The therapeutic effects of intensive insulin therapy in treatment of traumatic shock combined with multiple organ dysfunction syndrome （MODS） were investigated. A total of 114 patients with traumatic shock combined ...The therapeutic effects of intensive insulin therapy in treatment of traumatic shock combined with multiple organ dysfunction syndrome （MODS） were investigated. A total of 114 patients with traumatic shock combined with MODS were randomly divided into two groups： control group （n=56） treated with conventional therapy, and intensive insulin therapy group （n=58） treated with conventional therapy plus continuous insulin pumping to control the blood glucose level at range of 4.4-6.1 mmol/L. White blood cells （WBC） counts, prothrombin time （PT）, serum creatinine （SCr）, alanine aminotransferase （ALT）, serum albumin and PaO2 were measured before and at the day 1, 3, 5, 7 and 14 after treatment. The incidence of gastrointestinal dysfunction, the incidence of MODS, hospital stay and the mortality were also observed and compared. After intensive insulin therapy, the WBC counts, SCr, ALT and PT were significantly reduced （P0.05）, but the level of serum albumin was significantly increased （P0.05） at the day 3, 5, 7 and 14. In the meantime, the PaO2 was significantly elevated at the day 3, 5 and 7 （P0.01） after intensive insulin therapy. The incidence of gastrointestinal dysfunction, the incidence of MODS, the length of hospital stay and the mortality were markedly decreased （P0.01）. The results suggest early treatment with intensive insulin therapy is effective for traumatic shock combined with MODS and can decrease the length of hospital stay and the mortality.展开更多
AIM:To investigate the association of gastroesophageal reflux disease (GERD) and irritable bowel syndrome (IBS) in Iranian patients and examine the prevalence of functional symptoms of the gastrointestinal tract in pa...AIM:To investigate the association of gastroesophageal reflux disease (GERD) and irritable bowel syndrome (IBS) in Iranian patients and examine the prevalence of functional symptoms of the gastrointestinal tract in patients presenting with either IBS, GERD or both.METHODS: Six thousand four hundred and seventy six patients presented to the Gastro-intestinal (GI) clinic with symptoms of functional dysfunction of GI tract, 1419 patients (62.0% women, 38.0% men; mean age: 37.4±11.5 years) met Rome or Rome criteria(depending on the year of diagnosis)for IBS.2658 patients were diagnosed with GERD based on clinical presentation and endoscopic findings.We assessed other functional symptoms(epigastric pain,nausea,vomiting,belching,constipation and diarrhea)in patients suffering from GERD,IBS or both.RESULTS: Among IBS subjects, 63.6% (69.0% women, 31.0% men; mean age: 36.4±10.3 years) also hadGERD, whereas 34.7% of the non-IBS patients had GERD [odds ratio (OR) =3.2, 95% confidence interval (CI): 2.9-3.7, P<0.0001]. Among patients with GERD, 33.9% of subjects met Rome criteria compared to 13.5% of non-GERD patients (OR=3.6, 95% CI: 3.1-4.3, P<0.0001). Prevalence of all functional symptoms was higher in overlapping GERD and IBS subjects, when compared with their prevalence in the IBS subjects without GERD or GERD only subjects (P<0.05).CONCLUSION: This finding shows that in overlapping GERD and IBS, other functional abnormalities of the GI tract are also highly prevalent, suggesting a common underlying dysfunction.展开更多
This study aimed to assess the association between psychological disorders and erectile dysfunction(ED)in patients with different degrees of chronic prostatitis/chronic pelvic pain syndrome(CP/CPPS).This was a retrosp...This study aimed to assess the association between psychological disorders and erectile dysfunction(ED)in patients with different degrees of chronic prostatitis/chronic pelvic pain syndrome(CP/CPPS).This was a retrospective study conducted from June 2017 to October 2019 and included 182 outpatients.Patients were interviewed using the Structured Interview on Erectile Dysfunction(SIEDY)for pathogenic quantification.The National Institutes of Health-Chronic Prostatitis Symptom Index(NIH-CPSI)and the International Index of Erectile Function-5(IIEF-5)were used for the evaluation of CP/CPPS and ED.The Generalized Anxiety Disorder-7(GAD-7)and Patient Health Questionnaire-9(PHQ-9)were used to assess anxiety symptoms and depressive symptoms.The number of patients with mild CP/CPPS and mild ED,mild CP/CPPS and moderate-to-severe ED,moderate-to-severe CP/CPPS and mild ED,and moderate-to-severe CP/CPPS and moderate-to-severe ED was 69(37.9%),36(19.8%),35(19.2%),and 42(23.1%),respectively.The corresponding PHQ-9 scores of the four groups were 6.22,7.19,10.69,and 7.71,respectively.The corresponding GAD-7 scores of the four groups were 5.26,6.31,8.77,and 6.36,respectively.Among patients with moderate-to-severe CP/CPPS,the PHQ-9 and GAD-7 scores of the moderate-to-severe ED group were significantly lower than those of the mild ED group(P=0.007 and P=0.010,respectively).The prevalence of ED and premature ejaculation(PE)in patients with moderate-to-severe CP/CPPS was significantly higher than that in patients with mild CP/CPPS(P=0.001 and P=0.024,respectively).Our findings proved that the severity of ED was negatively associated with psychological symptoms in outpatients with moderate-to-severe CP/CPPS.展开更多
BACKGROUND：Gastrointestinal motility dysfunction in multiple organ dysfunction syndrome （MODS） has been reported to be related to damage to interstitial cells of Cajal （ICC）. In the entedc nervous system, ICC and...BACKGROUND：Gastrointestinal motility dysfunction in multiple organ dysfunction syndrome （MODS） has been reported to be related to damage to interstitial cells of Cajal （ICC）. In the entedc nervous system, ICC and smooth muscle cells are connected in a network to form a special functional unit. Many gastrointestinal motility dysfunction diseases are associated with damage to this network.OBJECTIVE：To investigate the morphological changes of intestinal ICC, and to explore the mechanisms underlying gastrointestinal motility dysfunction in rats with MODS.DESIGN, TIME AND SE＇I-FING：The randomized, controlled, experiment was performed at the Central Laboratory of the First Affiliated Hospital of Dalian Medical University of China between June 2007 and March 2009.MATERIALS：Escherichia coli （E. colistrain O127 H6） and bovine serum albumin were purchased from Sigma, USA.METHODS：A total of 40 Wistar rats were equally and randomly divided into MODS group and control group. Suspension of E. coil strain O127 H6 containing BaSO4 and saline were sterilely injected into the abdominal cavity of rats in the MODS and control groups, respectively.MAIN OUTCOME MEASURES：Immunohistochemical double-staining and confocal laser scanning microscopy were used to observe the morphological changes in intestinal cholinergic nerves and ICC in the deep muscular plexus network. Electron microscopy was employed to evaluate the ultrastructural features of ICC in the deep muscular plexus of rats with MODS.RESULTS：Compared with the control group, the distributions and densities of cholinergic/nitrergic newes and ICC in the deep muscular plexus were significantly decreased in the MODS group （P 〈 0.01）. The enteric nerve-ICC network were disrupted.CONCLUSION：There is ultrastructural injury in the ICC in the deep muscular plexus and enteric nerves of the intestine in rats with MODS, which may be associated with the dysmotility of the gastrointestinal tract in MODS.展开更多
BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory ...BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory mediators has become a hot topic in critical care medicine.Researchers hypothesize that continuous blood purification(CBP) can effectively eliminate a variety of inflammatory mediators which participate in the occurrence of MODS and adjust the immune imbalance.This study aimed to observe the effects of CBP in MODS patients.METHODS:In this retrospective clinical study,a total of 38 MODS patients,18 males and 20 females,were enrolled.After conventional therapy,all the patients received CBP.Biochemistry,blood gas analysis,oxygenation index,mean arterial blood pressure(MAP),acute physiology and chronic health evaluation(APACHE) II scores were monitored.RESULTS:After CBP,the vital signs of patients were rapidly stable,and electrolyte disorders and acid-base imbalance were corrected.Renal function,blood gas,oxygenation index were all improved.MAP was increased,and APACHE II score was significantly decreased.All patients had good tolerance,stable hemodynamics,and no obvious adverse reaction on CBP compared with pre-CBP.CONCLUSION:CBP can effectively clean toxins,correct electrolyte acid-base balance,and improve systemic inflammatory response syndrome and the organ function of MODS patients.展开更多
