BACKGROUND Both phases of euthyroid sick syndrome(ESS)are associated with worse prognosis in septic shock patients.Although there are still no indications for supplementation therapy,there is no evidence that both pha...BACKGROUND Both phases of euthyroid sick syndrome(ESS)are associated with worse prognosis in septic shock patients.Although there are still no indications for supplementation therapy,there is no evidence that both phases(initial and prolonged)are adaptive or that only prolonged is maladaptive and requires supplementation.AIM To analyze clinical,hemodynamic and laboratory differences in two groups of septic shock patients with ESS.METHODS A total of 47 septic shock patients with ESS were divided according to values of their thyroid hormones into low T3 and low T3T4 groups.The analysis included demographic data,mortality scores,intensive care unit stay,mechanical ventilation length and 28-day survival and laboratory with hemodynamics.RESULTS The Simplified Acute Physiology Score II score(P=0.029),dobutamine(P=0.003)and epinephrine requirement(P=0.000)and the incidence of renal failure and multiple organ failure(MOF)(P=0.000)were significantly higher for the low T3T4.Hypoalbuminemia(P=0.047),neutrophilia(P=0.038),lymphopenia(P=0.013)and lactatemia(P=0.013)were more pronounced on T2 for the low T3T4 group compared to the low T3 group.Diastolic blood pressure at T0(P=0.017)and T1(P=0.007),as well as mean arterial pressure at T0(P=0.037)and T2(P=0.033)was higher for the low T3 group.CONCLUSION The low T3T4 population is associated with higher frequency of renal insufficiency and MOF,with worse laboratory and hemodynamic parameters.These findings suggest potentially maladaptive changes in the chronic phase of septic shock.展开更多
BACKGROUND Low anterior resection syndrome(LARS)severely impairs patient postoperative quality of life,especially major LARS.However,there are few tools that can accurately predict major LARS in clinical practice.AIM ...BACKGROUND Low anterior resection syndrome(LARS)severely impairs patient postoperative quality of life,especially major LARS.However,there are few tools that can accurately predict major LARS in clinical practice.AIM To develop a machine learning model using preoperative and intraoperative factors for predicting major LARS following laparoscopic surgery of rectal cancer in Chinese populations.METHODS Clinical data and follow-up information of patients who received laparoscopic anterior resection for rectal cancer from two medical centers(one discovery cohort and one external validation cohort)were included in this retrospective study.For the discovery cohort,the machine learning prediction algorithms were developed and internally validated.In the external validation cohort,we evaluated the trained model using various performance metrics.Further,the clinical utility of the model was tested by decision curve analysis.RESULTS Overall,1651 patients were included in the present study.Anastomotic height,neoadjuvant therapy,diverting stoma,body mass index,clinical stage,specimen length,tumor size,and age were the risk factors associated with major LARS.They were used to construct the machine learning model to predict major LARS.The trained random forest(RF)model performed with an area under the curve of 0.852 and a sensitivity of 0.795(95%CI:0.681-0.877),a specificity of 0.758(95%CI:0.671-0.828),and Brier score of 0.166 in the external validation set.Compared to the previous preoperative LARS score model,the current model exhibited superior predictive performance in predicting major LARS in our cohort(accuracy of 0.772 for the RF model vs 0.355 for the preoperative LARS score model).CONCLUSION We developed and validated a robust tool for predicting major LARS.This model could potentially be used in the clinic to identify patients with a high risk of developing major LARS and then improve the quality of life.展开更多
BACKGROUND Low anterior resection syndrome(LARS)is a common complication of anuspreserving surgery in patients with colorectal cancer,which significantly affects patients'quality of life.AIM To determine the relat...BACKGROUND Low anterior resection syndrome(LARS)is a common complication of anuspreserving surgery in patients with colorectal cancer,which significantly affects patients'quality of life.AIM To determine the relationship between the incidence of LARS and patient quality of life after colorectal cancer surgery and to establish a LARS prediction model to allow perioperative precision nursing.METHODS We reviewed the data from patients who underwent elective radical resection for colorectal cancer at our institution from April 2013 to June 2020 and completed the LARS score questionnaire and the European Organization for Research and Treatment of Cancer Core Quality of Life and Colorectal Cancer Module questionnaires.According to the LARS score results,the patients were divided into no LARS,mild LARS,and severe LARS groups.The incidence of LARS and the effects of this condition on patient quality of life were determined.Univariate and multivariate analyses were performed to identify independent risk factors for the occurrence of LARS.Based on these factors,we established a risk prediction model for LARS and evaluated its performance.RESULTS Among the 223 patients included,51 did not develop LARS and 171 had mild or severe LARS.The following quality of life indicators showed significant differences between patients without LARS and those with mild or severe LARS:Physical,role,emotional,and cognitive function,total health status,fatigue,pain,shortness of breath,insomnia,constipation,and diarrhea.Tumor size,partial/total mesorectal excision,colostomy,preoperative radiotherapy,and neoadjuvant chemotherapy were identified to be independent risk factors for LARS.A LARS prediction model was successfully established,which demonstrated an accuracy of 0.808 for predicting the occurrence of LARS.CONCLUSION The quality of life of patients with LARS after colorectal cancer surgery is significantly reduced.展开更多
BACKGROUND Low anterior resection syndrome(LARS)is one of the common postoperative complications in patients with rectal cancer,which seriously affects their postoperative recovery and quality of life(QoL).Electroacup...BACKGROUND Low anterior resection syndrome(LARS)is one of the common postoperative complications in patients with rectal cancer,which seriously affects their postoperative recovery and quality of life(QoL).Electroacupuncture therapy is one of the characteristic therapies of traditional Chinese medicine.There are few reports on the prevention and treatment of LARS by electroacupuncture therapy.AIM To explore the clinical effectiveness of electroacupuncture in managing rectal cancer patients with postoperative LARS.METHODS A total of 50 patients with LARS after rectal cancer surgery were retrospectively selected as the research subjects.According to the treatment methods,they were divided into an observation group(n=25)and a control group(n=25).During the four-week treatment period,the control group received standard defecation function training,while the observation group received electroacupuncture care and traditional defecation function training.The anal pressure index(which includes anal resting pressure,anal systolic pressure,and maximum tolerable volume),European Organization of Research and Treatment of Cancer(EORTC)QoL C30(QLQ-C30)score,LARS Scale(LARSS)score,Wexner anal incontinence scale score,Xu Zhongfa five-item 10-point scale score,and the occurrence of adverse reactions were compared between the two groups before and after treatment.RESULTS The experimental group showed considerably enhanced LARSS scores compared to those in the control group after four weeks of treatment.In the first week,second week,and fourth week,the LARSS score and Wexner anal incontinence scale score decreased,and the Xu Zhong method five-item 10-point scale score increased,with significant differences(P<0.05).The experimental group showed substantial improvements in anal resting pressure,anal systolic pressure,and maximum tolerance volume after undergoing 4 wk of therapy in the untreated group(P<0.05).The experimental group's QLQ-C30 score on the EORTC QoL questionnaire was higher than that of the control group during the 1st,2nd,and 4th wk(P<0.05).No significant variation between the groups in the frequency of adverse reactions(P>0.05)was observed.CONCLUSION Electroacupuncture positively impacted LARS following rectal cancer surgery,effectively improving clinical symptoms and anal pressure indicators and patients’standard of life.展开更多
