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 9-month-old baby girl who failed to develop normally due to nutritional neglect and secondary immunodeficiency characterized by marked thymic involution is reported. The child died of systemic Pseudomonas aeruginosa (P. aeruginosa) infection manifested in pneumonia, lung abscesses, bacterial endocarditis and ecthyma gangrenosum. At autopsy the child was 64 cm in height (normal for a 4- to 5-month-old child) and 5.1 kg in weight (normal for a 2- to 3-month-old child). Multiple gangrenous ecthymas, consisting of deep ulcers, induration and inflammation, were observed in the skin over the entire body. The lungs showed hemorrhagic pneumonia, multiple lung abscesses, and necrotizing arteritis in the abscesses and surrounding areas. The thymus weighed 2.3 g and showed marked involution. Histological examination showed so-called nutritional thymectomy characterized by severe cortical atrophy and clustering, cystic dilation and amorphous changes of the Hassall's corpuscles. In the heart, dark brown verrucae were present at the attachment sites of the tendinous cords of the papillary muscle in the anterior and posterior cusps of the mitral valve, suggesting infectious endocarditis. Bacteriological examination demonstrated P. aeruginosa in the ecthymas, lung abscesses and blood. As primary immunodeficiency was considered unlikely, immunodeficiency secondary to thymic involution following malnutrition seemed to have led to a fatal systemic infection with P. aeruginosa, whose virulence is generally weak. This suggests a close association of the development of such infection and immunodeficiency with child neglect.
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PMID:Child neglect followed by marked thymic involution and fatal systemic pseudomonas infection. 191 16

The thymuses from 20 simian immunodeficiency virus (SIV)-infected and 4 uninfected rhesus monkeys were examined at intervals after infection to determine whether there were specific SIV-induced lesions, to document the serial distribution of SIV antigens, mRNA, and DNA, to quantitate the number of infected cells, and to correlate thymic changes with other parameters of infection. The following techniques were used: gross pathology, histopathology, immunohistochemistry, electron microscopy, in situ hybridization, polymerase chain reaction, and limiting dilution culture. Thymic involution due to loss of lymphocytes was apparent 8 weeks after inoculation. No epithelial damage or loss of Hassall's corpuscles was observed. Culture was the most sensitive technique for detecting SIV, being positive in 19 of 20 inoculated monkeys. The polymerase chain reaction was negative in one thymus that was positive at a low level by culture. In situ hybridization was positive in 14 of 19 thymuses examined, with a few macrophages in the cortex having a strong signal and numerous lymphocytes in the medulla having a weak signal. Mature viral particles and viral budding could not be demonstrated by electron microscopy. The number of cells positive for viral RNA by in situ hybridization correlated with the level of serum antigenemia. These observations suggest that thymic macrophages and lymphocytes are infected with SIV within 2 weeks after inoculation. SIV apparently directly causes loss of thymic lymphocytes and immunodeficiency without infecting or damaging the thymic epithelium. No specific SIV-induced lesions were recognized. The number of cells in the thymic medulla expressing SIV RNA correlates with the level of serum antigen, which has been previously shown to be correlated with disease progression.
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PMID:Thymus in simian immunodeficiency virus-infected rhesus monkeys. 192 30

The ts1 mutant of Moloney murine leukemia virus TB causes degenerative neurologic and immunologic disease in mice, characterized by development of spongiform encephalomyelopathy resulting in hindlimb paralysis, marked thymic atrophy associated with immunodeficiency, and generalized body wasting. To investigate the pathogenesis of the thymic atrophy caused by ts1, we constructed a chimeric virus, ts1-Cas(NS), in which a major portion of the U3 region of the long terminal repeat of ts1, a T-lymphotropic and neurovirulent murine leukemia virus, was replaced by the corresponding U3 region of Cas-Br-E, a B-lymphotropic and neurovirulent murine leukemia virus. In FVB/N mice, ts1-Cas(NS) induced paralytic and wasting disease with incidence, severity, and latency similar to that induced by ts1, but it failed to cause thymic atrophy as severe as that observed in ts1-infected mice. Furthermore, thymocytes cultured from ts1-Cas(NS)-infected mice died at a much slower rate than those of ts1-infected mice. The U3 substitution in ts1-Cas(NS) specifically diminished the ability of the virus to replicate in the thymus, whereas viral replication in the spinal cord was not significantly affected; thus, neurovirulence was not changed. The correlation of reduced thymic atrophy with decreased thymic viral titers and the decreased ability of ts1-Cas(NS) to cause thymocyte death in mice suggest strongly that the marked thymic atrophy in ts1-infected mice is not an indirect effect occurring secondary to neurodegenerative and wasting disease but is a direct cytopathic effect of high-level viral replication in the thymus.
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PMID:Alteration from T- to B-cell tropism reduces thymic atrophy and cytocidal effects in thymocytes but not neurovirulence induced by ts1, a mutant of Moloney murine leukemia virus TB. 192 61

