title: Congenital and infantile nephrotic syndrome in Thai infants.
abstract: Congenital and infantile nephrotic syndrome reported from the Eastern world is rare and might be a different entity from that in the West. In a retrospective review of 10 nephrotic syndrome in Thai infants (5 girls and 5 boys), 7 were diagnosed with congenital nephrotic syndrome and 3 with infantile nephrotic syndrome. Two had congenital nephrotic syndrome secondary to congenital syphilis. All had edema, ascites, and failure to thrive. Of the 3 patients tested for thyroid function, all showed hypothyroidism. Two patients developed renal failure. Renal tissue was examined from 4 patients from 3 biopsies and 2 autopsies; only 1 patient showed tubular microcysts. Symptomatic therapy was performed concurrently with penicillin therapy in 2 patients having congenital syphilis. Prednisolone, cyclophosphamide, captopril, and enalapril were tried in some patients, with little effect. Five patients died from respiratory failure complicated by later infection, 1 patient died from renal failure, and 4 patients were lost to follow-up. Nephrotic syndrome in the first year of life in the Eastern world is rare. Prognosis of nephrotic syndrome in Thai infants at this time is still poor.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat-Yai, Thailand.
authors.forename: Prayong
authors.initials: P
authors.lastname: Vachvanichsanong
authors.forename: Winyou
authors.initials: W
authors.lastname: Mitarnun
authors.forename: Kobkul
authors.initials: K
authors.lastname: Tungsinmunkong
authors.forename: Pornsak
authors.initials: P
authors.lastname: Dissaneewate
mesh_terms: D005260:Female; D006400:Hematocrit; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D008297:Male; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000175:diagnosis* / Q000628:therapy; D012189:Retrospective Studies; D013785:Thailand; D013963:Thyroid Hormones / Q000097:blood; D016896:Treatment Outcome
chemical_list: D007075:Immunoglobulin M; D013963:Thyroid Hormones