Effect of Parathyroidectomy on Left Ventricular Mass Index in Patients With Primary Hyperparathyroidism.

Aim Primary hyperthyroidism (PHPT) is known to affect left ventricular structure and function and may contribute to increased cardiovascular morbidity and mortality. Whether parathyroidectomy (PTX) reverses left ventricular hypertrophy/remodeling among PHPT patients remains controversial. Method In this prospective, single-center study, we enrolled patients with the diagnosis of PHPT who were scheduled for PTX. Patients underwent a complete biochemical workup and an echocardiographic examination at baseline and a six-month follow-up. The primary objective was to compare the left ventricular mass index (LVMI) at baseline and six-month follow-up. Result Eighteen patients (15 female, three male, mean age 58.7 years) were enrolled. PTH and serum calcium returned to normal immediately post-PTX and remained normal at six months. LVMI at baseline was within normal limits and reduced further at the six-month follow-up. The left ventricular ejection fraction was in the normal range before the PTX and remained unchanged during follow-up. Conclusion Curative PTX reduced LVMI further within the normal range at six months in patients with asymptomatic hyperparathyroidism, providing evidence for benefit in an important non-traditional disease manifestation.

Neurofibromatosis Type 1 Has a Wide Spectrum of Growth Hormone Excess.

Overgrowth due to growth hormone (GH) excess affects approximately 10% of patients with neurofibromatosis type 1 (NF1) and optic pathway glioma (OPG). Our aim is to describe the clinical, biochemical, pathological, and genetic features of GH excess in a retrospective case series of 10 children and adults with NF1 referred to a tertiary care clinical research center. Six children (median age = 4 years, range of 3−5 years), one 14-year-old adolescent, and three adults (median age = 42 years, range of 29−52 years) were diagnosed with NF1 and GH excess. GH excess was confirmed by the failure to suppress GH (<1 ng/mL) on oral glucose tolerance test (OGTT, n = 9) and frequent overnight sampling of GH levels (n = 6). Genetic testing was ascertained through targeted or whole-exome sequencing (n = 9). Five patients (all children) had an OPG without any pituitary abnormality, three patients (one adolescent and two adults) had a pituitary lesion (two tumors, one suggestive hyperplasia) without an OPG, and two patients (one child and one adult) had a pituitary lesion (a pituitary tumor and suggestive hyperplasia, respectively) with a concomitant OPG. The serial overnight sampling of GH levels in six patients revealed abnormal overnight GH profiling. Two adult patients had a voluminous pituitary gland on pituitary imaging. One pituitary tumor from an adolescent patient who harbored a germline heterozygous p.Gln514Pro NF1 variant stained positive for GH and prolactin. One child who harbored a heterozygous truncating variant in exon 46 of NF1 had an OPG that, when compared to normal optic nerves, stained strongly for GPR101, an orphan G protein-coupled receptor causing GH excess in X-linked acrogigantism. We describe a series of patients with GH excess and NF1. Our findings show the variability in patterns of serial overnight GH secretion, somatotroph tumor or hyperplasia in some cases of NF1 and GH excess. Further studies are required to ascertain the link between NF1, GH excess and GPR101, which may aid in the characterization of the molecular underpinning of GH excess in NF1.

Translation of the Pasieka's Parathyroid Assessment of Symptoms Questionnaire (PAS-Q) for Use in the Greek Population.

Introduction: In Europe, primary hyperparathyroidism is mainly considered an asymptomatic disorder, although there is evidence that patients' health-related quality of life is impaired. This aspect is mostly evaluated using Pasieka's Questionnaire: a disease-specific diagnostic tool. The purpose of this study was to translate the Pasieka's Questionnaire into the Greek language and adapt it to the Greek population. Materials and Methods: Pasieka's Questionnaire consists of 13 questions. Two bilingual, native Greek experts were selected for step one, each of whom offered a blinded Greek version of the questionnaire. In the second step, these two versions were merged into one which was retranslated back into the English language (step three) by two bilingual translators (English native speakers). In the fourth step, a committee was formed to draft the pre-final version of the questionnaire which was then submitted to the co-authors for final approval. Finally, after the approval of the final version, 50 patients with primary hyperparathyroidism were recruited for the pilot study of the questionnaire. Results: All 13 questions of the Pasieka's Questionnaire were translated without any major discrepancy. A high level of internal consistency was achieved (Cronbach's alpha was 0.904) and agreement between test-retest was excellent for every question. Conclusion: The Greek version of Pasieka's Questionnaire was validated and can be applied to evaluate the health-related quality of life of patients with primary hyperparathyroidism in Greek-speaking populations.

