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Risk Factors for Unplanned Initiation of Dialysis in Patients with Advanced Chronic Kidney Disease: A Retrospective Cohort Study. 晚期慢性肾病患者计划外开始透析的风险因素:回顾性队列研究
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_93_23
Tarek A Ghonimi, Mohamad M Alkadi, Mohamed T Abdellatif, Hany Ezzat, Tarek A Fouda, Mohamed A Elesnawi, Musab El-Gaali, Hussein Almarawi, Rajvir Singh, Hassan Al-Malki, Abdullah Hamad

Many patients with advanced chronic kidney disease (CKD) managed in a specialized multidisciplinary clinic start dialysis urgently during hospitalization rather than electively as outpatients. This study aimed to identify risk factors for starting unplanned dialysis among patients with advanced CKD who attended multidisciplinary low-clearance clinics between January 1, 2020, and December 31, 2021. Of these, 175 patients started dialysis: 101 (26.7%) started it urgently, whereas 74 (19.5%) started it electively. Patients with urgent initiation of dialysis received less education, had fewer clinic visits and follow-up and were seen less often in the vascular clinic. In the univariate regression analysis, congestive heart failure significantly increased the risk of acute dialysis. Moreover, the risk increased in patients who did not receive dialysis education. The risk increased in patients who were not seen in a vascular clinic and did not have a vascular access plan. Moreover, high albumin levels at initial presentation to the clinic had a lower risk for elective initiation of dialysis. In the multivariate regression analysis, use of renin-angiotensin-aldosterone system inhibitors and attending a vascular clinic reduced the risk of unplanned dialysis by 73% and 96%, respectively. Acute unplanned initiation of dialysis is common even in CKD patients followed in low-clearance clinics. Early referral to multidisciplinary low clearance clinics, timely education, compliance with timely follow-up periods, and creation of access in patients at risk may reduce hospital admissions, hospital stays, admission to intensive care units, costs, and morbidity in these patients.

许多在多学科专科门诊接受治疗的晚期慢性肾脏病(CKD)患者在住院期间紧急开始透析,而不是选择性地在门诊开始透析。本研究旨在确定 2020 年 1 月 1 日至 2021 年 12 月 31 日期间在多学科低通畅率门诊就诊的晚期 CKD 患者开始计划外透析的风险因素。其中,175 名患者开始了透析:101 人(26.7%)紧急开始透析,74 人(19.5%)选择性开始透析。紧急开始透析的患者接受的教育较少,门诊就诊和随访次数较少,在血管门诊就诊的次数也较少。在单变量回归分析中,充血性心力衰竭会显著增加急性透析的风险。此外,未接受透析教育的患者发生急性透析的风险也会增加。未在血管门诊就诊且未制定血管通路计划的患者风险更高。此外,初次就诊时白蛋白水平高的患者选择开始透析的风险较低。在多变量回归分析中,使用肾素-血管紧张素-醛固酮系统抑制剂和就诊于血管门诊可将非计划透析的风险分别降低 73% 和 96%。即使是在低通透率诊所接受随访的慢性肾脏病患者中,急性计划外透析也很常见。及早转诊到多学科低通畅率门诊、及时教育、遵守及时随访期以及为高危患者建立通路,可减少这些患者的入院率、住院时间、重症监护室入院率、费用和发病率。
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引用次数: 0
Incidence, Risk Factors, and Outcomes of Acute Kidney Injury in Preterm Neonates Hospitalized in the Neonatology Unit, North India: A Single-center Experience. 北印度新生儿科住院早产新生儿急性肾损伤的发病率、风险因素和预后:单中心经验。
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_264_23
Sajal Gupta, Bablu Kumar Gaur, Ritu Jain, Rupa R Singh

Acute kidney injury (AKI) is common in premature newborns and is associated with high mortality. It is unclear which risk factors lead to AKI in these neonates. We aimed to determine the incidence, risk factors, and outcomes of AKI in preterm neonates in the neonatal intensive care unit (NICU). They were screened and staged for AKI as per the amended neonatal criteria of Kidney Disease Improving Global Outcomes and followed up until discharge or death. Serum creatinine levels and urine output were measured. The incidence of AKI was 18.5% (37/200 neonates). The majority developed non-oliguric AKI. The risk factors significantly associated with AKI in neonates were the presence of sepsis, birth asphyxia, shock, respiratory distress syndrome, and hypothermia. The majority of neonates with AKI had a birthweight <1500 g and a gestational age of <32 weeks and had a higher risk of mortality, in contrast to than those without AKI. Mortality and NICU stay were significantly higher among those with Stage 3 AKI compared with Stage 2 and Stage 1 AKI. To prevent AKI and reduce the burden of high mortality in premature neonates, it is essential to prevent sepsis, birth asphyxia, and respiratory distress syndrome, as well as to detect shock and patent ductus arteriosus as early as possible. There is a need for good antenatal care to reduce the burden of prematurity.

急性肾损伤(AKI)在早产新生儿中很常见,并与高死亡率相关。目前尚不清楚哪些风险因素会导致这些新生儿发生急性肾损伤。我们旨在确定新生儿重症监护室(NICU)中早产新生儿 AKI 的发生率、风险因素和结果。我们根据 "肾脏疾病改善全球结果 "新生儿标准的修订版对他们进行了 AKI 筛查和分期,并随访至出院或死亡。对血清肌酐水平和尿量进行了测量。AKI 发生率为 18.5%(37/200 名新生儿)。大多数新生儿发生了非胆尿性 AKI。与新生儿 AKI 明显相关的风险因素包括败血症、出生时窒息、休克、呼吸窘迫综合征和体温过低。大多数出现 AKI 的新生儿的出生体重为
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引用次数: 0
Practice Patterns and Approach to Childhood Lupus Nephritis in Saudi Arabia. 沙特阿拉伯儿童狼疮性肾炎的治疗模式和方法。
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_215_23
Abdulaziz AlMutairi, Sulaiman M Al-Mayouf, Jameela Kari, Emtenan Basahl, Mohammed Nashawi

Renal involvement of systemic lupus erythematosus needs aggressive treatment. Despite the development of multiple international guidelines, differences in practices exist. This study aimed to explore the current practices of pediatric rheumatologists and nephrologists for the diagnosis, management, and monitoring of lupus nephritis (LN) in Saudi Arabia through a survey. Among the 61 respondents, 54.1% were pediatric nephrologists and 49.9% were pediatric rheumatologists. Predominantly, the participating physicians received training either nationally (57%) or in North America (45%). Most of the respondents (77%) did not have a combined rheumatology-nephrology clinic, primarily because of space or time limitations (75%), or a lack of the other specialty (13%). In terms of the decision to request a renal biopsy, the most common factors were nephrotic-range proteinuria (85%) and a lower level of proteinuria associated with hypocomplementemia or elevated anti-double-stranded (ds) DNA (73%). There was marginal agreement over monitoring the disease's activity and treatment response; Complements 3 and 4, anti-dsDNA, protein-creatinine ratio, and estimated glomerular filtration rate were the most popular parameters. The main reason for repeating a renal biopsy was a new renal manifestation that was inconsistent with the previous biopsy. There was considerable variability in the induction therapies used to initiate and taper corticosteroids and conventional immunosuppressive drugs. Most respondents (91%) used angiotensin-converting enzyme agents to control proteinuria. Considerable agreement exists among Saudi physicians managing children with LN but significant variations exist regarding the therapeutic strategies. Additional endeavors are needed to establish a unified national clinical approach for managing LN in children.

