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Simultaneous Presentation of Autoimmune Hepatitis and Wilson's Disease: A Systematic Review Study 自身免疫性肝炎和Wilson病同时发病的系统回顾性研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-10-01 DOI: 10.32598/JPR.9.4.988.1
Peiman Nasri, Seyed Esmaeil Hosseini-Kordkhyli, Azar Jafari-Koulaee, S. Hovsepian, H. Saneian, M. Khademian, F. Famouri
Background: The specialists should identify the features of Wilson disease and autoimmune hepatitis when both affect a patient to adopt appropriate treatment. Objectives: This study was conducted to determine features of the patient, disease, diagnostic studies, and therapeutic measures in cases of simultaneity of Wilson disease and autoimmune hepatitis. Methods: To find evidence related to the study objectives, we searched databases such as Barakat knowledge network system, SID, Magiran, Google Scholar, Web of Science, ProQuest, Springer, ScienceDirect, Medline via PubMed, and Scopus with specified Persian and English keywords, including “Wilson’s Disease”, “Autoimmune”, and “Hepatitis”. The inclusion criteria for the studies were 1) the study was observational and 2) the study was published in Persian or English. The exclusion criteria included low-quality studies based on the score obtained from the checklist. The obtained studies were screened in terms of titles, abstracts, and full text, and finally, the qualified studies entered the review process. The relevant data were extracted according to a designed checklist. Results: Finally, 10 studies were included in the review process. Information about 14 patients was reported. The Mean±SD age of the participants in the studies was 19±11 years. The direction of diagnosis was from autoimmune hepatitis to Wilson disease in 8 cases and from Wilson disease to autoimmune hepatitis in 3 cases. The simultaneity of autoimmune hepatitis and Wilson disease was considered in 3 patients with no primary and secondary diagnosis. Conclusions: The comorbidity of Wilson disease and autoimmune hepatitis is uncommon but is important. In the presence of relevant symptoms in these patients, the comorbidity of these two diseases should be considered. Accordingly, additional assessments such as serum ceruloplasmin, urinary 24-h copper, molecular genetic testing, MRI, serological tests, anti-nuclear antibody, anti-mitochondrial antibody, anti-smooth muscle antibody, complement level, gamma globulin, IgG, albumin, Kayser-Fleischer ring eye examination, and liver biopsy should be considered for correct diagnosis. If appropriate treatment was started for the disease with a diagnosis of Wilson disease or autoimmune hepatitis, but the response to treatment was insufficient, it is better to consider the simultaneous occurrence of two diseases or the initial misdiagnosis.
