BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with si...BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with significant long-term morbidity,and histological abnormalities such as crescentic glomerulonephritis are infrequently reported.PSAGN has also been linked to late-onset chronic kidney disease in some populations due to high levels of proteinuria.METHODS This prospective observational study was conducted at Lady Ridgeway Hospital(Colombo,Sri Lanka)over 15 months.Children with PSAGN were enrolled based on clinical and laboratory criteria.Persistent proteinuria≥2+for 2 weeks and serum creatinine>100μmol/L warranted renal biopsy,assessed via light microscopy and immunofluorescence.Normalization of complement 3(C3)within 6 to 8 weeks was required for inclusion.Data on clinical features,urine protein levels,and renal function were collected from patient records,and potential associations were analysed using Statistical Package for the Social Sciences and R language for statistical computing.Ethical approval was obtained from the Ethical Review Committee,Lady Ridgeway Hospital for Children(Ref No:LRH/ERC/2021/60).RESULTS Forty-four patients were recruited.There were 27(61.4%)male patients and 17(38.6%)female patients.Thirty-seven(84%)of them were above 5 years of age.Twenty(45%)patients had a history of skin sepsis,and eighteen(41%)had a history of throat infection.Among patients with proteinuria≥2+,53%had serum creatinine>100µmol/L,while among those with proteinuria<2+,7%had serum creatinine>100µmol/L.The association of high-degree proteinuria with elevated serum creatinine was significant(χ²=7.8,P=0.005)in PSAGN.The odds ratio of the logistic regression model was 1.049(95%confidence interval:1.003-1.098),indicating a positive direction with statistically significant association(P=0.037).There was no significant association between proteinuria and the degree of hypertension or estimated creatinine clearance.Ten children underwent renal biopsy.Crescents(less than 50%)were demonstrated in five children,while three children had typical diffuse proliferative glomer-ulonephritis.One child had severe acute tubular necrosis,and another had crescentic glomerulonephritis(crescents>50%).The immunofluorescence studies revealed deposition of immunoglobulin G and C3 in all biopsy specimens.CONCLUSION High-degree proteinuria was significantly associated with elevated serum creatinine(>100μmol/L)in children with PSAGN.The majority of children with persistent proteinuria≥2+for more than 2 weeks and the highest recorded serum creatinine>100μmol/L had atypical renal histological findings.展开更多
BACKGROUND Point-of-care ultrasound(POCUS)is the use of portable ultrasound devices by trained healthcare professionals to diagnose and monitor medical conditions directly at the patient's bedside,such as in emerg...BACKGROUND Point-of-care ultrasound(POCUS)is the use of portable ultrasound devices by trained healthcare professionals to diagnose and monitor medical conditions directly at the patient's bedside,such as in emergency settings.We described a case where POCUS use,in Pediatric Emergency Department,allowed an early diagnosis and timely management in the surgical department,with a favorable outcome for child.Therefore we write this case because it is desirable to po-pularize ultrasound as the fifth pillar of clinical examination especially in Emer-gency Department CASE SUMMARY A 13-year-old girl with acute gastrointestinal symptoms,such as vomit and abdominal and lumbar pains.Upon physical examination,the patient had lo-calized abdominal pain in the left lower quadrant.Ultrasonography performed at the bedside revealed an enlarged left ovary with an irregular structure containing a 3 cm cyst.These findings raised suspicion of ovarian torsion.The child tran-sferred to Gynecology Surgery Unit,where she was taken emergently to the ope-rating room CONCLUSION It is desirable to spread POCUS in emergency settings where it allows a sig-nificant saving of time in patient management.展开更多
BACKGROUND Disorders of gut-brain interaction(DGBI)are common,but knowledge about their physiopathology is still poor,nor valid tools have been used to evaluate them in childhood.AIM To develop a psycho-gastroenterolo...BACKGROUND Disorders of gut-brain interaction(DGBI)are common,but knowledge about their physiopathology is still poor,nor valid tools have been used to evaluate them in childhood.AIM To develop a psycho-gastroenterological questionnaire(PGQ)to assess the psycho-gastroenterological profile and social characteristics of a pediatric population with and without DGBI.METHODS One hundred and nineteen Italian children(age 11-18)were included:28 outpatient patients with DGBI(Rome IV criteria)and 91 healthy controls.They filled the PGQ,faces pain scale revised(FPS-R),Bristol stool chart,ga-strointestinal symptoms rating scale,state-trait anxiety inventory,Toronto alexithymia scale 20,perceived self-efficacy in the management of negative emotions and expression of positive emotions(APEN-G,APEP-G),irritable bowel syndrome-quality of life questionnaire,school performances,tobacco use,early life events,degree of digital-ization.RESULTS Compared to controls,patients had more medical examinations(35%of them went to the doctor more than five times),a higher school performance(23%vs 13%,P<0.05),didn’t use tobacco(never vs 16%,P<0.05),had early life events(28%vs 1%P<0.05)and a higher percentage of pain classified as 4 in the FPS-R during the examination(14%vs 7%,P<0.05).CONCLUSION Pediatric outpatients with DGBI had a higher prevalence of early life events,a lower quality of life,more medical examinations rising health care costs,lower anxiety levels.展开更多
BACKGROUND Type 1 diabetes mellitus(T1DM)is an autoimmune disease with a multifactorial pathogenesis.Viral infections have been proposed as contributing triggers,supported by the disease’s seasonal pattern,which typi...BACKGROUND Type 1 diabetes mellitus(T1DM)is an autoimmune disease with a multifactorial pathogenesis.Viral infections have been proposed as contributing triggers,supported by the disease’s seasonal pattern,which typically shows higher incidence in autumn and winter.The coronavirus disease 2019(COVID-19)pandemic and associated lockdowns created a unique context to examine the incidence and seasonality of T1DM during a period characterized by reduced circulation of common viral infections.AIM To investigate the incidence and seasonality of T1DM before and during COVID-19 pandemic in relation to global viral infection rates.METHODS This population-based retrospective study utilized a nationwide computerized database.Extracted data included the number of new T1DM cases over the 8 years preceding and during the COVID-19 pandemic,demographic characteristics of affected individuals,and nationwide respiratory virus polymerase chain reaction data from weekly nasal wash sample collections.RESULTS A total of 2176 patients were diagnosed with new-onset T1DM during the prepandemic period,compared to 348 cases during the pandemic.In the same periods,33727 respiratory virus-positive polymerase chain reaction results from nasal wash samples were recorded pre-pandemic,compared to 2603 during the pandemic.Additionally,363399 positive COVID-19 cases were reported during the pandemic period.Seasonality analysis revealed a higher rate of new-onset T1DM cases and a weaker seasonal pattern during the pandemic.Trend analysis showed a consistent increase in T1DM incidence prior to COVID-19,with a more variable trend observed during the pandemic.Correlation analysis between T1DM incidence and respiratory viruses demonstrated a weak correlation between T1DM incidence and a few respiratory viruses.CONCLUSION The observed increase in new-onset T1DM cases and the disruption of its typical seasonal pattern during the COVID-19 pandemic suggest a potential association between respiratory virus exposure and the development of T1DM.展开更多
BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD u...BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD using noninvasive methods is challenging.However,transient elastography(TE)may be useful for the non-invasive evaluation of FALD and prediction of clinical outcomes.AIM To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events.METHODS This retrospective single-center study(Hospital Universitario La Paz,Madrid),including 91 post-Fontan patients aged>18 years old.Laboratory and ultrasound findings,and liver stiffness measurements(LSM)by TE(FibroScan®)were assessed.FALD was defined using ultrasound criteria hepatomegaly,liver surface nodularity,parenchymal heterogeneity,hyperechoic lesions,spleno-RESULTS Patient characteristics were:60.4%male;Mean age,33.3±8.2 years;Mean elapsed time since surgery,24.3±7.7 years;89%with FALD;73%with advanced FALD.LSM by TE was associated with FALD[odds ratio(OR)=1.34;95%confidence interval(95%CI):1.10-1.64;P=0.003]and advanced FALD(OR=1.10;95%CI:1.01-1.19;P=0.023).Areas under the curve(AUC)were 0.905 and 0.764 for FALD and advanced FALD,respectively.FALD cut-off values comprised:Optimal,20 kPa(sensitivity:92.3%;specificity:80.0%);Rule-out,15 kPa(sensitivity:96.9%);Rule-in,25 kPa(specificity:100%).A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery(AUC:0.877;sensitivity,95.4%;specificity,80.0%;positive predictive value,96.9%;negative predictive value,72.7%).LSM by TE was associated with clinically relevant events(OR=1.07;95%CI:1.01-1.13;P=0.021)and all-cause mortality(OR=1.23;95%CI:1.02-1.47;P=0.026).CONCLUSION In adult patients post-Fontan surgery,TE is a useful noninvasive method for FALD diagnosis.The association between LSM by TE and clinically relevant events suggests a role in prognosis.展开更多
BACKGROUND A diagnosis of a chronic disease has been shown to predispose patients to the development of feeding and eating disorders(FEDs).AIM To screen children and adolescents with type 1 diabetes mellitus(T1DM)for ...BACKGROUND A diagnosis of a chronic disease has been shown to predispose patients to the development of feeding and eating disorders(FEDs).AIM To screen children and adolescents with type 1 diabetes mellitus(T1DM)for FEDs and compare them to their counterparts with short stature.METHODS A total of 110 children and adolescents(55 with T1DM and 55 with short stature)were enrolled in the study.The SCOFF questionnaire was used to screen for possible FEDs,while anthropometric and dietary data were also collected.RESULTS Approximately 60%of the children with T1DM screened positive for FEDs compared to 30.9%of the children with short stature.Having a T1DM tripled the chances of screening positive for FEDs and halved the annual growth rate of children with T1DM.No differences were noted in the dietary intake between groups.CONCLUSION The results necessitate the education of pediatric endocrinologists and diabetologists on proper screening and identification of children at risk for developing FEDs.A prompt diagnosis might help children catch up growth and attain their genetically predisposed height.展开更多
Video capsule endoscopy (CE) for evaluation the esophagus (ECE), small bowel (SBCE) and the colon (CCE) is particularly useful in pediatrics, because this imaging modality does not require ionizing radiation, deep sed...Video capsule endoscopy (CE) for evaluation the esophagus (ECE), small bowel (SBCE) and the colon (CCE) is particularly useful in pediatrics, because this imaging modality does not require ionizing radiation, deep sedation or general anesthesia. The risk of capsule retention appears to be dependent on indication rather than age and parallels the adult experience by indication, making SBCE a relatively safe procedure with a significant diagnostic yield. The newest indication, assessment of mucosal change, greatly enhances and expands its potential benefit. The diagnostic role of CE extends beyond the SB. The use of ECE also may enhance our knowledge of esophageal disease and assist patient care. Colon CCE is a novel minimally invasive and painless endoscopic technique allowing exploration of the colon without need for sedation, rectal intubation and gas insufflation. The limited data on ECE and CCE in pediatrics does not yet allow the same conclusions regarding efficacy; however, both appear to provide safe methods to assess and monitor mucosal change in their respective areas with little discomfort. Moreover, although experience has been limited, the patency capsule may help lessen the potential of capsule retention; and newly researched protocols for bowel cleaning may further enhance CE’s diagnostic yield. However, further research is needed to optimize the use of the various CE procedures in pediatric populations.展开更多
1 Introduction Kounis syndrome constitutes a coronary hypersensitivity disorder defined by the association of an anaphylactoid,anaphylactic,allergic or hypersensitivity reaction with an acute coronary syndrome,in a ph...1 Introduction Kounis syndrome constitutes a coronary hypersensitivity disorder defined by the association of an anaphylactoid,anaphylactic,allergic or hypersensitivity reaction with an acute coronary syndrome,in a physiopathological context involving various interrelated and interacting inflammatory cells,such as mast-cells,eosinophils and platelets.[1,2]Similar entities to Kounis syndrome might involve cerebral and mesenteric arteries.展开更多
Objective: Despite the presence of hand washing material and the training given to medical staff regarding hygiene measures and health care procedures in October 2015, the prevalence of nosocomial infections in the ne...Objective: Despite the presence of hand washing material and the training given to medical staff regarding hygiene measures and health care procedures in October 2015, the prevalence of nosocomial infections in the neonatal unit of the National University Teaching Hospital of Cotonou (CNHU-Cotonou) was estimated at 8% in January 2016. To determine the factors that contribute to these infections, this study assessed medical staff compliance with hand hygiene measures and procedures. Method: This research was a cross-sectional and observational study conducted from February 15 to March 31, 2016 through direct and cautious observation of 47 members of the medical and paramedical staff. The study variables were hand washing before entering the neonatal unit and before entering each treatment room, hand washing before and after seeing each patient, compliance with hand washing steps, the use of hydroalcoholic solutions and adhering to the ban on mobile phone use inside the treatment room. Results: Only 15% of the medical staff followed all of the rules and measures governing hand hygiene. The result showed that 76.6% of them did not wash their hands before entering the unit;32% washed their hands before each care session;95.7% washed their hands after each care session;and 85% did not comply with the hand washing steps. Only 21.3% of the personnel used hydroalcoholic solution, and only 85% of the personnel adhered to the ban on mobile phone use within the treatment room. Conclusion: Compliance with hand hygiene measures is insufficient. These low compliance rates facilitate the occurrence of nosocomial infections. Nosocomial infections could be prevented by identifying the reasons that medical personnel do not wash their hands and by implementing a program for education/awareness on hygiene measures based on an analysis of errors and care procedures and sustained by regular evaluations.展开更多
Transjugular intrahepatic portosystemic shunt(TIPS)placement is a standard procedure for the treatment of portal hypertension complications.When this conventional approach is not feasible,alternative procedures for sy...Transjugular intrahepatic portosystemic shunt(TIPS)placement is a standard procedure for the treatment of portal hypertension complications.When this conventional approach is not feasible,alternative procedures for systemic diversion of portal blood have been proposed.A one-step interventional approach,combining minilaparotomy-assisted transmesenteric(MAT)antegrade portal recanalization and TIPS,is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma(PC).A 16-yearold girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC.A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed.In the case of failed portal recanalization,this approach,would also be useful for varice endovascular embolization.Postoperative recovery was uneventful.Treatment consisting of propanolol,enoxaparin and a proton pump inhibitor was prescribed after the procedure.One month post-op,contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts.No residual peritoneal fluid was detected nor opacification of the large varices.Endoscopy showed good improvement of the varices.Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches.Three months post-op,TIPS maintained its hourglass shape despite a slight expansion.Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization.MAT-TIPS is feasible and safe for the treatment of PC even in children.This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients.展开更多
