Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Aortic pseudo-aneurysm is a rare manifestation of Takayasu arteritis. We present a 16-year-old girl who first complained of multiple arthritis, recurrent abdominal pain and malaise at the age of 15 years. The initial working diagnosis was juvenile rheumatoid arthritis. Follow-up abdominal ultrasonography for her hepatomegaly incidentally revealed an aortic aneurysm. Total aortography showed diffuse aortic narrowing and an infra-renal aortic pseudo-aneurysm. Vascular reconstruction with an interposition Dacron graft was performed with uneventful recovery. Early non-specific presentation of Takayasu arteritis often results in delay of diagnosis. The presence of a vascular bruit in a young female with non-specific symptoms should point to a differential diagnosis of Takayasu arteritis. We review the role of surgery in the management of this condition.
 ...
PMID:Takayasu arteritis--a case report of aortic aneurysm. 1006 89

Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn's disease, Prader-Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.
 ...
PMID:Takayasu arteritis in childhood: misdiagnoses at disease onset and associated diseases. 2968 55

Takayasu's arteritis, formerly known as "pulseless disease", is a chronic inflammatory disease which affects the aorta and its main branches. The prevalence of this disease is higher in Asian countries and among young women. Depending on the progress of the disease, the symptoms and prognosis of the disease is different. Herein, we report the case of a 31-year-old woman with Takayasu's arteritis in the Iranian population. In this case, the size of the aorta and the main branches increased and the large artery walls were thickened. Narrowing of the right and left subclavian artery, carotid artery and left vertebral artery were obvious in these patients. In addition, an anatomic variation was observed in the aortic arch branches. In this patient, the left vertebral artery was branched directly from the aortic arch instead of the left subclavian artery. Hepatomegaly and splenomegaly, indicating a chronic inflammatory disease, were also observed in this case.
 ...
PMID:A Rare Case of Takayasu's Arteritis with Aortic Arch Branching Pattern Variation in a Young Female. 3112 82