Child ; Diagnosis, Differential ; Humans ; Lung Diseases ; diagnosis ; Tuberculosis, Pulmonary ; diagnosis

BCG Vaccine ; adverse effects ; Child ; Child, Preschool ; Genetic Diseases, X-Linked ; complications ; Granulomatous Disease, Chronic ; complications ; genetics ; Humans ; Infant ; Infant, Newborn ; Lymphadenitis ; etiology ; Male ; Membrane Glycoproteins ; genetics ; NADPH Oxidase 2 ; NADPH Oxidases ; genetics

Child ; Enterovirus A, Human ; Enterovirus Infections ; Humans

2 g/L can′t excluded SCID.

Bronchial Diseases ; diagnosis ; diagnostic imaging ; physiopathology ; therapy ; Bronchioles ; pathology ; physiopathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Male ; Respiratory Function Tests ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

Child ; Chronic Disease ; Cough ; diagnosis ; etiology ; therapy ; Humans ; Practice Guidelines as Topic

Objective To study the effects of sulodexide on renal NF-κB activation and monocyte chemotactic protein 1 (MCP-1) expression in diabetic rats and elucidate the possible mechanism of sulodexide in preventing diabetic nephropathy (DN). Methods Wistar rats were fed with high-sucrose-high-fat diet and injected with a low dose of STZ (streptozotocin,35 mg/kg) into abdominal cavity to induce diabetes.DM rats were randomly divided into non-treated group of treatment,blood glucose (BG),triglyceride (TG),cholesterol,serum creatinine (Scr),urea nitrogen (BUN),24 h urinary albumin excretion (UAE) were measured.HE staining was performed in renal tissues for light microscopy examination of mean glomerular volume (MGV).MCP-1 expression was detected by immunohistochemical method.NF-κB activation was determined by Western blot. Results Compared with NC group,DM group and DMS group had significant elevated BG,TG and TC levels (all P＜0.01).There were no significant differences of BG,TG or TC levels between DM group and DMS group.Compared with NC group,DM group and DMS group had significant increased Scr,BUN,UAE levels (all P＜0.01).Scr,BUN,UAE levels were significantly lower in DMS group than those in DM group [(39.1±0.88) μmol/L vs (41.0±2.16) μmol/L,(9.12±1.06) mmol/L vs (9.87±0.19) mmol/L； (19.92±0.96) mg/24 h vs (25.99±0.52)mg/24 h,all P＜0.05].Compared with NC group,the MGV of DM group was significantly increased [(7.47±1.11)×105 μm3 vs (4.22±1.09)×105 μm3,P＜0.01].Compared with DM group,the MGA of DMS group was significantly reduced [(6.64±0.71)×105 μm3 vs (7.47±1.11)×105 μm3,P＜0.05],but was still increased compared with that of NC group (P＜0.01).Compared with NC group,the MCP-1 expression of DM group was significantly higher [(12.17±1.94)/HPF vs (1.19±0.70)/HPF,P＜0.01].MCP-1 expression in DSM group was significantly lower than that of DM group [(9.22± 1.61)/HPF vs (12.17±1.94)/HPF,P＜0.01],but still higher than that of control group (P＜0.01).Compared with NC group,the NF-κB activity was significantly higher in DM group [(0.89±0.07) vs (0.24±0.03),P＜0.01].Compared with DM group,NF-κB activity of DMS group was significantly lower [(0.27±0.01) vs (0.89±0.07),P＜0.01].There was no significant difference of NF-κB activity between DMS group and NC group. Conclusion Sulodexide has protective effects on diabetic nephropathy,and one of the mechanisms may involve the inhibition of NF-κB activation as well as the suppression of MCP-I expression.

Objective To explore the application of amide proton transfer (APT) imaging in differentiating glioma from treatment effect and to evaluate the diagnostic efficiency of the quantitative APT-related parameters.Methods A total of 23 patients (15 males, 8 females, age: 13-80 years) with 27 lesions who had underwent APT imaging in Tongji Hospital(Wuhan, China) from October 2014 to June 2015 were enrolled in this prospective study.The scan protocols were MRI normal plain scanning, diffusion WI, contrast-enhancement T1WI and APT imaging.Both the magnetization transfer ratio (MTR) and the relative MTR (rMTR) of lesions were manually measured by drawing ROI in the functional post-processing workstation.The results were compared with those of pathologic examinations and radiographic follow-up (≥3 months).Mann-Whitney u test was used to analyze the data.Results Compared with contralateral white matter, the primary gliomas (n=12) and recurrent gliomas (n=8) manifested hyper-intensity, while the treatment induced injuries (n=7) showed iso-or hypo-intensity.The difference of MTR between tumors and treatment effects was significant (102.78(101.93,103.84) vs 100.17(99.94, 100.63);z=-3.76, P<0.01), so was the difference of rMTR between tumors and treatment effects (3.92%(2.69%,4.67%) vs 0.47%(-0.79%,1.11%);z=-3.43, P<0.01).Both those two quantitative parameters exhibited excellent diagnostic performance with the AUC of 0.986 and 0.943.The sensitivity, specificity and accuracy of MTR were 100%(20/20), 6/7 and 96.3%(26/27) in the threshold of 100.68, while those of rMTR were 95.0%(19/20), 6/7 and 92.6%(25/27) in the threshold of 1.66%.Conclusions Combined with the routine MRI images, APT imaging can provide excellent qualitative and quantitative information in differentiating glioma from treatment effect.Both MTR and rMTR are helpful for the differentiation with high sensitivity and specificity and can be used as non-invasive imaging biomarkers in evaluating treatment effect of glioma.

OBJECTIVELeukocyte adhesion deficiency type 1 (LAD-I) is rare. We present 1 case of LAD-I patient diagnosed by gene analysis. His clinical manifestations and genetic mutation features are analyzed in this article.

METHODThe clinical material of the LAD-I patient who was diagnosed by gene analysis was retrospectively analyzed.

RESULTThe patient was a 2-month-old boy. He had a complaint of recurrent fever and cough for 30 days. Pulmonary CT indicated a small to moderate quantity pleural effusion on the right side. His peripheral blood leukocyte and C-reactive protein (CRP) was always significantly higher than normal. After hospitalization he had diarrheal diseases, routine stool test showed 2 RBC cells/high power (HP), WBC 30 cells/HP, stool cultures were negative, digestive tract ultrasonography showed an array of defects, in the sigmoid colon and rectal mucosa suggestive of ulcerative colitis. He was treated with cefoperazone and sulbactam and vancomycin. He had a history of impetigo in his neonatal period and without delayed umbilical cord exfoliation. His family history was normal. ITGB2 genetic mutation analysis revealed a homozygous mutation (1062A > T). His parents did not participate in this study. He had no fever but had diarrheal disease after 1 month of follow up.

CONCLUSIONThis patient had suffered from impetigo, pleural effusion, diarrheal diseases, markedly increased peripheral white blood cell and ITGB2 genetic mutation analysis showed that homozygous mutation (1062A > T). He received a diagnosis of LAD-I.

Asian Continental Ancestry Group ; Colitis, Ulcerative ; diagnosis ; etiology ; Cytoskeletal Proteins ; genetics ; DNA Mutational Analysis ; Flow Cytometry ; Homozygote ; Humans ; Infant ; Leukocyte Count ; Leukocyte-Adhesion Deficiency Syndrome ; complications ; diagnosis ; genetics ; Male ; Muscle Proteins ; genetics ; Pleural Effusion ; diagnosis ; etiology ; Point Mutation ; genetics ; Polymerase Chain Reaction ; Retrospective Studies