Marilyn R. Brown

Deliberate overfeeding in women and men: energy cost and composition of the weight gain.

1. Thirteen adult females and two males were overfed a total of 79-159 MJ (19,000-38,000 kcal) during a 3-week period at the Clinical Research Center, Rochester. The average energy cost of the weight gain was 28 kJ (6.7 kcal)/g, and about half the gain consisted of lean body mass (LBM) as estimated by 40K counting. 2. A survey of the literature disclosed twenty-eight normal males and five females who had been overfed a total of 104-362 MJ (25,000-87,000 kcal) under controlled conditions: twenty-five of these had assays of body composition, and three had complete nitrogen balances. 3. When these values were combined with those from our subjects (total forty-eight), there was a significant correlation between weight gain and total excess energy consumed (r 0.77, P less than 0.01) and between LBM gain and excess energy (r 0.49, P less than 0.01). Based on means the energy cost was 33.7 kJ (8.05 kcal)/g gain and 43.6% of the gain was LBM; from regression analysis these values were 33.7 kJ (8.05 kcal)/g gain and 38.4% of gain as LBM. 4. Individual variations in the response could not be explained on the basis of sex, initial body-weight or fat content, duration of overfeeding, type of food eaten, amount of daily food consumption or, in a subset of subjects, on smoking behaviour. 5. The average energy cost of the weight gain was close to the theoretical value of 33.8 kJ (8.08 kcal)/g derived from the composition of the tissue gained.

Parenteral Nutrition–Associated Cholestasis in Neonates: Multivariate Analysis of the Potential Protective Effect of Taurine

BACKGROUND Neonates receiving parenteral nutrition (PN) are at risk for PN-associated cholestasis (PNAC); however, no preventive factors for PNAC have been clearly identified. Despite reports suggesting that taurine may prevent PNAC in neonates, such an effect of taurine has not yet been definitively demonstrated. We determined whether taurine supplementation reduces the incidence of PNAC in premature or critically ill neonates. METHODS This study was part of a prospective, randomized, multi-institutional trial designed to assess cholecystokinin vs placebo as a potential preventive therapy of PNAC. Taurine supplementation of PN varied between institutions. The presence or absence of taurine in PN was analyzed by multivariate analysis, with a primary outcome measure of serum conjugated bilirubin (CB) as a measure of PNAC. RESULTS Taurine reduced PNAC in premature infants (estimated maximum CB [95% confidence interval] 0.50 mg/dL [-0.17 to 1.18] for those receiving taurine, vs 3.45 mg/dL [1.79-5.11] for neonates not receiving taurine, approaching significance, p = .07). Taurine significantly reduced PNAC in infants with necrotizing enterocolitis (NEC; estimated maximum CB 4.04 mg/dL [2.85-5.23], NEC infants receiving taurine, vs 8.29 mg/dL [5.61-10.96], NEC infants not receiving taurine, p < .01). There were too few neonates with surgical anomalies to evaluate the effect of taurine in this group. CONCLUSIONS Within specific subgroups of neonatal patients, taurine supplementation does offer a very significant degree of protection against PNAC. Patients with NEC or severe prematurity are most likely to benefit substantially from taurine supplementation.

Use of Cholecystokinin-Octapeptide for the Prevention of Parenteral Nutrition-Associated Cholestasis

Objective. To determine whether cholecystokinin-octapeptide (CCK-OP) would prevent or ameliorate parenteral nutrition-associated cholestasis (PNAC) among high-risk neonates treated with total parenteral nutrition. Study Design. This was a multicenter, double-blind, randomized, controlled trial conducted between 1996 and 2001. Patients. Neonates at risk for the development of PNAC included very low birth weight neonates and those with major surgical conditions involving the gastrointestinal tract. Setting. Tertiary care hospitals. Intervention. Patients were randomized to receive CCK-OP (0.04 μg/kg per dose, twice daily) or placebo. Eligible infants were all <30 days of age. Patients were enrolled within 2 weeks after birth or within 7 days after surgery. Outcome Measures. The primary outcome measure was conjugated bilirubin (CB) levels, which were measured weekly. Secondary outcome measures included incidence of sepsis, times to achieve 50% and 100% of energy intake through the enteral route, number of ICU and hospital days, mortality rate, and incidences of biliary sludge and cholelithiasis. Results. A total of 243 neonates were enrolled in the study. CCK-OP administration did not significantly affect CB levels (1.76 ± 3.14 and 1.93 ± 3.31 mg/dL for CCK-OP and placebo groups, respectively; mean ± SD). Secondary outcome measures also were not significantly affected by the study drug. Conclusions. Use of CCK-OP failed to reduce significantly the incidence of PNAC or levels of CB. CCK-OP had no effect on other secondary measures and should not be recommended for the prevention of PNAC.

Multiple esophageal rings: An association with eosinophilic esophagitis. Case report and review of the literature

Esophagitis may present endoscopically with erythema, edema, loss of vascular pattern, friability, and ulceration of the esophageal mucosa. Left untreated, chronic esophagitis may result in stricture formation. The presence of multiple concentric rings involving the entire esophagus has been cited as a chronic form of esophagitis. We present a case of an 8-yr-old boy with multiple concentric esophageal rings and histological evidence of eosinophilic esophagitis, who failed medical antireflux treatment and responded to an elimination diet.

