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Long-term follow up of children with the Wilson-Mikity syndrome
Insights
Survivors of Wilson-Mikity (W-M) syndrome show good long-term outcomes. Most children had normal lung function, but some exhibited airway obstruction and bronchial hyperreactivity, suggesting a favorable prognosis compared to bronchopulmonary dysplasia.
Area of Science:
- Pediatric Pulmonology
- Neonatology
- Respiratory Medicine
Background:
- Wilson-Mikity (W-M) syndrome is a rare, severe respiratory disorder affecting premature infants.
- Long-term sequelae in survivors of W-M syndrome require further investigation.
- Understanding the pulmonary and cardiac status of W-M syndrome survivors is crucial for prognosis.
Purpose of the Study:
- To evaluate the long-term respiratory and cardiac status of children who survived Wilson-Mikity syndrome.
- To assess for persistent respiratory symptoms, airway obstruction, and bronchial hyperreactivity.
- To determine the prognosis of W-M syndrome survivors in comparison to other neonatal lung diseases.
Main Methods:
- Longitudinal study of nine W-M syndrome survivors at a mean age of 7.6 years.
- Pulmonary function tests including methacholine challenge.
- Chest radiography and echocardiography to assess lung and cardiac structure and function.
Main Results:
- One child reported persistent respiratory symptoms.
- Three children demonstrated significant airway obstruction.
- Six children showed bronchial hyperreactivity on methacholine challenge.
- Radiographic abnormalities were noted in three children, including asthma-like features.
- Echocardiography revealed enlarged right ventricular cavities in two children, with normal systolic function.
Conclusions:
- Survivors of Wilson-Mikity syndrome generally exhibit a good prognosis.
- While some may have persistent airway issues like obstruction or hyperreactivity, severe long-term complications are uncommon.
- The findings suggest a better long-term outlook for W-M syndrome survivors compared to those with bronchopulmonary dysplasia.
Abstract:
Nine children who survived the development of Wilson-Mikity (W-M) syndrome were studied at a mean age of 7.6 +/- 0.3 years. Persistent respiratory symptoms during the year preceding the study were present in one child. Pulmonary function studies demonstrated a significant degree of airway obstruction in 3 children. Positive methacholine challenge was observed in 6 children indicating bronchial hyperreactivity. Abnormal chest radiographs were present in 3 children. One showed the characteristic appearances of asthma with air trapping, bronchial wall thickening and redistribution of pulmonary vessels. The other child showed minimal residual peribronchial thickening and the third child had normal lungs, but a rather prominent pulmonary artery segment. Echocardiographic studies revealed enlarged right ventricular cavity in 2 children. However, the pre-ejection period and the systolic time intervals were all within normal limits. It appears that unlike patients with bronchopulmonary dysplasia survivors of W-M syndrome have a good prognosis.