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The role of β-2-microglobulin and cystatin C as urinary biomarkers of focal segmental glomerulosclerosis in the setting of paediatric HIV infection β-2微球蛋白和胱抑素C作为小儿HIV感染局灶节段性肾小球硬化的尿液生物标志物的作用
Q4 PEDIATRICS Pub Date : 2023-05-24 DOI: 10.7196/sajch.2023.v17i2.1951
K Persad, L Nandlal, R Bhimma, T Naicker
Background. Africa has the highest rate of HIV infection, and HIV-associated nephropathy (HIVAN) is one of the most frequent kidneydiseases observed in children. HIVAN in children usually presents as a form of nephrotic syndrome, predominantly focal segmentalglomerulosclerosis (FSGS) on histopathology, that often leads to chronic kidney failure.Objective. This study determined the urinary concentrations of β-2-microglobulin (β2M) and cystatin C proteins in children with HIVANand primary FSGS.Methods. The study group comprised 34 children; 14 with HIVAN and 20 with primary FSGS. The control groups were 20 HIV-positiveand 20 HIV-negative children with no kidney disease. Urine samples collected from these 74 children were stored at -80°C. Bio-Plextechnology was used to analyse the urinary protein concentration of cystatin C and β2M.Results. A significant increase in urinary β2M levels was observed in the HIVAN group compared with the HIV-negative group(p=0.0240). No other statistically significant differences in urinary β2M concentrations were noted across the study groups. Urinarycystatin C levels were significantly increased in primary FSGS children compared with both HIV-negative (p=0.0041) and HIV-positivecontrols (p=0.0256). Urinary cystatin C displayed a significant increase in the primary FSGS compared with the HIVAN group (p=0.0150).No significant differences in urinary cystatin C levels were noted in the HIVAN group compared with the HIV-negative and HIV-positivecontrol groups.Conclusion. Urinary cystatin C has promising prognostic value to predict primary FSGS from HIVAN.
背景。非洲是艾滋病感染率最高的地区,艾滋病相关肾病(HIV -associated nephropathy, HIVAN)是儿童中最常见的肾脏疾病之一。儿童HIVAN通常表现为肾病综合征的一种形式,在组织病理学上以局灶节段性肾小球硬化(FSGS)为主,常导致慢性肾衰竭。本研究测定了hiv和原发性fsgs患儿尿中β-2-微球蛋白(β2M)和胱抑素C蛋白的浓度。研究小组包括34名儿童;14例HIVAN, 20例原发性FSGS。对照组为20名hiv阳性和20名hiv阴性无肾脏疾病的儿童。这74名儿童的尿液样本保存在-80°C。采用Bio-Plextechnology检测尿胱抑素C和β 2m蛋白浓度。与hiv阴性组相比,hiv组尿β2M水平显著升高(p=0.0240)。各研究组尿β2M浓度没有其他统计学上的显著差异。与hiv阴性(p=0.0041)和hiv阳性对照(p=0.0256)相比,原发性FSGS患儿尿胱抑素C水平显著升高。与HIVAN组相比,原发性FSGS患者尿胱抑素C水平显著升高(p=0.0150)。与hiv阴性和hiv阳性对照组相比,hiv组尿胱抑素C水平无显著差异。尿胱抑素C在预测由hiv引起的原发性FSGS方面具有良好的预后价值。
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引用次数: 0
Primary nephrotic syndrome in children in Cape Town, South Africa 南非开普敦儿童原发性肾病综合征
Q4 PEDIATRICS Pub Date : 2023-05-24 DOI: 10.7196/sajch.2023.v17i2.1945
D Reddy, A Coetzee, K Webb, M McCulloch, P Nourse
Background. Histopathological patterns of childhood primary nephrotic syndrome (PNS) and clinical response to steroids have beenassociated with certain race groups in parts of South Africa. However, there are no recent studies of childhood PNS in Cape Town.Objectives. To describe the demographics, histological subtypes and steroid response of patients with PNS who underwent kidney biopsiesat Red Cross War Memorial Children’s Hospital (RCWMCH) over a 10-year period.Methods. Details of patients with PNS who underwent kidney biopsies in the Paediatric Nephrology Department