PubMed Health⌕ Search

Biomedical subjects

R W Shepherd

Publications and source records attributed to R W Shepherd.

At least 19 recordsLinked to original sources
PubMed2001

Energy expenditure and the body cell mass in cystic fibrosis.

Poor nutritional status in patients with cystic fibrosis (CF) is associated with severe lung disease, and possible causative factors include inadequate intake, malabsorption, and increased energy requirements. Body cell mass (which can be quantified by measurement of total body potassium) provides an ideal standard for measurements of energy expenditure. The aim of this study was to compare resting energy expenditure (REE) in patients with CF with both predicted values and age-matched healthy children and to determine whether REE was related to either nutritional status or pulmonary function. REE was measured by indirect calorimetry and body cell mass by scanning with total body potassium in 30 patients with CF (12 male, mean age = 13.07 +/- 0.55 y) and 18 healthy children (six male, mean age = 12.56 +/- 1.25 y). Nutritional status was expressed as a percentage of predicted total body potassium. Lung function was measured in the CF group by spirometry and expressed as the percentage of predicted forced expiratory volume in 1 s. Mean REE was significantly increased in the patients with CF compared with healthy children (119.3 +/- 3.1% predicted versus 103.6 +/- 5% predicted, P < 0.001) and, using multiple regression techniques, REE for total body potassium was significantly increased in patients with CF (P = 0.0001). There was no relation between REE and nutritional status or pulmonary disease status in the CF group. In conclusion, REE is increased in children and adolescents with CF but is not directly related to nutritional status or pulmonary disease.

Adolescent↗
PubMed2001

Estimation of body water compartments in cirrhosis by multiple-frequency bioelectrical-impedance analysis.

Estimation of total body water by measuring bioelectrical impedance at a fixed frequency of 50 kHz is useful in assessing body composition in healthy populations. However, in cirrhosis, the distribution of total body water between the extracellular and intracellular compartments is of greater clinical importance. We report an evaluation of a new multiple-frequency bioelectrical-impedance analysis technique (MFBIA) that may quantify the distribution of total body water in cirrhosis. In 21 cirrhotic patients and 21 healthy control subjects, impedance to the flow of current was measured at frequencies ranging from 4 to 1012 kHz. These measurements were used to estimate body water compartments and then compared with total body water and extracellular water determined by isotope methodology. In cirrhotic patients, extracellular water and total body water (as determined by isotope methods) were well predicted by MFBIA (r = 0.73 and 0.89, respectively). However, the 95% confidence intervals of the limits of agreement between MFBIA and the isotope methods were +/-14% and +/-9% for cirrhotics (extracellular water and total body water, respectively) and +/-9% and +/-9% for cirrhotics without ascites. The 95% confidence intervals estimated from the control group were +/-10% and +/-5% for extracellular water and total body water, respectively. Thus, despite strong correlations between MFBIA and isotope measurements, the relatively large limits of agreement with accepted techniques suggest that the MFBIA technique requires further refinement before it can be routinely used to determine the nutritional assessment of individual cirrhotic patients.

Ascites↗
PubMed2000

Fulminant hepatitis A in indigenous children in north Queensland.

Since 1993, three Indigenous children in north Queensland have died of fulminant hepatitis A. Even if the children had been able to undergo liver transplantation, prolonged immunosuppressant therapy and the likelihood of opportunistic infections would inevitably have jeopardised any chance of long-term survival. As hepatitis A has become a leading infectious cause of death in young Indigenous children in north Queensland, hepatitis A vaccine has recently been introduced into the vaccination schedule for these children.

Child, Preschool↗
PubMed2000

Nutritional status of children with cystic fibrosis measured by total body potassium as a marker of body cell mass: lack of sensitivity of anthropometric measures.

OBJECTIVE: To investigate measures aimed at defining the nutritional status of cystic fibrosis (CF) populations, this study compared standard anthropometric measurements and total body potassium (TBK) as indicators of malnutrition. METHODS: Height, weight, and TBK measurements of 226 children with CF from Royal Children's Hospital, Brisbane, Australia, were analyzed. Z scores for height for age, weight for age, and weight for height were analyzed by means of the National Centre for Health Statistics reference. TBK was measured by means of whole body counting and compared with predicted TBK for age. Two criteria were evaluated with respect to malnutrition: (1) a z score < -2.0 and (2) a TBK for age <80% of predicted. RESULTS: Males and females with CF had lower mean height-for-age and weight-for-age z scores than the National Centre for Health Statistics reference (P <.01), but mean weight-for-height z score was not significantly different. There were no significant gender differences. According to anthropometry, only 7.5% of this population were underweight and 7.6% were stunted. However, with TBK as an indicator of nutritional status, 29.9% of males and 22.0% of females were malnourished. CONCLUSION: There are large differences in the percentage of patients with CF identified as malnourished depending on whether anthropometry or body composition data are used as the nutritional indicator. At an individual level, weight-based indicators are not sensitive indicators of suboptimal nutritional status in CF, significantly underestimating the extent of malnutrition. Current recommendations in which anthropometry is used as the indicator of malnutrition in CF should be revised.

