Total Syntheses of Polycyclic Diterpenes Phomopsene, Methyl Phomopsenonate, and iso-Phomopsene via Reorganization of C-C Single Bonds.

The first total syntheses of polycyclic diterpenes phomopsene (1), methyl phomopsenonate (2), and iso-phomopsene (3) have been accomplished through the unusual cascade reorganization of C-C single bonds. This approach features: (i) a synergistic Nazarov cyclization/double ring expansions in one-step, developed by authors, to rapid and stereospecific construction of the 5/5/5/5 tetraquinane scaffold bearing contiguous quaternary centers and (ii) a one-pot strategic ring expansion through Beckmann fragmentation/recombination to efficiently assemble the requisite 5/5/6/5 tetracyclic skeleton of the target molecules 1-3. This work enables us to determine that the correct structure of iso-phomopsene is, in fact, the C7 epimer of the originally assigned structure. Finally, the absolute configurations of three target molecules were confirmed through enantioselective synthesis.

A case of mental retardation autosomal dominant 35 with neonatal onset / 中华围产医学杂志

This article reported a male patient with neonatal onset mental retardation autosomal dominant 35 (MRD35). The boy presented with repeated convulsions, hypotonia, enlarged head circumference, congenital muscular torticollis and feeding difficulties in the neonatal period. Dynamic electroencephalogram showed paroxysmal epileptic discharges in the left central-temporal region. High-throughput whole-exome sequencing revealed a heterozygous mutation of c.139G>A (p.Glu47Lys) in the PPP2R5D gene, which was a de novo mutation not inherited from his parents. The child had significant developmental delay at the age of one year. MRD35 lacks typical clinical manifestations and requires whole-exome sequencing for definitive diagnosis. Currently, there is no specific treatment for MRD35 and symptomatic treatments, including rehabilitation training, language training and seizure control, are mostly adopted.

Clinical characteristics and risk factors for early-onset necrotizing enterocolitis in preterm infants with very/extremely low birth weight / 中国当代儿科杂志

OBJECTIVES@#To investigate the clinical characteristics and risk factors for early-onset necrotizing enterocolitis (NEC) in preterm infants with very/extremely low birth weight (VLBW/ELBW).@*METHODS@#A retrospective analysis was performed on the medical data of 194 VLBW/ELBW preterm infants with NEC who were admitted to Children's Hospital Affiliated to Zhengzhou University from January 2014 to December 2021. These infants were divided into early-onset group (onset in the first two weeks of life; n=62) and late-onset group (onset two weeks after birth; n=132) based on their onset time. The two groups were compared in terms of perinatal conditions, clinical characteristics, laboratory examination results, and clinical outcomes. Sixty-two non-NEC infants with similar gestational age and birth weight who were hospitalized at the same period as these NEC preterm infants were selected as the control group. The risk factors for the development of early-onset NEC were identified using multivariate logistic regression analysis.@*RESULTS@#Compared with the late-onset group, the early-onset group had significantly higher proportions of infants with 1-minute Apgar score ≤3, stage III NEC, surgical intervention, grade ≥3 intraventricular hemorrhage, apnea, and fever or hypothermia (P<0.05). The multivariate logistic regression analysis showed that feeding intolerance, blood culture-positive early-onset sepsis, severe anemia, and hemodynamically significant patent ductus arteriosus were independent risk factors for the development of early-onset NEC in VLBW/ELBW preterm infants (P<0.05).@*CONCLUSIONS@#VLBW/ELBW preterm infants with early-onset NEC have more severe conditions compared with those with late-onset NEC. Neonates with feeding intolerance, blood culture-positive early-onset sepsis, severe anemia, or hemodynamically significant patent ductus arteriosus have a higher risk of early-onset NEC.

Effects of probiotics on feeding intolerance and early growth and development of preterm and very low birth weight infants / 中华健康管理学杂志

