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Abstract Introduction We report the case of a patient with classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency who presented with unusual anatomical and biochemical features, namely massively enlarged adrenal glands, adrenogenital rest tissue and an unexpected endocrine profile. The contribution of the adrenocortical cells in the adrenals and testicles was determined by a cosyntropin stimulation test before and after adrenalectomy. To the best of our knowledge this is the first report of such a case in the literature. Case presentation A 35-year-old Caucasian man was admitted to the emergency room with an Addisonian crisis. He had been diagnosed with congenital adrenal hyperplasia in the neonatal period. He acknowledged poor adherence to treatment and irregular medical assistance. Physical examination revealed marked cutaneous and gingival hyperpigmentation, hypotension, and hard nodules in the upper pole of both testicles. Blood analysis showed mild anemia and hyponatremia and no evidence of acute infection. Endocrine evaluation showed very low cortisol levels, low dehydroepiandrosterone-sulfate and elevated corticotropin, 11-deoxycortisol and delta-4-androstenedione. The concentration of 17-hydroxyprogesterone was 20,400ng/dL. After the cosyntropin stimulation test the pattern was similar and there was no significant increase in cortisol or 17-hydroxyprogesterone. The abdominal computed tomography scan revealed grossly enlarged and heterogeneous adrenal glands (left, 12cm; and right, six cm). A bilateral adrenalectomy was performed and pathologic examination revealed adrenal myelolipomas with nodular cortical hyperplasia. The sonogram showed bilateral heterogeneous masses on the upper pole of both testes which corresponded to the nodular hyperplasia of adrenal rest tissues. The genetic study revealed compound heterozigoty (mutations R124H and R356W), suggestive of a phenotypically moderate disease. We performed a cosyntropin stimulation test after adrenalectomy. The steroidogenic profile displayed the same unusual features, indicating an important contribution from the adrenogenital cells. Conclusion This case illustrates that congenital adrenal hyperplasia due to 21-hydroxylase deficiency can progress to severe acute and chronic complications. The masses in the patient’s adrenal glands and testicles resulted from chronically elevated adrenocorticotropic hormone and growth of adrenocortical cells. The basal and stimulated steroid profile, before and after adrenalectomy, revealed an unexpected pattern, suggesting significant contribution of the testicular adrenal cells to the steroidogenesis.

Background Cancer screening has contributed to downward trends in cancer mortality, but is also associated with adverse effects, which highlights the importance of promoting the participation based on informed decisions. Objectives We aimed to describe the use of cancer screening (either in organized programmes or as opportunistic screening), awareness of organized programmes and perception of its potential benefits and adverse effects, depicting possible sex differences. Design and methods We evaluated 1624 Portuguese-speaking dwellers, aged between 16 and 79 years, through face-to-face interviews. To quantify sex differences, adjusted prevalence ratios and respective 95% confidence intervals were computed using Poisson regression. Results Among eligible age groups, the lifetime prevalence of screening for breast and cervical cancers was 89.8 and 71.9%, respectively. The prevalence was 23.7% for colorectal cancer and no significant sex differences were observed. Prostate cancer screening was reported by 63.8% of men. Over half of the participants referred that cancers such as prostate, skin, lung and stomach should be screened for, in addition to those for which organized programmes are recommended. Reassurance by negative results was identified as the main potential benefit of screening by nearly one-third of men and women. Anxiety while waiting for results was the most mentioned potential adverse effect (60.4%); men refer less often this and financial costs, although statistical significance of these results was borderline. Conclusions This study provides a benchmark to plan and monitor the effects of awareness-raising interventions, as well as for international comparisons across countries with different cancer prevention and control structures. [ABSTRACT FROM AUTHOR]

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Copyright of Revista de Enfermagem Referência is the property of Escola Superior de Enfermagem de Coimbra and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Hypoglycemia in apparently healthy adults is a rare finding in clinical practice requiring a thorough investigation of the cause. During the investigation, identification of hypoglycemia associated with inappropriately high levels of insulin and C-peptide should prompt the exclusion of rare causes of hypoglycemia, including pancreatic islet-cells disease and autoimmune hypoglycemia. In this paper, we describe two cases of hypoglycemia associated with endogenous hyperinsulinism, whose causes are uncommon in clinical practice, and review important aspects of the diagnosis and treatment of hyperinsulinemic hypoglycemia.

Objectives: Central neuropeptides modulate behaviour. Plasma levels of neuropeptides may reflect central levels due to specific brain-to-blood transport systems. We purposed to show the modulation of plasma corticotropin releasing hormone (CRH) levels in relation to induced emotions., Design: Three groups were defined. For experimental groups A and B, an emotionally significant movie fragment was projected for 20 min, while no film projection occurred in group C. Peripheral venous blood samples were collected before, 10 and 60 min after the film or at 0 and 30 min for group C. Total CRH was measured in plasma. Personality was evaluated by the Minnesota Multiphasic Personality Inventory (MMPI)., Results: Plasma CRH levels did not change in the condition with no movie projection - group C - 346 + or - 198 vs. 327 + or - 143 pg/mL. Plasma CRH levels dramatically increased with the projection of a dramatic movie - group A - 394 + or - 147 vs. 791 + or - 636 vs. 803 + or - 771 pg/mL, p<0.05. Plasma CRH increased less markedly in the condition with the projection of a comic movie - group B - 364 + or - 138 vs. 486 + or - 260 vs. 483 + or - 228 pg/mL, p<0.05 for differences between samples 1 and 3. Baseline plasma CRH was significantly and independently related to the neurotic triad and psychotic dyad - partial r=0.328 and 0.267, respectively, p<0.05., Conclusions: We conclude that plasma CRH levels increase with experimental emotion induction and that baseline levels are significantly related to behavioural traits. Plasma levels of neuropeptides may reflect central levels and may be useful in clinical medicine and in the study of behavioural disorders.