Case Reports in Nephrology and Dialysis最新文献

Introduction: Constrictive pericarditis (CP) is characterized by impaired diastolic filling of the ventricles, which typically causes right heart failure. Its diagnosis may be challenging because it mimics other disorders. Furthermore, prompt diagnosis and treatment are more crucial in cases of hemodialysis; otherwise, maintenance dialysis would be hampered by severe hypotension.

Case presentation: We report the case of a 53-year-old man on hemodialysis who developed CP with shock. His blood pressure was 55/30 mm Hg at the time of hospitalization due to presyncope. He continued hemodialysis but with some difficulty. However, right pleural effusion persisted because of impaired fluid removal by hemodialysis. Despite such severe manifestations, the distinctive clinical features of CP were lacking. By carefully evaluating the time course of computed tomography images, progressive calcification in the pericardium emerged as a significant clue. Cardiac catheterization subsequently revealed a dip-and-plateau pattern of right ventricular pressure, which led to a definitive diagnosis of CP. Soon after the diagnosis, he underwent successful surgery.

Conclusion: An important finding was that, unlike calcific lesions in the general dialysis population, the patient's calcific lesions were mostly confined to the pericardium and progressed on a monthly basis. Thus, the present case may provide deep insight into the diagnosis of CP and the management of hemodialysis patients with severe hypotension.

Introduction: A common complication of arteriovenous fistula (AVF) is thrombosis in the venous segment, which can impair vascular access unless a successful thrombectomy is performed.

Case presentation: In this manuscript, we describe the case of a diabetic patient who had primary AVF in a snuff-box with subsequent superficialization of the medial vein of the forearm. Unfortunately, this section of the vein was occluded, although the fistula was patent through the cephalic vein (CV). Due to insufficient flow, this vascular access was unsuitable for hemodialysis. Using a vein from the subcutaneous venous network (SVN), additional AVF was performed. Our goal was to accelerate maturation by doubling arteriovenous flow, which then increased the size of the CV in the arm. After maturation, a second superficialization was performed on the arm, which allowed for successful cannulation.

Conclusion: SNV may be considered for the creation of a new AVF to improve the maturation of the primary fistula.

Introduction: Colchicine has a narrow therapeutic index, and doses >0.5 mg/kg are considered toxic with a high mortality rate.

Case presentation: A previously healthy 15-year-old presents to the emergency department with abdominal pain and vomiting following intentional ingestion of colchicine (0.56 mg/kg) 12 h prior. By 24 h post-ingestion, they developed a multi-organ injury with hepatic dysfunction, coagulopathy, lactic acidemia, and pancytopenia, which prompted consideration of extracorporeal therapy (ECT). Considering the characteristics of colchicine, they were treated with continuous venovenous hemodiafiltration (CVVHDF) with single-pass albumin dialysis (SPAD) for 42 h. They were subsequently discharged from the intensive care unit 48 h after stopping CVVHDF with normal kidney function, resolved coagulopathy, and resolving pancytopenia and hepatic dysfunction. The rationale for CVVHDF with SPAD was based on the high protein binding, variably high volume of distribution, previous reports showing a sieving coefficient of 0.2 with CVVH, and the high mortality risk. We anticipated a high potential for rebound. Thus, continuous clearance would facilitate redistribution from the extravascular to intravascular space. SPAD was used to enhance the elimination of protein-bound fractions; the principle is that adding albumin to dialysate creates a protein-binding disequilibrium where the drug from the blood side may bind to albumin on the dialysate side.

Conclusion: Colchicine ingestion of >0.5 mg/kg is highly toxic, and in addition to supportive management, continuous kidney replacement therapy with SPAD may be considered.

Introduction: Corynebacterium species other than C. diphtheriae are being continuously reported as pathogens.

Case presentation: A patient visited the Urology Outpatient Department of a tertiary care centre in India reporting lower abdominal pain, urinary incontinence, and intermittent weak urine flow persisting for 12 years, intensifying over the last 15 days. She also experienced urgency, straining, weak stream, and incomplete voiding, along with a previous fever episode. The patient had a medical record of multiple urethral dilations and surgeries since 2014, with the most recent urethral dilatation in July 2023. Diagnostic tests revealed a thickened bladder with notable post-void residual urine. Uroflowmetry indicated obstructive uropathy. Urine analysis exhibited elevated leucocytes, epithelial cells, red blood cells, and abundant bacilli. Corynebacterium tuberculostearicum was identified through matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) following three pure growths of Gram-positive bacilli in urine cultures. The organism showed sensitivity to cotrimoxazole and tetracyclines. Treatment with doxycycline significantly improved the symptoms.

Conclusion: The organism Corynebacterium tuberculostearicum is a very rare cause of UTI and the patient responded well to treatment.

Introduction: The Seraph^® 100 Microbind^® Affinity Filter is a biomimetic adsorbent device that can remove pathogens from the blood.

Case presentation: Here, we report the successful use of the Seraph^® 100 to treat both a SARS-CoV-2 reinfection leading to severe COVID-19 pneumonia as well as subsequent secondary lung infections including Acinetobacter baumannii, Serratia marcescens, and Pseudomonas aeruginosa multidrug-resistant bacteria. To our knowledge, this 46-year-old black male is the first patient in which four treatments with this pathogen adsorber, one for a viral and three for different bacterial infections, have been successfully used.

Conclusion: The Seraph^® 100 can be easily and successfully used in conjunction with standard (anti-infective) treatment.

