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      Reduced Quality of Life and Sexual Satisfaction in Isolated Hypogonadotropic Hypogonadism

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          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.

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          Most cited references42

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          The global burden of mental disorders: An update from the WHO World Mental Health (WMH) Surveys

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            Self-rating depression scale in an outpatient clinic. Further validation of the SDS.

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              Expert consensus document: European Consensus Statement on congenital hypogonadotropic hypogonadism--pathogenesis, diagnosis and treatment.

              Congenital hypogonadotropic hypogonadism (CHH) is a rare disorder caused by the deficient production, secretion or action of gonadotropin-releasing hormone (GnRH), which is the master hormone regulating the reproductive axis. CHH is clinically and genetically heterogeneous, with >25 different causal genes identified to date. Clinically, the disorder is characterized by an absence of puberty and infertility. The association of CHH with a defective sense of smell (anosmia or hyposmia), which is found in ∼50% of patients with CHH is termed Kallmann syndrome and results from incomplete embryonic migration of GnRH-synthesizing neurons. CHH can be challenging to diagnose, particularly when attempting to differentiate it from constitutional delay of puberty. A timely diagnosis and treatment to induce puberty can be beneficial for sexual, bone and metabolic health, and might help minimize some of the psychological effects of CHH. In most cases, fertility can be induced using specialized treatment regimens and several predictors of outcome have been identified. Patients typically require lifelong treatment, yet ∼10-20% of patients exhibit a spontaneous recovery of reproductive function. This Consensus Statement summarizes approaches for the diagnosis and treatment of CHH and discusses important unanswered questions in the field.
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                Author and article information

                Contributors
                Role: Academic Editor
                Role: Academic Editor
                Journal
                J Clin Med
                J Clin Med
                jcm
                Journal of Clinical Medicine
                MDPI
                2077-0383
                14 June 2021
                June 2021
                : 10
                : 12
                : 2622
                Affiliations
                [1 ]Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland; pola.kompf@ 123456gmail.com (P.K.); kaziem@ 123456ump.edu.pl (K.Z.); mruchala@ 123456ump.edu.pl (M.R.)
                [2 ]Centre of Postgraduate Medical Education, Department of Reproductive Health, 01-004 Warsaw, Poland; mirab@ 123456cmkp.edu.pl
                [3 ]Department of Computer Science and Statistics, Poznan University of Medical Sciences, 60-806 Poznan, Poland; jmoczko@ 123456ump.edu.pl
                [4 ]Department of Otolaryngology, Head and Neck Surgery, Poznan University of Medical Sciences, 60-355 Poznan, Poland; jarek.kaluzny@ 123456gmail.com
                Author notes
                [* ]Correspondence: mkaluzna@ 123456ump.edu.pl ; Tel.: +48-69-5599966
                Author information
                https://orcid.org/0000-0002-2116-4384
                https://orcid.org/0000-0002-3121-3055
                https://orcid.org/0000-0001-5377-8834
                https://orcid.org/0000-0002-6296-7220
                Article
                jcm-10-02622
                10.3390/jcm10122622
                8232286
                8ce14a00-efca-4ec2-a022-1518643d743d
                © 2021 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( https://creativecommons.org/licenses/by/4.0/).

                History
                : 13 May 2021
                : 10 June 2021
                Categories
                Article

                isolated hypogonadotropic hypogonadism (ihh),kallmann syndrome (ks),health-related quality of life (hrqol)

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