Cystic fibrosis in Malaysian children.

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.

Local experience in paediatric flexible bronchoscopy.

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.

Home oxygen therapy for children with chronic lung diseases.

UNLABELLED: Home oxygen therapy programme is new in Malaysia. This programme enables children with respiratory insufficiency to be discharged home early. MATERIALS AND METHODS: Long term oxygen therapy was initiated using an oxygen concentrator in patients who i) remained hypoxic while breathing room air, ii) experienced desaturations of more than 20% during sleep as seen in patients with severe laryngomalacia and obstructive sleep apnoea syndrome and iii) had pulmonary hypertension with or without polycythaemia. The median with first and third quartile values are presented for the quantitative variables. RESULTS: A total of 71 patients mainly children with bronchopulmonary dysplasia (BPD) (32) and bronchiolitis obliterans (12) were discharged home on this programme. The median age at which home oxygen was initiated in children with BPD was 5.0 (Q1: 2, Q3: 8) months. The median total duration of oxygen requirement for BPD was 8.0 (Q1: 5, Q3: 12) months. The median duration of home oxygen dependency was 3.5 (Q1: 3, Q3: 6) months. However children with bronchiolitis obliterans required longer duration of oxygen therapy compared to children with BPD i.e. median duration of 28 months (Q1: 14.5, Q3: 66). In other respiratory conditions the mean duration of supplemental oxygen varies some of which may be life long. CONCLUSIONS: This paper has shown the importance of home oxygen program in children with respiratory disorders. It has significantly shortened hospital stay and thus saves hospital costs and prevents prolonged separation from the family.

Prevalence of childhood asthma and allergy in an inner city Malaysian community: intra-observer reliability of two translated international questionnaires.

OBJECTIVES: (a) To examine the intra-observer reliability of the Malay language versions of two international respiratory questionnaires i.e. the International Study of Asthma and Allergy in Children (ISAAC) and the American Thoracic Society (ATS) questionnaires, and (b) using the more reliable of these questionnaires, to estimate the prevalence of asthma and allergy related symptoms in an ethnically homogenous inner city community in Kuala Lumpur. METHODS: The study was conducted among 7 to 12 year old school children of Malay ethnic origin living in an inner city area of Kuala Lumpur. The sample consisted of 787 children attending the only primary school in the area. The Malay versions of both questionnaires were administered twice, one month apart, and were completed by parents. Agreement between the first and second responses to the same questions were assessed by Cohen's kappa. Kappa values < 0.4 were indicative of poor intra-observer reliability, 0.4-0.59 moderate reliability, 0.6-0.79 good reliability and > 0.79 excellent reliability. RESULTS: 77.9% and 36.3% of parents responded to the first and second administrations of the questionnaires respectively. Kappa values of > 0.4 were obtained in 15/16 (93.8%) and 17/27 (63.0%) questions of the ISAAC and ATS questionnaires respectively. Excellent kappa values were obtained in 4/16 (25%) questions of the ISAAC questionnaire versus only 1/27 (3.7%) questions of the ATS questionnaire. From the ISAAC questionnaire, all questions on wheeze had good reliability while those on asthma had excellent reliability. Questions on allergic symptoms had poor to moderate reliability. In contrast, from the ATS questionnaire, questions on wheeze had moderate reliability while questions on asthma were excellently reliable. Questions on allergic symptoms had moderate to good reliability while those on cough, phlegm and bronchitis had poor reliability. According to the ISAAC questionnaire the prevalence of ever wheeze, wheeze in the last 12 months, ever asthma and wheeze with exercise in the last 12 months was 12.5%, 6.6%, 10.3% and 5.9% respectively. The prevalence of ever sneeze or runny nose, sneeze or runny nose in the last 12 months, watery eyes in the last 12 months and ever eczema was 15.2%, 11.1%, 4.4% and 8.5% respectively. CONCLUSIONS: The translated ISAAC questionnaire was more reliable than the translated ATS questionnaire. Asthma and related symptoms were common among Malay school children in inner city Kuala Lumpur.

Asthma education: how much does it improve knowledge of childhood asthma amongst medical students and paramedics?

