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2.
Clin Rheumatol ; 28(10): 1229-33, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19455364

RESUMO

Primary hypertrophic osteoarthropathy, or pachydermoperiostosis (PDP), is an infrequent genetic condition characterized by digital clubbing, periostosis, and pachydermia and is distinct from a more common form, secondary hypertrophic osteoarthropathy, which always associates with an underlying cause (frequently pulmonary or cardiac disease). The diagnosis of this disorder as well as its clinical evaluation can be difficult. We report a 15-year-old boy presenting with intermittent arthralgias and clubbing of fingers and toes for the previous 2 years. The ankles and knees were enlarged, and X-rays showed periosteal apposition. The search for a secondary cause was negative. The skin appearance was normal, but a skin biopsy was indicative of pachydermia, further confirming the diagnosis of PDP. Bone turnover markers were increased at diagnosis and progressively decreased during follow-up; prostaglandin E(2), a recently implicated mediator of this disorder, was markedly elevated. In the present case, carrying out a skin biopsy helped us to diagnose this condition. In addition, bone turnover markers were useful for monitoring the disease activity; whereas, increased prostaglandin E(2) levels seems to confirm the role of this mediator in the etiopathogenesis of this disorder.


Assuntos
Osso e Ossos/metabolismo , Dinoprostona/sangue , Osteoartropatia Hipertrófica Primária/diagnóstico , Osteoartropatia Hipertrófica Primária/metabolismo , Adolescente , Fosfatase Alcalina/sangue , Biomarcadores/sangue , Biópsia , Reabsorção Óssea/fisiopatologia , Colágeno Tipo I/sangue , Humanos , Masculino , Osteoartropatia Hipertrófica Primária/fisiopatologia , Osteocalcina/sangue , Osteogênese/fisiologia , Fragmentos de Peptídeos/sangue , Peptídeos/sangue , Pró-Colágeno/sangue , Pele/patologia
3.
Acta otorrinolaringol. esp ; 51(6): 539-542, ago. 2000. ilus
Artigo em Es | IBECS | ID: ibc-8018

RESUMO

Las lesiones congénitas benignas son el resultado de una regresión defectuosa del tejido neuroglial, se conocen con el nombre de gliomas nasofrontales y suelen diagnosticarse en el período neonatal. Forman parte del diagnóstico diferencial de las masas nasofrontales de la línea media. Presentamos el caso de una paciente de 62 años en estudio por masa de aspecto poliposo en fosa nasal izquierda. Mediante la TAC se demuestra ocupación del receso etmoido-esfenoidal izquierdo y coana. Presentamos este caso por lo infrecuente del hallazgo en un adulto y por la importancia del diagnóstico diferencial, realizando una revisión de los gliomas craneofaciales (AU)


Benign congenital lesions resulting from the deficient regression of neuroglial tissue in normal embryonic development are called gliomas. They are usually located in the nasofrontal region and are diagnosed in the postnatal period. They are included in the differential diagnosis of nasofrontal midline masses. We review the case of a 62 year-old woman who was originally studied for a polypoid mass in the left nasal cavity. CT revealed occupation of the left ethmoidofrontal recess and turbinate by a glioma. We report this case as a rare finding in a 62 year-old woman because of its importance in differential diagnosis. We reviewed craniofacial gliomas (AU)


Assuntos
Pessoa de Meia-Idade , Feminino , Humanos , Seio Esfenoidal/patologia , Glioma/diagnóstico , Neoplasias dos Seios Paranasais/diagnóstico , Tomografia Computadorizada por Raios X , Imageamento por Ressonância Magnética , Glioma/patologia , Neoplasias dos Seios Paranasais/patologia
4.
Acta otorrinolaringol. esp ; 51(5): 457-459, jun. 2000. ilus
Artigo em Es | IBECS | ID: ibc-8046

RESUMO

Se presenta un caso de tumorfibroso solitario (TFS) de localización parafaríngea, con un cuadro clínico de afectación locorregional (hipoacusia, obstrucción nasal y parálisis velopalatina y de hemilengua). Se señala que el TFS, descrito inicialmente a nivel pleural y de origen mesotelial, hoy día se reconoce en otras múltiples localizaciones con un origen mesenquimal. En la región de cabeza y cuello se han descrito unos 50 casos y el presente es el quinto caso publicado a nivel parafaríngeo, habiéndose confirmado el diagnóstico mediante marcadores inmunohistoquímicos (vimentina, CD34 y CD99 positivos) y ultraestructurales (hábito fibroblástico) (AU)


A solitary fibrous tumor (SFT) of the parapharyngeal space presented with local symptoms (hearing loss, nasal obstruction, and paralysis of the soft palate and tongue). SFT, originally described as a mesothelial tumor of the pleura, now is recognized as a mesenchymal tumor that occurs in different locations. In the head and neck region, about 50 cases have been reported. This is the fifth published report of an SFT of the parapharyngeal space. The diagnosis was confirmed by immunohistochemical (positivity for vimentin, CD34, and CD99) and ultrastructural markers (fibroblastic characteristics) (AU)


Assuntos
Idoso , Masculino , Humanos , Mesotelioma/patologia , Neovascularização Patológica , Neoplasias Faríngeas , Vimentina/metabolismo , Antígenos CD34/metabolismo , Angiografia Cerebral , Imuno-Histoquímica
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