Management of congenital nephrotic syndrome

We reviewed the medical records of seven children with congenital nephrotic syndrome (CNS) treated by unilateral nephrectomy, captopril, and indomethacin since 1990. Clinical response to the treatment was analyzed using the Students' t-test. After a median period of 54 months (range 36-88 month...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2003-05, Vol.18 (5), p.426-430
Main Authors: KOVACEVIC, Larisa, REID, Christopher J. D, RIGDEN, Susan P. A
Format: Article
Language:English
Subjects:
Angiotensin-Converting Enzyme Inhibitors - administration & dosage
Anti-Inflammatory Agents, Non-Steroidal - administration & dosage
Babies
Biological and medical sciences
Captopril - administration & dosage
Combined Modality Therapy
Drug dosages
Drug Therapy, Combination
Edema
Female
Gestational age
Glomerulonephritis
Hemodialysis
Humans
Indomethacin - administration & dosage
Infant, Newborn
Infant, Newborn, Diseases - drug therapy
Infant, Newborn, Diseases - surgery
Kidney diseases
Male
Medical sciences
Nephrectomy
Nephrology
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
Nephrotic Syndrome - congenital
Nephrotic Syndrome - drug therapy
Nephrotic Syndrome - surgery
Patients
Pediatrics
Peritoneal dialysis
Plasma
Transplants & implants
Treatment Outcome
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Summary:We reviewed the medical records of seven children with congenital nephrotic syndrome (CNS) treated by unilateral nephrectomy, captopril, and indomethacin since 1990. Clinical response to the treatment was analyzed using the Students' t-test. After a median period of 54 months (range 36-88 months) follow-up, five patients were alive at a median age of 74 (range 43-88) months. Median (range) plasma albumin rose from 11 (6-17) g/l at the start of treatment to 18 (15-22) g/l and 21 (18-25) g/l after 6 and 12 months treatment, respectively ( P=0.001 and P=0.0006). Albumin infusions per patient per month decreased from 7 (0-18) to 0 (0-30) in the 6 months post treatment ( P=0.017). The median (range) height standard deviation scores at 12 months and 30 months from onset of treatment were -1.56 (-2.96 to 0.41) and -1.43 (-2.40 to 0.90), respectively. In conclusion, management of CNS with captopril and indomethacin therapy in combination with unilateral nephrectomy achieves significant improvements in plasma albumin and reduces the need for albumin infusions and time in hospital, while growth is maintained. Second nephrectomy, dialysis, and transplantation can be delayed until the 3rd year of life or longer.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-003-1131-3