Background Congenital fibrosis of extraocular muscles (CFEOM) affects patient' s appearance and quality of life,and no effective treatment for this disease is available.Imaging study is helpful for exploring the pathogenesis of CFEOM.Objective This study was to describe the characteristics of CFEOM associated with limb movement disorder using magnetic resonance imaging (MRI).Methods A family with CFEOM associated with limb movement disorder was investigated in Renmin Hospital of Wuhan University.Disease history was collected and the pedigree was investigated.Ophthalmologic examinations,including corrected visual acuity,refractive error,slitlamp examination,ophthalmoscopic examination,force of levator palpebrae superioris,ocular movement,eye position,forced duction test,and bell phenomenon examination,were performed.Ocular orbital and cranial MRI was performed in 4 CFEOM patients and 10 normal subjects to compare the structures of the extraocular muscles,motor nerve and cranium.Oral informed consent was obtained from each patient prior to any medical examination.Results A total of 1 1 members from 3 generations were investigated in this study,presenting with 4 cases of disease.The mode of inheritance of this family complied with the Mendelian autosomal dominant inheritance law.Clinical signs included disturbance of eye movement,deviation of eye position,ptosis,lack of Bell sign and positive reaction of passive pull test.In addition,unstable gait,improper body limb alignment,dysphasia and mental retardation were ohserved in 1 patient,which coincided with the diagnostic criteria of type 3 CFEOM.MRI results demonstrated that the levator palpebrae superioris,superior rectus and superior oblique muscle were clearly thinner,and the medial rectus,lateral rectus,inferior rectus muscle were thinning in the patients,showing significant differences in comparison with the normal controls(P＜O.05).The oculomotor and abducens nerves became thinner and even absent in the patients.Cranial MRI showed that Ⅲ-3 in the pedigree with callosum was shorter than that of the normal controls,suggesting that patient suffered from corpus callosum hypoplasia.Meanwhile,cranial MRI indicated the presence of cerebellar hypoplasia and the expansion of the fourth ventricle.Conclusions MRI demonstrates consistent abnormalities of the oculomotor nerves and abducens nerves in the affected individuals in this CFEOM 3 family,and some affected members exhibit two types of central nervous system abnormalities-corpus callosum and cerebellar hypoplasia.These findings suggest that CFEOM 3 is primarily a neuronal disease.

ObjectiveThe purpose of this study was to assess the efficacy,safety and optimal dose of tacrolimus monotherapy in patients with refractory lupus nephritis(LN) who were resistant to cyclophosphamide(CYC).MethodsA total of 14 LN patients (2 men and 12 women) with persistent proteinuria who were resistant to CYC treatment more than 8 g for half a year were enrolled.Tacrolimus was initiated at 2 mg/d (patient weight＜60 kg) or 3 mg/d(patient weight≥60 kg) which was administered in two divided doses.Prospective data on daily proteinuria,serum album level and serologic lupus activity were collected and followed for 6 months.ANOVA and Pearson correlation analysis were used for statistical analysis.Results Mean age at baseline was(30±9) years.Mean urinary protein decreased significantly from(6.2±5.1) g at baseline to (1.1±0.9) g at 6 months (F=16.21,P＜0.01).Mean serum album level increased significantly from (27.9±9.7) g/L at baseline to(37.8±2.2) g/L at 6 months(F=16.71,P＜0.01 ).Complete or partial response was observed in 86％ of patients receiving tacrolimus therapy.The effective dosage in this study was 0.03-0.06mg·kg-1·d-1 of the patients who had complete response or partial response to tacrolimus.The tacrolimus level in partially and completely responding patients was less than 3 ng/ml.There was no significant difference among blood tacrolimus levels of complete,partial,and no response patients [(1.6-±0.4),(2.0±0.6) and (22±1.1) ng/nl],respectively).No definite correlation was found between efficacy and tacrolimus level.Tacrolimus was well tolerated at current dose,besides one with new onset hypertension and one with alopecia.ConclusionOur results suggest that tacrolimus at low dosage and serum level is potentially effective and safe for the treatment　of patients with LN and persistent proteinuria resistant to CYC.The optimal dosage of tacrolimus for LN may　be 0.03-0.06 mg·kg-1·d-1.

