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)

We undertook this study to determine whether patients with late-onset hypogammaglobulinemia, who are at very high risk for gastric cancer, have a reduced secretion of gastrin after stimulation with food or bombesin, a potent gastrin-releasing stimulus. We compared the plasma gastrin responses to bombesin and to a standard test meal in 18 patients with late-onset hypogammaglobulinemia with those in patients with X-linked agammaglobulinemia, early-onset hypogammaglobulinemia, or hypogammaglobulinemia due to lymphoproliferative cancer, and in 30 normal control subjects. Thirteen of 18 patients with late-onset hypogammaglobulinemia (72 percent) had an abnormally low gastrin response to bombesin, as compared with none of 21 patients with other forms of hypogammaglobulinemia (P less than 0.05). After a test meal, abnormally low gastrin secretion was found in 6 of 14 patients with late-onset hypogammaglobulinemia (43 percent) and in 1 of 18 patients with other forms of the disease (6 percent) (P not significant). The plasma gastrin responses to stimulation with bombesin or food distinguished late-onset hypogammaglobulinemia from other forms, with sensitivities of 72 and 43 percent and specificities of 100 and 94 percent, respectively. Stimulated gastrin response can therefore be used as a marker for this type of immunodeficiency. The test responses also showed heterogeneity among patients with late-onset hypogammaglobulinemia and may help to identify patients with an increased risk for gastric cancer.
 ...
PMID:Decreased gastrin secretion in patients with late-onset hypogammaglobulinemia. 337 28

Kaposi's Sarcoma (KS) in young individuals is unusual and most often associated with cellular immunodeficiency caused by infective or other neoplastic diseases. It has recently been highly associated with the Acquired Immunodeficiency Syndrome (AIDS). We report the case of a heterosexual 29 year aged man with no evidence of underlying malignancy or infectious diseases. Antibodies to the Human Immunodeficiency Virus (HIV) were absent on repeat testing. His immunological profile demonstrated elevated number of CD8+ cells, normal number of CD3+ and CD4+ cells and hypogammaglobulinemia. These data are distinctly different from those described with AIDS associated KS. The development of KS in young individuals of mediterranean origin may reflect mild degree of immune abnormalities in the absence of infection with HIV.
 ...
PMID:Kaposi's sarcoma in a young man in absence of AIDS or other known causes of immune suppression. 343 54

Infection with the human immunodeficiency virus (HIV) is characteristically associated with hypergammaglobulinemia in both adult and pediatric cases. We report herein four infants who had an HIV infection in association with severe hypogammaglobulinemia and did not exhibit antibodies against HIV. HIV was isolated antemortem or postmortem in all four infants from either peripheral blood, cerebrospinal fluid, or body tissues. HIV infection could be presumed to be acquired transplacentally in two infants and by way of infected blood transfusions during the neonatal period in the other two. Each infant became symptomatic within the first year of life and developed rapidly progressive manifestations of the disease. Features that were common to all four infants include premature birth, failure to thrive, hepatomegaly, and progressive neurological abnormalities that were associated with intracranial calcifications. We concluded that, when infection occurs early in development either by transplacental exposure to the virus or from blood transfusion in small premature infants, hypogammaglobulinemia and deficiency of antibody production leading to the absence of antibody responses on which diagnosis is usually based can occur. Furthermore, progressive central nervous system disease may be a frequent finding in such infants, and this may lead to cerebral calcifications that must be attributed to the HIV infection itself and not to complicating infections--e.g., toxoplasmosis. It is suggested that patients with hypogammaglobulinemia, antibody deficiency syndrome, and central nervous system disease have an extremely bad prognosis.
 ...
PMID:Prematurity, hypogammaglobulinemia, and neuropathology with human immunodeficiency virus (HIV) infection. 347 85

