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Background Thrombotic thrombocytopenic purpura (TTP) is rare and severe thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and renal dysfunction. In contrast, essential thrombocythemia (ET) is a myeloproliferative disease associated with an abnormal increase in platelet numbers. Previous studies reported several cases of the development of ET in patients with TTP. However, the case of an ET patient complicated with TTP has not been previously reported. In this case study, we present a patient with TTP who was previously diagnosed with ET. Therefore, to the best of our knowledge, this is the first report of TTP in ET. Case presentation A 31-year-old Chinese female who was previously diagnosed with ET presented with anemia and renal dysfunction. The patient had been on long-term treatment with hydroxyurea, aspirin, and alpha interferon (INF-α) for ten years. The diagnosis of TTP was confirmed by clinical features, schistocytes noted on the peripheral blood smear, and lower ADAMTS13 activity (8.5%), together with the renal biopsy results. INF-α was discontinued, and the patient was then treated with plasma exchange and corticosteroids. After one year of follow-up, the patient had a normal hemoglobin level and platelet numbers, and her ADAMTS13 activity had improved. However, the patient’s renal function remains impaired. Conclusions We report a case of an ET patient complicated with TTP that was possibly due to INF-α, highlighting the potential complications associated with long-term ET therapy. The case also highlights the importance of considering TTP in patients with pre-existing ET who present with anemia and renal dysfunction, extending the spectrum of known studies.

Objective To investigate the relationship between serum uric acid (SUA) level and renal outcome in patients with type 2 diabetes mellitus (T2DM) and diabetic nephropathy (DN). Methods A total of 393 Chinese patients with T2DM and biopsy-proven DN and followed at least 1 year were enrolled in this study. Patients were stratified by the quartiles of baseline level of SUA: Q1 group: 286.02 ± 46.66 μmol/L (n = 98); Q2 group: 358.23 ± 14.03 μmol/L (n = 99); Q3 group: 405.50 ± 14.59 μmol/L (n = 98) and Q4 group: 499.14 ± 56.97μmol/L (n = 98). Renal outcome was defined by progression to end-stage renal disease (ESRD). Kaplan–Meier survival analysis and Cox proportional hazards model were used to analyze the association between SUA quartiles and the renal outcomes. Results During the median 3-year follow-up period, there were 173 ESRD outcome events (44.02%). No significant difference between SUA level and the risk of progression of DN (P = 0.747) was shown in the Kaplan–Meier survival analysis. In multivariable-adjusted model, hazard ratios for developing ESRD were 1.364 (0.621–2.992; P = 0.439), 1.518 (0.768–3.002; P = 0.230) and 1.411 (0.706–2.821; P = 0.330) for the Q2, Q3 and Q4, respectively, in comparison with the Q1 (P = 0.652). Conclusions No significant association between SUA level and renal outcome of ESRD in Chinese patients with T2DM and DN was found in our study. Besides, the role of uric acid-lowering therapy in delaying DN progression and improving ESRD outcome had not yet been proven. Further study was needed to clarify the renal benefit of the uric acid-lowering therapy in the treatment of DN.

Purpose: This study aimed to investigate the effects of intravitreal (IVT) VEGFi on long-term renal outcomes in patients with biopsy-proven diabetic kidney disease (DKD).Patients and methods: Patients prescribed IVT VEGFi (VEGFi group) were enrolled from a retrospective cohort with biopsy-proven DKD, and those not prescribed VEGFi (non-VEGFi group) were enrolled by 1:3 propensity score matching, adjusted for clinical and pathological baseline indicators. The primary endpoint is defined as end-stage renal disease (ESRD) and the secondary endpoint is defined as all-cause mortality.Results: Compared with patients in non-VEGFi group, patients with VEGFi had a higher proportion of diabetic retinopathy (DR) (50.9% vs 100%, p < 0.001) before matching. Standardized mean difference (SMD) of age, DR, duration of diabetes, the proportion of hypertension, eGFR, initial proteinuria, serum albumin, hemoglobin, the proportion of RAAS inhibitor and interstitial fibrosis and tubular atrophy (IFTA) were >10%. After matching, there was no significant difference in clinical pathology between the two groups. Except for the proportion of hypertension, the SMD of other indicators was p = 0.772) and all-cause mortality (Log-Rank p = 0.834) were not significantly different between the two groups.Conclusion: Our data suggested that IVT VEGFi did not increase the incidence of ESRD and all-cause mortality in patients with DKD.

