Objective To analyze the epidemiological characteristics and immunization history of pertussis cases in Yichang City, Hubei Province from 2018 to 2023. Methods Data on the incidence and immunization history of pertussis cases were collected in Yichang City from 2018 to 2023, and the epidemiological characteristics was analyzed and described. Results A total of 109 cases of pertussis were reported in Yichang from 2018 to 2023, and the annual average reported incidence rate was 0.45/100,000. The incidence rate reported in each year was between 0～1.58/100,000. The area with the highest annual reported incidence rate was Xiling District (1.19/100,000). There was a statistically significant difference in the incidence rate between different years (χ²=208.26, P < 0.001). The annual reported incidence rate showed a significant increasing trend (χ² trend =125.71, P < 0.001). The ratio of male to female cases was 1.22. There was no significant difference in the annual reported incidence rates between males and females (χ²=0.85, P=0.36). Children aged 3-9 years accounted for 60.55%. Students and scattered children accounted for 45.87% and 36.70%, respectively. Before the onset of the disease, 72.48% had a history of immunization with pertussis-containing vaccine, and 27.52% had no history of immunization. The shortest interval between the last dose of pertussis-containing vaccine and the onset of the disease was 8 days, the longest was 4057 days, and the median was 1882 days. Conclusion From 2018 to 2023, the reported incidence of pertussis in Yichang City has been on the rise, with the majority of cases occurring in children and students under the age of 9. It is recommended to strengthen pertussis disease monitoring.

Hereditary endocrine and metabolic diseases , caused by genetic factors, exhibit complex and diverse symptoms, including the possibility of concurrent sensorineural deafness. Currently, there is a limited clinical understanding of hereditary endocrine and metabolic diseases that manifest with deafness, the pathogenesis remains unclear,and there is a lack of effective diagnostic and treatment methods. This article summarizes the research progress of hereditary endocrine and metabolic diseases complicated with deafness from the pathogenesis, clinical phenotype, diagnosis and treatment. Understanding the current research progress and integrating genetic analysis into clinical practice are crucial for accurate diagnosis and treatment, evaluating clinical efficacy, and providing effective genetic counseling for these diseases.
Humans ; Deafness/genetics* ; Hearing Loss, Sensorineural/diagnosis* ; Phenotype ; Metabolic Diseases/genetics* ; Genetic Counseling

Objective:To develop and validate a predictive nomogram for 28-day mortality among very older patients with sepsis, to identify high-risk patients early and improve prognosis.Methods:This study was conducted from January 1, 2022, to November 30, 2022. Very older patients aged≥80 years with sepsis admitted to the emergency department of Beijing Chao-Yang Hospital, Capital Medical University were consecutively recruited. Their clinical data within 24 h of admission and 28-day mortality was recorded. The participants were divided into training (70%) and validation cohort (30%) (random number). In the training cohort, the risk factors of 28-day mortality were selected via least absolute shrinkage and selection operator (LASSO) regression analysis and multivariable Cox proportional hazard model, and a nomogram was developed. The prediction model was verified in validation cohort.Results:In total, 507 very older patients with sepsis were included, among which the mortality rate was 31.2%. In training cohort, the independent risk factors for 28-day mortality were identified: increased age [hazard ratio ( HR)=1.059, 95% confidence interval (95% CI)=1.017-1.103, P=0.005], cognitive impairment ( HR=2.100, 95% CI=1.322-3.336, P=0.002), frailty ( HR=2.561, 95% CI=1.183-5.545, P=0.017), decreased mean arterial pressure ( HR=0.987, 95% CI=0.976-0.998, P=0.017), decreased prealbumin ( HR=0.997, 95% CI=0.994-1.000, P=0.040), increased blood urea nitrogen ( HR=1.028, 95% CI=1.010-1.045, P=0.001), increased procalcitonin ( HR=1.008, 95% CI=1.001-1.016, P=0.019) via LASSO regression analysis and multivariable Cox regression analysis. The nomogram was developed using these seven predictors. In the training and validation cohorts, the calibration curves, time-dependent AUC curves, and decision curve analysis showed that the nomogram had good calibration degree, discrimination and clinical net benefits. Conclusions:Increased age, cognitive impairment, frailty, decreased mean arterial pressure, decreased prealbumin, increased blood urea nitrogen, and increased procalcitonin are independent risk factors for 28-day mortality in very older patients with sepsis. The nomogram, which included the seven predictors, have good predictive performance, and might be helpful for prognosis assessment.

