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A 4-month-old girl who was examined because of splenomegaly had portal vein thrombosis, which apparently resulted from a combination of cytomegalovirus infection and deficiency of both protein S and protein C. The cytomegalovirus infection, by damaging endothelial cells, may have triggered a cascade of events that was ultimately expressed as portal vein thrombosis resulting from deficiency of proteins S and C.

The extracellular matrix (ECM) expression is subject to distinct changes during ontogeny, and the natural course of liver fibrosis in neonates is thought to differ from that in adults. We compared the expression and distribution of main ECM components between neonatal and adult liver fibrosis. Liver biopsies from infants with neonatal cholestasis and fibrosis were compared to adult biopsies exhibiting an equivalent stage of fibrosis. All biopsies were examined by immunohistochemistry (indirect ABC method) for the ECM proteins, collagens I, III, IV, and VI, laminin, and fibronectin. Infants (aged 1-8 months) with neonatal hepatitis (n = 3), extrahepatic biliary atresia (EHBA) (n = 5), and normal histology (n = 2) were compared with 9 adults (aged 17-70 years) with chronic hepatitis (n = 3), primary biliary cirrhosis (PBC) (n = 4), and normal histology (n = 2). Collagens I, III, and IV and fibronectin were significantly increased in neonatal hepatitis with mild fibrosis (scoreor = 4) compared to adults with an equivalent fibrosis stage. This increase was particularly notable in perisinusoidal spaces. Laminin expression was increased in portal and perisinusoidal spaces both in neonatal hepatitis and extrahepatic biliary atresia with mild fibrosis. In infants with moderate to severe fibrosis (scoreor = 6), only collagen I was increased in comparison to adults, whereas collagen VI expression was identical in all groups, irrespective of the degree of fibrosis. Expression of matrix proteins was not different in infants and adults without fibrosis. The increased perisinusoidal deposition of certain ECM components in infants with active hepatitis and mild fibrosis may point to an underlying difference in the mechanism or stimulus of fibrogenesis in neonates as compared to adults.

Our objective was to investigate the relationship between demographic factors, nutrition, stool gas production, and the existence of infantile colic (IC) syndrome. Hydrogen and methane production from stool specimens of infants with and without infantile colic was quantified at two separate time points, the age at presentation of colic (12 weeks) and at6 months of age. The relationship between demographic variables and IC was also studied. A total of 59 infants with ages ranging from 2 to 12 weeks were enrolled in the study. Of these, 30 infants developed symptoms of colic. No correlation was found between IC and birth weight, gestational age, sex, type of feeding, mean time of feeding, stool frequency, and consistency. There was also no correlation between IC and the parents' age or education or the infant's number of siblings. Analysis of the stool samples revealed that methane was produced at concentrations2 ppm by 15.3% of the infants at age3 months and by 46.4% of infants at age6 months. The mean methane concentrations produced by stool increased with age (0.95 +/- 0.58 ppm at 3 months of age vs 1.29 +/- 0.65 ppm at 6 months of age. There was no difference in stool hydrogen concentration between infants with and without IC. In contrast, the mean methane level at 3 and 6 months of age was higher in infants without IC than with IC, but reached statistical significance only at 6 months of age (0.97 +/- 0.68 vs 0.93 +/- 0.46) (NS) at 3 months of age, and 1.56 +/- 0.55 vs 0.93 +/- 0.62 (P0.05) at 6 months of age respectively. Furthermore, infants that produced higher methane levels at 3 and 6 months of age had significantly (p0.05) less colic in the first months of life. In conclusions, methane production may play a role in the alleviation of IC. Future studies are needed to confirm our findings.

