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)

The case of a 3 month old child with severe combined sex linked immunodeficiency is presented. The diagnosis was well doccumented, during his life. The child presented as a case of mucocutaneous moniliasis resistant to treatment. There was a history of similar cases in the family; diagnosis was made at post-mortem in one cousin and death occurred at early age in all kins so affected. Blood marrow transplant was not feasible in our case because histocompatibility was lacking in the kins studied. Three units of transfer factor were given as well as hyperimmune plasma but the child died in respiratory failure. Autopsy demonstrated pulmonary infection by Pneumocystic carinii and generalized citomegalic inclussion virus infection; almost complete absence of immune tissue was also demonstrated.
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PMID:[Severe mixed immunodeficiency. Report of a case]. 17 1

The case of a 10.5-year-old girl, who was diagnosed with a case of thalassemia major at the age of 8 months and had been on regular blood transfusions since then, is related. Donor screening for HIV was started in mid-1988, thus she had received unscreened blood for a number of years. In February 1991, she presented with a dry persistent cough, moderate grade continuous fever, and breathlessness on exertion for over 2 weeks. Chest X-ray showed bilateral infiltrations. She was put on penicillin and chloramphenicol with a provisional diagnosis of bronchopneumonia. In March 1991, she had to be hospitalized for impending respiratory failure. After treatment with intravenous fluids and parenteral antimicrobials, her condition stabilized and she was discharged. In April 1991, she was readmitted because of complaints of difficulty in swallowing and weight loss. Her chest signs had persisted and she had developed oropharyngeal candidiasis with ulcerations. She also had alopecia, a generalized lymphadenopathy, digital clubbing, and bilateral parotid enlargement. Candidiasis responded to vigorous therapy with clotrimazole. Fine needle aspiration of lymph node showed a reactive hyperplasia. HIV antibodies were detected in the serum with ELISA and confirmed by Western blot. Immunologic tests showed evidence of severe immunodeficiency. The Multitest CMI, which simultaneously tests delayed skin hypersensitivity to seven common recall antigens, was totally nonreactive. She was classified as having AIDS according to World Health Organization criteria for children under 13 years of age. The diagnosis of lymphocytic interstitial pneumonitis (LIP) was also made based on the symptoms. Oral prednisolone was given 2 mg/kg/day in 3 divided doses for a month. The cough and dyspnea showed great improvement and the parotid swellings disappeared; lymphadenopathy, clubbing, and alopecia, however, persisted. The child was kept on maintenance therapy of prednisolone and on alternate day co-trimoxazole for prophylaxis against Pneumocystis carinii infection.
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PMID:Acquired immunodeficiency syndrome (AIDS) with lymphocytic interstitial pneumonitis (LIP) in a multi transfused child with thalassemia major. 129 97

Pneumocystis carinii pneumonia (PCP) occurs frequently in individuals infected with the HIV virus. Malignancy, immunosuppressive drugs, and congenital immune deficiency may be associated with PCP. We describe a patient with stage 1 testicular carcinoma who developed hypoxemic respiratory failure two days after retroperitoneal lymph node dissection. Pneumocystis carinii organisms were demonstrated by catheter lavage samples and confirmed on bronchoalveolar lavage. Testing for HIV antibody by ELISA and the Western blot test were negative; HIV viral culture and polymerase chain reaction were also negative. Pneumocystis carinii pneumonia is unusual in localized surgically cured malignancies without obvious immunodeficiency and, to our knowledge, has not been described as a cause of postoperative respiratory failure.
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PMID:Postoperative respiratory failure secondary to Pneumocystis carinii pneumonia. 131 51

A 67-year-old female was admitted to our hospital because of fever, dry cough, and exertional dyspnea. The findings of chest X-ray, transbronchial lung biopsy, and bronchoalveolar lavage were compatible with the diagnosis of idiopathic interstitial pneumonia. Prednisolone was administered and she felt better for a while. However, she developed severe dyspnea, and marked diffuse infiltrative shadows were observed on chest X-ray after 3 months of steroid therapy. In spite of pulse therapy with methylprednisolone, she died of severe respiratory failure. Complement fixation test and IgG antibody enzyme immunoassay for cytomegalovirus were positive, but there was no change the titers between admission and death. IgM antibody was negative. The lung findings at autopsy compatible with usual interstitial pneumonia and diffuse alveolar damage, moreover, cytomegalovirus infection was observed. We consider that recurrent cytomegalovirus pneumonia had been present due to secondary immunodeficiency caused by administration of steroid hormones.
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PMID:[A case of idiopathic interstitial pneumonia with cytomegalovirus infection]. 132 4

Patients at various stages of human immunodeficiency virus (HIV) infection require rehabilitation services. These patients present problems for each of the disciplines in a rehabilitation team, and all team members must confront the psychosocial and ethical issues involved with the disease. Patients with HIV infection may have polyneuropathy with multisystem involvement, including dysphagia, autonomic dysfunction, respiratory failure, bowel and bladder dysfunction, generalized weakness, a painful sensory neuropathy, and depression. Guidelines are presented for determining if inpatient rehabilitation or other settings are appropriate. Case management is a valuable strategy for the rehabilitation of patients with this complicated disorder.
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PMID:Human immunodeficiency virus infection and diffuse polyneuropathy. Implications for rehabilitation medicine. 186 48

