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Male Pseudohermaphroditism

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1. Moyamoya syndrome in a male pseudohermaphrodite patient with congenital adrenal hyperplasia - a rare association. Case report and review of literature. (PubMed)

Moyamoya syndrome in a male pseudohermaphrodite patient with congenital adrenal hyperplasia - a rare association. Case report and review of literature. Background and importance: Moyamoya syndrome causes progressive stenosis of intracranial internal carotid arteries and may be associated with genetic disorders like Down's or Turner's syndromes. We treated a male pseudohermaphrodite patient with congenital adrenal hyperplasia (CAH) with clinical and radiological features of moyamoya vasculopathy

2019 British Journal of Neurosurgery

2. A rare case of male pseudohermaphroditism-persistent mullerian duct syndrome with transverse testicular ectopia – Case report and review of literature (PubMed)

A rare case of male pseudohermaphroditism-persistent mullerian duct syndrome with transverse testicular ectopia – Case report and review of literature Persistent Mullerian duct syndrome (PMDS) is a rare type of male pseudohermaphroditism. Transverse testicular ectopia (TTE) is characterized by one testis moving to the opposite side and both testes traversing the same inguinal canal.An 11-month-old boy presented with bilateral cryptorchidism. The left testis was not palpable; the right testis (...) was canalicular with a right inguinal hernia. Ultrasound showed both testes located in the right inguinal canal. Right inguinal exploration revealed two testes with intact spermatic cords. A primitive uterus with fallopian tubes was also identified on opening the processus vaginalis. After herniotomy, bilateral orchidopexy was carried out (left orchidopexy through a trans-septal approach). Karyotyping confirmed a male gender (46XY). One year after the operation, ultrasound showed both testes to be in good

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2017 International journal of surgery case reports

3. Congenital adrenal hyperplasia and vanishing testis: rare case of male pseudohermaphroditism (PubMed)

Congenital adrenal hyperplasia and vanishing testis: rare case of male pseudohermaphroditism Congenital adrenal hyperplasia (CAH) and vanishing testes are uncommon diseases that can result from hormonal and mechanical factors. Classic CAH is determined by ambiguous genitalia and increase in amount of 17-Hydroxyprogesterone. Simultaneous occurrence of CAH and vanishing testes is a rare condition.A 22-year-old boy, known case of CAH who was diagnosed as female pseudohermaphroditism due

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2016 International Journal of Reproductive Biomedicine

4. Male Pseudohermaphroditism

Male Pseudohermaphroditism Male Pseudohermaphroditism Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Male Pseudohermaphroditism Male (...) Pseudohermaphroditism Aka: Male Pseudohermaphroditism From Related Chapters II. Pathophysiology Karyotype is 46XY III. Causes Testicular Dysgenesis Associated with degenerative renal disease Disorders of androgen synthesis Leydig cell hypoplasia Abnormal transport Deficiencies of steroidogenic enzyme activity Abnormal androgen receptor Maternal s or s Eponymic syndromes Persistent Mullerian Duct syndromes Vanishing Syndromes IV. Signs External and internal genitalia are ambiguous Incompletely masculinized Gonads

2018 FP Notebook

5. Male Hypogonadism

, tumour, torsion, inflammation, iatrogenic, surgical removal Secondary testicular dysfunction Medication, drugs, toxins, systemic diseases, varicocele (Idiopathic) testicular atrophy/testicular dysgenesis Male infertility (idiopathic or specific causes) Congenital anorchia (bilateral in 1 in 20,000 males, unilateral four times as often) Intra-uterine torsion is the most probable cause 46,XY disorders of sexual development (DSD) (formerly male pseudohermaphroditism) Disturbed testosterone synthesis due (...) Male Hypogonadism Male Hypogonadism | Uroweb › Male Hypogonadism Male Hypogonadism To access the pdfs & translations of individual guidelines, please as EAU member. Non-EAU members can view the web versions. To become an EAU member, click . G.R. Dohle (Chair), S. Arver, C. Bettocchi, T.H. Jones, S. Kliesch TABLE OF CONTENTS REFERENCES 1. Dohle, G.R., et al., EAU Guidelines Panel on Male Hypogonadism, Edn. presented at the EAU Annual Congress Paris 2012: Arnhem, the Netherlands. 2. Guyatt, G.H

