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)

Lung transplantation is an established procedure to treat patients with end-stage lung disease. The criteria for recipient selection are broadening to include patients with congenital defects of the immune system, such as X-linked hypogammaglobulinemia (XLA). We report 2 cases of successful double lung transplantation in patients with XLA. The 2 men had developed bronchiectasis and end-stage lung disease despite early institution of intravenous immunoglobulin (IVIG) replacement therapy. Before transplantation, hypogammaglobulinemia was well controlled with IVIG in both patients. After transplantation, IVIG was administered every 48 hours during the first 10 days and then tapered slowly in the following weeks until returning to an every 3 weeks schedule. One patient has been followed up for 12 months and the other for 6 months. Lung function normalized in the first case and showed a restrictive pattern in the second one. Lung transplantation may be considered as a therapeutic option for patients with XLA and end-stage lung disease. Regular administration of IVIG overcomes the high risk of infections due to the severe immunodeficiency and the intensive immunosuppressive therapy.
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PMID:Lung transplantation in patients with x-linked agammaglobulinemia. 1296 57

Respiratory syncytial virus (RSV) infection is the most frequent reason for hospitalization of infants in developed countries. Premature birth without or, especially, with chronic lung disease of prematurity, congenital heart disease, and T-cell immunodeficiency are conditions that predispose to more severe forms of RSV infection. Incomplete development of the airway, damage to the airway, and airway hyperreactivity underlie the increased morbidity of RSV infection in prematurely born infants. Pulmonary hypertension and cyanosis are associated with worse outcomes in infants with congenital heart disease, and prolonged viral replication accounts for more severe illness in immunocompromised individuals.
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PMID:Review of epidemiology and clinical risk factors for severe respiratory syncytial virus (RSV) infection. 1461 9

Mycobacterium avium (M. avium) has been described traditionally as an opportunistic organism that causes disseminated disease in the human immunodeficiency virus (HIV)-positive population and that acts as a pulmonary pathogen in patients with underlying lung disease such as chronic obstructive pulmonary disease (COPD) or previously diagnosed tuberculosis. Pulmonary involvement of M. avium may range from asymptomatic colonization of the airway to invasive parenchymal or cavitary disease. However, endobronchial lesions involved in M. avium infection are rare in either immunocompetent or immunosuppressed hosts. We report here endobronchial mycobacterial infection in a HIV-negative patient.
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PMID:Endobronchial lesions involved in Mycobacterium avium infection. 1468 4

Only 5 to 10% of immunocompetent humans are susceptible to tuberculosis, and over 85% of them develop the disease exclusively in the lungs. Human immunodeficiency virus (HIV)-infected humans, in contrast, can develop systemic disease that is more quickly lethal. This is in keeping with other evidence showing that susceptible humans generate some level of Th1 immunity to Mycobacterium tuberculosis (Mtb) infection. Tuberculosis in mice is also exclusively a lung disease that is progressive and lethal, in spite of the generation of Th1-mediated immunity. Thus mouse tuberculosis is a model of tuberculosis in susceptible humans, as is tuberculosis in guinea pigs and rabbits. Inability to resolve infection and prevent disease may not be a consequence of the generation of an inadequate number of Th1 cells but of an intrinsic deficiency in macrophage function that prevents these cells from expressing immunity. If this proves to be true, vaccinating susceptible humans against tuberculosis will be a difficult task.
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PMID:Immunity to tuberculosis. 1503 90

On 6-7 October 2002, the International Union Against Tuberculosis and Lung Disease (IUATLD), the International Pediatrics Association (IPA), the United States Centers for Disease Control and Prevention (CDC), the United States National Institutes for Health (NIH), and the World Health Organization (WHO) co-sponsored and organized a Workshop on 'Tuberculosis in Children' to assess research needs in childhood tuberculosis (TB) in conjunction with the 33rd IUATLD World Conference on Lung Health. Participants included approximately 40 researchers from the sponsoring organizations as well as from academic institutions, and National Tuberculosis (TB) Programs from a number of countries in sub-Saharan Africa, Asia, Latin America, and Eastern Europe. The goals of the workshop were to highlight current gaps in knowledge about childhood TB, to assess research opportunities, and to begin to establish working partnerships and identify funding sources. The workshop focused on six key topics: the global epidemiology of TB among children, clinical practice including diagnosis and case management of childhood TB, basic research, programmatic aspects of the control of TB among children, ethics, and effects of human immunodeficiency virus (HIV) on TB in children. Review papers on these topics were presented as the foundation for the workshop. Participants were then divided into groups to deliberate on critical areas of research and programmatic needs, recommendations for addressing the needs, and strategic planning to increase international focus on childhood TB. The following summary, including data reported and referenced within the review papers presented at the workshop, represents the proceedings of the workshop.
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PMID:New international efforts in childhood tuberculosis: proceedings from the 2002 Workshop on Childhood Tuberculosis, Montreal, Canada, 6-7 October 2002. 1513 47

