Anorchia, also known as embryonic testicular regression is characterized by the unilateral or bilateral absence of functioning testicular tissue in an individual who has 46, XY karyotype and is phenotypically male [1] [2] [3]. The congenital form has an incidence of one in twenty thousand [4] while an acquired form has been identified based on the presence of testicular tissue in the postnatal period but which disappears later [5]. Anorchia has also been reported in one out of 175 cases with cryptorchidism [6]. It is known to have a familial tendency [1] [4] [7] [8]. The etiology of the condition is still being debated with several proposed theories ranging from genetic to 46, XY abnormal gonads [9] to vascular occlusion during embryonic testicular descent [10].

Most patients with anorchia present with normal male external sexual characteristics but may also present with microphallus [11], ambiguous external genitals [7], and feminine appearance of external genitalia [12]. Cases of neonatal testicular torsion as a presenting symptom has been reported by various authors [11] [12]. Individuals with bilateral anorchia typically develop normally but the onset of puberty is either delayed or absent depending on the extent of functioning testicular tissue in the body. Therefore secondary sexual characteristics may develop partially or not at all. Other organ anomalies like renal ectopia can also be present in these individuals [12] [13] along with low bone density.

• Male Hypogonadism

  Subsequent chapters deal with male sexual precocity; "fertile eunuchism" (Leydig cells reduced in number and size with preserved spermatogenesis); male idiopathic hypogonadism associated with idiopathic hypothyroidism, and with idiopathic renal tubular [books.google.com]

  Discussions on the current methods for the diagnosis and treatment of male hypogonadism, male infertility and male sexual dysfunction follow, with detailed descriptions of types of androgen replacement and the benefits and risks of such treatment. [books.google.ro]

  Gynecomastia How can you classify male hypogonadism into 3 types? memorize this 1. Primary ( hyper gonadotropic) hypogonadism (testicular failure) - increased LH, FSH and decreased Te 2. [quizlet.com]

  hypogonadism in recruits and soldiers]. 61 Tomov T...Ankov V 7010789 1980 152 [Hypothalamo-hypophyseal-thyroid axis in recruits and soldiers with male hypogonadism]. 61 Tomov T...Ankov V 6777988 1980 153 A gonadotrophin responsive testosterone producing [malacards.org]

  Anti-Müllerian hormone and sertoli cell function in paediatric male hypogonadism. Horm Res Paediatr. 2010; 73: 81–92 Crossref | PubMed | Scopus (90) | Google ScholarSee all References]. [jpurol.com]

• Vietnamese

  Русский (Russian) ಕನ್ನಡ (Kannada) 한국어 (Korean) עברית (Hebrew) Український (Ukrainian) اردو (Urdu) Magyar (Hungarian) मानक हिन्दी (Hindi) Indonesia (Indonesian) Italiano (Italian) தமிழ் (Tamil) Türkçe (Turkish) తెలుగు (Telugu) ภาษาไทย (Thai) Tiếng Việt (Vietnamese [definitions.net]

• Ectomorph

  […] males with anarchism appear smaller with less muscular built, there is absence of hair in the face, pubic area, arms and thighs, voice may sound child-like, and they are more likely to assume a body appearance that is thin and linear instead of the ectomorphic [ehealthwall.com]

• Aura

  Votre fils aura besoin de quelques heures pour se réveiller de l’opération, et pourra probablement retourner à la maison après, sans avoir à passer la nuit à l’hôpital. [aboutkidshealth.ca]

• Testicular Disease

  From Wikidata Jump to navigation Jump to search human disease Testicular agenesis Bilateral anorchia edit Language Label Description Also known as English Anorchia human disease Testicular agenesis Bilateral anorchia Statements instance of disease 0 references [wikidata.org]

  Cromie WJ: Cryptorchidism and malignant testicular disease, in Hadziselimovic F (ed): Cryptorchidism: Management and Implications. New Yeok, Springer-Verlag, 1983; 83. 228. [auanet.org]

A clinician should suspect anorchia if a patient presents with complaints of delayed puberty and has masculine external genitals but no palpable testes. A thorough family history must be elicited as other family members are also likely to have complete or partial gonadal abnormalities with 46, XY karyotype [12]. Physical examination may reveal microphallus [14] or normal external male genitalia with bilateral or unilateral palpable intrascrotal [11] [12] or inguinal masses [11] or no palpable masses [12].