2019 European Association of Urology

6. Male Hypogonadism

, tumour, torsion, inflammation, iatrogenic, surgical removal Secondary testicular dysfunction Medication, drugs, toxins, systemic diseases, varicocele (Idiopathic) testicular atrophy/testicular dysgenesis Male infertility (idiopathic or specific causes) Congenital anorchia (bilateral in 1 in 20,000 males, unilateral four times as often) Intra-uterine torsion is the most probable cause 46,XY disorders of sexual development (DSD) (formerly male pseudohermaphroditism) Disturbed testosterone synthesis due (...) Male Hypogonadism Male Hypogonadism | Uroweb › Male Hypogonadism Male Hypogonadism To access the pdfs & translations of individual guidelines, please as EAU member. Non-EAU members can view the web versions. To become an EAU member, click . G.R. Dohle (Chair), S. Arver, C. Bettocchi, T.H. Jones, S. Kliesch TABLE OF CONTENTS REFERENCES 1. Dohle, G.R., et al., EAU Guidelines Panel on Male Hypogonadism, Edn. presented at the EAU Annual Congress Paris 2012: Arnhem, the Netherlands. 2. Guyatt, G.H

2018 European Association of Urology

7. Type II atresia ani associated with rectovaginal fistula in a male pseudohermaphrodite kitten (PubMed)

Type II atresia ani associated with rectovaginal fistula in a male pseudohermaphrodite kitten A combination of gastrointestinal and urogenital congenital abnormalities was diagnosed and surgically treated in a kitten. Physical examination, exploratory laparotomy, castration, histological examination, and cytogenetic karyotyping were utilized to determine the true gender of the kitten. The kitten was confirmed to be a male (38 XY) pseudohermaphrodite with Type II atresia ani and rectovaginal

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2013 The Canadian Veterinary Journal

8. Estradiol and Testosterone Secretion by Human, Simian, and Canine Testes, in Males with Hypogonadism and in Male Pseudohermaphrodites with the Feminizing Testes Syndrome (PubMed)

Estradiol and Testosterone Secretion by Human, Simian, and Canine Testes, in Males with Hypogonadism and in Male Pseudohermaphrodites with the Feminizing Testes Syndrome The role of the human testis in the production of 17beta-estradiol (E(2)) was investigated by determining the concentration of E(2) and testosterone in peripheral and spermatic vein plasma samples. Specimens were obtained from eight normal men, three men with hypogonadism, and two patients with the incomplete form (...) for charcoaladsorbed pooled male plasma. Pooled male and pooled female control plasmas averaged 17+/-0.71 pg/ml and 95+/-6.9 pg/ml, respectively; individual adult male specimens ranged between 8 and 28 with a mean of 18+/-1.4 pg/ml. In the eight normal men, the mean peripheral vein E(2) concentration was 20+/-1.6 pg/ml, while the spermatic vein concentration was 50 times as great, 1049+/-57 pg/ml. All three patients with testicular abnormalities had low spermatic vein E(2) concentrations (160, 280, and 416 pg/ml

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1972 Journal of Clinical Investigation

9. Male Pseudohermaphroditism

Male Pseudohermaphroditism Male Pseudohermaphroditism Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Male Pseudohermaphroditism Male (...) Pseudohermaphroditism Aka: Male Pseudohermaphroditism From Related Chapters II. Pathophysiology Karyotype is 46XY III. Causes Testicular Dysgenesis Associated with degenerative renal disease Disorders of androgen synthesis Leydig cell hypoplasia Abnormal transport Deficiencies of steroidogenic enzyme activity Abnormal androgen receptor Maternal s or s Eponymic syndromes Persistent Mullerian Duct syndromes Vanishing Syndromes IV. Signs External and internal genitalia are ambiguous Incompletely masculinized Gonads

2015 FP Notebook

10. Dihydrotestosterone binding by cultured human fibroblasts. Comparison of cells from control subjects and from patients with hereditary male pseudohermaphroditism due to androgen resistance. (PubMed)

