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Kartagener Syndrome (Primary Ciliary Dyskinesia)

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An autosomal recessive disorder characterized by a triad of DEXTROCARDIA; INFERTILITY; and SINUSITIS. The syndrome is caused by mutations of DYNEIN genes encoding motility proteins which are components of sperm tails, and CILIA in the respiratory and the reproductive tracts.

Also Known As:

Primary Ciliary Dyskinesia; Kartagener's Syndrome; Syndrome, Kartagener; Syndrome, Kartagener's; Kartagener's Triad; Polynesian Bronchiectasis; Siewert Syndrome; Bronchiectasis, Polynesian; Dyskinesia, Primary Ciliary; Kartageners Syndrome; Kartageners Triad; Polynesian Bronchiectases; Syndrome, Siewert; Triad, Kartagener; Triad, Kartagener's; Ciliary Dyskinesia, Primary; Kartagener Triad

Networked: 268 relevant articles (7 outcomes, 16 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cystic Fibrosis (Mucoviscidosis)
2.	Bronchiectasis
3.	Asthma (Bronchial Asthma)
4.	Chronic Obstructive Pulmonary Disease (COPD)
5.	Emphysema

Experts

1.	Omran, Heymut: 14 articles (10/2015 - 07/2005)
2.	Olbrich, Heike: 11 articles (10/2015 - 07/2005)
3.	Zariwala, Maimoona A: 10 articles (10/2014 - 07/2005)
4.	Knowles, Michael R: 10 articles (10/2014 - 07/2005)
5.	Mitchison, Hannah M: 10 articles (09/2014 - 08/2002)
6.	Chung, Eddie M K: 10 articles (09/2014 - 08/2002)
7.	Lucas, Jane S: 7 articles (01/2015 - 10/2012)
8.	Leigh, Margaret W: 7 articles (10/2014 - 12/2009)
9.	O'Callaghan, Christopher: 7 articles (09/2014 - 11/2008)
10.	Schmidts, Miriam: 7 articles (01/2014 - 12/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Kartagener Syndrome:

1.	Dyneins (Dynein)IBA 03/01/2000 - "The gold standard for the diagnosis of primary ciliary dyskinesia (PCD) is a dynein deficiency shown with transmission electron microscopy. " 06/01/1998 - "The results of this study show that patients with Kartagener's syndrome may have normal ciliary ultrastructure and the absence of dynein arms is not necessarily associated with ciliary immotility. " 04/01/1994 - "Ciliary motion analysis demonstrated a total absence of ciliary motion whereas, ultrastructural studies revealed typical features of primary ciliary dyskinesia (PCD) (absence or short outer/inner dynein arms in 90% of the cilia). " 01/01/1990 - "No abnormal ciliary ultrastructures specific to Kartagener's syndrome, such as absence of dynein arms, were detected in this study. " 10/01/2015 - "Loss-of-Function GAS8 Mutations Cause Primary Ciliary Dyskinesia and Disrupt the Nexin-Dynein Regulatory Complex."
2.	Nitric Oxide (Nitrogen Monoxide)FDA Link 02/01/2006 - "Nasal nitric oxide (NO) concentration is dramatically reduced in primary ciliary dyskinesia (PCD). " 12/01/2013 - "Several studies suggest that nasal nitric oxide (nNO) measurement could be a test for primary ciliary dyskinesia (PCD), but the procedure and interpretation have not been standardized. " 12/01/2011 - "The goal of the present work was to study nitric oxide synthases expression in upper airway tissues from patients with primary ciliary dyskinesia. " 02/01/2008 - "Several studies reported low nasal nitric oxide (nNO) levels in subjects with primary ciliary dyskinesia (PCD) and proposed nNO measurement as a diagnostic tool to screen for the disease. " 10/01/2015 - "Four patients (15.4%) had nitric oxide levels below the lower end of normal, with two (7.7%) having values below the cutoff for primary ciliary dyskinesia. "
3.	Colloids (Colloid)IBA 05/01/2005 - "A study was undertaken to assess the reliability of the nasal mucociliary transport test using 99mTc-albumin colloid as a screening test for primary ciliary dyskinesia (PCD) and to compare it with the gold standard nasal biopsy for study of ciliary motility and ultrastructure. " 05/01/2005 - "Mucociliary transport using 99mTc-albumin colloid: a reliable screening test for primary ciliary dyskinesia."
4.	Tissue Plasminogen Activator (Alteplase)FDA Link 01/01/2014 - "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. "
5.	Proteins (Proteins, Gene)IBA 03/01/2011 - "Genetic studies involving human patients and mouse models of primary ciliary dyskinesia over the last decade have uncovered a number of important ciliary proteins and have begun to elucidate the mechanisms underlying ciliary motility. " 05/01/2014 - "Mutations in radial spoke head proteins are a known cause of primary ciliary dyskinesia. " 01/01/2014 - "MCIDAS mutant respiratory epithelial cells carry only one or two cilia per cell, which lack ciliary motility-related proteins (DNAH5; CCDC39) as seen in primary ciliary dyskinesia. " 06/01/2013 - "Primary ciliary dyskinesia (PCD), resulting from defects in cilia assembly or motility, is caused by mutations in a number of genes encoding axonemal proteins. " 09/01/2009 - "In conclusion, RFX proteins not only regulate genes involved in ciliary assembly, but also genes that are involved in ciliary motility and that are associated with ciliopathies such as primary ciliary dyskinesia in humans."
6.	Nitric Oxide Synthase (NO Synthase)IBA 05/01/2008 - "Among other respiratory disorders, such as cystic fibrosis and primary ciliary dyskinesia, nNO levels are particularly low in the latter (possibly for the trapping and altered NO metabolism caused by dense secretions, and by decreased NO synthase activity) and suggest nNO as a good screening tool for such disease." 03/01/2011 - "No study has evaluated the correlation between different expression of nitric oxide synthase (NOS) isoforms in nasal epithelial cells and nasal NO (nNO) level in primary ciliary dyskinesia (PCD). " 12/01/2011 - "We therefore conclude that the drop in nasal nitric oxide in patients with primary ciliary dyskinesia is not secondary to the loss of nitric oxide synthase expression." 03/01/2011 - "Nasal nitric oxide and nitric oxide synthase expression in primary ciliary dyskinesia."
7.	Young SyndromeIBA 02/01/1997 - "[Clinical features and ultrastructure of primary ciliary dyskinesia and Young syndrome]." 02/01/1997 - "A study was conducted of the clinical manifestations and ultrastructure in a series of seven patients with repeated pulmonary infections and infertility (3 cases with primary ciliary dyskinesia [PCD] and 4 with the young syndrome [YS]). " 09/01/1996 - "Other conditions associated with nasal polyps are Churg-Strauss Syndrome, allergic fungal sinusitis, and cilia dyskinetic syndrome, (Kartagener's) and Young Syndrome. "
8.	human DNASE1 protein (dornase alfa)FDA Link 11/01/1995 - "The use of rhDNase should be considered in patients with Kartagener's syndrome and a multicenter trial may be justified." 02/22/2010 - "There are no controlled studies on rhDNase in primary ciliary dyskinesia or atelectasis."
9.	Protein Isoforms (Isoforms)IBA 03/01/2011 - "No study has evaluated the correlation between different expression of nitric oxide synthase (NOS) isoforms in nasal epithelial cells and nasal NO (nNO) level in primary ciliary dyskinesia (PCD). "
10.	Transforming Growth Factor beta (TGF-beta)IBA 10/01/2014 - "The aim of this study was to compare basement membrane (BM) thickness and the number of transforming growth factor beta 1 (TGF-β1) positive epithelial cells in children with asthma, cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and healthy controls. "

