431:
low doses and are gradually increased according to the body responses. Most of the studies regarding estrogen therapy have focused on girls with Turner
Syndrome. There are many formulations for estrogen therapy that include oral estradiol, oral conjugated estrogen, transdermal estrogen patches, and estrogen gel. The therapy is individualized and is initiated based on many factors including age, bone age, absolute height, and psychosocial issues. Progesterone therapy for a week per month in addition to estrogen allows for adequate uterine and breast development. Routine follow-ups for during and after pubertal inductions can include checkups for height, weight, body-mass index (BMI), and blood pressure three times a year and FSH/LH measurements every year.
150:, complex genital anomaly, or normal female phenotype. Children often have small or non-palpable testes and this can present with or without genital anomaly. As individuals progress onto adolescence, they may experience absent or delayed puberty or puberty that starts but fails to progress. Adult males with HH may exhibit gynecomastia, erectile dysfunction, reduced testicular volume (absence of testicular enlargement during puberty), abnormal testicular texture and consistency, small stature, decreased libido and sexual activity, infertility due to low (
422:
testosterone replacement therapy. However, it is important to note that testosterone treatment does not restore fertility in men. There are many infertility treatment options available for individuals with HH, such as selective estrogen receptor modulators (SERMs), aromatase inhibitors (AIs), and gonadotropins. Testicular sperm extraction, intracytoplasmic sperm injections, semen/embryo cryopreservation are also possible treatment options .
444:
hypogonadism. Neonatal testosterone therapy can be given to infants with HH. However, for children, testosterone should be avoided due to the possible adverse effects of rapid bone aging and growth acceleration. There are various formulation of testosterone including oral, intramuscular, and transdermal such as patches and gels. Testosterone therapy should be avoided in individuals with breast and/or prostate cancer.
166:
Females may present with low levels of estrogen and abnormal menstruation. Individuals with Turner syndrome may have short stature, dysmorphic features, gonadal dysgenesis, and delayed puberty. Other signs and symptoms associated with HH consist of intellectual disability or learning difficulties and
430:
In females with HH, estrogen therapy is done initially for breast development and pubertal induction. Pubertal induction should start no later than the age of 12 to maximize height growth and for benefits to outweigh adverse effects. Estrogen therapy, commonly using ethinylestradiol, should start at
421:
administration in males and females, respectively. Therapies should be individualized based on individuals needs to help develop and maintain secondary sexual characteristics. In males, androgen therapy is usually either done by induction of endogenous testosterone production by hCG or by exogenous
386:
Individuals with hypergonadotropic hypogonadism also exhibit gonadotropin levels (FSH and LH) that are above normal range and gonadal hormone levels (estrogen in females and testosterone in males) that are below normal range, so these biochemical parameters will be measured via a blood test. However
364:
Family history including age, healthy pubertal development of family members, and possibility of genetic disease will be evaluated. Prenatal history, such as maternal medication use, birthweight of the affected individual, childhood surgical interventions, and overall general health will also play a
373:
Individuals will be examined for height, weight, and any abnormal body features. Breast and genitalia examinations in presence of a chaperone may also be needed. Diagnostic imaging such ultrasound, computerized tomography (CT), and magnetic resonance imaging (MRI) can be done to evaluate for any
443:
and constitutional delay of growth and puberty (CDGP). The therapy is initiated as 15 to 25% of that of adults doses, then it gradually increased over 4 to 6 months. 50 to 100mg of testosterone ester is given intramuscularly every 2 to 4 weeks. Therapy is lifelong in boys who have permanent
400:
Hypergonadotropic hypogonadism can be caused by a variety of genetic and acquired factors. Common genetic causes include Turner syndrome and
Klinefelter syndrome, while acquired causes may involve infections, trauma, radiation, or chemotherapy affecting the gonads.
391:
can also be done to evaluate for any chromosomal abnormalities. Blood tests to check levels of prolactin, iron and thyroid hormones can be done to diagnose HH. Semen analysis can be another way to measure the sperm count to help diagnose individuals with HH.
387:
for young males before adolescence, anti-MĂĽllerian hormone (AMH) levels may be more indicative of HH since only small amounts of testosterone will be produced prior to the reactivation of the HPG axis during adolescence. Karyotyping and molecular
365:
significant role during diagnosis. In children with delayed puberty, hypogonadism can be distinguished from constitutional delay through family history, with constitutional delay being closely associated with positive family history.
1027:
108:
production. As compensation and the lack of negative feedback, gonadotropin levels are elevated. Individuals with HH have an intact and functioning hypothalamus and pituitary glands (of the
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923:
Kalkanli A, Akdere H, Cevik G, Salabas E, Cilesiz NC, Kadioglu A (2021). "Hypergonadotropic
Hypogonadism: Management of Infertility".
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1333:
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abnormalities of the internal genitalia, tumors in the pituitary gland or in the brain, and ovarian cysts for possibilities of
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215:
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1433:
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1478:
1448:
1305:
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158:) sperm count, loss of body hair, loss of muscle mass, hot flashes, psychological disturbances and poor sleep pattern.
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685:
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Other complications that can arise include anxiety and depression, osteoporosis and relationship problems.
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During birth and early infancy, HH in males can present as normal male phenotype with or without
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There are a multitude of etiologies for HH and it can include congenital or acquired causes.
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Symptoms can vary greatly depending on the stage of life, biological sex, and etiology.
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delayed puberty including amenorrhea and absent breast and pubic hair development.
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155:
105:
1182:
1130:
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828:
739:
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1105:
120:, but the majority of cases are of the former nature. HH can be treated with
317:
1190:
1142:
1066:
1004:
944:
902:
836:
747:
1290:
1212:
Papadakis MA, McPhee SJ, Bernstein J, eds. (2022). "Hypogonadism, Male".
530:
526:
494:
418:
414:
285:
281:
112:) so they are still able to produce FSH and LH. HH may present as either
345:
301:
1171:
Best
Practice & Research. Clinical Endocrinology & Metabolism
985:
Best
Practice & Research. Clinical Endocrinology & Metabolism
728:
Best
Practice & Research. Clinical Endocrinology & Metabolism
341:
1045:
Dye AM, Nelson GB, Diaz-Thomas A (January 2018). "Delayed
Puberty".
1223:
1026:
Vogiatzi MG (2022-01-24). Windle ML, Bercu BB, Griffing GT (eds.).
815:
Richard-Eaglin A (September 2018). "Male and Female
Hypogonadism".
498:
490:
325:
269:
89:
983:
Ladjouze A, Donaldson M (June 2019). "Primary gonadal failure".
726:
Ladjouze A, Donaldson M (June 2019). "Primary gonadal failure".
333:
332:), infection, kidney disease, liver disease, iron overload, and
247:
1227:
241:
in females, and LH and FSH resistance due to mutations in the
1131:"Hypogonadotropic Hypogonadism (HH) and Gonadotropin Therapy"
558:. In Winter WE, Holmquist B, Sokoll LJ, Bertholf RL (eds.).
556:"Chapter 5 – Endocrine disorders of the reproductive system"
1133:. In Feingold KR, Anawalt B, Boyce A, Chrousos G (eds.).
1082:"Etiology and Treatment of Hypogonadism in Adolescents"
1080:
Viswanathan, Vidhya; Eugster, Erica A. (October 2011).
869:"Etiology and treatment of hypogonadism in adolescents"
1167:"Management of hypogonadism from birth to adolescence"
439:
In males with HH, most of the studies have focused on
810:
808:
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862:
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858:
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854:
769:
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562:(Third ed.). Academic Press. pp. 157–180.
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1324:
1298:
1289:
1261:
686:"Low Sex Drive (Hypogonadism): Symptoms, Treatment"
45:
37:
32:
549:
547:
233:) – carbohydrate-deficient glycoprotein syndrome,
1525:Gonadotropin-releasing hormone and gonadotropins
1239:
605:
603:
601:
8:
1173:. Issue Update in paediatric endocrinology.
356:A diagnosis can be made from the following:
88:which is due to an impaired response of the
1311:17β-Hydroxysteroid dehydrogenase deficiency
1295:
1246:
1232:
1224:
867:Viswanathan V, Eugster EA (October 2011).
29:
1439:Cytochrome P450 oxidoreductase deficiency
1137:. South Dartmouth (MA): MDText.com, Inc.
1113:
892:
650:. University Science Books. p. 363.
