John D. Lloyd-Still

A Clinical Scoring System for Chronic Inflammatory Bowel Disease in Children

A clinical scoring system for the assessment of children with chronic inflammatory bowel disease has been devised. A close correlation is demonstrated between severity of disease and the level of serum albumin. The clinical score is simple to perform, sensitive to changes in clinical status, reproducible by different observers, and specifically designed to evaluate inflammatory bowel disease in children and adolescents. The clinical score is a useful adjunct in the management of children with chronic inflammatory bowel disease and can be used in prospective studies of various therapeutic modalities.

Crohn's disease and cystic fibrosis

Advances in investigative techniques have led to increasing reports of Crohns disease in CF patients. A retrospective review of the literature on IBD in CF showed findings characterized by ileocolitis with fistula formation; 83% required surgery. A prospective survey of 11,321 CF patients attending 49 CF centers revealed 28 with IBD (25 Crohns, three ulcerative colitis), ages 4–20 years, mean 15.6 years. The prevalence rate of IBD (247/105) was 7× controls and was accounted for by Crohns disease (221/105) which was 17× controls. The mechanisms that predispose CF patients to Crohns disease are discussed.

Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis

Essential fatty acid (EFA) deficiency is a predisposing factor for pulmonary infection with Staphylococcus aureus and Pseudomonas aeruginosa, the two major pathogenic microorganisms in cystic fibrosis (CF). Objective: The goal of this study was to investigate the essential fatty acid status of CF patients from infancy to 20 years old. Materials and methods. Plasma fatty acid profiles for phospholipid (PL) were determined for cord (n= 6), 4 months (n= 40), 16 months (n= 25), 3 y (n= 8), 5‐10 y (n= 10), and 10‐20 y (n= 10) aged CF patients and compared to their respective control; cord (n= 22), 1‐36 months (n= 38) and adult (n= 100). Significance was established by Students t‐test (p < 0.05). Results: The plasma PL fatty acid profile for all CF patients, except cord, revealed consistent deficiency in ω3 and ω6 EFAs. These deficiencies were most marked at infancy and more pronounced for patients with meconium ileus. Conclusions and relevance: EFA deficiency may contribute to the predisposition of CF infants to develop respiratory disease and to the excess cytotoxic activity found in bronchoalveolar lavage fluid at 2 months of age in the majority of screened infants.

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

Carnitine metabolites in infants with cystic fibrosis: a prospective study.

14,750 of charges saved per protocol patient.

Acylcarnitine is Low in Cord Blood in Cystic Fibrosis

Acylcarnitine is low in cord blood in patients with cystic fibrosis, suggesting that fatty acid metabolism is disturbed in utcro. Carnitine metabolites (total, free, short‐ and long‐chain acylcarnitine) were measured prospectively in 23 newly diagnosed infants with cystic fibrosis treated with a carnitine‐containing, predigested formula for 6–12 months. Total (p < 0.002), free (p < 0.004), and long‐chain (p < 0.001) plasma concentrations of carnitines were significantly less than controls (n = 48) at diagnosis. Total and free concentrations were corrected with nutritional management, whereas short‐and long‐chain acylcarnitines remained unchanged. By three years of age all plasma concentrations of carnitine metabolites were significantly less than controls despite a carnitine‐containing diet. Urinary carnitine metabolites were increased at diagnosis and follow‐up. The physiological significance of these observations in cystic fibrosis is unknown, but could be compatible with disturbed regulatory control with resultant increased utilization.

Complex carbohydrate intolerance: Diagnostic pitfaiis and approach to management

ABSTRACT. Carnitine metabolites (total, free, short and long chain) were analyzed in cord blood of cystic fibrosis (n=5), non‐CF siblings (n=7), and controls (n=8). Total acylcarnitine (short and long chain combined) was significantly lower (<0.001) in CF compared to both control groups. Total and free carnitine showed no significant differences between the three groups. These findings are compatible with disturbed fatty acid metabolism in utero and may be related to the increased energy expenditure characteristic of CF infants.

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

Complex carbohydrate intolerance occurred in three of 105 patients with protracted diarrhea of infancy. Nosocomial gastroenteritis complicated a primary disorder of carbohydrate absorption (primary glucose galactose malabsorption, two; primary sucrase isomaltase deficiency, one) in all patients. Their course was characterized by protracted diarrhea, variable degrees of villus atrophy on intestinal biopsy tissue, and negative caloric balance requiring intravenous alimentation for periods varying from 6 to 16 weeks. Dietary management required rigid exclusion of all offending carbohydrates from the diet. Delay in the diagnosis of primary carbohydrate intolerance varied from 2 weeks to 6 months. Complex carbohydrate intolerance may be more common than has been reported, and should be considered in all infants with protracted diarrhea of infancy when there is persistent carbohydrate intolerance.