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| 1.
論文（リポジトリ） |
論文（リポジトリ）
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Furuichi, Kengo ; Shimizu, Miho ; Yamanouchi, Masayuki ; Hoshino, Junichi ; Sakai, Norihiko ; Iwata, Yasunori ; Toyama, Tadashi ; Kitajima, Shinji ; Hara, Akinori ; Yuzawa, Yukio ; Kitamura, Hiroshi ; Suzuki, Yoshiki ; Sato, Hiroshi ; Uesugi, Noriko ; Ueda, Yoshihiko ; Nishi, Shinichi ; Nishino, Tomoya ; Samejima, Kenichi ; Kohagura, Kentaro ; Shibagaki, Yugo ; Makino, Hirofumi ; Matsuo, Seiichi ; Ubara, Yoshifumi ; Yokoyama, Hitoshi ; Wada, Takashi
概要:
Introduction The speed of declining kidney function differs among patients with diabetic nephropathy. This study was und
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ertaken to clarify clinical and pathological features that affect the speed of declining kidney function in patients with diabetic nephropathy.\nResearch design and methods This study was design as multicenter retrospective study. The subjects (377 patients with diabetic nephropathy diagnosed by kidney biopsy at 13 centers in Japan) were classified into three groups based on the estimated glomerular filtration rate (eGFR) declining speed. The eGFR increasing group, the control group, and the eGFR declining group were divided at 0 and 5 mL/min/1.73 m2/year, respectively. Characteristics of clinicopathological findings of declining kidney function were evaluated.\nResults The mean observation period of this study was 6.9 years. The control group, the eGFR increasing group, and the eGFR declining group included 81, 66, and 230 patients, respectively. The incidences of composite kidney events represented by 100 persons/year were 25.8 in the eGFR declining group and 2.0 in the eGFR increasing group. After adjustment for age, sex, systolic blood pressure, hemoglobin, and urinary albumin levels, three clinicopathological findings (urinary albumin levels, presence of nodular lesion, and mesangiolysis) were risk factors for inclusion in the eGFR declining group (the ORs were 1.49, 2.18, and 2.08, respectively). In contrast, the presence of subendothelial space widening and polar vasculosis were characteristic findings for inclusion in the eGFR increasing group (the ORs were 0.53 and 0.41, respectively).\nConclusions As well as urinary albumin elevation, nodular lesion and mesangiolysis were characteristic pathological features of patients with fast declining kidney function.
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| 2.
論文（リポジトリ） |
論文（リポジトリ）
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Imafuku, Aya ; Nozu, Kandai ; Sawa, Naoki ; Nakanishi, Koichi ; Ubara, Yoshifumi
概要:
Both thin basement membrane nephropathy (TBMN) and autosomal dominant Alport syndrome (ADAS) are types of hereditary nep
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hritis resulting from heterozygous mutations in COL4A3 or COL4A4 genes. Although TBMN is characterized by hematuria and thinning of the glomerular basement membrane (GBM) with excellent renal prognosis, some patients develop end-stage renal disease (ESRD) later in life. In contrast, although AS is characterized by progressive nephropathy with lamellation of the GBM, there are some patients diagnosed with ADAS from a family history of ESRD but who only suffer from hematuria with GBM thinning. These findings indicate a limitation in distinction between TBMN and ADAS. Diagnosis of AS is significant because it facilitates careful follow-up and early treatment, whereas diagnosis of TBMN can underestimate the risk of ESRD. However, some experts are against using the term ADAS as the phenotypes of heterozygous variants vary from no urinary abnormality to ESRD, even between family members with the same mutations, indicating that unknown secondary factors may play a large role in the disease severity. These diagnostic difficulties result in significant confusion in clinical settings. Moreover, recent studies revealed that the number of patients with chronic kidney disease caused by these gene mutations is far higher than previously thought. The aim of this article is to review differing opinions regarding the diagnosis of heterozygous COL4A3 or COL4A4 variants, and to highlight the importance for nephrologists to recognize this disease, and the importance of the need to reclassify this disease to minimize the current confusion.
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