Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although rather uncommon and multifactorial in etiology, liver cirrhosis is a severe and often rapidly fatal disease in pediatrics. In our institution, during the last 15 years, 22 children with liver cirrhosis have been followed. The underlying predisposing condition was HBV infection (8 cases), CMV perinatal infection (2 cases), Wilson's disease (4 cases), chronic cholestasis (2 cases) and alcohol abuse (2 cases); in 4 cases no predisposing condition was evident. In all cases the histological examination of the liver was the diagnostic cornerstone. The mean age at diagnosis was 6 years and 8 months, with an early onset especially in the posthepatitis cirrhosis. In 10 out of 22 patients, cirrhosis was not preceded by an history of chronic liver disease. Poor subjective symptomatology was present in 13 of the cases, hepatomegaly in all, splenomegaly in 18 cases, signs of hepatic failure in 13 cases. In all patients various impairments of hepatocellular synthesis were detectable, especially during the period preceding the development of hepatic insufficiency. The mean time to cirrhosis was 5 years. The average duration of the follow up was 3 years and 4 months: during the follow up 6 patients improved, 5 patients showed no clinical or functional modifications of their hepatic disease, 3 patients worsened and 8 died. In order to perform suitable treatment of liver cirrhosis the need of early diagnosis and etiological definition should be emphasized.
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PMID:[Liver cirrhosis in childhood. Considerations on 22 cases with different etiology]. 217 33

The prevalence of splenomegaly associated with portal hypertension was examined in a consecutive population of 111 patients who had portal hypertension diagnosed using specific endoscopic, sonographic, and Doppler signs. Splenic size was measured objectively via its cranio-caudal length on coronal section using ultrasound and by clinical examination. Sonographically, 52% of patients had a definitely large spleen and 35% a spleen less than one standard deviation from the normal mean, while a further 13% had equivocal splenomegaly. Only 52% of patients had splenomegaly on clinical assessment. Splenomegaly was less common in patients with alcoholic (41% definite, 15% equivocal) than in those with non-alcoholic liver disease (66% definite, 17% equivocal, p = 0.02) and splenic length was significantly smaller in alcoholic patients (12.7 +/- 0.5 cm) compared to patients with either non-alcoholic liver disease (15.0 +/- 0.6 cm, p = 0.003) or portal hypertension due to vascular occlusive diseases (16.5 +/- 2.0 cm, p = 0.006). Splenomegaly, whether assessed sonographically or clinically, is an insensitive sign of portal hypertension and its absence cannot be used as a negative predictor of the presence of portal hypertension in patients with chronic liver disease.
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PMID:Splenomegaly--an insensitive sign of portal hypertension. 229 25

Because growth failure is a frequent complication of chronic liver disease in childhood, we examined the growth hormone/insulin-like growth factor type I axis and its relationship to growth disturbances, nutritional status, and carbohydrate metabolism in nine children (2.1 to 18.6 years of age) with chronic cholestatic liver disease. Seven had cholestasis associated with splenomegaly and histologic findings of cirrhosis; two patients had Alagille syndrome. Stature was less than or equal to 15th percentile in all except the youngest subject and less than 5th percentile in five subjects. Ten-hour, nocturnal, integrated serum concentrations of growth hormone were considerably higher in patients with cholestasis than in control subjects (mean +/- SD) 9.7 +/- 3.8 vs 4.7 +/- 1.9 ng/ml; p less than 0.02). Serum concentrations of insulin-like growth hormone type I were less than 95th percentile confidence intervals for age- and sex-matched norms in five patients and at the lower limits of normal in the remaining four patients. Insulin sensitivity, determined with the minimal model intravenous glucose tolerance test, was not decreased in five patients despite elevated levels of circulating growth hormone. The estimated mean caloric and protein intake exceeded the recommended dietary allowance and the weight-for-height index was greater than 90% for six of nine patients. Triceps and subscapular skin-fold thicknesses, indicators of body fat stores, were greater than 25th percentile for five of nine and eight of nine patients, respectively, suggesting deficient lipolytic action of GH. We conclude that children with cholestatic liver disease have a resistance to the growth-promoting, diabetogenic, and lipolytic properties of growth hormone.
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PMID:Resistance to the growth-promoting and metabolic effects of growth hormone in children with chronic liver disease. 239 94

