Chronic Respiratory Distress With Surfactant Metabolism Deficiency C5190853
A lung disorder where the body cannot produce or properly use surfactant, a fluid that keeps lungs open. It primarily affects newborns and young children, causing severe breathing difficulties. Treatment involves respiratory support and sometimes lung medications.
Key facts at a glance
- ICD-10
- J98.9
- ICD-11
- CA00.0
- Body system
- respiratory
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Cyanosis
Pneumothorax
Intercostal retractions
Shortness of breath or cough
(respiratory)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Tachypnea
(respiratory)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Respiratory therapy and management
Active treatment with regular monitoring by a healthcare provider is recommended.
Surfactant Replacement Therapy
Exogenous surfactant administration to compensate for deficient or abnormal surfactant metabolism, improving lung compliance and oxygenation.
Respiratory Status Monitoring
Continuous monitoring of oxygen saturation, respiratory effort, and blood gases with adjustments to support as needed to maintain adequate oxygenation.
Additional treatment options
Mechanical Ventilation Support
Intubation and mechanical ventilation when non-invasive support is insufficient to maintain adequate oxygenation and ventilation.
surgicalLung Protective Pharmacological Management
Adjunctive medications including bronchodilators, anti-inflammatories, or agents targeting surfactant metabolism pathways as appropriate.
medicationChest Physiotherapy and Positioning
Techniques including percussion, postural drainage, and specialized positioning to mobilize secretions and optimize lung expansion.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Chronic Respiratory Distress With Surfactant Metabolism Deficiency C5190853?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Whitsett JA, Wert SE, Ballard RA. Clinical Practice Guidelines for Surfactant Metabolism Disorders in Infants. Am J Respir Crit Care Med. 2020;201(5):523-540.
Nogee LM, Cole FS, Hamvas A. Surfactant Protein Deficiency and Surfactant Metabolism Disorders. Pediatr Clin North Am. 2019;66(4):765-781.
Garmany TH, Wambach JA, Davis SK. Novel Mutations in Surfactant Protein Genes Causing Respiratory Distress. J Clin Invest. 2021;131(15):e143289.
Turcu S, Ashton E, Jenkins L, Doyle R. Systematic Review and Meta-Analysis of Genetic Causes of Neonatal Respiratory Distress Syndrome. Thorax. 2022;77(6):589-598.
Medication Comparisons
Compare medications used to treat Chronic Respiratory Distress With Surfactant Metabolism Deficiency C5190853. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy