Cortical Hormones Research Articles

A Rare Case of Co-occurrence of Multiple Endocrine Neoplasia Syndrome and Congenital Adrenal Hyperplasia

Multiple endocrine neoplasia type 1 (MEN1) and congenital adrenal hyperplasia (CAH) are rare monogenic hereditary endocrinopathies with a prevalence of 1–9 cases per 100,000 and 9–15 cases per 100,000, respectively. MEN1 is characterized by the development of multiple endocrine and nonendocrine organ tumors, including parathyroid, pituitary, and duodenopancreatic neuroendocrine tumors (NETs), which constitute the classical triad of the disease. CAH is associated with genetic defects in enzymes and transport proteins involved in the synthesis of adrenal cortical steroid hormones. Overall, cases of the combination of two hereditary diseases in one patient are extremely rare. In this article, we describe a clinical case of the combination of MEN-1 with all three classical components and CAH, which, taking into account the low prevalence of both diseases, represents scientific interest. To date, only one similar case has been described in the literature. In addition, the paper discusses the pathogenetically determined combination of CAH and Ehlers-Danlos syndrome, known as the CAH-X syndrome.

Cherry leaf decoction inhibits NMDAR expression and thereby ameliorates CUMS- induced depression-like behaviors through downregulation of α2δ-1

Depression is a complex and prevalent mental illness. Cherry leaf is a traditional Chinese herbal medicine, which has confirmed to exert a certain antidepressant effect, but its potential neural regulation mechanism is not clear. This paper aims to investigate the improved action of cherry leaf decoction (CLD) on chronic unpredictable mild stress (CUMS) rats and its potential neural regulation mechanism by verifying the role and function of NMDAR regulatory target α2δ-1 in depression due to CUMS. Male SD rats were subjected to random stressors persisting for 5 weeks to establish the CUMS depression rat model. CLD could effectively alleviate depression-like behaviors of CUMS rats in behavioral tests including sucrose preference test, forced swimming test, tail suspension test and open field test. After the administration of the CLD, the expression of corticotropic-releasing hormone (CRH) in the hypothalamus was inhibited. Moreover, the levels of CRH, adrenal cortical hormone (ACTH) and corticosterone (CORT) in serum also decreased significantly. CUMS upregulated the expressions of α2δ-1, N-methyl-d-aspartate receptor 1 (NR1), NR2A and NR2B, and enhanced the binding ability to of α2δ-1 and NR1, which were reversed by CLD. The results demonstrated that CLD could ameliorate depression-like behaviors due to CUMS, which was related to the fact that CLD down-regulated α2δ-1 level and interfered with α2δ-1 binding to NR1, thereby reducing NMDAR expression and ultimately inhibiting HPA axis activity.

Effect of different delivery modes on the level of cortical hormones in cord artery and vein blood

To investigate the difference of cortical hormones in cord artery and vein blood of newborns with different delivery modes. A total of 65 pregnant women who delivered in the People's Hospital of Danyang City, Jiangsu Province from June to September 2021 were selected as the study subjects, including 26 cases of spontaneous delivery and 39 cases of cesarean section. The basic information of 65 pregnant women and newborns was collected by questionnaire survey. According to the mode of delivery, the levels of corticosteroids in umbilical vein and umbilical artery blood were determined by liquid chromatography-tandem mass spectrometry(LC-MS/MS), including corticosterone, 11-desoxycorticosterone, aldosterone, cortisol, 11-deoxycortisol and cortisone. The data were statistically analyzed using IBM SPSS Statistics 26.0 statistical software. The levels of cortisol, 11-deoxycortisol, aldosterone, cortisol, 11-deoxycortisol and cortisone in the umbilical vein blood of the spontaneous delivery group were(2.44±1.87), (0.64±0.29), (0.49±0.35), (54.95±40.80), (3.20±1.23) and(142.27±57.42)ng/mL, respectively. The levels of corticosterone, 11-deoxycortisol, aldosterone, cortisol, 11-deoxycortisol and cortisone in umbilical artery blood were(4.51±4.47), (0.57±0.28), (0.42±0.29), (60.79±45.53), (2.69±1.25) and(123.10±46.32)ng/mL, respectively. The levels of corticosterone, 11-deoxycortisone, aldosterone, cortisol, 11-deoxycortisone and cortisone in umbilical vein blood of cesarean section group were(0.94±1.09), (0.47±0.14), (0.26±0.14), (22.63±19.82), (2.30±0.90) and(84.51±29.49)ng/mL, respectively. The levels of corticosterone, 11-deoxycortisol, aldosterone, cortisol, 11-deoxycortisol and cortisone in umbilical artery blood were(2.22±2.24), (0.43±0.17), (0.27±0.14), (30.09±25.93), (1.87±0.76) and(75.03±24.90)ng/mL, respectively. The levels of corticosterone, 11-desoxycorticosterone, aldosterone, cortisol, 11-deoxycortisol and cortisone in cord vein blood and cord artery blood in spontaneous labor group were significantly higher than those in cesarean section group(P<0.05). The levels of corticosterone and cortisol in cord vein blood were significantly lower in spontaneous labor group and cesarean section group than those in cord artery blood(P<0.05), the levels of 11-desoxycorticosterone, 11-deoxycortisol and cortisone in cord vein blood were significantly higher in spontaneous labor group and cesarean section group than those in cord artery blood(P<0.05). There are differences in the level of cortical hormones in cord artery and vein blood in different delivery modes.

