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1.
Nanoscale Adv ; 6(12): 3220-3228, 2024 Jun 11.
Artículo en Inglés | MEDLINE | ID: mdl-38868834

RESUMEN

Due to the unique and excellent optical performance and promising prospect for various photonics applications, cavity-enhanced superfluorescence (CESF) in perovskite quantum dot assembled superstructures has garnered wide attention. However, the stringent requirements and high threshold for achieving CESF limit its further development and application. The high threshold of CESF in quantum dot superstructures is mainly attributed to the low radiation recombination rate of the quantum dot and the unsatisfactory light field limiting the ability of the assembled superstructures originating from low controllability of self-assembly. Herein, we propose a strategy to reduce the threshold of CESF in quantum dot superstructure microcavities from two aspects: facet engineering optimization of quantum dot blocks and controllability improvement of the assembly method. We introduce dodecahedral quantum dots with lower nonradiative recombination, substituting frequently used cubic quantum dots as assembly blocks. Besides, we adopt the micro-emulsion droplet assembly method to obtain spherical perovskite quantum dot superparticles with high packing factors and orderly internal arrangements, which are more controllable and efficient than the conventional solvent-drying methods. Based on the dodecahedral quantum dot superparticles, we realized low-threshold CESF (Pth = 15.6 µJ cm-2). Our work provides a practical and scalable avenue for realizing low threshold CESF in quantum dot assembled superstructure systems.

2.
Transl Pediatr ; 13(1): 38-51, 2024 Jan 29.
Artículo en Inglés | MEDLINE | ID: mdl-38323179

RESUMEN

Background: Graves' disease (GD) is an autoimmune thyroid disorder. Our previous study has demonstrated a significant decrease in flavone levels among children with GD compared to the control group. Puerarin, a well-known flavonoid with anti-inflammatory and antioxidant properties. We wanted to investigate its potential impact on GD pathogenesis, aiming to determine whether increasing puerarin intake could prevent or delay the onset of GD. Methods: Adenovirus with TSHR-289 subunit was used to establish a GD mice model, and mice were intragastrically administered with puerarin or sterilized water daily. Thyroid function and inflammatory cytokine levels were quantified using ELISA, lymphocyte subsets were analyzed via flow cytometry, oxidative stress (OS) markers were measured with a microplate reader, and the expression of pertinent signaling pathway proteins were assessed by Western blot. Results: The results demonstrated that puerarin treatment significantly decreased thyroxin levels and alleviated thyroid pathological changes in GD mice. Furthermore, the immune imbalance of GD mice was improved, as evidenced by reduced inflammatory indexes, elevated antioxidant levels, and decreased malondialdehyde (MDA) levels compared to untreated GD mice. Puerarin-treated GD mice exhibited significantly lower expressions of heat shock protein (HSP): HSP70, HSP90, phosphorylated extracellular regulated kinases (p-ERK) and phosphorylated protein kinase B (p-AKT) than untreated GD mice. Moreover, low dosage puerarin (400 mg/kg) was associated with a better protective effect than high dosage (1,200 mg/kg). Conclusions: Puerarin may have the potential to mitigate GD by inhibiting inflammatory and OS, through downregulating the expression of HSP70 and HSP90 and suppressing the activation of the PI3K/AKT/ERK signaling pathway. Furthermore, a lower dose exhibited superior protective effects compared to a higher dose.

3.
Light Sci Appl ; 13(1): 34, 2024 Jan 30.
Artículo en Inglés | MEDLINE | ID: mdl-38291038

RESUMEN

The superfluorescence effect has received extensive attention due to the many-body physics of quantum correlation in dipole gas and the optical applications of ultrafast bright radiation field based on the cooperative quantum state. Here, we demonstrate not only to observe the superfluorescence effect but also to control the cooperative state of the excitons ensemble by externally applying a regulatory dimension of coupling light fields. A new quasi-particle called cooperative exciton-polariton is revealed in a light-matter hybrid structure of a perovskite quantum dot thin film spin-coated on a Distributed Bragg Reflector. Above the nonlinear threshold, polaritonic condensation occurs at a nonzero momentum state on the lower polariton branch owning to the vital role of the synchronized excitons. The phase transition from superfluorescence to polariton condensation exhibits typical signatures of a decrease of the linewidth, an increase of the macroscopic coherence as well as an accelerated radiation decay rate. These findings are promising for opening new potential applications for super-brightness and unconventional coherent light sources and could enable the exploitation of cooperative effects for quantum optics.

4.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 40(10): 1292-1295, 2023 Oct 10.
Artículo en Chino | MEDLINE | ID: mdl-37730234

RESUMEN

OBJECTIVE: To analyze the genetic characteristics of a child with Meier-Gorlin syndrome (MGS) due to a homozygous variant of the ORC6 gene. METHODS: A child who was admitted to the Children's Hospital Affiliated to Soochow University on March 25, 2019 due to growth retardation was selected as the study subject. Clinical data of the child was collected. Whole exome sequencing was carried out for the child. Candidate variant was validated by Sanger sequencing and bioinformatic analysis. RESULTS: The child, a 8-year-and-3-month-old male, has featured short stature, small ears, bilateral cryptorchidism and patellar dysplasia. His parents were of first cousins. The child was found to harbor a homozygous c.712A>T (p.K238*) missense variant of the ORC6 gene, which may lead to premature termination of protein translation. Sanger sequencing confirmed that both of his parents were heterozygous carriers. Based on the guidelines from the American College of Medical Genetics and Genomics, the variant was classified as pathogenic (PVS1_Moderate+PM2_Supporting+PM3+PP3+PP4). CONCLUSION: The homozygous c.712A>T (p.K238*) variant probably underlay the MGS in this child.


Asunto(s)
Microtia Congénita , Enanismo , Humanos , Lactante , Masculino , Biología Computacional , Microtia Congénita/genética , Enanismo/genética , Trastornos del Crecimiento/genética , Complejo de Reconocimiento del Origen/genética
5.
Nano Lett ; 23(17): 7797-7804, 2023 Sep 13.
Artículo en Inglés | MEDLINE | ID: mdl-37590122

RESUMEN

Symmetry plays an essential role in the fundamental properties of a physical system. In this work, we report on the realization of tunable single-mode polariton lasing from highly excited Rydberg states via symmetry engineering. By breaking the symmetry of the polaritonic wave function through potential wells and controlling the spatial overlap between the gain region and the eigen mode, we are able to generate single-mode polariton lasing, reversibly and dynamically, from quantized polariton states. Increasing the asymmetry of the potential well, single-mode lasing can be achieved even for the highly excited Rydberg state with a principle quantum number of N = 14. Moreover, as a result of the excellent reservoir-eigen mode overlap and efficient spatial confinement, the threshold of lasing can be reduced up to 6 orders of magnitude, compared with those conventional pumping schemes. Our results present a new strategy toward the realization of thresholdless polariton lasing with dynamical tunability.

6.
Adv Sci (Weinh) ; 10(21): e2301589, 2023 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-37127890

RESUMEN

Cavity-enhanced superfluorescence (CESF) in quantum dot (QD) system is an ultrafast and intense lasing generated by combination of quantum coupling effect and optically stimulated amplification effect, which can provide a new idea for realizing high quality blue light sources and address the limitation of conventional inefficient blue light sources. Modifying halide composition is a straightforward method to achieve blue emission in perovskite QD system. However, the spectral instability introduced by photoinduced halide phase segregation and low coupling efficiency between QDs and optical cavities make it challenging to achieve stable blue CESF in such halide-doped QD system. Herein, long-range-ordered, densely packed CsPbBr2 Cl QD-assembled superlattice microcavities in which the two core issues can be appropriately addressed are developed. The QD superlattice structure facilitates excitonic delocalization to decrease exciton-phonon coupling, thus alleviating photoinduced phase segregation. By combination of theoretical analysis and temperature-dependent photoluminescence (PL) measurements, the underlying photoinduced phase segregation mitigation mechanism in mixed halide superlattices is clarified. Based on the CsPbBr2 Cl QD superlattices with regularly geometrical structures, in which the gain medium can be strongly coupled to the naturally formed microcavity, stable and ultrafast (3 ps) blue CESF with excellent optical performance (threshold ≈33 µJ cm-2 , quality factor ≈1900) is realized.

