A rare case report: multiple intrahepatic masses in a pediatric patient with citrin deficiency.

Deficiency of citrin, the liver-type aspartate-glutamate carrier, arises from biallelic mutations of the gene SLC25A13. Although citrin deficiency (CD) is associated with higher risk of hepatocellular carcinoma (HCC) in adult patients, this association remains inconclusive in pediatric cases. The patient in this paper had been diagnosed to have CD by SLC25A13 analysis at the age 10 months, and then in response to dietary therapy, her prolonged jaundice and marked hepatosplenomegaly resolved gradually. However, she was referred to the hospital once again due to recurrent abdominal distention for 2 weeks at her age 4 years and 9 months, when prominently enlarged liver and spleen were palpated, along with a strikingly elevated serum alpha-fetoprotein (AFP) level of 27605 ng/mL as well as a large mass in the right liver lobe and a suspected tumor thrombus within the portal vein on enhanced computed tomography. After 4 rounds of adjuvant chemotherapy, right hepatic lobectomy and portal venous embolectomy were performed at her age 5 years and 3 months, and metastatic hepatoblastoma was confirmed by histopathological analysis. Afterwards, the patient underwent 5 additional cycles of chemotherapy and her condition remained stable for 7 months after surgery. Unfortunately, hepatoblastoma recurred in the left lobe at the age 5 years and 10 months, which progressed rapidly into liver failure, and led to death at the age 6 years and 1 month. As far as we know, this is the the first case of hepatoblastoma in a patient with CD, raising the possibility of an association between these two conditions.

Description of Aequorivita aurantiaca sp. nov. Isolated from Coastal Sediment, and Comparative Genomic Analysis and Biogeographic Distribution of the Genus Aequorivita.

A novel Gram-stain-negative, facultatively anaerobic, and non-motile bacterial strain, designated SDUM287046T, was isolated from the coastal sediments of Jingzi Port of Weihai, China. Cells of strain SDUM287046T were rod-shaped with widths of 0.4-0.5 µm and lengths of 0.7-1.4 µm and could produce flexirubin-type pigments. Optimum growth of strain SDUM287046T occurred at 33-35 °C, pH 7.0, and with 2% (w/v) NaCl. Oxidase activity was negative, but catalase activity was positive. Phylogenetic analysis based on 16S rRNA gene sequence revealed that strain SDUM287046T was most closely related to Aequorivita aquimaris D-24T (98.3%). The main cellular fatty acids were iso-C15:0, anteiso-C15:0, iso-C17:0 3-OH, and summed feature 9 (comprised of iso-C17:1 ω9c and/or C16:0 10-methyl). The sole respiratory quinone was MK-6. The polar lipids consisted of phosphatidylethanolamine (PE), one aminolipid (AL), three unidentified glycolipids (GL), and three unidentified lipids (L). The DNA G + C content was 39.3 mol%. According to the integrated results of phylogenetic, physiological, biochemical, and chemotaxonomic characteristics, we propose that strain SDUM287046T represents a novel species of the genus Aequorivita, for which the name Aequorivita aurantiaca sp. nov. is proposed. The type strain is SDUM287046T (=KCTC 92754T = MCCC 1H01418T). Comparative genomic analysis showed that the 16 Aequorivita species shared 1453 core genes and differed mainly in amino acid metabolism, cofactor metabolism, and vitamin metabolism. Biogeographic distribution analysis indicated that the marine environments were the primary habitat of Aequorivita bacteria.

Psychromarinibacter sediminicola sp. nov., a novel moderately halophilic, metabolically diverse bacterium isolated from a solar saltern sediment, and comparison between members of family Roseobacteraceae.

