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Neonatal Respiratory Distress Syndrome

Hyaline membrane disease

Neonatal respiratory distress syndrome, also known as hyaline membrane disease, is a condition due to insufficient surfactant synthesis and secretion, that characterizes prematurely born infants. Consequences such as atelectasis, abnormal ventilation-perfusion ratio, decreased oxygenation with consecutive hypoxia and acidosis are seen.


Presentation

Neonatal respiratory distress syndrome (NRDS) more frequently affects the Caucasian boys, especially born to diabetic mothers by cesarean section, second born twins and children with a positive family history. On the other hand, maternal hypertension, antenatal steroid administration and prolonged membrane rupture seem to act as protective factors. Keeping this in mind, mothers should be counseled upon discharge in case they ever want to have another child.

The more prematurely the baby is born, the higher the chances of suffering from this ailment are. However, late prematurity is also associated with this disease [1]. The problem becomes apparent soon after birth, progressively worsens and its signs consist of tachypnea, increased respiratory effort with intercostal, suprasternal and substernal retractions, flaring of the nasal alae, expiratory grunting and cyanosis. In extreme situations, irregular breathing, apnea, and hypothermia are encountered. The consciousness state may be diminished and severe fatigability may be reported. In advanced cases, the patient requires ventilatory support and intensive care [2] [3].

Clinical examination reveals decreased breath sounds and peripheral pulses. Oliguria and peripheral edema can be encountered.

The surfactant deficiency may be secondary to several pathological processes, such as infectious or meconium aspiration pneumonia or pulmonary hemorrhage, intrapartum asphyxia, pulmonary hypoplasia or oxygen toxicity. Furthermore, the newborn may have other associated diseases, such as congenital diaphragmatic hernia, necrotizing enterocolitis or patent ductus arteriosus.

