Efficacy and safety of leuprorelin 3-month depot (11.25 mg) for idiopathic central precocious puberty treatment of Chinese girls: a single-center retrospective study.

OBJECTIVES: This study aimed to evaluate the efficacy and safety of 3-month leuprorelin acetate (3-month LA, 11.25 mg) for the treatment of idiopathic central precocious puberty (ICPP) in Chinese girls. METHODS: We conducted a single-center retrospective study in China on 28 girls with ICPP who received at least one year of 3-month LA treatment. Data from anthropometry, biochemistry, bone age (BA), and pelvic ultrasonography were assessed before and every 6 months during medication. RESULTS: At CPP diagnosis, the mean chronological age (CA) was 7.8±0.8 years, with bone age advancement (BA-CA) of 1.5±0.8 years. After treatment initiation, growth velocity decreased significantly from 8.5±1.6 cm/year to 5.8±1.1 cm/year at month 12 (p<0.001). GnRH-stimulated peak LH ≤3IU/L, the primary efficacy criterion, was observed in 27 out of 28 (96.4 %) children at month 3. Basal estradiol <20 pg/mL was achieved by all 28 girls (100 %) at month 6 and remained stable at month 12. Basal follicle-stimulating hormone (FSH) decreased from 4.1±3.5 to 1.7±0.9 (p<0.001), and basal LH was also significantly reduced from 3.3±6.5 to 0.7±0.8 (p=0.035) at month 12. The mean predicted adult height (PAH) at treatment initiation was 152.7±5.8 cm, it increased significantly to 157.5±5.5 cm (p=0.007) after one-year treatment. Pubertal development was slowed in most patients, and in some cases, it was even reversed. Only one patient (3.6 %) reported local intolerance. CONCLUSIONS: Three-month leuprorelin acetate is a safe and effective treatment for suppressing the pituitary-gonadal axis and restoring impaired adult height in Chinese girls.

A combination of selenium and Bacillus subtilis improves the quality and flavor of meat and slaughter performance of broilers.

This study aimed to investigate the effects of the combination of selenium and Bacillus subtilis (Se-BS) on the quality and flavor of meat and slaughter performance of broilers. A total of 240 one-day-old Arbor Acres broilers were randomly allotted to four treatments of a basal diet supplemented with no selenium (control), sodium selenite (SS), BS, or Se-BS and raised for 42 days. Compared with the control group, Se-BS significantly increased the carcass weight, the half-eviscerated weight, the completely eviscerated weight, the carcass rate, and redness in broiler muscles; improved the antioxidant state by increasing glutathione peroxidase (GPx) and glutathione S-transferase activities, the total antioxidant capacity, and GPx-1 and thioredoxin reductase 1 messenger RNA (mRNA) levels; promoted biological activity by increasing the contents of glutamate, phenylalanine, lysine, and tyrosine; and increased Se and five types of nitrogenous volatile substances in muscles. On the other hand, Se-BS treatment decreased the shear force, drip loss, and the malondialdehyde, glutathione, and lead contents in muscles. Se-BS exerted a better effect on slaughter performance, the physicochemical quality of meat, the redox status, the amino acid contents, the trace element contents, and volatile substances compared with SS and BS. In conclusion, Se-BS had a positive effect on the quality and flavor of meat and slaughter performance of broilers, suggesting that Se-BS may be a beneficial feed additive.

Effect of deoxynivalenol on inflammatory injury on the glandular stomach in chick embryos.

Deoxynivalenol (DON) has a strong toxic effect on the gastrointestinal mucosa of poultry. In this study, we evaluated chicken embryo development and glandular stomach damage to clarify the immunotoxic effects of DON injected through the allantoic cavity of chicken embryos. The glandular stomach index, routine blood indices, plasma inflammatory factors, pathological changes in the glandular stomach, and transcriptome results were analyzed in the hatching chicks. The results showed that DON was supertoxic to chicken embryos, causing edema, shedding, and bleeding of the mucosa of the glandular stomach, which triggered inflammatory reactions. As the toxin concentration increased, the immune system was successively activated and inhibited, and regulation was carried out by the differential regulation of the mitogen-activated protein kinase (MAPK) signal pathway. These results suggested that the immunotoxic effect of DON on the glandular stomach of chicken embryos was closely related to the regulation of the MAPK signaling pathway.