Objective:To analyze the clinical characteristics, antibiotic resistance and prognostic risk factors of patients with Klebsiella pneumoniae bloodstream infection (Kp BSI).Methods:The clinical data of 188 patients diagnosed with Kp BSI from January 1，2017 to December 1，2021 in Beijing Shijitan Hospital, Capital Medical University were retrospectively analyzed.There were 118 patients males (62.8%) with a median age 77.0(63.0, 85.0) years old. The median length of hospital stay was 20.0 days, and 78 patients (41.5%) were admitted to intensive care unit(ICU). There were 121 cases with carbapenem-sensitive Klebsiella pneumoniae (64.4%, CSKP group) and 67 cases with carbapenem-resistant Klebsiella pneumoniae (35.6%, CRKP group).Fifty six patients died within 28 days after admission (death group), and 132 patients survived (survival group).The clinical characteristics and bacterial drug resistance of Kp BSI patients were analyzed, and univariate analysis and multivariate logistic regression analysis were used to explore factors related to the CRKP infection and patient mortality.Results:The most common infection sites were respiratory system, abdominal cavity and biliary tract accounting for 39.4% (74/188), 18.1% (34/188) and 14.4% (27/188), respectively.The common comorbidities were coronary heart disease, hypertension, chronic kidney disease and diabetes, accounting for 63.8% (120/188), 59.6% (112/188), 46.3% (87/188) and 43.1% (81/188), respectively and 118 patients (62.8%) had 3 or more comorbidities. Most patients (146 cases, 77.7%) underwent ≥1 invasive procedures before bloodstream infection;and 90 patients (47.9%) had a history of antibiotic use. CRKP strains showed higher resistance rates to piperacillin, quinolones, cephalosporins and carbapenems. Univariate analysis showed that there were statistically significant differences in age (69.0 vs. 83.0 years), ICU admission 25.6%(31/121) vs. 70.1%(47/67)], invasive procedures [67.8%(82/121) vs. 95.5 %(64/67)], and antibiotic use [37.2% (45/121) vs. 67.2%(45/67)] between the CSKP group and the CRKP group ( Z=-5.73, χ 2=35.22, χ 2=19.15, χ 2=15.53, all P<0.001). Multivariate logistic regression analysis showed that age, ICU admission, invasive procedures and antibiotic use in recent 30 days were independent risk factors for CRKP infection( OR=1.06, P<0.001; OR=3.22, P=0.003; OR=5.93, P=0.009; OR=2.40, P=0.022). The total fatality rate was 29.8%(56/188). Univariate analysis showed that there were statistically significant differences in CRKP infection [19.7%(26/132) vs. 73.2% (41/56)], albumin level (32.6 vs. 27.8 g/L) and sequential organ failure assessment score (SOFA score, 2 vs. 8 score) between the survival group and the death group (χ 2=49.10, Z=-4.64, Z=-10.36,all P<0.001). Multivariate logistic regression analysis suggested that CRKP infection, low albumin and high SOFA score on the day of bloodstream infection were risk factors for death of Kp BSI patients( OR=5.13, P=0.021; OR=0.86, P=0.044; OR=3.04, P<0.001). Conclusion:Kp BSI patients have a high fatality rate and fairly severe drug resistance. CRKP infection, low albumin, high SOFA score on the day of bloodstream infection are associated with poor prognosis in Kp BSI patients.

Objective: Comparing the benefit of Abidor, lopinavir/ritonavir and recombinant interferon α-2b triple combination antiviral therapy and lopinavir/ritonavir and interferon dual combination antiviral therapy to hospitalized novel coronavirus pneumonia 2019 in Zhejiang province. Methods: A multi-center prospective study was carried out to compare the effect of triple combination antiviral therapy with dual combination antiviral therapy in 15 medical institutions of Zhejiang Province. All patients were treated with recombinant interferon α-2b (5 million U, 2 times/d) aerosol inhalation. 196 patients were treated with abidol (200 mg, 3 times/d) + lopinavir / ritonavir (2 tablets, 1 time/12 h) as the triple combination antiviral treatment group. 41 patients were treated with lopinavir / ritonavir (2 tablets, 1 time/12 h) as the dual combination antiviral treatment group. The patients who received triple combination antiviral therapy were divided into three groups: within 48 hours, 3-5 days and > 5 days after the symptom onset. To explore the therapeutic effects of triple combination antiviral drugs and dual combination antiviral drugs, as well as triple combination antiviral drugs with different antiviral initiate time. SPSS17.0 software was used to analyze the data. Results: The time of virus nucleic acid turning negative was (12.2 ± 4.7) days in the triple combination antiviral drug group, which was shorter than that in the dual combination antiviral drug group [(15.0 ± 5.0) days] (t = 6.159, P < 0.01 ). The length of hospital stay [12 (9, 17) d] in the triple combination antiviral drug group was also shorter than that in the dual combination antiviral drug group [15 (10, 18) d] (H = 2.073, P < 0.05). Comparing the antiviral treatment which was started within 48 hours, 3-5 days and > 5 days after the symptom onset of triple combination antiviral drug group, the time from the symptom onset to the negative of viral shedding was 13 (10,16.8), 17 (13,22) and 21 (18-24) days respectively (Z = 32.983, P < 0.01), and the time from antiviral therapy to the negative of viral shedding was (11.8±3.9) , (13.5±5.1) and (11.2±4.3) d. The differences among the three groups were statistically significant (Z=32.983 and 6.722, P<0.01 or<0.05). Conclusions The triple combination antiviral therapy of Abidor, Lopinavir/Litonavir and recombinant interferon α-2b showed shorter viral shedding time and hospitalization time compared with the dual combination antiviral therapy. The earlier the time to initiate triple antiviral treatment, the shorter the time of virus shedding.

