Two Brothers in One Chinese Family With Collagen Type III Glomerulopathy

Chen, Nan; Pan, Xiaoxia; Xu, Yu; Wang, Zhaohui; Sheng, Hao; Yan, Fuhong; Dong, Xiaobei

doi:10.1053/j.ajkd.2007.08.021

Cited by 5 publications

(7 citation statements)

References 16 publications

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“…Persistent proteinuria is the most common presentation of collagen type III glomerulopathy. Among those with proteinuria, 30% of the Chinese patients had nephrotic syndrome, while 60% of patients fell into the nephritic range in the English literature (16). Hypertension was also prominent (in 75% of cases), which is similar to the results in the English literature (reported in two-thirds of all cases) (16).…”

Section: Discussionsupporting

confidence: 79%

“…Some consider it to be a systemic disease (9,24), while others consider it a primary renal disease (2,3). In China, procollagen type III peptide increased notably in the cases reported by Chen et al (16) and cases with coronary heart disease, thyroid tumor, gallbladder polyps or multiple swollen lymph nodes in the mediastinum have been reported (21); however, extra-renal type III collagen fibril deposition was not identified in any of the cases reported. The pathological manifestation of collagen type III glomerulopathy is significant (25).…”

Section: Discussionmentioning

confidence: 99%

“…Among those with proteinuria, 30% of the Chinese patients had nephrotic syndrome, while 60% of patients fell into the nephritic range in the English literature (16). Hypertension was also prominent (in 75% of cases), which is similar to the results in the English literature (reported in two-thirds of all cases) (16). Forty-five percent of patients in China presented with elevated creatinine levels, while renal function was usually normal or slightly decreased at presentation in previous studies (6).…”

Section: Discussionmentioning

confidence: 99%

“…In English publications, cases have occasionally been described within families (8,22,23); hence, it has been assumed that collagen type III glomerulopathy may have an autosomal recessive trait (24). In Chinese literature, cases within the same family were also noted (16). However, the pathogenesis of collagen type III glomerulopathy remains unclear.…”

Section: Discussionmentioning

confidence: 99%

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Collagen type III glomerulopathy: A case report and review of 20 cases

Dong

Wei

Han

et al. 2015

Experimental and Therapeutic Medicine

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Abstract. Collagen type III glomer ulopathy is a non-immune-mediated glomerular disease, characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space. The clinical manifestations of this disease are proteinuria, peripheral edema, hypertension and occasional progression to end-stage renal disease. Collagen type III glomerulopathy is extremely rare, and its etiology and pathogenesis remain elusive. To date, only case reports are available and the majority of these are from Japan. To investigate the idiographic features of collagen type III glomerulopathy in China, we report a case of collagen type III glomerulopathy with two differing renal biopsies and review 20 cases in China. The majority of the Chinese patients were adults. Thirty percent of the patients had nephrotic syndrome, and hypertension was observed in 75% of cases. Elevated creatinine was present in 45% of patients. The pathology of collagen type III glomerulopathy in the Chinese cases was similar to that observed in other ethnicities, although certain cases were IgA-positive by immunofluorescence microscopy, and electron-dense material could be observed in the mesangial area. The onset age, clinical manifestations and pathological features of the disease are not exactly the same in China as worldwide.

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Section: Discussionsupporting

confidence: 79%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Collagen type III glomerulopathy: A case report and review of 20 cases

Dong

Wei

Han

et al. 2015

Experimental and Therapeutic Medicine

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“…A summary of the previously published cases was compiled by Alchi et al . Thus far, five and 40 cases have been reported in China and in other countries, respectively . In total, the male:female ratios were 6:8 in China (including the patients presented in this paper) and 19:21 in other countries ( P = 0.764).…”

Section: Resultsmentioning

confidence: 65%

Clinical and morphological features of collagen type III glomerulopathy: a report of nine cases from a single institution

et al. 2015

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Two brothers with collagenofibrotic glomerulopathy

et al. 2014

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Collagenofibrotic glomerulopathy is a rare glomerular disease characterized by extensive accumulation of atypical type III collagen fibers within the mesangial matrix and subendothelial space. Laboratory evaluation of this disease shows a marked increase in serum procollagen III peptide (P III P) levels. Here, we report the case of two brothers with collagenofibrotic glomerulopathy confirmed by histology. Patient 1 presented with proteinuria and hypertension and patient 2 presented with nephrotic-range proteinuria. Immunohistochemistry revealed strong staining for antibodies to type III collagen in the widened subendothelial spaces in both patients. Electron microscopy revealed numerous collagenous fibers in the mesangium and subendothelial space. P III P levels were elevated in both patients. Most reported cases of collagenofibrotic glomerulopathy, including the adult-onset type, have been sporadic. Within the limits of our literature search, this is only the third report of adult siblings with collagenofibrotic glomerulopathy confirmed by histology. This report indicates that it may be beneficial to measure serum P III P levels in the siblings of patients diagnosed with adult-onset collagenofibrotic glomerulopathy.

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Two Brothers in One Chinese Family With Collagen Type III Glomerulopathy

Cited by 5 publications

References 16 publications

Collagen type III glomerulopathy: A case report and review of 20 cases

Collagen type III glomerulopathy: A case report and review of 20 cases

Clinical and morphological features of collagen type III glomerulopathy: a report of nine cases from a single institution

Two brothers with collagenofibrotic glomerulopathy

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