Selenium-enriched Bacillus subtilis Improves Growth Performance, Antioxidant Capacity, Immune Status, and Gut Health of Broiler Chickens.

This study aimed to investigate the effects of selenium (Se)-enriched Bacillus subtilis (Se-BS) on growth performance, antioxidant capacity, immune status, and gut health in broilers. A total of 240 one-day-old Arbor Acres broilers were randomly allotted to four groups and fed with basal diet (control group), 0.30 mg/kg Se (SS group), 3 × 109 CFU/g B. subtilis (BS group), and 0.30 mg/kg Se + 3 × 109 CFU/g B. subtilis (Se-BS group) for 42 days. The results showed that Se-BS supplementation increased body weight (BW), average daily gain, the activities of superoxide dismutase (SOD), glutathione peroxidase (GPx), catalase (CAT), and peroxidase (POD), total antioxidant capacity (T-AOC), and the contents of interleukin (IL)-2, IL-4, and immunoglobulin (Ig) G in plasma, the index and wall thickness of the duodenum, the villus height and crypt depth of the jejunum, and GPx-1 and thioredoxin reductase 1 mRNA levels in liver and intestine and decreased feed conversion ratio (FCR) and plasma malondialdehyde (MDA) content compared with the control group on day 42 (P < 0.05). Compared with groups SS and BS, Se-BS supplementation increased BW, the activities of GPx, CAT, and POD, and the contents of IL-2, IL-4, and IgG in plasma, the index and wall thickness of the duodenum, the crypt depth and secretory IgA content of the jejunum, and GPx-1 mRNA levels in liver and intestine and decreased FCR and plasma MDA content on day 42 (P < 0.05). In conclusion, Se-BS supplementation effectively improved the growth performance antioxidant capacity, immune status, and gut health of broilers.

Description of the molecular and phenotypic spectrum in Chinese patients with aggrecan deficiency: Novel ACAN heterozygous variants in eight Chinese children and a review of the literature.

Objective: This study analyzed eight Chinese short stature children with aggrecan deficiency, and aimed to investigate potential genotype-phenotype correlations, differences in clinical characteristics between the Chinese and the Western populations, and effectiveness of recombinant human growth hormone therapy in patients with ACAN variants through a review of the literature. Methods: Pediatric short stature patients with ACAN heterozygous variants were identified using whole-exome sequencing. Subsequently, a literature review was carried out to summarize the clinical features, genetic findings, and efficacy of growth-promoting therapy in patients with ACAN variants. Results: We identified seven novel ACAN mutations and one recurrent variant. Patients in our center manifested with short stature (average height SDS: -3.30 ± 0.85) with slight dysmorphic characteristics. The prevalence of dysmorphic features in the Chinese populations is significantly lower than that in the Western populations. Meanwhile, only 24.24% of aggrecan-deficient Chinese children showed significantly advanced bone age (BA). Promising therapeutic benefits were seen in the patients who received growth-promoting treatment, with an increase in growth velocity from 4.52 ± 1.00 cm/year to 8.03 ± 1.16 cm/year. Conclusion: This study further expanded the variation spectrum of the ACAN gene and demonstrated that Chinese children with short stature who carried ACAN heterozygous variants exhibited early growth cessation, which may remain unnoticed by clinicians as most of these children had very mild dysmorphic characteristics and showed BA that was consistent with the chronological age. Genetic testing may help in the diagnosis.

Clinical application of artificial intelligence in longitudinal image analysis of bone age among GHD patients.

