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Primary aldosteronism without hypertension:A case report and review of the literature 无高血压的原发性醛固酮增多症:病例报告和文献综述
Pub Date : 2023-12-20 Epub Date: 2023-12-11 DOI: 10.1684/ndt.2023.53
Yves Dimitrov, Julien Ott, Lisa Beguin, Brieuc Muller

We describe the case of a patient who presented with hyperaldosteronism without arterial hypertension. She had been referred for consultation for persistent severe hypokalaemia despite oral KCl supplementation. The absence of hypertension had been proven by repeated clinical measurements and by ABPM. Hyperaldosteronism had been demonstrated by hormonal assays and catheterization of the adrenal veins. Abdominal CT revealed a left adrenal adenoma. Finally, the anatomopathological examination of the surgical specimen confirmed the adenoma. After the intervention, serum potassium normalized. The clinical case is completed by a review of the literature of hyperaldosteronisms without arterial hypertension.

我们描述了一名患有高醛固酮症但无动脉高血压的患者的病例。她因口服氯化钾补充剂后仍持续出现严重低钾血症而转诊。反复的临床测量和 ABPM 证明她没有高血压。通过激素测定和肾上腺静脉导管检查,证实了高醛固酮血症。腹部 CT 显示左侧肾上腺腺瘤。最后,手术标本的解剖病理学检查证实了腺瘤的存在。介入治疗后,血清钾恢复正常。本临床病例通过对无动脉高血压的高醛固酮症文献的回顾而得以完善。
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引用次数: 0
Integrated clinical pharmacy activities into the pediatric kidney graft pathway 将临床药学活动纳入儿童肾移植途径
Pub Date : 2023-12-20 Epub Date: 2023-12-07 DOI: 10.1684/ndt.2023.56
Julie Martin, Élise Jandot, Valentine Bréant, Anne-Laure Sellier-Leclerc, Christelle Mouchoux, Claire Lattard, Justine Bacchetta, Magali Larger, Xavier Dode, Delphine Hoegy

The pediatric renal graft pathway is at risk of care discontinuation, even though therapeutic adherence is essential. The objective is to evaluate the integration of clinical pharmacy activities into this care pathway. This feasibility study is divided into three stages: structuring, implementing and evaluation. In pre-transplant, immediate and remote post-transplant, interviews were proposed as well as the pharmaceutical analysis of medication prescriptions. In 8 months duration, 32 patients were included. All patients included in pre-transplant and immediate post-transplant benefited from the activities. At M0, all the prescriptions analyzed resulted in at least one problem detected. Half of the transplanted patients benefited from M1 maintenance, one patient from M3 maintenance and no M6 follow-up could be carried out. This work concludes with the good feasibility and integration of clinical pharmacy activities within the care pathway.

儿童肾移植途径存在治疗中断的风险,即使治疗依从性是必不可少的。目的是评估临床药学活动整合到这一护理途径。本可行性研究分为构建、实施和评估三个阶段。在移植前、即刻和移植后远程进行访谈,并对药物处方进行药物分析。在8个月的时间里,32例患者被纳入研究。所有移植前和移植后患者均受益于该活动。在M0时,分析的所有处方至少发现了一个问题。半数移植患者采用M1维持,1例采用M3维持,无M6随访。这项工作总结了良好的可行性和整合临床药学活动的护理途径。
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引用次数: 0
Renal AA amyloidosis revealing extramedullary plasmocytoma 肾 AA 淀粉样变性揭示髓外浆细胞瘤
Pub Date : 2023-12-20 Epub Date: 2023-12-11 DOI: 10.1684/ndt.2023.54
Amel Harzallah, Hanen Abid, Meriam Hajji, Sahar Agrebi, Fethi Ben Hamida, Soumaya Chargui, Ezzedine Abderrahim

Introduction: Solitary plasmacytoma is a rare, localized malignancy. Bone localizations are the most common. Extramedullary plasmacytomas are much rarer. They are most often in the upper respiratory tract and can be complicated by amyloidosis. Here is an original report of a mediastinal extramedullary plasmacytoma revealed by type AA renal amyloidosis.

Case presentation: We present the case of a 52-year-old patient with mediastinal extramedullary plasmocytoma diagnosed by renal failure due to type AA renal amyloidosis. Treatment was based on surgery with chemotherapy based on prednisone and melphalan. The patient presented end-stage renal failure that required hemodialysis at discharge.

