Familial hemophagocytic lymphohistiocytosis （FHL） with central nervous system involvement as the initial clinical manifestation is very rare and has atypical clinical manifestations， which often leads to difficulties in early diagnosis and misdiagnosis. This article reports two cases of FHL confirmed by genetic testing in Department of Neurology， Women and Children’s Hospital of Qingdao University. One patient was a boy with 1‒3 years of age， while the other patient was a girl of pre-school age； both patients had the main clinical manifestations of unstable walking and dysarthria. Brain MRI findings of the boy suggested multiple abnormal signals in the bilateral hemispheres and the cerebellum， and brain MRI findings of the girl suggested multiple symmetrical hyperintensities in the bilateral frontal lobes， the periventricular region， and the basal ganglia. Both children were found to have PRF1 gene mutations. This article reviews relevant literature to improve the understanding of this disease among clinicians， and the possibility of this disease should be considered in case of unexplained central nervous system involvement. For suspected cases， it is recommended to conduct comprehensive examinations and genetic testing， so as to achieve early diagnosis， start treatment in a timely manner， and improve prognosis.
Ataxia

Objective To identify the characteristics of nutritional problems in patients with Parkinsonism who par-ticipated in multi-disciplinary team(MDT)and to investigate the correlation between nutritional status and dyspha-gia in these patients.The predictive value of video fluoroscopic swallowing study(VFSS)and EAT-10 scale for malnutrition was compared.Methods Subjects were patients with Parkinsonism participated in joint MDT consulta-tion project in Peking Union Medical College Hospital from November 2020 to January 2023.Subjective and objec-tive dysphagia were evaluated by EAT-10 scale score and VFSS dysphagia score.Nutritional status of the patients was evaluated by geriatric nutrition risk index(GNRI),albumin,prealbumin,serum folic acid,vitamin B12 and temporal muscle thickness.Results A total of 30 participants met the criteria and were included in the study.The age was 45-82(66.1±9.0)years old.Six(20%)were at risk of malnutrition shown b a comprehensive nutri-tional status assessment using GNRI.The areas under receiver operating characteristic(ROC)curves(AUC)of VFSS dysphagia score and EAT-10 score to predict malnutrition were 0.781(0.568-0.995)and 0.927(0.827-1.000),respectively.EAT-10 score was correlated with GNRI(r=-0.524,P＜0.01),BMI(r=-0.618,P＜0.001),prealbumin(r=-0.616,P＜0.001).The VFSS dysphagia score was only correlated with BMI(r=-0.446,P＜0.05)and prealbumin(r=-0.387,P＜0.05).Conclusions Patients with Parkin-sonism requiring MDT often have multiple micronutrient imbalance.Patients' subjective perception of dysphagia has a greater impact on their nutritional status than objective assessment of dysphagia.

Objective:To analyze the effect of analysis plan, do, check, and action (PDCA) cycle in improving the completion rate of sepsis bundle treatment in sepsis patients and the knowledge-attitude-practice of sepsis bundle treatment in medical staff.Methods:Using the historical control method, sepsis patients admitted to the Emergency Trauma Intensive Care Unit of Shandong Provincial Hospital Affiliated to Shandong First Medical University were selected as the research objects by convenience sampling. The 35 patients admitted from January to December 2021 will be included in the control group; from June 2022 to June 2023, 28 patients were admitted to the observation group. The control group received routine nursing care, while the observation group received intervention based on the PDCA cycle. The completion rate of sepsis bundle treatment before and after PDCA cycle implementation was compared. The 27 nurses and 5 doctors working in trauma care unit were investigated by using a self-designed questionnaire on their knowledge and practice level of sepsis bundle treatment. The completion rate of sepsis bundle treatment before and after the implementation of PDCA cycle was compared.Results:The control group included 19 males and 16 females, aged (61.77 ± 8.64) years. The observation group included 13 males and 15 females, aged (60.61 ± 10.20) years. After the implementation of PDCA cycle, the completion rate of 3h bundle treatment for sepsis in the observation group was 89.29% (25/28), which was higher than 31.42% (10/35) in the control group, with a statistically significant difference ( χ2=23.22, P<0.05). The completion rate of sepsis bundle treatment within 6 hours in the observation group was 11/11, which was higher than 5/9 in the control group, with a statistically significant difference ( χ2=6.11, P<0.05). Moreover, after the implementation of PDCA cycle, the total score and sub-scale scores of the knowledge-attitude-practice among medical staffs increased from 86.60 ± 10.33, 21.00 ± 4.74, 18.00 ± 1.58, and 47.60 ± 4.10 to 100.00 ± 5.20, 27.60 ± 2.51, 19.60 ± 0.55, and 52.80 ± 2.28 respectively, with statistically significant differences ( t values were -5.10 - -3.14, all P<0.05). Conclusions:PDCA cycle can improve the completion rate of sepsis bundle treatment and improve the level of knowledge, attitude and practice of medical staff.