Objective: This study aims to explore the clinical value of artificial intelligence (AI)-assisted bone age assessment (BAA) among children with growth hormone deficiency (GHD). Methods: A total of 290 bone age (BA) radiographs were collected from 52 children who participated in the study at Sun Yat-sen Memorial Hospital between January 2016 and August 2017. Senior pediatric endocrinologists independently evaluated BA according to the China 05 (CH05) method, and their consistent results were regarded as the gold standard (GS). Meanwhile, two junior pediatric endocrinologists were asked to assessed BA both with and without assistance from the AI-based BA evaluation system. Six months later, around 20% of the images assessed by the junior pediatric endocrinologists were randomly selected to be re-evaluated with the same procedure half a year later. Root mean square error (RMSE), mean absolute error (MAE), accuracy, and Bland-Altman plots were used to compare differences in BA. The intra-class correlation coefficient (ICC) and one-way repeated ANOVA were used to assess inter- and intra-observer variabilities in BAA. A boxplot of BA evaluated by different raters during the course of treatment and a mixed linear model were used to illustrate inter-rater effect over time. Results: A total of 52 children with GHD were included, with mean chronological age and BA by GS of 6.64 ± 2.49 and 5.85 ± 2.30 years at baseline, respectively. After incorporating AI assistance, the performance of the junior pediatric endocrinologists improved (P < 0.001), with MAE and RMSE both decreased by more than 1.65 years (Rater 1: ΔMAE = 1.780, ΔRMSE = 1.655; Rater 2: ΔMAE = 1.794, ΔRMSE = 1.719), and accuracy increasing from approximately 10% to over 91%. The ICC also increased from 0.951 to 0.990. During GHD treatment (at baseline, 6-, 12-, 18-, and 24-months), the difference decreased sharply when AI was applied. Furthermore, a significant inter-rater effect (P = 0.002) also vanished upon AI involvement. Conclusion: AI-assisted interpretation of BA can improve accuracy and decrease variability in results among junior pediatric endocrinologists in longitudinal cohort studies, which shows potential for further clinical application.

Effects of aflatoxin B1 on growth performance, antioxidant status, immune response, and pro-inflammatory cytokine mRNA expression in ISA chicks.

The research evaluated the effects of Aflatoxin B1 on growth performance, antioxidant status, immune response, and pro-inflammatory cytokine mRNA expression in ISA chicks. In total, 240 7-day-old ISA chicks were randomly assigned to four treatment groups. The control group comprised chicks fed a basal diet. The aflatoxin (AFB1)-treatment groups (T1, T2, and T3) comprised chicks fed the basal diet supplemented with AFB1 at concentrations of 5, 8, and 10 µg/kg, respectively. The growth performance, antioxidant status, immune responses, and pro-inflammatory cytokine mRNA expression in all groups were measured. In the T1 treatment group (receiving the lowest AFB1 dose), a reduction in the Newcastle disease virus antibody (NDV-Ab) titer, and increases in interleukin 2 (IL-2), IL-6, and interferon γ (IFN-γ) mRNA levels were observed on days 21 and 42 (P < 0.05). Treatment with the higher AFB1 doses (groups T2 and T3) reduced the chicks' growth performance on days 21 and 42, measured as reductions in body weight (BW) and average daily gain (ADG) compared with the control group. In the T2 and T3 groups, the total antioxidant capacity (T-AOC), glutathione peroxidase (GPX) and superoxide dismutase (SOD) activities, serum immunoglobulin A (IgA) and IgG levels, and IL-2, IL-6, and IFN-γ levels were also lower than in the control group. On days 21 and 42, these two groups also showed increased malondialdehyde (MDA) content, higher feed to gain ratio (F/G), and higher IL-2, IL-6, and IFN-γ mRNA levels than the control group (P < 0.05). The T2 and T3 groups also showed reduced T-AOC, NDV-Ab titer, IL-2 content, and GPx-1 mRNA levels on days 21 and 42 (P < 0.05), increased IL-6 and IFN-γ mRNA levels on day 21, and increased F/G and MDA content on day 42 (P < 0.05) compared with group (T1). Increased MDA content and IL-6 mRNA levels in the liver and ileum were observed in group T3 compared with group T2 on day 21, and lower IgM and IL-6 levels were observed on days 21 and 42 (P < 0.05). In conclusion, our data showed that AFB1 exposure resulted in dose-dependent oxidative and inflammatory damage, immunosuppression, and a decline in the growth performance of chicks.

SLC26A4 mutation in Pendred syndrome with hypokalemia: A case report.

RATIONALE: Pendred syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss, inner ear malformations, goiter, and abnormal organification of iodide. It is caused by mutations in SLC26A4 gene, which encodes pendrin (a transporter of chloride, bicarbonate, and iodide). Pendred syndrome is a common cause of syndromic deafness, but the metabolic abnormalities it causes are often overlooked. Here, we report the case of a patient diagnosed with Pendred syndrome with hypokalemia. PATIENT CONCERNS: A 53-year-old deaf-mute woman was hospitalized due to severe limb asthenia. The emergency examination showed that her blood potassium level was 1.8 mmol/L. DIAGNOSES: Through the genetic test, we found a mutation of SLC26A4 gene in NM_000441: c.2027T>A, p.L676Q, as well as the SLC26A4 exon 5-6 deletion. These genetic variations pointed to Pendred syndrome (an autosomal recessive disorder that mainly affects the inner ear, thyroid, and kidney) which is a common cause of syndromic deafness. INTERVENTIONS: The patient was treated with potassium supplements and screened for the cause of hypokalemia. OUTCOMES: The patient was discharged after her potassium levels rose to the normal range. LESSONS: Patients with Pendred syndrome may also have certain metabolic abnormalities; thus, more attention should be paid to them during clinical diagnosis.

