Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tangier disease is a rare, autosomally-inherited disorder of lipoprotein metabolism characterized by absence or marked deficiency of normal high density lipoprotein (HDL) cholesterol in plasma resulting in the accumulation of cholesteryl esters in various organs. The patient was a 55-yr-old male diagnosed as Tangier disease 16 years before. He had angina on exercise and his coronary angiogram revealed triple vessel disease including left main trunk (LMT) lesion. Stenosis of the right coronary artery was treated by percutaneous transluminal coronary angioplasty (PTCA). He was scheduled for a MIDCAB for further PTCA to be performed to relieve the stenosis of LMT. Preoperative laboratory data and physical examination showed total cholesterol 36 mg.dl-1, HDL-cholesterol 2 mg.dl-1, apoprotein A-I not-detected, pancytopenia, hyperplastic orange tonsils, splenomegaly and hepatomegaly. Clonidine 0.225 mg was orally given as a preanesthetic medication. Anesthesia was induced with fentanyl and midazolam and maintained with propofol, sevoflurane and supplemental fentanyl. Nitroglycerin and diltiazem were infused continuously. ST segment was elevated transiently during the clamping of the left anterior descending branch. Hemodynamic parameters were stable during the operation. He was extubated 2 hours after the end of the operation. No significant changes were found in postoperative EKG, total cholesterol, HDL-cholesterol and triglyceride. Perioperative course was uneventful.
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PMID:[Anesthesia for MIDCAB (minimally invasive direct coronary bypass) in a patient with Tangier disease: a case report]. 1079 27

We report a case of Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) with clonal karyotype abnormality. A 5-year-old boy was admitted to our hospital with persistent high-grade fever, hepatomegaly, and pancytopenia. Laboratory data disclosed a coagulation abnormality and severe liver damage. Clonal proliferation of EBV-infected cells was detected in the bone marrow by Southern hybridization, and bone marrow cells exhibited clonal chromosomal abnormality. Although the patient was treated with immunochemotherapy according to the HLH94 protocol, the disease recurred during the induction therapy, and the patient died of disseminated intravascular coagulopathy. Considering this aggressive and fatal clinical course, it is important to take intensive therapeutic measures if karyotype abnormality is noted in the treatment of EBV-HLH patients.
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PMID:Fatal Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with clonal karyotype abnormality. 1084 33

Multicentric Castleman's disease (MCD) is a lymphoproliferative disorder that can be defined based upon both clinical and pathological characteristics. The clinical features of this frequently fatal disease include fever, generalized lymphadenopathy, fatigue, splenomegaly, hepatomegaly, and pancytopenia. Recently, severe forms of this disease have been diagnosed in HIV positive patients. Human herpesvirus type 8 (HHV-8) DNA sequences have been detected in peripheral blood mononuclear cells (PBMCs) of patients with Kaposi's sarcoma and MCD, regardless of HIV infection status. Treatment and outcomes in HIV associated MCD are generally unfavorable. We recently treated two HIV-positive patients diagnosed with aggressive MCD with daily oral etoposide (50 mg). The first patient had relapsed on several occasions despite previous therapy with doxil, paclitaxel, and oral ganciclovir. The second patient was treatment naive. Both patients had HHV-8 detectable by polymerase chain reaction in PBMCs, widespread tumor, and B-type symptoms when therapy was initiated. In both cases remissions (documented by computerized tomography) have been durable, 1.5 and 6 months, respectively, with minimal side effects. Oral etoposide may be a safe, tolerable, and active agent in MCD.
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PMID:Treatment of HIV-associated multicentric Castleman's disease with oral etoposide. 1142 Dec 97

Persistent fever with pancytopenia and hepatomegaly with negative blood cultures and no obvious focus of infection in a child with Down syndrome should arouse a suspicion of leukemia. Bone marrow examination and clot biopsy from one such patient revealed hemophagocytosis and granulomas, with serologic evidence of recent Epstein-Barr virus infection. Bone marrow granulomas are not a feature of Epstein-Barr infection. Later, bone marrow culture and repeat blood culture grew Salmonella typhi. Thus, in a febrile child, when performing a bone marrow aspirate, a clot biopsy and culture for infectious etiology may be helpful, even when leukemia is strongly suspected.
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PMID:Hemophagocytosis and granulomas in the bone marrow of a child with Down syndrome. 1190 10

