{"title":"Case report: Patiromer-induced hypercalcemia","authors":"M. Wiederkehr, Ankit N. Mehta, M. Emmett","doi":"10.5414/CNCS109782","DOIUrl":"https://doi.org/10.5414/CNCS109782","url":null,"abstract":"Patiromer is a novel potassium-binding compound which has recently received FDA approval. This ion exchange resin releases calcium when it binds potassium. We describe the development of hypercalcemia after initiation of patiromer. The calcium levels fell when the drug was stopped but recurred when it was later resumed. Patiromer was again discontinued, and the serum calcium level fell back into the normal range. We believe this patient manifested patiromer-induced hypercalcemia.","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"22 1","pages":"51 - 53"},"PeriodicalIF":0.0,"publicationDate":"2019-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83260382","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":"ANCA vasculitis presenting with acute interstitial nephritis without glomerular involvement","authors":"Callie Plafkin, Weixiong Zhong, T. Singh","doi":"10.5414/CNCS109805","DOIUrl":"https://doi.org/10.5414/CNCS109805","url":null,"abstract":"ANCA-associated vasculitis (AAV) with renal involvement typically causes pauci-immune glomerulonephritis. We present a case of acute interstitial nephritis (AIN) as the sole renal lesion without glomerulonephritis with myeloperoxidase (MPO) AAV. A 45-year-old female with history of Crohn’s disease, gastroesophageal reflux disease (GERD) with Barrett’s esophagus, pulmonary embolism, and polyarthralgias was evaluated in nephrology clinic in 2018. AIN without glomerulonephritis was first diagnosed in 2011 on renal biopsy. p-ANCA was positive with MPO titer of > 100 U/mL. Serum creatinine improved from 2.1 to 0.9 mg/dL with prednisone and azathioprine. Repeat biopsy in 2013 for worsening renal function showed AIN without glomerular involvement. Serum creatinine improved from 1.9 to 1.2 mg/dL with prednisone and cyclosporine. Crohn’s disease was diagnosed in 2014. AIN was attributed to Crohn’s, and cyclosporine was stopped in 2016. Adalimumab was started in 2016, without improvement in renal function or urine sediment. Attempt was made to switch proton pump inhibitor (PPI) to H2-blocker, but the latter was not tolerated. Repeat biopsy in 2/2018 showed AIN with severe fibrosis and tubular atrophy and glomerulosclerosis but no active glomerular disease. MPO titers remained high at 132 U/mL. Mycophenolic acid and prednisone were started without response, followed by rituximab for AAV-associated AIN. Serum creatinine worsened to 6.0 mg/dL in 9/2018, with plan to start peritoneal dialysis. AAV may present with isolated AIN without glomerular involvement. The rarity of this presentation may contribute to delay in appropriate management. Alternative explanations for AIN, such as Crohn’s disease or PPI use should be considered with caution in the setting of high-titer ANCA positivity.","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"11 1","pages":"46 - 50"},"PeriodicalIF":0.0,"publicationDate":"2019-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75457366","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}
H. Selvaskandan, Krishanantham Ambalawaner, R. Westacott
{"title":"Diabetic myonecrosis: A case series of two dialysis-dependent patients","authors":"H. Selvaskandan, Krishanantham Ambalawaner, R. Westacott","doi":"10.5414/CNCS109626","DOIUrl":"https://doi.org/10.5414/CNCS109626","url":null,"abstract":"Diabetic myonecrosis (DMN) is a rare microangiopathic disorder that can present as an acutely painful and swollen limb in patients with established diabetes mellitus. The condition can be diagnosed noninvasively with magnetic resonance imaging and resolves with analgesia, bed rest, and glycemic control. Due to a relative lack of awareness regarding the condition, avoidable interventions such as muscle biopsies and even surgery are sometimes pursued, which have been associated with prolonged recovery times. The majority of patients with DMN have diabetic nephropathy, yet this condition is not widely recognized in the nephrology community, resulting in delayed diagnosis and patients undergoing unnecessary and potentially harmful investigations. There is therefore a need for increased awareness of the condition among renal physicians. Here, we report the cases of two patients on hemodialysis who were ultimately diagnosed with DMN, along with a review of the literature.","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"78 1","pages":"41 - 45"},"PeriodicalIF":0.0,"publicationDate":"2019-07-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80052758","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":"Comorbidity of inflammatory bowel disease with atypical hemolytic uremic syndrome in pediatric patients","authors":"H. Stella Shin, C. Nester, Bradley P. Dixon","doi":"10.5414/CNCS109511","DOIUrl":"https://doi.org/10.5414/CNCS109511","url":null,"abstract":"Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy mediated by dysregulation of the alternative complement pathway. Complement-amplifying conditions such as respiratory and gastrointestinal infections, pregnancy, malignancy, and systemic autoimmune diseases such as systemic lupus erythematosus have been associated with the clinical manifestation of aHUS. Inflammation of the gastrointestinal tract is a potent stimulus for complement activation, and we describe a series of three pediatric patients with aHUS and comorbidity of inflammatory bowel disease (IBD). In two of the three cases, the diagnosis of aHUS preceded the diagnosis of IBD, perhaps suggesting a mechanistic link between complement dysregulation and thrombotic microangiopathy in the gastrointestinal tract and the ensuing inflammatory changes of IBD.","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"32 1","pages":"35 - 40"},"PeriodicalIF":0.0,"publicationDate":"2019-06-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88543921","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}
Júlia Guasti P Vianna, Thiago Gabriel Simor, Pamella Senna, Michell Roncete De Bortoli, Everlayny Fiorot Costalonga, Antonio Carlos Seguro, Weverton Machado Luchi
{"title":"Atypical presentation of familial hypomagnesemia with hypercalciuria and nephrocalcinosis in a patient with a new claudin-16 gene mutation.","authors":"Júlia Guasti P Vianna, Thiago Gabriel Simor, Pamella Senna, Michell Roncete De Bortoli, Everlayny Fiorot Costalonga, Antonio Carlos Seguro, Weverton Machado Luchi","doi":"10.5414/CNCS109595","DOIUrl":"https://doi.org/10.5414/CNCS109595","url":null,"abstract":"<p><p>Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is an autosomal recessive tubular disorder caused by mutations in genes that encode renal tight junction proteins claudin-16 or claudin-19, which are responsible for magnesium and calcium paracellular reabsorption in the thick ascending limb of Henle's loop. Progressive renal failure is frequently present, and most of the patients require renal replacement therapy still during adolescence. In this case report, we describe a new homozygous missense mutation on <i>CLDN16</i> gene (c.592G>C, Gly198Arg) in a 24-year-old male patient diagnosed with FHHNC after clinical investigation due to incidental detection of altered routine laboratorial tests, who was firstly misdiagnosed with primary hyperparathyroidism. In addition, it illustrates an atypical presentation of this disease, with late onset of chronic kidney disease, improving the phenotype-genotype knowledge of patients with FHHNC.</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"27-34"},"PeriodicalIF":0.0,"publicationDate":"2019-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5414/CNCS109595","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37264198","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}
Sean Hacking, Nupur N Uppal, Neelofar Khan, Marina Ionescu, Vanesa Bijol
{"title":"Systemic p-ANCA vasculitis with fatal outcome, arising in the setting of methimazole use.","authors":"Sean Hacking, Nupur N Uppal, Neelofar Khan, Marina Ionescu, Vanesa Bijol","doi":"10.5414/CNCS109759","DOIUrl":"https://doi.org/10.5414/CNCS109759","url":null,"abstract":"<p><p>Here we report a fatal case of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) due to methimazole use in a 64-year-old woman. She was initially hospitalized for abdominal pain and possible colitis, and subsequently developed hematuria, renal failure, and hemoptysis. The serologic work-up revealed positive antinuclear antibody (ANA) and perinuclear-antineutrophilic cytoplasm antibodies (p-ANCA), with positive antimyeloperoxidase. Three weeks following admission, the patient was found to be pulseless, and expired. At autopsy, microscopic review included widespread transmural necrotizing vasculitis and crescentic glomerulonephritis in the kidney, and diffuse pulmonary alveolar hemorrhage; focal coronary artery intimal vasculitis and necrotizing pericarditis were also noted. Several drugs have been associated with the development of ANCA-positive diseases, including propylthiouracil, hydralazine, allopurinol, penicillamine, and levamisole in cocaine. Association of ANCA vasculitis with methimazole exposure is less known, and severe presentation with fatal outcome, as seen in our patient, is exceedingly rare. We reviewed clinical and histopathologic features of drug-induced ANCA vasculitis associated with methimazole to raise awareness of this potentially life-threatening complication associated with this agent.</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"23-26"},"PeriodicalIF":0.0,"publicationDate":"2019-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6470634/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37174041","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":"Successful Tenckhoff catheter salvage in a patient with peritoneovesical fistula: A case report.","authors":"Vamsikrishna Makkena, Varun Kumar Bandi, Deepashree G Anandkumar, Renuka Prasad Yelahanka, Manikantan Shekar, Ramprasad Elumalai, Jayakumar Matcha","doi":"10.5414/CNCS109656","DOIUrl":"https://doi.org/10.5414/CNCS109656","url":null,"abstract":"<p><strong>Introduction: </strong>Many techniques are available for inserting peritoneal dialysis (PD) or continuous ambulatory peritoneal dialysis (CAPD) catheters, with varying possible complications. We report a case of bladder perforation that was managed with catheter salvage.</p><p><strong>Case report: </strong>A 48-year-old man with end-stage renal disease (ESRD) underwent CAPD catheter placement percutaneously, with tip in the pelvis. On the 3<sup>rd</sup> day after placement, the patient complained of increase in urinary volume with PD flushing. Urine analysis showed 3(+) glucose and absent creatinine. Cystogram showed the catheter abutting the bladder wall. CT of the abdomen showed the catheter piercing the bladder and exiting through the posterior wall. The PD catheter was repositioned under fluoroscopy.</p><p><strong>Discussion: </strong>The complications surrounding insertion of CAPD catheter can be either mechanical or infectious. Peritoneo-vesical fistula or placement of the PD catheter into the urinary bladder is a very rare complication. The possibility of catheter salvage should be entertained while discussing management options.</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"17-22"},"PeriodicalIF":0.0,"publicationDate":"2019-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472324/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37174040","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}
Nicolas Hanset, Philippe Hantson, Franck Saint-Marcoux, Arnaud Devresse, Michel Jadoul, Laura Labriola
{"title":"Influence of hemodialysis on pramipexole pharmacokinetics: Lessons from two cases and literature review.","authors":"Nicolas Hanset, Philippe Hantson, Franck Saint-Marcoux, Arnaud Devresse, Michel Jadoul, Laura Labriola","doi":"10.5414/CNCS109641","DOIUrl":"https://doi.org/10.5414/CNCS109641","url":null,"abstract":"<p><strong>Background: </strong>Restless legs syndrome (RLS) is not a rare condition in patients on long-term dialysis. Pramipexole is a small molecule used in the treatment of idiopathic and uremic RLS. Although some information concerning the efficacy and safety of pramipexole in uremic patients is available, data concerning the pharmacokinetics of pramipexole in hemodialysis (HD) are lacking. Following the occurrence of accidental pramipexole intoxication in a chronic HD patient, we were concerned about the efficacy of HD in removing pramipexole. Our aim was thus to assess plasma pramipexole concentrations and pramipexole clearance in a stable chronic HD patient without any residual kidney function.</p><p><strong>Materials and methods: </strong>Our patient was a 63-year-old man on chronic HD for 5 years who had been treated uneventfully with oral pramipexole for uremic RLS since then. During a routine 4-hour high-flux HD session, blood, ultrafiltrate, and dialysate samples were collected every hour to determine pramipexole concentrations over time.</p><p><strong>Results: </strong>Pramipexole blood concentrations ranged from 12.1 to 23.9 µg/L. Pramipexole reduction ratio was 32.5%. Mean dialytic clearance of pramipexole was 76.8 mL/min. Postdialysis rebound was 5.6%.</p><p><strong>Conclusion: </strong>In the absence of any side effect, pramipexole blood concentrations at steady state were 2- to 4-fold higher than those observed in subjects with normal kidney function. Like other drugs with a high volume of distribution, pramipexole was poorly removed by HD. Therefore, HD is not recommended as a treatment option for pramipexole intoxication in patients with a glomerular filtration rate superior to 30 mL/min/1.73m².</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"11-16"},"PeriodicalIF":0.0,"publicationDate":"2019-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448051/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37174039","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}
