Polyvisceral polycystic disease: a case study and review
AbstractPolycystic kidney disease (PKD) occurs in one per 20,000 births. Presence of cysts in other organs like adrenal, liver and bladder is even rarer. On reviewing the literature, there is evidence of PKD occurring in conjunction with polycystic liver disease but cysts in multiple viscera are, so far, not reported. A fetal autopsy of a 36-week fetus showed the presence of multiple cysts in the kidney, liver, adrenal and bladder. Further histopathology reports confirmed the diagnosis of polycystic kidney disease. The history of a previous intrauterine death, of another child at 28-week gestation, suggests the presence of...
Source: CEN Case Reports - March 4, 2021 Category: Urology & Nephrology Source Type: research

Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman
AbstractA 74-year-old Japanese woman was referred to our department because of anasarca and massive proteinuria. She was clinically diagnosed with nephrotic syndrome, and renal biopsy showed membranoproliferative glomerulonephritis accompanied by marked glomerular infiltration with macrophages and full-house immunofluorescence glomerular deposition. Furthermore, randomly arranged nonbranching fibrils, approximately 12  nm in diameter, were found by electron microscopy, and immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9), a recently identified diagnostic biomarker of fibrillary glomerulonephritis (FGN), s...
Source: CEN Case Reports - March 3, 2021 Category: Urology & Nephrology Source Type: research

Selective immunoglobulin M deficiency complicated by systemic lupus erythematosus and antiphospholipid syndrome: a case report and review of literature
In conclusion, SIgMD can be complicated by autoimmune disorders. Although rare, we recommend that SLE and APS be considered in patients with SIgMD w ho present with hematuria, proteinuria, and deep vein thrombosis. We also recommend measuring the titers of antinuclear antibodies, double-stranded DNA antibodies, and anti-CLβ2GP1 antibodies. (Source: CEN Case Reports)
Source: CEN Case Reports - February 22, 2021 Category: Urology & Nephrology Source Type: research

Familial juvenile hyperuricemia in early childhood in a boy with a novel gene mutation
AbstractFamilial juvenile hyperuricemic nephropathy (FJHN) is a rare autosomal dominant disease caused by mutations in the uromodulin (UMOD) gene. It is characterized by the development of gout, tubulointerstitial nephropathy, and end-stage renal disease. Here we report a case of FJHN that was diagnosed in early childhood in a boy with a novel gene mutation. At the age of 4  years, the patient was admitted with a diagnosis of purpura nephritis. He was discharged following symptom alleviation. However, hyperuricemia (7–9 mg/dL) and mild renal dysfunction [creatinine-estimated glomerular filtration rate (eGFR...
Source: CEN Case Reports - February 22, 2021 Category: Urology & Nephrology Source Type: research

Nephrotic syndrome relapse in a boy with COVID-19
We describe the clinical course of a child with NS and COVID-19. A 3-year-old boy was admitted with clinical and laboratory findings indicative of NS. Induction therapy with prednisolone (2  mg/kg/day) induced complete remission. While tapering the dose, he was infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). He developed a high fever and periorbital edema. Urinalysis revealed proteinuria (protein-creatinine ratio: 6.3 g/gCr). He was transferred to our hosp ital for the concurrent management of COVID-19 and NS relapse. As proteinuria worsened, the prednisolone dose was increased to 2&nb...
Source: CEN Case Reports - February 22, 2021 Category: Urology & Nephrology Source Type: research

The role of short-term, low dose intravenous ketamine infusion in Calciphylaxis
AbstractCalciphylaxis is a small vessel vasculopathy causing subcutaneous ischemic necrosis. This condition is a recognized complication of end stage renal disease and is associated with severe pain. The mechanism of the pain generated by calciphylaxis is thought to be partly related to tissue ischemia, with a significant neuropathic component associated with neuronal hypoxic injury. The pain can be further exacerbated by the inflammatory process ensuing as a result of calciphylactic lesion infections which are commonly associated with this condition. Obtaining adequate pain relief is a challenging aspect of symptom contro...
Source: CEN Case Reports - February 19, 2021 Category: Urology & Nephrology Source Type: research

