[Neonatal hemochromatosis: Another entity that is no longer orphan. Advances in the diagnosis and management of the main cause of neonatal acute liver failure]. / Hemocromatosis neonatal: otra entidad que deja de ser huérfana. Avances en el diagnóstico y manejo de la principal causa de fallo hepático agudo neonatal.

Neonatal hemochromatosis is the most common cause of acute liver failure in the neonatal period. It is associated with high morbidity and mortality due to iron overload in hepatic and extra-hepatic tissues. New evidence has emerged during the last few years as regards its alloimmune etiology, which have had an important repercussion on the diagnosis, treatment and prognosis of these patients. Treatment with immunoglobulins and exchange transfusions has radically changed the prognosis without liver transplant. Another great success has been the preventive use of immunoglobulin in pregnant women with a past history of neonatal hemochromatosis, thus decreasing the rate of disease recurrence up to 70%. This new paradigm has led to an entity with a poor prognosis becoming a curable disease if diagnosed and treated early. Nevertheless, a large widespread ignorance of the disease persists, with medical implications that result in significant health problems, due to the delayed referral of these patients to specialized centers.

Anticuerpos antimúsculo liso sin hepatitis autoinmune / Positive smooth muscle antibody without autoimmune hepatitis

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Eficacia, tolerancia y seguridad a largo plazo del polietilenglicol 3350 con electrolitos en el tratamiento del estreñimiento funcional en niños / Efficacy, tolerance and safety of polyethylene glycol 3350 plus electrolytes for the treatment of functional constipation in children

OBJETIVO: Evaluar la seguridad renal del tratamiento con polietilenglicol 3350 con electrolitos durante 1, 3 y 6 meses, la tolerancia digestiva y la dosis de eficacia. PACIENTES Y MÉTODOS: Fueron evaluados 3 grupos de 30 pacientes sanos, 2-10 años (media 6,2) con estreñimiento funcional (criterios de Roma III), con 1, 3 y 6 meses de tratamiento. La eficacia fue evaluada por el número de deposiciones/semana y la consistencia de las heces (escala de Bristol). La natriuria y la osmolaridad urinaria se midieron al inicio, 1, 3 y 6 meses. Los principios inmediatos en heces (análisis de reflexión de infrarrojos [FENIR]) y un test de hidrógeno espirado fueron efectuados en el grupo de un mes de tratamiento. RESULTADOS: La dosis efectiva fue de 0,37 g/kg/día (rango 0,18-0,8). El número de deposiciones/semana en la inclusión (2,4 ± 0,64) muestra diferencia significativa (p < 0,001) vs. (6,21 ± 1,5) tras el tratamiento. También se demostró una diferencia significativa en la puntuación en la escala de Bristol (1,9 ± 0,75 vs. 4,9 ± 1,1 [p < 0,001]). La ingesta media de sodio fue de 112 mg (5 mg/kg/día [rango de 4-12 mg/kg/día]). Los valores de sodio y osmolaridad en orina fueron normales en todos los grupos sin diferencia estadística con respecto a controles (90 niños sanos sin tratamiento). Los valores de FENIR fueron normales en todos los pacientes. La prueba de aliento con hidrógeno fue normal, con una media de 7 ppm. CONCLUSIÓN: No se observaron parámetros bioquímicos renales adversos ni alteraciones digestivas. La tolerancia y la eficacia demostraron ser óptimas. El polietilenglicol 3350 con electrolitos puede ser recomendado con seguridad para el tratamiento del estreñimiento funcional en los niños a corto y largo plazo

OBJECTIVE: To assess the renal safety of treatment with polyethylene glycol 3350 with electrolytes at 1, 3 and 6 months, its gastrointestinal tolerance and dose effectiveness. PATIENTS AND METHODS: Three groups of 30 healthy patient aged 2-10 years (mean 6.2 years) who suffered functional constipation (Rome III criteria) with 1, 3 and 6 months of treatment were evaluated. Efficacy was evaluated by the change in the number of stools per week and stool consistency (Bristol scale). Urine screens, sodium and osmolality, were performed at the beginning and after 1, 3 and 6 months of treatment. Stool sample NIRA (near-infrared reflectance analysis) and hydrogen breath test analysis samples were performed on the one-month treatment group. RESULTS: The mean dose was 0.37 g/kg/day (range 0.18 to 0.8) titrated according to age, weigh tand response. The number of stools per week during treatment (2.4±0.64) showed a significant difference (P<0.001) vs (6.21±1.5) after treatment. There was also a significant difference in the Bristol scale score (1.9±0.75 vs 4.9±1.1 [P<0.001]). The mean sodium intake was 112 mg (5 mg/kg/day [range 4-12 mg/kg/day]). The values of sodium and urine osmolality were normal in all groups with no statistical difference compared to normal control values (90 healthy children without treatment). NIRA values were normal in all patients. The hydrogen breath test was normal with a median of 7 ppm. CONCLUSION: There were no adverse renal biochemical parameters or gastrointestinal disorders. Tolerance and efficacy was shown to be optimal. Polyethylene glycol 3350 with electrolytes can be safely recommended for the treatment of functional constipation in children in the short and long term

[Efficacy, tolerance and safety of polyethylene glycol 3350 plus electrolytes for the treatment of functional constipation in children]. / Eficacia, tolerancia y seguridad a largo plazo del polietilenglicol 3350 con electrolitos en el tratamiento del estreñimiento funcional en niños.

OBJECTIVE: To assess the renal safety of treatment with polyethylene glycol 3350 with electrolytes at 1, 3 and 6 months, its gastrointestinal tolerance and dose effectiveness. PATIENTS AND METHODS: Three groups of 30 healthy patient aged 2-10 years (mean 6.2 years) who suffered functional constipation (Rome III criteria) with 1, 3 and 6 months of treatment were evaluated. Efficacy was evaluated by the change in the number of stools per week and stool consistency (Bristol scale). Urine screens, sodium and osmolality, were performed at the beginning and after 1, 3 and 6 months of treatment. Stool sample NIRA (near-infrared reflectance analysis) and hydrogen breath test analysis samples were performed on the one-month treatment group. RESULTS: The mean dose was 0.37g/kg/day (range 0.18 to 0.8) titrated according to age, weight and response. The number of stools per week during treatment (2.4±0.64) showed a significant difference (P<.001) vs (6.21±1.5) after treatment. There was also a significant difference in the Bristol scale score (1.9±0.75 vs 4.9±1.1 [P<.001]). The mean sodium intake was 112mg (5mg/kg/day [range 4-12mg/kg/day]). The values of sodium and urine osmolality were normal in all groups with no statistical difference compared to normal control values (90 healthy children without treatment). NIRA values were normal in all patients. The hydrogen breath test was normal with a median of 7ppm. CONCLUSION: There were no adverse renal biochemical parameters or gastrointestinal disorders. Tolerance and efficacy was shown to be optimal. Polyethylene glycol 3350 with electrolytes can be safely recommended for the treatment of functional constipation in children in the short and long term.