title: Potential donor-recipient MYH9 genotype interactions in posttransplant nephrotic syndrome after pediatric kidney transplantation.
abstract: Recurrence of focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome is relatively common after kidney transplantation in young recipients whose predialysis course consists of heavy proteinuria, hypertension and subacute loss of kidney function. The gene(s) mediating this effect remain unknown. We report an unusual circumstance where kidneys recovered from a deceased African American male donor with MYH9-related occult FSGS (risk variants in seven of eight MYH9 E1 haplotype single nucleotide polymorphisms) were transplanted into an African American male child with risk variants in four MYH9 E1 risk variants and a European American female teenager with two MYH9 E1 risk variants. Fulminant nephrotic syndrome rapidly developed in the African American recipient, whereas the European American had an uneventful posttransplant course. The kidney donor lacked significant proteinuria at the time of organ procurement. This scenario suggests that donor-recipient interactions in MYH9, as well as other gene-gene and gene-environment interactions, may lead to recurrent nephrotic syndrome after renal transplantation. The impact of transplanting kidneys from donors with multiple MYH9 risk alleles into recipients with similar genetic background at high risk for recurrent kidney disease needs to be determined.
authors.affiliation: Departments of Internal Medicine (Nephrology), Wake Forest University School of Medicine, Winston-Salem, NC, USA. bfreedma@wfubmc.edu
authors.forename: B I
authors.initials: BI
authors.lastname: Freedman
authors.forename: S K
authors.initials: SK
authors.lastname: Nagaraj
authors.forename: J-J
authors.initials: JJ
authors.lastname: Lin
authors.forename: M D
authors.initials: MD
authors.lastname: Gautreaux
authors.forename: D W
authors.initials: DW
authors.lastname: Bowden
authors.forename: S S
authors.initials: SS
authors.lastname: Iskandar
authors.forename: R J
authors.initials: RJ
authors.lastname: Stratta
authors.forename: J
authors.initials: J
authors.lastname: Rogers
authors.forename: E L
authors.initials: EL
authors.lastname: Hartmann
authors.forename: A C
authors.initials: AC
authors.lastname: Farney
authors.forename: A M
authors.initials: AM
authors.lastname: Reeves-Daniel
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D005260:Female; D005838:Genotype; D006239:Haplotypes; D006801:Humans; D016030:Kidney Transplantation / Q000009:adverse effects*; D008297:Male; D020409:Molecular Motor Proteins / Q000235:genetics*; D018995:Myosin Heavy Chains / Q000235:genetics*; D009404:Nephrotic Syndrome / Q000209:etiology* / Q000235:genetics
chemical_list: C103814:MYH9 protein, human; D020409:Molecular Motor Proteins; D018995:Myosin Heavy Chains