Early manifestation of hypophosphatemic rickets in goslings: a potential role of insufficient muscular adenosine triphosphate in motility impairment of early P-deficient geese.

We aimed to determine the onset time of hypophosphatemic rickets and investigate the mechanism of motility impairment through adenosine triphosphate (ATP) production in goslings. Two hundred and sixteen 1-day-old male Jiangnan white geese were randomly divided into 3 groups, with 6 replicates and 12 geese per replicate. Birds were fed on 3 diets: a control diet (nonphytic phosphorus, NPP, 0.38%), a P-deficient diet (PD; NPP, 0.08%), and a high P diet (HP; NPP, 0.80%) for 14 d. Subsequently, all birds were shifted to the control diet for an additional 14 d. The cumulative incidence of lameness increased significantly (P < 0.01) starting on d 4, reaching over 80% on d 7 and 100% on d 12 in the PD group. Drinking and eating frequency decreased from d 4 and d 5, respectively, in the PD group compared to the other groups (most P < 0.01). The PD group exhibited shorter and narrower beaks, higher (worse) curvature scores of the beak and costochondral junctions, swelling caput costae, and dirtier feathers since d 4, in contrast to the control and HP groups (most P < 0.01). The HP had bigger (P < 0.05) beak and sternum sizes than the control groups on d 4 to 11. Leg muscle ATP levels were lower (P < 0.01 or 0.05) on d 4 to 11; in contrast, adenosine diphosphate (d 7-11) was higher in PD compared to the control (P < 0.05). Leg muscle ATP level had positive linear (R2 > 0.40) correlations (r > 0.60) with eating and drinking frequencies on d 7 and 11 (P < 0.01). Bone stiffness, feather cleanliness, and ATP levels recovered (P > 0.05) to the control level, whereas bone size did not recover (P < 0.05) in PD and HP after eating the control diet for 2 wk. The onset time of hypophosphatemic rickets was around 4 d in goslings, and insufficient leg muscle ATP was related to the impaired motility observed in early P-deficient geese.

Serum IL-17A and IL-6 in paediatric Mycoplasma pneumoniae pneumonia: implications for different endotypes.

Paediatric Mycoplasma pneumoniae pneumonia (MPP) is a heterogeneous disease with a diverse spectrum of clinical phenotypes. No studies have demonstrated the relationship between underlying endotypes and clinical phenotypes as well as prognosis about this disease. Thus, we conducted a multicentre prospective longitudinal study on children hospitalized for MPP between June 2021 and March 2023, with the end of follow-up in August 2023. Blood samples were collected and processed at multiple time points. Multiplex cytokine assay was performed to characterize serum cytokine profiles and their dynamic changes after admission. Cluster analysis based on different clinical phenotypes was conducted. Among the included 196 patients, the levels of serum IL-17A and IL-6 showed remarkable variabilities. Four cytokine clusters based on the two cytokines and four clinical groups were identified. Significant elevation of IL-17A mainly correlated with diffuse bronchiolitis and lobar lesion by airway mucus hypersecretions, while that of IL-6 was largely associated with lobar lesion which later developed into lung necrosis. Besides, glucocorticoid therapy failed to inhibit IL-17A, and markedly elevated IL-17A and IL-6 levels may correlate with lower airway obliterans. Our study provides critical relationship between molecular signatures (endotypes) and clustered clinical phenotypes in paediatric patients with MPP.

Effects of lycopene on the growth performance, meat quality, and antioxidant capacity of broiler chickens challenged with aflatoxin B1.

