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Biomedical subjects

G Maggiore

Publications and source records attributed to G Maggiore.

At least 73 records · Page 4Linked to original sources
PubMed1986

Difficult hGH treatment in a patient with type III glycogen storage disease.

The case of a boy affected by type III glycogen storage disease and total GH deficiency is reported. Substitutive treatment with hGH caused an extreme elevation of blood lipids. His lipid profile returned near to basal values 1 month after treatment was discontinued. The association of growth hormone and amylo-1-6-glucosidase deficiencies is unusual and difficult to treat; however growth hormone deficiency should be considered in patients with hepatic glycogenoses and severe growth retardation.

Adolescent↗
PubMed1986

Liver disease associated with anti-liver-kidney microsome antibody in children.

In the past 10 years we have examined 20 children with inflammatory liver disease associated with high serum titers of anti-liver-kidney microsome antibody (anti-LKM). The first hepatic symptoms were progressive fatigue and jaundice, the fortuitous finding of hepatomegaly or splenomegaly with raised transaminase activity, or an acute hepatitis-like illness. At the time of diagnosis, hepatomegaly was present in 18 children, splenomegaly in 16, jaundice in nine, and ascites in two. Serum alanine transferase activities were elevated in all but two, who had already received steroids. Serum total gammaglobulin values were greater than 2.0 gm/dl in 16 children, prothrombin activity less than or equal to 60% in six, and serum titer of anti-LKM between 1:100 and 1:100,000. All children but one had cirrhosis, and histologic signs of aggressivity were present in 14. In 11 children one or more extrahepatic diseases were present, including type 1 diabetes, vitiligo, glomerulonephritis, autoimmune hemolytic anemia, hypoglycemia with hyperinsulinism, autoimmune thyroiditis, chronic mucocutaneous candidiasis with hypoparathyroidism, and multiple cutaneous and visceral telangiectasias. Treatment with prednisone and azathioprine improved the liver condition in 16 of the 18 patients given treatment. In eight of them discontinuation of treatment resulted in rapid relapse; 14 are still receiving treatment and have stable hepatic function with follow-up from 8 months to 6 1/2 years. Only two are free of treatment. Four children died, two in spite of immunosuppressive therapy, one during a relapse, and one of extrahepatic disease. These results indicate that this autoimmune inflammatory liver disease may have onset early in life, with several clinical patterns; is frequently associated with certain types of extrahepatic manifestations of autoimmune origin; and is a potentially fatal disease for which immunosuppressive treatment must be started early.

Adolescent↗
PubMed1986

Assessment of HBV replicative status by receptors for polymerized human albumin.

Receptors for polymerized human albumin (pHSA-r) are expressed on HBsAg in larger numbers during complete viral replication. We have evaluated the usefulness of pHSA-r as a marker of high-level infection by a comparison with serum HBeAg, liver HBcAg and serum HBV-DNA. One-hundred-and-fifty-seven patients with HBsAg positive chronic liver disease were tested for HBeAg and pHSA-r titre. In 73 of them liver HBcAg was tested by immunofluorescence, while in the remaining 84 serum HBV-DNA was determined. Seventy-three subjects were HBeAg positive, 20/73 were HBcAg positive, and 60/84 had circulating HBV-DNA. The best cut-off for pHSA-r was found at 1:204,800 (sensitivity of 67.6% and specificity of 95.7% in detecting viral replication). At this cut-off the concordance rate between pHSA-r and viral replication (as assessed by HBV-DNA or liver HBcAg) was 83%, a figure similar to that of HBeAg (88%). Three HBeAg negative subjects who were actually complete replicators were identified by the pHSA-r test.

Carrier State↗
PubMed1985

Fecal chymotrypsin: a new diagnostic test for exocrine pancreatic insufficiency in children with cystic fibrosis.

The purpose of this report is to evaluate whether a new, simple, non-invasive method for chymotrypsin measurement in stools is useful for the diagnosis of exocrine pancreatic insufficiency in cystic fibrosis (CF). A hundred children aged from 2 months to 12 years were tested: 50 children had been admitted for chronic diarrhoea, 15 for cystic fibrosis and 40 acted as controls. Chymotrypsin in stools was assayed using a kinetic measurement with Succ-Ala-Ala-Pro-Phe-pNa as substrate in a simple photometric assay. In 13 of 15 children with cystic fibrosis, stool enzyme levels were always remarkably low, while all control subjects and all children not presenting cystic fibrosis had normal stool levels of chymotrypsin. Our data suggest that stool chymotrypsin measurement is a simple and reliable "tubeless" test for the evaluation of exocrine pancreatic insufficiency in children with cystic fibrosis.

Child↗
PubMed1985

Life-saving immunosuppressive treatment in severe autoimmune chronic active hepatitis.

A 13-year-old girl with a 1-year history of elevated serum alanine transferase was hospitalized because of liver failure. Low prothrombin time (12%) prevented needle liver biopsy. Because of a high titer of antismooth-muscle antibodies (1:500), a tentative diagnosis of "autoimmune" chronic active hepatitis was made and immunosuppressive therapy was started. Despite the severity of the liver disease, of her poor general condition, and of spontaneous bacterial peritonitis, she dramatically responded to treatment, prothrombin time returning to normal within 5 months. Diagnosis of chronic active hepatitis was later confirmed by liver biopsy. This report indicates that immuno-suppressive therapy can be life saving in children with severe chronic active hepatitis even when major signs of liver failure are present.

