RESUMEN
AIMS: To assess staffing levels of healthcare professionals involved in the care of children and young people with diabetes in the UK. METHODS: A web-based questionnaire was distributed to lead consultant paediatricians from all paediatric diabetes services in the UK between October and December 2014. Data on staffing levels and other aspects of diabetes services were collected and differences between the four nations of the UK and across the 10 English diabetes networks were explored. RESULTS: Some 175 services (93%) caring for 29 711 children and young people aged ≤ 24 years with diabetes participated in the survey. Northern Ireland and Wales had the lowest ratio of total staff to patient population. Nursing caseloads per one whole-time equivalent (WTE) nurse ranged from 71 patients in England to 110 patients in Northern Ireland with only 52% of the UK services meeting the Royal College of Nursing recommended nurse-to-patient ratio of > 1 : 70. Scotland and Northern Ireland had the highest ratio of consultants and fully trained doctors per 1000 patients (3.5 WTE). Overall, 17% of consultants had a Certificate of Completion of Training in Endocrinology and Diabetes. Some 44% of dietitians were able to adjust insulin dose. Only 43% of services provided 24-h access to advice from the diabetes team and 82% of services had access to a psychologist. Staffing levels adjusted for volume were not directly related to glycaemic performance of services in England and Wales. CONCLUSIONS: Wide variations in staffing levels existed across the four nations of the UK and important gaps were present in key areas.
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Servicios de Salud del Adolescente/estadística & datos numéricos , Diabetes Mellitus/enfermería , Servicios de Salud/estadística & datos numéricos , Adolescente , Atención Posterior/estadística & datos numéricos , Niño , Servicios de Salud del Niño/estadística & datos numéricos , Consultores/estadística & datos numéricos , Diabetes Mellitus/sangre , Hemoglobina Glucada/metabolismo , Encuestas de Atención de la Salud , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Humanos , Nutricionistas/provisión & distribución , Profesionales de Enfermería Pediátrica/provisión & distribución , Psicología/estadística & datos numéricos , Reino Unido , Recursos Humanos , Adulto JovenRESUMEN
AIM: To understand the scope for improving children's glycaemic outcomes by reducing variation between clinics and examine the role of insulin regimen and clinic characteristics. METHODS: Cross-sectional analysis of 2012-2013 National Paediatric Diabetes Audit data from 21 773 children aged < 19 years with Type 1 diabetes cared for at 176 clinics organized into 11 regional diabetes networks in England and Wales. Variation in HbA1c was explored by multilevel models with a random effect for clinic. The impact of clinic context was quantified by computing the per cent of total variation in HbA1c which occurs between clinics (intraclass correlation coefficient; ICC). RESULTS: Overall, 69 of the 176 diabetes clinics (39%) had a glycaemic performance that differed significantly from the national average after adjusting for patient case-mix with respect to age, gender, diabetes duration, deprivation and ethnicity. However, differences between clinics accounted for 4.7% of the total variation in HbA1c . Inclusion of within-clinic HbA1c standard deviation led to a substantial reduction in ICC to 2.4%. Insulin regimen, clinic volume and diabetes networks had a small or moderate impact on ICC. CONCLUSIONS: Differences between diabetes clinics accounted for only a small portion of the total variation in glycaemic control because most of the variation was within clinics. This implies that national glycaemic improvements might best be achieved not only by targeting poor centres but also by shifting the whole distribution of clinics to higher levels of quality.
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Glucemia/metabolismo , Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Diabetes Mellitus Tipo 1/epidemiología , Hipoglucemiantes/uso terapéutico , Adolescente , Variación Biológica Poblacional , Niño , Preescolar , Auditoría Clínica , Estudios Transversales , Inglaterra/epidemiología , Femenino , Hemoglobina Glucada/metabolismo , Humanos , Lactante , Recién Nacido , Insulina/uso terapéutico , Masculino , Análisis Multinivel , Autocuidado , Gales/epidemiologíaRESUMEN
BACKGROUND: The impact of ethnicity and socio-economic status (SES) on glycaemic control during childhood Type 1 diabetes is poorly understood in England and Wales. METHODS: We studied 18 478 children with Type 1 diabetes (< 19 years) attending diabetes clinics and included in the 2012-2013 National Paediatric Diabetes Audit. Self-identified ethnicity was categorized as white, Asian, black, mixed, other and 'not-stated' (did not to divulge ethnicity). A small area measure of SES was estimated from the Index of Multiple Deprivation. Multiple linear regression was used to assess associations between ethnicity, SES and glycaemic control (mean HbA1c levels) accounting for age, gender and diabetes duration. The impact of insulin pump use on the ethnicity/SES-HbA1c associations was tested in 13 962 children. RESULTS: All children from minority ethnic groups had higher mean HbA1c compared with white children, with largest differences observed in black and mixed ethnicities [8 mmol/mol (2.9%), 95% CI 5-11 and 7 mmol/mol (2.8%), 95% CI 5-9, respectively]. Lower SES was associated with higher mean HbA1c with a dose effect. The lowest SES group had a mean HbA1c that was 7 mmol/mol (2.8%) (95% CI 6-8) higher compared with the highest SES group, adjusted for ethnicity. Estimates for ethnicity were attenuated, but significant on adjustment for SES. Fewer non-white (white 20.3 vs. black 5.5%) and deprived (least deprived 21.1 vs. most deprived 13.2%) children were on insulin pump therapy. Ethnicity and SES remained significant predictors of HbA1c after accounting for insulin pump use. CONCLUSION: The association between ethnicity and glycaemic control persists after adjustment for deprivation and pump use. An alternative approach to intensive insulin therapy might benefit these vulnerable children.
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Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/etnología , Hemoglobina Glucada/metabolismo , Grupos Minoritarios/estadística & datos numéricos , Clase Social , Adolescente , Glucemia/metabolismo , Niño , Preescolar , Diabetes Mellitus Tipo 1/epidemiología , Inglaterra/epidemiología , Etnicidad , Femenino , Humanos , Lactante , Masculino , Gales/epidemiología , Adulto JovenRESUMEN
AIMS: Improving glycaemic control in people with Type 1 diabetes is known to reduce complications. Our aim was to compare glycaemic control among people with Type 1 diabetes using data gathered in regional or national registries. METHODS: Data were obtained for children and/or adults with Type 1 diabetes from the following countries (or regions): Western Australia, Austria, Denmark, England, Champagne-Ardenne (France), Germany, Epirus, Thessaly and Thessaloniki (Greece), Galway (Ireland), several Italian regions, Latvia, Rotterdam (The Netherlands), Otago (New Zealand), Norway, Northern Ireland, Scotland, Sweden, Volyn (Ukraine), USA and Wales) from population or clinic-based registries. The sample size with available data varied from 355 to 173 880. Proportions with HbA1c < 58 mmol/mol (< 7.5%) and ≥ 75 mmol/mol (≥ 9.0%) were compared by age and sex. RESULTS: Data were available for 324 501 people. The proportions with HbA1c 58 mmol/mol (< 7.5%) varied from 15.7% to 46.4% among 44 058 people aged < 15 years, from 8.9% to 49.5% among 50 766 people aged 15-24 years and from 20.5% to 53.6% among 229 677 people aged ≥ 25 years. Sex differences in glycaemic control were small. Proportions of people using insulin pumps varied between the 12 sources with data available. CONCLUSION: These results suggest that there are substantial variations in glycaemic control among people with Type 1 diabetes between the data sources and that there is room for improvement in all populations, especially in young adults.
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Diabetes Mellitus Tipo 1/tratamiento farmacológico , Hemoglobina Glucada/metabolismo , Hipoglucemiantes/uso terapéutico , Sistemas de Infusión de Insulina/estadística & datos numéricos , Insulina/uso terapéutico , Sistema de Registros , Adolescente , Adulto , Austria , Dinamarca , Diabetes Mellitus Tipo 1/metabolismo , Inglaterra , Femenino , Francia , Alemania , Grecia , Adhesión a Directriz , Humanos , Irlanda , Italia , Letonia , Masculino , Países Bajos , Nueva Zelanda , Irlanda del Norte , Noruega , Guías de Práctica Clínica como Asunto , Escocia , Suecia , Ucrania , Estados Unidos , Gales , Australia Occidental , Adulto JovenRESUMEN
BACKGROUND/AIMS: Treatment with intralesional triamcinolone/betamethasone is recommended for infantile sight-threatening periocular haemangiomas. This study investigates the endocrine and weight changes in 15 infants undergoing therapy over 12 years. METHODS: 15 infants, median age 19 weeks (range 10-56) receiving intra/perilesional triamcinolone/betamethasone underwent serial measurement of weight, early morning serum cortisol and adrenocorticotropic hormone (ACTH) before and after injection. RESULTS: Cortisol fell from a median (range) of 383 (112-594) to 28 (<10-506) nmol/l (p=0.005) and ACTH from 26 (14-134) to 9 (5-20) ng/l (p=0.05) from before injection to 4 weeks after treatment. Prolonged adrenal suppression occurred in 13 out of 15 cases with time to recovery being 19.5 (4-65) weeks. Failure to gain weight appropriately was observed in 14 infants but recovered once normal adrenal function was re-established. CONCLUSION: Prolonged adrenal suppression following triamcinolone/betamethasone injection for periocular haemangiomas is common and associated with faltering weight gain.
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Insuficiencia Suprarrenal/inducido químicamente , Neoplasias de los Párpados/tratamiento farmacológico , Glucocorticoides/efectos adversos , Hemangioma/tratamiento farmacológico , Insuficiencia Suprarrenal/sangre , Hormona Adrenocorticotrópica/sangre , Betametasona/administración & dosificación , Betametasona/efectos adversos , Preescolar , Combinación de Medicamentos , Femenino , Glucocorticoides/administración & dosificación , Humanos , Hidrocortisona/sangre , Lactante , Inyecciones Intralesiones , Masculino , Estudios Retrospectivos , Triamcinolona/administración & dosificación , Triamcinolona/efectos adversos , Aumento de Peso/efectos de los fármacosRESUMEN
INTRODUCTION: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with six affected cases from three families reported to date. Additional features, described previously, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay and facial dysmorphism. SUBJECTS: We report two new cases from unrelated families with distinct novel homozygous partial GLIS3 deletions. Both patients presented with neonatal diabetes mellitus, severe resistant hypothyroidism in the presence of elevated thyroglobulin and normal thyroid anatomy, degenerative liver disease, cystic renal dysplasia, recurrent infections and facial dysmorphism. These novel mutations have also resulted in osteopenia, bilateral sensorineural deafness and pancreatic exocrine insufficiency, features that have not previously been associated with GLIS3 mutations. Gene dosage analysis showed that the parents were carriers of a deletion encompassing exons 1-2 (case 1) or exons 1-4 (case 2) of the 11 exon gene. Genome-wide SNP analysis did not reveal a common ancestral GLIS3 haplotype in patient 2. CONCLUSIONS: Our results confirm partial gene deletions as the most common type of GLIS3 mutations, accounting for four of five families identified to date. We propose that mutations in GLIS3 lead to a wider clinical phenotype than previously recognised. We also report the first case of a recessive GLIS3 mutation causing neonatal diabetes and congenital hypothyroidism in a child from a non-consanguineous pedigree, highlighting the importance of molecular genetic testing in any patient with this phenotype.
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Mutación , Fenotipo , Factores de Transcripción/genética , Hipotiroidismo Congénito/genética , Proteínas de Unión al ADN , Diabetes Mellitus/genética , Exones/genética , Femenino , Dosificación de Gen/genética , Haplotipos/genética , Humanos , Recién Nacido , Masculino , Proteínas Represoras , TransactivadoresRESUMEN
OBJECTIVE: The study examined the degree to which male and female survivors of acute lymphoblastic leukaemia (ALL) perceive effort at low and moderate intensity exercise in association with related physiological variables. MATERIALS AND METHODS: Participants were 67 children. Thirty-five (14 boys and 21 girls) were long-time survivors of ALL and 32 (18 boys and 14 girls) were control subjects. The Children's Effort Rating Table (CERT) was used to measure whole-body perceived exertion at low and moderate intensity exercise. Peak oxygen uptake was measured using a motorized treadmill. CERT and physiological data were analysed using 2 x 2 mixed analyses of variance, appropriate t-tests and coefficients of correlation. RESULTS: In absolute terms, boys treated for ALL found perception of effort to be more strenuous at both low (3.9 vs. 3.5 units) and moderate (6.1 vs. 5.3 units) intensity exercise than control subjects, although differences were not significant (p > 0.05); girls treated for ALL found perception of effort to be the same as controls at low intensity exercise (3.1 vs. 3.1 units) but slightly higher than controls at moderate intensity exercise (5.6 vs. 5.2 units); neither of these differences were significant (p > 0.05). When CERT values were adjusted for (.-)VO(2) peak (%) and heart rate (HR) peak (%) differences remained non-significant. There were no significant interactions (Intensity x Group) in males, but the interaction for (.-)VO(2) peak (%) was significant in females (p < 0.05). The main effect for Intensity (low and moderate) was significant for all variables in boys and girls (p < 0.0001). The main effect for Group (ALL and controls) identified significantly greater absolute (b.p.m.) and relative (%) HR values in ALL boys at low and moderate intensity exercise. In female ALL and control subjects the interaction (Intensity x Group) distinguished between (.