Objective:To explore the chain mediating effect of basic activity of daily living and nutritional status the effects on comorbidity and frailty, so as to provide guidance for preventing and delaying the frailty of elderly patients with cardiovascular disease and abnormal glucose metabolism.Methods:The cross-sectional study method was adopted, 300 elderly patients with cardiovascular disease and abnormal glucose metabolism who were hospitalized in the cardiovascular medicine ward of Shandong Provincial Hospital Affiliated to Shandong First Medical University, were selected as the study objects from January to August 2022, and were surveyed using General Information Questionnaire, Frailty Scale, Charlson Comorbidity Index Scale, Barthel Index Rating Scale and Mini Nutritional Assessment-Short Form were used to investigate them.Results:A total of 300 questionnaires were collected and 291 valid questionnaires were returned. Out of 291 patients, 167 were male and 124 were female, with an age of (69.55 ± 7.01) years. Comorbidities in elderly patients with cardiovascular disease and abnormal glucose metabolism were positively correlated with frailty ( r=0.414, P<0.01), and negatively correlated with basic activity of daily living and nutritional status ( r=-0.399, -0.373, both P<0.01). Basic activity of daily living was positively correlated with nutritional status ( r=0.575, P<0.01) and negatively correlated with frailty ( r=-0.825, P<0.01). Nutritional status was negatively correlated with frailty ( r=-0.695, P<0.01). The chain mediating model showed that comorbidities had a significant direct effect on frailty (effect value of 0.102), basic activity of daily living partially mediated between comorbidity and frailty (effect value of 0.125). Basic activity of daily living and nutritional status partially chained between comorbidity and frailty (effect value of 0.036). Conclusions:The chain mediating roles of basic activity of daily living and nutritional status between comorbidity and frailty was established. Healthcare professionals should pay attention to the improvement of basic activity of daily living in elderly patients with cardiovascular diseases and abnormal glucose metabolism, guide them to have a reasonable diet to achieve balanced nutrition, and delay the onset and development of frailties.

Objective:To summarize the clinical features, electroencephalogram (EEG) and magnetoencephalogram (MEG) of patients with pure paroxysmal kinesigenic dyskinesia (PKD) and PKD with epilepsy, so as to better distinguish them and guide the treatments.Methods:The clinical data of 200 patients diagnosed with PKD in the Outpatient Department of Xuanwu Hospital, Capital Medical University from 2000 to 2023 were analyzed retrospectively. The patients were divided into 2 groups: pure PKD (174 cases) and PKD with epilepsy (26 cases) according to whether accompanied by epilepsy. The differences in clinical features, drug therapy, EEG and MEG were compared between the 2 groups.Results:The clinical features of the 2 groups were essentially similar, and the proportion of PKD dyskinesia induced by emotional stress in the pure PKD group (54/174, 31.03%) was higher than that in the PKD with epilepsy group (2/26, 7.69%; χ 2=5.010, P=0.025). In terms of pharmacological response, carbamazepine was the most commonly used medication in both groups, but patients with PKD with epilepsy may need a higher therapeutic dosages (0.2-0.4 g/d, and gradually increased to 0.8 g/d) to effectively manage both dyskinesia and seizures. Regarding the EEG and MEG, the proportion of EEG abnormalities was higher in PKD patients with epilepsy, mainly manifested as focal spikes [1/70(1.43%) vs 9/21(42.86%), χ 2=24.268, P<0.001], together with aberrant MEG discharge (4/18 vs 3/5, χ 2=1.155, P=0.282). The MEG dipoles were mainly distributed in the brain regions close to the frontal lobe and central region. Conclusions:The clinical manifestations of motor symptoms of pure PKD and PKD with epilepsy are similar, and carbamazepine remains the most effective treatment. PKD patients with epilepsy have a higher proportion of abnormal EEG, mainly manifested as focal spikes, and are more likely to show abnormal discharge of MEG, which could be used to distinguish them.