The objective of this study was to measure the knowledge of childhood asthma among medical students and paramedics. A previously validated questionnaire about childhood asthma was completed by 281 of 314, third and fifth year medical students at Universiti Kebangsaan Malaysia, Kuala Lumpur. Their knowledge of asthma was assessed during the first and last weeks of their paediatric rotation. A similar questionnaire was completed by 23 of 60 paramedics from various medical disciplines in Hospital Kuala Lumpur. They had attended a two-day seminar on respiratory diseases and their knowledge was assessed prior to and six weeks after the seminar. On the initial assessment the mean score for the final year medical students was 24.5, third year medical students 20.9 and paramedics 18.3. After intervention their mean scores increased significantly to 26.3 (p < 0.0001), 24.6 (p < 0.0001) and 21.3 (p < 0.0001). After intervention, the final year medical students improved significantly in all questions except in the management of acute asthma. Post intervention, third year medical students showed a significant increase in knowledge pertaining to symptomatology, pathophysiology, trigger factors and prophylactic drugs used in asthma management. Although the knowledge of paramedics improved post intervention, they had major deficiencies in knowledge about pathophysiology, trigger factors, preventive and acute asthma therapy, side effects of asthma treatment as well as clinical scenarios. Improvement after intervention was only seen in six of the 31 questions. This study demonstrated an increase in knowledge about childhood asthma among medical students and paramedics after a short intervention.

Prevalence, clinical predictors and diagnosis of gastro-oesophageal reflux in children with persistent respiratory symptoms.

OBJECTIVES: This was a cross sectional study conducted in the Paediatric Institute among infants and children with chronic respiratory symptoms with the following objectives: i) to determine the prevalence of gastro-oesophageal reflux in children with persistent respiratory symptoms, ii) to identify the clinical predictors of GOR (Gastro-oesophageal reflux) in children with persistent respiratory symptoms and iii) assess the validity of abdominal ultrasound, barium oesophagogram and chest radiograph in diagnosing GOR in these patients. MATERIALS AND METHODS: Forty-four patients were recruited over a period of six months. All the presenting symptoms were identified. The patients were subjected to chest radiograph, abdominal ultrasound, barium oesophagogram and 24-hour pH oesophageal monitoring. The predictive validity of clinical symptoms, chest radiograph, abdominal ultrasound and barium oesophagogram were assessed. Twenty-four hours oesophageal pH was the gold standard to diagnose GOR. RESULTS: The mean age of patients was 9.1 months (1-58 months). Thirty-one patients (70.5%) were confirmed to have GOR by pH study. Respiratory symptoms alone were not useful to predict GOR. Cough had the highest sensitivity of 51.6%. Stridor, wheeze and choking each had a specificity of 76%. Wheeze, vomiting, choking and stridor were identified to have high specificity (90-100%) in diagnosing GOR when any two symptoms were taken in combination. Collapse/consolidation was the commonest radiological abnormality but had low sensitivity (35.5%) and specificity (53.8%). However hyperinflation on chest radiograph had a specificity of 92.3% with positive predictive value of 80% in diagnosing GOR. Barium oesophagogram has low sensitivity (37.9%) and moderate specificity (75%) in diagnosing GOR in children with respiratory symptoms. Abdominal ultrasound was a valid mode of diagnosing GOR when there were three or more reflux episodes demonstrated during the screening period with a specificity of 90.9%. However the sensitivity was low i.e. 20-25%. The specificity increased to 90-100% when two positive tests were taken in combination (abdominal ultrasound and barium oesophagogram). However the sensitivity remained low (10-20%). Chest radiograph did not improve the predictive value when considered with the above tests. Combination of clinical symptoms were useful as clinical predictors of GOR. In the absence of a pH oesophageal monitoring, a combination of barium oesophagogram and ultrasound may be helpful in diagnosing GOR.

Acute respiratory distress syndrome in a paediatric intensive care unit.

Acute respiratory distress syndrome (ARDS) is the final outcome of a common pathway of a variety of unrelated but massive insults to the lung. It is commonly seen in adults but also occurs in the paediatric age group. A prospective study was carried out to determine the incidence, predisposing conditions, clinical course and outcome of children with ARDS admitted to a paediatric intensive care unit (PICU). Six patients (aged 0.8 to 11 years) who fulfilled the strict criteria for ARDS were identified prospectively during a one year study period. The incidence was 1.7% of all PICU admission. The most common underlying conditions were septicemia and pneumonia. The mortality rate was 83%. Death most often occurred during the early phase of the disease. Treatment of ARDS included elimination of the cause of ARDS, early institution of mechanical ventilation with PEEP, prompt recognition and treatment of superimposed infection and careful management of additional organ failure.

