Objectives: Hematopoietic stem cell transplant is increasingly being used as a curative therapeutic option for patients with hematologic malignancies and nonmalignant diseases. Here, we aimed to determine the frequency and features of typical and atypical central variants of posterior reversible encephalopathy syndrome in children who had undergone an allogeneic hematopoietic stem cell transplant procedure for any indication at a single center.
Materials And Methods: We retrospectively analyzed 101 pediatric patients between aged 6 months and 18 years who had undergone hematopoietic stem cell transplant for any indication between 2010 and 2023.
Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males.
Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey.
Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018.
Pyruvate kinase (PK) is a key enzyme of anaerobic glycolysis. The genetic heterogeneity of PK deficiency (PKD) is high, and over 400 unique variants have been identified. Twenty-nine patients who had been diagnosed as PKD genetically in seven distinct paediatric haematology departments were evaluated.
View Article and Find Full Text PDFBackground: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited.
Objectives: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection.
Method: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022.
Objectives: Allogeneic hematopoietic stem cell transplant is the only curative treatment for patients with transfusion-dependent thalassemia major. In recent years, a number of novel approaches have improved patient outcomes and quality of life by minimizing the toxicity of conditioning regimens. The objective of this study was to compare the role of treosulfan- and busulfan-based conditioning in transfusion-dependent thalassemia.
View Article and Find Full Text PDFIntroduction: Episodes of bleeding in patients with haemophilia (PwH) are associated with haemophilic arthropathy, limitations in physical performance, reduced quality of life (QoL), and gait disorders.
Aim: This non-randomized, controlled, interventional, prospective, single-centre pilot study aimed to assess the effects of an 8-week supervised therapeutic exercise program on musculoskeletal health, gait kinematic parameters (GKP), functional capacity, and QoL in adult PwH.
Methods: Nineteen PwH were allocated to an exercise group (n = 10) or a control group (n = 9).
Introduction: Effective physiotherapy interventions are required for haemophilic arthropathy (HA) of the elbow due to its biomechanical differences and contribution to upper limb functionality.
Aim: To investigate the effects of manual therapy & exercises on bleeding frequency, pain, range of motion (ROM), strength, joint health, functionality and quality of life (QoL) in HA of the elbow.
Methods: Seventeen participants with HA of the elbow were randomized as Manual Therapy & Exercises Group (MTEG = 9) and Home Exercises Group (HEG = 8).
Introduction: The effects of manual therapy (MT) on joint health and functional level in haemophilic arthropathy (HA) have relatively under-explored.
Aim: To investigate the effects of MT in HA of lower limb joints on musculoskeletal system, functional level, Functional Independence Score in Hemophilia (FISH), Hemophilia Joint Health Score (HJHS) and kinesiophobia.
Methods: Seventeen patients were randomized to the control group (CG = 9) and manual therapy group (MTG = 8).