BACKGROUND AND PURPOSE:The causative gene of the common congenital malformation referred to as CHARGE syndrome is CHD7. Affected individuals often undergo head and neck imaging to assess abnormalities of the olfactory structures, hypothalamus-pituitary axis, and inner ear. We encountered a few children with severe hypoplasia of the basiocciput during a radiologic assessment of patients with CHARGE syndrome. To our knowledge, this anomaly has not been reported. Our purpose was to evaluate the incidence and severity of this anomaly in this syndrome.
Closed tendon rupture is a well-known complication of Kienböck disease, but only 11 cases have been reported. We reviewed six cases of Kienböck disease with subcutaneous rupture of a tendon. There were five cases of 4th extensor tendons and one case of the flexor digitorum profundus tendons of the ring finger and little finger. Radiographs showed protrusion of the segmented lunate or the deformed lunate in all cases. Intraoperative findings confirmed rupture of the wrist joint capsule by these lunate lesions. We then reviewed 11 reported cases in English and 48 cases in Japanese and confirmed that all cases had similar clinical characteristics and radiological features to our own, except the involved digits of flexor tendon rupture. Our case was unique in that the ulnar side tendons were ruptured, while in the reported cases the radial flexor tendons were more susceptible to rupture than the ulnar ones. Closed rupture of tendons should be recognised as a complication in patients over middle age with stage IIIb or IV asymptomatic or less symptomatic Kienböck disease.
We herein report a case of Behçet's disease with renal infarction due to mucormycosis. A 76-year-old man with entero-Behçet's disease had been treated with glucocorticoid and tumor necrosis factor (TNF) inhibitors. His entero-Behçet's disease was refractory to these treatments, and ileocecal resection was performed. After the operation, renal infarction that was unresponsive to anticoagulation therapy developed. He ultimately died of renal failure due to renal infarction. At the autopsy, histopathology of abundant hyphae in the renal vessel wall revealed mucormycosis. Renal mucormycosis is an important cause of renal failure with renal infarction in immunocompromised patients.
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