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Published on: August 4, 2023
Vomiting in an infant with congenital nephrotic syndrome: Questions
Toshiyuki Takahashi1, Yasuyuki Sato2, Takeshi Yamazaki2
1Department of Pediatrics, Hokkaido University Hospital, Kita 15-jo Nishi 7-Chome, Kita Ward, Sapporo City, Hokkaido, Japan. t-toshi@med.hokudai.ac.jp.
Insights
Congenital nephrotic syndrome (CNS) infants may have a higher risk of hypertrophic pyloric stenosis (HPS). Early intervention for gastric outlet obstruction can improve poor weight gain in these patients.
Area of Science:
- Pediatric Nephrology
- Gastroenterology
Background:
- Congenital nephrotic syndrome (CNS) frequently causes failure to thrive.
- A prior study noted 12% of infants with CNS had hypertrophic pyloric stenosis (HPS).
- The association between CNS and HPS remains unclear.
Observation:
- A 4-month-old girl with CNS presented with poor weight gain.
- Ultrasound revealed thickened pyloric mucosal/submucosal layers and muscle hypertrophy, preceding typical HPS symptoms.
- Pyloroplasty was performed to address the gastric outlet obstruction.
Findings:
- The patient experienced improved weight gain after pyloroplasty.
- Gastric outlet obstruction may be linked to poor growth in CNS patients.
- Thickened pyloric antrum tissues could contribute to HPS prevalence in CNS.
Implications:
- Suspect HPS in CNS infants with poor weight gain, even without classic symptoms.
- Prompt surgical intervention like pyloroplasty can enhance growth outcomes.
- Understanding this association may improve management of CNS complications.
Background:
Most 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/Treatment:
We 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.
Conclusions:
Our 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.
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