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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic mucocutaneous candidiasis (CMCC) is a clinical syndrome characterized by persistent and recurrent Candida albicans infections of the mucous membranes and skin often associated with immunodeficiency. In order to gain insight into the frequency and severity of non-candidal infections in children with CMCC, four patients with CMCC are described in detail and 60 previously reported cases are reviewed. Fifty percent of children with CMCC had significant infections with other fungi, bacteria and viruses. Recurrent bacterial pneumonias and bronchiectasis were a major cause of morbidity and mortality. In addition, there were a large number of infections, in both the lung and other sites, due to opportunistic organisms. Thus the clincial syndrome of CMCC includes not only mucocutaneous candidiasis, endocrine failure and autoimmune phenomena, but patients with CMCC also show a remarkable susceptibility to non-candidal infections. These non-candidal infections represent a serious cause of morbidity for patients with CMCC.
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PMID:Non-candidal infections in children with chronic mucocutaneous candidiasis. 45 1

In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum concentrations of IgG and IgM were low, and serum and salivary IgA was not detected. T-lymphocytes were reduced in number and cell-mediated immunity in vivo and in vitro was impaired. Opsonisation, complement system, and neutrophil functions were normal.
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PMID:Immunodeficiency associated with laevocardia, bronchiectasis, and paranasal sinus anomalies. 72 96

The clinical and laboratory data for 15 patients with common variable immunodeficiency (CVI) (5 females and 10 males aged 3 years and 6 months to 40 years at first examination) were evaluated. The age of onset of infectious signs and symptoms ranged from 6 months to 35 years. Recurrent pulmonary infections predominated (86.6%), followed by chronic diarrhea (46.6%). Approximately 60% of the patients with pulmonary complaints presented chronic sequelae (bronchiectasis). Two developed a polymyositis-like picture. No neoplasms were observed. All patients presented immunoglobulin levels below 300 mg/dl and absence of antibody responses to poliovirus and to hemagglutinin. Two patients were negative when tested for autoimmunity. Cell immunity tested by the lymphoproliferative response in the presence of phytohemagglutinin was normal in 11 patients and depressed in 4. A decrease in the helper T population and inversion of the OKT4/8 ratio occurred in 13. Cimetidine treatment (1200 mg/day) applied to 5 patients for 4 weeks did not produce any clinical or laboratory improvement. Gamma globulin is the treatment of choice for these patients.
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PMID:Common variable immunodeficiency: a clinical and laboratory evaluation of 15 cases. 130 41

Chronic bronchitis is defined for epidemiologic and clinical purposes as the presence of productive cough for three months in each of two successive years. Based on symptoms, the term 'chronic bronchitis', therefore, does not describe one distinct disease. It is rather a collective name for the clinical manifestation of numerous different congenital or acquired chronic diseases of the trachea, the bronchi and the bronchioli. Cigarette smoking is the most consistently important (and preventable) determinant of chronic bronchitis. There are, however, other rare etiologic factors, including malformations, tumors, recurrent aspirations and bronchiectasis. The latter often occur in association with systemic disorders such as cystic fibrosis, immotile cilia syndrome, immunodeficiency, alpha 1-antitrypsin deficiency and others.
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PMID:[Chronic bronchitis: rare etiology]. 144 Apr 38

Primary humoral immunodeficiency is characterized by an abnormal immunoglobulin production. More than twelve forms are now known and a reviewed by the authors. The deficiency is responsible for bronchial infections which in the long term may result in bronchiectasis. It may also result in benign follicular hyperplasia. The specific treatment is substitutive and not devoid of hazards.
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PMID:[Bronchial complications of primary humoral immunodeficiency]. 180 44

Bacillus cereus is rarely a pulmonary pathogen but may cause pneumonia in immunocompromised patients. A patient with bronchiectasis and no recognisable immunodeficiency had this organism isolated during two infective exacerbations, once from respiratory secretions and once by blood culture. Ciprofloxacin treatment was effective on both occasions.
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PMID:Successful treatment of Bacillus cereus infection with ciprofloxacin. 190 95

