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Pituitary Adenomas in Multiple Endocrine Neoplasias Type1 and Other Endocrine Neoplasias Syndromes
No full textMultiple Endocrine Neoplasias (MEN) are very rare genetic syndromes. Pituitary adenomas (PA) are usually sporadic, but can be observed in MEN type 1 and in other genetic syndromes such as Carney’s complex syndrome, MEN type 4 (MEN4), and some hereditary pheochromocytomas/paragangliomas syndromes (HPPS).Our aim was to analyze PA’s frequency and characteristics in aforementioned syndromes observed in our department.Material and Methods: The retrospective study analyzed frequency and characteristics of PA associated to typical and atypical MENs. We excluded incomplete files and MEN2, as research for PA was not systematic, and we looked for MEN2 in PA too. The patients did not undergo genetic testing.Results: Among ten hereditary syndromes (six MEN1 and four atypical MENs: either NEM4 or HPPS), we observed eight PA =80%. Four PA were associated with MEN1 and four with non-classical MENs. PA revealed MENs in four cases. PA median height was 19.88mm (7-50). Six/eight were macro tumors (≥1cm in height), with cavernous sinus invasion in two. PA types were prolactinomas (two), somatotroph adenomas (two), gonadotroph adenoma (one) and non-secreting tumors (three). For the follow up, only two out of six treated patients achieved remission.Conclusion: In this series, PA were frequent (80%) in MEN1 and non-classical MENs. Macro adenomas and secreting tumors were prevailing with a variable aggressiveness. These syndromes genetically determined should have systematic genetic screening to specify MENs types and for rapid diagnosis and early management of affected patients and their families
Case Report: Insula Carcinoma of the Thyroid Combined with Hyperthyroidism and Revealed by Orbital and Osseous Metastases
No full textIt is unusual to find an association between thyroid cancer and hyperthyroidism. The coexistence of insular carcinoma which is a poorly differentiated tumor with toxic nodular goiter is rarely described. The insular carcinoma is very rare, which results in a poor prognosis in front of precocious distant metastases, yet the orbital location of metastases is exceptional. We report here a unique clinical case involving hyperthyroidism and insular carcinoma revealed by orbital and spinal metastases
Dominant-Negative Constructs of IRE-1alpha as an Effective way to Suppression of Tumor Growth through the Inhibition of Cell Proliferation
No full textActivation of cell proliferation and angiogenesis as well as the down-regulation of apoptosis are important for tumor growth through pathways of the unfolding protein response/endoplasmic reticulum stress, a fundamental phenomenon for secure protection of cells by maintaining the functional integrity of the endoplasmic reticulum. It is mediated by three sensor and signaling pathways: IRE-1a/ERN1 (inositol-requiring enzyme-1a/endoplasmic reticulum to nuclei 1), ATF6 (activating transcription factor 6), and PERK (double stranded RNA activated protein kinase (PKR)-like endoplasmic reticulum kinase (PERK). All three arms of the unfolded protein response are important for tumor cell survival and growth especially under hypoxic conditions, but the unfolded protein response signaling is mainly mediated through the ERN1 pathway.The inhibition of ERN1 by its dominant-negative constructs leads to a decrease of tumor growth through suppression of angiogenesis and cell proliferation as well as activation of apoptosis and tumor suppressors. Data concerning the molecular mechanisms of the effect a blockade of ERN1 signaling enzyme has on glioma growth is analyzed, including the expression of genes controlling angiogenesis, cell proliferation, cell cycle, and apoptosis. Moreover, the inhibition of ERN1 endoribonuclease only has more profound effect on the expression of most key regulatory genes as well as on cell proliferation than the blockade of both kinase and endonuclease activity of ERN1 in glioma cells. In conclusion, the inhibition ofERN1/IRE-1alpha coordinately regulated factors involved in tumor growth, lowering expression levels of pro-proliferative, pro-angiogenic and anti-apoptotic factors and enzymes and up-regulated the expression of anti-proliferative, anti-angiogenic and pro-apoptotic factors in a trend towards the level of these transcription factors in normal human astrocytes. These review attempts to summarize recent advances in the role of inhibition of ERN1 signalingby dominant/negative strategies in regulation of proliferation and apoptosis related genes and suppression of tumor growth, which will help to define the best therapeutic targets for the design of potent antitumor drug