系统性红斑狼疮的肾脏受累需要积极治疗。尽管已制定了多个国际指南,但实践中仍存在差异。本研究旨在通过一项调查,探讨沙特阿拉伯儿科风湿病学家和肾病学家目前在诊断、管理和监测狼疮肾炎(LN)方面的做法。在 61 名受访者中,54.1% 是儿科肾病专家,49.9% 是儿科风湿病专家。参与调查的医生主要在国内(57%)或北美(45%)接受过培训。大多数受访者(77%)没有风湿病学-肾脏病学联合门诊,主要原因是空间或时间限制(75%)或缺乏其他专科(13%)。在决定是否要求进行肾活检方面,最常见的因素是肾病范围蛋白尿(85%)和与低补体血症或抗双链(ds)DNA 升高相关的较低水平蛋白尿(73%)。在监测疾病活动性和治疗反应方面几乎没有一致意见;补体 3 和 4、抗 dsDNA、蛋白-肌酐比值和估计肾小球滤过率是最常用的参数。重复肾活检的主要原因是出现了与之前活检结果不一致的新的肾脏表现。用于启动和减量皮质类固醇和常规免疫抑制剂的诱导疗法存在很大差异。大多数受访者(91%)使用血管紧张素转换酶制剂来控制蛋白尿。治疗 LN 儿童的沙特医生之间存在相当大的共识,但在治疗策略方面存在显著差异。还需要进一步努力,建立全国统一的儿童 LN 临床治疗方法。
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引用次数: 0
A Comparison of the Spot Urine Protein-Creatinine Ratio with 24-h Urinary Protein for Quantification of Proteinuria: A Hospital-based Cross-sectional Study. 比较点滴尿蛋白-肌酐比值与 24 小时尿蛋白对蛋白尿定量的影响:一项医院横断面研究
IF 0.5 Q3 Medicine Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_261_22
Aditya Mahaseth, Bishnu Pahari, Madhav Ghimire, Sushil Rayamajhi

Quantifying the amount of proteinuria is mandatory in various disease conditions. The aim of this study was to study whether the spot urine protein-creatinine ratio (P-CR) correlates well with 24-h urinary total protein (UTP). The research hypothesis was that spot urine P-CR would correlate well with 24-h UTP. This was a cross-sectional, single-center study conducted in a tertiary care hospital. The spot urinary P-CR and 24-h urinary protein were determined from 70 patients with persistent glomerular proteinuria. This study included Nepalese patients aged 2-83 years, with a mean age of 36.56 years (standard deviation: 20.78). The number of males was slightly higher than females, and the male-female ratio was 1.26:1. Hypertension was present in 44.3% of patients, diabetes was present in 20% of patients, 74.3% of patients were suffering from acute glomerulonephritis with various causes, and 12.9% of patients had chronic kidney disease. A linear relationship existed between the spot urine P-CR and the 24-h UTP, with a correlation coefficient of 0.877 (P <0.01). The correlation was suboptimal at higher levels of protein excretion (>3.5 g/day). Random spot urine P-CR correlated well with the 24-h UTP, particularly at lower levels of protein excretion.

对各种疾病的蛋白尿量进行定量分析是非常必要的。本研究旨在探讨定点尿蛋白-肌酐比值(P-CR)与 24 小时尿总蛋白(UTP)是否有很好的相关性。研究假设是,定点尿蛋白-肌酐比值与 24 小时尿总蛋白相关性较好。这是一项横断面单中心研究,在一家三甲医院进行。对 70 名持续性肾小球蛋白尿患者的定点尿 P-CR 和 24 小时尿蛋白进行了测定。研究对象为尼泊尔患者,年龄在 2-8-33 岁之间,平均年龄为 36.56 岁(标准差:20.78)。男性人数略多于女性,男女比例为 1.26:1。44.3%的患者患有高血压,20%的患者患有糖尿病,74.3%的患者患有各种原因引起的急性肾小球肾炎,12.9%的患者患有慢性肾病。定点尿 P-CR 与 24 小时UTP 之间存在线性关系,相关系数为 0.877(P 3.5 克/天)。随机定点尿 P-CR 与 24 小时UTP 的相关性很好,尤其是在蛋白质排泄量较低的情况下。
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引用次数: 0
A Phosphaturic Mesenchymal Tumor Presenting as Reversible Metabolic Myopathy. 一种表现为可逆性代谢性肌病的磷酸盐间质瘤
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_250_23
Mansoor Abdulla

Tumor-induced osteomalacia (TIO) is a disorder in which the clinical signs and symptoms of osteomalacia and the biochemical abnormalities of hypophosphatemia, phosphaturia, and low serum levels of 1,25(OH)2 Vitamin D3 are secondary to a neoplasm. A 33-year-old woman presented with musculoskeletal pain and proximal myopathy with a duration of 2.5 years which was treated with Vitamin D supplements. On the basis of the biochemical tests and histopathology, she was reevaluated and found to have TIO secondary to a phosphaturic mesenchymal tumor. The tumor was resected (limb salvage with endoprosthesis), and she had no pain or weakness at followup. The case reminds the readers to consider the possibility of TIO when evaluating patients with isolated hypophosphatemia, which may lead to long-term disability and prolonged morbidity if untreated. Early recognition and diagnosis of TIO is crucial since resection of the tumor usually reverses its manifestations.

