title: [Investigation of multiple organ involvement in Fabry disease].
abstract: OBJECTIVE
  To investigate incidence and clinical features of multiple organ involvement in Chinese patients with Fabry disease.
  
  
  METHODS
  We collected 151 patients of 31 families with Fabry disease, all of whom were confirmed by classic pathology, decreased α-galactosidase A activity or GLA mutation from the year of 2011 to 2014 in Department of Neurology, Peking University First Hospital. The clinical data included incidence and onset of neuralgia, renal dysfunction, heart disease, hypertension and cerebral stroke. We calculate the incidence of these symptoms and analyze their gender difference using chi-square with SPSS 17.0 software.
  
  
  RESULTS
  Among 31 families with Fabry disease, 151 patients (82 males and 69 females) were affected, including 91 patients (60.26%) with neuralgia, 62 patients (41.06%) with renal disease, 33 patients (21.85%) with heart disease, 33 patients (21.85%) with hypertension, and 21 patients (13.91%) with cerebral stroke. The peak incidence of neuralgia was from 10 to 20 years old, followed by renal disease from 30 to 40 years old, heart disease and stroke from 40 to 50 years old. There is no peak period for hypertension. Except for males with higher incidence of renal dysfunction than females (P < 0.05), there were no gender difference in the incidence of other organ involvement.
  
  
  CONCLUSIONS
  The incidence and onset of multiple organ dysfunction in Chinese patients with Fabry disease displayed special regularity. Gender difference appeared only in renal disease.
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Mande
authors.initials: M
authors.lastname: Kang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Yawen
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Fan
authors.initials: F
authors.lastname: Li
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Junlong
authors.initials: J
authors.lastname: Shu
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Yuehuan
authors.initials: Y
authors.lastname: Zuo
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Liu
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China.
authors.forename: Yining
authors.initials: Y
authors.lastname: Huang
authors.affiliation: Department of Neurology, Peking University First Hospital, Beijing 100034, China; Email: yuanyun2002@sohu.com.
authors.forename: Yun
authors.initials: Y
authors.lastname: Yuan
mesh_terms: D044466:Asian People; D000795:Fabry Disease; D005260:Female; D006331:Heart Diseases; D006801:Humans; D006973:Hypertension; D015994:Incidence; D007674:Kidney Diseases; D008297:Male; D009154:Mutation; D009437:Neuralgia; D012727:Sex Characteristics; D020521:Stroke; D000519:alpha-Galactosidase
chemical_list: D000519:alpha-Galactosidase