Systemic sclerosis بالعربي

Systemic sclerosis (SSc) is a multisystem autoimmune disease in which there is increased fibroblast activity resulting in abnormal growth of connective tissue. This causes vascular damage and fibrosis. Fibrosis occurs in skin, the gastrointestinal (GI) tract, heart, lungs and other internal organs Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries.There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above. Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin (see the image below), subcutaneous tissue, muscles, and internal organs (eg, alimentary tract, lungs, heart, kidney, CNS); and immunologic disturbances accompany these findings Progressive Systemic Sclerosis. Progressive systemic sclerosis (PSS) is a condition associated with thickening of the skin and tethering to subcutaneous tissues as well as smooth muscle atrophy and fibrosis of internal organs such as the gastrointestinal tract, lungs, heart, and kidneys. From: Textbook of Clinical Neurology (Third Edition), 200

Systemic sclerosis (SS) is an autoimmune disorder. This means it's a condition in which the immune system attacks the body. Healthy tissue is destroyed because the immune system mistakenly. Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin (see the.

Progressive systemic sclerosis occurs more frequently in females, especially during the peak incidence years of 30 to 55, where the female-to-male ratio may be as high as 12 to 1. Major mechanisms accounting for the pathological changes in progressive systemic sclerosis include generalized fibrosis, systemic microangiopathy, and. Systemic sclerosis is a potentially devastating disease in which the aetiology and pathogenesis has not yet been fully understood. It has been associated with occupational exposure to silica, vinyl chloride, solvents and other chemical agents sclerosis بالعربي - ترجمة عربية لكلمة sclerosis برعاية Britannica English، قاموس وترجمة عربي - إنجليزي مجّانيّ، قاموس شامل ومعاصر يتيح تعلّم الإنجليزيّة، ويشمل: ترجمة كلمات وجمل، لفظ صوتيّ، أمثلة استخدام، تشكيل كامل للعربيّة، تحليل. Systemic sclerosis is not to be confused with a separate condition called localised scleroderma, which just affects the skin. The word scleroderma, which is an older term, specifically means hard skin. The following information is about the condition systemic sclerosis, in which skin and connective tissue inside the body are affected

Have a diagnosis of diffuse cutaneous SSc defined as systemic sclerosis with skin thickening on the upper arms proximal to the elbows, on the upper legs proximal to the knees, or on the trunk. Have skin thickening in a body area suitable for repeat biopsy. Have a mRSS at Screening of ≥15 to ≤40. FVC ≥50% of predicted at Screening The exact, underlying cause of systemic sclerosis is unknown. The cause appears to involve some injury to the cells that line blood vessels, resulting in excessive activation of dermal connective tissue cells, called fibroblasts.Fibroblasts normally produce collagen and other proteins. Build-up of collagen in the skin and other organs causes the signs and symptoms of the condition Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and kidneys) Juvenile systemic sclerosis is a rare multisystemic disease characterized by inflammation, vascular abnormalities and fibrosis that affects the skin and various internal organs . Onset of the disease occurs mainly in adults, but onset during childhood is reported in about 10% of cases with a mean age at onset of 8 years

Systemic Sclerosis (Scleroderma)

  1. INTRODUCTION. Systemic sclerosis (SSc) is a complex autoimmune rheumatic disease that is characterised by widespread skin (scleroderma) and internal organ fibrosis, immune system dysregulation, and vascular alterations. 1-4 SSc is a rare rheumatological condition (prevalence: ~20 per million) and is more common in females than males (~7:1). 5 The purpose of this review is to provide a.
  2. In diffuse cutaneous systemic scleroderma, the condition worsens quickly and organ damage occurs earlier than in other types of the condition. In the third type of systemic scleroderma, called systemic sclerosis sine scleroderma (sine means without in Latin), fibrosis affects one or more internal organs but not the skin
  3. Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic.
  4. Systemic sclerosis (SSc) is a systemic connective tissue disease. Characteristics of systemic sclerosis include essential vasomotor disturbances; fibrosis; subsequent atrophy of the skin (see the image below), subcutaneous tissue, muscles, and internal organs (eg, alimentary tract, lungs, heart, kidney, CNS); and immunologic disturbances acco..
  5. Systemic sclerosis (systemic scleroderma) is a connective tissue disease associated with autoimmunity, vasculopathy, and fibrosis. The annual incidence is estimated to be 10 to 20 cases per 1.
  6. systemic scleroderma: [ skle″ro-der´mah ] chronic hardening and shrinking of the connective tissues of any organ of the body, including the skin, heart, esophagus, kidney, or lung. The skin may be thickened, hard, and rigid, and pigmented patches may occur. The two main types are systemic scleroderma and localized scleroderma . The milder.

