Adolescent ; Diagnosis, Differential ; Female ; Humans ; Myositis Ossificans ; diagnosis

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by germline mutations of B-cell tyrosine kinase (BTK) gene. It is characterized by decreased serum immunoglobulins levels and circulating mature B cells. This defect in humoral immunity leads to increased susceptibility to infection. Pyoderma gangrenosum (PG) is an uncommon, ulcerating, neutrophilic dermatosis. Here we report PG in an 8-year-old patient with XLA. The patient received intravenous immunoglobulin treatment in conjunction with prednisone and topical application of 0.03% tacrolimus ointment and the ulcer was almost completely healed in the 2 weeks of follow-up. The coexistence has been rarely reported. XLA may be a possible cofactor in the pathogenesis of PG.
Agammaglobulinemia* ; B-Lymphocytes ; Child ; Follow-Up Studies ; Germ-Line Mutation ; Humans ; Immunity, Humoral ; Immunoglobulins ; Neutrophils ; Prednisone ; Protein-Tyrosine Kinases ; Pyoderma Gangrenosum* ; Pyoderma* ; Skin Diseases ; Tacrolimus ; Ulcer ; X Chromosome

To investigate the upregulated genes associated with cold acclimation, a cold acclimation model was established based on Balb/C mouse. mRNA of muscle and liver were isolated, and the upregulated genes of these tissues were studied by representational differential analysis (RDA). The upregulated genes then were sequenced and searched by Blast software in GenBank database. The results showed that some genes were upregulated and possibly associated with cold acclimation. Three of these genes, transferrin, fibrinogen B-beta-chains and a new gene fragment (Genbank ID: AF454762), were confirmed to be upregulated by RNA slot-blot analysis. The finding of these genes might contribute to further understanding of the molecular mechanisms of cold acclimation.
Acclimatization ; genetics ; Animals ; Cold Temperature ; Gene Expression ; Liver ; metabolism ; Male ; Mice ; Mice, Inbred BALB C ; Muscle, Skeletal ; metabolism ; Transcriptome ; Up-Regulation

0.05).Three days after interventional therapy,the values of BF,BV,MTT,PS,MVD and VEGF of VX_2 tumors in interventional group were (7.5?2.4)ml? 100g~(-1)?min~(-1),(1.20?0.23)ml/100g,(3.29?0.57)s,(4.0?1.5)ml?100g~(-1)?min~(-1), 16.0?2.4/HP and 0.215?0.008 respectively.Compared with the values of pre-interventional therapy and the control group,there were significant differences among them(P0.7,P0.05)but had a significant negative correlation with average A value of VEGF(r=-0.78,P

OBJECTIVETo observe blood uric acid levels and Goldstein grading, as well as their correlation in Wilson's disease (WD) patients with different Chinese medical syndrome types.

METHODSTotally 906 WD patients in line with inclusive criteria were assigned to 6 groups, i.e., the heart spirit confused by phlegm group (HSCP, 26 cases), the phlegm-fire disturbing heart group (PFDH, 90 cases), the retention of damp-heat group (RDH, 113 cases), deficiency of qi and blood group (DQB, 168 cases), the deficiency of Gan-yin and Shen-yin group (DGYSY, 327 cases), the deficiency of Gan and Shen group (DGS, 182 cases) due to different Chinese medical syndrome types. Recruited were another 160 healthy subjects having similar ages and diet structures, who came for medical examinations, as the healthy control group. Venous blood was collected from the medial cubital vein of each-patient on an empty stomach in early mornings to detect blood uric acid levels. Results Blood uric acid levels were lower in each syndrome type group than in the healthy control group (146.08 +/- 67.24 micromol/L in the HSCP group; 157.08 +/- 69.77 micromol/L in the PFDH group; 162.58 +/- 97.72 micromol/L in the RDH group; 156.20 +/- 62.63 micromol/L in the DQB group; 161.83 +/- 111.23 micromol/L in the DGYSY group; 194.41 +/- 90.01 micromol/L in the DGS group; 242.39 +/- 87.55 micromol/L in the healthy control group, P < 0.01). Blood uric acid levels were higher in the DGYSY group than in the other 5 syndrome groups (P < 0.01). Correlation analyses between Goldstein grading and blood uric acid showed that, along with increased Goldstein grade (that was aggravating disease conditions), WD patients' blood uric acid levels decreased (P < 0.01).

CONCLUSIONSWD patient's blood uric acid levels decreased more. Blood uric acid levels and Goldstein grading were different in various Chinese medical syndrome types. Blood uric acid levels had certain value in assessing the severity of WD.

