ACTA ENDOCRINOLOGICA (BUC)

Vitamin D is essential in efficient absorption of calcium and normal mineralization of bone. Severe vitamin D deficiency produces impairment of bone mineralization and osteomalacia. Less severe vitamin D deficiency, called vitamin D insufficiency or inadequacy, causes secondary hyperparathyroidism, increased bone turnover and bone loss. The aims of our study were: the evaluation of vitamin D status and its seasonal variation in postmenopausal women with osteoporosis, from Romania; we studied also the relationship between vitamin D and parathyroid hormone serum concentrations in order to establish the threshold level of vitamin D which determines secondary hyperparathyroidism. The study was performed on 834 postmenopausal (natural or surgical) women who attended the National Osteoporosis Center and were diagnosed with osteoporosis using dual absorptiometry with X ray (DEXA). None was receiving vitamin D supplementation and they were not taking drugs affecting bone and mineral metabolism and were not suffering from such diseases. Estimation of vitamin D and parathyroid hormone status was made by determining 25-hydroxyvitamin D (25OHD) and PTH-intact (PTH) serum concentrations from a single blood sample using immuno-enzyme methods. The results were compared using Student? t test for unpaired values and linear regression to establish the correlation. All data were expressed as mean value ? standard deviation and a value of p<0.05 was considered as statistically significant. In a previous study we reported for premenopausal normal women the mean value for 25OHD at 26.58?10ng/ml. At the same time, we defined the vitamin D deficiency as the levels below 12 ng/mL, and the insufficiency of vitamin D (vitamin D inadequacy) as the values between 12 and 26.58 ng/mL. The mean serum 25OHD concentration for our study group was 20.04?144.22 mg/mL. We defined arbitrary values (using data from international studies) for 25OHD serum levels to estimate the prevalence of vitamin D deficiency and vitamin D inadequacy in our study group. In 834 postmenopausal women with osteoporosis, living in Romania, without vitamin D supplementation or pharmacological therapy to treat or prevent osteoporosis, the prevalence of vitamin D deficiency was 32.2% and the prevalence of vitamin D inadequacy was 42.3%. Seasonal variation of 25OHD was found statistically significant (p<0.05), with lower values at the end of winter. Statistical tests applied to results have shown a significant negative correlation between PTH and 25OHD serum levels (p<0.001) and established the cut-off concentration for 25OHD which determine secondary hyperparathyroidism at 20 ng/mL. Our data underscore the need for adequate vitamin D supplementation in women with osteoporosis.

Context. The objectives of treatment of thyroid carcinoma include hormonal suppression, radioiodine ablation and follow-up with serum thyroglobulin (Tg). Tg levels should not be measured before six weeks postthyroidectomy. Objective. To describe the behaviour of early postoperative Tg in patients who underwent total thyroidectomy and its ability to predict the serum Tg levels after suppression. Design. This is a retrospective cohort study. Subjects and methods. Adult patients who underwent total thyroidectomy with at least two postoperative measurements of serum Tg, negative TgAb and concomitant serum TSH values were included. Tg, TgAb and TSH level measurements were completed two weeks postoperatively and during the follow-up period. Results. Twenty-nine patients fulfilled all criteria. The median serum Tg level at two weeks after surgery was 3.8 ug/L (0.3 -300) with a serum TSH level of 69.9 mU/L; 11-227. At the two-week measurement, 16 (55%) patients had serum Tg levels lower than 5 ug/L and 4 patients had levels between 5-10 μg/L. Conclusions. Postoperative early serum Tg could be an alternative to values measured six months after surgery and could be used as a predictive tool to make earlier therapeutic decisions.

Background. Pheochromocytoma, the great masquerader, can have a varied spectrum of clinical manifestations. It can often cause a diagnostic challenge despite the availability of modern investigation modalities. Case. We present the case of a 38-year-old male who presented with uncontrolled hypertension for the past 10 years and heart failure for one year. The diagnosis of pheochromocytoma was missed in the initial setting, leading to a biopsy of the retroperitoneal mass. Fortunately, the patient survived the procedure. Subsequently, with the involvement of a multi-disciplinary team, he was optimized for surgery under strict cardiac monitoring. After the complete excision of the tumour, he showed significant improvement not only in his clinical symptoms but also in his cardiac status. Conclusions. This case emphasizes the age-old medical phrase of ‘Primum non nocere or first, do no harm’. Any invasive procedure in a pheochromocytoma can lead to a massive release of catecholamines causing a hypertensive crisis, pulmonary oedema, and even cardiac arrest. Any young patient presenting with hypertension or heart failure should be investigated for secondary causes. Cardiomyopathy due to pheochromocytoma is because of catecholamine overload and usually reverses or improves after curative surgery.

