Case Presentation

Hanan Fathy Ass. lecturer Pediatric nephrology department

• Male patient, 3.5 years old, from ElBehera.

• Generalized edema • Hematuria • Headache

3 days before admission

• The patient suffered from gradual onset of edema that started by morning puffiness. • The mother noticed abdominal distension, and change in the color of urine. • The patient also complained of headache. • Upon seeking medical advice; U/S abdomen, renal functions and urine analysis were requested.

• The patient was puffy, he looked ill. • He was hypertensive, no fever. • Weight was slightly less than expected for age, and the patient was short. • There was generalized edema and moderate ascites.

• The patient had impaired renal functions. • Urine analysis revealed the presence of smoky colored urine, there was protinuria, hematuria and granular casts. • Urine culture was sterile. • The patient also had a low C3.

Trend of progression of renal functions Dialysis

250 200 150

urea

100 50 0 Day1

Day2

Day3

Day4

U/S abdomen at a private clinic:

The Rt kidney shows grade II hydronephrosis , dilated ureter, evidence of supra renal mass partially solid partially cystic. The left kidney is atrophic with dilated ureter.

The left kidney was not visualised, The left ureter was seen dilated. The right kidney shows malrotation, Atrophic thinned out parenchyma, grade III Hydronephrosis and dilated ureter.

Medical problem

Urologic illness

• The patient was dialyzed. • He was given pulse steroid therapy to halt pathology progression. • He was given pulse cyclophosphamide as the pulse steroids didn't stop deterioration of renal function. • We had to do five sessions of plasma pharesis on account of unsatisfying results of previous two measures.

There is crossed fused ectopia, where each kidney is malrotated and located in the right hypchondrium, each showing grade II-III hydro nephrosis No supra renal masses

HOW DID THE CASE PROGRESS • Following intensive medical measures to control deterioration in renal functions, the patient didn't show marked improvement. • Dialysis was done twice after cessation of pharesis sessions. • A urinary catheter was fixed in place, initial improvement in renal functions, however after a short while renal function deteriorated and we had to dialyze again.

HOW DID THE CASE PROGRESS • The patient was put on non dialytic therapy and chemoprophylaxis. • A DMSA scan was done that showed very poor function in both kidneys (worse on the left). • DTPA revealed a GFR in the Rt kidney 11.3 ml/min and in the Lt kidney 2.3 ml/min and a non obstructive pattern.

Urinary catheter was removed The patient was sent home on non dialytic therapy and chemoprophylaxis He was scheduled for a regular follow up and diyalysis when needed regimen( he has was polyuric, was not dialysis dependent)

In his follow up visits, no further deterioration of renal functions was noted Last BUN:46 mg/dl S.Cr:1.7 mg/dl We didn't have to re dialyze ever since he was sent home