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)

The clinical and immunological features of fifteen cases of cryptogenic pulmonary eosinophilia are reported. There were ten women (mean age 35.4 years) and five men (mean age 42 years). Eight gave a previous history of asthma and seven had none. Thirteen of the fifteen patients had negative skin test to common allergens. Many features of a systemic illness were present in the asthmatic and non-asthmatic groups including anaemia, weight loss, fever and a grossly raised ESR. An absolute polymorphonuclear leucocytosis was frequent as well as the obligatory increase in blood eosinophils used as one of our criteria for inclusion. Hepatomegaly (three cases), splenomegaly (four cases) and hilar node enlargement (one case) were seen in the group without asthma. Evidence of renal involvement or necrotizing vasculitis was notably absent and the response to small doses of corticosteroids was dramatic. Immunologically the striking feature was a disproportionate increase in blood eosinophils compared with only minor elevations in the total serum IgE levels. This stands in contrast to patients with bronchopulmonary aspergillosis and helminth infestation. Studies of cytophilic antibodies using histamine liberation after challenge with antibodies to immunoglobulin sub-classes in six patients showed a marked increase in IgG2 and lesser increases of IgE and IgG3. No evidence of antibodies specific to A. fumigatus was found. The amount of cytophilic antibody was also in contrast to that found in bronchopulmonary aspergillosis.
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PMID:Cryptogenic pulmonary eosinophilia. 5 41

The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS.
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PMID:Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. 179 57

Liver biopsies of a 58-year-old clinically healthy patient with a hepatomegaly and intracisternal PAS-negative globular hyaline bodies were immunofluorescent-optically examined for the content of the complement components C 1 q, C 4, C 9, C 1-inactivator, C 3-activator. Further examinations were performed for fibrinogen, IgG, IgA, IgM, IgD, IgE, L-chain (type chi and lambda), alpha 1-antitrypsin, alpha 1-fetoprotein, alpha 1- and alpha 2-glycoprotein, cholinesterase, ceruloplasmin, myoglobin, hemopexin, HBsAg and HBsAg. Th inclusion bodies reacted with antisera against the complement components C 4, C 3 and C 3-activator, as also identified by double immunofluorescence. Probably this is a disturbance of the protein metabolism of the liver cell with abnormal complement storage in the presence of normal total complement and normal complement components in the serum.
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PMID:Storage of the complement components C4, C3, and C 3-activator in the human liver as PAS-negative globular hyaline bodies. 628 41

The prognosis of the hypereosinophilic syndrome (HS) depends mainly on the development of endomyocardial fibrosis (EMF). This complication may be overlooked at an early stage, although its presence is an indication for steroid or antimitotic therapy of the HS. Even at an advanced EMF and associated intracardiac thrombi may not be visualised by angiography. This study was undertaken to assess the diagnostic value of 2D echocardiography in 12 patients. The patients were all men (12 of them) aged 22 to 64 years with unexplained eosinophilia 1 500/mm3 for over 6 months, and visceral lesions. The patients were divided into 3 clinical groups. Group A comprised 4 "allergic" patients with chronic asthma and a significant elevation of IgE; Group B comprised 5 "myeloproliferative" patients with splenomegaly and/or hepatomegaly and a significant elevation of serum B12 levels. The 3 remaining patients who could not be allocated to either Group A or B formed the third group (Group C). 2D echocardiography was carried out on average 30 months after diagnosis of the HS and six planes of examination were used systematically (two parasternal, two apical, one extreme apical and one subcostal). Right and left ventriculography was performed in 6 patients (less than one month before or after 2D-echo). Anatomical studies were obtained in 4 cases (2 operations, 3 autopsies). Echocardiographic signs of EMF were observed in 8 cases. Four patients had a restrictive cardiopathy associated to a large LV thrombus in 2 cases.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cardiac manifestations of the hypereosinophilic syndrome. The value of 2-dimensional echography (12 cases)]. 643 27

