Case Reports in Nephrology and Dialysis最新文献

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.

Introduction: Latvia faces a challenging shortage of available kidney donors, leading to a significant mismatch between demand for kidney transplantation and supply. Although older adult donors require a thorough pre-donation workup to rule out significant medical comorbidities, it offers hope for potential kidney transplantation candidates.

Case presentation: This case study presents the unique scenario of an 87-year-old living kidney donor, where individualized decision-making resulted in outstanding outcomes for both the donor and recipient.

Conclusions: The initial assessment for donation, which involves renal scintigraphy, serves as a preventive measure. In cases where one of the kidneys exhibits insufficient function, this approach avoids the necessity for further costly tests, thus preserving resources in the healthcare budget. The decision concerning an older donor should undergo thorough discussion by a multidisciplinary team to minimize perioperative and long-term risks. Nonetheless, a thoughtful approach to elderly donors offers a valuable opportunity to expand the living donor pool in the context of the organ shortage problem.

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease and the 4th leading cause of renal replacement therapy in the world. ADPKD is a systemic disorder as cysts may develop in several organs. Liver cysts are the most common extrarenal manifestations and are often incidentally detected. Even though cysts do not influence liver function, they can grow to a very great size and can significantly enlarge liver volume, causing structural distortion of the biliary tree and patient discomfort due to the mass effect. Nephrectomy is frequently considered in preparation for renal transplantation in patients with remarkable kidneys' enlargement. There are currently no globally recognized clinical guidelines for nephrectomy. Although cysts do not normally affect liver function in ADPKD, after nephrectomy cases of liver fibrosis and Budd-Chiari have been reported. These are uncommon disorders due to the obstruction of the blood flow in the hepatic venous causing spleen and liver volume enlargement, portal hypertension, and hepatic cirrhosis.

Case presentation: We present a case of hepatic fibrosis with splenomegaly and severe pancytopenia as a tardive complication after bilateral nephrectomy in 47-year-old ADPKD patient.

Conclusion: This finding underscores the critical significance of meticulously examining the anatomical relationship between polycystic kidneys and the liver before performing nephrectomy. Additionally, it highlights the importance of assessing liver involvement and associated complications. By integrating liver assessment into the criteria, we can significantly enhance patient care and improve the overall management of ADPKD before kidney transplantation.

Introduction: Anti-glomerular basement membrane (GBM) disease is a rare cause of glomerulonephritis usually mediated by IgG antibodies and is associated with ANCA-associated glomerulonephritis in up to 50% of cases. IgA-mediated anti-GBM disease is extremely rare and presents diagnostic difficulties as circulating IgA antibodies will not be detected by standard serological tests for anti-GBM disease.

Case presentation: We present the case of a 67-year-old man with rapidly progressive glomerulonephritis requiring haemodialysis at presentation. Serological testing was positive for anti-myeloperoxidase and negative for IgG anti-GBM antibodies. Kidney biopsy revealed necrotizing crescentic glomerulonephritis with linear staining of IgA along the GBM. He was treated with a combination of immunosuppression and plasma exchange and was able to become dialysis-independent.

Conclusion: To our knowledge, this is the first documented "double-positive" IgA anti-GBM disease and ANCA-associated glomerulonephritis.

Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, which is mainly caused by pathogenic variants in two particular genes: PKD1 and PKD2. ADPKD caused by variants in other genes (GANAB or IFT140) is very rare.

Case report: In a 6-year-old girl examined for abdominal pain, a cystic mass in the upper part of the right kidney was detected during an abdominal ultrasound. She was referred to pediatric oncology and urology for suspicion of a tumorous mass and the condition was assessed as a cystic nephroma. A heminephrectomy was then performed on the upper cystic part of the right kidney. The histological examination was inconclusive; therefore, genetic testing was recommended. Kidney and liver cysts were detected sonographically in the mother, but DNA analysis of the PKD1 and PKD2 genes did not reveal any pathogenic variant; the cause of the pathological formation in the kidneys remained unclear. Nine years later, next-generation sequencing of a panel of genes for kidney disease was performed and a heterozygous deletion was found on chromosome 16; this included exon 13 of the IFT140 gene. The same deletion was found in the patient's mother. Currently, the patient is 14 years old and has mild sonographic findings, normal glomerular filtration, mild proteinuria, and hypertension.

Conclusion: Pathogenic variants of the IFT140 gene very rarely cause ADPKD; however, they should be considered in all children with autosomal dominant forms of PKD and asymmetric/atypical cystic kidney involvement or negative findings of PKD1 and PKD2.

Introduction: Hemolytic uremic syndrome (HUS) is characterized by progressive kidney injury accompanied by thrombotic microangiopathy, which is clinically defined as microangiopathic hemolytic anemia with thrombocytopenia and organ injury. Shiga toxin-producing Escherichia coli (STEC)-HUS is caused by infection with pathogenic E. coli strains, typically O157, O26, and O111. However, the prevalence of other types of pathogenic E. coli has been increasing, and these pathogens sometimes cause atypical clinical manifestations of STEC-HUS.

