Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017168 (gastroesophageal reflux disease)
 11,783 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Wheezing in children is a common problem encountered by family physicians. Approximately 25 to 30 percent of infants will have at least one wheezing episode, and nearly one half of children have a history of wheezing by six years of age. The most common causes of wheezing in children include asthma, allergies, infections, gastroesophageal reflux disease, and obstructive sleep apnea. Less common causes include congenital abnormalities, foreign body aspiration, and cystic fibrosis. Historical data that help in the diagnosis include family history, age at onset, pattern of wheezing, seasonality, suddenness of onset, and association with feeding, cough, respiratory illnesses, and positional changes. A focused examination and targeted diagnostic testing guided by clinical suspicion also provide useful information. Children with recurrent wheezing or a single episode of unexplained wheezing that does not respond to bronchodilators should undergo chest radiography. Children whose history or physical examination findings suggest asthma should undergo diagnostic pulmonary function testing.
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PMID:The diagnosis of wheezing in children. 1848 58

The prevalence of hyponutrition in cystic fibrosis is high although it may vary according to the different studies. Detection of hyponutrition should be done by combining different methods, depending on their availability. However, the simplest and most validated criterion is to measure at each visit the weight (and height in children) in order to calculate the body mass index and categorizing hyponutrition according to absolute criteria: in adults < 18.5 kg/m(2), and in children as percentiles of the body mass index. Worsening of the nutritional status is directly related with the decrease in lung function parameters and it has been proposed as a morbidity (and even mortality) predictive factor in people with cystic fibrosis, independently of the level of pulmonary dysfunction. Exocrine pancreatic insufficiency is present is approximately 70-90% of the patients with cystic fibrosis and the genotype-phenotype correlation is high. Most of the patients with exocrine pancreatic insufficiency tolerate a high-fat diet provided that they are treated with pancreatic enzymes at appropriate doses. The prevalence of diabetes increases with age, reaching up 40% of the cases in patients older than 30 years. Clinical liver involvement is less prevalent (it approximately affects 1/3 of the patients). Other intestinal complications such as meconial ileus, gastroesophageal reflux, obstruction of the distal intestine, or fibrosing colon disease may also condition malnourishment. In patients with cystic fibrosis, a usual high-fat diet providing 120%-150% of the recommended calories is advised. If the nutritional goals are not achieved or maintained with diet modifications, artificial supplements may be added, although the recommendation for their use has not been endorsed by solid scientific evidences. The most frequently used preparations usually are polymeric or hypercaloric. The indications for enteral (through a tube, especially gastrostomy) or parenteral nutritional support are similar to those used in other pathologies. Dietary and nutritional control should be included in a multidisciplinary program allowing the improvement of the functional capacity and the quality of life and reducing, at least from a theoretical viewpoint, the morbimortality associated to malnourishment in these patients.
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PMID:[Nutrition, cystic fibrosis and the digestive tract]. 1871 14

Nontuberculous mycobacteria (NTM) are ubiquitous organisms with nearly 100 different species found in soil and water. The fatty-acid and wax-rich impermeable cell wall of the mycobacteria allow for adherence to solid substrates such as pipes and leaves, allowing the organism to persist despite treatment with common disinfectants. Mycobacteria can cause infection in both humans and animals. It is difficult to assess the incidence or prevalence of NTM disease due to multiple factors. Nontuberculous mycobacteria infection may be difficult to differentiate from colonization, and when NTM infection is diagnosed, it is not a reportable disease. Furthermore, some species such as Mycobacterium gordonae may be a contaminant. Nontuberculous mycobacteria infection is not a communicable disease, although health care-associated outbreaks have been reported, associated with a single facility or procedure. While the nontuberculous infection may affect other organs, the most common site is the lung, and the most common species is Mycobacterium avium complex, commonly referred to as MAC infection. An increasing occurrence of MAC has been reported, especially in certain populations such as middle-aged or elderly thin women, patients with chronic lung disease, human immunodeficiency virus infection, and cystic fibrosis. An association of NTM infection with gastroesophageal reflux disease has also been noted. The clinical presentation often includes chronic productive cough. Other less common symptoms include dyspnea and hemoptysis. With increased use of computed tomography and high-resolution computed tomography, patterns of MAC pulmonary infection have been described. Recently, the American Thoracic Society has outlined guidelines for the diagnosis and management of NTM infection. Treatment of NTM infection requires at least 3 effective drugs for a minimum of 12 months after sputum conversion to negative cultures. Surgical therapy may be considered for localized disease which has failed medical management. In this article, the clinical presentation, radiographic features, diagnostic evaluation, and management are discussed.
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PMID:Nontuberculous mycobacterial pulmonary infections in Non-HIV patients. 1902 Mar 69

