Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of pemphigus vulgaris in a six-year-old Japanese girl is presented. She first developed vesicles and ulcerations in oral and laryngeal mucous membranes, showing a hoarse voice and fits of coughing with excessive slavering. She had skin blisters six months later. Biopsy of the skin lesion demonstrated the intraepidermal blister in a suprabasal location. Direct immunofluorescence (IF) of the skin lesion revealed deposits of IgG and C3. Indirect IF showed serum anti-ICS antibody titer at 1:640. She was diagnosed as having pemphigus vulgaris. Treatment with oral prednisolone (1 mg/kg) proved effective. This is the first case of infantile pemphigus vulgaris in Japan.
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PMID:A case of pemphigus vulgaris in a six-year-old girl. 188 52

Primary ciliary dyskinesia (PCD) is a genetic disease characterized by abnormal ciliary structure and function and impaired mucociliary clearance. Because patients with PCD use cough clearance as an airway defense mechanism, we tested the hypothesis that aerosolized uridine-5'-triphosphate (UTP) would improve clearance during cough by its actions to stimulate Cl- secretion and mucin release by goblet cells. We measured clearance during cough in 12 patients with PCD (ages 14 to 71 yr, FEV1 43% to 89% predicted) in a double blind, randomized, crossover study after aerosolization of a single dose of UTP (5 mg/ml, 3.5 ml) or vehicle (0.12% saline, 3.5 ml). Clearance during cough (whole lung) was quantified during and after a series of controlled coughs by measuring the clearance of [99mTc]Fe2O3 particles via gamma camera scanning over 120 min. Safety parameters were recorded during and after drug delivery. Aerosolized UTP improved whole-lung clearance during cough as compared with vehicle (from 0 to 60 min: 0.40 +/- 0.07%/min [UTP] versus 0.26 +/- 0. 04%/min [vehicle] [mean +/- SEM], p = 0.01), and from 0 to 120 min: 0.38 +/- 0.05%/min [UTP] versus 0.25 +/- 0.04%/ min [vehicle], p = 0. 02). Aerosolized UTP is safe, with no serious adverse effects. Whole-lung clearance during cough in patients with defective ciliary function is enhanced after inhalation of UTP.
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PMID:Effect of aerosolized uridine-5'-triphosphate on airway clearance with cough in patients with primary ciliary dyskinesia. 1039 Mar 92

Airway secretions are cleared by mucociliary clearance (MCC), in addition to other mechanisms such as cough, peristalsis, two-phase gas-liquid flow and alveolar clearance. MCC comprises the cephalad movement of mucus caused by the cilia lining the conducting airways until it can be swallowed or expectorated. MCC is a very complex process in which many variables are involved, all of which may modify the final outcome. The structure, number, movement and co-ordination of the cilia present in the airways as well as the amount, composition and rheological properties of the periciliary and mucus layers are determinants of MCC. Physiological factors such as age, sex, posture, sleep and exercise are reported to influence MCC due to a change in the cilia, the mucus or the periciliary layer, or a combination of these. Environmental pollution is suspected to have a depressant effect on MCC dependent on different factors such as pollutant concentration and the duration of exposure. Most studies focus on sulphur dioxide, sulphuric acid, nitrogen dioxide and ozone. Tobacco smoke and hairspray have been noted to have a negative influence on MCC. Some diseases are known to affect MCC, mostly negatively. The underlying mechanism differs from one illness to another. Immotile cilia syndrome, asthma, bronchiectasis, chronic bronchitis, cystic fibrosis and some acute respiratory tract infections are among the most frequently reported. The present paper reviews normal mucociliary clearance and the effects of diseases on this process.
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PMID:Regulation of mucociliary clearance in health and disease. 1041 87

Primary ciliary dyskinesia is a recessively inherited group of disorders with abnormal ciliary activity leading to disturbed mucociliary clearance. Clinical manifestations as early as the first year of life are recurrent rhinitis, otitis media, sinusitis and lower respiratory tract infections. Another typical presentation is situs inversus. Biopsy of the ciliated mucosa in the nose or bronchi is required for study by vital microscopy and electronmicroscopy to confirm the diagnosis. Early diagnosis is important for initiation of rigorous treatment involving physiotherapy, inhalation with beta 2-mimetics and prompt antibiotic treatment to prevent irreversible damage such as bronchiectasis. Compared with cystic fibrosis the prognosis is better, because older children can compensate the absent mucociliary clearance with always-functioning cough clearance.
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PMID:[Clinical features of primary ciliary dyskinesia]. 1084 65

Primary ciliary dyskinesia (PCD) is an inherited condition characterised by functional and/or structural congenital abnormalities of cilia. Presentation is often in the neonatal period, but there are age-related differences in presentation, and diagnosis is often delayed. The usual clinical picture is of recurrent upper and lower respiratory symptoms (rhinitis, glue ear, recurrent cough and sputum production), with mirror image arrangement in 50% of the children. Around 50% males have immotile sperm, but male infertility is not invariable. There are known associations between PCD and complex congenital heart disease, severe oesophageal disease, and more rarely, hydrocephalus and biliary atresia. Diagnosis is with a combination of the saccharine test, nasal nitric oxide, ciliary beat frequency and electron microscopy. Patients should be followed up by specialists familiar with the different ways of managing the upper and lower airway complications.
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PMID:Primary ciliary dyskinesia. 1108 68

