Frequent occurrence of gastric cancer in Asian kindreds with Li–Fraumeni syndrome

Type of cancer and age of onset in individuals with inherited aberrations in the tumour suppressor gene TP53 are variable, possibly influenced by genetic modifiers and different environmental exposure. Since 2009, the modified Chompret criteria (MCC) have been used to identify individuals for TP53 mutation screening. Using the TP53 mutation database maintained by the International Agency for Research on Cancer (IARC), we investigated if the MCC, mainly developed for a Caucasian population, was also applicable in Asia. We identified several differences in Asian families compared with similar Caucasian cohorts, suggesting that identification and management of Li–Fraumeni syndrome in Asia do not completely mirror that of North America and Western Europe. Early gastric cancer (<40 years) may be considered a new addition to the MCC especially for Asian families.     

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.     

Highly sensitive pork meat detection using copper(ii) tetraaza complex by electrochemical biosensor

Three copper(ii) tetraaza complexes [Cu(ii)LBr]Br (1a), [Cu(ii)L(CIO₄)](CIO₄) (2a) and [Cu(ii)L](CIO₄)₂ (2b), where L = 5,5,7,12,12,14-hexamethyl-1,4,8,11-tetraazacyclotetradeca-7,14-diene were prepared and confirmed by FTIR, ¹HNMR and ¹³CNMR. The binding interaction of complex (1a, 2a, 2b) with calf thymus DNA (CT-DNA) was investigated using UV-vis absorption, luminescence titrations, viscosity measurements and molecular docking. The findings suggested that complex 1a, 2a and 2b bind to DNA by electrostatic interaction, and the strengths of the interaction were arranged according to 2b > 1a > 2a. The differences in binding strengths were certainly caused by the complexes'' dissimilar charges and counter anions. Complex 2b, with the biggest binding strength towards the DNA, was further applied in developing the porcine sensor. The developed sensor exhibits a broad linear dynamic range, low detection limit, good selectivity, and reproducibility. Analysis of real samples showed that the biosensor had excellent selectivity towards the pork meat compared to chicken and beef meat.

The electrochemical biosensor of porcine using copper(ii) tetraaza complex gave excellent performance with a broad dynamic range, low detection limit, good selectivity and reproducibility. The biosensor could sensitively detect pork DNA in real meat samples.     

Fatigue Reliability Characterisation of Effective Strain Damage Model Using Extreme Value Distribution for Road Load Conditions

The aim of this paper is to characterise the fatigue reliability for various random strain loads under extreme value distribution while considering the cycle sequence effect condition in fatigue life prediction. The established strain–life models, i.e., Morrow and Smith–Watson–Topper, considered a mean stress effect and strain amplitude; nevertheless, it excluded the load sequence effect, which involves the fatigue crack closure that is subjected to overload or underload. A FESEM-EDX analysis is conducted to characterise the failure features that occurred on the leaf spring. A finite element is simulated to determine the critical region in order to obtain the strain load behaviour. In addition, the strain signal is captured experimentally at 500 Hz for 100 s under operating conditions for three different road loads based on the critical location obtained from the finite element analysis. The fatigue life correlation shows that the Pearson correlation coefficients are greater than 0.9, which indicates the effective strain damage model is linearly correlated with the strain–life models. The fatigue life data are modelled using extreme value distribution by considering the random strain loads as extreme data. The reliability rate for the fatigue life is reported to be more than 0.59 within the hazard rate range of 9.6 × 10⁻⁸ to 1.2 × 10⁻⁷ based on the mean cycle to the failure point. Hence, the effective strain damage model is proposed for a fatigue reliability assessment under extreme conditions with higher reliability and provides fatigue life prediction when subjected to cycle sequence effects.     

Typhoid fever in hospitalized children in Kelantan, Malaysia

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.     

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.     

Concordant childhood acute lymphoblastic leukemia in monozygotic twins

Two 4-year-old monozygotic Chinese, female twins developed concordant childhood acute lymphoblastic leukemia (ALL) within an interval of about 2 weeks. Based on morphology and cytochemistry findings of the bone marrow blast cells, a diagnosis of ALL, L1 was made. Immunophenotyping showed the blast cells of both twins expressed similar antigens, i.e. HLA-DR, CD10, CD13, CD19, CD22 and CD34. Identical blood group, same HLA (human leucocyte antigen) genotype, sex and similar appearance suggest that the twins are monozygotic. Since the bone marrow leukemic cells of both twins were identical in morphology and expressed the same antigens with almost similar percentages of positivity, it is likely that the blast cells were derived from the same single clone. Based on the single clone hypothesis, the leukemogenic event must have arisen in utero in one twin and the cells from the abnormal clone then spread to the other twin via shared placental anastomoses.