Patients with Noonan syndrome (NS) are born with normal or slightly lower body length and weight compared to the normal ranges. However, their height gradually falls behind that of the general population, leading to growth retardation and delayed puberty. In China, the incidence of short stature in patients with NS is approximately 65%. Short stature in these patients arises from multiple causes, including feeding difficulties in infancy, comorbidities such as congenital heart disease, genetic heterogeneity, and disorders of the growth hormone/insulin-like growth factor-1 axis. Growth hormone is commonly used to alleviate symptoms of short stature. This article reviews the growth and development patterns at different stages of NS, analyzes the causes of short stature, and summarizes the latest advances in treatment to provide new insights for the diagnosis and management of short stature in patients with NS.
Noonan Syndrome/complications* ; Humans ; Body Height ; Growth Disorders/therapy*

Objective: To analyze the clinical epidemiological characteristics including composition of pathogens , clinical characteristics, and disease prognosis acute bacterial meningitis (ABM) in Chinese children. Methods: A retrospective analysis was performed on the clinical and laboratory data of 1 610 children <15 years of age with ABM in 33 tertiary hospitals in China from January 2019 to December 2020. Patients were divided into different groups according to age,<28 days group, 28 days to <3 months group, 3 months to <1 year group, 1-<5 years of age group, 5-<15 years of age group; etiology confirmed group and clinically diagnosed group according to etiology diagnosis. Non-numeric variables were analyzed with the Chi-square test or Fisher's exact test, while non-normal distrituction numeric variables were compared with nonparametric test. Results: Among 1 610 children with ABM, 955 were male and 650 were female (5 cases were not provided with gender information), and the age of onset was 1.5 (0.5, 5.5) months. There were 588 cases age from <28 days, 462 cases age from 28 days to <3 months, 302 cases age from 3 months to <1 year of age group, 156 cases in the 1-<5 years of age and 101 cases in the 5-<15 years of age. The detection rates were 38.8% (95/245) and 31.5% (70/222) of Escherichia coli and 27.8% (68/245) and 35.1% (78/222) of Streptococcus agalactiae in infants younger than 28 days of age and 28 days to 3 months of age; the detection rates of Streptococcus pneumonia, Escherichia coli, and Streptococcus agalactiae were 34.3% (61/178), 14.0% (25/178) and 13.5% (24/178) in the 3 months of age to <1 year of age group; the dominant pathogens were Streptococcus pneumoniae and the detection rate were 67.9% (74/109) and 44.4% (16/36) in the 1-<5 years of age and 5-<15 years of age . There were 9.7% (19/195) strains of Escherichia coli producing ultra-broad-spectrum β-lactamases. The positive rates of cerebrospinal fluid (CSF) culture and blood culture were 32.2% (515/1 598) and 25.0% (400/1 598), while 38.2% (126/330)and 25.3% (21/83) in CSF metagenomics next generation sequencing and Streptococcus pneumoniae antigen detection. There were 4.3% (32/790) cases of which CSF white blood cell counts were normal in etiology confirmed group. Among 1 610 children with ABM, main intracranial imaging complications were subdural effusion and (or) empyema in 349 cases (21.7%), hydrocephalus in 233 cases (14.5%), brain abscess in 178 cases (11.1%), and other cerebrovascular diseases, including encephalomalacia, cerebral infarction, and encephalatrophy, in 174 cases (10.8%). Among the 166 cases (10.3%) with unfavorable outcome, 32 cases (2.0%) died among whom 24 cases died before 1 year of age, and 37 cases (2.3%) had recurrence among whom 25 cases had recurrence within 3 weeks. The incidences of subdural effusion and (or) empyema, brain abscess and ependymitis in the etiology confirmed group were significantly higher than those in the clinically diagnosed group (26.2% (207/790) vs. 17.3% (142/820), 13.0% (103/790) vs. 9.1% (75/820), 4.6% (36/790) vs. 2.7% (22/820), χ²=18.71, 6.20, 4.07, all P<0.05), but there was no significant difference in the unfavorable outcomes, mortility, and recurrence between these 2 groups (all P>0.05). Conclusions: The onset age of ABM in children is usually within 1 year of age, especially <3 months. The common pathogens in infants <3 months of age are Escherichia coli and Streptococcus agalactiae, and the dominant pathogen in infant ≥3 months is Streptococcus pneumoniae. Subdural effusion and (or) empyema and hydrocephalus are common complications. ABM should not be excluded even if CSF white blood cell counts is within normal range. Standardized bacteriological examination should be paid more attention to increase the pathogenic detection rate. Non-culture CSF detection methods may facilitate the pathogenic diagnosis.
Adolescent ; Brain Abscess ; Child ; Child, Preschool ; Escherichia coli ; Female ; Humans ; Hydrocephalus ; Infant ; Infant, Newborn ; Male ; Meningitis, Bacterial/epidemiology* ; Retrospective Studies ; Streptococcus agalactiae ; Streptococcus pneumoniae ; Subdural Effusion ; beta-Lactamases

