Williams-Campbell Syndrome in a Pediatric Patient: A Case Report
Notice bibliographique
Résumé
Abstract Background: Williams-Campbell Syndrome (WCS) is a rare cause of bronchiectasis marked by the absence or significant reduction of cartilage in the bronchial tree. Typically, it affects bronchi between the 4th and 7th-8th generations, and is generally bilateral and diffuse. Its origins—whether congenital or acquired—remain uncertain. Common symptoms include cough, recurrent respiratory infections, wheezing, rales, and clubbing. Diagnosis is usually confirmed by chest computed tomography (CT), which reveals bronchiectasis in the segmental bronchi. Case Presentation: A 10-year-old girl was brought to our tertiary care center's emergency department with dyspnea, cough, fever, and worsening respiratory distress during an upper respiratory tract infection (URTI). She had an oxygen saturation of 91% on room air, wheezing in both lung fields, and diminished air entry in the left lung. Clubbing was observed. Initially diagnosed with status asthmaticus, she was admitted to the pediatric intensive care unit (PICU) for non-invasive ventilation (NIV), bronchodilators, and empirical antibiotics with azithromycin. Her past medical history included sporadic treatment with salbutamol and fluticasone for URTIs in infancy and an unremarkable family history for respiratory conditions. Two months prior, she had been admitted to the PICU at another center for NIV due to influenza complicated by right-sided pneumonia. Following that hospitalization, she never recovered her usual exercise tolerance. Infectious workup was negative except for entero/rhinovirus on nasopharyngeal swab. Chest X-ray revealed a 3.1cm cystic lesion. Given her atypical progression and newly noted clubbing, a chest CT was performed, revealing diffuse cylindrical, varicose, and cystic bronchiectasis. An extensive workup, including a sweat test, genetic testing for primary ciliary dyskinesia and immune deficiency, was normal. Her respiratory condition gradually improved and she was discharged at day 10 without supplementary oxygen. At 5-month follow-up, spirometry indicated a non-reversible severe obstructive pattern (FEV1 Z-score: -5.97; VEMS/CVF Z-score: -3,39). A 6-minute walk test was pathological with desaturation to 87% on room air. A chest CT showed ballooning and collapse of bronchiectasis during inspiration and expiration, respectively. A cardiac ultrasound was normal. Discussion: In the absence of pointers for other specific respiratory conditions, the findings are compatible with WCS. She is currently treated with azithromycin, fluticasone/salmeterol, and daily positive expiratory pressure physiotherapy. Conclusion: This case highlights WCS, a rare condition for which no specific curative treatment presently exists. Typical airway anomalies are well-identified on high-resolution CT. Preventative respiratory physiotherapy and infection control may help slow respiratory decline.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,003 | 0,002 |
| Méta-épidémiologie (sens large) | 0,002 | 0,001 |
| Bibliométrie | 0,004 | 0,003 |
| Études des sciences et des technologies | 0,003 | 0,002 |
| Communication savante | 0,002 | 0,003 |
| Science ouverte | 0,002 | 0,003 |
| Intégrité de la recherche | 0,006 | 0,004 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,003 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».