Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the more prevalent genetic disease at birth in Ile-de France area. Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to ischemic process, and infectious complications. In adults, acute vaso-occlusive crisis is the major clinical problem prompting admission to hospital and the main cause of death. It mainly manifests by osteoarticular pain and acute chest syndrome and can be complicated by multiorgan failure. Acute vaso-occlusive crisis can be also manifested by priapism, ischemic or haemorrhagic stroke, or abdominal pain. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.
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PMID:[Emergencies in adults with sickle cell disease]. 1558 59

Liver abscess is a rare complication of liver biopsy but has not been reported in a patient with sickle cell disease (SCD) undergoing this procedure. The authors report the case of a child with SCD, on treatment with chronic transfusions and desferrioxamine, who developed a delayed liver abscess after a liver biopsy in which Avitene, a topical hemostatic agent, was used. This is the first description of a liver abscess as a possible complication of liver biopsy in a child with SCD. The presence of a hepatic abscess should be considered in the differential diagnosis of atypical abdominal pain in patients with SCD.
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PMID:Pyogenic hepatic abscess after percutaneous liver biopsy in a patient with sickle cell disease. 1570 87

We report a case of portal vein thrombosis following prolonged laparoscopic intervention in a patient with Sickle cell disease. The operation combined a laparoscopic splenectomy, cholecystectomy, appendectomy. Presentation was insidious with vague abdominal pain and persistent postoperative pyrexia. The literature is reviewed, possible pathogeneses are discussed in the light of currently available data, and a strategy is suggested to recognize, avoid, and prevent this rare but potentially lethal complication.
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PMID:Portal vein thrombosis following laparoscopic surgery in a patient with sickle cell disease. 1576 50

Sickle cell disease is characterized by chronic hemolytic anemia and vaso-occlusive painful crises. The vascular occlusion in sickle cell disease is a complex process and accounts for the majority of the clinical manifestation of the disease. Abdominal pain is an important component of vaso-occlusive painful crises. It often represents a substantial diagnostic challenge in this population of patients. These episodes are often attributed to micro-vessel occlusion and infarcts of mesentery and abdominal viscera. Abdominal pain due to sickle cell vaso-occlusive crisis is often indistinguishable from an acute intra-abdominal disease process such as acute cholecystitis, acute pancreatitis, hepatic infarction, ischemic colitis and acute appendicitis. In the majority of cases, however, no specific cause is identified and spontaneous resolution occurs. This chapter will focus on etiologies, pathophysiology and management of abdominal pain in patients with sickle cell disease.
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PMID:Unusual causes of abdominal pain: sickle cell anemia. 1583 95

We report a case of a 6-year-old girl of Afro-Caribbean origin, known to have sickle cell disease (SCD), with recurrent history of jaundice and abdominal pain. She was extensively investigated, including endoscopic retrograde cholangiopancreatography (ERCP), which revealed diffuse cholangiopathy of both extrahepatic and intrahepatic bile ducts. A pigtail stent was placed and balloon dilatation was performed for stricture of the extrahepatic duct. Since then, she remains well and asymptomatic. We suggest that cholangiopathy is the consequence of sickling in the end arteries of the biliary arterial tree.
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PMID:Ischaemic cholangiopathy and sickle cell disease. 1621 23

Abdominal pain of presumed vasocclusive origin, often termed 'girdle syndrome' because of the circumferential distribution of the pain, is common in sickle cell anaemia (SCA). Evidence of progression to bowel infarction is rare. A 27-year-old man with SCA developed chest and abdominal pain unresponsive to opiate analgesia. Abdominal X-ray showed dilated bowel loops because of partial obstruction. Despite reduction of HbS to 23% by automated red cell exchange, abdominal pain worsened. A CT scan was the most informative investigation and showed free peritoneal air. He underwent emergency hemicolectomy and reversible ileostomy formation. Histology of the resected colon was consistent with acute ischaemic colitis. Early surgical intervention remains essential in SCA when abdominal pain does not respond to maximal therapy including red cell exchange: as this case illustrates, sickle girdle syndrome has the capacity to progress to irreversible ischaemic colitis and necrotic perforation of the bowel wall.
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PMID:Sickle cell 'girdle syndrome' progressing to ischaemic colitis and colonic perforation. 1643 Apr 62

Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0-21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality-a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.
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PMID:Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases. 1646 55

The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.
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PMID:Renal amyloidosis in a child with sickle cell anemia. 1657 Feb 2

Hemoglobinopathy S, Depranocytosis or Sickle Cell Disease is the most common hemoglobinopathy in the world. In its heterozygous form (Sickle Cell Trait), it affects 8% of the black population in the U.S. and 25% of the black population in Africa, and is found less frequently in the Mediterranean area, India, Middle East and Latin America. The basic alteration is a substitution of glutamic acid by valin in the sixth position of the beta globin chain, which causes polymerization at low oxygen tension thereby distorting the structure of erythrocytes and increasing blood viscosity, which, in turn, generates obstructions of the capillary arterial blood flow to different areas of the body thus causing microinfarctions. Although Splenic Infarction is rare, it is recognized as a serious complication of Heterozygous Sickle Cell Disease (Sickle Cell Trait). We present the case of a 21 year-old mestizo male patient who came in with an acute case of abdominal pain after arriving to work in the Casapalca mining city (located in the Peruvian Andes at 4200 m.a.s.l.) and was referred to our Hospital in Lima for exams. We present the case because it is an unusual cause of acute abdominal pain, and because this condition is rare in Peru and there are few publications about it.
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PMID:[Acute abdominal pain due to splenic infarction in a patient with heterozygous sickle cell disease exposed to high altitude]. 1721 89

Deferasirox is a once-daily, oral iron chelator developed for treating transfusional iron overload. Preclinical studies indicated that the kidney was a potential target organ of toxicity. As patients with sickle cell disease often have abnormal baseline renal function, the primary objective of this randomised, open-label, phase II trial was to evaluate the safety and tolerability of deferasirox in comparison with deferoxamine in this population. Assessment of efficacy, as measured by change in liver iron concentration (LIC) using biosusceptometry, was a secondary objective. A total of 195 adult and paediatric patients received deferasirox (n = 132) or deferoxamine (n = 63). Adverse events most commonly associated with deferasirox were mild, including transient nausea, vomiting, diarrhoea, abdominal pain and skin rash. Abnormal laboratory studies with deferasirox were occasionally associated with mild non-progressive increases in serum creatinine and reversible elevations in liver function tests. Discontinuation rates from deferasirox (11.4%) and deferoxamine (11.1%) were similar. Over 1 year, similar dose-dependent LIC reductions were observed with deferasirox and deferoxamine. Once-daily oral deferasirox has acceptable tolerability and appears to have similar efficacy to deferoxamine in reducing iron burden in transfused patients with sickle cell disease.
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PMID:A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. 1723 48


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