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Biomedical subjects

W A Ariffin

Publications and source records attributed to W A Ariffin.

At least 19 recordsLinked to original sources
PubMed1997

Intraabdominal desmoplastic small round cell tumour in an 11-year-old boy.

A case of desmoplastic small round cell tumour (DSRCT) is presented. This aggressive and rare neoplasm predominantly affects males and is almost exclusively intraabdominal in location. It is unique in that neural, mesenchymal and epithelial markers are co-expressed. Despite multi-modal therapy, the prognosis is extremely poor. The present report details the clinical features and typical pathological findings of DSRCT in an 11-year-old boy, who succumbed to the disease 16 months after diagnosis despite multiple chemotherapeutic regimes.

Abdominal Neoplasms↗
PubMed1996

Congenital dyserythropoietic anaemia: report of three cases.

Between January 1985 and June 1992, the Paediatric Department of Hospital Universiti Sains Malaysia has diagnosed congenital dyserythropoietic anaemia in three children, two of whom were siblings. The age of onset ranged from 1 to 3 years. All of them became transfusion-dependent before the age of 4 months. One of them was successfully treated with bone marrow transplantation.

Anemia, Dyserythropoietic, Congenital↗
PubMed1995

Empyema thoracis in hospitalized children in Kelantan, Malaysia.

In this study, 31 cases of childhood empyema thoracis admitted over 4 1/2 years to the Hospital Universiti Sains Malaysia, in Kelantan, Malaysia, were retrospectively reviewed. Twenty-two males and nine females were included, with a mean age of 1.9 years (range: 26 days to 7 years). Frequent symptoms were fever, cough, and dyspnoea, while common signs were temperature above 38 degrees C, decreased breath sounds, dullness to chest percussion, and intercostal recession. Radiography demonstrated unilateral moderate to large effusions in 68 per cent of cases, while consolidated lung was seen in 45 per cent of patients. Pleural fluid cultures were positive for Staphylococcus aureus (48 per cent), Streptococcus pneumoniae (7 per cent), while no growth was seen in 42 per cent of cases. Ninety-four per cent of children had a blood leukocytosis above 10 000 cell/mm3, but blood cultures were negative in 21 out of 26 patients (81 per cent). Most cases were treated with a combination of intravenous antibiotics and chest tube drainage. Antibiotics and pleural tap(s) were used in the remainder. Patients stayed in hospital for an average of 20.7 days (range: 4-52 days). Surgical intervention was necessary in only four children. The mortality rate at the time of discharge was zero, with 100 per cent radiographic resolution among the 23 patients who were followed-up.

Anti-Bacterial Agents↗
PubMed1994

Haemorrhagic disease in newborn and older infants: a study in hospitalized children in Kelantan, Malaysia.

This is a retrospective study of the epidemiology, clinical features, laboratory findings, treatment and outcome of haemorrhagic disease in 42 Kelantanese infants who were admitted to Hospital Universiti Sains Malaysia during a 2-year period (1987-1988). Classical haemorrhagic disease of the newborn was the commonest presentation (48%), followed by early onset (29%) and late onset (24%) disease. Home deliveries accounted for 81% of the affected infants. Most of these babies were not given vitamin K at birth in contrast to those delivered in hospitals. All except one infant were breastfed. The six commonest presenting clinical features were pallor, jaundice, umbilical cord bleeding, tense fontanelle, convulsions and hepatomegaly. All the infants had prolonged prothrombin and partial thromboplastin times which were corrected by administration of vitamin K. Subdural haemorrhage was the commonest form of intracranial haemorrhage, followed by subarachnoid haemorrhage. The overall case fatality rate was 14%. The results of this study once again emphasize the value of vitamin K prophylaxis in the newborn.

Age Factors↗
PubMed1994

Bone marrow transplantation in Malaysia.

The sole BMT centre in Malaysia caters only for children. Since 1987, 89 transplants have been performed using reverse barrier nursing techniques. The overall survival rate is 73% with the majority of survivors leading normal lives. The early and late infection rates of 46% and 13%, respectively, are comparable to those of other centres. Although the early septicaemia rate is 36% the immediate mortality rate is < 10%. GVHD is less frequent and severe and the interstitial pneumonitis rate lower than that in the West. The average cost of US $8000 per transplant is much lower than the cost of a transplant performed overseas. Thus we believe that our paediatric BMT programme is simple and cost-effective.

Adolescent↗
PubMed1994

A neonate with ectodermal dysplasia ectrodactyly clefting syndrome and ventricular septal defect.

