OBJECTIVE:To provide reference for improving the level of rational drug use in public hospitals at county level. METHODS:The relevant indicator data of rational drug use in our hospital was analyzed,and the relevant indicator data of the hos-pitals at county level around it was investigated and analyzed,a total of 36 parts of rational drug use management indicator system was established,including administrative management indicators,outpatient management indicators,hospital management indica-tors,and drug procurement management indicators,and it was constantly improved and optimized. Meanwhile,relevant indicator data of rational drug use from 2010 to 2014 was statistically analyzed to evaluate the effect of the effectiveness of the system. RE-SULTS:In the 5 years,the drug revenues accounted for revenue ratio(the ratio of drug)decreased by 10.3%;the use indicators of antibiotics in clinic showed gradual optimization,the proportion of outpatient antibiotic prescriptions decreased from 57.1% to 19.3%,the use rate of inpatient antibiotic decreased from 90.2% to 56.3%,the proportion of type I incision operation preventive use of antibiotics decreased from 94.6% to 28.6%,and the submission rate of microbiological sample of inpatients before they re-ceived the treatment of antibiotics increased from 8.4% to 32.7%;the proportion of essential medicines increased 25.5%,and pro-portion of use amount increased to 24.1%;the application of the system promoted the improvement of rational drug use and phar-macy management level in our hospital,and also helped the hospital successfully passed the national three class comprehensive hos-pital evaluation. CONCLUSIONS:The established rational drug use management indicator system is comprehensive and feasible, and can provide reference for strengthening rational drug use management and medical and health system decision in China.

Objective:To study the characteristics of the genotype and phenotypic in a family with X-linked retinoschisis (XLRS) due to RS1 mutation. Methods:A retrospective clinical study. An XLRS family of 4 generations of 26 people were included in the study. Among them, 8 participants were males and 7 participants were females. Routine ophthalmologic examination was performed on 3 patients in the family including the proband and 12 patients with normal phenotype. Optical coherence tomography was performed in 2 of the 3 patients. Peripheral venous blood was extracted from all participants, whole-genome DNA was extracted, and potential pathogenic genes were screened by Panel sequencing. Conservative analysis, pathogenicity analysis and protein structure prediction were carried out by software tools. The pathogenicity of gene mutations was analyzed according to the American Society of Medical Genetics and Genomics (ACMG) guidelines.Results:The proband was 3 years old. Optical coherence tomography (OCT) examination showed that the retinal core layer in the macular area of both eyes had a cystic change, which was segmented by vertical or oblique bridging tissue. The proband's uncle was 32 years old. OCT examination showed atrophy in the macular area of the left eye. The macular area of the right eye was cystoid, segmented by vertical or oblique bridging tissue. No abnormality was found in the fundus examination of the proband's parents and 10 members of his family. Panel sequencing showed that c.361C>T/ p.Q121X hemizygous mutation was found in the fifth exon of RS1 gene in the proband (Ⅳ3) and 2 patients (Ⅱ1, Ⅲ8). The mother was a heterozygous mutation carrier of the gene, while the father had no mutation. The mutant gene causes premature termination of RS1, a truncated protein encoding 224 amino acids to 120 amino acids. Of the 10 patients with normal fundus examination, 6 participants were normal. The mutation was carried by four people, which were women. Homology analysis of the protein sequence showed that the mutant site was highly conserved in 12 mammals. Three-dimensional structural analysis of RS1 protein showed that the c-terminal amino acid sequence of the mutant protein was more than 50% missing. Analysis of ACMG guidelines indicated that the mutation was pathogenic. Conclusion:The RS1 mutation site c.361C>T/p.Q121X is a new mutation site of XLRS.