Irritable bowel syndrome(IBS)is a chronic,recurring,and remitting functional disorder of the gastrointestinal tract characterized by abdominal pain,distention,and changes in bowel habits.Although there are several dru...Irritable bowel syndrome(IBS)is a chronic,recurring,and remitting functional disorder of the gastrointestinal tract characterized by abdominal pain,distention,and changes in bowel habits.Although there are several drugs for IBS,effective and approved treatments for one or more of the symptoms for various IBS subtypes are needed.Improved understanding of pathophysiological mechanisms such as the role of impaired bile acid metabolism,neurohormonal regulation,immune dysfunction,the epithelial barrier and the secretory properties of the gut has led to advancements in the treatment of IBS.With regards to therapies for restoring intestinal permeability,multiple studies with prebiotics and probiotics are ongoing,even if to date their efficacy has been limited.In parallel,much progress has been made in targeting low-grade inflammation,especially through the introduction of drugs such as mesalazine and rifaximin,even if a better knowledge of the mechanisms underlying the low-grade inflammation in IBS may allow the design of clinical trials that test the efficacy and safety of such drugs.This literature review aims to summarize the findings related to new and investigational therapeutic agents for IBS,most recently developed in preclinical as well as Phase 1 and Phase 2clinical studies.展开更多
AIM: To investigate the effects of a low fermentable, oligosaccharides, disaccharides, monosaccharides and polyols diet (LFD) and the probiotic Lactobacillus rhamnosus GG (LGG) in irritable bowel syndrome (IBS).
AIM: To investigate the phenotype and genotype of a family with X-linked recessive Lowe syndrome. METHODS: All the members in the Chinese pedigree underwent comprehensive ophthalmologic and systemic examinations. Geno...AIM: To investigate the phenotype and genotype of a family with X-linked recessive Lowe syndrome. METHODS: All the members in the Chinese pedigree underwent comprehensive ophthalmologic and systemic examinations. Genomic DNA was isolated from peripheral blood of the pedigree members and 100 unrelated healthy Chinese subjects. Direct sequencing was performed to screen the exons and intron boundaries of OCRL.RESULTS: The ophthalmological and systemic exami nations suggested that the affected individual had Lowe syndrome. The phenotype in the pedigree is severe and consistent among all the affected individuals except for an individual who additionally suffered from congenital heart disease and laryngeal cartilage dysplasia. Directio nal San ger sequenci ng identified a complex mutation c.(2368_2368delG;c.2370A>C) in the Rho-GTPase activating protein domain. This complex mutation causes termination of protein synthesis at amino acid 824 and result in a new peptide with 823 amino acids (p.Ala790ProfsX34). This mutation was not detected in 100 unrelated healthy Chinese subjects.CONCLUSION: Our findings expand the phenotypic and genotypic spectrum of Lowe syndrome.展开更多
In the present study we report on changes in irritable bowel syndrome-severity scoring system (IBS-SSS) and irritable bowel syndrome-quality of life (IBS-QoL) in 19 IBS patients, aged 18 to 74 years (F/M: 14/5), durin...In the present study we report on changes in irritable bowel syndrome-severity scoring system (IBS-SSS) and irritable bowel syndrome-quality of life (IBS-QoL) in 19 IBS patients, aged 18 to 74 years (F/M: 14/5), during 12 wk registering their symptoms on the web-application (www.ibs.constant-care.dk). During a control period of the first 6-wk patients were asked to register their IBS-SSS and IBS-QoL on the web-application weekly without receiving any intervention. Thereafter, low fermentable oligo-, di-, mono-saccharides and polyols (FODMAP) diet (LFD) was introduced for the next 6 wk while continuing the registration. Though a small sample size a significant improvement in disease activity (IBS-SSS) was observed during both the control period, median: 278 (range: 122-377), P = 0.02, and subsequently during the LFD period, median: 151 (range: 29-334), P < 0.01. The IBS-QoL solely changed significantly during the LFD period, median: 67 (37-120), P < 0.01. The significant reduction in disease activity during the control period shows a positive effect of the web-application on IBS symptoms when presented as a “traffic light”. However adding the diet reduced IBS-SSS to < 150, inactive to mild symptoms. In the future results from larger scale trials are awaited.展开更多
AIM: To investigate the predictive value of low freeT3 for long-term mortality in chronic hemodialysis (HD) patients and explore a possible causative role of chronic infammation.METHODS: One hundred fourteen HD pa...AIM: To investigate the predictive value of low freeT3 for long-term mortality in chronic hemodialysis (HD) patients and explore a possible causative role of chronic infammation.METHODS: One hundred fourteen HD patients (84 males) consecutively entered the study and were assessed for thyroid function and two established markers of inflammation, high sensitivity C-reactive protein (hsCRP) and interleukin-6 (IL-6). Monthly blood samples were obtained from all patients for three consecutive months during the observation period for evaluation of thyroid function and measurement of infammatory markers. The patients were then divided in two groups based on the cut-off value of 1.8 pg/mL for mean plasma freeT3, and were prospectively studied for a mean of 50.3 ± 30.8 mo regarding cumulative survival. The prognostic power of low serum fT3 levels for mortality was assessed using the Kaplan-Meier method and univariate and multivariate regression analysis.RESULTS: Kaplan-Meier survival curve showed a negative predictive power for low freeT3. In Cox regression analysis low freeT3 remained a significant predictor of mortality after adjustment for age, diabetes mellitus, hypertension, hsCRP, serum creatinine and albumin. Regarding the possible association with inflammation, freeT3 was correlated with hsCRP, but not IL-6, and only at the frst month of the study.CONCLUSION: In chronic hemodialysis patients, low plasma freeT3 is a significant predictor of all-cause mortality. Further studies are required to identify the underlying mechanisms of this association.展开更多