The present study aimed to investigate the effects of dietary lycopene (LYC) supplementation on the growth performance, meat quality, and antioxidant capacity of breast muscle in aflatoxin B1 (AFB1 )-challenged broilers. A total of 192 1-day-old healthy Arbor Acres broilers were randomly assigned to 3 treatments, each with 8 replicates (8 broilers per replicate). The broilers of the three treatments were fed a basal diet (control), a basal diet supplemented with 100 µg/kg AFB1 (CA), and a basal diet supplemented with 100 µg/kg AFB1 and 200 mg/kg LYC (CAL). The results demonstrated that the AFB1 diet increased the feed-to-gain (F/G) ratio (p < 0.05), yellowness and shear force of breast muscle (p < 0.05), and protein carbonyl (PC) content (p < 0.05) while decreasing the average daily gain (ADG) (p < 0.05), redness of breast muscle (p < 0.05), glutathione peroxidase activity (p < 0.05), and ability to clear OH· from breast muscle (p < 0.05) in comparison to the control group. Dietary LYC supplementation significantly decreased the F/G ratio (p < 0.05), yellowness and shear force (p < 0.05), and the content of PC and hydrogen peroxide (p < 0.05) while significantly increasing the ADG (p < 0.05), redness of breast muscle (p < 0.05), and ability of breast muscle to clear ABTS·+ (p < 0.05) compared to the CA diet. In conclusion, LYC can alleviate the negative impacts of AFB1 on the growth performance, meat quality, and antioxidant capacity of breast muscle in broilers. PRACTICAL APPLICATION: LYC, as a popular antioxidant, is beneficial to the growth and health of animals. The detailed application effects are still being investigated. In this study, by adding LYC to an AFB1 -contaminated diet, it was found that LYC could alleviate the adverse effects of AFB1 on the growth performance, meat quality, and muscle antioxidant capacity of broilers. These findings can provide a reference for the application of LYC and similar plant-derived materials in animal production.

Metabolomic comparison of meat quality and metabolites of geese breast muscle at different ages.

The purpose of this study was to distinguish the effect of age on the meat quality and chemical composition of Yangzhou goose breast meat. Nontargeted metabolomics analysis (UHPLC-MS/MS) was used to distinguish the metabolic composition of goose meat at different ages, and Pearson's correlations between differential metabolites and key meat parameters were assessed. Compared with goslings, adult geese had lighter, redder and chewier meat (p < 0.05). Metabolite analysis revealed significant differences in nucleosides, organic acids, amino acids and sugars. Levels of IMP, xanthosine, pretyrosine and l-threonine were significantly higher in older meat (p < 0.05) and positively correlated with meat freshness indicators. However, pyruvic acid, l-cysteine and glucose 6-phosphate were up-regulated in gosling meat (p < 0.05), which were important flavor compounds. These results facilitate the further investigation of changes in goose meat composition and provide biomarkers for determining goose meat quality at different ages.

[Effect and mechanism of Dahuang Zhechong Pills in improving liver aging in rats by regulating ROS-mediated PI3K/Akt/FoxO4 signaling pathway].

Recent studies have shown that the occurrence and development of common liver diseases, including non-alcoholic fatty liver disease, cirrhosis, and liver cancer, are related to liver aging(LA). Therefore, to explore the effect and mechanism of Dahuang Zhechong Pills(DHZCP), a traditional classic prescription in improving LA with multiple targets, the present study randomly divided 24 rats into a normal group, a model group, a DHZCP group, and a vitamin E(VE) group, with six rats in each group. The LA model was induced by continuous intraperitoneal injection of D-galactose(D-gal) in rats. For the LA model rats, the general situation was evaluated by aging phenotype and body weight(BW). LA was assessed by the pathological characteristics of hepatocyte senescence, hepatic function indexes, the staining characteristics of phosphorylated histone family 2A variant(γ-H2AX), and the expression levels of cell cycle arrest proteins(P21, P53, P16) and senescence-associated secretory phenotype(SASP) in the liver. The activation of the reactive oxygen species(ROS)-mediated phosphatidylinositol-3 kinase(PI3K)/protein kinase B(Akt)/forkhead box protein O4(FoxO4) signaling pathway was estimated by hepatic ROS expression feature and the protein expression levels of the key signaling molecules in the PI3K/Akt/FoxO4 signaling pathway. The results showed that after the treatment with DHZCP or VE for 12 weeks, for the DHZCP and VE groups, the characterized aging phenotype, BW, pathological characteristics of hepatocyte senescence, hepatic function indexes, relative expression of ROS in the liver, protein expression levels of key signaling molecules including p-PI3K, p-Akt, and FoxO4 in the liver, staining characteristics of γ-H2AX, and the protein expression levels of P16, P21, P53, interleukin-6(IL-6), and tumor necrosis factor-α(TNF-α) in the liver were improved, and the effects of DHZCP and VE were similar. Based on the D-gal-induced LA model in rats, this study demonstrates that DHZCP can ameliorate LA with multiple targets in vivo, and its effects and mechanism are related to regulating the activation of the ROS-mediated PI3K/Akt/FoxO4 signaling pathway in the liver. These findings are expected to provide new pharmacological evidence for the treatment of DHZCP in aging-related liver diseases.

