Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas.
Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas.
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胰腺囊性纤维化中汗液电解质成分异常。
DOI：
10.1542/peds.12.5.549
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发表时间：
1953
期刊：
影响因子：
--
通讯作者：
Ethel Shea
中科院分区：
文献类型：
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作者：
Paul A. DI SANT'AGNESE;Robert C. Darling;George A. Perera;Ethel Shea
在 43 名患有胰腺囊性纤维化的患者中，发现对标准温和热刺激做出反应而分泌的汗液电解质成分异常。汗液中钠和氯的浓度是 50 名患有各种其他疾病的患者的 2 至 4 倍。胰腺囊性纤维化患者的汗液钾水平也有所增加，尽管程度不一样。相比之下，胰腺纤维囊性疾病患者和对照患者的出汗量没有显着差异。
研究表明，胰腺缺乏症、慢性肺部疾病和服用某些药物本身并不影响汗液的电解质成分。代谢研究还表明，肾和肾上腺功能紊乱并不是纤维囊性疾病中高汗液氯化物和钠的原因。在限盐期间，汗液中增加的电解质与尿液中极低水平的电解质之间的解离是这种情况所特有的。
事实上，在炎热的天气、限盐期间或使用 DCA 时，汗腺不能有效地保留盐分，这表明汗腺本身受到干扰。这种异常可能不是胰腺囊性纤维化的特征，但这是一个预期的发现。
汗液成分的这些紊乱以及唾液腺分泌异常的初步观察表明，许多甚至可能所有外分泌腺的分泌活动在胰腺囊性纤维化中受到影响。鉴于至少两个非粘液分泌结构在这种情况下受到影响，术语“粘液粘稠症”似乎并不合理。
炎热天气中出汗导致的大量盐分流失，足以解释胰腺囊性纤维化的“热损伤”。讨论了其机制，并强调了症状是由纯粹的盐流失和随之而来的细胞外液体积减少引起的。由于如果不治疗，这种情况可能会导致致命的心血管衰竭，因此应将其视为紧急医疗紧急情况。
通常情况下，胰腺纤维囊性病变的血清电解质浓度是正常的。当这种疾病存在明显的肺功能障碍时，血浆中的碳酸氢盐升高，而氯化物补偿性降低。因此，有必要证明血清钠和氯化物降低，以检测炎热天气中可能发生的大量盐流失。
The electrolyte composition of sweat secreted in response to a standard mild thermal stimulus was found to be abnormal in 43 patients with cystic fibrosis of the pancreas. Sodium and chloride concentrations in sweat were 2 to 4 times as high as those found in 50 patients with a variety of other diseases. Sweat potassium levels were also increased in cystic fibrosis of the pancreas although not to the same extent. In contrast, the amount of sweat was not significantly different in patients with fibrocystic disease of the pancreas and control patients.
It was shown that pancreatic deficiency, chronic pulmonary disease and the administration of certain drugs do not per se affect the electrolyte composition of sweat. It was also demonstrated by metabolic studies that disturbances in renal and adrenal function are not responsible for the high sweat chloride and sodium in fibrocystic disease. The dissociation between increased electrolytes in the sweat and their very low levels in the urine during salt restriction are unique to this condition.
The fact that the sweat glands do not retain salt effectively in hot weather, during salt restriction or when DCA is administered, points to a disturbance in the sweat glands themselves. This abnormality may not be pathognomonic of cystic fibrosis of the pancreas, but it is an expected finding.
These disturbances in sweat composition together with preliminary observations of abnormal salivary gland secretion suggest that the secretory activity of many and perhaps all exocrine glands is affected in cystic fibrosis of the pancreas. The term "mucoviscidosis" does not seem justified in view of the fact that at least two nonmucus-secreting structures are affected in this condition.
Massive salt loss through sweating in hot weather accounts satisfactorily for the "heat casualties" in cystic fibrosis of the pancreas. The mechanisms are discussed and it is emphasized that the symptoms are initiated by pure salt loss and the consequent reduction in extracellular fluid volume. Since the condition may, if not treated, lead to fatal cardiovascular collapse, it should be regarded as an acute medical emergency.
Under the usual circumstances, serum electrolyte concentrations are normal in fibrocystic disease of the pancreas. In the presence of marked pulmonary dysfunction in this disease, the bicarbonate of the plasma is elevated and the chloride reduced in compensation. It is therefore necessary to demonstrate a lowered serum sodium as well as chloride to detect the massive salt loss which may occur in hot weather.