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Immunoglobulin G (IgG) nephropathy refers to a rare group of diseases characterized by deposits of IgG in the mesangial region. However, IgG nephropathy is controversial as a single disease entity, and its pathogenesis remains to be elucidated. In the present report, we discuss a case of IgG nephropathy in which we observed activation of the classical complement pathway.A 47-year-old woman was admitted to our hospital with nephrotic syndrome. Light-microscopic examination revealed neither proliferative nor sclerotic lesions in the glomeruli. However, unusual and large deposits were observed in the paramesangial area. An immunofluorescence study revealed predominant IgG and C1q and slight C3 deposits in the paramesangial area, suggesting immune-complex-type glomerular disease. An electron microscopic study also revealed different sizes of non-organized electron-dense deposits with a similar pattern of distribution, which were accompanied by foot process effacement. Clinically, there was no evidence of systemic diseases, such as infectious or autoimmune diseases (including systemic lupus erythematosus). Based on these findings, she was diagnosed with IgG nephropathy and treated with prednisolone. Steroid therapy was effective, and complete remission was maintained.Additional immunological examination revealed that IgG deposits were polyclonal and consisted mainly of the IgG1 and IgG3 subclasses. Furthermore, staining was positive for C4d and C5b-9. The present findings indicate that the pathogenesis of IgG nephropathy in our patient may have involved activation of the classical complement pathway.

DOI： 10.1007/s13730-022-00710-5

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A 44-year-old woman was admitted due to gross hematuria and progressive renal dysfunction. Poststreptococcal acute glomerulonephritis (PSAGN) was suspected due to her elevated anti-streptolysin O and anti-streptokinase titers and hypocomplementemia. A renal biopsy showed crescent formation and endocapillary hypercellularity with neutrophil infiltrate. An immunofluorescence analysis showed granular immunoglobulin G and C3 deposition, suggesting immune-complex-type glomerulonephritis. However, myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA) was positive, and peritubular capillaritis was observed. Furthermore, citrullinated histone H3-positive neutrophils were detected as markers for neutrophil extracellular trap formation. Therefore, she was diagnosed with ANCA-associated vasculitis superimposed on PSAGN that was the main contributor to her progressive renal injury.

DOI： 10.2169/internalmedicine.8690-21

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DOI： 10.1016/j.ekir.2022.08.020

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BACKGROUND: Febuxostat is recommended for lowering serum uric acid (sUA) concentration in chronic kidney disease (CKD) patients with hyperuricemia. However, it remains uncertain how febuxostat affects associations between several laboratory variables related to glomerular filtration and renal tubular reabsorption of uric acid. METHODS: We retrospectively analyzed the records of 148 patients with CKD and hyperuricemia: 122 were treated with febuxostat, and 26 were not. Clinical and laboratory variables were used to calculate estimated glomerular filtration rate (eGFR), fractional excretion of uric acid (FEUA), and estimated 24-h urinary excretion of uric acid (eEUA). We examined correlations of those variables and compared patients who did and did not receive febuxostat. RESULTS: eGFR and FEUA were significantly inversely regardless of febuxostat-treatment status. eGFR was significantly inversely correlated with sUA in patients who received febuxostat, but not in those who did not. Similarly, there was a significant positive correlation between FEUA and eEUA only in patients treated with febuxostat. CONCLUSIONS: FEUA increased as eGFR decreased in our patients. Febuxostat changed correlation patterns for clinical and laboratory variables. Additional administration of uricosuric agents might help further lower sUA by increasing FEUA and eEUA in patients treated with febuxostat.

DOI： 10.1272/jnms.JNMS.2022_89-401

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Glomerular capillary aneurysms are distinctly rare and specific glomerular lesions characterized by aneurysmal dilatation of the glomerular capillaries. This formation is associated with glomerular capillary injuries with focal mesangiolysis. Here, we report a case of proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) presenting with multiple glomerular capillary microaneurysms. A 53-year-old woman presented with persistent proteinuria and microhematuria. She had no underlying diseases, such as hematopoietic or lymphoproliferative disorders. A renal biopsy showed diffuse membranoproliferative lesions with foam cell infiltration and multiple microaneurysms of the glomerular capillary on light microscopy. Immunofluorescence analysis showed granular deposits of monoclonal immunoglobulin G3 kappa (IgG3κ), C1q, C3, and C4 in the glomeruli. Electron microscopy revealed different sizes of non-organized electron-dense deposits in the mesangial, subendothelial, and subepithelial areas. In addition, glomerular endothelial cells showed swelling and loss of fenestra or diffuse formation of fenestrated diaphragms, accompanied by irregular thinning of the glomerular basement membrane. Furthermore, immunostaining for CD31 (a marker for endothelial cell) and low-vacuum scanning electron microscopy study identified loss of endothelial cells in microaneurysm, suggesting severe glomerular endothelial cell injury. After a renal biopsy, only the medication for dyslipidemia was continued because there were no physical symptoms, such as edema, and urinary abnormalities continued with stable renal function. Further studies are needed to elucidate the pathogenesis of glomerular capillary injury in PGNMID and clarify the clinical and pathological characteristics of PGNMID with glomerular capillary microaneurysms.

