Skip to main content
NCBI home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1970 Aug;45(242):553–560. doi: 10.1136/adc.45.242.553

Progeria

Case Report with Long-term Studies of Serum Lipids

B G P Machamara, K T Farn, A K Mitra, June K Lloyd, Audrey S Fosbrooke
PMCID: PMC1647660  PMID: 5506943

Abstract

A girl with the typical features of progeria died at the age of 10 years from cardiac failure following repeated myocardial infarctions. Serial studies of the serum lipids were made from the age of 2 years 3 months. Variable hyperlipidaemia, increased β- and pre-β-lipoproteins, and impaired clearing of absorbed dietary fat were shown. A polyunsaturated fat diet, which was started at the age of 2 years 5 months and continued throughout the rest of her life, controlled the hyperlipidaemia and the clearing of fat became normal. Pre-β-lipoprotein could still, however, be seen occasionally. The dietary regimen did not delay the development of atherosclerosis, the pathogenesis of which, in progeria, may be due to factors other than hyperlipidaemia.

Full text

PDF
553

Images in this article

554FIG. 1.
on p.554
555FIG. 2.
on p.555
557FIG. 4.
on p.557
558FIG. 6.
on p.558

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. BRONSTEIN I. P., DALLENBACH F. D., PRUZANSKY S., ROSENTHAL I. M., ROSENWALD A. K. Progeria; report of a case with cephalometric roentgenograms and abnormally high concentrations of lipoproteins in the serum. Pediatrics. 1956 Oct;18(4):565–577. [PubMed] [Google Scholar]
  2. Bhakoo O. N., Garg S. K., Sehgal V. N. Progeria with unusual ocular manifestations: report of a case with a review of the literature. Indian Pediatr. 1965 May;2(5):164–169. [PubMed] [Google Scholar]
  3. CORNWELL D. G., KRUGER F. A., HAMWI G. J., BROWN J. B. Studies on the characterization of human serum lipoproteins separated by ultracentrifugation in a density gradient. I. Serum lipoproteins in normal, hyperthyroid and hypercholesterolemic subjects. Am J Clin Nutr. 1961 Jan-Feb;9:24–40. doi: 10.1093/ajcn/9.1.24. [DOI] [PubMed] [Google Scholar]
  4. DE LA HUERGA J., YESINICK C., POPPER H. Estimation of total serum lipids by a turbidimetric method. Am J Clin Pathol. 1953 Nov;23(11):1163–1167. doi: 10.1093/ajcp/23.11_ts.1163. [DOI] [PubMed] [Google Scholar]
  5. DOLE V. P., MEINERTZ H. Microdetermination of long-chain fatty acids in plasma and tissues. J Biol Chem. 1960 Sep;235:2595–2599. [PubMed] [Google Scholar]
  6. FERNANDES J., van de KAMER J. H., WEIJERS H. A. Differences in absorption of the various fatty acids studied in children with steatorrhea. J Clin Invest. 1962 Mar;41:488–494. doi: 10.1172/JCI104502. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Fosbrooke A. S., Rudd B. T. The determination of particulate fat in serum and its use in clinical studies. Clin Chim Acta. 1966 Feb;13(2):251–258. doi: 10.1016/0009-8981(66)90301-9. [DOI] [PubMed] [Google Scholar]
  8. GHOSH S., VARMA K. P. PROGERIA: REPORT OF A CASE WITH REVIEW OF THE LITERATURE. Indian Pediatr. 1964 Apr;1:146–155. [PubMed] [Google Scholar]
  9. Goldstein S. Lifespan of cultured cells in progeria. Lancet. 1969 Feb 22;1(7591):424–424. doi: 10.1016/s0140-6736(69)91404-4. [DOI] [PubMed] [Google Scholar]
  10. KEAY A. J., OLIVER M. F., BOYD G. S. Progeria and atherosclerosis. Arch Dis Child. 1955 Oct;30(153):410–414. doi: 10.1136/adc.30.153.410. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Kaiman H., Lambie R. W., Metzl K. Progeria. Case description. Clin Pediatr (Phila) 1969 Jul;8(7):411–415. doi: 10.1177/000992286900800716. [DOI] [PubMed] [Google Scholar]
  12. LLOYD J. K., JUKES H. R. Diet rich in unsaturated fat. Lancet. 1961 Feb 11;1(7172):312–313. doi: 10.1016/s0140-6736(61)91480-5. [DOI] [PubMed] [Google Scholar]
  13. MOSTAFA A. H., GABR M. Heredity in progeria; with follow-up of two affected sisters. Arch Pediatr. 1954 Jun;71(6):163–172. [PubMed] [Google Scholar]
  14. Macleod W. Progeria. Br J Radiol. 1966 Mar;39(459):224–226. doi: 10.1259/0007-1285-39-459-224. [DOI] [PubMed] [Google Scholar]
  15. NESTEL P. J. RELATIONSHIP BETWEEN PLASMA TRIGLYCERIDES AND REMOVAL OF CHYLOMICRONS. J Clin Invest. 1964 May;43:943–949. doi: 10.1172/JCI104980. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. SALT H. B., WOLFF O. H. The applications of serum lipoprotein electrophoresis in paediatric practice. Arch Dis Child. 1957 Oct;32(165):404–412. doi: 10.1136/adc.32.165.404. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. THOMSON J., FORFAR J. O. Progeria (Hutchinson-Gilford syndrome). Report of a case and review of the literature. Arch Dis Child. 1950 Sep;25(123):224–234. doi: 10.1136/adc.25.123.224. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Villee D. B., Nichols G., Jr, Talbot N. B. Metabolic studies in two boys with classical progeria. Pediatrics. 1969 Feb;43(2):207–216. [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES