Risk factors and etiology of childhood hearing loss: a cohort review of 296 subjects

Abstract

Background: The association between the Joint Committee on Infant Hearing (JCIH) risk factors and etiology of hearing loss (HL) is not studied well in children.

Objectives: To clarify the etiologic causes and evaluate the JCIH risk characteristics of children with HL.

Methods: A retrospective study of 296 children with HL born between 2009.01 and 2013.12 in Stokholm. Demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and analyzed.

Results: In 221 with bilateral hearing loss (BHL), family history and neonatal risk indicators were the most common risks (59 each), followed by syndrome related risks. In 75 with unilateral hearing loss (UHL), craniofacial anomaly was the most common risk, followed by family history. Etiology was established in 93 with BHL, in which syndromic HL accounted for 37.2%, chromosomal aberrations for 21.3%, and environmental causes for 19.1%. Etiology was established in 35 with UHL, in which ear malformation accounted for the most (74.3%), followed by environmental causes (14.3%).

Conclusions and significance: Childhood HL can be attributed to a variety of causes with an etiology identifiable in 42.5% of BHL and 46.7% of UHL. BHL and UHL have different patterns of JCIH risk exposure and etiology.     

孕前超重/肥胖的妊娠期糖尿病孕妇血糖管理：基于静息能量消耗计算每日总能量消耗

目的探讨基于静息能量消耗(REE)计算每日总能量消耗(TEE)，对孕前超重/肥胖的妊娠期糖尿病(GDM)孕妇血糖管理意义。 方法选择2018年6月至2019年6月，于首都医科大学附属北京潞河医院产科"糖尿病一日门诊"就诊时，被诊断为孕前超重/肥胖的67例GDM孕妇为研究对象。按照其首次就诊时间的奇、偶周，将其分为研究组(n＝34，首次就诊时间为奇数周者)和对照组(n＝33，首次就诊时间为偶数周者)。采用成组t检验对2组孕妇年龄、孕次、孕期体重增加值、新生儿出生体重、空腹血糖、餐后2 h血糖等进行统计学分析。采用Mann-Whitney U检验对2组孕妇分娩孕龄、产次等进行统计学分析。采用χ²检验、Fisher确切概率法、连续性校正χ²检验对2组孕妇尿酮体阳性率、巨大儿发生率等进行统计学分析。本研究经首都医科大学附属北京潞河医院医学伦理委员审核批准(审批文号：2018-LHKY-027-02)，并与所有受试者签署知情同意书。 结果①2组GDM孕妇年龄、孕次、产次、分娩孕龄及孕前人体质量指数(BMI)构成比比较，差异均无统计学意义(P>0.05)。②研究组孕妇TEE为(1 532.6±301.9)kcal/d，显著低于对照组的(1 824.1±313.2) kcal/d，并且差异有统计学意义(t＝3.311、P＝0.002)；2组孕妇血糖达标情况构成比比较，差异亦有统计学意义(P＝0.040)。③研究组孕妇孕期体重增加值、糖化血红蛋白(HbA1c)水平和新生儿出生体重分别为(13.4±6.2)kg、(5.6±0.3)%和(3 287.5±577.1)g，明显低于对照组的(18.2±4.8)kg、(6.2±0.2)%和(3 632.8±490.6)g，并且差异均有统计学意义(t＝2.235、P＝0.031，t＝2.882、P＝0.047，t＝2.121、P＝0.039)。 结论基于REE估算的TEE，较通用系数法估算的TEE，更接近于孕前超重/肥胖GDM孕妇的实际能量需求。因此，基于REE估算的TEE更有利于对孕前超重/肥胖的GDM进行孕期血糖管理。     

Gastric bypass surgery is associated with reduced subclinical myocardial injury and greater activation of the cardiac natriuretic peptide system than lifestyle intervention

