adrenocortical insufficiency

肾上腺皮质功能不全
  • 文章类型: Journal Article
    肾上腺皮质功能不全,也称为肾上腺功能不全(AI),是一种以肾上腺激素产生不足为特征的内分泌紊乱,包括糖皮质激素和盐皮质激素(MC)。这种情况可以归类为主要的,次要,或三级AI,取决于缺陷的位置。AI的经典症状包括虚弱,疲劳,腹痛,心动过速,低血压,电解质失衡,和色素沉着过度。在儿童中,AI的最常见原因是经典的先天性肾上腺增生,这是由于21-羟化酶的缺乏。21-羟化酶产生所有类固醇,如皮质醇和醛固酮。AI管理主要涉及激素替代疗法,通常与口服氢化可的松和MC补充。然而,对儿科患者给予氢化可的松带来了与缺乏可用剂量合适的制剂相关的挑战.历史上,压碎或分开的成人片剂用于儿科治疗AI,尽管这会增加治疗不足或过度的风险。儿科人群的剂量不足会对生长产生不利影响,发展,和代谢健康。AlkindiSprinkle是一种儿科特异性氢化可的松口服颗粒制剂,可管理皮质醇水平以帮助促进准确的治疗剂量。Alkindi提供了几个优势,包括准确的剂量,味道掩蔽,易于管理。目前的调查描述了人工智能,人工智能的管理,以及使用AlkindiSprinkle治疗小儿AI,包括临床疗效。
    Adrenocortical insufficiency, also known as adrenal insufficiency (AI), is an endocrine disorder characterized by inadequate production of adrenal hormones, including glucocorticoids and mineralocorticoids (MCs). The condition can be categorized as primary, secondary, or tertiary AI, depending on the location of the defect. Classical symptoms of AI include weakness, fatigue, abdominal pain, tachycardia, hypotension, electrolyte imbalances, and hyperpigmentation. In children, the most common cause of AI is classical congenital adrenal hyperplasia, which results from a deficiency in the 21-hydroxylase enzyme. The 21-hydroxylase enzyme produces all steroids, such as cortisol and aldosterone. AI management primarily involves hormone replacement therapy, typically with oral hydrocortisone and MC supplementation. However, the administration of hydrocortisone to pediatric patients presents challenges related to the lack of available dose-appropriate formulations. Historically, crushed or split adult tablets were used for the pediatric treatment of AI, although this poses an increased risk of under- or overtreatment. Inadequate dosing in the pediatric population can adversely affect growth, development, and metabolic health. Alkindi Sprinkle is a pediatric-specific hydrocortisone oral granule preparation that manages cortisol levels to help facilitate accurate therapeutic dosing. Alkindi offers several advantages, including accurate dosing, taste masking, and ease of administration. The present investigation describes AI, the management of AI, and the treatment of pediatric AI using Alkindi Sprinkle, including clinical efficacy.
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  • 文章类型: Case Reports
    原发性肾上腺淋巴瘤(PAL)是一种极为罕见的恶性肿瘤,约占非霍奇金淋巴瘤(NHL)的1%。肾上腺淋巴瘤的生长特征是在肾上腺中快速浸润和在其他组织和器官中进一步受累和转移。这份报告描述了一名67岁男子疲劳的情况,食欲不振,和减肥。正电子发射断层扫描和计算机断层扫描(PET-CT)扫描显示,在双侧肾上腺中发现了不规则的肿块状软组织密度阴影,免疫组织化学(IHC)研究证实了PAL的诊断并在全身多发转移。本报告描述了PAL患者的临床表现。当疾病进展为双侧肾上腺受累时,可伴有肾上腺功能不全甚至发生肾上腺危象。
    Primary adrenal lymphoma (PAL) is an extremely rare malignancy, and it accounts for approximately 1% of non-Hodgkin\'s lymphoma (NHL). The growth of adrenal lymphoma is characterized by rapid infiltration in the adrenal gland and further involvement and metastasis in other tissues and organs. This report describes the case of a 67-year-old man with fatigue, poor appetite, and weight loss. Positron emission tomography and computed tomography (PET-CT) scan showed irregular mass-like soft tissue density shadows were noted in the bilateral adrenal glands and immunohistochemical (IHC) studies confirmed the diagnosis of PAL with multiple metastases throughout the body. This report characterizes the clinical manifestations in patients with PAL. When the disease progresses to bilateral adrenal involvement, it may be accompanied by adrenal insufficiency or even adrenal crisis occurred.
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  • 文章类型: Case Reports
    背景:自身免疫性多腺体综合征2型(APS-2)是一种罕见的内分泌疾病,伴有自身免疫性甲状腺疾病和/或1型糖尿病。当患者在未识别的Addison病的背景下表现出甲状腺功能减退症的非特异性神经精神特征时,诊断更具挑战性。
    方法:我们报告一例亚临床自身免疫性甲状腺功能减退症,表现为应激引起的非特异性神经精神症状。尽管有左甲状腺素治疗,她的症状恶化,并因持续性呕吐和低血容量性休克入院。临床特征和实验室参数提示潜在的肾上腺皮质功能不全。先前存在的自身免疫性甲状腺功能减退症合并Addison病证实了未识别的APS-2的诊断。仅使用皮质类固醇治疗,她的甲状腺功能检查也得到了显着改善。
    方法:通过检索数据库,我们仅确定了5份已发表的病例报告。Neufeld和Betterle报告了他们的APS-2数据,并得出结论,该综合征的两个或多个组成部分的完整临床表现就像冰山一角。
    结论:应筛查APS-2的一种主要成分的患者,以寻找潜在病例。对左甲状腺素不耐受的甲状腺功能减退症患者应排除Addison病。
    BACKGROUND: Autoimmune polyglandular syndrometype-2 (APS-2) is an uncommon endocrine disorder of Addison\'s disease with an autoimmune thyroid disorder and/or type 1 diabetes mellitus. The diagnosis is more challenging when a patient presents with nonspecific neuropsychiatric features with hypothyroidism in the setting of unrecognized Addison\'s disease.
    METHODS: We report a case of subclinical autoimmune hypothyroidism presented with nonspecific neuropsychiatric symptoms precipitated by stress. Despite levothyroxine treatment, her symptoms deteriorated and she was admitted with persistent vomiting and hypovolemic shock. Clinical features and laboratory parameters were suggestive of underlying adrenocortical insufficiency. Preexisting autoimmune hypothyroidism combined with Addison\'s disease confirmed the diagnosis of unrecognized APS-2. She remarkably improved and her thyroid function tests also normalized with the treatment of corticosteroids only.
    METHODS: We identified only five published case reports of our title by searching the database. Neufeld and Betterle have reported their data of APS-2 and concluded that a full- blown clinical picture of two or more components of the syndrome is like the tip of the iceberg.
    CONCLUSIONS: The patients of one major component of APS-2 should be screened for other components of the disease to pick up latent cases. Addison\'s disease should be ruled out in patients of hypothyroidism who are intolerant to levothyroxine.
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