Acetates ; therapeutic use ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperplasia ; Infant ; Lung ; diagnostic imaging ; pathology ; physiopathology ; Lung Diseases, Interstitial ; diagnosis ; drug therapy ; physiopathology ; Methylprednisolone ; therapeutic use ; Neuroendocrine Cells ; pathology ; Quinolines ; therapeutic use ; Tomography, X-Ray Computed

OBJECTIVETo investigate bronchial responsiveness to acetylcholine in allergic airway inflammation of SD rats.

METHODSSD rats were immunized and challenged by chicken ovalbumin (OVA). Airway responsiveness, acetylcholine (Ach) provocation concentration needed to increase baseline airway resistance by 200% (PC(200)) were measured.

RESULTSThe value of baseline airway resistance in asthma group was significantly higher than that in control group (2.282 +/- 0.128 vs 3.193 +/- 0.239; P < 0.01). After multiple ovalbumin exposures, airway responsiveness to intravenous injection of acetylcholine decreased significantly (-LogPC(200): 4.006 +/- 0.554 vs 2.059 +/- 0.262; P < 0.01). Bronchial alveolar lavage fluid (BALF) and lung tissue specimen analysis indicated that airway allergic inflammation was present.

CONCLUSIONSThe study demonstrates a dissociation between the bronchoconstrictor response and bronchial hyper-responsiveness and indicates that multiple ovalbumin exposures induces persistent bronchoconstriction with airway hypo-responsiveness despite airway allergic inflammation.

Acetylcholine ; pharmacology ; Airway Resistance ; Allergens ; immunology ; Animals ; Bronchi ; drug effects ; physiology ; Bronchial Hyperreactivity ; etiology ; Bronchoconstriction ; Lung ; pathology ; Male ; Ovalbumin ; immunology ; Rats ; Rats, Sprague-Dawley

Objective To explore the clinical features of chronic granulomatous diseases and Mcleod syndrome caused by continuous X chromosome deletion. Methods The clinical data of two children diagnosed as chronic granulomatous disease and Mcleod syndrome by gene detection were retrospectively analyzed. Results Two males, 4 year 1 month and 1 year 9 month old, were both hospitalized due to persistent pulmonary infections. Both of them had a history of repeated severe infections and BCG vaccine associated lymphadenitis, and were diagnosed as X-linked chronic granulomatous disease for respiratory burst defects and deletion of all CYBB exons. Both of them had retarded motor development, and were diagnosed as DMD for detection of DMD gene exons and muscle speciifc promoter region and exon 1-2 deletion by MLPA. One case was found with obvious echinocytes, the other case showed whole exons deletion of XK gene. Both of them were diagnosed as Mcleod syndrome. Conclusion Continuous X chromosome deletion could lead to combination of Mcleod syndrome, DMD, and X-CGD, which may complicate the condition. Due to the lack of Kx antigen, repeated common blood transfusion can produce relative antibody, which lead to severe hemolytic crisis.

Objective To obtain information on the distribution of serotypes and antimicrobial agent susceptibilities to group B streptococcus (GBS) strains isolated in Beijing area from 1991 to 1996. Methods Bacterial isolates of GBS were obtained from vaginal and cervical tract of pregnant and nonpregnant women in Beijing Tian Tan Hospital by culture. A total of 76 GBS strains were identified finally by coagglutination. Serotyping was determined by Standard Lancefield method. Susceptibility to test agents was assessed by determining the minimal inhibitory concentrations (MICs) with agar dilution method that was established by the National Committee for Clinical Laboratory Standards (NCCLS).Results Seven serotypes were identified among 76 GBS strains isolates. Types Ⅱ (33%), Ⅲ (23%) and Ⅰa (16%) were the predominant serotypes in pregnant and nonpregnant women. MICs of penicillin G and ampicillin were ≤0.06 μg/ml. MICs of cephazolin, cefuroxime and cefoperozone were 0.003 μg/ml - 0.06 μg/ml. MICs of erythromycin were 0.003 μg/ml - 0.03 μg/ml. MICs of gentamycin were 1 μg/ml - 32 μg/ml. MICs of amikacin were 4 μg/ml - ≥64 μg/ml, nearly 12.8% and 40.4% of the strains were resistant to gentamycin and amikacin, respectively.Conclusions Our study provides useful epidemiologic data for preparation of GBS type-specific vaccines which can prevent GBS infections and antimicrobial agents susceptibility patterns in China. Routine reports on GBS susceptibilities by clinical laboratories and continuous surveillance for changes in the susceptibility are of considerable clinical importance.

Objective To investigate the prevalence and features of active human cytomegalovirus (HCMV) infection in children with systemic lupus erythematosus (SLE) and evaluate the diagnostic value of the HCMV using antigenemia assay, serum polymerase chain reaction (PCR) and serology test. Methods Twenty-one SLE children undergoing immunosuppressive therapy were enrolled in this study. Immunofluorescence assay, PCR and serology tests were used to determine HCMV pp65 and p72 antigens in leukocytes, HCMV DNA in sera, and HCMV specific IgM and IgG antibodies, respectively. As a control group, twenty-one immunocompetent children with skeletal malformation were involved in this study. Statistical analysis was performed using Chi-square test or Fisher's exact test (Systat, USA), P values less than 0.05 were considered significant.Results Active HCMV infection was diagnosed in 28.6% (6/21) of SLE patients, with none in the control group; the difference between the two groups was significant (P=0.027). Two out of 6 SLE patients developed active HCMV infection before immunosuppressive therapy and the remaining 4 patients developed SLE after immunosuppressive therapy. Among the 21 SLE children, HCMV pp65 antigenemia was detected in 5 patients, p72 antigenemia in 3 patients, serum HCMV DNA in 9 patients, serum HCMV-specific IgM in 2 patients, and IgG in 19 patients. The sensitivity and specificity for diagnosis of active HCMV infection were 83.3% and 100%, respectively for pp65 antigenemia; 50% and 100% for p72 antigenemia; 100% and 80% for serum PCR; 33.3% and 100% for HCMV IgM serology; 50% and 100% for HCMV IgG serology. Conclusions Compared with the control group, active HCMV infection is much more frequent in SLE children, and can occur before treatment with immunosuppressive agents, but most often occur after immunosuppressive therapy. In comparison with the other techniques used in this study, the pp65 antigenemia assay seems to be a better method for the early diagnosis and monitoring of active HCMV infection in children with SLE.

Objective To explore the diagnosis and treatment of atypical severe combined immunodeficiency disease (SCID). Methods The clinical data of atypical SCID in 7 children with IL2RG,JAK3,and RAG1 mutations were reviewed and analyzed from September 2012 to June 2017. Results In 7 cases (6 males and 1 female), there were 5 infants, 1 toddler and 1 school-age child. Cases 2, 4, and 6 were classic SCID clinical phenotypes. Cases 1, 3, 5, 7 were atypical SCID clinical phenotypes. Case 6 were diagnosed with Omenn syndrome. Cases 2, 5 were classic SCID immune phenotypes, cases 1, 3, 4, 6, 7 were atypical SCID immune phenotypes, and case 1 had maternal chimera. The next generation sequencing indicated that case 1 had a compound heterozygous JAK3 mutation with c.3097-1G>A/c.946-950GCGGA>ACinsGGT.Cases 2,3,and 4 had IL2RG mutations,with c.865C>T/p.R289X,c.664C>T/R222C,52delG,respectively.Case 5 had JAK3 mutations with c.2150A>G/p.E717G and c.1915-2A>G.Sanger sequencing indicated that case 6 had a RAG1 mutation of complex heterozygosity with c.994C>T/p.R332X and c.1439G>A/p.S480N. Case 7 had homozygous RAG1 mutation with c.2095C>T/p.R699W.Conclusion Under certain conditions,gene mutation can lead to atypical clinical and/or immune phenotypic SCID.