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[Investigation of multiple organ involvement in Fabry disease]
Wei Zhang1, Mande Kang1, Yawen Zhao1
1Department of Neurology, Peking University First Hospital, Beijing 100034, China.
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.
Conclusion:
The incidence and onset of multiple organ dysfunction in Chinese patients with Fabry disease displayed special regularity. Gender difference appeared only in renal disease.
