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Biomedical subjects

K J Gaskin

Publications and source records attributed to K J Gaskin.

At least 37 records · Page 2Linked to original sources
PubMed1995

Resting energy expenditure in children with phenylketonuria.

Reports have suggested that children with phenylketonuria (PKU) weigh more compared with reference data. We found lower body protein and bone mineral density in children with PKU. These children may have a predisposition becoming overweight because of an alteration in body composition, which may lower resting energy expenditure (REE). REE was measured in 30 (15 males, 15 females) children with PKU (aged 9.6 +/- 2.9 y) and in 65 (23 males, 42 females) control children (aged 11.2 +/- 3.1 y). There was a comparable range in body fat within each group (control group: 11-34%; PKU group: 10-34%). The mean REE was similar between the male and female children with PKU (5300 +/- 757 and 4703 +/- 1024 kJ/24 h, respectively) and the control subjects (5306 +/- 969 and 5164 +/- 701 kJ/24 h, respectively). The children with PKU had an REE similar to that predicted from control data (males 102.1 +/- 7.8% of predicted and females 100.2 +/- 8.5% of predicted). This study found no evidence of a reduced REE or of increased weight in children with PKU.

Adolescent↗
PubMed1995

Indirect calorimetry in mechanically ventilated children: a new technique that overcomes the problem of endotracheal tube leak.

OBJECTIVES: To develop indirect calorimetry to enable measurement of energy expenditure in mechanically ventilated children and to assess the effect of endotracheal tube leak on the accuracy of indirect calorimetry measurements. DESIGN: Prospective, observational study, using a convenience sample. SETTING: Tertiary pediatric intensive care unit in a university-associated children's hospital. PATIENTS: Eighteen patients, 3 months to 10 yrs of age, with various diagnoses, and requiring mechanical ventilation. INTERVENTIONS: Patients were intubated and received routine intensive care treatment. MEASUREMENTS AND MAIN RESULTS: Energy expenditure and respiratory quotient were measured using a new modification to the technique of indirect calorimetry, which includes an assessment of any expired gas lost around the endotracheal tube. Mean energy expenditure was 97% of predicted energy expenditure, but there was great variability between patients, and energy expenditure could not be estimated reliably from predictive equations. The amount of expired gas lost because of gas leak around the endotracheal tube was often a clinically important proportion of total expired gas, and this lost gas could not be predicted by audible endotracheal tube leak. CONCLUSIONS: Measurement of energy expenditure by indirect calorimetry may be useful in the nutritional management of critically ill children. Results may be inaccurate if the gas lost because of leak around uncuffed endotracheal tubes is not taken into account.

Calorimetry, Indirect↗
PubMed1995

Cholestatic jaundice and congenital hypopituitarism.

OBJECTIVE: The prevalence of cholestatic jaundice as a presenting feature of congenital hypopituitarism is assessed. METHODOLOGY: A retrospective case record analysis of the presenting features in all patients diagnosed as having congenital hypopituitarism between 1973-93. RESULTS: Seven of the 20 patients with congenital hypopituitarism presented with cholestatic jaundice as the major initial manifestation of the disorder. Liver biopsy findings in three revealed intracellular bile pigment accumulation and variable giant cell formation. CONCLUSION: Cholestatic jaundice was the major manifestation of congenital hypopituitarism in 35% of patients presenting in the neonatal or early infancy period.

Age of Onset↗
PubMed1995

Gastro-oesophageal reflux and adverse respiratory events in children under anaesthesia.

The incidence of gastro-oesophageal reflux in children undergoing general anaesthesia has not previously been studied. One-hundred-and-twenty children (ASA Class 1-2) were studied intraoperatively using continuous oesophageal pH monitoring. The incidence of reflux was 2.5% (3 of 120). None of these three patients had an adverse respiratory event. There was no correlation between reflux and adverse respiratory events. Thirteen patients had minor respiratory events without evidence of acid reflux. Gastro-oesophageal reflux does occur in healthy paediatric patients having minor surgery, but was not a significant cause of the adverse respiratory events that occurred in our study.

