Selective screening for inherited metabolic disorders in a tertiary care hospital of Karachi - A retrospective chart review.

Background & Objective: Selective high-risk screening of children suspected of having inherited metabolic disorders was conducted jointly by Chemical Pathology section and the Pediatric Department of Indus Hospital and Health Network- (IHHN) from October 2020-March 2022. Tandem mass spectrometry (MS) for newborn screening was recently introduced in a local laboratory. We did a selective high screening of children for metabolic disorders by using MS for neonates and other relevant tests for older children in our hospital. The present study was undertaken to get an estimate of the number of metabolic cases screened and identified after inclusion of an extended workup. Methods: This is a retrospective chart review of children who were selectively screened for IMDs. Patients' records with ages ranging from birth to fourteen years of age were retrieved from the electronic records department of IHHN from October 2020 to March 2022. Records were searched for demographic data, history, signs, symptoms, and lab investigations. All relevant information was recorded on a pre-designed questionnaire. Results: A total of 178 children were screened for inherited metabolic disorders. Majority of the children screened were less than one month of age 96 (54%). Consanguinity was noted in 74 (41.5%) children. Most common symptoms observed were failure to thrive in 77 children (43%), hypoglycemia in 45 children (25%), and feeding difficulty in 36 children (20%). Inherited metabolic disorders were confirmed in 12 children out of which five had congenital adrenal hyperplasia, four had cystic fibrosis and three children had congenital hypothyroidism. Conclusion: In the present study, we were able to screen several children after inclusion of an extended metabolic workup. However, confirmation of many disorders like fatty acid oxidation defects, disorders of carbohydrate metabolism, and sphingolipidosis could not be done due to lack of confirmatory tests. We recommend that confirmatory tests of these disorders be included in local labs.

Evaluation of pain experienced by orthodontic patients following elastomeric separator insertion: A cross-sectional study.

Objective: Pain following the insertion of separators and archwires varies with age, sex, race, ethnicity, threshold, and health status. This study aimed to evaluate the characteristics of pain in orthodontic patients after the insertion of elastomeric separators, its effects on daily life, and its association with age and sex in a population not previously studied in this regard. Methods: A cross-sectional study of 130 patients undergoing orthodontic treatment included collecting data on demographics, pain experienced following the placement of separators, time of onset, duration, characteristics, change in dietary pattern or chewing side, intake of analgesics, and severity of pain on the Wong Baker's scale. The results are reported as counts and percentages. Associations between sex and age were evaluated using Pearson's chi-square test. Results: Among the 130 patients, 56.2% were 9-20 years old, 63.8% experienced pain following the insertion of separators, 22.9% had their first episode of pain at 4 hours, 56.6% experienced intermittent pain, and 37.3% experienced discomfort; 18.1% males and 81.9% females experienced pain following the insertion of separators. Pearson's chi-square test showed a significant association between pain and sex (P = 0.04). Most patients (37.3%) reported "hurts little more" for pain intensity on Wong Baker's scale and 21.7% reported all four quadrants as sites of pain. Conclusions: The pain experienced after separator insertion was associated with sex and age. Females experienced more pain than males and patients between the age range of 21 and 36 years suffered more pain during mastication than between 9 and 20 years old.

Cholera outbreak in 2022 among children in Karachi: Study of cases attending to a Tertiary Care Hospital.

