POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin lesions) syndrome: a South America's report
Arq. neuropsiquiatr
; 65(2b): 516-520, jun. 2007. ilus
Article
in English
| LILACS
| ID: lil-456864
Responsible library:
BR1.1
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.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
POEMS Syndrome
Type of study:
Diagnostic study
Limits:
Adult
/
Humans
/
Male
Language:
English
Journal:
Arq. neuropsiquiatr
Journal subject:
Neurology
/
Psychiatry
Year:
2007
Document type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
FIOCRUZ/BR
/
Fluminense Federal University/BR
/
National Institute of Cancer/BR