The DiGeorge anomaly, DGA (formerly termed DiGeorge syndrome), is now known to be a developmental field defect in which pharyngeal pouch derivatives do not arise, usually because of inadequate neural crest contributions. The conditions in which this occurs include exposure to teratogens, cytogenetic abnormalities, and Mendelian disorders. As a result, the facies and cardiovascular defects which occur are very characteristic. Two rare conotruncal anomalies, type B interrupted aortic arch and truncus arteriosus account for over half of the cardiac lesions seen in DGA. Failure of descent of the thymus is extremely common in DGA, but immunodeficiency which requires correction occurs only in approximately 25% of the cases. The term, complete DGA, should be reserved for those patients in need of reconstitution of the immune system. One can identify those patients requiring treatment of the thymic defect by T cell enumeration and in vitro proliferation assays. Two alternatives for therapy are thymus transplantation and bone marrow transplantation from a HLA matched sibling.
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PMID:The DiGeorge anomaly. 193 Oct 5

We have investigated the effects of graft-versus-host disease on T cell differentiation in the murine thymus. We previously reported that GVH-induced thymic dysplasia results in a T cell immunodeficiency associated with a lack of IL-2 production. This deficiency in IL-2 production may be the result of a reduction in the number of L3T4+Lyt-2- IL-2-producing cells or of a functional defect in this population. To test these two alternatives, flow cytometry analysis of L3T4 and Lyt-2 antigen expression on thymocytes along with immunofluorescence microscopy were employed to assess T cell phenotypes in thymuses of GVH mice. GVH reactions were induced by injecting 40 x 10(6) C57BL/6 (B6) or A strain lymphoid cells into C57BL/6xAF1 (B6AF1) mice. Thymocyte populations were quantitated on different days after GVH induction. In the normal thymus, the ratio of L3T4/Lyt-2 single positive cells was greater than 2:1. In contrast, such a ratio was less than 1:1 in the atrophic GVH thymus, owing to a selective reduction in the number of L3T4+Lyt-2- cells. Following cortisone treatment the ratio of L3T4/Lyt-2 single positive thymocytes in normal F1 mice was approximately 3:1, whereas in GVH animals this ratio was reversed (1:2). This reversal was due to a selective reduction in the absolute numbers of L3T4+Lyt-2- cells. In adrenalectomized GVH animals, thymic cortical atrophy was prevented and normal ratios of L3T4/Lyt-2 single positive cells were observed. However, when these animals were treated with cortisone, the L3+T4/Lyt-2- population was more sensitive than was the L3T4- Lyt2+ population, thereby resulting in a 1:2 L3T4/Lyt-2 ratio. These results demonstrate that single positive L3T4 cells are present in the murine GVH thymus, yet they have not acquired cortisone resistance, a trait normally attributed to this mature thymic subset. It appears that the GVH dysplastic thymus can support the differentiation of L3T4+Lyt-2- cells--however, such a thymus is unable to confer cortisone resistance upon this population. Consequently, these cells appear to be eliminated when exposed to corticosteroids in peripheral lymphoid tissue.
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PMID:T cell subsets in the thymus of graft-versus-host immunosuppressed mice. Sensitivity of the L3T4+Lyt-2- subset to cortisone. 198 97

