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Early onset of childhood pulmonary lymphangiomyomatosis
E Nussbaum1, P Groncy, J Finklestein
1Children's Lung Institute, Miller Children's Hospital, Long Beach, California 90801-1428.
Clinical Pediatrics
|June 1, 1988
Insights
This study details the first pediatric case of lymphangiomyomatosis, a rare lung disease. Early diagnosis and understanding its non-pulmonary symptoms are crucial for young patients.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Medical Imaging
Background:
- Lymphangiomyomatosis (LAM) is a rare, progressive lung disease typically affecting women of reproductive age.
- Pediatric LAM is exceptionally rare, with limited documented cases.
- Understanding early disease presentation in children is vital for timely diagnosis and management.
Abstract:
The authors present the first case of lymphangiomyomatosis in a pediatric patient. Radiographic findings, biopsy, computerized tomography, and electron microscopy were useful in detailing the presentation of early onset of pulmonary lymphangiomyomatosis. The unusual presentation of this child includes a description of her disease prior to any pulmonary symptomatology and 4 years of follow-up.