Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Louse-borne relapsing fever (LBRF) is an acute febrile illness endemic Ethiopia. To date reports of childhood LBRF are few. The demographic, social and clinical features of eighty children with LBRF admitted to Ethio-Swedish Children's Hospital, Addis Abeba between 1989 and 1991 is presented. The mean age of patients was 8.8 years (range 4 months to 15 years). The male to female ratio was 1.2:1. Seventy-seven (97%) patients came from Addis Abeba. They came from poor families living in overcrowded homes. Fever, headache, right upper quadrant pain, chills and rigors were common symptoms. Fever and hepatosplenomegaly were common signs. Three drug regimens were used in the treatment of patients. A combination of penicillin and tetracycline, chloramphenicol alone and erythromycin alone, all given for 3 days. There was only one death. The literature on LBRF in adults is reviewed and the results are compared (1).
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PMID:Relapsing fever in children--demographic, social and clinical features. 145 20

A 16-Year-old boy with lymphoblastic lymphoma underwent an autologous bone marrow transplantation (ABMT) after conditioning with high-dose busulfan and cyclophosphamide. On day 39 post-transplant, right upper quadrant pain occurred with an increase in the size of the liver. Liver function tests showed a subsequent deterioration. Ultrasonographic studies of the abdomen disclosed hepatosplenomegaly, ascites, thickening of the gall bladder wall and a failure to visualize the major hepatic veins. The venocclusive disease of the liver (VOD) diagnosis was confirmed from these findings. Ultrasonographic monitoring reflected the disease status well and demonstrated a complete recovery from the VOD. We emphasize, thus, that abdominal ultrasonography can be applied easily, being a non-invasive procedure, and is useful in diagnosing VOD. Furthermore, the procedure can be repeated serially for evaluating the severity of VOD. Although engraftment was confirmed with granulocytes exceeding 500/microliters, platelet recovery was delayed; the megakaryocytes had not decreased in bone marrow aspirates and platelet-associated IgG was significantly elevated. Since autoimmune thrombocytopenia was highly suspected, to prevent immunosuppression danazol was given as an immune modulator instead of prednisolone, and a complete recovery was obtained. Accordingly, danazol can be used as an alternative to prednisolone for the treatment of autoimmune thrombocytopenia after bone marrow transplantation.
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PMID:Sonographic diagnosis of venocclusive disease of the liver and danazol therapy for autoimmune thrombocytopenia in an autologous marrow transplant patient. 219 89

Hemobilia is a hemorrhage into the biliary tract that may follow surgical trauma, liver biopsy, aneurysms, extra- or intra-hepatic tumors of the biliary tract, gallstones, and inflammatory lesion of liver, especially helminthic or pyogenic. Sometimes, it is associated with primary liver cancer. An 84 year-old woman was admitted because of continuous right upper quadrant pain 4 days before admission. Physical examination revealed decreased skin turgor, icteric sclerae and severe tenderness on right upper quadrant abdomen. She had no hepatosplenomegaly, and no rebound tenderness. She has been taking warfarin for 3 weeks before admission because of atrial fibrillation. On admission, serum bilirubin and transaminase were elevated. The level of hemoglobin and hematocrit were 11.3 g/dL and 37.4%, respectively. HBsAg was negative, but IgG anti-HBc and anti-HBs were positive and anti-HCV was negative. Parasite skin test and stool ova count demonstrated non-specific findings. Stool occult blood was strongly positive, and prothrombin time was markedly prolonged. According to endoscopic retrograde cholangiopancreatography, common bile duct was dilated, and filled with blood clot but there was no stone in bile tree. After two weeks, serum transaminase, bilirubin, hemoglobin, hematocrit, and CA19-9 were normalized. We report a case of hemobilia, occurring in a patient with continuous warfarin use.
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PMID:[A case of non-traumatic hemobilia due to warfarin therapy]. 1556 10

Disseminated infections with hemophagocytic syndrome caused by non-tuberculous mycobacteria (NTM) are rare. A 60-year-old woman, who presented with fever, chills, anorexia, and right upper quadrant pain, was admitted to our hospital. Hepatosplenomegaly, pancytopenia, elevated liver aminotransferases, and hyperferritinemia were noted after admission. A gallium scan and chest computed tomography revealed multiple mediastinal lymphadenopathy. A bone marrow examination revealed hemophagocytosis. Bone marrow and liver biopsies showed non-caseating granulomatosis and cultures from bone marrow and liver all yielded Mycobacterium kansasii. The patient responded well to azithromycin, isoniazid, rifampin, and ethambutol. No immunocompromised conditions such as malignancy, autoimmune disease, or HIV infection were detected initially. However, a right femoral tumor with pathological fracture was found five months later during follow-up at the outpatient clinic. Bone biopsy showed granulocytic sarcoma. To our knowledge, this is the first reported case of life-threatening hemophagocytosis due to M. kansasii. Patients with disseminated NTM infections should be closely monitored if any immunocompromising condition develops.
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PMID:Disseminated Mycobacterium kansasii infection associated with hemophagocytic syndrome. 1965 7

