Abstract:
:A 5-year-old Japanese boy showed nephritis similar to, but distinct from, that in Alport syndrome. Nephrotic syndrome without hematuria was noticed at age 2, although renal biopsy at age 4 revealed widespread irregular thickening of the glomerular basement membrane with splitting of the lamina densa on electron microscopy, characteristic of nephritis in Alport syndrome. Sensorineural deafness was noticed at age 4 weeks by no auditory brain stem response, unusually early for Alport syndrome. Goodpasture antigen and amyloid P component were found in the glomerular basement membrane. Thus, the antigenicity of the glomerular basement membrane was different from that in male patients with X-linked Alport syndrome. In addition, growth and developmental retardation, hyperkinesis, and cleft soft palate were seen. These features are a hitherto undescribed combination. The family history was negative for any of the features of the boy.
journal_name
Nephronjournal_title
Nephronauthors
Kawakami H,Murakami T,Murano I,Ushijima T,Taguchi T,Hattori S,Kajii Tdoi
10.1159/000186136subject
Has Abstractpub_date
1990-01-01 00:00:00pages
214-7issue
2eissn
1660-8151issn
2235-3186journal_volume
56pub_type
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