Effective long-term sirolimus treatment in hypoxemia mainly due to intrapulmonary right-to-left shunt in a patient with multiple vascular anomalies.

Pulmonary arteriovenous malformations (PAVMs), particularly where feeding artery/arteries to PAVMs ≥ 3 mm can be treated with embolization. The treatment for hypoxemia resulting from multiple small or diffuse PAVMs remains unclear.We report a girl aged 5 years and 10 months presented with cyanosis and decreased activity after exercise (83-85% of pulse oxygen saturation, SpO2). She had 1 skin lesion on her face and 1 suspected hemangioma on her left upper extremity at birth and that gradually disappeared spontaneously. Physical examination revealed clubbed fingers, and abundant vascular networks on her back. Contrast-enhanced lung CT (slice thickness:1.25 mm) with vascular three-dimensional reconstruction and abdominal CT revealed increased bronchovascular bundles, increased diameter of the pulmonary artery and ascending aorta, and intrahepatic portosystemic venous shunts due to patent ductus venosus. Echocardiography revealed increased diameter of aortic and pulmonary artery. Transthoracic contrast echocardiography was highly positive (bubble appearing in the left ventricle after 5 cardiac cycles). Abdominal doppler ultrasound revealed hepatic-portal venous shunt. Magnetic resonance imaging, artery and vein of the brain revealed multiple malformations of venous sinuses. The patient received sirolimus for 2 years and 4 months. Her condition improved significantly. SpO2 gradually increased to 98%. Her finger clubbing gradually normalized.Our report implicates sirolimus might be a potential treatment option in persistent hypoxemia mainly due to intrapulmonary right-to-left shunt even small multiple or diffusive PAVMs in pediatric patients with multiple cutaneous and visceral vascular anomalies.

A systematic review of prognosis of ABO blood group and rhesus factor on outcomes in patients with bladder cancer.

BACKGROUND: Bladder cancer (BC) is the 10th most common malignancy worldwide, and some studies reported that ABO blood type or/and rhesus factor has been identified as a prognostic oncologic marker for patients with BC. We carried out a systematic review to assess the prognosis of ABO blood group and rhesus factor on outcomes in patients with bladder cancer. METHODS: We searched databases through February 2022 for studies assessing blood group on outcomes in patients with bladder cancer. RESULTS: We included ten studies with 15,204 participants. We found that blood type A is relevant to non-muscle-invasive BC patients treated with transurethral resection of bladder tumor and blood type B patients have a lower incidence of disease recurrence and progression. Blood type O and non-O blood type have not been found to be related to disease recurrence. However, in multivariable analyses, blood type O and non-O blood type are associated with cancer-specific mortality (CSM). Other than that, blood type B doesn't have statistical significance for BC patients accepted radical cystectomy (RC). The same results showed in blood type AB non-muscle-invasive bladder cancer patients treated with RC. CONCLUSIONS: Our study confirmed that a particular association of blood type for prognosis of patients with BC, and ABO blood group antigen expression can be suitable biomarkers for BC. We also found that rhesus factor has no impact on prognosis of BC patients.

Genetic spectrum of Chinese children with cystic fibrosis: comprehensive data analysis from the main referral centre in China.

BACKGROUND AND OBJECTIVES: Cystic fibrosis (CF) is a heterogeneous disease with a diverse genetic spectrum among populations. Few patients with CF of Chinese origin have been reported worldwide. The objective of this study is to characterise the genotypic features of CF in Chinese children. METHODS: We recruited and characterised the genetic manifestations of 103 Chinese children with CF in Beijing Children's Hospital from 2010 to 2022. Whole-exome sequencing were performed to define the genotypes. Meanwhile, other 99 genetically confirmed patients with Chinese origin described in 45 references were also summarised. RESULTS: 158 different variants including 23 novel observations were identified after sequencing. The majority of CFTR variants (82.3%) in Chinese have been observed only once or twice. 43.7% of the variants were only identified in patients of Chinese origin. The c.2909G>A(p.Gly970Asp), c.1766+5G>T and c.1657C>T(p.Arg553X) were the most frequent variants among Chinese patients, with allele frequency of 12.1%, 5.4% and 3.6%, respectively. The first two variants both showed significant Chinese ethnic tendency, while the latter one most likely came from Europeans for historical reasons. They also demonstrated significant differences in geographical distribution. c.1521_1523delCTT(p.F508del) was rarely observed in patients of pure Chinese origin, with an allele frequency of 1.8%. Two de novo variants (c.960dupA[p.Ser321IlefsX43] and c.2491-2A>G) and two deep-intronic variants (c.3718-2477C>T and c.3874-4522A>G) were identified, which were also quite rare among Chinese. CONCLUSIONS: The genetic spectrum of CF in Chinese is unique and quite different from that observed in Caucasians. The geographical distributions of the most frequent variants were reported for the first time.