110:hypothalamic-pituitary-gonadal (HPA) axis
1354:Familial male-limited precocious puberty
1349:Complete androgen insensitivity syndrome
268:(due to damage to or dysfunction of the
212:complete androgen insensitivity syndrome
1344:Partial androgen insensitivity syndrome
1214:Quick Medical Diagnosis & Treatment
543:
182:Congenital causes include the following
1129:Hayes F, Dwyer A, Pitteloud N (2000).
225:Gonadotropin resistance (e.g., due to
1339:Mild androgen insensitivity syndrome
1160:
1158:
1028:"Hypogonadism Differential Diagnoses"
978:
976:
974:
972:
970:
968:
966:
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222:, and testicular regression sequence.
190:Disorders of Sex Development (DSD) –
7:
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916:
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912:
817:The Nursing Clinics of North America
721:
719:
717:
715:
713:
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709:
707:
705:
680:
678:
676:
674:
560:Handbook of Diagnostic Endocrinology
1165:Howard SR, Dunkel L (August 2018).
237:(or insensitivity to LH) in males,
1086:Pediatric Clinics of North America
873:Pediatric Clinics of North America
568:10.1016/B978-0-12-818277-2.00005-4
554:Ferguson AM, Cervinski MA (2021).
210:, partial androgen insensitivity,
25:
937:10.2174/1381612826666201102110456
777:Principles of Molecular Medicine
409:Treatment of HH is usually with
1494:Hypergonadotropic hypergonadism
1459:Estrogen insensitivity syndrome
1334:Androgen insensitivity syndrome
464:Hypergonadotropic hypergonadism
41:Peripheral/gonadal hypogonadism
18:Hypergonadotrophic hypogonadism
1464:Hypergonadotropic hypogonadism
819:. Syndromes in Organ Failure.
774:Runge MS, Patterson C (2006).
616:. Springer. pp. 207–208.
272:) include testicular torsion,
216:congenital adrenal hyperplasia
58:Hypergonadotropic hypogonadism
33:Hypergonadotropic hypogonadism
1:
1469:Hypogonadotropic hypogonadism
1434:Postorgasmic illness syndrome
925:Current Pharmaceutical Design
780:. Humana Press. p. 463.
647:Biology of Human Reproduction
459:Hypogonadotropic hypogonadism
322:sexually-transmitted diseases
1520:Gynaecological endocrinology
1479:Estrogen-dependent condition
1449:Androgen-dependent condition
1059:10.3928/19382359-20171215-01
104:(LH), and in turn a lack of
98:follicle-stimulating hormone
411:hormone replacement therapy
376:polycystic ovarian syndrome
294:ovarian resistance syndrome
122:hormone replacement therapy
1541:
1489:Gonadotropin insensitivity
1370:Sertoli cell-only syndrome
1183:10.1016/j.beem.2018.05.011
997:10.1016/j.beem.2019.101295
829:10.1016/j.cnur.2018.04.006
740:10.1016/j.beem.2019.101295
131:
84:which is characterized by
1515:Endocrine gonad disorders
1316:Aromatase excess syndrome
1306:5α-reductase 2 deficiency
1276:Premature ovarian failure
1271:Polycystic ovary syndrome
1098:10.1016/j.pcl.2011.07.009
885:10.1016/j.pcl.2011.07.009
290:premature ovarian failure
1444:Cytochrome b5 deficiency
613:Cancer and Sexual Health
435:Testosterone Replacement
252:pseudohypoparathyroidism
1474:Fertile eunuch syndrome
78:primary gonadal failure
441:Klinefelter's syndrome
235:Leydig cell hypoplasia
231:gonadotropin receptors
227:inactivating mutations
196:Klinefelter's syndrome
204:XX gonadal dysgenesis
1454:Aromatase deficiency
426:Estrogen Replacement
330:endocrine disruptors
74:gonadal hypogonadism
1484:Premature thelarche
610:Mulhall JP (2011).
102:luteinizing hormone
1407:Precocious puberty
879:(5): 1181–200, x.
258:Myotonic Dystrophy
128:Signs and symptoms
1502:
1501:
1378:
1377:
1326:Androgen receptor
931:(24): 2790–2795.