Mushroom Poisoning and Treatments

The poisoning mushroom can affect the human body and is even lethal. This review summarizes gastroenteritis, hemolytic and liver damage caused by poisoning mushroom, as well as therapy methods for a variety of toxins. The main symptoms of gastroenteritis include acute nausea, abdominal pain, and diarrhea. For that, the common treatments include vomiting, gastric lovage and catharsis. The manifestation of hemolytic contains interic, acute anemia, liver adds spleen enlargement. The main approaches used to alleviate the effects are cortisone and atrium biacrbonate. Moreover, serious anemic people can use adrenal cortical hormones or Transfusion Therapy. The symptoms of liver damage are quite similar to gastroenteritis and hemolytic, but the manifestation is instruction regarding form doctors and over-the counter medicines. With prompt treatment, most minor symptoms can be cured. However, if the toxin has damaged the endoplasmic reticulum from liver nucleus, it would be difficult for patients to recover. Future research may focuse on precise treatment methods of mushroom positioning. More efforts should be made to facilitate the awareness of people about poisoning mushrooms to prevent mushroom poisoning.

PSUN22 Outcomes of Adrenalectomy for Adrenocortical Carcinoma at a Single Institution

Abstract Background Adrenocortical carcinoma (ACC) is a rare malignancy with a poor overall prognosis. Complete surgical resection is the mainstay of therapy. Methods We performed a retrospective review of adult patients at a single institution undergoing index adrenalectomy for initial diagnosis of adrenocortical carcinoma from 2012 through 2021. Clinical and demographic variables were assessed. Results Fifteen patients were identified who underwent adrenalectomy for ACC during the study period with a mean age of 49.2 years (SD 17.8) with 6 (40%) male, 13 (87%) white, mean body mass index 29.5 kg/m2 (SD 7.5). On preoperative cross-sectional imaging nine (60%) were left sided tumors and the largest dimension was a mean of 11.1cm (SD 4.5), with pre-contrast density of 27.8 Hounsfield units (SD 9.6), five patients (36%) had evidence of local invasion and four patients (29%) had evidence of metastatic disease on imaging. Ten patients (67%) had biochemical evidence of hormone excess and eight had secretion of more than one cortical hormone, with overall ten (67%) glucocorticoid secretion, six (40%) androgen secretion, two (13%) mineralocorticoid secretion. Four patients (27%) had minimally invasive laparoscopic or robotic adrenalectomy. Three patients (20%) had a surgical complication including ileus, pancreatic leak and pleural effusion requiring intervention. Complications were not associated with hormone excess (p = 0.50). On final pathology, one patient (7%) was staged T0 who later presented with metastatic disease 18 months postoperatively, six (43%) were T2, 4 (29%) T3, and 3 (21%) T4. No patients had locoregional lymph node metastases. Ten patients (67%) had R0 resection with negative margins, 4 (27%) had microscopic positive margins, and 1 had grossly positive margins. Seven patients had Ki-67 assessed with a mean of 29% (SD 20.6). Ten patients (71%) received adjuvant mitotane, eight (53%) systemic chemotherapy, and 4 (27%) adjuvant radiation. Within the study period, nine patients (60%) had a recurrence and six patients died (40%) with a median survival of 24 months. Positive margin status was associated with mortality with mortality of one (10%) of patients with negative margins and 5 (100%) of those with positive margins (p = 0.002). Of the four patients who presented with initial evidence of metastatic disease on imaging, two died during the study period and the other two remain alive on therapy. Adjuvant treatment with mitotane, systemic chemotherapy, or radiation were not associated with survival. Conclusions In this cohort, complete surgical resection with negative margins was associated with improved survival compared with patients with microscopic or grossly positive margins. The overall median survival was 24 months. Differences in outcome by adjuvant therapy are limited by the sample size. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

The preclinical pharmacological study of a novel intravenous anesthetic, ET-26 hydrochloride, in aged rats.

