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Surfactant deficiency treatment

Surfactant Deficiency Syndrome: Causes And Treatmen

The primary treatments for surfactant deficiency include, Surfactant Replacement Therapy; the objective of this therapy is to provide surfactant to the infant till they are capable of producing their own surfactant. In such situations surfactant is directly pushed in to the infants lungs using a breathing tube Currently, there is no specific treatment for any of the surfactant protein deficiencies. For affected newborns, surfactant replacement therapy may improve respiratory status transiently but is ineffective in treating the underlying deficiency. Lung transplantation may be considered Surfactant treatment has become the standard of care in premature infants with respiratory distress syndrome (RDS). Pulmonary hemorrhage, pulmonary edema, pneumonia, and atelectasis have been shown to liberate inflammatory mediators and plasma proteins, which damage type II pneumocytes and inactivate surfactant SP-B deficiency can be diagnosed prenatally or postnatally. The only current treatment options available include lung transplantation or compassionate care. Current developments in gene therapy offer hope for future treatment

Neonates with surfactant metabolism dysfunctions, especially those with SP-B disorder, only have lung transplantation as one possible choice of treatment. Children with lung transplant due to surfactant metabolism dysfunction perform on similar level to those with transplant for due to other reasons Hamvas A, Nogee LM, Mallory GB Jr, et al. Lung transplantation for treatment of infants with surfactant protein B deficiency. J Pediatr 1997; 130:231. Palomar LM, Nogee LM, Sweet SC, et al. Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure

To the Editor: We wish to report initial success in treating a boy with surfactant protein C deficiency. The patient was first admitted to the hospital at five months of age with severe respiratory.. RDS from surfactant deficiency; and rescue or therapeutic treatment, in which surfactant is administered after the initiation of mechanical ventilation in infants with clini-cally confirmed RDS.3,19,25,29,31,32 Prophylactic surfactant administration to infants at risk of developing RDS is associated with lower risk of air lea

Surfactant mutations chILD Foundatio

  1. Exogenous surfactant replacement has been established as an appropriate preventive and treatment therapy for prematurity-related surfactant deficiency. Surfactant therapy also may be indicated for more mature infants with primary pulmonary hypertension or meconium aspiration syndrome
  2. ology. RDS is also known as hyaline membrane disease (not favored as reflects non-specific histological findings), neonatal respiratory distress syndrome, lung disease of prematurity (both non-specific terms), or as some authors prefer surfactant-deficiency disorder 2.. Epidemiology. The incidence is estimated at 6 per 1000 births 2.. Uncommon after 36 weeks' gestation due to development.
  3. istration is standard therapy in neonatal RDS and provides proof of concept for surfactant-based therapy of ARDS Trials of surfactant in adults have failed to identify a mortality benefit, but these negative results may reflect technical or patient selection issue
  4. Surfactant protein C deficiency is characterised by deficiency of the protein and its intracellular accumulation, with subsequent local inflammation and interstitial lung disease. There is no specific treatment, but hydroxychloroquine has been reported to be effective
  5. istration of exogenous pulmonary surfactant is an effective treatment of premature infants with RDS due to insufficient surfactant production. 4 Although exogenous surfactant therapy has proven to be an effective treatment for RDS, no similar current effective therapy exists for patients with ARDS
  6. Pulmonary surfactant is a complex mixture of lipids and protein, which works principally to lower the surface tension of the air liquid interface within the airways and reduce the work of breathing. Deficiency of surfactant in the premature newborn is a principal mechanism in the development of respiratory distress in that population. Over the past decade, surfactant replacement therapy in.

Lung transplantation has resulted in reconstitution of pulmonary surfactant function and long-term survival. SP-B deficiency represents the first opportunity to link physiologic characteristics of respiratory failure in infancy with specific molecular and cellular defects Exogenous surfactant therapy as adjunctive treatment for near-term and term neonates with severe hypoxemic respiratory failure has been studied in RCT and has shown promise. There is evidence for surfactant deficiency in some patients with PPHN (240, 241) Surfactant therapy substantially reduces mortality and respiratory morbidity for this population Surfactant medications can decrease the risk of death for very low- birth-weight infants who are hospitalized by 30%. Such small premature infants may remain ventilated for months. A study shows that an aerosol of a perfluorocarbon such as perfluoromethyldecalin can reduce inflammation in swine model of IRDS

