A case of multifocal choroiditis secondary to Candida albicans infection in a leukemic child.

Acute leukemia commonly renders a child immunocompromised, making them susceptible to opportunistic microorganism infection.We report an interesting case that presented to us with multifocal choroiditis secondary to Candida albicans infection in a Leukemic child. Appropriate management was undertaken and the infection subsided.

Nutritional and socio-economic determinants of cognitive function and educational achievement of Aboriginal schoolchildren in rural Malaysia.

A community-based cross-sectional study was carried out among Aboriginal schoolchildren aged 7-12 years living in remote areas in Pos Betau, Pahang, Malaysia to investigate the potential determinants influencing the cognitive function and educational achievement of these children. Cognitive function was measured by intelligence quotient (IQ), while examination scores of selected school subjects were used in assessing educational achievement. Blood samples were collected to assess serum Fe status. All children were screened for soil-transmitted helminthes. Demographic and socio-economic data were collected using pre-tested questionnaires. Almost two-thirds (67·6 %) of the subjects had poor IQ and most of them (72·6 %) had insufficient educational achievement. Output of the stepwise multiple regression model showed that poor IQ was significantly associated with low household income which contributed the most to the regression variance (r2 0·059; P = 0·020). Low maternal education was also identified as a significant predictor of low IQ scores (r2 0·042; P = 0·043). With educational achievement, Fe-deficiency anaemia (IDA) was the only variable to show significant association (r2 0·025; P = 0·015). In conclusion, the cognitive function and educational achievement of Aboriginal schoolchildren are poor and influenced by household income, maternal education and IDA. Thus, effective and integrated measures to improve the nutritional and socio-economic status of rural children would have a pronounced positive effect on their education.

Current prevalence and predictors of protein-energy malnutrition among schoolchildren in rural Peninsular Malaysia.

A cross-sectional study was carried out to determine the current prevalence of protein-energy malnutrition (PEM) among Orang Asli schoolchildren and to investigate the potential predictors of malnutrition. A total of 241 (120 males and 121 females) Orang Asli schoolchildren age 7-12 years living in remote areas of Pos Betau, Pahang participated voluntarily in this study. Anthropometric and socioeconomic data were collected and the children were screened for intestinal parasitic infections. The overall prevalences of mild and significant underweight conditions were 52.3% and 37.3%, respectively, and the prevalences of mild stunting and wasting were 43.6% and 43.1%, respectively, while the prevalences of significant stunting and wasting were 43.6% and 5.6%, respectively. There was a significant association between gender (male) and malnutrition (p = 0.029). The results also showed a higher prevalence of stunting among children age < or = 10 years than in older children (p = 0.001). Other independent variables, including socioeconomic status and intestinal parasitic infections, had no significant associations with malnutrition indices. PEM is prevalent among schoolchildren in rural Malaysia and therefore of public health concern since PEM diminishes immune function and impairs cognitive function and educational performance. School-based programs of prevention through health education and interventions should be considered as an essential part of measures to improve the quality of life of schoolchildren in rural Malaysia.

Anaemia and iron deficiency anaemia among aboriginal schoolchildren in rural Peninsular Malaysia: an update on a continuing problem.

A cross-sectional study to determine the current prevalence of anaemia and iron deficiency anaemia (IDA) and to investigate the possible risk factors for IDA was carried out on 241 aboriginal schoolchildren (120 boys, 121 girls) aged 7-12 years and living in remote areas in Pos Betau, Pahang, Malaysia. Haemoglobin (Hb) level was measured and serum iron status was assessed by serum ferritin (SF), serum iron (SI) and total iron binding capacity measurements. Socioeconomic data were collected using pre-tested questionnaires. All children were screened for intestinal parasitic infections. Overall, 48.5% (95% CI 42.3-54.8) of children were anaemic (Hb<12 g/dl). The prevalence of IDA was 34% (95% CI 28.3-40.2), which accounted for 70.1% of the anaemia cases. The prevalence of IDA was significantly higher in females than males. Low levels of mothers' education and low household income were identified as risk factors for IDA. Severe trichuriasis also found to be associated with low SF and SI. Logistic regression confirmed low levels of mothers' education and gender as significant risk factors for IDA. Improvement of socioeconomic status and health education together with periodic mass deworming should be included in public health strategies for the control and prevention of anaemia and IDA in this population.

Pattern and predictors of soil-transmitted helminth reinfection among aboriginal schoolchildren in rural Peninsular Malaysia.

