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Respiratory distress syndrome (RDS) was recognized to be caused by primary surfactant deficiency almost 70 years ago and continuous positive airway pressure was introduced approximately 50 years ago. Since then, there have been many developments in neonatology; we know many things but others are still controversial. The more we know, the more questions arise. However, this review aims to indicate what is more needed to understand and how should be the modern approach to RDS in the era of precision medicine. The review is divided between new concepts and new tools. We will explain the interaction between steroids, CPAP and surfactant, as well as the surfactant catabolism and the diagnosis of NARDS; lung ultrasound and new tools to optimize CPAP will also be covered. How these concepts are integrated in the author's personal experience is also illustrated.

Diffuse, interstitielle Lungenerkrankungen (ILD) des Sauglings- und Kindesalters (chILD) zeigen ein vom Erwachsenenalter deutlich abweichendes Krankheitsspektrum. Die etablierten Klassifikationen trennen dabei zwischen fur das fruhe Kindesalter typischen ILD und ILD ohne spezifischen Bezug zum Kindesalter. Die Einordnung beruht dabei auf einer synoptischen Betrachtung der Befunde von Klinik, Bildgebung, Genetik und Pathologie. Eine Lungenbiopsie wird in der Regel eingesetzt, um eine spezifische chILD-Diagnose bei bislang unklarem Befund zu erheben oder eine rasch progrediente Lungensymptomatik bzw. einen prolongierten Symptomverlauf abzuklaren. Da auch Pathologen auserhalb von Referenzzentren mit padiatrischen Lungenbefunden konfrontiert werden, stellt diese Ubersicht relevante klinische und morphologische Befunde charakteristischer chILD vor.

In premature neonates with a primary surfactant deficiency , there is ARDS and surfactants is the main line of therapy. Now it is well known that the virus is attached to and is replicated within type 11 pneumocytes, the main function of these cells is production of surfactants which decrease surface tension and keep the alveoli patent without collapse. In this condition there is a secondary surfactant deficiency which may play a role in pathogenesis and deterioration of patient clinical condition.So, I suggest that treatment by surfactant therapy either derived from endogenous or exogenous source in severe cases can improve clinical condition, delay or avoid patient ventilation by refilling debilitated surfactants and lavage of bronchial tree, this directly decrease mortality rate.In premature neonates with a primary surfactant deficiency , there is ARDS and surfactants is the main line of therapy. Now it is well known that the virus is attached to and is replicated within type 11 pneumocytes, the main function of these cells is production of surfactants which decrease surface tension and keep the alveoli patent without collapse. In this condition there is a secondary surfactant deficiency which may play a role in pathogenesis and deterioration of patient clinical condition

Pulmonary surfactant is a heterogeneous combination of lipids and proteins, which prevents alveolar collapse at the end of expiration cycle by decreasing the alveolar surface tension at the air-liquid interface. At birth, the expression of surfactant is very important for normal lung function and it is strictly correlated to gestational age. The best known genetic mutations associated with the onset of respiratory distress in preterm and full-term newborns and with interstitial lung disease later in childhood are those involving the phospholipid transporter (ABCA3) or surfactant proteins C and B (SP-C and SP-B) genes. In particular, mutations in the SP-B gene induce respiratory distress in neonatal period, while alterations on gene encoding for SP-C are commonly associated with diffuse lung disease in children or in adults. Both clinical phenotypes are present, if genetic mutations interest even the phospholipid transporter ABCA3 ambiguity in the sentence. Interstitial lung disease in children (chILD) is defined as a mixed category of mainly chronic and rare respiratory disorders with increased mortality and morbidity. Although genetic alterations are mainly responsible for the onset of these diseases, however, there are also other pathogenic factors that contribute to increase the severity of clinical presentation. In this review, we analyze all clinical features of these rare pulmonary diseases in neonatal and in pediatric age.

RESUMEN: El síndrome de distrés respiratorio o enfermedad de la membrana hialina se debe a un déficit de surfactante causado por la inmadurez pulmonar. Por ello, afecta a los recién nacidos prematuros, especialmente a aquellos cuya edad gestacional es inferior a las 28 semanas. El diagnóstico se puede realizar tanto antes como después del nacimiento. La clínica incluye cianosis central, polipnea y signos de dificultad respiratoria progresiva. En cuanto al tratamiento, es necesaria la administración de surfactante exógeno en la mayoría de los casos. También precisan de medidas suplementarias como oxigenoterapia o asistencia respiratoria con CPAP o ventilación mecánica dependiendo de la gravedad. Además de sufrir esta enfermedad, los prematuros tienen que hacer frente al entorno inhóspito en el que se encuentran: luz, ruido, dolor… La atención de enfermería es clave tanto para frenar la progresión de la enfermedad como para adecuar el ambiente de la unidad valorando las necesidades de los recién nacidos. Además, involucran a los padres en el cuidado de su hijos/as ayudándoles a establecer el vínculo afectivo que se rompe tras la separación. Debido a ello, el profesional de enfermería tiene un papel fundamental en el manejo de esta patología y en los cuidados centrados en el neurodesarrollo ABSTRACT: Respiratory distress syndrome or hyaline membrane disease is due to surfactant deficiency caused by lung inmaturity. Because of that, it affects premature newborns, especially those whose gestational age is less than 28 weeks. Clinical signs include central cyanosis, polypnea and signs of progressive respiratory distress. Regarding treatment, the administration of exogenous surfactant is necessary in most cases. Neonates also need additional measures like oxygen therapy or respiratory assistance with CPAP o mechanical ventilation depending on the severity. In addition to suffering from this disease, they need to face the luminous, noisy and painful environment. Nursing care is key to stop the progression of the illness as well as to adapt the environment of the unit to the needs of the newborns. In addition, nurses involve parents in the care of their offspring, helping them to stablish the affective bond that breaks after separation. Therefore, the nursing professional has a fundamental role in the management of this disease and in the care centered on neurodevelopment Grado en Enfermería