at RCWMCH between2006 and 2015 were retrospectively recorded.Results. A total of 103 patients were included in the study. Most patients were either of mixed race (42%) or black (36%), with a mean age of6.8 years and a male-to-female ratio of 1.19:1. The most identified histopathological subtype was mesangioproliferative glomerulonephritis(MesPGN; 60% (n/N=62/103)). Of the patients with focal segmental glomerulosclerosis (FSGS), MesPGN and minimal change disease(MCD) 45% (n/N=43/95) were steroid-resistant, and 54% (n/N=51/95) were steroid-sensitive. There was no significant associationbetween any race group and steroid response. Patients with FSGS were more likely to be black, while MCD was more common in mixed-race patients (p=0.04). There was no difference in the likelihood of being mixed race or black between patients with FSGS and MesPGN(p=0.472).Conclusion. MesPGN was the most common histopathological subtype found in our study. There was no significant association betweenrace and steroid response. Patients with FSGS were more likely to be black than mixed race when compared with MCD patients. Race wasnot otherwise significantly associated with any histopathological subtype
背景。在南非部分地区,儿童原发性肾病综合征(PNS)的组织病理学模式和对类固醇的临床反应与某些种族群体有关。然而,最近在开普敦没有关于儿童PNS的研究。描述在红十字战争纪念儿童医院(RCWMCH)接受肾活检的PNS患者10年期间的人口统计学、组织学亚型和类固醇反应。回顾性记录2006年至2015年期间在RCWMCH儿科肾内科接受肾活检的PNS患者的详细信息。研究共纳入103例患者。大多数患者为混血(42%)或黑人(36%),平均年龄为6.8岁,男女比例为1.19:1。最常见的组织病理学亚型是系血管增殖性肾小球肾炎(MesPGN;60% (n / n = 62/103))。局灶节段性肾小球硬化(FSGS)、MesPGN和最小改变病(MCD)患者中,45% (n/ n =43/95)为类固醇耐药患者,54% (n/ n =51/95)为类固醇敏感患者。任何种族和类固醇反应之间没有明显的联系。FSGS患者多为黑人,而MCD患者多为混血儿(p=0.04)。FSGS患者与MesPGN患者混血或黑人的可能性无差异(p=0.472)。MesPGN是我们研究中发现的最常见的组织病理学亚型。在种族和类固醇反应之间没有明显的关联。与MCD患者相比,FSGS患者更有可能是黑人而不是混血儿。种族与任何组织病理学亚型均无显著相关性
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引用次数: 0
Breastfeeding intentions and behaviours of doctor mothers in Bloemfontein, South Africa 南非布隆方丹医生母亲的母乳喂养意图和行为
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-03-10 DOI: 10.7196/sajch.2023.v17i1.1929
C. van der Bijl, W. J. Steinberg, T. E. Kellerman, J. Botes, C. van Rooyen
Background. Doctor mothers are a high-risk group for early cessation of breastfeeding. Improving breastfeeding among doctor mothers would not only improve the wellbeing of their babies, but the experience might also enhance their advocacy for breastfeeding to patients.Objective. To describe the infant feeding intentions and behaviours of doctor mothers in Bloemfontein, South Africa, focusing onbreastfeeding and factors that impact the decision on feeding method.Methods. This was a descriptive study. The target population included female medical doctors with a biological child under the ageof 5 years, recruited via snowball sampling. Respondents completed an electronic questionnaire, answering questions regarding infantfeeding intentions and behaviours.Results. There were 104 respondents who provided information on 132 children. The median intended duration of exclusivebreastfeeding for the first-born child was 6 months and 6.5 months for the second-born, but the actual median duration for bothchildren was 3 months shorter. The intention to express breastmilk at work was 67.7% for Child 1 and 57.7% for Child 2; however,the actual outcome was less than half of the intention (32.0%). Most respondents (71.0%) indicated there was no dedicated area forexpressing breastmilk at their place of work.Conclusion. The intention to exclusively breastfeed was high (6 months), but the actual duration was 3 months shorter. The percentage of doctor mothers who eventually expressed breastmilk at work was less than half of those who intended to do so. Breastfeeding female doctors need more support in the postpartum period, especially when returning to work after maternity leave