Palivizumab (Synagi) is a humanized monoclonal antibody that provides immunoprophylaxis against serious lower respiratory tract infections (LRTIs) caused by respiratory syncytial virus (RSV). RSV is the leading cause of hospitalization for LRTIs in infants, causing winter- or wet-season epidemics. In two double-blind, placebo-controlled trials, intramuscular palivizumab 15 mg/kg every 30 days for 5 months significantly reduced RSV-related hospitalizations by 55% in 1502 infants with prematurity and/or bronchopulmonary dysplasia/chronic lung disease (BPD/CLD) and by 45% in 1287 infants with hemodynamically significant congenital heart disease (HSCHD). Reductions were statistically significant versus placebo in infants with BPD/CLD, with all degrees of prematurity, and with acyanotic/other heart disease. Palivizumab was generally well tolerated, with < or =1.9% of recipients discontinuing treatment for tolerability reasons. In placebo-controlled trials, the most common potentially drug-related adverse events were fever, nervousness, injection-site reactions, and diarrhea. Drug-related events occurred in 7.2-11% of palivizumab recipients in controlled trials (vs 6.9-10% with placebo) and 0-7.9% in open-label trials. Very few serious potentially drug-related adverse events occurred in clinical trials; four occurred in 2 of 285 patients in one open-label trial. No significant anti-palivizumab antibodies developed during palivizumab use. Palivizumab trough serum concentrations were below the recommended 40 microg/mL in about 33% and up to 14% of children prior to their second and third palivizumab injections. In pharmacoeconomic studies, the cost of palivizumab per hospitalization averted was generally lowest in the highest-risk infants. Drug cost was generally the most influential factor in sensitivity analyses. In conclusion, prophylaxis with palivizumab significantly reduces the incidence of RSV-related hospitalization relative to placebo and is generally well tolerated in high-risk infants aged <2 years, including those with prematurity and BPD/CLD or HSCHD, which are risk factors for early or serious RSV infection. Palivizumab is approved for use in these patients. Other high-risk infants in whom palivizumab has not been formally assessed, such as those with immunodeficiency, cystic fibrosis, or location-specific risk factors (including extended hospital stays) might potentially benefit from palivizumab. The use of palivizumab in these other high-risk populations is likely to be determined as much by pharmacoeconomic considerations as by efficacy outcomes.
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PMID:Palivizumab: a review of its use as prophylaxis for serious respiratory syncytial virus infection. 1517 Mar 64

Genetic deficiencies in the purine catabolic enzyme adenosine deaminase (ADA) in humans results primarily in a severe lymphopenia and immunodeficiency that can lead to the death of affected individuals early in life. The metabolic basis of the immunodeficiency is likely related to the sensitivity of lymphocytes to the accumulation of the ADA substrates adenosine and 2'-deoxyadenosine. Investigations using ADA-deficient mice have provided compelling evidence to support the hypothesis that T and B cells are sensitive to increased concentrations of 2'-deoxyadenosine that kill cells through mechanisms that involve the accumulation of dATP and the induction of apoptosis. In addition to effects on the developing immune system, ADA-deficient humans exhibit phenotypes in other physiological systems including the renal, neural, skeletal, and pulmonary systems. ADA-deficient mice develop similar abnormalities that are dependent on the accumulation of adenosine and 2'-deoxyadenosine. Detailed analysis of the pulmonary insufficiency seen in ADA-deficient mice suggests that the accumulation of adenosine in the lung can directly access cellular signaling pathways that lead to the development and exacerbation of chronic lung disease. The ability of adenosine to regulate aspects of chronic lung disease is likely mediated by specific interactions with adenosine receptor subtypes on key regulatory cells. Thus, the examination of ADA deficiency has identified the importance of purinergic signaling during lymphoid development and in the regulation of aspects of chronic lung disease.
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PMID:Adenosine deaminase deficiency: metabolic basis of immune deficiency and pulmonary inflammation. 1570 18

