Metabolic liver diseases in childhood.
Explore the source record for details and available documents.
Child↗
Biomedical subjects
R W Shepherd
Publications and source records attributed to R W Shepherd.
Resting energy expenditure of children attending a rehabilitation programme following head injury.
STATEMENT OF PURPOSE: Increased resting energy expenditure following head injury is well documented, but whether this increase extends into rehabilitation and whether this is affected by changes in body composition have not been studied. The aim of this study was to determine whether children attending a rehabilitation program following head injury had altered energy expenditure and body composition. METHODS: Measurements of resting energy expenditure by indirect calorimetry were performed in 21 head injured children (mean age 10.2 +/- 3.8 years). Measurement of body composition was performed using total body potassium. RESULTS: Measured resting energy expenditure values were widely distributed, ranging from 52.3-156.4% of predicted values, yet the mean percentage predicted using Schofield weight, Schofield weight and height and World Health Organization predictive equations were 97.5%, 97.4% and 98.6%, respectively. Mean percentage of expected total body potassium for weight, height and age for head injured children were 85.1 +/- 15.5%, 89.1 +/- 14.1% and 86.9 +/- 15.9%, thus all showed significant depletion. CONCLUSIONS: During rehabilitation, using predictive equations to estimate resting energy expenditure in this group revealed a small bias on average but very large bias at the individual level. Head injured children had altered resting energy expenditure and body composition.
Adolescent↗
Reduced body cell mass following severe head injury in children: implications for rehabilitation.
STATEMENT OF PURPOSE: Increased proteolysis, muscle catabolism and altered body composition have been well documented after severe head injury, but the extent of these effects in children, and whether they extend into rehabilitation, have not been studied. This study determined nutritional status and body composition, with particular reference to the body cell mass (BCM), of head injured children at entry into a rehabilitation programme, and compared body composition analysis with anthropometric nutritional assessment. METHODS: Nineteen head injured children (nine males, 10 females, mean age 9.1 +/- 4.3 years range 1.2-15.1 years) were measured for height, weight and total body potassium (TBK, a measure of body cell mass) on referral to rehabilitation after the acute phase (mean 38.1 days post-injury). Data was compared with expected normative data derived from healthy age and gender matched children. Nutritional status was determined by two separate criteria based on either anthropometric or body composition methods. RESULTS: The mean percentage of expected TBK for height was 84.4 +/- 15%, significantly below the clinically acceptable level for body cell mass (90% of expected). Using the anthropometric definition, only 1/19 was undernourished, whereas 12/19 had poor nutritional status using body composition (chi 2 = 7.58, p = 0.006). CONCLUSIONS: The data revealed a significant depletion in the metabolically active BCM in the presence of normal anthropometry, suggestive of significant muscle wasting. These findings have important pathophysiological and clinical implications in the rehabilitation of children following major head trauma.
Adolescent↗
Evaluation of endoscopic sclerotherapy of esophageal varices in children.
Sixteen children (3.5-14.7 years) with portal hypertension and variceal hemorrhage were treated by direct endoscopic variceal sclerotherapy. Follow-up clinical and endoscopic evaluations have been carried out over a 1-6-year period (mean, 2.3 years). Prior to sclerotherapy, three patients had undergone unsuccessful surgical approaches, and the mean transfusional requirement from the time of the first hemorrhage was 12.3 units of blood per child per year for those with extrahepatic portal vein obstruction (n = 10) and 3.8 units for those with primary liver disease. Following sclerotherapy, transfusional requirements were significantly reduced in both groups, to 1.8 units and 2.2 units (85% and 43% reduction, respectively). Re-bleeding in four patients was due to the occurrence of gastric varices. One patient with extrahepatic portal hypertension has required subsequent shunt surgery for this complication, 5 years after sclerotherapy. Two patients have died, one with cystic fibrosis and hemorrhage from gastric varices and one from liver failure due to progressive biliary cirrhosis. Complications of sclerotherapy included transient retrosternal pain (eight occasions), esophageal ulceration with bleeding (n = 1) and esophageal stricture (n = 1). We conclude that sclerotherapy is an effective technique in obliterating esophageal varices and reducing the risk of hemorrhage in children with portal hypertension, with an acceptably low complication rate. We favor its use over more invasive surgical measures for control of acute and recurrent variceal hemorrhage, particularly for cases of extrahepatic portal hypertension in which a favorable natural history is likely.
Adolescent↗
Longitudinal study of the body composition of weight gain in exclusively breast-fed and intake-measured whey-based formula-fed infants to age 3 months.
Body composition and the composition of weight gain in 82 healthy infants during the first 3 months of life were measured in relation to the type of feeding and nutrient intakes by serial measurements of total body potassium (TBK), anthropometry, and formula intake. Infants were exclusively fed either breast milk (n = 34, B fed) or whey-based formula (n = 48, F fed). Formula intakes were substantially lower than WHO-FAO recommendations (although serial weights paralleled standards), and were significantly greater in male infants, who had greater gains in weight and lean tissue than females. In both sexes, tissues low in K (fat and extracellular water) were added faster to the body weight than lean tissue. Overall, compared with B fed infants, F fed infants gained less weight between birth and 10 days and more weight between 10 and 90 days, although mean actual weights at 90 days were similar; formula feeding caused greater fat deposition in males and greater daily gains in lean mass in females. Differences in both quantity and quality of ingested nutrients account for these differences although the biological importance of these findings is speculative. These studies further elucidate the nature of growth in relation to modern feeding practices and the question of optimal versus maximal nutrient intake and growth in early life.
Anthropometry↗
Pre-operative nutritional support in children with end-stage liver disease accepted for liver transplantation: an approach to management.
Pre-operative nutritional support was studied in 28 children with end-stage liver disease awaiting orthotopic liver transplantation. Nasogastric supplemental administration of a standard semi-elemental enteral nutritional formula was compared with a similar formula enriched with branched chain amino acids, and with a group receiving oral nutrition only. The duration of treatment in all groups was similar (mean 90 days). Energy intakes in the supplemented groups were 120-150% of recommended daily intakes (RDI), whereas ad libitum intakes in the oral group ranged 58-100% RDI. A significant improvement in mean Z-score for body weight (denoting catch-up) was noted only in those children who received nasogastric supplements enriched with branched-chain amino acids. The standard enterally-fed group maintained their body weight and Z-scores did not change significantly. In contrast, body weight Z-scores in those fed orally declined significantly. Nutritional supportive therapy of malnourished children with end-stage liver disease can minimize or improve nutritional status in children awaiting liver transplantation. The use of nutritional formulae rich in branched-chain amino acids may have nutritional advantages in children with chronic liver disease which require further study and evaluation.
Amino Acids, Branched-Chain↗
Therapeutic upper gastrointestinal endoscopy in children: an audit of 443 procedures and literature review.
The safety, effectiveness and capabilities of therapeutic upper fibreoptic endoscopy in children undergoing therapeutic endoscopic procedures (n = 443) was studied. Therapy for gastrointestinal bleeding formed the major group (injection sclerotherapy for varices, n = 197 procedures; thermocoagulation for haemorrhagic gastritis, n = 1; and photocoagulation for Dieulafoy's disease, n = 1). Sclerotherapy was 97% effective in controlling acute bleeding and 84% effective in obliterating varices with no serious complications or deaths. Oesophageal dilatations for surgical, caustic, congenital and peptic strictures and achalasia (n = 193) were performed with no oesophageal perforations or deaths. Foreign bodies were retrieved (n = 34) with no failures or complications. Percutaneous endoscopic gastrostomy was performed (n = 11) with one failure, proceeding to an unsuccessful surgical gastrostomy. Miscellaneous procedures included endoscopic transpyloric tube placement (n = 5) and endoscopic diathermy of pyloric web (n = 1). Therapeutic fibreoptic endoscopy is therefore concluded to be safe and effective in children, replacing rigid oesophagoscopy and some traditional surgical approaches.
Child, Preschool↗
Pathogenesis and assessment of malnutrition in liver disease.
Explore the source record for details and available documents.
Body Composition↗
Cytomegalovirus infection after liver transplantation in children.
Post-liver transplant cytomegalovirus (CMV) infection (seroconversion or virus isolation) and CMV disease (infection plus clinical signs and symptoms) were studied in relation to pretransplant recipient and donor serology, age, nutritional status and the effect of paediatric versus adult (reduced size) grafts. Of 70 children receiving 79 transplants, 26 (37%) had evidence of CMV infection, and eight (11.5%) had evidence of CMV disease, four of whom died. The primary infection rate (where the recipients were CMV negative) was 71% with mortality of 7% with most receiving a CMV-positive graft. The active secondary infection rate (reactivation or reinfection, where the recipients were CMV positive) was 60% with mortality of 12.5%. No significant differences in infection or disease rates were found comparing malnourished versus well-nourished patients, or between those who received whole or reduced-size grafts. The high prevalence of CMV infections supports the view that clinical signs alone are inadequate to direct investigations for CMV. Both primary and active secondary CMV infection can result in serious morbidity and mortality in children receiving liver transplants. These data do not support the strategy of providing immunoprophylaxis to seronegative recipients only, at least in paediatric liver transplantation.
Adolescent↗
Energy expenditure measurements in ventilated critically ill children: within- and between-day variability.
BACKGROUND: Energy expenditure measurement (EEM) by indirect calorimetry is used as a research and clinical tool in pediatric intensive care units. The aims of the study were to determine if a 30-minute EEM is representative of a 24-hour EEM (within-day variation); to determine if there is any diurnal variation during the 24-hour period of EEM; and to determine if there is a clinically significant between day variation of EEMs. METHODS: To determine within-day variation, energy expenditure was measured for a period of 24 hours for each subject (n = 11). The 24-hour period was then divided into 30-minute periods. The 30-minute means were compared with the 24-hour means. To determine between-day variation, EEMs were made daily for 30 minutes. RESULTS: In the within-day study, the overall mean percent coefficient of variation of the 30-minute measurements was 7.2% +/- 4.5%. There was no significant difference between the 30-minute means and the 24-hour means for each patient (p < .691). In 8 subjects the 30-minute means did not differ from the 24-hour mean by more than 20%. No diurnal variation was observed. The mean percent variation of between day EEM was 21% +/- 16%; the range was 1% to 69%. CONCLUSION: In critically ill ventilated children, clinically relevant within-day variations in EEM are uncommon and a single 30-minute EEM gives an acceptable guide to the level of nutrition support required. Between-day variations can, however, be large and daily EEMs are required.
Adolescent↗
Oral changes associated with end-stage liver disease and liver transplantation: implications for dental management.
In children, chronic liver disease has a variety of causes; its effects include malnutrition, bleeding tendencies, osteopenia, and rachitic changes in the skeleton. Chronic liver disease in children may have many oral manifestations, such as green staining of the teeth and gingiva, as well as enamel hypoplasia. Dental management may be complicated by many factors, like bleeding tendencies and inability to metabolize routine anesthetics. Patients who undergo increasingly successful liver transplantation require life-long immunosuppressive therapy, which has side-effects. Results of this study of nine patients, seven females and two males, show that the effects of end-stage liver disease are permanently recorded on the teeth. Six were examined both before and after liver transplantation; the other three, only afterwards. This study confirms and extends the findings of three previous reports describing the oral manifestations of congenital biliary atresia in childhood.
Biliary Atresia↗
Septic systems and Giardia intestinalis.
Explore the source record for details and available documents.
Australia↗
Leucine metabolism and body cell mass in cystic fibrosis.
