The open pediatric medicine journal最新文献

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Anaplastic Large Cell Lymphoma ALK-Negative: About a Rare Pediatric Case Report 间变性大细胞淋巴瘤alk阴性:关于一个罕见的儿科病例报告

The open pediatric medicine journal

Pub Date : 2020-06-16 DOI: 10.2174/1874309902010010003

Louachama Ouidad, Elbaz Meriem, El Houdzi Jamila

Anaplastic Large Cell Lymphomas (ALCL) are rare in childhood but clinically aggressive. The contemporary World Health Organization (WHO) classification of hematologic malignancies recognizes two distinct subtypes of systemic ALCL: Anaplastic Lymphoma Kinase (ALK)-negative, and ALK-positive. We report a rare pediatric observation of cutaneous nodule revealing an Anaplastic Large Cell Lymphoma (ALCL) ALK - cutaneous, muscular and ganglionic. It was classified according to who classification, treated by polychemotherapy with good outcome. ALCL encompasses several distinct clinicopathologic entities with unique genomic under printing. This rare pediatric observation of ALCL presents a new discussion on a pathology still incompletely known.

间变性大细胞淋巴瘤(ALCL)在儿童时期罕见，但临床上具有侵袭性。当代世界卫生组织(WHO)对血液恶性肿瘤的分类识别出系统性ALCL的两种不同亚型:间变性淋巴瘤激酶(ALK)阴性和ALK阳性。我们报告一个罕见的儿童皮肤结节显示间变性大细胞淋巴瘤(ALCL) ALK -皮肤，肌肉和神经节。根据who分类，采用综合化疗治疗，疗效良好。ALCL包括几种不同的临床病理实体，具有独特的基因组打印。这种罕见的儿科ALCL观察提出了一个新的病理尚不完全清楚的讨论。

引用次数: 0

Children and Coronavirus Infection (Covid-19): What to Tell Children to Avoid Post-traumatic Stress Disorder (PTSD) 儿童与冠状病毒感染(Covid-19):如何告诉儿童避免创伤后应激障碍(PTSD)

The open pediatric medicine journal

Pub Date : 2020-04-15 DOI: 10.2174/1874309902010010001

M. Roccella

Acute Stress Disorder (ASD) usually begins immediately after the traumatic event and lasts from three days to a month, while Post-traumatic Stress Disorder (PTSD) may be the continuation of an acute stress disorder and it may happen that it does not develop until 6 months after the event itself. Generally most people overcome the shock that a blatant event can cause without the need for additional support. In a percentage of cases, the victim's suffering can be prolonged for more than a month after exposure to trauma and significantly interfere with the individual's working, social or school life; in this case the diagnosis of PTSD must be made.

急性应激障碍(ASD)通常在创伤事件后立即开始，持续三天到一个月，而创伤后应激障碍(PTSD)可能是急性应激障碍的延续，可能在事件发生后6个月才发展起来。一般来说，大多数人在不需要额外支持的情况下克服了公然事件可能造成的震惊。在一定比例的情况下，受害者的痛苦在遭受创伤后可以延长一个多月，并严重干扰个人的工作、社交或学校生活;在这种情况下，必须做出创伤后应激障碍的诊断。

引用次数: 30

Simple Virilizing Congenital Adrenal Hyperplasia: A case Report of Sudanese 46, XY DSD male with G293D variant in CYP21A2 单纯性男性化先天性肾上腺增生:苏丹46,xy DSD男性CYP21A2基因G293D变异1例

The open pediatric medicine journal

Pub Date : 2019-12-31 DOI: 10.2174/1874309901909010007

M. Ellaithi, Idoia Martinez de Lapiscina, A. B. Hoz, G. P. D. Nanclares, Marwah Abdelrahman Alasha, M. A. Hemaida, L. Castaño

Congenital Adrenal Hyperplasia (CAH) is a family of inherited disorders that constitute the largest group of Disorders of Sexual Development (DSDs). The classical CAH has two types; the salt-wasting (SW-CAH) and the simple virilizing (SV-CAH). This study is a report of an SV-CAH regarding 46, XY DSD Sudanese male with early signs of puberty at the age of six years. We designed a customized panel that included 48 genes associated with Disorders of Sexual Development (DSDs) and using Next Generation Sequencing (NGS) technology, detected the pathogenic G293D alteration in the CYP21A2 gene. This variant has been reported in the salt-wasting (SW) form of 46, XX CAH.

