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Absence of lingual frenulum in children with Ehlers-Danlos Syndrome: a retrospective study of forty cases and literature review of a twenty years long debate. 埃勒斯-丹洛斯综合征患儿舌系带缺失:40例病例的回顾性研究和20年争论的文献综述。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-06-01 DOI: 10.23736/S0026-4946.19.05530-0
S. Savasta, Francesco Bassanese, Chiara Hruby, T. Foiadelli, B. Siri, Viviana Gori, M. Votto, C. Tinelli, G. Marseglia
BACKGROUNDEhlers-Danlos syndrome is part of connective tissue disorders and is characterized by skin hyperextensibility, joint hypermobility, easy bruising and other severe manifestations such as epilepsy, pneumothorax, arterial rupture and bowel perforation. In 2017 a new classification was published, indicating major and minor criteria for each form of EDS. Further reports in the past years tried to determine whether or not the absence of lingual frenulum should be included in minor criteria for the diagnosis of EDS, but a consensus has still not been reached. The aim of this study is to assess the clinical relevance of lingual frenulum absence, evaluating its prevalence in a cohort of EDS pediatric patients and comparing it to a group of controls.METHODSPatients with Ehlers-Danlos syndrome were observed at our Department of Pediatrics of Policlinico S.Matteo in Pavia, Italy. Each patient underwent clinical examination of the oral cavity, and controls were chosen among patients referred to our Department.RESULTSThirty-three over 40 patients showed absence of lingual frenulum and 3 of them showed frenulum hypoplasia. Absence or hypoplasia of lingual frenulum showed a prevalence of 90% in our population, whereas only 3/170 controls (1.8%), had lingual frenulum absence. Overall, absence of the lingual frenulum showed a sensibility of 90% and a specificity of 98.2% in our population.CONCLUSIONSIn agreement with other authors, we believe that the absence of lingual frenulum should be included in the minor diagnostic criteria for Ehlers-Danlos Syndrome.
背景hlers-Danlos综合征是结缔组织疾病的一部分,其特征是皮肤过度伸展、关节活动过度、容易瘀伤和其他严重表现,如癫痫、肺气肿、动脉破裂和肠穿孔。2017年发布了一种新的分类法,指出了每种EDS的主要和次要标准。过去几年的进一步报道试图确定是否应将舌系带缺失纳入EDS诊断的次要标准,但仍未达成共识。本研究的目的是评估舌系带缺失的临床相关性,评估其在EDS儿科患者队列中的患病率,并将其与对照组进行比较。方法在意大利帕维亚马泰奥警察诊所儿科对埃勒斯-丹洛斯综合征患者进行观察。每位患者都接受了口腔临床检查,并在转诊至我科的患者中选择对照组。结果40例患者中有3例出现舌系带缺失,其中3例出现系带发育不全。在我们的人群中,舌系带缺失或发育不全的患病率为90%,而只有3/170的对照组(1.8%)出现舌系带缺失。总体而言,在我们的人群中,舌系带缺失显示出90%的敏感性和98.2%的特异性。结论与其他作者一致,我们认为舌系带缺失应纳入埃勒斯-丹洛斯综合征的次要诊断标准。
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引用次数: 5
The effectiveness of indoor children's play center in relieving preoperative anxiety in paediatric surgical patients. 室内儿童游戏中心缓解小儿外科患者术前焦虑的效果。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-04-01 DOI: 10.23736/S0026-4946.19.05658-5
Shaoqiong Sun, Xueqin Liao, Qiujie Wang, Chaofan Xie, Bin Yang, Dan Wu
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引用次数: 0
Management of chronic cervical lymphadenopathy in children 儿童慢性颈部淋巴结病的治疗
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-04-01 DOI: 10.23736/S2724-5276.16.04297-3
Z. Önal, L. Soydan, B. Karadaş, T. Gürbüz, C. Nuhoglu, M. Şimşek
BACKGROUND It is important how to evaluate chronic cervical lymphadenopathies and when to perform excisional biopsy in children. We tried to analyze the usefulness of clinical and ultrasonographic findings in order to differentiate malign lymphadenopathies from benign ones. METHODS This prospective study included 100 children who had cervical lymphadenopathies, larger than 1. 5 cm for 4 weeks duration at least. Children were between 2 and 14 years old, the mean age was 6.88±3.38 years. They were examined by clinical symptoms and ultrasonographic appearances. Persistent lymphadenopathies were evaluated by excisional biopsy. RESULTS Cervical lymhadenopathies of 80 children with well clinical symptoms decreased and resolved within 10 weeks durations. Their ultrasonographic findings revealed regular margins, ovoid shapes and getting smaller than 1. 5 cm. The remaining 20 children persisting longer than 10 weeks at the same size, with worrisome clinical symptoms and susceptible ultrasonographic findings (round shapes, irregular margins) underwent excisional biopsy. According to the biopsy results, five had tubercular lymphadenopathies, three had Hodgkin'slenfoma, two had acute lymphoblastic leukemia. CONCLUSIONS Chronic cervical lymphadenopathies can persist up to 10 weeks, althoughthey are reactive and benign.
