High Prevalence of Respiratory Ciliary Dysfunction in Congenital Heart Disease Patients with Heterotaxy Running title: Nakhleh et al.; Ciliary dysfunction in heterotaxy patients
نویسندگان
چکیده
Nader Nakhleh, DO; Richard Francis, PhD; Rachel A. Giese, BS; Xin Tian, PhD, You Li, PhD; Maimoona A. Zariwala, PhD; Hisato Yagi, PhD; Omar Khalifa, MD; Safina Kureshi, MD; Bishwanath Chatterjee, PhD; Steven L. Sabol, PhD; Matthew Swisher, MD; Patricia S. Connelly, MS; Mathew P. Daniels, PhD; Ashok Srinivasan, PhD; Karen Kuehl, MD, MPH ; Nadav Kravitz; Kimberlie Burns, BS; Iman Sami, MD; Heymut Omran, MD; Michael Barmada, PhD; Kenneth Olivier, MD, MPH; Kunal K. Chawla, BS; Margaret Leigh, MD; Richard Jonas, MD; Michael Knowles, MD; Linda Leatherbury, MD; Cecilia W. Lo, PhD
منابع مشابه
High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy.
BACKGROUND Patients with congenital heart disease (CHD) and heterotaxy show high postsurgical morbidity/mortality, with some developing respiratory complications. Although this finding is often attributed to the CHD, airway clearance and left-right patterning both require motile cilia function. Thus, airway ciliary dysfunction (CD) similar to that of primary ciliary dyskinesia (PCD) may contrib...
متن کاملGenetics High Prevalence of Respiratory Ciliary Dysfunction in Congenital Heart Disease Patients With Heterotaxy
Background—Patients with congenital heart disease (CHD) and heterotaxy show high postsurgical morbidity/mortality, with some developing respiratory complications. Although this finding is often attributed to the CHD, airway clearance and left-right patterning both require motile cilia function. Thus, airway ciliary dysfunction (CD) similar to that of primary ciliary dyskinesia (PCD) may contrib...
متن کاملAirway ciliary dysfunction and respiratory symptoms in patients with transposition of the great arteries
BACKGROUND Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findi...
متن کاملCongenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia.
BACKGROUND Primary ciliary dyskinesia (PCD) is a recessive genetic disorder that is characterized by sinopulmonary disease and reflects abnormal ciliary structure and function. Situs inversus totalis occurs in approximately 50% of PCD patients (Kartagener's syndrome in PCD), and there are a few reports of PCD with heterotaxy (situs ambiguus), such as cardiovascular anomalies. Advances in diagno...
متن کاملDNAH6 and Its Interactions with PCD Genes in Heterotaxy and Primary Ciliary Dyskinesia.
Heterotaxy, a birth defect involving left-right patterning defects, and primary ciliary dyskinesia (PCD), a sinopulmonary disease with dyskinetic/immotile cilia in the airway are seemingly disparate diseases. However, they have an overlapping genetic etiology involving mutations in cilia genes, a reflection of the common requirement for motile cilia in left-right patterning and airway clearance...
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