[Factors related to respiratory complications in very low birth weight infants with respiratory distress syndrome]. / Factores en la morbilidad respiratoria de los recién nacidos de muy bajo peso con membrana hialina.

INTRODUCTION: Very low birth weight (VLBW) infants, with a birth weight below 1500 g and a structurally immature lung, are at high risk for developing bronchopulmonary dysplasia. This risk is even higher if respiratory distress syndrome is present. Other acute lung diseases, such as air leak and pulmonary hemorrhage, can also be present. The aim of this study was to analyze the statistical relevance of several neonatal factors in the development of pulmonary complications in a sample of VLBW infants with respiratory distress syndrome. PATIENTS AND METHODS: A total of 209 VLBW infants with respiratory distress syndrome were studied. The variables analyzed were delivery date, respiratory distress syndrome grade, sex, birth weight, gestational age, referral (from within the hospital or elsewhere), prenatal corticosteroid administration, type of gestation, type of delivery, amniorrhexis time, Apgar test at 1 and 5 minutes, surfactant administration, hours of life at which the first dose of surfactant was administered, and early sepsis. A multiple logistic regression analysis was developed using Hosmer-Lemeshow methodology. RESULTS: In the multivariate analysis, air leak was related to respiratory distress syndrome grade and surfactant administration. Pulmonary hemorrhage was related to lower birth weight and absence of prenatal corticosteroid administration. Bronchopulmonary dysplasia was related to single pregnancies, absence of prenatal corticosteroid administration, lower birth weight, lower Apgar score at 1 minute, and higher respiratory distress syndrome grade. CONCLUSIONS: Respiratory morbidity in VLBW infants with respiratory distress syndrome could be influenced by several interrelated intrinsic and extrinsic variables.

[Sickle cell disease: registry of the Spanish Society of Pediatric Hematology]. / Enfermedad de células falciformes: registro de la Sociedad Española de Hematología Pediátrica.

INTRODUCTION: Sickle cell disease (SCD), a genetic anemia, is currently an emerging health problem in Spain. Since 2000, The Spanish Society of Pediatric Hematology has maintained a registry of these patients. The data corresponding to 2004 are presented herein. PATIENTS AND METHODS: Information was sent by different national hospitals. Pediatric patients with SCD followed-up during 2003 were registered in the first quarter of 2004. Data on epidemiology, diagnosis, treatment and outcome in each patient were gathered. RESULTS: A total of 138 patients in 24 national hospitals were registered. Of these, 99 were still under follow-up. There was no significant difference in sex. The mean age was 8.2 years. Seventy-eight percent of the patients were homozygous. Forty-four percent were born in Africa but 76% had abnormal genes originating in Africa. Neurophysiologic disorders were detected in 36% of the patients. Symptomatic treatment was given in 65%, hydroxyurea in 27%, hypertransfusional therapy in 3%, and chelation therapy, indicated for ferric overload, was provided in 4%. None of the patients underwent stem cell transplantation. Acute complications requiring hospitalization occurred in 21%, and chronic complications were observed in 27%. The most frequent chronic complications were delayed height and weight gain and liver and biliary tract disorders. Two patients died. CONCLUSIONS: This study confirms a highly significant increase in the prevalence of pediatric patients with SCD in the last 4 years, requiring greater resources to be devoted to the diagnosis and follow-up of this disease.

Enfermedad de células falciformes: registro de la Sociedad Española de Hematología Pediátrica / Sickle cell disease: registry of the spanish society of pediatric hematology

Introducción La enfermedad de células falciformes (ECF), anemia de origen genético, es un problema de salud emergente en España. La Sociedad Española de Hematología Pediátrica realiza desde el año 2000 un registro de estos pacientes. Se presentan los datos correspondientes a la recogida de 2004. Pacientes y métodos De la información enviada por diferentes hospitales nacionales se registraron en el primer trimestre de 2004 los pacientes pediátricos con ECF en seguimiento en 2003 y se recogieron variables epidemiológicas, relativas al diagnóstico, tratamiento y evolución de cada paciente. Resultados Se registraron un total de 138 enfermos, 99 aún en seguimiento, de 24 hospitales nacionales, sin diferencia significativa entre sexos y con una media de edad de 8,2 años. Eran homozigotos (SS) el 78 %. El 44 % habían nacido en África, pero tenían genes anómalos procedentes de África el 76 %. Se detectaron anomalías neuropsiquiátricas en el 36 % de los pacientes investigados. Se hizo tratamiento sólo sintomático en el 65 %, tratamiento con hidroxiurea en el 27 %, terapia hipertransfusional en el 3 % y quelación por sobrecarga férrica en el 4 %. Ningún paciente fue sometido a trasplante de progenitores hematopoyéticos ese año. Presentaron complicaciones agudas con necesidad de hospitalización el 21 % y complicaciones crónicas el 27 %. De éstas, las más frecuentes fueron los retrasos en la curva ponderoestatural y trastornos hepatobiliares. Dos pacientes fallecieron. Conclusiones Se confirma en los últimos 4 años un incremento nacional muy significativo de pacientes pediátricos con ECF, que obligaría a un mayor esfuerzo en su diagnóstico y seguimiento