BackgroundET-26 hydrochloride (ET-26HCl) is a novel analogue of etomidate approved for clinical trials. However, all results from recent studies were accomplished in young adult animals. The objective of this study was to evaluate the efficacy and safety of ET-26HCl in aged rats.MethodsAged Sprague-Dawley rats were randomly divided into three groups (three males and three females in each group) were given dose of two-fold of median effective dose (ED50) of ET-26HCl, etomidate and propofol: the measurements of loss of the righting reflex (LORR) and cardiovascular and respiratory function after injection at the two-fold dose of the median effective dose were used for evaluation of effectiveness and safety, and the modified adrenocorticotropic hormone-stimulation experiment was used to evaluate the inhibition effect of the drugs on the synthesis of adrenal cortical hormones.ResultsThere was no significant difference in the onset time among propofol, etomidate and ET-26HCl. The duration of propofol (850.5 ± 77.4 s) was significantly longer than that caused by etomidate (489.8 ± 77.0 s, p = 0.007) and ET-26HCl (347.3 ± 49.0 s, p = 0.0004). No significant difference was observed in the time to stand and normal activity among drugs. A total of 66.7% of rats in the ET-26HCl group were evaluated to have mild hematuria. Then, etomidate and ET-26HCl had a milder blood pressure inhibition effect than propofol. Apnea was observed in all rats administered propofol and the duration for this side effect was 45.0 ± 9.0 s. For etomidate and ET-26HCl, no apnea was observed. No other clinical signs of side-effect were observed, and no rats died. No significant difference was observed in corticosterone concentrations between ET-26HCl and solvent group. However, rats administered etomidate had lower corticosterone concentrations than those administered ET-26HCl at 15, 30, and 60 min.ConclusionsOur results indicate ET-26HCl in aged rats is an effective sedative-hypnotic with stable myocardial and respiratory performance and also have mild adrenocortical suppression. Thus, these findings increase the potential for the clinical use of ET-26HCl in the elderly population.

Adding on Addison’s: A Case Report of Rare Co-Existent Pathology Complicating Acute Pre-Eclampsia (PET) in Pregnancy

Addison’s disease is a rare condition, which world-wide, may affect many millions of people. In pregnancy however, it has been rarely reported and, in this setting, remains poorly understood. What we do know, is that it is an endocrine disorder characterized by adrenal insufficiency leading to decreased production of cortical hormones including most notably, cortisol, but also the androgens and aldosterone. The sequaelae may be varied but most significantly, can lead to a loss of the body’s normal resilience to stress and injury. We present the case of a 35-year-old woman who presented in late pregnancy with signs and symptoms of fulminating pre-eclampsia (PET). Paradoxically, she was found to have electrolyte abnormalities which persisted despite appropriate treatment. These were suggestive of an underlying endocrine disorder and she was subsequently diagnosed with atypical Addison’s disease. With appropriate management, she was stabilized and delivered safely. We report this as a rare example of newly diagnosed Addison’s disease discovered for the first time in late pregnancy. It highlights that although rarely diagnosed, early identification can help avoid potentially life-threatening sequalae such as acute adrenal insufficiency as well as complications including preterm birth, neonatal hypoglycaemia and prolonged hospital admission. This calls for ongoing vigilance in clinical care, and the continuous improvement of shared knowledge through collaborative communication and awareness of evidence based, best practice guidelines.

