J. Blasco Alonso

Colitis alérgica en lactantes exclusivamente amamantados al pecho

Introduccion La proctocolitis eosinofilica esta inducida por antigenos presentes en las proteinas de leche de vaca contenidas en las formulas o en la leche humana y, en los ultimos anos, se describen cada vez mas casos en lactantes pequenos alimentados exclusivamente con la leche materna. Pacientes y metodos Se presenta un estudio retrospectivo de los 13 casos de colitis alergica diagnosticados en nuestra unidad entre enero de 1997 y enero de 2004, todos ellos lactantes que estaban recibiendo exclusivamente leche materna. Resultados La sintomatologia de inicio fue siempre la digestive (12 debutaron con heces con moco y hebras de sangre) y en el 77 % de los casos aparecio entre los 0 y los 3 meses de edad. Los datos analiticos del componente alergico fueron negativos. Las lesiones se localizaron, principalmente (75 %), en las zonas descendente y sigma del colon. La anatomia patologica demostro datos inflamatorios agudos, con infiltracion por polimorfonucleares y aumento de eosinofilos. En todos los pacientes se inicio tratamiento excluyendo la leche de vaca y sus derivados en la dieta de la madre. En 10/13 no se evidencio mejoria, por lo que requirieron administracion exclusiva de hidrolizado extenso de proteinas. En 3 ninos se mantuvo lactancia mixta (sin proteinas vacunas + hidrolizado). Conclusiones El diagnostico exige exclusion de otras causas de colitis especifica y hallazgos endoscopicos y anatomopatologicos caracteristicos; ademas, se debe demostrar respuesta adecuada al tratamiento dietetico. Se debe pensar en esta patologia al afrontar el diagnostico de lactantes amamantados que presenten sangrado rectal sin afectacion del crecimiento o del estado general.

Oral tacrolimus for pediatric steroid-resistant ulcerative colitis

BACKGROUND Ulcerative colitis (UC) occurring during childhood is generally extensive and is associated with severe flares that may require intravenous steroid treatment. In cases of corticosteroid resistance is necessary to introduce a second-line treatment to avoid or delay surgery. AIMS To describe the efficacy and safety of oral tacrolimus for the treatment of severe steroid-resistant UC. METHODS We performed a retrospective study that included all patients under age 18 suffering from severe steroid-resistant UC treated with oral tacrolimus during the period January 1998 to October 2012 and with a follow-up period after treatment of 24 months or more. RESULTS A total of ten patients were included. The age at baseline was 9.4±4.9 years, and the time from diagnosis was 1.3 months (IQR, 1-5.7). Seven of the patients were in their first flare of disease. All of them received an oral dose of 0.12 mg/kg/day of tacrolimus divided in two doses. Trough plasma levels of tacrolimus were maintained between 4 and 13 ng/ml. Response was seen in 5/10 patients at 12 months, colectomy was eventually performed in 60% of patients during the follow-up period. CONCLUSIONS Tacrolimus is useful in inducing remission in patients with severe steroid-resistant UC, preventing or delaying colectomy, and allowing the patient and family to prepare for a probable surgery. Tacrolimus may also be used as a treatment bridge for corticosteroid-dependent patients until the new maintenance therapy takes effect.

Ahogamientos y casi ahogamientos en niños

Objetivo Los accidentes por sumersion siguen causando importante morbimortalidad en ninos. Se realiza un estudio retrospective para identificar los factores pronosticos asociados a la evolucion clinica de ninos con accidente por sumergimiento, que puedan servir de ayuda para tomar decisiones terapeuticas, comparandolos con otros datos publicados. Pacientes y metodos Estudio retrospectivo mediante revision de las historias clinicas de los casos atendidos por accidente por sumergimiento entre enero de 1995 y abril de 2003. Los datos analizados se refieren al paciente, el accidente, situacion clinica en Urgencias y unidades donde ingresan en el hospital, valorando la evolucion final de cada caso. Resultados Se han incluido 62 pacientes, presentando mala evolucion 12 (7 fallecimientos y 5 secuelas permanentes). Han resultado significativos, como datos de mal pronostico,edad igual o superior a 4 anos, sexo femenino, tiempo inmersion 5 min o mas y, a la llegada al hospital: cianosis inicial que persistia en Urgencias; parada cardiaca, apnea o distres grave, hipotermia (temperatura Conclusiones La evolucion final del paciente esta estrechamente relacionada con la situacion clinica a su llegada al hospital.Aunque disponemos de datos orientativos sobre la evolucion posterior del paciente casi ahogado, se carece de modelos predictivos precoces que permitan vaticinar la evolucion del paciente y, por lo tanto, orientar en la actitud a tomar en las medidas iniciales de reanimacion y mantenimiento posterior.

