Clinico-pathological features in four patients with Fabry's disease The possible role of degenerative lesions in the interstitial vessels for renal dysfunction

Mitsunobu Matsubara, Yoshio Taguma, Takao Saito, Kaoru Yoshinaga, Takashi Furuyama, Hiroshi Sakaguchi

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2 Citations (Scopus)

Abstract

Clinical courses and histopathological changes of four patients with Fabry's disease were discussed in this report. All of them were male and revealed to have angiokeratomas of the skin and markedly decreased activity of α-galactosidase -A in leukocytes. They were from different families. No consanguinity was noted. Two of them, around 40 years of age, showed rapid impairment of renal function with simultaneously development of hypertension, progressing to uremia within several months. Their light and electron microscopic findings in renal biopsies were similar: severe degenerative changes in muscle cells of small arteries and arterioles with remarkable swelling of intima, and multiple sclerosing and collaptic lesions in glomeruli. The other 2 patients were about 20 years old and their renal functions were almost normal. No significant changes but fine vacuolization of epithelial cells of glomeruli were observed in renal biopsy specimens. It is concluded that marked degenerative changes in the vessels due to Fabry's disease might be the principal etiology of hypertension and rapid deterioration of renal function.

Original languageEnglish
Pages (from-to)237-243
Number of pages7
JournalThe Japanese Journal Of Nephrology
Volume32
Issue number2
DOIs
Publication statusPublished - 1990 Jan

Keywords

  • Fabry's
  • degenerative lesions in vessels
  • disease
  • renal dysfunction

ASJC Scopus subject areas

  • Nephrology

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