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Unusual cause of seizure in preterm infant 早产儿癫痫发作的不寻常原因
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_49_22
J. Alallah, Faisal Yonbawi, Yousef H Qari, Abdulaziz A Abu Alnasr, H. Kandil
Neonatal seizures are prevalent in premature babies and can be the earliest sign of neurological abnormalities. Seizures in newborns are clinically significant and further inquiry leading to a solid identification of the underlying disease can aid with prognosis and treatment options. We report a male neonate born at 27 + 6 weeks of gestation. An intractable seizure developed at 9 days of age. The diagnosis is confirmed based on genetic testing.
新生儿癫痫在早产儿中很普遍,可能是神经异常的最早迹象。新生儿癫痫发作具有重要的临床意义,进一步的调查可以确定潜在的疾病,有助于预后和治疗选择。我们报告一个男性新生儿出生在27 + 6周妊娠。9天大时出现难治性癫痫发作。该诊断是根据基因检测确定的。
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引用次数: 0
Congenital thrombotic thrombocytopenic purpura simulating alloimmune thrombocytopenia 先天性血栓性血小板减少性紫癜模拟同种免疫性血小板减少症
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_63_22
Muneer H Albagshi, A. A. Al Omran, Heba Elhakeem
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathic caused by severely reduced activity of the von Willebrand factor-cleaving protease A Disintegrin And Metalloproteinase with a Thrombospondin type 1 Motif, member 13 (ADAMTS-13), which leads to small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic hemolytic anemia (MHA) in the absence of neutralizing antibodies. We describe a case of cTTP in a female neonate presenting initially on the 1st day of life with asymptomatic thrombocytopenia thought to be alloimmune thrombocytopenia, then progressed to severe neonatal jaundice, pallor, and MHA, in whom ADAMTS-13 levels <5%, and the anti-ADAMTS-13 antibody titer was negative confirming the diagnosis of cTTP. The patient initially received intravenous immunoglobulin infusion before documenting MHA. She received fresh frozen plasma infusions which successfully reversed the MHA (by supplying ADAMTS-13) and prevented organ damage in this patient.
先天性血栓性血小板减少性紫癜(cTTP)是一种血栓性微血管疾病,由血管性血友病因子切割蛋白酶a崩解素和具有血小板反应蛋白1型Motif的金属蛋白酶13 (ADAMTS-13)活性严重降低引起,在缺乏中和抗体的情况下导致小血管血小板丰富的血栓、血小板减少和微血管致病性溶血性贫血(MHA)。我们描述了一例cTTP的女性新生儿,最初在出生的第一天出现无症状血小板减少症,被认为是同种免疫性血小板减少症,然后发展为严重的新生儿黄疸,苍白和MHA,其中ADAMTS-13水平<5%,抗ADAMTS-13抗体滴度为阴性,证实了cTTP的诊断。在记录MHA之前,患者最初接受静脉免疫球蛋白输注。她接受了新鲜的冷冻血浆输注,成功地逆转了MHA(通过提供ADAMTS-13),并防止了该患者的器官损伤。
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引用次数: 0
Below the threshold of “Viability” - A middle east experience 低于“生存能力”的门槛——中东的经验
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_55_22
B. Ofoegbu, A. Abdelshafy, Philip Simmons
Background: The Corniche Hospital, Abu Dhabi, is the largest and most advanced perinatal center in the United Arab Emirate (UAE), providing care in line with international standards within its 64-cot NICU, serving a mainly Muslim population. We share our experience on outcomes of babies born in our center at 22 weeks' gestation, below the threshold of viability. At this gestation, a senior neonatologist attends each delivery to assess the baby, offer stabilization whilst assessing the baby's response. We then looked simply at the outcomes of these births. Methods: Retrospective data was collected from June 2011 to Dec 2020 using electronic hospital records and our in-house neonatal database – a nine-and-a-half-year period. Information on all babies born between 22 + 0 to 22 + 6 weeks gestation was sought; Gestational age was calculated from last menstrual period (LMP). Results: Over a nearly 10-year period, just over 67,000 live births occurred at our center, of which 134 babies were between 22 + 0- and 22 + 6-week gestation at birth. Complete data was available in 114 babies who were assessed as alive at the onset of labor. Thirty-seven were still born and there were 77 live births, their average weight was 486 gm. Twenty-eight babies were offered comfort care, the other 49 babies were offered stabilization/resuscitation from which 20 babies were admitted to the NICU. Four babies, whose average weight was 536gms, survived to discharge. All four survivors have varying degrees of neuro-disability and chronic lung disease. Conclusion: This information may feed into the development of a framework, that supports parents and clinicians. This framework should reflect cultural, religious, and legal aspects applicable to the Middle East whilst providing direction on the care of babies born below the current definitions of viability within the region.
背景:阿布扎比滨海医院是阿拉伯联合酋长国(UAE)最大和最先进的围产期中心,其64床新生儿重症监护室提供符合国际标准的护理,主要服务于穆斯林人口。我们分享在我们中心出生的婴儿在妊娠22周,低于生存能力阈值的结果的经验。在这个妊娠期,一名高级新生儿科医生参加每一次分娩,以评估婴儿,提供稳定,同时评估婴儿的反应。然后,我们简单地观察了这些分娩的结果。方法:从2011年6月至2020年12月,使用电子医院记录和我们的内部新生儿数据库收集回顾性数据-为期9年半。收集了所有在妊娠22 + 0至22 + 6周之间出生的婴儿的信息;胎龄以末次月经(LMP)计算。结果:在近10年的时间里,在我们中心发生了67,000多例活产,其中134例婴儿出生时妊娠在22 + 0至22 + 6周之间。114名在分娩开始时被评估为活着的婴儿的完整数据可用。37例死产,77例活产,平均体重486克。28例患儿接受舒适护理,49例患儿接受稳定/复苏,其中20例患儿入住新生儿重症监护病房。4名平均体重为536克的婴儿存活并出院。所有四名幸存者都有不同程度的神经残疾和慢性肺病。结论:这些信息可能会被用于支持家长和临床医生的框架的发展。该框架应反映适用于中东的文化、宗教和法律方面,同时为该区域内低于目前生存能力定义出生的婴儿的护理提供指导。
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引用次数: 0
The trade-off between home oxygen and length of stay 家庭氧气和停留时间之间的权衡
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_71_22
Briana Hernandez, S. Manzar
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引用次数: 0
A rare case of aphallia associated with urethral atresia and multiple renal anomalies 尿道闭锁合并多处肾脏异常一例
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_82_22
M. Ahamed, K. Neethu
Aphallia, or penile agenesis, is an extremely rare congenital anomaly, resulting from the failure of the genital tubercle to develop normally during the early period of embryogenesis. We present the case of a baby born at term by normal vaginal delivery to a primi mother with an uneventful antenatal history, except for oligohydramnios detected in the third trimester. Examination of the baby revealed an absence of phallus and external urethral meatus, normally descended testes, a normal scrotum, and a normally positioned patent anal orifice. Subsequent workup of the baby by radiological studies revealed a malrotated right kidney in the midline, multicystic renal dysplasia of the left kidney, and urethral atresia.
