Hemofilia B, un sangrado atípicamente abundante durante una prueba rutinaria de un neonato / Hemophilia B, an atypically heavy bleeding during a routine test of a newborn

La hemofilia B es un trastorno hematológico producido por el déficit del factor IX. Aunque generalmente se diagnostica cuando el lactante inicia el gateo o deambulación en forma de hemartros o hemorragias musculares, en este caso el debut es a los 3 días de vida, con un sangrado abundante atípico durante una prueba de rutina. La exploración y pruebas complementarias (hemograma, coagulación, ecografías de partes blandas y grandes articulaciones) no revelaron datos de gravedad. El factor IX, que determina la gravedad, pasó del 1% a un 3% en 24 horas por lo que no precisó de tratamiento (AU)

Hemophilia B is a blood disorder caused by a deficiency of factor IX. Although it is usually diagnosed when the infant starts crawling or walking through the detection of hemarthrosis or muscle bleeds, in this case the onset occurred at 3 days of age with detection of heavy atypical bleeding during a routine test. The examination and additional testing (blood count, coagulation, ultrasound of soft tissue and large joints) did not evince severe disease. Factor IX, which determines the severity, increased from 1% to 3% in 24 hours, so treatment was not required. (AU)

Nutrición y trastorno por déficit de atención/hiperactividad: seguimiento evolutivo de las variables antropométricas de un grupo de pacientes en tratamiento con metilfenidato de liberación osmótica / Nutrition and attention deficit hyperactivity disorder: developmental follow-up of the anthropometric variables of a group of patients receiving treatment with osmotic controlled-release methylphenidate

Objetivo. Realizar un análisis evolutivo de las variables antropométricas de un grupo de pacientes diagnosticados de trastorno por déficit de atención/hiperactividad (TDAH) para determinar la repercusión del tratamiento con metilfenidato de liberación osmótica (MTF-OROS). Pacientes y métodos. Se han revisado retrospectivamente las historias clínicas de 187 pacientes con TDAH en tratamiento con MTF-OROS durante 30 meses, registrándose los pesos y tallas e índice de masa corporal al diagnóstico (basal) y a los 6, 12, 18, 24 y 30 meses de seguimiento. Resultados. La edad media al diagnóstico era de 8,14 ± 1,6 años. La dosis de MTF-OROS fue incrementándose progresivamente hasta 36,9 ± 12,1 mg/día (1,05 mg/kg/día) a los 30 meses del seguimiento. Al diagnóstico, el 34,9% de los pacientes tenía una situación nutricional deficiente (subnutrición o malnutrición), que a los 30 meses de tratamiento afectaba al 50,3% de los pacientes. El valor basal del peso (Z-score) disminuía progresivamente durante el tratamiento, llegando a alcanzar unos valores significativamente inferiores (p < 0,05) respecto al valor basal a los 12 meses, y se mantenía significativamente inferior hasta los 30 meses. El valor basal de la talla (Z-score) también disminuía progresivamente durante el tratamiento, llegando a alcanzar unos valores significativamente inferiores (p < 0,05) respecto al valor basal a los 24 y 30 meses. Conclusiones. En el momento del diagnóstico de TDAH, uno de cada tres pacientes se hallaba en una situación nutricional deficiente (subnutrición o malnutrición). El tratamiento continuado con MTF-OROS durante 30 meses ejerce una influencia negativa sobre la talla, que posiblemente podría atenuarse mejorando la nutrición de los pacientes (AU)

Aim. To perform a developmental analysis of the anthropometric variables of a group of patients diagnosed with attention deficit hyperactivity disorder (ADHD) in order to determine the repercussions of treatment with osmotic controlled-release methylphenidate (MTF-OROS). Patients and methods. The medical records of 187 patients with ADHD under treatment with MTF-OROS over a period of 30 months were reviewed. Data collected included weight, height and body mass index at diagnosis (baseline) and at 6, 12, 18, 24 and 30 months’ follow-up. Results. The mean age at diagnosis was 8.14 ± 1.6 years. The dose of MTF-OROS was progressively increased until 36.9 ± 12.1 mg/day (1.05 mg/kg/day) at day 30 of the follow-up. At diagnosis, 34.9% of patients had a deficient nutritional situation (subnutrition or malnutrition), which affected 50.3% of the patients at 30 months. The baseline value for weight (Z-score) progressively decreased during treatment until values that were significantly lower than the baseline value at 12 months were reached (p < 0.05); these values remained significantly lower until 30 months. The baseline value for height (Z-score) also progressively decreased during treatment until values that were significantly lower than the baseline value at 24 and 30 months were reached (p < 0.05). Conclusions. At the time they were diagnosed with ADHD, one out of every three patients was in a deficient nutritional situation (subnutrition or malnutrition). Continued treatment with MTF-OROS for 30 months had a negative influence on height, which could perhaps be attenuated by improving the patients’ nutrition (AU)

[Nutrition and attention deficit hyperactivity disorder: developmental follow-up of the anthropometric variables of a group of patients receiving treatment with osmotic controlled-release methylphenidate]. / Nutricion y trastorno por deficit de atencion/hiperactividad: seguimiento evolutivo de las variables antropometricas de un grupo de pacientes en tratamiento con metilfenidato de liberacion osmotica.

AIM: To perform a developmental analysis of the anthropometric variables of a group of patients diagnosed with attention deficit hyperactivity disorder (ADHD) in order to determine the repercussions of treatment with osmotic controlled-release methylphenidate (MTF-OROS). PATIENTS AND METHODS: The medical records of 187 patients with ADHD under treatment with MTF-OROS over a period of 30 months were reviewed. Data collected included weight, height and body mass index at diagnosis (baseline) and at 6, 12, 18, 24 and 30 months' follow-up. RESULTS: The mean age at diagnosis was 8.14 ± 1.6 years. The dose of MTF-OROS was progressively increased until 36.9 ± 12.1 mg/day (1.05 mg/kg/day) at day 30 of the follow-up. At diagnosis, 34.9% of patients had a deficient nutritional situation (subnutrition or malnutrition), which affected 50.3% of the patients at 30 months. The baseline value for weight (Z-score) progressively decreased during treatment until values that were significantly lower than the baseline value at 12 months were reached (p < 0.05); these values remained significantly lower until 30 months. The baseline value for height (Z-score) also progressively decreased during treatment until values that were significantly lower than the baseline value at 24 and 30 months were reached (p < 0.05). CONCLUSIONS: At the time they were diagnosed with ADHD, one out of every three patients was in a deficient nutritional situation (subnutrition or malnutrition). Continued treatment with MTF-OROS for 30 months had a negative influence on height, which could perhaps be attenuated by improving the patients' nutrition.