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 single most important respiratory pathogen in infancy and early childhood is respiratory syncytial virus (RSV). Approximately 40% of primary RSV infections in children result in lower respiratory tract disease. Approximately 1% of RSV-infected children require hospitalization. Especially in high-risk children, primary RSV infection results in significant morbidity and, sometimes, death. This high-risk group includes children with bronchopulmonary dysplasia, children with congenital heart disease, premature infants less than 6 months of age, and children with immunodeficiency diseases. It has been estimated that, in the United States, 14,000 infants with chronic lung disease and 16,400 infants with heart disease will be identified by 12 months of age. More than 91,000 children are hospitalized annually with lower respiratory tract disease caused by RSV, and 4500 deaths occur. In 1985 a report from the Institute of Medicine calculated that the annual hospitalization costs attributable to RSV infection were $300 million. Data collected at the New England Medical Center in 1991 show that the average cost of hospitalization of a child with RSV was $808 each day. Because of difficulty in developing a safe and effective RSV vaccine, attention is now focused on passive immunization using an RSV immune globulin. On the basis of a recently completed multiinstitutional trial, RSV immune globulin appears to be a safe and cost-effective option for prevention of severe RSV disease in high-risk children.
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PMID:Economic impact of viral respiratory disease in children. 816 53

The photosensitizer, benzoporphyrin derivative monoacid ring A (BPD-MA) has been studied regarding its ability to destroy enveloped viruses in blood and blood products when activated by light. Its maximum wavelength of absorption (690 nm) has proven useful in terms of activation of the photosensitizer in materials containing red blood cells. Experiments conducted on whole blood of patients infected with the human immunodeficiency virus (HIV) demonstrated that BPD-MA and light could effectively eliminate the virus when treated materials were placed in culture and tested for viral p24, but did not appear to damage blood cells or blood components. Since HIV is largely intracellular in infected individuals, these results were investigated further. We have shown, using flow cytometry, that in HIV-infected blood, BPD-MA and light appear to selectively destroy white cells that bear the interleukin 2 receptor and the DR antigen. These markers are prevalent on activated lymphocytes, and since HIV replicates only in CD4+ T cells which are activated, this finding provides an explanation for the selective killing of HIV.
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PMID:Photosensitizers as virucidal agents. 845 22

Respiratory syncytial virus (RSV) infection, which primarily manifests as bronchiolitis or pneumonia, is the leading cause of lower respiratory tract infection in infants and young children. It is associated with more than 100,000 pediatric hospitalizations each year in the United States. Infants who were premature; have chronic lung disease, congenital heart disease, or immunodeficiency disorders; or have underlying metabolic or neuromuscular disorders are at increased risk for especially severe RSV disease. Treatment of children hospitalized with RSV disease is primarily supportive, with administration of supplemental oxygen and fluid replacement therapy. Bronchodilators may benefit at least a subset of such patients. Antiviral therapy with aerosolized ribavirin is available for high-risk, severely ill patients. Handwashing, cleaning of environmental surfaces, and cohorting in hospital settings may decrease RSV transmission. In children born premature and younger than 1 year of age, and in patients with bronchopulmonary dysplasia younger than 2 years of age, passive protection against severe RSV disease may be achieved through monthly injections of anti-RSV antibody (palivizumab) during winter months. No vaccine is available to provide active immunity against RSV, but live attenuated and subunit cloned surface protein vaccines are in development.
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PMID:Respiratory Syncytial Virus: Update on Infection, Treatment, and Prevention. 1138 54

(1) RSV infection, the main cause of bronchiolitis, can necessitate hospitalisation, especially of infants at risk, i.e. those with a history of prematurity or bronchodysplasia. No drug prevention has been available. (2) Palivizumab, a monoclonal antibody directed against respiratory syncytial virus (RSV), is now marketed for preventing respiratory tract infection by RSV in certain infants. (3) The evaluation dossier barely answers the questions raised by the use of this drug. (4) The results of six trials suggest that the optimal dose is 15 mg/kg palivizumab by monthly injection throughout the seasonal epidemic period. (5) A double-blind trial in 1 502 infants either aged less than 6 months and born prematurely (35 weeks of gestation or earlier), or aged under 2 years with a history of bronchopulmonary dysplasia, has shown that, relative to a placebo, palivizumab reduces the hospitalisation rates by 5% in absolute values. It does not influence mortality or the need for mechanical ventilation. (6) Given the lack of relevant trials, we do not know if palivizumab is effective in infants with immunodeficiency or congenital heart diseases. We do not know, either, whether the definition of groups at risk used in the only relevant trial is appropriate. (7) No serious adverse effects attributable to palivizumab were reported in clinical trials. (8) Treatment with palivizumab is costly.
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PMID:Palivizumab in prevention of bronchiolitis: new preparation. Moderate efficacy in some infants. 1147 94