[Clinical Characteristic, Diagnosis and Treatment of Acute Lymphoblastic Leukemia Combined with Pneumocystis Carinii Pneumonia in Children].

OBJECTIVE: To investigate the clinical characteristics and treatment of pneumocystis carinii pneumonia (PCP) in children with acute lymphoblastic leukemia (ALL), in order to improve the early diagnosis and effective treatment. METHODS: Clinical data of five children with ALL developing PCP in the post-chemotherapy granulocyte deficiency phase were analyzed retrospectively. The clinical manifestations, laboratory tests, imaging findings, treatment methods and effect were summarized. RESULTS: The male-to-female ratio of the five children was 1∶4, and the median age was 5.5 (2.9-8) years old. All patients developed PCP during granulocyte deficiency phase after induction remission chemotherapy. The clinical manifestations were generally non-specific, including high fever, tachypnea, dyspnea, non-severe cough, and rare rales in two lungs (wet rales in two patients). Laboratory tests showed elevated C-reactive protein (CRP), serum procalcitonin (PCT), (1,3)-ß-D-glucan (BDG), lactate dehydrogenase (LDH) and inflammatory factors including IL-2R, IL-6 and IL-8. Chest CT showed diffuse bilateral infiltrates with patchy hyperdense shadows. Pneumocystis carinii(PC) was detected in bronchoalveolar lavage fluid (BALF) or induced sputum by high-throughput sequencing in all patients. When PCP was suspected, chemotherapy was discontinued immediately, treatment of trimethoprim-sulfame thoxazole (TMP-SMX) combined with caspofungin against PC was started, and adjunctive methylprednisolone was used. Meanwhile, granulocyte-stimulating factor and gammaglobulin were given as the supportive treatment. All patients were transferred to PICU receiving mechanical ventilation due to respiratory distress during treatment. Four children were cured and one died. CONCLUSION: PCP should be highly suspected in ALL children with high fever, dyspnea, increased LDH and BDG, and diffuse patchy hyperdense shadow or solid changes in lung CT. The pathogen detection of respiratory specimens should be improved as soon as possible. TMP/SMZ is the first-line drug against PCP, and the combination of Caspofungin and TMP/SMZ treatment for NH-PCP may have a better efficacy. Patients with moderate and severe NH-PCP may benefit from glucocorticoid.

Effect of omentin-1 on cancer stem cell surface markers and tumour-suppressive miRNA expression in a high-glucose environment associated with colorectal cancer.

Objective: To investigate how omentin-1 impacts colorectal cancer stem cell surface markers and the expression levels of tumour-suppressive micro ribonucleic acid in a colorectal cancer-associated high-glucose environment. METHODS: The study was conducted in the First Affiliated Hospital of Anhui Medical University，Anhui, China，from April 2018 to January 2019 and comprised cluster of differentiation133 and colorectal cancer stem cells from the SW480 cell line（the human colon adenocarcinoma cell line） obtained through immunomagnetic beads-based cell isolation. The colon cancer stem cells were divided into 6 groups: Z0 (control), Z1 (1ug/mL omentin-1), Z2 (2ug/mL omentin-1), G0 (5.0g/mL glucose), G1 (1ug/mL omentin-1 and 5.0g/mL glucose), and G2 (2ug/mL omentin-1 and 5.0 g/mL glucose). After 24 hours of intervention, quantitative polymerase chain reaction and western blot test were used for the detection of messenger ribonucleic acid and protein levels of stem cell surface markers. The colorectal cancer stem cells were divided into three groups: the control group, omentin group 1 (1ug/mL omentin-1) and omentin group 2 (2ug/mL omentin-1). After 24 hours of intervention, the expression of tumour suppressor micro ribonucleic acid was measured using quantitative polymerase chain reaction. Data was analysed using SPSS 23. RESULTS: Compared to the Z0 group, cluster of differentiation133 messenger ribonucleic acid expression reduced sharply in Z1 group (p<0.05), while Z2 group saw a marked increase in the expression (p<0.05). With respect to tumour-suppressive micro ribonucleic acid expression, micro ribonucleic acid 126, 145, 34a and 342-5P in omentin group 2 exhibited an expression level significantly higher than those in the control group and the omentin group 1 (p<0.05). Conclusion: High glucose levels were found to upregulate the expression of colorectal cancer stem cell surface markers cluster of differentiation133 messenger ribonucleic acid and protein. Also, omentin-1 was found to be associated with the downregulation of cluster of differentiation133 messenger ribonucleic acid and protein expression and the upregulation of cluster of differentiation 44 messenger ribonucleic acid expression in a high-glucose environment. Finally, omentin-1 was found to have the ability to promote the expression of relevant tumour-suppressive micro ribonucleic acids 126, 14, 34a and 342-5P.