Objective:To study the clinical characteristics of congenital tuberculosis (CTB).Methods:From June 2016 to February 2021, the clinical data of infants with CTB admitted to the neonatal department of our hospital were retrospectively analyzed, including their clinical characteristics, diagnosis, treatment and prognosis.Results:A total of 7 infants with CTB were enrolled into the study. 4 mothers had in vitro fertilization embryo transfer (IVF-ET) due to tubal obstruction and 2 mothers who were diagnosed of tubal tuberculosis. The 7 infants included 1 male and 6 female, 6 preterm and 1 full-term. The median age of onset was 18 d (7~30 d).All of the 7 infants had fever, 6 had shortness of breath and poor response, 5 had apnea, 3 were coughing, 1 showed pale skin colour, 1 had bilateral rales in the lung, 6 had hepatomegaly, 3 splenomegaly and 1 lymphadenopathy. Chest X-ray showed patchy exudation in 3 infants, miliary pattern of opacification in 2 infants, patchy opacification in 1 infant, bilateral increased lung markings in 1 infant. The positive rate of acid-fast bacilli (AFB) was 50% in all sputum samples. Specimens from glottic and subglottic area showed higher positive rate than oropharyngeal specimen. PCR MTB-DNA tests of sputum samples were positive in all 7 cases. 1 infant received invasive respiratory support for 1 d and died after discharge. 2 infants improved significantly after initial treatment, received 1~2 months of anti-TB therapy outside the hospital and died of severe respiratory failure. Of the 4 infants survived, 1 was cured and 3 significantly improved.Conclusions:Mothers with tubal tuberculosis receiving IVF-ET may cause high risk of CTB. Sputum AFB test and sputum PCR MTB-DNA test are preferred diagnostic tests. Genetic test of drug-resistant TB may guide clinical drug use. Glottic or deeper throat specimens can increase the positive rate.

Objective To investigate the prevalences of the obesity and hypertension in southern mountain regions of Ningxia. Methods A cross-sectional study was conducted among a representative sample of 10 639 adults using questionnaires, physical examinations, and blood pressure measurement in southern mountain areas with a population proportionate sampling method. Results The prevalences of overweight,obesity,central obesity,high percentage of body fat,and hypertension were 33.53%,10.71%,19.50%,27.69%,and 31.57% respectively, which were 30.31%,9.62%,16.70%,24.90%,and 27.61% after age-adjustment in rural areas of Ningxia,and increased with aging(Ptrend<0.05). The prevalences of overweight,obesity,and hypertension were higher in males than those in females(P<0.05),and that of central obesity was higher in females than in males(P=0.003).The prevalences of hypertension in subjects with overweight, obesity, central obesity, high percentage of body fat were 38. 14%, 53.75%,52.69%,and 48.90%,respectively. Body mass index,waist circumference,and percentage of body fat were positively correlated with systolic and diastolic blood pressure(P<0.05). The multivariable logistic model revealed that the risk of hypertension in different types of obesity increased about 1.5 times. Conclusion There is high prevalence of obesity and hypertension among the adults in southern mountain areas of Ningxia. The prevalence of hypertension in obesity,central obesity,and high percentage of body fat is closed to or more than half of the population investigated.

