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)

Susceptibility to recurrent staphylococcal cutaneous and respiratory infections beginning in infancy associated with extreme hyperimmunoglobulinemia E is a recently described primary immunodeficiency syndrome. Other clinical features include depressed cellular immunity and deficient antibody formation. Recurrent pneumonia and cyst formation with variable persistence and expansion characterized the radiographic couse in 11 patients. Five cysts resolved with continuous antistaphylococcal therapy; 2 were resected without recurrence; and 4 persisted after surgery and/or antibiotics (2--8 years). The cysts had dense, necrotic surfaces with fibrous walls, eosinophilic and other inflammatory cell infiltrates, and frequent, persistent, bronchial connections. Sinusitis (9/9) and mastoiditis (3/4) were also observed radiographically.
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PMID:Hyperimmunoglobulinemia E syndrome: radiographic observations. 45 Dec 23

A retrospective study was conducted at the Childrens Hospital Center at Jackson Memorial Hospital in Miami, FL, to evaluate the natural history of Mycobacterium tuberculosis infection in nine children with vertically acquired human immunodeficiency virus type 1 infection. The patients' ages ranged from 6 months to 7 years (median age, 42 months). Common presenting symptoms included prolonged fever, cough and anorexia. Only one patient had a positive tuberculin test. Five patients evidenced only pulmonary disease, three patients had pulmonary and extrapulmonary disease and one patient developed extrapulmonary tuberculosis (mastoiditis) and pulmonary interstitial disease that could not be attributed to mycobacterial infection because of lack of information. Organisms isolated before January, 1989, were susceptible to isoniazid and rifampin whereas isolates from three patients cultured after that time were resistant to multiple antituberculosis drugs. The median survival time after M. tuberculosis diagnosis for all children was 20 months. Our study suggests that children with human immunodeficiency virus type 1 infection who have tuberculosis have an increased risk for extrapulmonary disease. A high index of suspicion for the diagnosis of M. tuberculosis should be maintained in human immunodeficiency virus type 1-infected children with prolonged fever and respiratory symptoms. In areas of high endemicity of multidrug-resistant organisms, therapy with a broader panel of drugs may need to be instituted until susceptibility testing becomes available.
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PMID:Mycobacterium tuberculosis in children with human immunodeficiency virus type 1 infection. 145 38

Acquired immunodeficiency syndrome (AIDS) is a devastating disease that is affecting the human population in epidemic numbers. Patients with AIDS are known to have a significant incidence of otologic disease, including hearing loss, vertigo, tinnitus, otalgia, and infection with unusual pathogens. There has been no previous work on the histopathology of this disease. Ten temporal bones from five patients who were seropositive for the human immunodeficiency virus (HIV), the causative retrovirus of AIDS, were obtained. Seven specimens were analyzed using light microscopic techniques. Electron microscopy was performed on selected areas of pathology. A myriad of pathologic findings was seen, including severe petrositis with marrow replacement, mastoiditis, otitis media, ossicular destruction, precipitations in the perilymphatic and endolymphatic spaces of the vestibule and of the semicircular canals, and subepithelial elevation of the neurosensory epithelium of the saccule and utricle. The organ of Corti was relatively free of pathologic change. Many of the otologic symptoms encountered in these patients can be explained by the findings in this study. Further investigation using light and electron microscopy, and immunohistochemical techniques, is urged.
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PMID:Histopathologic and ultrastructural changes in the temporal bones of HIV-infected human adults. 160 48

A retrospective evaluation was performed on 28 cases of paediatric brain abscess (male: female ratio 2.5:1; mean age 9.4 years; range 2.8-16 years) diagnosed between 1967 and 1987. In 46%, congenital cyanotic heart disease was identified as a predisposing factor, likewise sinusitis, otitis media or mastoiditis in 29% and immunodeficiency in 11%. Pathogenesis remained unclear in 14%. Initial symptoms and signs were predominantly nonspecific; loss of consciousness occurred in 32% of cases, neurological deficit and seizures each in 25%. Since the availability of CT, both diagnostic delay after hospital admission and mortality were substantially reduced: mean delay from 8.4 to 3.0 days, and mortality from 23% to 0%. Seventeen patients (61%) had follow up examinations 9.6 years (mean) after the acute illness (range 1-21 years). Neurological sequelae were diagnosed in 35% of cases, epilepsy in 29%, epileptic potentials during EEG in 12%, and CNS scars in 50%. Psychological testing revealed no statistically significant differences compared to normal populations. CNS scars, and epilepsy and/or epileptic potentials were more common after excision (7 patients) when compared to patients treated by aspiration and/or antibiotics alone (21 patients). It is concluded that excision of brain abscess should be avoided whenever possible. Therapy of choice consists of the administration of adequate antibiotics with or without CT-guided needle aspirations.
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PMID:Brain abscess in childhood--long-term experiences. 202 23

