Dahabreh Muna M, Najada Abdelhamid S
Pediatric Pulmonology Division, Queen Rania Al-Abdullah Hospital for Children, Amman, Jordan.
Saudi J Kidney Dis Transpl. 2013 Mar;24(2):292-6. doi: 10.4103/1319-2442.109579.
Pseudo-Bartter Syndrome (PBS), although quite common in patients with cystic fibrosis (CF), is often missed as simple dehydration or Bartter syndrome. This study was performed in patients with PBS to compare the pattern and course of the disease with those with CF not manifesting with this syndrome. All patients with CF who attended the respiratory clinic at Queen Rania Al-Abdallah Hospital from January 2000 to April 2010 were included in this retrospective case-control study. A specially formulated data sheet was used and those with PBS and those not having the syndrome were identified. A total of 110 patients (51% female) with CF with a median age of seven years were followed-up. Eighteen (16.3%) of them had one or more episodes of PBS. The median follow-up period was 6.2 years. All the episodes occurred during summer and in infancy. Median age of the initial episode of PBS was three months. One-third of them were initially followed at the nephrology clinic. Three patterns of PBS were identified: single episode in three (16.6%) patients, recurrent in 12 (66.6%) patients and chronic in three (16.6%) patients. Early colonization of Pseudomonas spp before 1 st birthday was seen in 44% patients with PBS compared with 12% in other CF patients (P-value = 0.0075). The total number of colonized patients and other CF features at the time of the study did not differ significantly among patients, although the mean Shwachman-Kulczycki score is significantly lower in those with recurrent PBS (69 compared with 85 in other CF patients). Gene mutation was identified in only 30% of the entire cohort. PBS is common in patients with CF, and it should be kept in mind in any patient with hypotonic dehydration and metabolic alkalosis. Recurrent pattern is associated with earlier Pseudomonas colonization.
假性巴特综合征（PBS）在囊性纤维化（CF）患者中相当常见，但常被误诊为单纯脱水或巴特综合征。本研究对PBS患者进行，以比较该疾病的模式和病程与未表现出此综合征的CF患者。2000年1月至2010年4月在拉尼亚·阿卜杜拉王后医院呼吸科就诊的所有CF患者均纳入本回顾性病例对照研究。使用专门制定的数据表，识别出患有PBS和未患该综合征的患者。总共对110例CF患者（51%为女性）进行了随访，中位年龄为7岁。其中18例（16.3%）有一次或多次PBS发作。中位随访期为6.2年。所有发作均发生在夏季和婴儿期。PBS首次发作的中位年龄为3个月。其中三分之一最初在肾病科就诊。确定了三种PBS模式：3例（16.6%）患者为单次发作，12例（66.6%）患者为复发，3例（16.6%）患者为慢性。44%的PBS患者在1岁前有假单胞菌属早期定植，而其他CF患者为12%（P值 = 0.0075）。尽管复发型PBS患者的平均舒瓦克曼 - 库尔奇茨基评分显著低于其他CF患者（分别为69分和85分），但研究时定植患者总数和其他CF特征在患者之间无显著差异。整个队列中仅30%的患者检测到基因突变。PBS在CF患者中很常见，任何低渗性脱水和代谢性碱中毒患者都应考虑到该病。复发模式与假单胞菌早期定植有关。