7.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 40(3): 322-327, 2023 Mar 10.
Artículo en Chino | MEDLINE | ID: mdl-36854408

RESUMEN

OBJECTIVE: To explore the clinical manifestations and genetic characteristics of patients with congenital central hypothyroidism due to variants of IGSF1 gene. METHODS: Clinical data, results of genetic testing, and follow-up of four patients admitted to Children's Hospital of Soochow University during 2017 to 2021 were retrospectively analyzed. RESULTS: All of the four patients were males. Patient 1 had presented neonatal jaundice, patients 2 and 3 were admitted for growth retardation during childhood, and thyroid function test indicated slightly low free thyroxine (FT4), patient 4 was found to have reduced FT4 in the neonatal period. Genetic testing revealed that all of the four patients have harbored pathogenic variants of the IGSF1 gene, which were all inherited from their mothers. The thyroid functions in all patients were well controlled with oral levothyroxine and regular follow-up. CONCLUSION: Pathogenic variants of the IGSF1 gene probably underlay the congenital central hypothyroidism with a variety of clinical manifestations, and genetic testing can facilitate the diagnosis at an early stage.


Asunto(s)
Hipotiroidismo , Niño , Masculino , Recién Nacido , Femenino , Humanos , Estudios Retrospectivos , Hipotiroidismo/genética , Pruebas Genéticas , Madres , Inmunoglobulinas/genética , Proteínas de la Membrana/genética
8.
J Clin Endocrinol Metab ; 108(8): 2078-2086, 2023 Jul 14.
Artículo en Inglés | MEDLINE | ID: mdl-36669772

RESUMEN

CONTEXT: The evidence of long-term polyethylene glycol recombinant human GH (PEG-rhGH) in pediatric GH deficiency (GHD) is limited. OBJECTIVE: This study aimed to examine the effectiveness and safety of long-term PEG-rhGH in children with GHD in the real world, as well as to examine the effects of dose on patient outcomes. DESIGN: A prospective, observational, posttrial study (NCT03290235). SETTING, PARTICIPANTS AND INTERVENTION: Children with GHD were enrolled from 81 centers in China in 4 individual clinical trials and received weekly 0.2 mg/kg/wk (high-dose) or 0.1 to <0.2 mg/kg/wk (low-dose) PEG-rhGH for 30 months. MAIN OUTCOMES MEASURES: Height SD score (Ht SDS) at 12, 24, and 36 months. RESULTS: A total of 1170 children were enrolled in this posttrial study, with 642 patients in the high-dose subgroup and 528 in the low-dose subgroup. The Ht SDS improved significantly after treatment in the total population (P < 0.0001), with a mean change of 0.53 ± 0.30, 0.89 ± 0.48, 1.35 ± 0.63, 1.63 ± 0.75 at 6 months, 12 months, 24 months, and 36 months, respectively. In addition, the changes in Ht SDS from baseline were significantly improved in the high-dose subgroup compared with the low-dose subgroup at 6, 12, 24, and 36 months after treatment (all P < 0.05). A total of 12 (1.03%) patients developed serious adverse events. There was no serious adverse event related to the treatment, and no AEs leading to treatment discontinuation or death occurred. CONCLUSIONS: PEG-rhGH showed long-term effectiveness and safety in treating children with GHD. Both dose subgroups showed promising outcomes, whereas PEG-rhGH 0.2 mg/kg/wk might show additional benefit.