Anesthesia↗
PubMed1995

Hepatobiliary disease in cystic fibrosis patients with pancreatic sufficiency.

Focal and multilobular biliary cirrhosis are considered pathognomonic of cystic fibrosis (CF) and almost invariably have been reported in patients with steatorrhea. In contrast, patients with pancreatic sufficiency and normal absorption are considered less likely to develop liver or biliary tract problems. The authors report three patients with CF and pancreatic sufficiency, presenting with recurrent abdominal pain (unrelated to pancreatitis). All had common bile duct disease, one with multilobular cirrhosis and portal hypertension. Pancreatic sufficiency was proven by quantitative pancreatic stimulation tests, 3-day fecal fat analyses, and serum pancreatic isoamylases. All three patients had mild lung disease. Two were homozygous for the common delta F508 mutation, and the other, a delta F508 compound heterozygote. Hepatobiliary structure and function were determined by serial hepatobiliary scintigraphy, percutaneous transhepatic cholecystography, and biochemical liver function tests. Patients 1 and 3 had mild hepatomegaly, normal liver biochemistry, and distal common bile duct strictures. Patient 2 had a firm nodular liver with splenomegaly, abnormal liver biochemistry, and a cholangiographic appearance of sclerosing cholangitis. All have undergone operative treatment for persistent abdominal pain. These cases confirm the occurrence of common bile duct pathology and liver disease in patients with CF and pancreatic sufficiency. They demonstrate that liver and biliary tract disease can occur independently of the underlying disease severity and the presence of steatorrhea. Further, they suggest that obstruction of the biliary tract may be an additional factor in the evolution of liver disease in CF.

Biliary Tract Diseases↗
PubMed1994

Decreased bone mineral density in children with phenylketonuria.

Previous studies have suggested that children with phenylketonuria (PKU) have a reduction in bone mineralization compared with control subjects. To investigate this, bone mineral density (BMD) of the total body (TBMD) was measured in 32 prepubertal children with PKU and in 95 age-matched control subjects. Spine bone mineral density (SBMD) was also recorded in a subset, 24 with PKU and 55 control subjects. The effect of dietary intake on bone mass was assessed in 30 of the children with PKU and in 12 control subjects. In the children with PKU, TBMD and SBMD were significantly lower than in the control subjects after adjustment for height and weight (P = 0.03 and P = 0.003, respectively). The children with PKU had a higher intake of calcium (P < 0.0001), phosphorus (P = -0.0002), and magnesium (P < 0.0001), suggesting that their lower BMD occurred despite an adequate diet based on current recommendations. Further study is needed to establish the cause of this deficit in bone mass and the benefit of additional nutritional support to reverse this problem.

Absorptiometry, Photon↗
PubMed1994

Sclerosing cholangitis in children with inflammatory bowel disease.

BACKGROUND: Primary sclerosing cholangitis (PSC) with inflammatory bowel disease (IBD) has been rarely reported in children. AIM: To describe the clinical presentation, sequential liver function test abnormalities, radiological bile duct anomalies and liver histology in four children with PSC and IBD. METHODS: Over a period of 18 years, four of 130 patients with IBD developed abnormal liver function tests. Three of the four patients had ulcerative colitis and the other Crohn's disease. All four patients had baseline and follow-up liver function tests, percutaneous transhepatic cholangiography and a needle biopsy of the liver. RESULTS: The four patients at presentation had minimal symptoms or signs of liver disease. All had elevation of serum transaminases, gamma glutamyl transferase and/or alkaline phosphatase. Three had the typical onion skin fibrosis of bile ducts. Percutaneous transhepatic cholangiography demonstrated irregularity and beading of the hepatic and common bile ducts in three patients. The other with normal cholangiography had fibrosing cholangitis on liver biopsy and was considered to have small duct disease. CONCLUSIONS: We conclude that yearly biochemical assessment of liver function should be performed on all children with IBD, and if abnormal should raise the suspicion of PSC. The latter diagnosis can be confirmed by liver biopsy and cholangiography.

