2016年中国大陆期刊发表临床实践指南的报告质量评价

目的使用国际实践指南报告标准(RIGHT)评价2016年中国大陆期刊发表的临床实践指南的报告质量,为中国学者报告指南提供参考。方法计算机检索中国生物医学文献服务系统(CBM)、中国知网(CNKI)和万方数据库(Wan Fang Data),收集期刊公开发表的中国大陆临床实践指南,检索时限为2016年1月1日至2016年12月31日。由2位评价员独立筛选文献,提取资料并交叉核对,用Office Excel 2016软件处理数据。结果共纳入指南79篇。领域1(基本信息)的报告率最高(52.7%),领域5(评审和质量保证)和领域7(其他方面)的报告率最低(6.3%)。79篇指南均报告了条目1a(能否通过题目判断为指南),均未报告条目9a-1(如何遴选参与指南制订的所有贡献者)、条目11b-1(已发表系统评价的检索与评价)、条目11b-2(是否对系统评价进行更新)、条目14a(是否考虑目标人群的价值观和偏好)、条目17(是否经过质量控制程序)和条目18b(推荐意见的传播和实施)。结论 2016年中国大陆期刊指南总体报告质量不佳,建议在以后指南制订过程中参考RIGHT规范对指南进行报告。     

2016年中国大陆期刊发表临床实践指南的报告质量评价

目的：使用国际实践指南报告标准（RIGHT）评价2016年中国大陆期刊发表的临床实践指南的报告质量,为中国学者报告指南提供参考。方法：计算机检索中国生物医学文献服务系统（CBM）、中国知网（CNKI）和万方数据库（Wan Fang Data）,收集期刊公开发表的中国大陆临床实践指南,检索时限为2016年1月1日至2016年12月31日。由2位评价员独立筛选文献,提取资料并交叉核对,用Office Excel 2016软件处理数据。结果：共纳入指南79篇。领域1（基本信息）的报告率最高（52.7%）,领域5（评审和质量保证）和领域7（其他方面）的报告率最低（6.3%）。79篇指南均报告了条目1a（能否通过题目判断为指南）,均未报告条目9a-1（如何遴选参与指南制订的所有贡献者）、条目11b-1（已发表系统评价的检索与评价）、条目11b-2（是否对系统评价进行更新）、条目14a（是否考虑目标人群的价值观和偏好）、条目17（是否经过质量控制程序）和条目18b（推荐意见的传播和实施）。结论：2016年中国大陆期刊指南总体报告质量不佳,建议在以后指南制订过程中参考RIGHT规范对指南进行报告。     

中国科学引文数据库儿科期刊2010至2016年发表的病例报告质量评价

目的：评价中国儿科期刊发表的病例报告的报告质量,并分析其影响因素。方法：手工检索中国科学引文数据库（CSCD）收录的7本儿科期刊在2010年1月至2016年2月发表的病例报告,由2位研究者独立行文献筛选,资料提取,并采用2013年发表的病例报告规范条目表对所纳入文献进行评价,使用Stata12.0软件进行Meta分析。结果共纳入病例报告797篇,病例报告规范评分7～15 （11.1±1.5） 分,评分质量高（14～17分）36篇（6.0%）,评分质量中等（10～13分）638篇（62.7%）,评分质量差（～9分）123篇（31.2%）。报告率低于50%的病例报告规范条目有：题目、背景、时间轴、影响诊断的因素、影响预后的因素、疾病处理过程中值得借鉴的经验和存在的局限性。分层分析显示：病例报告整体报告质量,病例报告规范发表之后好于之前、有基金资助好于无基金资助;作者单位是否为三甲医院对病例报告整体报告质量影响不显著。结论：CSCD收录的7本儿科杂志中发表的病例报告质量有待提高,影响病例报告质量的因素主要有基金资助;合理利用病例报告规范将有助于提升病例报告的报告质量。     

2013临床试验方案规范指南（SPIRIT）及其解读

SPIRIT（Standard Protocol Items: Recommendations for Interventional Trials）声明于2013年在《Annals of Internal Medicine》上发表。该声明以清单条目的形式,并配套同年发表的《SPIRIT 2013解读和说明》,旨在促进高质量临床试验方案的形成。本文对SPIRIT的各项条目进行解读,并提出重点注意事项,为临床试验方案的形成及整个临床试验的顺利进行提供依据。     

