Early Age of Onset, Multiple Primary Malignancies and Poor Prognosis Are Indicative of an Inherited Predisposition to Esophageal Squamous Cell Carcinoma in Familial Rather Than Sporadic Disease- An Update Based on 14- to 23-year Follow-up

OBJECTIVE To demonstrate the effects of an inherited predisposition to familial esophageal squamous cell carcinoma （ESCC） through the comparison and analysis of the clinicopathologic differences between familial and sporadic ESCC cases. METHODS Differences in age of onset, prevalence rates of double primary ESCC, and survival rates between familial ESCC （n = 476） and sporadic ESCC cases （n = 1226） were analyzed. RESULTS Overall, familial ESCC cases showed a significantly younger age of onset （51.9±8.2 vs. 53.4 ±8.0, Pt.test = 0.00）, a significantly higher prevalence rate for double ESCC （2.73 % vs. 1.22%, adjusted with TNM：χMH2 = 4.029, P = 0.045）, and a lower survival rate than in sporadic cases （Pwald = 0.04）. The familial cases showed both a younger age of onset and poorer survival in most subgroups, and the differences were more marked in early-stage rather than in the .late-stage disease groups. CONCLUSION Theses findings confirm the existence of familial as opposed to sporadic ESCC. By the theory of the ＂two-hit＂ origin of cancer, these findings also suggest that the ＂first hit＂, a genetic predisposition, can affect the age of onset, number of primary carcinomas, and the prognosis for familial ESCC patients.

Correlation between age of onset and gastrointestinal stenosis in hospitalized patients with Crohn's disease

BACKGROUND Patients affected by Crohn's disease(CD)are more likely to develop gastrointestinal stenosis and often undergo surgery during the duration of disease.AIM To identify the risk factors for gastrointestinal stenosis in hospitalized CD patients in China.METHODS The clinical data of CD patients hospitalized at the Seventh Medical Center,Chinese People's Liberation Army General Hospital from January 2010 to December 2018 were included.Patients with gastrointestinal stenosis were compared to those without gastrointestinal stenosis for clinical variables.The risk factors for gastrointestinal stenosis were identified using univariate and multivariable logistic regression analyses.The treatments for patients with gastrointestinal stenosis were analyzed,and the characteristics of different treatment methods were discussed.RESULTS The incidence of gastrointestinal stenosis was 59.02%in the 122 hospitalized CD patients.Age of onset of more than 40 years(odds ratio[OR]=3.072,95%confidence interval[CI]:1.298-7.272,P=0.009)and duration of disease of more than 5 years(OR=2.101,95%CI:1.002-4.406,P=0.048)were associated with the occurrence of gastrointestinal stenosis.Fifteen(20.83%)patients did not undergo surgery and received internal medicine and nutrition treatment.Surgical treatments were performed in 72.22%(52)of cases.The rate of postoperative complications was 15.38%(8 cases),and during a median follow-up period of 46 mo,11.54%(6 cases)underwent reoperation.A total of 29.17%(21 cases)were treated with endoscopic therapy,and during a median follow-up period of 32 mo,76.19%(16 cases)had no surgical event,23.81%(5 cases)failed to avoid surgical treatments,and no serious postoperative complications occurred after endoscopic therapy.CONCLUSION Age of onset of more than 40 years and duration of disease of more than 5 years may be strongly correlated with a higher risk of gastrointestinal stenosis in hospitalized CD patients.Endoscopic therapy for gastrointestinal stenosis is relatively safe and effective,and may help to prevent or delay surgery.

Age at diagnosis of type 2 diabetes and cardiovascular risk factor profile:A pooled analysis

BACKGROUND The diagnosis of type 2 diabetes(T2D)in younger adults,an increasingly common public health issue,is associated with a higher risk of cardiovascular complications and mortality,which may be due to a more adverse cardiovascular risk profile in individuals diagnosed at a younger age.AIM To investigate the association between age at diagnosis and the cardiovascular risk profile in adults with T2D.METHODS A pooled dataset was used,comprised of data from five previous studies of adults with T2D,including 1409 participants of whom 196 were diagnosed with T2D under the age of 40 years.Anthropometric and blood biomarker measurements included body weight,body mass index(BMI),waist circumference,body fat percentage,glycaemic control(HbA1c),lipid profile and blood pressure.Univariable and multivariable linear regression models,adjusted for diabetes duration,sex,ethnicity and smoking status,were used to investigate the association between age at diagnosis and each cardiovascular risk factor.RESULTS A higher proportion of participants diagnosed with T2D under the age of 40 were female,current smokers and treated with glucose-lowering medications,compared to participants diagnosed later in life.Participants diagnosed with T2D under the age of 40 also had higher body weight,BMI,waist circumference and body fat percentage,in addition to a more adverse lipid profile,compared to participants diagnosed at an older age.Modelling results showed that each one year reduction in age at diagnosis was significantly associated with 0.67 kg higher body weight[95%confidence interval(CI):0.52-0.82 kg],0.18 kg/m^(2) higher BMI(95%CI:0.10-0.25)and 0.32 cm higher waist circumference(95%CI:0.14-0.49),after adjustment for duration of diabetes and other confounders.Younger age at diagnosis was also significantly associated with higher HbA1c,total cholesterol,low-density lipoprotein cholesterol and triglycerides.CONCLUSION The diagnosis of T2D earlier in life is associated with a worse cardiovascular risk factor profile,compared to those diagnosed later in life.

Impact of age and tumor size on the development of the Kasabach-Merritt phenomenon in patients with kaposiform hemangioendothelioma:a retrospective cohort study

Introduction The Kasabach–Merritt phenomenon(KMP)is a severe complication of kaposiform hemangioendothelioma(KHE).The risk factors for KMP need further investigation.Methods The medical records of patients with KHE were reviewed.Univariate and multivariate logistic regression models were used for the risk factors for KMP,and the area under the receiver operator characteristic(ROC)curve was used to assess the predictive power of risk factors.Results A total of 338 patients with KHE were enrolled.The incidence of KMP was 45.9%.Age of onset(P<0.001,odds ratio[OR]0.939;95%confidence interval[CI]0.914–0.966),lesion size(P<0.001,OR 1.944;95%CI 1.646–2.296),mixed type(P=0.030,OR 2.428;95%CI 1.092–5.397),deep type(P=0.010,OR 4.006;95%CI 1.389–11.556),and mediastinal or retroperitoneal lesion location(P=0.019,OR 11.864;95%CI 1.497–94.003)were correlated with KMP occurrence through multivariate logistic regression.ROC curve analysis revealed that the optimal cutoffs were 4.75 months for the age of onset(P<0.001,OR 7.206,95%CI 4.073–12.749)and a lesion diameter of 5.35 cm(P<0.001,OR 11.817,95%CI 7.084–19.714).Bounded by a lesion size of 5.35 cm,we found significant differences in tumor morphology,age of onset,treatments,and hematological parameters.Using an onset age of 4.75 months as a cutoff,we found significant differences in tumor morphology,lesion size,hematological parameters,and prognosis.Conclusion For KHE patients with an onset age<4.75 months and/or lesion diameter>5.35 cm,clinicians should be wary of the occurrence of KMP.Active management is recommended to improve the prognosis.

Effects of nape acupuncture combined with swallowing rehabilitation on dysphagia in pseudobulbar palsy

OBJECTIVE: To evaluate the therapeutic effects of nape acupuncture combined with rehabilitative swallowing training for dysphagia caused by pseudobulbar palsy, and to compare it with rehabilitative swallowing training alone, and to observe the improvement in quality of life after the therapy.METHODS: One hundred patients were randomly divided into two groups: the rehabilitative swallowing training group(control group, n = 50) and the nape acupuncture combined with rehabilitative swallowing training group(experimental group,n = 50). Each group had 8 weeks' therapy, 5 times a week. Patients in the control group received rehabilitative swallowing training, while those in the experimental group received nape acupuncture therapy based on swallowing rehabilitation. The out-comes were assessed by the repetitive saliva-swallowing test(RSST), water swallow test(WST), standardized swallowing assessment(SSA), and a swallow quality-of-life questionnaire(SWAL-QOL). Correlations of onset age, onset frequency and lesion location with the efficacy of the acupuncture treatment were also observed.RESULTS: The scores for RSST, WST, and SSA in both groups were lower than before the therapy(P <0.001), although the changes were more marked in the experimental group than in the control group(RSST and WST, P < 0.005; SSA, P < 0.001). Both groups recorded changes in SWAL-QOL index after the therapy(P < 0.001); and the experimental group scored higher than the control group(P < 0.001). The efficacy of acupuncture was not correlated with location(P > 0.05), but was related to onset age(P < 0.05) and onset frequency(P < 0.01).CONCLUSION: Nape acupuncture combined with rehabilitative swallowing training has an effect on dysphagia caused by pseudobulbar palsy and improves quality of life.