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Hypogonadotropic Hypogonadism Type 22

HH22


Presentation

  • 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]
  • 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]
  • I started having a chain of symptoms ,which came one after the other and are all present today, many of them gotten extremely worse and unbearable : -extreme fatigue - dry mouth , polyuria (about 6l per day) and nocturia – wake up to go to the toilet[allthingsmale.com]
  • 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]
Falling
  • In the event that a conventional test for hypogonadism is conducted, results typically fall within the normal reference range.[rxisk.org]
  • Testosterone treatment also caused a significant fall in circulating concentrations of free fatty acids, C-reactive protein, interleukin-1β, tumor necrosis factor-α, and leptin ( P 0.05 for all).[care.diabetesjournals.org]
Fishing
  • 40% proteins carbohydrates (the kind that remains for you to eat have minimal calories) for constipation and estrogen processing eat (carbohydrates) cabbage, brocoli, brussel sprouts, cauliflower the 40% protein ----- more than half of it should be fish[allthingsmale.com]
  • The karyotype, 46,XY,t(7;8)(p12.3;p11.2), was reconfirmed by GTG banding before breakpoint mapping by FISH.[jmg.bmj.com]
Inflammation
  • ) the effects of intramuscular testosterone replacement on insulin sensitivity, inflammation, and body composition.[care.diabetesjournals.org]
  • Many other mediators of inflammation are also suspected. Flares of SLE have also been linked to hyperestrogenic states 22.[ispub.com]
Overeating
  • Quinton Karger Medical and Scientific Publishers , ١٢‏/٠٤‏/٢٠١٠ - 184 من الصفحات Over the past decade, the understanding of the processes involved in the regulation of gonadotropin-releasing hormone and its dysfunction has greatly increased.[books.google.com]
  • While some have noted a remission of symptoms over time, most have been left fruitlessly attempting symptom management for years.[rxisk.org]
  • Drug Monographs 8000 prescription and over-the-counter drug monographs, including herbals and supplements Disease and Condition Articles 7600 diseases, conditions, and procedures articles enhanced with images and step-by-step videos Reference Tools Essential[medscape.org]
  • In men aged over 50, the doctor will usually also do a digital rectal examination to assess whether or not the man has prostate disease.[myvmc.com]
  • Save over 20% compared to the individual article price.[karger.com]
Constipation
  • -weight gain – not that noticeable,since I barely eat….that constipation is killing me!!![allthingsmale.com]
  • Anamnesis produced information about other symptoms such as tiredness (which could not, however, be quantified), skin dryness, dysphonia, oedema, constipation, and also showed that she had had her primary amenorrhoea controlled by the Gynaecologic Service[hormones.gr]
Dry Hair
  • - Fat gain on thighs and face, abdominal fat - Low blood pressure ( 80/40) and heart pulse (40 per min) - Ringing in the ears – 24/7 - Dry hair and face -Loss of pubic hair and absolutely NO libido,erectile disfunction - Brain fog – I cannot think straight[allthingsmale.com]
Loss of Pubic Hair
  • - Fat gain on thighs and face, abdominal fat - Low blood pressure ( 80/40) and heart pulse (40 per min) - Ringing in the ears – 24/7 - Dry hair and face -Loss of pubic hair and absolutely NO libido,erectile disfunction - Brain fog – I cannot think straight[allthingsmale.com]
Happy Personality
Fear
  • I have stopped dating girls for the fear of them wanting to have sex and me not even being able to. I have never had sex and now I will never get to experience how great it is and the wonderful orgasms I used to have.[rxisk.org]
Nocturia
  • I started having a chain of symptoms ,which came one after the other and are all present today, many of them gotten extremely worse and unbearable : -extreme fatigue - dry mouth , polyuria (about 6l per day) and nocturia – wake up to go to the toilet[allthingsmale.com]
Polyuria
  • I started having a chain of symptoms ,which came one after the other and are all present today, many of them gotten extremely worse and unbearable : -extreme fatigue - dry mouth , polyuria (about 6l per day) and nocturia – wake up to go to the toilet[allthingsmale.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]
  • Expert consensus document: European Consensus Statement on congenital hypogonadotropic hypogonadism-pathogenesis, diagnosis and treatment. Nat Rev Endocrinol 2015;11:547–64. Web of Science PubMed Google Scholar 2.[degruyter.com]
  • 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]

Prognosis

  • PMID: 11344554 Free PMC Article Prognosis Mitchell AL, Dwyer A, Pitteloud N, Quinton R Trends Endocrinol Metab 2011 Jul;22(7):249-58. Epub 2011 Apr 20 doi: 10.1016/j.tem.2011.03.002.[ncbi.nlm.nih.gov]
  • 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

  • Etiology Int J Pediatr Otorhinolaryngol 2018 Jul;110:135-139. Epub 2018 May 17 doi: 10.1016/j.ijporl.2018.05.011. PMID: 29859575 Gonçalves CI, Fonseca F, Borges T, Cunha F, Lemos MC Hum Reprod 2017 Mar 1;32(3):704-711. doi: 10.1093/humrep/dew354.[ncbi.nlm.nih.gov]
  • 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]
  • Our case reports suggest that maybe screening for hypogonadism in all HIV-infected men might help to understand its etiology.[edmcasereports.com]
  • 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]

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]
Sex distribution
Age distribution

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]
  • 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]
  • Seminara SB, Hayes FJ, Crowley WF Jr (1998) Gonadotropin-releasing hormone deficiency in the human (idiopathic hypogonadotropic hypogonadism and Kallmann's syndrome): pathophysiological and genetic considerations. Endocr Rev 19: 521–539.[journals.plos.org]

Prevention

  • 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]
  • […] 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]
  • 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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