Abstract: A 45-year-old obese man presented with persistent hematuria for 21 years. At the age of 37, he developed hypertension and proteinuria which later increased up to 1.6 g/g creatinine. Kidney biopsy revealed thin basement membrane nephropathy (TBMN) and focal segmental glomerulosclerosis (FSGS), which explained his urinary abnormalities. Although a subgroup of TBMN can be complicated by FSGS, his FSGS was associated with obesity because of its histological features. Reduction of body weight and increasing a dose of angiotensin-receptor blocker could transiently reduce the amount of proteinuria. Clinicopathological implications of proteinuria after long-term hematuria by TBMN and FSGS were further discussed. PubDate: Mon, 23 Dec 2019 14:50:00 +000
Abstract: Introduction. Antiangiogenic agents that inhibit vascular endothelial growth factor have emerged as important tools in cancer therapy and ocular diseases. Their systemic use can induce renal limited microangiopathy. Local use of anti-VEGF agent is supposed to be safe. We report here a unique case of early endothelial cells injury induced by intravitreal injection of bevacizumab. Case Presentation. A 72-year-old man was addressed for acute kidney injury with proteinuria. He was under treatment with intravitreal injections of bevacizumab for glaucoma. Kidney biopsy was performed and electron microscopy showed signs of early stages of glomerular microangiopathy. Bevacizumab was discontinued resulting in the improvement of renal function and albuminuria. Discussion. Bevacizumab, a humanized monoclonal antibody to VEGF is an approved therapy for metastatic cancer. Systemic adverse events including thrombotic microangiopathy have been mainly reported after its systemic injection. Podocytes produce VEGF that interacts with endothelial cells VEGF receptor-2 maintaining glomerular basement membrane integrity. Bevacizumab induce the detachment of endothelial cells from glomerular basement membrane leading to the proteinuria and renal function decline. Intravitreal bevacizumab is generally supposed to be safe. However, glomerular injury with microangiopathy features, even after intravitreal injection is possible. Conclusion. We report the electron microscopy evidence that intravitreal injection of anti-VEGF induces glomerular endothelial cells injury. Nephrologists and ophthalmologists should be aware of this complication. PubDate: Sun, 22 Dec 2019 08:35:01 +000
Abstract: Pasteurella multocida is an aerobic gram-negative coccobacillus usually found in the oral cavities of most healthy cats and dogs as part of their natural oral flora. This zoonotic pathogen can cause a variety of infections in humans through bites, scratches, or licking. Infections range from less severe cases, such as infected animal bites and cellulitis, to more severe cases of pneumonia, septic arthritis, osteomyelitis, sepsis, and meningitis. However, the number of reported cases of peritoneal dialysis-associated peritonitis caused by P. multocida has been limited worldwide. Here, we report the case of a 59-year-old man undergoing continuous cycling peritoneal dialysis who developed P. multocida peritonitis, believed to be secondary to domestic cat exposure to dialysis equipment. Due to the increasing trend of pet ownership, patients maintained on peritoneal dialysis should be educated on the importance of strict hygiene and avoiding pet contact with the dialysis equipment, especially in bag exchange areas. Although the best means of preventing such infections is to avoid having pets at home, the positive psychological effects of pet ownership should also be considered. Thus, patients in such situations should be continuously educated and encouraged to be mindful of the importance of environmental hygiene. PubDate: Mon, 16 Dec 2019 12:05:00 +000
Abstract: As a MYH9 disorder, Fechtner syndrome is characterized by nephritis, giant platelets, granulocyte inclusion bodies (Döhle-like bodies), cataract, and sensorineural deafness. Observation of peripheral blood smear for the presence of thrombocytopenia, giant platelets, and granulocyte inclusion bodies (Döhle-like bodies) is highly important for the early diagnosis of MYH9 disorders. In our two cases, sequencing analysis of the MYH9 gene indicated mutations in exon 24. Both cases were diagnosed as the MYH9 disorders Fechtner syndrome before end-stage renal failure on the basis of the observation of peripheral blood smear. PubDate: Tue, 26 Nov 2019 13:30:13 +000
Abstract: Cancer-associated thrombotic microangiopathy (TMA) refers to a group of disorders characterized by microangiopathic haemolytic anemia, thrombocytopenia, and ischemic organ damage. TMA manifestations can be induced by cancer or by chemotherapy. We report the case of a 64-year-old man with metastatic prostate cancer who experienced a Cabazitaxel-induced TMA manifestation. TMA responds to conservative therapy, dialysis without plasmaphoresis, with progressive recovered renal function. PubDate: Tue, 19 Nov 2019 11:05:03 +000
Abstract: Background. Sarcoidosis is a chronic disease characterized by noncaseating lesions involving any organ and tissue in the body. Hypercalcemia and acute kidney injury is a common renal presentation of sarcoidosis. Necrotizing sarcoid granulomatosis (NSG) is a granulomatous disease entity which presents with nodular masses of sarcoid like granuloma which primarily effects the lungs. It is a rare necrotizing variant of sarcoidosis. Extra pulmonary presentation of NSG is very rare. Case presentation. We present a 36-year-old female with hypercalcemia and acute kidney injury refractory to treatment. Whole body Flourine-18-fluorodeoxyglucose positron emission tomography computed tomography (18F-FDG PET/CT) showed increased metabolic uptake with ill-defined lesions in the liver, spleen, and pelvic lymph nodes. Biopsy of the ill-defined lesions in the liver showed necrotizing granulomatous lesions without angiitis. All the markers of tuberculosis were negative and angiotensin converting enzyme levels were elevated. Patient improved with 1 mg/kg/day oral steroid therapy and is on regular follow-up with minimal dose of steroids. Conclusion. Necrotizing sarcoid granulomatosis (NSG) is a rare systemic granulomatous disease. Due to its rarity and diagnostic difficulty, treatment is challenging for clinicians, pathologists and radiologists. Treatment of choice for symptomatic patients is steroid therapy. Prognosis is good with complete recovery. PubDate: Tue, 05 Nov 2019 09:05:05 +000
Abstract: Henoch-Schönlein nephritis or immunoglobulin A (IgA) vasculitis is characterized by purpura, arthralgia, abdominal pain, and glomerulonephritis with glomerular IgA deposition. Notably, the presence of purpura is essential to diagnose this disease. We report the case of a patient in whom proteinuria and haematuria were detected during screening tests and he was diagnosed with IgA nephropathy at 20 years of age. Corticosteroids were administered for 7 years and were subsequently tapered. At 35 years of age, he noticed purpura on his lower extremities and was diagnosed with anaphylactoid purpura. Following the appearance of purpura, urinalysis revealed an increase in urinary protein levels from 0.7 g/g creatinine (Cr) to 1.4 g/gCr, and his serum Cr levels increased from 1.1 mg/dL to 1.35 mg/dL. Two months later purpura subsided, and his urinary protein level and serum Cr level were restored to the former levels. Although the cause remains unknown, an interval may occasionally be observed between the appearance of purpura and urinary abnormalities. However, to our knowledge to date, a 15-year interval is the longest interval, in such cases, reported in the literature. PubDate: Tue, 29 Oct 2019 08:05:09 +000
Abstract: An 82-year-old man treated with phenytoin for the prevention of symptomatic epilepsy was hospitalized to treat consciousness disturbance, seizure, and hypocalcemia (serum calcium: 4.6 mg/dL). Serum 25-hydroxyvitamin D level was very low (5.4 ng/mL), whereas serum calcitriol level was normal (27 pg/mL) and serum intact parathyroid hormone level was increased (369 pg/mL). He was finally diagnosed with vitamin D deficiency associated with low sunlight exposure and long-term phenytoin use for symptomatic epilepsy: phenytoin is shown to accelerate catabolism of 25-hydroxyvitamin D. Combination treatment with eldecalcitol and maxacalcitol ointments successfully normalized corrected serum calcium level: both eldecalcitol and maxacalcitol are vitamin D receptor activators used for osteoporosis and psoriasis, respectively. Our case illustrates the importance of periodic serum calcium level monitoring in patients receiving anti-epileptic drugs and the usefulness of eldecalcitol and maxacalcitol ointment as a therapeutic option for hypocalcemia, especially in countries where native vitamin D and 25-hydroxyvitamin D are not available. PubDate: Tue, 15 Oct 2019 09:05:14 +000
Abstract: Idiopathic nephrotic syndrome (INS) represents 15%–30% of adulthood glomerulopathies. Corticosteroids have been the main treatment for decades and are effective in 70% of minimal-change disease patients and ~30% of focal segmental glomerulosclerosis patients. Multidrug-resistant (steroids, calcineurin-inhibitors, cyclophosphamide, mycophenolate-mofetil, rituximab) idiopathic nephrotic syndrome is a major therapeutic challenge in nephrology. Apheresis (double-filtration plasmapheresis or semi specific immunoadsorption) could act by eliminating the circulating factor (apolipoproteinA1b, solubleCD40L, suPAR) increasing glomerular permeability seen in INS. The aim of the study was to report the outcome of three patients with multidrug-resistant INS treated successfully with apheresis. PubDate: Wed, 09 Oct 2019 12:05:04 +000
Abstract: Atypical haemolytic uraemic syndrome (aHUS) is a form of thrombotic microangiopathy precipitated by unopposed complement activation, the treatment of which has been revolutionised by the availability of the monoclonal anti-complement (C5) antibody, eculizumab. Historically, women with aHUS would be unable to achieve a successful pregnancy due to the severity of their renal disease and for the few who could conceive, recurrence of aHUS was a significant risk. In spite of this, parenthood remains a priority for many. Experience with eculizumab use in the management of aHUS during pregnancy is growing and with it comes a significant change in the course of the disease. We present the case of a 28-year-old woman diagnosed with severe aHUS in the first trimester of her first pregnancy. She received rescue therapy with eculizumab and had a return to normal renal function. While this pregnancy was lost, she strongly desired a family. We managed her through a subsequent pregnancy while receiving eculizumab. This pregnancy was uncomplicated and carried to term and she birthed a healthy 2500 g baby girl. The complexities of managing a pregnancy in a woman with a history of aHUS are vast but not insurmountable, as demonstrated by this case. PubDate: Mon, 07 Oct 2019 00:05:37 +000
Abstract: Sedation during medical procedures poses a risk to any patient, and the use of specific anesthetic agents should be carefully considered to avoid adverse outcomes. We report on a patient with propofol infusion syndrome diagnosed during the post-operative period of a renal transplant. A 58-year-old female on chronic hemodialysis due to end stage kidney disease secondary to microscopic polyangiitis underwent kidney transplant from a deceased donor. Anesthetic induction was performed with fentanyl, propofol, and cisatracurium, and maintained with continuous propofol infusion. In the recovery room, the patient developed somnolence, tachypnea, and thoracoabdominal dissociation secondary to residual neuromuscular block. An arterial-blood gas test indicated acidemia, high pCO2, low HCO3, and mildly increased serum lactate. The patient remained hemodynamically stable, on volume-controlled ventilation, with sedation by continuous propofol infusion. Blood gas tests revealed persistent acidemia without tissue hypoperfusion. Doppler ultrasound of the renal graft reported adequate blood flow and serum triglycerides were elevated. A diagnosis of propofol infusion syndrome was made, and infusion ceased. A decrease in serum lactate levels was observed, with normalization 4 h later. This case highlights the importance of considering adverse effects of anesthetic agents as the cause of post-operative complications when prolonged sedation is required. PubDate: Wed, 25 Sep 2019 00:05:08 +000
Abstract: Lanthanum carbonate is a commonly prescribed oral phosphate binder for use in patients with acute or chronic kidney disease. The elemental form of lanthanum is a soft metal, which will appear radiopaque on a standard X-ray radiograph. This case report illustrates the potential for Lanthanum to masquerade as multiple radiopaque intestinal foreign bodies, leading to the extensive mobilization of medical resources and consultations including serial X-ray monitoring, multiple consultants including acute care and colorectal surgery. Given the few published reports describing this finding in the literature, it is essential to consider Lanthanum precipitates in the differential diagnosis of radiopaque intestinal foreign bodies in patients with chronic kidney disease to avoid unnecessary utilization of medical resources for this predominantly benign condition. PubDate: Mon, 02 Sep 2019 00:05:24 +000
Abstract: We report a case of type I cryoglobulinemic glomerulonephritis in a patient with chronic hepatitis C who presented with acute renal failure. The renal biopsy revealed membranoproliferative GN (MPGN) due to cryoglobulinemia with unexpected monoclonal Kappa restriction on immunofluorescence microscopy, suggesting an underlying hematopoietic malignancy. The bone marrow biopsy revealed presence of marginal zone lymphoma. Our case raises awareness regarding possibility of monoclonality in the renal biopsy of HCV-infected patients and exemplifies the crucial role the renal biopsy plays in detecting lymphoid malignancies where clinical features are ambiguous. PubDate: Sun, 18 Aug 2019 07:05:02 +000
Abstract: Acute esophageal necrosis (AEN) is an uncommon disease characterized by gastrointestinal bleeding and endoscopic findings of circumferential black-colored necrosis of the distal esophagus. Patients at risk include elderly males over the age of 65, who typically have multiple chronic medical issues including vascular disease and diabetes. Mortality is reported to be 32%. Here, we present a case of AEN in a renal transplant patient and describe potential inciting factors such as immunosuppression and opportunistic diseases. PubDate: Thu, 25 Jul 2019 12:05:07 +000
Abstract: Background. Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Mutations in CFI gene coding for complement regulation factors and in THBD gene coding for endothelial cell receptor thrombomodulin could predispose to the disease and hypertension can trigger the onset. Case Presentation. A 51-year-old female patient who had received kidney transplant eighteen years ago presented with hypertensive peak and hemolysis pattern. Normal ADAMTS13 levels as well as negative culture and serology for Shiga-toxin excluded, respectively, thrombotic thrombocytopenic purpura (TTP) and typical HUS caused by Shiga toxin-producing Escherichia coli (STEC-HUS). In suspicion of aHUS, we administered eculizumab and hemodialysis sessions were started as the patient showed severe renal failure. After an initial response, the patient developed cerebral hemorrhage. After last eculizumab administration, according to hematological parameters, an unsatisfactory response was observed: given the worsening clinical scenario, we withdrew eculizumab. Pathogenic mutations in CFI and THBD genes were found. After eculizumab reinitiation, looking at hemolysis indexes, we observed a suboptimal response as well as an otherwise adequate renal one: renal graft function was recovered despite persistence of hemolysis signs, after 6 months on regular dialysis. Conclusion. For the first time, we report an aHUS case in which a peculiar combination of mutations in CFI and THBD is found. We describe the importance of continuing eculizumab despite deterioration of patient’s clinical conditions. PubDate: Tue, 09 Jul 2019 12:05:03 +000
Abstract: The ketogenic diet (KD) is a high-fat, adequate-protein, and low-carbohydrate diet that leads to nutritional ketosis and weight loss. It is known to induce ketosis but is not an established cause of clinically significant ketoacidosis. Lactation ketoacidosis is well established in bovine literature but remains a rare phenomenon in humans. Here we present a life-threatening case of severe ketoacidosis in a nondiabetic lactating mother on a strict ketogenic diet. We review the available case reports of lactation ketoacidosis in humans and the mechanisms thereof. Although ketogenic diet has been shown to be safe in nonpregnant individuals, the safety of this diet in lactating mothers is not known. Health professionals and mothers should be made aware of the potential risk associated with a strict ketogenic diet when combined with lactation. Prompt diagnosis and immediate treatment cannot be overemphasized. To our knowledge, this is the first reported case of life-threatening lactation ketoacidosis associated with ketogenic diet while consuming an adequate number of calories per day. PubDate: Mon, 08 Jul 2019 10:05:06 +000
Abstract: Calciphylaxis, or calcification uremic arteriolopathy, is a rare disease thought to occur due to arteriolar calcifications of the dermis and is responsible for ischemia with cutaneous necrosis and painful panniculitis. Its mechanism remains poorly understood which makes its management challenging and difficult to standardize. We report our management of two patients diagnosed with calciphylaxis. In one patient, calciphylaxis was mentioned upon admission given the context of preexisting secondary hyperparathyroidism and the existence of multiple risk factors. In both patients, the diagnosis was confirmed histologically. Our two observations highlight the difficulty of the diagnosis and the complexity of the therapeutic management that has been personalized according to patient characteristics and clinical evolution. Several therapeutic means can be implemented once the diagnosis is made; nevertheless, its prognosis remains pejorative despite the therapeutic advances. Broad debridement, good phosphocalcic balance control, and the correction of the risk factors top the list of any therapeutic strategy. One of the major challenges of the therapy is normalizing the calcium-phosphate balance. Thus, Cinacalcet and sodium thiosulfate appear to be promising treatments. PubDate: Mon, 17 Jun 2019 14:05:01 +000
Abstract: ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide. PubDate: Thu, 13 Jun 2019 08:05:05 +000
Abstract: Membranoproliferative glomerulonephritis, one of the main causes of nephrotic syndrome, is associated with a state of hypercoagulability that leads to increased risk of thrombotic events. Portosystemic collaterals may reopen due to reversal of the flow within the existing veins and be a presenting feature of thrombosis. We describe a patient who presented with large portosystemic collaterals and signs of portal hypertension and was subsequently found to be affected by membranous proliferative glomerulonephritis. Proteinuria and microscopic haematuria in a patient with signs of portal hypertension and no pre-existing liver disease should raise the suspicion of an underlying kidney disease. PubDate: Tue, 28 May 2019 10:05:18 +000
Abstract: Allograft infiltration has been described in up to 20% of all patients with posttransplant lymphoproliferative disorder (PTLD), most representing EBV-positive B-cell lymphomas. Plasma cells are often observed in humoral rejection biopsies, but graft infiltration by plasmacytoma-like PTLD is rare. We report the case of a 54-year-old simultaneous pancreas-kidney transplant recipient (immunosuppression: OKT3, methylprednisolone, cyclosporine, and azathioprine), diagnosed with an IgG-kappa monoclonal gammopathy of undetermined significance eighteen years after transplant. Nine months later, pancreas allograft biopsy performed due to new-onset hyperglycemia (HgA1C 8.6%, C-peptide 6.15ng/mL and anti-GAD 0.9UI/mL) revealed a monotypic plasma cell infiltrate, CD19, CD79a, CD138 positive, with IgG-kappa light chain restriction, and EBV negative. PET-scan FDG uptake was limited to pancreas allograft. Tumor origin could not be established (using DNA microsatellite analysis). Despite treatment with bortezomib and dexamethasone, patient eventually died one month later. This is the first report of a late onset extramedullary plasmacytoma involving a pancreas allograft. PubDate: Sun, 19 May 2019 09:05:05 +000
Abstract: Acute lymphoblastic leukemia (ALL) is the second most common acute leukemia in adults. It is an aggressive hematologic neoplasm, with a bimodal age distribution typically presenting in childhood and the decade of life (Terwilliger and Abdul-Hay, 2017). Renal injury in ALL is common and can occur through many different mechanisms, such as prerenal acute kidney injury, acute tubular necrosis, renovascular disease, obstruction, glomerulonephritis, and parenchymal infiltration of tumor cells (Luciano and Brewster, 2014). Infiltration of kidneys by leukemia cells is common; however a resultant injury only occurs in about 1% of patients, and renal failure is even more rare (Luciano and Brewster, 2014). Renal failure due to bilateral infiltration of tumor cells has been reported in only a few cases and is thought to be a poor prognostic indicator (Luciano and Brewster, 2014; Sherief et al., 2015). Biopsy is essential to the diagnosis of renal infiltration of leukemia. We present a case of acute renal failure secondary to bilateral renal infiltration of ALL presenting as the first sign of relapse in a young man. PubDate: Mon, 13 May 2019 11:05:02 +000
Abstract: A 53-year-old woman is admitted with a serum creatinine of 16 mg/dl. Seven months earlier, she was diagnosed with heart failure and started on several medications, including Hydralazine. Laboratory studies revealed the presence of dual Anti-Neutrophil Cytoplasmic Antibodies (anti-MPO and anti-PR3), anti-nuclear and anti-histone antibodies. The clinical diagnosis was Drug-Induced ANCA Vasculitis (DIAV). Kidney histology, however, did not reveal crescents, but showed characteristic features of Alport’s syndrome. PubDate: Wed, 10 Apr 2019 08:05:04 +000
Abstract: Chronic kidney disease-mineral and bone disorder (CKD-MBD) is frequently present in advanced stages of chronic kidney disease (CKD) patients with high risk of fracture and elevated socioeconomic burden. Denosumab, an injectable human monoclonal antibody with affinity for nuclear factor-kappa ligand (RANKL), is an effective treatment for osteoporosis in postmenopausal women and men. Unlike the bisphosphonates, the pharmacokinetics and pharmacodynamics of denosumab are not influenced by the renal function and are being increasingly used for patients having CKD-MBD with low bone mineral density (BMD) to reduce the risk of fragility fractures. Hypocalcemia is a known side effect of this drug along with compensatory increase in parathyroid hormone (PTH). However, limited information is available in the literature regarding this potentially life-threatening side effect with denosumab in end-stage renal disease (ESRD) patients on dialysis. We present a patient with ESRD on peritoneal dialysis who developed severe symptomatic hypocalcemia and dramatic increase in PTH following denosumab therapy. She was conservatively managed with calcium supplementation and appropriate adjustment in calcium dialysate. We have also reviewed the literature on the use of denosumab in dialysis patients and looked at additional factors that may precipitate severe hypocalcemia in these patients. We believe that denosumab should be used with caution in dialysis patients since it may lead to profound hypocalcemia. Clinicians should ensure special attention in recognizing patients at risk of developing this serious adverse effect, so that prompt treatment and preventive strategies can be implemented. PubDate: Thu, 21 Mar 2019 12:05:01 +000
Abstract: Osmotic Demyelination Syndrome (ODS) occurs after rapid overcorrection of severe chronic hyponatremia usually in those with a predisposition such as chronic alcoholism, malnutrition, or liver disease. Rarely, do patients make a full recovery. We report a case of ODS secondary to overcorrection of severe hyponatremia with pathognomonic clinical and radiologic signs making a complete neurological recovery. A detailed course of events, review of literature, and optimal and aggressive management strategies are discussed. There is some controversy in the literature regarding the prognosis of these patients. Our aim here is to show that, with aggressive therapy and long-term care, recovery is possible in these patients. PubDate: Wed, 20 Mar 2019 00:05:10 +000
Abstract: Tumor necrosis factor receptor associated periodic syndrome (TRAPS) is a rare monogenic autoinflammatory disease. Its most severe manifestation is secondary amyloidosis. A 44-year-old male presented with nephrotic syndrome. Kidney biopsy was conclusive for secondary amyloidosis. The patient and his children had a history of recurrent febrile periods since infancy. All subjects were positive for a heterozygous variant of the TNFRSF1A gene, confirming TRAPS diagnosis. The patient progressed to end-stage renal failure and developed recurrent pericarditis episodes. He was started on anakinra while on hemodialysis with marked reduction of his serum amyloid A protein (SAA) levels. Meanwhile he received a cadaveric renal transplant and maintains anakinra treatment. Despite renal failure being the most feared complication of AA amyloidosis caused by TRAPS, little data is available about safety of anti-IL-1 treatment in patients with severe kidney failure. The authors report this case of a patient on dialysis treated with anakinra in which no complications were registered. Though amyloidosis is established, the authors believe containing its progression and reducing inflammatory activity can improve patient prognosis and reduce recurrence of amyloidosis in kidney transplant, as has been demonstrated in transplanted patients due to familial Mediterranean fever amyloidosis. PubDate: Sun, 17 Mar 2019 12:05:05 +000
