β-Thalassemia minor is associated with high rates of worsening anemia in pregnancy.

We identified 347 pregnancies in patients with beta thalassemia minor. Hemoglobin was below 9 g/dL in 31% during third trimester and 7.6% at delivery.

The "flipped visit": an innovative method to improve medical student self-efficacy through a structured approach in clinic.

Background: Specialty clinics can be challenging for students on the internal medicine clerkship. They often lack the specialized knowledge necessary to fully engage in the clinic and may be pushed into an observational role, rather than being afforded meaningful opportunities. These peripheral roles undermine self-efficacy, and, therefore, education and interest.

A Case Report of Rotational Thromboelastometry-Assisted Decision Analysis for Two Pregnant Patients With Platelet Storage Pool Disorder.

Platelet storage pool disorders (PSPDs) constitute a diverse group of hematologic abnormalities, which share the common feature of a deficiency in the ability of platelets to aggregate. Parturients with PSPD can present management challenges to their care team when they present for childbirth, especially with regard to neuraxial anesthesia. We report a series of 2 deliveries from unrelated patients affected by PSPD.

β-Thalassemia: evolving treatment options beyond transfusion and iron chelation.

After years of reliance on transfusion alone to address anemia and suppress ineffective erythropoiesis in β-thalassemia, many new therapies are now in development. Luspatercept, a transforming growth factor-β inhibitor, has demonstrated efficacy in reducing ineffective erythropoiesis, improving anemia, and possibly reducing iron loading. However, many patients do not respond to luspatercept, so additional therapeutics are needed.

Acquired von Willebrand Syndrome.

Acquired von Willebrand syndrome can occur in the setting of myeloproliferative neoplasms; plasma cell dyscrasias and other lymphoproliferative disorders; autoimmune conditions; and causes of increased shear forces, such as aortic stenosis or other structural heart disease and mechanical circulatory support. The depletion of von Willebrand factor, especially high-molecular-weight multimers, can lead to mucocutaneous bleeding and the formation of arteriovenous malformations, particularly in the gastrointestinal tract. Management focuses on correction of the underlying cause when possible, but may include intravenous immunoglobulins, von Willebrand factor concentrate, rituximab, or antiangiogenic therapy depending on the clinical context.

Building upon the foundational science curriculum with physiology-based grand rounds: a multi-institutional program evaluation.

: Vertically integrating physiology into patient care has the potential to improve clinical reasoning. Clinical Physiology Grand Rounds (CPGR) is a case-based teaching method that brings together students from all years of medical school to focus on linking clinical presentations to core basic science concepts including anatomy, physiology, and pathophysiology. In this study, we describe the implementation of CPGR at two different institutions in the United States and assess student-reported outcomes.

Long-term Outcomes of team-based learning.

Background: Team-based learning (TBL) is associated with improved end-of-course exam performance, but the impact on long-term retention is unknown. We compared the impact of three teaching methods: traditional case-based small group discussion (TSG), TBL or no small group reinforcement on short-term understanding and long-term retention after a haematology course.

Methods: Knowledge assessments were conducted prior to, immediately after and 14 months after course completion.

Hemolysis and Hemoglobin Structure and Function: A Team-Based Learning Exercise for a Medical School Hematology Course.

Introduction: This team-based learning (TBL) exercise focused on hemolysis and hemoglobin structure and function. The goal was to emphasize content that directly impacts clinical practice, but obliges students to understand underlying pathophysiology. The readiness assurance test (RAT) covers oxygen affinity, diagnosing hemolysis, inherited causes of hemolysis (G6PD deficiency, hereditary spherocytosis, sickle cell disease, thalassemia) and acquired causes of hemolysis (thrombotic microangiopathies, autoimmune hemolytic anemia).

Billing Codes Determine Lower Physician Income for Primary Care and Non-Procedural Specialties.

The income gap between specialists and primary care physicians and among specialists is well established, but the drivers of this difference are not well delineated. Using the Community Tracking Study (CTS) Physician Survey, we sought to isolate and compare premiums paid to physicians for specialization and the proportion of time spent on offices visit rather than procedures. We divided medical subspecialties according the proportion of Medicare billing for Evaluation and Management (E&M) codes for the specialty as a whole.

Inhaled steroids associated with decreased macrophage markers in nonasthmatic individuals with sickle cell disease in a randomized trial.

Inhaled mometasone was shown to improve pain scores and decrease soluble vascular cell adhesion molecule (sVCAM) concentration in a randomized controlled trial of nonasthmatic patients with sickle cell disease. We sought to explore potential changes in systemic inflammation as a mechanism underlying this effect. Serum samples from 41 trial participants (15 placebo- and 26 mometasone-treated) were analyzed using a 92 inflammatory marker panel at baseline and after 8 weeks of mometasone therapy.

Hemophagocytic Lymphohistiocytosis Due to Primary HHV-8 Infection in a Liver Transplant Recipient.

Human herpesvirus-8 (HHV-8) remains best known as an oncogenic virus, but nonneoplastic disease manifestations, such as bone marrow failure or hemophagocytic lymphohistiocytosis (HLH) have gained greater recognition in recent years. In organ transplantation, HHV-8 infection commonly occurs with reactivation of latent virus among recipients from endemic regions of the world or due to transmission from the organ donor. We describe a case of HHV-8-associated HLH in a liver transplant recipient at increased risk for primary infection.

Evaluating the safety of emicizumab in patients with hemophilia A.

Patients affected by hemophilia A often require frequent prophylactic and therapeutic self-infusion. For those who develop inhibitors, treatment options are limited and mortality is increased. Emicizumab, a bispecific antibody to Factors IXa and X that carries out the function of Factor VIII (FVIII), represents a novel therapeutic approach.

Hospital Admissions, Mortality and Comorbidities Among New York State Sickle Cell Patients, 2005-2013.

Analyses of administrative and large data sources in Sickle Cell Disease (SCD) can answer questions not suitable for prospective study but have been hampered by lack of validated methods to adjust for individual comorbidities and lack of baseline utilization data over time. We sought to develop a database to characterize inpatient SCD care across New York State and generate a re-weighted sickle-cell specific Charlson Comorbidity index (S-CCI) for use in future large data SCD research. We identified 18,541 individual SCD patients admitted to New York State hospitals between 2005 and 2013 from the SPARCS database.

Role of hepcidin-ferroportin axis in the pathophysiology, diagnosis, and treatment of anemia of chronic inflammation.

Anemia of chronic inflammation (ACI) is a frequently diagnosed anemia and portends an independently increased morbidity and poor outcome associated with multiple underlying diseases. The pathophysiology of ACI is multifactorial, resulting from the effects of inflammatory cytokines which both directly and indirectly suppress erythropoiesis. Recent advances in molecular understanding of iron metabolism provide strong evidence that immune mediators, such as IL-6, lead to hepcidin-induced hypoferremia, iron sequestration, and decreased iron availability for erythropoiesis.