Neonatal acute liver failure with pulmonary yellow hyaline membrane and kernicterus

Background Neonatal acute liver failure (NALF) is a rare and life-threatening condition. It causes bilirubin to accumulate to a dangerous level in the body, causing permanent damage to vital organs such as the brain and lungs. In many cases, the etiology of NALF remains unknown. Case presentation We described a case of an 8-day-old baby girl who presented with poor oral intake, lethargy, and jaundice. Her clinical condition rapidly deteriorated with progression to multi-organ failure, and despite intensive resuscitation efforts, she expired. At autopsy, the most significant findings were liver necrosis, yellow hyaline membrane deposition in the lungs, and bilirubin deposition in the brain (kernicterus). Conclusions NALF is a rare and potentially fatal condition necessitating prompt recognition and disease-specific treatment approaches. Toxic accumulation of bilirubin in the lungs can lead to hypoxia and precipitate further ischemic injury to the liver.

Uso convencional de surfactante en recién nacidos con enfermedad de membrana hialina / Conventional use of surfactant in newborns with hyaline membrane disease

La enfermedad de membrana hialina se debe a la deficiencia de surfactante en los pulmones de los recién nacidos especialmente los menores de 37 semanas de gestación. El manejo materno con corticoides prenatales en este grupo, disminuye la morbimortalidad asociada a esta patología neonatal. Se analiza desde el punto de la evidencia actualmente existente la administración de surfactante a estos prematuros y se revisa el tipo de surfactante a administrar, cuando es el mejor momento para administrarlo, la dosis y la forma de administrarlo.

Hyaline membrane disease is due to surfactant deficiency in the lungs of newborns, especially those younger than 37 weeks gestation. Maternal management with prenatal corticosteroids in this group reduces the morbidity and mortality associated with this neonatal pathology. The administration of surfactant to these preterm infants is analyzed from the point of the currently existing evidence and the type of surfactant to be administered is reviewed, when is the best time to administer it, the dose and the form of administration.

Betametasona fosfato para la prevención de Síndrome de Dificultad Respiratoria (SDR) del recién nacido de pretérmino / Betamethasone phosphate for the prevention of Respiratory Distress Syndrome (RDS) in the preterm newborn

RESUMEN Introducción: El estándar para inducción de madurez pulmonar en fetos con riesgo de nacer prematuramente es la administración de 12 mg de betametasona acetato/fosfato por dos veces espaciada cada 24 horas. El uso establecido en algunos hospitales públicos en Chile es con dos dosis de 12 mg betametasona fosfato aunque no existen estudios publicados sólo con betametasona fosfato sobre la incidencia de Síndrome de Distress Respiratorio (SDR). Objetivo: Evaluar efecto de betametasona en su forma fosfato como tratamiento antenatal para inducción de madurez fetal pulmonar en la incidencia SDR debido a membrana hialina en prematuros menores de 34 semanas de edad gestacional. Comparar el efecto de betametasona fosfato con el efecto publicado de betametasona acetato/fosfato. Material y método: Análisis de incidencia de SDR en prematuros nacidos en Hospital Padre Hurtado entre 24+0 y 34+0 semanas que recibieron betametasona fosfato para madurez pulmonar y aquellos que no la recibieron. Resultados: De 1.265 neonatos estudiados, 722 completaron dos dosis (57,5%); 436 sólo una dosis (34,5%) y 107 (8,5%) no recibieron corticoides antenatales. La incidencia de SDR debido a membrana hialina en el grupo con dos dosis fue 8,7%, una dosis 25,3% y 32,7% en los no tratados (p<0,001). Para SDR severo las incidencias fueron 6,7%, 12,6% y 16,8% respectivamente (p<0,001). Conclusiones: Inducción de madurez fetal pulmonar con betametasona fosfato en dos dosis de 12 mg IM separadas por 24 horas otorga una reducción significativa de incidencia de SDR semejante a la publicada con betametasona acetato/fosfato en iguales dosis.

ABSTRACT The standard for induction of lung maturity in fetuses at risk of being born prematurely is the administration of 12 mg of betamethasone acetate/phosphate two doses separated by 24 hours. The established use in some public hospitals in Chile is with two doses of 12 mg betamethasone phosphate although there are no studies published with betamethasone phosphate alone on the incidence of respiratory distress syndrome (RDS). Objective: To evaluate the effect of betamethasone in its phosphate form as antenatal treatment for the induction of fetal lung maturity in the incidence of RDS due to hyaline membrane in preterm infants less than 34 weeks of gestational age. To compare the effect of betamethasone phosphate with the published effect of betamethasone acetate/phosphate. Material and method: Analysis of the incidence of RDS in preterm infants born at Hospital Padre Hurtado between 24 + 0 and 34 + 0 weeks who received betamethasone phosphate for lung maturity and those who did not receive it. Results: Of 1,265 infants studied, 722 completed two doses (57.5%); 436 only one dose (34.5%) and 107 (8.5%) did not receive antenatal corticosteroids. The incidence of RDS due to hyaline membrane in the group with two doses was 8.7%, one dose 25.3% and 32.7% in the untreated ones (p <0.001). For severe RDS, incidences were 6.7%, 12.6% and 16.8% respectively (p <0.001). Conclusions: Induction of fetal lung maturity with betamethasone phosphate in two doses of 12 mg IM separated by 24 hours gives a significant reduction in the incidence of RDS similar to that published with betamethasone acetate/phosphate in equal doses.

