Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0162871 (abdominal aortic aneurysm)
 8,664 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Childhood sarcoidosis is a rare disease with multisystemic organ involvement. A wide spectrum of vasculitides, including large vessel involvement, have been reported in childhood sarcoidosis. We describe a 7-year-old boy with childhood sarcoidosis who presented with prolonged fever, iritis, and abdominal aortic aneurysm. He underwent successful surgical repair of the abdominal aortic aneurysm and followup for 12 months was uneventful. This is first report of abdominal aortic aneurysm complicating childhood sarcoidosis.
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PMID:Abdominal aortic aneurysm associated with childhood sarcoidosis. 873 Jan 40

AA amyloidosis is a relatively rare disease which complicates chronic inflammatory diseases, chronic infections, familial Mediterranean fever (FMF) and malignant diseases. Although amyloid deposition may be found in many organs, renal involvement dominates the clinical picture. We reviewed 63 patients with AA amyloidosis who presented to our nephrology department between 1995 and 2000. Prognostic markers, detailed history, physical examination and laboratory tests were evaluated. The causes of AA amyloidosis were as follows: FMF 42 (66.6%), pulmonary tuberculosis 9 (14.2%), chronic osteomyelitis 4 (6.3%), bronchiectasia 4 (6.3%), rheumatoid arthritis 1 (1.5%), juvenile idiopathic arthritis 1 (1.5%), inflammatory abdominal aortic aneurysm 1 (1.5 %), unknown aetiology 1 (1.5%). The diagnosis was made on renal biopsies in 63.4% of the patients, while the remaining 36.6% were diagnosed as a result of rectal biopsies. Sixteen patients died. A low serum albumin, high creatinine and high 24-hour urine albumin excretion were associated with high mortality.
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PMID:Clinical and biochemical outcome of renal amyloidosis. 1213 41

Superior mesenteric artery (SMA) syndrome is a rare disease in which the third portion of the duodenum is compressed by SMA. There are many causes leading to the SMA syndrome, however it's extremely rare that aortic aneurysm causes a SMA syndrome. We report a case of a successfully treated SMA syndrome due to an abdominal aortic aneurysm in a renal transplant recipient. The patient was a 52-yr-old woman with a thin stature (weight 40 kg, height 164 cm). She received a renal transplant 8 yr before, and had hypertension and abdominal aortic aneurysm. Her SMA syndrome developed in a prolonged supine position for the accidental rib fractures and was diagnosed by clinical and radiological findings. After a surgical correction (resection of an aneurysm and aortobiiliac bypass with an inverted Y graft), her symptoms relieved without deterioration of the graft function.
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PMID:Superior mesenteric artery syndrome due to an aortic aneurysm in a renal transplant recipient. 1217 55

Frontotemporal dementia with inclusion body myopathy and Paget's disease of bone (IBMPFD) is a rare, autosomal dominant disorder caused by mutations in the gene valosin-containing protein (VCP). The CNS pathology is characterized by a novel pattern of ubiquitin pathology distinct from sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive inclusions without VCP mutations. Yet, the ubiquitin-positive inclusions in IBMPFD also stain for TAR DNA binding protein, a feature that links this rare disease with the pathology associated with the majority of sporadic FTD as well as disease resulting from different genetic alterations. VCP, a member of the AAA-ATPase gene family, associates with a plethora of protein adaptors to perform a variety of cellular processes including Golgi assembly/disassembly and regulation of the ubiquitin-proteasome system. However, the mechanism whereby mutations in VCP lead to CNS, muscle, and bone disease is largely unknown. In this report, we review current literature on IBMPFD, focusing on the pathology of the disease and the biology of VCP with respect to IBMPFD.
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PMID:Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy. 1745 94

