儿童高钾及低钾血症发生机制及危重处置要点

钾是体内含量最多的细胞内离子,具有重要的生理功能,维持体内钾浓度的稳定具有十分重要的意义。钾离子失衡可影响肌肉的收缩力、神经传导和心肌电生理。无论是高钾血症还是低钾血症都可产生严重后果,快速明确病因并给予有效治疗可挽救患儿生命。     

先天性副肌强直 1例临床报告及基因检测分析

目的 报道1例先天性副肌强直(PMC)病人的临床资料和基因检测结果(包括病人及其父母、姐姐),并文献复习.方法 分析安徽医科大学第一附属医院内分泌科于2019年12月22日收治的1例反复发作低钾血症伴横纹肌溶解综合征病人的临床资料(包括病史、体格检查、实验室辅助检查结果),并完善基因全外显子组测序,同时检测该病人父母及姐姐的相关基因.查阅国内外先天性副肌强直的相关文献,总结该病的临床特点及突变基因类型.结果 病人编码钠通道α亚单位的骨骼肌钠通道基因(SCN4A基因)存在c.3318+10(内含子17)C>T的杂合突变,结合该病人的临床资料及基因检测结果诊断为先天性副肌强直.其父母和姐姐均不存在该致病基因的突变,说明该病人为散发性先天性副肌强直.结论 对临床上难以解释的反复发作的严重低钾血症病人要考虑到罕见疾病的可能,尽早对病人及其家庭成员进行基因检测分析,以明确诊断和精准治疗.     

急性重症低钾血症的快速补钾方案临床应用分析

目的应用快速补钾、常规补钾治疗方案治疗急性重症低钾血症患儿,评价快速补钾方案的临床疗效、安全性。方法用微量注射泵均匀静脉输入治疗严重低钾血症患儿（K＋〈2．5mmol／L）快速补钾速度按（0．4．0．5）mmol／（kg·h）标准,以注射用水或5％葡萄糖液将氯化钾溶液稀释成0．5％～1．5％浓度。K＋〉3．0mmol／L时快速补钾改为常规补钾。补钾前均常规检查心电图、血钾、血气分析,补钾全过程中持续进行心电监测以及经皮氧饱和度监测,每半小时测微量血钾。结果18例应用快速补钾方案,持续心电监护未发现任何与一过性血钾升高有关的心律紊乱。结论快速补钾治疗方案,对于急性重症低钾血症患儿安全有效。     

深静脉补钾对低钾患者治疗效果及依从性的观察

目的观察深静脉泵入高浓度氯化钾治疗低钾血症患者疗效,了解低钾血症患者对微泵深静脉注射治疗和传统静脉滴注治疗的依从性差异。方法对2010年1月-2011年1月入住我院重症医学科的50例低钾血症患者分组给予高浓度氯化钾微泵深静脉补钾和传统静脉滴注补钾,观察补钾6h、12h、24h后血清钾水平,比较不同时间段两种补钾方法的血清钾恢复正常例数及百分率、并发症发生例数及发生率、患者依从性差异。结果各时间段微泵深静脉补钾组血清钾恢复正常的例数和百分率均显著高于传统静脉滴注补钾组,微泵深静脉补钾组无静脉炎和注射局部疼痛发生,并发症发生率显著低于对照组,患者的依从性高（P〈0.05）。结论高浓度氯化钾微泵深静脉补钾见效快,明显优于静脉滴注补钾,患者依从性高。     

Severe metabolic acidosis and hypokalemia in a patient with enterovesical fistula

