Treatment of Bartter syndrome. Unsolved issue

Objective: To describe the results of a long‐term follow‐up of Bartter syndrome patients treated with different drugs. Method: Patients were diagnosed according to clinical and laboratory data. Treatment protocol was potassium supplementation, sodium, spironolactone, and non‐steroidal anti‐inflammat...

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Main Authors: Carla Lessa Pena Nascimento, Cecilia Lopes Garcia, Benita Galassi Soares Schvartsman, Maria Helena Vaisbich
Format: Article
Language:Portuguese
Published: Brazilian Society of Pediatrics 2014-09-01
Series:Jornal de Pediatria (Versão em Português)
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2255553614001062
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author Carla Lessa Pena Nascimento
Cecilia Lopes Garcia
Benita Galassi Soares Schvartsman
Maria Helena Vaisbich
author_facet Carla Lessa Pena Nascimento
Cecilia Lopes Garcia
Benita Galassi Soares Schvartsman
Maria Helena Vaisbich
author_sort Carla Lessa Pena Nascimento
collection DOAJ
description Objective: To describe the results of a long‐term follow‐up of Bartter syndrome patients treated with different drugs. Method: Patients were diagnosed according to clinical and laboratory data. Treatment protocol was potassium supplementation, sodium, spironolactone, and non‐steroidal anti‐inflammatory drug. Patients who developed proteinuria were converted to angiotensin conversion enzyme inhibitor. The variables evaluated for each drug were Z‐score for weight and stature, proteinuria, creatinine clearance, gastrointestinal complaints, amount of potassium supplementation, serum potassium and bicarbonate levels, and findings of upper digestive endoscopy. Results: 20 patients were included. Follow‐up was 10.1 ± 5.2 years. 17 patients received indomethacin for 5.9 ± 5.3 years; 19 received celecoxib, median of 35 months; and five received enalapril, median of 23 months. During indomethacin, a statistically significant increase was observed in the Z‐score for stature and weight, without a change in the creatinine clearance. Seven of 17 patients had gastrointestinal symptoms, and upper digestive endoscopy evidenced gastritis in three patients and gastric ulcer in four patients. During celecoxib use, a significant increase was detected in the Z‐score for stature and weight and a reduction of hyperfiltration; seven patients presented gastrointestinal symptoms, and upper digestive endoscopy evidenced mild gastritis in three. During enalapril use, no significant changes were observed in the Z‐score for stature, weight and creatinine clearance. The conversion to enalapril resulted in a significant reduction in proteinuria. Conclusion: The authors suggest starting the treatment with celecoxib, and replacing by ACEi if necessary, monitoring the renal function. The safety and efficacy of celecoxib need to be assessed in larger controlled studies.
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spelling doaj.art-2ca6f08517ae4c2e8ef7ff1c738ef5f52022-12-22T03:56:12ZporBrazilian Society of PediatricsJornal de Pediatria (Versão em Português)2255-55362014-09-0190551251710.1016/j.jpedp.2014.01.009Treatment of Bartter syndrome. Unsolved issueCarla Lessa Pena Nascimento0Cecilia Lopes Garcia1Benita Galassi Soares Schvartsman2Maria Helena Vaisbich3Unidade de Nefrologia, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, BrasilUnidade de Nefrologia, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, BrasilUnidade de Nefrologia, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, BrasilUnidade de Nefrologia, Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, BrasilObjective: To describe the results of a long‐term follow‐up of Bartter syndrome patients treated with different drugs. Method: Patients were diagnosed according to clinical and laboratory data. Treatment protocol was potassium supplementation, sodium, spironolactone, and non‐steroidal anti‐inflammatory drug. Patients who developed proteinuria were converted to angiotensin conversion enzyme inhibitor. The variables evaluated for each drug were Z‐score for weight and stature, proteinuria, creatinine clearance, gastrointestinal complaints, amount of potassium supplementation, serum potassium and bicarbonate levels, and findings of upper digestive endoscopy. Results: 20 patients were included. Follow‐up was 10.1 ± 5.2 years. 17 patients received indomethacin for 5.9 ± 5.3 years; 19 received celecoxib, median of 35 months; and five received enalapril, median of 23 months. During indomethacin, a statistically significant increase was observed in the Z‐score for stature and weight, without a change in the creatinine clearance. Seven of 17 patients had gastrointestinal symptoms, and upper digestive endoscopy evidenced gastritis in three patients and gastric ulcer in four patients. During celecoxib use, a significant increase was detected in the Z‐score for stature and weight and a reduction of hyperfiltration; seven patients presented gastrointestinal symptoms, and upper digestive endoscopy evidenced mild gastritis in three. During enalapril use, no significant changes were observed in the Z‐score for stature, weight and creatinine clearance. The conversion to enalapril resulted in a significant reduction in proteinuria. Conclusion: The authors suggest starting the treatment with celecoxib, and replacing by ACEi if necessary, monitoring the renal function. The safety and efficacy of celecoxib need to be assessed in larger controlled studies.http://www.sciencedirect.com/science/article/pii/S2255553614001062Bartter syndromeNon‐steroidal anti‐inflammatory drugEnalaprilProteinuria
spellingShingle Carla Lessa Pena Nascimento
Cecilia Lopes Garcia
Benita Galassi Soares Schvartsman
Maria Helena Vaisbich
Treatment of Bartter syndrome. Unsolved issue
Jornal de Pediatria (Versão em Português)
Bartter syndrome
Non‐steroidal anti‐inflammatory drug
Enalapril
Proteinuria
title Treatment of Bartter syndrome. Unsolved issue
title_full Treatment of Bartter syndrome. Unsolved issue
title_fullStr Treatment of Bartter syndrome. Unsolved issue
title_full_unstemmed Treatment of Bartter syndrome. Unsolved issue
title_short Treatment of Bartter syndrome. Unsolved issue
title_sort treatment of bartter syndrome unsolved issue
topic Bartter syndrome
Non‐steroidal anti‐inflammatory drug
Enalapril
Proteinuria
url http://www.sciencedirect.com/science/article/pii/S2255553614001062
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