Objective:To explore the effects of probiotics on feeding intolerance and early growth and development of preterm and very low birth weight infants (VLBWI).Methods:A total of 446 patients with VLBWI were enrolled in Henan Children′s Hospital from March 20, 2019 to March 20, 2022. They were divided into breast milk group (119 cases), breast milk+probiotic group (108 cases), formula group (115 cases) and formula+probiotic group (104 cases) according to the feeding mode and whether they received early probiotic supplement or not. The feeding programs of each group were carried out according to the “2013 Clinical Application Guide of nutritional support for newborn infants” and “the feeding guidelines for VLBWI”. On this basis, the probiotic supplement group was treated with Clostridium butyricum powder at a dose of 0.25 g/twice a day for 2 weeks, nasal feeding or bottle administration. The incidence of feeding intolerance, the time of recovery of birth weight, the time of reaching total gastrointestinal feeding, and the time of hospitalization in each group were observed, and the average increment of body mass, head circumference and body length in each group at 28 days after birth were compared.Results:In breast milk group, the incidence of feeding intolerance, total gastrointestinal feeding time, and hospital stay were significantly lower than those in the formula group [40.3% vs 47.8%, (13.2±2.4) vs (14.9±1.9) d, (26.5±7.5) vs (29.8±9.6) d], besides, the average increments of early growth and developmental indexes such as body mass, head circumference, and body length at 28 days of birth were significantly lower than those in the formula group [(15.4±2.7) vs (17.7±3.7) g/d, (5.8±1.0) vs (6.2±1.0) mm/week, (6.8±0.7) vs (7.3±0.8) mm/week], however, the recovery time of birth weight in the breast milk group was significantly higher than that in the formula group [(6.2±1.2) vs (5.3±1.4) d] (all P<0.05). The incidence of feeding intolerance, the recovery time of birth weight, the total gastrointestinal feeding time and the hospital stay in the breast milk+probiotics group were significantly lower than those in the breast milk group [34.3% vs 40.3%, (5.4±1.2) vs (6.2±1.2) d, (10.4±1.9) vs (13.2±2.4) d, (22.9±5.6) vs (26.5±7.5) d], besides, the average increments of body mass, head circumference and body length at 28 days of birth was significantly higher than that in the breast milk group [(17.2±3.5) vs (15.4±2.7) g/d, (6.2±1.0) vs (5.8±1.0) mm/week, (7.2±0.8) vs (6.8±0.7) mm/week] (all P<0.05). The incidence of feeding intolerance, total gastrointestinal feeding time and hospital stay in breast milk+probiotics group were significantly lower than those in formula+probiotics group [34.3% vs 47.1%, (10.4±1.9) vs (15.3±2.0) d, (22.9±5.6) vs (30.4±8.2) d] (all P<0.05), besides, there was no significant difference in the average increments of early growth and developmental indexes between the breast milk+probiotics group and the formula+probiotics group (all P>0.05). There were no significant difference in the above indexes between the formula+probiotics group and the formula group (all P>0.05). Conclusion:Probiotics can improve the clinical outcome of feeding intolerance and promote early growth and development of breast feeding VLBWI.

Association of Next Generation Sequencing Based Genotypic Profiling with MICM Characteristics in NPM1 Mutated Acute Myeloid Leukemia / 中国实验血液学杂志

OBJECTIVE@#To explain the clinicobiological heterogeneity of NPM1 mutated (NPM1mut) acute myeloid leukemia (AML) by analyzing the association between next-generation sequencing (NGS) profiles and MICM characteristics in patients with this AML subtype.@*METHODS@#Data of 238 NPM1mut patients with available NGS information on 112 genes related to blood disease was collected, and χ2 test and nonparametric test were used to analyze the distribution association between NGS-detecting mutations and conventional MICM parameters.@*RESULTS@#In entire NPM1mut cohort, totaling 240 NPM1 mutation events were identified, of whom 10 (10/240, 4.2%) were missense mutations, which did not involve any W288 or W290 locus and were found exclusively in NPM1mut/FLT3-ITD- group. All but one of these missense mutations (9/10, 90%) were accompanied by AML subtype-defining recurrent cytogenetic or molecular abnormalities, of which 7 cases were in the low risk and 2 in the high risk. NPM1mut occurred solely as an insertion/deletion (indel) type in the NPM1mut/FLT3-ITD+ group. The incidence of favorable plus unfavorable karyotypes in NPM1mut/FLT3-ITD- group was higher than in NPM1mut/FLT3-ITD+ group (6.4% vs. 0, P=0.031). The positive rates of CD34 and CD7 in NPM1mut/FLT3-ITD+ group were significantly higher than in NPM1mut/FLT3-ITD- group (CD34: 47.9% vs. 20.6%, P<0.001; CD7: 61.5% vs. 29.9%, P<0.001). Logistic analysis showed that FLT3-ITD independently predicted for CD34+ and CD7+ [odds ratio (OR)=5.29, 95%CI: 2.64-10.60, P<0.001; OR=3.47, 95%CI: 1.79-6.73, P<0.001; respectively]. Ras-pathway mutations independently predicted for HLA-DR+ (OR=4.05, 95%CI: 1.70-9.63, P=0.002), and KRAS mutation for MPO- (OR=0.18, 95%CI: 0.05-0.62, P=0.007). TET2/IDH1 mutations independently predicted for CD34- and CD7- (OR=0.26, 95%CI: 0.11-0.62, P=0.002; OR=0.30, 95%CI: 0.14-0.62, P=0.001; respectively), and MPO+ (OR=3.52, 95%CI: 1.48-8.38, P=0.004). DNMT3A-R882 independently predicted for CD7+ and HLA-DR+ (OR=3.59, 95%CI: 1.80-7.16, P<0.001; OR=13.41, 95%CI: 4.56-39.45, P<0.001; respectively), and DNMT3A mutation for MPO-(OR=0.35, 95%CI: 1.48-8.38, P=0.004).@*CONCLUSION@#Co-existing FLT3-ITD in NPM1mut AML independently predicts for CD34+ and CD7+, co-existing Ras-pathway mutation for HLA-DR+ and MPO-, co-existing TET2/IDH1 mutation for CD34-, CD7-, and MPO+, and co-existing DNMT3A mutation for HLA-DR+, CD7+, and MPO-, thereby providing a new mechanism explanation for the immunophenotypic heterogeneity of these AML patients.