The clinical case of one patient with fever of unknown origin, due to granulomatous hepatitis of tuberculous etiology was presented. The patient was a a 50-year-old woman, with 50 days illness characterized by chills, 39 degrees C fever and heavy diaphoresis. She had a record of seven malaria cases. She looked thin and pale at the initial physical examination. During the evolution, she developed pancytopenia, massive hepatosplenomegaly, jaundice, and anasarca. The patient underwent screening tests for infection, neoplasias, collagenosis, and granulomatous diseases. The laboratory tests showed transaminase-alkaline phosphatase dissociation, which led to the final diagnosis of tuberculosis, through the histological examination of the liver parenchyma. The specific treatment against tuberculosis caused remission of fever, ascites, and hepatomegaly and normalization of liver tests, with satisfactory clinical evolution.
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PMID:[Granulomatous tuberculous hepatitis as cause of fever of unknown origin]. 1252 48

We report two patients with isolated bone marrow non-Hodgkin's lymphoma and we review the additional 23 cases thus far described. Bicytopenia or pancytopenia and an elevated erythrocyte sedimentation rate were universally present. Some 84% of the patients manifested fever of unknown origin (FUO), 64% constitutional symptoms, 48% hepatomegaly, and 52% splenomegaly. Of the variety of therapeutic protocols used, corticosteroids, alone or in combination, appeared to be the most effective. Survival ranged from 1 week to 4.5 years. Early diagnosis would avoid unnecessary work-up in patients with FUO and enable optimization of therapy.
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PMID:Isolated bone marrow non-Hodgkin's lymphoma. Report of two cases and literature review. 1271 30

An unusual case of hepatosplenic gamma delta T-cell lymphoma with leukemic phase in a 39-year-old woman is reported. At the first presentation she had splenomegaly and pancytopenia diagnosed as hypersplenia treated by splenectomy. Subsequently, she developed hepatomegaly and progressive neoplastic lymphocytosis. The bone marrow showed a sinusoidal infiltrate of medium-sized cells. Flowcytometric analysis of peripheral blood mononuclear cells demonstrated expression of CD3, CD7, CD16, CD56 antigens and T-cell receptor gamma delta. A monoclonal TCR gamma- and beta-chain gene rearrangement were detected by polymerase chain reaction. The patient was treated by traditional chemotherapy and alpha-interferon, unsuccessfully. Therefore, 2-chlorodeoxyadenosine was introduced resulting in a complete remission for 6 months. The reported case demonstrates the usefulness of 2-chlorodeoxyadenosine in treatment of hepatosplenic gamma delta T-cell lymphoma.
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PMID:Hepatosplenic gamma delta T-cell lymphoma with leukemic phase successfully treated with 2-chlorodeoxyadenosine. 1280 28

The aim of the paper is to present a case of a 5-year-old boy hospitalised because of recurrent high temperature and reddish blue infiltration of skin and hypodermis lasting 3 months. Because of pancytopenia and hepatomegaly, we suspected a proliferative disease of the haematopoietic or lymphatic system. Several bone marrow biopsies, did not show neoplastic changes. Serological tests excluded virus and animal borne infections. In the histopathologic examination of skin and hypodermis biopsy there was an inflammatory infiltration of the adipose tissue without features of vessel inflammation (the Weber-Christian syndrome). Al though steroid therapy was applied, the infiltration of the adipose tissue did not regress, while liver dysfunction symptoms intensified and pancytopenia and fever continued. The patient died with symptoms or respiratory and circulatory failure. This case of a 5-year-old boy is presented because of extreme diagnostic difficulties, which occurred at each phase of investigations. The repeated tests, as well as several verifications of the sampled histopathological material did not bring any unequivocal proof of the postulated diagnosis.
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PMID:[5- year old boy with Weber-Christian Syndrome or histiocytic cytophagic panniculitis? Diagnostic difficulties. Case presentation]. 1573 95

