Vomiting in an infant with congenital nephrotic syndrome: Questions
Vomiting in an infant with congenital nephrotic syndrome: Questions
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先天性肾病综合征婴儿呕吐：问题
DOI：
10.1007/s00467-016-3529-8
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发表时间：
2017
影响因子：
3
通讯作者：
Okamoto Takayuki
中科院分区：
文献类型：
--
作者：
Takahashi Toshiyuki;Sato Yasuyuki;Yamazaki Takeshi;Hayashi Asako;Okamoto Takayuki
背景：大多数先天性肾病综合征（CNS）患者表现为不能茁壮成长。先前的一项研究报道41例（12%）患有中枢神经系统的婴儿中有5例患有肥厚性幽门狭窄（HPS），需要手术治疗。其原因尚不清楚，并且很少有关于这些疾病或其临床过程之间关系的报道。病例诊断/治疗我们报告一例4个月大的中枢神经系统女婴。她没有表现出HPS的典型表现，但在诊断HPS之前，反复超声检查发现粘膜和粘膜下层增厚，幽门肌肥大。进行了幽门成形术，以改善她的体重增加，并导致理想的生长。结论本病例提示中枢神经系统患者胃出口梗阻可能与体重增加不良密切相关。即使没有典型的症状，我们也应该怀疑与HPS有关，积极的干预可能会改善生长不良。幽门窦粘膜增厚水肿和粘膜下层有可能引起中枢神经系统HPS的高频率。
BackgroundMost patients with congenital nephrotic syndrome (CNS) exhibit a failure to thrive. A previous study reported that five of 41 (12 %) infants with CNS had hypertrophic pyloric stenosis (HPS) requiring surgery. The reason for this is undetermined, and there are few reports regarding the relationship between these conditions or their clinical course.Case diagnosis/treatmentWe present the case of a 4-month-old girl with CNS. She did not show typical manifestations of HPS, but thickened mucosal and submucosal layers and hypertrophy of the pyloric muscle were detected by repeated ultrasound examinations prior to the diagnosis of HPS. Pyloroplasty was performed to improve her poor weight gain and led to ideal growth.ConclusionsOur case indicates that obstruction of the gastric outlet may be strongly associated with poor weight gain in patients with CNS. We should suspect involvement with HPS even if typical symptoms are lacking, and an aggressive intervention may improve poor growth. Thickened edematous mucosal and submucosal layers at the pyloric antrum have the potential to cause the high frequency of HPS in CNS.