The Evaluation of Sagittal Pelvic-Femoral Kinematics in Patients with Cam-Type Femoracetabular Impingement.

OBJECTIVE: To investigate the sagittal hip-pelvic kinematics in symptomatic cam-type femoroacetabular impingement (FAI) patients in the process of sitting down and compare their difference between patients with sitting pain complaint and those without. METHODS: Twenty-nine symptomatic cam-type FAI patients were recruited from our clinic between May 2018 and October 2018. Patients were categorized into two groups depending on whether they complain of pain in prolonged sitting or not. The pelvic-femoral measurements were assessed with a set of lateral pelvic radiography in sitting and standing respectively. Pelvic incidence (PI), sacral slope (SS), and proximal femoral shaft angle (PFSA) were measured on lateral pelvic radiography, and then pelvic tilting, apparent hip flexion, true hip flexion, and the pelvic-femoral ratio were calculated to investigate the kinematic change from standing to sitting position. Demographic measurements, hip morphology measurements, functional measurements, visual analog scale (VAS), and pelvic-femoral measurements were compared between the two groups. RESULTS: Thirteen cases without sitting pain complaint and 16 cases with sitting pain complaint were stratified to Group N and Group P respectively. No was significant difference in age, body mass index (BMI), and gender between the two groups. Hip morphology measurements (α angle and lateral center-edge angle) and functional measurements (iHOT-12) showed no significant difference between the two groups. However, the mean VAS of pain while sitting was 0.5 ± 0.4 and 1.6 ± 0.6 in Group N and Group P respectively (P = 0.005). Patients with sitting pain complaint have increased pelvic PI compared to those without (50.1° ± 6.5° and 44.2° ± 7.6°, P = 0. 042). The changes in SS (pelvic tilting) from standing to sitting in Group N was significantly larger than that in Group P (21.8° ± 7.0° and 15.1° ± 6.5°, P = 0.012). Although no significant difference in apparent hip flexion and true hip flexion was found. Patients without sitting pain complaint demonstrated a higher pelvic-femoral ratio (22.8% ± 7.9% and 16.1% ± 7.5%, P = 0.010) compared to those with sitting pain complaint. CONCLUSION: Sagittal pelvic-femoral kinematics could have an influence on the symptomology of cam-type FAI. The small PI and insufficient sagittal pelvic tilting in the process of sitting down could be related to the complaint of sitting pain in patients with symptomatic cam-type FAI.

Arthroscopic Treatment for Femoroacetabular Impingement Syndrome with External Snapping Hip: A Comparison Study of Matched Case Series.

