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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The indications and management of blood transfusion in the haemoglobinopathies have been reviewed. The sickle cell diseases that require transfusion support are sickle cell anaemia, sickle haemoglobin-C and -D diseases and sickle beta-thalassaemia. Homozygous beta-thalassaemia (Cooley's anaemia) is the major problem among the thalassaemias. The pathophysiology of the sickle cell disorders is largely based on the secondary effects of increased blood viscosity, whereas in the thalassaemias the defect is ineffective haematopoiesis. In the former the major problems occur as manifestations of vaso-occlusive crises with disseminated bone and abdominal pain, priapism, stroke and leg ulcers. Bone infarction and aseptic necrosis occur but the widespread bone changes, underdevelopment and haemochromatosis that complicate the thalassaemia are not prominent. Transfusion therapy in the sickle cell diseases is mainly episodic and is guided by the frequency of crises and the severity of vaso-occlusive complications. Partial exchange transfusion and the maintenance of haemoglobin A concentrations at 40 to 50 per cent is frequently indicated. In the thalassaemias, maintenance of haemoglobin levels is essential for normal growth and development. The problem of haemochromatosis is very serious. With hypertransfusion regimens the haemoglobin and haemotocrit are maintained above 12-13 g/dl and 35 per cent. The resulting benefit appears to be reduced blood volume, less iron turnover, and less intestinal iron absorption. The splenomegaly in these disorders is frequently associated with hypersplenism requiring well-timed splenectomy. Chronic and intensive chelation is necessary to prevent the ravages of iron overload. The availability of automated equipment for in vivo and ex vivo blood cell separation has brought new possibilities for improving the management of these haemoglobinopathies. It is feasible, but not as yet practical, to offer transfusions of neocytes (red cells with a mean age of 30 days) which have a 50 per cent longer survival than routine red cell preparations (mean age of 60 days). Neocytes can be prepared ex vivo from fresh routine blood donations using blood cell separator devices. The result is reduced transfusion requirements. A more recent suggestion for using the new technology is to remove the patient's oldest and most abnormal corpuscles on the basis of buoyant density and replacing them with neocytes . Thus the short-lived abnormal red cells would be removed before they could unload their iron. With automation it is possible to perform these procedures on an outpatient basis.
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PMID:Transfusion support for haemoglobinopathies. 637 80

During the years 1969-1980 elective splenectomy for haematological disease was performed in 31 children, of which 17 had haemolytic anaemia (HA), 11 thrombocytopenic purpura (TP) and 3 hypersplenism (HS). The indications for splenectomy in the HA series were haemolytic crises in 6 children, constant anaemia in 4, hyperbilirubinaemia in 5 and abdominal pain in 2 children. Among children with TP there were 8 cases of idiopathic thrombocytopenic purpura, 2 cases combined TP with haemolytic anaemia and idiopathic pancytopenia in 1 child. In all patients the indication for splenectomy was therapy-resistant thrombocytopenia. In the HS series the underlying disease was Morbus Gaucher, thyrosinosis and cysta lienalis. Indication for splenectomy was thrombocytopenia in all children. In the HA series the results of splenectomy were good in all patients. The haemoglobin value rose. The increased reticulocyte count returned to normal values. Splenectomy was considered to be still indicated in all patients with hereditary spherocytosis. In the TP series good results were obtained in 6 children, 2 were markedly improved, but 3 showed no change. Splenectomy plays an important role in the management of chronic thrombocytopenia when medical treatment is without effect. In the HS series thrombocytes reached normal values in all patients. Failure of splenectomy to relieve the haematologic process is usually due to remaining accessory spleens. Splenectomy in children increases the risk of severe infections and the polyvalent pneumococcal vaccine should therefore be administered prior to splenectomy.
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PMID:Splenectomy in children with haematological disease. 689 45