AimsMorbid obesity is a risk factor for cardiovascular disease. The relative effects of Roux-en-Y gastric bypass surgery (GBS) and intensive lifestyle intervention (ILI) on subclinical myocardial injury, the activity of the cardiac natriuretic system, and systemic inflammation remain unclear.MethodsIn a 59-week non-randomized clinical trial that included 131 patients with morbid obesity, we compared the effects of GBS and ILI on concentrations of cardiac troponin T (cTnT) and I (cTnI), N-terminal pro-B-type natriuretic peptide (NT-proBNP) and C-reactive protein (CRP).ResultsIn the GBS and ILI group, median body mass index (BMI) was reduced by 14.4 kg/m² versus 3.9 kg/m², respectively (p value < 0.001). Cardiac troponins decreased after GBS, p = 0.014 (cTnT) and p = 0.065 (cTnI) and increased significantly in those treated with ILI (p values ≤ 0.021) (between-group differences for deltas: p ≤ 0.003). NT-proBNP increased in both groups, but significantly more in the GBS than in the ILI group (between-group differences for deltas: p = 0.008). CRP decreased significantly within the GBS and the ILI group, with this change significantly greater in the GBS group (between-group differences for deltas p < 0.001). The dominating mediator of the biomarker changes was weight loss. Prior coronary artery disease and diabetes were predictive of the magnitude of the changes in cTnI and NT-proBNP, respectively.ConclusionCompared to ILI, GBS was associated with reduced subclinical myocardial injury and systemic inflammation, and enhancement of the cardiac natriuretic peptide system. The biomarker changes were predominantly mediated by weight loss.     

消化道恶性肿瘤住院患者的能量消耗研究

目的评估消化道恶性肿瘤患者的能量消耗，探讨最佳计算公式及能量消耗的影响因素。方法采用连续入组法，纳入2016年3月至2016年12月在陆军军医大学第一附属医院肿瘤科住院治疗患者，运用代谢车测定其静息代谢能量（REE），使用Harris-Benedict公式和30kcal/（kg·d） 公式预测患者的一日总能量消耗（TEE）。收集研究对象的相关指标如年龄、身高、体重、病程、原位癌部位、是否荷瘤等。结果共纳入26例患者，其中包括食管癌11例，胃癌8例，结直肠癌7例，73%的患者处于高代谢状态，约69%的患者处于肿瘤Ⅳ期；其中不同病程和原位癌位置与静息能量消耗有差异，差异具有统计学意义；用30kcal/（kg·d）×体重估算TEE可能并不适用于消瘦的消化道肿瘤患者。结论消化道恶性肿瘤患者大多存在营养不良且处于高代谢状态，在给消化道恶性肿瘤患者提供能量时应适当考虑病程长短、肿瘤分期以及肿瘤部位等因素。尽量使用代谢车估算恶性肿瘤患者的TEE，若没有代谢车条件时，对于能下床活动的消化道恶性肿瘤患者，体质指数（BMI）≥18.5kg/m2者推荐使用30kcal/（kg·d）×实际体重的方法估算TEE，BMI<18.5kg/m2者推荐使用30kcal/（kg·d）×标准体重的方法估算TEE。     

Genetic testing has the potential to impact hearing preservation following cochlear implantation

Abstract

Background: Recent advances in less-invasive surgery and electrode design allow for a high degree of hearing preservation (HP) after cochlear implantation (CI), although residual hearing still deteriorates in some patients. To date, the factors predictive of preserving residual hearing remain a controversial topic.

Objective: The aim of this study was to investigate the predictive factors, including the etiology of hearing loss (HL) as a patient-related factor, influencing residual HP after CI.

Methods: Forty-four patients (50 ears, 41 families) with residual acoustic hearing who underwent CI were included. Auditory thresholds before and at 6 months after initial activation were measured. Genetic testing was performed to identify the responsible genes for HL.

Results: We identified the cause of HL in 21 families (51.2%). HP was marginally correlated with age at implantation, while it was independent of pre-operative low-frequency hearing thresholds, cochlear duct length, and electrode length. We found that patients who had pathogenic variants in the CDH23, MYO7A, or MYO15A gene showed statistically better HP scores compared with patients with HL due to other causes (p?=?.002).

Conclusions: Identification of the etiology of HL using genetic testing is likely to facilitate the prediction of HP after implant surgery.     

