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Cushing Syndrome

- Present History: (Compaint+Systems Review)

• Obesity: OCD+FOPSM Features (increased tummy fat – Facial fullness/moon face –
increased fats in the neck and the shoulders) – Onset (sudden- gradual) – Puberty – School
Performance – Measurements
• Other Concerns: Dysmorphic Features – Chronic Illness
• GIT: Tummy Size – Tummy Pain – Throwing-up esp. in the morning – Change in Poo
• Haematology: Pallor – Bruises – Bleeding - Bugs
• General: FWASL + Activity: Recent Illness
• CNS: Headache - Weakness – Facial Asymmetry – Eye Deviation – Concern about Vision,
Hearing or Speech – Abnormal Walk – Change in her mood
• Chest: Asthmatic? – Snoring During Sleep – Cough – Recurrent Bugs
• CVS: Fainting – Exercise Intolerance – Awareness of Heart Beat – Blood Pressure
• Urinary: Change in Pee amount? – colour – Pain in loin or during Peeing
• MSK: Skin Spots /Pigmentation – Stretch Marks – Acne – Increased Body Hair - Muscle
Weakness (difficulty in climbing stairs or in getting up) – Swellings (hands/feet)
• Endocrine: Cold Intolerance – Blood Glucose test
- Past: 5T IOA: Steroid Intake, How Long?
- Perinatal
- Puberty Did she start her menses? Is it regular? – Does she have a boyfriend? – OCPs
- Vaccination
- Allergy:
- Nutrition: Is She on a special diet? - Type of Food/ Amount
- Development:
- Family: History of PCO
- Social/Psychological/Impact: any Psychological Problem – School Performance (if not
asked) – Relation with Peers - Bullying

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DD of Cushing

i- Exogenous: in most cases: Steroid Intake for Chronic Condition (Asthma – IBD – SLE/JIA -
Nephrotic)

ii- Endogenous:
1. Corticotropin – Dependent (↑ ACTH) associated with skin pigmentation
- Pituitary Adenoma
- Ectopic Corticotropin from Lung Small Cell Carcinoma - Pheochromocytoma – Pancreatic
Neuroendocrine tumours – medullary thyroid cancer
2. Corticotropin – Independent (N or ↓ACTH)
- Adrenal Adenoma (or Carcinoma) = usually unilateral
- Rare Causes: McCune-Albright

iii- Pseudo-Cushing: PCO / Depression / Obesity

iv- Eating Disorders

v- Hypothyroidism

DD of Obesity
Short
Tall
Normal IQ ↓IQ

Endocrine: Syndromes:
- Growth Hormone Deficiency - Down - Simple
- Cushing - Prader Willi - Beckwith Wiedemann
- Hypoparathyroidism - Laurence Moon - Klinefelter
- Pseudo hypoparathyroidism - Bardet Biedl

- Hypothyroidism

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Discussion: Obesity/Cushing
Obesity: Def. According to the BMI:
• 91 th centile < overweight
• 98 th centile < obesity
• 99.6th centile < morbid obesity

Features:
- Acanthosis nigricans – obstructive sleep apnoea/snoring
- Excess Steroid = striae rubrae – hirsutism – moon face – buffalo hump – high Bl.Pr.
- PCO = similar features (Pseudo-Cushing)
- Non-Alcoholic Fatty Liver Disease
- Vision (Pituitary adenoma)

What to do next:
- Complete history
- Take Vitals (Bl. Pressure/Blood Glucose/Pulse oximetry)
- Plot Measurements: Wt. – Ht. – BMI

Investigations:
- For Diagnosis/Complications:
• Body measurements
• Puberty Assessment
• Blood Pressure
• Glucose Tolerance Test / HbA1C
• Pulse Oximetry
• Sleep Study / Over Night Saturation
• Liver Function Tests
• X-ray for Slipped Upper Femoral Head
- To reveal the cause in 2ry Obesity:
• Midnight Salivary Cortisol
• Thyroid Function Tests
• Genetic Testing for Laurence Moon
• PCO: LH, FSH, Testosterone, 17hydroxyprogesterone, Sex Hormone Binding Globulin, Pelvic U/S
• Pituitary Adenoma: CT/ MRI
• Adrenal Adenoma: Abdominal U/S, CT

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How to differentiate between exogenous(iatrogenic) and endogenous Cushing?
1- By history
2- Clinically
• Iatrogenic= Hypertrichosis + delayed puberty
• Endogenous = Hirsutism + Precocious Puberty
3- Lab: Measure free cortisol (24 hrs. urinary or salivary cortisol)
• Iatrogenic = low free cortisol
• Endogenous = high free cortisol
How to differentiate between types of endogenous: Pituitary vs Non-Pituitary?
1- High Dose Dexamethasone Suppression Test
• Pituitary= ↓ Serum Cortisol
• Non-Pituitary = No Difference
2- ACTH Stimulation Test
• Pituitary= ↑ Serum Cortisol
• Non-Pituitary = No Difference

Treatment
1- Family Based Therapy: all the family
• Life Style Modification: Diet – Exercise > 20m. > 3days/week
• Manage sedentary life
• Involve Parents
• Behavioural Strategies
2- Liaise with School:
• Exercise
• Food
3- Team Involvement:
• Dietitian
• Psychiatrist
• Bariatric Surgery if BMI > 40 or with Co-morbidities

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4- Management of Complications:
• Dyslipidaemia:
- Diet advice
- Statins if age > 10yrs.
• Type2 DM:
- Metformin once daily from age of 8 .
• Microalbuminuria: Albumin/Creatinine Ratio>3.5 mg/mmol
- Involve Nephrologist.
• HTN: systolic or diastolic> 95th Centile, confirmed by ambulatory Bl. Pr.
- Diet, exercise, life style modification.
- Medication: ACEI.
• Non-Alcoholic Fatty Liver
- Diet and Exercise
- Refer to Hepatologist if hepatic transaminases> 2x the upper limit of normal

• OSA:
- Discuss with consultant
- Refer to ENT for adenotonsillectomy
• Depression:
- Involve Psychiatrist
- Refer to child and mental health service

5- Iatrogenic Cushing: Discuss with the Consultant/ Respiratory/ Nephrologist/

Gastroenterologist: Steroid Treatment Dose, Tapering and Alternative
6- Regular Follow-Ups:
- Annual Screening for Complications
• 1ry Obesity:
- GP follow up
• 2ry Obesity:
- Paediatric follow up
- Relevant Referral according to cause

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