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Neuroprotective Effects Of D-Penicillamine In The Neonatal Period: Case Reports d -青霉胺在新生儿期的神经保护作用:病例报告
Pub Date : 2019-10-30 DOI: 10.9734/ijmpcr/2015/17239
L. Lakatos
“fetal copper is ….with Abstract D-penicillamine (D-PA) was first used as a potential benefit for neonatal hyperbilirubinemia (NHBI) caused by hemolytic diseases of the newborn infant or immaturity of UDP-glucuronyltransferase enzyme. In this time there was a remarkedly low incidence of retinopathy of prematurity (ROP) in the infants treated with D-PA. Later, our studies were replicated in other institutes in Hungary, Poland, the USA, India and Mexico. It is important to note that there was no intolerance or short- or long-term toxicity of the medication, in spite of the fact that D-PA was used 10-20 times higher doses in the newborn period, than those in adult age. To our concept, the bilirubin-induced neurologic dysfunction (BIND), ROP and Autism Spectrum Disorders (ASD) are neurodegenerative and neurodevelopmental diseases (NDs) of immature brain caused by accumulation of free metals, unconjugated bilirubin (UCB), and UCB-Cu complex (as prooxidant), respectively, in the basal ganglia (BG) and other relevant parts of the central nervous system (CNS). The main cause is the hemolysis of neonatal red blood cells producing a great amount of heavy metals (mainly iron and copper) which are inducing reactive oxygen species (ROS). These elements can be find in the bloodstream, and pass through the immature blood-brain-barrier (BBB). In addition, ROS contribute to increased BBB permeability creating a dangerous vitious circle in the neonatal brain.
胎铜是....d -青霉胺(D-PA)首次被用作治疗新生儿高胆红素血症(NHBI)的潜在药物,该疾病是由新生儿溶血性疾病或udp -葡萄糖醛基转移酶不成熟引起的。在此期间,接受D-PA治疗的婴儿的早产儿视网膜病变(ROP)发生率明显降低。后来,我们的研究在匈牙利、波兰、美国、印度和墨西哥的其他研究所得到了复制。值得注意的是,尽管新生儿使用的D-PA剂量比成人高10-20倍,但该药物没有不耐受或短期或长期毒性。在我们的概念中,胆红素诱导的神经功能障碍(BIND)、ROP和自闭症谱系障碍(ASD)是由于游离金属、未结合胆红素(unconjugated bilirubin, UCB)和UCB- cu复合物(作为促氧化剂)分别在基底节区(basal ganglia, BG)和中枢神经系统(central nervous system, CNS)的其他相关部位积累而引起的未成熟大脑的神经退行性和神经发育性疾病(neurodevelopmental diseases, NDs)。主要原因是新生儿红细胞溶血产生大量重金属(主要是铁和铜),这些重金属会诱导活性氧(ROS)。这些元素可以在血液中找到,并通过未成熟的血脑屏障(BBB)。此外,活性氧有助于增加血脑屏障的通透性,在新生儿大脑中形成危险的恶性循环。
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引用次数: 6
Detecting Ehlers-Danlos Syndrome Early in Life Is an Urgent Priority 在生命早期发现埃勒-丹洛斯综合征是当务之急
Pub Date : 2019-08-19 DOI: 10.32474/papn.2019.02.000139
H. C.
Ehlers-Danlos syndrome is a frequent hereditary disease that affects all the connective tissue and is transmitted to all the children of an affected person. A diagnosis is possible, from birth, or shortly thereafter, when observing a clubfoot, hip dislocation, intestinal intussusception, acute umbilical or inguinal parietal hernia, hemorrhages [cutaneous, oral, gastric, intestinal, nasal], persistent constipation, regurgitation and vomiting during bottle-feeding, or false roads. These symptoms are often the cause of false allegations of abuse with children withdrawal and wrongful parents or false accusations of Munchausen disease “by delegation” in a parent, the mother most often.
