurofilament has not been mentioned in the pathogenesis of papillaedema. Axoplasmic stasis, Axonal swelling and Extracellular edema have been specifically mentoined in the pathogenesis. Disruption of Neurofilament is therefore the single best answer of exclusion, Pathogenesis of Papilloedema + Papilloedema develops as a result of stasis of axoplasm in the prelaminar region of optic disc due to alteration in the pressure gradient across the lamina cribrosa ~ Khurana ‘©The axonal swelling in prelaminar region is the inital structural alteration which in turn produces venous congestion and ultimately extracellular oedema ~ Khurana + Note that in the final atrophic stage of papillaedema nerve fibres undergo degeneration and there is atrophy of neurons and associated gliosis. tissue pressure gradient across laminar region from various causes Stasis of Axoplasmic transport is the principal Pathophysiology of optic dise swelling and papilloedema = Yanoff Papilloedema Answer is C (Krabbe’s disease): Various References : Repeat Krabbe’s disease is a rare autosomal recessive neuro degenerative disorder. It is not associated with a cherry red + Gangliosidosis (Generalized Gangliosidosis, GMI) * Tay sach’s disease® ‘+ Niemen Pick disease® ‘+ Central Retinal Arterial Occlusion (CRAO)® '* Commontio Retinal (Blunt Trauma to eye)? © Quinine Amblyopia © Metachromatic Leukodystrophy® + Multiple sulfatase deficiency Gaucher's disease Cherry Red spot is not seen in central Retinal Venous Occlusion (CRVO). Note that certain books give on exhaustive list of differential diagnosis of cherry red spot. This list includes only the most ofien repeatedly asked options.. Ans is A (Morning Glory Syndrome) : Morning Glory syndrome — A Histopathological study British Journal of ‘ophthalmology; The Morning Glory syndrome" ophthaimologica 1983; 187 (7) : 222-230 1990, 74; 56- 58 Lamina Cribrosa is absent in Morning Glory syndrome * Lamina Cribrosa isa sieve like perforation in the posterior part of the sclera that allows passage of the retinal ganglion cell axons and central retinal vessels, #_Itis the weakest part of the sclera522» AIPGMEEXAMINATION ANSWERS AND EXPLANATIONS - 2007 269. Morning Glory syndrome is defined as a malformation of the optic disc associated with: + A Peripapillary scleral defect ‘+ Absence of Lamina Cribrosa +_ARecess formed by an Axial Retro displacement of the optic nerve ‘+ Itis called ‘Moming Glory Syndrome’ because the papillary region in the affected eye is (usually unilateral) reminiscent of the flower of * morning glory’ «Its believed to be a mesodermal defect. Answer is B (Best Disease) : Kanski 6"/672, 667, 693; Harrisons 16/2190 Best disease has an autosomal dominant inheritance with variable penetrance ~ Kanski 6"/627 Best disease (Autosomal Dominant) (Kanski 6" / 672) Juvenile Best Macular (Vitelliform) dystrophy isa type of Retinal dystrophy with Autosomal Dominant Inheritance with variable Penetrane R and expressively The gene locus is on 11q 13 Bassen — Kornzweig Syndrome (Autosomal Recessive) (Kanski 6" / 667) Bassen— Kornzweig syndrome is a systemic association of Retinitis Pigmentosa. It is caused by deficiency of betalipoprotein resulting in intestinal malabsorption Ithas an Autosomal Recessive inheritance Gyrate Atrophy (Autosomal Recessive) (Kanski 6" / 692) Gyrate Atrophy is a type of choroidal dystrophy with Autosomal recessive inheritance Lawrence - Moon — Biedl Syndrome (Autosomal Recessive) (Harrisons 16" /2190) (Kanski 6"Y/667) ‘Lawrence Moon Bied! syndrome is a systemic association of Retinitis pigmentosa. Ithas an autosomal Recessive inheritance and is characterized by Obesity, Hypogonadism, Mental retardation, Polydactyly and Retinitis pigmentosa. Disorders that may present with Retinal or Choroidal Dystrophies (Variabe Inheritance “Macular Dystrophiles ‘Retinal Macular Dystrophies ‘Retinal Macular Dystrophies * Stargart disease Juvenile Best disease * Retinitis Pigmentosa (Guvenile Macular Dystrophy)® (tlie dystrophy) (AD/ARXL) « Fundusflavimaculatus + Sorsby pseudoinflammatory macular _* Progressive‘Cone Dystrophy + Leber Congenital Amaurosis dystrophy (AR or XL) « Bieti Crysallene Dystrophy North Carolina macualr dystrophy * Alport syndrome (XL D) + Conditions associated with Retiniis _» Patter dystrophy tinal (Butterfly macular dystrophy °) ~ Bassen Karncweig Syndrome? + Benign concentric annular macular - Refsum's Syndrome dystrophy - Kearns Sayre Syndrome «+ Adult vtelliform foveomacular = Bardet-Biedl Syndrome (Laurence dystrophy ‘Moon Bied! syndrome®) «+ Fanlialdrusen - Ushers syndrome + Dominant Cystoid Macular Edema - Freireichs ataxia Ghoroidal Dystrophies Choroidal Dystrophies ‘Choroidal Dystrophies ~ Gyrate Atrophy Central Areolar Choroidal dystrophy Choroideremia (XLR) + HelicoidPeripapillarychorioretinal _—_ Pigmented Paravenous dystrophy Retinochoroidal Atrophy + Progressive bifocal chorioretinal atrophy[AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 * 523 270. Answer is A (Orbital varix): Yanoff and Ducker ophthalmology 2™/ 731 ~ 740; Kanski 6"/180 -188 The patient in question is presenting with characteristic signs of orbital varix. Proptosis from orbital varix is unilateral (most cases) non pulsalite and has no thrill or bruit. It is compressible and is characteristically precipitated by increasing venous pressure as in assuming a dependent position. (bending forwards). MRI may show echogenic shadows from phebolittis (Venous stasis leads to formation of pheboliths). ptosis with Orbital Varices is consistent with all presenting features in the question ; : — (Feature ‘Orbital Varices Encephalocele (Ciroticocavevnous Fistula t z (Direct) Pathology / Result from weakened orbital venous ‘Results from herniation Results from an AV fistula Pathogenesis system that enlarges with increased venous of intracranial content between carotid and cavernous 2 pressure through a congenital sinus usually after head injury S defect at base of skull 5 ‘Age of presentation Early Childhood to late Middle Age During Infancy ‘Traumatic: Any age (days or 2 ‘weeks after head injury) s ‘Spontaneous: Adults / Elderly & Unilateral ‘Bilateral Unilateral (most cases) Usually unilateral (ipsilateral) but 3 may be bilateral or contralateral" Palsatile/ ‘Non pulsatile May be pulsatile Pulsatile 5 Compressible Compresible Reducible but not = g compressible iy Brol & Thrill ‘No Brui/No Thrill ‘No Brui/ No trill Brul & Til present g Precipitating factor As the orbital veins are devoid of valves Cyst increase in size on ~ 2 rapidly reversible proptsis may be Siraining orerying = precipitated by increasing venous Pressure coughing, staining, valsava ‘manouvte, assuming Dependent Position external compression of Jugular vein — MRI/CT ‘Show Phleboliths Show Bony defects Prominence of superior Echogenic shadows) responsible for ophthalmic vein and diffuse herniation enlargement of extra ocular muscles Proptosis with neurofibromatosis is not compressible and is not precipitated on increasing venous pressure. Proptosis in Encephalocele is reducible but not compressible and Echogenic shadows are not a characteristic feature. + Proptosis in Caroticocavernous Fistula is pulsatile, associated with Brui and Thrill and is not precipitated or ‘aggrevated by increasing venous pressure 271. Answer is B (Palatal paralysis): Dhingra 6/315 Palatal paralysis is a cause of Rhinolalia Aperta (Hypernasality) and not Rhinolilia Clausa (Hyponasality) -Dhingra Rhinolilia Clausa (Hyponasality) Rhinolilia Aperta (Hypernasality) This refers to the lack of nasal resonance for words This is seen when certain words which have little nasal which are resonated in the nasal cavity eg m, n, ng. resonance are resonated through the nose. It is due to blockage of the nose or nasopharynx It is due to failure of nose to cut off from oropharynx or due to abnormal communication between the oral and nasal cavities Causes [Causes + Common cold '+ Velopharyngeal insufficiency + Nasal allergy (Allergic Rhinits) +» Congenitally short soft palate + Nasal polyps + Submucous palate + Nasal growth + Large nasopharynx + Adenoids * Cleft of soft palate ‘+ Nasopharyngeal mass « Paralysis of soft palate + Familial speech pattern + Post-adenoidectomy + Habitual + Oronasal fistula +» Familial speech pattern ‘+ Habitual speech pattern524 2n. 274. 275. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 Answer is A (Hypertrophy of the sebacious glands): Dhingra 6"/144 Rhinophyma or potato tumour isa slow growing benign tumour due to hypertrophy of the sebaceous glands of the tip of nose — Dhingra Rhinophy: ‘+ This is a slow growing, benign tumour® due to hypertrophy of sebacious glands® of tip of nose Often seen in cases of long standing Acne Rosacea®, «© Mostly affects Men post middle age Potato tumour Presentation ‘© Unsightly Appearance : Pink lobulated mass over nose® with superficial vascular dilatation ‘Obstruction in breathing and vision (Due to large size of the tumour) ‘Treatment + Debulking / Excision of Tumour with skin grafting, Answer is C (Cribriform Plate ) : Review article Journal Of Craniofacial Surgery; Repeat AI/1995 The cribriform plate and air cells of the ethmoid sinus account for the maximum number of leaks ‘The most common anatomic sites of CSF leaks are the areas of congenital weakness of the anterior cranial fossa. 40 4% of leaks occur in the region of the cribriform plate and air cells ofthe ethmoid sinus as described in most series. The frontal sinus and the area of the sella tursica and sphenoid sinus account for about 15 percent of leaks each respectively. In a rare situation, the leak can originate in the middle or posterior cranial fossa and can reach the nasal cavity by way of the middle ear and eustachian tube.’