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A rare cause of multiple airways narrowing in a 15-year-old girl
  1. Sergio Ghirardo1,
  2. Federica Porcaro2,
  3. Maria Beatrice Chiarini Testa2,
  4. Maria Giovanna Paglietti2,
  5. Nicola Ullmann2,
  6. May El Hachem3,
  7. Fabrizio De Benedetti4,
  8. Renato Cutrera2
  1. 1 Medicine, Surgery and Health Sciences, University of Trieste, Trieste, Italy
  2. 2 Pediatric Pulmonology & Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Department of Pediatrics, Bambino Gesu Pediatric Hospital Department of Paediatrics, Roma, Italy
  3. 3 Dermatology Unit, Bambino Gesu Pediatric Hospital Department of Paediatrics, Roma, Italy
  4. 4 Division of Rheumatology, Bambino Gesu Pediatric Hospital, Roma, Italy
  1. Correspondence to Dr Sergio Ghirardo, Medicine, Surgery and Health Sciences, University of Trieste, Trieste 34127, Italy; ghirardo.sergio{at}

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Sergio Ghirardo (SG): a 15-year-old girl was first admitted to our unit, with significant dyspnoea and fatigue at rest. Her clinical history was uneventful until she was 14 years old when she was hospitalised twice for respiratory symptoms, interpreted as post viral wheezing. Treatment with intravenous corticosteroids, salbutamol and azithromycin was of little clinical benefit. In the last 6 months, she complained of progressive exercise-induced dyspnoea, and her lung function showed a moderate-to-severe obstructive pattern (forced expiratory volume in one second, FEV1/forced vital capacity, FVC 56.6%), with no response to salbutamol. Her symptoms did not improve with inhaled corticosteroids, long-acting β2 agonists and montelukast treatment. Clinically, she reported moderate swelling of the nasal dorsum as a consequence of a blow with a ball received 5 months before admission. She had a bilateral transmissive mild hypoacusis and a significative weight loss of 18 kg in only 8 months.

On initial physical examination she appeared pale and tired with a mild redness of the ear helix sparing the lobe and a mild swelling of the nasal dorsum. She was tachypnoeic (20 breaths/min), with normal levels of oxygen saturation. Mild supraclavicular retractions were present during inspiration. On chest auscultation stridor and mild bilateral wheezing were detected. Heart rate was 109 beats/min. The remaining physical examination was normal.

Renato Cutrera (RC): we have a previously healthy female adolescent with a personal history of only partially remitting obstructive dyspnoea suggesting a chronic respiratory infection (like tuberculosis or pulmonary mycosis). However, the fatigue associated with significant weight loss should raise the suspicion of pulmonary hypertension. I suggest performing routine blood tests, chest X-ray, full blood count and cardiological evaluation. At the same time, I would administer oral corticosteroids and antibiotic coverage.

Federica Porcaro (FP): blood tests showed a mild rise of C reactive protein (CRP) 7.2 mg/L (normal range 0–5 mg/L); whereas …

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  • Presented at This case was scheduled for a presentation at American Thoracic Society conference of Philadelphia 2020 but the conference was cancelled due to Covid-19 pandemic.

  • Contributors SG, FP, MGP and MBCT cared the patient. MEH diagnosed the cutaneous aspect of the disease. FDB and RC made the final diagnosis and decided the therapeutic regimen. SG and FP wrote the first draft of the manuscript. MGP and MBCT revised the manuscript. MEH, FDB and RC finally revised and approved the paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.