✍ Scribed by Weymann, Sibylla ;Yonekawa, Yasuhiro ;Khan, Nadia ;Martin, Ernst ;Heppner, Frank L. ;Schinzel, Albert ;Kotzot, Dieter
No coin nor oath required. For personal study only.
We report on a 15-year-old boy with stenosis and occlusion of multiple cranial, renal, and celiac arteries, aneurysm of the basilar artery, bilateral cutaneous syndactyly between ®ngers IV-V, partial cutaneous syndactyly between ®ngers III-IV on the right hand, brachydactyly, and borderline mental retardation. The clinical course was characterized by recurrent abdominal pain, gastritis, and high blood pressure. The pattern of the clinical and radiological ®ndings is different from ®bromuscular dysplasia (FMD) and Moyamoya disease, and highly suggestive of a syndrome described by Grange in four siblings (MIM#602531) Grange et al. [1998: Am J Med Genet 75: 469±480].
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## Objective: The pathophysiology and symptomatology of the superior mesenteric artery syndrome (SMA syndrome) is discussed. Method: A review of much of the available literature concerning the SMA syndrome and the associated condition acute gastric dilatation is offered. Results: Two new cases of