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[Ehlers-Danlos syndrome type VII--case report]
T Nowakowski1, B Dembińska, D Ryś
1II Katedry Chorób Wewnetrznych Collegium Medicum UJ w Krakowie.
Polskie Archiwum Medycyny Wewnetrznej
|May 22, 1998
Abstract:
We described a 30-year old man with Ehlers-Danlos syndrome type VI, manifested by marked kyphoscoliosis and severe myopia, who was admitted to our department because of chest pain. Abnormalities of stature and joint system along with eye changes, occurred starting from the birth and they aggravated gradually. Signs of increased fragility of the skin blood vessels appeared during childhood. At the age of 26 years, the patient suffered from a spontaneous rupture of the right brachial artery. Spirometry revealed marked restrictive ventilatory insufficiency, which at present constitutes the predominant health risk for the patient.