Morbilidad y mortalidad por enfermedad de la membrana hialina en el Hospital General Docente Dr Agostinho Neto, Guantánamo 2016-2018 / Morbidade e mortalidade por doença da membrana hialina no Hospital Geral Dr Agostinho Neto, Guantánamo 2016-2018 / Morbidity and mortality due to hyaline membrane disease at the Dr Agostinho Neto General Teaching Hospital, Guantanamo 2016-2018

Introducción: la enfermedad de la membrana hialina es un problema de salud en la etapa neonatal. Objetivo: caracterizar a los recién nacidos ingresados en la unidad de cuidados intensivos neonatales del Hospital General Docente Dr Agostinho Neto por enfermedad de la membrana hialina durante los años 2016-2018. Método: se hizo un estudio observacional, descriptivo, prospectivo y longitudinal de 163 recién nacidos que ingresaron en la unidad. Resultados: el 16,4 por ciento de los neonatos ingresados en dicha unidad tenía esta enfermedad y la letalidad fue de 11,0 por ciento. La mayor proporción de éstos eran varones (55,8 por ciento), tenían entre 31,0 y 33,6 semanas de edad gestacional al nacer (28,2 por ciento), pesaron entre 1500,9 y 1999,9 g (27,0 por ciento), tuvieron un Apgar a los 5 minutos de nacidos entre 8 y 10 puntos (58,9 por ciento) y estuvieron en la unidad de 7 a 14 días (40,4 por ciento). El 93,3 por ciento se trató con fármacos inductores de maduración pulmonar y 100,0 por ciento con surfactante y ventilación mecánica convencional (100,0 por ciento). El 84,7 por ciento presentó complicaciones y el 55,6 por ciento falleció por hemorragia intracraneal (55,6 por ciento). Fue común que las madres tuvieran edad entre 19 y 35 años (76,6 por ciento), fueran cesareadas (65,0 por ciento) y presentaron complicaciones relacionadas con el embarazo (82,2 por ciento). Conclusión: la letalidad fue superior en la medida que disminuye la edad gestacional y el peso al nacer, en los que no fueron tratados con fármacos inductores de maduración pulmonar y que presentaron meningoencefalitis(AU)

Introduction: hyaline membrane disease is a health problem in the neonatal stage. Objective: to characterize the newborns admitted to the neonatal intensive care unit of the General Teaching Hospital Dr Agostinho Neto " due to hyaline membrane disease during the years 2016-2018. Method: an observational, descriptive, prospective and longitudinal study of 163 newborns who entered the unit was made. Results: 16.4per cent of the infants admitted to this unit had this disease and the lethality was 11.0per cent. The largest proportion of these were male (55.8per cent), were between 31.0 and 33.6 weeks of gestational age at birth (28.2per cent), weighed between 1500.9 and 1999.9 g (27.0 per cent), had an Apgar after 5 minutes of birth between 8 and 10 points (58.9per cent) and were in the unit for 7 to 14 days (40.4per cent). 93.3per cent were treated with pulmonary maturation inducing drugs and 100.0per cent with surfactant and conventional mechanical ventilation (100.0per cent). 84.7per cent presented complications and 55.6per cent died from intracranial hemorrhage (55.6per cent). It was common for mothers to be between 19 and 35 years old (76.6per cent), to be ceased (65.0per cent) and had pregnancy-related complications (82.2per cent). Conclusion: lethality was higher as the gestational age and birth weight decreased, in those who were not treated with pulmonary maturation inducing drugs and who presented meningoencephalitis(AU)

Introdução: a doença da membrana hialina é um problema de saúde no estágio neonatal. Objetivo: caracterizar os recém-nascidos internados na unidade de terapia intensiva neonatal do Hospital Geral de Ensino Dr Agostinho Neto por doença da membrana hialina durante os anos de 2016 a 2018. Método: estudo observacional, descritivo, prospectivo e longitudinal de 163 recém-nascidos que ingressaram na unidade. Resultados: 16,4 por cento dos lactentes internados nessa unidade apresentavam essa doença e a letalidade era de 11,0 por cento. A maior proporção deles era do sexo masculino (55,8 por cento), tinha entre 31,0 e 33,6 semanas de idade gestacional ao nascer (28,2 por cento), pesava entre 1500,9 e 1999,9 g (27,0 por cento), apresentou Apgar após 5 minutos de nascimento entre 8 e 10 pontos (58,9 por cento) e permaneceu na unidade por 7 a 14 dias (40,4 por cento). 93,3 por cento foram tratados com fármacos indutores de maturação pulmonar e 100,0 por cento com surfactante e ventilação mecânica convencional (100,0 por cento). 84,7 por cento apresentaram complicações e 55,6 por cento morreram de hemorragia intracraniana (55,6 por cento). Era comum as mães ter entre 19 e 35 anos (76,6 por cento), cessar (65,0 por cento) e apresentar complicações relacionadas à gravidez (82,2 por cento). Conclusão: a letalidade foi maior com a diminuição da idade gestacional e do peso ao nascer naqueles que não foram tratados com fármacos indutores da maturação pulmonar e que apresentaram meningoencefalite(AU)

Aplicación de escalas de alerta temprana en recién nacidos a término y prematuros tardíos. Estudio epidemiológico, transversal descriptivo documental basado en las historias clínicas de un grupo de neonatos / Application of early warning scales in newborns at term and late prematures. Epidemiological study, descriptive transversal documentary based on the clinical stories of a group of neonatos

Objetivo general: Aplicar escalas de alerta temprana en recién nacidos a término y prematuros tardíos. Objetivos específicos: Identificar los factores de riesgo prenatales que se encuentran en el grupo de pacientes estudiados; Recomendar el uso de la escala que detecte mejor las enfermedades y las complicaciones de los pacientes en riesgo. Población: Pacientes a término y prematuros tardíos, mayores de 35 semanas con factores de riesgo, nacidos en el HGONA. Materiales y Métodos: Se analizó la capacidad de cada escala para detectar patología según los puntajes obtenidos y luego se comparó entre escalas para analizar que escala obtuvo la mejor aproximación al número de pacientes enfermos. Resultados: Se encontró que las escalas NEWTT y NEW tuvieron una sensibilidad de 100% para síndrome adaptativo pulmonar y enfermedad de membrana hialina, NTS tuvo menor sensibilidad (71,4%) para esta patología. Para hipoglucemia la escala NEW tuvo una sensibilidad del 100%, en comparación de NEWTT y NTS que no la detectaron. En sepsis temprana la sensibilidad fue igual para NEW y NEWTT que obtuvieron puntaje de 2 y 3, NTS no la alertó. Para aspiración de meconio las tres escalas reportaron un puntaje bajo que debería analizarse dentro del contexto del inicio de la patología