Objective:To explore the path relationship among medical narrative competence and profession quality of life on professional identity of nurses, so as to provide reference for improving nurses ′professional identity. Methods:This study was across-sectional survey. From October 2022 to February 2023, totally 619 nurses in Shandong Provincial Hospital Affiliated to Shandong First Medical University were investigated using Self-designed Demographic Questionnaire, Medical Narrative Competence Scale, Professional Quality of Life Scale and Professional Identity Scale.Results:The score of nurses ′s medical narrative competence was (144.13 ± 22.09) points, compassion satisfaction was (34.82 ± 6.96) points, job burnout was (24.03 ± 5.48) points, secondary traumatic stress was (23.91 ± 5.89) points, and the scores of nurses ′ professional identity was (112.68 ± 19.05) points. Nurses ′ medical narrative competence was positively correlated with compassion satisfaction and professional identity ( r=0.585, 0.697, both P<0.01); nurses ′ medical narrative competence was negatively correlated with job burnout and secondary traumatic stress ( r=-0.516, -0.214, both P<0.01). Regression analysis showed that nurses ′ medical narrative ability, compassion satisfaction and job burnout were the influencing factors of nurses ′professional identity ( t=13.26, 10.52, -2.32, all P<0.05). Structural equation model indicated that medical narrative ability of nurses had an intermediary effect on professional identity through the dimension of compassion satisfaction, and the intermediary effect was 0.269, and the intermediary effect accounted for 36.88% of the total effect. Conclusions:Medical narrative ability of nurses can have positive emotional experience on nurses ′ psychology, and thus have an impact on professional identity. Nursing managers should pay attention to the level of nurses ′ medical satisfaction, and give full play to the intermediary effect of compassion satisfaction in nurses ′ medical narrative ability and professional identity.

Circadian rhythm is a phenomenon of diurnal changes in life activities formed by a transcription-translation feedback loop of biological clock genes affected by external environmental conditions. The circadian rhythm system controls almost all physiological processes in the organism, and these processes will change as the external environment changes. Previous studies have shown that the hypothalamic-pituitary-thyroid axis in mammals is regulated by the central diurnal pacemaker of the suprachiasmatic nucleus of the hypothalamus, so part of the thyroid function is controlled by the biological clock, and the secretion of thyroid hormones in blood can present a circadian rhythm. However, the molecular mechanism of the biological clock's regulatory effect on thyroid is still unclear. Whether circadian rhythm interference is related to the disorder of thyroid function or the occurrence of thyroid diseases is worthy of attention. This paper focused on the research progress of biological clock, circadian rhythm, and thyroid function, specifically the characteristics of circadian rhythm of thyroid physiological function and the effects of sleep deprivation, light at night, and night shift work on thyroid function, elaborated the relationships of circadian rhythm disorder with thyroid function and thyroid diseases represented by thyroid malignant tumors. The review summarized that circadian rhythm disorder may disrupt the rhythmic secretion of thyroid hormones, but no clear conclusion is reached yet on any effect on thyroid diseases, especially thyroid malignant tumors, so it is necessary to further strengthen the relevant epidemiological and molecular mechanism research.

Objective:To explore the different types and characteristics of rehabilitation exercise compliance of patients with first stroke at 2 weeks of onset, and analyze its influencing factors, so as to provide reference for the formulation of targeted health education.Methods:A cross-sectional investigation was conducted in Neurology Department of the Second Affiliated Hospital of Zhejiang University School of Medicine from January to June, 2021. 276 patients with first-episode stroke were investigated by the Questionnaire of Exercise Adherence at 2 weeks of the onset. The potential profile analysis was conducted to explore characteristics classification of the rehabilitation exercise compliance. And the chi-square test was used to compare demographic differences among different categories and ordered multi classification Logistic regression was used to explore the influencing factors of rehabilitation exercise compliance.Results:The patients were divided into 109 cases with high rehabilitation exercise compliance (39.5%), 114 cases with moderate rehabilitation exercise compliance (41.3%), and 53 cases with low rehabilitation exercise compliance (19.2%). There were statistically significant differences in education level, consciousness level at admission, complications and limb muscle strength among the three types of patients ( χ2 values were 6.17-31.50, all P<0.05). Ordered multi classification Logistic regression showed that the patient′s education level, the patient′s consciousness level at admission, whether there were complications and limb muscle strength would affect the rehabilitation exercise compliance of stroke patients ( P<0.05). Conclusions:There are three potential categories of rehabilitation exercise compliance in patients with first stroke. Patients with illiteracy, drowsiness, complications and poor limb muscle strength have poor rehabilitation exercise compliance.

