Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis
| Autor: | Sevgi Pekcan, Sevgi Bilgic Eltan, Melih Hangül, Hadice Selimoglu Sen, Ali Özdemir, Demet Can, Ozge Yilmaz, Zeynep Tamay, Mehmet Köse, Velat Şen, Haluk Cokugras, Hasan Yuksel, Ayşe Süleyman, Cem Murat Bal, Erdem Topal, Hakan Yazan, Abdurrahman Erdem Başaran, Ayse Tana Aslan, Ayşen Bingöl, Güzin Cinel, Ugur Ozcelik, Fazil Orhan, Nazan Cobanoglu, Derya Ufuk Altintas, Ebru Yalcin, Lina AlShadfan, Nural Kiper, Ayse Ayzit Kilinc, Zeynep Gökçe Gayretli Aydın, Gokcen Dilsa Tugcu, Ayşe Şenay Şaşihüseyinoğlu, Nagehan Emiralioglu, Şükrü Çekiç, Erkan Cakir, Tugba Sismanlar Eyuboglu, Ömür Ercan, Gönül Çaltepe, Deniz Dogru, Ozlem Keskin, Nihat Sapan, Tugba Ramasli Gursoy |
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| Přispěvatelé: | Ondokuz Mayıs Üniversitesi, Department of Pediatric Pulmonology, Faculty of Medicine, Gazi University, Ankara, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Hacettepe University, Ankara, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Bezmialem Vakif University, İstanbul, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Ankara University, Ankara, Turkey, Department of Pediatric Pulmonology, Meram Medicine Faculty, Necmettin Erbakan University, Konya, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Ankara Yıldırım Beyazıt University, Ankara, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey, Department of Pulmonology, Faculty of Medicine, Dicle University, Diyarbakır, Turkey, Department of Pediatric Allergy, Faculty of Medicine, Gaziantep University, Gaziantep, Turkey, Department of Pediatric Allergy and Immunology, Faculty of Medicine, Çukurova University, Adana, Turkey, Department of Pediatric Allergy and Immunology, Faculty of Medicine, Uludag University, Bursa, Turkey, Department of Pediatric Allergy and Pulmonology, Cerrahpasa Medicine Faculty, İstanbul University, İstanbul, Turkey, Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Akdeniz University, Antalya, Turkey, Department of Pediatric Pulmonology, Mersin City Research & Training Hospital, Mersin, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Erciyes University, Kayseri, Turkey, Department of Pediatric Pulmonology, Ankara Children's Hematology Oncology Training and Research Hospital, Ankara, Turkey, Department of Pediatric Pulmonology, Allergy and Immunology, Faculty of Medicine, Celal Bayar University, Manisa, Turkey, Department of Pediatric Allergy, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey, Department of Pediatric Infectious Disease, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey, Department of Pediatric Allergy, Faculty of Medicine, İnonu University, Malatya, Turkey, Department of Pediatric Allergy and Immunology, İstanbul Facultyof Medicine, İstanbul University, İstanbul, Turkey, Department of Pediatric Pulmonology, Faculty of Medicine, Balıkesir University, Balıkesir, Turkey, Department of Pediatric Pulmonology, Doctor Lufti Kirdar Kartal Training and Research Hospital, ıstanbul, Turkey, Department of Pediatric Gastroenterology, Faculty of Medicine, Ondokuz Mayıs University, Samsun, Turkey, ÇAKIR, Erkan, Bursa Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı., Sapan, Nihat, Çekiç, Şükrü, FUI-8766-2022 |
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Newborn screening
Male Scoring system Cystic Fibrosis Turkey Cystic Fibrosis Transmembrane Conductance Regulator registry Pediatrics Cystic fibrosis Turkey (republic) cystic fibrosis Turkey (bird) Registries Child Priority journal Diagnostic test Register Sismanlar E. Dogru D. Çakır E. Cobanoglu N. Pekcan S. Cinel G. Yalçın E. Kiper N. Sen V. Selimoglu S. et al. -Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.- Pediatric pulmonology 2020 Bacterial colonization Phenotype Body mass Child Preschool Female Forced expiratory volume Human Adult Pulmonary and Respiratory Medicine Registry medicine.medical_specialty Current age Adolescent Pseudo Bartter Syndrome Major clinical study Bartter syndrome Respiratory system Article Young Adult Neonatal Screening Internal medicine Genetic screening Genetics medicine Humans In patient Gene mutation Disease registry Pseudo-Bartter syndrome business.industry Body Weight Infant Newborn Bartter Syndrome Infant Mean age Alkalosis Mutational analysis Newborn Nonhuman Colonization status medicine.disease Body height Clinical feature Preschool child Mutation Pediatrics Perinatology and Child Health Pseudo-Bartter Syndrome Comparative study Complication business Controlled study |
| Popis: | Sismanlar Eyuboglu, Tugba/0000-0001-7284-4999; Dogru, Deniz/0000-0001-9931-9473; Aslan, Ayse Tana/0000-0002-5360-8517; COBANOGLU, NAZAN/0000-0002-3686-2927; ORHAN, FAZIL/0000-0002-4850-932X WOS: 000530442700001 PubMed: 32364312 Background Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population. Methods The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017. Clinical features, diagnostic test results, colonization status, complications, and genetic test results were compared in patients with and without PBS. Results Totally 1170 patients were recorded into the registry in 2017 and 120 (10%) of them had PBS. The mean age of diagnosis and current age of patients were significantly younger and newborn screening positivity was lower in patients with PBS (P < .001). There were no differences between the groups in terms of colonization status, mean z-scores of weight, height, BMI, and mean FEV1 percentage. Types of genetic mutations did not differ between the two groups. Accompanying complications were more frequent in patients without PBS. Conclusion PBS was detected as the most common complication in the registry. It could be due to warm weather conditions of our country. It is usually seen in younger ages regardless of mutation phenotype and it could be a clue for early diagnosis of CF. |
| Databáze: | OpenAIRE |
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