Systemic scleroderma - Wikipedi

INTRODUCTION. The systemic manifestations of systemic sclerosis (SSc, scleroderma) are diverse. Most prominent are abnormalities of the circulation (most notably Raynaud phenomenon) and involvement of multiple organ systems, including the musculoskeletal, renal, pulmonary, cardiac, and gastrointestinal (GI) systems, with fibrotic and/or vascular complications Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies

What is Progressive Systemic Sclerosis? (with pictures)Systemic Sclerosis: A Treatable Multi-System Disease - YouTube

Systemic Sclerosis: Background, Pathophysiology, Etiolog

Progressive Systemic Sclerosis - an overview

Scleroderma: Signs, Symptoms, and Complications

Systemic Sclerosis (Scleroderma): Pictures, Symptoms, Cause

STUDY TITLE: GWAS for systemic sclerosis identifies multiple risk loci and highlights fibrotic and vasculopathy pathways SUMMARY: Discovery of 13 novel genetic variants associated with the development of systemic sclerosis. OVERVIEW: Normally, the immune system works to protect the body against foreign pathogens such as bacteria and viruses.. Autoimmune diseases occur when the immune system. The subtype of systemic sclerosis is an important factor in the development and progression of PAH. PAH appears to be the major cause of death in limited cutaneous systemic sclerosis, while interstitial lung disease is the major cause of death in diffuse cutaneous systemic sclerosis. 28. A late complication of systemic sclerosis PURPOSE OF REVIEW: Systemic sclerosis (SSc) is a life-threatening autoimmune disease that causes debilitating skin fibrosis. The skin in SSc is easily accessible, and skin biopsies may provide rich biological data regarding underlying disease pathophysiology. Here, we review literature relevant to the potential for skin histology to serve as a.

Limited cutaneous systemic sclerosis (lcSSc): Skin involvement is limited to the hands/lower arms, feet/lower legs, and face. CREST Syndrome is a form of lcSSC. CREST stands for its most prominent features: calcinosis, Reynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias وسم: systemic sclerosis التصلب المجموعي (تصلب الجلد): الأسباب والأعراض والتشخيص والعلاج التصلب المجموعي systemic sclerosis SS هو اضطراب مناعي ذاتي، إذ يُعد حالة يهاجم فيها Systemic sclerosis 1. SyStemic ScleroSiS 2. Case scenario Manjees,27 yrs D1/1 Duration of illness-5 years c/o raynaud's phenomenon+fingertip ulceration Not a/w pain,h/o digital gangrene a/w skin binding down started from B/L forearms gradually increasing over the course of 4-5 years to involve whole body Restriction of hand and wrist movements Breathlessness Difficulty in swallowing with. Objectives Systemic sclerosis (SSc) is an autoimmune fibrotic disease affecting multiple tissues including the lung. A subset of patients with SSc with lung disease exhibit short telomeres in circulating lymphocytes, but the mechanisms underlying this observation are unclear. Methods Sera from the Johns Hopkins and University of California, San Francisco (UCSF) Scleroderma Centers were. Systemic sclerosis is a complex autoimmune disease characterized by a chronic and frequently progressive course and by extensive patient-to-patient variability. Like other autoimmune diseases, systemic sclerosis occurs more frequently in women, with a peak of onset in the fifth decade of life

Key Difference - Multiple Sclerosis vs Systemic Sclerosis Both multiple sclerosis and systemic sclerosis are autoimmune diseases whose pathogenesis is triggered by undiscovered environmental and genetic factors. Multiple Sclerosis is a chronic autoimmune, T-cell mediated inflammatory disease affecting the central nervous system whereas systemic sclerosis, also known as scleroderma, is an. Systemic sclerosis (SSc), also called scleroderma, is a chronic autoimmune disorder characterized by fibrosis of the skin and various organs. Early diagnosis and classification are important so that patients can be evaluated for organ involvement and/or damage. However, because SSc is a heterogeneous disease, clinical presentation and disease course vary, and manifest

What is systemic sclerosis (SSc)? - Medscap

Systemic sclerosis MedLink Neurolog

Diffuse scleroderma is a type of systemic sclerosis (scleroderma).. Systemic scleroderma can affect any part of the body. It often affects the skin, blood vessels, and internal organs, especially the lungs, heart, kidneys, and gastrointestinal tract Systemic sclerosis is a complex autoimmune disease characterized by a chronic and frequently progressive course and by extensive patient-to-patient variability. Like other autoimmune diseases.

