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Treatment options and anesthetic considerations for postnatal management of congenital lung malformation 先天性肺畸形产后处理的治疗方案和麻醉注意事项
Q4 Medicine Pub Date : 2022-09-30 DOI: 10.37897/rjp.2022.3.6
Valeria Vlasceanu Coman, G. Dragan, V. Plaiasu, M. Pavelescu, G. Jugulete, M. Oros
Congenital lung malformations or CLM have resulted in an apparent increase in their frequency with the advances in fetal ultrasound technology and more widely available investigations in prenatal and postnatal periods. The advantage of an early diagnosis came together with the difficulty of the treatment decision, in special in the postnatal management of asymptomatic CLM. In this article, we summarized findings from current literature, with a particular emphasis on the therapeutic strategies. Management of infants for thoracic surgery represents a challenge not only for pediatric surgeon but also for anesthesiologists because of the anatomic and physiologic variations particularities. There are two main important parts that must be obtained: one lung ventilation and specific techniques for optimal pain management, distinguishing pediatric thoracic anesthesia from other pediatric anesthetic disciplines A comprehensive multimodal approach is necessarily followed by a long-term assessment of respiratory function of children who underwent surgery for lung resection.
随着胎儿超声技术的进步以及产前和产后更广泛的研究,先天性肺畸形或CLM的频率明显增加。早期诊断的优势与治疗决策的困难同时存在,特别是在无症状CLM的产后管理方面。在这篇文章中,我们总结了当前文献的发现,特别强调了治疗策略。由于解剖和生理变化的特殊性,婴儿胸部手术的管理不仅对儿科外科医生,而且对麻醉师来说都是一个挑战。必须获得两个主要的重要部分:单肺通气和最佳疼痛管理的特定技术,将儿童胸部麻醉与其他儿童麻醉学科区分开来。必须遵循综合的多模式方法,对接受肺切除手术的儿童的呼吸功能进行长期评估。
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引用次数: 0
Epidemiological aspects in phenylketonuria patients from a region in northwestern Romania 罗马尼亚西北部一地区苯丙酮尿症患者的流行病学特征
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.2
A. Iuhas, Claudia Jurca, M. Bembea
Introduction. Phenylketonuria (PKU), a genetic disease with autosomal dominant transmission, is the most frequent inborn error in aminoacidic metabolism. The variations in phenylalanine-hydroxylase (PAH) gene lead to a lowered enzymatic activity causing hyperphenylalaninemia. PKU has a mean European prevalence of 1:10,000 newborns, with a large variation in different ethnicities and geographic regions. The large genetic variability (over 1200 genetic variants known) as well as other factors determines a wide spectrum of metabolic phenotypes. Untreated, PKU leads to irreversible intellectual disability, low stature, hypopigmentation, motor deficits, seizures, but the early diagnosis and treatment enables almost normal somatic and mental development. Aim. The aim of this study is the determination of the impact of non-genetic factors over the clinical phenotype of PKU patients in a region of north-west Romania. Material and method. The study group is formed from 44 patients diagnosed with phenylketonuria in the 1981 – 2021 period, found in the database of Bihor Regional Center for Medical Genetics, Emergency Clinical County Hospital, Oradea, Romania. The collected data was referring to the age, sex and domicile of the patients, the age of the diagnosis and the beginning of the treatment, also the metabolic control over the years, the metabolic phenotype of the patients and its impact on the clinical phenotype (IQ, the presence or absence of intellectual disability or the existence of a specific clinical phenotype). Results. The majority of patients (66%) were diagnosticated with phenylketonuria in the first 4 months of life, although there were cases with a late diagnosis, 20.5% of the patients were diagnosticated after the age of 1 year. Based on the pre-treatment plasmatic levels of