Systemic lupus erythematosus (SLE) and ulcerative colitis (UC) are multisystem autoimmune disorders that rarely coexist. We report a case history of a 21-year-old male, presenting with bloody diarrhea and, later, diagnosed to have ulcerative colitis on colonic biopsy. There was clinically silent renal impairment leading to end-stage kidney disease requiring hemodialysis possibly secondary to ongoing lupus nephritis as suggested by positive lupus-specific antibodies' detection. Besides this, the diagnosis of lupus associated with early communicating hydrocephalus was made on CT brain findings which clinically responded well to the initiation of immunosuppressive therapy. It is imperative to keep in mind the remote possibility of ulcerative colitis in an SLE patient with gastrointestinal (GI) manifestations. Communicating hydrocephalus is a rare neurological manifestation of SLE leading to seizures and can respond well to the initiation of steroids and immunosuppressants. Therefore, a trial of immunosuppressant medications must be given even in a patient with end-stage renal disease (ESRD) to halter extra renal rare lupus manifestations.
Acute oxalate nephropathy (AON) induced by high dietary intake of oxalate-rich food is a rare cause of acute kidney injury and end-stage renal disease (ESRD). We describe a 68-year-old man with adequate baseline renal function who developed severe AON and ESRD. Six months earlier, he started a daily oxalate-rich fruit and vegetable juice diet high in spinach, with a calculated daily oxalate dietary intake of 1500 mg, about 10 times a typical diet. Renal biopsy showed extensive tubular oxalate deposits and acute tubular damage; the renal tissue was relatively free of chronic changes such as glomerulosclerosis, tubular atrophy, and interstitial fibrosis. A year later, he remains dialysis dependent.
Introduction: C. auris has been associated not only with a variety of invasive fungal infections, including candidemia, sometimes related to central venous catheter, but also with pericarditis and respiratory tract and urinary tract infections.
Materials and methods: We describe the case of a patient with persistent fever despite antibiotics, who presented with Candida isolation in blood cultures, typified as Candida auris species.
Results: A 57-year-old male receiving peritoneal dialysis underwent kidney transplantation which was complicated by primary nonfunction due to arterial thrombosis necessitating graft nephrectomy. During the postoperative period, he presented with Pseudomonas aeruginosa pneumonia that was treated with levofloxacin and catheter-related Enterococcus faecalis bacteremia treated with linezolid. After hospital discharge, he then presented with herpes zoster infection treated with valacyclovir. Ten days later, he developed peritonitis and exit site infection with multidrug-resistant Pseudomonas aeruginosa treated with intraperitoneal aztreonam and peritoneal dialysis catheter removal. Despite broad-spectrum antibiotic therapy, the patient remained febrile. All microbiology laboratory tests were negative, so it was decided to stop antibiotic therapy for 48 hours and repeat cultures in order to avoid possible false negatives. In new blood cultures performed after suspension of antibiotic therapy, candidemia was observed, later typified as Candida auris species. After completing antifungal treatment (three weeks with intravenous amphotericin B 100 mg qd and two weeks of intravenous anidulafungin 100 mg qd), microbiological cultures remained negative and the patient made uneventful recovery.
Conclusion: Candida auris invasive infection has been mainly described in patients with severe underlying comorbidities and immunocompromise. Multidrug-resistant clusters of Candida auris are increasingly emerging.
Background: Alport syndrome results from a hereditary defect of collagen IV synthesis. This causes progressive glomerular disease, ocular abnormalities, and inner ear impairment. Case Presentation. Herein, we present a case of Alport syndrome in a 28-year-old woman caused by a novel mutation (Gly1436del) in the COL4A4 gene that was not unveiled until her first pregnancy. Within the 29th pregnancy week, our patient presented with massive proteinuria and nephrotic syndrome. Light microscopic examination of a kidney biopsy showed typical histological features of segmental sclerosis, and electron microscopy revealed extensive podocyte alterations as well as thickness of glomerular basement membranes with splitting of the lamina densa. One and a half years after childbirth, renal function deteriorated to a preterminal stage, whereas nephrotic syndrome subsided quickly after delivery.
Conclusion: This case report highlights the awareness of atypical AS courses and emphasizes the importance of genetic testing in such cases.