Introduction: In low-income countries, severe anemia represents a significant risk of mortality. It is generally the consequence of a lack of correct community-based management of mild and moderate anemia and its etio...Introduction: In low-income countries, severe anemia represents a significant risk of mortality. It is generally the consequence of a lack of correct community-based management of mild and moderate anemia and its etiologies. Our study aimed to evaluate the community practice in confronting anemia by mothers of children aged 0 - 5 years old before and during the COVID-19 period. Methods: We conducted a descriptive cross-sectional study in two phases at the pediatric ward of the regional hospital of Ngaoundere in Cameroon;before the period of the COVID-19 pandemic from December 2018 to May 2019 and during the pandemic period from December 2020 to May 2021. It was a volunteer sample. Were included in this study all mothers of children aged 0 - 5 years present in the pediatric ward at the time of the survey who agreed to freely answer our questionnaire. Results: Of the 152 mothers surveyed in the “Before COVID-19” group, 69% went to a health facility when they suspected their children were anemic, 20% gave grenadine juice (Red colored soda), 7% went to marabous, 2% bought street medicines and 2% used other home-made potions. Of the 92 mothers included in the “During COVID-19” group, the majority did not go to a health facility: 36% gave grenadine juice and other potions, 26% went to marabouts, 23% bought medicines from the street and only 13% went to a health facility;2% did nothing. The average Group rank is not statistically significantly different between the before and during the COVID-19 periods (p = 0.89). Conclusion: Community behavior and practice in confronting anemia deteriorated during the COVID-19 pandemic, with the majority of mothers resorting to non-effective out-of-hospital solutions. The consequence can be an increase in infant mortality. While waiting for the end of the pandemic, it is judicious and urgent to review the whole health system in Africa and especially in countries with limited resources. We propose developing multiple advanced strategies to promote community visits to prevent anemia and to develop anticipatory management of anemia as well as all the main child-killing pathologies.展开更多
The incident of the measles has diminished in the last decades thanks to the widespread use of the vaccine in The United States and Europe. Nevertheless, recently we are seeing new epidemics of measles due to the lack...The incident of the measles has diminished in the last decades thanks to the widespread use of the vaccine in The United States and Europe. Nevertheless, recently we are seeing new epidemics of measles due to the lack of coverage in some social areas. Measles during pregnancy is associated with high maternal morbidity and mortality, being a frequent cause of abortion in preterm deliveries. We report two cases of measles attended in the Unit of Neonatology held coinciding with the epidemic lived in this area during the last year.展开更多
The field of vascular medicine has undergone a profound transformation in the 21st century,transforming our approach to assessment and treatment.Athero-sclerosis,a complex inflammatory disease that affects medium and ...The field of vascular medicine has undergone a profound transformation in the 21st century,transforming our approach to assessment and treatment.Athero-sclerosis,a complex inflammatory disease that affects medium and large arteries,presents a major challenge for researchers and healthcare professionals.This condition,characterized by arterial plaque formation and narrowing,poses sub-stantial challenges to vascular health at individual,national,and global scales.Its repercussions are far-reaching,with clinical outcomes including ischemic heart disease,ischemic stroke,and peripheral arterial disease—conditions with esca-lating global prevalence.Early detection of vascular changes caused by athero-sclerosis is crucial in preventing these conditions,reducing morbidity,and averting mortality.This article underscored the imperative of adopting a holistic approach to grappling with the intricacies,trajectories,and ramifications of atherosclerosis.It stresses the need for a thorough evaluation of the vasculature and the implementation of a multidisciplinary treatment approach.By consi-dering the entire vascular system,healthcare providers can explore avenues for prevention,early detection,and effective management of this condition,ultima-tely leading to improved patient outcomes.We discussed current practices and proposed new directions made possible by emerging diagnostic modalities and treatment strategies.Additionally,we considered healthcare expenditure,resour-ce allocation,and the transformative potential of new innovative treatments and technologies.展开更多
BACKGROUND Acute liver failure(ALF)may be the first and most dramatic presentation of Wilson’s disease(WD).ALF due to WD(WD-ALF)is difficult to distinguish from other causes of liver disease and is a clear indication...BACKGROUND Acute liver failure(ALF)may be the first and most dramatic presentation of Wilson’s disease(WD).ALF due to WD(WD-ALF)is difficult to distinguish from other causes of liver disease and is a clear indication for liver transplantation.There is no firm recommendation on specific and supportive medical treatment for this condition.AIM To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native liver.METHODS A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to 2023.RESULTS During the study period,16 children(9 males)received a diagnosis of WD and 2 of them presented with ALF.The first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids,and survived without liver transplant.The second,exclusively treated with supportive therapy,needed a hepatotransplant to overcome ALF.CONCLUSION Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition,which is currently only treated with liver transplantation.展开更多
The posttreatment period is a key part of the management of pediatric cancer.During this time,school and psychological difficulties have been described in childhood cancer survivors(CCS)and can be prognostic for the s...The posttreatment period is a key part of the management of pediatric cancer.During this time,school and psychological difficulties have been described in childhood cancer survivors(CCS)and can be prognostic for the success of social reintegration.This study estimated the influence of the household’s socioeconomic status(SES)on these psychosocial difficulties.This study is based on a prospective multicentric database and focused on children who received a psychosocial evaluation during their follow-up from 2013 to 2020.We retrieved data on school and psychological difficulties.Household SES was estimated by a social deprivation score.Data from1003 patients were analyzed.School difficulties were noted in 22%of CCS.A greater social deprivation was significantly associated with school difficulty.Tumor relapse,treatment with hematopoietic stem cell transplantation,and central nervous system(CNS)tumors remained significant risk factors.In the subgroup of CNS tumors,school difficulties were increased and associated with greater social deprivation.Psychological difficulties were not associated with the deprivation score.There is a link between SES and school difficulties in CCS.Further investigations should be carried out for children with CNS tumors,which is the population of the greatest concern.展开更多