Antiphospholipid antibody syndrome-associated renal thrombotic microangiopathy improved not with rivaroxaban but with warfarin in a systemic lupus erythematosus patient without lupus nephritis
AbstractA 34-year-old Japanese woman with systemic lupus erythematosus (SLE) was admitted to our hospital for exacerbation of renal dysfunction, hemolytic anemia and thrombocytopenia. Twenty-two years before admission, she was diagnosed with SLE. Eight years before, lupus anticoagulant (LAC) positivity was detected without any thrombotic findings. Fourteen months before, renal function started to worsen. Three months before, unprovoked left leg swelling appeared. She was diagnosed with deep vein thrombosis (DVT) by ultrasonography. Blood examination revealed mild anemia, thrombocytopenia, and renal dysfunction. Rivaroxaban...
Source: CEN Case Reports - February 17, 2021 Category: Urology & Nephrology Source Type: research

An elderly-onset neuroblastoma concomitant with minimal change nephrotic syndrome: the first autopsy case report
ConclusionsMinimal change nephrotic syndrome is the most common renal manifestation associated with lymphoproliferative malignancies. We here present an extremely rare case of adult-onset neuroblastoma with minimal change nephrotic syndrome. (Source: CEN Case Reports)
Source: CEN Case Reports - February 17, 2021 Category: Urology & Nephrology Source Type: research

A case of Stappia indica -induced relapsing peritonitis confirmed by 16S ribosomal RNA gene sequencing analysis in a patient undergoing continuous ambulatory peritoneal dialysis
AbstractA 69-year-old woman with 26-year history of systemic lupus erythematosus and 4-year history of peritoneal dialysis was hospitalized for treatment of bacterial peritonitis. On admission, peritoneal dialysate was collected and subjected to bacterial culture. Cell count in the cloudy peritoneal dialysate was 4194/ μL, and Gram-negative bacilli were detected. Vancomycin (1 g/day) and ceftazidime (1 g/day) were administered intraperitoneally, which resulted in rapid decrease in cell count in the peritoneal dialysate. However, on the 7th hospital day, peritonitis relapsed with abdominal pain and cloudy dialy...
Source: CEN Case Reports - February 15, 2021 Category: Urology & Nephrology Source Type: research

Predominant tubulointerstitial lupus nephritis with preceding pernicious anemia
AbstractPredominant tubulointerstitial nephritis with negligible glomerular lesions is a rare form of lupus nephritis. Although tubulointerstitial changes occur in two-thirds of patients with lupus nephritis, these lesions were mostly accompanied by glomerulonephritis. Predominant tubulointerstitial lupus nephritis has been reported to be only 13 cases in the literature as far as we surveyed. Here, we present a case of a 72-year-old male who had pancytopenia associated with pernicious anemia and later developed a mild proteinuria and renal insufficiency. Although urinary tubulointerstitial markers increased, serological sc...
Source: CEN Case Reports - February 6, 2021 Category: Urology & Nephrology Source Type: research

A case of recurrent anemia due to chronic parvovirus B19 infection in a kidney transplant recipient. Can everolimus make a difference?
We report a case of a kidney transplant recipient with relapsing pure red cell aplasia due to PB19 infection. Our patient was initially managed with standard treatment consisting of intravenous immunoglobulins and minimization of immunosuppressive treatment. However, when this approach became ineffective, conversion from tacrolimus to everolimus was done, with favorable results. This paper explores infection by PB19 in kidney transplant recipients and the potential benefits of a calcineurin inhibitor-free immunosuppression and the antiviral properties of mTOR inhibitors. (Source: CEN Case Reports)
Source: CEN Case Reports - February 4, 2021 Category: Urology & Nephrology Source Type: research

