idiopathic thrombotic microangiopathies: Topics by WorldWideScience.org

Idiopathic (primary) achalasia

Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

2011-01-01

Selective endothelin A-receptor blockade attenuates coronary microvascular dysfunction after coronary stenting in patients with type 2 diabetes

Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition. It is a rare...Full Text Available

Ultrasonography and color Doppler of proximal gluteal enthesitis in juvenile idiopathic arthritis: a descriptive study

Background:Endothelin-1 may be involved in the development of diabetic microangiopathy. We studied the effect of endothelin-1 blockade on myocardial microcirculation during coronary...Full Text Available

2009-01-01

The use of etoricoxib to treat an idiopathic stabbing headache: a case report

BackgroundThe presence of enthesitis (insertional inflammation) in patients with juvenile idiopathic arthritis (JIA) is difficult to establish clinically and may influence classification...Full Text Available

Immature cell populations and an erythropoiesis gene-expression signature in systemic juvenile idiopathic arthritis: implications for pathogenesis

According to the International Headache Society, idiopathic stabbing headache (ISH), an indomethacin-responsive headache syndrome, is a paroxysmal disorder of short duration manifested as head pain...Full Text Available

Idiopathic venous thromboembolism and thrombophilia

IntroductionPrevious observations suggest that active systemic juvenile idiopathic arthritis (sJIA) is associated with a prominent erythropoiesis gene-expression signature. The aim...Full Text Available

2010-01-01

Combined photodynamic therapy and intravitreal bevacizumab for idiopathic polypoidal choroidal vasculopathy: one-year follow-up

During the past decade idiopathic venous thromboembolism has become a separate entity, a chronic illness which has required prolonged anticoagulation and other prevention strategies to avoid recurrences....Full Text Available

2011-02-15

Abnormalities of GATA-1 in Megakaryocytes from Patients with Idiopathic Myelofibrosis

ObjectiveTo report the efficacy and safety of combined photodynamic therapy (PDT) and intravitreal bevacizumab (IVB) injection in the treatment of idiopathic polypoidal choroidal...Full Text Available

2010-01-01

Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation

The abnormal megakaryocytopoiesis associated with idiopathic myelofibrosis (IM) plays a role in its pathogenesis. Because mice with defective expression of transcription factor GATA-1 (GATA-1^low...Full Text Available

2005-09-01

Personalized healthcare in clotting disorders

Disseminated intravascular coagulation (DIC) is a disorder characterized by both acute generalized, widespread activation of coagulation, which results in thrombotic complications due to the intravascular...Full Text Available

Nonbacterial thrombotic endocarditis associated with cancer of unknown origin complicated with thrombus in the left auricular appendage: case report

In terms of managing thrombotic disorders, genotype-based individualized patient care emerged as early as 1994 when the association of factor V Leiden (G1691A), and later, prothrombin (G20210A),...Full Text Available

2010-01-01

Loss of expression of neutrophil proteinase-3: a factor contributing to thrombotic risk in paroxysmal nocturnal hemoglobinuria

A 63-year-old man was admitted to our hospital with a complaint of right lateroabdominal pain. He was diagnosed with metastatic colon cancer, and then developed multiple brain embolic infarctions 7...Full Text Available

Intravascular ultrasound to guide the management of intracoronary thrombus: a Case Report

BackgroundA deficiency of specific glycosylphosphatidyl inositol-anchored proteins in paroxysmal nocturnal hemoglobinuria may be responsible for most of the clinical features of...Full Text Available

2011-07-01

Angiogenic cytokines and growth factors in systemic sclerosis.

