<strong>Introduction</strong><strong>:</strong> Warkany syndrome, also called trisomy 8 mosaicism (T8M), is a rare genetic abnormality characterized by a large phenotypic variability. This hete...<strong>Introduction</strong><strong>:</strong> Warkany syndrome, also called trisomy 8 mosaicism (T8M), is a rare genetic abnormality characterized by a large phenotypic variability. This heterogeneity leads to delayed diagnosis in the majority of cases. Frequently, development retardation is the first apparent anomaly that imposes genetic study, and hence diagnosis is done. In other situations, the revealing presentation is atypical. <strong>Aims:</strong> Report a case of T8M in a child followed up for recurrent respiratory infections and insists on the global assessment of patients. <strong>Case Description:</strong> The patient, aged six years old, has been followed up since his first year for recurrent infections. At birth, the medical assessment was normal apart from bilateral testis ectopia and hypospadias. By the age of nine months, he presented several respiratory infections associated with wheezing thereafter. Immunity investigations were normal and skin tests were positive for dog hair. By allergen eviction and asthma therapeutics, the infant improved. During his follow-up, development retardation has been noted in addition to facial dysmorphism and limb extremities aberrations. Imaging investigations showed the agenesis of both the corpus callosum and the right kidney. Karyotyping on peripheral leucocytes and fibroblast culture revealed T8M in 6% and 87% of examined cells respectively. <strong>Conclusion:</strong> In the present case, the patient’s complaint is related to allergy. However, a global assessment of the child led to a rare condition requiring more care and careful follow-up.展开更多
Objective:To explore the treatment strategies and clinical effects for recurrent respiratory infections in children.Methods:From May 2022 to May 2024,100 pediatric patients with recurrent respiratory infections were s...Objective:To explore the treatment strategies and clinical effects for recurrent respiratory infections in children.Methods:From May 2022 to May 2024,100 pediatric patients with recurrent respiratory infections were selected in this study and evenly divided into two groups.The control group(50 patients)was treated with conventional therapy supplemented with budesonide,while the observation group(50 patients)received pidotimod treatment in addition to the control group’s treatment.Subsequently,the duration of clinical symptom improvement,respiratory function enhancement,serological index changes,reinfection status,and parental satisfaction were compared between the two groups.Results:In terms of clinical symptoms,the observation group showed significantly shorter durations of fever reduction,cough relief,tonsil swelling reduction,and disappearance of fine wet rales compared to the control group(average reduction times were 1.6 days,2.3 days,2.1 days,and 1.9 days,respectively,P<0.05).Regarding respiratory function,the observation group experienced a 12%increase in peak expiratory flow rate variability,a 0.6-liter increase in lung capacity,a 0.7-liter increase in forced lung capacity,and a 0.5-liter increase in forced expiratory volume in the first second after treatment,all significantly higher than the control group(P<0.05).Serological testing revealed that interferon-γand interleukin-2 levels increased by 15%and 18%,respectively,while interferon-α,interleukin-5,and interleukin-4 levels decreased by 10%,12%,and 9%,respectively,in the observation group,showing significant differences compared to the control group(P<0.05).Additionally,the reinfection rate in the observation group(10%)was significantly lower than that in the control group(30%),with an average reduction of two reinfections within one year and a 3.2-day shorter infection control time(P<0.05).In terms of parental satisfaction,the observation group achieved 95%,significantly higher than the 70%in the control group(P<0.05).Conclusion:The addition of pidotimod to conventional therapy for pediatric patients with recurrent respiratory infections can significantly alleviate clinical symptoms,promote the recovery of respiratory function,regulate serological indicators,effectively reduce the risk of reinfection,and improve parental satisfaction.This method deserves widespread clinical application.展开更多
BACKGROUND Lower extremity lymphedema is a common complication following treatment for gynecological malignancies.Its incidence rate can reach up to 70%,affecting~20 million people worldwide.However,specialized treatm...BACKGROUND Lower extremity lymphedema is a common complication following treatment for gynecological malignancies.Its incidence rate can reach up to 70%,affecting~20 million people worldwide.However,specialized treatment centers are scarce,and there is a lack of consensus on treatment approaches.Furthermore,there are even fewer reports on the systematic and effective treatment of severe lymphedema with malformations.Effective management of this condition remains a significant challenge for clinicians.CASE SUMMARY A 40-year-old woman developed bilateral leg swelling 6 years after receiving treatment for endometrial cancer.Since August 2018,she experienced>30 episodes of lymphangitis.Upon presentation,she exhibited bilateral leg swelling and deformation,with four large swellings in the posterior thigh that impeded movement,and pain in the limbs.Skin manifestations included lichenoid lesions and features of deep sclerosis.Radionuclide lymphoscintigraphy confirmed the diagnosis of lower limb lymphedema.After 6 mo of complex decongestive therapy(CDT)and three lymphaticovenous anastomosis(LVA)treatments,the patient lost 49 kg in weight.She also experienced a maximum circumference reduction of 35.2 cm in the left lower limb and 37.5 cm in the right lower limb.The leg pain disappeared,her swelling significantly decreased,and she regained the ability to walk,cycle,and run normally.CONCLUSION The combined application of CDT and LVA therapy demonstrates significant positive effects in the treatment of severe,deformed stage III lymphedema.展开更多
BACKGROUND Facial herpes is a common form of the herpes simplex virus-1 infection and usually presents as vesicles near the mouth,nose,and periocular sites.In contrast,we observed a new facial symptom of herpes on the...BACKGROUND Facial herpes is a common form of the herpes simplex virus-1 infection and usually presents as vesicles near the mouth,nose,and periocular sites.In contrast,we observed a new facial symptom of herpes on the entire face without vesicles.CASE SUMMARY A 33-year-old woman with a history of varicella infection and shingles since an early age presented with sarcoidosis of the entire face and neuralgia without oral lesions.The patient was prescribed antiviral treatment with valacyclovir and acyclovir cream.One day after drug administration,facial skin lesions and neurological pain improved.Herpes simplex without oral blisters can easily be misdiagnosed as pimples upon visual examination in an outpatient clinic.CONCLUSION As acute herpes simplex is accompanied by neuralgia,prompt diagnosis and prescription are necessary,considering the pathological history and health conditions.展开更多
Selective immunoglobulin A deficiency(SIgAD)is considered to be the most common human primary immune-deficiency disease in the world.However,the incidence in China is obviously lower than Caucasian races.The definitio...Selective immunoglobulin A deficiency(SIgAD)is considered to be the most common human primary immune-deficiency disease in the world.However,the incidence in China is obviously lower than Caucasian races.The definition of SIgAD has changed over time with the progress of people’s understanding.The scientific community did not reach a consensus on the definition until 1999.As a result,many previously reported cases need to be excluded under the current definition.SIgAD can lead to several spectra of diseases including infections and autoimmune diseases.We retrospectively summarized the SIgAD patients in Peking Union Medical College Hospital(PUMCH),and summarized the Chinese SIgAD reported in China and abroad in past 40 years.Fourty three SIgAD patients were confirmed in the study,in which 9 were healthy without clinical symptoms.Of the 34 patients with clinical symptoms,recurrent infections were found in 29(85.3%)patients;13(38.2%)patients were with autoimmune diseases;6(17.6%)cases had allergic symptoms;3 patients(8.8%)were with tumors,only one case(2.9%)had a family history.Compared with other countries,sIgAD patients in China showed similar symptoms,but the rate of recurrent infections and autoimmune diseases were higher than some other countries;most of the allergic symptoms are drug allergy,different with the allergic sequelae reported in other countries,such as asthma,rhinitis,food allergy and atopic dermatitis;and it is rare to have family history in Chinese patients.We also figured out that more female SIgAD patients tend to have more autoimmune diseases than men(P=0.039).展开更多
