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Newborn Respiratory Distress Syndrome

Disease Details

Family Health Simplified

Description
Newborn respiratory distress syndrome (NRDS) is a condition primarily affecting premature infants, where the lungs are insufficiently developed to produce enough surfactant, leading to breathing difficulties.
Type
Newborn Respiratory Distress Syndrome (NRDS), also known as neonatal respiratory distress syndrome or hyaline membrane disease, is not typically classified as a genetic disorder and does not follow a specific pattern of genetic transmission. Instead, it is primarily caused by the insufficient production of surfactant in the lungs, which is more common in premature infants. Environmental and developmental factors play a significant role in the occurrence of this condition.
Signs And Symptoms
IRDS begins shortly after birth and is manifested by fast breathing (more than 60 breaths per minute), a fast heart rate, chest wall retractions (recession), expiratory grunting, nasal flaring, and blue discoloration of the skin during breathing efforts.As the disease progresses, the baby may develop ventilatory failure (rising carbon dioxide concentrations in the blood) and prolonged cessations of breathing ("apnea"). Whether treated or not, the clinical course for the acute disease lasts about two to three days. During the first day, the child worsens and requires more support. During the second day, the baby may be remarkably stable on adequate support and resolution is noted during the third day, heralded by a prompt diuresis. Despite huge advances in care, IRDS remains the most common single cause of death in the first month of life in the developed world. Complications include metabolic disorders (acidosis, low blood sugar), patent ductus arteriosus, low blood pressure, chronic lung changes and bleeding in the brain. The syndrome is frequently complicated by prematurity and its additional effect on other organ functions.
Prognosis
Newborn respiratory distress syndrome (NRDS), primarily affecting premature infants, has a variable prognosis dependent on factors such as gestational age, birth weight, and the promptness of medical intervention. With advances in neonatal care, including the use of surfactant therapy and mechanical ventilation, survival rates have significantly improved. However, more severe cases can lead to complications such as chronic lung disease or neurodevelopmental impairments. Early and aggressive treatment is crucial for a favorable outcome.
Onset
Newborn respiratory distress syndrome (NRDS) typically presents within the first few hours after birth.
Prevalence
The prevalence of Newborn Respiratory Distress Syndrome (RDS) varies depending on gestational age and the healthcare setting. It occurs in approximately 1% to 2% of all live births but is significantly higher in premature infants, particularly those born before 28 weeks of gestation, where it can affect up to 60% to 80% of newborns. The incidence decreases as gestational age increases.
Epidemiology
Infant respiratory distress syndrome (IRDS) is the leading cause of death in premature infants. Despite only 1% of all birth complications being attributed to respiratory distress syndrome, there is a significantly higher prevalence in prematurely born babies. Incidence rates of IRDS in premature infants born at 30 weeks of gestational age (GA) are at 50%, and rise even higher to 93% for infants born prematurely at 28 weeks of gestational age or younger. IRDS is diagnosed within hours of delivery and usually leads to morbidity and mortality in preterm infants. There are many risk factors that can potentially cause IRDS. The most common risks factors that can potentially cause IRDS include male gender, white race, late preterm delivery, maternal diabetes, perinatal hypoxia (exposure to low oxygen) and ischemia (decreased blood flow), and low birth weight. Seventy percent of babies diagnosed with respiratory distress syndrome are born between 29 and 34 weeks of gestational age and are 55% more likely to be male. A study conducted at the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network studied premature infants born between 22 and 37 weeks and the outcomes leading to IRDS. This study was conducted from 2002 to 2008. The incidence rate of IRDS for 24 weeks was 98%, for 34 weeks the incidence is 5%, and for 37 weeks the incidence rate was less than 1%. The results demonstrate that the incidence of IRDS increases with decreasing age at birth.According to a study from the University of Miami's Department of Pediatrics and Division of Neonatology, from the time range of 2003 to 2014, respiratory distress syndrome prevalence jumped from 170 per 1000 preterm live births to 360 per 1000 preterm live births nationwide in the United States. This study population's duration under hospital care averaged 32 days in 2003, increasing by nearly a week to 38 days in 2014. Additionally, this study yielded average prevalence rates of 260 cases per 1000 livebirths from the years of 2003 to 2014, which coincided with the results yielded by a report from the Vermont Oxford Network in 2008 of 300 per 1000 livebirths.
Intractability
Newborn respiratory distress syndrome (NRDS), also known as neonatal RDS, is not considered intractable. It is a common condition, predominantly in premature infants, where the lungs are not fully developed and lack sufficient surfactant, leading to breathing difficulties. With advancements in medical treatment, including surfactant replacement therapy, respiratory support, and other supportive measures, the outcomes for infants with NRDS have significantly improved. Early diagnosis and intervention are key to managing and reducing the severity of the condition.
Disease Severity
Newborn Respiratory Distress Syndrome (NRDS) can vary in severity. Mild cases might require minimal intervention, while severe cases might necessitate mechanical ventilation and intensive care. The severity largely depends on the infant's gestational age, as NRDS is more common and typically more severe in premature infants.

