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)

Although effective treatments are available for many children with LCH, there are many others for whom no definitive therapy yet exists. These patients include those with 1) multisystem disease and associated organ dysfunction, 2) chronic, relapsing disease, 3) new onset pituitary involvement associated with diabetes insipidus and 4) long-term complications such as pulmonary fibrosis, liver fibrosis or CNS involvement. This introductory paper discusses these clinical problem areas and then reviews several new therapeutic approaches including novel chemotherapeutic agents, immunosuppressive strategies, bone marrow transplantation and gene therapy.
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PMID:Treatment options--commentary. 807 10

We report two children, aged 2 and 2 1/2 years, with multisystem Langerhans cell histiocytosis (LCH). Both were administered chemotherapy, with apparently good response. However, hepatic fibrosis and portal hypertension were detected 5 and 1 1/2 years after therapy, respectively. The first child died after a bout of hematemesis. Hepatic fibrosis can proceed despite apparently successful chemotherapy in LCH.
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PMID:Portal hypertension secondary to Langerhans cell histiocytosis. 1167 37