背景:当Wilson病和自身免疫性肝炎都影响患者时,专家应确定其特征,以采取适当的治疗方法。目的:本研究旨在确定威尔逊病和自身免疫性肝炎并发病例的患者特征、疾病、诊断研究和治疗措施。方法:为了寻找与研究目标相关的证据,我们搜索了Barakat知识网络系统、SID、Magiran、Google Scholar、Web of Science、ProQuest、Springer、ScienceDirect、Medline via PubMed和Scopus等数据库,并使用指定的波斯语和英语关键词,包括“Wilson’s Disease”、“Autoimmune”和“Hepatitics”。这些研究的纳入标准是:1)该研究是观察性的,2)该研究以波斯语或英语发表。排除标准包括基于检查表得分的低质量研究。对获得的研究进行了标题、摘要和全文筛选,最终,合格的研究进入审查程序。相关数据是根据设计的检查表提取的。结果:最后,10项研究被纳入审查过程。报告了14名患者的相关信息。研究参与者的平均±标准差年龄为19±11岁。诊断方向由自身免疫性肝炎转为Wilson病8例,由Wilson病转为自身免疫性肝3例。考虑了3例无一级和二级诊断的患者同时患有自身免疫性肝炎和Wilson病。结论:Wilson病和自身免疫性肝炎的合并症并不常见,但很重要。在这些患者出现相关症状的情况下,应考虑这两种疾病的合并症。因此,应考虑进行额外的评估,如血清铜蓝蛋白、尿液24小时铜、分子遗传学检测、MRI、血清学检测、抗核抗体、抗线粒体抗体、抗平滑肌抗体、补体水平、丙种球蛋白、IgG、白蛋白、Kayser-Flescher环眼检查和肝活检,以进行正确诊断。如果对诊断为Wilson病或自身免疫性肝炎的疾病开始了适当的治疗,但对治疗的反应不足,最好考虑两种疾病同时发生或最初的误诊。
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引用次数: 0
Causes of Precocious Puberty in Children Referred to an Endocrine Clinic in Qazvin City, Iran From 2006 to 2018 2006年至2018年伊朗加兹温市一家内分泌诊所转诊儿童性早熟原因分析
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-10-01 DOI: 10.32598/jpr.9.4.960.1
A. Homaei, Roghayeh Golmohammadi, F. Saffari
Background: Puberty is an essential milestone in a person’s life. Studies show that precocious puberty is more common in girls than in boys. Objectives: The aim of this study was to survey the causes of precocious puberty in children referred to the endocrine clinic in Qazvin city, Iran, from 2006 to 2018. Methods: This case-series study was performed from March 2006 to June 2018 on patients referred to the endocrine clinic of Qazvin University of Medical Sciences, Qazvin City, Iran, for precocious puberty. To diagnose premature puberty and find its causes, we surveyed sex steroids levels, thyroid function, and, if necessary, performed GnRH test. The patients’ height and weight were measured, and their BMIs (Body Mass Index) were calculated. Uterine ultrasound was performed for all girls. Additionally, brain MRI was performed for all boys and girls under age 6 with precocious puberty. X-ray of the left hand was performed to assess skeletal growth acceleration. Based on the type of puberty, the patients were divided into three groups: Central Precocious Puberty (CPP), Peripheral Precocious Puberty (PPP), and normal variant of puberty. The study data were collected from patients› electronic files and analyzed using SPSS 23. Results: Out of 724 cases, 642 (88.70%) were girls. The mean age of all children was 7.07 (95%CI: 6.99-7.15) years. About 70.5% of cases had CPP, 5% PPP, and 24.5% normal variant of puberty. Cases of Idiopathic Precocious Puberty (IPP), PPP, Neurogenic Central Precocious Puberty (NCPP), and premature pubarche were significantly higher in girls than boys (P<0.001). None of the boys had NCPP. Most girls had normal BMI, but boys were more obese. Mean bone age and bone age/age ratio were higher in girls with NCPP (P<0.001). Conclusions: In our study, most girls and boys had idiopathic precocious puberty, and none of the boys had brain lesions. About half of the cases were overweight or obese, indicating the role of obesity in increasing bone age and the onset of puberty.
