التفاصيل البيبلوغرافية
| العنوان: |
Pseudo-Bartter Syndrome in Patients with Cystic Fibrosis and Clinical Features. |
| Alternate Title: |
Kistik Fibrozis Hastalarında Psödo-Bartter Sendromu ve Klinik Özellikleri. (Turkish) |
| المؤلفون: |
Özaslan, Mehmet Mustafa, Şenol, Handan Duman, Barlık, Meral, Çoksüer, Fevziye, Dindar, Bahar, Demir, Esen, Gülen, Figen |
| المصدر: |
Journal of Behcet Uz Children's Hospital; 2023, Vol. 13 Issue 2, p94-100, 7p |
| مصطلحات موضوعية: |
CYSTIC fibrosis, WATER-electrolyte imbalances, AGE differences, GENETIC mutation, WEATHER, HYPERHIDROSIS |
| Abstract (English): |
Objective: Pseudo-Bartter syndrome (PBS) is a complication of cystic fibrosis (CF) accompanied by electrolyte disorders. We aimed to compare the clinical features of patients diagnosed with CF with or without PBS in our clinic. Method: One hundred twenty-eight patients with the diagnosis CF data was recorded. Clinical features, diagnostic test results, colonization status, complications and genetic test results were compared in patients with and without PBS. Results: Totally 128 patients who were regularly followed diagnosis CF January 2017 and May 2022 and 18 of them (14%) developed PBS. Median age of CF diagnosis was significantly lower in patients with PBS (p<0.003). There was a significant difference between the two groups in terms of colonization. In the group with PBS, the chronic respiratory tract colonization was detected more. There were no significant differences for age, gender, weight, height, sweat test. The most common genetic mutation was c1521_1523delCTT (p. F508Del). Conclusion: PBS was the most common finding in our patients with CF. It may be exacerbated by the warm weather conditions in our country. It may be a clue for early diagnosis of CF. [ABSTRACT FROM AUTHOR] |
| Abstract (Turkish): |
Amaç: Psödo-Bartter sendromu (PBS) kistik fibrozis (KF) hastalığının elektrolit bozukluğu ile seyreden bir komplikasyonudur. Kliniğimizde KF tanısı olan, PBS gelişen ve gelişmeyen hastaların klinik özelliklerini karşılaştırmayı hedefledik. Yöntem: Kistik fibrosis tanısı olan 128 hastanın verileri kayıt edildi. PBS gelişen ve gelişmeyen hastaların klinik özellikleri, tanısal test sonuçları, kolonizasyon durumları, komplikasyonları ve genetik sonuçları karşılaştırıldı. Bulgular: Kistik fibrozis tanısıyla Ocak 2017-Mayıs 2022 tarihleri arasında kliniğimizde düzenli takip edilen 128 hastamız olup bunların 18’inde (%14) PBS gelişti. Hastaların ortalama tanı yaşı PBS olanlarda anlamlı olarak daha düşüktü (p<0,003). Yaş, cinsiyet, ağırlık, boy, ter testi, kronik solunum yolları bakteriyel kolonizasyonları ve KF komplikasyonlar arasında anlamlı farklılık yoktu. En sık görülen genetik mutasyon deltaF508 idi. Sonuç: PBS kistik fibrozis hastalarımızda en sık görülen bulguydu. Ülkemizde sıcak hava koşulları buna neden olabilir. Kistik fibrozis hastalığının erken tanısı için ip ucu olabilir. [ABSTRACT FROM AUTHOR] |
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