Kistik fibrozisli hastada psödo-bartter sendromu

Abstract

Bartter sendromunun karakteristik bulguları olan hipokalemi, hipokloremi ve metabolik alkaloz ile beraber plazma renin ve aldosteron düzeylerinde yükseklik Gitelman sendromu, diabetes insipitus ve kistik fibrozisinde içinde yer aldığı bir grup hastalıkta da tanımlanmıştır. Bu durum psödo-Bartter sendromu PBS olarak isimlendirilmektedir. Bu çalışmada PBS tablosu gösteren kistik fibrozisli bir hastanın klinik ve laboratuvar özellikleri incelenmiştir

Keywords

• Kistik fibrozis,psödo-Bartter sendromu

Pseudo-bartter's syndrome in a child wıth cystic fibrosis

Abstract

Characteristic findings of Bartter's syndrome which include hypokalemia, hypochloremia, metabolic alkalosis, and elevated plasma renin and aldosterone levels are detected in a group of diseases including Gitelman’s syndrome, diabetes insipidus and cystic fibrosis. This condition is known as pseudo-Bartter's syndrome PBS . In this study the clinical and laboratory fındings of a patient with cystic fibrosis and PBS were evaluated

Keywords

• Cystic fibrosis,pseudo-Bartter's syndrome

References

1. Ratjen F, Döring G. Cystic fibrosis. Lancet 2003; 36: 681 - 89
2. Davison AG, Snodgrass GJAL. Cystic fibrosis mimicking Bartter’s syndrome. Acta Pediatr Scand, 1983; 72: 781.
3. Özçelik U, Kiper N, Göçmen A, Coşkun T, Nik-Ain A. Kistik fibrozisli hastalarda psödo-Bartter sendromu. Çocuk Sağlığı ve Hastalıkları Dergisi. 1993; 36: 221.
4. Fustic S, Jordanova NP, Slaveska N, Koceva S, Efremov G. Metabolik alkalosis with hypoelectrolytemia in infants with cystic fibrosis. Pediatr Int, 2002; 44; 289 .
5. Kennedy JD, Dinwiddie R, Daman- Willems C, Dillon MJ, Matthew DJ. Pseudo-Bartter’s syndrome in cystic fibrosis. Arch Dis Child, 1990; 65: 786 - 7.
6. Baird J S. Metabolic alkalosis and cystic fibrosis. Chest, 2002; 122(2): 755 – 6.
7. Sanfelice NF, Zucchi L. Bartter's syndrome: two case reports in childhood. J Pediatr (Rio J), 1998; 74 (6): 473 - 8.
8. Weller F, Wiebicke W, Tummler B. Turkish infant with hypoelectrolytemia and metabolic alkalosis as the sole manifestations of a mild form of cystic fibrosis (mutation D110H). Klin Padiatr, 2000; 212 (1): 41 - 3.

9. Calo LA, Davis PA, Semplicini A. Bartter's - Gitelman's syndrome: a model for the relationships between hypertension, angiotensin II, oxidative stress and remodeling. Clin Nephrol, 2003; 59 (5): 393 - 4.
10. Simopoulos AP, Lapey A, Boat TF, Di Sant’Agnese PA, Bartter FC. The renin-angiotensin-aldosteron system in patients with cystic fibrosis of the pancreas. Pediatr Res, 1971; 5: 626 - 32.
11. Calo L, Davis PA, Milani M. Bartter's syndrome and Gitelman's syndrome: two entities sharing the same abnormality of vascular reactivity. Clin Nephrol.1998; 50 (1):65 -7.
12. MacLusky I, Levison H. Cystic fibrosis. In: Kendig’s Disorders of the Respiratory Tract in Children. Chernick V, Boat TF (eds). 6 th ed. Philadelphia: W.B. Saunders Co,1998:838-82.