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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To investigate the development of diabetes mellitus in patients with thalassemia major, plasma glucose and immunoreactive insulin (IRI) levels following oral glucose and intravenous tolbutamide and glucose disappearance rates following intravenous insulin were measured in 10 patients before and during five years on a high transfusion program (HTP). Plasma immunoreactive glucagon (IRG) levels following oral glucose, intravenous insulin, and arginine were measured during the sixth year. Serial percutaneous liver biopsies were performed on seven patients. The oral glucose tolerance tests (OGAT) and mean peak IRI levels were normal in nine of 10 patients before HTP. After HTP was begun a progressive deterioration of OGTT occurred despite normal IRI levels. Following tolbutamide, the mean per cent fall in plasma glucose in the patients before HTP was significantly less than in controls (p less than 0.01) and similar to that of controls during five years of HTP in spite of higher than normal peak IRI levels. Of seven survivors after six years of HTP, three had normal OGTT and four had chemical diabetes; mean peak IRI levels were normal, but fasting IRG levels were significantly higher than in controls (p less than 0.05). In all seven patients, plasma IRG failed to increase following insulin-induced hypoglycemia and was significantly higher than in controls after arginine (p less than 0.01); after oral glucose, plasma IRG fell significantly below that of fasting only in the patients with chemical diabetes (p less than 0.03). Following intravenous insulin, the mean per cent fall in glucose before and during HTP was significantly less than in controls (p less than 0.01). Hemosiderosis and cirrhosis were present in all biopsied patients. Four patients died; two had chemical and two had nonketotic insulin-dependent diabetes. These data suggest that diabetes mellitus occurs frequently in patients with thalassemia on HTP and that insulin resistance and hyperglucagonemia, possibly due to cirrhosis, are important etiologic factors.
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PMID:Carbohydrate metabolism and pancreatic islet-cell function in thalassemia major. 32 76

Yersinia enterocolitica systemic infections are uncommon and osteomyelitis due to this organism is very rare. We report a 9-year-old girl with thalassemia major, liver cirrhosis, and hemosiderosis who developed osteomyelitis of the rib caused by Y enterocolitica type 0:3. Serologic response to the infection was confirmed. No definite source of infection was identified.
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PMID:Yersinia enterocolitica osteomyelitis in a child. 65 39

In order to evaluate the interrelations of splenectomy, iron overload and cirrhosis, histologic specimens of liver and spleen were examined and correlated in 12 children with beta-thalassemia major. All patients had received blood transfusions since infancy. Correlations seemed to exist between splenic hemosiderosis and splenic weight, and between the latter and the age at time of splenectomy. All liver samples showed varying hemosiderosis, not correlated with the number of transfusions or the children's age. Irregular liver cirrhosis existed in three children, 7, 8, and 14 years after splenectomy. No cirrhosis existed in any of the children where the spleen was in situ. Splenectomy in children with thalassemia may carry the long-term risk of liver cirrhosis.
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PMID:Splenectomy, iron overload and liver cirrhosis in beta-thalassemia major. 82 69

Parameters of iron metabolism and humoral immunity were studied in patients with chronic diffuse diseases of the liver (cirrhosis, chronic hepatitis), beta-thalassemia major, dyserythropoiesis, hereditary hemochromatosis. High ferritin content has been recorded in the plasma of these patients, that leads to the formation of antibodies to this protein followed by the production of circulating immune complexes inducing metabolic disorders that aggravate the pathologic process. Plasmapheresis and deferoxamine therapy result in a decrease of ferritin and circulating immune complex content in the plasma, that produces a favourable effect on the patients' condition.
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PMID:[Relation between circulating immune complexes and serum ferritin in hemosiderosis of different etiologies]. 182 96

We report the results of a two-year non randomized prednisolone trial carried out in 18 thalassemia major patients with chronic active hepatitis and in 16 controls. We found a beneficial effect on the biochemical remission rate and on the extent of liver inflammation with no significant side effects and no overt reactivation of possible latent HBV infection at three-year follow-up. However, a more prolonged longitudinal study is necessary in order to evaluate whether steroid treatment can impede the evolution to cirrhosis without determining long-term consequences, depending on virus-host interactions such as liver cell carcinoma.
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PMID:Methyl-prednisolone treatment of serum HBsAg negative chronic active hepatitis occurring in transfusion-dependent thalassemia major. 661 92

The study of surgical liver biopsy specimens obtained during splenectomy in 86 children with thalassaemia indicated that such patients may develop liver disease that evolves into cirrhosis. Histological characteristics suggest that it is post-necrotic cirrhosis. Onset of cirrhosis in some patients may occur as early as 7-8 years old, and at age about 15-16 years most children with thalassaemia show features of cirrhosis. In addition to fibrosis, hepatitis, or even aggressive hepatitis may develop as has also been observed in patients without thalassaemia who have undergone multiple transfusions. This study presents the current probable evolution of liver disease in patients with thalassaemia and may thus serve as a reference from which to evaluate any future progress in the treatment and care of patients with Cooley's disease.
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PMID:Cirrhosis associated with multiple transfusions in thalassaemia. 669 98

