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Clinicopathological Features of Lupus Nephritis Patients in North-East India; A Single Center Retrospective Observational Study 印度东北部狼疮性肾炎患者的临床病理特征单中心回顾性观察研究
Pub Date : 2021-12-10 DOI: 10.15586/jrenhep.v6i1.130
S. Alam, M. Parry, Manjuri Sharma, H. Jeelani, M. Mazumder
Clinicopathological presentation of lupus nephritis (LN) patients varies with different race and ethinicity of the population. Only few studies describe clinicopathological spectrum of LN patients in the Indian population. The aim of this study was to determine the clinicopathological spectrum of LN in the North-East Indian population. This was a retrospective observational study that included patients with LN at a tertiary care center in North-East India from March 2007 to August 2018. Clinical and histopathological data at the time of presentation were collected from hospital records. Renal biopsies were examined by light microscopy and direct immunofluorescence techniques. A total of 340 patients of LN were included in this study. The mean age of presentation was 22.42 ± 4.3 years. The minimum age at presentation was 8 years and 18.8% belonged to the <18 year age group. The present study showed a male:female ratio of 1:8. The majority of patients were of class IV (71.9%). Arthralgia (47.1%) and anemia (60.3%) were the most common presenting symptom and sign, respectively. Immunoglobulin (Ig) G was the most abundant immunoglobulin (positive in 98.47%) and the least positive was IgA (positive in 41.18%). Complement (C) 3 and C1q were positive in all. Full house deposition was found in 59.3% of the biopsies. The rates of hypertension, microscopic hematuria, renal dysfunction, and nephrotic syndrome were 43.5, 59.12, 45.9, and 35.3%, respectively. Patients of LN in the North-East Indian population present at an earlier age with a more severe form of the disease (class IV) at the time of presentation.
狼疮性肾炎(LN)患者的临床病理表现随人种和民族的不同而不同。只有少数研究描述了LN患者在印度人口的临床病理谱。本研究的目的是确定LN在东北印度人口的临床病理谱。这是一项回顾性观察性研究,纳入了2007年3月至2018年8月在印度东北部一家三级医疗中心就诊的LN患者。发病时的临床和组织病理学资料收集自医院记录。肾活检采用光镜和直接免疫荧光技术检查。本研究共纳入340例LN患者。平均发病年龄为22.42±4.3岁。最小发病年龄为8岁,其中<18岁年龄组占18.8%。本研究显示男女比例为1:8。大多数患者为IV级(71.9%)。关节痛(47.1%)和贫血(60.3%)分别是最常见的症状和体征。免疫球蛋白(Ig)中G含量最多(阳性98.47%),IgA最少(阳性41.18%)。补体(C) 3和C1q均阳性。59.3%的活检发现全屋沉积。高血压、显微镜下血尿、肾功能不全和肾病综合征的发生率分别为43.5%、59.12%、45.9%和35.3%。印度东北部LN患者发病年龄较早,发病时病情较严重(IV级)。
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引用次数: 0
Kidney Injury in Children Infected with HIV, Followed at the Teaching Hospital of Borgou (Benin) 贝宁博古教学医院HIV感染儿童肾损伤研究
Pub Date : 2021-10-26 DOI: 10.15586/jrenhep.v5i2.120
S. Ahoui, F. Agbeille, G. Kpanidja, A. Noudamadjo, Toutche Bruno Leopold Agboton, E. Eteka, J. Vigan, J. Adédémy, J. Agossou
The history of kidney disease associated with HIV infection dates back to the years of HIV breakthrough. The objective was to study kidney damage in children infected with HIV at the Teaching Hospital of Borgou (Benin) in 2019. This was a cross-sectional, descriptive, analytical, matching-type study carried out from June 1, 2019 to September 30, 2019 at the pediatrics department of Teaching Hospital of Borgou (Benin). The study included HIV-positive children, followed in consultations, and whose parents gave their consent. The biological markers were demon-strated with urine dipstick. Glomerular filtration rate was calculated using the Schwartz test and classified according to stages. The dependent variable was the presence of at least one impairment (biological or functional). Sample size was determined by Schwartz’s method on the basis of one case for two controls. Sociodemographic, clinical, biological, and therapeutic data were collected. Comparisons were made using the Chi-square test or Fisher’s exact test. The identification of associated factors was possible using a multiple logistic regression model at 5% threshold. In total, we included 117 children, including 39 HIV-positive children. The average age was 8 ± 4.81 years and the gender ratio was 1:17. The frequency of kidney damage was 76.5%. Permanent proteinuria and at least two crosses on urine dipstick were present in 20.5%, leukocyturia in 2.6%, and proximal tubular dysfunction in 5.1%. Glomerular hyperfiltration was found in 38.5%, acute kidney injury in 38.5%, and chronic kidney injury in 5.1%. Associated factors were age (P = 0.004), presence of opportunistic infections (P = 0.00), and treatment adherence (P = 0.004). Kidney damage is common in HIV-positive children. Careful follow-up is necessary to avoid complications.