OBJECTIVE: To determine the effectiveness of hip arthroscopy combined with endoscopic iliotibial band (ITB) release in patients with both femoroacetabular impingement (FAI) syndrome and external snapping hip (ESH). METHODS: Retrospectively review the preoperative and minimum of 2-year follow-up data of patients with both FAI syndrome and ESH who underwent endoscopic ITB release during hip arthroscopy (FAI + ESH group) from January 2014 to December 2018. The same number of age- and gender-matched FAI syndrome patients without ESH undergoing hip arthroscopy were enrolled in the control group (FAI group). Patient-reported outcomes (PROs) including international Hip Outcome Tool (iHOT-33), modified Harris Hip Score (mHHS), visual analog scale for pain (VAS-pain), and abductive force of affected hip at 3 month and 2 years postoperatively were comparatively analyzed. The VAS-satisfaction score of two groups at 2 years postoperatively were also analyzed. RESULTS: The prevalence of ESH in FAI syndrome patients undergoing hip arthroscopy in our institution was 5.5% (39 of 715 hips), including nine males (10 hips) and 29 females (29 hips). The mean age at the time of surgery was 32.1 ± 6.9 years (range, 22-48 years). According to inclusion and exclusion criteria, 23 patients were enrolled in FAI + ITB group. Twenty-three age- and sex-matched FAI syndrome patients were enrolled in FAI group. At 24 months postoperatively, no patient still suffered ESH symptoms and painful palpation at lateral region in FAI + ITB group. The iHOT-33, mHHS, and VAS-pain score of patients in FAI + ESH group were significantly severer than patients in FAI group preoperatively (41.6 ± 7.5 vs 48.8 ± 7.2, 54.8 ± 7.2 vs 59.2 ± 6.9, 5.5 ± 0.9 vs 4.7 ± 1.0; P < 0.05), while there was no significant difference in these scores between the patients in FAI + ESH group and FAI group at 3-month and 24-month follow-up (73.6 ± 8.5 vs 76.1 ± 6.9, 85.3 ± 7.8 vs 84.2 ± 6.6, 0.8 ± 0.9 vs 0.6 ± 0.9; P > 0.05). At 3 months after surgery, the abductive force of operated hip was significantly smaller than that in FAI group (82.4 ± 12.4 N vs 91.9 ± 16.1 N, P < 0.05), whereas there was no significant difference at 24 months after surgery (101.6 ± 14.9 N vs 106.5 ± 13.7 N, P > 0.05). The VAS-satisfaction scores of patients in the two groups were at a similarly high level (90.5 ± 6.8 vs 88.8 ± 7.3, P > 0.05). There was no complication and no arthroscopic revision in either group until 2-year follow-up. CONCLUSION: Although abductive force recovery of the hip was delayed, hip arthroscopy combined with endoscopic ITB release addressed hip snapping in patients with both FAI syndrome and ESH, and could get similar functional improvement, pain relief, recovery speed, as well as patient satisfaction compared with the pure hip arthroscopy in FAI syndrome patients without ESH.

The Mutated Gene Sequenced by NGS and the Correlation of Their Coexistent Mutual Exclusiveness in NPM1 Mutated Acute Myeloid Leukemia / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinicobiological heterogeneity of NPM1 mutated (NPM1@*METHODS@#The NGS data based on 112 genes related to blood disease in 238 newly diagnosed patients with NPM1@*RESULTS@#Among all the patients, at least one co-mutation was detected out. The median number per case of the mutated genes, including NPM1@*CONCLUSION@#Prognoses of AML involving less common NPM1 missense mutations should be stated on a case by case basis. The mutational landscape and co-occurrence and mutual exclusivity correlations of NPM1

A study on the relationship between mean platelet volume and severe retinopathy of preterm infants / 中国新生儿科杂志

Objective To study the relationship between mean platelet volume (MPV) and severe retinopathy of prematurity (ROP) in preterm infants.Method From January 2012 to June 2017,a retrospective case-control study was performed on preterm infants with gestational age (GA)＜32 weeks admitted to our hospital.The preterm infants with severe ROP were assigned into severe ROP group,and preterm infants with the same GA and birth weight but without ROP were assigned into the control group.The MPV were compared between the two groups,and Logistic regression method was used to analyze the risk factors for severe ROP.Result A total of 82 preterm infants with severe ROP were enrolled,including 51 males and 31 females with GA of 24～31 weeks.The control group also included 82 patients.The mechanical ventilation (MV) duration and continuous positive pressure ventilation duration of preterm infants in severe ROP group were significantly longer than the control group [7.2 (3.9,10.8) d vs.5.6 (3.5,8.9) d,7.8 (4.7,11.6) d vs.5.3 (2.3,9.2) d];the incidences of sepsis and intracranial hemorrhage were significantly higher than the control group [32.9％ (27/82) vs.17.1％ (14/82),31.7％(26/82) vs.17.1％ (14/82)],and the breast feeding rate was significantly lower than the control group[15.8％(13/82)vs.52.4％ (43/82)] (P＜0.05).The MPV of the preterm infants in the severe ROP group was significantly higher than the control group at 36-and 40-week of corrected GA [(10.6± 1.8) fl vs.(10.1± 1.4) fl,(10.8± 1.8) fl vs.(10.2± 1.5) fl] (P＜0.05).Multivariate Logistic regression analysis showed that high birth weight (OR=0.998,95％CI 0.996～ 0.999) protective factor for severe ROP,increased MPV (OR=1.084,95％CI 1.011 ～ 1.163) was risk factor for severe ROP.The ROC curve analysis indicated that threshold values of MPV at corrected GA of 32-week,36-week,and 40-week were 9.9 fl,10.9 fl,and 10.8 fl,respectively;and the sensitivities and specificities were 93.2％ and 68.0％,84.1％,and 92.3％,88.6％ and 89.5％,respectively.Conclusion MPV may be associated with the development of severe ROP in preterm infants.