先天性肾上腺增生症(CAH)是一个家族的遗传性疾病，构成最大的组性发育障碍(dsd)。经典的CAH有两种类型;盐浪费(SW-CAH)和简单的男性化(SV-CAH)。本研究是一份关于46,xy DSD苏丹男性的SV-CAH报告，该男性在6岁时出现青春期的早期迹象。我们设计了一个包含48个与性发育障碍(dsd)相关基因的定制面板，并使用下一代测序(NGS)技术检测了CYP21A2基因的致病性G293D改变。这种变异在46,xx CAH的盐蚀(SW)形式中有报道。

引用次数: 1

Recurrent Hemolytic Anemia as an Inaugural Manifestation of Wilson Disease in Children: A Case Report 复发性溶血性贫血是儿童肝豆状核病的首要表现:1例报告

The open pediatric medicine journal

Pub Date : 2019-02-25 DOI: 10.2174/1874309901909010005

O. Louachama, A. Bourrahouat, Ibtissam Khattou, I. A. Sab, M. Sbihi

Wilson disease (WD) is a disorder of copper metabolism. Liver and brain disorders are the main presentations, hemolytic anemia in WD is a rare inaugural symptom. We report a case of a child who developed recurrent hemolytic anemia associated with liver failure in the second hemolysis episode as the first manifestation of WD. Wilson's disease is not exceptional in children with hemolytic anemia, but another differential diagnosis must be excluded.

威尔逊病(WD)是一种铜代谢障碍。肝脑功能障碍是主要的表现，溶血性贫血是一个罕见的首发症状。我们报告一个病例的儿童谁发展复发性溶血性贫血与肝功能衰竭在第二次溶血发作作为WD的第一表现。威尔逊氏病在儿童溶血性贫血中并不少见，但必须排除其他鉴别诊断。

引用次数: 0

Infantile GM1-Gangliosidosis Revealed by Slate-Grey Mongolian Spots 板灰色蒙古斑揭示婴儿gm1神经节脂质沉积症

The open pediatric medicine journal

Pub Date : 2019-01-31 DOI: 10.2174/1874309901909010001

G. Draiss, A. Fouad, N. Rada, O. Hocar, N. Fdil, M. Bouskraoui

GM1-gangliosidosis is an inherited metabolic disease caused by mutations in the GLB1 gene resulting in deficiency of β-galactosidase. Three forms have been identified: Infantile, juvenile, and adult. The infantile type progresses rapidly and aggressively and a delayed diagnosis hampers the prevention of many neurological deficits. This delay in diagnosis may be due to the variability of clinical expression of the disorder. Extensive Mongolian or slate-grey spots deserve special attention as possible indications of associated inborn errors of metabolism, especially GM1-gangliosidosis and mucopolysaccharidosis. Only symptomatic treatments are available for GM1-gangliosidosis; research is underway. In this article, we report a case of infantile GM1-gangliosidosis revealed by slate-grey Mongolian spots, a rare condition in Morocco, and a review of the literature. The finding of persistent and extensive slate-grey mongolian spots in infant could lead to early detection of GM1-gangliosidosis before irreversible organ damage occurs.