Asunto(s)
Enanismo Hipofisario , Hormona de Crecimiento Humana , Humanos , Niño , Estudios Prospectivos , Hormona de Crecimiento Humana/uso terapéutico , Trastornos del Crecimiento/tratamiento farmacológico , Enanismo Hipofisario/tratamiento farmacológico , Factor I del Crecimiento Similar a la Insulina , Polietilenglicoles/efectos adversos , Proteínas Recombinantes/efectos adversos
9.
Nat Commun ; 13(1): 6356, 2022 10 26.
Artículo en Inglés | MEDLINE | ID: mdl-36289225

RESUMEN

Gut dysbiosis has been linked to type 1 diabetes (T1D); however, microbial capacity in T1D remains unclear. Here, we integratively profiled gut microbial functional and metabolic alterations in children with new-onset T1D in independent cohorts and investigated the underlying mechanisms. In T1D, the microbiota was characterized by decreased butyrate production and bile acid metabolism and increased lipopolysaccharide biosynthesis at the species, gene, and metabolite levels. The combination of 18 bacterial species and fecal metabolites provided excellently discriminatory power for T1D. Gut microbiota from children with T1D induced elevated fasting glucose levels and declined insulin sensitivity in antibiotic-treated mice. In streptozotocin-induced T1D mice, butyrate and lipopolysaccharide exerted protective and destructive effects on islet structure and function, respectively. Lipopolysaccharide aggravated the pancreatic inflammatory response, while butyrate activated Insulin1 and Insulin2 gene expression. Our study revealed perturbed microbial functional and metabolic traits in T1D, providing potential avenues for microbiome-based prevention and intervention for T1D.


Asunto(s)
Diabetes Mellitus Tipo 1 , Microbioma Gastrointestinal , Ratones , Animales , Microbioma Gastrointestinal/fisiología , Diabetes Mellitus Tipo 1/genética , Lipopolisacáridos/farmacología , Estreptozocina , Butiratos/farmacología , Antibacterianos/farmacología , Ácidos y Sales Biliares/farmacología , Glucosa/farmacología
10.
Eur J Endocrinol ; 187(5): 709-718, 2022 Nov 01.
Artículo en Inglés | MEDLINE | ID: mdl-36130048

RESUMEN

Objective: To evaluate the safety and efficacy of weekly PEGylated-recombinant human growth hormone (PEG-rhGH) in children with idiopathic short stature (ISS) in China. Design and methods: This was a multicenter, phase II study in which all subjects were randomized 1:1:1 to weekly s.c. injections of PEG-rhGH 0.1 (low-dose (LD) group) or 0.2 mg/kg/week (high-dose (HD) group) or control for 52 weeks. The primary end point was change (Δ) in height s.d. score (HT-SDS) from baseline to week 52. Secondary end points were height velocity (HV), bone maturity, insulin-like growth factor-1 (IGF-1) SDS, and IGF-1/insulin-like growth factor-binding protein-3 (IGFBP-3) molar ratio. Results: A total of 360 children with ISS were recruited in the study (n = 120 in each group). At week 52, ΔHT-SDS was 0.56 ± 0.26, 0.98 ± 0.35, and 0.20 ± 0.26 in the LD, HD, and control groups, respectively (within-group P < 0.0001; intergroup P < 0.0001). Statistically significant values of ΔHV, IGF-1, IGF-1/IGFBP-3 ratio, and IGF-1 SDS at week 52 from baseline were observed in both treatment groups (P < 0.0001). There were clear dose-dependent responses for all auxological variables. PEG-rhGH was well tolerated throughout the treatment period with treatment-emergent adverse events (TEAEs) reported in 86.5%, 84.6%, and 91.3% of children in the HD, LD, and control groups, respectively. The incidence of TEAEs was similar in all treatment groups despite the difference in doses. A total of 27 (8.7%) children experienced drug-related TEAEs. Conclusion: Fifty-two-week treatment with PEG-rhGH 0.1 or 0.2 mg/kg/week achieved significant improvement in HT-SDS and other growth-related variables, including HV, IGF-1 SDS, and IGF-1/IGFBP-3 ratio, in a dose-dependent manner. Both doses were well tolerated with similar safety profiles.