phenylalanine, the majority of cases (72.7%) had a severe metabolic phenotype (classic PKU - cPKU), 20.5% of cases had a milder form of PKU (mPKU) and 6.8% of patients were found with a mild hyperphenylalaninemia (HPA). In the case of 23 patients, an optimal metabolic control was not obtained. The specific phenotype (blonde hair, light skin, blue eyes) was found in 22.7% of cases, 77.3% not having these features. At 68.2% of cases intellectual disability was found, with different levels of severity: 5 patients (11.5%) had liminal intellect, 9 patients (20.5%) had mild mental retardation, 6 patients (13.6%) had moderate mental retardation, 9 cases (20.5%) were with severe mental retardation and 1 patient (2.3%) had profound mental retardation; 31.8% of cases had normal intellect. The prevalence in Bihor county is 1:7,843 newborns. Discussions. A partial or, in rare cases, total lack of dietetic treatment was observed in all patients over 20 years old (current age). The delay in treatment initiation or an insufficient treatment, with a suboptimal metabolic control, will affect patient’s intellect, regardless of metabolic phenotype. If in 20 years old patients, or older,
介绍苯丙酮尿症(PKU)是一种常染色体显性遗传疾病,是氨基酸代谢中最常见的先天性错误。苯丙氨酸羟化酶(PAH)基因的变异导致酶活性降低,导致高苯丙氨酸血症。PKU在欧洲的平均发病率为1:10000新生儿,不同种族和地理区域的发病率差异很大。巨大的遗传变异性(已知1200多种遗传变异)以及其他因素决定了广泛的代谢表型。如果不治疗,PKU会导致不可逆转的智力残疾、身材矮小、色素沉着不足、运动缺陷、癫痫发作,但早期诊断和治疗可以使身体和精神发育几乎正常。目标本研究的目的是确定非遗传因素对罗马尼亚西北部地区PKU患者临床表型的影响。材料和方法。该研究小组由罗马尼亚奥拉迪亚县急诊临床县医院比霍尔地区医学遗传学中心的数据库中发现的1981年至2021年期间诊断为苯丙酮尿症的44名患者组成。收集的数据涉及患者的年龄、性别和住所、诊断年龄和治疗开始时间、多年来的代谢控制、患者的代谢表型及其对临床表型的影响(IQ、是否存在智力残疾或是否存在特定临床表型)。后果大多数患者(66%)在生命的前4个月被诊断为苯丙酮尿症,尽管也有诊断较晚的病例,但20.5%的患者在1岁后被诊断。根据治疗前血浆苯丙氨酸水平,大多数病例(72.7%)具有严重的代谢表型(典型的PKU-cPKU),20.5%的病例具有较轻形式的PKU(mPKU),6.8%的患者患有轻度高苯丙氨酸血症(HPA)。在23名患者的情况下,没有获得最佳的代谢控制。在22.7%的病例中发现了特定的表型(金发、浅色皮肤、蓝色眼睛),77.3%的病例没有这些特征。68.2%的病例发现智力残疾,其严重程度不同:5例(11.5%)为智力极限,9例(20.5%)为轻度智力迟钝,6例(13.6%)为中度智力迟钝,9例为重度智力迟钝,1例(2.3%)为深度智力迟钝;智力正常者占31.8%。比霍尔县的新生儿患病率为1:7843。讨论。在所有20岁以上(当前年龄)的患者中观察到部分或在极少数情况下完全缺乏饮食治疗。无论代谢表型如何,治疗开始延迟或治疗不足,代谢控制不理想,都会影响患者的智力。如果在20岁或以上的患者中,精神发育迟缓的主要原因是在生命的最初几年缺乏饮食治疗,那么对于年轻患者来说,精神发育迟滞的原因通常是缺乏对治疗的依从性。大多数代谢表型是cPKU,与文献数据一致;轻度表型(HPA)在一小部分患者中观察到,比文献中报道的数据要小。在研究的前二十年中,很少观察到温和的表型。在没有筛查测试或提示性临床表现的情况下,可以假设HPA患者仍未确诊,这可以解释研究组中HPA比例较小的原因。与老年患者相比,年轻患者的代谢控制有了显著改善,这意味着一方面可以更好地获得特定的营养,另一方面,患者及其家人对疾病有了更好的了解。此外,这项研究证实了一个已知的事实,即北大的饮食在疾病进化中具有重要意义。在这项研究中,包括具有严重代谢表型且代谢控制良好的患者,这些患者成年后没有智力缺陷,受过高等教育,社会融合,也包括具有轻度代谢表型但代谢控制不良并发展为智力缺陷的患者。结论。比霍尔县的PKU患病率高于全国估计值。无论代谢表型如何,诊断和治疗较晚或代谢控制不佳都会导致智力残疾。PKU筛查和更好的治疗机会使年轻一代的患者比前二十年的患者享有更高的生活质量。
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引用次数: 1
Placental changes in a group of full-term newborns with hypoxic-ischemic encephalopathy 一组足月新生儿缺氧缺血性脑病的胎盘变化
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.1
Andreea Calomfirescu-Avramescu, L. Ceauselu, Vlad Dima, Mihaela Demetrian, A. Bălănescu, P. Bălănescu, I. Gherghina
Objectives. The aim of this study was to present and describe specific macroscopic and microscopic placental findings in patients with hypoxic-ischemic encephalopathy (HIE). Materials and methods. We conducted an observational prospective study which included 84 patients diagnosed with HIE and had available placental histopathological examination. HIE diagnosis was performed according to Sarnat Score. Both macroscopic and microscopic placental lesions were identified. Patients were divided into 2 groups according to the presence of placental lesions. Outcomes. Placental lesions were associated with HIE. Patients with both HIE and placental lesions had lower pH value (p <0.001) and lower Apgar scores (p=0.001). Conclusion. Antenatal placental lesions are present in full-term infants with encephalopathy and seem to define exactly one of the causes of this syndrome.
