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Global Journal of Pediatrics (GJP)最新文献

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Generative Artificial Intelligence in Children and Adolescents: Impact and Future Directions 儿童和青少年的生成人工智能:影响和未来方向
Pub Date : 2024-01-31 DOI: 10.54026/gjp/1013
M. D. Navarro Rubio
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引用次数: 0
Malignant Hyperthermia and Absence of Ryanodine Receptors in a Child Presenting for Ptosis Repair 恶性高热和缺乏赖诺定受体在儿童提出上睑下垂修复
Pub Date : 2022-07-07 DOI: 10.54026/gjp/1012
Pierre Ghostine
Introduction: Malignant hyperthermia (MH) is a hypermetabolic condition caused by a genetic cell membranes channel disruption leading to increased calcium release from the sarcoplasmic reticulum after exposure to triggering agents. Central Core Disease (CCD) is a rare inherited neuromuscular disorder with a wide spectrum of phenotypic presentations. Clinically, there is muscle weakness of variable degree and histopathologically there is evidence of core formation in the muscle fibers. Additional features may occur, including skeletal abnormalities such as foot deformities, scoliosis, or hip displacement. Association of CCD with a mutation of the ryanodine receptor RYR1 and MH is high. If not properly treated, MH could be fatal. Purpose: This study aims towards increasing the awareness of pediatricians, surgeons and anesthesiologists about possible occurrence of MH in patients presenting with only mild “clinical” signs of congenital myopathies such as CCD. Careful diagnostic investigations should be performed prior to surgical interventions and extreme caution during anesthesia. Material and Methods: We report the case of a 3 years old girl, with a history of ptosis, scoliosis, and good muscle tone, who developed unexpected MH during the surgical repair of her ptosis. The critically ill child was transferred to Pediatric Intensive Care Unit (PICU) for management of her MH. The management was done following the guidelines of the European Malignant Hyperthermia Group published in the British Journal of Anesthesia 2010. The patient responded well to treatment. She had a muscle biopsy done after recovery. Discussion: Early management and dantrolene administration are the most important factors to minimize MH morbidity and mortality. MH susceptibility is known in patients carrying mutations in the RYR1 receptors. In our case, RYR1 staining was negative on the muscle biopsy specimen suggesting abnormal or absent RYR1 function, with the presence of occasional cores. These findings, in addition to the ophthalmological and orthopedic history were diagnostic of CCD. In Vitro Contracture Testing (IVCT) was not done because it was not available. Genetic testing was refused by the parents due to financial reasons. Conclusion: In patients presenting with symptoms suggestive of muscle weakness, such as ptosis and scoliosis, and variable muscle tone; congenital myopathies should be considered. CCD and RYR1 receptors deficiency or mutation, if present, may lead to a possible risk of fatal MH, which can be prevented.
