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Diabetic acido-ketosis revealing thiamine-responsive megaloblastic anemia
O Bouyahia1, M Ouderni, F Ben Mansour
1Service de médecine infantile A, hôpital d'Enfants de Tunis, Jebari, 1007 Bab Saadoun, Tunisia. olfa.bouyahia@rns.tn
Abstract:
Thiamine-responsive megaloblastic anemia (TRMA) is a rare autosomal recessive disorder characterized by megaloblastic anemia, diabetes mellitus and progressive sensorineural deafness. We report the cases of two infants, aged 4 and 5 months, hospitalized for diabetic ketoacidosis requiring insulin therapy. Laboratory tests revealed megaloblasic anemia, thrombocytopenia and normal thiamine level. Neurosensorial investigations showed bilateral deafness and ophthalmic involvement. Treatment with oral thiamine normalized hematological disorders and controlled diabetes; however, thiamine therapy had no impact on neurosensorial disorders.
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First, the pH level is assessed to determine whether the blood pH is normal (7.35–7.45), low (acidosis), or high (alkalosis).
Next, the PCO2 and HCO3− values are examined to...