Conclusion: Extramedullary plasmacytoma is a rare tumour that may be associated with amyloidosis, usually type AL. To our knowledge, its association with AA amyloidosis has not been reported in the literature. Treatment is based on surgery combined with radiotherapy or chemotherapy.

导言单发浆细胞瘤是一种罕见的局部恶性肿瘤。骨定位是最常见的。髓外浆细胞瘤则更为罕见。它们最常见于上呼吸道,可并发淀粉样变性。以下是一篇由 AA 型肾淀粉样变性揭示的纵隔髓外浆细胞瘤的原创报告:本病例是一名52岁的纵隔髓外浆细胞瘤患者,因AA型肾淀粉样变性导致肾功能衰竭而被诊断为纵隔髓外浆细胞瘤。治疗以手术为主,同时使用泼尼松和美法仑进行化疗。患者出现终末期肾衰竭,出院时需要进行血液透析:髓外浆细胞瘤是一种罕见肿瘤,可能与淀粉样变性(通常为AL型)有关。据我们所知,文献中从未报道过这种肿瘤与AA型淀粉样变性有关。治疗方法主要是手术结合放疗或化疗。
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引用次数: 0
[Impact of physical activity in non-dialysis CKD patients followed in a multidisciplinary care network on the progression of chronic renal failure – PHYSALYS study]. 多学科护理网络中非透析CKD患者的体育活动对慢性肾功能衰竭进展的影响——PHYSALYS研究
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.48
Noémie Rossello, Évelyne Decullier, Christelle Marolho, Anne Jolivot, Maurice Laville

Physical activity (PA), has a proven effect on overall health. The study assessed the difference in glomerular filtration rate (GFR) over one year in non-dialysis renal failure patients between those who practiced exercise (P) and those who did not (NP). Patients were categorised as P or not P using the Global Physical Activity Questionnaire (GPAQ2), completed by telephone, at inclusion and at 12 months. Among the 259 patients included, 195 (75.3%) practiced a PA and 64 (24.7%) did not practiced. There was no significant difference in the slope of GFR decline from inclusion to month 12 between the two groups, p = 0.4107. Only the type of kidney seemed to be significantly associated with the slope of GFR decline over the 12 months (p = 0.0039). These results may be explained by a follow-up time too short to identify an effect of behavioural change on the progression of kidney disease.

体育活动(PA)已被证明对整体健康有影响。该研究评估了非透析肾功能衰竭患者在一年内进行运动(P)和不进行运动(NP)的肾小球滤过率(GFR)的差异。通过电话、入选时和12个月时完成的全球体力活动问卷(GPAQ2)将患者分为P或非P。在259名患者中,195名(75.3%)进行了PA,64名(24.7%)未进行PA。两组之间从纳入到第12个月的GFR下降斜率没有显著差异,p=0.4107。在12个月内,似乎只有肾脏类型与肾小球滤过率下降的斜率显著相关(p=0.0039)。这些结果可能是由于随访时间太短,无法确定行为变化对肾脏疾病进展的影响。
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引用次数: 0
[Atypical hemolytic and uremic syndrome in Algeria: diagnostic difficulties and therapeutic constraints]. 阿尔及利亚的非典型溶血和尿毒症综合征:诊断困难和治疗限制
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.37
Souad Chelghoum, Farid Haddoum, Kamel Djenouhat, Mohamed-Taher Hamlaoui, Nawel Adjlane, Mourad Boukheloua

Atypical Hemolytic Uremic Syndrome (aHUS) is a systemic disease due to dysregulation of the alternate complement pathway, mortality is estimated at 10% and more than 50% of patients progress to end-stage renal disease. The aim of this study was to summarize the clinical data and biological results as well as the evolution and management of patients with aHUS seen over a period of four years in one specialized department in Algeria. Our study was observational and longitudinal. The inclusion criteria were: the clinical-biological triad of aHUS and age ≤ 16 years. The type of treatment, the presence of complement mutation or anti-complement factor autoantibodies were not eligibility conditions. On inclusion and every six months, demographic data, clinical and biological history and results after treatment were collected prospectively. Our workforce consisted of 21 children with aHUS. Thirteen patients benefited from a complement study; among them, 7 had complement abnormalities. Eleven children had familial HUS; among them 8 died and 6 were less than one year old. Plasma exchanges were performed in two children. Six patients received eculizumab, with an average age of 3.6 years. After the acute phase, 9 children recovered their kidney function, one child had developed a chronic kidney disease (CKD), and 11 died, among them 8 belong to aHUS families. Fifty percent of deaths occurred in the first 3 months. At 2 years of evolution, out of 7 children having reached this stage, five had renal sequelae and four of them had CKD. The severe prognosis of this disease makes early diagnosis and treatment essential.

