The 1-hr blood xylose test in the evaluation of malabsorption in infants and children.

One hundred ninety-seven 1-hr blood xylose absorption tests with 5 g of D-xylose were performed on 171 infants and children for the evaluation of malabsorption. The mean +/- 1 SD blood xylose level in 78 controls was 37.0 +/- 7.7 mg/dl, and 71 patients with post infectious diarrhea was 28.4 +/- 11.0 mg/dl. A significant reduction (P < 0.001) was found in 10 patients with Celiac disease (12.0 +/- 8.6 mg/dl) and 18 patients with intractable diarrhea syndromes (18.3 +/- 12.5 mg/dl). Forty-four patients had a blood xylose level less than 20 mg/dl, and the majority (35/44) were in the groups with postinfection and intractable diarrhea syndromes. Multiple tests were performed on 20 patients. Sixty-two intestinal biopsies were correlated with 1-hr blood xylose levels. The rate of false positives was 0. The rate of false negative tests varied between 4 to 32.7% depending on the assessment of intestinal damage. Our results confirm that the 1-hr blood xylose absorption test is a sensitive indicator of severe intestinal damage. Intestinal biopsy should be considered in all patients with a 1-hr blood xylose level less than 20 mg/dl. With borderline results (20 to 25 mg/dl), clinical decisions must be individualized.

Essential fatty acid status and fluidity of plasma phospholipids in cystic fibrosis infants.

The fatty acid (FA) patterns of cord serum phospholipids (PLs) were examined in 4 cystic fibrosis (CF) newborns, 8 non-CF siblings, and 22 normal control subjects. Plasma from 27 newly diagnosed CF infants and 38 normal infants aged less than 2 y were studied for comparison. CF cord-blood PLs had patterns similar to those of CF siblings and to normal newborns, but the pattern for CF did not shift toward adult patterns during infancy as did patterns for normal infants. CF infants at diagnosis exhibited a deficiency pattern in which 18: 2 omega 6, 20:4 omega 6, 22:4 omega 6, an omega 3 polyunsaturated fatty acids (PUFAs) were significantly subnormal and 18:3 omega 6, total saturated fatty acids, and total monounsaturated fatty acids were significantly elevated compared with normal infants. In PLs in CF infants, although mean chain length of FAs was low, mean melting point was elevated 2.4 degrees C and double-bond index was low (both P less than 0.001), implying a significantly lessened fluidity. Nutritional supplements of both omega 6 and omega 3 PUFAs are cated.

Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation.

The fatty acid compositions of serum phospholipids, cholesteryl esters, triglycerides, and free fatty acids were determined on a group of cystic fibrosis patients. These were compared with similar data from random hospitalized patients of the same age groups of both sexes. Fatty acid patterns in all lipid classes were skewed in the direction of essential fatty acid deficiency, but the differences were most dramatic in phospholipids. Many calculated parameters useful as indices of essential fatty acid status indicated that essential fatty acid deficiency exists in cystic fibrosis. Treatment of 11 cystic fibrosis patients with safflower oil (1 g/kg/day) failed to correct the aberrations in fatty acid pattern. The biochemical data suggest that there may be an impairment in conversion of linoleate to arachidonate as well as an impairment of absorption.

Cholestyramine therapy and intestinal obstruction in infants.

Two infants are reported who developed partial intestinal obstruction following cholestyramine therapy for intrahepatic cholestatic syndromes. The hazards of this potential side effect of cholestyramine therapy are emphasized.

Selenium and glutathione peroxidase levels in cystic fibrosis.

Whole blood selenium and glutathione peroxidase levels were measured in 20 infants and children (aged 6 months to 15 years) with cystic fibrosis. The whole blood selenium concentration in cystic fibrosis was 0.122 +/- 0.025 microgram/gm. Although the levels of selenium in cystic fibrosis children were below the levels found in a study of healthy children (0.223 +/- 0.007 microgram/gm), they are comparable to those found in children with phenylketonuria treated dietetically and exceed the blood selenium level of healthy children in New Zealand. Levels of the selenoenzyme glutathione peroxidase in children with cystic fibrosis (0.042 +/- 0.007 units/mg Hb) were in the normal range (0.035 +/- 0.003 units/mg of Hb). These results do not support the hypothesis that deficiency of selenium is reponsible for cystic fibrosis.

Negative effects of oral fatty acid supplementation on sweat chloride in cystic fibrosis.

Essential fatty acid supplementation with oral safflower oil (1 gm/kg/day) to 11 cystic fibrosis patients (aged 6 months to 14 years) for one year produced no significant change in sweat chloride concentration (mEq/liter) or sweat rate (gm/min/m2), Addition of vitamin E (10 mg/kg/day) to the safflower oil had no effect on sweat chloride concentration or rate compared to placebo. No clinical improvement could be detected compared to a control group. These results do not support previous reports of the effects of fatty acid supplementation on sweat electrolyte concentrations in cystic fibrosis.

Hepatobiliary scintigraphy in children with cystic fibrosis and liver disease.

