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-  2018 

Successful use of ofatumumab in two cases of early-onset juvenile SLE with thrombocytopenia caused by a mutation in protein kinase C δ

DOI: 10.1186/s12969-018-0278-1

Keywords: Ofatumumab, Juvenile SLE, Rituximab, Protein kinase C δ deficiency, Acute tubulointerstitial nephritis

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Abstract:

Pedigree of the family and renal histology. a The affected siblings (shaded) are homozygous for the PRKCD mutation as indicated and the parents are carriers of the mutation. WT, wild type. b Low power (Periodic acid–Schiff stain, original magnification ×?40) and Fig. 1c higher power (Haematoxylin and Eosin stain, original magnification ×?200) showing severe acute tubulointerstitial nephritis with marked predominantly mononuclear inflammatory infiltrate and associated mild acute tubular damag

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