Percutaneous coronary intervention can be associated with distal embolization of thrombotic material causing myocardial necrosis and infarction.We discuss the role of intravascular imaging to...Full Text Available

Pulmonary vein ablation for idiopathic atrial fibrillation: six month outcome of first procedure in 100 consecutive patients

Systemic sclerosis is an autoimmune connective tissue disorder characterized by a widespread microangiopathy, autoimmunity and fibrosis of the skin and of various internal organs. Microangiopathy is characterized by a reduced capillary density and an irregular chaotic architecture that lead to chronic tissue hypoxia. Despite the hypoxic conditions, there is no evidence for a sufficient compensative angiogenesis in SSc. Furthermore, vasculogenesis is also impaired. An imbalance between angiogenic and angiostatic factors might explain the pathogenetic mechanisms of SSc vasculopathy. As far as angiogenic factors are concerned, within the most important are vascular endothelial growth factor (VEGF) and its receptors, platelet derived growth factor (PDGF), transforming growth factor beta (TGF-?), fibroblast growth factor -2 (FGF-2), angiopoietin 1 (Ang-1), stromal cell-derived factor 1 (SDF-1/CXCL12), endothelin-1 (ET-1), monocyte chemoattractant ...

2011-04-28

Positional Stretching of the Coracohumeral Ligament on a Patient with Adhesive Capsulitis: A Case Report

Objectives: To report six month outcome in patients undergoing their first pulmonary vein ablation procedure for idiopathic atrial fibrillation (AF) at a “non-pioneering”...Full Text Available

2005-01-01

Paradoxical function for the receptor for advanced glycation end products in mouse models of pulmonary fibrosis

Idiopathic frozen shoulder is a common medical diagnosis for patients seeking physical therapy. Radiographic and surgical evidence exists that describes the coracohumeral ligament (CHL) as a major contributor...Full Text Available

2009-01-01

Increased wind-up to heat pain in women with a childhood history of functional abdominal pain

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor survival. The identification of therapeutic targets is essential to improving outcomes. Previous studies found that expression...Full Text Available

2011-03-31

Disability and Psychologic Distress in Patients with Nonspecific and Specific Arm Pain

Idiopathic or functional abdominal pain (FAP) is common in school-age children and typically reflects a functional gastrointestinal disorder (FGID). FGIDs in adults have been distinguished by...Full Text Available

2011-04-01

1 2

Pirfenidone in idiopathic pulmonary fibrosis: the CAPACITY program

Psychological illness influences the experience and expression of pain and disability. We tested three null hypotheses: (1) patients with nonspecific pain (medically unexplained and idiopathic) and...Full Text Available

2008-11-01

British Library Electronic Table of Contents (United Kingdom)

Idiopathic pulmonary fibrosis is the most lethal form of diffuse lung fibrosis, killing approximately half of those affected within 2-3 years of diagnosis. Until recently, no therapies had been shown to have an impact on disease progression. The Clinical Studies Assessing Pirfenidone (Esbriet) in IPF: Research of Efficacy and Safety Outcomes (CAPACITY) program comprised two almost identical double-blind placebo-controlled studies assessing the effects of pirfenidone on change in forced vital capacity, the primary end point, over a 72-week period. One of these studies was positive, matching in magnitude the benefit seen in two previous positive Japanese studies. The other study did not meet its primary end point but positive trends were consistent in this and a number of secondary end point...

2011-01-01

Preoperative factors predictive of postoperative decimal visual acuity ? 1.0 following surgical treatment for idiopathic epiretinal membrane

An evaluation of the utility of routine laboratory monitoring of juvenile idiopathic arthritis (JIA) patients using non-steroidal anti-inflammatory drugs (NSAIDs): a retrospective review

Purpose:To report the preoperative best-corrected visual acuity (BCVA) and foveal thickness (FT) values that lead to a postoperative decimal BCVA of ≥1.0 after surgical removal...Full Text Available

2011-01-01

Highlights from the III International Symposium of Thrombosis and Anticoagulation (ISTA), October 14?16, 2010, S?o Paulo, Brazil

BackgroundNo consensus evidence-based guidelines for the routine laboratory monitoring of children with JIA receiving non-steroidal anti-inflammatory drugs (NSAIDs) exist. The purpose...Full Text Available

British Library Electronic Table of Contents (United Kingdom)

