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Biomedical subjects

B T Steele

Publications and source records attributed to B T Steele.

24 records · Page 2Linked to original sources

Acute renal failure of short duration in minimal lesion nephrotic syndrome of childhood.

Two boys 12 and 8 years of age with biopsy proven minimal lesion nephrotic syndrome developed acute renal failure during the course of their illness. Renal failure developed in the first boy at the time of onset of his nephrotic syndrome while in the second boy it occurred at the time of a relapse of his disease, 5 1/2 years after onset. Both patients had a complete resolution of renal failure and nephrosis following peritoneal dialysis and oral prednisone. Acute renal failure requiring dialysis is an unexplained and rare complication of minimal lesion nephrotic syndrome in adults and children.

Acute Kidney Injury↗

Nephronophthisis.

Twenty-one patients with nephronophthisis are described with a follow-up of one to 16 years (mean 9.3 years). In 10 patients, there was a familial incidence. Autosomal recessive appears the likely mode of inheritance with a 20 per cent incidence noted (seven of 35) following correction for the bias of ascertainment by removing the probands. Seven patients had an associated and characteristic retinal degeneration from infancy. Associated neurologic problems, including mental retardation, seizures and cerebellar ataxis, were also seen in some patients. Previously described skeletal abnormalities and hepatic fibrosis were not seen in any of our patients. All presented at an advanced stage of chronic renal failure, usually associated with a history of polydipsia and polyuria from infancy. Renal cysts were noted in only one of the nine patients in whom tissue was obtained by needle biopsy. In seven patients in whom tissue was available at nephrectomy or autopsy, cysts were noted in six although only in two were they localized to the medulla. Eighteen patients have undergone dialysis, and 12 patients have received a renal transplant with no evidence of recurrence of the original disease. Sixteen patients are still alive. Many synonyms for nephronophthisis have appeared, with medullary cystic disease being the most common. Our experience suggests that nephronophthisis is a common cause of chronic renal failure and has commonly associated nonrenal abnormalities.

Adolescent↗