Denys-Drash syndrome (DDS).
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Biomedical subjects
Publications and source records attributed to Ahmad I Alomari.
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This brief report describes an unusual complication of femoral venous access placement. A 17-year-old male underwent cardiopulmonary transplantation for complex congenital heart disease, which was complicated by thrombotic thrombocytopenic purpura, quadrilateral limb gangrene, and stroke. In an effort to urgently obtain a venous access, a right femoral venous catheter was placed into the right groin in the cardiac care unit. The patient subsequently experienced severe hypotension and abdominal distension. Ultrasound and CT scans of the abdomen demonstrated a large retroperitoneal hematoma. Aggressive resuscitation failed to stabilize the patient. Angiography demonstrated an unusual bleeding source from an arterial collateral circulation in the right side of the pelvis, which was then successfully treated with embolization.
In four cases of hepatic epithelioid hemangioendothelioma (HEHE), multiple liver lesions were depicted on enhanced computed tomography (CT) and/or magnetic resonance imaging (MRI) scans. A peculiar sign, a hepatic or portal vein terminating at or just within the periphery of some of the liver lesions, was noted in all four; the images look like a lollipop. Imaging findings of HEHE can suggest other diseases, but this sign buttressed by other radiological findings, seems to be specific for this entity.
PURPOSE: To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients. MATERIALS AND METHODS: A 13-item survey questionnaire was sent to 74 patients who had undergone at least one sclerotherapy procedure in our hospital from January 1997 through January 2003. Information regarding the anatomic location, specific symptoms reported, history, treatment satisfaction, postprocedural complications, and number of treatment sessions was elicited. Four sclerosing agents (as single agents or in combination with other agents) were used: ethanol, sodium tetradecyl sulfate 3% (STS), OK-432, and doxycycline. RESULTS: Fifty-five patients or their caregivers completed the survey. The patients' ages ranged from 6 months to 48 years at the time of the first procedure (mean, 12 y; median, 4 y). A majority of LMs were located in the cervicofacial region. The size and location of the lesion, recurrent infection, and pain were the most frequent indications for treatment. Fifty-one percent of these patients received sclerotherapy alone or in conjunction with surgery as primary treatment. Ethanol was the most common sclerosing agent used, followed by doxycycline, STS, and OK-432. Response varied with the type of LM, with 100%, 86%, and 43% of the patients reporting good to complete response for macrocystic, microcystic, and combined-type LMs, respectively. Skin blistering and ulcers were the most common complications. Permanent complications were uncommon and were largely related to ethanol use. CONCLUSIONS: Percutaneous sclerotherapy provides effective midterm primary treatment for LMs. Treatment outcomes appear to vary according to the morphology of the malformation.