Objective: Apply early warning scales in term newborns and late preterm infants. Specific objectives: Identify the prenatal risk factors found in the group of patients studied; To recommend the use of the scale that best detects the diseases and complications of patients at risk. Population: Term and late preterm patients, older than 35 weeks with risk factors, born in the HGONA. Material and Methods: The ability of each scale to detect pathology according to the scores obtained was analyzed and then it was compared between scales to analyze which scale obtained the best approximation to the number of sick patients. Results: It was found that the NEWTT and NEW scales had a sensitivity of 100% for pulmonary adaptive syndrome and hyaline membrane disease, NTS had lower sensitivity (71.4%) for this pathology. For hypoglycemia, the NEW scale had a sensitivity of 100%, compared to NEWTT and NTS that did not detect it. In early sepsis the sensitivity was the same for NEW and NEWTT who scored 2 and 3, NTS did not alert it. For meconium aspiration syndrome, all three scales reported a low score that should be analyzed within the context of the onset of the pathology

Prevalencia del trastorno succión-deglución en neonatos pretérmino nacidos en el Hospital Universitario de Santander durante 2016 / Prevalence of suction-swallowing disorders of preterm newborn infants in Hospital Universitario de Santander and its associated variables during 2016

Resumen Introducción: La alimentación por vía oral después del nacimiento es llevada a cabo gracias a la adecuada coordinación de la triada funcional succión-deglución-respiración, sin embargo, cuando hay una disfunción en alguna de las variables, se presentan los trastornos de succión-deglución. Existen diversos factores que condicionan a la presencia de estos trastornos, uno de ellos es la prematurez la cual retrasa la adquisición de la coordinación y el desarrollo de cada función de la triada. Objetivo: Determinar la prevalencia de trastorno de succión-deglución en neonatos pretérmino nacidos en el Hospital Universitario de Santander de acuerdo al grado de prematurez así como las variables asociadas. Materiales y métodos: Estudio observacional de corte transversal. Se realizó una revisión de historias clínicas de pacientes prematuros de la unidad de neonatología del Hospital Universitario de Santander durante el 2016, incluyendo un total de 115 pacientes. Resultados: Los pacientes con diagnóstico de trastorno de deglución presentaron medias de edad gestacional, peso y talla mucho menores con respecto al grupo de pacientes que no presentaron este trastorno, al igual que estancias hospitalarias mucho más prolongadas. Variables como trastorno de hipertensión asociada al embarazo, infección materna al momento del parto, abrupcio de placenta, parto por cesárea, síndrome de dificultad respiratoria, sepsis e ictericia neonatal, íleo y anemia se encontraron en mayor porcentaje en pacientes con trastorno de deglución. Conclusiones: Existen variables que se encuentran asociadas con el trastorno de succión-deglución, las cuales deben ser identificadas e intervenidas para mitigar la carga de la patología. MÉD.UIS. 2017;30(3):73-81.

Abstract Introduction: The oral feeding after birth is carried out by proper coordination of the functional suction-swallow-breathing triad; however, when there is a dysfunction in any of the variables, the suction-swallowing disorders are presented. There are several factors which predetermines the presence of these disorders, one of them is prematurity which delays the acquisition of coordination and development of each function of the triad. Objective: To determine the prevalence of suction-swallowing disorder in preterm infants born at the University Hospital of Santander according to the degree of prematurity as well as the associated variables. materials and methods: Observational cross-sectional study. A clinical history review of preterm neonates at the University Hospital of Santander during 2016, including a total of 115 patients, was carried out. Results: Patients with a diagnosis of swallowing disorder had significantly lower mean gestational age, weight and height compared to the group of patients who did not present this disorder, as well as longer hospital stays. Variables such as hypertension associated with pregnancy, maternal infection at the time of delivery, abruptio of placenta, cesarean delivery, respiratory distress syndrome, sepsis and neonatal jaundice, ileus and anemia were found to be higher in patients with swallowing disorder. Conclusions: There are variables that are associated with suction-swallowing disorder, which must be identified and intervened to mitigate the burden of the disease. MÉD.UIS. 2017;30(3):73-81.

Efectividad del tratamiento con Alveofact® y Curosurf® en prematuros de 32 semanas o menos con enfermedad de membrana hialina en Buga, Valle del Cauca, entre los años 2006 y 2013 / Effectiveness of treatment with Alveofact® and Curosurf® in preterm infants born at 32 weeks or less with hyaline membrane disease in Buga, Valle del Cauca between 2006 and 2013

RESUMEN Introducción: la enfermedad de membrana hialina, es una patología que afecta a prematuros, principalmente los menores de 30 semanas. Existen en el momento actual varios tipos de surfactantes para el tratamiento de esta entidad, los cuales han mostrado eficacia similar, pero algunos resultan ser más costo-efectivos que otros. Objetivo: evaluar la efectividad de Alveofact® versus Curosurf®, en neonatos prematuros ≤ 32 semanas, con enfermedad de membrana hialina, efectos colaterales de la administración, complicaciones neonatales y comparar los costos de su administración. Materiales y método: estudio de cohorte retrospectiva, en 136 neonatos prematuros, ≥ 24 semanas y ≥ 500g de peso al nacer; 64 de ellos fueron tratados con Alveofact® y 72 con Curosurf®. La exposición consistió en la administración de una dosis de Alveofact® (bovactant) 100mg/Kg y Curosurf® (poractant alfa) 200mg/Kg inicialmente, y como segunda dosis se suministró 100mg/Kg para cada uno. Posteriormente, se evaluó tiempo de ventilación mecánica, de oxigenoterapia, estancia, necesidad de segunda dosis, efectos colaterales por la administración y complicaciones; adicionalmente, mortalidad y displasia broncopulmonar. El análisis estadístico se realizó con Stata® 11.0, empleando Chi2 o Prueba exacta de Fisher, prueba t-test no pareado o Wilcoxon rank-sum. Resultados: no se hallaron diferencias significativas para tiempo de ventilación mecánica, oxigenoterapia, segunda dosis, estancia y complicaciones entre Alveofact® y Curosurf®. Adicionalmente, la displasia broncopulmonar se presentó en el 22,8% y la mortalidad en el 30,1%, sin diferencia significativa entre los dos surfactantes. Conclusiones: los resultados de esta investigación muestran que ambos surfactantes son igual de efectivos, pero el Curosurf® resulta más costoso. MÉD.UIS. 2016;29(3):13-25.