Objective:To investigate the mortality and causes of death in neonates from different medical institutions in Henan province.Methods:A retrospective analysis was performed on the death cases of 62 different medical institutions in 18 cities of Henan province in 2018, in order to compare the differences of neonatal mortality, age of death and the causes of death between maternal and child health care hospitals and general hospitals.Results:(1) A total of 80 780 newborns were admitted to 62 hospitals and 311 neonates died with a mortality rate of 3.85‰.A total of 33 339 newborns were admitted to 24 maternal and child health care hospitals, and 102 neonates died with a mortality rate of 3.06‰.Among them, 54 cases(52.9%) were premature infants and 48 cases(47.1%)were full-term infants.A total of 47 441 newborns were admitted to 38 general hospitals, and 209 neonates died with a mortality rate of 4.41‰.Among them, 111 cases(53.1%) were premature infants and 98 cases (46.9%) were full-term infants.Neonatal mortality in general hospitals was higher than that in maternal and child health care hospitals( P<0.05). (2) Neonatal death mainly occurred within one week after birth, especially within the first day.There were 67 cases of death(65.7%) in 24 maternal and child health care hospitals within the first day, including 34 cases (50.7%) of full-term infants and 33 cases (49.3%)of premature infants.And there were 87 cases of death(41.6%) in 38 general hospitals within the first day, including 50 cases (57.5%) of premature infants and 37 cases (42.5%) of full-term infants.Neonatal mortality within the first day after birth in maternal and child health care hospitals was higher than that in general hospitals( P<0.05). (3) The leading causes of neonatal death were non-infectious pulmonary diseases(128 cases, 41.2%), followed by birth asphyxia(73 cases, 23.5%) and infection(51 cases, 16.4%), but the causes of death in sequence varies from maternal and child health care hospitals and general hospitals.(4) For early death (within one week after birth) in both general hospitals and maternal and child health care hospitals, the main causes were birth asphyxia for full-term neonates, and pulmonary diseases(mainly respiratory distress syndrome)and birth asphyxia for premature infants.For late-stage death (2-4 weeks after birth) of neonates, infection was the leading cause in both term and preterm infants in general hospitals.For maternal and child health hospitals, the main causes of death for full-term infants were infection, and pulmonary diseases (mainly pulmonary hemorrhage and respiratory distress syndrome) for premature infants. Conclusion:There are some differences between maternal and child health care hospitals and general hospitals in neonatal mortality, mortality within the first day after birth, and causes of death.Therefore, it is necessary to strengthen the corresponding software and hardware constructions for newborns in different medical institutions to further reduce the neonatal mortality rate.

Objective: To execute a hierarchical cluster of clinical laboratory indicators in patients with bunyavirus infection. Methods: From July 2015 to July 2017, 14 patients with bunyavirus infection in Zhoushan Hospital were selected.The blood routine, coagulation function and biochemical indicators were detected.Cluster analysis and grouping were carried out by hierarchical clustering method. Results: Hierarchical clustering classification was eventually divided into 2 cases of A category[with TT high and BNP high as the main characteristics(TThighBNPhigh)] and 12 cases of B category[with TT low and BNP low as the main characteristics (TTlowBNPlow)]. The days of hormone drugs and dosage of hormone drugs in A category were (7.43±3.53)d, (489.19±173.02)mg, respectively, which were higher than those in B category[(5.20±1.03)d and (115.11±46.58)mg], the differences were statistically significant(t=2.76, 55.56, all P<0.05). Conclusion It needs probably to pay more days and dose of hormonal drugs for patients with TThighBNPhigh.