肿瘤诱发的骨软化症(TIO)是一种继发于肿瘤的疾病,患者会出现骨软化症的临床症状和体征,以及低磷血症、磷酸盐血症和血清中 1,25(OH)2 维生素 D3 水平低下等生化异常。一名 33 岁女性出现肌肉骨骼疼痛和近端肌病,病程 2.5 年,曾服用维生素 D 补充剂治疗。根据生化检查和组织病理学检查,她接受了重新评估,发现她患有继发于磷酸盐间质瘤的 TIO。肿瘤被切除(用内假肢进行肢体修复),随访时她没有疼痛或无力感。本病例提醒读者,在评估孤立性低磷血症患者时应考虑到 TIO 的可能性,如果不及时治疗,可能会导致长期残疾和长期发病。早期识别和诊断 TIO 至关重要,因为切除肿瘤通常可以逆转其表现。
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引用次数: 0
Giant Renal Angiomyolipomas and Pulmonary Lymphangioleiomyomatosis: Follow-up Report after More than a Decade. 巨型肾血管肌脂肪瘤和肺淋巴管瘤病:十多年后的随访报告
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-11-01 Epub Date: 2024-05-09 DOI: 10.4103/sjkdt.sjkdt_324_21
Kiran Nasir, Aasim Ahmad

Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are two major presentations of tuberous sclerosis (TS), an autosomal dominant multisystem disorder. Renal AMLs can lead to life-threatening complications like hemorrhage and cause progressive renal failure requiring dialysis and kidney transplant. mTOR inhibitors showed promising results in TS patients with renal AMLs, LAM, and subependymal giant cell astrocytomas. This case report is a follow-up of a patient we reported in 2010 with giant AMLs and LAM who required an emergency nephrectomy for massive hemorrhage from giant left-sided AMLs.

肾血管肌脂肪瘤(AML)和肺淋巴管瘤病(LAM)是结节性硬化症(TS)的两种主要表现形式,TS是一种常染色体显性多系统疾病。mTOR抑制剂在患有肾AML、LAM和脐下巨细胞星形细胞瘤的TS患者中显示出良好的疗效。本病例报告是我们在 2010 年报告的一名巨型 AML 和 LAM 患者的随访报告,该患者因左侧巨型 AML 大出血而需要进行紧急肾切除术。
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引用次数: 0
Individualization of the Dosage Regimen of Erythropoietin is Crucial in End-stage Renal Disease Patients. 促红细胞生成素剂量方案的个体化对终末期肾病患者至关重要
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-09-01 Epub Date: 2024-03-11 DOI: 10.4103/1319-2442.397212
M K Krishnapriya, S Karthika, Anisha Babu, Antriya Annie Tom

The objective of this study was to understand the utilization pattern of erythropoietin in end-stage renal disease patients, along with the effect of body weight and sex on the patients' responses. In this retrospective single-center study, 120 patients were included who were on a once weekly (n = 79), twice weekly (n = 37), or thrice weekly (n = 4) regimen. The doses of erythropoiesis-stimulating agents (ESA) were collected, and the erythropoietin resistance index (ERI) was determined. The Kruskal-Wallis test was used to evaluate the dose schedules, and the once-weekly regimen produced a greater response (P = 0.001). The asymptotic significance of Pearson's Chi-square-test equating the mean ERI and body mass index (BMI) was 0.034. No statistically significant correlation was estimated between sex and mean ERI (P = 0.201). Our study demonstrated that the once-weekly regimen dominated over the others in terms of efficacy, and individuals with a higher BMI were found to respond better to the ESA therapy.

