MyMedR

Congenital dyserythropoietic anaemia: report of three cases

Ariffin WA, Karnaneedi S, Choo KE, Normah J

J Paediatr Child Health, 1996 Apr;32(2):191- 3.

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.

Fulltext Cassava (ubi kayu) poisoning in children

Ariffin WA, Choo KE, Karnaneedi S

Med J Malaysia, 1992 Sep;47(3):231-4.

PMID: 1491651

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.

Fulltext Phenylketonuria in a six year old Malay boy--a case report

Karnaneedi S, Choo KE, Ariffin WA, Norimi M

Med J Malaysia, 1989 Sep;44(3):248-51.

PMID: 2626139

A six year old Malay boy with phenylketonuria is presented. The history, clinical examination, biochemical findings and treatment are described followed by a discussion on phenylketonuria.

Snake bite in Kelantanese children: a five year experience

Tan KK, Choo KE, Ariffin WA

Toxicon, 1990;28(2):225-30.

PMID: 2339437

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.

Fulltext Serum ferritin concentrations in transfusion dependent beta-thalassaemia

George E, Wong HB, George R, Ariffin WA

Singapore Med J, 1994 Feb;35(1):62-4.

PMID: 8009283

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.

Fulltext Generalised lipodystrophy

Choo KE, Sharifah A, Ariffin WA, Mafauzy M

Singapore Med J, 1990 Jun;31(3):289-92.

PMID: 2392709

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.

Fulltext Case report--a neonate with nonimmune hydrops fetalis

Ram SP, Ariffin WA, Kassim Z

Singapore Med J, 1993 Oct;34(5):459-61.

PMID: 8153701

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.

Single-daily ceftriaxone plus amikacin versus thrice-daily ceftazidime plus amikacin as empirical treatment of febrile neutropenia in children with cancer

Ariffin H, Arasu A, Mahfuzah M, Ariffin WA, Chan LL, Lin HP

J Paediatr Child Health, 2001 Feb;37(1):38-43.

PMID: 11168867

OBJECTIVE: Empirical antibiotic treatment for febrile neutropenic patients has been the mainstay of treatment for many years. Beta-lactam antibiotics and aminoglycosides have been the most frequently used drug combination. The purpose of this study was to evaluate the efficacy, safety, tolerance and costs of single-daily ceftriaxone plus amikacin versus thrice-daily dose of ceftazidime plus amikacin.

METHODOLOGY: One hundred and ninety-one episodes of fever and neutropenia in 128 patients from October 1997 to December 1998 were included in a prospective, open-label, single-centre study. Patients were randomly assigned to either treatment group and evaluated as successes or failures according to defined criteria. Daily assessments were made on all patients and all adverse events recorded. Univariate and multivariate analysis of outcomes and a cost analysis were carried out.

RESULTS: There were 176 evaluable patient-episodes with 51.1% in the single-daily ceftriaxone-amikacin group and 48.9% in the ceftazidime-amikacin group. There were 50 positive blood cultures: 12 Gram-positive bacteria, 33 Gram-negative bacteria and five fungi. Pseudomonas aeruginosa (P. aeruginosa) accounted for 14% of total isolates. The overall success rate was 55.5% in the ceftriaxone group compared to 51.2% in the ceftazidime group (P = 0.56). Mean time to defervescence was 4.2 days in the single-daily group and 4.3 days in the thrice-daily group. There were nine infection-related deaths; five in the single-daily ceftriaxone group. The daily cost of the once-daily regime was 42 Malaysian Ringgit less than the thrice-daily regime. There was a low incidence of adverse effects in both groups, although ototoxicity was not evaluable.

CONCLUSIONS: The once-daily regime of ceftriaxone plus amikacin was as effective as the 'standard' combination of thrice-daily ceftazidime and amikacin with no significant adverse effects in either group. The convenience and substantial cost benefit of the once-daily regime will be particularly useful in developing countries with limited health resources and in centres with a low prevalence of P. aeruginosa.

