Multisistemik Ağır Form Psödohipoaldosteronizm: Gastrostomi Psödohipoaldosteronizm Yönetiminde Kullanılabilir mi?

Abstract

10 günlük erkek bebek kusma şikayeti ile hastaneye getirildi. Başvuruda hipotonikti ve orta derece dehidratasyonu vardı. Hastanın laboratuvar bulgularında hiponatremi, hiperpotasemi ve orta derecede metabolik asidoz mevcuttu. Hormon profili psödohipoaldosteronizm tanısını desteklemekteydi (plasma renin aktivitesi: 45 ng/ml/h, aldosteron >20 000 pg/ ml, ACTH: 53 pg/ml, 17 OH progesteron: 6.7 ng/ml). Oral ve İV NaCl, anti-potasyum tedavi (kayeksalat), düşük potasyumlu mama başlandı. Günlük tuz ihtiyacı 12 gramdı. Olgu tuz ihtiyacının tamamını oral alamamaktaydı. Olgu üç aylık olduğunda solunum semptomları ve diyare epizodları gelişti. Ter sodyum konsantrasyonu 106 mEq/L bulundu. Bu nedenle multisistemik ağır form psödohipoaldosteronizm tip 1 tanısı konuldu. Ağır tuz kaybı karşılanamadı. Tedavilerini etkin verebilmek için gastrostomi planlandı. Ancak olgu tuz kaybı krizi ile kaybedildi.

Keywords

• Çocuk, Gastrostomi, Psödohipoaldosteronizm

Multisystemic Severe Form Pseudohypoaldosteronism: Can Gastrostomy be Useful in the Management?

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

Keywords

• Child, Gastrostomy, Pseudohypoaldosteronism

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