Confirmation of the diagnosis requires hormonal evaluation such as gonadotrophin-releasing hormone (GnRH), follicle stimulating hormone (FSH), luteinizing hormone (LH), testosterone, inhibin B levels and provocation test with human chorionic gonadotrophin (hCG). In bilateral anorchia, low levels of testosterone along with elevated FSH levels and an absence of a rise in levels of testosterone after hCG [14] [15] [16] [17] is noticed. hCG provocation test is not required in 46, XY patients with elevated FSH and undetectable levels of anti-müllerian (AMH) and inhibin B as this scenario is diagnostic for anorchia [12].

Although ultrasonography can be performed as part of the workup, it cannot distinguish between testis and inguinal lymphadenopathy. Magnetic resonance imaging (MRI) is more useful for detecting testicular tissue especially if cryptorchidism is suspected. But imaging studies may not be required if hCG provocation fails to cause a rise in plasma testosterone levels [18].

Some authors have suggested surgical exploration for the testicular tissue as the most definitive method of diagnosis [19] especially in unilateral anorchia [17]. However, others [5] suggest that surgery is unnecessary in the workup of bilateral anorchia if endocrine tests confirm the absence of functioning testicular tissue.

In most cases, testosterone treatment stimulates the penile growth. [ncbi.nlm.nih.gov]

Growth, sexual and bone development in a boy with bilateral anorchia under testosterone treatment guided by the development of his monozygotic twin. JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, 31(3), 361–367. [biblio.ugent.be]

Treatment includes: Artificial (prosthetic) testicle implants Male hormones (androgens) Psychological support Outlook (Prognosis) The outlook is good with treatment. [sites.magellanhealth.com]

Prognosis - Anorchia Prognosis is good with treatment. Treatment - Anorchia Treatment includes androgen (testosterone) supplementation to artificially initiate puberty, testicular prosthetic implantation, and psychological support. [checkorphan.org]

Prognosis - Anorchia Prognosis is good with treatment. Treatment - Anorchia Treatment includes androgen (testosterone) supplementation to artificially initiate puberty, testicular prosthetic implantation, and psychological support. [checkorphan.org]

Expectations (prognosis) Prognosis is good with treatment. Complications Complications include infertility, psychological problems related to gender, and occasional face, neck, or back abnormalities. [health.am]

Bone density Surgery to look for male reproductive tissue Testosterone levels (low) Ultrasound or MRI X,Y karyotype Treatment Treatment includes: Artificial (prosthetic) testicle implants Male hormones (androgens) Psychological support Expectations (prognosis [coordinatedhealth.com]

Abstract Hypogonadism affecting the male pediatric population is uncommon, with that attributed to multiple unrelated etiologies being exceedingly rare. [ncbi.nlm.nih.gov]

Disorders of sexual development: etiology, evaluation, and medical management. In: Wein AJ, Kavoussi LR, Partin AW, Peters CA, eds. Campbell-Walsh Urology. 11th ed. Philadelphia, PA: Elsevier; 2016:chap 150. Updated by: Sovrin M. [nlm.nih.gov]

Definition / general Anorchism: absence of both testis Monoorchidism: absence of one testis Testicular regression syndrome: phenotypically normal male with vas deferens is present Etiology Causes: cryptorchidism, possibly testicular infarct, infection [pathologyoutlines.com]

The etiology of the condition is still being debated with several proposed theories ranging from genetic to 46, XY abnormal gonads to vascular occlusion during embryonic testicular descent. [symptoma.com]

[…] thyroid hormones 3.1.3 Clinical assessment of the thyroid patient 3.1.4 Thyroid function tests and the effects of drugs 3.1.5 Nonthyroidal illness 3.1.6 Thyroid imaging: nuclear medicine techniques 3.1.6.1 Thyroid imaging: nonisotopic techniques 3.1.7 Epidemiology [oxfordmedicine.com]

A short questionnaire for the measurement of habitual physical activity in epidemiological studies. Am J Clin Nutr 1982;36:936–42.CrossrefGoogle Scholar 6. Roelants M, Hauspie R, Hoppenbrouwers K. [degruyter.com]

If the vascular insult occur after 14 to 16 weeks Usually The external genitalia are normal. 16 pathophysiology 17. [slideshare.net]

Provided by Review Date: 9/7/2008 Reviewed By: Linda Vorvick, MD, Seattle Site Coordinator, Lecturer, Pathophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Louis S. [assets.aarp.org]

Images Review Date: 9/7/2008 Reviewed By: Linda Vorvick, MD, Seattle Site Coordinator, Lecturer, Pathophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Louis S. [lutheranhealthcare.org]

Update Date: 9/7/2008 Updated by: Linda Vorvick, MD, Seattle Site Coordinator, Lecturer, Pathophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine; and Louis S. [pacificschoolserver.org]

Pathophysiology Diminished functional activity of the gonads → reduced biosynthesis of sex hormones → impaired secondary sexual characteristics and infertility Clinical features Diagnostics Treatment [amboss.com]

Increased production of androgen precursors by the adrenal cortex may have prevented eunuchoidism without stimulating secondary sexual development. [jamanetwork.com]

Prevention - Anorchia Not supplied. Diagnosis - Anorchia Not supplied. Prognosis - Anorchia Prognosis is good with treatment. [checkorphan.org]

Among her publications on health sociology are "Crisis Prevention and Management during SARS Outbreak, Singapore", Emerging Infectious Diseases, 10, 2: 364-368, 2004, with HP Lee; "Traditional Healing Systems and the Ethos of Science," Social Science [books.google.ro]

Undescended testes usually descend into the scrotum during the first months after birth; if the testicle fails to descend into the scrotum, a surgical replacement or removal is necessary in order to relocate or remove the testicle and prevent a transformation [drelist.com]

It usually includes: Bracing Medication Physical therapy Preventing the condition from getting worse by treating the underlying condition causing the muscle weakness Surgery (sometimes) Your health care provider can tell you more about your condition [mclaren.org]

1. Abeyaratne MR, Aherne WA, Scott JES. The vanishing testis. Lancet. 1969;2: 822–824.
2. Sarto GE, Opitz JM. The XY gonadal agenesis syndrome. J Med Genet. 1973; 10:288–293.
3. Edman CD, Winters AJ, Porter JC, et al. Embryonic testicular regression. A clinical spectrum of XY agonadal individuals. Obstet Gynecol. 1977;49:208–217.
4. Bobrow M, Gough MH. Bilateral absence of testes. Lancet. 1970;1: 366.
5. Teo AQA, Khan AR, Williams MPL, et al. Is surgical exploration necessary in bilateral anorchia? Journal of Pediatric Urology. 2013; 9: e78-e81
6. Aynsley-Green A, Zachmann M, Illig R, et al. Congenital bilateral anorchia in childhood: a clinical, endocrine and therapeutic evaluation of twenty-one cases. Clin Endocrinol. (Oxf).1976;5:381–391.
7. Josso N, Briard ML. Embryonic testicular regression syndrome: variable phenotypic expression in siblings. J Pediatr.1980; 97: 200–204.
8. Naffah J. Familial testicular regression syndrome. Bull Acad Natl Med. 1989;173:709–714.
9. Marcantonio SM, Fechner PY, Migeon CJ, et al. Embryonic testicular regression sequence: a part of the clinical spectrum of 46,XY gonadal dysgenesis. Am J Med Genet 1994;49: 1–5.
10. Smith NM, Byard RW, Bourne AJ. Testicular regression syndrome-a pathological study of 77 cases. Histopathology 1991;19: 269–272.
11. Zenaty D, Dijoud F, Morel Y, et al. Bilateral anorchia in infancy: occurrence of microphallus and the effect of testosterone treatment. J Pediatr. 2006;149: 687–691.
12. Brauner R, Neve M, Allali S, et al. Clinical, Biological and Genetic Analysis of anorchia in 26 boys. PLoS One. 2011;6(8):e23292.
13. Lodh B, Kanwar V, Kaku Singh AK, Singh SR. Unfused crossed renal ectopia with ipsilateral anorchia- a rare entity. J. Clin. Diagn Res. 2013 Sep; 7(9): 2029-2030
14. Bernasconi S, Ghizzoni L, Panza C, Volta C, et al. Congenital anorchia: natural history and treatment. Horm.Res. 1992;37: 50-54.
15. Bablok L, Janczewski Z, Czaplicki M, Kwiatkowska Z.Plasma testosterone levels before and after stimulation with HCG in anorchism. Int Urol Nephrol. 1979;11: 57-60.
16. Jarow NP, Berkowitz GD, Migeon CJ, et al. Elevation of serum gonadotropins establishes the diagnosis of anorchism in prepubertal boys with bilateral cryptorchidism. J Urol. 1986;136: 277- 279
17. De Rosa M, Lupoli G, Mennitti M, Zarrilli S, et al. Congenital bilateral anorchia: clinical, hormonal and imaging study in 12 cases. Andrologica. 1996; 28 (5): 281-285
18. Rousso I, Illiopoulos F, Athanasiadou F, et al. Congenital bilateral anorchia: hormonal, molecular and imaging study of a case. Genetics and Molecular Research.642 Genetics and Molecular Research.2006 Oct 31;5(4):638-42
19. Belgorosky A, Rivarola MA. Sex hormone-binding globulin response to human chorionic gonadotropin stimulation in children with cryptorchidism, anorchia, male pseudohermaphroditism, and micropenis. J Clin Endocrinol Metab. 1982;54: 698-704.