背景:青春期是人生中一个重要的里程碑。研究表明,性早熟在女孩中比男孩更常见。目的:本研究的目的是调查2006年至2018年在伊朗Qazvin市内分泌诊所就诊的儿童性早熟的原因。方法:本病例系列研究于2006年3月至2018年6月对因性早熟转诊至伊朗加兹温市加兹温医学科学大学内分泌诊所的患者进行。为了诊断早熟并找出其原因,我们调查了性类固醇水平、甲状腺功能,并在必要时进行了GnRH测试。测量患者的身高和体重,并计算他们的BMI(体重指数)。对所有女孩进行了子宫超声检查。此外,对所有6岁以下性早熟的男孩和女孩进行了脑部MRI检查。对左手进行X光检查以评估骨骼生长加速。根据青春期类型,将患者分为三组:中枢性早熟(CPP)、外周性早熟(PPP)和青春期正常变体。研究数据来自患者›的电子文件,并使用SPSS 23进行分析。结果:724例中女生642例(88.70%)。所有儿童的平均年龄为7.07岁(95%可信区间:6.99-7.15)。约70.5%的病例患有CPP,5%患有PPP,24.5%患有青春期正常变异。女孩的特发性早熟(IPP)、PPP、神经源性中枢性早熟(NCPP)和耻骨过早的病例明显高于男孩(P<0.001)。男孩中没有一个患有NCPP。大多数女孩的BMI正常,但男孩更肥胖。患有NCPP的女孩的平均骨龄和骨龄/年龄比较高(P<0.001)。结论:在我们的研究中,大多数女孩和男孩都患有特发性性早熟,没有一个男孩有脑损伤。大约一半的病例超重或肥胖,这表明肥胖在增加骨龄和青春期开始中的作用。
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引用次数: 1
Jigsaw Teaching VS Small Group Teaching: A Comparative Study Among Phase 3 MBBS Students in the Department of Paediatrics 拼图教学与小组教学——儿科MBBS三期学生的比较研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-10-01 DOI: 10.32598/jpr.9.4.974.1
V. Singaravelu, Madhusudhan U
Background: The traditional large group didactic lectures have many shortcomings, so small group discussions have been proposed to overcome some of these shortcomings. However, a typical Small Group Discussion (SGD) remains a mini-interactive lecture in most cases. To improve students’ participation and their better understanding, many newer teaching-learning methods have been tried. Jigsaw teaching method, a type of cooperative learning, is one of these new methods. Obviously, the usefulness of jigsaw teaching must be compared with other small group teaching methods. Objectives: The aim of this study was to compare the effectiveness of the Jigsaw teaching technique with the small group teaching method. Methods: A quasi-experimental study was conducted over one month in the Paediatrics Department of DM WIMS medical college. After obtaining written informed consent, 30 students were randomly selected and allocated to the SGD and jigsaw groups (15 students in each group). Four topics were taken to both the groups who were crossed over after one session (a total of eight exposures). Their post-intervention mean scores were tabulated and analyzed. The Likert scale was used to assess the students’ evaluations of the jigsaw method. Results: The results showed that the jigsaw method had better students’ performance, which was statistically significant with a P<0.05. Also, the students’ evaluation showed that they appreciated the jigsaw teaching method, but time constraints were noted as a drawback. Conclusions: Jigsaw teaching is an excellent small group teaching method to ensure better students’ participation and understanding and can add to our repertoire of teaching-learning methods, which Competency-Based Medical Education (CBME) warrants.
背景:传统的大分组教学法存在许多不足,因此提出了小组讨论法来克服其中的一些不足。然而,在大多数情况下,典型的小组讨论(SGD)仍然是一个小型互动讲座。为了提高学生的参与度和理解力,人们尝试了许多新的教学方法。拼图教学法是一种新型的合作学习方法。显然,拼图教学的有用性必须与其他小组教学方法相比较。目的:比较竖锯教学法和小组教学法的教学效果。方法:在DM WIMS医学院儿科进行为期一个多月的准实验研究。在获得书面知情同意书后,30名学生被随机选择并分配到SGD和拼图组(每组15名学生)。四个主题被带到两组,他们在一次会议后被交叉(总共八次接触)。他们的干预后平均得分被制成表格并进行分析。Likert量表用于评估学生对拼图法的评价。结果:拼图法学生学习成绩较好,差异有统计学意义(P<0.05)。此外,学生们的评价显示,他们很欣赏拼图教学法,但时间限制是一个缺点。结论:拼图教学是一种优秀的小组教学方法,可以确保学生更好地参与和理解,并可以增加我们的教学方法,这是基于能力的医学教育(CBME)所保证的。
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引用次数: 0
Perceived Barriers and Facilitators of Adolescent Friendly Reproductive Health Services in the World: A Qualitative Systematic Review Protocol 感知障碍和促进青少年友好的生殖健康服务在世界:定性系统审查协议
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-10-01 DOI: 10.32598/jpr.9.4.905.2
M. Banaei, Haniyeh Nazem, Tayebeh Darooneh, Farzane Alidost
Background: Adolescents need services that improve their physiological, cognitive, emotional, and social alteration into adulthood. Since the adolescent health needs have been identified, the focus of service delivery has mainly been on access to sexual health care assistance, including HIV issues. Objectives: This systematic review aims to assess the perceived barriers and facilitators of adolescent friendly health services in the world because teens are a positive and energetic force for the present and future of a community. Methods: All qualitative studies from 2000 to 2019 have been considered in this review. The searched databases include Cochrane Central Register, MEDLINE, Google Scholar, Embase, ProQuest, Scopus, Web of Science, CINAHL, and the Ovid platform. The studies included those conducted on all gender and ethnic groups adolescents between 10 to19 years that received health services, as well as their families and health care providers. Selected studies will be critically appraised by two independent reviewers using the Joanna Briggs Institute (JBI) critical appraisal checklist for qualitative research. Qualitative data will be extracted from papers using the standardized data extraction tool from JBI SUMARI (System for the Unified Management, Assessment, and Review of Information) by two independent reviewers. Qualitative research findings will, where possible, be integrated using JBI SUMARI and the meta-aggregation approach, and the final synthesized findings will be graded according to the ConQual approach for establishing confidence in the output of qualitative research synthesis and illustrated in the summary of results. Conclusions: This systematic review addresses perceived barriers and facilitators of adolescent friendly health services in the world. Developing and extending young people’s demands is a concern for all societies. So the results of this study can help us to discover barriers and providers of adolescent friendly health services, and by removing barriers and strengthening facilitators, we will promote the possibility of adolescents accessing adolescent friendly health services.
背景:青少年需要服务,以改善他们的生理,认知,情感和社会转变到成年。由于确定了青少年的保健需要,提供服务的重点主要放在获得性保健援助,包括艾滋病毒问题。目的:本系统综述旨在评估世界上青少年友好型卫生服务的障碍和促进因素,因为青少年是社区现在和未来的积极和充满活力的力量。方法:本综述纳入了2000年至2019年的所有定性研究。检索的数据库包括Cochrane Central Register、MEDLINE、谷歌Scholar、Embase、ProQuest、Scopus、Web of Science、CINAHL和Ovid平台。这些研究包括对接受保健服务的所有性别和族裔群体的10至19岁青少年及其家人和保健提供者进行的研究。选定的研究将由两名独立审稿人使用乔安娜布里格斯研究所(JBI)定性研究的关键评估清单进行严格评估。定性数据将由两名独立的审稿人使用JBI SUMARI(信息统一管理、评估和审查系统)中的标准化数据提取工具从论文中提取。在可能的情况下,定性研究结果将使用JBI SUMARI和meta-aggregation方法进行整合,最终的综合研究结果将根据征服方法进行评分,以建立对定性研究综合产出的信心,并在结果摘要中加以说明。结论:本系统综述探讨了世界上青少年友好型卫生服务的障碍和促进因素。发展和扩大青年人的需求是所有社会都关心的问题。因此,本研究的结果可以帮助我们发现青少年友好卫生服务的障碍和提供者,并通过消除障碍和加强促进者,我们将促进青少年获得青少年友好卫生服务的可能性。
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引用次数: 0
Therapeutic Approaches of Infantile Acne: A Narrative Review Study 婴儿痤疮治疗方法的叙述性回顾研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-10-01 DOI: 10.32598/jpr.9.4.148.22
A. Kazeminejad, L. Davoodi, Z. Hajheydari, M. Ghahari
Background: Acne vulgaris is a skin condition in children and has various presentations and differential diagnoses. Objectives: The purpose of this review was to evaluate the therapeutic approaches of infantile acne. Methods: In this narrative review, we searched articles published in English on infantile acne in Google Scholar, PubMed, and Scopus from 1981 to 2019. Results: A total of 35 articles were selected for review. The treatment of acne often involves various medications that acne lesions. Different factors contribute to the pathogenesis of acne and its severity. The same principle and treatment strategy applies to all age groups diagnosed with acne. Conclusions: The treatment strategy for infantile acne is similar to acne treatment at any age. Treatment is based on the severity of the acne and the risk of a future scar.
背景:寻常痤疮是一种儿童皮肤病,有多种表现和鉴别诊断。目的:本综述的目的是评价婴儿痤疮的治疗方法。方法:在这篇叙述性综述中,我们检索了1981年至2019年在谷歌Scholar、PubMed和Scopus上发表的关于婴儿痤疮的英文文章。结果:共入选35篇文献。痤疮的治疗通常涉及各种药物,痤疮病变。不同的因素导致痤疮的发病和严重程度。同样的原则和治疗策略适用于所有被诊断患有痤疮的年龄组。结论:婴儿痤疮的治疗策略与任何年龄的痤疮治疗相似。治疗是基于痤疮的严重程度和未来疤痕的风险。
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引用次数: 0
Acute Generalized Exanthematous Pustulosis Due to Cephalexin: A Case Report and Literature Review 头孢氨苄引起的急性全身性水肿1例报告及文献复习
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-07-01 DOI: 10.32598/JPR.9.3.657.2
H. Jafarpour, M. Mousavi, Shirin Sheibani, J. Ghaffari, Abbas Dabbaghzadeh, S. Behzadnia
Introduction: Acute Generalized Exanthematous Pustulosis (AGEP) is a cutaneous reaction that may appear after using certain medications, such as cephalexin. This disease is characterized by non-follicular sterile pustules, erythematous, urticaria, fever over , and leukocytosis. Cephalexin belongs to the family of β-lactam antibiotics, which are widely used to treat infections. However, cephalexin skin sensitivities have been reported rarely. Herein, in this case, we aimed to report a patient presented with AGEP due to cephalexin usage. Case Presentation: A 12-year-old boy presented with warm skin lesions that gradually appeared on the limbs, trunk, face, and neck after using cephalexin powder on his left leg injury. Because of his symptoms, acetaminophen, fexofenadine hydrochloride, loxoprofen sodium, and ointment, including difluprednate and hydrocortisone, were prescribed. Over time, the patient’s fever subsided, and 8 days later, the symptoms of AGEP, including urticaria, erythematous, and pustules in the neck and trunk, disappeared. Conclusions: Cephalexin is one of the antibiotics that doctors and patients consider when there is a possibility of infection. AGEP is a rare but severe reaction that can manifest as skin rashes in any age and sex following the use of cephalexin, so the patient should be careful when using this antibiotic.
简介:急性全身性水肿性水肿(AGEP)是一种皮肤反应,可能在使用某些药物后出现,如头孢氨苄。本病以非滤泡性无菌性脓疱、红斑、荨麻疹、高热和白细胞增多为特征。头孢氨苄属于β-内酰胺类抗生素家族,广泛用于治疗感染。然而,关于头孢氨苄皮肤敏感性的报道很少。在这种情况下,我们旨在报告一名因使用头孢氨苄而出现AGEP的患者。病例介绍:一名12岁男孩在左腿受伤后,四肢、躯干、面部和颈部逐渐出现温热性皮肤损伤。由于他的症状,医生给他开了对乙酰氨基酚、盐酸非索非那定、洛索洛芬钠和软膏,包括二氟丙酸盐和氢化可的松。随着时间的推移,患者的发烧消退,8天后,AGEP的症状,包括荨麻疹、红斑和颈部和躯干的脓疱,消失了。结论:头孢氨苄是医生和患者在有感染可能时考虑的抗生素之一。AGEP是一种罕见但严重的反应,在使用头孢氨苄后,任何年龄和性别都可能表现为皮疹,因此患者在使用这种抗生素时都应该小心。
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引用次数: 0
Information Registry System on Congenital Hypothyroidism: A Systematic Review 先天性甲状腺功能减退症信息登记系统的系统评价
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-07-01 DOI: 10.32598/jpr.9.3.939.1
Morteza Ghasempour, H. Ehtesham, Nooshin Rostampou, Shahram Tahmasbian, L. Shahmoradi
Background: Congenital hypothyroidism is the most common congenital disorder of the endocrine system, leading to preventable mental retardation. Objectives: We aimed to evaluate the current status of congenital hypothyroidism information registry systems. Methods: In this systematic review 290 papers identified. A total of 254 articles were screened, of which 17 qualified articles were selected through the databases of Scopus, science direct ProQuest, PubMed, as well as the search engine Google scholar (no restriction on date of publication) up to 2020, were searched. Inclusion criteria were the Articles with the English language that examining information registry systems on congenital hypothyroidism. Articles whose full texts were not accessible, case reports, and letters to the editor had to be excluded. Results: This study showed that the primary goal of nearly half (50%) of the information registry