-)VO(2) peak (%) at moderate intensity exercise and HR peak (%) at low and moderate intensity exercise. Coefficients of correlation between perceived effort and (.-)VO(2) peak (%) in boys and girls were low to high (0.28-0.76), and between absolute and relative HR were also low to high (0.33-0.73). There were low correlations between time 'off therapy' and perceived effort, (.-)VO(2) peak (%) and HR peak (%) (-0.003 to -0.49). CONCLUSION: It was concluded that perception of effort in survivors of ALL at low and moderate intensity exercise was the same as that of control subjects. Correlations between perceived effort and physiological variables at moderate exercise were low to high, while those between perceived effort and time from treatment were generally weak.
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Esfuerzo Físico/fisiología , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatología , Análisis de Varianza , Estudios de Casos y Controles , Niño , Estudios Transversales , Prueba de Esfuerzo , Tolerancia al Ejercicio/fisiología , Femenino , Frecuencia Cardíaca/fisiología , Humanos , Masculino , Consumo de Oxígeno/fisiologíaRESUMEN
BACKGROUND: Craniopharyngiomas account for 2-5% of all primary intracranial tumours. Despite their benign histological appearance, they are often associated with an unfavourable prognosis and their optimal treatment remains controversial. AIM: To analyse the natural history and treatment outcome of children and adults presenting to the Departments of Paediatrics and Endocrinology with craniopharyngioma between 1964 and 2003. PATIENTS AND METHODS: The records of 121 patients (age range 2.5-83 years, 42 aged < 16 and 79 aged > or = 16) were identified. The mean follow-up period since presentation was 103 months (8.6 years) (range 0.3-468 months). Sixteen patients underwent gross total removal (A), 3 gross total removal + radiotherapy (B), 51 partial removal (C), 33 partial removal + radiotherapy (D), 6 cyst evacuation alone (E) and 3 cyst evacuation + radiotherapy (F). The clinical, imaging and endocrinological data at presentation and during follow-up were analysed. RESULTS: Headache and visual field defects were the most common presenting clinical features (64% and 55%, respectively). Ninety-four per cent of the tumours had an extrasellar component and 23% of them were associated with hydrocephalus. There was a significant difference in the recurrence-free survival rates between groups A-D [at 10 years: 100% (A), 100% (B), 38% (C) and 77% (D), P < 0.01], which persisted even when analysing patients operated after 1980. The median time of first recurrence was 2.5 years (range 0.5-36). The peri-operative mortality of the patients who had any type of neurosurgical intervention due to recurrence was higher than that observed after primary surgery (24%vs. 1.8%) (P < 0.01). The rate of re-accumulation of the cyst fluid was 58% during the first year in patients of group E, whereas none of the subjects of group F experienced such an event during their follow-up period. There was no reversal of pre-existing pituitary hormone deficits after any surgical intervention. The probabilities of GH, FSH/LH, ACTH, TSH deficiency and diabetes insipidus at the 10-year follow-up were 88%, 90%, 86%, 80% and 65%, respectively. After excluding the non-tumour-related deaths, the 10-year survival rate following presentation was 90%. Patients with recurrence had a significantly lower probability for survival compared with those without it (at 10 years: 70%vs. 99%, P < 0.01). At the 10-year follow-up the probability of the presence of major visual field defects was 48%, hyperphagia/obesity 39%, epilepsy 12% and hemi-/monoparesis 11%. In this large series no substantial differences in the outcome of tumours diagnosed during childhood or adult life were found. CONCLUSIONS: Craniopharyngiomas remain tumours associated with significant morbidity. Gross total removal provides favourable results in terms of recurrences. If this cannot be achieved safely, adjuvant radiotherapy is beneficial in preventing tumour re-growth. Childhood- and adult-onset lesions generally behave similarly.