Twenty-two patients with HBsAg positive acute viral hepatitis have been followed-up for two years after the acute episode. Besides the usual clinical, pathological and biological examinations, the following tests were carried out: serum HBsAg: circulating T and B lymphocytes: serum IgG, IgA, IgM: leukocyte migration test to HBsAg; serum complement: autoantibodies, rheumatoid factors, cryoglobulins, alpha-fetoprotein. Serum HBsAg persistence was correlated with some features of the acute episode: protracted clinical form: recurrences or relapses; splenomegaly; decrease of circulating T-lymphocytes; presence of lymphocyte sensibilization to HBsAg. Among the seven serum HBsAg carriers, three developed a chronic hepatitis. The state of healthy chronic HBsAg carrier was associated with high levels of serum IgA (over 300 IU/ml). A chronic hepatitis has been also diagnosed in four out of the 15 patients who cleared their serum HBsAg. The presence, on the one hand, of a chronic hepatitis among the patients who cleared their HBsAg and, on the other hand, its absence in some of the HBsAg carriers suggest that HBsAg persistence is not a sine qua non condition for the development of the chronic liver disease. The onset of autoimmune phenomena due to the disturbance of immunologic homeostasis and perhaps other genetic or environmental factors seemingly allow the perpetuation of a hepatic damage.
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PMID:A prospective study of postacute viral hepatitis: the role of HBsAg. 370 99

An unusual hepatic disease developed in 3 patients with a well-functioning kidney graft 16-24 months after transplantation. Vague abdominal pain, increased bleeding tendency and edema were initial complaints, and hepato- or splenomegaly and ascites were found as well. Liver function tests were not or only mildly disturbed; hemolysis and pancytopenia were always present. Colloid uptake was absent at liver scintigraphy and the hepatic venous wedge pressure was increased. Esophageal varices were demonstrated. Liver biopsy showed extensive midzonal and pericentral sinusoidal dilatation. After discontinuation of azathioprine the symptoms and the extent of sinusoidal dilatation disappeared gradually, but after 1-3 years fibrosis or micronodular cirrhosis had developed and splenomegaly with hypersplenism remained. These observations strongly suggest an association between chronic use of azathioprine and the development of venous congestion of the liver with sinusoidal dilatation, eventually resulting in chronic liver disease.
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PMID:Hepatic sinusoidal dilatation with portal hypertension during azathioprine treatment after kidney transplantation. 390 52

Six patients had chronic liver diseases of diverse origin, persistent unexplained thrombocytopenia, and no palpable splenic enlargement. Platelet-associated IgG levels were increased in all cases. Two patients underwent splenectomy; the spleens weighed 375 and 230 g. Each patient had prompt, complete, and sustained platelet count normalization. Two other patients treated briefly with prednisone had no response. These observations indicate that a minimally enlarged spleen may be responsible for thrombocytopenia in chronic liver disease and suggest that immune mechanisms may contribute to thrombocytopenia in this setting.
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PMID:Immune thrombocytopenia and response to splenectomy in chronic liver disease. 395 29

We have measured plasma fibronectin in 28 patients with well-compensated chronic liver disease, 4 patients with non-cirrhotic portal hypertension and 6 patients who have undergone shunt surgery for the relief of portal hypertension and splenectomy. 17 patients with portal hypertension had significantly lower levels of fibronectin (207 +/- 54; mean +/- SD) compared with 15 patients without portal hypertension (315 +/- 75, p less than 0.001) and the 6 patients who had undergone shunting and splenectomy (330 +/- 66, p less than 0.001). We suggest that in patients with portal hypertension and splenomegaly, low levels of fibronectin might result from increased consumption by the enlarged spleen.
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PMID:Low fibronectin in portal hypertension. 665 24