背景如何评估慢性颈部淋巴结病以及何时对儿童进行切除活组织检查具有重要意义。我们试图分析临床和超声检查结果的有用性,以区分恶性淋巴结病和良性淋巴结病。方法这项前瞻性研究包括100名患有颈部淋巴结病的儿童。5cm,至少4周。儿童年龄在2至14岁之间,平均年龄为6.88±3.38岁。通过临床症状和声像图检查。通过切除活组织检查评估持续性淋巴结病。结果80例临床症状良好的儿童宫颈淋巴结病在10周内症状减轻并得到缓解。他们的超声检查结果显示边缘规则,卵圆形,并且越来越小。5厘米。其余20名儿童在相同大小下持续超过10周,有令人担忧的临床症状和易感的超声检查结果(圆形,边缘不规则),接受了切除活组织检查。根据活检结果,5例患有结节性淋巴结病,3例患有霍奇金淋巴瘤,2例患有急性淋巴细胞白血病。结论慢性颈部淋巴结病可以持续10周,尽管它们是反应性的和良性的。
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引用次数: 1
Clinical characteristics predicting abnormal brain magnetic resonance image findings in hypoxic-ischemic encephalopathy infants. 预测缺氧缺血性脑病患儿脑磁共振异常表现的临床特征。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-02-01 DOI: 10.23736/S0026-4946.17.04791-0
S. Yum, C. Moon, Y. Youn, I. Sung
BACKGROUNDThe aim of this study was to elucidate the specific clinical characteristics associated with abnormal brain magnetic resonance image(MRI) findings in hypoxic ischemic encephalopathy(HIE) infants in order to discern how to predict poorer outcomes more accurately.METHODSA retrospective data analysis of HIE infants admitted to the neonatal intensive care unit of a tertiary hospital was performed. Baseline perinatal information and physical and neurological findings were compared in HIE infants according to the presence of abnormal brain MRI findings.RESULTSA total of 69 infants were enrolled in the study. Of these, 48(69.6%) infants received therapeutic hypothermia, and 60(87.0%) infants presented abnormal findings on brain MRI. Decreased muscle tone and lower Apgar scores were more often observed in infants with abnormal MRI results(p<0.05). The presence of hypotonia and 1- and 5-minute Apgar scores equal to or less than the yielded cut-off values(5.5 and 7.5, respectively)were associated with a 7.23-, 9.14-, 9.78-fold increased risk of having abnormal brain MRI results.CONCLUSIONSAs muscle tone and Apgar scores were associated with abnormal brain MRI findings in HIE infants, these clinical characteristics may serve as early indicators of poor neurodevelopmental outcomes and signal the need for special attention and in-depth follow-up by MRI.
背景:本研究的目的是阐明与缺氧缺血性脑病(HIE)婴儿异常脑磁共振图像(MRI)结果相关的特定临床特征,以便了解如何更准确地预测不良预后。方法对某三级医院新生儿重症监护病房收治的HIE患儿进行回顾性分析。根据脑MRI异常表现,比较HIE婴儿的围产期基线信息和身体和神经学表现。结果共69名婴儿入组研究。其中,48名(69.6%)婴儿接受了治疗性低温治疗,60名(87.0%)婴儿在脑MRI上表现出异常。在MRI结果异常的婴儿中,肌张力降低和Apgar评分较低更为常见(p<0.05)。低张力和1分钟和5分钟Apgar评分等于或小于产生的临界值(分别为5.5和7.5)的存在与出现异常脑MRI结果的风险增加7.23、9.14和9.78倍相关。结论肌张力和Apgar评分与HIE患儿的异常脑MRI表现相关,这些临床特征可作为不良神经发育结局的早期指标,提示需要特别关注并进行MRI深入随访。
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引用次数: 0
Childhood brain cancer risk from early life exposure to hyperinsulinemia in offspring of diabetic mothers: the exception that proves the rule? 糖尿病母亲的后代早期暴露于高胰岛素血症的儿童脑癌风险:证明这一规律的例外?