Introduction: Fibrillary glomerulonephritis (FGN) is a rare form of glomerular disease that accounts for less than 1 percent of all renal biopsies. It is characterized by pathognomonic electron microscopy findings of fibrillar deposits in the mesangium and glomerular capillary walls. FGN was initially considered to be an idiopathic disorder. However, approximately 30-50 percent of patients have a secondary cause, including a history of malignancy in up to 23% of cases. Chronic lymphocytic leukemia (CLL) is a rare cause of FGN, with limited data and poor prognosis.

Case presentation: In this report, we present the case of a 69-year-old male who was diagnosed with CLL in 2013 and was initially managed conservatively. In 2016, he developed nephrotic syndrome and renal impairment. Renal biopsy showed FGN, and treatment was targeted to the CLL with bendamustine and rituximab, which led to partial remission of nephrotic syndrome and improvement in renal function. After 3 years of clinical remission, the nephrotic syndrome relapsed, and he underwent a repeat renal biopsy confirming ongoing FGN. A bone marrow biopsy confirmed CLL relapse, and the patient was treated with ibrutinib (a tyrosine kinase inhibitor). The patient achieved a significant organ response and sustained remission.

Conclusion: This case highlights the success of treating a potentially identifiable cause of FGN and highlights that even at relapse, treatment can confer benefits and help prevent end-stage renal failure.

Introduction: Malakoplakia is a rare and chronic granulomatous disease that is pathologically characterized by the presence of Michaelis-Gutmann bodies and large macrophage clusters. Malakoplakia of the renal parenchyma is especially rare. In this report, we describe the long-term prognosis of a patient who was diagnosed with and treated for renal parenchymal malakoplakia in infancy.

Case presentation: Seventeen years after malakoplakia onset, the patient presented to us with worsening proteinuria. Computed tomography revealed structural abnormalities in the kidney, and focal segmental glomerulosclerosis (FSGS) was diagnosed based on renal biopsy findings. No Michaelis-Gutmann bodies were observed in von Kossa-stained biopsy specimens. Regular outpatient monitoring during the next 9 years showed gradual deterioration of renal function and a moderately high protein/creatinine ratio.

Conclusion: Our findings suggest that structural changes due to malakoplakia can cause FSGS. Moreover, structural changes indicate the healing of malakoplakia in infancy and the disappearance of its characteristic lesions over time. Owing to its long-term observation period, this unique case provides new insights into the outcomes of patients with renal parenchymal malakoplakia.

Introduction: JC-polyomavirus-associated nephropathy (JC-PVAN) is a rare cause of allograft dysfunction with only a few cases described in the literature.

Case presentation: We present 2 cases of JC-PVAN, both of which occurred >5 years after kidney transplantation. In both cases, transplant biopsies were performed because of worsening of kidney function. We found tubulitis and interstitial inflammation; immunohistochemistry was positive for SV40, but BK virus was not detected. The presence of JC virus confirmed the diagnosis of JC-PVAN. Immunosuppressive therapy was adopted, but in both cases graft function progressively deteriorated.

Conclusions: Our cases show that JC-PVAN, although much rarer than BK-PVAN, should be considered a possible cause of graft dysfunction even years after transplantation. Complete diagnostic workup, including kidney biopsy, is crucial for correct diagnosis and treatment.

Introduction: Nowadays, there is insufficient evidence for the recommendation of management patients with a primary membranoproliferative glomerulonephritis (MPGN). A better understanding of the pathogenesis has led to the reclassification of primary MPGN and distinction into the two main entities of either primary immune complex-MPGN or C3 glomerulopathy. Both entities share overlapping pathophysiological features with complement alternative pathway (AP) dysregulation. Iptacopan is an oral inhibitor of the complement factor B that effectively blocks the complement AP.

Case presentation: We report the first successful treatment of a 47-year-old man suffering from a primary immune complex-MPGN with iptacopan. So far established immunosuppressive therapies with prednisone and mycophenolate mofetil failed to control the current flare of the disease, mainly presenting with impaired kidney function and proteinuria within the nephrotic range. However, 3 months after starting the treatment with iptacopan urine protein-creatinine ratio decreased impressively to a level of 100-150 mg/mmol. Thereafter, low-level proteinuria and kidney function remained stable during follow-up. Do date, the treatment with iptacopan is continued as a monotherapy and is well tolerated.

Conclusion: To the best of our knowledge, this is the first case report which suggests that iptacopan may be an interesting treatment option for primary immune complex-MPGN.

Introduction: Drug-induced tubulointerstitial injury is a common cause of renal impairment. Since the mechanisms of drug-induced tubular injury are diverse, various treatment approaches are needed according to the pathogenesis. Renal biopsy is indispensable to determine not only the pathological diagnosis, but also the underlying mechanism, and to guide appropriate treatment. Most recently, one of the red yeast supplements has been widely highlighted as a novel cause of tubular damage, mainly in Japan and Asia. However, neither detailed pathological findings nor the mechanism of renal impairment has been sufficiently reported.

Case presentation: Two cases of renal impairment after taking red yeast supplement internally are presented. Both cases showed renal dysfunction with low uric acid, potassium, and phosphorus levels, characteristic features of Fanconi syndrome. The renal biopsy findings of both cases showed severe injury to the proximal tubules with mild inflammatory cell infiltration. The proximal tubules exhibited diffuse loss of the brush border, flattening, and tubular lumen dilation. Immunofluorescence showed no deposition of immunoglobulin and complement in the glomeruli and tubules. Electron microscopic findings indicated proximal tubular damage without crystal deposition. Moreover, immunohistochemistry using the proximal tubular marker CD10 and a marker for distal tubules including the loop of Henle, E-cadherin, collectively demonstrated that the focus of renal injury in both cases was mainly the proximal tubules.

Conclusions: The red yeast rice supplement itself, its metabolized product, or other unknown contaminant components might directly induce proximal tubulopathy rather than an allergic reaction-related tubulointerstitial nephritis.