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The article "Study on the functions and mechanism of immune functions of human telomerase reverse transcriptase regulating dendritic cells treating sepsis", by H.-M. Chen, L.-Q. Wang, H.-P. Wan, H.-Z. Wei, L.-C. Ke, C.-Y. Liu, Q.-Y. Tan, published in Eur Rev Med Pharmacol Sci 2016; 20 (21): 4500-4507-PMID: 27874963 has been retracted by the Editor in Chief for the following reasons. Some concerns were raised on PubPeer (https://pubpeer.com/publications/3604386A706802443E51758A893D6F) about Figures 3, 4, and 5 showing some overlaps and similar bands in Western blots figures. Furthermore, there is a lack of information regarding the ethics approval for the study involving rats. The journal contacted the authors to request the original raw data and information regarding the ethical approval of the manuscript but never received a reply. Therefore, due to major concerns detected, the Editor in Chief mistrusts the results presented and decided to withdraw the manuscript. The corresponding author has been informed about the retraction. This article has been retracted. The Publisher apologizes for any inconvenience this may cause. https://www.europeanreview.org/article/11476.
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In the early days of the People's Republic of China, Maternal and Child Health Exhibitions were widely held in urban and rural areas across the country. The vivid image and wide coverage of the Exhibition have directly promoted the popularization of a new and more progressive delivery methods in urban and rural areas across the country. While effectively protecting the health of mothers and children, the people's political power is closely linked to "progress".The purpose, organizer and content of the Maternal and Child Health Exhibition are related to the policies of new methods of midwifery, painless delivery, and birth control in the early days of People's Republic of China.It also partly carried the mission of learning from the Soviet Union to "learn from the advanced medical experience", therefore the content in the first ten years was slightly different.
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Salud Infantil , Madres , Niño , Femenino , Humanos , ChinaRESUMEN
Postpancreatectomy acute pancreatitis (PPAP) is an acute inflammation of the remnant pancreas in the early postoperative period caused by a variety of factors. With the progress of related research,PPAP has been confirmed as an independent risk factor for many severe complications such as postoperative pancreatic fistula. In some cases, it progresses to necrotizing PPAP, increasing the risk of mortality. Currently, the International Study Group for Pancreatic Surgery has standardized and graded PPAP as an independent complication, taking into account factors including serum amylase, radiological features, and clinical impact. This review summarizes how the concept of PPAP was proposed, as well as the latest progress in the research related to its etiology, prognosis, prevention, and treatment. However, given the large heterogeneity of relevant studies and the fact that they were mostly retrospective, in the future, it is necessary to place more emphasis on PPAP and elucidate the problems through more standardized studies to optimize strategies for the prevention and management of complications after pancreatic surgery.
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Pancreatitis , Humanos , Pancreatitis/complicaciones , Estudios Retrospectivos , Enfermedad Aguda , Páncreas , Complicaciones Posoperatorias/etiología , Fístula Pancreática/etiología , Pancreaticoduodenectomía/efectos adversosAsunto(s)
Angioplastia de Balón , Antitrombina III , Arteria Pulmonar , Embolia Pulmonar , Tromboembolia , Humanos , Angioplastia de Balón/efectos adversos , Antitrombina III/genética , Antitrombina III/metabolismo , Enfermedad Crónica , Embolia Pulmonar/etiología , Tromboembolia/genética , Tromboembolia/metabolismo , Tromboembolia/terapiaRESUMEN
In the era of vaccine hesitancy, highlighted by the current SARS-CoV2 pandemic, there is an acute need to develop an approach to reduce and address apprehension towards vaccinations. We sought to map and present an overview of existing educational interventions for healthcare providers (HCPs) on strategies to engage in effective vaccine discussion. We applied the Joanna Briggs Institute methodology framework in this scoping review. We searched five relevant databases (MEDLINE, CINAHL, EMBASE, PsycInfo, and SCOPUS) and grey literature through the Google search engine using keywords and subject headings that were systematically identified. We identified 3384 citations in peer-reviewed literature and 41 citations in grey literature. After screening for our inclusion criteria, we included 28 citations from peer reviewed literature and 16 citations from grey literature for analysis. We identified a total of 41 unique education interventions. Interventions were available from multiple disciplines, training levels, clinical settings, and diseases/vaccines. Interventions predominantly centered around two foci: knowledge sharing and communication training. Most interventions identified from peer-reviewed literature were facilitated and were applied with multiple modes of delivery. Interventions from grey literature were more topical and generally self-directed. We identified several gaps in knowledge. Firstly, accessibility and generalizability of interventions was limited. Secondly, distribution of interventions did not adequately address nursing and pharmacy disciplines, and did not cover the breadth of medical specialties for whom vaccine discussions apply. Thirdly, no interventions addressed self monitoring and the clinicians' recognition and management of emotions during difficult conversations. There is a need to address this gap and provide available, credible and comprehensive educational interventions that will support our healthcare providers in effective communication with vaccine hesitant patients.