BACKGROUND Low phospholipid-associated cholelithiasis(LPAC)syndrome is a very particular form of biliary lithiasis with no excess of cholesterol secretion into bile,but a decrease in phosphatidylcholine secretion,whic...BACKGROUND Low phospholipid-associated cholelithiasis(LPAC)syndrome is a very particular form of biliary lithiasis with no excess of cholesterol secretion into bile,but a decrease in phosphatidylcholine secretion,which is responsible for stones forming not only in the gallbladder,but also in the liver.LPAC syndrome may be underreported due to a lack of testing resulting from insufficient awareness among clinicians.AIM To describe the clinical and radiological characteristics of patients with LPAC syndrome to better identify and diagnose the disease.METHODS We prospectively evaluated all patients aged over 18 years old who were consulted or hospitalized in two hospitals in Paris,France(Bichat University Hospital and Croix-Saint-Simon Hospital)between January 1,2017 and August 31,2018.All patients whose profiles led to a clinical suspicion of LPAC syndrome underwent a liver ultrasound examination performed by an experienced radiologist to confirm the diagnosis of LPAC syndrome.Twenty-four patients were selected.Data about the patients’general characteristics,their medical history,their symptoms,and their blood tests results were collected during both their initial hospitalization and follow-up.Cytolysis and cholestasis were expressed compared to the normal values(N)of serum aspartate and alanine transaminase activities,and to the normal value of alkaline phosphatase level,respectively.The subjects were systematically reevaluated and asked about their symptoms 6 mo after inclusion in the study through an in-person medical appointment or phone call.Genetic testing was not performed systematically,but according to the decision of each physician.RESULTS Most patients were young(median age of 37 years),male(58%),and not overweight(median body mass index was 24).Many had a personal history of acute pancreatitis(54%)or cholecystectomy(42%),and a family history of gallstones in first-degree relatives(30%).LPAC syndrome was identified primarily in patients with recurring biliary pain(88%)or after a new episode of acute pancreatitis(38%).When present,cytolysis and cholestasis were not severe(2.8N and 1.7N,respectively)and disappeared quickly.Interestingly,four patients from the same family were diagnosed with LPAC syndrome.At ultrasound examination,the most frequent findings in intrahepatic bile ducts were comet-tail artifacts(96%),microlithiasis(83%),and acoustic shadows(71%).Computed tomography scans and magnetic resonance imaging were performed on 15 and three patients,respectively,but microlithiasis was not detected.Complications of LPAC syndrome required hospitalizing 18 patients(75%)in a conventional care unit for a mean duration of 6.8 d.None of them died.Treatment with ursodeoxycholic acid(UDCA)was effective and well-tolerated in almost all patients(94%)with a rapid onset of action(3.4 wk).Twelve patients’(67%)adherence to UDCA treatment was considered“good.”Five patients(36%)underwent cholecystectomy(three of them were treated both by UDCA and cholecystectomy).Despite UDCA efficacy,biliary pain recurred in five patients(28%),three of whom adhered well to treatment guidelines.CONCLUSION LPAC syndrome is easy to diagnose and treat;therefore,it should no longer be overlooked.To increase its detection rate,all patients who experience recurrent biliary symptoms following an episode of acute pancreatitis should undergo an ultrasound examination performed by a radiologist with knowledge of the disease.展开更多
BACKGROUND Indices such as the neutrophil-to-lymphocyte ratio(NLR),platelet-to-lymphocyte ratio(PLR),mean platelet volume(MPV),platelet distribution width(PDW),and red cell distribution width(RDW)are considered new ma...BACKGROUND Indices such as the neutrophil-to-lymphocyte ratio(NLR),platelet-to-lymphocyte ratio(PLR),mean platelet volume(MPV),platelet distribution width(PDW),and red cell distribution width(RDW)are considered new markers of the systemic inflammatory response(SIR),and have been widely implemented for the diagnosis of patients with inflammatory diseases.These new indicators have also been widely investigated in preeclampsia(PE)but less analyzed in hemolysis,elevated liver enzymes,and low platelet(HELLP)syndrome.AIM To compare SIR markers among HELLP patients,PE only patients,and healthy gravidae.METHODS This retrospective case-control study enrolled 630 cases,including 210 patients with HELLP syndrome(HELLP group),210 patients with only PE(PE group)and 210 healthy gravidae(control group).The three groups were matched by age,parity,status of assisted reproduction,and multiple pregnancies.Birthweight,gestational age at complete blood count collection,gestational age at delivery,mode of delivery,etc.were recorded.The main indices as NLR,PLR,MPV,PDW,and RDW among the groups were compared,as well as some secondary outcomes including neutrophil,platelets,and hemoglobin.RESULTS The NLR(6.4 vs 4.3 vs 3.5),MPV(11.9 vs 11.2 vs 10.7),PDW(16.4 vs 13.3 vs 14.2),leukocyte(12.4×10^(9)/L vs 9.7×10^(9)/L vs 8.7×10^(9)/L)and neutrophil count(9.9×10^(9)/L vs 7.3×10^(9)/L vs 6.1×10^(9)/L)were highest in the HELLP group,lower in the PE group,and lowest in the control group.Both the overall comparisons between the three groups(all bP<0.01)and pairwise comparisons between every two groups elicited statistically significant differences(all dP<0.01,except control vs PE:cP<0.05 in PDW).The average lymphocyte counts were 1.4(1.1,2.0)×10^(9)/L in the HELLP group,1.6(1.3,2.0)×10^(9)/L in the PE group and 1.7(1.4,2.0)×10^(9)/L in the control group.The overall comparison of lymphocyte count within the three groups had statistically significant differences(P=0.000).The pairwise comparisons between every two groups demonstrated that the HELLP group had a lower lymphocyte count than both the PE(P=0.019)and control groups(P=0.000),but the difference between the PE and control groups was not statistically significant(P=0.432).The overall comparisons on platelet counts and the PLR among these three groups also showed statistically significant differences(both P=0.000),from low to high being those in the HELLP group(43.4×10^(9)/L,64.0),control group(180.5×10^(9)/L,103.6)and PE group(181.5×10^(9)/L,112.8).Pairwise comparisons of neither index displayed statistically significant differences between the PE and control groups(both P>0.05),while the differences in the two indices between the HELLP group and the two other groups were still statistically significant(all P=0.000).RDW values were highest in the HELLP group(14.5%[13.6,15.3]),lower in the control group(14.1%[13.5,14.8])and lowest in the PE group(13.9%[13.4,14.9]).The difference between the PE and control group did not show statistical significance(P=1.000),while RDW values in the HELLP group were higher than those in the other two groups(cP<0.05 vs control,dP<0.01 vs PE).CONCLUSION SIR markers such as NLR,RDW,MPV,and PDW were increased and PLR was decreased in HELLP.These SIR markers may become new indicators in the evaluation of HELLP syndrome.展开更多
In spite of normal mean aterlal pressure,decrease of pulse pressure can induce a seriesof clinical symptoms,which is termed as low pulse pressure syndrorne.Acupuncture at bilateralNeiguan(PC 6)is Capable of raising ...In spite of normal mean aterlal pressure,decrease of pulse pressure can induce a seriesof clinical symptoms,which is termed as low pulse pressure syndrorne.Acupuncture at bilateralNeiguan(PC 6)is Capable of raising systolic pressure, lowering diastolic pressure, increasing pulsepressure difference and eliminating corresponding symptoms.The effective rate was more than 92%in this observation.展开更多
Objective: To determine the frequency and impact of metabolic syndrome (MS) in patients with low back pain in rheumatology unit in Benin. Patients and Methods: Analytic cross-sectional study conducted between June and...Objective: To determine the frequency and impact of metabolic syndrome (MS) in patients with low back pain in rheumatology unit in Benin. Patients and Methods: Analytic cross-sectional study conducted between June and December 2016 in the rheumatology hospital unit of National Hospital University Hubert Koutoukou Maga of Cotonou. 82 patients with mechanical low back pain were selected. The prevalence of MS was defined using the criteria of the International Diabetes Federation 2005. The data were analyzed using epi data and SPSS17.0 software. Results: The mean age was 50.4 ± 14.9 [12 - 90] years. The sex ratio was 0.82. 29.3% patients have met diagnostic criteria for MS. The mean duration of back pain was 40 ± 17.2 [3 - 120] days. Diseases associated with low back pain were as follow: lumbar intervertebral disk degeneration (34.1%), disk herniation (13.4%), facet joint arthrosis (18.3%), degenerative spondylolisthesis (11%), degenerative lumbar spinal stenosis (6.1%) and related forms (17.1%). MS observed in 24 patients was characterized by the frequent association of abdominal obesity (24 cases), arterial hypertension (22 cases), HDL hypocholesterolemia (8 cases), hyperglycemia (12 cases) or hypertriglyceridemia (7 cases). The presence of the MS was associated with a decreased response to the medical treatment (p 0.01). Conclusion: The MS is frequent in patients with low back pain in rheumatology unit at Cotonou and influence the treatment response. The management of these patients must be integrated into a multidisciplinary approach including the rheumatologist and nutritionist.展开更多
