Retransplantation after post transplant lymphoproliferative disorder: overcoming the obstacles!
We describe here the case of a young boy who underwent retransplantation after treatment of early Epstein Barr virus (EBV) related post transplant lymphoproliferative disorder. Our case highlights the various factors needing deliberation before retransplantation including time from remission of PTLD, EBV serostatus and choice of induction and maintenance immunosuppression agents. (Source: CEN Case Reports)
Source: CEN Case Reports - February 3, 2020 Category: Urology & Nephrology Source Type: research

Thrombotic thrombocytopenic purpura associated to dual checkpoint inhibitor therapy for metastatic melanoma
We present a case of a 70-year old man with advanced melanoma, presenting with severe thrombocytopenia, hemolytic anemia with schistocytes and suppressed ADAMTS-13 activity by ADAMTS-13 inhibitors. We discuss differential diagnoses and speculated mechanisms of this obviously therapy-related adverse event, which should be considered by clinicians prescribing these drugs. (Source: CEN Case Reports)
Source: CEN Case Reports - January 30, 2020 Category: Urology & Nephrology Source Type: research

Different effects of rituximab on a native kidney and a post-transplant kidney with recurrence of focal segmental glomerulosclerosis
We present the case of a 29-year-old woman with focal segmental glomerulosclerosis (FSGS) who was treated with rituximab administration under different conditions for refractory nephrotic syndrome and posttransplant FSGS recurrence. At the age of 13  years, she developed FSGS, which followed a refractory clinical course, and eventually necessitated her to undergo plasmapheresis and receive rituximab at the age of 25 years. However, both therapies were ineffective, and she subsequently had progressive renal failure, for which dialysis was init iated at the age of 26 years. At the age of 28 years, she rec...
Source: CEN Case Reports - January 30, 2020 Category: Urology & Nephrology Source Type: research

Dipeptidyl peptidase-4 inhibitor-associated bullous pemphigoid, likely triggered by scabies, in a hemodialysis patient with human leukocyte antigen-DQB1*03:01
AbstractBullous pemphigoid (BP) is the most common autoimmune subepidermal bullous diseases. Autoantibodies against hemidesmosomal adhesion proteins might be involved in the developing process. BP usually affects the elderly with high mortality whereas the drug-induced BP is often improved and rarely relapses after the withdrawal of the suspected drug. An accumulated evidence suggests that dipeptidyl peptidase-4 inhibitor (DPP-4I), which has been widely used as the antidiabetic drug improves glycemic control with little risk for hypoglycemia, could be an inducer of DPP-4I-associated BP (DPP-4I-BP). While the precise mechan...
Source: CEN Case Reports - January 28, 2020 Category: Urology & Nephrology Source Type: research

Catastrophic antiphospholipid antibody syndrome as a first manifestation of primary antiphospholipid antibody syndrome in a middle-aged man
AbstractCatastrophic antiphospholipid antibody syndrome (CAPS) is a severe form of antiphospholipid antibody syndrome (APS) that sometimes represents the first manifestation of the later syndrome. The clinical manifestations of CAPS are relatively non-specific. Hence, the diagnosis may be delayed, resulting in high mortality. We herein present a case of a 40-year-old male who presented with rapid-onset renal failure, gangrene of finger and toe tips and hematological abnormalities with no underlying secondary cause for this complication. The symptoms were precipitated by febrile illness of short duration. A provisional diag...
Source: CEN Case Reports - January 27, 2020 Category: Urology & Nephrology Source Type: research

A case of cerebral infarction caused by painless acute aortic dissection in autosomal dominant polycystic kidney disease
We report the case of a 71-year-old Japanese woman who complained of hemiparesis. She required maintenance hemodialysis therapy with a background of ADPKD. Cerebral infarction was initially diagnosed by excluding intracranial hemorrhage and aneurysm rupture that are recognized as common complications of ADPKD and thereby anticoagulation therapy was initiated. However, the patient was suspected as having painless aortic dissection because a chest X-ray examination showed expanded upper mediastinum. Sequential vascular imagings revealed dissection of the aorta, originating from brachiocephalic trunk to the right common carot...
Source: CEN Case Reports - January 27, 2020 Category: Urology & Nephrology Source Type: research