非典型溶血性尿毒症综合征(aHUS)是一种由于替代补体途径失调而引起的系统性疾病,死亡率估计为10%,超过50%的患者进展为终末期肾病。本研究的目的是总结阿尔及利亚一个专业科室四年来aHUS患者的临床数据和生物学结果,以及患者的演变和管理。我们的研究是观察性和纵向的。纳入标准为:aHUS临床生物学三联征,年龄≤16岁。治疗类型、补体突变或抗补体因子自身抗体的存在不是合格条件。纳入时和每六个月,前瞻性地收集人口统计学数据、临床和生物学史以及治疗后的结果。我们的员工包括21名患有aHUS的儿童。13名患者受益于一项补充研究;其中补体异常7例。11名儿童有家庭HUS;其中死亡8例,1岁以下6例。对两名儿童进行了血浆交换。6名患者接受了埃库珠单抗治疗,平均年龄3.6岁。急性期后,9名儿童肾功能恢复,1名儿童患上慢性肾脏疾病(CKD),11名儿童死亡,其中8名属于aHUS家庭。50%的死亡发生在前3个月。在2年的进化过程中,在达到这一阶段的7名儿童中,有5名患有肾脏后遗症,其中4名患有CKD。这种疾病的严重预后使得早期诊断和治疗至关重要。
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引用次数: 0
[Pruritus associated with chronic kidney disease in hemodialysis patients: a survey in French nephrologists]. 血液透析患者中与慢性肾脏疾病相关的瘙痒:法国肾脏病学家的调查
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.31
Maxime Touzot, Pierre Bataille, Guy Rostoker, Philippe Chauveau, Antoine Lanot, Laurent Misery

Background: Chronic kidney disease-associated pruritus (CKD-aP) is common in hemodialysis patients and severely impairs their quality of life, but the practices of nephrologists remain poorly known.

Methods: The objective of this on-line survey was to describe the management of CKD-aP in French nephrologists affiliated with the French-speaking Society of Nephrology, Dialysis and Transplantation (SFNDT) and involved in hemodialysis.

Results: In total, 122 questionnaires were completed and 100 were usable. Nephrologists reported they personally managed a median of 52 patients; they estimated that the CKD-aP prevalence in their hemodialysis patients was a median of 10% (IQR, 6.3-17.2); 6% of nephrologists reported not following any patient with CKD-aP. In case of CKD-aP, the first-intention intervention was the evaluation of phosphocalcic metabolism (53.5%) and verification of dialysis adequacy (52%). For moderate-to-severe CKD-aP, the first-line prescription was topical therapy (71.3%), antihistamine (23.2%) and membrane change (15.9%). Patients were referred to a dermatologist mainly in case of treatment failure (86.9%) or scratching lesions (40.4%). Available treatments were considered ineffective for 50.5% of nephrologists, partially effective for 45.5% and effective for only 4%.

Conclusion: These results show that according to the opinion of nephrologists, the pruritus prevalence is low in dialysis patients. This is inconsistent with studies based on systematic patient interviews, thus suggesting that pruritus is a symptom overlooked by nephrologists. In the context of the arrival of a new drug for pruritus, patients should be more questioned about this symptom in order to propose this treatment.

背景:慢性肾脏病相关瘙痒症(CKD-aP)在血液透析患者中很常见,严重损害了他们的生活质量,但肾脏科医生的做法仍然鲜为人知。方法:本在线调查的目的是描述法语肾脏病、透析和移植学会(SFNDT)附属并参与血液透析的法国肾脏病学家对CKD-aP的管理。结果:共完成问卷122份,可用问卷100份。肾病学家报告说,他们亲自管理了平均52名患者;他们估计血液透析患者的CKD-aP患病率中位数为10%(IQR,6.3-17.2);6%的肾脏科医生报告没有跟踪任何CKD-aP患者。在CKD-aP的情况下,第一意向干预是评估磷钙代谢(53.5%)和验证透析充分性(52%)。对于中重度CKD-aP,一线处方是局部治疗(71.3%)、抗组胺药(23.2%)和膜改变(15.9%)。患者主要在治疗失败(86.9%)或抓挠病变(40.4%)的情况下转诊给皮肤科医生。50.5%的肾科医生认为可用的治疗无效,部分有效45.5%,有效率仅4%。这与基于系统患者访谈的研究不一致,因此表明瘙痒是肾病学家忽视的症状。在一种治疗瘙痒的新药问世的背景下,为了提出这种治疗方法,应该更多地询问患者的这种症状。
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引用次数: 0
[Heart disease in hemodialysis: physiopathology and therapeutic breakthroughs]. 血液透析中的心脏病:生理病理学和治疗突破
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.47
Lucile Mercadal, Jean-Jacques Boffa, Jean-Philippe Collet

Especially exposed to frequent cardiovascular events and its related mortality, some cardiovascular therapies of the hemodialyzed population as well as coronary investigations remain controversial. We have collected data and discussed recent trials and guidelines dedicated to this patient subset.

特别是在频繁发生心血管事件及其相关死亡率的情况下,血液透析人群的一些心血管治疗以及冠状动脉研究仍然存在争议。我们已经收集了数据,并讨论了最近专门针对这一患者子集的试验和指南。
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引用次数: 0
[Immune modulation with extracorporeal photopheresis in renal transplantation: proof of concept clinical outcomes and perspective]. 体外光合疗法在肾移植中的免疫调节:概念验证的临床结果和前景
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.46
Thomas Crépin, Arnaud Lionet, Jean-François Augusto, Marc Hazzan, Cyril Garrouste, Anne-Elisabeth Heng, Didier Ducloux

For 30 years, photopheresis is used to treat graft versus host disease and heart or lung allograft rejection. In this review, we discuss the place of photopheresis in kidney transplantation both in prevention or treatment of rejection. Mechanisms of action in kidney transplantation are mainly based on results observed in graft versus host disease and in heart or lung transplantation. Photopheresis may induce innate and adaptive immunity changes with restauration of a favourable Th1/Th2 immune balance, an expansion of LT /LB reg subsets, and a local enrichment in IL-10. French national clinical and mechanistic studies are underway to define the place of photopheresis therapy in immunomodulation strategies in kidney transplantation.

30年来,光疗法一直被用于治疗移植物抗宿主疾病和心脏或肺移植物排斥反应。在这篇综述中,我们讨论了光刺激在肾移植中预防或治疗排斥反应的作用。肾移植的作用机制主要基于在移植物抗宿主疾病和心脏或肺移植中观察到的结果。光切可以诱导先天和适应性免疫变化,恢复有利的Th1/Th2免疫平衡,扩大LT/LB-reg亚群,并局部富集IL-10。法国国家临床和机制研究正在进行中,以确定光疗法在肾移植免疫调节策略中的地位。
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引用次数: 0
[Functional vesico-sphincteric disorders in children: clinical, urodynamic and evolutionary profile]. 儿童功能性膀胱括约肌疾病的临床、尿动力学和进化特征
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.38
Maryem Ferjani, Yousra Hammi, Taha Sayari, Rym Baati, Naija Ouns, Tahar Gargah

Background: Childhood vesico-sphincteric disorders are the cause of functional and psychological disability. They are also responsible for serious uronephrologic complications akin to neuro-bladder complications. In this study, we looked for the clinical manifestations linked to these disorders as well as the paraclinical and urodynamic anomalies and their therapeutic management.

Methods: We carried out a retrospective and descriptive study of patients with vesico-sphincteric disorders, followed in the pediatrics department of Charles Nicolle hospital in Tunis.

