Long-term tolerability of phosphodiesterase-5 inhibitors in pulmonary hypertension of sickle cell disease.

OBJECTIVES: Sickle cell disease-related pulmonary hypertension (SCD-PH) is a complex disorder with multifactorial contributory mechanisms. Previous trials have evaluated the efficacy of pulmonary arterial hypertension (PAH) therapies in SCD-PH with mixed results. We hypothesized that a subset of patients with right heart catheterization (RHC) confirmed disease may benefit from PAH therapy. METHODS: We performed a retrospective chart review of patients with SCD-PH diagnosed by RHC who were treated with phosphodiesterase 5 inhibitor (PDE5-I) therapy for ≥4 months between 2008 and 2019 at two institutions. RESULTS: Thirty-six patients were included in the analysis. The median age (IQR) upon PDE5-I initiation was 47.5 years (35-51.5 years); 58% were female and twenty-nine (81%) had HbSS disease. Of these, 53% of patients had a history of acute chest syndrome, 42% had a history of venous thromboembolism, and 38% had imaging consistent with chronic thromboembolic PH. Patients were treated for a median duration of 25 months (IQR 13-60 months). Use of PDE5-I was associated with a significant improvement in symptoms as assessed by NYHA Class (P = .002). CONCLUSIONS: In SCD patients with PH defined by RHC, PDE5-I therapy was tolerated long-term and may improve physical activity.

COPD overlap conditions: Clinical and therapeutic implications✰.

Chronic Obstructive Pulmonary Disease (COPD) is a complex pulmonary condition characterized by chronic airflow limitation. Within the spectrum of COPD, distinct overlap conditions exist, including Asthma-COPD Overlap (ACO), COPD-Obstructive Sleep Apnea (COPD-OSA), Combined Pulmonary Fibrosis and Emphysema (CPFE), and Bronchiectasis-COPD Overlap (BCO). This review provides a comprehensive overview of the clinical and therapeutic implications of these conditions, highlighting the differences in complications compared with COPD alone in addition to the diagnostic challenges of identifying these conditions. Therapeutically tailored approaches are necessary for COPD overlap conditions considering the unique complications that may arise. Optimal pharmacological management, disease-specific interventions, and comprehensive patient-centered care are crucial components of treatment strategies. This review provides insights for healthcare professionals by enhancing their understanding and management of these conditions. This emphasizes the importance of accurate diagnosis and individualized treatment plans, considering the specific complications associated with each COPD overlap condition.

Endophthalmitis as the initial manifestation of invasive fusariosis in an allogeneic stem cell transplant patient: A case report.

Fusarium species manifests as an opportunistic infection with intrinsic resistance to most antifungals. We present a case of a 63-year-old male with myelodysplasia who received allogeneic stem cell transplantation and presented with endophthalmitis as the initial manifestation of invasive fusariosis that progressed to a fatal outcome despite combined intravitreal and systemic antifungal therapies. We urge clinicians to consider this complication of fusarium infection especially with the widespread use of antifungal prophylaxis that may incur selection of more resistant, invasive fungal species.

Common Complications of Sickle Cell Disease: A Simulation-Based Curriculum.

Introduction: Sickle cell disease (SCD), the most common autosomal recessive genetic disorder worldwide, affects nearly every organ of the body and results in accelerated mortality. Nationally, internal medicine physicians lack a complete understanding of morbidity and mortality in this population leading to health care disparities. Methods: We created a 2-hour curriculum consisting of three SCD case vignettes representing common disease complications (acute stroke, acute chest syndrome, and septic shock) with the goal to increase medicine house staff knowledge and confidence in patient management. Residents completed a pretest to assess baseline knowledge and were divided into groups of four to five. Three simulation cases were completed by each group; learners needed to work through a differential diagnosis and describe key management steps. Each group was graded on achieving the 10 critical actions for each case. Following each case, there was a faculty-led debriefing session. Residents repeated the pretest 30 days after completion of the curriculum (posttest). Results: Thirty-six second year internal medicine residents participated in this curriculum. After completing this curriculum, residents improved their test score from 33% (SD = 12%) to 57% (SD = 18%) (p < .0001). Additionally, self-reported confidence in management scores increased from 2.6 (SD = 0.8) in the pretest to 3.5 (SD = 0.4) in the posttest (p = .02) on a 5-point Likert scale (1 = not very confident, 5 = very confident). Discussion: Use of a simulation curriculum increased knowledge and confidence of internal medicine residents in the management of critical illness in patients with SCD.