[Chronic hereditary nephritis with hearing loss (Alport's syndrome)].

Przewlekłe dziedziczne zapalenie nerek z upośledzeniem słuchu (zespoł Alporta).

Publisher: Copernicus International Country of Publication: Poland NLM ID: 0404453 Publication Model: Print Cited Medium: Print ISSN: 0030-6657 (Print) Linking ISSN: 00306657 NLM ISO Abbreviation: Otolaryngol Pol Subsets: MEDLINE

Publication: 2015- : Poland : Copernicus International
Original Publication: Warszawa, Lekarski Instytut Naukowo-Wydawniczy.

Hearing Loss/*genetics ; Hearing Loss, Bilateral/*genetics ; Nephritis, Hereditary/*genetics; Adult ; Chronic Disease ; Female ; Hearing Loss, Bilateral/complications ; Hearing Loss, Bilateral/diagnosis ; Humans ; Male ; Middle Aged ; Nephritis, Hereditary/complications ; Nephritis, Hereditary/diagnosis ; Pedigree

Two generations of one family with Alport syndrome were studied. The observation proved that women do transmit the disease, but men have signs more developed. As symptoms of the disease were pathological changes of chronic interstitial haemorrhagic pyelonephritis associated with bilateral hearing loss of cochlear type. In patients with pronounced hearing impairment (in the spiral organ) the kidney pathology was not advanced and the course of the illness rather mild. We believe that patients with kidney pathology should have their hearing examined as routine proceeding because hearing impairment is frequently associated with kidney pathology as in Alport's syndrome and other diseases. This coincidence may help in diagnosis, choice of treatment and prognostic evaluation.

Date Created: 19890101 Date Completed: 19900905 Latest Revision: 20151029

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