Pub Date : 2015-02-25DOI: 10.4172/2157-7536.1000.150
Xinxin Zhang, Jinru Liang, Yongming Zhang, Jian-li Liu, Wen-ji Sun, Y. Ito
Steroid saponins from Dioscorea zingiberensis C.H.Wright were separated for the first time using two chromatographic methods for comparison: counter-current chromatography (CCC) coupled with evaporative light scattering detector (ELSD) and preparative reversed phase high-performance liquid chromatography (RP-HPLC) with an ultraviolet detector. Ethyl acetate-n-butanol-methanol-water (4:1:2:4, v/v) was chosen as the two-phase solvent system for CCC, while the acetonitrile-water (25:75 for the first step and15:85 for the second step, v/v) was used as the mobile phase in the preparative RP-HPLC. The following five steroid saponins were purified by theses two chromatographic methods, in one-step operation by CCC and by two-step operation in preparative RP-HPLC: 1) 26-O-β-D- glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 26-triol-3-O-[β-D-glucopyranosyl-(1→3)-β-D-glucopyranosyl-(1→4)-α-L-rhamnopyranosyl-(1→2)]-β-D-glucopyranoside (compound A), 2) 26-O-β-D-glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 4) 26-triol-3-O-[β-D-glucopyranosyl-(1→3)-α-L-rhamnopyranosyl-(1→2)]-β-D-glucopyranoside (compound B), 3) 26-O-β-D-glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 26-triol-3-O-[α-L-rhamnopyranosyl-(1→4)]-β-D-glucopyranoside (compound C), 4) 26-O-β-D-glucopyranosyl-(25R)-furost-5, 20(22)-diene-3β, 26-diol-3-O-{α-L-rhamnopyranosyl-(1→4)-[β-D-glucopyranosyl-(1→3)-β-D-glucopyranosyl-(1→2)]}-β-D-glucopyranoside (compound D) and 5) 26-O-β-D-glucopyranosyl-(25R)-furost-5, 20(22)-diene-3β, 26-diol-3-O-[β-D-glucopyranosyl-(1→4)-α-L-rhamnopyranosy-(1→2)]-β-D-glucopyranoside (compound E). The purities of these five steroid saponins separated by both methods were over 95%, and structural identification of these compounds was performed by ESI-MS, and 13C NMR. Comparison of these two established approaches revealed that CCC required a longer separation time but with less solvent consumption, whereas preparative RP-HPLC gave a shorter separation time but with higher solvent consumption. These results demonstrated that either of these two methods of different separation mechanism is feasible, economical and efficient for rapid preparative isolation and purification of steroid saponins from Dioscorea zingiberensis C.H.Wright.
{"title":"Comparative studies on performance of CCC and preparative RP-HPLC in separation and purification of steroid saponins from Dioscorea zingiberensis C.H.Wright.","authors":"Xinxin Zhang, Jinru Liang, Yongming Zhang, Jian-li Liu, Wen-ji Sun, Y. Ito","doi":"10.4172/2157-7536.1000.150","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.150","url":null,"abstract":"Steroid saponins from Dioscorea zingiberensis C.H.Wright were separated for the first time using two chromatographic methods for comparison: counter-current chromatography (CCC) coupled with evaporative light scattering detector (ELSD) and preparative reversed phase high-performance liquid chromatography (RP-HPLC) with an ultraviolet detector. Ethyl acetate-n-butanol-methanol-water (4:1:2:4, v/v) was chosen as the two-phase solvent system for CCC, while the acetonitrile-water (25:75 for the first step and15:85 for the second step, v/v) was used as the mobile phase in the preparative RP-HPLC. The following five steroid saponins were purified by theses two chromatographic methods, in one-step operation by CCC and by two-step operation in preparative RP-HPLC: 1) 26-O-β-D- glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 26-triol-3-O-[β-D-glucopyranosyl-(1→3)-β-D-glucopyranosyl-(1→4)-α-L-rhamnopyranosyl-(1→2)]-β-D-glucopyranoside (compound A), 2) 26-O-β-D-glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 4) 26-triol-3-O-[β-D-glucopyranosyl-(1→3)-α-L-rhamnopyranosyl-(1→2)]-β-D-glucopyranoside (compound B), 3) 26-O-β-D-glucopyranosyl-(25R)-furost-5-en-3β, 22ζ, 26-triol-3-O-[α-L-rhamnopyranosyl-(1→4)]-β-D-glucopyranoside (compound C), 4) 26-O-β-D-glucopyranosyl-(25R)-furost-5, 20(22)-diene-3β, 26-diol-3-O-{α-L-rhamnopyranosyl-(1→4)-[β-D-glucopyranosyl-(1→3)-β-D-glucopyranosyl-(1→2)]}-β-D-glucopyranoside (compound D) and 5) 26-O-β-D-glucopyranosyl-(25R)-furost-5, 20(22)-diene-3β, 26-diol-3-O-[β-D-glucopyranosyl-(1→4)-α-L-rhamnopyranosy-(1→2)]-β-D-glucopyranoside (compound E). The purities of these five steroid saponins separated by both methods were over 95%, and structural identification of