Pulmonary Hyalinising Granuloma: A report of two cases.

Pulmonary hyalinising granuloma (PHG) is a rare fibrosclerosing inflammatory lung condition of unknown aetiology. It is characterised by solitary or multiple pulmonary nodules that are usually found incidentally while imaging the chest for other reasons. We report two cases of histologically proven PHG diagnosed at the Royal Hospital, Muscat, Oman. The first case was a 71-year-old male patient who presented in 2010 with a dry cough, weight loss and bilateral pulmonary nodules. The second case was a 58-year-old male patient who presented in 2012 and was found to have incidental bilateral pulmonary nodules on chest X-ray. Both patients were started on prednisolone and on follow-up the PHG nodules remained stable. Although there is no definitive treatment, PHG generally has an excellent prognosis.

A study of spectrum of pulmonary pathology and expression of thyroid transcription factor-1 during neonatal period.

CONTEXT: Neonatal period is the single most hazardous period of life. The major causes of neonatal death are prematurity and respiratory distress syndrome. We report a series of neonatal autopsies in our Neonatal Intensive Care Unit with special emphasis on pulmonary pathology. The spectrum of pathological changes in the lungs and thyroid transcription factor-1 (TTF-1) expression was studied in detail with reference to its spatial distribution. AIMS: This study aims to analyze the causes of neonatal death with special attention to pulmonary pathology along with associated histopathological changes in lungs. We also evaluated the expression of TTF-1 at different levels of the airway. MATERIALS AND METHODS: After taking consent and anthropometric measurements, autopsy was performed. Weights of all organs were taken, and histological sections were examined under hematoxylin and eosin stain. TTF-1 immunostaining was done on lung sections. Localization of TTF-1 was evaluated at the intrapulmonary level of terminal bronchioles (TBs), distal bronchioles, and alveoli. RESULTS: We performed a series of 25 autopsies in neonates. In our series, most of the neonates were preterm (64%), had low birth weight (44%), and died within the first 7 days of life (80%). Majority (60%) of the neonates died due to pulmonary causes, followed by septicemia (24%), congenital anomalies (12%), and birth injury (4%). Among the respiratory causes, hyaline membrane disease (HMD) was diagnosed in maximum number of cases (32%), followed by pneumonia (12%) and pulmonary hemorrhage (12%). The TTF-1 expression in TBs, distal airways, and alveoli was significantly reduced or absent in cases of HMD compared to the control group. CONCLUSIONS: In this study, we observed that HMD is the most common cause of perinatal death among respiratory disorders, and in this disease, the expression of TTF-1 is significantly reduced in TBs, distal airways, and alveoli compared to the control group.

Recomendaciones para la asistencia respiratoria en el recién nacido (III). Surfactante y óxido nítrico / Recommendations for respiratory support in the newborn (III). Surfactant and nitric oxide

Las recomendaciones incluidas en este documento forman parte de una revisión actualizada de la asistencia respiratoria en el recién nacido. Están estructuradas en 12 módulos y en este trabajo se presenta el módulo 7. El contenido de cada módulo es el resultado del consenso de los miembros del Grupo Respiratorio y Surfactante de la Sociedad Española de Neonatología. Representan una síntesis de los trabajos publicados y de la experiencia clínica de cada uno de los miembros del grupo (AU)

The recommendations included in this document will be part a series of updated reviews of the literature on respiratory support in the newborn infant. These recommendations are structured into twelve modules, and in this work module 7 is presented. Each module is the result of a consensus process including all members of the Surfactant and Respiratory Group of the Spanish Society of Neonatology. They represent a summary of the published papers on each specific topic, and of the clinical experience of each one of the members of the group (AU)

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

[Short-term respiratory outcome of late preterm newborn in a center of level III]. / Devenir respiratoire à court terme d'une population d'enfants nés à 34, 35 et 36 SA dans une maternité de niveau III.

