Pseudohypoparathyroidism(PHP) is a rare genetic disorder.The main character is parathyroid hormone resistance,and some with typical Albright's Hereditary Osteodystrothy malformation.The wide range of PHP symptom spectrum may lead to miss or misdiagnosis.This paper reviewed and summarized the pathogenesis,manifestation and the progress on the diagnosis and treatment of PHP Ⅰ,so as to improve the diagnostic level of this disease.

Objective To investigate the changes of plasma microRNA-223 (miR-223) in pediatric severe pneumonia patients and its relationship with severe pneumonia.Methods There were 50 children with severe pneumonia enrolled in the study (observation group),and 50 healthy children were selected as control group (normal control group).The expression levels of plasma miR-223 were detected by real time polymerase chain reaction.The relationship between miR-223 and procalcitonin (PCT),C-reactive protein (CRP),tumor necrosis factor-α (TNF)-α,and interleukin (IL)-10 were analyzed.The predictive value of miR-223 in plasma,PCT,and CRP to severe pneumonia was evaluated by receiver operating characteristic (ROC) curve.The relationship between miR-223,T cells,TNF-α,and IL-10 was analyzed.Results The plasma miR-223 expression levels in observation group were upregulated compared to those in the normal control group [(16.01 ± 5.17) × 10-4 mg/L vs (5.44 ± 2.21) × 10-4 mg/L,t =7.46,P ＜ 0.01].The CRP and PCT expression levels in severe pneumonia patients were higher than those of the normal control group [z =5.496,5.198,P ＜0.05,orP ＜0.01].The expressions of CD4+ CD25+Treg,miR-223,and IL-10 in observation group were higher than those of normal control group (P ＜0.05).The expression of CD4 + CD25 + Treg,miR-223,and IL-10 in the death children were higher.There was a positive correlation between miR-223 and IL-10 (r =0.335,0.571,P ＜ 0.01).The sensitivity and specific degrees to predict the severe pneumonia in mir-223,PCT,and CRP were 83.79％,86.12％,66.68％,91.05％,78.01％,and 44.23％,respectively.miR-223 was better than PCT and CRP to predict severe pneumonia in children.Conclusions The expression levels of plasma miR-223 in children with severe pneumonia could reflect the immunity,and it can be used as early prognostic markers to reflect the severity of inflammation in some degree.

Objective To investigate the clinical efficacy of conventional treatment combined with flupentixol and melitracen in patients with reflux esophagitis (RE).Methods From June 2012 to March 2015, a total of 182 patients were selected as study subjects from newly diagnosed RE patients.The anxiety and depression scores were evaluated according to Hamilton anxiety scale (HAMA) and Hamilton depression scale (HAMD).And then patients were divided into HAMA and HAMD negative conventional treatment group and combined treatment group, HAMA and HAMD positive conventional treatment group and combined treatment group.Rabeprazole and mosapride were administrated in conventional treatment group.For patients in combined treatment group, on the base of conventional treatment flupentixol and melitracen were added.The treatment course was eight weeks.The degree of anxiety and depression, RE symptoms and mucosal healing under gastroscope were evaluated before and after treatment.Adverse drug reaction was observed.Chi square test or t test was performed for statistical analysis.Results Eight weeks after treatment, the scores of HAMA and HAMD in HAMA and HAMD positive combined treatment group were 7.930 ±3.832 and 9.630 ± 3.650, which were both lower than those of conventional treatment group (11.660 ± 4.108 and 12.170 ± 4.459), and the differences were statistically significant (t=3.683 and 2.233;both P＜0.05).The symptom scores of heartburn, regurgitation, chest pain of HAMA and HAMD positive combined treatment group were 0.700±0.591,0.780± 0.629 and 0.720±0.621, respectively, which were lower than those of conventional treatment group (1.280 ± 0.502, 1.370 ± 0.610 and 1.040 ± 0.842), and the differences were statistically significant (t =5.133, 4.413 and 2.114, all P＜0.05).There were no statistical significance in symptoms scores between HAMA and HAMD negative combined treatment group and conventional treatment group (all P＞0.05).After treatment, the mucosal healing rate of HAMA and HAMD positive combined treatment group was 91.3％ (42/46), which was higher than that of conventional treatment group (71.7 ％, 33/46), and the difference was statistically significant (x2 =5.845, P =0.016).The incidence of adverse events of HAMA and HAMD negative combined treatment group was 4.8％ (2/42), and that of HAMA and HAMD positive combined treatment group was 2.2％(1/46).Conclusions The conventional treatment combined with flupentixol and melitracen in RE patients accompanied with anxiety and depression was remarkable and safe.RE patients without obvious anxiety or depression, preventive use of antianxiety and antidepressant medicine can not improve the efficacy.