Results: In total 26 patients were included in our study. The median age at the first consultation in a pediatric nephrology department was 9 years (IQR 25 = 6 years; IQR 75 = 11 years). There was a predominance of girls: 19 boys and 43 girls. The filling phase and the voiding phase were dominated by the combination of three or more symptoms. Bladder tenderness was reduced in 12% of cases. Nineteen percent of patients reported post micturition dribble in the post-voiding phase. The flow rate curve was polyphasic in 36% of cases and flat in 11% of cases. The median of the post void residual was 62, 2 mL (IQR 25 = 25 mL; IQR 75 = 102 mL). Cystometry showed reduced bladder sensation in 14% of cases, detrusor overactivity in 65% of cases, bladder hypocontractility in 8% of cases, hypocompliant bladder in 42% of cases and small capacity in 88% of cases. Sphincter dysynergia was noted in 34% of patients. Anticholinergics were the most used in the treatment of disorders (53%). Renal failure was noted in 45% of the patients of which 11% had end-renal stage failure. During follow-up 16% of our patients required kidney replacement therapy.

Conclusion: Given the seriousness of vesico-sphincteric disorders in children and the late discovery in the majority of cases at the complication stage, targeted primary prevention must be carried out based on continuous training of primary care physicians to minimize the appearance of complications involving the functional and vital prognosis of these children.

背景:儿童膀胱括约肌疾病是导致功能和心理残疾的原因。他们还负责类似于神经膀胱并发症的严重的肾功能并发症。在这项研究中,我们寻找与这些疾病相关的临床表现,以及临床旁和尿动力学异常及其治疗方法。方法:我们对突尼斯Charles Nicolle医院儿科的膀胱括约肌疾病患者进行了回顾性和描述性研究。结果:本研究共纳入26例患者。儿童肾脏科首次会诊时的中位年龄为9岁(IQR 25=6岁;IQR 75=11岁)。女孩占多数:19名男孩和43名女孩。充盈期和排尿期以三种或三种以上症状的组合为主。膀胱压痛减少了12%。19%的患者报告在排尿后阶段出现排尿后运球。36%的病例的流速曲线是多相的,11%的病例的流量曲线是平坦的。空隙后残留物的中位数为62.2 mL(IQR 25=25 mL;IQR 75=102 mL)。膀胱测量术显示14%的病例膀胱感觉减轻,65%的病例逼尿肌过度活动,8%的病例膀胱收缩力低下,42%的病例膀胱顺应性低下,88%的病例膀胱容量小。34%的患者出现括约肌功能障碍。抗胆碱能药物是治疗疾病最多的药物(53%)。45%的患者出现肾功能衰竭,其中11%的患者出现终末期肾功能衰竭。在随访期间,16%的患者需要肾脏替代治疗。结论:鉴于儿童膀胱括约肌疾病的严重性,以及大多数病例在并发症阶段发现较晚,必须在初级保健医生的持续培训基础上进行有针对性的初级预防,以尽量减少涉及这些儿童功能和生命预后的并发症的出现。
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引用次数: 0
[Cardiovascular calcifications in dialysis patients]. 透析患者的心血管钙化
Pub Date : 2023-11-02 DOI: 10.1684/ndt.2023.45
Pablo Ureña Torres, Charles Chazot

Patients with advanced chronic kidney disease and those already on dialysis have an increased prevalence of cardiovascular calcifications. They are the cause of severe complications and are associated with a reduced life expectancy in these patients. Recommendations and imaging scores have been developed to detect and assess their importance, to guide and improve the management of cardiovascular risk. However, despite these recommendations, current practice teaches us that they are only partially applied. The prevention and treatment of cardiovascular calcifications go through the correction of classic risk factors associated with atherosclerosis, mineral and bone metabolism disorders and by optimizing the dose and the efficiency of dialysis. New therapeutic strategies are beginning to emerge, others are being evaluated, such as sodium thiosulfate, rheopheresis, vitamin K, magnesium supplementation, and SNF-472.

晚期慢性肾脏病患者和已经接受透析的患者心血管钙化的患病率增加。它们是严重并发症的原因,并与这些患者的预期寿命缩短有关。已经制定了建议和成像评分,以检测和评估其重要性,指导和改进心血管风险的管理。然而,尽管有这些建议,目前的做法告诉我们,这些建议只是部分适用。心血管钙化的预防和治疗需要纠正与动脉粥样硬化、矿物质和骨代谢紊乱相关的经典风险因素,并优化透析的剂量和效率。新的治疗策略开始出现,其他策略正在评估中,如硫代硫酸钠、流变学、维生素K、补充镁和SNF-472。
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引用次数: 0
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Nephrologie & therapeutique
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