[Study of bone mass in patients with growth hormone deficiency]. / Estudio de la masa ósea en el déficit de hormona de crecimiento.

OBJECTIVES: To evaluate bone mineral density by radiogrametric study of metacarpal bone diameter and cortical thickness in patients with growth hormone deficiency (GHD) before and during treatment with growth hormone (GH). PATIENTS AND METHODS: We studied 92 children with GHD (60 boys and 32 girls) divided into two groups: group I: 66 previously untreated patients (42 boys and 24 girls) aged between 3 and 14 years old; group II: 66 patients (42 girls and 24 boys) treated with GH and with a mean age of 10.2 +/- 3.1 years at treatment onset. Bone mass was studied indirectly by radiogrametry; the bone diameter and cortical thickness of the 2nd-3rd and 4th metacarpal bones were measured with a magnifying glass. As reference standards we used the Spanish longitudinal growth and development study (Andrea Prader Center, Zaragoza) in children aged between 0.5 and 9 years and the Swiss longitudinal standards in children aged 10 years of age and older. Statistical significance was set at p < 0.05. RESULTS: Group I (spontaneous evolution): cortical thickness values were below the mean with statistically significant differences al 11, 12 and 13 years of age in girls and at 12, 13 and 14 years in boys. Bone diameter was diminished compared with controls in all the study periods and was significantly reduced at 8, 9, 10 and 11 years of age in girls and at 8, 10, 11, 12, 13 and 14 years in boys. Group II: (effect of GH treatment): cortical regression analysis showed a sharp increase in the first year of treatment with a subsequent moderate increase, which was statistically significant. Bone diameter showed a similar pattern with a significant increase which was more pronounced in the first period. CONCLUSIONS: Children with GHD have decreased bone mass before initiation of treatment and therefore show deficient acquisition of peak bone mass, which in normal conditions occurs during in the first 4-5 years of life and during adolescence. GH replacement therapy leads to recovery of bone mass, which is more pronounced in the first year of treatment and prevents the progressive reduction that appears in untreated patients. Therefore, GH treatment plays an important role in peak bone mass acquisition in children with GHD.

Estudio de la masa ósea en el déficit de hormona del crecimiento / Study of bone mass in patients with growth hormone deficiency

Objetivos. Evaluar la masa ósea con la medida de la cortical y el diámetro metacarpianos en pacientes con déficit de hormona de crecimiento (GH), antes y durante el tratamiento con GH. Pacientes y métodos. Se ha estudiado una población de 92 niños (60 varones, 32 mujeres) con déficit de GH, distribuidas en los siguientes grupos: grupo I, 66 pacientes (42 varones, 24 mujeres) que no habían recibido previamente tratamiento entre 3-14 años de edad; grupo II, 66 pacientes (42 varones, 24 mujeres) tratadas con GH, edad de inicio del tratamiento 10,2 6 3,1 años. La masa ósea se evaluó indirectamente por radiogrametría midiendo en la radiografía de la mano la cortical y el diámetro de 3 metacarpianos con una lupa de aumento. Como estándar de referencia se tomaron los propios españoles del estudio longitudinal del Centro Andrea Prader entre los 0,5 y los 9 años y los suizos de 10 años en adelante. La significación estadística p < 0,05. Resultados. Grupo I (evolución espontánea): la cortical evoluciona por debajo de la media y está disminuida significativamente a los 11, 12 y 13 años en las chicas y 12, 13 y 14 años en los chicos. El diámetro se encuentra disminuido en relación a los controles durante todo el período de observación y está disminuido significativamente a los 8, 9, 10 y 11 años en las chicas y a los 8, 10, 11, 12, 13 y 14 años en los chicos. Grupo II (impacto del tratamiento con GH): la cortical en su ecuación de regresión muestra un incremento brusco durante el primer año de tratamiento y a partir de allí mantiene un crecimiento moderado; estadísticamente es significativo. El diámetro sigue una evolución similar, aumenta de manera significativa de forma más marcada durante el primer período. Conclusiones. Los niños con déficit de GH, presentan un descenso de la masa ósea antes de iniciar el tratamiento, y por lo tanto, una adquisición deficiente del pico de masa ósea, que ocurre normalmente durante los primeros 4 y 5 años y durante la adolescencia. El tratamiento sustitutivo con GH provoca una recuperación de la masa ósea, más intensa durante el primer año de tratamiento y evita el deterioro progresivo que se aprecia en los pacientes no tratados y así desempeña un papel importante en la adquisición del pico de masa ósea en los niños con déficit de GH

Objectives. To evaluate bone mineral density by radiogrametric study of metacarpal bone diameter and cortical thickness in patients with growth hormone deficiency (GHD) before and during treatment with growth hormone (GH). Patients and methods. We studied 92 children with GHD (60 boys and 32 girls) divided into two groups: group I: 66 previously untreated patients (42 boys and 24 girls) aged between 3 and 14 years old; group II: 66 patients (42 girls and 24 boys) treated with GH and with a mean age of 10.2 6 3.1 years at treatment onset. Bone mass was studied indirectly by radiogrametry; the bone diameter and cortical thickness of the 2nd-3rd and 4th metacarpal bones were measured with a magnifying glass. As reference standards we used the Spanish longitudinal growth and development study (Andrea Prader Center, Zaragoza) in children aged between 0.5 and 9 years and the Swiss longitudinal standards in children aged 10 years of age and older. Statistical significance was set at p < 0.05. Results. Group I (spontaneous evolution): cortical thickness values were below the mean with statistically significant differences al 11, 12 and 13 years of age in girls and at 12, 13 and 14 years in boys. Bone diameter was diminished compared with controls in all the study periods and was significantly reduced at 8, 9, 10 and 11 years of age in girls and at 8, 10, 11, 12, 13 and 14 years in boys. Group II: (effect of GH treatment): cortical regression analysis showed a sharp increase in the first year of treatment with a subsequent moderate increase, which was statistically significant. Bone diameter showed a similar pattern with a significant increase which was more pronounced in the first period. Conclusions. Children with GHD have decreased bone mass before initiation of treatment and therefore show deficient acquisition of peak bone mass, which in normal conditions occurs during in the first 4-5 years of life and during adolescence. GH replacement therapy leads to recovery of bone mass, which is more pronounced in the first year of treatment and prevents the progressive reduction that appears in untreated patients. Therefore, GH treatment plays an important role in peak bone mass acquisition in children with GHD