Asunto(s)
Hormona de Crecimiento Humana , Estatura/fisiología , Niño , Trastornos del Crecimiento/tratamiento farmacológico , Hormona del Crecimiento/uso terapéutico , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina , Factor I del Crecimiento Similar a la Insulina/metabolismo , Polietilenglicoles/efectos adversos , Proteínas Recombinantes/efectos adversos
11.
BMC Endocr Disord ; 22(1): 201, 2022 Aug 09.
Artículo en Inglés | MEDLINE | ID: mdl-35945517

RESUMEN

BACKGROUND: To evaluate the effectiveness of individualized-dose polyethylene glycol recombinant human growth hormone (PEG-rhGH) for short stature. METHODS: This real-world study enrolled children with short stature in 19 hospitals throughout China. They were treated with PEG-rhGH for 6 months. The starting dosage ranged from 0.10 to 0.20 mg/kg/week. The primary outcome was the change in height standard deviation score (ΔHt SDS). RESULTS: Five hundred and ten patients were included and grouped based on dosage as A (0.10-0.14 mg/kg/week), B (0.15-0.16 mg/kg/week), C (0.17-0.19 mg/kg/week), and D (0.20 mg/kg/week). The mean 6-month ΔHt SDS for the total cohort was 0.49 ± 0.27, and the means differed among the four dose groups (P = 0.002). The ΔHt SDS was lower in group A than in groups B (LSM difference [95%CI], -0.09 [-0.17, -0.01]), C (LSM difference [95%CI], -0.10 [-0.18, -0.02]), and D (LSM difference [95%CI], -0.13 [-0.21, -0.05]) after adjusting baseline covariates. There were no significant differences among groups B, C, and D. When the baseline IGF-1 was < -2 SDS or > 0 SDS, the △Ht SDS was not different among the four groups (P = 0.931 and P = 0.400). In children with baseline IGF-1 SDS of -2 ~ 0 SDS, a higher dosage was associated with a better treatment effect (P = 0.003), and the △Ht SDS was lower in older children than in younger ones (P < 0.001). CONCLUSIONS: PEG-rhGH could effectively increase height in prepubertal short children. When the baseline IGF-1 was < -2 SDS, 0.10 mg/kg/week could be a starting dose. In other IGF-1 statuses, 0.15-0.20 mg/kg/week might be preferred. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03249480 , retrospectively registered.


Asunto(s)
Enanismo , Hormona de Crecimiento Humana , Estatura , Niño , Trastornos del Crecimiento/tratamiento farmacológico , Hormona de Crecimiento Humana/análogos & derivados , Hormona de Crecimiento Humana/uso terapéutico , Humanos , Factor I del Crecimiento Similar a la Insulina , Polietilenglicoles
12.
Front Microbiol ; 13: 909729, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35783418

RESUMEN

Schizophrenia, a common mental disorder, has a tremendous impact on the health and economy of people worldwide. Evidence suggests that the microbial-gut-brain axis is an important pathway for the interaction between the gut microbiome and the development of schizophrenia. What is not clear is how changes in the gut microbiota composition and structure during antipsychotic treatment improve the symptoms of schizophrenia. In this study, 25 patients with schizophrenia were recruited. Their fecal samples were collected before and after hospital treatment for 14-19 days. The composition and structure of the intestinal microbiota were evaluated by 16S rRNA sequencing analysis, and the results showed significant differences in fecal microbiota before and after treatment. Firmicutes (relative abundances of 82.60 and 86.64%) and Gemminger (relative abundances of 14.17 and 13.57%) were the first dominant species at the phylum and genus levels, respectively. The random forest algorithm and co-occurrence network analysis demonstrated that intestinal flora (especially the core species ASV57) could be used as biomarkers to distinguish different clinical states and match treatment regimens accordingly. In addition, after fecal microbiota transplantation, antibiotic-treated recipient mice showed multiple behavioral improvements. These included decreased psychomotor hyperactivity, increased social interaction, and memory. In conclusion, this study suggests that differences in the composition and structure of gut microbiota after treatment are associated with the development and severity of schizophrenia. Results may provide a potential target for the treatment of this disorder.

