Objective Congenital nephrogenic diabetes insipidus (CNDI) is a rare disease, the aim of this article is to help better understanding of this disease. Methods The clinical features, genetic analysis and treatments of two siblings with CNDI were retrospectively analyzed, and related literatures were reviewed. Results Both brothers had polydispia, polyuria and low concentrate urine continuously, and they both had a mutation in AQP 2 conifrmmed with Sanger sequencing. This novel frame shift mutation caused arginine of 254 to histidine, and prolonged AQP 2 protein. Conclusions Gene analysis can help diagnosis of CNDI. Amiloride is useful option for treatment.

Objective To investigate intraoperative changes of internal environment in infants undergoing living related liver transplantation (LRLT), and to explore appropriate treatment measures. Methods Twenty-five infants undergoing LRLT were retrospectively studied, including 12 males, 13 females, with age of (3. 4 ± 4. 6) months (ranging from 2-11 months), weight of (6. 8 ±1. 3) kg (ranging from 3. 1-8. 8 kg). Arterial blood samples were collected before the operation, at preanhepatic phase (5 min before cross-clamping), at anhepatic phase (5 min before opening inferior vena cava), 5 and 30 min after the opening inferior yena cava respectively, and at the completion of the surgery the pH value, bases excess (BE), the levels of sodium, potassium, calcium, glucose and lactate were determined. Results There were large fluctuations to the internal environment during operation. Compared with the preoperative values, the intraoperative concentrations of Na+ had no significant change; The pH value and blood level of K+ had no significant change at pre-anhepatic phase and anhepatic phase (P＞0. 05), the pH value was decreased at anhepatic phase Ⅰ (P＜0. 01 )and returned to the preoperative level at the end of the operation, and the blood level of K+ decreased at anhepatic phase and lasted till the completion of the surgery (P＜0. 01 ). The blood level of Ca2+ was decreased at pre-anhepatic phase and neohepatic phaseⅡ (P＜0. 05), and recovered at the end of the operation. Blood glucose concentration was increased significantly at preanhepatic phase to neohepatic Ⅱ, and still kept at the higher level until the end of operation. The lactate concentrations were increased significantly at pre-an.hepatic phase to neohepatic Ⅱ (P＜0. 01 ), and recovered at the end of operation. The BE was decreased at pre-anhepatic phase to neohepatic Ⅱ (P＜0. 05), and recovered at the end of the operation. Conclusion There are significant disruptions which are unique and inter-related to the internal environment parameters in infants during the operation of LRLT.Monitoring and accurate intraoperative managements for different physiological status at different phases are critical for the success of LRLT in infants.

Objective To explore the predictive value of serum soluble fms-like tyrosine kinase-1 (sFlt-1) and vascular endothelia growth factor (VEGF) levels in preeclampsia at second trimester. Methods Serum sFlt-1,VEGF concentrations were determined in 172 initial normal pregnant women at 26-28 gestation week.The outcomes of pregnancies were followed.In a cohort of 172 pregnant women, 16 cases of preeclampsia were developed (preeclampsia group),and 156 cases were with no complication (control group).Results The serum levels of sFlt-1 in preeclampsia group (11.4?6.2)?g/L were significantly higher than that in control group(4.5?2.1)?g/L(P＜0.01).The serum levels of sFlt-1 in precelampsia women with the onset before 32 gestation week and fetal growth retardation,(14.0?6.8)?g/L,(14.4?6.7)?g/L were significantly higher than that in women with the onset after 32 gestation week and with no fetal growth retardation (9.0?4.1)?g/L,(8.9?4.0)?g/L,respectively (P＜0.05).There was no significant difference in the serum levels of VEGF between preeclampsia group and control group.A sFlt-1 cutoff value of 8.75?g/L at 26-28 gestation week yielded a sensitivity of 87.5%,specificity of 97.4%,positive predictive value of 80.0%,negative predictive value of 88.5%,respectively,for subsequent onset of preeclampsia.Conclusion Maternal serum sFlt-1 concentration at second trimester can be used as an early predictive marker of preeclampsia.

To study the level of macrophage migration inhibitory factor (MIF) in serum and the expression of MIF mRNA in abdominal subcutaneous adipose tissue,and to investigate its impact on insulin resistance and islet β-cell dysfunction in gestational diabetes mellitus (GDM).120 pregnancy women from the Affiliated Hospital of Qingdao University Medical College and Taian Central Hospital were enrolled,including 60 GDM women and 60 women with normal glucose tolerance (NGT).The serum MIF in GDM group was higher than that of NGT group [(3.58±1.02 vs 1.23±0.62) ng/ml,P<0.01].Multiple stepwise regression analysis showed that body mass index was an independent affective factor of the serum levels of MIF (r2 =0.516).The serum levels of MIF and the expressions of MIF mRNA in abdominal subcutaneous adipose tissue were significantly higher in GDM group than NGT group.MIF may contribute to insulin resistance and β-cell dysfunction in GDM.Body mass index seems to be an independent factor in affecting the serum levels of MIF.

Objective To learn the status quo of core competency of registered nurses in tertiary level first-class hospitals in Liaoning Province and compare the level and characteristics of core competency of registered nurses in different working years in order to supply evidence to establish contrapuntal training protocol.Methods "The Competency Inventory for Registered Nurse scale"(CIRN,by LIU Ming)was distributed to investigate the core competency of registered nurses,the data underwent analysis.Results The overall score of core competency level was (174.11±31.89),the score of the nurses with 16～20 years of working experience was the highest,while the score of nurses working for 1～5 years was the lowest.The core competency varied with working years,evident difference existed in different dimensions and total scores.Conclusions The overall core competency is at the upper middle level for registered nurses from five tertiary level first-class hospitals in Liaoning Province.They are competent enough to provide health care.With the increasing length of working time,nurses' core competency level will strengthen gradually.

Objective To evaluate the role of 5-HT5A receptors (5-HT5A R) in activation of astroglia in the spinal dorsal horn in a rat model of neuropathic pain induced by vincristine. Methods Forty adult male SD rats weighing 180-200 g were randomly divided into 4 groups ( n = 10 each): control group (group C);neuropathic pain group (group P);Ad-X-HK group (group B) and Ad-5-HT5A-siRNA group (group S). Neuropathic pain was induced by repeated intraperitoneal (IP) injection of vincristine 0.1 mg/kg according to the method described by Weng et al in group P, B and S. On the 2nd day after the last IP injection, the animals received artificial cerebrospinal fluid, Ad-X-HK and Ad-5-HT5A-siRNA 25 μl administered intrathecally (IT) in group P, B and S respectively. Paw withdrawal threshold to mechanical stimulus was measured before and on the 7th day after IT administration. The animals were then sacrificed. The lumbar segment ( L4.5 ) of the spinal cord was removed for determination of 5-HT5A R and GFAP expression. Results Body weight and paw withdrawal threshold were significantly decreased after repeated IP vincristine administration in group P compared with group C. IT Ad-5-HT5A-siRNA reduced pain threshold further in group S compared with group P. Repeated IP vincristine significantly increased the expression of 5-HT5A R and GFAP in spinal dorsal horn, and IT Ad-5-HT5A-siRNA significantly decreased the expression of 5-HT5A R while increased the expression of GFAP in spinal dorsal horn in group S compared with group P. Conclusion 5-HT5AR is involved in the inhibition of astrocyte activation, resulting in reduction of vincristineinduced neuropathic pain.