Objective & Background: Repeated outbreaks of cholera have occurred in Karachi. Changing patterns in seasonality, serotypes and antibiotic resistance have been observed in these outbreaks. Recently, in the year 2022, a surge of cholera cases has been reported from Karachi during the months of April-June. This study aimed to identify clinical features, antibiotic susceptibility, complications, and response to treatment of V. cholerae infection among children attending Indus hospital, Karachi. Methods: A retrospective chart review of pediatric patients was conducted for children aged 0-16 years. All children treated for culture-proven cholera infection at Indus Hospital from March to June 2022 were included. Details of clinical features, complications, antibiotic susceptibility, and response to treatment were retrieved from the health management information system (HMIS) of the hospital. Results: Twenty children were included. The median age was 01 (0.50-3.75) years. There were 9 (45%) males and 11 (55%) females. All the culture isolates belonged to serogroup O1 Ogawa of the Vibrio cholerae. Vomiting and diarrhea were the most common symptoms. Dehydration, acute kidney injury, and shock were seen in 19 (95%), 6 (30%), and 2 (10%) children respectively. Eleven children were admitted with an average hospital stay of 5 (Median-IQR 3-6) days. The isolates were completely susceptible to tetracycline, ciprofloxacin, and azithromycin. Different antibiotics were given which included cefotaxime, ceftriaxone, doxycycline, and ciprofloxacin. All children responded completely to the antibiotics. Conclusion: In present study all V. cholerae isolates belonged to the O1 Ogawa serotype that showed complete susceptibility to tetracycline, ciprofloxacin, and azithromycin. Dehydration, electrolyte imbalance, and renal impairment were the most common complications observed. Drinking unboiled water was identified as a potential source of cholera in most children. Therefore, advocacy of hygienic practices and disinfection of water supplies is recommended to prevent future cholera outbreaks.

Diagnoses and Outcomes of Patients with Suspicion of Acute Coronary Syndrome and Raised High Sensitive Troponin I: A Single Center Study from Pakistan.

Objectives Troponins are classically raised in acute coronary syndrome (ACS) although other cardiovascular and non-cardiovascular causes are recognized. We aimed to see the association of high sensitivity (Hs) Troponin I values exceeding the sex-specific 99th percentile upper reference limit (URL) with diagnoses, emergency department (ED) outcomes, 30-day outcomes of admitted patients and predictors of ACS in both genders. Materials and Methods A retrospective study of all patients presenting to the emergency department from January 2019 to April 2021 with suspicion of ACS and Hs-Troponin I values greater than the sex-specific 99th percentile URL. Statistical Analysis SPSS version 24 was used, Pearson's chi-square tests, Fisher's exact test, Kruskal-Wallis test, Mann-Whitney U test, and odds ratios, including the 95% confidence intervals, for each characteristic were used for analysis. A p -value of < 0.05 was considered significant. Results There were a total of 5,982 patients (3,031 males, 2,951 females), out of which 878 patients were admitted under the cardiology specialty. In patients who were admitted to the ward, mortality was higher in females (8.2%) with less than a 10-fold rise in Hs-Troponin I while similar in both genders (7.6%) in patients with Hs-troponin I greater than 10-fold of sex-specific 99th percentile URL. Raised low-density lipoprotein-cholesterol was a significant factor associated with 2.4 times higher odds of ACS. Conclusion Women with Hs-Troponin values up to 10 times the URL, i.e., 15.6-160 ng/L have higher mortality than their male counterparts. LDL-cholesterol is a significant risk factor for ACS which should be controlled for its prevention.

Cytogenetic Profiling In Paediatric Acute Leukaemia; A Report On 746 Newly Diagnosed Paediatric Cases Analyzing The Spectrum Of Recurring Chromosomal Rearrangements In B Cell Lymphoblastic And Acute Myeloid Leukaemia.

Background: Cytogenetics is evolving and different molecular mechanisms we know now have proved to be of diagnostic and prognostic significance in both acute lymphoid (ALL) and myeloid leukaemia (AML). This study aims to find out and compare the occurrence of different cytogenetics in paediatric acute leukaemia. Methods: This is a cross-sectional study of diagnosed B-ALL and AML patients presenting at The Indus Hospital. We studied FISH and karyotype in BALL and FISH in AML patients. FISH analysis shows a total of 69 (12.8%) of B ALL patients had cytogenetic abnormalities. BCR-ABL1 was positive in 5.1%, ETV6/RUNX1T1 in 8.6% and KMT2A in 2.3% individuals. Karyotype reveals hyper diploidy in 24.3%, Monosomy in 1.94%, and t (1:19) and t (17:19) were observed in 5.8% and 0.24% cases respectively. FISH analysis in AML cases reveal positivity of t (8:21) in 26.4%, INV (16) in 6.1% while PML-RARA t(15:17) was done on morphological suspicion in 17 cases; all of which showed positivity; making 7.9% of the total AMLs. The study demonstrated a wide spectrum of heterogeneity in paediatric acute leukaemia. Conclusion: Hyperdiploidy was the most common cytogenetic abnormality. We report a lower incidence of t (12:21), compared to the world. We showed a higher prevalence of RUNX1/RUNX1T1 in young children. The prevalence of core binding factor AML was 32.5%.