Background: We report and review the literature of two rare complications of simultaneous pancreas-kidney transplantation (SPKT) occurring in one patient. Case Report. A 39-year-old man with dialysis-dependent kidney failure secondary to type 1 diabetes mellitus underwent successful SPKT in October 2018. Three months later, he presented with an acute kidney injury (AKI) and returned to dialysis. Kidney scintigraphy showed a central photopenic region, and angiograms showed absent flow in the kidney transplant artery without treatable thrombus and the incidental finding of two pseudoaneurysms of the pancreatic Y-graft. He remained dialysis-dependent for three weeks before spontaneous partial recovery of allograft function; repeat kidney scintigraphy showed significant improvement in perfusion. However, in April 2019 he was readmitted with a sudden deterioration in kidney allograft function again necessitating haemodialysis. Repeat imaging confirmed that the kidney allograft had shifted from the left iliac fossa to the midline. He underwent surgical exploration, during which torsion of the kidney allograft was confirmed and a nephropexy was performed. The kidney allograft was originally implanted in the left retroperitoneum via a midline transperitoneal approach, which likely predisposed it to torsion. The pseudoaneurysms of the pancreatic Y-graft were managed conservatively, and surveillance imaging demonstrated that they remained stable in size. The patient regained reasonable kidney allograft function (estimated glomerular filtration rate, eGFR, of 45 mL/min) and maintained normal pancreatic allograft function.
Conclusion: Kidney allograft torsion should be considered post-SPKT in patients with AKI and absent arterial flow. Although most case reports describe surgical management of pseudoaneurysms post-SPKT, our case demonstrates successful conservative management.
A 32-year-old female was admitted to our institution with thrombocytopenia, fever, serositis, hepatosplenomegaly, diffuse lymphadenopathy, and renal insufficiency. A diagnosis of systemic lupus erythematosus was made. Due to recalcitrant thrombocytopenia, serositis, and renal insufficiency methylprednisolone was prescribed in high doses. In addition to proteinuria and hematuria, she was found to have uric acid crystals in her urinalysis. A serum uric acid was found elevated at 18 mg/dL. Rasburicase infusions were started. Within 5 days of commencing rasburicase and continuing high-dose methylprednisolone, her serum creatinine normalized and proteinuria resolved. The microhematuria disappeared within 2 weeks of beginning rasburicase. The rapid reversal of renal insufficiency and all urinary abnormalities after the start of rasburicase infusions suggests that the renal injury was most likely due to uric acid-mediated renal injury and not lupus nephritis. Our case illustrates the co-occurrence of 2 distinct clinical entities, one common for the patient's age, sex, and foremost clinical findings, while the other uncommon and unexpected, but both associated to kidney injury. Clinicians must be aware that careful evaluation of symptoms and laboratory tests is needed to make a thorough differential diagnosis and provide the right treatment at the most opportune moment.
Nafamostat mesylate (NM) has been used to treat pancreatitis and disseminated intravascular coagulation during hemodialysis (HD). However, there have been some reports of adverse effects related to anaphylactic reactions. We present a case in which anaphylactic reactions caused by NM during preoperative HD caused repeated postponement of surgery for carpal tunnel syndrome. Symptoms including fever, shivering, chills, low blood pressure, tachycardia, nausea, and vomiting appeared during preoperative HD, and surgery was postponed thrice. Initially, the patient was misdiagnosed with sepsis because of elevated C-reactive protein and procalcitonin levels. However, since the symptoms appeared only when NM was administered and disappeared quickly after the administration of NM was terminated, the condition was diagnosed as anaphylactic reactions caused by NM. Therefore, it is essential to consider anaphylactic reactions caused by NM as differential diagnoses, when symptoms, such as fever, are observed during perioperative HD.
Full-house immunofluorescence and endothelial tubuloreticular inclusions are known as characteristic features of lupus nephritis. However, both features are not pathognomonic for lupus nephritis. A kidney biopsy specimen showing full-house immunofluorescence pattern in the absence of autoantibodies and classical clinical features of Systemic Lupus Erythematosus (SLE) is now considered as nonlupus full-house nephropathy (FHN). Nonlupus FHN may be idiopathic or due to other disease processes known as secondary nonlupus FHN. Here, we report the case of a 36-year-old female who presented with nephrotic proteinuria with bland urine sediment. Additional analyses revealed normal serum antinuclear antibody (ANA), normal anti-double-stranded DNA (anti-dsDNA) antibodies, and normal serum C3 and C4 levels. A renal biopsy showed a normal-appearing glomerulus without any proliferation or capillary wall thickening and widespread glomerular immune deposits (full-house effect; IgA, IgG, IgM, C3, and C1Q) on direct immunofluorescence. Renal electron microscopy showed diffuse effacement of visceral epithelial cell foot processes and mesangial electron dense deposits. The patient was diagnosed as nonlupus FHN. There is a controversial role of steroids and other immunosuppressive drugs in the treatment of nonlupus FHN patients, but our case patient responded favourably to steroid therapy. The term nonlupus FHN can be used as an umbrella term for patients who do not satisfy the clinical and serological criteria of SLE.