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Topic: Kartagener syndrome


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In the News (Mon 28 Dec 09)

  
  Kartagener Syndrome Information on Healthline
Kartagener syndrome is also known as the Siewert syndrome, after another physician, Siewert, who described the syndrome in the early 1900s.
Kartagener syndrome is caused by abnormalities of the cilia that line the respiratory tract and also form the flagella of sperm.
One theory behind the association of situs inversus with the underlying cause of Kartagener syndrome is that the lack of ciliary movement in the developing embryo may result in incorrect organ rotation in approximately 50% of affected individuals.
www.healthline.com /galecontent/kartagener-syndrome   (770 words)

  
 Manes Kartagener (www.whonamedit.com)
Hereditary syndrome with a triad of: situs inversus (transposition) of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis, and immobility of the cilia.
Kartagener was educated at the classical gymnasium in Przemysl, and later continued at a gymnasium for the natural sciences in Lemberg, where he graduated in 1915.
Kartagener obtained his doctorate in 1928 for a thesis on the thyroid gland and received further academic acknowledgement in 1935 for a dissertation on the aetiology of bronchiectasis.
www.whonamedit.com /doctor.cfm/625.html   (310 words)

  
 UAB Radiology Detail Page
Kartagener's syndrome may also be seen in connection with situs inversus.
Kartagener's syndrome represents a triad of situs inversus (seen in 50% of cases), sinusitis, and bronchiectasis.
In this syndrome, the spleen is absent, both lungs have three lobes, and the left and right lobes of the liver are similar.
www.rad.uab.edu:591 /tf/FMPro?-db=teachfile.fmp&-format=browse_record_detail.htm&-lay=webtf&-op=eq&Category=Vascular&ImagesPresent=1&-max=2147483647&-recid=32&-find=   (344 words)

  
 Kartagener syndrome -
Primary ciliary dyskinesia (PCD), also known as immotile ciliary syndrome, is a rare autosomal recessive genetic disorder caused by a defect in the action of cilia lining the respiratory tract.
Although the true incidence of the disease is unknown, it is estimated to be 1 in kartagener syndrome 32,000 or higher.
Sometimes the term is used broadly to include injuries, disabilities, syndromes, symptoms, deviant behaviors, and atypical variations of structure and function, while in other contexts these may be considered distinguishable categories.
www.medicalgeo.com /Med-Diseases-I---K/Kartagener-syndrome.html   (342 words)

  
 eMedicine - Kartagener Syndrome : Article Excerpt by: John P Bent III, MD
However, Manes Kartagener first recognized this clinical triad as a distinct congenital syndrome in 1933.
Kartagener syndrome (KS) is inherited via an autosomal recessive pattern.
In 1977, Eliasson and coworkers used the descriptive phrase immotile cilia syndrome to characterize male patients with sterility and chronic respiratory infections.
www.emedicine.com /med/byname/kartagener-syndrome.htm   (650 words)

  
 GDMCC - Genetic Diseases of Mucociliary Clearance Consortium
Kartagener syndrome is primary ciliary dyskinesia associated with situs inversus totalis, a medical term for the reversed, mirror-image orientation of the inner organs.
Although Kartagener initially described several patients who presented with situs inversus totalis, chronic sinusitis and bronchiectasis, also known as the Kartagener triad, situs inversus is present in only half of the cases of this syndrome.
The shortening or absence of dynein arms (inner, outer, or both) is the most common form of the syndrome, occurring in ninety percent of cases with ultrastructural defects, but other structural abnormalities (like absent radial spokes) can produce decreased or absent ciliary movement.
rarediseasesnetwork.epi.usf.edu /gdmcc/learnmore/faqs.htm   (1086 words)

  
 Kartagener's Syndrome: cause, treatments, symptoms, prevention, risks, complications, long-term outlook
Kartagener's Syndrome is a chronic lung disease that occurs when cilia are unable to move.
Kartagener's Syndrome is a rare genetic birth defect.
Kartagener's Syndrome is also called Primary Ciliary Dyskinesia, and Immotile Cilia Syndrome, Afzelius’ syndrome, Kartagener’s triad, Zivert's syndrome, and Zivert-Kartagener triad.
www.mamashealth.com /lung/kart.asp   (226 words)

  
 Primary Ciliary Dyskinesia
Kartagener, in 1933, was the first to recognize a clinical syndrome of situs inversus totalis, bronchiectasis, and chronic sinusitis.
Kartagener's syndrome, the triad of situs inversus, bronchiectasis, and chronic sinusitis, therefore has a prevalence of roughly 1 in 32,000 births.
It is important to note that situs inversus occurs without Kartagener's syndrome; only 1/4 to 1/5 of patients with situs inversus also have bronchiectasis and sinusitis.
www.bcm.edu /oto/grand/33095.html   (2954 words)

  
 McGraw-Hill Professional Books
The immotile cilia syndrome is a genetically determined disorder characterized by dysmotility or even complete immotility of the cilia in the airways and elsewhere.
Kartagener syndrome is a subgroup of the immotile cilia syndrome and is further characterized by situs inversus viscerum.
The clinical consequences of the immotile cilia syndrome include chronic cough and expectoration, bronchiectasis, chronic rhinitis and nasal polyposis, chronic or recurrent sinusitis, and often an agenesis of the frontal sinuses.
books.mcgraw-hill.com /getommbid.php?isbn=0071459960&template=ommbid&c=187   (385 words)

  
 Kartagener's Syndrome - Patient UK
Synonyms: Afzelius' syndrome, Kartagener's triad, Siewert's syndrome, Dextrocardia-bronchiectasis-
This is described as autosomal recessive inherited syndrome, although the genetic mechanisms involved in the expression of the various features require further elucidation.
Efforts to standardise the clinical criteria for the diagnosis of Kartagener's Syndrome have centred on dextrocardia, a ciliary beat frequency of less than 10 Hz/s, and a mean cross-section dynein arm count of less than 2.
www.patient.co.uk /showdoc/40001398   (1099 words)

  
 Expert Advice -- Kartagener Syndrome
The reason these cilia do not work properly in those with Kartagener syndrome is that a key component of the cilia is either missing or defective.
Kartagener syndrome is actually a subset of a larger group of diseases called primary cilia dyskinesia.
Those with the heart in normal position are simply categorized as having primary cilia dyskinesia syndrome while those with abnormally placed hearts have true Kartagener syndrome.
parenting.ivillage.com /tp/tphealth/0,,hfcg,00.html   (632 words)

  
 Health Information - Immotile Cilia Syndrome
Immotile Cilia Syndrome (or Primary Ciliary Dyskinesia or Kartagener's Syndrome) is a chronic lung disease which occurs when the cilia are unable to move.
Much of the treatment of Immotile Cilia Syndrome is based on the treatment of Cystic Fibrosis, which is commoner (1 in 2,000 persons) and has some similar features.
When patients with Immotile Cilia Syndrome have the heart on the right side, they are said to have Kartagener's Syndrome.
www.cheo.on.ca /english/9301a.shtml   (1001 words)

  
 ABCNews.com : Message Boards   (Site not responding. Last check: 2007-11-03)
They did not mention Kartagener syndrome, the one disease consistently associated with situs inversus and gave the mistaken impression that situs issues, outside of rare cardiac defects, are benign.
If you have Kartagener syndrome, it is important that you find a doctor familiar with the syndrome (look for doctors who diagnose and treat primary ciliary dyskinesia or PCD) because contrary to what you've been told, Kartagener syndrome, if not treated aggressively, is associated with a shortened life span.
PCD and its variant, Kartagener syndrome, account for close to 30% of all cases of organ reversal or misplacement, yet the condition was not even mentioned in this report.
forums.go.com /abcnews/Entertainment/messages?username=mmanion07   (1145 words)

