Unidad Multidisciplinar de Fibrosis Quística del Hospital Universitario 12 de Octubre / Cystic Fibrosis Multidisciplinary Unit of Hospital Universitario 12 de Octubre

La fibrosis quística (FQ) es la enfermedad genética letal más frecuente en la raza blanca. Se trata de una enfermedad multisistémica crónica minoritaria grave que altera profundamente la vida del paciente y de su familia y va a requerir una atención permanente y de por vida. Su diagnóstico y manejo son complejos y costosos, requiere la atención de un equipo multidisciplinar. El tratamiento optimizado de los niños con FQ ha provocado una mejoría progresiva de la calidad de vida y de la supervivencia media de estos pacientes. El cribado neonatal llevado a cabo en unidades especializadas puede mejorar el pronóstico de estos niños (AU)

Cystic fibrosis (CF) is the most frequent lethal genetic disorder in white people. It is a multisystemic, chronic disorder that severely interferes with the patient's life and his family's and Will require permanent care throughout life. Its diagnosis and management are complex and costly, and benefit from the work of a multidisciplinary team. Optimized treatment of children with CF has been proven to improve quality of life and mean survival rates for these patients. Newborn screening undertaken in specialized units further ameliorates prognosis (AU)

[Delay in the acquisition of sucking-swallowing-breathing in the preterm; efects of early stimulation]. / Retraso de la adquisición de la succión-deglución-respiración en el pretérmino; efectos de una estimulación precoz.

INTRODUCTION: Premature baby's oral feeding is not possible until the reflex of sucking-swallowing-breathing adquisition. Its delay extends hospital stay and increases the incidence of oral motor disorders in early childhood. AIMS: To analyze the transition from enteral to oral nutrition, the comorbidity associated with its delay and the impact of an early suction stimulation in a cohort of premature babies. PATIENTS AND METHODS: Retrospective checking of 95 infants less than 32 gestation weeks (GW) admitted to a neonatal ICU in the last 4 years. It was revised the gestational age, anthropometric at birth and discharge, comorbidity, duration of mechanical ventilation, oxygen requirements, time of beginning and end of enteral/oral nutrition, beginning of Kangaroo method and the suction stimulation and the daily weight gain average. RESULTS: Suction stimulation began between weeks 29 and 40 GW (average and median 32 GW). Oral nutrition was initiated between 31-40 GW (average and median 33 GW) and completed between 33-44 GW (average and median 35 GW). Oral nutrition was delayed in patients who required longer mechanical ventilation and oxygen therapy. There was a positive correlation between the beginning of suction stimulation and the time of acquisition of a complete oral nutrition (84% Spearman correlation test) and length of hospital stay (80% Spearman correlation test). CONCLUSIONS: [corrected] Early suction stimulation in a preterm patient seems to facilitate full oral nutrition at an early stage and it is associated with a hospital stay decrease and the improvement in the daily weight gain average.

Retraso de la adquisición de la succión-deglución-respiración en el pretérmino; efectos de una estimulación precoz / Delay in the acquisition of sucking-swallowing-breathing in the preterm; efects of early stimulation

Introducción: La nutrición oral del prematuro no es posible hasta la adquisición del reflejo de la succión-deglución-respiración. Su retraso prolonga la estancia hospitalaria y aumenta la incidencia de trastornos oro-motores en los primeros años de la infancia. Objetivos: Analizar la transición de la nutrición enteral a la oral, la comorbilidad asociada con su retraso y el impacto de una estimulación precoz de la succión en una cohorte de prematuros. Pacientes y métodos: Revisión retrospectiva de 95 prematuros menores de 32 semanas de gestación (SG) ingresados en una UCI neonatal en los últimos 4 años. Se revisó la edad gestacional, somatometría al nacimiento y al alta, comorbilidad asociada, duración de la ventilación mecánica, necesidades de oxígeno, momento de inicio/fin de nutrición enteral/oral, inicio del método canguro y estimulo de la succión y ganancia ponderal media diaria. Resultados: La estimulación de la succión se inició entre la semana 29 y 40 SG (media y mediana 32 SG). La nutrición oral se inició entre la 31-40 SG (media y mediana 33SG) y completó entre la 33-44 SG (media y mediana 35 SG). La nutrición oral se retrasó en los pacientes que necesitaron más tiempo de ventilación mecánica y oxigenoterapia. Hubo una correlación positiva entre el inicio de la estimulación de la succión y el momento de adquisición de una nutrición oral completa (84% test de correlación de Spearman) y el tiempo de estancia hospitalaria (80% test de correlación de Spearman). Conclusiones: El estímulo precoz de la succión en el paciente pretérmino parece facilitar la nutrición oral completa de manera precoz, asociándose a una disminución en la estancia hospitalaria y a una mejora en la ganancia ponderal media diaria (AU)