Recurrent Respiratory Infections
  • The infant presented at 23 months with a history of developmental delay, hyperkinesia, recurrent respiratory infections, neonatal respiratory distress, and hypothyroidism.[ncbi.nlm.nih.gov]
Pneumonia
  • However, surfactant therapy has been shown to be effective in the treatment of other diseases causing neonatal respiratory diseases such as pulmonary hemorrhage, meconium aspiration syndrome, pneumonia/sepsis, pulmonary edema or acute lung injury resulting[ncbi.nlm.nih.gov]
  • In such instances, we recommend that the diagnosis of BA pneumonia be kept in mind and that exogenous surfactant be given as soon as possible, even in the presence of indices of normal lung maturity in the amniotic fluid.[ncbi.nlm.nih.gov]
  • The surfactant deficiency may be secondary to several pathological processes, such as infectious or meconium aspiration pneumonia or pulmonary hemorrhage, intrapartum asphyxia, pulmonary hypoplasia or oxygen toxicity.[symptoma.com]
  • Pulmonary infection - eg group B beta-haemolytic streptococcal pneumonia. Pulmonary haemorrhage. Meconium aspiration pneumonia. Oxygen toxicity along with pressure or volume trauma to the lungs.[patient.info]
Tachypnea
  • The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts.[orpha.net]
  • Transient tachypnea of the newborn ( wet lung disease ) Congenital diaphragmatic hernia Pneumothorax Meconium aspiration syndrome : Neonates with meconium aspiration are usually post-term rather than preterm children .[amboss.com]
  • Symptoms manifest shortly after birth and include tachypnea, tachycardia, increased breathing effort, and/or cyanosis. The diagnosis is suspected based on clinical features and confirmed by evaluating the extent of atelectasis via chest x-ray.[amboss.com]
  • The problem becomes apparent soon after birth, progressively worsens and its signs consist of tachypnea, increased respiratory effort with intercostal, suprasternal and substernal retractions, flaring of the nasal alae, expiratory grunting and cyanosis[symptoma.com]
Nasal Flaring
  • The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts.[orpha.net]
  • There are 3 classic symptoms associated with respiratory distress syndrome: Intracostal retractions as the infant uses accessory muscles to support the work of ventilation Grunting as the epiglottis attempts to keep air in the lungs Nasal flaring as the[sharecare.com]
  • flaring ) ; episodes of desaturation Diagnostics Therapy : controlled oxygenation ; , high-calorie food, diuretics , consider glucocorticoids References: [4] [1] [8] [9] We list the most important complications.[amboss.com]
Rales
  • […] within minutes of birth may not be recognized for several hours in larger preterm  Tachypnea ( 60 breaths/min), nasal flaring, subcostal and intercostal retractions, cyanosis & expiratory grunting  Breath sounds may be normal or diminished and fine rales[slideshare.net]
  • Breath sounds can be clear or reveal rales on auscultation.[aafp.org]
Pulmonary Disorder
  • Respiratory Distress Syndrome of the Newborn Hyaline Membrane Disease General Considerations Acute pulmonary disorder of the newborn characterized by Generalized atelectasis Intrapulmonary shunting Ventilation-perfusion abnormalities Reduced lung compliance[learningradiology.com]
Cyanosis
  • A condition of the newborn marked by dyspnea with cyanosis, heralded by such prodromal signs as dilatation of the alae nasi, expiratory grunt, and retraction of the suprasternal notch or costal margins, most frequently occurring in premature infants,[icd9data.com]
  • […] neonatal respiratory distress syndrome a condition of the newborn marked by dyspnea with cyanosis, preceded by symptoms such as dilatation of the nostrils, grunting on exhalation, and retraction of the suprasternal notch or costal margins.[web.archive.org]
  • The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts.[orpha.net]
  • Later signs of the infant's deteriorating condition include a change in the respiratory rate and apnea and the presence of cyanosis, especially around the oral area.[sharecare.com]
  • Definition (NCI_FDA) A condition of the newborn marked by dyspnea with cyanosis, most frequently occurring in premature infants, children of diabetic mothers and infants delivered by cesarean section, and sometimes with no predisposing cause.[fpnotebook.com]
Tachycardia
  • The symptoms usually appear shortly after birth and may include tachypnea, tachycardia, chest wall retractions (recession), expiratory grunting, nasal flaring and cyanosis during breathing efforts.[orpha.net]
  • Symptoms manifest shortly after birth and include tachypnea , tachycardia , increased breathing effort, and/or cyanosis . The diagnosis is suspected based on clinical features and confirmed by evaluating the extent of atelectasis via chest x-ray .[amboss.com]
  • Symptoms manifest shortly after birth and include tachypnea, tachycardia, increased breathing effort, and/or cyanosis. The diagnosis is suspected based on clinical features and confirmed by evaluating the extent of atelectasis via chest x-ray.[amboss.com]
Oliguria
  • Oliguria and peripheral edema can be encountered.[symptoma.com]
  • These manifestations progress to apnea, flaccidity, unresponsiveness, mottling, peripheral edema, oliguria, hypotension, and bradycardia.[medical-dictionary.thefreedictionary.com]
  • ., seizures, oliguria) 36. Pathophysiology 37. Chest x-ray: Areas of hyperexpansion mixed with patchy densities and atelectasis 38.[slideshare.net]
Cervical Cyst
  • Magnetic resonance imaging confirmed a left cervical cyst displacing the trachea and esophagus laterally. Surgical excision was performed via a cervical approach on the 5th day, and pathological examination revealed a bronchogenic cyst.[ncbi.nlm.nih.gov]
Cervical Incompetence

Workup

An amniocentesis is able to predict if NRDS will occur after birth, based on the lecithin-to-sphingomyelin ratio the presence of phosphatidylglycerol in the amniotic fluid. If these tests are not available, birth is in progress at the time the mother comes to the hospital or fetal distress is seen and childbirth is required, the diagnosis is made after the delivery is done.

Once respiratory distress is noticed, the physician should determine its severity and arterial blood gases offer a good measurement tool. Central (umbilical) or peripheral arterial blood should be analyzed [4] and may show hypoxemia, hypercapnia and respiratory (caused by alveolar atelectasis) and metabolic (due to lactic acid accumulation) acidosis. If a congenital right to left shunt heart malformation coexists, hypoxia will be severe and the child is likely to be cyanotic. A patent ductus arteriosus can have the same results. Blood workup should include a blood glucose level, that is often low, a complete cell count, bilirubin level, renal and liver function and electrolytes.

Oxygenation may also be monitored using a less invasive method, pulse oximetry. Probes should be placed on the right wrist or palm in order to establish the preductal saturation and on either foot for postductal saturation. Saturation should be kept at 91-95% [5]. This is a good screening technique for congenital heart disease, but the diagnosis should finally be evaluated by echocardiography in selected cases.

A thoracic radiography is done in order to observe the pulmonary aspect. Pleural effusion, atelectasis or air bronchograms, interstitial emphysema, pneumothorax, bronchogenic cyst or pulmonary sequestration may be described by this exam. Cardiomegaly is often found and further characterization by echocardiography is required. Doppler echocardiography is reliable in determining if pulmonary hypertension is present. Hyperinflation (in a non-ventilated patient) or a normal aspect at 6 hours of age exclude the NRDS.