背景医生母亲是提前停止母乳喂养的高危人群。改善医生母亲的母乳喂养不仅会改善婴儿的健康,而且这种经历还可能加强她们对患者母乳喂养的宣传。客观的描述南非布隆方丹医生母亲的婴儿喂养意图和行为,重点关注母乳喂养和影响喂养方法决策的因素。方法。这是一项描述性研究。目标人群包括通过滚雪球抽样招募的有5岁以下亲生子女的女医生。受访者填写了一份电子问卷,回答了有关婴儿喂养意图和行为的问题。后果共有104名受访者提供了132名儿童的信息。第一个孩子纯母乳喂养的预期持续时间中位数为6个月,第二个孩子为6.5个月,但两个孩子的实际持续时间中位数均短3个月。1岁儿童和2岁儿童在工作中表达母乳的意愿分别为67.7%和57.7%;然而,实际结果还不到预期的一半(32.0%)。大多数受访者(71.0%)表示,他们的工作场所没有专门的挤母乳区域。结论纯母乳喂养的意愿很高(6个月),但实际持续时间短了3个月。母乳喂养的女医生在产后需要更多的支持,尤其是在产假后重返工作岗位时
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引用次数: 0
The value of routine empiric antibiotic use in neonates born to mothers with prolonged rupture of membranes 常规经验性抗生素应用于长期胎膜破裂母亲所生新生儿的价值
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-03-10 DOI: 10.7196/sajch.2023.v17i1.xx
T. C. Gcaba, R. Singh, P. Jeena
Background. The routine use of empiric antibiotics in neonates born to mothers with prolonged rupture of membranes (PROM) iscontroversial.Objectives. To determine the incidence of probable and proven sepsis in such neonates and identify risk factors for sepsis and theiroutcomes.Methods. This was a retrospective chart review conducted at King Edward VIII Hospital over two years. Study participants included 200neonates and 181 mothers. Data were captured onto Microsoft Excel, collated and analysed using descriptive statistics and comparative data utilising the R Core Team’s R Statistical Computing Software, 2020.Results. Seven neonates (3.5%) had proven sepsis, 58 (29%) had probable sepsis, and 135 (67.5%) were without sepsis. Two (1.0%) neonates died and 188 (94.0%) received antibiotics. White cell count was normal in all cases without sepsis and abnormal in 65.5% and 28.6% of cases with probable and proven sepsis, respectively. A raised C-reactive protein was observed in only 22.4% and 14.3% of neonates with probable and proven sepsis, respectively. One hundred and sixty-seven (83.5%) mothers had no Group B Streptococcus (GBS) screening. Of those screened, three had GBS infection, but two did not receive antibiotics.Conclusions. The incidence of sepsis following PROM is low. Identifying neonates at risk is challenging, but the absence of clinical features and normal rapidly obtained supportive laboratory markers of sepsis provides reassurance that antibiotics could be temporarily withheld. Better GBS screening programmes and appropriate antibiotic responses for pregnant women should be implemented rigorously.
背景长期胎膜早破(PROM)母亲所生新生儿常规使用经验性抗生素具有争议性。目标。确定此类新生儿中可能和已证实的败血症的发生率,并确定败血症的危险因素及其结果。方法。这是爱德华八世国王医院两年来进行的一次回顾性图表审查。研究参与者包括200名新生儿和181名母亲。数据被收集到Microsoft Excel中,使用描述性统计数据和比较数据进行整理和分析,使用R核心团队的R统计计算软件,2020。结果:7名新生儿(3.5%)被证实患有败血症,58名新生儿(29%)可能患有败血症,135名新生儿(67.5%)没有败血症。2例(1.0%)新生儿死亡,188例(94.0%)接受抗生素治疗。在所有没有败血症的病例中,白细胞计数正常,在可能和已证实的败血症病例中,分别有65.5%和28.6%的白细胞计数异常。在可能和已证实败血症的新生儿中,C反应蛋白升高的比例分别为22.4%和14.3%。167名(83.5%)母亲未进行B组链球菌(GBS)筛查。在接受筛查的患者中,有三人感染了GBS,但有两人没有接受抗生素治疗。结论。胎膜早破术后败血症的发生率较低。识别有风险的新生儿具有挑战性,但由于缺乏败血症的临床特征和正常快速获得的支持性实验室标志物,可以保证抗生素可以暂时保留。应严格执行更好的GBS筛查计划和孕妇适当的抗生素反应。