Quality of Life in Patients With Asymptomatic Primary Hyperparathyroidism After Parathyroidectomy: A 3-Year Longitudinal Study.

OBJECTIVE: Impaired quality of life (QoL) is considered as a nonclassical manifestation of primary hyperparathyroidism (PHPT). This study aimed to detect and compare changes in the QoL of patients with asymptomatic PHPT who had successful curative parathyroidectomy (PTX) 3 months and 3 years after the procedure. METHODS: Patients with diagnosed PHPT were eligible for the study. There were 2 groups: the PTX group, with patients who underwent PTX, and the non-PTX group, with patients who were treated conservatively. QoL was assessed using Pasieka's Parathyroid Assessment of Symptoms Questionnaire (PAS-Q) at baseline, 3 months, and 3 years. RESULTS: Thirty-eight patients were included in the study: 18 in the PTX group and 20 in the non-PTX group. In the PTX group, the mean PAS-Q total score before PTX was 518, which was reduced significantly at the 3-month (P = .003) and 3-year assessments (P = .001). However, in the non-PTX group, the mean PAS-Q total score was 326 at baseline and increased continuously for 3 years (P = .019). At the 3-year evaluation, the mean total score was significantly higher compared to that of the PTX group (P = .021). Finally, there was a positive correlation between total serum calcium and PAS-Q score in the non-PTX group (r = 0.524, P = .018). CONCLUSION: QoL of patients with PHPT improved significantly compared to that in conservative surveillance as early as 3 months after successful, curative PTX, and remained improved for 3 years. This finding strengthens, even more, the hypothesis that PTX contributes to better QoL, suggesting that the derangement of QoL may be considered as an individual indication for surgery.

A mutation in NOTCH2 gene first associated with Hajdu-Cheney syndrome in a Greek family: diversity in phenotype and response to treatment.

INTRODUCTION: Hajdu-Cheney Syndrome (HCS) is a rare genetic autosomal dominant disorder, characterized by distinctive facial features, acroosteolysis, and severe osteoporosis. Very rarely HCS is associated with polycystic kidney disease, splenomegaly or Crohn's disease (CD). It is caused by gain-of-function mutations in NOTCH2 gene. Treatment with bisphosphonates or denosumab is reported to result in BMD increase. OBJECTIVE: We report a mutation in exon 34 of NOTCH2 gene, in a Greek pedigree, with diverse phenotypes among members. DESCRIPTION OF THE PEDIGREE: The 48-year-old mother had a history of a T12 vertebral fracture, postpartum at the age of 21 and two subsequent uneventful full-term pregnancies and never received treatment. Her 29-year-old son, presented with severe osteoporosis and multiple morphological vertebral fractures. Her 21-year-old daughter had recurrent vertebral fractures starting at 10 years of age. At 17 years, she developed severe CD, resistant to treatment with biologic agents, and functional hypothalamic hypogonadism. One male pedigree died of cystic fibrosis. All subjects bore the typical facial characteristics and acroosteolysis, while none had splenomegaly or renal defects. Zoledronate infusion led to BMD increase. GENETIC TESTING: Mutation in c.6758 G > A (NM_008163.1), leading to a Trp2253Ter replacement. This mutation has been reported as possibly pathogenic (SCV000620308), but not in association with HCS. CONCLUSIONS: Bone involvement can present with diverse severity in the same pedigree, ranging from low BMD to multiple fragility fractures. Antiresorptive therapy improves BMD, but its anti-fracture efficacy remains to be shown. The presence of CD might indicate the significant role of NOTCH2 signaling in different tissues.