Therapies and Procedures

1.	Chest Wall Oscillation 06/01/2014 - "Comparison of conventional pulmonary rehabilitation and high-frequency chest wall oscillation in primary ciliary dyskinesia." 01/01/2008 - "High Frequency Chest Wall Oscillation (HFCWO) has been shown to be effective in helping to clear secretions from the lungs of patients with cystic fibrosis, bronchiectasis, asthma, primary ciliary dyskinesia, emphysema, COPD, and many others. "
2.	Analgesia 12/01/1998 - "[Epidural anesthesia and analgesia in the perioperative treatment of a patient with Kartagener syndrome]." 07/01/2004 - "Improved ventilatory function associated with relief of chest pain in a patient with Kartagener's syndrome treated by subcutaneous analgesia."
3.	Gamma Cameras (Gamma Camera) 04/01/2000 - "Patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) have been shown to have impaired large airway clearance of radiolabelled particles as measured by external gamma camera scanning up to 6 hours post deposition. "
4.	Lung Transplantation 07/01/2013 - "Bilateral lung transplantation was performed in a 52-year old man with end-stage Kartagener's syndrome. " 06/01/2001 - "Living-donor lobar lung transplantation for primary ciliary dyskinesia." 02/01/2000 - "Very few cases of lung transplantation have been described for patients with Kartagener's syndrome. " 09/01/1999 - "[Primary ciliary dyskinesia treated with living-donor lobar lung transplantation]." 07/01/1994 - "Lung transplantation in Kartagener's syndrome."
5.	Transplants (Transplant) 02/01/2000 - "[Sequential double lung transplant in Kartagener's syndrome]." 09/01/1999 - "We report a case of primary ciliary dyskinesia in which a living-donor lobar lung transplant was performed. " 08/05/2015 - "This mucociliary defense mechanism is essential for pulmonary health, because respiratory ciliary motion defects, such as those in patients with primary ciliary dyskinesia (PCD) or congenital heart disease, can cause severe sinopulmonary disease necessitating organ transplant. " 04/01/2004 - "We report the anaesthetic management of a patient with Kartagener's syndrome and postrenal transplant immunosuppression, presenting for repair of uterovaginal prolapse. " 02/01/1998 - "In children with primary ciliary dyskinesia, tympanoplasty has a high probability of graft success and auditory improvement, despite the frequent recurrence of serous otitis media."