Case presentation: We report the case of a 3-year-old girl diagnosed with STEC-HUS associated with a rare O80:H2 stx2 serotype, characterized by an atypical clinical course. She presented with severe hemolytic anemia and mild renal dysfunction but did not have enterohemorrhagic diarrhea. The first culture test of her stool sample collected using a swab upon admission yielded no signs of STEC, leading to an initial diagnosis of atypical HUS; thus, eculizumab was administered adding to red blood cell transfusion and recombinant thrombomodulin alfa and haptoglobin. However, a subsequent culture test of her second stool sample revealed the presence of O80:H2 stx2, confirming the diagnosis of STEC-HUS. Subsequently, the patient's condition improved, and her serum creatinine level gradually normalized over the course of 3 months.

Conclusion: Diligently diagnosis is crucial in cases lacking typical STEC-HUS symptoms. We advocate for repeated stool culture testing to ensure accurate identification and timely management of such cases.

Introduction: Salbutamol is a moderately selective beta-2-adrenergic agonist. Various side effects can occur because of beta-1 and beta-2 receptor activation. Due to the large volume of distribution, it is not considered dialyzable.

Case presentation: A patient with salbutamol intoxication, which developed as a result of a medical error in a patient with sepsis, Down syndrome, and liver cirrhosis, is presented. Initial treatment was partially successful and antibiotic adjustments were made. After his respiratory failure worsened, the patient needed non-invasive ventilation, and previously undiagnosed chronic obstructive pulmonary disease was suspected. He was prescribed intravenous methylprednisolone but accidently received 5 mg of salbutamol (albuterol), which led to immediate severe arrhythmic tachycardia with hemodynamic collapse. After unsuccessful cardioversion and treatment with landiolol infusion, salvage hemodialysis was commenced to decrease suspectedly highly elevated serum salbutamol levels. After 30 min, sinus rhythm with normocardia was observed. After the hemodialysis termination, no rebound tachycardia was noted, but due to severe septic shock, the hypotension was ongoing and vasoactive medications were adjusted. However, the measured levels of plasma salbutamol and data from literature do not support the view that hemodialysis was the cause of the described improvement: the total amount of the drug cleared was very small (2.8% of total dose).

Conclusion: Our results confirm a large volume of salbutamol distribution; the measured levels are within observed therapeutic levels; and the measured half-life time during hemodialysis (3.1 h) is comparable to observed half-life times in therapeutic settings. The observed favorable clinical benefit associated with dialysis may be fortuitous, highlighting potential bias toward positive clinical outcomes and unproven ("salvage") therapies.

Introduction: Emphysematous pyelonephritis is a rare but potentially life-threatening urinary tract infection characterized by the formation of gas in the renal parenchyma, collecting system, and perinephric tissue. The condition typically develops in patients with specific predisposing factors such as diabetes mellitus, congenital or acquired obstructive uropathies, or individuals taking immunosuppressive agents. Rarely can the disease occur in patients with other predisposing factors, such as the use of SGLT2 inhibitors, but this is quite uncommon. The incidence of urinary tract infections associated with their use is still debatable, but cases of emphysematous pyelonephritis associated with SGLT2 inhibitors have been described in medical literature.

Case presentation: We present a rare case of a patient with emphysematous pyelonephritis without classical risk factors for the disease, who was taking an SGLT2 inhibitor.

Conclusion: Although the frequency of urinary tract infections following the use of SGLT2 inhibitors is relatively low, their widespread application for treatment of numerous socially significant diseases underscores the necessity for specialists to be aware with all potential risks associated with their use, including the development of severe urinary tract infections.

Introduction: Acute pancreatitis is an infrequent but challenging cause of peritonitis in peritoneal dialysis (PD). Presentation is often indistinguishable from infectious peritonitis, interpretation of pancreatic enzymes is not straight-forward, and multiple etiologies need to be considered.

Case presentation: A 74-year-old PD patient presented with cloudy dialysate and subtle symptoms of malaise and abdominal pain. WBC was 26,000/µL, CRP was 250 mg/L, and dialysis effluent contained 1,047 leucocytes/μL (90% polymorphs). Infectious peritonitis was presumed, and antibiotic treatment started. However, dialysate cultures remained negative, effluent leucocyte count remained high, and clinical condition deteriorated. Abdominal ultrasound was unremarkable (pancreas not visible). Acute pancreatitis was diagnosed by elevated lipase level (serum: 628 U/L, dialysis fluid: 15 U/L) and CT scan. Disentangling etiological factors was challenging. The patient had gallstones, consumed alcoholic beverages, was recently on doxycycline and dialyzed with icodextrin. In addition, PD treatment itself may have been a contributory factor. Antibiotic therapy was stopped, and PD was temporarily suspended. Systemic and effluent markers of inflammation took 4 weeks to normalize. The patient did not regain his usual state of health until several weeks after discharge. Follow-up CT scan showed considerable pancreatic sequelae.

Conclusion: Acute pancreatitis is an important cause of PD peritonitis. Negative dialysate cultures and unsatisfactory clinical response should trigger evaluation for acute pancreatitis and its multiple potential causes, including PD treatment itself. Serum lipase levels >3 times ULN and elevated dialysis fluid lipase can be expected. Timely performance of imaging is advisable. Prognosis can be poor, and close monitoring is recommended.