恶性热疗(MH)是一种高代谢疾病,是由于暴露于触发剂后,遗传细胞膜通道破坏导致肌浆网钙释放增加而引起的。中央核心疾病(CCD)是一种罕见的遗传性神经肌肉疾病,具有广泛的表型表现。临床表现为不同程度的肌无力,组织病理学上可见肌纤维核心形成的证据。可能会出现其他特征,包括骨骼异常,如足部畸形、脊柱侧凸或髋关节移位。CCD与ryanodine受体RYR1突变和MH的相关性很高。如果治疗不当,MH可能是致命的。目的:本研究旨在提高儿科医生、外科医生和麻醉师对只有轻微“临床”症状的先天性肌病(如CCD)患者可能发生MH的认识。手术干预前应进行仔细的诊断调查,麻醉期间应极其谨慎。材料和方法:我们报告了一个3岁的女孩,有上睑下垂,脊柱侧凸的病史,肌肉张力良好,在手术修复她的上睑下垂时发生了意想不到的MH。危重患儿被转移到儿科重症监护室(PICU)进行MH管理。管理遵循欧洲恶性高热组在2010年英国麻醉杂志上发表的指导方针。病人对治疗反应良好。她在康复后做了肌肉活检。讨论:早期治疗和丹曲林是减少MH发病率和死亡率的最重要因素。已知携带RYR1受体突变的患者对MH易感性。在我们的病例中,肌肉活检标本的RYR1染色为阴性,表明RYR1功能异常或缺失,偶尔存在核心。这些发现,加上眼科和骨科病史,是CCD的诊断。体外挛缩测试(IVCT)没有做,因为它是不可用的。由于经济原因,父母拒绝了基因检测。结论:出现肌无力症状的患者,如上睑下垂、脊柱侧凸、肌张力变化;应考虑先天性肌病。CCD和RYR1受体缺乏或突变,如果存在,可能导致致命的MH风险,这是可以预防的。
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引用次数: 0
Update of Diagnosis and Treatment in Atypical Kawasaki Disease 不典型川崎病的诊断和治疗进展
Pub Date : 2022-01-10 DOI: 10.54026/gjp/1011
Lei Sun
Kawasaki Disease (KD) is an unknown etiology of systemic small vasculitis as the main lesion of acute febrile extranuclear disease, mainly affecting children under five years old, is also the main disease causing acquired heart disease in children. Its etiology is unknown but it is thought to be related to genes, infection and immunity. In particular, the diagnosis and treatment of atypical Kawasaki disease is a major clinical challenge. This paper reports the progress of the treatment of atypical Kawasaki disease, including the causes, clinical manifestations, diagnosis and treatment of Kawasaki disease, especially the progress of drug therapy in recent years.
川崎病(Kawasaki Disease, KD)是一种病因不明的以全身小血管炎为主要病变的急性发热性核外疾病,主要累及5岁以下儿童,也是引起儿童获得性心脏病的主要疾病。其病因不明,但被认为与基因、感染和免疫有关。特别是,非典型川崎病的诊断和治疗是一项重大的临床挑战。本文报道了近年来不典型川崎病的治疗进展,包括川崎病的病因、临床表现、诊断和治疗,特别是药物治疗的进展。
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引用次数: 0
Research Progress on the Effect of Vitamin D on the Pathogenesis of Kawasaki Disease and the Prediction of Coronary Artery Lesion 维生素D在川崎病发病机制及冠状动脉病变预测中的研究进展
Pub Date : 2021-11-09 DOI: 10.54026/gjp/1010
F. Jiao
Kawasaki Disease (KD), also known as mucocutaneous lymph node syndrome, is a kind of fever rash pediatric disease with systemic vasculitis as the main lesion. Its pathogenesis is not yet completely clear, the main pathological changes is systemic vasculitis, involving the small and medium-sized blood vessels of the body, especially coronary artery lesions are more obvious, can lead to coronary artery aneurysm, even serious sudden death. Because the disease can appear serious cardiovascular complications, has gradually attracted attention, in recent years, its incidence has gradually increased trend, has become one of the common pediatric acquired heart disease etiology. Vitamin D acts on the VDR in various tissues and cells of the human body. Many studies have shown that vitamin D not only participates in the traditional calcium and phosphorus metabolism, but also participates in immune regulation through a variety of mechanisms.
