Prader–Willi syndrome: From genetics to behaviour,with special focus on appetite treatments

Prader–Willi syndrome (PWS) is a neurodevelopmental disorder resulting from a deletion in the expression of the paternally derived alleles in the region of 15q11–q13. PWS has a prevalence rate of 1:10,000–1:30,000 and is characterized by marked endocrine abnormalities including growth hormone deficiency and raised ghrelin levels.The hyperphagic phenotype in PWS is established over a number of phases and is exacerbated by impaired satiety, low energy expenditure and intellectual difficulties including obsessive–compulsive disorder and/or autistic behaviours. Clinical management in PWS typically includes familial/carer restriction and close supervision of food intake. If the supervision of food is left unmanaged, morbid obesity eventuates, central to the risk of cardiorespiratory disorder. None of the current appetite management/intervention strategies for PWS include pharmacological treatment, though recent research shows some promise.We review the established aberrant genetics and the endocrine and neuronal attributes which may determine disturbed regulatory processes in PWS. Focusing on clinical trials for appetite behaviours in PWS, we define the effectiveness of pharmacological treatments with a view to initiating and focusing research towards possible targets for modulating appetite in PWS.     

PTTG在脑膜瘤组织芯片中的表达及意义

目的:应用免疫组化技术,研究垂体瘤转化基因(PTTG)在脑膜瘤组织芯片中的表达,进一步探讨其在脑膜瘤恶变、侵袭中的作用。方法:收集手术切除的脑膜瘤蜡块标本84例,以17例正常人硬脑膜为对照,采用免疫组化组织芯片技术检测PTTG在不同病理级别和不同侵袭性脑膜瘤中的表达。结果:正常硬脑膜组织中未见PTTG的表达,84例脑膜瘤中PTTG的阳性表达率为86.90%。良性及非良性脑膜瘤和侵袭性及非侵袭性脑膜瘤组间的PTTG表达阳性率差异无统计学意义(P>0.05);但组间染色阳性系数≥4分的阳性表达率差异有统计学意义(P<0.05)。结论:PTTG在大多数的脑膜瘤中有表达,恶性、侵袭性脑膜瘤中表达显著,提示其在人脑膜瘤发生、恶变、侵袭中发挥关键作用。     

半枝莲总黄酮对ApoE基因敲除小鼠血清PLTP、IL-6、CRP表达的影响

 目的探讨半枝莲总黄酮对载脂蛋白E基因敲除小鼠AS病变形成早期血脂水平与血清磷脂转运蛋白（PLTP）、Il-6、C
反应蛋白（CRP）表达的影响。方法载脂蛋白E 基因敲除小鼠随机分为模型组、辛伐他汀组、银杏叶胶囊组、半枝莲总黄酮高、
中、低剂量组,取正常C57BL/6 小鼠为对照组,各6 只。除模型组、对照组给予等容量羧甲基纤维素钠外,其他各组分别给予阳性
对照药与受试药8 周。处死小鼠,ELISA 法检测血脂、PLTP、IL-6 及CRP 的表达水平。取主动脉制作切片、HE染色,观察形态学
变化。结果半枝莲总黄酮中、高剂量组TG、TC、LDL-C 水平明显低于模型组,高剂量组HDL-C 水平高于模型组,半枝莲总黄
酮各组PLTP、IL-6、CPR的表达水平较模型组明显降低,有统计学意义。PLTP与IL-6 水平呈正相关,与CRP 水平呈正相关,IL-6
与CRP 水平呈正相关。结论半枝莲总黄酮可通过降低载脂蛋白E 基因敲除小鼠TG、TC、LDL-C 水平,升高HDL-C 水平,降
低PLTP、IL-6、CPR 水平而发挥抗AS 的作用。     

Halofuginone inhibits TNF-α-induced the migration and proliferation of fibroblast-like synoviocytes from rheumatoid arthritis patients

Fibroblast-like synoviocytes (FLSs) display an aggressive phenotype that is a critical factor in cartilage destruction in rheumatoid arthritis (RA). Increased FLS migration and proliferation are essential to the pathology of RA. Halofuginone has been found to inhibit cell migration and proliferation in cancer cells. However, whether halofuginone has a role in the treatment of RA FLSs is unclear. In this study, we found that halofuginone reduced migration, invasion, cell proliferation and MMPs expression in RA FLSs. In addition, we demonstrated that halofuginone inhibited reorganization of the actin cytoskeleton during cell migration. To gain insight into the molecular mechanisms, we evaluated the effect of halofuginone on the MAPK and AKT pathways. Our results indicated that halofuginone inhibited the activity of MAPK and AKT. Taken together, these results suggest that halofuginone may protect against joint destruction in RA by regulating synoviocyte migration, invasion and cell proliferation by inhibiting MAPK and AKT activation.     

Quinoline alkaloids isolated from Scolopendra subspinipes mutilans

Objective To study the quinoline alkaloids from the ethanol extract of Scolopendra subspinipes mutilans (SSM). Methods The chemical constituents were isolated and purified by macroporous resin column, medium pressure preparation chromatography, and semi-preparative HPLC. Their structures were elucidated by IR, MS, and NMR experiments. Results Three quinolone alkaloids were obtained and identified as 3-hydroxy-4-methoxyquinolin-8-yl hydrogen sulfate (1), jineol-8-sulfate (2), and jineol (3), respectively. Conclusion Compound 1 is a new compound from SSM.     

周仲瑛教授病机辨证颅内肿瘤的临床经验

介绍了周仲瑛教授辨治颅内肿瘤的临床经验和学术思想。认为颅内肿瘤基本病机为肝肾亏虚、风痰瘀阻、清阳失用。周老辨治颅内肿瘤以病证结合为主,重视基本病机辨证,临证围绕基本病机,因机立法,随证加减。治疗强调标实急于本虚,祛邪先于扶正。      

Progression of quality of life in patients with grade 3 obesity after a 36 months care pathway with and without bariatric surgery

ObjectivesBeyond somatic consequences, excess weight affects quality of life. In the literature this has markedly improved in the first 6 months, but has decreased later. The originality of our work in that is based on the comparison of two groups of patients, surgical and non-surgical ones, monitored by a multi-professional team for 3 years.MethodsThe sample is comprised of 32 subjects divided into two groups: 17 subjects from the non-surgical pathway and 15 from the surgical one. The Quality of Life, Obesity, and Dietetics questionnaire (QOLOD), the Hospital Anxiety and Depression Scale (HADS) and the Dutch Eating Behaviour Questionnaire (DEBQ), age, BMI, and sex were recorded.ResultsAt baseline, age was significantly different between two groups (surgery vs. non-surgery). BMI and QOLOD improvement were significantly more important in surgical group (?13.6 kg [?19.2; ?10.8] vs. 0.0 kg [?2.8; 3.0] P < 0.001 and 16.9 [8.5; 32.2 vs. 2.1 [?9.4; 16.2], P = 0.004). The progression of the QOLOD score correlated negatively with the progression of BMI (r = ?0.5, P = 0.005)ConclusionThere is a link between post-surgery weight loss and improved quality of life at 36 months. We suggest a qualitative follow-up survey for surgical and non-surgical obese patients.     

Complications hépatiques de la nutrition artificielle parentérale chez l’adulte en 2022 : de la surveillance au traitement

Hepatopathy related to parenteral nutrition, because of its possible evolution towards cirrhosis and liver failure, has a negative impact on the prognosis of patients with intestinal failure. The pathogenesis of this complication is multifactorial, ranging from the composition of the parenteral nutrition to the length of the remaining small intestine, and to date there is no consensual definition. Its diagnosis is essentially based on a range of biological, radiological and/or histological arguments. Its treatment is based on prevention, identification of risk factors, adaptation of carbohydrate-lipid intake and the composition of lipid emulsions and the earliest possible management of any sepsis, related to venous access or bacterial translocation. In the future, additional studies are needed to better understand the pathophysiological mechanisms of this complication, to propose a diagnostic strategy, adapted follow-up and innovative preventive and curative therapies.     

Troubles des conduites alimentaires : prévalence et facteurs de risque chez des étudiants en médecine tunisiens

ObjectivesYoung students, in particular those in the medical field, seem to be at risk of developing psychiatric disorders, especially eating disorders (ED).We aimed to determine the prevalence of ED in Tunisian medical students and to study the risk factors associated to it.Materials and methodsWe conducted a cross-sectional study at the Tunis medical university including 250 students. Each student filled out a self-administered questionnaire with the necessary individual information as well as the HAD (Hospital Anxiety and Depression) scale assessing anxiety and depression, EDI2 (Eating Disorder Inventory 2) assessing the cognitive behavioral profile of participants and PSS (Perceived Stress Scale), assessing their level of perceived stress. Eating disorders were screened using the EAT40 and BITE self-questionnaires. We used the DSM-5 classification to specify the types of ED.ResultsThe sex ratio (M/F) was 0.4. Anxiety, depressive symptoms and high stress level were found in 34%, 17% and 75.2% of cases, respectively. The prevalence of ED was 16.4%. Independent factors associated with ED were female sex (P = 0.009), physical activity (P = 0.04), body dissatisfaction (P < 0.001) and the feeling of ineffectiveness (P = 0.032). The risk of ED was five times higher in students using anxiolytics (P < 0.001), laxatives (P = 0.046), or induced vomiting (P = 0.045) and 9.55 times higher in students on a diet (P < 0.001).ConclusionThese results justify the need for systematic screening for ED in medical students in order to ensure early and effective treatment and thus preserve the physical and mental health of future caregivers.