OBJECTIVES: To study the clinical characteristics of pediatric Behçet syndrome (BS). METHODS: A retrospective review was conducted on the medical records of children hospitalized in the Department of Pediatrics at the Second Hospital of Hebei Medical University between December 2014 and December 2024 who met diagnostic criteria for BS. RESULTS: Among 45 children with BS, 26 (58%) were male. Oral aphthous ulcers were the most common manifestation (43/45, 96%), followed by genital ulcers (23/45, 51%) and gastrointestinal involvement (18/45, 40%). Genital ulcers were more frequent in girls, whereas ocular involvement was more common in boys (P<0.05). The pathergy test was positive in 10 (22%), and HLA-B51 was positive in 13 (29%). Fecal calprotectin (FC) was elevated in 16 (36%); gastrointestinal involvement was more frequent in children with elevated FC than in those with normal FC (P<0.05). According to the respective criteria, 17 (38%) patients met the International Study Group criteria (1990), 33 (73%) met the International Criteria for Behçet Disease (2014), and 13 (29%) met the Pediatric Behçet Disease criteria (2015). CONCLUSIONS Pediatric BS shows marked clinical heterogeneity. HLA-B51 is associated with disease susceptibility.
Humans ; Behcet Syndrome/genetics* ; Male ; Female ; Child ; Retrospective Studies ; Adolescent ; Child, Preschool ; Leukocyte L1 Antigen Complex/analysis* ; HLA-B51 Antigen

OBJECTIVES: To improve the understanding of the clinical phenotypes and genetic characteristics of nephronophthisis (NPHP) and related syndromes in children. METHODS: A retrospective analysis was performed on the medical data of eight children with NPHP and related syndromes who were diagnosed and treated in the Department of Pediatrics of the Second Hospital of Hebei Medical University, from January 2018 to November 2022. The clinical characteristics and genetic testing results were analyzed. RESULTS: Among these eight children, there were five boys and three girls, with an age of onset ranging from 15 months to 12 years. All 8 children exhibited different degrees of renal function abnormalities when they attended the hospital. Among the eight children, two had the initial symptom of delayed development, two had the initial symptom of anemia, and two were found to have abnormal renal function during physical examination. The extrarenal manifestations included cardiovascular abnormalities in two children, skeletal dysplasia in two children, liver dysfunction in one child, retinitis pigmentosa in one child, and visceral translocation in one child. All eight children had renal structural changes on ultrasound, and four children had mild to moderate proteinuria based on routine urine test. Of all eight children, five had NPHP1 gene mutations and one each had a gene mutation in the NPHP3, IFT140, and TTC21B genes, and four new mutation sites were discovered. CONCLUSIONS Children with NPHP and related syndromes often have the initial symptom of delayed development or anemia, and some children also have extrarenal manifestations. NPHP and related syndromes should be considered for children with unexplained renal dysfunction, and high-throughput sequencing may help to make a confirmed diagnosis.
Child ; Humans ; Retrospective Studies ; Syndrome ; Kidney Diseases, Cystic/genetics* ; Mutation ; Phenotype

China/epidemiology* ; Coronary Angiography ; Coronary Artery Disease/epidemiology* ; Cross-Sectional Studies ; Humans ; Risk Assessment ; Risk Factors

OBJECTIVETo investigate UbcH10 expression in hepatocellular carcinoma and explore its clinicopathological implications.

METHODSWe detected UbcH10 mRNA expression using RT-PCR in normal liver cell line, cancer cell lines, surgically removed hepatocellular carcinoma tissue and corresponding adjacent non-tumor tissue and evaluated the clinicopathological significance of UbcH10. Immunohistochemistry was performed to investigate UbcH10 protein expression in hepatocellular carcinoma tissue, the adjacent tissue, and normal liver tissue specimens.

RESULTSNormal liver cell line L02 showed significantly lower UbcH10 mRNA expression levels than the cancer cell lines BEL-7402, Hep3B, HepG2 and SMMC-7721 (P<0.05). UbcH10 mRNA expression was also was significantly higher in hepatocellular carcinoma tissues than in the corresponding non-tumor tissues (P<0.05). Clinicopathological evaluation suggested that UbcH10 expression was associated with tumor invasion of the portal vein, tumor size, TNM staging, and tumor differentiation (P<0.05). Immunohistochemistry identified stronger UbcH10 expression in hepatocellular carcinoma tissues than in the adjacent tissues and normal liver tissues (68.6%, 28.6%, and 26.7%, respectively).

CONCLUSIONUbcH10 is over-expressed in hepatocellular carcinoma and may serve as a novel biomarker as well as a therapeutic target of hepatocellular carcinoma.

Adult ; Aged ; Biomarkers, Tumor ; Carcinoma, Hepatocellular ; metabolism ; pathology ; Female ; Hep G2 Cells ; Humans ; Liver Neoplasms ; metabolism ; pathology ; Male ; Middle Aged ; RNA, Messenger ; genetics ; metabolism ; Ubiquitin-Conjugating Enzymes ; genetics ; metabolism

Objective To explore the association between psychological health level and the characteristic and coping style in nurses working in infectious disease department. Methods 98 nurses in infectious disease department were selected as experimental group and 118 nurses in internal department were selected as control group. All the subjects were from four grade-3 hospitals in Xi' an city and investigated by SCL-90. Individuals in experimental group were investigated by EPQ and CSQ, besides the SCL-90. Results Compared with common mode of healthy Chinese women, interpersonal sensitivity, paranoid scores in SCL-90 was lower and the differences were statistically significant (all P ＜ 0. 05), other factors did not reach the statistical significance (P＞0. 05), and differences of all the factors score and total score in SCL-90 between the two groups did not reach the statistical significance (P ＞ 0. 05). Seeking help coping style in experimental group was negatively correlated with interpersonal sensitivity and depression degree (r = -0. 28, -0. 29,P ＜0. 05 or P ＜0. 01),other coping styles such as self-blame, fantasy, avoidance, and rationalization were positively correlated with majority factors in SCL-90 (P ＜0. 05 or P ＜0. 01). P dimension in EPQ in experimental group was positively correlated with terror, paranoia, and psychosis in SCL-90 (r = - 0. 26,0. 34,0. 28, P ＜ 0. 05 or P ＜ 0. 01)and N dimension in EPQ in experimental group was positively correlated with all the factors in SCL-90(P ＜0. 01), E dimension in EPQ in experimental group was negatively correlated with interpersonal sensitivity,depression degree, anxiety, and terror factors in SCL-90 (P ＜ 0. 05 or P ＜ 0. 01). Conclusions Overall psychological level of nurses working in infectious disease department of comprehensive hospitals in Xi' an city was good. The psychological events were not statistically different between the nurses in infectious department and nurses in internal department. Psychological health level and the characteristic were correlated with coping style in nurses working in infectious disease department of general hospital in Xi' an city.