Eficacia y seguridad de tacrolimus oral para el tratamiento de la enfermedad inflamatoria intestinal pediátrica

BACKGROUND In certain clinical situations, such as acute and severe episodes of ulcerative colitis (UC) or Crohns Disease (CD), that do not respond to conventional intravenous steroid treatment, we need potent, fast-acting drugs to induce clinical remission and avoid surgery. OBJECTIVES To evaluate the efficacy and safety of oral tacrolimus treatment of acute and severe UC or CD to induce their remission, and also to assess its efficacy in delaying or avoiding surgery. MATERIAL AND METHODS We present a retrospective study that included all patients under 18 years of age with acute and severe bouts of CD (colonic or ileocolonic location) or UC who were treated with oral tacrolimus at our institution from January 1998 to December 2007. RESULTS We included a total of 8 patients (4 males and 4 females), 6 presented with UC and 2 had CD. The mean age of our patients at the start of the treatment was 11.8 years (range 2.75-16.58 y) and the mean time from diagnosis to the start of tacrolimus therapy was 4 months (range 1-96 m). An initial response was obtained in 50% of patients. Plasma trough levels of tacrolimus remained between 5-11 ng/ml. Six of the eight patients (75%) required surgery. In one patient with UC and in another with CD, surgery was avoided. In 2 of the 6 patients with UC, surgery was postponed beyond 6 months. CONCLUSIONS Tacrolimus is useful in inducing clinical remission in patients with acute and severe bouts of UC or CD, and so can avoid or delay the surgery; it may also be used as a bridging agent until the new maintenance therapy with other immunosuppressants is effective.

Plasma citrulline as a marker of loss of enterocitary mass in coeliac disease in childhood

INTRODUCTION Plasma citrulline is not incorporated in endogenous or exogenous proteins so it is a theoretical marker of villous atrophy. Our aim was to correlate plasma citrulline levels with severity of villous atrophy in celiac patients. METHODS Observational case-control study longitudinal in children 16 month-old to 14 year-old: 48 with untreated celiac disease, 9 celiac children under gluten free diet and 35 non-celiac healthy children. Plasma amino acids concentration is determined, expressed in μmol/L, and so are other clinical and analytical data. RESULTS No statistically significative difference found in the referring to BMI, age or renal function. Small increase in fecal fat in celiac children. Citrulline, arginine and glutamine are significantly lower in cases (17.7 μmol/l, 38.7 μmol/l, 479.6 μmol/l respectively) than in controls (28.9 μmol/l, 56.2 μmol/l, 563.7 μmol/l). Citrulline levels are significantly lower in the severe degrees of atrophy than in mild ones (13.8 μmol/l vs. 19.7 μmol/l, p < 0.05), not happening so with rest of amminoacids. SUMMARY Postabsortive mean of plasma citrulline is a good marker of reduction in enterocyte mass in celiac patients with villous atrophy; secondary reduction in plasma arginine too. Just a small histological alteration in intestinal biopsy is enough to differentiate citrulline in cases and controls and besides it can be seen that high levels of atrophy present with lower plasma citrulline.

Disminución de la densidad mineral ósea y otros factores de riesgo en niños prepuberales con fractura del antebrazo distal

AIM To investigate the relationship between bone mineral density (BMD), age, sex, anthropometric measurements, dietary calcium intake and daily physical activity, in prepubertal children with distal forearm fracture (DFF). METHODS 160 children (80 males, 80 females) 3-10 years of age with DFF; control group of 160 children (age-sex matched) were studied. Age, sex, weight, height, tricipital skin fold thickness (TS), body mass index, daily calcium intake, and level of physical activity (extra-school physical exercise: low <1hour/day, high >1hour/day) registered. BMD assessed by peripheral DXA densitometer. RESULTS Most fractures were caused by mild-moderate precipitating trauma by accidental fall (85.6%), with radius fracture in most cases (87.5%). BMI was higher in patients (19.14 kg/m(2)+/-3.50 vs. 17.46 kg/m(2)+/-2.67; p<0.0001). TS thickness was similar in both groups (20.28 mm+/-8.24 vs. 19.61 mm+/-6.60; p>0.05). Physical activity was lower in study group (extra-school physical activity >1h: 21.25% vs. 46.87%; p<0.001). Daily calcium intake was not associated with DFF incidence (918.75 mg/day+/-338.04 vs. 886.13 mg/day+/-382.77; p>0.05). BMD was lower in fractured children (0.2591 g/cm(2)+/-0.0413 vs. 0.2801 g/cm(2)+/-0.0300; p<0.0001) SUMMARY Our results suggest that significantly reduced bone mineral density, overweight and low physical activity are potential risk factors for fracture of the distal forearm, whilst low dietary intakes of calcium do not seem to be associated. The current epidemic of infantile overweight might explain the increased incidence of fractures of the distal forearm.