AIM:To evaluate dry eye disease(DED)symptomatology and mental health status in different COVID-19 patients.METHODS:A cross-sectional observational design was used.Totally 123 eligible adults(46.34%of men,age range,18-...AIM:To evaluate dry eye disease(DED)symptomatology and mental health status in different COVID-19 patients.METHODS:A cross-sectional observational design was used.Totally 123 eligible adults(46.34%of men,age range,18-59y)with COVID-19 included in the study from August to November,2022.Ocular Surface Disease Index(OSDI),Five-item Dry Eye Questionnaire(DEQ-5),Hospital Anxiety and Depression Scale(HADS),and Pittsburgh Sleep Quality Index(PSQI)were used in this study.RESULTS:OSDI scores were 6.82(1.25,15.91)in asymptomatic carriers,7.35(2.50,18.38)in mild cases,and 16.67(4.43,28.04)in recurrent cases,with 30.00%,35.56%,and 57.89%,respectively evaluated as having DED symptoms(χ2=7.049,P=0.029).DEQ-5 score varied from 2.00(0,6.00)in asymptomatic carriers,3.00(0,8.00)in mild cases,and 8.00(5.00,10.00)in recurrent cases,with 27.50%,33.33%,and 55.26%,respectively assessed as having DED symptoms(χ2=8.532,P=0.014).The prevalence of clinical anxiety(50.00%)and depression(47.37%)symptoms were also significantly higher in patients with recurrent infection(χ2=24.541,P<0.001;χ2=30.871,P<0.001).Recurrent infection was a risk factor for high OSDI scores[odds ratio,2.562;95%confidence interval(CI),1.631-7.979;P=0.033]and DEQ-5 scores(odds ratio,3.353;95%CI,1.038-8.834;P=0.043),whereas having a fixed occupation was a protective factor for OSDI scores(odds ratio,0.088;95%CI,0.022-0.360;P=0.001)and DEQ-5 scores(odds ratio,0.126;95%CI,0.039-0.405;P=0.001).CONCLUSION:Patients with recurrent COVID-19 have more severe symptoms of DED,anxiety,and depression.展开更多
Hepatitis B virus (HBV)-related liver disease is the leading indication for liver transplantation (LT) in Asia,especially in China.With the introduction of hepatitis B immunoglobulin (HBIG) and oral antiviral drugs,th...Hepatitis B virus (HBV)-related liver disease is the leading indication for liver transplantation (LT) in Asia,especially in China.With the introduction of hepatitis B immunoglobulin (HBIG) and oral antiviral drugs,the recurrent HBV infection rate after LT has been evidently reduced.However,complete eradication of recurrent HBV infection after LT is almost impossible.Recurrent graft infection may lead to rapid disease progression and is a frequent cause of death within the fi rst year after LT.At present,the availability of new oral medications,especially nucleoside or nucleotide analogues such as adefovir dipivoxil,entecavir and tenofovir disoproxil fumarate,further strengthens our ability to treat recurrent HBV infection after LT.Moreover,since combined treatment with HBIG and antiviral agents after liver re-transplantation may play an important role in improving the prognosis of recurrent HBV infection,irreversible graft dysfunction secondary to recurrent HBV infection in spite of oral medications should no longer be considered an absolute contraindication for liver re-transplantation.Published reviews focusing on the therapeutic strategies for recurrent HBV infection after LT are very limited.In this article,the current therapeutic strategies for recurrent HBV infection after LT and evolving new trends are reviewed to guide clinical doctors to choose an optimal treatment plan in different clinical settings.展开更多
Objective:To explore the association between dietary habits and recurrent respiratory infection(RRI)in children aged 0e14 years.Methods:This caseecontrol study compared dietary data of children with(cases)and without ...Objective:To explore the association between dietary habits and recurrent respiratory infection(RRI)in children aged 0e14 years.Methods:This caseecontrol study compared dietary data of children with(cases)and without RRI(controls)collected via structured questionnaire.Participants were recruited from Chinese medicine clinics,hospitals,and children’s learning institutions in Beijing.A logistic regression analysis and odds ratio(OR)calculations were conducted using SPSS 17.0 software.Results:A total of 241 questionnaires were collected(case:control ratio:approximately 2:1).Frequent consumption of processed foods(OR Z 2.988,95%confidence intervals 1.375e6.491)and high-sugar foods(OR Z 2.268,95%confidence intervals 1.163e4.424),frequent picky eating(OR Z 2.614,95%confidence intervals 1.363e5.014),and a meat-heavy diet with fewer vegetables(OR Z 1.830,95%confidence intervals 1.358e2.467)correlated positively correlated with RRI.Additionally,57.80%of the children with RRI were addicted to high-sugar foods,compared with 41.57%of the children without RRI(P Z.015).Furthermore,63.16%of the children with RRI were picky eaters,compared with 48.31%of the children without RRI(P Z.024).Finally,30.92%of the children with RRI frequently consumed processed foods,compared with only 17.98%of the children without RRI(P Z.027Conclusion:Although RRI correlates positively with several dietary habits,in the future,prospective cohort studies with larger samples are needed to generalize these findings.展开更多
BACKGROUND Tofacitinib is an oral Janus kinase(JAK)inhibitor that is currently approved by the United States Food and Drug Administration for the treatment of rheumatoid arthritis(RA).Varicella zoster virus reactivati...BACKGROUND Tofacitinib is an oral Janus kinase(JAK)inhibitor that is currently approved by the United States Food and Drug Administration for the treatment of rheumatoid arthritis(RA).Varicella zoster virus reactivation leading to herpes zoster(HZ)is an adverse effect of this drug;however,recurrent HZ at the same site is a rare clinical condition.CASE SUMMARY A 70-year-old female RA patient had undergone 1-year of tofacitinib treatment(10 mg daily).About 1 mo after initiation of oral tofacitinib,she developed blisters on the left flank and abdomen and was diagnosed with HZ;antiviral therapy with acyclovir was resolutory.However,5 d prior to presentation at our hospital,erythema and blisters with severe pain recurred at the same site.Small clustered blisters and bullous were visible on the left lumbar abdomen and perineum,with a pain score of 8(visual analogue scale).Antiviral,nutritional supplement,analgesic and other treatments led to healing but over an atypically long period(approximately 26 d,vs approximately 1 wk).HZ is a common and serious adverse reaction of JAK inhibitors,but it rarely recurs.Our patient’s experience of HZ recurrence at the same site,with a wider affected area,more severe pain and longer healing period,is inconsistent with previous reports.CONCLUSION Same-anatomical site HZ recurrence may occur during oral tofacitinib treatment,with more severe clinical manifestations than in the initial occurrence.展开更多
