Lipoprotein glomerulopathy with a novel apolipoprotein E variant, APOE Kanto (Asp 151dup).

A 33-year-old Japanese man was admitted for possible kidney disease because of massive proteinuria. Laboratory findings were characterized by marked urinary protein of 4.7 g/day and high-serum triglyceride levels of 266 mg/dL. Renal biopsy revealed segmental proliferation in the mesangium and lipoprotein thrombi in the glomerular capillary. These results suggested that the diagnosis was lipoprotein glomerulopathy (LPG), although serum apolipoprotein E (apo E) levels were within normal ranges. The APOE phenotype was identified as E2/3 by isoelectric focusing polyacrylamide gel electrophoresis. Direct DNA sequencing analyses for apo E identified a duplication of amino acid 151, aspartic acid, and the gene mutation was named APOE Kanto. APOE gene mutations due to amino acid duplication are rare, and this is the first report showing that amino acid duplication among apo E gene mutations is involved in LPG. It is also noteworthy that the patient developed end-stage kidney disease after over than 10 years despite fibrate treatment.

Risk factors for blood vessel rupture during vascular access intervention therapy for hemodialysis patients.

Blood vessel rupture is a major complication associated with vascular access intervention therapy (VAIVT). However, information regarding the risk factors for ruptures related to VAIVT is limited. The purpose of this study was to investigate the risk factors for rupture during VAIVT. This was a single-center, retrospective observational study. Demographic, clinical, anatomical, and VAIVT procedure variables were reviewed and analyzed using multivariate logistic regression. The 211 patients included in the study underwent 628 VAIVT procedures from November 2019 to December 2021, and 20 blood vessel ruptures occurred. Patients with ruptures had significantly lower BMI (p = 0.043), shorter access vintage(p = 0.017), underwent VAIVT for the first time (p = 0.006), and had lower blood flow quantity (p = 0.005), lower brachial artery flow volume (p = 0.018), and higher resistance index (p = 0.011). The multivariate logistic regression revealed that receiving VAIVT for the first time (OR 5.95, 95%CI 1.01-34.84; p = 0.048) and high resistance index (OR 1.86, 95%CI 1.01-3.16; p = 0.02) were significantly associated with a high risk for rupture. Furthermore, receiver operating characteristic curve analysis to assess the sensitivity-specificity profiles of the resistance index for ruptures showed that the optimal threshold was 0.70 (sensitivity/specificity, 0.69/0.70). Heightened surveillance during vascular access intervention therapy is warranted, especially in patients undergoing VAIVT for the first time or patients with a high resistance index (> 0.70).

Aldosterone Response in Severe Hypokalemia and Volume Depletion: A Case Report and Review of the Recent Research.

We report a case of severe hypokalemia and volume depletion complicated by chronic watery diarrhea resulting from chronic alcoholism in a 57-year-old man. Prompt replacement of normal saline with potassium chloride and cessation of alcohol intake resulted in a favorable outcome. We discuss the pathophysiology of the case, emphasizing the response of aldosterone in both hypokalemia and volume depletion, and provide a review of recent research.

Recurrent solitary fibrous tumor of the pleura with malignant transformation and non-islet cell tumor-induced hypoglycemia due to paraneoplastic overexpression and secretion of high-molecular-weight insulin-like growth factor II.

A 41-year-old man was diagnosed with a solitary fibrous tumor (SFT) of the pleura in the posterior mediastinum. Despite two surgeries for excision, the SFT recurred and progressed with direct invasion of the chest wall and bone metastases. He was hospitalized because of cerebral infarction and presented with recurrent severe hypoglycemia fourteen years later. High-molecular-weight (HMW) insulin-like growth factor II (IGF-II) was identified in the serum and tumor using Western blotting and immunohistochemistry. These findings suggested that the cause of the recurrent severe hypoglycemia was SFT production of HMW IGF-II, a mediator of non-islet cell tumor-induced hypoglycemia (NICTH).

Granulomatous interstitial nephritis in a renal allograft.

We describe a middle-aged woman in whom granulomatous interstitial nephritis (GIN) developed in a renal allograft. She had undergone bowel resection due to an uncertain diagnosis of active granulomatous bowel disease 30 years earlier. Thereafter, frequent hyperoxaluria as well as calcium oxalate stone and recurrent urinary tract infections had resulted in a progressive deterioration in kidney function over a period of 20 years. She underwent living donor kidney transplantation; however, her kidney function progressively deteriorated, despite transplantation. A biopsy of the renal allograft revealed GIN with granulomatous vasculitis accompanied by calcium oxalate crystals. These as well as the laboratory findings indicated a diagnosis of sarcoidosis. We considered that the aggravated granulomatous inflammation on the allograft was caused by recurrent sarcoidosis accompanied by hyperoxaluria.