A 31-year-old woman, heroin addict since ten years, and infected by the human immunodeficiency virus (HIV) since one year, was admitted to the intensive care unit for respiratory failure (PaO2 = 40 mmHg and PaCO2 = 14.8 mmHg, despite breathing pure oxygen). She had been followed up for 6 months for increasing dyspnoea due to chronic cor pulmonale for which no satisfactory explanation had been put forward. Artificial ventilation with 8 cmH2O positive end-expiratory pressure and 100% oxygen was completely inefficient. She died within a few hours. Postmortem lung biopsy revealed talc particles within interalveolar walls and alveolar macrophages as well as the expected alterations in blood vessels. Pulmonary hypertension due to talc microemboli is a well-known cause of respiratory failure in heroin addicts. Such a diagnosis should not be overlooked in a patient infected with HIV. Respiratory failure may not be only due to opportunist infections, or tumours related to the HIV infection.
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PMID:[Respiratory failure in a HIV seropositive heroin addict female]. 200 73

Cytomegalovirus (CMV) is frequently isolated from respiratory secretions of human immunodeficiency virus (HIV)-infected patients. Even in the presence of histopathologic evidence of CMV cytopathic abnormalities, the true clinical significance of CMV pneumonitis is not well established. Airways disease is increasingly recognized in HIV-infected patients, but its etiology is unclear. We describe an HIV-infected patient who presented with fever, wheeze, and micronodular interstitial infiltrates and developed severe hypercapnic and hypoxemic respiratory failure. Open lung biopsy showed necrotizing bronchiolitis with cytopathic changes characteristic of CMV infection; no other pathogens were isolated. He responded well to treatment with ganciclovir.
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PMID:Cytomegalovirus necrotizing bronchiolitis with HIV infection. 215 9

Pulmonary disease remains a major complication of the human immunodeficiency virus (HIV). Over the past decade several changes in the pattern of disease have occurred. Pneumocystis carinii pneumonia (PCP) remains the most common opportunistic pathogen in AIDS patients, though its incidence on bronchoscopy has declined and empiric therapy often occurs without a specific diagnosis. Changes in the management of patients with PCP have included different dosages and routes of administration for chemotherapy, improved overall survival, and a recent increase in the number of patients surviving episodes of respiratory failure. In addition, infection with mycobacteria tuberculosis (M.Tb.) has emerged as a major public health problem. The pattern of M.Tb. is distinct from non-immunocompromised patients though response to therapy usually occurs.
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PMID:Pulmonary disease in AIDS patients. 224 21

The clinical records and bronchoalveolar lavage (BAL) cell differential counts were analyzed in 96 patients at risk for Pneumocystis carinii pneumonia (PCP) from human immunodeficiency virus (HIV) infection to determine if this information may be prognostically useful and to identify possible mechanisms of BAL neutrophilia. In 60 patients with PCP, 15 fatalities or episodes of respiratory failure occurred, and 14 of these patients had greater than 5% BAL neutrophils. Only one of 33 patients with PCP and less than 5% BAL neutrophils died. In contrast, there was no correlation between survival and BAL neutrophil percentages in 33 patients who did not have PCP. Three patients with HIV infection without lung disease had normal BAL cell differentials. Intra-alveolar and interstitial leukocytes found in 17 transbronchial lung biopsies in patients with PCP indicate that the alveolar and interstitial compartments of the lung may be the source of BAL neutrophils. Pathologic evidence of increased severity of diffuse alveolar damage to explain BAL neutrophilia was not found. As BAL neutrophil percentages in PCP had both positive and negative predictive value, this information may be useful to stratify therapeutic trials or to identify the patient with PCP who is at high risk of a complicated or fatal outcome.
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PMID:Prognostic implications of bronchoalveolar lavage neutrophilia in patients with Pneumocystis carinii pneumonia and AIDS. 232 42

The value of differential cell counts in bronchoalveolar lavage fluid in patients who were serologically positive for the human immunodeficiency virus (HIV) was studied in 30 patients with classified into four groups according to the severity of illness: (1) seven subjects with the AIDS related complex without clinical or radiological evidence of pulmonary infection; (2) eight patients with the AIDS related complex and pulmonary tuberculosis; (3) eight patients with AIDS and Pneumocystis carinii pneumonia; and (4) seven patients with AIDS, Pneumocystis carinii pneumonia, and severe respiratory failure. All four groups had a similar percentage of lymphocytes, significantly higher than that of a control group of 15 healthy volunteers. A significant increase in the percentage of neutrophils was observed in groups 2, 3, and 4. The lavage fluid differential cell count does not therefore appear to help in the differential diagnosis of pulmonary infections in HIV positive patients. The abnormal percentage of lymphocytes observed in some patients with the AIDS related complex without clinical evidence of pulmonary infection suggests that lung injury may exist before clinical or radiological abnormalities develop. This might be related to an immunological mechanism or might be caused by an undetected subclinical infection.
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PMID:Bronchoalveolar lavage cell analysis in patients with human immunodeficiency virus related diseases. 278 19


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