Hana Flogelová, E. Karaskova, K. Bouchalová, M. Rohanova, V. Latalova, T. Tichý, V. Tesar
{"title":"Antineutrophil Cytoplasmic Autoantibody-Associated Glomerulonephritis as a Complication of Home Parenteral Nutrition","authors":"Hana Flogelová, E. Karaskova, K. Bouchalová, M. Rohanova, V. Latalova, T. Tichý, V. Tesar","doi":"10.1159/000522150","DOIUrl":"https://doi.org/10.1159/000522150","url":null,"abstract":"Patients on long-term home parenteral nutrition (HPN) occasionally develop glomerulonephritis due to chronic central venous catheter (CVC)-related infection. Most previously reported cases were membranoproliferative glomerulonephritis (MPGN). This is a case report of a 16-year-old girl receiving HPN for short bowel syndrome. After 11 years on HPN, she developed acute kidney injury with macroscopic hematuria, nephrotic-range proteinuria, and a reduced glomerular filtration rate (GFR). Initially, MPGN associated with chronic bacteremia was suspected with the assumption that the condition would be treated with antibiotics and CVC replacement. However, her kidney biopsy revealed antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis (AAG). This was consistent with the fact that the patient tested positive for proteinase 3-ANCA. Immunosuppressive therapy with methylprednisolone pulses (followed by oral prednisone) and rituximab led to remission. Her GFR and protein excretion returned to normal. Chronic bacteremia as a complication of long-term HPN may cause various types of glomerulonephritis including, rarely, AAG requiring immunosuppressive therapy.","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"12 1","pages":"22 - 30"},"PeriodicalIF":0.7,"publicationDate":"2022-03-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49340289","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Thinking Beyond Acute Kidney Injury","authors":"Emran El-Alali, Cesar Moreno, Emad Al Jaber","doi":"10.1159/000522312","DOIUrl":"https://doi.org/10.1159/000522312","url":null,"abstract":"Acute kidney injury (AKI) can be a significant clue to solving a puzzling patient presentation. Postrenal AKI should be suspected if imaging shows any degree of hydronephrosis and can be caused by a variety of conditions. Diagnosis of urinary obstruction without significant dilatation of the pelvic-ureteral system requires a higher degree of suspicion, and hence, its identification can become late. In patients without prior cancer screening, the etiology of obstructive uropathy must be broadened to include primary or metastatic malignancy. Clinicians should look beyond the AKI to properly evaluate the etiology of the patient’s presentation and symptoms. In this report, we present the case of a middle-aged female with no known past medical history who presented with AKI secondary to malignant retroperitoneal fibrosis as the first manifestation of metastatic breast cancer. Her AKI was associated with acute onset anuria and was found to have nondilated postrenal AKI with no significant abnormalities on renal imaging. Early onset anuria in the setting of AKI, which persists despite fluid resuscitation, can suggest complete urinary tract obstruction even with reassuring results of initial renal images, and in the patient with no history of cancer screening, malignancy should be suspected as a primary cause of obstructive uropathy.","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"12 1","pages":"16 - 21"},"PeriodicalIF":0.7,"publicationDate":"2022-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46386685","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
R. Chieochanthanakij, Wasin Manuprasert, N. Udomsantisuk, L. Pearson, T. Kanjanabuch
{"title":"Genetically Confirmed Edwardsiella tarda Peritonitis was Associated with Improper Caregiver's Hand Hygiene during Peritoneal Dialysis Bag Exchange","authors":"R. Chieochanthanakij, Wasin Manuprasert, N. Udomsantisuk, L. Pearson, T. Kanjanabuch","doi":"10.1159/000521351","DOIUrl":"https://doi.org/10.1159/000521351","url":null,"abstract":"Edwardsiella tarda is a Gram-negative bacillus and is responsible for waterborne disease. This is the first case report of peritoneal dialysis (PD)-associated peritonitis caused by genetically confirmed E. tarda, which was transmitted from the caregiver’s hand during PD bag exchange. Aside from that, the caregiver was a fishmonger and a gastrointestinal carrier of the pathogen. Prior to the onset of peritonitis, the caregiver reported that she did not wash her hands every time when performing the PD bag exchange. Although extraintestinal edwardsiellosis usually poses a poor outcome, PD-associated peritonitis with this species is paradoxical if diagnosed early, and treatment is promptly provided, as presented here. This case emphasizes the importance of hand hygiene in preventing environment-bound infection in patients on PD and demonstrates the unusual route of infection, contamination during PD bag exchange.","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"12 1","pages":"11 - 15"},"PeriodicalIF":0.7,"publicationDate":"2022-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44941076","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Babak S. Jazayeri-Moghadass, Ryan Sutherland, Lakir D. Patel, Valeriu Cebotaru