Introduction. Hypoparathyroidism is a rare endocrine disorder, leading to complications affecting the kidneys, the eyes, the cerebrum (Fahr’s syndrome, epilepsy, parkinsonism, cognitive impairment), and the heart. Case report. We present the case of a 24-year old male that presented to our clinic for generalized tonicclonic seizures increasing in frequency for the last year or so. Furthermore, he was diagnosed with subcapsular cataract of both eyes and had intraocular lens implant surgery 12 years ago. CT scan performed at admission showed calcium deposits on the dentate nuclei, basal ganglia, thalamus, cerebellum and bilateral subcortical fronto-parietal white matter compatible with Fahr’s disease. The results of the laboratory tests were normal except for hypocalcemia, hyperphosphoremia and low intact parathormone (iPTH). The patient was diagnosed with primary hypoparathyroidism which led to Fahr’s syndrome. The patient was prescribed oral medication including calcium carbonate, calcidiol, magnesium orotate and levetiracetam. The follow-up after three months revealed a seizure free status and normalization of magnesium, calcium, and phosphorus levels. Conclusion. Fahr’s syndrome can be a cause of epileptic seizures and should be considered in the clinical approach to an epileptic patient, especially in children, teenagers, and young adults.

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).

Introduction. Pseudoxanthoma elasticum (PXE) is a generalized progressive connective tissue disorder which affects elastic fibers and has multiple systemic manifestations. Case presentation. The patient, female, 39 years old, who was diagnosed in the teenage years with PXE, was hospitalized presenting asymptomatic nephrocalcinosis and increased plasma parathyroid hormone (PTH) levels. The laboratory data revealed: normal levels of calcium and phosphorus in serum, very low serum level of 25-hydroxycholecalciferol [25(OH)D] and low serum level of 1,25-dihydroxycholecalciferol [1,25(OH)2D3]. It was considered that our patient presented secondary hyperparathyroidism. The patient received therapy with alphacalcidol 0.5 μg/day and after 6 months of therapy, the evaluation of the plasma PTH level showed a normal value associated with an improvement of the serum level of the 25(OH)D (27.5 μg/L). Conclusions. Our case is original in terms of association of pseudoxanthoma elasticum with nephrocalcinosis and elevated plasma levels of PTH, which requires a differential diagnosis between normocalcemic primary hyperparathyroidism and secondary hyperparathyroidism. The very low level of 25(OH)D in serum associated with low levels of 1,25 (OH)2 D3 and a normal level of phosphorus led the diagnosis towards secondary hyperparathyroidism.

A 9 member independent panel of endocrinologists and pediatricians was convened to discuss basic issues with respect to definition, diagnosis, and clinical management of children born small for gestational age (SGA). SGA is defined as the situation when birth weight and/or length are at least 2 standard deviations (SD-s) below the mean for gestational age (<2 SD). The seven consensus guidelines agreed by the panel members are as follows: i. Children born SGA have a serious and permanent handicap of growth in adulthood if not treated. ii. Any term newborn lighter than 2500 g (male) or 2000 g (female) may be considered SGA. iii. When 4 years of age, all children born SGA will be evaluated for height, height velocity, bone age. Preterm children and multiplets should be included even if they were AGA. All children having concomitantly a SD score of < -2.5, a HVSD score < -1 and a bone age (Greulich-Pyle) smaller or equal to the chronological age are to be considered candidates to GH therapy. iv. All recognizable short stature syndromes must be extracted from the contingent of SGA by clinical and laboratory thorough evaluation and subjected to their distinctive growth disturbance protocol. v. The rest of candidates will receive GH therapy at 0.35 mg/kg/week daily dosage. A\r\nbiochemical screening including hormonal and IGF I determinations and also hematology should be performed before administering the first injection. GH dynamic tests are not recommended as a rule. vi. Effectiveness of the therapy will be appreciated after 6-12 months of uninterrupted therapy. Annualized growth rates higher than 7 cm/yr are considered proofs in favor of successful therapy. IGF I levels higher than 600 ng/ml should be avoided. vii. After the inclusion in therapy and the first evaluation, the patients follow-up should align to the standard provisions of the national or regional programs of GH therapy.