川崎病(Kawasaki Disease, KD),又称粘膜皮肤淋巴结综合征,是一种以全身性血管炎为主要病变的发热皮疹型儿科疾病。其发病机制尚不完全清楚,主要病理改变为全身性血管炎,累及全身中小血管,尤其是冠状动脉病变更为明显,可导致冠状动脉瘤,严重时甚至猝死。由于本病可出现严重的心血管并发症,已逐渐引起人们的重视,近年来其发病率有逐渐增加的趋势,已成为小儿获得性心脏病的常见病因之一。维生素D作用于人体各种组织和细胞中的VDR。许多研究表明,维生素D不仅参与传统的钙、磷代谢,还通过多种机制参与免疫调节。
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引用次数: 0
Analysis of 3 Cases of Refractory Urinary Tract Infection Caused by Enterococcus Faecium and Literature Review 粪肠球菌致难治性尿路感染3例分析并文献复习
Pub Date : 2021-08-21 DOI: 10.54026/gjp/1009
Jiao Fuyong
Objective: To explore the clinical features, drug resistance, treatment and prognosis of refractory urinary tract infection caused by Enterococcus faecium infection. Methods: A retrospective analysis of 3 cases of Enterococcus faecium infection admitted to the Children’s Hospital of Shaanxi Provincial People’s Hospital from January 2017 to December 2019, And the clinical manifestations of refractory urinary tract infections caused by it, routine laboratory examinations, mid-stage urine culture and drug sensitivity, urinary ultrasound, magnetic resonance (or CT) examination, treatment process and prognosis, and search and review relevant literature. Result: The three children in this group were all women, aged 5 years, 2 months, 9 years and 11 years old. One case had renal abscess, one case had acute pyelonephritis, and one case had bladder-ureteritis. Clinical features: 2 cases had fever with a temperature of 38-39.5°C, and children with renal abscess were accompanied by chills; 2 cases had frequent urination, dysuria, and urethral irritation; 1 case of 5-year-old child had only transient urinary retention, All 3 cases were treated with conventional antibiotics orally and intravenously outside the hospital. During the course of 2 cases, the leukocytes were more than 25×109/L, 3 cases had neutrophils above 70%, CRP was high, and 3 cases of mid-stage urine culture were Enterococcus faecium;2 cases were sensitive to vancomycin and linezolid, Others are resistant.3 cases had negative blood cultures, 1 case of urinary B-mode ultrasound had a thicker bladder wall, and a slightly thicker wall in the lower right ureter. Considering the inflammatory changes, 1 case had left hydronephrosis and 1 case had left kidney urinary salt crystals. MRI plain scan of both kidneys and ureters + MRU showed: 1 case had a thicker bladder wall, and the wall of the lower ureter was slightly thickened, considering the inflammatory changes. One case had a slight dilation of the upper left ureter and the renal pelvis and calyces. 1 case of CT enhanced scan + CTU showed: 1. Left nodular superior nodules and strip low-density shadow, considering the repeated deformity of the left renal pelvis and ureter with dilation of the ureter (upper renal pelvis is small, hypoplasia); [2]. Abnormal strengthening of the left kidney and a slight thickening of the fascia around the kidney; consider pyelonephritis with abscess formation or cystic lesions. 3. Mild water accumulation in the left kidney and upper middle ureter [4]. There are multiple lymph nodes in the retro peritoneum and the left side of the spine, and some are swollen. Treatment 3 cases were initially ineffective with three generations of cephalosporins, and 2 cases had obvious effect of intravenous infusion of vancomycin based on drug sensitivity. After 7-10 days of treatment, cefepime was changed for consolidation treatment and cured. One case of meropenem treatment improved. Three cases were followed up for 1 year without r
