INSULIN-LIKE GROWTH FACTOR TYPE 1 IN PATIENTS WITH ACUTE INTERMITTENT PORPHYRIA AND ITS RELATIONSHIP WITH HEME BIOSYNTHESIS INTERMEDIATES

MORA SANDRA; OLIVERI LEDA; CALCAGNO MARIA LUJÁN; PARERA VICTORIA ESTELA; ROSSETTI MARÍA VICTORIA; GEREZ, ESTHER

Congreso Virtual

Congreso; LXV REUNIÓN ANUAL DE LA SOCIEDAD ARGENTINA DE INV...; 2020

Sociedad Argentina de Investigación Clínica

Acute Intermittent Porphyria (AIP) is caused by an hereditary disorder of heme biosynthesis, produced by a decrease in the activity of porphobilinogen deaminase, associated with an increase in the expression of the regulatory enzyme of this pathway, d-aminolevulic synthetase 1 (ALAS1), accumulating the neurotoxic precursor d-minolevulic acid (ALA) generating recurrent and intense seizures with neurological involvement.Carbohydrate treatment rapidly reduces ALA production; the therapeutic effect is related to the ability of both glucose and insulin to modulate ALAS1 activity. Decreased levels of insulin-like growth factor 1 (IGF1) may alter the metabolism of specific carbohydrates and lipids, altering the flow of nutrients to the liver.As IGF1 acts complementary to insulin, we decided to evaluate IGF1levels and its relationship with heme metabolism in an AIP patients.In a population of 82 genetically diagnosed individuals, the biochemical parameters (BP) of AIP: ALA, porphobilinogen (PBG) and totalporphyrins (TP) and the IGF1 levels were measured. Three groupswere classified according to symptoms: Latent (L): no symptoms;Manifested (M): presented attack and BP values returned to normal;Subclinical Manifested (SM): presented attack and their BP valuesremained elevated.To compare the BP and IGF1 levels between L, M and SM groups,the variables were categorized as: IGF1-n (normal) and IGF1-l (low)and for the BP: ALA, PBG and TP as normal and elevated.There is only a significant association in the SM group (p = 0.0029):84% of the patients have elevated ALA / IGF1-l and when we verifythe relation between IGF1 with the three high BP simultaneously,the SM group shows a significant association (p = 0.008): 80% ofpatients with IGF1-l have the three BP high. [Irwin-Fischer bilateraland Chi square Pearson, p