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.

Adolescent↗

PubMedDate not supplied

Nervous and ocular disorders in children with cholestasis and vitamin A and E deficiencies.

Thirteen children, aged 10 months to 20 years, presenting with chronic cholestasis from the first month of life and with low serum levels of vitamins A and/or E, have been investigated for neurological and ophthalmological symptoms. Clinical findings consisted of 4 types: peripheral neuropathy; cerebellar dysfunction; abnormalities of eye movement, and retinal degenerative changes. The results of electrophysiological and morphological studies of muscle and nerves were consistent with neurono-axonal degeneration. Electrical abnormalities of the retina, especially a decrease of the b wave of electroretinogram, appear to be the first sign of the syndrome, allowing early detection. Evidence for vitamin deficiency (E or E+A) suggests substitutive parenteral treatment in such patients.

Adolescent↗

PubMedDate not supplied

Pigment gallstones of the common bile duct in infancy.

Ten infants of less than 6 months of age presented with cholestatic jaundice and gallstones. Jaundice occurred after a lag period, and sepsis was present in three children. Ultrasound examination showed dilatation of intrahepatic and extrahepatic bile ducts in eight patients and detected cholelithiasis in three. Percutaneous transhepatic cholangiography and/or surgery allowed separation of the patients into two groups: (i) six children with lithiasis in the distal common bile duct, and (ii) four children with lithiasis associated with bile duct perforation at the junction between the cystic and common bile ducts with gallstones probably secondary to bile stasis and infection. Surgical treatment was confined to removal of calculi and drainage in eight children; biliary reconstructive surgery was necessary in the other two who had serious biliary duct lesions. No recurrence was observed after 1 to 7 years. The pigmentary nature of cholelithiasis was established by stone morphology in all cases, and by bile and stone analysis in several cases.

Bile↗

PubMedDate not supplied

Extrahepatic biliary atresia.

Extrahepatic biliary atresia is defined as partial or total absence of permeable bile duct between porta hepatis and the duodenum. The incidence varies from 1:8,000 to 1:10,000. Cholestasis is total and permanent. 131I Rose Bengal test and needle liver biopsy allow correct identification of 95% of cases before surgery. Before the surgical procedure described by Kasai in 1959, all patients died between 1 and 2 years of age. Through the use of different types of Kasai's procedures by experienced groups, 30 to 35% of patients are successfully operated on. In our group, 248 infants underwent surgery, 121 before 1977: 44 (36.6%) are alive at least 5 years after surgery. Growth is within normal limits in all children still alive, even in those who presented with numerous and severe episodes of cholangitis during the first two postoperative years. Portal hypertension was progressive in 23 children with splenomegaly and esophageal varices developing between 1 and 2 years of age. Five patients bled profusely between 2 and 4 years of age: portal systemic shunts were performed in 4. Surgical liver biopsies were performed in 20 children, 5 to 8 years old: cirrhosis was present in all and was micronodular in 13 and macronodular in 7. The most impressive histologic change was absence of biliary ducts or ductules. This histologic finding can be related with percutaneous transhepatic cholangiographic pictures. Despite the abnormal appearance of intrahepatic bile ducts and the constant presence of cirrhosis with portal hypertension, all 44 patients who are alive at least 5 years after surgery seem to lead almost normal lives.

Bile Ducts↗

PubMedDate not supplied

A retrospective study of the role of delta agent infection in children with HBsAg-positive 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.

Adolescent↗

PubMedDate not supplied

Biochemical indicators of vitamin A depletion in children with cholestasis.

Biochemical indicators of vitamin A status were measured in 24 children (1 month to 6 years old) with severe cholestasis starting early in life and in 21 children (3 months to 13 years old) with liver disease but without cholestasis. Liver vitamin A concentrations, expressed as micrograms of retinol per gram of liver (mean +/- S.D.), were 6.3 +/- 7.1 (range: 0.14 to 28) and 143 +/- 108 (range: 18 to 424), respectively, in cholestatic and non-cholestatic children. In infants less than 6 months of age, liver vitamin A values less than 10 micrograms per gm were found in 14 of 17 cholestatic children but in none of 3 non-cholestatic subjects. Plasma vitamin A values, expressed as micrograms of retinol per deciliter (mean +/- S.D.), were 23 +/- 18 (range: 3 to 62) and 46 +/- 33 (range: 14 to 125), respectively, for the two groups. Plasma retinol values less than 10 micrograms per dl were always associated with liver concentrations less than 10 micrograms per gm. Plasma retinol-binding protein was only reduced to 71% of control values in cholestatic children. The fatty acid composition of liver retinyl esters was unaffected by any condition studied. Infants with chronic cholestasis are in a precarious nutritional status very early in life relative to liver reserves of vitamin A. Plasma vitamin A values, unless less than 10 micrograms retinol per dl, are poor indicators of inadequate vitamin A status.

Adolescent↗

PubMedDate not supplied

Long-term neuropsychological outcome in children undergoing portal-systemic shunts for portal vein obstruction without liver disease.

Forty-two children with portal vein obstruction (cavernoma) and without liver disease had a portosystemic surgical shunt performed at different ages. All of them had severe gastrointestinal hemorrhage prior to surgery. To evaluate the long-term neurological and psychological effects of shunt procedures in children with normal liver function, they were studied up to 24 years postoperatively with psychometric testing. A comparison with two control groups as to the impact of surgery, family situation, and neonatal events was considered. Twenty-four children with portal vein obstruction who were not operated on and 19 children splenectomized for hematological reasons acted as the control groups. No differences were observed in schooling and employment. Psychometric tests were almost identical in shunted patients and in the control groups. Minimal portal systemic encephalopathy appeared to be undetectable clinically either by electroencephalogram or by venous ammonia levels. The results of psychometric tests appear to show no loss of intellectual and psychological capacity in patients operated on for portal obstruction. Only visual memory and spatiotemporal tests showed slightly worse results in those children who underwent this procedure. Further longitudinal studies are required to substantiate these findings.

Adolescent↗