本研究旨在了解终末期肾病患者使用促红细胞生成素的模式,以及体重和性别对患者反应的影响。在这项回顾性单中心研究中,共纳入了 120 名患者,他们分别采用每周一次(79 人)、每周两次(37 人)或每周三次(4 人)的治疗方案。研究人员收集了红细胞生成刺激剂(ESA)的剂量,并测定了红细胞生成素抵抗指数(ERI)。采用 Kruskal-Wallis 检验来评估剂量方案,结果显示每周一次的方案产生的反应更大(P = 0.001)。对平均 ERI 和体重指数(BMI)进行的 Pearson's Chi-square 检验的渐近显著性为 0.034。据估计,性别与平均 ERI 之间没有统计学意义上的相关性(P = 0.201)。我们的研究表明,每周一次的治疗方案在疗效方面优于其他方案,而且体重指数越高的人对 ESA 治疗的反应越好。
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引用次数: 0
The Relationship between Serum TWEAK Levels and Carotid Intima-media Thickness in Patients with Fabry Disease. 法布里病患者血清 TWEAK 水平与颈动脉内膜中层厚度之间的关系
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-09-01 Epub Date: 2024-03-11 DOI: 10.4103/1319-2442.397202
Kultigin Turkmen, Ismail Baloglu, Talat Aykut, Salih Demir, Ebru Altın, Zeynep Aybike Akguzel, Muhammet Kocabas, Fatma Humeyra Yerlikaya

Fabry disease (FD) is associated with inflammation, proteinuria, and chronic kidney disease. Tumor necrosis factor-like weak inducer of apoptosis (TWEAK) plays an important role in inflammation in diabetic nephropathy and lupus nephritis. Since there is a close relationship linking serum TWEAK (sTWEAK), inflammation, and carotid intima-media thickness (CIMT) in various kidney diseases, we aimed to determine the relationship between sTWEAK levels and CIMT in subjects with and without proteinuria in a cross-sectional study involving 15 FD patients (seven females, eight males) and seven healthy controls (four females, three males). There were no differences in age, sex, estimated glomerular filtration rate, and biochemical parameters (serum glucose, albumin, creatinine, uric acid, C-reactive protein (CRP), low-density lipoprotein, and high-density lipoprotein) between FD patients and healthy controls. The spot urine protein-creatinine ratios of healthy controls and FD patients were 90 mg/g and 185 mg/g, respectively (P = 0.022). STWEAK levels were higher in FD patients than in healthy controls (P = 0.007). The CIMT of FD patients and healthy controls was 0.55 ± 0.14 mm and 0.42 ± 0.04 mm, respectively (P = 0.007). STWEAK was positively correlated with CRP and CIMT, and negatively with proteinuria (P = 0.005, P = 0.013, and P = 0.018, respectively). In the multivariate analysis, only sTWEAK was an independent variable of increased CIMT. We demonstrated that sTWEAK and CIMT were increased in FD patients. STWEAK might have a role in the pathogenesis of subclinical atherosclerosis in FD.

法布里病(FD)与炎症、蛋白尿和慢性肾病有关。肿瘤坏死因子样细胞凋亡弱诱导因子(TWEAK)在糖尿病肾病和狼疮肾炎的炎症中发挥着重要作用。由于血清 TWEAK(sTWEAK)、炎症和各种肾脏疾病中的颈动脉内膜中层厚度(CIMT)之间存在密切联系,因此我们在一项横断面研究中测定了 15 名 FD 患者(7 名女性,8 名男性)和 7 名健康对照组(4 名女性,3 名男性)中有蛋白尿和无蛋白尿者的 sTWEAK 水平与 CIMT 之间的关系。FD 患者和健康对照组在年龄、性别、估计肾小球滤过率和生化指标(血清葡萄糖、白蛋白、肌酐、尿酸、C 反应蛋白(CRP)、低密度脂蛋白和高密度脂蛋白)方面没有差异。健康对照组和 FD 患者的定点尿蛋白-肌酐比率分别为 90 毫克/克和 185 毫克/克(P = 0.022)。FD患者的STWEAK水平高于健康对照组(P = 0.007)。FD患者和健康对照组的CIMT分别为0.55 ± 0.14 mm和0.42 ± 0.04 mm(P = 0.007)。STWEAK 与 CRP 和 CIMT 呈正相关,与蛋白尿呈负相关(分别为 P = 0.005、P = 0.013 和 P = 0.018)。在多变量分析中,只有 sTWEAK 是 CIMT 增加的独立变量。我们证实,sTWEAK 和 CIMT 在 FD 患者中均有所增加。STWEAK 可能在 FD 亚临床动脉粥样硬化的发病机制中发挥作用。
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引用次数: 0
Risk Factors of Anxiety among Hemodialyzed Patients in Ténès, Algeria: A Cross-Sectional Study. 阿尔及利亚泰内斯血液透析患者焦虑的风险因素:一项横断面研究
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-09-01 Epub Date: 2024-03-11 DOI: 10.4103/1319-2442.397205
Amina Bekara, Mohammed El Amine Bekara, Lallia Amara, Nassima Amamra