{"title":"Small Bowel Obstruction with a Transition Point in a Patient on Peritoneal Dialysis","authors":"Babak S. Jazayeri-Moghadass, Ryan Sutherland, Lakir D. Patel, Valeriu Cebotaru","doi":"10.1159/000521350","DOIUrl":"https://doi.org/10.1159/000521350","url":null,"abstract":"Small bowel obstruction (SBO) is a rare complication of peritoneal dialysis (PD) that is usually seen in patients with encapsulating peritoneal sclerosis. We present a case of SBO that was caused by mechanical obstruction from omental adhesions around the PD catheter. This is the case of 71-year-old female with end-stage renal disease who was recently started on PD and presented with recurrent syncopal episodes and altered mental status. During hospitalization, the patient began experiencing incomplete drainage of the PD solution. Abdominal computerized tomography revealed SBO with a transition point near the PD catheter. The patient then underwent laparoscopy, which revealed omental adhesions around the PD catheter near the obstruction area, but no adhesion of the intestine was observed. The adhesions were dissected by laparoscopy, and the PD catheter was removed. This case highlights the challenges of PD access.","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"12 1","pages":"6 - 10"},"PeriodicalIF":0.7,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41347812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Transient Hyperkalemia Following Treatment of Chronic Hypokalemia: A Case Report and Review of Distal Tubule Physiology","authors":"Matthew C. Breeggemann, S. Gluck","doi":"10.1159/000521477","DOIUrl":"https://doi.org/10.1159/000521477","url":null,"abstract":"Hypokalemia is a relatively common electrolyte disorder usually resulting from gastrointestinal wasting. Transient hyperkalemia in those treated for hypokalemia has been previously described to occur in 16% of hospitalized patients. The majority of those patients had acute, hospital-acquired hypokalemia. Here, we report a case of a young man with alcohol use disorder and chronic hypokalemia who was hospitalized for muscle weakness, abdominal pain, and intractable emesis. His potassium was 2.5 mEq/L on the day of admission. Four days later, with a creatinine at baseline (0.9 mg/dL), potassium abruptly increased to 6.7 mEq/L. He did not have evidence of hyperaldosteronism. In cases of chronic hypokalemia, we propose that the adaptive mechanisms of the distal tubule with total body potassium deficits require time to revert back to a nonactive state and that transient hyperkalemia may be observed during these “refractory” periods during which potassium supplementation is continued. The time required for disassembly of with no lysine kinases following resolution of hypokalemia is unknown. Hyperkalemia is an important consideration when treating patients with chronic hypokalemia.","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"12 1","pages":"1 - 4"},"PeriodicalIF":0.7,"publicationDate":"2022-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43783407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Reda Laamech, Florian Terrec, Camille Emprou, Anne Claire Toffart, Thomas Pierret, Hamza Naciri-Bennani, Lionel Rostaing, Johan Noble
{"title":"Efficacy of Plasmapheresis in Nivolumab-Associated ANCA Glomerulonephritis: A Case Report and Pathophysiology Discussion.","authors":"Reda Laamech, Florian Terrec, Camille Emprou, Anne Claire Toffart, Thomas Pierret, Hamza Naciri-Bennani, Lionel Rostaing, Johan Noble","doi":"10.1159/000518304","DOIUrl":"https://doi.org/10.1159/000518304","url":null,"abstract":"<p><p>Immune checkpoint inhibitors (ICIs) have revolutionized solid organ and hematologic cancer treatments by improving overall prognoses. However, they can lead to overactivation of the immune system and several immune-related adverse events and sometimes affecting the renal system. Although acute interstitial nephritis is well described, we know little about ICI-associated glomerular injury. Herein, we report an exceptional case of renal ANCA positive-associated vasculitis (AAV) after nivolumab therapy. Three weeks after the last nivolumab injection, the patient presented with proteinuria at 1.73 g/g of creatininuria, hematuria, and acute kidney injury needing dialysis associated with lung hemorrhage; anti-neutrophil cytoplasmic antibody (ANCA titer ≥1,280 with myeloperoxidase specificity of 780 U/mL) was positive, and kidney biopsy confirmed glomerular injury with crescents. The patient underwent treatment with steroid pulses, rituximab, and plasmapheresis, resulting in an improvement of the renal function and lung hemorrhage and produced a negative ANCA titer. Despite the results of the PEXIVAS study and the absence of clear benefit of plasmapheresis demonstrated in idiopathic AAV, we suggest that drug-induced AAV may be effectively treated by plasmapheresis, steroids, and rituximab.</p>","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"11 3","pages":"376-383"},"PeriodicalIF":0.7,"publicationDate":"2021-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/61/84/cnd-0011-0376.PMC8787507.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39883056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Acknowledgement to the Reviewers","authors":"","doi":"10.1159/000520195","DOIUrl":"https://doi.org/10.1159/000520195","url":null,"abstract":"","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"11 1","pages":"374 - 375"},"PeriodicalIF":0.7,"publicationDate":"2021-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49633506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Severe Acute Respiratory Syndrome Corona Virus-2 Infection in a Pediatric Kidney Transplant Recipient: A Case Report from India.","authors":"Ravi Raju Tatapudi, Venkateswara Rao Kopparti, Anusha Poosapati, Srinivas Metta, Vedita Palli, Balakrishna Vedulla","doi":"10.1159/000520558","DOIUrl":"https://doi.org/10.1159/000520558","url":null,"abstract":"<p><p>COVID-19 pandemic affected millions of people across India. COVID-19 cases are fewer in children with less severity and better outcomes than in adults. However, a small proportion develop severe