The rate of infants born at 34-36 weeks gestation has increased over the last 10 years. These babies are at higher risk of morbidity and mortality than full-term infants. At present, prenatal steroids are given until 34 weeks. The purpose of this study was to present the epidemiologic data of the late preterm infants and look for respiratory distress risk factors. This is a descriptive, single-center study including 59, 55 and 72 children born at 34, 35 and 36 weeks gestation, respectively, in a level III center in 2005 and 2006 for babies born at 34 weeks and in 2006 for the babies born at 35 and 36 weeks. Of the mothers who delivered at 34 and 35 weeks, 63% and 49%, respectively, had a morbidity. The cesarean-section delivery rate before labor was 36% for the infants born at 34 weeks and 25% for the infants born at 35 weeks. Prenatal steroids were used for 57% of the mothers who delivered at 34 weeks and for 27% of the mothers who delivered at 35 weeks. In the population of the babies born at 34 weeks, a mean delay between the last dose of steroid and delivery was 18.9 days. Of the infants born at 34, 35 and 36 weeks, 27%, 18% and 8% suffered from respiratory distress. The mechanical ventilation rate was 8.5% and 5.5% for the infants born at 34 and 35 weeks' gestation. Surfactant was given to all infants born at 34 weeks who were intubated. Twenty percent of the 34-week-gestation infants and 12.7% of the 35-week-gestation infants required mechanical ventilation or noninvasive continuous positive airway pressure. Respiratory distress was mainly caused by respiratory distress syndrome or transient tachypnea of the newborn. There were no cases of meconium aspiration syndrome. There was 1 case of infection and 2 cases of pneumothorax. One-third of the infants born at 34-35 weeks were admitted to the neonatal intensive care unit. The number dropped to 11% at 36 weeks' gestation. The gestational age was the only significant risk factor for respiratory distress. There was a strong tendency of the respiratory distress rate to decrease in the babies whose mothers had received steroids (odds ratio = 0.39, p = 0.06).

Auditory neuropathy associated with postnatally acquired cytomegalovirus infection in a very preterm infant.

Neurosensory hearing loss is a well-known complication of antenatally acquired cytomegalovirus (CMV) infection. We here report an infant who developed auditory neuropathy after a postnatally acquired CMV infection. Infection probably occurred through ingestion of infected breast milk. Following a cochlear implant, there is normal language perception and a mildly delayed language expression at age 4. We speculate that the long-term effects of perinatal CMV infections are more dependent on the postconceptional age at which infection occurs than on whether the infection occurs antenatally or postnatally. An early acquired neonatal CMV infection in very preterm infants may therefore have long-term neurological sequelae, including auditory deficits.

Surfactant Metabolism Dysfunction and Childhood Interstitial Lung Disease (chILD).

Surfactant deficiency and the resultant respiratory distress syndrome (RDS) seen in preterm infants is a major cause of respiratory morbidity in this population. Until recently, the contribution of surfactant to respiratory morbidity in infancy was limited to the neonatal period. It is now recognised that inborn errors of surfactant metabolism leading to surfactant dysfunction account for around 10% of childhood interstitial lung disease (chILD). These abnormalities can be detected by blood sampling for mutation analysis, thereby avoiding the need for lung biopsy in some children with chILD.

ABCA3 Deficiency: an unusual cause of respiratory distress in the newborn.

Respiratory Distress Syndrome (RDS) is due to deficiency of surfactant and commonly occurs in preterm babies. We report the first confirmed case in Northern Ireland of ABCA3 transporter deficiency which is a rare but important cause of RDS in term babies.A 38 week gestation female infant developed respiratory distress at four hours of age. Chest radiography was consistent with RDS. The baby required repeated doses of surfactant, each resulting in transient periods of decreased ventilatory requirement and improvement in blood gases, but unfortunately she did not survive.DNA sequencing demonstrated two different mutations in the ABCA3 gene, one inherited from each parent. The baby was therefore a compound heterozygote, and both mutations were thought to be functionally significant.ABCA3 transporter deficiency is a genetic disorder that is increasingly recognized as a cause of RDS in term babies in whom congenital deficiency of surfactant B and abnormalities of surfactant protein C have been excluded. It should be considered in mature babies who develop severe RDS.

