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The Role of Zinc in Acute Bronchiolitis in children less than 2 years old 锌在2岁以下儿童急性细支气管炎中的作用
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68309
Rumy Tabrez Hyder, Shafi Ahmed, B. Yasmeen
Background: Bronchiolitis is an acute respiratory illness which is the leading cause of respiratory distress in infancy and early childhood with its greatest morbidity in infants. Although zinc supplementation is effective in both preventing and treating pneumonia but its role in acute bronchiolitis has rarely been examined. Objective: To assess the effect of zinc sulfate to improve the clinical manifestations of acute bronchiolitis (Severe cases) in children less than 2 years of age. Materials and Methods: This was a double blind randomized clinical trial on 70 patients aged 2 to 23 months admitted in the Pediatric ward of Khwaja Yunus Ali Medical College and Hospital from September 2019 to September 2020 with the diagnosis of acute bronchiolitis. Patients were randomly divided equally into 2 groups: a case group was assigned oral zinc sulfate and the control group received placebo. Results: Mean age of case group was 174.63±98.7 days and control group 176.86±97.43 days ( p=0.924) with male predominance in both groups. Symptoms and signs of acute bronchiolitis (Severe cases) were compared between the 2 groups during admission and then at 24, 48 and 72 hours after initiation of treatment. No statistically significant differences were observed between the 2 groups in improvement of symptoms and signs including fever, fast breathing, chest indrawing and wheezing on 48 and 72 hours after treatment with zinc sulfate ( p>0.05). The duration of hospitalization was 4.2±2.6 days in the case group and 4.4±2.2 days in the control group and this difference was not significant. Conclusion: The present study showed that zinc sulfate has no benefit in improving the clinical manifestations of acute bronchiolitis. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 535-538
背景:毛细支气管炎是一种急性呼吸道疾病,是婴幼儿呼吸窘迫的主要原因,以婴幼儿发病率最高。虽然补充锌对预防和治疗肺炎都有效,但其在急性细支气管炎中的作用却很少被研究。目的:探讨硫酸锌对2岁以下儿童急性细支气管炎(重症)临床表现的改善作用。材料与方法:这是一项双盲随机临床试验,研究对象为2019年9月至2020年9月在赫瓦贾尤努斯阿里医学院附属医院儿科病房收治的70例2至23个月的急性细支气管炎患者。患者随机分为两组:病例组给予口服硫酸锌,对照组给予安慰剂。结果:病例组平均年龄为174.63±98.7 d,对照组平均年龄为176.86±97.43 d (p=0.924),两组均以男性为主。比较两组患者入院时及治疗开始后24、48、72小时急性细支气管炎(重症)的症状和体征。两组患者在硫酸锌治疗后48、72小时发热、呼吸急促、胸闷、喘息等症状体征改善情况比较,差异均无统计学意义(p>0.05)。病例组住院时间为4.2±2.6 d,对照组为4.4±2.2 d,差异无统计学意义。结论:本研究表明硫酸锌对改善急性细支气管炎临床表现无明显作用。《北方国际医学院学报》12卷第2期,2021年1月,第535-538页
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引用次数: 0
Telemedicine - the virtual healthcare 远程医疗——虚拟医疗
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68307
B. Yasmeen
Abstract not available Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 523-525
《北方国际医学院学报》2021年1月第12卷第2期,523-525页
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引用次数: 0
Risk factors of persistent diarrhea in children below 5 years of age 5岁以下儿童持续性腹泻的危险因素
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68310
Madhabi Baidya, Salauddin Mahmud, U. Ghosh, D. Begum, Syed Shafi Ahmed
