Objective To investigate the iodine nutrition level of vulnerable people in Inner Mongolia after adjustment of iodized salt standard and to provide theoretical bases for scientific iodine supplementation. Methods In 2013, 3 cities were selected from eastern, central and western parts of Inner Mongolia in accordance with the random number table, 3 or 4 counties were selected from each target city, 5 units according to their sub-area position of east, south, west, north and center were selected from each county, and then 1 township was selected from each unit, 5 groups of target population including school children aged 8- 10, women of childbearing age, pregnant and lactating women and infants each at least 10 people were investigated in each township. Edible salt samples from their homes and urine samples were collected. The direct titration method among the generic methods of iodide testing for salt production industry (GB/T 13025.7-2012) was used to determine the salt iodine level, and As3+-Ce4+catalytic spectrophotometry using ammonium per sulfate digestion (WS/T 107-2009) was used to test the urinary iodine level. Results Totally 3 300 samples of edible salt from local residents had been examined and median iodine was 26.20 mg/kg. The median of urinary iodine was 190.6μg/L of 1 289 school-age children;was 183.6μg/L of 621 women of childbearing age; was 178.2 μg/L o f 876 pregnant women; was 178.6 μg/L of 664 lactating women and was 167.7μg/L of 599 infants. Conclusion After adjustment of iodized salt standard, iodine nutrition level is suitable in all vulnerable people.

Objective:To study the genotyping characteristics of Brucella strains isolated from Lianyungang City (non-brucellosis epidemic area) of Jiangsu Province. Methods:Preliminary identification of 13 suspected strains of Brucella isolated from blood culture in Clinical Microbiology Laboratory of the First People's Hospital of Lianyungang City in 2018 was conducted; at the same time, the specific gene bcsp31 and insertion sequence IS-711 of Brucella were detected by quantitative real-time PCR (Real-time PCR), and the identification results were rechecked and typed. Multiple locus variable-number tandem repeat analysis (MLVA) was applied for genotyping, and the sequencing results were edited by Mega 4.0 software. Results:All the 13 strains were identified as Brucella by preliminary identification. Real-time PCR confirmed that all the 13 strains were Brucella melitensis. The results of MLVA showed that 13 strains of Brucella melitensis were divided into 12 genotypes and clustered in the "middle Mediterranean cluster". Among 13 strains of Brucella melitensis, 3 strains were biovar 1, 2 strains were biovar 2 and 8 strains were biovar 3. Conclusion:All the Brucella strains isolated from Lianyungang City are Brucella melitensis and the MLVA cluster is in the "middle Mediterranean cluster".

Objective:To explore the value of the age, atrial fibrillation, dysphagia, sex, stroke severity (A2DS2) score, the prestroke independence, sex, age, National Institutes of Health stroke scale (ISAN) score, acute ischemic stroke-associated pneumonia score (AIS-APS), and intracerebral hemorrhage associated pneumonia score without hematoma volume included (ICH-APS-A) in predicting risk of stroke-associated pneumonia (SAP).Methods:From January to June 2019, 304 patients with acute stroke who were hospitalized in the Lianyungang Hospital Affiliated to Xuzhou Medical University were analyzed retrospectively. There were 164 patients with acute ischemic stroke (AIS), including 82 patients with SAP. And there were 140 patients with intracerebral hemorrhage (ICH), including 70 patients with SAP. They were divided into SAP group (152 cases) and non-SAP group (152 cases) depending on whether they had SAP. The area under the receiver operating characteristic curve (AUC) was used to compare the predictive value of the four scores.Results:When predicting risk of SAP in patients with the stroke, the A2DS2 score had the largest AUC compared to the ISAN score, AIS-APS score, and ICH-APS-A score. When predicting risk of SAP in patients with AIS, the AUC (0.875, 95% CI 0.815 to 0.922) of the A2DS2 score was greater than the AIS-APS score and the ISAN score. When predicting risk of SAP in patients with ICH, the AUC (0.950, 95% CI 0.900 to 0.980) of the A2DS2 score was greater than the ICH-APS-A score and the ISAN score. When predicting risk of SAP in patients with AIS and ICH: 0.911 (95% CI 0.873 to 0.94) vs. 0.882 (95% CI 0.840 to 0.916), Z = 2.319, P = 0.020, the A2DS2 score was significantly better than the ISAN score ( P<0.05). When predicting risk of SAP in patients with AIS, the A2DS2 score, ISAN score, and AIS-APS score all have good predictive value ( P>0.05). When predicting SAP in patients with ICH, the A2DS2 score, ISAN score, and ICH-APS-A score all have good predictive value ( P>0.05). Conclusions:When predicting risk of SAP in patients, the A2DS2 score is a reliable prediction tool, with good predictive value.

OBJECTIVE: To explore the clinical and genetic characteristics of a patient with hypertrophic cardiomyopathy as the initial manifestation of Mucopolysaccharidosis type Ⅲ A (MPS Ⅲ A). METHODS: A female patient with MPS Ⅲ A who was admitted to the Affiliated Hospital of Jining Medical University in January 2022 and her family members (seven individuals from three generations) were selected as the study subjects. Clinical data of the proband were collected. Peripheral blood samples of the proband was collected and subjected to whole exome sequencing. Candidate variants were verified by Sanger sequencing. Heparan-N-sulfatase activity was determined for the disease associated with the variant site. RESULTS: The proband was a 49-year-old woman, for whom cardiac MRI has revealed significant thickening (up to 20 mm) of left ventricular wall and delayed gadolinium enhancement at the apical myocardium. Genetic testing revealed that she has harbored compound heterozygous variants in exon 17 of the SGSH gene, namely c.545G>A (p.Arg182His) and c.703G>A (p.Asp235Asn). Based on guidelines from the American College of Medical Genetics and Genomics (ACMG), both variants were predicted to be pathogenic (PM2_Supporting +PM3+PP1Strong+PP3+PP4; PS3+PM1+PM2_Supporting +PM3+PP3+PP4). Sanger sequencing confirmed that her mother was heterozygous for the c.545G>A (p.Arg182His) variant, whilst her father, sisters and her son were heterozygous for the c.703G>A (p.Asp235Asn) variant. Determination of blood leukocyte heparan-N-sulfatase activity suggested that the patient had a low level of 1.6 nmol/(g·h), whilst that of her father, elder and younger sisters and son were all in the normal range. CONCLUSION The compound heterozygous variants of the SGSH gene probably underlay the MPS ⅢA in this patient, for which hypertrophic cardiomyopathy is an associated phenotype.
Female ; Humans ; Cardiomyopathy, Hypertrophic ; Contrast Media ; East Asian People ; Gadolinium ; Mucopolysaccharidosis III ; Mutation ; Pedigree ; Male ; Middle Aged

Objective:To investigate the correlation of monocyte/lymphocyte ratio (MLR) with the prognosis and adverse event in critically ill patients.Methods:Basic information of patients were extracted from Medical Information Mart for Intensive Care-Ⅲ (MIMIC-Ⅲ), including demographics, blood routine, biochemical indexes, systemic inflammatory response syndrome score (SIRS), sequential organ failure assessment (SOFA) score, and outcome, etc. MLR on the first day of intensive care unit (ICU) admission was calculated. The receiver operating characteristic curve (ROC curve) was applied to evaluate the prognostic value of MLR on the 30-day mortality and its cut-off value. According to the cut-off value, the patients were divided into two groups, and the differences between the groups were compared. Logistic regression model was used to analyze the relationship of MLR with 30-day mortality, continuous renal replacement therapy (CRRT), mechanical ventilation, the length of ICU stay, and total hospitalization time.Results:① A total of 43 174 critically ill patients were included. ROC curve showed that area under ROC curve (AUC) of MLR in predicting 30-day mortality was 0.655 [95% confidence interval (95% CI) was 0.632-0.687]. The cut-off value of MLR calculated according to the maximum Yoden index was 0.5. There were 16 948 patients with MLR ≥ 0.5 (high MLR group) and 26 226 patients with MLR < 0.5 (low MLR group). ② Compared with the low MLR group, the high MLR group had higher age, proportion of male, body mass index (BMI) [age (years old): 66.0 (51.7, 78.4) vs. 57.6 (27.1, 74.6), proportion of male: 57.2% vs. 52.5%, BMI (kg/m 2): 26.5 (22.5, 31.1) vs. 24.7 (14.3, 29.7)]. The high MLR group also had higher incidence of complications (hypertension: 49.2% vs. 44.6%, chronic heart failure: 32.6% vs. 21.7%, diabetes mellitus: 27.0% vs. 23.4%, chronic obstructive pulmonary disease: 21.5% vs. 16.1%, renal insufficiency: 19.3% vs. 13.1%), and higher white blood cell count (WBC), blood glucose, lactate (Lac), serum creatinine (SCr), SIRS score and SOFA score [WBC (×10 9/L): 13.8 (9.6, 19.2) vs. 11.5 (8.4, 15.6), blood glucose (mmol/L): 8.66 (6.88, 11.49) vs. 8.27 (6.55, 10.88), Lac (mmol/L): 2.2 (1.5, 3.7) vs. 2.1 (1.4, 3.3), SCr (μmol/L): 106.1 (70.7, 176.8) vs. 88.4 (70.7, 132.6), SIRS score: 3 (2, 4) vs. 2 (2, 3), SOFA score: 4 (2, 7) vs. 3 (1, 5)]. The 30-day mortality, and the proportion of patients with length of ICU stay > 5 days, total hospitalization time > 14 days, CRRT and mechanical ventilation > 5 days were significantly higher in high MLR group (30-day mortality: 20.0% vs. 8.3%, length of ICU stay > 5 days: 33.2% vs. 20.4%, total hospitalization time > 14 days: 33.7% vs. 16.2%, CRRT: 3.6% vs. 0.7%, mechanical ventilation > 5 days: 18.4% vs. 5.7%), with statistically significant differences (all P < 0.05). ③ After adjusted with the related factors, multivariate Logistic regression analysis showed that elevated MLR was an independent risk factor for increased 30-day mortality [odd ratio ( OR) = 1.54, 95% CI was 1.37-1.72, P < 0.001]. Moreover, the increased MLR was independently associated with the increased risk of usage of CRRT ( OR = 2.77, 95% CI was 2.18-3.51), mechanical ventilation > 5 days ( OR = 2.45, 95% CI was 2.21-2.72), the length of ICU stay > 5 days ( OR = 2.29, 95% CI was 2.10-2.49), and total hospitalization time > 14 days ( OR = 2.28, 95% CI was 2.08-2.49), all P < 0.001. Conclusions:Retrospective analysis of large sample shows that MLR elevation is an independent risk factor for 30-day mortality, usage of CRRT, prolonged mechanical ventilation time, prolonged hospitalization, prolonged length of ICU stay. MLR can be used for risk stratification of severe patients.