Sociodemographic and clinical predictors of quality-of-life outcome in children and young people with primary brain tumour in Karachi, Pakistan: a prospective cohort study.

Background: Children and young people (CYP) with primary brain tumour (PBT) are at high risk for developing late effects, potentially affecting long-term quality of life (QoL). In low-income and middle-income countries, QoL has not been studied in depth in CYP. In the present study, CYP treated for PBTs in Pakistan were evaluated regarding (A) mean change in QoL scores pretreatment and 12 months post-treatment and (B) predictors of change in QoL scores 12 months post-treatment.

Addressing the critical gap: conscious sedation for pediatric oncology patients in LMICs.

Most newly diagnosed pediatric cancer patients reside in LMICs. These countries face challenges in providing quality treatment, particularly with procedures requiring stillness or causing pain. Conscious sedation (CS) is underutilized in LMICs due, causing treatment delays and adverse outcomes.

Initiation of Haploidentical Stem Cell Transplantation With Post-Transplant Cyclophosphamide in Children: A Low-Middle-Income Country Institutional Experience.

Background: Haploidentical hematopoietic stem cell transplant (HSCT) is a curative treatment especially for countries where bone marrow registries are nonexistent. We present our experience with haploidentical HSCT in pediatric patients.

Methods: Retrospective data collected and analyzed for patients ≤18 years, from January 2017 to December 2022.

Factors associated with changes in the quality of life and family functioning scores of primary caregivers of children and young people with primary brain tumors in Karachi, Pakistan: a prospective cohort study.

Background: There are limited data available, particularly in low- and middle-income countries (LMICs), on the long-term quality of life (QoL) and family functioning of primary caregivers of children and young people (CYPs) affected by primary brain tumors (PBTs). This study aimed to assess the factors associated with the mean change in QoL and family functioning scores of primary caregivers of CYP patients with PBTs 12 months posttreatment.

Methods: This prospective cohort study enrolled CYPs aged 5-21 years with newly diagnosed PBTs and their primary caregivers.

Predictors of neurocognition outcomes in children and young people with primary brain tumor presenting to tertiary care hospitals of Karachi, Pakistan: a prospective cohort study.

Introduction: Primary brain tumors are a common cause of morbidity and mortality in children and young people (CYP) globally. Impaired neurocognitive function is a potential severe consequence in primary brain tumor (PBT) survivors. There are no in-depth studies from low- and middle-income countries (LMICs) to inform management and follow-up.

Socio-demographic and antenatal risk factors of brain tumor in children and young people: A matched case-control study from Karachi, Pakistan.

Background: Brain tumors are a common cause of morbidity, disability, cognitive deterioration and mortality in children, even after treatment. Little is know about the specific causes. The study aimed to assess potential socio-demographic and antenatal factors in primary brain tumor (PBTs) in children and young people (CYP) in Karachi, Pakistan.

Case Study of Pediatric Cerebral Sinus Venous Thrombosis Center of a Low Middle-Income Country.

Pediatric cerebral venous sinus thrombosis (CVST) is rare but a potentially fatal disease requiring its understanding in local setting. In this study, we observed the clinical course, management, and outcome of pediatric patients with sinus thrombosis in a tertiary care center at Pakistan. Patients between age 0 to 18 years of both genders diagnosed with sinus thrombosis during 2011 to 2020 were included.

Risk Assessment and Outcome of Venous Thromboembolism in Pediatric Population in an Academic Care Center of a Low-Middle Income Country.

Venous thromboembolism (VTE) is a recognized complication of hospital stay in young patients in many developed countries, but such an information is largely unavailable from a low middle-income country (LMIC). This study aimed at identifying the frequency, risk factors, treatment options and outcome of deep venous thrombosis/pulmonary embolism (DVT/PE) in pediatric population in a tertiary care center from a LMIC. International classification of disease, ninth revision (ICD-9) was used to identify VTE in patients aged 0-18 years during January 2011 to September 2019.

Clinical Features and Treatment Outcomes of Children With Anaplastic Large Cell Lymphoma in Pakistan: A Multicenter Study.

Background: Different approaches have been adopted in the treatment of anaplastic large cell lymphoma (ALCL); there is a lack of consensus with regard to standard treatment. Because of paucity of data from low and middle-income countries, we reviewed the clinical features and treatment outcomes of children with ALCL.

Methods: All ALCL patients under 16 years of age diagnosed from 2005 to 2015 at Aga Khan University Hospital and The Indus Hospital were identified.

Establishment of a thalassaemia major quality improvement collaborative in Pakistan.

