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1.
J. vasc. bras ; 7(4): 389-392, dez. 2008. ilus
Article Dans Anglais, Portugais | LILACS | ID: lil-506112

Résumé

Os sangramentos no retroperitônio são, em sua grande maioria, secundários a eventos traumáticos envolvendo grande energia cinética, com poucos relatos na literatura caracterizados como espontâneos. No presente relato, descrevemos paciente gestante, portadora de doença de Von Recklinghausen e com volumoso hematoma retroperitoneal diagnosticado durante o parto cesariano, secundário a ruptura espontânea de artéria lombar. A doença de Von Recklinghausen apresenta manifestações vasculares bem descritas, caracterizando-se principalmente por estenoses que são secundárias a tumores intramurais (proliferação das células de Schwann) e raramente dilatações aneurismáticas, assintomáticas em sua maioria. No presente caso, foi realizada a aortografia com cateterização seletiva e embolização da artéria sangrante com sucesso.


Retroperitoneal bleeding is mainly due to traumatic events with a high amount of kinetic energy, with few reported cases of spontaneous events in the literature. We report on a case of a pregnant woman with Von Recklinghausen"s disease and bulky retroperitoneal hematoma diagnosed during cesarean delivery secondary to spontaneous lumbar artery rupture. Von Recklinghausen"s disease has well-described vascular manifestations, mainly characterized by stenoses related to intramural tumors (Schwann cell proliferation) and rarely asymptomatic aneurysmal dilatations. In this case, aortography was performed with successful selective catheterization and embolization of the bleeding artery.


Sujets)
Humains , Femelle , Adulte , Embolisation thérapeutique/méthodes , Embolisation thérapeutique , Faux anévrisme/complications , Faux anévrisme/sang , Hématome/complications
2.
Arq. bras. endocrinol. metab ; 52(1): 131-133, fev. 2008. ilus
Article Dans Portugais | LILACS | ID: lil-477443

Résumé

A neurofibromatose tipo 1 (NF1), também conhecida como doença de von Recklinghausen, é uma doença autossômica dominante com alto grau de variabilidade da expressão clínica, comumente envolvida na formação de tumorações na maioria das vezes de origem benigna, localizadas principalmente na região da cabeça e do pescoço, sendo a tireóide acometida raramente. Porém existe na literatura a associação com carcinoma medular da tireóide (CMT), necessitando sempre sua exclusão. Relatamos o caso de uma paciente com NF1, com um nódulo de tireóide não-funcionante e sintomas obstrutivos. Foi realizada ressecção cirúrgica da lesão, com achados histopatológicos compatíveis com neurofibroma em tecido tireoidiano. A importância desse caso deve-se não só à raridade dessa apresentação da NF1, mas também à possibilidade de associação desta com CMT, tumor agressivo com possibilidade de cura pela ressecção cirúrgica.


The neurofibromatosis type 1 (NF1), also known as von Recklinghausen’s disease, is an autosomal dominant disorder, with high degree of variability of clinical expression, usually involved with formation of tumors, with benign origin in the majority of cases mainly localized in the region of the head and neck and rarely incident in the thyroid area. However, the association with medullary carcinoma of the thyroid (MCT) exists in literature and needs to be excluded. We report a case of a patient with NF1, nonfunctional thyroid nodule and obstructive symptoms. Surgical resection of lesion was performed, with histopathologic findings compatible with neurofibroma in thyroid tissue. This case is relevant not only because of the rarity of the presentation of NF1, but also due to the likely association with MCT, an aggressive tumor that can be cured by surgery.


Sujets)
Adulte , Femelle , Humains , Carcinome médullaire/anatomopathologie , Neurofibrome/anatomopathologie , Neurofibromatose de type 1/anatomopathologie , Glande thyroide/anatomopathologie , Tumeurs de la thyroïde/anatomopathologie , Biopsie , Carcinome médullaire/chirurgie , Diagnostic différentiel , Neurofibrome/chirurgie , Neurofibromatose de type 1/chirurgie , Glande thyroide/chirurgie , Tumeurs de la thyroïde/chirurgie , Nodule thyroïdien/anatomopathologie
3.
Clinics ; 63(1): 39-42, 2008. ilus, tab
Article Dans Anglais | LILACS | ID: lil-474926

Résumé

OBJECTIVE: The aim of this study was to investigate the presence of growth hormone receptor in plexiform neurofibromas of neurofibromatosis type 1 patients. INTRODUCTION: The development of multiple neurofibromas is one of the major features of neurofibromatosis type 1. Since neurofibromas commonly grow during periods of hormonal change, especially during puberty and pregnancy, it has been suggested that hormones may influence neurofibromatosis type 1 neurofibromas. A recent study showed that the majority of localized neurofibromas from neurofibromatosis type 1 patients have growth hormone receptor. METHODS: Growth hormone receptor expression was investigated in 5 plexiform neurofibromas using immunohistochemistry. RESULTS: Four of the 5 plexiform neurofibromas were immunopositive for growth hormone receptor. CONCLUSION: This study suggests that growth hormone may influence the development of plexiform neurofibromas in patients with neurofibromatosis type 1.


Sujets)
Adolescent , Adulte , Enfant , Femelle , Humains , Neurofibrome plexiforme/composition chimique , Neurofibromatose de type 1/métabolisme , Récepteur STH/analyse , Marqueurs biologiques tumoraux/analyse , Immunohistochimie , Neurofibrome plexiforme/étiologie , Neurofibromatose de type 1/complications
4.
Korean Journal of Dermatology ; : 1179-1183, 1995.
Article Dans Coréen | WPRIM | ID: wpr-42793

Résumé

Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.


Sujets)
Humains , Mâle , Jeune adulte , Taches café-au-lait , Malformations , Hypertrichose , Mélanose , Neurofibres , Neurofibrome plexiforme , Neurofibromatoses , Neurofibromatose de type 1 , Pigmentation , Scoliose , Rachis
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