Antioxidant defense of children and adolescents with atopic dermatitis: Association with disease severity

Background: Atopic dermatitis (AD) is a relapsing, chronic cutaneous inflammatory disease with onset, in general, in early childhood. Chronic skin inflammation is associated with overproduction of reactive oxygen species (ROS) such as superoxide and hydrogen peroxide. Oxidative stress, an imbalance between the production of free radicals and antioxidant defense, results in tissue inflammation due to the upregulation of genes that encode inflammatory cytokines. This condition plays an important role in the pathogenesis of AD. Objective: To compare the antioxidant defense in children and adolescents with AD with that of healthy individuals and to verify the association of antioxidant defense with disease severity and nutritional status. Methods: Cross-sectional study that evaluated 48 children and adolescents with AD and 25 controls for nutritional assessment (body mass index z score [BMIZ] and height for age z score [HAZ]) and levels of vitamins A, C, E, and D, zinc (Zn), copper (Cu), antioxidant enzymes (superoxide dismutase [SOD], catalase [CAT], glutathione peroxidase [GPx]), high-sensitivity C-reactive protein (CRP) and interleukin 33 (IL-33). Results: There was no significant difference in the comparison between AD and control groups for serum levels of vitamins (A, D, C, and E), copper, and antioxidant enzymes. Serum zinc levels were higher in the AD group (β = 24.20; 95% CI 13.95–34.91; P < 0.001) even after adjusting the BMIZ, HAZ, gender, IL-33, and CRP. Children and adolescents with moderate or severe AD compared to mild AD (SCORAD – 36.7±17.4 vs 11.8 ± 3.9; P < 0.001) had lower values of the vitamin E/total lipid ratio (3.68 [0.29;12.63] vs 5.92 [3.27;17.37]; P = 0.013) (AU)

Antioxidant defense of children and adolescents with atopic dermatitis: Association with disease severity.

BACKGROUND: Atopic dermatitis (AD) is a relapsing, chronic cutaneous inflammatory disease with onset, in general, in early childhood. Chronic skin inflammation is associated with overproduction of reactive oxygen species (ROS) such as superoxide and hydrogen peroxide. Oxidative stress, an imbalance between the production of free radicals and antioxidant defense, results in tissue inflammation due to the upregulation of genes that encode inflammatory cytokines. This condition plays an important role in the pathogenesis of AD. OBJECTIVE: To compare the antioxidant defense in children and adolescents with AD with that of healthy individuals and to verify the association of antioxidant defense with disease severity and nutritional status. METHODS: Cross-sectional study that evaluated 48 children and adolescents with AD and 25 controls for nutritional assessment (body mass index z score [BMIZ] and height for age z score [HAZ]) and levels of vitamins A, C, E, and D, zinc (Zn), copper (Cu), antioxidant enzymes (superoxide dismutase [SOD], catalase [CAT], glutathione peroxidase [GPx]), high-sensitivity C-reactive protein (CRP) and interleukin 33 (IL-33). RESULTS: There was no significant difference in the comparison between AD and control groups for serum levels of vitamins (A, D, C, and E), copper, and antioxidant enzymes. Serum zinc levels were higher in the AD group (ß = 24.20; 95% CI 13.95-34.91; P < 0.001) even after adjusting the BMIZ, HAZ, gender, IL-33, and CRP. Children and adolescents with moderate or severe AD compared to mild AD (SCORAD - 36.7±17.4 vs 11.8 ± 3.9; P < 0.001) had lower values of the vitamin E/total lipid ratio (3.68 [0.29;12.63] vs 5.92 [3.27;17.37]; P = 0.013). CONCLUSION: Children and adolescents with AD had higher concentrations OF elevated levels of zinc compared to controls, a fact not observed for other biomarkers of antioxidant defense. AD in moderate or severe forms presented lower concentrations of vitamin E, a potent antioxidant fat soluble.

Molecular characterization of phenylketonuria patients from the North Region of Brazil: State of Pará.

BACKGROUND: Phenylketonuria (PKU) is an autosomal recessive disease resulting from a deficiency of the enzyme phenylalanine hydroxylase (PAH). Hyperphenylalaninemias (HPA) due to PAH deficiency are accompanied by a wide variety of clinical, biochemical, and molecular features. To identify and characterize pathogenic variants in the PAH gene and establish a correlation between genotype and biochemical phenotype in patients with PKU from state of Pará in the North Region of Brazil. METHODS: All 13 exons of the PAH gene from 32 patients (21 PKU and 11 non-PKU HPA) were amplified by PCR and submitted to DNA sequencing (Sanger). Biochemical data were obtained from the patients' medical records. RESULTS: Molecular analysis identified 17 pathogenic variants and 3 nonpathogenic variants. The most frequent pathogenic variants were IVS10-11G>A (7.9%), p. Arg261Gln (7.9%), p. Val388Met (6.3%) and p. Ile65Thr (4.7%). Was observed correlations and inconsistencies between genotype and biochemical phenotype. CONCLUSION: In PKU patients from state of Pará, North Region of Brazil, a heterogeneous mutation spectrum was revealed, in which the most frequent mutations are variants commonly observed in other Brazilian studies and in the region of the Iberian Peninsula.