Clinical gastro-intestinal manifestations were studied in 34 patients in the initial phase of schistosomiasis mansoni. The patients, all men, were of similar age and in similar nutritional condition and had been infected simultaneously at the same transmission site. Most (85%) showed some gastro-intestinal sign or symptom, generally of light or moderate intensity; 56% had liquid or pasty diarrhoea, 41% abdominal pain, 29% hepatomegaly, 21% dysentery, 15% anorexia, 12% pain on colon palpation and 9% nausea and/or vomiting. High worm burden was associated with blood in faeces but apparently not with any other clinical manifestation. There was no apparent association between any clinical manifestation and peripheral-blood eosinophil counts or titres of IgE specific for Schistosoma mansoni (evaluated by the area of immediate intradermal reaction to injected adult worm antigen). The absence of association between worm burden and nearly all the clinical gastro-intestinal manifestations strengthens the concept that factors other than worm burden, such as host reactivity, constitute important pathogenetic elements in the initial phase of schistosomiasis mansoni.
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PMID:Gastro-intestinal manifestations of the initial phase of schistosomiasis mansoni. 766 18

Schistosoma mansoni was introduced in the Richard Toll area (Senegal) around 1988, probably due to man-made ecological changes in the Senegal river basin. Since 1991, we investigate the community of Ndombo, close to Richard Toll. Four random population samples of approximately 400 subjects are surveyed, starting at 8 months intervals. Each cohort is examined parasitologically (Kato-Katz), clinically, serologically (circulating antigen and antibody profiles); treated with praziquantel 40 mg/kg; and followed up 6-12 weeks, 1 and 2 years after treatment. Water contact patterns and snail densities are longitudinally surveyed. In the first cohort, prevalence of infection was 91%, with 41% excreting over 1000 eggs per gram (epg); the mean egg count was 646 epg, individual counts up to 24,000 epg. Prevalences remained almost 100%, but egg counts declined strongly in adults, in spite of continued exposure and the supposed lack of acquired immunity. Antigen detection in serum and urine confirmed that the egg counts genuinely reflect variations of worm burdens. Serum circulating anodic antigen (CAA) provided intriguing epidemiological information on worm burdens, while circulating cathodic antigen (CCA) showed promise for non-invasive diagnosis and screening. So far, similar epidemiological results were found in subsequent cohorts, although some variations were observed, possibly due to seasonal transmission fluctuations. IgE levels increased with age, while IgG4 peaked in the age-group 10-19 years. IgE and IgG4-levels against adult worm antigen (AWA) and soluble egg antigen (SEA) increased between cohort 1 and cohort 3 in almost all age-groups. In all 3 cohorts examined so far a strong correlation between IgG4 and pre-treatment egg-load was observed. Further follow-up and analysis, and comparison with chronically infected populations will provide insight in the development of acquired immunity. Abdominal discomfort was reported by 61% and diarrhoea by 33% of the subjects in the first cohort; mild hepatomegaly was found in 16%, splenomegaly in 0.5%. There was no correlation between frequency of symptoms and egg counts. This low morbidity, in spite of intense infections, was confirmed by ultrasound, and may be due to the recent nature of the focus. In the first cohort, 82% of treated subjects still excreted eggs 12 weeks after treatment, though egg counts declined strongly. Antigen detection confirmed these results. Parasitological negativation rates in subsequent cohorts, followed up sooner after treatment, improved but remained remarkably low. The low drug efficacy may be due to very rapid reinfection (though further reinfection after one year was limited), and/or to the lack of immunity in the population. Reduced susceptibility of the local schistosome strain can not be excluded, however. Praziquantel treatment provoked impressive but transient side effects (colics, vomiting, urticaria, oedema), the frequency of which correlated with intensity of infection.
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PMID:Epidemiology, immunology and chemotherapy of Schistosoma mansoni infections in a recently exposed community in Senegal. 782 23

