Harry A. Cynamon

Rifampin relieves pruritus in children with cholestatic liver disease

Chronic cholestatic liver disease in children frequently results in severe intractable pruritus. Current forms of therapy, including cholestyramine, are usually ineffective. Therefore, a 6-wk, double-blind, crossover study was designed to test the ability of rifampin to relieve pruritus in children with chronic cholestasis. Rifampin proved effective in alleviating pruritus in all five children tested compared with a placebo-treated group. After the 6-wk study period, rifampin was continued for 6 mo, and its effectiveness was maintained. No complications resulted from rifampin use. This study and a similar study in older patients with primary biliary cirrhosis suggest that a highly effective form of therapy is available for treatment of severe pruritus in patients with chronic cholestasis. These patients must be carefully selected and frequently monitored.

Diagnosis and management of colonic polyps in children

mon in toddlers and preschoolers, but nearly half of the homes had no syrup of ipecac. Stairways are a common site for falls, and yet over half of homes had ungated accessible stairways. The DCHs operated by younger, less well educated women had a higher level of hazards. These operators would be an appropriate target group for education. The DCHs in apartments or in mobile homes, and those in homes that were rented rather than owned, also had a higher level of hazards. These variables probably reflect family income and, taken with the findings about operator education, suggest that lower socioeconomic status of the DCH operator may place the children cared for at greater environmental risk of injury. The DCHs with younger children and increased numbers of children under care were no safer than other homes, despite the increased risk presented by these factors, How generalizable are these results? Because all these operators had chosen to register with the state and had allowed outsiders to visit their homes, we suspect that our population of DCH operators was a relatively motivated group. This study may therefore underestimate the true level of injury hazards in family DCHs that are not regularly visited by outsiders. Conversely, the study may overestimate the level of family DCH injury hazards in states where regular inspections are mandatory. Do family DCHs have more injury hazards than childrens own homes or than other child care settings? Are children in family DCHs actually injured at higher rates? These important questions need to be addressed in future studies. In light of the present findings, however, we suggest that family DCH operators will require more eduation or increased regulation to decrease injury hazards in home day care. In addition, we suggest that pediatricians advise parents whose children are in DCHs to discuss injury prevention with their DCH operators.

Effect of vitamin E deficiency on neurologic function in patients with cystic fibrosis

We evaluated neurologic function in 18 patients, ages 5 to 26 years, with cystic fibrosis. Eight were deficient in vitamin E. Sural nerve conduction latency was increased and nerve action potential amplitude decreased in the vitamin E-deficient group in comparison with the vitamin E-sufficient group. Two vitamin E-deficient patients had absent deep tendon reflexes; findings of clinical neurologic examinations were otherwise normal. We recommend early supplementation with vitamin E for patients with cystic fibrosis who have pancreatic insufficiency, to prevent neurologic dysfunction.

Measurement of plasma prednisolone level to evaluate a prednisone treatment failure in an adolescent with Crohn's disease

A 14-year-old boy experienced multiple hospitalizations because of symptoms due to Crohns disease involving the stomach, duodenum, and ileum. He maintained that oral corticosteroids were not effective for control of his symptoms. However, i.v. corticosteroids always relieved his symptoms. To resolve the question of noncompliance versus altered corticosteroid absorption or metabolism, our patient underwent an oral prednisone absorption study. Prednisolone, the active metabolite of prednisone, was measured in his plasma using a high-pressure liquid chromatography technique. The results led to the discovery of an elaborate deception by the patient and his subsequent need for psychotherapy. This report documents the importance of measuring plasma prednisolone concentrations to diagnose noncompliance, especially in adolescents who are overly concerned about their body image.

Support for a conservative approach to mixed intrahepatic and extrahepatic biliary hypoplasia

This report describes the pitfalls in the diagnosis of extrahepatic biliary atresia in an infant with paucity of the interlobular bile ducts.

637 A FUNCTIONAL MEASURE OF VITAMIN E VALIDATED

A sensitive, standardized method of measuring a biological function of vitamin E(E) is needed to define a protective E level. We therefore evaluated the release of thiobarbituric acid reacting substances(TBARS) from red blood cell(RBC) membranes after paired incubations in buffered H2O2, pH 7.4, alone and with azide to promote maximal release. TBARS was quantitated spectro-photometrically. Values were expressed as the ratio of TBARS release without azide/maximal release × 100 (%TBARS). Individual fasting plasma lipid levels were added to give total lipids(TL). Three groups were compared: A) E sufficient noncholestatic children (N=14); B) E sufficient noncholestatic adults (N=6); C) E deficient cholestatic children (N=11).Results show a strong negative correlation in all subjects between %TBARS and E levels, E/TL, E/cholesterol (r=-.84, -.89, -.89, p<.001). Significant differences in E and E/TL in groups A or B vs C are reflected by marked differences in %TBARS release (p<.001). Groups A+B had higher maximal TBARS release vs C (p<.05). Values listed below are mean ±SD.We conclude that %TBARS release provides a sensitive measure of E level and biologic function that could be utilized in clinical assessment of E-sufficiency.