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

Essential thrombocythemia (ET) is a rare disorder in children. An 11-year-old white boy was first seen in January 1986 with symptoms of abdominal pain. His platelet count was 1.5 million/mm3. Other hematological values and coagulation studies, including bleeding time, were normal. There was laboratory evidence of mild platelet dysfunction. Using the criteria of the Polycythemia Vera Study Group, a diagnosis of ET was made. He developed frequent headaches. Aspirin was prescribed for the next 2 years at varying doses and frequency. During the period, platelet counts ranged between 1 and 3 million/mm3. In view of progressive headaches and evidence of increasing platelet dysfunction, further treatment was indicated. The use of a new agent, anagrelide, reported effective in adults with ET, resulted in amelioration of symptoms and improvement in quantitative and qualitative platelet control with no significant untoward effects.
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PMID:Essential thrombocythemia in a child: management with anagrelide. 202 80

Budd-Chiari syndrome is caused by the obstruction of the hepatic veins or of the inferior vena cava. It is characterized by the classic symptomatological triad: ascites, hepatomegaly, and abdominal pain. In 2/3 cases its etiology remains unknown. Budd-Chiari syndrome may be associated with polycythemia vera, neoplasms, chronic leukemia, congenital abnormalities, hypercoagulation conditions, pregnancy, oral contraceptives, and constrictive pericarditis. Even though its clinical diagnosis is difficult, radiology plays a decisive role with US, CT, MR imaging and, above all, angiography; the latter, together with liver biopsy, generally provides with an unquestionable diagnosis. Through the definition of stage of the disease, of level (intrahepatic, venous, caval, cardiac), of type (intrinsic or extrinsic), and degree of both obstruction and consequent development of collateral channels, radiology determines which patients should undergo a medical or a surgical treatment. In some case, percutaneous angioplasty can be performed. Four cases of Budd-Chiari syndrome, including two children, were investigated with US, CT, angiography, and liver biopsy; MR imaging was also employed in one case. The underlying cause was identified in 3 patients: constrictive pericarditis of probable congenital origin and web occlusion of the inferior vena cava near the right atrium in the 2 children; hepatic vein thrombosis due to essential thrombocythemia in the third case. In the fourth patient, thrombosis of the inferior vena cava and hepatic veins was unexplained. The diagnosis was established by means of liver biopsy and phlebography of the hepatic veins. Good diagnostic information was also supplied by non-invasive techniques, such as US, CT, and MR imaging.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Diagnostic imaging of Budd-Chiari syndrome in adults and children]. 218 5

Of 501 patients with chronic myeloproliferative diseases (c-MPD) 18 developed thrombosis of major abdominal vessels including 6 with hepatic vein thrombosis (Budd-Chiari syndrome). The complication was seen in 14 of 140 (10%) patients with polycythemia vera (PV), 3 of 23 (13%) patients with essential thrombocythemia (ET), 1 of 106 (1%) patients with idiopathic myelofibrosis (IMF), and none of 232 patients with chronic myelogenous leukemia (CML). Leading symptoms and signs were abdominal pain, progressive splenomegaly, widening abdominal girth, ascites, venous collaterals, and nausea and vomiting. The diagnostic modalities with highest specificity were angiography and explorative laparotomy. A causal relationship between the thrombotic event and hematocrit, thrombocyte count, or hemostatic abnormalities at the time of diagnosis could not be established. Detailed laboratory tests of platelet function and coagulation and fibrinolytic parameters of 5 surviving patients did not show any specific defect. Despite medical and surgical intervention, 39% of the patients died within 2 months after diagnosis of the thrombosis. The majority of the survivors developed further complications like liver cirrhosis with portal hypertension and esophageal varices or the short bowel syndrome after extensive bowel resection for mesenterial infarction.
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PMID:Budd-Chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases. 279 52

Lately, myeloprolipherative disorders are frequently reported as causes of portal vein thrombosis, probably due to the early detection of latent cases of this condition. We report two patients with portal vein thrombosis that presented with abdominal pain, nausea, vomiting and clinical consequences of portal hypertension such as variceal hemorrhage, splenomegaly and ascites. Diagnosis was made by a CAT scan in one patient and doppler ultrasound in the other. Both patients had high platelet counts and an essential thrombocytosis in the bone marrow.
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PMID:[Portal vein thrombosis associated with essential thrombocytosis. Clinical cases and review of the literature]. 900 49

A 17-year-old girl known to suffer from essential thrombocytosis (ET) was admitted with severe abdominal pain that was due to sonographically verified portal and hepatic vein thrombosis. ET is a well-documented disease and portal vein thrombosis has often been reported in adults but not in children and adolescents suffering from ET. Coincidence of ET, urinary tract infection, oral contraception, and cessation of acetylsalicylic acid therapy might be responsible for the early manifestation of portal and hepatic vein thrombosis in our patient.
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PMID:Portal vein thrombosis in a 17-year-old female adolescent with essential thrombocytosis. 926 78

