Neonatal Bartter Sendromu: İki Olgunun Sunumu

Year 2011, Volume: 5 Issue: 2, 108 - 113, 01.08.2011

Nilay Hakan Nurullah Okumuş Mustafa Aydın Ayşegül Zenciroğlu Özlem Erdoğan Ali Fettah Arzu Dursun Ahmet Afşin Kundak Mehmet Şah İpek

Abstract

Bartter sendromunun neonatal varyantı, intrauterin dönemde başlayan poliürinin neden olduğu ciddi polihidramniyoz ile karakterize otozomal resesif geçişli nadir bir doğumsal renal tübüler bozukluktur. Ağır klinik seyri nedeniyleyaşamı tehdit eden bir durumdur. Burada neonatal Bartter sendromu tanısı konulan iki olgu nadir görülmesi nedeniyle ve uygun sıvı-elektrolit desteğiyle birlikte indometazin tedavisinin erken dönemde başlanmasının doğru bir yaklaşım olacağının vurgulanması amacıyla sunuldu.

Keywords

Neonatal, Bartter sendromu, renal tübüler bozukluk, preterm doğum, polihidramniyoz, nefrokalsinozis, indometazin

NEONATAL BARTTER’S SYNDROME: REPORT OF TWO CASES

Abstract

Neonatal Bartter’s syndrome is a rare autosomal recessive congenital renal tubular disorder that characterized by severe polyhydramnios resulting from intrauterine onset polyuria. It is a life-threatening condition because of its severe clinic course. We report here two rare cases of neonatal Bartter’s syndrome in order to stress that early indomethacin treatment in addition to appropriate fluid and electrolyte support would be an appropriate approach

Keywords

Neonatal, Bartter’s syndrome, renal tubular disorder, premature delivery, polyhydramnios, nephrocalcinosis, indomethacin

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