Five cases (3 men, 2 women) of late-onset variable immunodeficiency syndrome (CVID), characterized by similar clinical and immunological findings as well as histological demonstration of chronic granulomatous infection, are reported. All patients had frequent attacks of respiratory infections with recurrent bronchitis and pneumonia. In addition to predominating basally localized streaky-nodular lung changes all patients had hepatosplenomegaly and granulomatous infections of other organs. Immunologically, marked hypogammaglobulinaemia of all Ig classes, lymphopenia, and absence of terminal B-cell maturation were predominant. In-vitro tests under pokeweed-mitogen failed to demonstrate terminal plasma-cell differentiation of B-lymphocytes and thus Ig synthesis. Without pokeweed-mitogen there were largely nonsecretory B-blasts with abnormal granulated cytoplasmic Ig formation. Skin testing with Multitest application revealed almost complete anergy, both in the Arthus (24 h) and the late reactions (48 and 72 h). Nonetheless, T-cell reaction in-vitro was much less affected than B-cell function. "Natural killing" and antibody-dependent cytotoxicity were normal or slightly increased.
 ...
PMID:[Acquired immunodeficiency syndrome with chronic granulomatous inflammation. Clinically definable special form of the variable immunodeficiency syndrome]. 348 49

We investigated the prevalence of phenotypically immature IgG B cells (i.e., coexpressing surface IgG and IgM) in the peripheral blood of 12 patients with hypogammaglobulinemia and in normal individuals. Patients had ataxia-telangiectasia (N = 1), hyper-IgM combined immunodeficiency (N = 1), or common variable immunodeficiency (CVI). IgG/IgM-positive B cells were evaluated by two-color immunofluorescence using fluorescein- or rhodamine-conjugated goat antiserum; to minimize artifacts due to in vivo cytophilic binding of autologous IgG, cell-bound cytophilic Ig were eluted at pH 4 and Fc receptors were blocked by heat-aggregated rabbit IgG before fluorescent staining. All patients, except two with late-onset CVI, had markedly increased proportions of double-stained IgG B cells (56 to 100% of IgG-bearing B cells) in comparison with normal individuals (11 to 33%).
 ...
PMID:Phenotypically immature IgG-bearing B cells in patients with hypogammaglobulinemia. 348 52

A simian acquired immunodeficiency syndrome (SAIDS) associated with retroperitoneal fibromatosis (RF) has been observed in several species of macaque at the Washington Regional Primate Research Center. Clinical signs were recurrent diarrhea, weight loss, mesenteric lymphadenopathy, and opportunistic infections. Most affected macaques in the later stages of illness showed marked immunodeficiency. Response of peripheral blood mononuclear cells to mitogens was impaired significantly. There was sharply depressed primary and secondary antibody response to the T-cell dependent antigen, bacteriophage phi X174. Affected monkeys did not switch from IgM to IgG antibody following a secondary immunization, as did normal macaques. Twenty-four (67%) of 36 affected animals with progressive RF or deteriorated stages of illness had hypoproteinemia and hypoalbuminemia. Quantitative serum immunoglobulins of 23 cases showed that eight (35%) had hypogammaglobulinemia, six (26%) had hypergammaglobulinemia, and the remainder (39%) were within the normal range. Opportunistic infections were predominantly bacterial pathogens. Type D retrovirus appeared to be closely associated with RF-affected macaques (12/12 or 100%). The case fatality rate (including animals sacrificed after prolonged illness) was 98%. The leading cause of death was due directly to RF lesions in 43%, to enterocolitis in 36%, septicemia in 12%, amyloidosis in 5%, and malignant lymphoma (2%). Clinical, immunologic and pathologic changes reveal an acquired immunodeficiency syndrome that has many similarities to human AIDS. SAIDS and RF may be a useful model for studying human AIDS.
 ...
PMID:Retroperitoneal fibromatosis and acquired immunodeficiency syndrome in macaques: clinical and immunologic studies. 348 18