Body Composition↗
PubMed2000

Energy expenditure in 100 ventilated, critically ill children: improving the accuracy of predictive equations.

OBJECTIVE: The purpose of this study was to evaluate current methods of predicting energy requirements and to develop and validate new equations derived from energy expenditure measurements of ventilated, critically ill children. DESIGN: Prospective, observational, sequential study. SETTING: Pediatric intensive care unit. PATIENTS: A total of 100 ventilated, critically ill children who fit the criteria of energy expenditure measurement. Additional patients (n = 25) were included in the validation study. INTERVENTION: An indirect calorimeter was used to measure energy expenditure for a period of 30 mins. MEASUREMENTS AND MAIN RESULTS: The mean measured energy expenditure was 185+/-51 kJ/kg per day. Predicted energy expenditure from standard equations was compared with measured energy expenditure by using the Bland and Altman "methods comparison procedure," and poor precision and accuracy were observed. Patient variables were collected at the time of measurement, and multiple regression analysis was performed to determine the independent contribution of each variable to measure energy expenditure. New predictive equations were formulated and validated with additional energy expenditure measurements. Patient variables that did not correlate significantly with energy expenditure were gender, Pediatric Risk of Mortality score, and commencement of nutritional support. An equation was derived from patient variables (age, weight, weight for age Z score, body temperature, number of days after intensive care admission, and primary reason for admission) that correlated significantly (r2 = .898) with measured energy expenditure. A second, simplified equation (energy expenditure kJ/day = ¿17 x age [months]¿ + ¿48 x weight [kg]¿ + ¿292 x body temperature degrees C¿ - 9,677) was produced (r2 = .867). Validation found no significant difference between measured and predicted energy expenditure by the new equations; however, the equations did not predict accurately for patients <2 months of age. CONCLUSION: The new equations provide a more accurate alternative to current predictive methods in assessing energy requirements of ventilated, critically ill children.

Calorimetry, Indirect↗
PubMed2000

Value of total body potassium in assessing the nutritional status of children with end-stage liver disease.

Malnutrition is a common problem in children with end-stage liver disease (ESLD), and accurate assessment of nutritional status is essential in managing these children. In a retrospective study, we compared nutritional assessment by anthropometry with that by body composition. We analyzed all consecutive measurements of total body potassium (TBK, n = 186) of children less than 3 years old with ESLD awaiting transplantation found in our database. The TBK values obtained by whole body counting of 40K were compared with reference TBK values of healthy children. The prevalence of malnutrition, as assessed by weight (weight Z score < -2) was 28%, which was significantly lower (chi-square test, p < 0.0001) than the prevalence of malnutrition (76%) assessed by TBK (< 90% of expected TBK for age). These results demonstrated that body weight underestimated the nutritional deficit and stressed the importance of measuring body composition as part of assessing nutritional status of children with ESLD.

Anthropometry↗
PubMed1999

Growth failure in cystic fibrosis.

