Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0043167 (pertussis)
 19,595 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Recurrent respiratory tract infections are responsible for about 85% of all diseases in childhood. Early diagnosis helps to prevent infections and start the appropriate treatment, what in turn prevents lungs from irreversible damage. The aim of this study was the analysis of possible causes of recurrent respiratory tract infections in children in Lodz region. We analyzed cases of 6335 children with recurrent respiratory tract infections, age 3 months to 17 years, referred to the clinic in our hospital by family doctors from 2000 to 2002. Among all children, 41.5% were diagnosed with allergy, 26.9% of patients had persistent Mycoplasma pneumoniae and 1.4% Bordetella pertussis infection. Very disturbing was the fact of very late cystic fibrosis diagnose at the age of 7,11 and 16. Congenital immune disorders were diagnosed late in five children at the age of 6,7,8,9,11, what delayed appropriate therapy and early prevention of infections.
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PMID:[Analysis of possible causes of recurrent respiratory tract infections in children from Lodz, Poland]. 1458 30

The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is a cAMP-dependent Cl(-) channel that is defective in CF disease. CFTR activity has been shown to be regulated by the G(q)/phospholipase C-linked P2Y2 subtype of P2Y nucleotide receptors (P2YR) in various systems. Here, we tested whether other P2YR may exert a regulation on CFTR activity and whether CFTR may in turn exert a regulation on P2YR signaling. Using reverse transcriptase-polymerase chain reactions, antisense oligodeoxynucleotide knockdown, and measurements of intracellular calcium concentration ([Ca(2+)](i)), we showed that, in addition to P2Y2R, Chinese hamster ovary (CHO) cells also express functional P2Y1R. P2Y1R were activated by 2-methylthioadenosine 5'-diphosphate > 2-methylthioadenosine-5'-triphosphate > ADP with an EC(50) of 30 nM, 0.2 microM, and 0.8 microM, respectively. Activation of P2Y1R increased [Ca(2+)](i), which was prevented by the P2Y1R antagonists pyridoxalphosphate-6-azophenyl-2',4'-disulfonic acid (PPADS) (10 microM) and N6-methyl 2'-deoxyadenosine 3',5'-bisphosphate (MRS2179) (10 microM) and by pretreatment with P2Y1R antisense oligodeoxynucleotides. In CHO-K1 and CHO-KNUT (mock-transfected) cells lacking CFTR, both P2Y1R and P2Y2R caused [Ca(2+)](i) mobilization via pertussis toxin (PTX)-insensitive G(q/11)-proteins. In contrast, in CFTR-expressing CHO cells (CHO-BQ1), the P2Y1R response was completely PTX-sensitive, indicating that P2Y1R couples to G(i/o)-proteins, whereas the P2Y2R response remained PTX-insensitive. In CHO-BQ1 cells, P2Y1R activation by ADP (100 microM) failed to inhibit both forskolin (1 microM)-induced CFTR activation, measured using iodide ((125)I) efflux, and forskolin (0.1-10 microM)-evoked cAMP increase. Together, our results indicate that, in contrast to P2Y2R, P2Y1R does not modulate CFTR activity in CHO cells and that CFTR expression may alter the G-protein-coupling selectivity of P2Y1R.
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PMID:Pharmacological and signaling properties of endogenous P2Y1 receptors in cystic fibrosis transmembrane conductance regulator-expressing Chinese hamster ovary cells. 1474 36

Bronchiectasis is becoming less common as the treatment for acute lower respiratory tract infections improves and immunization programmes decrease the frequency of pertussis and measles. However bronchiectasis is still a challenge to the paediatric chest physicians in many developing parts of the world and it remains a frequent problem being the final common pathway of several different lower respiratory tract insults such as cystic fibrosis, immunodeficiency, ciliary dyskinesia. Although the treatment of patients with bronchiectasis is primarily medical, surgical treatment is required in a small group of patients with recurrent episodes of pneumonia and atelectasis localized to one area, severe or recurrent hemoptysis and in those unresponsive to aggressive medical treatment with abnormal growth and development. There are unanswered questions about childhood bronchiectasis, mainly on aetiology and treatment which require more research.
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PMID:Non cystic fibrosis bronchiectasis. 1626 48