阴茎缺失,或称阴茎发育不全,是一种极其罕见的先天性异常,是由于生殖结节在胚胎发生早期未能正常发育所致。我们提出的情况下,在足月出生的婴儿正常阴道分娩的原母与一个平静的产前史,除了在第三个月发现羊水过少。婴儿检查发现阴茎和外尿道道缺失,睾丸正常下降,阴囊正常,肛门孔正常定位未闭。随后对婴儿的影像学检查显示右肾中线旋转不良,左肾多囊性发育不良,尿道闭锁。
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引用次数: 0
Persistent left superior vena cava and ipsilateral intraventricular hemorrhage in a preterm infant 早产儿持续性左上腔静脉和同侧脑室出血
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_78_22
Adenike Karunwi, S. Manzar, Ryan Jones
We describe a preterm infant with a peripherally inserted central catheter (PICC) placement. The PICC line was suspected to be in the persistent left superior vena cava (PLSVC), confirmed by echocardiogram. PLSVC is a rare anomaly. On routine screening, the infant was noted to have an ipsilateral intraventricular hemorrhage (IVH) postulated to be secondary to the altered retrograde blood flow through the PLSVC. No such association has been reported previously. The case presentation is followed by evidence supporting the postulated mechanism. Although the association between PLSVC and IVH could be coincidental, clinicians should be aware of the blood flow-related problem associated with PLSVC in preterm infants.
我们描述了一例放置外周插入中心导管(PICC)的早产儿。经超声心动图证实,PICC线疑似位于持续性左上腔静脉(PLSVC)。PLSVC是一种罕见的异常现象。在常规筛查中,婴儿发现同侧脑室内出血(IVH),推测为继发于PLSVC逆行血流的改变。此前没有此类关联的报告。案例陈述之后是支持假定机制的证据。尽管PLSVC和IVH之间的关联可能是巧合,但临床医生应该意识到早产儿PLSVC的血流相关问题。
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引用次数: 0
Neonatal ABO incompatibility, influence of blood group, and coomb's test on outcome 新生儿ABO血型不合、血型及库姆试验对预后的影响
IF 0.2 Pub Date : 2022-10-01 DOI: 10.4103/jcn.jcn_64_22
Sarhan H. Alshammari, Azzam Alqashami, Saleh Alhumud, Meshari Aladadh, S. Alsaif, K. Ali
Background: Better understanding of the clinical characteristics of ABO hemolytic disease in neonates helps optimize care. Objective: To assess the morbidity associated with ABO incompatibility. Materials and Methods: Neonates with blood groups A or B born to mothers with blood group O were studied. Need for phototherapy (PT), intensive PT, intravenous immunoglobulin (IVIG) therapy, and packed red blood cell (PRBC) transfusions was studied. Results: The O–A group accounted for 57.2% and the O–B group 42.8%. Three-hundred and seventy-nine (46%) were Direct Coomb's test (DCT) positive. Need for PT and intensive PT was significantly higher in DCT-positive infants compared to DCT-negative infants (P < 0.001). DCT-positive infants required more PRBC transfusions (5.3% vs. 0.9%, P < 0.001) and IVIG therapy (6% vs. 0.2%, P < 0.001) compared to DCT-negative infants. Sixty percent of the blood group B infants were DCT positive compared to 35% of blood group A infants (P < 0.001). Need for PT (62% vs. 39%, P < 0.001), intensive PT (17% vs. 4%, P < 0.001), and IVIG therapy (5.4% vs. 0.8%, P < 0.001) was higher in infants with blood group B. TcB measured at median age of 12 h was predictive of the need for PT (area under the receiver operator characteristic [AUROC] =0.867) and intensive PT (AUROC = 0.917). The maximum reticulocyte percentage was predictive for both the need for IVIG therapy (AUROC = 0.978) and PRBC transfusion (AUROC = 0.863). Conclusions: DCT-positive infants and infants with blood group B suffered significantly higher adverse outcomes. TcB measured at 12 h is predictive of the need for PT and intensive PT. Maximum reticulocytes count is predictive of the severity of ABO incompatibility as evidenced by the need for PRBC transfusions and IVIG therapy.