OBJECTIVE: The aim of this study was to describe and compare the nutritional status of children aged 0-18 years attending the cystic fibrosis (CF) clinic at the Royal Children's Hospital, Brisbane, Australia, as outpatients in 1986 and 1996. METHODOLOGY: The heights, weights and pulmonary function of children attending the CF clinic as outpatients in 1986 (n = 97) and 1996 (n = 227) were retrospectively analysed using a computerized database maintained by the CF clinic. The heights and weights were analysed in terms of z scores for height for age (HAZ), weight for age (WAZ) and weight for height (WHZ). Pulmonary function data is not available for all children. Forced expiratory volume in one second (FEV1), forced vital capacity (FVC) and forced mid expiratory flows (FEF) were expressed as a percentage of predicted and are presented here. RESULTS: The 1986 sample consisted of 41 males (age range 0.18-14.59 years, mean age 6.52 (4.33)) and 56 females (age range 0.15-14.97 years, mean age 7.75 (3.70)). The 1996 sample consisted of 111 males (age range 0.09-17.97 years, mean age 8.80 (5.49)) and 114 females (age range 0.12-17.98 years, mean age 8.49 (5.26)). In 1986, males were shorter than females (P = 0.0096) and females had a lower mean FVC than males (P = 0.0438). In 1996, males were shorter, lighter and more wasted than females (P = 0.0357, P = 0.0034 and P = 0.0273, respectively) and females had a lower mean FEV1 and mean FVC than males (P = 0.0176 and P = 0.0079, respectively). Males in 1996 were lighter and more wasted than males in 1986 (P = 0.0023 and P = 0.0139, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P = 0.0012 and P = 0.0069, respectively). Females in 1996 were shorter and lighter than females in 1986 (P = 0.0273 and P = 0.0405, respectively) and had a lower mean FEV1, mean FVC and mean FEF (P < 0.0001, P < 0.0001 and P < 0.0001, respectively). When subjects were classified according to FEV1 (FEV1 > or = 75% or FEV1 < 75%), there were no significant differences in z scores between the 1986 group and 1996 group. Similarly, when the 1986 group were matched for gender and FEV1 with the 1996 group, there were no significant differences in z scores for males or females. CONCLUSIONS: It is suggested that the apparent worsening of nutritional status among the 1996 group of CF patients is in fact due to an effect of increased survival of patients with more severe clinical symptoms. The findings from this study highlight the continuing, and in fact, worsening problem of growth failure in children with CF.

Adolescent↗
PubMed1999

Feeding problems in infants with gastro-oesophageal reflux disease: a controlled study.

OBJECTIVE: Gastro-oesophageal reflux disease (GORD) in infants is commonly associated with feeding problems but has not been subject to systematic controlled study. We evaluated feeding, dietary, behavioural data obtained from systematic objective studies of six-month old infants with and without GORD. METHODS: Infants with GORD (defined by 24-h pH monitoring, n = 20), and age, gender, gestation, and socio-economic matched healthy infants (n = 20) had standardised assessments of dietary intake, oromotor function by videoanalysis (Feeding Assessment Schedule, FAS), and infant feeding behaviour by Testers and Maternal Ratings (TRIB and MRIB). Videofluoroscopic analyses of swallowing was undertaken in 11/20 GORD infants and analysed by standardised paediatric check list. RESULTS: Compared with control data: GORD infants had significantly lower energy intakes; the FAS showed GORD infants to have significantly fewer adaptive skills and readiness behaviour for solids, significantly more food refusal and food loss; the TRIB showed GORD infants to be significantly more demanding and difficult with feeds; and the MRIB revealed that mothers of GORD infants had significantly more negative feelings, significantly less enjoyment of feeds, and reported significantly more crying behaviour. On videofluoroscopy, oral-preparatory and oral phase problems predominated, particularly with solids, silent aspiration occurred during the pharyngeal phase in 2/11, and delayed oesophageal transit occurred in 4/11. CONCLUSIONS: Feeding problems affecting behaviour, swallowing, food intake, and mother-child interaction occur in infants with GORD, who displayed a lack of development of age-appropriate feeding skills. The contribution of feeding problems to morbidity in GORD in infants has been underestimated in the past.

Bottle Feeding↗
PubMed1999

Liver transplantation in Japanese and Australian/New Zealand children with biliary atresia: a 10-year comparative study.

OBJECTIVE: To compare Japanese with Australian/New Zealand (ANZ) children with biliary atresia who were treated by liver transplantation, and evaluate the indications for and timing of transplantation. DESIGN: Retrospective study. SETTING: Queensland Liver Transplant Service (QLTS), Australia. SUBJECTS: 43 Japanese and 30 ANZ children with biliary atresia who required transplantation between 1985 and 1992. INTERVENTIONS: The 43 Japanese children had 52 transplants, and the 30 ANZ children had 33. MAIN OUTCOME MEASURES: Morbidity, mortality, and long term survival. RESULTS: The Japanese children had significantly lower serum albumin concentrations than the ANZ children preoperatively (mean (SD) 32 (7) g/L compared with 37 (5), p<0.05). The actuarial survival at 7 years of the ANZ children was significantly higher than that of the Japanese children (79% compared with 49%, p<0.05). There were 24 deaths (17 Japanese, 40%, and 7 ANZ, 23%); 2 of the ANZ and 7 of the Japanese children died more than a year after transplantation. All 26 children who were well-nourished at the time of transplantation defined as a Z-score (weight or height minus mean weight or height for age, sex, and race, divided by the SD) of -1 or more were alive at 1 month compared with 11 of the 47 poorly-nourished children (Z-score <-1). Survival among the Japanese declined after 1 year, and there was no association with Z-scores. Overall, Z-scores for weight improved significantly after transplantation, whereas those for height improved a little, but not significantly so. Japanese children were significantly shorter than ANZ children, and their Z-scores for height did not improve after transplantation. CONCLUSION: liver transplantation should be done as soon as possible for children with biliary atresia to maximise survival and growth.

