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Biomedical subjects

N J Shaw

Publications and source records attributed to N J Shaw.

At least 19 recordsLinked to original sources
PubMed2007

Body fat estimation using bioelectrical impedance.

BACKGROUND/AIMS: Although childhood obesity is a major problem, routine assessment methods do not reflect fat mass. Body mass index, which is most commonly used, gives an indication of weight for height and not a degree of adiposity. METHODS: Bioelectrical impedance and dual-energy X-ray absorptiometry (DEXA) were used in a group of obese children to assess body fat. RESULTS: Comparison between DEXA and commercial bioelectrical impedance scales in 46 children showed a highly significant correlation (R = 0.944, p < 0.001) in fat mass. Fat mass measured using bioelectrical impedance was 2.4 kg lower compared to measurement using DEXA. CONCLUSION: These bioelectrical scales may prove useful in the management of childhood obesity as they are able to provide important clinical information regarding fat mass and adiposity.

Absorptiometry, Photon↗
PubMed2006

Preschool healthcare utilisation related to home oxygen status.

OBJECTIVE: To determine, in prematurely born children who had bronchopulmonary dysplasia (BPD), if respiratory morbidity, healthcare utilisation, and cost of care during the preschool years were influenced by use of supplementary oxygen at home after discharge from the neonatal intensive care unit. DESIGN: Observational study. SETTING: Four tertiary neonatal intensive care units. PATIENTS: 190 children, median gestational age 27 weeks (range 22-31), 70 of whom received supplementary oxygen when discharged home. INTERVENTIONS: Review of hospital and general practitioner records together with a parent completed respiratory questionnaire. MAIN OUTCOME MEASURES: Healthcare utilisation, cost of care, cough, wheeze, and use of an inhaler. RESULTS: Seventy children had supplementary oxygen at home (home oxygen group), but only one had a continuous requirement for home oxygen beyond 2 years of age. There were no significant differences in the gestational age or birth weight of the home oxygen group compared with the rest of the cohort. However, between 2 and 4 years of age inclusive, the home oxygen group had more outpatient attendances (p = 0.0021) and specialist attendances (p = 0.0023), and, for respiratory problems, required more prescriptions (p<0.0001). Their total cost of care was higher (p<0.0001). In addition, more of the home oxygen group wheezed more than once a week (p = 0.0486) and were more likely to use an inhaler (p<0.0001). CONCLUSIONS: Children with BPD who have supplementary oxygen at home after discharge have increased respiratory morbidity and healthcare utilisation in the preschool years.

Birth Weight↗
PubMed2006

Incidence of symptomatic vitamin D deficiency.

A survey of West Midlands paediatricians in 2001 identified 24 cases of symptomatic vitamin D deficiency in children less than 5 years of age. The overall incidence was 7.5 per 100,000 children per year with notable differences in incidence per ethnic group.

Africa↗
PubMed2006

Asian MODY: are we missing an important diagnosis?

AIMS: Maturity onset diabetes of the young (MODY) is a monogenic form of diabetes where correct diagnosis alters treatment, prognosis and genetic counselling. The first UK survey of childhood MODY identified 20 White, but no Asian children with MODY. We hypothesized that MODY causes diabetes in UK Asians, but is underdiagnosed. METHODS: Children with dominant family histories of diabetes were recruited. Direct sequencing for mutations in the two most common MODY genes; HNF1A (TCF1) and GCK was performed in autoantibody-negative probands. We also compared MODY testing data for Asian and White cases from the Exeter MODY database, to 2001 UK census data. RESULTS: We recruited 30 families and identified three Asian families with MODY gene mutations (two HNF1A, one GCK) and three White UK families (two HNF1A, one GCK). Heterozygous MODY phenotypes were similar in Asians and Whites. Only eight (0.5%) of 1369 UK referrals for MODY testing were known to be Asian, but in 2001 Asians represented 4% of the English/Welsh population and have a higher prevalence of diabetes. CONCLUSIONS: We identified three cases of childhood MODY in UK Asians and demonstrated reduced rates of MODY testing in Asians, which has negative implications for treatment. It is unclear why this is. MODY should be considered in autoantibody-negative Asian diabetes patients lacking evidence of insulin resistance.

Adolescent↗
PubMed2005

Factors determining patient choice of device for GH therapy.