目标。本研究的目的是介绍和描述缺氧缺血性脑病(HIE)患者胎盘的具体宏观和微观表现。材料和方法。我们进行了一项观察性前瞻性研究,包括84名被诊断为HIE的患者,并进行了胎盘组织病理学检查。根据Sarnat评分进行HIE诊断。肉眼和显微镜下均发现胎盘病变。根据胎盘病变的存在,将患者分为2组。结果。胎盘病变与HIE相关。HIE和胎盘病变患者的pH值较低(p<0.001),Apgar评分较低(p=0.001)。产前胎盘病变存在于患有脑病的足月婴儿中,似乎正是这种综合征的原因之一。
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引用次数: 0
Interrupted aortic arch – case report 主动脉弓中断1例报告
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.5
Heidrun Adumitrăchioaiei, A. Luca
Interrupted aortic arch is a rare ductal-dependent congenital heart malformation, accounting for 1.5% of all MCC, 2 in 100,000 cases of live newborns are diagnosed with IAA. We present the case of a 3-month-old infant who came to our clinic on the recommendation of the family doctor. On clinical examination, he presented an affected general state, peri oronasal cyanosis during moments of agitation, grade III/6 systolic murmur present throughout the precordial area, heart rate = 160 beats/min, respiratory rate = 45 breaths/min, SaO2 = 86 – 91% in atmospheric air, liver 4 cm below the costal rim. Echocardiography revealed a complete ductal-dependent congenital heart malformation, interrupted aortic arch, persistence of ductus arteriosus, wide muscular ventricular septal defect, hypoplasia of the ascending aorta. The angioCT examination confirmed the diagnoses from the echocardiography. During the genetic examination, the suspicion of DiGeorge syndrome was raised, which is why MLPA for microdeletions was required. The first therapeutic act performed was represented by maintaining the patency of the arterial channel. The patient was transferred to a clinic in Italy for the surgical intervention.
主动脉弓中断是一种罕见的导管依赖性先天性心脏畸形,占所有MCC的1.5%,每10万例活产儿中就有2例被诊断为IAA。我们提出的情况下,3个月大的婴儿谁来到我们的诊所在家庭医生的建议。临床检查表现为全身受累,躁动时口鼻周围发绀,心前区出现III/6级收缩期杂音,心率160次/分,呼吸频率45次/分,大气中SaO2 = 86 - 91%,肝脏位于肋缘以下4cm。超声心动图显示完全性导管依赖性先天性心脏畸形,主动脉弓中断,动脉导管持续存在,宽肌性室间隔缺损,升主动脉发育不全。血管oct检查证实了超声心动图的诊断。在基因检查过程中,提出了diggeorge综合征的怀疑,这就是为什么需要对微缺失进行MLPA的原因。第一个治疗行为表现为维持动脉通道通畅。患者被转移到意大利的一家诊所进行手术干预。
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引用次数: 0
Why should the neonatologist give a closer look at the alkaline phosphatase? 为什么新生儿学家应该仔细观察碱性磷酸酶?
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.7
Vlad Dima, R. Bohîlțea, Raluca Mariana Stanescu, Andreea Avramescu-Calomfirescu, V. Varlas, A. Davitoiu
Background. Alkaline phosphatase (ALP) has been intensively studied and proved to be useful in Neonatology. The first condition studied in correlation with increased ALP values was osteopenia of prematurity. In recent years there was an increase interested in correlations between ALP and bilirubin levels. In this article, we want to review the literature to find other benefits of ALP dosing in neonatal patients. Methods. Clinical trials were searched and analyzed by a single individual (via dedicated search engines such as Google Scholar, PubMed, and Scopus). We have included clinical studies from the last 7 years that present statistically significant results regarding the role of ALP dosing in the screening or diagnosis of other neonatal pathologies (jaundice with hyperbilirubinemia, necrotizing enterocolitis, liver dysfunction, renal failure and oncological diseases). Results. After excluding the clinical trials that did not meet the eligibility criteria, 6 clinical trials remained (n = 859). Following the analysis of these studies, correlations were highlighted between high ALP values in the umbilical cord and the need for phototherapy. It was also shown that all newborns with elevated ALP values required treatment for hyperbilirubinemia, as well as being a good predictor of severe jaundice and helping to predict its onset. Also, evidence about the correlation between high values of ALP and prematurity osteopenia was reaffirmed in recent studies. Conclusions. ALP is an easily dosed biomarker that can be successfully used in Neonatology. The existing studies to date encourage the use of ALP as a biomarker especially for the detection of osteopenia of prematurity and for the early detection of newborns who will develop intense forms of jaundice. There are still differences regarding the serum ALP values considered as cut-offs for the diagnosis of various neonatal conditions, but performing multicenter studies on a large number of cases could establish a valid consensus in order to use the same ALP cut-off values in all Neonatology departments.
背景。碱性磷酸酶(ALP)已被广泛研究并被证明在新生儿学中是有用的。与ALP值升高相关的第一个研究条件是早产儿骨质减少。近年来,人们对ALP和胆红素水平之间的相关性越来越感兴趣。在这篇文章中,我们想回顾文献,以发现ALP给药在新生儿患者中的其他好处。方法。临床试验由单个人(通过谷歌Scholar、PubMed和Scopus等专用搜索引擎)进行搜索和分析。我们纳入了过去7年的临床研究,这些研究在ALP剂量在筛查或诊断其他新生儿病理(黄疸伴高胆红素血症、坏死性小肠结肠炎、肝功能障碍、肾功能衰竭和肿瘤疾病)中的作用方面显示了具有统计学意义的结果。结果。在排除不符合入选标准的临床试验后,还剩下6项临床试验(n = 859)。在对这些研究进行分析后,强调了脐带高ALP值与光疗需求之间的相关性。研究还表明,所有ALP值升高的新生儿都需要治疗高胆红素血症,同时也是严重黄疸的良好预测指标,有助于预测其发病。此外,最近的研究也证实了高ALP值与早产儿骨质减少之间的相关性。结论。ALP是一种易于计量的生物标志物,可以成功地用于新生儿。到目前为止,现有的研究鼓励使用ALP作为一种生物标志物,特别是用于检测早产儿骨质减少和早期检测将发展为强烈黄疸的新生儿。血清ALP值作为诊断各种新生儿疾病的截断值仍然存在差异,但对大量病例进行多中心研究可以建立有效的共识,以便在所有新生儿科使用相同的ALP截断值。
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引用次数: 0
Prognostic value of hematological parameters in childhood respiratory tract infections 血液学参数对儿童呼吸道感染的预后价值
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.3
Katharina Schäfer, M. Săsăran
Introduction. Respiratory tract infections (RTIs) represent a growing burden among pediatric populations, as their potential progression towards acute respiratory insufficiency (ARI), which requires hospitalization and can be light-threatening without adequate supportive measures. Several recent studies have tried to investigate the role of non-invasive depictable parameters, such as those easily determined through a complete blood count (CBC), in the prediction of potentially complicated RTIs. The aim of the current study is to assess whether changes in hematological parameters can predict the development of respiratory insufficiency in children with RTIs. Material and methods. A retrospective study was conducted on 80 patients hospitalized for RTIs, divided into two groups based on their complication by ARI: children with RTI and ARI (group 1-30 patients) and children with RTI, uncomplicated with ARI (group 2-50 patients). Patients with a previously known chronic or hematological disorder were excluded from the study. A CBC was carried out in each patient and C-reactive protein (CRP) values were also determined. Moreover, RT-PCR testing for SARS-COV2 infection was carried out in each child. Results. There were no significant differences in most of the hematological parameters analyzed between the two groups, with the exception of mean corpuscular hemoglobin concentration (MCHC), which exhibited lower values in study group 1. Insignificant differences in parameters of CBC were also found when performing a separate analysis on the subgroup of patients infected with SARS-COV2. However, significantly higher CRP values were found in subjects with RTIs complicated by ARI. An important decrease in hemoglobin levels, under 10 mg/dl, was not positively associated with a complicated outcome of RTIs. Conclusion. Hematological parameters, with the exception of MCHC, are not predictive of ARI in children diagnosed with RTIs. A separate analysis on cases diagnosed with SARS-COV2 infection revealed similar, insignificant results. Further studies, conducted on larger populations, could provide more data regarding the role of hematological parameters in the prediction of RTIs’ severity.
