POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) syndrome: a South America's report
Arq. neuropsiquiatr
;
65(2b): 516-520, jun. 2007. ilus
Article
Dans Anglais
| LILACS
| ID: lil-456864
ABSTRACT
The POEMS syndrome, also known as Crow-Fukase syndrome, is an unusual systemic disorder described mainly in Asian individuals. It is characterized by the presence of (P)polyneuropathy, (O)organomegaly, (E)endocrinopathy, (M) M-protein, and (S) skin changes. Several other associated condictions such as sclerotic bone lesions, Castleman disease, low-grade fever, edema and hematologic disorders are usually seen. We describe five Brazilian patients with this syndrome. Two patients presented Castleman disease, one patient presented osteosclerotic myeloma and in two patients no associated conditions were found.
RESUMO
A síndrome POEMS, também conhecida como síndrome de Crow-Fukase é uma desordem sistêmica rara descrita principalmente em asiáticos. Ela é caracterizada pela presença de (P) polineuropatia, (O) organomegalia, (E) endocrinopatia, (M) proteína M e (S) alterações de pele. Diversas outras manifestações, tais como lesões osteoescleróticas, doença de Castleman, febre baixa, edema e distúrbios hematológicos são freqüentemente observados. Apresentamos cinco pacientes brasileiros com esta síndrome. Dois pacientes apresentaram diagnóstico de doença de Castleman, um paciente com mieloma osteoesclerótico e em dois pacientes, nenhuma condição associada foi encontrada.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Syndrome POEMS
Type d'étude:
Etude diagnostique
Limites du sujet:
Adulte
/
Humains
/
Mâle
langue:
Anglais
Texte intégral:
Arq. neuropsiquiatr
Thème du journal:
Neurologie
/
Psychiatrie
Année:
2007
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
FIOCRUZ/BR
/
Fluminense Federal University/BR
/
National Institute of Cancer/BR
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