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

The diagnoses which may be arrived at by examination of peroral small bowel mucosal biopsy specimens are presented. Celiac sprue, unclassified sprue (refractory sprue), infectious gastroenterititis, stasis syndrome and kwashiorkor have a severe mucosal lesion. Other clinical conditions are required to establish the diagnosis in these diseases. A number of diseases have specific diagnostic features. Included are Whipple's disease, abetalipoproteinemia, collagenous sprue, primary intestinal lymphoma, eosinophilic gastroenteritis, giardiasis, coccidiosis, strongyloidiasis, lymphangiectasis and the intestinal immunodeficiency diseases. Mucosal abnormalities may be present in other diseases but the diagnoses are usually made on other criteria than small bowel biopsy. These include vitamin B12 or folic acid deficiency, Crohn's disease, gastrinoma, acrodermatitis enteropathica, amyloidosis, chronic granulomatous disease, lipid storage diseases, histoplasmosis, capillariasis, cytomegalovirus infection, schistosomiasis and macroglobulinemia.
 ...
PMID:Histologic diagnosis of diseases of malabsorption. 51 56

Zinc deficiency is a common nutritional problem observed both in human and in animal populations that has profound effects on host defense mechanisms. Using the young adult mouse as a model, it has been demonstrated that a moderate period of suboptimal zinc causes thymic atrophy, lymphopenia, and alterations in the proportions of the various subsets of lymphocytes and mononuclear phagocytes. As a result, antibody-mediated responses to both T cell-dependent and T cell independent antigens are significantly reduced. Cytolytic T cell responses, natural killer (NK) cell activity, and delayed-type hypersensitivity (DTH) reactions are also depressed. Suboptimal zinc during in utero development of mice causes persistent states of immunodeficiency in the offspring that can even be transferred to subsequent generations. In regard to human immunological consequences of zinc deficiency, patients with the genetic disorder of zinc absorption, acrodermatitis enteropathica, also exhibit atrophic thymuses, lymphopenia, anergic DTH responses, and reduced NK cell activity. Patients suffering from sickle cell anemia or uremia with associated deficiencies in zinc exhibit similar immune deficiencies. An additional outcome of these studies has been shown to be an essential cofactor for thymulin, one of the thymic hormones. Furthermore, addition of zinc salts to culture can polyclonally activate lymphocytes as well as augment responses to mitogens in adjuvant-like manner.
 ...
PMID:Interrelationships between zinc and immune function. 348 44

The ability to define subpopulations of immunologically competent lymphocytes has permitted an enhanced understanding of the interaction between functionally distinct components of the immune system. T cells can provide help in antibody formation or they may suppress antibody production. Abnormal immunoregulatory mechanisms have been demonstrated in the hyperimmunoglobulin E-recurrent infection syndrome. This disorder is associated with a marked elevation of IgE and specific elevations of IgE antibodies directed toward staphylococcal antigens. Abnormal T cell regulation of immune responses has been demonstrated. Graft-versus-host disease (GVHD) occurs in an immunodeficient patient who has received an infusion of immunocompetent cells. The diagnosis of graft-versus-host (GVH) reaction may be complicated by the protean manifestations of the disorder. The acute form, consisting of a maculopapular rash, fever, and diarrhea, may be confused with acute infection or drug reaction. Chronic GVHD has been incorrectly diagnosed as histiocytosis X, acrodermatitis enteropathica, or scleroderma. Utilizing chromosome markers and/or identification of histocompatibility antigens, the presence of circulating lymphocytes from donor immunocompetent cells (blood transfusion, maternal source) can be documented. The development of sensitive technics for identifying cells can establish a precise diagnosis. Certain immunodeficiency disorders can be identified by biochemical means. Biotin-dependent multiple carboxylase enzyme deficiency is associated with a chronic dermatitis, alopecia, ataxia, and secondary infection of the skin with Candida. The disorder responds promptly to the administration of biotin with correction of dermatologic, neurologic, and immunologic abnormalities.
 ...
PMID:New insight into the causes of immunodeficiency disorders. 638 1

