title: Persistent Tachypnea of Infancy. Usual and Aberrant.
abstract: BACKGROUND
  Persistent tachypnea of infancy (PTI) is a specific clinical entity of undefined etiology comprising the two diseases neuroendocrine cell hyperplasia of infancy (NEHI) and pulmonary interstitial glycogenosis. The outcome of typical NEHI is favorable. The outcome may be different for patients without a typical NEHI presentation, and thus a lung biopsy to differentiate the diseases is indicated.
  
  
  OBJECTIVE
  To determine whether infants with the characteristic clinical presentation and computed tomographic (CT) imaging of NEHI (referred to as "usual PTI") have long-term outcome and biopsy findings similar to those of infants with an aberrant presentation and/or with additional localized minor CT findings (referred to as "aberrant PTI").
  
  
  METHODS
  In a retrospective cohort study, 89 infants with PTI were diagnosed on the basis of clinical symptoms and, if available, CT scans and lung biopsies. Long-term outcome in childhood was measured on the basis of current status.
  
  
  RESULTS
  Infants with usual PTI had the same respiratory and overall outcomes during follow-up of up to 12 years (mean, 3.8 yr) as infants who had some additional localized minor findings (aberrant PTI) visualized on CT images. Both usual and aberrant PTI had a relatively favorable prognosis, with 50% of the subjects fully recovered by age 2.6 years. None of the infants died during the study period. This was independent of the presence or absence of histological examination.
  
  
  CONCLUSIONS
  PTI can be diagnosed on the basis of typical history taking, clinical findings, and a high-quality CT scan. Further diagnostic measures, including lung biopsies, may be limited to rare, complicated cases, reducing the need for an invasive and potentially harmful procedure.
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Daniela
authors.initials: D
authors.lastname: Rauch
authors.affiliation: 2 Department of Pediatric Pneumology, Allergology, and Neonatology, Hannover Medical School, German Center for Lung Research, Hannover, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Wetzke
authors.affiliation: 3 Department of Pathology, Ludwig Maximilians University of Munich, German Center for Lung Research, Munich, Germany.
authors.forename: Simone
authors.initials: S
authors.lastname: Reu
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Waltraud
authors.initials: W
authors.lastname: Wesselak
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Andrea
authors.initials: A
authors.lastname: Schams
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Meike
authors.initials: M
authors.lastname: Hengst
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Birgit
authors.initials: B
authors.lastname: Kammer
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Julia
authors.initials: J
authors.lastname: Ley-Zaporozhan
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Matthias
authors.initials: M
authors.lastname: Kappler
authors.affiliation: 4 Department of Pediatric Pneumology, University Hospital Gasthuisberg, Leuven, Belgium.
authors.forename: Marijke
authors.initials: M
authors.lastname: Proesmans
authors.affiliation: 5 Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Lange
authors.affiliation: 6 Department of Pediatrics, Obstetrics, and Gynecology, School of Medicine, University of Valencia, Valencia, Spain.
authors.forename: Amparo
authors.initials: A
authors.lastname: Escribano
authors.affiliation: 7 Department of Pediatrics, Hadassah Medical Center, Jerusalem, Israel.
authors.forename: Eitan
authors.initials: E
authors.lastname: Kerem
authors.affiliation: 8 Children's Hospital "Altona", Hamburg, Germany; and.
authors.forename: Frank
authors.initials: F
authors.lastname: Ahrens
authors.affiliation: 9 Department of Pathology, Academic Teaching Hospital Bielefeld, Bielefeld, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Brasch
authors.affiliation: 3 Department of Pathology, Ludwig Maximilians University of Munich, German Center for Lung Research, Munich, Germany.
authors.forename: Nicolaus
authors.initials: N
authors.lastname: Schwerk
authors.affiliation: 1 Dr. von Hauner Children's Hospital and.
authors.forename: Matthias
authors.identifier: http://orcid.org/0000-0003-0113-912X
authors.initials: M
authors.lastname: Griese
mesh_terms: D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006008:Glycogen Storage Disease / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D006965:Hyperplasia / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D008297:Male; D055099:Neuroendocrine Cells / Q000000981:diagnostic imaging / Q000473:pathology; D009490:Neurosecretory Systems / Q000000981:diagnostic imaging* / Q000473:pathology*; D012189:Retrospective Studies; D059246:Tachypnea / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology*; D014057:Tomography, X-Ray Computed
keywords: children’s interstitial lung disease; diffuse parenchymal lung disease; infants; neuroendocrine cell hyperplasia of infancy; pulmonary interstitial glycogenosis