Bladder Dysfunction in Children with Neurofibromatosis Type I: Report of Four Cases and Review of the Literature
| dc.rights.license | open | en_US |
| dc.contributor.author | BOUTY, A. | |
| dc.contributor.author | DOBREMEZ, E. | |
| dc.contributor.author | HARPER, L. | |
| hal.structure.identifier | Bordeaux population health [BPH] | |
| dc.contributor.author | HARAMBAT, Jerome
IDREF: 110567358 | |
| dc.contributor.author | BOUTEILLER, C. | |
| dc.contributor.author | ZAGHET, B. | |
| dc.contributor.author | WOLKENSTEIN, P. | |
| dc.contributor.author | DUCASSOU, S. | |
| dc.contributor.author | LEFEVRE, Y. | |
| dc.date.accessioned | 2020-10-26T14:12:22Z | |
| dc.date.available | 2020-10-26T14:12:22Z | |
| dc.date.issued | 2018 | |
| dc.identifier.issn | 0042-1138 | en_US |
| dc.identifier.uri | https://oskar-bordeaux.fr/handle/20.500.12278/11477 | |
| dc.description.abstractEn | AIM: Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disorder. Malignant transformation into malignant peripheral nerve sheath tumors (MPNST) can occur. However, urinary tract involvement is rare. We report 4 cases of NF1 with bladder dysfunction. METHODS: A retrospective single center analysis of 4 patients was conducted over a 17-year period, focusing on urinary tract involvement. RESULTS: NF1 was diagnosed at a median of 16.5 months (4-36) and urinary involvement occurred at a median of 5.25 years (4-9) after diagnosis. Bladder dysfunction was due to spinal cord compression in 2 cases, bladder invasion in 1 case, and cerebral lesions in 1 case. Malignant transformation of neurofibromas into MPNST occurred in 2 patients. Mechanisms of urinary involvement in NF1 are diverse and no pre-established protocol of management and follow-up exists. CONCLUSION: Although rare, dysfunction of the bladder can arise in NF1 and innovative strategies then need to be considered. This is best achieved with the help of a multidisciplinary team and a national reference center when available. | |
| dc.language.iso | EN | en_US |
| dc.subject.en | LEHA | |
| dc.title.en | Bladder Dysfunction in Children with Neurofibromatosis Type I: Report of Four Cases and Review of the Literature | |
| dc.title.alternative | Urol Int | en_US |
| dc.type | Article de revue | en_US |
| dc.identifier.doi | 10.1159/000487193 | en_US |
| dc.subject.hal | Sciences du Vivant [q-bio]/Santé publique et épidémiologie | en_US |
| dc.identifier.pubmed | 29514154 | en_US |
| bordeaux.journal | Urologia internationalis | en_US |
| bordeaux.page | 339-345 | en_US |
| bordeaux.volume | 100 | en_US |
| bordeaux.hal.laboratories | Bordeaux Population Health Research Center (BPH) - UMR 1219 | en_US |
| bordeaux.issue | 3 | en_US |
| bordeaux.institution | Université de Bordeaux | en_US |
| bordeaux.team | LEHA_BPH | |
| bordeaux.team | LEHA_BPH | |
| bordeaux.peerReviewed | oui | en_US |
| bordeaux.inpress | non | en_US |
| hal.identifier | hal-02978533 | |
| hal.version | 1 | |
| hal.date.transferred | 2020-10-26T14:12:27Z | |
| hal.export | true | |
| bordeaux.COinS | ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Urologia%20internationalis&rft.date=2018&rft.volume=100&rft.issue=3&rft.spage=339-345&rft.epage=339-345&rft.eissn=0042-1138&rft.issn=0042-1138&rft.au=BOUTY,%20A.&DOBREMEZ,%20E.&HARPER,%20L.&HARAMBAT,%20Jerome&BOUTEILLER,%20C.&rft.genre=article |
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