Mwana wenyu anonoka kusvika pakuyaruka here? Haana kana kunzwa kwekunhuhwidza here? Ingava Kallmann Syndrome here?

Kunyangwe mwana wako akura zvishoma, haaratidzi zviratidzo zvekuyaruka seshamwari dzake here? Kana kuti wakaona here kuti haanzwi kunhuhwirira kumwe chete, semuenzaniso, kunhuhwirira kwemaruva kana chikafu? Zvinhu zvakaita seizvi zvinogona kukonzera kushushikana uye kunetseka mupfungwa dzedu sevabereki. Ndosaka, nhasi tichataura nezvechirwere chinoratidza zviratidzo izvi, asi chisingataurwi zvakanyanya munharaunda, asi chakakosha kuziva. Icho chirwere chinonzi Kallmann Syndrome .

Chii chinonzi Kallmann Syndrome iyi?

Zvichitaurwa zviri nyore, Kallmann Syndrome chirwere chisingawanzoitiki chemajini . Chinhu chikuru chinoitika muizvi ndechekuti kuyaruka kwemwana kunononoka zvikuru . Mune zvimwe zviitiko, kuyaruka kunogona kumira zvachose. Pamusoro pezvo, vanhu vazhinji vane chirwere ichi vanorasikirwa zvakanyanya kana kuti zvachose nekunzwa kwekunhuhwidza . Tinodaidzawo izvi kuti "anosmia" mumashoko ekurapa.

Chikonzero chechirwere ichi ndechekuti mwana achiri mudumbu raamai vake, dzimwe shanduko dzinoitika mune mamwe majini ane chekuita nekukura kwemuviri wake. Funga nezvazvo sekukanganisa kudiki mupurogiramu yekombuta. Dzimwe nguva shanduko idzi dzemajini dzinogona kugara nhaka kubva kuvabereki kuenda kuvana. Kureva kuti, mwana anowana hunhu uhwu hwemajini kubva kuna amai kana baba. Zvisinei, mune dzimwe nguva, vanachiremba vanoti shanduko idzi dzemajini dzinogona kuitika zvisina tsarukano, pasina nhoroondo yemhuri yakajeka.

Dambudziko iri rinowanzoitika kuvakomana kupfuura kuvasikana. Kazhinji, vabereki navanachiremba vanoritarisa zvakanyanya kana mwana asvika pakuyaruka zvisina kujairika. Saka, kazhinji, Kallmann Syndrome inoonekwa pakati pemakore masere negumi nemashanu.

Vanachiremba dzimwe nguva vanoshandisa rimwe zita rechirwere ichi, iro rinonzi "hypogonadotropic hypogonadism" . Kunyange zvazvo izvi zvinganzwika zvakaoma, zvinongoreva kuti machende evakomana nemazai evasikana haabudisi mahomoni epabonde akakosha pakuyaruka uye kushanda pabonde. Unonzwisisa here? Zvinoreva kuti pane kushomeka kwemamwe mahomoni mumuviri.

Ndezvipi zviratidzo zveKallmann syndrome?

Iye zvino ngationei zviratidzo zvinoratidzwa nemunhu ane Kallmann Syndrome. Zvimwe zvezviratidzo izvi zvinogona kuonekwa achiri mudiki, uye zvimwe zvinogona kuonekwa kunyangwe mushure mekukura. Havasi vese vanozova nezviratidzo zvakafanana.

Chinhu chikuru hunhu hune chekuita nekuyaruka:

• Muvasikana, kukura kwemazamu hakubvi zvachose kana kuti kushoma kwazvo .
• Kune vasikanaKuenda kumwedzi (kuenda kumwedzi) kungasaitike zvachose, kana kuti kungaitika kunonoka kupfuura zvakajairika uye zvisina kurongeka.
• Nhengo yemurume nemboro yevakomana idiki pane yakajairika .
• Kusabereka zvinoreva kudzikira kana kurasikirwa nekukwanisa kubereka vana muvarume nevakadzi panguva yekukura.