[Juvenile hyaline fibromatosis]. / Juvenile hyaline Fibromatose.

Juvenile hyaline fibromatosis is a rare autosomal recessive disease of the connective tissue. We present the case of a 6-year-old normal mental developed boy with confluent pearly papules behind the ears and in the paranasal folds, firm nodules of the scalp, the back and metaphalangs, and severe gingival hypertrophy.

Calibration of respiratory inductance plethysmograph in preterm infants with different respiratory conditions.

Respiratory inductance plethysmography (RIP) is a method for respiratory measurements particularly attractive in infants because it is noninvasive and it does not interfere with the airway. RIP calibration remains controversial in neonates, and is particularly difficult in infants with thoraco-abdominal asynchrony or with ventilatory assist. The objective of this study was to evaluate a new RIP calibration method in preterm infants either without respiratory disease, with thoraco-abdominal asynchrony, or with ventilatory support. This method is based on (i) a specifically adapted RIP jacket, (ii) the least squares method to estimate the volume/motion ribcage and abdominal coefficients, and (iii) an individualized filtering method that takes into account individual breathing pattern. The reference flow was recorded with a pneumotachograph. The accuracy of flow reconstruction using the new method was compared to the accuracy of three other calibration methods, with arbitrary fixed RIP coefficients or with coefficients determined according to qualitative diagnostic calibration method principle. Fifteen preterm neonates have been studied; gestational age was (mean +/- SD) 31.7 +/- 0.8 weeks; birth weight was 1,470 +/- 250 g. The respiratory flow determined with the new method had a goodness of fit at least equivalent to the other three methods in the entire group. Moreover, in unfavorable conditions--breathing asynchrony or ventilatory assist--the quality of fit was significantly higher than with the three other methods (P < 0.05, repeated measures ANOVA). Accuracy of tidal volume measurements was at least equivalent to the other methods, and the breath-by-breath differences with reference volumes were lower, although not significantly, than with the other methods. The goodness of fit of the reconstructed RIP flow with this new method--even in unfavorable respiratory conditions--provides a prerequisite for the study of flow pattern during the neonatal period.

Enfermedad de la membrana hialina: síndrome del pulmón inmaduro / Sickness of the hyaline membrane: syndrome of the inmature lung

Se realizó un estudio observacional, descriptivo, de tipo transversal en la Unidad de Cuidados Intensivos Neonatales del Hospital General Docente “ Dr Ernesto Guevara de la Serna” en Las Tunas, desde el primero de enero del 2003 hasta el 31 de diciembre del 2005, con el objetivo de determinar las características de la población neonatal con enfermedad de membrana hialina. Se tomó como universo a todos los nacidos en el período y la muestra quedó constituía por los 31 recién nacidos con enfermedad de la membrana hialina .Predominó la edad gestacional de menos de 28 semanas y el peso al nacer inferior a 1500 gramos fue el más frecuentemente encontrado. El 61.3 por ciento de los pacientes no recibió tratamiento prenatal inductores de la madurez pulmonar. Predominó el nacimiento por cesárea. Se obtuvo una respuesta favorable en la mayoría de los pacientes con el uso de surfacen. El mayor número de pacientes requirió asistencia ventilatoria(AU)

An observational, descriptive and transversal study was carried out in the Neonatal Intensive Care Unit at ¨Dr Ernesto Guevara de la Serna ¨ General Hospital in Las Tunas, from January the 1st, 2003 to December the 31st, 2005 . The universal set was taken as all newborns in the aforementioned period and the sample consisted of 31 newborns with Hyaline Membrane Disease. There was predominance in the gestation period less than 28 weeks and the weight inferior to 1500 grams was most frequently encountered. 61.3 per sent of the patients did not receive prenatal treatment with betametazona .There was a predominance in Cesarian -section births. The majority of the patients obtained a favourable response with the use of surfacen. The Major number of patients required ventilatory assistance. Aggravated jaundice was the most frequent complication, followed by Re-opening of the Ductus Arteriosus(AU)