ABSTRACT Introduction: hyaline membrane disease, is a condition that affects preterm infants, especially those under 30 weeks. There are at present various types of surfactants for the treatment of this entity, which have shown similar efficacy, but some turn out to be more cost-effective than others. Objective: to evaluate the effectiveness of Alveofact® versus Curosurf® in preterm infants ≤32 weeks with hyaline membrane disease, side effects of administration, neonatal complications and to compare the costs of administration of the two surfactants. Materials and method: retrospective cohort study in 136 preterm infants ≥ 24 weeks and ≥ 500 g birth weight; 64 of them were treated with Alveofact ® and 72 with Curosurf ®. The exposure was the administration of a first dose of Alveofact ® (bovactant) 100mg/Kg and Curosurf ® (poractant alpha) 200mg/Kg and 100mg/Kg second dose for each group. Duration of mechanical ventilation, oxygen therapy, stay, need for second dose, side effects and complications administration were evaluated; additionally, mortality and bronchopulmonary dysplasia. Statistical analysis was performed with Stata ® 11.0 using Chi2 or Fisher exact test, t-test or unpaired Wilcoxon rank-sum. Results: no significant differences in duration of mechanical ventilation, oxygen therapy, second dose, stay and complications between Alveofact ® and Curosurf ® were found. The bronchopulmonary dysplasia occurred in 22.8% and 30.1% mortality, with no significant difference between the two surfactants. Conclusions: the results of this research show that both surfactants are equally effective, but Curosurf ® is more expensive. MÉD.UIS.2016;29(3):13-25.

Kniest dysplasia due to mutation of COL2A1 gene / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To detect potential mutation of COL2A1 gene in two children suspected for Kniest dysplasia.</p><p><b>METHODS</b>The 54 exons and splicing regions of the COL2A1 gene were amplified with PCR and the product was subjected to direct sequencing.</p><p><b>RESULTS</b>A missense mutation (c.905C>T, p.Ala302Val) was found in the coding region of the COL2A1 gene, which has been previously reported in abroad. The patients appeared to have short trunk dwarfism, enlarged joints and midface hypoplasia.</p><p><b>CONCLUSION</b>The probands are the first cases of Kniest dysplasia described in China, and so was the p.Ala302Val mutation.</p>

Presión positiva continua en la vía aérea comparada con la respiración mecánica asistida en prematuros de 28 a 32 semanas de gestación con administración precoz de surfactante pulmonar / Nasal CPAP versus mechanical ventilation in 28 to 32-week preterm infants with early surfactant administration

Introducción. La presión positiva continua en la vía aérea ( Continuous Positive Airway Pressure , CPAP) es útil en prematuros de 28 a 32 semanas de gestación con síndrome de dificultad respiratoria, pero no se ha precisado si es mejor que la respiración mecánica asistida después de la administración precoz de surfactante pulmonar. Objetivo. Comparar la incidencia de eventos adversos en prematuros de 28 a 32 semanas de gestación con síndrome de dificultad respiratoria atendidos con surfactante y respiración mecánica asistida o CPAP de burbuja. Materiales y métodos. Se atendieron 147 neonatos con respiración mecánica asistida y 176 con CPAP, ninguno de los cuales presentaba asfixia perinatal o apnea. Resultados. La incidencia de fracaso de la CPAP fue de 6,5 % (IC 95% 11,3-22,8 %). Fallecieron 29 pacientes, 7 de los cuales habían recibido CPAP (4,0 %) y, 22, respiración mecánica asistida (15,0 %; p<0,001). El riesgo relativo (RR) de morir de quienes recibieron CPAP, comparado con el de quienes recibieron respiración mecánica asistida, fue de 0,27 (IC 95% 0,12-0,61), pero, al ajustar por los factores de confusión, el uso de CPAP no implicó mayor riesgo de morir (RR=0,60; IC 95% 0,29-1,24). La letalidad con respiración mecánica asistida fue de 5,70 (IC 95% 3,75-8,66) muertes por 1.000 días-paciente, mientras que con CPAP fue de 1,37 (IC 95% 0,65-2,88; p<0,001). La incidencia de neumopatía crónica fue menor con CPAP (RR=0,71, IC 95% 0,54-0,96), al igual que la de hemorragia cerebral (RR=0,28; IC 95% 0,09-0,84) y la de sepsis (RR=0,67; IC 95% 0,52-0,86), pero fue similar en cuanto a escapes de aire (RR=2,51; IC 95% 0,83-7,61) y enterocolitis necrosante (RR=1,68; IC 95% 0,59-4,81). Conclusión. La incidencia de neumopatía crónica, hemorragia ventricular y sepsis es menor con el uso de CPAP.

