[Connective tissue pathology in patients with aneurysms of the cerebral arteries (Ehlers-Danlos syndrome)].
Results of morphological examination of the skin and vessels in patients with large and giant brain artery aneurysms are presented. Ehlers-Danlos syndrome (type IV) was diagnosed in half of the cases; very thin skin and vessel walls, the disturbance of the collagen fibril structure in dermis, increased permeability and the tendency to rupture of the inner elastic membrane of brain arteries combined with a severe intimal atrophy. The absence of the III type collagen in the skin biopsies was shown immunohistochemically. The feasibility of the morphological diagnosis of this syndrome by skin biopsies and the significance of such diagnosis for prevention of complications in a surgical treatment are demonstrated.
Shishkina
L V
LV
Lazarev
V A
VA
Meshcheriakova
A V
AV
Printseva
O Iu
OIu
D000293:Adolescent; D000328:Adult; D002536:Cerebral Arteries / Q000473:pathology*; D003094:Collagen / Q000648:ultrastructure; D003238:Connective Tissue / Q000473:pathology*; D003937:Diagnosis, Differential; D004535:Ehlers-Danlos Syndrome / Q000473:pathology*; D004730:Endothelium, Vascular / Q000473:pathology; D005260:Female; D006801:Humans; D002532:Intracranial Aneurysm / Q000473:pathology*; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology; D008875:Middle Aged; D010012:Osteogenesis / Q000502:physiology; D012867:Skin / Q000473:pathology
D003094:Collagen
Patologiia soedinitel'noĭ tkani u bol'nykh s anevrizmami arteriĭ golovnogo mozga (sindrom Elersa-Danlo).