背景:更好地了解新生儿ABO溶血性疾病的临床特点有助于优化护理。目的:探讨ABO血型不合的发病率。材料与方法:对O型血母亲所生A、B型血新生儿进行研究。对光疗(PT)、强化PT、静脉免疫球蛋白(IVIG)治疗和填充红细胞(PRBC)输注的必要性进行了研究。结果:O-A组占57.2%,O-B组占42.8%。379例(46%)直接库姆氏试验(DCT)阳性。与dct阴性婴儿相比,dct阳性婴儿对PT和强化PT的需求明显更高(P < 0.001)。与dct阴性婴儿相比,dct阳性婴儿需要更多的PRBC输注(5.3% vs. 0.9%, P < 0.001)和IVIG治疗(6% vs. 0.2%, P < 0.001)。60%的B血型婴儿DCT阳性,而35%的A血型婴儿DCT阳性(P < 0.001)。b型血婴儿对PT的需求(62%对39%,P < 0.001)、强化PT(17%对4%,P < 0.001)和IVIG治疗的需求(5.4%对0.8%,P < 0.001)更高。中位年龄12 h时测量的TcB可预测对PT的需求(受者操作者特征下面积[AUROC] =0.867)和强化PT (AUROC = 0.917)。最大网织红细胞百分比预测是否需要IVIG治疗(AUROC = 0.978)和PRBC输血(AUROC = 0.863)。结论:dct阳性婴儿和B血型婴儿的不良结局明显更高。在12小时测量TcB可以预测是否需要PT和强化PT。最大网织网细胞计数可以预测ABO不相容的严重程度,这可以通过PRBC输注和IVIG治疗来证明。
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引用次数: 1
Enhancing the quality of neonatal care by improving mothers' knowledge, attitude, and practice through checklist intervention 通过检查表干预改善母亲的知识、态度和实践,提高新生儿护理质量
IF 0.2 Pub Date : 2022-07-01 DOI: 10.4103/jcn.jcn_20_22
S. Arunakumar, Sujatha Ramabhatta, S. Lakshmipathy, B. Raghunandan, K. Rashmi, Dudala Kumaraswamy, P. Mol, C. Anushree
Objectives: The objective of this study is to improve the newborn and self-care practices among postnatal mothers through quality improvement techniques and to create awareness about newborn and perinatal care practices in the Mother and Child Protection (MCP) Card. Materials and Methods: The barriers to low newborn and self-care practices were identified and evaluated using fishbone analysis. An operational team was formed, and a checklist was created. A total of four Plan-Do-Study-Act (PDSA) cycles were carried out. Baseline knowledge was collected using the questionnaire from the checklist. Appropriate education and counseling regarding newborns and self-care practices were taught. A variety of measures were used during counseling. The educated mothers were recalled after 5–7 days to assess their knowledge. Results: A total of 102 postnatal mothers were enrolled in the study, and 78 were available for follow-up after 5–7 days. During all study cycles, median checklist scores increased from 38 to 57 (PDSA 1), 37 to 58 (PDSA 2), 37 to 57 (PDSA 3), and 32.5 to 56 (PDSA 4). The increase in median checklist scores in all the PDSA cycles was statistically significant (P < 0.05). The median difference in checklist score from baseline in the chronological order of PDSA cycles was 19, 21.5, 22, and 23. After educating the mothers about the MCP Card, the percentage of mothers who read the information in the MCP Card increased from <5% to 38%. Conclusion: Quality Improvement principles such as the introduction of a checklist can be successfully implemented to improve the quality of newborns and self-care practices among postnatal mothers.