Adolescent↗
PubMed1985

[Role of children with chronic hepatitis in the intrafamilial spread of hepatitis B virus infection].

In order to evaluate the intrafamilial spread of hepatitis B virus (HBV) infection we studied the serological markers of HBV in 101 relatives of 35 children with chronic hepatitis B. Sixty per cent of relatives had markers of infection and 25% showed persistent HBs antigenemia. The high prevalence of HBeAg versus anti-HBe in chronically infected relatives suggests a close temporal relationship of the infection between adults and children. These results support the hypothesis that the child is the main carrier of HBV infection in his family.

Adolescent↗
PubMed1984

[Chronic HB virus hepatitis in children. A study of 29 cases].

Clinical, biochemical and histological features of chronic hepatitis type B were studied in 29 children aged 8 months to 13 years. On entry into the study, all were known to have had hepatitis B surface antigen (HBsAg) with elevated serum transaminase levels for at least six months. A possible source of infection was found in 15 children. When they entered the study, all patients were anicteric and all but one asymptomatic. Hepatomegaly was detected in 15 patients and was associated with splenomegaly in two. Hypergammaglobulinemia was present in 4 children. Serological evaluation of hepatitis B virus markers showed evidence of complete viral replication (HBeAg positivity) in 24 cases and incomplete replication (anti-HBeAg positivity) in 5. Liver histology showed chronic persistent hepatitis (CPH) in 18 children, and chronic aggressive hepatitis (CAH) in 10 (3 moderately active and 7 with major signs of aggressivity ) associated with cirrhosis in 5. One patient had only minimal histological changes. Evaluation of clinical, biochemical and virological parameters did not strictly parallel the histological diagnosis in terms of "activity" of the disease. Follow-up for a mean period of 13 months showed good clinical tolerance to the disease in both CPH and CAH patients. Only 2 children with CAH were given corticosteroids and/or azathioprine for a short period. During follow-up no children with active disease developed liver insufficiency or evidence of portal hypertension. No significant difference in the percentage of children who had seroconversion to antiHBe was found between CPH and CAH groups. Only one child with CAH became HBsAg negative.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗
PubMed1984

Hepatitis B virus infection and Schönlein-Henoch purpura.

Sch onlein -Henoch purpura developed in two children in association with hepatitis B virus (HBV) infection. The first child, an 8-year-old boy, first had a clinical picture of Sch onlein -Henoch purpura and then was found to have HBV-related chronic persistent hepatitis. In the second child, a 6-year-old girl, characteristic skin lesions, arthralgia, and proteinuria developed during acute hepatitis B. Immunofluorescence demonstrated IgA deposition in the renal glomeruli of the first patient. We suggest that evidence of HBV infection should be sought in patients with Sch onlein -Henoch purpura.

Acute Disease↗
PubMed1984

Treatment of autoimmune chronic active hepatitis in childhood.

Seventeen children with chronic active hepatitis and high serum titers of smooth-muscle or liver-kidney microsomal antibodies were given prednisone and azathioprine. Clinical and biochemical remission was obtained in all but two, who died of progressive liver failure. Evaluations in 14 children after a mean period of 22 months of treatment showed normal transaminase activity and gammaglobulin levels in 12, and serum autoantibody titers of less than 1: 100 in 10; liver histologic findings showed absence of inflammation in seven children, moderate portal or lobular inflammation in five, and minor features of aggressivity in two. Cessation of therapy was then attempted in nine children. Relapse occurred in all but one and could not be attributed to any previously recorded biologic or histologic feature. After follow-up of 18 months to 7 years, all but two patients are still receiving maintenance therapy with prednisone and azathioprine. Cirrhosis was present before treatment in 13 children and is now present in all but one. These results suggest that in most children with autoimmune chronic active hepatitis, immunosuppressive therapy can prevent further deterioration of liver function but must be pursued for several years before discontinuation is attempted.

Adolescent↗
PubMed1983

Defective neutrophil motility in children with chronic liver disease.

Neutrophil motility was assessed in 31 children with chronic liver disease to estimate the eventual increased susceptibility of these patients to bacterial infections. Twelve children had chronic hepatitis (seven with chronic persistent hepatitis and five with chronic active hepatitis), which was mostly related to hepatitis B virus (HBV) infection. Nineteen children had chronic intrahepatic or extrahepatic cholestasis. A total of six serious bacterial infections occurred in four of the 31 patients during the study. Twenty of the 31 children had a persistent defect of neutrophil chemotaxis. This defect was found in four types of childhood chronic liver disease: HBV-related chronic hepatitis and idiopathic intrahepatic cholestasis of infancy, in which the defect did not seem to predispose significantly to bacterial infection, and in Byler's disease and biliary atresia, in which this neutrophil defect was associated with an increased frequency of severe infections.

Adolescent↗