Enfermedad de Graves con predominio de T3 en la edad pediátrica / T3-predominant Graves' disease in paediatric patients

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Factores de riesgo cardiovascular en niños y adolescentes españoles con diabetes mellitus tipo 1: evolución a lo largo de 9 años / Nine-year longitudinal study of cardiovascular risk factors in spanish children and adolescents with type 1 diabetes

OBJETIVOS: Analizar la prevalencia, evolución de factores de riesgo cardiovascular (FRCV) y su relación con el control metabólico en pacientes pediátricos con diabetes mellitus tipo 1 (DM1). PACIENTES Y MÉTODOS: Estudio longitudinal ambispectivo en 75 niños y adolescentes españoles con DM1 diagnosticados en los años 1996-2003 y seguidos durante 9 años. Analizamos los FRCV y su evolución al segundo, sexto y noveno años tras el diagnóstico, y los antecedentes familiares (AF) de FRCV. RESULTADOS: El 46,6% tenía AF de FRCV. En el segundo, sexto y noveno años encontramos una prevalencia de HbA1c > 7,5% del 45,3, el 53,3 y el 56%, respectivamente; de obesidad (índice de masa corporal > 2 desviaciones estándar) del 5,3, el 5,3 y el 6,7%, y de HTA (presión arterial > p90) del 14,6, el 8 y el 13,3%. Colesterol total > 200 mg/dl en el 25,3, el 13,3 y el 16%; lipoproteína de alta densidad del colesterol (HDL-c) < 40 mg/dl en el 1,3, el 1,3 y el 4%; lipoproteína de baja densidad del colesterol (LDL-c) > 100 mg/dl en el 38,6, el 34,6 y el 38,6%; triglicéridos (TG) > 150 mg/dl en el 0, el 1,3 y el 2,6%, respectivamente. Encontramos un aumento significativo en la prevalencia de TG/HDL-c≥ 2 entre el sexto y el noveno años de evolución de la enfermedad (1,3% y 9,3%, p < 0,05). Una HbA1c≥ 7,5% en el segundo y el sexto años se asoció de forma significativa a una disminución en el HDL-c z-score de 0,94, y una HbA1c < 7,5% durante ese mismo periodo se asoció significativamente a un aumento del HDL-c z-score del 0,55(p = 0,015). CONCLUSIONES: El peor control metabólico de la DM1 en los primeros años de evolución se asocia a una disminución del HDL-c z-score. El cociente TG/HDL-c podría ser un marcador precoz de riesgo cardiovascular

OBJECTIVES: To analyse the prevalence, evolution of cardiovascular risk factors (CVRF) and their relationship with follow-up of metabolic control in pediatric patients with Type 1 Diabetes (T1DM). PATIENTS AND METHODS: Longitudinal ambispective study including 75 children and adolescents with T1DM diagnosed from 1996 to 2003 and followed-up for nine years. Family history of CVRF was registered. Data from the second, sixth and ninth year after diagnosis were analysed. RESULTS: Family history of CVRF was found in 46.6% of the patients. The prevalence of HbA1c > 7.5% in the second, sixth and ninth year after diagnosis was 45.3%, 53.3% y 56%, respectively. The prevalence of obesity (BMI > 2SDS) in the three visits was 5.3%, 5.3% y 6.7%, respectively. Hypertension (BP > p90) was found in 14.6%, 8% and 13.3% of the patients in the three visits, respectively. Total cholesterol > 200 mg/dl: 25.3%, 13.3% and 16%; high density cholesterol lipoprotein < 40mg/dl: 1.3%, 1.3% and 4%; low density cholesterol lipoprotein > 100mg/dl: 38.6%, 34.6% and 38.6%; triglyceride > 150 mg/dl: 0%, 1.3% and 2.6%, respectively. There was a significant increase in the prevalence of TG/HDL-C≥2 between the sixth and the ninth year after diagnosis (1.3% and 9.3%, P < .05). A persistent HbA1c≥7.5% showed a statistically significant relationship to a 0.94 decrease in HDL-C z-score between the second and the sixth year, and a persistent HbA1c < 7.5% was significantly associated with a 0.55 increase in HDL-C z-score (P = .015) in the same period. CONCLUSIONS: A non-optimal metabolic control in first years of DM1 is associated with a decrease in HDL-C z-score. TG/HDL-C ratio could be an early marker of cardiovascular risk