Known for its species abundance and evolutionary status complexity, family Roseobacteraceae is an important subject of many studies on the discovery, identification, taxonomic status, and ecological properties of marine bacteria. This study compared and analyzed the phylogenetic, genomic, biochemical, and chemo taxonomical properties of seven species from three genera (Psychromarinibacter, Lutimaribacter, and Maritimibacter) of the family Roseobacteraceae. Moreover, a novel strain, named C21-152T was isolated from solar saltern sediment in Weihai, China. The values of 16S rRNA gene sequence similarity, the average nucleotide identity (ANI), the average amino acid identity (AAI), and the digital DNA-DNA hybridization (dDDH) between genomes of the novel strain and Psychromarinibacter halotolerans MCCC 1K03203T were 97.19, 78.49, 73.45, and 21.90%, respectively. Genome sequencing of strain C21-152T revealed a complete Sox enzyme system related to thiosulfate oxidization as well as a complete pathway for the final conversion of hydroxyproline to α-ketoglutarate. In addition, strain C21-152T was resistant to many antibiotics and had the ability to survive below 13% salinity. This strain had versatile survival strategies in saline environments including salt-in, compatible solute production and compatible solute transport. Some of its physiological features enriched and complemented the knowledge of the characteristics of the genus Psychromarinibacter. Optimum growth of strain C21-152T occurred at 37 â„ƒ, with 5-6% (w/v) NaCl and at pH 7.5. According to the results of the phenotypic, chemotaxonomic characterization, phylogenetic properties and genome analysis, strain C21-152T should represent a novel specie of the genus Psychromarinibacter, for which the name Psychromarinibacter sediminicola sp. nov. is proposed. The type strain is C21-152T (= MCCC 1H00808T = KCTC 92746T = SDUM1063002T).

Antibiotic and Heavy Metal Co-Resistant Strain Isolated from Enrichment Culture of Marine Sediments, with Potential for Environmental Bioremediation Applications.

Antibiotics and heavy metals have caused serious contamination of the environment and even resulted in public health concerns. It has therefore become even more urgent to adopt a sustainable approach to combating these polluted environments. In this paper, we investigated the microbial community of marine sediment samples after 255 days of enrichment culture under Cu (II) and lincomycin stress and ZC255 was the most resistant strain obtained. The 16S rRNA gene sequence confirmed that it belonged to the genus Rossellomorea. Strain ZC255 was resistant to 12 kinds of antibiotics, and had a superior tolerance to Cu (II), Pb (II), Ni (II), Zn (II), Cr (III), and Cd (II). Moreover, it exhibits strong bioremoval ability of Cu and lincomycin. The removal efficiency of Cu (II) and lincomycin can achieve 651 mg/g biomass and 32.5 mg/g biomass, respectively. Strain ZC255 was a promising isolate for pollution bioremediation applications.

The influence of "small private online course + flipped classroom" teaching on physical education students' learning motivation from the perspective of self-determination theory.

Objective: The study aimed to enhance the learning motivation of college physical education students and improve their learning outcomes. Based on the perspective of the self-determination theory, this study explores the influence of "Small Private Online Course (SPOC) + flipped classroom" teaching on the learning motivation of students majoring in physical education and profoundly analyzes the influencing factors and promotion paths of learning motivation using this model. Materials and methods: A total of four classes (64 students) of physical education majors in a university were selected and randomly divided into an experimental group (34 students) and a control group (30 students). The experimental group received "SPOC + flipped classroom" teaching, the control group received traditional teaching. Before and after the 16-week intervention, learning motivation, teacher support perception, basic psychological need satisfaction, and academic emotions of the 64 students were measured, and the data were analyzed by repeated-measures analysis of variance and partial least square regression. Results: (1) The instructional intervention reduced non-regulation, external regulation, and introjected regulation, while increased identified regulation, intrinsic regulation, and self-determination levels in the students. The levels of non-regulation, external regulation, identified regulation, and self-determination were also significantly different from those of the control group. (2) After the intervention, the scores of support for autonomy, support for competence, support for relatedness, and need for relatedness in the experimental group were significantly higher than those in the control group. (3) Support for autonomy, support for competence, support for relatedness, need for competence and need for relatedness positively predicted the self-determination level, and intrinsic regulation and identified regulation negatively predicted non-regulation, external regulation, and introjected regulation. Conclusion: "SPOC + flipped classroom" teaching has a positive impact on students' learning motivation of basketball skills and promotes students' motivation autonomy. The improvement of support for autonomy, support for competence, support for relatedness, need for competence, and need for relatedness may be related to the improvement of learning motivation of college students majoring in Physical Education (PE). "SPOC + flipped classroom" teaching enables students to obtain more demand satisfaction by giving them more demand support, while demand support and demand satisfaction can promote the internalization of learning motivation so that students can maintain high autonomy motivation.