目的:探讨粪肠球菌感染致难治性尿路感染的临床特点、耐药性、治疗及预后。方法:回顾性分析2017年1月至2019年12月在陕西省人民医院儿童医院收治的3例粪肠球菌感染病例,并对其引起的难治性尿路感染的临床表现、实验室常规检查、中期尿培养及药敏、尿超、磁共振(或CT)检查、治疗过程及预后进行分析,并检索和复习相关文献。结果:本组3例患儿均为女性,年龄分别为5岁、2个月、9岁和11岁。肾脓肿1例,急性肾盂肾炎1例,膀胱输尿管炎1例。临床特点:发热2例,体温38 ~ 39.5℃,肾脓肿患儿伴寒战;尿频、排尿困难、尿道刺激2例;1例5岁患儿仅出现短暂性尿潴留,3例患儿均在院外给予常规抗生素口服和静脉注射治疗。2例患者病程中白细胞≥25×109/L, 3例中性粒细胞≥70%,CRP较高,3例中期尿培养为屎肠球菌;2例对万古霉素、利奈唑胺敏感,其余耐药。3例血培养阴性,1例尿b超膀胱壁增厚,右下输尿管壁稍增厚。考虑炎性改变,1例遗留肾积水,1例遗留肾尿盐结晶。双肾、输尿管MRI平扫+ MRU显示:1例膀胱壁增厚,考虑到炎性改变,下输尿管壁稍增厚。1例左上输尿管、肾盂及肾盏轻微扩张。1例CT增强扫描+ CTU显示:左侧结节上结节及条状低密度影,考虑左侧肾盂及输尿管反复畸形伴输尿管扩张(上肾盂小,发育不全);[2]. 左肾异常强化,肾周围筋膜轻度增厚;考虑肾盂肾炎伴脓肿形成或囊性病变。3.左肾及输尿管中上段轻度积水[4]。后腹膜及脊柱左侧有多发淋巴结,部分肿大。3例采用三代头孢菌素治疗初期无效,2例根据药物敏感性静脉输注万古霉素效果明显。治疗7-10 d后改用头孢吡肟巩固治疗,治愈。1例美罗培南治疗效果改善。3例随访1年无复发。1例出院后20天复发,静脉滴注头孢吡肟17天,预防服用呋喃妥因2周后复发。结论:粪肠球菌感染引起的尿路感染多为难治性上尿路感染,并发症多,需及时及中期尿培养,行尿路B超检查。双肾和输尿管的磁共振成像和水文学对并发症的诊断有重要作用。耐药高,根据药敏及时调整治疗,选择有效的药物非常重要,给予充分的疗程,可改善预后。
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引用次数: 0
Renal Lesions of Juvenile Idiopathic Arthritis in Children: A Literature Review 儿童特发性关节炎的肾损害:文献综述
Pub Date : 2021-04-20 DOI: 10.54026/gjp/1008
Borysova T
The literature review presents current data on the frequency, mechanisms of development, clinical manifestations, diagnosis and treatment of renal AA-amyloidosis and describes different forms of glomerulonephritis (GN) in children with juvenile idiopathic arthritis (JIA). The frequency of AA renal amyloidosis in JIA in children ranges from 0.8% to 2%, in adults with JIA duration of 28.3 years - 8.9%. In recent years, against the background of immunobiological therapy, the incidence of AA-amyloidosis of the kidneys in adults has decreased to 2%. AA-amyloidosis of the kidneys most often develops in children with the systemic form, in adults - with systemic and polyarticular forms of JIA. The first symptom of AA-amyloidosis of the kidneys is isolated proteinuria, which transforms into nephrotic syndrome. The peculiarity of the nephrotic syndrome is the absence of hypercholesterolemia in most cases, combined in some patients with arterial hypertension, hematuria, and impaired renal function. The available literature presents clinical cases of renal GN in children with JIA, viz: ANCA-associated GN, mesangioproliferative GN, including IgA- and IgM-nephropathy, membranous nephropathy, focal-segmental glomerulosclerosis, minimal change disease, extracapillary GN. Most publications are devoted to ANCA-associated HN, which developed in patients with a torpid course and a high degree of activity of polyarticular and systemic forms of JIA. The peculiarity of ANCA-associated HN was the presence of hypercreatininemia and, in almost half of cases, the development of terminal renal failure, despite the ongoing immunosuppressive therapy. The main method confirming the diagnosis of AA-amyloidosis and renal HN is the intravital renal morphological study. The use of immunobiological drugs in renal AA-amyloidosis and HN in children with JIA has therapeutic efficacy.