Introduction: Premature baby's oral feeding is not possible until the reflex of sucking-swallowing-breathing adquisition. Its delay extends hospital stay and increases the incidence of oral motor disorders in early childhood. Aims: To analyze the transition from enteral to oral nutrition, the comorbidity associated with its delay and the impact of an early suction stimulation in a cohort of premature babies. Patients and methods: Retrospective checking of 95 infants less than 32 gestation weeks (GW) admitted to a neonatal ICU in the last 4 years. It was revised the gestational age, anthropometric at birth and discharge, comorbidity, duration of mechanical ventilation, oxygen requirements, time of beginning and end of enteral/oral nutrition, beginning of Kangaroo method and the suction stimulation and the daily weight gain average. Results: Suction stimulation began between weeks 29 and 40 GW (average and median 32 GW). Oral nutrition was initiated between 31-40 GW (average and median 33 GW) and completed between 33-44 GW (average and median 35 GW). Oral nutrition was delayed in patients who required longer mechanical ventilation and oxygen therapy. There was a positive correlation between the beginning of suction stimulation and the time of acquisition of a complete oral nutrition (84% Spearman correlation test) and length of hospital stay (80% Spearman correlation test). Conclussions: Early suction stimulation in a preterm patient seems to facilitate full oral nutrition at an early stage and it is associated with a hospital stay decrease and the improvement in the daily weight gain average (AU)

Diagnóstico y tratamiento de la leishmaniasis visceral infantil / Visceral childhood leishmaniasis: Diagnosis and treatment

Introducción: La leishmaniasis visceral es una enfermedad potencialmente grave y endémica en España. Recientes avances en las técnicas diagnósticas y el tratamiento permiten mejorar el abordaje de la enfermedad. Objetivos: Analizar las características clínicas y epidemiológicas de los pacientes diagnosticados de leishmaniasis visceral, evaluar las técnicas diagnósticas utilizadas y la eficacia y la seguridad de los tratamientos empleados. Métodos: Se revisaron de forma retrospectiva las historias clínicas de los niños diagnosticados de leishmaniasis visceral desde enero de 1994 hasta diciembre de 2007 en un hospital de tercer nivel del área sur de Madrid. Se consideró diagnóstico de enfermedad la existencia de clínica compatible y la visualización del parásito, o el aislamiento en cultivo del aspirado de médula ósea (AMO) o la detección del ADN del parásito mediante técnicas moleculares (reacción en cadena de la polimerasa). Resultados: En el periodo de tiempo estudiado se diagnosticó de leishmaniasis visceral a 11 pacientes. La mediana de edad fue de 21 meses (rango: 4 meses–13 años). La fiebre estaba presente en todos los casos, y la hepatomegalia y esplenomegalia en el 91%. La anemia fue el hallazgo hematológico más frecuente (100%). El AMO se realizó en todos los pacientes. El examen microscópico del AMO detectó la presencia de amastigotes intracelulares en el 73% de los casos. Se detectó la presencia de ADN del parásito en todos los casos. Los títulos de inmunofluorescencia indirecta fueron superiores a 1/40 en el momento del diagnóstico en el 63%. La determinación del antígeno del parásito en la orina fue positiva en 4 de 6 pacientes (67%). Se trató a 3 pacientes con antimoniato de N-metil-glucamina y a 8 pacientes (73%) con anfotericina B liposómica. Se observó una respuesta clínica precoz en todos los pacientes. Un paciente tratado con anfotericina B liposómica presentó una recaída de la enfermedad. No se registraron reacciones adversas graves al tratamiento. Conclusiones: La leishmaniasis visceral sigue siendo una enfermedad frecuente en nuestro medio. Las características clínicas y analíticas al diagnóstico son similares a las observadas en otros estudios del área mediterránea. La técnica de reacción en cadena de la polimerasa en el AMO mostró una sensibilidad mayor que las técnicas tradicionales. Las técnicas no invasivas pueden ser de utilidad en pacientes con cuadro clínico compatible. La anfotericina B liposómica en pauta corta se mostró segura y eficaz en el tratamiento de la leishmaniasis visceral (AU)

