Familial glucocorticoid deficiency type 4(FGD4) is a rare autosomal recessive disorder caused by mutations in the nicotinamide nucleotide transhydrogenase(NNT) gene. The article presented clinical data, laboratory results, and genetic mutation findings of a child with FGD4. Additionally, a retrospective analysis of the clinical data of FGD4 patients reported domestically and internationally was conducted, summarizing the types of gene mutations and clinical characteristics. This case identifies novel mutation sites in the NNT gene, providing a basis for the early diagnosis and treatment of FGD4 patients.

Objective:To explore the effect of combining hand-brain perception training with hand-brain motor training based on mirror neuron theory on the recovery of upper limb function after a stroke.Methods:A group of 105 stroke survivors with upper limb dysfunction were randomly divided into a hand-brain perception (HP) group, a hand-brain motor (HM) group, and a combination (C) group, each of 35. In addition to conventional rehabilitation treatment (including exercise therapy, occupational therapy and physical factor therapy), the HP and HM groups were given hand-brain perception training and hand-brain motor training respectively, while group C was provided with both. Before the intervention and after 4 weeks, the upper limb motor functioning of all of the participants was assessed using the simplified version of the Fugl-Meyer upper limb motor function scale (FMA-UE). Sensory functioning was quantified using the tactile Semmes Weinstein monofilament examination (SWME), and the modified Barthel index (MBI) was used to quantify the participants′ ability in the activities of daily living.Results:After the intervention the average FMA-UE, MBI and SWME scores of all three groups had improved significantly, with group C′s average FMA-UE and MBI scores significantly better than the other two groups′ averages. The average SWME score of group C was then significantly better than that of group HM.Conclusions:Hand-brain perception combined with hand-brain motor training based on mirror neuron theory can further promote the recovery of upper limb sensory and motor functioning of stroke survivors., Such therapy is worthy of clinical promotion and application.

Objective To explore the risk factors for prolonged stay of menopausal female patients in intensive care unit(ICU)and construct a predictive model.Methods A total of 260 menopausal women hospitalized in ICU of Taizhou Central Hospital from June 2019 to December 2022 were selected as study subjects.According to the length of ICU stay,the patients were divided into extended group(≥5d,156 cases)and non-extended group(<5d,104 cases).The clinical data of patients were collected,and the factors affecting the length of stay in ICU patients were analyzed by multivariate Logistic regression.A nomogram prediction model was constructed,receiver operating characteristic curve was drawn,and the area under the curve(AUC)was calculated.Results The proportion of coma,invasive operation and using of broad spectrum antibiotic in extended group were significantly higher than those in non-extended group(P<0.05).The incidence of abnormal blood pressure,muscle/bone joint pain,abnormal heart rate,abnormal respiratory function and infection in extended group were significantly higher than those in non-extended group(P<0.05).Multivariate Logistic regression analysis showed that coma,invasive operation,using of broad spectrum antibiotic,abnormal blood pressure,abnormal heart rate,abnormal respiratory function and infection were the risk factors leading to prolonged ICU stay in menopausal women(P<0.05).The constructed nomogram model had a good fit(χ2=5.072,P=0.285).The AUC of patients with prolonged ICU stay predicted by nomogram model was 0.857(95%CI:0.778-0.937),the sensitivity was 78.16%,and the specificity was 83.67%.Conclusion Unstable conscious state,invasive operation,using of broad spectrum antibiotic,abnormal blood pressure,abnormal heart rate,abnormal respiratory function,and infection are all risk factors leading to prolonged ICU stay in menopausal women,which should be paid close attention to and timely targeted treatment.

