Gene/Protein Disease Symptom Drug Enzyme Compound
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 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pediatric pharmacotherapy is often challenging due to the paucity of available clinical data on the safety and efficacy of drugs that are commonly used in children. This quandary is even more prevalent in children with rare diseases. Although extrapolations for dosing and administration are often made from available adult data with similar disease states, this translation becomes even more problematic in rare pediatric diseases. Understanding of rare disease pathophysiology is typically poor, and few, if any, effective therapies have been studied and identified. One condition that illustrates these issues is plastic bronchitis, a rare, most often pediatric disease that is characterized by the production of obstructive bronchial airway casts. This illness primarily occurs in children with congenital heart disease, often after palliative surgery. Plastic bronchitis is a highly clinically relevant and therapeutically challenging problem with a high mortality rate, and, a generally accepted effective pharmacotherapy regimen has yet to be identified. Furthermore, the disease is ill defined, which makes timely identification and treatment of children with plastic bronchitis difficult. The pharmacotherapies currently used to manage this disease are largely anecdotal and vary between the use of macrolide antibiotics, mucolytics, bronchodilators, and inhaled fibrinolytics in a myriad of combinations. The purpose of this review is 2-fold: first, to highlight the dilemma of treating plastic bronchitis, and second, to bring attention to the continuing need for studies of drug therapies used in children so safe and effective drug regimens can be established, particularly for rare diseases.
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PMID:Pharmacotherapy challenges of Fontan-associated plastic bronchitis: a rare pediatric disease. 2368 15

Plastic bronchitis is a rare, life-threatening condition characterized by the formation of mucofibrinous casts within the pulmonary bronchi. In patients with congenital heart disease, it is most frequently observed in single ventricular anatomies after Fontan palliation. The pathophysiology of plastic bronchitis remains unknown, and a consistently effective treatment strategy has yet to be identified. We report two cases of plastic bronchitis in patients with Fontan physiology. The first was treated with Fontan conversion and, despite encouraging short-term results, experienced recurrence of cast formation seven months postoperatively. The second underwent cardiac transplantation and has been free of bronchial casts for over one year. In addition, we explore the similarities between plastic bronchitis and protein-losing enteropathy, considering theories of their pathophysiologic mechanisms and reports of mutually effective treatment strategies. We propose that bronchial cast formation may result from the confluence of genetic makeup, inflammation, and the Fontan physiology and conclude that further investigation into therapies directed at these factors is merited.
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PMID:Plastic bronchitis in patients with fontan physiology: review of the literature and preliminary experience with fontan conversion and cardiac transplantation. 2380 71

Gymnema sylvestre R. Br., one of the most important medicinal plants of the Asclepiadaceae family, is a herb distributed throughout the World, predominantly in tropical countries. The plant, widely used for the treatment of diabetes and as a diuretic in Indian proprietary medicines, possesses beneficial digestive, anti-inflammatory, hypoglycemic and anti-helmentic effects. Furthermore, it is believed to be useful in the treatment of dyspepsia, constipation, jaundice, hemorrhoids, cardiopathy, asthma, bronchitis and leucoderma. A literature survey revealed that some other notable pharmacological activities of the plant such as anti-obesity, hypolipidemic, antimicrobial, free radical scavenging and anti-inflammatory properties have been proven too. This paper aims to summarize the chemical and pharmacological reports on a large group of C-4 gem-dimethylated pentacyclic triterpenoids from Gymnema sylvestre.
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PMID:C-4 gem-dimethylated oleanes of Gymnema sylvestre and their pharmacological activities. 2430 85

Understanding patterns and identifying common clusters of chronic diseases may help policymakers, researchers, and clinicians to understand the needs of the care process better and potentially save both provider and patient time and cost. However, only limited research has been conducted in this area, and ambiguity remains as those limited previous studies used different approaches to identify common clusters and findings may vary with approaches. This study estimates the prevalence of common chronic diseases and examines co-occurrence of diseases using four approaches: (i) identification of the most occurring pairs and triplets of comorbid diseases; performing (ii) cluster analysis of diseases, (iii) principal component analysis, and (iv) latent class analysis. Data were collected using a questionnaire mailed to a cross-sectional sample of senior Australians, with 4574 responses. Eighty-two percent of respondents reported having at least one chronic disease and over 52% reported having at least two chronic diseases. Respondents suffering from any chronic diseases had an average of 2.4 comorbid diseases. Three defined groups of chronic diseases were identified: (i) asthma, bronchitis, arthritis, osteoporosis and depression; (ii) high blood pressure and diabetes; and (iii) cancer, with heart disease and stroke either making a separate group or "attaching" themselves to different groups in different analyses. The groups were largely consistent across the approaches. Stability and sensitivity analyses also supported the consistency of the groups. The consistency of the findings suggests there is co-occurrence of diseases beyond chance, and patterns of co-occurrence are important for clinicians, patients, policymakers and researchers. Further studies are needed to provide a strong evidence base to identify comorbid groups which would benefit from appropriate guidelines for the care and management of patients with particular disease clusters.
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PMID:Multimorbidity and comorbidity of chronic diseases among the senior Australians: prevalence and patterns. 2442 5

