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1.
目的分析GATA3基因变异导致甲状旁腺功能减退症(hypoparathyroidism, HP)患者的临床特点及分子机制。方法在1975年至2020年间于北京协和医院内分泌科随诊并行靶向基因捕获联合二代测序的198例未成年人(≤18岁)起病的非手术性HP患者中筛查到5例GATA3基因致病/可疑致病性变异, 回顾性收集分析其临床资料, 并对基因检测结果进行生物信息学分析。结果 5例患者HP的起病年龄为0.5(0.1, 1.3)岁, 发病至诊断为HP和甲状旁腺功能减退-耳聋-肾发育不良(hypoparathyroidism-deafness-renal dysplasia, HDR)综合征的时间分别为(7.0±5.2)年和(15.0±5.4)年。临床表现为手足搐搦伴癫痫样发作、颅内钙化各5例, 白内障1例, 听力减退4例, 肾脏畸形或缺如2例。治疗前血钙和血甲状旁腺激素(PTH)分别为(1.65±0.31)mmol/L和(4.64±2.63)ng/L。5例患者GATA3基因的杂合变异, 分别引起无义突变、移码突变和剪接位点突变, 经Clin Var数据库预测及美国医学遗传学和基因组学学会(...  相似文献   
2.
巨噬细胞活化综合征(MAS)是成人Still病(AOSD)一类少见但严重的并发症,临床表现各异,预后欠佳。早期诊断及治疗对于改善临床预后有积极意义。目前对于成人Still病合并巨噬细胞活化综合征尚无统一的诊断标准及治疗指南。文章旨在对成人Still病合并巨噬细胞活化综合征的发病机制、诊断标准、临床表现、治疗进展等作一综述。  相似文献   
3.
黄帅立 《河南中医》2020,40(4):500-502
2019年为己亥之年,土运不足,厥阴风木司天,少阳相火在泉。下半年相火淫胜,火气流行,克金、刑水。风木盛则克制土运,土运本不足,致虚者更虚,强者更强,此谓之不相得,不相得则病;且气克运,古称之为天刑,天刑之年多灾害。2019年,冬季主气太阳寒水之上少阳相火客临,致冬不藏反长,天不寒反暖。地气封藏不固,水中阳气不藏,本应该被抑制或杀灭的微生物却因火热而未灭,并得少阳火毒助长而毒性更猛,化生为火疫。2020年为庚子年,主行金运,金克木,可平复2019年偏盛之木运,故瘟毒会被2020年之金运削弱或肃杀。针对此次瘟疫,未病之人预防要点为:虚邪贼风,避之有时;精神内守,病安从来;正气存内,邪不可干;饮食宜忌,尤为重要;避秽化浊,芳香化湿。已病之人的治疗,依据此次瘟疫"少阳火毒夹湿"病机,以咸补之,以甘泻之,以咸耎之,岁谷宜丹,间谷宜豆。此次瘟毒的性质虽为湿热火毒,但患者体质却有寒热虚实之别,感染后症状、病情和预后亦不同,故中医临证时应遵循辨证论治,三因治宜的原则;若体质素有湿热,邪从本气而化,可遵循运气病机施治;若体质本热,湿去而化为火毒,则应以制火为主;若体质本寒,热去而化寒湿,则应温化寒湿,芳香化浊;若体质素虚,出现危重证候,则应在祛邪时,重视固护元气。  相似文献   
4.
Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as glutaric acidemia type II, is classically caused by a congenital defect in electron transfer flavoprotein (ETF) or ETF dehydrogenase (ETFDH). Flavin adenine dinucleotide synthase (FADS) deficiency caused by mutations in FLAD1 was recently reported as a novel riboflavin metabolism disorder resembling MADD. Here, we describe a Japanese boy with FADS deficiency due to a novel mutation (p.R249*) in FLAD1. In the asymptomatic male infant born at full term, newborn screening showed positive results with elevated C5 and C14:1 acylcarnitine levels and an increased C14:1/C2 ratio. Biochemical studies were unremarkable except for lactic acidosis (pH 7.197, lactate 61 mg/dL). A diagnosis of MADD was suspected because of mild abnormalities of the acylcarnitine profile and apparent abnormalities of urinary organic acids, although mutations in the ETFA, ETFB, ETFDH, and riboflavin transporter genes (SLC52A1, SLC52A2, and SLC52A3) were not detected. Administration of riboflavin and L-carnitine was initiated at one month of age based on the diagnosis of “biochemical MADD” despite a lack of symptoms. Nevertheless, the acylcarnitine profile was not normalized. Symptoms resembling bulbar palsy, such as vocal cord paralysis and dyspnea with stridor, were present from 3 months of age. At 4 months of age, he became bedridden because of hypoxic-ischemic encephalopathy due to fulminant respiratory failure with aspiration pneumonia. At 2 years and 5 months of age, a homozygous c.745C > T (p.R249*) mutation in the FLAD1 gene was identified, confirming the diagnosis of FADS deficiency. His severe clinical course may be caused by this nonsense mutation associated with poor responsiveness to riboflavin. Persistent lactic acidosis and neuropathy, such as bulbar palsy, may be important for diagnosing FADS deficiency. Although the biochemical findings in FADS deficiency are similar to those in MADD, their clinical symptoms and severity may not be identical.  相似文献   
5.
《Clinical neurophysiology》2019,130(1):128-137
ObjectiveHigh frequency oscillations (HFO) between 80–500 Hz are markers of epileptic areas in intracranial and maybe also scalp EEG. We investigate simultaneous recordings of scalp and intracranial EEG and hypothesize that scalp HFOs provide important additional clinical information in the presurgical setting.MethodsSpikes and HFOs were visually identified in all intracranial scalp EEG channels. Analysis of correlation of event location between intracranial and scalp EEG as well as relationship between events and the SOZ and zone of surgical removal was performed.Results24 patients could be included, 23 showed spikes and 19 HFOs on scalp recordings. In 15/19 patients highest scalp HFO rate was located over the implantation side, with 13 patients having the highest scalp and intracranial HFO rate over the same region. 17 patients underwent surgery, 7 became seizure free. Patients with poor post-operative outcome showed significantly more regions with HFO than those with seizure free outcome.ConclusionsScalp HFOs are mostly located over the SOZ. Widespread scalp HFOs are indicative of a larger epileptic network and associated with poor postsurgical outcome.SignificanceAnalysis of scalp HFO add clinically important information about the extent of epileptic areas during presurgical simultaneous scalp and intracranial EEG recordings.  相似文献   
6.
7.
ObjectivesThe authors sought to assess the association between admission time with patient’s care, procedure characteristics, and clinical outcomes within a contemporary ST-segment elevation myocardial infarction (STEMI) network of patients referred for primary percutaneous coronary intervention (PCI).BackgroundThe effect of admission time on STEMI patient's outcomes remains controversial when primary PCI is the preferred reperfusion strategy.MethodsCharacteristics and clinical outcomes of 2,167 consecutive STEMI patients admitted in a tertiary PCI-capable center were collected. On-hours were defined as admission from Monday through Friday between 8 am and 6 pm and off-hours as admission during night shift, weekend, and nonworking holidays. In-hospital and 1-year all-cause mortality were assessed as well as key time delays.ResultsA total of 1,048 patients (48.3%) were admitted during on-hours, and 1,119 patients (51.7%) during off-hours. Characteristics were well-balanced between the 2 groups, including rates of cardiac arrest (7.9% vs. 8.8%; p = 0.55) and cardiogenic shock (12.3% vs. 14.7%; p = 0.16). Median symptom-to-first medical contact time and median first medical contact-to-sheath insertion time did not differ according to on- versus off-hours admission (120 min vs. 126 min; p = 0.25 and 90 min vs. 93 min; p = 0.58, respectively), as well as the rate of radial access for catheterization (85.6% vs. 87.5%; p = 0.27). There was no association between on- versus off-hours groups and in-hospital (8.1% vs. 7.0%; p = 0.49) or 1-year mortality (11.0% vs. 11.1%; p = 0.89), respectively.ConclusionsIn a contemporary organized STEMI network, patients admitted in a high-volume tertiary primary PCI center during on-hours or off-hours had similar management and 1-year outcomes.  相似文献   
8.
