Williams-Campbell Syndrome in a Pediatric Patient: A Case Report
Bibliographic record
Abstract
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.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.003 |
| Meta-epidemiology (narrow) | 0.003 | 0.002 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.004 | 0.003 |
| Science and technology studies | 0.003 | 0.002 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.002 | 0.003 |
| Research integrity | 0.006 | 0.004 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".