We present a case of symptomatic perihepatic adhesions,which developed after a pregnancy complicated by hemolysis,elevated liver enzymes and low platelet(HELLP)syndrome,in which a subcapsular liver hematoma occurred.O...We present a case of symptomatic perihepatic adhesions,which developed after a pregnancy complicated by hemolysis,elevated liver enzymes and low platelet(HELLP)syndrome,in which a subcapsular liver hematoma occurred.Our patient presented with complaints of persistent,severe right-sided upper abdominal pain.The complaints developed gradually,one year after a pregnancy that had been complicated by HELLP syndrome with a subcapsular liver hematoma.The hematoma had resolved spontaneously.An upperabdominal magnetic resonance imaging revealed a density between liver and diaphragm at the site of the former subcapsular hematoma,suspect of perihepatic adhesions.The presence of perihepatic adhesions was confirmed during a laparoscopy and treated by adhesiolysis in the same session.The adhesions may have developed in response to the degradation process of the subcapsular liver hematoma during conservative treatment.This case of perihepatic adhesions may therefor be the first presentation of a long term sequel of subcapsular liver hematoma in HELLP syndrome.展开更多
AIM: To evaluate the ophthalmic and anesthesiologic management of cataract surgery in children with Lowe syndrome receiving lens removal, the development and management of secondary glaucoma.METHODS: This retrospectiv...AIM: To evaluate the ophthalmic and anesthesiologic management of cataract surgery in children with Lowe syndrome receiving lens removal, the development and management of secondary glaucoma.METHODS: This retrospective case series included 12 eyes of 6 children with genetically verified Lowe syndrome receiving cataract removal. Information regarding the type and duration of surgery and total anesthesia time were recorded. Additionally, intra-and postoperative complications were noted as well as clinical examinations such as visual acuity and funduscopy. RESULTS: All children received simultaneous bilateral cataract surgery at the mean age of 8.98±3.58 wk. Lensectomy combined with posterior capsulotomy and anterior vitrectomy was performed in all children. The mean time for cataract surgery per eye was 35.83±8.86 min, whereas the total time of surgery was 153.33±22.11 min. The mean extubation time and duration at recovery room was 42.33±22.60 min and 130.00±64.37 min, respectively. During surgery, a decrease of oxygen saturation below 93% was found in only one child. During the postoperative followup, nystagmus(6 children) and strabismus(5 children) was commonly found in contrast to no case of visual axis opacification. Secondary glaucoma developed in five eyes of three children, which was treated with topical eye drops in only one child. A trabeculectomy was performed in both eyes of one child, whereas removal of syechia and an iridectomy in one eye of one child. CONCLUSION: Bilateral simultaneous cataract surgery under general anesthesia is a safe surgical procedure in Lowe syndrome children. The glaucoma screening with intraocular pressure measurements is crucial in the postoperative management of Lowe syndrome patients to avoid additional visual impairment.展开更多
Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering fr...Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.展开更多
Background:Charles Bonnet syndrome(CBS)is characterized by vivid lifelike visual hallucinations and is typically seen in individuals with visual impairment.In this systematic review,we summarize current knowledge on t...Background:Charles Bonnet syndrome(CBS)is characterized by vivid lifelike visual hallucinations and is typically seen in individuals with visual impairment.In this systematic review,we summarize current knowledge on the prevalence of CBS in patients with low vision.Methods:We searched 11 literature databases on 21 April 2021 for studies on the prevalence of CBS in low vision populations.Our protocol was registered in the PROSPERO database(reg.No.CRD42021255021).Eligible studies were defined as those on a population of low vision patients wherein the prevalence of CBS was evaluated(without any further restriction on the method for diagnosis of CBS).No restriction was enforced on study design,but we expected observational cross-sectional studies due to the nature of our research question.Two authors independently extracted data and evaluated risk of bias of individual studies using the Agency for Healthcare Research and Quality checklist for cross-sectional studies.Studies underwent qualitative review in the text and quantitatively in a prevalence meta-analysis using the random-effects model.Sensitivity analysis was made to evaluate the robustness of the summary estimate.Results:We identified 11 studies,which summarized data on 4,521 individuals with visual impairment.Diagnosis of CBS was based on screening questions or interviews,which in positive cases often was explored in more detail with follow-up questions or interviews.Most studies either had normal mental state as a participant eligibility criterion or as a part of the diagnosis of CBS.The pooled prevalence of CBS in low vision patients aged≥40 years was 19.7%(95%CI:13.8%to 26.4%).The sensitivity analysis showed certain robustness in the summary prevalence estimate wherein prevalence estimates ranged from 16.9%to 22.5%.Conclusions:CBS is a prevalent condition in patients with low vision at a rate of approximately one in five patients.Considering that an estimated 239 million individuals have moderate visual impairment or worse,we estimate a global number of patients with CBS at approximately 47.2 million.展开更多
Non-alcoholic fatty liver disease(NAFLD)and irritable bowel syndrome(IBS)are two very common diseases in the general population.To date,there are no studies that highlight a direct link between NAFLD and IBS,but some ...Non-alcoholic fatty liver disease(NAFLD)and irritable bowel syndrome(IBS)are two very common diseases in the general population.To date,there are no studies that highlight a direct link between NAFLD and IBS,but some recent reports have found an interesting correlation between obesity and IBS.A systematic PubMed database search was conducted highlighting that common mechanisms are involved in many of the local and systemic manifestations of NAFLD,leading to an increased cardiovascular risk,and IBS,leading to microbial dysbiosis,impaired intestinal barrier and altered intestinal motility.It is not known when considering local and systemic inflammation/immune system activation,which one has greater importance in NAFLD and IBS pathogenesis.Also,the nervous system is implicated.In fact,inflammation participates in the development of mood disorders,such as anxiety and depression,characteristics of obesity and consequently of NAFLD and,on the other hand,in intestinal hypersensitivity and dysmotility.展开更多
基金approved by the Institutional Review Board(IRB)of Cantonal Hospital Zenica,and the protocols used in the study were approved by the Ethical Committee of Cantonal Hospital Zenica(00-03-35-38-14/22).
文摘BACKGROUND Both phases of euthyroid sick syndrome(ESS)are associated with worse prognosis in septic shock patients.Although there are still no indications for supplementation therapy,there is no evidence that both phases(initial and prolonged)are adaptive or that only prolonged is maladaptive and requires supplementation.AIM To analyze clinical,hemodynamic and laboratory differences in two groups of septic shock patients with ESS.METHODS A total of 47 septic shock patients with ESS were divided according to values of their thyroid hormones into low T3 and low T3T4 groups.The analysis included demographic data,mortality scores,intensive care unit stay,mechanical ventilation length and 28-day survival and laboratory with hemodynamics.RESULTS The Simplified Acute Physiology Score II score(P=0.029),dobutamine(P=0.003)and epinephrine requirement(P=0.000)and the incidence of renal failure and multiple organ failure(MOF)(P=0.000)were significantly higher for the low T3T4.Hypoalbuminemia(P=0.047),neutrophilia(P=0.038),lymphopenia(P=0.013)and lactatemia(P=0.013)were more pronounced on T2 for the low T3T4 group compared to the low T3 group.Diastolic blood pressure at T0(P=0.017)and T1(P=0.007),as well as mean arterial pressure at T0(P=0.037)and T2(P=0.033)was higher for the low T3 group.CONCLUSION The low T3T4 population is associated with higher frequency of renal insufficiency and MOF,with worse laboratory and hemodynamic parameters.These findings suggest potentially maladaptive changes in the chronic phase of septic shock.
基金Supported by the National Natural Science Foundation of China,No.82173368 and 81903047.
文摘BACKGROUND Low anterior resection syndrome(LARS)severely impairs patient postoperative quality of life,especially major LARS.However,there are few tools that can accurately predict major LARS in clinical practice.AIM To develop a machine learning model using preoperative and intraoperative factors for predicting major LARS following laparoscopic surgery of rectal cancer in Chinese populations.METHODS Clinical data and follow-up information of patients who received laparoscopic anterior resection for rectal cancer from two medical centers(one discovery cohort and one external validation cohort)were included in this retrospective study.For the discovery cohort,the machine learning prediction algorithms were developed and internally validated.In the external validation cohort,we evaluated the trained model using various performance metrics.Further,the clinical utility of the model was tested by decision curve analysis.RESULTS Overall,1651 patients were included in the present study.Anastomotic height,neoadjuvant therapy,diverting stoma,body mass index,clinical stage,specimen length,tumor size,and age were the risk factors associated with major LARS.They were used to construct the machine learning model to predict major LARS.The trained random forest(RF)model performed with an area under the curve of 0.852 and a sensitivity of 0.795(95%CI:0.681-0.877),a specificity of 0.758(95%CI:0.671-0.828),and Brier score of 0.166 in the external validation set.Compared to the previous preoperative LARS score model,the current model exhibited superior predictive performance in predicting major LARS in our cohort(accuracy of 0.772 for the RF model vs 0.355 for the preoperative LARS score model).CONCLUSION We developed and validated a robust tool for predicting major LARS.This model could potentially be used in the clinic to identify patients with a high risk of developing major LARS and then improve the quality of life.
基金the Zhejiang Provincial Education Department Project,No.Y202249777 and No.Y201941473.
文摘BACKGROUND Low anterior resection syndrome(LARS)is a common complication of anuspreserving surgery in patients with colorectal cancer,which significantly affects patients'quality of life.AIM To determine the relationship between the incidence of LARS and patient quality of life after colorectal cancer surgery and to establish a LARS prediction model to allow perioperative precision nursing.METHODS We reviewed the data from patients who underwent elective radical resection for colorectal cancer at our institution from April 2013 to June 2020 and completed the LARS score questionnaire and the European Organization for Research and Treatment of Cancer Core Quality of Life and Colorectal Cancer Module questionnaires.According to the LARS score results,the patients were divided into no LARS,mild LARS,and severe LARS groups.The incidence of LARS and the effects of this condition on patient quality of life were determined.Univariate and multivariate analyses were performed to identify independent risk factors for the occurrence of LARS.Based on these factors,we established a risk prediction model for LARS and evaluated its performance.RESULTS Among the 223 patients included,51 did not develop LARS and 171 had mild or severe LARS.The following quality of life indicators showed significant differences between patients without LARS and those with mild or severe LARS:Physical,role,emotional,and cognitive function,total health status,fatigue,pain,shortness of breath,insomnia,constipation,and diarrhea.Tumor size,partial/total mesorectal excision,colostomy,preoperative radiotherapy,and neoadjuvant chemotherapy were identified to be independent risk factors for LARS.A LARS prediction model was successfully established,which demonstrated an accuracy of 0.808 for predicting the occurrence of LARS.CONCLUSION The quality of life of patients with LARS after colorectal cancer surgery is significantly reduced.
基金Supported by Jiangsu Province Traditional Chinese Medicine Technology Development Plan Project,No.ZD201903China Medical Education Association,No.2022KTZ005.