Assessing Patient Reported Outcomes after Septorhinoplasty with and without Nasal Packing Using the Glasgow Benefit Inventory
No full textIntroduction: Patient-reported outcomes are an invaluable tool to guide clinical decision-making. Nasal packing is a traumatic, painful event for patients, which could negatively impact patient-reported outcomes. We aimed to evaluate if avoiding postoperative nasal packing in septorhinoplasty and rhinoplasty affects patient-reported outcomes.Methods: All septorhinoplasty and rhinoplastyoperations performed over 5 years were identified. The case notes were reviewed to obtain patient demographics, operative details and any post-operative complications. The Glasgow Benefit Inventory (GBI) was administered via telephone to measure patient-reported outcomes.Results: 167 patients were identified. In 11 the case notes were unavailable. Of the 156 patients remaining, 126 completed the GBI questionnaire (who had undergone 132 operations). No significant difference in GBI scores was found with or without the use of nasal packing.Conclusion: This study suggests that routine nasal packing can be avoided in the majority of patients, but can be used with confidence that patient-reported outcome is not being compromised
Clinical Application of Partial and Total Ossicular Replacement Prostheses of Hydroxyapatite Based Scaffolds Cultured with Human Mesenchymal Stromal Cells
No full textObjective: To evaluate the feasibility of hydroxyapatite PORP and TORP as a scaffold with human mesenchymal stromal cells (hMSCs) and their use as replacement prosthesis during ossiculoplasty.Study Design: Application of tissue engineering in a prospective clinical study.Subjects and Methods: This study was conducted in 8 patients (6 canal wall down and 2intact canal wall tympanomastoidectomy) between April 2008 and June 2009.Prosthesis Preparation: In all the cases, 2 days before transplantation, hydroxyapatite implants were loaded by the cells obtained from culture obtained from patients’ own bone marrow. The cell suspension was applied to seed on scaffolds placed in 12-well tissue culture plates at a density of 2×106cells/scaffold. All pre and post operative audiometric evaluations (pure tone air and bone conduction thresholds) were carried out in the same center and by the same audiologist before and 3 months, 6 months , and one year after the operation.Setting: Otolaryngology, head and neck surgery department in a tertiary academic medical center.Results: The hMSCs were positive for CD10, CD44, CD166, CD106, HLA-ABC, CD90, CD54, and CD105, but were negative for CD34, CD45, CD117, and CD31. Mean air-bone gap closure was 19.57 dB. There was no sign of prosthesis extrusion at the end of twelve month follow-up period in all cases.Conclusion: The idea of tissue-engineered ear ossicles is a feasible and interesting option for the replacement of the ear ossicles. However, the final outcome needs longer follow-ups
Efficacy of Orange Peel Extract in Psoriasis
No full textMonoterpenes, and especially d-Limonene, display anti-inflammatory and anti-angiogenic effects which could be efficient in such inflammatory diseases as psoriasis. A pilot study was initiated to test this hypothesis in an open non-randomized setting enrolling nine patients. Orange peel extract containing known amounts of d-Limonene were given twice daily as soft gel capsules ingested and/or emptied and massaged on skin lesions, for 45 days. In spite of the small size of the cohort, highly significant positive effects were observed, with a reduction of clinical scores such as Psoriasis Area Severity Index and Videodermoscopy Scalp Psoriasis Severity Index. Moreover, adding to the global subjective satisfaction of the patients and improvement of the objective Dermatology Life Quality Index was also recorded. This encouraging pilot study should serve to prompt a larger randomized blind study. Indeed, implementation of a non-toxic substance of natural origin in the widespread condition of psoriasis could represent a significant advance in the treatment of this disease
HAIR-AN Syndrome Associated with Scleroderma
No full textThe HAIR-AN syndrome is a rare multisystem disorder in women, that consists of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN). The IR is likely due to a primary defect of the insulin receptor. We report the case of a 25-year-old Caucasian woman with HAIR-AN syndrome, impaired glucose tolerance (IGT), mild hyperlipemia, and hypertension, associated with sclerodermatous skin changes on the extremities and trunk. Though scleroderma may coexist with other autoimmune diseases, its presentation with HAIR-AN syndrome has not yet been described
Total Parathyroidectomy without Autotransplantion Effective Treatment in Renal “Refractory†Chronic Kidney Disease - Metabolic Bone.
