The etiology of reversible posterior leukoencephalopathy syndrome(RPLS)is more complex.Its pathogenesis has a lot of controversies,and its clinical manifestations are headache, nausea,vomiting,epileptic seizure,consciousness disturbance and visual abnormality.Neuroim- aging examinations have shown that diffuse symmetrical white matter edema is predominantly in the bilateral posterior brain regions.Clinical symptoms and neuroimaging changes can be recov- ered completely after timely and effective treatment.If the treatment is delayed,it may compli- cate with cerebral infarction or hemorrhage,and lead to irreversible damage.

Objective To evaluate 18F-fluorodeoxyglucose (FDG) PET/CT in detecting occult malignancy in patients with suspected paraneoplastic neurological syndrome (PNS).Methods Twenty consecutive patients who underwent PET/CT scanning with the indication of suspected PNS were retrospectively reviewed.The gold standard of PNS was either cytology or clinical follow-up, and the final diagnosis was compared with PET/CT findings.Results Of the 20 patients, six were PNS.PET/CT detected nine cases.Six were true positive and three were false positive.The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of PET/CT were 100% (6/6), 78.57% (11/14), 85.00% (17/20),66.7% (6/9) and 100.00% ( 11/11 ) respectively.The treatment plan was modified based on the PET/CT results in 4 patients.Conclusions 18F-FDG PET/CT may play a role in detecting the underlying malignancy of PNS.It is also valuable in staging of the malignancy thus providing information for therapy decision making.

OBJECTIVETo improve the accuracy of the diagnosis of the disease on the basis of the clinical features and genetic characteristics of patients with Silver Russell syndrome (SRS).

METHODPatients diagnosed with SRS by Price criteria in 2006 to 2011 were reviewed for their clinical manifestations, physical signs, laboratory examinations and treatments.

RESULTTwenty cases with SRS were 0.08-12.17 yr old. Fifteen were male and 5 were female. The clinical characteristics included more than 80% of cases had postnatal growth retardation 100% (20/20), craniofacial dysmorphism 100% (20/20), small for gestation age 95% (19/20), asymmetry and thinning of the face and/or limbs 90% (18/20), fifth finger clinodactyly 80% (16/20), BMI < -2 SDS 80% (16/20). Their height was obviously lagging behind in the bone age. HD SDS/average of bone retardation was 3.08. The two patients with the chief complaint of external genital abnormalities would have aggressive surgical treatment and they did not use the growth hormone (GH) treatment. Only six patients had used the GH treatment. GH treatment at a dose of 0.1 IU/(kg·d) used in 2 cases achieved a growth velocity (GV) 8 - 11 cm/yr but in another 2 cases < 5 cm/yr. In genetic study, 6 patients were found to have 11p15 low methylation, 1 had low and high methylation, 1 had duplication, no relation between clinical and methylation of 11p15 was found.

CONCLUSIONThere were great variations of clinical features in SRS characterized by small for gestation age and/or postnatal growth retardation, craniofacial dysmorphism, asymmetry of the face and/or limbs or ultrafine limbs, fifth finger clinodactyly. Severely low BMI was seen and height was obviously lagging behind in the bone age. The findings of laboratory tests and imaging of SRS were not specific. Some of SRS had 11p15 imprinting defects. The treatment of SRS is mainly symptomatic.

Abnormalities, Multiple ; diagnosis ; genetics ; Adolescent ; Body Height ; Bone Density ; Child ; Child, Preschool ; Chromosomes, Human, Pair 11 ; genetics ; DNA Methylation ; Female ; Genetic Association Studies ; Genomic Imprinting ; Growth Disorders ; diagnosis ; genetics ; Humans ; Infant ; Male ; Retrospective Studies ; Silver-Russell Syndrome ; diagnosis ; genetics

Loss of transforming growth factor (TGF)-beta signaling has been implicated in malignant transformation of various tissues. Smad4 plays a central role in the signal transduction of TGF-beta. Deletion or mutation of Smad4 has been described in a number of cancers. This study was aimed to investigate a potential role of Smad4 in leukemia including its expression and location in blast cells. The mononuclear cells were separated from bone marrow of leukemia patients. The samples, blast cells of which were more than 90% in mononuclear cells, were selected. The expression and location of Smad4 protein were analyzed by immunohistochemistry methods. The results showed that the Smad4 protein located mainly in nucleus, part of this protein located in cytoplasma, the expressions of Smad4 were not detected in 6 out of 9 ALL patients, in 7 out of 24 AML patients and in 1 out of 2 CML patients; these leukemia patients, in whose cells the expression of Smad4 was not detected, included one L1 and one L3, four L2, one M0, one M1, two M2a, one M3a, one M4b, one M6 and one CML. In conclusion, the Smad4 protein was mainly in nucleus, the deletion or functional change of Smad4 may related with the pathogenesis of human AML.
Humans ; Leukemia, Myeloid, Acute ; genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; genetics ; Signal Transduction ; Smad4 Protein ; biosynthesis ; genetics ; Transforming Growth Factor beta ; biosynthesis ; genetics

OBJECTIVETo explore the feasibility of a dual-energy computed tomographic angiography (DECTA) protocol using test-bolus injection with reduction of contrast material (CM) dose in second generation dual-source CT system.

