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Unraveling the impact of adenomyosis on obstetrical outcomes: a comprehensive review 揭示子宫腺肌病对产科结果的影响:一项全面的综述
Pub Date : 2023-08-07 DOI: 10.15406/ipcb.2023.09.00288
Ranjitha Vasa, O. Muneyyirci-Delale
Objective: Adenomyosis’ impact on obstetrical outcomes is investigated with detailed discussion of plausible pathogenesis. Discussion of management strategies to improve outcomes included. Methods of study: Terms utilized include ‘adenomyosis’, ‘fertility’, ‘ pregnancy’, ‘obstetrical outcomes’, ‘preeclampsia, ‘fetal growth restriction’, ‘preterm labor’, ‘preterm prelabor rupture of membranes, ‘in vitro fertilization, ‘prelabor rupture of membranes’, ‘ultrasound’, ‘small for gestational age’, ‘cesarean section’ Inclusion criteria are English, between January 1990-January 2023, randomized controlled trials, case controls, cohorts, case series, case reports, systematic reviews, and meta-analyses. Exclusion criteria are studies/articles completed prior to 1990, non-relevant, and non-English. Results: Limited literature exists evaluating the relationship between adenomyosis impact on obstetrical outcomes. However, amongst available literature there exists statistically significant relationship between adenomyosis and adverse obstetrical, neonatal outcomes such as: ectopic pregnancy, placental abruption, pre-eclampsia (PEC), gestational diabetes (GDM), low birth weight (LBW), intra-uterine growth restriction (IUGR), and preterm prelabor rupture of membranes (PPROM). Proposed physiologic mechanisms include disordered anatomic, functional, and immunological environment in the uterus. Proposed management strategies to improve obstetrical outcomes include removal of focal adenomyotic lesions and pre-treatment with GnRH agonists before conception. Conclusion: Adenomyosis has a multifactorial impact on obstetrical outcomes; treatment modalities do exist to improve the chances of conception and retaining a pregnancy. However, more research is required to not only further substantiate treatment modalities relationship to improve pregnancy; but also to clarify adenomyosis impact on infertility
目的:探讨子宫腺肌病对产科结局的影响,并详细讨论其可能的发病机制。讨论了改善结果的管理策略。研究方法:使用的术语包括“子宫腺肌病”、“生育能力”、“妊娠”、“产科结果”、“子痫前期”、“胎儿生长受限”、“早产”、“早产前胎膜破裂”、“体外受精”、“产前胎膜破裂”、“超声”、“小于胎龄”、“剖宫产”。纳入标准为英文,时间跨度为1990年1月至2023年1月,随机对照试验、病例对照、队列、病例系列、病例报告、系统评价和荟萃分析。排除标准是在1990年之前完成的研究/文章,非相关和非英语。结果:评价子宫腺肌病对产科结局影响的文献有限。然而,在现有文献中,子宫腺肌病与宫外孕、胎盘早剥、先兆子痫(PEC)、妊娠糖尿病(GDM)、低出生体重(LBW)、子宫内生长受限(IUGR)和早产胎膜破裂(PPROM)等不良产科和新生儿结局存在统计学意义上的关系。提出的生理机制包括子宫内的解剖、功能和免疫环境紊乱。建议的管理策略,以改善产科结果包括切除局灶性腺肌病病变和妊娠前用GnRH激动剂预处理。结论:子宫腺肌病对产科结局有多因素影响;治疗方式确实存在,以提高受孕和保持妊娠的机会。然而,需要更多的研究来进一步证实治疗方式与改善妊娠的关系;同时也阐明了子宫腺肌病对不孕症的影响
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引用次数: 0
Clinical profile, surgical approach and outcomes of children with Fontan procedure in a country with limited resources 在一个资源有限的国家,儿童Fontan手术的临床概况、手术方法和结果
Pub Date : 2023-07-05 DOI: 10.15406/ipcb.2023.09.00286
A. Maloku, R. Bejiqi, Aferdita Mustafa, N. Zeka, R. Bejiqi
Background: For more than five decades, the Fontan or Fontan-Kreutzer procedure has been the mainstay treatment for congenital heart disease with a single functioning ventricle. Data concerning epidemiological profiles are poor, especially in countries with limited resources. Here, we present the cases of children with complex congenital heart disease (CHD) born in Kosovo who underwent some forms of Fontan palliation measuring the renal resistive index (RRI) to assess ventricular function and renal complications after the Fontan procedure. Objectives: This study aimed to describe the primary pathology, age, place of surgical intervention, and