目的:本研究的目的是通过质量改进技术改善产后母亲的新生儿和自我护理实践,并在母婴保护(MCP)卡中提高对新生儿和围产期护理实践的认识。材料与方法:采用鱼骨分析法对低新生儿和自我保健实践的障碍进行识别和评价。成立了一个操作团队,并创建了一个检查清单。共进行了四个计划-执行-研究-行动周期。使用检查表中的问卷收集基线知识。教授了有关新生儿和自我保健做法的适当教育和咨询。在咨询过程中使用了多种措施。接受教育的母亲在5-7天后被召回,以评估其知识水平。结果:共有102名产后母亲入组,其中78名在5-7天后可随访。在所有研究周期中,检查表得分中位数从38上升到57 (PDSA 1),从37上升到58 (PDSA 2),从37上升到57 (PDSA 3),从32.5上升到56 (PDSA 4)。在所有PDSA周期中,检查表得分中位数上升有统计学意义(P < 0.05)。按PDSA周期的时间顺序,检查表得分与基线的中位数差异分别为19、21.5、22和23。在对母亲进行MCP卡的教育后,阅读MCP卡上信息的母亲比例从<5%增加到38%。结论:采用检查表等质量改进原则可以有效地提高新生儿质量和产后母亲的自我保健行为。
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引用次数: 0
A case of neonatal alloimmune neutropenia with an uncommon presentation 新生儿异免疫性中性粒细胞减少症一例
IF 0.2 Pub Date : 2022-07-01 DOI: 10.4103/jcn.jcn_28_21
M. Silva, V. Carvalho, F. Raposo, M. Lima, Miguel Costa
Neonatal alloimmune neutropenia (NAN) is a rare condition in neonates, with an incidence of <0.1%. It occurs when maternal antibodies against neutrophil antigens inherited from the father cross the placenta, destroying the neutrophils of the fetus and/or the newborn. We report a case of a female neonate who started exhibiting scattered petechiae over the trunk and lower limbs, a day after birth. The blood count revealed severe neutropenia and mild thrombocytopenia. Infections were excluded. Flow cytometry analysis confirmed the presence of antineutrophil immunoglobulin G antibodies in the newborn's neutrophil membrane and in the mother's serum. When screened for polymorphisms in the genes encoding for human neutrophil antigens (HNA), we identified HNA-1b and/or HNA-3a as potential causes of the mother/daughter incompatibility. Even though neutropenia is usually self-limiting, neonates with NAN are at risk of developing severe infections. Therefore, early identification of these patients can prevent serious complications.
新生儿异源性免疫性中性粒细胞减少症(NAN)是新生儿中一种罕见的疾病,发病率<0.1%。当母亲从父亲那里遗传的针对中性粒细胞抗原的抗体穿过胎盘,破坏胎儿和/或新生儿的中性粒细胞时,就会发生这种情况。我们报告了一例女性新生儿,出生后一天,其躯干和下肢开始出现分散的瘀点。血细胞计数显示严重的中性粒细胞减少症和轻度血小板减少症。已排除感染。流式细胞术分析证实,新生儿中性粒细胞膜和母亲血清中存在抗中性粒细胞免疫球蛋白G抗体。当筛选编码人类中性粒细胞抗原(HNA)的基因的多态性时,我们确定HNA-1b和/或HNA-3a是母子不相容的潜在原因。尽管中性粒细胞减少症通常是自限性的,但患有NAN的新生儿有患严重感染的风险。因此,早期发现这些患者可以预防严重并发症。
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引用次数: 0
A neonate with peromelia: congenital transverse deficiency of an upper limb 新生儿腓骨发育迟缓:先天性上肢横位缺损
IF 0.2 Pub Date : 2022-07-01 DOI: 10.4103/jcn.jcn_146_21
Rakesh Kumar, Sanober Wasim, G. Gupta, Mansi Naithani
Peromelia is a rare condition leading to the absence of limb. Birth of such a baby is an unexpected and emotionally disturbing event for the parents, if it was not diagnosed antenatally. Family members and especially the mother at such time need proper counseling from the health-care workers during the initial days. The management aspects including prosthesis and timing of intervention need to be informed to the parents. Here, we describe a case of peromelia with emphasis on the psychological aspect of management.
缺肢症是一种罕见的导致肢体缺失的疾病。如果没有产前诊断,这种婴儿的出生对父母来说是一件意想不到的、情感上令人不安的事情。在这种情况下,家庭成员,特别是母亲,需要在最初几天得到保健工作者的适当咨询。管理方面,包括假体和干预的时机需要告知家长。在这里,我们描述了一个peromelia的案例,重点是管理的心理方面。
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引用次数: 0
期刊
Journal of Clinical Neonatology
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