Background: Persistent diarrhea is a known cause of mortality, morbidity, and malnutrition in developing countries. With recent improvement of rehydration therapy death due to acute diarrhea has been reduced. Though persistent diarrhea accounts for 2-20% of total diarrhea cases, it accounts for 23-62% of all diarrhea related deaths. Objective: The study was done to identify the risk factors associated with persistent diarrhea in children below 5 years of age. Methods: This prospective observational study was conducted in the department of Pediatric Gastroenterology, Hepatology and Nutrition of Dhaka Shishu hospital from July 2019 to June 2020. It included 50 children with persistent diarrhea age between 1 month to <5 years. Detailed history, examination and appropriate investigations were done for all children. Crude odd ratio was calculated for each risk factor by univariate analysis and adjusted odds ratio was calculated by multivariate logistic regression. Results: Most of the children (52%) were under the age of 6 months to 1 year. Male (84%) were more affected than female (16%). Acute malnutrition was observed in 44% cases; among them severe acute malnutrition was in 40% cases. Unsafe drinking water, severe acute malnutrition, diarrhea within past 2 months, bottle feeding, persistent of dehydration more than 24 hours, feeding of cow’s milk, lack of exclusive breast feeding, prior antibiotic used, UTI, LRTI were statistically significant risk factors by univariate analysis. Unsafe drinking water (OR-1.6; 95% CI- 0.27- 2.78), severe acute malnutrition (OR-1.92; 95% CI-0.64 - 5.72), Feeding of cow’s milk (OR-3.90; 95% CI- 1.21- 12.49), lack of exclusive breastfeeding (OR-5.77; 95% CI- 1.44- 23.10), irrational use of antibiotics (OR-3.37; 95% CI- 1.01- 11.38), LRTI (OR-1.16; 95% CI- 0.49- 1.53) were found to be independent risk factors by multivariate logistic regression analysis. Conclusions: The results of this study concluded that use of unsafe drinking water, severe acute malnutrition, feeding of cow’s milk, lack of exclusive breastfeeding, irrational use of antibiotics are significant risk factors for the incidence of diarrhea in the present study population. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 539-543
背景:在发展中国家,持续性腹泻是导致死亡率、发病率和营养不良的已知原因。随着近年来补液疗法的改进,急性腹泻导致的死亡已经减少。虽然持续性腹泻占腹泻病例总数的2-20%,但它占所有腹泻相关死亡的23-62%。目的:本研究旨在确定5岁以下儿童持续性腹泻的相关危险因素。方法:本前瞻性观察研究于2019年7月至2020年6月在达卡石树医院儿科消化、肝脏和营养科进行。该研究包括50名持续腹泻的儿童,年龄在1个月至5岁以下。对所有儿童进行了详细的病史、检查和适当的调查。单因素分析计算各危险因素的粗奇数比,多因素logistic回归计算校正优势比。结果:年龄在6个月~ 1岁的患儿占52%。男性(84%)比女性(16%)更受影响。急性营养不良占44%;其中严重急性营养不良占40%。单因素分析显示,饮用水不安全、严重急性营养不良、近2个月内腹泻、奶瓶喂养、持续脱水24小时以上、牛奶喂养、缺乏纯母乳喂养、既往使用抗生素、尿路感染、下呼吸道感染是有统计学意义的危险因素。不安全饮用水(OR-1.6;95% CI- 0.27- 2.78),严重急性营养不良(OR-1.92;95% CI-0.64 - 5.72),牛奶喂养(OR-3.90;95% CI- 1.21- 12.49),缺乏纯母乳喂养(OR-5.77;95% CI- 1.44- 23.10),不合理使用抗生素(OR-3.37;95% ci - 1.01- 11.38), lrti (or-1.16;多因素logistic回归分析发现95% CI(0.49 ~ 1.53)为独立危险因素。结论:使用不安全饮用水、严重急性营养不良、食用牛奶、缺乏纯母乳喂养、不合理使用抗生素是本研究人群发生腹泻的重要危险因素。《北方国际医学院学报》12卷第2期,2021年1月,第539-543页
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引用次数: 0