787:978-1-58829-202-5
657:978-1-891389-12-2
623:978-1-60761-915-4
577:978-0-12-818277-2
239:FSH insensitivity
192:Turner's syndrome
64:), also known as
55:
54:
27:Medical condition
16:(Redirected from
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1429:Hyperestrogenism
1424:Hyperandrogenism
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1255:Gonadal disorder
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1092:(5): 1181–1200.
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1047:Pediatric Annals
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644:Piñón R (2002).
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1414:Hypoandrogenism
1395:Delayed puberty
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469:Delayed puberty
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389:genetic testing
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382:Further Testing
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274:ovarian torsion
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264:Acquired causes
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1216:. McGraw Hill.
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1177:(4): 355–372.
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1072:
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51:Endocrinology
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1146:. Retrieved
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816:
791:. Retrieved
776:
731:
727:
693:. Retrieved
689:
661:. Retrieved
646:
639:
627:. Retrieved
612:
589:. Retrieved
559:
523:Sex hormones
478:Hypothalamus
454:Hypogonadism
438:
429:
408:
399:
385:
372:
363:
355:
310:chemotherapy
306:autoimmunity
267:
242:
220:galactosemia
185:
177:
169:
165:
152:oligospermia
145:
137:
134:hypogonadism
117:
113:
86:hypogonadism
77:
73:
69:
65:
61:
57:
56:
987:. Puberty.
730:. Puberty.
473:infertility
369:Examination
156:azoospermia
106:sex steroid
38:Other names
1509:Categories
1291:Testicular
1148:2022-07-28
695:2022-07-28
591:2022-07-28
538:References
318:infections
132:See also:
114:congenital
100:(FSH) and
70:peripheral
1299:Enzymatic
1106:0031-3955
1013:198131801
953:226244221
756:198131801
586:225119774
531:estrogens
527:androgens
495:testicles
405:Treatment
352:Diagnosis
314:radiation
278:vanishing
208:mosaicism
186:Sources:
154:) or no (
82:condition
47:Specialty
1199:51934183
1191:30086863
1143:25905304
1135:Endotext
1067:29323692
1032:Medscape
1005:31327696
945:33138760
903:21981955
845:51966781
837:30100005
748:31327696
486:HPG axis
448:See also
419:estrogen
415:androgen
396:Etiology
378:(PCOS).
286:orchitis
282:anorchia
254:type 1A)
250:(termed
118:acquired
1383:General
1263:Ovarian
1115:4102132
894:4102132
793:10 June
663:10 June
629:10 June
499:ovaries
360:History
346:alcohol
342:opioids
336:(e.g.,
328:(e.g.,
320:(e.g.,
302:surgery
229:in the
162:Females
92:to the
80:, is a
66:primary
1197:
1189:
1141:
1112:
1104:
1065:
1011:
1003:
951:
943:
901:
891:
843:
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754:
746:
654:
620:
584:
574:
491:Gonads
484:, and
326:toxins
298:trauma
270:gonads
174:Causes
90:gonads
1363:Other
1195:S2CID
1009:S2CID
949:S2CID
841:S2CID
752:S2CID
582:S2CID
334:drugs
142:Males
1187:PMID
1139:PMID
1102:ISSN
1063:PMID
1001:PMID
941:PMID
899:PMID
833:PMID
795:2012
782:ISBN
744:PMID
665:2012
652:ISBN
631:2012
618:ISBN
572:ISBN
529:and
515:and
507:and
505:GnRH
497:and
471:and
417:and
248:gene
244:GNAS
1179:doi
1110:PMC
1094:doi
1055:doi
993:doi
933:doi
889:PMC
881:doi
825:doi
736:doi
564:doi
513:FSH
348:).
324:),
116:or
76:or
68:or
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1193:.
1185:.
1175:32
1169:.
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1108:.
1100:.
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1030:.
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989:33
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911:^
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887:.
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875:.
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803:^
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742:.
732:33
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673:^
600:^
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570:.
546:^
517:LH
480:,
344:,
340:,
316:,
312:,
308:,
304:,
300:,
296:,
292:,
288:,
284:,
276:,
218:,
214:,
206:,
202:,
198:,
194:,
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62:HH
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698:.
667:.
633:.
594:.
566::
533:)
525:(
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511:(
501:)
493:(
280:/
72:/
60:(
20:)
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