[Study of bone mass in Turner syndrome]. / Estudio de la masa ósea en el síndrome de Turner.

OBJECTIVES: To evaluate bone mass in patients with Turner syndrome by measuring metacarpal cortical thickness and bone diameter before and after treatment with oxandrolone, growth hormone (GH) and estrogens. PATIENTS AND METHODS: We studied 42 girls with Turner syndrome divided into the following groups: group I: 31 patients aged between 3 and 15 years who were not treated before the study; group II: 15 patients treated with GH at start ages of between 5.2-14.8 years; group III: 17 patients treated with oxandrolone at start ages of between 5.3 and 15.2 years; group IV: 17 patients treated with estrogens and divided in different subgroups: IVa: seven patients treated with GH and estrogens at start ages of between 6.1 and 12.9 years; IVb: five patients treated with oxandrolone and estrogens at start ages of between 13.4 and 17.4 years, and IVc: five patients treated with oxandrolone, GH and estrogens at start ages of between 10.3 and 16.1 years. Bone mass was evaluated by a radiogrammetric method that measures the cortical thickness and bone diameter of three metacarpal bones with a magnifying glass. The results are expressed in SD according to Spanish longitudinal reference standards (Andrea Prader Center of Growth and Development) from 0.5 to 9 years of age and to Swiss standards from the age of 10 years onwards. Statistical significance was set at p < 0.05. RESULTS: Group I (spontaneous development): cortical development was below the mean and was significantly diminished at the ages of 9, 13 and 14 years; bone diameter was decreased in relation to controls throughout the study period; group II (impact of GH treatment): cortical thickness showed a nonsignificant increase of 0.6 SD from baseline to years 3-4 of treatment and diameter increased by 0.5 SD from baseline to year 4 of treatment; group III (impact of oxandrolone): cortical thickness increased from -0.8 SD before treatment to 0.0 SD at years 2 and 3 of treatment; bone diameter increased from -1.5 SD at baseline to -1 SD at 3 years of treatment; group IV (impact of treatment with estrogens); IVa: cortical thickness and bone diameter increased; IVb: cortical thickness increased but bone diameter was unchanged; IVc: both cortical thickness and bone diameter increased. CONCLUSIONS: The results of this study show that cortical thickness and bone diameter are decreased in untreated girls with Turner syndrome; cortical thickness was significantly decreased at the ages of 9, 13 and 14 years, while bone diameter was diminished at all ages, suggesting the presence of osteopenia in these patients. GH treatment produced a nonsignificant increase in cortical thickness and bone diameter. Oxandrolone treatment showed a positive effect on bone mass during the first few years of therapy. Because of the small number of patients, conclusions cannot be reached on the effectiveness of estrogens.

Estudio de la masa ósea en el síndrome de Turner / Study of bone mass in Turner syndrome

Los cuidados paliativos son esenciales en las unidades de cuidados intensivos pediátricos (UCIP). Dada la frecuencia de la muerte en las UCIP y la presencia de condiciones médicas que amenazan la vida del niño mientras este está ingresado, existe una necesidad de que el pediatra esté preparado para proporcionar cuidados paliativos, con independencia de los tratamientos curativos. En este artículo se revisan algunos temas, como el proceso de toma de decisiones en la UCIP, las necesidades psicosociales del personal sanitario y la vulnerabilidad al burnout, y los sentimientos y actitudes del personal sanitario ante la muerte del niño. Se proporcionan recomendaciones sobre cómo actuar cuando un niño muere, para concertar reuniones post mortem con los padres y para llevar a cabo el seguimiento del duelo, y se sugieren estrategias que pueden ayudar a afrontar las múltiples pérdidas que experimenta el pediatra de la UCIP

Palliative care is essential in the pediatric intensive care unit (PICU). Because of the mortality rates and the presence of life-threatening conditions in children admitted to the PICU, pediatricians must be prepared to provide palliative care independently of cure-directed therapies. The present article reviews certain issues, including the decision- making process in the PICU, psychosocial needs and susceptibility to burnout among PICU staff, and the emotions and attitudes of the staff when a child dies. We provide some guidelines on how to act when a child dies, how to meet with parents after the child’s death and how to follow- up parental bereavement. Strategies that can help PICU pediatricians to cope with the numerous loses they experience are suggested

[Marshall-Smith syndrome. Apropos of a personal case]. / El síndrome de Marshall-Smith. A propósito de una observación personal.

The first familial observation of the Marshall-Smith syndrome is showed. The propositi are a male and female relatives with a double consanguinity and whose family comes from a small area with a high rate of inbreeding. According to this hypothesis autosomal recessive inheritance for this syndrome is proposed as very suggestive. These two new cases seem to prove that the mental retardation may not be a typical clinic features and that survival of the cases longer that expected may be considered. One of our patients is a three years old female and the other one is nearly eleven years old male. The syndrome delineation is given by very closely homogeneous patterns. Said patterns also single it out from the Weaver syndrome.