Successful treatment of Finnish congenital nephrotic syndrome with captopril and indomethacin,☆☆,

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Abstract

Two infants with biopsy-proven microcystic Finnish congenital nephrotic syndrome (onset at birth) were treated with a combination of captopril and indo methacin for 21/2 and 2 years, respectively; they had a marked reduction of urinary protein excretion without further need for albumin infusions. One infant has end-stage renal disease; the other infant's glomerular filtration rate has remained within normal limits. (J PEDIATR 1995;126:140-2)

Section snippets

Patient 1

An Israeli Arab boy was born to nonconsanguineous parents at 38 weeks of gestation after an uneventful pregnancy. At delivery, a large placenta was noted. During the first 10 days after birth, edema developed. Laboratory evaluation revealed the following: proteinuria, 500 mg/dl; serum albumin, 7 gm/L; serum creatinine, 35.4 μmol/L (0.4 mg/dl); urea, 14.4 mmol/L (87 mg/ dl); and cholesterol, 7.3 mmol/L (284 mg/dl). The family history was noncontributory; a 3-year-old sister is healthy. A renal

DISCUSSION

These two infants had severe proteinuria within the first month of life. Renal biopsy specimens from both infants showed findings typical of FCNS (Prof. R. Habib, Hôpital des Enfants Malades, Necker Hôpital, Paris, corroborated our interpretation). The onset of the nephrotic syndrome within 3 months of birth, coupled with the biopsy findings, established the diagnosis of microcystic FCNS.

Accepted treatment regimens for FCNS have included a high-protein, high-calorie, low-salt diet; repeated

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    Also, therapy with agents reducing the glomerular filtration pressure were not successful in diminishing the proteinuria. Previously, non-Finnish CNS patients responding to ACE inhibitors and indomethacin have been published21,22. Our patient material, however, included one important exception: a child with a Fin-major mutation in one gene and a missense mutation (a change of arginine-743 to cysteine in the extracellular Ig-5 domain) in the other one.

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From the Departments of Nephrology, Pediatrics, and Pathology, Meir General Hospital, Kfar Saba, and the Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.

☆☆

Reprint requests: J. Bernheim, MD, Head, Department of Nephrology, Meir General Hospital, Kfar Saba, Israel.

0022-3476/95/$3.00 + 0 9/26/59437

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