Acetaminophen (APAP) overdose is the leading cause of drug-induced liver injury, and its prognosis depends on the balance between hepatocyte death and regeneration. Sirtuin 6 (SIRT6) has been reported to protect against oxidative stress-associated DNA damage. But whether SIRT6 regulates APAP-induced hepatotoxicity remains unclear. In this study, the protein expression of nuclear and total SIRT6 was up-regulated in mice liver at 6 and 48 h following APAP treatment, respectively.

Ferroptosis is a non-apoptotic regulated cell death caused by iron accumulation and subsequent lipid peroxidation. Currently, the therapeutic role of ferroptosis on cancer is gaining increasing interest. Baicalin an active component in

OBJECTIVE: To explore clinical effects of micro external fixator for the treatment of supracondylar fracture of humerus in children. METHODS: From October 2017 to December 2018, 20 children with supracondylar fracture of humerus (treatment group) were admitted and treated with micro-external fixation after closed reduction, including 14 males and 6 females, aged from 6 to 14 years old with an average of (7.9±2.4) years old, classified to Gartland Ⅲ. Thirty nine children with supracondylar fracture of humerus were admitted as control group from January 2015 to September 2017, and treated with closed reduction Kirschner wire fixation. Among them, including 24 males and 15 females, aged from 6 to 14 years old with an average of (8.1±1.9) years old, classified to GartlandⅢ. Operation time, times of intraoperative fluoroscopy, and complications between two groups were observed and compared, Mayo scoring system at the latest follow up was used to evaluate clinical effect. RESULTS: Twenty children in treatment group were followed up from 6 to 12 months with an average of (8.0±2.5) months, operation time was(30.10±12.50) min, times of intraoperative fluoroscopy was(10.00±2.50). Fifteen patients got excellent results, 3 good, 2 fair according to Mayo elbow joint scoring. No ulnar nerve injury moderate or severe elbow varus occurred in treatment group. Thirty-nine children in control group were followed up from 5 to 13 months with an average of (9.0±3.1) months, operation time was(45.60±18.90) min, times of intraoperative fluoroscopy was(19.00±5.60). Twenty-three patients got excellent results, 12 good, 3 fair and 1 poor according to Mayo elbow joint scoring. One child occurred ulnar nerve injury and recovered at the final follow-up, 1 child occurred severe cubitus varus and 2 children occurred moderate cubitus varus. There was no statistical difference in clinical effect between two groups ( CONCLUSION Treated with closedreduction and mini external fixator fixed on children humerus condyle fracture could receive the same clinical result as closed as reduction gram needle fixation, which has advantages of shorter operation time, less times of intraoperative fluoroscopy, not esay to damage ulnar nerve during operation, less incidence of moderate and severe cubitus varus deformity after operation, and the permeation was simple.
Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; External Fixators ; Female ; Fracture Fixation, Internal ; Humans ; Humeral Fractures/surgery* ; Humerus ; Male

Objective: To explore the association between soil selenium levels and the risk of diabetes in Chinese adults aged 35-74 years. Methods: Data for this study were derived from the China Chronic Diseases and Behavioral Risk Factors Surveillance 2010 survey. Selenium concentrations in soil were obtained from the Atlas of Soil Environmental Background Values in China. A two-level binary logistic regression model was used to determine the association between soil selenium concentrations and the risk of diabetes, with participants nested within districts/counties. Results: A total of 69,332 participants aged 35-74 years, from 158 districts/counties were included in the analysis. Concentrations of selenium in soil varied greatly across the 158 districts/counties, with a median concentration of 0.219 mg/kg ( : 0.185-0.248). The results showed that both Quartile 1 (0.119-0.185 mg/kg) and Quartile 4 (0.249-0.344 mg/kg) groups were positively associated with diabetes compared to a soil selenium concentration of 0.186-0.219 mg/kg (Quartile 2), crude odds ratios ( s) (95% ) were 1.227 (1.003-1.502) and 1.280 (1.048-1.563). The values were 0.045 and 0.013, for Quartile 1 and Quartile 4 groups, respectively. After adjusting for all confounding factors of interest, the Quartile 1 group became non-significant, and the Quartile 4 group had an adjusted (95% ) of 1.203 (1.018-1.421) relative to the reference group (Quartile 2), the values was 0.030. No significant results were seen for the Quartile 3 group (0.220-0.248 mg/kg) compared to the reference group. Conclusion Excessive selenium concentrations in soil could increase the risk of diabetes among Chinese adults aged 35-74 years.
Adult ; Aged ; China ; epidemiology ; Diabetes Mellitus ; chemically induced ; epidemiology ; Diet ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; Risk Factors ; Selenium ; deficiency ; metabolism ; Soil ; chemistry

Objective: Liver fibrosis is an important predictor of mortality in nonalcoholic fatty liver disease (NAFLD). Peripheral artery disease (PAD) and liver fibrosis share many common metabolic dysfunctions. We aimed to explore the association between PAD and risk of fibrosis deterioration in NAFLD patients. Methods: The study recruited 1,610 NAFLD patients aged ≥ 40 years from a well-defined community at baseline in 2010 and followed up between August 2014 and May 2015. Fibrosis deterioration was defined as the NAFLD fibrosis score (NFS) status increased to a higher category at the follow-up visit. PAD was defined as an ankle-brachial index of < 0.90 or > 1.40. Results: During an average of 4.3 years' follow-up, 618 patients progressed to a higher NFS category. PAD was associated with 92% increased risk of fibrosis deterioration [multivariable-adjusted odds ratio ( ): 1.92, 95% confidence interval ( ): 1.24, 2.98]. When stratified by baseline NFS status, the for progression from low to intermediate or high NFS was 1.74 (95% : 1.02, 3.00), and progression from intermediate to high NFS was 2.24 (95% : 1.05, 4.80). There was a significant interaction between PAD and insulin resistance (IR) on fibrosis deterioration ( for interaction = 0.03). As compared with non-PAD and non-IR, the coexistence of PAD and IR was associated with a 3.85-fold (95% : 2.06, 7.18) increased risk of fibrosis deterioration. Conclusion PAD is associated with an increased risk of fibrosis deterioration in NAFLD patients, especially in those with IR. The coexistence of PAD and IR may impose an interactive effect on the risk of fibrosis deterioration.
Adult ; Aged ; Aged, 80 and over ; Ankle Brachial Index ; China ; epidemiology ; Female ; Humans ; Liver Cirrhosis ; epidemiology ; etiology ; Male ; Middle Aged ; Non-alcoholic Fatty Liver Disease ; epidemiology ; etiology ; Peripheral Arterial Disease ; complications ; Prevalence ; Prospective Studies ; Risk Factors

AIM: To study the reliability of Pentacam, OCT, Tomey OA-2000, IOL Master700, and A ultrasound pachymetry in the measurement of central corneal thickness(CCT)in myopic eyes.

METHODS: CCTs of 56 myopic patients(112 eyes)were measured using Pentacam, RTVue OCT, Tomey OA-2000, IOL Master 700, and A ultrasound pachymetry(NIDEK US-500). The difference, correlation, and consistency were statistically analyzed using average values taken from the five devices and the measured values produced by each device.

RESULTS: CCTs of 112 eyes were determined using Pentacam(530.17±25.08)μm, OCT(519.79±26.90)μm, Tomey OA-2000(521.75±26.51)μm, IOL Master 700(519.53±28.15)μm, and A ultrasound pachymetry(542.23±26.88)μm. The average value across all five devices was 526.69±26.08μm. The results of root mean square error(RMSE)and theil inequality coefficient(TIC)showed that the degree of deviation from the average of the values measured using Pentacam was the smallest. The other four devices were, from least deviation to most, Tomey OA-2000, OCT, IOL Master 700, and A ultrasound pachymetry. Typical correlation analysis showed that the correlation coefficients between the measured values of Pentacam, OCT, Tomey OA-2000, IOL Master 700, and A ultrasound pachymetry and the average values were ρ=0.957, P=0.950, P=0.953, P=0.930, and ρ=0.949, respectively. The results of correlation coefficient showed that Pentacam and Tomey OA-2000 had the closest correlation with the average value. The other three were, in order of closest to furthest correlation, OCT, A ultrasound pachymetry and IOL master 700. The results also showed that the measurements of Pentacam and Tomey OA-2000 were more reliable, which was consistent with the clinical situation. The results of Bland-Altman analysis showed that the five groups of measurements were closely consistent with the average values, and the CCT measurements of Pentacam and Tomey OA-2000 were the most consistent with the average values.