Pulmonary ultrasonography may be a reliable method [6] [7] that visualizes the existing pleural effusions [8]. However, pneumothorax is harder to find by lung ultrasonography [9].

If infection signs are detected, tracheal aspirate, cerebrospinal fluid and blood cultures should be obtained.

In situations where the outcome is unfavorable, a histology evaluation will describe the presence of hyaline membranes - an amorphous material, lining the alveolar spaces, collapsed alveoli alternating with hyper aerated ones and vascular congestion.

Bilateral Atelectasis
  • atelectasis (grade IV); (5) Arterial blood gas analysis showing hypoxia, hypercapnia and oxygen tension/fraction of inspired oxygen ratio (PaO 2 /FiO 2 ) 26.7 kPa.[omicsgroup.org]
Hypercapnia
  • Central (umbilical) or peripheral arterial blood should be analyzed and may show hypoxemia, hypercapnia and respiratory (caused by alveolar atelectasis) and metabolic (due to lactic acid accumulation) acidosis.[symptoma.com]
  • Surfactant deficiency : little or no reduction of alveolar surface tension reduced pulmonary unfolding atelectasis decreased lung compliance and functional residual capacity hypoxemia and hypercapnia References: [1] [3] Clinical features History of premature[amboss.com]
  • Surfactant deficiency little or no reduction of alveolar surface tension reduced pulmonary unfolding atelectasis decreased lung compliance and functional residual capacity hypoxemia and hypercapnia References: [1] [3] Clinical features History of premature[amboss.com]
  • Anoxia and hypercapnia cause acidosis, leading to peripheral vasodilatation and pulmonary vasoconstriction. This situation, in turn, leads to the reestablishment of a partial fetal circulatory pattern.[histopathology-india.net]
  • Clinical evaluation ABG (hypoxemia and hypercapnia) Chest x-ray Blood, CSF, and tracheal aspirate cultures Diagnosis of RDS is by clinical presentation, including recognition of risk factors; ABGs showing hypoxemia and hypercapnia; and chest x-ray.[msdmanuals.com]

Treatment

  • Sixty babies were recruited, with 29 in the bLES and 31 in the Survanta treatment group.[ncbi.nlm.nih.gov]
  • Curosurf for “early treatment” (the new treatment) was donated by Serono Nordic, Copenhagen, Denmark.[doi.org]
  • […] and treatment with surfactant.[doi.org]
  • Treatment with digoxin has no proven value in infants solely affected with respiratory distress syndrome.[ncbi.nlm.nih.gov]
  • Inhaled nitric oxide may reduce oxygen requirements by reducing ventilation-perfusion mismatching, and early treatment with corticosteroids may reduce pulmonary inflammation. All of these treatments are currently undergoing clinical trials.[ncbi.nlm.nih.gov]

Prognosis

  • […] ground-glass” opacification of both lungs with air bronchograms and hypoaeration Hypoaeration from loss of lung volume (may be counteracted by respiratory therapy) Fine granular pattern Prominent air bronchograms Bilateral and symmetrical distribution Prognosis[learningradiology.com]
  • The prognosis is much better for babies weighing over 1500 g. Prevention Antenatal corticosteroids (dexamethasone) accelerate fetal surfactant production and lung maturation.[patient.info]
  • Prognosis : guarded. Print off the Owner factsheet on Respiratory problems in your horse - not a good wheeze! to give to your clients. Pathogenesis Etiology Infection EHV1 Equine herpesvirus or EVA Equine viral arteritis virus (less common).[vetstream.com]

Etiology

  • Etiology Pathophysiology Surfactant : Pulmonary surfactant is a mixture of phospholipids and proteins produced by lamellar bodies of type II alveolar cells .[amboss.com]
  • Etiology Impaired synthesis and secretion of surfactant Risk factors Premature birth Genetic predisposition Scheduled cesarean section Maternal diabetes mellitus Hydrops fetalis Multifetal pregnancies Rarely, hereditary References: [1] Pathophysiology[amboss.com]
  • Pathogenesis Etiology Infection EHV1 Equine herpesvirus or EVA Equine viral arteritis virus (less common). Prematurity/dysmaturity Reproduction: prematurity / dysmaturity. Stress at parturition.[vetstream.com]
  • Pathogenesis Etiology Infection EHV1 Equine herpesvirus or EVA Equine viral arteritis virus (less common). Prematurity/dysmaturity Reproduction: prematurity / dysmaturity . Stress at parturition.[vetstream.com]
  • The etiology, pathogenesis and methods of preventing and treating near-term and term infants with RDS have attracted increased attention.[nature.com]

