Objective:To explore the clinical effect of "information knowledge belief practice" model, namely "IKAP theory" nursing intervention model on patients with peritoneal dialysis(PD).Methods:The clinical data of 120 patients with PD treated in The Second Hospital of Tianjin Medical University from January 2020 to December 2020 were analyzed retrospectively. The patients were randomly divided into control group and observation group, with 60 cases in each group. In the observation group, there were 32 males and 28 females with an age range of (56.16±10.25) years, including 18 cases of diabetic nephropathy, 22 cases of chronic glomerulonephritis and 20 cases of hypertensive nephropathy. In the control group, there were 34 males and 26 females, with an age range of (56.27±10.34) years, including 14 cases of diabetic nephropathy, 18 cases of chronic glomerulonephritis and 28 cases of hypertensive nephropathy. The patients in the control group were given routine nursing. In addition to the routine nursing, the patients in the observation group also adopted IKAP mode for nursing intervention. The levels of hemoglobin (HB), serum albumin (ALB), comprehensive nutrition assessment score (SGA) and nursing satisfaction of the two groups were compared before and 6 months after the intervention. The comparison of measurement data before and after intervention in the intervention group was analyzed by paired sample t-test. Frequency data were compared and analyzed by Chi-square ( χ2) test. Results:Before the intervention, there was no significant difference in ALB, Hb levels and SGA between the observation group and the control group( t=1.001~1.743, all P>0.05). After the intervention, the SGA score of the observation group was lower than that of the control group, while the levels of ALB and Hb were higher than those of the control group, and the differences between the two groups were statistically significant ( t=3.411~5.050, all P<0.05). The satisfaction of patients in the observation group was 95%, while that in the control group was 78.33%, and the difference between the two groups was statistically significant ( χ2=7.212, P<0.05). Conclusions:For peritoneal dialysis patients, IKAP nursing intervention can not only effectively improve their nutritional status, but also increase their satisfaction with nursing. IKAP nursing intervention model is worthy of clinical promotion.

Objective: To investigate the clinical application and effect evaluation of failure mode and effect analysis (FMEA) in the optimization of vascular recanalization in patients with ST-segment elevation myocardial infarction (STEMI). Methods: A total of 389 STEMI patients admitted to the emergency department of the Fifth Central Hospital in Tianjin from January 2014 to January 2015 were served as the control group, and 398 STEMI patients admitted to the chest pain center of the Fifth Central Hospital in Tianjin from January 2016 to October 2017 were served as the experimental group. In the control group, routine emergency treatment was used. At the same time, the intervention room was 24-hour prepared for emergency vascular recanalization. The experimental group used FMEA. Through the usage of FMEA, the main factors those caused the delay in revascularization treatment were determined, and the revascularization process was optimized for these influencing factors, thereby shortening the "criminal" blood vessel opening time of patients. The door-to-balloon dilatation time (D-to-B time), troponin testing time, placement time of the catheterization room, initiation of the catheterization room to balloon dilatation time, and preoperative and 1 week postoperative N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, heart function parameters [left ventricular ejection fraction (LVEF), left ventricular short axis shortening rate (FS), left ventricular end-systolic diameter (LVESD), and left ventricular end-diastolic diameter (LVEDD)] within 1 week, 3 months and 6 months after intervention, and the incidence of main cardiovascular adverse events within 1 month after intervention, hospital mortality, the length of hospital stay, and readmission within 1 year in the patients of two groups were recorded. Results: D-to-B time (minutes: 70.6±3.6 vs. 79.4±8.7), troponin testing time (minutes: 17.1±2.3 vs. 65.2±6.5), placement time of the catheterization room (minutes: 28.9±9.8 vs. 52.3±12.2) and activation of the catheterization room to balloon expansion time (minutes: 47.3±9.3 vs. 65.1±7.2) in the experimental group were significantly shorter than those in the control group (all P < 0.01). The NT-proBNP levels at 1 week after intervention in the two groups were lower than the preoperative levels, slightly lower in the experimental group, but the difference was not statistically significant. There was no significant difference in cardiac function at 1 week and 3 months after intervention between the two groups. The LVEF and FS at 6 months after intervention in the experimental group were significantly higher than those in the control group [LVEF: 0.622±0.054 vs. 0.584±0.076, FS: (38.1±4.3)% vs. (35.4±6.2)%, both P < 0.01], and LVESD and LVEDD were decreased significantly [LVESD (mm): 31.2±3.8 vs. 34.7±4.2, LVEDD (mm): 49.2±5.3 vs. 52.4±5.6, all P < 0.01]. The length of hospital stay in the experimental group was significantly shorter than that in the control group (days: 8.3±3.2 vs. 13.2±6.8, P < 0.01), the incidence of major cardiovascular adverse events within 1 month after intervention [13.6% (54/398) vs. 19.8% (77/389)], hospital mortality [1.8% (7/398) vs. 4.9% (19/389)], and readmission rate within 1 year [9.5% (38/398) vs. 14.5% (56/389)] in the experimental group were significantly lower than those in the control group (all P < 0.05). Conclusion The usage of FMEA to optimize the vascular recanalization procedure can shorten the emergency treatment time of STEMI patients, reduce the occurrence of adverse events, and improve the prognosis.

Objective: To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) . Methods: A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. The Chinese and PUBMED databases were searched with the key words "NFκB2" and "immune deficiency" and related literatures were reviewed. Results: The patient had 4 episodes of pneumonias and one otitis media since the age of 6 months. The serum immunoglobulin levels were IgG 2.73 g/L, IgA<0.07 g/L, and IgM 0.12 g/L. The percentage of peripheral lymphocyte subsets demonstrated increased CD3⁺T lymphocyte (81.8%), increased CD4⁺ naïve T cell (39.1%), normal B cell (14.1%), low switched memory B and plasmablast B (respectively 0.1% and 0), and lightly diminished natural killer(NK) cell (4.13%). Within the peripheral CD4⁺T cells, the percentage of regulatory T cells (1.49% (control 4.08%)), T follicular helper (3.66% (control 11.0%)), and T helper 17 (9.65% (control 15.7%)) were decreased, while the percentage of T helper 2 (60.9% (control 46.5%)) was elevated. T lymphocyte proliferative response and T cell receptor repertoire diversity were normal. NK-cell cytotoxic activity was impaired. The whole-exome sequencing harbored a de novo heterozygous nonsense mutation in exon 22 (c.2557C>T; p. Arg853X) in the C-terminus of NF-κB2. The western blotting confirmed the decreased expression of NF-κB2 (p52) protein. The patient received intravenous immunoglobulin infusion monthly (400-600 mg/kg), followed by improvement of pulmonary infection. After searching the databases, a total of 28 cases (1 Chinese and 27 non-Chinese) were identified. There were 12 cases of nonsense mutation (5 were gain-of-function mutation), and 8 cases of missense and frameshift mutations, respectively. The main clinical manifestation was respiratory infection, followed by autoimmune diseases such as alopecia and trachyonychia. Fifteen cases developed adrenocorticotrophic hormone (ACTH) deficiency. Conclusions NF-κB2 signaling pathway played an important role in T and B lymphocyte differentiation, and NK-cell cytotoxic activity. NFκB2 mutation should be considered in cases with recurrent infections, hypogammaglobulinemia, and decreased memory B cells and plasma cells, especially when combined with ACTH deficiency.

