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JPNIM Vol. 3 N. 2 October 2014 - Contents JPNIM第三卷N. 2014年10月2日-目录
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-11-01 DOI: 10.7363/030278
Jpnim
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引用次数: 0
JPNIM Vol. 3 N. 2 October 2014 - Disclaimer JPNIM第三卷N. 2014年10月2日-免责声明
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-30 DOI: 10.7363/030276
Jpnim
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引用次数: 0
JPNIM Vol. 3 N. 2 October 2014 - Title - Board - Colophon JPNIM Vol. 3 N. 2014年10月2日- Title - Board - Colophon
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-30 DOI: 10.7363/030277
Jpnim
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引用次数: 0
Acute kidney injury in neonatal age 新生儿期急性肾损伤
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-22 DOI: 10.7363/030246
G. Ottonello, A. Dessì, P. Neroni, M. E. Trudu, D. Manus, V. Fanos
Acute kidney injury (AKI) is a pathology characterized by a sudden decrease in kidney function that results in the accumulation of nitrogenous waste products and alteration of the regulation of extracellular fluid volume, electrolytes, and acid-base homeostasis. Previously known as acute renal failure (ARF), in the most recent classifications the term “failure” is used only in conditions requiring renal replacement therapy, peritoneal dialysis or hemodialysis. The diagnosis and therapy of AKI, especially in the neonatal period, still present great difficulties and are the subject of ongoing research in the attempt to improve the prognosis of a pathology still featuring high rates of morbidity and mortality. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
急性肾损伤(AKI)是一种以肾功能突然下降为特征的病理,导致含氮废物的积累和细胞外液容量、电解质和酸碱稳态调节的改变。以前称为急性肾功能衰竭(ARF),在最近的分类中,术语“衰竭”仅用于需要肾脏替代治疗、腹膜透析或血液透析的情况。AKI的诊断和治疗,特别是在新生儿时期,仍然存在很大的困难,并且是正在进行的研究的主题,以试图改善这种仍然具有高发病率和死亡率的病理的预后。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 28
Drug-related perinatal damage from the pharmacological point of view 从药理学角度看药物相关的围产期损害
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-22 DOI: 10.7363/030253
L. Cuzzolin
Drug dosage in the perinatal period represents a continuous challenge for the neonatologist because of interindividual variability of drug metabolism. The human liver plays a central role in the uptake, transport, metabolism and excretion of the vast majority of xenobiotics and drugs. The protein products of human CYP3A account for the largest portion of CYP450 proteins in human liver. At least 50% of currently used drugs in neonatal intensive care units (NICUs) are substrates of CYP3A4 including antibiotics, antivirals, antifungals, immunomodulators, benzodiazepines, proton pump inhibitors, steroid hormones and acetaminophen. The variable CYP3A4 and CYP3A7 expression recently reported in neonatal liver suggests the existence of a marked interindividual variability in drug metabolism during the intrauterine and neonatal lives and, as a consequence, the need of an individualized tailored therapeutic approach in NICUs. The increased risk for adverse effects reported for some drugs in neonates could be related to pharmacokinetic peculiarities of the newborn liver. The fetal and neonatal liver in infants undergoing drug-induced liver injury (DILI) is always characterized by the overlapping between developmental and pathological changes, the differential diagnosis between these changes representing often a challenge for the pathologist. Data