Cases Of Nephropathy Research Articles

MO105: Study of 45 Cases of Membranous Nephropathy from a Kidney Biopsy Record at the Level of the Nephrology Department in Marrakech

Abstract BACKGROUND AND AIMS Membranous nephropathy is the most common cause of nephrotic syndrome in adults. However, the epidemiological data of this disease are not yet sufficiently known in our country. The aim of our study was to determine the clinical, biological and histological profile of MN and to evaluate its therapeutic approaches and evolutive aspects. METHOD We conducted a single-centre retrospective study in the nephrology department of the University Hospital Center Mohammed VI, over a period of 3 years (2016–2019) including 45 cases of MN confirmed by renal biopsy, with a minimum follow-up of 6 months. All patients were started on supportive therapy and then reassessed at 6 months. The nature of the next treatment was determined on an individual basis according to the risk of progression of each patient. The primary endpoint of the study was to obtain a remission of nephrotic syndrome. Complete remission was defined as proteinuria <0.3 g/day, and normal renal function partial remission was defined as proteinuria of 0.3–3 g /day and a stabilization of creatininemia. RESULTS The mean age of the patients was 45.24 ± 10.98 years. The clinical presentation was dominated by edematous syndrome found in 75.8% of cases. Systemic manifestations were found in 50% of cases. Nephrotic syndrome was objectified in 66.67% of cases. The histological study showed a predominance of the stage 3 in 56.7% of cases. Membranous lupus nephritis was found in 20.1% of cases, viral hepatitis B in 3% of cases, secondary syphilis in 3% of cases and primary MN in 73.9% of cases. Anti-PLA2R antibodies were positive in four patients with primary MN. After a minimal 6-month follow-up, we achieved remission in 74% of patients. Eighteen patients received aetiopathogenic treatment. Total remission was found in 48% of patients, partial in 39.4% of patients and an aggravation was noted in 15.1% of the cases progressing towards end-stage renal failure. CONCLUSION The evolution of membranous nephropathy is highly variable, but it remains a considerable cause of end-stage renal failure; hence, the need for rapid and continuous management is high in order to achieve remission and reduce the morbimortality related to membranous nephropathy.

MO221: Glomerulopathies After COVID-19 Vaccination. Report of Cases in Our Centre

BACKGROUND AND AIMSAfter the start of the vaccination campaign against the SARS-COV-2 pandemic, it is possible that the number of cases of de novo and relapsing glomerulopathies will increase.So far, most of the publications related to post-vaccination minimal change disease (also associated with other vaccines against influenza virus, hepatitis B, pneumococcus, etc.) and IgA nephropathy. However, post-vaccination cases of membranous nephropathy, ANCA vasculitis or anti-glomerular basement membrane glomerulonephritis are anecdotal.Likewise, there are no cases of segmental and focal glomerulonephritis published in the literature.We present the cases that have appeared in our centre in order to support the theory of a more than probably causal relationship. We highlight two cases of focal and segmental glomerulonephritis, making this publication the first to report this pathology post-vaccination.METHODWe present a total of 8 cases reported in our centre since the start of the vaccination campaign. We only considered cases that started with symptoms within 1 month after the first or second dose of the SARS Cov2 vaccine.Histological diagnosis was obtained in four patients with de novo glomerulopathy. In the other four patients with a flare of their baseline disease, renal biopsy was not performed. In these patients, the diagnosis of recurrence was made on the basis of laboratory and clinical data.RESULTSFive patients of the total that appeared in our centre developed minimal change disease (4 of them were recurrences and 1 de novo), 2 cases of de novo focal and segmental glomerulomephritis and 1 case of de novo IgA nephropathy.Nephrotic range proteinuria and macroscopic haematuria were the most frequent symptoms in our patients and completely reversed after immunosuppressive treatment or, in some cases, with supportive care.All of them received mRNA-based vaccines (six patients with Pfizer and two with Moderna). The mean time to onset of symptoms after vaccination was 13 days.There was no difference in sex and the mean age was 40 years.CONCLUSIONThe development of vaccines has been a key factor in the control of the SARS Cov-2 pandemic. The cases reported so far are minimal, compared with the millions of doses administered, so the benefits exceed the risks.However, according to the literature, we recommend closer follow-up in patients with known glomerulopathies and to perform renal biopsy as soon as possible after the onset of symptoms related to post-vaccination renal disease.In our centre, most of the cases appeared after the first dose of vaccination and we induced the administration of the remaining doses because all our patients did well with treatment. However, we suggested that one option might be to delay, but not to stop, the second dose in order not to interfere with the immune response to vaccination in patients who are receiving immunosuppression.