systems for congenital hypothyroidism is to evaluate the efficiency and effectiveness of the screening program. Other information registry systems for congenital hypothyroidism have examined the epidemiology of the disease in 4 studies (23.6%), monitoring iodine deficiency in 3 studies (18%), surveillance disease in 1 study (6%), and describing the clinical and familial features of the patient in 1 study (6%). Conclusions: Few studies have been done on developing information registry systems, and most studies have focused on the use of information systems in this field. Further comprehensive reviews are recommended to investigate the infrastructure of the commission national registry and world network to record information units on congenital hypothyroidism.
背景:先天性甲状腺功能减退症是最常见的先天性内分泌系统疾病,可导致可预防的精神发育迟缓。目的:我们旨在评估先天性甲状腺功能减退症信息登记系统的现状。方法:在本系统综述中,共鉴定出290篇论文。共筛选了254篇文章,其中17篇合格文章是通过Scopus、science direct ProQuest、PubMed以及搜索引擎Google scholar(不限制发表日期)的数据库筛选到的,截至2020年。纳入标准为检查先天性甲状腺功能减退症信息登记系统的英文文章。全文无法查阅的文章、案例报告和给编辑的信必须被排除在外。结果:这项研究表明,近一半(50%)先天性甲状腺功能减退症信息登记系统的主要目标是评估筛查计划的效率和有效性。先天性甲状腺功能减退症的其他信息登记系统在4项研究中检查了该疾病的流行病学(23.6%),在3项研究中监测了碘缺乏症(18%),在1项研究中监控了疾病(6%),并在1项研究中描述了患者的临床和家族特征(6%)。结论:关于开发信息登记系统的研究很少,大多数研究都集中在信息系统在该领域的使用上。建议进行进一步的全面审查,以调查委员会国家登记处和世界网络的基础设施,以记录先天性甲状腺功能减退症的信息单位。
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引用次数: 1
Potts Shunt in Children With Familial Primary Pulmonary Hypertension: A Case Report and Brief Literature Review 家族性原发性肺动脉高压患儿Potts分流一例报告及简要文献回顾
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-07-01 DOI: 10.32598/JPR.9.3.913.1
Mahya Mobinikhaledi, A. Shabestari, Y. Ghandi
Introduction: The primary concern about Familial Pulmonary Arterial Hypertension (FPAH) is the development of right heart failure, which ultimately leads to sudden death. Despite recent advances in pharmacological therapy, the mortality rate in children is still high, emphasizing the importance of novel treatments. Moreover, there is still no definitive treatment for children with severe pulmonary hypertension. The consequences of right heart failure led to the proposal of a surgical approach, the Potts shunt, to decompress the right ventricle, providing shunting of deoxygenated blood to lower extremities and improving overall cardiac output. This surgical technique creates an anastomosis between the left pulmonary artery and the descending aorta, providing a palliative treatment to off-load the right ventricle. Case Presentation: We report an 11-year-old girl with familial pulmonary arterial hypertension and right ventricular failure who benefited from a Potts shunt with good mid-term results. The patient was diagnosed at the age of 5 when she had a history of fainting and persistent syncope. The patient was under medical treatment therapy with bosentan, sildenafil, acetylsalicylic acid, and warfarin for six years. The results of molecular genetic testing, which was confirmed with direct sequencing of the Bone Morphogenetic Protein Receptor Type 2 (BMPR2) gene, revealed a heterozygous pathogenic mutation. Since she was diagnosed with Pulmonary Arterial Hypertension (PAH), she lost her grandmother, an aunt, and father because of PAH. Her 14-year-old sister also had mutated the BMPR2 gene without developing FPAH. Conclusions: The Potts shunt provides an interventional step for palliation of patients with familial pulmonary hypertension and severe right heart failure refractory to medical treatment. It opens the door to the possibility of lung transplantation in the future. We did not see any complications within 6 years after placing the Potts shunt.
