Sudden infant death syndrome with Harlequin fetus

Harlequin fetus ihtiyozisin şiddetli bir varyantı olup, bu hastalar genellikle yaşamın ilk günlerinde kaybedilirler. Erken retinoid tedavisi iyileşme ve yaşama oranını arttırabilir. Ani bebek ölümü sendromu genellikle sağlıklı görünen, sabahları yataklarında ölmüş olarak bulunan ve ölüm nedeni saptanamayan bebeklerdir. Yaklaşık %0.1-0.3 oranında görülür. Bu olgu raporunda harlequin fetus ile birlikte ani bebek ölümü sendromu olabileceği düşünülen bir olgu sunulmaktadır.

Harlequin fetus ile ani bebek ölümü sendromu birlikteliği

The harlequin fetus, a severe variant of ichthyosis, occurs rarely, and these babies die within the first few days of life. Early retinoid therapy may improve the disorder and help increase survival rates. The exact cause of the sudden infant death syndrome of the suckling is not known and the incidence approximately is 0.1-0.3 %. In general, these babies looked well and healthy at the time of the sleeping but were found dead in their bed in the morning. We report a harlequin fetus with sudden infant death syndrome.

___

  • 1. Akiyama M. Harlequin ichthyosis and other autosomal recessive congenital ichthyoses: the underlying genetic defects and pathomechanisms. J Dermatol Sci 2006; 42: 83-89.
  • 2. Akiyama M. The pathogenesis of severe congenital ichthyosis of the neonate. J Dermatol Sci 1999; 21: 96-104.
  • 3. Chan YC, Tay YK, Ton LKS, et al. Harlequin ichthyosis in association with hypothyroidism and juvenile rheumatoid arthritis. Pediatr Dermatol 2003; 20: 421-426.
  • 4. Shwayder T, Out F. All about ichthyosis. Pediatr Clin North Am 1991; 38: 835-857.
  • 5. Roberts LJ. Long term survival of a harlequin fetus. J Am Acad Dermatol 1989; 16: 553-569.
  • 6. Guneş T, Akçakuş M, Kurtoğlu S, et al. Harlequin fetus with ecthyma gangrenosum. Pediatr Dermatol 2003; 20: 529-530.
  • 7. Singb S, Bbura M, Mabesbwari A, et al. Succesful treatment of harlequin ichthyosis with acitretin. Int J Dermatol 2001; 40: 472- 484.
  • 8. Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI. An appraisal of acitretin therapy in children with inherited disorders of keratinisation. Br J Dermatol 1996; 134: 1023- 1029.
  • 9. Hay WW, Hayward AR, Lewin M, Sondheimer JM. Current Pediatrics. 16th edition. New York: McGraw-Hill Companies 2003; 540-541.