AIM: To assess the factors determining patient choice of GH device, and whether offering free patient choice improves compliance with GH therapy. METHODS: A prospective cross-sectional study performed on patients offered free choice of GH device in a regional growth clinic. In a subgroup having home delivery, GH compliance was assessed using ampoule counts. RESULTS: 125 patients (74 (59%) male), median (range) 9.30 (1.0-18.3) years were commenced on GH from January 2001 to March 2004, and offered free choice of device. 68 (54%) chose a needled device, and 57 (46%) needle-free. There was no statistical difference in age, sex or diagnostic category between the two groups. Light blue devices were more likely to be chosen by males (p=0.056). Questionnaires giving reasons for choosing a device were available in 40, and a further 50 gave reasons for both choosing a specific device and not choosing others. Other than choice of needled/needle-free device, the factor most likely to determine choice was 'ease of use'. Only 6 (4.8%) subsequently changed device, and compliance remained high but unchanged at approximately 90%. CONCLUSIONS: There are no specific features which determine what GH device a patient will choose. For those units offering free patient choice, a wide range of different devices should be made available.

Adolescent↗
PubMed2005

Pediatric in vivo cross-calibration between the GE Lunar Prodigy and DPX-L bone densitometers.

Dual energy x-ray absorptiometry (DXA) machine cross-calibration is an important consideration when upgrading from old to new technology. In a recent cross-calibration study using adult subjects, close agreement between GE Lunar DPX-L and GE Lunar Prodigy scanners was reported. The aim of this work was to cross-calibrate the two machines for bone and body composition parameters for pediatrics from age 5 years onwards. One-hundred ten healthy volunteers aged 5-20 years had total body and lumbar spine densitometry performed on DPX-L and Prodigy densitometers. Cross-calibration was achieved using linear regression and Bland-Altman analysis. There was close agreement between the machines, with r2 ranging from 0.85 to 0.99 for bone and body composition parameters. Paired t-tests demonstrated significant differences between machines that were dependent on scan acquisition mode, with the greatest differences reported for the smallest children. At the lumbar spine, Prodigy bone mineral density (BMD) values were on average 1.6% higher compared with DPX-L. For the total body, there were no significant differences in BMD; however, there were significant differences in bone mineral content (BMC) and bone area. For small children, the Prodigy measured lower BMC (9.4%) and bone area (5.8%), whereas for larger children the Prodigy measured both higher BMC (3.1%) and bone area (3.0%). A similar contrasting pattern was also observed for the body composition parameters. Prodigy values for lean body mass were higher (3.0%) for small children and lower (0.5%) for larger children, while fat body mass was lower (16.4%) for small children and higher (2.0%) for large children. Cross-calibration coefficients ranged from 0.84 to 1.12 and were significantly different from 1 (p<0.0001) for BMC and bone area. Bland-Altman plots showed that within the same scan acquisition modes, the magnitude of the difference increased with body weight. The results from this study suggest that the differences between machines are mainly due to differences in bone detection algorithms and that they vary with body weight and scan mode. In general, for population studies the differences are not clinically significant. However, for individual children being measured longitudinally, cross-over scanning may be required.

Absorptiometry, Photon↗
PubMed2005

Management of infants with chronic lung disease of prematurity in the United Kingdom.

The management of chronic lung disease of prematurity (CLD) is challenging for the neonatologist. There are few well-powered randomised controlled trials to inform practice and longer-term outcomes of some interventions have only recently been identified (for example the possible association between the use of corticosteroids and neurodevelopmental abnormalities). As a result, many neonatologists rely on empirical management derived from the evidence available. We describe, in this article, our own approach to the management of CLD, and acknowledge that practice may vary between units within the United Kingdom.

Adrenal Cortex Hormones↗
PubMed2005

Randomized trial of continuous positive airways pressure to prevent reventilation in preterm infants.

A prospective randomized controlled trial was performed comparing the use of a short period (24 hr) of postextubation nasal continuous positive airways pressure (CPAP) with direct extubation into headbox oxygen on the outcome of the need for reintubation within 7 days of initial extubation. Infants at less than 32 weeks of gestation who had received mechanical ventilation in the first 28 postnatal days and were being extubated for the first time were recruited. Ninety-seven babies were entered into the study (48 CPAP and 49 headbox oxygen). Twenty-four (49%) babies in the headbox group were reventilated within a week, compared to 16 (33%) in the CPAP group (P=0.17). By 14 days after initial extubation, 25 babies (51%) in the headbox group and 23 (48%) in the CPAP group required reventilation (P=0.9). There was a trend toward babies in the CPAP group requiring fewer reintubations (median, 2; range, 1-6) compared to those in the headbox group (median, 3; range, 1-7) (P=0.063). There was no significant difference between groups with respect to total number of days of ventilation (headbox median, 4; range, 1-24; CPAP median, 2; range, 1-20). In conclusion, this study showed that a short period of nasal CPAP is not associated with a reduction in reventilation.