Supplementation of 5-aminosalicylic acid (5-ASA) and of iron are among the principal therapies in patients with inflammatory bowel disease. Therapeutic iron, as well as heme iron from chronic mucosal bleeding, can increase iron-mediated oxidative stress in colitis. This study was designed to examine the influence of iron supplementation on histological expression and oxidative status relative to 5-ASA treatment and antioxidant treatment.Colitis was induced using the iodoacetamide rat model, and rats were divided into different dietary groups of 6 rats each: 1, normal chow diet (control); 2, diet supplemented with iron; 3, iron supplementation and lycopene; 4, iron and Beta-carotene; 5, 5-ASA; 6, 5-ASA and lycopene; 7, 5-ASA and iron; 8, 5-ASA, iron, and lycopene. The animals were killed after 3 days and the weight of the ulcerated area recorded. Mucosal specimens were histologically evaluated. Myeloperoxidase (MPO) was measured to evaluate inflammatory status (U/g). Malondialdehyde (MDA) was measured in colonic tissue ( micro mol/g) and superoxide dismutase (SOD) in erythrocytes to assess the degree of tissue oxidative stress.Significantly more severe colitis, including necrosis, ulceration, and hemorrhage, was seen in colonic biopsies of rats with colitis when iron was supplemented. This pathology was attenuated when iron was given in combination with 5-ASA and/or lycopene. There was no significant benefit from adding Beta-carotene.Iron supplementation can amplify the inflammatory response and subsequent mucosal damage in a rat model of colitis. We suggest that the resultant oxidative stress generated by iron supplementation leads to the extension and propagation of crypt abscesses, either through direct membrane disruption by lipid peroxidation or through the generation of secondary toxic oxidants. Simultaneous treatment with 5-ASA and/or lycopene minimizes the potential hazard of iron. Therefore, we suggest giving iron supplementation with 5-ASA or lycopene or both.

Budesonide has been found effective in patients with mild and moderate Crohn disease and has been found to cause fewer side effects than prednisone. The use of oral budesonide has not been prospectively evaluated in children with Crohn disease. Therefore, the authors initiated a trial to compare remission and tolerance to budesonide and prednisone in children with mild or moderately active Crohn disease.A prospective randomized open controlled 12-week trial was carried out comparing pH modified release budesonide, 9 mg, versus prednisone, 40 mg, in children with active mild to moderate pediatric Crohn disease.Thirty-three patients (20 boys and 13 girls; mean age, 14.3 years) enrolled and completed the study. The groups treated with budesonide and prednisone did not differ by age, onset of disease, location of disease, or disease activity. The remission rate at 12 weeks was 47% in the budesonide treatment group and 50% in the prednisone treatment group. Side effects occurred in 32% and 71% of patients treated with budesonide and prednisone, respectively (P0.05). Severity of cosmetic side effects was significantly lower in patients treated with budesonide (P0.01).Remission rates for Crohn disease with budesonide and prednisone treatment in this study were similar. Pediatric patients treated with budesonide had significantly fewer side effects than patients treated with prednisone. Budesonide should be considered an alternative to prednisone in pediatric patients with mild to moderate disease activity.

The growth and maturation of the gastrointestinal tract during development is influenced by diverse genetic and growth factors. Since prolactin is abundant in amniotic fluid and breast milk, we hypothesized that it may also affect gut development. The effect of prolactin on thymidine incorporation and tissue alkaline phosphatase, maltase and lactase activity was studied on jejunal explants from fetal, newborn and 2 week-old rats. The results were compared with the effects of epidermal growth factor (EGF) under identical experimental conditions. Prolactin induced a significant increase in proliferation and a two- to threefold increase in maltase and alkaline phosphatase activity of the newborn explants. The effect of prolactin in this group compared to that of EGF was significantly greater with respect to proliferation, and almost identical with respect to the hydrolases studied. These results suggest that prolactin might have a role in the process of growth and maturation of the gut mucosa during ontogeny.

Objectives: Oral budesonide has been found to be efficacious for mild to moderate Crohn's disease in adults, with equal improvement rates for budes-onide and prednisone. We report the results of a retrospective study of budesonide treatment in mild to moderate Crohn's disease in children. Study design: Charts of patients treated with budesonide (n = 62) with a pediatric Crohn's Disease Activity Index of 12.5 to 40 were compared with a cohort of 58 age-matched patients treated with prednisone. Results: Among children treated with budesonide, 48% had remission compared with 77% of the children treated with prednisone ( P = .001). Among patients who had failed previous medical therapy with mesalamine, 59% had remission with budesonide (9 mg/day). Remission with prednisone occurred in 73% of children who failed to achieve remission with budesonide. Patients responding to budesonide had significantly milder disease compared with nonresponders who had remission while taking prednisone. Conclusions: Budesonide is useful in mild to moderate Crohn's disease in children. It is more effective than mesalamine and antibiotics but less effective than prednisone. Budesonide should be considered for first-line therapy in mild to moderate Crohn's disease. (J Pediatr 2002;140:75-80)