13.
Front Pediatr ; 10: 911954, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35813374

RESUMEN

Background: Coffin-Siris syndrome (CSS) is a multiple congenital anomaly syndrome characterized by coarse facial features, sparse scalp hair, hypertrichosis, and hypo/aplastic digital nails and phalanges. Mutations in the BAF (SWI/SNF)-complex subunits (SMARCE1, SMARCB1, SMARCA4, SMARCA2, ARID1B, and ARID1A) have been shown to cause CSS. People diagnosed with BAF pathway related diseases are increasing, and ARID2 (NM_152641.4) is the least common of these genes. Mutations in the ARID2 gene is the cause for Coffin-Siris syndrome 6 (CSS6). By now only 16 individuals with CSS have been reported to have pathogenic variants in ARID2. Case Presentation: In this article, we introduced two individuals with clinical features consistent with CSS6 (Coffin-Siris syndrome 6). This article increases the number of reported cases, provides better phenotypic information for this rare syndrome, and allows everyone to better understand the disease. Conclusion: Our observations indicate that ARID2 mutations could have variable phenotypes, even in patients from the same family.

14.
World J Pediatr ; 18(10): 671-679, 2022 10.
Artículo en Inglés | MEDLINE | ID: mdl-35902493

RESUMEN

BACKGROUND: The real-world exposure levels of non-therapeutic antibiotics and neonicotinoids in type 1 diabetes (T1D) children and their associations as environmental triggers through gut microbiota shifts remained unknown. We thus investigated the antibiotics and neonicotinoids' exposure levels and their associations with gut microbiota in pediatric T1D. METHODS: Fifty-one newly onset T1D children along with 67 age-matched healthy controls were recruited. Urine concentrations of 28 antibiotics and 12 neonicotinoids were measured by mass spectrometry. Children were grouped according to the kinds of antibiotics' and neonicotinoids' exposures, respectively. The 16S rRNA of fecal gut microbiota was sequenced, and the correlation with urine antibiotics and neonicotinoids' concentrations was analyzed. RESULTS: The overall detection rates of antibiotics were 72.5% and 61.2% among T1D and healthy children, whereas the neonicotinoids detection rates were 70.6% and 52.2% (P = 0.044). Children exposed to one kind of antibiotic or two or more kinds of neonicotinoids had higher risk of T1D, with the odd ratios of 2.579 and 3.911. Furthermore, co-exposure to antibiotics and neonicotinoids was associated with T1D, with the odd ratio of 4.924. Antibiotics or neonicotinoids exposure did not affect overall richness and diversity of gut microbiota. However, children who were exposed to neither antibiotics nor neonicotinoids had higher abundance of Lachnospiraceae than children who were exposed to antibiotics and neonicotinoids alone or together. CONCLUSION: High antibiotics and neonicotinoids exposures were found in T1D children, and they were associated with changes in gut microbiota featured with lower abundance of butyrate-producing genera, which might increase the risk of T1D.


Asunto(s)
Diabetes Mellitus Tipo 1 , Microbioma Gastrointestinal , Antibacterianos/efectos adversos , Butiratos , Niño , Diabetes Mellitus Tipo 1/inducido químicamente , Diabetes Mellitus Tipo 1/epidemiología , Humanos , Neonicotinoides , ARN Ribosómico 16S/genética
15.
J Pers Med ; 12(5)2022 May 10.
Artículo en Inglés | MEDLINE | ID: mdl-35629193

