Acute leukaemia in children with Down syndrome in a low middle-income country.

Down syndrome (DS) is the commonest chromosomal disorder and is considered to be the most common syndrome associated with acute leukaemia. The objective of this study was to determine the characteristics of acute leukaemia in children with DS in Pakistan. It was a retrospective, cohort study conducted over a 2-year period, and the data was analysed in SPSS 20.0 in terms of descriptive statistics. Nineteen DS patients with acute leukaemia were enrolled. The proportion of DS-acute leukaemia was found to be 1.84% among all cases of paediatric acute leukaemia. The mean age of presentation was 5.5 years ± 4.3 SD with a male to female ratio of 1.1:1. The precursor B-cell ALL was found in 13 (68.4%) and acute myeloid leukaemia was found in 6 (31.6%) patients of DS. Thirteen patients (68.4%) completed treatment, while 6 (31.6%) expired due to treatment-related toxicity. Mean overall survival was 38 months ± 5.34 SD. The status of diagnosis of DS before presentation with acute leukaemia was the only statistically significant factor associated with the outcome. Few distinct characteristics of DS-acute leukaemia have been found in our population. Treatment toxicity was the sole cause of treatment failure.

Supramolecular solvent-based microextraction for the preconcentration of Pb2+ and Cd2+ prior to spectrophotometric detection.

Supramolecular solvent-based dispersive liquid-liquid microextraction technique has been developed as a preconcentration tool for the determination of trace level of Pb2+ and Cd2+. Dodecanol dispersed in tetrahydrofuran has been utilized as a supramolecular-solvent system for the extraction of analytes prior to their quantitative determination with graphite furnace atomic absorption spectrophotometer. Both Pb2+ and Cd2+, which were efficiently extracted by supramolecular solvent system, were complexed with dithizone followed by the addition of supramolecular solvent. The experimental variables that could possibly influence the extraction efficiency, i.e. pH value, temperature, sample volume, centrifugation time, rate of centrifugation, ionic strength, etc. were subjected to the optimization step. An interference study was also conducted to check the selectivity of developed method. Limit of detection calculated for Pb2+ and Cd2+ was 0.015 and 0.061 mg L-1, respectively. The limit of quantification was 0.05 and 0.2 mg L-1 for Pb2+ and Cd2+, respectively. The analytical signal was enhanced to 30 times in case of Pb2+ and 27 times in case of Cd2+. The results obtained revealed that the developed method is rapid, simple, sensitive, and efficient for the determination of both analytes in real water samples.

Prediction and evaluation of the effect of pre-centrifugation sample management on the measurable untargeted LC-MS plasma metabolome.

Optimal handling is the most important means to ensure adequate sample quality. We aimed to investigate whether pre-centrifugation delay time and temperature could be accurately predicted and to what extent variability induced by pre-centrifugation management can be adjusted for. We used untargeted liquid chromatography-mass spectrometry (LC-MS) metabolomics to predict and evaluate the influence of pre-centrifugation temperature and delayed time on plasma samples. Pre-centrifugation temperature (4, 25 and 37 °C; classification rate 87%) and time (5-210 min; Q2 = 0.82) were accurately predicted using Random Forest (RF). Metabolites uniquely reflecting temperature and temperature-time interactions were discovered using a combination of RF and generalized linear models. Time-related metabolite profiles suggested a perturbed stability of the metabolome at all temperatures in the investigated time period (5-210 min), and the variation at 4 °C was observed in particular before 90 min. Fourteen and eight metabolites were selected and validated for accurate prediction of pre-centrifugation temperature (classification rate 94%) and delay time (Q2 = 0.90), respectively. In summary, the metabolite profile was rapidly affected by pre-centrifugation delay at all temperatures and thus the pre-centrifugation delay should be as short as possible for metabolomics analysis. The metabolite panels provided accurate predictions of pre-centrifugation delay time and temperature in healthy individuals in a separate validation sample. Such predictions could potentially be useful for assessing legacy samples where relevant metadata is lacking. However, validation in larger populations and different phenotypes, including disease states, is needed.

T lymphoblastic lymphoma with BCR-ABL negative chronic myeloid leukaemia: a novel association.

