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Titre du document / Document title

Fibromatosis hialina juvenil = A case of juvenile hyaline fibromatosis

Auteur(s) / Author(s)

GOMEZ-VALENCIA Luis (1) ; ULLOA-PATINO Patricia (2) ; BULNES-MENDIZABAL David Pablo (2) ; MADRINAN-RIVAS Jorge Eduardo (3) ; ADEMAR TUYUB-DOMINGUEZ Wilber (3) ; OSUNA-HUERTA Antonio (4) ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

(1) Servicio de Genética, Hospital del Niño Dr. Rodolfo Nieto Padrón, Villahermosa, Tabasco, MEXIQUE
(2) Servicio de Patologia, Hospital del Niño Dr. Rodolfo Nieto Padrón, Villahermosa, Tabasco, MEXIQUE
(3) Servicio de Cirugía Pediátrica, Hospital del Niño Dr. Rodolfo Nieto Padrón, Villahermosa, Tabasco, MEXIQUE
(4) Servicio de Infectologia Pediátrica, Hospital del Niño Dr. Rodolfo Nieto Padrón, Villahermosa, Tabasco, MEXIQUE

Résumé / Abstract

Introduction. Juvenile hyaline fibromatosis (JHF) is a rare, autosomic recessive, connective tissue disorder characterized by multiple subcutaneous nodules, gingival hypertrophy, osteolytic lesions, and articular contractures. Case report. An 8 years old female presented with a history of multiple subcutaneous nodules, articular contractures, and gingival hypertrophy since 2 months of life. Familial history and consanguinity were negative. Tumors, from different regions were resected several times. Dense hyaline fibrous tissue forming irregular bands with extensive amorphous collagen zones, fibroblasts and calcifications were observed in the histopathological study. Conclusion. JHF should be suspected in the first weeks of life, in order to establish an early diagnosis and to offer opportune genetic counseling.

Revue / Journal Title

Boletín Médico del Hospital Infantil de México   ISSN 0539-6115   CODEN BMHIAK 

Source / Source

2001, vol. 58, no2, pp. 107-113 (30 ref.)

Langue / Language

Espagnol

Editeur / Publisher

Ediciones Médicas del Hospital Infantil de México Federico Gómez, México, MEXIQUE  (1944) (Revue)

Mots-clés anglais / English Keywords

Juvenile fibromatosis ; Case study ; Diagnosis ; Child ; Human ; Skin disease ; Systemic disease ; Genetic disease ; Benign neoplasm ; Stomatology ; Diseases of the osteoarticular system ;

Mots-clés français / French Keywords

Fibromatose juvénile ; Etude cas ; Diagnostic ; Enfant ; Homme ; Peau pathologie ; Maladie système ; Maladie héréditaire ; Tumeur bénigne ; Stomatologie ; Système ostéoarticulaire pathologie ;

Mots-clés espagnols / Spanish Keywords

Fibromatosis juvenil ; Estudio caso ; Diagnóstico ; Niño ; Hombre ; Piel patología ; Enfermedad sistémica ; Enfermedad hereditaria ; Tumor benigno ; Estomatología ; Sistema osteoarticular patología ;

Localisation / Location

INIST-CNRS, Cote INIST : 20051, 35400009828451.0040

Nº notice refdoc (ud4) : 999526

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