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引用次数: 0
Clinical, biochemical, and histopathological diagnosis of Egyptian paediatric patients with suspected mitochondrial diseases: A hospital‑based study 疑似线粒体疾病的埃及儿科患者的临床、生化和组织病理学诊断:一项基于医院的研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-03-10 DOI: 10.7196/sajch.2023.v17i1.1779
D. Abdou, L. Selim, R. Coster, J. Smet, G. Nakhla, D. Mehaney
Background. Mitochondrial respiratory chain (RC) disorders are a growing group of disorders with a large variety of clinical presentations ranging from well-defined clinical syndromes to nonspecific manifestations, such as failure to thrive, exercise intolerance and seizures.Objective. To describe the clinical, biochemical, and histochemical spectrum of 38 Egyptian patients clinically suspected of havingmitochondrial RC disorders.Methods. A total of 38 patients (female, n=18 (47.4%); male, n=20 (52.6%)) clinically suspected of having mitochondrial diseases had been referred to the Inherited Metabolic Diseases Laboratory at Cairo University Children’s Hospital. Laboratory investigations and analyses included histochemical staining of cytochrome c oxidase and succinate dehydrogenase in muscle biopsies, as well as spectrophotometric assays of RC complexes in muscle homogenates.Results. Twenty-three patients (60.5%) were diagnosed with different RC enzyme deficiencies. Fifteen patients (65%) had complex Ideficiency and all of them also had lactic acidosis (mean (standard deviation)) plasma lactate concentration of 4 (1.65) mmol/L). Twopatients (9%) with marked complex IV deficiency both showed COX-negative ragged red fibers (RRFs) on histochemical staining.Combined complex I and complex II deficiency with scattered COX-stain deficiency and RRFs was diagnosed in 2 patients (5.25%), whilea further 2 patients (5.26%) had combined (complex I, II+III, complex IV) deficiencies. Isolated complex II deficiency was diagnosed in2 patients (5.26%) and 15 (39.5%) patients had normal RC enzyme activities.Conclusion. Biochemical assay of RC complexes is considered the cornerstone for diagnosis of RC complex mitochondrial disorders.These disorders are common among Egyptian paediatric patients.
背景。线粒体呼吸链(RC)疾病是一种越来越多的疾病,具有各种各样的临床表现,从明确的临床综合征到非特异性表现,如发育不良、运动不耐受和癫痫发作。描述38例埃及临床怀疑线粒体RC疾病患者的临床、生化和组织化学谱。共38例患者(女性18例,占47.4%);男性,n=20(52.6%)),临床怀疑患有线粒体疾病,已被转介到开罗大学儿童医院的遗传代谢疾病实验室。实验室研究和分析包括肌肉活检组织中细胞色素c氧化酶和琥珀酸脱氢酶的组织化学染色,以及肌肉匀浆中RC复合物的分光光度测定。23例(60.5%)被诊断为不同程度的RC酶缺乏症。15例(65%)患者有复合有效率,且均伴有乳酸酸中毒(平均(标准差)血浆乳酸浓度为4 (1.65)mmol/L)。2例复合体IV缺乏的患者(9%)在组织化学染色上均显示cox阴性的粗糙红纤维(RRFs)。2例(5.25%)诊断为复合物I和复合物II缺乏症合并cox -染色缺乏症和RRFs,另外2例(5.26%)诊断为复合物I、复合物II+复合物III、复合物IV缺乏症。分离性复合体II缺乏症2例(5.26%),RC酶活性正常的15例(39.5%)。RC复合物的生化分析被认为是RC复合物线粒体疾病诊断的基石。这些疾病在埃及儿科患者中很常见。
