Uma Ali

Superior sagittal sinus thrombosis in a child with nephrotic syndrome

A 3-year-old male with steroid-responsive nephrotic syndrome developed a rare complication, sagittal sinus thrombosis during an episode of gastroenteritis, while on steroid therapy. Anticoagulation, as assessed by partial thromboplastin time, was difficult to maintain, despite administering high doses of heparin, infusions of fresh-frozen plasma to provide antithrombin III, and, subsequently, maximum doses of warfarin (0.3 mg/kg per day). Despite these problems the child made a complete neurological recovery.

Renal tubular dysgenesis associated with in utero exposure to Nimuselide

Maternal ingestion of the selective cyclo-oxygenase-2 (COX-2) inhibitor Nimuselide has been reported to be associated with the development of oligohydramnios and neonatal renal failure in some cases. We report a case of neonatal renal failure associated with maternal ingestion of Nimuselide in the third trimester of pregnancy. The neonate presented with metabolic acidosis and non-oliguric renal failure on the second day of life. The renal histopathology showed evidence of renal tubular dysgenesis. The child continues to have elevated serum creatinine and hypertension at 10 months of age.

Glomerular basement membrane abnormalities in infants with heavy proteinuria

Two Indian male children with infantile-onset heavy proteinuria (with nephrotic syndrome in 1) had thickening of the glomerular basement membrane with splitting and basket-weave appearance of lamina densa on electron microscopic evaluation of kidney tissue (like Alports syndrome), with normal light microscopic findings and negative immunofluorescence. The proteinuria was non-familial and was not associated with microhaematuria in patient 1; transient microhaematuria, perhaps associated with urinary tract infection, was noted in patient 2. There was no neurosensory deafness in the patients or their parents. The nephrotic syndrome remitted totally in one patients over a 7-month period. The proteinuria, as well as the renal disease, was non-progressive in the second patient over a 27-month period. The significance of these basement membrane abnormalities (classically described in Alports syndrome) in early-onset nephrotic syndrome/heavy proteinuria that is non-familial and non-progressive needs to be evaluated.

Follow-up of two children with basement membrane abnormalities

We would like to report the follow-up of two children aged 10 months and 15 months who presented with nephrotic-range proteinuria. Kidney biopsy revealed Alport-like abnormalities, namely basket-weave appearance with thickening and splitting of the lamina densa of basement membrane on electron microscopy. Light microscopy was unremarkable in both these patients. We have published these cases previously in Pediatric Nephrology [1]. Currently both the boys, now 10.5 years and 11 years of age, are well. One has no proteinuria, edema, azotemia, or hypertension with normal growth. The other has nephrotic-range proteinuria (urinary protein to creatinine ratio between 4 and 5 mg/mg), but has no edema, azotemia, or hypertension, and has normal growth parameters. The boy with proteinuria has been receiving oral enalapril for the last 4 years. We have not repeated the kidney biopsies. Neither boy has developed hematuria, deafness, or eye abnormalities described in Alport syndrome. It was interesting to read a report by Akhtar and Sabban [2] of a similar case in a 17-year-old Saudi girl, and we wanted to share our experience with readers. There must be more cases with basement membrane abnormalities that were thought to be characteristic of Alport syndrome in other glomerular diseases.

Acute respiratory distress during paradoxical reaction to antituberculous therapy in an 8-month-old child.

Paradoxical reaction during antituberculosis treatment (ATT) is commonly seen as tuberculous lymphadenitis of peripheral lymph node, cerebral tuberculomas, pulmonary infiltrates, and pleural disease. This phenomenon is more commonly associated with extrapulmonary tuberculosis and disseminated tuberculosis. Respiratory distress, as presentation of paradoxical reaction, is rare. We report an 8-month-old child with primary progressive tuberculosis without mediastinal adenopathy, who developed paradoxical reaction with extensive mediastinal adenopathy within 15 days of ATT and presented with severe respiratory distress. The child responded to short course of high-dose steroids.