Background Diaphragm weakness occurs rapidly in adult animals treated with mechanical ventilation (MV), but the effects of MV on the neonatal diaphragm have not been determined. Furthermore, it is unknown whether co-existent lung disease exacerbates ventilator-induced diaphragmatic dysfunction (VIDD). We investigated the impact of MV (mean duration = 7.65 h), either with or without co-existent respiratory failure caused by surfactant deficiency, on the development of VIDD in newborn lambs. Methods Newborn lambs (1–4 days) were assigned to control (CTL, non-ventilated), mechanically ventilated (MV), and MV + experimentally induced surfactant deficiency (MV+SD) groups. Immunoblotting and quantitative PCR assessed inflammatory signaling, the ubiquitin-proteasome system, autophagy, and oxidative stress. Immunostaining for myosin heavy chain (MyHC) isoforms and quantitative morphometry evaluated diaphragm atrophy. Contractile function of the diaphragm was determined in isolated myofibrils ex vivo. Results Equal decreases (25–30%) in myofibrillar force generation were found in MV and MV+SD diaphragms compared to CTL. In comparison to CTL, both MV and MV+SD diaphragms also demonstrated increased STAT3 transcription factor phosphorylation. Ubiquitin-proteasome system (Atrogin1 and MuRF1) transcripts and autophagy indices (Gabarapl1 transcripts and the ratio of LC3B-II/LC3B-I protein) were greater in MV+SD relative to MV alone, but fiber type atrophy was not observed in any group. Protein carbonylation and 4-hydroxynonenal levels (indices of oxidative stress) also did not differ among groups. Conclusions In newborn lambs undergoing controlled MV, there is a rapid onset of diaphragm dysfunction consistent with VIDD. Superimposed lung injury caused by surfactant deficiency did not influence the severity of early diaphragm weakness. Electronic supplementary material The online version of this article (10.1186/s13054-019-2409-6) contains supplementary material, which is available to authorized users.

To determine whether 2006 Australian national guidance to delay elective caesarean section until 39 weeks’ gestation would reduce the need for neonatal retrieval for respiratory compromise following elective caesarean. This is a retrospective cohort study comparing infants born by elective caesarean section who required retrieval for respiratory distress in Western Australia before and after the national guidance (2003–2006 vs. 2008–2014). The proportion of infants born by elective caesarean section who required retrieval for respiratory distress was reduced in the later cohort (0.77% (153/19 780) vs. 0.55% (227/40 875); p = 0.0012). The diagnosis of surfactant-deficient lung disease amongst retrieved infants was also reduced (26% (40/153) vs. 17.4% (40/227); p = 0.04). A reduction in the proportion of elective caesarean sections before 39 weeks in Western Australia was associated with a reduction in retrieval for respiratory compromise related to surfactant deficiency in infants born by elective caesarean section.

The aim of the present study was to investigate the efficacy of L-carnitine therapy on the occurrence and prognosis of respiratory distress syndrome (RDS). A single blind, randomized controlled trial study was conducted on 130 infants with gestational ages of 28-36 weeks. Infants were assigned to experimental groups (groups 1 and 2) and control groups (groups 3 and 4). Groups 1 and 3 consisted of infants with RDS, and groups 2 and 4 groups were composed of infants without RDS. The experimental groups were treated with carnitine. No statistically significant differences in serum carnitine levels were detected between the study and the control groups on day 1 of treatment (P=0.06). However, on day 7 of treatment, serum carnitine levels in the experimental groups were significantly increased (P=0.02), as compared with the control groups. The surfactant requirement value, which is how many rounds of surfactant therapy were required, was 1.56+/-0.97 in group 1, and 2.12+/-0.99 in group 3 (P

Mary Ellen Avery’s research is recognized as a milestone in biomedical research. She had discovered the underlying cause of Hyaline Membrane Disease, surfactant deficiency, fostering ever more vigorous efforts to reduce neonatal mortality in the burgeoning practice of Neonatology. Neonatology is the only clinical discipline that began as an experiment, making it a model for biomedical research. Avery knew that the concerted effort to treat preterm newborns could potentially do more harm than good, violating her oath to Hippocrates, if not held to the highest scientific standards. She remained true to that pledge throughout her career, as recounted in this Review.

