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Osteosynthetic reconstruction in a patient with Gardner-Diamond syndrome.

Based on clinical observations in one female patient who suffered from Gardner-Diamond syndrome, we noticed that surgery can be performed without any bleeding complications, although operations normally are contraindicated in persons with this condition. Additionally, a correlation seems to exist between ASS and sexual and hormonal maturity.

Autoimmune Diseases↗

Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma.

Children with a large vascular tumor and associated Kasabach-Merritt coagulopathy respond inconsistently to therapy and have a high mortality rate. For this reason, we undertook a retrospective study of 21 such patients, and focused on clinical, radiographic, and histopathologic features. The male to female ratio was 1:1.6. Tumor was noted at birth in 50 percent of patients; the remainder appeared throughout infancy. The location was cervicofacial (n = 2), shoulder/upper limb (n = 4), trunk including retroperitoneum (n = 11), and lower limb (n = 4). These tumors grew rapidly to large size and were characterized by cutaneous purpura, edema, and an advancing ecchymotic margin. In contrast to common hemangioma, magnetic resonance imaging showed diffuse enhancement with ill-defined margins, cutaneous thickening, stranding of subcutaneous fat, hemosiderin deposits, and small feeding and draining vessels. All tumors were Kaposiform hemangioendothelioma (KHE); none were infantile hemangioma. Light microscopy showed irregular lobules or sheets of poorly formed, small vascular channels infiltrating and entrapping normal tissues. Characteristic features included spindle-shaped endothelial cells, diminished pericytes and mast cells, microthrombi, and hemosiderin deposits. Wide endothelial intercellular gaps and incomplete basement membranes were seen by electron microscopy. Dilated, hyperplastic, lymphaticoid channels were prominent in one tumor. KHE in 14 infants was treated with interferon alpha-2a: 6 had accelerated regression; 2 had stabilization of growth; and 6 evidenced no response. The mortality rate was 24 percent (5 of 21); this included three infants with retroperitoneal KHE. Kasabach-Merritt phenomenon does not occur with common hemangioma. Rather it is associated with the more aggressive KHE and rarely with other vascular neoplasms. Variable response to current pharmacologic therapy underscores our inadequate knowledge of the pathogenesis of thrombocytopenia in KHE.

Adipose Tissue↗

Bicycle spoke injuries in the lower extremity.

This study reports the authors' 5-year experience with treating lower extremity injuries in bicycle passengers caused by the spokes. This patient group was selected from 716 lower extremity injuries that received treatment at our outpatient plastic surgery clinic. A total of 26 patients were treated during the study. Patients ranged from 2 to 19 years old, with a mean age of 5.6 years. The authors treated more female passengers (62 percent) than male passengers. The right foot (52 percent) was involved more often than was the left. Most patients were injured in the afternoon, from 2 to 7 PM (62 percent), and between May and October (77 percent). The rear wheel (89 percent) injured the majority of patients. The Achilles tendon was the most common site of injury (63 percent). The typical types of wounds observed included the following: type I, laceration with partial avulsion of skin and subcutaneous tissue (41 percent) and laceration forming a distally based flap (33 percent); type II, abrasions with ecchymoses and friction burn from the shearing effect of the spokes creating a partial- to full-thickness skin defect (26 percent). Of the type I injuries, full-thickness skin lacerations (33 percent) were closed primarily. Partial-thickness skin lacerations, abrasions, ecchymoses, and skin defects (67 percent) were treated conservatively with wound irrigation and dressing. The wound healing time for type I injuries was 29 days; for type II injuries, it was 27 days. These healing times were prolonged compared with healing by first intention. No significant difference was found in healing time when comparing both types of injury. Four patients required hospitalization. No patient required skin grafting. No fractures were noted because these patients were selected from the outpatient plastic surgery clinic and did not include patients from the emergency room. Since the first report of bicycle spoke injuries a half-century ago, prevention has not improved.

Achilles Tendon↗

Clinically significant hemorrhage due to warfarin-carbamazepine interaction.

A 61-year-old woman receiving warfarin for postoperative atrial fibrillation and carbamazepine for a peripheral neuropathy had extreme elevation of prothrombin time associated with clinically significant hemorrhage when the carbamazepine therapy was discontinued. The mechanism of this significant drug interaction involves the withdrawal of carbamazepine-induced enhancement of hepatic warfarin breakdown with resultant delay of warfarin excretion and excessive hypoprothrombinemia. The clinically significant hemorrhage associated with warfarin-carbamazepine interaction is an indication for caution when these two agents are prescribed concomitantly.

Carbamazepine↗

Rodenticide-induced coagulopathy in a young child. A case of Munchausen syndrome by proxy.

PURPOSE: To present the diagnosis and management of superwarfarin ingestion, a cause of serious and prolonged coagulopathy. METHODS: Specific identification of the anticoagulant was made by high-pressure liquid chromatography. RESULTS: A 24 month-old child developed bruises and a prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT) after receiving multiple doses of brodifacoum, a superwarfarin rodenticide. The coagulopathy was treated successfully with large doses of parenteral and oral vitamin K1; fresh frozen plasma was administered as a precautionary measure on two occasions. After the first 10 days of the child's hospitalization, the mother was identified as the source of brodifacoum, exemplifying the behavior described as Munchausen syndrome by proxy. Oral vitamin K1 was initiated and continued in an outpatient setting with tapering doses over nine months, using the PT as a guide for therapy. CONCLUSIONS: This report emphasizes the necessity of recognizing rodenticide poisoning and investigating its source. Frequent monitoring of the PT is essential to prevent hemorrhagic complications due to repeat exposure, inadequate vitamin K1 therapy, or noncompliance.

4-Hydroxycoumarins↗