与艾滋病毒感染相关的肾脏疾病的历史可以追溯到艾滋病毒突破的年代。目的是研究2019年在博古教学医院(贝宁)感染艾滋病毒的儿童的肾脏损害。这是一项横断面、描述性、分析性、匹配型研究,于2019年6月1日至2019年9月30日在贝宁Borgou教学医院儿科进行。这项研究包括艾滋病毒阳性的儿童,经过协商,并得到父母的同意。用尿试纸检测生物标志物。使用Schwartz试验计算肾小球滤过率并按分期进行分类。因变量是至少存在一种损伤(生物或功能)。样本量由Schwartz方法确定,基于一病例两对照。收集了社会人口学、临床、生物学和治疗数据。比较采用卡方检验或费雪精确检验。使用5%阈值的多元逻辑回归模型可以识别相关因素。我们总共纳入了117名儿童,其中包括39名艾滋病毒阳性儿童。平均年龄8±4.81岁,性别比为1:17。肾损害发生率为76.5%。永久性蛋白尿和尿试纸上至少两个十字出现20.5%,白细胞尿2.6%,近端小管功能障碍5.1%。肾小球高滤过发生率为38.5%,急性肾损伤发生率为38.5%,慢性肾损伤发生率为5.1%。相关因素为年龄(P = 0.004)、机会性感染(P = 0.00)和治疗依从性(P = 0.004)。肾脏损伤在hiv阳性儿童中很常见。仔细的随访是必要的,以避免并发症。
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引用次数: 1
Orthotopic Liver Transplantation in a Case of Novel YAP1-TFE3 Hepatic Epithelioid Hemangioendothelioma [HEHE] 新型YAP1-TFE3肝上皮样血管内皮瘤原位肝移植1例
Pub Date : 2021-10-14 DOI: 10.15586/jrenhep.v5i2.122
Sugi Srv, Elankumaran Krishnan, K. Haribabu, Harintharavimal Balakrishnan, Shantha Ravishankar
Epithelioid hemangioendothelioma is an extremely rare vascular neoplasm arising in soft-tissues and different visceral organs, with liver being the most commonly involved viscera. Hepatic epithelioid hemangioendothelioma (HEHE) is a malignant tumour with an indolent behaviour and unpredictable clinical course. It has a better prognosis among the malignant tumours of the liver, in spite of being a diffuse multifocal liver disease or metastatic at the time of presentation. HEHE is usually found to be noted in the fifth decade with slight female preponderance. No single treatment strategy has yet been established owing to its variable clinical course, ranging from an indolent tumour with prolonged survival to an aggressive, metastatic disease with a fatal outcome. Here, we present a case of a novel HEHE in a 25-year-old female who was treated successfully with orthotopic living donor liver transplantation and discuss the presentation, histopathology and management of this rare, fatal yet treatable malignant tumour.