Correction to: A mutation in NOTCH2 gene first associated with Hajdu-Cheney syndrome in a Greek family: diversity in phenotype and response to treatment.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Comparative effectiveness of glucagon-like peptide-1 receptor agonists versus dipeptidyl peptidase-4 inhibitors on noninvasive indices of hepatic steatosis and fibrosis in patients with type 2 diabetes mellitus.

BACKGROUND AND AIMS: Nonalcoholic fatty liver disease (NAFLD) is highly prevalent in patients with type 2 diabetes mellitus (T2DM). There is currently no approved treatment for NAFLD. The main aim was the evaluation of the effect of glucagon-like peptide-1 receptor agonists (GLP-1 RA) vs. dipeptidyl peptidase-4 inhibitor (DPP-4i) treatment on noninvasive indices of hepatic steatosis and fibrosis in patients with T2DM. METHODS: In this retrospective study, three noninvasive indices of hepatic steatosis [HSI, NAFLD ridge score, and triglycerides (TG) to high-density lipoprotein cholesterol (HDL-C) ratio] and five of fibrosis (APRI, FIB-4, NAFLD fibrosis score, BAAT and BARD) were calculated before and after (6-18 months) the addition of a DPP-4i (n = 152) or a GLP-1 RA (n = 37) in patients with T2DM. RESULTS: Regarding steatosis indices, NAFLD ridge score was significantly decreased in the GLP-1 RA group (baseline: 0.90 ± 0.34, follow-up: 0.67 ± 0.24; p = 0.001), but not in the DPP-4i group (p = 0.25); the difference for group∗time interaction was significant (p = 0.02). HSI showed a trend between groups, being significantly different at baseline and follow-up (p < 0.001) with no significant difference in group∗time interaction. Indices of fibrosis were not essentially changed within or between groups. CONCLUSIONS: NAFLD ridge score was significantly decreased after the addition of GLP-1 RA in patients with T2DM. This study warrants further prospective clinical trials.

Association between vitamin D receptor gene polymorphisms and Graves' disease: a systematic review and meta-analysis.

PURPOSE: The pathogenesis of Graves' disease (GD) and orbitopathy (GO) is not completely elucidated. On the other hand, vitamin D receptor (VDR) gene polymorphisms have been associated with vulnerability to a plethora of chronic autoimmune diseases. The primary aim of this study was to synthesize evidence on the association between VDR gene polymorphisms and GD. Secondary aim was to investigate their association with GO. METHODS: A comprehensive search was conducted in PubMed, CENTRAL and Scopus, up to December 8, 2018. Data were expressed as odds ratio (OR) with 95% confidence intervals (CI). Heterogeneity was quantified with I2 index. RESULTS: Ten studies were included in the qualitative and quantitative analysis. TT subtype of TaqI polymorphism was associated with an increased risk of GD compared with Tt and tt polymorphisms (OR: 1.42; 95% CI, 1.05-1.94, p = 0.025), whereas tt was associated with a lower risk of GD, compared with TT and Tt polymorphisms (OR: 0.79; 95% CI, 0.62-0.99, p = 0.043). No association was found for ApaI, BsmI, and FokI polymorphisms. The bb subtype of BsmI polymorphism was associated with a lower risk in Asian, but with a higher GD risk in Caucasian populations, compared with BB/Bb subtypes. No eligible study was found regarding the association between VDR gene polymorphisms and the risk of GO. CONCLUSIONS: The TT subtype of the TaqI polymorphism was associated with a higher susceptibility for GD compared with Tt and tt. Regarding BsmI, the bb subtype was associated with increased GD risk in Caucasians, whereas it is protective in Asians.

Could Lipid Profile be Used as a Marker of Autonomous Cortisol Secretion in Patients with Adrenal Incidentalomas?