This is a phase 2, open-label, single-cohort, multicenter trial of belumosudil in subjects with Diffuse Cutaneous Systemic Sclerosis (dcSSc). An estimated total of 12 to 15 subjects will receive belumosudil 200 mg administered orally (PO) twice daily (BID) for 52 weeks. The primary analysis will be at 24 weeks Systemic sclerosis is a rare and heterogeneous autoimmune disease characterized by immune dysregulation, microvascular damage, and organ fibrosis. 1 Interstitial lung disease (ILD) is a common. progressive systemic sclerosis: Sclerodema An idiopathic connective tissue disorder, characterized by localized or generalized induration of skin due to ↑ collagen deposition in skin, GI tract, lungs, heart, muscle, kidneys; PSS may be confined to skin for long periods of time, but usually encroaches on the viscera, causing malabsorption,. Close monitoring of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) and awareness of the variable course of progression is highly important in considering when to initiate treatment, according to study results published in the Annals of the Rheumatic Diseases.. Some patients with SSc-ILD may develop progressive ILD, which is associated with higher mortality, and. Systemic sclerosis is a rare autoimmune disease characterized by immunological alterations, small vessel vasculopathy, and skin and other organ fibrosis. Despite progress in the knowledge of pathogenic steps and therapeutic options, when compared with other rheumatic conditions, it shows the lowest life expectancy rate. Early diagnosis is important as early treatment could lead to better.

Systemic-sclerosis-related myocardial disease death was 14% with most causes being related to arrhythmias. Curr Opin Rheumato (Expired Article). Causes of Death in Systemic Sclerosis. Mortality And Cause Of Death In An Australian Systemic Sclerosis Cohort. Pulmonary involvement in the form of PAH, ILD or both is the leading cause of death among. دليل كيفية النطق: تعلّم كيف تنطق systemic sclerosis فى الإنجليزية بطريقة المتحدث الأصلى. الترجمة الانجليزية لـ systemic sclerosis Limited systemic sclerosis (also known as sine scleroderma) is a type of systemic scleroderma that is characterized by Raynaud's phenomenon and the buildup of scar tissue (fibrosis) on one or more internal organs but not the skin. While the exact cause of limited systemic sclerosis is unknown, it is believed to originate from an autoimmune reaction which leads to the overproduction of collagen.

The WSF is the host and organizing body of the World Scleroderma Congress, which takes place every second year in differing locations. 6th Systemic Sclerosis World Congress. The first 5 congresses in Florence 2010, Madrid 2012, Rome 2014, Lisbon 2016 and Bordeaux 2018 were a significant successes Systemic sclerosis mimics include a variety of diseases that may resemble systemic connective tissue diseases such as SSc. Above all, the most common diseases are discussed in this chapter. The basis of proper diagnosis and treatment is the interdisciplinary collaboration of rheumatology and dermatology with the possibility of biopsy tissue.

called systemic sclerosis and, as such, both subgroups share some features in common, including: • Raynaud Phenomenon. This occurs in about 90 percent of patients with systemic sclerosis. • Heartburn and other esophagus problems (particularly trouble swallowing foods) When other organs are involved, systemic sclerosis is a more accurate description of the disease. Systemic sclerosis is associated with immune dysregulation, microvascular damage, and organ fibrosis. A common manifestation of systemic sclerosis is interstitial lung disease which is a leading cause of death in patients with systemic sclerosis Patients with primary biliary cirrhosis (PBC) often have concurrent limited systemic sclerosis (SSc). Conversely, up to one-fourth of SSc patients are positive for PBC-specific antimitochondrial antibodies (AMA). The mechanisms responsible for the co-occurrence of these diseases are largely unknown. Genetic, epigenetic, environmental, and infectious factors appear to be important for the. Systemic sclerosis (SSc, scleroderma) is a connective tissue disorder with unclear etiology. Patients with SSc can experience significant pain and functional limitations in affected hands. Common hand manifestations of SSc include joint contractures, skin ulcerations, digital tip ischemia, Raynaud phenomenon, and calcinosis cutis The American College of Rheumatology and European League Against Rheumatism criteria for the classification of systemic sclerosis van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55. https://www.rheumatology.org.