Continuous Positive Airway Pressure↗
PubMed2005

Bone mineral density at diagnosis and following successful treatment of pediatric Cushing's disease.

Bone mineral density (BMD) is frequently reduced in children and adolescents with Cushing's disease (CD), but there is little follow-up data after cure. BMD was determined by dual energy X-ray absorptiometry (DEXA) in two groups of patients with CD. Group 1 comprised 8 patients, 5 males and 3 females, aged 12.4 yr (8.2-16.8), assessed at diagnosis. Group 2 comprised 11 subjects, 6 males and 5 females, diagnosed at age 13.3 yr (6.4-17.4), cured by transsphenoidal surgery (TSS) (no.=7) or TSS + pituitary irradiation (no.=4). They had measurement of BMD, at mean age of 18.3 yr (11.1-28.5), i.e. 4.5 yr (0.8-11.4) after cure. Four patients, mean age 20.2 yr (17.6-22.4), had repeated DEXA'scans, 1-4 times, for up to 5.8 yr. After cure, GH deficiency was present in 9 patients and treated with hGH in 8. In Group 1, patients' L2-L4 volumetric (v)BMD Z-score was variable with a mean of -1.04 (-3.21-0.11). L2-L4 vBMD Z-score values correlated negatively with midnight cortisol (p < 0.05). In Group 2, mean L2-L4 vBMD was -0.38 (-1.0-0.13); and in 7/11, mean femoral neck (FN) areal (a)BMD Z-score was 0.14 (-1.62-2.46). FN aBMD Z-score was higher than L2-L4 aBMD Z-score (p < 0.05). In patients with repeated scans, mean change in L2-L4 vBMD Z-score was 0.20 (-0.15-0.45), and mean change in FN aBMD Z-score 0.03 (-0.53-0.38). These findings show variability of BMD at diagnosis and near normal BMD after cure of pediatric CD, suggesting that with appropriate replacement of pituitary hormone deficiency normal peak bone mass is achievable.

Absorptiometry, Photon↗
PubMed2005

Permanent neonatal diabetes in an Asian infant.

We describe a novel homozygous missense glucokinase mutation (R397L) resulting in insulin-treated neonatal diabetes in an infant from a consanguineous Asian family. Both parents were heterozygous for R397L and had mild hyperglycemia. Glucokinase mutations should be considered in infants of all ethnic groups with neonatal diabetes and consanguinity.

Asian People↗
PubMed2005

Some chronic obstructive pulmonary disease will originate in neonatal intensive care units.

Chronic lung disease is the most common adverse outcome in survivors of prematurity. These infants experience frequent hospitalisation because of respiratory-related illness in their first year, as well as persistent cough, wheeze and oxygen dependence. Although the severity of respiratory illness decreases and supplemental oxygen is needed less as their lungs mature, childhood is still complicated by persistent wheeze, cough and reduced exercise tolerance in comparison with their peers. Although there is little longitudinal follow-up data beyond adolescence, imaging studies suggest that these infants are highly likely to suffer with respiratory problems akin to chronic obstructive pulmonary disease in later adulthood. The nature of their long-term respiratory problems, the impact of cigarette smoking and the effect on life expectancy are all unanswered questions that need addressing as these infants grow up.

Humans↗
PubMed2005

Mutation analysis of POUF-1, PROP-1 and HESX-1 show low frequency of mutations in children with sporadic forms of combined pituitary hormone deficiency and septo-optic dysplasia.

OBJECTIVES: Mutations in the genes encoding the transcription factors PROP1 and POUF-1 (Pit-1) have been reported as common causes of combined pituitary hormone deficiency (CPHD), and HESX1 mutations have been identified in children with septo-optic dysplasia (SOD). There are few data on UK children. We have performed mutation analysis in a large cohort of affected children within the West Midlands region to assess the feasibility of a screening strategy for molecular diagnosis in CPHD and SOD. DESIGN AND PATIENTS: The three coding exons of PROP1, and six exons of POUF-1 in 27 children from 26 families with CPHD, and three exons of HESX1 in 23 children from 22 families with SOD were directly sequenced from a well-characterized regional cohort. RESULTS: We identified a C to T transition in exon 6 of POUF-1, resulting in a known missense mutation (R271W) in a mother and daughter from one family with CPHD. We also found a novel homozygous T to C transition in exon 6 of POUF-1, resulting in a missense mutation (F233L) in a twin with CPHD. This mutation was excluded in 100 ethnically matched control alleles. We did not identify any mutations in the PROP1 gene or HESX1. The median maternal age at delivery for the CPHD children was 27 years, compared to 21 years for the mothers of SOD children (P = 0.04). CONCLUSIONS: Mutations in POUF-1, PROP1 and HESX1 are rare causes of CPHD and SOD, respectively, in children from the West Midlands. In particular, we did not confirm the reported 'hotspot' in PROP1. A screening strategy that targets familial cases is highly likely to increase the mutation yield. The young maternal age at conception of children with SOD and potential teratogen exposure indicate the predominance of environmental factors in this condition compared with CPHD.