To discuss and share knowledge around advances in the care of patients with thrombotic disorders, the Third International Symposium of Thrombosis and Anticoagulation was held in S?o Paulo, Brazil, from October 14?16, 2010. This scientific program was developed by clinicians for clinicians, and was promoted by four major clinical research institutes: the Brazilian Clinical Research Institute, the Duke Clinical Research Institute of the Duke University School of Medicine, the Canadian VIGOUR Centre, and the Uppsala Clinical Research Center. Comprising 3?days of academic presentations and open discussion, the symposium had as its primary goal to educate, motivate, and inspire internists, cardiologists, hematologists, and other physicians by convening national and international visionaries, th...

2011-01-01

Use of sodium chromate Cr/sub 51/ in diagnosing childhood idiopathic pulmonary hemosiderosis

Idiopathic renal hematuria in a military working dog.

The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy spece Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases.

1984-08-01

[Pulmonary vascular manifestations in hereditary hemorrhagic telangiectasia].

A 1.5-year-old male Belgian Malinosis Military Working Dog presented with a 1-month history of intermittent hematuria. Diagnostic ultrasound and contrast radiography demonstrated large blood clots in the urinary bladder and a filling defect in the right renal pelvis. At surgery, clotted blood was present in the right ureter and bladder. Following right nephrectomy, the dog returned to training. One month later, elevations in urea nitrogen and creatinine were noted. Hematuria recurred at 3 months and the dog was found dead in its kennel. Necropsy showed a blood-filled left renal pelvis and ureter. PMID:1454185

1992-10-01

Treatment of asymptomatic internal resorption of a maxillary premolar tooth in a military working dog.

Hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber disease) is a genetic disorder with autosomal dominance, variable penetrance, and an estimated prevalence of 1/10,000 inhabitants in France. Diagnosis is based on clinical criteria including epistaxis, telangiectasia, visceral manifestations, and familial occurrence. Pulmonary arteriovenous malformations, present in 15-33% of patients, are its primary visceral complications. The disease may be revealed by infectious and ischemic neurological manifestations due to paradoxical embolism. The high frequency of neurologic complications even in asymptomatic patients justifies systematic screening for pulmonary arteriovenous malformations. Treatment of these malformations is based on percutaneous transcatheter coil embolization of the feeding artery. Pulmonary arterial hypertension is rare in this disease. It may be due to systemic arteriovenous shunting in the liver, which increases cardiac output, or be similar to ...

2005-11-01

The use of peritoneal venous shunting for intractable neonatal ascites: a short case series

An asymptomatic pink discoloration of a maxillary right fourth premolar tooth was discovered during a routine oral examination on a 9 year-old Belgian Malinois dog. A radiolucent lesion was seen in the pulpal chamber on radiographic examination. The lesion had perforated the mesiobuccal root of the tooth. The primary differential diagnosis was idiopathic internal resorption. The tooth was treated by partial resection (removal of the mesiobuccal root and associated crown). A vital pulpotomy and amalgam restoration was performed on the remaining tooth structure. A follow-up 1 year later demonstrated a successful treatment outcome. The animal was asymptomatic and able to perform military duties. Clinical and radiographic signs of healing were evident and the tooth was functional. PMID:10518874

1998-12-01

British Library Electronic Table of Contents (United Kingdom)

Introduction: Intractable ascites in neonates has a varied etiology; and often, the cause is idiopathic. The management usually consists of observation, diuretics, paracentesis, albumin replacements, and self correction. However, in some cases, the above treatment remains unsuccessful. Results: We present 2 cases of intractable ascites causing metabolic abnormalities, severe protein and immunoglobulin loss, and respiratory compromise. Although the use of peritoneovenous shunts for intractable ascites has been reported previously, our cases differ in both technique and patient size. Our first patient is an ex-28-week premature, 1.4-kg infant with intractable ascites for which a peritoneal drain was initially placed. After 3 weeks and putting out nearly 300 mL of ascitic fluid daily, we plac...