-Review article: Journal Of Craniofacial Surgery Answer is B (Beta 2 transferrin): Dhingra 6°/164 CSF contains glucose which can be demonstrated by oxidase peroxidase paper strip or biochemical tests. Beta 2 transferrin is specific for CSF — Dhingra 4°/155 History ‘asl or sinus surgery, head injury or Sneezing, nasal suffinss itching in the nose or intracranial tumour Jacrimation Flow of discharge ‘A few drops ora steam of fluid gushes down Continuous, No effet of bending forward or when bending forward o staining: straining, Can be sniffed back Cannot be sniffed back Character of discharge? « When collected in a test tube and + When collected ina test tube and allowed allowed to stand it remains clear? to stand it leaves a sediment because of mucus & other proteins '* Does not stiffen a handkerchief? * Stiffens a handkerchief? Taste Sweet salty Sugar content? More than 30 mia Less than 10 mg (compare with sugar in CSF after lumbar puncture as sugar i es in CSF in meningitis) | Presence of transferrin Always preset 11s specific for CSF Always absent Answer is A (Cranial Nerve 5): Dhingra 6"/112 Vth cranial nerve is the earliest nerve to be involved — Dhingra 4/11]276. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 * 525 PRESENTATION OF ACOUSTIC NEUROMA. (Age group of 4060 years) Cochleo-vestibular symptoms due 0 (Cranial nerve involvement [> Brainstem involvement pressure on Cochlear/Vestibular fibres J+ Cerebellar involvement 1 V® + aries nerve involved? IS Raised ICT Cochlear Vestibular + Reduced comeal Se «s Imbelance sensitivity & Numbness of face VII" Hypoesthesia of inter + Unni eae neural hearing 1085° | Yerigo ter + Tinnitus pea + Difficulty in IX &X Dysphagia / Hoarseness understanding Pxith, Xifth, Hrd, IVth, Vith nerves may also speech [be involved when tumor i very large Remember: ‘© Most common tumor of the cerebello-pontine angle is : Acoustic neuroma. * The commonest nerve of origin of Acoustic Neuroma is the Superior Vestibule Nerve, followed by the inferior vestibular and rarely the cachlear™. - Logan Turner /339 (Old Edition) Commonest cause of unilateral deafness with tinnitus is : Acoustic neuroma. ® ‘Most common presenting symptom of Acoustic neuroma is : progressive U/L sensorineural deafness with tinnitus, ® Accu nearoma tay preset with edu eorel seni 8 wel spi, tingling, mmbnest co fee: dew involvement of 5" cranial nerve. '* Acoustic Neuroma is a benign, encapsulated, extremely slowly growing tumor of the 8" nerve (commonly vestibular division, rarely cochlear division). Answer is A (Glomus Tumor): Dhingra 6"/109 Brown sign or pulsation sign is a classical sign seen in Glomus tumor ‘Glomus Tumor in a = ‘extremely = ‘tumor thet originates from Glomus = ‘© Glomus tumour is the most common benign tumour of the middle ear® © Glomus bodies resemble carotid bodies and may be found in two places and accordingly Glomus tumors may be differentiated into two types *-Gioimas Tympanien ‘They arise from glomus bodies found on the dome of Jugular bulb ‘promontary along the course of tympanic branch of DE CN Wacobson's nerve)? “Tumour intially fills ness and (Earliest symptoms) the Middle ear Deafness : Conductive Tinnitus : Pulsalile®, Swishing, Synchronus with the pulse? & can be temporarily stopped by carotid pressure ‘May present with “Tumour later perforates Red reflex: Rising sun Bleeding ‘through tympanic *# Otoscopy shows a Red Reflex? through intact tympanic + Dizziness “+ membrane to present as ‘membrane © Vertigo = ‘© Rising sun appearance® is seen when tumor arises from floor * Otorthea of middle ear ‘+ Facial Nerve paralysis # 1X" to Xt cranial [Tumour may invade (Special test: Pulsation sign : Brown sign’ nerves may be Labrynth, Petrous ‘When ear canal pressure is raised with Siege’s speculum, tumor paralyzed + Pyramid, Mastoid, pulstes vigrously and then blanches. Reverse happens with Jugular foramen and. release of pressure base of skull bruit tall stages systolic mastoid may be present ‘Some glomus tumors secrete catecholamines (10%) and may produce symptoms Tike headache, sweating, palpitation, hypertension and anxiety ~ Dhingra‘526 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 277. ‘SKIN 278. 279. Answer is B (Common with transverse fracture): Dhingra 6°/97 Longitudinal fractures of Temporal bone are more common than transverse fracture but, Facial nerve injury is ‘most commonly associated with Transverse fracture of the temporal bone - Dhingra Difference in longitudinal and transverse fractures of temporal bone: Ve beputtiecoan tones Frequency More common (80%)® ‘Less common (20%) ‘Type of injury Parctal blow Occipital blow FFractureline Runs parallel to long axis of petrous pyramid. Runs across the petrous Starts of foramen magnum or Start at squamous part of temporal bone to end jugular foramen towards the foramen spinosum, at foramen lacerum Bleeding from ear Common, due to injury to tegmen and tymopanic Absent because tympanic membrane is intact. membrane aemotympanum may be seen. ‘CSF. otorrhoea Present, often mixed with blood Absent or unmanifested ‘Structures injured Tegmen, ossicles and tympanic membrane Labyrinth or CN VI Hearing loss Conductive? Sensorineural® Vertigo Less often; due to concussions Severe, due to injury to labyrinth oF CN VINE ‘Facial paralysis® — Less common (20%), Delayed onset. Most common (50%). Immediate onset. Nerves is injured in tympanic segment, distal to _Injury to nerve in meatal or labyrinthine segment geniculate ganglion proximal to geniculate ganglion. Answer is A (Self resolving) Ref: Harrison 16/292; Roxburgh's-Common Skin disease 17"/17; Fitzpatrick's dermatology 5/369; Illustrated textbook of dermatology: Pasricha 3/134; Ilustrated Synopsis of dermatology & STDs-Neena Khanna I"/42-44 Pityriasis rosea is an acute exanthematous papulosquamous eruption often with a characteristic self limiting course®. The etiology is not known (It is not caused by dermatophytes). © The etiology of Pityriasis Rosea is unknown ‘ei (Herpes virus isthe suspected etiologic agent) (itis NOT caused by Dermatophytes) eon Most commonly affects young adults (F>M) ‘+ Predominant involvement is limited to the Trunk® * The first feature isa single annular plague usually on the trunk (Herald Patch). ‘Christmas (Fir) Tree pattern * Principal lesions are oval or circular with Long axis of oval lesion following the lines of cleavage in a Christmas tree pattern (Fir tree pattern)” 2 Lesions may have fine scaling (cigarette paper scales) ® ‘Answer is B (Atopic Dermatitis) Hearvison 16*/268: Roxburgh's-Common Skin disease 17%/105-112; Ilstrated textbook of dermatology: Pasricha 3/152; Fitzpatrick’s Dermatology in General Medicine 6¥/1 181-1203; Rooks Textbook of Dermatology 7*/18.1-18.30] History of atophy (asthma) in family, with erythematous lesions on face (cheek) and extensor surface of limbs is suggestive of atopic dermatitis.[AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~ 2007 * 527 AD is a cutaneous expression of the atopic state, characterized by a family history of asthma, hay fever or dermatitis® in ~ 70% of patients. «so, Eiio= Pathology ‘Glnical Presentation ~ Biology is only partly defined but ~ The clinical presentation often varie with ags. HalFof he pacts present ther is a clear genetic predisposition with inthe fist year of ite, & 80% present by 5 years of age When both parents are affected, over + The infantile patern is characterized by weeping inflanmatory patches ‘80% and when only one parent is and crusted plaques that occur on face, neck and extensor surfaces®. affected ~50% oftheir children manifest (Infantile eczema) the disease ~ The childhood und adolescent pattern is marked by dematits of xual yp = Increased IgE synthesis; increased serum skin particularly in antecubital & popliteal fossa® 2 + IgE; increased specific IgE wo food, = Clinical course lasting longer than 6 weeks? 2 acroalergins, actra & bacterial ~ About 80% ulimately co express alergie rhinitis or asthma. & products, + AD may resolve spontaneously, but over half of affected children will have z ~ Increased expression of CD23 (low dermatitis in adult life, i.e. cowre is marked by exacerbations and affinity IgE receptor) on monocyte & B- remissions®, However, adults frequently have localized disease, & call Ianifesting as hand eczema ot lichen simple chronic. = Impaired delayed type hypersensitivity + Clinical features can be remembered in an easy way by under standing the reactions. cause. 5 Clinical Features: a iu — 4 gE a aa T Personal or Family History 3 = of atophy < = ‘© Severely itchy, erythematous papular 7 Pallor on skin = Asthma® ‘0r papulo-vesicular lesions® mostly especially cheeks (ie. = Hay fever involve face popliteal fossa, perioral pallor) = Rhinitis allerie) a antecubital fossae, wri? Ucivessis |_| ~ White + Chronic urea made worse by dermatographism® - Food allergi ~ Minor environmental alteration (running a blunt object + Eczema = Sundry (in rainy seasons)? as key on affected skin + Changes in temperature® produces a white line) “Associated with = Rough clothings (woolen) L + Perpetual rubbing & seatching Vt ST ~ Excoriation® (simple, linear scratch ~ Susceptibility to skin infections® particularly mata) cont Treatment to stapylococcus aureus, warts & moluscus + Lichenfiaton® (exaggeration of skin ' peepee markings) i = Antena sign - Follicular opening fill = Drmess/ Xero dena eae Tome pgs nn eet + Hope plans romins in 3. Tar prepanons ~ Headlight sign ~ chilis i iolammation markings on plam) +4, Systemic agents ~ of skin on & around lips (perioral pallor) ~ Denny Morgan fld® exraoldofskin || Seed eyeloporn, |_| ~ Hertoghe's sign ~ thinning of acral half of benoaioesagiet PUVA, anthistamiic eyebroms Answer is B (Lichen Scrofulosorum): Beh! 10/202; Dorland's 28/1756 Tuberculides are considered to comprise Erythema Induratum, Lichen Scrofulosorum, Papulonecrotic tuberculides, Lupus Miliaris Disseminatus Faciei and Rosaceous Tuberculides. Tuberculides include a group of skin reactions that exhibit tuberculoid features histologically. ‘There origin is still debated and different theories of pathogenesis exist: ‘© They are believed to occur due to Hypersensitivity reaction to Mycobacterial Antigens and hence the name "Tuberculides. ~ Some believe tuberculides occur due toLocal Hypersensitivity reaction to mycobacteria. = Some believe tuberculides occur due to Hypersensitivity reaction to mycobacterial antigens that have reached the skin by Haematogenous spread from foci of active infection. * Others believe that these lesions are unrelated to Tuberculosis because : ~ AFB have not been isolated, cultured or produced in animal inoculation = These conditions often do not respond to Antituberculous drugs but may respond to steroids.528 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007, Conditions included in Tuberculides ‘Lichen Scrofulosorum + Erythema Induratum ‘+ Papulonecrotic Tuberculides (Acne Scrofulorosum) ‘© Lupus Miliaris disseminatus Faciei + Rosaceous Tuberculide Cutancous Tuberculosis (Non Tuberculides) + Lupus Vulgaris * Sacroftloderma + Tubercularis Cutis Verrucosus + Tuberculosis Cutis Orificialis * Miliary tuberculosis and Metastatic Tuberculosis Abscess may also present as Cutaneous tuberculosis (According to 'Behl' Erythema Nodosum is also a Tuberculide while some texts do not include it as a Tuberculide). ANAESTHESIA 281, Answer is B (Procaine): Lee's 12"/592 Procaine is an ester linked local anaesthetic. Lignocaine, Bupivacaine and Dibucaine are amide linked + Lidocaine/Lignoeaine xylocaine) + Bupivacaine © Procaine * Dibueaine * Chlorprocaine * Prilocaine © Tetracaine Ropivacaine ‘+ Benzocaine 282. Answer is C (Suecinyl Choline): Lee Synopsis 13/190; Miller 6491 ‘Muscle pain or Myalgia is a common adverse effect of succinyl choline Itis common in women and young to middle aged adults and in those who are ambulant shortly after surgery The young adult in question has recieved succinylcholine and is now ambulant after surgery. He is classically presenting with myalgia secondary to succinyl choline se ‘Myalgia (Muscle Pain after succinyl choline + The incidence of muscle pain after administration of succinyl choline varies from 0.2 % to 89% ‘+ Women young to middle aged adults + Afier minor surgery (day case) In those who are ambulatory shortly after surgery (rather than bedridden patients) “ «Pain is believed to be secondary to damage produced in muscle by unsynchronized contraction of adjacent muscle fibres just prior to the onset of paralysis. + Myalgia may be prevented (or attenuated) by a small dose of non depolarizing neuro muscular block few minutes before suecinylcholine administration 283. Answer is C (Acumulation of Laudanosine) (most probably). Miller 6" / 509, 831 Laudanosine is a metabolite of Atracurium and has CNS stimulating properties. Laudonosine may produce convulsions from its CNS stimulating action, when high plasma concentration of Laudanosine are reached. In clinical practice, in the operating room and ICU setting, such high concentrations are usually not reached, but the patient in question has been on atracurium for 3 consequestive days before he develops epilepsy and hence high plasma concentration of Laudanosine could well be a probable cause for epilepsy.284. 285. ‘Confusion : ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS ~2007 ¢ 529 Although most text books clearly mention Laudanosine to be Epileptogenic (in animals), Epileptogenesis in humans with Laudanosine has been mentoined to be highly unlikely a clinical doses of atracurium. Neverthless accumulation of Laudanosine to high levels may cause epilepsy and hence this has been pi single best answer of choice. In The Intensive ed up as the Canadian Journal Of Anaesthesia : Review article by Michael D. Sharpe MD FRCPC ‘© Laudanosine is a metabolite of Atracurium. ‘+ Hofmann elimination of each molecule of Atracurium releases two molecules of laudanosine. ‘© Laudanosine is a central system stimulant and has caused seizures in dogs following large intravenous boluses The seizure threshold level in humans is not known but case reports suggest that clinical doses are unlikely