- On the complexity of the diagnosis of pseudo-Bartter’s syndrome (a clinical case in a child with cystic fibrosis)
On the complexity of the diagnosis of pseudo-Bartter’s syndrome (a clinical case in a child with cystic fibrosis)
SOVREMENNAYA PEDIATRIYA.2018.5(93):45-48; doi 10.15574/SP.2018.93.45
Ilchenko S. I., Fialkovska A. O., Ivanus S. G.
SI «Dnіpropetrovsk Medical Academy MoH of Ukraine», Dnipro, Ukraine
CI «Dnipropetrovsk Children's City Hospital No.2», Dnipro, Ukraine
The article describes a clinical case of pseudo-Bartter's syndrome (PBS) in a 5-month-old child with cystic fibrosis. Pseudo-Bartter's syndrome is one of the major complications of cystic fibrosis with potential lethal outcome, which develops in patients in consequence of salt losing in the excessive sweating in hot climate conditions, in infants with an imperfect thermal control system associated with febrile states, alongside non-compliance with recommendations for additional sodium and chlorine salt supply and correction of water load, and can be enhanced by electrolyte spillage in vomiting and diarrhoea. PBS manifests predominantly during the first year of life and is characterised by hypokalaemia, hypochloraemia, metabolic alkalosis, dehydration, plasma renin overactivity, increased high blood aldosterone concentration. The clinical picture is so much non-specific that with no diagnosis of cystic fibrosis, the identification of hypochloremic alkalosis is significantly complicated, and untimely emergency medical treatment quickly leads to death. Awareness of physicians, electrolyte level control, training of parents to recognize the first alarm signs, to carry out preventive measures in situations that boost the metabolic disorders, allows providing adequate correction in due time and prevent the development of life-threatening conditions.
Key words: cystic fibrosis, metabolic alkalosis, pseudo-Bartter's syndrome.
1. Asherova IK, Kapranov NI. (2012). Electrolyte disorders in cystic fibrosis patients. Journal of New Medical Technologies. 12;2 : 96–98.
2. Voronkova AYu, Sherman VD. (2014). Case of late diagnosed Pseudo-Bartter’s syndrome in a 6-months-old baby with cystic fibrosis. Pediatria. Journal named after G.N. Speransky. 4: 172–174.
3. Ledneva VS, Ulyanova LV, Neretina AF. (2011). Experience in diagnosing and treating Рseudo-Bartters syndrome in children with cystic fibrosis. Rossiiskii vestnik perinatologii i pediatrii. 5: 26 – 29.
4. Dahabreh MM, Najada AS. (2013). Pseudo-bartter syndrome, pattern and correlation with other cystic fibrosis features. Saudi J Kidney Dis Transpl. l. 24;2: 292—296. doi 10.4103/1319-2442.109579.
5. Galaviz-Ballesteros MJ, Acosta-Rodriguez-Bueno CP, Consuelo-Sanchez A et al. (2016). Pseudo-Bartter syndrome as manifestation of cystic fibrosis with DF508 mutation. Bol Med Hosp Infant Mex. 73; 5: 331—334.
6. Hizal MG, Ciki K, Esref S et al. (2017). Clinical features of pseudo-bartter syndrome in cystic fibrosis. European Respiratory Journal. 50; 61: 1343. doi 10.1183/1393003.congress-2017.PA1343.
7. Kintu B, Brightwell A. (2014). Episodic Seasonal Pseudo-Bartter Syndrome in Cystic Fibrosis. Paediatric Respiratory Reviews. 15; 1: 19—21. https://doi.org/10.1016/j.prrv.2014.04.015.
8. Ozdogan S, Atli B, Kizilkan NU. (2014). Pseudo-bartter Syndrome in an Infant. Case Study and Case Report. 4; 2: 49—53.
9. Vilotijevic-Dautovic G, Stojanovi V. (2015). Pseudo-Bartter's Syndrome in Patients with Cystic Fibrosis: A Case Series and Review of the Literature. Srp Arh Celok Lek. 143 (11—12): 748—751.
Article received: Feb 22, 2018. Accepted for publication: Aug 26, 2018.