No full textIntroduction: Aim of our study attempts to demonstrate the superiority and benefits of total parathyroidectomy without autotransplantation (TPtx) versus subtotal parathyroidectomy (STPtx) or total parathyroidectomy with autotransplantation (TPtx+At) in cases of refractory, adenomised “renal†hyperparathyroidism, actually renamed Chronic Kidney Disease – Metabolic Bone Disease (CKD – BMD).Patients Study Design: Sixtytwo cases with CKD – MBD underwent surgery between 1994-2013. There were 25 SPtx and 9 TPtx+At both procedures determining 7 (18,4%) recurrences so in the last decade we performed preponderantly TPtx in 28 selected patients (12 males and 16 females, with median age of 43,6 (range) 22 – 66 years and median hemodialysis before surgery of 8,5 (range 3-12) years. Demography, clinical and biological data, imaging procedures, surgical protocols and pathology reports together with immediate and long-term results were recorded.Results and Discussions: Main indications for TPtx were severe bone disease, soft tissue calcifi-cations, neuromuscular features, grossly elevated iPTH and sometimes hypercalcemia. TPtx were done in 27 subjects in the 28th one suffering a completion PTx one year after outward exeresis of only two gland. One patient required a re-exploration for a cervical hematoma but none presen-ted permanent hypocalcemia or recurrent hyperparathyroidism. Pathology revealed nodular hyperplasia in all cases but a parathyroid carcinoma of the fourth gland and an incidentally papi-llary thyroid microcarcinoma were found in each one case. Follow-up at 6-60 months showed in 24 cases (87,7%) an effective clinical and biological improvement stable over time, continuing their hemodialysis regimen.Conclusions: TPtx alone proved to be an an equally safe and successful as another techniques currently used in management of CKD – MBD eliminating the hyperparathyroid status but being superior in regard to apparition of recurrences. The procedure is indicated especially in patients with aggressive, refractory forms of this condition without the prospect of renal transplant
Evolution of Radiotherapy for the Prostate Cancer Treatment
No full textProstate cancer is considered as one of the most frequently diagnosed cancer among men in the USA and world. In the last 10 years, there has been an increasing use of radiotherapy for prostate cancer treatment. This article briefly summarizes latest radiotherapy techniques that are currently used for the prostate cancer treatment
EFFICACY OF MANIPAL MANUAL OF SWALLOWING ASSESSMENT IN IDENTIFYING ASPIRATION
No full textBackground: There are also many formal assessment tools available for the assessment of swallowing. Among these, there are few published clinical dysphagia assessment tools that provide detailed assessment of swallowing. One of the tool which has been psychometrically validated and standardized for the Indian population is Manipal Manual for Swallowing Assessment (MMSA) which involves the assessment of structure, sensory and motor functions along with trial feed. This test is used in the present study to check for its efficacy in the identification of aspiration.Method: A total of 25 individuals participated in the study. All the individuals were diagnosed to have Dysphagia based on MMSA [35]. All the 25 individuals underwent MMSA test. Individuals with suspected aspiration and no aspiration were identified by experienced speech pathologists in the field of swallowing disorders. All these individuals were subjected to videofluroscopy testings to confirm aspiration. Concurrent validity for MMSA was established through the measurement of sensitivity, specificity, positive predictive value, negative predictive value and the efficiency.Results: Results revealed that 77% of the individuals with aspiration were actually aspirators whereas 67 % of the predicted individuals with no aspiration were actually the non aspirators and hence this test can be used to identify individuals with aspiration with overall efficiency of 72 %.Conclusions: Results revealed a high sensitivity and specifivicity for MMSA. Thus, MMSA is proven a valuable tool in the dysphagia assessment, in particularly aspiratio