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Hypogonadotropic Hypogonadism

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1. Assessing Sex Steroid Influence on Kisspeptin Responsiveness in Idiopathic Hypogonadotropic Hypogonadism (PubMed)

Assessing Sex Steroid Influence on Kisspeptin Responsiveness in Idiopathic Hypogonadotropic Hypogonadism Individuals with idiopathic hypogonadotropic hypogonadism (IHH), even those with evidence of some hypothalamic reproductive endocrine activity, fail to complete puberty and fail to respond to physiologic doses of kisspeptin.This case series examined whether treatment with sex steroids could stimulate kisspeptin responsiveness in patients with IHH.This was a case series.This study

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2018 Journal of the Endocrine Society

2. Clinical management of congenital hypogonadotropic hypogonadism. (PubMed)

Clinical management of congenital hypogonadotropic hypogonadism. The initiation and maintenance of reproductive capacity in humans is dependent upon pulsatile secretion of the hypothalamic hormone gonadtropin-releasing hormone, GnRH. Congenital hypogonadotropic hypogonadism (CHH) is a rare disorder that results from the failure of the normal episodic GnRH secretion, leading to delayed puberty and infertility. CHH can be associated with an absent sense of smell, also termed Kallmann syndrome (...) a significant portion of male patients (around 10-20%) exhibit a spontaneous recovery of their reproductive function. Finally, fertility can be induced with pulsatile GnRH treatment or gonadotropin regimens in a majority of patients. In summary, this review is a comprehensive synthesis of the current literature available regarding the diagnosis, patient management and genetic foundations of congenital hypogonadotropic hypogonadism relative to normal reproductive development.

2019 Endocrine Reviews

3. Effect of testosterone on FGF2, MRF4 and myostatin in hypogonadotropic hypogonadism: relevance to muscle growth. (PubMed)

Effect of testosterone on FGF2, MRF4 and myostatin in hypogonadotropic hypogonadism: relevance to muscle growth. Basic Fibroblast growth factor (FGF2) is an important stimulatory modulator of satellite cells in the skeletal muscle, which play a cardinal role in muscle growth and repair.We evaluated whether the skeletal muscle expression of FGF2 and muscle growth and differentiation factors are reduced in patients with hypogonadotropic hypogonadism (HH), and whether testosterone replacement

2019 Journal of Clinical Endocrinology and Metabolism

4. GENETICS IN ENDOCRINOLOGY: Genetic counseling for congenital hypogonadotropic hypogonadism and Kallmann syndrome: new challenges in the era of oligogenism and next-generation sequencing. (PubMed)

GENETICS IN ENDOCRINOLOGY: Genetic counseling for congenital hypogonadotropic hypogonadism and Kallmann syndrome: new challenges in the era of oligogenism and next-generation sequencing. Congenital hypogonadotropic hypogonadism (CHH) and Kallmann syndrome (KS) are rare, related diseases that prevent normal pubertal development and cause infertility in affected men and women. However, the infertility carries a good prognosis as increasing numbers of patients with CHH/KS are now able to have

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2018 European Journal of Endocrinology

5. Efficacy and Safety of Long Term Use of hCG or hCG Plus hMG in Males With Isolated Hypogonadotropic Hypogonadism (IHH)

Efficacy and Safety of Long Term Use of hCG or hCG Plus hMG in Males With Isolated Hypogonadotropic Hypogonadism (IHH) Efficacy and Safety of Long Term Use of hCG or hCG Plus hMG in Males With Isolated Hypogonadotropic Hypogonadism (IHH) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum (...) number of saved studies (100). Please remove one or more studies before adding more. Efficacy and Safety of Long Term Use of hCG or hCG Plus hMG in Males With Isolated Hypogonadotropic Hypogonadism (IHH) (IHH) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our for details

2018 Clinical Trials

6. Assisted reproductive techniques with congenital hypogonadotropic hypogonadism patients: a systematic review and meta-analysis. (PubMed)