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Craneofaringioma , Recurrencia Local de Neoplasia , Neoplasias Hipofisarias , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Distribución de Chi-Cuadrado , Niño , Preescolar , Craneofaringioma/diagnóstico , Craneofaringioma/mortalidad , Craneofaringioma/cirugía , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/mortalidad , Recurrencia Local de Neoplasia/cirugía , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/mortalidad , Neoplasias Hipofisarias/cirugía , Estadísticas no Paramétricas , Tasa de SupervivenciaRESUMEN
BACKGROUND: Body fat mass (FM) and fat free mass (FFM) in childhood are often estimated by conversion of a measured variable into compartmental body composition using constants or regression equations that have been previously derived in healthy individuals. Application of such constants or equations to children with disease states may lead to inappropriate conclusions since the "normal" relationships may become altered. AIMS AND METHODS: To test this hypothesis by taking measurements of body composition using dual energy x ray absorptiometry (DEXA) as a "gold standard" method and calculating hydration and body potassium constants using isotopic water dilution and whole body potassium counting. Measurements of bioelectrical impedance (BIA) by two different analysers (RJL and Holtain) were also performed to allow comparison with body water measurements. RESULTS: Measurements were performed in 35 children treated for acute lymphoblastic leukaemia (ALL) and compared to those in 21 children treated for a variety of other malignancies and 32 healthy sibling controls. The mean hydration and potassium content of FFM was significantly reduced in the ALL group compared to both other malignancies and controls. Application of equations derived from controls for the measurement of FFM derived from bioelectrical impedance led to an underestimation of 1.15 kg when compared to that derived from DEXA in children treated for ALL but not in other malignancies. For all groups combined, BIA was significantly different in the two analysers. CONCLUSION: Care needs to be taken in the application of equations derived from the normal population to body composition measurement in children treated for ALL.
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Composición Corporal , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatología , Absorciometría de Fotón , Tejido Adiposo/química , Índice de Masa Corporal , Agua Corporal/química , Niño , Estudios de Cohortes , Impedancia Eléctrica , Humanos , Potasio/análisis , SobrevivientesRESUMEN
UNLABELLED: The extent to which childhood GHD affects adult fracture risk is unclear. We measured femoral strength in adult transgenic growth-retarded rats as a model of GHD. Long-term, moderate GHD was accompanied by endocrine and morphometric changes consistent with a significant reduction in femoral strength. INTRODUCTION: Childhood growth hormone deficiency (GHD) is associated with osteopenia, but little is known about its effects on subsequent adult bone strength and fracture risk. MATERIALS AND METHODS: We have therefore measured femoral strength (failure load measured by three-point bending) in a new model of moderate GHD, the transgenic growth-retarded (Tgr) rat at 15, 22-23, and 52 weeks of age, and have quantified potential morphological and endocrine determinants of bone strength. RESULTS: Skeletal growth retardation in Tgr rats was accompanied by a sustained reduction in the anterior-posterior diameter of the femoral cortex, whereas mid-diaphyseal cortical wall thicknesses were largely unaltered. Total femoral strength was significantly impaired in Tgr rats (p < 0.01), and this impairment was more pronounced in males than females. Compromised bone strength in Tgr rats could not be accounted for by the reduction in mechanical load (body weight) and was not caused by impairment of the material properties of the calcified tissue (ultimate tensile stress), despite marked reductions in femoral mineral density (areal bone mineral density; p < 0.001). Microcomputerized tomographical analysis revealed significant modification of the architecture of trabecular bone in Tgr rats, with reductions in the number and thickness of trabeculae (p < 0.05) and in the degree of anisotropy (p < 0.01). The marked reduction in plasma insulin-like growth factor-1 in Tgr rats was accompanied by the development of high circulating leptin levels (p < 0.01). CONCLUSION: These results show that the changes in endocrinology and bone morphology associated with long-term moderate GHD in Tgr rats are accompanied by changes consistent with a significant reduction in the threshold for femoral fracture.