Retroperitoneal fibrosis (RPF) is a relatively rare disease which shows a periaortic mass in the retroperitoneal area and predisposes to an obstructive uropathy. We report a case of idiopathic RPF occurring in a patient who was suspected of impending rupture of abdominal aortic aneurysm. A 60-year-old male, with a 2-week history of abdominal pain, was transferred for evaluation of the periaortic mass. Computed tomographic (CT) scan revealed radiological findings such as leakage of contrast media from the aortic lumen and expansion of the periaortic mass. The patient underwent laparotomy, which revealed retroperitoneal fibrotic plaques in the absence of aortic aneurysm. The pathological findings of the biopsy specimen were consistent with idiopathic RPF. The patient received ureteral stent placement and was treated with steroid therapy. When a similar case is encountered, our recommendations are as follows: (i) Both CT scan and magnetic resonance (MR) imaging should be performed to determine whether the retroperitoneal mass is due to idiopathic or secondary RPF. If idiopathic RPF is suspected, the patient should receive primary steroid therapy. (ii) Retroperitoneal periaortic mass indicates a need for the assessment of obstructive uropathy. Early placement of the ureteral stent is necessary for urinary decompression and preservation of the renal function in patients with obstructive uropathy.
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PMID:Idiopathic retroperitoneal fibrosis in a patient suspected of impending rupture of the abdominal aortic aneurysm. 1829 44

An abdominal aortic aneurysm is a rare disease in the paediatric population and is mainly caused by intrauterine infection, connective tissue diseases, such as Ehlers-Danlos syndrome and Marfan's syndrome, and iatrogenic trauma due to umbilical artery catheterization. Although several cases have been reported in the English literature, they were rarely diagnosed prenatally. Vascular obstruction in utero is also believed to be the major cause of porencephaly. Recently, gene mutations have been reported as the cause of both the above-mentioned diseases. We present a prenatally diagnosed case of congenital abdominal aortic aneurysm with porencephaly.
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PMID:Congenital abdominal aortic aneurysm with porencephaly: a case report. 2116 Jan 69

Heterotopic mesenteric ossification (HMO) is a rare disease that results in intra-abdominal ossification of unknown origin. An 88-year-old man developed an intestinal obstruction 2 weeks after undergoing an operation for a ruptured abdominal aortic aneurysm, resulting in intestinal obstructions those did not improved concervatively. During relaparotomy performed 30 days after the first operation, hard adhesions of the small intestine and mesentery were found; these adhesions were difficult to separate without damaging the serosa of the small intestine. We removed 240 cm of the small intestine and performed a jejuno-ileo anastomosis. Microscopically, trabecular bone tissue had increased irregularly in the fat tissue of the nodules with fibrosis, which were partially lined with osteoblasts. Accordingly, we histopathologically diagnosed the patient as having HMO. The patient was treated with NSAIDs and cimetidine to prevent the recurrence of HMO. No signs of recurrence have occurred as of one year after the second operation.
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PMID:Heterotopic mesenteric ossification after a ruptured abdominal aortic aneurism: case report with a review of literatures. 2505 88

Acute mesenteric ischemia (AMI) is generally thought to be a rare disease, but in fact, it is more common cause of acute abdomen than appendicitis or ruptured abdominal aortic aneurysm in patients over 75 years of age. In occlusive AMI, surgical treatment without revascularization is associated with as high as 80% overall mortality. It has been shown that early diagnosis with contrast-enhanced computed tomography and revascularization can reduce the overall mortality in AMI by up to 50%. However, only a minority of patients with AMI are being treated actively with revascularization in the United States, and the situation is very likely similar in Europe as well. What can we do to improve diagnostic performance, so that more patients get proper treatment? The diagnosis is a collaborative effort of emergency department surgeons, gastrointestinal and vascular surgeons, and radiologists. The etiological categorization of AMI should be practical and guide the therapy. Furthermore, the limitations of the diagnostic examinations need to be understood with special emphasis on computed tomography findings on patients with slowly progressing "acute-on-chronic" mesenteric ischemia.
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PMID:Acute mesenteric ischemia (part I) - Incidence, etiologies, and how to improve early diagnosis. 2839 84