Childhood epilepsy: what parents know, believe and do.

This was a descriptive study to assess parents' knowledge of epilepsy in their children at the Klinik Pakar Pediatrik in Universiti Kebangsaan Malaysia from 1.1.93-31.6.93. Factors that influence the level of knowledge were examined. Our hypothesis was that the level of knowledge was low and level of education and social factors were important. Fifty consecutive parents were interviewed during the clinic appointments. The questionnaire consisted of 25 questions which had been used in a survey on epilepsy in Australia. In order to cater for the local population the questions were modified by adding new questions pertaining to local situation. The results showed that 90% (45/50) of parents were unaware of the type of epilepsy their children were suffering from. 50% (25/50) of parents knew the underlying cause of epilepsy of which 32% (8/25) attributed it to brain disease, 8% (2/25) to birth defects and 10% (3/25) to fever. Factors such as duration of epilepsy, parental education and racial differences between Malay and other races (Chinese, Indians) did not reach any statistical significance (p > 0.05). 80% of patients (30/50) were on monotherapy. However, 90% (45/50) of parents were unaware of their children's medications. 82% of parents (31/50) knew that the anti-convulsants would only control their children's convulsions. Only 10% (8/50) of parents knew the acute management of seizures. Wrong practices such as inserting spoons (5/50) or massaging their limbs (17/50) during an acute attack were still common. 70% of parents (35/50) attended the follow-up clinics hoping that their children's epilepsy would be cured. Parents with low economic status and of children with duration of epilepsy of less than five years had been coming to the clinic regularly. (p = 0.01 and p = 0.02 respectively). In conclusion, the overall knowledge of these parents was poor. In order to improve the management of epilepsy, it is necessary to educate parents with reading materials and effective educational packages.

Interstitial lung disease in children--a report of four cases.

Interstitial lung disease (ILD) is very rare in children. In the majority of cases the aetiology is unknown. Very little is known about the clinical course of this condition in children. Prognosis may be influenced by sex, age of onset of symptoms, radiographic features, presence of right ventricular hypertrophy and histopathology. We report our experience in managing four children with interstitial lung disease. All these children presented in early infancy with cough, respiratory distress, cyanosis and failure to thrive. Three of these children had finger clubbing and right ventricular hypertrophy. All patients received oral steroids. Chloroquine was added in two patients who showed no response. A trial of oral cyclophosphamide was started in one patient who failed with both drugs. One child is oxygen independent while another is on home oxygen therapy. The other two patients eventually died.

Parental reactions to febrile seizures in Malaysian children.

The reactions of 117 parents to the febrile seizure experienced by their children; and their fears and worries were investigated. A standard questionnaire was used and clinical information was abstracted from the notes. In 88.9% of the cases, the adult present at the seizure was one of the parents usually the mother. Most of the parents (66.7%) did tepid sponging to bring the fever down but a third tried to open the clenched teeth of the child. The adults present placed the child supine in 62.9%, on the side in 9.5% and prone in 6.0%. Over half of the parents brought the child to a private clinic first before bringing to hospital. A fifth of the children were given antipyretics by the parent or the doctor and an anticonvulsant was given in 7.7% of cases. Interestingly, in 12% of children traditional treatment was given for the seizure. Three quarters of the parents knew that the febrile seizure was caused by high fever (which we have taken as the correct knowledge of febrile seizure). However "ghosts" and "spirits" were blamed as the cause of the seizure by 7% of parents. Factors significantly associated with correct knowledge were higher parental education and higher family income. The most common fear expressed was that the child might be dead or might die from the seizure (70.9%). Fear of death was associated with low paternal education. We concluded that the majority of our parents had reacted appropriately to a febrile seizure and their knowledge of the cause of febrile seizure was generally correct. Their fears and worries were similar to those elsewhere. However, traditional beliefs and practices may have to be taken into consideration during counselling.

Cystic fibrosis in Malay children--a report of three cases.

Cystic fibrosis (CF) is a rare disease among Asians. Three Malay children with CF presenting with recurrent pulmonary symptoms, malabsorption and failure to thrive are reported. Problems in their management include availability of pancreatic enzymes, compliance to medications and climate factors.

Gastro-oesophageal reflux in children with severe respiratory symptoms--clinical spectrum and management.

Respiratory symptoms in children may be associated with underlying gastro-oesophageal reflux (GOR). We reviewed the case notes of 20 children who presented to us from June 1993 to June 1994 with respiratory symptoms and GOR. The patients consisted of 16 Malays, two Chinese and two Indians with equal number of males and females. Their age at diagnosis was less than one year in 17 patients. The earliest age at presentation was at the third day of life. All patients had major respiratory manifestations i.e. recurrent wheezing, recurrent cough and pneumonia. In addition, three patients had stridor and six patients had apparent life threatening episodes (ALTE). Fourteen patients required ventilation because of respiratory failure. Diagnosis of GOR was based on clinical grounds supported by barium oesophagogram in seven patients and ultrasound examination in 11 patients. Eight patients were fundoplicated because of ALTE and recurrent severe bronchospasm. On follow up, 14 patients had hyperactive airways requiring inhaled bronchodilator and steroid therapy.

An evaluation of the agreement between three peak flow meters: Wright's, Miniwright's, and Pocket, when used in children.

Three peak flow meters (PFM), Wright's, Miniwright's II (MPFM), and Pocket, were evaluated in 50 children in a pediatric outpatient clinic. It was found that there was close agreement between the Pocket and the MPFM as shown by an intraclass correlation (rho) of 0.95. Less agreement was noted between the MPFM and the Pocket, respectively, with the Wright's. It was also noted that the coefficient of correlation (r) was not a suitable test for agreement in this study. Both the Pocket and the MPFM also read higher than the Wright's. We conclude that children should be monitored using the same type of peak flow meter.

Clinical patterns of X linked agammaglobulinemia in Malaysian children.

X linked agammaglobulinemia (XLA) is rarely reported from developing countries especially from South East Asia. It appears that X linked agammaglobulinemia is less common in certain ethnic groups. It is very uncommon in black people in USA and South Africa. In multiracial Malaysia we have documented five XLA in Malays and Indians but not in the Chinese that constitute about 31% of the population. First degree relatives afflicted with XLA or other primary immunodeficiencies occurred more often in our study. All showed lung involvement although the etiologic organisms involved were atypical, being Gram negative.

Protective and risk factors for acute respiratory infections in hospitalized urban Malaysian children: a case control study.

We performed a case control study to examine protective and risk factors for acute respiratory infections (ARI) in hospitalized children in Kuala Lumpur. Consecutive children between the ages of one month and five years hospitalized for pneumonia (n = 143), acute bronchiolitis (n = 92), acute laryngotracheobronchitis (n = 32) and empyema (n = 4) were included as cases and were compared with 322 children hospitalized during the same 24 hour period for non-respiratory causes. Potential risk and protective factors were initially analysed by univariate analysis. Logistic regression analysis confirmed that several home environmental factors were significantly associated with ARI. The presence of a coughing sibling (OR = 3.76, 95%CI 2.09, 6.77), a household with more than five members (OR = 1.52, 95%CI 1.03, 2.19) and sleeping with three other persons (OR = 1.45, 95%CI 1.00, 2.08) were independent risk factors. Significant host factors were history of allergy (OR = 2.50, 95%CI 1.74, 3.61) and ethnicity (Malay race) (OR = 2.07 95%CI, 1.27, 3.37). Breast feeding for at least one month was confirmed as an independent protective factor (OR = 0.58, 95%CI 0.38, 0.86). However, the study was not able to demonstrate that domestic air pollution had an adverse effect. This study provides further evidence that home environmental factors, particularly those associated with crowding, may predispose to ARI and that breast feeding is an important protective factor.

Indoor air pollution and asthma in hospitalized children in a tropical environment.

We performed a hospital-based study to examine a hypothesis that indoor air pollution was associated with acute asthma in young children living in Kuala Lumpur City. A total of 158 children aged 1 month to 5 years hospitalized for the first time for asthma were recruited as cases. Controls were 201 children of the same age group who were hospitalized for causes other than a respiratory illness. Information was obtained from mothers using a standardized questionnaire. Univariate analysis identified two indoor pollution variables as significant factors. Sharing a bedroom with an adult smoker and exposure to mosquito coil smoke at least three nights in a week were both associated with increased risk for asthma. Logistic regression analysis confirmed that sharing a bedroom with an adult smoker (OR = 1.91, 95% CI 1.13, 3.21) and exposure to mosquito coil smoke (OR = 1.73, 95% CI 1.02, 2.93) were independent risk factors. Other factors independently associated with acute asthma were previous history of allergy, history of asthma in first-degree relatives, low birth weight, and the presence of a coughing sibling. There was no association between asthma and exposure to kerosene stove, wood stove, aerosol mosquito repellent, type of housing, or crowding. We conclude that indoor air pollution is an avoidable factor in the increasing morbidity due to asthma in children in a tropical environment.