CONCLUSION: Among the five instruments, Pentacam and Tomey OA-2000 produced results that were closest to the average value in CCT measurement, with the best correlation and consistency. Their measurement methods are more objective and safer than A-scan, so they may provide better reference data for clinical practice.

Objective To investigate the macular vascular density after successful repair of rhegmatogenous retinal detachment (RRD) for one year using optical coherence tomography angiography (OCTA),and discuss the correlation between the macular vascular density and visual acuity.Methods Totally 42 patients of the RRD (42 eyes),their contralateral eyes (A group) and 42 patients of the normal eyes (B group) were recruited into this study.All participants underwent examination with best corrected visual acuity (BCVA) and OCTA.The difference in macular vascular density was compared and the correlation between BCVA and the vascular density was analyzed.Results The macular vascular density of superficial layer,deep layer and choroidal capillary layer was 0.422 4 ±0.089 3,0.4836 ±0.0748,0.527 1 ±0.039 0 in RRD group,respectively,0.469 3 ±0.112 5,0.550 0 ±0.074 0,0.546 2 ±0.034 3 in A group,respectively,0.5619 ±0.053 7,0.611 2 ±0.035 2,0.562 6 ±0.030 4 in B group,respectively.The macular vascular density was significantly decreased in RRD group when compared with A and B groups (all P ＜ 0.05).There was a positive correlation between BCVA and the macular vascular density in the deep layer and choroidal capillaries layer (r =0.629,0.654,both P =0.000).However,there's no correlation between the macular vascular density of superficial layer and BCVA (P =0.103).Conclusion All the macular vascular densities are decreased in patients of RRD after successful repair of retinal detachment one year later,which indicated that the blood flow does not completely recover.And there is a positive correlation between BCVA and macular vascular densities in deep layer and choroidal capillaries layer.And meanwhile,OCTA can objectively and effectively quantify the status of macular region blood flow.

AIM:To investigate the influence of signal transducer and activator of transcription 3(STAT3)on Warburg effect in the malignant transformation of WB-F344 rat hepatic oval cells.METHODS:The WB-F344 cells were treated with N-methyl-N'-nitro-N-nitrosoguanidine(MNNG)and hydrogen peroxide(H2O2)to induce the malignant trans-formation.Evaluation of the transformed cells were measured by the soft agar colony formation assay and DNA aneuploidy with flow cytometry.The levels of glucose and lactate in the culture medium of the cells were detected by chromatography. The protein levels of alpha-fetoprotein(AFP),STAT3,p-STAT3 and glucose transporter 2(GLUT2)in the cells were ex-amined by Western blot analysis.The cell proliferation were evaluated by WST-1 assay,viable cell counting,measuring the S-phase fraction(SPF)and proliferation index(PI)using the data from flow cytometry analysis,and detecting proliferating cell nuclear antigen(PCNA)protein expression by Western blot.RESULTS:Compared with the control cells,the forma-tion of colonies in soft agar(P<0.05)and DNA aneuploidy(P<0.01)were elevated in transformed cells,and the ex-pression level of AFP was also augmented(P<0.05).The increases in the level of both glucose consumption(P<0.05) and lactate production(P<0.01)show that Warburg effect was enhanced in transformed cells.Meanwhile, the protein levels of GLUT2(P<0.01)and p-STAT3(P<0.01)in transformed cells were higher than those in the control cells.The cell proliferation parameters including SPF(P<0.01),PI(P<0.01), viable cell number and PCNA expression(P<0.01)in transformed cells were also elevated as compared with the control cells.Interestingly, stattic, an inhibitor of STAT3 activation,resulted in declines in glucose consumption(P<0.05)and lactate production(P<0.01)in the trans-formed cells.In addition,compared with transformed cells,formation of colonies in soft agar(P<0.01),DNA aneuploidy (P<0.01),AFP(P<0.05), GLUT2(P<0.05), and cell proliferation parameters including SPF(P<0.01), PI (P<0.01),viable cell number(P<0.05)and PCNA expression(P<0.05)were also decreased following stattic treat-ment in transformed cells.CONCLUSION:STAT3 promotes Warburg effect and cell proliferation probably by upregula-ting GLUT2 expression in the malignant transformation of hepatic oval cells.