Objective To investigate the difference and treatment strategy of malignancy-associated dermatomyositis and other para-neoplastic neurological syndromes (PNS).Methods The clinical characteristics of a patient with malignancy-associated dermatomyositis and poly radiculoneuropathy was reported and the relevant literature was reviewed.Results Patients with dermatomyositis had increased risk of malignancies,and should routinely screened.Dermatomyositis and polyradiculoneuropathy was clinically similar,but could rarely be seen in the same malignant patient.Malignancy -associated dermatomyositis and PNS had similar pathogenesis.The treatment strategy of both was similar.Malignancy specific treatment should be initiated and immune suppressive agents should be prescribed concurrently.Conclusion Rheumatolgists should aware the association between dermatomyositis and potential underlying malignancies.Multiple para-neoplastic syndromes could be seen in the same patient,but the diagnosis should be considered as one.

0.05).DBP and DEHP obviously induced a decrease in organ body weight ratios of testis and epididymis and an increase in organ body weights ratios of liver.Obvious decrease in the sperm counts,spermatozoon survival rate and significant increase in the rate of the sperm deformation were observed.There was synergism between DBP and DEHP on the testis,epididymis and liver organ body weight ratio,as well as sperm counts,sperm survival and deformation rates.Pathological examination showed the seminiferous tubules were irregular shape,degenerative atrophy and interstitial substance broadening,and the seminiferous epithelium were degeneration.Epididymis epithelium was damaged and few of mature sperm was seen.Conclusion DBP combined with DEHP can cause obvious toxic effects on reproductive function in male rats.DBP and DEHP mixture can strongly affect the sperm quantity and quality.Also,some toxic effects to epididymis are observed.

Nonconvulsive status epilepticus (NCSE)is a peculiar kind of status epilepticus,which is not un-common in children,but it always be misdiagnosed due to its unnoticeable clinical signs.So far,there is no international unifying diagnose standard of NCSE.Therefore,electroencephalography(EEG)monitoring is recommended.Now,the progress in this field of clinical manifestation and EEG features of NCSE was reviewed.

Objective To study the combined toxic effects of di-n-butyl phthalate(DBP)and di-2-ethylhexyl phthalate(DEHP)on sex hormone and lipid peroxidation in male rats.Methods According to 2?2 factorial analysis,thirty-two healthy and clean adult male SD rats were randomly divided into 4 groups,including a control group(given coin oil) and three experimental groups:DBP(1/20 LD50,1.0 g/kg,dissolved in coin oil),DEHP(1/20 LD50,1.7 g/kg,dissolved in coin oil) and DBP+DEHP(1.0 g/kg + 1.7 g/kg,dissolved in coin oil),8 rats in each group,through gavage,once a day,for 8 consecutive weeks.The spectrophotometric method was used to measure the activity of lipid peroxidation SOD,GSH and GSH-Px level in testis homogenate.The activities of ACP,AKP and ?-GT were assessed in testis homogenate.Radioimmunoassay was used to determine the testosterone,LH and FSH levels in the serum.Results There was synergism between DBP and DEHP on the SOD activity in testicle and testosterone level in serum,and there was antagonism between DBP and DEHP on the ?-GT and ACP activity in testicle,as well as the FSH level in serum.Conclusion DBP combined with DEHP can cause obvious toxic effects on reproductive function in male rats.The change of testosterone biosynthetic enzymes and the levels of T in serum and disordered physiologic balances of hypothalamic-pituitarytestis axis may be key factors contributing to the decrease of testosterone,then the decrease of reproductive function in male rats.

Objective To investigate the change of plasma marinobufagenin (MBG) level and the expression of its receptor Na+-K+-ATPase (NKA) in renal biopsy specimens of chronic glomerulonephritis (CGN) patients. Methods Twenty-eight CGN patients and 14 healthy people were enrolled in the study. The plasma MBG concentration was measured by competitive inhibition ELISA system. Immunofluorescence and immunohistochemistry staining were applied to detect the expression of NKA in renal biopsy specimens of 28 CGN patients and analyzed by semi quantitively. Results Compared with healthy controls, CGN patients had significant lower plasma MBG concentration [(0.579±0.214) nmol/L vs (0.715±0.154) nmol/L, P＜0.05], without further significant difference between CGN patients with hypertension and with normal blood pressure [(0.595±0.231) nmol/L vs (0.557±0.197) nmol/L, P＞0.05]. Meanwhile, proximal tubular staining of NKA was decreased compared with normal controls. The NKA positive staining area of the CGN group was lower than that of normal controls [2.1% (0.5%-6.2%) vs 5.6% (3.5%-10.8%), P＜0.01] and correlated with 24-hour urinary sodium excretion (r=0.551, P＜0.01).Conclusion Decreased plasma MBG level and its receptor expression on proximal tubules may play a role in the regulation of sodium in CGN.

Objective To explore the clinical features and gene mutations of blepharophimosis-ptosis-intellectual-disability syndrome (BPID). Methods The clinical data, diagnosis and treatment of a child with BPID in neonatal intensive care unit (NICU) were reviewed. Based on the literature retrieved from PubMed database, the common classification, clinical features, diagnosis and genetic counseling of BPID and its affiliated blepharophimosis-mental retardation syndromes (BMR) were reviewed. Results This male infant was 39 weeks of gestational age with birth weight of 1920 g, and was admitted to NICU 15 min after birth due to dyspnea. The main clinical manifestations were facial deformity such as biepharophimosis, ptosis and micromandible, inspiratory dyspnea with laryngeal cartilage softening, malformations of the thorax and feeding difficulties. A heterozygous mutation in UBE3B gene was identified by complete exon sequencing and he was diagnosed of BPID, a rare genetic disorder. Reviewing the literature, there was no relevant report in domestic. While one foreign literature was found to report 5 patients from 4 families having a subtype of BMR, a kind of autosomal recessive diseases caused by mutations in the UBE3B gene. Conclusion BPID is a rare clinical entity of BMR. Complete exon sequencing can be used to diagnose the disease.

