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Male Pseudohermaphroditism

Aka: Male Pseudohermaphroditism

Pathophysiology
1. Karyotype is 46XY
Causes
1. Testicular Dysgenesis
  1. Associated with degenerative renal disease
2. Disorders of androgen synthesis
  1. Leydig cell hypoplasia
  2. Abnormal Cholesterol transport
  3. Deficiencies of steroidogenic enzyme activity
3. Abnormal androgen receptor
4. Maternal Estrogens or Progestins
5. Eponymic syndromes
6. Persistent Mullerian Duct syndromes
7. Vanishing Testes Syndromes
Signs
1. External and internal genitalia are ambiguous
  1. Incompletely masculinized
2. Gonads recognizable as Testes

Male Pseudohermaphroditism (C0238395)
Concepts	Disease or Syndrome (T047)
MSH	D058490
ICD10	Q56.1
SnomedCT	111332007
English	PSEUDOHERMAPHRODITISM, MALE, Pseudohermaphroditism syndrome, male, Pseudohermaphroditism male, Male pseudohermaphroditism NOS, Male Pseudohermaphroditism, Pseudohermaphroditism, Male, Male Pseudohermaphroditisms, Pseudohermaphroditisms, Male, merm, male pseudohermaphroditism, Male pseudohermaphroditism, Merms, Male pseudohermaphroditism (disorder), pseudohermaphroditism; male
Dutch	mannelijk pseudohermafroditisme, pseudohermafroditisme; bij man, pseudohermafroditisme mannelijk
German	maennlicher Pseudohermaphroditismus, Pseudohermaphroditismus masculinus, Männlicher Pseudohermaphroditismus, Pseudohermaphroditismus des Mannes
Japanese	男性仮性半陰陽, ﾀﾞﾝｾｲｶｾｲﾊﾝｲﾝﾖｳ
Czech	Mužský pseudohermafroditismus, mužský pseudohermafroditismus
French	Pseudohermaphrodisme masculin, Pseudo-hermaphrodisme masculin, Pseudo-hermaphrodisme mâle
Italian	Pseudoermafroditismo maschile, Maschio XY con ridotta mascolinizzazione
Hungarian	Férfi pseudohermaphroditismus, Férfi álkétneműség
Spanish	merms, pseudohermafroditismo masculino (trastorno), pseudohermafroditismo masculino, Pseudohermafroditismo masculino
Portuguese	Pseudo-hermafroditismo masculino
Sources	Derived from the NIH UMLS (Unified Medical Language System)

Family Practice Notebook

Patients should address specific medical concerns with their physicians.

Although access to this page is not restricted, the information found here is intended for use by medical providers.

This page was written by Scott Moses, MD, last revised on 10/21/2007 and last published on 5/14/2013.

This is one of 5887 pages in the Family Practice Notebook