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Background: Evidence supports the benefits of hydroxyurea (HU) in adults with sickle cell disease (SCD), but reservations remain due to long-term concerns of fertility. Retrospective analysis of clinical records of SCD patients (haemoglobin SS genotype) have identified gender-related differences in disease progression. This could inform risk stratification during SCD at diagnosis with the possibility to guide therapeutic decisions., Methods: This systemic review and meta-analysis evaluated fertility parameters in both children (aged ≥ 6 years) and adults with SCD receiving HU therapy. Studies were sourced from PubMed and EMBASE from inception to July 2023. A total of 160 potentially relevant articles were identified., Results: Four studies were included that evaluated the effects of HU on sperm parameters in males. A further 4 studies assessed anti-mullerian hormone (AMH) levels and ovarian reserves in females. Differences from baseline values were used to identify compromised fertility. Amongst males, HU treatment negatively impacted the concentration of spermatozoa (MD = -15.48 million/mL; 95% CI: [-20.69, -10.26]; p< 0.001), which continued following treatment cessation (MD = -20.09 million/mL; 95% CI: [-38.78, -1.40]; P = 0.04). HU treatment also led to lower total sperm counts (MD = -105.87 million; 95% CI: [-140.61, -71.13]; P< 0.001) which persisted after treatment (MD = -53.05 million; 95% CI: [-104.96, -1.14]; P = 0.05). Sperm volume, initial forward motility and morphology were unaffected by HU treatment. In females, HU treatment decreased the mean AMH levels 1.83 (95% CI [1.42, 2.56]. A total of 18.2.% patients treated with HU showed reduced ovarian reserves., Interpretation & Conclusions: This systemic review and meta-analysis suggest that the use of HU for SCD impacts seminal fluid parameters in males and can diminish AMH levels and ovarian reserves in females., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Sewaralthahab et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Background Cancer patients suffer from variable degrees of distress. The distress thermometer (DT) is a valuable tool for screening those patients for distress. Few studies have addressed the utility of DT in screening cancer patients in Saudi Arabia. We aimed to measure the distress level of adult cancer patients utilizing the DT and identify the appropriate measures and interventions required to improve this population's well-being. Methods This cross-sectional study was carried out at the oncology center of King Saud University Medical City (KSUMC), Riyadh, Saudi Arabia. Enrollment criteria were Saudi adults (≥14 years old), with a diagnosis of cancer, who gave informed consent. They were screened for distress using the DT and its associated problem list (PL). A workflow for a psycho-oncology supportive program was suggested. Results Using DT at a cut-off score of ≥4, 22% of patients had significant distress. The most frequent problems reported were loss/change of physical activity, swelling/edema, change in eating, family health problems, and child care. The multivariable binary regression analysis showed that sadness, depression, worry/anxiety, fear, loss of interest, change in appearance, taking care of myself, swelling/edema, and memory/concentration problems were independent factors for significant distress in our cohort. The suggested workflow could effectively be implemented among cancer patients. Conclusion The current study's findings support previous reports concerning the utility of DT in screening cancer patients for distress. A considerable number of Saudi cancer patients suffered from significant distress, which was significantly related to the emotional, spiritual, social, and religious aspects of their problems. We suggested a workflow by which cancer centers can implement DT screening after developing a plan for timely distress evaluation, with further proper management and referrals accordingly. Additional studies are warranted., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Alghamdi et al.)

A 57-year-old man with mild haemophilia B was admitted for coronary artery bypass graft surgery. His factor IX (FIX) activity was 15% on admission. Our goal was to maintain his FIX activity at 80%-100% for post-op days (PODs) 0-3, and at 60%-80% for PODs 4-14. Preoperatively, the patient was given recombinant FIX (rFIX) bolus using the formula:Dosage needed=%(desired FIX level-current level of FIX)×weight (kg)×1.3.This increased his activity to 100%. One IU of rFIX increased FIX activity by 0.8%; the half-life of rFIX is 18-24 hours. The rFIX infusion was started intraoperatively and continued after surgery to maintain target FIX activity. He was discharged on POD 9 on rFIX bolus dosing of 5000 IU every 12 hours for an additional 5 days. Using continuous factor infusion, we managed to decrease the amount rFIX used by >60% while maintaining steady state FIX activity level., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)