here reported clearly evidence the peculiarity of the histological examination of the newborn liver, as compared to the adult liver. In conclusion, the role of the pathologist in the interpretation of liver reactions to drugs may be relevant, only when supported by the dialogue with neonatologists. A deep knowledge of the events taking place during liver development at different gestational ages is necessary for a dedicated neonatal pathologist, in order to avoid misinterpretation of the histological changes related to liver development, giving them a pathological significance. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
由于药物代谢的个体差异,围产期的药物剂量对新生儿科医生来说是一个持续的挑战。人体肝脏在绝大多数外源药物和药物的摄取、运输、代谢和排泄中起着核心作用。人CYP3A蛋白产物在人肝脏CYP450蛋白中占最大比例。目前在新生儿重症监护病房(NICUs)使用的药物中至少有50%是CYP3A4的底物,包括抗生素、抗病毒药物、抗真菌药物、免疫调节剂、苯二氮卓类药物、质子泵抑制剂、类固醇激素和对乙酰氨基酚。最近报道的新生儿肝脏中CYP3A4和CYP3A7的表达变化表明,在子宫内和新生儿生命期间,药物代谢存在显著的个体间差异,因此,需要在新生儿重症监护病房中采用个性化的治疗方法。据报道,一些药物对新生儿产生不良反应的风险增加可能与新生儿肝脏的药代动力学特性有关。药物性肝损伤(DILI)婴儿的胎儿和新生儿肝脏总是以发育和病理变化重叠为特征,这些变化的鉴别诊断对病理学家来说往往是一个挑战。这里报告的数据清楚地证明了新生儿肝脏的组织学检查的特殊性,与成人肝脏相比。总之,病理学家在解释肝脏药物反应中的作用可能是相关的,只有在与新生儿医生对话的支持下。对于专门的新生儿病理学家来说,深入了解不同胎龄肝脏发育过程中发生的事件是必要的,以避免误解与肝脏发育相关的组织学变化,赋予它们病理学意义。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 1
Multiple organ failure in the newborn: the point of view of the pathologist 新生儿多器官衰竭:病理学家的观点
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-21 DOI: 10.7363/030265
C. Gerosa, Eleonora Obinu, D. Fanni, R. Ambu, G. Faa
One of the most severe events occurring in critically ill patients admitted to a neonatal intensive care unit (NICU) center is represented by the multiple organ failure (MOF), a systemic inflammatory response leading to a progressive organ dysfunction and mortality in newborns. MOF may occur in newborns primarily affected by multiple single organ diseases, including respiratory distress syndrome neonatal sepsis with acute kidney injury, post-asphyxial hypoxic-ischemic encephalopathy and pandemic influenza A (H1N1) infection. In a previous article from our group, based on the histological examination of all organs at autopsy of newborns affected by MOF, all organs studied did not escape to be damaged, including thymus and pancreas normally not mentioned in the literature of MOF. The aim of this article is to review the most important pathological changes pathologists should look for in every case of MOF occurring in the perinatal period, with particular attention to systemic endothelial changes occurring in blood vessels in all organs and sytems. On the basis of our experience, matching data during the last phases of the clinicopathological diagnosis represents a useful method, much more productive as compared to the method based on giving pathological answers to the clinical questions prospected before autopsy. As for the pathological features observed in neonatal MOF, one of them deserves a particular attention: the vascular lesions, and in particular the multiple changes occurring during MOF development in endothelial cells, ending with the loss of the endothelial barrier, probably the most relevant histological lesion followed by the insurgence of interstitial edema and disseminated intravascular coagulation. Small vessels should be observed at high power, with particular attention to the size and shape of endothelial nuclei, in order to evidence endothelial swelling, probably the initial modification of the endothelial cells leading to their death. Finally, only the clinical pathological discussion may lead to a good diagnosis, correlating the morphological evidences with the clinical history and the sequence of clinical events that, at the best of our experience, are always different in a new case of MOF. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
