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[Two cases of Kallmann's syndrome].

F Kondo, H Fujii, Kohji Okamura, Sohei Tokunaka, T Takamura, S Yachiku

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Abstract

Herein we report two cases of hypogonadism with anosmia or hyposmia (Kallmann's syndrome), a 23-year-old single man (case 1) and a 34-year-old single man. (case 2). Micropenis and small testes were seen in both cases. Central anosima and central hyposmia were found in cases 1 and 2, respectively. Their karyotypes were normal (46, XY). The plasma levels of LH and FSH were low and the reaction to LH-RH test was poor in both cases. After repetitious LH-RH administrations, a good response of plasma LH was recognized in both cases except for a poor response of plasma FSH in case 1, while plasma testosterone remained low in both cases. Their testicular biopsies revealed immature testes without any developed Sertoli and Leydig cells.

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What this paper is about

Herein we report two cases of hypogonadism with anosmia or hyposmia (Kallmann's syndrome), a 23-year-old single man (case 1) and a 34-year-old single man. (case 2). Micropenis and small testes were seen in both cases. Central anosima and central hyposmia were found in cases 1 and 2, respectively. Their karyotypes were normal (46, XY). The plasma levels of LH and FSH were low and the reaction to LH-RH test was poor in both cases. After repetitious LH-RH administrations, a good response of plasma LH was recognized in both cases except for a poor response of plasma FSH in case 1, while plasma testosterone remained low in both cases. Their testicular biopsies revealed immature testes without any developed Sertoli and Leydig cells.

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Available abstract

Herein we report two cases of hypogonadism with anosmia or hyposmia (Kallmann's syndrome), a 23-year-old single man (case 1) and a 34-year-old single man. (case 2). Micropenis and small testes were seen in both cases. Central anosima and central hyposmia were found in cases 1 and 2, respectively. Their karyotypes were normal (46, XY). The plasma levels of LH and FSH were low and the reaction to LH-RH test was poor in both cases. After repetitious LH-RH administrations, a good response of plasma LH was recognized in both cases except for a poor response of plasma FSH in case 1, while plasma testosterone remained low in both cases. Their testicular biopsies revealed immature testes without any developed Sertoli and Leydig cells.

Key concepts: Hyposmia, Micropenis, Kallmann syndrome, Anosmia, Endocrinology, Internal medicine, Luteinizing hormone, Testosterone (patch)

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