Lymphoblastic lymphoma and chronic myeloid leukaemia (CML) are two distinct neoplasms with different pathogenesis and clinical presentation. We hereby share a challenging case of a child presenting with fever, leucocytosis, generalised lymphadenopathy and massive splenomegaly. He was diagnosed as having novel association of concurrent T-lymphoblastic lymphoma diagnosed on cervical lymph node biopsy with BCR-ABL negative CML on bone marrow aspirate. The study of more such cases is needed for optimal patient management.

Emotion regulation difficulties and psychopathology among Pakistani adolescents.

The main aim of this study was to examine the associations between emotion dysregulation and psychopathology in adolescence. A representative sample of 1,500 adolescent students (50% female) aged 12 to 19 years (M = 15.08, SD = 1.44) was recruited from schools and colleges located in the province of district Punjab, Pakistan, using a stratified sampling technique. Structural equation modeling (SEM) was used to test associations between five dimensions of emotion regulation difficulties and five forms of psychopathology by gender (male/female). The model provided an adequate fit to the data among girls and boys. In the model tested among boys, seven positive associations between emotion regulation difficulties and psychopathology variables were found. The model tested with girls included one negative and 13 positive associations between the study variables. Findings can be used for designing universal prevention programs to prevent the development of psychopathology.

Hepaotoprotective and nephroprotective activities of Pistacia integerrima fruit extract in paracetamol intoxicated male rabbits with effect on blood cells count.

The beneficial effects of Pistacia integerrima (PI) fruit methanol extract on some liver and kidney related parameters and blood cells count of paracetamol (PCM) intoxicated male rabbits were studied. Paracetamol intoxication caused remarkable increase in the serum ALT, AST and ALP levels. The PCM intoxicated rabbits that received PI extract orally at doses of 200 mg and 400 mg/kg b.w. /oral/day for 16 days showed significant reduction in serum ALT, AST and ALP levels (P<0.05). Liver microsections from PCM intoxicated rabbits treated with PI fruit methanol extract showed improvement in the liver histoarchitecture. The urine output of PCM intoxicated control rabbits group was significantly lower (P<0.05). The PCM intoxicated rabbits that received PI extract showed significant increase in urine output (P<0.05). The PCM intoxicated rabbits treated with PI extract also showed significant reduction in the levels of serum urea and creatinine (P<0.05). The renal creatinine clearance of PCM rabbits treated with PI extract improved significantly (P<0.05). Microsections of kidneys from PCM intoxicated rabbits treated with PI fruit methanol extract showed improvement in renal histoarchitecture. During this study, PI extract caused no improvement in the RBC count of PCM intoxicated rabbits. However, the extract caused significant increase in WBC and platelets count (P < 0.05) of PCM intoxicated rabbits. From the findings of the present research, it was concluded that oral administration of P. integerrima fruit methanol extract is beneficial for the liver and kidney related biochemical parameters and blood cells count of paracetamol intoxicated male rabbits.

Clinical features and outcome in paediatric newly diagnosed immune thrombocytopenic purpura in a tertiary care centre.

OBJECTIVE: The study aimed to demonstrate the pattern of clinical presentations and outcome of acute Immune Thrombocytopenia (ITP) in our Centre. METHODS: A descriptive, observational study was conducted by collecting and analysing the data of 103 patients of acute ITP, ageing between 1-14 years, at The Children's Hospital, Lahore from January 2016 to December 2016. We collected the data regarding age, sex, clinical presentations, history of preceding viral infections, vaccination history, laboratory values, different treatment options used, and response to the treatment concerning complete response, partial response and poor responders. Statistical analysis performed by using IBM SPSS statistics version 20. RESULTS: We retrospectively, reviewed total 103 patients cases. The median age, at the time of presentation, was 5±3.4 years while mean age was 4.5±2.9 years. The male to female ratio was 1.28:1. Mean platelet count on presentation was 7 x 109/L (range: 0-24). Twenty three (22.3%), patients had the history of preceding illness. Bruises, petechiae, epistaxis and hematemesis remained the common presentations. Six (5.8%) patients showed spontaneous recovery while 97 (94%) patients received treatment for ITP. Overall, 71 (68.9%) showed a response after treatment. Sixty-two patients (59.22%) showed loss of response and received treatment again. Among these patients, thirty-four patients (33%) developed chronic disease. CONCLUSION: Majority of patients presenting to our tertiary care centre had severe acute ITP on presentation. After management and follow-up, almost 1/3 of the patients develop chronic disease hence the incidence of developing chronic disease remained high as compared to the other centers.