Ingestion of a salty meal induces secretion of guanylin (GN) and uroguanylin (UGN) into the intestinal lumen, where they inhibit Na+ absorption and induce Cl-, HCO3-, and water secretion. Simultaneously, these hormones stimulate renal electrolyte excretion by inducing natriuresis, kaliuresis, and diuresis. GN and UGN therefore participate in the prevention of hypernatremia and hypervolemia after salty meals. The signaling pathway of GN and UGN in the intestine is well known. They activate enterocytes via guanylate cyclase C (GC-C), which leads to cGMP-dependent inhibition of Na+/H+ exchange and activation of the cystic fibrosis transmembrane regulator. In GC-C-deficient mice, GN and UGN still produce renal natriuresis, kaliuresis, and diuresis, suggesting different signaling pathways in the kidney compared with the intestine. Signaling pathways for GN and UGN in the kidney differ along the various nephron segments. In proximal tubule cells, a cGMP- and GC-C-dependent signaling was demonstrated for both peptides. In addition, UGN activates a pertussis toxin-sensitive G-protein-coupled receptor. A similar dual signaling pathway is also known for atrial natriuretic peptide. Recently, a cGMP-independent signaling pathway for GN and UGN was also shown in principal cells of the human and mouse cortical collecting duct. Because GN and UGN activate different signaling pathways in specific organs and even within the kidney, this review focuses on more recent findings on cellular effects and signaling mechanisms of these peptides and their pathophysiologic implications in the intestine and the kidney.
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PMID:Cellular effects of guanylin and uroguanylin. 1638 16

Tissue injury enhances homing and engraftment of mesenchymal stem cells (MSCs). However, the mechanisms by which MSCs sense the signals released by injured tissues and migrate toward injury sites have not been fully defined. In the current report, we investigated whether human MSCs express the N-formyl peptide receptor (FPR) and the formyl peptide receptor-like-1 (FPRL1). These receptors bind to N-formylated peptides by which phagocytes migrate to inflammatory sites and fibroblasts repopulate wounds to remodel the damaged tissues. Reverse-transcription polymerase chain reaction (PCR) demonstrated that MSCs express both FPR and FPRL1 at the transcriptional level. Flow cytometric analyses revealed expression of both receptors at the protein level. Fusion of the enhanced green fluorescence protein (eGFP) to the C terminus of each receptor showed localization to the cell surface. Moreover, MSCs responded to stimulation by N-formyl methionyl leucyl phenylalanine (fMLP), a prototypic N-formyl peptide, demonstrating rapid intracellular calcium mobilization that can be blocked by pertussis toxin or cyclosporin H. It is noteworthy that the fMLP-stimulated MSCs had an enhanced adhesion to extracellular matrix protein-coated surfaces. In addition, MSCs migrated toward gradients of increasing fMLP concentration, indicating that the receptors were functionally involved in positive chemotaxis to formylated peptides. Therefore, the N-formyl peptide receptors present in MSCs may play an important role in signaling stem cell adhesion, migration, and homing to injured and inflamed tissue for repair. Such a mechanism could potentially be exploited to direct the stem cells to target specific tissue sites, such as cystic fibrosis lungs, for therapy. Disclosure of potential conflicts of interest is found at the end of this article.
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PMID:Functional expression of N-formyl peptide receptors in human bone marrow-derived mesenchymal stem cells. 1723 90

In this paper we present an interesting case of cystic fibrosis patient with rare genotype de12,3/2184insA and atypical clinical image including: mild symptoms in an early phase of disease, quick progress of lung disease, complicated with pneumothorax after Bordetella pertussis infection and very good response to systemic and inhaled steroid therapy.
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PMID:Rare genotype del2,3/2184insA in a cystic fibrosis patient. 1737 46

Although asthma is the most common cause of cough, wheeze, and dyspnea in children and adults, asthma is often attributed inappropriately to symptoms from other causes. Cough that is misdiagnosed as asthma can occur with pertussis, cystic fibrosis, primary ciliary dyskinesia, airway abnormalities such as tracheomalacia and bronchomalacia, chronic purulent or suppurative bronchitis in young children, and habit-cough syndrome. The respiratory sounds that occur with the upper airway obstruction caused by the various manifestations of the vocal cord dysfunction syndrome or the less common exercise-induced laryngomalacia are often mischaracterized as wheezing and attributed to asthma. The perception of dyspnea is a prominent symptom of hyperventilation attacks. This can occur in those with or without asthma, and patients with asthma may not readily distinguish the perceived dyspnea of a hyperventilation attack from the acute airway obstruction of asthma. Dyspnea on exertion, in the absence of other symptoms of asthma or an unequivocal response to albuterol, is most likely a result of other causes. Most common is the dyspnea associated with normal exercise limitation, but causes of dyspnea on exertion can include other physiologic abnormalities including exercise-induced vocal cord dysfunction, exercise-induced laryngomalacia, exercise-induced hyperventilation, and exercise-induced supraventricular tachycardia. A careful history, attention to the nature of the respiratory sounds that are present, spirometry, exercise testing, and blood-gas measurement provide useful data to sort out the various causes and avoid inappropriate treatment of these pseudo-asthma clinical manifestations.
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PMID:Pseudo-asthma: when cough, wheezing, and dyspnea are not asthma. 1816 77