Plasma Macrophage Migration Inhibitory Factor and CCL3 as Potential Biomarkers for Distinguishing Patients with Nasopharyngeal Carcinoma from High-Risk Individuals Who Have Positive Epstein-Barr Virus Capsid Antigen-Specific IgA / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The purpose of this study was to identify novel plasma biomarkers for distinguishing nasopharyngeal carcinoma (NPC) patients from healthy individuals who have positive Epstein-Barr virus (EBV) viral capsid antigen (VCA-IgA). MATERIALS AND METHODS: One hundred seventy-four plasma cytokines were analyzed by a Cytokine Array in eight healthy individuals with positive EBV VCA-IgA and eight patients with NPC. Real-time polymerase chain reaction, Western blotting, enzyme-linked immunosorbent assay (ELISA), and immunohistochemistry were employed to detect the expression levels of macrophage migration inhibitory factor (MIF) and CC chemokine ligand 3 (CCL3) in NPC cell lines and tumor tissues. Plasma MIF and CCL3 were measured by ELISA in 138 NPC patients, 127 EBV VCA-IgA negative (VN) and 100 EBV VCA-IgA positive healthy donors (VP). Plasma EBV VCA-IgA was determined by immunoenzymatic techniques. RESULTS: Thirty-four of the 174 cytokines varied significantly between the VP and NPC group. Plasma MIF and CCL3 were significantly elevated in NPC patients compared with VN and VP. Combination of MIF and CCL3 could be used for the differential diagnosis of NPC from VN cohort (area under the curve [AUC], 0.913; sensitivity, 90.00%; specificity, 80.30%), and combination of MIF, CCL3, and VCA-IgA could be used for the differential diagnosis of NPC from VP cohort (AUC, 0.920; sensitivity, 90.00%; specificity, 84.00%), from (VN+VP) cohort (AUC, 0.961; sensitivity, 90.00%; specificity, 92.00%). Overexpressions of MIF and CCL3 were observed in NPC plasma, NPC cell lines and NPC tissues. CONCLUSION: Plasma MIF, CCL3, and VCA-IgA combination significantly improves the diagnostic specificity of NPC in high-risk individuals.

Adipose Stem Cells with Conditioned Media for Treatment of Acne Vulgaris Scar.

This study was to investigate the effect of subcutaneous injection of the adipose stem cells (ASCs) with conditioned media (CM) in the treatment of acne vulgaris scar. We used Adult male New Zealand white rabbit ears as an animal model and induced acne formation by Kignman method. Adipose tissue was isolated and harvested from the scapula of rabbits, and ASCs were cultured and expanded until passage 1. There have four groups in our experiment, include phosphate buffered saline (PBS), ASCs with PBS (ASC + PBS), CM, and ASCs with CM (ASC + CM) group. This solution of 0.6 ml injected to subcutaneous in each group. ASC + PBS and ASC + CM groups were containing ASCs of 5.0 × 106 cells/ml. We analyzed the treatment of 4 groups to scar tissue after 2 and 4 weeks by hematoxylin and eosin stain, immunohistochemistry, and RNA expression level of tumor necrosis factor-α (TNF-α), interleukin-1α (IL-1α), and matrix metalloproteinase-2 (MMP-2). Also, the expression of keratin 16 (K16) was detected by western blot analysis. H&E stain showed that infiltration of inflammation cells was significantly reduced at 2 and 4 weeks, as well as re-epithelialization was improved in the ASC + CM group. The ASC + CM gourp was reduced both expression levels of TNF-α, IL-1α, and MMP-2 and K16 protein level. In conclusion, the ASCs with CM has a significant curative effect on acne vulgaris scar, more to the point, the CM has a key role on treatment. It could be applied to a therapeutic approach to regenerate to treat acne vulgaris scar.

