Osteoporosis in children with severe congenital neutropenia: Bone mineral density and treatment with bisphosphonates

dc.contributor.authorBorzutzky, Arturo
dc.contributor.authorReyes, Maria Loreto
dc.contributor.authorFigueroa, Valeria
dc.contributor.authorGarcia, Cristian
dc.contributor.authorCavieres, Mirta
dc.date.accessioned2024-01-10T13:51:40Z
dc.date.available2024-01-10T13:51:40Z
dc.date.issued2006
dc.description.abstractA high incidence of decreased bone mineral density (BMD) has been described in patients with severe congenital neutropenia (SCN). The objectives of the study are to describe changes in BMD in children with SCN treated with granulocyte colony-stimulating factor and evaluate the response to treatment with bisphosphonates in those two had osteoporosis. A prospective open-label study was performed evaluating BMD and metabolism in 9 Chilean patients with SCN, administrating bisphosphonates in those with osteoporosis. Follow-up ranged between 7 months and 3.5 years. Six out of 9 patients had reduced BMD on initial assessment: 3 had osteoporosis (z score < - 2) and 3, had osteopenia (z score < - 1). Four children presented vertebral fractures. Two presented osteopenia on follow-up without Clinical Symptoms. Five patients were treated with biphosphonates, increasing their BMD z Score (mean increase 1.2. range 0.27 to 2.62). z Score of hydroxyproline/ creatinine ratios, which was elevated in 4 patients with osteoperosis, decreases during treatment (mean decrease 2.18, range 1.56 to 2.53). Four patients remodeled and reexpanded fractured vertebrae during treatment. No side effects of bisphosphonate were seen on follow-up. Osteoporosis is an important comorbidity in SCN patients probably due to an increased bone resorption. Bisphosphonates seem to be an effective treatment for osteoporosis in these patients.
dc.fechaingreso.objetodigital2024-05-14
dc.format.extent5 páginas
dc.fuente.origenWOS
dc.identifier.doi10.1097/01.mph.0000210409.48877.c3
dc.identifier.issn1077-4114
dc.identifier.pubmedidMEDLINE:16679916
dc.identifier.urihttps://doi.org/10.1097/01.mph.0000210409.48877.c3
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/79617
dc.identifier.wosidWOS:000237535600003
dc.information.autorucMedicina;Garcia C;S/I;99686
dc.information.autorucMedicina;Reyes ML;S/I;100324
dc.issue.numero4
dc.language.isoen
dc.nota.accesoSin adjunto
dc.pagina.final209
dc.pagina.inicio205
dc.publisherLIPPINCOTT WILLIAMS & WILKINS
dc.revistaJOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
dc.rightsregistro bibliográfico
dc.subjectsevere congenital neutropenia
dc.subjectosteoporosis
dc.subjectbisphosphonates
dc.subjectbone metabolism
dc.subjectKostmann syndrome
dc.subjectCOLONY-STIMULATING FACTOR
dc.subjectKOSTMANN-SYNDROME
dc.subjectNORMAL VALUES
dc.subjectCHILDHOOD
dc.subjectTURNOVER
dc.subjectMICE
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titleOsteoporosis in children with severe congenital neutropenia: Bone mineral density and treatment with bisphosphonates
dc.typeartículo
dc.volumen28
sipa.codpersvinculados99686
sipa.codpersvinculados100324
sipa.indexWOS
sipa.indexScopus
sipa.trazabilidadCarga SIPA;09-01-2024
Files
Original bundle
Now showing 1 - 1 of 1
Loading...
Thumbnail Image
Name:
Osteoporosis in Children With Severe Congenital Neutropenia Bone Mineral Density and Treatment With Bisphosphonates.pdf
Size:
106.54 KB
Format:
Adobe Portable Document Format
Description: