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<11/11>
共217条结果
  • 1.

    【2hr】Bladder Rupture following Conversion to Enteric Drainage after Pancreatic Transplantation

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    机译 胰腺移植后转换为肠引流后的膀胱破裂
    摘要：Complications associated with bladder-drained pancreatic transplant are not uncommon and include urinary tract infections and reflux pancreatitis. Bladder rupture with peritoneal leak is a rare complication after pancreatic transplantation and can present as an acute abdomen with rapidly deteriorating renal function. We describe the first case of a urine leak into the peritoneal cavity occurring after conversion from bladder to enteric drainage. A high index of suspicion is required to diagnose such a complication.
  • 2.

    【2hr】Thyroid Storm Masked by Hemodialysis and Glucocorticoid Therapy in a Patient with Rheumatoid Arthritis

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    机译 类风湿关节炎患者的血液透析和糖皮质激素治疗掩盖了甲状腺风暴
    摘要：Thyroid function test values are generally at low levels in patients with end-stage kidney disease. Life-threatening thyrotoxicosis or thyroid storm is rare, especially in hemodialysis (HD) patients, and is characterized by multisystem involvement and a high mortality rate if not immediately recognized and treated. Here, we report a female patient with severe symptomatic thyroid storm, receiving long-term HD and glucocorticoid therapy. Methimazole at a dose of 15 mg per day, β-adrenergic blockade and HD succeeded in controlling the patient's condition by gradually adjusting the target dry weight for hyperthyroidism-induced weight loss. When she was discharged from the hospital, her dry weight was reduced from 47.2 to 39.2 kg. The management of patients with severe symptomatic thyroid storm on HD represents a rare scenario. It is essential to initiate the available treatments as early as possible to reduce its mortality.
  • 3.

    【2hr】Bilateral Nephrectomy as a Rescue Therapy for Hemodialyzed Patient with Malignant Hypertension – Case Report

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    机译 双边肾切除术作为血液透析恶性高血压患者的抢救疗法–病例报告
    摘要：We present the case of a 64-year-old male patient in whom malignant phase of hypertension developed during dialysis therapy. Hypertension was resistant to pharmacological therapy with seven antihypertensive drugs and dialysis therapy with ultrafiltration. In this patient bilateral nephrectomy was performed as a rescue therapy. It led to better control of blood pressure and allowed to reduce the number and dosage of antihypertensive medications.
  • 4.

    【2hr】A Japanese Family Suffering from Familial Juvenile Hyperuricemic Nephropathy due to a Rare Mutation of the Uromodulin Gene

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    机译 一个日本家庭患有尿少调节素基因的罕见突变，导致家族性青少年高尿酸血症性肾病
    摘要：We report the case of a Japanese family suffering from familial juvenile hyperuricemic nephropathy (FJHN) due to a rare missense mutation of the uromodulin (UMOD) gene. An 18-year-old male presented with gout, hyperuricemia, and stage 3 chronic kidney disease. Mostly, FJHN is caused by a mutation altering the cystine residue of UMOD/Tamm-Horsfall protein. However, in the present case, a T688C mutation was identified in exon 4, resulting in amino acid substitution with arginine replacing tryptophan at position 230 (Trp230Arg). This mutation was also found in his brother and father with the same phenotype, indicating autosomal dominant inheritance. The affected amino acid was conserved in 200 healthy Japanese controls. Therefore, mutation T688C most likely causes rare structural and/or functional abnormalities in UMOD/Tamm-Horsfall protein.
  • 5.

    【2hr】Massive Colchicine Overdose with Recovery

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    机译 大量秋水仙碱过量与恢复
    摘要：Colchicine is an active alkaloid that is commonly used for treatment of multiple diseases including gout, primary biliary cirrhosis and familial Mediterranean fever. Less commonly, it has been implicated in several fatal overdoses. Deaths from colchicine overdoses are usually due to multi-organ failure, whether directly from colchicine toxicity or due to ensuing sepsis. We report an extreme case of colchicine ingestion (1.38 mg/kg), which is the largest reported non-fatal colchicine overdose. The patient was a 47-year-old First Nations woman with a history of depression and no other comorbidities. Ingestion was intentional and initial presentation was within 2 h of ingestion, at which point she had normal clinical and laboratory parameters. Early implementation of a targeted therapeutic strategy directed at the predicted multi-organ failure which included aggressive use of a GI decontamination protocol, timely supportive measures including ventilator support and renal replacement therapy, as well as the utilization of broad-spectrum antibiotics and G-CSF for sepsis and leucopenia management, resulted in successful support and discharge of this patient off dialysis.
  • 6.

