Sclerosing

BACKGROUND: Idiopathic orbital inflammatory syndrome (IOIS) is an inflammatory condition of unknown etiology. The inflammation may affect all the structures within the orbit (anterior, diffuse, apical, myositic, dacryoadenitis) and corresponds to uniquely orbital inflammation without an identifiable local cause or systemic disease. The goal of this study is to describe the clinical and radiographic characteristics of IOIS and discuss the role of orbital biopsy in this condition. PATIENTS AND METHODS: This is a retrospective review of the charts of 24 patients diagnosed with IOIS at Fattouma Bourguiba hospital, Monastir, Tunisia, from January 2007 to December 2015...

OBJECTIVE: To derive an optimal liver stiffness measurement cut point to discriminate METAVIR fibrosis stage F4 and to validate both METAVIR fibrosis stage F3-F4 and F4 cut points in a separate cohort. STUDY DESIGN: Patients at Boston Children's Hospital with liver stiffness measurement from 2006 to 2016 and liver biopsy ≤12 months before screening were eligible. Patients enrolled 2006-2011 were used to calibrate liver stiffness measurement cut points and those enrolled 2011-2016 for validation...

BACKGROUND AND PURPOSE: The aim of this prospective study is to investigate and evaluate in clinical practice the diagnostic impact of 3DFLAIR in regards to 2DT2/PD in terms of infratentorial lesions detection in multiple sclerosis (MS). MATERIAL AND METHODS: 164 MS patients from the OFSEP database were reviewed retrospectively. MR examinations were performed on 1.5T or 3T systems from four different centers. Infratentorial lesions were counted and allocated to different regions of the posterior fossa by three raters independently (junior resident, resident with an expertise in neuroradiology, and senior neuro-radiologist) on the 3DFLAIR and 2DT2/PD...

Autoimmune pancreatitis (AIP) is an increasingly recognized form of acute pancreatitis characterized by obstructive jaundice with a rapid and dramatic treatment response to steroid therapy. Recently, AIP has been divided into two distinct phenotypes: lymphoplasmocytic sclerosing pancreatitis AIP (type 1) and idiopathic duct-centric pancreatitis AIP (type 2); each of which have their own distinct demographics, diagnostic criteria, and histopathological features. We report, to the best of our knowledge, the first case of a multifocal pattern of type 2 AIP characterized with both CT and MR imaging...

Primary sclerosing cholangitis (PSC) is a rare idiopathic condition with immunopathogenic mechanisms where there is chronic progressive destruction of the biliary tree. Secondary sclerosing cholangitis (SSC) is clinically comparable to PSC, but is caused by specific processes which directly damage the biliary tree; examples include recurrent pancreatitis, bile duct malignancy, congenital bile duct abnormalities. A new cause of SSC has been described during or following significant critical illness associated with severe respiratory insufficiency, vasopressor requirement, shock and sepsis...

BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6)...

Biliary atresia (BA) is a neonatal T cell-mediated inflammatory, sclerosing cholangiopathy. In the Rhesus rotavirus (RRV)-induced neonatal mouse model of BA (murine BA), mice lacking B cells do not develop BA, and the lack of B cells is associated with loss of T cell and macrophage activation. The aim of this study was to determine the mechanism of B cell-mediated immune activation (antigen presentation versus cytokine production) in murine BA. RESULTS: Normal neonatal B cells in the liver are predominantly at pro-B and pre-B cellular development...

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Hepatic fibrosis is characterized by abnormal accumulation of extracellular matrix (ECM) that can lead to ductopenia, cirrhosis, and even malignant transformation. In this review, we examine cholestatic liver diseases characterized by extensive biliary fibrosis such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), polycystic liver disease (PLD), and MDR2-/- and BDL mouse models. Following biliary injury, cholangiocytes, the epithelial cells that line the bile ducts, become reactive and adopt a neuroendocrine phenotype in which they secrete and respond to neurohormones and neuropeptides in an autocrine and paracrine fashion...

A 58-year-old female, a known diabetic and hypertensive, presented with left-sided swelling on the anterior aspect of the neck of 1-year duration, which was rapidly increasing in size for the past 6 months. She was on Eltroxin for hypothyroidism for the past 1 year. Computed tomography study of the neck showed a nodule in the left lobe of thyroid which on fine-needle aspiration was suspicious for malignancy. Total thyroidectomy with left posterolateral lymph node dissection was done. Histopathological examination showed sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid gland with lymph node metastasis...