Introduction: Continuous positive airway pressure (CPAP) is useful in low birth weight infants with respiratory distress, but it is not known if it is a better alternative to mechanical ventilation after early pulmonary surfactant administration. Objective: To compare the incidence of adverse events in 28 to 32-week newborns with respiratory distress managed with mechanical ventilation or CPAP after early surfactant administration. Materials and methods: In total, 176 newborns were treated with CPAP and 147 with mechanical ventilation, all with Apgar scores >3 at five minutes and without apnea. Results: The incidence of CPAP failure was 6.5% (95% CI: 11.3-22.8%); 29 patients died: 7 with CPAP (4.0%) and 22 with mechanical ventilation (15.0%, p<0.001). The relative risk of dying with CPAP versus mechanical ventilation was 0.27 (95% CI: 0.12-0.61), but after adjusting for confounding factors, CPAP use did not imply a higher risk of dying (RR=0.60; 95% CI: 0.29-1.24). Mechanical ventilation fatality rate was 5.70 (95% CI: 3.75-8.66) deaths/1,000 days-patient, while with CPAP it was 1.37 (95% CI: 0.65-2.88, p<0.001). Chronic lung disease incidence was lower with CPAP than with mechanical ventilation (RR=0.71; 95% CI: 0.54-0.96), as were intracranial hemorrhage (RR=0.28, 95% CI: 0.09-0.84) and sepsis (RR=0.67; 95%CI: 0.52-0.86), and it was similar for air leaks (RR=2.51; 95% CI: 0.83-7.61) and necrotizing enterocolitis (RR=1.68, 95% CI: 0.59-4.81). Conclusion: CPAP exposure of premature infants with respiratory distress syndrome is protective against chronic lung disease, intraventricular hemorrhage and sepsis compared to mechanical ventilation. No differences were observed regarding air leak syndrome or death.

A doença da membrana hialina em prematuros de baixo peso / Hyaline membrane disease in low birth weight preterm infants

Introdução: O objetivo do estudo foi determinar a prevalência da Doença da Membrana Hialina (DMH) em prematuros de baixo peso e suas principais complicações. Métodos: Foi realizado um estudo descritivo do tipo série de casos. A população em estudo foram 34 prematuros com peso inferior a 1500 gramas e/ou idade gestacional inferior a 32 semanas nascidos no período de julho de 2010 a julho de 2011 no Hospital Universitário de Canoas/RS. Resultados: Pré-eclâmpsia e trabalho de parto prematuro foram as causas mais frequentes de parto pré-termo. DMH ocorreu em todos os recém-nascidos com peso inferior a 1000 gramas. Em prematuros com peso ao nascer entre 1001 e 1250 gramas e 1251 e 1499 gramas, a prevalência da DMH foi de 71,4% e 44,4%, respectivamente. A complicação da DMH mais frequente foi a persistência do canal arterial. Conclusões: A prevalência da DMH encontrada foi de 100% nos prematuros com peso até 1000 gramas e de 71,4% nos RNs com peso entre 1001g e 1250g. Nos prematuros com peso de 1251g a 1499g, a prevalência foi de 44,4% (AU)

Introduction: The aim of the study was to determine the prevalence of hyaline membrane disease (HMD) in preterm, low birth weight infants and its major complications. Methods: A descriptive study of the case series was conducted. The study population were 34 preterm infants weighing less than 1500 grams and/or gestational age less than 32 weeks born from July 2010 to July 2011 at the University Hospital of Canoas, RS. Results: Pre-eclampsia and preterm labor were the most frequent causes of preterm birth. HMD occurred in all newborns weighing less than 1000 grams. In premature infants with birth weights between 1001 and 1250 grams and 1251 grams and 1499, the prevalence of DMH was 71.4% and 44.4%, respectively. The most frequent complication of DMH was the persistent ductus arteriosus. Conclusions: The prevalence of DMH was 100% in preterm infants weighing up to 1000 grams and 71.4% in newborns weighing between 1001g to 1250g. In premature infants weighing 1251g to 1499g, the prevalence was 44.4% (AU)

Conceitos atuais sobre avaliação da maturidade pulmonar fetal / Current concepts on the assessment of fetal lung maturity

A Síndrome do Desconforto Respiratório (SDR), também conhecida como Doença da Membrana Hialina, é uma das principais causas de morbidade e mortalidade neonatal. O principal fator associado à SDR é a produção insuficiente de surfactante pulmonar, o que geralmente está associada à prematuridade. Alguns protocolos internacionais recomendam que a confirmação da maturidade pulmonar fetal seja realizada em partos eletivos antes de 39 semanas de gestação. Diversos são os métodos capazes de avaliar a maturidade pulmonar fetal, como a Relação Lecitina/Esfingomielina,a Pesquisa de Corpos Lamelares, a Relação Surfactante/Albumina, o percentual deFosfatidilglicerol, o Índice de Estabilidade da Espuma e o Shake Test ou Teste de Clements. Este estudo visa apresentar os principais métodos disponíveis e as recomendações atuais sobre quando realizar a avaliação da maturidade pulmonar fetal.(AU)

The Respiratory Distress Syndrome (RDS), also known as hyaline membrane disease, is a major cause of neonatal morbidity and mortality. The main factor associated with RDS is the insufficient production of pulmonary surfactant, which is usually associated with prematurity. Some international guidelines recommend that the confirmation of fetal lung maturity is performed in elective deliveries before 39 weeks of gestation. There are several methods to assess fetal lung maturity, such as the Lecithin/Sphingomyelin ratio, the Lamellar Body Count, the Surfactant/Albumin ratio, the percentage of phosphatidylglycerol, the Foam Stability Index and the Shake Test or Clements test. This study aims to present the main available methods and current recommendations on when to conduct the evaluation of fetal lung maturity.(AU)

Eficacia de tres tipos de surfactante exógeno en prematuros con enfermedad de membrana hialina / Effectiveness of three types of exogenous surfactant in preterm infants with hyaline membrane disease

