Claris Appiani A, Marra G, Tirelli S A, Goj V, Romeo L, Cavanna G, Assael B M
Acta Paediatr Scand. 1986 Nov;75(6):970-4. doi: 10.1111/j.1651-2227.1986.tb10325.x.
Fractional excretion of electrolytes, renal acidification capacity and the renin-aldosterone system have been studied in 5 non-azotemic children, 19-25 months old, with mineralocorticoid resistant hyperkalemia, discovered in the first month of life. Although fractional potassium excretion was similar in patients and in a group of control healthy children (13.8 +/- 5.2% vs. 8.7 +/- 6.4%) it was inappropriately low in the patients for their higher potassium concentration. Fractional sodium excretion was significantly increased in the patients (1.6 +/- 0.3% vs. 0.67 +/- 0.4, p less than 0.02). Normal net acid and ammonium excretion and intact ability to lower urinary pH during acid loading were observed in all patients. Mean values for plasma aldosterone (37.0 +/- 9.1 vs. 13.9 +/- 11.2 ng/dl), plasma renin activity (12.5 +/- 3.9 vs. 8 +/- 2.8 ng/ml/h) and plasma aldosterone/plasma potassium ratio (7.11 +/- 1.5 vs. 3.08 +/- 1.7) were higher in the patients than in the control subjects (all p less than 0.001). These data support the hypothesis that a partial lack of response of the renal tubule to endogenous mineralocorticoids was present in the patients. This type of pseudohypoaldosteronism is less severe than that described for the classic form and for early childhood renal acidosis.
对5名年龄在19至25个月、无氮质血症、出生后第一个月即发现有盐皮质激素抵抗性高钾血症的儿童进行了电解质排泄分数、肾脏酸化能力和肾素 - 醛固酮系统的研究。尽管患者组和一组健康对照儿童的钾排泄分数相似（分别为13.8±5.2%和8.7±6.4%），但对于患者较高的钾浓度而言，其钾排泄分数却异常低。患者的钠排泄分数显著增加（分别为1.6±0.3%和0.67±0.4，p<0.02）。所有患者均观察到正常的净酸和铵排泄，以及在酸负荷期间降低尿液pH值的完整能力。患者的血浆醛固酮平均值（37.0±9.1对13.9±11.2 ng/dl）、血浆肾素活性（12.5±3.9对8±2.8 ng/ml/h）和血浆醛固酮/血浆钾比值（7.11±1.5对3.08±1.7）均高于对照组（所有p<0.001）。这些数据支持了患者肾小管对内源性盐皮质激素存在部分反应缺失的假说。这种类型的假性醛固酮减少症比经典型和儿童早期肾性酸中毒所描述的情况要轻。