介绍。呼吸道感染(RTIs)是儿科人群日益增长的负担,因为它们可能发展为急性呼吸功能不全(ARI),需要住院治疗,如果没有适当的支持措施,可能会对光线造成威胁。最近的几项研究试图调查非侵入性描述参数的作用,例如那些容易通过全血细胞计数(CBC)确定的参数,在预测潜在的复杂RTIs中的作用。本研究的目的是评估血液学参数的变化是否可以预测呼吸道感染儿童呼吸功能不全的发展。材料和方法。回顾性研究80例RTI住院患者,根据其ARI并发症分为两组:RTI合并ARI患儿(1-30例)和RTI未合并ARI患儿(2-50例)。先前已知的慢性或血液系统疾病患者被排除在研究之外。对每位患者进行全血细胞计数,并测定c反应蛋白(CRP)值。此外,对每个儿童进行SARS-COV2感染RT-PCR检测。结果。除研究1组平均红细胞血红蛋白浓度(MCHC)较低外,两组间分析的大多数血液学参数均无显著差异。在对感染SARS-COV2的患者亚组进行单独分析时,也发现CBC参数的差异不显著。然而,在RTIs合并ARI的受试者中,CRP值明显较高。血红蛋白水平显著下降,低于10 mg/dl,与RTIs的复杂结局没有正相关。结论。除MCHC外,血液学参数不能预测诊断为RTIs的儿童是否患有ARI。对诊断为SARS-COV2感染的病例进行的另一项分析显示了类似的、不显著的结果。在更大的人群中进行的进一步研究,可以提供更多关于血液学参数在预测RTIs严重程度中的作用的数据。
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引用次数: 0
Hyperkyphotic posture among adolescents – still a public health problem 青少年过度后凸姿势-仍然是一个公共卫生问题
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.6
Clara Dima, B. Mitoiu, R. Nartea, Vlad Dima, A. Mirea
Posture, hyper-kyphosis or “the postural round back”, and balance are topics that have been extensively studied, although with some conflicting findings. Body position is representative of physical and mental health. Bad posture can lead to spinal complications (affects the muscular system, deforms the skeletal bone, and causes abnormal development) and the same can be said the other way around. As the standard of living increases, there is an increase in consumerism for technological devices, especially among teenagers. Identified as a public health problem, postural changes among children and adolescents have a high prevalence, emphasizing the need for prevention, diagnosis, and early initiation of treatment, especially from the growth and development phase of the body, because during this period teenagers are most vulnerable to live changes. Taking these elements into consideration, we conducted an extensive literature review of the subject, considering adolescence a key element for early diagnosticating and the institution of a complex rehabilitation program, with full functional restoration. Efforts to reduce the prevalence of bad posture will improve not only body posture but will also reduce the prevalence and the severity of spine deformities.
