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Juvenile localized scleroderma is a rare childhood disease, the specific pathogenesis of this disease has not been fully clarified.Early recognition and timely diagnosis are crucial, early intervention is associated with good prognosis.But there is a lack of relatively reliable evaluation of disease activity and treatment response.At present, the main diagnostic basis of the disease is clinical manifestations.When there is doubt about the diagnosis, skin or subcutaneous tissue biopsy is required.In addition, high frequency Doppler ultrasound, shear-wave elastography, infrared thermal imaging, MRI, cone beam computed tomography, and dermatology evaluation tools, etc., all can reflect the patient′s condition to a certain extent.However, systematic evaluation should be improved in the process of diagnosis and treatment.In terms of treatment, hormones and methotrexate are still the first-line treatment for the disease, while evidence for second-line therapy is limited.Mycophenolate, hydroxychloroquine, cyclosporine A, tocilizumab and infliximab may play a certain role in the treatment of this disease.
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Type Ⅰ interferonopathies is a relatively new group of diseases in the last decade, which belong to auto-inflammatory disorders characterized by robust inflammation.Its low incidence and diverse clinical manifestations make it difficult for the clinical identification and diagnosis of this disease.In this article, the concept, pathogenesis, diagnosis and treatment options of this disease was introduced briefly, in order to enhance clinicians′ knowledge of them, thus achieving the goal of early recognition, early detection, early identification and early intervention for the benefit of more patients and their families.
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A 15-year-old female was referred to the hospital with intermittent fever, where multiple systemic abnormalities were found, such as splenomegaly, secondary hypersplenism, retinitis pigmentosa, and ectodermal dysplasia. Medical history revealed that she had suffered recurrent respiratory infections, blurred vision at night, and dysplasia of teeth and nail beds since childhood. Then she was suspected to be experiencing ROSAH syndrome, a rare disease newly recognized in recent years, which was finally confirmed by gene sequencing results. During a course of treatment with tumor necrosis factor inhibitors, recurrent fever with elevated inflammatory markers reappeared, and the child developed headaches. To guide the comprehensive treatment and improve the patient's quality of life, the multidisciplinary team in Peking Union Medical College Hospital discussed together and directed the following treatment.
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Autoinflammatory diseases (AIDs) are a group of genetic disorders characterized by generalized inflammatory responses and multiorgan involvement primarily caused by dysregulated innate immunity. Since the introduction of this concept, AIDs has been a rapidly advancing research field including at least 56 diseases, deepening the understanding of the interaction between innate and adaptive immunity. Despite distinct features displayed by AIDs of different categories, genetic testing remains essential for highly suspected cases. The diagnosis of undifferentiated systemic autoinflammatory diseases, omics-powered precision stratification and targeted therapy for AIDs are promising research areas in the future. This article introduces the rapid progresses in AIDs concept, mechanism, and classification. We present a summary of the characteristic clinical phenotype, as well as the current diagnostic challenges and treatment experiences, in the hope of raising the awareness of these disorders.
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Objective:To establish the detection method for the interferon stimulated genes(ISGs), calculate the cut-off value and test it in clinical practice.Methods:Patients with type I interferonopathies who were admitted to Peking Union Medical College Hospital from November 2017 to September 2021 were chosen as the disease group, and healthy children were included as the control group. A total of 18 children were in the disease group, including 8 males and 10 females, with a median age of 8.5 years for the first test. From them 25 blood specimens were collected. A total of 28 healthy children, aged 1 to 18 years, with a median age of 10.5 years, including 15 males and 13 females, were included in the control group. Blood samples of 34 controls and 18 interferonopathies patients were collected, then total RNA extraction and cDNA synthesis were performed. Real-time quantitative polymerase chain reaction assays were run in duplicate to measure the expression of six ISGs: interferon induced protein with tetratricopeptide repeats 1 (IFIT1), interferon α inducible protein 27 (IFI27), interferon induced protein 44 like (IFI44L), interferon stimulated genes 15 (ISG15), sialic acid binding Ig like lectin 1 (SIGLEC1), and radical S-adenosyl methionine domain containing 2 (RSAD2). The relative abundances of each target transcript was normalized to the expression level of β-Actin and OAZ. The median fold change of the six ISGs was used to create an interferon score (IS) for each individual. Samples with abnormal expressions were removed and the cDNA mix of the remaining samples was used as a calibrator to calculate the IS. We define an abnormal IS as being greater than+2 standard deviations above the mean of controls. Differences in IS between groups were compared using t-test or Mann-Whitney U-test. Results:The mean IS of controls was 1.046, standard 0.755, and the cut-off value was 2.556. A total of 25 samples from 18 interferonopathies patients were tested. The mean value was 27.010 with a 15/18 abnormality rate. Compared with the control group, IS in patients was significantly higher, t=4.247( P=0.000 1). The accuracy, precision, sensitivity, and specificity were 91.30% (42/46), 7.47%(0.084/1.124), 15/18, and 96.43% (27/28), respectively. Conclusion:This study provides a new and reliable method for clinical screening and dynamic monitoring of type Ⅰ interferonopathies by detecting ISGs expression and creating an IS.
