Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
JCR Impact Factor (IF) – 1.736
5-Year Impact Factor – 2.135
Index Copernicus  – 168.52
MEiN – 70 pts

ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
Periodicity – monthly

Download original text (EN)

Advances in Clinical and Experimental Medicine

2017, vol. 26, nr 3, May-June, p. 515–525

doi: 10.17219/acem/62132

Publication type: review article

Language: English

Download citation:

  • BIBTEX (JabRef, Mendeley)
  • RIS (Papers, Reference Manager, RefWorks, Zotero)

Energy exchangers with LCT as a precision method for diet control in LCHADD

Renata Mozrzymas1,A,B,C,D, Klaudia Konikowska2,A,B,C,D, Bożena Regulska-Ilow2,E,F

1 Regional Specialist Hospital in Wrocław, Research and Development Center, Poland

2 Department of Dietetics, Wroclaw Medical University, Poland


Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare genetic disease. The LCHADD treatment is mainly based on special diet. In this diet, energy from long-chain triglycerides (LCT) cannot exceed 10%, however energy intake from the consumption of medium-chain triglycerides (MCTs) should increase. The daily intake of energy should be compatible with energy requirements and treatment should involve frequent meals including during the night to avoid periods of fasting. In fact, there are no recommendations for total content of LCT in all of the allowed food in the LCHADD diet. The aim of the study was to present a new method of diet composition in LCHADD with the use of blocks based on energy exchangers with calculated LCT content. In the study, the diet schema was shown for calculating the energy requirements and LCT content in the LCHADD diet. How to create the diet was also shown, based on a food pyramid developed for patients with LCHADD. The blocks will make it possible, in a quick and simple way, to create a balanced diet which provides adequate energy value, essential nutrients and LCT content. This method can be used by doctors and dietitians who specialize in treating rare metabolic diseases. It can also be used by patients and their families for accurate menu planning with limited LCT content.

Key words

diet, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD), long-chain triglycerides (LCT), intake of energy

References (38)

  1. Vallisneri A, 1721. Cited in Insler V, Lunesfeld B. Polycystic ovarian disease: A challenge and controversy. Gynecol Endocrinol. 1990;4:51-69.
  2. Chereau, Achilles. Memoires pour Servir a l’Etude des Maladies des Ovaries. Paris: Fortin, Masson & Cie; 1844.
  3. Rokitansky C. A Manual of Pathological Anatomy – Vol II. Philadelphia: Blanchard & Lea; 1855, 246.
  4. Bulius G, Kretschmar C. Angiodystrophia. Stuttgart: Verlag von Ferdinand Enke; 1897.
  5. Tait L. Removal of normal ovaries. Br Med J. 1879;813:284.
  6. Martin A. Ergebnisse der Ovarien und Tubenresektion. Verhandl Dtsch Ges Gynak. 1891;4:242–257.
  7. von Kahlden C. Über die kleincystische Degenerationder Ovarien und ihre Beziehungen zu den sogenannten Hydrops follicul. In: Ziegler E, ed. Beitrage zur pathologischen Anatomie und zur allgemeinen Pathologie. Jena, Germany: Verlag von Gustav Fischer; 1902:1–102.
  8. McGlinn JA. The end results of resection of the ovaries for microcystic disease. Am J Obstst Dis Women Child. 1916;73:435–439.
  9. Stein IF, Leventhal ML. Amenorrhoea associated with bilateral polycystic ovaries. Am J Obstet Gynecol. 1935;29:181–191.
  10. Stein IF, Cohen MR, Elson RE. Results of bilateral ovarian wedge resection in 47 cases of sterility. Am J Obstet Gynecol. 1948;58:267–273.
  11. Greenblatt RB. Chemical induction of ovulation. Fertil Steril. 1961;12:402–404.
  12. Kovacs GT, Pepperell RJ, Evans JH. Induction of ovulation with human pituitary gonadotrophin (HPG): the Australian experience. Austral NZ J Med. 1989;29:315–318.
  13. Wang CF, Gemzell C. The use of human gonadotrophins for induction of ovulation in women with polycystic ovarian disease. Fertil Steril. 1980;33:479–486.
  14. Geist SH, Gains JA. Diffuse luteinization of the ovaries associated with the masculinization syndrome. Am J Obstet Gynecol. 1942;43:975–983.
  15. Culiner A, Shippel S. Virilism and theca-cell hyperplasia of the ovary; a syndrome. J Obstet Gynaecol Br Emp. 1949;56:439–445.
  16. Forgue E, Massabuau G. L’ovarie a petits kystes (cont.). Rev Gynecol Chirurg Abdom. 1910;14:209–284.
  17. Yen SSC, Vela P, Rankin J. Inappropiate secretion of follicle-stimulating hormone and luteinizing hormone in polycystic ovarian disease. J Clin Endocrinol Metab. 1970;30:435–442.
  18. Hill HT. Ovaries secrete male hormones: I. Restoration of the castrate type seminal vesicle and prostate gland to normal by grafts of ovaries in mice. Endocrinology. 1937;21:495–502.
  19. Deanesly R. The androgenic activity of ovarian grafts in castrated male rats. Proc R Soc Lond B Biol Sci. 1938;126:122–135.
  20. Plate WP. Hirsutism in ovarian hyperthecosis. Acta Endocrinol (Copenh). 1951;8:17–32.
  21. Jones GE, Howard JE, Langford H. The use of cortisone in follicular phase disturbances. Fertil Steril. 1953;4:49–62.
  22. Greenblatt RB. Cortisone in treatment of hirsute women. Am J Obstet Gynecol. 1953;66:700–710.
  23. Netter MA, Lambert A. Therapeutique medicale de l’ovarite sclero-kystique. C R Soc Fr Gyncol. 1954;24:78–81.
  24. McArthur JW, Ingersoll FW, Worcester J. The urinary excretion of interstitial-cell and follicle-stimulationing hormone activity by women with disease of the reproductive system. J Clin Endocrinol Metab. 1958;18:1202–1215.
  25. Axelrod LR, Goldzieher JW. The polycystic ovary. III. Steroid biosynthesis in normal and polycystic ovarian tissue. J Clin Endocrinol Metab. 1962;22:431–440.
  26. Rebar R, Judd HL, Yen SS, Rakoff J, Vandenberg G, Naftolin F. Characterization of the inappropriate gonadotropin secretion in polycystic ovary syndrome. J Clin Endocrinol Metab. 1976;57:1320–1329.
  27. Shoupe D, Kumar DD, Lobo RA. Insulin resistance in polycystic ovary syndrome. Am J Obstet Gynecol. 1983;147:588–592.
  28. Robinson S, Rodin DA, Deacon A, Wheeler MJ, Clayton RN. Which hormone tests for the diagnosis of polycystic ovary syndrome? Br J Obstet Gynaecol. 1992;9:232–238.
  29. Stein IF, Cohen MR. Surgical treatment of bilateral polycystic ovaries – amenorrhea and sterility. Am J Obstet Gynecol. 1939;38:465–480.
  30. Dignam WJ, Pion RJ, Lamb EJ, Simmer HH. Plasma androgens in women. II Patients with polycystic ovaries and hirsutism. Acta Endocrinol (Copenh). 1964;45:254–271.
  31. Fauser BC, Pache TD, Lamberts SW, Hop WC, de Jong FH, Dahl KD. Serum bioactive and immunoreactive luteinizing hormone and follicle-stimulating hormone levels in women with cycle abnormalities, with or without polycystic ovarian disease. J Clin Endocrinol Metab. 1991;73:811–817.
  32. Gjoanness H. Polycystic ovarian syndrome treated by ovarian electrocautery through the laparoscope. Fertil Steril. 1984;41:20–25.
  33. Swanson M, Sauerbrei EE, Cooperberg PL. Medical implications of ultrasonically detected polycystic ovaries. J Clin Ultrasound. 1981;9:219–222.
  34. Fox R, Corrigan E, Thomas PA, Hull MG. The diagnosis of polycystic ovaries in women with oligo-amenorrhoea: Predictive power of endocrine tests. Clin Endocrinol (Oxf). 1991;34:127–131.
  35. Azziz R, Carmina E, Dewailly D, et al. Positions statement: Criteria for defining polycystic ovary syndrome as a predominantly hyperandrogenic syndrome: An Androgen Excess Society guideline. J Clin Endocrinol Metab. 2006;91:4237–4245.
  36. Szydlarska D, Grzesiuk W, Bar-Andziak E. Evolution of polycystic ovary syndrome. Ginekologia i Położnictwo Medical Project. 2010;4:63–68.
  37. Franks S. Diagnosis of polycystic ovary syndrome: in defense of Rotterdam criteria. J Clin Endocrinol Metab. 1991;3:786–789.
  38. The Rotterdam ESHRE/ASRM-Sponsored PCOS Consensus Workshop Group. Revised consensus on diagnostic criteria and long-term health risk related to polycystic ovary syndrome (PCOS). Hum Reprod. 2004;19:41–47.