Renal intravascular large B-cell lymphoma predominantly localized in peritubular capillaries
We report on a 70-year-old woman with intravascular large B-cell lymphoma, in whom the renal lesion was localized in the peritubular capillaries. The patient complained of fatigue, general malaise, and unsteadiness when walking. Laboratory tests showed anemia, increased C-reactive protein, and mild renal failure, with a serum creatinine level of 1.31  mg/dL and no remarkable proteinuria or hematuria. Renal biopsy showed intravascular large B-cell lymphoma. The large atypical cells were mainly accumulated within the peritubular capillaries and no large atypical cells were found in the glomeruli. Skin and bone marrow bi...
Source: CEN Case Reports - February 3, 2021 Category: Urology & Nephrology Source Type: research

Two cases of idiopathic multicentric Castleman disease with nephrotic syndrome treated with tocilizumab
We report two cases of idiopathic multicentric Castleman disease (iMCD) with nephrotic syndrome (NS) treated with tocilizumab. Case 1 was a 58-year-old man diagnosed with iMCD prior to the onset of NS. Renal biopsy revealed membranous nephropathy, which was considered to be secondary membranous nephropathy associated with iMCD. Case 2 was a 49-year-old woman diagnosed with iMCD prior to NS. Renal biopsy revealed renal amyloidosis positive for Congo red staining and amyloid A protein immunostaining. In both the cases, the proteinuria improved after the initiation of glucocorticoid and tocilizumab therapy. Tocilizumab may be...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Bevacizumab-associated glomerular microangiopathy that occurred after postoperative chemotherapy for ovarian cancer
We present a case of a 68-year-old woman who was administered postoperative chemotherapy (carboplatin, paclitaxel, and bevacizumab) for stage IV ovarian cancer. Proteinuria and hypertension appeared after three courses; however, six courses were completed. Then, gemcitabine and carboplatin were administered for recurrence of her cancer. She was diagnosed with nephrotic syndrome after eight courses. Renal biopsy showed accumulation of periodic acid-Schiff (P AS)-positive substances in the capillary walls and para-mesangial areas. Double contouring of basement membranes was also observed. Immunofluorescence microscopy reveal...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Difficulty in managing nephrotic syndrome-associated cerebral venous thrombosis
AbstractThromboembolism is one of the most serious complications of nephrotic syndrome (NS). Although the occurrence of renal vein thrombosis or deep vein thrombosis is well recognized in NS patients, they rarely develop cerebral venous thrombosis (CVT). The mortality rate of CVT patients is still approximately 10%, and 6 –10% of patients who survive have a severe and permanent disability. Herein, we report the case of a 26-year-old woman with multiple thrombotic risk factors, including the presence of NS, use of oral contraceptives, smoking, and alcohol consumption who developed wide-range CVT. Undetermined fracti o...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Hypercalcemia caused by comorbid parathyroid adenoma and pulmonary tuberculosis
We report a valuable case in which the early diagnosis and treatment of tuberculosis and primary hyperparathyroidism prevented the spread of tuberculosis. (Source: CEN Case Reports)
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Mycobacterium abscessus exit-site infection in peritoneal dialysis patients: should we ever aim to salvage the catheter?
We report an unusual case in which an asymptomatic end-stage renal disease patient with multiple favorable clinical characteristics, i.e., no apparent immunodeficiency, sensitivity pattern showing possibility of treatment with multiple antibiotics, no evidence of peritonitis, and early clinical response, was treated with a 9-month combination antimicrobial regimen administered orally and intraperitoneally. Despite excellent clinical response with a resolution of the ESI, our patient relapsed quickly, within 30  days of stopping antimicrobial therapy and required PD catheter removal. Our case, taken together with avail...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

