Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0003090 (arthrodesis)
 8,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED) is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate.
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PMID:Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation. 2568 24

Hemophilia is a disorder of blood coagulation with X-linked recessive inheritance. It is characterized by uncontrollable hemorrhage, and 80% of these occur intraarticularly. With recurrent hemarthrosis, recurrent synovitis occurs, which eventually leads to the formation of articular contractures. The key to the prevention of hemophilic joint complications is successful prevention of bleeding and management of the initial hemarthrosis. However, after the development of a rigid contracture, surgical correction remains the only method to correct the deformity. Achilles tendon lengthening, synovectomy, anterior osteophyte resection, corrective osteotomies, external fixators, or arthrodesis should be considered as surgical options. In the present report, we describe our experience using hybrid-type external fixators to manage bilateral neglected rigid equinus contractures in a hemophilic patient, with 78 months of follow-up data.
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PMID:Surgical Treatment of Neglected Bilateral Hemophilic Equinus Contracture With External Fixation: A Case Report. 2947 70

Hemophilia is an X-linked recessive inherited coagulation disorder. We report the anesthesia management of a hemophilia patient who underwent arthroscopic ankle arthrodesis after living-donor liver transplantation due to cirrhosis. The 35-year-old male patient with hemophilia B was diagnosed with cirrhosis due to hepatitis C virus at the age of 23 years and underwent biologically-related partial liver transplantation at the age of 29 years. As a result, the activity of factor IX activity became normal and blood product treatment became unnecessary, but the patient required long-term immunosuppression. Perioperative coagulation factor activity monitoring was performed and an immunosuppressive drug that had been preoperatively administered were continued. General anesthesia was administered by inhalation. There was no significant fluctuation in perioperative factor IX activity. This case illustrates that even in patients with hemophilia B after living-donor liver transplantation undergoing an orthopedic surgical procedure, anesthesia management can safely be performed without perioperative coagulation factor replacement.
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PMID:Anesthesia management of arthroscopic ankle arthrodesis for a hemophilia patient after living-donor liver transplantation. 3088 60