Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 10-year-old girl with eczema, asthma, recurrent abscesses, middle ear infections and pneumonias developed a histiocytic lymphoma of the brain. Immunologic evaluation revealed hyperimmunoglobulinemia E, depressed neutrophil chemotaxis, absent delayed hypersensitivity skin test tests, low T-lymphocyte numbers and depressed in vitro lymphocyte responses. The occurrence in this patient of a histiocytic lymphoma, which is commonly associated with more classic immunodeficiency syndromes or immunosuppression for transplantation, suggests that individuals with hyperimmunoglobulinemia E and recurrent infections may also be at increased risk for developing unusual malignancies.
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PMID:Fatal histiocytic lymphoma of the brain associated with hyperimmunoglobulinemia-E and recurrent infections. 32 74

Among patients with recurrent, protracted or chronic infections of the respiratory tract involving the middle ear, 18 were found to have immunodeficiencies. In 10 of the patients, deficiency of immunoglobulins belonging to the IgG, IgA and IgM classes was found. Seven patients had an isolated IgA deficiency. One patient had a combined immunodeficiency with defects of the T-cell system and the B-cell system. One patient had an isolated T-cell deficiency.
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PMID:Immunodeficiency syndromes with otorhinolaryngological manifestations. 108 76

ORL inflammations present particular pathogenetic and clinical characteristics due to the close anatomic and physiological connection among them and to their particular anatomic characteristics. The ear communicates with the rhinopharynx through the Eustachian tube, and this relationship explains how bacterial infections of the upper respiratory tract can cause otitis and how tubal dysfunctions are often responsible for chronic ORL infections, also favoured by the microcavity structure of the middle ear and the mastoid bone. Also the macro- and microcavity anatomic structure of the paranasal sinuses favours chronic infectious diseases, and their adjacency with the endocranial and facial structures justifies the secondary complications of these infections. The palatine tonsil and other structures of the Waldeyer's lymphatic ring play an important immune role in the first respiratory and digestive tract, and infections occurring in these structures (for example, streptococcal infection) can cause pathologies such as rheumatic disease. Other ORL infections are connected to immunodeficiency or atopy.
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PMID:[The clinical problems of bacterial infections of the upper respiratory tract]. 129 2

A case of primary Pneumocystis carinii infection involving the left middle ear of a patient with acquired immunodeficiency syndrome is described, and the literature on the otic and ophthalmic pneumocystosis is reviewed. Otic pneumocystosis typically presents as a unilateral polypoid mass, and it is clinically manifested as otalgia, hearing loss, or, sometimes, otorrhea without evidence of current respiratory disease or previous Pneumocystis pneumonia. In contrast, choroidal pneumocystosis usually occurs in a patient with acquired immunodeficiency syndrome with at least one previous episode of Pneumocystis pneumonia and aerosolized pentamidine treatment, it is usually asymptomatic and bilateral, and it may be discovered only because of other concurrent human immunodeficiency virus-related ophthalmic disease. The diagnosis is made clinically, and intravenous antiparasite treatment is successful.
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PMID:Otic and ophthalmic pneumocystosis in acquired immunodeficiency syndrome. Report of a case and review of the literature. 158 Jul 53

We report a case of Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. This is the third reported case of malignancy in the hyper-IgE syndrome. The other two cases were an 18-year-old man with Hodgkin's disease and a 10-year-old girl with histiocytic lymphoma. The patient developed retroperitoneal Burkitt's lymphoma with probable metastasis to the brain. His short life was characterized by recurrent staphylococcal skin, middle ear, and lung infections associated with extremely elevated serum concentrations of IgE. There was also an associated disturbance of bone metabolism with osteoporosis and pathologic fractures and absence of parathormone, findings that have been observed in other patients with hyper-IgE syndrome and other forms of T cell immunodeficiency. At the age of 5 years, inadequate B cell responses to immunization with antigens derived from diphtheria, tetanus, and Haemophilus influenzae type b organisms and with the OX174 bacteriophage were demonstrated in the patient. In his terminal state his in vitro lymphocyte analysis demonstrated findings of anergy. Although the precise immunologic defect in hyper-IgE syndrome is unknown, these cases of associated malignancy stress the role that a completely normal immune system plays in preventing the premature appearance of cancer.
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PMID:Burkitt's lymphoma developing in a 7-year-old boy with hyper-IgE syndrome. 278 97

It is generally agreed that middle ear reconstructive surgery performed with tympano-ossicular homografts produces superior functional results compared with prosthetic material, especially with respect to extrusion rate. The use of homografts, though, has been seriously hampered recently by the fear of transmission of human immunodeficiency virus (HIV) infection. In HIV-infected patients, the virus is primarily found in the cells of the lymphoid and monocytic lineage. The nature of the tissues in the eardrum and ossicles, mostly fibrous tissue and compact bone without marrow, suggests that little virus load should be found in homografts. Indeed, culturing minced homograft tissue from two HIV-infected donors with acquired immune deficiency syndrome (AIDS) in a sensitive culture system with PHA-stimulated lymphoblasts produced no virus. Before use, homografts undergo a fixation procedure in 5% formaldehyde and then are kept in a solution containing Cialit as a preservative. The authors therefore examined the capacity of formaldehyde and Cialit to reduce the infectivity of HIV in models of infected tissue as measured in vitro. The reduction of in vitro infectivity due to these treatments was at least 10(5)-fold and 10(2)-fold, respectively. Coupled with the low virus burden in tympano-ossicular tissue, our data suggests that the fixation procedure affords such a reduction in infectivity that the risk of HIV transmission, even from an HIV-infected donor, is vanishingly low.
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PMID:Risk of transmission of human immunodeficiency virus infection during tympano-ossicular homograft: an experimental study. 861 99