Determination and level investigation of 45 hormones in edible bird's nest by solid phase extraction-high performance liquid chromatography-tandem mass spectrometry

建立了固相萃取-高效液相色谱-串联质谱(SPE-HPLC-MS/MS)同时测定食用燕窝中皮质激素、雌激素、雄激素及孕激素等5类45种激素的多残留分析方法。采用乙腈-乙酸乙酯(1∶1, v/v)超声辅助提取、亲水亲脂平衡固相萃取柱净化,甲醇洗脱。分别在正、负电喷雾电离源、多反应监测模式下检测45种激素。正离子模式下的流动相为乙腈-0.1%甲酸水溶液,负离子模式下的流动相为乙腈-水,色谱柱为Phenomenex Kinetex C18柱(100 mm×2.1 mm, 2.6 μm)。在优化条件下,45种激素在0.20~20.0 μg/L范围内线性关系良好,相关系数(R2)≥0.9990,方法的检出限(LOD)为0.04~0.70 μg/kg,定量限(LOQ)为0.16~2.00 μg/kg。按三水平(2.0、4.0、20.0 μg/kg)进行加标回收试验,氟米龙、布地奈德、醛固酮、醋酸氟轻松、炔雌醇的回收率为40.2%~63.6%,可对这5种激素进行定性分析,其余40种激素的平均加标回收率为72.2%~112.3%,相对标准偏差(RSD)为2.5%~11.6%,该方法可对40种激素进行准确定性定量,精密度好,灵敏度高,简便、快速。从2017~2021年,通过研究建立的方法对来自马来西亚、印度尼西亚、泰国和越南等国家的1021个燕窝样品进行监测,仅勃地酮、雄烯二酮、孕酮有检出(大于检出限),其他激素均小于检出限。孕酮检出率为100%,勃地酮、雄烯二酮检出率分别为79%和89%, 3种激素含量范围分别为0.097~3.58、0~0.096和0~1.77 μg/kg。与同为动物源性食品的鸡蛋、纯牛奶、乳制品相比,所有测定的鸡蛋样品中均检出雄烯二酮,含量比其他3类产品略高;勃地酮在4类产品中的含量差别不大,均为微量;孕酮含量在鸡蛋中最高,其次是纯牛奶,燕窝中含量最低。研究结果表明,食用燕窝带入的激素种类少,含量低,对健康影响小。

Evaluation of Adrenal Cortical Function in Neonatal and Weanling Laboratory Beagle Dogs.

The most common target organ for toxicity in the endocrine system is the adrenal gland, and its function is dependent upon the hypothalamus and pituitary gland. Histopathologic examination of the adrenal glands and pituitary gland is routinely performed in toxicity studies. However, the function of the adrenal gland is not routinely assessed in toxicity studies. Assessment of adrenal cortical function may be necessary to determine whether a histopathologic finding in the adrenal cortex results in a functional effect in the test species. As juvenile toxicity studies are more commonly performed in support of pediatric indications for pharmaceuticals, it is important to establish historical control data for adrenal gland function. In this study, adrenal cortical function was assessed in control neonatal and weanling beagle dogs as part of an ongoing juvenile toxicology program. Measurements of serum adrenocorticotropic hormone (ACTH), cortisol prior to and following administration of exogenous ACTH, and aldosterone were conducted beginning at 2 weeks of age continuing through 26 weeks of age. Serum electrolyte concentrations were determined at 4, 13, and 26 weeks of age. Dogs as young as 2 weeks of age synthesize and secrete adrenal cortical hormones and exhibit a functional hypothalamic pituitary adrenal axis.

Self-Empowerment, Affirmation And Relaxation Training Against Stress Levels And Cortisol Levels In Pregnant Women: Systematic Literature Review

The incidence of stress in pregnancy in the world is 14-54%. The incidence of stress in pregnant women in Indonesia reaches 55.6%. interventions can potentially reduce stress and the hormone cortisol during pregnancy. To analyze self-empowerment, affirmation, and relaxation training which have the potential to reduce stress and cortical hormone scores in pregnant women based on literature studies that have been conducted. Systematic Literature Review research using the PRISMA method. The period studied is from 2016-2020. The potential to reduce levels of stress and the hormone cortisol is more effective using the Self-empowerment method during pregnancy, which means that it can reduce stress during pregnancy and the impact it will have on the mother and the fetus. It was concluded that the intervention (self-empowerment, affirmation, and relaxation) was proven to reduce stress scores and the hormone cortisol in pregnant women.

GIPC2 is an endocrine-specific tumor suppressor gene for both sporadic and hereditary tumors of RET- and SDHB-, but not VHL-associated clusters of pheochromocytoma/paraganglioma

Pheochromocytoma/paraganglioma (PPGL) is an endocrine tumor of the chromaffin cells in the adrenal medulla or the paraganglia. Currently, about 70% of PPGLs can be explained by germline or somatic mutations in several broadly expressed susceptibility genes including RET, VHL, and SDHB, while for the remaining, mainly sporadic cases, the pathogenesis is still unclear. Even for known susceptible genes, how mutations in these mostly ubiquitous genes result in tissue-specific pathogenesis remains unanswered, and why RET-mutated tumors almost always occur in the adrenal while SDHB-mutated tumors mostly occur extra-adrenal remains a mystery. By analyzing 22 sporadic PPGLs using SNP 6.0 genotyping arrays combined with expression profiling of 4 normal and 4 tumor tissues, we identified GIPC2, a gene located at 1p31.1 with preferential expression in adrenal and inducible by adrenal glucocorticoid, as a novel putative tumor suppressor gene for PPGLs. Copy number deletion and GIPC2 promoter hypermethylation but not GIPC2 mutation, accompanied with reduced GIPC2 expression, were observed in 39 of 55 PPGLs in our cohort. Examination of a published expression database consisting of 188 PPGLs found little GIPC2 expression in Cluster 1A (SDHx-associated) and Cluster 2A (NF1/RET-associated) tumors, but less pronounced reduction of GIPC2 expression in Cluster 1B (VHL-associated) and Cluster 2B/2C tumors. GIPC2 induced p27, suppressed MAPK/ERK and HIF-1ɑ pathways as well as cancer cell proliferation. Overexpressing GIPC2 in PC12 cells inhibited tumor growth in nude mice. We found GIPC2 interacted with the nucleoprotein NONO and both proteins regulated p27 transcription through the same GGCC box on p27 promoter. Significantly, low expression of both GIPC2 and p27 was associated with shorter disease-free survival time of PPGLs patients in the TCGA database. We found that PPGL-causing mutations in RET and in SDHB could lead to primary rat adrenal chromaffin cell proliferation, ERK activation, and p27 downregulation, all requiring downregulating GIPC2. Notably, the RET-mutant effect required the presence of dexamethasone while the SDHB-mutant effect required its absence, providing a plausible explanation for the tumor location preference. In contrast, the PPGL-predisposing VHL mutations had no effect on proliferation and GIPC2 expression but caused p53 downregulation and reduced apoptosis in chromaffin cells compared with wild-type VHL. Thus, our study raises the importance of cortical hormone in PPGL development, and GIPC2 as a novel tumor suppressor provides a unified molecular mechanism for the tumorigenesis of both sporadic and hereditary tumors of Clusters 1A and 2A concerning SDHB and RET, but not tumors of Cluster 1B concerning VHL and other clusters.

Research Protocol For A Pilot Study. The Effect Of ST (36) And Ren12 In The Control Of Hyperglycemia In Diabetic Patients

These acupunture points stimulated with EA have shown their hypoglycemic effect in animal models. One of the main mechanisms by which this effect is supported is the regulation of the hypothalamic-pituitary-adrenal axis, regulating the secretion of adrenal cortical hormone.

Effects of transcranial direct current stimulation on cognitive function in delayed encephalopathy after carbon monoxide poisoning

Objective: To investigate the effect of transcranial direct current stimulation (tDCS) on cognitive function of delayed encephalopathy after carbon monoxide poisoning (DEACMP) . Methods: A total of 58 patients with DEACMP admitted to the Emergency Medicine Department of Hudson International Peace Hospital from January 2018 to January 2020 were included. According to the random number table, the patients were randomly divided into study group and control group, with 29 patients in each group. Patients in both groups were given nutritional nerve therapy, improved microcirculation, adrenal cortical hormone and other drugs, as well as hyperbaric oxygen and rehabilitation training, once per day. The study group was treated with tDCS (electrode pads were placed and current stimulation was given 30 min/time) 1/d on the basis of conventional treatment, while the control group was treated with tDCS pseudo stimulation (electrode pads were placed and current stimulation was given for 10 s) 1/d for 30 days consecutively. The auditory event-related potential P300 (ERP-P300) was tested before and 30 days after treatment, and the Mini-Mental State Examination (MMSE) scale, Barthel index (BI) and the National Institutes of Health Neurological Impairment score (NIHSS) were used to evaluate and analyze the patients. Results: There was no significant difference in scores between the two groups before treatment (all P>0.05) . After 30 days of treatment, the MMSE score, orientation, memory, attention and computing power, and language ability of the study group were all higher than those of the control group, and the differences were statistically significant (P<0.05) . 90 days after treatment, the BI of the study group was higher than that of the control group (P<0.05) . After 30 days of treatment, the NIHSS score of the study group was significantly lower than that of the control group (P<0.05) . The latency of ERP-P300 in the study group was shorter than that in the control group, and the amplitude was higher than that in the control group at 30 days after treatment, and the difference was statistically significant (P<0.05) . The latency of ERP-P300 was negatively correlated with the total MMSE score before and after DEACMP treatment (r=-0.837, -0.819, P<0.05) . The latency of ERP-P300 was negatively correlated with orientation, attention and computing power, and language ability before treatment (r=-0.698, -0.675, -0.742, P<0.05) . Conclusion: TDCS treatment can help improve the cognitive function of DEACMP patients, and ERP-P300 test can help determine the cognitive function severity of patients.