Introduction: Visceral leishmaniasis is endemic in Spain. New diagnostic tools and shorter regimens of treatment are been increasingly being used in children. Objectives: To analyze the clinical and epidemiological characteristics of cases of visceral leishmaniasis, to evaluate the diagnostic techniques tested and the safety and efficacy of treatments used. Methods: We retrospectively reviewed the medical records of children diagnosed with visceral leishmaniasis between January 1994 and December 2007 in a tertiary public Hospital in the South of Madrid. The diagnosis of visceral leishmaniasis was based on visualization of Leishmania sp. in bone marrow aspirate or culture or positive PCR analysis of the bone marrow aspirate. Results: Eleven immunocompetent children were identified. Median age was 21 months (range: 4 months – 13 years). Fever was present in all cases, and hepatomegaly and splenomegaly in 10 (91%). Anemia was the most frequent haematological finding (100%). A bone marrow aspirate was obtained in all cases. Leishmania amastigotes were observed in 8 (73%) cases. Leishmania DNA in the bone marrow aspirate was detected in all patients who underwent this procedure. Positive immunofluorescent-antibody test (IFAT) analysis at baseline was observed in 63% of cases tested. The threshold titer for positivity was 1/40. Urinary antigen detection test was positive in 4 out of 6 (67%) children in whom I was performed. Initial treatment consisted of meglumine antimoniate in 3 patients and liposomal amphotericin B (LAB) in 8 (73%) patients. All children had an early clinical response. Only one child treated with LAB relapsed. No severe adverse events were observed with treatment. Conclusions: Visceral leishmaniasis is still a common disease in our area. Clinical and laboratory findings of visceral leishmaniasis are similar to other Mediterranean area reports. PCR analysis of the bone marrow aspirate was more sensitive than traditional diagnostic techniques. Non-invasive diagnostic techniques may be used as an aid in the diagnosis of visceral leishmaniasis in children. Short course treatment of visceral leishmaniasis with liposomal amphotericin B has been safe and effective (AU)

[Visceral childhood leishmaniasis: diagnosis and treatment]. / Diagnóstico y tratamiento de la leishmaniasis visceral infantil.

INTRODUCTION: Visceral leishmaniasis is endemic in Spain. New diagnostic tools and shorter regimens of treatment are been increasingly being used in children. OBJECTIVES: To analyze the clinical and epidemiological characteristics of cases of visceral leishmaniasis, to evaluate the diagnostic techniques tested and the safety and efficacy of treatments used. METHODS: We retrospectively reviewed the medical records of children diagnosed with visceral leishmaniasis between January 1994 and December 2007 in a tertiary public Hospital in the South of Madrid. The diagnosis of visceral leishmaniasis was based on visualization of Leishmania sp. in bone marrow aspirate or culture or positive PCR analysis of the bone marrow aspirate. RESULTS: Eleven immunocompetent children were identified. Median age was 21 months (range: 4 months - 13 years). Fever was present in all cases, and hepatomegaly and splenomegaly in 10 (91%). Anemia was the most frequent haematological finding (100%). A bone marrow aspirate was obtained in all cases. Leishmania amastigotes were observed in 8 (73%) cases. Leishmania DNA in the bone marrow aspirate was detected in all patients who underwent this procedure. Positive immunofluorescent-antibody test (IFAT) analysis at baseline was observed in 63% of cases tested. The threshold titer for positivity was 1/40. Urinary antigen detection test was positive in 4 out of 6 (67%) children in whom I was performed. Initial treatment consisted of meglumine antimoniate in 3 patients and liposomal amphotericin B (LAB) in 8 (73%) patients. All children had an early clinical response. Only one child treated with LAB relapsed. No severe adverse events were observed with treatment. CONCLUSIONS: Visceral leishmaniasis is still a common disease in our area. Clinical and laboratory findings of visceral leishmaniasis are similar to other Mediterranean area reports. PCR analysis of the bone marrow aspirate was more sensitive than traditional diagnostic techniques. Non-invasive diagnostic techniques may be used as an aid in the diagnosis of visceral leishmaniasis in children. Short course treatment of visceral leishmaniasis with liposomal amphotericin B has been safe and effective.

[Shigella spp. infections in Getafe Hospital between 2001 and 2006]. / Infección por Shigella spp. en el Hospital de Getafe entre 2001 y 2006.

