Relationship between Bone Mineral Density and Selected Parameters of Calcium-Phosphate Economy with Dietary Management and Metabolic Control in Polish Pediatric Patients with Classical Homocystinuria-A Preliminary Study.

BACKGROUND: Classical homocystinuria (HCU) is an inborn defect of methionine metabolism caused by a deficiency of the enzyme cystathionine ß-synthase (CBS). The main symptoms of classical homocystinuria are lens subluxation, bone lesions, vascular disease and developmental delay/intellectual disability. The treatment method for HCU is a methionine-poor diet supplemented with amino acid preparations. The aim of the study was to examine the relationship of dietary factors, metabolic compensation and selected skeletal parameters in patients with HCU. METHODS: Bone mineral density measurements (DXA) were performed in pediatric patients with HCU, and blood levels of selected amino acids, minerals and vitamins, as well as dietary nutritional value, were analyzed. RESULTS: A total of 11 patients with HCU whose median age was 9.3 years were enrolled in the study. The median DXA total body less head of HCU patients was -0.4 z-score, and the lumbar spine was -1.4 z-score. Despite supplementation, calcium intake was below the age norm. Average vitamin D3 intake was in line with recommendations, but 36% of patients had reduced blood levels. Bone mineral density depended on blood levels of 25-hydroxyvitamin D, homocysteine and methionine, as well as on BMI, age and intake of natural protein (R2 = 98.5%, p = 0.015; R2 = 86.7%, p = 0.0049) and protein from an amino acid preparation (r = 0.69, p = 0.026). CONCLUSION: The results of the study indicate the need for regular densitometry in patients with HCU and also the use of additional calcium and vitamin D3 supplementation. It is also necessary to perform a comprehensive analysis of the diet and metabolic controls.

[Influence of oxidative stress on tissue regeneration]. / Wpllyw stresu oksydacyjnego na procesy regeneracji tkankowej.

The redox reactions involving oxidants and antioxidants play a key role in many biochemical pathways and cellular biosynthesis and regulation processes at the molecular level. Normally, there is the balance between oxidative and reductive processes in the organism. If oxidative processes are prevalent, it will result in oxidative stress. It leads to damage of many biological structures, resulting in impairment of many processes at the molecular level. Lipids and proteins are most sensitive to oxidative stress structures. Lipid oxidation can result in degradation of the cell membrane, whereas the oxidation of proteins can result in a change of their properties. It is suggested that oxidative processes accompany inflammation and affect their growth. This situation can lead to a number of serious diseases such as tumors. Many scientists claim that oxidative stress causes many pathological conditions. Enzymes and antioxidants, which are one of the body's defenses, can prevent oxidative processes.