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Hypogonadotropic Hypogonadism Type 22
Hypogonadotropic Hypogonadism 22

Presentation

The clinical findings of the family presented in this study are very similar to the two cases reported by Al-Awadi and Mégarbané (Table), and it may be suggested that this is the report of a 3rd family with a similar clinical presentation. [jpma.org.pk]

GnRH could not be analysed in the present patient because of technical and financial difficulties. Empty sella is a radiological diagnosis based on CT or MRI investigation. [atmph.org]

Abstract Hormon therapy of the female Kallmann’s Syndrome patient in the setting of a pulmonary embolism presents a challenge. [pagepress.org]

Page 259 - any substance that, when present at low concentrations compared with those of an oxidizable substrate, significantly delays or prevents oxidative damage to that substrate". [books.google.com]

Figure 5 Table 2: Genetic disorders presenting with delayed puberty, adapted from Clarisa When prepubertally acquired male hypogonadism presents with scanty body and terminal facial hair, high pitched voice, female distribution of pubic hair, small testis [ispub.com]

Workup

The patient had brought in his complete file with workups from the eight previous physicians, which included normal pulmonary function tests, normal EKG and Holter monitor evaluation, normal echocardiography, normal chemistry, complete blood count, urine [healio.com]

Diagnosis and Treatment After our usual workup, and the obvious diagnosis of hyopogonadal hypogonadism, treatment was started with HCG (human chorionic gonadotropin), an LH analog which stimulates testicular testosterone production. [jeffreydachmd.com]

Treatment

Duration of treatment cycle will be up to 14 days, or maximum of 21 days (if follicular maturation is imminent, based upon follicular growth and E2 levels). Total duration will be of 3 treatment cycles. [clinicaltrials.gov]

Basal serum inhibin B levels were slightly higher before transdermal estradiol treatment (phase 1) than during estrogen treatment (phase 2) in both subjects. [jeffreydachmd.com]

Others have noted that it gets gradually worse on treatment and then continues to worsen when treatment is stopped. [rxisk.org]

Cancer treatment. Chemotherapy or radiation therapy for the treatment of cancer can interfere with testosterone and sperm production. The effects of both treatments often are temporary, but permanent infertility may occur. [mayoclinic.org]

I am currently not on testosterone treatment. Instead, I take hCG (human chorionic gonadotropin). [kallmannsyndrome.wordpress.com]

Prognosis

In these cases, the correction of hypogonadism neither affects the progress of the disease nor improves prognosis 24. [ispub.com]

Prognosis Many forms of hypogonadism are potentially treatable and have a good prognosis. Prevention People should maintain normal body weight and have healthy eating habits to prevent anorexia nervosa. Other causes may not be preventable. [healthofchildren.com]

Guild The course and prognosis of reticulsendotheliosis (eosinophilic granuloma SchullerChrstian disease and leterer-Siwe disease) Amer. J. Med, 32 (1957), pp. 636 [23.] P. Enriquez, D.C. Dahlin, A.B. Hayles, E.D. [archbronconeumol.org]

The prognosis for successful stimulation of spermatogenesis in men with hypogonadotropic hypogonadism treated with hCG/hMG is good and not adversely affected by prior androgen treatment. [atmph.org]

Etiology

Causes [ edit ] There are a multitude of different etiologies of HH. [en.wikipedia.org]

If the patient has normal pubertal development and a uterus, the most common etiology is congenital outflow tract obstruction with a transverse vaginal septum or imperforate hymen. [aafp.org]

The empty sella: A reappraisal of etiology and pathogenesis. Acta Neurol Scand Suppl 1990;130:1-25. [ PUBMED ] 5. Pocecco M, De Campo O, Marinoni S, Tommasini G, Basso T, Muzzolini C, et al. [atmph.org]

Our case reports suggest that maybe screening for hypogonadism in all HIV-infected men might help to understand its etiology. [edmcasereports.com]

Zinkham Multifocal eosinophilic granuloma Natural history, etiology and management. Amer. J. Med, 60 (1976), pp. 452 [25.] Case Records of the M. G. H N. Engl J Med Case, 5 (1978), [26.] R.V. Randall, E.C. Clark, R.C. [archbronconeumol.org]

Epidemiology

Relevant External Links for FEZF1 Genetic Association Database (GAD) FEZF1 Human Genome Epidemiology (HuGE) Navigator FEZF1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: FEZF1 No data available for Genatlas for FEZF1 Gene Mutations in [genecards.org]

Epidemiologic studies have led to the division of febrile seizures into 3 groups, as follows: Simple febrile seizures Complex febrile seizures Symptomatic febrile seizures Children with simple febrile seizures are neurologically and developmentally healthy [centogene.com]

[…] described the phenotype and prevalence of CCDC141 mutations in idiopathic hypogonadotropic hypogonadism/Kallmann syndrome confirmed that inactivating CCDC141 variants cause normosmic idiopathic hypogonadotropic hypogonadism but not Kallmann syndrome. [24] Epidemiology [emedicine.medscape.com]

The epidemiology of polycystic ovary syndrome. Prevalence and associated disease risks. Endocrinol Metab Clin North Am. 1999;28:247–63. 23. Chang RJ, Katz SE. Diagnosis of polycystic ovarian syndrome. [aafp.org]

Epidemiology 3.10. List of Distributors 3.11. Key Participants Market Presence (Intensity Map) By Region 4. Global Male Hypogonadism Market Analysis and Forecast 2012-2026 4.1. [gii.co.jp]

Pathophysiology

Kallmann syndrome caused by mutations in the PROK2 and PROKR2 genes: pathophysiology and genotype-phenotype correlations. Front Horm Res 2010;39:121–32. Crossref PubMed Google Scholar 22. Kim HG, Ahn JW, Kurth I, Ullmann R, Kim HT, et al. [degruyter.com]

Gonadotropin-releasing hormone deficiency in the human (idiopathic hypogonadotropic hypogonadism and Kallmann’s syndrome): pathophysiological and genetic considerations. Endocr Rev 19: 521, 1998. [link.springer.com]

In most cases, the pathophysiological mechanisms are hypothalamic dysfunction in conjunction with direct gonadal involvement. [ispub.com]

Pathophysiology Deficient hypothalamic GnRH secretion underlies the markedly abnormal gonadotropin secretion patterns in most patients with Kallmann syndrome or idiopathic hypogonadotropic hypogonadism. [emedicine.medscape.com]

Gonadotropin-releasing hormone deficiency in the human (idiopathic hypogonadotropic hypogonadism and Kallmann’s syndrome): pathophysiological and genetic considerations Endocr Rev 1998 ; 19 : 521-539 [cross-ref] [3] Young J, Vincent D, Bachelot A, Kottler [em-consulte.com]

Prevention

[…] of HIV HIV Transmission and Prevention in Specific Populations Systematic Review of HIV Behavioral Prevention Research in African Americans Lynae A. [hivinsite.ucsf.edu]

Prevention People should maintain normal body weight and have healthy eating habits to prevent anorexia nervosa. Other causes may not be preventable. [healthofchildren.com]

Page 259 - any substance that, when present at low concentrations compared with those of an oxidizable substrate, significantly delays or prevents oxidative damage to that substrate". [books.google.com]

More information Testosterone Deficiency (Primary Hypogonadism and Secondary/Hypogonadotrophic Hypogonadism) Prevention In some cases, hypogonadism is caused by genetic factors and cannot be prevented. [myvmc.com]

In one patient, isolated bioinactive luteinizing hormone (LH) was present as a result of a homozygous mutation in the LH beta subunit gene, which prevented binding of LH to its receptor. [emedicine.medscape.com]

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