Search results for "Retrospective"

Guidelines for palliative surgery of cholangiocarcinoma

2008

AbstractThe aims of the guidelines are to help assess the evidence for palliation surgery in patients with cholangiocarcinoma (CCA). The guidelines are classified in accordance with the location of the primary lesion, i.e. intrahepatic, hilar, and distal. They are based on comprehensive literature surveys, including results from randomized controlled trials, systematic reviews and meta-analysis, and cohort, prospective, and retrospective studies. Intrahepatic CCA, i.e. resection of lymph-node-positive tumors and R1/R2 resections have not been shown to provide survival benefit: Evidence levels: 2b, 4; Recommendation grade C. Hilar CCA: R1 resection is justified as a very efficient palliation…

The use of infliximab in pediatric ulcerative colitis: A retrospective survey

2006

Critical assessment of chitotriosidase analysis in the rational laboratory diagnosis of children with Gaucher disease and Niemann-Pick disease type A…

2006

Laboratory diagnosis of lysosomal storage disorders, especially sphingomyelinase deficiency (Niemann–Pick disease type A/B) and Niemann–Pick disease type C (NPC) can be challenging. We therefore aimed to analyse the feasibility of first-step screening with specific chitotriosidase cut-off values in children ≤ 10 years of age with visceral organomegaly (hepatomegaly, splenomegaly, or hepatosplenomegaly) in whom a storage disorder was suspected. We conducted a retrospective, cross-sectional, referral, single-centre study to assess diagnostic test properties in 106 individuals. Median chitotriosidase activity was 12 655 nmol/h per ml (interquartile range 4693–20982) in Gaucher disease (GD); 78…

Systematic search for neutropenia should be part of the first screening in patients with poikiloderma.

2011

Poikiloderma occurs in a number of hereditary syndromes, the best known of which is Rothmund-Thomson syndrome (RTS). Differential diagnoses include Dyskeratosis Congenita (DC) with high genetic heterogeneity and Clericuzio-type Poikiloderma with Neutropenia (CPN) due to mutations in the C16orf57 gene. Mutations in the RECQL4 gene are only observed in two thirds of RTS patients. In this study, 10 patients referred for syndromic poikiloderma and negative for RECQL4 sequencing analysis were investigated for C16orf57 mutations. Two C16orf57 heterozygous nonsense mutations (p.W81X and p.Y89X) were identified in a 5-year-old female child presenting with generalized poikiloderma, dental dysplasia,…

LI-RADS ancillary features favoring benignity: is there a role in LR-5 observations?

2021

The Liver Imaging Reporting and Data System algorithm allows category downgrade in the presence of ancillary features (AFs) favoring benignity, even in observations categorized as LR-5. This study aims to assess the role of AFs favoring benignity in LR-5 observations and their impact on category downgrade. This study included high-risk patients with at least one LR-5 observation imaged with gadoxetate disodium MRI. Three readers with different experience levels independently evaluated the presence of AFs favoring malignancy (not hepatocellular carcinoma (HCC) in particular and HCC in particular) and AFs favoring benignity. Category downgrade was considered possible in the presence of ≥ 1 AF…

Treatment Persistence and Safety of Apremilast in Psoriasis: Experience With 30 Patients in Routine Clinical Practice

2020

Apremilast is a phosphodiesterase-4 inhibitor taken orally. Little information about its use in routine clinical practice is available. We aimed to assess treatment safety and persistence rates in patients on apremilast for different forms of plaque psoriasis. This observational retrospective study included 30 patients with psoriasis who were treated with apremilast between January 2016 and December 2017 in our hospital. Twelve patients had palmar-plantar psoriasis, 8 had plaque psoriasis mainly on the scalp, and 10 had plaque psoriasis in other locations. The probable period of treatment persistence in patients in the 50th percentile was 18.5 months according to survival analysis of the se…

Lipoprotein apheresis for Lp(a)-hyperlipoproteinemia with progressive cardiovascular disease--Additional particular aspects of the Pro(a)LiFe multice…

2015

Lipoprotein apheresis (LA) can lower LDL-cholesterol and Lp(a) by 60%-70% and is the final escalating option in patients with hyperlipoproteinemias involving LDL or Lp(a) particles. Major therapeutic effect of LA is preventing cardiovascular events. In Germany since 2008 a reimbursement guideline has been implemented accepting to establish the indication for LA not only for familial or severe forms of hypercholesterolemia but also based on Lp(a)-hyperlipoproteinemia associated with a progressive course of cardiovascular disease, that persists despite effective treatment of other concomitant cardiovascular risk factors. The Pro(a)LiFe-study confirmed with a prospective multicenter design tha…

Retrospective Analysis of Whole-Body Magnetic Resonance Imaging of Bone Manifestations in Long-Term Treated Patients with Gaucher Disease Type 1

2018

We retrospectively assessed bone and visceral manifestations in patients with Gaucher disease type 1 (GD1) with whole-body magnetic resonance imaging (WB-MRI) to determine the effects of different timing in initiating long-term enzyme replacement therapy.In 17 patients with GD1, we performed 2 WB-MRI examinations at a median interval of 13 months. Patients had received enzyme replacement therapy with alglucerase/imiglucerase for a median of 13 years prior to the first examination. MRI results were retrospectively stratified based on treatment initiation into 2 groups: "early" (age ≤12 years, median 5 years) and "late" (during adulthood, median 32 years). We evaluated occurrence of irreversi…

Systematic review and meta-analysis of cardiovascular risk in rheumatological disease: Symptomatic and non-symptomatic events in rheumatoid arthritis…

2022

Abstract Although each autoimmune disease is associated with specific tissue or organ damage, rheumatic diseases share a pro-inflammatory pattern that might increase cardiovascular risk. Retrospective and prospective studies on patients affected by systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) highlighted the concept of “accelerated atherosclerosis”. Therefore, the purpose of this systematic review and meta-analysis is the assessment of symptomatic or asymptomatic cardiovascular events among patients with rheumatic diseases as RA and SLE. The literature research obtained all manuscripts published in the English language between 2015 and 2019 for a total of 2355 manuscript…

Perioperative chemotherapy versus surgery alone for resectable colorectal liver metastases: an international multicentre propensity score matched ana…

2021

Abstract Background There is still uncertainty regarding the role of perioperative chemotherapy (CTx) in patients with resectable colorectal liver metastases (CRLM), especially in those with a low-risk of recurrence. Methods Multicentre retrospective analysis of patients with CRLM undergoing liver resection between 2010–2015. Patients were divided into two groups according to whether they received perioperative CTx or not and were compared using propensity score matching (PSM) analysis. Then, they were stratified according to prognostic risk scores, including: Clinical Risk Score (CRS), Tumour Burden Score (TBS) and Genetic And Morphological Evaluation (GAME) score. Results The study includ…