Lung transplantation has become a viable option for those cystic fibrosis (CF) patients with end-stage lung disease. Despite the challenges that the CF patients present, the survival seen after lung transplantation is more favorable than seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. Although the CF patients with severe respiratory disease usually are infected with organisms that display in vitro resistance to the commonly used antibiotics, these patients usually have successful outcomes with transplantation. The other challenges include the presence of nontuberculous mycobacteria, the significant incidence of liver involvement, the development of an ileus or the development of the distal intestinal obstruction syndrome, and the presence of gastroesophageal reflux. Most of the patients have metabolic bone disease, even preoperatively, that warrants treatment, especially with the significant loss of bone density seen in the first year after transplant, thought to be related, in part, to the high dose of corticosteroids. Diabetes mellitus and its consequences are not uncommon. The malabsorption of fat seen in the pancreatic-insufficient patients complicates the absorption kinetics of the anti-rejection drugs. In May 2005 the United Network of Organ Sharing instituted a lung-allocation score to better distribute the donated lungs to those patients who would achieve the most benefit. This score uses several variables to balance the likelihood of the patients living one year with a transplant versus one year without a transplant. With this change in the allocation of organs, the median waiting times have significantly decreased, the mortality on the waiting list has decreased, and the number of CF patients transplanted has not changed. With substantial experience, more programs are now transplanting patients who require constant mechanical ventilation or patients who have undergone previous pleural procedures, especially in the treatment of a pneumothorax. The limiting factor now in lung transplantation is the number of organs available. Efforts to increase the donor pool, such as alveolar recruitment strategies to improve gas exchange, have been effective in allowing more patients to be transplanted. Lung transplantation is now an accepted form of therapy in those patients who are developing progressive respiratory failure.
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PMID:Lung transplantation in cystic fibrosis. 1946 64

Cystic fibrosis (CF) is a complex multisystem disorder affecting mainly the gastrointestinal tract and respiratory system. Intestinal malabsorption occurs in approximately 90% of patients. In the past, malnutrition was an inevitable consequence of disease progression, leading to poor growth, impaired respiratory muscle function, decreased exercise tolerance and immunological impairment. A positive association between body weight and height and survival has been widely reported. The energy requirements of patients with CF vary widely and generally increase with age and disease severity. For many young adults requirements will be 120-150% of the age-related estimated average requirement. To meet these energy needs patients are encouraged to eat a high-fat high-energy diet with appropriate pancreatic enzyme supplements. Many patients are unable to achieve an adequate intake as a result of a variety of factors including chronic poor appetite, infection-related anorexia, gastro-oesophageal reflux and abdominal pain. Oral energy supplements and enteral tube feeding are widely used. Nutritional support has been shown to improve nutritional status and stabilise or slow the rate of decline in lung function. With such emphasis on nutritional intake and nutritional status throughout life, poor adherence to therapies and issues relating to body image are emerging. The median survival of patients with CF is increasing. CF is now considered a life-limiting disease of adulthood rather than a terminal childhood illness. With increased longevity new challenges are emerging that include the transition of young adults with CF to adult services, CF-related diabetes, disordered eating, osteoporosis, liver disease and transplantation.
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PMID:Symposium 6: Young people, artificial nutrition and transitional care. The nutritional challenges of the young adult with cystic fibrosis: transition. 1969

This study aimed to determine the aetiology, clinical presentation, co-morbidity, severity and the lobar distribution of non cystic fibrosis bronchiectasis (NCFB). We performed a retrospective review of clinical, radiological, immunological and microbiological data from 92 non-CF patients with a High resolution thoracic CT (HRCT) diagnosis of bronchiectasis in the three Dublin Children's referral Hospitals for the period 1996-2006. Of 92 patients (50 female), the median age at diagnosis was 6.4 years. The aetiology of bronchiectasis was as follows; idiopathic 29 (32%), post-pneumonia 16 (17%), immune deficiency 15 (16%), recurrent aspiration 15 (16%), primary ciliary dyskinesia 8 (9%), chronic aspiration with immune deficiency 5 (5%), post foreign body inhalation 2 (2%), tracheomalacia 1 (1%) and Obliterative bronchiolitis 1 (1%). Bronchial asthma and gastroesophageal reflux disease (GORD) were concurrently present in 18 (20%) and 10 (11%) respectively. Left lower lobe was commonly involved followed next by the right middle lobe. The common isolates were Haemophilus influenza (50), Streptococcus pneumoniae (34) and Staphylococcus aureus (14), Moraxella catarrhalis (9) and Pseudomonas auerginosa (8). Surgical interventions were performed in 23 (25%) of patients, lobectomy 11 (12%), pneumectomy 2 (2%), laryngeal cleft repair 4 (5%), rigid bronchoscopy for foreign body removal 2 (2%), Nissan's fundoplication 2 (2%), tracheoesophageal fistula repair 2 (2%). We conclude NCFB is under-recognised in Irish children and diagnosis is often delayed and Bronchial Asthma and GORD are common co morbidity. A high index of suspicion and early HRCT can expedite the diagnosis.
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PMID:A decade of non-cystic fibrosis bronchiectasis 1996-2006. 2066 70

