Introduction 10% of the world’s population suffers from chronic kidney disease. Kidney transplants provide an improvement in the quality of life of those patients. Sexual dysfunction is common after kidney transplantation, and its etiology is presumed to be multifactorial. It has a negative impact on sexual satisfaction and health-related quality-of-life. The integration of a new organ into the body can imply an adjustment of body image, which may eventually have a negative influence on intimacy and sexual behaviors. Aim To evaluate male sexual function, sexual satisfaction, and body image satisfaction among a convenience sample of patients who have had a kidney transplant. Methods This is a cross-sectional study that included 460 patients, from a single healthcare center, who had undergone a kidney transplant procedure >4 weeks ago. A total of 112 respondents (mean = 55.5 years, SD = 11.4) answered the questionnaires properly. Main Outcome Measures All recruited patients answered a self-reported sociodemographic questionnaire, in addition to the International Index of Erectile function, the New Scale of Sexual Satisfaction, the Brief Symptom Inventory, and the Body Image Scale. Results A correlation was found between sexual function and sexual satisfaction (r = 0.598, P < .001, n = 112), as well as between body image satisfaction and sexual function (r = −0.193, P = .042, n = 112). The length of time after a kidney transplant (≤ or >36 months) was not associated with a difference in sexual functioning or sexual satisfaction. Clinical Implications This study showed the obvious implications of sexual function on sexual satisfaction, which should alert healthcare professionals to the importance of identifying and managing sexual dysfunction in patients with chronic kidney disease, to optimize their global and sexual health satisfaction. Strength & Limitations This study identified a high prevalence of sexual dysfunction among kidney transplant recipients. This should reinforce the need for the medical community to evaluate the quality-of-life domains of patients with chronic disease. There is still a lack of information concerning any longitudinal evaluation of kidney transplant patients’ sexual function and the effects that this surgery has on sexuality. Conclusions This study corroborated the severe effects that kidney transplant patients often report regarding their sexuality. Among the patients who participated in the study, sexual function proved to be relevant in relation to sexual satisfaction.

IntroductionPrimary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI.AimsTo characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI.MethodsThis multicentre retrospective study examined PAI patients in 12 Portuguese hospitals.ResultsWe investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 µg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment, 17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances.ConclusionThis is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.

Background. The incidence of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) has been increasing in the last five decades, but there is no large-scale data regarding these tumours in Portugal. We conducted a cross-sectional, multicentric study in main Portuguese centers to evaluate the clinical, pathological, and therapeutic profile of GEP-NENs. Methods. From November, 2012, to July, 2014, data from 293 patients diagnosed with GEP-NENs from 15 centers in Portugal was collected and registered in an online electronic platform. Results. Median age at diagnosis was 56.5 (range: 15-87) years with a preponderance of females (54.6%). The most frequent primary sites were the pancreas (31.1%), jejunum-ileum (24.2%), stomach (13.7%), and rectum (8.5%). Data regarding hormonal status was not available in most patients (82.3%). Stratified by the tumour grade (WHO 2010 classification), we observed 64.0% of NET G1, 24.7% of NET G2, and 11.3% of NEC. Poorly differentiated tumours occurred mainly in older patients (p=0.017), were larger (p<0.001), and presented more vascular (p=0.004) and lymphatic (p=0.001) invasion. At the time of diagnosis, 44.4% of GEP-NENs presented metastatic disease. Surgery (79.6%) and somatostatin analogues (30.7%) were the most frequently used therapies of GEP-NENs with reported grading. Conclusion. In general, Portuguese patients with GEP-NENs presented similar characteristics to other populations described in the literature. This cross-sectional study represents the first step to establish a national database of GEP-NENs that may aid in understanding the clinical and epidemiological features of these tumours in Portugal.

Thyroid lymphomas are rare clinical entities that may result from either the primary intrathyroid de novo or secondary thyroid gland involvement of a lymphoma. Among these, the Hodgkin's subtype is quite uncommon, accounting for 0.6–5% of all thyroid malignancies. The authors report on a 76-year-old female presenting with a thyroid nodule that, upon surgical excision, was found to be a nodular lymphocyte predominant Hodgkin lymphoma of the thyroid. So far, thyroid involvement by this variant has never been reported. Upon reporting on this clinical case, the authors emphasize the difficulties usually found in establishing the diagnosis and in defining the best management strategy. A thorough review of the available literature is done.

ObjectiveMutations in the genes coding for succinate dehydrogenase (SDHx) are the most frequent germline alterations in pheochromocytomas and paragangliomas. Evidence for the advantages associated with presymptomatic screening for SDHx mutation carriers is scarce. This study describes a nationwide cohort of these mutation carriers and aims to compare patients with clinical manifestations of the disease and those diagnosed through genetic screening. DESIGN: Cross‐sectional study.PatientsSDHx mutation carriers (n = 118) followed through the Portuguese Oncology referral centres: 41 probands and 77 nonprobands.MeasurementsAll participants were subjected to biochemical and body imaging examinations for a complete assessment of the extent and spread of disease. Clinical data obtained this way were further analysed.ResultsThe mean age of this cohort was 44.5 ± 17.4 years, and more than half carried the same founder SDHB mutation. About 50.8% of the mutation carriers developed pheochromocytomas or paragangliomas. Compared to patients diagnosed through genetic screening, those diagnosed clinically were characterized by larger tumours (P < .001), more frequent metastases (P = .024), were more frequently subjected to surgery (P = .011) and radiotherapy (P = .013), and had worse outcomes, such as macroscopic positive margins (P = .034). Persistent and/or unresectable disease and disease‐related mortality were also more frequent in symptomatic patients compared to those diagnosed through genetic screening (P = .014).ConclusionsIn this nationwide cohort study, a large proportion of mutation carriers were found to develop SDHx‐related neoplasia. Genetic testing and subsequent follow‐up resulted in the diagnosis of smaller and nonmetastatic tumours, fewer treatment procedures, fewer complications and greater number of disease‐free patients.

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Objective Parathyroid Carcinoma is a rare malignant neoplasm, accounting for less than 1% of primary hyperparathyroidism cases. Parathyroid carcinomas are characterized by markedly elevated levels of PTH, severe hypercalcemia and established target organ damage. The authors report the experience of a single centre regarding the management and outcome of patients with parathyroid carcinomas and revise relevant literature. Design Retrospective review of all patients with parathyroid carcinoma evaluated at a tertiary oncologic centre from 1991 until 2021. Results Seventeen patients were identified (10 males), with a mean age at diagnosis of 53 ± 16 years and a median follow‐up of 16.5 years. Most patients presented with hypercalcemia (n = 15), with a mean serum calcium concentration of 13.5 mg/dl (9.6–16.5) and mean PTH of 1173 pg/ml (276–2500). Hyperparathyroidism‐mediated organ damage was observed in most patients (n = 16), with predominant renal (n = 12) and skeletal (n = 9) complications. En bloc surgical resection was performed in nine patients. Three patients underwent adjuvant radiotherapy. Recurrence was observed in 8 cases (47.1%) after a median of 24 months following surgery and no independent predictors of recurrence were identified. The overall survival and disease specific survival at 5‐year was 88% and 94%, respectively. CDC73 mutations were present in 38.5% of analysed patients and one patient was diagnosed with MEN1. Conclusion Parathyroid carcinoma is associated with a significant rate of recurrence and limited effective treatment beyond initial complete surgical resection. Therefore, preoperatively high index of suspicion is paramount to optimize patient care. This is, to our knowledge, the largest Portuguese cohort published so far.

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