Indicators of glucose dysregulation and the relationship with iron overload in Chinese children with beta thalassemia major.

INTRODUCTION: Patients with beta thalassemia major (TM) have a higher risk of diabetes and an abnormal oral glucose tolerance test (OGTT), but there is no single agree monitoring parameter that reflects glycemic status. The possible mechanisms include iron overload and blood transfusion, but they require further investigation. PURPOSE: This study explored the role of glycated hemoglobin A1c (HbA1c), fructosamine, and glycated albumin (GA) in evaluating the glucose dysregulation and to determine the potential relationship between iron deposition and glucose metabolism disorder in beta TM. METHODS: A cross-sectional study was performed on 118 patients with beta TM and the control group consisted of 33 healthy children with no statistical differences in age, sex, and body mass index (BMI). Fast plasma glucose (FPG), fast insulin (FINS), insulin resistance index (HOMA-IRI), and insulin sensitivity index (HOMA-ISI) were compared between the patient and control groups. HbA1c, GA, fructosamine, and serum ferritin (SF) were measured in the patient group. OGTT, as well as heart and liver magnetic resonance imaging (MRI) T2*, was performed. For all statistical analyses, SPSS 21.0 was used and p < 0.05 was accepted as statistically significant. RESULTS: FPG, FINS, and HOMA-IRI were significantly increased while HOMA-ISI decreased in the beta TM patients when compared with those in the control group. In patients with beta TM, 17 (14.41%) of patients had been diagnosed with diabetes, while 48 (40.68%) had both impaired fasting glucose and impaired glucose tolerance. HbA1c, GA, and fructosamine were increased according to the degree of abnormal glucose metabolism. Statistically significant differences were found in age, SF, and cardiac T2* between the abnormal and normal OGTT groups. CONCLUSION: HbA1c may be used as a significant measure for monitoring glycemic levels in patients with beta TM. Furthermore, GA and fructosamine were alternative indicators of glucose status. Patients with heart iron deposition or an SF > 4000 µg/L were prone to abnormal glucose metabolism, so chelation therapy should be reinforced.

Selenide Chitosan Sulfate Improved the Hepatocyte Activity, Growth Performance, and Anti-oxidation Capacity by Activating the Thioredoxin Reductase of Chickens In Vitro and In Vivo.

Chicken hepatocytes were cultured in vitro and 240 specific pathogen-free (SPF) white leghorns chickens (7 days old) were obtained. The hepatocytes and chickens were randomly allocated to one of six treatment groups: control group; chitosan (COS) group; sodium selenite (Na2SeO3) group; selenide chitosan (COS-Se) group; chitosan sulfate (LS-COS) group; and selenide chitosan sulfate (LS-COS-Se) group. Our results showed that LS-COS-Se increased (P < 0.05) the activities of thioredoxin reductase (TXNRD), anti-superoxide anion radical (antiO2-), and superoxide dismutase (SOD), the mRNA levels of thioredoxin reductase 1 (TXNRD1) and thioredoxin reductase 3 (TXNRD3), and the chicken body weight, but reduced (P < 0.05) the malondialdehyde (MDA) content and the lactate dehydrogenase (LDH) activity. Compared with COS and LS-COS, the LS-COS-Se treatment increased (P < 0.05) the activities of TXNRD, SOD, catalase (CAT), and the mRNA levels of TXNRD1 and TXNRD3, but reduced (P < 0.05) the MDA content in vitro, whereas, in vivo, it increased (P < 0.05) body weight on day 28; the activities of TXNRD, antiO2-, and SOD; and the mRNA levels of TXNRD1 and TXNRD3. Compared with Na2SeO3 and COS-Se, the LS-COS-Se treatment increased (P < 0.05) the TXNRD and SOD activities, the mRNA levels of TXNRD1 and TXNRD3 in vitro, increased (P < 0.05) the chicken body weight on day 28, and the TXNRD, antiO2-, and SOD activities, but reduced (P < 0.05) the MDA content. These results indicated that LS-COS-Se was a useful antioxidant that improved hepatocyte activity, growth performance, and anti-oxidation capacity in hepatocytes (in vitro) and SPF chicken (in vivo) by activating the TXNRD system.

