Case conference

5/6/56

Chief complaint
5 . . .

CC : 5

Present illness
5 . 2 2 . 2 1 . .

Past history
6 . nephrotic syndrome .. 2555 .. 2556 prednisolone 1x1 pc 3 2556

History
: : : autoimmune

Physical examination
V/S : T 36.5 C, BP 96/76 mmHg HR 96 /min, RR 22 /min Weight 15 Kg GA : Active, mild pale, no jaundice, no dyspnea, generalized pitting edema, puffy eyelids

Physical examination
HEENT : dental caries, pharynx & tonsil not injected CVS : normal S1 and S2, no murmur RS : normal breath sounds both lungs Abdomen : distension, soft, not tender , active BS liver and spleen not palpable, shifting dullness + Genitalia : Ext: pitting edema 2+ both legs NS : Within normal limit

Problem List
1. Generalized pitting edema with puffy eyelids for 5 days

Differential Diagnosis
Renal disease: - Acute glomerulonephritis - Nephrotic syndrome

Investigation
UA : 10 ml pH 8.0 , S.G.1.015 , Protein 1000 mg/dl 4+ Glc : neg , ketone : neg , blood : neg , bilirubin :neg , nitrite : neg WBC 3-5 Epi 0-1 , no RBC

Lab Investigation
Stool exam : not found parasite Spot urine protein to Cr ratio: 5.1 mg/mg (random urine protein 360) (random protein Cr 70.53)

Lab investigation
23/5/56 CBC: Hb 12 g/dl, Hct 35.2%, MCV 79 fL, MCH 27 , RDW 14 % WBC 13600 (N31,L60%) cell/mm3, Platelets 439000 /mm3 PMN 46.3 L 46.8 Mo 6.3 Eo 0.5 Ba 0.1 Albumin0.8

23/5/56 Bun: 11 / Cr: 0.09 / alb: 0.8 / Lipid profile : chol 582/ TG 440 / HDL 55/ LDL 365/ Na 137 / K 4.25 / Cl 107.8 / CO 20.1 Lipemic serum : 1+

25/5/56 alb 1 26/5/56 Na 139, K 4.29 , Cl 107 , HCO3 24.2 mEq/dL Total chol 441, Albumin 1.4 , Ca 7.70 ,Po 4.8 , Mg 1.9 27/5/56 alb 2.5

Progress note
Admit On heplock Record v/s, I/O 20% Alb 75 ml + lasix 20 mg IV drip in 4 hr Record V/S Alb Start Prednisolone (5) 3-2-3 oral pc 24/5/56 20% Alb 75 ml + lasix 20 mg IV drip in 4 hr Serum alb 2

Progress note
25/5/56 20% Alb 75 ml + lasix 20 mg IV drip in 4 hr Elyte Ca Mg Po Alb C3 , C4 (lab ) 26/5/56 20% Alb 75 ml + lasix 20 mg IV drip in 4 hr Serum Alb , UA

Progress note
D/C today f/u 1 wk + UA , serum alb ,BUN ,Cr HM Prednisolone(5) 3-2-3 oral pc //90 Alum milk 1 tsp oral pc //1

Nephrotic syndrome
Criteria 1. Generalized edema 2. Massive proteinuria >40 mg/m2/hr or >50 mg/kg/d or Spot UPCR > 2 mg protein/mg creatinine 3. Hypoalbuminemia (<2.5 g/dl) 4. Hypercholesterolemia (>250 mg/dl)

Complication of proteinuria
Infection Urinary loss of Ig -> loss of immunity function (Opsonization) Spontaneous bacterial peritonitis is most frequent type of infection ascites + bacterial translocation from swollen bowel with loss of immunity function pneumococci is most frequent organism

Complication of proteinuria
Thromboembolic event related to increased prothrombotic factors and decreased fibrinolytic factors Prophylactic anticoagulation is not recommended in children unless they have had a previous thromboembolic event Cardiovascular event myocardial infarction is a rare complication in children

Primary (idiopathic):
Minimal change disease (Most common cause in children ) Membranous Nephropathy (Most common cause in Adults) Focal Segmental Glomerulosclerosis (Most Common cause in African Americans) Membranoproliferative Glomerulonephritis

Secondary DM (the leading cause of secondary nephrotic syndrome) SLE Amyloidosis ect. Infections: Hepatitis B and C, HIV, Syphilis Malignancy: Multiple myeloma , Hodgkins disease, Solid
Organ Tumor

Drugs: NSAIDs, Gold, Penicillamine ,Heavy metals etc Anatomic Dysfunctions Reflux Nephropathy, Hypoxic
Nephropathy

Characteristics of primary nephrotic syndrome

 Generalized Edema -The face, particularly the periorbital area, is swollen in the morning& lower extremities and genital area later in the day -In advanced disease: the whole body Anasarca : shortness of breath Frothy urine and urine dipstick proteinuria value of 3+

Clinical presentation

Supportive Management
Diet High protein diet ~ 130-140% of requirement Avoid saturated fat compound Edema Salt restriction IV albumin in severe pleural effusion ,scrotal swelling ,skin infection Vaccination : pneumococcal vaccine , VZV vaccine

Specific Management
Preparation Investigate & Eradicate infection : dental examination,stool for parasite, tuberculin skin test Start oral prednisolone 2 mg/kg/day not more than 60 mg/day in single dose/day Duration 2-4 weeks or negative for protein for 3 consecutive days but not more than 8 week Tapering off steroid dosage

Strategy to tapering off steroid

2 mg/kg/d max to 60 mg/day x 6 consecutive weeks 90% remission within 2 weeks but initial 6 week course will lower relapse rate Then tapering to alternate day single morning dose and slowly tapered and discontinued within 2-3 months

Specific Management
Alternative therapy eg. cyclophosphamide Relapse on prednisolone>0.5 mg/kg/alternate day with one of the following Unacceptable SE of steroid therapy High risk of toxicity approaching puberty or DM Unusally severe relapse ARF ,sepsis ,thrombosis Inadequate facilities for F/U or concerning about compliance Relapse on prednisolone>1 mg/kg/alternate day

Form of Response to treatment

Remission : Urine protein <40 mg/m2/hr or strip 0 - trace for 3 consecutive day Relapse : Urine protein >40 mg/m2/hr or strip 1+ up for 3 consecutive day and previous remission Frequent relapse : 2+ relapse within 6 months of initial response or 4+ relapse within any 12 months Late responder : remission after 4 weeks of prednidolone 60mg/d without other drug

Form of Response to treatment

Steroid responsive : remission archived with single steriod alone Steroid dependence : 2 consecutive relapse occuring during steriod treatment or within 14 days of its cessation Steroid resistance : failure to archive response in spite of 4 weeks of prednisolone 60 mg/d

Complication of steroid use

Growth retardation, excessive weight gain, fluid retention (Cushinoid Face) (Hyperglycemia) (Proximal Muscle Weakness) (Hirsutism) (Osteoporosis) (Sodium Retention) (Buffalo Hump) (Striae) Skin (Hyper / Hypopigment)