Search results for "Arid"
showing 10 items of 1382 documents
Soluble CD14 monocyte antigen in suction blister fluid and serum of patients with psoriasis.
1993
The purpose of this study was to measure soluble CD14 (sCD14) molecules in the skin and in serum of patients with psoriasis. CD14 is a newly discovered cell surface marker on monocytes that is shed after cell activation. The following procedures were used: suction blisters were raised over the abdominal skin of 9 healthy control individuals and 8 patients with psoriasis. Serum of 17 healthy controls and 17 patients with psoriasis was collected. sCD14 was determined in suction blister fluid and serum by the ELISA technique. The clinical status of psoriasis was rated by the psoriasis area and severity index (PASI score). We found that sCD14 levels in suction blisters of healthy skin (1,050 +/…
Focal nodular hyperplasia in normal and fatty liver: a qualitative and quantitative evaluation with contrast-enhanced ultrasound
2004
The aim of this study was to describe gray-scale appearance of liver parenchyma and focal nodular hyperplasia (FNH) by pulse inversion (PI) ultrasound (US) at baseline and after contrast agent administration in patients with normal and fatty liver. Sixteen consecutive patients (12 women, 4 men) with 29 previously diagnosed FNHs (15 of 29 located in normal liver and 14 of 29 in fatty liver) underwent PI US before and after SH U 508A (Levovist) injection. Signal intensity values were measured within the FNHs and the adjacent liver parenchyma in selected images. Baseline echogenicity of fatty liver was higher (15.19 +/- 2.90 dB +/- SD) than normal liver (10.91 +/- 3.15 dB +/- SD; p<0.001). Aft…
Mortality and cause of death in mucopolysaccharidosis type II-a historical review based on data from the Hunter Outcome Survey (HOS).
2009
Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a progressive, multisystemic disease caused by a deficiency of iduronate-2-sulfatase. Patients with the severe form of the disease have cognitive impairment and typically die in the second decade of life. Patients with the less severe form do not experience significant cognitive involvement and may survive until the fifth or sixth decade of life. We studied the relationship of both severity of MPS II and the time period in which patients died with age at death in 129 patients for whom data were entered retrospectively into HOS (Hunter Outcome Survey), the only large-scale, multinational observational study of patients with MPS II.…
Pravastatin reverses the membrane cholesterol reorganization induced by myocardial infarction within lipid rafts in CD14(+)/CD16(-) circulating monoc…
2012
International audience; Large numbers of monocytes are recruited in the infarcted myocardium. Their cell membranes contain cholesterol-rich microdomains called lipids rafts, which participate in numerous signaling cascades. In addition to its cholesterol-lowering effect, pravastatin has several pleiotropic effects and is widely used as secondary prevention treatment after myocardial infarction (MI). The aim of this study was to investigate the effects of pravastatin on the organization of cholesterol within monocyte membrane rafts from patients who had suffered myocardial infarction. Monocytes from healthy donors and acute MI patients were cultured with or without 4μM pravastatin. Lipid raf…
Prevalence and characterization of cardiac involvement in Hunter syndrome.
2011
To assess the prevalence of cardiovascular signs and symptoms in a large group of patients with Hunter syndrome, an X-linked metabolic disorder caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase.The Hunter Outcome Survey was established to characterize the natural history of Hunter syndrome and to assess the response to enzyme replacement therapy. Echocardiographic and electrocardiographic examination results were available for 102 patients who were enzyme replacement therapy-naïve in the Hunter Outcome Survey (median age at examination, approximately 8 years) as of Jan 23, 2009.The most common cardiovascular finding was valve disease, which was present in 63% of patients.…
Alterations in speech and voice in patients with mucopolysaccharidoses.
2013
The mucopolysaccharidoses are a group of lysosomal disorders characterized by abnormal accumulation of glycosaminoglycans within cell lysosomes leading to a variety of signs and symptoms including alterations in speech and voice production. These changes were analysed in 44 patients with mucopolysaccharidosis (MPS) types I, II, and VI using standardized protocols. Compared to healthy individuals the diadochokinetic rate for the fast repetition of syllables was slower and more irregular, the voice-onset time for the voiceless consonant /p/ was shorter, and most patients had a hoarse voice. The fundamental frequency (F0) of sustained spoken vowels was in the normal range for most women and ch…
Lung Preservation With Perfadex or Celsior in Clinical Transplantation: A Retrospective Single-Center Analysis of Outcomes.
2015
BACKGROUND Despite improvement of lung preservation by the introduction of low-potassium dextran (LPD) solution, ischemia-reperfusion injury remains a major contributor to early post-lung transplant graft dysfunction and mortality. After favorable experimental data, Celsior solution was used in our clinical lung transplant program. Data were compared with our historic LPD cohort. METHODS Between January 2002 and January 2005, 209 consecutive lung transplantations were performed with LPD. These were compared to 208 transplants between February 2005 and September 2007 with Celsior. Endpoints included posttransplant PaO2/FiO2 ratio at different timepoints after intensive care unit (ICU) admiss…
Effects of enzyme replacement therapy on growth in patients with mucopolysaccharidosis type II
2010
Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is an X-linked, recessive, lysosomal storage disorder caused by deficiency of iduronate-2-sulfatase. It has multisystemic involvement, with manifestations in the brain, upper respiratory tract, heart, abdomen, joints and bones. Bone involvement leads to decreased growth velocity and short stature in nearly all patients. A therapeutic option for patients with MPS II is enzyme replacement therapy (ERT) with idursulfase (Elaprase®). We compared annual growth rates before and during ERT in 18 patients from Mainz, Germany, and Manchester, UK. Group 1 included nine patients who started ERT before 10 years of age; group 2 contained nine patie…
Safety and Efficacy of Microporous Polysaccharide Hemospheres in Neurosurgery
2011
BACKGROUND: Effective hemostasis is mandatory for brain tumor surgery. Microporous polysaccharide hemosphere (MPH) powder, a white powder compounded from potato starch, was recently introduced for surgical and emergency application. OBJECTIVE: To evaluate the safety and efficacy of MPHs in brain tumor surgery. METHODS: Thirty-three patients (mean age, 58 years; range, 22-84 years) underwent microsurgical brain tumor resection. Final hemostasis was performed by topical application of MPHs, video recorded, and subsequently analyzed. Blood samples were taken before surgery, before application of hemospheres, and postoperatively. Volume measurements of the tumor, resection cavity, and postopera…
Wheat consumption leads to immune activation and symptom worsening in patients with familial mediterranean fever : a pilot randomized trial
2020
We have identified a clinical association between self-reported non-celiac wheat sensitivity (NCWS) and Familial Mediterranean Fever (FMF). Objectives: A) To determine whether a 2-week double-blind placebo-controlled (DBPC) cross-over wheat vs. rice challenge exacerbates the clinical manifestations of FMF