illness and succumb to the disease. Clinical manifestations and optimal management of COVID-19 in immunocompromised children are not clearly known. Remdesivir was shown to be efficient in reducing the recovery time in COVID-19 patients requiring supplemental oxygen. Remdesivir is approved for use in children with severe COVID-19, but there are no guidelines in patients with risk factors like recent solid organ transplantation. We report a case of a 10-year-old kidney transplant recipient (KTR) infected with severe acute respiratory syndrome corona virus-2, 2.5 months after the transplantation. Unlike most children, he presented with high fever, cough, and vomiting. His inflammatory markers were elevated. In this case report, we discussed management and clinical outcomes of this patient. In view of recent kidney transplantation and the severity of infection with emergent oxygen requirement, we gave him remdesivir. We continued prednisolone and tacrolimus and stopped mycophenolate. He recovered completely in 7 days. We feel that severely immunosuppressed KTR children with COVID-19 will benefit with remdesivir administration. Monitoring tacrolimus trough levels is essential for maintaining adequate immunosuppression.</p>","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"11 3","pages":"367-373"},"PeriodicalIF":0.7,"publicationDate":"2021-12-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/11/b1/cnd-0011-0367.PMC8738907.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39724783","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mohammad Khaled Alsultan, Zeina Nizar Bdeir, Qussai Hassan, Tahani Ali
{"title":"Successful Kidney Transplant for Nephropathic Cystinosis in a Patient with Von Willebrand Disease Type III: The First Case Report.","authors":"Mohammad Khaled Alsultan, Zeina Nizar Bdeir, Qussai Hassan, Tahani Ali","doi":"10.1159/000520794","DOIUrl":"https://doi.org/10.1159/000520794","url":null,"abstract":"<p><p>Nephropathic cystinosis (NC) is a rare autosomal recessive disease, which causes cysteine-crystals accumulation with progression to end-stage renal disease (ESRD). Von willebrand disease (VWD) type III is a rare subtype of von willebrand factor (VWF) abnormality, which is characterized by severe reduction of VWF and factor VIII activity. A 16-year-old patient with NC and VWD type III presented with uremic symptoms due to ESRD. Dialysis access was inserted and followed by hemodialysis (HD) for 4 months with a proper infusion of blood products. While renal transplant remains the treatment of choice of NC and superior to chronic HD, bleeding complications were a major concern in this case with coexisting VWD type III. However, with the meticulous implementation of the Hematology team's daily recommendations, renal transplantation was successfully performed. This is the first case that mentions a new association between two inherited rare disorders, NC and VWD type III, and this entity has not been reported before. Moreover, successful kidney transplantation in our patient supports the possibility of these procedures in hereditary clotting disorders.</p>","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"11 3","pages":"362-366"},"PeriodicalIF":0.7,"publicationDate":"2021-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4d/14/cnd-0011-0362.PMC8739642.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39724784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Fabry Nephropathy in a Young Female Patient Presenting with Only Urinary Mulberry Bodies Treated with Chaperone Therapy.","authors":"Tsugumi Fukunaga, Shingo Nakayama, Takuo Hirose, Kishin Muramatsu, Akari Endo, Yuka Miyake, Go Anan, Ikuko Oba-Yabana, Atsuhiro Kanno, Hannah Nakamura, Junichi Tani, Kimitoshi Nakamura, Kazuhiro Sumitomo, Wako Yumura, Katsutoshi Furukawa, Takefumi Mori","doi":"10.1159/000520157","DOIUrl":"https://doi.org/10.1159/000520157","url":null,"abstract":"<p><p>Fabry disease (FD) is an X-linked disorder of the sphingolipid metabolism, caused by deficiency or decreased activity of α-galactosidase A. We report a rare case of Fabry nephropathy (FN) in a 21-year-old Japanese female patient presenting with only urinary mulberry bodies; she was treated with pharmacological chaperone therapy (PCT) after renal biopsy. The patient underwent a detailed examination because her mother was diagnosed with FD in the Division of Community Medicine of our hospital. She did not have renal dysfunction or proteinuria, and only mulberry bodies were detected in the urine. The activity of α-galactosidase A was low, and genetic analysis revealed the R301Q mutation. A percutaneous renal biopsy was performed, and the findings revealed enlargement and vacuolation of glomerular podocytes by light microscopy, and myelin and zebra bodies were detected in podocytes by electron microscopy. She was diagnosed with FN by renal biopsy and gene analysis. PCT was selected as the treatment to prevent cardiac events and renal dysfunction. The present case suggests that renal biopsy may be necessary even for young women with only mulberry bodies for the diagnosis of FN. It could be useful to evaluate the effect of treatment using the counts of mulberry bodies in the urine. In addition, due to its oral administration, PCT may be suitable for patients who are unable to visit the hospital frequently.</p>","PeriodicalId":9599,"journal":{"name":"Case Reports in Nephrology and Dialysis","volume":"11 3","pages":"355-361"},"PeriodicalIF":0.7,"publicationDate":"2021-11-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/21/ba/cnd-0011-0355.PMC8739663.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39863397","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}