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

Introducción: Los recién nacidos de muy bajo peso (RNMBP), menor de 1.500 g al nacer, con un pulmón estructuralmente inmaduro, tienen un alto riesgo de desarrollar una displasia broncopulmonar (DBP), aún mayor si estuvieran afectados de enfermedad de membrana hialina (EMH). Además pueden presentar otras patologías pulmonares agudas como son el aire ectópico o la hemorragia pulmonar. Analizamos la importancia estadística de una serie de variables neonatales recogidas en una muestra de RNMBP con EMH que presentaron alguna de estas complicaciones. Pacientes y métodos: En 209 RNMBP con EMH estudiamos las variables 'sexo', 'tipo de gestación', 'administración de corticoides prenatales', 'amniorrexis', 'semanas de gestación', 'tipo de parto', 'fecha de parto', 'procedencia', 'peso al nacimiento', 'puntuación Apgar al primer y quinto minuto de vida', 'EMH', 'administración de sustancia tensoactiva' y 'sepsis precoz'. Con la metodología de selección de variables de Hosmer-Lemeshow se realizó un análisis de regresión logística múltiple. Resultados: En el análisis multivariante la aparición de aire ectópico se relacionó con el grado de EMH y con la administración de surfactante. El diagnóstico de hemorragia pulmonar se vinculó con la falta de administración de corticoides prenatales y con el menor peso del niño. En la DBP encontramos una relación estadística peyorativa cuando hubo una gestación única, carencia de administración prenatal de corticoides, menor peso al nacimiento, baja puntuación Apgar al primer minuto y mayor gravedad de EMH. Conclusiones: En la aparición de morbilidad respiratoria en el RNMBP con EMH podrían influir interrelacionándose variables intrínsecas y extrínsecas al neonato

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

Diagnóstico 'cronológico' en neonatología / Chronological diagnosis in neonatology

Una parte importante de la morbilidad y mortalidad neonatal ocurre en los recién nacidos prematuros, especialmente en los de muy bajo peso. Los acontecimientos que pueden presentar estos niños suelen tener una secuencia temporal, y el conocimiento de las razones que los provocan permite al neonatólogo 'esperar' el problema antes de que ocurra, detectarlo de forma inmediata y, de esta forma, hacer más eficaz el tratamiento. El objetivo de este artículo es mostrar esta secuencia temporal, las razones que la explican y algunas consideraciones terapéuticas al respecto. Obviamente, no pretendemos ser exhaustivos, pero sí didáctivos (formación de nuevos residentes en neonatología). Además, creemos que comprender esta idea tiene importantes implicaciones clínicas para todos los que asistimos a neonatos

A substantinal part of current routine practice in neonatology is focused on premature infants, especially those having a very low birth weight, the group associated with the highest rates of morbidity and mortality. The problems that these children may develop usually follow a temporal sequence. The knowledge of this common pattern enables the clinician to concentrate his attention on each predictable event as it occurs, aiding him in the diagnosis and the treatment of the patient. The aim of this paper is to describe this temporal pattern, the reasons that explain it and some considerations regarding treatment, for possible use as a tool in the training of residents in neonatology. We feel that the understanding of this issue has important implications for all the professionals involved in the care of newborn infants

Beneficio de la asistencia ventilatoria de alta frecuencia en el recién nacido / Benefits of the high frequency ventilatory support in the newborn