Dr. Leibel created a model using induced pluripotent stem cells, or embryonic stem cells, and differentiated them into three-dimensional lung organoids. She's using these models to test a possible surfactant protein b deficiency treatment Focusing on immunomodulation and aggressive anti-inflammatory therapy, a triple-drug combination was chosen with hydroxychloroquine, azithromycin, and methylprednisolone pulse based on sporadic success in surfactant protein C deficiency (4) Developed by renowned radiologists in each specialty, STATdx provides comprehensive decision support you can rely on - Surfactant Deficiency Disease. link. Bookmarks (0) Pediatrics. Diagnosis. Chest. Neonatal Chest Issues. Surfactant Deficiency Disease. Respiratory distress syndrome (RDS) is defined as respiratory difficulty starting shortly after birth, commonly in a preterm newborn, and is due to deficiency of pulmonary surfactant. It occurs in 15-30% of those between 32 and 36 weeks of gestational age, in about 5% beyond 37 weeks and rarely at term

Surfactant Deficiency chILD Foundatio

Surfactant deficiency, dysfunction, or inactivation. 14. Exogenous surfactant replacement therapy was successfully used to treat what? Neonatal RDS. 15. Surfactant replacement therapy is also researching and investigating other patient populations like? ARDS, asthma and cystic fibrosis. 16. What is the most abundant component of surfactant The best treatment option for an infant ultimately depends on gestational age, clinical status, and the experience of the clinician. The differences in surfactants are minor given the low rates of complications with any surfactant treatment. The clinical management of RDS is the greatest success story in neonatology Currently, pulmonary surfactant is regarded as standard treatment for children with acute respiratory failure [4, 5].Considering the impact of pulmonary surfactant on adult ARDS patients, a number of studies have explored the clinical benefits of administering pulmonary surfactant to adult patients with ARDS The content of phospholipid, surfactant protein B, and total protein were determined in large aggregate (active) surfactant. At 24 h, surfactant treatment transiently increased surfactant protein.

Secondary surfactant deficiency in neonate

  1. Surfactant replacement with artificial surfactant. This is most effective if started in the first six hours of birth. Surfactant replacement has been shown to reduce the severity of RDS. Surfactant is given as prophylactic (preventive) treatment for some babies at very high risk for RDS. For others it is used as a rescue method
  2. Dani C, Ravasio R, Fioravanti L, et al; Analysis of the cost-effectiveness of surfactant treatment (Curosurf(R)) in respiratory distress syndrome therapy in preterm infants: early treatment compared to late treatment. Ital J Pediatr. 2014 May 240:40. doi: 10.1186/1824-7288-40-40
  3. As surfactant therapy becomes standard practice, patients with RDS can benefit at any age. Surfactant-replacement therapy is, unquestionably, the single most important advance in neonatal medicine of the past 20 years, and it is responsible for the largest decrease in neonatal mortality during that time.1 Since surfactant deficiency was first implicated in neonatal respiratory distress.

This report reviews the following: (1) surfactant synthesis and composition, (2) surfactant functions, (3) clinical states of surfactant deficiency, and (4) the status of surfactant replacement. Therefore, studies of replacement therapy for surfactant deficiency have used complete natural surfactants or derivatives of natural surfactant which contain the defined components of surfactant. The surfactant used in the clinical trial was derived from human amniotic fluid. Two basic different strategies for surfactant treatment of. which surfactant deficiency is the primary factor. On the other hand, suboptimal response (ventilatory index >0.03 or FiO2 >0.3 and MAP >6 cm H2O) seen in some of the surfactant recipients are related to several factors: (a) some degree of early lung injury due to surfactant deficiency occurring before surfactant therapy (27); (b Surfactant replacement therapy (SRT) has a proven role in the treatment of neonatal respiratory distress syndrome and severe meconium aspiration syndrome in infants, and may have a role in the treatment of pediatric patients with ARDS. Although newer delivery mechanisms and strategies are being studied, the classic surfactant administration paradigm consists of endotracheal intubation. INTRODUCTION. Respiratory distress syndrome (RDS), formerly known as hyaline membrane disease, is a common problem in preterm infants. This disorder is caused primarily by deficiency of pulmonary surfactant in an immature lung

Surfactant protein B deficiency in infants with

Surfactant treatment was also evaluated for its effects on hemodynamic, ventilatory, and oxygenation-related variables; the duration of mechanical ventilation, the length of stay in the intensive. Summary Pulmonary surfactant is a complex mixture of specific lipids, proteins and carbohydrates, which is produced in the lungs by type II alveolar epithelial cells. The mixture is surface active and acts to decrease surface tension at the air-liquid interface of the alveoli. The presence of such molecules with surface activity had been suspected since the early 1900s and was finally.