Background: Anti-TNFalpha agents are commonly used for ulcerative colitis (UC) therapy in the event of non-response to conventional strategies or as colon-salvaging therapy. The objectives were to assess the appropriateness of biological therapies for UC patients and to study treatment discontinuation over time, according to appropriateness of treatment, as a measure of outcome. Methods: We selected adult ulcerative colitis patients from the Swiss IBD cohort who had been treated with anti-TNFalpha agents. Appropriateness of the firstline anti-TNFalpha treatment was assessed using detailed criteria developed during the European Panel on the Appropriateness of Therapy for UC. Treatment discontinuation as an outcome was assessed for categories of appropriateness. Results: Appropriateness of the first-line biological treatment was determined in 186 UC patients. For 64% of them, this treatment was considered appropriate. During follow-up, 37% of all patients discontinued biological treatment, 17% specifically because of failure. Time-to-failure of treatment was significantly different among patients on an appropriate biological treatment compared to those for whom the treatment was considered not appropriate (p=0.0007). Discontinuation rate after 2 years was 26% compared to 54% between those two groups. Patients on inappropriate biological treatment were more likely to have severe disease, concomitant steroids and/or immunomodulators. They were also consistently more likely to suffer a failure of efficacy and to stop therapy during follow-up. Conclusion: Appropriateness of first-line anti-TNFalpha therapy results in a greater likelihood of continuing with the therapy. In situations where biological treatment is uncertain or inappropriate, physicians should consider other options instead of prescribing anti-TNFalpha agents.

Oral budesonide in adult studies is a potent corticosteroid with decreased systemic bioavailability and an improved adverse effect profile in comparison with prednisone. It has recently been introduced for the treatment of inflammatory bowel disease in Europe, Canada, and Israel. Benign intracranial hypertension has rarely been associated with corticosteroid therapy but has not been reported in association with budesonide therapy. Three adolescents with Crohn's disease and poor nutritional status developed benign intracranial hypertension while receiving oral budesonide. All three patients had previously received multiple courses of prednisone during the course of their disease, without developing intracranial hypertension. Benign intracranial hypertension resolved after medication withdrawal and did not recur with subsequent use of prednisone. Evaluation for benign intracranial hypertension should be considered in patients with inflammatory bowel disease who develop headache while receiving oral budesonide. This side effect may be associated with poor nutritional status. (J Child Neurol 2001;16:458-461).

Hyperbilirubinemia and liver enzyme abnormalities are commonly observed in sepsis. However, the frequency in premature neonates and the specific relation to gram-negative bacteria are not known.Charts of all preterm infants who had positive blood cultures for either gram-negative bacteria or coagulase-negative staphylococci were reviewed. Neonates with gram-negative bacteremia (n = 54) were compared with neonates with coagulase-negative staphylococcal bacteremia (n = 31). In addition infants with gram-negative bacteremia and elevated liver enzymes (n = 25) were compared with infants with gram-negative bacteremia and normal liver enzymes (n = 29).Liver enzyme abnormalities accompanied 46.3% (25 of 54) of gram-negative bacteremia and 12.9% (4 of 31) of episodes of coagulase-negative staphylococcal bacteremia (P = 0.002). Serum concentrations of liver enzymes were significantly higher in infants with gram-negative bacteremia than in those with coagulase-negative staphylococcal bacteremia (P0.0001), but no difference in alkaline phosphatase serum values was observed. Infants with gram-negative bacteremia and elevated liver enzymes were not fed for a longer period than infants with gram-negative bacteremia and normal liver enzymes (7.3 +/- 6.3 days vs. 4.0 +/- 4.3 days, P = 0.03), and this was accompanied by significant conjugated hyperbilirubinemia (P0.0001). Ventilation, total parenteral nutrition and medications were not responsible for the observed differences. Klebsiella pneumoniae bacteremia was commonly associated with elevated liver enzymes (12 of 18), whereas none of the infants with Pseudomonas aeruginosa bacteremia had elevated liver enzymes.Gram-negative bacteremia is commonly associated with cholestasis in premature neonates. Liver enzyme abnormalities are more common than elevated conjugated bilirubin, not all gram-negative bacteria have the same effect and the lack of enteral feeding seems to play a more significant role than the administration of parenteral nutrition.

