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Moshe Lapidoth - One of the best experts on this subject based on the ideXlab platform.
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telangiectasis in crest syndrome and systemic sclerosis correlation of clinical and pathological features with response to pulsed dye laser treatment
Lasers in Medical Science, 2014Co-Authors: Shlomit Halachmi, Osama Gabari, Sarit Cohen, Romelia Koren, Dan Ben Amitai, Moshe LapidothAbstract:Telangiectasia are cardinal features of systemic sclerosis (SS) and calcinosis, Raynaud's syndrome, esophageal motility, sclerodactyly, Telangiectasias (CREST) syndrome. The etiology of Telangiectasia in these syndromes is unknown, but vascular dysfunction has been proposed. However, the Telangiectasia of CREST have anecdotally been considered relatively resistant to pulse dye laser (PDL), the treatment of choice for classic Telangiectasia. The study was designed to test whether SS/CREST Telangiectasia require more treatments than sporadic Telangiectasia and to identify clinical and histological features that could explain such an effect. Nineteen skin biopsies from patients with SS or CREST and 10 control biopsies were examined and compared for features that may predict a differential response to PDL. Sixteen cases of SS or CREST treated with PDL between 1997 and 2007 were evaluated and response to treatment was compared with 20 patients with sporadic telangiectasis. Relative to normal skin, CREST/scleroderma Telangiectasia exhibited thickened vessels in 17 out of 19 sections and thickened collagen fibers in the reticular or deep dermis in all sections. The number of treatments required to clear SS/CREST Telangiectasia was approximately twofold higher. SS/CREST Telangiectasia are more resistant to PDL but can be effectively cleared with more treatments.
Robert A Weiss - One of the best experts on this subject based on the ideXlab platform.
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rejuvenation of photoaged skin 5 years results with intense pulsed light of the face neck and chest
Dermatologic Surgery, 2002Co-Authors: Robert A Weiss, Margaret A Weiss, Karen L BeasleyAbstract:BACKGROUND. Photorejuvenation involves the use of lasers or light sources to reverse signs of photoaging. Multiple devices have been shown to be effective over the short-term. OBJECTIVE. To investigate the long-term clinical results on the face, neck and chest at 4 years using filtered flashlamp intense pulsed light (IPL) for treatment of photoaging changes of Telangiectasias, dyspigmentation, and rough skin texture. METHODS. A chart review of 80 randomly selected patients with skin types I-IV who were treated by IPL during 1996 and 1997 was performed. Photos and patient self-assessment were graded for features of textural smoothness, Telangiectasia severity, and blotchy pigmentation into four categories of worse, no change, slightly better (less than 50% improvement) and much better (greater than 50% improvement). RESULTS. At 4 years following initial treatment, skin textural improvement was noted in 83% of the subjects. Telangiectasias were improved in 82% of subjects, while pigmentation remained improved in 79%. The median number of treatments was 3. The face responded slightly better than the chest or neck. Most common side-effects included temporary mild crusting (19%), erythema (15%) and purpura (6%). CONCLUSION. Signs of photoaging including Telangiectasias and mottled pigmentation of the face, neck, and chest, can be improved by IPL with a long-lasting result. Minimal or no downtime with minimal adverse effects can be achieved with the settings reported. Skin textural smoothing, although not easily quantified, is an additional benefit observed long-term. THIS STUDY WAS PRESENTED IN PART AT THE 2000 ASDS ANNUAL MEETING IN DENVER. THE AUTHORS ARE CONSULTANTS AND PRECEPTORS FOR LUMENIS. THE DEVICES USED IN THIS STUDY WERE PURCHASED AT A DISCOUNT. NO DIRECT FUNDING WAS PROVIDED FOR THIS STUDY. THE APPEARANCE of aging skin due to chronic exposure to ultraviolet light can be distinguished from chronologically or intrinsically aging skin. l Photoaging, resulting from chronic exposure to UV light, includes wrinkling of the skin, rough texture, dyspigmentation, and loss of elasticity. 2,3 These findings are explained by histologic evidence of epidermal thinning but more importantly, dermal changes of disorganized collagen bundles and clumping of elastic fibers. Changes within the matrix of the dermis and at the dermal-epidermal junction include reductions in procollagens I and III, collagen VII, and the fibrillin-rich microfibrillar apparatus at the dermo-epidermal junction.4 Photoaged skin displays prominent alterations in the cellular component and the extracellular matrix of the connective tissue with an accumulation of disorganized elastin and a severe loss of interstitial collagens.5 Similar changes may also affect superficial capillaries which can become skin surface Telangiectasias. A variety of treatments to reverse this photodamage aging process have been employed. On one side of the
