Objective: To analyze the electrocardiographic (ECG) characteristics in patients with primary hyperparathyroidism (PHPT) and meanwhile, to explore the relationship between ECG abnormality and serum calcium level in relevant patients. Methods: Our study included 2 groups: Observation group,n=84 PHPT patients treated in our hospital from 2010-01 to 2014-04 and Control group,n=80 normal subjects from regular physical examination at the same period. The ECG manifestation was compared between 2 groups. Meanwhile, according to tertile levels of serum calcium, PHPT patients were further divided into 3 subgroups:①Ca2+ level (2.0-2.8) mmol/L,②Ca2+ level (2.8-3.1) mmol/L,③Ca2+ level >3.1 mmol/L andn=28 in each subgroup. The ECG abnormalities were compared among 3 subgroups and the relationship between ECG changes and serum calcium levels were studied. Results: The ECG abnormality rates in Observation group and Control group were as 83.3% vs 18.8%, P<0.001. Compared with Control group, Observation group presented more frequent ST-T changes, QRS duration widening, high left ventricular pressure and QT interval shortening,P<0.001 orP<0.05. In observation group, with the rising of serum calcium, the incidences of ST-T changes and high left ventricular pressure were increased accordingly, the ST-T changes among 3 subgroups were significant,P<0.05; while with the rising of serum calcium, the QT interval was shortened and QRS duration was widened accordingly, the differences were obvious among 3 subgroups, allP<0.05.Conclusion: PHPT patients have the higher incidence with abnormal ECG, while ECG abnormality has beenrelated to serum calcium level in relevant patients.

Objective To predict the length of peripherally inserted central venous catheters(PICC) with electronic chest radiograph scale measurement techniques, and observe its clinical effect. Methods A total of 185 breast cancer patients from October 2012 to December 2013 who were treated by PICC combined with MST guided by ultrasonic technology were as control group. A total of 192 breast cancer patients from January to November 2014 were as observation group. Control group adopted from the puncture point to right sternoclavicular joints impreaaion nip down again to the third floor of PICC in vitro measurement method. Observation group used to right sternoclavicular joints from the puncture point plus right sternoclavicular joints to subcarinal 1 vertebral body length, right sternoclavicular joints to subcarinal 1 vertebral body length measurement by electronic chest radiograph scale technology directly measured from the electronic chest radiographs. The accuracy rates and complications between the two measurement methods were compared. Results The accuracy rate in observation group was 97.92%(188/192), which was higher than that in control group (68.11%,126/185 ),and there was significant differencek,χ2=60.15, P<0.01. The complication rate in observation group was 6.77%(13/192), which was lower than that in control group (20.54%, 38/185) ,and there was significant difference,χ2=9.58, P<0.01. Conclusions The electronic chest radiograph scale measurement techniques could effectively improve the accuracy of PICC catheter placed, improve the quality of venipuncture, decrease the complications .

Objective:To construct a self-management intervention program for patients with adenocarcinoma chemotherapy based on chronic disease trajectory model, and to provide reference for clinical nursing intervention.Methods:From October 2021 to June 2022, a research team was established. Based on the chronic disease trajectory model and self-management theory, through preliminary quantitative research, literature review and semi-structured interview, the first draft of the intervention plan was prepared. 24 experts were selected for two rounds of Delphi expert correspondence consultation to determine the self-management intervention plan for breast cancer patients undergoing chemotherapy based on the trajectory model of chronic disease.Results:The effective questionnaire recovery rates of the two rounds of expert letter consultation were 100%, and the coefficient of expert authority degree was 0.805 and 0.863, respectively. The mean values of importance were 4.21- 4.96 and 4.46 - 5.00, the coefficients of variation were 0.00 - 0.19 and 0.00-0.17, and the Kendall concordant coefficients were 0.123 and 0.149 ( both P<0.01). Finally, the self-management intervention scheme for breast cancer chemotherapy patients based on the trajectory model of chronic disease was constructed, including 3 first-level indicators, 6 second-level indicators and 27 third-level indicators. Conclusions:The self-management intervention program for patients with adenocarcinoma chemotherapy based on chronic disease trajectory model constructed in this study can provide a certain reference for clinical nurses to carry out targeted self-management interventions.

OBJECTIVE: To explore the clinical features, lysosomal enzymatic [acid α-glucosidase (GAA)] activities and genetic variants in a child with late-onset Pompe disease (LOPD). METHODS: Clinical data of a child who had presented at the Genetic Counseling Clinic of West China Second University Hospital in August 2020 was retrospectively analyzed. Blood samples were collected from the patient and her parents for the isolation of leukocytes and lymphocytes as well as DNA extraction. The activity of lysosomal enzyme GAA in leukocytes and lymphocytes was analyzed with or without addition of inhibitor of GAA isozyme. Potential variants in genes associated with neuromuscular disorders were analyzed, in addition with conservation of the variant sites and protein structure. The remaining samples from 20 individuals undergoing peripheral blood lymphocyte chromosomal karyotyping were mixed and used as the normal reference for the enzymatic activities. RESULTS: The child, a 9-year-old female, had featured delayed language and motor development from 2 years and 11 months. Physical examination revealed unstable walking, difficulty in going upstairs and obvious scoliosis. Her serum creatine kinase was significantly increased, along with abnormal electromyography, whilst no abnormality was found by cardiac ultrasound. Genetic testing revealed that she has harbored compound heterozygous variants of the GAA gene, namely c.1996dupG (p.A666Gfs*71) (maternal) and c.701C>T (p.T234M) (paternal). Based on the guidelines from the American College of Medical Genetics and Genomics, the c.1996dupG (p.A666Gfs*71) was rated as pathogenic (PVS1+PM2_Supporting+PM3), whilst the c.701C>T (p.T234M) was rated as likely pathogenic (PM1+PM2_Supporting+PM3+PM5+PP3). The GAA in the leukocytes from the patient, her father and mother were respectively 76.1%, 91.3% and 95.6% of the normal value without the inhibitor, and 70.8%, 112.9% and 128.2% of the normal value with the inhibitor, whilst the activity of GAA in their leukocytes had decreased by 6 ~ 9 times after adding the inhibitor. GAA in lymphocytes of the patient, her father and mother were 68.3%, 59.0% and 59.5% of the normal value without the inhibitor, and 41.0%, 89.5% and 57.7% of the normal value with the inhibitor, the activity of GAA in lymphocytes has decreased by 2 ~ 5 times after adding the inhibitor. CONCLUSION The child was diagnosed with LOPD due to the c.1996dupG and c.701C>T compound heterozygous variants of the GAA gene. The residual activity of GAA among LOPD patients can range widely and the changes may be atypical. The diagnosis of LOPD should not be based solely on the results of enzymatic activity but combined clinical manifestation, genetic testing and measurement of enzymatic activity.
Humans ; Child ; Male ; Female ; Glycogen Storage Disease Type II/pathology* ; Retrospective Studies ; alpha-Glucosidases/genetics* ; Mothers ; Lysosomes/pathology* ; Mutation