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COVID-19 , Vacunas , Humanos , Vacilación a la Vacunación , ARN Viral , COVID-19/prevención & control , SARS-CoV-2 , Personal de Salud/educaciónRESUMEN
The aim of this study was to evaluate the resorption of the iliac bone after maxillary reconstruction with a vascularized free iliac flap. Twenty-seven patients with maxillary defects who underwent maxillary reconstruction with the vascularized free iliac flap between January 2017 and January 2021 were included. Computed tomography (CT) images taken at 1 week, approximately 6 months, and 1 year after the surgery were used for evaluation. The total iliac bone thickness and height, cortical bone thickness, and cancellous bone density were measured in the CT images. Compared with 1 week after the surgery, the total thickness and height of the iliac bone were reduced significantly 1 year after the surgery, and the cortical bone thickness and cancellous bone density were reduced significantly at 6 months and 1 year after the surgery. Compared with 6 months after the surgery, cancellous bone density was reduced significantly 1 year after the surgery. In conclusion, during the first year after maxillary reconstruction with a vascularized free iliac flap, there was significant resorption of iliac bone, including the total iliac bone thickness and height, the cortical bone thickness, and the cancellous bone density.
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Resorción Ósea , Colgajos Tisulares Libres , Humanos , Maxilar/cirugía , Tomografía Computarizada por Rayos X , Ilion , Trasplante Óseo/métodosRESUMEN
Objective: To analyze the impact of metabolic risk factors on the epidemiological characteristics of the reactivation of inactive HBsAg carriers (IHC) and provide effective intervention measures to standardize the management of chronic hepatitis B infections. Methods: Based on the chronic hepatitis B infection cohort established in 2010 in Jiangsu province, six follow-up visits from 2012 to 2020 were conducted to analyze the characteristics and influencing factors of the hepatitis B reactivation of IHC and the impact of metabolic risk factors, including obesity, high blood pressure, diabetes and hyperglycemia. Results: From 2012 to 2020, 2 527 IHC and 17 730 person-years were observed during a median follow-up period of 7.0 person-years. Ninety-eight cases of hepatitis B reactivation, with a cumulative reaction rate, was 3.9%, and the incidence density was 5.53/1 000 person-years. Multivariate Cox proportional risk regression analysis showed that age and baseline HBV DNA were independent risk factors of HBV reactivation. Compared with the patients ≥60 years, 40-49 age group (aHR=2.16, 95%CI:1.20-3.90) and 20-29 age group (aHR=5.48, 95%CI:2.07-14.48) were significantly associated with hepatitis B reactivation. Compared with the HBV DNA negative patients at baseline, the risk of hepatitis B reactivation was higher in the group with low HBV DNA level 100-1 999 IU/ml (aHR=1.67, 95%CI:1.11-2.52). Stratification analysis results showed that compared with those without metabolic risk factors, in the ≥50 age group, patients with ≥2 metabolic risk factors showed adjusted HR of 2.73 (95%CI:1.08-6.96). Conclusions: The risk of hepatitis B being reactive is the persistent existence of IHC in communities in Jiangsu province, especially young adults, low-level HBV DNA carriers, and IHC with ≥2 metabolic risk factors. Follow-up for these IHC should be strengthened to reduce the risk of disease progression by antiviral treatment at the right time.