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treatment of poikiloderma of civatte on the neck with an intense pulsed light source
Plastic and Reconstructive Surgery, 2001Co-Authors: Mitchel P. Goldman, Robert A WeissAbstract:: Effective treatment of poikiloderma of Civatte is difficult. The ideal treatment combines elimination of both the vascular and pigmented components simultaneously. Treatment with a broad-spectrum noncoherent intense pulsed light source delivers multiple wavelengths with software-controlled pulse durations and sequencing that permit treatment of both vascular and pigmented lesions simultaneously. The objective of this study was to determine the response and side effects of treating this condition with intense pulsed light. In the study, 66 patients with typical changes of poikiloderma of Civatte on the neck were treated with intense pulsed light at various settings every 4 weeks until the desired improvement occurred. A 50 to 75 percent improvement in the extent of Telangiectasias and hyperpigmentation was observed after an average of 2.8 treatments. The incidence of hypopigmentation was 5 percent. It was concluded that intense pulsed light is an effective mode of therapy for poikiloderma of Civatte. It seems to offer a reduction in both pigmentation and Telangiectasia-associated erythema, with minimal side effects.
Carol A Burke - One of the best experts on this subject based on the ideXlab platform.
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The prevalence of hereditary hemorrhagic Telangiectasia in juvenile polyposis syndrome
Diseases of the Colon and Rectum, 2012Co-Authors: Margaret O'malley, James Church, Joseph Parambil, Brandie Heald, Matthew F. Kalady, L. Laguardia, Charis Eng, Carol A BurkeAbstract:BACKGROUND: Juvenile polyposis syndrome is a dominant GI polyposis syndrome defined by ≥ 5 GI juvenile polyps or ≥ 1 juvenile polyps with a family history of juvenile polyposis. Mutations in BMPR1A or SMAD4 are found in 50% of individuals. Hereditary hemorrhagic Telangiectasia is a dominant disorder characterized by epistaxis, visceral arteriovenous malformations, and Telangiectasias. Hereditary hemorrhagic Telangiectasia is diagnosed when ≥ 3 criteria including clinical manifestations or a family history, are present. A juvenile polyposis-hereditary hemorrhagic Telangiectasia overlap syndrome has previously been reported in 22% of patients with juvenile polyposis due to a SMAD4 mutation.\n\nOBJECTIVE: Our objective was to determine the prevalence and clinical manifestations of hereditary hemorrhagic Telangiectasia by Curacao criteria in our juvenile polyposis SMAD4 patients.\n\nDESIGN, PATIENTS, AND SETTING: This was a cohort study of juvenile polyposis patients in our inherited colon cancer registries. Hereditary hemorrhagic Telangiectasia manifestations were obtained from medical records, patient contact, and/or prospective hereditary hemorrhagic Telangiectasia screening. The Curacao criteria was used for diagnosis of hereditary hemorrhagic Telangiectasia (≥ 3 criteria diagnostic; 2 criteria suspect of).\n\nMAIN OUTCOME MEASURES: Prevalence and clinical manifestations of hereditary hemorrhagic Telangiectasia in juvenile polyposis SMAD4 patients.\n\nRESULTS: Forty-one juvenile polyposis families were identified. Genetic testing was available for individuals within 18 families. SMAD4 mutations were found in 21 relatives in 9 families. Eighty-one percent of SMAD4 patients had hereditary hemorrhagic Telangiectasia and 14% were suspected of having hereditary hemorrhagic Telangiectasia. Epistaxis and asthma are the most common symptoms in our overlap patients. Symptomatic and subclinical arteriovenous malformations were noted near universally.\n\nLIMITATIONS: There was a single, tertiary referral center.\n\nCONCLUSIONS: Nearly all juvenile polyposis SMAD4 patients have the overlap syndrome. The clinical implications and need for hereditary hemorrhagic Telangiectasia screening are important factors for genetic testing in juvenile polyposis. Health care providers must be cognizant of the juvenile polyposis-hereditary hemorrhagic Telangiectasia overlap syndrome and the implications for management of these patients.