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-02-01 DOI: 10.23736/S0026-4946.19.05582-8
R. Mormile
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引用次数: 0
Vitamin B12 deficiency among asymptomatic healthy infants: its impact on the immune system. 无症状健康婴儿维生素B12缺乏症:对免疫系统的影响。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-02-01 DOI: 10.23736/S2724-5276.16.04274-X
P. Boran, Selin Yıldırım, E. Karakoc‐Aydiner, I. Ogulur, A. Ozen, G. Haklar, A. Koc, T. Akkoç, I. Barlan
AIMThe immunomodulatory effects of vitamin B12 deficiency in children have not yet been established in the literature. In the current study, the effects of vitamin B12 on the immune system in asymptomatic and otherwise healthy infants have been studied.METHODSThe study was conducted at Marmara University, "well-child" outpatient clinic. Vitamin B12 level was measured in a cohort of 611 healthy term infants, followed regularly for at least 6 months. Immunoglobulin measurements, lymphocyte subset analysis, cytokine production analysis, lymphocyte proliferation assays and evaluation of lymphocyte apoptosis were performed in a subset of 60 infants.RESULTSIn this cohort, one out of three babies displayed vitamin B12 deficiency. The percentage of CD4+CD25+ regulatory T cells (Tregs) was lower in vitamin B12 deficient babies than in controls. Although the percentage of Tregs increased after treatment, the change was not significant. There was no difference of cytokine levels between vitamin B12 deficient and control groups. However, proinflammatory cytokines were reduced after treatment. No significant difference was observed for immunoglobulins, early apoptosis and lymphocyte proliferation.CONCLUSIONVitamin B12 deficiency is an underestimated health problem among the developing countries. The clinical consequences of the decreased percentage of Tregs associated with vitamin B12 deficiency, and reduction of proinflammatory cytokines after vitamin supplementation needs to be further studied, especially in terms of emerging allergies, autoimmune disorders and anti-inflammatory effects.
目的:维生素B12缺乏对儿童的免疫调节作用尚未在文献中得到证实。在目前的研究中,研究了维生素B12对无症状和其他健康婴儿免疫系统的影响。方法本研究在马尔马拉大学“well-child”门诊进行。对611名健康足月婴儿的维生素B12水平进行了测量,并定期随访至少6个月。免疫球蛋白测定、淋巴细胞亚群分析、细胞因子产生分析、淋巴细胞增殖试验和淋巴细胞凋亡评估在60名婴儿中进行。结果:在这个队列中,三分之一的婴儿表现出维生素B12缺乏症。缺乏维生素B12的婴儿CD4+CD25+调节性T细胞(Tregs)的百分比低于对照组。治疗后Tregs百分比虽有所增加,但变化不显著。维生素B12缺乏组和对照组之间的细胞因子水平没有差异。然而,治疗后促炎细胞因子减少。免疫球蛋白、早期凋亡和淋巴细胞增殖无显著差异。结论维生素B12缺乏症是发展中国家被低估的健康问题。维生素B12缺乏导致Tregs百分比下降的临床后果,以及补充维生素后促炎细胞因子的减少,需要进一步研究,特别是在新出现的过敏、自身免疫性疾病和抗炎作用方面。
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引用次数: 9
Beneficial therapeutic effects of vitamin C on recurrent respiratory tract infections in children: preliminary data. 维生素C对儿童复发性呼吸道感染的有益治疗效果:初步数据。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-02-01 DOI: 10.23736/S2724-5276.16.04621-1
P. Ferrara, F. Ianniello, V. Bianchi, F. Quintarelli, Michela Cammerata, Enrica Quattrocchi, G. Terranova, G. Miggiano, M. Casale
BACKGROUNDTo demonstrate whether supplementation of vitamin C has a beneficial effect in the prevention of recurrent respiratory tract infections (RTIs) in children. Moreover, we evaluate the main risk factors that predispose to the development of this disease.METHODSSixty children have been enrolled in the study and randomized into two groups: the control group (G1 n = 33) and the group at risk of recurrent RTIs (G2 n = 27). To G2 group was administered every day 100% orange juice with the content of vitamin C 70 mg.RESULTSSignificant reduction in the incidence rate of RTIs (episodes pre-treatment: 182-6.75 episodes/child, after-treatment: 71-2.62 episodes/child, P <0.05), were observed in G2 group.CONCLUSIONSThe administration of vitamin C had a beneficial effect in our group of children with recurrent RTIs, reducing the number of infective episodes.