Regarding "nan," if this refers to "not a number," it does not apply directly to the severity of NRDS. If it refers to something else, please provide additional context.
Healthcare Professionals
Disease Ontology ID - DOID:12716
Pathophysiology
The lungs of infants with respiratory distress syndrome are developmentally deficient in a material called surfactant, which helps prevent the collapse of the terminal air spaces (the future site of alveolar development) throughout the normal cycle of inhalation and exhalation. This deficiency of surfactant is related to inhibition from the insulin that is produced in the newborn, especially those of diabetic mothers.Pulmonary surfactant is a complex system of lipids, proteins and glycoproteins that is produced in specialized lung cells called Type II cells or Type II pneumocytes. The surfactant is packaged by the cell in structures called lamellar bodies, and extruded into the air spaces. The lamellar bodies then unfold into a complex lining of the air space. This layer reduces the surface tension of the fluid that lines the alveolar air space. Surface tension is responsible for approximately 2/3 of the inward elastic recoil forces. In the same way that a bubble will contract to give the smallest surface area for a given volume, so the air/water interface means that the liquid surface will tend toward being as small as possible, thereby causing the air space to contract. By reducing surface tension, surfactant prevents the air spaces from completely collapsing on exhalation. In addition, the decreased surface tension allows reopening of the air space with a lower amount of force. Therefore, without adequate amounts of surfactant, the air spaces collapse and are very difficult to expand.Microscopically, a pulmonary surfactant-deficient lung is characterized by collapsed air spaces alternating with hyperexpanded areas, vascular congestion, and, in time, hyaline membranes. Hyaline membranes are composed of fibrin, cellular debris, red blood cells, rare neutrophils and macrophages. They appear as an eosinophilic, amorphous material, lining or filling the air spaces and blocking gas exchange. As a result, blood passing through the lungs is unable to pick up oxygen and unload carbon dioxide. Blood oxygen levels fall and carbon dioxide rises, resulting in rising blood acid levels and hypoxia. Structural immaturity, as manifested by a decreased number of gas exchange units and thicker walls, also contributes to the disease process. Therapeutic oxygen and positive-pressure ventilation, while potentially life-saving, can damage the lung.
Carrier Status
Newborn Respiratory Distress Syndrome (NRDS) is not a condition associated with a carrier status as it is not inherited genetically in this manner. The syndrome primarily occurs due to the insufficient production of surfactant in the lungs of premature infants, leading to breathing difficulties.
Mechanism
Newborn Respiratory Distress Syndrome (NRDS), also known as Neonatal Respiratory Distress Syndrome or Hyaline Membrane Disease, primarily affects premature infants. It is characterized by insufficient surfactant production in the lungs.

**Mechanism:**
Surfactant is a lipid-protein complex that reduces surface tension within the alveoli, preventing their collapse during exhalation. In NRDS, premature infants often have underdeveloped type II alveolar cells, which are responsible for producing surfactant. The deficiency of surfactant leads to increased surface tension, causing alveolar collapse, atelectasis, impaired gas exchange, and resultant hypoxemia.

**Molecular Mechanisms:**
1. **Surfactant Proteins and Lipids:** Surfactant is composed of phospholipids (primarily dipalmitoylphosphatidylcholine) and surfactant proteins (SP-A, SP-B, SP-C, SP-D). In NRDS, these components are underproduced.
- **Phospholipids:** Critical for lowering surface tension.
- **Surfactant Proteins:** SP-B and SP-C are crucial for surfactant spreading and stability. SP-A and SP-D are involved in immune responses.

2. **Gene Regulation and Expression:** The genes responsible for surfactant protein production (such as the SFTPB and SFTPC genes for SP-B and SP-C) may be under-expressed in premature infants. Transcription factors like thyroid transcription factor-1 (TTF-1) play a key role in surfactant protein gene expression.

3. **Hormonal Influence:** Cortisol and thyroid hormones promote surfactant production. Premature infants often have insufficient levels of these hormones, contributing to underdeveloped surfactant systems.

The interplay between genetic regulation, hormonal signals, and the maturity of lung tissue collectively impacts the onset and severity of NRDS.
Treatment
Oxygen is given with a small amount of continuous positive airway pressure (CPAP), and intravenous fluids are administered to stabilize the blood sugar, blood salts and blood pressure. CPAP application to preterm neonates with respiratory distress is associated with a reduction in respiratory failure, mechanical ventilation and mortality. However, CPAP is associated with an increased rate of pneumothorax compared to spontaneous breathing with or without supplemental oxygen. If the baby's condition worsens, an endotracheal tube (breathing tube) is inserted into the trachea and intermittent breaths are given by a mechanical device. An exogenous preparation of pulmonary surfactant, either synthetic or extracted from animal lungs, is given through the breathing tube into the lungs. 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. Chronic lung disease, including bronchopulmonary dysplasia, is common in severe RDS. The etiology of BPD is problematic and may be the result of oxygen, overventilation or underventilation. The mortality rate for babies greater than 27 weeks of gestation is less than 20%.
Compassionate Use Treatment
Newborn Respiratory Distress Syndrome (RDS), primarily affecting premature infants, may necessitate treatments beyond the standard care. For compassionate use or off-label treatments:

