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首页> 外文期刊>Hormone and Metabolic Research >The prevalence of familial hyperaldosteronism in apparently sporadic primary aldosteronism in Germany: A single center experience
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The prevalence of familial hyperaldosteronism in apparently sporadic primary aldosteronism in Germany: A single center experience

机译:家族性醛固酮增多症在德国明显零星的原发性醛固酮增多症中的流行:单中心经验

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摘要

Primary aldosteronism (PA) is the most frequent cause of secondary arterial hypertension. To date 3 forms of familial hyperaldosteronism (FH) have been described accounting for a small percentage of all PA cases. In Germany, the prevalence of FH is currently unknown. Our aim was to determine the prevalence of familiarity in a large cohort of patients with PA. A total of 166 patients with apparently sporadic PA in Munich were investigated. FH types I, II, and III were identified using established clinical, biochemical, and molecular criteria. Among the 166 patients with PA, 2 patients (1.2%) reported a family history suggestive of FH. None of the 166 patients showed clinical, endocrine, or genetic evidence of FH type I. The 2 families had characteristic features of FH type II. Family A had 3 subjects affected out of 11 evaluated family members. Family B had 3 out of 4. Bilateral adrenal hyperplasia and unilateral adrenal adenoma were found within the same family. FH type I and FH type III are rare in Germany. With a prevalence of 1.2%, FH type II seems to be more common in apparently sporadic PA than had been assumed so far.
机译:原发性醛固酮增多症(PA)是继发性动脉高压的最常见原因。迄今为止,已经描述了3种形式的家族性醛固酮增多症(FH),占所有PA病例的一小部分。在德国,FH的患病率目前未知。我们的目的是确定大量PA患者的熟悉程度。在慕尼黑共调查了166例明显散发的PA患者。使用既定的临床,生化和分子标准确定了FH I,II和III型。在166例PA患者中,有2例(1.2%)报告有家族史提示FH。 166例患者均未显示出I型FH的临床,内分泌或遗传学证据。这两个家族均具有FH II型的特征。在评估的11位家庭成员中,家庭A有3位受试者受到影响。 B族中有3个(共4个)。在同一家庭中发现了双侧肾上腺增生和单侧肾上腺腺瘤。 FH I型和FH III型在德国很少见。 II型FH的患病率约为1.2%,似乎在零星的PA中比迄今为止所假设的更为普遍。

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