Data on soil-transmitted helminth (STH) infections and reinfection among Orang Asli (aborigine) schoolchildren and their nutritional and socioeconomic status were analyzed to investigate the pattern and the possible predictors of STH reinfection. In this longitudinal study, 120 (60 males and 60 females) Orang Asli primary schoolchildren aged 7-12 years and living in remote areas in Pos Betau, Kuala Lipis, Pahang were screened for the presence of STH using modified cellophane thick smear and Harada Mori techniques. The overall prevalence of ascariasis, trichuriasis and hookworm infections were 65.8, 97.5 and 10.8%, respectively. After complete deworming with a 3-day course of 400mg/daily of albendazole tablets, children were re-examined at 3 and 6 months from baseline. The reinfection rate, by one or more of STH species, at 3 months after deworming was high (49.5%) while 79.6% of the children were reinfected at 6 months after deworming. Logistic regression analyses showed that females, stunted children and those living in houses without toilets had significantly higher reinfection rates than others at 3 months (P<0.05). At 6 months, maternal employment status emerged as another predictor where children of working mothers had significantly higher reinfection rates (P=0.026). In conclusion, reinfection rate of STH is high and thus necessitates frequent and periodic deworming among children. Public health personnel need to re-look at the current control measures and identify innovative and integrated ways in order to reduce STH significantly in the rural communities.

An unceasing problem: soil-transmitted helminthiases in rural Malaysian communities.

Despite great development in socioeconomic status throughout 50 years of independence, Malaysia is still plagued with soil-transmitted helminthiases (STH). STH continue to have a significant impact on public health particularly in rural communities. In order to determine the prevalence of STH among rural Orang Asli children and to investigate the possible risk factors affecting the pattern of this prevalence, fecal samples were collected from 292 Orang Asli primary schoolchildren (145 males and 147 females) age 7-12 years, from Pos Betau, Kuala Lipis, Pahang. The samples were examined by Kato-Katz and Harada Mori techniques. Socioeconomic data were collected using pre-tested questionnaires. The overall prevalence of ascariasis, trichuriasis, and hookworm infections were 67.8, 95.5 and 13.4%, respectively. Twenty-nine point eight percent of the children had heavy trichuriasis, while 22.3% had heavy ascariasis. Sixty-seven point seven percent of the children had mixed infections. Age > 10 years (p = 0.016), no toilet in the house (p = 0.012), working mother (p = 0.040), low household income (p = 0.033), and large family size (p = 0.028) were identified as risk factors for ascariasis. Logistic regression confirmed low income, no toilet in the house and working mother as significant risk factors for ascariasis. The prevalence of STH is still very high in rural Malaysian communities. STH may also contribute to other health problems such as micronutrient deficiencies, protein-energy malnutrition and poor educational achievement. Public health personnel need to reassess current control measures and identify innovative and integrated ways in order to reduce STH significantly in rural communities.

Haemophagocytic lymphohistiocytosis in Malaysian children.

OBJECTIVES: To study the clinical presentation, therapy and outcome of children diagnosed with both primary and secondary haemophagocytic lymphohistiocytosis (HLH) at the University of Malaya Medical Centre. METHODS: All patients diagnosed with HLH between 1998 and 2004 were studied. Clinico-pathological data of these patients were prospectively collected and analysed. RESULTS: Thirteen consecutive patients (eight boys) with a median age of 28 months were seen. All patients presented with high-grade unremitting fever and almost all, with hepatosplenomegaly and cytopenias. Neurological manifestations, which ranged from irritability to seizures and coma, were seen in 10 (77%) patients. Other common presenting features include liver dysfunction (46%) and skin rash (38%). All patients were treated using the HLH-94 protocol chemotherapy which consisted of a combination of etoposide, dexamethasone and cyclosporine. Complete response was seen in seven patients while two required bone marrow transplantation and one developed secondary acute myeloid leukaemia. Two patients died before treatment could be commenced. Overall mortality rate in our series was 46%. CONCLUSIONS: Haemophagocytic lymphohistiocytosis is an uncommon disease with a high fatality rate. Due to its protean clinical manifestations, it may be underdiagnosed. Early detection and prompt institution of appropriate therapy is necessary to improve the outcome in affected patients.

Treating childhood acute myeloid leukaemia with the AML-BFM-83 protocol: experience in a developing country.

Treatment for childhood acute myeloid leukaemia (AML) consists of remission induction chemotherapy followed by postremission chemotherapy with or without bone marrow transplantation. The AML Berlin-Frankfurt-Munster (BFM)-83 protocol with induction-consolidation-maintenance chemotherapy for 2 years has been reported to result in a 6-year event-free survival (EFS) and event-free interval (EFI) of 49% and 61% respectively. A total of 174 Malaysian children were treated with this protocol between 1985 and 1999. The 5-year EFS and EFI was 30.7% and 48.0% respectively. The overall mortality from sepsis was 24%, which needs urgent address. The 5-year EFS for patients treated before 1993 and after 1993 was 18.6% and 41.3%, respectively (P = 0.04), while the EFI was 32% and 60.6% respectively (P = 0.034). The improvement seen after 1993 was related to a reduction in induction deaths for that period and probably reflected increased capability and familiarity to cope with the demands of the AML-BFM-83 protocol and accompanying complications in the treatment of AML.

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

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.

Infantile myofibromatosis.

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.

Acquired immunodeficiency syndrome presenting as childhood non-Hodgkin's lymphoma.