Identification and Validation of Circulating MicroRNA Signatures for Breast Cancer Early Detection Based on Large Scale Tissue-Derived Data / 한국유방암학회지

PURPOSE: Breast cancer is the most commonly occurring cancer among women worldwide, and therefore, improved approaches for its early detection are urgently needed. As microRNAs (miRNAs) are increasingly recognized as critical regulators in tumorigenesis and possess excellent stability in plasma, this study focused on using miRNAs to develop a method for identifying noninvasive biomarkers. METHODS: To discover critical candidates, differential expression analysis was performed on tissue-originated miRNA profiles of 409 early breast cancer patients and 87 healthy controls from The Cancer Genome Atlas database. We selected candidates from the differentially expressed miRNAs and then evaluated every possible molecular signature formed by the candidates. The best signature was validated in independent serum samples from 113 early breast cancer patients and 47 healthy controls using reverse transcription quantitative real-time polymerase chain reaction. RESULTS: The miRNA candidates in our method were revealed to be associated with breast cancer according to previous studies and showed potential as useful biomarkers. When validated in independent serum samples, the area under curve of the final miRNA signature (miR-21-3p, miR-21-5p, and miR-99a-5p) was 0.895. Diagnostic sensitivity and specificity were 97.9% and 73.5%, respectively. CONCLUSION: The present study established a novel and effective method to identify biomarkers for early breast cancer. And the method, is also suitable for other cancer types. Furthermore, a combination of three miRNAs was identified as a prospective biomarker for breast cancer early detection.

The efficacy and safety of oral propranolol in the treatment of retinopathy of prematurity / 中国新生儿科杂志

Objective To study the changes of vascular endothelial growth factor ( VEGF) and sE－Selectin in serum before and after oral propranolol therapy for retinopathy of prematurity (ROP), and to study the safety and efficacy of oral administration of propranolol in the treatment of ROP.Method Preterm infants whose gestational age ＜32 weeks and ROP Ⅱstage without plus disease were selected as the objects of our study.The infants were randomly enrolled into treatment and placebo groups in a 1∶1 allocation.The propranolol dosage was 0.25 mg/(kg· d), twice daily orally.The duration of treatment was to complete retinal vessel development or discharge , the longest oral propranolol treatment did not exceed 30 days. Result The incidence of severe ROP in the treatment group was significantly reduced (17.1%vs.37.2%, P＝0.033), and the number requiring laser treatment of the eyes was significantly reduced (3.7%vs.12.8%, P＝0.048).After 10 days of treatment, the serum sE－Selectin decreased significantly in the treatment group, it was significantly lower than that in the placebo group ( P＜0.001).There were no mortalities in the treatment group and the placebo group.The heart rate of the treatment group was lower than that of the placebo group, however, there was no significant difference between the two groups (P＞0.05).There were no significant differences in mean arterial pressure , body weight gain, and urine volume between the two groups (P＞0.05).The serum potassium level in the treatment group was significantly higher than that in the placebo group after the treatment of 20 days and 30 days, [(4.2 ±0.9) mmol/L vs.(3.8 ± 0.4) mmol/L, ( 4.4 ±0.9 ) mmol/L vs.( 3.9 ±0.4 ) mmol/L ], the differences were statistically significant (P＜0.05).However, all the children had normal serum potassium.During treatment, there was no significant differences between the two groups for the incidence of oxygen inhalation and the number of apnea in all children (P＞0.05).Conclusion Propranolol may have a certain therapeutic effect on the progression of ROP.The oral administration was relatively safe and without significant adverse effects.

Clinical features of chronic granulomatous disease :analysis of 27 cases / 中华传染病杂志

