Literature DB >> 31250196

Ehlers-Danlos syndrome and other heritable connective tissue disorders that impact pregnancies can be detected using next-generation DNA sequencing.

Krystal VanderJagt1, Merlin G Butler2.   

Abstract

Ehlers-Danlos syndromes (EDS) are a genetically heterogeneous group of inherited connective tissue disorders classified into six major types with a variable collection of findings and different inheritance patterns. Although complications occur in about one-half of pregnancies in women with EDS, the majority can have a good outcome if managed appropriately. Classic EDS is characterized by joint hypermobility, loose skin with poor healing and easy bruising, musculoskeletal problems with chronic pain and at risk for pre-term delivery. In addition, the vascular form of EDS can have cardiac anomalies, aneurysms, gastrointestinal perforation and uterine rupture during pregnancy. Due to overlapping features among the connective tissue disorders, it is difficult to categorize the disorder into specific types without detailed genetic testing which is now available through advanced genomic technology using next-generation DNA sequencing, searching genomic databases and bioinformatics approach. Therefore, obstetrical complications are variable but relate to specific connective tissue disorders requiring an exact diagnosis. There are several dozen genes causing connective tissue disorders that are currently available for testing using next-generation sequencing and bioinformatics to provide pertinent care, treatment and surveillance of the affected pregnant woman but also for her at-risk fetus related to the specific heritable condition.

Entities:  

Keywords:  Advanced genomic technology and next generation sequencing; Connective tissue disorders impacting pregnancies; Ehlers–Danlos syndrome and obstetric complications; Genetic screening

Mesh:

Substances:

Year:  2019        PMID: 31250196      PMCID: PMC8034485          DOI: 10.1007/s00404-019-05226-5

Source DB:  PubMed          Journal:  Arch Gynecol Obstet        ISSN: 0932-0067            Impact factor:   2.344


  22 in total

1.  Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type.

Authors:  M Pepin; U Schwarze; A Superti-Furga; P H Byers
Journal:  N Engl J Med       Date:  2000-03-09       Impact factor: 91.245

Review 2.  Ehlers-Danlos syndrome: insights on obstetric aspects.

Authors:  Natalie Volkov; Victoria Nisenblat; Gonen Ohel; Ron Gonen
Journal:  Obstet Gynecol Surv       Date:  2007-01       Impact factor: 2.347

3.  Orthopaedic aspects of the Ehlers-Danlos syndrome.

Authors:  P Beighton; F Horan
Journal:  J Bone Joint Surg Br       Date:  1969-08

Review 4.  Management of pregnancy in women with genetic disorders: Part 2: Inborn errors of metabolism, cystic fibrosis, neurofibromatosis type 1, and Turner syndrome in pregnancy.

Authors:  Shilpa P Chetty; Brian L Shaffer; Mary E Norton
Journal:  Obstet Gynecol Surv       Date:  2011-12       Impact factor: 2.347

5.  Obstetrical problems in patients with Ehlers-Danlos syndrome type IV; a case report.

Authors:  A De Paepe; B Thaler; M Van Gijsegem; D Van Hoecke; M Matton
Journal:  Eur J Obstet Gynecol Reprod Biol       Date:  1989-11       Impact factor: 2.435

Review 6.  Diagnosis, natural history, and management in vascular Ehlers-Danlos syndrome.

Authors:  Peter H Byers; John Belmont; James Black; Julie De Backer; Michael Frank; Xavier Jeunemaitre; Diana Johnson; Melanie Pepin; Leema Robert; Lynn Sanders; Nigel Wheeldon
Journal:  Am J Med Genet C Semin Med Genet       Date:  2017-03       Impact factor: 3.908

7.  Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome.

Authors:  Y Sorokin; M P Johnson; N Rogowski; D A Richardson; M I Evans
Journal:  J Reprod Med       Date:  1994-04       Impact factor: 0.142

8.  Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome.

Authors:  Bradley S Hurst; Sara S Lange; Susan M Kullstam; Rebecca S Usadi; Michelle L Matthews; Paul B Marshburn; Megan A Templin; Kathryn S Merriam
Journal:  Obstet Gynecol       Date:  2014-03       Impact factor: 7.661

9.  Pregnancy-related deaths and complications in women with vascular Ehlers-Danlos syndrome.

Authors:  Mitzi L Murray; Melanie Pepin; Suzanne Peterson; Peter H Byers
Journal:  Genet Med       Date:  2014-06-12       Impact factor: 8.822

10.  Spinal Anaesthesia for Cesarean Section in a Patient with Vascular Type Ehlers-Danlos Syndrome.

Authors:  Jeffrey M Carness; Mark J Lenart
Journal:  Case Rep Anesthesiol       Date:  2018-01-28
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  1 in total

1.  Actionable Genomics in Clinical Practice: Paradigmatic Case Reports of Clinical and Therapeutic Strategies Based upon Genetic Testing.

Authors:  Merlin G Butler; Daniel Moreno-De-Luca; Antonio M Persico
Journal:  Genes (Basel)       Date:  2022-02-10       Impact factor: 4.096

  1 in total

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