Breast feeding Status of Infants Born in a Baby Friendly Hospital Up to 180 days of life 在爱婴医院出生的婴儿直至生命180天的母乳喂养状况
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68308
Nanda Lal Das, Md Shafiul Alam, Md Fazlul Kader Khan, K. T. Islam, Md. Shahadat Hossain Khan, Md Shaiful Azam, R. Rana, Farzana Afrooz, Sajani Islam, Anwesa Sarker, S. Khatun
Background : Initiation of breast-feeding (BF) within 1 hour after birth has been associated with reduced neonatal mortality. Baby Friendly Hospital Initiative has profound effects on appropriate practice of BF and thus helps to reduce infant mortality and morbidity. Objective : This study was conducted in the Department of Gynae and Obstetrics and Department of Paediatrics of Shaheed Suhrawardy Medical College Hospital (ShMCH), Dhaka, to observe the rate of exclusive breast feeding and home-based compliance. Methodology : Hundred (100) term babies of normal birth weight irrespective of sex, born in ShSMCH by normal vaginal delivery (NVD) or caesarian section were included. Data was collected from the mother with a pretested questionnaire at the postnatal period, at 6th week, 14th week and 6th month (180 days) of age. Result : The mean age of studied newborn (100) was 20±19.5 hours. All the infants started breast feeding after birth and among them 56% initiated with in 1 hour. Difficulty in breast feeding was found in 36(36.0%) infants, which was resolved mostly (94%) by nurse. Exclusive breastfeeding (EBF) was found in 100% of infants during discharge from hospital. During 1st and 2nd follow up we found 95 (95.0%) and 89 (89.0%) infants respectively were exclusively breastfed. In 3rd follow up at completed 6th month (180 days) 78 (78.0%) infants were exclusively breast fed. In our study 5 (5%) and 6 (6%) infants received supplementary food in 1st follow up and in 2nd follow up respectively. In the last follow up the number were 11 (11%) and the total number was 22 (22%). Conclusion : Exclusive breast feeding up to completion of 6 months (180 days) was found in 78.0% of infants. Maternal and relative’s misperception of baby’s crying due to insufficient breast milk and joining to job outside home were the causes of introduction of formula milk in the 1st, 2nd and 3rd visit respectively. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 530-534
背景:出生后1小时内开始母乳喂养(BF)与新生儿死亡率降低有关。爱婴医院倡议对适当的BF做法产生了深远的影响,从而有助于降低婴儿死亡率和发病率。目的:本研究在达卡沙希德苏赫拉瓦迪医学院附属医院(ShMCH)的妇产科和儿科进行,观察纯母乳喂养率和家庭依从性。方法:纳入100例正常出生体重的足月婴儿,不论性别,在ShSMCH正常阴道分娩(NVD)或剖腹产出生。在产后、6周、14周和6个月(180天)时通过预测问卷从母亲处收集数据。结果:新生儿平均年龄(100)为20±19.5小时。所有婴儿在出生后开始母乳喂养,其中56%在1小时内开始母乳喂养。36例(36.0%)患儿出现母乳喂养困难,多数(94%)患儿由护士解决。100%的婴儿出院时采用纯母乳喂养(EBF)。在第一次和第二次随访中,我们分别发现95例(95.0%)和89例(89.0%)的婴儿被纯母乳喂养。在第3次随访中,在完成第6个月(180天)时,78例(78.0%)婴儿接受纯母乳喂养。在我们的研究中,第1次随访中有5例(5%)和第2次随访中有6例(6%)婴儿接受补充食物。末次随访11例(11%),总22例(22%)。结论:纯母乳喂养至6个月(180天)的婴儿占78.0%。在第1次、第2次和第3次访问中,母亲和亲属因母乳不足而对婴儿啼哭产生误解和外出工作分别是引入配方奶的原因。《北方国际医学院学报》12卷第2期,2021年1月,530-534页
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引用次数: 0
Presenting clinical features of COVID-19 in neonatal age group 新生儿年龄组COVID-19的临床特征
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68306
Maksudur Rahman, N. Jahan, M. Hoque, K. Ghosh