INTRODUCTION: Shigella spp. is a bacterium that frequently causes diarrhoea in underdeveloped countries, but not so much in Spain, where it has been published that its incidence has decreased in the last few years. OBJECTIVE: The aim of this study has been to asses changes in the epidemiology of Shigella spp. infection over the last 5 years in children of Getafe, as well as the subspecies of Shigella spp. that cause illness in this area (area 10, Madrid), the need for hospitalisation, the complications and the treatment. METHOD: A retrospective study was carried out, based on a review of the medical charts of children less than fifteen years old and had a diagnosis of Shigella spp. infection during 2000-2006. They were identified through the Getafe Hospital Microbiology Service. RESULTS: Nineteen patients were identified, of which only four needed to be hospitalised. There were no differences between males and females. The Shigella spp. subspecies identified were S. sonnei and S. flexneri. The most frequent symptoms were vomiting, abdominal pain, diarrhoea, and fever. Only one had dehydration as a complication. The treatment was symptomatic, except in the four hospitalised patients, who required fluid therapy and antibiotics. The outcome was good in all patients. CONCLUSION: In this study, there appears to be an increase in the Shigella spp. incidence over the last few years in Area 10 of Madrid. Due to the fact that Shigella spp. is not a frequent bacterium in Spain, there are not enough studies on it, therefore it would be of interest to carry out prospective studies, in order to confirm this increase in incidence.

Infección por Shigella spp. en el Hospital de Getafe entre 2001 y 2006 / Shigella spp. infections in Getafe Hospital between 2001 and 2006

Introducción: Shigella spp. es un patógeno que produce gastroenteritis aguda de forma frecuente en países en desarrollo, pero no tan habitual en España, donde se ha publicado que está disminuyendo su incidencia en los últimos años Objetivo: El objetivo de este estudio fue determinar cambios en la epidemiología de Shigella spp. en los últimos 5 años en la población infantil de Getafe, así como el tipo de Shigella spp. que produce enfermedad en el área 10 de Madrid (área que depende de nuestro hospital), la necesidad de ingreso, sus complicaciones y tratamiento. Método: Se llevó a cabo un estudio descriptivo, retrospectivo, basado en revisión de historias clínicas de los pacientes, menores de 15 años, afectados por shigellosis entre los años 2001 y 2006, que fueron identificados a partir del Servicio de Microbiología del Hospital Universitario de Getafe. Resultados: Se identificaron 19 pacientes, de los cuales sólo 4 precisaron ingreso. Las subespecies de Shigella spp. identificadas fueron S. sonnei y S. flexneri. La clínica más frecuente fue diarrea, que se acompañaba de vómitos, dolor abdominal tipo cólico y fiebre elevada. Sólo uno de los pacientes presentó deshidratación hiponatrémica como complicación. El tratamiento fue sintomático, salvo en los pacientes ingresados en los que se pautó fluidoterapia intravenosa y antibioterapia, con resolución de la enfermedad. Conclusión: En el estudio realizado parece existir un aumento de la incidencia de Shigella spp. en los últimos años en el área 10 de Madrid. Al no ser un patógeno frecuente en España, no existen muchos estudios, con lo que sería interesante realizar estudios prospectivos para confirmar este aumento de incidencia (AU)

Introduction: Shigella spp. is a bacterium that frequently causes diarrhoea in underdeveloped countries, but not so much in Spain, where it has been published that its incidence has decreased in the last few years. Objective: The aim of this study has been to asses changes in the epidemiology of Shigella spp. infection over the last 5 years in children of Getafe, as well as the subspecies of Shigella spp. that cause illness in this area (area 10, Madrid), the need for hospitalisation, the complications and the treatment. Method: A retrospective study was carried out, based on a review of the medical charts of children less than fifteen years old and had a diagnosis of Shigella spp. infection during 2000-2006. They were identified through the Getafe Hospital Microbiology Service. Results: Nineteen patients were identified, of which only four needed to be hospitalised. There were no differences between males and females. The Shigella spp. subspecies identified were S. sonnei and S. flexneri. The most frequent symptoms were vomiting, abdominal pain, diarrhoea, and fever. Only one had dehydration as a complication. The treatment was symptomatic, except in the four hospitalised patients, who required fluid therapy and antibiotics. The outcome was good in all patients. Conclusion: In this study, there appears to be an increase in the Shigella spp. incidence over the last few years in Area 10 of Madrid. Due to the fact that Shigella spp. is not a frequent bacterium in Spain, there are not enough studies on it, therefore it would be of interest to carry out prospective studies, in order to confirm this increase in incidence (AU)