Distal Tubulopathy. Gitelman Syndrome
Alexander A. Baranov,
Leyla S. Namazova-Baranova,
Tatyana V. Sergeeva,
Olga V. Chumakova,
Svetlana S. Paunova,
Nurali Z. Zokirov,
Olga V. Komarova,
Tea V. Margieva,
Vladimir K. Tatochenko,
Maya D. Bakradze,
Elena N. Tsygina,
Olga I. Zrobok,
Tatiana V. Vashurina,
Irina N. Lupan,
Mikhail Yu. Kagan,
Alexey N. Tsygin https://doi.org/10.15690/pf.v16i3.2025
Abstract
The clinical recommendations on management of children with Gitelman syndrome developed by the experts of the Union of pediatricians of Russia are presented in this article. Gitelman syndrome is a dysfunction of distal renal tubules with further development of hypomagnesemia, hypocalciuria and secondary aldosteronism which determine hypokalemia and metabolic alkalosis. Features of epidemiology, etiology and pathogenesis, disease progression, differential diagnostics and evidence-based treatment are presented.
Supporting agencies: Not specified
About the Authors
Alexander A. Baranov
Central Clinical Hospital of the Russian Academy of Sciences
Russian Federation
Russian Federation
Moscow
Leyla S. Namazova-Baranova
Central Clinical Hospital of the Russian Academy of Sciences; Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
Moscow
Tatyana V. Sergeeva
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Olga V. Chumakova
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Svetlana S. Paunova
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Nurali Z. Zokirov
The Central Pediatric Clinical Hospital of FMBA of Russia
Russian Federation
Russian Federation
Moscow
Olga V. Komarova
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Tea V. Margieva
Central Clinical Hospital of the Russian Academy of Sciences
Russian Federation
Russian Federation
Moscow
Vladimir K. Tatochenko
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Maya D. Bakradze
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Elena N. Tsygina
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Olga I. Zrobok
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Tatiana V. Vashurina
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
Irina N. Lupan
South Ural State Medical University
Russian Federation
Russian Federation
Chelyabinsk
Mikhail Yu. Kagan
Regional Children's Clinical Hospital
Russian Federation
Russian Federation
Orenburg
Alexey N. Tsygin
National Medical Research Center of Children's Health
Russian Federation
Russian Federation
Moscow
References
1. . Detskaya nefrologiya: prakticheskoe rukovodstvo. Ed by E Lojman, AN Tsygin, AA Sarkisyan. Moscow: Litterra; 2010. 400 p. (In Russ).
2. Fremont OT, Chan JC. Understanding Bartter syndrome and Gitelman syndrome. World J Pediatr. 2012;8(1):25–30. doi: 10.1007/s12519-012-0333-9.
3. Knoers NV, Levtchenko EN. Gitelman syndrome. Orphanet J Rare Dis. 2008;3:22. doi: 10.1186/1750-1172-3-22.
Review
For citations:
Baranov A.A., Namazova-Baranova L.S., Sergeeva T.V., Chumakova O.V., Paunova S.S., Zokirov N.Z., Komarova O.V., Margieva T.V., Tatochenko V.K., Bakradze M.D., Tsygina E.N., Zrobok O.I., Vashurina T.V., Lupan I.N., Kagan M.Yu., Tsygin A.N. Distal Tubulopathy. Gitelman Syndrome. Pediatric pharmacology. 2019;16(3):144-148. (In Russ.) https://doi.org/10.15690/pf.v16i3.2025
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