Asian Continental Ancestry Group ; Heart ; Hepatolenticular Degeneration ; blood ; classification ; diagnosis ; Humans ; Medicine, Chinese Traditional ; Syndrome ; Uric Acid ; blood

0.05).Seven cases in LDH group and 9 cases in HID group were found in FT picture.The mean DCavg value in annulus fibrosus disruption was significantly larger (1.01?0.10)?10~(-9)mm~2/s and the mean FA value(0.15?0.03)was significantly smaller than those in normal place(P

Humans ; Magnetic Resonance Imaging ; Shoulder ; anatomy & histology

OBJECTIVETo study the pathologic features, differential diagnosis and role of open lung biopsies (OLB) in usual interstitial pneumonia (UIP).

METHODThe authors reviewed the pathologic, clinical and radiologic features of five cases of UIP (one autopsy case and four OLB cases), with follow-up information.

RESULTSThe typical histologic features were a non-uniform distribution of alveolar inflammation, fibroblastic foci, interstitial fibrosis and honeycomb change. There also was associated metaplasia of bronchiolar epithelium, type II pneumocyte hyperplasia and accumulation of alveolar macrophages.

CONCLUSIONSCharacteristically, UIP exhibits temporal heterogeneity under low-power light microscopy, which includes changes in both the early and end stages. Open lung biopsy is an important diagnostic adjunct for suitable patients with atypical radiologic features on computerized tomography. Correlation between clinical, radiologic and pathologic findings is also essential for a correct diagnosis.

Aged ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; diagnosis ; diagnostic imaging ; pathology ; Male ; Middle Aged ; Pulmonary Fibrosis ; diagnosis ; diagnostic imaging ; pathology ; Respiratory Function Tests ; Tomography, X-Ray Computed

OBJECTIVETo investigate the correlation of expression of vascular endothelial growth factor (VEGF) and proliferating cell nuclear antigen (Ki67) with sensitivity to neoadjuvant chemoradiation in rectal adenocarcinoma.

METHODSSamples of pretreatment biopsies and the resected specimens after neoadjuvant therapy in 32 patients with rectal adenocarcinoma were collected, and the expression of Ki67 and VEGF were detected by immunohistochemistry using specific antibodies. The correlation of Ki67 and VEGF expression with clinicopathological factors were analyzed.

RESULTSThe level of VEGF expression was significantly correlated with lymph node metastasis (P = 0.033), depth of tumor invasion (P = 0.007) and TNM stage (P = 0.016), but not with histological type, tumor size, age and gender of the patients (P > 0.05). However, VEGF expression was found to be negatively and significantly correlated with the sensitivity to neoadjuvant chemoradiation (P = 0.016), and a transient increase of VEGF expression was detected in the resected specimens after neoadjuvant therapy (P = 0.035). Ki67 labeling index (Ki67-LI) was found to be significantly correlated with lymph node metastasis (P = 0.028), but not with tumor size, age and gender of the patients (P > 0.05). It was also found that tumors with lower Ki67-LI expression were more sensitive to neoadjuvant therapy than that with higher expression of Ki67-LI (P = 0.032). In contrast with VEGF, the Ki67 expression level decreased after neoadjuvant therapy, but no statistical significance was found between pretreatment and posttreatment specimens (P > 0.05).

CONCLUSIONThe preliminary results of this study demonstrate that the expression of VEGF and Ki67 in pretreatment biopsy of rectal adenocarcinoma may be used as a biomarker to predict tumor response to neoadjuvant chemoradiation.

Adenocarcinoma ; metabolism ; pathology ; surgery ; therapy ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Biomarkers, Tumor ; metabolism ; Female ; Humans ; Ki-67 Antigen ; metabolism ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoadjuvant Therapy ; Neoplasm Invasiveness ; Neoplasm Staging ; Radiotherapy, Conformal ; Rectal Neoplasms ; metabolism ; pathology ; surgery ; therapy ; Vascular Endothelial Growth Factor A ; metabolism ; Young Adult

OBJECTIVETo analyze the clinical and pathological features of microscopic polyangiitis (MPA) to improve the diagnosis and treatment of the disease.

METHODSTwenty-five cases of MPA were retrospectively analyzed.

RESULTSThe onset symptoms of MPA, often nonspecific, included fever, muscle and joint pain, fatigue, loss of weight, etc, with varying degrees of proteinuria, hematuria and renal insufficiency. The pathological types revealed by renal biopsy were mainly focal segmental necrotizing glomerulonephritis or pauci-immune crescentic glomerulonephritis. Timely immunosuppressive therapy could improve the outcome.

CONCLUSIONThe early symptoms of MPA are often nonspecific to easily result in misdiagnosis. Examination of ANCA titers and timely renal biopsy are helpful to establish an early diagnosis. Immune suppression therapy and plasma exchange when necessary should be initiated after the establishment of the diagnosis. The disease activity and drug toxicity should be carefully monitored to improve the prognosis.

Adult ; Aged ; Female ; Humans ; Male ; Microscopic Polyangiitis ; diagnosis ; pathology ; therapy ; Middle Aged ; Prognosis ; Retrospective Studies