LETTER TO THE EDITOR
Year : 2013 | Volume
: 24 | Issue : 3 | Page : 590--591
Response to steroids in early-onset nephrotic syndrome
Bilal Aoun1, Sami Sanjad2, Claus Peter Schmitt3, Ghinwa Kalkas4, Hassan Fakhoury4, Tim Ulinski5,
1 Department of Pediatric Nephrology, American University Hospital; Department of Pediatric Nephrology, Rafk Hariri University Hospital, Beirut, Lebanon; Department of Pediatric Nephrology at Armand Trousseau, AP-HP, Paris, France
2 Department of Pediatric Nephrology, American University Hospital; Department of Pediatric Nephrology, Rafk Hariri University Hospital, Beirut, Lebanon
3 Division of Pediatric Nephrology, Center for Pediatric and Adolescent Medicine, Heidelberg, Germany
4 Department of Pediatric Nephrology, Rafk Hariri University Hospital, Beirut, Lebanon
5 Department of Pediatric Nephrology at Armand Trousseau, AP-HP, Paris, France
Department of Pediatric Nephrology, American University Hospital; Department of Pediatric Nephrology, Rafk Hariri University Hospital, Beirut, Lebanon; Department of Pediatric Nephrology at Armand Trousseau, AP-HP, Paris, France
|How to cite this article:|
Aoun B, Sanjad S, Schmitt CP, Kalkas G, Fakhoury H, Ulinski T. Response to steroids in early-onset nephrotic syndrome.Saudi J Kidney Dis Transpl 2013;24:590-591
|How to cite this URL:|
Aoun B, Sanjad S, Schmitt CP, Kalkas G, Fakhoury H, Ulinski T. Response to steroids in early-onset nephrotic syndrome. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2022 Jan 28 ];24:590-591
Available from: https://www.sjkdt.org/text.asp?2013/24/3/590/111076
To the Editor,
Infantile nephrotic syndrome (INS) is a rare kidney disorder characterized by heavy proteinuria, hypoproteinemia and edema, manifesting mainly in first three to 12 months of life. , The majority of these cases have a genetic origin; therefore, the treatment of INS is considered a challenge to pediatric nephrologists because of the prevalence of the high resistance rate to steroids (up to 90%).  Once end-stage renal disease develops or following preemptive bilateral nephrectomy in the treatment of resistant patients' kidney transplantation is considered as a feasible option with success rates similar to other renal diseases.  We describe two infants who were treated with steroids and attained complete remission.
A 7-month-old male, previously healthy and born by normal vaginal delivery with a smooth course of pregnancy to second-degree consanguineous parents, presented with progressive generalized edema. All blood investigations including C3, C4 levels, toxoplasmosis, HIV, hepatitis B antigen, antinuclear antibodies and anti-DNA levels, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) levels were negative. The patient had an albumin level of 13 g/L and 24-h urine proteinuria was 5 g. No abnormality in the NPHS1 gene (coding for nephrin) was detected. Kidney biopsy showed focal and segmental glomerulosclerosis. The child was started on steroids (prednisone) at a dose of 60 mg/m 2 day, which was decreased progressively over four months and 15 days, with administration of vitamin D, L-thyroxin, and daily intravenous albumin perfusions (2 g/kg), and anticoagulant administration (antivitamin K). Seven days after starting steroids, the proteinuria cleared. Six months later, the patient was still in remission. Steroids were maintained at 5 mg every other day. The child is growing well, in addition to having normal psychomotor development.
The second patient is a 6-month-old female known to have myelomeningocele operated at the age of two months. She was born by cesarean section to first-degree consanguineous parents, presented with progressive generalized edema. All investigations for secondary nephrotic syndrome were negative. Serum albumin level was 15 g/L and 24-h proteinuria was 7 g. Open kidney biopsy showed the presence of changes as those seen in Finnish type nephrotic syndrome (irregularly dilated proximal and distal tubules with increased number of cytoplasmic vacuoles filled with colloid-like material). Prednisone at a dosage of 60 mg/ m 2 /day was started in association with vitamin D and calcium supplementation, and was tapered over four months 15 days. On day 10, proteinuria cleared. In this case, no genetic mutation was found. At four months follow-up, the patient was still in remission and she was maintained on steroids at 5 mg every other day.
The treatment of nephrotic syndrome manifested in the first year of life is considered a challenge to pediatric nephrologists.  Both our patients responded to steroids in contrast to other studies that showed no benefit of steroids and other immunosuppressive treatments. Considering a trial of steroids in patients with INS seems to be entertained, since a considerate number may benefit from such treatment and can possibly have a good long term prognosis.
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