Multicenter prospective clinical study to evaluate children short-term neurodevelopmental outcome in congenital heart disease (children NEURO-HEART) : study protocol

Altres ajuts: RETICS funded by the PN 2018-2021 (Spain).Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. While advances in early diagnosis and postnatal management have increased survival in CHD children, worrying long-term outcomes, particularly neurodevelopmental disability, have emerged as a key prognostic factor in the counseling of these pregnancies. Eligible participants are women presenting at 20 to < 37 weeks of gestation carrying a fetus with CHD. Maternal/neonatal recordings are performed at regular intervals, from the fetal period to 24 months of age, and include: placental and fetal hemodynamics, fetal brain magnetic resonance imaging (MRI), functional echocardiography, cerebral oxymetry, electroencephalography and serum neurological and cardiac biomarkers. Neurodevelopmental assessment is planned at 12 months of age using the ages and stages questionnaire (ASQ) and at 24 months of age with the Bayley-III test. Target recruitment is at least 150 cases classified in three groups according to three main severe CHD groups: transposition of great arteries (TGA), Tetralogy of Fallot (TOF) and Left Ventricular Outflow Tract Obstruction (LVOTO). The results of NEURO-HEART study will provide the most comprehensive knowledge until date of children's neurologic prognosis in CHD and will have the potential for developing future clinical decisive tools and improving preventive strategies in CHD. , on 4th December 2016 (retrospectively registered)

Resonancia magnética de la región hipotálamo-hipofisaria en la patología endocrinológica durante la infancia y adolescencia

La presente tesis tiene como propósito evaluar las alteraciones encontradas en las exploraciones de Resonancia Magnética (RM) de la región hipotálamo-hipofisaria realizadas en niños y adolescentes remitidos por sospecha de patología desde el servicio de Endocrinología Pediátrica de nuestro centro terciario durante un período de 10 años (2007-2017). Se han relacionado y clasificado los hallazgos encontrados por RM en las principales entidades patológicas o síndromes clínico-radiológicos (hipopituitarismo, SÍNDROME diencefálico, trastornos del desarrollo puberal, diabetes insípida central, trastornos hipersecretores y hallazgos incidentales). Se evaluaron en total 279 niños y adolescentes, con rango de edad de los pacientes desde los 2 meses hasta los 22 años (media 9.63 años), siendo la distribución por sexos de 56.39% varones y 43.61% hembras. En cuanto a la metodología, se realiza un estudio retrospectivo observacional de las exploraciones de RM realizadas en el departamento de Radiología Pediátrica del Hospital Vall d’Hebron en dicho período, habiendo sido las exploraciones realizadas en tres imanes, dos de 1.5 Teslas –Magnetom y Avanto (Siemens, Erlangen, Germany)- y otro de 3.0 Teslas –Trio (Siemens, Erlangen, Germany). Respecto a los síndromes clínicos que presentaban estos pacientes, un 55% fueron remitidos por hipopituitarismo; 26% por trastornos de la pubertad; 8% presentaban diabetes insípida y un 11%, presentaban trastornos hipersecretores, con sospecha de adenoma pituitario u otro tipo de tumor. Los hallazgos de las exploraciones fueron después correlacionados con los hallazgos clínicos y los parámetros hormonales referidos en la información suministrada por los endocrinólogos pediátricos y consultada en el sistema informático del hospital o en la historia clínica compartida de Catalunya. La exploración revisada fue normal en un 75% de pacientes, mientras que un 25% presentaba alguna anomalía, que se correlacionaba generalmente bien con los síntomas o síndrome clínico que presentaba el paciente. En cada entidad se comentan más profundamente los hallazgos, se aporta la bibliografía actualizada y se presentan los casos más representativos.The purpose of this thesis is to evaluate the alterations found in Magnetic Resonance (MRI) scans of the hypothalamus-pituitary region performed in children and adolescents referred for suspicion of pathology from the Pediatric Endocrinology Unit of our tertiary center during a period of 10 years (2007-2017). The findings found by MRI have been related and classified in the main pathological entities or clinical-radiological syndromes (hypopituitarism, diencephalic syndrome, pubertal development disorders, central diabetes insipidus, hypersecretory disorders and incidental findings). A total of 279 children and adolescents were evaluated, with the age range of the patients from 2 months to 22 years (mean 9.63 years), with a sex distribution of 56.39% males and 43.61% females. Regarding the methodology, a retrospective observational study of the MRI scans performed in the Department of Pediatric Radiology of the Hospital Vall d'Hebron during this period was carried out. Exams were performed on 3 different magnets, two of 1.5 Teslas -Magnetom and Avanto (Siemens, Erlangen, Germany) - and another 3.0 Teslas -Trio (Siemens, Erlangen, Germany). Regarding the clinical syndromes presented by these patients, 55% were referred for hypopituitarism; 26% due to puberty disorders; 8% had diabetes insipidus and 11% had hypersecretory disorders, with suspicion of pituitary adenoma or other type of tumor. The findings of the explorations were later correlated with the clinical findings and the hormonal parameters referred in the information provided by the pediatric endocrinologists and also consulted in our hospital's computer system or in the shared clinical history of Catalonia. The revised examinations were normal in 75% of patients, while 25% had some anomaly, which was generally well correlated with the symptoms or clinical syndrome that the patient presented. In each entity the imaging findings are discussed more deeply, the updated bibliography is provided and the most representative cases are presented for teaching purposes

