Risk of gastrointestinal bleeding during adolescence and early adulthood in children with portal vein obstruction.

Objective: To estimate the risk of bleeding during adolescence and early adulthood in a group of children with portal vein obstruction who had not undergone an effective treatment beforehand.

Study Design: Children (n = 44) were followed up from age 12 years to a mean age of 20 years (range, 15-34 years). Actuarial risk of bleeding, related to previous occurrence of gastrointestinal bleeding and to pattern of varices at age 12, was calculated yearly.

Autoimmune hepatitis associated with anti-actin antibodies in children and adolescents.

The clinical, biochemical, morphological, and evolutive features of autoimmune hepatitis associated with serum smooth muscle antibodies of anti-actin specificity were retrospectively analyzed in 31 children and adolescents. Cirrhosis was present at diagnosis in all but six patients, including nine of the 12 diagnosed within 6 months from the onset. In 15 children, one or more associated diseases of an immune-mediated mechanism were present, including chronic arthritis, sclerosing cholangitis, inflammatory bowel disease, and cutaneous vasculitis.

Incidence of cirrhosis in children with chronic hepatitis.

To determine the incidence of liver cirrhosis in children with chronic hepatitis, we investigated 92 children (64 were girls; mean age was 8 years 2 months) with chronic hepatitis for the presence of cirrhosis by the combined use of laparoscopy and needle liver biopsy, between 1975 and 1985. Forty-six children had hepatitis B virus-related chronic hepatitis; cirrhosis was present in 13 (32%). Cirrhosis was diagnosed by laparoscopy in 14 children and by needle liver biopsy in eight.

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.

Ultrasonic demonstration of porto-caval anastomosis in portal hypertension in children.

Seventy-one children with portal hypertension and a portocaval anastomosis were studied by ultrasound. The patency of the shunt may be inferred when it directly visualised. There are four indirect signs of patency: (1) decrease of the thickness of the lesser omentum; (2) decrease of the gastro-oesophageal collaterals; (3) decrease of the portal vein size; and (4) increase of the diameter of the inferior vena cava, as compared to pre-operative ultrasound.

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.

Portal obstruction in children. II. Results of surgical portosystemic shunts.

Seventy-six children with portal vein obstruction underwent surgical portosystemic shunt, for severe gastrointestinal tract bleeding in 64 and for prophylactic purposes in 12. Endoscopy and angiography or both showed shunt patency in 70 children; thrombosis occurred in the remaining six. The mean age at successful shunt surgery was 6 years 10 months.

Portal obstruction in children. I. Clinical investigation and hemorrhage risk.

We examined 108 children with obstruction of the portal vein. Symptoms included splenomegaly and gastrointestinal tract hemorrhage. Obstruction was secondary to portal vein injury in 44 children and was combined with congenital malformations in 17 others.

[Portal hypertension in children. Hemorrhagic risk and measures for prevention (author's transl)].

The retrospective analysis of 201 children who presented with portal hypertension and were investigated by esophageal endoscopy provides the following results : 1) esophageal varices were present in 90% of the children examined; 2) the hemorrhagic risk is much higher in children with extra hepatic portal obstruction (gastrointestinal bleeding occurred in 80% of these children) than in children with intrahepatic or postsinusoidal portal hypertension (29%); 3) endoscopic signs of major portal hypertension such as tension of varices and/or congestion of the esophageal mucosa were present in virtually all children who experienced one or more episodes of gastrointestinal bleeding; 4) esophageal or gastric lesions not related to varices were responsible for bleeding in 6 children; 5) gastric varices were present in 21 of 105 children in whom a thorough gastric endoscopy was carried out; 6) partial or complete regression of varices occurred in 11 of 27 children in whom several endoscopies were performed with a follow-up of 9 months to 6 years; complete regression occurred only when varices were of small size; 7) measures used to prevent gastrointestinal bleeding in children with varices often failed when endoscopic signs of major portal hypertension were present. These results confirm the major importance of eso-gastric endoscopy in the investigation of children with portal hypertension and suggest that porto-systemic surgical shunts should be considered in children who experience at least one spontaneous episode of gastrointestinal bleeding provided the responsibility of varices is ascertained by endoscopy.

[Circumscribed peritonitis of genital origin or gonococcal perihepatitis Fitz-Hugh Curtis syndrome (author's transl)].

Circumscribed peritonitis with perihepatitis resulting from previous female gonococcal infection is now commmonly known as Fitz-Hugh Curtis (FHC) syndrome. Contamination of the peritoneum through the Fallopian tubes and the fact that the gonococcal inflammation (frequency, though not always confirmed) remains superficial with little production of pus explain why the perihepatic lesions are fibrinous, adhesion-forming and tend to subside spontanesouly. The pathophysiological mechanisms, however, are complicated by recent reports of FHC syndrome in males.

[Intravascular coagulation and severe hepatic failure in infants].

Diagnostic criteria for disseminated intravascular coagulation occurring in severe liver failure were reviewed by analyzing 11 pediatric cases. In this series, intravascular coagulation was often latent, but became overt following inconsiderate administration of procoagulant concentrates. Exchange-transfusion of infusion of fresh-frozen plasma, with or without addition of heparin, appeared to be the best mode for correcting potential bleeding tendencies.