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Neuroendocrine cell hyperplasia of infancy: a prospective follow-up of nine children
  1. Heikki Lukkarinen1,
  2. Anna Pelkonen2,
  3. Jouko Lohi3,
  4. Kristiina Malmström2,
  5. L Pekka Malmberg2,
  6. Merja Kajosaari4,
  7. Harri Lindahl5,
  8. Anna Föhr6,
  9. Olli Ruuskanen1,
  10. Mika J Mäkelä2
  1. 1Department of Pediatrics, Turku University Central Hospital, Turku, Finland
  2. 2Department of Allergy, Helsinki University Central Hospital, Skin and Allergy Hospital, Helsinki, Finland
  3. 3Department of Pathology, Helsinki University Central Hospital, Helsinki, Finland
  4. 4Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland
  5. 5Department of Pediatric Surgery, Helsinki University Central Hospital, Helsinki, Finland
  6. 6Department of Radiology, Helsinki University Central Hospital, Helsinki, Finland
  1. Correspondence to Dr Heikki Lukkarinen, Department of Pediatrics, Turku University Central Hospital, PB 52, Turku FIN-20520, Finland; hepelu{at}utu.fi

Abstract

Neuroendocrine cell hyperplasia of infancy (NEHI) has recently been described as an obstructive airway disease that affects infants aged 1–24 months, and presents typically with tachypnoea, crackles and hypoxia. The pathogenesis of the disease is unknown. We describe the clinical course of nine infants with radiologically and histologically confirmed NEHI. Host or environmental factors were not associated with the disease development. All infants with lung function tests demonstrated findings consistent with severe irreversible peripheral airway obstruction, assessed with whole body plethysmography (6/6) or the rapid thoracoabdominal compression technique (5/5). While the symptoms abated in all infants, six infants developed a non-atopic asthma during the follow-up. Systemic or inhaled corticosteroid treatment did not affect the duration of the symptoms. NEHI may mimic severe asthma and thus this entity should be taken into account when evaluating infants with chronic respiratory symptoms.

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