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Hepatitis B Crónica , Hepatitis B , Estudios de Cohortes , ADN Viral , Hepatitis B/epidemiología , Antígenos de Superficie de la Hepatitis B , Virus de la Hepatitis B/genética , Hepatitis B Crónica/epidemiología , Humanos , Factores de Riesgo , Adulto JovenRESUMEN
The modulation p-doping technique has emerged as an effective way to optimize the carrier dynamics process of quantum dot (QD) structures. Here, the laser structures based on the 1.3µm multiple-layer InAs/GaAs QD were fabricated with and without modulation p-doping. The carrier relaxation rate was increased after modulation p-doping, as demonstrated by transient absorption spectroscopy. The higher relaxation rate in p-doped QDs could be explained by more rapid carrier-carrier scattering process originating from increasing of the hole quasi-Fermi-level movement that increases the probability of occupancy of the valence states. In addition, the lasing behavior of Fabry-Perot lasers with and without modulation p-doping was investigated and compared. It was found that the ground state (GS) lasing in the absence of facet coating was successfully achieved in a p-doped laser diode with short cavity length (400µm), which can be attributed to the higher GS saturation gain caused by p-doping. With assistance of a designed TiO2/SiO2facet coating whose central wavelength (â¼1480 nm) is far beyond the lasing wavelength of 1310 nm, the GS lasing could be realized in a laser diode with short cavity lengths (300µm) under continuous wave operation at room temperature, implying great potential for the development of low-cost and high-speed directly modulated lasers.
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Objective: To evaluate the safety and effectiveness of combined hepatic artery resection for the treatment of hilar cholangiocarcinoma. Methods: We searched Pubmed, The Cochrane Library, Embase, Web of Science, China Knowledge Network, Wanfang Data Resource System, Vip-Chinese Sci-tech Journal System Database, and China Biomedical Literature Database, and collected the randomized controlled studies or retrospective studies on the safety and efficacy of combined hepatic artery resection and non-hepatic artery resection in the treatment of hilar cholangiocarcinoma. The search period is from January 1, 2006 to December 31, 2019. Review Manager 5.3 software was used to analyze the extracted data indicators. Results: A total of 14 articles were collected, and a total of 2 374 patients with hilar cholangiocarcinoma were included in the study. Meta-analysis results showed that the perioperative mortality in the hepatic artery resection (HAR) group was higher than that of the control group (OR=1.70, 95%CI=0.02-2.90, P=0.05), and the total postoperative morbidity rate was higher than that of the control group (OR=1.28, 95%CI= 0.93-1.76, P=0.13), both of which were not statistically significant compared with the control group. Subgroup analysis showed that the incidence of liver failure (OR=1.15, 95%CI= 0.73-1.82, P=0.54), biliary fistula (OR=1.20, 95%CI= 0.78-1.84, P=0.40), and abdominal infection in the two groups (OR=0.98, 95%CI= 0.53-1.83, P=0.95) was without significant difference. The R0 resection rate of the HAR group was higher than that of the control group, and the difference was not statistically significant (OR=1.08, 95%CI=0.66-1.75, P=0.77). The rates of lymph node metastasis in the HAR group were higher than that in the control group (OR= 2.48, 95%CI= 1.05-5.84, P=0.04). One-year(OR=0.48, 95%CI= 0.32-0.72, P=0.000 5), 3-year (OR= 0.51, 95%CI=0.36-0.72, P=0.000 1), and 5-year (OR=0.50, 95%CI=0.35-0.70, P<0.000 1) survival rates of HAR group were lower than those of the control group. The survival rates of patients in HAR group treated with combined chemotherapy drugs after operation were significantly improved (OR= 7.33, P=0.02). Conclusions: The safety of combined HAR treatment for hilar cholangiocarcinoma is acceptable, but poor postoperative survival may be related to the high lymph node metastasis rate. Therefore, it is still necessary to be cautious in carrying out this operation. Combined with adjuvant chemotherapy after surgery may improve survival.
