Effects of hydroxyurea on fertility in male and female sickle cell disease patients. A systemic review and meta-analysis.

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.

Utility and Implementation of the Distress Thermometer for Cancer Patients: A Cross-Sectional Study From Saudi Arabia.

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.

Prevalence of Asymptomatic Deep Vein Thrombosis in Preoperative State of Spine Surgeries.

BACKGROUND: Deep venous thrombosis (DVT) is considered the most common cause of preventable death among hospitalized patients. A few studies have investigated the risk of venous thromboembolic events in patients undergoing elective spine surgery and reported varying incidences. We aim to assess the incidence of preoperative lower limb DVT in patients with lumbar degenerative disease undergoing elective surgery. METHODS: This is a retrospective review of prospectively collected data on adult patients with lumbar degenerative spine diseases (lumbar stenosis [LST], lumbar spondylolisthesis [SPL], or lumbar disc herniation) who were scheduled for operative management. Each patient underwent a preoperative lower limb venous Duplex (LLVD). Incidence of DVT, risk factors, and clinical course of patients were reported. RESULTS: Forty-nine patients (32 females; 65%) were included. All patients were diagnosed clinically and radiologically with lumbar degenerative diseases; LST (44.9%), SPL (36.7%), and lumbar disc herniation (18.4%), requiring surgical management. Three patients (6.1%) were found to have positive DVT studies, leading to the cancellation or rescheduling of their procedures (P < 0.01). These included 1 patient with LST (P = 0.045) and 2 patients with SPL (P = 0.006). Among patients with LST and SPL, higher disability (modified Rankin scale ≥3) was significantly associated with positive LLVD (P = 0.035). CONCLUSION: Patients with LST or SPL with higher preoperative disability scores were at higher risk to have preoperative DVT. Further research is needed to evaluate the feasibility and value of preoperative LLVD to detect DVT in patients planned for lumbar degenerative surgery.

Continuous infusion factor replacement in haemophilia B during and after cardiac surgery: the better choice?

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.

Successful Use of Veno-Venous Extracorporeal Membrane Oxygenation in an Adult Patient with Sickle Cell Anemia and Severe Acute Chest Syndrome.

A 25-year-old female with sickle cell anemia presented with respiratory failure due to acute chest syndrome (ACS). Given her severely compromised cardiopulmonary status, she was started on veno-venous extracorporeal membrane oxygenation (VV-ECMO). After 20 days, the patient's respiratory status improved and she was successfully decannulated. Veno-venous extracorporeal membrane oxygenation can be utilized for severe ACS in adult patients with sickle cell disease. Prompt initiation of this modality may improve outcomes in adult patients with sickle cell disease complicated by life threatening ACS.

Potential association between membranous nephropathy and sargramostim therapy for pulmonary alveolar proteinosis.

We present the case of a 43-year-old woman with a diagnosis of pulmonary alveolar proteinosis, on chronic treatment with sargramostim, a recombinant granulocyte-macrophage colony-stimulating factor, who presented with the nephrotic syndrome secondary to biopsy-proven membranous nephropathy. We discuss potential underlying mechanisms, including speculated effects of sargramostim on mesangial cells and the kidney resident macrophages, and review the existing literature on the potential association between these two disorders.