[Protein malnutrition state and deficiency state in children of Biafra].
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Biomedical subjects
Publications and source records attributed to J Goasguen.
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The authors report Kasabach-Merritt syndrome (KMS) in a patient with thrombocytopenia and splenic hemangioma. A 13-month-old boy with a history of anemia, thrombocytopenia, and abdominal mass was admitted to the hospital. The scintigraphic studies showed that a large mass contiguous to the spleen was responsible for the platelet uptake. After partial splenectomy, the platelet count returned to normal. This report of KMS in a child with splenic hemangioma suggests that the scintigraphic studies are mandatory to confirm diagnosis. Indium-111-labeled platelets are useful in identifying hemangiomatous sequestration of platelets in patients with thrombocytopenia.
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We report the case of a child presenting with abdominal Burkitt's lymphoma in whom a relapse presented as orbital and muscle involvement. This clinical feature is extremely rare. Two muscle and one orbital biopsies were necessary to obtain proper diagnosis. A new extension check-up showed bone marrow invasion and normal cerebrospinal fluid. This relapse was successfully treated by conventional chemotherapy and consolidated with high-dose chemotherapy, total body irradiation and autologous bone marrow transplantation. Eighteen months after transplantation, the child may be considered as definitively cured.
It is proposed that the vein of Galen aneurysm represents a venous ectasia secondary to an increased flow (usually caused by a deep-seated arteriovenous shunt draining either directly into the vein of Galen aneurysm or into a tributary of the vein of Galen) associated with obstruction of a dural sinus distal to the aneurysm. The closer the venous obstruction is to the vein of Galen, the better the chances are of developing obstructive (noncommunicating) hydrocephalus and the more likely it is that the venous drainage from the rest of the brain will be unaffected. The farther the venous obstruction is from the vein of Galen aneurysm, the better the chances are of developing a communicating type of hydrocephalus. The development of cardiac failure is related to the magnitude of the arteriovenous shunt. Brain damage, seizures, and hemorrhage may be related to the retrograde venous engorgement, causing impaired drainage of the healthy brain. Careful attention should be paid to the venous drainage characteristics of the lesion because the types of dural venous obstructions and anomalies vary from case to case. The term "vein of Galen aneurysm" should be abandoned in favor of the term "vein of Galen ectasia."
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