Assuntos
Educação Médica/história , Endocrinologia/história , Ginecologia/história , Hospitais Universitários/história , Pesquisa Biomédica/educação , Pesquisa Biomédica/história , Endocrinologia/educação , França , Ginecologia/educação , História do Século XX , História do Século XXI , Hospitais Universitários/normas , HumanosRESUMO
We describe the case of a 20-year-old patient with salt-wasting congenital adrenal hyperplasia (CAH) related to 21-hydroxylase deficiency. Bilateral craggy testicular tumours were found, requiring histological evaluation. Prior to the surgical procedure, the patient was treated with dexamethasone (he presented cortisol deficiency) and was stimulated with ACTH. High levels of 11beta-OH steroids measured in the gonadal vein, compared with peripheral blood samples suggested the presence of adrenal rests. Incubation of the tumours (which could not be differentiated histologically, from Leydig tissue), with radioactive steroid precursors was carried out. The results revealed the testicular tumours were of adrenal tissue origin, associated with 21-hydroxylase deficiency. The patient's non-compliance to glucocorticoid treatment was the main cause of his hypogonadotropic hypogonadism.
Assuntos
Hiperplasia Suprarrenal Congênita/complicações , Tumor de Resto Suprarrenal/etiologia , Esteroide 21-Hidroxilase/metabolismo , Neoplasias Testiculares/etiologia , Tumor de Resto Suprarrenal/diagnóstico , Tumor de Resto Suprarrenal/diagnóstico por imagem , Tumor de Resto Suprarrenal/tratamento farmacológico , Tumor de Resto Suprarrenal/patologia , Tumor de Resto Suprarrenal/cirurgia , Hormônio Adrenocorticotrópico/farmacologia , Adulto , Sulfato de Desidroepiandrosterona/sangue , Dexametasona/uso terapêutico , Diagnóstico Diferencial , Fludrocortisona/uso terapêutico , Hormônio Foliculoestimulante/sangue , Seguimentos , Glucocorticoides/uso terapêutico , Hormônio Liberador de Gonadotropina/metabolismo , Humanos , Hidrocortisona/deficiência , Hipogonadismo/etiologia , Inibinas/sangue , Tumor de Células de Leydig/diagnóstico , Hormônio Luteinizante/sangue , Masculino , Prolactina/sangue , Renina/sangue , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/diagnóstico por imagem , Neoplasias Testiculares/tratamento farmacológico , Neoplasias Testiculares/patologia , Neoplasias Testiculares/cirurgia , Recusa do Paciente ao Tratamento , UltrassonografiaRESUMO
Hodgkin's disease appearing as, or associated with, fibrous thyroiditis has only been described rarely. We report the observation of a patient presenting with a goitre, fibrosis of the thyroid and adjacent structures, and hypothyroidism. The histological examination was compatible with fibrosclerotic thyroiditis. This diagnosis was reviewed 6 months later when the biopsy of a supraclavicular nodule that had subsequently appeared led to the diagnosis of a nodular-sclerosis type of Hodgkin's disease. The plasmatic levels of interleukin 6 (IL-6) and tumour necrosis factor alpha (TNF-alpha) were very high compared to the levels in healthy subjects (12 and 40 IU/l vs. 0.05 and 2.0 IU/l, respectively). These cytokine levels decreased when the initial illness was treated, and their normalization was associated with the disappearance of the cervical and thyroidal fibroses. A parallel in vitro study of these cytokines and of TNF-alpha receptors and IL-13 was performed. The results suggest a possible cause-and-effect relationship between IL-6 and IL-13 produced locally by the tumoral tissue and the development of cervical fibrosis.