Monocyte Distribution Width, a Novel Biomarker for Early Sepsis Screening and Comparison with Procalcitonin and C-Reactive Protein.

Objectives Monocyte distribution width (MDW) can be used for the early recognition of sepsis. The study compared the diagnostic accuracy of the MDW with two well-known sepsis biomarkers, procalcitonin (PCT) and C-reactive protein (CRP). Materials and Methods A study was conducted from July 2021 to October 2021, on 111 patients admitted to the Indus Hospital and Health Network. Patients from the ages of 1 to 90 years were enrolled if hospitalized for more than 24 hours for suspected sepsis to avoid inclusion of patients who had short-term stay in the emergency department. According to the Sequential Organ Failure Assessment score, the clinical team did the characterization of cases as with sepsis or without sepsis. SPSS version 24 was used, and the diagnostic accuracy of MDW was assessed and compared using the area under the curves (AUCs) acquired from receiver operating characteristic curves. Pearson's chi-square/Fisher's exact test (as per need) was applied to determine the association. A p -value of less than 0.05 was considered significant. Results Among 111 patients, 81 (73%) patients were labeled with sepsis and 30 (27%) were without sepsis. We have reported significantly higher MDW, PCT, and CRP levels in septic patients ( p < 0.001). The AUC of MDW was comparable with PCT (0.794). Significant cutoff value for the MDW was greater than 20.24 U with 86% sensitivity and 73% specificity. Conclusion MDW may have a predictive ability similar to PCT and CRP in terms of sepsis and, thus, can be used as a standard parameter for the timely diagnosis of sepsis.

Infantile B-lymphoblastic leukemia: a case series and review of the literature.

Infantile leukemia is a rare hematological malignancy that occurs in the first year of life. It is an aggressive disease with peculiar immunophenotypic, cytogenetic, and molecular characteristics. It can be myeloid or lymphoid in origin. More than 80% of cases involve KMT2A gene rearrangement in the lymphoblastic subset, versus 50% in the myeloid subset. In this study, we present three cases of this rare entity to add knowledge about its clinical presentation and diagnostic profiles. These cases of infantile B-lymphoblastic leukemia (B-ALL) were retrospectively reviewed at the Department of Hematology, Section Cytogenetics at Indus Hospital and Health Network. The clinical characteristics, complete diagnostic profile, immunophenotypic profile, fluorescence in situ hybridization (FISH) results, treatments, and outcomes of the patients were assessed. All three infants were girls who presented with hyperleukocytosis, and they were diagnosed by eight-color flow cytometry. FISH studies revealed KMT2A gene rearrangement in two of the three patients. Infantile B-ALL is a biologically distinct disease carrying a poor prognosis. Female preponderance, hyperleukocytosis, and hepatosplenomegaly are common findings in this subgroup. No standard protocol for this rare entity has proven ideal for managing these young infants.

Trend analysis of multi-level determinants of maternal and newborn postnatal care utilization in Pakistan from 2006 to 2018: Evidence from Pakistan Demographic and Health Surveys.

