ACTA ENDOCRINOLOGICA (BUC)

In this study we aimed to evaluate and quantify optic nerve damage caused by long standing compressive pituitary macroadenomas with conventional (ophthalmoscopy) and modern techniques such as fundus camera, confocal scanning laser tomography for quantitative measurements of the thickness of retinal layers as well as visual evoked potentials (VEP) for electrophysiological quantification. Seven patients with large, long standing pituitary macroadenomas were submitted to ophthalmologic evaluation, including a visual field (VF), visual acuity (VA) and eye fundus (F). Heidelberg retinal tomography (HRT) was used for retinal thickness and evaluation of nerve fibers loss, and VEP were measured by pattern reversal and flash stimulus. In addition, all patients underwent tumor imaging (MRI/CT) and specific endocrine evaluation. All cases presented with macroadenomas with suprasellar extension and residual or progressive optic chiasma syndrome; all but one (prolactinoma) were nonfunctioning adenomas, after radical treatment (surgery ? radiotherapy). Adrenal and thyroid substitutive treatment was instituted in all cases due to associated pituitary failure. Evaluation of VF showed 9 eyes with temporal hemianopia, 2 with nasal islands of vision and 1 with nasal hemianopia in a homonymous hemianopia case; another case presented for left 3rd nerve palsy due to a cavernous sinus syndrome, therefore the visual field was not measurable in 2 eyes. Visual acuity was very low (counting fingers) in 4 eyes, while in the rest the VA was between 0.5-0.9. The fundus revealed total atrophy in 2 eyes, band atrophy in 4, temporal pallor in 5 and global pallor in 1. Cup/disk ratio in the case with 3rd nerve palsy was 0.5 (RE) and 0.3 (LE). HRT II stereometric analysis of the optic nerve head showed abnormal values, documenting retinal nerve fiber layer (RNFL) loss that correlated with fundus appearance and visual field defects. Mean RNFL thickness had abnormal values in 8 eyes (from 0.074 to 0.173 μm), correlated with RNFL cross sectional area in 7 eyes (from 0.362 to 0.846 μm2) and 1 eye with low limit values (1000 μm2). In agreement with these data, VEP–P100 presented increased latency over 120 ms in 8 eyes, borderline (100-120 ms) in 5 and 97.5 ms in only 1 eye. In conclusion, HRT can document the papilla and nerve fiber layer more objective, permitting quantification of the disc’s alterations due to compressive pituitary macroadenomas. HRT is useful in quantifying RNFL loss in other conditions than glaucoma, when other optic disc imaging tools are not available.

We report a case of an infant with syndromic 46,XY disorder of sexual development. The subject was born at term, to unrelated parents with no relevant medical history. At birth the infant was assigned female. Physical examination showed dysmorphic features and ambiguous external genitalia. Cytogenetic analysis of cultured peripheral blood lymphocytes revealed a male karyotype. The result of the chromosomal investigation showing male genetic sex, together with the ambivalent aspect of the external genitalia (Prader IV) and gonads that are exclusively testes led to the diagnosis of 46,XY disorder of sexual development. The clinical management will help the child and the family deal effectively with this condition A multidisciplinary approach to this problem involving pediatricians, specialists in the field of endocrinology, genetics, surgery and psychiatry is necessary in order to reach a prompt and correct diagnosis and treatment.

Background. The association between high blood pressure and hypokalemia is usually caused by primary or secondary hyperaldosteronism. Recent studies indicate that\r\nprimary hyperaldosteronism is a much more common cause of hypertension than had been previously demonstrated. Arterial hypertension is often present in hypothyroid patients, but almost never associated with hypokalemia.\r\nCase report. We report the case of a 69 years old male admitted for shortness of breath, inferior limbs edema and fatigue. From his medical history we mention: essential\r\narterial hypertension (for about 25 years), ischaemic coronary artery disease (for 20 years), for which he underwent PTCA (two years ago), atrial fibrillation electrically converted to sinus rhythm (a year a ago). Despite taking four antihypertensive drugs his blood pressure was far from being controlled. Blood analysis revealed an important hypopotasemia (K 2.4mmol/l) and consequently the loop diuretic was replaced with a potassium-sparing diuretic. The measured proved to be unsuccessful and potassium supplements had to be\r\nadded, but with modest results (K 2.94mmol/l). Further specific investigations revealed almost normal levels of aldosteron, low renin, normal cortisol. Associated was a high Thyroid-stimulating hormone (TSH). Computer tomografy (CT) showed bilateral suprarenal glands adenomas.\r\nConsidering the laboratory findings, we interpret the case as a primary hyperaldosteronism and a successful treatment with spironolactone was initiated.