RESUMEN

Objective: To provide new information about androgen insensitivity syndrome (AIS), we studied growth patterns in Chinese children with AIS. Subjects: Data are from 118 untreated AIS patients who were admitted to eight pediatric endocrine centers from January 2010 to December 2019. Methods: In this retrospective cohort study, clinical data were collected from a multicenter database. We compared physical assessment data among AIS patients and standard growth charts for Chinese pediatric population. Results: 1. Children with AIS grew slightly less than the mean before 6 months of age, and then, height gradually increased before 12 years of age, from the median to +1 standard deviation (SD), according to the standard reference for Chinese pediatric population. After 12 years of age, height showed differently in profiles: The mean height in AIS patients gradually decreased from the mean to −1 SD, according to the standard for Chinese boys, and increased from the mean to +2 SD, according to the standard for Chinese girls. 2. The weights of children with AIS were greater than the mean standards of Chinese pediatric population from newborn to 11 years of age. From 12−16 years of age, the mean weight of children with AIS showed different profiles, from the mean to −1 SD, according to the standard for Chinese boys and from the mean to +1.5 SD, according to the standard for Chinese girls. 3. Weight standard deviation (WtSDS) and target height (THt) in northern Chinese AIS patients were significantly higher than those from the southern region (p = 0.035, 0.005, respectively). Age in northern Chinese AIS patients was significantly younger than those from the southern region (p = 0.034). No difference was found among birth weight (BW), birth length (BL), height standard deviation (HtSDS) and body mass index (BMI) in AIS patients from different regions (p > 0.05). 4. HtSDS and WtSDS in complete AIS (CAIS) patients were higher than those in partial AIS (PAIS) patients without significant difference (p > 0.05). Conclusions: Growth of children with AIS varied to different degrees. AIS patients seemed not to experience a puberty growth spurt. CAIS and PAIS patients show little difference in their growth. Regional differences have no effect on the height but influence the weight of AIS patients.

16.
Front Endocrinol (Lausanne) ; 13: 864908, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35573994

RESUMEN

Background: To evaluate the safety and efficacy of daily somatropin (Jintropin®), a recombinant human growth hormone, in prepubertal children with ISS in China. Methods: This study was a multicenter, randomized, controlled, open-label, phase 3 study. All subjects were randomized 3:1 to daily somatropin 0.05 mg/kg/day or no treatment for 52 weeks. A total of 481 subjects with a mean baseline age of 5.8 years were enrolled in the study. The primary endpoint was change in (△) height standard deviation score (HT-SDS) for chronological age (CA). Secondary endpoints included △height from baseline; △bone age (BA)/CA; △height velocity (HV) and △insulin-like growth factor 1 (IGF-1 SDS). Results: △HT-SDS at week 52 was 1.04 ± 0.31 in the treatment group and 0.20 ± 0.33 in the control group (P < 0.001). At week 52, statistical significance was observed in the treatment group compared with control for △height (10.19 ± 1.47 cm vs. 5.85 ± 1.80 cm; P < 0.001), △BA/CA (0.04 ± 0.09 vs. 0.004 ± 0.01; P < 0.001), △HV (5.17 ± 3.70 cm/year vs. 0.75 ± 4.34 cm/year; P < 0.001), and △IGF-1 SDS (2.31 ± 1.20 vs. 0.22 ± 0.98; P < 0.001). The frequencies of treatment-emergent adverse events (TEAEs) were similar for the treatment and the control groups (89.8% vs. 82.4%); most TEAEs were mild to moderate in severity and 23 AEs were considered study-drug related. Conclusions: Daily subcutaneous administration of somatropin at 0.05 mg/kg/day for 52 weeks demonstrated improvement in growth outcomes and was well tolerated with a favorable safety profile. Trial Registration: ClinicalTrials.gov (identifier: NCT03635580). URL: https://clinicaltrials.gov/ct2/show/NCT03635580.


