Acta endocrinologica (Bucharest, Romania : 2005)最新文献_第2页

PERSISTENT ASYMPTOMATIC SEVERE HYPOGLYCAEMIA DUE TO TYPE 0A GLYCOGENOSIS - GENERAL AND ORO-DENTAL ASPECTS. 由0a型糖原病引起的持续性无症状严重低血糖-一般和口腔-牙齿方面。

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Acta endocrinologica (Bucharest, Romania : 2005) Pub Date : 2019-10-01 DOI: 10.4183/aeb.2019.526

L Matei, M I Teodorescu, A Kozma, A D Iordan Dumitru, S M Stoicescu, S Carniciu

{"title":"PERSISTENT ASYMPTOMATIC SEVERE HYPOGLYCAEMIA DUE TO TYPE 0A GLYCOGENOSIS - GENERAL AND ORO-DENTAL ASPECTS.","authors":"L Matei, M I Teodorescu, A Kozma, A D Iordan Dumitru, S M Stoicescu, S Carniciu","doi":"10.4183/aeb.2019.526","DOIUrl":"https://doi.org/10.4183/aeb.2019.526","url":null,"abstract":"Background: Type 0 glycogenosis is a genetic metabolic disorder characterized by the absence of glycogen synthesis of hepatic synthase and hence of liver glycogen stores in normal amounts. It is an extremely rare condition.Case study: This case is a 5-year and 11-month-old female child with asymptomatic severe hypoglycemia in the last two years. During the admission and afterwards, an extensive panel of paraclinical and imaging investigations was carried out to diagnose and document the case, which led to the specific genetic test. The result was positive for 2 heterozygous mutations in the GYS2 gene (hepatic glycogen synthase), the p.547C> T mutation was pathogenic (class 1) and c.465del, frameshift likely pathogenic (class 2). In order to integrate the clinical picture of patients with this condition and to establish potential correlations regarding the specific aspects with the general development and the phenotype, the oro-dental status was investigated.Conclusion: The investigations showed a positive correlation with literature data in several respects: low stature, hypoglycemia with hyperketonemia but normal plasma lactate, postprandial and contradictory hyperglycemia, delayed bone development, etc. Oro-buco-maxillary aspects showed a slight delay in the dental eruption. Dietary therapy and stricter dental care and additional prophylaxis are required.","PeriodicalId":520542,"journal":{"name":"Acta endocrinologica (Bucharest, Romania : 2005)","volume":" ","pages":"526-530"},"PeriodicalIF":1.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200112/pdf/aeb.2019.526.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37909580","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

ARE THERE RELIABLE PREDICTORS FOR THE IMPAIRED QUALITY OF LIFE IN PATIENTS WITH CUSHING'S SYNDROME? 库欣综合征患者生活质量受损是否有可靠的预测因素?

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Acta endocrinologica (Bucharest, Romania : 2005) Pub Date : 2019-10-01 DOI: 10.4183/aeb.2019.482

A B Nankova, M Yaneva, A Elenkova, K Kalinov, S Zacharieva

{"title":"ARE THERE RELIABLE PREDICTORS FOR THE IMPAIRED QUALITY OF LIFE IN PATIENTS WITH CUSHING'S SYNDROME?","authors":"A B Nankova, M Yaneva, A Elenkova, K Kalinov, S Zacharieva","doi":"10.4183/aeb.2019.482","DOIUrl":"https://doi.org/10.4183/aeb.2019.482","url":null,"abstract":"Context: Patients with Cushing's syndrome (CS) of any etiology experience a number of physical and psychological symptoms which impact negatively on health-related quality of life (HRQoL).Subjects and methods: HRQoL was measured using CushingQoL questionnaire.Results: The first part of our study was a cross-sectional analysis of 141 patients with CS over a 10-year period. CushingQoL score was lower in pituitary CS compared to adrenal CS. Remission and older age were associated with better outcome on item 7 (physical appearance anxiety). In a multivariate regression analysis after adjustment for etiology, remission status, age, UFC, duration of hypercortisolism and presence of hypercortisolism-associated comorbidities the female gender was the only negative predictor associated with poorer outcome on each of the three scores. The presence of hypercortisolism-associated comorbidities independently predicted poorer outcome on the psychological and the global subscales.The second part of our research was a prospective study of 27 patients with adrenal adenoma. Achievement of remission independently predicted improvement of the total score of any patient.Conclusion: Studying in details and understanding the mechanisms of the impaired HRQoL in patients with CS is the only way to become aware of the problem and create methods that could help these patients.","PeriodicalId":520542,"journal":{"name":"Acta endocrinologica (Bucharest, Romania : 2005)","volume":" ","pages":"482-490"},"PeriodicalIF":1.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200113/pdf/aeb.2019.482.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37909689","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