Bacterial peritonitis presents with classic symptoms of fever and abdominal pain. Some patients, however, are completely asymptomatic. Death in the short term is considerable, especially in patients with alcoholic cirrhosis. Cystic fibrosis patients occasionally develop biliary cirrhosis and may have secondary hypersplenism, varices, and ascites. These patients should be at risk for spontaneous bacterial peritonitis. Spontaneous bacterial peritonitis is described in two patients with longstanding hepatic cirrhosis secondary to cystic fibrosis. Both had required splenectomy for complications of portal hypertension. This is a previously unreported, but potentially fatal, complication of cystic fibrosis liver disease. Early diagnostic paracentesis is essential so that appropriate acute management, including antimicrobial treatment can be started. In the long term, these patients deserve immediate paracentesis for any evidence of recurrence. Whether the patient is treated with chronic (continuous) antimicrobial prophylaxis or only receives antimicrobial treatment during periods when bacteraemia is possible (for example, dental work, bronchoscopy), it would seem reasonable in patients with cystic fibrosis to use a wide spectrum antimicrobial agent with activity against Pseudomonas aeruginosa, other common Gram negative organisms, and Staphylococcus aureus.
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PMID:Spontaneous bacterial peritonitis in cystic fibrosis. 820 May 73

A 12-year-old girl who underwent a splenectomy followed by heterotopic splenic autotransplantation for treatment of hypersplenism secondary to Gaucher disease at the age of 3 years presented with acute lower abdominal pain. Radiological investigations were highly suggestive of torsion of the autotransplanted splenic tissue, which was confirmed during subsequent laparotomy. This is the first known report of torsion of autotransplanted splenic tissue.
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PMID:Torsion of autotransplanted splenic tissue in Gaucher disease. 916 68

The aim of this study was to elusidate the change in residual spleen volume after partial splenic embolization (PSE) in 43 cirrhotic patients with marked hypersplenism. Residual spleen volume was indicated as the rate (%) of residual spleen to initial spleen before PSE. Furthermore, the platelet count after PSE was observed in 23 patients followed up for 2 years. Residual spleen volume in patients with infarction rates of more than 80% (group A) had been maintained within 20 % even after 2 years, while they had obviously increased during the early stage after PSE in patients with infarction rates under 80% (group B), especially in patients with lower infarction rates (under 60%). Mean platelet count improved significantly in both groups after PSE (p < 0.001, respectively), but increased more in group A than in group B (p < 0.01). High fever and abdominal pain were observed in all cases of PSE. Other adverse effects such as pleural effusion and ascites that were frequent in group A were transient. These results suggest that PSE performed with a high infarction rate of the spleen provides effective, long-lasting results in the treatment of hypersplenism in cirrhosis.
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PMID:[Prognosis of residual spleen after partial splenic embolization for the treatment of hypersplenism in cirrhosis]. 958 59

Despite the prevalence of splenomegaly as a sign in many disorders, there have been no studies that correlate the degree of organomegaly with the symptoms generally ascribed to splenic enlargement. The degree of splenomegaly was compared with five overt symptoms of mechanical displacement, i.e. chronic abdominal pain, abdominal discomfort, early satiety, pain while lying on the side, or attacks of acute (colicky) left upper quadrant pains. We have also employed splenomegaly as seen in Gaucher disease as a paradigm to determine whether there is a correlation between the degree of splenomegaly and the parameters of hypersplenism. Although there was a statistically significant correlation between degree of splenomegaly and blood counts, this proved to be clinically negligible. Surprisingly, there was also no correlation between degree of splenomegaly and any of symptoms investigated.
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PMID:Is there a correlation between degree of splenomegaly, symptoms and hypersplenism? A study of 218 patients with Gaucher disease. 1046 78

In recent years, partial splenic embolization (PSE) has been widely used in patients with cirrhosis and hypersplenism caused by portal hypertension. We investigated the complications associated with PSE cases seen in our hospital. Seventeen cases of liver cirrhosis that had undergone PSE were examined to investigate the complications associated with it. Mean infarcted area of the spleen was 66.2%. Leukocyte and platelet counts in 16 of 17 patients were seen to improve after PSE and persisted for at least one year. The most frequent side effects were abdominal pain (82.4%) and fever (94.1%). Severe side effects were seen in two of those 17 patients. One patient died from acute on chronic liver failure. The other patients contracted bacterial peritonitis and splenic abscess and needed drainage of splenic abscess before recovery. These two cases were in Child-Pugh class B. In conclusions, PSE is a useful treatment for patients with cirrhosis and hypersplenism caused by portal hypertension. However, the possibility of severe complications, especially in patients with noncompensated cirrhosis, should be kept in mind.
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PMID:Complications of partial splenic embolization in cirrhotic patients. 1185 56