1. **Surfactant Therapy:** While surfactants are standard for RDS, different formulations or timing strategies might be considered experimental or off-label.
2. **Inhaled Nitric Oxide:** Primarily approved for term or near-term infants with hypoxic respiratory failure, it might be used off-label in preterm infants to improve oxygenation.
3. **Steroids:** Postnatal steroids, such as dexamethasone or hydrocortisone, may be considered to reduce inflammation and improve lung function, though there is caution due to potential neurodevelopmental side effects.
4. **High-Frequency Oscillatory Ventilation (HFOV):** Although generally a second-line treatment, HFOV might be utilized off-label or experimentally for infants not responding to conventional ventilation.
5. **Stem Cell Therapy:** Still largely in experimental phases, mesenchymal stem cells are being investigated for their potential to promote lung repair and reduce inflammation.
6. **Liquid Ventilation:** This experimental approach uses perfluorocarbon liquids to enhance gas exchange and provide lung protection.

These treatments are typically considered within the context of clinical trials or under compassionate use protocols, with careful consideration of potential benefits and risks.
Lifestyle Recommendations
For newborn respiratory distress syndrome (NRDS), lifestyle recommendations for the infant are not applicable as it is a medical condition affecting premature newborns. Management and care are primarily focused on medical interventions rather than lifestyle changes. This typically includes:

1. **Administering Surfactant:** Artificial surfactant can be given to help the newborn's lungs function better.
2. **Oxygen Therapy:** Supplemental oxygen or mechanical ventilation may be needed to maintain adequate oxygenation.
3. **Temperature Regulation:** Keeping the newborn warm to prevent additional stress on their system.
4. **Monitoring:** Continuous monitoring of vital signs and blood gases to ensure adequate respiratory function.

Care is usually provided in a neonatal intensive care unit (NICU) under the supervision of healthcare professionals. Parents are encouraged to participate in the care process through practices like kangaroo care (skin-to-skin contact) when appropriate.
Medication
Newborn Respiratory Distress Syndrome (NRDS) primarily requires careful management in a neonatal intensive care unit. Medications used might include surfactant therapy, which involves administering surfactant directly into the baby's lungs to reduce surface tension and improve breathing. Other supportive treatments may involve the use of nasal Continuous Positive Airway Pressure (CPAP) or mechanical ventilation, and in some cases, corticosteroids might be given before birth to mothers at risk of preterm delivery to help mature the baby's lungs. Specific medication details would be tailored to the individual case by healthcare professionals.
Repurposable Drugs
Repurposable drugs for newborn respiratory distress syndrome (RDS) include:

1. **Caffeine Citrate**: Commonly used to treat apnea of prematurity, caffeine has been studied for its potential benefits in improving respiratory function in newborns with RDS.

2. **Surfactant Therapy**: While not a repurposed drug in the traditional sense, synthetic or animal-derived surfactants (such as calfactant, beractant) are crucial in treating RDS by reducing surface tension in the lungs and improving breathing.

3. **Corticosteroids**: Antenatal corticosteroids, such as betamethasone and dexamethasone, are given to pregnant women at risk of preterm birth to accelerate fetal lung maturation and decrease the incidence or severity of RDS.

Further research and clinical trials are needed to explore and confirm the efficacy and safety of these treatments in different settings.
Metabolites
Newborn Respiratory Distress Syndrome (NRDS), also known as neonatal respiratory distress syndrome, primarily affects premature infants. The metabolism of surfactant, a substance crucial for lung function, plays a significant role in this condition. Key metabolites involved include:

1. **Phosphatidylcholine (PC):** The main component of surfactant.
2. **Phosphatidylglycerol (PG):** Another essential phospholipid contributing to surfactant function.
3. **Sphingomyelin:** Often used as a reference in lecithin-sphingomyelin (L/S) ratio testing, indicative of lung maturity.

Understanding and identifying these metabolites can facilitate the diagnosis and management of NRDS.
Nutraceuticals
Nutraceuticals are not a primary treatment for Newborn Respiratory Distress Syndrome (NRDS). The condition is primarily managed with medical interventions such as surfactant replacement therapy, mechanical ventilation, and continuous positive airway pressure (CPAP). Nutraceuticals, which include dietary supplements and probiotics, do not have an established role in the treatment of NRDS.
Peptides
Newborn Respiratory Distress Syndrome (NRDS) is primarily treated through the administration of surfactant therapy, which helps reduce surface tension in the alveoli. Peptides used in NRDS treatment are often components of these surfactants. Examples include:

1. **SP-A (Surfactant Protein A)**
2. **SP-B (Surfactant Protein B)**
3. **SP-C (Surfactant Protein C)**
4. **SP-D (Surfactant Protein D)**

These peptides enhance the spread and stability of the surfactant, thereby improving lung function and oxygenation in affected newborns.