A 42-year-old white woman, who was a general practitioner referral to the medical team, presented with a 3-day history of left upper quadrant pain; an urgent private ultrasound scan had showed splenomegaly. She was initially admitted with sepsis without an obvious cause but with a differential diagnosis of a haematological malignancy. Her admission blood tests showed a mildly reduced white cell count and low platelets. Her symptoms progressed and she developed right upper quadrant pain. Her blood counts deteriorated showing a disseminated intravascular coagulation (DIC) picture and mildly deranged liver function tests. Blood films were non-diagnostic. A CT scan of the abdomen/pelvis showed splenomegaly and also hepatomegaly and ascites, not seen in her initial ultrasound scan. Multiple cultures of blood/urine/ascites and infective serology were unremarkable.She was transferred to a larger tertiary centre under the care of the surgeons with presumed abdominal sepsis and underwent an open laparotomy, which showed a big firm liver and spleen but no obvious cause for sepsis. The infectious disease team were unable to find a cause, and haematology became involved to investigate the possibility of a haematological malignancy. The patient underwent two bone marrow biopsies, a percutaneous liver biopsy and had flow cytometry of her ascitic fluid, which revealed the diagnosis of a natural killer cell leukaemia. After some slight improvement on steroids, the patient was given cyclophosphamide, doxorubicin, vincristine, prednisone, rituximab (CHOP-R) chemotherapy. The patient had an initial response to chemotherapy, with reduction in ascitic volume and hepatosplenomegaly, and normalisation of her coagulation. This was accompanied by an overall improvement in her physical condition. She had a second cycle of CHOP-R, but unfortunately approximately 2 weeks after that, she deteriorated rapidly. She was too weak for salvage chemotherapy, so she was put on comfort care. She died peacefully.
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PMID:Natural killer cell leukaemia. 2188 53

Infection with the Epstein-Barr virus (EBV) is a common disease and is mainly asymptomatic during childhood, whereas infectious mononucleosis with clinical signs such as fever, pharyngitis, lymphadenopathy and hepatosplenomegaly often occurs in adolescents and adults with primary infection. Acalculous cholecystitis has been reported as a rare complication. We report herein a case of acalculous cholecystitis accompanied by infectious mononucleosis by EBV, which was treated successfully by medical treatment. A 33-year-old woman who had been admitted by fever, pharyngitis and lymphadenopathy developed a right upper quadrant pain, that was diagnosed as acalculous cholecystitis based on an imaging study. Antibiotic treatment did not resolve the symptoms, and surgical intervention was considered. We diagnosed her as having infectious mononucleosis based on a typical physical presentation and seropositivity for the EBV viral capsid antigen, suggesting that the acalculous cholecystatis might have been a complication of the EBV infection. After the administration of glucocorticoid and acyclovir, the patient became afebrile and the abdominal pain disappeared. Though acalculous cholecystitis rarely accompanies infectious mononucleosis caused by EBV, clinicians should be aware of this complication to avoid unnecessary cholecystectomy.
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PMID:[A Case of Acute Acalculous Cholecystitis During Infectious Mononucleosis Caused by the Epstein-Barr Virus in a Young Woman]. 2752 70

We report a case of Budd-Chiari syndrome occurring in a patient with coeliac disease, who presented with symptoms of increased abdominal girth, right upper quadrant pain and shortness of breath for three weeks prior to admission. Initial assessment revealed the presence of moderate ascites, hepatosplenomegaly and right-sided pleural effusion. Further diagnostic work-up established a diagnosis of chronic Budd-Chiari syndrome. Interestingly, complete screening for pro-thrombotic factors was negative. A review of the literature on this association disclosed only 28 similar cases, with the majority of them describing individuals of North African origin. Interestingly, in the majority of cases no specific thrombotic factor could be identified, suggesting that coeliac disease may play a role in this thrombotic disorder.
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PMID:Budd-Chiari syndrome associated with coeliac disease: case report and literature review. 2760 77