Objective: This study aims to systematically examine the existing evidence regarding the clinical benefits of carbocysteine as an adjunctive treatment in acute bronchopulmonary and otorhinological processes. Design: S...Objective: This study aims to systematically examine the existing evidence regarding the clinical benefits of carbocysteine as an adjunctive treatment in acute bronchopulmonary and otorhinological processes. Design: Systematic review and meta-analysis. Data sources: An electronic search was conducted across PubMed, Cochrane Library, clinicaltrials.gov, and the European Clinical Trial Register, with the search dated to May 2023. Bibliographic references from other literature reviews and meta-analyses were also reviewed. The search was limited to randomized clinical trials published in any language and year. It was completed by cross-checking the references of the located articles. Methods: Inclusion criteria covered studies assessing systemic or inhaled carbocysteine, regardless of dosing regimen. Concomitant medication use was acceptable if balanced between intervention and control groups. Authors independently extracted data, resolving disagreements through consensus. Methodological quality assessment relied on critical reading of each study. Dichotomous variables were analyzed using odds ratio (OR), and a final effect size was calculated. Statistical significance was established when confidence intervals did not cross the neutral value. Heterogeneity was assessed via the X<sup>2</sup> test and I<sup>2</sup> index. Results: Out of 318 initially identified studies, 4 met inclusion criteria. The meta-analysis for poor general condition yielded an OR of 0.45 in favor of intervention, p = 0.013, with non-significant heterogeneity. Cough events showed a percentage of 15.8% for carbocysteine vs. 27.2% for placebo. On the seventh day, expectoration rates were 18.37% for carbocysteinevs 33.3% for placebo. Conclusions: The observed clinical benefits align with carbocysteine’s mucoactive and muco-regulatory properties, complemented by anti-inflammatory and antioxidant actions. Carbocysteine stands out among mucolytic agents. In the context of persistent infectious diseases, the study emphasizes the need for further exploration of carbocysteine’s therapeutic potential as an adjunctive treatment for acute respiratory infections. These findings underscore its significance in the evolving landscape of respiratory healthcare.展开更多
Developmental dysplasia of the hip(DDH) describes the spectrum of structural abnormalities that involve the growing hip. Early diagnosis and treatment is critical to provide the best possible functional outcome. Persi...Developmental dysplasia of the hip(DDH) describes the spectrum of structural abnormalities that involve the growing hip. Early diagnosis and treatment is critical to provide the best possible functional outcome. Persistence of hip dysplasia into adolescence and adulthood may result in abnormal gait, decreased strength and increased rate of degenerative hip and knee joint disease. Despite efforts to recognize and treat all cases of DDH soon after birth, diagnosis is delayed in some children, and outcomes deteriorate with increasing delay of presentation. Different screening programs for DDH were implicated. The suspicion is raised based on a physical examination soon after birth. Radiography and ultrasonography are used to confirm the diagnosis. The role of other imaging modalities, such as magnetic resonance imaging, is still undetermined; however, extensive research is underway on this subject. Treatment depends on the age of the patient and the reducibility of the hip joint. At an early age and up to 6 mo, the main treatment is an abduction brace like the Pavlik harness. If this fails, closed reduction and spica casting is usually done. After the age of 18 mo, treatment usually consists of open reduction and hip reconstruction surgery. Various treatment protocols have been proposed. We summarize the current practice for detection and treatment of DDH, emphasizing updates in screening and treatment during the last two decades.展开更多
AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed ...AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012.Patients diagnosed with DIOS were defined as the study group.The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan.For the control group,CF patients with no DIOS were matched to the patients in the study group for age,sex,and cystic fibrosis transmembrane conductance regulator(CFTR) mutations.For both groups,the collected data included age,sex,CFTR genotype,weight,height,and body mass index.Clinical data included respiratory function tests in the last five years prior to the study,respiratory function test immediately before and after the DIOS event,number of hospitalizations,sputum culture results,and CFrelated conditions diagnosed according to the CF clinical practice guidelines.In the study group,data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.RESULTS: The medical charts for a total of 350 CF patients were reviewed.Of the 350 CF patients,26(7.4%) were diagnosed with DIOS.The control group included 31 CF patients with no DIOS diagnosis.The mean follow-up period was 21.6 ± 8.2 years.The total of DIOS episodes in the follow-up period was 60.The distribution of DIOS episodes was as follows: 6/26(23.1%) study patients had one episode of DIOS intheir lifetime,7/26(26.9%) had two episodes,7/26(26.9%) had three episodes,and 6/26(23.1%) had four or more episodes.Compared to the control group,DIOS patients had a significantly higher incidence of meconium ileus in the past(65.4% vs 0%,respectively,P < 0.02),more Aspergillus spp.colonization(34.6% vs 3.2%,respectively,P < 0.02),and a higher number of hospitalizations due to respiratory exacerbations(8.6 vs 6.2 mean total hospitalizations per follow-up period,respectively,P < 0.02).No other significant differences were found between the control and study groups.The conservative treatment of DIOS,which mainly includes hydration and stool softeners,was successful in 82% of the episodes.The survival rate was similar for both groups.CONCLUSION: CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition.Although recurrence of DIOS is common,conservative treatment is successful in most patients.展开更多
AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 ...AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 （sICAM-1） and clinical outcome in BA patients after surgical treatment, METHODS： Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA rnebhod from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups： patients without jaundice and those with persistent jaundice. RESULTS： There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1 genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1 polymorphism and the status of jaundice in BA patients after Kasai operation. CONCLUSION： ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469E ICAM-1 polymorphism.展开更多
Diet therapies are currently under-utilised in optimising clinical outcomes for patients with active ulcerative colitis(UC).Furthermore,existing dietary therapies are framed by poorly defined mechanistic targets to wa...Diet therapies are currently under-utilised in optimising clinical outcomes for patients with active ulcerative colitis(UC).Furthermore,existing dietary therapies are framed by poorly defined mechanistic targets to warrant its success.There is good evidence to suggest that microbial production of gaseous metabolites,hydrogen sulfide(H2S)and nitric oxide(NO)are implicated in the development of mucosal inflammation in UC.On a cellular level,exposure of the colonic epithelium to excessive concentrations of these gases are shown to promote functional defects described in UC.Hence,targeting bacterial production of these gases could provide an opportunity to formulate new dietary therapies in UC.Despite the paucity of evidence,there is epidemiological and clinical data to support the concept of reducing mucosal inflammation in UC via dietary strategies that reduce H2S.Several dietary components,namely sulphurcontaining amino acids and inorganic sulphur have been shown to be influential in enhancing colonic H2S production.More recent data suggests increasing the supply of readily fermentable fibre as an effective strategy for H2S reduction.Conversely,very little is known regarding how diet alters microbial production of NO.Hence,the current evidence suggest that a whole diet approach is needed.Finally,biomarkers for assessing changes in microbial gaseous metabolites in response to dietary interventions are very much required.In conclusion,this review identifies a great need for high quality randomised-controlled trials to demonstrate the efficacy of a sulphide-reducing dietary therapy for patients with active UC.展开更多
