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1.
J Pediatr ; 169: 232-7, 2016 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-26593106

RESUMEN

OBJECTIVE: To use peripheral quantitative computed tomography to determine the cross-sectional area (CSA) of subcutaneous fat and muscle (fat CSA, muscle CSA) in transverse forearm scans in patients with osteogenesis imperfecta (OI). STUDY DESIGN: Fat and muscle CSA were quantified in 266 individuals (142 female) aged 5-20 years who had a diagnosis of OI type I, III, or IV and who had mutations in COL1A1 or COL1A2. Results were compared with those of 255 healthy controls. RESULTS: In a subgroup of 39 patients with OI type I, % fat CSA correlated closely with total body percentage fat mass as determined by dual-energy x-ray absorptiometry (R(2) = 0.69; P < .001). In the entire study cohort, muscle CSA adjusted for age, sex, and forearm length was lower in OI type I and III than in controls (P < .05 each), but fat CSA was similar between OI types and controls. No relationship between the type of disease-causing mutation in the COL1A1 or COL1A2 genes and fat CSA or muscle CSA was found. CONCLUSIONS: Children and adolescents with OI have low muscle size but a normal amount of subcutaneous fat at the forearm.


Asunto(s)
Composición Corporal , Osteogénesis Imperfecta/metabolismo , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos , Adulto Joven
2.
J Pediatr ; 163(4): 1095-8.e4, 2013 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-23746867

RESUMEN

OBJECTIVE: To evaluate metabolic consequences of growth hormone (GH) treatment in children with type 1 diabetes. STUDY DESIGN: This study is an analysis of metabolic changes in 37 patients with childhood-onset GH deficiency and type 1 diabetes, documented in the Diabetes Patienten Verlaufsdocumentationsystem database. Main outcome measures were changes in hemoglobin A1c and daily insulin requirements during GH therapy in children with GH deficiency and type 1 diabetes compared with a large cohort of adolescents with type 1 diabetes. RESULTS: Thirty-seven patients with type 1 diabetes and a diagnosis of idiopathic GH deficiency after onset of diabetes were compared with 48856 patients with type 1 diabetes. After adjustment for age, sex, duration of diabetes, and migration background, a significant difference in mean daily insulin requirement was seen between the 2 groups (1.0 IU/kg/day in subjects with GH deficiency and type 1 diabetes vs 0.85 IU/kg/day in controls; P < .01) and height-SDS (-2.0 in subjects with GH deficiency and diabetes vs +0.03 in controls; P < .0001). There was no significant between-group difference in hemoglobin A1 concentration, however (8.1% ± 1.4% in patients with GH deficiency and type 1 diabetes vs 8.2% ± 1.7% in those with type 1 diabetes only; P > .05). CONCLUSION: An increased daily insulin requirement should be considered in patients with type 1 diabetes treated with GH. With adequate adaptation of insulin dosage, metabolic control is not impaired during GH treatment.


Asunto(s)
Complicaciones de la Diabetes/sangre , Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/terapia , Hormona de Crecimiento Humana/deficiencia , Hormona de Crecimiento Humana/uso terapéutico , Adolescente , Índice de Masa Corporal , Niño , Estudios de Cohortes , Complicaciones de la Diabetes/metabolismo , Diabetes Mellitus Tipo 1/metabolismo , Femenino , Hemoglobina Glucada/metabolismo , Humanos , Incidencia , Insulina/metabolismo , Insulina/uso terapéutico , Masculino , Resultado del Tratamiento
4.
J Pediatr ; 146(6): 764-8, 2005 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-15973315

RESUMEN

OBJECTIVES: To investigate the relation between volumetric bone mineral density (vBMD) and speed of sound (SOS). STUDY DESIGN: Total and trabecular vBMD were measured by peripheral quantitative computed tomography at the forearm in a population of 216 individuals of a pediatric outpatient clinic. Moreover, SOS was measured by a quantitative ultrasound device (QUS) at the thumb, patella, and os calcis. RESULTS: Linear regression analysis revealed that the prediction of SOS by vBMD is relatively weak (R2 < 0.1). Moreover, body height and measures of bone size have a stronger influence on SOS than vBMD. The influence of bone size on SOS also depends on the location of measurement (highest prediction of SOS by body height at patella with R2 = 0.56). Anthropometric characteristics have a stronger influence on SOS than measures of bone mineral density at the thumb and patella in comparison to os calcis (body height predicts SOS at os calcis, with R2 = 0.03). Conclusions QUS is not a suitable method to assess bone density. If QUS is applied for the assessment of bone development and of bone fracture risk, the measurement should be performed with consideration of anthropometric measurements.


Asunto(s)
Densidad Ósea/fisiología , Sonido , Adolescente , Adulto , Estatura/fisiología , Calcáneo/diagnóstico por imagen , Niño , Preescolar , Femenino , Antebrazo/diagnóstico por imagen , Humanos , Modelos Lineales , Masculino , Rótula/diagnóstico por imagen , Pulgar/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Ultrasonografía
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