Common variable immunodeficiency(CVID)is the most common clinically significant primary antibody deficiency diagnosed in adults.The early symptoms are not specific.They include common infections,mainly of the respirat...Common variable immunodeficiency(CVID)is the most common clinically significant primary antibody deficiency diagnosed in adults.The early symptoms are not specific.They include common infections,mainly of the respiratory tract,caused by typical microorganisms,so cases can be missed in primary care.In the majority of patients increased susceptibility to infections coexists with signs or symptoms of autoimmunity,inflammation or polyclonal lymphoproliferation,which can divert diagnosis from immune deficiency.The overall incidence of malignancy is increased in CVID and certain cancers are significantly more common.Lymphomas and gastric carcinoma are the most frequently reported malignancies in CVID,so a high index of suspicion is recommended.Diagnostic delay in CVID is seen worldwide.The main goal of this paper is to increase the awareness about CVID among health care professionals.We aim to present features which can be helpful in CVID diagnosis in order to shorten the“latency”of proper management of CVID patients.We review clinical symptoms,complications and laboratory abnormalities of CVID.Immunoglobulin replacement therapy is regarded as the cornerstone of pharmacological intervention.New modes of Ig application,mainly subcutaneously and via the hyaluronidase-facilitated subcutaneous route,help to adjust therapy to patients’needs and preferences.Still there remain unmet needs.It remains to be seen whether CVID complications can be avoided by earlier diagnosis,treatment and thorough monitoring in the context of increased risk of malignancy.Development of patient tailored protocols depending on the clinical phenotype and risk factors might be more appropriate.The most important consideration is to diagnose suspected cases and stratify patients in a precise and timely way.Work is needed to define features predictive of unfavorable prognosis.展开更多
The adenosine deaminase(ADA) activities in blood lymphocytes of 41normal children and 17 with recurrent respiratory tract infections were examined,and the T-lymphocytes of two children whose ADA activities were obviou...The adenosine deaminase(ADA) activities in blood lymphocytes of 41normal children and 17 with recurrent respiratory tract infections were examined,and the T-lymphocytes of two children whose ADA activities were obviously lower than those of others were cultared in vilro. Then the exogenous human ADA gene was transfected into these cells by means of lipofectin mediated gene transfer. The results showed that the ADA activities in cultured T-lymphocytes were raised and the immunological were also improved.展开更多
Objective:To explore the relationship between gastrointestinal heat retention syndrome and the incidence of pneumonia and recurrent respiratory tract infections(RRTIs)in children.Methods:A prospective cohort study was...Objective:To explore the relationship between gastrointestinal heat retention syndrome and the incidence of pneumonia and recurrent respiratory tract infections(RRTIs)in children.Methods:A prospective cohort study was conducted in the pediatric outpatient department of Beijing Dongfang Hospital.Children without respiratory tract infections(RTIs)were consecutively recruited according to the selection criteria.A semi-structured questionnaire was used to record traditional Chinese medicine(TCM)symptoms and demographic and physiological characteristics.Gastrointestinal heat retention syndrome was considered to be a predisposing factor and was diagnosed according to a scale with reliability and validity.The participants were followed up for 12 months.Participants and their parents or guardians were contacted via clinical interviews and telephone every 6 months.Episodes of pneumonia and RTIs were recorded in detail.Results:A total of 420 children were included.Of participants,370(88.10%)were followed up for 12 months.The mean number of RTI episodes per participant was 5.37(95%CI:5.14 to 5.60).In total,186 participants in the gastrointestinal heat retention syndrome group and 184 participants in the nongastrointestinal heat retention syndrome group completed the 12-month follow-up period.The baseline of both groups was comparable.The incidence of RRTIs in children with gastrointestinal heat retention syndrome was 1.27(95%CI:1.01 to 1.59)times that in children without gastrointestinal heat retention syndrome.Logistic regression analysis revealed that abnormally increased appetite with frequent hunger,foul breath,dry stools,and dark red or purple fingerprints were positively correlated with the incidence of pneumonia.Irascibility and feverish feelings in the palms and soles were positively correlated with the occurrence of RRTI.Conclusions:Gastrointestinal heat retention syndrome is a risk factor for RRTIs in children.Studies with larger sample sizes and longer follow-up time are warranted to confirm the degree of causal risk associated with RTIs.展开更多
This paper describes the similarities and differences between three-character scripture school from Shandong, Shanghai pediatric massage and Hunan Liu Kaiyun pediatric massage in the treatment of Recurrent respiratory...This paper describes the similarities and differences between three-character scripture school from Shandong, Shanghai pediatric massage and Hunan Liu Kaiyun pediatric massage in the treatment of Recurrent respiratory tract in order to standardize operation, collect references and provide the basis for local infantile massage school.展开更多
BACKGROUND In recent decades,an increasing number of patients have received minimally invasive intervention for infected pancreatic necrosis(IPN)because of the benefits in reducing postoperative multiple organ failure...BACKGROUND In recent decades,an increasing number of patients have received minimally invasive intervention for infected pancreatic necrosis(IPN)because of the benefits in reducing postoperative multiple organ failure and mortality.However,there are limited published data regarding infection recurrence after treatment of this patient population.AIM To investigate the incidence and prediction of infection recurrence following successful minimally invasive treatment in IPN patients.METHODS Medical records for 193 IPN patients,who underwent minimally invasive treatment between February 2014 and October 2018,were retrospectively reviewed.Patients,who survived after the treatment,were divided into two groups:one group with infection after drainage catheter removal and another group without infection.The morphological and clinical data were compared between the two groups.Significantly different variables were introduced into the correlation and multivariate logistic analysis to identify independent predictors for infection recurrence.Sensitivity and specificity for diagnostic performance were determined.RESULTS Of the 193 IPN patients,178 were recruited into the study.Of them,9(5.06%)patients died and 169 patients survived but infection recurred in 13 of 178 patients(7.30%)at 7(4-10)d after drainage catheters were removed.White blood cell(WBC)count,serum C-reactive protein(CRP),interleukin-6,and procalcitonin levels measured at the time of catheter removal were significantly higher in patients with infection than in those without(all P<0.05).In addition,drainage duration and length of the catheter measured by computerized tomography scan were significantly longer in patients with infection(P=0.025 and P<0.0001,respectively).Although these parameters all correlated positively with the incidence of infection(all P<0.05),only WBC,CRP,procalcitonin levels,and catheter length were identified as independent predictors for infection recurrence.The sensitivity and specificity for infection prediction were high in WBC count(≥9.95×109/L)and serum procalcitonin level(≥0.05 ng/mL)but moderate in serum CRP level(cut-off point≥7.37 mg/L).The catheter length(cut-off value≥8.05 cm)had a high sensitivity but low specificity to predict the infection recurrence.CONCLUSION WBC count,serum procalcitonin,and CRP levels may be valuable for predicting infection recurrence following minimally invasive intervention in IPN patients.These biomarkers should be considered before removing the drainage catheters.展开更多
Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to ...Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to assess its association with the development of wheezing and recurrent wheezing illness.Methods We searched the literature published between January 1,2010 and June 2,2022 in seven databases.Outcomes included common manifestations and long-term respiratory outcomes of RSV infection in children.Random-and fixed-effect models were used to estimate the effect size and their 95%confidence intervals.Subgroup analysis was conducted by age and HDI levels.This review was registered in PROSPERO(CRD42022379401).Results The meta-analysis included 47 studies.The top five manifestations were cough(92%),nasal congestion(58%),rhinorrhea(53%),shortness of breath(50%),and dyspnea(47%).The clinical symptoms were most severe in infants.In our analysis,compared to very high and high HDI countries,fewer studies in medium HDI countries reported related manifestations,and no study in low HDI countries reported that.The RSV-infected infants were more likely to develop wheezing than the non-infected infants[odds ratio(OR),3.12;95%CI,2.59–3.76]and had a higher risk of developing wheezing illnesses after recovery(OR,2.60;95%CI,2.51–2.70).Conclusions Cough and shortness of breath are common manifestations of RSV infection.More attention should be given to infants and areas with low HDI levels.The current findings confirm an association between RSV infection and wheezing or recurrent wheezing illness.展开更多
Background: Diaphragmatic agenesis or complete absence of a hemidiaphragm in adulthood is rare. The significance of presence of a lung cyst occupying the common thoracoabdominal space lies in the absence of associated...Background: Diaphragmatic agenesis or complete absence of a hemidiaphragm in adulthood is rare. The significance of presence of a lung cyst occupying the common thoracoabdominal space lies in the absence of associated diaphragmatic hernia which has not been documented previously. Case Presentation: We report a case of previously undiagnosed complete absence of a hemidiaphragm in a 62-year-old male who presented with recurrent respiratory infection, episodes of nausea and postprandial fullness. Computed Tomography scan showed a large cyst in relation to the lower lobe of left lung. During surgery, he was found to have complete absence of a left hemidiaphragm and a large cyst arising from the lower lobe of left lung occupied the common thoracoabdominal space. Abdominal viscera were found displaced downwards and fixed in a thick fibrous membrane. There was no herniation of abdominal organs into the thoracic cavity after excision of the cyst. The left lung could be fully expanded and chest was closed after putting a chest drain. Diaphragmatic agenesis did not require any additional procedure. The cyst was excised with closure of bronchocystic opening. The patient had an uneventful recovery. Conclusions: The association of lung cyst with diaphragmatic agenesis (presented in the adulthood) is rare. It has precluded a diaphragmatic hernia and survived the patient acting as a protective barrier. The significance of lung cyst in presence of diaphragmatic agenesis lies in the absence of associated diaphragmatic hernia which has not been documented previously.展开更多
Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this conditi...Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 deficiency.Our search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.展开更多
Importance:Recurrent respiratory tract infection(RRTI)is common in children.Inappropriate RRTI treatment will lead to asthma and other diseases,thereby seriously affecting the growth and physical health of children.Im...Importance:Recurrent respiratory tract infection(RRTI)is common in children.Inappropriate RRTI treatment will lead to asthma and other diseases,thereby seriously affecting the growth and physical health of children.Immune function modulation can prevent and alleviate childhood RRTI.Yupingfeng(YPF),a patented traditional Chinese medicine(TCM),has immunomodulatory effects and is widely used in China to treat children with RRTI.Objective:To evaluate the safety and efficacy of YPF monotherapy in treating children with RRTI.Methods:This multicenter,randomized,double-blind,double-simulation,noninferiority clinical trial was conducted from January 2015 to August 2017,with an 8-week treatment period and 52-week follow-up after the drug withdrawal.Children aged 2–6 years with RRTI meeting the inclusion and exclusion criteria were enrolled in 13 hospitals in China and divided randomly into three groups(2:2:1 ratio)to receive YPF,pidotimod,or placebo.The primary outcome was the proportion of RRTI returning to normal standard level during the follow-up.The secondary outcomes were reduction in the number of RRTI recurrences,effect on clinical symptoms(in accord with TCM practice),effect per symptom,and safety.The trial was registered at the Chinese Clinical Trials Registry(www.chictr.org.cn)under the unique identifier ChiCTR-IPR-15006847.Results:Three hundred and fifty-one children were enrolled and randomly assigned to 3 groups;124,125,and 61 children in the YPF,pidotimod,and placebo groups,respectively,had completed the trial.During the follow-up,the proportion of RRTI returning to normal standard level was 73.13%,67.15%,and 38.81%with YPF,pidotimod,and placebo,respectively(P<0.0001).The proportion of cases who returned to normal standard level in the YPF group was 34.32%higher than that in the placebo group.The safety profile did not significantly differ among the groups.Interpretation:YPF granules were noninferior to the active control drug pidotimod oral solution for the treatment of RRTI in children,and were superior to placebo,with a high safety profile.展开更多
Objective: To explore the correlation between single acupoints used and the recurrence rate of cystitis among cystitis-prone women receiving acupuncture as a prophylactic treatment. Methods: In all, 58 cystitis-pron...Objective: To explore the correlation between single acupoints used and the recurrence rate of cystitis among cystitis-prone women receiving acupuncture as a prophylactic treatment. Methods: In all, 58 cystitis-prone women were included in the analysis. Customised acupuncture treatments were given twice a week, over 4 weeks. The main effect parameter was the number of cystitis episodes during the 6-month observation time. Residual urine was measured at baseline, 2, 4 and 6 months using portable ultrasound equipment. Sympathetic and vagotone nerve activities were measured by using skin conductance and respiratory sinus arrhythmia, respectively. Results: The main acupoints used for patients with Kidney (Shen) qi/yang deficiency were Shenshu (BL23), Taixi (KI3), Zhongji (CV3), Sanyinjiao (SP6) and Pangguangshu (BL2.8), compared with Taichong (LR3), CV3, BL28, Yinlingquan (SP9) and SP6 for Liver (Gan) qi stagnation, and SP6, CV3, BL28, Zusanli (ST36) and SP9 for Spleen (Pi) qi/yang deficiency patients. The combination BL23 and KI3 were used in 16 women, 13 of which were Kidney pattern related patients. When used, the number of symptomatic episodes were reduced to a third compared with what occurred in the 42 women where this combination was not used (3/16 vs. 28/42, P〈0.05). BL23 application correlated to a significant reduction in residual urine measured a few days after treatment. Patients with the pattern of Spleen qi/yang deficiency had an initial increase in residual urine after treatments. Conclusion: Treating Kidney pattern related patients with the combination of BL23 and KI3 resulted in far better outcome than other points/combination of points for other Chinese medicine diagnoses. The acupoint SP6 may be less indicated than previously assumed when treating cvstitis-Drone women DroDhvlacticalIv.展开更多
文摘<strong>Introduction</strong><strong>:</strong> Warkany syndrome, also called trisomy 8 mosaicism (T8M), is a rare genetic abnormality characterized by a large phenotypic variability. This heterogeneity leads to delayed diagnosis in the majority of cases. Frequently, development retardation is the first apparent anomaly that imposes genetic study, and hence diagnosis is done. In other situations, the revealing presentation is atypical. <strong>Aims:</strong> Report a case of T8M in a child followed up for recurrent respiratory infections and insists on the global assessment of patients. <strong>Case Description:</strong> The patient, aged six years old, has been followed up since his first year for recurrent infections. At birth, the medical assessment was normal apart from bilateral testis ectopia and hypospadias. By the age of nine months, he presented several respiratory infections associated with wheezing thereafter. Immunity investigations were normal and skin tests were positive for dog hair. By allergen eviction and asthma therapeutics, the infant improved. During his follow-up, development retardation has been noted in addition to facial dysmorphism and limb extremities aberrations. Imaging investigations showed the agenesis of both the corpus callosum and the right kidney. Karyotyping on peripheral leucocytes and fibroblast culture revealed T8M in 6% and 87% of examined cells respectively. <strong>Conclusion:</strong> In the present case, the patient’s complaint is related to allergy. However, a global assessment of the child led to a rare condition requiring more care and careful follow-up.
文摘Objective:To explore the treatment strategies and clinical effects for recurrent respiratory infections in children.Methods:From May 2022 to May 2024,100 pediatric patients with recurrent respiratory infections were selected in this study and evenly divided into two groups.The control group(50 patients)was treated with conventional therapy supplemented with budesonide,while the observation group(50 patients)received pidotimod treatment in addition to the control group’s treatment.Subsequently,the duration of clinical symptom improvement,respiratory function enhancement,serological index changes,reinfection status,and parental satisfaction were compared between the two groups.Results:In terms of clinical symptoms,the observation group showed significantly shorter durations of fever reduction,cough relief,tonsil swelling reduction,and disappearance of fine wet rales compared to the control group(average reduction times were 1.6 days,2.3 days,2.1 days,and 1.9 days,respectively,P<0.05).Regarding respiratory function,the observation group experienced a 12%increase in peak expiratory flow rate variability,a 0.6-liter increase in lung capacity,a 0.7-liter increase in forced lung capacity,and a 0.5-liter increase in forced expiratory volume in the first second after treatment,all significantly higher than the control group(P<0.05).Serological testing revealed that interferon-γand interleukin-2 levels increased by 15%and 18%,respectively,while interferon-α,interleukin-5,and interleukin-4 levels decreased by 10%,12%,and 9%,respectively,in the observation group,showing significant differences compared to the control group(P<0.05).Additionally,the reinfection rate in the observation group(10%)was significantly lower than that in the control group(30%),with an average reduction of two reinfections within one year and a 3.2-day shorter infection control time(P<0.05).In terms of parental satisfaction,the observation group achieved 95%,significantly higher than the 70%in the control group(P<0.05).Conclusion:The addition of pidotimod to conventional therapy for pediatric patients with recurrent respiratory infections can significantly alleviate clinical symptoms,promote the recovery of respiratory function,regulate serological indicators,effectively reduce the risk of reinfection,and improve parental satisfaction.This method deserves widespread clinical application.
文摘BACKGROUND Lower extremity lymphedema is a common complication following treatment for gynecological malignancies.Its incidence rate can reach up to 70%,affecting~20 million people worldwide.However,specialized treatment centers are scarce,and there is a lack of consensus on treatment approaches.Furthermore,there are even fewer reports on the systematic and effective treatment of severe lymphedema with malformations.Effective management of this condition remains a significant challenge for clinicians.CASE SUMMARY A 40-year-old woman developed bilateral leg swelling 6 years after receiving treatment for endometrial cancer.Since August 2018,she experienced>30 episodes of lymphangitis.Upon presentation,she exhibited bilateral leg swelling and deformation,with four large swellings in the posterior thigh that impeded movement,and pain in the limbs.Skin manifestations included lichenoid lesions and features of deep sclerosis.Radionuclide lymphoscintigraphy confirmed the diagnosis of lower limb lymphedema.After 6 mo of complex decongestive therapy(CDT)and three lymphaticovenous anastomosis(LVA)treatments,the patient lost 49 kg in weight.She also experienced a maximum circumference reduction of 35.2 cm in the left lower limb and 37.5 cm in the right lower limb.The leg pain disappeared,her swelling significantly decreased,and she regained the ability to walk,cycle,and run normally.CONCLUSION The combined application of CDT and LVA therapy demonstrates significant positive effects in the treatment of severe,deformed stage III lymphedema.
文摘BACKGROUND Facial herpes is a common form of the herpes simplex virus-1 infection and usually presents as vesicles near the mouth,nose,and periocular sites.In contrast,we observed a new facial symptom of herpes on the entire face without vesicles.CASE SUMMARY A 33-year-old woman with a history of varicella infection and shingles since an early age presented with sarcoidosis of the entire face and neuralgia without oral lesions.The patient was prescribed antiviral treatment with valacyclovir and acyclovir cream.One day after drug administration,facial skin lesions and neurological pain improved.Herpes simplex without oral blisters can easily be misdiagnosed as pimples upon visual examination in an outpatient clinic.CONCLUSION As acute herpes simplex is accompanied by neuralgia,prompt diagnosis and prescription are necessary,considering the pathological history and health conditions.
基金CAMS Innovation Fund for Medical Sciences(CIFMS)(2016-I2M-1-008)The Capital Health Research and Development of Special(2016-2-40114)+1 种基金Public Welfare Scientific Research Project of China(201402012)Golden Brige Engineering Seed Found of Beijing Association for Science and Technology(JQ17032).
文摘Selective immunoglobulin A deficiency(SIgAD)is considered to be the most common human primary immune-deficiency disease in the world.However,the incidence in China is obviously lower than Caucasian races.The definition of SIgAD has changed over time with the progress of people’s understanding.The scientific community did not reach a consensus on the definition until 1999.As a result,many previously reported cases need to be excluded under the current definition.SIgAD can lead to several spectra of diseases including infections and autoimmune diseases.We retrospectively summarized the SIgAD patients in Peking Union Medical College Hospital(PUMCH),and summarized the Chinese SIgAD reported in China and abroad in past 40 years.Fourty three SIgAD patients were confirmed in the study,in which 9 were healthy without clinical symptoms.Of the 34 patients with clinical symptoms,recurrent infections were found in 29(85.3%)patients;13(38.2%)patients were with autoimmune diseases;6(17.6%)cases had allergic symptoms;3 patients(8.8%)were with tumors,only one case(2.9%)had a family history.Compared with other countries,sIgAD patients in China showed similar symptoms,but the rate of recurrent infections and autoimmune diseases were higher than some other countries;most of the allergic symptoms are drug allergy,different with the allergic sequelae reported in other countries,such as asthma,rhinitis,food allergy and atopic dermatitis;and it is rare to have family history in Chinese patients.We also figured out that more female SIgAD patients tend to have more autoimmune diseases than men(P=0.039).
文摘AIM:To evaluate dry eye disease(DED)symptomatology and mental health status in different COVID-19 patients.METHODS:A cross-sectional observational design was used.Totally 123 eligible adults(46.34%of men,age range,18-59y)with COVID-19 included in the study from August to November,2022.Ocular Surface Disease Index(OSDI),Five-item Dry Eye Questionnaire(DEQ-5),Hospital Anxiety and Depression Scale(HADS),and Pittsburgh Sleep Quality Index(PSQI)were used in this study.RESULTS:OSDI scores were 6.82(1.25,15.91)in asymptomatic carriers,7.35(2.50,18.38)in mild cases,and 16.67(4.43,28.04)in recurrent cases,with 30.00%,35.56%,and 57.89%,respectively evaluated as having DED symptoms(χ2=7.049,P=0.029).DEQ-5 score varied from 2.00(0,6.00)in asymptomatic carriers,3.00(0,8.00)in mild cases,and 8.00(5.00,10.00)in recurrent cases,with 27.50%,33.33%,and 55.26%,respectively assessed as having DED symptoms(χ2=8.532,P=0.014).The prevalence of clinical anxiety(50.00%)and depression(47.37%)symptoms were also significantly higher in patients with recurrent infection(χ2=24.541,P<0.001;χ2=30.871,P<0.001).Recurrent infection was a risk factor for high OSDI scores[odds ratio,2.562;95%confidence interval(CI),1.631-7.979;P=0.033]and DEQ-5 scores(odds ratio,3.353;95%CI,1.038-8.834;P=0.043),whereas having a fixed occupation was a protective factor for OSDI scores(odds ratio,0.088;95%CI,0.022-0.360;P=0.001)and DEQ-5 scores(odds ratio,0.126;95%CI,0.039-0.405;P=0.001).CONCLUSION:Patients with recurrent COVID-19 have more severe symptoms of DED,anxiety,and depression.
文摘Hepatitis B virus (HBV)-related liver disease is the leading indication for liver transplantation (LT) in Asia,especially in China.With the introduction of hepatitis B immunoglobulin (HBIG) and oral antiviral drugs,the recurrent HBV infection rate after LT has been evidently reduced.However,complete eradication of recurrent HBV infection after LT is almost impossible.Recurrent graft infection may lead to rapid disease progression and is a frequent cause of death within the fi rst year after LT.At present,the availability of new oral medications,especially nucleoside or nucleotide analogues such as adefovir dipivoxil,entecavir and tenofovir disoproxil fumarate,further strengthens our ability to treat recurrent HBV infection after LT.Moreover,since combined treatment with HBIG and antiviral agents after liver re-transplantation may play an important role in improving the prognosis of recurrent HBV infection,irreversible graft dysfunction secondary to recurrent HBV infection in spite of oral medications should no longer be considered an absolute contraindication for liver re-transplantation.Published reviews focusing on the therapeutic strategies for recurrent HBV infection after LT are very limited.In this article,the current therapeutic strategies for recurrent HBV infection after LT and evolving new trends are reviewed to guide clinical doctors to choose an optimal treatment plan in different clinical settings.
基金a National Nature Science Foundation Project(No.81373769)。
文摘Objective:To explore the association between dietary habits and recurrent respiratory infection(RRI)in children aged 0e14 years.Methods:This caseecontrol study compared dietary data of children with(cases)and without RRI(controls)collected via structured questionnaire.Participants were recruited from Chinese medicine clinics,hospitals,and children’s learning institutions in Beijing.A logistic regression analysis and odds ratio(OR)calculations were conducted using SPSS 17.0 software.Results:A total of 241 questionnaires were collected(case:control ratio:approximately 2:1).Frequent consumption of processed foods(OR Z 2.988,95%confidence intervals 1.375e6.491)and high-sugar foods(OR Z 2.268,95%confidence intervals 1.163e4.424),frequent picky eating(OR Z 2.614,95%confidence intervals 1.363e5.014),and a meat-heavy diet with fewer vegetables(OR Z 1.830,95%confidence intervals 1.358e2.467)correlated positively correlated with RRI.Additionally,57.80%of the children with RRI were addicted to high-sugar foods,compared with 41.57%of the children without RRI(P Z.015).Furthermore,63.16%of the children with RRI were picky eaters,compared with 48.31%of the children without RRI(P Z.024).Finally,30.92%of the children with RRI frequently consumed processed foods,compared with only 17.98%of the children without RRI(P Z.027Conclusion:Although RRI correlates positively with several dietary habits,in the future,prospective cohort studies with larger samples are needed to generalize these findings.
基金Supported by the Doctoral Startup Fund of Affiliated Hospital of Weifang Medical University,No.2021BKQ01.
文摘BACKGROUND Tofacitinib is an oral Janus kinase(JAK)inhibitor that is currently approved by the United States Food and Drug Administration for the treatment of rheumatoid arthritis(RA).Varicella zoster virus reactivation leading to herpes zoster(HZ)is an adverse effect of this drug;however,recurrent HZ at the same site is a rare clinical condition.CASE SUMMARY A 70-year-old female RA patient had undergone 1-year of tofacitinib treatment(10 mg daily).About 1 mo after initiation of oral tofacitinib,she developed blisters on the left flank and abdomen and was diagnosed with HZ;antiviral therapy with acyclovir was resolutory.However,5 d prior to presentation at our hospital,erythema and blisters with severe pain recurred at the same site.Small clustered blisters and bullous were visible on the left lumbar abdomen and perineum,with a pain score of 8(visual analogue scale).Antiviral,nutritional supplement,analgesic and other treatments led to healing but over an atypically long period(approximately 26 d,vs approximately 1 wk).HZ is a common and serious adverse reaction of JAK inhibitors,but it rarely recurs.Our patient’s experience of HZ recurrence at the same site,with a wider affected area,more severe pain and longer healing period,is inconsistent with previous reports.CONCLUSION Same-anatomical site HZ recurrence may occur during oral tofacitinib treatment,with more severe clinical manifestations than in the initial occurrence.
文摘Common variable immunodeficiency(CVID)is the most common clinically significant primary antibody deficiency diagnosed in adults.The early symptoms are not specific.They include common infections,mainly of the respiratory tract,caused by typical microorganisms,so cases can be missed in primary care.In the majority of patients increased susceptibility to infections coexists with signs or symptoms of autoimmunity,inflammation or polyclonal lymphoproliferation,which can divert diagnosis from immune deficiency.The overall incidence of malignancy is increased in CVID and certain cancers are significantly more common.Lymphomas and gastric carcinoma are the most frequently reported malignancies in CVID,so a high index of suspicion is recommended.Diagnostic delay in CVID is seen worldwide.The main goal of this paper is to increase the awareness about CVID among health care professionals.We aim to present features which can be helpful in CVID diagnosis in order to shorten the“latency”of proper management of CVID patients.We review clinical symptoms,complications and laboratory abnormalities of CVID.Immunoglobulin replacement therapy is regarded as the cornerstone of pharmacological intervention.New modes of Ig application,mainly subcutaneously and via the hyaluronidase-facilitated subcutaneous route,help to adjust therapy to patients’needs and preferences.Still there remain unmet needs.It remains to be seen whether CVID complications can be avoided by earlier diagnosis,treatment and thorough monitoring in the context of increased risk of malignancy.Development of patient tailored protocols depending on the clinical phenotype and risk factors might be more appropriate.The most important consideration is to diagnose suspected cases and stratify patients in a precise and timely way.Work is needed to define features predictive of unfavorable prognosis.
文摘The adenosine deaminase(ADA) activities in blood lymphocytes of 41normal children and 17 with recurrent respiratory tract infections were examined,and the T-lymphocytes of two children whose ADA activities were obviously lower than those of others were cultared in vilro. Then the exogenous human ADA gene was transfected into these cells by means of lipofectin mediated gene transfer. The results showed that the ADA activities in cultured T-lymphocytes were raised and the immunological were also improved.
基金funded by the National Natural Science Foundation of China(81373769)Beijing Educational Committee cooperation projects(1000062520115)+1 种基金National Key R&D Program of China(2018YFC1704101)granted to Prof.Xiaohong Gufunded by China Postdoctoral Science Foundation(2020T130009ZX)。
文摘Objective:To explore the relationship between gastrointestinal heat retention syndrome and the incidence of pneumonia and recurrent respiratory tract infections(RRTIs)in children.Methods:A prospective cohort study was conducted in the pediatric outpatient department of Beijing Dongfang Hospital.Children without respiratory tract infections(RTIs)were consecutively recruited according to the selection criteria.A semi-structured questionnaire was used to record traditional Chinese medicine(TCM)symptoms and demographic and physiological characteristics.Gastrointestinal heat retention syndrome was considered to be a predisposing factor and was diagnosed according to a scale with reliability and validity.The participants were followed up for 12 months.Participants and their parents or guardians were contacted via clinical interviews and telephone every 6 months.Episodes of pneumonia and RTIs were recorded in detail.Results:A total of 420 children were included.Of participants,370(88.10%)were followed up for 12 months.The mean number of RTI episodes per participant was 5.37(95%CI:5.14 to 5.60).In total,186 participants in the gastrointestinal heat retention syndrome group and 184 participants in the nongastrointestinal heat retention syndrome group completed the 12-month follow-up period.The baseline of both groups was comparable.The incidence of RRTIs in children with gastrointestinal heat retention syndrome was 1.27(95%CI:1.01 to 1.59)times that in children without gastrointestinal heat retention syndrome.Logistic regression analysis revealed that abnormally increased appetite with frequent hunger,foul breath,dry stools,and dark red or purple fingerprints were positively correlated with the incidence of pneumonia.Irascibility and feverish feelings in the palms and soles were positively correlated with the occurrence of RRTI.Conclusions:Gastrointestinal heat retention syndrome is a risk factor for RRTIs in children.Studies with larger sample sizes and longer follow-up time are warranted to confirm the degree of causal risk associated with RTIs.
基金Scientific research project of Shanghai municipal health and family planning commission(20164Y0147)Research project of Shanghai hospital of traditional Chinese medicine(2019LC008)
文摘This paper describes the similarities and differences between three-character scripture school from Shandong, Shanghai pediatric massage and Hunan Liu Kaiyun pediatric massage in the treatment of Recurrent respiratory tract in order to standardize operation, collect references and provide the basis for local infantile massage school.
基金Supported by Beijing Municipal Science and Technology Commission,No.Z171100001017077Beijing Municipal Administration of Hospitals Clinical Medicine Development of special funding support,No.XMLX201404Construction Project of Advanced Clinical Medicine Discipline of Capital Medical University,No.1192070312.
文摘BACKGROUND In recent decades,an increasing number of patients have received minimally invasive intervention for infected pancreatic necrosis(IPN)because of the benefits in reducing postoperative multiple organ failure and mortality.However,there are limited published data regarding infection recurrence after treatment of this patient population.AIM To investigate the incidence and prediction of infection recurrence following successful minimally invasive treatment in IPN patients.METHODS Medical records for 193 IPN patients,who underwent minimally invasive treatment between February 2014 and October 2018,were retrospectively reviewed.Patients,who survived after the treatment,were divided into two groups:one group with infection after drainage catheter removal and another group without infection.The morphological and clinical data were compared between the two groups.Significantly different variables were introduced into the correlation and multivariate logistic analysis to identify independent predictors for infection recurrence.Sensitivity and specificity for diagnostic performance were determined.RESULTS Of the 193 IPN patients,178 were recruited into the study.Of them,9(5.06%)patients died and 169 patients survived but infection recurred in 13 of 178 patients(7.30%)at 7(4-10)d after drainage catheters were removed.White blood cell(WBC)count,serum C-reactive protein(CRP),interleukin-6,and procalcitonin levels measured at the time of catheter removal were significantly higher in patients with infection than in those without(all P<0.05).In addition,drainage duration and length of the catheter measured by computerized tomography scan were significantly longer in patients with infection(P=0.025 and P<0.0001,respectively).Although these parameters all correlated positively with the incidence of infection(all P<0.05),only WBC,CRP,procalcitonin levels,and catheter length were identified as independent predictors for infection recurrence.The sensitivity and specificity for infection prediction were high in WBC count(≥9.95×109/L)and serum procalcitonin level(≥0.05 ng/mL)but moderate in serum CRP level(cut-off point≥7.37 mg/L).The catheter length(cut-off value≥8.05 cm)had a high sensitivity but low specificity to predict the infection recurrence.CONCLUSION WBC count,serum procalcitonin,and CRP levels may be valuable for predicting infection recurrence following minimally invasive intervention in IPN patients.These biomarkers should be considered before removing the drainage catheters.
基金supported by the Non-profit Central Research Institute Fund of the Chinese Academy of Medical Sciences(CAMS)(grant number 2021-RC330-002)the Disease Burden of Respiratory Syncytial Virus and Policy Recommendations Project(grant number 202108180001).
文摘Background Respiratory syncytial virus(RSV)infection in infants is a global health priority.We aimed to investigate the common manifestations of RSV infection by age group and human development index(HDI)level and to assess its association with the development of wheezing and recurrent wheezing illness.Methods We searched the literature published between January 1,2010 and June 2,2022 in seven databases.Outcomes included common manifestations and long-term respiratory outcomes of RSV infection in children.Random-and fixed-effect models were used to estimate the effect size and their 95%confidence intervals.Subgroup analysis was conducted by age and HDI levels.This review was registered in PROSPERO(CRD42022379401).Results The meta-analysis included 47 studies.The top five manifestations were cough(92%),nasal congestion(58%),rhinorrhea(53%),shortness of breath(50%),and dyspnea(47%).The clinical symptoms were most severe in infants.In our analysis,compared to very high and high HDI countries,fewer studies in medium HDI countries reported related manifestations,and no study in low HDI countries reported that.The RSV-infected infants were more likely to develop wheezing than the non-infected infants[odds ratio(OR),3.12;95%CI,2.59–3.76]and had a higher risk of developing wheezing illnesses after recovery(OR,2.60;95%CI,2.51–2.70).Conclusions Cough and shortness of breath are common manifestations of RSV infection.More attention should be given to infants and areas with low HDI levels.The current findings confirm an association between RSV infection and wheezing or recurrent wheezing illness.
文摘Background: Diaphragmatic agenesis or complete absence of a hemidiaphragm in adulthood is rare. The significance of presence of a lung cyst occupying the common thoracoabdominal space lies in the absence of associated diaphragmatic hernia which has not been documented previously. Case Presentation: We report a case of previously undiagnosed complete absence of a hemidiaphragm in a 62-year-old male who presented with recurrent respiratory infection, episodes of nausea and postprandial fullness. Computed Tomography scan showed a large cyst in relation to the lower lobe of left lung. During surgery, he was found to have complete absence of a left hemidiaphragm and a large cyst arising from the lower lobe of left lung occupied the common thoracoabdominal space. Abdominal viscera were found displaced downwards and fixed in a thick fibrous membrane. There was no herniation of abdominal organs into the thoracic cavity after excision of the cyst. The left lung could be fully expanded and chest was closed after putting a chest drain. Diaphragmatic agenesis did not require any additional procedure. The cyst was excised with closure of bronchocystic opening. The patient had an uneventful recovery. Conclusions: The association of lung cyst with diaphragmatic agenesis (presented in the adulthood) is rare. It has precluded a diaphragmatic hernia and survived the patient acting as a protective barrier. The significance of lung cyst in presence of diaphragmatic agenesis lies in the absence of associated diaphragmatic hernia which has not been documented previously.
基金funded by National Natural Science Foundation of China(82101908,82371823)Postdoc Fund of Chongqing Natural Science Foundation(cstc2021jcyj-bshX0226).
文摘Background ELF4 deficiency has been recently recognized as a novel disorder within the spectrum of inborn errors of immunity(IEIs),specifically categorized as a“disease of immune dysregulation.”Cases of this condition,reported by our team and others,are very limited worldwide.As such,our current knowledge of this new disease remains preliminary.This review aims to provide a brief overview of the clinical manifestations,pathogenesis,and treatment strategies for this novel IEI.Data sources A comprehensive review was conducted after an extensive literature search in the PubMed/Medline database and websites concerning transcriptional factor ELF4 and reports concerning patients with ELF4 deficiency.Our search strategy was“ELF4 OR ETS-related transcription factor Elf-4 OR EL4-like factor 4 OR myeloid Elf-1-like factor”as of the time of manuscript submission.Results The current signature manifestations of ELF4 deficiency disorder are recurrent and prolonged oral ulcer,abdominal pain,and diarrhea in pediatric males.In some cases,immunodeficiency and autoimmunity can also be prominent.Targeted Sanger sequencing or whole exome sequencing can be used to detect variation in ELF4 gene.Western blotting for ELF4 expression of the patient’s cells can confirm the pathogenic effect of the variant.To fully confirm the pathogenicity of the variant,further functional test is strongly advised.Glucocorticoid and biologics are the mainstream management of ELF4 deficiency disorder.Conclusions Pediatric males presenting with recurring ulcerations in digestive tract epithelium with or without recurrent fever should be suspected of DEX.When atypical presentations are prominent,variations in ELF4 gene should be carefully evaluated functionally due to the complex nature of ELF4 function.Experience of treating DEX includes use of glucocorticoid and biologics and more precise treatment needs more patients to identify and further mechanistic study.
文摘Importance:Recurrent respiratory tract infection(RRTI)is common in children.Inappropriate RRTI treatment will lead to asthma and other diseases,thereby seriously affecting the growth and physical health of children.Immune function modulation can prevent and alleviate childhood RRTI.Yupingfeng(YPF),a patented traditional Chinese medicine(TCM),has immunomodulatory effects and is widely used in China to treat children with RRTI.Objective:To evaluate the safety and efficacy of YPF monotherapy in treating children with RRTI.Methods:This multicenter,randomized,double-blind,double-simulation,noninferiority clinical trial was conducted from January 2015 to August 2017,with an 8-week treatment period and 52-week follow-up after the drug withdrawal.Children aged 2–6 years with RRTI meeting the inclusion and exclusion criteria were enrolled in 13 hospitals in China and divided randomly into three groups(2:2:1 ratio)to receive YPF,pidotimod,or placebo.The primary outcome was the proportion of RRTI returning to normal standard level during the follow-up.The secondary outcomes were reduction in the number of RRTI recurrences,effect on clinical symptoms(in accord with TCM practice),effect per symptom,and safety.The trial was registered at the Chinese Clinical Trials Registry(www.chictr.org.cn)under the unique identifier ChiCTR-IPR-15006847.Results:Three hundred and fifty-one children were enrolled and randomly assigned to 3 groups;124,125,and 61 children in the YPF,pidotimod,and placebo groups,respectively,had completed the trial.During the follow-up,the proportion of RRTI returning to normal standard level was 73.13%,67.15%,and 38.81%with YPF,pidotimod,and placebo,respectively(P<0.0001).The proportion of cases who returned to normal standard level in the YPF group was 34.32%higher than that in the placebo group.The safety profile did not significantly differ among the groups.Interpretation:YPF granules were noninferior to the active control drug pidotimod oral solution for the treatment of RRTI in children,and were superior to placebo,with a high safety profile.
基金the Norwegian Research Council and the Eckbos Legacy for funding the study
文摘Objective: To explore the correlation between single acupoints used and the recurrence rate of cystitis among cystitis-prone women receiving acupuncture as a prophylactic treatment. Methods: In all, 58 cystitis-prone women were included in the analysis. Customised acupuncture treatments were given twice a week, over 4 weeks. The main effect parameter was the number of cystitis episodes during the 6-month observation time. Residual urine was measured at baseline, 2, 4 and 6 months using portable ultrasound equipment. Sympathetic and vagotone nerve activities were measured by using skin conductance and respiratory sinus arrhythmia, respectively. Results: The main acupoints used for patients with Kidney (Shen) qi/yang deficiency were Shenshu (BL23), Taixi (KI3), Zhongji (CV3), Sanyinjiao (SP6) and Pangguangshu (BL2.8), compared with Taichong (LR3), CV3, BL28, Yinlingquan (SP9) and SP6 for Liver (Gan) qi stagnation, and SP6, CV3, BL28, Zusanli (ST36) and SP9 for Spleen (Pi) qi/yang deficiency patients. The combination BL23 and KI3 were used in 16 women, 13 of which were Kidney pattern related patients. When used, the number of symptomatic episodes were reduced to a third compared with what occurred in the 42 women where this combination was not used (3/16 vs. 28/42, P〈0.05). BL23 application correlated to a significant reduction in residual urine measured a few days after treatment. Patients with the pattern of Spleen qi/yang deficiency had an initial increase in residual urine after treatments. Conclusion: Treating Kidney pattern related patients with the combination of BL23 and KI3 resulted in far better outcome than other points/combination of points for other Chinese medicine diagnoses. The acupoint SP6 may be less indicated than previously assumed when treating cvstitis-Drone women DroDhvlacticalIv.