Asunto(s)
Hormona de Crecimiento Humana , Estatura , Preescolar , China/epidemiología , Trastornos del Crecimiento/tratamiento farmacológico , Hormona de Crecimiento Humana/efectos adversos , Humanos , Proteínas Recombinantes/efectos adversos
17.
Nano Lett ; 22(7): 3026-3032, 2022 Apr 13.
Artículo en Inglés | MEDLINE | ID: mdl-35343702

RESUMEN

We report the observation of coherent oscillations in the relaxation dynamics of an exciton-polariton condensate that were driven by parametric scattering processes. As a result of the interbranch scattering scheme and the nonlinear polariton-polariton interactions, such parametric scatterings exhibit a high scattering efficiency that leads to the fast depletion of the polariton condensate and the periodic shut-off of the bosonic stimulation processes, eventually causing relaxation oscillations. Employing polariton-reservoir interactions, the oscillation dynamics in the time domain can be projected onto the energy space. In theory, our simulations using the open-dissipative Gross-Pitaevskii equation are in excellent agreement with experimental observations. Surprisingly, the oscillation patterns, including many excitation pulses, are clearly visible in our time-integrated images, implying the high stability of the relaxation oscillations driven by polariton parametric scatterings.

18.
Front Endocrinol (Lausanne) ; 12: 752496, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34867796

RESUMEN

Objective: Graves' disease (GD) related hyperthyroidism (HT) has profound effects on metabolic activity and metabolism of macromolecules affecting energy homeostasis. In this study, we aimed to get a comprehensive understanding of the metabolic changes and their clinical relevance in GD children. Methods: We investigated serum substances from 30 newly diagnosed GD children and 30 age- and gender-matched healthy controls. We explored the metabolomics using ultra-high-performance liquid chromatography-quadrupole time-of-flight mass spectrometry (UHPLC-QTOF/MS) analysis, and then analyzed the metabolomic data via multivariate statistical analysis. Results: By untargeted metabolomic analysis, a total of 730 metabolites were identified in all participants, among which 48 differential metabolites between GD and control groups were filtered out, including amino acids, dipeptides, lipids, purines, etc. Among these metabolites, 33 were detected with higher levels, while 15 with lower levels in GD group compared to controls. Pathway analysis showed that HT had a significant impact on aminoacyl-transfer ribonucleic acid (tRNA) biosynthesis, several amino acids metabolism, purine metabolism, and pyrimidine metabolism. Conclusion: In this study, via untargeted metabolomics analysis, significant variations of serum metabolomic patterns were detected in GD children.


Asunto(s)
Enfermedad de Graves/metabolismo , Metabolómica , Sistemas de Transporte de Aminoácidos/genética , Sistemas de Transporte de Aminoácidos/metabolismo , Aminoácidos/metabolismo , Biomarcadores , Niño , Preescolar , Cromatografía Líquida de Alta Presión , Femenino , Enfermedad de Graves/genética , Humanos , Masculino , Redes y Vías Metabólicas , Purinas/metabolismo , Pirimidinas/metabolismo , ARN de Transferencia Aminoácido-Específico/biosíntesis , ARN de Transferencia Aminoácido-Específico/genética , Espectrometría de Masas en Tándem
20.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 38(10): 942-946, 2021 Oct 10.
Artículo en Chino | MEDLINE | ID: mdl-34625928

RESUMEN

OBJECTIVE: To analyze the clinical and genetic characteristics of five Chinese pedigrees affected with short stature. METHODS: A retrospective analysis was carried out for the clinical data and results of genetic testing for the probands. A literature search was also conducted. RESULTS: The five probands have all featured short stature with a family history. Genetic testing has revealed that they have harbored variants of the ACAN gene, including p.Val2042Argfs*6, p.Val1597del, c.630-1G>A, c.23delT and c.2026+1G>A(previously reported). CONCLUSION: Except for short stature, children harboring heterozygous variants of the ACAN gene may have no involvement of other systems. Some of these children may response to short-term growth hormone treatment.


Asunto(s)
Agrecanos , Estatura/genética , Agrecanos/genética , Niño , China , Pruebas Genéticas , Humanos , Linaje , Estudios Retrospectivos
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