Kawasaki disease in Hong Kong, 1994 to 2000.

OBJECTIVE: To describe the epidemiology, clinical characteristics, and management of Kawasaki disease in children in Hong Kong. DESIGN: Retrospective survey of medical records from July 1994 to June 1997, and prospective data collection from July 1997 to June 2000. SETTING: Hospitals with a paediatric unit in Hong Kong. PATIENTS: Patients diagnosed with Kawasaki disease between July 1994 and June 2000 in public hospitals in Hong Kong. MAIN OUTCOME MEASURES: Incidence of Kawasaki disease and coronary artery aneurysm rates. RESULTS: A total of 696 cases of Kawasaki disease were reported. There were 435 (62.5%) boys and 261 (37.5%) girls giving a male to female ratio of 1.7:1. The age ranged from 1 month to 15 years 5 months with a median of 1.7 years. Infants (<1 year) constituted the largest group of patients (223, 32.0%) and overall, 638 (91.7%) were younger than 5 years. Skin rash, conjunctivitis, and oral signs were among the principal clinical features present in over 80% of cases. Prominent cervical lymph nodes larger than 1.5 cm were less commonly found (24%). Coronary artery aneurysms or ectasia were present in 15.7% (109/696), 8.5% (59/696), and 5.0% (35/696) of patients at 2, 4, and 8 weeks, respectively. The incidence of Kawasaki disease per 100,000 children under 5 years was significantly higher in the prospective study period than in the retrospective period (39 vs 26, P<0.001). CONCLUSION: The incidence of Kawasaki disease is high in Hong Kong and is 39 per 100,000 children below 5 years of age. The coronary artery aneurysm prevalence is 5%. Intravenous gamma-globulin and high-dose aspirin is the mainstay of treatment.

Infection control for SARS in a tertiary paediatric centre in Hong Kong.

Severe acute respiratory syndrome (SARS) is an emerging infectious disease. After the appearance of an index patient in Hong Kong in February 2003, SARS outbreaks occurred rapidly in hospitals and spread to the community. The aim of this retrospective study is to evaluate the effectiveness of a triage policy and risk-stratified infection control measures in preventing nosocomial SARS infection among paediatric healthcare workers (HCWs) at the Prince of Wales Hospital, a general hospital to which children with SARS are referred in Hong Kong. The acute paediatric wards were stratified into three areas: (1) ultra high-risk area, (2) high-risk area and (3) moderate-risk area according to different risk levels of nosocomial SARS transmission. The implementation of different levels of infection control precautions was guided by this risk stratification strategy. Between 13 March and 23 June, 38 patients with probable and suspected SARS, 90 patients with non-SARS pneumonia, and 510 patients without pneumonia were admitted into our unit. All probable SARS cases were isolated in negative-pressure rooms. Twenty-six HCWs worked in the ultra high-risk area caring for SARS patients and 88 HCWs managed non-SARS patients in other ward areas. None of the HCWs developed clinical features suggestive of SARS. In addition, there was no nosocomial spread of SARS-associated coronavirus to other patients or visitors during this period. In conclusion, stringent infection control precautions, appropriate triage and prompt isolation of potential SARS patients may have contributed to a lack of nosocomial spread and HCW acquisition of SARS in our unit.

Hurler's syndrome with cor pulmonale secondary to obstructive sleep apnoea treated by continuous positive airway pressure.

A 6-year-old boy with Hurler's syndrome presented with right heart failure and pulmonary hypertension secondary to severe obstructive sleep apnoea. Both his sleep apnoea and cor pulmonale were effectively controlled with continuous positive airway pressure therapy.

A longitudinal study of cardiac function in children with cancer over 40 months.

A previous study demonstrated impaired systolic function in 29% of patients treated with anthracycline as part of their therapy for malignant disease. A follow-up echocardiographic study was performed to determine whether there had been further deterioration of cardiac function. At least 40 months after the first study, those patients in whom abnormal systolic function had been detected and who had not received further anthracycline were studied by echocardiography using the same protocol as the initial study (group A). A second group of pediatric oncology patients who had not been given anthracycline but who had previously had cardiac assessment was selected as a control group (group N). The age and sex distributions of the two groups were comparable. Group A comprised 29 patients assessed on 2 occasions at mean times of 46 months and 89 months from the last dose of anthracycline. The mean dose of anthracycline received was 233 mg/m2 (range 20-400). Nine of 16 patients and 4 of 5 patients who had abnormal ejection fraction (EF) and fractional shortening (FS) at first assessment had normal EF and FS at the second assessment. There were no significant changes in EF, FS, and left ventricular wall stress (LVWS) between the two examinations. In group N, 20 patients were assessed after a mean interval of 43 months. There were no significant changes in EF, FS, or LVWS between the two examinations. At the first but not the second examination there were significant differences in the left ventricular internal diameters, EF, FS, and LVWS between group A and group N. Mildly abnormal cardiac indices detected in children after cessation of treatment with anthracycline did not deteriorate in 3 to 4 years follow-up. A longer cardiac follow-up study is indicated to assess the late outcome.

