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| Name | Class |
|---|---|
| Fundação de Amparo à Pesquisa do Estado de São Paulo | OTHER_GOV |
This study intends to verify the interference of N acetyl cysteine in the progression of chronic kidney disease in patients with Nephropathic Cystinosis.
Patients with Nephropathic Cystinosis have an increased oxidative stress and go to end-stage renal disease, even when all steps of the treatment are done. Therefore, this study is conducted to verify the interference of the stress oxidative in the progression of the renal disease with the use of an oxidant drug, N acetyl cysteine (NAC). The investigators selected patients with good compliance of the treatment. In these patients the investigators evaluate the serum creatinine, creatinine clearance estimated by Schwartz Formula, cystatin C and a marker of oxidative stress, TBARS ( thiobarbituric acid substances). The variables are analyzed 6 months before the introduction of NAC and the three months after NAC.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| N acetyl cysteine | Experimental | NAC will be given to cystinosis patients and we will observe the renal function status and a marker of oxidative stress (TBARS) |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| N acetyl cysteine | Drug | N acetyl cysteine to cystinosis patients with CKD stages 1 to 4 |
|
| Measure | Description | Time Frame |
|---|---|---|
| creatinine clearance | creatinine clearance will be evaluate 6 months beforw and at baseline and after 3 months with NAC | change in creatinine clearance evaluated 6 months before , at baseline and after 3 months with NAC |
| cystatin c | Cystinosis patients will be evaluated 6 months before and at baseline and after 3 months after the use of NAC | change in cystatin C from baseline and after 3 months with NAC |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Maria Helena Vaisbich, Doctor | University of Sao Paulo | Principal Investigator |
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| ID | Term |
|---|---|
| D007674 | Kidney Diseases |
| D003554 | Cystinosis |
| ID | Term |
|---|---|
| D014570 | Urologic Diseases |
| D052776 | Female Urogenital Diseases |
| D005261 | Female Urogenital Diseases and Pregnancy Complications |
| D000091642 | Urogenital Diseases |
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| ID | Term |
|---|---|
| D000111 | Acetylcysteine |
| ID | Term |
|---|---|
| D003545 | Cysteine |
| D000603 | Amino Acids, Sulfur |
| D013457 | Sulfur Compounds |
| D009930 | Organic Chemicals |
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| N acetyl cysteine | Drug | N acetyl cysteine for cystinosis patients CKD stages 1 to 4 |
|
| D052801 | Male Urogenital Diseases |
| D016464 | Lysosomal Storage Diseases |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D000596 |
| Amino Acids |
| D000602 | Amino Acids, Peptides, and Proteins |