outcomes of children in Kosovo, a country with limited resources, who underwent the Fontan procedure in different countries. Results: from January 2018 to December 2021, 40 patients (28 male and 12 female) aged 6 to 19 years (mean 6.03 years) after a total Cavo-pulmonary connection were examined for renal insufficiency and thrombotic complications. The renal resistive index (RRI) and hematological parameters were analyzed as criteria for possible early and late complications. Two patients only developed complications in the cohort group. In both cases, the second and third stages of surgery were performed late, at the ages of 12 and 9 years. The first patient manifested a severe form of protein-lousing enteropathy, renal insufficiency, and plastic bronchitis, while the other patient presented with initial signs of protein-lousing enteropathy and recurrent ventricular tachycardia. Conclusion: Survival after Fontan operation in our group was excellent. Survival is closely dependent on the primary diagnosis, associated anomalies, age at palliation, and place of surgery
背景:50多年来,Fontan或Fontan- kreutzer手术一直是单侧心室先天性心脏病的主要治疗方法。关于流行病学概况的数据很少,特别是在资源有限的国家。在这里,我们介绍了在科索沃出生的患有复杂先天性心脏病(CHD)的儿童的病例,他们接受了一些形式的Fontan姑息治疗,测量肾抵抗指数(RRI)来评估Fontan手术后的心室功能和肾脏并发症。目的:本研究旨在描述科索沃(一个资源有限的国家)儿童在不同国家接受Fontan手术的主要病理、年龄、手术地点和结果。结果:2018年1月至2021年12月,40例患者(男28例,女12例),年龄6至19岁(平均6.03岁),经全腔肺连接后检查肾功能不全和血栓性并发症。分析肾阻力指数(RRI)和血液学参数作为早期和晚期可能出现并发症的标准。队列组中仅有2例患者出现并发症。在这两种情况下,手术的第二和第三阶段都是在12岁和9岁时进行的。第1例患者表现为严重的蛋白脱落性肠病、肾功能不全和可塑性支气管炎,而另1例患者表现为蛋白脱落性肠病和复发性室性心动过速的初始症状。结论:本组丰坦手术后生存率高。生存密切依赖于最初的诊断、相关的异常、姑息时的年龄和手术地点
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引用次数: 0
Current ethical dilemmas in obstetrical practice and some suggestions regarding how to resolve them 当前产科实践中的伦理困境及解决建议
Pub Date : 2023-06-27 DOI: 10.15406/ipcb.2023.09.00285
E. Howe
As new ethical conundrums arise in obstetrics, providers are ever-increasingly challenged with having to know the best means of resolving these problems since Roe v. Wade was overturned. New ethical challenges have arisen, for example, as to how clinicians should respond when patients would need to travel to another state to get an abortion. A second illustrative example involves minors. In some states in the U.S., these patients legally can request permission from a judge to have an abortion without their having to tell their parents. If these adolescents don’t know this, providers must decide whether they should take initiative to inform them of this possibility and if so, when. This question is still more challenging when these patients, too, live in a state that does not allow them the option of abortion. Providers confronted with ethical problems such as those above may struggle to determine how to best resolve these dilemmas. This piece will review some key and especially more subtle, ethical approaches that providers may face to better resolve them. Initially, I will highlight approaches to resolving ethical outcomes that providers may not know or may overlook. These will include how providers may best apply both core ethical principles and an additional, adjunctive framework, known as the care perspective or ethics of care. I will provide specific examples to illustrate each
随着产科出现新的伦理难题,自从罗伊诉韦德案被推翻以来,医疗服务提供者面临着越来越多的挑战,他们必须知道解决这些问题的最佳方法。新的伦理挑战已经出现,例如,当病人需要前往另一个州堕胎时,临床医生应该如何应对。第二个说明性的例子涉及未成年人。在美国的一些州,这些患者可以合法地向法官申请堕胎许可,而不必告诉父母。如果这些青少年不知道这一点,提供者必须决定他们是否应该主动告知他们这种可能性,如果是,何时告知。当这些病人也生活在一个不允许他们选择堕胎的州时,这个问题就更具挑战性了。供应商面临的道德问题,如上述可能难以确定如何最好地解决这些困境。这篇文章将回顾一些关键的,特别是更微妙的,道德的方法,供应商可能面临更好地解决这些问题。首先,我将强调解决供应商可能不知道或可能忽视的道德结果的方法。这些将包括提供者如何最好地应用核心伦理原则和附加的辅助框架,即护理观点或护理伦理。我将提供具体的示例来说明每一个
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引用次数: 0