to result in important central effects in humans. ‘+ Nervetheless Laudanosine may promote seizure activity in susceptible patients with liver dysfunction due to its dependency upon hepatic clearance. The clearance and elimination half-life are prolonged in patients with liver failure, (Renal elimination accounts for only 4 9% while the remaining portion is dependent upon hepatic metabolism) Answer is A (Remifentanil): Goodman and Gillman 11" / $73 Remifentanit is not used intrathecally because glycine in the drug vehicle can cause temporary motor paralysis. It is generally given by continuous, intravenous infusion. — Goodman Gillman Opioids such as Morphine, Diamorphine, Pethiden (Meperidene), Fentanyl and Sufentanil may all be used intra thecally - Lee 13" /492 Answer is B (Patient on aspirin) Lee's Anesthesia 13%/477-84, 658, 661-62, 369, 17; Morgan's Anesthesia #*/299; Text book of Anesthesia Aitkenhead 4", P- 561; Miller's Anesthesia 6th/1654 Centrineuraxial anesthesia is not associated with increased risk with most antiplatelet agents (eg. aspirin® & NSAIDs). Contraindications of Centrineuraxial (Spinal Epidural) Anesthesia ‘Absolute Contraindications Relative contraindieations = Patients efisal = Patient's inability to maintain stillness during the needle puncture (cg. dementia, psychosis) = Raised intra cranial pressure® (papilledema, cerebral edema, tumors in posterior fos, suspected subarachnoid emorthage) - Severe hypovolemia = Severe stenotic valvular heart disease, the patient maybe unable to compensate for vasolton because ofa fined cardiac output Marke skin sepsis & marked spinal deformity = Marked coagulopathy, Blood dyscaressor fll anicougulant therapy? ‘Un coperative patient (may be performed in conjuction with GA) Preexisting neurological deficit (eg demyeinating lesions). All severe & marked diseases in lesser degree ie, spinal deformity, sepsis et. Pre eclamptictoxacmia ~ epidural block has been used with great benefit inthis condition, but a platelet count of less than 100 «10° L" usually preclude epidural or subarachnoid block Mildly impaired coagulation? Patients with platelet <80000 nt?530 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 286. 287. Contri ~ Neura © Oral anti coagulants (warfarin)? rust be stopped & normal PT & INR should be documented prior to block in = Long term therapy = Initial dose was given more than 24 hours prior to block = More than one dose was given * NBs best avoided ifa patient has recieved fibrinolytic or thrombolytic therapy '* NB should be avoided in patients on theraputic dose of standard heparin® & with increased PTT. If the patient i started on heparin after placement of epidural catheter, the catheter should be removed only after discontinuation of heparin & evaluation of coagulation status ‘+ Low molecular weight heparin (LMWH) catheter should be removed atleast 10 hr after a dose and subsequent dosing should not occur for lockade (NB) In setting of Anticoagulant & Antiplatelet Agents Most antiplatelet (asprin & NSAIDS) donot appear to increase the risk of spinal hematoma {from nenrasial anesthesia or epidural catheter removatl In contest, mor potent agents shouldbe Stopped & NB Should be administered only ster ther ffs have wor off. The waiting periods are Tielopidine = 14 days Clopidgrel = 7 days Abciximab = 48 hours Epifibatide = 8 hours Minidose subcutaneous prophylaxis with standard (unfractioned) heparin is nota contraindication. For patients who are to receive heparin intraoperatively, block may be performed 1 thor more before and eatheter removal should occur [Ur prior to, or 4 hr following subsequent heparin another 2 hout. dosing. ‘Neuraxial anesthesia best avoided ‘Neuraxial anesthesia can be given Answer is A (Propanolol / Metoprolol) ‘Morgan's Anesthesia 4/ 989-92; Textbook of Anaesthesia by Wiley & Davidson 7140- 41, 743 Non selective long acting f blockers are avoided for controlling arrythmias (or heart rate) during surgery®. Answer is B (Impedence pulmonometry) Lee’s Anesthesia 13/ 622- 23; Miller’s anesthesia 6/2394 - 95; Morgan’s Anesthesia 4/ 932 — 33; Text book of Anesthesia aitkenhead 4/371, 476-77; Textbook of Anaesthesia by Wiley & Davidson 7437-40, 975 The preterm baby in question is not intubated. Impedence pulmonometry isa recommended method for monitoring respiration in a non intubated patient, Capnography and Infrared end tidal CO, measurement are recommended methods for monitoring respiration in intubated patients. Monitoring Respiration 1 Intubated Patient 4 + Capnography® Non-Intubated Patient + + Impedence pulmonometry? © Infrared End Tidal CO; measurment® + Pulse oximetry * Transcutaneous gas analysis. (ony effective in infants d/ thin skin) RADIOLOGY 288. Answer is C (CT sean): Refer Text blow Bony architecture is best visualized by a CT sean The single best investigation of choice for assessing a lesion of the temporal bone is a CT scan MRL is now regarded the investigation of choice for all space occupying lesions of the brain whether in the anterior, ‘middle or posterior cranial fossa (Most useful for infratentorial posterior fossa lesions) but itis not the investigation of choice for assessing a lesion of the temporal bone. Thus an Intra cranial space occupying lesion within the temporal lobe may be better assessed by an MRI scan, but a lesion of the temporal bone such as fracture of the temporal bone is better investigated with a CT scan.289. 