Publications by authors named "Zulfikar B"

Perceptions of death can greatly impact the ability to cope with grief, making it either easier or unbearable. Research on the importance of religion and spirituality in the field of oncology, particularly among parents who have lost a child to cancer, is still in its emerging stage. This study aimed to describe the religious coping strategies of Muslim mothers who lost their children to cancer.

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Childhood cancer survival rates have improved, but survivors face an increased risk of second malignant neoplasms (SMNs), particularly thyroid cancer. This study examines the demographic, clinical, genetic, and treatment characteristics of childhood cancer survivors who developed thyroid cancer as a second or third malignancy, emphasizing the importance of long-term surveillance. A retrospective review was conducted for childhood cancer survivors treated between 1990 and 2018 who later developed thyroid cancer as a second or third malignancy.

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Childhood cancers, with leukemia at the forefront, comprise 97% acute leukemia and 3% chronic leukemia, with 75% of acute leukemias being of lymphoblastic origin. Over the past 50 years, survival rates have witnessed a remarkable increase, progressing from around 10% to achieving cure rates exceeding 90% in certain childhood ALL subgroups with the advent of combined therapies. Between 1999 and 2018, a total of 123 patients diagnosed with B-ALL were initially identified, but after applying exclusion criteria, 105 patients were included in the evaluation, who were treated with COG protocols at our center.

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Introduction: Radiosynovectomy (RS), which uses beta particle-emitting radiocolloids, has proven highly effective in significantly reducing the occurrence of haemarthroses and breaking the cycle of bleeding and synovitis. This study now presents the long-term outcomes of repeated RS in individuals with haemophilia.

Materials And Methods: Repeated RS was administered to 48 joints in 37 people with haemophilia (PwHs) diagnosed with chronic haemophilic synovitis.

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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.

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Objective: Increased survival rates in childhood cancer have led to an emphasis on the importance of treatment-related infertility. Fertility preservation methods should be explained to every patient and their families (PaFs) before treatment. Establishing good communication with PaFs is crucial in this regard despite many barriers such as cultural and financial barriers.

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Article Synopsis
  • During the COVID-19 pandemic lockdown, the study assessed respiratory viral infections in children with cancer, highlighting their clinical impact.
  • Of the 265 episodes evaluated, human rhinovirus/enterovirus was the most common pathogen identified, with significant complications noted, including a higher rate of lower respiratory tract infections.
  • The findings emphasize the necessity for quicker detection and treatment of respiratory viruses to prevent severe outcomes in this vulnerable population.
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Background: Patient-reported outcomes (PROs) reflect patient perceptions of disease and treatment and are important for evaluating new therapies.

Objectives: Evaluate the effects of once-daily concizumab prophylaxis on health-related quality of life (HRQoL), treatment burden, and treatment preference in males aged ≥12 years with hemophilia A/B with inhibitors.

Methods: Patients enrolled in the multicenter, open-label explorer7 phase 3 study (ClinicalTrials.

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Article Synopsis
  • Once-weekly efanesoctocog alfa was tested in a phase 3 study for children under 12 with severe hemophilia A, showing promising results in preventing bleeding and maintaining factor VIII activity.
  • The study enrolled 74 patients, none of whom developed factor VIII inhibitors, and most experienced non-serious adverse events during the treatment.
  • With low annualized bleeding rates and a significant percentage of patients experiencing no bleeding episodes, efanesoctocog alfa demonstrated safety and effectiveness for this age group.
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 Activated prothrombin complex concentrate (aPCC) is indicated for bleed treatment and prevention in patients with hemophilia with inhibitors. The safety and tolerability of intravenous aPCC at a reduced volume and faster infusion rates were evaluated.  This multicenter, open-label trial (NCT02764489) enrolled adults with hemophilia A with inhibitors.

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Fitusiran, a subcutaneous investigational small interfering RNA therapeutic, targets antithrombin to rebalance hemostasis in people with hemophilia A or B (PwHA/B), irrespective of inhibitor status. This phase 3, open-label study evaluated the efficacy and safety of fitusiran prophylaxis in males aged ≥12 years with hemophilia A or B, with or without inhibitors, who received prior bypassing agent (BPA)/clotting factor concentrate (CFC) prophylaxis. Participants continued their prior BPA/CFC prophylaxis for 6 months before switching to once-monthly 80 mg fitusiran prophylaxis for 7 months (onset and efficacy periods).

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Background: Concizumab is an anti-tissue factor pathway inhibitor monoclonal antibody designed to achieve hemostasis in all hemophilia types, with subcutaneous administration. A previous trial of concizumab (explorer4) established proof of concept in patients with hemophilia A or B with inhibitors.

Methods: We conducted the explorer7 trial to assess the safety and efficacy of concizumab in patients with hemophilia A or B with inhibitors.

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Objective: Patients with inherited bleeding disorders faced problems in accessing healthcare during coronavirus disease 2019 pandemic. This study aimed to investigate the health problems of patients with inherited bleeding disorders during the coronavirus disease 2019 pandemic.

