The Comparison of Iodine-Type and MnO-Type Oxidation for Measuring the Levels of Urine Neopterin and Biopterin in Patients with Hyperphenylalaninemia: A Descriptive-Analytic Study in Iran.

Fresh urine sample is required for measurement of pterins in patients with hyperphenylalaninemia (HPA) for detection of tetrahydrobiopterin (BH) coenzyme deficiency. The present study aimed to measure the levels of neopterin and biopterin in the urine of patients with HPA using manganese dioxide (MnO) and iodine oxidation methods. The levels of neopterin and biopterin in the urine of 124 patients were measured using two methods of oxidation (MnO and iodine) and the results were statistically analyzed using correlation test, Roc-Curve and ANOVA in SPSS-20 software.

Clinical, Biochemical and Genetic Analysis of Biotinidase Deficiency in Iranian Population.

Background: Biotinidase deficiency (BTD) is an autosomal recessive disorder of biotin metabolism. Biotin is a coenzyme that enhances the action of the four enzymes that play an important role in carbohydrates, amino acid, and fatty acid metabolism. Defects in these pathways cause severe metabolic disorder in the body.

Four Years of Diagnostic Challenges with Tetrahydrobiopterin Deficiencies in Iranian Patients.

Hyperphenylalaninemia (HPA) is a condition caused by tetrahydrobiopterin (BH) and phenylalanine hydroxylase (PAH) deficiencies. It is essential that differential diagnosis be conducted to distinguish these two causes of HPA, because BH deficiency is a more severe disease involving progressive neurologic deterioration. Based on the biological findings, HPA is defined as a plasma phenylalanine level of >2.

Evaluation and comparison of soluble transferrin receptor in thalassemia carriers and iron deficient patients.

Iron is an essential component in the structure of certain molecules such as hemoglobin (Hb), myoglobin, cytochrome C and some enzymes. The iron gateway to cells is transferrin receptor (TfR). Soluble transferrin receptor (sTfR) is a product of the TfR that circulates in plasma, its concentration therefore, is proportional to the total concentration of cellular TfR.