The Journal of the Singapore Paediatric Society最新文献

Head banging is the rhythmic movement of the head against a solid object and is marked by an almost compulsive repetitiveness. The reported incidence in childhood varies from 5 to 15%. The male to female ratio is approximately 3 or 4 males to each female. While the exact etiology is unknown, the suggested etiologies include: an integral part of normal development, a kinesthetic drive, a tension releasing maneuver, an attention-seeking device, a response to restricted activity, a consequence of emotional deprivation, and a response to various acute illnesses. The onset is usually in the latter half of the first year of life and generally ends spontaneously by four years of age. Head banging generally occurs before normal sleep. The duration may vary from a few minutes to an hour. The frontal-parietal region of the head is the most frequently struck. Although head banging appears alarming, the child seldom inflicts significant damage to the head. The physical examination in children who are head bangers is usually normal. Laboratory investigations are generally not indicated. The appropriate treatment of head banging is to offer the parents a supportive and reassuring explanation that brain damage is unlikely and that the child will outgrow the problem.

Rhabdomyolysis-induced acute renal failure is rare in a child. In this paper, we describe a young girl with this condition who unfortunately succumbed to adult respiratory distress syndrome. Renal biopsy confirmed the presence of tubular myoglobin casts and acute tubular necrosis but no cause for the rhabdomyolysis could be documented.

A new method using enzymatically amplified DNA sequences for the prenatal diagnosis of alpha-thalassaemia was evaluated. DNA from a foetus at risk for alpha(0)-thalassaemia was analysed to detect the presence of alpha-globin genes. The procedure involved amplification of a 136-base-pair (bp) region of the alpha-globin gene complex between the psi alpha and alpha 2 region. Amplification was performed using a pair of oligonucleotide primers and a heat stable DNA polymerase which allowed repeated cycles of DNA synthesis at 72 degrees C. A 136 bp product was detected by gel electrophoresis indicating the foetus was not positive for Bart's hydrops foetalis. The result was confirmed using the gene mapping technique. Prenatal diagnosis of alpha-thalassaemia by DNA amplification offers two advantages over the gene mapping technique since radionucleotides are not used and results can be obtained in 3 days.

From 1979 to 1985, 8 children with clinical and biochemical features of Reye's Syndrome developed acute renal failure. Within the same period, a total of 28 cases of Reye's Syndrome were admitted to the Paediatric Units of Singapore General Hospital. Six of them were females and 2 were males. The mean age at presentation was 28.4 months and the range was between 9 months to 47 months. Four of them died eventually. There appeared to be a correlation between the eventual outcome and severity of renal failure and neurological state of admission.

126 Malaysian children, 65 boys and 61 girls from higher income families were followed-up regularly from birth to six years of age in the University Hospital, Kuala Lumpur. The study shows that for boys and girls, there is a progressive increase in the left mid-upper arm circumference from birth to six years of age. However the rate of growth is not even, being most rapid during the first four month of infancy, then rapidly decreases during the middle third of infancy and thereafter it decreases slowly and by the second year of life, there is hardly any increase in the arm circumference. The left triceps skinfold thickness curves for boys and girls rise rapidly after birth to reach a peak at about three to five months before commencing to decline and then flatten off from the second year of life. The study also shows that on the whole, boys have slightly bigger arm circumference than girls during the first two years of life. From two years of age, girls on the average have more fat than boys. However this difference is statistically not significant at the ages tested. This paper also presents the left mid-upper arm circumference and left triceps skinfold percentile charts of Malaysian boys and girls from birth to six years of age.

Kawasaki disease is diagnosed with increasing frequency in countries outside Japan. It has attracted widespread interest because of the possibility of coronary aneurysms and sudden death in the patients. While advances in treatment have been achieved, and in particular, the value of intravenous gamma-globulin therapy, the etiology of the disease is still uncertain. This paper is a brief review of some recent studies which have shed light on the pathogenesis of the disease. Studies from animal models and information from immunological abnormalities, platelet immune complex interactions and coagulation abnormalities point to the importance of immunoregulatory mechanisms in pathogenesis. Evidence so far do not support a major role for genetic factors.

Non-toxic goitres are commonly encountered in clinical practice, but in the majority the aetiology is unknown. Possible aetiological factors include iodine deficiency, biosynthesis defects, immunologic factors or goitrogens. The consequential effect on thyroid function may be clinically undetectable. In this study, serum thyrotrophin (TSH) response to thyrotrophin-releasing hormone (TRH) stimulation was studied in a group of school children with non-toxic goitres to delineate those who may benefit from treatment. Evaluation of possible aetiological factors responsible for goitrogenesis was made by assessment of thyroid autoantibodies and history of exposure to goitrogens.

To identify the factors predictive of development of TPN-related cholestasis, we conducted a historical cohort analysis of 62 VLBW infants who received TPN of which 17 developed cholestasis (27.4%). In the cholestatic group, the mean duration of TPN administration was significantly longer (25.7 days vs 8 days, p less than 0.001), the maximum daily amino-acids and lipids in the TPN infusate was significantly higher (amino-acids 2.25 vs 1.25 gm/kg/day, p less than 0.001; lipids 2.0 gm/kg/day vs 1.25 gm/kg/day, p less than 0.01), the duration of fasting was significantly longer (20.7 days vs 6.3 days, p less than 0.001) and the incidence of necrotizing enterocolitis was significantly higher (58.8% vs 15.5%, p 0.02). Using a linear discriminant analysis model with development of cholestasis as the dependent variable, the duration of TPN (p 0.0000) and the maximum daily amino-acid in infusate (p 0.0000) were found to be independent variables predictive of development of cholestasis. This model may serve to identify VLBW infants who will develop cholestasis during TPN.

Five girls, aged 2.5 years to 10 years, with Rett Syndrome are presented. The girls (four Chinese and one Indian) demonstrate the classic features of developmental regression with dementia, loss of hand function and stereotyped hand movements. There are as yet no pathognomonic laboratory markers and diagnosis can be difficult especially in the early stages before evolution of the full clinical spectrum. Adherence to current diagnostic criteria is essential. Rett Syndrome is probably not an uncommon cause of severe progressive neurological disability in girls.