文摘BACKGROUND Post-streptococcal acute glomerular nephritis(PSAGN)is mostly a benign condition.The usual sequelae of PSAGN include hypertension,its complications,and acute kidney injury.Severe PSAGN is associated with significant long-term morbidity,and histological abnormalities such as crescentic glomerulonephritis are infrequently reported.PSAGN has also been linked to late-onset chronic kidney disease in some populations due to high levels of proteinuria.METHODS This prospective observational study was conducted at Lady Ridgeway Hospital(Colombo,Sri Lanka)over 15 months.Children with PSAGN were enrolled based on clinical and laboratory criteria.Persistent proteinuria≥2+for 2 weeks and serum creatinine>100μmol/L warranted renal biopsy,assessed via light microscopy and immunofluorescence.Normalization of complement 3(C3)within 6 to 8 weeks was required for inclusion.Data on clinical features,urine protein levels,and renal function were collected from patient records,and potential associations were analysed using Statistical Package for the Social Sciences and R language for statistical computing.Ethical approval was obtained from the Ethical Review Committee,Lady Ridgeway Hospital for Children(Ref No:LRH/ERC/2021/60).RESULTS Forty-four patients were recruited.There were 27(61.4%)male patients and 17(38.6%)female patients.Thirty-seven(84%)of them were above 5 years of age.Twenty(45%)patients had a history of skin sepsis,and eighteen(41%)had a history of throat infection.Among patients with proteinuria≥2+,53%had serum creatinine>100µmol/L,while among those with proteinuria<2+,7%had serum creatinine>100µmol/L.The association of high-degree proteinuria with elevated serum creatinine was significant(χ²=7.8,P=0.005)in PSAGN.The odds ratio of the logistic regression model was 1.049(95%confidence interval:1.003-1.098),indicating a positive direction with statistically significant association(P=0.037).There was no significant association between proteinuria and the degree of hypertension or estimated creatinine clearance.Ten children underwent renal biopsy.Crescents(less than 50%)were demonstrated in five children,while three children had typical diffuse proliferative glomer-ulonephritis.One child had severe acute tubular necrosis,and another had crescentic glomerulonephritis(crescents>50%).The immunofluorescence studies revealed deposition of immunoglobulin G and C3 in all biopsy specimens.CONCLUSION High-degree proteinuria was significantly associated with elevated serum creatinine(>100μmol/L)in children with PSAGN.The majority of children with persistent proteinuria≥2+for more than 2 weeks and the highest recorded serum creatinine>100μmol/L had atypical renal histological findings.
文摘BACKGROUND Point-of-care ultrasound(POCUS)is the use of portable ultrasound devices by trained healthcare professionals to diagnose and monitor medical conditions directly at the patient's bedside,such as in emergency settings.We described a case where POCUS use,in Pediatric Emergency Department,allowed an early diagnosis and timely management in the surgical department,with a favorable outcome for child.Therefore we write this case because it is desirable to po-pularize ultrasound as the fifth pillar of clinical examination especially in Emer-gency Department CASE SUMMARY A 13-year-old girl with acute gastrointestinal symptoms,such as vomit and abdominal and lumbar pains.Upon physical examination,the patient had lo-calized abdominal pain in the left lower quadrant.Ultrasonography performed at the bedside revealed an enlarged left ovary with an irregular structure containing a 3 cm cyst.These findings raised suspicion of ovarian torsion.The child tran-sferred to Gynecology Surgery Unit,where she was taken emergently to the ope-rating room CONCLUSION It is desirable to spread POCUS in emergency settings where it allows a sig-nificant saving of time in patient management.
文摘BACKGROUND Disorders of gut-brain interaction(DGBI)are common,but knowledge about their physiopathology is still poor,nor valid tools have been used to evaluate them in childhood.AIM To develop a psycho-gastroenterological questionnaire(PGQ)to assess the psycho-gastroenterological profile and social characteristics of a pediatric population with and without DGBI.METHODS One hundred and nineteen Italian children(age 11-18)were included:28 outpatient patients with DGBI(Rome IV criteria)and 91 healthy controls.They filled the PGQ,faces pain scale revised(FPS-R),Bristol stool chart,ga-strointestinal symptoms rating scale,state-trait anxiety inventory,Toronto alexithymia scale 20,perceived self-efficacy in the management of negative emotions and expression of positive emotions(APEN-G,APEP-G),irritable bowel syndrome-quality of life questionnaire,school performances,tobacco use,early life events,degree of digital-ization.RESULTS Compared to controls,patients had more medical examinations(35%of them went to the doctor more than five times),a higher school performance(23%vs 13%,P<0.05),didn’t use tobacco(never vs 16%,P<0.05),had early life events(28%vs 1%P<0.05)and a higher percentage of pain classified as 4 in the FPS-R during the examination(14%vs 7%,P<0.05).CONCLUSION Pediatric outpatients with DGBI had a higher prevalence of early life events,a lower quality of life,more medical examinations rising health care costs,lower anxiety levels.
文摘BACKGROUND Type 1 diabetes mellitus(T1DM)is an autoimmune disease with a multifactorial pathogenesis.Viral infections have been proposed as contributing triggers,supported by the disease’s seasonal pattern,which typically shows higher incidence in autumn and winter.The coronavirus disease 2019(COVID-19)pandemic and associated lockdowns created a unique context to examine the incidence and seasonality of T1DM during a period characterized by reduced circulation of common viral infections.AIM To investigate the incidence and seasonality of T1DM before and during COVID-19 pandemic in relation to global viral infection rates.METHODS This population-based retrospective study utilized a nationwide computerized database.Extracted data included the number of new T1DM cases over the 8 years preceding and during the COVID-19 pandemic,demographic characteristics of affected individuals,and nationwide respiratory virus polymerase chain reaction data from weekly nasal wash sample collections.RESULTS A total of 2176 patients were diagnosed with new-onset T1DM during the prepandemic period,compared to 348 cases during the pandemic.In the same periods,33727 respiratory virus-positive polymerase chain reaction results from nasal wash samples were recorded pre-pandemic,compared to 2603 during the pandemic.Additionally,363399 positive COVID-19 cases were reported during the pandemic period.Seasonality analysis revealed a higher rate of new-onset T1DM cases and a weaker seasonal pattern during the pandemic.Trend analysis showed a consistent increase in T1DM incidence prior to COVID-19,with a more variable trend observed during the pandemic.Correlation analysis between T1DM incidence and respiratory viruses demonstrated a weak correlation between T1DM incidence and a few respiratory viruses.CONCLUSION The observed increase in new-onset T1DM cases and the disruption of its typical seasonal pattern during the COVID-19 pandemic suggest a potential association between respiratory virus exposure and the development of T1DM.
文摘BACKGROUND Fontan-associated liver disease(FALD)often occurs in patients with single-ventricle physiology following Fontan surgery,and ranges from liver congestion to cirrhosis.The assessment of the severity of FALD using noninvasive methods is challenging.However,transient elastography(TE)may be useful for the non-invasive evaluation of FALD and prediction of clinical outcomes.AIM To evaluate the role of TE in the diagnosis of FALD and its association with clinically relevant events.METHODS This retrospective single-center study(Hospital Universitario La Paz,Madrid),including 91 post-Fontan patients aged>18 years old.Laboratory and ultrasound findings,and liver stiffness measurements(LSM)by TE(FibroScan®)were assessed.FALD was defined using ultrasound criteria hepatomegaly,liver surface nodularity,parenchymal heterogeneity,hyperechoic lesions,spleno-RESULTS Patient characteristics were:60.4%male;Mean age,33.3±8.2 years;Mean elapsed time since surgery,24.3±7.7 years;89%with FALD;73%with advanced FALD.LSM by TE was associated with FALD[odds ratio(OR)=1.34;95%confidence interval(95%CI):1.10-1.64;P=0.003]and advanced FALD(OR=1.10;95%CI:1.01-1.19;P=0.023).Areas under the curve(AUC)were 0.905 and 0.764 for FALD and advanced FALD,respectively.FALD cut-off values comprised:Optimal,20 kPa(sensitivity:92.3%;specificity:80.0%);Rule-out,15 kPa(sensitivity:96.9%);Rule-in,25 kPa(specificity:100%).A FALD algorithm was proposed based on LSM by TE and elapsed time since surgery(AUC:0.877;sensitivity,95.4%;specificity,80.0%;positive predictive value,96.9%;negative predictive value,72.7%).LSM by TE was associated with clinically relevant events(OR=1.07;95%CI:1.01-1.13;P=0.021)and all-cause mortality(OR=1.23;95%CI:1.02-1.47;P=0.026).CONCLUSION In adult patients post-Fontan surgery,TE is a useful noninvasive method for FALD diagnosis.The association between LSM by TE and clinically relevant events suggests a role in prognosis.
文摘BACKGROUND A diagnosis of a chronic disease has been shown to predispose patients to the development of feeding and eating disorders(FEDs).AIM To screen children and adolescents with type 1 diabetes mellitus(T1DM)for FEDs and compare them to their counterparts with short stature.METHODS A total of 110 children and adolescents(55 with T1DM and 55 with short stature)were enrolled in the study.The SCOFF questionnaire was used to screen for possible FEDs,while anthropometric and dietary data were also collected.RESULTS Approximately 60%of the children with T1DM screened positive for FEDs compared to 30.9%of the children with short stature.Having a T1DM tripled the chances of screening positive for FEDs and halved the annual growth rate of children with T1DM.No differences were noted in the dietary intake between groups.CONCLUSION The results necessitate the education of pediatric endocrinologists and diabetologists on proper screening and identification of children at risk for developing FEDs.A prompt diagnosis might help children catch up growth and attain their genetically predisposed height.
文摘Video capsule endoscopy (CE) for evaluation the esophagus (ECE), small bowel (SBCE) and the colon (CCE) is particularly useful in pediatrics, because this imaging modality does not require ionizing radiation, deep sedation or general anesthesia. The risk of capsule retention appears to be dependent on indication rather than age and parallels the adult experience by indication, making SBCE a relatively safe procedure with a significant diagnostic yield. The newest indication, assessment of mucosal change, greatly enhances and expands its potential benefit. The diagnostic role of CE extends beyond the SB. The use of ECE also may enhance our knowledge of esophageal disease and assist patient care. Colon CCE is a novel minimally invasive and painless endoscopic technique allowing exploration of the colon without need for sedation, rectal intubation and gas insufflation. The limited data on ECE and CCE in pediatrics does not yet allow the same conclusions regarding efficacy; however, both appear to provide safe methods to assess and monitor mucosal change in their respective areas with little discomfort. Moreover, although experience has been limited, the patency capsule may help lessen the potential of capsule retention; and newly researched protocols for bowel cleaning may further enhance CE’s diagnostic yield. However, further research is needed to optimize the use of the various CE procedures in pediatric populations.
文摘1 Introduction Kounis syndrome constitutes a coronary hypersensitivity disorder defined by the association of an anaphylactoid,anaphylactic,allergic or hypersensitivity reaction with an acute coronary syndrome,in a physiopathological context involving various interrelated and interacting inflammatory cells,such as mast-cells,eosinophils and platelets.[1,2]Similar entities to Kounis syndrome might involve cerebral and mesenteric arteries.
文摘Objective: Despite the presence of hand washing material and the training given to medical staff regarding hygiene measures and health care procedures in October 2015, the prevalence of nosocomial infections in the neonatal unit of the National University Teaching Hospital of Cotonou (CNHU-Cotonou) was estimated at 8% in January 2016. To determine the factors that contribute to these infections, this study assessed medical staff compliance with hand hygiene measures and procedures. Method: This research was a cross-sectional and observational study conducted from February 15 to March 31, 2016 through direct and cautious observation of 47 members of the medical and paramedical staff. The study variables were hand washing before entering the neonatal unit and before entering each treatment room, hand washing before and after seeing each patient, compliance with hand washing steps, the use of hydroalcoholic solutions and adhering to the ban on mobile phone use inside the treatment room. Results: Only 15% of the medical staff followed all of the rules and measures governing hand hygiene. The result showed that 76.6% of them did not wash their hands before entering the unit;32% washed their hands before each care session;95.7% washed their hands after each care session;and 85% did not comply with the hand washing steps. Only 21.3% of the personnel used hydroalcoholic solution, and only 85% of the personnel adhered to the ban on mobile phone use within the treatment room. Conclusion: Compliance with hand hygiene measures is insufficient. These low compliance rates facilitate the occurrence of nosocomial infections. Nosocomial infections could be prevented by identifying the reasons that medical personnel do not wash their hands and by implementing a program for education/awareness on hygiene measures based on an analysis of errors and care procedures and sustained by regular evaluations.
文摘Transjugular intrahepatic portosystemic shunt(TIPS)placement is a standard procedure for the treatment of portal hypertension complications.When this conventional approach is not feasible,alternative procedures for systemic diversion of portal blood have been proposed.A one-step interventional approach,combining minilaparotomy-assisted transmesenteric(MAT)antegrade portal recanalization and TIPS,is described in an adolescent with recurrent esophageal varice bleeding and portal cavernoma(PC).A 16-yearold girl was admitted to our Unit because of repeated bleeding episodes over a short period of time due to esophageal varices in the context of a PC.A portal vein recanalization through an ileocolic vein isolation with the MAT approach followed by TIPS during the same session was performed.In the case of failed portal recanalization,this approach,would also be useful for varice endovascular embolization.Postoperative recovery was uneventful.Treatment consisting of propanolol,enoxaparin and a proton pump inhibitor was prescribed after the procedure.One month post-op,contrast enhanced computed tomography confirmed the patency of the portal and intrahepatic stent grafts.No residual peritoneal fluid was detected nor opacification of the large varices.Endoscopy showed good improvement of the varices.Doppler ultrasound confirmed the accelerated flow in the portal stent and hepatopetal flow inside the intrahepatic portal branches.Three months post-op,TIPS maintained its hourglass shape despite a slight expansion.Portal hypertension and life threatening conditions related to PC would benefit from one-step portal recanalization.MAT-TIPS is feasible and safe for the treatment of PC even in children.This minimally invasive procedure avoids or delays surgical treatment or re-transplantation when necessary in pediatric patients.
文摘Introduction: In low-income countries, severe anemia represents a significant risk of mortality. It is generally the consequence of a lack of correct community-based management of mild and moderate anemia and its etiologies. Our study aimed to evaluate the community practice in confronting anemia by mothers of children aged 0 - 5 years old before and during the COVID-19 period. Methods: We conducted a descriptive cross-sectional study in two phases at the pediatric ward of the regional hospital of Ngaoundere in Cameroon;before the period of the COVID-19 pandemic from December 2018 to May 2019 and during the pandemic period from December 2020 to May 2021. It was a volunteer sample. Were included in this study all mothers of children aged 0 - 5 years present in the pediatric ward at the time of the survey who agreed to freely answer our questionnaire. Results: Of the 152 mothers surveyed in the “Before COVID-19” group, 69% went to a health facility when they suspected their children were anemic, 20% gave grenadine juice (Red colored soda), 7% went to marabous, 2% bought street medicines and 2% used other home-made potions. Of the 92 mothers included in the “During COVID-19” group, the majority did not go to a health facility: 36% gave grenadine juice and other potions, 26% went to marabouts, 23% bought medicines from the street and only 13% went to a health facility;2% did nothing. The average Group rank is not statistically significantly different between the before and during the COVID-19 periods (p = 0.89). Conclusion: Community behavior and practice in confronting anemia deteriorated during the COVID-19 pandemic, with the majority of mothers resorting to non-effective out-of-hospital solutions. The consequence can be an increase in infant mortality. While waiting for the end of the pandemic, it is judicious and urgent to review the whole health system in Africa and especially in countries with limited resources. We propose developing multiple advanced strategies to promote community visits to prevent anemia and to develop anticipatory management of anemia as well as all the main child-killing pathologies.
文摘The incident of the measles has diminished in the last decades thanks to the widespread use of the vaccine in The United States and Europe. Nevertheless, recently we are seeing new epidemics of measles due to the lack of coverage in some social areas. Measles during pregnancy is associated with high maternal morbidity and mortality, being a frequent cause of abortion in preterm deliveries. We report two cases of measles attended in the Unit of Neonatology held coinciding with the epidemic lived in this area during the last year.
文摘The field of vascular medicine has undergone a profound transformation in the 21st century,transforming our approach to assessment and treatment.Athero-sclerosis,a complex inflammatory disease that affects medium and large arteries,presents a major challenge for researchers and healthcare professionals.This condition,characterized by arterial plaque formation and narrowing,poses sub-stantial challenges to vascular health at individual,national,and global scales.Its repercussions are far-reaching,with clinical outcomes including ischemic heart disease,ischemic stroke,and peripheral arterial disease—conditions with esca-lating global prevalence.Early detection of vascular changes caused by athero-sclerosis is crucial in preventing these conditions,reducing morbidity,and averting mortality.This article underscored the imperative of adopting a holistic approach to grappling with the intricacies,trajectories,and ramifications of atherosclerosis.It stresses the need for a thorough evaluation of the vasculature and the implementation of a multidisciplinary treatment approach.By consi-dering the entire vascular system,healthcare providers can explore avenues for prevention,early detection,and effective management of this condition,ultima-tely leading to improved patient outcomes.We discussed current practices and proposed new directions made possible by emerging diagnostic modalities and treatment strategies.Additionally,we considered healthcare expenditure,resour-ce allocation,and the transformative potential of new innovative treatments and technologies.
文摘BACKGROUND Acute liver failure(ALF)may be the first and most dramatic presentation of Wilson’s disease(WD).ALF due to WD(WD-ALF)is difficult to distinguish from other causes of liver disease and is a clear indication for liver transplantation.There is no firm recommendation on specific and supportive medical treatment for this condition.AIM To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native liver.METHODS A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to 2023.RESULTS During the study period,16 children(9 males)received a diagnosis of WD and 2 of them presented with ALF.The first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids,and survived without liver transplant.The second,exclusively treated with supportive therapy,needed a hepatotransplant to overcome ALF.CONCLUSION Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition,which is currently only treated with liver transplantation.
基金supported by a grant from SFCE INCa (Institut National du Cancer)GOCE (Grand Ouest Cancer de l’Enfant).
文摘The posttreatment period is a key part of the management of pediatric cancer.During this time,school and psychological difficulties have been described in childhood cancer survivors(CCS)and can be prognostic for the success of social reintegration.This study estimated the influence of the household’s socioeconomic status(SES)on these psychosocial difficulties.This study is based on a prospective multicentric database and focused on children who received a psychosocial evaluation during their follow-up from 2013 to 2020.We retrieved data on school and psychological difficulties.Household SES was estimated by a social deprivation score.Data from1003 patients were analyzed.School difficulties were noted in 22%of CCS.A greater social deprivation was significantly associated with school difficulty.Tumor relapse,treatment with hematopoietic stem cell transplantation,and central nervous system(CNS)tumors remained significant risk factors.In the subgroup of CNS tumors,school difficulties were increased and associated with greater social deprivation.Psychological difficulties were not associated with the deprivation score.There is a link between SES and school difficulties in CCS.Further investigations should be carried out for children with CNS tumors,which is the population of the greatest concern.