家族性肺动脉高压(FPAH)的主要关注点是右心衰的发展,最终导致猝死。尽管最近在药物治疗方面取得了进展,但儿童的死亡率仍然很高,这强调了新型治疗方法的重要性。此外,对于患有严重肺动脉高压的儿童仍然没有明确的治疗方法。右心衰的后果导致了一种手术方法的提出,Potts分流,减压右心室,提供缺氧血液分流到下肢,提高总心输出量。这种手术技术在左肺动脉和降主动脉之间建立了吻合口,为减轻右心室负荷提供了姑息性治疗。病例介绍:我们报告一位患有家族性肺动脉高压和右心衰的11岁女孩,她从Potts分流术中获益,中期结果良好。患者在5岁时被诊断出有昏厥和持续性晕厥的病史。患者接受波生坦、西地那非、乙酰水杨酸、华法林等药物治疗6年。骨形态发生蛋白受体2型(BMPR2)基因的直接测序证实了分子基因检测结果,发现了一个杂合致病突变。由于她被诊断为肺动脉高压(PAH),她失去了祖母、阿姨和父亲。她14岁的妹妹也发生了BMPR2基因突变,但没有发生FPAH。结论:Potts分流术为缓解家族性肺动脉高压合并严重右心衰患者的药物治疗难治性提供了介入手段。它为未来肺移植的可能性打开了大门。我们在放置Potts分流器后的6年内没有发现任何并发症。
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引用次数: 0
Peritrochanteric Unicameral Bone Cyst in A 9-year-old Girl: A Case Report and Review of Literature 9岁女童股骨转子周围一院性骨囊肿1例报告及文献复习
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-07-01 DOI: 10.32598/jpr.9.3.873.1
S. Ghaffari, Mehran Razavipour, Kosar Maleki, Ali Akbar Farsavian
Introduction: The Unicameral Bone Cyst (UBC) is a benign osteolytic lesion primarily found in the metaphyseal part of long bones in children. It is important as it can involve growth plate involvement, cause pathological fractures and deformities of the affected limb. We report this case to emphasize that hip area pathologies can be represented with knee pain and discomfort. Case Presentation: The patient was a 9-year-old girl with a bone cyst in the right proximal femur, with functional knee pain and limping. The diagnosis was made after two years of pain in the knee area. As the cyst was symptomatic and the signs of impending pathological fracture were seen, the lesion was managed by curettage and fibular strut allograft and proximal humerus locking plate. Conclusions: The patient has been examined for knee joint problems for a long time, and her femur bone cyst was diagnosed after two years of pain. However, it could become a pathologic fracture or involve the growth plate and stop the limb’s growth, thus affecting the patient’s quality of life. Therefore, in children with chronic knee pain, careful assessment of the hip area is recommended.
