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• W2613161623 abstract "IntroductionSickle cell anemia is a common hereditary disabling disorder profoundly affecting human morbidity, mortality and quality of life. The sickle cell disease (SCD) is an autosomal recessively inherited condition caused by a point mutation in the s-globin gene, resulting in substitution of the valine for glutamic acid at 6th position of the s-globin chain (Glu6Val). This mutation results in abnormal sickle hemoglobin (Hb S) which is when deoxygenated, polymerizes damaging the sickle erythrocyte. Sickling is dependent on multiple variables including hemoglobin concentration, hydration status, pH and degree of deoxygenation. Sickling is more common in vascular beds with low-flow states including the splenic sinusoids, bone marrow, and the penile corpora during an erection, or in any inflamed tissue. The combined influence of these factors can result in a vicious cycle of inflammation, hypoxia, and acidosis resulting in increased sickling, vessel occlusion, and ischemia. Priapism is a patho-physiologically sustained, painful, and unwanted erection of the penis due to either increased arterial inflow (i.e., high flow) or more commonly, the failure of venous outflow (i.e., low flow or veno-occlusive flow) resulting in blood trapping within the erectile bodies. Although uncommon in the general population, priapism was recognized as a serious complication of the SCD as early as 1934 (1). Since then, many researchers have estimated its incidence and prevalence including various treatment strategies and choices in different populations.Considering the embarrassing nature of the problem and the dire consequences to erectile function, it is important to inform patients, parents, and health providers about the relationship of SCD to prolonged painful erections. Ideally, patients at high risk for the development of such episodes should be identified early to increase the education of risks of prolonged priapism and to institute potential prophylactic strategies. Increased awareness of the signs of priapism and educational needs for early treatment may be the best way to prevent long term sequelae.The occurrence of priapism since time immemorial is not uncommon in India. It is widely encountered in length and breadth of the country. It is a common practice in India that any problem related to sex or sex organs is intentionally kept hidden because of shyness or psycho-social implications or due to a stigma attached to it. It is never brought to the notice of elderly members of the family or to a medical doctor. Further, there is utter lack of sex education in India, resulting in deterioration of health related complications. The case of priapism especially its occurrence in sickle cell disease is one of such examples.This article emphasizes the need of imparting sex education to rural masses especially associated with the sickle cell disease in India.MethodsThis study was a part of our major ongoing research project undertaken on 'Reproductive Outcome in Carrier Couples of Hemoglobinopathies in a Tertiary Hospital in Central India.' Ethical approval of this project was obtained from the Human Ethical Committee, Regional Medical Research Centre for Tribals (ICMR), Jabalpur. A total of 1,279 subjects suspected to be suffering from anemia/ hemoglobinopathies referred from a tertiary hospital, Netaji Subhash Chandra Bose Medical College & Hospital, Jabalpur in Central India were screened for different hemoglobinopathies during the cross-section period from March 2010 to December 2012.About 2-3ml. of blood was intravenously taken under aseptical conditions with disposable syringes and needles using disodium salt of ethylene diamine tetra acetic acid (EDTA) as anticoagulant after taking informed/written consent from each individual who was free of blood transfusion at least one month prior to screening for hemoglobinopathies. Blood so collected was transported to laboratory at RMRCT, Jabalpur under wet-cold conditions for analysis. …" @default.
• W2613161623 created "2017-05-19" @default.
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• W2613161623 date "2015-07-01" @default.
• W2613161623 modified "2023-09-27" @default.
• W2613161623 title "Priapism in Sickle Cell Disease: Need for Imparting Sex Education in Rural India" @default.
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