18q deletion in a cystic fibrosis infant, increased morbidity and challenge for correct treatment choices: a case report
2011

Case Report of Cystic Fibrosis with 18q Deletion

Sample size: 1 publication Evidence: low

Author Information

Author(s): Spinelli Elide, Timpano Silviana, Fogazzi Annalisa, Dester Silvia, Milianti Susanna, Padoan Rita

Primary Institution: Centro Regionale di Supporto per la Fibrosi Cistica, Ospedale dei Bambini, AO Spedali Civili, Brescia, Italy

Hypothesis

Can co-existing conditions like 18q deletion syndrome complicate the treatment of cystic fibrosis in infants?

Conclusion

The combination of innovative therapies improved the clinical condition of a cystic fibrosis infant with an 18q deletion.

Supporting Evidence

  • The infant was diagnosed with cystic fibrosis through neonatal screening.
  • Co-morbidities included severe muscular hypotonia and congenital cardiopathy.
  • Use of a gastrostomy device and Ez-PAP improved the infant's growth and reduced hospitalizations.

Takeaway

This study talks about a baby with cystic fibrosis who also had a special chromosome problem, and how doctors helped him get better using new treatments.

Methodology

The case was managed with a multi-disciplinary approach including nutritional support and innovative respiratory therapies.

Limitations

The study is based on a single case, limiting generalizability.

Participant Demographics

Male infant, aged 2, born to non-consanguineous Albanian parents.

Digital Object Identifier (DOI)

10.1186/1824-7288-37-22

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