A 37-week gestation male boy was born to a gravida seven para six mother by spontaneous vertex delivery at home. The baby cried at birth. On day 3 of life, he was admitted for respiratory distress. Physical examination revealed ectrodactyly, thin dry skin, anomalous tear duct with cardiomegaly. X-ray revealed absent radii, cardiomegaly and hemivertebra at L1. Echocardiogram revealed perimembranous type of ventricular septal defect. A diagnosis of Ectodermal Dysplasia Ectrodactyly Clefting Syndrome with ventricular septal defect was made. He was managed conservatively in the nursery. However, he expired on day 27 of life following short spell of fever apnoeic episode due to neonatal sepsis.

Abnormalities, Multiple↗
PubMed1994

Serum ferritin concentrations in transfusion dependent beta-thalassaemia.

Patients on a moderate red cell transfusion programme have iron overload where the concentrations of the serum ferritin were inappropriate to increases in the transfusion load as a result of limitations of apoferritin synthesis and conversion of ferritin into haemosiderin. This study confirms the limitations for the use of estimations of the serum ferritin to evaluate the iron status in patients with expected high overload as would be seen in patients on many years of maintenance red cell transfusions in the absence of iron chelation therapy. Poor compliance, inadequate dosage of Desferal (deferoxamine), and the late initiation of iron chelation therapy were factors that were considered in the patients with failure of response to iron chelation.

Blood Transfusion↗
PubMed1993

Usefulness of the Widal test in diagnosing childhood typhoid fever in endemic areas.

Data are presented for 2382 children investigated for fever in a Malaysian hospital between 1984 and 1987 when Widal tests and blood cultures were a routine part of every fever screen. There were 145 children who were culture positive (TYP-CP) for Salmonella typhi, while 166 were culture negative but were diagnosed as having typhoid (TYP-CN). Analyses of the sensitivity and specificity of combinations of initial Widal titres in predicting a positive S. typhi culture in a febrile child (culture positive vs the rest) showed the best model to be an O- and/or H-titre of > or = 1 in 40 (sensitivity 89%; specificity 89%). While the negative predictive value of the model was high (99.2%) the positive predictive value remained below 50% even for very high titres of O and H (> 1 in 640), at which point the specificity was 98.5%, supporting the clinical view that a high proportion of the TYP-CN patients really were typhoid but were missed by culture. The TYP-CN patients showed a very similar clinical and age profile to TYP-CP patients. The length of history of fever did not affect the initial Widal titre in culture positive cases. The Widal test in children remains a sensitive and specific 'fever screen' for typhoid although it will not identify all cases. In children, lower cut-off points for O- and H-titres should be used than are generally recommended.

Agglutinins↗
PubMed1993

Case report--a neonate with nonimmune hydrops fetalis.

A post-dated intra-uterine growth retarded male Malay baby was born to a 30-year-old mother gravida II by Caesarean section. Her previous pregnancy ended in still-birth. The baby was severely asphyxiated at birth. He was intubated and immediately admitted to the neonatal intensive care unit. He had anasarca, anaemia, purpura and firm, massive hepatosplenomegaly. X-rays revealed ascites and bilateral metaphysiitis of the long bones. The haemoglobin level was 5.0 gm/dl and PCV 18.3%. Coombs' test was negative. Prothrombin time (PT) and partial thromboplastin time (PTT) were prolonged. The baby and mother were positive for Venereal Disease Research Laboratory (VDRL) and the treponema pallidum haemagglutination assay (TPHA) tests. The baby was actively resuscitated but expired at three and a half hours of life due to overwhelming sepsis associated with severe anaemia and disseminated intravascular coagulation.

Adult↗
PubMed1993

Spectrum of beta-thalassaemia mutations in transfusion dependent thalassaemia patients: practical implications in prenatal diagnosis.

The study concerned the identification of the beta-thalassaemia mutations that were present in 24 patients with beta-thalassaemia major who were transfusion dependent. The application of a modified polymerase chain reaction, the amplification refractory system (ARMS) was found to be an effective and rapid method for the identification of the beta-thalassaemia mutations. Six different mutations were detected. Seventy five percent of the patients were Chinese-Malaysians and showed the commonly occurring anomalies: 1. frameshift codon 41 and 42 (-TCTT); 2. the C to T substitution at position 654 of intron 2 (IVS-2); 3. the mutation at position -28(A to G); and the nonsense mutation A to T at codon 17. In the Malays, the common mutations seen were: 1. the G to C mutation at position 5 of IVS-1; 2. the G to T mutation at position 1 of intron 1 (IVS-1); and the A to T at codon 17. The delineation of the specific mutations present will enable effective prenatal diagnosis for beta-thalassaemia to be instituted.

Base Sequence↗
PubMed1992

Cassava (ubi kayu) poisoning in children.

Three sisters with cassava poisoning are described. A review of the toxic properties of cassava is presented together with discussion on the methods of its preparation, its adverse effects on man, its detoxification in the body, and the treatment of its poisoning.

