Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eight patients received PUVA for mastocytosis. Five women had typical adult-onset urticaria pigmentosa, without evidence of systemic disease. Another woman had suspected hepatic involvement while the remaining female had early-onset familial urticaria pigmentosa with morphologically atypical mast cells. The only male patient had cirrhosis with hepatic deposits of mast cells in addition to polycythaemia rubra vera. In all patients, except the man with systemic disease, there was reduced pruritus and wealing and partial to almost complete fading of the macules. The manifestations of urticaria pigmentosa recurred after treatment was discontinued. In both lesional and uninvolved skin there was no significant change in either the mean mast cell counts or mast cell ultrastructure after an average of twenty-seven PUVA exposures. In addition, PUVA did not cause a significant alteration in the histamine content of the skin. The beneficial effect of PUVA in urticaria pigmentosa therefore does not appear to be directly related to a change in mast cell numbers or morphology, or to the histamine concentration in the skin.
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PMID:Photochemotherapy (PUVA) in the treatment of urticaria pigmentosa. 686 May 73

A 26-year-old woman, after cesarean section in the 33rd week of gestation, developed after delivery thrombosis of the popliteal vein, pulmonary embolism and thrombosis of the portal vein. After completion of a six month period of oral anticoagulation, laboratory investigations revealed diminished levels of plasminogen and free protein S antigen as well as APC-resistance due to heterozygous FV R506Q mutation. After six uneventful years, abdominal sonography and magnetic resonance examination, performed because of abdominal pain, showed liver cirrhosis with Budd-Chiari syndrome. Additional hematological investigations led to the diagnosis of polycythemia vera. Association of myeloproliferative disorders, mainly polycythemia vera, with splanchnic venous thrombosis is well known and should always be looked for.
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PMID:[A 26-year-old woman with splanchnic vein thrombosis as the initial manifestation of polycythemia vera]. 1051 20

Elevated levels of serum cobalamin may be a sign of a serious, even life-threatening, disease. Hematologic disorders like chronic myelogeneous leukemia, promyelocytic leukemia, polycythemia vera and also the hypereosinophilic syndrome can result in elevated levels of cobalamin. Not surprisingly, a rise of the cobalamin concentration in serum is one of the diagnostic criteria for the latter two diseases. The increase in circulating cobalamin levels is predominantly caused by enhanced production of haptocorrin. Several liver diseases like acute hepatitis, cirrhosis, hepatocellular carcinoma and metastatic liver disease can also be accompanied by an increase in circulating cobalamin. This phenomenon is predominantly caused by cobalamin release during hepatic cytolysis and/or decreased cobalamin clearance by the affected liver. Altogether it can be concluded that an observed elevation of cobalamin in blood merits the a full diagnostic work up to assess the presence of disease.
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PMID:Significance of elevated cobalamin (vitamin B12) levels in blood. 1463 71

A qualitative abnormality of platelet function should be considered in patients with mucocutaneous bleeding in the absence of thrombocytopenia or von Willebrand disease. Antiplatelet drugs are the most common cause of acquired platelet disorders leading to bleeding. Uremia, hepatic cirrhosis, myeloma and related disorders, polycythemia vera, essential thrombocythemia, and cardiopulmonary bypass have long been recognized as clinical situations in which platelet dysfunction may contribute to bleeding. When an acquired platelet disorder is suspected, it is useful to examine platelet function by measuring the bleeding time, examining platelet-dependent closure time in a platelet function analyzer and performing platelet aggregometry. When a specific acquired platelet disorder is diagnosed, many treatment options are available including controlling the underlying disease, giving platelet transfusions and administering a hemostatic drug.
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PMID:Acquired disorders of platelet function. 1630 11

Endoscopic band ligation is an effective technique for primary and secondary prevention of gastro-esophageal variceal bleeding (GEVB), but can also result in rebleeding from postbanding ulcers. Its use in primary and secondary prevention of GEVB in anticoagulated patients has not been systematically studied. The aim of the study was to evaluate the feasibility of band ligation in primary and secondary prevention of GEVB in anticoagulated patients. Five patients (age 60.2+/-7.3 SD years: 3 males, 2 females) with esophageal varices on anticoagulation were studied using a retrospective chart review in a tertiary hospital setting. Patients were on mandatory anticoagulation with warfarin (international normalized ratio >2), on nonselective beta-blocker therapy if tolerated and were not transvenous intrahepatic porto-systemic shunting candidates. One patient had polycythemia vera (noncirrhotic), the rest were cirrhotics Child class B/C (1 cardiogenic, 1 primary sclerosing cholangitis, 1 Budd-Chiari, and 1 cryptogenic cirrhosis). Two patients had experienced prior acute GEVB; band ligation performed during acute bleeding was not included in the study. All patients had at least grade III-IV esophageal varices on outpatient follow-up for band ligation. Three bands were placed/patient and study patients underwent 3 banding sessions on an average. None of the patients developed GEVB after band ligation. In 3 patients banding resulted in complete variceal eradication, the remaining 2 are still being followed-up for outpatient band ligation. In conclusion, this case series suggests that endoscopic band ligation can potentially be used in anticoagulated patients without alternatives for prevention of acute GEVB.
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PMID:Endoscopic band ligation of esophageal varices in patients on anticoagulation. 1866 2

