Adrenal hemorrhage in a newborn with Bechwith-Wiedemann syndrome.
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Biomedical subjects
Publications and source records attributed to G Maggiore.
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A seroimmunologic evaluation of 57 children with chronic hepatitis is presented. Twenty-one patients had chronic persistent hepatitis and 36 had chronic active hepatitis. Serum samples obtained before treatment were tested for HBsAg, anti-HBs, anti-HBc, smooth muscle antibody, and antibody to endoplasmic reticulum. A persistently positive HBsAg was observed in the serum of 18 of the 21 patients with chronic persistent hepatitis. The chronic active hepatitis group was divided into three subgroups according to the presence of hepatitis B-virus markers (7 patients), smooth muscle antibody (10 patients), and endoplasmic reticulum antibody (9 patients). Determination of these markers could be useful for classifying children with chronic hepatitis.
The prevalence of intrahepatic delta antigen and/or anti-delta antibody was retrospectively investigated in 102 children with chronic HBsAg-positive hepatitis who were seen consecutively in three medical institutions between 1974 and 1982. Delta infection markers were found in 13 patients (12.7%) who exhibited high serum titers of anti-delta antibody; intrahepatic delta antigen was detected in ten. Eleven of the 13 children had severe progressive liver disease associated in all but one with absence of hepatitis B virus replication as evaluated by analysis of serum hepatitis B virus DNA. The factors which seem to increase the risk of delta infection in children who are hepatitis B virus carriers are geographic origin, a history of exposure to blood derivatives and age. A further 37 of 102 children had chronic active hepatitis (20 patients) or cirrhosis (17 patients) without evidence of delta infection. These results indicate that delta infection occurs in children with chronic hepatitis. This possibility should be considered in investigation of children with HBsAg-positive chronic liver disease. Although the delta agent is an important cause of progressive liver disease in children who are chronic HBsAg carriers, severe liver injury and especially cirrhosis can occur without evidence of delta infection.
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A case of celiac disease presenting as an asymptomatic chronic persistent hepatitis in an 11-year-old girl is reported. Liver biopsy performed because of long-standing elevation of serum transaminase levels showed a mild portal fibrosis with mononuclear infiltrate. Immunofluorescence staining did not reveal deposits of immunoglobulins or complement in the liver specimen. Although the girl was totally asymptomatic, she had steatorrhea, a delayed bone age, and an abnormal D-xylose test. A jejunal biopsy showed villous atrophy and increased intraepithelial lymphocytes. On a gluten-free diet the level of transaminases fell to normal within 1 month and remained normal. According to biological remission, a second intestinal biopsy performed after 1 year of gluten-free diet revealed a normal intestinal mucosa. Our report suggests that an underlying chronic intestinal disorder, and particularly celiac disease, must be ruled out when evaluating a child with elevated levels of serum transaminase.
A 10-year-old Italian boy with chronic liver disease and copper overload is described. Biological and histopathological findings are similar to those described in children with Indian childhood cirrhosis. This report suggests that a disease akin to Indian childhood cirrhosis but different from Wilson disease can be found in non-Indian children probably representing a new, possibly inherited, disease of copper metabolism leading to copper overload in the liver.
Barrett's esophagus is a gastrointestinal metaplasia of the esophageal epithelium occurring frequently in adults with long-standing peptic esophagitis. Recent reports of Barrett's esophagus in children with gastroesophageal reflux (GER) showed that also at the pediatric age intestinal metaplasia of the esophagus may occur in association with peptic esophagitis. Recently a close association between Campylobacter-like organisms (CLOs) and gastritis has been found in the stomach of both adults and children with a variety of peptic diseases, but evidence of such infection in specimens of Barrett's epithelium has never been described in children. We report here a child with Barrett's esophagus and GER, treated with H2 blockers, who showed a Barrett's ulcer in association with CLO infection. The addition of amoxicillin to antireflux treatment was accompanied by healing of the ulcer, suggesting that bacterial infection of Barrett's epithelium may have an important role in determining its inflammation and possibly ulceration.
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