Adrenal incidentalomas (AIs) have been associated with an increased risk of metabolic syndrome and dyslipidemia, though evidence regarding the latter is limited. Lipid abnormalities in patients with AIs have been associated with subclinical hypercortisolism. The current study aims to test whether lipid profile in patients with AIs predicts "autonomous cortisol secretion" (ACS). Patients with AIs found on either computerized tomography (CT) or magnetic resonance imaging (MRI), were included in a prospective cohort study. All patients were followed up for at least three years. Alterations in their hormonal and lipid profiles were recorded. Ninety-four patients (69 women) harboring 111 AIs were included. There were no differences between patients with ACS and those without, with respect to their baseline lipid profile [total cholesterol, low-density-lipoprotein cholesterol (LDL-C), triglycerides, high-density lipoprotein cholesterol (HDL-C) and non-HDL-C] and blood pressure (systolic and diastolic). Non-HDL-C concentrations decreased over time (Repeated Measures ANOVA, p=0.013), despite patients' body mass index (BMI) remaining unchanged. Logistic regression analysis revealed that the only predictor of ACS was the size of AIs, as calculated by CT or MRI. The current study demonstrated that lipid profile at baseline or during follow-up cannot predict ACS in patients with AIs. However, larger AIs may have a greater probability of ACS.

Enduring remission of active and sight-threatening Graves' orbitopathy with rituximab: report of two cases.

Intravenous (i.v.) glucocorticosteroids (GCs) constitute the first-line treatment for active and moderate-to-severe Graves' orbitopathy (GO). In cases of persistent disease, rituximab, a monoclonal anti-CD20 antibody, may be used, although studies have yielded conflicting results. In case 1, a 50-year-old female heavy smoker presented with severe bilateral disfiguring eyelid edema of four months, bilateral exophthalmos and a clinical activity score (CAS) of 5/7. Laboratory investigation showed thyrotoxicosis and high thyroid-stimulating immunoglobulin (TSI) levels [32 IU/L (normal <1.75]. After minor improvement by i.v. methylprednisolone and standard retrobulbar radiotherapy (20 Gy), her visual acuity progressively declined to "hand motion". Rituximab was administered (two pulses of 500 mg, two weeks apart), with significant response. At 3 1/2 years of follow-up, CAS is 0/7 and CD20+ lymphocytes remain at the lower normal range. In case 2, a 78-year-old non-smoker male was referred for management of severe active GO, one month after total thyroidectomy for Graves' thyrotoxicosis (TSI: 6.74 IU/L). Over the preceding two-three months, severe GO manifested with chemosis, constant diplopia, loss of color vision and acuity of 1/10 bilaterally (CAS: 7/7). Following partial response to i.v. methylprednisolone and concomitant radiotherapy, rituximab (two pulses of 500 mg each, two weeks apart), was administered. Vision partially recovered and GO remains in remission one year later, even after 131I (100 mCi) administration for papillary thyroid carcinoma (TSI: 0.9 IU/L and CD20+ count at the lower normal range). In conclusion, rituximab may be an effective second-line therapy in GO patients, providing long-lasting remission.

Comparative Effect of Atorvastatin and Rosuvastatin on 25-hydroxy-Vitamin D Levels in Non-diabetic Patients with Dyslipidaemia: A Prospective Randomized Open-label Pilot Study.

AIMS: Low 25-hydroxy-vitamin D [25(ΟΗ)D] levels have been associated with increased risk for cardiovascular disease. Conflicting data exist regarding the effect of statins on [25(OH)D] levels. The aim of this study was to compare the effect of atorvastatin and rosuvastatin on 25(OH)D levels in non-diabetic patients with dyslipidaemia. METHODS: This was a prospective randomized open-label study. Patients were assigned to atorvastatin 20 mg/day (n=28, age: 56.1±2.2 years, 22 females) or rosuvastatin 10 mg/day (n=24, age: 57.4±1.9 years, 20 females). Total cholesterol (TC), low- (LDL-C) and high-density lipoprotein cholesterol (HDL-C), triglycerides (TG), fasting plasma glucose, insulin, glycosylated haemoglobin A1c (HbA1c) and high sensitivity C-reactive protein (hsCRP) levels were measured, and homeostatic model of assessment insulin resistance (HOMA-IR) was calculated at baseline and 12 weeks post-treatment. RESULTS: There were no within or between group significant differences in 25(OH)D levels (atorvastatin: 21.7±1.9 ng/ml at baseline and 23.5±2.3 ng/ml at week 12; rosuvastatin: 25.3±1.8 and 27.0±2.4 ng/ml, respectively; p=0.172 and p=0.306 for between groups, respectively). Both statins significantly reduced TC, TG and LDL-C levels, with a greater LDL-C reduction being observed by rosuvastatin. CONCLUSION: Atorvastatin and rosuvastatin did not significantly affect 25(OH)D levels in this study.