Fulltext Clinical features and associated radiological abnormalities in 54 patients with cavum septi pellucidi

Guru Raj AK, Pratap RC, Jayakumar R, Ariffin WA

Med J Malaysia, 1998 Sep;53(3):251-6.

PMID: 10968162

To determine the clinical and radiological features of the patients who were found to have cavum septum pellucidum (CSP) on the cranial computerized tomographic (CT) scans.

Fulltext Providing a cure for beta thalassaemia major

Chan LL, Lin HP, Ariffin WA, Ariffin H

Med J Malaysia, 2001 Dec;56(4):435-40.

PMID: 12014762

The current treatment options for beta thalassaemia major patients include conservative treatment with blood cell transfusions and iron chelation or stem cell transplantation. Regular blood transfusions inevitably lead to multi-organ haemosiderosis and are attended by risks of blood-borne infections. Results from stem cell transplantation are good and suggest that this should be offered as first line therapy when a matched sibling donor is available because the patient is often cured and able to live a normal life. Of 38 Malaysian children who underwent bone marrow or cord blood transplantations using matched sibling donors, 29 (76%) are now cured.

Fulltext Infantile myofibromatosis

Ariffin H, Teh KH, Looi LM, Ariffin WA, Lin HP

Med J Malaysia, 2001 Dec;56(4):497-9.

PMID: 12014771

Infantile myofibromatosis (IMF) is a rare tumour with a wide spectrum of disease activity ranging from a solitary cutaneous nodule through to a multicentric form with widespread visceral involvement. It is characterised by its unique ability to spontaneously regress and has a typical histological appearance of actin-positive fibroblasts arranged in whorls or fascicles and vessels in a pericytomatous pattern. A male infant with multiple lesions involving the subcutaneous tissue and bone from birth is described and followed-up for two years. Treatment of IMF is dependent on the location of the tumour/s with surgery or chemotherapy reserved for rapidly progressive or symptomatic disease. However, due to the low rate of recurrence and the possibility of spontaneous tumoral regression, therapeutic abstention, as practised in our patient, is justified.

Fulltext Treating high risk childhood solid tumours with autologous peripheral blood stem cell transplantation--early experience in University Hospital, Kuala Lumpur

Chan LL, Lin HP, Ariffin WA, Ariffin H, Saw MH

Med J Malaysia, 1999 Jun;54(2):175-9.

PMID: 10972026

Although survival rates for childhood cancers have improved steadily over the past two decades, the outcome for advanced stage solid tumours remains poor. Many of these tumours are chemosensitive but most chemotherapeutic regimens are limited by their haematological toxicities. Much attention is now focused on mega-dose chemotherapy followed by stem cell rescue in the treatment of disseminated neuroblastoma, rhabdomyosarcoma, germ cell tumour and brain tumours. There is a preferential shift towards peripheral blood stem cell transplantation instead of bone marrow transplantation because of its advantages of faster engraftment, decreased transfusion and antibiotic usage and shortened hospitalisation. This mode of therapy is dependent on technologies including peripheral blood stem cell harvesting, cell cryopreservation and thawing. These technologies were recently made available in Malaysia and we report our early experience.

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

Choo KE, Tan KK, Chuah SP, Ariffin WA, Gururaj A

Ann Trop Paediatr, 1994;14(3):231-7.

PMID: 7825997

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.

Fulltext Second malignant neoplasms: an increasingly recognized complication of childhood cancer

Ariffin H, Ariffin WA, Chan LL, Lam SK, Lin HP

Med J Malaysia, 1997 Jun;52(2):174-7.

PMID: 10968078

Second malignant neoplasms (SMN) are an increasingly recognized late complication seen in childhood cancer survivors. A total of 3 cases of SMN have been found in the Department of Paediatrics, University Hospital Kuala Lumpur after a 15-year experience of treating childhood malignancies. Two cases are described here. The first developed abdominal non-Hodgkin's lymphoma 3 years after undergoing an allogeneic bone marrow transplant for second relapse of acute lymphoblastic leukaemia, while the second child developed myeloid leukaemia two years after completing treatment for acute lymphoblastic leukaemia. Progress in the management of childhood cancer in Malaysia and the availability of bone marrow transplantation facilities have increased the number of childhood cancer survivors; leading to increased incidence of SMN.