Objective To establish a UV spectrophotometry method and an HPLC method respectively for the determination of the total content of coumarin and contents of four main constituents of coumarin in Fraxini Cortex extract.Methods UV spectrophotometry was used for the determination of the content of total coumarin in Fraxini Cortex extract. The reference substance was Aesculin, and the maximum ultraviolet absorption wavelength was 334 nm. The HPLC method was used to determine the contents of Aesculin, Fraxin, Aesculetin and Fraxetin in Fraxini Cortex extract, using gradient elution with acetonitrile-phosphate solution (0.01%) as mobile phase on Agilent ZORBAX SB-C18 chromatographic column (4.6 mm × 250 mm, 5μm) at room temperature.Results For the UV method, the linear range of the mass concentration of Aesculin was 5.76-23.04μg/mL (r=0.999 9), and the average recovery was 100.6% (RSD=1.8%). For the HPLC method, the linear ranges of the mass of Aesculin, Fraxin, Aesculetin and Fraxetin were 0.055 0-3.850 0μg (r=0.9997), 0.053 9-3.773 0μg (r=0.999 8), 0.060 0-0.660 0μg (r=0.999 9), and 0.056 2-0.618 2μg (r=0.999 9), respectively, and the average recoveries were 96.97% (RSD=1.26%), 100.80% (RSD=2.22%), 99.04% (RSD=2.47%), and 98.77% (RSD=1.94%), respectively.Conclusion Both of the two methods are simple, accurate and reliable, and can be used for the quality control of total coumarin and the main constituents of coumarin in Fraxini Cortex extract.

Objective To analyze MRI imaging features of patients with Wernicke encephalopathy after major surgical operation.Methods Clinical data including cranial MRI features were reviewed on five patients of Wernicke encephalopathy after major surgical operation from Jan 2012 to Nov 2013.Results The clinical features of patients with Wernicke encephalopathy after major surgical operation were noncharacterized and most of them had no specific value for diagnosis except for a definite history of vit B1 insufficiency.MRI imaging showed abnormal signal in the medial thalamus,third ventricle,surrounding area of the aqueduct of midbrain,column of fornix and the dorsal midbrain and pons ; Lesions were showed with long T1 and long T2 signal intensity and high signal intensity on fluid-attenuated inversion recovery (FLAIR),and high signal intensity on DWI(b equals 1 000 s/mm2).ADC value in all lesions increased from [(0.46-0.59) ×10-3 mm2/s] before treatment to [(0.81-1.10) ×10-3 mm2/s] after vit B1 supplement.Conclusions Wernicke encephalopathy has typical MRI imaging presentations.Early diagnosis and timely supplement of vitamin B1 can improve the prognosis.

Objective To preliminarily explore the role of serum procalcitonin (PCT) quantization in differntiating coagulase-negative staphylococci (CNS) blood stream infection and contamination derived from blood culture in children. Methods Clinical data of 83 cases of CNS derived from blood culture in People’s Hospital of Changning city, Hunan province between January, 2013 and December, 2013 were retrospectively reviewed. The cases were divided into blood stream infection group and contamination group. The basic demographic characteristics, origins of patients, C reactive protein, time to positivity of blood culture (TP), procalcitonin were compared between the two groups, and the diagnostic performance of PCT according to the ROC curve were analyzed. Results Age, gender, C reactive pretein and TP showed no difference between blood stream infection group (n=38) and contamination group (n=45)(P>0.05), while the proportion of contamination of patients from medical wards was higher than that from surgery wards (P<0.05). PCT was signiifcantly higher in blood stream infection group than that of contamination group (P<0.001). The diagnostic sensitivity, spectivity, accuracy of PCT in differentiating the two groups were 86.8%, 82.2%and 84.3%, respectively, at the best cutoff of 0.195 ng/ml. Conclusions PCT may be a good marker for differentiating CNS blood stream infection and contamination derived from blood culture in children, and may help clinicians make early and reasonable selection of antibiotics.

Objective To report clinical and laboratory features of a rare case who was primary myelofibrosis (PMF) with positive JAK2 V617F terminating in T-acute lymphoblastic leukemia (T-ALL).Methods A rare case of PMF terminating in ALL was found, the quantity and quality of JAK2 gene mutation were defined by PCR methods, collected the clinical dates. Results The JAK2 V617F gene mutations were all positive pre and post-blast, the mutant rates respectively were 71.21% and 83.56 %. The interval from PMF to leukemia was 22 months, and from leukemia to death was 27 days. Conclusion PMF with positive JAK2 V617F terminating in ALL is a rare case, clinical prognosis is poor and survival time is short.