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)

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
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PMID:Cutaneous histiocytosis syndromes. 299 40

Primary central nervous system lymphoma is a rare presentation of lymphoma and is usually seen in adults, often in association with immunodeficiency. Evaluation of these patients classically shows discrete intracranial lesions, and long-term prognosis despite treatment is poor. The case of a child is presented who had no identifiable predisposing factors, no radiologic evidence of intracranial mass, no evidence of systemic disease, and in whom the diagnosis of primary CNS lymphoma was made based on documentation of a monoclonal population of malignant B-cells in the cerebrospinal fluid. The patient, in whom irreversible blindness developed, was treated with a combination of cranial radiation, and intrathecal and systemic chemotherapy, and is currently alive and disease-free 15 months after diagnosis.
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PMID:Primary central nervous system lymphoma without intracranial mass in a child. Diagnosis by documentation of monoclonality. 387 78

Human cytomegalovirus (HCMV) causes retinitis and is the leading cause of blindness in patients infected with the human immunodeficiency virus (HIV). While most patients with HIV are HCMV seropositive, not all will develop clinical complications from it. The immune responses that can prevent the development of HCMV retinitis are unknown. The levels of anti-HCMV antibodies, including responses to the two major envelope proteins, gpUL55 (gB) and gpUL75 (gH), which are the targets of neutralizing antibody (NA), were examined in HIV-infected patients with and without retinitis. No specific deficiency in the antibody response of retinitis patients was observed. However, higher levels of NA were associated with a more favorable clinical course. These results indicate that antibodies may modulate progression of disease, and they suggest a possible role for the exogenous administration of NA in patients who develop HCMV retinitis.
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PMID:Virus-specific antibody responses to human cytomegalovirus (HCMV) in human immunodeficiency virus type 1-infected persons with HCMV retinitis. 779 60

Cytomegalovirus (CMV) retinitis is the most common retinal opportunistic infection in AIDS patients and is the main cause of blindness. It is generally associated with a CD4+ lymphocyte count below 50/microL. CMV retinitis is often asymptomatic (54% of the cases), frequent ophtalmoscopic screening is very important. Two virostatic drugs (Cymevan and Foscavir) have been approved for the treatment of CMV retinitis. Both are effective in preventing the progression of the lesion within 3 weeks of induction therapy. Long-term use of virostatic maintenance therapy delays the onset of relapses. The differential diagnosis of CMV retinitis are: human immunodeficiency virus retinopathy, varicella-zoster virus retinitis, ocular toxoplasmosis, syphilis, candida endophthalmitis in intravenous drug users, and unfrequently, tuberculosis, choroidal pneumocystosis, intraocular lymphoma.
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PMID:[Retinal manifestations of AIDS]. 894 82