Background : In December, 2019 a cluster of pneumonia cases spread in Wuhan City, Hubei Province, China caused by a novel corona virus (named as “2019-nCoV”). Although it is thought to be a disease of adult but it can occur in children and neonates also. Objective : The aim of this study was to see the incidence of Neonatal COVID-19 and its mode of presentation in neonatal age group. Methods : This cross sectional study was done in Dhaka Shishu (Children) Hospital from April, 2020 to October, 2020. All suspected neonates for COVID-19, RT PCR were done from nasopharyngeal swab. Suspecting sign were fever, respiratory difficulty, reluctant to feed, neonate not responding to conventional treatment, or referred from endemic area or having any household contact. Routinely RT PCR was done preoperatively who needed surgery. Then all the data were collected and analyzed using Statistical Package of Social Science (SPSS), version 26. Results : Total 5521 neonates were admitted and 299 neonates were suspected for COVID-19 infection during this study periods and undergone RT PCR, out of which 47 cases were test positive. The incidence of COVID-19 cases was 0.85% among hospitalized neonates. Thirty one were male and 16 were female. Male female ratio was 1.9:1. Thirtythree cases admitted in medicine and 14 cases in surgery division. Twenty three (49%) cases presented symptoms after 7 days of age and 13(28%) cases got admitted within 3 days, among them 2 cases were admitted at 24 hours of age with symptoms. Respiratory difficulty (25.5%), convulsion (19.1%), fever (17%) and reluctant to feed (17%) were common presenting symptoms. The associated diagnosis with COVI-19 cases were mostly perinatal asphyxia, septicemia and pneumonia. Regarding chest X-ray, one neonate had patchy opacities in right lower lobe, another had bilateral ground-glass opacity and in case of third one few patchy opacities was found in the right perihilar region. Out of 47 positive cases 23 patients could discharge after improvement, 14 patients referred to COVID specialized hospital, 2 cases went as LAMA (Leave against medical advice) and 8 patients died at our hospital. Conclusion : In this Study the incidence of COVID-19 in newborn was 0.85% among hospitalized newborn. The common clinical features were respiratory difficulty, convulsion, fever and reluctant to feed. It could not be differentiated whether these clinical features were due to COVID-19 or associated diseases. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 526-529
背景:2019年12月,中国湖北省武汉市发生由新型冠状病毒(简称“2019- ncov”)引起的聚集性肺炎病例。虽然它被认为是一种成人疾病,但它也可以发生在儿童和新生儿。目的:了解新生儿COVID-19的发病率及其在新生儿年龄组的表现方式。方法:本横断面研究于2020年4月至2020年10月在达卡Shishu(儿童)医院进行。所有疑似感染COVID-19的新生儿均采用鼻咽拭子RT - PCR检测。可疑体征为发热、呼吸困难、不愿进食、新生儿对常规治疗无反应、来自流行地区或有任何家庭接触者。术前行常规RT - PCR。然后使用社会科学统计软件包(SPSS)第26版对所有数据进行收集和分析。结果:本研究期间共收治新生儿5521例,其中疑似COVID-19感染患儿299例,RT - PCR检测阳性47例。住院新生儿新冠肺炎病例发生率为0.85%。31名男性,16名女性。男女比例为1.9:1。内科33例,外科14例。23例(49%)在7天后出现症状,13例(28%)在3天内入院,其中2例在24小时出现症状入院。呼吸困难(25.5%)、惊厥(19.1%)、发热(17%)和不愿进食(17%)是常见的症状。与covid -19病例相关的诊断多为围产期窒息、败血症和肺炎。胸部x线检查,1例新生儿右下肺叶斑片状影,1例患儿双侧磨玻璃影,3例患儿右侧肺门周围可见少量斑片状影。47例阳性病例中,23例好转出院,14例转诊至专科医院,2例病休,8例在我院死亡。结论:本组住院新生儿新冠肺炎发病率为0.85%。常见临床特征为呼吸困难、惊厥、发热、不愿进食。无法区分这些临床特征是由COVID-19还是相关疾病引起的。《北方国际医学院学报》12卷,2021年1月第2期,526-529页