THYROID SURGERY, IONM AND SUGAMMADEX SODIUM RELATIONSHIPS: BENEFITS IN SUGAMMADEX SODIUM USE FOR IONM. 甲状腺手术，离子和糖madex钠的关系:糖madex钠用于离子的益处。

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Acta endocrinologica (Bucharest, Romania : 2005) Pub Date : 2019-10-01 DOI: 10.4183/aeb.2019.454

T Donmez, V M Erdem, O Sunamak, H Ozcevik

{"title":"THYROID SURGERY, IONM AND SUGAMMADEX SODIUM RELATIONSHIPS: BENEFITS IN SUGAMMADEX SODIUM USE FOR IONM.","authors":"T Donmez, V M Erdem, O Sunamak, H Ozcevik","doi":"10.4183/aeb.2019.454","DOIUrl":"https://doi.org/10.4183/aeb.2019.454","url":null,"abstract":"Background: It is important to protect recurrent laryngeal nerve (RLN) during thyroid surgery. Thus, intra- operative neuromonitoring (IONM) has got popularity. But, the half life of neuromuscular blocking agents used has a reverse correlation with reliability and effectiveness of IONM. This study aimed to research the effect of Sugammadex Sodium, a specific nemuromuscular blocking agent antagonist, on nerve conduction and IONM.Materials and methods: Twenty patients who underwent thyroidectomy under IONM followed an enhanced NMB recovery protocol-rocuronium 0.6 mg/kg at anesthesia induction and sugammadex 2 mg/kg at the beginning of operation. To prevent laryngeal nerve injury during the surgical procedures, all patients underwent intraoperative monitoring. At the same time, the measurement of TOF-Watch acceleromyograph of the adductor pollicis muscle response to ulnar nerve stimulation was performed; recovery was defined as a train-of-four (TOF) ratio ≥ 0.9. Age, sex, recurrent laryngeal nerve transmission speeds prior to and after operation, BMI, duration of surgery, the change in nerve transmission after drug administration and complications were analyzed.Results: The mean age and the mean BMI were 47.6±11.82 years and 28.74±3.20, respectively. The mean operation duration was 52.65±5.51 minutes. There was no difference in either right or left RLN monitoring values before and after surgery. Following the drug injection, the TOF guard measurements on the 1st, 2nd, 3rd and 4th minutes were 23.5±4.90; 69.5±6.86; 88±4.1 and 135.9±10.62, respectively.Conclusion: Neuromuscular blocking antagonist use and monitoring nerve transmission speed with TOF-guard can provide a safer resection.","PeriodicalId":520542,"journal":{"name":"Acta endocrinologica (Bucharest, Romania : 2005)","volume":" ","pages":"454-459"},"PeriodicalIF":1.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200106/pdf/aeb.2019.454.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37908127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

ARE THYROID NODULES AN OBSTACLE TO MINIMAL INVASIVE PARATHYROID SURGERY? A SINGLE-CENTER STUDY FROM AN ENDEMIC GOITER REGION. 甲状腺结节是微创甲状旁腺手术的障碍吗?地方性甲状腺肿地区的单中心研究。

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Acta endocrinologica (Bucharest, Romania : 2005) Pub Date : 2019-10-01 DOI: 10.4183/aeb.2019.531