173 Morphoea (Localized Scleroderma) | Plastic Surgery Key

Systemic sclerosis in an anaesthetist Occupational

Introduction. Systemic sclerosis (SSc) is a connective tissue disease clinically characterized by different degrees of skin fibrosis and visceral organ involvement ().The etiology of SSc remains obscure; the disease appears to be the result of a multistep and multifactorial process, including immune system alterations, genetic and exogenous, and toxic or infectious factors () Objectives To identify overall disease course, progression patterns and risk factors predictive for progressive interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD (SSc-ILD), using data from the European Scleroderma Trials And Research (EUSTAR) database over long-term follow-up. Methods Eligible patients with SSc-ILD were registered in the EUSTAR database and had. Introduction. Systemic sclerosis (SSc) is a rare connective-tissue disease characterized by autoimmune pathogenesis, alterations to the vascular system, and fibrosis of the skin and internal organs. 1 It is estimated that about 30% of SSc patients develop digital ulcers (DUs) yearly and up to 50% of subjects develop this complication during the natural course of the disease. 2,3 DUs represent.

‫ترجمة sclerosis في العربيّة قاموس إنجليزي - عربي

Systemic sclerosis aka Scleroderma is an autoimmune connective tissue disorder. Other similar diseases include SLE, RA, Sjogren's syndrome and mixed connective tissue disease. There is a lot of overlap in the symptoms of these diseases. Many will require immunosuppressive therapy Understanding the totality of systemic sclerosis (SSc) is key to successful diagnosis and management of this rare disease. Systemic sclerosis is one of the most complex, systemic autoimmune diseases in which inflammation and fibrosis play a crucial role, leading to severe damage and failure of multiple organs. 1,2 Its cause is unknown, and it is characterized by immune system activation. Systemic sclerosis is a chronic autoimmune connective tissue disease characterized by vascular injury and fibrosis and by an impaired angiogenesis which cannot ensure an efficient vascular recovery. Vascular injury is responsible for hypoxia and tissual ischemia which are the primary triggers for angiogenesis and are not able to induce a compensatory angiogenesis Systemic sclerosis (SSc) is an autoimmune inflammatory disease with unknown etiology characterized by microvascular injury and fibrosis of the skin and certain internal organs. A growing body of evidence suggests that Fli1, which is downregulated by epigenetic mechanism in SSc dermal fibroblasts, has a pivotal role in the pathogenesis of SSc

Periarticular calcification of the hands and feet is characteristic of systemic sclerosis involving the musculoskeletal system. Other conditions where multiple sites of soft tissue periarticular calcification is seen include: gout end stage re.. INTRODUCTION. Systemic sclerosis (SSc) is a connective tissue disorder characterized by Raynaud phenomenon and other vascular abnormalities; a disturbance in fibroblast function culminating in the telltale skin thickening and fibrosis of visceral organs including the lungs, gastrointestinal tract, and heart; and immune system activation manifested by SSc-related antibodies, in particular anti. Systemic sclerosis is a rare disease. Systemic sclerosis is diagnosed in approximately 67 male patients and 265 female patients per 100,000 people each year. International. Systemic sclerosis is estimated to occur in 2.3-10 people per 1 million. Systemic sclerosis is rare in the resident population of Japan and China. Rac Introduction. Systemic sclerosis is a multisystemic autoimmune disorder affecting predominantly the skin, lungs, gut, and kidneys. The criteria to be fulfilled for labeling a patient as a case of systemic sclerosis (established by the Subcommittee for Scleroderma Criteria of the American Rheumatology Association) include one major criterion, i.e., sclerosis of skin proximal to the digits.

Systemic sclerosis Scleroderma Versus Arthriti

Systemic Sclerosis - Treatments. At present, there is no cure for systemic sclerosis, limited or diffuse. However, much can be done to help. The aims of treatment are: To relieve symptoms. To prevent the condition from progressing, as much as possible. To detect and treat complications early. To minimise any disability. Treatment aim 1 - to. Systemic sclerosis (SSc) is an autoimmune systemic disease characterized by small vessel involvement that leads to tissue ischemia and fibroblast stimulation resulting in accumulation of collagen (fibrosis) in the skin and internal organs. The peak incidence of the disease is found between the third and fifth decade of life Systemic sclerosis can be difficult to diagnose as it can develop gradually and can take different forms. There is no single test or feature which gives a yes/no answer. Instead, the diagnosis is made after looking at the whole person, taking into account their symptoms, physical examination and blood tests Systemic sclerosis (also sometimes referred to as scleroderma, systemic scleroderma, or CREST syndrome) is an autoimmune sclerosing disease of the skin and the internal organs. Autoimmune diseases occur when the immune system, which normally protects you from infections and cancer, makes a mistake and begins to attack parts of your body Systemic sclerosis is an multisystem inflammatory autoimmune condition of unknown aetiology. It is associated with fibroblast proliferation and increased collagen deposition, causing damage to vasculature and fibrosis. The term scleroderma is sometimes used interchangeably - this literally means 'hardening of the skin', one of the main.