Family centered early childhood development: a case of Rongo Subcounty, Migori Kenya 以家庭为中心的儿童早期发展:以肯尼亚米戈里县朗戈县为例
Pub Date : 2023-06-15 DOI: 10.15406/ipcb.2023.09.00284
Teresa Mwoma, Phillip Omondi, S. Mudhune, Erick Auko, Mercy Ongono, L. Njoki, Hellen Kerubo, Jane Wamae, Lou L. Goore, Stephen Okong, Mumma Edelquinn, Ash Rogers
The early childhood period from conception to three years is critical in a child’s health, well-being and brain development. Proper care and stimulation therefore, contributes to a child’s productivity that lasts through childhood to adulthood. It is estimated that globally, 43% of children under five years are at risk of failing to achieve their developmental potential. In Sub-Saharan Africa (SSA), this portion is approximately 66%. The Kenya Demographic Health Survey (KDHS) 2022 revealed that 21% of children in Kenya aged 24-59 months are not developmentally on track. A higher percentage of children in urban (87%) than rural (73%) areas are developmentally on track. The percentage of children developmentally on track increases with increasing mothers’ education from 51% among children whose mothers have no education to 91% among those whose mothers have more than secondary education. The percentage of children developmentally on track also increases with increasing household wealth from 61% among children in the lowest wealth quantile to 93% among those in the highest quantile. This therefore calls for parenting/caregiving programmes that targets parents/caregivers from the lowest household wealth index as well as those with mothers having lowest education levels. Lwala Community Alliance with funding from Comic Relief piloted a Family Centered earlcy childhood development (ECD) program in North Kamagambo ward, Rongo Subcounty, Migori County Kenya in 2021. The long-term goal of the program was to ensure that children under the age of five years reach their developmental potential in a nurturing care environment supported by responsive and sensitive men and women caregivers. The community health volunteers (CHW) through household visitation, group parenting sessions, and facility-based education sessions implemented the program. This was meant to improve outcomes across child health, caregiver support, health worker capacity, and the enabling environment. Building on the success of the pilot, an evaluation and baseline study was conducted to explore the outcome of the pilot intervention and to upscale the program into three new wards in Rongo Subcounty. In order to accomplish this, baseline data was collected in three areas (East Kamagambo, Central Kamagambo and South Kamagambo) where scaling up of the program will happen before commencement of intervention activities. Findings revealed that the primary caregiver plays a critical role in caring for young children during the day by providing care for children’s basic needs. It was noted that households in North Kamagambo introduced complementary foods after the recommended six months as compared to the other three wards where other foods were introduced to babies before six months are over. Findings further revealed that there was a significant increase in knowledge especially on child rights and abuse from baseline, midline and end line in North Kamagambo with 86%, 90% and 91%, respectively. Car
