The aetiology and pathogenesis of Reye's syndrome (RS) are incompletely understood. A number of environmental toxins and biological agents, including viruses, have been postulated to cause RS, either acting alone or synergistically. Most investigations have suggested that the primary insult is in the liver mitochondria, leading to a complex biochemical catastrophe, with death from encephalopathy. Margosa oil (MO), a long-chain fatty acid compound, has been shown to cause a Reye-like syndrome with death from hepatoencephalopathy, in children in Malaysia and India. The present time-course study performed in MO-administered mice showed the development of hepatic lesions with many features of RS. MO acts rapidly, within 30 min, on the nuclei of hepatocytes inducing mitoses and binucleated cells. This is followed by mitochondrial injury, with swelling, rarefaction of matrix, loss of dense bodies, pleomorphism, and loss of ribosomes starting at 60 min. There is loss of liver glycogen, and proliferation and hypertrophy of the endoplasmic reticulum (ER), followed by the presence of lipid droplets in the hyaloplasm, and globules within dilated cisterns of the ER. Additional fatty acids from lipolysis of body adipocytes, and fat globules from intestinal MO ingestion further aggravate the liver fatty change. There is evidence of fat globule ingestion by endocytosis into hepatocytes at the level of the sinusoids. The development of microvesicular liver steatosis and glycogen depletion due to involvement of liver cell organelles occur rapidly as in RS.
Concerned by the alarming decline in breast feeding in the urban and rural areas of Malaysia, we conducted a critical review of infant feeding practices among nursing personnel from representative centres using questionnaires. It was found that although 75% of mothers breast-fed their babies at birth only 19% did so at 2 months and 5% at 6 months respectively. Chinese mothers initiated breast feeding less frequently compared with Indian or Malay mothers. The prevalence of breast feeding was higher among lower category nurses, lower income groups and those from health centres. Decision for breast feeding was based in most instances on conviction derived from reading, lectures or advice from relatives. The vast majority of mothers listed "work' as the main reason for termination of breast feeding followed by "insufficient breast milk' and satisfactory past experience with bottle feeding. The ramifications of these findings and measures to improve the prevalence and duration of breast feeding are discussed.
To verify the actual immunisation coverage in Kuala Lumpur, City Hall Health Department and the Malaysian Paediatric Association (NGO ) carried out a survey. The survey revealed that the immunisation coverage determined at the child's first birthday for BCG was 95%, DPT 3 94%, OPV 3 94%, and measles = 27% (59% at 2 years). These figures correspond closer to City Hall's estimated coverage rather than the rates projected by the Ministry of Health. The main reasons for immunisation failure were, child ill 31.8% (not brought = 20.1%, brought but not given vaccine 11.7%), lack of information 28.6%, lack of motivation 9.1%, mother too busy 9.1%. Measles immunisation coverage at 1 year was low because of wrong information on schedules. Tetanus toxoid immunisation coverage of pregnant women was low. Only 27% of children were protected against neonatal tetanus although 97% of pregnant women received antenatal care and 50% had attended other health facilities as well during pregnancy. Private medical practitioners were responsible for more than 40% of all immunisations but were not submitting returns to the Health Department. Recommendations to improve immunisation coverage include education and motivation of the public and also doctors and health personnel on prevention of missed opportunities, contraindictions to immunisation and correct schedules. (Copied from article).
A survey of workers and families resident in an oil palm estate in Malaysia revealed high incidence of parasitic infections. The commonest parasites are T. trichiura (56%), A. lumbricoides (52%), hookworm (28%), Entamoeba coli (11.3%) and Giardia lamblia (11.3%). Mixed infections by two or more parasites was seen in 46% of the subjects. Anaemia was present in 70% of children with hookworm infection. Eosinophilia was observed in 69% of subjects. One each of Hymenolepis nana and Hymenolepis diminuta infection was detected. This is the first report of Hymenolepis diminuta infection in man in Malaysia.
An outline is given of a simple cost-effective strategy aimed at the immunization of all children and pregnant women residing in the plantation sector of Malaysia. It is based on a partnership between government, nongovernmental organizations and the private sector, and is supported by UNICEF.
PIP: A cost-effective strategy aimed at the immunization of all children and pregnant women residing in the plantation sector of Malaysia is outlined. It is based on a partnership between government, nongovernmental organizations and the private sector, and is supported by UNICEF. Over a million people reside on the Malaysian plantation estates: only 17% of the estates have their own hospitals; immunization services exist on only 1.5%; 40% of the estates are at least 5 kilometers from the nearest government health facility; and 64% lack transport for workers and their dependents to seek care away from the plantations. Two nongovernmental organizations, the Malaysian Paediatric Association and the Malaysian Society of Health, initiated discussions with the United Planting Association of Malaysia. A pilot study was undertaken by the groups on 6 estates in Selangor State, which included all the children at their first birthday. Tuberculosis, diphtheria/pertussis/tetanus, poliomyelitis, and measles immunization coverages were 88%, 44%, 59%, and 66%, respectively. The association of plantations accepted the organizations' proposals for all estates to: register all births; provide free transportation to government health clinics for the immunization of all eligible children and pregnant women; and enforce immunization schedules and record-keeping. The Ministry of Health agreed to provide free immunization of children and pregnant women; send mobile teams to estates that could assemble 20 or more eligible people for immunization; provide the estates with educational materials dealing with immunization; arrange that the maintenance of the cold chain be supervised by local medical officers of health; consider the training of estate hospital assistants with the help of the nongovernmental organizations. The total immunization plan was launched in September 1990. A manual was distributed to the estate managers, hospital assistants on the estates, and the medical officers who would implement and monitor the program. It is expected that total child immunization will be achieved in the foreseeable future in the estate sector.