these compounds was performed by ESI-MS, and 13C NMR. Comparison of these two established approaches revealed that CCC required a longer separation time but with less solvent consumption, whereas preparative RP-HPLC gave a shorter separation time but with higher solvent consumption. These results demonstrated that either of these two methods of different separation mechanism is feasible, economical and efficient for rapid preparative isolation and purification of steroid saponins from Dioscorea zingiberensis C.H.Wright.","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"5 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2015-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81213536","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-02-25DOI: 10.4172/2157-7536.1000.152
R. McGrath, S. Glastras, S. Hocking, Iona Tjoeng, M. Krause, G. Fulcher
The identification of the glucagon-like peptide-1 receptor in the central nervous system has led to an array of studies exploring the functions of central GLP-1 signalling. Originally identified as a gastrointestinal incretin hormone responsible for the potentiation of insulin secretion following ingestion of nutrients, the role of GLP-1 has been expanded to include specific neural activities. Two distinct actions of GLP-1 receptor activation in the brain have been identified, namely the regulation of appetite via promotion of satiety, as well as anti-inflammatory and anti-apoptotic activity to promote neuronal cell survival. Both of these features are now being exploited clinically, with GLP-1 receptor agonists, initially designed and marketed for the treatment of hyperglycaemia in type 2 diabetes, now being directed towards use in obesity and as potential neuroprotective agents. This review gives a summary of the functional role of GLP-1 in the central nervous system, in terms of promoting satiety, modulating food intake and aiding in the regulation of peripheral glycaemia. In addition, the molecular mechanisms underpinning the beneficial effects of central GLP-1 receptor agonist therapy in protecting against neuronal cell inflammation and death, including neurodegenerative processes, are described.
{"title":"Central Functions of Glucagon-like Peptide-1: Roles in Energy Regulation andNeuroprotection","authors":"R. McGrath, S. Glastras, S. Hocking, Iona Tjoeng, M. Krause, G. Fulcher","doi":"10.4172/2157-7536.1000.152","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.152","url":null,"abstract":"The identification of the glucagon-like peptide-1 receptor in the central nervous system has led to an array of studies exploring the functions of central GLP-1 signalling. Originally identified as a gastrointestinal incretin hormone responsible for the potentiation of insulin secretion following ingestion of nutrients, the role of GLP-1 has been expanded to include specific neural activities. Two distinct actions of GLP-1 receptor activation in the brain have been identified, namely the regulation of appetite via promotion of satiety, as well as anti-inflammatory and anti-apoptotic activity to promote neuronal cell survival. Both of these features are now being exploited clinically, with GLP-1 receptor agonists, initially designed and marketed for the treatment of hyperglycaemia in type 2 diabetes, now being directed towards use in obesity and as potential neuroprotective agents. This review gives a summary of the functional role of GLP-1 in the central nervous system, in terms of promoting satiety, modulating food intake and aiding in the regulation of peripheral glycaemia. In addition, the molecular mechanisms underpinning the beneficial effects of central GLP-1 receptor agonist therapy in protecting against neuronal cell inflammation and death, including neurodegenerative processes, are described.","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"27 1","pages":"1-8"},"PeriodicalIF":0.0,"publicationDate":"2015-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83490767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-02-25DOI: 10.4172/2157-7536.1000.149
M. Abdallah, Ah madi, M. A. Rana
Corticosteroids are one of the most common medications that are used in the intensive care units (ICUs); corticosteroids are used for a variety of indications, including septic shock, acute respiratory distress syndrome (ARDS), bacterial meningitis, tuberculous meningitis, lupus nephritis, severe chronic obstructive pulmonary disease (COPD) exacerbations and many others.