The effect of different dietary levels of sodium and chloride on performance, blood parameters and excreta quality in goslings at 29 to 70 days of age.

The purpose of this study was to ascertain the appropriate levels of dietary sodium (Na+ ) and chloride (Cl- ) for 29- to 70-day-old goslings and to investigate the effects of different levels of Na+ and Cl- on the growth performance, water consumption, blood parameters and excreta quality of goslings to provide a reference for the healthy production of goslings. In Experiment 1, a total of 432 29-day-old male Jiangnan White goslings were randomly allotted to nine treatments according to a 3 × 3 factorial design, with six pens containing eight birds per treatment. The goslings were fed diets with three concentrations of Na+ (0.10%, 0.15% and 0.20%) and three concentrations of Cl- (0.15%, 0.20% and 0.25%). The experimental period was 42 days. In Experiment 2, a total of 24 70-day-old Jiangnan White goslings were selected for four treatments (0.10% Na+  × 0.15% Cl- ; 0.10% Na+  × 0.25% Cl- ; 0.20% Na+  × 0.15% Cl- and 0.20% Na+  × 0.25% Cl- ) and housed separately in metabolic cages. The faeces were collected for 3 consecutive days. In Experiment 1, the average daily feed intake (ADFI), average daily gain (ADG) and feed/gain (F/G) ratio of goslings were unaffected by the treatments. However, low levels of Na+ and Cl- significantly reduced the water consumption of goslings in the later growth period (p < 0.05). The average water consumption of goslings fed with 0.10% Na+  × 0.15% Cl- was significantly lower than that of the goslings fed with 0.20% Na+  × 0.25% Cl- (56 days, 1304.2 ml vs. 1471.7 ml; 63 days, 1452.8 ml vs. 1610.8 ml; 70 days, 1540.0 ml vs. 1775.4 ml; p < 0.05). The interaction between Na+ and Cl- (Na+  × Cl- ) had a significant impact on the blood haemoglobin (HGB) and haematocrit (HCT) levels in the goslings (p < 0.05). HGB increased linearly with increasing levels of Na+ . HGB and HCT first increased and then decreased with increasing levels of Cl- . In Experiment 2, Na+ and Cl- levels had significant effects on the excreta moisture content (p < 0.05). Goslings fed with 0.10% Na+  × 0.15% Cl- had a low moisture content of 5.58% compared to the goslings fed with 0.20% Na+  × 0.25% Cl- (87.51% vs. 93.09%; p < 0.05). The levels of dietary Na+ had a significant effect on the retention ratio of Na (p < 0.05), with the value for the 0.20% Na+ group being significantly higher than that for the 0.10% Na+ group (p < 0.05). In summary, different levels of Na+ and Cl- did not affect the growth of goslings. To reduce the water consumption and moisture content of excreta, the Na+ and Cl- levels in the diet can be as low as 0.10% and 0.15%, respectively.

Pilot study of archimedes virtual bronchoscopic navigation system-guided biopsy to diagnose lung nodules in children.

Background: Peripheral pulmonary lesions are uncommon in children. Bronchoscopy is a minimal invasive method to obtain a diagnostic lung biopsy. However, due to the lack of effective guidance methods, the diagnostic efficacy of transbronchial lung biopsy for peripheral solitary pulmonary diseases is still limited. Research question: Is the Archimedes virtual bronchoscopic navigation system safe and effective for the diagnosis of peripheral pulmonary lesions in children? Study design and methods: This pilot study retrospectively analyzed the clinical features, radiological characteristics, operation processes, intra-and postoperative complications, and pathological results of five children who underwent Archimedes-guided biopsy of peripheral pulmonary lesions in Beijing Children's Hospital from May 2021 to May 2022. Results: The cohort comprised five children (all males) with age of 7.1-15.8 years. A guide sheath was inserted through the bronchoscope under the guidance of Archimedes combined with radial endobronchial ultrasound to complete the biopsy under general anesthesia. The fused fluoroscopy technique was used to reconfirm the location of the forceps prior to biopsy in all children. The forceps reached the lesion under the guidance of the navigation and the samples were collected successfully in all children. Pathological examination of the biopsy specimens showed Epstein-Barr virus infection-related lymphoproliferative disease in one child, pulmonary metastasis of rhabdomyosarcoma in one child, and pulmonary vasculitis in one child; high-throughput sequencing of the biopsy tissue sample identified Mycobacterium tuberculosis (sequence no. 80) in one child and Aspergillus (sequence no. 40) in another child. All five children tolerated the biopsy procedure without developing postoperative complications, such as pneumothorax and hemoptysis. Interpretation: Archimedes-guided bronchoscopic lung biopsy is a feasible and efficient way to diagnose peripheral pulmonary lesions in children with manageable complications.

