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- Title
Multisystemic Severe Form Pseudohypoaldosteronism: Can Gastrostomy be Useful in the Management?
- Authors
Önder, Aşan; Çetinkaya, Semra; Kara, Cengiz; Zenciroğlu, Ayşegül; Aycan, Zehra
- Abstract
A ten-day-old male infant was brought to the hospital with vomiting. On admission, he was hypotonic and had accompanying mild dehydration. The laboratory findings revealed hyponatremia, hyperkalemia, and mild metabolic acidosis. The hormone profile (plasma renin activity: 45 ng/ml/h, aldosterone >20 000 pg/ml, ACTH: 53 pg/ml, 17 OH progesterone: 6.7 ng/ml) revealed pseudohypoaldosteronism (PHA). Oral and IV NaCl, anti-potassium therapy (kayexalate), and low-potassium formula were started. His daily salt requirement was 12 grams. He was not able to take this total salt requirement orally. Respiratory symptoms and diarrhea episodes developed at the age of 3 months. The sodium concentration of sweat was 106 mEq/L. He was therefore diagnosed with multisystemic severe form of type 1 PHA. Severe salt wasting could not be treated. We planned to perform gastrostomy to administer his medications effectively but he died due to a salt-wasting crisis.
- Subjects
GASTROSTOMY; HYPOALDOSTERONISM
- Publication
Journal of Pediatric Disease / Türkiye Çocuk Hastalıkları Dergisi, 2016, Vol 10, Issue 2, p134
- ISSN
1307-4490
- Publication type
Case Study
- DOI
10.12956/tjpd.2015.146