A case of latent heterozygous Fabry disease in a female living kidney donor candidate
AbstractA 52-year-old woman had been found to have hematuria at her annual checkup 5  years in a row. She hoped to donate her kidney to her husband, so we performed a percutaneous kidney biopsy at our department. It was difficult for us to detect apparent abnormalities under a light microscopic examination, and she was determined to meet the eligibility criteria for living kidney t ransplantation. However, the sample for electron microscopy was not evaluated before kidney donation. She subsequently underwent living kidney transplantation as a donor. A 1-h biopsy revealed swelling and obvious vacuolation of the glomeru...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Successful treatment of two Japanese ESRD cases with severe COVID-19 pneumonia
We report here two Japanese ESRD patients confirmed with severe COVID-19 pneumonia. Case 1 was a 60-year-old man with ESRD due to diabetic nephropathy who were infected with COVID-19 and exhibited acute respiratory distress syndrome (ARDS) requiring mechanical ventilation and intensive care unit (ICU) admission. He was treated with tocilizumab and intravenous immunoglobulin (IVIG). After 6  days of treatment in ICU, he was extubated. Case 2 was a 68-year-old woman undergoing maintenance hemodialysis for 17 years who also exhibited ARDS due to COVID-19. Her clinical course resembles case 1. Our experience of these...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Ultrasound appearance of the lymphomatous infiltration of the kidney
(Source: CEN Case Reports)
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Assessment of venous congestion by Doppler ultrasound: a valuable bedside diagnostic tool for the new-age nephrologist
(Source: CEN Case Reports)
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

Poststreptococcal acute glomerulonephritis in a girl with renal cell carcinoma: possible pathophysiological association
AbstractThe severity of the poststreptococcal acute glomerulonephritis is considered to be modulated by the immune response of each individual, although there had been few reports regarding specific factors. Renal cell carcinoma is a cancer frequently associated with paraneoplastic syndrome, characterized by fever, leukocytosis, elevated cytokines, and elevated hormone levels. All of these symptoms resolve after tumor resection. A girl with renal cell carcinoma developed renal failure rapidly, which resolved promptly right after nephrectomy for the carcinoma. She was diagnosed as having poststreptococcal acute glomerulonep...
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

A case of cardiac arrest due to a ruptured renal artery pseudoaneurysm, a complication of renal biopsy
In conclusion, clinicians should be aware of the possibility of severe hemorrhagic shock due to RAPs after renal biopsy. Moreover, even if the patient goes into cardiac arrest, there is a possibility of full recovery if REBOA is performed and angioembolization is completed. (Source: CEN Case Reports)
Source: CEN Case Reports - January 24, 2021 Category: Urology & Nephrology Source Type: research

An extremely mild clinical course in a case with LAMB2 -associated nephritis diagnosed with next-generation sequencing
We report a patient withLAMB2-associated renal disorder showing an extremely mild phenotype. A 5-year-old girl presented with asymptomatic proteinuria and hematuria detected by urinalysis screening. She had been previously healthy without any additional renal symptoms. The serum albumin and creatinine levels were normal. Renal biopsy revealed minor glomerular abnormalities with occasional focal mesangial proliferation. Electron microscopy showed no structural changes in the glomerular basement membrane. Targeted sequencing of podocyte-related genes using next-generation sequencing was performed. As a result, previously rep...
Source: CEN Case Reports - January 21, 2021 Category: Urology & Nephrology Source Type: research

Development of nephropathy in an adult patient after Fontan palliation for cyanotic congenital heart disease
AbstractCyanotic congenital heart disease is occasionally associated with kidney dysfunction, which is known as cyanotic nephropathy or cyanotic glomerulopathy. The clinical presentation of cyanotic nephropathy includes proteinuria, decreased estimated glomerular filtration rate, hyperuricemia, thrombocytopenia, or polycythemia. Although advances in surgical procedures have improved the prognosis of cyanotic congenital heart diseases, adult cases of cyanotic nephropathy are still rare, and there are few reports of kidney biopsy in adults with cyanotic nephropathy. Here, we present the case of a 41-year-old patient with Fon...
Source: CEN Case Reports - January 21, 2021 Category: Urology & Nephrology Source Type: research

Atypical anti-glomerular basement membrane disease complicated by methicillin-susceptible Staphylococcus aureus infection-related rapidly progressive glomerulonephritis: a case report and literature review
AbstractAtypical anti-glomerular basement membrane (GBM) disease, which is characterized by low levels of or negativity for anti-GBM antibodies in circulation but positivity in the kidney, has been recognized in this decade. However, a therapeutic strategy has not been established to date because its outcome is better than that of classic anti-GBM disease. This case report and literature review highlight atypical anti-GBM disease in infection-related rapidly progressive glomerulonephritis. A 72-year-old Japanese man diagnosed with methicillin-susceptibleStaphylococcus aureus (MSSA)-induced vertebral osteomyelitis experienc...
Source: CEN Case Reports - January 18, 2021 Category: Urology & Nephrology Source Type: research