To determine the best predictors of chronic pulmonary disease in patients with hypogammaglobulinemia, we evaluated the clinical records, chest x-ray films, and pulmonary function tests of 10 patients with X-linked agammaglobulinemia (XLA) followed for a mean of 12.5 years, and 12 patients with common variable immunodeficiency (CVID) followed for a mean of 10.5 years. These patients, most of whom were treated with intramuscular gamma globulin and long-term oral antibiotics, had very few pneumonias after diagnosis. The patients with XLA had 0.10 pneumonias per treatment year, and the patients with CVID had 0.18 pneumonias per treatment year. Seven of the 10 patients with XLA had normal chest x-ray films 8 to 15 years after diagnosis, and none had bronchiectasis. Pulmonary disease was more common and more severe in the group with CVID, but five patients in this group also had normal chest x-ray films after long follow-up. In the entire group of 22 patients, nine of the 10 patients with abnormal chest x-ray films on most recent evaluation already had pulmonary disease at the initial visit (p = 0.00002). These studies indicate that the best predictors of good pulmonary function in patients with hypogammaglobulinemia are early diagnosis and good compliance with gamma globulin replacement therapy and oral antibiotics.
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PMID:Retrospective analysis of the incidence of pulmonary disease in hypogammaglobulinemia. 207 89

We studied a nine-year-old boy with severe, recurrent infections. The patient was exposed in utero to azathioprine and prednisone. He had autoimmune hemolytic anemia, bronchiectasis, and Hodgkin's disease. The patient's circulating lymphocytes were normal in number and phenotype, but stimulation of the T-cell receptor by antigens, mitogens, and monoclonal antibodies failed to induce interleukin-2-receptor expression, interleukin-2 synthesis, or lymphocyte proliferation. The early biochemical events necessary to initiate lymphocyte activation--accumulation of the second messenger diacylglycerol, activation of the enzyme protein kinase C, and elevation of the free intracellular calcium concentration--failed to occur in this patient's lymphocytes. The defect in the lymphocyte could be corrected in vitro by two agents that bypass the receptor-mediated signal mechanism (the diacylglycerol analogue phorbol and the calcium ionophore ionomycin). Further studies localized the defect in signal transduction to the interaction between cell-surface receptors and the guanine nucleotide-binding protein. We conclude that this patient's immunodeficiency was caused by a defective coupling of surface receptors to signal-transducing proteins in his T lymphocytes, resulting in failure of lymphocyte activation.
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PMID:An immunodeficiency characterized by defective signal transduction in T lymphocytes. 278 45

The correction of immunodeficiency requires T-cells, B-cells and antigen-presenting cells, all cooperating with each other in a balanced manner, and able to protect the tissues of the host from intracellular viral infections. Full B-cell function may require more than one genetic haplotype, and has only been achieved between host and donor cells when these are very well matched. Otherwise it has been necessary for the donor T- and B-cells to displace the host B-cells. In infants, displacement is better achieved using Busulphan rather than irradiation, (which also impairs the tolerising influence of the host thymus). Full correction has been achieved in 4 kinds of lymphopaenic SCID (+/- reticular dysgenesis or cartilage-hair dysplasia) without induction. For 16 other errors of lymphocyte function, displacement induction is preferred to ensure donor T-B-cell cooperation, although Cyclophosphamide alone has worked for matched sibling donors. For 9 other defects all expressed in phagocytes, which nevertheless occupy bone marrow space, displacement induction is essential. Elective transplants into fit hosts (e.g. no bronchiectasis) from matched sibling donors enabled 74 of 75 patients to leave hospital alive and well, with only 1 fatal acute GvHD. Currently, experienced teams can therefore consider another 20 diseases which might be better treated by bone marrow transplantation from matched siblings. In contrast, emergency transplants into unfit recipients produce only 60% survival. Transplants from donors sharing one genetic haplotype have reached 50% survival and there is room for improvement, but they are preferred to the use of unrelated donors or foetal tissues.
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PMID:Immunodeficiencies better treated by transplantation. 293 2

Common variable hypogammaglobulinemia (immunodeficiency), a disorder characterized by late-onset immunoglobulin deficiency and lack of humoral immunity, has a variable association with bronchiectasis, cholelithiasis, nodular lymphoid hyperplasia, gastrointestinal neoplasia, megaloblastic anemia, and malabsorption. The patient described in this report had all of the above except neoplasia. In addition, he had calcium oxalate renal stones probably secondary to his malabsorption. The first case demonstrating the beneficial effect of home hyperalimentation in patients with severe malabsorption refractory to other treatments is described. Home hyperalimentation overnight allows the patient freedom for daily activities while also being more cost-effective than in-hospital parenteral nutrition.
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PMID:Home hyperalimentation for common variable hypogammaglobulinemia with malabsorption secondary to intestinal nodular lymphoid hyperplasia. 311 40


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