Long-term follow-up of a case of MEN1 and literature review.

OBJECTIVE: To investigate the diagnosis and treatment of Multiple Endocrine Neoplasia Type 1 (MEN1), improve our understanding of the disease and highlight the importance of life-long follow-up of affected individuals. METHODS: A retrospective analysis was performed on 1 MEN1 patient with long-term follow-up at the First Affiliated Hospital of Anhui Medical University. RESULTS: A 51-year-old woman was diagnosed with MEN1 14 years ago, but exhibited a suspected disease course of at least 20 years. Prior to admission, the patient reported a cough lasting for two months. The patient's thyroid hormone, sex hormone, insulin, cortisol, parathyroid hormone, and ACTH circadian rhythm findings were within normal ranges. The patient exhibited elevated blood calcium levels. Examination led to the detection of thymoma and pancreatic neoplasms, whereas no obvious abnormalities were detected in her parathyroid gland or adrenal gland as determined via computed tomography (CT). Genetic analyses revealed a mutation in the MEN1 gene in this patient. As the patient had no relevant clinical symptoms, she refused surgical treatment, and follow-up was continued. It was learned through follow-up that the patient underwent anterior mediastinal lesion resection and partial rib resection in June 2020 because she re-examined the chest CT showed that the anterior mediastinal mass was significantly larger than that in 2019. Pathology suggested neuroendocrine tumors. The patient is currently recovering well. CONCLUSION: MEN1 is an uncommon condition in clinical settings, and it is important that clinicians be made aware of this disorder so that they can provide patients with appropriate and timely treatments.

Gender roles and behavioral problems in children with 21-hydroxylase deficiency in Southern China.

PURPOSE: To assess the gender roles and behavioral outcomes in children with 21-hydroxylase deficiency (21-OHD) in Southern China. METHODS: A total of 50 individuals with 21-OHD participated in our study, (30 boys and 20 girls), as well as another 19 age-matched non-affected relatives of patients (12 boys and 7 girls). Psychological adjustment was assessed with a preschool activity survey and a Conner parent symptom questionnaire was modified for retrospective reporting. RESULTS: The response rate of the questionnaire in the control group was only 36.5%. All the patients were diagnosed with salt-wasting of 21-OHD. Our study revealed that the masculine score was higher in male patients with 21-OHD than male controls and female patients. Compared with that in the female 21-OHD patient group, the masculine score in the female control group was lower, while comparative masculinization was found in the male controls. Regarding behavioral problems, there was a higher incidence of parent-reported problems among children with 21-OHD than controls, including conduct problems, impulsive hyperactivity, anxiety, and hyperactivity index. CONCLUSION: Parents of 21-OHD patients in Southern China were unwilling to disclose the condition of their children to the society. Masculinization and behavioral problems were prevalent among patients with 21-OHD, which highlighted the importance of psychological and social support for 21-OHD patients and their families.

Antithyroid arthritis syndrome: A case report and review of the literature.

A 29-year-old woman was diagnosed with Graves' disease because of her symptoms of thyrotoxicosis. After a 15-day treatment with methimazole, she began to suffer from a repeated fever, rash, and polyarticular migratory arthralgias. The clinical examination on admission showed that her white blood cell count, neutrophil count, and erythrocyte sedimentation rate (ESR) were within normal limits, while the concentration of C-creative protein (CRP) was 26.14 mg/L (ref. 0 - 10) and anti-nuclear immune body (ANA) and anti-neutrophil cytoplasmic antibody (ANCA) were both negative. Upon stopping the drug treatment, the patient's symptoms promptly disappeared. Antithyroid arthritis syndrome is poorly characterized, and the findings from our literature review indicate that this syndrome exhibits serological features that are distinct from those of antithyroid agent-induced vasculitis syndrome. Furthermore, physician's awareness of this syndrome is essential for its diagnosis in clinical practice.