Chunmei Qin,1– 3 Yiting Wang,1,2 Lijun Zhao,1,2 Junlin Zhang,1,2 Honghong Ren,1,2 Yutong Zou,1,2 Yucheng Wu,1,2 Tingli Wang,1,2 Yuancheng Zhao,1,2 Rui Zhang,1,2 Huan Xu,4 Jie Zhang,5 Mark E Cooper,6 Fang Liu1,2 1Division of Nephrology, West China Hospital of Sichuan University, Chengdu, People’s Republic of China; 2Laboratory of Diabetic Kidney Disease, Centre of Diabetes and Metabolism Research, West China Hospital of Sichuan University, Chengdu, People’s Republic of China; 3Department of Nephrology, Luzhou People’s Hospital, Luzhou, People’s Republic of China; 4Division of Pathology, West China Hospital of Sichuan University, Chengdu, People’s Republic of China; 5Key Laboratory of Transplant Engineering and Immunology, Chengdu, People’s Republic of China; 6Department of Diabetes, Central Clinical School, Monash University, Melbourne, AustraliaCorrespondence: Fang Liu, Division of Nephrology, West China Hospital of Sichuan University, No. 37, Guoxue Alley, Chengdu, Sichuan, 610041, People’s Republic of China, Tel +86-28-18980601214, Fax +86-28-85422335, Email liufangfh@163.comObjective: The rate of kidney function decline in patients with diabetic kidney disease (DKD) is known to differ. This study analyzed the clinicopathologic features and related risk factors affecting long-term renal survival in Chinese type 2 diabetic patients with rapid estimated glomerular filtration rate (eGFR) decline.Methods: In this retrospective descriptive study, 191 DKD patients were first classified as rapid eGFR decliners and slow eGFR decliners on the basis of the median eGFR slope value (− 8.0 mL/min/1.73 m2/year). In total, 96 patients with rapid eGFR decline were included in the analyses and subsequently allocated to end-stage renal disease (ESRD) and non-ESRD groups. Baseline clinicopathological data of rapid eGFR decliners were collected. Cox proportional hazard analysis was performed to calculate the hazard ratios (HRs) for progression to ESRD.Results: During a median follow-up of 25 months, 52 (54.2%) rapid eGFR decliners progressed to ESRD. These 52 rapid eGFR decliners had poorer renal function, lower hemoglobin and albumin concentrations, higher total cholesterol and baseline proteinuria levels, and more severe interstitial inflammation than those who did not progress to ESRD. After adjustment for age, gender, baseline eGFR, proteinuria, hemoglobin level, serum albumin concentration, and histopathologic parameters, multivariate Cox proportional hazard analysis revealed that eGFR (HR 0.973, 95% CI 0.956– 0.989) and proteinuria (HR 1.125, 95% CI 1.030– 1.228) were associated with the increased risk of progression to ESRD.Conclusion: Higher proteinuria and lower eGFR were independent risk factors for renal progression in Chinese patients with type 2 diabetes and rapid eGFR decline.Keywords: diabetic kidney disease, rapid eGFR decline, clinical, pathological, type 2 diabetes

Several studies show that patients with early-onset diabetes have higher risk of diabetic complications than those diagnosed in middle age. However, whether early-onset of type 2 diabetes mellitus (T2DM) is a risk factor for diabetic nephropathy (DN) progression remains unclear, especially a lack of data in biopsy-confirmed cohort. In This study, we enrolled 257 patients with T2DM and biopsy-confirmed DN to investigate the role of early-onset T2DM in DN progression. Participants were divided into two groups according to the age of T2DM diagnosis: early-onset group (less than 40 years) and later-onset group (40 years or older). We found that patients with early-onset T2DM had higher glomerular grades and arteriolar hyalinosis scores than those in later-onset group. After adjusted for confounding factors, early-onset of T2DM remained an independent predictor of end-stage renal disease (ESRD) for patients with DN. In conclusion, although with the comparable renal function and proteinuria, patients with early-onset T2DM and DN had worse renal pathological changes than those with later-onset. Early-onset of T2DM might be an important predictor of ESRD for patients with DN, which called more attention to early supervision and prevention for patients with early-onset T2DM and DN.