Adolescent↗
PubMed1998

Lack of sensitivity of weight targets compared with body cell mass for determining recovery from malnutrition in adolescents with anorexia nervosa.

OBJECTIVE: Using body weight targets to assess recovery from malnutrition in adolescents with anorexia nervosa (AN) may lead to premature discontinuation of refeeding programs. We examined body cell mass (BCM) to determine nutritional recovery, comparing with conventional indices based on weight. METHOD: Body mass index (BMI), Z-score, percentage of ideal body weight (IBW), and BCM (by total body potassium) were studied in adolescent females with AN (n = 92). RESULTS: Fourteen percent had "normal" weights (> 85% of IBW), 24% had "acceptable" BMI values (> 16), and 15% had "acceptable" Z-score values (> -1) when their BCM was not fully restored. DISCUSSION: The use of percentage of IBW, Z-scores, or BMI lacks sensitivity in determining adequacy of nutritional rehabilitation. Body compartmental analysis particularly of BCM and body fat is recommended, and the use of BMI particularly should be discarded.

Adolescent↗
PubMed1998

Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia.

Extrahepatic biliary atresia is a severe neonatal liver disease resulting from a sclerosing cholangiopathy of unknown etiology. Although biliary obstruction may be surgically corrected by a "Kasai" hepatoportoenterostomy, most patients still develop progressive hepatic fibrosis, although the source of increased collagen deposition is unclear. This study examined the role of hepatic stellate cells (HSCs) and assessed the source of transforming growth factor-beta (TGF-beta) production in hepatic fibrogenesis in patients with biliary atresia. Liver biopsies from 18 biliary atresia patients (including 5 pre- and post-Kasai) were subjected to immunohistochemistry for alpha-smooth muscle actin and in situ hybridization for either procollagen alpha1 (I) mRNA or TGF-beta1 mRNA. Sections were also subjected to immunohistochemistry for active TGF-beta1 protein. The role of Kupffer cells in TGF-beta1 production was assessed by immunohistochemistry for CD68. Procollagen alpha1 (I) mRNA was colocalized to alpha-smooth muscle actin-positive HSCs within the region of increased collagen protein deposition in fibrotic septa and surrounding hyperplastic bile ducts. The number of activated HSCs was decreased in only one post-Kasai biopsy. TGF-beta1 mRNA expression was demonstrated in bile duct epithelial cells and activated HSCs and in hepatocytes in close proximity to fibrotic septa. Active TGF-beta1 protein was demonstrated in bile duct epithelial cells and activated HSCs. This study provides evidence that activated HSCs are responsible for increased collagen production in patients with biliary atresia and therefore play a definitive role in the fibrogenic process. We have also shown that bile duct epithelial cells, HSCs, and hepatocytes are all involved in the production of the profibrogenic cytokine, TGF-beta1.

Actins↗
PubMed1998

Malnutrition, gastroenteritis and trypsinogen concentration in hospitalised Aboriginal children.

OBJECTIVE: To explore relationships between malnutrition and pancreatic damage in hospitalised aboriginal children. METHODS: Immunoreactive trypsinogen (IRT) concentrations were measured in two populations of hospitalised aboriginal children in Australia: 472 children aged 0-3 years, in Alice Springs (Northern Territory); and 187 children aged 0-16 years in Mount Isa (Queensland). Correlation of whole blood IRT with height and weight z-scores, four-site skinfold thickness and upper arm circumference was sought. RESULTS: In Mount Isa, the geometric mean IRT concentration rose with decreasing weight z-score. The IRT concentration was otherwise unrelated to nutritional indices. Sixty percent of the 39 Mount Isa patients with gastroenteritis and 24.5% of the 358 Alice Springs patients with gastroenteritis had an IRT concentration in the upper quartile for their population, compared with 16% for patients with other diagnoses in both populations. CONCLUSIONS: A high IRT concentration in patients with low weight z-scores is a confounding effect of gastroenteritis, and may result from subclinical pancreatic disease in gastroenteritis.