上皮样血管内皮瘤是一种极为罕见的血管性肿瘤,多发于软组织和不同内脏器官,以肝脏为最常累及的脏器。肝上皮样血管内皮瘤(HEHE)是一种恶性肿瘤,具有惰性和不可预测的临床过程。它在肝脏恶性肿瘤中预后较好,尽管在表现时是弥漫性多灶性肝病或转移性疾病。HEHE通常出现在50岁左右,女性略占优势。由于其多变的临床病程,从无痛的肿瘤到具有致命结果的侵袭性转移性疾病,目前还没有确定单一的治疗策略。在此,我们报告一例25岁女性的新型HEHE,她成功地接受了原位活体肝移植治疗,并讨论了这种罕见、致命但可治疗的恶性肿瘤的表现、组织病理学和治疗。
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引用次数: 0
A Neglected Case of Wilson’s Disease Presenting as Symptomatic Urolithiasis and Proteinuria 以症状性尿石症及蛋白尿为表现的威尔逊氏病一例被忽视
Pub Date : 2021-10-07 DOI: 10.15586/jrenhep.v5i2.123
E. Zare, Zahra Mahbubi, M. Panahi
We report a short-statured, 39-year-old male presenting with recurrent kidney stones, history of refractory rickets, and bone deformity. He had been consuming multiple doses of calcium supplements and multiple courses of vitamin D over past 30 years beforeprior to reporting in our clinic without any significant laboratory or clinical improvement. The patient was diagnosed as having Fanconi’s syndrome attributable to Wilson’s disease. This patient highlighted that in case of resistant rickets, a high index of uncertainty must be invoked for Wilson’s disease. Appropriate timely recognition of this entity results in prompt ministrations and prevention of disability. We also presented and discussed reviews on Wilson’s disease from literature.
我们报告一位身材矮小的39岁男性,表现为复发性肾结石、难治性佝偻病和骨畸形。在我们的诊所报告之前,他在过去的30年里一直在服用多剂量的钙补充剂和多疗程的维生素D,没有任何明显的实验室或临床改善。患者被诊断为可归因于威尔逊病的范可尼综合征。该患者强调,在耐药佝偻病的情况下,威尔森氏病必须采用高不确定性指数。适当及时地认识到这一实体可以迅速提供服务和预防残疾。我们还从文献中介绍和讨论了威尔逊氏病的综述。
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引用次数: 1
The Role of the Liver in Iron Homeostasis and What Goes Wrong? 肝脏在铁稳态中的作用和哪里出了问题?
Pub Date : 2021-09-18 DOI: 10.15586/jrenhep.v5i2.110
Ernesto S. Robalino Gonzaga, I. R. Guiance, R. Henriquez, G. Mortimore, J. Freeman
Iron is an essential mineral that is vital for growth development, normal cellular function, synthesis of hormones and connective tissue, and most importantly, serves as a component of hemoglobin to carry oxygen to body tissues. The body finely regulates the amount of circulating and stored iron within the body to maintain concentration levels within range for optimal physiologic function. Without iron, the ability for cells to participate in electron transport and energy metabolism decreases. Furthermore, hemoglobin synthesis is altered, which leads to anemia and decreased oxygen delivery to tissue. Problems arise when there is too little or too much iron. This review explores the role of the liver in iron physiology, iron overload and discusses the most common causes of primary and secondary hepatic iron overload.
铁是一种必需的矿物质,对生长发育、正常细胞功能、激素合成和结缔组织至关重要,最重要的是,它是血红蛋白的组成部分,将氧气输送到身体组织。人体精细地调节体内循环和储存铁的量,以维持最佳生理功能的浓度水平。没有铁,细胞参与电子传递和能量代谢的能力就会下降。此外,血红蛋白合成被改变,导致贫血和组织供氧减少。铁过少或过多都会产生问题。这篇综述探讨了肝脏在铁生理和铁超载中的作用,并讨论了原发性和继发性肝铁超载的最常见原因。
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引用次数: 1
Hepatic Vein and Inferior Vena Cava Reconstruction during Hepatic Surgery Resection for Cancer 肝癌肝手术切除中肝静脉及下腔静脉重建
Pub Date : 2021-09-09 DOI: 10.15586/jrenhep.v5i2.113
D. Coco, S. Leanza
Invasion of tumor in the liver requires surgical interventions that may reduce the effects or may eliminate the tumor-affected cells. The renewal of the hepatic vein and inferior vena cava has enabled most specialized oncologists and medical specialists to use advanced diagnostic methods in the treatment of the liver tumors. Liver resection has prolonged the lives of many patients and the invention of live donor organ transplants has effectively enabled the use of liver resection in most cancer centers across the world. By reviewing data from 10 articles, 21 surgical analyses were investigated and analyzed for the risks involved in the applications of reconstructions of hepatic vein and inferior vena cava in the surgical liver resection. The postoperative complications and the indications of reconstructions were mentioned. The results indicated that with these surgical procedures, complications are still involved but may be successful for particular patients.