BACKGROUND: Postnatal care (PNC) is crucial for maternal and newborn health. Healthcare-seeking practices within the postpartum period help healthcare providers in early detection of complications related to childbirth and post-delivery period. This study aims to investigate trends of PNC utilization from 2006 to 2018, and to explore the effects of multi-level determinants of both maternal and newborn PNC in Pakistan. METHODS: Secondary data analysis of the last three waves of the nationally representative Pakistan Demographic and Health Surveys (PDHSs) was conducted Analysis was limited to all those women who had delivered a child during the last 5 years preceding each wave of PDHS Bivariate and multivariate logistic regression was applied to determine the association of maternal and newborn PNC utilization with multi-level determinants at individual, community, and institutional levels. RESULTS: In Pakistan, an upward linear trend in maternal PNC utilization was found, with an increase from 43.5 to 63.6% from 2006 to 2018. However, a non-linear trend was observed in newborn PNC utilization, with an upsurge from 20.6 to 50.5% from 2006 to 2013, nonetheless a decrease of 30.7% in 2018. Furthermore, the results highlighted that the likelihood of maternal and newborn PNC utilization was higher amongst older age women, who completed some years of schooling, were employed, had decision-making and emotional autonomy, had caesarean sections, and delivered at health facilities by skilled birth attendants. Multivariate analysis also revealed higher odds for women of older age, who had decision-making and emotional autonomy, and had caesarean section deliveries over the period of 2006-2018 for both maternal and newborn PNC utilization. Further, higher odds for maternal PNC utilization were found with parity and size of newborn, while less for ANC attendance and available means of transportation. Furthermore, increased odds were recorded for newborn PNC utilization with the number of children, ANC attendance, gender of child and mass media exposure from 2006 to 18. CONCLUSION: A difference in maternal and newborn PNC utilization was found in Pakistan, attributed to multiple individual (socio-demographic and obstetrics), community, and institutional level determinants. Overall, findings suggest the need to promote the benefits of PNC for early diagnosis of postpartum complications and to plan effective public health interventions to enhance women's access to healthcare facilities and skilled birth assistance to save mothers' and newborns' lives.

Impact of dedicated pediatric neuro-oncological services in a developing country: A single-institution, Pakistani experience.

INTRODUCTION: Brain tumors are the most common solid neoplasms and the second most common malignancy in the pediatric age group. Due to the complexity of their management, pediatric central nervous system (CNS) tumors are not a priority in low- and middle-income countries (LMICs). METHODS: In an attempt to improve the survival rate and overall care, we introduced a dedicated pediatric neuro-oncology service in our institute and evaluated its impact by dividing the pre- and post-era into two cohorts and comparing them: 1998-2013 (16 years: cohort A) and 2014-2019 (6 years: cohort B, after the start of dedicated neuro-oncology services). RESULTS: We observed that after the implementation of a proper neuro-oncology service, the proportion of patients treated with curative intent increased, and survival improved in cohort B. The patient volume also increased from 15.5 per year in cohort A to 44.8 per year in cohort B. The percentage of children given radiation therapy also increased significantly, while the proportion of children treated with chemotherapy remained stable. CONCLUSION: A dedicated multidisciplinary team trained and knowledgeable in the specialty of pediatric neuro-oncology can enhance and improve outcomes, and supportive care and help can provide good quality of life to children and their families with brain neoplasms.

High-Sensitivity Cardiac Troponin I Levels Below 99th Percentile Upper Reference Limit in Patients Presenting with Suspicion of Acute Coronary Syndrome (ACS) in Emergency Department at a Tertiary Care Hospital in Karachi, Pakistan.

INTRODUCTION: Troponin I levels are biomarkers of choice for diagnosis of acute myocardial infarction (AMI). However, prognostic significance of values below the 99th percentile upper reference limit (URL) in patients presenting with symptoms suggestive of Acute coronary syndrome (ACS) need further evaluation. AIM: The objectives of the study were to find the association of High sensitivity (hs)-Troponin I values below 99th percentile URL with age and the Emergency Department (ED) outcome, to determine single cut-off for safe discharge of these patients from the ED and to determine the 30-day outcome of the patients admitted under cardiac speciality. METHODS: This is a retrospective study of patients presenting with suspicion of ACS in the ED between January 2019 till April 2021 and hs-Troponin I values below 99th percentile URL. RESULTS: Among 15,441 patients, 8034 (52%) were males and 7407 (48%) were females. 9677 (63%) of the patients had hs-Troponin I values < 5 ng/L while 5764 (37%) had values between 5 ng/L and 99th percentile URL. Higher hs-Troponin I values were associated with a worse ED outcome. Serial troponin I levels were performed in only 2.4% of the cohort. Receiver operating characteristics for ACS demonstrated an AUC of 0.84 at a cut off value of 12.75 ng/L, with sensitivity (76.9%) and specificity was 75.1%. The 30-day outcome of the patients admitted under cardiac speciality revealed no mortality in either group. CONCLUSION: An overall single cut-off value of 12.75 ng/L can be used in our population for ruling our ACS provided it is unaccompanied by other supportive clinical and ECG findings.

Comparison of Clinical and Diagnostic Features of Pediatric Oncology Patients With or Without COVID-19 Infection: A Retrospective Chart Review.

There is a scarcity of data summarizing the clinical picture, laboratory, and imaging findings and outcome in children with malignancy and coronavirus disease 2019 (COVID-19) infection. This study characterizes a detailed comparison of pediatric oncology patients with and without COVID infection. A retrospective study was conducted at The Indus Hospital, Karachi, from March 2020 to June 2020. Clinical presentation, laboratory and imaging findings, disease severity, and outcome were compared between cohorts. The mean age of children with and without COVID was 8.0±4.9 and 7.4±4.1 years, respectively. Hematologic malignancy comprised the largest number of patients, followed by solid tumors. Lymphocytosis and low neutrophil-lymphocyte ratio was observed in the COVID positive group. Cardiac dysfunction (1.4% vs. 0%), acute respiratory distress syndrome (8% vs. 0%) and lower peripheral capillary oxygen saturation/fraction of inspired oxygen ratio (473 vs. 486) found to be associated with severe disease in COVID positive group (P<0.05). Overall mortality in children with COVID was 6.8% versus 2.7% in children without COVID. Pediatric patients with malignancy have different clinical features and laboratory parameters as compared with children without malignancy. Acute respiratory distress syndrome, absolute lymphocytosis and low neutrophil-lymphocyte ratio is associated with severe disease in children with malignancy and COVID infection. In contrast to adults, biochemical markers and complete blood count parameters do not help recognize COVID infection in pediatric patients with malignancy.

Impact of COVID-19 on the Children with Cancer in Six Pediatric Oncology Units (POUs) of Pakistan- A Multi-Center Study.

The study is to evaluate the impact of COVID-19 in the Pediatric Oncology Units (POUs) of Pakistan. Data from 1 April 2019 to 30 June 2019 and 1 April 2020 to 30 June 2020 for the first and second cohort, respectively, in order to compare the registration, abandonment rate, and delay in treatment. Six hundred and thirty-four were registered cases, 379 and 255 in the first and second cohort, respectively, which was significantly different <0.005. Seventy-seven were abandoned, 45 and 32 in the first and second cohort, respectively. Fifty-nine COVID-19 positive cases, 24, 4, 27, and 4 were admitted, referred, home isolated, and leave against medical advice (LAMA), respectively. Delayed treatment and reduction in new cases were observed.

Determinants of overall knowledge and health behaviours in relation to hepatitis B and C among ever-married women in Pakistan: evidence based on Demographic and Health Survey 2017-18.

