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 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 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 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.

Objective To study the effect and possible mechanism of diazoxide on myocardial ischemia/reperfusion (I/R) injury in diabetic rats, and the influence of insulin intervention which aims to maintain blood sugar levels within the normal range on the protective function of cardiomyocytes. Methods 126 health male Sprague-Dawley (SD) rats were intraperitoneally injected with one dose of 60 mg/kg streptozotocin (STZ) to reproduce diabetic model. The diabetic rats were randomly divided into seven groups, with 18 rats in each group. Myocardial I/R model was established by 30 minutes ligation of the left anterior descending branch of the coronary artery, and 120 minutes blood circulation recover. Sham group was only threaded without ligation. Rats in I/R group, diazoxide group (DZ group), and Ottawa vine penicillin (WNT) group were infused intravenously with 2 mL of 0.1% dimethyl sulphoxide (DMSO), DZ (7 mg/kg), and WNT (15 μg/kg), respectively, after 25 minutes of ischemia. Sham group was only injected with 2 mL of 0.1% DMSO. DZ+WNT group was infused with WNT 5 minutes before the injection of DZ. Insulin intervention (RI) group received a continuous insulin infusion to maintain the blood sugar at the level of 4-6 mmol/L. RI+DZ group was infused with DZ after ischemia for 25 minutes based on blood sugar control. Hemodynamic parameters in each group were monitored continuously. The pathological changes of myocardium were observed with hematoxylin and eosin (HE) staining. The expressions of phosphorylated protein kinase B (p-Akt) and phosphorylated glycogen synthase kinase-3β (p-GSK-3β) were determined by Western Blot. Results Compared with sham group, the cardiac functions of the intervention groups were significantly decreased, and severe myocardial injury was observed. Compared with I/R group, the cardiac functions of intervention groups were not obviously improved. However, after insulin intervention by which blood sugar was maintained within normal range, the cardiac function and myocardial injury were further aggravated. Compared with sham group (the expression value of sham group was set as 1), the expressions of p-Akt in other groups including I/R group, DZ group, RI group, and RI+DZ group showed no statistically significant difference (gray value: 1.07±0.09, 1.03±0.07, 1.07±0.07, 1.02±0.08 vs. 1.00, all P > 0.05). However, the expressions of p-Akt were decreased in WNT group and DZ+WNT group as compared with those of sham group and I/R group (gray value: 0.54±0.06, 0.51±0.05 vs. 1.00 and 1.07±0.09, all P < 0.05). The expressions of p-GSK-3βshowed no statistically significant difference in I/R group, DZ group, WNT group, and DZ+WNT group as compared with sham group (gray value: 0.97±0.08, 1.00±0.11, 0.98±0.06, 0.97±0.09 vs. 1.00, all P > 0.05). However, the expression of pGSK-3β was increased in RI group, RI+DZ group as compared with sham group and I/R group (gray value: 1.68±0.08, 1.70±0.05 vs. 1.00 and 0.97±0.08, all P < 0.05), and it was significantly higher in RI+DZ group than that of DZ group (gray value: 1.70±0.05 vs. 1.00±0.11, P < 0.05). Conclusions Diazoxide after myocardial injury could not protect the myocardium from I/R injury in diabetic rats, and did not trigger the activation of the phosphoinositide 3-kinase (PI3K)/Akt signaling pathway. Insulin intervention by which blood sugar was maintaine d within the normal range exacerbates myocardial I/R injury in diabetic rats.

Antidiuretic Hormone Receptor Antagonists ; Heart Failure ; drug therapy ; Humans

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.

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.