This study aimed to evaluate the level of anxiety and its risk factors among patients undergoing hemodialysis in the Ténès area, Chlef Province, Algeria. The survey was conducted on 45 hemodialyzed patients at the public hospital of Ténès (Ahmed Bourass). Sociodemographic data were collected using a questionnaire, and anxiety was diagnosed by the hospital anxiety and depression scale. Among 45 patients, 44.44% were anxious. Anxiety was significantly associated with profession and antidepressant treatment (P <0.05). The other factors were insignificantly related to anxiety, such as age, sex, education level, and comorbidities. Anxiety is prevalent among end-stage renal disease patients. Unemployment and medications were identified as the main risk factors. Multidisciplinary strategies need to be established to prevent the occurrence of anxiety and improve patients' quality of life.

本研究旨在评估阿尔及利亚 Chlef 省 Ténès 地区血液透析患者的焦虑程度及其风险因素。调查对象是泰内斯(Ahmed Bourass)公立医院的 45 名血液透析患者。通过问卷调查收集了社会人口学数据,并通过医院焦虑和抑郁量表对焦虑进行了诊断。在 45 名患者中,44.44% 患有焦虑症。焦虑与职业和抗抑郁治疗明显相关(P
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引用次数: 0
Effectiveness of Supramaximal Angiotensin-converting Enzyme Inhibition in Controlling Proteinuria in Congenital Nephrotic Syndrome with Cytomegalovirus Infection and an NPHS1 Mutation. 超大剂量血管紧张素转换酶抑制剂对巨细胞病毒感染和 NPHS1 基因突变的先天性肾病综合征患者控制蛋白尿的有效性
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2023-09-01 Epub Date: 2024-03-11 DOI: 10.4103/1319-2442.397210
Nimisha Dange, Kiran P Sathe, Alpana Kondekar, Vishal Sawant

Congenital nephrotic syndrome (NS) is characterized by early-onset heavy proteinuria. Most cases of congenital NS are associated with genetic mutations in the podocyte proteins. The causal relationship of perinatal infections with congenital NS has not yet been proven. Inadequate response to the treatment of such infections should prompt us to conduct genetic testing for congenital NS. The heavy proteinuria associated with congenital NS is usually difficult to control with conventional treatment. It often results in progressive kidney disease with a high risk of mortality in early life. Here, we describe an infant who developed congenital NS and was found to have a coexisting Cytomegalovirus infection and an underlying NPSH1 mutation. Proteinuria did not respond to a standard dose of enalapril. A supramaximal dose of enalapril was tried and was effective and safe in controlling the proteinuria. It was associated with improved growth, complete resolution of edema, normal serum albumin, and normal renal function beyond 2 years of age.

先天性肾病综合征(NS)以早发重型蛋白尿为特征。大多数先天性肾病综合征病例与荚膜蛋白质的基因突变有关。围产期感染与先天性 NS 的因果关系尚未得到证实。对此类感染的治疗效果不佳,应促使我们对先天性 NS 进行基因检测。与先天性 NS 相关的大量蛋白尿通常很难通过常规治疗得到控制。它通常会导致进行性肾脏疾病,在生命早期有很高的死亡风险。在此,我们描述了一名患先天性NS的婴儿,发现他同时患有巨细胞病毒感染和潜在的NPSH1基因突变。蛋白尿对标准剂量的依那普利没有反应。他尝试了超大剂量的依那普利,结果有效而安全地控制了蛋白尿。该疗法改善了患儿的生长发育,完全消除了水肿,使其血清白蛋白正常,并在患儿两岁后使其肾功能恢复正常。
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引用次数: 0
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Saudi Journal of Kidney Diseases and Transplantation
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