OBJECTIVE: To investigate the clinical application and effect evaluation of failure mode and effect analysis (FMEA) in the optimization of vascular recanalization in patients with ST-segment elevation myocardial infarction (STEMI). METHODS: A total of 389 STEMI patients admitted to the emergency department of the Fifth Central Hospital in Tianjin from January 2014 to January 2015 were served as the control group, and 398 STEMI patients admitted to the chest pain center of the Fifth Central Hospital in Tianjin from January 2016 to October 2017 were served as the experimental group. In the control group, routine emergency treatment was used. At the same time, the intervention room was 24-hour prepared for emergency vascular recanalization. The experimental group used FMEA. Through the usage of FMEA, the main factors those caused the delay in revascularization treatment were determined, and the revascularization process was optimized for these influencing factors, thereby shortening the "criminal" blood vessel opening time of patients. The door-to-balloon dilatation time (D-to-B time), troponin testing time, placement time of the catheterization room, initiation of the catheterization room to balloon dilatation time, and preoperative and 1 week postoperative N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, heart function parameters [left ventricular ejection fraction (LVEF), left ventricular short axis shortening rate (FS), left ventricular end-systolic diameter (LVESD), and left ventricular end-diastolic diameter (LVEDD)] within 1 week, 3 months and 6 months after intervention, and the incidence of main cardiovascular adverse events within 1 month after intervention, hospital mortality, the length of hospital stay, and readmission within 1 year in the patients of two groups were recorded. RESULTS: D-to-B time (minutes: 70.6±3.6 vs. 79.4±8.7), troponin testing time (minutes: 17.1±2.3 vs. 65.2±6.5), placement time of the catheterization room (minutes: 28.9±9.8 vs. 52.3±12.2) and activation of the catheterization room to balloon expansion time (minutes: 47.3±9.3 vs. 65.1±7.2) in the experimental group were significantly shorter than those in the control group (all P < 0.01). The NT-proBNP levels at 1 week after intervention in the two groups were lower than the preoperative levels, slightly lower in the experimental group, but the difference was not statistically significant. There was no significant difference in cardiac function at 1 week and 3 months after intervention between the two groups. The LVEF and FS at 6 months after intervention in the experimental group were significantly higher than those in the control group [LVEF: 0.622±0.054 vs. 0.584±0.076, FS: (38.1±4.3)% vs. (35.4±6.2)%, both P < 0.01], and LVESD and LVEDD were decreased significantly [LVESD (mm): 31.2±3.8 vs. 34.7±4.2, LVEDD (mm): 49.2±5.3 vs. 52.4±5.6, all P < 0.01]. The length of hospital stay in the experimental group was significantly shorter than that in the control group (days: 8.3±3.2 vs. 13.2±6.8, P < 0.01), the incidence of major cardiovascular adverse events within 1 month after intervention [13.6% (54/398) vs. 19.8% (77/389)], hospital mortality [1.8% (7/398) vs. 4.9% (19/389)], and readmission rate within 1 year [9.5% (38/398) vs. 14.5% (56/389)] in the experimental group were significantly lower than those in the control group (all P < 0.05). CONCLUSIONS The usage of FMEA to optimize the vascular recanalization procedure can shorten the emergency treatment time of STEMI patients, reduce the occurrence of adverse events, and improve the prognosis.
Chest Pain ; Emergency Service, Hospital ; Healthcare Failure Mode and Effect Analysis ; Humans ; Myocardial Infarction ; Prognosis

Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children′s Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed. Result: The patient was a four years old girl. The clinical manifestation consisted of autoimmune pancytopenia, lymphadenopathy and recurrent infections. Multiple exams showed that peripheral blood leukocyte count was (2.2-4.9)×10⁹/L, red blood cell count was (2.09-5.75)×10⁹/L, hemoglobin level was 64-165 g/L, platelet count was (52-138) ×10⁹/L. Percentages of lymphocyte subsets showed that CD3⁺ T lymphocyte was 0.716 0 (CD4⁺ T lymphocyte was 0.326 0, CD8⁺ T lymphocyte was 0.323 0 and CD4^- CD8^-T TCRαβ⁺ lymphocyte was 0.029 0), CD19⁺ B lymphocyte was 0.235 0 (transitional B was 0.004 3), NK was 0.032 0. Percentages of CD4⁺ T lymphocyte release IL-4, IFN-γ, IL-17 and IL-21 were 0.014 9, 0.213, 0.024 0 and 0.021 0, respectively. Lymphocyte proliferation function and TCRVβ diversity were normal. The serum immunoglobulin levels were 16.4 g/L (IgG), 1.53 g/L (IgA), 3.99 g/L (IgM) and 3.20 kU/L (IgE). The patient carried a missense variant in the 21^st exon of STAT3, c. 1974G>C, p.K658N, which was previously described as a gain-of-function mutation. The patient was treated with methylprednisolone and prednisone intermittently. There were significant improvements of hepatosplenomegaly, lymphadenopathy and pancytopenia. We searched internal database and literature for cases with gain-of-function mutations in STAT3. A total of 19 cases were identified, all were non-Chinese. Among 16 cases who had clinical data, age of onset of 11 patients was less than 5 years. 14 cases had autoimmune hemolytic anemia, autoimmune thrombocytopenia or autoimmune neutropenia. Twelve patients had lymphadenopathy while 11 had infections and 5 had endocrine abnormalities. Conclusion The patient with Primary immunodeficiency disease (PID) due to gain-of-function mutation in STAT3 gene often has early-onset autoimmune disorders, lymphadenopathy and recurrent infections. Since the routine immunological examination may be normal or slightly abnormal, comprehensive evaluation of immune function should be done. Genetic testing ultimately helps to confirm the diagnosis.