Objective:To explore the effect of traditional Chinese medicine comprehensive nursing on psychological state, skin degree and quality of life in patients with rectal cancer.Methods:A total of 88 cases with rectal cancer chemotherapy in Nanyang Nanshi Hospital from August 2017 to August 2020 were as the research object, all patients were divided into two groups with 44 cases in each group by random number table method. The control group received routine nursing, the observation group was given traditional Chinese medicine comprehensive nursing based on the control group. The more satisfaction with the two groups, psychological state before and after nursing, cancer fatigue degree, the quality of life, adverse reaction after chemotherapy were compared between the two groups.Results:The satisfaction score of the observation group was (80.24±9.02) points, which was higher than that of the control group (74.64±9.02) points, there was statistically significant ( t value was 2.912, P<0.05). The probability of adverse reactions was 4.55%(2/44), which was lower than that of the control group (20.45%, 9/44), there was statistically significant ( χ2 value was 5.090, P<0.05). There were no differences in psychological state, cancer fatigue degree and quality of life between the two groups before intervention ( P>0.05). The scores of anxiety, depression, cognitive fatigue, body fatigue, emotional fatigue and behavioral fatigue after intervention were (58.24±3.56), (57.82±3.36), (5.64±1.32), (6.56±0.88), (6.04±0.64), (5.98±1.52) points in the observation group, and (60.12±3.72), (59.27±3.12), (6.28±1.48), (7.24±0.90), (6.48±0.72), (6.78±1.56) points in the control group, there were statistically significant( t values were 2.141-3.583, P<0.05).The quality of life score of the observation group after intervention was (211.68±17.12) points, which was higher than that of the control group (199.08±17.36) points, there was statistically significant( t value was 3.428, P<0.05). Conclusions:Comprehensive nursing of traditional Chinese medicine for patients with rectal cancer chemotherapy can make patients more satisfied with nursing service, relieve negative emotions and cancer fatigue, reduce the occurrence of adverse reactions to chemotherapy, and promote the improvement of patients' quality of life. It is worth popularizing.

Objective:To explore the damage of panretinal photocoagulation (PRP) to the subbasal nerve plexus (SNP) and its related mechanisms by comparing SNP changes in wide-field mosaic between before and after PRP treatment in diabetic patients.Methods:A randomized controlled study was conducted.Fifty-seven patients (114 eyes) with type 2 diabetes mellitus and binocular diabetic retinopathy (DR) stage IV to receive PRP treatment in Shanxi Eye Hospital from April to November 2019 were enrolled.The subjects were randomly divided into horizontal-vertical laser group and vertical-horizontal laser group according to a random number table.Twenty-nine eyes from 29 patients were assigned to the horizontal-vertical laser group with the photocoagulation sequence of temporal-nasal-inferior-superior.Twenty-eight eyes from 28 patients were assigned to the vertical-horizontal laser group with the photocoagulation sequence of inferior-superior-temporal-nasal.The severer eyes of each subject were chosen as the treatment eye and the contralateral eyes were chosen as the control eye.Corneal confocal laser scanning microscopy (CCM) was performed before PRP treatment, 1 week after each photocoagulation, and 1 month after the completion of PRP treatment to collect images of the SNP over an area of 2-3 mm around the whorl-like pattern.Captured images at each time were merged into one image by using the Photoshop CC 2017 image processing software, and then the nerve fiber length (NFL) of whorl-like pattern was measured by Neuron J image analysis software.McGill pain questionnaire was used to investigate the pain of patients after each photocoagulation.The NFL changes of SNP at different time points were compared between different eyes and different photocoagulation sequence groups.The study protocol adhered to the Declaration of Helsinki and was approved by an Ethics Committee of Shanxi Eye Hospital (No.201804b). Written informed consent was obtained from each patient prior to entering the study cohort.Results:After PRP treatment, there were different degrees of neural structure loss of SNP nerve fibers in 11 treatment eyes, but there was no significant change in SNP nerve fibers in the control eyes.There were significant differences in NFL between the treatment eyes and the control eyes at various time points ( Feyes=2.020, P=0.039; Ftime=4.062, P=0.001). In the horizontal-vertical laser group, different degrees of neural structure loss on the photocoagulation side were found in SNP nerve fibers after the first and second photocoagulation.In the vertical-horizontal laser group, different degrees of neural structure loss on the photocoagulation side were found in SNP nerve fibers after the third and fourth photocoagulation.There was no significant difference in NFL of treatment eyes between the two groups ( Fgroup=0.099, P=0.754), but there was a significant difference in NFL at various time points before and after treatment ( Ftime=5.231, P<0.001). There were 9 (9/57) patients who complained of pain after PRP, which occurred at the first time of photocoagulation in 7 of them. Conclusions:SNP damage may occur after PRP in patients with DR, and SNP is prone to be damaged on the photocoagulation side when performing horizontal photocoagulation.