Prognosis and treatment of 46 Chinese pediatric cystic fibrosis patients.

BACKGROUND: Since public awareness of cystic fibrosis (CF) has increased, more children have been diagnosed with CF in China. This study aimed to investigate medical and other challenges faced by pediatric CF patients in China. METHOD: Treatments and treatment outcomes were retrospectively analyzed for 46 pediatric CF patients diagnosed from August 2009 to June 2019. Pre- and post-treatment results were compared using independent samples t-test. RESULTS: Of 46 pediatric CF study patients, four died and five were lost to follow-up. Thirty-seven patients were monitored for 0.03 to 9.21 years; patients exhibited fewer attacks of respiratory tract infections after diagnosis (4.49 ± 2.13 episodes/year before diagnosis vs 1.97 ± 1.87 times/year after 1-year treatment, p < 0.05), significantly reduced sputum production and experienced 1.62 ± 1.71 exacerbations/year. Patient mean body mass index was 16.87 ± 3.53 and pancreatic malfunction persisted in 15 patients. For 17 children, no significant differences in lung function were found at follow-up as compared to lung function at diagnosis (FEV1: 82.45% ± 16.56% vs 75.26% ± 22.34%, FVC: 87.18% ± 13.64% vs 86.99% ± 19.95%, FEF75%: 46.51% ± 28.78% vs 36.63% ± 24.30%, P = 0.27, 0.97, 0.20, respectively). Pseudomonas aeruginosa (17/27) and bronchiectasis (22/22) were found during follow-up evaluation. Twenty-four patients (64.8%) maintained good adherence to therapies. Overall, azithromycin and tobramycin treatments were administered for 0.5-62 months and 0.5-48 months, respectively, and triggered no obvious adverse reactions. CONCLUSION: No obvious declines in clinical presentation or lung function were found in Chinese pediatric CF patients after receiving standard therapeutic and active treatments, although malnutrition and low compliance were persistent challenges.

Clinical significance of interventional therapeutic bronchoscopy combined with bronchial arterial embolization in the treatment of hypervascular primary airway tumors in children.

IMPORTANCE: Pediatric hypervascular primary airway tumors are progressive, fatal lesions with a low incidence, and the disease is often more serious than that in adults. OBJECTIVE: To evaluate the clinical efficacy and safety of interventional therapeutic bronchoscopy combined with conservative treatment and bronchial arterial embolization in children with primary airway tumors. METHODS: We retrospectively analyzed the clinical data of four pediatric patients with hypervascular primary airway tumor between 2017 and 2019 at Beijing Children's Hospital. RESULTS: Two patients were low-grade bronchial mucoepidermoid carcinoma, one patient was pleomorphic adenoma, and one was bronchial leiomyoma. Interventional therapeutic bronchoscopy combined with bronchial arterial embolization was used for treatment (all four patients received general anesthesia). The tumors were safely resected in all patients via interventional bronchoscopy. There were no severe complications related to the procedures. All patients were followed up for 5-12 months, and one low-grade bronchial mucoepidermoid carcinoma recurred. INTERPRETATION: Interventional therapeutic bronchoscopy combined with bronchial arterial embolization appears to be a safe and efficient therapeutic method associated with less trauma and fewer complications, including no serious adverse events, in children with hypervascular primary airway tumors without bronchus wall infiltration.

Clinical and Genetic Spectrum of Children With Primary Ciliary Dyskinesia in China.