Diabetes mellitus tipo 2 en adolescentes en una unidad de diabetes pediátrica / Type 2 diabetes mellitus in adolescents in a pediatric diabetes unit

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[Nine-year longitudinal study of cardiovascular risk factors in Spanish children and adolescents with type 1 diabetes]. / Factores de riesgo cardiovascular en niños y adolescentes españoles con diabetes mellitus tipo 1: evolución a lo largo de 9 años.

OBJECTIVES: To analyse the prevalence, evolution of cardiovascular risk factors (CVRF) and their relationship with follow-up of metabolic control in pediatric patients with Type 1 Diabetes (T1DM). PATIENTS AND METHODS: Longitudinal ambispective study including 75 children and adolescents with T1DM diagnosed from 1996 to 2003 and followed-up for nine years. Family history of CVRF was registered. Data from the second, sixth and ninth year after diagnosis were analysed. RESULTS: Family history of CVRF was found in 46.6% of the patients. The prevalence of HbA1c>7.5% in the second, sixth and ninth year after diagnosis was 45.3%, 53.3% y 56%, respectively. The prevalence of obesity (BMI>2SDS) in the three visits was 5.3%, 5.3% y 6.7%, respectively. Hypertension (BP>p90) was found in 14.6%, 8% and 13.3% of the patients in the three visits, respectively. Total cholesterol>200mg/dl: 25.3%, 13.3% and 16%; high density cholesterol lipoprotein< 40 mg/dl: 1.3%, 1.3% and 4%; low density cholesterol lipoprotein>100mg/dl: 38.6%, 34.6% and 38.6%; triglyceride>150 mg/dl: 0%, 1.3% and 2.6%, respectively. There was a significant increase in the prevalence of TG/HDL-C ≥ 2 between the sixth and the ninth year after diagnosis (1.3% and 9.3%, P<.05). A persistent HbA1c ≥ 7.5% showed a statistically significant relationship to a 0.94 decrease in HDL-C z-score between the second and the sixth year, and a persistent HbA1c<7.5% was significantly associated with a 0.55 increase in HDL-C z-score (P=.015) in the same period. CONCLUSIONS: A non-optimal metabolic control in first years of DM1 is associated with a decrease in HDL-C z-score. TG/HDL-C ratio could be an early marker of cardiovascular risk.

Evaluación de la transición desde la Unidad de Diabetes Pediátrica a la de Adultos en adolescentes con diabetes mellitus tipo 1 / Evaluation of the transition from pediatric to adult diabetic unit for adolescents with type 1 diabetes

OBJETIVO: Analizar el control metabólico y grado de satisfacción en un grupo de pacientes con DM1 tras cambiar de Unidad (Pediátrica [UDP], Adultos [UDA]). PACIENTES Y MÉTODOS: Estudio retrospectivo: 49 pacientes, 43% mujeres. Analizamos: edad al diagnóstico y paso a adultos, tiempo evolución, control metabólico (HbA1c), complicaciones diabéticas y características del seguimiento. Estudio estadístico: programa SPSS-versión-17.0. RESULTADOS: Edad media ± desviación estándar al diagnóstico 8,3 ± 4,6 y en transferencia 19,2 ± 1,8 años. Tiempo seguimiento en UDP, UDA y global: 10,8 ± 5,0, 4,1 ± 2,6 y 15,0 ± 5,7 años; el 6% perdió el seguimiento médico. En UDA: el 6% desconocía última HbA1c y el 29%las complicaciones crónicas. El control metabólico empeoró en 52% pacientes (aumento HbA1c +0,79 ± 0,70%). No encontramos correlación entre el tiempo evolución y la HbA1c. El 96% de los pacientes refirieron grado satisfacción bueno/muy bueno respecto a UDP y el 74% respecto a UDA. CONCLUSIÓN: Parece necesaria una mejor planificación de la transición de pacientes con DM1 alas UDA para evitar el empeoramiento del control clínico y/o la pérdida de seguimiento

