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Long-term pulmonary sequelae of the Wilson-Mikity syndrome
Insights
Children who survived Wilson-Mikity syndrome show significantly reduced air flow rates. Abnormalities in their lung function suggest uneven emptying of lung units, potentially linked to focal increases in compliance or resistance.
Area of Science:
- Pediatric Pulmonology
- Respiratory Physiology
- Neonatal Medicine
Background:
- Wilson-Mikity syndrome is a severe respiratory distress affecting premature infants.
- Long-term pulmonary sequelae in survivors are not fully understood.
- Assessing lung function in these children is crucial for understanding disease impact.
Purpose of the Study:
- To evaluate the long-term pulmonary function in survivors of Wilson-Mikity syndrome.
- To compare airflow dynamics in survivors with healthy premature and full-term infants.
- To identify potential residual respiratory abnormalities.
Main Methods:
- Flow volume curves were measured in three groups: Wilson-Mikity survivors, healthy premature infants, and healthy full-term infants.
- Breathing air and a helium-oxygen mixture (80% He/20% O2) was used to assess airflow.
- Statistical analysis compared flow rates and isoflow volumes between groups.
Main Results:
- Survivors of Wilson-Mikity syndrome exhibited significantly lower airflow rates in air compared to both control groups (P < 0.01 and P < 0.001).
- Three out of five survivors displayed marked concavity in their flow-volume curves, indicating abnormal emptying.
- No significant difference in the volume of isoflow between air and HeO2 was observed across groups.
Conclusions:
- Survivors of Wilson-Mikity syndrome have persistent airflow limitations.
- Abnormalities suggest focal increases in compliance or resistance, leading to asynchronous lung emptying.
- The underlying pathology may be consistent between survivors and fatal cases, with long-term implications for lung growth.
Abstract:
Five children born prematurely, eight to ten years ago, who survived the Wilson-Mikity syndrome were compared with six apparently normal prematurely born children and eight normal children born at term, by means of flow volume curves obtained while breathing air and while breathing a mixture of 80% helium and 20% oxygen. The flow rates in air of the group who survived the Wilson-Mikity syndrome were significantly lower than those of the prematurely born normal children (P less than 0.01), and than those of the eight normal children (P less than 0.001). The volume of isoflow while breathing air compared to that while breathing HeO2 was highly variable, but not significantly different in the three groups. Three of the five survivors of the Wilson-Mikity syndrome had a marked concavity in the shape of their flow volume curves. It is concluded that these abnormalities are due to focal increases of compliance or resistances or both of terminal lung units and result in adjacent areas of the lung emptying at different rates. The relationship between physiologic and anatomic abnormalities seen at autopsy in those who die to the disease suggests that they are due to the same pathologic process. Whether or not these abnormalities will disappear with future lung growth remains to be seen.