290. ‘AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007» 537 USG and X- Ray can only give limited information and cannot be regarded as investigation of choice Remember ‘+ Investigation of choice for multiple Bone Metastasis : Bone Scan® ‘+ Investigation of choice for Spine Metastasis : MRI® (Also visualizes spinal cord involvement) Investigation of choice for Acute Head injury : CT scan ® Answer is D (Diffuse axonal injuries): Dahnert 5/275; Osborn 2/212; Harrison 16/2448; Chapman 4/417 Diffuse axonal injuries (DAI) are shearing injuries caused by sudden rotational or accelerating / decelerating ‘forces. Patient typically lose consciousness at the time of impact, and on CT scan multiple small petechial ‘hemorrhages are seen. aa 7 a ‘SUBDURAL [MULTIPLE coo Saas | ee | HEMORRHAGE —_ INFARCTS Axonal shear — strain are Induced by brain striking on ‘Tearing of A gradual & global induced by sudden stationary calvarium bridging cortical reduction in cortical acceleration ‘deceleration = Coup veins? blood flow of normal ‘or rotational forces on = Contrecoup caging subjects brain. Seen in high velocity (vascular dementia) ‘rauma Location Lobar white mater Multiple B/L lesions ~ Between dura & — often asymmetrical particularly at grey commonly in fromtal & arachnoid + Frontal white matter interface temporal ibe = 95% = Temporal = Corpus callosum supratentorial Parietal lobe with ~ Dorsolatral aspect of (frontoparietl) —~ rnultiple areas of ‘upper brainstem iminished flow Imaging-CT Multiple small petechial + Easly Crescentic ext» = Covtical & hemorrhage <2 em = patchy ill defined low axial hematoma subcortical infarct diameter in cerebral density lesion (edema) mixed crossing suture - Large ventricles and hemisphere? with small hyperdense foci lines cortical sulci of petechial heamorthage = White matter + Delayed (24-48 hrs) Jeucencies ~ delayed heamorrhage = edema & mass effect + enhance after contrast administration MRI Most sensitive modality - Decreased intensity Multiple small face of| (hemorthage) surrounded by ‘decreased signal intensity _hypercense edema on T; WI Answer is A (Meningioma) Osborn Neuroradiology/584-585; Diagnostic Radiology by manorama Berry 2/ 361, Chapman 4/ 431] ‘Meningioma are the most common intracranial, extraaxial dural based neopalsm. Nearly all meningiomas enhance intensely following contrast administration. ENS Neoplasm el Intra-axial Extr | | = Neuronal + Pitutary® © Astrocytoma (Glioma)? + Schwannoma® «Lymphoma + Meningioma® * Of these only Meningioma is ‘dural based’ lesion. AIPGMEE 2007 - EXPLANATIONS532 AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 291. 292. 293. Answer is B (Wilson’s disease): Dahnert 5"/ 240; Chapman 4"/ 430 Wilson's disease has not been mentioned in association with Basal Ganglia calcification. 1. Physiological with aging 2. Endocrine - Hypoparathyroidism®, Pseudohypoparathyroidism®, Hypothyroidism®, Hyperparathyroidism® 3. Metabolic - — Fakr’s disease®, Mitochondrial defects, Cockayne’s syndrome 4. Toxic - Birth anoxia®, co poisoning, Lead poisoning 5. Infection = Toxoplasmosis, congenital rubella, CMY, cysticercosis, 6 Chemotherapy / Radiation 7._ Vascular malformation? Answer is D (Obesity): Harrison 16°/1647; Sutton 7°/927; Grainger 106 Obesity itself has not been mentioned as contraindication for the use of radiocontrast ‘+ Contrast nephropathy classically presents as rise in blood urea nitrogen and creatinine and is more common in individuals with pre existing: = Chronic renal insufficiency? ~ Diabetes mellitus - CCF = Hypovolumia (dehydration)? ~ Multiple myeloma ~ Patient on metfomin® ‘+ Interaction between Metformin & contrast agent ‘Metformin is excreted by kidneys as an active compound. In case of renal insufficiency metformin accumulate and ‘may increase the risk of lactic acidosis. Both metformin & radioconstrast causes renal insufficiency and should not be used together®. Answer is A (I) olf gang Dahnert 5*/ 1087 1123 is used as an isotope for RAIU. RAIU: Radio active iodine uptake/ Thyroid count /capsule count is a thyroid imaging method that measures the fraction of orally administered iodine isotope taken up by the thyroid. It is measured at intervals of 4 & 24 hours after administration of the isotope. ities of thyroid scintigraphy are: + Te 99m pertechnitate sean + 1 scan [Agent of choice/® . Interpretation of RATU Normal Decreased In Grave's disease # <25% at 4 hours # Insubacute thyroiditis, © < 35% at 24 hrs * However RAIU is not diagnostic of hyper thyroidism without measurement of hormonal levels./AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 @ 533 PSYCHIATRY 294, 298. 