BACKGROUND: Primary ciliary dyskinesia (PCD) is a heterogeneous disease with a diverse clinical and genetic spectrum among populations worldwide. Few cases of pediatric PCD have been reported from China. RESEARCH QUESTION: What are the clinical and genotypic characteristics of children with PCD in China? STUDY DESIGN AND METHODS: Clinical characteristics, laboratory findings, and genetic results obtained for 75 patients with PCD were reviewed retrospectively at a single center in China. Genetic sequencing was conducted using whole-exome screening. RESULTS: Patient median age at diagnosis was 7.0 years (range, 2 months-14 years). Of 75 patients, 88% (66/75) had chronic wet cough, 77% (58/75) had recurrent sinusitis, 76% (57/75) had bronchiectasis, 40% (30/75) had neonatal respiratory distress, and 28% (21/75) had coexistent asthma. Notably, postinfectious bronchiolitis obliterans (PIBO) as first presentation was found in 8% of children (6/75). Genes with the highest incidence of mutations were DNAH11 (15/51), followed by DNAH5 (9/51), CCDC39 (5/51), DNAH1 (4/51), and CCNO (3/51). Four genes (DNAI1, HEATR2, RSPH9, and DNAAF3) each were respectively found in two patients, and seven genes (CCDC40, LRRC6, SPAG1, RSPH4A, ARMC4, CCDC114, and DNAH14, a novel gene) each were mutated once. No differences in classical clinical features were observed among patients with commonly observed PCD-associated genotypes. However, three of six PIBO patients carried DNAH1 mutations. INTERPRETATION: Besides typical clinical features, PIBO was observed as the first presentation of pediatric PCD in China. An association of the novel gene DNAH14 with PCD was observed, expanding the PCD genotypic spectrum.

Identification of a Novel Cancer Stemness-Associated ceRNA Axis in Lung Adenocarcinoma via Stemness Indices Analysis.

The aim of this study was to identify a novel cancer stemness-related ceRNA regulatory axis in lung adenocarcinoma (LUAD) via weighted gene coexpression network analysis of a stemness index. The RNA sequencing expression profiles of 513 cancer samples and 60 normal samples were obtained from the TCGA database. Differentially expressed mRNAs (DEmRNAs), lncRNAs (DElncRNAs), and miRNAs (DEmiRNAs) were identified with R software. Functional enrichment analysis was conducted using DAVID 6.8. The ceRNA network was constructed via multiple bioinformatics analyses, and the correlations between possible ceRNAs and prognosis were analyzed using KaplanMeier plots. WGCNA was then applied to distinguish key genes related to the mRNA expression-based stemness index (mRNAsi) in LUAD. After combining the weighted gene coexpression and ceRNA networks, a novel ceRNA regulatory axis was identified, and its biological functions were explored in vitro and vivo. In total, 1,825 DElncRNAs, 291 DEmiRNAs, and 3,742 DEmRNAs were identified. Functional enrichment analysis revealed that the DEmRNAs might be associated with LUAD onset and progression. The ceRNA network was constructed with 14 lncRNAs, 10 miRNAs, and 52 mRNAs. KaplanMeier analysis identified 2 DEmiRNAs, 5 DElncRNAs, and 41 DEmRNAs with remarkable prognostic power. One gene (MFAP4) in the ceRNA network was found to be closely related to mRNAsi by using WGCNA. Functional investigation further confirmed that the C8orf34-as1/miR-671-5p/MFAP4 regulatory axis has important functions in LUAD cell migration and stemness. This study provides a deeper understanding of the lncRNAmiRNAmRNA ceRNA network and, more importantly, reveals a novel ceRNA regulatory axis, which may provide new insights into novel molecular therapeutic targets for inhibiting LUAD stem characteristics.

Effects of lycopene on abdominal fat deposition, serum lipids levels and hepatic lipid metabolism-related enzymes in broiler chickens.