Postictal psychoses are brief psychotic episodes that usually occur after poorly controlled partial complex seizure clusters. The psychosis commonly appears following a lucid interval, ranging from a few hours to days after seizure termination. An underlying structural brain abnormality is common and usually involves the temporal lobe. Postictal psychosis, while well known in adults, has not been described previously in children. We describe a 9-year-old boy with right hemiparesis due to a neonatal stroke, who developed a postictal schizophrenia-like psychosis following status epilepticus. Electroencephalography showed left-sided slowing. A brain computed tomographic scan and magnetic resonance imaging revealed left hemisphere hypoplasia. A 99mTc-ECD single photon emission computed tomographic scan of the brain revealed decreased left-hemisphere perfusion, most pronounced to the medial temporal lobe. The psychosis resolved gradually over 7 days without antipsychotic therapy. To the best of our knowledge, this is the first description of postictal psychosis in a child. ( J Child Neurol 1999; 14:818-819).

Background. Resolution ofHelicobacter pylori infection is important in the management of peptic ulcer disease and reduces peptic ulcer recurrence in both adults and children. Various anti-H pyloritreatment regimens have been proposed, reflecting the incomplete clinical success of each. A combination of omeprazole, clarithromycin, and tinidazole, given for 1 week, has been shown to be highly tolerable and effective, achieving a success rate of >90% in the adult population. Objective. The aim of this study was to evaluate this short-term regimen in pediatric and adolescent populations. Methods. The study group consisted of 35 children referred for evaluation of dyspeptic symptoms. They all underwent upper gastrointestinal endoscopy, in which H pylori infection was confirmed by rapid urease test and/or histologic staining. They were given omeprazole (20 mg twice daily), clarithromycin (250 mg twice daily), and tinidazole or metronidazole (500 mg twice daily) for 1 week. The patients were divided into two groups: those who received the first course of anti-H pylori therapy during this study (group 1) and those who had previously received standard metronidazole and bismuth combination therapies that failed to eradicate H pylori (group 2). Therapeutic efficacy was assessed by a13C-urea breath test performed 4 weeks after completion of treatment. Results. The 35 study patients had a mean age of 15.9 years (range, 10 to 19) and included 19 males and 16 females, of whom 22 were born in Israel and 13 were immigrants from the former USSR. There were 27 patients (77.1%) in group 1 and 8 patients (22.9%) in group 2. Endoscopic findings were nodular gastritis (14), gastritis (11), gastric ulcer (1), duodenal ulcer (5), and duodenitis (4).H pylori resolution was significantly higher in group 1 patients (24/27, 88.9%) than in group 2 patients (1/8, 12.5%). There was no difference between patients with nodular gastritis and those with nonnodular gastritis, and between Israeli-born patients and patients born in the former USSR. Compliance in both groups was equally good, and no major side effects were recorded. Conclusions. One-week omeprazole/clarithromycin/tinidazole triple therapy is highly tolerable and effective for treating H pylori in the pediatric age group, but previous treatment failure diminishes the likelihood of success with this regimen.

Background: Triple therapy with omeprazole, clarithromycin, and tinidazole (OCT) has been found to be highly effective against Helicobacter pylori infection. However, its efficacy as a second line regimen for patients who failed metronidazole-based triple therapy has not been evaluated. Aim: The aim of this study was to evaluate the efficacy of low-dose, short-term OCT therapy in an Israeli population, and to compare results obtained in previously treated and untreated patients. Methods: Patients with duodenal or gastric ulcers and chronic antral gastritis with H. pylori infection as assessed by rapid urease test and/or 14C urea breath test (14C-UBT), were studied. All patients received omeprazole 20 mg b.d., clarithromycin 250 mg b.d. and tinidazole 500 mg b.d. for 7 days. Eradication was assessed by 14C-UBT 4 weeks after treatment. Results: One hundred and fourty-four patients (M/F=81/63) were enrolled (mean age 48.1 years, range 12–78). Eradication of H. pylori was significantly different between patients who were initially treated with this regimen (90/94, 96%) and patients who had previously failed to eradicate H. pylori with standard triple therapy (27/50, 54%). Moreover, the eradication rate was significantly decreased in patients with more than one previous failure (9/22, 41%) compared to that in patients with only one failure (18/29, 62%). No other differences such as age, gastric pathology, ethnic origin, smoking habits, or pre-treatment urease activity were found to influence the eradication rate. Conclusions: One-week low-dose triple therapy with OCT is highly effective as an initial therapy in eradicating H. pylori infection. The efficacy is significantly lower when given as a second line treatment in patients who have previously failed to eradicate H. pylori with bismuth-based standard triple therapy.