{"title":"The Best Use of Systemic Corticosteroids in the Intensive Care Units, Review","authors":"M. Abdallah, Ah madi, M. A. Rana","doi":"10.4172/2157-7536.1000.149","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.149","url":null,"abstract":"Corticosteroids are one of the most common medications that are used in the intensive care units (ICUs); corticosteroids are used for a variety of indications, including septic shock, acute respiratory distress syndrome (ARDS), bacterial meningitis, tuberculous meningitis, lupus nephritis, severe chronic obstructive pulmonary disease (COPD) exacerbations and many others.","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"78 1","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2015-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83738528","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-01-01DOI: 10.4172/2157-7536.S1.001
G. E. Hoffman
{"title":"G E Hoffman received her PhD in Pharmacology","authors":"G. E. Hoffman","doi":"10.4172/2157-7536.S1.001","DOIUrl":"https://doi.org/10.4172/2157-7536.S1.001","url":null,"abstract":"","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"28 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2015-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81971828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-01-01Epub Date: 2015-01-12DOI: 10.4172/2157-7536.1000.e114
Anthony C Hackney, Amy R Lane
Anthony C Hackney1,2,3* and Amy R Lane1 1Human Movement Science Curriculum, University of North Carolina Chapel Hill, NC, USA 2Department of Exercise & Sport Science, University of North Carolina Chapel Hill, NC, USA 3Department of Nutrition Gillings School of Global Public Health, University of North Carolina Chapel Hill, NC, USA *Corresponding author: Anthony C. Hackney, Human Movement Science Curriculum, Department of Exercise & Sport Science, Department of Nutrition Gillings School of Global Public Health, University of North Carolina Chapel Hill, NC, USA, Tel: +1 919-962-2211; E-mail: thackney@med.unc.edu
{"title":"Exercise Endocrinology: Guidance for Future Research Direction and Focus.","authors":"Anthony C Hackney, Amy R Lane","doi":"10.4172/2157-7536.1000.e114","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.e114","url":null,"abstract":"Anthony C Hackney1,2,3* and Amy R Lane1 1Human Movement Science Curriculum, University of North Carolina Chapel Hill, NC, USA 2Department of Exercise & Sport Science, University of North Carolina Chapel Hill, NC, USA 3Department of Nutrition Gillings School of Global Public Health, University of North Carolina Chapel Hill, NC, USA *Corresponding author: Anthony C. Hackney, Human Movement Science Curriculum, Department of Exercise & Sport Science, Department of Nutrition Gillings School of Global Public Health, University of North Carolina Chapel Hill, NC, USA, Tel: +1 919-962-2211; E-mail: thackney@med.unc.edu","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"6 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2015-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4172/2157-7536.1000.e114","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36012411","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}
Pub Date : 2015-01-01DOI: 10.4172/2157-7536.1000163
P. Geoffroy, Bl., ine Ressault, M. Miesch
Hoodia gordonii is a succulent plant (asclepiadaceae family) which grows in the Kalahari desert in South Africa. On a historical point of view, it was claimed that the San people, a Bushmen tribe leaving in the Kalahari desert, were able to make long hunting trips without feeling thursty and hungry after chewing fresh sap from H. gordonii. Therefore, it was claimed that H. gordonii could represent a new help for fighting obesity, which is one of the major health problems in the 21st century. Indeed, in 2014, more than 1.9 billion adults, 18 years and older, were overweight. Of these over 600 million were obese [1].
