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Autoimmune Sclerosing Cholangitis in Children: A Prospective Case-Control Study
Kumar, Nagendra,Poddar, Ujjal,Yadav, Rajnikant,Lal, Hira,Pani, Krushna,Yachha, Surender Kumar,Srivastava, Anshu,Pandey, Rakesh The Korean Society of Pediatric Gastroenterology 2021 Pediatric gastroenterology, hepatology & nutrition Vol.24 No.2
Purpose: In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC. Methods: Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded. Results: The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups. Conclusion: The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.
Simin Liu,Weiwei Chen,Min Wang,Tong Wu,Lingli Dong,Chu Pan,Wenzhen Zhu 대한영상의학회 2019 Korean Journal of Radiology Vol.20 No.3
Objective: To evaluate the secretory function of parotid glands by dynamic magnetic resonance (MR) sialography and determine the clinical performance of this technique in diagnosing and evaluating Sjögren’s syndrome (SS) patients. Materials and Methods: This study enrolled 29 healthy volunteers (25 women and 4 men; mean age, 34.8 ± 6.3 years; age range, 26–47 years) and 25 primary SS (pSS) patients (23 women and 2 men; mean age, 37.7 ± 7.9 years; age range, 25–50 years) with decreased secretory function. The volume of the parotid gland ducts was precisely measured for both groups at single pre- and 6 post-gustatory-stimulated phases. Time-dependent volume change ratio curves were generated, four parameters were derived from the curves: the slope of the increase in the first post-stimulation phase (slope1st), the peak value, the time-to-peak, the total saliva secretion post-stimulation. All values were used to quantitatively evaluate the secretory function of the parotid gland. The repeated measurement analysis, Mann-Whitney U test and receiver operating characteristic curve were applied. Results: Time-dependent volume change ratio curves demonstrated that there is a statistically significant difference between the two groups (F = 8.750; p = 0.005). A quickly increasing curve was shown in the volunteer group, whereas a slowly increasing curve was shown in the pSS patient group. The slope1st, peak value and total saliva secretion post-stimulation of the patient group were significantly lower than those of the volunteer group (p = 0.005, p = 0.003, and p = 0.002, respectively). The timeto-peak between the two groups was not significantly different (p = 0.383). The slope1st can be used as a discriminator to diagnose SS patients (p = 0.015; odds ratio = 4.234; area under the curve = 0.726). Conclusion: Dynamic MR sialography is proven to be an effective method in evaluating salivary gland function and has a great potential in diagnosing and evaluating pSS patients.
Immunoglobulin G4-Related Disease of the Ovary Mimicking Bilateral Ovarian Malignancies
심용식,정택,정대철,김현수,오영택 대한영상의학회 2020 대한영상의학회지 Vol.81 No.4
Immunoglobulin G4-related disease (IgG4-RD) is a fibro-inflammatory condition characterized by several pathological features that can theoretically involve all organs. Ovarian involvement in IgG4-RD has been reported by two studies only. Herein, we report a pathologically confirmed case of ovarian involvement of IgG4-RD, which mimicked bilateral ovarian malignancies on computed tomography and magnetic resonance imaging.