Autosomal-dominant tubulointerstitial kidney disease with a novel UMOD mutation, overlapping with Sjogren's syndrome: a case report.

Autosomal-dominant tubulointerstitial kidney disease caused by UMOD (encoding uromodulin) mutation (ADTKD-UMOD) is a rare hereditary disease. A strong family history of hyperuricemia or gout and inherited kidney disease raises the suspicion of ADTKD-UMOD. Genetic testing can confirm the diagnosis without a kidney biopsy.

Assessing fluid volume and determining outcomes of acute heart failure using plasma human atrial natriuretic peptide.

Background: The post-dialysis plasma level of human atrial natriuretic peptide (hANP) reflects the fluid volume in patients on hemodialysis. The threshold hANP level is reportedly 100 pg/mL; however, the clinical usefulness of the threshold hANP level for volume control has not been sufficiently studied.

Methods: We conducted a single-center, retrospective, observational study that included 156 hemodialysis patients without atrial fibrillation.

Aortic Angiosarcoma in Association with Endovascular Aneurysm Repair: Case Report and Review of the Literature.

BACKGROUND Primary aortic sarcoma often poses diagnostic challenges for pathologists and clinicians because of a very low incidence and controversy over nomenclature and definition. We report a case of aortic angiosarcoma in association with a graft. We also conducted a clinicopathological review of cases of primary aortic sarcomas associated with implanted grafts.

An Autopsy Case of Mesenteric Panniculitis with Massive Pleural Effusions.

BACKGROUND Mesenteric panniculitis (MP) is an idiopathic chronic inflammatory condition of the mesentery. The main symptoms include abdominal pain, abdominal distention, weight loss, fever, nausea, and vomiting. The patients also present with chylous ascites in 14% of the cases and chylous pleural effusion (CPE) in very rare occasions.

Kidney morphological parameters measured using noncontrast-enhanced steady-state free precession MRI with spatially selective inversion recovery pulse correlate with eGFR in patients with advanced CKD.

Background: It is well known that atrophic renal changes are associated with chronic kidney disease (CKD) progression, but conventional diagnostic imaging methods such as noncontrast-enhanced computed tomography and magnetic resonance imaging (MRI) have been insufficient for precisely assessing kidney function because they cannot clearly distinguish between the medulla and cortex. Hence, here we used noncontrast-enhanced steady-state free precession (SSFP) MRI with a spatially selective inversion recovery (IR) pulse to improve visibility for renal corticomedullary differentiation and evaluated the association between morphological parameters and kidney function in patients with CKD.

Methods: Kidney corticomedullary contrast ratio, cortical and medullary areas, and minimal cortical thickness of 107 patients with CKD G1-G5 were measured using SSFP MRI with a spatially selective IR pulse and the association between these morphological parameters and kidney function were evaluated.

Comparison of methods of steroid administration combined with tonsillectomy for IgA nephropathy patients.

Background: IgA nephropathy (IgAN) is a chronic glomerular disease that causes end-stage renal disease in 20-40 % of patients within 20 years. The efficacy of tonsillectomy combined with steroid pulse (SP) administration (TSP) for clinical remission of IgAN has been reported. Particularly in Japan, TSP has been performed widely.

Acute tubulointerstitial nephritis with multiple organ involvement including fatal adrenalitis: a case report with autopsy findings.

A 68-year-old woman with Alzheimer's disease developed renal dysfunction after starting carbamazepine for epilepsy. Although Ga-67 citrate scintigraphy strongly suggested interstitial nephritis, renal biopsy was not possible due to her overall state. At 61 days after admission, she died of unexplained shock.

Systemic capillary leak syndrome.

A 40-year-old woman was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein with the presence of monoclonal IgG-lambda. She had had a similar episode of generalized edema 2 years previously.