PMID- 10374130
OWN - NLM
STAT- MEDLINE
DCOM- 19990729
LR  - 20190513
IS  - 0268-1161 (Print)
IS  - 0268-1161 (Linking)
VI  - 14
IP  - 1
DP  - 1999 Jan
TI  - Secondary infertility as early symptom in a man with multiple endocrine 
      neoplasia-type 1.
PG  - 252-4
AB  - Multiple endocrine neoplasia-type 1 (MEN1) is an autosomal dominant familial 
      cancer syndrome characterized by parathyroid hyperplasia, pancreatic endocrine 
      tumours and pituitary adenomas. Here, we report a patient with a history of 
      insulinoma who developed secondary infertility as a further symptom of the 
      disease. When he was first examined at the age of 36 years, he complained of 
      weakness, reduced libido and impotence. Laboratory evaluation revealed 
      non-obstructive azoospermia and hyperprolactinaemia. In contrast to sexual 
      activity and serum prolactin, semen quality did not significantly respond to 
      bromocriptine therapy. During follow-up, a growing pituitary adenoma caused 
      acromegaly with elevated serum concentrations of growth hormone, insulin-like 
      growth factor 1 (IGF-1), and prolactin. After microsurgery of the tumour at the 
      age of 44 years, sperm concentration persistently increased up to 5.6 x 10(6)/ml. 
      In accordance with the clinical diagnosis of MEN1, DNA sequencing revealed a 
      mutation in exon 2 of the menin gene which results in a truncated, inactive 
      protein product. In conclusion, MEN1 with pituitary lesions may cause severe 
      hypogonadism and infertility. Both hyperprolactinaemia and overproduction of 
      growth hormone and IGF-1 seem to be involved in testicular dysfunction in the 
      present case. The possible role of menin in the testis, however, remains to be 
      elucidated.
FAU - Schuppe, H C
AU  - Schuppe HC
AD  - Department of Dermatology, Heinrich Heine University, Dusseldorf, Germany.
FAU - Neumann, N J
AU  - Neumann NJ
FAU - Schock-Skasa, G
AU  - Schock-Skasa G
FAU - Hoppner, W
AU  - Hoppner W
FAU - Feldkamp, J
AU  - Feldkamp J
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - Hum Reprod
JT  - Human reproduction (Oxford, England)
JID - 8701199
RN  - 0 (Hormone Antagonists)
RN  - 0 (MEN1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 3A64E3G5ZO (Bromocriptine)
SB  - IM
MH  - Adenoma/complications/surgery
MH  - Adult
MH  - Bromocriptine/therapeutic use
MH  - Erectile Dysfunction/etiology
MH  - Exons
MH  - Hormone Antagonists/therapeutic use
MH  - Humans
MH  - Hyperprolactinemia/drug therapy/etiology
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*complications/genetics
MH  - Mutation/genetics/physiology
MH  - Neoplasm Proteins/genetics
MH  - Oligospermia/*etiology
MH  - Pituitary Neoplasms/complications/surgery
MH  - Postoperative Period
MH  - *Proto-Oncogene Proteins
MH  - Sperm Count
EDAT- 1999/06/22 00:00
MHDA- 1999/06/22 00:01
CRDT- 1999/06/22 00:00
PHST- 1999/06/22 00:00 [pubmed]
PHST- 1999/06/22 00:01 [medline]
PHST- 1999/06/22 00:00 [entrez]
AID - 10.1093/humrep/14.1.252 [doi]
PST - ppublish
SO  - Hum Reprod. 1999 Jan;14(1):252-4. doi: 10.1093/humrep/14.1.252.