背景:为了证明补充维生素C是否对预防儿童复发性呼吸道感染(RTIs)有有益作用。此外,我们评估了主要的危险因素,易导致这种疾病的发展。方法60名儿童被纳入研究,随机分为两组:对照组(G1 n = 33)和RTIs复发危险组(G2 n = 27)。G2组每天给予含维生素C 70 mg的100%橙汁。结果G2组RTIs发生率显著降低(治疗前182 ~ 6.75次/例,治疗后71 ~ 2.62次/例,P <0.05)。结论在本组复发性呼吸道感染患儿中给予维生素C治疗有良好效果,可减少感染发作次数。
{"title":"Beneficial therapeutic effects of vitamin C on recurrent respiratory tract infections in children: preliminary data.","authors":"P. Ferrara, F. Ianniello, V. Bianchi, F. Quintarelli, Michela Cammerata, Enrica Quattrocchi, G. Terranova, G. Miggiano, M. Casale","doi":"10.23736/S2724-5276.16.04621-1","DOIUrl":"https://doi.org/10.23736/S2724-5276.16.04621-1","url":null,"abstract":"BACKGROUND\u0000To demonstrate whether supplementation of vitamin C has a beneficial effect in the prevention of recurrent respiratory tract infections (RTIs) in children. Moreover, we evaluate the main risk factors that predispose to the development of this disease.\u0000\u0000\u0000METHODS\u0000Sixty children have been enrolled in the study and randomized into two groups: the control group (G1 n = 33) and the group at risk of recurrent RTIs (G2 n = 27). To G2 group was administered every day 100% orange juice with the content of vitamin C 70 mg.\u0000\u0000\u0000RESULTS\u0000Significant reduction in the incidence rate of RTIs (episodes pre-treatment: 182-6.75 episodes/child, after-treatment: 71-2.62 episodes/child, P <0.05), were observed in G2 group.\u0000\u0000\u0000CONCLUSIONS\u0000The administration of vitamin C had a beneficial effect in our group of children with recurrent RTIs, reducing the number of infective episodes.","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2021-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47195994","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 5
Longitudinal effects of biopsychosocial variables on physical activity after menarche. 生物心理社会变量对月经初潮后身体活动的纵向影响。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-02-01 DOI: 10.23736/S0026-4946.18.05062-4
M. L. Santos, T. B. Simões, L. A. Monteiro, J. Novaes, H. M. Fernandes
BACKGROUNDThe aim of the current study is to analyze the longitudinal effects of certain biopsychosocial variables (socioeconomic status, body mass index (BMI), body image and well-being (QL)), measured at three different menarche stages (S1: before menarche, S2: soon after menarche, and S3: one year after S2), on physical activity and inactivity levels in early adolescence.METHODSThe sample comprised 136 Brazilian teenagers (10-13 years old), showing BMI 18.98 kg/m2 at the initial assessment, who voluntarily participated in the study. Data concerning the following variables were collected in 2010 (S1), 2011 (S2) and 2012 (S3): family income (FI) (socioeconomic anamnesis), BMI (body mass and height), well-being (Autoquestionnaire Qualité de Vie Enfant Imagé) and body image (Body Shape Questionnaire). The physical activity and inactivity levels were measured in S3, only, through the International Physical Activity Questionnaire. The Pearson's correlation and the longitudinal path analysis technique were used to statistically analyze the data.RESULTSThe main results indicated that just family income and BMI, measured soon after menarche (S2), showed significant effect on physical activity and inactivity levels. More specifically, family income has positively influenced (β = 0.25) physical inactivity levels and negatively influenced (β = -0.14) moderate physical activity levels, whereas BMI has positively influenced (β = 0.15) walking levels and negatively influenced (β = -0.13) vigorous physical activity levels.CONCLUSIONSThese results suggest that the body image and well-being perceived by the teenagers at different menarche stages are not determining factors of their physical activity levels in early adolescence. On the other hand, the effects of socioeconomic status and body mass index on early adolescent girls' physical activity and inactivity levels depend on the assessment period.