METHODSTotally 57 consecutive patients underwent CT angiography scan covering the cervical and cerebral arteries. CT was performed with second generation dual-source CT system. The time to peak (T) using a test-bolus injection was calculated. The patients were divided into three groups (A, B, and C) with different CM doses (40, 45, and 50 ml) and different delay time points [ (T+1) , (T+1) , and (T+2) s] . All the patients were followed by a 48 ml saline flush. Arterial enhancements were quantified by measuring attenuation values of the aortic arch, bifurcation of common carotid artery, contralateral internal jugular vein of the CM injection, superior vein cava, proximal middle cerebral artery, basilar artery, and straight sinus on source images. Visualizations of intracranial artery and ipsilateral venous effect of the CM injection were rated on a four-point grading scale on CTA images for qualitative assessment.

RESULTSAlthough the attenuation of internal jugular vein and straight sinus were significantly lower in group A than in groups B and C (P<0.05) , the attenuation of aortic arch, superior vein cava, common carotid artery, middle cerebral artery, and basilar artery vessels showed no significant differences among these three groups. The scores of the visualizations of intracranial artery and ipsilateral venous effect of the CM injection were also not significantly different among these three groups.

CONCLUSIONBased on the delay time calculated by a test-bolus injection, a reduced-dose contrast material may provide an equal degree of arterial attenuation and a lower attenuation of vein for dual-energy CTA covering the craniocervical region in second generation dual-source CT system.

Adult ; Aged ; Angiography ; methods ; Contrast Media ; administration & dosage ; Feasibility Studies ; Female ; Head ; diagnostic imaging ; Humans ; Male ; Middle Aged ; Neck ; diagnostic imaging ; Radiation Dosage ; Tomography, X-Ray Computed ; methods

OBJECTIVETo study the relationship on the prevalence rate of hepatitis B virus (HBV) infection and hepatitis B vaccination in urban citizens aged over 20 years old which would led to the development of strategies on HBV control.

METHODSA total of 3744 subjects from general population were randomly selected in this study. Both ELISA and radio immunoassay were used to test five items of HBV infection, including HBsAg, anti-HBs, HBeAg, anti-HBe and anti-HBc.

RESULTSThe overall standardized infection rate of HBV was 51.7%, and HBsAg, anti-HBs, HBeAg, anti-HBe and anti-HBc were 4.5%, 48.5%, 0.3%, 3.5% and 51.4%, respectively. The two lowest HBsAg positive rates were found in the groups under 30 years old (2.9%) and students (2.6%). Anti-HBc rate among men was significantly higher than seen in women (P < 0.05), and showing a trend of increase with age (chi2 for trend = 256.2, P < 0.001). The standardized rates of HB vaccination in this population was 17.6% and decreasing rapidly with age (P < 0.05). People who had been vaccinated had both lower rates of HBsAg and HBV infection but higher rate of anti-HBs than those who had not (P < 0.05).

CONCLUSIONHB vaccination in adults showed a reducing rate of HBV infection in the general population. Together with the enhancement of expanded program on immunization towards HB vaccination in neonates, much attention should be paid to HB vaccination in adults.

Adult ; Aged ; Aged, 80 and over ; China ; epidemiology ; Cross-Sectional Studies ; Female ; Hepatitis B ; epidemiology ; immunology ; prevention & control ; Hepatitis B Vaccines ; immunology ; Hepatitis B virus ; immunology ; Humans ; Immunity, Innate ; Male ; Middle Aged ; Prevalence ; Young Adult

OBJECTIVEData obtained from a differentially expressed cDNA library constructed previously in this laboratory demonstrated that the extracellular matrix molecule osteopontin (OPN) is one of most considerably over-expressed genes in non-small cell lung cancers (NSCLCs). The purpose of the present study was to explore the expression status of OPN in a large scale NSCLC tissue samples, and estimate its significance in progression of the malignant disease.