BACKGROUND: In 2019, around 5 million and 10 million people were affected by hepatitis B virus (HBV) and hepatitis C virus (HCV) respectively in Pakistan. On World Hepatitis Day 2019, Pakistan's Government announced the Prime Minister's Plan to eliminate HBV and HCV from the country by 2030. In order to achieve this goal, adequate knowledge about HBV and HCV regarding mode of transmission, symptoms of the disease, and awareness about available treatments and vaccines is imperative. The present study aims to investigate the determinants related to overall knowledge about and behaviour in relation to HBV and HCV amongst married women in Pakistan. METHODS: Secondary data analysis was carried out using the Pakistan Demographic and Health Survey (PDHS) 2017-18. A series of questions regarding women's knowledge about how to avoid HBV and HCV and their health behaviour in relation to HBV and HCV were posed to 12,364 ever-married women of reproductive age (15-49 years). Bivariate and multivariable logistic and linear regression was applied to examine the effects of sociodemographic characteristics and covariates on women's overall knowledge and health behaviour regarding HBV and HCV. RESULTS: The findings highlight that the majority of women (88.3%) have heard of HBV and HCV. Nonetheless, only 34.8% had comprehensive knowledge about how to avoid HBV and HCV. Few women (11.3%) had been tested for HBV or HCV during the year preceding the survey. Furthermore, the results indicate that women living in urban areas, being older, and having more than 10 years of schooling, reported better knowledge and health behaviours regarding HBV and HCV. CONCLUSION: This study provides evidence that women's sociodemographic characteristics create differences in their overall knowledge about and attitudes towards HBV and HCV. This research emphasized that there is a need to create awareness about the causes and prevention of HBV and HCV in order to achieve the goal of eliminating these diseases in Pakistan by 2030.

An Outcome Analysis of Childhood Acute Promyelocytic Leukemia Treated with Atra and Arsenic Trioxide, and Limited Dose Anthracycline.

The overall survival of Acute Promyelocytic Leukemia (APL), reported in recent studies, is approaching to 90% wherein, arsenic trioxide (ATO) and all-trans retinoic acid (ATRA) are used as the mainstay of treatment with either limited or no use of anthracycline and cytarabine. This study is aimed to ascertain the outcome of children with APL using similar approach. A total of 30 patients with APL, registered from January 2015 to December 2018, were reviewed. Diagnosis was established on bone marrow aspirate and confirmed by the presence of PML-RARA translocation. Treatment protocol was based on Australian APML 4 study performed by Australian Leukemia Lymphoma Group (ALLG). Lumbar puncture was not performed as it was not part of the protocol due to the risk of bleeding. The mean age in current cohort was 9 years with 53% males. Seven (23.3%) patients died and three (10%) abandoned treatment during induction. Twenty patients completed the intensive phase of chemotherapy and all (100%) of them attained molecular remission (MR). One patient dropped out after MR whereas, 19 remain on follow up with no evidence of disease, reflecting disease free survival (DFS) of 95%. With a median follow up of 2.5 years (range 2.1-4.8 years) the 5 years Kaplan-Meier estimate of OS was 63% and 73%, with and without abandonment, respectively. Analysis of outcome according to risk groups revealed inferior outcome of high risk (HR) group (38% and 50% with and without abandonment, respectively) in contrast to standard risk (SR) group which showed better outcome (82% and 88% with and without abandonment, respectively). The attainment of 100% molecular remission and absence of relapse supports the effectiveness of this regimen. Moreover, it is found to be less toxic and therefore, can be conveniently managed in day-care settings.

Usefulness of convalescent plasma transfusion for the treatment of severely ill COVID-19 patients in Pakistan.

