Wolfram Syndrome with Primary Gonadal Insufficiency

Serhat Özcelik, Mehmet Sariyadin, Suleyman Bas, Bünyamin Aydin, Sibel Temiz, Kenan Caglayan, Mehmet Celik, Muhammed Kizilgul and Hulya Iliksu Gözür
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Bünyamin Aydin: Haydarpaşa Training and Research Hospital, Endocrinology and Metabolism Section, Turkey
Kenan Caglayan: Department of Endocrinology and Metabolism, Trakya University, Turkey
Mehmet Celik: Endocrinology and Metabolism Section, Kilis State Hospital, Turkey
Hulya Iliksu Gözür: Department of Endocrinology and Metabolism, Marmara University, Turkey

Global Journal of Reproductive Medicine, 2018, vol. 4, issue 3, 40-42

Abstract: A 24-year old male patient admitted to our clinic with the complaints of mouth dryness, polyuria, and polydipsia. He was diagnosed with tip 1 Diabetes Mellitus (DM) at the age of seven and intensive insulin therapy was instituted subsequently. Despite the stable blood glucose levels, the daily liquid intake of the patient was 10 liters, and the urine output was 9.5 liters. The patient also had complaints of loss of libido, impotence, erectile and ejaculatory dysfunction in the last 6 months. The insulin therapy was given as insulin aspart 4-unit t.i.d and insulin detemir 12 unit per day. In the physical examination, bilateral gynecomastia was observed. In the hormonal panel; FSH: 45 U/L (N:1.5-12.7), LH:28.4 U/L (N:1.7-8.6), total testosterone: 3.83ng/ml (N:3-10ng/ml), free testosterone: 2.15pg/ml (N:12,00-30,00pg/ml). Bone mineral densitometry revealed low bone mass for chronologic age with the Z score of -2.3 SD. Spermiogram revealed azoospermia. Male karyotype 46, XY was confirmed in the cytogenetic studies. The fluid deprivation test was done and according to the test results the patient was diagnosed with diabetes insipidus. In the fundus examination, bilateral optic atrophy was found out. In the audiogram, the hearing loss with high-frequency sounds was considered to be associated with sensorineural deafness. As the treatment of primary hypogonadism, testosterone was given. As in our case, the patients diagnosed with WS requires long-term follow-up. With early diagnosis and appropriate hormone replacement the quality of patient’s life and prognosis ameliorates

Keywords: Journal of Reproductive Medicine; Reproductive Medicine; journal of reproductive medicine; reproductive medicine articles; reproductive medicine articles impact factor; reproductive medicine research journals; reproductive journals list; open access; juniper publishers journals; juniper publishers reivew (search for similar items in EconPapers)
Date: 2018
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Persistent link: https://EconPapers.repec.org/RePEc:adp:jgjorm:v:4:y:2018:i:3:p:40-42

DOI: 10.19080/GJORM.2018.04.555636

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