Congenital and neonatal viral infections usually display their acute manifestations in highly recognisable ways, for example, congenital rubella, cytomegalovirus (CMV), varicella, human immunodeficiency (HIV) and herpes simplex virus (HSV) infection. By contrast, congenital hepatitis B virus (HBV) infection may go undetected for years. Some of these are preventable, but what is not immediately apparent is that the long-term consequences are being prevented as well. The long-term consequences of congenital and neonatal infections include endocrine, immunological and cardiovascular disease, deafness, visual problems, intellectual handicap and cerebral palsy. With the survival of HIV-infected infants into adulthood the long-term consequences will soon be described. Maternally and neonatally transmitted HBV infection predisposes to carriage, liver cirrhosis and hepatocellular carcinoma in young adults. Neonatal HBV vaccination prevents adult cancer. Acquired viral infections may predispose to subsequent lung disease, malabsorption, fertility problems or neurological disability. In the prevention of acquired rubella, varicella, HBV, influenza, poliovirus, measles and hepatitis A, one should mention the added bonus of preventing secondary cases by preventing transmission from infants and children to other children and adults. Preventing paediatric HSV, HBV and HIV infection in females may even be preventing subsequent transmission to future generations. Turning to paediatric bacterial infections, vaccinating infants and young children against pertussis could not only prevent transmission to older children and adults but also break the cycle, which then transmits from adults back to infants and young children. There is evidence that disease in older age groups, including adults, has been prevented by virtue of herd immunity from paediatric vaccination, e.g. Neisseria meningitidis Group C and Streptococcus pneumoniae. The add-on benefits for other generations, including for adults, arising from the prevention of paediatric infections are considerable.
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PMID:Paediatric infections: prevention of transmission and disease--implications for adults. 1575 76

Bronchiectasis is associated with heterogeneous predisposing conditions that cause abnormal dilatation and persistent inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Recent investigations have focused on the inflammatory mediators involved in the pathogenesis of bronchiectasis. High-resolution computed tomography is now the diagnostic modality of choice and may also contribute to clinical management. Computed tomography and high-resolution computed tomography have identified bronchiectasis in individuals with HIV and alpha-1-antitrypsin deficiency. Early identification of predisposing disorders and aggressive management of symptoms has already been demonstrated by prolonged survival and decreased morbidity in cystic fibrosis patients, and similar management may benefit other populations with bronchiectasis. New treatments such as recombinant human DNase have been shown to improve pulmonary function and quality of life in cystic fibrosis patients and may prove useful in other chronic inflammatory lung disease.
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PMID:An update on bronchiectasis. 1578 1

Over the period, 1st October 1999 to 30th April 2002 a clinical trial of the modified short-course chemotherapy (SCC) in newly diagnosed cases of pulmonary tuberculosis with human immunodeficiency virus (HIV) infection in Ibadan, Nigeria was carried out. The modified SCC used was adopted by World Health Organisation (WHO)/International Union against Tuberculosis and Lung Diseases (IUALTD) for developing countries and also by the Nigerian National Tuberculosis and Leprosy Control Programmed (NTLCP). The regimen used consisted of ethambutol (E), isoniazid (H), rifampicin (R) and pyrazinamide (Z) in the intensive phase of 2 months. The continuation phase was 6 months of ethambutol (E) and isoniazid(H), i.e. 2EHRZ/6EH. Sputum conversion was 90% at the second month of treatment and there was no bacteriological relapse after 18 months of follow-up. Side effects were few and consisted mainly of acne vulgaris in 20 (22.5%) of 89 patients during the continuation phase. It is concluded that this modified 8-month chemotherapy regimen adopted by NTLCP is efficacious in treatment of smear positive pulmonary tuberculosis (PTB) patients with background HIV infection.
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PMID:Evaluation of modified short course chemotherapy in active pulmonary tuberculosis patients with human immunodeficiency virus infection in University College Hospital, Ibadan, Nigeria--a preliminary report. 1581 74


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