Objective:To search for and summarize the best evidence on non-pharmacological interventions for orthostatic hypotension in patients with Parkinson disease.Methods:Based on the 6S model, the relevant guidelines, clinical decisions, evidence summaries, systematic reviews and expert consensus on non-pharmacological interventions for orthostatic hypotension in Parkinson disease patients were systematically retrieved from domestic and foreign databases. The search time was from the establishment of the database to December 2022. Two researchers independently screened the literature, evaluated the quality of the included literature, and extracted and summarized evidence that met the quality standards.Results:A total of 15 articles were included, including 4 guidelines, 2 clinical decision-making articles, 1 evidence summary article, 3 systematic evaluations and 5 expert consensus articles. A total of 24 pieces of best evidence were summarized from 7 aspects, including purpose, evaluation, capacity intervention, exercise intervention, posture intervention, physical intervention, health education and support.Conclusions:The best evidence on non-pharmacological intervention for orthostatic hypotension in patients with Parkinson disease can provide a reference for the practice of clinical medical staffs. It is suggested to apply the best evidence in combination with the patient's condition, preference and clinical environment, so as to reduce the incidence of orthostatic hypotension in patients with Parkinson disease and to ensure the safety of patients.

The epidemic of COVID-19 has become the topmost public health threat worldwide. The authors suggested that in addition to strengthening the organization and leadership of the abovementioned work, greater attention be paid to establishing and improving the prevention and control mechanism.Furthermore, special efforts should be given to the safety of the medical workers, by strengthening their infection monitoring and outbreak management. Medical workers in different work areas and positions should be placed under careful protection with due cleaning and disinfection measures. The protection during specimen collection, transportation and medical waste management should also be prioritized. Such special issues in the management of pediatric patients, as allocation of nurses, the screening and management of caregivers, the problem of breastfeeding, and the disinfection of children′s toys and diapers were discussed. This paper also put forward management suggestions for the outpatient department, isolation ward and other key departments, which aim to guide the prevention and control of COVID-19 nosocomial infection in the pediatric outpatient and ward.

The pneumonia caused by the novel coronavirus (2019-nCoV), which began in December 2019, has become the most serious public health problem, threatening people's health and life. This threat is posing a severe challenge on the diagnosis and treatment of 2019-nCoV infection, the prevention and control of hospital cross infection of medical staff. It is suggested that in addition to strengthening the organization and leadership of the abovementioned work, establishing and improving the prevention and control mechanism deserve greater attention. Furthermore, special attention should be given to the safety of the medical staff, strengthening their infection monitoring and outbreak management. Medical staff in different work areas and positions should be placed under careful protection, cleaning and disinfection measures. The protection during specimen collection, transportation and medical waste management should also be prioritized. This paper also put forward management suggestions for the outpatient department, isolation ward and other key departments. These measures are proposed to provide a guidance for the prevention and control of 2019-nCoV nosocomial infection in the pediatric outpatient and ward.

Objective: To determine interleukin (IL)-23 and IL-17 level in latent autoimmune diabetes in adult (LADA) patients, and to explore the relationship of IL-23, IL-17and β-cell function in these patients. Methods: Forty LADA patients from 2011 to 2016 in our hospital were selected as LADA group, and forty participants were as normal control group. Clinical and biochemical data was collected and the level of the IL-23 and IL-17 was measured with the enzyme linked immunosorbent assay (ELISA). The differences in interleukin levels among the two groups were compared. Pearson correlation analysis was used for investigating the relationship between the dependent of statistical significant interleukins and the independent data in the LADA patients, all closely related variables then were included in a stepwise multiple linear regression analysis. Results: The levels of serum IL-23 , IL-17 and IL-23/IL-17 were significantly higher in LADA group than those in control groups [3.54(2.88~5.24)μg/L vs 1.98(1.62~2.18)μg/L, P<0.05], [22.42(17.71~26.07)ng/L vs 17.97(17.15~20.70)ng/L, P<0.05], (175.79±38.67 vs 105.22±19.08, P<0.01). IL-23 and IL-17 in the LADA group were negatively correlated with fasting C peptide (FCP) (r=-0.42, r=-0.48, P<0.05), and the ratio of IL-23/IL-17 was positively correlated with fasting plasma glucose (FPG) (r=0.44, P=0.00). Stepwise multiple liner regression analysis showed that serum IL-23 and IL-17 level were independently associated with the FCP in LADA group. Conclusions IL-23 and IL-17 were possibly important proinflammatory factor in LADA patients, and can provide the new immunodiagnosis markers for LADA.