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Neoplasias de los Conductos Biliares , Colangiocarcinoma , Tumor de Klatskin , Neoplasias de los Conductos Biliares/cirugía , Conductos Biliares Intrahepáticos , China , Colangiocarcinoma/cirugía , Hepatectomía , Arteria Hepática , Humanos , Tumor de Klatskin/cirugía , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
BACKGROUND: Fever, as an adverse event, is well documented in a wide array of drugs including multiple tyrosine kinase inhibitors however, it is not a previously well described consequence of the novel multi-targeted tyrosine kinase inhibitor, cabozantinib. CASE PRESENTATION: In this paper we document the first detailed review of high-grade fevers in a 54 year old male (Caucasian) with a background of metastatic clear cell renal cell carcinoma recently commenced on cabozantinib. After detailed investigation, we exclude infection and other common causes of fever as the causative agent and further, definitively resolve the recurrent fever by ceasing cabozantinib and starting a short course of oral corticosteroids. CONCLUSIONS: We have demonstrated that cabozantinib should always be considered in the aetiology of high-grade fever in relevant patients. Further, we demonstrate that temporary cessation of cabozantinib and a course of short-term steroids can induce resolution of fever and allow for recommencement of cabozantinib safely thereafter.
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Carcinoma de Células Renales , Neoplasias Renales , Anilidas/efectos adversos , Carcinoma de Células Renales/tratamiento farmacológico , Fiebre/inducido químicamente , Humanos , Neoplasias Renales/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Inhibidores de Proteínas Quinasas/efectos adversos , PiridinasRESUMEN
Objective: To investigate the association of age-related white matter hyperintensity (WMH) with brain atrophy and cognitive impairment in patients with Parkinson's disease (PD). Methods: Consecutive samples of a prospective PD cohort with complete 3-dimensional magnetic resonance imaging in the Department of Movement Disorders in Beijing Tiantan Hospital, Capital Medical University, from October 2018 to August 2019 was retrospectively analyzed. Cognition was evaluated by Mini-Mental State Scale (MMSE) and Montreal Cognitive Assessment (MoCA). The severity of WMH was semi-quantitatively measured by Fazekas scale (0-6 points), and the mean cortical thickness and thalamus volume were calculated by FreeSurfer software. The demographic and disease characteristics, the severity of WMH, the mean cortical thickness and thalamus volume were respectively compared between PD patients with and without dementia. Moreover, univariate and multivariate generalized linear models were used to analyze the correlation of the severity of WMH with brain atrophy and MoCA. Results: A total of 225 patients with PD were included in the study, with a median age of 66 years old. Comparisons between groups suggested that patients with dementia were with severer WMH, older, and had lower levels of serum cholesterol and low-density lipoprotein and more reduced mean cortical thickness than those without dementia (all P<0.05), but no significant difference in the thalamus volume was found between the two groups. The generalized linear model showed that the cognitive impairment of PD patients was significantly correlated with WMH (ß=-0.021, 95%CI:-0.040--0.002, P=0.032), but independent of age, cortical thickness, and levels of serum cholesterol and low-density lipoprotein. Conclusion: WMH may worsen PD cognitive impairment independent of brain atrophy. Clinical prevention and treatment of cerebral small vessel disease may have protective effects on cognitive function in patients with PD.
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Disfunción Cognitiva , Enfermedad de Parkinson , Sustancia Blanca , Anciano , Atrofia/patología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Disfunción Cognitiva/etiología , Disfunción Cognitiva/patología , Humanos , Imagen por Resonancia Magnética , Pruebas Neuropsicológicas , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/patología , Estudios Prospectivos , Estudios Retrospectivos , Sustancia Blanca/patologíaRESUMEN
