Table 2 Sex hormone effects on metabolism

Estrogen

Increases total cholesterol, triglycerides, and high-density lipoproteins, decreases low-density lipoproteins

Cholesterol biosynthesis disorders, Cholesterol storage disorders, GSD

Winkler et al. [48], Nash et al. [49], Ruoppolo et al. [50]

Increases circulating glucose and insulin

FAOD, GSD, disorders with risk of hypoglycemia

Winkler et al. [48], Godsland et al. [51]

Decreases muscle glucose uptake via repression of GLUT4 expression

Muscle-predominant GSD

Barros et al. [52]

Decreased absorption of B vitamins, specifically riboflavin, thiamine, and pyridoxine

PDH deficiency, Homocystinuria

Anderson et al. [53], Webb et al. [54], Rose et al. [55]

Increased risk of venous thromboembolism

Homocystinuria, Cobalaminopathies

Den Heijer et al. [23]

Progestins

Increase triglycerides and high-density lipoproteins, decreases low-density lipoprotein

Cholesterol biosynthesis disorders, Cholesterol storage disorders, GSD

Godsland et al. [51], Butler et al. [56]

Decrease bone mineral density with DMPA

Disorders with risk for poor bone health

ACOG [4]

Combined estrogen and progestin

Changes in plasma amino acid profiles: decreases glutamine, glycine, proline, lysine, hydroxyproline, ornithine, tyrosine and increases isoleucine and phenylalanine

PKU, IVA, GAMT deficiency, Lysinuric Protein Intolerance

Ruoppolo et al. [50], Wang et al. [57]

Increase total cholesterol, triglycerides, and high-density lipoproteins

Cholesterol biosynthesis disorders, Cholesterol storage disorders, GSD

Wang et al. [57]

Decrease free and total carnitine

Carnitine Uptake deficiency, Secondary carnitine deficiency

Bach et al. [58]

Increase cholic acid

Disorders of bile acid metabolism

Connolly et al. [44]

Decrease chenodeoxycholic acid

Cerebrotendinous Xanthomatosis

Connolly et al. [44]

Increase ceruloplasmin

Wilson disease

Roberts et al. [59]