Denosumab treatment for juvenile Paget's disease: results from two adult patients with osteoprotegerin deficiency ("Balkan" mutation in the TNFRSF11B gene).

CONTEXT: Most patients with juvenile Paget's disease (JPD) have homozygous loss-of-function mutations in the TNFRSF11B gene resulting in osteoprotegerin deficiency. Because recombinant osteoprotegerin is not available for clinical use, an alternative therapeutic approach could be denosumab, which acts on the same pathway. MAIN OBJECTIVE: The aim was to study the effect of denosumab on bone turnover markers in two adult patients with JPD ("Balkan" mutation) previously treated with calcitonin and bisphosphonates. SETTING: The study was conducted at two tertiary hospitals in Greece. PATIENTS: Patient 1 (a 36-year-old woman) developed a severe and long-term hypocalcemia after a single dose (3.5 mg) of zoledronic acid. Her bone disease remained active despite treatment. Patient 2 (a 67-year-old man) had satisfactorily controlled bone disease with only intermittent risedronate treatment during the last 10 years, but suffered from progressive loss of hearing and vision. Low doses (20-40 mg) of denosumab every 3-6 months were administered in both patients. RESULTS: Bone markers (including total and bone-specific alkaline phosphatase, procollagen I N-terminal peptide, and osteocalcin) were reduced to normal levels in both patients, with nadir observed 2-4 months after each denosumab injection. Retinal and hearing involvement remained unchanged, but patient 2 developed a rapid progression of cataract in the right eye. CONCLUSIONS: Low-dose denosumab every 3-6 months for about 2 years in two patients with JPD successfully controlled their bone disease. The long-term effect of denosumab on the nonskeletal complications remains to be elucidated.

Severe eyelid oedema in Graves' ophthalmopathy.

We report a case of severe eyelid oedema due to Graves' ophthalmopathy (GO). The aim was to present a case report and review of the literature about eyelid oedema due to GO. The case report includes a history of patient data and literature review. The patient was offered intravenous methylprednisolone and gave consent. A dosage of 500 mg intravenous methylprednisolone once weekly for 6 weeks, followed by 250 mg intravenous methylprednisolone once weekly for 6 weeks, with a total treatment period of 12 weeks was given. Up to day, minor improvement has been observed. Severe eyelid oedema due to GO is a rare manifestation of Graves' disease. In cases of active and moderate-to-severe disease, treatment with intravenous glucorticoids is recommended alone or with orbital radiotherapy, followed by rehabilitative surgery.

Neck abscess associated with a piriform fossa sinus tract in an adult.

Perithyroidal inflammatory masses deriving from developmental remnants of the 3rd and 4th brancial pouches are rare. We report a case of an otherwise healthy 31-year-old man, who presented with fever and swelling of the left side of the neck. Neck ultrasound showed a displaced left thyroid lobe with induration of the strap muscles. Fine-needle aspiration biopsy revealed pus, positive for Staphylococcus aureus species. Antibiotic treatment led to rapid clinical improvement and reversal of imaging features, without the need for surgical intervention. This rare clinical entity should be always considered in the differential diagnosis of an inflammatory neck mass.

The effect of monthly ibandronate on bone mineral density and bone turnover markers in patients with haemophilia A and B and increased risk for fracture.