Biopsy, Needle↗
PubMed1994

Total body nitrogen in children with chronic renal failure and short stature.

OBJECTIVE: To directly assess the body protein content of children with chronic renal failure (CRF) and short stature. SETTING: A tertiary referral paediatric hospital. SUBJECTS: There were: (i) 17 patients (10 male, nine pre-pubertal; mean age 12.90 +/- 3.20 years) with CRF and height standard deviation (SD) score < -2.00, and (ii) 43 normal children (18 male, 27 pre-pubertal; mean age 10.34 +/- 3.34 years). INTERVENTIONS: CRF patients had the following measurements: anthropometry, total body nitrogen (TBN) by neutron capture analysis, 4 day weighed food record and serum albumin levels. Control subjects had TBN and anthropometric measurements only. RESULTS: Although older than the controls, the CRF patients had significantly lower TBN values (645 +/- 265 vs 930 +/- 365 g, P < 0.01). Mean values for TBN and TBN/height (percentages of expected) in the CRF patients were significantly reduced to 54% and 63% respectively, when predicted from age. However, their TBN predicted from height was 100% of expected. %TBN (predicted from age) correlated significantly with height SD score (r = 0.79), weight SD score (r = 0.87), upper arm muscle area percentile (r = 0.62) and serum albumin (r = 0.62). Mean oral energy and protein intakes were 65% and 172% of recommended dietary intake respectively. CONCLUSIONS: Children with CRF and short stature are significantly protein-depleted for age although not for height. Chronic energy deficiency may contribute to impaired protein deposition which, in turn, may be important in the pathogenesis of growth failure in CRF.

Adolescent↗
PubMed1993

Development of a paediatric indirect calorimeter.

An indirect calorimeter was developed to measure resting energy expenditure (REE) and respiratory quotient (RQ) in a clinical paediatric population. The design is suitable for any child spontaneously breathing room air. Measurements are possible from small babies to large hypermetabolic adolescents due to the ability to set and control flow from the patient headbox across the range 2-40 l/min. The calorimeter is mobile, it is electrically safe and the patient interface may be disinfected. An alcohol burning technique, that can emulate a clinical test for children of various sizes, is used to check the system, which has a high level of accuracy across a range of conditions. The indirect calorimeter has been successfully used to measure a large number of children of different sizes.

Adolescent↗
PubMed1992

Partial sacral agenesis with constipation: a report of one family.

Seven first degree relatives in three generations of a family with partial sacral agenesis are reported. Anterior sacral meningoceles were found in five cases, four of whom had severe constipation, one with urinary voiding dysfunction, and another without constipation who had low pressure headaches and dyspareunia. The anomalad was transmitted in an autosomal dominant fashion with incomplete penetrance and variable expression. This entity should be considered in all children and adults with severe constipation from birth, a family history of constipation and/or where constipation is refractory to medical therapy.

Abnormalities, Multiple↗
PubMed1991

A total body nitrogen facility for paediatric use.

The design, calibration and evaluation of a facility for in vivo prompt gamma neutron capture analysis of total body nitrogen in children is described. The patient is scanned in both supine and prone positions, across a vertically collimated beam from a 1 GBq 252Cf fission source. Two NaI(T1) detectors are placed on either side of the patient, perpendicular to both the neutron beam and the scanning direction. The effective dose equivalent delivered to a child during an 840 s scan is approximately 0.14 mSv (QF = 10). Correction factors for nitrogen background (width-dependent), hydrogen background (1-4% of hydrogen gamma ray peak) and the differential attenuation of nitrogen and hydrogen gamma rays (width-dependent) can be applied to the measured nitrogen-to-hydrogen gamma ray counts ratio. By using the mass of hydrogen (based on body mass and fat mass) as an internal standard, the nitrogen mass can then be determined. Measurements with a urea-containing box phantom show that the current precision (CV) of the net nitrogen counts and of the nitrogen-to-hydrogen counts ratio is +/- 2.0% and +/- 1.5%, respectively. Using small anthropomorphic and other phantoms, estimation of the mass of nitrogen has a precision of +/- 1.4 to 5.4% and an accuracy of 97.1 to 101.5%.