A cohort analysis was performed in Ndombo, Senegal, a community of about 4000, in the epicenter of the schistosomiasis outbreak. Four randomly selected cohorts of +or- 400 subjects were surveyed. Each cohort was examined parasitologically, clinically, and serologically (circulating antigen and antibody profiles); treated with praziquantel 40 mg/kg; and followed up at 6-12 weeks and at 1 and 2 years after treatment. The first cohort numbered 422 individuals, of which 91% had positive egg counts, with a mean egg count of 663 eggs per gram feces (epg). Quantitative egg counts in those aged 10-14 were 1409 epg and then declined to 632 epg in the age group 20-29 and to 266 epg in the age group over 40. In cohorts 2 and 3, examined in the spring and autumn, egg counts were substantially lower, particularly in adults, as compared with cohorts 1 and 4, which were both examined in the summer season. 94% of the subjects were positive in the serum circulating anodic antigen (CAA) ELISA, 83% in the serum CAA ELISA, and 95% in the urine circulating cathodic antigen (CCA) ELISA; CAA in urine was less sensitive, and was negative in half of the urine samples. Positivity rates for all assays increased with rising egg counts, and circulating antigen concentrations in both serum and urine correlated well with egg counts. IgE showed a significant increase with age, while IgG4 peaked in the age groups 10-15 and/or 15-19 years. A strong correlation between IgG, IgGl, and IgG4 against both crude antigens with pretreatment egg load was observed. Of the subjects in the first cohort, 61% reported abdominal pain, 33% diarrhea; only 16% showed mild hepatomegaly and only a few children had mild splenomegaly. In the first cohort, 82% of 298 reexamined subjects were still positive for S. mansoni 12 weeks after treatment with praziquantel 40 mg/kg. One year after treatment, cohort 1 showed mean egg counts in children (5-19 years) at 358 epg as compared with 1188 epg pretreatment.
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PMID:Immuno-epidemiology of Schistosoma mansoni infections in a recently exposed community in Senegal. 853 70

Specific IgG4 and IgE responses to adult worm antigen and soluble egg antigen (SEA) were examined in 267 individuals from an area in which schistosomiasis mansoni is endemic. Based on information obtained from clinical and sonographic examinations of this sample, the individuals were divided in three groups: 1) 204 individuals without periportal fibrosis, and liver and spleen enlargements; 2) 41 individuals without periportal fibrosis, but presenting with organopathy, with or without organomegaly; and 3) 22 individuals with periportal fibrosis, regardless of their status as having hepatomegaly and/or splenomegaly. Levels of IgG4 to SEA were significantly higher in sera from patients with fibrosis compared with the patients from the other two groups. We also found significantly higher levels of IgG4 against SEA in egg-negative patients with fibrosis compared with egg-negative patients from the other two groups. This report demonstrates a specific humoral response in patients presenting with initial fibrosis, a form of schistosomiasis transient between intestinal and severe hepatosplenic.
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PMID:High levels of IgG4 to Schistosoma mansoni egg antigens in individuals with periportal fibrosis. 1220 88

A 63-year-old man was admitted because of general malaise, fever, headache, generalized lymphadenopathy and hepatomegaly in July 2002. He was diagnosed as having multiple myeloma (MM) (IgG-kappa type) with atypical plasma cells in the bone marrow, lymph nodes and cerebrospinal fluid. Systemic and intrathecal chemotherapy were effective. Because of an increase of polyclonal IgE, electrophoretic patterns revealed an M-peak which was not as sharp as that in IgG myeloma. IgE production is not impaired by the pathologic process in MM patients.
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PMID:[Multiple myeloma (IgG-kappa) infiltrating central nervous system, lymph nodes, liver, and kidneys, and with elevation of IgE]. 1519 53

Symptoms of human leishmaniasis range from subclinical to extensive systemic disease with splenomegaly, hepatomegaly, skin lesions, anemia and hyperglobulinemia, but the basis of this variation is unknown. Association of progression of the disease with Th2 lymphocyte response was reported in mice but not in humans. As most genetic studies in Leishmania major (L. major)-infected mice were restricted to skin lesions, we analyzed the symptomatology of leishmaniasis in mice by monitoring skin lesions, hepatomegaly, splenomegaly and seven immunological parameters. We detected and mapped 17 Leishmania major response (Lmr) gene loci that control the symptoms of infection. Surprisingly, the individual Lmr loci control 13 different combinations of pathological and immunological symptoms. Seven loci control both pathological and immunological parameters, 10 influence immunological parameters only. Moreover, the genetics of clinical symptoms is also very heterogeneous: loci Lmr13 and Lmr4 determine skin lesions only, Lmr5 and Lmr10 skin lesions and splenomegaly, Lmr14 and Lmr3 splenomegaly and hepatomegaly, Lmr3 (weakly) skin lesions, and Lmr15 hepatomegaly only. Only two immunological parameters, IgE and interferon-gamma serum levels, correlate partly with clinical manifestations. These findings extend the paradigm for the genetics of host response to infection to include numerous genes, each controlling a different set of organ-specific and systemic effects.
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PMID:Genetics of susceptibility to leishmaniasis in mice: four novel loci and functional heterogeneity of gene effects. 1651 55


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