gm1神经节脂质病是由GLB1基因突变导致β-半乳糖苷酶缺乏引起的遗传性代谢性疾病。已经确定了三种形式:婴儿、少年和成人。婴儿型发展迅速，积极和延迟诊断阻碍了许多神经功能障碍的预防。这种延迟诊断可能是由于疾病的临床表现的可变性。广泛的蒙古灰斑或石板灰斑值得特别注意，因为它们可能是相关的先天性代谢错误的指示，特别是gm1神经节脂质病和粘多糖病。gm1神经节脂质沉积症只有对症治疗;研究正在进行中。在这篇文章中，我们报告了一例由石板灰色蒙古斑显示的婴儿gm1神经节脂质沉积症，这是一种罕见的情况，在摩洛哥，并回顾了文献。在婴儿中发现持续和广泛的石板灰色蒙古斑，可以在发生不可逆器官损害之前早期发现gm1神经节脂质沉积症。

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引用次数: 1

Spartan and Sambian Societies: Psychosocial Development Stifling Emotional Empathy and Supporting Violent Behaviors 斯巴达和桑比亚社会:抑制情感同理心和支持暴力行为的社会心理发展

The open pediatric medicine journal

Pub Date : 2014-12-11 DOI: 10.2174/1874309901408010001

G. Katzman

The male children of the ancient Spartans of Greece and the Sambia of New Guinea are exposed to programmed mental maltreatment and physically abusive practices. Through these exposures and societal narratives enforced by coercion and intimidation, aggressive warriors ready to fight to maintain their engrained desire to dominate or fulfill the military goals of the community are produced. Unfortunately, the indoctrination process appears to suppress the capacity for emotional empathy, further facilitating aggressive impulses that are unimpaired due to the lack of orientation to commonly accepted moral values. The implications of the indoctrinations are discussed with suggestions as to what can be done to promote more desirable traits during growth and development.

希腊的古斯巴达人和新几内亚的桑比亚人的男孩受到有计划的精神虐待和身体虐待。通过这些暴露和社会叙事的强制和恐吓，积极的战士准备战斗，以维持他们根深蒂固的欲望，统治或实现社区的军事目标。不幸的是，灌输过程似乎抑制了情感共鸣的能力，进一步促进了由于缺乏普遍接受的道德价值观而未受损害的攻击冲动。讨论了灌输的含义，并提出了在生长和发育过程中可以做些什么来促进更理想的特征的建议。

引用次数: 3

Breastfeeding During Early Infancy is Associated with Higher Weight-Based World Health Organization Anthropometry. 婴儿早期母乳喂养与较高的基于体重的世界卫生组织人体测量有关。

The open pediatric medicine journal

Pub Date : 2013-06-28 DOI: 10.2174/1874309920130610001

Daniel H Libraty, Rosario Z Capeding, Anamae Obcena, Job D Brion, Veronica Tallo

The World Health Organization (WHO) Expert Committee on Physical Status: The Use and Interpretation of Anthropometry established reference anthropometric standards for the growth of healthy infants and children. As part of a prospective clinical study of dengue virus infections in infants, we measured the length and weight of healthy infants in San Pablo, Laguna, Philippines at two scheduled study visits. We examined the correlation between breastfeeding and WHO anthropometric z scores during early infancy in San Pablo, Laguna, Philippines. We found that breastfeeding status and the frequency of breastfeeding during early infancy positively correlated with weight-based WHO anthropometric z scores.

世界卫生组织（WHO）体格状态专家委员会：人体测量的使用和解释》为健康婴儿和儿童的生长制定了参考人体测量标准。作为登革热病毒感染婴儿前瞻性临床研究的一部分，我们在菲律宾拉古纳省圣巴勃罗市的两次预定研究访问中测量了健康婴儿的身长和体重。我们研究了菲律宾拉古纳省圣巴勃罗市婴儿早期母乳喂养与世界卫生组织人体测量 Z 值之间的相关性。我们发现，婴儿早期的母乳喂养状况和母乳喂养频率与基于体重的世卫组织人体测量 z 分数呈正相关。