Protracted diarrhoea in early infancy gives rise to many diagnostic and therapeutic problems. Jejunal biopsy often reveals villous atrophy of varying degrees. Severe reduction of small intestinal absorptive area causes secondary monosaccharide malabsorption, as well as secondary disaccharide deficiency, consequences which are relevant in any attempts at oral feeding. Morphologic, metabolic, endocrinological and microbiological studies have to be undertaken in order to establish a definitive diagnosis in protracted diarrhoea, but these studies often fail to reveal the aetiology of the disease. Immunologic abnormalities like phagocyte dysfunction, thymic atrophy and hypoplasia of B-cell regions in lymph nodes might be secondary events, but some types of immunodeficiency are of primary importance in the development of protracted diarrhoea. Total parenteral nutrition in many cases has to be instituted, with all its implications and hazards: septicaemia is the most dangerous of these. Zinc deficiency and acrodermatitis enteropathica may occur during total parenteral nutrition, and zinc deficiency secondarily contributes to the symptoms of diarrhoea. Parenteral administration of zinc is able to overcome these effects.
 ...
PMID:Protracted diarrhoea: secondary monosaccharide malabsorption and zinc deficiency with cutaneous manifestations during total parenteral nutrition. 677 99

Malnutrition has been linked in field studies with increased susceptibility to infection, often associated with severe marasmus or kwashiorkor. However, studies by Jose and colleagues revealed an apparent paradox: while B-cell immunity was decreased by chronic moderate malnutrition, several aspects of T-cell immunity were enhanced. In extensive experimental studies we have analyzed the effects of dietary restriction in immunologic function and development of disease. Our investigations may be grouped into three related areas. 1) Differential effects of protein or protein-calorie malnutrition on B-cell and T-cell immunity. While antibody-mediated immunity was impaired in animals moderately restricted with respect to protein or total calories, several T-cell functions were consistently enhanced in mice, rats, guinea pigs, and monkeys. 2) Influence of restricting a single nutritional element, the trace metal zinc, on immunologic function. Zinc deficiency produces progressive thymic involution and a progressive loss of T-cell immunity functions in mice and rats. While congenital failure to absorb this element normally is the single cause of hereditary acrodermatitis enteropathica, a frequently lethal disease both in humans and in cattle, acrodermatitis enteropathica has also been linked with common variable immunodeficiency disease, total parenteral alimentation with preparations lacking zinc, several forms of cancer, marasmus, and kwashiorkor. 3) Inhibition by dietary restriction of development of the diseases of aging. Disorganization of thymus-derived immunity, and development with aging of genetically determined diseases in several strains of mice, can be sharply curtailed or even prevented by reducing the intake of total calories or fat. Similarly, development of mammary cancer in C3H female mice is prevented by restricting dietary intake of fat.
 ...
PMID:Nutritional modulation of immune responses. 696 92

Zinc and immune function relationship has been extensively studied. Both in experimentally induced mineral deficit and in genetically determined deficit observable in acrodermatitis enteropathica and in enteropathy of Danish A-46 cattle, a B and T dependent antibody response decrease, a T dependent cytolytic response decrease and a natural killer cytotoxic activity decrease are present noteviously. Serious reduction of the immune function is present, in proportion to the value of low zinc plasmatic level, in elderly patients, in malnourished and seriously zinc deficient children, in patients subjected to total parenteral supply, in HIV infections and especially in evident AIDS: in this condition the plasmatic zinc level can be considered, together with the CD4+ lymphocytes amount and the B2-microglobulin value, a disease progression marker. Zinc immunostimulating action mechanisms are complex, although thymic hormone (of which zinc is essential cofactor) stimulation seems to be most important. Zinc supplementation, also parenterally, can be useful in immunodeficiency (in the elderly, in the post-surgical patients, in genetically determined or alimentary induced deficit, in AIDS.
 ...
PMID:Zinc and immune function. 747 75

The results of more than three decades of work indicate that zinc deficiency rapidly diminishes antibody- and cell-mediated responses in both humans and animals. The moderate deficiencies in zinc noted in sickle cell anemia, renal disease, chronic gastrointestinal disorders and acrodermatitis enteropathica; subjects with human immunodeficiency virus; children with diarrhea; and elderly persons can greatly alter host defense systems, leading to increases in opportunistic infections and mortality rates. Conversely, short periods of zinc supplementation substantially improve immune defense in individuals with these diseases. Mouse models demonstrate that 30 d of suboptimal intake of zinc can lead to 30-80% losses in defense capacity. Collectively, the data clearly demonstrate that immune integrity is tightly linked to zinc status. Lymphopenia and thymic atrophy, which were the early hallmarks of zinc deficiency, are now known to be due to high losses of precursor T and B cells in the bone marrow. This ultimately leads to lymphopenia or a failure to replenish the lymphocytic system. Glucocorticoid-mediated apoptosis induced by zinc deficiency causes down-regulation of lymphopoiesis. Indeed, zinc itself can modulate death processes in precursor lymphocytes. Finally, there is substantial evidence that zinc supplementation may well reduce the impact of many of the aforementioned diseases by preventing the dismantling of the immune system. The latter represents an important area for research.
 ...
PMID:The dynamic link between the integrity of the immune system and zinc status. 1080 51

Dominant types of viral hepatitis are presently A, B, and C with prophylactic immunization available only for A and B. Hepatitis B and C and human immunodeficiency virus (HIV) infection constitute a worldwide scourge and treatment is far from satisfactory. Each produces severe oxidative stress (OS) and secondary cellular damage of varying severity and, as in toxic hepatitis, progression and regression are dependent on redox balance between oxidation and antioxidation. Experimental and clinical studies suggest that xenobiotics and co-infections exert cumulative, detrimental effects on their pathogeneses and further deplete antioxidants. It is proposed therefore that in the clinical management of these infections and especially in their early stages, considerable benefit should accrue from antioxidant repletion at dosages substantially above recommended daily allowances (RDAs) in conjunction with a nutritious high protein diet. Because plasma zinc and selenium concentrations are very low, their replenishment by high dosages is urgent and mandatory particularly in advanced HIV infections bordering on acrodermatitis enteropathica. Also recommended is their long-term continuance at high normal levels.
 ...
PMID:Oxidative stress in viral hepatitis and AIDS. 1535 Dec 35

Atopic is the most common of the dermatitides seen in infancy and childhood, but there are numerous other diseases that can mimic the skin findings. These include seborrheic dermatitis, immunodeficiency, and psoriasis in infancy; scabies, tinea corporis infection, perioral, nummular, contact, and molluscum dermatitis in childhood. It is sometimes extremely difficult to differentiate between ichthyosis and AD, and it is also important to differentiate AD from erythrodermic conditions including acrodermatitis enteropathica, biotin deficiency, and Netherton syndrome. A rare condition in children that may mimic AD is mycosis fungoides.
 ...
PMID:The differential diagnosis of atopic dermatitis in childhood. 1666 89

Plasma copper and zinc concentrations were measured in 58 patients with a laboratory-confirmed primary or secondary immunodeficiency. Patients with severe combined immunodeficiency, collagen vascular disease with depressed cell-mediated immunity, and acquired or congenital acrodermatitis enteropathica had mean plasma zinc concentrations substantially below the lower limit of normal. In contrast, patients with primary humoral and polymorphonuclear leukocyte defects had normal plasma zinc concentrations. Patients with primary polymorphonuclear leukocyte defects had a mean plasma copper concentration substantially above the upper limit of normal. Those subjects with primary humoral immunity defects also had significantly elevated plasma copper concentrations in comparison to controls. Plasma copper concentrations in patients with severe combined immunodeficiency or acrodermatitis enteropathica were normal. Cutis laxa patients had low plasma zinc and copper concentrations. These data demonstrate that zinc and copper homeostasis are altered in come immunodeficiency disorders and may be important factors in host defenses. Since it is known that cellular immunity is impaired by zinc deficiency, patients with primary and secondary immunodeficiency states with appropriately documented mild or severe zinc deficiency should receive zinc supplements.
...
PMID:Plasma zinc and copper in primary and secondary immunodeficiency disorders. 2426 85


1