Introducción: La enfermedad de membrana hialina es causa importante de mortalidad neonatal. El objetivo de esta investigación fue evaluar la eficacia de tres tipos de surfactante exógeno en prematuros. Pacientes y Método: Estudio de cohorte retrospectiva, en 93 neonatos prematuros, > 24 semanas y > 500 g de peso al nacer, 31 para cada surfactante. La exposición fue la administración de 1ª dosis bovactant (Alveofact®) 50 mg/kg, beractant (Survanta®) 100 mg/kg inicial, y poractant alfa (Curosurf®) 200 mg/kg. Las variables en estudio incluyeron tiempo de ventilación mecánica, tiempo de oxigenoterapia, estancia hospitalaria, necesidad de segunda dosis de surfactante, eventos adversos por la administración del surfactante y complicaciones por prematuridad. Además, se evaluó mortalidad, displasia broncopulmonar (DBP) y mortalidad o DBP. Análisis estadístico mediante Stata® 11.0, empleando X² o Prueba Exacta de Fisher para variables cualitativas y Pruebas ANOVA o Kruskal-Wallis para cuantitativas y riesgo relativo para las asociaciones, todas con su intervalo de confianza de 95%. Resultados: No hubo diferencias para sexo, peso y edad gestacional al nacer entre los 3 grupos. No se hallaron diferencias estadísticamente significativas para tiempo de ventilación mecánica, tiempo de oxigenoterapia, administración de una segunda dosis de surfactante, estancia hospitalaria y complicaciones entre los 3 grupos. Los eventos adversos por administración de surfactante se presentaron para beractant y poractant alfa. Ocurrieron 30 (32,3 por ciento) muertes, 8 (25,8 por ciento) para bovactant, 10 (32,3 por ciento) beractant y 12 (38,7 por ciento) poractant alfa (p > 0,05). La mortalidad y/o DBP ocurrió en 10 (32,2 por ciento) neonatos con bovactant, 10 (32,2 por ciento) con beractant y 14 (45,2 por ciento) con poractant alfa (p > 0,05). Conclusiones: Los resultados primarios y secundarios entre los tres surfactantes evaluados fueron muy similares...

Introduction: Hyaline membrane disease is an important cause of neonatal mortality. The objective of this research is to evaluate the efficacy of three different exogenous surfactants in premature infants. Patients and Method: A retrospective cohort analysis in 93 preterm infants > 24 weeks and birth weight > 500 g was performed, 31 infants for each surfactant. Exposure consisted of the 1st dose of bovactant (Alveofact®) 50 mg/kg, beractant (Survanta®) 100 mg/kg initially, and poractant alfa (Curosurf®) 200 mg/kg. The variables included duration of mechanical ventilation, duration of oxygen therapy, hospital stay, need for second dose of surfactant, adverse events surfactant administration and prematurity complications. Mortality and bronchopulmonary dysplasia (BPD) were evaluated. Statistical analysis was performed using Stata® 11.0, X² or Fisher exact test for qualitative variables and ALNOVA or Kruskal-Wallis tests for quantitative and association relative risk, all with 95 percent confidence level. Results: There were no gender, weight and gestational age differences at birth among the three groups. No statistically significant differences were found regarding duration of mechanical ventilation, duration of oxygen therapy, administration of a second dose of surfactant, hospital stay and complications among the three groups. Adverse events related to surfactant administration occurred for beractant and poractant alpha. There were 30 (32.3 percent) deaths, 8 (25.8 percent) associated to bovactant, 10 (32.3%) to beractant and 12 (38.7 percent) to poractant alpha (p > 0.05). Mortality and/or BDP occurred in 10 (32.2 percent) infants who received bovactant, 10 (32.2 percent) beractant and 14 (45.2 percent) with poractant alpha (p > 0.05). Conclusions: The primary and secondary outcomes among the three surfactants tested were similar, taking into account the limitations of the work.

Enfermedad de membrana hialina en recién nacidos de pacientes preeclámpticas / Hyaline membrane disease in newborns of preclamptic patients

Comparar la incidencia de la enfermedad de membrana hialina del recién nacido de pacientes preeclámpticas con embarazadas normotensas sanas. Se realizó un estudio de cohortes en todas las pacientes con embarazos simples entre 24 y 36 más 5 semanas de gestación con fetos vivos en el período de enero de 1999 a septiembre de 2008. El diagnóstico de la enfermedad de membrana hialina se realizó por los criterios clínicos y radiológicos. Servicio de Ginecología y Obstetricia. Hospital Central “Dr. Urquinaona”. Maracaibo. Estado Zulia. El número de recién nacidos con diagnóstico de enfermedad de membrana hialina durante el período de estudio fue de 2 491 casos, representando un 7,73 por ciento de los recién nacidos vivos. Se encontraron 56 casos (11,24 por ciento) de la enfermedad de membrana hialina en los recién nacidos de los casos de pacientes preeclámpticas y 206 recién nacidos (10,33 por ciento) entre las pacientes controles (OR 1,098; IC 95 por ciento 0,803 - 1,502). Al seleccionar los recién nacidos con edad gestacional menor de 32 semanas, se encontró en las pacientes preeclámpticas un total de 92 recién nacidos de los cuales 28 de ellos (30,43 por ciento) desarrollaron enfermedad de membrana hialina, mientras que en el grupo de los controles se encontraron 261 recién nacidos de los cuales 70 (26,81 por ciento) desarrollaron la enfermedad (OR 1,194; IC 95 por ciento 0,708 - 2,012). No existe diferencia en la incidencia de la enfermedad de membrana hialina de recién nacido de pacientes preeclámpticas comparado con embarazadas normotensas

To compare the incidence of hyaline membrane disease in newborns of preeclamptic patients with normotensive pregnant patients. A cohort study was done with all patients with single pregnancies between 24 + 0 and 36 + 5 weeks of gestation with live fetuses in the period January 1999 to September 2008. Diagnosis of hyaline membrane disease was done over clinical criteria. Servicio de Ginecologia y Obstetricia. Hospital Central “Dr. Urquinaona”. Maracaibo. Estado Zulia. The number of newborn with diagnosis of hyaline membrane disease during the period was of 2 491 cases, representing 7.73 percent of all live newborns. There were 56 cases (11.45 percent) of hyaline membrane disease in newborn of cases and 206 newborns (10.33 percent) between control patients (OR 1.098; 95 percent IC 0.803 - 1.502). When newborn less than 32 weeks were selected, in preeclamptic patients, there were 92 newborns of whom 28 (30.43 percent) developed hyaline membrane disease, while in control group there were found 70 of 261 newborns who developed disease (OR 1.194; 95 percent IC 0.708 - 2.012). There is not difference in the incidence of hyaline membrane disease in newborn of preeclamptic patients compared with normotensive pregnant patients