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Takayasu arteritis is a a rare, chronic large-vessel vasculitis that predominantly affects aorta, its major branches and the pulmonary arteries; it is the most common, granulomatous inflammation of large arteries in children.It induces a variety of nonspecific inflammatory symptoms and ischemic symptoms due to stenotic lesions.Recent advances in imaging modalities including magnetic resonance angiography, computed tomography(CT), sonography, and fluorodeoxy glucose positron emission tomography/CT(FDG-PET/CT)allow accurate diagnosis of Takayasu arteritis and shorter duration between onset of the disease and diagnosis.Medical treatment for Takayasu arteritis is also changing.In addition to the traditional glucocorticoids and immunosuppressants, many new biological agents such as TNF-α antagonists and tocilizumab are being applied to patients with Takayasu arteritis refractory to conventional treatment with favorable results.This review critically discusses recent advances in medical management of Takayasu arteritis, with a special focus on the rationale and evidence to support the use of biologic agents in this disease.
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There is accumulating evidence to suggest that immunoglobulin E(IgE)plays a significant role in autoimmunity and inflammation in recent years, and the most concerned is the role of IgE in systemic lupus erythematosus(SLE). Multiple studies demonstrated a pathogenic role of autoreactive IgE in SLE, including direct damage on tissue-containing autoantigens, activation and migration of basophils to lymph nodes, and induction of type Ⅰ interferon responses from plasmacytoid dendritic cells(pDCs). It is now recognized that autoimmune diseases such as bullous pemphigoid(BP), chronic spontaneous urticaria(CSU)and hyperthyroid Graves′ disease are partly mediated by IgE.The situations in other conditions with increased IgE levels, such as autoimmune uveitis, multiple sclerosis, as far less clear and some studies have pointed towards IgE autoantibodies as the main culprit.Now anti-human IgE monoclonal antibody is approved for use in asthma and CSU.And autoreactive IgE and FcεRI-bearing effector cells are as new promising therapeutic targets in autoimmune diseases.In this review, the mechanism and clinical effects of IgE mediated inflammation and autoimmune response will be discussed.
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Objective@#To summarize the clinical characteristics of patients with haploinsufficiency of A20 (HA20).@*Methods@#The clinical manifestations, laboratory examinations, treatment, outcome and genetic analysis of 4 cases with HA20 hospitalized in Peking Union Medical College Hospital were analysed.Further literature review was done after searching articles in PubMed and Wangfang databases with the key words "HA20" "A20 haploinsufficiency" "TNFAIP3" up to the date of September 2019.@*Results@#The 4 patients were a father and a daughter, as well as a mother and a daughter. Their phenotypes were quite variable, but all of them have been suffering from recurrent oral ulcer since childhood. Elevation of C-reactive protein (13-33 mg/L) and erythrocyte sedimentation rate (21-60 mm/1h) were found in these 4 patients, and there was positive antinuclear antibody in proband 1.The father in pedigree 1 and the 2 patients in pedigrees 2 have been diagnosed with Behçet disease and the proband 1 with undifferentiated connective tissue disease. The 2 patients in pedigree 1 have developed Hashimoto′s thyroiditis. After gene sequencing analysis, it was found that all the 4 patients have heterozygous nonsense mutations in TNFAIP3 gene, that is, c.811C>T, p.R271X in pedigree 1 and c.133C>T, p.R45X in pedigree 2.The diagnosis of HA20 was eventually established when sequencing results and their clinical manifestations were both compatible with this disease.A total of 21 articles were retrieved, all in English, with a total of 91 cases from 39 families (including the 4 cases reported in this paper). HA20 was reported more often in female (57, 64.8%). Most patients develop symptoms from childhood, but as many as 53.4% (47 cases) are not correctly diagnosed until adulthood. Oral ulcers, genital ulcers, periodic fever, gastrointestinal symptoms, rashes, and arthritis are the primary manifestations.Hashimoto′s thyroiditis is the most common autoimmune diseases that HA20 patients coexist with. Laboratory tests were characterized by significantly elevated inflammatory markers and low to moderate titers of autoantibodies in some patients.Most HA20 patients were reported to have nonsense mutations or shift mutations of TNFAIP3 gene, which leads to truncation of A20 protein, and only a small number of patients have missense mutation. In terms of treatment, anti-TNF treatment and anti-interleukin 1 is believed to be an effective and the most optimal therapy. The treatment effect is variable and requires long term observations.@*Conclusions@#The clinical phenotypes of HA20 are complex. For patients with both autoinflammatory and autoimmune characteristics, family history should be inquired in detail and gene sequencing should be performed if necessary.
