[Evaluation of the efficacy and safety of intravenous infusion of ferric derisomaltose in the treatment of iron deficiency anemia: a single-center retrospective analysis].

Objective: To investigate the clinical efficacy and safety of ferric derisomaltose injection versus iron sucrose injection in the treatment of iron deficiency anemia (IDA) . Methods: A total of 120 patients with iron deficiency anemia admitted from June 2021 to March 2023 were given intravenous iron supplementation with ferric derisomaltose to assess the efficacy and safety of hemoglobin (HGB) elevation before and after treatment. Simultaneously, the clinical effects of iron supplementation with iron sucrose were compared to those of inpatient patients during the same period. Results: Baseline values were comparable in both groups. Within 12 weeks of treatment, the elevated HGB level in the ferric derisomaltose group was higher than that of the iron sucrose group, with a statistical difference at all time points, and the proportion of HGB increased over 20 g/L in the patients treated for 4 weeks was higher (98.7%, 75.9% ). During the treatment with ferric derisomaltose and iron sucrose, the proportion of mild adverse reactions in the ferric derisomaltose group was slightly lower than that of the iron sucrose group, and neither group experienced any serious adverse reactions. The patients responded well to the infusion treatment, with no reports of pain or pigmentation at the injection site. Conclusion: The treatment of IDA patients with ferric derisomaltose has a satisfactory curative effect, with the advantages of rapidity, accuracy, and safety. Therefore, it is worthy of widespread clinical use.

[Hyperglycemia caused by mutation of GCK gene in 10 patients analysis of clinical and mutation characteristics].

Objective: To explore the gene mutation characteristics and detailed clinical presentations of hyperglycemia caused by GCK mutations in 10 patients. Methods: The clinical and follow-up data of 10 patients with hyperglycemia caused by mutation of GCK gene were reviewed. The patients were ascertained between January 1, 2014 and August 31, 2018 at the Department of Pediatrics, the First Affiliated Hospital of Zhejiang University and Ningbo Women & Children's Hospital. Clinical data were collected, including age, gender, main complaint, family history, fasting blood glucose, fasting blood insulin, 2-hour blood glucose, 2-hour blood insulin after oral glucose tolerance test, glycosylated hemoglobin, anti-glutamic acid decarboxylase antibody and body mass index. Mutations of GCK gene were detected by Sanger sequencing or high-throughput sequencing of diabetes-related genes in the patients and their family members. Results: There were ten patients, 8 of them were male, 2 were female.The ages at diagnosis varied between 4.7 to 12.3 years. The patients usually did not have obvious clinical symptoms of diabetes mellitus. Most of them were unexpectedly found to have hyperglycemia and with impaired glucose metabolism in three consecutive generations. The fasting blood glucose of patients was 6.8-7.7 mmol/L, 2-hour postprandial blood glucose was 7.8-11.6 mmol/L. Fasting blood insulin was 0.5-8.5 mU/L, glucose tolerance test results showed that 2 h postprondial blood insulin was 1.3-55.4 mU/L. The level of glycosylated hemoglobin was 6.1%-6.8%. Anti-glutamic acid decarboxylase antibody was negative in all patients. The GCK mutations identified in patients and one of their parents were located at exon5 (4 cases), exon9 (2 cases), exon2 (1 case), exon4 (1 case), exon6 (1 case) and exon7 (1 case). Conclusions: Most of the hyperglycemia patients caused by GCK mutations did not have typical clinical symptoms of diabetes. The fasting blood glucose was slightly elevated. Abnormal glucose tolerance test results were found in all 10 patients. Three consecutive generations of family had impaired glucose metabolism. GCK mutations located at exon 5 were common in 10 cases. There was no correlation between type of mutations and plasma glucose levels in domestic and international researches. When fasting glucose was found abnormal in clinic, a complete family history should be taken and the GCK gene should be sequenced to confirm the diagnosis in time.