We report a case of a 59-year-old woman who had severe metabolic acidosis and hypokalemia due to an enterovesical fistula. The patient came to our hospital complaining of systemic weakness and numbness of the fingers. She was found to have hyperchloremic metabolic acidosis (arterial bicarbonate, 2.8 mEq/l) and hypokalemia (serum potassium, 1.9 mEq/l) and was admitted for treatment. Following the correction of metabolic acidosis and hypokalemia, the patient was examined for the underlying cause of these electrolyte and acid-base disorders. She had a history of total hysterectomy followed by radiotherapy due to uterine cancer 30 years previously. After the surgery, she had suffered postoperative neurogenic bladder dysfunction, necessitating intermittent self-catheterization. Two years before admission, she had begun to experience watery diarrhea. A radiographic study after recovery from the acid-base and electrolyte disorders revealed the presence of an enterovesical fistula. The fistula was surgically resected and the metabolic acidosis completely cleared. Unexplained hyperchloremic metabolic acidosis with hypokalemia may suggest the presence of an enterovesical fistula in patients with a surgical history of malignant pelvic tumor and neurogenic bladder dysfunction.     

SFE/SFHTA/AFCE primary aldosteronism consensus: Introduction and handbook

The French Endocrinology Society (SFE) French Hypertension Society (SFHTA) and Francophone Endocrine Surgery Association (AFCE) have drawn up recommendations for the management of primary aldosteronism (PA), based on an analysis of the literature by 27 experts in 7 work-groups. PA is suspected in case of hypertension associated with one of the following characteristics: severity, resistance, associated hypokalemia, disproportionate target organ lesions, or adrenal incidentaloma with hypertension or hypokalemia. Diagnosis is founded on aldosterone/renin ratio (ARR) measured under standardized conditions. Diagnostic thresholds are expressed according to the measurement units employed. Diagnosis is established for suprathreshold ARR associated with aldosterone concentrations > 550 pmol/L (200 pg/mL) on 2 measurements, and rejected for aldosterone concentration < 240 pmol/L (90 pg/mL) and/or subthreshold ARR. The diagnostic threshold applied is different if certain medication cannot be interrupted. In intermediate situations, dynamic testing is performed. Genetic forms of PA are screened for in young subjects and/or in case of familial history. The patient should be informed of the results expected from medical and surgical treatment of PA before exploration for lateralization is proposed. Lateralization is explored by adrenal vein sampling (AVS), except in patients under 35 years of age with unilateral adenoma on imaging. If PA proves to be lateralized, unilateral adrenalectomy may be performed, with adaptation of medical treatment pre- and postoperatively. If PA is non-lateralized or the patient refuses surgery, spironolactone is administered as first-line treatment, replaced by amiloride, eplerenone or calcium-channel blockers if insufficiently effective or poorly tolerated.     

SFE/SFHTA/AFCE consensus on primary aldosteronism,part 1: Epidemiology of PA,who should be screened for sporadic PA?

Depending on the study, the prevalence of primary aldosteronism (PA) in patients with hypertension varies from 6 to 18%. Prevalence is higher in each of the following conditions, any one of which requires screening for PA: severe hypertension (systolic blood pressure [BP] ≥ 180 mmHg and/or diastolic BP ≥ 110 mmHg); resistant hypertension (systolic BP ≥ 140 mmHg and/or diastolic BP ≥ 90 mmHg despite adherence to a tritherapy including a thiazide diuretic); hypertension associated with hypokalemia (either spontaneous or associated with a diuretic); Hypertension or hypokalemia associated with adrenal incidentaloma. It should be borne in mind that PA can induce hypertension without hypokalemia or, less frequently, hypokalemia without hypertension. Finally, as cardiovascular and renal morbidity in PA is greater than in essential hypertension of equivalent level, screening for PA is indicated when cardiovascular or renal morbidity is more severe than predicted from BP level.     

激素致扭转性室性心动过速一例

在临床工作中，激素是经常使用的一种药物，但是它也存在一些并发症容易被忽视，特别是低钾血症。低钾血症是诱发恶性室性心律失常的重要原因之一，尤其在器质性心脏病或原发性心电疾病等心肌细胞电生理特性已经存在异常的情况下，伴发低钾血症更容易发生恶性室性心律失常。现报告1例激素致扭转性室性心动过速病例。     

Mild elevation of extracellular potassium greatly potentiates the effect of sodium channel block to cardiovert atrial fibrillation: The Lankenau approach

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