{"title":"Clinical, biochemical, and histopathological diagnosis of Egyptian paediatric patients with suspected mitochondrial diseases: A hospital‑based study","authors":"D. Abdou, L. Selim, R. Coster, J. Smet, G. Nakhla, D. Mehaney","doi":"10.7196/sajch.2023.v17i1.1779","DOIUrl":"https://doi.org/10.7196/sajch.2023.v17i1.1779","url":null,"abstract":"Background. Mitochondrial respiratory chain (RC) disorders are a growing group of disorders with a large variety of clinical presentations ranging from well-defined clinical syndromes to nonspecific manifestations, such as failure to thrive, exercise intolerance and seizures.Objective. To describe the clinical, biochemical, and histochemical spectrum of 38 Egyptian patients clinically suspected of havingmitochondrial RC disorders.Methods. A total of 38 patients (female, n=18 (47.4%); male, n=20 (52.6%)) clinically suspected of having mitochondrial diseases had been referred to the Inherited Metabolic Diseases Laboratory at Cairo University Children’s Hospital. Laboratory investigations and analyses included histochemical staining of cytochrome c oxidase and succinate dehydrogenase in muscle biopsies, as well as spectrophotometric assays of RC complexes in muscle homogenates.Results. Twenty-three patients (60.5%) were diagnosed with different RC enzyme deficiencies. Fifteen patients (65%) had complex Ideficiency and all of them also had lactic acidosis (mean (standard deviation)) plasma lactate concentration of 4 (1.65) mmol/L). Twopatients (9%) with marked complex IV deficiency both showed COX-negative ragged red fibers (RRFs) on histochemical staining.Combined complex I and complex II deficiency with scattered COX-stain deficiency and RRFs was diagnosed in 2 patients (5.25%), whilea further 2 patients (5.26%) had combined (complex I, II+III, complex IV) deficiencies. Isolated complex II deficiency was diagnosed in2 patients (5.26%) and 15 (39.5%) patients had normal RC enzyme activities.Conclusion. Biochemical assay of RC complexes is considered the cornerstone for diagnosis of RC complex mitochondrial disorders.These disorders are common among Egyptian paediatric patients.","PeriodicalId":44732,"journal":{"name":"South African Journal of Child Health","volume":" ","pages":""},"PeriodicalIF":0.4,"publicationDate":"2023-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46272392","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
An atypical presentation of non-IgE-mediated cow’s milk allergy associated with Staphylococcus aureus: A case study 与金黄色葡萄球菌相关的非IgE介导的牛奶过敏的非典型表现:一例病例研究
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-02-21 DOI: 10.7196/sajch.2023.v17i1.1884
T. Mabelane, Cert. Allerg, M. C. Khaba, FC Path, E. Kiragu, MMed Paeds
Cow’s milk allergy (CMA) is one of the most common food allergies in the first years of life. CMA is classified as immunoglobulin E (IgE)or non-IgE-mediated reactions. IgE-mediated reactions are typically of immediate onset, with symptoms manifesting within 2 hours after ingestion. However, non-IgE-mediated reactions are delayed and mostly occur between 2 and 48 hours post exposure. This report describes a 17-year-old who presented with a long-standing history of pustular lesions immediately after cow’s milk ingestion. Allergy tests, which included skin-prick test (SPT); specific IgE to cow’s milk; cow’s milk IgE components; and Cellular antigen stimulation test (CAST) yielded insignificant results. However, an oral food challenge resulted in pustular lesions and abdominal pain within 30 and 120 minutes, respectively. A swab from a pustule cultured Staphylococcus aureus. CMA was confirmed and managed with dietary restriction. It is uncommon for non- IgE reactions to occur with immediate symptoms. S. aureus may be associated with atypical skin manifestation of CMA.