2011-01-01

Mucosal Inflammation in Spondylarthritides: Past, Present, and Future

British Library Electronic Table of Contents (United Kingdom)

Spondylarthritides (SpA) and inflammatory bowel disease (IBD) are idiopathic, chronic inflammatory disorders. Although they are very distinct and well-defined entities, there is clinical and genetic evidence supporting some degree of overlap between the pathogenesis of the two. Subclinical gut inflammation is present in up to two thirds of all SpA patients and can evolve into IBD. This subclinical gut inflammation has been shown to be strongly associated with joint inflammation, providing a clue for a common pathophysiologic background. Despite extensive research progress in the field over the past few years, many questions remain unanswered. In this paper, we focus on the clinical, genetic, and pathophysiologic overlap of SpA and IBD. Furthermore, we discuss some of the targets that may i...

2011-01-01

Manganese Induces IGF-1 and Cyclooxygenase-2 Gene Expressions in the Basal Hypothalamus during Prepubertal Female Development

British Library Electronic Table of Contents (United Kingdom)

Precocious puberty is a significant child health problem, especially in girls, because 95% of cases are idiopathic. Our earlier studies demonstrated that low-dose levels of manganese (Mn) caused precocious puberty via stimulating the secretion of luteinizing hormone-releasing hormone (LHRH). Because glial-neuronal communications are important for the activation of LHRH secretion at puberty, we investigated the effects of prepubertal Mn exposure on specific glial-derived puberty-related genes known to affect neuronal LHRH release. Animals were supplemented with MnCl2 (10 mg/kg) or saline by gastric gavage from day 12 until day 22 or day 29, then decapitated, and brains removed. The site of LHRH release is the medial basal hypothalamus (MBH), and tissues from this area were analyzed by real-...

2011-01-01

Assessment of chromosomal abnormalities in sperm of infertile men using sperm karyotyping and multicolour fluorescence in situ hybridization (FISH)

Percutaneous intervention in the menagement of insufficient native arteriovenous hemodialysis fistulae

Individuals with male factor infertility resulting from idiopathic oligo-, astheno- or teratozoospermia are frequently offered IVF in an attempt to increase their chances of having a child. A concern remains whether these infertile males have an elevated risk of transmitting chromosomal abnormalities to their offspring. Sperm chromosomal complements from these men were assayed using the human sperm/hamster oocyte fusion system and fluorescence in situ hybridization (FISH) on sperm nuclei. For each of 5 infertile patients, 100 sperm karyotypes were analyzed and multicolour FISH analysis was performed on a minimum of 10,000 sperm nuclei for each chromosome-specific DNA probe for chromosomes 1 (pUC1.77), 12 (D12Z3), X (XC) and Y (DYZ3). As a group, the infertile patients showed increased frequencies of both numerical ({chi}{sup 2}=17.26, {proportional_to} <0.001) and total abnormalities ({chi}{sup 2}=7.78, {proportional_to} <0.01) relative to control donors when ...

1994-09-01

One-year follow-up of the effects of sildenafil on pulmonary arterial hypertension and veno-occlusive disease

To determine the effectiveness and patency of percutaneous intervention in insufficient native arteriovenous hemodialysis fistulae (AVFs). Between March 1997 and September 2001, 67 cases of insufficient native AVFs resulted from central vein lesion, PTA was performed in 48 cases, and thrombolytic therapy with or without PTA in 11. In eight of the cases, in which central vein stenosis had led to the insufficency, percutaneous transluminal angioplasty (PTA) was performed, and in three of the eight, a stent was inserted. Angiography findings and complications, as well as success and patency rates in the non-thrombosis and thrombosis group, were evaluated; the central vein lesion group was analysed separately. Among 84 lesions observed at angiography, three were 54 cases of stenosis, 17 of occlusion, and 13 of combined thrombosis. The lesions were located in a proximal vein (n=51), distal vein (n=14), artery (n=6), and at the site of anastomosis (n=13). In the central vein lesion group ...