新生儿重症监护病房(NICU)中心收治的危重患者中最严重的事件之一是多器官功能衰竭(MOF),这是一种全身炎症反应,导致新生儿器官功能障碍和死亡。MOF可能发生在主要受多个单一器官疾病影响的新生儿中,包括呼吸窘迫综合征新生儿败血症伴急性肾损伤、窒息后缺氧缺血性脑病和甲型H1N1流感感染。在我们小组之前的一篇文章中,根据MOF新生儿尸检时所有器官的组织学检查,所有器官都没有逃脱损伤,包括MOF文献中通常未提及的胸腺和胰腺。本文的目的是回顾在围产期发生MOF的每一个病例中病理学家应该寻找的最重要的病理改变,特别注意发生在所有器官和系统血管中的全身内皮改变。根据我们的经验,在临床病理诊断的最后阶段匹配数据是一种有用的方法,与基于在尸检前对临床问题给出病理答案的方法相比,这种方法更有成效。关于新生儿MOF的病理特征,其中一个值得特别关注:血管病变,特别是MOF发生过程中内皮细胞发生的多种变化,最终以内皮屏障的丧失而结束,这可能是最相关的组织学病变,随后是间质水肿的发作和弥散性血管内凝血。应在高倍镜下观察小血管,特别注意内皮细胞核的大小和形状,以证明内皮细胞肿胀,可能是内皮细胞的初始修饰导致其死亡。最后,只有临床病理讨论才能导致良好的诊断,将形态学证据与临床病史和临床事件的顺序联系起来,在我们最好的经验中,在新的MOF病例中总是不同的。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 0
Bronchopulmonary dysplasia: an old and new disease 支气管肺发育不良:一种新老疾病
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-21 DOI: 10.7363/030268
C. Franco, Flavia Petrillo, A. Vecchio
Bronchopulmonary dysplasia (BPD) is one of the most common and significant medical complications associated with prematurity. It is made more serious by its morbidity and mortality rates. Although recent advances in clinical practice (prenatal steroids, surfactants, new ventilatory strategies, nutritional support) have contributed to improving the clinical course and outcomes of neonates with BPD, its overall incidence has not changed in the last decade owing to a concomitant increase in survival of prematures. The incidence of BPD is in fact inversely proportional to birth weight: 30% for neonates weighing less than 1,000 g, with different percentages in the single centres depending on clinical management and the ventilation criteria applied. However, to date, BPD represents not only a chronic pulmonary pathology in infancy that prevalently affects premature neonates who undergo mechanical ventilation and oxygen therapy for respiratory distress syndrome (RDS), but also prematures with minor signs of initial pulmonary pathology or term neonates requiring aggressive ventilatory support due to an acute and severe lung pathology. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
支气管肺发育不良(BPD)是与早产相关的最常见和最重要的医学并发症之一。它的发病率和死亡率使其更加严重。尽管临床实践的最新进展(产前类固醇、表面活性剂、新的通气策略、营养支持)有助于改善BPD新生儿的临床病程和预后,但由于早产儿存活率的增加,其总体发病率在过去十年中没有改变。BPD的发病率实际上与出生体重成反比:体重小于1,000 g的新生儿为30%,根据临床管理和应用的通气标准,单个中心的百分比不同。然而,迄今为止,BPD不仅是婴儿期的一种慢性肺部病理,普遍影响接受机械通气和氧气治疗呼吸窘迫综合征(RDS)的早产儿,而且也存在轻微的初始肺部病理迹象的早产儿或由于急性和严重肺部病理而需要积极通气支持的足月新生儿。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 0
Cerebral hypoxia and ischemia in preterm infants 早产儿脑缺氧和脑缺血
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-21 DOI: 10.7363/030272
A. Ravarino, M. A. Marcialis, Maria Cristina Pintus, V. Fanos, L. Vinci, M. Piras, G. Faa
Premature birth is a major public health issue internationally affecting 13 million babies worldwide. Hypoxia and ischemia is probably the commonest type of acquired brain damage in preterm infants. The clinical manifestations of hypoxic-ischemic injury in survivors of premature birth include a spectrum of cerebral palsy and intellectual disabilities. Until recently, the extensive brain abnormalities in preterm neonates appeared to be related mostly to destructive processes that lead to substantial deletion of neurons, axons, and glia from necrotic lesions in the developing brain. Advances in neonatal care coincide with a growing body of evidence that the preterm gray and white matter frequently sustain less severe insults, where tissue destruction is the minor component. Periventricular leukomalacia (PVL) is the major form of white matter injury and consists classically of focal necrotic lesions, with subsequent cyst formation, and a less severe but more diffuse injury to cerebral white mater, with prominent astrogliosis and microgliosis but without overt necrosis. With PVL a concomitant injury occurs to subplate neurons, located in the subcortical white matter. Severe hypoxic-ischemic insults that trigger significant white matter necrosis are accompanied by neuronal degeneration in cerebral gray and white matter. This review aims to illustrate signs of cerebral embryology of the second half of fetal life and correlate hypoxic-ischemic brain injury in the premature infant. This should help us better understand the symptoms early and late and facilitate new therapeutic strategies. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
早产是一个重大的国际公共卫生问题,影响到全世界1300万婴儿。缺氧和缺血可能是早产儿获得性脑损伤最常见的类型。早产幸存者缺氧缺血性损伤的临床表现包括脑瘫和智力残疾。直到最近,早产儿广泛的大脑异常似乎主要与破坏性过程有关,这种破坏性过程导致发育中的大脑坏死性病变中的神经元、轴突和胶质细胞大量缺失。新生儿护理的进步与越来越多的证据相吻合,这些证据表明,早产儿的灰质和白质经常遭受不太严重的损害,其中组织破坏是次要的。脑室周围白质软化症(PVL)是白质损伤的主要形式,典型表现为局灶性坏死病变,伴囊肿形成,脑白质损伤较轻但弥漫性较强,伴明显星形胶质和小胶质增生,但无明显坏死。PVL同时损伤位于皮层下白质的板下神经元。严重的缺氧缺血性损伤可引起显著的白质坏死,并伴有脑灰质和白质的神经元变性。这篇综述旨在说明胎儿后半生的脑胚胎学迹象和早产儿缺氧缺血性脑损伤的相关。这将有助于我们更好地了解早期和晚期的症状,并促进新的治疗策略。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 4
Multiple organ failure in the newborn 新生儿多器官衰竭
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-21 DOI: 10.7363/030254
R. Aufieri, S. Picone, P. Paolillo
Multiple organ failure (MOF), or multiple organ dysfunction syndrome (MODS) as more recently known, is a clinical syndrome characterized by the failure of two, or more, organs which are unable to maintain homeostasis without intervention. Described causative factors for MODS in the neonatal period are sepsis, shock due to any cause, tissue hypoperfusion, prematurity, hypoxic ischemic encephalopathy, necrotizing enterocolitis (NEC), surgery, congenital heart disease and others. MOF can be considered as the final common pathway of immunological, cytokine and hormonal changes, occurred as physiologic re- sponse to different infectious or non-infectious inflammatory insults, who lead to systemic inflammation, a procoagulant state and progressive organ dysfunction. The clinical presentation of MODS can widely vary, depending on the primary causes, nature, number and severity of the organ systems involved. Pre-MODS conditions should be promptly identified and treated. In case of severe sepsis and septic shock, the available guidelines should be followed. When MODS already occurred, supportive care for single organ dysfunction should be provided and adequate oxygenation and organ perfusion maintained. More studies in term and preterm infants (with the development of specific neonatal scoring systems) are needed, to further understand neonatal MODS and assess strategies for early prevention and treatment. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
多器官功能衰竭(MOF),或多器官功能障碍综合征(MODS),如最近所知,是一种临床综合征,其特征是两个或两个以上的器官在没有干预的情况下无法维持体内平衡。已描述的新生儿期MODS的病因包括败血症、任何原因引起的休克、组织灌注不足、早产、缺氧缺血性脑病、坏死性小肠结肠炎(NEC)、手术、先天性心脏病等。MOF可以被认为是免疫、细胞因子和激素变化的最终共同途径,是对不同感染性或非感染性炎症损伤的生理反应,导致全身性炎症、促凝状态和进行性器官功能障碍。MODS的临床表现可能有很大差异,这取决于主要原因、性质、器官系统的数量和严重程度。应及时发现和治疗mods前的情况。在严重败血症和感染性休克的情况下,应遵循现有的指导方针。当MODS已经发生时,应提供单器官功能障碍的支持治疗,并维持充足的氧合和器官灌注。需要对足月和早产儿进行更多的研究(随着特定新生儿评分系统的发展),以进一步了解新生儿MODS并评估早期预防和治疗策略。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 9