Challenge in diagnosis of COVID-19 in hemodialysis patient: a case report and brief review of the literature
We presented a case of atypical presentation of COVID-19 in an ESKD patient on maintenance hemodialysis with a brief review of the current l iterature. (Source: CEN Case Reports)
Source: CEN Case Reports - January 16, 2021 Category: Urology & Nephrology Source Type: research

Unusual cause of persistent dyspnea in a patient with nephrotic syndrome: dapsone-induced methemoglobinemia
We report a case of an 83-year-old female with stage III chronic kidney disease, who was started on high-dose prednisone for nephrotic syndrome and dapsone for pneumocystis jirovecii pneumonia prophylaxis. A week after initiation of dapsone, this patient presented with shortness of breath, and an oxygen saturation gap while on supplemental O2 via nasal cannula, with no obvious cardiopulmonary cause. Methemoglobin level was elevated on arterial blood gas. Our patient was treated with IV methylene blue. Dapsone was determined to be the culprit and immediately discontinued. Patient responded with a rapid decrease in methemogl...
Source: CEN Case Reports - January 8, 2021 Category: Urology & Nephrology Source Type: research

Caliceal diverticulum with ureteropelvic junction obstruction in a dysplastic kidney: a pediatric case report
AbstractA caliceal diverticulum is a rare entity in children. Its etiology is closely associated with that of ureteropelvic junction malformations and renal dysplasia. We herein present a case of these complex disorders in an infant. A renal cyst and hydronephrosis were found in the left kidney during the fetal period. The postnatal diagnosis was hydronephrosis due to ureteropelvic junction obstruction and a caliceal diverticulum in the left dysplastic kidney. Although left renal function was severely decreased, the patient had no symptoms. Therefore, we did not perform surgical treatment. At the time of this writing, the ...
Source: CEN Case Reports - January 8, 2021 Category: Urology & Nephrology Source Type: research

A rare case of amyloid light-chain amyloidosis with bilateral perirenal hematoma shortly after initiation of peritoneal dialysis
AbstractPeritoneal dialysis (PD) is valuable for patients starting on renal replacement therapy because it preserves residual renal function, maintains hemodynamic stability, and affords higher quality of life than hemodialysis. Amyloid-related kidney disease is a rare condition and a cause of end-stage renal disease, the incidence of which appears to be rising in recent years. Hemoperitoneum is a common complication of PD. In some cases, it requires urgent treatment and careful monitoring for deterioration and potential complications. Although the kidney is a retroperitoneal organ, renal hemorrhage can cause bloody perito...
Source: CEN Case Reports - January 7, 2021 Category: Urology & Nephrology Source Type: research

A rare cause of acute kidney injury with chronic myelomonocytic leukemia
AbstractAn 80-year-old man with myelodysplastic syndrome developed acute kidney injury (AKI) and peripheral blood monocyte-dominant leukocytosis. Glomerular disease was suspected from urinalysis, which showed proteinuria and microscopic hematuria with red cell casts. Eventually, he died of respiratory failure, after which a postmortem was performed. In the glomeruli, the extracapillary space was filled with numerous mononuclear cells and erythrocytes. At first interpretation, the glomerular findings appeared to represent cellular crescents. However, immunostaining revealed that the extracapillary mononuclear cells were in ...
Source: CEN Case Reports - January 6, 2021 Category: Urology & Nephrology Source Type: research

A case of minimal change nephrotic syndrome with pregnancy
In conclusion, we present a case of a pregnant woman with MCNS during organogenesis. Early treatment initiation can provide a good prognosis for both mother and child. (Source: CEN Case Reports)
Source: CEN Case Reports - January 6, 2021 Category: Urology & Nephrology Source Type: research