Objective: Co-existence of Cushing disease and Rathke?s Cleft Cyst (RCC) has been reported in a few cases in the literature so far. We herein describe a rare condition of Cushing disease that might originate from epithelium of RCC. \r\nCase: A 48-year-old woman was admitted to the hospital with complaints of headache, weakness, and weight gain. The patient underwent endoscopic transsphenoidal surgery due to Cushing Disease. Histopathological examination revealed cyst contents and walls compatible with RCC, and normal adenohypophysis and neurohypophysis tissues. Immunhistochemical staining with ACTH, GH, and prolactin were positive on the epithelium of the cyst. \r\nConclusion: In our case Cushing disease might be associated with hormonal activity derived from cyst wall of RCC or disappearance of a small microadenoma during surgical or pathological processing. According to recent data, origin of this lesion and histogenetic link between RCC with Cushing disease could not be explained.

Background. Advances in imaging techniques have led to increasing discovery of\r\nadrenal and pituitary &#8220;incidentalomas&#8221;, tumors with normal endocrine function and no\r\ncompression mass effects. We evaluated the age at diagnosis (AD) in patients with benign\r\nnon-functioning adrenal incidentalomas, as compared to pituitary non-functioning tumors,\r\nin a series of patients from a national center of endocrinology. Methods. From 2,123\r\nconsecutive patients with adrenal and pituitary tumors hospitalized between 1977 - 2009,\r\n2,069 patients were analysed. The study groups included: group A - 137 patients with\r\nadrenal incidentalomas (AI), group B - 534 patients with pituitary incidentalomas (PI).\r\nControl groups included 1,398 patients: group C1 147 patients with adrenal carcinomas or\r\nbenign hormone-secreting adrenal tumors, and group C2, 1,251 patients with pituitary\r\nsecreting adenomas or large non-functioning pituitary macroadenomas (NFA). Imaging was\r\ndone by computed tomography and/or magnetic resonance after 1981 and by skull X-ray or\r\npneumoencephalography before 1981. Results. Mean age AD is more advanced in patients\r\nwith AI (53 ? 11.9 years, range 21 - 78 yr) than in patients with PI (36.8 ? 13.1 years, range\r\n10 - 81 yr), p < 0.01. AD was higher in AI than in patients with secreting adrenal tumors,\r\nbut similar in patients with adrenal malignancy. There is an age-related increase in the\r\nproportion of AI among patients with adrenal tumors, and of NFA, but not of PI, among\r\npatients with pituitary tumors. In patients aged over 65 years, 74% of patients with adrenal\r\ntumors have AI, while only 18% of patients with pituitary tumors have PI and 42% have\r\nNFA. AD in NFA (49.3 ? 13.1 yr, range 12 - 79 yr) was more advanced than in PI (p < 0.01).\r\nAD does not correlate with tumor size. Tumor growth occurred in 24% of AI (follow-up 3.0\r\n? 2.8 yr) and only in 0.7% of PI, p<0.01 (follow-up 3.1 ? 2.5 yr).\r\nConclusions. Adrenal non-functioning benign tumors show a clear association with ageing,\r\nin contrast with pituitary incidentalomas. It seems unlikely that most pituitary incidentalomas in\r\nyoung patients become large NFA, whose development seems to be also age-related. It is tempting\r\nto suggest that pituitary tumorigenesis starts earlier than adrenal tumorigenesis.

Objective. A partial or complete deficiency of hormone secretion by pituitary gland (hypopituitarism) is commonly seen after a pituitary apoplexy caused by an infarction of a pituitary adenoma or pituitary hyperplasia (as in Sheehan’s syndrome). Hypopituitarism may also follow surgery, when hypovolemia, anticoagulation, fat/ air/bone marrow microemboli can provoke a pituitary infarction/hemorrhage. Other causes of abrupt hypophyseal hypoperfusion, as hypovolemia during a septic shock, could also contribute. In the last mentioned situation, due to the complex endocrine-immune interrelation, sepsis could be masked and improperly managed. Case report. We report a case of a 72 years-old Caucasian woman, previously healthy, who underwent an orthopedic surgery for a femoral fracture. This event apparently triggered a central-origin hypothyroidism, misinterpreted as “post-surgical psychosis”, which, in turn, masked a symptomatology of a subsequent severe sepsis. The patient was admitted in the infectious diseases department with a severe gut-origin sepsis, needing surgery and long course antibiotics. The pituitary insufficiency was reversed. Conclusion. Pituitary apoplexy is an uncommon but potentially life-threatening disease, and could be precipitated by successive events – in our case an orthopedic surgery and a subsequent severe sepsis. It needs recognizing (has intrinsic severity and could mask other serious conditions), treat and monitor (could progress and/or reverse).