Pérdida del lenguaje

CASO CLÍNICO Varón de 7 años y 3 meses de edad, que es remitido a consulta de neurología pediátrica por presentar en los últimos 20 días pérdida del lenguaje hablado acompañado de una disminución de comprensión de lo que oye, manifestados por una llamativa disminución del rendimiento escolar. No referían cambio de carácter ni de comportamiento. Exploración física y neurológica dentro de la normalidad. Paciente colaborador, no comprende las órdenes verbales, y responde a órdenes gestuales de manera coherente con lenguaje pobre, casi monosilábico. Antecedentes personales: Desarrollo psicomotor acorde a su edad. Buena conducta y rendimiento escolar muy bueno. Diagnosticado 5 meses antes de epilepsia parcial idiopática, en tratamiento desde entonces con oxcarbazepina, y había precisado aumento de dosis un mes antes por continuar con crisis, aunque se mantenía en rango terapéutico. Se realizaron audiometría y potenciales evocados auditivos que fueron normales y la unidad de Salud Mental Infantil descartó alteraciones de conducta y del estado de ánimo. Se realizó también un EEG con privación de sueño en el que había una actividad de base normal con descargas focales de punta-onda en zona temporal del hemisferio izquierdo (similar al EEG previo) y en el que durante el sueño aumentaba la frecuencia de esas descargas dando un registro de punta-onda continua durante el sueño (POCS) (tabla 1). Se realizó RM cerebral con contraste que no mostró alteraciones. Se sustituyó progresivamente la oxcarbazepina por ácido valproico, con muy buena evolución; a partir del quinto día mejoró la comprensión del lenguaje, y posteriormente la expresión verbal, que fue completa a las 2 semanas. Tras 2 meses no había presentado nuevas crisis convulsivas. En el EEG persistía una focalidad temporal pero no había complejos POCS durante el sueño. PREGUNTAS 1.a ¿Cuál es su diagnóstico? 2.a ¿Qué actitud tomaría? 3.a ¿Qué evolución cabe esperar?

Lavados colonicos anterogrados para el estrenimiento refractario en ninos sin encefalopatia

A descriptive review of 12 patients who underwent appendicocecostomy or caecostomy for antegrade colonic lavage from January 2002 to February 2008. There were 9 appendicocecostomies performed patients from 3 to 13 years suffering from myelomeningocele, of which 8 of them had a very good outcome, with one case withdrawn due to poor use by the family. Three caecostomies were performed in non-mentally retarded constipated children. One was an otherwise healthy 7 year-old boy with hard stools since he was 10 months old, in spite of multiple laxative treatments, with normal morphology and function. He had a percutaneous caecostomy five years ago, with some improvement and a good quality of life, but still some occasional partial impactions. Another healthy 12 year-old boy with daily constipation associated faecal incontinence since he was 3 years old (normal manometry and rectal biopsy with signs of mild neuronal dysplasia) had a percutaneous caecostomy performed three years ago, with improvement in the faecal incontinence and better psychological outcome. The last caecostomy patient was an 8-year-old boy, with a similar clinical history and good progress in last three years after placing a Chaits button using an endoscopic procedure. Stubborn constipation continuing into adult life has a negative impact on the social and emotional adaptation of the paediatric patient, affecting family interactions. Antegrade colonic lavage allows independence and improves the quality of life in patients affected by recurrent faecal impactions. This technique needs to be performed on more patients to find out its true effectiveness.

Colestasis intrahepática familiar progresiva tipo 3. Defecto de MDR3

182 An Pediatr (Barc). 2008;69(2):181-96 series publicadas, se presenta entre el 3,75 y el 6,6%4-8 de los casos. Según Bagwell, las HDC con secuestro extralobar presentan una mayor incidencia de malformaciones asociadas (15-50%)9. Sólo existe un caso publicado en la bibliografía de duplicación intestinal y poliesplenia, que necesitó un segundo tiempo quirúrgico para su exéresis5. Queremos recalcar que a pesar del mal pronóstico inicial, la paciente presentó una evolución favorable que permitió su extubación a las 48 h tras la cirugía, y que fue dada de alta de la UCIN a los 13 días. Con respecto a los métodos de diagnóstico prenatal, éstos han demostrado tener una elevada sensibilidad y una baja especificidad10, como vemos en nuestro caso, en que se detectó la HDC y el secuestro pero no las otras malformaciones asociadas.

Carta al editorHemocromatosis neonatal: un nuevo y esperanzador horizonteNeonatal haemochromatosis: a new and promising horizon

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