体位、过度后凸或“体位性驼背”和平衡是已经被广泛研究的主题,尽管有一些相互矛盾的发现。体位是身体和心理健康的代表。不良姿势会导致脊椎并发症(影响肌肉系统,使骨骼变形,并导致异常发育),反之亦然。随着生活水平的提高,对科技设备的消费也在增加,尤其是在青少年中。儿童和青少年的姿势变化被确定为一个公共卫生问题,其发病率很高,强调需要预防、诊断和早期开始治疗,特别是从身体的生长和发育阶段开始,因为在这一时期,青少年最容易受到生活变化的影响。考虑到这些因素,我们对该主题进行了广泛的文献综述,将青春期视为早期诊断和制定复杂康复计划的关键因素,并进行了全面的功能恢复。努力减少不良姿势的发生率不仅会改善身体姿势,还会降低脊柱畸形的发生率和严重程度。
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引用次数: 0
The importance of SARS-CoV-2 testing in pediatric population 严重急性呼吸系统综合征冠状病毒2型检测在儿科人群中的重要性
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.4
Rodica Demenciuc, L. Meliț, Tudor Fleșeriu, C. Mărginean
Pediatric population associates multiple peculiarities in terms of SARS-CoV-2 regarding the differences of symptoms in comparison to adults, but also the higher incidence of asymptomatic cases. The aim of these case reports is to underline the importance of SARS-CoV-2 testing in pediatric patients. The first case describes the case of a 2-year and nine-month-old child admitted to the Pediatrics Clinic I Targu Mures for involuntary ingestion of toxic substance. The clinical exam pointed out a mild hyperemia of pharynx and small lymph nodes under the mandibula, on the side area of the neck and above the clavicula; and the laboratory tests pointed out leukocytosis, thrombocytosis and one of the liver transaminases above the normal limit. In spite of the presentation concerns, the patient was tested for SARS-CoV-2 infection, with positive result. The evolution was favorable during the admission. The second case describes a 3-year and 8-month-old male patient brought in the emergency department with the suspicion of foreign body aspiration, but the bronchoscopy revealed only purulent secretion in the trachea and bronchia. The RTPCR test for SARS-CoV-2 infection was negative on the admission day, but due to the slowly favorable evolution, the patient was retested on the 5th day of admission, with positive result. The testing of pediatric patients independently of the symptoms and the clinical setting might carry a higher importance in limiting the viral transmission.
与成人相比,儿童在SARS-CoV-2的症状差异方面存在多种特点,但无症状病例的发生率也更高。这些病例报告的目的是强调在儿科患者中进行SARS-CoV-2检测的重要性。第一个病例描述了一名2岁零9个月大的儿童因非自愿摄入有毒物质而被送入Targu Mures儿科诊所。临床检查发现咽部轻度充血,下颌骨下、颈侧及锁骨上方有小淋巴结;实验室检查发现白细胞增多、血小板增多、一种肝转氨酶高于正常限度。尽管存在表现方面的担忧,但该患者接受了SARS-CoV-2感染检测,结果为阳性。在入院期间,进化是有利的。第二例为3岁零8个月男患者,因怀疑异物吸入就诊于急诊科,但支气管镜检查仅发现气管和支气管内脓性分泌物。入院当天SARS-CoV-2感染的RTPCR检测为阴性,但由于病情发展缓慢,入院第5天重新检测,结果为阳性。对儿童患者进行独立于症状和临床环境的检测可能在限制病毒传播方面具有更高的重要性。
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引用次数: 0
Selective head cooling or whole body cooling? That is the question 选择性头部冷却还是全身冷却?这就是问题所在
Q4 Medicine Pub Date : 2022-06-30 DOI: 10.37897/rjp.2022.2.8
Vlad Dima, R. Bohîlțea, Raluca Mariana Stanescu, A. Toma, V. Varlas, A. Davitoiu
Background. Studies to date support the beneficial effect that therapeutic hypothermia (TH) has on neonates with hypoxic-ischemic encephalopathy. TH can be delivered in two ways: selective head cooling (SHC) or therapeutic whole-body cooling (WBC). The purpose of this review is to examine the literature to expose the advantages and disadvantages of the two methods of performing TH in neonates diagnosed with moderate or severe HIE and to investigate whether one method is superior to the other. Methods. We started from the data from the Cochrane review published in 2013 [1] and we want to bring new information from recent clinical studies comparing the two TH methods (SHC vs WBC). Clinical studies were searched and analyzed by a single individual (through dedicated search engines such as Google Scholar, PubMed, and Scopus). We have included clinical studies from the last 7 years comparing the two ways of performing TH (SHC vs. WBC). We selected only the articles that compared the two methods and excluded the articles describing only one method. Results. After excluding the clinical trials that did not meet the eligibility criteria, 5 clinical trials remained (n = 256 neonates). Analysis of these studies supports the idea that both modalities of therapeutic hypothermia cool central nervous system (CNS) structures and have a similar neurodevelopmental prognosis among surviving neonates. Also, there are no significant differences in terms of short-term neurological prognosis, as well as the adverse effects that may occur in neonates with moderate or severe HIE (hypoxic-ischemic encephalopathy) treated with SHC or WBC; although some studies note the presence of more severe, statistically significant lesions among patients treated with SHC compared to those treated with WBC. Conclusions. Prospective, randomized studies on a much larger scale are needed to track the adverse effects that the two HT methods may have on patients, as well as long-term neurological and cognitive prognosis. Until now, there is no clear evidence that one of these two methods is definitely safer or more reliable than the other.
