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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixteen cases of chronic Q fever are described. In eight there was a history of exposure to infection from farms or farm products. All had valvular heart disease, involving the mitral valve in nine and the aortic valve in seven. Infection occurred on a prosthetic valve in two patients. Arterial embolism was common. Venous thrombosis occured in three patients, and pulmonary embolism occurred in three other patients. Complement fixing antibodies to phase 1 antigen were found in a titre of 1:200 or greater in all except two patients. In one of these post-mortem examination revealed rickettsial bodies in mitral valve vegetations, and in the other Coxiella burneti was isolated from heart valve tissue. The majority presented with infective endocarditis but two presented primarily with liver disease. All patients had evidence of liver involvement and in one this led to death from cirrhosis. Abnormal tests of liver function, particularly hyperglobulinaemia, raised alkaline phsophatase and abnormal bromsulphthalein retention were found in all patients. Hepatic histology was abnormal in all eight patients in whom it was studied. The commonest features were mononuclear cell infiltration of the portal tracts and prominence of the sinusoidal Kupffer cells. Patchy focal necrosis of parenchymal cells, granulomata, fatty change, and eosinophilia of the sinusoidal walls were also noted in several patients and cirrhosis developed in one. Six patients had a purpuric rash, and in 12 there was thrombocytopenia. It is suggested that the presence of hepatomegaly and liver involvement and thrombocytopenia may help to differentiate Q fever endocarditis from bacterial endocarditis. Raised serum IgM and IgA levels occured frequently, but with only a moderate dominance of IgM. Sheep cell agglutination and latex fixation tests for rheumatoid factor were occasionally positive. Several features of the disease suggest the possibility that immune-complex mechanisms may play a role in chronic Q fever. Treatment was with prolonged courses of tetracycline usually combined with lincomycin. Seven patients underwent valve replacement surgery for haemodynamic reasons. Five patients died; two from heart failure, one from cirrhosis, one seven days after valve replacement and one from intraperitoneal haemorrhage following percutaneous liver biopsy. Three patients have survived for more than five years, and another six for more than three and a half years after diagnosis. Of these nine patients, three received medical therapy alone and six required valve replacement as well. Antibiotics have been discontinued in four patients who have had valve surgery and three others. Six patients had received antibiotics for continuous periods varying from 29-62 months. In the period after stopping therapy varying from 15-21 months, no relapse has occured. A seventh patient, who had received antibiotics for four months prior to valve replacement, has survived 43 months after the withdrawal of antibiotics...
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PMID:Chronic Q fever. 94 Sep 18

Four infants with Lues connata, three with the early stage of the disease (patients 1-3), are reported. Diagnosis was made after exclusion of other diseases. Initially an infectious disease was expected, since anemia, leucocytosis, thrombocytopenia, hepatomegaly and/or splenomegaly and a bad condition were found. In two patients bone structure was abnormal. Elevated serum concentrations of liver enzymes (ALAT, ASAT) were the indication for liver biopsy in one patient, in whom an accompanying hepatitis was diagnosed. Treatment was performed with penicillin, no JARISCH-HERXHEIMER reaction was observed. The Lues tests were negative during pregnancy but a displacental transfer of pathogenic agents could be assumed. Patient 4 was diagnosed at 9 months of age. Infection of the mother probably occurred in the last 6 weeks of pregnancy. It can not be decided if the baby has a connatal or acquired Lues. The titer decrease of the CMT-test after the end of the penicillin therapy is a marker for a successful treatment. If treatment was started at 2 years of age a total clinical recovery can be expected. The case reports demonstrate that negative Lues test during pregnancy do not exclude Lues connata in newborns. The Lues diagnosis should be considered if an infectious disease in a newborn can not be diagnosed. A general Lues serodiagnostic test is recommended in all newborns before they leave the obstetrics department.
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PMID:[Congenital syphilis]. 130 79