Glucose and steroids have been used in the treatment of children with Reye's syndrome, while carnitine and coenzyme Q10 have been the subject of some recent studies which suggest that these agents may have a role in the treatment of Reye's syndrome and Reye-like syndrome due to margosa oil poisoning. Because of the paucity of causes of Reye's syndrome seen at any one centre, the clinical variability of the disease, and limited knowledge of definite aetiologic factors, controlled clinical trials are not easy to carry out or to interpret in human cases. These caveats were overcome by evaluation of these four treatment modalities in an established margosa-oil-induced animal model of Reye's syndrome. Effectiveness of the treatment modalities was determined from clinical response and histopathologic parameters (grading of light microscopic fatty changes and ultrastructural changes in the hepatocytes). Results show that carnitine per se produces a small improvement in survival, but statistically, more significant benefit is seen with glucose administration. Carnitine plus 10% dextrose appears to produce better results. Evaluation of coenzyme Q10 and carnitine on histopathologic parameters in the liver after a sublethal dose of margosa oil showed no obvious ameliorating effect on liver pathology. Steroids (dexamethasone/methylprednisolone) had no beneficial effects in reducing mortality, affecting glycogen storage or lipid accumulation. Changes in the mitochondria, ribosomes and endoplasmic reticulum were unaltered from the groups treated with margosa oil alone. While glucose and carnitine supplements appear to be beneficial, the other modes of therapy do not seem to hold much promise in the treatment of Reye-like syndrome in the margosa-oil-induced animal model.
A review of 20 cases of neuroblastoma at the University Hospital, Kuala Lumpur from 1967 to 1980 reveals six infants aged 2 to 13 months with stage IV·S disease, associated with an unusually good prognosis. Four of the six patients presented with hepatomegaly, one had skin nodules and another paresis of the lower limbs. The primary tumour was located infra-diaphragmatically in all cases, four had disease in the bone marrow but none had radiological evidence of bone involvement. Although not systematic, with limited treatment of low dose radiation and mild chemotherapy, four patients are alive and well, one absconded and one died of septicaemia. It is important to define this special category as an unexpectedly good survival is possible with minimal therapy. Death is more likely to result from over-zealous treatment than from the disease itself.
Margosa oil (MO), a fatty acid-rich extract of the seeds of the neem tree and a reported cause of Reye's syndrome, has been used in the induction of an experimental model of Reye's syndrome in rats. It has been reported that MO causes a decrease in in vivo mitochondrial enzyme activity similar to that seen in Reye's syndrome. We have attempted to uncover some of the biochemical mechanisms of MO's toxicity by examining its effect in vitro on isolated rat liver mitochondria. Male rat liver mitochondria were isolated by centrifugation; oxygen uptake, reduced forms of cytochrome b, c + c1, a + a3, and flavoprotein, intramitochondrial concentrations of acetyl coA, acid-soluble coA, acid-insoluble coA, and ATP content were measured after incubation with and without MO. Our results reveal that MO is a mitochondrial uncoupler. State 4 respiration was increased while the respiratory control ratio was decreased. The intramitochondrial content of ATP was also decreased. There were substantial changes in the reduction of the respiratory chain components after incubation of mitochondria with MO. This decelerative effect on mitochondrial electron transport was alleviated by the addition of coenzyme Q and/or carnitine. These effects of MO on mitochondrial respiration may be due to changes in fatty acid metabolism caused by MO as MO caused a shift in the proportion of acid-soluble or acid-insoluble coA esters. Supplementary therapy with L-carnitine and coenzyme Q may be useful in the management of MO-induced Reye's syndrome.
Malignant lymphoma constitutes the third most common childhood malignancy seen at the University Hospital, Kuala Lumpur and can be categorised into Hodgkin's disease and non-Hodgkin's lymphoma. Both diseases demonstrate a higher preference for Chinese males. The majority of patients presented with stage IV disease. High default rate and poor compliance to treatment were associated with poor overall cure rates but encouraging results have been obtained in those who adhered to therapy. There is an obvious need to educate the public on the improved outlook 'or childhood malignancies and 'or earlier referral to help reduce the higher mortality and morbidity associated with advanced disease.
A review of all cases of nephroblastoma admitted to the University Hospital over a 10 year period reveals that its incidence relative to the other childhood tumours and epidemiological features are similar to other centres. The majority of patients presented with either stage III or IV disease. During the period 1968·1972 the number of defaulters was high and survival was poor. Following the introduction of treatment protocol, default rate has fallen and 5 of 7 patients have survived more than 2 years. Earlier referral and education of the parents should help improve the outcome for children with Wilms' tumour in Malaysia.
Two Malaysian Chinese male children are described with Wiskoll-Aldrich syndrome, which has rarely been reported in the East. Classical features were found, with bleeding and infections, low titres of isohaemagglutinins and IgM, but variable IgG and IgA. The immune response was broadly disturbed. Survival of one child was sufficiently long for a Iymphoreticular malignancy or appear.
A 13-year old boy with acute lymphoblastic leukemia on chemotherapy developed neutropenia and acute cellulitis progressing to fulminating septicemia due to Pseudomonas pseudomallei. Septicemic melioidosis should be considered in the differential diagnosis of a febrile illness in children who are susceptible to infections.