Objective:To investigate the correlation between enhanced CT features and clinicopathological features of renal clear cell carcinoma.Methods:The clinicopathological data of 33 patients with renal clear cell carcinoma after radical operation in the Third People′s Hospital of Huzhou from January 2015 to January 2019 were analyzed retrospectively.The relationship between enhanced CT signs and pathological features was analyzed by speraman correlation analysis.Results:The pathological findings showed that there were 17 cases of capsule invasion and 16 cases of no capsule invasion, and there were statistically significant differences in lesion diameter, CT enhancement difference and enhancement rate between the two groups[(7.74±1.85)cm vs.(4.15±1.41)cm, (30.19±10.00)HU vs.(48.25±24.12)HU, (70.7±22.6)% vs.(137.0±86.6)%, t=-6.239, 2.841, 3.050, P=0.000, 0.008, 0.005]. There were statistically significant differences in lesion diameter and enhancement rate between patients with vascular tumor thrombus (12 cases) and without vascular tumor thrombus(21 cases)[(7.86±2.35)cm vs.(4.93±1.79)cm, (70.0±19.9)% vs.(121.0±81.7)%, t=-4.025, 2.074, P=0.000, 0.046], but there was no statistically significant difference in CT enhancement difference[(30.78±11.71)HU vs.(43.62±22.63)HU, t=1.823, P=0.078]. Spearman correlation analysis showed that there was a moderate negative correlation between CT enhancement difference and capsule invasion and vascular carcinoma thrombus( r=-0.593, -0.540, P=0.000, 0.001). The diameter of the lesion was positively correlated with capsule invasion( r=0.781, P=0.000). Conclusion:The diameter, CT enhancement difference and enhancement rate of renal clear cell carcinoma are different.The larger the diameter of the lesion and the smaller the enhancement difference indicate the capsule invasion and vascular carcinoma thrombus.

Objective:Digestive endoscopy is an important diagnostic and therapeutic tool for digestive system diseases.The artificial intelligence(AI)-assisted system in endoscopy(hereinafter referred to as AI in digestive endoscopy)has broad application prospects in the field of digestive endoscopy.The trust and acceptance of endoscopic subjects are the cornerstone of the research,application,and promotion of AI in digestive endoscopy.Currently,the tools for measuring the acceptance of AI in digestive endoscopy by subjects are limited at home and abroad.This study aims to develop a scale for measuring the acceptance of AI in digestive endoscopy by subjects,then to evaluate its reliability and validity. Methods:By conducting literature research,an item pool and dimensions were constructed,and a preliminary scale was constructed using Delphi method.Through the first stage of the survey on the subjects,the reliability and validity of the scale were tested,and the revised scale was used for the second stage of survey on the subjects to further verify the structural validity of the scale. Results:The acceptance scale for AI in digestive endoscopy included 11 items in 3 dimensions:accuracy,ethics,benefit and willingness.In the first stage of the survey,351 valid questionnaires were collected,and the Cronbach's α was 0.864.The correlation coefficient between the total score of the scale and the score of the test item was 0.636,and the Kaiser-Meyer-Olkin(KMO)value in exploratory factor analysis was 0.788.In the second stage of the survey,335 valid questionnaires were collected,and in confirmatory factor analysis,the χ2/df was 3.774,while the root mean squared error of approximation(RMSEA)was 0.091. Conclusion:Acceptance scale for AI in digestive endoscopy by subjects developed in this study has good reliability and validity.

OBJECTIVE: To evaluate the efficacy and safety of glyceryl phenylbutyrate (GPB) therapy for patients with Ornithine transcarbamylase deficiency (OTCD). METHODS: Two children with OTCD were selected as the study subjects, and their clinical manifestations, blood ammonia, liver enzymes, growth and development information following the treatment with GPB were retrospectively analyzed. A literature review was also carried out by searching the PubMed database for studies on the GPB treatment for urea cycle disorders. RESULTS: With the GPB treatment, the blood ammonia and liver enzyme level in both patients have decreased to the normal range within 3 months. Motor development in child 2 has improved. No adverse reaction was noted, except for transient palmar greasy smell and loss of appetite in child 1. Analysis of the literature showed that patients had lower ammonia exposure, lower annual incidence of hyperammonemic crisis, more actual protein intake and fewer adverse events during GPB treatment. CONCLUSION GPB is safe and effective for the treatment of OTCD.
Child ; Humans ; Ornithine Carbamoyltransferase Deficiency Disease/drug therapy* ; Phenylbutyrates/therapeutic use* ; Ammonia ; Retrospective Studies

Tumor-induced osteomalacia (TIO), also known as oncogenic osteomalacia, is a rare paraneoplastic syndrome characterized by hypophosphatemia resulting from decreased tubular phosphate reabsorption, with a low or inappropriately normal level of active vitamin D. The culprit tumors of TIO could produce fibroblast growth factor 23 which plays a role in regulating renal Pi handling and 25-hydroxyvitamin D 1α-hydroxylase activity. Chronic hypophosphatemia could eventually lead to inadequate bone mineralization, presenting as osteomalacia. The diagnosis should be considered when patients manifest as hypophosphatemia and osteomalacia, or rickets and needs to be differentiated from other disorders of phosphate metabolism, such as the inhereditary diseases like X-linked hypophosphataemic rickets, autosomal dominant hypophosphataemic rickets, autosomal recessive hypophosphataemic rickets and acquired diseases like vitamin D deficiency. Localization of responsible tumors could be rather difficult since the vast majority are very small and could be everywhere in the body. A combination of thorough physical examination, laboratory tests and imaging techniques should be applied and sometimes a venous sampling may come into handy. The technology of somatostatin-receptor functional scintigraphy markedly facilitates the localization of TIO tumor. Patients undergoing complete removal of the causative neoplasm generally have favorable prognoses while a few have been reported to suffer from recurrence and metastasis. For those undetectable or unresectable cases, phosphate supplements and active vitamin D should be administrated and curative intended radiotherapy or ablation is optional.
Calcification, Physiologic ; Diagnosis ; Fibroblast Growth Factors ; Humans ; Hypophosphatemia ; Metabolism ; Neoplasm Metastasis ; Osteomalacia ; Paraneoplastic Syndromes ; Physical Examination ; Prognosis ; Radionuclide Imaging ; Radiotherapy ; Recurrence ; Rickets ; Vitamin D ; Vitamin D Deficiency

Inborn Errors of Metabolism (IEM) are diseases caused by genetic defects in the biosynthesis of certain enzymes, receptors, carriers and membrane pumps composed of peptides and/or proteins necessary to maintain normal metabolism of the body, namely, mutations in the genes encoding such peptides (proteins). Through metabolomic analysis of sugars, amino acids, organic acids, fatty acids, carnitine and other substances in blood or urine, as well as cellular enzymology, molecular biology and proteomics analysis. We can identify and quantify abnormal biomarks, determine biomarkers as diagnostic and therapeutic monitoring tools. At the same time, through proteomic research. We can provide scientific basis for the pathogenesis of hereditary metabolic diseases.

Citrin deficiency is an autosomal recessive disorder caused by mutation of the SLC25A13 gene, and is one of the most important causes of infant cholestasis in China. The metabolic mechanism of CD is complex, involving the urea cycle, the malate aspartate cycle, the citrate malate cycle, fatty acid metabolism, carbohydrate motabolism and other metabolic pathways. Metabolomics has some applications in CD by analyzing metabolite alterations. This article provides a review for research progress of metabolomics in CD.