Decreased cholestasis with enteral instead of intravenous protein in the very low-birth-weight infant

Thirty to 50% of very low-birth-weight infants have parenteral nutrition-associated cholestasis. To test the hypothesis that the incidence of cholestasis would be decreased if parenteral amino acids were avoided and protein given enterally, infants with a gestational age of less than 30 weeks were randomized to two groups. One group received amino acid-free parenteral nutrition and whey protein enterally with added premature infant formula. The control group received standard parenteral nutrition with amino acids and enteral premature formula. At the end of 3 weeks of parenteral nutrition, infants who had a direct serum bilirubin level of greater than 3 mg/dl were considered to have significant cholestasis. Twenty-nine infants required parenteral nutrition for 3 weeks, 17 in the whey group and 12 in the control group. No instances of significant cholestasis were observed in the whey group (0/17), whereas seven of 12 infants (58%) in the amino acid control group had cholestasis (p less than 0.001).

“Liver function tests” are not always tests of liver function

A child with Wilms tumor and a child with immune thrombocytopenic purpura (ITP) were each noted to have persistent elevations of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH). Both children underwent thorough evaluation for liver disease and, as a result, experienced delays in treatment of the Wilms tumor and ITP. Eventually both children were found to have extremely elevated serum creatine kinase (CK). Muscle biopsy confirmed diagnoses of Duchennes muscular dystrophy in one child, and Beckers muscular dystrophy in the second. Hematologists/oncologists should consider obtaining a serum CK to rule out muscle disease in patients with unexplained elevations of AST, ALT, and LDH. Am. J. Hematol. 66:46–48, 2001.

Viral Serology (Hepatitis B Virus, Cytomegalovirus, Epstein‐Barr Virus) and Abnormal Liver Function Tests in Transfused Patients with Hereditary Hemorrhagic Diseases

Posttransfusion hepatitis and asymptomatic liver disease remain serious and unresolved problems in transfused patients with hereditary hemorrhagic diseases. We studied the occurrence of antibody and antigen to hepatitis B virus (anti‐Hbs and HBsAg) as well as antibody to cytomegalovirus (anti‐CMV) and Epstein‐Barr virus (anti‐EBV) in this population and correlated this with liver function tests. The study population was divided into two groups: Group I, consisting of 35 patients (moderate to mild disease) requiring less than 12 transfusions per year and Group II, consisting of 38 patients (severe disease) requiring more than 12 transfusions per year. Frequency of transfusion correlated with detectable anti‐HBs and anti‐CMV (48.6% and 25.7% in Group I, 94.7% and 47.4% in Group II). Three patients, all in Group I, were HBsAg positive while none in Group II had demonstrable antigen. Anti‐EBV occurred with similar frequency in both groups. The abnormal liver funtion tests present in 62.9 per cent of Group I and 89.5 per cent of Group II correlated poorly with the presence of anti‐HBs, anti‐CMV and anti‐EBV. Splenomegaly was not detected in any of the 73 patients. HBV and CMV appear to be transmissible by transfusion, and other viruses such as non‐A, non‐B may account for this liver dysfunction.

Multiple esophageal rings: an association with eosinophilic esophagitis

Esophagitis may present endoscopically with erythema, edema, loss of vascular pattern, friability, and ulceration of the esophageal mucosa. Left untreated, chronic esophagitis may result in stricture formation. The presence of multiple concentric rings involving the entire esophagus has been cited as a chronic form of esophagitis. We present a case of an 8-yr-old boy with multiple concentric esophageal rings and histological evidence of eosinophilic esophagitis, who failed medical antireflux treatment and responded to an elimination diet.

Parenteral Fish Oil-Associated Burr Cell Anemia

We report the development of burr cell anemia in an infant with short bowel syndrome who received parenteral fish oil (Omegaven, Fresenius-Kabi, Graz, Austria) after development of total parenteral nutrition-associated liver disease. Parenteral fish oil was discontinued, and the burr cell anemia disappeared, suggesting that parenteral fish oil might be associated with hemolytic anemia.

Use of psyllium in the management of chronic nonspecific diarrhea of childhood.

The etiology and treatment of chronic nonspecific diarrhea of childhood is still poorly understood. We evaluated 23 children with this disorder in whom other etiologies of chronic diarrhea had been ruled out. The children were treated first with an unrestricted diet for 1 week, and if no response was obtained they were then treated with psyllium bulk agents for 2 weeks (1 tablespoon b.i.d.). Eighty-seven percent of the patients responded to therapy. Seven patients responded to an unrestricted diet only, and 13 responded to psyllium. Only three patients (13%) did not respond. Most children were on a restricted diet at presentation, but the amount of dietary fat intake did not correlate with the response, contrary to other reports. Treatment with normalization of the diet and psyllium bulk agents seems to be an effective mode of therapy for chronic nonspecific diarrhea of childhood.