Objective To investigate the relationship between mild cognitive impairment and quality of life in hemodialysis patients with diabetic nephropathy.Methods One hundred and twenty-five hemodialysis patients with diabetic nephropathy were selected in Affiliated Hospital of North China University of Science and Technology from December 2014 to December 2016,and were divided into two groups according to mild cognitive impairment,including 73 patients without mild cognitive impairment as control group,and 52 patients with mild cognitive impairment as observation group.Patients received Montreal cognitive assessment scale and kidney disease quality of life scale.Patients received clinical index detection.Results Montreal cognitive assessment scale(visual space and execution(3.0±0.7) points,naming(2.4±0.3) points,attention(4.3±0.7) points,language(2.1±0.4) points,abstraction(1.1±0.2) points,delayed recall(1.6±0.3) points,orientation(5.4±0.5) points) score,kidney disease quality of life scale(physical(70.3±3.5) points,physical constraints due to work and life(14.6±2.8) points,pain(56.4±7.1) points,general health(36.2±3.4) points,emotional status(51.3±2.6) points,social function(52.7±3.4) points,mental status(43.2±3.6) points,emotional constraints due to work and life(41.3±2.8) points,symptoms(73.6±2.1) points,work status(20.3±1.6) points,cognitive function(72.7±1.8) points,nephropathy effect(49.1±3.2) points,sexual function(53.2±4.0) points,burden of nephropathy(21.3±2.6) points,sleep quality(70.1±1.5) points,social quality(43.3±1.9) points,social support(71.2±2.3) points) score in observation group were lower than control group((4.4±0.8) points,(3.0±0.0) points,(5.4±0.5) points,(2.7±0.3) points,(1.8±0.4) points,(3.4±0.6) points,(6.0±0.0) points),((78.6±4.1) points,(25.7±3.6) points,(69.8±4.2) points,(41.0±2.7) points,(58.5±3.0) points,(61.2±3.8) points,(53.4±2.8) points,(46.5±2.4) points,(82.9±3.2) points,(24.2±2.7) points,(81.6±2.3) points,(65.3±4.0) points,(62.8±3.9) points,(35.7±2.8) points,(79.3±2.0) points,(59.7±2.4) points,(82.4±2.6) points)(t=7.667,5.502,5.558,5.857,9.364,14.250,4.112,4.038,10.603,5.321,4.326,4.403,4.613,5.361,4.262,4.315,4.923,4.224,6.265,4.804,6.712,4.316,6.787,4.573,all P<0.05).Conclusion Hemodialysis patients with diabetic nephropathy has mild cognitive impairment,while quality of life is low.

Objective:To study the clinical effect of letrozole combined with triptorelin acetate (GnRH-a) on the serum estrogen level and ovulation quality of patients with polycystic ovary syndrome(PCOS).Methods:112 cases of PCOS patients in our hospital from August 2014 to January 2016 were selected and randomly divided into the control group (50 cases) and the observation group (62 cases) according to the wishes of patients.During the first 3～7 days of the menstrual cycle,the patients in the observation group were treated by LE,2.5 mg/d;the patients in the control group were given intramuscular injection of human menopausal gonadotropin (HMG),75 IU/d.when the diameter of the largest follicle (MFD)≥ 18 mm,the patients in the observation group were were given subcutaneous injection of 0.1 mg triptorelin acetate induced ovulation,patients in the control group were given intramuscular injection of Human chorionic gonadotropin HCG6000～10000IU induced ovulation.The ovulation induced ovulation effect and the serum hormone levels and pregnancy outcome were compared between two groups.Results:On the induced ovulation day,no significant difference was found in the endometrial thickness,the number of mature follicles,serum LH and P levels,rate of pregnancy and the luteal function between two groups of patients (P＞0.05),the number of dominant follicles,serum E2 and T levels,multiple pregnancy rate,the incidence of OHSS and ovarian cyst in observed group were significantly lower than those of the control group (P＜0.05).Conclusion:LE combined with GnRH-a could effectively improve the quality of ovulation in patients with PCOS,reduce the serum level of estrogen,and prevent the occurrence of OHSS,improve the outcome of pregnancy.