牛奶过敏(CMA)是生命最初几年最常见的食物过敏之一。CMA分为免疫球蛋白E(IgE)或非IgE介导的反应。IgE介导的反应通常是立即发生的,症状在摄入后2小时内显现。然而,非IgE介导的反应是延迟的,并且大多发生在暴露后2至48小时之间。本报告描述了一名17岁的患者,他在摄入牛奶后立即出现长期的脓疱病变史。过敏试验,包括皮肤点刺试验;牛奶特异性IgE;牛奶IgE成分;细胞抗原刺激试验(CAST)结果不显著。然而,口服食物攻击分别在30分钟和120分钟内导致脓疱病变和腹痛。从培养金黄色葡萄球菌的脓疱中取出拭子。CMA得到确认,并在饮食限制的情况下进行管理。出现非IgE反应并立即出现症状的情况并不常见。金黄色葡萄球菌可能与CMA的非典型皮肤表现有关。
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引用次数: 0
Outcome of infants with necrotising enterocolitis at Charlotte Maxeke Johannesburg Academic Hospital, South Africa 南非Charlotte Maxeke Johannesburg学术医院坏死性小肠结肠炎婴儿的预后
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-02-21 DOI: 10.7196/sajch.2023.v17i1.1939
M. Selse, RT Saggers, E. Hentz, A. Elfvin, DE Ballot
Background. Necrotising enterocolitis (NEC) is an inflammatory disease almost exclusively affecting preterm infants. Previous research has presented a higher mortality rate in infants requiring surgical treatment compared with infants receiving medical treatment. However, the knowledge of mortality and morbidity of the disease in low- and middle-income countries is still limited.Objectives. To review infants with NEC admitted to the neonatal unit at Charlotte Maxeke Johannesburg Academic Hospital (CMJAH),determine a potential difference in mortality between medically and surgically treated infants, and to identify characteristics and factors associated with mortality among these infants.Methods. This retrospective study described infants with NEC born between 1 January 2016 and 31 December 2018 who were admittedto the neonatal unit. The characteristics and survival of these infants were compared using univariate and multivariate analyses.Results. During the study period, 5 061 infants were admitted to the neonatal unit, of which 218 infants were diagnosed with NEC.The period prevalence of NEC was 4.3% among all neonatal infants and 11.0% among very-low-birthweight (VLBW) infants. Mortalitywas significantly higher among surgically treated infants with NEC compared with medically treated infants (p=0.025, odds ratio 1.888(95% confidence interval 1.082 - 3.296)). Late-onset sepsis was significantly more common among VLBW infants with NEC (71.3%)compared with VLBW infants without NEC (27.1%). Among infants with late-onset sepsis, Gram-negative bacteria, multidrug-resistantbacteria and fungal sepsis was significantly more common in the group of infants with NEC.Conclusions. Infants with NEC treated surgically at CMJAH have an increased risk of dying compared with those receiving medicaltreatment, likely due to the severity of disease. Furthermore, this study emphasised the burden of sepsis among infants with NEC and may contribute to a better knowledge of NEC in South Africa.
背景。坏死性小肠结肠炎(NEC)是一种几乎只影响早产儿的炎症性疾病。先前的研究表明,与接受内科治疗的婴儿相比,需要手术治疗的婴儿死亡率更高。然而,低收入和中等收入国家对该病的死亡率和发病率的了解仍然有限。本研究回顾了在Charlotte Maxeke约翰内斯堡学术医院(CMJAH)新生儿病房收治的NEC患儿,确定药物治疗和手术治疗患儿死亡率的潜在差异,并确定这些患儿死亡率的特征和相关因素。本回顾性研究描述了2016年1月1日至2018年12月31日期间出生并入住新生儿病房的NEC婴儿。采用单因素和多因素分析对这些婴儿的特征和生存率进行比较。在研究期间,5061名婴儿被送入新生儿病房,其中218名婴儿被诊断为NEC。在所有新生儿中,NEC的期间患病率为4.3%,在极低出生体重(VLBW)婴儿中为11.0%。手术治疗的NEC患儿死亡率明显高于药物治疗的患儿(p=0.025,优势比1.888(95%可信区间1.082 - 3.296))。晚发型脓毒症在伴有NEC的VLBW婴儿中(71.3%)比无NEC的VLBW婴儿(27.1%)更为常见。在迟发性脓毒症患儿中,革兰氏阴性菌、耐多药菌和真菌性脓毒症在nec患儿中更为常见。与接受药物治疗的婴儿相比,在CMJAH接受手术治疗的NEC婴儿死亡风险增加,可能是由于疾病的严重程度。此外,这项研究强调了NEC婴儿败血症的负担,可能有助于更好地了解南非的NEC。
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引用次数: 0
Crescentic glomerulonephritis in children: A retrospective review of data from Chris Hani Baragwanath Academic Hospital 儿童新月体肾小球肾炎:Chris Hani Baragwanath学术医院数据的回顾性分析
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-02-21 DOI: 10.7196/sajch.2023.v17i1.1885
S. Mansoor, K. Petersen, UK Kala, P. Mosiane