肝脏肿瘤的侵袭需要手术干预,这可能会减少影响或可能会消除肿瘤影响的细胞。肝静脉和下腔静脉的更新使大多数专业肿瘤学家和医学专家能够在治疗肝肿瘤时使用先进的诊断方法。肝切除术延长了许多患者的生命,活体供体器官移植的发明有效地使肝切除术在世界上大多数癌症中心得以使用。通过回顾10篇文献的资料,我们对21例外科分析进行了调查和分析,以探讨肝静脉和下腔静脉重建在外科肝切除术中的应用所涉及的风险。并对术后并发症及重建指征进行了讨论。结果表明,这些外科手术仍然涉及并发症,但对于特定患者可能是成功的。
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引用次数: 1
Sewing Needle as Foreign Body in Urethra of an Adolescent Boy 缝衣针作为异物进入青春期男孩尿道
Pub Date : 2021-09-08 DOI: 10.15586/jrenhep.v5i2.119
K. Bangash, Muhammad Naeem, H. Mumtaz, Aaliah Akhtar Hayat, Mubashar Mazhar, Shahzaib Ahmad
Self-insertion of a foreign body in the urethra is an uncommon presentation clinically. The cases usually arise due to fulfillment of sexual desire, for recreation, play, or exploration, or the foreign body insertion may take place accidentally. We present a case of an adolescent boy with a foreign body urethra presenting to the emergency room with urinary retention, pain, and dysuria. Attending urologist suspected urethral stricture and ordered ultrasonography to investigate which turned out to be a sewing needle in his urethra. The patient was then enquired about the foreign body. He tried to self-dilate his urethra as he was experiencing lower urinary tract symptoms. The sewing needle was removed by endoscopy and he was administered with antibiotics and painkillers. The urethral foreign bodies may present with pain, dysuria, or urinary incontinence and these foreign bodies are mostly seen in the male population in the adolescent age group.
摘要异物自插入尿道是临床上罕见的表现。这种情况通常是由于满足性欲、娱乐、玩耍或探索,或意外发生异物插入。我们提出一个病例的青春期男孩与异物尿道呈现到急诊室尿潴留,疼痛和排尿困难。主治泌尿科医生怀疑是尿道狭窄,并安排超声检查,结果发现是一根缝衣针扎进了他的尿道。然后向病人询问异物的情况。由于出现下尿路症状,他试图自行扩张尿道。内窥镜检查取出了缝衣针,并给他注射了抗生素和止痛药。尿道异物可表现为疼痛、排尿困难或尿失禁,这些异物多见于青少年男性人群。
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引用次数: 0
Comparison of Standard Percutaneous Nephrolithotomy with Mini-Percutaneous Nephrolithotomy for Removal of Renal Stones in Adults 标准经皮肾镜取石术与微型经皮肾镜取石术治疗成人肾结石的比较
Pub Date : 2021-09-08 DOI: 10.15586/jrenhep.v5i2.118
A. Alam Khan, I. Malkani, Junaid Jameel Khattak, H. Mumtaz, Mubashir Mazhar, F. Naz, Arsalan Riaz
Renal stones are the third most common problem affecting about 10% of global population. The management of nephrolithiasis has undergone a complete transformation since the 1980s. Percutaneous nephrolithotomy (PCNL) has established itself an effective and safe technique that delivers high stone-free rate as well as overall shorter treatment time. We aim to compare the outcome of mini-PCNL with standard-PCNL in patients presenting with renal stones. In all, 90 patients fulfilled the selection criteria and randomized into two groups. Group A underwent mini-PCNL whereas Group B underwent standard-PCNL. Pre-operative hemoglobin level was recorded. Duration of procedure as well as drop in hemoglobin level was also recorded. A kidney, ureter, and bladder (KUB) X-ray was performed to confirm the presence of of stone and stone-free status. The mean age of patients in mini-PCNL group was 43.11 years and in standard-PCNL group, it was 36.91 years. The mean stone size in patients of mini-PCNL group was 29.53 mm and 31.58 mm in standard-PCNL group. The mean duration of renal stone in mini-PCNL group was 1.91 years and that in standard-PCNL group 1.80 years. The mean operative time in mini-PCNL group was 59.56 min and 61.22 min in standard-PCNL group. The mean fall in hemoglobin in mini-PCNL group was 0.38 g/dL and that in standard-PCNL group 0.51 g/dL. In mini-PCNL group, stone clearance was observed in 42 (93.3%) patients, while in standard-PCNL group, it was observed in 45 (100%) patients. This difference was insignificant (P > 0.05). Mini-PCNL and standard-PCNL have no significant differences in terms of outcome, operative time, and stone clearance, although fall in hemoglobin level was less in mini-PCNL group, which showed less blood loss in this group, thereby making it a more appropriate method for renal stone removal. 