To further investigate the nature of malnutrition in cystic fibrosis (CF), specifically to evaluate the influence of body composition on leucine metabolism and determine whether measures used as reference standards alter comparative differences in protein metabolism, we measured [13C]leucine kinetics in nine well-nourished and eight malnourished, height-, FEV1-, and sex-matched CF subjects. Body cell mass was measured by total-body potassium (K40) analysis. Leucine turnover did not significantly differ between groups in absolute values or when corrected for body weight, body height, or surface area. However, malnourished CF patients had accelerated leucine turnover based on body cell mass measurements. Mean leucine synthesis per unit body cell mass in the malnourished group was 147% higher than that of the well-nourished group (p < 0.001). These data indicate that altered body composition is an important factor influencing protein metabolism in CF and should be considered in future metabolic studies. The importance of potentially serious but possibly correctable deficits in body cell mass in CF is emphasized.
Adolescent↗
Clinical applicability of bioelectric impedance to measure body composition in health and disease.
The applicability of bioelectric impedance analysis (BIA) as a measure of body composition in children and young adults in both health and disease was studied in 155 subjects (5 mo to 54 yr of age) who were healthy (n = 21), had cystic fibrosis (n = 16), or had end-stage liver disease with variable clinical ascites or edema (n = 62). BIA and phase angle measured at a frequency of 50 kHz between the wrist and contralateral ankle by use of a tetrapolar measuring technique was compared with fat-free mass (FFM) estimated from skin-fold (SK) thickness measurements (n = 57), and body cell mass (grams potassium) measured by total-body potassium (TBK) counting. In a subgroup of subjects with end-stage liver disease, BIA was compared with total-body water measured by deuterium dilution (n = 21). High levels of correlation were found in healthy subjects, cystic fibrosis patients, and patients with end-stage liver disease when impedance (height2/resistance) was used to predict TBK (r = 0.96, 0.96, 0.98, respectively), and SK was used to predict FFMs (r = 0.96, 0.99, 0.90, respectively), by linear regression analysis. However, less satisfactory relationships were found when the methods were more appropriately analyzed with an estimated limits of agreement procedure (1 SD = 8.5, 5.0, 27.7% [TBK] and 9.4, 6.7, 23.0% [FFMs], respectively). A poor level of technique agreement (1 SD = 14%) was found when this method was compared with total-body water measured by isotope dilution in patients with liver disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Nutritional growth retardation is associated with defective lung growth in cystic fibrosis: a preventable determinant of progressive pulmonary dysfunction.
Evidence for a relationship between nutritional growth retardation in cystic fibrosis (CF) and progressive pulmonary dysfunction was evaluated by a prospective longitudinal study of changes in nutritional growth parameters, in relation to changes in pulmonary function data, in 61 moderately affected CF patients, aged 5-17 yr, during the equilibrated phase of lung growth. Age, sex, initial and serial weight and height Z scores, body cell mass (BCM) by total-body potassium (TBK) analysis, and changes in forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC), excluding data during pulmonary exacerbations, were analyzed by multiple regression analyses. The only significant predictor of change in FVC (best-fit model) was change in BCM, expressed as TBK (g/yr), TBK for age (percentage predicted), and TBK for height (percentage predicted) (p < 0.01). Standard anthropometric variables were not predictive. No reliable predictive model emerged for changes in FEV1. Relative decline in TBK for age was strongly predictive of decline in FVC (percentage predicted) accounting for 23% of this change. Patients with normal growth of the BCM had significantly less decline in FVC than those with retarded growth of the BCM (a fall of 2.5 vs. 6.8%/yr, p < 0.01). Impaired growth of the metabolically active BCM appears to be associated with progressive lung dysfunction in CF, possibly mediated by impaired lung growth. Achieving optimal nutrition and growth may minimize the progressive decline in pulmonary function commonly seen in this disease.
Adolescent↗