Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0262471 (ENT)
 5,307 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The hyper-IgE syndrome (HIS) is a complex immunologic disease, caused by an unknown basic defect. We report on two cases showing complications, which have not been described so far. Case 1: A 15-year-old boy suffering from HIS developed a liver tumour with severe eosinophilic infiltration and degranulation. The transformation process of the liver histologically resembled focal nodular hyperplasia. Therapy with cyclosporine A did not lead to clinical benefit. Now, a therapeutic attempt with interferon gamma is made. Case 2: In a 17-year-old female HIS patient, multiple papillomas and ulcers of the mucous membrane, caused by infection with human papilloma virus, emerged in the ENT region. Under treatment with interferon alpha, papillomatosis could be restrained. As therapy of the hyper-IgE syndrome only symptomatic treatment has been recommended so far. Future therapies should strive for systemic immunomodulation by application of cytokines or soluble cytokine receptors like interferons or sIL-4R.
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PMID:[Hyper-IgE syndrome--two case reports of unusual complications]. 792 82

In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL). Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.
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PMID:[Advances in the diagnosis of ENT tumors in childhood]. 1272 79

The role of certain viruses (human papilloma virus, Epstein-Barr virus, cytomegalovirus, and herpes virus) in the development and malignization of some pre-cancer conditions in human ENT organs is considered. The results of genetic (with the use of PCR) and histological studies of tissue biopsies and surgical specimens are presented. A total of 107 patients presenting with prolifertaive diseases of ENT organ mucosa were available for tissue sampling along with 30 other patients with histologically verified cancer of the larynx, pharynx, nasal cavity, and paranasal sinuses. The clinical data obtained are discussed.
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PMID:[About the role of chronic viral infection in etiology of proliferative diseases of the ENT organs]. 2198 60

A worldwide rise in the incidence of oropharyngeal squamous cell cancer has been recorded over the past decade. This rise has been attributed to an increase in oncogenic human papilloma virus (HPV) infections. The biology of HPV-positive oropharyngeal cancer is distinct; it includes p53 degradation, retinoblastoma pathway inactivation, and p16 upregulation. The best method for detecting HPV in tumour material is controversial; both in-situ hybridisation and PCR are commonly used. However, p16 can serve as a potential surrogate marker, and therefore, p16 immunohistochemistry is widely used in clinical practice. Compared to HPV-negative cancers, HPV-positive oropharyngeal cancer appears to be more responsive to chemotherapy and radiotherapy. Thus, p16 can serve as a prognostic marker for enhanced overall survival and disease-free survival. In this review, we aimed to highlight the current understanding of the epidemiology, biology, detection, and management of HPV-related oropharyngeal head and neck squamous cell carcinoma.
B-ENT 2015
PMID:HPV-positive head and neck tumours, a distinct clinical entity. 2656 6