title: Management of congenital nephrotic syndrome.
abstract: 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.
authors.affiliation: Department of Pediatric Nephrology, Children's Hospital of Michigan, 3901 Beaubien Boulevard, Detroit, MI 48201, USA. k_larissa@yahoo.com
authors.forename: Larisa
authors.initials: L
authors.lastname: Kovacevic
authors.forename: Christopher J D
authors.initials: CJ
authors.lastname: Reid
authors.forename: Susan P A
authors.initials: SP
authors.lastname: Rigden
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000008:administration & dosage*; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D002216:Captopril / Q000008:administration & dosage*; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007213:Indomethacin / Q000008:administration & dosage; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000188:drug therapy* / Q000601:surgery*; D008297:Male; D009392:Nephrectomy; D009404:Nephrotic Syndrome / Q000151:congenital / Q000188:drug therapy* / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000894:Anti-Inflammatory Agents, Non-Steroidal; D002216:Captopril; D007213:Indomethacin