Moyamoya disease (MMD) is a chronic and progressive cerebrovascular disease which is characterized by the bilateral internal carotid artery ends and (or) stenosis or occlusion of anterior cerebral artery and middle cerebral artery initial segments,compensatory proliferation of small blood vessels in the skull base and formation of abnormal vascular network.Its etiology and pathogenesis remains unclear.The present studies speculate that MMD may be a polygenic disease,inflammation,immune response,abnormal cytokine secretion,endothelial progenitor cell change and nitric oxide level change are associated with the occurrence and development of MMD.This article reviews the advances in research on the genetics and pathophysiological mechanism of MMD.

Cholesteatoma ; diagnosis ; pathology ; Humans ; Petrous Bone ; pathology

Objective To investigate general and clinicopathological characteristics of male breast cancer and analyze the factors affecting the outcomes of the patients.Methods Fifty-nine male breast cancer patients treated at Cancer Hospital of Zhengzhou University from January 2002 to December 2011 were included into the study.The clinicopathologic features and 5-year survival rate were retrospectively analyzed.The clinicopathologieal characteristics were investigated by univariate analysis to evaluate the impact of these factors on patient survival.Results The median age at diagnosis was 64 years old in these patients.The positive rate of ER/PR was 74.6 ％ (44/59).The patients were followed up for 9-123 months.The 5-year survival rate was 61％.Patients in stages Ⅰ and Ⅱ had better overall survival than those in stages Ⅲ and Ⅳ.Conclusion The male breast cancer patient has special clinical characteristics.TNM stage is a significant predictor of the overall survival.