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the prevalence of hereditary hemorrhagic Telangiectasia in juvenile polyposis syndrome patients with smad4 mutations
Hereditary Cancer in Clinical Practice, 2011Co-Authors: Margaret Omalley, Joseph Parambil, Matthew F. Kalady, L. Laguardia, Charis Eng, James M Church, Brandie Leach, Carol A BurkeAbstract:Background Juvenile Polyposis Syndrome (JPS) is defined by the presence of ≥ 5 colorectal juvenile polyps or any number of juvenile polyps in an individual with a family history of JPS. Genetic alterations including either point mutations or large rearrangements in BMPR1A or SMAD4 are found in 50% of affected individuals. Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease diagnosed upon the presence of epistaxis, visceral arteriovenous malformations (AVM) or mucutaneous Telangiectasias. HHT is diagnosed when there are ≥ 3 manifestations and is suspected when there are at least 2 manifestations. Most HHT cases are caused by a germline mutation in ALK1 or ENG ,m embers of the TGFb signaling pathway. Approximately 22% of patients with Juvenile Polyposis Syndrome (JPS) due to a SMAD4 mutation have been reported to also have HHT [1]. Most prior publications have few patients and no systematic approach to screening, so the true incidence of the combined JPS/HHT syndrome is not known. Our aim was to determine the prevalence of HHT in our patients with JPS with a SMAD4 mutation including those who underwent systematic screening for AVM’s. Methods JPS patients were identified from a comprehensive polyposis database using Cologene© software. Families carrying a germline SMAD4 mutation were studied by screening affected patients for cutaneous telangiectases and with cardiac bubble ECHO, CAT scan chest, or MRI of brain for other AVMs. Results
Jeanfrancois Cordier - One of the best experts on this subject based on the ideXlab platform.
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pulmonary vascular manifestations of hereditary hemorrhagic Telangiectasia rendu osler disease
Respiration, 2007Co-Authors: Vincent Cottin, Gaetan Lesca, Sophie Dupuisgirod, Jeanfrancois CordierAbstract:Hereditary hemorrhagic Telangiectasia (HHT) or Rendu-Osler-Weber disease is a genetic disorder with autosomal dominance and variable penetrance, characterized by epistaxis, Telangiectasia and visceral
Sandra Lee Sessoms - One of the best experts on this subject based on the ideXlab platform.
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gastrointestinal hemorrhage in patients with systemic sclerosis and crest syndrome
The American Journal of Gastroenterology, 1998Co-Authors: Andrea Duchini, Sandra Lee SessomsAbstract:Abstract Objectives: Systemic sclerosis (SSc) and calcinosis, Raynaud’s phenomenon, esophageal disease, sclerodactyly, Telangiectasia (CREST) syndrome present distinctive microvasculature lesions that are thought to be responsible for tissue damage and disease progression. Involvement of the gastrointestinal tract may lead to the occurrence of profuse hemorrhage. We performed a study to assess the incidence and characteristics of gastrointestinal hemorrhage in a large group of patients with SSc and CREST syndrome. Methods: We reviewed the medical records of 144 patients with SSc/CREST seen at our institution during the period 1985–1996. Endoscopic findings and clinical data were correlated. Data are expressed as means ± SD. Results: Twenty-two of 144 (15.2%) patients had at least one episode of gastrointestinal hemorrhage (16 women, 6 men; mean age, 59.4 ± 17.6 yr). Eight patients (8/22; 36%) had multiple episodes and four (4/22; 18%) required chronic transfusion therapy. Mucosal Telangiectasias were the most common cause of bleeding (9/22; 40.9%), followed by peptic ulcer disease (7/22; 31.8%) and erosive gastritis (3/22; 13.6%). Bleeding Telangiectasias occurred in the entire gastrointestinal tract, including oral cavity (n = 1), esophagus (n = 1), stomach (n = 3), duodenum (n = 1), ileum (n = 1), cecum (n = 2), and colon (n = 2). Mortality was 22.7% in patients with gastrointestinal bleeding, compared with 7.3% in patients without bleeding. Conclusions: Patients with SSc/CREST syndrome are at risk of developing severe gastrointestinal hemorrhage. This complication is associated with frequent hospitalization, blood transfusions, and increased mortality. Mucosal Telangiectasias are the most common source of bleeding. Appropriate endoscopic intervention is recommended in evaluating and preventing bleeding in patients with SSc/CREST.