肾结石是影响全球约10%人口的第三大常见问题。自20世纪80年代以来,肾结石的治疗经历了彻底的转变。经皮肾镜取石术(PCNL)是一种有效且安全的技术,具有高结石去除率和较短的治疗时间。我们的目的是比较迷你pcnl和标准pcnl在肾结石患者中的预后。共有90例患者符合选择标准,随机分为两组。A组行迷你pcnl, B组行标准pcnl。记录术前血红蛋白水平。同时记录手术时间和血红蛋白水平的下降。行肾、输尿管和膀胱(KUB) x线检查以确认结石和无结石的存在。迷你pcnl组患者平均年龄为43.11岁,标准pcnl组患者平均年龄为36.91岁。迷你pcnl组患者的平均结石大小为29.53 mm,标准pcnl组患者的平均结石大小为31.58 mm。迷你pcnl组肾结石的平均持续时间为1.91年,标准pcnl组为1.80年。迷你pcnl组平均手术时间为59.56 min,标准pcnl组平均手术时间为61.22 min。迷你pcnl组血红蛋白平均下降0.38 g/dL,标准pcnl组血红蛋白平均下降0.51 g/dL。mini-PCNL组有42例(93.3%)患者结石清除,而标准pcnl组有45例(100%)患者结石清除。差异不显著(P > 0.05)。Mini-PCNL组与标准pcnl组在预后、手术时间、结石清除等方面无显著差异,但Mini-PCNL组血红蛋白水平下降较少,出血量较少,是更合适的肾结石清除方法。
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引用次数: 1
Metabolic Syndrome in Adults with Nonalcoholic Fatty Liver Disease 成人非酒精性脂肪性肝病的代谢综合征
Pub Date : 2021-09-01 DOI: 10.15586/jrenhep.v5i2.99
Zhahid Hassan, M. Latief, Mahroosa Ramzan, F. Abbas, Summyia Farooq
Nonalcoholic fatty liver disease (NAFLD) is associated with insulin resistance, obesity, and other features of metabolic syndrome. It is identified as the most common cause of liver enzyme derangement. Lately, NAFLD has generated interest in exploring treatment options, including weight loss and dietary interventions. An association of NAFLD with metabolic syndrome has been suggested in contemporary literature. In this study, we attempted to look into the association of NAFLD with metabolic syndrome. In this study, 80 adult NAFLD patients were recruited from a tertiary care hospital. Among these, 42 were males and 38 females with a mean age of 44.46±13.146 years (range 18–82 years). Grades of fatty liver and presence or absence of metabolic syndrome were studied in this patient population. Patients who did not qualify for the criteria of met-abolic syndrome were placed in Group 1 and those who fulfilled the stated criteria were considered in Group 2. There were 29 (36.25%) patients in Group 1 and 51 (63.75%) in Group 2. All the patients in Group 1 were having Grade I fatty liver whereas patients in Group 2 were found to having varying grades of fatty liver, with six patients having Grade III fatty liver. We found statistically significant difference in various parameters of study (liver enzymes, high-density lipoprotein (HDL), triglycerides, and blood pressure) between Group 1 and Group 2. Ultrasound evidence of a fatty liver should be considered as a predictor of metabolic syndrome, and these patients must be investigated for the different components of metabolic syndrome so as to have early diagnosis and intervention to alter development of long-term metabolic disorders and their inherent complications.