We present the case history of a patient who was severely immunocompromised due to infection with the human immunodeficiency virus (HIV), and who subsequently developed acute mastoiditis due to Aspergillus fumigatus. Fungal otomastoiditis is a rarely reported complication of HIV infection. A high index of suspicion is required in these patients to facilitate early diagnosis and appropriate therapy.
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PMID:Aspergillus mastoiditis in a patient with acquired immunodeficiency syndrome. 923 Oct 92

In order to increase the awareness of otolaryngologists of severe combined immunodeficiency syndrome (SCIDS) so they may contribute to an earlier diagnosis of this disorder, we performed a retrospective chart review of a multicenter series from 2 children's hospital medical centers. Eighteen cases were identified, and 14 had an otolaryngological presentation. The average age of presentation was 3.3 months, and 72% were males. Most cases were inherited in an X-linked fashion. Five patients had thrush; 4 had recurrent otitis media. Other otolaryngological presentations included cough, mouth ulcers, pharyngitis, mastoiditis, and bilateral neck abscess. The most severe form of immunodeficiency, SCIDS is a rare condition that involves a disorder in both T and B cell functions. The manifestations involving the head and neck include recurrent upper respiratory tract infections, otitis media, thrush, oral ulcers, and abscesses. It is important that SCIDS be considered in any infant with recurrences of these common infections.
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PMID:Severe combined immunodeficiency: otolaryngological presentation and management. 1021 90

Penicillin-resistant Streptococcus pneumoniae (PRSP) is a frequently detected pathogen of intractable acute otitis media and is associated with prolonged or recurrent infection. The use of antibiotics has made the incidence of secondary acute mastoiditis following acute otitis media relatively rare, but when it does occur, its severe complications may be life-threatening. We report a case of pediatric recurrent acute mastoiditis caused by PRSP in a 6-year-old boy suffering from PRSP acute mastoiditis on 4 occasions, twice undergoing simple mastoidectomy. Although we initially suspected PRSP to be the chief factor in iterative infection, immunological analysis demonstrated significantly decreased IgG and IgA antibodies in serum and the patient was diagnosed as having common variable immunodeficiency (CVID). As the first middle ear infection occurred at the age of 6 and there was no history of upper respiratory tract infection, CVID may be the main pathological factor of recurrent mastoiditis, although infection occurred, only in the ear and did not involve other organs. This suggests that recurrent mastoiditis in the present case involved the coexistence of PRSP and CVID.
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PMID:[A case of pediatric recurrent acute mastoiditis caused by penicillin-resistant Streptococcus pneumonia complicated by primary immunodeficiency]. 1176 94

Pneumocystis carinii is an opportunistic infection found in patients with impaired immunity. Under favourable conditions the parasite can spread via the blood stream or lymphatic vessels and cause extrapulmonary dissemination. We report a case of P carinii infection presenting as bilateral aural polyps, otitis media and mastoiditis in human immunodeficiency (HIV)-positive patient with no history of prior or concomitant P carinii infection.
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PMID:Pneumocystis carinii infection in bilateral aural polyps in a human immunodeficiency virus-positive patient. 1194 91

Some 5% to 10% of all infants and toddlers suffer from four or more episodes of otitis per year. Usually, this is a temporary problem that resolves with increasing age. In a minority of cases, otitis episodes are frequent or have an abnormal course, with complications such as mastoiditis. In these cases, immunologic screening is indicated, to exclude an immunodeficiency. Agammaglobulinemia or hypogammaglobulinemia is rare among these patients. Other immune defects that occur more often are deficient or lowered immunoglobulin (Ig)A or decreased levels of one or more IgG subclass, in particular IgG2. The specific antibody response to bacterial capsular polysaccharides often is disturbed. These findings can give direction to the treatment of children with frequent, recurrent otitis.
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PMID:Immunologic screening of children with recurrent otitis media. 1596 72

Fungal mastoiditis caused by Aspergillus fumigatus predominantly occurs in immunocompromised patients. Invasive temporal bone mycoses are rare. They are usually associated with host immunodeficiency, are difficult to diagnose, and many cases are fatal. Treatment consists of antifungal chemotherapy, surgical debridement, and attempts to control the underlying immunological condition. Published reports describe patients with previous ear pathology and associated facial nerve dysfunction. We report a case in a patient with systemic lupus erythematosus. A good outcome followed surgical debridement and the use of a new triazole antifungal agent, voriconazole. Our patient's facial nerve function was unaffected. The presence of normal facial nerve function, however, does not exclude the possibility of invasive fungal mastoiditis.
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PMID:Aspergillus mastoiditis in a patient with systemic lupus erythematosus: a case report. 1614 71


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