The signs that may arise after perinatal infection with human immunodeficiency virus type 1 (HIV-1) have been classified by the Centers for Disease Control, but the clinical usefulness of the classification system and the prognostic importance of each disease pattern have not been established. We sought to address these issues by analysing data from the Italian Register for HIV infection in children. We studied 1887 children born to HIV-1-seropositive mothers. 1045 were identified at birth and the others were registered later (median age 4.8 [range 0.4-72] months). HIV-1-associated signs developed in 433 (81.8%) of 529 seropositive infected children at a median age of 5 (0.03-84) months. These signs appeared significantly earlier in the 102 children who died of HIV-1-related illness than in those who are still alive (median 3 [0.03-55] vs 6 [0.03-84] months; p less than 0.001). The cumulative proportion surviving at age 9 years was 49.5% (95% confidence interval 27-65%) and the median survival time was 96.2 months. Separate analysis of the 112 seropositive infected children followed from birth and older than 15 months gave similar results. Hepatomegaly, splenomegaly, lymphadenopathy, parotitis, skin diseases, and recurrent respiratory tract infections formed the mildest disease pattern. Lymphoid interstitial pneumonitis and thrombocytopenia were signs of intermediate disease. By contrast, in multivariate analysis specific secondary infectious diseases, severe bacterial infections, progressive neurological disease, anaemia, and fever were significant and independent negative predictors of survival. Growth failure, persistent oral candidosis, hepatitis, and cardiopathy were associated in univariate analysis with significantly shorter survival. Our findings suggest that the outlook for children with perinatal HIV-1 infection is better than previously thought and that a new clinical staging system of single disease patterns is needed.
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PMID:Prognostic factors and survival in children with perinatal HIV-1 infection. The Italian Register for HIV Infections in Children. 134 67

HIV is efficiently transmitted through transfusion with HIV-infected blood. Accordingly, 203 multitransfused children with thalassemia attending the thalassemia clinic of the Charak Palika Hospital in New Delhi were screened for antibodies to HIV using ELISA and Western blot tests. 8.37% of the sample tested HIV-seropositive (HIV+). These 17 children were joined by 3 others referred from a neighboring state to constitute a group to be matched against 20 HIV-children for the purpose of comparing psychosocial aspects. The control group was matched for age, sex, educational level, and socioeconomic status with mean age 10.8 years ranging over 1-16 years. 4 members of the HIV+ sample were diagnoses as having clinical AIDS according to WHO criteria. The remaining 14 boys and 2 girls were HIV+, but asymptomatic. 25% were of lower class, 63.5% middle class, and 12.5% upper class. Of those with AIDS, 50% were diagnosed in their first year of life and 82% were diagnosed by year 3. Symptoms generally developed after 4-6 months of life. Lymphadenopathy and hepatomegaly tend to be visible at birth, while chronic diarrhea, prolonged fever, oral thrush, recurrent bacterial infections, and hepatosplenomegaly may also be presented. 7.1% of cases aged 2-3 years exhibited rocking and head banging problems worse than did control subjects. Furthermore, 28.5% had temper tantrums and 21.5% ground teeth. These children may have delayed developmental milestones as well as behavioral problems. The small sample size, however, precludes concluding that psychosocial differences exist between those with HIV/AIDS and those with thalassemia major. In fact, behavioral problems in these children were due to child illness and not of HIV-positivity, for children tend to be unaware of HIV/AIDS infections and its consequences. The author recommends that HIV+ children continue to attend school unless they can not control bodily secretions, have uncoverable oozing lesions, have unacceptable behaviors, or if there is extreme possibility of contracting infectious diseases at school. The author also stresses parents' and families' need for long-term medical and psychological care.
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PMID:Psycho-social aspects of HIV infection and AIDS in multiple transfused thalassemic children. 145 60

Echinococcosis is an infectious disease of humans caused by the larval (metacestode) stage of the cestode species Echinococcus granulosus (cystic echinococcosis or hydatid disease) or Echinococcus multilocularis (alveolar echinococcosis or alveolar hydatid disease). Clinical manifestations depend primarily on localization and size of hepatic lesions and may include hepatomegaly, obstructive jaundice, or cholangitis. Prognostically, alveolar echinococcosis is considered similar to liver malignancies, including a lethality rate of 90% for untreated cases. Diagnosis is based on imaging techniques coupled with immunodiagnostic procedures. Antibody detection tests for E. multilocularis have markedly improved with the use of affinity-purified Em2 antigen and recombinant antigen II/3-10 in enzyme immunoassays. Antigens of corresponding quality for E. granulosus are still unavailable. The detection of circulating antigens and immune complexes in the sera of patients with cystic echinococcosis, the demonstration of in vitro lymphocyte proliferation in response to stimulation with Echinococcus antigens, and the discrimination of serum immunoglobulin isotype activity to various Echinococcus antigens in both cystic and alveolar echinococcosis have been suggested for diagnostic purposes as well as for monitoring patients after treatment. New diagnostic molecular tools include DNA probes for Southern hybridization tests and polymerase chain reaction for the amplification of E. multilocularis and E. granulosus species-specific DNA fragments.
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PMID:Molecular and immunological diagnosis of echinococcosis. 149 67