单侧骨囊肿(UBC)是一种主要发生在儿童长骨干骺端的良性溶骨性病变。这很重要,因为它会累及生长板,导致病理性骨折和患肢畸形。我们报告这个病例是为了强调髋部病变可以表现为膝盖疼痛和不适。病例介绍:患者为一名9岁女孩,右股骨近端骨囊肿,伴有膝关节功能性疼痛和跛行。诊断是在膝盖区域疼痛两年之后做出的。由于囊肿有症状且有病理性骨折的迹象,病变采用刮除、异体腓骨支架移植和肱骨近端锁定钢板治疗。结论:患者长期检查膝关节问题,经过两年的疼痛诊断为大腿骨囊肿。然而,它可能成为病理性骨折或累及生长板,停止肢体的生长,从而影响患者的生活质量。因此,对于患有慢性膝关节疼痛的儿童,建议仔细评估髋关节面积。
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引用次数: 0
Evaluation of the Drug Hypersensitivity Reactions Prevalence in Hospitalized Children in Sari City from 2014 to 2018: An Original Research 2014 - 2018年沙丽市住院儿童药物超敏反应发生率评价:原创性研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2021-07-01 DOI: 10.32598/jpr.9.3.901.1
H. Houshmand, M. Shiran, J. Ghaffari, M. Abounoori, M. Maddah, Alireza Razavi, S. Askari, S. Bayat, G. Houshmand
Background: Drug allergy is a subtype of Adverse Drug Reaction (ADR) mediated by the immunologic system. Extreme drug allergy is poorly known in children that contribute to hospitalization in our region. Objectives: This research aimed to study major drug hypersensitivity reactions in the north of Iran among the hospitalized children. Methods: This cross-sectional study was conducted at Bou Ali Sina Hospital in the north of Iran. We analyzed the medical files of 140 children admitted to the hospital with drug allergies from 2014 to 2018. The inclusion criteria were based on the DRESS (drug reaction with eosinophilia and systemic symptoms) scoring system, and those who scored more than four were enrolled. Patients with incomplete medical charts were excluded. The sampling methodology was based on the consecutive census method. Results: In the current study, 62 patients were females (44.29%), and 78 were males (55.71%). Ninety-six cases (68.57%) were children under five, and 44 cases (31.43%) were over five years old. Most ADRs were seen in patients from urban areas (58.57%) than in rural areas (41.43%). In 116 children (82.6%), maculopapular rashes were seen. Phenobarbital and cefalexin had the highest levels of drug reactions, including 85 persons (55.19%) and 18 persons (11.69%), respectively. Pruritus, fever, and scaling were the most common symptoms observed in the patients. The most commonly used treatment was antihistamines. Conclusions: The most causative medication inducing ADR was phenobarbital. The maculopapular rash was the most common skin manifestation, and there were no relationships between sex, age, medication type, and type of hypersensitivity reactions.
背景:药物过敏是由免疫系统介导的药物不良反应(ADR)的一种亚型。在我们地区,极端药物过敏在导致住院的儿童中所知甚少。目的:本研究旨在研究伊朗北部住院儿童的主要药物过敏反应。方法:本横断面研究在伊朗北部的Bou Ali Sina医院进行。我们分析了2014年至2018年住院的140名药物过敏儿童的医疗档案。纳入标准基于DRESS(药物反应伴嗜酸性粒细胞增多和全身性症状)评分系统,评分大于4分者入组。排除病历不完整的患者。抽样方法采用连续普查法。结果:本组患者中,女性62例(44.29%),男性78例(55.71%)。5岁以下儿童96例(68.57%),5岁以上44例(31.43%)。不良反应发生率以城市(58.57%)高于农村(41.43%)。116例(82.6%)患儿出现黄斑丘疹。苯巴比妥和头孢氨苄的药物反应发生率最高,分别为85人(55.19%)和18人(11.69%)。瘙痒、发热和结垢是患者最常见的症状。最常用的治疗方法是抗组胺药。结论:引起不良反应的最主要药物是苯巴比妥。黄斑丘疹是最常见的皮肤表现,与性别、年龄、药物类型和过敏反应类型无关。
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Journal of Pediatrics Review
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