Child↗
PubMed1992

Changing trends in the epidemiology and management of gonococcal ophthalmia neonatorum.

Between June 1985 and December 1988, 58 cases of gonococcal ophthalmia neonatorum were admitted to the neonatal unit at the University Hospital (USM). Of these, 15 (25.9%) cases were due to penicillin-resistant strains of Neisseria gonorrhoea. Of the 58 cases, 56 cases were treated effectively with a single dose of antibiotic given systemically. The mean period of recovery was shorter with spectinomycin in doses of 40 mg/kg than with cefotaxime (100 mg/kg). There was no permanent sequelae in the treated cases. An increasing incidence of infection with penicillin-resistant strains of N. gonorrhoea has been observed in the area of study.

Humans↗
PubMed1990

Snake bite in Kelantanese children: a five year experience.

The records associated with 83 children from 16 months to 12 years of age who were admitted with snake bite to Kota Bharu General Hospital and University Hospital, Universiti Sains Malaysia over a 5 year period were reviewed. Elapid bites were more common than viper bites while sea-snake bites were not recorded. Symptoms were relatively mild, the common clinical features being pain and local swelling. Antivenom therapy was required in 11 children. Only three of the 11 children developed minor adverse reactions to antivenom. Four of the 83 required ventilatory support for respiratory failure and two children died.

Antivenins↗
PubMed1990

Pyogenic meningitis in hospitalized children in Kelantan, Malaysia.

A 2.5-year retrospective study of pyogenic meningitis in hospitalized children in Kelantan was carried out with regard to aetiology, clinical features, investigation, treatment and outcome. There were 58 children with 43 cases (74.1%) occurring below the age of 1 year. Frequent presenting symptoms included fever (98.3%), fits (77.6%), anorexia (39.7%), vomiting (34.5%) and drowsiness (12.1%). On admission, 37 (63.7%) had neck stiffness, 10 (17.2%) had Kernig's sign and 32 (55.2%) had coma. CSF cultures were positive for Haemophilus influenzae in 29 (50%), Streptococcus pneumonia in 13 (22.4%) and Neisseria meningitidis in 3 (5.2%). The antibiotic sensitivity profiles showed that the three main organisms were 100% sensitive to Chloramphenicol, Streptococcus pneumoniae was 100% sensitive to penicillin, Neisseria meningitidis was 100% sensitive to penicillin and ampicillin, and Haemophilus influenzae was 90% sensitive to penicillin and ampicillin. The total hospital mortality was 18.9%. All but two of the eleven deaths occurred in children younger than 1 year. Nineteen of the 35 (54.3%) survivors attended for at least one follow-up after discharge from hospital. Of these 19 children, 47.4% had neurological sequelae.

Anti-Bacterial Agents↗
PubMed1990

A clinical, laboratory and echocardiographic profile of children with acute rheumatic fever.

A retrospective study of 42 children with acute rheumatic fever admitted to Hospital Universiti Sains Malaysia from April 1985 to March 1989 was undertaken to assess the clinical, laboratory, echocardiographic aspects and outcome. The ages of the children ranged from 5 years 9 months to 11 years 11 months. There was no significant sex difference. 69.4% were admitted between November and April with a seasonal low between May and August. Sixteen children (38.1%) were hospitalised for recurrence of rheumatic fever. Carditis was the commonest manifestation and was seen in 28 (66.6%) children, followed by arthritis in 24 (57.1%), and chorea in 3 (7.1%). Echocardiography detected abnormalities in 24 out of 35 cases and the most common echocardiographic findings were poor coaptation of mitral valve (ten) left ventricular dilatation (ten), thickened mitral valve cusps (seven) and pericardial effusion (seven). In those children followed up, there were 2 recurrences while on secondary prophylaxis and complete recovery was seen only in 11 (26.9%).

Child↗
PubMed1990

Generalised lipodystrophy.

We report a Malay girl suffering from generalised lipodystrophy, with clinical features of absence of body adipose tissue, hepatomegaly, hyperpigmentation and muscular hypertrophy. She also had hyperlipaemia, hypercholesterolemia and non-ketotic insulin-resistant diabetes mellitus. The possibility of malnutrition-related diabetes mellitus was excluded because of (a) no personal or family history of malnutrition (b) no pancreatic calcification (c) total loss of subcutaneous fat and (d) her requirement for insulin was more than 21.2 units/kg body weight which would be too high even for malnutrition-related diabetes mellitus. Attempts were made to control her diabetes initially with subcutaneous boluses insulin, then continuous intravenous insulin infusion (CIVII) and finally orally with fenfluramine and chlorpropamide.

Child↗