A proposed mechanism for central centrifugal cicatricial alopecia

Central centrifugal cicatricial alopecia (CCCA) has an unknown mechanism. Analyzing other scarring diseases (lichen planopilaris, fibrotic kidney disease and scleroderma) may help to clarify the mechanism of scarring in CCCA. These diseases were chosen for comparison due to either their location of disease (skin or scalp specifically), or prominence in patients of African descent. Genetics, possible triggers, an autoimmune lymphocytic response, and epithelial to mesenchymal transition are potentially involved. Possible common pathways in scarring diseases and a better understanding of the CCCA mechanism will lead to further research into the pathogenesis and potential treatments of CCCA.     

Nobel clinician软件引导上颌窦内提升术在磨牙缺失修复中的应用研究

目的:研究Nobelclinician软件引导上颌窦内提升术在磨牙缺失的应用效果,为临床应用提供指导。方法:选择2016年10月-2018年10月在笔者医院进行手术的86例上颌磨牙缺失患者为研究对象,按照随机数表法分为观察组和对照组,每组43例。对照组:采用常规上颔窦内提升术;观察组:在Nobelclinician软件引导下进行上颌窦内提升术,两组均使用nobelreplace种植体种植。比较两组患者的种植体存留率及种植体周围组织情况[包括术前上颌窦底距牙槽嵴骨高度(Bone height of the maxillary sinus floor from the alveolar ridge,RBH)、术后6个月新骨获得量、术后1年垂直骨丧失、牙周探诊深度(Periodontal probing depth,PPD)];比较两组患者治疗后的X片检查结果及治疗满意度。结果:观察组的RBH、PPD及术后1年垂直骨丧失均显著小于对照组,术后6个月新骨获得量显著大于对照组,两组比较差异具有统计学意义(P<0.05)。治疗后随访6个月,观察组的种植体存留率(100.00%)与对照组(94.55%)相比,差异不具有统计学意义(P>0.05),X片检查结果显示,两组患者的种植体及周围骨组织均结合良好,术后1年均已生成新的上颌窦底壁。观察组患者的满意度(100.00%)显著高于对照组(90.70%),差异具有统计学意义(P<0.05)。结论:Nobelclinician软件引导上颌窦内提升术能够降低垂直骨丧失量,增加新骨获得量,有助于提高磨牙缺失修复的美学效果,提高患者满意度。     

Outcomes of Acetabular Reconstructions for the Management of Chronic Pelvic Discontinuity: A Systematic Review

BackgroundA number of articles have been published reporting on the clinical outcomes of various acetabular reconstructions for the management of chronic pelvic discontinuity (PD). However, no systematic review of the literature has been published to date comparing the outcome and complications of different approaches to reconstruction.MethodsThe US National Library of Medicine (PubMed/MEDLINE) and EMBASE were queried for publications from January 1980 to January 2019 using keywords pertinent to total hip arthroplasty, PD, acetabular dissociation, clinical or functional outcomes, and revision total hip arthroplasty or postoperative complications.ResultsOverall, 18 articles were included in this analysis (569 cases with chronic PD). The overall survival rate of the acetabular components used for the treatment of chronic PD was 84.7% (482 of 569 cases) at mid-term follow-up, whereas the most common reasons for revision were aseptic loosening (54 of 569 hips; 9.5%), dislocations (45 of 569 hips; 7.9%), periprosthetic joint infection (30 of 569 hips; 5.3%), and periprosthetic fractures (11 of 569 hips; 1.9%). Both pelvic distraction technique (combined with highly porous shells) and custom triflanges resulted in less than 5% failure rates (96.2% and 95.8%, respectively) at final follow-up. Also, highly effective in the treatment of PD were cup-cages and highly porous shells with and/or without augments with 92% survivorship free of revision for aseptic loosening for both reconstruction methods. Inferior outcomes were reported for conventional cementless shells combined with acetabular plates (72.7%) as well as ilioischial cages and reconstruction rings (66.7% and 60.6% survivorship, respectively).ConclusionThe current literature contains moderate quality evidence in support of the use of custom triflange implants and pelvic distraction techniques for the treatment of chronic PD, with a less than 5% all-cause revision rate and low complication rates at mean mid-term follow-up. Cup-cages and highly porous shells with or without augments could also be considered for the treatment of PD because both resulted in greater than 90% survival rates. Finally, there is still no consensus regarding the impact of different types of acetabular reconstruction methods on optimizing the healing potential of PD, and further studies are required in this area to better understand the influence of PD healing on construct survivorship and functional outcomes with each reconstruction method.