Background. Crescentic glomerulonephritis (CGN) as a cause of progressive renal failure is rare. Crescent formation on kidney biopsyrepresents a response to injury of the glomerular capillary walls. There are limited published reports on paediatric CGN in Africa.Objectives. To describe the clinical presentation and outcome of children with CGN in Soweto, South Africa, over a 22-year period.Methods. A retrospective study was conducted at the Paediatric Renal Unit at Chris Hani Baragwanath Academic Hospital. Childrenyounger than 14 years with crescent formation in more than 50% of glomeruli on renal biopsy were included in the study. Kidney biopsy specimens were examined by light microscopy, immunofluorescence and electron microscopy. Demographic and clinical data were extracted from the patient files.Results. During the study period, 961 kidney biopsies were performed. Fourteen patients (1.5%) met inclusion criteria of crescents in>50% of glomeruli. Common clinical findings were oedema (n=13; 93%), microscopic haematuria (n=12; 86%), hypertension (n=11; 79%)and proteinuria (n=10; 71%). The median estimated glomerular filtration rate (eGFR) at presentation was 23.9 mL/min/1.73m2. Thirteen patients (93%) had immune-complex-mediated glomerulonephritis. The underlying cause was acute post-infectious glomerulonephritis in 9 patients (64%), membranoproliferative glomerulonephritis and IgA nephropathy in 2 patients each (14%) and global sclerosis in 1 patient (7%). Prolonged duration of symptoms resulted in a lower eGFR at follow-up. Treatment included peritoneal dialysis, methylprednisolone and cyclophosphamide. Seven (54%) patients had a normal eGFR at a median (range) follow-up of 36.7 (4.5 - 61.5) months. Six (46%) patients had progressed to chronic kidney disease stages 2 - 5. One patient was followed up for less than 3 months, and therefore was not included in the follow-up results.Conclusion. Poor outcomes were observed in patients who presented late. The clinical findings of haematuria, hypertension and acutekidney injury warrant early referral and kidney biopsy to determine management.
背景Crescentic肾小球肾炎(CGN)作为进行性肾功能衰竭的原因是罕见的。肾脏活检上新月形的形成代表了对肾小球毛细血管壁损伤的反应。关于非洲儿科CGN的已发表报告有限。目标。描述南非索韦托22年来CGN儿童的临床表现和结果。方法。在Chris Hani Baragwanath学术医院的儿科肾脏科进行了一项回顾性研究。本研究纳入了14岁以下的儿童,在肾活检中,50%以上的肾小球形成新月形。肾活检标本采用光学显微镜、免疫荧光和电子显微镜检查。从患者档案中提取人口统计学和临床数据。后果在研究期间,共进行了961次肾活检。14名患者(1.5%)符合50%以上肾小球新月体的纳入标准。常见的临床表现为水肿(n=13;93%)、镜下血尿(n=12;86%)、高血压(n=11;79%)和蛋白尿(n=10;71%)。肾小球滤过率(eGFR)的中位数估计值为23.9 mL/min/1.73m2。13名患者(93%)患有免疫复合物介导的肾小球肾炎。根本原因是9例(64%)感染后急性肾小球肾炎,2例(14%)膜增殖性肾小球肾炎和IgA肾病,1例(7%)全身硬化。症状持续时间延长导致随访时eGFR降低。治疗包括腹膜透析、甲基强的松龙和环磷酰胺。7名(54%)患者在中位(范围)随访36.7(4.5-61.5)个月时eGFR正常。6名(46%)患者进展为慢性肾脏病2-5期。一名患者的随访时间不到3个月,因此不包括在随访结果中。结论在出现较晚的患者中观察到不良结果。血尿、高血压和急性肾损伤的临床表现值得早期转诊和肾活检以确定治疗方法。
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引用次数: 0
A review of spinal muscular atrophy in black South African paediatric patients 南非黑人儿童脊髓性肌萎缩症的回顾
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-02-21 DOI: 10.7196/sajch.2023.v17i1.1942
K. Flack, M. Hauptfleisch, L. Scher
Background. Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder that is present in all populations andresults in muscle weakness owing to anterior horn cell degeneration. SMA is divided into three clinical subtypes and is an importantgenetic cause of morbidity and mortality but has not been well studied in sub-Saharan Africa.Objective. This study aims to describe the clinical features and genetic findings in black patients with SMA presenting to the Division ofPaediatric Neurology at Chris Hani Baragwanath Academic Hospital (CHBAH) over a 30-year period.Method. This study was a retrospective review of patient records. The study population was black paediatric neurology patients withclinical SMA, who attended CHBAH Neurology Clinic between 1988 and 2018. Patients were categorised into SMA type 1, 2 or 3 basedon their neurology assessment and clinical features were recorded.Results. The clinical findings in the study population (with SMA), i.e. hypotonia, areflexia and tongue fasciculations, were similar to those found in international studies. More than half of the patients (65.6%; n=86/131) had genetic tests, of which 84.8% were positive for SMA. This value was significantly higher than previously reported results from South Africa. At least 23.6% (n=31/131) had facial involvement.Conclusions. This study adds to the limited body of research on SMA in sub-Saharan Africa and highlights the lower frequency of ahomozygous deletion seen in the black South African population compared with the expected 95% worldwide.