We report a case of a patient with a diagnosis of myeloproliferative neoplasm, unclassifiable, manifested only portal vein thrombosis and followed by cirrhosis of the liver. 37-year-old patient, previously healthy, without congenital thrombophilia, without prior thrombosis, with normal peripheral blood morphology were signs of extensive portal vein system, with massive collateral circulation. Patient did not meet the criteria for diagnosis of any of the classic myeloproliferative neoplasms. Bone marrow examination revealed hyperplasia and presence of single polymorphic megakaryocytes. Positive JAK2V617F mutation status was typical for myeloproliferative neoplasm. Therefore, that the portal system thrombosis is, sometimes accompanying symptom of other myeloproliferative neoplasm, caused by mutations, including polycythemia vera, essential thrombocythaemia and primary myelofibrisis, one can assume that between this mutation and observed in this patient thrombosis is relationship, despite the absence of changes in peripheral blood. This may suggest that we are dealing with myeloproliferative neoplasm, in which platelets are indeed produced in normal numbers, but they are functionally activated, causing disturbances apparently unusual for cancer. This requires confirmation in further studies.
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PMID:[Portal vein thrombosis as the main symptom of unclassified JAK2-positive myeloproliferative neoplasm--case report]. 2299 7

Portal hypertension is a clinical syndrome defined as a portal venous pressure that exceeds 10mmHg. Cirrhosis is the most common cause of portal hypertension and thrombosis of the splenoportal axis not associated with liver cirrhosis is the second cause of portal hypertension in the Western world. The primary myeloproliferative disorders are the main cause of portal venous thrombosis and somatic mutation of Janus Kinase 2 gene (JAK2 V617F) can be found in approximately 90% of polycythemia vera, 50% of essential thrombocyrosis and 50% primary myelofibrosis. A a 55-year-old man with JAK2 mutation-associated splenoportal axis hypertension and bleeding complications due to oesophageal varices is reported. A massive upper bleeding episode made an emergent surgery to be done immediatelly at seventh day. The patient was discharged home at fifteenth day after surgery.
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PMID:Massive upper gastrointestinal bleeding due to splenoportal axis thrombosis in a patient with a tested JAK2 mutation: A case report and review literature. 2769 37

Endoscopic variceal sclerotherapy and ligation are standard treatment modalities used for the management of esophageal varices. Reportedly, sclerotherapy and ligation are associated with complications such as hematuria, pulmonary thrombus formation, pleural effusion, renal dysfunction, and esophageal stenosis. However, hemothorax following sclerotherapy and ligation has not yet been reported. We treated a patient who presented with liver cirrhosis and polycythemia vera and later developed hemothorax following the above-mentioned procedures. An 86-year-old man diagnosed with liver cirrhosis due to chronic hepatitis type B and alcohol abuse underwent variceal sclerotherapy using ethanolamine oleate to treat his esophageal varices. Oozing from the esophageal varices continued even after the sclerotherapy procedure; therefore, we performed endoscopic variceal ligation. The patient developed left-sided hemothorax within 24 h after treatment of his varices, and an emergency thoracotomy was performed. A pulmonary ligament of the left lung was bulging and ripping because of mediastinal hematoma, and oozing was noted. Cessation of bleeding was noted after the laceration of the left pulmonary ligament had been sutured. Ours is the first case of hemothorax reported in a patient following an uncomplicated procedure of sclerotherapy and ligation.
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PMID:Hemothorax following Uncomplicated Endoscopic Variceal Sclerotherapy and Ligation for Esophageal Varices. 2903 74

In patients with liver cirrhosis the contribution of inherited and acquired prothrombotic disorders in the development of non-malignant portal vein thrombosis (PVT) is inconclusive. The purpose of this retrospective study was to examine the prevalence of thrombophilia in this setting at our center from January 2012 to November 2019. Tests included gene mutational analysis for Factor V Leiden, prothrombin G20210A, JAK2 (V617F), Calreticulin (CARL), in addition to activated protein C resistance, antithrombin III, protein C and S levels, and antiphospholipid antibodies. We included 77 patients, six of whom (7.8%) had a thrombophilic disorder: antiphospholipid syndrome in four patients, prothrombin gene mutation in one and factor V Leiden mutation in one. This latter patient had also been diagnosed with polycythemia vera years before PVT development. Complete thrombosis of the main portal vein and re-thrombosis after stopping anticoagulation were more frequent in patients with thrombophilia, but the rates of recanalization under anticoagulant therapy were similar among groups. No other difference was accounted between groups. The low prevalence of acquired and inherited thrombophilia found in patients with cirrhosis and PVT support testing for these disorders on an individual basis and avoiding universal screening to reduce costs and unwarranted testing.
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PMID:Portal Thrombosis in Cirrhosis: Role of Thrombophilic Disorders. 3287 64


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