Selenium-enriched Saccharomyces cerevisiae improves the meat quality of broiler chickens via activation of the glutathione and thioredoxin systems.

The aim of this study was to investigate the effects of selenium (Se)-enriched Saccharomyces cerevisiae (SSC) on meat quality and to elucidate the underlying mechanisms in broilers. A total of 200 one-day-old Arbor Acres broiler chickens were randomly allocated to one of four treatments with 5 replications of 10 chickens each. Group 1 served as a control and was fed a basal diet without Se supplementation, while groups 2, 3, and 4 were fed the basal diet supplemented with S. cerevisiae (SC), sodium selenite (SS), and SSC, respectively. Breast muscle samples were collected to evaluate meat quality, selenium concentration, oxidative stability, and the mRNA levels of antioxidant enzyme genes on day 42. As compared with groups 1 and 2, SS and SSC supplementation increased Se concentration, glutathione peroxidase (GPx) and thioredoxin reductase (TR) activities, total antioxidant capacity, and the mRNA levels of GPx-1, GPx-4, TR-1, and TR-3 (P < 0.05) and decreased drip loss and malondialdehyde (MDA) content (P < 0.05). As compared with group 3, SSC supplementation increased pH, lightness, yellowness, Se concentration, GPx and superoxide dismutase activities, and the mRNA levels of GPx-1 and GPx-4 (P < 0.05) but decreased drip loss and MDA content (P < 0.05). Thus, SSC improved meat quality and oxidative stability by activating the glutathione and thioredoxin systems, which should be attributed to the combined roles of Se and SC.

Effects of selenide chitosan sulfate on glutathione system in hepatocytes and specific pathogen-free chickens.

This study aimed to investigate the effects of selenide chitosan sulfate (Se-CTS-S) on glutathione (GSH) system in hepatocytes and chickens. Chitosan, sodium selenite (Na2SeO3), selenide chitosan, chitosan sulfate (CTS-S), and Se-CTS-S were added to the culture medium and the basal diets; glutathione peroxidase (GSH-Px) activity, GSH content, total antioxidant capacity (T-AOC), and mRNA levels of cellular GPx (GPx-1) and phospholipid hydroperoxide GPx (GPx-4) in vivo and in vitro were determined. The results showed that Se-CTS-S increased (P < 0.05) GPx-1 and GPx-4 mRNA levels in hepatocytes and livers, and GSH-Px activity, GSH content, and T-AOC in the medium, hepatocytes, plasma, and livers compared with the control and chitosan treatments. Compared with CTS-S, Se-CTS-S treatments increased (P < 0.05) GPx-1 and GPx-4 mRNA levels in hepatocytes and livers, and GSH-Px activity, GSH content, and T-AOC capacity in the medium, hepatocytes, and livers. Compared with Na2SeO3 and CTS-Se, Se-CTS-S increased (P < 0.05) GPx-1 mRNA levels in hepatocytes and livers, GPx-4 mRNA levels in hepatocytes and livers, GSH-Px activity in the medium, hepatocytes, and livers, GSH contents in plasma and livers, and T-AOC in the medium, plasma, and livers. Thus, Se-CTS-S showed better biological activity that mainly benefited from the synergistic effects of Se and sulfate on GSH system.

A mitophagic response to iron overload-induced oxidative damage associated with the PINK1/Parkin pathway in pancreatic beta cells.