Haemophilia A and B have been associated with increased prevalence of low bone mineral density (BMD). However, no study has so far evaluated the effects of anti-osteoporotic therapy on BMD in haemophilia.The primary endpoint of this prospective study was to estimate the effect of 12-month therapy of oral ibandronate 150 mg/month on BMD in patients with haemophilia A and B. Secondary endpoint was its effect on turnover markers (BTM) of bone resorption [serum C-terminal telopeptide of type 1 collagen (sCTX), tartrate-resistant acid phosphatase band 5b] and bone formation (osteocalcin and bone-specific alkaline phosphatase. Ten adult patients with T-score < -2.5 SD or Z-score < -2 and/or increased risk of fracture according to FRAX model were included. All received 1,000 mg/day calcium carbonate with 800 IU/d cholecalciferol. Males with haemophilia A (n=7) or B (n=3) (mean age 43.5 ± 13.5 years) were studied. Ibandronate resulted in an increase in lumbar BMD (from 0.886 ± 0.169 to 0.927 ± 0.176 g/cm2, 4.7%, p=0.004). No change in BMD of total hip (from 0.717 ± 0.128 to 0.729 ± 0.153 g/cm2, p=0.963) or femoral neck (0.741 ± 0.135 to 0.761 ± 0.146 g/cm2, p=0.952) was noticed. Ibandronate led to a decrease in sCTX (from 0.520 ± 0.243 to 0.347 ± 0.230 ng/ml, -29.9%, p=0.042). No change was observed in other BTM. Ibandronate was generally well-tolerated. In conclusion, ibandronate significantly improved BMD in lumbar spine and reduced bone resorption in adults with haemophilia at increased risk of fracture. Its effect on hip BMD and bone formation markers was not significant.

Predictors of long-term remission in patients with Graves' disease: a single center experience.

Antithyroid drugs (ATDs) remain the first-line therapy in patients with Graves' disease (GD), despite a high relapse rate. The purpose of this study was to identify the predictors of remission in patients with GD treated with ATDs-retrospective study at an endocrine referral service in Northern Greece. Two-hundred and eleven patients met the study's criteria. Females (p = 0.049), non-smokers (p = 0.017), patients without ophthalmopathy (p = 0.033), and those developing pharmaceutical hypothyroidism (p = 0.018) experienced longer duration of remission. Duration of remission was positively associated with therapy duration (r s = 0.151, p = 0.030), maximum TSH levels during (r s = 0.241, p = 0.001), at the end (r s = 0.280, p < 0.001) and 3 months after therapy (r s = 0.341, p = 0.003). There was a negative association with free T4 (FT4) (r s = -0.426, p < 0.001) and free triiodothyronine (FT3) (r s = -0.467, p = 0.038) levels at 6 months after ATDs discontinuation. In multiple-regression analysis, only duration of the first ATDs course for more than 24 months independently predicted duration of remission. Female gender, non-smoking, the absence of orbitopathy, treatment duration, pharmaceutical hypothyroidism, higher TSH levels during, at the end and 3 months after ATDs discontinuation, and lower FT4 and FT3 levels 6 months after therapy were associated with longer duration of remission. However, only duration of ATDs therapy for more than 24 months independently predicted predict long-term remission in GD.

11beta-Hydroxysteroid dehydrogenase type 1 inhibitors: novel agents for the treatment of metabolic syndrome and obesity-related disorders?

