Adult-onset Idiopathic Hypogonadotropic Hypogonadism due to Isolated Pituitary Gonadotropin Deficiency

Suzuki, Fumiyuki; Shimizu, Chisato; Umetsu, Masaaki; Nagai, So; Takeuchi, Jun; Endo, Mareyuki; Miyoshi, Hideaki; Yoshioka, Narihito; Kubo, Masaaki; Koike, Takao

doi:10.2169/internalmedicine.43.571

Cited by 4 publications

(2 citation statements)

References 15 publications

(15 reference statements)

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“…Most clinical studies have found that administration of pulsive GnRH therapy can restore gonadotropin secretion and spermatogenesis, suggesting that GnRH neurons in the hypothalamus were impaired [3,10]. However, recent evidence from two cases determined that pituitary gonadotrophs were impaired because no increase of gonadotropins was evoked by pulsive GnRH stimulation [9,11]. As for our patients, the extremely low levels of gonadotropins and testosterone, which could not be stimulated by GnRHa, indicate severe and specifically damaged pituitary gonadotrophs.…”

Section: Dear Editormentioning

confidence: 53%

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Adult-onset idiopathic hypogonadotropic hypogonadism: possible aetiology, clinical manifestations and management

Mao¹,

Nie²,

Lu³

et al. 2010

Asian J Androl

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. Male idiopathic hypogonadotropic hypogonadism (IHH) is a congenital disease that manifests as small testes, a short penis and no pubertal development. Some patients exhibit cryptorchism and ambiguous genitalia [1]. IHH has been reported being caused by gene mutations in KAL-1, FGFR1, GnRHR, GPR54, and several other ligands and receptors [2]. For these patients, gonadotropins or testosterone is the primary choice of therapy. However, adult-onset IHH, defined as idiopathic isolated hypogonadotropic hypogonadism, is very rare, occurring in sexually mature men who have experienced full pubertal development, normal sexual activity and spermatogenesis [3]. The possible aetiology, clinical manifestations, management and prognosis in this disease are quite different from those of congenital IHH.We describe two male patients who experienced full pubertal development had active sexual lives and had impregnated their wives before experiencing symptoms of decreased libido, erectile dysfunction (ED) and gynaecomastia. Laboratory tests showed significantly decreased gonadotropins and testosterone as well as impaired spermatogenesis, while the other adenohypophyseal hormones were unaffected. Patient 1 received a recombinant human chorionic gonadotropin (HCG) + recombinant human FSH (rhFSH) combination regimen for 6 months, and his sperm count increased to be within the normal range. Patient 2 received testosterone therapy, and his sexual capability was restored. The aetiology of this disease is presumed to be autoimmunerelated, considering that only the thalamic-pituitarytestis axis is involved. Combined HCG and rhFSH therapy should be administered if the patient wants to regain spermatogenesis.Patient 1 was a 30-year-old man who was referred to our hospital because of decreased libido, ED for 5 months, and gynaecomastia for 3 months. He had experienced normal pubertal development and had been married for 8 years. He had four to five sexual activities per week and had impregnated his wife eight times. He did not have fever, fatigue, rapid weight loss, or shedding of pubic and axillary hair. Physical examination found his blood pressure to be 120/80 mmHg. He had gynaecomastia at P4, according to the Tanner-Stage score system. His testes were 20/20 mL in vo lume, according to Prader orchidometry, and soft when palpated; his pubic hair was stage V and penis length was 8 cm. Laboratory analysis found his transaminase, creatine and other parameters were all within the normal range. Routine blood and urinary tests were negative. Luteinizing hormone (LH) level was 0.0 mIU mL −1 (normal range 1.1-11.2 mIU mL ). Other laboratory data and the

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Section: Dear Editormentioning

confidence: 53%

“…Since then, only 10 more cases have been reported [9], including two patients from Japan [10,11]. The underlying mechanism has not yet been elucidated because of the rarity of this disease and lack of pathological and immunohistochemical information from hypothalamic or pituitary tissues.…”

Section: Dear Editormentioning

confidence: 99%

Adult-onset idiopathic hypogonadotropic hypogonadism: possible aetiology, clinical manifestations and management

Mao¹,

Nie²,

Lu³

et al. 2010

Asian J Androl

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Hormonal Treatment of Male Idiopathic/Isolated Hypogonadotropic Hypogonadism

Zhang

2010

Journal of Reproduction and Contraception

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An unusual cause of isolated secondary ovarian failure due to cerebral toxoplasmosis in an African woman with AIDS

Yoganathan

Sadiq

Yoganathan³

2017

BMJ Case Reports

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Primary ovarian failure is common. However, isolated secondary ovarian failure due to gonadotrophin deficiency is rare. A few cases of isolated gonadotrophin deficiency, due to congenital cerebral toxoplasmosis, have been described in children. We report the case of a 34-year-old African woman positive for HIV, who developed secondary amenorrhoea following the successful treatment of cerebral toxoplasmosis. Investigations revealed that she developed an isolated gonadotrophin deficiency due to pituitary lesion. The rest of the pituitary function dynamic tests were normal.

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Adult-onset Idiopathic Hypogonadotropic Hypogonadism due to Isolated Pituitary Gonadotropin Deficiency

Cited by 4 publications

References 15 publications

Adult-onset idiopathic hypogonadotropic hypogonadism: possible aetiology, clinical manifestations and management

Adult-onset idiopathic hypogonadotropic hypogonadism: possible aetiology, clinical manifestations and management

Hormonal Treatment of Male Idiopathic/Isolated Hypogonadotropic Hypogonadism

An unusual cause of isolated secondary ovarian failure due to cerebral toxoplasmosis in an African woman with AIDS

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