Introduction Sickle cell disease (SCD), a genetic anemia, is currently an emerging health problem in Spain. Since 2000, The Spanish Society of Pediatric Hematology has maintained a registry of these patients. The data corresponding to 2004 are presented herein. Patients and methods Information was sent by different national hospitals. Pediatric patients with SCD followed-up during 2003 were registered in the first quarter of 2004. Data on epidemiology, diagnosis, treatment and outcome in each patient were gathered. Results A total of 138 patients in 24 national hospitals were registered. Of these, 99 were still under follow-up. There was no significant difference in sex. The mean age was 8.2 years. Seventy-eight percent of the patients were homozygous. Forty-four percent were born in Africa but 76 % had abnormal genes originating in Africa. Neurophysiologic disorders were detected in 36 % of the patients. Symptomatic treatment was given in 65 %, hydroxyurea in 27 %, hypertransfusional therapy in 3 %, and chelation therapy, indicated for ferric overload, was provided in 4 %. None of the patients underwent stem cell transplantation. Acute complications requiring hospitalization occurred in 21 %, and chronic complications were observed in 27 %. The most frequent chronic complications were delayed height and weight gain and liver and biliary tract disorders. Two patients died. Conclusions This study confirms a highly significant increase in the prevalence of pediatric patients with SCD in the last 4 years, requiring greater resources to be devoted to the diagnosis and follow-up of this disease

Factores de riesgo en la mortalidad de los recién nacidos de muy bajo peso con membrana hialina / Risk factors for mortality in very low birth weight infants with respiratory distress syndrome

Introducción: La expectativa de vivir de los recién nacidos de muy bajo peso (RNMBP) ha mejorado en los últimos años. Cuando estos pacientes presentan, además, enfermedad de membrana hialina (EMH), es difícil conocer con seguridad cuáles son los factores que más influyen en su mortalidad. Este trabajo tiene el objetivo de averiguar, dentro de un conjunto de variables, las más influyentes en la mortalidad desde una perspectiva multifactorial. Pacientes y métodos Se tomó una muestra de 209 RNMBP con EMH nacidos en un período amplio, 15 años y 7 meses. Se consideraron las variables: "fecha de parto", "grado de enfermedad de membrana hialina", "sexo", "peso al nacimiento", "semanas de gestación", "procedencia", "administración de corticoides prenatales", "tipo de gestación", "tipo de parto", "momento de la amniorrexis", "puntuación del test de Apgar al minuto y a los 5 min", "administración de surfactante", "horas de vida en la administración de la primera dosis de surfactante" y "sepsis precoz". Siguiendo la metodología de selección de variables de Hosmer-Lemeshow se realizó un análisis de regresión logística múltiple. Resultados: Resultaron significativas las variables peso al nacimiento; test de Apgar, 5; corticoides prenatales, grado de membrana hialina y tratamiento con agente tensioactivo, quedando la importancia del resto de las variables diluida en ellas. Conclusiones: El incremento de peso y el test de Apgar a los 5 min, la administración de agente tensioactivo y corticoides, así como un grado bajo de membrana hialina hacen que la mortalidad disminuya. El modelo de regresión logística encontrado cuantifica cómo actúan estos factores y permite estimar la probabilidad de fallecer para un nuevo RNMBP con EMH

Introduction: In the last few years the life expectancy of very low birth weight (VLBW) infants has improved. When these patients have respiratory distress syndrome, it is difficult to know with any certainty which factors have the greatest influence on mortality. The aim of this study was to determine which variables, among a series, have the greatest influence on mortality from a multivariate perspective. Patients and methods: A sample of 209 VLBW infants born over a long period (15 years and 7 months) was studied. The following variables were analyzed: date of birth, degree of respiratory distress syndrome, sex, birth weight, weeks of gestation, born within or elsewhere, prenatal corticoid administration, type of gestation, type of delivery, amniorrhexis time, Apgar test at 1 and 5 minutes, surfactant administration, hours of life at which the first dose of surfactant was administered, and early sepsis. A multiple logistic regression analysis was developed using Hosmer-Lemeshow methodology. Results: The following variables were identified as significant: birth weight, Apgar test at 5 minutes, prenatal corticoids, degree of respiratory distress syndrome, and surfactant administration. The remaining variables were less important in the multivariate analysis. Conclusions: Higher birth weight and Apgar score at 5 minutes, prenatal corticoid and surfactant administration, and a lower degree of respiratory distress syndrome reduce mortality. The logistic regression model used quantifies how these factors behave and allows the probability of mortality in VLBW infants with respiratory distress syndrome to be estimated

[Risk factors for mortality in very low birth weight infants with respiratory distress syndrome]. / Factores de riesgo en la mortalidad de los recién nacidos de muy bajo peso con membrana hialina.

INTRODUCTION: In the last few years the life expectancy of very low birth weight (VLBW) infants has improved. When these patients have respiratory distress syndrome, it is difficult to know with any certainty which factors have the greatest influence on mortality. The aim of this study was to determine which variables, among a series, have the greatest influence on mortality from a multivariate perspective. PATIENTS AND METHODS: A sample of 209 VLBW infants born over a long period (15 years and 7 months) was studied. The following variables were analyzed: date of birth, degree of respiratory distress syndrome, sex, birth weight, weeks of gestation, born within or elsewhere, prenatal corticoid administration, type of gestation, type of delivery, amniorrhexis time, Apgar test at 1 and 5 minutes, surfactant administration, hours of life at which the first dose of surfactant was administered, and early sepsis. A multiple logistic regression analysis was developed using Hosmer-Lemeshow methodology. RESULTS: The following variables were identified as significant: birth weight, Apgar test at 5 minutes, prenatal corticoids, degree of respiratory distress syndrome, and surfactant administration. The remaining variables were less important in the multivariate analysis. CONCLUSIONS: Higher birth weight and Apgar score at 5 minutes, prenatal corticoid and surfactant administration, and a lower degree of respiratory distress syndrome reduce mortality. The logistic regression model used quantifies how these factors behave and allows the probability of mortality in VLBW infants with respiratory distress syndrome to be estimated.