2002-11-01

Scientific Electronic Library Online (English)

Abstract in english We hypothesized that chronic oral administration of the phosphodiesterase-5 inhibitor sildenafil could improve the exercise capacity and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) on the basis of previous short-term studies. We tested this hypothesis in 14 subjects with PAH, including seven patients with the idiopathic form and seven patients with atrial septal defects, but no other congenital heart abnormalities. Patients were subjected (more) to a 6-min walk test and dyspnea was graded according to the Borg scale. Pulmonary flow and pressures were measured by Doppler echocardiography. Patients were given sildenafil, 75 mg orally three times a day, and followed up for 1 year. Sildenafil therapy resulted in the following changes: increase in the 6-min walk distance from a median value of 387 m (range 0 to 484 m) to 462 m (range 408 to 588 m; P

2005-02-01

Citrat og nyresten

DEFF Research Database (Denmark)

Citrate is an important naturally occurring inhibitor of calcium stone formation in urine. Urinary citrate excretion was examined in 43 consecutive patients with recurrent idiopathic calcium nephrolithiasis and in 50 normal controls by a specific enzymatic technique. Hypocitraturia (<1.6 mmol/24h) was found in 14 (33%) stone formers compared to 6 (12%) normal controls (p = 0.03). Citrate excretion expressed as citrate-creatinine ratio in 24 hour urine samples was significantly lower in the stone formers than in the healthy controls (p = 0.03), and significantly lower in healthy men, compared to healthy females (p = 0.006). There was a great variability in urinary citrate levels in both groups, and a considerable overlap in the urinary citrate excretion between normal subjects and stone formers. Factors other than urinary citrate excretion must therefore be of importance in the pathophysiology of calcium stone formation. Citrate-calcium ratio in urine proved to ...

1993-01-01

Pituitary disease in childhood: utility of magnetic resonance; Patologia hipofisaria en la edad pediatrica: unidad de la resonance magnetica

Disorders of brain development and phakomatosis

To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone ...

2000-07-01

International Nuclear Information System (INIS)

Full text: Disorders of brain development and phakomatosis are resulting from disturbed embryonic-foetal development One third of all major embryological anomalies involve CNS, and over 2000 different anomalies have been described. Anomalies of the brain often cause foetal and neonatal death, and mental and physical retardation in pediatric group. The majority of disorders of brain development and phakomatosis are idiopathic, and most of them are not hereditary or familial. Ultrasonography plays the important role in screening foetal and neonatal brain, but after closure of fontanels it is difficult to find the acoustic window. CT has limited contrast resolution, and disadvantage exposing infant to ionizing radiation. It is helpful to demonstrate the presence of calcifications. MR imaging has proved to be a diagnostic tool of major importance in children with disorders of brain development and phakomatosis. The excellent grey/white matter differentiation and ...

Assessment of bone formation and bone resorption in osteoporosis: a comparison between tetracycline-based iliac histomorphometry and whole body /sup 85/Sr kinetics

SPIO-enhanced MR angiography for the detection of venous thrombosis in an animal model; SPIO-unterstuetzte MR-Angiographie zur Detektion venoeser Thromben im Tiermodell

Bone formation and resorption have been measured in patients with idiopathic osteoporosis by histomorphometry of 7.5-mm trephine biopsies and in the whole body by 85Sr radiotracer methodology and calcium balances. The studies were synchronized and most were preceded by double in vivo tetracycline labeling. Correlations between histological and kinetic bone formation indices were better when better when based on the extent of double tetracycline labels than on measurements of osteoid by visible light microscopy. Correction of the kinetic data for long-term exchange, using 5 months' serial whole body counting of retained 85Sr, improved the fit of the kinetic to the histological data. A statistical analysis of the measurement uncertainties showed that the residual scatter in the best correlations (between exchange-corrected bone formation rates and double-labeled osteoid surface indices) could be attributed to measurement imprecision alone. The ...

1987-12-01