We experienced Hepatitis A, B, C and fulminant hepatitis due to Herpes simplex virus type 1 (HSV-1) in our hospital for the severely multi-disabled (SMD) who had both severe motor and intellectual disabilities, and some of whom might be further complicated by blindness and/or deafness. In this hospital, 100 SMDs are hospitalized. Case 1: The disabled, 25 year old male, was transmitted Hepatitis A from a nurse. Case 2: The disabled, 60 year old female carrier of Hepatitis B virus (HBV) who has been cared for more than 10 years. Case 3: The disabled, 46 year old male carrier of Hepatitis C virus (HCV) (RNA type 3), has been cared for more than 4 years. Case 4: The disabled, 39 year old male, had a fever of 39 degrees C for 9 days and suddenly died. He was diagnosed as fulminant hepatitis due to HSV-1 by necropsy. The hospitals for SMD are characteristic in prevention of nosocomial infections; 1) The disabled infected is not aware of the fact that he or she is the source of infection and that the other disabled living with him or her are in risk of infection, because of their severe mental condition. 2) All of the disabled need complete or incomplete helps for activities of daily life (ADL), so that the disabled who is the carrier of some pathogen constantly gives risk of infection to staffs, including medical staffs (doctor, nurse and therapist), psychologist and helpers by bloody secretion from wounds, saliva, urine, feces as well as menstrual blood. 3) If a carrier of some pathogen is hospitalized, the staffs should serve under risk of infection involving blood-mediated infectious disease for many years, because SMDs are permitted lifelong stay in the hospitals for SMD, which also play a role of care house or institution, by public expense in Japan. In case of an outbreak of Hepatitis A, nosocomial infection ended in the original case (a nurse), another nurse and a case of the disabled by general treatment and care against communicable diseases of the digestive organs. In care of HBV and HCV carriers, an ordinary program to prevent nosocomial infection has been practiced in our hospital more strictly than in conventional hospital. HBV vaccine is injected to staffs caring the HB carriers who are negative on HBs antibody. Thus, during more than 10 years of care of HBV carrier and more than 4 years of care of HCV carrier, nosocomial infection has never been experienced clinically as well as serologically in our hospital. However, we have often been faced by difficulty to guarantee QOL (quality of life) of the carriers, because carrier states of HBV or HCV have been long-lasting and they have been occasionally and inevitably separated physically and/or psychologically in order to prevent nosocomial infection. In case 4, it was suspected that previously latently infected HSV-1 would be activated by another viral infection which had elicited fever for 9 days before death. The patient had neither history nor sign or symptom of immunodeficiency and had never been given drugs known as to be immunosuppressive as side effect.
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PMID:[Characteristic situation on prevention of nosocomial infection in the hospital for the severely multi-disabled--experiences in care and treatment of 4 kinds of viral hepatitis]. 948 86

The paper retrospectively reviews the spectrum of ophthalmic manifestations in human immunodeficiency virus (HIV) infection in Singapore between May 1995 and October 1996. One hundred and eighteen patients were examined for ocular abnormalities. Criteria for examination were 1) visual complaints, 2) absolute CD4 count of less than 50 cells/ul, 3) patients with acquired immunodeficiency syndrome-defining illness or 4) any relevant systemic illnesses which may have ocular involvement. Only 25 patients (21.2%) had visual symptoms. Eighteen patients (15.3%) had abnormalities associated with microvasculature. Forty-four patients (37.3%) had opportunistic infection involving the eye of which 37 were that of cytomegalovirus retinitis (CMVR). Seven patients (5.9%) had neuro-ophthalmic disorders. One patient presented with proptosis due to orbital lymphoma. Four patients (3.4%) had episcleritis and 3 patients (2.5%) had symptomatic dry eyes. It is still not known if episcleritis and dry eyes are associated with HIV infection or are coincidental. Fifty-one patients (43.2%) had no ocular pathology and remained so throughout the period of study. Nine patients (7.6%) had more than one pathology. The major cause of visual loss was due to ocular infections, with CMVR being most prevalent. Recognising the ophthalmic signs in HIV patients will facilitate early diagnosis. Prompt treatment of eye involvement can prevent or delay blindness, which is psychologically and functionally important to these patients.
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PMID:Ophthalmic manifestations in human immunodeficiency virus infection in Singapore. 949 60

Recent studies of subjects infected with human immunodeficiency virus (HIV-1) have produced conflicting results about the extent of reconstitution possible in the CD4+ lymphocyte repertoire after highly active antiretroviral therapy (HAART). The effect of HAART on the incidence of opportunistic infections will probably depend on reconstitution of antigen-specific CD4+ lymphocyte responses to important pathogens, including cytomegalovirus (CMV), the leading cause of blindness in AIDS. Several studies have demonstrated an important role for CD4+ lymphocytes in controlling CMV replication in vitro and in clinical studies. It is now possible to quantitate antigen-specific CD4+ lymphocyte responses by flow cytometry. Using this method, we studied CMV-specific CD4+ lymphocyte responses in individuals infected with HIV-1 with and without a history of active CMV-associated end organ disease (EOD), and in those with quiescent CMV EOD after ganciclovir therapy and HAART. The presence of active CMV-associated EOD strongly correlated with loss of CMV-specific lymphocyte responses (P = 0.0004). In contrast, patients with no history of CMV-associated EOD and most patients with quiescent EOD after HAART demonstrated strong CMV-specific CD4+ lymphocyte responses. These data indicate that the loss of CMV-specific CD4+ lymphocyte responses in individuals infected with HIV-1 who have active CMV EOD may be restored after ganciclovir therapy and HAART, which provides evidence for functional immune reconstitution to an important pathogen.
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PMID:Restoration of cytomegalovirus-specific CD4+ T-lymphocyte responses after ganciclovir and highly active antiretroviral therapy in individuals infected with HIV-1. 970 Dec 50

