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Biomedical subjects

G Maggiore

Publications and source records attributed to G Maggiore.

At least 37 records · Page 2Linked to original sources
PubMed1998

Antibody-negative chronic hepatitis C virus infection in immunocompetent children.

Antibody-negative hepatitis C virus (HCV) infection, defined by the presence of HCV viremia in the absence of a serologic response to HCV, was detected in two immunocompetent and symptom-free children; each had a history of exposure to blood products. HCV infection may occasionally explain cryptogenic elevation of aminotransferases, even in the absence of serum anti-HCV. HCV-RNA should be investigated in these cases, particularly in the presence of previous exposure to blood products.

Adolescent↗
PubMed1998

Failure to increase insulin-like growth factor-I synthesis is involved in the mechanisms of growth retardation of children with inherited liver disorders.

OBJECTIVE: Growth retardation is a prominent secondary feature of chronic liver disease. We investigated the hypothalamic-pituitary-liver axis in six patients with inherited liver disease and growth failure. The objectives were to determine (1) whether there were any abnormalities in the GH/IGF-I/IGFBPs/GH binding protein (GHBP) axis, (2) whether any abnormalities were nutrition-dependent, and (3) whether recombinant human (rh) GH could be efficaciously and safely administered. MEASUREMENTS: The evaluation included two standard GH provocative tests, GHRH test, night-time GH secretion, GHBP; and IGF-I, IGFBP-3 and IGFBP-1 before and after 0.1 and 0.3 U/kg/day of rhGH given i.m., for 4 days. Two patients were enrolled for rhGH treatment. RESULTS: Quantitative nutritional assessment showed the patients' calorie and protein intake to be compatible with the recommended daily allowance in liver disease. The mean baseline GH level was higher in patients than in controls (8.4 +/- 3.8 vs 2.6 +/- 2.0 mU/l, P < 0.005) and the GH response to stimuli was normal; spontaneous GH secretion was apparently normal. The mean baseline IGF-I value in the patients was significantly below the mean of controls (31.6 +/- 16.4 vs 260 +/- 35.2 micrograms/l, P = 0.00001) and similar to that of children with GH-deficiency (40.8 +/- 18.4 micrograms/l). The mean peak IGF-I response after 0.1 U/kg/day of rhGH increased (84.9 +/- 28.2 micrograms/l, P = 0.009) but remained lower than the mean IGF-I response in GH-deficient patients and in controls (P = 0.00001). The mean peak IGF-I response after 0.3 U/kg/day (113.3 +/- 52.3 micrograms/l) was significantly higher than that after 0.1 U/kg/day (P = 0.002). The mean standard deviation score (SDS) peak for IGF-I response to 0.1 and 0.3 U/kg/day of rhGH decreased significantly from -1.7 to -1.0 (P = 0.02) and from -1.9 to -0.9 (P = 0.005), respectively. There was no difference between patients and controls in serum GHBP activity or in mean baseline IGFBP-3 and IGFBP-1 levels. IGFBP-3 levels did not change significantly in response to rhGH at either 0.1 or 0.3 U/kg/day, while IGFBP-1 significantly decreased after 0.3 U/kg/day (56.3 +/- 35.6 vs 45.9 +/- 33.1 micrograms/l, P = 0.04). A significant positive correlation was present between albumin and peak IGF-I responses to rhGH at the dose of 0.1 and 0.3 U/kg/day (R = 0.83, P = 0.03; R = 0.78, P = 0.03 respectively), as well as between height SDS and baseline or stimulated IGF-I after rhGH 0.1 U/kg/day (R = 0.81, P = 0.04; R = 0.88, P = 0.01 respectively). In the two patients treated with rhGH at 22-25 U/m2/week, the growth rate doubled in one and trebled in the other during the first year of treatment, and in both was maintained in the second year without acceleration of bone maturation or evidence of adverse effects. CONCLUSIONS: The underlying cause of growth retardation in patients with inherited liver disease seems to be a progressive failure to increase IGF-I synthesis (at the conventional rhGH dose) and the consequent lack of its growth-promoting effect. The moderate increase in baseline GH values, the greater IGF-I response to the higher rhGH dose and the improvement in growth rate following rhGH administration suggest at least a degree of sensitivity to rhGH which could be of therapeutic value.

Adolescent↗
PubMed1995

Circulating levels of interleukin-6, interleukin-8, and tumor necrosis factor-alpha in children with autoimmune hepatitis.

Circulating levels of the proinflammatory cytokines interleukin-6 (IL-6), IL-8, and tumor necrosis factor-alpha (TNF-alpha) were measured in 13 children with autoimmune hepatitis (AIH) (seven with type 1 and six with type 2). In untreated children with type 1 AIH, TNF-alpha, IL-6, and IL-8 levels were elevated when compared to those of healthy controls (p < 0.005, p < 0.02, p = 0.06, respectively), whereas in children with type 2 AIH, cytokine levels were normal in all except one sample. A significant decrease in circulating IL-6, IL-8, and TNF-alpha was observed when patients were evaluated during a subsequent remission. We found no significant correlation of cytokine levels with alanine aminotransferase (ALT) activity, total serum gamma-globulins, or prothrombin activity. In patients with cirrhosis, serum IL-8 and IL-6 levels were higher (significantly in the case of IL-8) than those of patients without cirrhosis. In conclusion, activation of the in vivo production of the proinflammatory cytokines IL-6, IL-8, and TNF-alpha appears to be associated with type 1 but not with type 2 AIH.

Adolescent↗
PubMed1995

Chronic hepatitis in children.