Objective To analyze the clinical and epidemiological characteristics of human avian influenza A (H7N9 ) epidemic in Jiaxing City,and to provide scientific basis for the control and prevention of the disease.Methods The epidemiological and clinical information of 15 cases of human infection with avian influenza A (H7N9)reported from April, 2013 to May,2015 were collected.Sample of patients and external environmental samples of suspected exposure were collected and detected by real-time PCR.The epidemic distribution and clinical characteristics were analyzed.Results Fifteen cases of human infection with H7N9 were confirmed from April,2013 to May,2015,including 11 cases of death (77.33%).The confirmed cases mostly reported in winter and spring,and 9 confirmed cases were over 60 years,and male case proportion was higher than women (1.5∶1 ).Most of cases (66.67%)were sporadic and occurred in rural areas,but no epidemiological association was observed among the cases.Fever (100%),cough (66.67%),and wheezing and tightness in chest (33.33%)were common initial symptoms.The chest imaging examination of patients showed ground glass opacity and patchy consolidation,and 7 cases had simple pleural effusion.Twelve of confirmed cases had other chronic diseases.Eleven cases of death had appeared multiple medical comorbidities,such as respiratory failure (6/1 1 ),pulmonary embolism (4/1 1 ),multiple systemic organ failure (3/1 1 ),and heart cerebrovascular embolization (2/11).Twelve cases had been exposed to the live poultry markets where the positive rate of novel influenza A (H7N9) virus in environmental specimens was 7.69%(25/325).One of the 252 close contacts to confirmed cases appeared mild flu-like symptoms.No positive result related to novel influenza A (H7N9)virus nucleic acid was detected from each close contact's throat swabs.Conclusion Human infections with avian influenza A (H7N9)virus showed obvious seasonal distribution in Jiaxing.Elderly men,especially those with chronic diseases were the high risk people of human infection with H7N9 avian influenza.The infection might be associated with the exposure to live birds.Standard management of live poultry markets is the key measure in preventing and controlling H7N9 virus infection among population.

OBJECTIVETo observe early intervention effects of Modified Shuyu Pill (MSP) on vascular cognitive impairment no dementia (VCIND).

METHODSTotally 100 patients VCIND were randomly assigned to the treatment group (43 cases) and the control group (33 cases). On the basis of the treatment targeting risk factors of blood vessels, patients in the treatment group were treated by MSP, while those in the control group were treated by donepezil hydrochloride. The therapeutic course was 16 weeks. The neuropsychological scales [mini-mental state examination (MMSE) and Montreal cognitive assessment (MOCA) score] and Chinese medicine dementia syndromes scales were observed before and after treatment.

RESULTSThe MMSE and MOCA score of the two groups increased when compared with the same group before treatment (P < 0.01). But there was no statistical difference in MMSE or MOCA score after treatment between the two groups (P > 0.05). The Chinese medicine dementia syndromes scales significantly decreased in the treatment group when compared with before treatment (P < 0.01). But there was no statistical difference in Chinese medicine dementia syndromes scales in the control group between before and after treatment (P > 0.05). There was statistical difference in Chinese medicine dementia syndromes scales after treatment between the two groups (P < 0.01).

CONCLUSIONMSP could effectively intervene the progress of VCIND.

Aged ; Cognition Disorders ; prevention & control ; Drugs, Chinese Herbal ; therapeutic use ; Early Medical Intervention ; Female ; Humans ; Indans ; therapeutic use ; Male ; Middle Aged ; Piperidines ; therapeutic use

SARS-CoV-2 has infected more than 600 million people worldwide and caused more than 6 million deaths. The emerging novel variants have made the epidemic rebound in many places. Meteorological factors can affect the epidemic spread by changing virus activity, transmission dynamic parameters and host susceptibility. This paper systematically analyzed the currently available laboratory and epidemiological studies on the association between the meteorological factors and COVID-19 incidence, in order to provide scientific evidence for future epidemic control and prevention, as well as developing early warning system.
Humans ; SARS-CoV-2 ; COVID-19 ; Meteorological Concepts ; Laboratories ; Epidemiologic Studies