Dihydrotestosterone binding by cultured human fibroblasts. Comparison of cells from control subjects and from patients with hereditary male pseudohermaphroditism due to androgen resistance. Dihydrotestosterone binding was measured in culture fibroblasts from 14 control subjects and from 12 patients with five different types of hereditary male pseudohermaphroditism. Two assays of binding were used--an intact monolayer assay and density gradient centrifugation of cell extracts. In the intact (...) ). In the density gradient assay in 5-10% sucrose, the major peak of dihydrotestosterone binding was in the 8S region in low molarity buffer and in the 4S region in 0.5 M KCl. High affinity binding was normal in cells from two patients with familial incomplete male pseudohermaphroditism, type 2, an autosomal recessive defect in which dihydrotestosterone formation is deficient, and in cells from a patient with male pseudohermaphroditism due to 17 beta-hydroxysteroid dehydrogenase deficiency, an autosomal

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1976 Journal of Clinical Investigation

11. Male pseudohermaphroditism presumably due to target organ unresponsiveness to androgens. Deficient 5alpha-dihydrotestosterone binding in cultured skin fibroblasts. (PubMed)

Male pseudohermaphroditism presumably due to target organ unresponsiveness to androgens. Deficient 5alpha-dihydrotestosterone binding in cultured skin fibroblasts. Maximum specific 5alpha-dihydrotestosterone (DHT) binding activity (Bmax) had been measured in intact confluent monolayers representing fibroblast strains derived form nongenital and genital (labium majus) skin of normal individuals and of 11 patients fulfilling the clinicogenetic criteria of complete testicular feminization (TF (...) ). Nine labium majus strains from adult females had a mean Bmax value three times greater than that of seven nongenital strains from adult females (33 vs. 11 fmol/mg cell protein). The Bmax results for 13 adult nongenital strains varied from 5.6 to 23.3 fmol/mg protein; the values for males and females had very similar means and ranges. The variation could not be correlated with the chronologic age of adult skin explant donors or with the in vitro age (mean population doubling level) of the cultures

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1976 Journal of Clinical Investigation

12. Male pseudohermaphroditism. (PubMed)

Male pseudohermaphroditism. 5922416 1967 01 22 2010 11 18 0035-9157 59 9 1966 Sep Proceedings of the Royal Society of Medicine Proc. R. Soc. Med. Male pseudohermaphroditism. 833 Ward A P AP eng Journal Article England Proc R Soc Med 7505890 0035-9157 IM Adolescent Disorders of Sex Development surgery Humans 1966 9 1 1966 9 1 0 1 1966 9 1 0 0 ppublish 5922416 PMC1901207

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1966 Proceedings of the Royal Society of Medicine

13. Schizophrenia in a male pseudohermaphrodite. (PubMed)

Schizophrenia in a male pseudohermaphrodite. 6039651 1967 10 31 2013 11 21 0007-1447 3 5568 1967 Sep 23 British medical journal Br Med J Schizophrenia in a male pseudohermaphrodite. 783-4 Trick K L KL eng Case Reports Journal Article England Br Med J 0372673 0007-1447 U42B7VYA4P Chlorpromazine AIM IM Adult Chlorpromazine therapeutic use Disorders of Sex Development complications Electric Stimulation Electroshock Humans Male Psychological Tests Schizophrenia complications drug therapy 1967 9 23

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1967 British medical journal

14. Male pseudohermaphroditism: diagnosis in cell culture. (PubMed)

Male pseudohermaphroditism: diagnosis in cell culture. Testicular feminization is a classic form of complete male pseudohermaphroditism. The individuals have a normal XY karyotype but unambiguously female external genitalia. They have congenital complete insensitivity to androgen due to an X-linked mutation. In four patients (from tow families with several affected members) with the typical phenotype of testicular feminization, a severe deficit of specific androgen-binding activity was detected (...) in cultured fibroblasts from labium majus skin. Measurement of this activity in genital skin fibroblasts improves the differential diagnosis in patients with complete or imcomplete male pseudohermaphroditism before puberty.