Febrile seizures in Malaysian children: epidemiology and clinical features.

A child with a febrile seizure is a common cause of admission in a general paediatric ward in Malaysia. We set out to look prospectively into the clinical and epidemiological features of these children. A total of 117 children were admitted into the study. The ratio of boys to girls were 1.5:1.0. The racial breakdown was 62.4% Malays, 26.5% Indians, 8.5% Chinese and 2.6% others. The first febrile seizure occurred before the age of three years in 92.9% of our patients. The highest number of febrile seizures was in the six to 12 months age group. The average length of seizure was 9.5 minutes and the majority were non-recurrent. Febrile seizures with complex features occurred in 33.3% of the children. Upper respiratory tract infection was the most common cause of fever in our patients. There was a family history of seizures (febrile or afebrile) in 26.5% of patients.

Parents' views of lumbar puncture in children with febrile seizures.

A study was carried out to ascertain the views of parents regarding the performance of lumbar punctures on their children admitted for febrile seizures. One hundred and seventeen (117) children with febrile seizures were recruited over nine months. Either one of the parents was interviewed a day after admission. In most cases, this was usually the mother. The ethnic groups of the patients were Malays (62.4%), Indians (26.5%), Chinese (8.5%) and others (2.6%). Lumbar punctures were requested by the doctor in attendance in only 28 (23.9%) patients This showed that the rate of request for lumbar punctures in febrile seizures was low. Parents of eight of them refused. The main reasons for the refusal were: fears that the child might be paralysed, advice from relatives and fear that the child might die from the procedure, or might find it too painful. All the parents who refused were Malays. A lumbar puncture was also more likely to be refused in a girl. Those who consented to lumbar puncture did so because they wanted the doctor to get to the diagnosis. Another reason given was that it might be therapeutic. Parents whose children did not require a lumbar puncture also thought that lumbar puncture may cause paralysis. The main sources of information on lumbar punctures for the parents were their relatives and/or friends. In only 85% of the cases were the reasons for the lumbar puncture explained to the parent. In 71.4% of the time the explanation was done by the medical officer, and in only 4.8% of the time was the consultant involved.(ABSTRACT TRUNCATED AT 250 WORDS)

Circumstances surrounding accidental poisoning in children.

Circumstances surrounding accidental ingestion of poisons were investigated in families of 70 hospitalised children. Indian children were more likely to be hospitalised for poisoning. Poor safety measures directly contributed to poisoning incidents. Poisons had not been stored safely and only in 12.9 per cent of families were the poisons allegedly kept in cupboards. Kerosene had been ingested from soft drink containers in 70 per cent of instances. Medications had been taken mainly from plastic envelopes (57.9%) or bottles (31.6%). Safety practices in the homes of cases and 140 controls were compared. As a whole parents of victims had poor safety practices when compared with parents of controls. Poisoning incidents were discovered by mothers in 75.0 per cent of the cases. Vomiting was induced by parents in 32.3 per cent of cases involving medications and non-volatile items, and in 41.6 per cent of kerosene ingestions. Before hospitalisation 59.4 per cent were brought to general practitioners and 22.7 per cent to government clinics. Mothers were the main attendants when children were brought for medical care. This study highlights the need for measures to prevent childhood poisoning and reduce its morbidity. Household products and medications should be made safer to children through improved packaging, prescribing instructions and education. Safety and first aid education may be directed towards parents, particularly mothers, through the media and health facilities.

Severe and fatal postoperative bronchospasm in a child with a pulmonary artery sling.

We report a case of left pulmonary artery sling in a child who also had duodenal atresia. He was admitted for respiratory failure requiring prolonged respiratory support due to a bronchiolitis-like illness at two months of age. Diagnostic procedures confirmed the presence of left pulmonary artery sling. He had a corrective procedure which relieved the compression. However postoperatively he had intermittent episodes of severe bronchospasm in addition to a persistent airway obstruction. Finally one such episode of severe bronchospasm did not respond to medical and resuscitative therapy and the baby succumbed. To our knowledge no case of left pulmonary artery sling has been described previously in a Malaysian child. This case also highlights the postoperative airway problems that may be encountered.