Two children with non-Hodgkin's lymphoma (NHL) as the presenting illness of acquired immunodeficiency syndrome (AIDS) are described. There was a delay in diagnosing the underlying AIDS in both cases. In the first case, an 18-month-old boy with stage IV, high-grade,T-cell NHL, the diagnosis of underlying AIDS was suspected only when he developed recurrent and profound opportunistic infection during chemotherapy. The second case, an eight-month-old female infant presented initially with hepatosplenomegaly and thrombocytopenia of undetermined cause. She had progressive abdominal distension and swelling of her right eye one year later due to high grade B-cell NHL. She was later found to be sero-positive for HIV during pre-chemotherapy screening. As the prevalence of HIV infection continues to increase, HIV infection should be considered in the differential diagnoses of childhood hepatosplenomegaly and thrombocytopenia, and as a possible underlying cause of childhood cancer, especially NHL.

Clinical features and treatment outcome of children with myeloid antigen coexpression in B-lineage acute lymphoblastic leukemia: a study of 151 Malaysian children.

The purpose of the study was to evaluate the incidence of myeloid antigen coexpression and its prognostic significance in childhood acute lymphoblastic leukemia (ALL) in Malaysia. A retrospective study was conducted of all ALL cases (< or = 12 years old) diagnosed and treated in University Hospital, Kuala Lumpur, Malaysia between 1 January 1992 and 30 May 1995, with available immunophenotype data. Presenting features and treatment outcome of 39 B-lineage ALL patients with myeloid antigen coexpression (My+B) were compared with 112 B-lineage ALL patients without myeloid antigen coexpression (My-B) for similarity in demographic, clinical and laboratory features and their treatment outcome. My+B and My-B patients were treated with a uniform treatment protocol. Myeloid antigen coexpression was defined as more than 30% isolated leukemic cells positive for CD13 and/or CD33. The ages at diagnoses ranged from 2 months to 12 years. Median age was 4 years. The incidence of myeloid antigen coexpression was 23 per cent. Univariate analyses showed that presenting features were similar between My+B and My-B with regard to age, sex, race, FAB morphology, white cell count, hemoglobin level, platelet count, liver/spleen size, central nervous system or mediastinal involvement, presence of lymphadenopathy, and proportion of blast cells detected in the marrow. Treatment outcome were not significant between the two groups. The 2-year event free survival was achieved in 44 per cent of My+B and 57 per cent of My-B (p = 0.11). The 2-year overall survival rates were 62 per cent for My+B vs. 77 per cent for My-B (p = 0.08). This study demonstrates that myeloid antigen coexpression is fairly common and constitutes 23 per cent of childhood ALL within the Malaysian population and that it is not an adverse risk factor in childhood ALL.

Age, sex, haemoglobin level, and white cell count at diagnosis are important prognostic factors in children with acute lymphoblastic leukemia treated with BFM-type protocol.

The presenting features and treatment outcome for 575 Malaysian children (< or = 12 years of age) with newly diagnosed acute lymphoblastic leukemia (ALL), admitted to the University Hospital, Kuala Lumpur, Malaysia between 1 January 1980 and 30 May 1995 were evaluated to determine their prognostic significance. Two-year overall survival was achieved in 67 per cent of all patients and 55 per cent of patients were relapse-free at 2 years. All except 10 patients, with identified French-American-British L3 morphology were treated with the modified Berlin-Frankfurt-Munster 78 treatment protocol. Univariate analyses of failure rate conferred age, sex, white cell count and hemoglobin level as potentially significant prognostic factors. All four presenting features retained their prognostic strength in a multivariate analysis. Race, platelet count, morphological subtype, liver/spleen size, lymphadenopathy, central nervous system and mediastinal mass involvement did not show any significant effect on treatment outcome. The 2-year survival rate was significantly different with regard to age, white cell count and hemoglobin level. However, sex was not significantly related to overall survival. These prognostic factors may have implications on future stratification of risk-adjusted initial treatment in the management of childhood ALL. Our analysis of Malaysian children is similar to what could be predicted based on previous studies in other populations.

Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature.

Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the right lobe that was overlooked initially during a left lateral segmentectomy at 18 months of age for a mesenchymal hamartoma. Malignant mesenchymoma is a rare and aggressive tumor. The origin of this tumor is not well understood. There has been no direct support to the hypothesis that malignant mesenchymoma may be the malignant counterpart of mesenchymal hamartoma. The authors provide clinical and histopathologic evidence in our case that suggests the possibility of malignant mesenchymoma arising from a mesenchymal hamartoma. This case emphasizes the need for complete removal of mesenchymal hamartoma and the need for long-term follow-up to detect multifocal lesion or malignant transformation.

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

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.

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

PURPOSE: 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. METHODS: Fifty four consecutive cases of cavum septum pellucidum were detected amongst 1,281 patients who underwent cranial CT scans; their clinical and radiological features were studied. RESULTS: Recurrent seizures and developmental delay were the commonest presenting symptoms seen. Significant neurological deficits were present in 75.9% of these cases. Additional cerebral abnormalities were observed in the CT scan in 76% of cases, the commonest being cortical atrophy, cerebral infarction and hydrocephalus. CONCLUSIONS: There seems to be a strong association between CSP and certain neurological abnormalities in the population studied. Further interpretation of this study would be possible if normal population in this geographical area is screened for CSP using cranial CT scans or magnetic resonance imaging.

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

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.

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.

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.