Simões e Silva Ana Cristina, Oliveira Eduardo A, Gomes Camila Romano, Lima Flávio Souza, Diniz José S S
Departamento de Pediatria, Universidade Federal de Minas Gerais, Belo Horizonte, MG.
J Pediatr (Rio J). 2002 May-Jun;78(3):251-4. doi: 10.2223/jped.839.
The aim of this article is to highlight the importance of a rare disease that causes severe arterial hypertension in children. It is important to advise pediatricians to measure arterial pressure in children in order to avoid late diagnosis and renal insufficiency.
We report a case of a 13-year-old patient that presented typical clinical and laboratorial features of Liddle's syndrome. The diagnosis was established based on the clinical picture associated with the presence of chronic hypokalemia, increase of urinary potassium excretion with sodium retention and reduction of renin plasma activity, and circulating levels of angiotensin II and aldosterone. In a spite of the initial therapeutic response to triamterene, the patient developed progressive renal failure due to the delay in the diagnosis and the poorly controlled hypertension.
Liddle's syndrome consists of a form of pseudoaldosteronism characterized by arterial hypertension, hypokalemia, metabolic alkalosis and failure to thrive. Some aspects regarding physiopathology, diagnosis and treatment are discussed.
本文旨在强调一种导致儿童严重动脉高血压的罕见疾病的重要性。建议儿科医生测量儿童动脉血压，以避免延迟诊断和肾功能不全，这一点很重要。
我们报告了一例13岁患者，其表现出典型的利德尔综合征临床和实验室特征。诊断基于与慢性低钾血症、尿钾排泄增加伴钠潴留以及肾素血浆活性降低、血管紧张素II和醛固酮循环水平相关的临床表现。尽管最初对氨苯蝶啶有治疗反应，但由于诊断延迟和高血压控制不佳，患者出现了进行性肾衰竭。
利德尔综合征是一种假性醛固酮增多症，其特征为动脉高血压、低钾血症、代谢性碱中毒和发育不良。讨论了有关病理生理学、诊断和治疗的一些方面。