A Case of Membranous Nephropathy Hypothesized to be Associated With COVID-19 Vaccine

A 56-year-old male patient with a medical history of essential hypertension was referred to the emergency room after he was found to have a serum creatinine level of 13 mg/dL at his primary care physician’s office. The patient reported that he had developed a coronavirus disease 2019 (COVID-19)-like infection six months prior that was not confirmed. Two months later, he started to notice dyspnea on exertion and bilateral lower limb swelling and was started on furosemide. He received the first dose of the Moderna COVID-19 vaccine a month before the presentation but did not receive the second dose. Subsequently, his lower limb swelling and exertional dyspnea started worsening. He denied any new medication, dysuria, oliguria, hematuria, fever, or any other symptoms. Initial evaluation was consistent with kidney failure. Hypocalcemia and hyperphosphatemia were noted, along with medical renal disease on renal ultrasound. Eosinophils and nephrotic-range proteinuria were found in the urine. His serum phospholipase A2 receptor (PLA2R) antibodies were positive. A renal biopsy showed membranous glomerulonephritis with moderate segmental sclerosis, as well as tubulointerstitial fibrosis with neutrophils, consistent with acute interstitial nephritis. Positive staining for PLA2R in the glomerular deposits suggested primary membranous nephropathy (MN). He was treated with prednisone first, and when the kidney biopsy was conclusive for membranous glomerulopathy, he was started on rituximab. On admission, he received hemodialysis intermittently, but this was stopped a month after discharge as his renal function normalized. Recently, there have been numerous cases reported with new onset of glomerular disease after receiving the COVID-19 vaccine. Further studies of vaccinated patients are needed to determine whether the severe acute respiratory syndrome coronavirus 2 virus vaccination is associated with a higher risk of MN and to identify potential predisposing factors and mechanisms of kidney injury in patients in whom it occurs.

Patterns of biopsy-proven renal disease in people living with HIV: 10\xa0years experience in Sydney, Australia

BackgroundAcute and chronic kidney diseases are important comorbidities in People Living With HIV (PLWH). Biopsy is often pursued in this cohort with ongoing renal impairment without a clear aetiology, in order to establish the diagnosis and to guide management. Despite the importance of renal disease in PLWH, there is a paucity of biopsy data—especially in the Australian setting. Consequently, who and when to biopsy is mainly based on clinical experience. The aims of this study were to describe biopsy-proven renal disease in PLWH at our institution and to assess for correlation between any demographic or laboratory characteristics with histological diagnosis.MethodsA retrospective review of all PLWH who underwent renal biopsy between January 2010 and December 2020 at Royal Prince Alfred Hospital, Sydney, Australia was performed. All PLWH over 18 years, who were not transplant recipients were included. Demographic, laboratory and biopsy data was extracted from the electronic medical records. Basic descriptive statistics were performed, and correlation was assessed using chi square and Kendall’s coefficient of rank test.Results19 renal biopsies were included in the study. The majority of PLWH were Australian born (53%), male (84%) and had a mean age of 48 years (SD 13). Comorbid hypertension and diabetes were present in 74% and 21% of people respectively. The mean serum creatinine was 132 µmol/L (SD 55) and the mean estimated glomerular filtration rate (eGFR) was 61 ml/min/1.73m2 (SD 24). The most common histological diagnosis was tubulointerstial nephritis in 5 people (24%). Hypertensive glomerulosclerosis and IgA nephropathy were present in 4 (19%) and 3 (14%) people respectively. There were no cases of HIV-associated nephropathy. There was no significant correlation between any cohort characteristics and diagnoses.ConclusionsThis study represents the first description of biopsy-proven kidney disease in the HIV-infected population of Australia. Our results support the use of renal biopsy in PLWH with ongoing renal impairment for accurate diagnosis and to guide further management. Although a small sample size, our study is larger than other published international biopsy studies.