Biosynthesis and Industrial Production of Androsteroids

Steroids are a group of organic compounds that include sex hormones, adrenal cortical hormones, sterols, and phytosterols. In mammals, steroid biosynthesis starts from cholesterol via multiple steps to the final steroid and occurs in the gonads, adrenal glands, and placenta. This highly regulated pathway involves several cytochrome P450, as well as different dehydrogenases and reductases. Steroids in mammals have also been associated with drug production. Steroid pharmaceuticals such as testosterone and progesterone represent the second largest category of marketed medical products. There heterologous production through microbial transformation of phytosterols has gained interest in the last couple of decades. Phytosterols being the plants sterols serve as inexpensive substrates for the production of steroid derivatives. Various genes and biochemical pathways involved in phytosterol degradation have been identified in many Rhodococcus and Mycobacterium species. Apart from an early investigation in mammals, presence of steroids such as androsteroids and progesterone has also been demonstrated in plants. Their main role is linked with growth, development, and reproduction. Even though plants share some chemical features with mammals, the biosynthesis is different, with the first C22 hydroxylation as an example. This is performed by CYP11A1 in mammals and CYP90B1 in plants. Moreover, the entire plant steroid biosynthesis is not fully elucidated. Knowing this pathway could provide new processes for the industrial biotechnological production of steroid hormones in plants.

Characterization of Group I Metabotropic Glutamate Receptors in Rat and Human Adrenal Glands.

Glutamate and its receptors have been demonstrated to promote both basal and nicotine-evoked catecholamine release in bovine chromaffin cells. Multiple glutamate receptors, including metabotropic glutamate receptors (mGluRs), are found in the adrenal glands of several species, as well as in chromaffin cells. However, there is limited information available regarding the expression of glutamate metabotropic receptor (GRM)1-8 mRNAs and the detailed localization of group I mGluRs (mGluR1 and mGluR5) in the rat and human adrenal cortex and medulla. Therefore, we examined mRNA expression of GRM1-8 subunits using reverse transcription-polymerase chain reaction (RT-PCR) and the distribution of mGluR1 and mGluR5 by immunostaining. The results showed that the GRM1-8 mRNAs were expressed in both the cortex and medulla of rat and human adrenal glands with the exception of GRM1, which was not detectable in the rat adrenal cortex. Immunostaining of mGluR1 revealed that it was localized only in the adrenal medulla of rats but was present in both the adrenal cortex and medulla in humans. In the adrenal medulla, the central part of the adrenal glands, mGluR1 was detected in chromaffin cells but not in nerve fibers and ganglion cells. Immunoactivity of mGluR5 was visible in the capillary wall throughout the adrenal cortex and medulla in rat and human samples. Its immunoactivity was also observed in ganglion cells in the rat adrenal medulla. There was no mGluR5 immunoactivity detected in chromaffin cells and nerve fibers in the rat and human adrenal medulla. Using dissected rat adrenal medulla as a model, we found that treatment with a mGluR1 agonist activated extracellular signal-regulated kinase (ERK) 1/2 and increased the expression of tyrosine hydroxylase (TH), the rate-limiting enzyme of catecholamine synthesis. Moreover, these results showed that mGluR1 signaling was involved in hypoxia-induced upregulation of TH in the rat adrenal medulla. This study shows the expression of GRM1-8 mRNAs in rat and human adrenal glands and indicates that glutamate, through the activation of mGluRs, may play various physiological roles in the adrenal gland. Furthermore, mGluR1 may be involved in catecholamine biosynthesis by regulating TH, and mGluR5 may affect cortical and medullar hormone levels by regulating microvascular function.

MON-076 Adrenal Hypoplasia Congenita - Is It Possible to Make This Diagnose in Previous “Idiopathic” Adrenal Insufficient Patients? Series of Cases

Background: Adrenal hypoplasia congenita (AHC) is a rare disease (1:70.000 men) characterized by reduction in all cortical adrenal hormones and also by hypogonadism. NR0B1 -related AHC includes both X-linked AHC and Xp21 deletion. AHC gene mutation was first described in 1994 and occurs in less than 1% of adrenal insufficiency (AI) cases and it is imperative that clinical endocrinologists increase their knowledge on this condition to recognize it as promptly as they were used to do so in other causes of pediatric AI.Clinical Cases: We describe here two sporadic cases and three familial cases from one family, all of them attending at our Endocrine Unit at HCPA, in the South of Brazil. The median age of AI first symptoms was 1 month old in two patients (acute infantile onset due to a salt wasting crises), and eleven years old in the remaining patients (childhood onset). Their exams confirmed the clinical suspicion of primary AI because of low plasma cortisol (from < 0.5 to 4 mcg/dl - reference range from 7-24 mcg/dl), as well as all adrenal steroids levels, high ACTH levels (from 672 to 2225 pg/ml - reference range from 7-63 pg/ml) and high plasmatic renin (from 92 to 448 mcUI/ml - reference range from 2.8-39.9 mcUI/ml). They were also extensively investigated searching for the AI pathophysiology. After some years of lost follow-up, one of the familial cases that had first been seen in 1993, returned to our hospital and was diagnosed with hypogonadism, leading to NR5OB1 gene evaluation.Regarding the genetic etiology, one of the sporadic cases developed a clinical picture including profound developmental delay, seizures and strabismus suggestive of a contiguous gene syndrome that involves Duchenne muscular dystrophy, glycerol kinase deficiency and AHC. A CGH array was performed and identified a Xp21.3-p21.1 deletion. The affected family had an extensive history suggestive of a genetic disease with X-linked inheritance pattern, as shown by the premature death of 3 uncles and a male cousin. A deleterious variant (c.131_212delinsTGAGACCTGTACCGT) in NR5OB1 gene was identified by Sanger sequencing in hemizygous state in the 46,XY affected patients.Conclusion: Albeit a rare disease, it is crucial that endocrinologist all around the World could be aware about clinical characteristics of this condition in order to properly diagnose it. And it is even possible, as it occurred with one of our patient, to make a late pathophysiologic diagnosis, making possible to treat the associated hypogonadism. Reference: Acherman JC, Vilain EJ. NR0B1- related adrenal hypoplasia congenita. GeneReviews 1993-2019.

Effects of dietary γ-aminobutyric acid supplementation on antioxidant status, blood hormones and meat quality in growing-finishing pigs undergoing transport stress.

γ-Aminobutyric acid (GABA) is a natural nonprotein amino acid distributed in animals, plants and microbes. GABA is an inhibiting neurotransmitter which takes great effect in mammalian central nervous system. We carried out the research to study the influence of GABA on blood hormone concentrations, antioxidant status and meat quality in fattening pigs after transportation. The 72 pigs with a starting weight of approximately 32.67±0.62kg were randomly allocated to 2 groups based on dietary treatments, containing 6 replicates with 6 pigs in each. The pigs were fed dietary supplementation of GABA (0 or 30mg/kg of diets) for 74days. Twelve pigs were randomly selected from each group and assigned to the either 1hr of transport (T group) or no transport (N group), resulting in two-factor factorial design. Compared to the control, GABA supplementation increased average daily gain (ADG) (p<.01) and decreased feed-gain ratio (F/G) (p<.05). The pH45min was lower and drip loss was greater in the longissimus muscles (LM) of post-slaughter of transported pigs (p<.05). The pH45min of 0/T group (group with 0mg/kg GABA and transport) was significantly lower than the pH45min of the 30/T group (diet×transport; p<.05). GABA supplementation significantly increased serum glutathione peroxidase (GSH-Px) concentration (p<.05) before transportation. Following transport, pigs fed GABA had decreased concentrations of serum malonaldehyde (MDA), adrenal cortical hormone and cortisol (p<.05). The results indicate that feeding GABA significantly increased the growth performance of growing-finishing pigs. The transportation model negatively impacted meat quality, antioxidant indexes and hormone parameters, but dietary supplementation of GABA could suppress the rise of drip loss of LM, ACTH and COR and suppress the drop of pH45min of LM after transportation stress in growing-finishing pigs. Feeding GABA alleviated transportation stress in pigs.