《儿科重症监护输血和贫血专家倡议：危重儿童红细胞输注专家共识》介绍

贫血是危重症患儿常见表现, 红细胞（red blood cell, RBC）输注是儿科重症监护病房（pediatric intensive care unit, PICU）常用治疗手段, 在挽救患儿生命的同时, 也带来输血相关性严重危害。为促进危重症患儿合理输注RBC,降低输血相关的严重危害, 儿科重症监护输血和贫血专家倡议（transfusion and anemia expertise initiative, TAXI）专家组制订了危重症儿童RBC输注的专家共识。共识由12篇独立文章组成, 包括共识推荐意见汇总1篇, 涉及一般危重症、 呼吸衰竭、 非失血性休克、 非危及生命的出血或失血性休克、 急性脑损伤、 获得性/先天性心脏病、 血液系统和肿瘤性疾病、 体外膜氧合/心室辅助装置/肾脏替代支持治疗共9类特殊人群的RBC输注的专家共识8篇, RBC的选择和处理、 专家共识制订方法及共识应用的共识各1篇; 包括102项推荐意见, 其中57项为临床推荐意见（20项基于循证医学证据, 37项基于专家共识）, 45项为对相关研究的推荐意见。该文仅介绍临床推荐意见。     

《儿科重症监护输血和贫血专家倡议：危重儿童红细胞输注专家共识》介绍

贫血是危重症患儿常见表现， 红细胞（red blood cell， RBC）输注是儿科重症监护病房（pediatric intensive care unit， PICU）常用治疗手段， 在挽救患儿生命的同时， 也带来输血相关性严重危害。为促进危重症患儿合理输注RBC，降低输血相关的严重危害， 儿科重症监护输血和贫血专家倡议（transfusion and anemia expertise initiative， TAXI）专家组制订了危重症儿童RBC输注的专家共识。共识由12篇独立文章组成， 包括共识推荐意见汇总1篇， 涉及一般危重症、 呼吸衰竭、 非失血性休克、 非危及生命的出血或失血性休克、 急性脑损伤、 获得性/先天性心脏病、 血液系统和肿瘤性疾病、 体外膜氧合/心室辅助装置/肾脏替代支持治疗共9类特殊人群的RBC输注的专家共识8篇， RBC的选择和处理、 专家共识制订方法及共识应用的共识各1篇； 包括102项推荐意见， 其中57项为临床推荐意见（20项基于循证医学证据， 37项基于专家共识）， 45项为对相关研究的推荐意见。该文仅介绍临床推荐意见。     

新生儿白血病的诊疗现状

新生儿白血病属于先天性白血病,为新生儿期的罕见病,国内外报道的病例不多。到目前为止其病因和发病机制仍不清楚,临床表现也与较大儿童有一定的区别,治疗方法以化疗为主,但大多数预后差。近年开展的造血干细胞移植给治愈该病带来了希望。     

新生儿白血病的诊疗现状

新生儿白血病属于先天性白血病，为新生儿期的罕见病，国内外报道的病例不多。到目前为止其病因和发病机制仍不清楚，临床表现也与较大儿童有一定的区别，治疗方法以化疗为主，但大多数预后差。近年开展的造血干细胞移植给治愈该病带来了希望。     

中国儿科领域随机对照试验报告质量横断面调查

摘要目的评估CONSORT声明自1997年引入中国以来,中国儿科杂志发表RCT的报告质量是否得到提高。方法手工检索中国大陆被中国科学引文数据库（CSCD）收录的6种国内儿科杂志（《中华儿科杂志》、《中华小儿外科杂志》、《中国当代儿科杂志》、《中国实用儿科杂志》、《中国循证儿科杂志》和《临床儿科杂志》）,检索时间均为创刊至2010年12月。对照CONSORT声明的评价条目清单,设计标准的资料提取表格,评估纳入文献的报告质量,使用SPSS 18.0软件进行统计分析。结果619篇RCT文献进入分析。1997至2010年与1997年前发表的RCT相比,其在文题、摘要、引言、受试者招募、基线资料和辅助分析等方面的报告质量有一定的改善（P<0.05）,但在方法学的报告方面存在报告不充分或不准确的问题,如确定样本量的方法（0.6% vs 1.1%）、随机序列产生的方法（3.2% vs 7.6%）、分配隐藏（0 vs 1.1%）、盲法（0 vs 2.6%）等。进一步对稿约中要求CONSORT声明的《中国循证儿科杂志》发表的9篇RCT进行评估,其CONSORT条目符合率均高于其他儿科杂志,特别在方法学的报告方面,如确定样本量的方法、随机序列产生的方法、分配隐藏和盲法等。结论总体而言,CONSORT声明的引入并没有使中国儿科领域RCT的报告质量从根本上提高。在稿约中要求CONSORT声明的杂志可显著提高RCT的报告质量。     

中国儿童咳嗽诊断与治疗临床实践指南(2023基层版)

