Embryonic Disorganization Syndrome

The syndrome of the embryonic disorganization or English Human Disorganization Syndrome is a very rare malformation syndrome characterized by congenital defects, reminiscent of an amniotic ligament syndrome , associated with hamartous skin changes, supernumerary and ectopic limbs and double arrangement of internal organs.

The abnormalities can affect multiple parts of the body and all three cotyledons . The point in time of the damage lies in the blastogenesis , in which midline structures and extremities are created.

The syndrome should be considered in the presence of multiple malformations that do not fit a known clinical picture such as syndrome or sequence.

literature

OG Vallejo, M. d. Benítez Sánchez, CS Cánovas, JD Ontiveros, JI Ruiz Jiménez, E. Bermejo-Sánchez, ML Martínez-Frías: Patient with disorganization syndrome: surgical procedures, pathology, and potential causes. In: Birth Defects Research Part A - Clinical and Molecular Teratology . Vol. 97, No. 12, December 2013, pp. 781-785, ISSN 1542-0760 . doi : 10.1002 / bdra.23203 . PMID 24307594 .

Individual evidence

^ JR Wax, MG Pinette, R. Smith, A. Cartin, S. Butler, J. Blackstone: Prenatal sonographic findings in human disorganization syndrome. In: Journal of ultrasound in medicine: official journal of the American Institute of Ultrasound in Medicine. Vol. 29, No. 2, February 2010, pp. 301-305, ISSN 1550-9613 . PMID 20103803 .
^ WN Delgado Luengo, M. Luisa Hernández Rodríguez, I. Valbuena Pirela, S. González Ferrer, P. Estrada Corona, I. Chacón Fonseca, J. Delgado Luengo, A. Morales-Machín, L. Borjas Fuentes, M. Caridad Martínez Basalo, J. Chacín: Human disorganization complex, as a polytopic blastogenesis defect: a new case. In: American journal of medical genetics. Part A. Vol. 125A, No. 2, March 2004, pp. 181-185, ISSN 1552-4825 . doi : 10.1002 / ajmg.a.20307 . PMID 14981721 .

Web links

Embryonic Disorganization Syndrome. In: Orphanet (Rare Disease Database).

[Wax-1] JR Wax, MG Pinette, R. Smith, A. Cartin, S. Butler, J. Blackstone: Prenatal sonographic findings in human disorganization syndrome. In: Journal of ultrasound in medicine: official journal of the American Institute of Ultrasound in Medicine. Vol. 29, No. 2, February 2010, pp. 301-305, ISSN 1550-9613 . PMID 20103803 .

[2] WN Delgado Luengo, M. Luisa Hernández Rodríguez, I. Valbuena Pirela, S. González Ferrer, P. Estrada Corona, I. Chacón Fonseca, J. Delgado Luengo, A. Morales-Machín, L. Borjas Fuentes, M. Caridad Martínez Basalo, J. Chacín: Human disorganization complex, as a polytopic blastogenesis defect: a new case. In: American journal of medical genetics. Part A. Vol. 125A, No. 2, March 2004, pp. 181-185, ISSN 1552-4825 . doi : 10.1002 / ajmg.a.20307 . PMID 14981721 .