Hepatic venous outflow obstruction caused by hepatic vein thrombosis (HVT) is a manifestation of a hypercoagulable state, most commonly a myeloproliferative disorder (MPD). In the past, HVT was thought to have a poor prognosis unless treated surgically with portosystemic shunt or orthotopic liver transplantation (OLT). The aim of this study was to assess whether early diagnosis of the underlying hematologic disorder and institution of appropriate medical therapy have altered outcome. We reviewed the charts of 22 patients with HVT evaluated at our center from January 1986 to January 1995. The median age was 32 years (range, 14 to 59 years). Underlying etiologies were MPD, 13 (polycythemia vera, 8; essential thrombocythemia, 4; undefined, 1); dysfibrinogenemia, 1; anticardiolipin antibody, 1; oral contraceptive use, 3; and idiopathic, 4. All patients had ascites, hepatomegaly, and/or abdominal pain. Two underwent mesocaval shunting, and 1 had a peritoneal-venous shunt. Seven patients, including 1 with a mesocaval shunt, underwent OLT. The median duration of symptoms before transplantation was 6 months (range, 1.5 to 11 months). Six transplant patients are alive on long-term anticoagulation therapy at a mean post-OLT follow-up of 42 months (range, 2 to 77 months), without recurrence. Of 13 patients treated medically, 10 (77%) are alive at a median follow-up of 40 months (range, 17 months to 14 years 8 months), 1 has died, and 2 have been lost to follow-up. In a majority of patients, symptoms improve with prompt treatment of the underlying hematologic disorder, with a favorable long-term prognosis. Patients with decompensated liver disease can successfully undergo OLT with a low risk of recurrence on long-term oral anticoagulation.
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PMID:Reassessing the role of medical therapy in the management of hepatic vein thrombosis. 934 78

The pharmacology, pharmacokinetics, clinical efficacy, adverse effects, and dosage and administration of anagrelide are reviewed. Anagrelide is a selective thrombocytopenic agent with FDA-approved labeling for the treatment of essential thrombocythemia. Clinical trials have shown that the drug may have a role in the treatment of other chronic myeloproliferative disorders, including polycythemia vera, chronic myeloid leukemia, and agnogenic myeloid metaplasia. The mechanism by which anagrelide reduces platelet count is not yet clear. The current hypothesis is that anagrelide affects the late (postmitotic) phases of megakaryocyte development. Anagrelide has a large volume of distribution and is extensively metabolized; less than 1% is recovered unchanged in the urine. Plasma half-life after a 0.5-mg dose is 1.3 hours. Anagrelide's efficacy and safety have been evaluated in open-label, noncomparative trials, in which the response rate was 60-93%. Adverse effects include headache, diarrhea, edema, palpitations, and abdominal pain. Patients with renal or hepatic dysfunction need to be closely monitored for signs of toxicity. The recommended starting dosage is 0.5 mg four times a day or 1 mg twice a day, with dosage adjustment to the lowest effective amount required to reduce and maintain platelet count below 600 x 10(9)/L. The wholesale acquisition price for 0.5-mg capsules is $350 per 100. Whether anagrelide will replace hydroxyurea as first-line therapy in some or all patients remains to be determined. Anagrelide is effective in the treatment of essential thrombocythemia and may have a role in the treatment of other myeloproliferative disorders.
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PMID:Anagrelide, a selective thrombocytopenic agent. 978 84

A 60-year-old woman was admitted in August 1995 complaining of abdominal pain. A diagnosis of essential thrombocythemia had been made in 1987, and myelofibrosis developed in the patient thereafter. Physical examination revealed massive hepatosplenomegaly, and the peripheral blood showed leukoerythroblastosis with chromosomal abnormalities in peripheral blood cells. In May, 1996, blastic transformation occurred. Based on the findings of surface marker analysis, the blasts met the diagnostic criteria for acete myelogenous leukemia because they were negative for peroxidase and positive for CD13. In June, the patient died of multiple organ failure. Postmortem examination revealed multiple tumor emboli.
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PMID:[Transformation of myelofibrosis into acute myelogenous leukemia (M0) with multiple tumor emboli]. 978 83

A 59-year-old man with essential thrombocytosis was examined for abdominal pain. Splenic infarction was diagnosed on a computed tomographic scan. The Tc-99m heat-denatured RBC scan showed viable splenic tissue that was not evident on the computed tomographic scan or Tc-99m sulfur colloid scintigraphy.
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PMID:Assessment of viable splenic tissue in massive splenic infarction with a Tc-99m heat-damaged RBC scan. 1131 22

Essential thrombocythemia and polycythemia vera are both chronic progressive myeloproliferative disorders of insidious onset. If the excessive production of red cells and/or platelets is controlled, patients with these disorders may have prolonged survival. However, the clinical course of these patients can be complicated by a variety of events, including thrombotic episodes, bleeding episodes, arthropathies, pruritus, weakness, weight loss, neurologic impairment, erythromelalgia, fever, abdominal pain, and the life-threatening consequences of progression to myelofibrosis and/or acute leukemia. Effective control of hematopoiesis by phlebotomy or a variety of therapeutic agents has resulted in a reduction or elimination of many of these clinical events, but has not altered the evolution to myelofibrosis or acute leukemia. Use of each of these therapeutic strategies is also associated with a range of adverse events. Monitoring overall survival or a reduction in the frequency of clinical events has previously served as a means of assessing the results of these therapeutic interventions. Quality-of-life instruments have not been applied in a systematic fashion to the evaluation of outcomes in patients with these chronic myeloproliferative disorders. Quality-of-life assessments evaluate not only the state of well-being of a patient that results from an assessment of the individual's ability to perform everyday activities, which are reflective of physical, psychological, and social well-being, but also patient satisfaction with the control of disease and/or treatment-related symptoms. Quality-of-life instruments have been used to assess the clinical course of patients suffering from a variety of disorders, ranging from cancer to renal failure to chronic fatigue syndrome. Information about quality-of-life outcomes can contribute to the evaluation of variations in dose and timing of administration of therapeutic agents. It is possible that the side effects of a particular therapy may outweigh the disease regression achieved with a particular therapy. In the future, quality-of-life instruments may prove useful in prospectively evaluating therapeutic end points in patients with essential thrombocythemia and polycythemia vera.
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PMID:Quality of life issues in patients with essential thrombocythemia and polycythemia vera. 1209 51


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