ABSTRACT
Objective:To investigate the clinical manifestations, laboratory and imaging features, treatment and prognosis of systemic lupus erythematosus (SLE) with primary sclerosing cholangitis (PSC).Methods:This is a retrospective case series study describing the clinical, laboratory and imaging manife-stations, treatment and prognosis of 3 SLE patients with PSC. The related literatures were reviewed.Results:In total, 10 patients were included. SLE with PSC, with an average age of (43±17) years old, was more common with hematological and renal involvement, jaundice and arthralgia, positive anti-double-stranded DNA (anti-dsDNA) antibody, hypocomplementemia, elevated erythrocyte sedimentation rate (ESR) and abnormal liver function with predominately elevated alkaline phosphatase (ALP). The classic magnetic resonance cholangio-pancreatography (MRCP) features of PSC include multifocal strictures, beading and areas of dilatation involving the intra- and/or extrahepatic bile ducts. The treatment included glucocorticosteroids, immunosup-pressants and ursodeoxycholic acid (UDCA), and some patients required biliary drainage. Most patients had a good prognosis.Conclusion:Although PSC is rare, attention should be paid to SLE patients with abnormal liver function, especially with elevated ALP, in order to differentiate from PSC.
ABSTRACT
Objective To investigate changes of the interhemispheric coordination integrity in patients with hemi-parkinsonism using resting-state functional magnetic resonance imaging(rs-fMRI) homotopy technique called voxel-mirrored homotopic connectivity(VMHC).Methods Sixteen Parkinson disease(PD) patients with right body side motor symptom onset(RPD),15 patients with left side onset(LPD),and 19 age-,gender-,and education-matched healthy controls(HC) were included in this study.rs-fMRI scanning and pre-processed the raw data were performed.Then by using analysis of variance(ANOVA) and two sample t tset,we performed VMHC analyses on rs-fMRI data of these participants(P＜0.05,corrected with AlphaSim,clusters≥16 voxels).Exploratory linear correlations analyses were performed between the VMHC of regions showing significant group differences and the clinical features of LPD or RPD patients.Results Compared with HCs,patients with LPD had significantly reduced VMHC in visual regions,sensorimotor regions,and the cerebellar cortex(voxels size of 17-77,t=-5.06--3.42,P＜0.05).Patients with RPD exhibited decreased VMHC in the prefrontal cortex and sensorimotor regions.Both LPD and RPD groups had increased VMHC in subcortical regions.When compared with the RPD group,the LPD group displayed decreased VMHC in the visual regions,sensorimotor regions(voxels=16 and 18; t=-3.68and-3.87,respectively,both P＜0.05) and increased VMHC in the supramarginal gyrus(voxels=4,t=4.72,P＜0.05);ROI-based correlation analyses indicated that the VMHC in the inferior occipital gyrus and the postcentral gyrus was negatively correlated with the BDI-Ⅱ scores in the LPD group(r=-0.58 and-0.59,respectively; both P＜0.05),and positively correlated with the MMSE scores(r=0.56 and 0.52,respectively;both P＜0.05).In the RPD group,a positive correlation was found for the VMHC in the supramarginal gyrus and the illness duration(r=0.56,P＜0.05) and for the VMHC in the postcentral gyrus and the(mini-mental state exam) MMSE(r=0.53,P＜0.05).Conclusions The differential pattern of deficits in the interhemispheric coordination integrity in hemi-parkinsonism reflected by VMHC may provide insights into the neurological pathophysiology underlying the asymmetry of symptom appearance in PD.