[A preliminary study on SLAMF6 expression in patients with severe aplastic anemia].

Objective: To explore the expression of SLAMF6 on CD8(+) T cells in patients with severe aplastic anemia (SAA) and its correlation with disease immune status. Methods: By flow cytometry (FCM), SLAMF6 expression level in peripheral blood CD8(+) T cells was detected in 21 patients with SAA and 15 normal controls respectively from February 2017 to April 2018. The correlation between SLAMF6 expression level and hematopoietic functions, including HGB, PLT, the neutrophil granulocyte and reticulocyte absolute value in peripheral blood, hyperplasia degree (percentage of granulocytes, erythrocytes, lymphocytes and megakaryocytes in bone marrow) and perforin, granzyme B, IFN-γ expression level in CD8(+) T cells were evaluated. To further confirm the effect of SLAMF6 on CD8(+) T cells, anti-SLAMF6 Ab was used to block SLAMF6 pathway (IgG as control), and FCM was used to detect the perforin, granzyme B, and IFN-γ production of CD8(+) T cells. Results: The expression of SLAMF6 on CD8(+) T cells in untreated SAA patients[(56.29±12.97)%]was significantly lower than that of normal controls[(80.96±7.36)%](t=-7.672, P<0.001). The expression of SLAMF6 on CD8(+) T cells in SAA patients were positively correlated with the HGB, PLT, the neutrophil granulocyte and reticulocyte absolute value in peripheral blood, percentage of granulocytes, erythrocytes in bone marrow (all P<0.05), but they were negatively correlated with the percentage of lymphocytes in bone marrow, and the expression of perforin, granzyme B, and IFN-γ of CD8(+) T cells (all P<0.05). After blocking SLAMF6 pathway by anti-SLAMF6 Ab, the expression levels of perforin, granzyme B and IFN-γ in SAA patients were significantly higher than those in the untreated group, and the differences were statistically significant (all P<0.05). Conclusions: SLAMF6 is significantly down-regulated on CD8(+) T cells in SAA patients, which may act as a negative immunoregulatory molecule participating in the mechanism of SAA by affecting the functional molecules secretion on CD8(+) T cells.

Primary male development of two sequentially hermaphroditic groupers, Epinephelus akaara and Epinephelus awoara (Perciformes: Epinephelidae).

Gonad ontogeny of the Hong Kong grouper Epinephelus akaara (a bi-directional sex changer) and the yellow grouper Epinephelus awoara (a protogynous hermaphrodite) was examined for the first time from post-larval phase until first sexual maturation, by histology. Approximately 20 specimens of each species were collected randomly every 2-7 weeks from rearing tanks with natural sea water and temperature between June 2013 and June 2014. The paired gonadal primordia (GP) were observed at 6 weeks after hatching (wah) for both species; however, gonia were first observed in GP at 16 wah for E. akaara and at 8 wah for E. awoara. The timings for the appearance of primary-growth stage oocytes (O1) and the completion of ovarian lumen (OL) varied; both at 27 wah for E. akaara, and at 18 and 23 wah for E. awoara respectively. A bisexual-phase gonad with an OL, O1 and scattered spermatogenic cysts (SC) was observed at 27-29 wah for both E. akaara and E. awoara. Sexual differentiation was subsequently observed from the bisexual-phase gonad at 34 wah for E. akaara, and 41 wah for E. awoara, with the appearance of cortical-alveolus stage oocytes (O2) for developing female and the proliferation of SC for developing primary male (i.e. from juvenile directly). Ovaries of mature females contained the vitellogenic stage oocytes (O3) and scattered SC; testes of mature primary males had sperm in sperm sinuses within the gonadal wall and remained O1. Minimum age of first sexual maturation for both female and primary male of E. akaara was at 41 wah; minimum total length (LT ) of female (143 mm) was larger than that of primary male (137 mm L(T)). Minimum age and size of first sexual maturation for female of E. awoara (47 wah and 149 mm L(T), respectively) were larger than those of E. akaara. Developing primary males of E. awoara were found at 41-58 wah, however, mature males were not observed, indicating inconsistency in first sexual maturation for E. awoara. This study provided strong evidences of primary male pathway in E. akaara and E. awoara; the latter is confirmed to be diandric.

