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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The frequency and nature of adverse events (AEs) are important safety endpoints in clinical trials of therapies for cystic fibrosis (CF) subjects, yet published tables of background AE rates in the CF population are not readily available. Our objective in this study was to produce tables of respiratory AE rates for placebo subjects (pediatric and adult) for inhaled therapy trials in CF subjects. Respiratory AE rates in inhaled therapy trials were computed by combining data on placebo subjects from early-phase dosing studies and middle/late-phase studies, where placebo consisted of 4 or 5 mL of inhaled saline solution. AE rates were computed as number of events divided by number of placebo-subject days of observation, and 95% confidence intervals were computed based on a Poisson model. AEs were categorized as both broad (e.g., respiratory, reactive airway disease) and specific (e.g., cough, chest tightness, hemoptysis). In short-term studies, respiratory AE rates (95% confidence interval) were 1.1(0.7, 1.6)/person-week and 1.0(0.7, 1.4)/person-week in pediatric and adult subjects, respectively. In long-term studies, respiratory AE rates were 1.7(1.6, 1.8)/person-month and 2.2(2.1, 2.3)/person-month in pediatric and adult subjects, respectively. Stepwise Poisson models were fit to determine if baseline covariates were important in predicting AE rates. Forced expiratory volume in one second (FEV(1)) percent of predicted and age in short-term studies, and FEV(1) percent predicted and gender in long-term studies were statistically important in predicting respiratory AE rates. Although these variables were statistically significant, the models' predictive abilities were low, with adjusted R(2)'s of 0.06 and 0.12 in the short- and long-term studies, respectively. Combining placebo-subject AE data recorded from multiple CF clinical trials yields better estimates of true rates of occurrence in the CF population. The tables published from this study can be used to assist those charged with safety monitoring in CF clinical trials.
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PMID:Respiratory adverse event profiles in cystic fibrosis placebo subjects in short- and long-term inhaled therapy trials. 1687 84

Restless leg syndrome (RLS) and periodic limb movement disorder (PLMD) are considered to be a continuum of a neurological sleep disorder associated with abnormal iron metabolism or deficiency. I describe a case of RLS and PLMD in a cystic fibrosis patient with iron deficiency from chronic hemoptysis. This is the first case that reports RLS and PLMD manifesting from iron deficiency caused by chronic hemoptysis in advanced cystic fibrosis lung disease.
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PMID:Restless leg syndrome manifested by iron deficiency from chronic hemoptysis in cystic fibrosis. 1698 19

Multiple cases with various types of pediatric malabsorption syndromes were evaluated. The clinical manifestations, laboratory findings, pathophysiology, and histopathological descriptions of each patient were analyzed in an effort to clear the pathogenesis of the malabsorption syndromes and the treatments were undertaken. The cases studied, included one patient with cystic fibrosis, two with lactose intolerance with lactosuria (Durand type), one with primary intestinal lymphangiectasia, two with familial hypobetalipoproteinemia, one with Hartnup disease, one with congenital chroride diarrhea, one with acrodermatitis enteropathica, one with intestinal nodular lymphoid hyperplasia (NLH), five with intractable diarrhea of early infancy and four with glycogenosis type Ia. Each case description and outcome is described below: 1. A 15-year-old Japanese boy with cystic fibrosis presented with severe symptoms, including pancreatic insufficiency, bronchiectasis, pneumothorax and hemoptysis. His prognosis was poor. Analysis of the CFTR genes of this patient revealed a homozygous large deletion from intron 16 to 17b. 2. In the sibling case of Durand type lactose intolerance, the subjects'disaccaridase activity of the small bowel, including lactase, were within normal limits. The results of per oral and per intraduodenal lactose tolerance tests confirmed lactosuria in both. These observations suggested, not only an abnormal gastric condition, but also duodenal and intestinal mucosal abnormal permeability of lactose. 3. In the case of primary intestinal lymphangiectasia, the subject had a lymphedematous right arm and hand, a grossly coarsened mucosal pattern of the upper gastrointestinal tract (identified via radiologic examination) and the presence of lymphangiectasia (confirmed via duodenal mucosal biopsy). The major laboratory findings were hypoalbuminemia, decreased immunoglobulin levels and lymphopenia resulting from loss of lymph fluid and protein into the gastro-intestinal tract. 4. In two cases of heterozygous familial hypobetalipoproteinemia, serum total cholesterol and betalipoprotein levels were very low. The subjects presented with symptoms and signs of acanthocytosis and fat malabsorption. Further, one subject had neurological abnormalities such as mental retardation and severe convulsions. Treatment with MCT formula diet corrected the lipid malabsorption. 5. A 5-year-old girl presented with pellagra-like rashes, mental retardation and cerebellar ataxia. An oral tryptophan (Trp) and dipeptide (Trp-Phe) loading test were conducted and the renal clearance of amino acids was also evaluated in this patient and in controls. Following the oral Trp loading test, plasma levels of Trp indicated a lower peak in the case, reaching a maximum at 60 minutes. On the other hand, the oral dipeptide (Trp-Phe) loading test in the Hartnup patient showed the peak Trp plasma level was the same as the control subjects. The renal clearance of neutral amino acids in this case increased to levels 5 to 35 times normal. 6. In the case of congenital chloride diarrhea, the subject had secondary lactose intolerance, dehydration, hyponatremia, hypokalemia, hypochloremia, hyperreninemia and metabolic alkalosis. The chloride content of her fecal fluid was very high. The concentrations were 89-103 mEq/l. In contrast, her urine was chloride-free. The subject's growth and development improved after treatment with lactose free formura and oral replacement of the fecal loses of water, NaCl and KCl. Unfortunately, the patient died of a small bowel intussusception. The kidney histopathological finding was juxtaglomerular hyperplasia by a necropsy. 7. In the case of acrodermatitis enteropathica, the subject had characteristic skin lesions, low serum zinc levels and ALPase activity. An oral ZnSO4 loading test and intestinal mucosal histology by a peroral biopsy were conducted. The serum zinc peak level was 2 hours after the oral ZnSO4 loading test. Infant formula alone could not maintain normal serum zinc ranges. Light microscopic studies of the intestinal villous architecture showed a normal pattern. However, ultrastructual examination of several epithelial cells revealed numerous intracellular vesicles. After zinc therapy, these changes were decreased. The lesions were postulated as the secondary result of zinc deficiency. 8. A 12-year-old girl presented with hypogammaglobulinemia, recurrent infections, chronic diarrhea and intestinal NLH. A barium meal and follow-through examination showed multiple nodules throughout the stomach and intestine. The nodules, all uniform in size, were 2 mm diameter. The barium enema did not show NLH in the colon. Mucosal biopsy of the stomach and jejunum revealed the typical histology of NLH in the lamina propria. Also, achlorhydria was present in this patient and her serum gastrin levels were very high; 315-775 pg/ml. 9. In 4 cases of intractable diarrhea in early infancy (by Avery G B), a jejunal biopsy showed shortening villi and nonspecific enterocolitis. Some patients were found with only low lactase or low lactase and sucrase levels. An electron microscope analysis of the small bowel in 2 cases showed alterations: increased pinocytosis in microvillus membranes and lysosomes by endocytosis of undigested macromolecular substances. I postulated that the stated evidence was causative of this clinical profile. 10. I frequently observed diarrhea as a clinical manifestation in glycogenosis type Ia and lipid malabsorption in one case. The light and electron photomicrographs showed intestinal absorption cells with the glycogen deposits in the inferior devision of nuclei.
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PMID:[Clinical studies of pediatric malabsorption syndromes]. 1722 86

The authors reviewed adult cystic fibrosis patients followed in the Pulmonology Unit from 1994-2004 (n=8), five female and three male, aged 20-34 years old (median= 27 years). Patients were diagnosed at 18 months - 31 years old by sweat testing (positive in six patients) and genotyping (four patients homozygous for Delta F508 mutation). Respiratory involvement was characterised by sinusitis and bronchiectasis. Pulmonary involvement was accompanied by functional abnormalities and gas exchange impairment in the majority of the patients. Bronchial tree was colonised permanently in five patients: Pseudomonas aeruginosa in four and Staphilococcus aureus in four (three patients affected by both agents simultaneously). The main causes of exacerbation were respiratory infections and haemoptysis. Non-respiratory involvement was variable. Four patients had digestive involvement (one with hepatic cirrhosis), one had renal failure and only one had a sperm count to document infertility. Four patients had osteopaenia. Treatment included chest physiotherapy, bronchodilators, dornase alfa, mucolytics, digestive enzymes, vitamins, antibiotics and oxygen therapy. At review, one had left follow-up, one had died, one was awaiting lung transplant and the others evidenced no difference in clinical characteristics. In this group of patients the severity of the pulmonary disease was not related to a late diagnosis. It can be explained by the diversity of cystic fibrosis presentation in adults.
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PMID:[Cystic fibrosis in adults]. 1763 73