Autoimmune hepatitis is an inflammatory disease of the liver of unknown etiology that progresses toward cirrhosis and liver failure and is generally responsive to immunosuppressive treatment. The presence of anti-smooth muscle antibodies with anti-actin specificity and of anti-liver kidney microsomal antibodies defines two distinct subgroups of the disease. An autoantibody against liver cytosolic antigens has recently been described. Management of autoimmune hepatitis relies on immunosuppressive therapy with steroids alone or combined with azathioprine. When the disease is poorly controlled, despite good patient compliance to therapy, cyclosporin should be recommended. Progressive liver disease in chronic hepatitis B in adults has been associated with the presence of precore mutants of hepatitis B virus. In children, the presence of precore mutants seems not to affect the rate of seroconversion to anti-hepatitis B e antigen. However, high viremic levels of precore mutants are associated with persistent viral replication and liver disease. Interferon alfa seems to be less effective in children than in adults in the treatment of chronic hepatitis B; however, it hastens the seroconversion rate to anti-hepatitis B e antigen, accelerating the spontaneous clearance of the virus in children with already low levels of viral replication. Blood transfusions, especially those received in the perinatal period, are the single most important source of infection with hepatitis C in childhood. HIV coinfection is a major risk factor for vertical transmission of hepatitis C virus in pregnant women. Chronic hepatitis C in children is usually an asymptomatic disease associated with mild to moderate fluctuation of aminotransferase activities and histologic features of mildly active hepatitis. Severe active hepatitis and cirrhosis are infrequent during childhood and adolescence. Interferon may have a place in the treatment of chronic hepatitis C in children.

Adult↗
PubMed1994

Hepatitis C virus infection in children treated for acute lymphoblastic leukemia.

We studied 102 consecutive subjects after their completion of acute lymphoblastic leukemia (ALL)-directed chemotherapy, for evidence of hepatitis C virus (HCV) infection by enzyme immunoassay 2 and 3, second generation recombinant immunoblot assay and reverse transcription-polymerase chain reaction (PCR) for detection of circulating HCV-RNA. Forty-four patients (43%) had evidence of exposure to HCV; 30 of these were anti-HCV+. Of the 23 patients who were positive for both anti-HCV and HCV-RNA, 16 (69%) had a moderate increase in serum alanine aminotransferase (ALT) activity without clinical signs of liver disease. Fourteen patients were seronegative despite the presence of HCV-RNA in the serum. The proportion of different HCV genotypes was not significantly different from other anti-HCV+ patient groups. Although half of the patients with genotype III had normal ALT value, patients with normal ALT levels were represented in all genotype groups. Our study documents the prevalence of HCV infection in childhood ALL survivors, which is responsible for the majority of cases of non-B chronic liver disease in these patients. Whereas serologic screening identifies over 70% of patients with ongoing HCV infection, real HCV infection may be present even in the absence of a detectable humoral immune response to the virus. Based on this observation, determination of HCV-RNA by PCR should be recommended in patients in prolonged remission even if they test negative on serological assay. Normal ALT levels do not exclude the presence of HCV infection because the values were repeatedly normal in over half of our viremic patients.

Adolescent↗
PubMed1994

A new cause of progressive intrahepatic cholestasis: 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency.

There have been a few reports of infants with severe neonatal cholestasis related to a defect in primary bile acid synthesis. To assess the importance of such deficiency among children with progressive intrahepatic cholestasis (Byler disease), screening for inborn errors in bile acid synthesis was performed by fast atom bombardment ionization-mass spectrometry of urine samples from 30 affected children. Bile acid analysis revealed a specific fast atom bombardment ionization-mass spectrometry profile for 3 beta-hydroxy-C27 steroid dehydrogenase/isomerase deficiency in five children who had jaundice, hepatosplenomegaly, and fatty stools beginning at ages ranging from 4 to 46 months. None of them had pruritus. Liver function tests showed persistently normal serum gamma-glutamyltransferase activity, low serum cholesterol and vitamin E levels, normal serum bile acid concentrations despite raised serum bilirubin levels, and decreased prothrombin time and clotting factor V. In four of the cases a similar disease was observed in siblings. Liver function returned to normal after oral ursodeoxycholic acid therapy. We conclude that 3 beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency should be considered when idiopathic cholestatic liver disease with clinical features akin to Byler disease is characterized by the association of normal serum gamma-glutamyltransferase activity, normal serum bile acid concentration, absence of pruritus, and a return to normal liver function during ursodeoxycholic acid therapy. Early identification of these children is essential because they benefit from bile acid therapy and might thus avoid the need for liver transplantation.

Alanine Transaminase↗
PubMed1994

Lymphocytic gastritis: a positive relationship with celiac disease.

Lymphocytic gastritis is characterized by lymphocytic infiltration of the surface and pit epithelium. Its cause has not been established, but an association with Helicobacter pylori infection or celiac disease has been suggested. We evaluated the histologic features of both gastric and duodenal biopsy specimens from 245 consecutive children and adolescents, and found chronic gastritis in 60 children and celiac disease in 25. Chronic gastritis was associated with H. pylori infection in 36 children and with celiac disease in 15. Lymphocytic gastritis was found in nine children with celiac disease. Children with lymphocytic gastritis had a mean of 40.64 lymphocytes per 100 epithelial cells, compared with a mean of 3.92 lymphocytes per 100 epithelial cells in children with H. pylori-associated gastritis and 5.15 lymphocytes in normal control subjects. Immunohistochemical studies showed that the intraepithelial lymphocytes in lymphocytic gastritis were T cells. No child with lymphocytic gastritis had serologic evidence of past H. pylori infection. We conclude that lymphocytic gastritis in children is associated with celiac disease. Dyspeptic symptoms are frequent; the endoscopic appearance is not characteristic.

Adolescent↗