Polymorphic microsatellite markers in the false kelpfish Sebastiscus marmoratus: isolation, characterization, and cross-species amplification.

A (GT/CA)13-microsatellite-enriched genomic library of the false kelpfish Sebastiscus marmoratus was constructed, and 20 polymorphic microsatellite loci were isolated and characterized. The polymorphisms were investigated in 48 wild individuals from a single population collected from the northern Yellow Sea. The numbers of alleles per locus varied from 4-22 with an average of 9. The observed and expected heterozygosities of each locus ranged from 0.196-0.958 and from 0.487-0.942, with an average of 0.693 and 0.765, respectively. One locus significantly deviated from Hardy-Weinberg equilibrium, and one pair of loci was in linkage disequilibrium determined by Bonferroni's correction. Cross-species amplification was also conducted in the related species Inimicus japonicus, collected from East China Sea. The result showed that six loci could be amplified from I. japonicus DNAs. These polymorphic markers would be useful for assessment of genetic variation and population structure of scorpionfish.

Novel tetranucleotide microsatellite markers for Chinese beard eel (Cirrhimuraena chinensis Kaup).

In this study, we characterized 12 novel polymorphic microsatellite loci for Chinese beard eel (Cirrhimuraena chinensis Kaup) from a tetranucleotide microsatellite-enriched library. Loci screened on a sample of 37 individuals from Xiamen wild stocks revealed 8 to 24 alleles per locus, with a mean of 13.83 over all loci. Observed and expected heterozygosities ranged from 0.270 to 0.944 and 0.439 to 0.942, respectively. These efficient genetic markers thus provide useful tools in the study of the population genetics and phylogeography of Chinese beard eel.

Epinephelus moara: a valid species of the family Epinephelidae (Pisces: Perciformes).

This study documents the major external and internal morphological differences between Epinephelus bruneus and Epinephelus moara, and analyses the complete mitogenomes of both species. The partial cytochrome oxidase subunit I (coI) sequence divergence between E. bruneus and E. moara is significantly higher than specimens within the same species (P < 0·05). Analyses of gene flow (Nm = 0·02) and genetic differentiation (ϕst = 0·92995, P > 0·05) reveal reproductive isolation between E. bruneus and E. moara. These results support the hypothesis that E. moara is a valid species. Further molecular comparisons between E. bruneus and E. moara obtained in this study and a specimen identified in GenBank as E. bruneus from South Korea reveal that the latter is identical to E. moara rather than to E. bruneus.

Characterization and bacterial-binding activity of a novel C-type lectin from the red-spotted grouper, Epinephelus akaara.

Because of their specific binding to carbohydrates, lectins play a crucial role in pathogen recognition and clearance in vertebrate animals. Previously, only two types of collectins had been isolated from bony fish: mannan-binding lectin (MBL) and galactose-binding lectin (GalBL). We sequenced a novel collectin (designated EALec1) from the red-spotted grouper, Epinephelus akaara. The gene structure of EALec1 and the alignment of the carbohydrate recognition domain of the three collectins demonstrated that EALec1 is a new type of collectin derived from MBL. We examined the expression pattern of the EALec1 transcripts in 12 tissues of the red-spotted grouper. The EALec1 gene was found to have multiple copies; their transcripts were detected in all 12 tissues. EALec1 was also recombined and expressed in Escherichia coli to investigate its immune functions and carbohydrate-binding characterization. We concluded that EALec1 belongs to the mannan-binding lectin group, despite its different Ca²âº-dependent sites in the carbohydrate recognition domain, and that it is involved in the recognition and clearance of invaders in the red-spotted grouper.