Hyaline membrane disease (HMD): the role of the perinatal pathologist 透明膜病(HMD):围产期病理学家的作用
IF 0.4 Q4 PEDIATRICS Pub Date : 2014-10-21 DOI: 10.7363/030255
Giorgia Locci, V. Fanos, C. Gerosa, G. Faa
Hyaline membrane disease (HMD), the pathologic correlate of respiratory distress syndrome (RDS) of the newborn, is an acute lung disease of premature infant caused by inadequate amounts of surfactant. Decreased surfactant results in insufficient surface tension in the alveolus during expiration, leading to atelectasis, decreased gas exchange, severe hypoxia and acidosis. HMD predominantly occurs in infants younger than 32 weeks of gestation and weighing less than 1,200 g. In the interpretation of perinatal lung pathology, it is necessary to consider the development of the immature lung, particulary in the third trimester. Microscopically HMD is characterized by the occurrence of dilated terminal and respiratory bronchioles and of alveolar ducts lined by acellular eosinophilic hyaline membranes. The membranes are composed of necrotic alveolar lining cells, amniotic fluid constituents and fibrin. Retinopathy of prematurity and bronchopulmonary dysplasia are late complications of RDS that usually occur in infants who weigh less than 1,500 g and were maintained on a mechanical respiration more than 6 days. Here a pratical approach to a microscopic analysis of the lung in newborns died with the clinical setting of RDS is presented. The most important pathological findings for a complete clinical pathological diagnosis are: the evaluation of the architectural lung development; the endothelial cell lesions; the interstitial edema; the occurrence of disseminated intravascular coagulation; the presence of associated inflammatory lesions. The usefulness of some immunohistochemical stains is also underlined, including anti-surfactant, anti-smooth muscle actin and anti-CD31 to better evaluate surfactant production, pulmonary artery maturation and endothelial cell damage, respectively. Finally, the prevalent role of endothelial dysfunction and endothelial barrier loss is underlined, representing a major pathological event in the deposition of HMD. Proceedings of the International Course on Perinatal Pathology (part of the 10 th International Workshop on Neonatology · October 22 nd -25 th , 2014) · Cagliari (Italy) · October 25 th , 2014 ·  The role of the clinical pathological dialogue in problem solving Guest Editors: Gavino Faa, Vassilios Fanos, Peter Van Eyken
透明膜病(HMD)是新生儿呼吸窘迫综合征(RDS)的病理相关,是由表面活性剂量不足引起的早产儿急性肺部疾病。表面活性剂的减少导致呼气时肺泡表面张力不足,导致肺不张、气体交换减少、严重缺氧和酸中毒。HMD主要发生在妊娠32周以下、体重低于1200克的婴儿中。在围产期肺病理的解释中,有必要考虑未成熟肺的发育,特别是在妊娠晚期。显微镜下HMD的特征是出现扩张的终末细支气管和呼吸性细支气管,肺泡管内衬无细胞嗜酸性透明膜。膜由坏死的肺泡衬里细胞、羊水成分和纤维蛋白组成。早产儿视网膜病变和支气管肺发育不良是RDS的晚期并发症,通常发生在体重低于1500克并维持机械呼吸6天以上的婴儿中。在这里,一个实用的方法,以显微镜分析的肺在新生儿死亡与临床设置RDS提出。完整的临床病理诊断最重要的病理表现是:评价肺的建筑发育;内皮细胞病变;间质性水肿;弥漫性血管内凝血的发生;伴有炎性病变。一些免疫组织化学染色的作用也被强调,包括抗表面活性剂、抗平滑肌肌动蛋白和抗cd31,分别更好地评估表面活性剂的产生、肺动脉成熟和内皮细胞损伤。最后,强调了内皮功能障碍和内皮屏障丧失的普遍作用,这代表了HMD沉积的主要病理事件。围产期病理学国际课程论文集(第10届新生儿国际研讨会的一部分,2014年10月22日至25日)·卡利亚里(意大利)·2014年10月25日·临床病理对话在解决问题中的作用特邀编辑:Gavino Faa, Vassilios Fanos, Peter Van Eyken
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引用次数: 7
期刊
Journal of Pediatric and Neonatal Individualized Medicine
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