The use of blind proficiency testing (PT) to examine analytic performance of human immunodeficiency virus type 1 (HIV-1) antibody testing. A total of 32 hospital, blood bank, public health, and commercial laboratories were included in this study. Test sera were introduced as clinical specimens for HIV-1 testing from private practitioners, group practices, clinics, and hospitals in Southern California. A total of 26 laboratories were located throughout California, with six laboratories located in six other states. Results from 306 enzyme immunoassay screening tests and 192 supplemental tests for HIV-1 were reported. Although one positive specimen was reported as indeterminate in almost 30% of results, screening and supplemental testing performances were excellent, with accuracy levels comparable to performance reported on open PT and performance evaluation surveys in the United States. The indeterminate results were attributed to the interpretive criteria used rather than to laboratory errors. Blind PT can be an important tool in improving the quality of total laboratory testing, the usefulness of laboratory results in patient care, and ultimately the health of the public.
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PMID:Analytic results of HIV-1 testing using blind proficiency testing. 1014 24

Blind proficiency testing was used to examine nonanalytic performance indicators for human immunodeficiency virus type 1 (HIV-1) antibody testing. Physician offices, clinics, and hospitals located throughout Southern California submitted simulated patient specimens to laboratories as routine test requests. A total of 32 laboratories were involved during five blind proficiency testing surveys. Turnaround time for a reactive specimen ranged from three to 17 days. Laboratory charges for evaluating a reactive specimen varied depending on the volume of testing, prevalence of reactive specimens, and whether screening and confirmatory tests were billed separately or as a package price. Charges for an enzyme immunoassay screening test plus supplemental tests ranged from $11.75 to $114.50, with a median of $31.00 for 24 laboratories that participated in one of the five surveys. Evaluation of laboratory report content revealed that 37% of the 16 screening reports and 71% of the 14 supplemental reports contained information that was unrelated to the patient results. Evaluation of the testing system documents the need to monitor multiple outcomes of the total laboratory testing process, not just the analytic testing phase.
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PMID:Identification of nonanalytic issues in HIV-1 antibody testing using blind proficiency testing. 1014 25

Children with hypertension, seizures, lethargy, encephalopathy, headache, and occipital blindness are reviewed. After undergoing antihypertensive therapy, most children improve. Some patients have a similar syndrome associated with chemotherapy, transplantation, transfusion, or human immunodeficiency virus-1 (HIV-1) infection. These latter children can develop symptoms with only minimal or no discernible elevations in blood pressure and improve, in the case of cancer-associated encephalopathy, after discontinuing chemotherapy. The reported children with this distinctive clinical condition are compared to adults with reversible posterior leukoencephalopathy syndrome. Since both gray and white matter are involved, we had suggested previously that the name be changed to (reversible) occipitoparietal encephalopathy syndrome. However, reversible posterior leukoencephalopathy has been used in the adult population and probably should be employed in children for the sake of uniformity, since both children and adults have the same clinical presentation and presumably a similar pathophysiology for the encephalopathy syndrome. The diagnosis is confirmed by reversible posterior abnormalities seen on T2-weighted brain magnetic resonance imaging, and by the presence of either headache, altered mental status, seizures, or visual disturbances.
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PMID:Hypertensive encephalopathy, reversible occipitoparietal encephalopathy, or reversible posterior leukoencephalopathy: three names for an old syndrome. 1034 93


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