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Long-term evolution of neuroendocrine cell hyperplasia of infancy: the FRENCHI findings

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Abstract

Only few studies report long-term evolution of patients with neuroendocrine cell hyperplasia of infancy (NEHI). We report data from a 54-patient cohort followed up in the French network for rare respiratory diseases (RespiRare). Demographic characteristics and respiratory and nutritional evolution were collected at the time of the patient’s last scheduled visit. The mean duration of follow-up was 68 months (5 months to 18 years). Fifteen patients (27.8%) were considered clinically cured. During follow-up, hospitalizations for wheezy exacerbations were reported in 35 patients (55%), and asthma diagnosed in 20 (37%). Chest CT scan improvement was noted in 25/44 (56.8%). Spirometry showed a persistent obstructive syndrome in 8/27 (29.6%). A sleep disorder was rare (2/36, 5.5%). Oxygen weaning occurred in 28 of the 45 patients initially treated (62.2%) and was age-dependent (35.7% under 2 years, 70.5% between 2 and 6 years, and 100% after 7 years). Oxygen duration was linked to a biopsy-proven diagnosis (p = 0.02) and to the use of a nutritional support (p = 0.003). Corticosteroids were largely prescribed at diagnosis, with no evident respiratory or nutritional effect during follow-up. Among 23 patients with an initial failure to thrive, 12 (52.2%) had no weight recovery. Initial enteral feeding (17/54, 31.5%) was stopped at a mean age of 43 months (3 to 120), with no effect on cure and oxygen liberation at the last visit.

  Conclusion: Our results show that NEHI has a globally positive, but unequal, improvement over time. Further prospective studies are needed to better clarify the different trajectories of patients with NEHI.

What is Known:

• Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease whose long-term outcome is considered positive from very few studies including heterogeneous populations.

What is New:

• The 68-month follow-up of our 54-patient cohort showed respiratory/nutritional symptom persistence in 72.2%, oxygen requiring in 34%, and asthma in 37%. When controlled, radiological or functional improvement was noted in 56.8 and 40.7%. Further prospective studies are needed to better clarify the different trajectories of patients with NEHI.

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Data were anonymized but are available in an Excel file.

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Abbreviations

chILD:

Childhood interstitial lung disease

FEV1:

Forced expiratory volume in one second

NEHI:

Neuroendocrine cell hyperplasia of infancy

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Author information

Authors and Affiliations

  1. Pediatric Pulmonology Department, University Timone Hospital for Children, AP-HM, Marseille, France

    Morgane Dervaux, Candice Fabre, Julie Mazenq & Jean-Christophe Dubus

  2. Pediatric Pulmonology and Allergy Department, Jeanne de Flandre Hospital, CHU Lille, Univ. Lille, Pole Enfant, Lille, France

    Caroline Thumerelle

  3. Reference Center for Rare Lung Diseases, Pediatric Pulmonology Department, University Hospital of Necker-Enfants Malades, AP-HP, Paris, France

    Rola Abou-Taam, Christophe Delacourt & Alice Hadchouel

  4. Chronic Childhood Diseases Unit, Pediatric Department, Nantes University Hospital, Nantes, France

    Tiphaine Bihouee

  5. Service de Pédiatrie Médicale, CHU Caen, Caen, et UMR1311 DYNAMICURE, Normandie Université, UNICAEN, UNIROUEN, Rouen, France

    Jacques Brouard

  6. Pediatric Pulmonology Department and Reference Center for Rare Lung Diseases (RespiRare), AP‑HP, Sorbonne University, Inserm UMR S-933 Childhood Genetic Disorders, Armand Trousseau Hospital, Paris, France

    Annick Clement & Nadia Nathan

  7. Center for Rare Lung Diseases (RespiRare), Centre Hospitalier Intercommunal of Créteil, University Paris Est Créteil, INSERM, IMRB, Créteil, France

    Céline Delestrain & Ralph Epaud

  8. Pediatric Unit, Grenoble-Alpes University Hospital, Grenoble, France

    Sofiane Ghdifan

  9. Pediatric Pulmonology Department, INSERM UMR S 976 Human Immunology, AP-HP, Paris University Robert Debre Hospital, Paris, France

    Véronique Houdouin

  10. Pediatric Pulmonology Department, University Hospital for Children, Toulouse, France

    Géraldine Labouret

  11. Pediatric Unit, University Hospital Sud Reunion, Saint-Pierre, France

    Caroline Perisson

  12. Pediatric Pulmonology, Allergology Cystic Fibrosis Department, Hospices Civils of Lyon, Hôpital Femme Mère Enfant, Bron, France

    Philippe Reix

  13. UMR 5558, CNRS Equipe, EMET University, Claude Bernard Lyon 1, Lyon, France

    Philippe Reix

  14. Paediatric Cardiology and Pulmonology Department, Montpellier University Hospital, Montpellier, France

    Marie-Catherine Renoux

  15. Pediatric Department, University Hospital, Angers, France

    Françoise Troussier

  16. Specialized Pediatric Department, University Hospital of Strasbourg, Strasbourg, France

    Laurence Weiss

  17. Aix Marseille University, IRD, MEPHI, IHU Méditerranée Infection, Marseille, France

    Jean-Christophe Dubus

  18. Unité de Pneumopédiatrie, CHU Timone-Enfants, 13385 Cedex 5, Marseille, France

    Jean-Christophe Dubus

Authors
  1. Morgane Dervaux
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  2. Caroline Thumerelle
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  3. Candice Fabre
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  4. Rola Abou-Taam
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  5. Tiphaine Bihouee
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  6. Jacques Brouard
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  7. Annick Clement
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  8. Christophe Delacourt
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  9. Céline Delestrain
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  10. Ralph Epaud
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  11. Sofiane Ghdifan
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  12. Alice Hadchouel
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  13. Véronique Houdouin
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  14. Géraldine Labouret
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  15. Caroline Perisson
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  16. Philippe Reix
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  17. Marie-Catherine Renoux
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  18. Françoise Troussier
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  19. Laurence Weiss
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  20. Julie Mazenq
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  21. Nadia Nathan
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  22. Jean-Christophe Dubus
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by M. Dervaux, C. Thumerelle, C. Fabre, N. Nathan, and J. C. Dubus. The first draft of the manuscript was written by M. Dervaux, C. Thumerelle, C. Fabre, J. Mazenq, N. Nathan, and J. C. Dubus and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Jean-Christophe Dubus.

Ethics declarations

Ethics approval

This is an observational study. The Comité d’éthique de la recherche de la Société Française de Pédiatrie (CER_SFP_2020_123) and the Commission Nationale de l’Informatique et des Libertés (CNIL 2219327) gave their consents.

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The authors declare no competing interests.

Additional information

Communicated by Peter de Winter

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Dervaux, M., Thumerelle, C., Fabre, C. et al. Long-term evolution of neuroendocrine cell hyperplasia of infancy: the FRENCHI findings. Eur J Pediatr 182, 949–956 (2023). https://doi.org/10.1007/s00431-022-04734-y

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  • DOI: https://doi.org/10.1007/s00431-022-04734-y

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