Surfactant metabolism dysfunction - Wikipedi

Neonatal respiratory distress syndrome (NRDS), or surfactant deficiency disorder, is a lung disorder in infants that is caused by a deficiency of pulmonary surfactant. It is most common in preterm infants , with the incidence and severity decreasing with gestational age Pulmonary surfactant is a complex mixture of lipids and specific proteins that stabilizes the alveoli at the end of expiration. Mutations in the gene coding for the triphosphate binding cassette transporter A3 (ABCA3), which facilitates the transfer of lipids to lamellar bodies, constitute the most frequent genetic cause of severe neonatal respiratory distress syndrome and chronic interstitial. Lindsay C. Johnston, MD, MEd & Christie J. Bruno, DO146NF0

Two basic strategies for surfactant replacement have emerged: prophylactic or preventive treatment, in which surfactant is administered at the time of birth or shortly thereafter to infants who are at high risk for developing RDS from surfactant deficiency; and rescue or therapeutic treatment, in which surfactant is administered after the. Introduction. Surfactant-replacement therapy with animal-derived surfactant preparations is an established treatment modality for respiratory distress syndrome that revolutionized the care of preterm babies in intensive care units.1,2 Pulmonary surfactant is a complex mixture of phospholipids and at least four apoproteins that reduces surface tension at the alveolar surface.3 The mixture has. Respiratory distress syndrome (RDS) is primarily due to surfactant deficiency in preterm infants. Surfactant treatment is the standard of care in preterm neonates with RDS. Surfactant therapy has been shown to decrease pneumothorax and improve newborn and infant survival.1 Timing of surfactant..

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NRDS is caused by the deficiency of pulmonary surfactants present in the alveolar spaces of the lungs. Pulmonary surfactants are slimy, mucus-like substances made of proteins and lipids. The surfactant lines the air sacs (alveoli) to reduce the surface tension, thus helping the lungs stay inflated and preventing their collapse SURFACTANT : DEFICIENCY 16. PRESSURE VOLUME LOOP 17. •There is no indication that exogenously administered surfactant inhibits the synthesis and secretion of endogenous surfactant •Two major benefits result from surfactant treatment: - The biophysical effects of the surfactant on the surfactant-deficient lungs - And the provision of. Objective: To evaluate lung transplantation for treatment of surfactant protein B (SP-B) deficiency. Study design: We compared surfactant composition and function from pretransplantation and posttransplantation samples of bronchoalveolar lavage fluid, somatic and long growth, neurodevelopmental progress, pulmonary function, and pulmonary immunohistology in 3 infants with SP-B deficiency who. The lack of consistency in the results with surfactant replacement may reflect the changing pathogenesis of BPD and the multiplicity of factors involved among which surfactant deficiency is only one. AB - Bronchopulmonary dysplasia (BPD) is the most common respiratory complication in preterm infants who survive prolonged mechanical ventilation

If a unit dose of aerosolized surfactant for treatment of surfactant deficiency in a 1 kg neonate is 40 mg (e.g. 1.0 ml of a 40 mg/ml liquid surfactant composition), the method of the present invention using a vibrating aperture-type aerosol generator with a flow rate of 0.4 ml/min will generate 90% of the unit dose in less than 3 minutes. To provide guidance on surfactant administration in preterm and term neonates who either present with, or are at high risk of developing, surfactant deficiency respiratory distress syndrome. The document outlines the indications, dosage, formulation and procedure for surfactant administration & timing/strategies for surfactant use

Surfactant deficiency is a recognized cause of respiratory distress syndrome in the preterm neonate. Secondary surfactant deficiency also contributes to acute respiratory morbidity in late-preterm and term neonates with meconium aspiration syndrome, pulmonary haemorrhage, and pneumonia/sepsis A treatment plan may include injecting heparin into your skin for the first week, and then taking an oral medication after the first week. What's the outlook? Protein C deficiency isn't common Surfactant therapy for primary surfactant deficiency Surfactant therapy for RDS in the preterm newborn Surfactant synthesis starts early in fetal life and increases with gestational age. Over the last 10 years, meta-analyses have confirmed that exogenous surfactant treatment decreases overall morbidity and mortality in preterm newborns with RDS.

