title: Imerslund-Grasbeck syndrome in a 5-year-old Iranian boy.
abstract: Imerslund-Grasbeck syndrome (IGS) is a rare syndrome characterized by clinical symptoms and signs of Vitamin B12deficiency and proteinuria. Our patient was a 5-year-old boy with pallor, lack of appetite, and low weight gain. Laboratory studies showed severe macrocytic anemia, normal reticulocyte count, negative direct coombs test, normal osmotic fragility, and autohemolysis test. He has had intermittent proteinuria since 3 years ago despite normal creatinine level and absence of hematuria or hypertension. Finally, based on low level of serum B12vitamin and normal folate level accompanied by asymptomatic proteinuria, the diagnosis of IGS was made. Furthermore, his sister has had laboratory abnormalities without any symptoms. IGS responded to B12replacement therapy dramatically but intermittent proteinuria persisted even after appropriate therapy.
authors.affiliation: Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: K
authors.initials: K
authors.lastname: Goudarzipour
authors.affiliation: Pediatric Congenital Hematologic Disorders Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: N
authors.initials: N
authors.lastname: Zavvar
authors.affiliation: Functional Neurosurgery Research Center, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: B
authors.initials: B
authors.lastname: Behnam
authors.affiliation: Functional Neurosurgery Research Center, Shohada Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: M A
authors.initials: MA
authors.lastname: Ahmadi
keywords: Imerslund-Grasbeck syndrome; megaloblastic anemia; pessary cell; proteinuria