Adolescent↗
PubMed1998

The sleep patterns of infants and young children with gastro-oesophageal reflux.

OBJECTIVE: Sleep disturbance in gastro-oesophageal reflux disease (GORD) in infants and young children has not been systematically studied nor has this manifestation been compared with population norms. METHODS: Sleep patterns of 102 infants and children aged 1 to 36 months with and without GORD, defined by pH monitoring, were analysed using the same questionnaire as in recent studies of normal sleep behaviour in this age range. Main outcome measures included time taken to settle at night, the number of night time wakenings requiring parental intervention, day time sleep patterns and parents problems with their childs' sleep behaviour. RESULTS: Compared with the population norms (n=3102), those with GORD (n=76) had greater prevalence of night time waking >3/night (50% vs 13% aged 3-12 months; 60% vs 10% aged 12-24 months, P<0.001), requirement of parental intervention (82% vs 55% aged 3-12 months, P<0.05; 92% vs 55% aged 12-24 months, P<0.001), significantly delayed onset of sleeping through the night, and greater prevalence of daytime sleep beyond 24 months. Similar but less striking differences were seen comparing those with (n=76) and without GORD (n=26). CONCLUSIONS: Sleep interruption occurs more frequently in infants and children with GORD than population norms. Objective evaluation of infants and children with sleep disturbance after the age of 3 months may avoid unnecessary over or under diagnosis of GORD. Systematic investigation of the contribution of GORD to sleep disturbance in infants and young children is warranted.

Child, Preschool↗
PubMed1998

Nutritional management of regurgitation in infants.

Infantile regurgitation is a frequently occurring problem. Throughout the world, anxious parents are imploring physicians to eliminate their infant's regurgitation. General practitioners, pediatricians and pediatric gastroenterologists strive to alleviate infantile regurgitation and its related parental stress. In this paper we define the scope of the problem and analyze the optimal, cost-efficient management approach to simple regurgitation in infants. The intent of this paper is to disseminate this information to practicing physicians and other health care professionals in an attempt to minimize the impact of this annoying problem of infancy and to eliminate confusion and expensive diagnostic tests and use of sub-optimal treatment modalities. Parental reassurance and dietary management by feeding thickened formula are important components in managing regurgitation in infants while maintaining optimal nutritional intake for adequate growth and development.

Gastroesophageal Reflux↗
PubMed1998

Growth hormone resistance and somatomedins in children with end-stage liver disease awaiting transplantation.

BACKGROUND: The success of orthotopic liver transplantation as treatment for end-stage liver disease has prompted investigation of strategies to maintain or improve nutrition and growth in children awaiting transplantation, because malnutrition is an adverse prognostic factor. The purpose of this study was to evaluate the effect of recombinant human growth hormone therapy on body composition and indices of liver function in patients awaiting transplant. METHODS: The study was designed as a placebo-controlled, double-blind, crossover trial. Patients received 0.2 U/kg growth hormone, subcutaneously, or placebo daily for 28 days during two treatment periods, separated by a 2-week washout period. Ten patients (mean age, 3.06 +/- 1.15 years; range, 0.51-11.65 years, five men), with extrahepatic biliary atresia (n = 8) or two with Alagille's syndrome (n = 2), with end-stage liver disease, completed the trial while awaiting orthotopic liver transplantation. Height, weight, total body potassium, total body fat, resting energy expenditure, respiratory quotient, hematologic and multiple biochemical profile, number of albumin infusions, insulin-like growth factor-1 and 1, growth hormone binding protein (GHBP), and insulin-like growth factor binding protein-1 (IGFBP-1) and insulin-like growth factor binding protein (IGFBP-3) were measured at the beginning and end of each treatment period. RESULTS: Growth hormone treatment was associated with a significant decline in serum bilirubin (-34.6 +/- 16.5 mumol/l vs. 18.2 +/- 11.59 mumol/l; p < 0.02) but there was no significant effect on any anthropometric or body composition measurements, or on any biochemical or hematologic parameters. CONCLUSIONS: These children with end-stage liver disease displayed growth hormone resistance, particularly in relation to the somatomedin axis. Exogenous growth hormone administration may be of limited value in these patients.

Bilirubin↗