Deceased donor kidney transplant complicated by spontaneous rupture of native kidney in a HIV patient
AbstractSpontaneous native kidney rupture (SNKR) is a rare occurrence, commonly associated with underlying renal tumors or acquired renal cystic disease in both the kidney transplant (KT) and non-KT populations. Herein, we present a 65-year-old African American man who experienced a non-malignant SNKR 6  days after a deceased donor KT and underwent emergent native nephrectomy. The patient’s hospital course was complicated by thrombocytopenia and refractory hypertension. He experienced delayed graft function and was maintained on hemodialysis until post-operative day 30. This case demonstrates an unusual presenta...
Source: CEN Case Reports - January 27, 2020 Category: Urology & Nephrology Source Type: research

Plasma exchange combined with bortezomib-based chemotherapy is effective for early renal recovery in a patient with IgD- λ type multiple myeloma
AbstractThe immunoglobulin (Ig) D type is a rare variant of multiple myeloma (MM), that accounts for 1 –2% of all cases. Compared to the more common types of MM, IgD MM is known to have more severe symptoms at presentation, and a poorer prognosis. A woman was admitted to our hospital for severe acute kidney disease and disorder (AKD) and back pain, and was started on hemodialysis. The renal biopsy revealed light chain cast nephropathy. She was diagnosed with IgD-λ MM based on Bence-Jones protein expression and high IgD serum levels, and started bortezomib therapy with plasma exchange (PE). After three sessions...
Source: CEN Case Reports - January 23, 2020 Category: Urology & Nephrology Source Type: research

Rituximab-induced serum sickness in a 6-year-old boy with steroid-dependent nephrotic syndrome
AbstractRituximab (RTX) is a murine –human chimeric monoclonal antibody against CD20 that has been proven effective for preventing relapse in frequently-relapsing or steroid-dependent nephrotic syndrome (NS). Serum sickness, a type-3 hypersensitivity reaction resulting from injection of foreign proteins, has been reported in patient s treated with RTX. Herein, we describe a case of RTX-induced serum sickness (RISS) in a 6-year-old boy with steroid-dependent NS. He presented to the hospital with fever and polyarthralgia at 10 days after his fourth dose of RTX. Although he was started on empiric intravenous antibi...
Source: CEN Case Reports - January 22, 2020 Category: Urology & Nephrology Source Type: research

Unique case of ANCA-negative pauci-immune necrotizing glomerulonephritis with diffuse alveolar hemorrhage, potentially associated with midostaurin
We present a 61-year-old male withFLT3-mutated acute myeloid leukemia treated with midostaurin who developed acute kidney injury requiring hemodialysis and pulmonary renal syndrome. Antibodies to proteinase-3, myeloperoxidase, and glomerular basement membrane were negative. Renal biopsy confirmed acute pauci-immune focal necrotizing glomerulonephritis (GN) with fibrin crescents indicating rapidly progressing glomerulonephritis. He improved with pulse methylprednisolone, intravenous cyclophosphamide, and plasma exchange with resolution of hemoptysis. This case highlights the importance of prompt renal biopsy to guide early ...
Source: CEN Case Reports - January 18, 2020 Category: Urology & Nephrology Source Type: research

Metabolic alkalosis due to short bowel syndrome in a hemodialysis patient
AbstractA 53-year-old man on hemodialysis suffered from short bowel syndrome after subtotal colectomy and partial resection of the small intestine. Laboratory tests showed multiple electrolyte disorders and enlarged sodium and chloride ion (Cl−) gaps despite treatment with large volume of sodium chloride replacement via central venous infusion. Blood gas analysis showed slightly high bicarbonate ion levels and metabolic alkalosis was suspected, which is uncommon in end stage kidney disease. The measurement of electrolytes in feces from ileostomy showed relatively high Cl− excretion. The loss of Cl− to fec...
Source: CEN Case Reports - January 18, 2020 Category: Urology & Nephrology Source Type: research

Melanotan II: a possible cause of renal infarction: review of the literature and case report
We present a review of Melanotan II and the possible effects of this drug on the kidneys by including a case of a renal infarction most likely attributed to Melanotan II. In the mechanism of renal injury with Melanotan II, thrombotic pharmacological influence and possible direct toxic effect on renal parenchyma must be considered. (Source: CEN Case Reports)
Source: CEN Case Reports - January 18, 2020 Category: Urology & Nephrology Source Type: research