INTRODUCTION: The transition between pediatric and adult care for young people with type 1 diabetes (T1D) is often poorly managed, with adverse consequences for health, as well as a decrease in the follow-up OBJECTIVE: To analyze the metabolic control and the degree of satisfaction in a group of patients with T1D after being transferred from the Pediatric Diabetes Unit (PDU) to Adult Diabetes Unit (ADU). PATIENTS AND METHODS: Retrospective study in a cohort of 49 patients (43% female) with T1D. The age at diagnosis and transfer to ADU, time of onset of the disease, metabolic control (HbA1c), presence of diabetic complications and characteristics of medical follow-up wereanalysed using the statistics program: SPSS, version 17.0. RESULTS: Mean age at diagnosis 8.3±4.6 years and transfer to ADU 19.2±1.8 years. Mean time since onset of T1D in pediatrics, adults and overall: 10.8±5.0, 4.1±2.6 and 15.0±5.7 years, respectively. The 6% of adult patients were not being medically tracked. Among adults, 25% did not provide data about chronic complications, and 6% did not know their last HbA1c. The metabolic control after their transfer to the ADU worsened in 52% of the patients (HbA1c +0.79±0.70%). No correlation was found between the time since onset and the HbA1c value. Degree of satisfaction was either good or very good in 96% of patients in the PDU and 74% in ADU. CONCLUSION: Better planning for the transfer of pediatric patients with T1D to ADU is highly recommended, in order to avoid deterioration of control and/or loss of follow-up

Diabetes latente autoinmune en el joven / Latent autoimmune diabetes in the young

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Eficacia del tratamiento con 131I en la enfermedad de Graves pediátrica / Efficacy of treatment with I 131 in paediatric Graves disease

INTRODUCCIÓN: El tratamiento con radioyodo en la enfermedad de Graves (EG) es una opción terapéutica curativa cada vez más utilizada en niños por encima de 5 años. En Estados Unidos su uso está muy extendido, pero en Europa sigue existiendo controversia respecto a su indicación en la edad pediátrica. OBJETIVO: Presentar nuestra experiencia con la administración de I131 en la EG en edad pediátrica y analizar su eficacia y seguridad. PACIENTES Y MÉTODOS: Estudio retrospectivo descriptivo de los pacientes pediátricos (< 18 años) diagnosticados de EG en nuestro hospital desde 1982 hasta 2012. Al alcanzar la pubertad, se ofreció una opción curativa a aquellos pacientes que no habían respondido al tratamiento con fármacos antitiroideos (AT). Analizamos las características de los pacientes, niveles de hormona tirotropa, T3 y T4, y autoanticuerpos, respuesta a AT, frecuencia de la remisión de la enfermedad post-I131, aparición de hipotiroidismo y efectos secundarios del I131.ResultadosDesde 1982 hasta 2012 fueron diagnosticados de EG 50 pacientes. Todos recibieron como tratamiento inicial AT, con una duración media 35,3 ± 25,9 meses. Se consiguió remisión permanente en el 46%. Se realizó tiroidectomía a 5 pacientes y se administró I131 a 14 pacientes. La dosis de yodo administrada osciló entre 8,5 y 13 mCi (10,9 ± 1,09). Se obtuvo remisión en el 100%. La tasa de hipotiroidismo permanente fue del 90%. No se observaron progresión de la oftalmopatía ni efectos secundarios en ningún paciente tratado con I131. CONCLUSIONES: El tratamiento con 131I en la EG pediátrica es seguro, lleva a la remisión completa a costa de hipotiroidismo y no exacerba la oftalmopatía. Puede considerarse su utilización en mayores de 5 años cuando no existe respuesta a AT o ante efectos secundarios importantes con esta medicación