文摘BACKGROUND Low anterior resection syndrome(LARS)is one of the common postoperative complications in patients with rectal cancer,which seriously affects their postoperative recovery and quality of life(QoL).Electroacupuncture therapy is one of the characteristic therapies of traditional Chinese medicine.There are few reports on the prevention and treatment of LARS by electroacupuncture therapy.AIM To explore the clinical effectiveness of electroacupuncture in managing rectal cancer patients with postoperative LARS.METHODS A total of 50 patients with LARS after rectal cancer surgery were retrospectively selected as the research subjects.According to the treatment methods,they were divided into an observation group(n=25)and a control group(n=25).During the four-week treatment period,the control group received standard defecation function training,while the observation group received electroacupuncture care and traditional defecation function training.The anal pressure index(which includes anal resting pressure,anal systolic pressure,and maximum tolerable volume),European Organization of Research and Treatment of Cancer(EORTC)QoL C30(QLQ-C30)score,LARS Scale(LARSS)score,Wexner anal incontinence scale score,Xu Zhongfa five-item 10-point scale score,and the occurrence of adverse reactions were compared between the two groups before and after treatment.RESULTS The experimental group showed considerably enhanced LARSS scores compared to those in the control group after four weeks of treatment.In the first week,second week,and fourth week,the LARSS score and Wexner anal incontinence scale score decreased,and the Xu Zhong method five-item 10-point scale score increased,with significant differences(P<0.05).The experimental group showed substantial improvements in anal resting pressure,anal systolic pressure,and maximum tolerance volume after undergoing 4 wk of therapy in the untreated group(P<0.05).The experimental group's QLQ-C30 score on the EORTC QoL questionnaire was higher than that of the control group during the 1st,2nd,and 4th wk(P<0.05).No significant variation between the groups in the frequency of adverse reactions(P>0.05)was observed.CONCLUSION Electroacupuncture positively impacted LARS following rectal cancer surgery,effectively improving clinical symptoms and anal pressure indicators and patients’standard of life.
文摘Irritable bowel syndrome(IBS)is a chronic,recurring,and remitting functional disorder of the gastrointestinal tract characterized by abdominal pain,distention,and changes in bowel habits.Although there are several drugs for IBS,effective and approved treatments for one or more of the symptoms for various IBS subtypes are needed.Improved understanding of pathophysiological mechanisms such as the role of impaired bile acid metabolism,neurohormonal regulation,immune dysfunction,the epithelial barrier and the secretory properties of the gut has led to advancements in the treatment of IBS.With regards to therapies for restoring intestinal permeability,multiple studies with prebiotics and probiotics are ongoing,even if to date their efficacy has been limited.In parallel,much progress has been made in targeting low-grade inflammation,especially through the introduction of drugs such as mesalazine and rifaximin,even if a better knowledge of the mechanisms underlying the low-grade inflammation in IBS may allow the design of clinical trials that test the efficacy and safety of such drugs.This literature review aims to summarize the findings related to new and investigational therapeutic agents for IBS,most recently developed in preclinical as well as Phase 1 and Phase 2clinical studies.
文摘AIM: To investigate the effects of a low fermentable, oligosaccharides, disaccharides, monosaccharides and polyols diet (LFD) and the probiotic Lactobacillus rhamnosus GG (LGG) in irritable bowel syndrome (IBS).
基金Supported by the National Natural Science Foundation of China(No.81700812)the Ph.D.Start-up Fund of Natural Science Foundation of Guangdong Province(No.2017A030310214)the Guangdong Provincial Foundation for Medical Scientific Research(No.A2017016)
文摘AIM: To investigate the phenotype and genotype of a family with X-linked recessive Lowe syndrome. METHODS: All the members in the Chinese pedigree underwent comprehensive ophthalmologic and systemic examinations. Genomic DNA was isolated from peripheral blood of the pedigree members and 100 unrelated healthy Chinese subjects. Direct sequencing was performed to screen the exons and intron boundaries of OCRL.RESULTS: The ophthalmological and systemic exami nations suggested that the affected individual had Lowe syndrome. The phenotype in the pedigree is severe and consistent among all the affected individuals except for an individual who additionally suffered from congenital heart disease and laryngeal cartilage dysplasia. Directio nal San ger sequenci ng identified a complex mutation c.(2368_2368delG;c.2370A>C) in the Rho-GTPase activating protein domain. This complex mutation causes termination of protein synthesis at amino acid 824 and result in a new peptide with 823 amino acids (p.Ala790ProfsX34). This mutation was not detected in 100 unrelated healthy Chinese subjects.CONCLUSION: Our findings expand the phenotypic and genotypic spectrum of Lowe syndrome.
文摘In the present study we report on changes in irritable bowel syndrome-severity scoring system (IBS-SSS) and irritable bowel syndrome-quality of life (IBS-QoL) in 19 IBS patients, aged 18 to 74 years (F/M: 14/5), during 12 wk registering their symptoms on the web-application (www.ibs.constant-care.dk). During a control period of the first 6-wk patients were asked to register their IBS-SSS and IBS-QoL on the web-application weekly without receiving any intervention. Thereafter, low fermentable oligo-, di-, mono-saccharides and polyols (FODMAP) diet (LFD) was introduced for the next 6 wk while continuing the registration. Though a small sample size a significant improvement in disease activity (IBS-SSS) was observed during both the control period, median: 278 (range: 122-377), P = 0.02, and subsequently during the LFD period, median: 151 (range: 29-334), P < 0.01. The IBS-QoL solely changed significantly during the LFD period, median: 67 (37-120), P < 0.01. The significant reduction in disease activity during the control period shows a positive effect of the web-application on IBS symptoms when presented as a “traffic light”. However adding the diet reduced IBS-SSS to < 150, inactive to mild symptoms. In the future results from larger scale trials are awaited.
文摘AIM: To investigate the predictive value of low freeT3 for long-term mortality in chronic hemodialysis (HD) patients and explore a possible causative role of chronic infammation.METHODS: One hundred fourteen HD patients (84 males) consecutively entered the study and were assessed for thyroid function and two established markers of inflammation, high sensitivity C-reactive protein (hsCRP) and interleukin-6 (IL-6). Monthly blood samples were obtained from all patients for three consecutive months during the observation period for evaluation of thyroid function and measurement of infammatory markers. The patients were then divided in two groups based on the cut-off value of 1.8 pg/mL for mean plasma freeT3, and were prospectively studied for a mean of 50.3 ± 30.8 mo regarding cumulative survival. The prognostic power of low serum fT3 levels for mortality was assessed using the Kaplan-Meier method and univariate and multivariate regression analysis.RESULTS: Kaplan-Meier survival curve showed a negative predictive power for low freeT3. In Cox regression analysis low freeT3 remained a significant predictor of mortality after adjustment for age, diabetes mellitus, hypertension, hsCRP, serum creatinine and albumin. Regarding the possible association with inflammation, freeT3 was correlated with hsCRP, but not IL-6, and only at the frst month of the study.CONCLUSION: In chronic hemodialysis patients, low plasma freeT3 is a significant predictor of all-cause mortality. Further studies are required to identify the underlying mechanisms of this association.
