Histological variant of nephrotic syndrome with atypical presentation in children.

Aims of the present study were to find out the histological pattern of atypically presenting nephrotic syndrome children and their response with oral corticosteroid therapy. This prospective study was carried out in the Paediatric Nephrology unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, for a period of two years, from September 2001 to September 2003. Forty children with nephrotic syndrome with atypical presentations such as gross haematuria, renal impairment, hypertension and hypocomplementaemia were enrolled for the study. For each patient proper investigations were done to screen out infection and necessary treatment was given accordingly. Renal biopsy was done in all cases after treating infection and controlling hypertension and specimen were evaluated by light and immunoflourescence microscopy. All the patients were treated with oral prenisolone 60 mg/m2/day for 4 weeks and response were observed by heat coagulation test. In present study it was observed that mean age of presentation was 8.73+/-4.36 years. Hypertension, gross haematuria, impaired renal function and hypo-complementaemia were observed in 50%, 45%, 19% and 15% cases respectively. When histopathologies were considered, 90% cases had other than minimal change disease (MCD) and Mesangial proliferative glomerulonephritis (MesPGN) was the most common histopathology (47.5%). Regarding steroid therapy only 43.2% patients were responsive to oral prednisolone therapy within 28 days. Atypically presented nephrotic syndrome usually manifested with higher age of onset, gross haematuria, hypertension, renal insufficiency and hypocomplementaemia. Mesangial proliferative glomerulonephritis was the predominant histological type. The majority (56.8%) of these atypically presented nephrotic syndromes were resistant to steroid therapy.

A case of renal transplantation.

A 12-year-old boy was admitted in paediatric nephrology unit of Bangabandhu Sheikh Mujib Medical University (BSMMU) with massive proteinuria, hypertension, respiratory distress and anaemia and diagnosed as nephrotic syndrome. Percutaneous needle biopsy was consistent with diffuse endocapillary proliferative glomerulonephritis and initially managed conservatively with injection methyl prednisolone, cyclophosphamide, lisinopril etc. without any improvement. Living-related renal transplantation was done successfully from paternal uncle. Two episodes of acute rejection occurred, one immediately after transplantation and another after one month. These were managed with IV methyl prednisolone for 3 days. At present, he is on oral prednisolone, cyclosporine, azathioprine and antihypertensives with normal haemoglobin and stable serum creatinine level (pre-transplant level 12.5mg/dl to post-transplant level 1.5mg/dl). He has been maintaining his normal life including schooling for last few months. It is concluded that a patient with uncommon presentation of nephrotic syndrome should be confirmed by renal biopsy and renal transplantation may be considered if conservative measures fail.

Congenital nephrotic syndrome, an uncommon presentation of cytomegalovirus infection.

Cytomegalovirus (CMV) infection is a very rare infection in children as well as in infancy. In CMV infection extra renal manifestations in central nervous system (CNS), eyes and hematological are prominent and common than renal manifestation. We are describing two case reports one at the age of 5 months and another at one month with features of both extra renal and renal manifestations of nephritic syndrome. Our first case presented with predominant features of CNS manifestations like convulsion and spastic monoplegia, sensory type of deafness and absence of light reflexes along with nephritic features. Deafness and absence of light reflex were confirmed by audiometric and ophthalmological examination and brain atrophy was confirmed by CT scan. Our second case had features of hypothyroidism along with nephritic features. Hypothyroid status was confirmed by elevated serum TSH level and reduced T4 level. In both cases nephritic features were confirmed at bed site urine examination by heat coagulation test and other relevant investigations. CMV infection was confirmed in both cases by detecting anti CMV IgM by ELISA method. Both patients were clinically improved with intravenous gangcyclovir therapy. In these two cases reports, clinical, serological and therapeutic observation established the causal relationship between the CMV infection and nephrotic syndrome.