The register of Latvian children with portal hypertension (PH) includes 34 patients from 2 to 16 years old. In 26 children the PH is caused by a prehepatic obstruction. The most widespread symptoms of PH are variceal bleeding and hypersplenism. The traditional management of hypersplenism has been splenectomy. We have used partial splenic embolization (PSE) as an alternative to splenectomy. From 1998 to 2000 we carried out PSE in 8 patients with PH. A percutaneous femoral artery approach to the splenic artery was used to deliver microspheres with a diameter of 0.3 mm into the spleen, minimizing blood circulation to the organ by 60 - 70 %. In 7 patients symptoms of hypersplenism disappeared and blood tests returned to normal status. In one patient we carried out PSE twice. In one case PSE was not successful and the patient underwent partial resection. Abdominal pain and subfebrility were observed on average 2 weeks after PSE. In 7 patients the 4 - 20 months follow-up period demonstrated the efficiency of the non-operative hypersplenism management method. We conclude that PSE is the method of choice to treat hypersplenism.
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PMID:The first experience with non-operative treatment of hypersplenism in children with portal hypertension. 1246 54

Pancytopenia, although mainly reported in adults, has also been described in children with brucellosis. However, bone marrow hypoplasia is a rare feature of the infection. An 11-year-old boy was admitted with fever, vomiting, and abdominal pain of 10 days' duration. On physical examination, pallor and high fever were detected in the absence of lymphadenopathy and hepatosplenomegaly. His hemoglobin was 8.6 g/dL, white blood cell count 1,100/mm(3), neutrophil count 500/mm(3), platelets 56,000/mm(3), and reticulocytes 0.1%. Hypocellular bone marrow was found by aspiration, and bone marrow biopsy revealed hypocellularity. The agglutination titer was greater than 1/640. Trimethoprim/sulfamethoxazole was prescribed. His fever subsided and pancytopenia subsequently improved. Pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular. Bone marrow hypoplasia is rarely reported and should be kept in mind in the etiology of aplastic anemia in a country where brucellosis is frequently encountered.
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PMID:Bone marrow hypoplasia during Brucella infection. 1254 75

DIAGNOSTIC CIRCUMSTANCES: Portal vein thrombosis is the second cause of portal hypertension after cirrhosis in Western countries. Diagnosis can be either made at the acute stage in the context of abdominal pain or after appearance of a porto-portal collateral venous circulation leading to the formation of a portal cavernoma, the diagnosis being made in the circumstance of rupture of oesophageal varicose veins or manifestations of hypersplenism. AETIOLOGICAL SURVEY: In the absence of hepatocellular carcinoma, causes that need to be investigated are cirrhosis, local factors (intra-abdominal sepsis, abdominal surgery, splenectomy or pancreatitis), and one or several prothrombotic affections (acquired or inherited prothrombotic states are present in 70% of cases, with myeloproliferative disease ranking first). REGARDING TREATMENT: Anticoagulant therapy generally allows recanalisation of the thombosed veins in recently constituted thrombosis. Some patients at the portal cavernoma stage can also benefit from anticoagulant therapy: patients with a prothrombotic state without large oesophageal-gastric varicose veins. In the case of large oesophageal-gastric varicose veins that have never bled, treatment to prevent haemorrhages due to portal hypertension according to the same modalities as in cirrhosis must be associated with the prescription of an anticoagulant. In the absence of prothrombotic affection or in patients having already suffered from haemorrhages due to portal hypertension, the benefit of anticoagulant therapy is less clearly established.
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PMID:[Portal vein thrombosis]. 1453 80


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