{"title":"Synthesis of Hoodigogenin A, the Aglycone of Hoodigosides Extractedfrom Hoodia gordonii","authors":"P. Geoffroy, Bl., ine Ressault, M. Miesch","doi":"10.4172/2157-7536.1000163","DOIUrl":"https://doi.org/10.4172/2157-7536.1000163","url":null,"abstract":"Hoodia gordonii is a succulent plant (asclepiadaceae family) which grows in the Kalahari desert in South Africa. On a historical point of view, it was claimed that the San people, a Bushmen tribe leaving in the Kalahari desert, were able to make long hunting trips without feeling thursty and hungry after chewing fresh sap from H. gordonii. Therefore, it was claimed that H. gordonii could represent a new help for fighting obesity, which is one of the major health problems in the 21st century. Indeed, in 2014, more than 1.9 billion adults, 18 years and older, were overweight. Of these over 600 million were obese [1].","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"229 1","pages":"1-5"},"PeriodicalIF":0.0,"publicationDate":"2015-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85575130","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-01-01DOI: 10.4172/2157-7536.1000.E115
Bas Brouwers Creemers
{"title":"Unexpected Phenotypes in Mouse Models Carrying the Human Growth Hormone Minigene to Enhance Transgene Expression","authors":"Bas Brouwers Creemers","doi":"10.4172/2157-7536.1000.E115","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.E115","url":null,"abstract":"","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"13 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2015-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72788718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2015-01-01DOI: 10.4172/2157-7536.1000159
El-Tawil Am
{"title":"Colon and Rectal Cancers and Chlorination By-Products in Treated Water:A Strategy for Management Based on Hormonal Studies","authors":"El-Tawil Am","doi":"10.4172/2157-7536.1000159","DOIUrl":"https://doi.org/10.4172/2157-7536.1000159","url":null,"abstract":"","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"123 9","pages":""},"PeriodicalIF":0.0,"publicationDate":"2015-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91472180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2014-11-04DOI: 10.4172/2157-7536.1000148
M. Modrzejewska, E. Lachowicz, D. JoannaKot, W. Lubiński, J. Rudnicki, B. Czeszyńska, Anna Modrzejewska, J. Patalan
Aims: To study the efficacy of diode laser-systemic steroid therapy in extremely and very-low-birth-weight prematures with severe Retinopathy of Prematurity (ROP). Methods: 36 eyes of 18 prematures, mean gestational age 25.67 weeks (SD ± 1.28) and 811, 83 g birth weight (SD ± 299.08) with aggressive-posterior ROP (AP-ROP) and threshold 3 ROP with plus sign and Extraretinal Fibrovascular Proliferation (EFP) were enrolled. Indirect diode laser combined with intravenous course of Dexamethason was applied. Analyzed risk factors were correlated with the same ones received in cohort treated only with laser. Shapiro-Wilk, t-Student, U Mann-Whitney tests were involved in the statistical analysis (significance levels at p<0.05). Results: Favorable anatomical results after mean 11.29 (SD ± 2.29) days of therapy were noted in 32 eyes (88.88% ), ROP 5 developed in four eyes, but this difference was statistically non-significant (p=0.0612). Transient cortisol decreasing, hyperglycemia and partial adrenal fatigue were noted in some babies. During therapy, arterial systolic and diastolic pressures rose (p<0.05; p<0.01), significantly, which were related with severity of ROP, such as, C-reactive protein, erythrocyte and hematocrit, ventilation duration and number of transfusion (p<0.01). Conclusion: Laser-steroid treatment might be additional effective alternative for severe ROP. Short- and long-term complications should be taken into account when starting this type of therapy