Background/Aim: Inflammatory bowel disease (IBD) is a chronic gastrointestinal disorder which includes ulcerative colitis (UC), Crohn's disease (CD), and indeterminate colitis (IC). The natural history of pediatric IBDs is poorly understood and generally unpredictable. We aim to study the natural history of IBD in Saudi children including the extraintestinal manifestations, changes in diagnosis, disease behavior, medical management, and surgical outcome. Patients and Methods: A retrospective review of all the charts of children less than 14 years of age who were diagnosed as IBD and followed up in King Faisal Specialist Hospital and Research Center (KFSH and RC) from January 2001 to December 2011 was performed...

AIM: To investigate the prevalence and causes of cholestasis in patients with inflammatory bowel diseases in the Swiss Inflammatory Bowel Diseases Cohort. METHODS: A retrospective cohort study was performed of all the patients in the Swiss Inflammatory bowel disease Cohort. Total bile acid was measured for all patients and cholestasis was defined as a concentration > 8 μmol/L. The characteristics of patients with or without cholestasis were compared. Bile acid profiles were then determined for 80 patients with high total bile acid and 80 matched patients with low total bile acid...

Background: Extra-intestinal manifestations (EIMs) can impact morbidity in patients with inflammatory bowel diseases (IBD; Crohn's disease [CD] and ulcerative colitis [UC]). This study compared incidence rates of EIMs in patients with moderate to severe IBD receiving gut-selective vedolizumab (VDZ) vs those receiving systemic anti-tumor necrosis factor (anti-TNF) therapies. Methods: Adult IBD patients receiving VDZ or anti-TNFs were identified from the MarketScan claims database from September 28, 2012, through September 30, 2016...

RATIONALE: Sclerosing thymoma is an extremely rare mediastinal neoplasm; it was recognized for the first time in 1994 and to date only 15 cases have been reported. PATIENT CONCERNS: The present study report a case of a 65-year-old man who was incidentally found to have an anterior mediastinal nodule, without clinical symptoms including fever, chest pain, and myasthenia gravis. DIAGNOSES: The chest computed tomography (CT) revealed the nodule was 4...

Severe alcoholic hepatitis (AH) is a life-threatening condition lacking good serologic markers to tailor treatment and predict recovery. We examined the cholesterol metabolism in severe AH to explore prognostic markers and evaluate the profile of cholesterol precursors, cholestanol and phytosterols, in this context. We assessed serum cholesterol, cholesterol precursors, cholestanol, phytosterols, and biochemical markers in 24 patients with severe AH treated with prednisolone and randomized to ciprofloxacin in the ratio 1:1...

BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...

Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied...

BACKGROUND: Up to 3.4% of Crohn's disease (CD) patients will be diagnosed with concomitant primary sclerosing cholangitis (PSC). Despite the worldwide increase incidence of CD, data on the clinical characteristics of PSC-CD patients are scarce. OBJECTIVES: To clinically characterize CD in patients who have concomitant PSC. METHODS: A retrospective case-control analysis was conducted with 18 CD patients with concomitant PSC who attended the Inflammatory Bowel Disease Center at the Tel Aviv Sourasky Medical Center between 2011-2014 (PSC-CD patients)...

We report our experience of renal disease associated with bodybuilders who had been on high-protein diet, anabolic androgenic steroids (AASs), and growth hormone (GH) for years. A total of 22 adult males who volunteered information about use of high protein diet and AAS or GH were seen over a six-year period with renal disease. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) in eight, nephroangiosclerosis in four, chronic interstitial nephritis in three, acute interstitial nephritis in two, nephrocalcinosis with chronic interstitial nephritis in two, and single patients with membranous glomerulopathy, crescentic glomerulopathy, and sclerosing glomerulonephritis...

PURPOSE OF REVIEW: The group of sclerosing bone disorders encompasses a variety of disorders all marked by increased bone mass. In this review, we give an overview of the genetic causes of this heterogeneous group of disorders and briefly touch upon the value of these findings for the development of novel therapeutic agents. RECENT FINDINGS: Advances in the next-generation sequencing technologies are accelerating the molecular dissection of the pathogenic mechanisms underlying skeletal dysplasias...