BACKGROUND: Convalescent plasma(CP) was utilized as potential therapy during COVID-19 pandemic in Pakistan. The study aimed at appraisal of CP transfusion safety and usefulness in COVID pneumonia. METHODS: Single arm, MEURI study design of non-randomized open label trial was conducted in five centers. Patients werecategorized as moderately severe, severe, and critical. The primary endpoint was a) improvement in clinical status and change in category of disease severity; secondary endpoint was b) CP ability to halt disease progression to invasive ventilation. CP transfused to hospitalized patients. Statistical tests including median (interquartile ranges), Mann-Whitney U test, Fisher's exact test using SPSS ver. 23, ANOVA and Chi-square test were applied for the analysis of results parameters before and after CP treatment. SOFA score was applied for multiorgan failure in severe and critical cases. RESULTS: A total of 50 adult patients; median age 58.5 years (range: 29-92 years) received CP with infusion titers; median 1:320 U/mL (Interquartile range 1:80-1:320) between April 4 to May 5, 2020. The median time from onset of symptoms to enrollment in trial was 3 to 7 days with shortness of breath and lung infiltration as severity criterion. In 35 (70%) recipients, oxygen saturation improved from 80 to 95% within 72h, with resolution of lung infiltrates. Primary endpoint was achieved in 44 (88%) recipients whereas secondary endpoint was achieved in 42 (84%). No patient experienced severe adverse events. A high SOFA score (> 7) correlated with deaths in severe and critical patients. Eight (16%) patients expired due to comorbidities; cardiac arrest in 2 (4%), multiorgan failure secondary to cytokine storm in 5 (10%) and ventilator associated complications in 1 (2%). CONCLUSION: CP transfusion can be used as a safe and useful treatment in moderately severe and severe patients. TRIAL REGISTRATION: The trial registration number is NCT04352751  ( https://www.irct.ir/search/result?query=IRCT20200414047072N1 ). Trial Registration date is 28th April 2020.

Clinical features and outcome of COVID-19 positive children from a tertiary healthcare hospital in Karachi.

: As the coronavirus disease 2019 (COVID-19) pandemic continues to evolve, differences in epidemiological and clinical features among pediatrics have been noticed across different countries. We describe the spectrum of COVID-19 in pediatric patients treated in tertiary health care. We conducted a retrospective chart review of pediatric patients admitted to Indus Hospital & Health care network, Korangi campus, Karachi; from April 1st, 2020 to July 31st, 2020. A total of 141 COVID-19 cases were reported, males were 81 (57%) and the median age was 8 (0.3-17) years. Moderate and severe infections were noted in 36(26%), and 17(12%) children respectively. Fever (50%) was the most common clinical feature. The SF ratio less than 264 was significantly associated with severe disease (p < .05). Lab investigations that differed significantly across disease severity groups included IL-6 levels (p < .01) and Prothrombin time (p < .05). Majority of children were advised home isolation 89 (63%), 29 (20.5%) were admitted while mortality was observed in 10 (7%) children. No significant difference was observed between children with and without malignancy. Pre-existing comorbidities are significantly associated with COVID-19 infections among children. Reduced SF ratio, elevated Prothrombin time, and interleukin-6 levels are associated with greater disease severity.

Stress-related growth of HIV/AIDS patients: Role of religion and self-esteem in mitigating perceived discrimination.

OBJECTIVE: Objectives of the present research were to i) examine association between levels of religiosity, self-esteem, perceived discrimination and stress related growth among HIV/AIDS patients and ii) find out the mediating effect of self-esteem on perceived discrimination and stress related growth among HIV/AIDS patients. METHODS: A cross sectional study was conducted on respondents registered in VCCT (Voluntary Confidential Counseling & testing) center at Civil Hospital of Jalal Pur Jattan, Gujrat. Total sample of 247 patients was selected on the basis of two stage stratified simple random sampling technique. After taking verbal consent from respondents, data was collected from HIV/AIDS patients by using a structured questionnaire. Analysis was performed by using SPSS version 21. RESULTS: Results of descriptive analysis showed that majority of respondents belonged to age group 29-37 (39.5%). Educational attainment of majority of the respondents was 6-11 years 95(38.3%), whereas, 27.4% of the respondents had monthly household income between Rs.10001-20000. Religiosity had strong correlation with self-esteem whereas it was negatively correlated with perceived discrimination. Perceived discrimination and level of stress related growth were negatively correlated with each other. CONCLUSIONS: Results of correlation and regression analysis showed that level of religiosity and self-esteem had significant effects in reducing perceived discrimination among HIV/AIDS patients. The reduced perceived discrimination further assisted in improving the stress related growth among respondents.

Hypercalcemia and Disseminated Osteolytic Lesions With Normal Blood Counts and Absence of Circulating Blasts: A Rare Presentation of Childhood B-Lymphoblastic Leukemia.