Hydroxychloroquine and Surfactant Protein C Deficiency NEJ

But surfactant protein B deficiency may cause RDS in term infants as well. Administration of natural surfactant produce is well known to a rapid improvement in oxygenation within 15 to 20 minutes shown to improve lung transplant function, but the effect is variable. We investigated whether SP-A enrichment of surfactant improved the efficacy of surfactant treatment in lung transplantation. Methods. Left and right lungs of Lewis rats, inflated with 50% O2, were stored for 20 hr at 8°C. Surfactant in bronchoalveolar lavage fluid from right lungs was investigated after storage (n=6). Left. The presentation, treatment and prognosis is variable depending on the surfactant associated protein that is deficient. Newborns with unexplained respiratory distress or failure and older children with unclear chronic respiratory insufficiency, especially in the context of a family history of lung disease, should be evaluated for surfactant. Surfactant administration catheter for the treatment of premature or full-term high-risk neonates or with respiratory distress syndrome caused by pulmonary surfactant deficiency. (hyaline membrane disease - HMD).. Surfcath™ is a PEBA catheter of 20 cm long, with an outer diameter of 6Fr and a centimeter marking to guide insertion

PPT - RESPIRATORY DISTRESS SYNDROME PowerPoint

Background: Surfactant replacement therapy is an established modality of treatment in preterm neonates with respiratory distress syndrome. In addition, there are various neonatal respiratory disorders which are characterized by surfactant deficiency in which surfactant therapy can be a feasible and safe option Surfactant therapy is a treatment for respiratory distress syndrome and the primary therapy to address an underlying surfactant deficiency. Current delivery of animal-derived surfactant in these infants is highly invasive by way of endotracheal intubation frequently along with mechanical ventilation, each of which may result in serious.

Unless there is evidence of secondary surfactant deficiency, surfactant treatment has not been recommended in cases of BPD exacerbation. Despite the fact that surfactant abnormalities in ARDS are not the essential pathogenic variables, surfactant insufficiency may result from primary or secondary inactivation of pulmonary surfactant in the. Title:Genetic Disorders of Surfactant Deficiency and Neonatal Lung Disease VOLUME: 15 ISSUE: 3 Author(s):Maria Papale, Giuseppe Fabio Parisi*, Amelia Licari, Raffaella Nenna and Salvatore Leonardi Affiliation:Department of Clinical and Experimental Medicine, University of Catania, Catania, Department of Clinical and Experimental Medicine, University of Catania, Catania, Department of. The targeted treatment for surfactant deficiency is intratracheal surfactant replacement therapy via an endotracheal tube. Surfactant administered within 30 to 60 minutes of the birth of a premature neonate is found to be beneficial. Surfactant hastens recovery and decreases the risk of pneumothorax, interstitial emphysema, intraventricular. Treatment of pulmonary surfactant deficiency. A clinical and experimental study. / Oetomo, Sidarto Bambang. [S.n.], 1988. 131 p. Research output: Thesis › Thesis fully internal (DIV Respiratory distress syndrome is caused by pulmonary surfactant deficiency in the lungs of neonates, most commonly in those born at < 37 weeks gestation. Risk increases with degree of prematurity. Symptoms and signs include grunting respirations, use of accessory muscles, and nasal flaring appearing soon after birth

Surfactant Replacement Therapy for Respiratory Distress

  1. istered intratracheally, followed by mechanical ventilation. In recent years, the growing interest in noninvasive ventilation has led to novel approaches of ad
  2. Surfactant Replacement Therapy (SRT), which involves instillation of a liquid-surfactant mixture directly into the lung airway tree, is a major therapeutic treatment in neonatal patients with.
  3. istration appears to favourably change the haemodynamics of the lungs and may be a potentially promising therapy for severe bronchiolitis. This is an update of a review published in Issue 9, 2012
  4. al respiratory units leading to atelectasis, increased ventilation-perfusion mismatch, and potential lung injury due to a marked pulmonary inflammatory response
  5. istration (LISA) aims to provide an adequate dose of surfactant while the.