IgA nephropathy relapse following COVID-19 vaccination treated with corticosteroid therapy: case report

BackgroundThe flare of immune-mediated disease following coronavirus disease of 2019 (COVID-19) vaccination is a rare adverse event following immunization. De novo, as well as relapsing IgA nephropathy (IgAN) cases, have been reported following either mRNA-1273 (Moderna) or BNT162b2 (Pfizer-BioNTech) vaccination. To our knowledge, the majority of IgAN relapses did not result in severe acute kidney injury (AKI) and resolved spontaneously.Case presentationThis is a case of a 54-year-old female with a previous diagnosis of IgAN who developed IgAN relapse following the second dose of Moderna vaccine. Gross hematuria developed 2 days after vaccination, which was accompanied by significant AKI. Kidney biopsy showed mild tubular atrophy and IgA staining in mesangium without crescent formation. Significant improvement in serum creatinine (Cr) was observed on day 10 after initiating prednisone. Cr came back to normal within 3 months after initiating corticosteroid.ConclusionCOVID-19 vaccination is associated with a flare of IgAN that may cause significant AKI. Steroid therapy is associated with recovery. IgAN flare after COVID-19 vaccination should be closely monitored to elucidate any adverse effect associated with the novel vaccine.

Two Cases of BK Polyoma Virus Nephropathy in Patients with Isolated Heart Transplantation: Clinical Usefulness of Urinary Cytology

<h3>Introduction</h3> BK polyoma virus (BKpV) nephropathy (BKpVN) has been often reported in patients with kidney transplantation, whereas reports regarding BKpVN in patients with heart transplantation (HTx) is still sparse. <h3>Case Report</h3> We recently encountered two patients who developed BKpVN after HTx. Both patients (Case 1 and 2) coincidentally received HTx at the age of 56. A standard triple-drug regimen consisting of tacrolimus, mycophenolate mofetil (MMF), and a corticosteroid was started after surgery and the regimen was converted to everolimus (EVL) with reduced tacrolimus within 6 months after HTx because of donor transmitted atherosclerosis in both patients. Case 1 further underwent percutaneous coronary intervention 3.5 years after HTx. Despite renal-sparing immunosuppresion, values of creatinine begun to increase after HTx in both patients and they were diagnosed with BKpVN through high viral DNA copy number in the urine and blood. Regarding case 1, pathological analysis of kidney biopsy specimens disclosed interstitial infiltrate with lymphocytes associated with tubular injury which also lead to the diagnosis of BKpVN. Retrospectively, both patients were positive for decoy cell within 1 year after HTx therefore, these patients should have been recognized as high risk for BKpVN. After the diagnosis of BKpVN, immunosuppressive regimen of them was further changed to minimum dose of EVL (a trough concentration of 6-7 ng/ml) and MMF (500 mg daily) respectively under the guidance of myocardial biopsy and they have been uneventful for over a year after the diagnosis of BKpVN despite severe chronic kidney disease. <h3>Summary</h3> We presented two rare cases of BKpVN after HTx. In our institution, urinary cytology is regularly examined if there are decoy cells which we think are the sign of over immunosuppression. However, decoy cells detected by urinary cytology also suggest that there is subclinical BKpV infection. Urinary cytology, a noninvasive and cost-effective examination, should be routinely performed for the risk assessment of BKpVN even in HTx patients, since detecting decoy cell would be helpful for the early diagnosis of BKpVN.

168 A Novel Case of C1q Nephropathy in an HIV Patient With Kaposi Sarcoma and TTP

168 A Novel Case of C1q Nephropathy in an HIV Patient With Kaposi Sarcoma and TTP

Prevalence of nephropathy in Indian children and youth with type 1 diabetes mellitus.

Children with type 1 diabetes (T1D) having diabetic nephropathy (DN) are at increased risk of developing end stage renal disease. The present study aimed to determine the prevalence of DN and its predictors in Indian children and youth with T1D. This cross-sectional study included 319 children and youth (2.6-21 years) with T1D having disease duration of at least 2 years. Demographic data and laboratory findings were obtained using standard questionnaires and protocols. Diagnosis of diabetic nephropathy was based on albuminuria on two occasions within a period of 3months. The prevalence of DN in our study subjects was13.4%. 7.5% subjects were known cases of diabetic nephropathy on treatment with enalapril. Hypertension was found in 14.3% subjects with DN in contrast to 4.1% without DN (p<0.05). Duration of diabetes and estimated glucose disposal rate were the important predictors of DN. Interestingly, of the 43 children with DN, 11.3% (n=8) were under 10 years age. We found a high prevalence of DN in children and youth with T1D including in children under the age of 10 years. Early screening and timely intervention are required to retard the disease progression and avoid end stage renal disease.

