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Hemophagocytic lymphohistiocytosis and related disorders.
Filipovich AH. Filipovich AH. Curr Opin Allergy Clin Immunol. 2006 Dec;6(6):410-5. doi: 10.1097/01.all.0000246626.57118.d9. Curr Opin Allergy Clin Immunol. 2006. PMID: 17088644 Review.
Related autosomal recessive defects of secretory cytotoxic lysosomes - LYST 1 (Chediak-Higashi syndrome), Rab27A (Griscelli syndrome), and X-linked lymphoproliferative disorder - also carry a very high risk of fatal hemophagocytic lymphohistiocytosis. ...
Related autosomal recessive defects of secretory cytotoxic lysosomes - LYST 1 (Chediak-Higashi syndrome), Rab27A (Griscelli syndrome) …
The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome.
Young LR, Gulleman PM, Bridges JP, Weaver TE, Deutsch GH, Blackwell TS, McCormack FX. Young LR, et al. Am J Respir Crit Care Med. 2012 Nov 15;186(10):1014-24. doi: 10.1164/rccm.201207-1206OC. Epub 2012 Oct 4. Am J Respir Crit Care Med. 2012. PMID: 23043085 Free PMC article.
OBJECTIVES: To decipher which cell lineage(s) in the alveolar compartment is the predominant driver of fibrotic susceptibility in HPS. METHODS: We used five different HPS and Chediak-Higashi mouse models to evaluate genotype-specific fibrotic susceptibility. ...
OBJECTIVES: To decipher which cell lineage(s) in the alveolar compartment is the predominant driver of fibrotic susceptibility in HPS. METHO …