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Enanismo/fisiopatología , Fémur/fisiología , Hormona del Crecimiento/deficiencia , Factores de Edad , Animales , Animales Modificados Genéticamente , Fenómenos Biomecánicos , Densidad Ósea , Desarrollo Óseo , Calcificación Fisiológica , Fuerza Compresiva , Enanismo/genética , Femenino , Fémur/crecimiento & desarrollo , Fémur/metabolismo , Hormona del Crecimiento/genética , Factor I del Crecimiento Similar a la Insulina/análisis , Leptina/sangre , Masculino , Ratas , Caracteres Sexuales , Aumento de PesoRESUMEN
BACKGROUND: Until recently, only two cases of acute adrenal crisis associated with inhaled corticosteroids (ICS) had been reported worldwide. We identified four additional cases and sought to survey the frequency of this side effect in the United Kingdom. METHODS: Questionnaires were sent to all consultant paediatricians and adult endocrinologists registered in a UK medical directory, asking whether they had encountered asthmatic patients with acute adrenal crisis associated with ICS. Those responding positively completed a more detailed questionnaire. Diagnosis was confirmed by symptoms/signs and abnormal hypothalamic-pituitary-adrenal axis function test results. RESULTS: From an initial 2912 questionnaires, 33 patients met the diagnostic criteria (28 children, five adults). Twenty-three children had acute hypoglycaemia (13 with decreased levels of consciousness or coma; nine with coma and convulsions; one with coma, convulsions and death); five had insidious onset of symptoms. Four adults had insidious onset of symptoms; one had hypoglycaemia and convulsions. Of the 33 patients treated with 500-2000 micro g/day ICS, 30 (91%) had received fluticasone, one (3%) fluticasone and budesonide, and two (6%) beclomethasone. CONCLUSIONS: The frequency of acute adrenal crisis was greater than expected as the majority of these patients were treated with ICS doses supported by British Guidelines on Asthma Management. Despite being the least prescribed and most recently introduced ICS, fluticasone was associated with 94% of the cases. We therefore advise that the licensed dosage of fluticasone for children, 400 micro g/day, should not be exceeded unless the patient is being supervised by a physician with experience in problematic asthma. We would also emphasise that until adrenal function has been assessed patients receiving high dose ICS should not have this therapy abruptly terminated as this could precipitate adrenal crisis.
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Corticoesteroides/efectos adversos , Enfermedades de las Glándulas Suprarrenales/inducido químicamente , Asma/tratamiento farmacológico , Enfermedad Aguda , Administración por Inhalación , Adolescente , Corticoesteroides/administración & dosificación , Enfermedades de las Glándulas Suprarrenales/epidemiología , Enfermedades de las Glándulas Suprarrenales/fisiopatología , Adulto , Anciano , Anciano de 80 o más Años , Antropometría , Asma/fisiopatología , Niño , Preescolar , Femenino , Encuestas de Atención de la Salud , Humanos , Hipoglucemia/inducido químicamente , Sistema Hipotálamo-Hipofisario/fisiopatología , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Reino Unido/epidemiologíaRESUMEN
Four cases of asthma (one adult, three children) developing acute adrenal crisis after introduction of high-dose inhaled fluticasone proprionate are presented. The three children, aged 7-9 yrs, had been prescribed inhaled fluticasone, dosage 500-2,000 microg x day(-1) and duration 5 months-5 yrs. All presented with convulsions due to hypoglycaemia (blood glucose 1.3-1.8 mM). The fourth case was a male of 33 yrs with difficult-to-control asthma and had been taking fluticasone propionate 1,000-2,000 microg x day(-1) for 3 yrs. He presented with fatigue, lethargy, nausea and postural hypotension. Acute adrenal crisis in each case was confirmed by investigations which included measurement of acute phase cortisol levels, short and long Synacthen stimulation tests and glucagon stimulation tests. Other cases of hypthoalamic-pituitary-adrenal axis suppression were excluded.