Effect of pemafibrate, a novel selective peroxisome proliferator-activated receptor-alpha modulator (SPPARM α), on urinary protein excretion in IgA nephropathy with hypertriglyceridemia
AbstractLipid abnormalities, including hypertriglyceridemia, are one of the most common comorbidities in patients with chronic kidney disease (CKD) and are independently associated with disease progression. However, it remains uncertain whether treatment for hypertriglyceridemia has favorable effects on the clinical course of IgA nephropathy (IgAN). Pemafibrate is a novel selective peroxisome proliferator-activated receptor-alpha modulator and may be distinct from conventional fibrates in terms of its pharmacological activity and hepatic and renal safety. A recent clinical study demonstrated that pemafibrate was safe and e...
Source: CEN Case Reports - January 16, 2020 Category: Urology & Nephrology Source Type: research

Vaginal bleeding as primary presentation of renal cell carcinoma
In conclusion, the incidence of RCC metastasis to the vagina is extremely rare; but, in cases of vaginal bleeding or lesions we have to keep in mind the possibility of metastatic RCC. (Source: CEN Case Reports)
Source: CEN Case Reports - January 8, 2020 Category: Urology & Nephrology Source Type: research

Dietary potassium restriction attenuates urinary sodium wasting in the generalized form of pseudohypoaldosteronism type 1
In conclusion, dietary potassium restriction is one of the indispensable treatments for generalized PHA1. (Source: CEN Case Reports)
Source: CEN Case Reports - January 3, 2020 Category: Urology & Nephrology Source Type: research

A case of lupus vasculopathy presenting favorable renal outcome
AbstractNoninflammatory necrotizing vasculopathy, also referred to as lupus vasculopathy, is not infrequently observed in the pathology of lupus nephritis. It affects vessels causing them to become severely narrowed and occluded by a mechanism involving immune complexes. We experienced a 51-year-old woman with lupus nephritis class IV  + V, which was accompanied by lupus vasculopathy. Renal biopsy and light microscopy showed eosinophilic hyaline-like material in the afferent and/or efferent arterioles, which narrowed the lumen, and which were positive for IgG by immunofluorescent analysis. Electron microscopy...
Source: CEN Case Reports - December 20, 2019 Category: Urology & Nephrology Source Type: research

Systemic lupus erythematosus complicated by a Gitelman-like syndrome in an 8-year-old girl
AbstractAn 8-year-old girl with recently diagnosed Systemic Lupus Erythematosus (SLE) (class 4 lupus nephritis with autoimmune hemolytic anemia) presented to the pediatric nephrology clinic with polyuria, tiredness and cramps; laboratory investigations revealed refractory hypokalemia, hypomagnesemia, metabolic alkalosis, hypocalciuria and hyperchloriuria. There was no history of diuretic administration. These features were consistent with the Gitelman syndrome. She required large doses of potassium and magnesium supplementation along with spironolactone, for normalization of the serum potassium and magnesium levels. Immuno...
Source: CEN Case Reports - December 18, 2019 Category: Urology & Nephrology Source Type: research

Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility
AbstractCalciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis. Herein, we report five patients of hemodialysis patients with skin biopsy-proven calciphylaxis at a single facility. One patient had undergone parathyroidectomy (PTx) due to severe secondary hyperparathyroidism, four had been treated with vitamin D receptor activators, and two were on warfarin therapy. All patients had hyperphosphatemia, and one had hypercalcemia. The intact parathyroid hormone level at diagnosis was 2  pg/ml in the patient after PTx, while three patients were within the target range. The avera...
Source: CEN Case Reports - December 17, 2019 Category: Urology & Nephrology Source Type: research

Unusual manifestation of monoclonal gammopathy of undetermined significance: a false serum creatinine elevation
AbstractA 72-year-old Japanese man with diabetes mellitus and hypertension presented with an acutely elevated serum creatinine level, from 1.02 to 4.13  mg/dL over 2 months as measured by the enzymatic method by pure-auto S CRE-N®. Renal biopsy could not identify the etiology of the elevating sCr. However, an elevated total protein level (8.2  g/dL) and lowering of the BUN and sCr ratio from 14.5 to 2.7 were found, and bone marrow biopsy showed less than 10% lymphoplasmacytic infiltration, compatible with monoclonal gammopathy of undetermined significance. The diagnosis of a false serum creatinine elevat...
Source: CEN Case Reports - December 14, 2019 Category: Urology & Nephrology Source Type: research

