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)

We describe an 18-year-old white male who developed lower extremity ischemia requiring amputation. He presented at 14 with pulmonary infiltrates, hepatosplenomegaly, fever, rash, adenopathy, uveitis, and arthralgias; clinical and laboratory findings were consistent with Mycoplasma pneumoniae infection. Despite adequate treatment with antibiotics, he developed chronic arthralgias and fevers, with rash and pericardial effusion. Criteria for the diagnosis of systemic lupus erythematosus were not met; juvenile rheumatoid arthritis was diagnosed presumptively. Over the subsequent 4 years he developed lymphadenopathy with biopsy-proven nonnecrotizing granulomas, chronic leg ulceration with granulomatous histology, and acute-onset impending gangrene of the left foot. A biopsy of the posterior tibial artery demonstrated giant cell arteritis. Although the histologic features were consistent with Takayasu's arteritis, complete aortic arteriography was normal. Examination of the amputated leg showed multifocal segmental giant cell arteritis. Clinicopathologic features suggested, but were not fully consistent with, juvenile systemic granulomatosis. His disease may represent a separate sarcoid-like entity in the broad spectrum of vasculitis.
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PMID:Giant cell vasculitis with extravascular granulomas in an adolescent. 205 10

A 17-year-old male had fever of unknown origin (FUO) for 3 months with positive c-reactive protein, lymph node swelling, and hepatosplenomegaly. Biopsy specimens of the liver and lymph node disclosed nonspecific inflammation. Lymph node swelling and hepatosplenomegaly subsided gradually, while vascular murmur and pulselessness appeared. Computerized tomography and magnetic resonance imaging showed thickened arterial wall, while angiography disclosed arterial narrowing. From these findings he was diagnosed to have Takayasu's arteritis. The therapy with prednisolone was effective. Takayasu's arteritis is rarely manifested by hepatosplenomegaly and lymphadenopathy in its prepulseless stage.
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PMID:Takayasu's arteritis in prepulseless stage manifesting lymph node swelling and hepatosplenomegaly. 764 21

Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches and pulmonary arteries. Diagnosis of TA during childhood remains challenging due to the non-specific symptoms. We report a four-year-old girl presenting with fever, fatigue, weight loss, and elbow pain who was later diagnosed as childhood TA. On admission, she had fever, hypertension, decreased pulses, bruits, hepatosplenomegaly, and increased erythrocyte sedimentation rate and C-reactive protein level. Computed tomography angiography showed luminal narrowing and wall thickening in ascending aorta, brachiocephalic, left common carotid and left vertebral arteries and descending aorta. Oral corticosteroid (prednisone, 2 mg/kg/day) was instituted, later followed by oral methotrexate (12.5 mg/m2/week). TA is rare in children; however, childhood TA must be considered in children who present with non-specific systemic symptoms, hypertension and increased acute phase reactants.
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PMID:Takayasu arteritis in a 4-year-old girl: case report and brief overview of the pediatric literature. 2342 21

Takayasu arteritis (TA) is the third most common childhood vasculitis and its clinical manifestations depend on the arteries involved. We report a case of a 9-year-old boy with multiple aneurysms in carotid and iliac arteries, subclavian and coronary arteries, and abdominal aorta. At the age of 7 years, he presented with recurrent fever and hepatosplenomegaly. An angio-computed tomography scan showed aneurysms in the left subclavian artery, abdominal aorta, and both proximal iliac arteries. He was diagnosed with TA and was treated with corticosteroids, aspirin, and enalapril. One year later, he was admitted to Dr Roberto Gilbert Children's Hospital because of intracranial hemorrhage. Angiography revealed enlargement of aneurysms enlargement and new aneurysms. He also developed portal hypertension. Treatment with intravenous corticosteroids, azathioprine, and monthly intravenous cyclophosphamide was begun. After 6 months of no improvement, infliximab was begun. The aim of this article was to report the concurrence of coronary involvement and portal vein hypertension in pediatric TA because there were scarce reports on this matter.
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PMID:Portal hypertension: an uncommon clinical manifestation of Takayasu arteritis in a 9-year-old child. 2789 19