Abnormalities of cell-mediated immunity-anergy to delayed hypersensitivity skin testing, diminished T-lymphocyte concentrations and mitogen responsiveness, and the presence of serum inhibitors--have been shown to be present in patients with portal venous obstruction and normal hepatic function. In contrast, tests of humoral immunity--antibody titiers to viral and bacterial antigens and immunoglobulin concentrations--were normal. These abnormalities in cell-mediated immunity are probably the result of the development of portal hypertension, portosystemic shunting, and congestive splenomegaly. These studies raise the possibility that qualitatively similar defects in patients with chronic liver disease and portal hypertension may, in part, be attributable to the same mechanisms.
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PMID:Immune function in patients with extrahepatic portal venous obstruction and the effect of splenectomy. 696 95

Forty-three patients with spontaneous bacterial peritonitis (SBP) between 1973 and 1978 were identified. Criteria for SBP included a positive ascites culture and polymorphonuclear cell concentration greater than 250 cells per mm3. Chronic liver disease was documented by varices in 91%, severe histologic fibrosis or cirrhosis in 94%, splenomegaly in 91%, and past hospitalization for liver disease in 57% of the patients. SBP was detected within 7 days of admission in 17 patients (40%) and within 35 days in 38 patients. Single organisms were isolated from 38 patients and multiple organisms from 5 patients. Twenty-six of 43 patients survived the episode of SBP, but only 13 survived the hospitalization. Analysis of the survival curve from the onset of SBP revealed a rapid death rate and a slow death rate set of patients. Rapid death (less than or equal to 7 days from SBP onset) correlated with a lack of prior hospitalization for liver disease (p less than 0.001), hepatomegaly (p less than 0.001), increased serum bilirubin (p less than 0.005), serum creatinine (p less than 0.05), and peripheral white blood cell concentrations (p less than 0.05). Survival during hospitalization was associated with prior hospitalization with liver disease (p less than 0.001) and chills during the episode of SBP (p less than 0.001). The 43 patients were divided into Group 1 patients on the basis of a serum bilirubin greater than 8 mg% and/or serum creatinine greater than 2.1 mg%; Group 2 patients had lower values. Survival was greater in Group 2 patients with advanced, relatively quiescent liver disease compared to Group 1 patients for both the episode of SBP (91 vs. 29%; p less than 0.001) and for hospitalization (50 vs. 9%; p less than 0.05). Death in Group 2 patients was related to inadequate antibiotic therapy (p less than 0.05), nonhepatic factors, and new onset of renal failure. Although SBP in the setting of severe acute liver injury has a dismal prognosis, SBP with minimal acute liver injury has a relatively good prognosis for hospital survival even with advanced chronic liver disease. Long-term survival is also possible since 4 of 9 patients with prolonged follow-up have survived 3 years.
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PMID:Spontaneous bacterial peritonitis. 709 41

Increased numbers of bone marrow mast cells were found in 45 (2.2%) of 2,000 bone marrow specimens obtained from patients who had hematologic disorders. Mast cells were most frequently seen in the marrows of patients who had preleukemic syndromes, lymphoproliferative disorders, and acute leukemia. The 16 patients who had preleukemic syndromes included those with refractory sideroblastic and megaloblastic anemia (with or without an excess of blasts), idiopathic pancytopenia or pure erythrocytic aplasia, paroxysmal nocturnal hemoglobinuria, idiopathic refractory neutropenia, agranulocytosis or thrombocytopenia, and persistent eosinophilia. Five of the seven patients who had acute leukemia had nonlymphoblastic leukemia; two had blastic crisis of chronic granulocytic leukemia. Of the 13 patients who had lymphoproliferative disorders, eight had chronic lymphocytic leukemia, three had macroglobulinemia, and two had non-Hodgkin's lymphoma. Three patients who had chronic renal failure associated with severe anemia and two who had chronic liver disease, splenomegaly, or hypersplenism were also encountered. In this study there appeared to be a consistent relationship between the presence of increased numbers of mast cells and the lymphocyte and plasma cell counts in the bone marrow. The significance of the presence of secondary mastocytosis in premalignant lesions, neoplasia, and, in particular, lympho- and myeloproliferative disorders, is still unclear.
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PMID:Increased bone marrow mast cells in preleukemic syndromes, acute leukemia, and lymphoproliferative disorders. 745 27


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