Ovarian Adrenal Rest Tumors Undetected by Imaging Studies and Identified at Surgery in Three Females with Congenital Adrenal Hyperplasia Unresponsive to Increased Hormone Therapy Dosage.

Patients with congenital adrenal hyperplasia have a predisposition for developing adrenal rest tumors. In contrast to testicular adrenal rest tumors, ovarian adrenal rest tumors are less common, and only a few cases have been reported in the literature. This report presents three Chinese female congenital adrenal hyperplasia patients (9 to 15 years of age) with small ectopic adrenal cortical nodules that were not detected by imaging but were diagnosed at surgery. All three patients developed virilization with elevation of 17- hydroxyprogesterone, androstenedione, and androgen levels despite receiving maximum adrenal hormone replacement therapy. Ultrasound and magnetic resonance imaging of the abdomen and pelvis suggested bilateral expansion of the adrenal glands, but no lesions of the ovaries were observed. Laparoscopy and/or laparotomy revealed small nodular lesions surrounding the pelvic gonad in all three cases. Histopathological examination of the resected tissue in all cases revealed hyperplasic nodules of cells surrounded by fibrous tissue. The cells were arranged as nests with abundant cytoplasm, which were partially lightly stained with a small centered nucleus. Immunohistochemistry staining revealed the cells to be synaptophysin positive, melan-A positive, and chromogranin A negative, indicating the cells were adrenocortical tissue and not adrenal medullary cells. Thus, the findings of the histopathological examination were consistent with ovarian adrenal rest tumors. Female congenital adrenal hyperplasia patients with virilization who showed an inadequate response to hormone therapy and had negative imaging results may benefit from laparoscopic examination or laparotomy in order to confirm the diagnosis of ovarian adrenal rest tumors while receiving unilateral subtotal adrenalectomy or subtotal bilateral adrenalectomy.

Usefulness of Sonography in Evaluating Children Suspected of Malrotation: Comparison With an Upper Gastrointestinal Contrast Study.

OBJECTIVES: The purpose of this study was to prospectively evaluate the value of sonography in working up or ruling out malrotation by 3 sonographic features (inversion of the superior mesenteric artery and superior mesenteric vein, the whirlpool sign, and an intraperitoneal transverse duodenum) and comparing it with an upper gastrointestinal (GI) contrast study. METHODS: A total of 70 pediatric patients who underwent detailed abdominal sonography to rule out intestinal malrotation were included. Twenty-three of them also underwent an upper GI contrast study. Surgery or clinical follow-up was taken as the reference standard. Statistical analysis was performed with the χ(2) test. RESULTS: Twenty-three patients had a diagnosis of malrotation by surgical findings. With the combination of all 3 sonographic features, the sensitivity, specificity, and accuracy of sonography for determining malrotation were 100% (23 of 23), 97.8% (46 of 47), and 98.6% (69 of 70), respectively, whereas the sensitivity, specificity, and accuracy of the upper GI study were 40% (4 of 10), 64.3% (9 of 14), and 56.5% (13 of 23; P < .001). Combined anomalies in 2 patients and complications in 2 patients were also detected by sonography. CONCLUSIONS: By combining inversion of the superior mesenteric artery and superior mesenteric vein, the whirlpool sign, and an intraperitoneal transverse duodenum, sonography might be more valuable for accurately working up or ruling out pediatric malrotation than an upper GI contrast study. In addition, sonography could provide extra information, such as combined anomalies and intestinal necrosis, to help management.

Intestinal obstruction due to dual gastrointestinal atresia in infants: diagnosis and management of 3 cases.

BACKGROUND: Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue. CASE PRESENTATION: All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition. CONCLUSIONS: Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting.