Hyperkalemic Renal Tubular Acidosis

腎小管酸血症之高血鉀

溫崧峰(Sung-Feng Wen)

《Acta Nephrologica》 14卷1期 (2000/01) Pp. 1-11

摘要

腎小管酸血症之高血引一種遠端小管對鉀及氫離子運送的損害。雖然腎小管酸症之高血鉀常伴隨輕度腎功能不全（renal ineufficieng）。高血鉀興腎功能不全的程度不成比例。其病理生理機轉牽涉腎臟鉀離子運送，特別是與醛固酮(aldosterone)，遠端腎小管鈉離子傳送，遠端腎小管陰電荷（electronegatrivity），氨生成（ammonicqenecys）及銨的運送（ammoniunr traneport）。均被回顧實驗室的損標，如腎小管內外鉀離子梯度（tramstubular potassium gradient）鉀離子排泄率（fractional potassium eceretion）及尿液淨電荷可作為有用的診斷工具。臨床上造成腎小管酸血症之高血鉀可能是遺傳或後天礦物性增質繫muneralocorticord不足或抵抗（resistance），或一些藥物所造成。糖尿病腎病變，阻塞性腎病變鐮狀細胞疾，止痛藥腎病變，腎小管間質疾病，紅斑性浪瘡腎炎及HIV腎病變較易患腎小管酸血症之高血鉀。非類固醇抗炎藥物，保鉀離子利尿劑（potassiun-sparing diureties），血管升壓素轉化酶抑制（ACEI）, 環孢靈（cyclosporine），乙型阻斷劑（٢-blockers）及其他藥物也較易誘發生腎小管酸血症之高血鉀。治療之目的是矯正高血鉀。方法為改正基本的病理生理，限制食物的鉀離子量，停用會造成高血鉀之藥物及使用作用於享利氏蹄系管利尿劑（loop diuretics）或併用鹼性治療以誘生排鈉及排鉀效果以矯正高血鉀及代謝性酸血症。需要時鈉鉀離子交換樹脂也可被使用。礦物性間質性（mineralocorticoids）的使用對某些選擇性病患有效但必須避免高血壓及鈉離子滯留。

關鍵字

高血鉀 ； 第四型腎小管酸血症 ； 醛固酮 ； 氨生成 ； 腎小管間質疾病

並列摘要

Hyperkalemic renal tubular acidosis (RTA) is a disorder of distal tubular transport with impaired secretion of potassium and hydrogen ion. Although hyperkalemic RTA is frequently associated with mild renal insufficiency, the hyperkalemia is out of proportion to the degree of renal dysfunction. The pathophysiological mechanisms involving renal potassium transport, especially in relation to aldosterone, distal sodium delivery, distal electronegativity, ammoniagenesis and ammonium transport, are reviewed. Certain laboratory parameters such as transtubular potassium gradient (TTKG), fractional potassium excretion (FEk) and urine net charge (UNC) may serve as useful diagnostic tools. The clinical disorders leading to hyperkalemic RTA may be caused by hereditary or acquired mineralocorticoid deficiency or resistance, or induced by a number of drugs. Certain renal diseases are more prone to be associated with hyperkalemic RTA. They include diabetic nephropathy , obstructive nephropathy, sickle cell disease, analgesic nephropathy and other tubulointerstitial diseases, lupus nephritis and HIV nephropathy. The drugs which are more likely to induce hyperkalemia are nonsteroidal anti-inflammatory drugs (NSAIDs), potassium-sparing diuretics, angiotensin-converting enzyme (ACE) inhibitors, cyclosporine, β-blockers and others. Treatment is aimed at correction of hyperkalemia. The measures to rectify the underlying pathophysiology, dietary potassium restriction, discontinuation of the offending drugs, and the use of loop diuretics with or without alkali therapy to induce natriuresis and kaliuresis should all help to correct hyperkalemia and metabolic acidosis. Na(+)-K(+) exchange resins may be used when needed. Mineralocorticoids may be of value in selected patients but should be avoided in the presence of hypertension or salt retention.

並列關鍵字

hyperkalemia ； type IV renal tubular acidosis ； aldosterone ； ammoniagenesis ； tubulointerstitial disease