Care of patients with hemoglobin disorders during the COVID-19 pandemic: An overview of recommendations.

The outbreak of Coronavirus Disease 2019 (COVID-19) caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has led to a global health emergency.1 Compared to the general population, patients with hemoglobin disorders such as sickle cell disease (SCD) or thalassemia are expected to be more severely affected by COVID-19 due to their preexisting chronic morbidities.2 The Centers for Disease Control and Prevention does not report any specific indications for patients with hemoglobinopathies. However, it can be hypothesized that the rapid spread of the virus may render these patients fragile when fighting the infection. SCD, a hematological condition with functional asplenia, puts patients at a greater risk to develop acute pulmonary complications, including viral infections.2 A study by Hussain et al reported four SCD cases that tested positive for COVID-19.3 These cases initially presented to the emergency department for a typical vaso-occlusive crisis (VOC), and the clinical course of their SARS-CoV-2 infection was rather mild. Patients had a history of respiratory complications, such as acute chest syndrome (ACS), asthma, or pulmonary embolism, which may be potential risk factors for progressive COVID-19 pulmonary disease in patients with SCD.3 A series of isolated cases of ACS in SCD patients positive for COVID-19 has been recently reported.4,5 Therefore, very little clinical experience of infected patients with SCD currently exists. For this reason, we believe that certain recommendations must be followed by healthcare professionals treating any SCD patient infected with SARS-CoV-2

Distribution of G-concurrence of random pure states

Average entanglement of random pure states of an N x N composite system is analyzed. We compute the average value of the determinant D of the reduced state, which forms an entanglement monotone. Calculating higher moments of the determinant we characterize the probability distribution P(D). Similar results are obtained for the rescaled N-th root of the determinant, called G-concurrence. We show that in the limit $N\to\infty$ this quantity becomes concentrated at a single point G=1/e. The position of the concentration point changes if one consider an arbitrary N x K bipartite system, in the joint limit $N,K\to\infty$, K/N fixed.Comment: RevTeX4, 11 pages, 4 Encapsuled PostScript figures - Introduced new results, Section II and V have been significantly improved - To appear on PR

Finding and treating gaucher disease type 1 - The role of the haematologist

Gaucher disease (GD) type 1 is the most common lysosomal storage disease and the most common genetic disorder among Ashkenazi Jews. The majority of patients with GD present with unexplained splenomegaly and/or thrombocytopenia, and the disorder often affects children; consequently, haematologists and paediatricians are ideally placed to diagnose this condition. Prompt management of GD type 1 using enzyme-replacement therapy or substrate reduction therapy can reduce the risk of developing long-term GD complications and reverse many of the initial signs/symptoms, thereby improving both quality and duration of life. Treatment is most effective when initiated early; consequently, a prompt diagnosis is essential. Despite this, the average time to diagnosis following the onset of clinical symptoms is 4 years. Reasons for the delay include the heterogeneous nature of the disease, together with a lack of awareness of rare haematological disorders and the benefits of early treatment. Indeed, studies show that only 20% of haematologists consider GD type 1 in their differential diagnosis for patients presenting with splenomegaly and/or thrombocytopenia. To help raise awareness of GD, reduce the diagnostic delay and prevent unnecessary tissue biopsies, simple diagnostic algorithms and screening tools have been developed and validated, both in adults and in children

Hyponatremia: how much attention do we pay to it?

The objective is to describe hyponatremia in patients hospitalized in geriatric (GU) and Internal medicine units (IM), its characteristics and diagnostic approach in order to improve its management and treatment. It is a retrospective study. We collected data through the informatic system of the hospital, performing statistical analysis with SmallStata14. We analyzed patients admitted during 2015 who showed hyponatremia at admission or developed it in any moment during hospitalization. On 824 patients admitted to the Geriatric (n=351) and Internal Medicine (n=473) wards during 2015 (mean age 77.5\ub114.6 yrs; 56% males), 140 patients (17%) had hyponatremia. Hyponatremia was already present at admission in the emergency unit in 49 patients (74% mild; 14% moderate and 12% severe); 10 patients had hypernatremia but developed hyponatremia thereafter. More than half of the patients was taking at least one drug or had a comorbidity possibly causing hyponatremia. 78 patients (56%) developed it during hospitalization (82% mild, 17% moderate and 1% severe). The mean sodium nadir during hospitalization was 131.8\ub12.4mEq/l. Further analyses to improve the diagnostic accuracy were performed in a minority of cases (plasmatic osmolarity has never been measured, urinary osmolarity and sodium in only 3 and 14 patients respectively). At hospital discharge 48 (34%) patients were still hyponatremic (in 79% mildly) and 71 patients were still assuming at least one drug possibly causing hyponatremia. The hyponatremia was often neglected in the letter of discharge. Hyponatremia is a common observation in GU and IM and it can be due to several causes thus guidelines for its treatment are often useless. The lack of appropriate investigations often lead to improper management. Since even mild hyponatremia has been associated with bad clinical outcomes, more attention should be given, in order to improve the management. Further studies are ongoing