本文综述了肾aa -淀粉样变的发病频率、发生机制、临床表现、诊断和治疗等方面的最新资料,并描述了青少年特发性关节炎(JIA)患儿不同形式的肾小球肾炎(GN)。儿童JIA中AA肾淀粉样变的发生率为0.8% - 2%,成人JIA病程为28.3年- 8.9%。近年来,在免疫生物学治疗的背景下,成人肾脏aa -淀粉样变的发病率已降至2%。肾脏aa -淀粉样变最常发生在全身性JIA的儿童中,发生在全身性和多关节性JIA的成人中。肾脏aa -淀粉样变的第一个症状是孤立性蛋白尿,然后转化为肾病综合征。肾病综合征的特点是在大多数病例中没有高胆固醇血症,在一些患者中合并动脉高血压、血尿和肾功能受损。现有文献报道JIA患儿肾脏GN的临床病例,包括:anca相关性GN、间系血管增生性GN,包括IgA和igm肾病、膜性肾病、局灶节段性肾小球硬化、微小病变、毛细血管外GN。大多数出版物都致力于与anca相关的HN,它发生在病程缓慢且多关节和全身形式的JIA高度活跃的患者中。anca相关HN的特点是存在高肌酐血症,在几乎一半的病例中,尽管正在进行免疫抑制治疗,但仍发展为终末期肾衰竭。确诊aa -淀粉样变合并肾HN的主要方法是活体肾脏形态学检查。应用免疫生物学药物治疗JIA患儿肾aa -淀粉样变性和HN有疗效。
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引用次数: 0
Spinal Anesthesia in Children 小儿脊髓麻醉
Pub Date : 2021-03-22 DOI: 10.54026/gjp/1007
Mikhailichenko Ev
Justificativa e Objetivos Tem aumentado muito o emprego da anestesia subaracnóidea em crianças, principalmente neonatos com risco de desenvolver apnéia neonatal. O objetivo deste trabalho foi rever as diferenças anatômicas, fisiológicas e farmacológicas desta técnica em crianças. Conteúdo A anestesia subaracnóidea em crianças, apesar de ter sido técnica empregada desde o início do século XX, teve sua popularidade diminuída com o advento dos anestésicos inalatórios e bloqueadores neuromusculares, para ser novamente resgatada em 1979. As características favoráveis desta técnica em pediatria são relativas à estabilidade cardiovascular, em crianças de até 8 anos de idade, à analgesia satisfatória e ao relaxamento muscular. Os anestésicos mais utilizados em crianças são a tetracaína e a bupivacaína, cujas doses são ajustadas tomando-se por base o peso corporal. Esta técnica é limitada pela duração relativamente curta, devendo ser utilizada para procedimentos cirúrgicos que não ultrapassem 90 minutos e também pela analgesia não abranger o pós-operatór io . As compl icações são as mesmas encontradas no paciente adulto, incluindo cefaléia por punção dural e irritação radicular transitória. As indicações são várias: cirurgias de abdômen inferior, genitália, membros inferiores, região perineal e, em alguns casos, até em cirurgias torácicas. Seu emprego tem particular interesse nos recém-nascidos prematuros, pelo risco de apresentarem a apnéia da prematuridade. Conclusões A anestesia subaracnóidea em crianças é técnica relativamente segura, com poucas complicações e pode ser considerada como opção para anestesia geral, principalmente nos recém-nascidos prematuros com risco de apresentarem complicações respiratórias no pós-operatório.
