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Leukoencephalopathy with vanishing white matter (VWM) is one of the most prevalent inherited childhood white-matter disorders, and the pathogenic gene has been confirmed as EIF2B gene. VWM is characterized by chronic progressive neurological deterioration with cerebellar ataxia, usually less prominent spasticity and relatively mild mental decline. There are episodes of rapid and major neurological deterioration provoked by stresses, such as fever, minor physical trauma and acute fright, which is a characteristic clinical feature of VWM. Brain magnetic resonance imaging findings are diagnostic in almost all patients,and the disappearance of the cerebral white matter occurs in a diffuse "melting away" pattern. The onset of VWM can be at any age from fetal stage to adult stage, and the clinical phenotypes vary immensely. Gene diagnosis is the golden standard for VWM. This article reported a patient with a course of 17 years, who was misdiagnosed as Wilson′s disease because of low serum ceruloplasmin, and was finally diagnosed as VWM by reinterpretation of whole exome sequencing, which is worthy of clinicians′ vigilance and consideration.
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Objective:To investigate the curative efficacy and application value of convalescent plasma (CP)in severe and critical coronavirus disease 2019 (COVID-19) caused by Delta variant.Methods:The treatment process and results of CP therapy for a patient with critical COVID-19 caused by Delta variant were reported. The clinical application value of CP for COVID-19 caused by Delta variant was analyzed along with the literature review.Results:Our case was a 50-year-old male, who was imported from abroad and had not been vaccinated against COVID-19. The novel coronavirus nucleic acid test was negative before entry. On the second day after entry, fever occurred, novel coronavirus nucleic acid test was positive. Chest CT images showed bilateral multiple mottling and ground-glass opacity with symptoms of nausea, headache, loss of appetite, diarrhea, but no running nose, nasal obstruction, dyspnea, abnormal smell and taste. The infection rapidly developed from medium to critical. On the basis of standard treatment, Delta variant CP was intravenous dripped on the 10th day of hospital admission (the 6th day after becoming severe). The patient's condition improved rapidly.Conclusion:The curative efficacy evaluation of this patient proved that CP therapy is of great value in the treatment of severe and critical COVID-19.
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Objective:To analyze evaluation outcomes and influential indexes of 116 continuing medical education (CME) programs, and to offer references for ascertaining the quality evaluation focuses of CME programs.Methods:Chi-Square test and Fisher exact test were adopted to perform univariate analysis on evaluation outcomes of 28 indexes, and logistic regression was used for multivariate analysis.Results:Logistic regression showed that the indexes influencing the evaluation outcomes of programs qualified or not included consistency of basic information [ OR=6.712, 95%CI (1.314, 34.275)], whether textbooks provided by host institutions [ OR=109.386, 95%CI (2.577, 4 642.659)], whether the number of people in the meeting room equaled with the signature list [ OR=13.296, 95%CI (2.440, 72.456)], feedback of learners examination materials [ OR=19.069, 95%CI (3.713, 97.929)], feedback of teachers evaluation materials [ OR=13.497, 95%CI (2.672, 68.181)] and whether submit learners' credits according to regulations or not [ OR=51.326, 95%CI (4.999, 526.996)]. Conclusion:The administrative departments of CME should complete program evaluation regulations, adjust and perfect evaluation indexes based on research outcomes, emphasize on influential indexes of evaluation outcomes, and give full play of the guiding role of evaluation indexes.