UNLABELLED: Intra- and extrahepatic impairment of biliary drainage is important in the pathogenesis of liver disease in cystic fibrosis. Distal common bile duct obstruction is reported to occur in 13% to 96% of these patients. Between 1975 and 1993, 17 of 372 children (4.5%) with cystic fibrosis attending The Children's Memorial Medical Center in Chicago had liver disease based on clinical and laboratory findings. METHODS: Hepatobiliary scintigraphy (HBS) with 99mTc-DISIDA was performed on 12 of the 17 children (mean age at the time of exam was 9 yr, with a range of 1 mo to 21 yr). RESULTS: All had hepatomegaly, four had splenomegaly and two had bleeding esophageal varices. Twenty HBS exams on these 12 patients documented nonvisualization of the gallbladder in 7, dilated intrahepatic ducts in 6 (only the left lobe was involved in 3 patients), nonvisualization of bowel in two, delayed peaking time in the liver (> 10 min) in four patients, and delayed clearance from the liver parenchyma (T1/2 > 20 min) in 11. There appears to be a spectrum of abnormal HBS findings in cystic fibrosis patients with liver disease. These are delayed clearance of liver parenchyma, nonvisualization of the gallbladder and dilated intrahepatic ducts with a predilection for the left lobe of the liver. These abnormal findings fluctuate in time and may not correlate with the findings on ultrasonography. CONCLUSION: Quantitative hepatobiliary scintigraphy is a valuable tool in the evaluation and management of the liver disease in this patient population.

Serum glycine-conjugated bile acids in pediatric hepatobiliary disorders.

Measurements of serum bile acids (glycine conjugates of cholic, chenodeoxycholic, deoxycholic, and lithocholic acids) by radioimmunoassay in a variety of pediatric hepatobiliary disorders showed elevations in neonatal hepatitis syndromes, cholestasis, and hepatitis of extrahepatic or intrahepatic origin. Measurements of individual serum bile acids failed to differentiate between the various neonatal hepatitis syndromes. In one patient with cholestasis, the increased levels of bile acids observed returned to normal following therapy with cholestyramine and phenobarbital. In chronic active hepatitis the serum bile acid values correlated well with the bilirubin and SGOT in response to therapy with corticosteroids. These data confirm suggestions that serum cholylglycine and chenodeoxycholylglycine levels are a sensitive indicator of disturbed hepatic function and can be used in monitoring the course, activity, and therapeutic response in various hepatitis syndromes. In Reye's syndrome and protracted diarrhea of infancy, elevations in serum bile acids were detected without associated hyperbilirubinemia and provided additional evidence of disturbed hepatic function.

Fat intake is low in cystic fibrosis despite unrestricted dietary practices.

A prospective study of caloric intake and specific nutrient composition was performed in 64 cystic fibrosis patients aged 0.3 to 18 yr. Linear regressions and multiple stepwise regressions revealed no significant correlations between any individual dietary parameter and age, height, weight, percentile of weight or height, Shwachman Score, and pulmonary parameters. The caloric intake varied widely in individual patients and in different age groups. Despite unrestrictive dietary practices, the fat intake (33.7% +/- 8) was below that recommended for these linoleate deficient patients.

Improved nutritional management reduces length of hospitalization in intractable diarrhea.

Sixteen patients were managed by one of two specific refeeding protocols to compare the efficacy of two enteral formulas in the nutrition restoration of infants with intractable diarrhea (IDI). The protocols outlined specific nutritional therapy including transition from parenteral to enteral nutrition; concentration, volume, and steps of advancement of formulas. Patient progress was monitored daily. Average length of stay, number of days on parenteral nutrition support, and number of formula changes for the IDI protocol groups were compared with a retrospective chart audit group of 29 IDI patients. The protocol groups had substantially fewer days of parenteral nutrition support, significantly fewer formula changes (p less than 0.01), and fewer days of hospitalization. The differences resulted in $14,750 of charges saved per protocol patient.

Hirschsprung's enterocolitis, prostaglandins, and response to cholestyramine.

An infant with Hirschsprung's enterocolitis developed a fulminant secretory diarrhea unresponsive to all conventional therapy until cholestyramine was administered. A 12-fold decrease in prostaglandin E (PGE) levels in the colostomy fluid was documented in response to cholestyramine therapy. It is postulated that increased PGE activity, enterotoxin, and bile acid malabsorption may be involved in the enterocolitis of Hirschsprung's disease.

Menetrier's disease in childhood: report of two cases and a review of the literature.

Two new cases plus 13 previously reported cases of Menetrier's disease in childhood are reviewed. The most common features of this illness are severe upper gastrointestinal symptoms (abdominal pain, nausea, vomiting, hematemesis) followed by the onset of generalized edema and ascites. Significant laboratory findings include hypoalbuminemia, eosinophilia, and anemia. Gastrointestinal protein loss and decreased gastric acid secretion can be documented. Upper gastrointestinal radiographs demonstrate the characteristic hypertrophic gastric rugae. Histologic features include hypertrophic tortuous gastric glands, basilar cysts, and interstitial round cell inflammation. The natural course of this disease in childhood is usually benign and self-limited. Uncommonly, the course may be severe and require gastric resection. This is in contrast to the adult form, where chronicity and severity is the rule. The etiology of this problem remains unknown. Endoscopy and biopsy are the diagnostic procedures of choice, although laparotomy may be necessary in equivocal cases. Therapy should be supportive except for those few patients who require surgery to control hemorrhage.

Mortality from liver disease in children. Implications for hepatic transplantation programs.

Mortality from liver disease in a pediatric hospital was reviewed to assess the implications for hepatic transplantation programs. Between 1976 and 1983, 81 children died of hepatic failure that included biliary atresia (n = 20), metabolic disorders (n = 22), Reye's syndrome (n = 7), infections (n = 15), cholestatic syndromes (n = 12), and miscellaneous causes (n = 5). Hepatic failure was considered the secondary cause of death in only five patients. Acute hepatic failure was present in 42% (34/81) of patients, whereas 58% (47/81) had preexisting chronic liver disease. Forty (49.4%) of the 81 patients died in infancy, including two thirds of the patients with biliary atresia. Liver disease accounted for 6.6% (81/1,225) of all deaths in our hospital during this eight-year period. During this interval, eight patients (six alive) underwent liver transplantation. Approximately 2.9 patients per year will be candidates for liver transplantation.