La ventilación de alta frecuencia es una nueva modalidad de terapia ventilatoria muy útil en los servicios de neonatología que reporta numerosas ventajas para el recién nacido con complicaciones complejas. Se realizó un estudio retrospectivo de 17 neonatos que fueron asistidos con esta modalidad en el servicio de cuidados intensivos neonatales del Hospital Ginecoobstétrico Ramón González Coro, durante el período del 2002 al 2004. Se estimó como universo a todos los RN que fueron ventilados en el período en que se realizó la investigación. Se conformó la muestra con los 17 neonatos que fueron asistidos con ventilación de alta frecuencia por presentar problemas respiratorios graves. El objetivo de este estudio fue divulgar los beneficios de este nuevo proceder. La ventilación de alta frecuencia se aplicó con mayor reiteración en los recién nacidos pretérminos, los diagnósticos médicos que más repeticiones tuvieron para su indicación fueron la enfermedad de la membrana hialina en el 49,1 por ciento y el bloqueo aéreo en el 41,2 pòr ciento; la bronconeumonía adquirida, presente en el 17,6 por ciento de la muestra, fue la complicación que más se registró. La supervivencia de niños relacionado con este soporte ventilatorio representó el 70,6 por ciento. Se demostró que la ventilación de alta frecuencia constituye una alternativa de ventilación segura y muy efectiva cuando se indica correctamente y se aplican estrictamente los cuidados de enfermería(AU)

The ventilation of high frequency is a new modality of therapy very useful ventilatoria in the neonatología services that it reports numerous advantages for the recently born with complex complications. Was he/she carried out a retrospective study of 17 neonatos that you/they were attended with this modality in the service of cares intensive neonatales of the Hospital Ginecoobstétrico Ramón González Choir, during the period of the 2002 at the 2004. He/she was considered as universe to all the RN that were ventilated in the period in that he/she was carried out the investigation. He/she conformed to the sample with the 17 neonatos that were attended with ventilation of high frequency to present serious breathing problems. The objective of this study was to disclose the benefits of this new one to proceed. The ventilation of high frequency was applied with more reiteration in the recently born pretérminos, the medical diagnoses that more repetitions had for its indication were the illness of the membrane hialina in 49,1 percent and the air blockade in the 41,2 pòr hundred; the acquired bronchopneumonia, present by 17,6 percent of the sample, it was the complication that more he/she registered. The survival of children related with this support ventilatorio represented 70,6 percent. It was demonstrated that the ventilation of high frequency constitutes a sure and very effective ventilation alternative when it is indicated correctly and they are applied the infirmary cares strictly(AU)

[Dynamic change in respiratory mechanic dynamics and its clinical significance during mechanical ventilation in hyaline membrane disease of children].

OBJECTIVE: To explore the characteristics of changes in respiratory mechanic dynamics and clinical significance in hyaline membrane disease (HMD) under mechanical ventilation. METHODS: One hundred and twenty-six newborns with HMD undergoing mechanical ventilation were divided into two groups: complication group with 43 cases and no-complication group with 83 cases. The blood gases and indices of respiratory mechanic dynamics were monitored 2, 24, 48 and 72 hours after the first ventilation and before the first weaning from ventilation. RESULTS: Pulmonary compliance [(0.55+/-0.10) ml.cm H(2)O(-1).kg(-1), (0.43+/-0.10) ml.cm H(2)O(-1).kg(-1)] and minute volume [MV, (0.65+/-0.10) L/min, (0.62+/-0.30) L/min] were elevated compared with that after ventilation for 2-72 hours, however the oxygenation index [OI, (10.2+/-1.9)mm Hg vs. (13.6+/-4.3) mm Hg] significantly lower. The compliance and MV in no-complication group were higher than that in complication group 24 and 48 hours after ventilation. There were no differences in the airway resistance and lung inflation index between two groups. The pulmonary compliance was negatively correlated with OI (r=-0.208, P<0.01) and corrected with MV (r=0.218, P<0.01). In no-complication group, all cases ventilation was weaned successfully at once in all the patients,and their mean compliance and MV were (0.55+/-0.10) ml.cm H(2)O(-1).kg(-1) and (0.65+/-0.20) L/min respectively. However, in complication group, weaning failed 38 patients, their mean compliance and MV were (1.03+/-0.30) ml.cm H(2)O(-1).kg(-1) and (0. 33+/-0.30) L/min respectively. CONCLUSION: Respiratory mechanic dynamics monitoring is beneficial in evaluating the severity of hyaline membrane disease and complications, guiding mechanical ventilation management and weaning.

Síndrome de distrés respiratorio neonatal o enfermedad de membrana hialina / Neonatal respiratory distress syndrome or hyaline membrane disease

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