背景:本研究的目的是分析在三个不同的初潮阶段(S1:初潮前,S2:初潮后不久,S3:初潮后一年)测量的某些生物心理社会变量(社会经济地位,体重指数(BMI),身体形象和幸福感(QL))对青春期早期身体活动和不活动水平的纵向影响。方法136名自愿参加研究的巴西青少年(10-13岁),初始评估时BMI为18.98 kg/m2。2010年(S1)、2011年(S2)和2012年(S3)收集了以下变量的数据:家庭收入(FI)(社会经济记忆)、BMI(体重和身高)、幸福感(Autoquestionnaire qualit de Vie Enfant image1)和身体形象(body Shape Questionnaire)。通过国际身体活动问卷,仅在S3中测量了身体活动和不活动水平。采用Pearson相关和纵向路径分析技术对数据进行统计分析。结果主要结果表明,家庭收入和初潮后测量的身体质量指数(BMI)对身体活动和不活动水平有显著影响。更具体地说,家庭收入对不运动水平有正面影响(β = 0.25),对中等运动水平有负面影响(β = -0.14),而BMI对步行水平有正面影响(β = 0.15),对剧烈运动水平有负面影响(β = -0.13)。结论不同月经初潮阶段青少年的身体形象和幸福感不是青少年早期体育活动水平的决定因素。另一方面,社会经济地位和身体质量指数对青春期早期女孩身体活动和不活动水平的影响取决于评估期。
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引用次数: 0
A rare case of pediatric linear syringocystadenoma papilliferum of the chest. 一例罕见的儿童胸部乳头状线状汗管囊腺瘤。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-01-13 DOI: 10.23736/S0026-4946.20.06083-1
M. Giovannini, T. Oranges, F. Portelli, A. Bassi, F. Pedaci, L. Lodi, C. Azzari, C. Filippeschi
{"title":"A rare case of pediatric linear syringocystadenoma papilliferum of the chest.","authors":"M. Giovannini, T. Oranges, F. Portelli, A. Bassi, F. Pedaci, L. Lodi, C. Azzari, C. Filippeschi","doi":"10.23736/S0026-4946.20.06083-1","DOIUrl":"https://doi.org/10.23736/S0026-4946.20.06083-1","url":null,"abstract":"","PeriodicalId":18533,"journal":{"name":"Minerva pediatrica","volume":null,"pages":null},"PeriodicalIF":2.6,"publicationDate":"2021-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42025914","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Early diagnosis and post-operative follow-up of 50 Chinese children with craniopharyngioma. 50 名中国颅咽管瘤患儿的早期诊断和术后随访。
IF 2.6 4区 医学 Q2 Medicine Pub Date : 2021-01-13 DOI: 10.23736/S0026-4946.20.06177-0
Xue Ma, Fengxue Wang, Shule Zhang, Guimei Li

Background: Craniopharyngioma is a relatively common congenital intracranial tumour for children. But only few available studies focused on the endocrine evaluation before diagnosis and post-operative endocrine evaluations of children with craniopharyngioma.

Aim: This study aims to aid in the early diagnosis of craniopharyngioma (CP) and follow-up post-operative children suffered from craniopharyngioma.

Methods: Craniopharyngioma patients, as the CP group (n = 50), and healthy children, as the control group (n = 30), the symptoms and pituitary hormone levels were reviewed and investigated.

Results: The pre-operative levels of peak of GH, IGF-1, FT4, ACTH, COR and PRL of CP patients were significantly lower than those of the control group (all the P ≤ 0.001). Levels of pituitary-hormones after surgery were significantly lower than both those before surgery and those of the control group (all the P ≤ 0.001). HGH treatment could significantly improve the growth velocity of post-operative children (3.8 ± 1.5 cm/year vs 13.0 ± 3.4 cm/year for males, P ≤ 0.001; 4.0 ± 1.3 cm/year vs 12.7 ± 1.8 cm/year for females, P ≤ 0.001).

Conclusions: Children presenting with endocrine disturbance symptoms combined with pituitary hormone deficits should be assessed by MRI to exclude craniopharyngioma earlier. Also, long-term follow-up study was very essential to craniopharyngioma survivors.

背景:颅咽管瘤是一种比较常见的儿童先天性颅内肿瘤。目的:本研究旨在帮助早期诊断颅咽管瘤(CP),并对颅咽管瘤患儿进行术后随访:方法:以颅咽管瘤患者为CP组(50人),以健康儿童为对照组(30人),回顾并调查其症状和垂体激素水平:结果:CP患者术前GH、IGF-1、FT4、ACTH、COR和PRL的峰值水平明显低于对照组(P均≤0.001)。术后垂体激素水平明显低于术前和对照组(P均≤0.001)。HGH治疗可明显改善术后儿童的生长速度(男性3.8±1.5厘米/年 vs 13.0±3.4厘米/年,P≤0.001;女性4.0±1.3厘米/年 vs 12.7±1.8厘米/年,P≤0.001):结论:儿童出现内分泌紊乱症状并伴有垂体激素缺乏时,应进行磁共振成像评估,以尽早排除颅咽管瘤。此外,长期随访研究对颅咽管瘤幸存者也非常重要。
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引用次数: 0
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Minerva pediatrica
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