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Insuficiencia Suprarrenal/inducido químicamente , Androstadienos/efectos adversos , Antiinflamatorios/efectos adversos , Asma/tratamiento farmacológico , Enfermedad Aguda , Adulto , Androstadienos/administración & dosificación , Antiinflamatorios/administración & dosificación , Niño , Femenino , Fluticasona , Humanos , MasculinoAsunto(s)
Estatura , Peso Corporal , Estado Nutricional , Índice de Masa Corporal , Niño , Preescolar , Humanos , Reproducibilidad de los ResultadosRESUMEN
Osteoporosis in adult life is associated with a significant morbidity and may be predisposed to by osteopenia and failure to reach peak bone mass in childhood. Children treated for acute lymphoblastic leukemia (ALL) may be at risk of osteopenia as a result of previous therapy or as a consequence of the disease process itself. Dual energy x-ray absorptiometry measurements of bone mineral content (BMC) for the whole body and at the lumbar spine and hip were taken in 35 (14 male) long-term survivors of ALL and compared with results in 20 (10 male) survivors of other malignancies and 31 (17 male) healthy sibling controls. The measured BMC was expressed as a percentage of a predicted value derived from the control group and based on the variables that had influence upon it. BMC (%) was reduced at the spine in the ALL group compared with controls [92.4 (8.0)% versus 100.4 (9.7)%, respectively; p < 0.005] and at the hip compared with both other malignancies and controls [89.0 (11.5)% versus 96.1 (11.7)% and 100.4 (9.2)%, respectively; p < 0.0005]. Increasing length of time off therapy was associated with a significant increase in %BMC at both the spine and the hip. For the spine, this association was significantly different between the ALL group and other malignancies, suggesting that any gain in %BMC after therapy was slower in children treated for ALL. Both exercise capacity and levels of physical activity were correlated with %BMC at the hip (r = 0.44, p < 0.001 and r = 0.29, p < 0.01, respectively). Previous exposure to methotrexate, ifosfamide, and bleomycin was associated with a reduction in %BMC at the spine. Exposure to 6-mercaptopurine and cisplatin was associated with a reduction at the hip. In conclusion, children treated for ALL are osteopenic. The mechanism is probably multifactorial but is partially related to previous chemotherapy, limited exercise capacity, and relative physical inactivity.
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Enfermedades Óseas Metabólicas/etiología , Neoplasias/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Estatura , Peso Corporal , Densidad Ósea , Enfermedades Óseas Metabólicas/epidemiología , Niño , Femenino , Estudios de Seguimiento , Humanos , Masculino , Neoplasias/fisiopatología , Consumo de Oxígeno , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatología , Pubertad , Radioterapia/efectos adversos , Valores de Referencia , Análisis de Regresión , Inducción de Remisión , Columna Vertebral/fisiopatología , Sobrevivientes , Factores de TiempoRESUMEN
Preterm infants fed human milk have been shown to grow poorly and develop mineral deficiencies that may lead to osteopenia. This study has investigated the efficacy of a human milk fortifier made up of glucose polymers, a mixture of bovine milk protein fractions and free amino acids, minerals and vitamins designed to improve these nutritional deficiencies. Growth and bone mineral deficiencies were compared in 38 preterm infants fed fortified mother's milk and 21 preterm infants fed a preterm formula until they reached 1800 g; all had a birthweight below 1600 g. Weight gain was similar in each group with a mean (SD) increase of 19.6 (3.5) g/kg/day in the fortified group and 19.9 (4.1) g/kg/day in the preterm formula group. There were also no significant differences in linear growth, head circumference, skin fold thickness or mid-arm circumference. Serum phosphate, alkaline phosphatase and plasma urea concentrations were similar and there was no clinical evidence of osteopenia. These results indicate that the growth and metabolic disadvantages associated with feeding human milk to preterm infants are ameliorated by the addition of a milk fortifier that increases the calorific, protein and mineral content of breast milk.