De Novo Membranous Nephropathy Associated With Antibody-Mediated Rejection in Kidney Transplant Recipients

Membranous nephropathy (MN) is a rare autoimmune disease that can develop a persistent nephrotic syndrome and end-stage kidney disease, with a recurrence rate of 30% to 40% after kidney transplant. Retrospective case series of membranous nephropathy observed in a cohort of kidney transplant recipients with donor-specific anti-human leukocyte antigen antibodies and biopsy-proven antibody-mediated rejection (AMR). We report 4 cases of membranous nephropathy associated with AMR. MN was diagnosed 10 to 92 months posttransplant, associated with de novo donor-specific antibodies, specific to class I in 2 cases, and class II in another 2. All cases presented typical morphology of membranous nephropathy, with subepithelial deposits with spikes at electron microscopy. Immunostaining for immunoglobulin G4 was negative in all cases, and podocyte-expressed M-type phospholipase A2 receptor was detected in glomerular basement membrane of 3 cases. Biopsy specimens from patients with longer follow-up showed more intense microvascular inflammation and chronic injury markers, possibly because of subclinical immunologic injury. AMR therapy included immunoglobulin 2g/kg in 3 patients, isolated or associated with plasmapheresis. One patient was not treated because of an active disseminated infection. Two patients remain with functioning grafts and under antiproteinuric therapy. Two grafts were lost, 1 because of chronic failure and the other because of death secondary to infection. Despite treatment, donor-specific antibodies remain detectable in a 6-month follow-up. De novo MN is a rare manifestation associated with AMR in kidney transplant recipients. The occurrence of podocyte-expressed M-type phospholipase A2 receptor in de novo MN suggests antibody-mediated activation, despite the use of maintenance immunosuppression.

A case of membranous nephropathy with Kimura’s disease

The association between membranous nephropathy (MN) and Kimura's disease (KD) has been reported in recent years. The treatment, effect, and prognosis of KD are still unclear. A 47-year-old KD patient developed a left axillary mass for 3 years and received surgical resection because of the lager mass in August 2016. Then he developed nephrotic syndrome 3 months later. Laboratory index revealed increased eosinophil count, decreased albumin and heavy proteinuria. Lymph node biopsy suggested KD, and renal biopsy suggested MN. He relapsed after a treatment with methylprednisolone (52 mg/d) alone and then tacrolimus (1.5 mg/12h) was added. The patient had no symptoms of relapse in the next 2 years. The combination therapy of surgery, methylprednisolone, and immunosuppressive agents may be effective in KD with MN.

Паранеопластическая нефропатия. Клиническое наблюдение

The case of membranous nephropathy manifested in nephrotic syndrome in a patient, newly diagnosed with lung cancer, is reported. Brief data on the paraneoplastic glomerular disease are provided, the need for cancer screening in elderly patients with nephrotic syndrome is emphasized.

A case report of aneurysmal subarachnoid hemorrhage in a Young with IgA: A mere co-existence?

Introduction and importanceAneurysmal Subarachnoid hemorrhage (SAH) can be associated with various other conditions like Polycystic kidney disease, Ehler Danlos syndrome, Co-arctation of the aorta, etc.Case presentationHere we have reported a 21-year young lady with IgA nephropathy and aneurysmal SAH managed successfully.Clinical findings and investigationsThe patient was a known case of IgA nephropathy diagnosed 18 months back from a kidney biopsy. She came to the emergency with a headache and vomiting for 2 weeks. Her initial headache was the worst she has had experienced and then the headache was continuous and not relieved by medications. Then she underwent a noncontrast CT scan of the head which showed Subarachnoid hemorrhage (SAH).Interventions and outcomesLeft pterional craniotomy and microsurgical clipping of left ICA aneurysm performed. The patient was discharged without deficits.Relevance and impactAneurysmal Subarachnoid hemorrhage(SAH) can be due to histopathological changes to the vessels following IgA nephropathy. These correlations could be studied further or else it could be only a mere coincidence.

Schistosoma japonicum infection associated with membranous nephropathy: a case report

BackgroundSchistosomiasis is one of the most contagious parasitic diseases affecting humans; however, glomerular injury is a rare complication mainly described with Schistosoma mansoni infection. We report a case of membranous nephropathy associated with Schistosoma japonicum infection in a Chinese man.Case presentationA 51-year-old Chinese male with a long history of S. japonicum infection presented to the hospital with a slowly progressing severe lower limb edema and foaming urine for over 5 months. Serum S. japonicumantigen test was positive and immunohistochemistry showed that the glomeruli were positive for the antigens. The renal pathologic diagnosis was stage III membranous nephropathy. The patient was treated with glucocorticoid, praziquantel, and an angiotensin-converting enzyme inhibitor. The edema in both lower limbs disappeared within 2 weeks, but his renal function declined progressively and proteinuria persisted after 5 months of therapy.ConclusionsDifferent classes of schistosomal glomerulopathy have completely different clinical manifestation and prognosis. Therefore, efforts should focus on alleviating symptoms, prevention, and early detection. S. japonicumassociated with membranous nephropathy may show a good curative effect and prognosis. However, it is necessary to monitor the renal function in such patients.