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引用次数: 0
COVID-19 In Children 儿童感染COVID-19
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68312
Mirza Md Ziaul Islam, B H Nazma Yasmeen
Diagnosis of SARS-CoV-2 infection is based on detection of viral RNA by RT-PCR of nasopharyngeal or oropharyngeal swabs. Consider alternative diagnoses in children who are unwell, even in the presence of a positive SARS-CoV-2 PCR result. Children with mild to moderate Covid19 do not routinely need admission or investigations such as blood tests and radiology, unless otherwise clinically indicated. Children with severe or critical disease as a minimum should have the following investigations: Blood cultures, Full Blood Count (FBC), Coagulation profile, D-dimer, Urea and Electrolytes, LFT, CRP, Troponin, Ferritin, Lactate dehydrogenase (LDH) and Blood Gas Analysis. Remdesivir may be considered for children > 12 years and >40kg with COVID-19 requiring supplemental oxygen. Consider chest x-ray in children who do not follow the expected clinical course, for example, those still requiring oxygen on day three of admission, those with worsening hypoxemia or those requiring respiratory support. Decision to escalate respiratory support to Non-Invasive Ventilation (NIV) should be made by a senior member of the pediatric team, in discussion with critical care. For children with clinical findings consistent with the Pediatric Inflammatory Multisystem Syndrome temporally associated with SARS-CoV-2 (PIMS-TS) should be managed as per specialized guidelines. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 548-554
SARS-CoV-2感染的诊断是基于鼻咽或口咽拭子RT-PCR检测病毒RNA。即使存在SARS-CoV-2 PCR阳性结果,也要考虑对身体不适的儿童进行其他诊断。患有轻至中度covid - 19的儿童通常不需要住院或血液检查和放射检查,除非临床另有指示。患有严重或危重疾病的儿童至少应进行以下检查:血培养、全血细胞计数(FBC)、凝血谱、d -二聚体、尿素和电解质、LFT、CRP、肌钙蛋白、铁蛋白、乳酸脱氢酶(LDH)和血气分析。Remdesivir可用于儿童;12岁40公斤的COVID-19患者需要补充氧气。考虑对不符合预期临床过程的儿童进行胸部x光检查,例如,入院第三天仍需要吸氧的儿童,低氧血症恶化的儿童或需要呼吸支持的儿童。将呼吸支持升级为无创通气(NIV)的决定应由儿科团队的高级成员在与重症监护讨论后做出。对于临床表现符合与SARS-CoV-2暂时相关的儿童炎症性多系统综合征(PIMS-TS)的儿童,应按照专门指南进行管理。《北方国际医学院学报》12卷第2期,2021年1月,第548-554页
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引用次数: 0
Variable presentation of Covid 19 infection Covid - 19感染的不同表现
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68313
R. Akhter, B. Yasmeen
Abstract not available Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 555-557
《北方国际医学院学报》2021年1月第12卷第2期,第555-557页
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引用次数: 0
Clinical Spectrum of Major Infection in Hospitalized Children with Nephrotic Syndrome at a Tertiary Care Hospital 某三级医院肾病综合征住院患儿主要感染的临床谱分析
Pub Date : 2023-08-23 DOI: 10.3329/nimcj.v12i2.68311
Khondaker Mobasher Ahmed, Md Manajjir Ali, P. R. Dey, M. Islam, Md Nazmul Hassan
