Daniela Rauch1, Martin Wetzke2, Simone Reu3, Waltraud Wesselak1, Andrea Schams1, Meike Hengst1, Birgit Kammer1, Julia Ley-Zaporozhan1, Matthias Kappler1, Marijke Proesmans4, Joanna Lange5, Amparo Escribano6, Eitan Kerem7, Frank Ahrens8, Frank Brasch9, Nicolaus Schwerk3, Matthias Griese1. 1. 1 Dr. von Hauner Children's Hospital and. 2. 2 Department of Pediatric Pneumology, Allergology, and Neonatology, Hannover Medical School, German Center for Lung Research, Hannover, Germany. 3. 3 Department of Pathology, Ludwig Maximilians University of Munich, German Center for Lung Research, Munich, Germany. 4. 4 Department of Pediatric Pneumology, University Hospital Gasthuisberg, Leuven, Belgium. 5. 5 Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Poland. 6. 6 Department of Pediatrics, Obstetrics, and Gynecology, School of Medicine, University of Valencia, Valencia, Spain. 7. 7 Department of Pediatrics, Hadassah Medical Center, Jerusalem, Israel. 8. 8 Children's Hospital "Altona", Hamburg, Germany; and. 9. 9 Department of Pathology, Academic Teaching Hospital Bielefeld, Bielefeld, Germany.
Abstract
RATIONALE: 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. OBJECTIVES: 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. MEASUREMENTS AND MAIN 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.
RATIONALE: 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. OBJECTIVES: 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. MEASUREMENTS AND MAIN 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: Jason P Weinman; Christina J White; Deborah R Liptzin; Robin R Deterding; Csaba Galambos; Lorna P Browne Journal: Pediatr Radiol Date: 2018-04-23
Authors: Robin R Deterding; Emily M DeBoer; Michal J Cidon; Terry E Robinson; David Warburton; Gail H Deutsch; Lisa R Young Journal: Am J Respir Crit Care Med Date: 2019-11-15 Impact factor: 21.405
Authors: Deborah R Liptzin; Christopher D Baker; Jeffrey R Darst; Jason P Weinman; Megan K Dishop; Csaba Galambos; John T Brinton; Robin R Deterding Journal: Pediatr Pulmonol Date: 2018-07-18
Authors: Mara Lelii; Maria Francesca Patria; Raffaella Pinzani; Rossana Tenconi; Alessandro Mori; Nicola Bonelli; Nicola Principi; Susanna Esposito Journal: Int J Environ Res Public Health Date: 2017-09-25 Impact factor: 3.390