Clinical evaluation of the effects of morphine in mechanical ventilation of neonates / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the effects of morphine infusion analgesia on behavioural and neuroendocrine stress response and short term outcome in ventilated neonates.</p><p><b>METHODS</b>A randomized, double-blind clinical trial was conducted between August 2010 and April 2011 at the neonatal intensive care unit of Nanjing Children's Hospital Affiliated to Nanjing Medical University. A total of 46 ventilated preterm infants (≥ 32 weeks) and term infants were divided into 2 groups at random. Twenty-two infants in test group received a loading dose (100 µg/kg) of morphine (> 1 h) followed by a continuous infusion [10 µg/(kg·h)] for (70.05 ± 29.05) h, and 24 infants in control group received 5% glucose with the same infusion rate. (1) The ventilatory parameters [respiratory rate (R), frequence (f), peak inspiratory pressure (PIP), positive end expiratory pressure (PEEP), fraction of inspired oxygen (FiO2)], mean blood pressure (MBP) and heart rate (HR) before treatment, at 30 min, 2 h, 6 h, 12 h, 24 h, 48 h after treatment between two groups were compared. (2) Pain was measured by two assessment tools [neonatal pain, agitation and sedation scale (N-PASS) and COMFORT scale] at the same periods. (3) The ventilation duration, the time from withdrawal to extubation, the total oxygen-inhaled time, the side effects and the clinical outcomes [e.g., pulmonary hemorrhage, air leak, patent ductus arteriosus (PDA), necrotizing enterocolitis (NEC), intraventricular hemorrhage (IVH)] between two groups were compared.</p><p><b>RESULTS</b>(1) There were no significant differences in the different ventilatory parameters before and after treatment between two groups at different periods (P > 0.05). There was no significant difference in the average blood pressure of two groups at different periods, but the heart rate reduced at 24 - 48 h after treatment in test group with significant difference as compared to control group (t = -2.152 and -2.513, P < 0.05). (2) The N-PASS score and COMFORT score in test group were lower than that in control group at different time points 2 h after treatment (P < 0.05), especially 12 h after treatment (P < 0.01). (3) There were no significant differences in the ventilation duration, the time from withdrawal to extubation and the total oxygen time between two groups, and also in side effects, the incidence of IVH, white matter damage and the clinical outcomes.</p><p><b>CONCLUSION</b>Continuous infusion of morphine could relieve pain in ventilated neonates, reduce the stress response and promote the human-machine coordination, but the medication did not show any effects on neurobehavioral development and short term outcome.</p>

New Synthetic Surfactants for Neonates

The benefits of exogenous synthetic or animal-derived natural surfactants for treatment of respiratory distress syndrome (RDS) are well established. Although synthetic surfactants have potential safety advantages over animal-derived products, they seem to be clinically inferior to animal-derived natural surfactant, based on the results of numerous comparative trials. In recent years, however, synthetic surfactant has experienced a surge in breakthroughs to the point of rivaling natural surfactant, mainly due to the development of protein-containing synthetic surfactant. This article will review the historical background on the development of artificial pulmonary surfactant, compositional and physicochemical aspects on pulmonary surfactant lipids and proteins, results of comparative trials among natural, protein-free and protein-containing surfactants, and current status of development of protein-containing surfactants for treatment of RDS.

Incidencia y evolución de membrana hialina en menores de 35 semanas según crecimiento intrauterino / Incidence and evolution of hyaline membrane in newborns under 35 weeks of gestational age according intrauterine growth

Introduction: It is thought that intrauterine growth restriction induces respiratory maturation. The information varies if the studies consider analysis based on birth weight or gestational age. Objective: The goal of this study is to compare the incidence and evolution of hyaline membrane disease (HMD) between small and adequate premature babies under 35 weeks of gestational age (< 35 wGA) based on data in the literature. Patients and Methods: Two databases were created and analyzed: a) 2 022 newborns < 35 wGA admitted to the Service, whose incidence of HMD was calculated, and b) 733 newborns < 35 wGA with HMD and treated with surfactant, to describe the evolution. Results: Analysis of GA group shows a higher incidence of HMD (35.2 percent) among small for GA, and less (29.1 percent) among those who are not small for GA (p: 0.026). If a subset is formed for the newborns < 1 500 g in birth weight, those small for gestational age have a lower incidence (47.5 percent) than those adequate for GA (60.7 percent). Logistic regression analysis for discharge with oxygen of newborns with HMD shows association with lower z score for birth weight, corticosteroid use and oxygen dependence at 36 weeks. Conclusions: Preterm newborns small for GA show a higher incidence of HMD and oxygen dependence when comparing for GA.

Introducción: Tradicionalmente se ha considerado que la restricción de crecimiento intrauterina produce maduración respiratoria, pero la información es diferente según si los estudios consideran el análisis por grupos de peso de nacimiento o edad gestacional. Objetivo: El objetivo de este análisis fue comparar la incidencia y evolución de membrana hialina, de los prematuros menores de 35 semanas de edad gestacional según fueran pequeños o no para edad gestacional. Pacientes y Método: Se analizaron dos bases de datos: 2 022 menores de 35 semanas hospitalizados en el Servicio para determinar incidencia de membrana hialina y 733 menores de 35 semanas tratados con surfactante con diagnóstico de membrana hialina para comparar evolución de ésta. Resultados: El análisis por grupos de edad gestacional muestra una incidencia de membrana hialina mayor, de 35,2 por ciento, en los pequeños para la edad gestacional, y de 29,1 por ciento en los no pequeños (p: 0,026). Si se analiza sólo menores de 1 500 gramos de peso de nacimiento, el grupo pequeño tiene una incidencia menor, de 47,5 por ciento, y los no pequeños de 60,7 por ciento. El análisis de regresión logística para alta con oxígeno de los que tuvieron membrana hialina, muestra asociación con menor puntaje z de peso de nacimiento, uso de corticoides y dependencia de oxígeno a las 36 semanas. Conclusiones: El recién nacido pretérmino pequeño para edad gestacional tiene mayor incidencia de membrana hialina y evoluciona con mayor dependencia de oxígeno al comparar por edad gestacional.

