Document Detail


Primary ciliary dyskinesia causing neonatal respiratory distress
MedLine Citation:
PMID:  20013568     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Primary ciliary dyskinesia (PCD) is a hereditary disorder of structure and function of the cilia of respiratory epithelium of the upper and lower airways. Prevalence is estimated with 1:15 000 to 1:30 000 births. We present a newborn infant with respiratory distress caused by PCD. PATIENT: On the first day of life, the male newborn developed dyspnoe and cyanosis, so that CPAP and short term ventilation was necessary. Varying atelectasis impressed on the chest radiographs and the diagnosis of PCD was made by nasal brush biopsies. Causative is a lack of the inner dynein arms of the cilia. The clinical features of newborns with the diagnoses of PCD are listed and compared with the own case. CONCLUSION: PCD is a rare cause of neonatal respiratory distress and should be considered in term infants with unknown and prolonged course even if Situs inversus is lacking.
Authors:
C H?nseler; L Lange; A Kribs; A Vierzig; B Roth
Publication Detail:
Type:  English Abstract; Journal Article    
Journal Detail:
Title:  Klinische P?diatrie     Volume:  221     ISSN:  1439-3824     ISO Abbreviation:  Klin Padiatr     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-12-16     Completed Date:  2010-03-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0326144     Medline TA:  Klin Padiatr     Country:  Germany    
Other Details:
Languages:  ger     Pagination:  440-3     Citation Subset:  IM    
Copyright Information:
(c) Georg Thieme Verlag KG Stuttgart New York.
Affiliation:
Bereich Neonatologie, Kinderklinik der Universit?t zu K?ln. christoph.huenseler@uni-koeln.de
Vernacular Title:
Prim?re Ziliendyskinesie als Ursache neonataler Atemnot.
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MeSH Terms
Descriptor/Qualifier:
Administration, Inhalation
Albuterol / administration & dosage
Axonemal Dyneins / deficiency,  genetics
Biopsy
Bronchoscopy
Combined Modality Therapy
Continuous Positive Airway Pressure
Diagnosis, Differential
Humans
Infant, Newborn
Kartagener Syndrome / diagnosis,  genetics*,  pathology,  therapy
Male
Oxygen Inhalation Therapy
Physical Therapy Modalities
Positive-Pressure Respiration
Pulmonary Atelectasis / etiology
Respiratory Distress Syndrome, Newborn / diagnosis,  genetics*,  pathology,  therapy
Chemical
Reg. No./Substance:
18559-94-9/Albuterol; EC 3.6.4.2/Axonemal Dyneins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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