This report describes an infant with clinical features consistent with the yellow nail syndrome (YNS), a rare autosomal dominant disorder. He presented at birth with congenital lymphoedema and was referred at 6 months of age for investigation of recurrent cough and wheeze. He had clinical and radiological evidence of bilateral pleural effusions and a pericardial effusion. Following a lung biopsy and pericardial window these were shown to be manifestations of his lymphatic abnormality. He also had persisting middle ear effusions causing conductive deafness requiring hearing aids and secondary immunodeficiency requiring regular immunoglobulin infusions.
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PMID:Yellow nail syndrome in infancy. 940 96

Recent studies using the polymerase chain reaction (PCR) have identified bacterial and viral genomic sequences in culture-negative pediatric middle ear effusions. To evaluate this technique in adults, 19 effusions were analyzed to compare bacterial and viral culture and PCR detection of Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, and adenovirus. Effusions from 4 subjects positive for human immunodeficiency virus (HIV) were analyzed by PCR for HIV virus. Three of 19 effusions were culture-positive for bacteria, and 0 of 19 for viruses. Fifteen of 19 effusions were PCR-positive for bacterial genomic sequences, and 0 of 19 for adenovirus. Thirteen of 15 PCR-positive specimens demonstrated S pneumoniae, 5 of 15 H influenzae, and 0 of 13 M catarrhalis. All 4 effusions from HIV-positive subjects were PCR-positive for HIV. No effusion was culture-positive and PCR-negative. These results confirm that culture-negative middle ear effusions contain genomic sequences from bacterial pathogens. Finding of HIV RNA and DNA in effusion from HIV-positives suggests replicating virus in this fluid.
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PMID:Analysis of adult otitis media: polymerase chain reaction versus culture for bacteria and viruses. 943 82

Human immunodeficiency virus (HIV) infection continues to be a vexing problem in the pediatric population. Otitis media, a common entity in immunocompetent as well as immunocompromised children, is prevalent in pediatric patients with HIV infection. Recurrent infections and complications secondary to otitis media are also common in this population. The purpose of this review was to evaluate the records of a large group of children with HIV infection undergoing treatment for otitis media at a tertiary care center. Incidence of infections, severity of infections, and pathogens responsible were the key points of data collection in relation to each patient's HIV clinical and immunologic classification. We reviewed 72 patients with vertically-acquired HIV infection undergoing treatment at the Hospital for Sick Children, Great Ormond Street, London. All patients were born to HIV infected mothers of sub-Saharan African origin. A total of 32 (44.4%) of these children presented to our institution with one or more episodes of otitis media, six of whom suffered systemic complications secondary to middle ear pathology. Middle ear culture data, when available from actively draining ears, was similar to those of immunocompetent children in the general population. The most common complications were systemic bacteremia and recalcitrant infections requiring intravenous antibiotic therapy. Severity of immunosuppression was associated with higher incidence and severity of otitis media. Otitis media in an HIV infected child must be treated aggressively at its outset in an attempt to minimize possible complications. Administration of prophylactic antibiotics to these children may reduce the frequency of acute and chronic infections. Improvement in immunologic status using antiretrovirals or protease inhibitors is a primary goal in diminishing the severity of otitis media as well as other infections in these children.
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PMID:Otitis media in children with vertically-acquired HIV infection: the Great Ormond Street Hospital experience. 980 22

To rehabilitate most cases of conductive hearing loss closure of ear drum perforations and rebuilding of the ossicular chain can be performed. Due to the great number of biocompatible bone substitute materials available it is occasionally difficult for the surgeon to choose the most favorable substitute. Autogenous structures (ossicles, cortical bone, cartilage) and allogenous tissues (ossicles, cortical bone, cartilage, dentin) are possible bone replacement materials. Xenogenic tissue is currently not used in middle ear surgery. Ionomer cement is a hybrid material for replacement of bone but does not fit direct classification of the various classes of alloplastic materials in current use: that is, metals (gold, steel wire, platinum, titanium), plastics (polyethylene, polytetrafluorethylene) and ceramics (ceramic oxide, carbon, calcium-phosphate ceramic, vitreous ceramic). For restoration of the sound conductive apparatus preference is given to autogenous ossicles because cortical bone is resorbed and cartilage weakens over time. Most surgeons do not use allogenous tissue, because of the possible transmission of such infectious disease as immunodeficiency syndrome or Creutzfeldt-Jakob disease. Only dentin deserves special attention as a possible bone substitute in the middle ear because its form can be preserved during sterilization. Based on the observations available to date, it becomes apparent that titanium implants hold greater promise than gold. Form-stable synthetic materials are not generally recommended due to foreign body reactions which have been confirmed by many investigators. Ceramic materials (e.g. ceramic oxide, carbon, calcium-phosphate ceramic, glass ceramic) are well tolerated in the middle ear and have also proved to be useful over time. Hybrid bone substitute ionomer cement is easily workable and well integrated, showing a good functional outcome. For many years good results in otosclerosis surgery have been achieved with a prosthesis made of platinum-wire and Teflon. Short-term follow-up periods hold great promise with pistons made of gold. Autogenous ossicles, ionomer cement and recently titanium protheses--as far as usable--are employed by the author for reconstructing the middle ear. For the time being platinum-Teflon prostheses and gold are used in otosclerosis surgery.
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PMID:[Materials for reconstruction of the middle ear]. 1019 73


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