Objective To evaluate the clinical features and treatment of chronic granulomatous disease (CGD ) . Methods A total of 27 CGD patients diagnosed by neutrophil respiratory burst in Zhengzhou Children Hospital from January 2007 to June 2017 were selected .The clinical ,pathogenic and radiological characteristics were retrospectively analyzed ,and the treatment and prognosis were evaluated . Chi-square test was used for statistical analysis .Results Among the 27 cases ,all patients presented with pulmonary infection (27/27) and 55 .6％ had cutaneous infection (15/27) .Lymph node enlargement was presented in 48 .1％ (13/27) of patients .Eleven neonates and four infants had cutaneous infection ,which showed significant difference (χ2= 11 .408 , P < 0 .05 ) . The results of pathogenic detection revealed 66 .7％ (18/27) fungal infection ,25 .9％ (7/27) bacterial infection and 44 .4％ (12/27) mycobacterial infection .The incidence rate of fungal infection in neonates was significant higher than that of children (χ2 = 6 .075 , P < 0 .05) .Radiologic examination showed that 12 cases had calcification of subaxillary lymph nodes ,4 cases had pneumonia and thick-walled cavity ,13 cases had multiple nodules ,5 cases had massive high-density shadow and 4 cases had pulmonary abscess .The clinical pathogenic and radiologic features were similar between neonates and children .Treatment options included long-term combination therapy of bactericidal and fungicidal medicines .Conclusions Clinical manifestations of CGD are mainly pulmonary infection as well as cutaneous infection . Combination therapy with multiple medicines is effective .Hematopoietic stem cell transplatation plus retroviral vectors genetic therapy may be the trend of CGD treatment in the future .

Heterogeneity in Spinal Bone Mineral Density Among Young Adults From Three Eastern Provincial Capital Cities in Mainland China.

This study compares spinal volumetric bone mineral density (vBMD) with spinal areal bone mineral density (aBMD) among young adults from 3 eastern provincial capital cities in Mainland China. A total of 416 young adults (age range: 20-40 yr) from 3 eastern provincial capital cities (Beijing, Shanghai, and Guangzhou) in Mainland China were recruited in this study. From each subject, the vBMD of the lumbar spine was measured by the Mindways quantitative computed tomography system. Moreover, the aBMD of the lumbar spine, measured by the dual-energy X-ray absorptiometry, was extracted from a previous multicenter large-scale study, and the 420 participants were matched by age, gender, height, weight, as well as geographic territory. The vBMD and the aBMD values were further compared and analyzed. Generally, the bone mineral density (BMD) results were significantly different among participants from the 3 cities (p <0.05). Specifically, both vBMD and aBMD values of participants from Beijing were significantly different from those from Guangzhou (p <0.05). Additionally, a statistically significant difference in aBMD values was also found between participants from Beijing and Shanghai (p <0.05). However, no significant differences were found between participants from Shanghai and Guangzhou in terms of the aBMD and vBMD values (p1 > 0.05 and p2 > 0.05). Interestingly, the overall mean vBMD value was 5.9% greater in women than those in men for all the 3 cities (p <0.001). This study demonstrated an overall heterogeneity in spinal BMD among young adults from 3 eastern provincial capital cities in Mainland China. Specifically, the taller and heavier young adults from the northern part of China have smaller spinal vBMD but higher spinal aBMD values than those who were shorter and lighter from the southern part of China.

A study on the relationship between the Wnt receptor signaling pathway and severe retinopathy of prematurity / 中国新生儿科杂志

Objective To study the relationship of Wnt receptor signaling pathway and severe retinopathy of prematurity (ROP).Method From January 2011 to June 2015,preterm infants with severe ROP admitted to the NICU of our hospital were enrolled prospectively.Preterm infants with similar gestational age,gender,and age (in days) admitted to our hospital during the same period were selected as the control group.FZD4,LRP5,and ND gene mutations in Wnt receptor signaling pathway were examined.Result A total of 61 Chinese preterm infants were screened for these three candidate genes of Wnt receptor signaling pathway,32 in ROP group and 29 in control group.ND and FZD4 gene mutations were not found among all cases.Eight types of LRP5 mutations were found in 26 cases of ROP group,including 7 cases of Exon18 missense mutation [c.3989C ＞ T;p.Ala1330Val (rs3736228)],5 cases of Exon8 synonymous mutation (c.1647T ＞ C;p.Phe549Phe),5 cases of Exon6 intronic mutation [c.1412 + 8G ＞ A (rs4988319)],3 cases of Exon2 missense mutation [c.266A ＞ G;p.Gln89Arg (rs41494349)],2 cases of Exon21 intronic mutation [c.4349-17C ＞ T (rs372086596)],2 cases of Exon19 synonymous mutation (c.4089C ＞ T;p.Asp 1363 Asp),one case of Exon9 synonymous mutation (c.1 932G ＞ A;p.Glu644Glu),and one case of Exon16 missense mutation (c.3580C ＞T;p.Arg1194Cys).Three types of LRP5 mutations were found in 6 cases of the control group,including 4 cases of Exon8 synonymous mutation,one case of Exon19 synonymous mutation,and one case of Exon9 synonymous mutation.The positive rates of Exonl8 missense mutation and Exon6 intronic mutation in severe ROP group were significantly higher than the control group (P ＜ 0.05).Conclusion LRP5 gene mutations in Wnt receptor signaling pathway may be associated with the occurrence of severe ROP.