Fever can be recognized as a higher set-point of the normal temperature regulation which is controlled by the center in the anterior part of hypothalamus. The change in this set-point is induced by interleukin-1 (IL-1) which is the common mediator of exogenic and endogenic pyrogenic factors. IL-1 is believed to act through an induction of a prostaglandin E cascade. The normal diurnal variation in temperature can often be recognized in infectious diseases but not always in non-infectious conditions. Four different fever curves can be defined but are without differential diagnostic importance, however, septic fever curves are more likely to occur in bacteremic patients. Comparison of the most important investigations about PUO since 1960 shows that the follow-up investigations revealed a high percentage of undiagnosed cases and that the mortality due to conditions related to PUO was 6-8%. Among the other investigations, a total of 83% were diagnosed: 23% had cancer, 33% had infections, 11% had collagenic diseases, 17% had other causes and 16% were undiagnosed. To establish the diagnosis in cases of PUO, liver biopsy can be of diagnostic value especially in patients with hepatomegaly. Abdominal CT-scan, ultrasonography and Gallium 67 scintigraphy are equal in sensitivity and specificity and can supplement each other with diagnostic information. Leucocyte scintigraphy can detect local inflammatory processes. Laparotomy or laparoscopy have high diagnostic values and can be considered in patients with signs of involvement of abdominal organs if no diagnosis has been established after noninvasive investigations. Lymphography gives only limited diagnostic information in cases of PUO.
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PMID:[Fever of unknown origin (febris continua e causa ignota)]. 153 51

The medical records of 370 patients treated for typhoid fever between 1986 and 1988 at the Communicable Disease Centre, Singapore, were reviewed. The disease was generally mild. There was no mortality. Fever was found in 98.4% of patients on admission and diarrhoea in 21%. Cough was predominantly a symptom of children and occurred in 7.1% of patients aged below 15 years. Other symptoms were uncommon. Hepatomegaly was found in 71% and splenomegaly in 47%. Leucopenia was not a helpful diagnostic marker. Chloramphenicol was the drug of choice. The relapse rate was 5.4% and the convalescent and temporary carrier rates 11.6%. The risk of developing the carrier state was significantly higher among patients who were afebrile on admission compared with those who were febrile (P less than 0.001); it was also higher in patients treated with ampicillin as compared to those treated with chloramphenicol (P less than 0.001, chi 2 = 22.7, odds ratio = 5.25, 95% confidence limits: 2.46 and 11.29). The role of ampicillin as a first line treatment for acute typhoid fever may need further re-evaluation.
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PMID:Typhoid fever in Singapore: a review of 370 cases. 194 16

