IMERSLUND-SYNDROME IN 6 CHILDREN

Background. Imerslund syndrome, a recessive autosomal disease, initial ly described by Imerslund and Grasbeck in 1960, associates megaloblast ic anemia and proteinuria. Case report. - We report on six cases, stud ied in five different proteinuria was present in five, at the time of diagnosis. Hemogram and decreased serum vitamin B12 levels were consis tent with the diagnosis in all cases. Intra-muscular injections of cya nocobalamine was instituted on a life-time basis and the long term pro gnosis is good. Conclusion. - The diagnosis should be evoked when the three typical features are present: macrocytic anemia, decreased serum B12 level and proteinuria. It will be confirmed by the bone marrow me galoblastic aspects and the Schilling test findings.