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KMID : 0387820090160010043
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Clinical Pediatric Hematology-Oncology
2009 Volume.16 No. 1 p.43 ~ p.48
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Thiamine Deficiency Manifested by Wernicke’s Encephalopathy and Wet Beriberi in a Patient with Rhabdomyosarcoma Following Chemotherapy and Total Parenteral Nutrition
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Joo Ji-Won
Baek Hee-Jo
Han Dong-Kyun
Yang Eun-Mi
Jeong Tae-Woong
Kook Hoon
Hwang Tai-Ju
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Abstract
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Beriberi and Wernicke’s encephalopathy (WE) are potentially lethal, serious diseases caused by thiamine deficiency. The most common cause is malnutrition associated with chronic alcoholism, but it may occur in such conditions as total parenteral nutrition (TPN) use, persistent vomiting and malnutrition. We report the case of WE and wet beriberi that developed following chemotherapy and TPN in a 6-year-old boy with rhabdomyosarcoma. He presented with diplopia, ptosis, personality change, disorientation, sleep disorder and hypotension. Echocardiogram showed decreased ejection fraction with pericardial effusion. The Brain MRI revealed bilateral symmetrical hypersignal intensities in the medial thalami on T2 weighted images. Serum thiamine level was 35.2 nmol/L (nl, 59∼213 nmol/L). Intravenous replacement of thiamine (300 mg/day) was started and his cardiac function improved after 5 days. Unfortunately, several spontaneous intracranial and intraventricular hemorrhage developed during severe thrombocytopenia, resulting in coma and neurologic sequelae. However, his intellectual function has completely recovered and motor has gradually improved over the last year.
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KEYWORD
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Beriberi, Wernicke’s encephalopathy, Thiamine deficiency
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