title: Does phase angle correlate with hyperhomocysteinemia? A study of patients with classical homocystinuria.
abstract: The role of the phase angle in hyperhomocysteinemia has yet to be assessed. Classical homocystinuria is a rare genetic disease characterized by severe hyperhomocysteinemia, as well as increased levels of methionine and reduced levels of cysteine. The objective of this study was to investigate the potential relationship between phase angle and homocysteine, cysteine, and methionine levels in patients with classical homocystinuria. Eight patients were included in the study. Phase angle was measured with a tetrapolar bioimpedance analyzer. Serum homocysteine, cysteine, and methionine levels were measured by HPLC. Only three patients had adequate metabolic control of their disease. Median phase angle was 5.9° (range = 5.4°-8.5°). There was a significant correlation between phase angle and levels of homocysteine (r = -0.807, p = 0.015), methionine (r = -0.711, p = 0.048), and cysteine (r = 0.836, p = 0.010). Was also positively correlated with BMI and arm muscle circumference (p < 0.05). Two patients had phase angles below the 5th percentile, and only one above the 50th percentile. Our findings suggest that cellular integrity is affected in patients with high homocysteine levels, thus indicating that phase angle could be a valuable indicator of prognosis and classical homocystinuria. It also suggests a role for this indicator in other forms of hyperhomocysteinemia and other inborn errors of metabolism.
authors.affiliation: Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Brazil.
authors.forename: Soraia
authors.initials: S
authors.lastname: Poloni
authors.forename: Ingrid D
authors.initials: ID
authors.lastname: Schweigert Perry
authors.forename: Vânia
authors.initials: V
authors.lastname: D'Almeida
authors.forename: Ida V D
authors.initials: IV
authors.lastname: Schwartz
mesh_terms: D000293:Adolescent; D000328:Adult; D003545:Cysteine / Q000097:blood; D005260:Female; D006710:Homocysteine / Q000097:blood; D006712:Homocystinuria / Q000097:blood* / Q000175:diagnosis*; D006801:Humans; D020138:Hyperhomocysteinemia / Q000097:blood*; D008017:Life Expectancy; D008297:Male; D008715:Methionine / Q000097:blood; D011379:Prognosis; D055815:Young Adult
chemical_list: D006710:Homocysteine; D008715:Methionine; D003545:Cysteine