Tolerance, clinical effects and kinetics of an unmodified immunoglobulin preparation for intravenous use were investigated in 4 patients with advanced chronic lymphocytic leukemia. Previously, good tolerance of the preparation had been found in 49 immunologically normal patients. The four patients with secondary humoral immunodeficiency received doses of 140-360 mg IgG/kg per infusion as outpatients at monthly intervals. With one exception, no acute infections (pneumonitis), as commonly seen before, were observed during the observation time of 24 to 68 weeks, and the pre-existing chronic infections (bronchitis, sinusitis etc.) remained compensated without antibiotics. In all four patients tolerance of the preparation was good. In all cases of hypogammaglobulinemia a dose-dependent increase in the serum IgG concentration was observed immediately after the infusion. However, persistence of the serum IgG increase showed considerable interindividual differences. The half life of the tetanus and HBs antibodies (21.7 to 34.4 and 19.7 to 25.7 days respectively) found in 4 healthy volunteers is within the biological range. This indicates an unmodified structure of the antibodies of the IgG class contained in the preparation used.
 ...
PMID:[Tolerability and pharmacokinetics of an intravenous immunoglobulin preparation in immunologically normal subjects and tolerability in patients with hypogammaglobulinemia resulting from chronic lymphatic leukemia]. 351 30

Patients with primary immunodeficiency disorders were evaluated for three aspects of natural defense: natural killer (NK) cells which lyse HSV-infected fibroblasts [NK(HSV-FS)], NK cells which lyse K562 tumor targets [NK(K562)], and interferon-alpha generation. In addition, capacity to make interferon upon challenge with other commonly used inducers was also evaluated. Most patients with severe combined immunodeficiency disease (SCID) and deficits of both T- and B-cell function demonstrated normal NK function with one or both targets. Six of eight SCID patients generated interferon-alpha at or below the lower limit of normal while only two made clearly normal levels. Six of 10 patients with Wiskott-Aldrich syndrome (WAS) had normal NK(K562) and five of 10 generated normal levels of interferon-alpha but all had severely deficient NK(HSV-FS). Patients with Bruton's agammaglobulinemia demonstrated normal NK and interferon generation, as did patients with common variable immunodeficiency, even when subdivided into patients with T-cell proliferative deficiencies and those with only hypogammaglobulinemia. Natural defense parameters may help categorize patients with SCID and WAS and help define these heterogeneous diseases.
 ...
PMID:Natural killer cell function and interferon generation in patients with primary immunodeficiencies. 369 44

Two brothers presented with unusual facial features, microcephaly, developmental delay, and severe postnatal growth retardation. They both developed eczema in infancy and have had recurrent infections. Additional physical findings in both boys included hypogonadism, flexion contractures, hypoplastic patellae, and scoliosis. Their facial similarity was striking with sloping foreheads, beaked noses, large, protruding ears, and micrognathia. Low levels of serum gammaglobulins and defective chemotaxis were present in both boys in infancy. The hypogammaglobulinaemia was transient and improved, reaching normal levels by 3 1/2 years and 15 months, respectively. Defective chemotaxis and recurrent infections have persisted to the present. Both parents were normal. The mode of inheritance was not clear, as both X linked and autosomal recessive patterns were possible. Although patients with congenital malformations who also had immunodeficiency have previously been reported, immune system abnormalities, especially those of a transient nature, may frequently go unrecognised.
 ...
PMID:Microcephaly, short stature, and developmental delay associated with a chemotactic defect and transient hypogammaglobulinaemia in two brothers. 374 38

A case of common variable immunodeficiency in a pregnant woman is presented. Lobar pneumonia developed in the sixth month of the pregnancy and her serum immunoglobulin levels were found to be extremely low. She was treated successfully with immune human serum globulin and antibiotics. She delivered a full-term baby without any troubles. Various immunologic studies were done in the peripheral blood of the patient and the neonate. Consequently, functional abnormalities of helper T cells were considered to be responsible for the hypogammaglobulinemia.
 ...
PMID:Agammaglobulinemia in a pregnant woman. 377 61


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>