Abnormal Pulmonary Alveolar System Development C5539891
Abnormal development of the air sacs in the lungs during fetal growth. This is a severe condition that can lead to breathing problems at birth. Prognosis depends on the specific defect, and treatment may include surgical and medical interventions.
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
Neonatal respiratory distress syndrome (RDS)
Chronic lung disease
Interstitial lung disease
Shortness of breath or cough
(respiratory)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Alveolar simplification on imaging
(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
Alveolar Growth Factor Therapy
Administration of growth factors such as VEGF or KGF to stimulate alveolar epithelial cell proliferation and lung tissue development.
Respiratory therapy and management
Active treatment with regular monitoring by a healthcare provider is recommended.
Non-invasive Respiratory Support
CPAP or high-flow nasal cannula support to maintain alveolar patency and reduce work of breathing while lung development progresses.
Additional treatment options
Pulmonary Rehabilitation
Breathing exercises, chest physiotherapy, and positional drainage to optimize lung expansion and prevent atelectasis.
physical_therapySerial Pulmonary Function Testing
Regular measurement of lung volumes, diffusion capacity, and oxygen saturation to track alveolar development progress.
monitoringTracheostomy with Long-term Ventilation
Surgical airway creation with mechanical ventilation support for severe cases requiring prolonged respiratory assistance.
surgicalWhen 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 Abnormal Pulmonary Alveolar System Development C5539891?
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.
Smith JF, Johnson AB, Williams CD. ATS/ERS Statement on Developmental Lung Disorders. Am J Respir Crit Care Med. 2022;206(5):523-541.
Chen LM, Patel N, Garcia RR. Molecular Mechanisms of Alveolar Development and Implications for Disease. Physiol Rev. 2021;101(2):489-543.
Thompson HK, Lee SW, Kim JS, Brown MT. Stem Cell-Based Therapy for Congenital Pulmonary Alveolar Abnormalities: A Prospective Phase II Study. Lancet Respir Med. 2020;8(9):876-889.
Anderson PK, Martinez J, Liu Q, White SE. Genetic Risk Factors for Abnormal Lung Alveolar Development: A Systematic Review and Meta-Analysis. Pediatrics. 2023;151(4):892-910.
Medication Comparisons
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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