Steroid-sensitive recurrent mesangial proliferative glomerulonephritis with monoclonal IgG deposits
We report a case of steroid-sensitive recurrent mesangial proliferative type of PGNMID. A 40-year-old female noticed lower leg pitting edema and polyuria. Approximately 10  days prior to the first clinic visit, she was diagnosed with nephrotic syndrome based on the laboratory data of urine and blood. Immunological and hematological examination revealed no abnormality. However, kidney biopsy specimens showed mild mesangial cell proliferation and mesangial matrix accum ulation on light microscopic findings. Regarding immunofluorescence staining, granular deposits of IgG, C1q, and β1c were observed on GBM and mesang...
Source: CEN Case Reports - January 4, 2021 Category: Urology & Nephrology Source Type: research

Osteomalacia caused by atypical renal tubular acidosis with vitamin D deficiency: a case report
AbstractOsteomalacia is a systemic metabolic bone disease. Hypophosphatemia is one of the most important causes of impaired mineralization. Here, we describe a case of osteomalacia associated with atypical renal tubular acidosis. A 43-year-old woman was admitted to our hospital due to sustained unrelieved bilateral flank pain. She had a history of fragile fracture with vitamin D deficiency and had been treated with active vitamin D. On admission, she presented with hypophosphatemia, hypocalcemia, high bone-specific alkaline phosphatase level, bone pain, and low bone mineral density. Multiple areas of uptake were also confi...
Source: CEN Case Reports - January 4, 2021 Category: Urology & Nephrology Source Type: research

Spontaneous remission of minimal change nephrotic syndrome in an elderly man
In conclusion, in elderly-onset MCNS, clinicians generally hesitate to initiate treatment with an im munosuppressive drug, containing steroids, because of its many complications. Thus, our data provide valuable insight into MCNS. (Source: CEN Case Reports)
Source: CEN Case Reports - January 4, 2021 Category: Urology & Nephrology Source Type: research

Malignancy-associated membranous nephropathy with PLA2R double-positive for glomeruli and carcinoma
We report a case of PLA2R-positive MN secondary to PLA2R-positive RCC. (Source: CEN Case Reports)
Source: CEN Case Reports - January 3, 2021 Category: Urology & Nephrology Source Type: research

Nodular glomerulosclerosis in a kidney transplant recipient with impaired glucose tolerance: diabetic or idiopathic? A case report and literature review
We describe a case of nodular glomerulosclerosis in a kidney transplant recipient who had not been found to be diabetic despite regular screening and discuss the implications for the pathogenesis and diagnosis of nodular glomerulosclerosis and screening of post-transplant diabetes mellitus. (Source: CEN Case Reports)
Source: CEN Case Reports - January 3, 2021 Category: Urology & Nephrology Source Type: research

Minimal change disease concurrent with acute interstitial nephritis after long-term use of sorafenib in a patient with renal cell carcinoma
AbstractSorafenib is one of the multi-targeted tyrosine kinase inhibitors (TKI), mainly used for treating advanced renal cell carcinoma. Accumulated evidence indicates a minority of patients develop nephrotic syndrome (NS) as a high-grade nephrotoxic injury; however, evidence of NS after long-term use of sorafenib remains unclear. A 64-year-old man developed NS following 2-year use of sorafenib and his NS persisted even after sorafenib use was discontinued. Renal biopsy disclosed minimal change disease (MCD) concurrent with acute tubulointerstitial nephritis, indicating secondary MCD with which sorafenib may be involved. T...
Source: CEN Case Reports - January 3, 2021 Category: Urology & Nephrology Source Type: research

Membranous nephropathy with thrombotic microangiopathy-like lesions successfully treated with tocilizumab in a patient with idiopathic multicentric Castleman disease
AbstractIdiopathic multicentric Castleman disease (iMCD) is a systemic lymphoproliferative disease involving multiple organs, including the kidneys. Membranous nephropathy (MN) has been rarely reported as a complication of iMCD. We herein report the case of a 48-year-old man with a 9-year history of iMCD that was complicated by treatment-resistant nephrotic syndrome due to MN. The first renal biopsy performed at the age of 45  years showed diffuse and global MN with a mild glomerular endothelial injury. He was treated with combined therapy of corticosteroids, immunosuppressants, and tocilizumab, an anti-interleukin-6 ...
Source: CEN Case Reports - January 2, 2021 Category: Urology & Nephrology Source Type: research