Diabetic kidney disease (DKD) is the most common cause of end-stage renal disease (ESRD) and is associated with increased morbidity and mortality in patients with diabetes. Identification of risk factors involved in the progression of DKD to ESRD is expected to result in early detection and appropriate intervention and improve prognosis. Therefore, this study aimed to establish a risk prediction model for ESRD resulting from DKD in patients with type 2 diabetes mellitus (T2DM).Between January 2008 and July 2019, a total of 390 Chinese patients with T2DM and DKD confirmed by percutaneous renal biopsy were enrolled and followed up for at least 1 year. Four machine learning algorithms (gradient boosting machine, support vector machine, logistic regression, and random forest (RF)) were used to identify the critical clinical and pathological features and to build a risk prediction model for ESRD.There were 158 renal outcome events (ESRD) (40.51%) during the 3-year median follow up. The RF algorithm showed the best performance at predicting progression to ESRD, showing the highest AUC (0.90) and ACC (82.65%). The RF algorithm identified five major factors: Cystatin-C, serum albumin (sAlb), hemoglobin (Hb), 24-hour urine urinary total protein, and estimated glomerular filtration rate. A nomogram according to the aforementioned five predictive factors was constructed to predict the incidence of ESRD.Machine learning algorithms can efficiently predict the incident ESRD in DKD participants. Compared with the previous models, the importance of sAlb and Hb were highlighted in the current model.Highlights

The accuracy of reverse transcription quantitative real-time PCR (RT-qPCR) is strongly depended on the stability of reference gene. Callerya speciosa, as a traditionally Chinese medicine, has a long cultivation history in south China. It is essential to select the suitable reference gene to obtain reliable RT-qPCR results when gene expression changes were evaluated. However, suitable reference genes in C. speciosa have not yet been investigated for accurate gene expression quantification under different experimental conditions. In this study, eight candidate reference genes (GAPDH, 60S, ACTIN, TUA2, TUB1, TIF5, UBQ, EF2) were selected from the transcriptome databases, and their expression stability under six experimental conditions (developmental stages, tissues, MeJA treatment, GA3 treatment, CPPU and PP333 treatment) was evaluated using ΔCT, geNorm, NormFinder, BestKeeper, RefFinder programs. The results showed that GAPDH was the optimal reference gene for all different experimental conditions, whereas ACTIN showed the most stability under the hormone treatments in C. speciosa. GAPDH and EF2 were proved to be the most stable genes for developmental stages, while different genes (GAPDH and TUB1) were stable reference genes for tissues. For treatments, ACTIN was identified as the most stable gene under most of hormone treatments. TUBI and ACTIN were at the beginning of the ranking order in PP333 treatment, while GAPDH and ACTIN were adequate for normalization in MeJA and GA3 treatments. TUBI and GAPDH were the most stable genes for CPPU treatment, while ACTIN was proved to be the most stable gene for three different treatments (MeJA, GA3 and PP333). Validation of reference genes was carried out by the target gene CsMYB36, which further confirmed their reliability. These results provided a theoretical basis for subsequent research on the regulation of functional gene expression in C. speciosa.

The purpose of this report is to determine the clinicopathological and immuno-phenotypical characteristics of myoid hamartoma of the breast (MHB). The clinical data, histological morphology, and immunohistochemical results of 2 patients diagnosed with MHB were analyzed, and 15 cases of MHB reported in China were reviewed. Both patients were female, aged 28 and 35 years old, and their lesions were located in the upper outer quadrant of the left breast and the right breast respectively. The lesions measured 3 cm × 3 cm × 2.5 cm and 6.5 cm × 6 cm × 4.5 cm. A gross examination indicated a grayish solid block with clear boundaries. A microscopic examination showed different proportions of ducts and acini, beam myoid cells, adipose tissue, and fibrous stroma. The myoid cell bundles of both specimens were positive for vimentin, SMA, h-caldesmon and desmin, but negative for ER, PR, PCK, s-100, Calponin, and P63. Both cases were confirmed as MHB based on their clinical, histological and immuno-phenotypical characteristics. Our findings provide further insights into the pathological basis of MHB, which can help avoid both misdiagnosis and missed diagnosis.