咳嗽是儿童呼吸系统疾病的常见症状和就诊原因。我国基层医疗机构限于诊疗技术和条件, 对儿童咳嗽诊断和治疗等方面的认知普遍不全相同, 且具有一定的特殊性。由此, 中华医学会儿科学分会临床药理学组等联合制订"中国儿童咳嗽诊断与治疗临床实践指南(2023基层版)", 通过前期调研, 充分征询基层医生的意见, 针对儿童咳嗽评估、诊断、用药、转诊和健康教育等方面9个迫切关注的重要问题, 形成17条循证推荐意见和建议, 旨在指导基层医务工作者规范、科学地诊治儿童咳嗽, 同步提升对儿童咳嗽的管理水平。     

Incidents and errors in neonatal intensive care: a review of the literature

OBJECTIVES: To examine the characteristics of incident reporting systems in neonatal intensive care units (NICUs) in relation to type, aetiology, outcome and preventability of incidents. METHODS: Systematic review. Search strategy: Medline, Embase, Cochrane Library. Included: relevant systematic reviews, randomised controlled trials, observational studies and qualitative research. Excluded: non-systematic reviews, expert opinions, case reports and letters. PARTICIPANTS: hospital units supplying neonatal intensive care. INTERVENTION: none. Outcome: characteristics of incident reporting systems; type, aetiology, outcome and preventability of incidents. RESULTS: No relevant systematic reviews or randomised controlled trials were found. Eight prospective and two retrospective studies were included. Overall, medication incidents were most frequently reported. Available data in the NICU showed that the total error rate was much higher in studies using voluntary reporting than in a study using mandatory reporting. Multi-institutional reporting identified rare but important errors. A substantial number of incidents were potentially harmful. When a system approach was used, many contributing factors were identified. Information about the impact of system changes on patient safety was scarce. CONCLUSIONS: Multi-institutional, voluntary, non-punitive, system based incident reporting is likely to generate valuable information on type, aetiology, outcome and preventability of incidents in the NICU. However, the beneficial effects of incident reporting systems and consecutive system changes on patient safety are difficult to assess from the available evidence and therefore remain to be investigated.     

Evaluation of a national resource to identify and study rare diseases: The Australian Paediatric Surveillance Unit

Aims:   To evaluate the Australian Paediatric Surveillance Unit (APSU).
Methods:   We used criteria recommended by the Centres for Disease Control and Prevention (CDC) for evaluating surveillance systems and reviewed productivity, response rates, completeness of the mailing list and impacts of APSU studies. Anonymous evaluation questionnaires were sent to 1260 reporting clinicians, 42 researchers and 86 public health professionals to seek their feedback as users of the APSU.
Results:   APSU provides national epidemiological and clinical data about rare childhood conditions that inform public health policy and clinical practice. Between 2000 and 2007, APSU data were disseminated in 106 journal articles, 207 scientific presentations and 85 media items. Of paediatricians and paediatric sub-specialists actively practicing in Australia and listed as Fellows of the Royal Australasian College of Physicians, 92% participate in APSU surveillance. An average 96% of monthly report cards were returned per annum since 2000. Sensitivity of case ascertainment was difficult to calculate for many conditions because alternative sources of ascertainment do not exist. Evaluation questionnaires were returned by 818 (65%) reporting clinicians, who believe APSU is valuable for generating knowledge 665 (81%), identifying research priorities 635 (78%), guiding clinical practice 572 (70%) and informing public health policy 575 (70%). Similar responses were received from researchers and public health professionals.
Conclusions:   The APSU fulfils its objectives and meets relevant CDC criteria for usefulness, simplicity, acceptability and representativeness, sensitivity and timeliness of data quality. However, stability is threatened by lack of continuing core funding. APSU is highly productive and valued by child health clinicians, researchers and public health professionals.     

Pediatric thymomas: report of two cases and comprehensive review of the literature

Purpose

Thymomas are rare pediatric malignancies with indolent behavior. There are fewer than 50 reported cases and no comprehensive review. We sought to evaluate our recent experience with pediatric thymomas, and comprehensively review the extant literature.

Methods

A systematic search of the PubMed database was performed using keywords: “thymoma”, “pediatric”, “juvenile”, “childhood”, and “child”. Additional studies were identified by a manual search of the reference list.

Results

We report two patients with thymomas. We identified 22 case reports or series that described 48 patients; 62 % were male, 15 % presented with myasthenia gravis. Fifty percent were Masaoka Stage I, 15 % were Stage II, 13 % were Stage III, and 23 % were Stage IV. Four patients with early stage (I or II) disease were treated with adjuvant therapies in addition to surgical excision, while five patients with late stage (III or IV) disease treated with surgical excision alone. Of studies reporting at least 2-year follow-up, survival was 71 %.

Conclusion

Pediatric thymomas are rare tumors with a slight male predominance. Wide variations were observed in the treatment of thymomas across all stages. Our review indicates a need for large database and multi-institutional studies to clearly elucidate clinical course, prognostic factors and outcome.     

