title: [Disorders of sex development and proximal hypospadias].
abstract: BACKGROUND
  Children with ambiguous genitalia due to different chromosome or gonadal sex are a particular challenge concerning the diagnostic and therapeutic implications. Proximal hypospadias patients with normal gonadal development should be distinguished from children with DSD (disorders of sex development) to guarantee normal gender identity and the best possible surgical therapy.
  
  
  OBJECTIVE
  This paper focuses on the terminology, embryology, and pathophysiology of the different manifestations of DSD. The state of knowledge about this disease pattern with particular emphasis on proximal hypospadias based on national and international scientific discussions is presented.
  
  
  METHODS
  The different clinical pictures as well as therapeutic options of DSD with a special focus on recent literature and giving particular attention to patients with proximal hypospadias are presented.
  
  
  RESULTS
  Because of the complexity of patients suffering from disorders of sex development an interdisciplinary DSD healthcare team including a paediatric endocrinologist as well as paediatric urologist should be provided. These specialists enable an accurate diagnosis in severe hypospadias patients without reference to DSD diseases patterns.
authors.affiliation: Abteilung für Kinderurologie, Krankenhaus der Barmherzigen Schwestern, Seilerstätte 4, 4010, Linz, Österreich. josef.oswald@bhs.at.|Universität Innsbruck, Innsbruck, Österreich. josef.oswald@bhs.at.
authors.forename: J
authors.initials: J
authors.lastname: Oswald
mesh_terms: D002648:Child; D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000175:diagnosis* / Q000523:psychology / Q000628:therapy*; D019317:Evidence-Based Medicine; D005260:Female; D005783:Gender Identity; D005858:Germany; D006801:Humans; D007021:Hypospadias / Q000175:diagnosis* / Q000523:psychology / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods*; D013520:Urologic Surgical Procedures / Q000379:methods*
keywords: Chicago Consensus; Chromosomal, gonadal, anatomical gender; Congenital adrenal hyperplasia; Hypospadias repair; Intersexuality