OBJECTIVE: The present study was conducted to investigate the effects of lycopene on growth performance, abdominal fat deposition, serum lipids levels, activities of hepatic lipid metabolism related enzymes and genes expression in broiler chickens. METHODS: A total of 256 healthy one-day-old male Arbor Acres broiler chicks were randomly divided into four groups with eight replicates of eight birds each. Birds were fed basal diet supplemented with 0 (control), 100, 200, and 400 mg/kg lycopene, respectively. RESULTS: Dietary 100 mg/kg lycopene increased the body weight at 21 day of age compared to the control group (p<0.05). Compared to the basal diet, broilers fed diet with 100 mg/kg lycopene had decreased abdominal fat weight, and broilers fed diet with 100 and 200 mg/kg lycopene had decreased abdominal fat percentage (p<0.05). Compared to control, diets with 100, 200, and 400 mg/kg lycopene reduced the levels of total triglyceride and total cholesterol in serum, and diets with 100 and 200 mg/kg lycopene reduced the level of serum low density lipoprotein cholesterol (p<0.05). The activity of fatty acid synthase (FAS) in 400 mg/kg lycopene treated broilers and the activity of acetyl-CoA carboxylase (ACC) in 100, 200, and 400 mg/kg lycopene treated broilers were lower than those fed basal diet (p<0.05). Lycopene increased the mRNA abundance of adenosine monophosphate activated protein kinase α (AMPK-α), whereas decreased the mRNA abundance of sterol regulatory elementbinding protein 1, FAS, and ACC compared to the control group (p<0.05). CONCLUSION: Dietary lycopene supplementation can alleviate abdominal fat deposition and decrease serum lipids levels, possibly through activating the AMPK signaling pathway, thereby regulating lipid metabolism such as lipogenesis. Therefore, lycopene or lycopenerich plant materials might be added to poultry feed to regulate lipid metabolism.

The mediation of pigeon egg production by regulating the steroid hormone biosynthesis of pigeon ovarian granulosa cells.

The aim of this study was to determine the molecular mechanism of miR-205b targeting 11ß-hydroxysteroid dehydrogenase type 1 (HSD11B1) on the apoptosis and proliferation of granulosa cells (GC) of pigeons. Our previous studies suggested that HSD11B1 was the target gene of miR-205b and played a key role in steroid hormone biosynthesis and GC development. The adenovirus-miR-205b recombinant virus and adenovirus-cli-miR-205b-sh recombinant virus were generated, verified, and their characteristics determined. The recombinant viruses were used to infect the GC of pigeons, with real time quantitative PCR used to examine the expressions of HSD11B1 and related genes. The HSD11B1 antibody was obtained and verified, and Western blotting was used to detect the protein level of HSD11B1. The Cell Counting Kit-8 assay kit was used to detect cell viability, and the Annexin V-FITC/PI kit was used for the apoptosis assays. The expression of HSD11B1 was significantly lower in the overexpression (OE) than in OE negative control (OE-NC) treatments and significantly higher in short hairpin (SH) than in SH negative control (SH-NC) treatments. The expression levels of cytochrome P4503A5 was significantly higher in SH and lower in OE treatments, and the rhythms of cytochrome P450 aromatase mRNA levels were similar. The mRNA level of cytochrome P450scc in OE was lower than in OE-NC treatments and higher in SH than in SH-NC treatments. The protein expressions of HSD11B1 were decreased in the GC of OE, whereas increased in the SH group. The Cell Counting Kit-8 assay revealed that overexpression of miR-205b significantly suppressed proliferation of the GC of pigeons, whereas interference of miR-205b significantly induced the proliferation of the GC. The overexpression and the interference of miR-205b did not have a significant effect on cell cycle. The overexpression of miR-205b significantly increased the number of apoptotic cells, whereas the interference of miR-205b decreased the number of apoptotic cells. These findings indicated that miR-205b mediated pigeon egg production by regulating the steroid hormone biosynthesis of the pigeon ovarian GC by targeting HSD11B1, which may be useful in increasing pigeon egg production.

Cobalamin C deficiency presenting with diffuse alveolar hemorrhage and pulmonary microangiopathy.

OBJECTIVE: Combined methylmalonic acidemia and homocysteinemia is a genetically heterogeneous disorder of cobalamin (cbl; vitamin B12) metabolism, which consists of five subtypes including cblC, cblD, cblF, cblJ, and cblX deficiencies. The purpose of this study is to summarize new clinical features mainly diffuse alveolar hemorrhage (DAH) in cblC deficiency. METHODS: We made a retrospective analysis of four pediatric patients diagnosed with DAH and pulmonary microangiopathy due to cblC deficiency between January 2017 and December 2018 in Beijing Children's Hospital. RESULTS: This study describes four patients with their ages ranging from 4 years 2 months to 7 years 6 months with cblC deficiency who developed late-onset diffuse lung disease (DLD). Of these, the first three patients presented predominantly with DAH, and the last patient with pulmonary microangiopathy confirmed by thoracoscopic lung biopsy. All patients were accompanied by pulmonary arterial hypertension (PAH), two accompanied by respiratory failure, and two accompanied by moderate megaloblastic anemia. Diffuse ground-glass opacification and poorly defined ground-glass centrilobular nodules were seen on high-resolution computed tomography in one patient and three patients, respectively. All patients were suspected of having idiopathic pulmonary hemosiderosis or interstitial lung disease at other hospitals. All of them received treatment with corticosteroid before admission, but the symptoms did not improve. Moreover, all patients carried compound heterozygous mutations (c.80A>G, c.609G>A) in MMACHC and improved significantly after being treated for cblC deficiency and PAH. CONCLUSIONS: CblC deficiency should be considered in the differential diagnosis of DAH especially with PAH, and pulmonary microangiopathy be the main reason of DLD in these patients.

