Yip Wc

Immunosuppressive therapy in acute myocarditis: an 18 year systematic review

Background: Immunosuppressive therapy is reportedly ineffective in adults with acute myocarditis. Aims: To systematically review the impact of immunosuppressive therapy on the outcome of acute myocarditis in children. Methods: A literature search for articles published from 1984 to 2003 was conducted with the following keywords: myocarditis, dilated cardiomyopathy, and immunosuppression. The relevant studies were systematically reviewed and comparison of treatment effect was made by calculating the odds ratio (OR) and confidence interval (CI) using the exact method based on the exact discrete reference distribution. Results: Of the 1470 articles found, only nine studies were eligible. The odds for improvement with immunosuppression was between 4.33 (95% CI 0.52 to 52.23) and 2.7 (95% CI 0.59 to 14.21). Addition of a second immunosuppressive agent to prednisolone only proved effective in one randomised controlled trial (OR 0.09, 95% CI 0.01 to 0.52). Heterogeneity of these studies precluded pooled odds ratio. Conclusion: Current data suggest that immunosuppressive therapy does not significantly improve outcomes in children with acute myocarditis and there is insufficient evidence for its routine use. However, statistical power to detect a significant difference in the treatment effect may be limited because of the small number of subjects. This, together with problems of diagnosis, varying treatment practices, and a relative lack of evidence based guidelines would support efforts for a large multicentre, randomised controlled trial to better define the role of immunosuppression in acute myocarditis.

Analysis of P wave and P dispersion in children with secundum atrial septal defect.

Background: P maximum and P dispersion are evaluated as predictors of paroxysmal atrial fibrillation in adults. In this study, these variables are being investigated in children with secundum ASD in comparison with that of normal controls and in relation to size of ASD and the presence or absence of atrial dilation.

Outcome of surgical closure of doubly committed subarterial ventricular septal defect.

BACKGROUND From 1986 to March 1997, 128 patients diagnosed to have doubly committed subarterial ventricular septal defects (VSD) were reviewed. Patients with aortic regurgitation (AR), and aortic valve (AV) deformity or a large left-to-right shunt across the VSD were offered operation. Forty-five patients (27 men, 18 women) agreed to surgical closure of their VSDs. METHODS Thirty-eight patients had VSD closure alone, and 7 had an additional AV repair. Other associated defects corrected at operation were closure of atrial septal defects, closure of other ventricular septal defects, ligation of patent ductus arteriosus, and repair of ruptured sinus Valsalva aneurysm. RESULTS There was no mortality nor major morbidity associated with operation. In the 26 patients with AR and AV deformity preoperatively, valve repair was performed in 6 patients. The condition of AR improved in 4, and remained unchanged in 22 patients. In the 10 patients with a deformity of the AV and no AR preoperatively, the condition remained unchanged in 5 patients, from whom 1 had valve operation, but progressed in 5 patients postoperatively at a mean follow-up of 6.4 years. In 9 patients with no deformity of the AV and no AR preoperatively, there was no postoperative AR and no progress of valve deformity. CONCLUSIONS Excellent results were obtained with VSD closure and AV repair. Surgical closure of VSD, if performed before the onset of AV deformity, may prevent progressive AR. If AV repair is performed after the onset of AV deformity, progressive AR may not always be prevented.

Value of abdominal sonography in the assessment of children with abdominal pain

We retrospectively evaluated the usefulness of sonography in the diagnostic assessment of children with abdominal pain.

Abdominal Coarctation and Alagille Syndrome

Structural cardiac defects such as peripheral pulmonary stenosis are well-described in Alagille syndrome (AS), which is transmitted in an autosomal dominant inheritance. The genetic defect, with incomplete penetrance and variable expression, is localized to the short arm of chromosome 20. Abdominal coarctation is an uncommon congenital anomaly, with a spectrum of symptoms that may range from hypertension, intermittent claudication to abdominal pain. The association of abdominal coarctation with AS is rarely described. We report such a patient who also had aberrations of the visceral vascular supply involving the celiac, splenic, and superior mesenteric arteries. The indications to treat the coarctation, and in the context of a patient with AS, in whom liver transplantation may be contemplated at some stage, merit discussion.

Influence of surgery on aortic valve prolapse and aortic regurgitation in doubly committed subarterial ventricular septal defect

Doubly committed subarterial ventricular septal defects should be closed surgically once aortic valve deformity is present before the onset of aortic regurgitation.

A fatal congenital myopathy with severe type I fibre atrophy, central nuclei and multicores

An unusual fatal congenital myopathy in a Chinese female infant is described. Muscle biopsy showed type I fibre smallness with central nuclei and focal decrease in oxidative enzyme activities affecting mainly larger type II fibres. Longitudinal sections from glutaldehyde-fixed araldite-embedded material stained with toluidine blue revealed multiple small foci of myofibrillar degeneration (multicores) along the muscle fibres. Electron-microscopic examination confirmed the presence of multicore lesions affecting mainly the larger fibres. In addition, there were definite degenerative changes involving the smaller fibres with central nuclei. The degenerative process started around the pericentronuclear zones with diffuse extension along the whole length of the muscle fibres resulting in severe atrophy. These degenerative changes were similar to those described in pericentronuclear myopathy. It is therefore suggested that the patient might have either had 2 co-existing myopathies viz. multicore disease and pericentronuclear myopathy or a single entity with combinations of features which had not hitherto been described.

Emotional and social effects of congenital heart disease in Singapore.

A cohort of patients with congenital heart disease (n=29) who had undergone corrective cardiac surgery was studied and compared with normal controls. The mean age of cardiac patients was 10.3 years with a range of 6 years to 16 years. Their emotional maladjustment as measured on a behavioural checklist was significantly higher (P < 0.01) than controls, and their social adjustment was also similarly less satisfactory (P < 0.05). Regression analysis indicated that emotional maladjustment in the child was related to maternal maladjustment (P < 0.01), maternal guilt (P < 0.01) and maternal anxiety (P < 0.05). Social maladjustment in the child was significantly related to maternal guilt (P < 0.05) and pampering (P < 0.02). It was considered that the child with heart disease was perceived and ‘labelled’ by the mother as abnormal even after surgery and was therefore the object and focus of maternal guilt and anxiety. Hence, the childs emotional and social maladjustment is probably the result of maternal factors rather than the physical handicaps of the illness.

Nephrotic syndrome in Kawasaki disease.

Abstract Renal manifestations in Kawasaki disease are rare, and nephrotic syndrome in this disorder has not been described previously. We report a 3 month old infant with Kawasaki disease, presenting with nephrotic syndrome which responded to steroid therapy. The patient, however, developed coronary aneurysms and died of acute myocardial infarction.

The prevalence of obesity in Singapore primary school children

ABSTRACT. The purpose of this study is to survey the prevalence of obesity in Singapore primary school children from 1976 to 1980. The number of children screened comprised of 221,988 in primary I and 218,104 in primary VI. The male to female ratio was 1.04:1. Obesity is defined as body weight above 120% of Harvard standard weight for height. The overall prevalence rate was 3.51% with a significantly higher rate in boys (3.95%) than in girls (3.06%), p < 0.0001. This sex difference prevailed in both the primary I and primary VI group. Furthermore, primary VI students had a higher prevalence rate (4.29%) compared to the primary I students (2.75%) p < 0.0001. The prevalence rates were rising over the years with a rate of 5.33% in 1980 compared to that in 1976 (1.80%). Our observations suggest that the problem of obesity is an increasing one. The tendency to become obese increases with age and boys are more prone to obesity.