Bacillus amyloliquefaciens BLCC1-0238 Can Effectively Improve Laying Performance and Egg Quality Via Enhancing Immunity and Regulating Reproductive Hormones of Laying Hens.

In this study, we sought to evaluate the effects of dietary Bacillus amyloliquefaciens (B. amyloliquefaciens) BLCC1-0238 supplementation on laying performance, egg quality, antioxidant enzyme activities, reproductive hormone, and immunity of laying hens. A total of 240 Hy-Line Brown laying hens (28weeks old) were randomly divided into four groups, and three replicates per group (n = 20 per replicate). The control group was fed a standard basal diet, and the three treatment groups were provided the basal diet supplemented with either 0.01%, 0.03%, or 0.06% B. amyloliquefaciens BLCC1-0238 (2×1010CFU/g), respectively. Hens were allowed 2weeks to acclimate prior to initiation of the 8-week experiment. It was observed that dietary supplementation with 0.01% or 0.03% B. amyloliquefaciens BLCC1-0238 significantly increased egg production and egg mass. However, no significant differences in feed intake, egg weight, and feed conversion ratio among the four groups were observed. Different levels of B. amyloliquefaciens BLCC1-0238 supplementation also significantly increased egg shell strength and thickness. With respect to the levels of reproductive hormones in the hens, B. amyloliquefaciens BLCC1-0238 supplementation significantly reduced serum adrenal cortical hormone (ACTH) levels, while increasing estradiol (E2) and follicle-stimulating hormone (FSH) secretion in the treatment groups compared to the control group. Relative to the control group, supplementation with 0.03% and 0.06% B. amyloliquefaciens BLCC1-0238 was observed to significantly increase serum glutathione S-transferase (GST) concentration, and supplementation significantly reduced serum IL-1 and IL-6 levels, whereas IL-4 levels increased for all concentrations tested. In conclusion, supplementation of a basal chicken diet with B. amyloliquefaciens BLCC1-0238 can improve laying performance and egg quality through the reduction of stress responses, up-regulation of growth hormones, and supporting immunity in laying hens.

Clinical analysis of blood purification technology in the treatment of 23 cases with severe toadstool poisoning

Objective To observe the curative effect and experience of blood purification technology in the treatment of severe mushroom poisoning. Methods A total of 23 patients with severe mushroom poisoning in the Central Hospital of Pulandian District of Dalian were collected.On the basis of general therapy (emetic, gastric lavage, oral activated charcoal, catharsis, atropine, adrenal cortical hormone, symptomatic and supportive treatment), the patients were given blood purification therapy: hemoperfusion, 2 hours each time, 1 time every two days; application in series with hemodialysis, hemoperfusion; bedside hemofiltration was performed (8-10)h/ time. Results Of the 23 patients, 22 cases had liver injury (95.7%), 7 cases had renal injury (30.4%), 3 cases had central nervous system damage (13%), and 4 cases had other 17.4%.Hemoperfusion was performed in 22 patients, with an average of (3.6±0.4)times per day, and 19 patients with bedside hemofiltration, with an average of (2.9±0.5)times per patient.Outcome: 21 cases were improved, 2 cases died. Conclusion Blood purification treatment should be done as early as possible for mushroom poisoning patients, and it is an effective method for the treatment of severe mushroom poisoning. Key words: Hemodafitration; Mycotoxicosis; Poisoning; Multiple organ failure

Surgical treatment of ACTH-ectopic syndrome

ACTH-ectopic syndrome is a severe, multiple-symptom disease characterized by secretion of adrenocorticotropic hormone (ACTH) by ectopic tumor, increased release of adrenal cortical hormones and clinical picture of hypercorticism. Diagnosis and treatment of ACTH-ectopic syndrome is still difficult problem despite the achievements of modern medicine. There are several unresolved issues including optimal diagnostic algorithm, indications for various surgical procedures and their optimal dates. This review is devoted to these questions.