文摘BACKGROUND Low phospholipid-associated cholelithiasis(LPAC)syndrome is a very particular form of biliary lithiasis with no excess of cholesterol secretion into bile,but a decrease in phosphatidylcholine secretion,which is responsible for stones forming not only in the gallbladder,but also in the liver.LPAC syndrome may be underreported due to a lack of testing resulting from insufficient awareness among clinicians.AIM To describe the clinical and radiological characteristics of patients with LPAC syndrome to better identify and diagnose the disease.METHODS We prospectively evaluated all patients aged over 18 years old who were consulted or hospitalized in two hospitals in Paris,France(Bichat University Hospital and Croix-Saint-Simon Hospital)between January 1,2017 and August 31,2018.All patients whose profiles led to a clinical suspicion of LPAC syndrome underwent a liver ultrasound examination performed by an experienced radiologist to confirm the diagnosis of LPAC syndrome.Twenty-four patients were selected.Data about the patients’general characteristics,their medical history,their symptoms,and their blood tests results were collected during both their initial hospitalization and follow-up.Cytolysis and cholestasis were expressed compared to the normal values(N)of serum aspartate and alanine transaminase activities,and to the normal value of alkaline phosphatase level,respectively.The subjects were systematically reevaluated and asked about their symptoms 6 mo after inclusion in the study through an in-person medical appointment or phone call.Genetic testing was not performed systematically,but according to the decision of each physician.RESULTS Most patients were young(median age of 37 years),male(58%),and not overweight(median body mass index was 24).Many had a personal history of acute pancreatitis(54%)or cholecystectomy(42%),and a family history of gallstones in first-degree relatives(30%).LPAC syndrome was identified primarily in patients with recurring biliary pain(88%)or after a new episode of acute pancreatitis(38%).When present,cytolysis and cholestasis were not severe(2.8N and 1.7N,respectively)and disappeared quickly.Interestingly,four patients from the same family were diagnosed with LPAC syndrome.At ultrasound examination,the most frequent findings in intrahepatic bile ducts were comet-tail artifacts(96%),microlithiasis(83%),and acoustic shadows(71%).Computed tomography scans and magnetic resonance imaging were performed on 15 and three patients,respectively,but microlithiasis was not detected.Complications of LPAC syndrome required hospitalizing 18 patients(75%)in a conventional care unit for a mean duration of 6.8 d.None of them died.Treatment with ursodeoxycholic acid(UDCA)was effective and well-tolerated in almost all patients(94%)with a rapid onset of action(3.4 wk).Twelve patients’(67%)adherence to UDCA treatment was considered“good.”Five patients(36%)underwent cholecystectomy(three of them were treated both by UDCA and cholecystectomy).Despite UDCA efficacy,biliary pain recurred in five patients(28%),three of whom adhered well to treatment guidelines.CONCLUSION LPAC syndrome is easy to diagnose and treat;therefore,it should no longer be overlooked.To increase its detection rate,all patients who experience recurrent biliary symptoms following an episode of acute pancreatitis should undergo an ultrasound examination performed by a radiologist with knowledge of the disease.
基金Supported by the People’s Wellbeing Project of Suzhou City,No.SS201710the Clinical Expert Team Introduction Project of Suzhou City,No.SZYJTD201709and the Research Project on Maternal and Child Health of Jiangsu Province,No.F202045.
文摘BACKGROUND Indices such as the neutrophil-to-lymphocyte ratio(NLR),platelet-to-lymphocyte ratio(PLR),mean platelet volume(MPV),platelet distribution width(PDW),and red cell distribution width(RDW)are considered new markers of the systemic inflammatory response(SIR),and have been widely implemented for the diagnosis of patients with inflammatory diseases.These new indicators have also been widely investigated in preeclampsia(PE)but less analyzed in hemolysis,elevated liver enzymes,and low platelet(HELLP)syndrome.AIM To compare SIR markers among HELLP patients,PE only patients,and healthy gravidae.METHODS This retrospective case-control study enrolled 630 cases,including 210 patients with HELLP syndrome(HELLP group),210 patients with only PE(PE group)and 210 healthy gravidae(control group).The three groups were matched by age,parity,status of assisted reproduction,and multiple pregnancies.Birthweight,gestational age at complete blood count collection,gestational age at delivery,mode of delivery,etc.were recorded.The main indices as NLR,PLR,MPV,PDW,and RDW among the groups were compared,as well as some secondary outcomes including neutrophil,platelets,and hemoglobin.RESULTS The NLR(6.4 vs 4.3 vs 3.5),MPV(11.9 vs 11.2 vs 10.7),PDW(16.4 vs 13.3 vs 14.2),leukocyte(12.4×10^(9)/L vs 9.7×10^(9)/L vs 8.7×10^(9)/L)and neutrophil count(9.9×10^(9)/L vs 7.3×10^(9)/L vs 6.1×10^(9)/L)were highest in the HELLP group,lower in the PE group,and lowest in the control group.Both the overall comparisons between the three groups(all bP<0.01)and pairwise comparisons between every two groups elicited statistically significant differences(all dP<0.01,except control vs PE:cP<0.05 in PDW).The average lymphocyte counts were 1.4(1.1,2.0)×10^(9)/L in the HELLP group,1.6(1.3,2.0)×10^(9)/L in the PE group and 1.7(1.4,2.0)×10^(9)/L in the control group.The overall comparison of lymphocyte count within the three groups had statistically significant differences(P=0.000).The pairwise comparisons between every two groups demonstrated that the HELLP group had a lower lymphocyte count than both the PE(P=0.019)and control groups(P=0.000),but the difference between the PE and control groups was not statistically significant(P=0.432).The overall comparisons on platelet counts and the PLR among these three groups also showed statistically significant differences(both P=0.000),from low to high being those in the HELLP group(43.4×10^(9)/L,64.0),control group(180.5×10^(9)/L,103.6)and PE group(181.5×10^(9)/L,112.8).Pairwise comparisons of neither index displayed statistically significant differences between the PE and control groups(both P>0.05),while the differences in the two indices between the HELLP group and the two other groups were still statistically significant(all P=0.000).RDW values were highest in the HELLP group(14.5%[13.6,15.3]),lower in the control group(14.1%[13.5,14.8])and lowest in the PE group(13.9%[13.4,14.9]).The difference between the PE and control group did not show statistical significance(P=1.000),while RDW values in the HELLP group were higher than those in the other two groups(cP<0.05 vs control,dP<0.01 vs PE).CONCLUSION SIR markers such as NLR,RDW,MPV,and PDW were increased and PLR was decreased in HELLP.These SIR markers may become new indicators in the evaluation of HELLP syndrome.
文摘In spite of normal mean aterlal pressure,decrease of pulse pressure can induce a seriesof clinical symptoms,which is termed as low pulse pressure syndrorne.Acupuncture at bilateralNeiguan(PC 6)is Capable of raising systolic pressure, lowering diastolic pressure, increasing pulsepressure difference and eliminating corresponding symptoms.The effective rate was more than 92%in this observation.
文摘Objective: To determine the frequency and impact of metabolic syndrome (MS) in patients with low back pain in rheumatology unit in Benin. Patients and Methods: Analytic cross-sectional study conducted between June and December 2016 in the rheumatology hospital unit of National Hospital University Hubert Koutoukou Maga of Cotonou. 82 patients with mechanical low back pain were selected. The prevalence of MS was defined using the criteria of the International Diabetes Federation 2005. The data were analyzed using epi data and SPSS17.0 software. Results: The mean age was 50.4 ± 14.9 [12 - 90] years. The sex ratio was 0.82. 29.3% patients have met diagnostic criteria for MS. The mean duration of back pain was 40 ± 17.2 [3 - 120] days. Diseases associated with low back pain were as follow: lumbar intervertebral disk degeneration (34.1%), disk herniation (13.4%), facet joint arthrosis (18.3%), degenerative spondylolisthesis (11%), degenerative lumbar spinal stenosis (6.1%) and related forms (17.1%). MS observed in 24 patients was characterized by the frequent association of abdominal obesity (24 cases), arterial hypertension (22 cases), HDL hypocholesterolemia (8 cases), hyperglycemia (12 cases) or hypertriglyceridemia (7 cases). The presence of the MS was associated with a decreased response to the medical treatment (p 0.01). Conclusion: The MS is frequent in patients with low back pain in rheumatology unit at Cotonou and influence the treatment response. The management of these patients must be integrated into a multidisciplinary approach including the rheumatologist and nutritionist.
文摘We present a case of symptomatic perihepatic adhesions,which developed after a pregnancy complicated by hemolysis,elevated liver enzymes and low platelet(HELLP)syndrome,in which a subcapsular liver hematoma occurred.Our patient presented with complaints of persistent,severe right-sided upper abdominal pain.The complaints developed gradually,one year after a pregnancy that had been complicated by HELLP syndrome with a subcapsular liver hematoma.The hematoma had resolved spontaneously.An upperabdominal magnetic resonance imaging revealed a density between liver and diaphragm at the site of the former subcapsular hematoma,suspect of perihepatic adhesions.The presence of perihepatic adhesions was confirmed during a laparoscopy and treated by adhesiolysis in the same session.The adhesions may have developed in response to the degradation process of the subcapsular liver hematoma during conservative treatment.This case of perihepatic adhesions may therefor be the first presentation of a long term sequel of subcapsular liver hematoma in HELLP syndrome.
文摘AIM: To evaluate the ophthalmic and anesthesiologic management of cataract surgery in children with Lowe syndrome receiving lens removal, the development and management of secondary glaucoma.METHODS: This retrospective case series included 12 eyes of 6 children with genetically verified Lowe syndrome receiving cataract removal. Information regarding the type and duration of surgery and total anesthesia time were recorded. Additionally, intra-and postoperative complications were noted as well as clinical examinations such as visual acuity and funduscopy. RESULTS: All children received simultaneous bilateral cataract surgery at the mean age of 8.98±3.58 wk. Lensectomy combined with posterior capsulotomy and anterior vitrectomy was performed in all children. The mean time for cataract surgery per eye was 35.83±8.86 min, whereas the total time of surgery was 153.33±22.11 min. The mean extubation time and duration at recovery room was 42.33±22.60 min and 130.00±64.37 min, respectively. During surgery, a decrease of oxygen saturation below 93% was found in only one child. During the postoperative followup, nystagmus(6 children) and strabismus(5 children) was commonly found in contrast to no case of visual axis opacification. Secondary glaucoma developed in five eyes of three children, which was treated with topical eye drops in only one child. A trabeculectomy was performed in both eyes of one child, whereas removal of syechia and an iridectomy in one eye of one child. CONCLUSION: Bilateral simultaneous cataract surgery under general anesthesia is a safe surgical procedure in Lowe syndrome children. The glaucoma screening with intraocular pressure measurements is crucial in the postoperative management of Lowe syndrome patients to avoid additional visual impairment.
文摘Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.
文摘Background:Charles Bonnet syndrome(CBS)is characterized by vivid lifelike visual hallucinations and is typically seen in individuals with visual impairment.In this systematic review,we summarize current knowledge on the prevalence of CBS in patients with low vision.Methods:We searched 11 literature databases on 21 April 2021 for studies on the prevalence of CBS in low vision populations.Our protocol was registered in the PROSPERO database(reg.No.CRD42021255021).Eligible studies were defined as those on a population of low vision patients wherein the prevalence of CBS was evaluated(without any further restriction on the method for diagnosis of CBS).No restriction was enforced on study design,but we expected observational cross-sectional studies due to the nature of our research question.Two authors independently extracted data and evaluated risk of bias of individual studies using the Agency for Healthcare Research and Quality checklist for cross-sectional studies.Studies underwent qualitative review in the text and quantitatively in a prevalence meta-analysis using the random-effects model.Sensitivity analysis was made to evaluate the robustness of the summary estimate.Results:We identified 11 studies,which summarized data on 4,521 individuals with visual impairment.Diagnosis of CBS was based on screening questions or interviews,which in positive cases often was explored in more detail with follow-up questions or interviews.Most studies either had normal mental state as a participant eligibility criterion or as a part of the diagnosis of CBS.The pooled prevalence of CBS in low vision patients aged≥40 years was 19.7%(95%CI:13.8%to 26.4%).The sensitivity analysis showed certain robustness in the summary prevalence estimate wherein prevalence estimates ranged from 16.9%to 22.5%.Conclusions:CBS is a prevalent condition in patients with low vision at a rate of approximately one in five patients.Considering that an estimated 239 million individuals have moderate visual impairment or worse,we estimate a global number of patients with CBS at approximately 47.2 million.
文摘Non-alcoholic fatty liver disease(NAFLD)and irritable bowel syndrome(IBS)are two very common diseases in the general population.To date,there are no studies that highlight a direct link between NAFLD and IBS,but some recent reports have found an interesting correlation between obesity and IBS.A systematic PubMed database search was conducted highlighting that common mechanisms are involved in many of the local and systemic manifestations of NAFLD,leading to an increased cardiovascular risk,and IBS,leading to microbial dysbiosis,impaired intestinal barrier and altered intestinal motility.It is not known when considering local and systemic inflammation/immune system activation,which one has greater importance in NAFLD and IBS pathogenesis.Also,the nervous system is implicated.In fact,inflammation participates in the development of mood disorders,such as anxiety and depression,characteristics of obesity and consequently of NAFLD and,on the other hand,in intestinal hypersensitivity and dysmotility.