Australia↗
PubMed1991

Nitrogen deposition in malnourished children with cystic fibrosis.

To determine the protein nutritional status of 21 malnourished children with cystic fibrosis (CF), total body nitrogen (TBN) was measured and the results were compared with 21 control subjects. CF patients demonstrated a lower TBN (P less than 0.001). When matched for height (n = 10) or bone age (n = 13), the CF patients still had a depressed TBN/height or TBN/lean body mass (P less than 0.05). To assess nitrogen deposition during nutritional rehabilitation, repeat TBN measurements were performed on the 21 CF patients. Nitrogen deposition ranged from -230 to 550 g/y and correlated with weight velocity (r = 0.78, P less than 0.001). Increased nitrogen deposition (greater than 150 g/y) was generally associated with normal height gain (height velocity SD score greater than -2.00) and weight gain (greater than 2.0 kg/y). Decreased nitrogen deposition was associated with poor weight gain but did not preclude normal linear growth. These data suggest an important role for TBN estimations in defining protein nutritional status in children and indicate that skeletal growth can continue in the presence of minimal nitrogen deposition.

Adolescent↗
PubMed1991

Infantile hepatic haemangioma: investigation and treatment.

Infantile hepatic haemangiomas are benign tumours which tend to regress in late infancy. We describe five infants with hepatic haemangioma, three of whom developed congestive cardiac failure. Technetium-99m labelled red blood cell scan was useful in the diagnosis and follow-up of the lesion. The three symptomatic infants were treated with prednisone (2 mg/kg per day) for 2 months in addition to digoxin and diuretics. Significant clinical improvement occurred within 3 weeks in each case. We conclude that the red blood cells scan is a good non-invasive diagnostic test and recommend corticosteroids as initial management for symptomatic infantile hepatic haemangiomas.

Diagnostic Imaging↗
PubMed1991

Abnormal epithelial transport in cystic fibrosis jejunum.

Abnormal epithelial electrolyte transport has been identified in a range of cystic fibrosis (CF) organs and appears to account for the various clinical manifestations of the disease. The aim of this study was to further define the Cl- secretion defect in CF jejunum. Excised jejunum was obtained from 11 CF patients and 12 controls. Transport studies were performed on stripped epithelium in vitro under short-circuited conditions in Ussing Chambers. 3-Isobutyl-1-methylxanthine (IBMX) (300 microM) significantly increased Cl- secretion in control (-2.3 +/- 0.6 to -3.3 +/- 0.7 mueq.cm-2.h-1; P less than 0.01, paired t test; n = 5 subjects) but not in CF jejunum (-0.5 +/- 0.3 to -0.1 +/- 0.4; n = 4). However in contrast to control jejunum, net Na+ absorption in CF jejunum was higher in the IBMX (1.3 +/- 0.5 mueq.cm-2.h-1) compared with basal periods (0.6 +/- 0.3; P less than 0.05, paired t test). IBMX stimulation of tissue adenosine 3',5'-cyclic monophosphate (cAMP) was similar in both control and CF jejunum. A range of secretagogues known to induce secretion in mammalian intestine, including dibutyryl cAMP (DBcAMP), DBcGMP, Ca2+ ionophore A23187, and the protein kinase C activator 4 beta-phorbol 12,13-dibutyrate, failed to induce secretion in CF jejunum. In conclusion, CF jejunum failed to exhibit Cl- secretion and also demonstrated abnormalities of Na+ absorption. These results support the view that the defect lies at a site distal to the intracellular messengers. Moreover, these abnormalities of intestinal electrolyte transport may account for some of the gastrointestinal manifestations of the disease such as meconium ileus and distal intestinal obstruction syndrome.

1-Methyl-3-isobutylxanthine↗