埃勒-丹洛斯综合征是一种常见的遗传性疾病,会影响所有结缔组织,并会传染给患者的所有孩子。当观察到畸形足、髋关节脱位、肠套叠、急性脐或腹股沟壁疝、出血(皮肤、口腔、胃、肠、鼻)、持续便秘、喂奶时反胃和呕吐或假路时,从出生或出生后不久就可以诊断。这些症状通常会导致错误地指控虐待儿童的退缩和错误的父母,或错误地指控父母“委托”患有孟乔森病,最常见的是母亲。
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引用次数: 0
Unusual Presentation of Myasthenia Gravis 重症肌无力的不寻常表现
Pub Date : 2019-04-05 DOI: 10.32474/PAPN.2019.02.000135
J. Rawal, O. Davies, Shatha Al-ani
A 12 year old, previously fit & well girl, presented to A&E withh/o worsening Cough for 3 days and difficulty in breathing for 24hours. She needed 15 litres of oxygen to maintain saturations of94%.
一名12岁女孩,既往健康,因咳嗽加重3天,呼吸困难24小时而就诊于急诊科。她需要15升氧气来维持94%的血氧饱和度。
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引用次数: 0
Ifosfamide Encephalopathy in A 4yr Old with Ewing’s Sarcoma 4岁儿童尤因氏肉瘤异环磷酰胺性脑病
Pub Date : 2019-02-08 DOI: 10.32474/papn.2019.02.000133
E. Dias, A. Dias
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引用次数: 0
Assessing Health Risks in Children and Adolescents in Pediatric Care: Let’s Not Forget Social Determinants of Health 评估儿童和青少年的健康风险:让我们不要忘记健康的社会决定因素
Pub Date : 2019-02-05 DOI: 10.32474/papn.2019.02.000131
Jeanne Cartier
Within the practice of pediatric medicine, a family and social history has historically been a part of the assessment of children. However, the scope of said history was usually limited to understanding the family composition, family health history and developmental behavior and activities of the child. Ongoing advances in basic and clinical sciences have increased knowledge of underlying disease etiology, including the role of genetics and genomics, brain physiology and immune functioning in disease origins and development, have led to the discovery of new and effective pharmacological agents and have informed the development of evidenced based treatment protocols. These advances have clearly improved treatment of existing diseases and conditions. There has also been advances in understanding the role of social factors as contributors to both immediate and long term health outcomes for patients throughout childhood and reaching into adulthood. Medical care as well as genetics, social circumstances, behavior, environmental and physical influences have been defined as the major determinants of health [1]. While access to medical care and clinical care contribute to health outcomes, more than 80% of the health outcomes are attributable to health behaviors as well as social and environmental factors [2]. Pediatrics is well situated to take the lead for including a broader and more robust social history to deepen our understanding, assessment and treatment of these risk factors.
在儿科医学实践中,家庭和社会病史历来是儿童评估的一部分。然而,所述病史的范围通常限于了解家庭组成、家庭健康史以及儿童的发育行为和活动。基础科学和临床科学不断取得进展,增加了对潜在疾病病因学的认识,包括遗传学和基因组学、脑生理学和免疫功能在疾病起源和发展中的作用,导致发现了新的有效药理学制剂,并为制定循证治疗方案提供了信息。这些进步明显改善了对现有疾病和病症的治疗。在了解社会因素对患者从童年到成年的直接和长期健康结果的影响方面也取得了进展。医疗保健以及遗传、社会环境、行为、环境和身体影响已被定义为健康的主要决定因素[1]。虽然获得医疗保健和临床护理有助于健康结果,但超过80%的健康结果可归因于健康行为以及社会和环境因素[2]。儿科学在包含更广泛和更健全的社会历史方面处于领先地位,以加深我们对这些风险因素的理解、评估和治疗。
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引用次数: 0
Effect of Early Amino Acids Supplementation on Serum Glucose and Calcium of Very Low Birth Weight Premature Infants 早期补充氨基酸对极低出生体重早产儿血清葡萄糖和钙的影响
Pub Date : 2019-02-05 DOI: 10.32474/papn.2019.02.000132
Ghassan S. A. Salama
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引用次数: 0
Use Judiciously, or I Will Be Useless” A Clinical Audit on Use of Antibiotics Within First 72 hrs of Life in Symptomatic Term Babies With no Maternal Risk for Infection 明智地使用,否则我将是无用的“一项对无母亲感染风险的有症状的足月婴儿在生命最初72小时内使用抗生素的临床审计
Pub Date : 2019-02-01 DOI: 10.32474/papn.2019.02.000128
K. Salameh, Abedal khalik Ahmad Khedr, R. Valappil, A. Tomerak
Over use of antibiotics lead to resistance and unwanted sideeffects in newborns. Since 1992, professional societies or public health agencies have issued several generations of recommendations for prevention or management of early-onset neonatal sepsis (EOS) [1-5]. Despite these efforts, recommendations remain inconsistent, clarifications are necessary, local adaptations are common, and compliance rates are low [6-7]. It is common clinical practice to discontinue antibiotic treatment of asymptomatic babies if the blood cultures are negative at 48 hours [8-10]. But it is very common to have prolonged antibiotic courses for more than 48 hours due to delayed release of blood culture, high CRP, abnormal CBC or delayed decision by the Physician to stop antibiotics. In a previous study, McDonald et al. [11] found this as a common occurrence in neonatal intensive care units. The purpose of this audit is to rationalize the use of antibiotics in symptomatic term babies, with no risk for infection.
过度使用抗生素会导致新生儿产生耐药性和不良副作用。自1992年以来,专业协会或公共卫生机构已经发布了几代关于预防或管理早发性新生儿脓毒症(EOS)的建议[1-5]。尽管做出了这些努力,但建议仍然不一致,需要澄清,地方适应很常见,遵守率很低[6-7]。如果48小时血培养呈阴性,临床通常会停止对无症状婴儿的抗生素治疗[8-10]。但由于血培养物释放延迟、CRP升高、CBC异常或医生停止使用抗生素的决定延迟,延长抗生素疗程超过48小时是很常见的。在之前的研究中,McDonald等人[11]发现这种情况在新生儿重症监护病房很常见。本次审核的目的是合理化使用抗生素在有症状的足月婴儿,没有感染的风险。
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引用次数: 0
Phytochemical Analysis of Euphorbia Hirta and Cytotoxic Activity on Ma104 大戟的植物化学分析及对Ma104的细胞毒活性
Pub Date : 2019-02-01 DOI: 10.32474/papn.2019.02.000130
P. Arun, Kaveri Krishnasamic, Palani Gunasekeranc, V. Padmanabhan
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引用次数: 1
Greig Syndrome: A Rare Disease - Case Report 格里格综合征:一种罕见疾病病例报告
Pub Date : 2019-01-25 DOI: 10.32474/papn.2019.02.000129
A. Mithwani, Muhammad Ziad Shama, Assem Ahmad Kadrey, Abdullah AlomarAlmeshrif, Amerullah Malik
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引用次数: 0
Juvenile Myasthenia Gravis: A Short Review 青少年重症肌无力:简要回顾
Pub Date : 2018-11-29 DOI: 10.32474/PAPN.2018.02.000127
Shubhankar Mishra
Juvenile myasthenia gravis (JMG) is an autoimmune disorder of neuromuscular transmission caused by production of antibodies against components of the postsynaptic membrane of the neuromuscular junction. The patients present with a wide range of symptoms-from isolated intermittent ocular symptoms to general muscle weakness with or without respiratory insufficiency. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. It must be differentiated from congenital myasthenia which is a channelopathy rather than autoimmune disease. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG) are effective in preparation for surgery and in treatment of myasthenic crisis.
青少年重症肌无力(JMG)是一种神经肌肉传递的自身免疫性疾病,由产生针对神经肌肉连接处突触后膜成分的抗体引起。患者表现出广泛的症状-从孤立的间歇性眼部症状到全身肌肉无力伴或不伴呼吸功能不全。特别是青春期前的儿童,孤立性眼部症状的发生率较高,乙酰胆碱受体抗体的发生率较低,并且获得缓解的可能性较高。它必须与先天性重症肌无力鉴别,后者是一种血管病变而非自身免疫性疾病。治疗通常包括抗胆碱酯酶,皮质类固醇伴或不伴类固醇保留剂,和新的免疫调节剂。血浆置换和静脉注射免疫球蛋白(IVIG)在手术准备和治疗肌无力危象中是有效的。
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Progressing Aspects in Pediatrics and Neonatology
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