297. 298. 299. Answer is A (Thought): Kaplan & Sadock’s 9°/281- 83; Neeraj Ahuja 5"/ 86 4"/13; Current Diagnosis & Treatment In Paychiatr p-95-113; Shorter Oxford Textbook of Psychiatry 5%/2-20 Delusion is a disorder with disturbed content of though’, Hallucination & illusion are disorders of perception®. Delirium & dementia are disorders of cognition’; and insight is disturbed in psychosis®, Answer is B (Depression alone): Kaplan & Sadock's 9"/542 ,534 ~60; Harrison 16/2536; Neraj Alnja 50/74; Current Diagnosis & Treatment In Psychiatry/312-322; Shorter Oxford Textbook of Psychiatry 5"/227, 247-251 Patients who are afflicted only with major depressive episodes are said to have — major depressive disorder or unipolar depression. Patients with both manic and depressive episodes or patient with manic episodes alone are said to have bipolar disorder. The term unipolar mania, pure mania, or euphoric mania are sometimes used for bipolar patients who do not have depressive episodes. Answer is B (Fluoxetine): Harrison 16"/2552; Kaplan & Sadock's 9"/622-23, 209; Neerak Ahuja 6/100 ‘Selective Serotonin Reuptake Inhibitors (SSRI) especially Fluoxetine are the drugs of choice for OCD? ‘Management of OCD © Is treatment of choice® ‘© SSRIS are drug of choice®, Fluoxetine®, fluvoxamine, paroxetine, sertraline have been ‘+ Includes ~ exposure & response , prevention® (most important), Sy eae desensitization, thought stopping, flooding, ‘+ Clomipramine: of all the tricyclic & tetra cyclic implosion therapy, and aversion drugs, clomipramine is the most selective for conditioning reuptake exceeded in this respect only Second line drugs include Valproate, lithium, ot carbamazepine®, venlafaxine, pindolol, & MAO inhi hydroxy tryptamine (5-HT), L-tryptophan, & clonazepam. itors esp phenelzine, buspirone, 5 — Answer is B (Tremor): Kaplan & Sadock's 9"/403-405; Neraj Ahuja 5"/40; Current Diagnosis & Treatment In Psychiatry 240-259 Tremulousness (shake, tremor or Jitter) is the classical & most common sign of alcohol withdrawl. Hangover (next morning) is the most comomon symptom® of aleohol withdraw! syndrome. Answer is C (To prevent relapse): Kaplan & Sadock's 9/1082 -87, 448-456; Neraj Ahuja 5/46.) KDT 5"/425; Shorter Oxford Textbook of Psychiatry 5§/358-361; Neeraj Ahuja: A Short textbook of psychiatry 6°/45-48 Naltraxone is a long acting opioid antagonist. It is used in patients of opioid dependence for detoxification and withdrawal, It discourages substance seeking behaviour and thus prevents relapse. It may lead to withdrawal symptoms? in addicts d/t blockage of opioid receptors. Answer is A (Cannabis) Kaplan & Sadock's 9"/444, 424.27; Park 18/635; Shorter Oxford Textbook of Psychiatry 5"/332-342; Neeraj Ahuja 6%/48 By most estimates, cannabis (Indian hemp plant) remains the world’s most commonly used illicit drug. Cannabis is perhaps the most widely used drug in India also.534 © AIPGME EXAMINATION ANSWERS AND EXPLANATIONS - 2007 300. Answer is A (Autism): Ghai 6/65; CPDT 18"V186, 187 Difficulty in concentration and communication, delayed speech and problems in forming social relationships (not ‘making any friends) in early childhood (onset of symptoms before the age of 3 years) suggests the diagnosis of Autism, «Onset in infancy or early childhood (before age of 3 years) + Abnormal language and speech development (Abnormal communication) ~ Inability to develop normal social skill lack of eye contact gestures and facial expression) ~ Understand little or no language (therefore fail 0 acquire speech) - Deficient comprehension and communicative use of speech and gesture. * Behavioural peculiarities such as ritualized repetitive or stereotyped behaviour and rigidity * Difficulty in concentration. + Severe deficit in social responsiveness and interpersonal skills ~ Intrusive sterotypes (Repetitive behaviour) together with inability to concentrate may prevent children from engaging in meaningful activity or social interaction (difficulty in making friends) = Do not engage in pretended play (which starts before age of 2 in normal children) © Mental retardation (about 75% of children with autism are mentally retarded) # Epilepsy develops in one fifth to one third of autistic individuals «EEG abnormalities are noted in half ofthe patients.