非酒精性脂肪性肝病(NAFLD)与胰岛素抵抗、肥胖和代谢综合征的其他特征相关。它被认为是肝酶紊乱的最常见原因。最近,NAFLD引起了人们对探索治疗方案的兴趣,包括减肥和饮食干预。NAFLD与代谢综合征的关联已在当代文献中提出。在这项研究中,我们试图探讨NAFLD与代谢综合征的关系。在这项研究中,80名成年NAFLD患者从三级护理医院招募。其中男性42例,女性38例,平均年龄44.46±13.146岁(18 ~ 82岁)。在该患者群体中研究了脂肪肝分级和有无代谢综合征。不符合代谢综合征标准的患者被分为第一组,符合上述标准的患者被分为第二组。1组29例(36.25%),2组51例(63.75%)。1组患者均为I级脂肪肝,2组患者有不同程度的脂肪肝,其中6例为III级脂肪肝。我们发现组1和组2在各种研究参数(肝酶、高密度脂蛋白(HDL)、甘油三酯和血压)上有统计学差异。脂肪肝的超声证据应被视为代谢综合征的预测因素,必须对这些患者进行调查,了解代谢综合征的不同组成部分,以便早期诊断和干预,以改变长期代谢紊乱及其固有并发症的发展。
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引用次数: 1
Automobile Paint Reducer Induced Acute Kidney Injury 汽车油漆减色剂致急性肾损伤
Pub Date : 2021-08-18 DOI: 10.15586/jrenhep.v5i2.116
Muzamil Latief, Zhahid Hassan, Mohd Latief Wani, F. Abbas, Summyia Farooq
The various aspects of the automobile industry also carry with it the risk for occupational health hazards with it. Toluene has also evolved as a commonly used drug by substance abusers. Accidental exposure or self-poisoning with these substances has been reported in literature. These substances can also cause distal renal tubular acidosis (RTA), acute tubular necrosis, glomerulonephritis and interstitial nephritis, rhabdomyolysis and myoglobinemia.In this series, we report about three patients who developed renal manifestations because of organic solvents. Two of the three patients had ingested the paint reducer substance and the third one was addicted to sniffing the toluene based paint reducer. All the patients had in taken these substances with suicidal intent and developed acute kidney injury (AKI) and severe metabolic acidosis. One of the patients had features of rhabdomyolysis as well. The third patient was a substance abuser and had inhaled higher than usual dose and developed severe and refractory acidosis and mild kidney injury and required Renal Replacement Therapy (RRT) for acidosis. All the patients eventually recovered their kidney functions and were doing well during their follow-up.Toluene based organic solvents lead to acute neurological symptoms, accompanied by severe metabolic alterations, organ injury and dysfunc-tion. An association of the development of hypokalemic paralysis and metabolic acidosis with toluene intoxication has been observed. The management of acute toluene toxicity is mainly conservative, consisting of electrolytes correction, acid-base and fluid abnormalities and renal replacement therapy in severe AKI.Organic solvent exposure may result in acute tubular necrosis, rhabdomyolysis, RTA and AKI irrespective of the intake route. Clinical suspicion of organ dysfunction and failure and timely induction of supportive care leads to a good outcome.
汽车工业的各个方面也伴随着职业健康危害的风险。甲苯也逐渐成为药物滥用者常用的药物。这些物质的意外暴露或自我中毒已在文献中报道。这些物质还可引起远端肾小管酸中毒(RTA)、急性肾小管坏死、肾小球肾炎和间质性肾炎、横纹肌溶解和肌红蛋白血症。在这个系列中,我们报告了三例因有机溶剂而出现肾脏症状的患者。三名患者中有两名摄入了降漆剂物质,第三名患者对吸入甲苯类降漆剂成瘾。所有患者均有自杀倾向,并发生急性肾损伤(AKI)和严重代谢性酸中毒。其中一名患者也有横纹肌溶解的特征。第三例患者是药物滥用者,吸入剂量高于正常水平,出现严重难治性酸中毒和轻度肾损伤,需要肾替代治疗(RRT)。所有患者最终都恢复了肾功能,并在随访期间表现良好。甲苯基有机溶剂可导致急性神经系统症状,并伴有严重的代谢改变、器官损伤和功能障碍。低钾性麻痹和代谢性酸中毒的发展与甲苯中毒的关系已被观察到。急性甲苯毒性的处理主要是保守的,包括电解质纠正、酸碱和液体异常以及严重AKI的肾脏替代治疗。有机溶剂暴露可导致急性肾小管坏死、横纹肌溶解、RTA和AKI,与摄入途径无关。临床怀疑器官功能障碍和衰竭并及时诱导支持性护理可获得良好的结果。
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引用次数: 0
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Journal of Renal and Hepatic Disorders
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