15-Year-Old Boy with Nail Dystrophy from Lesion on Hand

Effects of FT011 in Systemic Sclerosis - Full Text View

Systemic sclerosis is a rare autoimmune disease of unknown etiology characterized by skin fibrosis and internal organ involvement. The incidence and prevalence of systemic sclerosis have been reported to vary widely with incidence estimated between 4 and 43/million person years [1,2,3], and prevalence between 88 and 443/million [4, 5].It seems the incidence and prevalence may be influenced by. Systemic sclerosis (SSc) is a disease characterized by immunologic abnormalities, microvascular involvement and tissue fibrosis. In previous studies, 10-year survival rates ranged from 50-84%. However, there are concerns that these studies, using prevalent cohorts, are underestimating mortality. You Might Also Like Low Blood Pressure Increases Risk of Death in Patients with Systemic.

Systemic scleroderma Genetic and Rare Diseases

Systemic sclerosis (SSc) is a chronic disease caused by abnormal growth of connective tissue, which leads to diffuse thickening and hardening of the skin and often the inner organs. SSc is categori.. Denton CP, Khanna D. Systemic sclerosis. Lancet 2017;390:1685-99. Allanore Y, et al. Systemic sclerosis. Nat Rev Dis Primers 2015;1:1-21. Derrett-Smith EC, Denton CP. Systemic sclerosis: clinical features and management. Medicine 2013;42(3):167-73. van den Hoogen F, et al. Classification criteria for systemic sclerosis: An ACR-EULAR. Patients with systemic sclerosis that overlaps with systemic lupus erythematous are generally younger at the time of diagnosis, more frequently demonstrate pulmonary arterial hypertension and less.

Scleroderma Symptoms - Scleroderma Education Project

Systemic Sclerosis - Musculoskeletal and Connective Tissue

Ultrasound in the Assessment of Interstitial Lung Disease in Systemic Sclerosis: A Systematic Literature Review by the OMERACT Ultrasound Group. Marwin Gutierrez, Carina Soto-Fajardo, Carlos Pineda, Alfonso Alfaro-Rodriguez, Lene Terslev, George Bruyn, Annamaria Iagnocco, Chiara Bertolazzi, Maria Antonietta D'Agostino and Andrea Delle Sedie Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality Scleroderma is a group of conditions affecting approximately 300,000 people in the United States. When scleroderma only affects the skin, it is considered localized. However, if it affects the skin and internal organs, it is viewed as systemic, also called Systemic Sclerosis (SSc). SSc affects approximately 100,000 people, or about one third of. Systemic sclerosis (SSc; scleroderma) is an autoimmune connective tissue disease characterized by autoantibodies, fibrosis, and microvascular injury and endothelial cell activation that result in vascular damage. Vascular injury induces both innate and acquired immune responses, resulting in fibroblast activation and organ fibrosis 1 Systemic sclerosis and its subtypes differ significantly from other diseases in rheumatology and clinical immunology, as the aberrant activation of the immune system does not result in an inflammation-driven destruction but in a progressive matrix synthesis, especially of the skin. Owing to the recently established networks in Germany (DNSS) as.

Limited cutaneous systemic sclerosis - Differentials | BMJCardiopulmonary exercise testing for detecting pulmonaryOverview of Symptoms of Scleroderma - YouTube

Systemic scleroderma, also called systemic sclerosis (SSc), is a multi-system autoimmune disease characterized by the accumulation of scar tissue in the skin and several internal organs such as the heart, kidney, lungs, and gastrointestinal tract.. Risk factors for systemic scleroderma. Most patients develop systemic scleroderma in their 30s or 40s.The exact cause of the disease is unknown. Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc. There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation Background/Purpose: There is a high unmet need for systemic sclerosis (SSc) treatments. Ziritaxestat (ziri; GLPG1690) is an autotaxin inhibitor with a novel mechanism of action. NOVESA (NCT03798366) is a phase 2a randomized, double-blind, placebo (PBO)‑controlled trial evaluating the efficacy, safety, and tolerability of ziri for diffuse cutaneous (dc) SSc. Methods: Patients with dcSSc were.

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