Kartagener, Syndrome

Running head : KARTAGENER SYNDROME

Kartagener Syndrome

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Subject

Kartagener Syndrome

Introduction

Kartagener syndrome is a variety of primary ciliary dyskinesia , which is a hereditary dis of special cells identified as cilia . In 1904 Siewert initially gave a picture of the bronchiectasis , chronic sinusitis , and situs inversus combination . Nevertheless , in 1933 it was Manes Kartagener who first acknowledged the medical triad as a distinct hereditary syndrome (Bent , 2008 . It is inherited through an autosomal recessive pattern , and symptoms are brought about by the malfunctioning

of cilia motility . Kartagener syndrome affects both males and females and the rate of occurrences in the United States is one for every 32 ,000 live births (Bent , 2008 . Usually , for patients with primary ciliary dyskinesia half of them by chance develops the situs inversus as a result , for each patient with Kartagener syndrome , another patient has primarily ciliary dyskinesia , and not situs inversus

Causes

In general , Kartagener syndrome is caused by alteration in the axonemal dynein intermediary chain gene identified as DNAI1 , which is in chromosome region 9p21-p13 (MedicineNet .com , 2002 . Linkage studies however , have recorded the disease gene to 5p and 19q in a number of families , signifying that Kartagener syndrome is more than a single genetic unit

Symptoms

Clinical symptoms of Kartagener syndrome consist of chronic lower and upper respiratory tract disease ensuing from futile mucociliary clearance . A common manifestation is the mucopurulent and rhinorrhea discharges from the time of birth . Nearly all of the symptoms of Kartagener syndrome develop from the...