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劳伦斯-穆恩-巴德-比德尔综合征在巴基斯坦人群中的罕见性及其治疗不佳的情况。

Rarity of Laurence Moon Bardet Biedl Syndrome and its Poor Management in the Pakistani Population.

作者信息

Khan Omair A, Majeed Ramsha, Saad Muhammad, Khan Asad, Ghassan Ayesha

机构信息

Internal Medicine, Fauji Foundation Hospital, Islamabad, PAK.

Medical Education and Simulation, Fauji Foundation Hospital, Islamabad, PAK.

出版信息

Cureus. 2019 Feb 21;11(2):e4114. doi: 10.7759/cureus.4114.

Abstract

Laurence Moon Bardet Biedl syndrome is characterized as a rare genetic disorder, with a wide range of presenting symptoms such as mental retardation, decreased visual acuity, obesity, hypogonadism, and polydactyly. The diagnosis of this syndrome is easily overlooked due to its rarity, with a prevalence rate of one in 125,000-160,000 reported within Europe. Delayed diagnosis and inappropriate management may lead to an irreversible loss of functions. The most significant of these losses include loss of vision, cardiac problems, and renal abnormalities. These dysfunctions critically impact the mental faculties and personal life of a patient. Our case presented with striking features of this syndrome, but due to a lack of awareness, her family was not adequately counseled. Both the family and the patient were not equipped with the necessary knowledge regarding the nature of her disease and its prognosis. The patient was mismanaged and kept ignorant of the importance of a proper follow-up. This necessitates a multidisciplinary team approach towards such cases so that their disease can be adequately managed. The early diagnosis and symptomatic management of complications as they arise remain the most important and vital step in the management of this illness. We hope that our case sheds further light on the existing knowledge of this syndrome.

摘要

劳伦斯-穆恩-巴德特-比德尔综合征是一种罕见的遗传性疾病,有多种临床表现,如智力发育迟缓、视力下降、肥胖、性腺功能减退和多指畸形。由于该综合征罕见,其诊断很容易被忽视,欧洲报告的患病率为12.5万至16万人中1例。诊断延迟和处理不当可能导致不可逆转的功能丧失。其中最严重的功能丧失包括视力丧失、心脏问题和肾脏异常。这些功能障碍严重影响患者的智力和个人生活。我们的病例呈现出该综合征的显著特征,但由于缺乏认识,其家人未得到充分的咨询。家人和患者都不具备关于其疾病性质及其预后的必要知识。患者管理不当,对正确随访的重要性一无所知。对于此类病例,需要采取多学科团队方法,以便对其疾病进行充分管理。并发症出现时的早期诊断和对症处理仍然是该疾病管理中最重要和关键的步骤。我们希望我们的病例能进一步阐明对该综合征的现有认识。

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