• 제목/요약/키워드: Sickle cell disorders

검색결과 3건 처리시간 0.018초

Effectiveness of premarital screening program for thalassemia and sickle cell disorders in Ras Al Khaimah, United Arab Emirates

  • Salama, Rasha Aziz Attia;Saleh, Abeer Kamal
    • Journal of Genetic Medicine
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    • 제13권1호
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    • pp.26-30
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    • 2016
  • Purpose: Genetic disorders can be prevented by basic public health measures and activities that focus primarily on education and approaches in Primary Health Care. Premarital screening is one such approach that can identify asymptomatic carriers of hemoglobinopathies and provide genetic counseling to couples for a healthy reproductive life. This study aimed to estimate the prevalence of beta thalassemia and sickle cell disorders in the adult population screened as a part of the United Arab Emirates Premarital Screening Program and to measure the effectiveness of the program in decreasing high-risk marriages in Ras Al Khaimah (RAK). Materials and Methods: A retrospective, population-based study was conducted at the RAK Primary Health Care Center, where the National Premarital Screening Program is implemented. The study included data collected from the premarital screening records of all couples who had applied for a marriage license during 2008-2015. Results: Of the 17,826 individuals screened during the studied period, 4.02% (717) were diagnosed as positive for hemoglobinopathies. The prevalence of beta thalassemia and sickle cell disorders among the total study population was 2.98% and 1.05%, respectively. The hemoglobinopathy trait was more prevalent than the disease. Among these 8,913 couples who were issued certificates for compatibility based on screening tests, 28 (0.31%) couples were declared high-risk (unmatched). Seventy percent of these unmatched couples reported consanguineous marriages. Conclusion: The program was successful in achieving its objective of identifying high-risk marriages. In spite of the counseling, however, all of the high-risk couples still married each other.

Papaya: A gifted nutraceutical plant - a critical review of recent human health research

  • Karunamoorthi, Kaliyaperumal;Kim, Hyung-Min;Jegajeevanram, Kaliyaperumal;Xavier, Jerome;Vijayalakshmi, Jayaraman
    • 셀메드
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    • 제4권1호
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    • pp.2.1-2.17
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    • 2014
  • The plant kingdom is considered to be a repository of modern medicine, attributable to their rich source of bio-active molecules and secondary metabolites. It is indeed the Nutraceuticals that enhance immunity and ensure a healthier life because of their prophylactic and therapeutic values. Over centuries, papaya [Caricaceae; (Carica papaya Linn.)] is a renowned nutritious and medicinal plant. Each part of the papaya like root, stem, leaf, flower, fruit, seed, rinds, and latex has its own nutraceutical properties. It serves as food, cooking aid, and Ethnomedicine to prevent and treat wide-range of diseases and disorders. It has also been traditionally used as appetite enhancer, meat tenderizer, purgative, medicinal acne, abortifacient and vermifuge. Over decades, a series of scientific attempts were made to authenticate the nutraceutical properties of papaya. These studies validated that the papaya has antiplasmodial, antitrichochramal, antitrichomonal, antidengue, and anti-cancer activities. They have also exhibited that papaya possesses antiseptic, antiparasitic, anti-inflammatory, antidiabetic, and contraceptive features, and it helps in the management of sickle-cell anaemia, HIV, heart diseases and digestional disorders too. Nevertheless, the responsible bio-active molecules and their mode of actions remain indistinct and imprecise, and this calls for further pharmacological and clinical research on them. Conclusively, papaya is one of the naturally gifted plants; though its nutraceutical properties as a food or as a quasi-drug are poorly understood or undervalued by people. Accordingly, this scrutiny, demand for instigation of public health awareness campaigns to promote papaya consumption, so that the society shall acquire optimal benefits of papaya and in turn prevent and alleviate various diseases and illness.

소아뇌졸중의 보험의학적 고찰 (Review of pediatric cerebrovascular accident in terms of insurance medicine)

  • 안계훈
    • 보험의학회지
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    • 제29권2호
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    • pp.29-32
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    • 2010
  • Moyamoya disease (MMD) is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. MMD is one of cerebrovacular accident,which is treated with sugical maeuver in pediatic neurosurgery. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis. The exact etiology of moyamoya disease is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with moyamoya disease, including the following: 1) Immunological - Graves disease/thyrotoxicosis 2) Infections - Leptospirosis and tuberculosis 3) Hematologic disorders - Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus 4) Congenital syndromes - Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease 5) Vascular diseases - Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, 6)cranial trauma, radiation injury, parasellar tumors, and hypertension etc. These associations may not necessarily be causative but do warrant consideration due to impact on treatment.(Mainly neurosurgical operation.) The incidence of moyamoya disease is highest in Japan. The prevalence of MMD is 1 person per 100,000 population. The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.4:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade(5-10yr) or in the third and fourth decades (30-40yr)of life. Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. In aspect of life insurance, MR is 1700%, EDR is 16 per 1000 persons. Children and adults with moyamoya disease (MMD) may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present. Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. Recently increasing diagnosis of MMD with MRI, followed by surgical operation is noted. MMD needs to be considered as the "CI" state now in life insurance fields.

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