Blood aspiration syndrome is a distinct diagnostic entity that can result in significant respiratory distress in the neonate due to secondary surfactant deficiency. Few cases were reported with blood aspiration syndrome and surfactant therapy was tried in some cases. I reported one case of prevention of imminent blood aspiration syndrome associated with birth asphyxia due to ruptured uterus, where blood aspiration using the meconium aspirator was done to decrease the blood load to the alveoli to prevent secondary surfactant deficiency. Blood aspiration syndrome, if suspected, a trial of blood aspiration should be tried to avoid secondary surfactant deficiency. More observations and judgments are needed to define and explain the indications for blood aspiration in cases of suspected blood aspiration syndrome.

Many premature infants have deficient pulmonary surfactant, which is required to reduce surface tension and prevent lungs from collapsing. A recent study may provide a new avenue for therapy by showing that vascular endothelial growth factor (VEGF) helps to regulate surfactant production. When given to premature mice near birth, VEGF can reduce symptoms of surfactant deficiency and relieve respiratory distress (pages 702–710).

We would like to report a series of four newborns in an extended family who have had spontaneous pneumothorax soon after birth without any obvious cause (fig 1). There is no history of pneumothorax in anyone else in the family. Figure 1 Genetic map showing the positions of the neonates with spontaneous pneumothorax in the extended family. A female infant was born in good condition at term by emergency caesarian section after a normal pregnancy. She had a spontaneous right sided tension pneumothorax within a few minutes of birth. The pneumothorax was drained. There was no evidence of surfactant deficiency on any of the initial chest radiographs. After discharge she went on to have three other …

In a randomized trial in 102 preterm newborns with respiratory distress syndrome (RDS) it has been shown that early Ambroxol treatment (30 mg kg(-1) over the first 5 days) significantly reduces the incidence of RDS-associated complications [bronchopulmonary dysplasia (BPD), intraventricular haemorrhage, post-natal acquired pneumonia]. The aim of the present analysis was to investigate the effect of Ambroxol treatment on lung function in newborns who developed BPD. Respiratory function testing (RFT) was performed immediately after extubation and at day 28. Tidal volume (VT) and respiratory frequency (f) were measured during tidal breathing using the deadspace free flow-through technique. The lung mechanic parameter VT/maxPes was determined by measuring the maximal oesophageal pressure changes, maxPes, with a catheter tip pressure transducer. In the placebo group 36/50 infants were extubated within the first 28 days of life and 13/36 (36%) developed BPD. In the Ambroxol group 44/52 were extubated and 9/44 (20%) developed BPD. After extubation, RFT showed (i) no statistically significant difference in the ventilatory parameters of either treatment group, (ii) improved (P

In this report we present data of three premature infants who received 1 to 2 doses of surfactant for an acute respiratory deterioration and after initial surfactants treatment for RDS.

The most consistently cited diagnostic criterion for respiratory distress syndrome (RDS) in premature infants is a reticulogranular pattern on chest x-ray (CXR). RDS is considered the clinical manifestation of surfactant deficiency. We undertook a study to determine the degree to which the x-ray diagnosis of RDS correlates with response to surfactant treatment.

Introduction: ITPV is a new experimental modality of ventilator support. During ITPV there is continuous gas flow through a small reverse thrust catheter which sits inside the ETT with the tip near the carina allowing ventilation with low Tidal volume. Our objective was to compare ITPV to Conventional ventilation in a model of surfactant deficiency.

NITRIC OXIDE (NO) SYNTHASE INHIBITION PREVENTS SURFACTANT-INDUCED CEREBRAL VASODILATATION IN NEWBORN PIGLETS WITH SURFACTANT DEFICIENCY. 2123

Treatment with surfactant is associated with a rapid improvement in oxygenation, but no work has yet demonstrated a rapid increase in respiratory compliance (Crs). Using a computerised passive expiratory flow technique, we measured respiratory compliance immediately before, at 3 hours and 12 hours after the first dose of surfactant (Curosurf) in 33 infants with respiratory distress syndrome and a/A ratio < 0.22. Using the same technique in an earlier reference population, no infant with Crs ≥ 2ml/cm H2O/m had biochemical evidence of surfactant deficiency. There was an increase of 6% in mean Crs at 3 hours after surfactant (NS: p = 0.227). Excluding 3 infants with initial Crs ≥ 2 ml/cm H2O/m, the increase in Crs at 3 hours was 13% (p = 0.016). In keeping with previous experience, there was rapid improvement in oxygenation, with a mean reduction in FiO2 of 45% of its initial value 3 hours after surfactant (p < 0.001). All 3 infants with an initial Crs of ≥2 ml/cm H2O/m had lower Crs 3 hours after surfactant. In the infants whose initial Crs was < 2 ml/cm H2O/m Crs increased by 38% (p

Efficient ventilation relieves pulmonary vasospasm in persistent fetal circulation (PFC). Evidence on surfactant deficiency