Abstract: We describe a patient who had chronic lymphocytic leukaemia (CLL) Binet stage A at presentation with further evidence of disease at multiple sites but who initially required no treatment. However, several years later, her peripheral blood lymphocyte count started to increase, and soon after that she suffered an acute myocardial infarct (in the absence of coronary atheroma) together with proteinuric renal failure due to membranoproliferative glomerulonephritis. Her renal function improved markedly following anti-CLL chemotherapy. We postulate that her cardiac and renal disease were both complications of her CLL. In patients with CLL who develop new clinical signs or symptoms (even if apparently unrelated), consideration should be given as to whether these may be disease complications as this may serve as an indication to commence anti-CLL therapy; close liaison between different specialties is vital. PubDate: Tue, 12 Mar 2019 07:05:00 +000
Abstract: A 51-year-old woman with rheumatoid arthritis presented with mild hypertension 20 months after tacrolimus treatment and developing proteinuria 24 months after the treatment. Tacrolimus was discontinued 27 months after the treatment, followed by heavy proteinuria, accelerated hypertension, and deteriorating renal function without ocular fundus lesions as a clinical sign of malignant hypertension. Renal biopsy revealed malignant nephrosclerosis characterized by subacute and chronic thrombotic microangiopathy (TMA), involving small arteries, arterioles, and glomeruli. Focal segmental glomerulosclerosis, probably secondary to chronic TMA, was identified as a cause of heavy proteinuria. The zonal tubulointerstitial injury caused by subacute TMA may have mainly contributed to deteriorating renal function. The presence of nodular hyalinosis in arteriolar walls was indicative of tacrolimus-associated nephrotoxicity. Together with other antihypertensive drugs, administration of aliskiren stabilized renal function with reducing proteinuria. Owing to the preexisting proteinuria prior to severe hypertension and the complex renal histopathology, we postulated that chronic TMA, which was initially triggered by tacrolimus, was aggravated by severe hypertension, resulting in overt renal TMA. PubDate: Wed, 06 Mar 2019 08:05:04 +000
Abstract: A 41-year-old woman developed nephrotic syndrome at the age of 32 and was diagnosed with minimal change nephrotic syndrome based on a renal biopsy. Although remission was achieved with administration of prednisolone (PSL) and cyclosporine, the nephrotic syndrome recurred. She was also started on rituximab (RTX). She developed late-onset neutropenia after RTX treatment (R-LON) and improved 17 days later. Although the majority of R-LON cases undergo spontaneous remission, cases of death have been reported. This report is intended to warn about R-LON, since the use of RTX for adult-onset nephrotic syndrome is expected to increase in the future. PubDate: Tue, 05 Mar 2019 09:05:04 +000
Abstract: Lupus nephritis is a common manifestation of systemic lupus erythematosus (SLE). IgA nephropathy is a common type of primary glomerulonephritis. Renal manifestations in SLE patients are often due to lupus nephritis; however, renal diseases unrelated to lupus nephritis are rarely reported. While crescentic IgA nephropathy with rapid clinical progression is rare, its development in patients with SLE in the absence of lupus nephritis is even more unusual. A 74-year-old woman with a history of SLE without known renal involvement, chronic kidney disease stage IIIa, congestive heart failure, hypertension, and type 2 diabetes mellitus presented with acute kidney injury. Her creatinine continued to rise rapidly. Renal biopsy revealed mesangial proliferative glomerulonephritis with crescent formation. Immunofluorescent staining showed IgA and C3 mesangial deposition and absence of C4 and C1q, consistent with IgA nephropathy. She received a course of methylprednisolone and plasmapheresis. Unfortunately, her renal function continued to deteriorate, and she was started on hemodialysis which was continued after hospital discharge. This case illustrates crescentic IgA nephropathy without lupus nephritis as the cause of acute kidney injury in a patient with SLE. It highlights the observation that renal diseases other than lupus nephritis can develop in SLE patients. PubDate: Tue, 05 Mar 2019 09:05:03 +000
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