目的:探讨二极管激光全身类固醇治疗极低出生体重早产儿合并严重早产儿视网膜病变(ROP)的疗效。方法:选取18例36眼，平均胎龄25.67周(SD±1.28)，出生体重811.83 g (SD±299.08)，伴有侵袭性后路ROP (AP-ROP)和阈值3路ROP，伴有正征和视网膜外纤维血管增生(EFP)的早产儿。采用间接二极管激光联合静脉注射地塞米松。所分析的危险因素与仅接受激光治疗的队列相同。采用Shapiro-Wilk、t-Student、U Mann-Whitney检验进行统计学分析(显著性水平p<0.05)。结果:治疗后平均11.29 (SD±2.29)d, 32只眼(88.88%)解剖效果良好，4只眼出现ROP 5，但差异无统计学意义(p=0.0612)。一些婴儿出现短暂的皮质醇下降、高血糖和部分肾上腺疲劳。治疗期间动脉收缩压、舒张压升高(p<0.05;p<0.01)，且与ROP严重程度有关，如c反应蛋白、红细胞和红细胞压积、通气时间、输血次数等(p<0.01)。结论:激光类固醇治疗可能是治疗严重ROP的又一有效选择。在开始这种治疗时，应考虑到短期和长期的并发症
{"title":"Early Experience with Diode Laser Combined with Systemic Steroid Therapy for Severe Stages of Retinopathy of Prematurity","authors":"M. Modrzejewska, E. Lachowicz, D. JoannaKot, W. Lubiński, J. Rudnicki, B. Czeszyńska, Anna Modrzejewska, J. Patalan","doi":"10.4172/2157-7536.1000148","DOIUrl":"https://doi.org/10.4172/2157-7536.1000148","url":null,"abstract":"Aims: To study the efficacy of diode laser-systemic steroid therapy in extremely and very-low-birth-weight \u0000prematures with severe Retinopathy of Prematurity (ROP). \u0000Methods: 36 eyes of 18 prematures, mean gestational age 25.67 weeks (SD ± 1.28) and 811, 83 g birth weight (SD ± 299.08) with aggressive-posterior ROP (AP-ROP) and threshold 3 ROP with plus sign and Extraretinal Fibrovascular Proliferation (EFP) were enrolled. Indirect diode laser combined with intravenous course of Dexamethason was applied. Analyzed risk factors were correlated with the same ones received in cohort treated only with laser. Shapiro-Wilk, t-Student, U Mann-Whitney tests were involved in the statistical analysis (significance levels at p<0.05). \u0000Results: Favorable anatomical results after mean 11.29 (SD ± 2.29) days of therapy were noted in 32 eyes (88.88% ), ROP 5 developed in four eyes, but this difference was statistically non-significant (p=0.0612). Transient cortisol decreasing, hyperglycemia and partial adrenal fatigue were noted in some babies. During therapy, arterial systolic and diastolic pressures rose (p<0.05; p<0.01), significantly, which were related with severity of ROP, such as, C-reactive protein, erythrocyte and hematocrit, ventilation duration and number of transfusion (p<0.01). \u0000Conclusion: Laser-steroid treatment might be additional effective alternative for severe ROP. Short- and long-term complications should be taken into account when starting this type of therapy","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"1 1","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2014-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91529240","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2014-10-24DOI: 10.4172/2157-7536.1000.144
S. Tung, Daw-Yang Hwang, Joseph W. Yang, H. Ng, Chien-Te Lee
Purpose: To evaluate the clinical manifestations of Cushing’s Syndrome (CS) and associated genetic mutation in patients with Primary Pigmented Nodular Adrenocortical Disease (PPNAD). Methods: Seven family members were screened for mutations of the PRKAR1A gene. Gene mutation screening used genomic DNA (from peripheral blood leukocytes and, in some cases, adrenal gland tissue) and subsequent DNA sequencing. The five patients showing genetic mutation were assessed clinically for baseline cortisol and adrenocorticotropic hormone and adrenal imaging studies (abdominal computed tomography and adrenal scintigraphy). Low-dose and high-low dexamethasone suppression tests were performed in these five patients. Results: PRKAR1A gene mutation was detected in five of the seven family members. Four of the five gene mutation-positive patients presented with overt CS due to pathology-proven PPNAD. After unilateral adrenalectomy in these four patients, overt CS persisted