Reactive airway disease is often triggered by an upper respiratory viral infection and readily responds to anti-inflammatory and bronchodilator therapy. The differential diagnosis for unresponsive disease includes poorly controlled asthma, noncompliance with medical regimen, vocal cord dysfunction, rhinosinusitis, gastroesophageal reflux disease or recurrent aspiration, foreign body aspiration, allergic bronchopulmonary aspergillosis, Churg-Strauss vasculitis, cardiac disorders such as congestive heart failure or mitral stenosis, or other pulmonary disorders such as chronic obstructive pulmonary disease, alpha-1 antitrypsin deficiency, interstitial lung disease, bronchiectasis, sarcoidosis, hypersensitivity pneumonitis, pulmonary embolism, cystic fibrosis, airway neoplasms, or laryngotracheomalacia. As is often the case, a meticulous history can expeditiously direct the clinician to the diagnosis, especially in a patient without a smoking, asthmatic, or atopic history.
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PMID:A 41-year-old male with cough, wheeze, and dyspnea poorly responsive to asthma therapy. 2081 28

This paper aims to present our experience in the pharmacological approach of the management of azole antifungal drugs in cystic fibrosis lung transplant patients. Cystic fibrosis (CF) lung transplantation is associated with multi-factorial care management, because of immunosuppressive requirements, risk of infections, frequency of gastro-oesophageal reflux disease, hepatic alterations and CF pharmacokinetics (PK) specificities that result in important PK variability. CF is associated with frequent colonization of the airways by filamentous fungi, especially by Aspergillus species. Today the antifungal therapeutic arsenal offers several possibilities for long-term oral therapy including azole drugs (itraconazole, voriconazole and posaconazole). Therefore, nephrotoxic amphotericin B should be avoided. The liver is important in the pharmacological profile of azole drugs, due to metabolic elimination, hepatotoxicity and PK drug-drug interaction (DDI) involving CYP3A4 metabolic inhibition. Targets for such DDI are numerous, but immunosuppressive drugs are of major concern, justifying combined therapeutic drug monitoring (TDM) of both azoles (inhibitors) and immunosuppressants (targets) on an individualized patient basis to adjust the coprescription quantitatively. The risk of long under-dosed periods, frequently addressed in this population, could justify, on a PK basis, the need for combination with an exclusive parenteral antifungal while waiting for azole relevant drug level. High PK variability, the risk of low exposure, therapeutic issues and DDI management in this complex underlying disease justify close monitoring with systematic combined TDM of azole and immunosuppressants, in case of coprescription.
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PMID:Pharmacological considerations for azole antifungal drug management in cystic fibrosis lung transplant patients. 2106 31

Cystic Fibrosis (CF) is the most frequent recessive disease of Caucasian patients. Association with other diseases or syndromes has previously been reported. Co-morbidity may be a challenge for clinicians, who have to face more severe problems. We have described a CF infant, F508del homozygote, diagnosed by neonatal screening, who also had a chromosome 18q terminal deletion [del (18)(q22-qter)]. Some clinical features of the 18q deletion: e.g., cardiopathy, gastro-oesophageal reflux and severe muscular hypotonia, worsened the CF clinical picture and his quality of life, with repeated pulmonary exacerbations and failure to thrive in the first six months of life. The treatment strategy was chosen following an accurate multi-disciplinary team study of overlapping chromosome syndrome and CF symptoms. The use of a gastrostomy device for enteral nutrition together with a new device (Ez-PAP) for chest physiotherapy led to normal growth, a notably reduced hospitalization rate and improved quality of life. This case shows how co-morbidities worsening the clinical course of a "complicated patient" can be faced thanks to unconventional therapies that represent a challenge for clinicians.
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PMID:18q deletion in a cystic fibrosis infant, increased morbidity and challenge for correct treatment choices: a case report. 2158 41

Cystic fibrosis (CF) is an inherited disease that affects both the lungs and the digestive system in children and adults. Thick mucus fills the gut and blocks lumens of the pancreas and hepatobiliary systems, creating insufficient pancreas function and liver disease. Chronic gastrointestinal (GI) complications, including intestinal obstruction, occur in neonates, and poor digestion and gastroesophageal reflux disease (GERD) in children. Although GI symptoms tend to improve with age, CF and associated GERD eventually create respiratory insufficiency; the only available treatment option at this stage is a bilateral lung transplant, which carries considerable morbidity and mortality. While GERD may reoccur as a complication of lung transplantation, GERD symptoms are often reduced following a fundoplication.
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PMID:Gastroesophageal reflux in cystic fibrosis: current understandings of mechanisms and management. 2252 61


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