Although aggressive public health measures have greatly reduced the number of brucellosis cases in the United States, there is a resurgence of interest in this worldwide zoonosis because of its potential as a bioweapon and its 8-fold higher incidence in California, Texas, and the other borderlands between the United States and Mexico compared with the national rate. Accordingly, we reviewed the clinical records of 28 patients diagnosed at a university hospital in San Diego, CA, between 1979 and 2002 to look for new epidemiologic trends and to test the hypothesis that there are species-specific differences in clinical presentations. In contrast to the latest California-wide study completed in 1992, Brucella abortus infections were more common (73%) than Brucella melitensis after 1992, and women were more commonly infected (77% compared with 39%) than men. Major risk factors remained Hispanic ethnicity, travel to Mexico, and ingestion of nonpasteurized dairy products. Analysis of diagnostic procedures suggested that the traditional practice of prolonged incubation of blood cultures increased their sensitivity for Brucella, even in automated radiometric systems. Direct comparison of the clinical manifestations of infections with B. abortus and B. melitensis strongly supported differences in acute presentations. B. melitensis presented more acutely as fevers of unknown origin with statistically significant higher rates of abdominal tenderness, hepatomegaly, splenomegaly, thrombocytopenia, pancytopenia, and hepatic dysfunction. These results suggest that the epidemiology of brucellosis in California may be evolving, and they show, to our knowledge for the first time in a single series, that species-specific differences in presentations may account for some of the protean manifestations of brucellosis. Familiarity with manifestations of brucellosis and the optimal laboratory techniques for its diagnosis could help physicians protect the public against this reemerging, under-recognized zoonosis.
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PMID:Brucellosis in San Diego: epidemiology and species-related differences in acute clinical presentations. 1587 7

Before the mid-1980s, haemophilia often was unknowingly treated with contaminated plasma products, resulting in high rates of human immunodeficiency virus (HIV-1), hepatitis C virus (HCV) and hepatitis B virus (HBV) infections. To estimate the impact of these infections, a new cohort was established. All HCV-seropositive patients, age 13-88 years, at 52 comprehensive haemophilia treatment centres were eligible. Cross-sectional data collected during April 2001 to January 2004 (median June 2002) were analysed. Plasma HIV-1 and HCV RNA were quantified by polymerase chain reaction. Highly active antiretroviral therapy (HAART) was defined as use of at least three recommended medications. Among 2069 participants, 620 (30%) had HIV-1. Of 1955 with known HBV status, 814 (42%) had resolved HBV and 90 (4.6%) were HBV carriers. Although 80% of the HIV-1-positive participants had > or = 200 CD4+ cells microL(-1), only 59% were on HAART. HIV-1 RNA was undetectable in 23% of those not taking antiretroviral medications. Most (72%) participants had received no anti-HCV therapy. HCV RNA was detected less frequently (59%) among participants treated with standard interferon plus ribavirin (P = 0.0001) and more frequently among HIV-1-positive than HIV-1-negative participants (85% vs. 70%, P < 0.0001). HIV-1-positive participants were more likely to have pancytopenia and subclinical hepatic abnormalities, as well as persistent jaundice, hepatomegaly, splenomegaly and ascites. HAART recipients did not differ from HIV-negative participants in the prevalence of ascites. The clinical abnormalities were more prevalent with older age but were not confounded by HBV status or self-reported alcohol consumption. Eleven participants presented with or previously had hepatocellular carcinoma or non-Hodgkin lymphoma. Although prospective analysis is needed, our data reveal the scale of hepatic and haematological disease that is likely to manifest in the adult haemophilic population during the coming years unless most of them are successfully treated for HIV-1, HCV or both.
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PMID:Prevalence of conditions associated with human immunodeficiency and hepatitis virus infections among persons with haemophilia, 2001-2003. 1612 97


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