ObjectiveTo study the role of Kukoamine B (KB) in inhibiting the inflammatory response of small intestine in septic mice and its molecular mechanisms.Methods Twenty-four male ICR mice were randomly divided into control group, model group, and KB intervention group (each,n= 8). Sepsis model was reproduced by intra-peritoneal injection of 20 mg/kg lipopolysaccharide (LPS), while equivalent normal saline was given in control group, and 20μg/kg KB was injected through caudal vein 4 hours after LPS challenge in KB intervention group. The blood/tissue samples (jejunum and ileum) were harvested 8 hours after LPS injection. The levels of plasma LPS, tumor necrosis factor-α (TNF-α) and interleukin-1β (IL-1β) were measured. The pathological changes in small intestine tissues were observed under light microscope, while the levels of inflammatory cytokines TNF-α and IL-1β in the tissue homogenates (jejunum and ileum) were assessed by enzyme linked immunosorbent assay (ELISA). The activity of myeloperoxidase (MPO) was measured by colorimetry. The expression of intercellular adhesion molecule-1 (ICAM-1) was determined by immunohistochemistry. The expressions of inducible nitric oxide synthase (iNOS) mRNA was assayed by reverse transcription-polymerase chain reaction (RT-PCR). The activation of nuclear factor-κΒ (NF-κΒ) was determined by Western Blot.Results The mice in model group were found to have an increase in microvascular permeability, interstitial edema, and infiltration of white blood cells, and the levels of LPS, TNF-α and IL-1β in their plasma, with an increase in concentrations of TNF-α and IL-1β, activity of MPO, positive expression of ICAM-1, expression of iNOS mRNA and NF-κB protein in small intestine (jejunum and ileum). Compared with model group, in mice with KB intervention, microvascular permeability, interstitial edema, and infiltration of white blood cells were reduced significantly, while the levels of LPS, TNF-α and IL-1β in plasma, the concentration of TNF-α and IL-1β, the activity of MPO, the positive expression of ICAM-1, the expression of iNOS mRNA and NF-κB protein in small intestine (jejunum and ileum) were significantly decreased [plasma LPS (kEU/L): 654.09±28.13 vs. 1 155.65±47.15, TNF-α (ng/L): 12.75±0.47 vs. 30.61±0.71, IL-1β (ng/L): 53.06±5.32 vs. 64.47±2.61; jejunum TNF-α(ng/L): 43.27±1.20 vs. 64.82±2.09, IL-1β (ng/L): 326.38±14.47 vs. 535.22±13.48, MPO (U/g): 0.14±0.01 vs. 0.32±0.02, iNOS mRNA (2-ΔΔCt): 2.39±0.13 vs. 10.80±0.22, NF-κB protein (gray value): 0.687±0.062 vs. 1.404±0.046; ileum TNF-α (ng/L): 62.75±3.92 vs. 104.24±2.82, IL-1β(ng/L): 408.06±1.70 vs. 521.97±1.16, MPO (U/g): 0.36±0.08 vs. 0.66±0.05, iNOS mRNA (2-ΔΔCt): 1.65±0.11 vs. 3.59±0.29, NF-κB protein (gray value):0.830±0.114 vs. 1.609±0.051, allP< 0.05].Conclusion KB can combine with LPS and inhibit LPS/Toll-like receptor 4 (TLR4) signaling pathway, thereby significantly inhibit the inflammatory response and protect the function of the small intestine in LPS-induced septic mice.

Objective To investigate histopathological and ultrastructural differences between melasma tissues and normal skin tissues around pigmented nevus. Methods Eight patients with melasma and 16 patients with facial pigmented nevus were included in this study. Two millimeter punch biopsies were taken from melasma lesions and adjacent normal skin of facial pigmented nevus. Biopsy specimens were then subjected to hematoxylin?eosin (HE) staining, Fonton?Masson staining, Verhoeff?van Gieson staining, and immunohistochemical staining with monoclonal antibodies HMB45 and NKI/beteb. Transmission electron microscopy was used to observe the tissue specimens. Semi?quantitative analysis was performed under a light microscope, and quantitative analysis by using a computerized image analysis system. Results Histopathological study revealed increased number of melanin granules mainly in the basal and prickle cell layers, sometimes in the dermis, in melasma tissues compared with normal skin tissues. Melanocytes were only observed in the epidermis of melasma tissues. Compared with normal skin tissues, melasma tissues showed no significant difference in the quantity of melanocytes, but a significant increase in the volume, staining intensity and dendrite number of melanocytes. In all of the 8 patients with melasma, mild to moderate lymphocytic infiltration was observed in the superficial dermis and around capillaries, with moderate telangiectasis in the superficial dermis. Electron microscopy revealed that there were more melanosomes in melanocytes and keratinocytes, and melanocyte dendrites extended into the dermis in melasma tissues. Conclusions Among the 8 patients, there were only two types of melasma, i.e., epidermal melasma and mixed melasma, and no dermal melasma was found. Inflammation and telangiectasis may induce or aggravate melasma.