Carboplatin-related acute interstitial nephritis in a patient with pancreatic neuroendocrine tumor
AbstractCarboplatin is characterized by low nephrotoxicity, including acute tubular necrosis (ATN), compared to a conventional platinum complex due to its low accumulative property in the renal tubules. Therefore, there are extremely few reports of carboplatin-induced kidney injury and only one case has been histologically examined. Herein, we describe the case of a 53-year-old man who presented with acute kidney injury (AKI) that occurred after carboplatin administration and was diagnosed with biopsy-proven acute interstitial nephritis (AIN). To our knowledge, this is the second case report of carboplatin-related AIN. The...
Source: CEN Case Reports - December 13, 2019 Category: Urology & Nephrology Source Type: research

Kidney transplant patient with immunoglobulin A nephropathy subsequently diagnosed as concurrent autosomal dominant polycystic kidney disease during 17-year follow-up
AbstractA 14-year-old Japanese boy was diagnosed with immunoglobulin A nephropathy resulting in end-stage kidney disease (ESKD). He underwent ABO-compatible living kidney transplantation from his father at the age of 27. In the process of selecting a donor before the transplantation, it turned out that his mother had polycystic kidneys and that her family had a history of hypertension and cerebrovascular diseases. The patient himself also had bilateral multiple kidney cysts, with a normal-sized kidney, confusing us to make the diagnosis of acquired cystic kidney disease (ACKD) or ADPKD difficult at that point. Seventeen ye...
Source: CEN Case Reports - December 2, 2019 Category: Urology & Nephrology Source Type: research

Difficulty of predicting early-onset super-imposed preeclampsia in pregnant women with hemodialysis due to diabetic nephropathy by serum levels of sFlt-1, PlGF, and sEng
AbstractThere are few case reports in which circulating levels of soluble fms-like tyrosine kinase 1 (sFlt-1), placental growth factor (PlGF), and soluble endoglin (sEng) were measured before the onset of super-imposed preeclampsia in women with hemodialysis. A 40-year-old Japanese nulliparous women with hemodialysis due to diabetic nephropathy became pregnant by frozen embryo transfer. Intensive hemodialysis was started at 5  weeks of gestation. Her blood pressure (BP) in the first trimester was around 130/80 mmHg. At 20+3 weeks, she was admitted for close monitoring; her BP was 137/75  mmHg. Her BP increas...
Source: CEN Case Reports - November 14, 2019 Category: Urology & Nephrology Source Type: research

Complete oculocerebrorenal phenotype of Lowe syndrome in a female patient with half reduction of inositol polyphosphate 5-phosphatase
We present a female case with congenital cataracts, severe intellectual impairment, sensorineural hearing loss, and renal tubular dysfunction as Lowe syndrome. A 9-year-old Japanese girl visited our hospital due to prolonged proteinuria. Her renal biopsy revealed diffuse mesangium proliferation, sclerosis and dilatation of renal tubules, and mild IgA deposition in the mesangial region. Furthermore, she had congenital cataracts, severe intellectual impairment, and sensorineural hearing loss. Genetic screening did not identify mutations of theORCL gene encoding inositol polyphosphate 5-phosphatase (IPP-5P) (46 XX, female). H...
Source: CEN Case Reports - November 9, 2019 Category: Urology & Nephrology Source Type: research

Combination of furosemide and fludrocortisone as a loading test for diagnosis of distal renal tubular acidosis in a pediatric case
We report the first pediatric case of distal RTA in a patient who was successfully diagnosed by a drug loading test with the combination of furosemide and fludrocortisone without any side effects. We also performed genetic analysis and detected a known pathogenic variant in theSLC4A1 gene. The combination loading test of furosemide and fludrocortisone is a useful and safe diagnostic tool for pediatric cases of RTA. (Source: CEN Case Reports)
Source: CEN Case Reports - November 8, 2019 Category: Urology & Nephrology Source Type: research

Membranous nephropathy associated with thrombospondin type-1 domain-containing 7A (THSD7A) in an adult woman with eosinophilia
AbstractA 30-year-old woman on steroid therapy for eosinophilia presented with nephrotic syndrome during steroid tapering. She was diagnosed with membranous nephropathy (MN) stage II –III (positive for IgG1 and IgG4) by renal biopsy. There was no evidence of secondary MN. Her urinary protein level was controlled to 0.5 g/day or less, and her eosinophil count in white blood cell differential was stabilized at less than 10% without increasing the steroid dosage. The renal speci men did not show any enhanced granular expression of PLA2R along the glomerular basement membrane, and PLA2R was not detected in the patie...
Source: CEN Case Reports - November 8, 2019 Category: Urology & Nephrology Source Type: research

Brachiocephalic vein compression caused by a mediastinal cystic tumor presenting with rapidly progressive upper limb swelling and pain in a patient on hemodialysis with a newly created arteriovenous graft
AbstractA 61-year-old man was hospitalized for creating vascular access for maintenance hemodialysis. Chronic interstitial nephritis was the cause of his end-stage kidney disease. An arteriovenous graft (AVG) was selected because superficial veins in his bilateral upper limbs were not suitable for arteriovenous fistula (AVF). Venography did not show any stenotic lesions in the drainage veins bilaterally. Soon after creation of the AVG, his left arm began to swell. Obstruction of the drainage vessels downstream of the AVG was highly suspected. Magnetic resonance imaging disclosed that the left brachiocephalic vein was compr...
Source: CEN Case Reports - November 7, 2019 Category: Urology & Nephrology Source Type: research

Autosomal dominant Alport syndrome due to a COL4A4 mutation with an additional ESPN variant detected by whole-exome analysis
We report a woman in her 20  s who exhibited persistent haematuria with normal renal function and sensorineural hearing loss. Her family members exhibited the same clinical findings among three generations and were suspected of having autosomal dominant AS (ADAS). Renal biopsy showed minor glomerular abnormalities on light mi croscopy and extensive thinning of the glomerular basement membrane on electron microscopy. Whole-exome analysis revealed a knownCOL4A4 (type IV collagen α4) mutation (c. 2510 G >  C: p. Gly837Ala). Two pedigrees with the same variant have been reported previously, one as A...
Source: CEN Case Reports - November 1, 2019 Category: Urology & Nephrology Source Type: research

List of referees
(Source: CEN Case Reports)
Source: CEN Case Reports - October 26, 2019 Category: Urology & Nephrology Source Type: research

Efficacy of Secukinumab for Plaque Psoriasis in a Patient on Hemodialysis
We report a 60-year-old Japanese woman. Plaque psoriasis was diagnosed at the age of 25  years and hemodialysis was started at the age of 39 years. Her skin lesions persisted despite use of topical agents such as maxacalcitol and betamethasone. Accordingly, administration of secukinumab was started at a dose of 150 mg. The psoriasis area and severity index (PASI) score decreased fro m 49.8 to 14.8 after 2 weeks and to 0 after 6 weeks, with remission being maintained after 28 months. No adverse reactions were seen. This case indicates that secukinumab may be effective for severe psoriasis in pa...
Source: CEN Case Reports - October 25, 2019 Category: Urology & Nephrology Source Type: research

A case of ischemic osteopathy in a hemodialysis patient with advanced peripheral artery disease
AbstractPeripheral artery disease (PAD) or arteriosclerosis obliterans is a lethal complication highly prevalent in pre-dialysis CKD and dialyzed patients. PAD is driven by atherosclerotic process and causes ischemia of the affected limb. Given that normal bone metabolism is based on sufficient blood supply and PAD decreases intraosseous blood flow of the affected limb, it is probable that PAD can directly cause ischemic osteopathy or osteoporosis in affected limbs. We herein present a 69-year-old female patient receiving 18  years of maintenance hemodialysis therapy was hospitalized for the treatment of bilateral PAD...
Source: CEN Case Reports - October 21, 2019 Category: Urology & Nephrology Source Type: research

The TIE fighter sign on point of care ultrasonography
(Source: CEN Case Reports)
Source: CEN Case Reports - October 21, 2019 Category: Urology & Nephrology Source Type: research

A case of anti-neutrophil cytoplasmic antibody-associated vasculitis with anti-glomerular basement membrane antibodies that was successfully treated with mizoribine as a safe and effective remission maintenance therapy with prednisolone and plasma exchange
AbstractWe herein report the case of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with anti-glomerular basement membrane (anti-GBM) antibody positivity that successfully treated with mizoribine (MZR) as an immunosuppressive drug for remission maintenance therapy after the initiation of dialysis in addition to plasma exchange (PE) and glucocorticoid treatment to control the disease condition. A 79-year-old woman developed serious renal dysfunction and pulmonary alveolar hemorrhaging due to MPO –ANCA and anti-GBM antibody double-positive vasculitis. She was started on hemodial...
Source: CEN Case Reports - October 14, 2019 Category: Urology & Nephrology Source Type: research

Acute tubulointerstitial nephritis and IgM deposits on glomerular capillary walls after immunotherapy with nivolumab for metastatic renal cell carcinoma
AbstractNivolumab is an anti-programmed cell death-1 antibody that is utilized as an immune checkpoint inhibitor for several malignancies. However, this agent is associated with immune-related adverse events (irAEs), mainly in the spectrum of autoimmune disease including interstitial pneumonia, colitis, type 1 diabetes, and renal impairment. We herein present the case of a 59-year-old man with renal cell carcinoma who developed worsening renal function approximately 4  months after initiation of nivolumab. Urinalysis showed proteinuria and microscopic hematuria along with increase levels ofN-acetyl- β-d-glucosami...
Source: CEN Case Reports - October 11, 2019 Category: Urology & Nephrology Source Type: research

Axillary arterial angiosarcoma in a nonfunctioning arteriovenous fistula limb of a patient undergoing hemodialysis: case report with literature review
AbstractA 54-year-old man was admitted to our hospital with a painful left axillary mass. He had a 27-year history of hemodialysis for end-stage kidney disease because of chronic glomerulonephritis. He had a right radial artery –cephalic vein arteriovenous fistula and left nonfunctioning arteriovenous fistula. Computed tomography imaging showed a left axillary arterial mass with peripheral hematoma and multiple lung tumors. On hospital day 3, he showed disturbances in consciousness as well as enlargement of the axillary mass and hematoma. We performed emergency surgery to resect the left axillary tumor. The patient w...
Source: CEN Case Reports - October 10, 2019 Category: Urology & Nephrology Source Type: research

Hypocomplementemic urticarial vasculitis syndrome with gastrointestinal vasculitis and crescentic membranoproliferative glomerulonephritis without immune complex deposits
AbstractHypocomplementemic urticarial vasculitis syndrome (HUVS) is a small vessel vasculitis characterized by hypocomplementemia and urticaria-like exanthema. Some cases also display abdominal pain and membranoproliferative glomerulonephritis (MPGN) with immune complex deposits. We treated a case of HUVS with biopsy-proven gastrointestinal vasculitis and atypical histological findings in a kidney biopsy. The 36-year-old Japanese man, who was previously diagnosed with diffuse panbronchiolitis, visited our hospital due to transient urticaria-like exanthema and rapid deterioration of kidney function. On admission, the skin l...
Source: CEN Case Reports - October 4, 2019 Category: Urology & Nephrology Source Type: research

Early dialysis in a rare case of combined toxic alcohols ingestion
We describe the case of a 40-year-old male who was brought to our emergency department for reported paint thinner ingestion. He was unable to protect his airway and required intubation. Blood gas showed respiratory acidosis, an initial AG, corrected by albumin of 12.75, lactic acid 5.26  mmol/L, and an OG of 170. Patient was treated with bicarbonate drip, fomepizole and emergent HD, which improved his neurologic status. Days after his admission, alcohol levels came positive for a co-ingestion of ethylene glycol, diethylene glycol, and methanol. Most of the TA are metabolized into their toxic byproducts by the enz...
Source: CEN Case Reports - October 1, 2019 Category: Urology & Nephrology Source Type: research

A case of denosumab-associated membranous nephropathy in a patient with rheumatoid arthritis
AbstractWe herein report a case of anti-RANKL monoclonal antibody-associated membranous nephropathy (MN). A 67-year-old woman with a history of rheumatoid arthritis treated with prednisolone and methotrexate for more than 30  years and osteoporosis treated with eldecalcitol and teriparatide for 4 years had achieved a stable disease condition. Her kidney function was normal and her urinalysis was negative for hematuria and proteinuria. An anti-RANKL monoclonal antibody (denosumab) was administered for the treatment of osteoporosis. Four months later, proteinuria appeared (2.3 g/g creatinine) and remained posi...
Source: CEN Case Reports - September 23, 2019 Category: Urology & Nephrology Source Type: research

A case of female Fabry disease revealed by renal biopsy
AbstractFabry disease (FD) is an X-linked inherited glycosphingolipid metabolism disorder, therefore, heterozygous female FD patients display highly variable clinical symptoms, disease severity, and pathological findings. This makes it very challenging to diagnosing female patients with FD. A 69-year-old Japanese female was introduced to the nephrologist for the evaluation of proteinuria. A renal biopsy was performed. Although the light microscopic examinations revealed that most of the glomeruli showed minor glomerular abnormalities, however, vacuolation was apparently found in the tubular epithelial cells. Immunofluoresc...
Source: CEN Case Reports - September 21, 2019 Category: Urology & Nephrology Source Type: research

A novel truncating PAX2 mutation in a boy with renal coloboma syndrome with focal segmental glomerulosclerosis causing rapid progression to end-stage kidney disease
We report a case of RCS with a novelPAX2 mutation that was pathologically diagnosed as FSGS and rapidly progressed to end-stage kidney failure (ESKD) with a review of past literature. A 6-year-old boy, who had bilateral coloboma and loss of vision in the left eye, was noted non-nephrotic proteinuria and renal dysfunction via school urine screening. Abdominal ultrasound showed no renal and urinary tract malformations and kidney biopsy showed FSGS. Genetic analysis revealed a novel insertion-deletion mutation inPAX2 (NM003987.4: c.70_72delinsA; p.Gly24Argfs*29). His kidney function deteriorated gradually during the following...
Source: CEN Case Reports - September 19, 2019 Category: Urology & Nephrology Source Type: research

Rapid formation of large pseudostones and acute cholangitis caused by ceftriaxone treatment for bacterial pneumonia in a patient on kidney transplantation: potential risk of ceftriaxone use in patients with decreased kidney function
(Source: CEN Case Reports)
Source: CEN Case Reports - September 17, 2019 Category: Urology & Nephrology Source Type: research

Regression of renal amyloid deposits by VAD therapy plus autologous stem cell transplantation in a patient with primary AL amyloidosis
We report a 58-year-old Japanese woman who presented with nephrotic syndrome. Steroid therapy and cyclosporine A administration were initiated, but hematological remission and renal response were not achieved. Renal biopsy revealed amyloid deposits in the mesangial region and the small arteries. Proteomic analysis based on laser microdissection and mass spectrometry showed that the amyloid deposits were composed of the constant region of the lambda light chain. She received vincristine, adriamycin, and dexamethasone therapy followed by high-dose melphalan and autologous stem cell transplantation, resulting in hematological...
Source: CEN Case Reports - September 14, 2019 Category: Urology & Nephrology Source Type: research

Efficacy of plasma exchange with a high dose of acyclovir for disseminated varicella infection
AbstractIn individuals treated with immunosuppressive therapies, the varicella-zoster virus (VZV) infection can become disseminated and lead to a life-threatening condition. There is currently no established treatment strategy for this life-threatening condition. Here, we describe a case where plasma exchange (PE) with a high dose of acyclovir (ACV) ameliorated the severe effects, including VZV-hemophagocytic lymphohistiocytosis (VZV-HLH) and disseminated intravascular coagulation (DIC), in a 9-year-old girl with steroid-dependent nephrotic syndrome. This 9-year-old girl experienced frequent relapse steroid-dependent nephr...
Source: CEN Case Reports - September 13, 2019 Category: Urology & Nephrology Source Type: research

Misdiagnosis of high anion gap acidosis owing to instrument error of a device
AbstractThe anion gap (AG) is a tool to diagnose metabolic acid –base disorders in the physiological approach to acid–base assessment. It is used to detect high AG acidosis, a type of metabolic acidosis caused by serum concentration increase in usually unmeasured anions; AG larger than the reference for it indicates the presence of high AG acidosis. This rep ort presents a case of hyperlactatemia which was not detected as high AG acidosis possibly because of instrument error of a device in measurement of serum sodium and chloride concentrations. The case indicates that the error will make AG unable to detect hi...
Source: CEN Case Reports - August 29, 2019 Category: Urology & Nephrology Source Type: research