Karim Soliman, Johann Herberth, Tibor Fülöp, Angie Duong, Rachel L Sturdivant
{"title":"Refractory ascites as a presenting feature of extramedullary plasmacytoma in an end-stage renal disease patient with HIV infection.","authors":"Karim Soliman, Johann Herberth, Tibor Fülöp, Angie Duong, Rachel L Sturdivant","doi":"10.5414/CNCS109560","DOIUrl":"https://doi.org/10.5414/CNCS109560","url":null,"abstract":"<p><p>Refractory ascites as the only presenting feature of an extramedullary plasmacytoma complicating end-stage renal disease and HIV infection has not been described yet. We describe a case of a 39-year-old female with HIV-associated nephropathy manifesting with ascites formation after transition from peritoneal dialysis (PD) to hemodialysis (HD). Earlier on, she received cycler-assisted PD for 5 years uneventfully. A few weeks after HD transition, a striking refractory ascites developed requiring multiple paracenteses (5 - 7 L every second week). Serum protein electrophoresis showed hypoalbuminemia with only small amount of monoclonal IgG-κ at 0.30 g/dL. Serum immunofixation electrophoresis showed polyclonal immunoglobulins with polyclonal light chains. Both κ and λ light chains were increased, at 66.86 mg/dL (reference range: 0.33 - 1.94) and 18.55 mg/dL (reference range: 0.57 - 2.63), respectively, with a ratio of 3.6 (reference range: 0.26 - 1.65). However, an ascitic fluid analysis showed a marked increase in plasma cells with a κ : λ ratio greater than 5 : 1. Omental biopsy confirmed κ-restricted plasma cells. Multiple myeloma work-up with skeletal survey showed no evidence of focal osseous lesions, while bone marrow aspiration and biopsy also remained unremarkable. Accordingly, the diagnosis of omental extramedullary plasmacytoma with malignant ascites was confirmed. Conversion from PD to HD may unmask an underlying pathology favoring ascites formation.</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"7-10"},"PeriodicalIF":0.0,"publicationDate":"2019-02-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374990/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37028201","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}
Sai Sudha Mannemuddhu, William Clapp, Renee Modica, Melissa E Elder, Kiran Upadhyay
{"title":"End-stage renal disease secondary to anti-glomerular basement membrane disease in a child with common variable immunodeficiency.","authors":"Sai Sudha Mannemuddhu, William Clapp, Renee Modica, Melissa E Elder, Kiran Upadhyay","doi":"10.5414/CNCS109510","DOIUrl":"https://doi.org/10.5414/CNCS109510","url":null,"abstract":"<p><strong>Background: </strong>Anti-glomerular basement membrane (GBM) disease is caused by autoantibodies against the α3-chain of type IV collagen in the GBM. Common variable immunodeficiency (CVID) is a primary immunodeficiency manifested by hypogammaglobulinemia, inability to make functional antibody, and recurrent infections. This report extends the phenotype of CVID-associated autoimmune diseases to include anti-GBM disease.</p><p><strong>Case presentation: </strong>A 15-year-old Caucasian female with prior normal renal function presented with nephrotic proteinuria, pedal edema, oliguria, acute kidney injury, and was found to have positive serum anti-GBM antibody. She had been diagnosed with CVID at 3 years of age. Her renal biopsy showed crescentic glomerulonephritis (50%), and immunofluorescence showed linear staining for IgG along the glomerular capillary wall. There was no clinical or imaging evidence of pulmonary hemorrhage. She was treated with pulse IV steroids, cyclophosphamide, rituximab, and several sessions of plasmapheresis. Her serum anti-GBM antibody level decreased from 194 U/mL at presentation to 0 U/mL after therapy. However, she progressed to end-stage renal disease (ESRD) within weeks, despite aggressive therapy, and required chronic renal replacement therapy in the form of dialysis. Her clinical course was also complicated by hypertensive encephalopathy, CMV viremia and meningoencephalitis, status epilepticus, and she passed away a few months later from lower respiratory tract complications.</p><p><strong>Conclusion: </strong>Anti-GBM disease is a rare autoimmune condition that has not been reported in association with a primary immunodeficiency syndrome. ESRD secondary to anti-GBM disease in a patient with CVID is an interesting association and supports the role of immune dysregulation in systemic autoimmune disease.</p>","PeriodicalId":10398,"journal":{"name":"Clinical Nephrology. Case Studies","volume":"7 ","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2019-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37028200","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}