Identification of Characteristics and Risk Factors Associated with Mortality in Hydrops Fetalis

PURPOSE: The objectives were to identify the characteristics of neonates with hydrops fetalis, and to identify the risk factors associated with mortality. METHODS: A retrospective review of AMC (Asan Medical Center) dataset was performed from January 1990 to June 2009. The characteristics of 71 patients with hydrops fetalis were investigated and they were divided into two groups: the survived group and the expired group. Various perinatal and neonatal factors in two groups were compared to find out risk factors associated with mortality based on univariate analysis, followed by multiple regression analyses (SPSS version 18.0). RESULTS: Of those 71 neonates (average gestational age: 33 weeks, birth weight: 2.6 kg), 38 survived, 33 died, resulting in overall mortality rate of 46.5%. The most common etiology was idiopathic followed by chylothorax, cardiac anomalies, twin-to-twin transfusion, meconium peritonitis, cardiac arrythmias, and congenital infections. Factors that were associated independently with mortality in logistic regression analyses were low 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days. CONCLUSION: In this study, 5-minutes Apgar score, hyaline membrane disease and delayed in achieving 50th percentile ideal body weight for appropriate gestational age by 10 days were significant risk factors associated with mortality in hydrops fetalis. Therefore, the risk of death among neonates with hydrops fetalis depends on the illness immediately after birth and severity of hydrops fetalis. Informations from this study may prove useful in prediction of prognosis to neonates with hydrops fetalis.

Estrategia de intervención para disminuir la mortalidad por Síndrome de Dificultad Respiratoria del recién nacido en Maternidad Provincial, de Matanzas / Interventional strategy to diminish mortality by Respiratory Distress Syndrome of the newborn at the Provincial Maternity Hospital, of Matanzas

En la presente investigación se expone una estrategia de intervención dirigida a disminuir la mortalidad por Síndrome de Dificultad Respiratoria del Recién Nacido, en el Hospital Provincial Ginecobstétrico Docente Julio Alfonso Medina, de Matanzas. Sobre la base de la propia experiencia de los autores y mediante la aplicación de métodos científicos, basados en la literatura internacional actual, se presenta el resultado de un minucioso estudio de 48 recién nacidos que padecieron la enfermedad y que fueron tratados en la Unidad de Cuidados Intensivos Neonatales de esa institución hospitalaria durante los años 2006 y 2007. Las acciones estratégicas que se dan a conocer como resultado de la investigación, unido a las recomendaciones ofrecidas por los autores, constituyen una herramienta imprescindible para emprender un mejor manejo con los pacientes que padecen la enfermedad. Apoyados en el uso de una secuencia correcta del CPAP y del surfactante porcino cubano denominado SURFACEN, se dan a conocer nuevos enfoques en el tratamiento del Síndrome de Dificultad Respiratoria del Recién Nacido. Se propone el nuevo término de Enfermedad Pulmonar por Inmadurez Congénita (EPIC), para designar esta patología en lugar del término anatomopatológico de membrana hialina...

In the current investigation we expose the interventional strategy to diminish mortality by Respiratory Difficulty Syndrome of the Newborn, in the Provincial Gynecoobstetric Teaching Hospital Julio Alfonso Medina, of Matanzas. On the basis of the authors' proper experience and applying scientific methods, taking into account the current international literature, we present the results of a detailed study of 48 newborns who suffered the disease and were treated in the Neonatal Intensive Care Unit of this institution during 2006 and 2007. The strategic actions resulting from our investigation, together with the recommendations offered by the authors are indispensable for a better management of the patients suffering the disease. We offer new approaches in the treatment of the Respiratory Distress Syndrome of the Newborn on the basis of the usage of a correct sequence of the Continuous Positive Airway Pressure and the porcine Cuban surfactant called SURFACEN. We propose the new term Pulmonary Disease by Congenital Immaturity, to denominate this pathology in the place of the anatomopathologic term of hyaline membrane...

Relationship between serum estradiol levels in the early postnatal period and the occurrence of hyaline membrane disease and bronchopulmonary dysplasia in neonates / 中国当代儿科杂志

<p><b>OBJECTIVE</b>This study examined the changes of serum levels of estradiol during the early postnatal period in neonates in order to investigate the possible relationship between the serum estradiol levels and the occurrence of pulmonary hyaline membrane disease (HMD) and bronchopulmonary dysplasia (BPD).</p><p><b>METHODS</b>Fifty-nine premature infants with the gestational age between 26 and 32 weeks and 61 full-term infants with the gestational ages between 37 and 42 weeks were enrolled. Serum levels of estradiol were measured on postnatal days 1, 3 and 7.</p><p><b>RESULTS</b>Serum levels of estradiol decreased rapidly after birth in both premature and term infants and there were significant differences among different postnatal ages groups. However, there were no significant differences in the serum estradiol levels between the premature and term groups on postnatal days 1, 3 and 7. Serum estradiol levels measured in premature infants with HMD were not statistically different from those in premature infants without HMD on all time points. Serum estradiol levels in premature infants with BPD were higher than those in premature infants without BPD on postnatal day 3, but there were no noticeable differences on postnatal days 1 and 7.</p><p><b>CONCLUSIONS</b>Serum estradiol levels decline rapidly within 7 days after birth in both premature and term infants. Serum estradiol levels in the early postnatal period are not associated with the occurrence of HMD and BPD, suggesting that serum estradiol in the early postnatal period can not be used as a marker for predicting the development of HMD and BPD.</p>