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Objective@#To establish comprehensive laboratory reference intervals for Chinese children.@*Methods@#This was a cross-sectional multicenter study. From June 2013 to December 2014, eligible healthy children aged from 6-month to 17-year were enrolled from 20 medical centers with informed consent. They were assessed by physical examination, questionnaire survey and abdominal ultrasound for eligibility. Fasting blood samples were collected and delivered to central laboratory. Measurements of 15 clinical laboratory parameters were performed, including estradiol (E2), testosterone(T), luteinizing hormone(LH), follicle-stimulating hormone(FSH), alanine transaminase(ALT), serum creatinine(Scr), cystatin C, immunoglobulin A(IgA), immunoglobulin G(IgG), immunoglobulin M(IgM), complement (C3, C4), alkaline phosphatase(ALP), uric acid(UA) and creatine kinase(CK). Reference intervals were established according to central 95% confidence intervals for reference population, stratified by age and sex.@*Results@#In total, 2 259 children were enrolled. Finally, 1 648 children were eligible for this study, including 830 boys and 818 girls, at a mean age of 7.4 years. Age- and sex- specific reference intervals have been established for the parameters. Reference intervals of sex hormones increased gradually with age. Concentrations of ALT, cystatin C, ALP and CK were higher in children under 2 years old. Serum levels of sex hormones, creatinine, immunoglobin, CK, ALP and urea increased rapidly in adolescence, with significant sex difference. In addition, reference intervals were variable depending on assay methods. Concentrations of ALT detected by reagents with pyridoxal 5'-phosphate(PLP) were higher than those detected by reagents without PLP. Compared with enzymatic method, Jaffe assay always got higher results of serum creatinine, especially in children younger than 9 years old.@*Conclusion@#This study established age- and sex- specific reference intervals, for 15 clinical laboratory parameters based on defined healthy children.
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Objective@#To analyze the clinical characteristics of spondyloenchondrodysplasia with immune dysregulation (SPENCDI).@*Methods@#The clinical manifestations, laboratory examinations, treatment and genetic analysis of a patient diagnosed with SPENCDI who was admitted to the Department of Pediatrics in Peking Union Medical College Hospital in October 2016 were analyzed. Then literature review was done after searching articles in PubMed and several Chinese databases with the key words "spondyloenchondrodysplasia with immune dysregulation" up to the date of November 2017.@*Results@#A 12-year-old girl was admitted to local hospital for complaint of "recurrent fever over one month" in October 2016. She was diagnosed with type Ⅱ autoimmune hepatitis for abnormal liver function, elevated immunoglobulin G, positive anti-liver-kidney microsomal antibody and medium to severe interface hepatitis verified by liver biopsy. Systemic lupus erythematosus was also suspected based on positive antinuclear antibody and anti-dsDNA antibody, decreased complements, reduced white blood cells and hemoglobin. Methylprednisolone and azathioprine were started based on the diagnosis. However, she experienced mycoplasma pneumoniae and suspected fungal infections during the treatment. Detailed history revealed the history of developmental retardation since birth, and cerebral palsy diagnosed when she was 2 years old. She also underwent surgery at the age of eight for eversion of her right foot. Based on the abnormal findings of immune system, skeleton and nervous system, certain primary immunodeficiency disease was speculated. Gene sequencing was performed, which revealed compound heterozygous mutations in ACP5 gene (NM_001111035.2) (c.798dupC, p. S267Lfs*20, paternal; c.716G>A, p. G239D, maternal). With X-ray of the vertebrae showed multiple platyspondyly, the diagnosis was corrected as SPENCDI and type Ⅱ autoimmune hepatitis. Then she was treated with prednisone (60 mg/d) and mycophenolate mofetil (1.5 g/d). All symptoms resolved on 3-month follow-up, with normalized activity indexes of autoimmune hepatitis and systemic lupus erythematosus. A total of 25 articles (1 Chinese, 24 English) were reviewed, with 74 SPENCDI patients reported. The most common manifestations were skeletal abnormalities (74/74, 100%), autoimmune diseases (47/74, 63.5%), dwarfism (45/74, 60.8%), and nervous system symptoms (25/74, 33.8%). A few patients with simple spondyloenchondrodysplasia were treated with growth hormone, and those who with autoimmune diseases were treated with immunosuppressants, all of whom were improved to certain extent.@*Conclusions@#Vertebral and metaphyseal dysplasia, nervous system symptoms, and strong predisposition to autoimmune diseases are the hallmarks of SPENCDI. SPENCDI should be considered in dwarf with or without autoimmune diseases or nervous system symptoms.