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Objective To investigate the clinical features of recurrent Guillain-barré syndrome. Methods The data on demographic information and clinical features of 286 inpatients with Guillain-barré syndrome were collected. Patients were divided into recurrent group and non-recurrent group based on the number of episodes. The data from those patients with recurrent Guillain-barré syndrome whose information was missing was collected by telephone. Results we identified 22 (7.7%) cases with recurrent Guillain-barré syndrome including one case of Miller fisher syndrome. The total episodes were 55. Sixteen patients had 2 episodes, 3 patients had 3 episodes, 1 patients had 4 episodes and 2 patients had 5 episodes. Compared with non-recurrent group, patients with recurrent Guillain-barré syndrome had younger onset [(36.36 ±14.51)y vs. (45.72 ±16.13)y, t=-2.633, P=0.009], the shorter interval between the initial manifestations to peak [(2.18±0.73)d vs.(4.24±2.98)d, t=-8.537, P=0.000], the lower GBS disability score (68.2% vs. 31.8% , χ2=4.209, P=0.040) and less involvement in the cranial nerve (13.6% vs. 37.5% , χ2=5.040, P=0.025). Conclusion The recurrent risk may be considered in patients with Guillain-barré syndrome when younger patients, have lower GBS disability score and the shorter interval between the initial manifestations to peak.
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Objective To design a scientific, standardized, fully functional, easy-to-use Guillain-Barré syndrome (GBS) clinical informatization management system, with the purpose to meet the needs of diagnosis and clinical research in patients with GBS. This system can manage and excavate clinical data and provide a platform for clinical research of GBS. Methods The clinical data items of GBS were identified. The Visual Basic 6.0 and Vista DB software development environment was used to develop the system and the related database based on embedded database. Results A GBS database including 560 clinical indicators were set up. Through the built-in data mining and analysis functions, the system can realize the functions of visualized data input, combination search, statistical analysis, data exchange and literature update. This system has been used in Xijing Hospital, and a total 274 clinical and prognostic records were recorded from the patients with GBS. The practical results proved that the system can achieve the design goals. Conclusions Based on the information technology and GBS-related clinical epidemiology and neurology expertise, a standard GBS clinical data management and analysis system and related database were established, which could provide a basis for GBS information storage, update, epidemiological investigation and prognosis.
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Objective To investigate the clinical manifestations, auxiliary examination, diagnosis and therapy of Aspergillus tracheobronchitis (ATB).Methods Clinical data of 4 ATB patients treated in Qingdao Municipal Hospital from June 2012 to September 2015 were analyzed retrospectively. The related literature was also reviewed.Results The main clinical manifestations were cough, sputum production, short of breath, fever and hemoptysis. All the four patients had predisposing factors. The ifndings of CT scan mainly included bronchial wall thickening, obstruction of bronchial lumen or no significant abnormality. Bronchoscopy showed mucosal erosion, necrosis, tracheal stenosis and obstruction of airway by pseudo-membrane. The diagnosis was confirmed by histopathology in all the four cases. They were treated with anti-Aspergillus therapy. The two patients with airway stenosis were treated with interventional therapy via bronchoscope. As for the outcome, one patient was improved after therapy. Another was cured. The remaining two patients died.Conclusions The clinical manifestations and chest imaging of ATB are nonspecific. Bronchoscopy is an important tool for diagnosis of ATB. Early diagnosis and proper anti-Aspergillus treatment are critical for improving prognosis.
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Objective To investigate the clinical characteristics and prognosis in the patients with type 2 diabetes mellitus and invasive pulmonary aspergillosis(IPA) for better management of the disease .Methods Clinical data of 9 cases of type 2 diabetes associated with IPA treated in Qingdao Municipal Hospital from January 2008 to December 2013 were analyzed retrospectively . Results The diagnosis of IPA was proven in 5 and probable in 4 of the 9 patients .The main clinical manifestations were fever , cough and expectoration .The findings of CT scan mainly showed pulmonary nodules along the bronchovascular bundle and cavity signs .Bronchoscopy showed congestion ,edema ,and erosion of bronchial mucosa covered with yellow‐white or brown pus ,partially or completely blocking the lumen .Antifungal treatment was effective for 4 patients .The other five patients died . Conclusions Type 2 diabetes mellitus is a risk factor for developing invasive pulmonary aspergillosis .Early diagnosis and proper treatment are critical for improved prognosis .
