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Hutchinson-Gilford progeria syndrome in siblings

Report of three new cases

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Abstract

The Hutchinson-Gilford progeria syndrome is a rare, inherited, pediatric condition with features of premature and accelerated aging. The pattern of inheritance is uncertain though both autosomal dominant and autosomal recessive modes have been proposed. The patients usually present after the 1st year of life with progressive skin and skeletal changes that give rise to a characteristic physical appearance. Three siblings seen at the University of Benin Teaching Hospital are described in this report, the third documenting the occurrence of progeria in African black patients. The two older siblings show the classic physical and radiologic changes described in progeria whereas the third, a 2-year-old boy, manifests only the early physical and radiologic changes of the disease. We compare the radiologic features of progeria with those of other progeroid conditions: acrogeria, Werner's and Cockayne's syndromes.

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References

  1. Alton DJ, McDonald P, Reilly BJ (1972) Cockayne syndrome. A report of three cases. Radiology 102:403

    Google Scholar 

  2. Badame JA (1989) Progeria. Arch Dermatol 125:540

    Google Scholar 

  3. Beauregard S, Gilchrest BA (1987) Syndromes of premature aging. Dermatol Clin 5:109

    Google Scholar 

  4. Brown W (1987) Premature aging syndromes. Curr Probl Dermatol 17:152

    Google Scholar 

  5. Brown WT, Little JB, Epstein J, Williams JR (1978) DNA repair defect in progeric cells. Birth Defects 14:417

    Google Scholar 

  6. Chawla V, Tawodzera PBP, Chukwu JN (1986) Progeria: report of two cases and literature review. East Afr Med J 63:749

    Google Scholar 

  7. Cockayne EA (1946) Dwarfism with retinal atrophy and deafness. Arch Dis Child 21:52

    Google Scholar 

  8. De Busk BL (1972) The Hutchinson-Gilford progeria syndrome. J Pediatr 80:697

    Google Scholar 

  9. Escalante A, Beardmore TD, Kaufman RL (1989) Musculo-skeletal manifestations of Werner's syndrome. Semin Arthritis Rheum 19:1

    Google Scholar 

  10. Feingold M, Kid R (1971) Progeria and scleroderma in infancy. Am J Dis Child 122:61

    Google Scholar 

  11. Franklyn PP (1976) Progeria in siblings. Clin Radiol 27:327

    Google Scholar 

  12. Gabr M (1954) Progeria. Review of literature with report of a case. Arch Pediatr 71:35

    Google Scholar 

  13. Gaetani SA, Ferraris AM, D'Agosta A (1988) Case report 485: Werner's syndrome. Skeletal Radiol 17:298

    Google Scholar 

  14. Goldstein S, Moerman EJ (1978) Heat labile enzyme in circulating erythrocytes of a progeria family. Am J Hum Genet 30:167

    Google Scholar 

  15. Goto MT, Kindynis P, Resnick D, Sartoris DJ (1989) Osteosclerosis of the phalanges in Werner's syndrome. Radiology 172:841

    Google Scholar 

  16. Hamer L, Kaplan F, Fallon M (1988) The musculoskeletal manifestations of progeria. A literature review. Orthopaedics 11:763

    Google Scholar 

  17. Ho A, White SJ, Rasmussen JE (1987) Skeletal abnormalities of acrogeria, a progeroid syndrome. Skeletal Radiol 16:463

    Google Scholar 

  18. Khalifa MM (1989) Hutchinson-Gilford progeria syndrome: report of a Libyan family and evidence of autosomal recessive inheritance. Clin Genet 1989:35:125

    Google Scholar 

  19. Maciel AT (1988) Evidence for autosomal recessive inheritance of progeria. Am J Med Gen 31:483

    Google Scholar 

  20. Macleod V (1966) Progeria. Br J Radiol 39:224

    Google Scholar 

  21. Margolin FB, Steinbach HL (1968) Progeria Hutchinson-Gilford syndrome. AJR 103:173

    Google Scholar 

  22. Moen C (1982) Orthopaedic aspects of progeria. J Bone J Surg [Am] 64:542

    Google Scholar 

  23. Mostafa AH, Gabr M (1954) Heredity in progeria with follow-up of two affected sisters. Arch Pediatr 71:163

    Google Scholar 

  24. Moynahan EJ (1962) Progeria (Hastings-Gilford) presenting as scleroderma in early infancy. Proc R Soc Med 55:233

    Google Scholar 

  25. Ogihara T, Hata T, Tanaka K, Fukuchi K, Tabuchi Y, Kumahara Y (1986) Hutchinson-Gilford progeria syndrome in a 45 year old man. Am J Med 81:135

    Google Scholar 

  26. Ozonoff MB, Clemett AB (1967) Progressive osteolysis in progeria. AJR 100:75

    Google Scholar 

  27. Paddison RM, Mossy J, Derbes VJ, Kloepfer W (1963) Cockayne's syndrome.A report of five new cases with biochemical, chromosomal dermatologic, genetic and neuropathologic observations. Dermatol Tropica Ecol Geogr 2:195

    Google Scholar 

  28. Ramesh V, Jain RK (1987) Progeria in two brothers. Australas J Dermatol 28:33

    Google Scholar 

  29. Rautenstrauch T, Snigula F (1977) Progeria: a cell culture study and clinical report of familial incidence. Eur J Pediatr 124:101

    Google Scholar 

  30. Riggs W, Seibert J (1972) Cockayne syndrome. Roentgen findings. AJR 116:623

    Google Scholar 

  31. Runge P, Asnis MS, Brumley GN, Grossman H (1978) Hutchinson-Gilford progeria syndrome. South Med J 71:877

    Google Scholar 

  32. Ruvalcaba RHA, Churesigaew S, Myhre SA, Kelley VC, Martin GM (1977) Children who age rapidly — progeroid syndromes. Case report of a new variant. Clin Paediatr 16:248

    Google Scholar 

  33. Soffer D, Grotsky HW, Rapin I, Suzulu K (1979) Cockayne syndrome: unusual neuropathological findings and review of the literature. Ann Neurol 6:340

    Google Scholar 

  34. Taybi H, Lachman RS (1990) Radiology of syndromes, metabolic disorders, and skeletal dysplasias. Acrogeria, 3rd edn. Year Book Medical Publishers, Chicago, pp 16

    Google Scholar 

  35. Viegas J, Souza PLR, Falzano FM (1974) Progeria in twins. J Med Genet 11:384

    Google Scholar 

  36. Villee DB, Nichols G Jr, Talbot NB (1969) Metabolic studies in two boys with classical progeria. Paediatrics 43:207

    Google Scholar 

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Authors and Affiliations

  1. Department of Radiology, University of Wisconsin Hospital and Clinics, Madison, Wisconsin, USA

    J. U. V. Monu M.D.

  2. Department of Radiology, University of Benin Teaching Hospital, Benin City, Nigeria

    J. U. V. Monu M.D. & L. B. O. Benka-Coker M.D.

  3. Department of Child Health, University of Benin Teaching Hospital, Benin City, Nigeria

    Yemisi Fatunde M.D.

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  1. J. U. V. Monu M.D.
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  2. L. B. O. Benka-Coker M.D.
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  3. Yemisi Fatunde M.D.
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Monu, J.U.V., Benka-Coker, L.B.O. & Fatunde, Y. Hutchinson-Gilford progeria syndrome in siblings. Skeletal Radiol. 19, 585–590 (1990). https://doi.org/10.1007/BF00241281

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