Another case of cystic fibrosis complicated by meconium ileus associated with Hirschsprung’s disease: a rare and important association

We are reporting the case of a neonate which died on 16th day of life due to a fatal evolution of a rare association of cystic fibrosis and Hirschsprung's disease.     

Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives

Choroid plexus (CP) carcinoma is a rare pediatric brain neoplasm. Recent studies have highlighted the potential of genome‐wide methylation and gene expression profiling to provide additional layers of information to improve tumor risk‐stratification. There is a lack of data regarding the best therapy, and approaches have been heterogeneous. Despite multidisciplinary treatment approaches, the outcome remains guarded and treatments have been based on case series and expert opinions. In this study, we discuss the recent wealth of data regarding CP carcinoma molecular biology and current management. We also briefly highlight the remaining barriers to formulate the best treatment strategies, and future therapeutic potentials.     

Giant coronary artery aneurysms in juvenile polyarteritis nodosa: a case report

Juvenile polyarteritis nodosa (PAN) is a rare, necrotizing vasculitis, primarily affecting small to medium-sized muscular arteries. Cardiac involvement amongst patients with PAN is uncommon and reports of coronary artery aneurysms in juvenile PAN are exceedingly rare. We describe a 16 year old girl who presented with fever, arthritis and two giant coronary artery aneurysms, initially diagnosed as atypical Kawasaki disease and treated with IVIG and methylprednisolone. Her persistent fevers, arthritis, myalgias were refractory to treatment, and onset of a vasculitic rash suggested an alternative diagnosis. Based on angiographic abnormalities, polymyalgia, hypertension and skin involvement, this patient met criteria for juvenile PAN. She was treated with six months of intravenous cyclophosphamide and high dose corticosteroids for presumed PAN related coronary vasculitis. Maintenance therapy was continued with azathioprine and the patient currently remains without evidence of active vasculitis. She remains on anticoagulation for persistence of the aneurysms. This case illustrates a rare and unusual presentation of giant coronary artery aneurysms in the setting of juvenile PAN.     

Child abuse consultations initiated by child protective services: the role of expert opinions

ObjectiveTo describe consultations provided by child abuse pediatricians for cases referred by child protective services (CPS); compare the opinions of the likelihood of child maltreatment of the initial physician, CPS, and the child abuse pediatrician; and examine predictors of the experts’ opinions.MethodsCases were referred by CPS for consultations between March 1, 1998, and June 30, 2005, to 2 child abuse pediatricians at Yale–New Haven Children’s Hospital. We abstracted demographic and clinical information and the opinions of the initial physician, CPS, and the child abuse expert, each coded using a 5-point scale from definite maltreatment to definite benign cause (eg, accident).ResultsOf 187 cases, 50.3% occurred in children younger than 1 year of age. Children’s most serious injuries were fractures (50.8%), burns (16.6%), and bruises/abrasions (15.0%). The child abuse experts’ opinions were 47.6% definite or probable maltreatment, 8.6% uncertain, and 43.9% definite or probable benign. Of the 119 cases with opinions from all 3 assessors, the expert agreed with the physician in 57.1% of cases (κ = 0.34) and with CPS in 64.7% (κ = 0.42). The best predictor of the expert’s opinion that the injury was due to maltreatment was agreement between the physician and CPS that maltreatment had occurred.ConclusionsLevels of agreement were fair to poor between the child abuse expert and either the physician or CPS. Child abuse experts’ opinions have important value in selected cases to confirm previous assessments by the physician and/or CPS, or to change the opinion of the case.     

Disseminated intravascular coagulation with intracranial haematoma in neonatal congenital syphilis

Disseminated intravascular coagulation (DIC) although a well known complication in neonatal sepsis is extremely rare in congenital syphilis and there are scanty reports of this entity in the literature. Intracranial bleeding following DIC in neonatal congenital syphilis is even rarer, and has been reported only once earlier. We are reporting the second case of neonatal DIC with intracranial haematoma due to congenital syphilis in a newborn. Our patient also had clinical and biochemical evidence of hepatitis which predisposes to DIC. Extensive investigations and emergent use of imaging modalities including ultrasound and CT scan led to early diagnosis and treatment in our patient, who could therefore be salvaged from an otherwise life threatening disease.     

Some new treatment strategies for lung involvement in patients with mucoviscidosis

Autogenic drainage, PEP mask breathing as well as physical exercise are now well established in the treatment of pulmonary disease in cystic fibrosis, but there are different opinions about oxygen therapy over a long period as well as corticosteroid treatment. First reports on amiloride inhalations seem to be hopeful, but there is no experience with cystic fibrosis children till now, therefore clinical use can not be recommended. Heart lung transplants are not to consider as an alternative treatment for all cystic fibrosis patients, in special cases it may be successful.