Objective To optimize the volatile oil extraction and inclusion process of Wenweiyang capsules. Methods An orthogonal test was adopted in this study. The extraction technology was optimized for the yield of volatile oil regarding the amount of water loaded, grain size of medicinal material, and decoction time as factors. The inclusion technology was optimized for the inclusion yield and volatile oil inclusion rate using the ratio ofβ-CD:oil, amount of water and grinding time as factors. Results The optimized extraction parameters were as follows:breaking medicinal material through 10 mesh screen, adding 6 fold volume of water and extracting for 5 h. The optimized inclusion progress was grinding at theβ-CD:oil ratio of 81, loading equivalent amount of water and grinding for 30 minutes. The average yield of volatile oil is 1. 72%, the average inclusion rate is 93. 01% and the average volatile oil inclusion rate is 74. 82%. Conclusion The extraction and inclusion technology is simple, reliable, which can effectively retain the volatile oil and provide evidence for the preparation of Wenweiyang capsules.

Objective To investigate the inhibitory effect of kukoamine B (KB) on lung inflammatory responses in mice with sepsis and its possible molecular mechanism.Methods Twenty-eight male mice were randomly divided into control group (n=8),lipopolysaccharide (LPS) group (n=10),and LPS + KB group (n=10).Sepsis model was reproduced by intra-peritoneal injection of 20 mg/kg LPS,while equivalent normal saline was given in control group,and 20 μg/kg KB was injected through caudal vein 4 hours after LPS challenge in LPS + KB group.After 8 hours of LPS challenge,the concentration of LPS in plasma and the activity of myeloperoxidase (MPO) in the lung tissue were determined.The contents of tumor necrosis factor-α (TNF-α) and interleukin-lβ (IL-1β) in plasma,alveolar lavage fluid and lung tissue homogenates were assessed by enzyme linked immunosorbent assay (ELISA).The activation of nuclear factor-κB (NF-κB) and the expression of inducible nitric oxide synthase (iNOS) in lung tissue were determined by Western Blot.The pathological changes in lung tissues were observed with hematoxylin-eosin (HE) staining.The expression of intercellular adhesion molecule-1 (ICAM-1) in lung tissue was determined by immunohistochemistry.Results Compared with control group,the concentration of LPS in plasma (kEU/L:1 155.650 ± 147.149 vs.31.390 ± 18.859),MPO activity (U/g:1.177 ±0.093 vs.0.775 ±0.166),NF-κB activity (gray value:1.557 ±0.105 vs.0.824 ±0.032) and the expression of iNOS (gray value:0.650 ±0.129 vs.0.392 ±0.097) were significantly increased in LPS group (all P＜0.05).After KB intervention,the concentration of LPS (624.461 ± 149.012),MPO activity (0.919 ±0.023),NF-κB activity (1.127 ±0.074) and the expression ofiNOS (0.425 ± 0.066) were significantly lowered (all P＜0.05).Compared with control group,the contents of TNF-α (ng/L:47.325 ± 13.864 vs.6.534 ± 0.544,13.382 ± 2.231 vs.3.748 ± 0.692,31.127 ± 7.399 vs.14.948 ± 4.673) and IL-1β (ng/L:74.329 ± 11.890 vs.29.921 ± 6.487,9.422 ± 2.674 vs.1.105 ± 0.364,528.509 ± 32.073 vs.109.945 ± 13.561) in plasma,alveolar lavage fluid and lung tissue homogenates were obviously enhanced in LPS group (all P＜0.05).With KB intervention,the contents of TNF-α (20.331 ± 7.789,7.145 ± 1.202,15.966 ± 2.946) and IL-1β (57.707 ±8.098,2.212 ± 0.878,426.154 ± 11.270) were markedly reduced (plasma TNF-α:F=16.052,P=0.002; IL-1β:F=20.649,P=0.000; lung tissue homogenates TNF-α:F=31.134,P=0.001; IL-1β:F=22.792,P=0.002;alveolar lavage fluid TNF-α:F=10.013,P=0.009; IL-1β:F=319.857,P=0.000).In addition,leukocyte infiltration to the lung tissue was attenuated,and the expression of ICAM-1 was reduced by KB in histological examination.Conclusion KB,as a neutralizer of LPS,can inhibit the release of inflammatory mediators,reduce the pulmonary inflammatory response and protect the function of lung in septic mice.