Hypercalcemia and disseminated osteolytic bone lesions are a rare presentation of pediatric acute lymphoblastic leukemia (ALL). The authors report a 3-year-old boy who presented with hypercalcemia and diffuse osteolytic lesions involving axial and appendicular bones. He had normal complete blood count and the absence of blasts in peripheral smear; however, bone marrow aspirate and trephine were consistent with B-cell ALL. A review of the literature highlights the variable clinical outcome of this rare presentation depending on the presence of hypercalcemia and osteolytic lesions with or without chromosomal translocation t(17;19) and coagulation abnormalities. The patient had no coagulopathy and normal karyotype, and showed excellent response to initial treatment in terms of complete remission and negative minimal residual disease after standard-risk induction chemotherapy. Hypercalcemia with diffuse osteolytic lesions warrants bone marrow examination to rule out leukemia even in the absence of any abnormality in complete blood count. The case was reported for awareness of this rare presentation of ALL so that delays can be avoided for this potentially curable but life-threatening disease.

Outcome of Core Binding Factor Acute Myeloid Leukemia in Children: A Single-Center Experience.

Childhood acute myeloid leukemia (AML) harboring core binding factor (CBF)-associated translocations are considered as a favorable cytogenetic subgroup. The 2 major subtypes of CBF-AML include t(8;21) and inversion of chromosome 16, accounting for ∼25% of patients. Because of expensive and toxic treatment, which may require hospitalization during the entire course of induction chemotherapy, most of the centers in Pakistan neither workup for this low-risk entity nor offer curative treatment. Therefore, we adopted an approach of screening AML cases for the presence of CBF with the rationale of offering curative treatment to this subgroup. Data of 244 cases were reviewed, and translocations were found in 72 (34%) patients among them, 59 (82%) had t(8;21) and 13 (18%) showed inversion of chromosome 16. The event-free survival with and without abandonment was 36% and 40%, respectively. Among 44 patients who completed treatment, 26 (59%) are leukemia-free, while 18 (41%) relapsed. None of the relapsed patients received salvage chemotherapy or hematopoietic stem cell transplant. Treatment-related mortality and abandonment was found in 24% and 10% of patients, respectively. The frequency of CBF-AML is higher in our study; however, poor outcome demands holistic measures in supportive care to improve the survival.

A distinct molecular mutational profile and its clinical impact in essential thrombocythemia and primary myelofibrosis patients.

BACKGROUND: Classical MPNs including ET and PMF have a chronic course and potential for leukaemic transformation. Timely diagnosis is obligatory to ensure appropriate management and positive outcomes. The aim of this study was to determine the mutational profile, clinical characteristics and outcome of ET and PMF patients in Pakistani population. METHODS: This was a prospective observational study conducted between 2012 and 2017 at NIBD. Patients were diagnosed and risk stratified according to international recommendations. Response to treatment was assessed by IWG criteria. RESULTS: Of the total 137 patients analysed, 75 were ET and 62 were PMF. JAK2 positivity was seen in 51 cases (37.2%), CALR in 41 cases (29.9%), while triple-negative in 17 (12.4%) cases. None of the patients in the present study were MPL positive. Overall survival for patients with ET and PMF was 92.5 and 86.0% respectively and leukaemia free survival was 100 and 91.6% respectively, at a median follow-up of 12 months. Leukaemic transformation occurred in 6.5% of MF patients; among them, JAK2 mutation was frequently found. Molecular mutations did not influence the OS in ET whereas in PMF, OS was shortest in the triple-negative PMF group as compared to the JAK2 and CALR positive patient groups. CONCLUSION: This study shows a different spectrum of molecular mutations in ET and PMF patients in Pakistani population as compared to other Asian countries. Similarly, the risk of leukaemic transformation in ET and PMF is relatively lower in our population of patients. The factors responsible for these phenotypic and genotypic differences need to be analysed in large scale studies with longer follow-up of patients.