Empyema thoracis in hospitalized children in Kelantan, Malaysia

Maziah W, Choo KE, Ray JG, Ariffin WA

J Trop Pediatr, 1995 06;41(3):185-8.

PMID: 7636941 DOI: 10.1093/tropej/41.3.185

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.

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

George E, George R, Ariffin WA, Mokhtar AB, Azman ZA, Sivagengei K

Med J Malaysia, 1993 Sep;48(3):325-9.

PMID: 8183146

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.

Pyogenic meningitis in hospitalized children in Kelantan, Malaysia

Choo KE, Ariffin WA, Ahmad T, Lim WL, Gururaj AK

Ann Trop Paediatr, 1990 Mar;10(1):89-98.

PMID: 1694651

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.

Typhoid fever in hospitalized children in Kelantan, Malaysia

Choo KE, Razif A, Ariffin WA, Sepiah M, Gururaj A

Ann Trop Paediatr, 1988 Dec;8(4):207-12.

PMID: 2467604

A retrospective study of 137 patients with blood culture-positive typhoid fever admitted to the paediatric unit of the Hospital Universiti Sains Malaysia was carried out to study epidemiological, clinical, laboratory and treatment aspects of typhoid fever in Kelantanese children in hospital. The male:female ratio was 1:1.1. School-children were the most affected. Cases were seen throughout the year. The five most frequently presenting features were fever, hepatomegaly, diarrhoea, vomiting and cough. Rose spots were seen in only two patients. Complications included gastritis, bronchitis, ileus, psychosis, encephalopathy, gastro-intestinal bleeding and myocarditis. Relative bradycardia was not seen. Blood and stool cultures were positive in the 1st, 2nd and 3rd weeks of illness. There was no significant difference between percentages of elevated O and H titres, whether done during or after the 1st week of illness. A four-fold rise in (O) titres occurred in 50% of cases tested. We would miss 50% of typhoid fever cases if a titre (O) equal to more than 1/160 were relied upon for diagnosis. Altogether, 46% of patients had leucopenia. Chloramphenicol was the most commonly used antibiotic. There were two deaths.

Neonatal septicaemia in Kelantan, Malaysia

Choo KE, Wan Ariffin WA, Chua SP

Ann Acad Med Singap, 1988 Jul;17(3):438-42.

PMID: 3218934

A retrospective study of 84 cases of neonatal septicaemia admitted into a neonatal unit in a rural area of Malaysia for 1 year between 1st September 1985 to 31st August 1986 was carried out to determine the spectrum of micro-organisms and predisposing factors in relation to early and late onset septicaemia. The incidence of neonatal septicaemia was 2.13 per 1,000 live-births per year and the case fatality was 41.7% with higher case fatality in those who were premature, those who presented as early onset and those who had gram negative septicaemia. The mean age of onset of septicaemia was 7.8 days (range from 2 hours to 27 days). Forty four (52%) neonates had early onset septicaemia with mean age of onset at 2.7 days; forty (48%) neonates had late onset septicaemia presenting at 13.6 days of life. Gram negative organisms such as Klebsiella, Pseudomonas, sp., E. coli and Streptococcus, especially group B Streptococcus were the major organisms in the early onset septicaemia. Staphylococcus aureus and Staphylococcus epidermidis were the major organisms responsible for the late onset septicaemia. Obstetrical factors played an important role in early onset septicaemia. Prematurity was the most common predisposing factor. Invasive diagnostic and therapeutic procedures including surgery highlighted once again the importance of these procedures in predisposing the newborn to infection.

Bone marrow transplantation in Malaysia

Lin HP, Chan LL, Tan A, Ariffin WA, Lam SK

Bone Marrow Transplant, 1994 Jun;13(6):725-9.

PMID: 7920303

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.

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