Asunto(s)
Alimentos Infantiles , Recien Nacido Prematuro/crecimiento & desarrollo , Recien Nacido Prematuro/metabolismo , Leche Humana , Animales , Bovinos , Femenino , Alimentos Fortificados , Humanos , Recién Nacido , Masculino , Aumento de PesoRESUMEN
Changes in body composition, in particular the onset of obesity, may result from reductions in total daily energy expenditure (TDEE) as a consequence of relative physical inactivity. Children previously treated for acute lymphoblastic leukemia (ALL) become obese, yet the mechanism remains undefined. TDEE and physical activity levels [PAL = TDEE/basal metabolic rate (BMR)] were measured in 34 long-term survivors of ALL and compared with results from 21 survivors of other malignancies and 32 healthy sibling control subjects using the flex-heart rate technique. Body composition was measured by dual energy x-ray absorptiometry. The median TDEE was reduced in the ALL group (150 kJ x kg d(-1)) compared with other malignancies and controls (207 and 185 kJ x kg d(-1), respectively, p < 0.01). This reduction was accounted for mainly by a relative decrease in the PAL of the ALL group (1.24) compared with both other malignancies and controls (1.58 and 1.47, respectively, p < 0.01). TDEE and PAL were correlated with percentage body fat (r = -0.39, p < 0.001 and r = -0.24, p < 0.05, respectively). Obesity in survivors of ALL may, in part, be explained by a reduction in TDEE as a consequence of reduced PAL. The cause of such reduction is uncertain.
Asunto(s)
Metabolismo Basal , Metabolismo Energético , Frecuencia Cardíaca , Neoplasias , Esfuerzo Físico , Sobrevivientes , Adolescente , Análisis de Varianza , Niño , Ingestión de Energía , Femenino , Humanos , Masculino , Obesidad/epidemiología , Obesidad/etiología , Postura , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamiento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/radioterapia , Valores de Referencia , Análisis de RegresiónRESUMEN
Dual-energy X-ray absorptiometry (DEXA) is a rapid and precise technique for the assessment of bone mineralization in children. Interpretation of the results in growing children is complex as results are influenced by age, body size (height and weight) and puberty. Conventionally, bone mineral data derived from DEXA have been presented as an areal density [BMD; bone mineral content (BMC, g)/projected bone area (BA, cm2)], yet this fails to account for changes in BMC that result from changes in age, body size or pubertal development. Measurement of BMC and BA of the whole body, lumbar spine and left hip were made in 58 healthy boys and girls using DEXA. The relationship between BMC and BA was curvilinear, with the best fit being that of a power model (BMD = BMC/BAlambda, where lambda is the exponent to which BA is raised in order to remove its influence on BMC). The value of lambda changed when measures of body size and puberty were taken into account (e.g. for lumbar spine from 1.66 to 1.49). Predictive formulae for BMC were produced using regression analysis and based on the variables of age, body size and pubertal development. This provides a method for interpreting the measured BMC which is independent of such variables and a constant reference range for children aged 6-18 y.
Asunto(s)
Estatura/fisiología , Peso Corporal/fisiología , Densidad Ósea/fisiología , Pubertad/fisiología , Absorciometría de Fotón , Adolescente , Factores de Edad , Antropometría , Niño , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/fisiopatología , Enfermedad de Crohn/terapia , Femenino , Humanos , Masculino , Análisis Multivariante , Valores de Referencia , Análisis de Regresión , Sensibilidad y Especificidad , Factores Sexuales , Testosterona/uso terapéuticoRESUMEN
AIMS: To define outcome measures for auditing the clinical care of children and adolescents with insulin dependent diabetes mellitus (IDDM) and to assess the benefit of appointing a dedicated paediatric trained diabetes specialist nurse (PDSN). METHODS: Retrospective analysis of medical notes and hospital records. Glycaemic control, growth, weight gain, microvascular complications, school absence, and the proportion of children undergoing an annual clinical review and diabetes education session were assessed. The effect of the appointment of a PDSN on the frequency of hospital admission, length of inpatient stay, and outpatient attendance was evaluated. RESULTS: Children with IDDM were of normal height and grew well for three years after diagnosis, but grew suboptimally thereafter. Weight gain was above average every year after diagnosis. Glycaemic control was poor at all ages with only 16% of children having an acceptable glycated haemoglobin. Eighty five per cent of patients underwent a formal annual clinical review, of whom 16% had background retinopathy and 20% microalbuminuria in one or more samples. After appointing the PDSN the median length of hospital stay for newly diagnosed patients decreased from five days to one day, with 10 of 24 children not admitted. None of the latter was admitted during the next year. There was no evidence of the PDSN affecting the frequency of readmission or length of stay of children with established IDDM. Non-attendance at the outpatient clinic was reduced from a median of 19 to 10%. CONCLUSIONS: Outcome measures for evaluating the care of children with IDDM can be defined and evaluated. Specialist nursing support markedly reduces the length of hospital stay of newly diagnosed patients without sacrificing the quality of care.