Objective: To investigate the clinical and immunological laboratory features, mutations in SH2D1A gene and SAP protein expression in four children of two families with X-linked lymphoproliferative disease type 1(XLP-1). Method: Four patients (Family A including Patient 1 and Patient 2, Family B including Patient 3 and Patient 4) and their maternal relatives were enrolled in this study. The clinical manifestation, EBV infection status and chest CT scan were analyzed. The absolute and relative numbers of lymphocyte subsets, T lymphocyte proliferative response, SAP protein expression were assessed by flow cytometry. Quantification of signal joint TCR rearrangementexcision circle (sjTRECs), CDR3 spectratyping of TCRvβ and gene mutation of SH2D1A were detected by PCR based on genomic DNA or cDNA. Result: Four male patients from two families were diagnosed with XLP-1. The ages of disease onset were more than 1 year, more than 1 year, more than 1 month and 6 months. The ages at diagnosis were nine years and ten months, sixteen years and eight months, fourteen years and ten months, four years and nine months. All patients had recurrent infections and EBV infection. Patients 1, 2, and 3 had agammaglobulinemia and Patient 4 had hypogammaglobulinemia. Chest CT scan showed all patients had atelectasis and pneumonia, and Patient 3 had bronchiectasis. Patient 3 was diagnosised as Burkitt lymphoma. For immunological function, all patients exhibited reduced CD4/CD8 ratios, increased numbers of exhausted T lymphocyte, decreased number of NK cell. The numbers of total B lymphocyte and naïve B lymphocyte were normal, but the number of memory B lymphocyte declined in all cases. Four patients′ copy numbers of sjTRECs were low and CDR3 spectratypings of TCRvβ showed mildly skewed. But their T lymphocyte proliferative response was normal. SAP protein expression in four cases were measured by flow cytometry. Two patients from Family A were absent and two patients from Family B showed decreased values. SH2D1A gene sequence analysis showed that the patients of Family A harbored a nonsense mutation (c.163 C>T; p.R55X) in exon 2. Their mother and two sisters were carriers. A missense mutation of SH2D1A gene (c.278 G>A; p.G93D) in exon 3 was found in the patients of Family B. The mother was carrier. Four patients remain survived, Patient 3 gave up treatment, other three patients received IVIG therapy. Conclusion Four patients with XLP-1 from two families characterized by agammaglobulinemia have an extreme vulnerability to Epstein-Barr virus (EBV) infection. The functions of T cell, B cell and NK cell are impaired at different stages. The detection of SAP protein and SH2D1A gene are the key methods for diagnosis of XLP-1.

Objective To know the status of mental health knowledge in Tai'an city community,and thus to provide the basis for the popularization of community about mental health knowledge.Methods A self-designed mental health knowledge questionnaire was used to survey 151 cases of community residents.Results The community mental health knowledge score was (6.73±2.72),and lower than the average.The mental health knowledge score had significant difference between male (6.26±2.50)and female (7.15±2.85) (t=-2.026,P＜0.05).The mental health knowledge score also showed significant difference in community residents with different age,culture degree and occupation (P＜ 0.05).Conclusion The community residents in Tai'an city generally lack of mental health knowledge.The relevant departments should actively take effective measures to popularize the mental health knowledge in different groups so as to enhance public awareness of mental health knowledge and thus to do better in prevention and control of mental disorders.

Objective To study the efficacy and complications of reconstruction of failed urethro-plasty for hypospadias with pedicle flap , bladder mucosa , buccal mucosa , and biological patch . Methods 23 patients were enrolled from Jul .2005 to Dec.2011.8 patients, with good local skin condition , were performed with pedicle flap urethroplasty .The other 15 patients, with bad local skin condition or with long segment urethral stricture , were performed with free grafts , including 6 bladder mucosa , 7 buccal muco-sal and 2 biological patch. Results Of the 23 cases, 7 cases were cured by one phase operation .There were 16 (16/25) cases had complications.3 (3/16) cases were failed because of serious infection (2 pedi-cle flap, 1 bladder mucosa ) .The failed cases were implemented with urethroplasty 6 months later by the buccal mucosa installments operation .4 (4/16) cases had solitary urethral fistula (1 pedicle flap, 2 bladder mucosa, and 1 buccal mucosal), who were successfully treated with simple fistula repair 3 to 6 months later. 9 ( 9/16) cases had urethral stricture ( 2 pedicle flap , 3 bladder mucosa , 3 buccal mucosal , and 1 biologi-cal patch graft ) , who were treated with urethral sound and got good result .We had reconstructed the urethra using mucosa graft onlay urethroplasty .All of the 23 patients were followed up with an average of 14.5 ( 6-24) months.23 cases were satisfied with the stretched penis , urination and no need to expand the urethra more than 6 months.3 cases were not satisfied with penile appearance .After communication, these patients did not require a further penis orthopedic surgery . Conclusions Pedicle flap, bladder mucosa , buccal mucosa and biological patch can be used in urethral repair and construction of failed urethroplasty for hypos -padias.Urethral sound dilation plays an important role in hypospadias repair .