Assisted reproductive techniques with congenital hypogonadotropic hypogonadism patients: a systematic review and meta-analysis. After hormonal replacement therapy (HRT) including androgen replacement or sequential therapy of estrogen and progesterone, The combination of human chorionic gonadotropin (hCG) and human menopausal gonadotropin (hMG) and pulsatile GnRH, is not sufficient to produce sufficient gametes in some patients with Congenital hypogonadotropic hypogonadism (CHH). A Systematic (...) . The keywords and major terms covered "hypogonadotropic hypogonadism", "kallmann syndrome", "assisted reproductive techniques", "intrauterine insemination", "intracytoplasmic sperm injection", "testicular sperm extraction", "in vitro fertilization", "embryo transplantation" and "intra-Fallopian transfer".A total of 388 pregnancies occurred among 709 CHH patients who received ART (effectiveness 46, 95% confidence interval 0.39 to 0.53) in the 20 studies we included. The I2 in trials assessing overall

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2018 BMC Endocrine Disorders

7. Xanthomatous hypophysitis causing hypogonadotropic hypogonadism resulting in delayed presentation of slipped capital femoral epiphysis. (PubMed)

Xanthomatous hypophysitis causing hypogonadotropic hypogonadism resulting in delayed presentation of slipped capital femoral epiphysis. An 18-year-old man who underwent bilateral pinning of his hip joints after a left unstable Slipped Capital Femoral Epiphysis (right pinned prophylactically) was noted to have delayed secondary sexual characteristics and post-operative diabetes insipidus. The patient also described a history of fatigue, headache and polydipsia for the past 4 years. Endocrine (...) versa the risk of SCFE should be considered in patients with prolonged hypogonadotropic hypogonadism.

2018 British Journal of Neurosurgery

8. Hypogonadotropic Hypogonadism in Men With Diabesity. (PubMed)

Hypogonadotropic Hypogonadism in Men With Diabesity. One-third of men with obesity or type 2 diabetes have subnormal free testosterone concentrations. The lower free testosterone concentrations are observed in obese men at all ages, including adolescents at completion of puberty. The gonadotropin concentrations in these males are inappropriately normal; thus, these patients have hypogonadotropic hypogonadism (HH). The causative mechanism of diabesity-induced HH is yet to be defined

2018 Diabetes Care

9. Congenital Hypogonadotropic Hypogonadism and Constitutional Delay of Growth and Puberty Have Distinct Genetic Architectures. (PubMed)

Congenital Hypogonadotropic Hypogonadism and Constitutional Delay of Growth and Puberty Have Distinct Genetic Architectures. Congenital hypogonadotropic hypogonadism (CHH) and constitutional delay of growth and puberty (CDGP) represent rare and common forms of GnRH deficiency, respectively. Both CDGP and CHH present with delayed puberty, and the distinction between these two entities during early adolescence is challenging. More than 30 genes have been implicated in CHH, while the genetic basis

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2018 European Journal of Endocrinology

10. MRI of ectopic posterior pituitary gland with dysgenesis of pituitary stalk in a patient with hypogonadotropic hypogonadism (PubMed)

MRI of ectopic posterior pituitary gland with dysgenesis of pituitary stalk in a patient with hypogonadotropic hypogonadism The ectopic posterior pituitary is a rare condition which is characterized by the ectopic location of posterior lobe of pituitary, pituitary stalk abnormalities, and associated clinical manifestations of anterior lobe related growth hormone dysfunction or less commonly multiple anterior pituitary dysfunctions. We present a rare case of posterior ectopic pituitary (...) and pituitary stalk hypoplasia with isolated hypogonadotropic hypogonadism in a 36-year-old female patient.