Respiratory syncytial virus (RSV) is the most common pathogen of the lower respiratory tract in infants. Groups at risk for severe disease include preterm infants, infants with pulmonary disease such as bronchopulmonary dysplasia, infants with congenital heart disease, and infants suffering from immunodeficiency. However, most infants getting severely ill from RSV are otherwise healthy and born at term. The incidence of hospitalisation caused by RSV is increasing, and there is an association between diagnosed RSV infection and subsequent development of wheeze and asthma. No vaccine or causal therapy is available. However, prophylaxis with a humanized monoclonal antibody of murine origin, palivizumab, reduces the risk of hospitalisation in high-risk infants, but the treatment is expensive. The most important prophylaxis methods at the present time are therefore hygienic measures with the purpose of preventing nosocomial infection in hospitals.
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PMID:[Respiratory syncytial virus]. 1252 6

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

Chronic lung disease (CLD) in children represents a heterogeneous group of many distinct clinicopathological entities. The prevalence of CLD has increased in the past decade because of the more advanced and intensive respiratory support provided for compromised children and additionally the overall improved survival of preterm babies. The disorders which constitute CLD generally have a slow tempo of progression over many months or even years. The most common causes of CLD in children are cystic fibrosis (CF), and other causes of bronchiectasis (such as immunodeficiency, and in the third world, post-infective bronchiectasis, for example, measles), bronchopulmonary dysplasia (BPD) (or lung disease of prematurity), asthma, chronic gastro-oesophageal reflux/aspiration pneumonitis, and constrictive obliterative bronchiolitis.
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PMID:The radiology of chronic lung disease in children. 1590 25

RSV infection is a leading cause of lower respiratory tract infection, especially in High-risk infants with a history of prematurity, bronchopulmonary dysplasia (BPD), congenital heart disease (CHD), neuromusculair impairment, immunodeficiency, and Down syndrome. Host related risk factors that have been identified to be associated with severe RSV related lower respiratory tract infection include young age below 6 months at the beginning of RSV season, multiple birth, male sex, low socioeconomic status and parental education, crowded living conditions, young siblings, maternal smoking and indoor smoke pollution, malnutrition/small for gestational age, family history of atopy or asthma, low cord serum RSV antibody titers, and living at altitude.Risk factors increasing the risk of acquisition of RSV have been identified to be birth before and/or during RSV season, day care attendance, presence of older siblings in school or day-care, and lack of breast feeding. Some of these risk factors are discussed controversially and some of them are found continuously throughout the literature.Given the high cost of RSV prophylaxis, especially for the large population of late preterm infants, algorithms and risk score systems have been published that could identify high-risk infants for treatment with palivizumab out of this gestational age group. Several models reported on an average sensitivity and specificity of 70 percent and, thus, are helpful to identify infants at high risk for severe RSV infection and need for prophylaxis with palivizumab.
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PMID:Risk factors for severe respiratory syncytial virus lower respiratory tract infection. 2226 87

Regenerative therapy based on stem cells is applied as standard therapy in pediatric oncology. Furthermore, they are frequently used to treat immunodeficiency disorders of infants. For severe neonatal diseases, e. g. hypoxic-ischemic encephalopathy in term neonates or bronchopulmonary dysplasia in preterm infants, animal models have been established. According to some first preclinical results stem cell administration appears as a promising tool to improve the clinical outcome in high-risk infants. Provided the benefit of regenerative therapies can further be evaluated in appropriate preclinical neonate models, carefully controlled clinical trials to assess the significance of regenerative therapies, such as autologous stem cell administration, are indicated.
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PMID:Regenerative therapies in neonatology: clinical perspectives. 2281 28

Development of Pediatric Pulmonology as a speciality in India is steadily improving over past few decades. Present profile of Indian pediatric chest services include: asthma, recurrent infections, bronchiectasis, etc. It is expected to change and the emerging pulmonary illnesses include: human immunodeficiency virus (HIV infection) associated pulmonary illnesses, cystic fibrosis, primary ciliary dyskinesia, bronchopulmonary dysplasia, interstitial lung diseases, gastroesophageal reflux diseases, neuromuscular illnesses, sleep disorders, disorders due to malformations and opportunistic pulmonary infections. Respiratory infections constitute major load in pediatric outpatient services and are the leading cause of mortality in under-five children. To reduce morbidity and mortality due to respiratory tract infections, Indian Academy of Pediatrics (IAP) has developed Respiratory Tract Infection Group Education Module (RTIGEMS). After initial increase in prevalence of asthma, it seems to have stabilized now but going by the numbers, it will remain a major health problem in India. Diagnosis of pulmonary tuberculosis was always a challenge to pediatricians and with emergence of drug resistant tuberculosis, it is even more challenging. Presently few centers are providing specialized Pediatric pulmonology services in India. There is a need to develop more centers to enhance services including (a) assessment of pulmonary physiology by performing pulmonary function testing in all age groups, (b) improving diagnostic and therapeutic role of bronchoscopy and bronchoalveolar lavage, (c) sweat testing, (d) molecular diagnostics for various respiratory illnesses, and (e) utilizing advance imaging and minimally invasive technologies for diagnosis and treatment of respiratory illnesses. At present there is no degree course in Pediatric Pulmonology in India. Initially middle level pediatricians wanting to pursue their career in pediatric pulmonology should undergo training in existing centers. Trained persons should develop a network to collect data and answer relevant research questions.
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PMID:50 years of pediatric pulmonology, progress and future. 2339 81


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