Background : Children with Nephrotic Syndrome (NS) are exposed to multiple infections resulting in significant morbidity and mortality. Besides being a common cause of mortality, infections may also be responsible for a poor response to steroid therapy or induce relapse in a child with Nephrotic Syndrome. Aims: To assess the clinical spectrum of major infection in hospitalized children with Nephrotic Syndrome. Materials and Methods: This hospital based cross-sectional study was done in the Department of Paediatrics, Sylhet M A G Osmani Medical College Hospital, Sylhet, Bangladesh. In this study, sample size was 44 diagnosed cases of Nephrotic Syndrome with infection. After taking consent, detailed history and thorough physical examination were done in each patient. All data were collected in a preformed questioner and finally collected data were expressed in tabulated form. Results: In our study total 44 children with Nephrotic Syndrome were enrolled with the mean age of 5.98 ± 2.93 years and most of the childhood Nephrotic Syndrome was aged between 2 to 8 years (75.0%); 23 (52.3%) patients were male, and 21 (47.7%) patients were female with a ratio of male to female of 1.1:1. The common clinical symptoms of major infection were fever (79.5%) and cough (70.5%). Other clinical presentations were throat pain (18.2%), abdominal pain (15.9%), runny nose (11.4%), burning micturition (9.1%), respiratory distress (6.8%), vomiting (4.5%) and leg pain (4.5%). Relapse of Nephrotic Syndrome was found in 33 (75.0%) cases. The most common major infection was pneumonia (50.0%), followed by urinary tract infection (20.5%), Upper respiratory tract infection (15.9%), pneumonia with UTI (6.8%), cellulitis (4.5%) and pneumonia with UTI with spontaneous bacterial peritonitis (2.3%). The most common isolated organisms in urine were Escherichia coli (27.3%), followed by Klebsiella pneumoniae (2.3%). Conclusion: The common symptoms of major infection in Nephrotic Syndrome are fever and cough. The most common major infection was pneumonia and urinary tract infection. Northern International Medical College Journal Vol. 12 No.2 Jan 2021, Page 544-547
背景:儿童肾病综合征(NS)暴露于多种感染,导致显著的发病率和死亡率。感染除了是导致死亡的常见原因外,还可能导致对类固醇治疗反应不佳或导致肾病综合征患儿复发。目的:了解住院肾病综合征患儿主要感染的临床特征。材料和方法:这项以医院为基础的横断面研究是在孟加拉国Sylhet的Sylhet M A G Osmani医学院医院儿科完成的。本研究的样本量为44例诊断为肾病综合征并发感染的病例。在征得同意后,对每位患者进行详细的病史和全面的体格检查。所有的数据都是在事先准备好的问卷中收集的,最后收集到的数据以表格的形式表示。结果:本研究共纳入44例肾病综合征患儿,平均年龄为5.98±2.93岁,多数患儿年龄在2 ~ 8岁之间(75.0%);男性23例(52.3%),女性21例(47.7%),男女比例为1.1:1。主要感染的常见临床症状为发热(79.5%)和咳嗽(70.5%)。其他临床表现为喉咙痛(18.2%)、腹痛(15.9%)、流鼻涕(11.4%)、排尿灼烧(9.1%)、呼吸窘迫(6.8%)、呕吐(4.5%)和腿痛(4.5%)。复发33例(75.0%)。最常见的主要感染为肺炎(50.0%),其次为尿路感染(20.5%)、上呼吸道感染(15.9%)、肺炎合并UTI(6.8%)、蜂窝织炎(4.5%)和肺炎合并UTI合并自发性细菌性腹膜炎(2.3%)。尿液中最常见的分离菌是大肠杆菌(27.3%),其次是肺炎克雷伯菌(2.3%)。结论:肾病综合征主要感染的常见症状为发热、咳嗽。最常见的主要感染是肺炎和尿路感染。《北方国际医学院学报》12卷,2021年1月第2期,544-547页
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引用次数: 0
Three consecutive recurrent ectopic pregnancy-A Case Report 连续3例复发异位妊娠1例报告
Pub Date : 2022-09-13 DOI: 10.3329/nimcj.v12i1.61593
S. Akhter, B. Yasmeen, Chishti Tanhar Bakth Choudhury