The authors report the striking increase in lymph node density due to hemochromatosis observed with computed tomography (CT) in nine patients with Cooley anemia treated with multiple blood transfusions. The CT appearance and pathologic findings of hemochromatosis of the liver and spleen in three of these patients were also observed and correlated with pathologic specimens. CT density of the liver seemed to relate to the degree of hepatic fibrosis or cirrhosis, rather than the amount of iron. Previous reports have not emphasized dense ferritinized lymph nodes in treated Cooley anemia patients.
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PMID:CT in B-thalassemia: iron deposition in the liver, spleen, and lymph nodes. 678 34

Glucose intolerance is a common consequence of transfusion therapy in patients with thalassemia major (TM), but the relative contribution of pancreatic damage and insulin resistance to glucose intolerance is unclear. We have investigated oral (OGTT) and intravenous (IVGTT) glucose tolerance, insulin sensitivity, and fasting concentrations of insulin, proinsulin, and des 31,32 proinsulin in 12 patients with TM (seven hepatitis C virus [HCV] antibody-negative and five-positive), eight patients with hepatic cirrhosis, and nine healthy controls. Two-hour plasma glucose concentrations were marginally higher in anti-HCV-negative (median, 7.4 mmol/ L; range, 4.0 to 8.2) and significantly so in anti-HCV-positive thalassemics (median, 8.5 mmol/L; range, 6.4 to to 23.0) and cirrhotics (median, 8.0 mmol/L; range, 4.7 to 17.6) than in controls (median, 5.5 mmol/L; range, 3.0 to 6.3). Insulin sensitivity was also reduced in the three patient groups (P < .05). Insulin resistance was the main determinant of oral glucose intolerance in all patient groups (partial r2 = .49, P < .0001, n = 28). In turn, the main determinants of insulin insensitivity in TM patients were liver damage (albumin, r = .67, P = .02) and serum ferritin concentration (r = -.62, P = .03). There was no relationship of either 2-hour or incremental insulin concentrations with ferritin levels or with HCV status in TM subjects. Moreover, these patients showed no elevation of concentrations of proinsulin and des 31,32 proinsulin, markers of pancreatic beta-cell damage, in excess of those observed in cirrhotic patients. In conclusion, the glucose intolerance of TM, like that of cirrhosis, is associated with insulin resistance, not insulin deficiency, and may be a direct or indirect consequence of hepatic damage.
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PMID:Glucose intolerance in thalassemia major is related to insulin resistance and hepatic dysfunction. 862 11

Hepatitis C virus (HCV) infection is a common cause of liver disease among polytransfused thalassemics. We treated a cohort of subjects with beta-thalassemia major and chronic hepatitis C with alpha-interferon. The aims of the study were to assess the long-term biochemical and virologic efficacy of alpha-interferon and to evaluate the influence of HCV type and liver siderosis on the outcome of therapy. Seventy subjects (mean age, 14.1 years) with chronic HCV infection and abnormal aminotransferases received recombinant alpha-interferon for 12 months and were observed after therapy for at least 24 months. Sixty-three subjects (90%) were HCV-RNA positive at the start of therapy. HCV type 1b was found in 41 subjects (65.1%), non-1b types in 13 (20.6%), and mixed HCV types in 9 (14.3%). Liver biopsy showed cirrhosis in 11 subjects (15.7%) and siderosis grade 3-4 in 24 patients (34.2%). Three patients stopped therapy due to adverse events. Twenty-eight subjects (40%) had normal aminotransferases and had cleared HCV-RNA when last observed (mean follow-up, 36.5 months; range, 25 to 49 months). Of 41 patients who did not normalize aminotransferases, 9 had become HCV-RNA negative at the end of follow-up. The absence of cirrhosis, low liver iron content, and infection with non-1b HCV type were independently associated to complete sustained response upon multivariable analysis. In conclusion, alpha-interferon may induce a sustained virologic and biochemical remission of hepatitis in beta-thalassemic patients with chronic HCV infection and nonadvanced liver disease.
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PMID:Long-term efficacy of alpha-interferon in beta-thalassemics with chronic hepatitis C. 931 Apr 71

Hepatitis C infection is common in patients receiving life-long blood transfusion therapy. Interferon-alpha induces long-term viral clearance in 25-30% of patients suffering from Cooley's anemia. Ribavirin, an orally active guanoside analogue together with interferon-alpha produces a sustained response in up to 40% of patients with cirrhosis, who had previously failed single agent treatment. Growth retardation in iron-overloaded patients is the result of growth hormone deficiency in up to 30% of patients. Height gain can be successfully achieved in these patients with growth hormone treatment. Pregnancy in women with Cooley's anemia is now a reality, and over 100 pregnancies have been documented. Conception may be spontaneous or the result of ovulation induction. Cardiomyopathy and diabetes require careful assessment in these patients before a decision is made to treat with gonadotrophins to induce ovulation.
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PMID:New approaches to the management of hepatitis and endocrine disorders in Cooley's anemia. 966 45


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