[Sickle cell crisis: inpatient management].

Sickle cell anemia is the world's most common monogenic disease, with a steady rise in the number of cases also in Switzerland. It presents as regenerative anemia and recurrent vaso-occlusive episodes, which can impact all organs. Acute and chronic complications increase morbidity and mortality, significantly reducing life expectancy compared to the general population.

Constitutive activation and oncogenicity are mediated by loss of helical structure at the cytosolic boundary of thrombopoietin receptor mutant dimers.

Dimerization of the thrombopoietin receptor (TpoR) is necessary for receptor activation and downstream signaling through activated Janus kinase 2. We have shown previously that different orientations of the transmembrane (TM) helices within a receptor dimer can lead to different signaling outputs. Here we addressed the structural basis of activation for receptor mutations S505N and W515K that induce myeloproliferative neoplasms.

Adenosine reverses life-threatening persistent pulmonary hypertension of the neonate refractory to triple vasodilator therapy.

Persistent pulmonary hypertension of the neonate can cause acute and life-threatening hypoxia, but preterm neonates are not suitable candidate to extra-corporeal life support. We report the unique case of an extremely preterm neonates with life-threatening persistent pulmonary hypertension refractory to triple vasodilator therapy (nitric oxide, iloprost, and bosentan), which has been successfully treated with the addition of adenosine continuous infusion.

Coping strategies, anxiety and depression related to the COVID-19 pandemic in lung transplant candidates and recipients. Results from a monocenter series.

Background: The COVID-19 pandemic has been associated with an increase in anxiety and depression symptoms in people. We investigated the impact of the pandemic on coping strategies and anxiety and depression in lung transplantation (LT) recipients and patients with end-stage chronic lung disease awaiting LT.

Methods: We retrospectively investigated coping strategies by using the Coping Inventory for Stressful Situations questionnaire and anxiety and depression symptoms by the Hospital Anxiety and Depression scale in 115 LT candidates and recipients.