  
 Ask an Expert: Transposed organs and A Lack of Silia
Kartagener syndrome is part of a larger group of diseases called primary cilia dyskinesia.
Kartagener syndrome is inherited as an autosomal recessive disorder.
If both parents are carriers for Kartagener syndrome – they would both be normal, but they have a 25% chance to both pass the gene causing Kartagener syndrome on at the same time.
www.netwellness.org /question.cfm/27758.htm   (600 words)

  
 Kartagener Syndrome - Talk Medical
The syndrome is inherited in an autosomal recessive manner.
Kartagener syndrome is usually due to mutation in the gene called DNAI1 (axonemal dynein intermediate chain) which is in chromosome region 9p21-p13.
However, linkage studies have mapped the disease gene to 5p and 19q in some families, indicating that Kartagener syndrome is more than one genetic entity.
www.talkmedical.com /medical-dictionary/7937/Kartagener-Syndrome   (110 words)

  
 eMedicine - Kartagener Syndrome : Article by John P Bent III, MD
Consideration should be given to this imaging technique early in the presentation of PCD syndromes, when a chest radiograph may not be sensitive enough to identify disease processes or when another differential is being considered.
Rayner CF, Rutman A, Dewar A: Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome.
Schidlow DV: Primary ciliary dyskinesia (the immotile cilia syndrome).
www.emedicine.com /med/topic1220.htm   (3744 words)

  
 EURORAD
Due to failure to thrive and unimproved respiratory distress with hypoxic polycythaemia, the patient was deemed candidate for surgery and underwent left intercostal thoracotomy: a hugely overinflated middle lobe projecting to the right hemithorax and a collapsed right upper lobe were found.
Dyskinetic cilia syndrome is a rare genetic disorder (1 new case over 30.000 new borns) in which ciliary motility is abnormal and mucociliary transport is impaired.
Fifty per cent of patients affected by dyskinetic cilia syndrome present with additional situs viscerum inversus and are classified as Kartagener's syndrome.
www.eurorad.org /eurorad/case.php?id=700   (512 words)

  
 Barclay's Protomorphosis Syndrome - Memory Alpha, the Star Trek Wiki
Barclay's Protomorphosis Syndrome is an artificial disease, accidentally created by Dr. Beverly Crusher aboard the USS Enterprise-D in 2370.
Attempting to cure Lieutenant Reginald Barclay of Urodelan flu, Dr. Crusher used a synthetic T-cell to activate the gene that would fight off the infection.
The syndrome was cured by using amniotic fluid to create a retrovirus to eliminate the effects.
memory-alpha.org /en/wiki/Barclay's_Protomorphosis_Syndrome   (331 words)

  
 Acute Respiratory Distress Syndrome: symptoms, treatment, cause, prevention, complications, risks, long-term outlook
Acute respiratory distress syndrome is a life-threatening condition in which inflammation of the lungs and accumulation of fluid in the air sacs leads to low blood oxygen levels.
When acute respiratory distress syndrome occurs, blood and fluid leak into the spaces between the air sacs, and eventually into the air sacs themselves, causing major breathing difficulties.
Acute respiratory distress syndrome can be caused by any disease that directly or indirectly injures the lungs.
www.mamashealth.com /lung/ards.asp   (265 words)

  
 Rare Pediatric Disease Database   (Site not responding. Last check: 2007-11-03)
Kartagener Syndrome (KS) is an inherited disorder of defective ciliary movement.
Provides an overview of PCD and Kartagener Syndrome and has links to related websites.
Bent J, Olearczyk M, “Kartagener Syndrome,” emedicine, www.emedicine.com/med/topic1220.htm, 2002.
www.madisonsfoundation.org /content/3/1/display.asp?did=216   (1297 words)

