N-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorGUIMARAES, Luciana Pache de Faria
dc.contributor.authorSEGURO, Antonio Carlos
dc.contributor.authorSHIMIZU, Maria Heloisa Mazzola
dc.contributor.authorNERI, Leticia Aparecida Lopes
dc.contributor.authorSUMITA, Nairo Massakasu
dc.contributor.authorBRAGANCA, Ana Carolina de
dc.contributor.authorVOLPINI, Rildo Aparecido
dc.contributor.authorSANCHES, Talita Rojas Cunha
dc.contributor.authorFONSECA, Fernanda Andrade Macaferri da
dc.contributor.authorMOREIRA FILHO, Carlos Alberto
dc.contributor.authorVAISBICH, Maria Helena
dc.date.accessioned2014-09-30T14:43:57Z
dc.date.available2014-09-30T14:43:57Z
dc.date.issued2014
dc.description.abstractNephropathic cystinosis is an autosomal recessive systemic severe disease characterized by intralysosomal cystine storage. Cysteamine is an essential component of treatment. There is solid evidence that cystine accumulation itself is not responsible for all abnormalities in cystinosis; there is also a deficiency of glutathione in the cytosol. Patients with cystinosis can be more susceptible to oxidative stress. The patient cohort comprised 23 cystinosis patients (16 males) aged < 18 years (mean age 8.0 +/- 3.6 years) with chronic kidney disease class I-IV with good adherence to treatment, including cysteamine. Oxidative stress was evaluated based on the levels of serum thiobarbituric acid-reactive substances (TBARS), and renal function was evaluated based on serum creatinine and cystatin C levels and creatinine clearance (Schwartz formula). N-Acetylcysteine (NAC), an antioxidant drug was given to all patients for 3 months (T1) at 25 mg/kg/day divided in three doses per day. The measured values at just before the initiation of NAC treatment (T0) served as the control for each patient. Median serum TBARS levels at T0 and T1 were 6.92 (range 3.3-29.0) and 1.7 (0.6-7.2) nmol/mL, respectively (p < 0.0001). In terms of renal function at T0 and T1, serum creatinine levels (1.1 +/- 0.5 vs. 0.9 +/- 0.5 mg/dL, respectively; p < 0.0001), creatinine clearance (69.7 +/- 32.2 vs. T1 = 78.5 +/- 33.9 mL/min/1.73 m(2), respectively; p = 0.006), and cystatin c level (1.33 +/- 0.53 vs. 1.15 +/- 0.54 mg/l, respectively; p = 0.0057) were all significantly different at these two time points. Serum creatinine measurements at 6 (T -6) and 3 months (T -3) before NAC initiation and at 3 (T +3) and 6 months (T +6) after NAC had been withdrawn were also evaluated. During the 3-month period that our 23 cystinosis patients were treated with NAC, oxidative stress was reduced and renal function significantly improved. No side-effects were detected. Larger and controlled studies are needed to confirm these findings.
dc.description.indexMEDLINE
dc.description.sponsorshipFundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [2010/10622-0]
dc.identifier.citationPEDIATRIC NEPHROLOGY, v.29, n.6, p.1097-1102, 2014
dc.identifier.doi10.1007/s00467-013-2705-3
dc.identifier.eissn1432-198X
dc.identifier.issn0931-041X
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/7627
dc.language.isoeng
dc.publisherSPRINGER
dc.relation.ispartofPediatric Nephrology
dc.rightsrestrictedAccess
dc.rights.holderCopyright SPRINGER
dc.subjectNephropathic cystinosis
dc.subjectOxidative stress
dc.subjectGlomerular filtration rate
dc.subjectN-Acetylcysteine
dc.subjectCysteamine
dc.subject.otherinduced nephrotoxicity
dc.subject.othercysteamine therapy
dc.subject.otheracetylcysteine
dc.subject.othertransplantation
dc.subject.otherchildren
dc.subject.otherglutathione
dc.subject.otheradolescents
dc.subject.otherprogression
dc.subject.othermechanisms
dc.subject.otheroutcomes
dc.subject.wosPediatrics
dc.subject.wosUrology & Nephrology
dc.titleN-acetyl-cysteine is associated to renal function improvement in patients with nephropathic cystinosis
dc.typearticle
dc.type.categoryoriginal article
dc.type.versionpublishedVersion
dspace.entity.typePublication
hcfmusp.citation.scopus16
hcfmusp.contributor.author-fmusphcLUCIANA PACHE DE FARIA GUIMARAES
hcfmusp.contributor.author-fmusphcANTONIO CARLOS SEGURO
hcfmusp.contributor.author-fmusphcMARIA HELOISA MASSOLA SHIMIZU
hcfmusp.contributor.author-fmusphcLETICIA APARECIDA LOPES NERI
hcfmusp.contributor.author-fmusphcNAIRO MASSAKAZU SUMITA
hcfmusp.contributor.author-fmusphcANA CAROLINA DE BRAGANCA VICIANA
hcfmusp.contributor.author-fmusphcRILDO APARECIDO VOLPINI
hcfmusp.contributor.author-fmusphcTALITA ROJAS CUNHA SANCHES
hcfmusp.contributor.author-fmusphcFERNANDA ANDRADE MACAFERRI DA FONSECA NUNES
hcfmusp.contributor.author-fmusphcCARLOS ALBERTO MOREIRA FILHO
hcfmusp.contributor.author-fmusphcMARIA HELENA VAISBICH
hcfmusp.description.beginpage1097
hcfmusp.description.endpage1102
hcfmusp.description.issue6
hcfmusp.description.volume29
hcfmusp.origemWOS
hcfmusp.origem.pubmed24326786
hcfmusp.origem.scopus2-s2.0-84901587201
hcfmusp.origem.wosWOS:000335158000020
hcfmusp.publisher.cityNEW YORK
hcfmusp.publisher.countryUSA
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hcfmusp.remissive.sponsorshipFAPESP
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