文摘Objective: This study aims to systematically examine the existing evidence regarding the clinical benefits of carbocysteine as an adjunctive treatment in acute bronchopulmonary and otorhinological processes. Design: Systematic review and meta-analysis. Data sources: An electronic search was conducted across PubMed, Cochrane Library, clinicaltrials.gov, and the European Clinical Trial Register, with the search dated to May 2023. Bibliographic references from other literature reviews and meta-analyses were also reviewed. The search was limited to randomized clinical trials published in any language and year. It was completed by cross-checking the references of the located articles. Methods: Inclusion criteria covered studies assessing systemic or inhaled carbocysteine, regardless of dosing regimen. Concomitant medication use was acceptable if balanced between intervention and control groups. Authors independently extracted data, resolving disagreements through consensus. Methodological quality assessment relied on critical reading of each study. Dichotomous variables were analyzed using odds ratio (OR), and a final effect size was calculated. Statistical significance was established when confidence intervals did not cross the neutral value. Heterogeneity was assessed via the X<sup>2</sup> test and I<sup>2</sup> index. Results: Out of 318 initially identified studies, 4 met inclusion criteria. The meta-analysis for poor general condition yielded an OR of 0.45 in favor of intervention, p = 0.013, with non-significant heterogeneity. Cough events showed a percentage of 15.8% for carbocysteine vs. 27.2% for placebo. On the seventh day, expectoration rates were 18.37% for carbocysteinevs 33.3% for placebo. Conclusions: The observed clinical benefits align with carbocysteine’s mucoactive and muco-regulatory properties, complemented by anti-inflammatory and antioxidant actions. Carbocysteine stands out among mucolytic agents. In the context of persistent infectious diseases, the study emphasizes the need for further exploration of carbocysteine’s therapeutic potential as an adjunctive treatment for acute respiratory infections. These findings underscore its significance in the evolving landscape of respiratory healthcare.
文摘Developmental dysplasia of the hip(DDH) describes the spectrum of structural abnormalities that involve the growing hip. Early diagnosis and treatment is critical to provide the best possible functional outcome. Persistence of hip dysplasia into adolescence and adulthood may result in abnormal gait, decreased strength and increased rate of degenerative hip and knee joint disease. Despite efforts to recognize and treat all cases of DDH soon after birth, diagnosis is delayed in some children, and outcomes deteriorate with increasing delay of presentation. Different screening programs for DDH were implicated. The suspicion is raised based on a physical examination soon after birth. Radiography and ultrasonography are used to confirm the diagnosis. The role of other imaging modalities, such as magnetic resonance imaging, is still undetermined; however, extensive research is underway on this subject. Treatment depends on the age of the patient and the reducibility of the hip joint. At an early age and up to 6 mo, the main treatment is an abduction brace like the Pavlik harness. If this fails, closed reduction and spica casting is usually done. After the age of 18 mo, treatment usually consists of open reduction and hip reconstruction surgery. Various treatment protocols have been proposed. We summarize the current practice for detection and treatment of DDH, emphasizing updates in screening and treatment during the last two decades.
文摘AIM: To investigate the long-term follow-up of distal intestinal obstruction syndrome(DIOS) in Israeli cystic fibrosis(CF) patients.METHODS: This is a multi-center,comparative,retrospective study in which we reviewed the medical records of all CF patients from three major CF centers in Israel who were treated in the period from 1980 to 2012.Patients diagnosed with DIOS were defined as the study group.The patients were diagnosed with DIOS based on their clinical presentation and typical findings on either abdominal X-ray or computerized tomography scan.For the control group,CF patients with no DIOS were matched to the patients in the study group for age,sex,and cystic fibrosis transmembrane conductance regulator(CFTR) mutations.For both groups,the collected data included age,sex,CFTR genotype,weight,height,and body mass index.Clinical data included respiratory function tests in the last five years prior to the study,respiratory function test immediately before and after the DIOS event,number of hospitalizations,sputum culture results,and CFrelated conditions diagnosed according to the CF clinical practice guidelines.In the study group,data on the DIOS treatment and tendency for DIOS recurrence were also analyzed.RESULTS: The medical charts for a total of 350 CF patients were reviewed.Of the 350 CF patients,26(7.4%) were diagnosed with DIOS.The control group included 31 CF patients with no DIOS diagnosis.The mean follow-up period was 21.6 ± 8.2 years.The total of DIOS episodes in the follow-up period was 60.The distribution of DIOS episodes was as follows: 6/26(23.1%) study patients had one episode of DIOS intheir lifetime,7/26(26.9%) had two episodes,7/26(26.9%) had three episodes,and 6/26(23.1%) had four or more episodes.Compared to the control group,DIOS patients had a significantly higher incidence of meconium ileus in the past(65.4% vs 0%,respectively,P < 0.02),more Aspergillus spp.colonization(34.6% vs 3.2%,respectively,P < 0.02),and a higher number of hospitalizations due to respiratory exacerbations(8.6 vs 6.2 mean total hospitalizations per follow-up period,respectively,P < 0.02).No other significant differences were found between the control and study groups.The conservative treatment of DIOS,which mainly includes hydration and stool softeners,was successful in 82% of the episodes.The survival rate was similar for both groups.CONCLUSION: CF patients with DIOS suffer from recurrent hospitalizations and airway pathogen acquisition.Although recurrence of DIOS is common,conservative treatment is successful in most patients.
文摘AIM： To determine whether there was an association between inter-cellular adhesion molecule-1 （ICAM-1） gene polymorphism and biliary atresia （BA）, and to investigate the relationship between serum soluble ICAM-1 （sICAM-1） and clinical outcome in BA patients after surgical treatment, METHODS： Eighty-three BA patients and 115 normal controls were genotyped. K469EICAM-1 polymorphism was analyzed using PCR assay. Serum sICAM-1 was determined using ELISA rnebhod from 72 BA patients. In order to evaluate the association between these variables and their clinical outcome, the patients were categorized into two groups： patients without jaundice and those with persistent jaundice. RESULTS： There were no significant differences between BA patients and controls in terms of gender, K469E ICAM-1 genotypes, and alleles. The proportion of patients having serum sICAM-1 ≥3 500 ng/mL in persistent jaundice group was significantly higher than that in the other group. In addition, there was no association between K469EICAM-1 polymorphism and the status of jaundice in BA patients after Kasai operation. CONCLUSION： ICAM-1 possibly plays an important and active role in the disease progression. However, the process is not associated with genetic variation of K469E ICAM-1 polymorphism.
文摘Diet therapies are currently under-utilised in optimising clinical outcomes for patients with active ulcerative colitis(UC).Furthermore,existing dietary therapies are framed by poorly defined mechanistic targets to warrant its success.There is good evidence to suggest that microbial production of gaseous metabolites,hydrogen sulfide(H2S)and nitric oxide(NO)are implicated in the development of mucosal inflammation in UC.On a cellular level,exposure of the colonic epithelium to excessive concentrations of these gases are shown to promote functional defects described in UC.Hence,targeting bacterial production of these gases could provide an opportunity to formulate new dietary therapies in UC.Despite the paucity of evidence,there is epidemiological and clinical data to support the concept of reducing mucosal inflammation in UC via dietary strategies that reduce H2S.Several dietary components,namely sulphurcontaining amino acids and inorganic sulphur have been shown to be influential in enhancing colonic H2S production.More recent data suggests increasing the supply of readily fermentable fibre as an effective strategy for H2S reduction.Conversely,very little is known regarding how diet alters microbial production of NO.Hence,the current evidence suggest that a whole diet approach is needed.Finally,biomarkers for assessing changes in microbial gaseous metabolites in response to dietary interventions are very much required.In conclusion,this review identifies a great need for high quality randomised-controlled trials to demonstrate the efficacy of a sulphide-reducing dietary therapy for patients with active UC.