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The clinical manifestations,laboratory test results,treatments and prognoses of 5 adult patients with hyperimmunoglobulin E syndrome (HIES) were retrospectively analysed.All 5 patients experienced disease onset within 1 year of birth and had recurrent multiple infections,all had recurrent rash and 4 had skeletal abnormalities.Recurrent infections lasted for a mean of 19.6 years and predominantly affected the skin and respiratory tract.Other manifestations included chronic otitis media,renal abscess and osteomyelitis.Two cases had skin abscess (methicillin-susceptible Staphylococcus aureus infection),which was alleviated after antibiotic treatment and abscess drainage.Two cases had respiratory tract infection and one case had a foot skin ulcer,and all symptoms improved after antibiotic treatment.HIES should be suspected when an adult patient experiences repeated infection since birth,particularly when skeletal abnormalities and a history of neonatal rash is also present.Early diagnosis facilitates targeted anti-infection therapy,leading to earlier remission and an improved prognosis.
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Objective To assess the clinical value of percutaneous bone cement fusion in treating stress fracture of vertebral body that is adjacent to pseudoarthrosis in patients with ankylosing spondylitis.Methods The clinical data of 4 ankylosing spondylitis patients with stress fracture of vertebral body adjacent to pseudoarthrosis,which was treated with percutaneous bone cement fusion,were retrospectively analyzed.Bone cement fusion through injection of bone cement was performed for 4 vertebral segments.Visual analogue scale (VAS) of pain and Oswesty disability index (ODI) were determined before and after operation,the results were compared,and the improvements of pain and daily activity were evaluated.Results The operation was successfully accomplished in all the 4 patients.The mean used amount of bone cement for each vertebral segment was 14.5 ml.Small amount of bone cement extravasation was observed in one patient,but no severe clinical complication occurred.The mean VAS score decreased from preoperative 9 points to postoperative 3.5 points;ODI score decreased from preoperative 43.8 points to postoperative 14.5 points.After the treatment,the pain was obviously relieved and the daily activity was markedly improved.Conclusion For the treatment of stress fracture of vertebral body that is adjacent to pseudoarthrosis in patients with ankylosing spondylitis,percutaneous bone cement fusion is minimally-invasive,safe and effective.
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Objective@#To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism.@*Method@#Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism.@*Result@#Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them.Eight cases were misdiagnosed as infection, while 3 cases were missed.Among patients with pulmonary embolism, 10 patients suffered from deep vein thrombosis at the same time, mainly in lower extremities.2 cases had thrombotic recurrence, which happened only in primary APS patients, because of irregular monitoring of International Normalized Ratio, or not taking aspirin after quitting warfarin.Positive anticardiolipin (ACL) and lupus anticoagulant (LA) were found in 10 and 9 patients respectively.Four primary APS patients had positive anti-nuclear antibodies (ANA). During follow-up of 3-100 months (median 23 months) of primary APS, no one had evolved manifestations of systemic lupus erythematosus.Primary APS was more often seen in males (M∶F 5∶1 vs. 0∶6) and the patients were much younger ((15±1) vs. (17±0) years old) than those with secondary APS.Besides that, no statistically significant difference was seen between primary and secondary APS (P all>0.05). Compared with APS patients without pulmonary embolism, pulmonary hypertension was more common in patients suffered from pulmonary embolism (3/12 vs. 0, P<0.05).@*Conclusion@#Pulmonary embolism can be the first symptom in pediatric APS patients and all of them are non-shock type, which tends to be misdiagnosed or missed. A majority of them suffer from deep vein thrombosis in the lower extremities.Rethrombosis takes place when the anticoagulant therapy is irregular.Positive anti-nuclear antibodies can be seen in primary APS patients, but no manifestations of lupus come out during follow-up.There is no significant difference between primary APS and secondary APS.Pulmonary hypertension is more common in APS patients suffered from pulmonary embolism.
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Objective@#To explore the clinical and immunological features, gene mutations, treatment and prognosis in patients with activated phosphoinositide 3-kinase δ syndrome (APDS) caused by PIK3CD gene heterozygous germline mutation.@*Method@#The data of clinical, immunological phenotype, treatment, and prognosis of 15 patients with APDS, who visited Children′s Hospital of Chongqing Medical University, Peking Union Medical College Hospital, and Shenzhen Children′s Hospital from June 2014 to November 2016, were collected and analyzed.@*Result@#Of the 15 patients, 11 were males, remaining 4 patients were females. The median age of disease onset was 1 year, and median age at diagnosis was 4 years and 4 months. All patients had the de novo heterozygous germline mutation in PIK3CD (c. 3061G>A, p. E1021K). The common initial symptoms were respiratory infections, including pneumonia (12 cases) , bronchiectasis (5 cases). Other common clinical manifestations were recurrent and chronic diarrhea (11 cases), Epstein-Barr virus (EBV) and/or cytomegalovirus (CMV) viremia (10 cases), hepatosplenomegaly (13 cases), and lymphadenopathy (10 cases). The main immunological features were increased IgM (11 cases), decreased IgG (6 cases), decreased numbers of CD4+ T cell (7 cases) especially naïve CD4+ T cell (9 cases), reduced numbers of B cells (11 cases) particularly naïve B cells (9 cases), increased numbers of transitional B cells (5 cases) and CD8+ terminally differentiated effector memory T cells (5 cases). After 1-29 months follow up, 13 of the 15 cases remain survived, of whom 5 cases received regular intravenous immunoglobulin (IVIG) therapy, with reduced frequency of infections and improved severity of infections; of whom 3 cases received oral rapamycin therapy at the dosage of 1 mg/ (m2·d) and with a decrease in nonneoplastic lymphoproliferation.@*Conclusion@#E1021K is a hotspot for mutation in the PIK3CD gene in patients with APDS. Regular IVIG can improve their quality of life. Targetel treatment with rapamycin could mitigate hepatosplenomegaly.
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Objective@#To explore the key points of diagnosis and treatment of familial Mediterranean fever(FMF).@*Method@#The clinical data of 3 cases with FMF misdiagnosed as Juvenile idiopathic arthritis(JIA)seen from January 2014 to June 2016 in Peking Union Medical College Hospital were retrospectively collected. The clinical manifestations, gene mutation characteristics, treatment and prognosis were also evaluated.@*Result@#Two cases were male and 1 was female. The mean age of onset was 17 months (3 months to 36 months), while the average age of diagnosis was 6 years and 8 months (24 months to 11 years). All the 3 cases presented with periodic fever, red rash and arthritis.Two of them suffered from anemia, 2 of them showed lymphadenopathy, and 1 of them presented with hepatosplenomegaly. All of the 3 cases were diagnosed as JIA by excluding infectious diseases and neoplastic diseases and respondiug poorly to anti-infection treatment, but they benefitted little from glucocorticoids and a variety of immunosuppressive therapy. The mutations of MEFV gene were found in 3 cases by gene detection, and all of them were complex heterozygous mutations. Four reported pathogenic mutations were found: R202Q, E148Q, L110P, P369S. All the 3 cases are currently receiving oral colchicine (in accordance with the initial dose of children under the age of 5 recommended ≤ 0.5 mg/d, 5 to 10 years old children 0.5-1.0 mg/d, 10 years old children and older children 1.0-1.5 mg / d) , and the symptoms were significantly improved.@*Conclusion@#The familial Mediterranean fever can be characterized by repeated remittent fever, red rash, arthritis, and is easy to be confused with JIA in clinical manifestation.In this paper, 3 cases were diagnosed as complex heterozygous MEFV gene mutation by gene analysis.During the 6 months follow-up, all of the 3 patients responded well to colchicine.
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Objective To analyze the clinical features of juvenile-onset clinically amyopathic dermatomyositis complicated by progressive interstitial pneumonia.Methods A retrospective analysis of a case of juvenile-onset clinically amyopathic dermatomyositis on clinical features,diagnosis and treatment was performed.Data of the other three reported cases were also reviewed.Results The patient was an adolescent girl presented with Gorrton's sign.The patient did not have fatigue and got normal result in creatine kinase and elctromyogram test.The HRCT exam showed interstitial pneumonia.The mean age of the four cases at the time of onset is 12.3 years old.Gottron's sign (3/4) and fever (2/4) are the most common symptoms of onset.Anti-nuclear antibody (ANA),anti-Jo-1 antibody are 100％ negative in the four patients.Two of the four patients who received anti-Ro-52 antibody test are both positive.Three of the four patients were asymptomatic when the CT scan showed interstitial pneumonia.The interstitial pneumonia was progressive and three of the four patients died of respiratory failure within six months.Treatment with glucocorticoid and immunosuppressant was successful in one case.Conclusions Juvenile CADM can be complicated by progressive interstitial pneumonia.Children suspected CADM should perform pulmonary imaging examinations to find interstitial pneumonia.Children diagnosed as CADM complicated by interstitial pneumonia should receive glucocorticoid and immunosuppressant treatment to prevent progression.
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Familial mediterranean fever (FMF) is a hereditary inflammatory disorder,which caused by mutations in MEFV gene located on short arm of Chromosome 16 p13.3.The majority of patients onset before the age of 10 years,which is characterized clinically by the episodes of inflammatory reaction and serositis,including fever,peritonitis,synovitis and pleurisy.AA-amyloidosis with kidney failure is the most important complication.The children patients were diagnosed mainly on clinical evaluation and the criteria developed by Yal(c)inkaya.The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimise subclinical inflammation in between attacks.The only agent that decreases the frequency and severity of the episodes and the development of amyloidosis is colchicine,which should be startcd as soon as a clinical diagnosis is made,alternative biological treatments such as IL-1 receptor antagonist are indicated in patients with non-responders or resistant to colchicine.
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Objective To evaluate the characteristics of the PREVI Isola automated plate streaker (bioMérieux,SA).Methods 80 respiratory tract specimens,70 sterile fluids,52 stools,69 swabs,12 cerebrospinal fluids and 80 urines were collected in Peking Union Medicd College Hospital.Specimens were processed with manual streaking and PREVI Isola system.PREVI Isola system were evaluated comparing to the manual streaking.The quality of results were analyzed by SPSS 16.0, doing Wilcoxon’s Sign Rank Test for the results of finally isolated species,overall numbers of isolated colonies and semi-quantita-tive of the species were both isolated by the two methods.Results PREVI Isola system was highly automatic,which could select the right plates and stake the bar code on the back of the plate indicating the type of the agar and inoculation time, PREVI Isola system could accurately absorb the liquid specimen and use a novel comb streaking procedure for processing of fluid specimens on standard agar plates,like 17 inoculating loops work together.It also had a good reproducibility.The quali-ty of PREVI Isola system results:As to the finally isolated species,there was significant statistical difference between PREVI Isola system and manual streaking method in respiratory tract and stools specimen,there were more species isolated by manual streaking method than PREVI Isola system.There were no differences between the two methods for the other of specimen types.As to the amount of pure clones of the species were both isolated by the two methods,there were significant statistical differences between the two methods for respiratory tract,sterile fluid and stool specimens.The amount of clones isolated by PREVI Isola system was more than manual streaking method.In semi-quantitative results,there were significant statistical differences between the two methods for respiratory tract and urine specimen,Species had wider distribution of PREVI Isola system than manual streaking method.Inoculation efficiency:if the batch of specimen type was simple (mainly the urine and so on),using the same plates,PREVI Isola system was more efficient than manual streaking method.Howev-er,if the batch of specimen type was complicated,manual method was high-performance.Besides,not all specimen type could be inoculated by PREVI Isola system,such as cerebrospinal fluid,catheter and tissues.Conclusion If the lab had simple specimen type,or utilize the specimen type using the same agar plates to be inoculated together,PREVI Isola system belongs to a good performance automated plate streaker.
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Objective To investigate the antimicrobial resistance proifle in the clinical bacterial strains isolated from Peking Union Medical College Hospital during 2014.Methods A total of 8 295 nonduplicate clinical isolates were collected. Disc diffusion test (Kirby-Bauer method) and automated systems were employed to study the antimicrobial susceptibility. The data were analyzed by using WHONET 5.6 software according to CLSI 2014 breakpoints.Results Of the 8 295 isolates, 67.4% were gram-negative, and 32.6% were gram-positive. The top 10 most frequently isolated bacteria were:E. coli(18.1%),P. aeruginosa (10.8%),K. pneumoniae (10.2%),S. aureus (9.8%), A. baumannii(9.2%),E. faecalis (6.3%),E. faecium (4.1%), coagulase-negativeStaphylococcus (4.1%),E. cloacae (3.1%) andS. maltophilia (2.9%). Methicillin resistant strains inS. aureus (MRSA) and coagulase negativeStaphylococcus (MRCNS) accounted for average of 28.4% and 66.5%, respectively. The resistance rates of MR strains to β-lactams and other antimicrobial agents were much higher than those MS strains. Overall, 81.3% of MRSA strains were still susceptible to trimethoprim-sulfamethoxazole, while 81.1% of MRCNS strains were susceptible to rifampin. No staphylococcal strains were resistant to vancomycin, teicoplanin or linezolid. The resistance rate ofE. faecalis strains to most of the drugs tested (except chloramphenicol) was much lower than those ofE. faecium. Several strains of bothE. faecium andE. faecalis were found resistant to vancomycin and teicoplanin, which were Van-A and Van-B types based on their phenotype. No linezolid resistant enterococcal strains were found. Data showed that 90.8% ofβ-hemolyticStreptococcus strains were susceptible to penicillin. ESBLs-producing strains accounted for 54.2%, 31.0% and 28.9% inE. coli,Klebsiella spp (K. pneumoniae andK. oxytoca) andP. mirabilis, respectively.Enterobacteriaceae isolates were still highly susceptible to carbapenems. Overall, no more than 3.3% of these strains were resistant to carbapenems. A few extensively drug-resistant strains ofK. pneumoniae (1.3%, 11/842) were identiifed. The resistance rates ofP. aeruginosa to imipenem and meropenem were 17.5% and 11.8%, respectively.P. aeruginosa isolates showed the lowest resistance rate (5.9%) to amikacin. And 69.0% and 67.4% ofA. baumanniiisolates were resistant to imipenem and meropenem.A. baumannii isolates showed the lowest resistance rates to cefoperazone-sulbactam and minocycline (47.8% and 28.7%), respectively. The prevalence of extensively drug-resistant strains was 32.3% inA. baumannii and 1.8% inP. aeruginosa. The prevalence of β-lactamase inH. inlfuenzae was 33.7%. More than 93.0% ofS. pneumoniae strains were resistant to erythromycin and clindamycin.Conelusions Bacterial resistance is still increasing in this hospital, especially carbapenem resistantEnterobacteriaceae. It is necessary to take effective hospital infection control measures and use antibiotics rationally.
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Fat hypertrophy, intravascular coagulation, and fat emboli are important risk factors of steroid-induced ischemic bone necrosis [SI-IBN] which may develop during the initial one year after commencing the use of steroids. This pathology is best studied by MRI, particularly for its staging. The cautious strategies such as low dose, oral route, short duration of steroid usage, use of steroid sparing agent, and alcohol avoidance should be followed as a traditional therapy. The objective of this review article was to recognize and evaluate various treatment strategies for steroid-induced ischemic bone necrosis of femoral head. Various electronic databases including PubMed, Google and Cochrane library were comprehensively searched for articles on steroid-induced ischemic bone necrosis of femoral head and its treatment strategies. Ninety four articles were reviewed, examined and importantly appraised and the most appropriate 32 papers were used to write this review article. Bisphosphonates, alendronate, and hyperbaric oxygen [HBO] treatments have been reported to be effective against IBN. To recommend the regular use of bisphosphonate in IBN patients, more evidences with a larger number of patients are required to verify its therapeutic effectiveness. Core decompression, osteotomy, bone graft and tantalum rod are the surgical approaches for the management of IBN. Advance form of IBN [bone tissue collapse] is advised to be treated with arthroplasty which should be durable, particularly in young patients