Clinical and Laboratory Characteristics in Children with Alagille Syndrome: Experience of a Single Center.

Background: This study aimed to explore the clinical predictors of Alagille syndrome (ALGS) in children and to provide a basis for early diagnosis. Methods: We retrospectively analyzed the clinical data of 14 children diagnosed with ALGS at the First People's Hospital of Lianyungang City from March 2016 to March 2021 and followed up the children. Results: Among the 14 patients, 9 (64.28%) had cholestasis, 12 (85.71%) had heart malformations, 13 (92.85%) had characteristic facial features, 2 (14.28%) had pruritus, and 2 (14.28%) had a positive family history. Among the 13 patients who were examined by pediatric ophthalmologists, 3 patients had ocular lesions. Among the 13 patients who underwent spine radiography, 2 had typical butterfly vertebrae. Among the 6 patients with hepatic pathology, 2 had intracellular cholestasis, 2 had reduced or no small bile duct in the portal area, 2 had small bile duct hyperplasia with massive fibrous hyperplasia and extensive inflammatory cell infiltration, and 2 underwent biliary tract exploration. Genetic testing of 12 children with ALGS revealed JAG1 gene mutations in 7 cases and NOTCH2 gene mutations in 2 cases. The abovementioned two mutant genes were not detected in any of the 3 cases. Among the 12 followed-up patients, 7 were in stable condition, 5 underwent liver transplantation, and 1 died of severe pneumonia. Conclusion: Cholestatic liver disease, cardiac malformations, and abnormal facial development are predictors of ALGS in children and can be definitively diagnosed by genetic testing.

Investigation of serum IL-12, IL-16, and IL-17A as diagnostic biomarkers in children with cow's milk protein allergy.

BACKGROUND: Cow's milk protein allergy (CMPA) is an abnormal immune response caused by milk proteins and is most common in infancy and early childhood. Statistics revealed up to 7.5% of children suffered from milk allergy. Its clinical symptoms were characterized by diversity, non-specificity, and can affect multiple systems, including the digestive tract, skin, and respiratory tract. In this study, we aimed to investigate the effects of IL-12, IL-16, and IL-17A on diagnosing and monitoring CMPA in children for clinical treatment. METHODS: A total of 158 infants with CMPA and 89 healthy babies were recruited and evaluated. Demographic and clinical information of all participants were recorded. An extensive analysis of inflammatory cytokine levels, including IL-12, IL-16, and IL-17A, was performed in blood samples from 247 infants younger than 9 months. Meanwhile, the serological specificity immunoglobulin E (sIgE) levels were evaluated. In addition, the area under the curve (AUC) values of IL-12, IL-16, and IL-17A in differentiating CMP from healthy babies were measured by receiver operating characteristic analysis. Finally, the correlation between sIgE and IL-12, IL-16, and IL-17A levels were detected using Spearman correlation analysis. RESULTS: Compared with healthy control, infants who developed CMPA had decreased IL-12, increased IL-16, and IL-17A. Moreover, a significant correlation between serum IL-12, IL-16, IL-17A and sIgE levels was observed in the CMPA group. In addition, AUC values of IL-12, IL-16, and IL-17A in discriminating CMPA from healthy infants were 0.8425, 0.9196, and 0.8813, respectively. Finally, IL-12 was increased while IL-16 and IL-17A levels were decreased in the CMPA group after three months of milk avoidance treatment. CONCLUSIONS: We found that IL-12, IL-16, and IL-17A levels in children with CMPA were associated with SCORAD scores, sIgE levels, and disease severity, functioning as valuable disease-monitor markers in CMPA.

Effect of Whole-Course Continuous Nursing Intervention Combined with a Nursing Practice Teaching Method on Quality of Life of Children with Functional Dyspepsia and Parents' Satisfaction Based on Smart Health.

With the development of information technology, it has become a part of people's lives. WeChat is not only a popular chatting tool in daily life but can also be used in the medical field. Functional dyspepsia is a common pediatric disease, with complex pathogenic factors, which are usually attributed to abnormal gastric acid secretion and gastrointestinal dysfunction. In our research, we aim to investigate the effects of whole-course continuous nursing intervention combined with a nursing practice teaching method on the quality of life (QOL) of children with functional dyspepsia and parents' satisfaction. One hundred and twenty children with functional dyspepsia admitted to our hospital (February 2019-February 2020) were retrospectively analyzed and equally divided into the experimental group (EG) and the control group (CG) according to the order of admission. The CG received whole-course continuous nursing intervention, and the EG received whole-course continuous nursing intervention combined with the nursing practice teaching method. Children's psychological states and QOL and parents' satisfaction of both groups were compared. After discharge, compared with the CG, the negative emotion scores in the EG were remarkably lower (P < 0.001). After discharge, compared with the CG, the QOL scores (P < 0.001), the proportion of children with good and excellent QOL (P < 0.05), and parents' satisfaction (P < 0.001) in the EG were remarkably higher. For children with functional dyspepsia, whole-course continuous nursing intervention combined with the nursing practice teaching method can improve their negative emotions, QOL, and parents' satisfaction, which should be popularized in practice.

Circ_0132817 facilitates cell proliferation, migration, invasion and glycolysis by regulating the miR-432-5p/NOL4L axis in neuroblastoma.

Neuroblastoma (NB) is one of the most common extracranial solid tumors in children. Circular RNAs (circRNAs) have been shown to be involved in the development of NB. However, the function of circ_0132817 in NB is currently unclear. In this paper, the levels of circ_0132817 and NOL4L were induced in NB tissues and cells, and miR-432-5p expression was on the contrary. MiR-432-5p was verified as a target of circ_0132817 and miR-432-5p could bind to NOL4L. The inhibitory effects of miR-432-5p overexpression on cell proliferation, migration, invasion and glycolysis could be reversed by circ_0132817 facilitation. The suppression of NOL4L knockdown on NB cells progression could be rescued by miR-432-5p inhibition. Besides, knockdown of circ_0132817 repressed tumor growth in vivo. Thus, we came to a conclusion that circ_0132817 promoted the tumorigenesis of NB cells by up-regulating NOL4L and acting as a sponge for miR-432-5p.