Introduction. Over the past decades, several definitions and classifications of cervico-mediastinal goiters have been proposed. We analyzed and discussed the clinical presentation, the diagnostic procedures and the surgical technique in relation to post-operative complications and long-term results in a case of a sixtysix years old obese, hypertensive female admitted in the Thoracic Surgery Department with respiratory distress (inspiratory dyspnea, stridor) progressively aggravating during the latest month. Methods. Cervico-thoracic CT scan revealed the existence of a cervico-mediastinal huge goiter which developed mostly intrathoracic (2/ 3 of the goiter). It determined a tracheal compression, reducing its caliber by two thirds, and its displacement to the right side. The proposed surgical procedure was total thyroidectomy and it involved a bipolar approach (transcervical and transsternal) through a partial upper cervico-sternotomy. Results. The complete removal of the goiter and the decompression of the trachea have been achieved. Postoperative results were very satisfactory, with the absence of the respiratory distress. The histological examination revealed a multinodular goiter with epithelium hyperplasia. Conclusion. The presence of a complicated cervico-mediastinal goiter with severe respiratory distress required a surgical excision as the main and immediate treatment option. The surgical procedure represented a milestone for both the anesthesiologist (difficult intubation, with a thin tracheal tube in the absence of the jet ventilation technology) and for the surgeon. The goiter’s excision from the visceral mediastinum was very difficult because of its huge dimensions and close relations with trachea and great vessels (anterior) and esophagus, erector spinal muscles and the spine (posterior).

Context. Both human immunodeficiency virus (HIV) infection and hepatitis C virus (HCV) infection represent systemic diseases that may develop metabolic complications, thus HIV/HCV coinfection metabolic changes need to be depicted. Objective. We aimed to evaluate the body composition changes in patients with either HIV and HCV monoinfections or HIV/HCV coinfection. Methods. 123 young men divided into three groups: 41 with HIV/HCV coinfection, 42 with HIV-monoinfection, and 40 with HCV-monoinfection were evaluated for total and regional bone and soft tissue body composition assessments using a Dual-energy X-ray absorptiometry (DXA) and were compared with 40 healthy men with age and body mass index similar to the study groups. To detect sarcopenia, we calculated the appendicular limbs’ lean mass index (ALMI), for obesity, we used the percent of body fat, and for lipodystrophy, we calculated the trunk/limbs index. Results. HIV/HCV coinfection is associated with a significant higher bone demineralization in all regions of interest compared to HCV or HIV monoinfections and to controls. The prevalence of bone demineralization in HIV/ HCV patients was 31.7%, more frequently at lumbar spine. Fat mass and lean mass were significantly lower in HIV/ HCV-coinfected patients than in controls. Lipodystrophy was found in similar percentages in all three evaluated groups (80.4% in HIV/HCV, 92.5% in HIV, and 95% in the HCV group). Sarcopenia was higher in HIV/HCV group (43.9%) and important in HCV-monoinfection group (30%). Conclusions. HIV/HCV-coinfected patients had the highest prevalence of bone demineralization, fat mass, and lean mass loss, compared to controls and to HIV and HCV monoinfections.

Context. Metabolic surgery is currently the most efficient treatment for obesity, but concern is raised about the possible long-term nutritional side effects. Bone metabolism is often adversely affected after surgery, but literature data are contradictory. Objective. The aim of this study was to evaluate the evolution of bone mass parameters in the first year after laparoscopic sleeve gastrectomy in relation to anthropometric and body composition parameters and specific hormones of obesity. Design. We conducted a prospective study on 75 patients with obesity that underwent metabolic surgery over a course of 18 months at our center, with a follow-up period of 12 months. Subjects and Methods. All patients underwent a complex preoperative assessment and were required to return for medical follow-up at 6 and 12 months after surgery. Each visit included anthropometric parameters, DEXA and determination of specific hormonal parameters. Results. We noticed a significant improvement in anthropometric and body composition parameters after surgery. The value of adiponectin presented a significant increase after surgery and leptin showed a significant decrease at 6 and 12 months postoperative; ghrelin level decreased postoperative compared to preoperative, but without statistical significance. We observed no reduction in BMD after surgery, but a significant improvement in BMC at 12 months after surgery compared to preoperative. Ghrelin negatively correlated to BMD preoperative. Conclusions. Despite the significant alterations in anthropometric, body composition and hormonal parameters, we found no negative effect on BMD and BMC in our study population.