S Özden, B Saylam, G Daglar, Y N Yuksek, M Tez

{"title":"ARE THYROID NODULES AN OBSTACLE TO MINIMAL INVASIVE PARATHYROID SURGERY? A SINGLE-CENTER STUDY FROM AN ENDEMIC GOITER REGION.","authors":"S Özden, B Saylam, G Daglar, Y N Yuksek, M Tez","doi":"10.4183/aeb.2019.531","DOIUrl":"https://doi.org/10.4183/aeb.2019.531","url":null,"abstract":"Context: Minimally invasive parathyroidectomy (MIP) procedure has become a widely accepted alternative to the standard four-gland exploration nowadays.Objective: The aim of this study was to evaluate patients with primary hyperparathyroidism (PHPT), who had been treated with thyroidectomy and bilateral neck exploration (BNE), rather than MIP alone, due to coexisting thyroid nodules and to determine the benefits of simultaneous thyroidectomy and the possible negative outcomes of not performing this additional procedure.Design: There were 185 patients who were operated for PHPT at our clinic from January 2014 to November 2016.Subjects and methods: 50 patients meet inclusion criteria: have thyroidectomy at the same time of parathyroid surgery, have concordant findings of parathyroid adenoma localization at preoperative MIBI-SPECT and the cervical US and have not had malignancy on fine needle aspiration biopsy (FNAB).Results: The mean age of the patients was 55.3±10.4, and female to male ratio was 7:1. All patients had parathyroidectomy with BNE and thyroidectomy: 11 (22%) patients had micropapillary thyroid cancer (mPTC), 2 (4%) had papillary thyroid cancer (PTC).Conclusion: The results were inconclusive in clearly demonstrating which patients presenting with coexisted thyroid nodules should undergo thyroidectomy, rather than MIP, and which should be monitored for thyroid nodules after MIP. However, we consider that in cases who are not clearly indicated for thyroidectomy, MIP followed by monitoring of thyroid nodules can be the treatment approach.","PeriodicalId":520542,"journal":{"name":"Acta endocrinologica (Bucharest, Romania : 2005)","volume":" ","pages":"531-536"},"PeriodicalIF":1.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200102/pdf/aeb.2019.531.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37909582","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

METHIMAZOLE-INDUCED AGRANULOCYTOSIS AND SEPSIS: WAS THYROID STORM PRESENT OR JUST BEING MIMICKED? 甲巯咪唑诱导的粒细胞缺乏症和败血症:甲状腺风暴是存在的还是只是模仿的?

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Acta endocrinologica (Bucharest, Romania : 2005) Pub Date : 2019-10-01 DOI: 10.4183/aeb.2019.522

M A M Stumpf, G C A Schrut, M Ramthun, S Onuma, H E C G Osternack

{"title":"METHIMAZOLE-INDUCED AGRANULOCYTOSIS AND SEPSIS: WAS THYROID STORM PRESENT OR JUST BEING MIMICKED?","authors":"M A M Stumpf, G C A Schrut, M Ramthun, S Onuma, H E C G Osternack","doi":"10.4183/aeb.2019.522","DOIUrl":"https://doi.org/10.4183/aeb.2019.522","url":null,"abstract":"Introduction: Agranulocytosis induced by thioamides is rare, occurring only in 0.2-0.5% of cases.Case presentation: We present the case of a 45-year-old woman previously diagnosed with Graves' disease that discontinued the use of methimazole on her own. She attended the Emergency Department presenting fever (40.5¯C), agitation and diaphoresis. A thyroid storm diagnosis was initially thought, but after laboratory results showing neutrophil count near 0.06x109/L, sepsis due to neutropenia seemed the most logical hypothesis. Cephepime was promptly initiated. For thyrotoxicosis management, cholestyramine and atenolol were prescribed. In her second day of hospitalization, subcutaneous granulocyte colony-stimulating factor was started for an earlier medullar response. The patient was discharged after 7 days with atenolol 50mg/day and instructed to have a definite treatment for Graves disease as soon as possible.Conclusion: Such case purpose is to remember clinicians that sepsis diagnosis can be challenged, especially when a thyroid storm is a possible diagnosis as well. In this particular case, both conditions should be treated, but life-threatening sepsis should have the focus for a quick therapeutic approach.","PeriodicalId":520542,"journal":{"name":"Acta endocrinologica (Bucharest, Romania : 2005)","volume":" ","pages":"522-525"},"PeriodicalIF":1.0,"publicationDate":"2019-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200104/pdf/aeb.2019.522.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37910123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4