O.N. MOSKALJUK, A.V. NALYOTOV 

1. Gorky Donetsk State Medical University, Donetsk, Donetsk People’s Republic

Contact details:

Nalyotov A.V. — MD, Professor, Head of the Department of Pediatrics No. 2

Address: 16 prospekt Ilyicha, Donetsk, Donetsk People`s Republic, Russian Federation, 283003, tel.: +7-949-311-13-82, e-mail: nalyotov-a@mail.ru

 The purpose — to study the content of amino acids in the blood and urine of children with undifferentiated connective tissue dysplasia, having spine and chest deformities.

Material and methods. 81 children with undifferentiated connective tissue dysplasia aged 7–17 years were examined. The main group consisted of 49 children with spinal and thoracic deformities; the comparison group consisted of 32 children without spinal and thoracic deformities. The state of the free amino acid pool was assessed by thin-layer chromatography. The blood content and daily urinary excretion of 22 free amino acids and their derivatives were determined.

Results. We found that in children with undifferentiated connective tissue dysplasia, changes in the content of proline in the blood were most common in 73 (90.1%) and hydroxyproline in 63 (77.8%) patients. In patients with spinal and thoracic deformities, the content of all 22 amino acids in the blood was changed; among children without spinal and thoracic deformities, the concentration of 11 amino acids was changed. Changes in the content of amino acids in the urine of children with undifferentiated connective tissue dysplasia were detected more often than changes in their content in the blood. At the same time, there were changes in the content of all amino acids in urine among patients. Most often, in children with undifferentiated connective tissue dysplasia, changes in the content of proline were recorded in urine in 73 (90.1%) and hydroxyproline in 65 (80.3%) patients. Less frequently, there was a change in the content of lysine — in 49 (49.4%), ornithine — in 32 (39.5%), phenylalanine — in 29 (35.8%). In children with deformities of the spine and chest, the altered content of amino acids in urine was more common than in patients without deformities.

Conclusions. The revealed changes in the content of amino acids in the blood and urine of children with undifferentiated connective tissue dysplasia reflected a violation of their metabolism in the structural components of connective tissue, which led to a violation of its structure and functions. The formation of deformities of the spine and chest can be considered as a clinical manifestation of deep biochemical disorders of connective tissue metabolism in children with undifferentiated connective tissue dysplasia.

Key words: amino acids, undifferentiated connective tissue dysplasia, spinal and thoracic deformities, children.

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