Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Endomyocardial fibrosis (EMF) is a tropical cardiomyopathy reported in many tropical countries. Patients were seen at the Children's Hospital-Khartoum, Sudan during September 2007-08 where cardiac evaluation was done. Six patients were identified (18% of all children with cardiomyopathy); all were males aged 8-17 years. All patients presented with abdominal distention, stunted growth, raised jugular venous pressure, ascites and hepatosplenomegaly. Electrocardiogram abnormalities included atrial fibrillation, first and third degree AV block and tall P wave. Echocardiography revealed huge right atrium dilatation and right ventricle apex obliteration by fibrous tissue. One patient had left-side affection with moderate mitral regurgitation. Eosinophilia was present in three patients. Two patients received antituberculosis drugs with no improvement, two had history of treated bilharziasis and two had been labeled as having Ebstein disease. EMF is an important cause of cardiomyopathy in Sudan that is often misdiagnosed. Clinical and echocardiographic examinations can lead to the correct diagnosis.
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PMID:Endomyocardial fibrosis: an under-diagnosed cause of cardiomyopathy in Sudanese children. 1926 61

We present a patient who developed carbamazepine (CBZ)-induced Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome associated with high serum procalcitonin (PCT). The presentation (high fever, hepatosplenomegaly, leukocytosis), high PCT and CRP initially suggested sepsis, and he was treated with antibiotics, while CBZ was continued. The rash and hepatitis worsened. After withdrawal of CBZ, corticosteroid therapy was administered and the patient recovered with normalization of PCT. This case demonstrates that PCT may be increased in patients with DRESS. This is the first report of CBZ-induced DRESS associated with high PCT, and the second case of increased PCT in DRESS.
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PMID:High procalcitonin in a patient with drug hypersensitivity syndrome. 1968 1

DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is a potentially life-threatening syndrome, which reflects a serious hypersensitivity reaction to drugs, presenting by generalized skin rash, fever, eosinophilia, atypical lymphocytosis, and internal organ involvement. Herein a 21-month old male infant with DRESS and Encephalopathy syndrome is presented who complicated after phenobarbital usage that persisted due to phenytoin cream usage. The case received phenobarbital after a seizure disorder presented as "status epilepticus". He developed drug eruption, fever, hepatosplenomegaly, increased liver enzymes, encephalopathy and progressive loss of consciousness with extensive hyperintense white matter lesions in brain MRI. After discontinuation of phenobarbital and phenytoin, all symptoms were resolved, while brain MRI became normal after two months. To our best knowledge, this is the first reported case that developed leukoencephalopathy along with DRESS syndrome.
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PMID:DRESS Syndrome Presents as Leukoencephalopathy. 2741 28