CLINICAL CHARACTERISTICS OF ENDOCRINOPATHIES IN CHINESE PATIENTS WITH HEREDITARY HAEMOCHROMATOSIS

BackgroundThis study aimed to cluster newly diagnosed patients and patients with long-term diabetes and to explore the clinical characteristics, risk of diabetes complications, and medication treatment related to each cluster.Research Design and MethodsK-means clustering analysis was performed on 1,060 Chinese patients with type 2 diabetes based on five variables (HbA1c, age at diagnosis, BMI, HOMA2-IR, and HOMA2-B). The clinical features, risk of diabetic complications, and the utilization of elven types of medications agents related to each cluster were evaluated with the chi-square test and the Tukey–Kramer method.ResultsFour replicable clusters were identified, severe insulin-resistant diabetes (SIRD), severe insulin-deficient diabetes (SIDD), mild obesity-related diabetes (MOD), and mild age-related diabetes (MARD). In terms of clinical characteristics, there were significant differences in blood pressure, renal function, and lipids among clusters. Furthermore, individuals in SIRD had the highest prevalence of stages 2 and 3 chronic kidney disease (CKD) (57%) and diabetic peripheral neuropathy (DPN) (67%), while individuals in SIDD had the highest risk of diabetic retinopathy (32%), albuminuria (31%) and lower extremity arterial disease (LEAD) (13%). Additionally, the difference in medication treatment of clusters were observed in metformin (p = 0.012), α-glucosidase inhibitor (AGI) (p = 0.006), dipeptidyl peptidase 4 inhibitor (DPP-4) (p = 0.017), glucagon-like peptide-1 (GLP-1) (p <0.001), insulin (p <0.001), and statins (p = 0.006).ConclusionsThe newly diagnosed patients and patients with long-term diabetes can be consistently clustered into featured clusters. Each cluster had significantly different patient characteristics, risk of diabetic complications, and medication treatment.

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Neural Epidermal Growth Factor–Like 1 Protein–Positive Membranous Nephropathy in Chinese Patients

Clinical Journal of the American Society of Nephrology ◽

10.2215/cjn.11860720 ◽

2021 ◽

pp. CJN.11860720

Author(s):

Guoqin Wang ◽

Lijun Sun ◽

Hongrui Dong ◽

Yanyan Wang ◽

Xiaoyi Xu ◽

...

Keyword(s):

Membranous Nephropathy ◽

Clinical Characteristics ◽

Chinese Patients ◽

Double Positive ◽

Immunohistochemistry Staining ◽

Phospholipase A2 Receptor ◽

Pathologic Features ◽

Glomerular Deposits ◽

Epidermal Growth

Background and objectivesThe neural EGF-like 1 (NELL-1) protein is a novel antigen in primary membranous nephropathy. The prevalence and clinical characteristics of NELL-1–positive membranous nephropathy in Chinese individuals with primary membranous nephropathy are unclear.Design, setting, participants, & measurementsA total of 832 consecutive patients with biopsy-proven primary membranous nephropathy were enrolled. The glomerular expression of phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain-containing 7A (THSD7A) was screened. Glomerular immunohistochemistry staining for NELL-1 was performed in 43 patients with PLA2R- and THSD7A-negative membranous nephropathy, 31 patients with PLA2R-positive membranous nephropathy, and two patients with PLA2R and THSD7A double positivity. The NELL-1 antibody was also detected in the sera of patients with NELL-1–positive membranous nephropathy by western blot. Clinical and pathologic features were comparable between patients with isolated NELL-1–positive, isolated PLA2R/THSD7A-positive, and triple antigen–negative membranous nephropathy.ResultsAmong the 832 patients with primary membranous nephropathy, 11 of 54 (20%) patients with PLA2R-negative membranous nephropathy had THSD7A-positive membranous nephropathy. NELL-1–positive membranous nephropathy accounted for 35% (15 of 43) of all patients with PLA2R- and THSD7A-negative membranous nephropathy. One patient was double positive for NELL-1 and PLA2R in glomerular deposits and positive for only the PLA2R antibody in the serum. Most patients with NELL-1–positive membranous nephropathy were women. No tumors were found. There were significant differences in the prevalence of IgG subtypes between patients with different antigen positivity. Among patients with isolated NELL-1–positive membranous nephropathy, although 80% (12 of 15) were IgG4 staining positive, the proportion of IgG4 dominance was only 67% (ten of 15).ConclusionsAbout one third of patients who were PLA2R and THSD7A negative were NELL-1 positive in Chinese patients with primary membranous nephropathy. NELL-1–positive membranous nephropathy was more common than THSD7A-positive membranous nephropathy in PLA2R-negative membranous nephropathy.

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The Clinical Characteristics and Prognosis of Chinese Patients With Primary Light-Chain Amyloidosis: A Retrospective Multicenter Analysis

10.21203/rs.2.17507/v1 ◽

2019 ◽

Author(s):

Donghua He ◽

Fangshu Guan ◽

Minli Hu ◽

Gaofeng Zheng ◽

Pan Hong ◽

...

Keyword(s):

Prognostic Factors ◽

Light Chain ◽

Clinical Characteristics ◽

Cardiac Involvement ◽

Renal Involvement ◽

Chinese Patients ◽

Response To Treatment ◽

Light Chain Amyloidosis ◽

Hepatic Involvement ◽

Serum And Urine

Abstract Objective To retrospectively identify the critical characteristics and prognostic factors of primary light-chain amyloidosis. Patients and Methods: Data were collected and compared from 91 patients who were diagnosed with primary light-chain amyloidosis at four hospitals between January 2010 and November 2018. We analyzed the clinical characteristics and performed an overall survival (OS) analysis. Results: Patients (median age, 60 years) were diagnosed with organ involvement of the kidney (91.2%), heart (56%), liver (14.3%), soft tissue (18.7%), or gastrointestinal tract (15.4%), and 68.1% of patients had more than two organs involved. Patients were most commonly treated with bortezomib-based regimens (56%), and only one patient had autologous stem cell transplantation (auto-ASCT). The median OS was 36.33 months and was affected by the ECOG score, renal involvement, cardiac involvement, hepatic involvement and negative immunofixation in the serum and urine after treatment. Multivariate analysis indicated that cardiac involvement and negative immunofixation in the serum and urine after treatment were independent prognostic factors for OS. Conclusion: Cardiac involvement and the hematologic response to treatment were independent prognostic factors for OS in primary light-chain amyloidosis patients. The type and number of organs involved is more important than the number of organs involved for the OS.

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