Hemoptysis or pneumothorax that recurs with the onset of menses is strongly suggestive of thoracic endometriosis syndrome (TES). TES is a rare disorder, with relatively few cases reported in the literature. A 32-year-old woman with cystic fibrosis, who over a period of several months had experienced recurrent catamenial hemoptysis and pneumothoraces, including an episode of life-threatening hemoptysis that coincided with menstruation, is presented. Thoracic computed tomography and magnetic resonance imaging scans, as well as a bronchoscopic evaluation that demonstrated endobronchial lesions that disappeared after menses, support the diagnosis of TES in the present patient. The patient was treated empirically with danazol and subsequently underwent a successful double-lung transplantation. Danazol was discontinued postoperatively, and she was started on an oral contraceptive. Eighteen months post-transplant, she has not experienced a recurrence of her catamenial symptoms, despite having resumed a regular menstrual cycle.
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PMID:Catamenial hemoptysis and pneumothorax in a patient with cystic fibrosis. 1770 46

Cystic fibrosis (CF) has a variety of pulmonary manifestations that include pneumonia, pulmonary abscess, bronchiectasis, hemoptysis, and pneumothorax. Although newer therapies have greatly improved survival of patients with CF, surgical interventions for definitive treatment of these sequelae are often required. Several reports have illustrated that, with the current advances in the perioperative treatment and care of CF patients, surgical interventions for these pulmonary manifestations may be performed safely, resulting in a greatly improved quality of life. Also, although improvements in lung transplantation offer new hope for definitive treatment of those patients with cystic fibrosis, special considerations regarding other surgical issues, such as the prevalence of gastroesophageal reflux, need to be addressed.
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PMID:Pulmonary surgery in cystic fibrosis. 1815 43

Early diagnosis, treatments of acute exacerbations, and chronic therapies have all improved the lifespan of cystic fibrosis patients; however, the natural history remains one of worsening bronchiectasis and obstructive airways impairment. The progression of disease leads to eventual respiratory failure, but some will have other acute respiratory complications that require intervention. In this report, we review the most common life-threatening respiratory complications of cystic fibrosis, including pneumothorax, massive hemoptysis, and respiratory failure.
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PMID:Pulmonary complications in adult patients with cystic fibrosis. 1819 85

Haemoptysis varies in significance from trivial to fatal. The most common causes in children are tuberculosis, fungal infections and bronchiectasis of any cause. In populations of European origin, cystic fibrosis is the most common predisposing factor. Chronic or life-threatening haemoptysis requires further investigation. Various forms of treatment are possible, and management is optimized when a multidisciplinary team is available. Bronchial artery embolization (BAE) is effective in controlling haemoptysis in most cases, although recurrent bleeding is not uncommon. BAE is often technically challenging, however, and requires angiographic skills that are not always available in children's hospitals. Although the procedure is usually regarded as relatively safe, complications are not uncommon and may be severe or even fatal.
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PMID:Haemoptysis and bronchial artery embolization in children. 1851 69

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

Earlier diagnosis, treatment of exacerbations, and the use of long-term therapies have all improved the lifespan of patients with cystic fibrosis (CF). However, the natural history of CF airways disease remains one of worsening bronchiectasis and obstructive airways impairment. The progression of airways disease leads to eventual respiratory failure, but some will suffer other acute respiratory complications that require intervention, including pneumothorax, massive hemoptysis, and respiratory failure. Here I discuss the pathophysiology of these complications and the patient-related and treatment-related factors associated with their occurrence. Knowledge of these associations may play great importance in treatment decisions regarding the care of the patient and the respiratory therapist should be aware of the implications. Since disease severity is associated with all 3 conditions, aggressive treatment of the underlying condition is imperative, which includes the performance of airway-clearance therapies. Though some might argue that airway-clearance therapies might aggravate or even precipitate complications such as hemoptysis and pneumothorax, others will defend that there are airway-clearance therapies that might be safely performed. Aerosolized medications such as dornase alfa and tobramycin have been associated with a lower incidence of massive hemoptysis and are recommended therapies for patients with advanced airways disease, yet they are also associated with a higher incidence of pneumothorax, which suggests careful assessment of their potential bronchospastic effect in patients with advanced airways disease. The respiratory therapist also plays a key role in the care of the patient with respiratory failure. Here is also discussed the role of ventilatory support and airway-clearance therapies in the patient with advanced stage disease. Now, more than ever, the patient needs caregivers with the knowledge and sensitivity to provide appropriate palliative care.
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PMID:Pulmonary complications of cystic fibrosis. 1939 6


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