title: Treatment management during the adolescent transition period of girls and young women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS): a systematic literature review.
abstract: In health services research, there is a special emphasis on the transition from adolescence into adulthood. During this transition period, adolescents change from pediatric to adult medical care. This process must be carefully structured, particularly when special medical care is required. Challenges and difficulties become apparent particularly in the case of rare diseases. This is increasingly so when the rare disease affects the adolescence-specific development of patients, such as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), also known as Müllerian agenesis.
  
  
  
  A systematic literature review identified the care requirements of girls and young women with MRKHS, as well as studies of medical care during the adolescent transition period for various other diseases. This investigation was carried out in the years 2012 and 2013, and was updated in 2014/2015. In addition, the reference lists of the identified studies were reviewed.
  
  
  
  Nine publications on MRKHS and ten publications on the transition from adolescence to adulthood were included. Medical care requirements and measures were identified for the following areas: diagnosis during adolescence and organization of medical care, reactions to the diagnosis, functional infertility, psychological stress and threat to self-image, contact with others, and dealing with MRKHS coping strategies.
  
  
  
  There is still a great demand for research in the area of care during the transition period from adolescence into adulthood, particularly for rare diseases. The recommendations for treating MRKHS patients derived from the literature should be implemented and evaluated with regard to their effectiveness.
authors.affiliation: Institute of Occupational and Social Medicine and Health Services Research, University Hospital Tuebingen, Wilhelmstraße 27, 72074, Tübingen, Germany. anke.wagner@med.uni-tuebingen.de.
authors.forename: Anke
authors.identifier: 0000-0002-5053-1753
authors.initials: A
authors.lastname: Wagner
authors.affiliation: Centre of Women's Health, University Hospital Tübingen, Calwerstraße 7, 72076, Tübingen, Germany.
authors.forename: Sara Yvonne
authors.initials: SY
authors.lastname: Brucker
authors.affiliation: Women's Health Research Institute, Calwerstraße 7, 72076, Tübingen, Germany.
authors.forename: Esther
authors.initials: E
authors.lastname: Ueding
authors.affiliation: Institute of Occupational and Social Medicine and Health Services Research, University Hospital Tuebingen, Wilhelmstraße 27, 72074, Tübingen, Germany.
authors.forename: Dagmar
authors.initials: D
authors.lastname: Gröber-Grätz
authors.affiliation: Centre of Women's Health, University Hospital Tübingen, Calwerstraße 7, 72076, Tübingen, Germany.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Simoes
authors.affiliation: Centre of Women's Health, University Hospital Tübingen, Calwerstraße 7, 72076, Tübingen, Germany.
authors.forename: Katharina
authors.initials: K
authors.lastname: Rall
authors.affiliation: Institute of General Practice, University of Tübingen, Österbergstraße 9, 72074, Tübingen, Germany.
authors.forename: Andrea
authors.initials: A
authors.lastname: Kronenthaler
authors.affiliation: Department of Psychosomatic Medicine and Psychotherapy, University Hospital Tübingen, Germany, Osianderstraße 5, 72076, Tübingen, Germany.
authors.forename: Norbert
authors.initials: N
authors.lastname: Schäffeler
authors.affiliation: Institute of Occupational and Social Medicine and Health Services Research, University Hospital Tuebingen, Wilhelmstraße 27, 72074, Tübingen, Germany.
authors.forename: Monika A
authors.initials: MA
authors.lastname: Rieger
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000523:psychology* / Q000601:surgery; D000293:Adolescent; D000013:Congenital Abnormalities / Q000523:psychology* / Q000601:surgery; D005260:Female; D006801:Humans; D009095:Mullerian Ducts / Q000002:abnormalities* / Q000601:surgery; D012649:Self Concept; D055815:Young Adult
keywords: Rare disease; Systematic literature review; Transition; Transition care