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. RESULTS: Twenty-four children presented with gastrointestinal bleeding after age 12, and 20 did not bleed. The overall actuarial probability of bleeding was 49% at age 16 and 76% at age 24. Probability of bleeding at age 23 was higher in children who had bled before age 12 than in children who had not bled (93% vs 56%; P =.007). Probabilities of bleeding at age 18 and at age 23 were 60% and 85%, respectively, in patients who had grade II or III esophageal varices at age 12. The 9 children without varices or with grade I varices only on endoscopy did not bleed between the ages of 12 and 20 years. CONCLUSIONS: Children with portal vein obstruction have a >50% risk of bleeding during adolescence; the pattern of varices on endoscopy at age 12 may have a prognostic value.

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. All patients were treated with prednisone and azathioprine with normalization or improvement of liver function tests: 28 children are currently alive after a mean follow-up of 4 years, 10 months. Treatment was interrupted in four patients only. Two patients died of liver failure in spite of immunosuppressive therapy before the era of liver transplantation. In spite of prolonged therapy, five other patients ultimately required liver transplantation during adolescence or early adulthood. These results (a) further define a group of autoimmune hepatitis in children characterized by the presence of serum anti-actin antibodies; (b) indicate that immunosuppressive therapy improves liver function, although in most cases it must be continued for a long period to maintain remission; and (c) suggest that progressive liver failure may occur in early adulthood and may require liver transplantation.

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. In six patients, cirrhosis was diagnosed within the first 12 months after the clinical onset of liver disease. Forty-six children had autoimmune hepatitis; cirrhosis was present in 41 (89%). Cirrhosis was diagnosed by laparoscopy in all 41 children and by needle liver biopsy in 23 children. Cirrhosis was already present in all 10 children studied 2 to 5 months after the first sign of liver disease. Our results indicate that the incidence of cirrhosis is high in children with chronic hepatitis, especially of the autoimmune type, and that cirrhosis may occur early, irrespective of cause. A combination of laparoscopy and biopsy is more reliable than biopsy alone for the diagnosis of cirrhosis in children with chronic hepatitis.

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.

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. 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.

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. Ultrasonography provided the correct diagnosis in 36 of the 37 children in whom it was performed. Angiography, performed in 101 children, showed that the obstruction extended to the superior mesenteric vein in 14 children and to the entire portal venous system in seven; intrahepatic branches were involved in half the cases. Natural splenorenal shunts were visible in 19 children but were not clearly associated with a lower risk of gastrointestinal tract bleeding; in five of 30 children, cavography displayed abnormalities of the inferior vena cava. Spontaneous gastrointestinal tract hemorrhage occurred in 78 children. Fiberoptic endoscopy showed esophageal varices in 79 of the 81 children studied. The presence of tense varices and congestion of esophageal mucosa clearly augmented the risk of bleeding. These results suggest a simple method of investigation based on ultrasonography for diagnosis and on endoscopy for prognosis. Angiography should be limited to children with a history of gastrointestinal tract bleeding for whom a surgical portosystemic shunt is being considered.

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. Early postoperative assessment of shunt patency was judged from regression of splenomegaly and thrombocytopenia when splenectomy was not performed; when done, early postoperative ultrasonography correctly indicated the result. Significant regression of endoscopy was most often delayed postoperatively for up to six months. Children with a proved patent shunt did not have any further episodes of gastrointestinal tract bleeding, displayed no clinical signs of encephalopathy, and often exhibited a striking increase in growth velocity. These results strongly support the contention that a portosystemic shunt is the best treatment for portal vein obstruction after the first spontaneous bleeding episode, even in young children.

[Portal hypertension in children. Hemorrhagic risk and measures for prevention (author's transl)]. / Hypertension portale de l'enfant. Le risque hémorragique et sa prévention.

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)]. / Péritonite localisée d'origine génitale ou pérhépatite gonococcique. Syndrome de Fitz-Hugh Curtis.

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. Beside Neisseria gonorrhoeae, other organisms growing in the genital tract are probably involved. When necessary, coelio-laparoscopy is the method of choice to confirm the diagnosis. The four cases reported here illustrate the various symptomatic and evolutive aspects of the syndrome.

[Intravascular coagulation and severe hepatic failure in infants]. / Coagulation intravasculaire et insuffisance hépatique grave du nourrisson

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.