Bilateral testicular adrenal rest tissue in congenital adrenal hyperplasia: US and MR features.

We describe magnetic resonance (MR) and ultrasonography (US) features of bilateral testicular adrenal rest tissue in a 20-yr-old man with congenital adrenal hyperplasia (CAH). Scrotal ultrasonology detected bilateral homogeneous hypoechoic lesions with well-defined margins and without evidence of sound attenuation. MR is useful in defining the size of lesions, because the contrast resolution is better than with sonography and allows an accurate definition of the extent of disease. This case suggests that US evaluation should be included in periodical follow-up of patients with CAH, while MR may be used in the case of rapid increase in the size of the testicular mass.

Development of autoimmune thyroid diseases during long-term treatment of hematological malignancies with alpha-interferons.

In recent years recombinant alpha-interferons (alpha-IFNs) have been widely used in the treatment of several hematological malignancies. Prolonged courses of IFN have been shown to induce autoantibodies and to support the exacerbation or even the development of autoimmune diseases. In this report we describe the development of symptomatic autoimmune thyroid diseases in 4 (7.4%) out of 54 patients in chronic treatment with recombinant alpha-IFNs in our department. Two patients developed a disease resembling Hashimoto's thyroiditis after 17 and 49 months of continuous IFN treatment, while the other two developed a typical Graves' disease after 41 and 52 months of therapy. The mechanism by which IFN induces autoimmune thyroid diseases, the choice of searching for anti-thyroid autoantibodies before starting long-term IFN treatment, the option of discontinuing IFN therapy in the presence of overt thyroid diseases, and the management of these diseases are discussed.

[Autoimmune polyendocrinopathy and chronic mucocutaneous candidiasis]. / Poliendocrinopatia autoimmune e candidiasi mucocutanea cronica.

A chronic mucocutaneous infection due to yeasts to the Candida genus can be part of the autoimmune polyglandular syndrome, described as APECS, APECED, SPA type I, CES. The authors describe the case of a young patient affected by mucocutaneous candidiasis, hypoparathyroidism and adrenal insufficiency. Chronic mucocutaneous candidiasis was diagnosed first in early childhood and was followed by the development of hypoparathyroidism (4 yrs) and adrenal insufficiency (5 yrs). The initial lesion of this syndrome is still unknown as the reason of the marked susceptibility to mucocutaneous candidiasis without systemic candidiasis. The treatment of mucocutaneous candidiasis has been improved by the introduction of orally active antifungal drugs as Ketoconazole, first, and fluconazole now.

Idiopathic anterior hypopituitarism: magnetic resonance imaging and clinical correlation.

We describe the anatomical abnormalities in the hypothalamic-pituitary region revealed by magnetic resonance imaging (MRI) in 10 patients, 8 men and 2 women, affected by idiopathic anterior hypopituitarism (IAH). In all cases contiguous sagittal and coronal T-1 weighted images were obtained. In 9 patients the high-intensity posterior lobe was missing, in almost all patients the pituitary stalk was thin and/or transected, and in 8 a bright T-1 nodule was detected at the infundibular apex, near the tuber cinereum, in the median eminence. None had diabetes insipidus. MRI proves to be very useful in understanding the etiopathogenesis of IAH.

[Mixed gonadal dysgenesis (MGD). Description of a case]. / Disgenesia gonadica mista (DGM). Descrizione di un caso.

The Authors report a case of mixed gonadal dysgenesis (DGM), with a mosaic karyotype consisting of two cell lines, one with 45 chromosomes and monosomic X (50%), the other with 46 chromosomes and one Y dicentric chromosome (50%). The patient presents a male phenotype, hypospadias (3rd degree), left cryptorchidism, hypotrophic right testis, short stature and a gonadal asymmetry.

[Changes in secretion of prolactin in obesity]. / Alterata secrezione della prolattina nella obesità.

The purpose of this study was to evaluate prolactin secretion in obesity. Basal level of serum prolactin and prolactin response after thyrotropin releasing hormone (TRH) were investigated in 20 obese patients and 20 healthy subjects well matched for age and sex. Obese patients had higher basal levels of serum prolactin than those of healthy subjects. Conversely, no significant difference in serum prolactin concentration was found at any time during TRH stimulating test. However obese patients showed incremental areas of serum prolactin slightly, but not significantly, lower compared to healthy subjects, probably because their basal levels were higher. In conclusion, the present study confirms that prolactin secretion is altered in obese patients. The discrepancy between basal levels and prolactin response during TRH stimulating test seems to indicate a deficiency in the dopaminergic inhibitor system.

[Histological, ultrastructural and clinical study of a malignant adrenal neoplasm producing minor mineralocorticoids]. / Studio istologico, ultrastrutturale e clinico di neoplasia maligna surrenalica con produzione di mineralcorticoidi minori.

The authors describe a case of an adrenal tumor of unusual dimensions with a benign histological appearance. After a short period diffuse metastases developed. The metastases should the features a poorly differentiated carcinoma. The size of the tumor appears to be a useful element of judgement in differential diagnosis between adenoma and carcinoma of the adrenal glands.

Mixed-sclerosing-bone-dystrophy (osteopathia striata and osteopetrosis) and Cushing's syndrome.

We present a 33-year-old female patient with the unusual association of mixed sclerosing bone dysplasia and Cushing's syndrome. The patient had the classical features of cortisol overproduction and investigation showed bilateral adrenal adenomas and radiographic abnormalities in bone of osteopathia striata and osteopetrosis: the mixed-sclerosing-bone-dystrophy (MSBD).

Pseudo-Cushing's syndrome: an example of alcohol-induced central disorder in corticotropin-releasing factor-ACTH release?

Six chronic alcoholics with stigmata of Cushing's syndrome were studied before and after a period of alcohol abstinence. In all of them, after a minimum period of 3-4 weeks, a marked clinical and laboratory improvement was noted. The authors suggest that damage at brain level, with neurotransmitters' disturbance, caused by chronic alcoholism underlies the Pseudo-Cushing's Syndrome. The primum movens could be a disorder of the pituitary-adrenal axis secondary to a dysfunction of neurotransmitters with stimulation of ACTH-secreting cells of the adenohypophysis by the certicotropin-releasing factor (CRF).