Objective:To explore the effects of 5E rehabilitation nursing model on self-management behavior and fatigue symptoms of patients with chronic obstructive pulmonary disease (COPD) .Methods:Totally 110 COPD patients admitted in Yantai Yuhuangding Hospital from October 2018 to September 2019 were selected by convenient sampling, and divided into the control group ( n=53) and the experimental group ( n=57) according to the random number table. Excluding missing patients and patients with incomplete data, finally 48 patients were included in the control group and 51 patients were included in the experimental group. Patients in the control group received routine care and health guidance, while patients in the experimental group received care based on the 5E rehabilitation nursing model. The Self-Management Scale for COPD Patients and the Functional Assessment of Chronic Illness Therapy-Fatigue Scale (FACIT-F) were used to evaluate the effects of interventions in the two groups. Results:There was no statistically significant difference in the scores of the Self-Management Scale between the two groups before intervention ( P>0.05) ; the scores of all dimensions the Self-Management Scale of the experimental group were higher than those of the control group 12 weeks after discharge, and the differences were statistically significant ( P<0.05) . There was no statistically significant difference in fatigue scores between the two groups before intervention and 12 weeks after discharge ( P>0.05) . Conclusions:The 5E rehabilitation nursing model can improve the self-management behavior of COPD patients, but it cannot ameliorate the fatigue symptoms of patients. Large-sample, multi-center, and long-term research will be further needed in the future to explore the effects of the 5E rehabilitation nursing model on patients' fatigue symptoms.

Objective: To explore the application effect of standardized video combined with feedback method in health education for patients with urinary calculi after double J tube indwelling operation, and to provide basis for improving the effect of clinical health education. Methods: One hundred and fourteen patients were selected from October 2018 to March 2019 for the first time after the operation of urinary calculi with double J-tube indwelling after holmium laser lithotripsy. Fifty-six patients were selected as the control group and 58 cases as the experimental group by random number table method. The control group was given routine nursing and oral health education, while the experimental group was given standardized video combined with feedback education on the basis of routine nursing. The level of knowledge, belief and behavior, the incidence of complications and satisfaction with nursing health guidance were compared between the two groups after intervention. Results: The scores of knowledge, belief and action in the experimental group were (7.14 ± 1.39), (30.17 ± 2.62) and (67.50 ± 3.40) respectively, which were higher than those in the control group (5.39 ± 1.29), (28.54 ± 2.29) and (62.23 ± 4.39). The difference was statistically significant (t=5.83, 3.55, 7.18, P<0.01). The incidence of complications was 34.48% (20/58) in the experimental group, which was significantly lower than 62.50% (35/56) in the control group (χ²=8.957, P<0.01). The average rank of satisfaction with nursing health guidance in the experimental group was 66.03, which was significantly higher than that in the control group. The difference was statistically significant (Z=-3.157, P=0.02). Conclusions Standardized video education combined with feedback method can improve patients′ awareness of vascular behavior after double J tube indwelling for urinary calculi, reduce complications related to double J tube indwelling, and improve patients′ satisfaction with nursing education.

Objective? To investigate the hope level of elderly patients undergoing total knee arthroplasty (THA) and analyze its influencing factors, so as to provide evidence for improving the hope level and quality of life of patients after THA. Methods? A cluster random sampling method was used to select 308 patients with bilateral THA for the first time who were admitted to two ClassⅢ Grade A general hospitals in Harbin from January 2017 to July 2018. Questionnaire surveys were conducted using Herth Hope Scale, the Second Edition of Beck Depression Scale and Social Support Scale. Results? The total score of hope level in elderly THA patients was (29.96±8.72). Among them, 76.62% (236/308) had moderate to high level of hope and the total score of depression was (19.20±11.75) which was at a moderate level. There were significant differences in the hope level of patients with different gender, place of residence, educational level, marital status and family per capita monthly income (P＜ 0.05). The results of multiple stepwise regression analysis showed that age, sex, family income per capita, depression, negative coping style, subjective support and objective support were the influencing factors of elderly THA patients' hope level (P ＜ 0.05). Conclusions? The THA patients' hope are at a medium level. Medical staff should pay attention to the relevant factors affecting the level of hope, adopt targeted nursing intervention measures, give psychological guidance to patients, provide social support, and further improve the level of hope of the THA patients.