Objective To establish a model of multiple organ dysfunction syndrome in the elderly (MODSE) by intraperitoneal injection of different doses of zymosan, and to compare the multiple organ dysfunction syndrome (MODS) in...Objective To establish a model of multiple organ dysfunction syndrome in the elderly (MODSE) by intraperitoneal injection of different doses of zymosan, and to compare the multiple organ dysfunction syndrome (MODS) in adult and in the elderly rats. Methods Adult and senile rats, injected with different doses of zymosan intraperitoneally were examined for the changes in the function and morphology of the vital organs, including heart, liver, brain, lungs, and kidneys using blood gas and biochemistry analysis and histopathological examination methods. Results Compared with the normal controls of the adult and the elderly rats, the blood gas and blood biochemistry changed in different degrees in the different dosed zymosan groups. Pathological changes were also found in the vital organs including lungs, heart, liver, brain, kidneys, erc in the experimental groups. Under the same concentrations of zymosan, the reductions in respiratory, cardiac and renal functions in the senile groups were much more severe than those in the corresponding adult group. In the similar degree of model duplication, the senile rats had the tendency to die later than the adult rats. Conclusions Zymosan can be used in both elderly and adult rats to induce MODS model, and the best dosage for MODSE was 0.Sg/kg injected peritoneally. The model would hopefully be used in the study of mechanisms and the therapeutics on MODSE.展开更多
The pathophysiology of irritable bowel syndrome（IBS） is complex and not fully understood, so the aim of this study was to evaluate whether visceral and somatic hypersensitivity, autonomic cardiovascular dysfunction,...The pathophysiology of irritable bowel syndrome（IBS） is complex and not fully understood, so the aim of this study was to evaluate whether visceral and somatic hypersensitivity, autonomic cardiovascular dysfunction, and low-grade inflammation of the gut wall are associated with diarrhea-predominant IBS（D-IBS）. Sixty-two patients with D-IBS and 20 control subjects participated in the study. Using the ascending method of limits（AML） protocol, we demonstrated that D-IBS patients had significantly lower sensory thresholds compared with healthy controls（P〈0.001）. Using diverse methods, especially the ischemic sensitivity test, for the first time in China, we confirmed that D-IBS patients have somatic hypersensitivity. They had a significantly higher systolic blood pressure and heart rate after a cold stimulus, indicative of autonomic cardiovascular dysfunction. Compared with the control group, D-IBS patients had a significantly higher level of calprotectin（P〈0.001）. We also found significant correlations between visceral and somatic hypersensitivity, visceral hypersensitivity and autonomic cardiovascular dysfunction, and somatic hypersensitivity and autonomic cardiovascular dysfunction. Our findings may provide valuable suggestions for the treatment of D-IBS.展开更多
Aim: To determine the relationship between metabolic syndrome (MS) and erectile dysfunction (ED) and to see which risk factors correlated the best with ED. Methods: Seventy-nine cardiology clinic outpatients with coro...Aim: To determine the relationship between metabolic syndrome (MS) and erectile dysfunction (ED) and to see which risk factors correlated the best with ED. Methods: Seventy-nine cardiology clinic outpatients with coronary artery disease (CAD) and lipid metabolism disorder were recruited. They were categorized as having MS, hypertension (blood pressure greater than 130/85 mmHg) and dyslipidemia. ED was classified based on International Index of Erectile Function scores. Patients were grouped into quartiles based on body mass index (BMI). Chi-square, Pearson's correlation and regression tests were used for statistical analysis. Results: The mean age of the patients was 56.6 years. ED was diagnosed in 59 (74.7 %) of the 79 patients. In the 38 patients with MS, all had ED. ED was not significantly correlated with cholesterol levels (P > 0.05), but was found often in patients who had both hyperc-holesterolemia and HT (P<0.01). Nineteen(76 %) of the 25 patients who had dyslipidemia had ED. However, ED was not significantly correlated with dyslipidemia (P > 0.05). Tweenty-two of the 23 patients who had BMI greater than 30 had ED, which was significantly more prevalent than that in those who had normal BMI (P<0.01). ED was seen in 38 of 53 smoker patients. Although ED was more prevalent in cigarette smokers, it was not significantly different from non-smokers (P>0.5). Conclusion: ED is present in a high percentage of patients with MS. Among multiple risk factors for ED, MS correlates the most highly. The next most important risk group is the patients with hypertension +hypercholestrolemia and obesity (BMI > 30).展开更多
Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family ...Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family with ARC syndrome. All 23 coding exons and flanking introns of the VPS33B gene were amplified and sequenced using peripheral lymphocyte genomic DNA of the patient and her parents. Genetic testing revealed two novel mutations (c.1033delA and c.1567C>T) in the VPS33B gene. The patient is a compound heterozygote and her parents were heterozygous for each of the mutations.展开更多
Aim： To evaluate the effects of metabolic profiles on lower urinary tract symptoms （LUTS） in men with erectile dysfunction （ED）. Methods： A total of 75 impotent men aged 25-75 years old （mean 58.1 years） were ...Aim： To evaluate the effects of metabolic profiles on lower urinary tract symptoms （LUTS） in men with erectile dysfunction （ED）. Methods： A total of 75 impotent men aged 25-75 years old （mean 58.1 years） were included in the study on a prospective basis. Patients were evaluated with a complete history, physical examination, anthropometry and metabolic profiles. LUTS were assessed using the International Prostate Symptom Score （IPSS）. Results： Overall, there was no correlation between the IPSS and continuous parameters. However, when continuous variables were categorized, some parameters were significantly associated with LUTS. Patients with triglyceride level of 150 mg/dL or higher had more severe symptoms than those with tiglyceride levels less than 150 mg/dL （19.4±2.4 vs. 14.3±1.1, P = 0.033）. When 40 mg/dL was chosen as the high-density lipoprotein （HDL）-cholesterol cut-off level, the IPSS was significantly different between the two groups divided by 40 mg/dL （19.4±2.6 for HDL-cholesterol 〈 40 mg/dL vs. 14.4±1.0 for HDL-cholesterol 2 40 mg/dL, P = 0.042）. The area under the receiver operating characteristic curve （AUROCC） of triglyceride was 65.7% （95% confidence interval [CI], 52.6%-82.3%; P = 0.034） for severe LUTS. However, the AUROCC for ＇HDL-cholesterol＇ was not significant （area, 65.4%; 95% CI, 48.2%-82.7%; P = 0.062）. No other factors were determined to be significant in this regard. Conclusion： The results of the present study indicate that some metabolic profiles might influence LUTS in men with ED.展开更多
BACKGROUND:Blood hemoperfusion with resin adsorption can clean larger molecules that exceed the molecular weight cutoff of combined continuous veno-venous hemofiltration(CWH).Hence blood hemoperfusion with resin adsor...BACKGROUND:Blood hemoperfusion with resin adsorption can clean larger molecules that exceed the molecular weight cutoff of combined continuous veno-venous hemofiltration(CWH).Hence blood hemoperfusion with resin adsorption combined CWH(HP+CWH) has higher ability of mediator clearance,and can improve clinical outcomes in theory.This study aimed to investigate the effect of blood hemoperfusion with resin adsorption combined continuous veno-venous hemofiltration(HP+CWH) on plasm cytokines like TNF-α,IL-1β,IL-6,cellular immunity and prognosis in patients with multiple organ dysfunction syndrome(MODS).METHODS:This was a prospective,randomized clinical trial.A total of 30 patients who had been diagnosed with MODS were enrolled in this study.Patients were randomly allocated to routine treatment+HP+CWH group(treatment group) and routine treatment+only CWH group(control group).In the treatment group,patients received blood hemoperfusion with resin adsorption for 2hours,and then received CWH for 10 hours every day.In the control group,patients received CWH for 12 hours only every day.The patients in the two groups received blood purification therapy for three days.The plasma of patients in the treatment group was obtained at 0,2,12,24,26,36,48,50,60 hours,5th day,7th day and 10 th day,respectively.The plasma of patients in the control group was obtained at 0,12,24,36,48,60 hours,5th day,7th day and 10 th day,respectively.APACHE Ⅱscore,T-lymphocytes subpopulations,blood lactate acid concentration,heart rate,breathing rate,and oxygenation index were observed.RESULTS:Plasma cytokines like TNF-α,IL-1β,IL-6 decreased markedly after HP(P<0.01);T-lymphocytes subpopulations CD3+,CD4+,CD8+,CD4+/CD8+ increased after HP+CWH or only CWH.The plasma concentrations of TNF-α,IL-1β and IL-6 in the two groups were not markedly different at 12,36,and 50 hours.But on the 5th day,the plasma concentrations of TNF-α,IL-1β and IL-6 in the treatment group were lower than those in the control group(P<0.05).On the 28 th day,5patients died in the treatment group,and 6 patients in the control group.CONCLUSIONS:Both HP+CWH and CWH can clean plasma cytokines like TNF-α,IL-1β,and IL-6,and improve cellular immunity and clinical symptoms and signs of patients.Compared with only CWH,the plasma concentrations of TNF-α,IL-1β and IL-6 were lower on the 5th day,and patients have an increased survival rate on the 28 day in the HP+CWH group.展开更多
Aim: A new concept of locomotive syndrome has been proposed by the Japanese Orthopaedic Association. The aim of this study is to clarify the utility of its self-checklist, “loco-check,” as a tool for estimating the ...Aim: A new concept of locomotive syndrome has been proposed by the Japanese Orthopaedic Association. The aim of this study is to clarify the utility of its self-checklist, “loco-check,” as a tool for estimating the physical dysfunction of elderly people. Methods: Subjects were 1124 community-dwelling Japanese people, 557 men and 567 women, aged 40-89 years. Information about the seven “loco-check” items was obtained from present inquiry sheets. Physical functions were examined by grip strength, knee extension strength, walking speed and one-leg standing time with open eyes. The averages of these test values, controlled for age and BMI, were compared between the “loco-check” (+) group and the “loco-check” (-) group. Also we examined about the trend of decline of physical function, together with SF36 physical function subscale score, as the number of the items chosen increased. Results: Adjusted average values of all four physical function examinations in the “lococheck” (+) group were significantly lower than those of the “loco-check” (-) group (all, p . Also the adjusted average values of the majority of four tests were significantly lower in those who checked each of the “loco-check” items than those who did not, for most of the items. It was also revealed that the more items subjects checked, the lower the adjusted average values were, except for one-leg standing time. It was also the case with SF36 physical function subscale score. Conclusion: We showed the utility of “loco-check” as a simple tool not only for noticing the physical dysfunction of elderly people, but also for estimating the extent of it, except for balancing ability, particularly by counting the number of checked items.展开更多
AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a majo...AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a major form of aqueous deficient DED and meibomian gland dysfunction(MGD), a common cause of evaporative DED.METHODS: A total 135 eyes of 135 subjects included in this study: patients with DED associated with coGVHD(n=30), patients with SS(n=35), patients with MGD(n=35), and normal controls(n=35). All participants completed the Ocular Surface Disease Index(OSDI) questionnaire, ocular surface examination [Schirmer test, tear film breakup time(TFBUT), and ocular surface staining], and meibomian gland assessment [meiboscore(gland dropout detected on meibography using infrared camera of the Keratograph 5 M), meibum expressibility score(MES), meibum quality score(MQS), lid margin abnormality]. In addition, correlations of meibomian gland characteristics with ocular surface parameters as well as disease severity score were investigated in coGVHD group.RESULTS: The coGVHD group showed significantly higher meiboscore, MES, and MQS than the other 3 groups(all P<0.05). In the coGVHD group, parameters of meibomian gland showed a significant correlation each other and those of ocular surface. The correlation between meibomian gland parameters and severity score of co GVHD was also established(meiboscore, r=0.62; MES, r=0.47; MQS, r=0.47; lid margin abnormality score, r=0.55; all P<0.05).CONCLUSION: Patients with DED associated with co GVHD show poorer gland morphology and worse glandfunction than other types of DED. In addition, meibomian gland damage is not only associated with ocular surface damage but also disease severity of coGVHD.展开更多
A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated in...A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.展开更多
Objective： To provide the clinical evidence to evaluate the feasibility and refine the protocol for acupotomy combined with fire needle and pudendal nerve block therapy in treatment of sacral nerve dysfunction syndr...Objective： To provide the clinical evidence to evaluate the feasibility and refine the protocol for acupotomy combined with fire needle and pudendal nerve block therapy in treatment of sacral nerve dysfunction syndrome（SNDS）. Methods： Seventy-five patients with SNDS were randomized into the treatment group（acupotomy and fire needle and pudendal nerve block therapy） and the control group（pudendal nerve block therapy）. After a course of treatment, Visual Analogue Scales（VAS） of anorectal pain, defecation disorders, anal incontinence, VAS of lumbar pain or soreness, VAS of abdominal distension and pain were compared before and after the treatment. Result： Scores of defecation disorders, including defecation interval time index, defecation time index, fecal property index and defecation difficulty index, of patients with SNDS in the two groups were statistically different before and after the treatment in the same group（all P〈0.05）, but the differences of those indexes between two groups were not statistically significant（all P〈0.05） after the treatment,. Scores of anal incontinence, VAS scores of lumbar pain or soreness, VAS scores of abdominal pain and distension in the two groups were statistically different before and after the treatment（all P〈0.05）. However, after treatment, the differences between two groups were not statistically significant（all P〈0.05）. VAS scores of anorectal pain in the two groups were statistically different before and after the treatment（both P〈0.05）, and that of the treatment group was statistically lower than control group after the treatment（1.61 ± 0.95 vs. 3.04 ± 1.81, P〈0.01）, the total effective rate of the treatment group was higher than that of the control group, and the difference was statistically significant（94.74% vs. 81.08%, P〈0.01）, there was no difference in self-evaluation between the two groups（P〈0.05）. Conclusion： In treating SNDS, acupotomy combined with fire needle and pudendal nerve block therapy can more effectively alleviate anorectal pain and improve the total effective rate.展开更多
BACKGROUND Metabolic syndrome(MetS)is related to poor cognitive function.However,the results of previous studies were inconsistent,and whether the ApoEε4 allele modifies the association remains unclear.AIM To elucida...BACKGROUND Metabolic syndrome(MetS)is related to poor cognitive function.However,the results of previous studies were inconsistent,and whether the ApoEε4 allele modifies the association remains unclear.AIM To elucidate the relationships among MetS,ApoEε4,and cognitive dysfunction in an elderly population in China.METHODS One hundred elderly patients with MetS and 102 age-and gender-matched controls were included in the study.Baseline clinical characteristics and biochemical index for glucose and lipid metabolism were obtained.The distribution of ApoEε4 was assessed with PCR restriction fragment length polymorphism analysis.Cognitive function was evaluated by mini-mental status examination at the 1-year follow-up examination.RESULTS Compared with controls,MetS patients had worse cognitive function and decreased ability to participate in activities of daily life(P=0.001 and 0.046,respectively).Patients with cognitive dysfunction had higher prevalence of MetS(62.1%vs 36.4%,P<0.001)and were more likely to carry the ApoEε4 allele(22.3%vs 10.1%,P=0.019).Multivariate logistic regression analyses showed that diagnosis with MetS,severe insulin resistance,status as an ApoEε4 carrier,higher systolic blood pressure,and larger waist circumference were risk factors for cognitive dysfunction(P<0.05).Repeated-measures analysis of variance,performed with data collected at the 1-year follow-up,revealed continuous influences of MetS and ApoEε4 on the deterioration of cognitive function(time×team,P<0.001 for both).CONCLUSION Diagnosis of MetS and ApoEε4 carrier status were persistently associated with cognitive dysfunction among an elderly population in China.展开更多
BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and...BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and systematically review the previous literature.CASE SUMMARY Here, we report a 9.6-month-old Chinese girl who was diagnosed with MSMDS based on her history and symptoms, such as recurrent cough, wheezing, and complications with congenital fixed dilated pupils. Chest high-resolution computed tomography revealed inhomogeneous lung transparency, obvious exudative lesions, and some lung fissures that were markedly thickened. Cranial magnetic resonance imaging excluded bleeding and infarction but showed abnormal signals in the centrum ovale majus and bilateral periventricular regions. Echocardiography only showed patent foramen ovale, and no patent ductus arteriosus, pulmonary artery dilatation, or pulmonary hypertension was found. Bronchoscopy indicated moderate bronchial malacia. These examinations in conjunction with the typical eye abnormality suggested a diagnosis of MSMDS, and sequencing of exon 6 of the ACTA2 gene demonstrated the heterozygous mutation c.536 G>A, p.R179 H. However, her parents' gene analyses were normal.CONCLUSION MSMDS is a rare genetic disease mainly caused by the mutation of the ACTA2 gene p.R179 H. Early genetic diagnosis should be performed for children presenting with congenital fixed dilated pupils and patent ductus arteriosus.During the process of diagnosis and treatment, clinicians should be on high alert for cerebrovascular, cardiovascular, and pulmonary complications.展开更多
BACKGROUND ACTA2 gene is a specific gene that encodes actinα2.Multisystem smooth muscle dysfunction syndrome(MSMDS)is a multisystem disease characterized by aortic and cerebrovascular lesions caused by ACTA2 gene mut...BACKGROUND ACTA2 gene is a specific gene that encodes actinα2.Multisystem smooth muscle dysfunction syndrome(MSMDS)is a multisystem disease characterized by aortic and cerebrovascular lesions caused by ACTA2 gene mutations.There have been many reports of cardiac,pulmonary and cerebrovascular lesions caused by MSMDS;however,few studies have focused on seizures caused by MSMDS.CASE SUMMARY Our patient was a girl aged 7 years and 8 mo with recurrent cough,asthma and seizures for 7 years.She was diagnosed with severe pneumonia,congenital heart disease,cardiac insufficiency,and malnutrition in the local hospital.Cardiac ultrasonography revealed congenital heart disease,patent ductus arteriosus(with a diameter of 0.68 cm),left coronary arteriectasis,patent oval foramen(0.12 cm),tricuspid and pulmonary regurgitation,and pulmonary hypertension.Cerebral magnetic resonance imaging and magnetic resonance angiography indicated stiffness in the brain vessels,together with multiple aberrant signaling shadows in bilateral paraventricular regions.A heterozygous mutation(c.536G>A)was identified in the ACTA2 gene,resulting in generation of p.R179H.Finally,the girl was diagnosed with MSMDS combined with epilepsy.The patient had 4 episodes of seizures before treatment,and no onset of seizure was reported after oral administration of sodium valproate for 1 year.CONCLUSION MSMDS has a variety of clinical manifestations and unique cranial imaging features.Cerebrovascular injury and white matter injury may lead to seizures.Gene detection can confirm the diagnosis and prevent missed diagnosis or misdiagnosis.展开更多
Background:Multiple mitochondrial dysfunction syndromes(MMDS)presents as complex mitochondrial damage,thus impairing a variety of metabolic pathways.Heart dysplasia has been reported in MMDS patients;however,the speci...Background:Multiple mitochondrial dysfunction syndromes(MMDS)presents as complex mitochondrial damage,thus impairing a variety of metabolic pathways.Heart dysplasia has been reported in MMDS patients;however,the specific clinical symptoms and pathogenesis remain unclear.More urgently,there is a lack of an animal model to aid research.Therefore,we selected a reported MMDS causal gene,Isca1,and established an animal model of MMDS complicated with cardiac dysplasia.Methods:The myocardium-specific Isca1 knockout heterozygote(Isca1 HET)rat was obtained by crossing the Isca1 conditional knockout(Isca1 cKO)rat with theαmyosin heavy chain Cre(α-MHC-Cre)rat.Cardiac development characteristics were determined by ECG,blood pressure measurement,echocardiography and histopatho-logical analysis.The responsiveness to pathological stimuli were observed through adriamycin treatment.Mitochondria and metabolism disorder were determined by activity analysis of mitochondrial respiratory chain complex and ATP production in myocardium.Results:ISCA1 expression in myocardium exhibited a semizygous effect.Isca1 HET rats exhibited dilated cardiomyopathy characteristics,including thin-walled ventri-cles,larger chambers,cardiac dysfunction and myocardium fibrosis.Downregulated ISCA1 led to deteriorating cardiac pathological processes at the global and organiza-tional levels.Meanwhile,HET rats exhibited typical MMDS characteristics,including damaged mitochondrial morphology and enzyme activity for mitochondrial respira-tory chain complexesⅠ,ⅡandⅣ,and impaired ATP production.Conclusion:We have established a rat model of MMDS complicated with cardiomyopathy,it can also be used as model of myocardial energy metabolism dysfunction and mitochondrial cardiomyopathy.This model can be applied to the study of the mechanism of energy metabolism in cardiovascular diseases,as well as research and development of drugs.展开更多
BACKGROUND The VPS33B(OMIM:608552)gene is located on chromosome 15q26.1.We found a female infant with autosomal recessive arthrogryposis,renal dysfunction and cholestasis syndrome 1(ARCS1)caused by mutation in VPS33B....BACKGROUND The VPS33B(OMIM:608552)gene is located on chromosome 15q26.1.We found a female infant with autosomal recessive arthrogryposis,renal dysfunction and cholestasis syndrome 1(ARCS1)caused by mutation in VPS33B.The child was diagnosed with ARCS1(OMIM:208085)after the whole exome sequencing revealed two heterozygous mutations(c.96+1G>C,c.242delT)in the VPS33B gene.CASE SUMMARY We report a Chinese female infant with neonatal cholestasis disorder,who was eventually diagnosed with ARCS1 by genetic analysis.Genetic testing revealed two new mutations(c.96+1G>C and c.242delT)in VPS33B,which is the causal gene.The patient was compound heterozygous,and her parents were both heterozygous.CONCLUSION This study extends the mutational spectrum of the VPS33B gene to provide a molecular basis for the etiological diagnosis of ARCS1 and for genetic counseling of the family.展开更多
基金supported by a grant from National Natural Sciences Foundation of China (No. 30870951)
文摘The therapeutic effects of intensive insulin therapy in treatment of traumatic shock combined with multiple organ dysfunction syndrome （MODS） were investigated. A total of 114 patients with traumatic shock combined with MODS were randomly divided into two groups： control group （n=56） treated with conventional therapy, and intensive insulin therapy group （n=58） treated with conventional therapy plus continuous insulin pumping to control the blood glucose level at range of 4.4-6.1 mmol/L. White blood cells （WBC） counts, prothrombin time （PT）, serum creatinine （SCr）, alanine aminotransferase （ALT）, serum albumin and PaO2 were measured before and at the day 1, 3, 5, 7 and 14 after treatment. The incidence of gastrointestinal dysfunction, the incidence of MODS, hospital stay and the mortality were also observed and compared. After intensive insulin therapy, the WBC counts, SCr, ALT and PT were significantly reduced （P0.05）, but the level of serum albumin was significantly increased （P0.05） at the day 3, 5, 7 and 14. In the meantime, the PaO2 was significantly elevated at the day 3, 5 and 7 （P0.01） after intensive insulin therapy. The incidence of gastrointestinal dysfunction, the incidence of MODS, the length of hospital stay and the mortality were markedly decreased （P0.01）. The results suggest early treatment with intensive insulin therapy is effective for traumatic shock combined with MODS and can decrease the length of hospital stay and the mortality.
基金Supported by Digestive disease research center,Tehran University of Medical Sciences
文摘AIM:To investigate the association of gastroesophageal reflux disease (GERD) and irritable bowel syndrome (IBS) in Iranian patients and examine the prevalence of functional symptoms of the gastrointestinal tract in patients presenting with either IBS, GERD or both.METHODS: Six thousand four hundred and seventy six patients presented to the Gastro-intestinal (GI) clinic with symptoms of functional dysfunction of GI tract, 1419 patients (62.0% women, 38.0% men; mean age: 37.4±11.5 years) met Rome or Rome criteria(depending on the year of diagnosis)for IBS.2658 patients were diagnosed with GERD based on clinical presentation and endoscopic findings.We assessed other functional symptoms(epigastric pain,nausea,vomiting,belching,constipation and diarrhea)in patients suffering from GERD,IBS or both.RESULTS: Among IBS subjects, 63.6% (69.0% women, 31.0% men; mean age: 36.4±10.3 years) also hadGERD, whereas 34.7% of the non-IBS patients had GERD [odds ratio (OR) =3.2, 95% confidence interval (CI): 2.9-3.7, P<0.0001]. Among patients with GERD, 33.9% of subjects met Rome criteria compared to 13.5% of non-GERD patients (OR=3.6, 95% CI: 3.1-4.3, P<0.0001). Prevalence of all functional symptoms was higher in overlapping GERD and IBS subjects, when compared with their prevalence in the IBS subjects without GERD or GERD only subjects (P<0.05).CONCLUSION: This finding shows that in overlapping GERD and IBS, other functional abnormalities of the GI tract are also highly prevalent, suggesting a common underlying dysfunction.
基金the Fundamental Research Funds for the Central.Universities of Central South University(20191039 to ZCL)the National Natural Science of China(81570627 to ZYT)+1 种基金the Natural Science Foundation of Hunan Province,China(2020115906 to DIL)the China Postdoctoral Science Foundation(2020M670107ZX to DJL).
文摘This study aimed to assess the association between psychological disorders and erectile dysfunction(ED)in patients with different degrees of chronic prostatitis/chronic pelvic pain syndrome(CP/CPPS).This was a retrospective study conducted from June 2017 to October 2019 and included 182 outpatients.Patients were interviewed using the Structured Interview on Erectile Dysfunction(SIEDY)for pathogenic quantification.The National Institutes of Health-Chronic Prostatitis Symptom Index(NIH-CPSI)and the International Index of Erectile Function-5(IIEF-5)were used for the evaluation of CP/CPPS and ED.The Generalized Anxiety Disorder-7(GAD-7)and Patient Health Questionnaire-9(PHQ-9)were used to assess anxiety symptoms and depressive symptoms.The number of patients with mild CP/CPPS and mild ED,mild CP/CPPS and moderate-to-severe ED,moderate-to-severe CP/CPPS and mild ED,and moderate-to-severe CP/CPPS and moderate-to-severe ED was 69(37.9%),36(19.8%),35(19.2%),and 42(23.1%),respectively.The corresponding PHQ-9 scores of the four groups were 6.22,7.19,10.69,and 7.71,respectively.The corresponding GAD-7 scores of the four groups were 5.26,6.31,8.77,and 6.36,respectively.Among patients with moderate-to-severe CP/CPPS,the PHQ-9 and GAD-7 scores of the moderate-to-severe ED group were significantly lower than those of the mild ED group(P=0.007 and P=0.010,respectively).The prevalence of ED and premature ejaculation(PE)in patients with moderate-to-severe CP/CPPS was significantly higher than that in patients with mild CP/CPPS(P=0.001 and P=0.024,respectively).Our findings proved that the severity of ED was negatively associated with psychological symptoms in outpatients with moderate-to-severe CP/CPPS.
基金Supported by the National Natural Science Foundation of China,No. 30772860
文摘BACKGROUND：Gastrointestinal motility dysfunction in multiple organ dysfunction syndrome （MODS） has been reported to be related to damage to interstitial cells of Cajal （ICC）. In the entedc nervous system, ICC and smooth muscle cells are connected in a network to form a special functional unit. Many gastrointestinal motility dysfunction diseases are associated with damage to this network.OBJECTIVE：To investigate the morphological changes of intestinal ICC, and to explore the mechanisms underlying gastrointestinal motility dysfunction in rats with MODS.DESIGN, TIME AND SE＇I-FING：The randomized, controlled, experiment was performed at the Central Laboratory of the First Affiliated Hospital of Dalian Medical University of China between June 2007 and March 2009.MATERIALS：Escherichia coli （E. colistrain O127 H6） and bovine serum albumin were purchased from Sigma, USA.METHODS：A total of 40 Wistar rats were equally and randomly divided into MODS group and control group. Suspension of E. coil strain O127 H6 containing BaSO4 and saline were sterilely injected into the abdominal cavity of rats in the MODS and control groups, respectively.MAIN OUTCOME MEASURES：Immunohistochemical double-staining and confocal laser scanning microscopy were used to observe the morphological changes in intestinal cholinergic nerves and ICC in the deep muscular plexus network. Electron microscopy was employed to evaluate the ultrastructural features of ICC in the deep muscular plexus of rats with MODS.RESULTS：Compared with the control group, the distributions and densities of cholinergic/nitrergic newes and ICC in the deep muscular plexus were significantly decreased in the MODS group （P 〈 0.01）. The enteric nerve-ICC network were disrupted.CONCLUSION：There is ultrastructural injury in the ICC in the deep muscular plexus and enteric nerves of the intestine in rats with MODS, which may be associated with the dysmotility of the gastrointestinal tract in MODS.
文摘BACKGROUND:The complications of systemic inflammatory response syndrome(SIRS)include acute lung injury,acute kidney injury,shock,and multiple organ dysfunction syndrome(MODS).In recent years,how to clear inflammatory mediators has become a hot topic in critical care medicine.Researchers hypothesize that continuous blood purification(CBP) can effectively eliminate a variety of inflammatory mediators which participate in the occurrence of MODS and adjust the immune imbalance.This study aimed to observe the effects of CBP in MODS patients.METHODS:In this retrospective clinical study,a total of 38 MODS patients,18 males and 20 females,were enrolled.After conventional therapy,all the patients received CBP.Biochemistry,blood gas analysis,oxygenation index,mean arterial blood pressure(MAP),acute physiology and chronic health evaluation(APACHE) II scores were monitored.RESULTS:After CBP,the vital signs of patients were rapidly stable,and electrolyte disorders and acid-base imbalance were corrected.Renal function,blood gas,oxygenation index were all improved.MAP was increased,and APACHE II score was significantly decreased.All patients had good tolerance,stable hemodynamics,and no obvious adverse reaction on CBP compared with pre-CBP.CONCLUSION:CBP can effectively clean toxins,correct electrolyte acid-base balance,and improve systemic inflammatory response syndrome and the organ function of MODS patients.
文摘Objective To establish a model of multiple organ dysfunction syndrome in the elderly (MODSE) by intraperitoneal injection of different doses of zymosan, and to compare the multiple organ dysfunction syndrome (MODS) in adult and in the elderly rats. Methods Adult and senile rats, injected with different doses of zymosan intraperitoneally were examined for the changes in the function and morphology of the vital organs, including heart, liver, brain, lungs, and kidneys using blood gas and biochemistry analysis and histopathological examination methods. Results Compared with the normal controls of the adult and the elderly rats, the blood gas and blood biochemistry changed in different degrees in the different dosed zymosan groups. Pathological changes were also found in the vital organs including lungs, heart, liver, brain, kidneys, erc in the experimental groups. Under the same concentrations of zymosan, the reductions in respiratory, cardiac and renal functions in the senile groups were much more severe than those in the corresponding adult group. In the similar degree of model duplication, the senile rats had the tendency to die later than the adult rats. Conclusions Zymosan can be used in both elderly and adult rats to induce MODS model, and the best dosage for MODSE was 0.Sg/kg injected peritoneally. The model would hopefully be used in the study of mechanisms and the therapeutics on MODSE.
基金Project supported by the National Science & Technology Pillar Program during the Twelfth Five-year Plan Period of China(No.2014BAI08B02)the National Science & Technology Major Projects(Major Drug Discovery)of China(No.2012ZX09103201-021)
文摘The pathophysiology of irritable bowel syndrome（IBS） is complex and not fully understood, so the aim of this study was to evaluate whether visceral and somatic hypersensitivity, autonomic cardiovascular dysfunction, and low-grade inflammation of the gut wall are associated with diarrhea-predominant IBS（D-IBS）. Sixty-two patients with D-IBS and 20 control subjects participated in the study. Using the ascending method of limits（AML） protocol, we demonstrated that D-IBS patients had significantly lower sensory thresholds compared with healthy controls（P〈0.001）. Using diverse methods, especially the ischemic sensitivity test, for the first time in China, we confirmed that D-IBS patients have somatic hypersensitivity. They had a significantly higher systolic blood pressure and heart rate after a cold stimulus, indicative of autonomic cardiovascular dysfunction. Compared with the control group, D-IBS patients had a significantly higher level of calprotectin（P〈0.001）. We also found significant correlations between visceral and somatic hypersensitivity, visceral hypersensitivity and autonomic cardiovascular dysfunction, and somatic hypersensitivity and autonomic cardiovascular dysfunction. Our findings may provide valuable suggestions for the treatment of D-IBS.
文摘Aim: To determine the relationship between metabolic syndrome (MS) and erectile dysfunction (ED) and to see which risk factors correlated the best with ED. Methods: Seventy-nine cardiology clinic outpatients with coronary artery disease (CAD) and lipid metabolism disorder were recruited. They were categorized as having MS, hypertension (blood pressure greater than 130/85 mmHg) and dyslipidemia. ED was classified based on International Index of Erectile Function scores. Patients were grouped into quartiles based on body mass index (BMI). Chi-square, Pearson's correlation and regression tests were used for statistical analysis. Results: The mean age of the patients was 56.6 years. ED was diagnosed in 59 (74.7 %) of the 79 patients. In the 38 patients with MS, all had ED. ED was not significantly correlated with cholesterol levels (P > 0.05), but was found often in patients who had both hyperc-holesterolemia and HT (P<0.01). Nineteen(76 %) of the 25 patients who had dyslipidemia had ED. However, ED was not significantly correlated with dyslipidemia (P > 0.05). Tweenty-two of the 23 patients who had BMI greater than 30 had ED, which was significantly more prevalent than that in those who had normal BMI (P<0.01). ED was seen in 38 of 53 smoker patients. Although ED was more prevalent in cigarette smokers, it was not significantly different from non-smokers (P>0.5). Conclusion: ED is present in a high percentage of patients with MS. Among multiple risk factors for ED, MS correlates the most highly. The next most important risk group is the patients with hypertension +hypercholestrolemia and obesity (BMI > 30).
基金Supported by National Natural Science Foundation of China,No.81070281
文摘Arthrogryposis, renal dysfunction and cholestasis (ARC) syndrome is a rare genetic disorder and has not been described in China. We present a female infant with neonatal intrahepatic cholestasis from a Chinese family with ARC syndrome. All 23 coding exons and flanking introns of the VPS33B gene were amplified and sequenced using peripheral lymphocyte genomic DNA of the patient and her parents. Genetic testing revealed two novel mutations (c.1033delA and c.1567C>T) in the VPS33B gene. The patient is a compound heterozygote and her parents were heterozygous for each of the mutations.
文摘Aim： To evaluate the effects of metabolic profiles on lower urinary tract symptoms （LUTS） in men with erectile dysfunction （ED）. Methods： A total of 75 impotent men aged 25-75 years old （mean 58.1 years） were included in the study on a prospective basis. Patients were evaluated with a complete history, physical examination, anthropometry and metabolic profiles. LUTS were assessed using the International Prostate Symptom Score （IPSS）. Results： Overall, there was no correlation between the IPSS and continuous parameters. However, when continuous variables were categorized, some parameters were significantly associated with LUTS. Patients with triglyceride level of 150 mg/dL or higher had more severe symptoms than those with tiglyceride levels less than 150 mg/dL （19.4±2.4 vs. 14.3±1.1, P = 0.033）. When 40 mg/dL was chosen as the high-density lipoprotein （HDL）-cholesterol cut-off level, the IPSS was significantly different between the two groups divided by 40 mg/dL （19.4±2.6 for HDL-cholesterol 〈 40 mg/dL vs. 14.4±1.0 for HDL-cholesterol 2 40 mg/dL, P = 0.042）. The area under the receiver operating characteristic curve （AUROCC） of triglyceride was 65.7% （95% confidence interval [CI], 52.6%-82.3%; P = 0.034） for severe LUTS. However, the AUROCC for ＇HDL-cholesterol＇ was not significant （area, 65.4%; 95% CI, 48.2%-82.7%; P = 0.062）. No other factors were determined to be significant in this regard. Conclusion： The results of the present study indicate that some metabolic profiles might influence LUTS in men with ED.
文摘BACKGROUND:Blood hemoperfusion with resin adsorption can clean larger molecules that exceed the molecular weight cutoff of combined continuous veno-venous hemofiltration(CWH).Hence blood hemoperfusion with resin adsorption combined CWH(HP+CWH) has higher ability of mediator clearance,and can improve clinical outcomes in theory.This study aimed to investigate the effect of blood hemoperfusion with resin adsorption combined continuous veno-venous hemofiltration(HP+CWH) on plasm cytokines like TNF-α,IL-1β,IL-6,cellular immunity and prognosis in patients with multiple organ dysfunction syndrome(MODS).METHODS:This was a prospective,randomized clinical trial.A total of 30 patients who had been diagnosed with MODS were enrolled in this study.Patients were randomly allocated to routine treatment+HP+CWH group(treatment group) and routine treatment+only CWH group(control group).In the treatment group,patients received blood hemoperfusion with resin adsorption for 2hours,and then received CWH for 10 hours every day.In the control group,patients received CWH for 12 hours only every day.The patients in the two groups received blood purification therapy for three days.The plasma of patients in the treatment group was obtained at 0,2,12,24,26,36,48,50,60 hours,5th day,7th day and 10 th day,respectively.The plasma of patients in the control group was obtained at 0,12,24,36,48,60 hours,5th day,7th day and 10 th day,respectively.APACHE Ⅱscore,T-lymphocytes subpopulations,blood lactate acid concentration,heart rate,breathing rate,and oxygenation index were observed.RESULTS:Plasma cytokines like TNF-α,IL-1β,IL-6 decreased markedly after HP(P<0.01);T-lymphocytes subpopulations CD3+,CD4+,CD8+,CD4+/CD8+ increased after HP+CWH or only CWH.The plasma concentrations of TNF-α,IL-1β and IL-6 in the two groups were not markedly different at 12,36,and 50 hours.But on the 5th day,the plasma concentrations of TNF-α,IL-1β and IL-6 in the treatment group were lower than those in the control group(P<0.05).On the 28 th day,5patients died in the treatment group,and 6 patients in the control group.CONCLUSIONS:Both HP+CWH and CWH can clean plasma cytokines like TNF-α,IL-1β,and IL-6,and improve cellular immunity and clinical symptoms and signs of patients.Compared with only CWH,the plasma concentrations of TNF-α,IL-1β and IL-6 were lower on the 5th day,and patients have an increased survival rate on the 28 day in the HP+CWH group.
文摘Aim: A new concept of locomotive syndrome has been proposed by the Japanese Orthopaedic Association. The aim of this study is to clarify the utility of its self-checklist, “loco-check,” as a tool for estimating the physical dysfunction of elderly people. Methods: Subjects were 1124 community-dwelling Japanese people, 557 men and 567 women, aged 40-89 years. Information about the seven “loco-check” items was obtained from present inquiry sheets. Physical functions were examined by grip strength, knee extension strength, walking speed and one-leg standing time with open eyes. The averages of these test values, controlled for age and BMI, were compared between the “loco-check” (+) group and the “loco-check” (-) group. Also we examined about the trend of decline of physical function, together with SF36 physical function subscale score, as the number of the items chosen increased. Results: Adjusted average values of all four physical function examinations in the “lococheck” (+) group were significantly lower than those of the “loco-check” (-) group (all, p . Also the adjusted average values of the majority of four tests were significantly lower in those who checked each of the “loco-check” items than those who did not, for most of the items. It was also revealed that the more items subjects checked, the lower the adjusted average values were, except for one-leg standing time. It was also the case with SF36 physical function subscale score. Conclusion: We showed the utility of “loco-check” as a simple tool not only for noticing the physical dysfunction of elderly people, but also for estimating the extent of it, except for balancing ability, particularly by counting the number of checked items.
基金Supported by the Chonnam National University Hospital Biomedical Research Institute(CRI 18093-1)Basic Science Research Program through the National Research Foundation of Korea(NRF)funded by the Ministry of Science,ICT&Future Planning(No.2017R1A2B4003367)
文摘AIM: To investigate the abnormalities in the meibomian gland in patients with dry eye disease(DED) associated with chronic ocular graft-versus-host disease(coGVHD) in comparison with Sj?gren's syndrome(SS), a major form of aqueous deficient DED and meibomian gland dysfunction(MGD), a common cause of evaporative DED.METHODS: A total 135 eyes of 135 subjects included in this study: patients with DED associated with coGVHD(n=30), patients with SS(n=35), patients with MGD(n=35), and normal controls(n=35). All participants completed the Ocular Surface Disease Index(OSDI) questionnaire, ocular surface examination [Schirmer test, tear film breakup time(TFBUT), and ocular surface staining], and meibomian gland assessment [meiboscore(gland dropout detected on meibography using infrared camera of the Keratograph 5 M), meibum expressibility score(MES), meibum quality score(MQS), lid margin abnormality]. In addition, correlations of meibomian gland characteristics with ocular surface parameters as well as disease severity score were investigated in coGVHD group.RESULTS: The coGVHD group showed significantly higher meiboscore, MES, and MQS than the other 3 groups(all P<0.05). In the coGVHD group, parameters of meibomian gland showed a significant correlation each other and those of ocular surface. The correlation between meibomian gland parameters and severity score of co GVHD was also established(meiboscore, r=0.62; MES, r=0.47; MQS, r=0.47; lid margin abnormality score, r=0.55; all P<0.05).CONCLUSION: Patients with DED associated with co GVHD show poorer gland morphology and worse glandfunction than other types of DED. In addition, meibomian gland damage is not only associated with ocular surface damage but also disease severity of coGVHD.
文摘A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.
基金Youth Foundation Project of Jiangsu Natural Science Foundation of China,NO.BK20160135The twelfth batch of high-level talents training target of "Six Talent Peaks",NO.2015-WSW-070+1 种基金Third level of training target candidates of the fifth phase of the Jiangsu"333 Project":2016Ⅲ-0094Nanjing Medical Science and Technology Development Project,NO.YKK15127~~
文摘Objective： To provide the clinical evidence to evaluate the feasibility and refine the protocol for acupotomy combined with fire needle and pudendal nerve block therapy in treatment of sacral nerve dysfunction syndrome（SNDS）. Methods： Seventy-five patients with SNDS were randomized into the treatment group（acupotomy and fire needle and pudendal nerve block therapy） and the control group（pudendal nerve block therapy）. After a course of treatment, Visual Analogue Scales（VAS） of anorectal pain, defecation disorders, anal incontinence, VAS of lumbar pain or soreness, VAS of abdominal distension and pain were compared before and after the treatment. Result： Scores of defecation disorders, including defecation interval time index, defecation time index, fecal property index and defecation difficulty index, of patients with SNDS in the two groups were statistically different before and after the treatment in the same group（all P〈0.05）, but the differences of those indexes between two groups were not statistically significant（all P〈0.05） after the treatment,. Scores of anal incontinence, VAS scores of lumbar pain or soreness, VAS scores of abdominal pain and distension in the two groups were statistically different before and after the treatment（all P〈0.05）. However, after treatment, the differences between two groups were not statistically significant（all P〈0.05）. VAS scores of anorectal pain in the two groups were statistically different before and after the treatment（both P〈0.05）, and that of the treatment group was statistically lower than control group after the treatment（1.61 ± 0.95 vs. 3.04 ± 1.81, P〈0.01）, the total effective rate of the treatment group was higher than that of the control group, and the difference was statistically significant（94.74% vs. 81.08%, P〈0.01）, there was no difference in self-evaluation between the two groups（P〈0.05）. Conclusion： In treating SNDS, acupotomy combined with fire needle and pudendal nerve block therapy can more effectively alleviate anorectal pain and improve the total effective rate.
基金Supported by Basic and Clinical Cooperation Project of Capital Medical University,No.16JL82.
文摘BACKGROUND Metabolic syndrome(MetS)is related to poor cognitive function.However,the results of previous studies were inconsistent,and whether the ApoEε4 allele modifies the association remains unclear.AIM To elucidate the relationships among MetS,ApoEε4,and cognitive dysfunction in an elderly population in China.METHODS One hundred elderly patients with MetS and 102 age-and gender-matched controls were included in the study.Baseline clinical characteristics and biochemical index for glucose and lipid metabolism were obtained.The distribution of ApoEε4 was assessed with PCR restriction fragment length polymorphism analysis.Cognitive function was evaluated by mini-mental status examination at the 1-year follow-up examination.RESULTS Compared with controls,MetS patients had worse cognitive function and decreased ability to participate in activities of daily life(P=0.001 and 0.046,respectively).Patients with cognitive dysfunction had higher prevalence of MetS(62.1%vs 36.4%,P<0.001)and were more likely to carry the ApoEε4 allele(22.3%vs 10.1%,P=0.019).Multivariate logistic regression analyses showed that diagnosis with MetS,severe insulin resistance,status as an ApoEε4 carrier,higher systolic blood pressure,and larger waist circumference were risk factors for cognitive dysfunction(P<0.05).Repeated-measures analysis of variance,performed with data collected at the 1-year follow-up,revealed continuous influences of MetS and ApoEε4 on the deterioration of cognitive function(time×team,P<0.001 for both).CONCLUSION Diagnosis of MetS and ApoEε4 carrier status were persistently associated with cognitive dysfunction among an elderly population in China.
基金Supported by the National Natural Science Foundation of China,No.81573167Science and Technology Project of Jiangsu,No.BE2017657
文摘BACKGROUND Multisystemic smooth muscle dysfunction syndrome(MSMDS) is a rare genetic disease worldwide. The main mutation is the actin alpha 2(ACTA2) gene p.R179 H. In this paper, we report a Chinese MSMDS patient and systematically review the previous literature.CASE SUMMARY Here, we report a 9.6-month-old Chinese girl who was diagnosed with MSMDS based on her history and symptoms, such as recurrent cough, wheezing, and complications with congenital fixed dilated pupils. Chest high-resolution computed tomography revealed inhomogeneous lung transparency, obvious exudative lesions, and some lung fissures that were markedly thickened. Cranial magnetic resonance imaging excluded bleeding and infarction but showed abnormal signals in the centrum ovale majus and bilateral periventricular regions. Echocardiography only showed patent foramen ovale, and no patent ductus arteriosus, pulmonary artery dilatation, or pulmonary hypertension was found. Bronchoscopy indicated moderate bronchial malacia. These examinations in conjunction with the typical eye abnormality suggested a diagnosis of MSMDS, and sequencing of exon 6 of the ACTA2 gene demonstrated the heterozygous mutation c.536 G>A, p.R179 H. However, her parents' gene analyses were normal.CONCLUSION MSMDS is a rare genetic disease mainly caused by the mutation of the ACTA2 gene p.R179 H. Early genetic diagnosis should be performed for children presenting with congenital fixed dilated pupils and patent ductus arteriosus.During the process of diagnosis and treatment, clinicians should be on high alert for cerebrovascular, cardiovascular, and pulmonary complications.
基金Zhejiang Medical and Health Science and Technology Program,No.2020KY327 and No.2017KY660.
文摘BACKGROUND ACTA2 gene is a specific gene that encodes actinα2.Multisystem smooth muscle dysfunction syndrome(MSMDS)is a multisystem disease characterized by aortic and cerebrovascular lesions caused by ACTA2 gene mutations.There have been many reports of cardiac,pulmonary and cerebrovascular lesions caused by MSMDS;however,few studies have focused on seizures caused by MSMDS.CASE SUMMARY Our patient was a girl aged 7 years and 8 mo with recurrent cough,asthma and seizures for 7 years.She was diagnosed with severe pneumonia,congenital heart disease,cardiac insufficiency,and malnutrition in the local hospital.Cardiac ultrasonography revealed congenital heart disease,patent ductus arteriosus(with a diameter of 0.68 cm),left coronary arteriectasis,patent oval foramen(0.12 cm),tricuspid and pulmonary regurgitation,and pulmonary hypertension.Cerebral magnetic resonance imaging and magnetic resonance angiography indicated stiffness in the brain vessels,together with multiple aberrant signaling shadows in bilateral paraventricular regions.A heterozygous mutation(c.536G>A)was identified in the ACTA2 gene,resulting in generation of p.R179H.Finally,the girl was diagnosed with MSMDS combined with epilepsy.The patient had 4 episodes of seizures before treatment,and no onset of seizure was reported after oral administration of sodium valproate for 1 year.CONCLUSION MSMDS has a variety of clinical manifestations and unique cranial imaging features.Cerebrovascular injury and white matter injury may lead to seizures.Gene detection can confirm the diagnosis and prevent missed diagnosis or misdiagnosis.
基金The present work was supported in part by the Beijing Natural Science Foundation(5212017)CAMS Innovation Fund for Medical Sciences(CIFMS,2016-I2M-1-015)National Natural Science Foundation(31872314 and 31970508).
文摘Background:Multiple mitochondrial dysfunction syndromes(MMDS)presents as complex mitochondrial damage,thus impairing a variety of metabolic pathways.Heart dysplasia has been reported in MMDS patients;however,the specific clinical symptoms and pathogenesis remain unclear.More urgently,there is a lack of an animal model to aid research.Therefore,we selected a reported MMDS causal gene,Isca1,and established an animal model of MMDS complicated with cardiac dysplasia.Methods:The myocardium-specific Isca1 knockout heterozygote(Isca1 HET)rat was obtained by crossing the Isca1 conditional knockout(Isca1 cKO)rat with theαmyosin heavy chain Cre(α-MHC-Cre)rat.Cardiac development characteristics were determined by ECG,blood pressure measurement,echocardiography and histopatho-logical analysis.The responsiveness to pathological stimuli were observed through adriamycin treatment.Mitochondria and metabolism disorder were determined by activity analysis of mitochondrial respiratory chain complex and ATP production in myocardium.Results:ISCA1 expression in myocardium exhibited a semizygous effect.Isca1 HET rats exhibited dilated cardiomyopathy characteristics,including thin-walled ventri-cles,larger chambers,cardiac dysfunction and myocardium fibrosis.Downregulated ISCA1 led to deteriorating cardiac pathological processes at the global and organiza-tional levels.Meanwhile,HET rats exhibited typical MMDS characteristics,including damaged mitochondrial morphology and enzyme activity for mitochondrial respira-tory chain complexesⅠ,ⅡandⅣ,and impaired ATP production.Conclusion:We have established a rat model of MMDS complicated with cardiomyopathy,it can also be used as model of myocardial energy metabolism dysfunction and mitochondrial cardiomyopathy.This model can be applied to the study of the mechanism of energy metabolism in cardiovascular diseases,as well as research and development of drugs.
基金Supported by the Hainan Province Clinical Medical Center,No.(2021)75 and(2021)276。
文摘BACKGROUND The VPS33B(OMIM:608552)gene is located on chromosome 15q26.1.We found a female infant with autosomal recessive arthrogryposis,renal dysfunction and cholestasis syndrome 1(ARCS1)caused by mutation in VPS33B.The child was diagnosed with ARCS1(OMIM:208085)after the whole exome sequencing revealed two heterozygous mutations(c.96+1G>C,c.242delT)in the VPS33B gene.CASE SUMMARY We report a Chinese female infant with neonatal cholestasis disorder,who was eventually diagnosed with ARCS1 by genetic analysis.Genetic testing revealed two new mutations(c.96+1G>C and c.242delT)in VPS33B,which is the causal gene.The patient was compound heterozygous,and her parents were both heterozygous.CONCLUSION This study extends the mutational spectrum of the VPS33B gene to provide a molecular basis for the etiological diagnosis of ARCS1 and for genetic counseling of the family.