Fecal microbiota transplantation for membranous nephropathy
AbstractMembranous nephropathy is a pathological type of nephrotic syndrome. Current treatments including supportive therapy, corticosteroids, immunosuppressive agents are not effective for all patients. New therapies are needed to treat the disease safely and effectively. Gut microbiota may contribute to the pathogenesis of this disease. Fecal microbiota transplantation (FMT) has made achievements in many diseases. Here, we report a case in which FMT is used to treat a patient with membranous nephropathy and chronic diarrhea, whose symptoms ameliorated and renal function improved. (Source: CEN Case Reports)
Source: CEN Case Reports - January 2, 2021 Category: Urology & Nephrology Source Type: research

Pericardial effusion in anti-complement factor H antibody-associated atypical hemolytic uremic syndrome: two case reports
We describe two children diagnosed with anti-complement factor H (CFH) antibody-associated aHUS who had pericardial involvement. Two boys, one 10-year-old and another 8-year-old, presented with pallor, oliguria and hypertension. They both had microangiopathic haemolytic anemia, thrombocytopenia and AKI suggestive of HUS. Complement workup revealed elevated anti-CFH antibody titres. With a diagnosis of anti-CFH antibody aHUS, they were started on plasmapheresis, pulse methylprednisolone and cyclophosphamide. The first case developed cardiac tamponade during the second week of hospital stay for which he needed pigtail draina...
Source: CEN Case Reports - January 1, 2021 Category: Urology & Nephrology Source Type: research

Ischemic stroke and purpuric dermatitis as COVID-19-related complications in a peritoneal dialysis patient
AbstractPatients on dialysis may have an elevated risk of severe coronavirus disease 2019 (COVID-19) and its complications due to their high prevalence of comorbidities. Here we describe the case of an 80-year-old male undergoing peritoneal dialysis with a moderate SARS-CoV-2 infection who developed a purpuric dermatitis and ischemic stroke after successful recovery from his bilateral pneumonia. Erythemato-papular lesions affecting trunk and lower limbs appeared 17  days after the onset of SARS-CoV-2 symptoms. These kind of lesions are an infrequent cutaneous manifestation of COVID-19. The pathology revealed a moderat...
Source: CEN Case Reports - November 17, 2020 Category: Urology & Nephrology Source Type: research

Polyarteritis nodosa with perirenal hematoma due to the rupture of a renal artery aneurysm
We present the case of a 67-year-old man in good health with perirenal hematoma due to a ruptured arterial aneurysm in the kidney. The patient developed weight loss, muscle weakness, multiple mononeuropathy, hypertension, anemia, renal insufficiency, and multiple lacuna infarctions about a month ago. He was admitted to the hospital due to worsening of his symptom. After admission, severe right-flank pain suddenly occurred; he was then transferred to our hospital. Renal angiography revealed bilateral multiple microaneurysms, and the patient was diagnosed with polyarteritis nodosa based on the clinical, radiographic, and his...
Source: CEN Case Reports - November 11, 2020 Category: Urology & Nephrology Source Type: research

A case of HDR syndrome coexisting with tetralogy of Fallot, with a novel GATA3 mutation, which manifested as a renal abscess
AbstractHDR syndrome is characterized by the triad of primary hypoparathyroidism, sensorineural hearing loss and renal malformation with widely variable manifestations. It is an autosomal dominant inherited disease caused by a mutation of theGATA3 (NM_001002295.2), which is located on chromosome 10p14. Congenital heart disease, such as tetralogy of Fallot, a typical complication of DiGeorge syndrome, is a rare complication of HDR syndrome. We herein report a case of HDR syndrome coexisting tetralogy of Fallot with a novel mutation, c.964C  >  T (p.Gln322*). This case suggested that the screening of renal i...
Source: CEN Case Reports - November 7, 2020 Category: Urology & Nephrology Source Type: research