Resonancia magnética de la región hipotálamo-hipofisaria en la patología endocrinológica durante la infancia y adolescencia

La presente tesis tiene como propósito evaluar las alteraciones encontradas en las exploraciones de Resonancia Magnética (RM) de la región hipotálamo-hipofisaria realizadas en niños y adolescentes remitidos por sospecha de patología desde el servicio de Endocrinología Pediátrica de nuestro centro terciario durante un período de 10 años (2007-2017). Se han relacionado y clasificado los hallazgos encontrados por RM en las principales entidades patológicas o síndromes clínico-radiológicos (hipopituitarismo, SÍNDROME diencefálico, trastornos del desarrollo puberal, diabetes insípida central, trastornos hipersecretores y hallazgos incidentales). Se evaluaron en total 279 niños y adolescentes, con rango de edad de los pacientes desde los 2 meses hasta los 22 años (media 9.63 años), siendo la distribución por sexos de 56.39% varones y 43.61% hembras. En cuanto a la metodología, se realiza un estudio retrospectivo observacional de las exploraciones de RM realizadas en el departamento de Radiología Pediátrica del Hospital Vall d’Hebron en dicho período, habiendo sido las exploraciones realizadas en tres imanes, dos de 1.5 Teslas –Magnetom y Avanto (Siemens, Erlangen, Germany)- y otro de 3.0 Teslas –Trio (Siemens, Erlangen, Germany). Respecto a los síndromes clínicos que presentaban estos pacientes, un 55% fueron remitidos por hipopituitarismo; 26% por trastornos de la pubertad; 8% presentaban diabetes insípida y un 11%, presentaban trastornos hipersecretores, con sospecha de adenoma pituitario u otro tipo de tumor. Los hallazgos de las exploraciones fueron después correlacionados con los hallazgos clínicos y los parámetros hormonales referidos en la información suministrada por los endocrinólogos pediátricos y consultada en el sistema informático del hospital o en la historia clínica compartida de Catalunya. La exploración revisada fue normal en un 75% de pacientes, mientras que un 25% presentaba alguna anomalía, que se correlacionaba generalmente bien con los síntomas o síndrome clínico que presentaba el paciente. En cada entidad se comentan más profundamente los hallazgos, se aporta la bibliografía actualizada y se presentan los casos más representativos.The purpose of this thesis is to evaluate the alterations found in Magnetic Resonance (MRI) scans of the hypothalamus-pituitary region performed in children and adolescents referred for suspicion of pathology from the Pediatric Endocrinology Unit of our tertiary center during a period of 10 years (2007-2017). The findings found by MRI have been related and classified in the main pathological entities or clinical-radiological syndromes (hypopituitarism, diencephalic syndrome, pubertal development disorders, central diabetes insipidus, hypersecretory disorders and incidental findings). A total of 279 children and adolescents were evaluated, with the age range of the patients from 2 months to 22 years (mean 9.63 years), with a sex distribution of 56.39% males and 43.61% females. Regarding the methodology, a retrospective observational study of the MRI scans performed in the Department of Pediatric Radiology of the Hospital Vall d'Hebron during this period was carried out. Exams were performed on 3 different magnets, two of 1.5 Teslas -Magnetom and Avanto (Siemens, Erlangen, Germany) - and another 3.0 Teslas -Trio (Siemens, Erlangen, Germany). Regarding the clinical syndromes presented by these patients, 55% were referred for hypopituitarism; 26% due to puberty disorders; 8% had diabetes insipidus and 11% had hypersecretory disorders, with suspicion of pituitary adenoma or other type of tumor. The findings of the explorations were later correlated with the clinical findings and the hormonal parameters referred in the information provided by the pediatric endocrinologists and also consulted in our hospital's computer system or in the shared clinical history of Catalonia. The revised examinations were normal in 75% of patients, while 25% had some anomaly, which was generally well correlated with the symptoms or clinical syndrome that the patient presented. In each entity the imaging findings are discussed more deeply, the updated bibliography is provided and the most representative cases are presented for teaching purposes

Resonancia magnética de la región hipotálamo-hipofisaria en la patología endocrinológica durante la infancia y adolescencia /