Objective: To investigate the efficacy and safety of rapamycin in children with tuberous sclerosis complex (TSC) associated renal disease. Methods: A prospective self-control study was conducted. The clinical data of 92 children diagnosed with tuberous sclerosis complex associated kidney disease at the People's Liberation Army General Hospital from January 2011 to January 2019 were collected. The long-term rapamycin treatment for all patients initiated at 1 mg/(m(2)·d), which was gradually adjusted to reach a blood concentration of 5-10 µg/L. The changes of the maximum diameter of renal lesions in children after rapamycin treatment were observed and analyzed with Wilcoxon test. Results: Ninety-two children, including 52 males and 40 females, who met the criteria were analyzed. Sixty patients had only renal angiomyolipoma(RAML), while 24 patients had only multiple renal cysts(MRC), and 8 patients had both lesions. The age of TSC diagnosis was 16.0 (7.0, 42.0) months, and the age of initial treatment with rapamycin was 63.5 (21.0, 103.0) months. The follow-up lasted for 12.0 (4.0, 23.0) months. Sequencing of TSC1 and TSC2 genes was performed in 54 children with TSC, including 3 patients (6%) with mutations in TSC1 gene and 51 patients (94%) with mutations in TSC2 gene. The maximum RAML diameter before treatment was 7.0 (4.0, 9.0) mm. The best effect reached at 3 months of treatment, with the diameter of 4.0 (0,7.0) mm. The maximum diameters at 6 months, 1 year and 1-2 years were 5.0 (0,9.8) mm, 5.0 (1.5, 8.5) mm, 5.5 (3.0, 9.0) mm, respectively, and were significantly different from the baseline (Z=-2.404,-2.350,-2.750,P=0.016,0.019,0.006, respectively). The maximum diameter after 2-3 years, and ≥3 years were 5.0 (3.9,7.0) mm and 6.0 (1.0, 11.0) mm, without significant difference from the baseline (Z=-0.856,-0.102,P=0.393,0.919, respectively).The maximum diameters of MRC after 3 months, 6 months, 1 year,1-2 years, 2-3 years, and ≥3 years were 11.0 (5.0, 14.0) mm,3.0 (0.0,11.0) mm,5.0 (0,21.0) mm,0 (0,14.0) mm,0 (0,10.0) mm, and 0 (0,18.3) mm, respectively, but were not significantly different rom the baseline (7.0 (5.0, 15.7) mm)(Z=-0.944,-1.214,-1.035,-1.896,-1.603,-1.214,P=0.345,0.225,0.301,0.058,0.109,0.225, respectively).Twenty-nine patients (32%) had oral ulcers during the entire treatment period, and no serious adverse reactions were observed. Conclusions: Rapamycin could decrease the diameter of TSC-related RAML, but could not inhibit the growth of cysts. It is well tolerated in the treatment of renal diseases associated with tuberous sclerosis complex.
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Angiomiolipoma/tratamiento farmacológico , Antineoplásicos/uso terapéutico , Neoplasias Renales/tratamiento farmacológico , Sirolimus/uso terapéutico , Esclerosis Tuberosa/complicaciones , Angiomiolipoma/etiología , Niño , China , Femenino , Humanos , Recién Nacido , Neoplasias Renales/etiología , Masculino , Estudios Prospectivos , Sirolimus/administración & dosificaciónRESUMEN
We experimentally demonstrate the first use of 1550-nm InAs/GaAs quantum dot semiconductor saturable absorber mirror (QD-SESAM) in the dual-wavelength passively Q-switched (QS) erbium doped fiber (EDF) laser. The dual-wavelength QS lasing was obtained at a pump threshold of 180â mW with the average output power of 2.2â mW and the spacing between the two lasing wavelengths is 14â nm. A large absorption ranging from 1520 to 1590â nm has been realized when no substrate rotation was employed during the molecular beam epitaxy growth of the QD-SESAM indicating the potential to generate a 60â nm spacing of the dual-wavelength QS lasing peaks by changing the positions in the QD-SESAM and replacing EDF by co-doped fiber as gain medium. These results have provided a new opportunity towards achieving the stable and wide wavelength-tunable dual-modes fiber lasers.
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Through transcription, reprinting and continuous study by the Japanese, the research topic of Suwen(, Plain Questions) has been continuously integrated into Japanese traditional medicine. The editions of Suwen from Edo Period (1603-1867) are Chinese originally printed and Japanese reprinted, which called "Japan block-printed edition" in today's Japan. The survey shows the editions of Chinese Suwen in Japan are divided into three categories: the total-24-volume editions from Chinese original copy are 8 versions; the total-12-volume editions are 7 versions, and the versions with notations and comments are 10 kinds; about the "Japan block-printed edition" , the total-24-volume editions are 7 versions, the total-12-volume editions are 1 version, and the versions with notations and comments are 11 kinds.
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Objective: To evaluate the feasibility and clinical value of suprapubic-assisted laparoendoscopic single-site surgery (SA-LESS) in nephroureterectomy using method of transvaginal natural orifice specimen extraction (NOSE) (SA-LESS+TV-NOSE NU). Methods: Four patients (three cases of renal pelvic carcinoma and one case of ureteral carcinoma) undergoing SA-LESS+TV-NOSE NU were enrolled between April 2015 and January 2016. After general anesthesia, the patients were placed in the lithotomy position with the affected side elevated by 60°. Two trocars were inserted at the medial margin of umbilicus, and the third one was inserted into abdominal cavity at the superior margin of pubic symphysis. The operation was performed under a direct vision with a 5.4 mm 0° flexible-tip laparoscope. Firstly, the distal ureter was isolated completely and blocked by a Hem-O-lok clip. Then, the laparoscopic nephrectomy was performed according to the standard method. Finally, the bladder cuff excision was executed and the incision was sutured. The intact specimen was placed inside a homemade bag and removed through the incision at posterior vaginal fornix. Results: All the procedures were successfully performed. The median operative time was 150 (range: 120 to 210) minutes, and the median estimated blood loss was 180 (range: 80 to 350) ml. No major perioperative complications occurred. The mean visual analogue score (VAS) of 24 hours and 48 hours after operation were 3.25 (range: 2 to 5) and 2.25 (range: 2 to 3). All the patients resumed ambulation on postoperative day 1. Pelvic drainage tube was removed on postoperative day 2-4. On postoperative day 7, urethral catheter was removed. The patients were discharged on postoperative day 7-9. During the follow-up of 20-29 months, the patient recovered well with no case of incisional hernia and pelvic, abdominal infections. The vaginal fornix incision healed well, and the umbilical and suprapubic puncture scars were not obvious. All the patients completed the patient-assessed acromegaly symptom questionnaire PASQ. The average PSAQ score of 3 months after surgery was 34.5. Three of them restarted their sex lives, with an average female sexual function index score of 16.0, which was not significantly different with that of preoperation (15.6). There was no tumor recurrence, metastasis and implantation in all cases. Conclusion: SA-LESS+TV-NOSE NU is safe and feasible for upper tract urothelial carcinoma with faster postoperative recovery, less pain, shorter hospitalization time, better cosmetic results, and does not cause negative effect on the female sexual function.
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Neoplasias Renales , Laparoscopía , Femenino , Humanos , Neoplasias Renales/cirugía , Recurrencia Local de Neoplasia , Nefrectomía , Nefroureterectomía , OmbligoRESUMEN
OBJECTIVE: To explore the correlation between the rs2067853 polymorphism in angiotensinogen (AGT) gene and neonatal hypoxic-ischemic encephalopathy (HIE). PATIENTS AND METHODS: A total of 96 neonatal patients with HIE and 123 healthy neonates were selected. General clinical data were collected and TaqMan-MGB probe method was adopted to detect the rs2067853 polymorphism in angiotensinogen (AGT) gene. RESULTS: The frequency of advanced maternal age, low maternal age, maternal renal insufficiency, abnormal labor, amniotic fluid contamination and umbilical cord abnormality in the observation group was higher than that in the control group (p<0.05), and there was no significant difference between the two groups in the frequency of pregnancy-induced hypertension or eclampsia, maternal anemia, routine prenatal examination, natural childbirth, placental abnormality and abnormal birth weight (p>0.05). There was a difference in genotype distribution frequency between the two groups (p<0.05), while there was no difference in the allele distribution frequency between the two groups (p>0.05). The recessive model had differences between the two groups (p<0.05), while the dominant and additive model had no differences between the two groups (p>0.05). CONCLUSIONS: HIE is correlated with maternal factors, fetal growth, uterine environment and labor process, and the rs2067853 polymorphism in AGT gene is associated with HIE.
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Angiotensinógeno/genética , Hipoxia-Isquemia Encefálica/genética , Polimorfismo de Nucleótido Simple , Estudios de Casos y Controles , Femenino , Estudios de Asociación Genética , Predisposición Genética a la Enfermedad , Humanos , Recién Nacido , Masculino , Edad Materna , Factores de RiesgoRESUMEN
Objective: To explore the clinical and pathological features and mutational types and their relations with WT1 mutation-associated nephropathy (WT1MAN). Methods: The clinical and pathological data and the results of WT1 mutation analysis of the cases from Nanfang Hospital of Southern Medical University, Sun Yat-sen Memorial Hospital and The First Affiliated Hospital of Sun Yat-sen University whom we recruited recently and reported during the last ten years were analyzed. Results: Totally, 20 cases (6 males and 14 females), included 5 newly diagnosed cases, were recruited. (1) Ten children were diagnosed with Denys-Drash syndrome (DDS): The median onset age of proteinuria was 1 year and 7 months. Diffuse mesangial sclerosis (DMS) were revealed in 3 cases, minimal lesions (MCD) in 4 cases, and focal segmental glomerulosclerosis (FSGS) in 1 case; renal pathology was not available in the other 2 cases. Glomerular basement membrane (GBM) thickening was observed in 2 cases. Calcineurin inhibitors (CNIs) were administered in 5 cases, complete remission of proteinuria was observed in 3 cases, partial remission in the other 2 cases. Genetic analysis revealed that six cases had WT1 missense mutation, 3 had nonsense mutation, and 1 had frameshift mutation. (2) Two cases were diagnosed with Frasier syndrome (FS): proteinuria was observed at 1 year and 1 month of age and 1 year and 9 months of age, respectively. FSGS with GBM layering were observed in both cases. They progressed to ESRD at 1 year and 6 months of age and 6 years and 6 months of age, respectively. CNI was tried in 1 case with partial proteinuria remission. Both patients were detected to have WT1 splice mutation. (3) Isolated nephropathy (IN) was observed in 8 cases: three had splice mutation, 5 had missense mutation. Of the 3 patients with splice mutation, one was found to have nephropathy and renal failure at the age of 5 months. The other two cases (1 was FSGS and another MCD), both had GBM layering. CNIs were tried on both of them, one got partial remission with normal renal function at the age of fourteen years, the other one had no response and entered ESRD at the age of 6 years and 9 months. Of the 5 cases with missense mutation, 3 had DMS, 2 of them entered ESRD within 6 months of age, another case had DMS entered ESRD at 9 years of age. One case with FSGS, was treated with CNIs and got complete remission. Conclusions: Slow progression (7/10) nephropathy was observed in DDS patients. Missense mutation (11/20) was the most common type of WT1 variants, followed by splice mutation (5/20) in this group of patients. Early onset nephropathy (4/5), rapid progression (4/5) and GBM layering (4/4) wereobserved in patients with splice mutation. CNI was effective in reducing or even eliminating proteinuria in WT1 MAN patients (8/9).
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Síndrome de Denys-Drash , Enfermedades Renales , Síndrome Nefrótico , Proteínas WT1 , Niño , Síndrome de Denys-Drash/genética , Progresión de la Enfermedad , Femenino , Humanos , Enfermedades Renales/genética , Masculino , Mutación Missense , Síndrome Nefrótico/genética , Resultado del Tratamiento , Proteínas WT1/genéticaRESUMEN
BACKGROUND: LTCBDE combined with or without modified techniques is safe and efficacious for the management of gallstones and concomitant, even large, common bile duct (CBD) stones. METHODS: To describe the surgical indications and procedure strategies of laparoscopic transcystic common bile duct exploration (LTCBDE), a retrospective analysis of 205 patients with concomitant gallstones and CBD stones treated using LTCBDE between June 2008 and June 2015 was performed. Clinical data on disease characteristics, methods for cystic duct incision and CBD stone extraction (with or without laser lithotripsy), and surgical outcomes were collected and reviewed. RESULTS: CBD stones were successfully cleared in all patients. No patient was converted to choledochotomy or laparotomy. The cystic duct diameter ranged 3-8 mm, and 85 patients with cystic duct diameter ≥ 5 mm. The mean time for CBD stone extraction was 25.3 min, with the operative time ranged from 63 to 170 min. Lithotripsy was used in 74 (36.1%) patients among which 26 patients with cystic duct diameter ≥ 5 mm. Estimated blood loss during surgery was 10-120 ml per patient, and no intra-operative blood transfusions were needed. The mean postoperative hospital stay was 5.1 (range 3-7) days, and postoperative complications developed in seven patients. No bile duct injury, stricture, remnant, recurrent stones, or other adverse events were observed during the mean follow-up of 8 months. CONCLUSIONS: Based on preoperative MRCP and intra-operative IOC findings about cystic duct diameter, the diameter of CBD, CBD stone size, we summarized and proposed the surgical indications and suitable techniques and strategies during LTCBDE.