INTRODUCTION: Radioiodine is an important therapeutic option in young patients with Grave's disease (GD). In the United States it is a widespread therapy, but in Europe its use in paediatrics is still controversial. AIM: To report our experience in radioiodine therapy of paediatric GD patients and analyse its effectiveness and safety. PATIENTS AND METHODS: We retrospectively studied our paediatric population (<18 years of age) with GD, diagnosed from 1982 to 2012. A curative option was offered to patients who did not respond to anti-thyroid drug (AT) at puberty. We analysed, the patient characteristics, TSH, T4, T3 and thyroid antibodies levels, AT response, remission post I131, side effects, and hypothyroidism rates. RESULTS: A total of 50 patients were diagnosed with GD from 1982 to 2012. All patients received AT as initial treatment (mean duration: 35.3±25.9 months). Permanent remission was achieved in 46%. Thyroidectomy was performed in 5 patients, and 14 patients received I131 (mean dose: 10.9±1.09 mCi). Remission with I131 was obtained in 100%. The rate of permanent hypothyroidism was 90%. There was no progression of ophthalmopathy or side effects in any patientst reated with I131.CONCLUSION: Radioiodine treatment of paediatric GD patients is safe, leads to complete remission at the expense of hypothyroidism, and does not exacerbate ophthalmopathy. It can be considered in patients older than 5 years, who do no not respond to AT or with significant side effects with this medication

[Efficacy of treatment with I(131) in paediatric Graves disease]. / Eficacia del tratamiento con (131)I en la enfermedad de Graves pediátrica.

INTRODUCTION: Radioiodine is an important therapeutic option in young patients with Grave's disease (GD). In the United States it is a widespread therapy, but in Europe its use in paediatrics is still controversial. AIM: To report our experience in radioiodine therapy of paediatric GD patients and analyse its effectiveness and safety. PATIENTS AND METHODS: We retrospectively studied our paediatric population (<18 years of age) with GD, diagnosed from 1982 to 2012. A curative option was offered to patients who did not respond to anti-thyroid drug (AT) at puberty. We analysed, the patient characteristics, TSH, T4, T3 and thyroid antibodies levels, AT response, remission post I(131), side effects, and hypothyroidism rates. RESULTS: A total of 50 patients were diagnosed with GD from 1982 to 2012. All patients received AT as initial treatment (mean duration: 35.3±25.9 months). Permanent remission was achieved in 46%. Thyroidectomy was performed in 5 patients, and 14 patients received I(131) (mean dose: 10.9±1.09 mCi). Remission with I(131) was obtained in 100%. The rate of permanent hypothyroidism was 90%. There was no progression of ophthalmopathy or side effects in any patients treated with I(131.) CONCLUSION: Radioiodine treatment of paediatric GD patients is safe, leads to complete remission at the expense of hypothyroidism, and does not exacerbate ophthalmopathy. It can be considered in patients older than 5 years, who do no not respond to AT or with significant side effects with this medication.

[Evaluation of the transition from pediatric to adult diabetic unit for adolescents with type 1 diabetes]. / Evaluación de la transición desde la Unidad de Diabetes Pediátrica a la de Adultos en adolescentes con diabetes mellitus tipo 1.