背景脊髓性肌萎缩(SMA)是一种常染色体隐性遗传的神经肌肉疾病,存在于所有人群中,并由于前角细胞变性导致肌肉无力。SMA分为三种临床亚型,是发病率和死亡率的重要遗传原因,但在撒哈拉以南非洲尚未得到很好的研究。客观的这项研究旨在描述在30年的时间里,在Chris Hani Baragwanath学术医院(CHBAH)儿科神经科就诊的SMA黑人患者的临床特征和基因发现。方法这项研究是对患者记录的回顾性回顾。研究人群是患有临床SMA的黑人儿科神经病学患者,他们在1988年至2018年间参加了CHBAH神经病学诊所。根据他们的神经病学评估和临床特征,将患者分为SMA 1型、2型或3型。后果研究人群(SMA患者)的临床发现,即肌张力减退、屈折和舌束,与国际研究中发现的结果相似。超过一半的患者(65.6%;n=86/131)进行了基因检测,其中84.8%的SMA呈阳性。这一数值明显高于南非先前报告的结果。至少23.6%(n=31/131)有面部受累。结论。这项研究增加了对撒哈拉以南非洲SMA的有限研究,并强调了在南非黑人人群中发现的异常缺失频率较低,而全球预计为95%。
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引用次数: 0
Asthma control among adolescents in the inner provinces of South Africa: Perception v. reality 南非内省青少年哮喘控制:感知与现实
IF 0.4 Q4 PEDIATRICS Pub Date : 2023-02-16 DOI: 10.7196/sajch.2023.v17i1.1958
AO Olajide-Ibiejugba, V. Nkosi, F. Rathogwa-Takalani, J. Shirinde, J. Wichmann, R. Green, K. Voyi
Proper asthma control can lessen the negative effects of asthma in adolescents, such that they can lead a normal and physically activelife. The study aimed to compare self-reported asthma control and actual test scores among adolescents in the inner provinces (Gauteng and North West) of South Africa. The asthma control test (ACT) questionnaire was administered to 123 of adolescents who had reported doctor-diagnosed asthma during the cross-sectional study. There was a statistically significant difference between self-reported asthma control and actual test scores among study participants (p=0.003). Out of the 90 study participants who claimed that their asthma was controlled, only 25 (27.78%) were correctly classified as ‘controlled’ according to the ACT. A high percentage (72.22%) of adolescents misclassified their asthma as ‘controlled’, whereas it was not. There is a lack of studies on asthma control in Africa.
适当的哮喘控制可以减轻青少年哮喘的负面影响,使他们能够过上正常的体育活动生活。这项研究旨在比较南非内陆省份(豪登省和西北部)青少年自我报告的哮喘控制和实际测试分数。在横断面研究中,对123名报告医生诊断为哮喘的青少年进行了哮喘控制测试(ACT)问卷调查。研究参与者自我报告的哮喘控制与实际测试分数之间存在统计学上的显著差异(p=0.003)。在90名声称哮喘得到控制的研究参与者中,只有25名(27.78%)根据ACT被正确归类为“控制”。高比例(72.22%)的青少年错误地将他们的哮喘归类为“受控”,而事实并非如此。非洲缺乏关于哮喘控制的研究。
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引用次数: 0
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South African Journal of Child Health
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