Abstract
(1) Background: Isolated hypogonadotropic hypogonadism (IHH) is a genetic condition characterized by impaired puberty and fertility. IHH can significantly impact patient health-related quality of life (HRQoL), sexual satisfaction (SS) and mood. (2) Methods: Participants included 132 IHH subjects (89 men and 43 women) and 132 sex- and age-matched controls. HRQoL, depressive symptoms, erectile dysfunction (ED), and SS were assessed in an online survey using the Zung Self-Rating Depression Scale (SDS), 15D instrument of HRQoL (15D), Sexual Satisfaction Questionnaire (SSQ), and 5-item International Index of Erectile Function (IIEF-5). (3) Results: QoL and SS were significantly lower in the IHH group vs. controls. There was a high rate of ED (53.2% vs. 33%, p = 0.008) and depressive symptoms (45.00 ± 17.00 vs. 32.00 ± 12.00, p < 0.001) in patients vs. controls. The age of patients at IHH diagnosis inversely correlated with their overall 15D scores. An alarming non-compliance rate was seen (51.6%). No differences were found between scores of patients receiving hormone replacement therapy (HRT) and untreated subjects in any of the scales. (4) Conclusions: The HRQoL, SS, ED, and depression levels observed in IHH patients, despite HRT, are alarming. Late IHH diagnosis may have a particularly negative impact on HRQoL. More attention should be devoted to HRT adherence and various HRQoL aspects of IHH patients.
Highlights
Isolated hypogonadotropic hypogonadism (IHH), known as isolated gonadotropinreleasing hormone (GnRH) deficiency or idiopathic hypogonadotropic hypogonadism, is a developmental disorder of the hypothalamic–pituitary axis, causing GnRH deficiency
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A highly significant difference was seen between patients and controls in the education level
Summary
Isolated hypogonadotropic hypogonadism (IHH), known as isolated gonadotropinreleasing hormone (GnRH) deficiency or idiopathic hypogonadotropic hypogonadism, is a developmental disorder of the hypothalamic–pituitary axis, causing GnRH deficiency. IHH can be subdivided into two main types characterized by varying degrees of smell disturbances—the normosmic IHH (nIHH) and Kallmann syndrome (KS). A diminished sense of smell (hyposmia) or its absence (anosmia) is one of the most distinguishing features of KS. At least 10% of IHH develops in adulthood [5]. A spontaneous or druginduced reversal form of hypogonadism, which can occur in about 10–22% of IHH cases, could be overlooked by patients and physicians [6,7,8]. IHH is a condition that is highly heterogeneous epidemiologically, clinically, and genetically [3,4]
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