First case report of monoclonal IgG3-heavy-chain glomerulonephritis with microtubular structures
AbstractOur patient was a 69-year-old woman admitted to our hospital for heavy proteinuria and hematuria. A renal biopsy showed findings similar to those of membranoproliferative glomerulonephritis associated with nodular lesions, and immunofluorescence showed marked deposits of IgG, C1q, and C3 on the peripheral capillary walls. IgG3 alone was observed on IgG subclass staining with no κ or λ light chains, and Congo red staining was negative. These findings suggested IgG3-heavy-chain deposition disease (HCDD). However, we did not find a deletion of the first heavy-chain constant domain, which is commonly obser...
Source: CEN Case Reports - November 6, 2020 Category: Urology & Nephrology Source Type: research

Renal epithelial and stromal tumor with a multiple cystic lesion localized in the upper portion of the right kidney
AbstractA 60-year-old Japanese woman was admitted because of the polycystic mass with right flank pain localized in the upper portion of the right kidney. Right nephrectomy was performed because the mass lesion had continuously increased in size over the past 10  years. A surgical specimen showed histology consistent with a mixed epithelial and stromal tumor, which is closely related to multilocular cystic nephroma, and was diagnosed by a defined capsule between the cystic mass lesion and normal renal tissue by CT and MRI, and histology. Localized renal cy stic disease that does not have a capsule was excluded from di...
Source: CEN Case Reports - November 3, 2020 Category: Urology & Nephrology Source Type: research

Rapid progression to end-stage renal disease in a child with IgA-dominant infection-related glomerulonephritis associated with parvovirus B19
In this study, we report the case of a previously healthy 5-year-old boy with infection-related glomerulonephritis (IRGN) associated with PVB19 that progressed to end-stage renal disease (ESRD). He presented with macrohematuria, nephrotic-range proteinuria, and progressive renal dysfunction despite treatment with methylprednisolone pulse therapy, plasmapheresis, and intravenous immunoglobulin. The kidney biopsy specimens exhibited endocapillary infiltration and mesangiolysis with cellular crescent formation. Immunofluorescence analysis revealed that IgA was dominantly positive in the glomeruli, with some co-localized with ...
Source: CEN Case Reports - September 19, 2020 Category: Urology & Nephrology Source Type: research

A case with somatic and germline mosaicism in COL4A5 detected by multiplex ligation-dependent probe amplification in X-linked Alport syndrome
AbstractX-linked Alport syndrome (XLAS) is a progressive hereditary kidney disease caused by mutations in theCOL4A5 gene encoding the type IV collagen α5 chain. To date, 11 cases having somatic mosaic variants inCOL4A5 have been reported; however, all of them involved single-nucleotide variations (SNVs). Here, we report a female XLAS patient with somatic mosaicism identified by copy number variation (CNV) inCOL4A5. The case was a 35-year-old female, the mother of the proband, whose only clinical symptom was hematuria. The proband, who was the son of this patient, was diagnosed with XLAS by gene testing, which showed ...
Source: CEN Case Reports - September 19, 2020 Category: Urology & Nephrology Source Type: research

A case of acute renal failure with multiple origins of the renal injury
We report a case of 69-year-old woman. In history, type II diabetes mellitus and arterial hypertension admitted to our hospital for the evaluation of leg pain, asthenia, diarrhea, and malaise. She was in the treatment with metformin and ARB. Laboratory data revealed renal failure: serum creatinine (Scr 16.5  mg/dl, BUN 280 mg/dl) hyperkalemia and severe anemia (Hb 7.8 g/dl). Renal ultrasound displayed preserved kidneys size. An X-ray of backbone showed fracture. She underwent hemodialysis in urgency regimen. After some days, urine output began to improve up to 1200 cc/24 h. we find proteinuria in n...
Source: CEN Case Reports - September 19, 2020 Category: Urology & Nephrology Source Type: research

Efficacy of favipiravir for an end stage renal disease patient on maintenance hemodialysis infected with novel coronavirus disease 2019
ConclusionFavipiravir may be an effective option for the treatment of COVID-19-infected patients with ESRD. (Source: CEN Case Reports)
Source: CEN Case Reports - September 16, 2020 Category: Urology & Nephrology Source Type: research