Selenomethionine Improves Antioxidant Capacity of Breast Muscle in Geese Via Stimulating Glutathione System and Thiol Pool.

The antioxidant capacity of breast muscle in geese fed diets with sodium selenite (SS) or selenomethionine (SeMet) were investigated in the present study. Two hundred healthy 28-day-old male geese were randomly allotted into four groups (one inorganic group and three organic groups) with five replicates per group. Geese in the four groups were fed the basal diet with 0.3 mg selenium (Se)/kg SS, and the basal diet with 0.2, 0.3 and 0.4 mg Se/kg SeMet, respectively. The experiment lasted for 42 days. Diets with SS or SeMet had no significant effect on growth performance of geese. Geese fed diets with SeMet had higher Se content of breast muscle than SS (P < 0.001). Compared to SS, SeMet increased scavenging abilities of 2, 2-azino-bis (3-ethylbenzothiazoline-6-sulfonic acid) diammonium salt free radical, hydroxyl radical and superoxide radical, the concentrations of reduced glutathione (GSH), total thiol and non-protein thiol, as well as the activity of glutathione peroxidase in breast muscle of geese (P < 0.05). Moreover, dietary SeMet reduced the concentrations of reactive oxygen metabolites, malondialdehyde and protein carbonyl in breast muscle of geese compared to SS (P < 0.05). Therefore, SeMet improved the antioxidant capacity of breast muscle in geese, which might be related to the stimulated GSH-system and thiol pool. The recommended inclusion level of SeMet in goose diet is 0.2 mg Se/kg.

Etiologic spectrum of interstitial lung diseases in Chinese children older than 2 years of age.

BACKGROUND: Childhood interstitial lung diseases (ILD) (chILD) refer to a rare heterogeneous group of disorders. Global collaborations have been working on the etiologies and classification scheme of chILD. With the development of medical technologies, some new diseases were identified to be associated with chILD and its etiologic spectrum is expanding. The aim of this study is to describe the etiologic spectrum of chILD in children older than 2 years of age and summarize the approaches to diagnosis of chILD. METHODS: We made a retrospective analysis of children older than 2 years of age with chILD who referred to Beijing Children's Hospital from 21 provinces all over China from 2013 to 2018. After excluding pulmonary infection, congenital heart disease, bronchopulmonary dysplasia, bronchiolitis obliterans and bronchiectasis, 133 patients were included and categorized by etiology. Clinical manifestations, high-resolution computed tomography, laboratory data, genetic data and pathologic findings were all collected and reviewed. RESULTS: Systemic disease associated ILD were the most common causes, accounting for 49.6% of the patients, followed by alveolar structure disorder-associated ILD (27%), exposure related ILD (13.5%), and disorders masquerading as ILD (3.8%). In systemic disease associated ILD, in addition to common etiologies such as vasculitis (10.5%) and connective tissue diseases (9.0%), primary immunodeficiency diseases (PID) associated ILD (9.8%), interstitial pneumonia with autoimmune features (6.8%), and metabolic diseases (6.8%) were not rarely found. Some newly reported etiologies such as STING-associated vasculopathy with onset in infancy, COPA syndrome and STAT3 mutation were included in PID associated ILD. Genetic tests contributed to 15% of the diagnoses which mainly distributed in PID associated ILD, metabolic diseases and surfactant dysfunction disorders, and contributed to the final diagnoses more than lung biopsies (13.5%) and biopsies of rashes or other tissues (12%). CONCLUSIONS: This study first demonstrated an etiologic spectrum of chILD in Chinese children older than 2 years of age and summarized the approaches to diagnosis. The etiologic spectrum of chILD is expanding with more genetic etiologies being recognized.