Objective To determine the effects of intrauterine growth retardation(IUGR) caused by malnutrition during pregnancy on the lung structure and expression of Clara cell protein (CCSP) and interferon (IFN)-γ in the fetal lungs,and to explore their relation ship with pulmonary disease.Methods Fetal rats from maternal protein-malnutrition dams were studied on day 20(term 21.5 day).The lung pathology was examined by means of Hematoxylin and eosin(HE) stain.Plasma was collected to determine the CCSP and IFN-γ concentration.Lungs were harvested to measure the expression of CCSP and IFN-γ mRNA by using fluorescent quantization reverse transcription (RT)-PCR and the levels of CCSP and IFN-γ protein were assessed by using enzyme-linked immunosorbent assay.Results Malnutrition fetus body weight significantly less compared to control group,so did the lung weight.However,lung weight,expressed as a percentage of body weight between the 2 groups was not different.The IUGR group had significantly decreased alveolar number manifested by lower radial alveolar count,and significantly increased mean linear intercept of alveoli.Both the CCSP mRNA expression and protein level in lung of IUGR rats were decreased compared with control rats (all P ＜ 0.05).A decline in plasma CCSP protein concentration was also noted compared with control group (P ＜0.05).Furthermore,IUGR group fetus showed lower IFN-γ levels both in circulation and in the lung tissue (all P ＜ 0.05).Conclusions Intrauterine malnutrition significantly alters lung structure and cytokine IFN-γ level,and the latter may further inhibit the transcription of CCSP gene.These alterations may contribute to both early and late postnatal respiratory morbidity.

Objective To assess the application of MRI in diagnosis of oblique vaginal septum syndrome (OVSS).Methods Clinical and imaging data of 41 patients with OVSS confirmed by surgery from March 2011 to November 2016 were retrospectively analyzed.Results The average age of patients was 20.5 year (10-46 years).The primary clinical symptoms were menorrhalgia (16 cases) and menorrhagia (13 cases).There were 12 cases of type Ⅰ,23 cases of type Ⅱ,5 cases of type Ⅲ and 1 case of type Ⅳamong 41 cases of OVSS.The resections for OVSS were performed in 35 cases.Forty two cases were diagnosed as OVSS by MRI scan,and 41 were confirmed by surgery,the accuracy of MRI diagnosis was 97.6％ (41/42).MRI showed uterus didelphys,hydrocolpos or hematocolpos with varying degrees,and revealed ipsilateral renal agenesis in all 41 cases.Conclusion MRI scan can accurately diagnose oblique vaginal septum syndrome and provide comprehensive information for clinical treatment.

Objective To investigate the effects of H3K9ac hyperacetylation mediated by histone acetylases on the overexpression of MEF2C in the hearts of fetal mice exposed to alcohol during pregnancy,and provide new interven-tion targets for prevention and treatment of cardiac dysplasia caused by alcohol exposure.Methods C57BL/6 mice were divided into 5 groups randomly,blank control group,dimethylsulfoxide (DMSO)group,alcohol group,alcohol +anacardic acid group and anacardic acid group,and then the hearts of fetal mice were collected to be analyzed.Chroma-tin immunoprecipitation and Western blot were used in assaying the binding of histone acetylases and the level of H3K9ac to the promoter of MEF2C in the hearts of fetal mice.The mRNA expression of MEF2C was tested by adopting real -time PCR.Results The level of H3K9ac in the promoter of MEF2C in the hearts of fetal mice exposed to alcohol was higher than that in the hearts of fetal mice exposed to saline (1 .30 ±0.1 9 vs 0.45 ±0.01 ),there was statistically significant difference (P <0.05),while the binding of E1 A -binding protein (p300),p300 /cyclic adenosine mono-phosphate response element binding protein -associated factor (PCAF)and steroid receptor coactivator -1 (SRC1 )to the promoter of MEF2C were abnormally elevated in the hearts of fetal mice treated by alcohol (1 .68 ±0.08 vs 0.82 ± 0.08,1 .08 ±0.05 vs 0.42 ±0.02,1 .1 8 ±0.05 vs 0.39 ±0.08),and there were statistically significant differences (all P <0.05).The expression of MEF2C mRNA in alcohol group was higher than that in blank control group (1 .36 ± 0.1 2 vs 0.29 ±0.03),there was statistically significant difference(P <0.05).However,a pan -acetylases inhibitor, anacardic acid,could decrease significantly the binding of p300 and PCAF to the promoter of MEF2C (1 .52 ±0.05 vs 0.63 ±0.09,1 .1 3 ±0.04 vs 0.45 ±0.04),and correct abnormal hyperacetylation of H3K9ac induced by alcohol (1 .58 ±0.08 vs 0.67 ±0.05),and down -regulate the over -expression of MEF2C in the hearts of fetal mice exposed to alcohol (1 .36 ±0.1 2 vs 0.41 ±0.05 ),and there were statistically significant differences (all P <0.05 ). Conclusions Hyperacetylation of H3K9ac mediated by p300 and PCAF may be a key regulatory factor in the over -expression of cardiac nuclear transcription factor MEF2C in the hearts of fetal mice exposed to alcohol during pregnan-cy.Anacardic acid can significantly attenuate the level of H3K9ac through inhibiting the binding of p300 and PCAF to the promoter of MEF2C,and down -regulate the over -expression of cardiac nuclear transcription factor MEF2C in the hearts of fetal mice.