The recent mass immigration to Israel from the former U.S.S.R. and Ethiopia has significantly increased the hepatitis B virus (HBV) carrier rate. Since January 1992, all newborns in Israel have been routinely immunized against HBV. The same routine of vaccination has been used for premature and term newborns. The aim of the present study was to investigate the immune status against HBV in term and preterm infants at the age of 1 year. All infants were vaccinated at day 1 and at 1 and 6 months with Engerix. Hepatitis B antigen antibody (Ab) serum titers were determined at the age of 1 year. All infants had detectable Ab titers (responders); 92.5% had protective titers (10 mlU/ml), out of which 26.5% were low responders (100 mlU/ml) and 65% were high responders (100 mlU/ml). There was no significant difference in immune responses between term and preterm infants.

Malnutrition is known to have adverse effects on the physiology and morphology of the liver. The aim of this investigation was to examine the effect of protein restriction on the content of plasma membrane proteins residing in the sinusoidal and bile canalicular domains of rat liver. Post-weanling rats maintained on low protein isocaloric diets showed marked growth retardation concomitant with reduced liver protein concentration compared to control animals. The content of leucine aminopeptidase, a bile canalicular enzyme, and asialoglycoprotein receptor, a sinusoidal receptor, in livers of protein-restricted rats was 66% and 50%, respectively, of control livers. In contrast, the relative concentrations of dipeptidyl peptidase IV and a cell adhesion molecule (GP 110), both canalicular proteins, were 160% and 121%, respectively, in rat livers upon protein restriction. After a 4-week rehabilitation period, the concentrations of all canalicular membrane proteins were similar to those in control livers, while the sinusoidal receptor was only 68% of control values. Protein restriction was found to adversely affect the concentrations of protein constituents, but not their localization in the hepatocyte plasma membrane. In general, altered concentrations of hepatocyte membrane proteins were reversed on the administration of a normal protein diet.

In the past, biliary lipids of infants and children were studied in duodenal aspirates. This study was performed on original bile aspirated from the gallbladder. The analysis of lipids in bile demonstrated a significantly lower total lipid content in the bile of infants than in children (3.3 g/dl vs. 9.1 g/dl). The most prominent difference was demonstrated in the bile salt concentration (43.2 mM vs. 126.7 mM) and thereafter in the phospholipid content. Infants had a shorter nucleation time and a higher cholesterol saturation index than did children. These results may explain the increased tendency of infants to produce sludge and gallstones during total parenteral nutrition.

PURPOSE: We have used a novel leukocyte adhesiveness/ aggregation test (LAAT) to show that many patients with inflammatory bowel disease (TOD) in clinical remission have a subclinical low grade inflammation. METHODS: Included in the study are 500 controls, 96 patients with IBD in remission, and 106 patients in relapse. RESULTS: The percent of aggregated white blood cells detected in the peripheral blood was 5.9±3.9, 9.1±5.9, and 18.8±9.4, respectively. The difference between each group and any other was significant atP

Peptic disease in the adult population has been estimated to affect as many as 10% of people in the United States. In children, peptic ulcer is uncommon but the exact incidence is unknown. The literature quotes numbers such as four to six new ulcer cases per year or 1 in 2500 pediatric hospital admissions in large pediatric centers. These numbers include primary and secondary ulcers. Since the primary method of diagnosis was changed from radiography to endoscopy, the diagnosis of peptic disease in children has become more accurate, and the histopathology more definite. Endoscopy also has demonstrated what was shown first in adults: the linkage between Helicobacter pylori and peptic disease in children.