DOI： 10.1007/s13730-021-00676-w

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BACKGROUND: Tolvaptan is the first effective drug treatment for autosomal dominant polycystic kidney disease (ADPKD) patients, but few long-term observations of the effects of tolvaptan have been reported. METHODS: In this single center, retrospective cohort study, we investigated nine patients who participated in a phase 3 trial of tolvaptan for ADPKD patients at our hospital between 2008 and 2014. Six of the patients discontinued tolvaptan at the end of the clinical trial and were defined as the discontinuation group, and three continued to take it; these were defined as the continuation group. The observation period was 3 years before and after the end of the tolvaptan trial, and we compared the following data in each group: serum creatinine, estimated glomerular filtration rate (eGFR), total kidney volume, serum sodium concentration, and urine specific gravity. RESULTS: eGFR was significantly improved after the end of the trial in the continuation group (P = 0.0446), but there was no significant change in the regression line before and after the end of the trial in the discontinuation group. The increases in mean total kidney volume rates over the 3 years before and after the trial were 0.01%/year vs. 0.067%/year in the discontinuation group (P = 0.0247). On the other hand, serum sodium concentration and urine specific gravity showed no change during the observation period. CONCLUSION: This study suggested that long-term administration of tolvaptan may improve renal function and inhibit total kidney volume growth.

DOI： 10.1272/jnms.JNMS.2022_89-303

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INTRODUCTION: Focal segmental glomerular sclerosis (FSGS) is caused by podocyte injury. It is characterized by obliteration of glomerular capillary tufts with increased extracellular matrix (ECM). Altered communication between podocytes and glomerular endothelial cells (ECs) contributes to sclerosis progression. We focused on EC injury in the FSGS. METHODS: A total of 29 FSGS and 18 control biopsy specimens were assessed for clinicopathologic characteristics. CD34 (a marker for EC)-positive capillaries and ECM accumulation were evaluated quantitatively for each variant using computer-assisted image analysis. RESULTS: The estimated glomerular filtration rate (eGFR) in the FSGS group was significantly lower than that in the control group. The frequency of FSGS variants was 51.7% for cellular; 13.8% for perihilar (PH), tip, and not otherwise specified (NOS); and 6.9% for collapsing. Regarding sclerotic lesions in all FSGS, narrowing or loss of CD34-positive capillaries was observed. Electron microscopy results showed loss of fenestrae, subendothelial space enlargement, and cytoplasmic swelling, indicating EC injury. Computer-assisted image analysis revealed significantly smaller areas of glomerular capillaries in FSGS with or without sclerotic lesions, with increased ECM. Moreover, in comparison with each variant, narrowed capillaries and ECM accumulation were most prominent in the collapsing variant, whereas the tip variant had the least change. CONCLUSION: EC injury was observed in all FSGS cases, not only in sclerotic lesions but also in nonsclerotic lesions. Severity of EC injury may vary in each variant due to diverse alterations of glomerular capillary networks.

DOI： 10.1016/j.ekir.2022.03.007

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BACKGROUND: The number of patients on peritoneal dialysis (PD) in our hospital has increased during the past 5 years, but the number discontinuing PD has also increased. The purpose of this study was to identify the risk factors for PD discontinuation by analyzing the association between technical survival period (defined as the duration of PD) and various clinical factors. METHODS: We retrospectively investigated 87 patients who were started on PD at our hospital and attended regularly from April 2015 to March 2020, and we analyzed the association between technical survival period and various clinical factors. We also looked for associations between technical survival period and hospitalizations for heart failure, peritonitis, and exit-site infections among patients undergoing PD. RESULTS: The patients using renin-angiotensin-aldosterone system inhibitors (RASi) (P = 0.0218), those with left ventricular ejection fraction (LVEF) > 50% (P = 0.0194) when they started PD, and those with estimated glomerular filtration rate (eGFR) ≥ 6 (mL/min/1.73 m2) (P = 0.0013) at the initiation of PD showed significantly longer technical survival period, and those who were hospitalized for heart failure had significantly shorter period (P = 0.0008). CONCLUSION: Treatment of RASi, LVEF > 50% and eGFR ≥ 6 mL/ min/1.73 m2 when the initiation of PD and better volume control to prevent ultrafiltration failure and heart failure may improve technical survival period in patients undergoing PD.

DOI： 10.1272/jnms.JNMS.2022_89-201

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Combination therapy, consisting of immune checkpoint inhibitors and traditional chemotherapeutic agents, has significantly improved the clinical outcomes of non-small cell lung cancer. Therefore, it will be a promising first-line therapy, whereas, there is a prospect that associated kidney injury may increase during treatment. We presented four patients, diagnosed with advanced non-small cell lung cancer, who received combination therapy, consisting of pembrolizumab, cisplatin, and pemetrexed as first-line treatment. All of them had been referred to nephrologists and had undergone renal biopsy. We observed that three of four patients presented a very rapid time course for acute kidney injury development. Notably, the three patients received only one or two cycles of the combined chemotherapy. In a renal biopsy, one patient showed severe acute tubular injury rather than interstitial nephritis. Another patient presented focal segmental glomerular sclerosis concomitant with tubulointerstitial nephritis. However, it was challenging to distinguish which agent was primarily responsible for kidney injury. Regarding the treatment, all the patients discontinued pembrolizumab and received corticosteroid treatment. We adjusted the dose and duration of corticosteroid according to the pathological results and patient conditions. The current cases provide a further understanding of clinical features and appropriate management in patients treated with combination therapy including pembrolizumab.

DOI： 10.1007/s13730-021-00636-4

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BACKGROUND: There are numerous causes of liver function disorder in patients undergoing peritoneal dialysis (PD). Infection with the Hepatitis E virus (HEV) is a rare cause of liver injury, and the behavior of HEV in patients with PD is unclear. Since patients undergoing dialysis are frequently polypharmatic, liver injury caused by HEV infection may be misdiagnosed as drug-induced liver injury. CASE PRESENTATION: A 61-year-old woman with PD developed abrupt elevation of blood transaminase levels on a routine outpatient session. Since the patient has been receiving tolvaptan as the only new medication, we suspected tolvaptan induced liver injury. In further investigating the cause of liver injury, the blood screening test was found to be positive for HEV-IgA. The patient was diagnosed with HEV infection, and had a self-limited course. CONCLUSION: When encountered with patients developing liver injury during PD, HEV infection should be included in the differential diagnosis.

DOI： 10.2147/IMCRJ.S385393

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Non-typhoidal Salmonella infection is a typical food-poisoning disease, which rarely causes bacteremia, except in immunocompromised individuals. We report the case of a healthy adult in whom a varicose vein associated with a lower-leg venous malformation was considered the source of Salmonella bacteremia.

DOI： 10.1272/jnms.JNMS.2022_89-614

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BACKGROUND: Peritonitis is one of the most common complications in patients undergoing peritoneal dialysis, (PD) but it is difficult to predict or prevent. In this study, we analyzed the risk of endogenous peritonitis in patients receiving PD. METHODS: We included all patients who underwent PD at our hospital from April 2015 to March 2020. There were 22 cases of peritonitis, including 18 cases of endogenous peritonitis without evidence of exit-site infection or technical failure. We evaluated older age, female sex, obesity, diabetes, diverticulosis, and constipation as potential important risk factors for endogenous peritonitis and included these as confounding factors, along with a current or previous history of smoking, in univariate logistic regression models. RESULTS: A previous or current history of smoking (p = 0.0065) was the most significant risk factor for endogenous peritonitis in the univariate logistic regression model. In addition, smoking was the most significant independent risk factor for endogenous peritonitis (p = 0.0034) in multivariate logistic regression models. Diabetes was also significant in univariate and multivariate logistic regression analysis. CONCLUSIONS: Smoking is a significant independent risk factor for endogenous peritonitis in patients undergoing PD. Cessation of smoking may lower the risk of endogenous peritonitis in this patient group.

DOI： 10.1272/jnms.JNMS.2021_88-604

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DOI： 10.1093/omcr/omab103

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Language：Japanese   Publisher：(株)東京医学社  

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Endocapillary proliferation occurs in various types of glomerulonephritis (GN), with varying prognoses. We examined 42 renal biopsy samples representing endocapillary proliferative lesions from post-streptococcal acute GN (PSAGN), Henoch-Schönlein purpura nephritis (HSPN), and lupus nephritis (LN). In PSAGN, the glomerular capillary network was maintained, although severe lesions displayed dots or short, curved lines, indicating CD34-positive capillaries and suggesting capillary obstruction. Conversely, patients with LN and HSPN displayed obstruction of CD34-positive capillaries with dissociation from the glomerular basement membrane even in mild lesions. According to computer-assisted morphologic analysis, the cell density did not differ between the diseases. However, in PSAGN, the number of capillary loops was significantly increased, with a larger glomerular capillary luminal area than in the other groups. In addition, the number and frequency of CD163-positive cells (M2 macrophages) tended to be higher in PSAGN, while there were no significant differences in the number of CD68-positive (total) macrophages. These results indicate that in PSAGN, endothelial cell damage is less severe, and angiogenesis may be promoted. The severity of endothelial cell injury in each disease may be associated with differences in infiltrating inflammatory cell phenotypes.

DOI： 10.1038/s41598-021-92655-5

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Background Despite the abundant experience of tonsillectomy with steroid pulse therapy (TSP) for patients with immunoglobulin A (IgA) nephropathy, the therapeutic efficacy of TSP on renal prognosis remains controversial. The purpose of this study was to evaluate the efficacy of whether TSP effectively prevents chronic kidney disease (CKD) progression. Methods This was a single-center, retrospective observational study. A total of 149 patients were enrolled in the current study who were confirmed with IgA nephropathy by renal biopsy between February 2011 and August 2019. The impact of TSP on CKD progression was compared with conservative treatment during a follow-up period of 3 years. Results In total, 110 patients received TSP and 39 patients received conservative treatment. There were no differences between the two groups in the initial CKD stages: 65.1% of patients had CKD G1-2, 32.2% had CKD G3, and 2.7% had CKD G4-5. The initial urine protein was 0.7 g/gCr, which was not different between the two groups. Kaplan-Meier analysis showed that patients with TSP had a significantly better renal prognosis than those in the conservative treatment group after one and a half years (p = 0.007). Multivariable analysis revealed that TSP had a significant impact on the prevention of CKD progression, with an adjusted odds ratio of 0.07 (95% confidence interval, 0.01-0.87; p=0.039). However, we could not confirm the predictive value of the Oxford Classification on TSP efficacy. Additionally, the initial urinary protein level was a risk factor for CKD progression. Conclusions TSP was associated with a lower risk of CKD progression. In this regard, our study supports that TSP may be a reasonable treatment option for patients with IgA nephropathy. In the featured study, it needs to be elucidated which histopathological classifications benefit from TSP treatment.

DOI： 10.7759/cureus.15736

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Language：Japanese   Publisher：(株)東京医学社  

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Leriche syndrome is an aortoiliac occlusive disease with three chief symptoms: claudication, impotence, and weak femoral pulse. It can also cause occlusion of the aorta up to the level of the renal arteries. We report a case in which aortoiliac bypass and renal artery thrombectomy were effective in ameliorating acute kidney injury caused by bilateral renal artery thrombosis.

DOI： 10.1272/jnms.JNMS.2021_88-610

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PURPOSE: Peritoneal dialysis (PD)-related peritonitis and lower limb ulcer are the important complications in patients undergoing PD. Although the association between lower limb ulcer and peritonitis in patients undergoing PD is unclear, based on our clinical experience and the clinical importance of the complications in patients undergoing PD, we hypothesized that lower limb ulcer is associated with peritonitis in patients on PD. PATIENTS AND METHODS: In this single center, retrospective cohort study, we studied 87 patients who started undergoing PD at our hospital from April 2015 to March 2020. We compared these 8 patients with lower limb ulcer with the other 79 patients without lower limb ulcer. We compared between the patients in the objection period of this study about peritonitis using Log rank test, and used the unpaired t-test and Fisher's exact test to compare the clinical factors between the two groups. Moreover, we used univariate and multivariate logistic regression analyses to study the association of PD-related peritonitis with the clinical factors. RESULTS: The period developed first peritonitis of the patients on PD with lower limb ulcer was significant shorter than those without lower limb ulcer in Log rank test (P = 0.011). The Fisher's exact test and unpaired t-test showed that the difference in the prevalence of PD-related peritonitis (P = 0.009), peritonitis/patient years (P = 0.036), the BMI (P = 0.007) and icodextrin (P = 0.001) were significant. Lower limb ulcer had significant associations with peritonitis in patients on PD in both univariate [odds ratio (OR) 8.461, 95% confidence interval (CI) 1.854-45.60, P = 0.006] and multivariate [OR 7.169, 95% CI 1.519-39.480, P = 0.013] logistic regression analysis. CONCLUSION: In conclusion, lower limb ulcer may be associated with peritonitis in patients undergoing PD. Further large-scale, prospective studies are required to confirm these results.

DOI： 10.2147/IJNRD.S295948

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A 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 therapy was started followed by oral administration of prednisolone. Urinary protein excretion diminished after 1 year of treatment. Eleven years later, the same patient was admitted to our hospital again with relapse of nephrotic syndrome. Laboratory findings upon second admission, wherein both anti-nuclear and anti-double-stranded DNA antibodies revealed positive, fulfilled the ACR criteria. Renal biopsy was performed again, resulting in a diagnosis of lupus nephritis. Steroid therapy combined with administration of mycophenolate mofetil led to an incomplete remission. Immunofluorescence studies confirmed the presence of IgG, IgM, C3, and C1q in renal biopsy specimens both at first and second admissions. Furthermore, immunofluorescence studies confirmed the presence of IgG1-4 in the first biopsy and tubuloreticular inclusions (TRIs) were revealed using electron microscopy. The present case represents the possibility that characteristic pathological findings of lupus nephritis, including TRIs, can reveal themselves before a diagnosis of SLE.

DOI： 10.1007/s13730-019-00412-5

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Language：Japanese   Publisher：(株)日本メディカルセンター  

＜文献概要＞透析患者は,インフルエンザのハイリスク群であり,重症化しやすく,死亡率も高いといわれている.透析施設は,集団で長時間にわたり透析治療を行うことが必要であり,インフルエンザに感染しても透析を続ける必要があるため,インフルエンザ流行時には,アウトブレイクする可能性がある.このため日頃より患者教育・スタッフ教育・環境整備などの対策を講じる必要があり,流行状況には細心の注意を払い早期発見および早期からの抗インフルエンザ薬の投与を行うことが重要である.

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A 61-year-old woman was admitted to our hospital because of muscle paralysis and was found to have severe hypokalemia. A gallium-67 scintigram revealed a positive accumulation in the bilateral salivary glands, and a labial minor salivary gland biopsy demonstrated a massive lymphocyte infiltrate around the salivary ducts. She was diagnosed with Sjögren's syndrome (SS) associated with renal tubular acidosis. Renal biopsy revealed tubulointerstitial nephritis with a mild focal infiltration of lymphocytes and plasma cells. These pathological features were compatible with SS with renal involvement. Acidosis and hypokalemia were corrected with sodium bicarbonate and potassium chloride, which relieved the patient's symptoms. Although steroid therapy has been reported to be effective in SS-associated tubulointerstitial nephritis, the patient's serum potassium level could be controlled without administering steroids during the first admission. Five years later, she was admitted again because of severe liver dysfunction attributed to autoimmune hepatitis. Oral administration of prednisolone resulted in the normalization of her transaminase levels, and the control of her serum potassium level became easier. It has been reported that patients with SS with salivary gland involvement tend to have hepatic complications, and those with hepatic complications tend to have renal involvement. Physicians should be aware of hepatic involvement, even if there is no liver dysfunction at the initial diagnosis of SS with salivary gland and renal involvement. It remains uncertain whether the administration of a low dose of steroids before the onset of autoimmune hepatitis might have prevented the development of liver dysfunction in our patient.

DOI： 10.1272/jnms.2018_85-18

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We report here a case of systemic lupus erythematosus (SLE) with pulmonary hemorrhage and anti-glomerular basement membrane (anti-GBM) antibodies. A 42-year-old woman was admitted to our hospital with complaints of exanthema, arthralgia, shortness of breath, and hemoptysis. Plain chest computed tomography (CT) scan revealed pericardial effusion, bilateral pleural effusions, and pulmonary hemorrhage. Laboratory findings on admission revealed proteinuria, microscopic hematuria, anemia, leukopenia, hypoalbuminemia, hypocomplementemia, and slightly elevated levels of serum creatinine. Serological tests revealed elevated titers of serum anti-GBM antibodies, proteinase 3-antineutrophil cytoplasmic antibodies (PR3-ANCA), and anti-double stranded deoxyribonucleic acid (dsDNA)-immunoglobulin G (IgG) antibodies. Early treatment with steroid pulse therapy combined with plasma exchange resolved the patient's pulmonary hemorrhage and renal dysfunction. Renal biopsy carried out after the treatment revealed a recovery phase of acute tubular injury with minor glomerular abnormalities without linear IgG deposition along the GBMs. For a good prognosis, it is necessary to start treatment immediately in patients with anti-GBM antibody-positive SLE associated with pulmonary hemorrhage.

DOI： 10.1272/jnms.2018_85-21

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BACKGROUND: The physical condition of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated rapidly progressive glomerulonephritis (RPGN) is at times too critical for physicians to undertake a renal biopsy. In such cases, physicians need to start treatments without determining the pathological features of the disease. However, the prevalent clinical practice guidelines for ANCA-associated RPGN in Japan do not necessitate the pathological findings in a renal biopsy for determining first-line treatments. The aim of this study was to assess the correlation between the pathological findings and the clinical guideline-based treatments of ANCA-associated RPGN. METHODS: We investigated clinical and pathological features of patients who were admitted to our hospital to undergo a percutaneous renal biopsy for the diagnosis of ANCA-associated RPGN and were treated per clinical practice guidelines formulated by the Japanese Society of Nephrology. We divided patients into the following three groups according to their treatments: (a) group A, oral intake of prednisolone (PSL) only; (b) group B, methylprednisolone (mPSL) pulse therapy followed by oral intake of PSL; and (c) group C, mPSL pulse therapy followed by oral intake of PSL and the administration of immunosuppressive agents. We compared the crescent formation ratio (CFR) in glomeruli in each treatment group. RESULTS: The median CFR (%; ±standard deviation [SD]) in group A (8.7%±7.7%) was significantly lower than that in groups B (30.2%±15.7%) and C (71.3%±25.5%); group A vs. group B, P<0.05; group A vs. group C, P<0.0001. In addition, the median CFR in group B was significantly lower than that in group C (P<0.01). The median serum creatinine levels (mg/dL; ±SD) in both groups A (2.03±0.96 mg/dL) and B (1.90±0.86 mg/dL) were significantly lower than that in group C (4.30±1.63 mg/dL); group A vs. group C, P<0.01; group B vs. group C, P<0.01. No significant difference was observed in the serum levels of C-reactive protein and ANCA in each treatment group. Although the pathological features of renal biopsy are not included in the factors in the clinical scores described in the guidelines, guideline-based treatments reflected the severity of the crescent formation in each treatment group. CONCLUSIONS: This study suggests that the clinical practice guidelines for ANCA-associated RPGN used in Japan facilitate physicians to determine disease management in patients presenting difficulties in undergoing a renal biopsy.

DOI： 10.1272/jnms.JNMS.2018_85-41

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Language：Japanese   Publisher：臨床体液研究会  

症例は45歳女性で、過睡眠、意識障害で受診し、MRIにて大脳皮質に異常病変を認めた。頭部MRIのFLAIR強調画像で視床、視床下部、脳梁膨大部に高信号領域を認めた。経過などからバソプレシン分泌過剰症(SIADH)による低Na血症を疑った。診断基準に沿って浸透圧や各種ホルモンなどの検査を追加し、診断基準と照合して矛盾しない所見が得られた。MRIにおける中枢性病変と抗AQP4抗体陽性が判明し、SIADHを併発した視神経脊髄炎スペクトラム疾患(NMOsd)と診断した。低Na血症に対する初期治療として高張食塩水の点滴静注を行い、NMOsdの診断後からはステロイドパルス療法を施行した。意識障害は改善したが見当識障害や計算能力の低下などの高次脳機能障害が残存したため、単純血漿交換を施行し、意思疎通が可能な状態にまで回復した。血漿交換後に施行したMRIは入院時と比較して高信号部位の減弱を認めた。

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Language：Japanese   Publisher：(一社)日本透析医学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：日本内科学会-関東地方会  

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Language：Japanese   Publisher：(一社)日本アフェレシス学会  

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Language：Japanese   Publisher：日本医科大学医学会  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J04260&link_issn=&doc_id=20201204310016&doc_link_id=10.1272%2Fmanms.16.222&url=https%3A%2F%2Fdoi.org%2F10.1272%2Fmanms.16.222&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：日本医科大学医学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本透析医学会  

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Language：Japanese   Publisher：(一社)日本内科学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本内科学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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Language：Japanese   Publisher：(一社)日本透析医学会  

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Language：Japanese   Publisher：(一社)日本腎臓学会  

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