F508del is the most common cystic fibrosis-causing mutation that induces early degradation and poor trafficking of cystic fibrosis transmembrane conductance regulator (CFTR) chloride channels to the apical membrane of epithelial cells. Our previous work in bronchial serous cells showed that vasoactive intestinal peptide (VIP) stimulation of the VPAC(1) receptor enhances CFTR-dependent chloride secretion by increasing its membrane insertion by a protein kinase C (PKC)-dependent pathway. In the present study, we investigated the effect of VIP on F508del-CFTR activity and membrane insertion in the human nasal epithelial cell line JME/CF15, which also expresses the VPAC(1) receptor. At reduced temperature (27 degrees C), which rescues F508del-CFTR trafficking, acute stimulation by VIP of rescued F508del-CFTR channels was protein kinase A (PKA)- and PKC-dependent. One hour of treatment with VIP strongly increased F508del-CFTR activity, with iodide efflux peaks three times higher than with untreated cells. At 37 degrees C, VIP-treated cells, but not untreated controls, showed significant iodide efflux peaks that were sensitive to the CFTR inhibitor 3-[(3-trifluoromethyl)phenyl]-5-[(4-carboxyphenyl)methylene]-2-thioxo-4-thiazolidinone (CFTR(inh)-172). Immunostaining, biotinylation assays, and Western blots confirmed a VIP-induced maturation and membrane insertion of F508del-CFTR at 37 degrees C. The corrector effect of VIP was abolished by the PKA inhibitor N-[2-(p-bromocinnamylamino)ethyl]-5-isoquinolinesulfonamidedihydrochloride (H89), whereas Galpha(s) stimulation by cholera toxin significantly increased F508del-CFTR trafficking. On the other hand, membrane localization, but not maturation, of F508del-CFTR was significantly reduced by the PKC inhibitor bisindolylmaleimide X and the G(i/o) protein inhibitor pertussis toxin. VIP treatment had no effect on intracellular calcium or proteasome activity. These results indicate that, in human nasal cells, VIP rescues trafficking and membrane insertion of functional F508del-CFTR channels at physiological temperature by stimulating both PKA- and PKC-dependent pathways.
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PMID:Rescue of functional F508del cystic fibrosis transmembrane conductance regulator by vasoactive intestinal peptide in the human nasal epithelial cell line JME/CF15. 1958 7

Since children with chronic diseases represent a primary target for immunization strategies, it is important that their immunization coverage and timeliness of vaccines is optimal. We performed a study to measure immunization coverage and timeliness of vaccines in children with type 1 diabetes, HIV infection, Down syndrome, cystic fibrosis, and neurological diseases. A total of 275 children aged 6 months-18 years were included in the study. Coverage for diphtheria-tetanus-pertussis (DTP), polio (Pol), and hepatitis B (HBV) vaccines approximated 85% at 24 months, while measles-mumps-rubella (MMR) coverage was 62%. Immunization coverage for seasonal influenza was 59%. The analysis of timeliness revealed that there was heterogeneity among children with different chronic diseases. A proportional hazard model showed that children with HIV infection had the longest time to complete three doses of DTP, Pol, and HBV, and those with neurological diseases received the first dose of MMR later than the other categories. Causes of missing or delayed vaccination mostly included a concurrent acute disease. Children with chronic diseases should be strictly monitored for routine and recommended vaccinations, and health care providers and families should be properly informed to avoid false contraindications.
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PMID:Immunization coverage and timeliness of vaccination in Italian children with chronic diseases. 2141 80

Despite vaccination, pertussis is still endemic in the Netherlands. A literature search was performed to verify what is known about the role of Bordetella species in children with cystic fibrosis, with regard to the incidence of Bordetella infections, the involvement in pulmonary exacerbations and the influence on chronic course. Little is known about the frequency of Bordetella infections and the involvement of Bordetella species both in relation to the chronic course of cystic fibrosis and to pulmonary exacerbations. Since it is difficult to detect Bordetella species in cultures and few sputum cultures investigated have been obtained during an exacerbation, it is likely that the frequency of Bordetella species in CF patients is underestimated. Identification of Bordetella species in these patients may have serious consequences for the treatment of exacerbations in CF. Future research investigating the role of Bordetella species in cystic fibrosis should use specific techniques to detect Bordetella in cultures.
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PMID:Bordetella species in children with cystic fibrosis: what do we know? The role in acute exacerbations and chronic course. 2171 61


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