    【2hr】Symptomatic Anti-Neutrophil Cytoplasmic Antibody-Positive Disease Complicating Subacute Bacterial Endocarditis: To Treat or Not to Treat?

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    机译 对症中性粒细胞胞浆抗体阳性疾病并发亚急性细菌性心内膜炎：要治疗还是不治疗？
    摘要：A 54-year-old man was diagnosed with Streptococcus mutans endocarditis of the mitral valve. Serological tests disclosed the presence of multiple autoantibodies including c-ANCA, anti-PR3 and anti-MPO. While the fever subsided with antibiotics, mental status and renal function deteriorated rapidly. Kidney biopsy revealed pauci-immune glomerulonephritis and acute eosinophilic interstitial nephritis. The abnormal clinical features improved rapidly after addition of corticosteroids and cyclophosphamide to the antibiotics. Immunosuppressive agents may be required in a fraction of the patients with infective endocarditis who develop ANCA and ANCA-mediated renal disease. Histological identification of the type of renal disease is imperative for the choice of the treatment.
  • 7.

    【2hr】Posterior Reversible Encephalopathy Syndrome during Recovery from Acute Kidney Injury after Hepatitis A Infection

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    机译 甲型肝炎感染后急性肾损伤恢复期间的后可逆性脑病综合征
    摘要：The patient was a 25-year-old healthy male who experienced fever, chills, and abdominal pain for 5 days prior to the hospital visit. He was diagnosed with acute hepatitis A, and at admission he presented with anuric acute kidney injury and hepatic encephalopathy. He received continuous renal replacement therapy followed by intermittent regular hemodialysis. His urine output increased to 1,610 ml/day after 31 days. On day 32, he suddenly developed a headache and visual disturbance and experienced three short convulsions, which were followed by postictal confusion and high fever. T2 and FLAIR MRI images of the brain revealed hyperintense signal alterations in bilateral subcortical regions of the temporoparietal and occipital lobes, consistent with posterior reversible encephalopathy syndrome. His mental status was fully recovered after 7 h of conservative treatment, including antihypertensive therapy. On hospital day 56, the renal function of the patient had recovered, and he was discharged without neurologic sequelae.
  • 8.

    【2hr】A Trial of Complement Inhibition in a Patient with Cryoglobulin-Induced Glomerulonephritis

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    机译 冷球蛋白诱导的肾小球肾炎患者的补体抑制试验
    摘要：Cryoglobulinemia induces an immune complex-mediated glomerulonephritis that is characterized by the presence of large immune deposits, including complement C3 and C5b-9, marked macrophage influx and mesangial cell proliferation. The precise role of complement in cryoglobulin-induced glomerulonephritis in humans remains unclear, whereas in mice there has been evidence that complement activation might be a central factor favoring glomerular inflammation, particularly by the recruitment of neutrophils. We report on an exceptional case of cryoglobulin-induced glomerulonephritis in a patient with mixed essential cryoglobulinemia type II. The clinical features included relapsing proteinuria and renal function impairment that were controlled by plasmapheresis. Complement was low in plasma and two renal biopsies at 1-year interval showed prominent immunoglobulin and complement deposits, with unusual high numbers of neutrophils. In a 1-patient clinical trial, we tested whether the monoclonal anti-C5 antibody eculizumab would be sufficient to control renal function at the time of a relapse. Although during the initial weeks renal function was stabilized, slow increase in creatinine could not be controlled by this treatment, so that plasmapheresis was reinstituted. This result suggests that despite evidence for a role of complement in enhancing renal damage in this patient, other inflammatory processes dominated.
  • 9.

    【2hr】A Case of Recurrent Proliferative Glomerulonephritis with Monoclonal IgG Deposits after Kidney Transplant Treated with Plasmapheresis

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    机译 血浆置换治疗肾脏移植术后复发性增殖性肾小球肾炎合并单克隆IgG沉积的病例
    摘要：Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare and recently identified disease with a poor prognosis irrespective of the treatment. Recently, the possibility of recurrent or de novo PGNMID after kidney transplantation has been reported, which is associated with a better prognosis compared to PGNMID on native kidneys. Nevertheless, at present, due to the very few cases of recurrent PGNMID diagnosed, there is no proven effective treatment. Here, we report a case of recurrent PGNMID successfully treated with plasmapheresis, steroids and mycophenolate mofetil. Our report suggests that plasmapheresis might be a valid therapeutic option to treat recurrent PGNMID.
  • 10.

    【2hr】Successful Treatment of Adult IgA Nephropathy with Nephrotic-Level Proteinuria by Combination Therapy Including Long-Term Coadministration of Mizoribine

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    机译 长期联合服用米佐立滨的联合疗法成功治疗了具有肾病水平蛋白尿的成人IgA肾病
    摘要：A 41-year-old male patient was admitted to our hospital due to massive proteinuria and hematuria. His 24-hour urinary protein excretion and the number of urinary erythrocytes were 3.91 g/day and 50–99/high-power field, respectively. A renal biopsy showed a severe pathological pattern of immunoglobulin A nephropathy (IgAN) that involved marked endocapillary proliferation and segmental sclerosis (Oxford-MEST score: M0, E1, S1, T0). Because he had nephrotic-level proteinuria with severe pathological findings, which are tonsillectomy and corticosteroid pulse therapy-resistant characteristics, he received mizoribine for a long period as part of the combination therapy using corticosteroid, tonsillectomy, dipyridamole, warfarin and renin-angiotensin-aldosterone system blockers. Twelve months after the beginning of treatment, his urinary findings were normal. In this report, we describe the pathological findings and successful treatment course, and discuss the potential effects of long-term coadministration of mizoribine for adult IgAN treatment.
  • 11.

    【2hr】Steroid-Responsive Nephrotic Syndrome and Bilateral Renal Artery Stenosis: A Possible Role for Angiotensin-Mediated Podocyte Injury

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    机译 类固醇反应性肾病综合征和双侧肾动脉狭窄：血管紧张素介导的足细胞损伤的可能作用。
    摘要：Nephrotic syndrome (NS) associated with renal artery stenosis is not widely recognized or investigated as a cause of the NS. The mechanisms are incompletely understood, but have largely focused on hemodynamic factors resulting in hyperfiltration injury-induced focal and segmental glomerulosclerosis (FSGS) in the nonstenosed kidney with sparing of the stenotic kidney protected from hemodynamic stress. However, separation of hemodynamic from circulating factors (such as angiotensin II) as the cause of the nephrosis remains difficult. We report a patient presenting with NS who was incidentally discovered to have high-grade bilateral renal artery stenosis from fibromuscular dysplasia. Kidney biopsy revealed FSGS. Proteinuria in our patient did not initially respond to angiotensin-converting enzyme inhibition (ACEI) and correction of stenoses with angioplasties. There was prompt response to steroid treatment. A brief relapse several months later (without associated hypertension) responded to ACEI alone. This is the first reported case of an association between fibromuscular dysplasia and steroid-responsive nephrotic syndrome due to FSGS. This may shed insight into the nature of podocyte injury in patients with high angiotensin states and suggest a possible role for activated renin-angiotensin-aldosterone system (RAAS) triggering an immune-mediated injury, rather than hemodynamic insult. Furthermore the lack of initial response to angioplasty and ACEI suggests that RAAS-activated injury may in some cases require more aggressive immune modulatory therapy with steroids over and above angiotensin inhibition alone. This case also highlights the importance of being aware of possible occult renovascular disease contributing to idiopathic NS with FSGS even when hypertension is only modest.
  • 12.

    【2hr】Balloon-Assisted Creation and Maturation of an Arteriovenous Fistula in a Patient with Small-Caliber Vasculature

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    机译 小口径脉管系统患者的动静脉瘘的球囊辅助产生和成熟。
    摘要：The major problem associated with the creation of an arteriovenous fistula (AVF), which is the optimal form of vascular access for hemodialysis, is the fact that fistulas do not necessarily mature into a usable vascular access, requiring subsequent revision and construction of another access. The caliber of the vessels used for fistula creation is a pivotal factor predicting the presumable maturation of the constructed fistula, and veins less than 2.5 mm in diameter have poor outcomes. This report describes the balloon-assisted creation and maturation of an autogenous radial-cephalic AVF in a patient with a small-caliber vein and a radial artery measuring approximately 2 and 1.5 mm in diameter, respectively. The clinical impact of percutaneous radiological intervention for expediting the maturation of an autogenous AVF with small-caliber vessels is also discussed.
  • 13.

    【2hr】Successful Treatment of Cryoglobulinaemia with Rituximab

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    机译 利妥昔单抗成功治疗冰球蛋白血症
    摘要：Cryoglobulinaemia is a systemic inflammatory condition characterised by immune complex-mediated small-to-medium-sized vasculitis. It has a wide variety of presentations ranging from bruising, neuropathy, and hepatosplenomegaly to acute renal failure. Mixed cryoglobulinaemia is the most common type and is strongly associated with hepatitis C. Management approaches include use of cyclophosphamide, prednisolone, and plasmapheresis, with differing views on alternative treatments in resistant cases. Rituximab has emerged as an attractive option in resistant cases on account of its potent immunosuppressive effects on B cells. We describe a case of type 2 mixed cryoglobulinaemia in association with non-Hodgkin's lymphoma resistant to standard treatments which responded well to rituximab. This case is remarkable as mixed cryoglobulinaemia associated with non-Hodgkin's lymphoma presenting with nephritis is unusual, and, contrary to the high rate of recurrence in lymphoma-related cryoglobulinaemia, our patient has not shown any recurrence over 24 months. This highlights an alternative treatment modality which can be used in patients not responsive to existing managements for this condition with benefits of minimal side effects and no oncogenetic potential.
  • 14.

    【2hr】Severe Proteinuria Secondary to Amyloidosis Requiring Bilateral Renal Artery Embolization

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    机译 淀粉样变性继发的严重蛋白尿，需要双侧肾动脉栓塞
    摘要：In the case reported here, after prolonged medical therapy resistance, severe proteinuria subsided following bilateral renal artery embolization (RAE). Thereafter, respiratory distress, anasarca edema, muscle mass, and serum albumin level improved after regular hemodialysis. Although RAE is reported to be a safe and effective therapeutic procedure, it is rarely used for severe proteinuria with prolonged medical therapy resistance. The limited use of bilateral RAE for nephrological purposes may be partly related to its tendency to destroy renal function, which results in anuria and subsequent regular dialysis. However, delayed RAE could cause the patient to reach a life-threatening cachexic state and could increase the risk of morbidity and mortality due to severe proteinuria-induced hypoalbuminemia. Our case and selected previous reports reveal important information for physicians and patients while discussing prognoses and considering the pros and cons of bilateral RAE.
  • 15.

    【2hr】Spontaneous Renal Hemorrhage in Hemodialysis Patients

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    机译 血液透析患者的自发性肾出血
    摘要：Dialysis patients have a tendency to bleed, and clinicians sometimes encounter cases with a significant amount of spontaneous hemorrhage. We herein report two cases of spontaneous renal hemorrhage in hemodialysis patients.Case 1A 70-year-old male who had received hemodialysis for 8 years presented with right abdominal pain. He had a history of renal failure due to diabetes mellitus. CT showed a right perirenal hemorrhage. Angiography revealed a right renal artery hemorrhage, and catheter embolization was performed.
  • 16.

    【2hr】Immune Complex-Mediated Complement Activation in a Patient with IgG4-Related Tubulointerstitial Nephritis

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    机译 IgG4相关性肾小管间质性肾炎患者的免疫复合物介导的补体激活。
    摘要：A 59-year-old man was diagnosed with IgG4-related tubulointerstitial nephritis. His symptoms as well as laboratory and imaging findings were improved after initiation of steroid therapy. Serologically, he showed hypocomplementemia (C3 23 mg/dl, C4 <2 mg/dl, CH50 <7 U/ml) with high levels of IgG (IgG4 1,970 mg/dl) and immune complexes (C1q assay 8.1 μg/ml) and a low level of C1q (<2.0 mg/dl). Histologically, he also showed linear depositions of IgG, IgM, C3, C4d, C1q, membrane attack complex and all IgG subclasses (IgG1, IgG2, IgG3 and IgG4) along the tubular basement membrane, as well as granular depositions of these components in the renal interstitium. However, mannose-binding lectin and L-ficolin were not detected in these tissues. Homogeneous electron-dense deposits were observed by electron microscopy in the tubular basement membrane. It appears that the immune complexes might activate the classical pathway of the complement in both blood and local tissues in a patient with IgG4-related tubulointerstitial nephritis.
  • 17.

    【2hr】Three Cases of Gastric Antral Vascular Ectasia in Chronic Renal Failure

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    机译 慢性肾功能衰竭胃窦血管性蜕膜炎3例
    摘要：Gastric antral vascular ectasia (GAVE) is currently recognized as an important cause of gastrointestinal bleeding. Chronic kidney disease (CKD) is associated with a high incidence of GAVE. We report 3 patients with CKD who presented with severe anemia and were diagnosed with GAVE; they were resistant to endoscopic argon plasma coagulation. However, remission of anemia and improvement in GAVE lesions were observed after the initiation of hemodialysis. The pathogenesis of GAVE remains largely unknown, but mechanical stress of the antrum could play an important role. This stress may be reduced by hemodialysis through improvement of uremia-associated gastrointestinal symptoms. Therefore, the initiation of hemodialysis might be effective for intractable GAVE in CKD patients.
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