BRACHIO-OTO-RENAL SYNDROME (BOR-SYNDROME) WITH OLIGOMEGANEPHRONIA - ALONG-TERM STUDY OF RENAL-INSUFFICIENCY

The branchio-oto-renal syndrome is an inherited disorder ambining sens oneurinal hearing loss? preauricular pits, auricular abnormalities, la teral cervical fistulas, nasolacrimal duct stenosis, facial paralysis, mental retardation and renal abnormalities. Frequently the renal dise ase is oligomeganephronia, a bilateral, congenital renal abnormality w ith a reduced number of nephrons. We report on a 32-year-old male pati ent with BOR-syndrome and oligomeganephronia, whose renal insufficienc y was known since infancy but not treated for the first 10 years of hi s life. Thereafter, under conservative therapy, the renal insufficienc y showed only very slow progression. The major problem throughout the whole course was the management of severe hyperparathyroidism. After a ll, this course of events demonstrates the difficulties in predicting the prognosis of renal failure in childhood.