Internal medicine
-
A 50-year-old man diagnosed with anti-contactin 1 (CNTN1) antibody-associated chronic inflammatory demyelinating polyneuropathy (CIDP) was referred to our department for the evaluation of proteinuria. A kidney biopsy revealed membranous nephropathy (MN). ⋯ Immunofluorescence showed a full-house pattern, and several autoantibodies, such as anti-nuclear antibody, anti-double-strand DNA antibody, and anti-cardiolipin antibody, were detected in the patient's serum. Although limited autoantibodies have been investigated in some of the reported cases, a variety of autoantibodies might be produced in anti-CNTN1 antibody-associated CIDP, accompanied by MN.
-
Review
Transition of Patients with Childhood-onset Rheumatic Diseases to Adult Medical Care in Japan.
Medical advances in childhood-onset chronic diseases (CCDs) have significantly improved the prognosis of these diseases; however, they have also resulted in an increase in the number of cases requiring continued medical care in adulthood. The transition from the pediatric to adult healthcare system has recently received worldwide attention. In Japan, in the last decade, there has been a growing awareness of the transition to adult health care in each specialized chronic disease area with a childhood onset. ⋯ The characteristics of pediatric rheumatic diseases, which are rarer than non-pediatric rheumatic diseases, and the status and problems in transition will be outlined. The mission of pediatricians is not only to follow up diseases but also to support patients' independence. All medical staff and multidisciplinary professionals must cooperate toward this new goal.
-
In 2014, Japan was estimated to have approximately 27 million patients with hypertension (HT), and the ultimate goal of treatment is to prevent complications of HT, including heart failure (HF). The major structural changes in the heart that cause HF are left ventricular (LV) hypertrophy (LVH) and the resulting LV diastolic dysfunction. ⋯ Thus, detecting HF stage B HT and providing appropriate treatment lead to the suppression of HF onset. This review focuses on the detection and treatment of organic heart disease in HT.
-
Review
The Versatile Role of Uromodulin in Renal Homeostasis and its Relevance in Chronic Kidney Disease.
Uromodulin, also known as the Tamm-Horsfall protein, is predominantly expressed in epithelial cells of the kidney. It is secreted mainly in the urine, although small amounts are also found in serum. Uromodulin plays an important role in maintaining renal homeostasis, particularly in salt/water transport mechanisms and is associated with salt-sensitive hypertension. ⋯ Uromodulin has been shown to be associated with the renal function, age, nephron volume, and metabolic abnormalities and has been proposed as a novel biomarker for the tubular function or injury. These findings suggest that uromodulin is a key molecule underlying the mechanisms or therapeutic approaches of chronic kidney disease, particularly nephrosclerosis and diabetic nephropathy, which are causes of end-stage renal disease. This review focuses on the current understanding of the role of uromodulin from a biological, physiological, and pathological standpoint.
-
Case Reports
Late-onset Wilson Disease in a Patient Followed-up for Nonalcoholic Fatty Liver Disease.
A 73-year-old woman was referred to our hospital for persistent liver dysfunction. When the patient was 45 years old, her youngest sister had been diagnosed with Wilson disease (WD). ⋯ A liver biopsy and genetic testing were performed, and she was diagnosed with WD; chelation therapy was then initiated. In patients with hepatic disorders and a family history of WD, multiple medical examinations should be conducted, as the development of WD is possible regardless of age.