RETRACTED: Effect of thioredoxin-interacting protein on Wnt/ß-catenin signaling pathway and diabetic myocardial infarction.

OBJECTIVE: To explore the regulatory role of thioredoxin-interacting protein (TXNIP) in Wnt/ß-catenin signaling pathway and therefore to elucidate its function in diabetic myocardial infarction. METHODS: Diabetic myocardial infarction models were generated in mice. The expression levels of TXNIP and ß-catenin and level of reactive oxygen species (ROS) were determined and compared with those in control group. Human umbilical vein endothelial cells were treated with high-concentration glucose and/or silencing TXNIP and/or H2O2. After 24 h, expression levels of TXNIP, ß-catenin and its downstream protein Cyclin D1, and C-myc gene were determined by real-time PCR, Western blot and immunofluorescence method. The cell proliferation and ROS production capability in different groups were determined by methyl thiazolyl tetrazolium assay. RESULTS: Compared with control group, hyperglycemia significantly up-regulated TXNIP expression and ROS level in the myocardium and endothelial cells of myocardial infarction area, whereas the ß-catenin expression was down-regulated, and the difference was statistically significant (P < 0.05). In comparison with Human umbilical vein endothelial cells in the control group, high glucose level increased the levels of TXNIP expression and ROS level in cells, but reduced cell proliferation as well as migration capability and expression levels of ß-catenin, Cyclin D1 and C-myc; the difference was statistically significant (P < 0.05). However, this trend can be partially reversed by silencing TXNIP. CONCLUSIONS: Diabetic myocardial ischemia could up-regulate levels of TXNIP expression and ROS production in endothelial cells of myocardial infarction area. The regulation effect of TXNIP on ß-catenin was partially achieved by changing ROS levels.

Dual-energy CT angiography for the diagnosis of intracranial dural arteriovenous fistula.

PURPOSE: To investigate the clinical utility of dual-source dual-energy CT angiography (DSDECTA) for diagnosing intracranial dural arteriovenous fistula (DAVF). METHODS: Nine intracranial DAVF patients were examined using Siemens DSDECTA and cerebral digital subtraction angiography (DSA). Imaging data were retrospectively analyzed to evaluate the concordance between the imaging modalities. RESULTS: DSDECTA examination showed that the blood-supplying arteries were thickened and the draining veins and dural sinuses were expanded in all 9 patients. The presence and characteristics of intracranial DAVF were confirmed using DSA. Head CT showed subarachnoid hemorrhage in 4 cases and intracerebral hematoma in 3 cases. CONCLUSION: Although DSA is the gold standard for DAVF diagnosis, DSDECTA is less invasive and more suitable for revealing the three-dimensional structure of secondary intracranial lesions as well as other DAVF characteristics. Thus, DSDECTA may be a new alternative for noninvasive screening of suspected DAVF patients before interventional embolization and surgical resection.

Transcatheter closure of atrial septal defects improves cardiac remodeling and function of adult patients with permanent atrial fibrillation.

BACKGROUND: Permanent atrial fibrillation (AF) is the most common form of dysrhythmia associated with atrial septal defects (ASDs) in patients older than 40 years. However, little is known about cardiac remodeling after transcatheter closure in patients with permanent AF. This study was designed to compare cardiac events and remodeling effects after transcatheter closure in such patients. METHODS: Clinical data of 289 adult patients older than 40 years who underwent ASD closure at our center were analyzed retrospectively. Of them, 63 patients with permanent AF were assigned to the case group, and the other 226 patients without permanent AF were assigned to the control group. Cardiac events and changes in left and right cardiac cavity dimensions before the procedure and 6 months after the procedure were compared between the two groups. RESULTS: Patients in the case group were significantly older than those in the control group. The right ventricular (RV) volume and right atrial (RA) volume were decreased significantly in both the groups during a median follow-up period of 6 months after closure (P < 0.001). The left atrial dimensions, left ventricular end-systolic dimensions, left ventricular end-diastolic dimensions and left ventricular ejection fraction showed no significant change before and after the procedure in both the groups. Changes of the RV volume and RA volume in the case group were significantly smaller than those in the control group (P = 0.005 and P < 0.001). The New York Heart Association cardiac function was improved in both the groups during the 6 months follow-up period. CONCLUSIONS: The transcatheter closure of ASD can improve the cardiac remodeling and cardiac function in patients with or without AF.