Coronavirus disease 2019 (COVID-19) is associated with poor cardiovascular outcomes in patients with heart failure (HF) of all categories of ejection fraction (EF), but mainly in patients with HF with reduced EF. Moreover, cardiac transplant patients exhibit worse cardiovascular prognosis, high mortality, and more admissions to the intensive care unit. In general, COVID-19 seems to de-teriorate the clinical status of HF and favors the development of acute respiratory distress syndrome and multiorgan failure, especially in the presence of cardiovascular comorbidities such as diabetes mellitus, kidney dysfunction, and older age. COVID-19 may induce new-onset HF with complex mechanisms that involve myocardial injury. Indeed, myocardial injury comprises a large category of detrimental effects for the myocardium, such as myocardial infarction type 1 or type 2, Takotsubo cardiomyopathy, microvascular dysfunction and myocarditis, which are not easily distinguished by HF. The pathophysiologic mechanisms mainly involve direct myocardial damage by severe acute respiratory syndrome coronavirus 2, cytokine storm, hypercoagulation, inflammation, and endothelial dysfunction. The proper management of patients with COVID-19 involves careful patient evaluation and ongoing monitoring for complications such as HF.  相似文献   
9.
目的 观察凝血、甘油三酯(triglyceride, TG)在早发型重度子痫前期(early onset severe pre-eclampsia, EOSP)孕妇中的表达水平,并分析凝血、TG水平预测EOSP孕妇分娩结局的价值。方法 回顾性分析2019年1月至2020年12月于邯郸市中心医院产检并完成分娩的55例EOSP不良分娩结局孕妇的临床资料,设为不良组;另收集同期于本院产检并完成分娩的55例EOSP分娩结局良好孕妇的临床资料,设为良好组;收集孕妇病历资料,记录基线资料、凝血四项[凝血酶原时间(prothrombin time PT)、凝血酶时间(thrombin time, TT)、纤维蛋白原(fibrinogen, FIB)、活化部分凝血活酶时间(activatedpartial thromboplastin time, APTT)]及TG水平,并分析凝血、TG水平预测EOSP孕妇不良分娩结局风险的价值。结果 两组年龄、分娩时孕周、产妇类型、体质量指数、TT水平比较,差异无统计学意义(P>0.05);不良组孕妇PT、APTT、高密度脂蛋白胆固醇(high density lipoprotein cholesterol, HDL-C)水平均低于良好组,FIB、TG、总胆固醇(total cholesterol, TC)、低密度脂蛋白胆固醇(low density lipoprotein cholesterol, LDL-C)水平均高于良好组(P<0.05);相关性检验发现,EOSP孕妇分娩结局与PT、APTT、HDL-C水平呈负相关(r<0,P<0.05),与FIB、TG、TC、LDL-C水平呈正相关(r>0,P<0.05),与TT水平无相关性(P>0.05);绘制ROC曲线,血清PT、APTT低表达、TG、FIB过表达预测EOSP孕妇不良分娩结局风险的曲线下面积(AUC)>0.80,有一定预测价值,且当四者cut-off值分别取10.13 s、23.47 s、2.64 mmol/L、4.54 g/L时,可获得最佳预测价值。结论 凝血、TG与EOSP孕妇分娩结局有关,PT、APTT低表达、TG、FIB过表达预测EOSP孕妇不良分娩结局风险有一定价值,可考虑早期监测EOSP孕妇凝血、TG指标,指导不良分娩结局风险预测。  相似文献   
10.
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