health department

Immune-mediated deficiency of the von Willebrand factor–cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis. This results in thrombocytopenia, hemolytic anemia, and tissue ischemia. These are the hallmarks of  acquired thrombotic thrombocytopenic purpura (TTP). Caplacizumab is an anti–von Willebrand factor humanized. Caplacizumab is not a full antibody, but just a fragment if it, as you can see in the video below. It inhibits interaction between von Willebrand factor multimers and platelets. In this double-blind, controlled trial, 145 patients with TTP received caplacizumab (10-mg intravenous loading bolus, followed by 10 mg daily subcutaneously) or placebo during plasma exchange and for 30 days thereafter. The median time to normalization of the platelet count was shorter with caplacizumab than with placebo (2.69 days vs. 2.88 days). Patients who received caplacizumab were 1.55 times as likely to have a normal...

A quote from this NEJM article : "Socrates underscored the perils of an unexamined life. Yet for some physicians, a closely examined professional life would force a painful recognition of what’s missing. Robert Pirsig, whose philosophical writing focused on quality and values, crystallized the problem decades ago: “We’re in such a hurry most of the time we never get much chance to talk. The result is a kind of endless day-to-day shallowness, a monotony that leaves a person wondering years later where all the time went and sorry that it’s all gone.”" The author of the article lists a few interventions he had tried at this workplace in the second article below. Many of those don't seem to reach the root of the problem though. References: RVU Medicine, Technology, and Physician Loneliness | NEJM https://buff.ly/2MEUBwd Navigating Loneliness in the Era of Virtual Care | NEJM https://buff.ly/2DKhICP

C. Nicholas Cuneo, M.D. in the NEJM : "With every faux interaction I felt myself being forced to shed another layer of authenticity, and I quickly grew to dread the whole tedious charade. PEARLS, it spelled out: Partnership, Empathy, Apology, Respect, Legitimization, and Support. With a smirk, I tossed it in the trash." Better understanding and educational approach are needed. -- Just as a side note, here is an overview of some empathy/communication mnemonics with the corresponding references: "PEARLS – which stands for partnership, empathy, apology/acknowledgment, respect, legitimation, and support" https://www.mdedge.com/familypracticenews/article/88977/neurology/use-pearls-build-relationships-patients "The NURSE mnemonic is a useful memory aid to assist you to comprehensively and appropriately respond to patients’ emotions" https://www.stepsforward.org/modules/empathetic-listening The NURSE mnemonic has been reproduced from Back A, Arnold R, Tulsky J....

From the NEJM: What if health care were designed so that in-person visits were the second, third, or even last option for meeting routine patient needs, rather than the first? At Kaiser Permanente, 52% of 100 million patient encounters each year are now “virtual visits”. It spends 25% of its annual $3.8 billion budget on information technology. "Payment models are an obvious barrier to deemphasizing in-person visits, but every provider’s business success depends on market share. The best way to win market share is to design and deliver better care, then modify the payment system to support it. Moreover, payment systems are already evolving to support nonvisit care. For example, use of bundled payment programs and accountable care organizations — which reward nontraditional care delivery models that reduce spending and meet patients’ needs — is growing." Patients are increasingly asking, “Isn’t there a way to do this without my having to drive to your office?” References: In-P...

Hemophilia B Gene Therapy with a High-Specific-Activity Factor IX Variant: the researchers infused a single-stranded adeno-associated viral (AAV) vector consisting of a bioengineered capsid, liver-specific promoter and factor IX Padua (factor IX–R338L) transgene in 10 men with hemophilia B who had factor IX coagulant activity of 2% or less of the normal value. They found sustained therapeutic expression of factor IX coagulant activity after gene transfer in the 10 participants with hemophilia who received the same vector dose. Transgene-derived factor IX coagulant activity enabled the termination of baseline prophylaxis and the near elimination of bleeding and factor use. More info here: http://www.nejm.org/doi/full/10.1056/NEJMoa1708538

The hepatitis C virus (HCV), a single-stranded RNA virus of the family Flaviviridae with six major genotypes, infects up to 150 million people worldwide. Chronic hepatitis C virus infection causes progressive liver fibrosis, which can lead to cirrhosis and hepatocellular carcinoma. There is now an e ffective oral regimen . New research findings are summarized in this short video from NEJM: This is a ribavirin-free single-tablet regimen. There 2 medications in the single tablet: - Sofosbuvir is a nucleotide analogue inhibitor of the HCV NS5B polymerase approved for the treatment of HCV in combination with a variety of other agents, including NS5A inhibitors, ribavirin, and peginterferon–ribavirin. - Velpatasvir is a new pangenotypic HCV NS5A inhibitor with antiviral activity against HCV replicons in genotypes 1 through 6. The rate of sustained virologic response among patients receiving sofosbuvir–velpatasvir was 99%. See the NEJM article, "Sofosbuvir and Velpatasvir for HCV Geno...