Cerebral blood flow during vasovagal syncope induced by active standing or head up tilt.

BACKGROUND: Vasovagal syncope is usually associated with a sudden drop of blood pressure and/or heart rate. However, occasionally the symptoms of syncope induced by orthostatic stress testing are not associated with obvious haemodynamic changes. The mechanisms of syncope in these patients are not clear. AIM: To evaluate changes in cerebral blood flow velocities during orthostatic stress testing in children and adolescents with vasovagal syncope. METHODS: Electrocardiogram, instantaneous arterial blood pressure, and right middle cerebral artery blood flow velocity were recorded at rest, during active standing, and 80 degrees head up tilt. 32 children and adolescents aged between 7 and 18 years with a history of repeated vasovagal syncope and 23 healthy control subjects were studied. RESULTS: Presyncope occurred in 10 patients during standing, and 13 patients during head up tilt. None of the controls had symptoms during the test. The transcranial Doppler study showed that the symptoms were associated with significant decreases of diastolic cerebral blood flow velocity and an increase of pulsatility. There was no significant change of the systolic cerebral blood flow velocity. The changes of cerebral blood flow velocities occurred in all episodes of presyncope, including those not associated with severe drop of blood pressure or heart rate. CONCLUSIONS: Diastolic cerebral blood flow velocity decreased significantly during episodes of presyncope induced by orthostatic stress. Impairment of autoregulation of cerebral blood flow might play an important role in the pathophysiology of syncope.

Prenatal diagnosis of cardiac osteoma: a case report.

Primary benign intracardiac tumours in the infant period are rare. We report a case of a cardiac osteoma detected at 17 weeks of gestation. Ultrasonographically, it appeared as a calcified mass with a sharp margin and was associated with hypoplastic right ventricle. The gross and histological findings are presented.

Comparing sulindac with indomethacin for closure of ductus arteriosus in preterm infants.

OBJECTIVES: A prospective study comparing the efficacy and side-effects of oral sulindac with intravenous indomethacin in clinically stable preterm infants (< 1750 g) requiring non-invasive closure of haemodynamically significant patent ductus arteriosus. METHODOLOGY: As maturity and birthweight are the two major determinants of ductal closure, infants were matched as closely as possible for these parameters. An eligible patient was first assigned to the sulindac group and a subsequent patient with similar gestational age (+/-1 week) and birthweight (+/-100 g) to the previously recruited infant would automatically receive indomethacin. A total of eight infants were enrolled in each group. RESULTS: The ductus arteriosus was successfully closed in all eight infants receiving indomethacin, and in seven of eight infants receiving sulindac. No significant differences were found with regards to the ductal size between the two groups at diagnosis or on each of the consecutive days of treatment (P > 0.25). More renal adverse effects were encountered in the indomethacin group. Significant differences in changes from baseline value for urine output, plasma sodium, urea and creatinine concentrations were noted at 24, 48 and 72 h after commencement of treatment between the two groups (P < 0.05). All the parameters returned to normal or pre-treatment levels 48 h after stopping therapy. Unexpectedly, severe gastrointestinal complications were encountered in the sulindac group. CONCLUSIONS: Sulindac is capable of promoting ductal constriction in clinically stable preterm infants without compromising the renal function. The spectrum of gastrointestinal complications observed in sulindac treated infants were similar to those described for indomethacin. The use of sulindac for ductal closure in the preterm infant should remain experimental.

Isolated congenital tricuspid valve dysplasia: a rare condition mimicking persistent pulmonary hypertension of the newborn.

Isolated congenital tricuspid valve dysplasia is a rare and potentially lethal congenital heart disease that can be easily confused with persistent pulmonary hypertension of the newborn. We describe a neonate with isolated congenital tricuspid valve dysplasia who did not respond to mechanical ventilation but improved by tolazoline. Clinicians should be aware that the initial fulminant course of this condition may be reversed by reducing the pulmonary vascular resistance, thereby allowing time for spontaneous recovery.

Mitral valve replacement for severe mitral regurgitation in infants with anomalous left coronary artery from the pulmonary artery.

Infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) may present with heart failure, mitral regurgitation, and dilated cardiomyopathy. Reestablishment of a two coronary artery system markedly improves the morbidity and mortality. However, the mitral regurgitation may continue to deteriorate despite surgical correction of the ALCAPA because of previous ischemic damage to the papillary muscles and chordae. Surgical intervention, including mitral valve replacement, may be required even during infancy. We present two infants with ALCAPA who required early mitral valve replacement for severe mitral regurgitation and have done well subsequently.