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2018 Radiology Case Reports

11. Prevalence and Associated Phenotypes of PLXNA1 Variants in Normosmic and Anosmic Idiopathic Hypogonadotropic Hypogonadism. (PubMed)

Prevalence and Associated Phenotypes of PLXNA1 Variants in Normosmic and Anosmic Idiopathic Hypogonadotropic Hypogonadism. Idiopathic hypogonadotropic hypogonadism (IHH) can be divided into two major forms, normosmic IHH and Kallmann syndrome (KS). Genetic mutations are responsible for the majority of IHH. PLXNA1 has recently been implicated in the GnRH neuron migration and the etiology of KS. We aimed to investigate the prevalence and associated phenotypes of PLXNA1 variants in a large cohort

2018 Clinical Genetics

12. Evaluation of Sexual Function in Women with Hypogonadotropic Hypogonadism Using the Female Sexual Function Index (FSFI) and the Beck Depression Inventory (BDI) (PubMed)

Evaluation of Sexual Function in Women with Hypogonadotropic Hypogonadism Using the Female Sexual Function Index (FSFI) and the Beck Depression Inventory (BDI) BACKGROUND Hypogonadotropic hypogonadism (HH), or secondary hypogonadism, results from reduced secretion of gonadotropins, including follicle-stimulating hormone (FSH) and luteinizing hormone (LH), by the pituitary gland, resulting in lack of production of sex steroids. The aim of this study was to evaluate self-reported sexual function

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2018 Medical science monitor : international medical journal of experimental and clinical research

13. Hypogonadotropic Hypogonadism and Kleefstra Syndrome due to a Pathogenic Variant in the EHMT1 Gene: An Underrecognized Association (PubMed)

Hypogonadotropic Hypogonadism and Kleefstra Syndrome due to a Pathogenic Variant in the EHMT1 Gene: An Underrecognized Association Kleefstra syndrome is a genetic condition characterized by intellectual disability, childhood hypotonia, and facial dysmorphisms. Genital anomalies such as micropenis, cryptorchidism, and hypospadias have been reported in 30-40% of males diagnosed with the disease. However, endocrinological investigations have been limited. We describe a case of an adolescent male (...) with Kleefstra syndrome due to a pathogenic variant in the EHMT1 gene whose workup for isolated micropenis is suggestive of a partial hypogonadotropic hypogonadism. A possible endocrine mechanism of the genital anomaly associated with Kleefstra syndrome is discussed.

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2018 Case reports in endocrinology

14. Mutation profiles and clinical characteristics of Chinese males with isolated hypogonadotropic hypogonadism. (PubMed)

Mutation profiles and clinical characteristics of Chinese males with isolated hypogonadotropic hypogonadism. To investigate the mutation profiles and clinical characteristics of Chinese males with isolated hypogonadotropic hypogonadism (IHH) and discover new pathogenic genes that cause IHH.A gene panel, including 31 known IHH genes and 52 candidate genes, was used to perform semiconductor next-generation sequencing.University hospital.One hundred thirty-eight sporadic male IHH patients and 10

2018 Fertility and Sterility

15. Predictive factors for pituitary response to pulsatile GnRH therapy in patients with congenital hypogonadotropic hypogonadism (PubMed)

Predictive factors for pituitary response to pulsatile GnRH therapy in patients with congenital hypogonadotropic hypogonadism Pulsatile gonadotropin-releasing hormone (GnRH) may induce spermatogenesis in most patients with congenital hypogonadotropic hypogonadism (CHH) by stimulating gonadotropin production, while the predictors for a pituitary response to pulsatile GnRH therapy were rarely investigated. Therefore, the aim of our study is to investigate predictors of the pituitary response

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2018 Asian journal of andrology

16. Semen quality in patients with pituitary disease and adult-onset hypogonadotropic hypogonadism (PubMed)

Semen quality in patients with pituitary disease and adult-onset hypogonadotropic hypogonadism Gonadotropins (luteinizing hormone (LH) and follicle-stimulating hormone (FSH)) are released from the pituitary gland and stimulate Leydig cells to produce testosterone and initiates spermatogenesis. Little is known about how and when the deterioration of semen quality occurs in patients with adult-onset gonadotropin insufficiency.A retrospective study comprising 20 testosterone-deficient men (median