UCD line 140 chickens have been previously reported to develop a syndrome of spontaneous 7s immunoglobulin deficiency and the presence of autoantibodies. Earlier studies demonstrated that these inbred birds have normal peripheral blood T and B cell numbers; they also respond normally to allogeneic stimulation. Although the 7s immunodeficiency does not manifest itself until several months of age, line 140 birds have a premature degeneration of bursa. Because of the recent development of monoclonal reagents specific for bursal elements, including surface epithelium, basement membrane associated epithelium, follicle associated epithelium, and lymphoid subpopulations, we have examined line 140 and control birds for the expression of bursal epithelial cell antigens. Line 140 birds, in contrast to control chickens, have a dramatic early alteration in the expression of an epithelial cell marker in the bursa, thymus, and intestine. Moreover, to further address this issue, we transplanted bursa from 10-day embryos onto the chorioallantoic membrane, a privileged site. Bursae from control birds became abnormal when transplanted onto line 140 CAM; they remained normal when transplanted among several control chicken lines. In contrast, line 140 bursa remained abnormal independent of the transplant procedure. Due to the marked bursal abnormality observed specific to the dysgammaglobulinemia chicken line, we propose that the microenvironmental features of line 140 bursa may predispose these birds to the development of humoral immunodeficiency and autoantibodies.
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PMID:Abnormalities associated with the bursal microenvironment in spontaneous dysgammaglobulinemia of UCD line 140 chickens. 200 41

HIV infection results in the destruction of the thymus-dependent cellular immune system and death due to opportunistic infection and malignancy. Immunosuppressive influences (other sexually or blood-transmitted viruses, HIV-derived peptides, semen, poor nutrition, drugs, etc.) favor the progression of the disease. Although immunorestorative agents may be expected to delay progression of the disease, John Hadden argues that no agent has yet proven useful in reversing the immunodeficiency in full-blown AIDS. However, two thymomimetic drugs, isoprinosine and diethyldithiocarbamate, inhibit the development of infections in patients with pre-AIDS in large multicenter trials, and preliminary data from trials with two thymomimetic peptides, thymopentin and ImReg-1, in pre-AIDS patients are encouraging.
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PMID:Immunotherapy of human immunodeficiency virus infection. 205 87

We have investigated in utero human immunodeficiency virus type 1 (HIV-1) transmission by analyzing human fetal tissues for the presence of viral DNA by means of the polymerase chain reaction (PCR). Thirty three fetal samples: thymus, spleen, and peripheral mononuclear blood cells (PMBC) were obtained at abortion (16 to 24 weeks) from HIV-1-infected asymptomatic women. The results of HIV-1-DNA detection were considered only in 9 cases where contamination of fetal samples by infected mother cells could be definitely eliminated by using primers specific for a polymorphic cellular locus. PCR allowed the identification of HIV-1 DNA sequences in 6/8, 8/9, and 5/9 of specimens from thymus, spleen, and PMBC, respectively. Positive results were shown in fetuses as early as 16 weeks. Viral cultures as well as assays for serum p24 HIV-1 antigen were negative in 9.9 and 33/33 tested, respectively. Therefore, our results indicate early and frequent in utero HIV-1 infection. Different patterns of viral activation after birth might then lead to either rapid or delayed onset of acquired immunodeficiency syndrome.
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PMID:Frequent and early in utero HIV-1 infection. 206 30

Graft-versus-host disease (GVHD) is caused by the disorder of self-tolerance mechanisms in the thymus and of peripheral tolerance mechanisms. Several cytokines also modulate GVHD, thus inducing complicated clinical features; therefore, it is important to determine which cytokines are mainly involved in the induction of GVHD in order to suppress the production of such cytokines for controlling GVHD. GVHD is closely related to immunodeficiency through T cell and B cell dysfunction. Although cytokines can potentiate these immunological dysfunction, GVHD may be simultaneously augmented by exogenous administration of cytokines. On the contrary, cytokines regulate hematopoiesis as well. Therefore, we should analyze the kinetics of cytokines and their receptor expressions as well as the influences of these factors on immune system and hematopoiesis to control GVHD and immunodeficiency.
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PMID:[The control of GVHD and immunodeficiency]. 206 81

Morphological examinations of 58 autopsy cases of visceral candidiasis in newborns were performed. Direct correlation between the severity of thymus lesions and the degree of changes in organs produced by candidiasis was observed. The most severe candidiasis was found in cases of congenital immunodeficiency. This suggests that the morphological features of candidiasis may be the manifestation of immunodeficiency state.
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PMID:[Morphologic manifestations of candidiasis in newborns with immunodeficiency]. 208 65


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