INTRODUCTION: The transition between pediatric and adult care for young people with type 1 diabetes (T1D) is often poorly managed, with adverse consequences for health, as well as a decrease in the follow-up. OBJECTIVE: To analyze the metabolic control and the degree of satisfaction in a group of patients with T1D after being transferred from the Pediatric Diabetes Unit (PDU) to Adult Diabetes Unit (ADU). PATIENTS AND METHODS: Retrospective study in a cohort of 49 patients (43% female) with T1D. The age at diagnosis and transfer to ADU, time of onset of the disease, metabolic control (HbA1c), presence of diabetic complications and characteristics of medical follow-up were analysed using the statistics program: SPSS, version 17.0. RESULTS: Mean age at diagnosis 8.3±4.6 years and transfer to ADU 19.2±1.8 years. Mean time since onset of T1D in pediatrics, adults and overall: 10.8±5.0, 4.1±2.6 and 15.0±5.7 years, respectively. The 6% of adult patients were not being medically tracked. Among adults, 25% did not provide data about chronic complications, and 6% did not know their last HbA1c. The metabolic control after their transfer to the ADU worsened in 52% of the patients (HbA1c +0.79±0.70%). No correlation was found between the time since onset and the HbA1c value. Degree of satisfaction was either good or very good in 96% of patients in the PDU and 74% in ADU. CONCLUSION: Better planning for the transfer of pediatric patients with T1D to ADU is highly recommended, in order to avoid deterioration of control and/or loss of follow-up.

Hiperostosis cortical neonatal (enfermedad de Caffey) / Neonatal cortical hyperostosis (Caffey disease)

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Influencia étnica en la prevalencia de síndrome metabólico en población pediátrica obesa / Ethnic influence on the prevalence of metabolic syndrome in an obese pediatric population

Introducción: La obesidad en la edad pediátrica es cada vez más prevalente. La población hispana inmigrante tiene un alto riesgo de obesidad y de diabetes tipo 2. Objetivo: Analizar la influencia étnica en la prevalencia del síndrome metabólico (SM) y sus componentes en una población pediátrica obesa. Pacientes y métodos: Estudio retrospectivo de 616 niños y adolescentes obesos ([IMC>2 DE (Hernández 1998-2004)]: 142 hispanos y 474 caucásicos. Se comparan la prevalencia de SM y sus componentes (criterios modificados Cook 2003): obesidad y ≥2 de los siguientes: colesterol HDL<40mg/dl, triglicéridos >110mg/dl, presión arterial sistólica y/o diastólica >p90 (Task Force 2004) y alteración del metabolismo hidrocarbonado (ADA 2011). Se evalúa también: historia familiar de SM, HbA1c, insulinorresistencia (HOMA), función hepática y evolución del IMC al año de tratamiento con cambios en el estilo de vida. Resultados: El 30,5% de los hispanos presentan SM frente al 15,5% de los caucásicos (OR=2,4 [IC del 95%, 1,5-3,8]), p<0,005]) y OR de 2,5 al ajustar por sexo, IMC-DE y pubertad. Los hispanos presentan mayor insulinorresistencia (58,6% vs 42,86%, p<0,005) y peor evolución del IMC al año de seguimiento. No hubo diferencias en el resto de datos analizados. Encontramos una peor adherencia a las medidas de cambio de alimentación y ejercicio físico en el grupo hispano. Conclusiones: La prevalencia de SM es superior en la población pediátrica hispana obesa que en la caucásica. La peor adherencia al tratamiento de los niños y adolescentes obesos hispanos hace que este grupo de pacientes tenga un riesgo potencialmente mayor de enfermedades cardiovasculares en la vida adulta (AU)

Introduction: Obesity in children is becoming more prevalent. Obesity and type 2 diabetes is higher in the Latin American immigrant population. Objective: To analyze the influence of ethnicity on the prevalence of metabolic syndrome (MS) and its components in an obese pediatric population. Patients and methods: A retrospective study of 616 obese children and adolescents (BMI ≥2 SD [Hernández 88-04]), was conducted on 142 Latin American children and 474 Caucasians, which compared the prevalence of metabolic syndrome and its components according to modified Cook criteria (2003): obesity+2 or more of the following components: HDL-cholesterol <40mg/dl, triglycerides >110mg/dl, systolic and/or diastolic blood pressure >p90 (Task Force 2004), and impaired glucose metabolism (ADA 2011). Hepatic function, family history of MS, HbA1c, insulin resistance (HOMA) and BMI evolution at one year of treatment with changes in lifestyle (diet and exercise) were also assessed. Results: Almost one-third (30.5%) of Latin American children had MS compared to 15.5% of Caucasians (OR=2.4 [CI 95%: 1.5-3.8]), P<0.005] and OR=2.5 adjusting for sex, SD-BMI and puberty. Latin American children also had a higher insulin resistance (58.6% vs 42.8%, P<.005) and a worse outcome after one year of treatment. Conclusion: There is a higher prevalence of MS in our Latin American obese pediatric population with poor adherence to the measures of change in lifestyle, making these patients a group with potentially increased risks of cardiovascular disease in adulthood(AU)

