Abstract
The study includes 17 infants with nephrotic syndrome. Their ages at the onset of disease ranged from 10 days to 10 months (median 2 months), and included 10 males and 7 females. Ten patients had consanguineous parents and 4 had a history of similar illness in the sibling. Renal biopsy revealed diffuse mesangial proliferation in 10 patients, diffuse mesangial sclerosis in 3, Finnish microcystic disease in 2, and focal segmental glomerulosclerosis and atypical glomerulopathy in 1 each. Unilateral nephrectomy was performed in 8 patients with intractable proteinuria associated with multiple complications. Five are well after 4–36 months follow-up, 1 died of septicaemia soon after the procedure, 1 needed dialysis and is stable at 26 months and 1 showed no reduction in the degree of proteinuria. We conclude that unilateral nephrectomy is a better alternative to bilateral nephrectomy in some infants with severe nephrotic syndrome.
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References
Sibley RK, Mahan J, Mauer SM, Vernier RL (1985) A clinicopathologic study of forty-eight infants with nephrotic syndrome. Kidney Int 27: 544–552
Mahan JD jr, Vernier RL (1987) Congenital nephrotic syndrome. In: Holliday MA, Barratt TM, Vernier RL (eds) Pediatric nephrology, 2nd edn. Williams and Wilkins, Baltimore, pp 457–462
Mahan JD, Mauer SM, Sibley RK, Vernier RL (1984) Congenital nephrotic syndrome: evolution of medical management and results of renal transplantation. J Pediatr 105: 549–557
Kaplan BS, Bureau MA, Drummond KN (1974) The nephrotic syndrome in the first year of life: is a pathologic classification possible, J Pediatr 85: 615–621
Mattoo TK, Giangreco AB, Afzal M, Akhtar M (1990) Cystic lymphangiectasia of the kidneys in an infant with nephrotic syndrome. Pediatr Nephrol 4: 228–232
Report of the International Study of Kidney Disease in Children (1981) The primary nephrotic syndrome in children. Identification of patients with minimal change from initial response to prednisone. J Pediatr 98: 561–564
Mattoo TK, Akhtar M (1990) Familial glomerulopathy with proximal tubular dysfunction: a new syndrome. Pediatr Nephrol 4: 223–227
Mattoo TK, Mahmood MA, Al-Harbi MS (1990) Nephrotic syndrome in Saudi children, clinicopathological study of 150 cases. Pediatr Nephrol 4: 517–519
Holmberg C, Jalanko H, Leijala M, Salmela K, Ahonen J, Koskimies O (1988) Results of active treatment of 17 children with congenital nephrotic syndrome of the Finnish type (CNF). Pediatr Nephrol 2: C124
Coulthard MG (1989) Management of Finnish congenital nephrotic syndrome by unilateral nephrectomy. Pediatr Nephrol 3: 451–453
Thorner PS, Arbus GS, Celermayer DS, Baumal R (1984) Focal segmental glomerulosclerosis and progressive renal failure associated with a unilateral kidney. Pediatrics 73: 806–810
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Mattoo, T.K., Al-Sowailem, A.M., Al-Harbi, M.S. et al. Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy. Pediatr Nephrol 6, 16–18 (1992). https://doi.org/10.1007/BF00856821
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DOI: https://doi.org/10.1007/BF00856821