[Clinical trial of mannose treatment of hemolytic anemia caused by congenital deficiency of erythrocyte glucosephosphate isomerase].
A therapeutic trial with mannose given intravenously as a 5% solution during 7 consecutive days (daily doses 12.5 g, 25 g and 50 g) was performed in a GPI deficient girl with nonspherocytic hemolytic anemia. The aim of the trial was to substitute glucose--the main red cells metabolic substrate--with mannose, since the glucose metabolism, due to the GPI deficiency, was significantly decreased. An initial good effect of treatment was disturbed with viral infection. No complications due to treatment with mannose were observed.
Jabłońska-Skwiecińska
E
E
Zakładu Diagnostyki Laboratoryjnej CMKP, Warszawie.
Giro
E
E
Rokicka-Milewska
R
R
D000746:Anemia, Hemolytic, Congenital Nonspherocytic / Q000097:blood / Q000178:diet therapy* / Q000209:etiology; D002648:Child; D004912:Erythrocytes / Q000201:enzymology*; D005260:Female; D006400:Hematocrit; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007262:Infusions, Intravenous; D008358:Mannose / Q000008:administration & dosage*; D008661:Metabolism, Inborn Errors / Q000097:blood* / Q000150:complications; D013997:Time Factors
D006454:Hemoglobins; D008358:Mannose
Próba leczenia mannoza niedokrwistości hemolitycznej na tle wrodzonego niedoboru izomerazy glukozofosforanowej krwinek czerwonych.