Respiratory distress syndrome Radiology Reference

  1. Surfactant deficiency. Alveoli contain a substance called surfactant that helps them stay open. When there is too little of it, the alveoli collapse. This treatment is commonly used in people.
  2. Deficiencies of surfactant components are classically observed in the neonatal respiratory distress syndrome, where surfactant replacement therapies have been the mainstay of treatment. However, functional or compositional deficiencies of surfactant are also observed in a variety of acute and chronic lung disorders
  3. US7201167B2 US11/080,279 US8027905A US7201167B2 US 7201167 B2 US7201167 B2 US 7201167B2 US 8027905 A US8027905 A US 8027905A US 7201167 B2 US7201167 B2 US 7201167B2 Authority US
  4. Surfactant B deficiency is generally not responsive to surfactant therapy, although some patients may show some initial response. Hamvas et al. (1997) reported successful treatment of surfactant B deficiency with lung transplantation in 2 affected infants
  5. The research resulting in the understanding of surfactant metabolism and function and subsequent treatment of RDS is a highlight of progress from science to cure strategies in pulmonary medicine., keywords = ABCA3 deficiency, Antenatal corticosteroids, Induced lung maturation, Oxygenation, Pressure-volume curve, Respiratory distress syndrome.
  6. ate surface tension of the air tissue interfaces'. 1 After expanding the lung with air and liquid, he concluded that 'a lower surface tension would be useful for the respiratory mechanism' and that.
  7. Surfactant protein A2 (SP-A2) plays an essential role in surfactant metabolism and lung host defense. SP-A2 mutations in the carbohydrate recognition domain have been related to familial pulmonary fibrosis and can lead to a recombinant protein secretion deficiency in vitro. In this study, we explored the molecular mechanism of protein secretion deficiency and the subsequent biological effects.

Surfactant Worth Studying as Treatment for COVID-19

  1. With treatment, most newborns survive. Natural production of surfactant increases after birth. With continued production of surfactant and sometimes with breathing support and synthetic surfactant therapy (see Treatment below), respiratory distress syndrome usually resolves within 4 or 5 days
  2. factant deficiency and action of sur-factant inhibitors. (4-17) After birth, the lungs of neonates are still full of fluid, and functionally and morphologi-cally immature. They have low func-tional residual capacity and insufficient amount of surfactant. (4-17) Surfactant Treatment Pulmonary surfactant used as a medicinal product is a natural.
  3. Objective To assess whether late surfactant treatment in extremely low gestational age (GA) newborn infants requiring ventilation at 7-14 days, who often have surfactant deficiency and dysfunction, safely improves survival without bronchopulmonary dysplasia (BPD). Study design Extremely low GA newborn infants (GA ≤28 0/7 weeks) who required.
  4. This pattern is more similar to neonatal respiratory distress syndrome (RDS) secondary to surfactant deficiency, which has been shown to benefit from exogenous surfactant. We present our experience with exogenous surfactant treatment in a COVID-19 patient suffering from COVID-19 related ARDS
  5. The value of surfactant replacement in models of acute lung injury other than quantitative surfactant deficiency states is, however, uncertain. In this study an acute lung injury model using rats with chronic indwelling arterial catheters, injured with N-nitroso-N-methylurethane (NNNMU), has been developed
  6. Knowledge of ARDS and surfactant pathobiology has significantly advanced during recent years, and new concepts have been introduced, such as the role of secretory phospholipase A2 enzymes in the surfactant catabolism [11, 46], the vicious cycle connecting surfactant catabolism and inflammation , the deficiency and injury of surfactant proteins.