Objectives: The aim of this study was to establish multidisciplinary care for patients with transfusion-dependent thalassaemia (TDT) by creating a TDT quality improvement (QI) collaborative in a resource-constrained setting. This study presents our initial experience of creating this collaborative, the baseline characteristics of the participants, the proposed QI interventions and the outcome metrics of the collaborative.

Design And Setting: TDT QI collaborative is a database comprising patients with TDT from four centres in Karachi, Pakistan.

Primitive myxoid mesenchymal tumor of infancy with brain metastasis: first reported case.

Introduction: Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a recently diagnosed entity, with only a handful of cases reported to date.

Case Description: Herein, we present the occurrence of this tumor in a 2-year-old boy, initially diagnosed as primitive neuroectodermal tumor of the extremity and treated with chemotherapy and surgical resection. He later presented with a cerebellar lesion, and biopsy was consistent with PMMTI.

Clinical utility of endocrine markers predicting myocardial siderosis in transfusion dependent thalassemia major.

Background: Endocrinopathy due to iron overload is the most common morbidity whereas myocardial siderosis causing toxic cardiomyopathy is the leading cause of mortality among patients with transfusion dependent thalassemia major (TDTM). If detected early, this can be treated with aggressive chelation. T2* cardiac magnetic resonance imaging (CMR) guided chelation protocols are now the gold standard but have limited availability in low and middle-income countries.

Delayed Methotrexate Clearance Despite Carboxypeptidase-G2 (Glucarpidase) Administration in 2 Patients With Toxic Methotrexate Levels.

High-dose methotrexate has been a treatment for osteosarcoma; however, its nephrotoxic effects are considerable. Carboxypeptidase-G2 (glucarpidase) was approved by the US Food and Drug Administration in 2012 for treatment of toxic methotrexate levels. We report our experience using glucarpidase under compassionate use before Food and Drug Administration approval in 2 patients who had delayed methotrexate clearance and prolonged kidney injury despite glucarpidase administration.

Burden of Cardiac Siderosis in a Thalassemia-Major Endemic Population: A Preliminary Report From Pakistan.

Objective: To describe the initial experience and demographics of T2* cardiac magnetic resonance-based myocardial-iron quantification of transfusion-dependent thalassemia-major (TM) patients from Pakistan and the correlation with serum ferritin.

Methods: Eligible TM patients presenting between April 2014 and April 2015 to Aga Khan University Hospital, Pakistan, for T2*CMR were included. The severity of myocardial-iron deposition was defined as follows: normal T2*>20 ms, mild-moderate T2*10 to 20 ms, and severe T2*<10 ms.

Age-related toxicity in patients with rhabdomyosarcoma: a report from the children's oncology group.

On the Fourth Intergroup Rhabdomyosarcoma study, older children experienced excessive neurotoxicity, whereas younger children had increased myelosuppression. The purpose of this study was to determine whether the same pattern of toxicity was seen on the successor study when use of growth factor was required and dosing of chemotherapy was different by performing a retrospective cohort analysis on patients treated on Children's Oncology Group protocol D9803. Toxicity data were analyzed by stratifying children into 4 age groups.

High-BMI at diagnosis is associated with inferior survival in patients with osteosarcoma: a report from the Children's Oncology Group.

Background: Body mass index (BMI), at diagnosis has been associated with lower survival and increased toxicity in cancer patients. We analyzed the effect of BMI at diagnosis on therapy related toxicities and outcome in pediatric osteosarcoma patients treated on Children's Oncology Group (COG) trial INT0133.

Procedures: All patients enrolled on COG-INT0133 with height, weight and toxicity information were eligible.

Diffuse large B-cell lymphoma in an adolescent female presenting with Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis: a case report.

Introduction: Hemophagocytic lymphohistiocytosis is characterized by multisystem inflammation, resulting from prolonged and intense activation of macrophages, histiocytes and CD8+ T-cells. Due to its variable presentation and non-specific findings, timely diagnosis can be challenging. This condition has been associated with malignancies, most commonly with lymphomas and leukemias of T-cell lineage.

Pretransplant conditioning with Campath-1H (alemtuzumab) in pediatric matched unrelated hematopoietic stem cell transplants: an institutional experience.

Graft versus host disease (GVHD) remains a major cause of mortality and morbidity after matched unrelated hematopoietic stem cell transplantation (HSCT). Campath-1 H (alemtuzumab), a humanized monoclonal antibody to CD52 antigen, is thought to reduce GVHD incidence through in vivo T-cell depletion. Through the same mechanism it can potentially increase the risk of relapse by reducing the graft versus leukemia effect and possibly increase the risk of infection due to delayed immune recovery.