Cases reported "Splenomegaly"

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1/6. vitamin a toxicity secondary to excessive intake of yellow-green vegetables, liver and laver.

    We report a case of sudden onset of vitamin a poisoning. A 20-year-old Japanese woman had been eating pumpkin and only a very limited amount of other foods on a daily basis for 2 years. She was overly concerned about weight reduction. Aurantiasis cutis and abnormal liver function tests were noted by her family doctor in 1995 when she was 18 years old. At that time, she stopped eating pumpkin. However, she secretly continued an excessive intake of other beta-carotene-rich vegetables, liver and laver for about 2 years. Two and one-half years after being seen by her family physician, she experienced sudden onset of low-grade fever, limb edema, cheilitis, dry skin, and headache. These symptoms worsened daily. A liver needle biopsy was performed, and it showed a normal portal tract along with fat-laden Ito cells in the space of Disse. A final diagnosis of vitamin a poisoning and hepatic injury secondary to an eating disorder was made. Her symptoms and serum beta-carotene levels returned to normal with successful adjustment of her diet.
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2/6. Improvement of splenomegaly and pancytopenia by enzyme replacement therapy against type 1 gaucher disease: a report of sibling cases.

    gaucher disease is a genetic lipid storage disease and represents a potentially serious health problem. It arises from a deficiency of glucocerebrosidase activity with secondary accumulation of large quantities of glucocerebroside. Symptoms are usually multisystemic, often debilitating or disabling, and sometimes disfiguring, and they can lead to death. We report objective clinical response's to repeated infusion of human placental and recombinant glucocerebrosidase in 2 patients with type 1 gaucher disease and increased hemoglobin levels and platelet counts. Splenic volume decreased during the period of enzyme administration. enzyme replacement therapy has improved the treatment of type 1 gaucher disease by safely and effectively arresting, decreasing, or normalizing many of its major signs and symptoms. Consideration by physicians must be given to gaucher disease, and appropriate treatments must be given when confronted with cryptogenic pancytopenia or hepatosplenomegaly.
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3/6. Nonoperative management of spontaneous splenic rupture in infectious mononucleosis: the role for emerging diagnostic and treatment modalities.

    infectious mononucleosis (IM) is a self-limiting lymphoproliferative disorder affecting teenagers and young adults. splenomegaly is a common manifestation of IM and results in a compromised organ that may rarely rupture spontaneously, with significant morbidity and mortality. The IM spleen should be protected from even minor trauma. Although traditional management of spontaneous splenic rupture in IM has been splenectomy, the role of nonoperative management is evolving. The advent of endovascular interventional modalities has augmented the physician's armamentarium in managing these patients nonoperatively. We report a case of spontaneous splenic rupture in a patient with IM managed conservatively with the aid of splenic angiography. The option of arteriography, with or without embolization, should be considered in the management of all patients with spontaneous splenic rupture in the setting of IM.
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4/6. An unusual presacral mass: extramedullary hematopoiesis.

    Presacral masses are a rare finding in the adult patient, confronting the physician with diagnostic and therapeutic challenges. We present an unusual case of a symptomatic presacral mass caused by extramedullary hematopoietic tissue in a thalassemic patient and review the unique aspects of this entity.
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5/6. Evidence of hyposplenism in the presence of splenomegaly.

    Evidence of hyposplenism (Howell-Jolly bodies, Pappenheimer bodies and nucleated red cells in the blood) was found in a patient with splenomegaly and Hb C disease. Splenic haemangiosarcoma was found at operation. Evidence of hyposplenism in the presence of a normal size or enlarged spleen should alert the physician to the possibility of a complicating factor.
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6/6. The limping child: a manifestation of acute leukemia.

    Nine patients who presented to our institution with the chief complaint of a limp and no history of trauma were subsequently diagnosed with leukemia. A review of these patients identified clinical and laboratory findings that helped to establish the diagnosis. The presence of an antalgic gait with complaints of pain of variable intensity and duration, an irritable hip or knee, a mild to moderate elevation in body temperature, lymphadenopathy, hepatosplenomegaly, an increased erythrocyte sedimentation rate, thrombocytopenia, anemia, decreased neutrophils, increased lymphocytes, or blast cells on the peripheral blood smear should cause the physician to suspect leukemia in a limping child. bone marrow biopsy confirms the diagnosis.
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