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2018 Endocrine connections

17. Congenital hypogonadotropic hypogonadism, functional hypogonadotropism or constitutional delay of growth and puberty? An analysis of a large patient series from a single tertiary center. (PubMed)

Congenital hypogonadotropic hypogonadism, functional hypogonadotropism or constitutional delay of growth and puberty? An analysis of a large patient series from a single tertiary center. What diagnoses underlie delayed puberty (DP) and predict its outcome?A multitude of different diagnoses underlie DP, and in boys a history of cryptorchidism, small testicular size and slow growth velocity (GV) predict its clinical course.DP is caused by a variety of underlying etiologies. Hormonal markers can (...) etiologies that underlie DP were identified. No markers of clinical value could be identified in the girls, whereas a history of cryptorchidism in the boys was associated with an increase in the risk of permanent hypogonadism (odds ratio 17.2 (95% CI; 3.4-85.4, P < 0.001)). The conditions that cause functional hypogonadotropic hypogonadism were more frequent in boys with a GV below 3 cm/yr than in those growing faster (19% vs 4%, P < 0.05). In this series, the most effective markers to discriminate

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2016 Human Reproduction

18. Efficacy and Safety of Corifollitropin Alfa (MK-8962) in Combination With Human Chorionic Gonadotropin (hCG) in Adolescent Males With Hypogonadotropic Hypogonadism (HH)

Efficacy and Safety of Corifollitropin Alfa (MK-8962) in Combination With Human Chorionic Gonadotropin (hCG) in Adolescent Males With Hypogonadotropic Hypogonadism (HH) Efficacy and Safety of Corifollitropin Alfa (MK-8962) in Combination With Human Chorionic Gonadotropin (hCG) in Adolescent Males With Hypogonadotropic Hypogonadism (HH) - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search (...) for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Efficacy and Safety of Corifollitropin Alfa (MK-8962) in Combination With Human Chorionic Gonadotropin (hCG) in Adolescent Males With Hypogonadotropic Hypogonadism (HH) The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been

2017 Clinical Trials

19. Human chorionic gonadotropin (hCG) for Improved Virility without Negative Behavioral Changes in Hypogonadotropic Hypogonadic Adolescent Males with Prader-Willi Syndrome

Human chorionic gonadotropin (hCG) for Improved Virility without Negative Behavioral Changes in Hypogonadotropic Hypogonadic Adolescent Males with Prader-Willi Syndrome "Human chorionic gonadotropin (hCG) for Improved Virility without Negat" by Peter Bodmer < > > > > > Title Author Date of Graduation Summer 8-8-2015 Degree Type Capstone Project Degree Name Master of Science in Physician Assistant Studies First Advisor Mark Pedemonte, MD Rights . Abstract Background: Prader-Willi Syndrome (PWS (...) and potential treatments, including depo-testosterone, etc. Improved studies (including RCTs) and study designs could also further improvements in the management of PWS-hypogonadism. Challenges include the global rarity of the disease, and finding enough subjects to provide adequate study populations. Recommended Citation Bodmer, Peter, "Human chorionic gonadotropin (hCG) for Improved Virility without Negative Behavioral Changes in Hypogonadotropic Hypogonadic Adolescent Males with Prader-Willi Syndrome

2015 Pacific University EBM Capstone Project

20. Expanding the genetic spectrum of ANOS1 mutations in patients with congenital hypogonadotropic hypogonadism. (PubMed)

Expanding the genetic spectrum of ANOS1 mutations in patients with congenital hypogonadotropic hypogonadism. What is the prevalence and functional consequence of ANOS1 (KAL1) mutations in a group of men with congenital hypogonadotropic hypogonadism (CHH)?Three of forty-two (7.1%) patients presented ANOS1 mutations, including a novel splice site mutation leading to exon skipping and a novel contiguous gene deletion associated with ichthyosis.CHH is characterized by lack of pubertal development

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2017 Human Reproduction

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