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1977 Canadian Medical Association Journal

15. 5-alpha-Reductase deficiency causing male pseudohermaphroditism. (PubMed)

5-alpha-Reductase deficiency causing male pseudohermaphroditism. An infant with male pseudohermaphroditism due to deficiency of 5-alpha-reductase is described, the elder of two affected male siblings. These patients, who come from Pakistan, are the first to be described outside America.

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1978 Archives of Disease in Childhood

16. Hereditary Male Pseudohermaphroditism Associated with an Unstable Form of 5α-Reductase (PubMed)

Hereditary Male Pseudohermaphroditism Associated with an Unstable Form of 5α-Reductase The properties of 5alpha-reductase have been compared in genital skin fibroblasts cultured from five patients from three families (Los Angeles, Dallas, and Dominican Republic) in which hereditary male pseudohermaphroditism has been established to result from deficient conversion of testosterone to dihydrotestosterone. Despite the fact that 5alpha-reductase was immeasurable in a homogenate of epididymis (...) higher than that of the controls. We conclude that the mutations in the Dallas and Dominican Republic families are similar and result in low activity of the enzyme as the result of a decreased affinity for testosterone.Thus, two distinct types of mutations can produce male pseudohermaphroditism due to deficient dihydrotestosterone formation.

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1978 Journal of Clinical Investigation

17. Male Pseudohermaphroditism (PubMed)

Male Pseudohermaphroditism 13042261 2003 05 01 2018 12 01 0007-1447 1 4824 1953 Jun 20 British medical journal Br Med J A case of male pseudohermaphroditism. 1369-70 BEATTY D C DC CHAMP C J CJ SWYER G I M GI eng Journal Article England Br Med J 0372673 0007-1447 OM Disorder of Sex Development, 46,XY Disorders of Sex Development Humans Male 5324:12600:297 HERMAPHRODITISM 1953 6 20 1953 6 20 0 1 1953 6 20 0 0 ppublish 13042261 PMC2016696 J Clin Endocrinol Metab. 1952 Apr;12(4):423-38 14917732 Br

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1953 British medical journal

18. Male Pseudohermaphroditism (PubMed)

Male Pseudohermaphroditism 13051595 2003 05 01 2018 12 01 0007-1447 2 4827 1953 Jul 11 British medical journal Br Med J Male pseudohermaphroditism. 94-5 FAIRBAIRN W R WR eng Journal Article England Br Med J 0372673 0007-1447 OM Disorder of Sex Development, 46,XY Disorders of Sex Development Humans Male 5324:22132:297 HERMAPHRODITISM 1953 7 11 1953 7 11 0 1 1953 7 11 0 0 ppublish 13051595 PMC2028461

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1953 British medical journal

19. Male Pseudohermaphroditism: A Hitherto Undescribed Form (PubMed)

Male Pseudohermaphroditism: A Hitherto Undescribed Form 13250193 2003 05 01 2018 12 01 0007-1447 2 4941 1955 Sep 17 British medical journal Br Med J Male pseudohermaphroditism: a hitherto undescribed form. 709-12 SWYER G I GI eng Journal Article England Br Med J 0372673 0007-1447 OM Disorder of Sex Development, 46,XY Disorders of Sex Development Humans Male 5629:1996 HERMAPHRODITISM 1955 9 17 1955 9 17 0 1 1955 9 17 0 0 ppublish 13250193 PMC1980764 J Clin Endocrinol Metab. 1952 Apr;12(4):423-38

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1955 British medical journal

20. Male Pseudohermaphroditism with XO Chromosomal Constitution on Bone-marrow Cells (PubMed)

Male Pseudohermaphroditism with XO Chromosomal Constitution on Bone-marrow Cells 14014313 1998 11 01 2018 12 01 0007-1447 2 5312 1962 Oct 27 British medical journal Br Med J Male pseudohermaphroditism with XO chromosomal constitution on bone-marrow cells. 1100-1 BOTTURA C C FERRARI I I eng Journal Article England Br Med J 0372673 0007-1447 OM Bone Marrow Bone Marrow Cells Chromosomes Disorder of Sex Development, 46,XY Disorders of Sex Development Humans Male BONE MARROW CHROMOSOMES

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1962 British medical journal

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