引用次数: 0

Breastfeeding During Early Infancy is Associated with a Lower Incidence of Febrile Illnesses. 婴儿早期母乳喂养可降低发热性疾病的发病率。

The open pediatric medicine journal

Pub Date : 2013-06-24 DOI: 10.2174/1874309920130621002

Daniel H Libraty, Rosario Z Capeding, Anamae Obcena, Job D Brion, Veronica Tallo

Human breast milk is known to contain immunoprotective, antimicrobial, and anti-inflammatory agents. In a prospective clinical study of dengue virus infections during infancy, we examined the correlation between breastfeeding and the development of febrile illnesses in an infant population. We found that breastfeeding status and the frequency of breastfeeding during early infancy was associated with a lower incidence of febrile illnesses.

已知人类母乳含有免疫保护、抗菌和抗炎剂。在一项对婴幼儿登革热病毒感染的前瞻性临床研究中，我们研究了母乳喂养与婴幼儿发热性疾病发展之间的相关性。我们发现，母乳喂养状况和母乳喂养的频率在婴儿期早期与发热性疾病的发生率较低有关。

引用次数: 2

Pediatric Shock: An Overview 儿科休克:综述

The open pediatric medicine journal

Pub Date : 2013-02-22 DOI: 10.2174/1874309901307010002

D. Wheeler, Rajit K. Basu

Shock is one of the most frequently diagnosed, yet poorly understood disorders in the pediatric intensive care unit (PICU). The very definition of what constellation of physical signs and symptoms that comprise shock remains controversial, in part due to the vast array of disorders that cause shock in critically ill and injured children. Early management and reversal of the shock state is associated with significantly improved outcomes. However, early management is critically dependent upon the early recognition and diagnosis of shock at the bedside. Failure to recognize the signs and symptoms of shock and to institute timely and appropriate care leads to higher mortality rates in both children and adults. Clinical recognition of shock requires a high index of suspicion - as such, all pediatric health care providers should be cognizant of the clinical presentation, pathophysiology, and early management of shock.

休克是儿科重症监护病房(PICU)最常见的疾病之一，但人们对其了解甚少。对于哪些体征和症状构成休克的定义仍然存在争议，部分原因是在重症和受伤儿童中引起休克的疾病种类繁多。早期治疗和休克状态的逆转与显著改善的预后相关。然而，早期处理是关键依赖于休克的早期识别和诊断在床边。未能认识到休克的体征和症状，并采取及时和适当的护理，导致儿童和成人的死亡率较高。临床对休克的识别需要高度的怀疑，因此，所有儿科医疗保健提供者都应该认识到休克的临床表现、病理生理和早期处理。

引用次数: 8

Editorial - “The Rude Unhinging of the Machinery of Life” 社论-“生命机器的粗暴解体”

The open pediatric medicine journal

Pub Date : 2013-02-22 DOI: 10.2174/1874309901307010001

D. Wheeler

Shock is one of the most common reasons that children are admitted to the pediatric intensive care unit (PICU). Early recognition and reversal of the shock state are essential to improved outcomes. Unfortunately, shock as a distinct disease state is frequently misunderstood and underrecognized. For example, the clinical definition of shock remains controversial, in large part due to vast number of disorders that cause shock in critically ill and injured children. Shock frequently represents the final stage of clinical deterioration in a number of these disorders, so that the initial signs and symptoms of shock are frequently attributed to the underlying cause and not shock per se.

休克是儿童入住儿科重症监护病房(PICU)最常见的原因之一。早期识别和逆转休克状态对改善预后至关重要。不幸的是，休克作为一种独特的疾病状态经常被误解和低估。例如，休克的临床定义仍然存在争议，这在很大程度上是由于大量疾病导致重症和受伤儿童休克。休克通常是这些疾病临床恶化的最后阶段，因此休克的初始体征和症状通常归因于潜在原因，而不是休克本身。

引用次数: 0

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