Objective:To investigate the clinicopathological characteristics of non-Hodgkin lymphoma (NHL) in Shandong province.Methods:The clinicopathological data of 2 886 NHL cases in Shandong Cancer Hospital from January 2002 to December 2017 were retrospectively analyzed, the clinicopathological characteristics of patients were summarized and compared with other regions in China and abroad.Results:The median age of all NHL cases was 52 years old (4-90 years old), and the ratio of male to female was 1.57∶1. The subtypes distribution analysis revealed that B-cell NHL (B-NHL) accounted for 66.7% (1 925 cases) of all cases and T-cell NHL (T-NHL) accounted for 27.3% (788 cases) of all cases. The common subtypes were diffuse large B-cell lymphoma (36.0%, 1 039/2 886), NK/T-cell lymphoma (8.8%, 254/2 886), follicular lymphoma (8.2%, 237/2 886) and peripheral T-cell lymphoma (7.4%, 214/2 886). Of all the cases, the nodal lymphomas accounted for 45.8% (1 322 cases) and the extra nodal lymphomas accounted for 54.2% (1 564 cases); there were 389 patients (13.5%) with stage Ⅰ, 678 patients (23.5%) with stage Ⅱ, 975 patients (33.8%) with stage Ⅲ, and 722 patients (25.0%) with stage Ⅳ. The distribution of NHL subtypes in Shandong province was consistent with the domestic multicenter study. However, T-NHL subtype ratio was significantly higher than the foreign studies.Conclusions:The overall incidence of NHL in Shandong province of China is dominated by middle-aged people, and the proportion of B-NHL is higher than that of T-NHL. The distribution of NHL subtypes in Shandong province of China are different from those in the European and American countries, but are roughly consistent with the domestic multicenter study.

Objective:To compare the efficacy of the combination of abidol, lopinavir/ritonavir plus recombinant interferon α-2b (rIFNα-2b) and the combination of lopinavir/ritonavir plus rIFNα-2b for patients with COVID-19 in Zhejiang province.Methods:A multicenter prospective study was carried out to compare the efficacy of triple combination antiviral therapy and dual combination antiviral therapy in 15 medical institutions of Zhejiang province during January 22 to February 16, 2020. All patients were treated with rIFNα-2b (5 million U, 2 times/d) aerosol inhalation, in addition 196 patients were treated with abidol (200 mg, 3 times/d) + lopinavir/ritonavir (2 tablets, 1 time/12 h) (triple combination group) and 41 patients were treated with lopinavir/ritonavir (2 tablets, 1 time/12 h) (dual combination group). The patients who received triple combination antiviral therapy were further divided into three subgroups: <48 h, 3-5 d and >5 d according the time from the symptom onset to medication starting. The therapeutic efficacy was compared between triple combination group and dual combination group, and compared among 3 subgroups of patients receiving triple combination antiviral therapy. SPSS 17.0 software was used to analyze the data.Results:The virus nucleic acid-negative conversion time in respiratory tract specimens was (12.2±4.7) d in the triple combination group, which was shorter than that in the dual combination group [(15.0±5.0) d] ( t=6.159, P<0.01). The length of hospital stay in the triple combination group [12.0 (9.0, 17.0) d] was also shorter than that in the dual combination group [15.0 (10.0, 18.0) d] ( H=2.073, P<0.05). Compared with the antiviral treatment which was started within after the symptom onset of in the triple combination group, the time from the symptom onset to the viral negative conversion was 13.0 (10.0, 17.0), 17.0 (13.0, 22.0) and 21.0 (18.0, 24.0) d in subgroups of 48 h, 3-5 d and >5 d, respectively ( Z=32.983, P<0.01), while the time from antiviral therapy to viral negative conversion was (11.8±3.9), (13.5±5.1) and (11.2±4.3) d, respectively( Z=6.722, P<0.05). Conclusions:The triple combination antiviral therapy of abidol, lopinavir/litonavir and rIFNα-2b shows shorter viral shedding time and shorter hospitalization time, compared with the dual combination antiviral therapy; and the earlier starting triple combination antiviral therapy will result in better antiviral efficacy.

Objective:To explore the clinical features and pathogenic mechanisms of a special syndrome with congenital sensorineural hearing loss, albinism, heterochromia iridis, nystagmus and myelin dysplasia.Methods:Detailed medical history, systematic audiology tests, ophthalmic and neurological examinations were carried out to analyze the clinical features of the child, and further molecular genetic tests including chromosome karyotype analysis, and deafness gene screening were conducted.Results:A new de novo heterozygous mutation (c.336G>T/p.Met112Ile) was detected in the child, while both his parents were demonstrated to be wild-type and symptom free. The analysis of clinical features indicated the diagnosis of PCW syndrome. Conclusion:This study identified a new mutation of SOX10 gene, which enriched the mutation spectrum of this gene. And the analysis of clinical characteristics of this patient also expanded the phenotype of this gene. This study provided a reference for clinical diagnosis and genetic diagnosis of PCW syndrome.