A case of membranous nephropathy diagnosed with lupus nephritis 11 years after onset
AbstractA 34-year-old female patient presented to our hospital with lower extremity edema and proteinuria during pregnancy. Renal biopsy was performed and the patient was diagnosed with nephrotic syndrome due to lupus-like membranous nephropathy. This diagnosis was reached upon as laboratory findings upon admission, wherein both anti-nuclear and anti-double-stranded DNA antibodies revealed negative, did not fulfill the criteria for systemic lupus erythematosus (SLE) proposed by the American College of Rheumatology (ACR) and the patient did not reveal any typical physical manifestations of SLE. Methylprednisolone pulse ther...
Source: CEN Case Reports - August 9, 2019 Category: Urology & Nephrology Source Type: research

Dynamics of d -serine reflected the recovery course of a patient with rapidly progressive glomerulonephritis
AbstractWe experienced a case of a 36-year-old female with rapidly progressive glomerulonephritis (RPGN) due to anti-neutrophil cytoplasmic antibody (ANCA)-associated nephritis and systemic lupus erythematosus (SLE) nephritis. Chiral amino acid metabolomics revealed a prominent profile ofd-serine in this patient. At the fulminant period of RPGN, the level of plasmad-serine, a potential biomarker in CKD that reflects actual glomerular filtration ratio (GFR), was extremely high. On the other hand, urinary fractional excretion (FE) ofd-serine, which was usually much higher than that ofl-isoform, was 0% in this patient. These ...
Source: CEN Case Reports - July 29, 2019 Category: Urology & Nephrology Source Type: research

Dabigatran-induced anticoagulant-related nephropathy with undiagnosed IgA nephropathy in a patient with normal baseline renal function
This report demonstrates that ARN could occur in patients with normal baseline renal function. Our case and prior reports suggest that IgA nephropathy could be a risk factor for dabigatran-induced ARN. (Source: CEN Case Reports)
Source: CEN Case Reports - July 25, 2019 Category: Urology & Nephrology Source Type: research

Acute kidney injury in an adult patient with IgA nephropathy and chronic replicative Epstein –Barr virus infection
AbstractMost of the adult population are infected with Epstein –Barr virus (EBV), but as EBV replication is usually under immune system control, the majority of individuals remain asymptomatic. On the other hand, some individuals continuously retain a high EBV antibody titer and a high EBV DNA load in their blood, suggesting a defect of EBV replication contro l. To date, only a limited number of reports have addressed the relationship between this chronic form of EBV infection and renal involvement. Here, we describe an 80-year-old woman who developed acute kidney injury shortly after an episode of mosquito bites, ac...
Source: CEN Case Reports - June 19, 2019 Category: Urology & Nephrology Source Type: research

Arteriovenous fistula-related renal bleeding 5  days after percutaneous renal biopsy
AbstractA 32-year-old Japanese woman was admitted to our hospital for evaluation of microscopic hematuria with a positive family history. Percutaneous renal biopsy was performed under real-time ultrasound guidance using a 16-gauge automated needle and three specimens were obtained. She had no risk factors for hemorrhage. However, macroscopic hematuria developed from 5  days after biopsy and persisted for 4 days. Her Hb decreased markedly from 15.0 to 8.1 g/dL. Enhanced computed tomography revealed urinary tract hematoma, while the early arterial phase showed inflow of contrast medium into the left renal vein...
Source: CEN Case Reports - June 18, 2019 Category: Urology & Nephrology Source Type: research

Readers response to “Sequential nonarteritic anterior ischemic optic neuropathy in patient on chronic hemodialysis”
(Source: CEN Case Reports)
Source: CEN Case Reports - June 17, 2019 Category: Urology & Nephrology Source Type: research

Uremic encephalopathy in patients undergoing assisted peritoneal dialysis: a case series and literature review
AbstractWe sometimes hesitate to switch renal replacement therapy from peritoneal dialysis (PD) particularly in elderly patients due to their physical tolerance levels and lifestyles. Here, we describe the cases of three patients treated with PD alone despite an anuric status who subsequently developed uremic encephalopathy, which was successfully treated with hemodialysis (HD). The first patient was a 75-year-old woman who developed uremic encephalopathy with an anuric status and inadequate PD after 7  months of treatment. HD immediately improved her condition; encephalopathy did not recur with combined therapy of PD...
Source: CEN Case Reports - June 8, 2019 Category: Urology & Nephrology Source Type: research