背景迄今为止的研究支持治疗性低温(TH)对新生儿缺氧缺血性脑病的有益作用。TH可以通过两种方式提供:选择性头部冷却(SHC)或治疗性全身冷却(WBC)。本综述的目的是审查文献,揭示两种方法在诊断为中度或重度HIE的新生儿中进行TH的优缺点,并探讨一种方法是否优于另一种方法。方法。我们从2013年发表的Cochrane综述[1]中的数据开始,我们希望从最近比较两种TH方法(SHC与WBC)的临床研究中获得新的信息。临床研究由一个人进行搜索和分析(通过谷歌学者、PubMed和Scopus等专用搜索引擎)。我们纳入了过去7年的临床研究,比较了两种治疗TH的方法(SHC与WBC)。我们只选择了比较这两种方法的文章,而排除了只描述一种方法的论文。后果在排除不符合资格标准的临床试验后,仍有5项临床试验(n=256名新生儿)。对这些研究的分析支持这样一种观点,即两种治疗性低温模式都能冷却中枢神经系统(CNS)结构,并在存活新生儿中具有相似的神经发育预后。此外,在短期神经预后以及SHC或WBC治疗的中度或重度HIE(缺氧缺血性脑病)新生儿可能发生的不良反应方面没有显著差异;尽管一些研究注意到,与WBC治疗的患者相比,SHC治疗患者存在更严重的、具有统计学意义的病变。结论。需要进行更大规模的前瞻性随机研究,以跟踪这两种HT方法可能对患者产生的不良影响,以及长期的神经和认知预后。到目前为止,还没有明确的证据表明这两种方法中的一种肯定比另一种更安全或更可靠。
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引用次数: 0
Endocrinological disorders in children with cutis-laxa syndromes 儿童皮肤松弛综合征的内分泌障碍
Q4 Medicine Pub Date : 2022-03-31 DOI: 10.37897/rjp.2022.1.4
M. Iancu, A. Albu, R. Vlad, D. Albu
Cutis laxa syndromes is a rare, multisystem disorder, which primary involves the skin, caused by various mutations in genes that code structural or functional components of the elastic fiber, resulting in heterogenous manifestations. Diagnosis is primarily based on the physical examination, but supported by molecular tests, and guides treatment and monitoring of the patients. Endocrinological complications are sparsely described, with short stature, osteoporosis and fractures being the most frequent. The precise mechanisms are not elucidated and reports about condition-specific treatments are rare. This review provides an overview of the endocrinological disturbances reported in association with cutis laxa syndromes.
皮肤松弛综合征是一种罕见的多系统疾病,主要涉及皮肤,由编码弹性纤维结构或功能成分的基因的各种突变引起,导致异质性表现。诊断主要基于身体检查,但由分子测试支持,并指导患者的治疗和监测。内分泌并发症的描述很少,其中身材矮小、骨质疏松和骨折最为常见。确切的机制尚未阐明,关于特定条件治疗的报道也很少。本文综述了与皮肤松弛综合征相关的内分泌紊乱。
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引用次数: 0
期刊
Revista Romana de Pediatrie
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