OBJECTIVE: Metabolic syndrome (MetS) and Cushing's syndrome share common features. It has been proposed that increased glucocorticoid activity at peripheral tissues may play a role in the pathogenesis of MetS and obesity-related disorders. It is well-known that intracellular cortisol concentrations are determined not only by plasma levels but also by the activity of 11ß-hydroxysteroid dehydrogenase type 1 (11ß-HSD1) which catalyzes the conversion of inactive cortisone to active cortisol, especially in the liver and adipose tissue. Another isoenzyme exists, the 11ß-hydroxysteroid dehydrogenase type 2, which acts in the opposite direction inactivating cortisol to cortisone in the kidney. This review considers the significance of the 11ß-HSD1 inhibition in the treatment of several features of MetS and provides current data about the development of 11ß-HSD1 inhibitors, as new agents for this purpose. MATERIALS/METHODS: Using PubMed, we searched for publications during the last 20years regarding the development of 11ß-HSD1 inhibitors. RESULTS: Emerging data from animal and human studies indicate an association of 11ß-HSD1 over-expression with obesity and disorders in glucose and lipid metabolism. This has led to the hypothesis that selective inhibition of 11ß-HSD1 could be used to treat MetS and diabetes. Indeed, natural products and older agents such as thiazolidinediones and fibrates seem to exert an inhibitory effect on 11ß-HSD1, ameliorating the cardiometabolic profile. In view of this concept, novel compounds, such as adamantyltriazoles, arylsulfonamidothiazoles, anilinothiazolones, BVT2733, INCB-13739, MK-0916 and MK-0736, are currently under investigation and the preliminary findings from both experimental and human studies show a favourable effect on glucose and lipid metabolism, weight reduction and adipokine levels. CONCLUSIONS: Many compounds inhibiting 11ß-ΗSD1 are under development and preliminary data about their impact on glucose metabolism and obesity-related disorders are encouraging.

Location, number and morphology of parathyroid glands: results from a large anatomical series.

Surgical management of parathyroid gland disease may sometimes be difficult, due mainly to the surgeon's failure to successfully detect parathyroids in unusual locations. The records of 942 cadavers (574 men and 368 women) who underwent autopsy in the Department of Forensic Medicine in Athens during the period 1988-2009 were reviewed. In total, 3,796 parathyroid glands were resected and histologically verified. Parathyroid glands varied in number. In 47 cases (5 %), one supernumerary (fifth) parathyroid was found, while in 19 cases (2 %) three parathyroid glands found. Superior glands were larger than inferior ones. However, there was no significant difference between the genders with respect to gland size. In 324 (8.5 %) out of 3,796, the glands were detected in an ectopic location: 7 (0.2 %) in the thyroid parenchyma, 79 (2 %) in different sites in the neck and 238 (6.3 %) in the mediastinum, 152 (4.1 %) of which were found in the upper and 86 (2.2 %) in the lower mediastinum. Significant anatomical variations of normal parathyroid glands may exist regarding number and location-knowledge that is essential for their successful identification and surgical management.

Long term follow-up of patients with prolactinomas and outcome of dopamine agonist withdrawal: a single center experience.

Dopamine agonists (DA) are the mainstay of treatment for patients with prolactinomas. To describe the efficacy of treatment and the outcomes of DA withdrawal. Retrospective review of electronic medical records of patients with prolactinomas from 1985 to 2009. Seventy-nine patients (17 men/62 women), aged 35.3 ± 1.6 years at diagnosis were studied. The mean follow-up time was 84.7 ± 9.2 months (range 0-336). The mean initial size of microadenomas was 0.74 ± 0.10 cm (range 2.41 ± 0.39) and of macrodenomas 2.41 ± 0.39 cm (range 1.1-8) and serum prolactin (PRL) levels were 112 ± 19 and 263 ± 59 ng/ml, respectively (normal range 0-40). Fifty-one (65%) prolactinomas were micro- and 28 (35%) were macroadenomas. DA led to a decrease in adenoma size in 71% of them, while 53% of microadenomas were not visible during follow-up. In 26 patients, DA withdrawal was decided. After therapy of >24 months and a mean follow-up time of 49 ± 11 months (range 3-168), 15 subjects (58%) showed no recurrence of hyperprolactinemia. Higher remission rates, although not statistically significant, were observed with cabergoline (75%). The mean PRL levels before DA discontinuation were 12.2 ± 2.3 ng/ml (range 0.5-44.7) and after discontinuation they were significantly lower than pre-treatment values. Recurrence of hypeprolactinemia was evident during the first year in all but one patient. Remission rates were not associated with age or size of adenoma at diagnosis, initial or before DA discontinuation PRL levels and duration of treatment. DA withdrawal was followed by remission of hyperprolactinamia in about half of patients after >2 years of treatment.