Banday, Aaqib Zaffar published the artcileRefractory Autoimmune Cytopenia in a Young Boy with a Novel LRBA Mutation Successfully Managed with Sirolimus, SDS of cas: 55981-09-4, the main research area is autoimmune cytopenia LRBA mutation sirolimus.
In this article the author is discussing about refractory autoimmune cytopenia in a young boy with a novel LRBA mutation successfully managed with sirolimus. A 9-yr-old boy presented with history of recurrent diar-rhea and anemia requiring multiple blood transfusions. On examination, he had pallor, hepatosplenomegaly, severe wasting, and stunting. Replacement i.v. Ig (IVIg) therapy (400 mg/kg/mo) and cotrimoxazole prophylaxis (5 mg/kg/day of the trimethoprim component) were initiated along with iron, folic acid, and vitamin B12 supplementation. He showed some clin. improvement in diarrhea and anemia. After 6 mo of follow-up, he had to be initiatedonoral prednisolone(1mg/kg/day)in view of falling Hb and DCT positivity (both IgG and C3d). A clin. possibility of monogenic CVID with autoimmune cytopenia and benign lymphoproliferation was considered and targeted next generation sequencing was carried out. He has been taking sirolimus for past 9 mo now,and no blood transfusions were given after initiation of sirolimus. Index patient showed a transient response to oral cortico-steroids and IVIg.
Journal of Clinical Immunology published new progress about Anemia. 55981-09-4 belongs to class esters-buliding-blocks, name is 2-((5-Nitrothiazol-2-yl)carbamoyl)phenyl acetate, and the molecular formula is C12H9N3O5S, SDS of cas: 55981-09-4.
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