The ICS Standardisation Committee (Abrams et al, 2002) defines the symptom of stress urinary incontinence as the complaint of involuntary leakage on effort or exertion, or on sneezing or coughing. Genuine stress incontinence (now known as urodynamically proven stress incontinence) is the involuntary loss of urine occurring when, in the absence of a detrusor contraction, the intravesical pressure exceeds the maximal urethral pressure. These definitions tend to look at the urinary mechanism in isolation, and many clinicians now advocate that a more holistic view should be taken of this problem.
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PMID:Pelvic floor muscle exercises. 1259 90

Aims of this study were: to evaluate changes in lung function in wheezing children with detected MP and CP infection according to treatment; to measure the response to inhaled corticosteroids in children with significant wheezing who were selected as having a high risk of progressing into childhood asthma. 54 children were randomly assigned 2:1 into 2 groups-the main group (36 patients), in which inhaled corticosteroids were administered, and the control group (18 patients), without inhaled corticosteroids. Serum IgE levels were determined using the ELISA (reagents: IBL-Hamburg). Serologic studies were performed by the ELISA for IgM and IgG antibodies to MP and for IgG and IgA antibodies to CP (reagents: ImmunoLISA, Orgenics, Israel) on the Hiperion MRIII (USA). Pulmonary function testing was done with SpiroLab II (DEGO GmbH, Medizin-Elektronik, Germany). The patients of both groups were administered macrolides: azitromycin during five days. Patients of the first group received inhaled fluticasone propionate 125 mg twice daily. The parents were asked to record symptoms. Each symptom (wheezing, cough, and shortness of breath) were scored on a scale of 0 to 3--daily symptom score (DSS). Scores were calculated every 4 weeks for a total treatment period 16 weeks. The days within each period on which the DSS equalled zero were pointed as symptom free days (SFD). It had been shown an significant improvement in DSS, an increase in SFD and significant improvement of the lung functions following the treatment with inhaled fluticasone and macrolide in children with wheezing and documented MP or CP infection, compared to control group treated only with antibiotics. In conclusion, the use of ICS should be seriously considered in children with wheezing and the risk of persisting symptoms.
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PMID:Non-viral wheezing in preschool children: the effect of inhaled fluticasone on symptoms and lung function. 1657 35

Primary ciliary dyskinesia (PCD) is a genetic disease associated with defective ciliary structure and function. Chronic oto-sino-pulmonary infection is the most common clinical presentation. Patients should be monitored in centres with expertise in PCD. Regular respiratory monitoring consists of lung function tests, adapted to the patient's age, and cough swab or sputum cultures. Chest X-rays are insensitive but the use of high-resolution computed tomography should be considered carefully to avoid excessive radiation. Treatment of the chronic suppuration of the lower airways relies on antibiotics to which the isolated bacteria are sensitive, together with respiratory physiotherapy and exercise to clear excessive bronchial secretions. Bronchodilators and anti-inflammatory agents have no proven benefit and should be prescribed, if at all, on an individual basis. Treatment of the lower airways should always be in conjunction with that of the upper airways. Importantly in PCD, respiratory disease may be controlled with the use of early, adequate and aggressive management.
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PMID:Management of primary ciliary dyskinesia: the lower airways. 1941 Feb 2

Bronchiectasis, or the irreversible dilatation of bronchi, can present with a host of nonspecific clinical symptoms, including hemoptysis, cough, and hypoxia. The radiologist, then, can play an important role in its detection and characterization. Bronchiectasis must be differentiated from motion artifact and transient bronchial dilatation in acute lung disease. When diagnosed, a logical approach may allow for proper triage of the patient to prevent progression of disease. The radiologic approach usually begins with CT, which is fast and accurate. The diagnostic approach should be based on the mechanisms of development of bronchiectasis (bronchial wall damage, endobronchial obstruction, and traction) and the location. Once an endobronchial lesion or adjacent fibrosis is excluded, location of the abnormality can be used to help narrow the differential diagnosis. When the bronchiectasis is upper lobe predominant, CF should first be considered but occasionally MAC infection may present with this finding. When the bronchiectasis is mid-upper lobe, then ABPA or chronic hypersensitivity pneumonitis might lead the list of diagnoses. Lower lobe bronchiectasis is usually the sequela of recurrent infection and conditions that predispose to recurrent infections, including Mounier-Kuhn, hypogammaglobulinemia, PCD, and recurrent infections. By using this approach, the radiologist can remain an integral part of the pulmonary team.
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PMID:Bronchiectasis. 1924 57

Primary ciliary dyskinesia (PCD) is a rare genetic condition that causes impaired mucociliary clearance due to poorly functioning cilia. PCD is one disease manifestation of the many recently recognized associations with ciliary malfunction, referred to as "ciliopathies." Manifestations of PCD commonly begin in the neonatal period with cough, pneumonia, and chronic ear infections or effusions. Approximately half of the affected individuals have situs inversus totalis. The diagnosis is often made in later childhood or early adulthood, because symptoms mimic more common childhood illnesses and because the definitive diagnosis of PCD can be challenging. Treatment recommendations are largely based on therapies used for other conditions with impaired mucociliary clearance in the absence of evidence-based research specific for PCD. Early recognition and initiation of both otolaryngologic and pulmonary management might reduce potential long-term morbidities. The purpose of this article is to update primary care providers, allergists, and pediatric pulmonologists on recent advances in this interesting condition.
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PMID:Primary Ciliary Dyskinesia in Children: A Review for Pediatricians, Allergists, and Pediatric Pulmonologists. 2227 27


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