Plastic bronchitis is a rare complication of a variety of respiratory diseases and congenital heart disease surgery, particularly Fontan procedure. Bronchial casts with rubber-like consistency develop acutely and may cause severe life-threatening respiratory distress. The management of plastic bronchitis is yet not well defined. Early intermittent, self-administered nebulization of tissue plasminogen activator was found to be effective in preventing deterioration of acute respiratory symptoms in a patient with primary ciliary dyskinesia and recurrent cast formation. Further investigation into new therapeutic strategies for this devastating disease is advocated.
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PMID:Management of plastic bronchitis with nebulized tissue plasminogen activator: another brick in the wall. 2452 76

We report the case of a five-year-old girl with plastic bronchitis after repaired complex congenital heart disease, who became asymptomatic after a short course of Augmentin. We report the disease regression as response either to antibiotic or as coincidental with spontaneous resolution.
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PMID:Spontaneous resolution of plastic bronchitis in a patient post hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of great arteries after course of Augmentin. 2554 22

The recent decline in cardiovascular disease mortality in Western countries has been linked with changes in life style and treatment. This study considers periods of decline before effective medical interventions or knowledge about risk factors. Trends in annual age-standardized death rates from cerebrovascular disease, heart disease and circulatory disease, and all cardiovascular disease are reviewed for three phases, 1881-1916, 1920-1939, and 1940-2000. There was a consistent decline in the cerebrovascular disease death rate between 1891 and 2000, apart from brief increases after the two world wars. The heart disease and circulatory disease death rate was declining between 1891 and 1910 before cigarette smoking became prevalent. The early peak in cardiovascular mortality in 1891 coincided with an influenza pandemic and a peak in the death rate from bronchitis, pneumonia and influenza. There is also correspondence between short-term fluctuations in the death rates from these respiratory diseases and cardiovascular disease. This evidence of ecological association is consistent with the findings of many studies that seasonal influenza can trigger acute myocardial infarction and episodes of respiratory infection are followed by increased risk of cardiovascular events. Vaccination studies could provide more definitive evidence of the role in cardiovascular disease and mortality of influenza, other viruses, and common bacterial agents of respiratory infection.
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PMID:Long-term trends in cardiovascular disease mortality and association with respiratory disease. 2624 37

Plastic bronchitis is a rare disorder, characterized by formation of thick fibrinous bronchial casts which can obstruct the airway and present as a life threatening emergency (1). It is more common in the pediatric population after corrective or palliative surgery for congenital heart disease like fontan procedure but has rarely been reported in adults as well (1). Pregnancy is a relative contraindication for bronchoscopy. Bronchoscopy in the pregnant patient poses significant risks as manipulation of the airway can lead to impaired oxygenation and ventilation. In addition, the drugs used during this procedure to provide sedation can have a direct impact on the developing fetus (2). In spite of these risks bronchoscopy should not be withheld in an emergent situation as it can be a lifesaving measure. We report a case of successful bronchoscopy using Propofol as the sedating agent in a pregnant female with plastic bronchitis who presented with respiratory distress.
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PMID:Successful bronchoscopy in a pregnant patient with plastic bronchitis. 2705 88

An adolescent with plastic bronchitis due to congenital heart disease had altered mental status after an interventional lymphatic procedure in which lipiodol contrast was used. Neuroimaging revealed cerebral lipiodol embolization due to direct shunting between lymphatic channels and pulmonary veins. Cerebral lipiodol embolization is a potential neurologic morbidity associated with interventional lymphatic procedures.
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PMID:Cerebral Lipiodol Embolism after Lymphatic Embolization for Plastic Bronchitis. 2729 8

Cancer is now the leading cause of death in Japan. A rapid increase in cancer mortality is expected as Japan is facing a super-aged society. Many causes of cancer are known to be closely linked to life style factors, such as smoking, drinking, and diet. The oral environment is known to be involved in the pathogenesis and development of various diseases such as bronchitis, pneumonia, diabetes, heart disease, and dementia. Because the oral cavity acts as the bodily entrance for air and food, it is constantly exposed to foreign substances, including bacteria and viruses. A large number of bacteria are endemic to the oral cavity, and indigenous oral flora act to prevent the settlement of foreign bacteria. The oral environment is influenced by local factors, including dental plaque, tartar, teeth alignment, occlusion, an incompatible prosthesis, and bad lifestyle habits, and systemic factors, including smoking, consumption of alcohol, irregular lifestyle and eating habits, obesity, stress, hormones, and heredity. It has recently been revealed that the oral environment is associated with cancer. In particular, commensal bacteria in the oral cavity are involved in the development of cancer. Moreover, Candida, human papilloma virus and Epstein-Barr virus as well as commensal bacteria have been reported to be associated with the pathogenesis of cancer. In this review, we introduce recent findings of the correlation between the oral environment and cancer.
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PMID:Oral environment and cancer. 2748


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