POS-106 CONTINUOUS AMBULATORY PERITONEAL DIALYSIS FOR ACUTE KIDNEY INJURY

Continuous ambulatory peritoneal dialysis is a modality used for Renal replacement in patients with End stage renal disease. Herein we report a patient who developed recurrent cardiac arrest with hemodialysis, and hence CAPD was done to treat her Acute Kidney Injury (AKI). A 55 year old woman presented with complaints of pedal edema, progressive dyspnea from last 1 month, with reduced urine output from last 2 days. Known case of diabetic nephropathy since 1 year, with baseline creatinine of 3.2 mg/dl. History of native medication use for one week. She was suspected to have worsening CKD due to native medicines with a serum creatinine of 10.2 mg/dl, and planned for hemodialysis. She was started on hemodialysis and developed cardiac arrest within half hour of initiation, and further developed seizures within half hour of starting second session, and another episode of cardiac arrest within ten minutes of starting third session despite change of dialyzers and heparin. A possibility of dialyzer reaction was considered and she was planned for continuous ambulatory peritoneal dialysis for her AKI. She underwent bedside insertion of Swan neck Tenckhoff catheter and initiated on CAPD, with 3 exchanges per day and discharged home after 4 days. After two weeks, she developed gradual recovery of renal function and became dialysis independent with creatinine of 4.4 mg/dl. CAPD has rarely been used as a modality for treatment of dialysis for AKI. We report a case of successful use of CAPD for AKI in a patient with repeated cardiac arrests. CAPD can therefore be used with good outcomes in resource limited settings.

POS-006 ACUTE KIDNEY INJURY DUE TO CRYSTAL NEPHROPATHY SECONDARY TO DARUNAVIR

Kidney disease continues to be a problem with antiretorviral therapy in patients with HIV infection, its incidence varies from 2.7 to 6.9 per 100 person-years. There is growing evidence that antiretorviral therapy can cause a wide variety of nephrotoxic effects and severe acute kidney injury, however, reported cases of crystalline nephropathy from the use of Darunavir (DRV) are rare.

POS-513 PLASMA EXCHANGE IN NEPHROLOGY

Plasma exchange is a therapeutic tool with broad indications in different diseases. The aim of this study was to determine its indications and possible complications in the nephrology setting. This is a retrospective study of therapeutic plasma exchange performed in our department over 15 years [2006 to 2021]. Our study included 20 patients with an average age of 46.45 years [18-77] and a sex ratio of 0.66. The initial symptomatology was variable. A glomerular syndrome was present in all patients associated with rapidly progressive renal failure in 15 cases with an average blood creatinine level of 645.05 µmol/L [144-2017], 12 cases of Haemoptysis on chest CT scan or bronchoalveolar lavage and one case of cerebellar syndrome. Immunologically, 7 cases were positive for antiGBM antibodies, 4 cases positive for anti-MPO antibodies, 3 cases positive for antiPR3 antibodies, and serum complement was consumed in 3 patients. Renal biopsy performed in 10 patients revealed 1 case of myeloma nephropathy, 2 cases of lupus nephropathy, 3 cases of vasculitis, and 4 cases of Goodpasture's disease. Indications were diverse and included rapidly progressive glomerulonephritis, acute humoral rejection in a renal transplant patient, hyperviscosity syndrome in a myeloma patient, and a case of acute polyradiculoneuritis. The products used were albumin with fresh frozen plasma in all patients and albumin alone for the renal transplant patient, with an exchange volume of 50cc/kg. The average number of sessions was 6.3 [1-16] with a frequency of 1 to 3 sessions per week. Complications ranged from 2 episodes of circuit clotting to 2 episodes of low blood pressure, one case of acute coronary syndrome, one case of seizure followed by cardiac arrest and death. The progression was marked by an improvement of the renal function in 8 patients, the use of extrarenal purification in 9 patients, and the death of 2 patients. The indications for therapeutic plasma exchange are increasingly broad in the nephrology setting, including neurological and hematological disorders in patients with various kidney diseases.

Multiplex Immunofluorescence Assay of Infiltrating Mononu-Clear Cell Subsets in Acute T-Cell-Mediated Rejection and BK Virus-Associated Nephropathy in the Allograft Kidney.

Renal allograft biopsy is the gold standard procedure for diagnosis of kidney rejection via specific pathological changes. To provide a better assessment of immunologic events in acute T-cell-mediated rejection (acute TCMR) and BK virus-associated nephropathy (BKVAN) cases, we used multiplex immunofluorescence staining to identify infiltrating mononuclear cell subsets in the cortex area of transplanted kidneys. Antibodies to CD4, CD8, CD20, CD68, Foxp3, and cytokeratin were used. In cortical interstitium, CD8+ cells were significantly more prevalent in acute TCMR than BKVAN cases (34% vs. 22.8%, p = 0.034). In medulla, CD20+ cells were significantly more prevalent in BKVAN than acute TCMR cases (51.9% vs. 11.3%, p = 0.028).

Spectrum of Biopsy Proven Primary Tubulointerstitial Diseases of the Kidney: A Single Centre Study

Background: The tubulointerstitial diseases can be due to a wide variety of etologies and mechanisms which vary with age, race and geographical area. In this study we aim to study the clinicopathological spectrum of biopsy proven primary tubulointerstitial diseases among patients in North India.&#x0D; Methods: This is a 5 year retrospective, single centre cohort, observational study.The data base was searched to identify all patients with primary tubulointerstitial diseases in their native kidney biopsies.&#x0D; Results: A total of 3091 renal biopsies were studied of which 283 were primary tubulointerstitial diseases. The mean age of patients was 43.2 (±14.6) years. Most of the patients were males with acute kidney injury as the most common presentation. Tubulointerstitial nephritis was the most common disease occurring in 126 patients. Acute interstitial nephritis was seen in 81 patients and chronic interstitial nephritis was seen in 45 patients. Granulomatous interstitial nephritis was seen in 27 patients. There were four patients of pigment nephropathy, 83 cases of acute tubular necrosis and 19 cases of cast nephropathy.&#x0D; Conclusion: Primary tubulointerstitial diseases comprise about 9.1% of the total cases. Tubulointerstitial nephritis is the most common diagnosis in these patients. Tuberculosis is still a leading cause of granulomatous interstitial nephritis in our country followed by fungal etiology. Acute cortical necrosis affects mostly females post obstretric complications in our setup. Given the enemic nature of malaria in India, G-6PD deficiency is an important differential in patients presenting with pigment nephropathy.&#x0D; Keywords: Biopsy, Tubulointerstitial Diseases of the Kidney, Tuberculosis, Acute cortical necrosis

Clinicopathologic Features of Mitochondrial Nephropathy

IntroductionThe clinicopathologic characteristics of nephropathy associated with mitochondrial disease (MD) remain unknown. We retrospectively analyzed a cohort of patients with proteinuria, decreased glomerular filtration rate, or Fanconi syndrome who had a genetic mutation confirmed as the cause of MD, defined as mitochondrial nephropathy.MethodsThis nationwide survey included 757 nephrology sections throughout Japan, and consequently, data on 81 cases of mitochondrial nephropathy were collected.ResultsThe most common renal manifestation observed during the disease course was proteinuria. Hearing loss was the most common comorbidity; a renal-limited phenotype was observed only in mitochondrial DNA (mtDNA) point mutation and COQ8B mutation cases. We found a median time delay of 6.0 years from onset of renal manifestations to diagnosis. Focal segmental glomerular sclerosis (FSGS) was the most common pathologic diagnosis. We then focused on 63 cases with the m.3243A>G mutation. The rate of cases with diabetes was significantly higher among adult-onset cases than among childhood-onset cases. Pathologic diagnoses were more variable in adult-onset cases, including diabetic nephropathy, nephrosclerosis, tubulointerstitial nephropathy, and minor glomerular abnormalities. During the median observation period of 11.0 years from the first onset of renal manifestations in patients with m.3243A>G, renal replacement therapy (RRT) was initiated in 50.8% of patients. Death occurred in 25.4% of the patients during the median observation period of 12.0 years. The median estimated glomerular filtration rate (eGFR) decline was 5.4 ml/min per 1.73 m2/yr in the cases, especially 8.3 ml/min per 1.73 m2/yr in FSGS cases, with m.3243A>G.ConclusionHere, we described the clinicopathologic features and prognosis of mitochondrial nephropathy using large-scale data.

Yellish kidney: a case of cast nephropathy

Yellish kidney: a case of cast nephropathy