从怀孕到三岁的幼儿期对儿童的健康、福祉和大脑发育至关重要。因此,适当的照顾和刺激有助于儿童从童年持续到成年的生产力。据估计,全球有43%的五岁以下儿童面临无法实现其发展潜力的风险。在撒哈拉以南非洲(SSA),这一比例约为66%。2022年肯尼亚人口健康调查(KDHS)显示,在肯尼亚,年龄在24-59个月的儿童中,有21%没有走上发育轨道。城市儿童(87%)比农村儿童(73%)发展正常的比例更高。随着母亲受教育程度的提高,发育正常的儿童比例也随之增加,母亲未受教育的儿童比例为51%,母亲受过中等以上教育的儿童比例为91%。随着家庭财富的增加,发育正常的儿童比例也在增加,从最低财富分位数儿童的61%上升到最高财富分位数儿童的93%。因此,需要针对家庭财富指数最低的父母/照料者以及母亲受教育程度最低的父母/照料者制定养育/照料规划。2021年,在Comic Relief的资助下,Lwala社区联盟在肯尼亚米戈里县朗戈县北卡马甘博区试点了一个以家庭为中心的儿童早期发展(ECD)项目。该方案的长期目标是确保五岁以下儿童在由反应灵敏和敏感的男女照料者支持的养育照料环境中实现其发展潜力。社区卫生志愿者(CHW)通过家庭访问、小组育儿会议和以设施为基础的教育会议来实施该计划。这是为了改善儿童健康、照料者支持、卫生工作者能力和有利环境方面的结果。在试点成功的基础上,开展了一项评估和基线研究,以探索试点干预措施的结果,并将该项目扩展到朗戈县的三个新病房。为了实现这一目标,在三个地区(东卡马甘博、中卡马甘博和南卡马甘博)收集了基线数据,这些地区将在开始干预活动之前扩大方案的规模。调查结果显示,主要照顾者通过提供儿童的基本需求,在日间照顾幼儿方面发挥着关键作用。有人指出,北卡马甘博的家庭在建议的6个月后才引入辅食,而其他三个区在婴儿6个月前就引入了其他食物。调查结果进一步显示,北卡马甘博的基线、中线和终点的儿童权利和虐待知识显著增加,分别为86%、90%和91%。在这四个地方,看护人识别受虐儿童迹象的能力似乎都很好。与其他病房的护理人员相比,北卡马甘博的护理人员为其儿童提供了更高水平的养育护理
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引用次数: 0
Monstrous myoma previa and pregnancy 畸形前肌瘤与妊娠
Pub Date : 2023-05-22 DOI: 10.15406/ipcb.2023.09.00283
Yassine Outifa, Kenza Benchaaboune, A. Melhouf, Fatimazahrae Alaoui Fdili
{"title":"Monstrous myoma previa and pregnancy","authors":"Yassine Outifa, Kenza Benchaaboune, A. Melhouf, Fatimazahrae Alaoui Fdili","doi":"10.15406/ipcb.2023.09.00283","DOIUrl":"https://doi.org/10.15406/ipcb.2023.09.00283","url":null,"abstract":"","PeriodicalId":211817,"journal":{"name":"International Journal of Pregnancy & Child Birth","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134019934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Severe fetal anaemia due to metastatic antenatal choriocarcinoma 转移性胎儿绒毛膜癌引起的严重胎儿贫血
Pub Date : 2023-05-11 DOI: 10.15406/ipcb.2023.09.00282
Mohamed Ali Mustafa Khalil, Shamma AlInizi, Angharad Mai James
{"title":"Severe fetal anaemia due to metastatic antenatal choriocarcinoma","authors":"Mohamed Ali Mustafa Khalil, Shamma AlInizi, Angharad Mai James","doi":"10.15406/ipcb.2023.09.00282","DOIUrl":"https://doi.org/10.15406/ipcb.2023.09.00282","url":null,"abstract":"","PeriodicalId":211817,"journal":{"name":"International Journal of Pregnancy & Child Birth","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-05-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130739330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Umbilical cord hematoma – a successful case of a non-frequent acute fetal distress etiology 脐带血肿-一个不常见的急性胎儿窘迫病因的成功案例
Pub Date : 2023-04-21 DOI: 10.15406/ipcb.2023.09.00281
A. C. Ferreira, Inês Pereira Soares, Sara Sousa, Patrícia Ferreira, Daniela Ramos
Background: Umbilical cord hematoma is a rare condition, with mortality rates up to 50%. Most of the literature describes cases with poor prognosis. Case description: In this case, a healthy pregnant woman in labor presented some variable decelerations on the fetal heart rate monitoring ending in sustained bradycardia. An emergency C-section was performed, giving birth to a 40-weeks male newborn with an umbilical cord hematoma until placental insertion and, slightly stump bleeding. Newborn got pale and, had respiratory distress in the first hour of life. He presented transient metabolic acidosis with hyperlacticaemia and, anemia. There was a favorable evolution with supportive therapy. No risk factor was identified and, histological examination confirmed the diagnosis. The patient remained asymptomatic and, presented normal neurodevelopment during follow-up. Discussion: This case report aims to demonstrate a good outcome when a high level of suspicion is taken and immediate medical and, surgical interventions are performed
背景:脐带血肿是一种罕见的疾病,死亡率高达50%。大多数文献描述的病例预后较差。病例描述:本病例中,一名健康孕妇在分娩过程中,胎儿心率监测出现了一些可变减速,最终出现了持续性心动过缓。进行了紧急剖腹产,生下了一个40周的男婴,脐带血肿直到胎盘插入,并有轻微的残肢出血。新生儿面色苍白,在出生的第一个小时就出现呼吸窘迫。他表现为一过性代谢性酸中毒伴高乳酸血症和贫血。支持性治疗有良好的进展。没有发现危险因素,组织学检查证实了诊断。患者无症状,随访期间神经发育正常。讨论:本病例报告旨在证明,当高度怀疑并立即进行医疗和手术干预时,结果良好
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引用次数: 0
Nutritional status of children and its association with parental smoking in Uttar Pradesh 北方邦儿童营养状况及其与父母吸烟的关系
