We report a rare case of aortic valve thrombosis in a 55-year-old man. He had reduced urine output and generalized edema four days before admission. He felt sick and had chest pain on the day of admission. Severe hypoalbuminemia and massive proteinuria were present, and a diagnosis of nephrotic syndrome was made. An electrocardiogram showed findings suspicious for acute myocardial infarction, and emergency catheterization was performed. However, no coronary artery stenosis or occlusion was found at that time. A subsequent contrast-enhanced CT scan and transesophageal echocardiography revealed a thrombus in the aortic valve. The thrombus nearly occluded the left coronary artery. Emergency surgery was performed to remove the thrombus. His postoperative course was stable, but nephrotic syndrome persisted. A renal biopsy diagnosed minimal change disease. Treatment with 50 mg/day of prednisolone induced remission, and he was discharged on day 43. There were no aortic valve abnormalities. Nephrotic syndrome was considered the main contributor to thrombus formation. Aortic valve thrombosis associated with nephrotic syndrome is a very rare but potentially fatal condition that requires attention.

We herein report a case of IgA nephropathy in a 20-year-old male who maintained a complete remission of minimal change nephrotic syndrome (MCNS) through the administration of rituximab (RTX). He was diagnosed with nephrotic syndrome at 4 years of age. After he relapsed frequently, he was diagnosed with MCNS at 8 years of age based on the findings of a kidney biopsy. At 13 years of age, RTX therapy was initiated to maintain a complete remission after steroid treatment. MCNS recurred twice, including the time in which the interval between the RTX administrations was long. Whenever he relapsed, remission induction was achieved using steroids, and remission maintenance was achieved using RTX. Five months after the 7th RTX administration, the serum IgA level started to increase. After the 9th RTX administration, he demonstrated microhematuria despite the urinary protein level indicating complete remission. At the 10th administration, the urinary protein and the red-blood cell casts were also observed. A renal biopsy was performed 84 months after the initial administration of RTX, and the patient was diagnosed with complications of IgA nephropathy. RTX is not considered to be a useful treatment for IgA nephropathy. The reasons for this are due to the fact that IgA1 does not decrease even following the administration of RTX, because B cells residing in the mucosa may not be deleted by RTX, and IgA production may also continue due to the presence of CD20- long-lived plasma cells. Even when administering RTX, if there are findings of glomerulonephritis on urine testing, the possibility of IgA nephropathy must be considered.

BACKGROUND: Minimal change nephrotic syndrome (MCNS) can be complicated by thymoma; however, no standard therapy for thymoma-associated MCNS has yet been established. We herein describe a case of steroid-resistant MCNS associated with thymoma, treated effectively with rituximab.
METHODS: A 71-year-old Japanese man was referred to our department with severe proteinuria (20 g/gCr). Renal biopsy showed minimal change disease and computed tomography revealed an anterior mediastinal mass. Based on these findings, he was diagnosed with thymoma-associated MCNS. He was treated with oral prednisolone (50 mg/day) and cyclosporine, and underwent thymectomy and plasma exchange. However, no improvement in proteinuria was observed. He therefore received intravenous rituximab 500 mg, resulting in a marked decrease in proteinuria from 5328 to 336 mg/day after 1 week.
CONCLUSIONS: This case suggests that rituximab might be an effective therapy in patients with steroid-resistant MCNS associated with thymoma.

We report a case of a sudden onset of minimal change nephrotic syndrome (MCNS) in a 33-year-old woman with type 1 diabetes mellitus (T1DM), stable microalbuminuria, and chronic thyroiditis. She was successfully treated with intravenous corticosteroids to finally attain a complete remission. Four years later, she also experienced a relapse of MCNS in the same season as the first onset. The chronological levels of serum immunoglobulin E (IgE) showed that extremely high serum IgE levels preceded the onset or the relapse of MCNS, which may suggest an allergic mechanism of MCNS. Eicosapentaenoic acid (EPA) was reported to be beneficial in treating allergic diseases. Suplatast tosilate is an anti-allergic medication that suppresses serum IgE and was reported to be beneficial in reducing corticosteroid dose in nephrotic syndrome in a pilot study. Therefore, during the tapering of corticosteroids to the relapse of MCNS, suplatast tosilate and EPA were administered, and the IgE levels were considerably controlled. The patient was able to maintain remission even after the cessation of corticosteroids. In conclusion, suppressing IgE levels using suplatast tosilate and EPA may be beneficial in maintaining complete remission without corticosteroids in T1DM.

The patient was a 64-year-old man who had been receiving methotrexate (MTX) for rheumatoid arthritis for 8 years. Computed tomography (CT) obtained one month prior to admission revealed numerous enlarged lymph nodes. Lower leg edema appeared two weeks prior to admission. Severe proteinuria was confirmed, and the patient was admitted. A renal biopsy revealed minimal changes in glomeruli. CT on day 14 revealed shrinking lymph nodes, and the patient was diagnosed with Hodgkin lymphoma by a neck lymph node biopsy. This is the first report of secondary minimal change nephrotic syndrome caused by an MTX-associated lymphoproliferative disorder.

UNASSIGNED: Minimal change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS) are the two common causes of nephrotic syndrome (NS) in both children and adults with overlapping clinical features, but with distinct prognostic and therapeutic implications. The distinction between these relies entirely on histopathology, which can sometimes be difficult. CD44 is expressed by activated parietal epithelial cells, plays a role in matrix deposition and thus in the pathogenesis of FSGS.
UNASSIGNED: To assess the expression of CD44 in MCNS and FSGS and to evaluate its association with the known clinical and histopathological prognostic factors.
UNASSIGNED: Thirty cases each of MCNS and FSGS were studied. The clinical, laboratory, histopathological, and CD 44 immunohistochemical data were recorded. The findings were analyzed and correlated. A P value of < 0.05 was considered statistically significant.
UNASSIGNED: Statistical association was noted between CD44 positivity and serum creatinine (p = 0.031), estimated glomerular filtration rate (p = 0.040), segmental sclerosis (p < 0.001), tubular atrophy (p = 0.027), interstitial fibrosis (p = 0.027), and histological diagnosis (p < 0.001). The sensitivity, specificity, positive predictive, and negative predictive values were 90%, 76.67%, 79.41% and 88.46%, respectively.
UNASSIGNED: CD44 immunostain can effectively distinguish MCNS from FSGS. The congruent results of CD44 positivity with known prognostic factors support the possibility of using the CD44 marker as a predictive tool in selecting high-risk patients and offering appropriate therapeutic measures.

Kimura disease (eosinophilic granuloma of the soft tissue) is a benign granulomatous disease complicated by nephrotic syndrome. Herein, we report a case of recurrent minimal change nephrotic syndrome (MCNS) complicated by Kimura disease that was successfully treated with rituximab. A 57-year-old man presented to our hospital with relapsed nephrotic syndrome with worsening swelling of the right anterior ear and elevated serum IgE. MCNS was diagnosed on renal biopsy. Treatment with 50 mg of prednisolone rapidly placed the patient in remission. Therefore, RTX 375 mg/m2 was added to the treatment regimen, and steroid therapy was tapered. Early steroid tapering was successful, and the patient is currently in remission. In this case, the nephrotic syndrome flare-up was accompanied by worsening Kimura disease. Rituximab reduced the worsening of symptoms related to Kimura disease, including head and neck lymphadenopathy and elevated IgE levels. Kimura disease and MCNS may share a common IgE-mediated type I allergic condition. Rituximab effectively treats these conditions. In addition, rituximab suppresses Kimura disease activity in patients with MCNS, enables early tapering of steroids, and reduces the total dose of steroids.

A 64-year-old man visited the outpatient department of our hospital for the first time due to bilateral lower limb edema, which he noticed 1 week before the visit. Pain suddenly developed in the left lower limb while the patient was in the waiting room. Nephrotic syndrome was suspected based on blood and urine test results. Acute arterial thromboembolism in the left lower limb associated with hypercoagulation due to nephrotic syndrome was suspected, and a diagnosis was made using computed tomography angiography. Arterial thrombectomy was urgently performed, and the limb was salvaged without sequelae. Based on renal biopsy, minimal change nephrotic syndrome was diagnosed, and the patient underwent remission induction with steroid therapy. Heparin was drip infused and apixaban was orally administered to prevent recurrent thrombosis. Nephrotic syndrome in the acute phase is often complicated by thrombosis. Particularly, arterial thromboembolism requires prompt treatment, and prophylactic anticoagulation therapy needs to be considered.

BACKGROUND: Patients with idiopathic minimal change nephrotic syndrome (MCNS) undergoing immunosuppressive therapy are susceptible to infectious complications. Study specifically focusing on adult population\'s infectious complications is lacking.
METHODS: We retrospectively collected 101 adult patients with biopsy-proven idiopathic MCNS and analyzed for the infectious complications. Published literatures were also reviewed aiming to evaluate the feasibility of prophylactic antibiotic treatment.
RESULTS: Infectious complications developed in 17 of 101 (16.8%) patients, with pneumonia (n=4), cellulitis/fasciitis (n=4) and urinary tract infection (UTI) (n=4) being the dominant diseases, and Gram-negative bacilli the main cause. AKI stage ≥2 (Hazard ratio=6.1; 95% CI: 1.2-31.9, p=0.031) and non-remission by treatment (Hazard ratio=4.4; 95% CI: 1.2-15.6, p=0.023) were the two independent risk factors relevant to developing infectious complications. Review of 16 published literatures and our data showed that even no prophylactic antibiotic therapy, only one case of Pneumocystis jirovecii pneumonia developed among the 1,787 accumulative cases of MCNS. In contrast, 16 (44%) of acute flare cases were reported among the 36 patients with positive hepatitis B surface antigen that did not receive antiviral prophylactic therapy.
CONCLUSIONS: Advanced acute kidney injury and non-remission by treatment are the risk factors toward developing infectious complications in adult MCNS undergoing immunosuppressive therapy. It appears unnecessary to use prophylactic antibiotic for Pneumocystis jirovecii pneumonia or other bacterial infections, while screening and prophylactic therapy for hepatitis B and latent tuberculosis are critical for patients in prevalent area. This article is protected by copyright. All rights reserved.

Patients with minimal change nephrotic syndrome (MCNS) are prone to loss of motor skills due to urinary protein leakage, steroid myopathy, and other factors. Acute kidney injury (AKI) is a common complication that contributes to the loss of physical function. Rehabilitation is crucial, but its efficacy and safety are unknown. Here we present a case of a patient with MCNS complicated by AKI, who commenced rehabilitation after dialysis was discontinued and experienced improved mobility. The patient, a woman in her 70s, was admitted to our hospital with bilateral lower limb edema and decreased urine output for approximately 5 days. Treatment with prednisolone and furosemide was initiated, but then dialysis was initiated due to AKI. Rehabilitation was started after dialysis was discontinued. The patient\'s muscle strength and physical activity improved, and her exercise capacity and exercise tolerance improved without adverse effects. Rehabilitation may contribute to the improvement of exercise capacity without worsening renal function and urinary protein in patients with MCNS complicated by AKI.