We evaluated the efficacy of an oral formulation of 5-amino-salicylic acid in lowering the relapse rate after remission of Crohn's disease. Included were 59 patients who had proven Crohn's disease of at least 1 year's duration, and who had been in continuous remission for at least 6 months, while taking only 5-aminosalicylic acid or no therapy at all. Remission was defined as a Harvey Bradshaw index score (Softley-Clamp modification) of < 4. Patients were given coded mesalzaine 250 mg or placebo tablets (2 x 2 day). They were seen at 0, 1, and 2 months, and then every 2 months until the end of the study. Trial endpoints were 1 year of follow-up, or clinical relapse results. After randomization, 31 patients were included in the placebo arm, and 28 in the treatment arm. There were no significant differences between the two groups at entry. Ten patients were withdrawn from the trial because of noncompliance, loss of follow-up, or headache. There were more clinical relapses in the placebo arm (15 patients, 55%) than in the treatment arm (6 patients, 27%) (p < 0.05). Mesalazine had a significant advantage over placebo (p < 0.05) only in the subgroups of patients with ileal Crohn's disease and in those older than 30 years. We conclude that mesalazine has a moderate but significant benefit in preventing relapse in Crohn's disease in remission; this occurred only in patients with small-bowel involvement or in those older than 30 years.

A 22-year-old female with protein losing enteropathy due to congenital intestinal lymphangiectasia who developed non-Hodgkin's lymphoma of the bone, is described. Complete remission of the lymphomatous process and disappearance of the gastrointestinal symptoms were achieved following treatment with combination chemotherapy and local irradiation.

AIM: To study the management and outcome of children with extrahepatic portal vein obstruction (EHPVO) in a whole country population. METHODS: A nationwide multicenter retrospective case series of children with EHPVO was conducted. Data on demographics, radiographic studies, laboratory workup, endoscopic and surgical procedures, growth and development, were extracted from the patients’ charts. Characteristics of clinical presentation, etiology of EHPVO, management and outcome were analyzed. RESULTS: Thirty patients, 13 males and 17 females, 19 (63.3%) Israeli and 11 (36.7%) Palestinians, were included in the analysis. Age at presentation was 4.8 ± 4.6 years, and mean follow-up was 4.9 ± 4.3 years. Associated anomalies were found in 4 patients. The incidence of EHPVO in Israeli children aged 0-14 years was 0.72/million. Risk factors for EHPVO were detected in 13 (43.3%) patients, including 9 patients (30%) with perinatal risk factors, and 4 patients (13.3%) with prothrombotic states: two had low levels of protein S and C, one had lupus anticoagulant, and one was homozygous for methyltetrahydrofolate reductase mutations. In 56.6% of patients, no predisposing factors were found. The most common presenting symptoms were an incidental finding of splenomegaly (43.3%), and upper gastrointestinal bleeding (40%). No differences were found between Israeli and Palestinian children with regard to age at presentation, etiology and clinical symptoms. Bleeding occurred in 18 patients (60%), at a median age of 3 years. Sclerotherapy or esophageal banding was performed in 20 patients. No sclerotherapy complications were reported. Portosystemic shunts were performed in 11 patients (36.6%), at a median age of 11 (range 3-17) years: splenorenal in 9, mesocaval in 1, and a meso-Rex shunt in 1 patient. One patient underwent splenectomy due to severe pancytopenia. Patients were followed up for a median of 3 (range 0.5-15) years. One patient died aged 3 years due to mucopolysaccharidase deficiency type III. None of the patients died due to gastrointestinal bleeding. CONCLUSION: EHPVO is a rare disorder. The etiological factors are still mostly unknown, and the endoscopic and surgical treatment options ensure a good long-term prognosis.

Superoxide dismutase is the main scavenger of superoxide radicals in the mammalian body. The liver has high levels of two types of superoxide dismutase enzymes, cytosolic Cu-Zn and mitochondrial Mn enzymes. The aim of the present study was to investigate the content of two distinct superoxide dismutases in liver during the perinatal transition from a hypoxic to a hyperoxic environment. Both isozymes were purified to homogeneity and used as immunogens in rabbits. Antisera raised were found to recognize only polypeptides of molecular weight 16,900 or 23,400, which correspond to Cu-Zn and Mn superoxide dismutases, respectively. It was found that the level of Cu-Zn superoxide dismutase enzymatic activity and protein as assessed by immunoquantitation increased 10-fold during the postnatal period, reaching adult levels by 3 weeks. In contrast, the amount of Mn superoxide dismutase content increased only twofold to adult levels during the first week of life. Neither of the superoxide dismutases showed an alteration in specific activity or apparent molecular weight in rat livers during ontogeny. These results show that the levels of two intracellular superoxide dismutases are differentially elevated during the perinatal period. It is suggested that each dismutase plays a different physiological role for superoxide scavenging in liver as a function of the hypoxic/hyperoxic environment at birth.