[Ethnic influence on the prevalence of metabolic syndrome in an obese pediatric population]. / Influencia étnica en la prevalencia de síndrome metabólico en población pediátrica obesa.

INTRODUCTION: Obesity in children is becoming more prevalent. Obesity and type 2 diabetes is higher in the Latin American immigrant population. OBJECTIVE: To analyze the influence of ethnicity on the prevalence of metabolic syndrome (MS) and its components in an obese pediatric population. PATIENTS AND METHODS: A retrospective study of 616 obese children and adolescents (BMI ≥2 SD [Hernández 88-04]), was conducted on 142 Latin American children and 474 Caucasians, which compared the prevalence of metabolic syndrome and its components according to modified Cook criteria (2003): obesity+2 or more of the following components: HDL-cholesterol <40mg/dl, triglycerides >110mg/dl, systolic and/or diastolic blood pressure >p90 (Task Force 2004), and impaired glucose metabolism (ADA 2011). Hepatic function, family history of MS, HbA1c, insulin resistance (HOMA) and BMI evolution at one year of treatment with changes in lifestyle (diet and exercise) were also assessed. RESULTS: Almost one-third (30.5%) of Latin American children had MS compared to 15.5% of Caucasians (OR=2.4 [CI 95%: 1.5-3.8]), P<.005] and OR=2.5 adjusting for sex, SD-BMI and puberty. Latin American children also had a higher insulin resistance (58.6% vs 42.8%, P<.005) and a worse outcome after one year of treatment. CONCLUSION: There is a higher prevalence of MS in our Latin American obese pediatric population with poor adherence to the measures of change in lifestyle, making these patients a group with potentially increased risks of cardiovascular disease in adulthood.

Alteraciones hidrocarbonadas en pacientes impúberes con fibrosis quística / Abnormal glucose tolerance in prepubertal patients with cystic fibrosis

Introducción: El consenso internacional recomienda el cribado de las alteraciones hidrocarbonadas (AH) en fibrosis quística (FQ) mediante sobrecarga oral de glucosa (SOG) anual desde los 10 años de edad y/o iniciada la pubertad. Objetivos: Evaluar la presencia de AH y su posible repercusión clínica (cambios nutricionales y de función pulmonar) en pacientes impúberes con FQ. Pacientes y métodos: Estudio retrospectivo en 19 pacientes impúberes con FQ (68% varones). Según la SOG, clasificamos (Consenso 2010): tolerancia normal glucosa (TNG) o AH (alteración tolerancia glucosa [ATG], alteración indeterminada glucosa (AIG), diabetes [DRFQ]). Analizamos: SOG (glucemia e insulinemia), estado nutricional (IMC), función pulmonar (espirometría forzada) y función pancreática exocrina. Estudio estadístico con programa SPSS, versión-15.0 mediante pruebas no paramétricas. Resultados: Edad media primera SOG: 8,5 años (5,8-9,8); seguimiento medio: 2 años (2-3). Al inicio: el 53% TNG y el 47% AH; evolutivamente: 4/10 pacientes con TNG desarrollan AH (3ATG, 1DRFQ), 3/4 AIG desarrollan 2ATG y 1DRFQ. Edad media aparición AH: 8,6 años (6,4-11,1). El 69% tuvo deterioro nutricional y/o de la función pulmonar el año previo al diagnóstico de AH. Dos pacientes con AH eran suficientes pancreáticos exocrinos. La insulinemia basal y el área bajo la curva de la SOG fueron comparables entre TNG y AH. El índice insulinogénico fue inferior en AH (p=0,006). Todos los pacientes tuvieron un pico de secreción de insulina retrasado. Conclusiones: La frecuente detección de AH en pacientes impúberes con FQ y su repercusión clínica plantean la necesidad de adelantar su cribado. La suficiencia pancreática exocrina no excluye el desarrollo de AH(AU)

Introduction: Annual screening for abnormal glucose tolerance (AGT) with oral glucose test should begin by age 10 years in cystic fibrosis (CF) patients (Consensus-2010). Aims: To examine the frequency of AGT and its outcome in prepubertal CF patients and the changes in glycemic and nutritional status and lung function over the preceding year. Patients and methods: Retrospective study of 19 prepubertal CF patients (68% males). All subjects underwent an oral glucose tolerance test (OGTT). Results were classified as: normal glucose tolerance (NGT) or AGT (impaired glucose tolerance [IGT], CF related diabetes [CFRD] or indeterminate glucose tolerance [INDET]). We analyzed: OGTT (glucose and insulin levels), nutritional status (BMI-SD) and lung function (forced spirometry). Statistical analysis was performed with SPSS program-version-15.0, non parametric tests. Results: Mean age at first OGGT: 8.5 years (5.8-9.8). Mean follow-up: 2 years (2-3). Initially, 47% patients had AGT and 53% NGT. In follow-up: 4/10 NGT patients developed AGT (3 IGT, 1 CFRD). Among initial AGT patients, of 4 INDET: 2 developed IGT, 1 CFRD. Mean age of AGT onset: 8.6 years (6.4-11.1). In 69% AGT patients a declining BMI-DS and/or lung function was found in the preceding year. In OGTTs performed, fasting and 2h AUC insulin levels were comparable between NGT and AGT; however, insulinogenic index was lower in AGT patients (p=.006). Insulin secretion was delayed in all patients. Conclusions: The high frequency of AGT in prepubertal CF patients and their negative clinical impact supports the usefulness of an earlier glycemic screening(AU)

[Abnormal glucose tolerance in prepubertal patients with cystic fibrosis]. / Alteraciones hidrocarbonadas en pacientes impúberes con fibrosis quística.

INTRODUCTION: Annual screening for abnormal glucose tolerance (AGT) with oral glucose test should begin by age 10 years in cystic fibrosis (CF) patients (Consensus-2010). AIMS: To examine the frequency of AGT and its outcome in prepubertal CF patients and the changes in glycemic and nutritional status and lung function over the preceding year. PATIENTS AND METHODS: Retrospective study of 19 prepubertal CF patients (68% males). All subjects underwent an oral glucose tolerance test (OGTT). Results were classified as: normal glucose tolerance (NGT) or AGT (impaired glucose tolerance [IGT], CF related diabetes [CFRD] or indeterminate glucose tolerance [INDET]). We analyzed: OGTT (glucose and insulin levels), nutritional status (BMI-SD) and lung function (forced spirometry). Statistical analysis was performed with SPSS program-version-15.0, non parametric tests. RESULTS: Mean age at first OGGT: 8.5 years (5.8-9.8). Mean follow-up: 2 years (2-3). Initially, 47% patients had AGT and 53% NGT. In follow-up: 4/10 NGT patients developed AGT (3 IGT, 1 CFRD). Among initial AGT patients, of 4 INDET: 2 developed IGT, 1 CFRD. Mean age of AGT onset: 8.6 years (6.4-11.1). In 69% AGT patients a declining BMI-DS and/or lung function was found in the preceding year. In OGTTs performed, fasting and 2h AUC insulin levels were comparable between NGT and AGT; however, insulinogenic index was lower in AGT patients (p=.006). Insulin secretion was delayed in all patients. CONCLUSIONS: The high frequency of AGT in prepubertal CF patients and their negative clinical impact supports the usefulness of an earlier glycemic screening.

Infundíbulo-neurohipofisitis: evolución a 10 años de un caso en la edad pediátrica / Infundibulo-hypophysitis in a girl: 10 years follow-up

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