Material And Methods: Children and adult patients with inherited bleeding disorders who had a coronavirus disease 2019 infection between March 2020 and November 2021 were retrospec- tively evaluated.

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Objective: Hemophilia is an uncommon disorder that is difficult to diagnose and manage. Effective movement and individual physiotherapy interventions can improve physical activity levels, quality of life, and participation in children with hemophilia. This study aimed to investigate the effects of individually planned exercise on joint health, functional level, pain, participation, and quality of life in children with hemophilia.

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Article Synopsis
  • The study aimed to analyze the efficacy and safety of recombinant von Willebrand factor (rVWF) in patients with type 3 von Willebrand disease (VWD).
  • It involved a post hoc analysis of a phase 3 trial, comparing annualized bleeding rates (ABRs) in two groups: those with a history of on-demand treatment and those switching from plasma-derived VWF prophylaxis.
  • Results showed a significant reduction in annualized bleeding events for the on-demand group (91.6% decrease) and a maintained control in the switching group (47% decrease), with minimal adverse events reported.
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Article Synopsis
  • * A study was conducted comparing 36 hemophilic patients and 37 non-hemophilic patients undergoing TKA, revealing that hemophilic patients used significantly more pain medication and had longer hospital stays.
  • * The findings suggest that pain management for hemophilia patients post-surgery should be tailored and proactive to better control acute pain.
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Objective: The purpose of this study was to describe factors affecting the place of death of children with cancer at the end of life.

Methods: The descriptive phenomenological approach was used. Eighteen mothers who lost their children to cancer participated in in-depth interviews.

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Article Synopsis
  • Haemophilia is an X-linked bleeding disorder that leads to frequent bleeding episodes due to low levels of coagulation factors (factor VIII for Haemophilia A and factor IX for Haemophilia B), with treatment primarily involving factor replacement.
  • Current treatments face challenges like short factor half-life, the need for frequent injections, and potential side effects, but advancements like extended half-life factors and gene therapy show promise for better outcomes.
  • The review discusses the importance of addressing the unmet needs in haemophilia treatment, particularly through gene therapy, while highlighting challenges related to vector immunogenicity and patient selection, especially in developing countries.
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Introduction: Inherited factor VII (FVII) deficiency (FVIID) is the most common of inherited rare bleeding disorders. Other determinants of clinical severity apart from FVII level (FVIIL) include genetic and environmental factors. We aimed to identify the cut-off FVIILs for general and severe bleedings in patients with FVIID by using an online national registry system including clinical, laboratory, and demographic characteristics of patients.

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Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding disorder characterized by impaired platelet aggregation due to defects in integrin αIIbβ3, a fibrinogen receptor. Platelet phenotypes and allelic variations in 28 Turkish GT patients are reported. Platelets αIIbβ3 expression was evaluated by flow cytometry.

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Background/aim: Glanzmann thrombasthenia (GT) is a rare autosomal recessively inherited bleeding disorder characterized by the quantitative (type 1 and type 2) or qualitative (type 3) deficiency in platelet membrane glycoprotein (GP) IIb/IIIa (CD41a/CD61) fibrinogen receptors. In type 1, 2, and 3, CD41a/CD61 expression is 5%, 5%–20% and above 20%, respectively. In this study, diagnosis of GT was confirmed and subgroups were identified in 32 Turkish patients by flow cytometry analysis.

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This multicenter cohort study aimed to determine the attitudes and expectations of persons with hemophilia of Generation Y (PwH-Y) toward hemophilia and its treatment comparatively with the opinions of their non-hemophiliac relatives. The study was representative regarding quota-control variables of hemophiliacs registered to the provincial representatives of the Hemophilia Society of Turkey in 4 geographic regions and Istanbul. Sixty-four PwH-Y (62 males) and their 56 first-degree relatives (17 males; Generation X/baby boomers) were interviewed face-to-face using mixed data collection method.

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Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints.

Materials And Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3, 6, 9, and 12 month visits).

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Article Synopsis
  • The study evaluates the effectiveness of two assays (one-stage and chromogenic) for measuring factor VIII (FVIII) levels in patients with hemophilia A, which is caused by FVIII gene defects.
  • It involved analyzing 66 blood samples to determine the assays' precision, linearity, and comparability, revealing generally good results but significant bias in samples with FVIII activity below 40 IU.
  • Findings indicate the one-stage assay often yields higher FVIII levels compared to the chromogenic method, particularly for samples with lower concentrations, attributed to the chromogenic assay’s lower detection limit.
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  • The study analyzed 110 children diagnosed with acute lymphoblastic leukemia (ALL) to identify acute complications during their treatment and track survival rates.
  • The majority of patients had pre-B-cell ALL, with a notable occurrence of infections being the most common complication (88.5%), alongside other complications such as gastrointestinal and neurological issues.
  • The 5- and 10-year survival rates were both 85.9%, indicating that while childhood ALL is treatable, comprehensive care from a skilled medical team is essential due to potential acute complications.
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