Ectopic pregnancy (EP) is a potentially life-threatening condition, leads to increased maternal mortality and morbidity till date. About 2% of all pregnancies are Ectopic one and recurrence rate is as high as 1 in every 10 pregnancies. Here we present a case who had a history of 2 Ectopic pregnancies – 1st one treated surgically and 2nd one by medical method. Unfortunately, 3rd pregnancy was also an Ectopic one and needed surgical management. Thus, her natural reproductive ability went down and had to go for In vitro fertilization (IVF) pregnancy and successfully got twin baby (Girl, on March,2020).Northern International Medical College Journal Vol. 12 No.1 July 2020, Page 511-514
宫外孕(EP)是一种潜在的危及生命的疾病,导致孕产妇死亡率和发病率上升。大约2%的妊娠是异位妊娠,复发率高达1 / 10。我们在此报告一个有2次异位妊娠史的病例,第一次是手术治疗,第二次是内科治疗。不幸的是,第三次妊娠也是异位妊娠,需要手术治疗。因此,她的自然生殖能力下降,不得不进行体外受精(IVF)怀孕,并成功生下了一对双胞胎婴儿(女孩,2020年3月)。《北方国际医学院学报》2020年7月第12卷第1期,第511-514页
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引用次数: 0
Crigler Najjar Syndrome - A Rare case of Jaundice in Children Crigler - Najjar综合征-儿童黄疸的罕见病例
Pub Date : 2022-09-13 DOI: 10.3329/nimcj.v12i1.61594
Bodhrun Naher, M. Mazumder, S. Ghosal, A. R. Rahman, A. Karim
Crigler-Najjar syndrome (CNS) was first described in 1952 in Maryland, USA as congenital familial non-hemolytic jaundice with kernicterus by Crigler JF and Najjar VA.1 CNS is a rare genetic disorder characterized by abnormalities in bilirubin metabolism and evident by persistent increase of unconjugated bilirubin. During the first days of life, the syndrome clinically manifests as intense unconjugated hyperbilirubinemia without evidence of hemolysis. It consists of two types, type I and type II. Crigler-Najjar Syndrome is mostly autosomal recessive disorder, but variation may occur in the inheritance of CNS II.2 The key pathogenesis is defect in bilirubin conjugation due to complete or partial deficiency of uridine 5'-diphosphate-glucuronosyl transferase (UGT). This enzyme is required for the conjugation and further excretion of bilirubin from the body. In type I CNS the enzyme activity is completely absent and in type II there is partial absence of the enzyme. Therefore, Type I is more severe form and usually fatal with kernicterus at the age of 1-2 years.3-5 TypeII is less severe and has better prognosis. Patients with CN type II suffer from less jaundice, less neurological impairment, and show a fair response to phenobarbitone therapy (serum bilirubin levels decrease by at least 25%).6 Both males and females are equally affected by CNS. The incidence is approximately 1 in 750,000-1,000,000 in the general population.7 Here we report such a rare case.Northern International Medical College Journal Vol. 12 No.1 July 2020, Page 515-517
Crigler-Najjar综合征(Crigler-Najjar syndrome, CNS)于1952年在美国马里兰州首次被Crigler JF和Najjar va描述为先天性家族性非溶血性黄疸伴核黄疸。CNS是一种罕见的遗传性疾病,以胆红素代谢异常为特征,以非共轭胆红素持续升高为明显特征。在生命的最初几天,该综合征的临床表现为强烈的非共轭高胆红素血症,没有溶血的证据。它包括两种类型,I型和II型。Crigler-Najjar综合征多为常染色体隐性遗传病,但CNSⅱ的遗传也可能发生变异。2主要发病机制是由于尿苷5′-二磷酸-葡萄糖醛酸转移酶(UGT)完全或部分缺乏导致胆红素偶联缺陷。这种酶是结合和进一步从体内排出胆红素所必需的。在I型中枢神经系统中,酶活性完全缺失,而在II型中枢神经系统中,酶部分缺失。因此,I型是更严重的形式,通常在1-2岁时与核黄疸致命。3-5型病情较轻,预后较好。CN II型患者黄疸较少,神经功能受损较少,对苯巴比妥治疗反应良好(血清胆红素水平至少降低25%)男性和女性都同样受到中枢神经系统的影响。在一般人群中发病率约为75 - 100万分之一在此我们报告这样一个罕见的病例。《北方国际医学院学报》2020年7月第12卷第1期,第515-517页
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Northern International Medical College Journal
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