• Daniela Rauch, Martin Wetzke, Simone Reu, Waltraud Wesselak, Andrea Schams, Meike Hengst, Birgit Kammer, Julia Ley-Zaporozhan, Matthias Kappler, Marijke Proesmans, Joanna Lange, Amparo Escribano, Eitan Kerem, Frank Ahrens, Frank Brasch, Nicolaus Schwerk, Matthias Griese, and PTI (Persistent Tachypnea of Infancy) Study Group of the Kids Lung Register.
• 1 Dr. von Hauner Children's Hospital and.
• Am. J. Respir. Crit. Care Med. 2016 Feb 15; 193 (4): 438-47.

RationalePersistent 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.ObjectivesTo 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").MethodsIn 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 ResultsInfants 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.ConclusionsPTI 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.

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