Objective:The study aimed to evaluate the effect of systemic-pulmonary shunt(SPS) on the infants with tetralogy of Fallot(TOF) with hypoplasia pulmonary arteries.Methods:Among 949 infants less than three-year-old who underwent surgical intervention between January 2010 and December 2015, 853 infants underwent one-stage primary repair(group Ⅰ), whereas 96 infants underwent SPS(group Ⅱ). Among them, 546 were males and 403 were females. The average age and average weight at operation were 13 months and 9.4 kg, respectively. 20.8% infants had clinical symptoms before operation. Perioperative and follow-up parameters were assessed including age, weight, sex, transcutaneous oxygen saturation(SPO 2), preoperative clinic symptom, ratio of McGoon, NAKATA index, Z score of pulmonary annulus, left ventricular end diastolic diameter(LVEDD). Results:There were 28(3.0%) early postoperative deaths in the whole group. And no significant difference in mortality was found between group Ⅰ(2.9%) and group Ⅱ(3.1%). Compared with group Ⅰ, age and weight were significantly lower in patients in group Ⅱ[(0.95±0.56)years old vs.(1.09±0.59)years old] and[(8.6±1.8)kg vs.(9.5±2.1)kg], respectively. Patients in group Ⅱ had a lower SPO 2(0.75±0.09 vs. 0.85±0.09) and was more prone to appear hypoxic spell(30.2% vs. 19.7%) than patients in group Ⅰ. Patients in group Ⅱ had a more poor development in pulmonary artery. The ratio of McGoon, NAKATA index and Z score of pulmonary annulus in group Ⅱ were significantly less than that in group Ⅰ[1.4±0.3 vs. 1.9±0.5, (124±43)mm 2/m 2 vs.(222±88)mm 2/m 2 and-4.3±2.6 vs.-2.3±2.1], respectively. There was no difference of extubation time bewteen two groups. SPO 2 rose significantly from 0.75 to 0.83 after SPS. And after SPS, the diameter of main pulmonary artery, the ratio of McGoon, NAKATA index and Z score of pulmonary annulus increased significantly from 6.4 mm, 1.42, 126.4 mm 2/m 2 and -4.3 to 9.2 mm, 1.83, 212.6 mm 2/m 2 and -2.4, respectively. Moreover, LVEDD also increased significantly from 21.2 mm to 24.5 mm after SPS. Conclusion:SPS is safe and effective for little infants with anoxic spell and hypoplasia pulmonary arteries. SPS can improve oxygenation significantly and delay the age for radical surgery. SPS also promote the growth of the pulmonary arteries, pulmonary valve annulus and left ventricular, and it facilitates the preservation of pulmonary valve annulus and reduce the rate of transannular patching in a portion of infants.

Objective To investigate the prevalence of nonalcoholic fatty liver disease and the risk factors in Jingyuan county of Ningxia Autonomous Region.Methods The population proportionate sampling method was applied to enroll a representative sample of 10 639 adults in Jingyuan county and the study was conducted using questionnaires and physical examinations.A total of 10 553 people were included in the analysis after excluding those with missing data.High-resolution ultrasound was used to examine the liver and fasting blood was collected in the morning for measurement of blood glucose,blood lipid,and uric acid.The participants were divided into two groups of those with and without nonalcoholic fatty liver disease;the difference in blood biochemical indexes between fatty liver and non-fatty liver groups was compared,and the logistic regression model was used to explore the risk factors affecting the prevalence of fatty liver.Results The prevalence of nonalcoholic fatty liver disease was 7.60％.The prevalence of thyroid nodules was higher in men than in women (8.60％ vs.6.82％,x2=1 1.772,P=0.001).The prevalence rate of fatty liver increased with age (x2=57.336,P＜0.001),the prevalence rates among ≥18 years-＜29 years,≥30 years-＜39 years,≥40years-＜49 years,≥50 years-＜59 years,≥60 years-＜69 years,and above 70 years were 2.92％,6.50％,8.81％,9.59％,8.08％,and 4.77％ respectively.The detection rate of overweight,obesity,abdominal obesity,impaired fasting glucose,impaired glucose tolerance,diabetes,hypertension,hyperuricemia,and dyslipidemia were higher in nonalcoholic fatty liver disease group than in the normal group (P＜0.05).Logistic regression analysis showed that nonalcoholic fatty liver disease group had a higher risk for overweight,obesity,abdominal obesity,impaired fasting glucose,impaired glucose tolerance,diabetes,hypertension,hyperurcemia,and dyslipidemia (OR=5.41,12.45,2.99,1.85,2.05,3.30,1.41,2.23,and 1.98).Conclusion The incidence of nonalcoholic fatty liver in Jingyuan county of Ningxia Autonomous Region was higher.The groups of overweight,obesity,abdominal obesity,impaired fasting glucose,impaired glucose tolerance,diabetes,hypertension,hyperuricemia,and dyslipidemia were high risk factors for nonalcoholic fatty liver disease.