Communicating esophageal duplication and true congenital esophageal diverticulum are entities rarely encountered during childhood. The authors report an unusual case of midesophageal communicating duplication with secondary upper esophageal obstruction.

This paper reports our experience with transabdominal ultrasound in both the acute phase and follow-up in a patient with corrosive gastritis. The case presented demonstrates that serial sonography can localise the injury, demonstrate its depth and reveal the presence of peristalsis, thereby reducing the radiation exposure resulting from barium studies.

Methane production was studied in 28 cystic fibrosis patients aged 3-16 years and in 290 healthy children. The percentages of methane producers in the cystic fibrosis and control groups were 60.7 and 20.6%, respectively. In the patient group there was no difference between methane producers and nonproducers with respect to the degree of malabsorption or the administration of pancreatic supplements or antibiotics. We hypothesize that the high proportion of methane producers in cystic fibrosis patients may be associated with the presence of specific substrate(s) (glycoprotein?) in their intestinal contents. Changes of the intestinal microenvironment may favor the development of a methanogenic flora.

A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, stea

A 5-year-old boy with known severe combined immunodeficiency disease presented with chronic diarrhea, malabsorption and retarded growth. Candida albicans was found in distal duodenal fluid, and invading the intestinal mucosa. Chronic diarrhea persisted after antimycotic therapy, but responded to treat ment with cholestyramine. Repeated courses of cholestyramine resin over a 6-month period were required for complete resolution of the gastrointestinal symptomatology.

The chymotryptic activity was assessed in 40 newborns and infants age 3-180 days using the NBT-PABA test. Nine newborns were studied serially at different age periods. A group of 18 cystic fibrosis patients and a group of 17 healthy children served as pancreatic insufficient and pancreatic sufficient controls, respectively. The results demonstrate a gradual increase with age of chymotryptic activity, approaching the levels of older children at about 180 days. The newborns who were studied serially demonstrated an individual pattern of increase in their chymotryptic activity. In the first days of life, newborns show low chymotryptic activity similar to that found in cystic fibrosis patients.

Several recent studies have demonstrated the ability of techniques based on immunoadsorption to selectively isolate specialized subregions of membranes, termed domains, which are derived from a larger more complex parent membrane like the plasma membrane. The immunoadsorbent is directed against a specific antigen that resides exclusively or predominantly in the membrane domain to be isolated. Thus, a monospecific antibody to the domain-specific antigen is required. In the present study we developed a method employing a modified immunoblotting strategy which could utilize polyspecific antibodies to isolate membrane vesicles derived from a specific membrane domain of the hepatocyte plasma membrane. We also used specific cell surface labeling of the hepatocyte plasma membrane by lactoperoxidase-catalyzed iodination at 4 °C and preparation of different sized vesicles by sonication to facilitate isolation of the specific domain. For this study, polyspecific antisera were raised in goats against a membrane fraction, denoted N 2u , which is enriched in bile canalicular proteins. This antiserum recognizes, among other antigens, a 110,000 M r polypeptide previously shown to be localized in the bile canaliculus (J. Cook et al. (1983) J. Cell. Biol. 97 , 1823–1833). A monospecific antiserum was raised in rabbits against the rat hepatocyte asialoglycoprotein receptor, a sinusoidal domain-specific set of glycoproteins whose major form has a M r of 43,000. These antisera were each coupled indirectly to different pieces of nitrocellulose by the immunoblotting protocol and were used to isolate membrane vesicles from a crude extract of liver plasma membrane prepared by sonication. The ratio of iodinated asialoglycoprotein receptor to the 110,000 M r polypeptide in vesicles isolated by the affinity nitrocellulose immunoadsorbent method indicate a 10- to 15-fold enrichment of sinusoidal-derived vesicles relative to bile canalicularderived membrane vesicles. These results show that the affinity nitrocellulose immunoadsorbent method can be used to isolate domain-specific vesicles. Further, the affinity immunoadsorbent method described here for the isolation of domains of the plasma membrane is an integrative one allowing isolation of vesicles present in relatively small concentration in crude cell extracts and it requires minimal ultracentrifugation time.