We report a 17-year-old Malay boy with cystic fibrosis who over a 14-month period experienced worsening respiratory symptoms and deteriorating lung function. Burkholderia pseudomallei was eventually isolated from his sputum. He improved clinically following treatment for meliodosis and his lung function returned to normal.
All children who underwent flexible bronchoscopy in the respiratory unit at Paediatric Institute, Hospital Kuala Lumpur from June 1997 to June 2002 were reviewed. A hundred and ten children underwent the procedure under sedation or general anaesthesia. The median age of these children was eight months. (Q1 3, Q3 30) The commonest indication for performing flexible bronchoscopy was for chronic stridor (50 cases) followed by persistent or recurrent changes such as lung infiltrates, atelectasis and consolidation on the chest radiographs (22). Laryngomalacia was found to be the commonest cause of stridor in 29 children. Two patients were diagnosed with pulmonary tuberculosis. With regard to safety, three procedures were abandoned due to recurrent desaturation below 85%. One of these patients had severe laryngospasm that required ventilation for 48 hours but recovered fully. Two neonates developed pneumonia requiring antibiotics following bronchoscopy. No patients developed pneumothorax or bleeding following the procedure. Bronchoscopy is a safe procedure when performed by well-trained personnel. Since it is an invasive procedure the benefits must outweigh the risks before it is performed.
Cystic fibrosis (CF) is an autosomal recessive disease commonly found among the Caucasian population. The availability of sweat test and with increasing experience have made it possible to diagnose more cases of CF. Our first case of CF was diagnosed 16 years ago and to date we have managed sixteen cases of CF. Sixteen children were diagnosed with CF in our units at the Paediatric Institute and University Malaya Medical Centre (UMMC). They were referred with either one or all of the following symptoms: i) recurrent pneumonia, ii) bronchiectasis, iii) failure to thrive, iii) malabsorption or iv) history of meconium ileus obstruction during the neonatal period. When the clinical features suggested strongly of CF, sweat tests will be performed in duplicates and considered positive when the sweat chloride or sweat sodium was more than 60 mmol/l for both results. Seventy- two hours fecal fat excretion or stool for fat globule was performed to document malabsorption. From the year 1987 to 2003, 16 patients were confirmed to have cystic fibrosis in Malaysia by positive sweat tests. Thirteen patients were diagnosed in Paediatric Institute while the remaining three were diagnosed in UMMC. On follow-up two patients died due to severe bronchopneumonia at the age of two years old. Although once considered rare, CF should now be considered in any children with clinical presentations of recurrent chest infections, bronchiectasis, in the presence or absence of malabsoption stmptoms and in neonates with meconium ileus obstruction.