METHODSRT-PCR was performed with the tumor and adjacent normal tissues from 35 patients with NSCLC, at transcriptional levels of OPN. To determine the expression of OPN protein in the tumor tissues, immunohistochemical (IHC) staining was subsequently carried out on paraffin-embedded sections in tissue microarrays containing 662 samples derived from NSCLC cases. The correlation between the expression level of OPN and clinical characteristics was analyzed statistically.

RESULTSComparing with the paired normal lung tissue, high level RNA of OPN was detected in 80.0% (28/35) of the NSCLC tumor tissues by RT-PCR, which confirmed the information obtained previously by our differentially expressed cDNA library. The results of IHC analysis showed that positively stained OPN protein was observed in 59.6% (331/555) of the tumor tissues, which was remarkably higher than that (25.2%, 27/107) detected in the normal control tissues (P < 0.001). Among the NSCLCs investigated, over-expressed OPN was more frequently found in squamous cell carcinomas (SCCs) than in adenocarcinomas. A further analysis on SCCs demonstrated that the rate of over-expressed OPN was significantly different between the primary tumors with and without lymphatic metastases (68.6% vs. 49.7%, P = 0.001), but similar in the primary tumors and their corresponding metastases in lymph nodes (68.6% vs. 75.5%, P = 0.171).

CONCLUSIONExpression of OPN protein is distinctly increased in NSCLCs, particularly in SCCs. OPN over-expression is considerably correlated with lymph node metastasis, increasing the risk of tumor metastasis (OR = 2.212). The resulting data suggest that OPN facilitates the progression of NSCLCs.

Adenocarcinoma ; metabolism ; pathology ; Adult ; Aged ; Aged, 80 and over ; Carcinoma, Non-Small-Cell Lung ; metabolism ; pathology ; Carcinoma, Squamous Cell ; metabolism ; pathology ; Female ; Gene Expression Regulation, Neoplastic ; Humans ; Lung Neoplasms ; metabolism ; pathology ; Lymphatic Metastasis ; Male ; Middle Aged ; Osteopontin ; genetics ; metabolism ; Up-Regulation

Objective To observe the efficacy of Chinese medicine in the treatment of pregnancy complicated with thalassemia and to investigate its influence on pregnancy outcomes.Methods A retrospective cohort study was carried out in 175 pregnant women complicated with thalassemia who met the inclusion criteria.According to the treatment methods,the patients were divided into Chinese medicine group(105 cases),iron supplementation group(41 cases)and untreated group(29 cases).The changes of hematological indicators and pregnancy outcomes in the second and third trimesters of pregnancy were compared among the three groups.The Chinese medicine group was further divided into three subgroups:56 cases in the Chinese patent medicine group,20 cases in the single Chinese medicine group and 29 cases in the Chinese herbal compound group.The changes of hematological indexes in the second and third trimesters of pregnancy in the three subgroups were compared.Moreover,the normative management of pregnant women with thalassemia during pregnancy was explored.Results(1)The concentration of hemoglobin(Hb)in the third trimester of the Chinese medicine group was(2.28±7.27)g/L higher than that in the second trimester,and the curative effect of Chinese medicine group was superior to that in the iron supplementation group and the untreated group(P＜0.05).The Hb concentration in the Chinese medicine group before delivery was(12.17±10.81)g/L higher than that in the second trimester,and the increase in the Chinese medicine group was more significantly than that in the other two groups(P＜0.05).(2)The level of serum ferritin(FER)in the third trimester of the three groups was lower than that in the second trimester,and the decrease of FER level in the iron supplementation group was less significantly than that in the other two groups(P＜0.05).(3)The Hb concentration in the third trimester of the single Chinese medicine group and the Chinese herbal compound group increased by(4.50±4.66),(3.62±8.77)g/L respectively compared with that in the second trimester,and the increase in the above two groups was more significantly than that in the Chinese patent medicine group(P＜0.05).(4)There was no significant difference in pregnancy outcomes among the three groups of pregnant women with thalassemia(P＞0.05).(5)Only 52.0％(91/175)of pregnant women with thalassemia underwent three or more blood routine tests after 20-24 weeks of pregnancy,34.3％(60/175)of pregnant women failed in re-examining the FER levels,and 21.2％(37/175)of pregnant women had no standardized iron supplementation.Conclusion Chinese medicine therapy is effective on improving anemia in pregnant women with thalassemia.Chinese herbal compound and single Chinese medicinal are more effective than Chinese patent medicine,and have not increased the risk of adverse pregnancy outcomes.Oral administration of iron can increase the iron reserve of pregnant women with thalassemia,but its effect on improving anemia is not as good as that of Chinese medicine.Attention should be got to the monitoring of anemia and iron metabolism indicators as well as the standardized use of iron supplements and Chinese patent medicines in pregnant women with thalassemia.

OBJECTIVETo establish DNA microarrays-based microRNA (miRNA) expression profiles of squamous cell carcinoma of larynx, using archived formalin-fixed paraffin-embedded tissue blocks, and to screen out and identify the differentially expressed miRNAs associated with the biological characteristics of this malignant disease.

METHODSTotal RNA was prepared from the formalin-fixed paraffin-embedded tissue blocks. After quality identification and fluorescent labeling, the RNA samples were hybridized with the Agilent human miRNA microarrays which contains 723 probes for human miRNAs. The data was processed with the softwares GeneSpring GX and R-Project.

RESULTSFrom the formalin-fixed paraffin-embedded tumor blocks collected, 24 RNA samples were obtained with the quality accorded to the requirement of miRNA microarray analysis, and both the hybridization and consequent data processing were accomplished. A total of 319 miRNAs were identified and among them 96 were detected in all the 24 formalin-fixed paraffin-embedded blocks of laryngeal carcinoma; and 5 differentially expressed miRNAs (false discovery rate < 0.05) were found to be associated significantly with the lymphatic metastasis of laryngeal squamous cell carcinoma (P < 0.05), including miR-23a(*), miR-28-5p, miR-15a, miR-16 and miR-425.

CONCLUSIONSHistopathological archives of well-annotated formalin-fixed paraffin-embedded tissue specimens are the valuable resources for miRNA study including to collect RNA samples for miRNA microarray analysis. A panel of differentially expressed miRNAs (miR-23a(*), miR-28-5p, miR-15a, miR-16 and miR-425) derived from the miRNA expression profile may serve as the potential molecular biomarkers for the prediction of metastasis development in laryngeal squamous cell carcinoma.

Carcinoma, Squamous Cell ; genetics ; metabolism ; pathology ; Gene Expression Profiling ; Humans ; Laryngeal Neoplasms ; genetics ; metabolism ; pathology ; Lymphatic Metastasis ; MicroRNAs ; metabolism ; Oligonucleotide Array Sequence Analysis ; methods ; Paraffin Embedding

BACKGROUNDIn central precocious puberty (CPP), the pulse secretion and release of gonadotropin-releasing hormone (GnRH) are increased due to early activation of the hypothalamic-pituitary-gonadal axis, resulting in developmental abnormalities with gonadal development and appearance of secondary sexual characteristics. The CPP without organic disease is known as idiopathic CPP (ICPP). The objective of the study was to evaluate the clinical efficacy and safety of domestic leuprorelin (GnRH analog) in girls with ICPP.

METHODSA total of 236 girls with ICPP diagnosed from April 2012 to January 2014 were selected and were randomized into two groups. One hundred fifty-seven girls in the test group were treated with domestic leuprorelin acetate, 79 girls in the control group were treated with imported leuprorelin acetate. They all were treated and observed for 6 months. After 6-month treatment, the percentage of children with peak luteinizing hormone (LH) ≤3.3 U/L, the percentage of children with peak LH/peak follicle stimulating hormone (FSH) ratio <0.6, the improvements of secondary sexual characteristics, gonadal development and sex hormone levels, the change of growth rate of bone age (BA) and growth velocity, and drug adverse effects between two groups were compared.

RESULTSAfter the treatment, the percentage of children with a suppressed LH response to GnRH, defined as a peak LH ≤3.3 U/L, at 6 months in test and control groups were 96.80% and 96.20%, respectively, and the percentage of children with peak LH/FSH ratio ≤0.6 at 6 months in test and control groups were 93.60% and 93.70%, respectively. The sizes of breast, uterus and ovary of children and the levels of estradiol (E 2 ) were significantly reduced, and the growth rate of BA was also reduced. All the differences between pre- and post-treatment in each group were statistically significant (P < 0. 05), but the differences of the parameters between two groups were not significant (P > 0.05).

CONCLUSIONSDomestic leuprorelin is effective and safe in the treatment of Chinese girls with ICPP. Its effectiveness and safety are comparable with imported leuprorelin.

Body Height ; drug effects ; Body Weight ; drug effects ; Child ; Child, Preschool ; Female ; Follicle Stimulating Hormone ; blood ; Gonadotropin-Releasing Hormone ; blood ; Humans ; Leuprolide ; adverse effects ; therapeutic use ; Luteinizing Hormone ; blood ; Puberty, Precocious ; blood ; drug therapy ; Treatment Outcome