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Ectrodactyly-ectodermal
dysplasia-cleft syndrome
Ectrodactyly
• A.K.A. lobster claw syndrome
• “derived from the Greek ektroma (abortion)
  and daktylos             (finger) = literally,
                 abortion (of a) finger.”
   (medterms.com)
• rare deformation that                   happens
  during the                pregnancy
• It can be a morphogenesis(process that
  chooses the shape of the fetus)Or a
  chromosomal deformity since the time of
  fertility
• It is caused by a dominant mutant allele
• Only happens in hands or feet with effects in
  other parts of the body
• Deformation in the ectoderm layer
• Can happen with no family history
• Can suffer from Ectodermal
  Dysplasia(irregluar skin          and hair
  glands) at the same time
• Also with a Cleft Lip/Palate
• Hypopigmentation of the skin and hair
• Abnormalities in hair, lip, tooth and palate
Consequences
• Physical challenge
• Social isolated

• Problems in many parts of the body some are in the
   – Skin
      • dry skin
      • lack of sweat pores
      • thined skin
      • thickened skin
– Hair
   • dry, brittle hair
   • generalized depigmentation of
     hair
   • fine hair


– Eyes
   • nasolacrimal duct obstruction
     (tear duct obstruction)
   • excessive lacrimation (tears)
   • corneal ulcers and scarring
   • photophobia
Diagnosis
• DNA tests can help
• Ectoderm examinations( not that precise
  because of the overlap in symptoms)
• Kidney structure abnormality
• X rays help view the abnormalities
• Skin biopsy( sweat glands not normal in shape
  and size, skin cells abnormal)
Treatment and Management
•   No cure
•   Surgery
•   Dental Care
•   Prevention with abnormalities in
    hypohydrosis( over sweating)
Examples (Videos)
•   http://www.youtube.com/watch?v=XP4HPj4-EU
•   http://wtfwear.blogspot.com/2007/04/entire-po
•
• http://en.wikipedia.org/wiki/Ectrodactyly
• http://www.medterms.com/script/main/art.asp?articlekey=13613
• http://specialchildren.about.com/od/diagnosisindex/g/ectrodactyly.h
• http://medical-dictionary.thefreedictionary.com/EEC+syndrome
• http://www.healthline.com/galecontent/ectrodactyly-ectodermal-dys
• http://images.google.com/imgres?
  imgurl=http://dermatology.cdlib.org/124/case_reports/dysplasi
  a/1.jpg&imgrefurl=http://dermatology.cdlib.org/124/case_repo
  rts/dysplasia/sankhyan.html&usg=__5rqqYa7_aiP3_RgyXRW
  Hr71VfhM=&h=559&w=512&sz=53&hl=en&start=17&tbnid
  =ScDfdqJaZBJR_M:&tbnh=133&tbnw=122&prev=/images
  %3Fq%3DEctrodactyly%26gbv%3D2%26hl%3Den
• www.google.com

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Student Work Ectrodactyly

  • 2. Ectrodactyly • A.K.A. lobster claw syndrome • “derived from the Greek ektroma (abortion) and daktylos (finger) = literally, abortion (of a) finger.” (medterms.com) • rare deformation that happens during the pregnancy
  • 3. • It can be a morphogenesis(process that chooses the shape of the fetus)Or a chromosomal deformity since the time of fertility • It is caused by a dominant mutant allele • Only happens in hands or feet with effects in other parts of the body
  • 4. • Deformation in the ectoderm layer • Can happen with no family history • Can suffer from Ectodermal Dysplasia(irregluar skin and hair glands) at the same time
  • 5. • Also with a Cleft Lip/Palate • Hypopigmentation of the skin and hair • Abnormalities in hair, lip, tooth and palate
  • 6. Consequences • Physical challenge • Social isolated • Problems in many parts of the body some are in the – Skin • dry skin • lack of sweat pores • thined skin • thickened skin
  • 7. – Hair • dry, brittle hair • generalized depigmentation of hair • fine hair – Eyes • nasolacrimal duct obstruction (tear duct obstruction) • excessive lacrimation (tears) • corneal ulcers and scarring • photophobia
  • 8. Diagnosis • DNA tests can help • Ectoderm examinations( not that precise because of the overlap in symptoms) • Kidney structure abnormality • X rays help view the abnormalities • Skin biopsy( sweat glands not normal in shape and size, skin cells abnormal)
  • 9. Treatment and Management • No cure • Surgery • Dental Care • Prevention with abnormalities in hypohydrosis( over sweating)
  • 10. Examples (Videos) • http://www.youtube.com/watch?v=XP4HPj4-EU • http://wtfwear.blogspot.com/2007/04/entire-po •
  • 11. • http://en.wikipedia.org/wiki/Ectrodactyly • http://www.medterms.com/script/main/art.asp?articlekey=13613 • http://specialchildren.about.com/od/diagnosisindex/g/ectrodactyly.h • http://medical-dictionary.thefreedictionary.com/EEC+syndrome • http://www.healthline.com/galecontent/ectrodactyly-ectodermal-dys • http://images.google.com/imgres? imgurl=http://dermatology.cdlib.org/124/case_reports/dysplasi a/1.jpg&imgrefurl=http://dermatology.cdlib.org/124/case_repo rts/dysplasia/sankhyan.html&usg=__5rqqYa7_aiP3_RgyXRW Hr71VfhM=&h=559&w=512&sz=53&hl=en&start=17&tbnid =ScDfdqJaZBJR_M:&tbnh=133&tbnw=122&prev=/images %3Fq%3DEctrodactyly%26gbv%3D2%26hl%3Den • www.google.com