Pub Date : 2023-04-11 DOI: 10.15406/ipcb.2023.09.00280
Mohd Al Uzair, Sharmin Ansar, Mohd Salman Kavish, Akbar Ghani, Ajit Kumar Jaiswal
Background: Exposure to passive smoke due to smoking affects foetus and children. Previous studies have linked this exposure to an increased risk of malnutrition, infant death, respiratory illness and other disorders among infants. The investigation was made to explore the association between stunting of growth and children under 5 years of age who are underweight and are exposed to parental smoking. Exposure to second hand or passive smoke, has adverse impact during pregnancy on the mother as well as the baby. This could include cases of premature birth, birth defects or infant death and respiratory problems among the children. This study discusses the current evidence regarding the effects of parental smoking on their children's health in Uttar Pradesh. Methods: Parental smoking, and child anthropometry (undernutrition) were examined in a stratified, two-stage cluster sample of 232,920 kids under the age of 5 from National Family Health Survey (2015-16), out of which 35,766 were selected in the study from Uttar Pradesh. Prominent outcomes were that of stunting of growth and a proclivity in children towards remaining underweight. Results: Prevalence of parental smoking was found in 38.28% of households in Uttar Pradesh. Using the new World Health Organization child growth standards, prevalence of stunted growth, and underweight children was found in 39.34%, 31.15% of the households respectively. After adjusting for potential confounders parental (couple) smoking was associated with increased risk of stunting (AOR 1.17, 95%CI 1.0-1.37, p<0.05), and underweight children (AOR 1.09, 95%CI 0.93-1.28). Conclusion: In Uttar Pradesh, parental smoking is associated with birth factors nutritional status and may divert household funds from food and other necessities towards smoking habits. Parental smoking was also associated with increased risk of stunting of growth and underweight children in the children. The result also suggests that strategies to prevent passive smoke exposure in young children to minimize tobacco smoke exposure are an urgent public and child health priority
背景:吸烟导致的被动吸烟会影响胎儿和儿童。以前的研究已经将这种接触与婴儿营养不良、婴儿死亡、呼吸系统疾病和其他疾病的风险增加联系起来。这项调查的目的是探讨发育迟缓与5岁以下儿童体重过轻和父母吸烟之间的关系。在怀孕期间接触二手烟或被动烟对母亲和婴儿都有不利影响。这可能包括早产、出生缺陷或婴儿死亡以及儿童的呼吸问题。本研究讨论了目前关于父母吸烟对北方邦儿童健康影响的证据。方法:对来自全国家庭健康调查(2015-16)的232,920名5岁以下儿童进行分层两阶段整群抽样,对父母吸烟和儿童人体测量(营养不良)进行调查,其中35,766名儿童来自北方邦。突出的结果是发育迟缓和儿童倾向于保持体重不足。结果:北方邦38.28%的家庭存在父母吸烟。使用新的世界卫生组织儿童生长标准,生长发育迟缓和体重不足的发生率分别为39.34%和31.15%。在调整了潜在的混杂因素后,父母(夫妇)吸烟与发育迟缓(AOR 1.17, 95%CI 1.0-1.37, p<0.05)和体重不足儿童(AOR 1.09, 95%CI 0.93-1.28)的风险增加相关。结论:在北方邦,父母吸烟与出生因素营养状况有关,并可能将家庭资金从食品和其他必需品转移到吸烟习惯上。父母吸烟也与儿童发育迟缓和体重不足的风险增加有关。结果还表明,预防幼儿被动吸烟以尽量减少烟草烟雾暴露的策略是公共和儿童健康的紧急优先事项
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引用次数: 0
Humanized childbirth, a link to humanitarian treatment, synonymous with quality obstetric care at the 1st level of care in Mexico 人性化分娩,与人道主义治疗相联系,是墨西哥一级护理质量产科护理的代名词
Pub Date : 2023-04-10 DOI: 10.15406/ipcb.2023.09.00279
Velázquez Perez Guadalupe, Gilberto Espinoza Anrubio, Ivonne Analí Roy Garcia, R. Rivas Ruiz, Angélica Elizabeth Garcia Perez, Ruth Ramirez Fuentevilla
Bringing a new being into the world is a relevant and transcendent event in a woman's life, which will be marked for the rest of her life. However, it can be experienced as a painful and traumatic experience. Objective: Analyze the most effective actions to improve the quality of humanized delivery care in low-risk pregnant women in hospitals of 1st and 2nd level of care. Methodology: A narrative review was carried out that includes a critical, reflective analysis of humanized childbirth. The review of: original articles, revision of doctoral and master's theses was carried out. The adopted search strategy included keywords or descriptors, connected by means of the boolean operator and. The keywords used were "humanization", "delivery", "human needs", and breastfeeding, these being identified through Medical Subject Headings 2020 Serach MeSH, in this way the referred articles, descriptors in language were used for the search. Spanish and English. The search was carried out in various databases. Information collection time 6 months, (August 2022 to February 2023). Results: The review allowed us to analyze in a standardized way the historical evolution of humanized childbirth and the transcultural significance that it represents and to a large extent to be carried out, and how it will reinforce the multidisciplinary health team that cares for women in this process, to offer different strategies. to be this stage an unforgettable and humanistic experience that you will never forget. Conclusions: Humanized childbirth is translated into a birth with a humanistic character which includes: the prenatal stage, labor, delivery and the puerperium, where the protagonists are the pregnant woman and the newborn, receiving dignified, free treatment. of violence and based on human and ethical rights
在一个女人的生命中,生下一个新生命是一件重要而卓越的事情,这将在她的余生中留下印记。然而,它可能是一种痛苦和创伤的经历。目的:分析提高一、二级医院低危孕妇人性化分娩护理质量的最有效措施。方法:进行了一个叙述性的审查,其中包括一个关键的,反思的分析人性化分娩。进行了论文评审,博士、硕士论文的修改。所采用的搜索策略包括关键字或描述符,通过布尔运算符和连接。使用的关键词是“人性化”、“分娩”、“人类需求”和母乳喂养,这些都是通过医学主题标题2020搜索网格确定的,这样就可以使用参考文章、语言描述符进行搜索。西班牙语和英语。搜索是在不同的数据库中进行的。信息收集时间6个月,(2022年8月至2023年2月)。结果:该综述使我们能够以标准化的方式分析人性化分娩的历史演变及其在很大程度上所代表的跨文化意义,以及它将如何加强在此过程中照顾妇女的多学科卫生团队,并提供不同的策略。让这个舞台成为你永远难忘的人文体验。结论:人性化分娩被翻译成一种具有人文性格的分娩,它包括:产前、分娩、分娩和产褥期,其中的主角是孕妇和新生儿,他们接受有尊严的、免费的治疗。暴力和基于人权和道德权利
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引用次数: 0
Isolated hepatomegaly became Her’s – a case report 孤立性肝肿大致Her 's 1例报告
Pub Date : 2023-03-28 DOI: 10.15406/ipcb.2023.09.00278
P. Jose, Solai Ganesh, A. A, Peter Prasanth Kumar Kommu
Glycogen storage diseases (GSDs) are a group of inherited metabolic disorders caused by mutations in one of the several enzymes involved in glycogen synthesis or breakdown. GSD VI is caused by mutations of PYGL gene encoding hepatic glycogen phosphorylase on chromosome 14q22.1, which has a major role in glycogen metabolism. We report a case of 2year old girl child presented with complaints of slowly progressive abdominal distention of one year duration. Abdominal examination revealed hepatomegaly (liver span 9cm). Lab investigations revealed mild anemia, hypertriglyceridemia and normal liver function. Liver biopsy showed marked elevation of glycogen content in liver with structurally normal glycogen which was consistent with GSD. Sanger sequencing done to identify the subtype of GSD showed PYGL gene mutation, pathogenic variant of type-VIGSD. In the case of isolated hepatomegaly, a glycogen storage disease must be considered in the differential diagnosis and Sanger sequencing must be done for confirmation and prognostication
糖原储存病(GSDs)是一组遗传性代谢性疾病,由参与糖原合成或分解的几种酶中的一种突变引起。GSD VI是由染色体14q22.1上编码肝糖原磷酸化酶的PYGL基因突变引起的,该基因在糖原代谢中起主要作用。我们报告一个2岁女童的情况下提出的投诉缓慢进行性腹胀持续一年。腹部检查显示肝脏肿大(肝跨度9cm)。实验室检查显示轻度贫血,高甘油三酯血症和肝功能正常。肝活检显示肝脏糖原含量明显升高,糖原结构正常,与GSD一致。Sanger测序鉴定GSD亚型显示PYGL基因突变,为致病性变异体- vigsd型。在孤立性肝肿大的情况下,鉴别诊断时必须考虑糖原蓄积性疾病,必须进行Sanger测序以进行确认和预测
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引用次数: 0
期刊
International Journal of Pregnancy &amp; Child Birth
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