After the initial phase of surfactant deficiency of the very preterm infant, inhibition and destruction of surfactant are common, the inflammation of the oxygen exposed preterm lung is probably important in the pathophysiology of bronchopulmonary dysplasia, and the adverse effects on surfactant function have led to trials of later surfactant supplementation, in the hope tha Surfactant protein B (SPB) deficiency is a rare but fatal disease that affects full-term babies after an apparently uncomplicated pregnancy and delivery. To date the only effective treatment. This review documents the evolution of surfactant therapy, beginning with observations of surfactant deficiency in respiratory distress syndrome, the basis of exogenous surfactant [ncbi.nlm.nih.gov] Early treatment with nasal continuous positive airway pressure probably mitigated the development of RDS in some infants with a low-degree. Hyaline Membrane Disease (HMD) or as known in common terms Respiratory Distress Syndrome (RDS) or Surfactant Deficiency Disorder is one of the most common problems that arise in premature infants or new-borns owing to the need of more oxygen to breathe Surfactant deficiency in immature lungs triggers a cascade of alveolar instability and collapse, capillary leak edema, and hyaline membrane formation. The term respiratory distress syndrome (RDS) has come to represent the clinical expression of surfactant deficiency and its nonspecific histologic counterpart, hyaline membrane disease.

Neonatal respiratory distress syndrome (NRDS) is a disorder of surfactant deficiency, especially preterm infants <32 weeks old with very low birth weight whose type II pneumocytes are too immature to produce sufficient surfactant to support normal alveolar expansion . Treatment includes respiratory support and exogenous surfactant Prophylactic treatment with human surfactant also substantially reduced the period of neonatal intensive care. We conclude that treatment with human surfactant offers promise for improving the survival of very premature infants with a surfactant deficiency and for reducing the pulmonary sequelae of the respiratory distress syndrome Deficiency in ATP binding cassette A3 (ABCA3) causes neonatal respiratory distress, hypoxemic respiratory failure, and interstitial lung disease. ABCA3 transports phospholipids into the lamellar bodies of type II alveolar cells, a critical step in alveolar surfactant production

Is Treatment With Hydroxychloroquine Effective in

Pulmonary surfactant is an endogenous substance produced in the lungs that functions to decrease surface tension at the air:fluid interface on the alveolar surface. In premature infants with pulmonary surfactant deficiency, surface tension can increase to the point where sections of lung collapse and respiratory distress syndrome (RDS) develops Treatment and prognosis. Treatment depends on the severity of the protein C deficiency. In severe cases of protein C deficiency, protein C concentrate and fresh frozen plasma has historically been used. However, some cases are now treated with novel oral anticoagulants (e.g. rivaroxaban) 5

Surfactant for the Treatment of ARDS in a Patient With

Natural surfactant is currently recommended for use, but synthetic surfactant that contains proteins to mimic surfactant proteins is being investigated. In general, prophylactic use of surfactant is recommended over rescue treatment in infants at high risk for developing RDS, but the determination of which infants are at high risk for. @article{osti_20634852, title = {Cell-specific modulation of surfactant proteins by ambroxol treatment}, author = {Seifart, Carola and Clostermann, Ursula and Seifart, Ulf}, abstractNote = {Ambroxol [trans-4-(2-amino-3,5-dibromobenzylamino)-cyclohexanole hydrochloride], a mucolytic agent, was postulated to provide surfactant stimulatory properties and was previously used to prevent surfactant. The infants with initial values of static Crs suggesting surfactant deficiency (< 1 *8 ml/cm H20/m) who received Exosurf had slightly greater ventilation requirements (VEI) before treatment than those treated with Curosurf. There were 15 infants whose static Crs before treatment was not consistent with surfactant deficiency (-1-8 mi/cm H2O/m) Exosurf Neonatal (colfosceril palmitate) is a synthetic lung surfactant that was approved for use in August of 1990 by the Food and Drug Administration (FDA) for treatment of infant respiratory distress syndrome. The treatment consists of a single dose given 30 minutes after birth to high-risk infants N2 - We present the case of two twin brothers with surfactant protein C deficiency who were treated with hydroxychloroquine for three years, with apparent success. The exact physiopathology of this disease is not known and there is no specific treatment for it

CiteSeerX - Document Details (Isaac Councill, Lee Giles, Pradeep Teregowda): Surfactant treatment is given on the assumption that surfactant deficiency is the major cause of respiratory distress syndrome (RDS). However, we know that babies with RDS are also very immature. They have a number of interrelated problems which are difficult to separate from sur-factant deficiency: difficulty.

Surfactant replacement therapy : RDS & beyondSecondary Surfactant Deficiency in Neonates | Journal ofHyaline Membrane Disease: Causes, SymptomsRespiratory distress syndrome due to a novel homozygous
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