La presente tesis tiene como propósito evaluar las alteraciones encontradas en las exploraciones de Resonancia Magnética (RM) de la región hipotálamo-hipofisaria realizadas en niños y adolescentes remitidos por sospecha de patología desde el servicio de Endocrinología Pediátrica de nuestro centro terciario durante un período de 10 años (2007-2017). Se han relacionado y clasificado los hallazgos encontrados por RM en las principales entidades patológicas o síndromes clínico-radiológicos (hipopituitarismo, SÍNDROME diencefálico, trastornos del desarrollo puberal, diabetes insípida central, trastornos hipersecretores y hallazgos incidentales). Se evaluaron en total 279 niños y adolescentes, con rango de edad de los pacientes desde los 2 meses hasta los 22 años (media 9.63 años), siendo la distribución por sexos de 56.39% varones y 43.61% hembras. En cuanto a la metodología, se realiza un estudio retrospectivo observacional de las exploraciones de RM realizadas en el departamento de Radiología Pediátrica del Hospital Vall d'Hebron en dicho período, habiendo sido las exploraciones realizadas en tres imanes, dos de 1.5 Teslas -Magnetom y Avanto (Siemens, Erlangen, Germany)- y otro de 3.0 Teslas -Trio (Siemens, Erlangen, Germany). Respecto a los síndromes clínicos que presentaban estos pacientes, un 55% fueron remitidos por hipopituitarismo; 26% por trastornos de la pubertad; 8% presentaban diabetes insípida y un 11%, presentaban trastornos hipersecretores, con sospecha de adenoma pituitario u otro tipo de tumor. Los hallazgos de las exploraciones fueron después correlacionados con los hallazgos clínicos y los parámetros hormonales referidos en la información suministrada por los endocrinólogos pediátricos y consultada en el sistema informático del hospital o en la historia clínica compartida de Catalunya. La exploración revisada fue normal en un 75% de pacientes, mientras que un 25% presentaba alguna anomalía, que se correlacionaba generalmente bien con los síntomas o síndrome clínico que presentaba el paciente. En cada entidad se comentan más profundamente los hallazgos, se aporta la bibliografía actualizada y se presentan los casos más representativos.The purpose of this thesis is to evaluate the alterations found in Magnetic Resonance (MRI) scans of the hypothalamus-pituitary region performed in children and adolescents referred for suspicion of pathology from the Pediatric Endocrinology Unit of our tertiary center during a period of 10 years (2007-2017). The findings found by MRI have been related and classified in the main pathological entities or clinical-radiological syndromes (hypopituitarism, diencephalic syndrome, pubertal development disorders, central diabetes insipidus, hypersecretory disorders and incidental findings). A total of 279 children and adolescents were evaluated, with the age range of the patients from 2 months to 22 years (mean 9.63 years), with a sex distribution of 56.39% males and 43.61% females. Regarding the methodology, a retrospective observational study of the MRI scans performed in the Department of Pediatric Radiology of the Hospital Vall d'Hebron during this period was carried out. Exams were performed on 3 different magnets, two of 1.5 Teslas -Magnetom and Avanto (Siemens, Erlangen, Germany) - and another 3.0 Teslas -Trio (Siemens, Erlangen, Germany). Regarding the clinical syndromes presented by these patients, 55% were referred for hypopituitarism; 26% due to puberty disorders; 8% had diabetes insipidus and 11% had hypersecretory disorders, with suspicion of pituitary adenoma or other type of tumor. The findings of the explorations were later correlated with the clinical findings and the hormonal parameters referred in the information provided by the pediatric endocrinologists and also consulted in our hospital's computer system or in the shared clinical history of Catalonia. The revised examinations were normal in 75% of patients, while 25% had some anomaly, which was generally well correlated with the symptoms or clinical syndrome that the patient presented. In each entity the imaging findings are discussed more deeply, the updated bibliography is provided and the most representative cases are presented for teaching purposes

Multicenter prospective clinical study to evaluate children short-term neurodevelopmental outcome in congenital heart disease (children NEURO-HEART) : study protocol

Altres ajuts: RETICS funded by the PN 2018-2021 (Spain).Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns. While advances in early diagnosis and postnatal management have increased survival in CHD children, worrying long-term outcomes, particularly neurodevelopmental disability, have emerged as a key prognostic factor in the counseling of these pregnancies. Eligible participants are women presenting at 20 to < 37 weeks of gestation carrying a fetus with CHD. Maternal/neonatal recordings are performed at regular intervals, from the fetal period to 24 months of age, and include: placental and fetal hemodynamics, fetal brain magnetic resonance imaging (MRI), functional echocardiography, cerebral oxymetry, electroencephalography and serum neurological and cardiac biomarkers. Neurodevelopmental assessment is planned at 12 months of age using the ages and stages questionnaire (ASQ) and at 24 months of age with the Bayley-III test. Target recruitment is at least 150 cases classified in three groups according to three main severe CHD groups: transposition of great arteries (TGA), Tetralogy of Fallot (TOF) and Left Ventricular Outflow Tract Obstruction (LVOTO). The results of NEURO-HEART study will provide the most comprehensive knowledge until date of children's neurologic prognosis in CHD and will have the potential for developing future clinical decisive tools and improving preventive strategies in CHD. , on 4th December 2016 (retrospectively registered)