Patient | Presentation at onset | Physical development | Management | Long-term treatment of lung disease (method/ starting age/ ending age) | Improvement | Symptoms at last observation (age) |
---|---|---|---|---|---|---|
1 | Severe pneumonia Respiratory failure | Normal height Low weight at 7 m (approximate to P3) | Mechanical ventilation Antibiotics Oral prednisone Montelukast Aminophylline Digoxin, Diuretic | Home oxygen therapy | Moderate | Exercise tolerance reduction, Tachypnea after strenuous exercise (5y) |
2 | Severe pneumonia Respiratory failure | Normal weight (P10–25) | Mechanical ventilation Surfactant Systemic steroids Antibiotics, Diuretic Cardiac stimulant Aminophylline | Persistent mechanical ventilation at ICU (15 m to 22 m until giving up treatment) | No improvement | Recurrent pneumothorax Respiratory failure (22 m died) |
3 | Pneumonia | Low weight at 9 m (below P3) | Persistent low flow oxygen therapy Antibiotics | Family oxygen therapy(7d to 19 m), HCQ(10 mg·kg− 1·d− 1 from 13 m to 36 m at present) | Significant (withdrawal of oxygen and weight rises to normol after 6 m HCQ treatment) | Asymptomatic (3y) |
4 | Severe pneumonia Pectus excavatum | Low weight at 5 m(below P3) | High flow and low flow oxygen inhalation Antibiotics Nasal feeding | Family oxygen therapy (5 m to 8 m), HCQ(10 mg·kg−1·d− 1 last for a month until dead) | No improvement | Recurrent fever at home Failure to thrive (7 m) Deceased (8 m) |
5 | Pneumonia | Low height and low weight at 1y(height below P3, and weight far below P3) | Persistent low flow oxygen therapy Antibiotics | Family oxygen therapy (7 m to 15 m), HCQ(10 mg·kg− 1·d− 1 from 11 m to 24 m at present) | Significant (withdrawal of oxygen after 4 m HCQ treatment), and slight weight gain (P3 at 24 m) | Language retardation Mild dysphagia (24 m) |
6 | Pneumonia Pectus excavatum Repeated diarrhea (food allergy) | Low weight at 2y (below P3) | Mask oxygen inhalation Antibiotics Nasal feeding | Family oxygen therapy (5 m to 27 m), HCQ (5 mg·kg−1·d−1 from 24 m for a month then 10 mg·kg− 1·d− 1 for 5 months at present) | Moderate (Weight gain slightly), Slight decrease in oxygen demand(from initial 3.5–4 L·min− 1 to 3 L·min− 1 at present) | Increase in food intake Psychomotor retardation (30 m) |