BACKGROUND: Iron overload can result in a disorder in glucose metabolism. However, the underlining mechanism through which iron overload induces beta cell death remains unknown. METHODS: According to the concentration of ferric ammonium citrate (FAC) and N-acetylcysteine, INS-1 cells were randomly divided into four groups: normal control (FAC 0 µM) group, FAC 80 µM group, FAC 160 µM group, FAC 160µM + NAC group. Cell proliferation was assessed by Cell Counting Kit-8. Reactive oxygen species (ROS) level was further evaluated using flow cytometer with a fluorescent probe. The mitochondrial membrane potential was detected by JC-1 kit, and transmission electron microscopy was used to observe the mitochondrial changes. The related protein expressions were detected by western bolt to evaluate mitophagy status. RESULTS: It was shown that FAC treatment decreased INS-1 cell viability in vitro, resulted in a decline in mitochondrial membrane potential, increased oxidative stress level and suppressed mitophagy. Furthermore, these effects could be alleviated by the ROS scavenger. CONCLUSIONS: We proved that increased iron overload primarily increased oxidative stress and further suppressed mitophagy via PTEN-induced putative kinase 1/Parkin pathway, resulting in cytotoxicity in INS-1 cells.

Biosafety, and improvement of osteoporosis in cage layers through using chOPG protein.

Thirty six 56-week-old ISA cage layers were divided into two groups randomly. The cage layers in control group (12 birds) and experiment group (24 birds) were respectively injected with 300 µL sodium chloride and 300 µg eucaryon recombinant plasmid pcDNA3.1(+)-chOPG. Eighty 56-week-old ISA cage layers were divided into group A, B, C and D randomly. Group A is for control group, while plasmid pcDNA3.1(+)-chOPG was injected to B, C, D groups in muscle at the dosage of 200 µg, 400 µg, 600 µg at 57, 59, 61, 63th weeks respectively. After the detection on the expression of chOPG protein after 3 h, it reached the peak at 7 d and then fell down. After 28 d, nothing was detected in the injected skeletal muscles. The other organs did not express exogenous chOPG protein. Plasmid in liver had the fastest metabolism. The pathological effects in main organs were not observed by histological section. The concentration of plasma calcium in B, C and D groups significantly decreased, while the activity of alkaline phosphatase was significantly improved, compared to control group. The total average value of abnormal and broken eggs of group C, D was significantly higher than those of group A. The bone biomechanical property and bone radiographic density of tibia and femur in experiment group were significantly higher than control group. Therefore, one conclusion is drawn that the expression of chOPG from foreign plasmid pcDNA3.1(+)-chOPG have contribute to bone formation, chOPG can increase bone density and strength by inhibiting bone resorption. Nevertheless, it was cleared out from cellular system in a short-term after intramuscular injection and cannot integrate into host chromosome genomic in cage layers. There were no pathological effects observed in the main tissues. It is believed that 200 µg plasmid pcDNA3.1(+)-chOPG should be within the safe range for application, because it can improve bone metabolism and will not affect the production of cage layer during the post cycle.

Analysis of phenotypes and genotypes in 84 patients with 21-Hydroxylase deficiency in southern China.

OBJECTIVE: 21-hydroxylase deficiency (21-OHD) caused by mutation in CYP21A2 gene is the most common form of Congenital adrenal hyperplasia (CAH). This study aimed to analyze the gene mutation frequency and the phenotype-genotype correlation of 21-OHD patients from southern China. METHOD: The clinical features, laboratory tests and gene mutational analysis of 84 patients with 21-OHD were retrospectively investigated. Subsequently, the correlation between phenotypes and genotypes of these patients was analyzed. RESULTS: 59 of 84 cases of 21-OHD (70.2%) were classified as salt-wasting (SW) forms presenting adrenal crisis or other signs of salt loss at the age between neonatal period and 2 months, and other 25 cases were classified as simple virilizing (SV) forms. Mutations of CYP21A2 gene on both alleles were found in all 84 patients (168 alleles). The most common types of mutations included micro-conversions (129/168, 76.8%), large gene conversions and deletions (23/168, 13.7%), and bona fide point mutations (16/168, 9.5%). In increasing order of frequency, the most common micro-conversions were I2G (41.1%), p.I172N (13.1%), p.R356W (7.7%), p.Q318* (7.7%) and E6 Cluster (3.0%). Genotypes and phenotypes correlated in 86.1% of the patients analyzed. CONCLUSION: Micro-conversions were the most common types of CYP21A2 gene mutations in our study, and the frequency of the identified mutations was not significantly different compared with most other Chinese areas and different ethnic regions. However, fewer large gene conversions and deletions were found compared to studies in other ethnic populations. Genotype-phenotype correlation was found in patients with the SW and SV forms of 21-OHD. This study expanded the number of mutations affecting CYP21A2 gene in Chinese patients with 21-OHD, providing additional information for a precise clinical diagnosis and genetic counseling.