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Proceedings of the Royal Society of... Sep 1952
Topics: Adrenal Cortex; Adrenal Cortex Diseases; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Electrolytes
PubMed: 13003948
DOI: No ID Found -
Proceedings of the Royal Society of... May 1963
Topics: Addison Disease; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Disorders of Sex Development; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases
PubMed: 13936834
DOI: No ID Found -
Schweizerische Medizinische... Nov 1964
Topics: 17-Ketosteroids; Adrenal Cortex Hormones; Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Body Fluids; Child; Cortisone; Drug Therapy; Urine
PubMed: 14340254
DOI: No ID Found -
Medicina Clinica Feb 1963
Topics: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Humans
PubMed: 13947792
DOI: No ID Found -
Geburtshilfe Und Frauenheilkunde Apr 1958
Topics: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Hormones
PubMed: 13548469
DOI: No ID Found -
Zeitschrift Fur Urologie Und Nephrologie May 1977
Topics: Adrenal Gland Neoplasms; Adrenal Glands; Adrenal Hyperplasia, Congenital; Desoxycorticosterone; Female; Glucocorticoids; Gonadal Steroid Hormones; Humans; Male
PubMed: 899308
DOI: No ID Found -
Meditsinskaia Sestra Nov 1980
Topics: Adolescent; Adrenal Hyperplasia, Congenital; Adult; Child; Child, Preschool; Female; Humans; Male
PubMed: 6906561
DOI: No ID Found -
Nihon Rinsho. Japanese Journal of... 1977
Topics: Adrenal Hyperplasia, Congenital; Female; Humans; Male
PubMed: 612772
DOI: No ID Found -
Journal of Pediatric Urology Apr 2018The adrenogenital syndrome is an autosomal recessive disorder in which an enzyme defect in the steroid pathway leads to excessive prenatal exposure of androgens. In the...
The adrenogenital syndrome is an autosomal recessive disorder in which an enzyme defect in the steroid pathway leads to excessive prenatal exposure of androgens. In the female fetus, masculinization of the external genitalia is observed. Surgery aims for functional and aesthetical reconstruction. Many techniques have been described. A video of our modified pull-through reconstruction technique is hereby presented. A retrospective descriptive database was created with patients who underwent genitoplasty for a CAH-associated genital condition. A video demonstrating the reconstructive technique was recorded while operating on a 9-month-old girl. Prior to surgery a cystoscopy is performed to evaluate the length of the urogenital sinus. Surgery starts with creating a reversed U-flap, after which the urogenital sinus is mobilized. The corpora cavernosa are released and the neurovascular bundle is isolated. To create vaginal space the urogenital sinus is subsequently separated. The vaginal introitus is anchored to the perineal skin flap. Labia minora are created by splitting the preputial skin. Finally excessive skin tissue is resected. Twenty-two female patients underwent reconstructive surgery for the adrenogenital syndrome in a tertiary referral centre over 16 years. Median age at surgery was 3 months (0-190). Median follow-up was 36 months (0-108) after surgery. A good functional and aesthetical outcome was observed. The modified pull-through technique, illustrated by this video, provided satisfactory results with a low complication rate. Follow-up until adulthood is needed to evaluate long-term outcomes.
Topics: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Cohort Studies; Databases, Factual; Female; Follow-Up Studies; Genitalia, Female; Humans; Infant, Newborn; Plastic Surgery Procedures; Recovery of Function; Retrospective Studies; Treatment Outcome; Urogenital Surgical Procedures; Video-Assisted Surgery
PubMed: 29551555
DOI: 10.1016/j.jpurol.2018.02.011 -
Bratislavske Lekarske Listy Apr 1962
Topics: Adrenal Hyperplasia, Congenital; Adrenogenital Syndrome; Humans; Medical Records
PubMed: 13886701
DOI: No ID Found