for two patients and CS symptoms abated for the other two. The remission period of CS was >11 years in one instance (case III-2) and continues for >12 years in the other (case II-4), with no observable disease of the contralateral non-resected adrenal gland. For each of the two remission patients, one adrenal gland had been larger (by abdominal computed tomography) and had had stronger function (by 131I-6β- iodomethyl-19-norcholesterol scintigraphy) than the other. In one (case III-2) of the two remission patients, one adrenal displayed focal PPNAD while the other displayed diffuse PPNAD. The clinical manifestations of CS did not resolve after unilateral adrenalectomy in case II-2 and III-7. The fifth gene mutation-positive patient (case III-4) is still in the latent stage of CS. Conclusions: The clinical manifestations of CS and adrenal image features in patients with PPNAD are heterogeneous. Detailed adrenal imaging of these patients is necessary to assist with the decision to perform unilateral adrenalectomy.
{"title":"Heterogeneous Clinical Manifestations of Cushing's Syndrome in a Family with Primary Pigmented Nodular Adrenocortical Disease","authors":"S. Tung, Daw-Yang Hwang, Joseph W. Yang, H. Ng, Chien-Te Lee","doi":"10.4172/2157-7536.1000.144","DOIUrl":"https://doi.org/10.4172/2157-7536.1000.144","url":null,"abstract":"Purpose: To evaluate the clinical manifestations of Cushing’s Syndrome (CS) and associated genetic mutation in \u0000patients with Primary Pigmented Nodular Adrenocortical Disease (PPNAD). \u0000Methods: Seven family members were screened for mutations of the PRKAR1A gene. Gene mutation screening \u0000used genomic DNA (from peripheral blood leukocytes and, in some cases, adrenal gland tissue) and subsequent \u0000DNA sequencing. The five patients showing genetic mutation were assessed clinically for baseline cortisol and \u0000adrenocorticotropic hormone and adrenal imaging studies (abdominal computed tomography and adrenal \u0000scintigraphy). Low-dose and high-low dexamethasone suppression tests were performed in these five patients. \u0000Results: PRKAR1A gene mutation was detected in five of the seven family members. Four of the five gene \u0000mutation-positive patients presented with overt CS due to pathology-proven PPNAD. After unilateral adrenalectomy \u0000in these four patients, overt CS persisted for two patients and CS symptoms abated for the other two. The remission \u0000period of CS was >11 years in one instance (case III-2) and continues for >12 years in the other (case II-4), with no \u0000observable disease of the contralateral non-resected adrenal gland. For each of the two remission patients, one \u0000adrenal gland had been larger (by abdominal computed tomography) and had had stronger function (by 131I-6β- \u0000iodomethyl-19-norcholesterol scintigraphy) than the other. In one (case III-2) of the two remission patients, one \u0000adrenal displayed focal PPNAD while the other displayed diffuse PPNAD. The clinical manifestations of CS did not \u0000resolve after unilateral adrenalectomy in case II-2 and III-7. The fifth gene mutation-positive patient (case III-4) is still \u0000in the latent stage of CS. \u0000Conclusions: The clinical manifestations of CS and adrenal image features in patients with PPNAD are \u0000heterogeneous. Detailed adrenal imaging of these patients is necessary to assist with the decision to perform \u0000unilateral adrenalectomy.","PeriodicalId":17132,"journal":{"name":"Journal of steroids & hormonal science","volume":"61 1","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2014-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77736408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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