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BACKGROUND: Amfetamine, one of the levarterenols, can improve the recovery of motor function of animals after cerebral ischemia. Chlorimipramine can inhibit the re-intake of 5-serotonin and levarterenol and improve the level of them in brain. OBJECTIVE: To observe the effect of chlorimipramine on motor function of rats with focal cerebral ischemic injury.DESIGN: Randomized grouping and controlled animal study. SETTING: Neuropsychiatry Team of Aerial Clinical Medical Department of the Fourth Military Medical University of Chinese PLA. MATERIALS: The experiment was completed in the Morphological Laboratory of Aerial Aerospace Medical Department of the Fourth Military Medical University of Chinese PLA. All the 24 SD rats were divided into sham operation group, ischemia group and ischemic medication group with 8 in each group. Rats in ischemic medication group were perfused with 10 mg/kg chlorimipramine solution (2.5 g/L) once a day through mouth 24 hours after ischemia, and rats in sham operation group and ischemia group were perfused with the same volume of distilled water through mouth. METHODS: Middle cerebral artery (MCA) models of ischemia/reperfusion rats were established with inserted lining method. After modeling, the operations were performed as followed: ① holding test of net screen: Net screen was put horizontally, and then rats were put on it. One side of the screen was raised gradually; then it was turned over 125° within 2 s, and maintained at this placement. Time of holding rats on net screen was recorded. ② Test of struggling to tear off rubberized cloth: 0.5 cm2 medical rubberized cloth was adhered to the ventral of anterior claws of rats, and then rats were sent to the observing box to record the time of tearing off rubberized cloth. 1, 3, 7, 14 and 28 days after operation were the observing time points. MAIN OUTCOME MEASURES: Times of holding rats on the net screen and tearing off the rubberized cloth of rats with focal cerebral is chemia.RESULTS: ① Time of muscular strength test of rats in ischemia group and ischemic medication group was shorter than that of rats in sham operation group, and there was significant difference [3 days after operation:(54±4), (20±5) and (21±4) s in sham operation group, ischemia group and ischemic medication group respectively, P ＜ 0.01]. Holding time was longer in ischemic medication group than that in ischemia group, and there was significant difference [28 days after operation: (51±5) and (54±5) s in ischemia group and ischemic medication group respectively, P ＜ 0.05]. ②Time of tearing off the rubberized cloth was longer in ischemia group and ischemic medication group than that in sham operation group, and there was significant difference [3 days after operation: (47±9), (188±20) and (172±22) s in sham operation group, ischemia group and ischemic medication group respectively, P ＜ 0.01]. The time in ischemic medication group was shorter than that in ischemia group, and there was no significant difference (P ＞ 0.05).CONCLUSION: Chlorimipramine has great effect on the recovery of muscular strength after focal cerebral ischemia, but has poor effect on sense and refined motor function, which is in coincidence with the poor recovery of refined motor function of limbs after hemiplegia.
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Objective To compare the curative effects and safety of immunoabsorption(IA) and double filtration plasmapheresis(DFPP) in treatment of Guillain-Barre syndrome(GBS).Methods 60 patients of GBS were randomly divided into two groups and treated with IA and DFPP,respectively.The neurological functions were assessed and the immunoglobulin,complement and total protein in the blood were also measured before and after treatments.Results The rating of the neurological function deficiency in both groups improved with time,but there was no significant difference in Hughes rating between two groups.After half year,the MRC rating in IA group was better than that in DFPP group((P
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Objective To investigate the effect of electroacupuncture applied at Baihui acupoint on cognitive function in lithium-pilocarpine induced spontaneous recurrent epileptic rat and provide some theoretical basis for acupoint stimulation treatment seizure.Methods Male mature Sprague Dawley rats were subjected to spontaneous recurrent epileptic model induced by lithium-pilocarpine,and divided into different groups randomly: acupoint group(electroacupuncture applied at Baihui acupoint),non-acupoint group(electroacupuncture located in close vicinity to the Baihui acupoint),and epileptic control group.Normal rats were subjected into normal control group.The Morris water maze test and step though test were carried out respectively to explore the effect of electroacupuncture applied at Baihui acupoint on the rats' learning and memory capacities.Results Compared with control group,acupoint rats in Morris water maze test time of finding the platform under the water surface decreased(P