Uso de leite processado em altas temperaturas por paciente com alergia ao leite de vaca - relato de caso / Use of milk processed at high temperatures by a patient with cow's milk allergy: case report

Objetivo: Relatar a evolução clínica de um escolar com alergia ao leite de vaca (ALV) que fez uso de leite de vaca processado em altas temperaturas (LVPAT). Descrição: H.B.M., sexo masculino, 7 anos, com ALV IgE mediada diagnosticada com 1 ano e 3 meses. Aos 2 anos foi submetido a teste de provocação oral (TPO) aberto para leite de vaca (LV) in natura, evoluindo com urticária, congestão nasal e vômito após a primeira dose (1 mL). Mãe relatou alguns episódios de exposição acidental ao LV acompanhados de sintomas. As Imunoglobulinas E para LV e frações mantiveram-se elevadas (IgE leite total: 4,69 KU/L) até os 6 anos, quando a criança realizou TPO com LVPAT, sob a forma de bolo, evoluindo sem intercorrências. Passou a consumir diariamente uma porção do bolo contendo leite processado durante 6 meses. Aos 7 anos e com IgEs específicas mais baixas (IgE específica leite total: 2,2 KU/L), realizou TPO com LV in natura sem sintomas, sendo liberado na dieta. Comentários: O uso do leite de vaca processado em altas temperaturas em pacientes com ALV IgE mediada é uma estratégia promissora com impacto na tolerância futura ao alimento, tendo resultados favoráveis com ênfase na qualidade de vida e inclusão social. No entanto, vale ressaltar a importância da avaliação individualizada dos pacientes e a segurança da equipe na aplicação desses protocolos, além de levar em consideração que a alergia pode ser transitória, mesmo sem o uso do leite processado.

Objective: To report the clinical evolution of a school-age boy with cow's milk allergy CMA) who made use of cow's milk processed at high temperatures (CMPHT). Description: H.B.M., male, 7 years old, was diagnosed with IgE-mediated CMA at 1 year and 3 months of age. At 2 years of age, the patient underwent an open oral food challenge for raw cow's milk (CM) and developed urticaria, nasal congestion and vomiting after the first dose (1 mL). The mother reported some episodes of accidental exposure to CM, accompanied by symptoms. Specific IgEs against CM and its fractions remained elevated (total cow's milk: 4.69 KU/L) until 6 years of age, when the patient underwent a new oral food challenge for CMPHT, in the form of a cake, without symptoms. He then began to consume a portion of cake containing baked milk daily, for 6 months. At the age of 7 and with lower levels of specific IgEs (total cow's milk: 2.2 KU/L), he underwent another oral food challenge for raw CM, without symptoms. An unrestricted diet was then authorized. Comments: The use of cow's milk processed at high temperatures in patients with IgE-mediated cow's milk allergy is a promising strategy, with impact on future food tolerance, favorable results, with emphasis on quality of life and social inclusion. However, an individualized assessment of each patient is extremely important, as is the confidence of the team while applying these protocols. It should be taken into consideration that the allergy may be transient even without the use of processed milk.

Phenylalanine and Tyrosine Metabolism Analysis in Heterozygotes for Phenylketonuria and in Healthy Individuals

Abstract Phenylketonuria (PKU) is an inherited metabolic disorder derived from a deficiency in the enzyme phenylalanine hydroxylase, which converts the amino acid phenylalanine (Phe) into tyrosine (Tyr). Here we aimed to examine the metabolism of Phe and Tyr in heterozygotes for PKU during fasting and after oral overload of Phe (25 mg/kg). Plasma concentration of Phe and Tyr and Phe2-Tyr ratio were determined under fasting condition or 30, 45, 60, and 90 minutes after Phe overload. The sample consisted of 50 participants: 23 heterozygotes for PKU (10 men and 13 women) and a control group of 27 healthy individuals (13 men and 14 women). The dosage of Phe at 45 and 90 minutes and the micromolar fraction of Phe2/Tyr after 90 minutes of overload efficiently differentiated PKU heterozygotes. The discriminant function revealed 86% of accuracy. In fact, 94.4% of heterozygotes for PKU were correctly classified.