Child↗
PubMed2005

Bisphosphonate treatment of bone disease.

In 1997 a review article on bisphosphonates in this journal identified 24 published articles relating to children at that time. Since then there has been a considerable increase in their use in clinical paediatric practice and research with there being nearly a further one hundred articles published at the time of writing.

Administration, Oral↗
PubMed2005

Nasal potential difference increases with gestation in moderately preterm neonates on the first postnatal day.

At birth the mammalian airway switches from liquid secretion to absorption, an important mechanism in lung liquid clearance. Airway ion transport was examined on the first postnatal day in 38 moderately preterm infants (29-36 weeks gestation). The absorptive airway ion transport capacity was well developed regardless of respiratory condition and there was little capacity for Cl- secretion.

Absorption↗
PubMed2004

From bone biology to bone analysis.

Bone development is one of the key processes characterizing childhood and adolescence. Understanding this process is not only important for physicians treating pediatric bone disorders, but also for clinicians and researchers dealing with postmenopausal and senile osteoporosis. Bone densitometry has great potential to enhance our understanding of bone development. The usefulness of densitometry in children and adolescents would be increased if the physiological mechanisms and structural features of bone were given more consideration in the design and interpretation of densitometric studies. This review gives an overview on the most relevant techniques of quantitative noninvasive bone analysis. Furthermore it describes the relationship between bone biology, selected surrogates describing the biological processes and the possibilities of measuring these surrogates specifically and precisely by the different devices. The overall recommendation for researchers in this field is to describe firstly the biological process to be analyzed (bone growth in length, remodeling or modeling, or all together), secondly the bone parameter which describes this process, and thirdly the reason for selecting a special device.

Absorptiometry, Photon↗
PubMed2004

The relationship between lean body mass and bone mineral content in paediatric health and disease.

INTRODUCTION: The correct interpretation of DXA data is critical to the diagnosis and management of children with suspected bone disease. This study examines the various influences on bone mineral content (BMC), as measured by dual-energy X-ray absorptiometry (DXA). MATERIALS AND METHODS: Six hundred and forty-six healthy school children and forty-three children with chronic diseases, aged 5-18 years, had their lumbar spine and whole body measured using a Lunar DPX-L DXA scanner. RESULTS: Stepwise linear regression identified lean body mass (LBM) as the strongest single predictor of BMC in the lumbar spine and the total body. A significant gender difference was observed in the relationship between BMC and LBM with girls having significantly more bone per unit LBM from 9 years of age in the spine and 13 years of age in the total body. To investigate the relationship between LBM and BMC in children with chronic disease, a two-stage algorithm based upon calculation of Z scores from the normative data was applied. Stage 1 assessed LBM for height and stage 2 assessed BMC for LBM. Ten children with spinal muscular atrophy had a mean LBM for height Z score of -1.8(1.4) but a mean BMC for LBM Z score of 1.2(1.3) indicating their primary abnormality was reduced muscle mass (sarcopenia) with no evidence of osteopenia. In contrast, 21 children with osteogenesis imperfecta had a mean LBM for height Z score of 0.4(1.7) but a mean BMC for LBM Z score of -2.5(1.8) indicating normal LBM for size but significantly reduced BMC for LBM (i.e. osteopenia) confirming a primary bone abnormality. A third group consisting of 12 children with low trauma fractures demonstrated little evidence of sarcopenia [mean LBM for height Z score -1.1(2.1)] but significant osteopenia [mean BMC for LBM Z score -1.9(1.5)]. CONCLUSION: The results from this study demonstrate how the relationship between height and lean body mass, and lean body mass and bone mineral content can be a useful method of diagnosing osteoporosis in children and how the relationships can be used to identify if the primary abnormality is in muscle or bone.

Adolescent↗