Search results for "Dysplasia"

Combined treatment of epidermodysplasia verruciformis with etretinate and α-interferon*

1992

Epidermodysplasia verruciformis (EV) is an uncommon cutaneous disease in which a focal and genetically determined immunological impairment is associated with chronic human papilloma virus (HPV) infection. In sun-exposed areas, when an oncogenic HPV type is the agent, skin cancer may occur. The treatment of EV is difficult and often unsatisfactory; etretinate has been reported in some instances as effective in improving lesions. We report a typical case of EV with pityriasis versicolor-like lesions on the trunk and many flat, erythematous wart-like lesions on the face, dorsal areas of the hands and legs. We performed a treatment with etretinate (1 mg/kg/day for 6 weeks) and subsequently with…

The molecular changes driving the carcinogenesis in Barrett's esophagus: which came first, the chicken or the egg?

2013

Esophageal adenocarcinoma originates from columnar metaplastic epithelium of the distal esophagus. Various steps for this carcinogenetic process are known. Before the onset of high-grade dysplasia and adenocarcinoma, endoscopic surveillance is possible. However, because of the high cost of long-term surveillance, predictive factors for cancer are being evaluated to identify subjects with metaplasia who have a higher risk of developing malignancy. Molecular changes seem suitable for this purpose, but could require a high resource expenditure. While trying to identify the best predictive factors for cancer risk, molecular changes and differences in miRNA expression profile between the various…

Prosthetic rehabilitation of a young patient affected by Ectodermal Dysplasia with the new Eclipse Resin System

2012

Ectodermal dysplasia is a genetic disease caused by incorrect development of the epidermis and cutaneous adnexa (for example hair, nails and sweat glands). It is often associated with skeletal and dental development anomalies. The main clinical manifestations of these syndromes are hypotrichosis, hypohydrosis and hypodontia or anodontia. Polymethyl methacrylate (PMMA) is still the most frequently used material in denture bases, but it can lead to irritation, inflammation and allergic reactions in the oral mucosa, due to the release of residual monomers. This clinical report describes a case of Hypohydrotic Ectodermal Dysplasia, rehabilitated with removable dentures made with a new monomer-f…

A novel EDA variant causing X-linked hypohidrotic ectodermal dysplasia: Case report

2021

Abstract Hereditary ectodermal dysplasias are a complex group of inherited disorders characterised by abnormalities in two or more ectodermal derivatives (skin, nails, sweat glands, etc.). There are two main types of these disorders – hidrotic and hypohidrotic/anhidrotic ectodermal dysplasias. Hypohidrotic ectodermal dysplasia (HED) or Christ-Siemens-Touraine syndrome (OMIM: 305100 ) occurs in 1 out of 5000–10,000 births [19] and has an X-linked recessive inheritance pattern (X-linked hypohydrotic ectodermal dysplasia – XLHED) [2] . The main cause of XLHED is a broad range of pathogenic variants in the EDA gene (HGNC:3157, Xq12-13) which encodes the transmembrane protein ectodysplasin-A [4]…

Hypohidrotic Ectodermal Dysplasia with total anodontia: A case report

2011

Ectodermal dysplasia is a hereditary disorder that occurs as a consequence of disturbances in the ectoderm of the developing embryo. The triad of nail dystrophy, alopecia or hypotrichosis and palmoplantar hyperkeratosis is usually accompanied by a lack of sweat glands and a partial or complete absence of primary and/ or permanent dentition. A case report illustrating the prosthetic rehabilitation of a seven year old boy with hypohidrotic ectodermal dysplasia associated with total anodontia is presented.

Dental implants in patients with oral mucosal alterations : an update

2010

Objective: To determine whether a series of diseases of the oral mucosa - Sjogren syndrome, ectodermal dysplasia, epidermolysis bullosa and lichen planus - reduce the survival rate of dental implants. Material and Method: A Medline search was carried out using the key words: "Sjogren syndrome", "ectodermal dysplasia", "epidermolysis bullosa", "lichen planus" and "dental implants", including those publications involving clinical series comprising more than one patient with the mentioned disorders and treated with dental implants, in the last 10 years. Results: The study included three articles involving patients with Sjogren syndrome subjected to dental implant treatment, representing a tota…

Ventricular arrhythmias in children: the uselessness of MRI.

2009

Ileo-ileal invagination--a cause of recurrent mid-gastrointestinal bleeding: diagnostic and endoscopic therapy by means of push-and-pull enteroscopy.

2008

The types of lesions that cause bleeding in the small bowel are similar to those found in other areas in the gastrointestinal tract, such as vascular malformations, ulcers and inflammatory lesions, neoplasms and other less common lesions like Meckel's diverticulum. This report describes three patients with suspected mid-gastrointestinal bleeding with no significant past medical history. Before presenting to our unit the diagnostic work-up such as oesophagogastroduodenoscopy, colonoscopy and radiological small bowel imaging such as conventional enteroclysis or magnet resonance imaging enteroclysis had been performed without detecting any bleeding source. Capsule endoscopy suspected an angiod…

ag) Push-and-pull enteroscopy in the small bowel using the double-balloon technique: results of a prospective European multicenter study.

2005

BACKGROUND AND STUDY AIMS The aim of this study was to evaluate the feasibility, safety, and clinical impact of push-and-pull enteroscopy (PPE) in patients with suspected or documented small-bowel diseases, in a prospective multicenter trial in three European medical centers. PATIENTS AND METHODS A total of 100 patients (mean age 56 +/- 16 years; range 13 - 90) were included at the three institutions between July and November 2004. The leading symptoms were: acute recurrent or chronic gastrointestinal bleeding (n = 64), polyposis syndrome (n = 8), chronic abdominal pain (n = 7), chronic diarrhea (n = 7), and others (n = 14). RESULTS No major PPE-associated complications such as perforation,…

Follow-up of patients after revascularisation for peripheral arterial diseases: a consensus document from the European Society of Cardiology Working …

2019

International audience; Peripheral arterial diseases comprise different clinical presentations, from cerebrovascular disease down to lower extremity artery disease, from subclinical to disabling symptoms and events. According to clinical presentation, the patient's general condition, anatomical location and extension of lesions, revascularisation may be needed in addition to best medical treatment. The 2017 European Society of Cardiology guidelines in collaboration with the European Society for Vascular Surgery have addressed the indications for revascularisation. While most cases are amenable to either endovascular or surgical revascularisation, maintaining long-term patency is often chall…