Scurati-Manzoni Elisabetta, Fossali Emilio F, Agostoni Carlo, Riva Enrica, Simonetti Giacomo D, Zanolari-Calderari Maura, Bianchetti Mario G, Lava Sebastiano A G
Pediatric Emergency Unit, De Marchi Hospital, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Pediatr Nephrol. 2014 Jun;29(6):1015-23. doi: 10.1007/s00467-013-2712-4. Epub 2013 Dec 11.
Cystic fibrosis per se can sometimes lead to hyponatremia, hypokalemia, hypochloremia or hyperbicarbonatemia. This tendency was first documented 60 years ago and has subsequently been confirmed in single case reports or small case series, most of which were retrospective. However, this issue has not been addressed analytically. We have therefore systematically reviewed and analyzed the available literature on this subject.
This was a systematic review of the literature.
The reports included in this review cover 172 subacute and 90 chronic cases of electrolyte imbalances in patients with cystic fibrosis. The male:female ratio was 1.57. Electrolyte abnormalities were mostly associated with clinically inapparent fluid volume depletion, mainly affected patients aged ≤2.5 years, frequently tended to recur and often were found before the diagnosis of cystic fibrosis was established. Subacute presentation often included an history of heat exposure, vomiting, excessive sweating and pulmonary infection. History of chronic presentation, in contrast, was often inconspicuous. The tendency to hypochloremia, hypokalemia and metabolic alkalosis was similar between subacute and chronic patients, with hyponatremia being more pronounced (P < 0.02) in subacute compared to chronic presentations. Subacute cases were treated parenterally; chronic ones were usually managed with oral salt supplementation. Retention of urea and creatinine was documented in 38 % of subacute cases.
The findings of our review suggest that physicians should be aware that electrolyte abnormalities can occur both as a presenting and a recurring feature of cystic fibrosis.
囊性纤维化本身有时可导致低钠血症、低钾血症、低氯血症或高碳酸氢盐血症。这种倾向于60年前首次被记录，随后在单个病例报告或小病例系列中得到证实，其中大多数是回顾性的。然而，这个问题尚未得到分析性探讨。因此，我们对关于这个主题的现有文献进行了系统的回顾和分析。
这是一项对文献的系统回顾。
本综述纳入的报告涵盖了172例亚急性和90例慢性囊性纤维化患者电解质失衡病例。男女比例为1.57。电解质异常大多与临床上不明显的液体量减少有关，主要影响年龄≤2.5岁的患者，经常易于复发，且常在囊性纤维化诊断确立之前就已出现。亚急性表现常包括有受热、呕吐、多汗和肺部感染史。相比之下，慢性表现的病史往往不明显。亚急性和慢性患者低氯血症、低钾血症和代谢性碱中毒的倾向相似，与慢性表现相比，亚急性表现中的低钠血症更为明显（P < 0.02）。亚急性病例采用胃肠外治疗；慢性病例通常通过口服补充盐分处理。38%的亚急性病例记录有尿素和肌酐潴留。
我们的综述结果提示，医生应意识到电解质异常可能作为囊性纤维化的首发和复发特征出现。
