Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cell histiocytosis (LCH) can involve multiple organs. "Primary" or isolated pulmonary LCH is a well-described entity in young adults but is exceedingly rare in children younger than 15 years of age. The authors report a new case in a 2-year-old girl and review other reported cases in the pediatric population. The patient had had respiratory symptoms since early infancy suggestive of hyperactive airway disease. At 2 years of age, she had severe pulmonary insufficiency with remarkable cystic changes noted on chest imaging studies. Biopsy of a pulmonary lesion confirmed the diagnosis of LCH. She had no other organ involvement. Pulmonary histiocytosis, though rare, should be considered in any child with chronic respiratory disease such as bronchial asthma, especially when the response to anti-asthma treatment is poor and/or there are cystic changes on the chest x-ray.
 ...
PMID:"Primary" pulmonary Langerhans cell histiocytosis in a two-year-old child: case report and literature review. 1646 78

A 4-year old boy was admitted to hospital with progressive respiratory failure. A chest roentgenogram revealed hyperinflated lungs and a diffuse reticular pattern. There was no smoking history in the family environment. A thin slice computed tomography of the lungs exhibited extended and diffuse cystic lung disease with no zonal predominance. An open lung biopsy showed pulmonary Langerhans cell histiocytosis. No other organs were involved. Despite treatment initiation the boy succumbed to his pulmonary insufficiency 3 weeks later. Isolated pulmonary Langerhans cell histiocytosis in children may have an unfavorable prognosis and in contrast with adults, it may present on computed tomography with lower lobe predominance and without sparing the lung bases or the anterior parts of middle lobe and lingula.
 ...
PMID:Atypical CT findings of isolated pulmonary Langerhans cell histiocytosis in a 4-year-old boy. 1752 24