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Rhinology and Facial Plastics
Published in Adnan Darr, Karan Jolly, Jameel Muzaffar, ENT Vivas, 2023
Adnan Darr, Karan Jolly, Shahzada Ahmed, Claire Hopkins
Examination: Telangiectasia is usually through direct examination: Anterior rhinoscopyNasendoscopyOropharyngeal examinationSkin (fingers, face and ears)
Skin
Published in A. Sahib El-Radhi, Paediatric Symptom and Sign Sorter, 2019
Telangiectasia indicates permanently dilated superficial blood vessels in the skin or mucous membranes. One or two telangiectases on the face (occasionally on the dorsa of the hands) are common and trivial in children, and highly characteristic in the vascular centre, from which radiates fine vessels that give the appearance of a spider web. Unlike petechiae, which have a similar appearance, they blanch with pressure and refill immediately after the pressure is released. Other lesions may suggest serious systemic disease such as ataxia telangiectasia (progressive cerebellar ataxia, sinopulmonary infection and immunodeficiency) and hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu disease), which is autosomal dominant, characterised by arteriovenous malformation on the skin, mucous membranes and internal organs such as brain, liver and lungs.
Analysis of variance and some other types of regression
Published in Antony Stewart, Basic Statistics and Epidemiology, 2018
For example, a total of 303 alcohol-abusing men were studied, to ascertain whether diagnosis of liver cirrhosis could be made on the basis of clinical symptoms alone, without the need to perform a surgical liver biopsy (Hamberg et al., 1996). Six symptoms were studied: facial telangiectasia, vascular spiders, white nails, abdominal wall veins, fatness and peripheral oedema. In this case, the ‘dichotomous variables’ were the symptoms (because patients either have or do not have a particular symptom) and the dichotomous ‘outcome’ was liver cirrhosis. Logistic regression was used to predict the likelihood that a person having any combination of the symptoms actually had liver cirrhosis. A concise explanation of the logistic regression analysis used in this study was subsequently published in Bandolier (Freeman, 1997), and is available online at: www.medicine.ox.ac.uk/bandolier/band37/b37-5.html Results of the analysis were used to predict that people who experienced all six symptoms had a 97% chance of having cirrhosis, whereas there was a 20% chance in those who only had white nails and fatness.
Clinical efficacy analysis of 110 cases of childhood vitiligo with non-surgical combined therapy
Published in Journal of Dermatological Treatment, 2022
Yuan-Yuan Liu, Jun-Feng Zhou, Yu Zhen, Yan Cui, Yang Song, Lei Yao, Shan-Shan Li
Thirty-two patients experienced adverse effects during the therapy. Eleven patients developed marked erythema, and seven patients developed a blister at the application site. These problems led to a temporary interruption of phototherapy, but the patients were all safely restarted on NB-UVB therapy at a lower dose. Among the children treated with a TCI, six experienced pruritus with tacrolimus 0.03% ointment, which was gradually tolerated by reducing the dosage, and none had discomfort with pimecrolimus 1% cream. Five patients who were administered a TCS experienced telangiectasia. Three adolescents treated with an SC developed weight gain, which returned to normal following the withdrawal of the SC. None of the patients who were administered a TCS or SC experienced Cushing’s syndrome or adrenal suppression.
Coats like response in healed choroiditis
Published in Ocular Immunology and Inflammation, 2022
Saurabh Verma, Indrish Bhatia, Mousumi Banerjee, Vinod Kumar
Abnormal telangiectatic retinal vessels accompanied with subretinal exudation with or without retinal detachment in association with other ocular or systemic disorder are included in Coats’-like response.1 The etiology of retinal telangiectasia, either congenital or secondary remains unclear till now. Tarkkanen et al.7 studied the histopathological specimen of retina in Coats’ disease and inferred functional or structural loss of the blood-retinal barrier with disorganization of mural architecture. Reese8 postulated that endothelial cell dysfunction causes localized microvascular atresia and occlusion, which leads to vascular ectasia and formation of collateral vessels. Thus, the possible etiology could be any vaso-occlusive event, autoimmune vasculopathy or chronic inflammation causing structural damage to retinal vasculature which may precede the development of retinal telangiectasias.6 The clinical significance of this entity arises from the fact that such an exudative response further deteriorates the visual acuity due to its involvement of the posterior pole. In our case, the damage to the retinal vasculature secondary to inflammatory response might have lead to the development of Coats’-like response. Early diagnosis of this response can halt the ongoing neovascular drive and stabilize the blood retinal barrier by treating with intravitreal anti-VEGFs/steroids and/or laser photocoagulation. It also reduces the need for vitrectomy in the future.
Cutaneous Granulomatosis and Class Switching Defect as a Presenting Sign in Ataxia-Telangiectasia: First Case from the National Iranian Registry and Review of the Literature
Published in Immunological Investigations, 2020
Parisa Amirifar, Reza Yazdani, Tannaz Moeini Shad, Alireza Ghanadan, Hassan Abolhassani, Martin Lavin, Soheila Sotoudeh, Asghar Aghamohammadi
Gait abnormality is usually the first diagnostic criterion of A-T, appearing in the first years of life. Telangiectasia is the second important clinical manifestation of the disease, having its onset commonly after the age of 3 years (Jozwiak 2005). Measurement of serum AFP concentration is another diagnostic hallmark, as it is increased in more than 90 to 95% of patients with A-T (Stray-Pedersen et al. 2007). In our patient, despite a large deletion in the ATM gene, predicted to result in ATM protein loss, the first clinical symptoms were skin lesions and autoimmunity, while ataxia and telangiectasia appeared after 6 years of age. This atypical manifestation caused at hree-year delay between the appearance of initial symptoms and the final diagnosis of A-T. Early diagnosis improves both the quality of life and survival in patients with A-T by antibiotic therapy and vaccination, IVIg substitution, limitation of exposure to ionizing radiation, and physiotherapy for respiratory symptoms (Navratil et al. 2015). Moreover, early diagnosis helps genetic counseling for the identification of the carriers who have an elevated risk of malignancy, especially breast cancer in women and may also reduce the risk of another child with A-T in the family (Swift et al. 1991).