  
 Medical Dictionary: Kartagener syndrome - WrongDiagnosis.com
Kartagener syndrome is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH).
This means that Kartagener syndrome, or a subtype of Kartagener syndrome, affects less than 200,000 people in the US population.
Terms that may be interchangeable with Kartagener syndrome:
www.wrongdiagnosis.com /medical/kartagener_syndrome.htm   (268 words)

  
 Primary Ciliary Dyskinesia (PCD) - American Lung Association site
It is estimated that the prevalence of PCD is approximately 1 in 16,000 live births.
Kartagener's Syndrome is a variant of PCD that results from a combination of chronic sinusitis, bronchiectasis and situs inversus, where some of the patient's organs are reversed.
The reversed organs do not usually pose a problem for the patient.  About 50 percent of patients with PCD have Kartagener's Syndrome.
www.lungusa.org /site/pp.asp?c=dvLUK9O0E&b=35690   (545 words)

  
 Kartagener syndrome   (Site not responding. Last check: 2007-11-03)
Kartagener's syndrome is a very rare genetic disorder inherited as an autosomal recessive trait.
Additional cardiac malformations such as atrial septal defect, hypoplasia of the vascular tree of the right lung, or transposition of the great arteries have been reported.
However, detection and early management of patients with Kartagener's syndrome is important.
www.orpha.net /static/GB/kartagener_syndrome.html   (204 words)

  
 Situs Inversus Dextrocardia
It is usually caused by acquired disease of the lungs, the membrane around the lungs (pleura), or diaphragm.
Kartagener Syndrome is a combination of Dextrocardia with chronic dilatation of the bronchi, difficulty breathing, recurrent respiratory infection (bronchiectasis) and infection of the sinuses (sinusitis).
Clubbed fingers and bluish discoloration of the skin (cyanosis) may also be present.
www.laughs.com.au /sid   (1241 words)

  
 Primary ciliary dyskinesia
Primary ciliary dyskinesia (PCD) is a heterogeneous group of disorders characterised by chronic bronchorrhea with bronchiectasis and chronic sinusitis.
Its association with situs inversus occurs in 50% of the cases and constitutes the Kartagener syndrome (KS).
In the case of Kartagener syndrome, the situs inversus is most often total, although it can be partial in some cases (isolated dextrocardia or isolated transposition of abdominal viscerae).
www.orpha.net /static/GB/primary_ciliary_dyskinesia.html   (528 words)

  
 Medical Information Resources for Patients of Kartagener Syndrome
Kartagener Syndrome articles from PubMed of the National Library of Medicine.
"I would like those who have Kartagener's syndrome with the variation of dextrocardia, partial or total situs inversus, infertility, and other symptoms a place where we can talk to unite as a group.
"This is a place for anyone who has been diagnosed with "Primary Ciliary Dyskinesia (PCD) or Kartagener's Syndrome (KS)or Immotile Cilia Syndrome (ICS)" or who has had their life touched by someone with PCD or KS or ICS...a place to gather for support and information.
www.medconsumer.info /topics/Kartagener.htm   (953 words)

  
 Kartagener's syndrome (www.whonamedit.com)
Hereditary syndrome comprising triad of: situs inversus (transposition) of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis, and immobility of the cilia.
Kartagener reported a group of cases in 1933.
More on Kartagener's syndrome and the contributions of Afzelius and A.K. Siewert.
www.whonamedit.com /synd.cfm/880.html   (288 words)

  
 Can a person with Kartagener's Syndrome marry? - Miscellaneous - DoctorNDTV
I have a distant relative having Kartagener's Syndrome but is living a normal life.
You must be aware that people with Kartageners syndrome are usually infertile and some of them can have fairly severe sinusitis & bronchiectasis.
This should certainly be disclosed to the future spouse and advice taken by the couple from a genetics expert.
www.doctorndtv.com /FAQ/detailfaq.asp?id=6537   (92 words)

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