Non-immune Hemolysis in Gaucher Disease and Review of the Literature.

Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of "Gaucher cells." These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.

Ideal lag time from ovulation to oocyte aspiration using a GnRH agonist trigger.

BACKGROUND: In ART, oocyte maturation (M2) and ovulation is stimulated by a hormonal trigger. For maturation to occur, sufficient "lag time" must elapse between the trigger and aspiration, ranging from 32 to 38 hours. Premature aspiration can result in poor yields; late aspiration risks spontaneous ovulation. AIM: Our study examines optimal lag time using a GnRH antagonist protocol and GnRH agonist trigger for ICSI. METHODS AND MATERIALS: We analyzed data from 220 women undergoing GnRH antagonist protocol using a GnRH agonist trigger for ICSI at our clinic between 02/2012-03/2018. Patients were divided into 4 groups based on lag time: 34.00-34.99 hours (n = 32), 35.00-35.99 hours (n = 113), 36.00-36.99 hours (n = 57) and 37.00 h or more (n = 18). Analyses were performed with the Kruskal-Wallis test, Chi-Square, and Spearman's rho correlation. RESULTS: A positive correlation was found for the number of M2 oocytes aspirated and lag time (ρ = 0.138, p = 0.04) and for the total number of oocytes aspirated and lag time, (ρ = 0.174, p = 0.01). No correlation was found between the proportion of M2 oocytes aspirated and lag time (p = 0.217). The third group (36 h) had significantly more M2 oocytes aspirated than the second group (35 h) (12.4 ± 7.1 vs 9.4 ± 6.2; p = 0.039). The four groups did not differ for the proportion of mature M2 oocytes (H = 2.453, p = 0.484). The four groups differed in the frequency of live births per fresh embryos transferred (χ2 = 9.364, p = 0.025). CONCLUSION: Our study identified a positive correlation between lag time and both the number of M2 oocytes and the total number of oocytes aspirated-factors which lead to an increased rate of successful pregnancies. Further research is necessary.

Electronic Media Abstinence in Sabbath Observant Jews: A Comparison Between the Weekday and Sabbath.

BACKGROUND: Dependence on technology and electronic media devices (EMDs) is a significant phenomenon of modern life with many people experiencing adverse symptoms during abstention. Orthodox Jews abstain from using all forms of EMDs for 25 consecutive hours every week on the Sabbath but do not appear to experience significant adverse reactions during this abstention. OBJECTIVES: To better examine whether Sabbath observant Jews experience fewer and less severe adverse symptoms while abstaining from EMDs on the Sabbath compared to weekdays. METHODS: Ten Sabbath observant Jews abstained from using all forms of EMDs for 25 hours on a Sabbath and again on a weekday. At the end of each 25-hour period participants completed a 12-item Likert-type scale self-assessment of 1-5, once as a report of their condition at 10:00 and again after 25 hours of abstaining. The authors compared the mean results of Sabbath and weekday using Wilcoxon signed ranks test. P ≤ 0.05 was considered significant. RESULTS: Overall, discomfort on Sabbath was less than on weekdays. A statistically significant decrease on the Sabbath was found at both the 10:00 reporting time and after 25 hours in anxiety, restlessness, thoughts and plans of using devices, and overall difficulty to abstain. Significance was found for feelings of not knowing what to do with time (10:00) and moodiness and irritability, being drawn to devices, and cravings achieved significance (after 25 hours). CONCLUSIONS: Sabbath observant Jews reported statistically significant less adverse reactions while abstaining from EMDs on the Sabbath compared to on a weekday.

Pharma Websites and "Professionals-Only" Information: The Implications for Patient Trust and Autonomy.

BACKGROUND: Access to information is critical to a patient's valid exercise of autonomy. One increasingly important source of medical information is the Internet. Individuals often turn to drug company ("pharma") websites to look for drug information. OBJECTIVE: The objective of this study was to determine whether there is information on pharma websites that is embargoed: Is there information that is hidden from the patient unless she attests to being a health care provider? We discuss the implications of our findings for health care ethics. METHODS: We reviewed a convenience sample of 40 pharma websites for "professionals-only" areas and determined whether access to those areas was restricted, requiring attestation that the user is a health care professional in the United States. RESULTS: Of the 40 websites reviewed, 38 had information that was labeled for health care professionals-only. Of these, 24 required the user to certify their status as a health care provider before they were able to access this "hidden" information. CONCLUSIONS: Many pharma websites include information in a "professionals-only" section. Of these, the majority require attestation that the user is a health care professional before they can access the information. This leaves patients with two bad choices: (1) not accessing the information or (2) lying about being a health care professional. Both of these outcomes are unacceptable. In the first instance, the patient's access to information is limited, potentially impairing their health and their ability to make reasonable and well-informed decisions. In the second instance, they may be induced to lie in a medical setting. "Teaching" patients to lie may have adverse consequences for the provider-patient relationship.

'Model' versus 'everyday' patients: can randomized controlled trial data really be applied to the clinic?

New drug approval requires a new drug to undergo rigorous clinical trials to determine its efficacy and safety. A drug is approved only for the population on which it was tested, i.e. those who meet the inclusion criteria of the trial. The aim of this study was to determine what percentage of 'real life' patients in our clinic meet the inclusion and exclusion criteria used in large-scale clinical trials required for drug registration in the field of assisted reproduction. All 265 consecutive patients with pertinent data treated in a tertiary centre IVF Unit during 2015 were surveyed. Their demographic and clinical parameters were compared with inclusion and exclusion criteria used in nine major clinical trials. Only 97 out of 265 (37%) patients met the consensus inclusion criteria as defined by the nine clinical trials. The number of oocytes retrieved was 9.10 ± 5.34 in the patients that met the inclusion criteria (n = 97) versus 6.90 ± 5.23 (P = 0.00122) in those that did not (n = 168). Most 'real life' patients who come for treatment at a tertiary IVF centre do not meet the consensus of inclusion and exclusion criteria used for major clinical trials.