Patients with the acquired immune deficiency syndrome (AIDS) frequently develop hepatic dysfunction. Although hepatic injury may indirectly result from malnutrition, hypotension, administered medications, sepsis, or other conditions, the hepatic injury is frequently due to opportunistic hepatic infection, directly related to AIDS. Infection with Mycobacterium avium intracellulare typically occurs in patients with advanced immunocompromise and with systemic symptoms due to widely disseminated infection. In contrast, hepatic tuberculosis often occurs with less advanced immunocompromise. Cytomegaloviral infection may produce a hepatitis. Cytomegaloviral and cryptosporidial infections have been implicated as causes of acalculous cholecystitis and of a secondary sclerosing cholangitis. About 10-20% of patients with AIDS have chronic hepatitis B infection. These patients tend to develop minimal hepatic inflammation and necrosis. The clinical findings in patients with hepatic cryptococcal infection are usually due to concomitant extrahepatic infection. Hepatic histoplasmosis usually develops as part of a widely disseminated infection with systemic symptoms. Hepatic involvement by Kaposi's sarcoma is rarely documented ante mortem because an unguided liver biopsy is an insensitive diagnostic procedure. Patients with non-Hodgkin's lymphoma of the liver typically have lymphadenopathy, hepatomegaly, and systemic symptoms. As a pragmatic approach, patients with liver dysfunction and HIV-related disease should have a sonographic or computerized tomographic examination of the liver. Patients with dilated bile ducts should undergo endoscopic retrograde cholangiopancreatography because opportunistic infection may produce biliary obstruction. Patients with a focal hepatic lesion should be considered for a guided liver biopsy. Patients with a significantly elevated serum alkaline phosphatase level should be considered for a percutaneous liver biopsy. When performed for these indications, liver biopsy will demonstrate a significant disease involving the liver in about 50% of patients with AIDS and in about 25% of patients who are HIV seropositive but who are not known to have AIDS. The clinical impact of a diagnostic biopsy is blunted by a lack of efficacious therapy for many opportunistic infections.
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PMID:Hepatobiliary manifestations of the acquired immune deficiency syndrome. 198 33

We retrospectively studied the incidence of familial hemophagocytic lymphohistiocytosis (FHL) in children during the 16-year period 1971-86. First, all departments of pediatrics, pathology, and infectious diseases were enquired for children with FHL or disorders resembling FHL. Secondly, the causes of death of all children who died during the study period in Sweden (n = 19,542) were also investigated. Files and histological specimens were further studied in selected children. By using a set of inclusion/exclusion criteria, we found 32 children with FHL. The incidence was 1.2/1,000,000 children per year. One child per 50,000 live borns developed FHL during this period. The sex ratio was close to 1:1. Prominent early clinical signs were fever (91%), splenomegaly (84%), hepatomegaly (90%), rash (43%), and lymph node enlargement (42%). Neurological symptoms, which developed in 47%, could totally dominate the clinical picture and develop prior to other symptoms and signs. Common laboratory findings were pancytopenia, hypertriglyceridemia, hypofibrinogenemia, elevated serum transaminases, hyperbilirubinemia, hyponatremia, hypoalbuminemia, and a moderate spinal fluid pleocytosis. Chest X-ray often revealed mostly discrete pulmonary infiltrates. FHL is an underdiagnosed disease and in only 11/32 children was diagnosis made during their lifetime. It is important to be aware of the disorder as potential therapy now exists.
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PMID:Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. 205 92

From 1956 through 1989, 38 men and 26 women were seen at the Mayo Clinic with biopsy-proven AA. The underlying disorder was rheumatic disease in 42, infectious disease in 11, inflammatory bowel disease in 6, and other causes in 5. All patients were symptomatic at the time of diagnosis. Fifty-eight of the 64 patients had proteinuria or renal insufficiency. Fourteen also had significant symptoms of gastrointestinal amyloid, and 6 had amyloid goiter. None of the patients had symptomatic cardiac involvement, and only 3 had palpable hepatomegaly. Renal, gastric, rectal, fat, and marrow biopsies were positive for amyloid in 100%, 94%, 82%, 58%, and 46% of tested patients, respectively. The median survival of the entire group was 24.5 months. Thirty-five of the 47 deceased patients died as a direct result of their amyloidosis, primarily from complications of renal failure. Nine were successfully treated and had regression of the disease. Two with bronchiectasis responded to long-term cyclic antibiotic therapy, as did 1 patient with osteomyelitis. One patient with inflammatory bowel disease responded to surgical resection, and 1 with familial Mediterranean fever responded to colchicine. Four patients with rheumatic disease were treated with cyclophosphamide (in 2) and methotrexate (in 2), with complete resolution of their renal disease. All 9 successfully treated patients are alive, with a median follow-up of 58 months. Statistical analysis revealed that creatinine values greater than or equal to 2.0 mg/dl (P less than 0.003) and a serum albumin value less than 2.5 g/dl (P less than 0.02) were associated with a poorer survival. The single strongest variable associated with poor survival was a serum creatinine level greater than 2 mg/dl at presentation, with a median survival of 11.2 months compared to patients with a creatinine level less than 2.0 mg/dl, with a median survival of 56.9 months.
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PMID:Secondary systemic amyloidosis: response and survival in 64 patients. 206 9


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