Epidemiology

  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Epidemiology The incidence and severity are related inversely to the gestational age of the infant. It affects approximately one half of infants born at 28-32 weeks of gestation. It may (rarely) occur at term.[patient.info]
  • Hjalmarson O (1981) Epidemiology and classification of acute, neonatal respiratory disorders. A prospective study. Acta Paediatr Scand 70:773–783 PubMed Google Scholar 19.[link.springer.com]
Sex distribution
Age distribution

Pathophysiology

  • […] groups, the GT genotype and A allele were less frequent among the RDS groups in 26-32.9 weeks of gestational age and in a birth weight subgroup of CONCLUSION: Our study raises the possibility that a genetic variation of NOS3 could be implicated in the pathophysiology[ncbi.nlm.nih.gov]
  • The initial management of infants with RDS has almost become 'too routine' with little thought about the pathophysiological processes that lead to the disease and how the clinician can use the existing therapeutic interventions to optimize care.[ncbi.nlm.nih.gov]
  • The exact pathophysiological mechanism responsible for IMg increase, and whether determination of plasma IMg level, including umbilical cord blood IMg measurement, can be used as an early or predictive indicator of RDS in the diagnosis remain to be determined[ncbi.nlm.nih.gov]
  • Historical Perspective: Failure of Oxygen Treatment Before discussing the pathophysiology of RDS and the current modalities of treatment, it is useful first to consider why the historical mode of treatment, administration of oxygen, was ...[accessmedicine.mhmedical.com]
  • Pathophysiology Prematurity/dysmaturity Insufficient surfactant incomplete inflation (atelectasis) of the lungs increased respiratory effort and distress increased susceptibility to pneumonia.[vetstream.com]

Prevention

  • Calfactant (Infasurt) is a natural bovine surfactant which has been evaluated for intratracheal use in the prevention and rescue treatment of respiratory distress syndrome (RDS) in preterm infants.[ncbi.nlm.nih.gov]
  • Ambroxol has been studied as a potential agent to prevent RDS, but effectiveness and safety has yet to be evaluated.[ncbi.nlm.nih.gov]
  • SELECTION CRITERIA: Randomized controlled trials of digoxin in either the prevention or treatment of respiratory distress syndrome are included in this overview.[ncbi.nlm.nih.gov]
  • SELECTION CRITERIA: Randomized and quazi-randomized controlled trials of digoxin in either the prevention or treatment of RDS are included in this overview.[ncbi.nlm.nih.gov]
  • Strong evidence exists for the role of antenatal steroids in RDS prevention, but it is not clear if repeated courses are safe.[ncbi.nlm.nih.gov]

References

Article

  1. Wang ML, Dorer DJ, Fleming MP, et al. Clinical outcomes of near-term infants. Pediatrics. 2004;114:372-6.
  2. Escobar GJ, Clark RH, Greene JD. Short-term outcomes of infants born at 35 and 36 weeks’ gestation: we need to ask more questions. Semin Perinatol. 2006;30:28-33.
  3. Jain L, Eaton DC. Physiology of fetal lung fluid clearance and the effect of labor. Semin Perinatol. 2006;30:34-43
  4. Billman GF, Hughes AB, Dudell GG, et al. Clinical performance of an in-line, ex vivo point-of-care monitor: a multicenter study. Clin Chem. 2002;48(11):2030-43.
  5. Tarnow-Mordi W, Stenson B, Kirby A, et al. Outcomes of Two Trials of Oxygen-Saturation Targets in Preterm Infants. N Engl J Med. 2016; 374(8):749-60.
  6. Liu J, Cao HY, Liu Y. Lung ultrasonography for the diagnosis of neonatal respiratory distress syndrome: a pilot study. Zhonghua Er Ke Za Zhi. 2013; 51(3):205-10.
  7. Vergine M, Copetti R, Brusa G, et al. Lung ultrasound accuracy in respiratory distress syndrome and transient tachypnea of the newborn. Neonatology. 2014;106(2):87-93.
  8. Ahuja CK, Saxena AK, Sodhi KS, et al. Role of transabdominal ultrasound of lung bases and follow-up in premature neonates with respiratory distress soon after birth. Indian J Radiol Imaging. 2012;22(4):279-83.
  9. Sawires HK, Abdel Ghany EA, Hussein NF, et al. Use of lung ultrasound in detection of complications of respiratory distress syndrome. Ultrasound Med Biol. 2015;41 (9):2319-25.

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Last updated: 2019-07-11 19:59