理由和目的大大增加了蛛网膜下腔麻醉在儿童中的应用,特别是有新生儿呼吸暂停风险的新生儿。本研究的目的是回顾该技术在儿童中的解剖、生理和药理差异。儿童蛛网膜下腔麻醉虽然自20世纪初以来一直是一种技术,但随着吸入麻醉剂和神经肌肉阻滞剂的出现,它的普及程度有所下降,并于1979年再次得到挽救。该技术在儿科的有利特点是心血管稳定性,在8岁以下的儿童,令人满意的镇痛和肌肉放松。儿童最常用的麻醉剂是丁卡因和布比卡因,它们的剂量是根据体重调整的。这种技术的限制是持续时间相对较短,必须用于不超过90分钟的外科手术,也因为镇痛不包括术后。其并发症与成人患者相同,包括硬膜穿刺性头痛和短暂的根刺激。适应症有很多:下腹、生殖器、下肢、会阴区域的手术,在某些情况下,甚至是胸部手术。它的使用对早产儿特别感兴趣,因为有早产儿呼吸暂停的风险。结论儿童蛛网膜下腔麻醉是一种相对安全的技术,并发症少,可作为全身麻醉的选择,特别是对术后有呼吸并发症风险的早产儿。
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引用次数: 0
A Case Presented with Tracheal Compression Caused by An Anatomical Anomaly: The Innominate Artery Syndrome 解剖异常导致气管受压1例:无名动脉综合征
Pub Date : 2021-03-22 DOI: 10.54026/gjp/1006
Şule Gökçe, MD
Innominate artery compression syndrome is a rare congenital anomaly. The condition is an important consideration in the differential diagnosis of patients presenting with noisy breathing, a barky cough, and expiratory stridor. Here we report a case of Innominate Artery syndrome that presented with persistent and /or biphasic stridor in a 3-month-old. This case provides us to highlights the importance of unequivocally identifying the vascular anomalies and to emphasize once again the importance of detailed history and observing/hearing of the breathing during the examination.
无名动脉压迫综合征是一种罕见的先天性异常。这种情况是鉴别诊断有嘈杂的呼吸,吠叫的咳嗽和呼气喘鸣的病人的重要考虑因素。在这里,我们报告一例无名动脉综合征,表现为持续和/或双相喘鸣在3个月大。本病例使我们强调明确识别血管异常的重要性,并再次强调详细病史和检查时观察/听呼吸的重要性。
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引用次数: 0
Current Status and Research Progress of Chinese Children’s Immunization During The COVID-19 the Pandemic Situation Fuyong Jiao1*, Fen Ma2 , Siqiong Li3 , Feng Yang4 , Wenxing Qiao5 andYang Xue5 新冠肺炎大流行期间中国儿童免疫现状及研究进展焦福勇1*,马芬2,李思琼3,杨峰4,乔文星5,杨雪5
Pub Date : 2021-03-10 DOI: 10.54026/gjp/1005
Fuyong Jiao, C
Vaccination is the most economical and effective means to prevent control and even eliminate infectious diseases, and it is one of the most basic public health service items provided by the government to the masses. Every year, April 25th is the Publicity Day for Children’s Vaccination in China. Due to the new crown pneumonia, the city is closed to fight the epidemic, and the vaccination is suspended. My country’s rapid control of the new crown epidemic has started in an orderly manner with child vaccinations. In the 70 years since the founding of New China, my country’s public health, especially the prevention and control of infectious diseases, has made world-renowned achievements, which are inseparable from vaccination. This article aims to carry out the vaccination work under the prevention and control of the epidemic situation in my country, the types of domestic Class I and II vaccines and the vaccination procedures, as well as the methods for replanting children after they are not vaccinated in time due to the epidemic or other uncontrollable factors. Vaccine research and application development of innovative vaccines are summarized and sorted out.
预防接种是预防控制甚至消除传染病最经济有效的手段,是政府向群众提供的最基本的公共卫生服务项目之一。每年的4月25日是中国儿童预防接种宣传日。因新冠肺炎疫情,我市封闭抗击疫情,暂停疫苗接种。我国迅速控制新冠流行病的工作已开始有序地为儿童接种疫苗。新中国成立70年来,我国公共卫生特别是传染病防控取得了举世瞩目的成就,这与疫苗接种是分不开的。本文旨在开展我国疫情防控下的疫苗接种工作,以及国内一、二类疫苗的种类和接种程序,以及由于疫情或其他不可控因素导致儿童未及时接种疫苗后的补种方法。对疫苗研究和创新疫苗的应用进展进行了总结和整理。
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引用次数: 0
E Learning in Medical Education 医学教育中的电子学习
Pub Date : 2021-03-05 DOI: 10.54026/gjp/1004
Logutina Nataliia Vladimirovna
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引用次数: 0
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