Successful treatment of Finnish congenital nephrotic syndrome with captopril and indomethacin☆,☆☆,★
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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Cited by (33)
Hereditary Nephrotic Syndrome
2008, Comprehensive Pediatric Nephrology: Text with CD-ROMHereditary Nephrotic Syndrome
2008, Comprehensive Pediatric NephrologyEvaluation of therapeutic strategies in congenital nephrotic syndrome of the Finnish type
2006, Anales de PediatriaCongenital and infantile nephrotic syndrome
2005, Nephrologie et TherapeutiqueCongenital and infantile nephrotic syndrome
2004, EMC - PediatrieCongenital nephrotic syndrome (NPHS1): Features resulting from different mutations in Finnish patients
2000, Kidney InternationalCitation Excerpt :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.
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Reprint requests: J. Bernheim, MD, Head, Department of Nephrology, Meir General Hospital, Kfar Saba, Israel.
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