Mukuwedzera, mamwe mabasa anogona kuonekwa:

• Kurasikirwa zvachose nekunzwa kwekunhuhwidza (anosmia) kana kunhuhwidza kushoma zvakanyanya chimwe chinhu chinoratidza Kallmann syndrome.
• Vamwe vanhu vanogona kusangana nematambudziko ekuenzanisa muviri pavanenge vachifamba kana kumira .
• Kashoma , palate yakatsemuka , kureva kuti, pakatsemuka pamuromo wepamusoro, panogona kuitika.
• Matambudziko emazino , semuenzaniso, mazino asina kana mazino madiki asingawanzoitiki (kusagadzikana kwemazino).
• Matambudziko ekufamba kwemaziso , akadai se nystagmus, inova mamiriro ezvinhu apo maziso anofamba nekukurumidza uye asingadzoreki.
• Kugara ndichinzwa kuneta zvakanyanya (Kuneta) .
• Vakadzi vanoenda kumwedzi zvisina kurongeka .
• Kuda zvepabonde zvishoma .
• Kuchinja kamwe kamwe kwemanzwiro , dzimwe nguva kuchiperekedzwa nekunzwa kunetseka, kusuruvara, uye hasha.
• Kashoma, chirwere chinonzi "Renal agenesis" chinoberekwa chisina itsvo imwe chete .
• Vamwe vanhu vanotanga kukombama kwemusana (Scoliosis) .
• Kuwedzera uremu pasina chikonzero chakajeka .

Chinhu chakakosha ndechekuti havasi vese vane zviratidzo izvi zvese. Vamwe vanhu vanogona kutorasikirwa nekunzwa kwekunhuhwidza. Muzviitiko zvakadaro, vanachiremba vanodana chirwere ichi kuti ``normosmic idiopathic hypogonadotropic hypogonadism (nIHH)``. Izvi zvinoreva kuti kunzwa kwekunhuhwidza kwakajairika, asi dambudziko remahormone riripo.

Sei mamiriro ezvinhu aya achiitika?

Zvakanaka, ikozvino ungangove uchishamisika kuti, 'Sei izvi zvichiitika? Chii chinokonzera?' Chikonzero chikuru cheKallmann Syndrome kuchinja kana kukanganisa mune mamwe majini mumuviri wedu . Kuchinja uku kunokanganisa maitiro akaomarara anodzora kuyaruka kwemwana, ayo anotangira muuropi.

Kazhinji, maitiro aya ekuyaruka anotangira mugland rinokosha muuropi hwemwana rinonzi hypothalamus, iro rinoburitsa hormone inonzi Gonadotropin-Releasing Hormone (GnRH).Nekugadzirwa kwekemikari inonzi. Funga nezvazvo `(GnRH)` iyi yakafanana ne``master switch`` inotanga maitiro ese ekuyaruka. Mumwana ane Kallmann syndrome, hypothalamus haibudisi zvakakwana iyi `(GnRH)` hormone, kana kuti pamwe haibudisi zvachose. Saka, nekuti iyo ``master switch`` haisi ``on``, maitiro ekuyaruka haatangi nemazvo.

Homoni iyi `(GnRH)` ndiyo inobatsira pakukura pabonde, chishuwo chepabonde, uye kugona kuva nevana mune ramangwana (kubereka). Homoni iyi inofamba nemuropa ichienda kugland repituitary, rimwe gland rinokosha muuropi. Homoni iyi `(GnRH)` inobva yapa chiratidzo kugland repituitary kuti rigadzire mamwe mahormone maviri. Aya ndiwo:

• Homoni inokurudzira follicle (FSH)
• Hormone yeLuteinizing (LH)

Mahormone maviri aya, `(FSH)` uye `(LH)`, anoenda zvakananga kumaovari evasikana nematesticles evakomana, zvichivabatsira kugadzira mahormone epabonde (akadai seestrogen netestosterone) uye zvichikonzera kukura kwepabonde. Saka, kana pasina `(GnRH)`, cheni yese iyi inokanganiswa.

Chikonzero chekushaikwa kwekunhuhwirira kwakabatanawo nekuchinja kwemajini uku. Kumwe kuchinja kwemajini kunokanganisawo kugona kweuropi hwemwana kunhuhwirira. Kazhinji, masero etsinga dzinonhuhwirira mumhino dzedu anoona kunhuhwirira kwakasiyana uye anotumira ruzivo irworwo kubhabhu rinonhuhwirira muuropi (chikamu cheuropi chinokonzera kunhuhwirira). MuKallmann syndrome, pane dambudziko nekukura kwemasero aya etsinga dzinonhuhwirira kana kubatana kwawo neuropi. Nekuda kweizvozvo, zviratidzo pamusoro pekunhuhwirira hazvisvike kuuropi zvakanaka.

Simba remajini riri sei?

Vaongorori vaona kusiyana kwemajini kunopfuura makumi maviri neshanu kunokonzera Kallmann syndrome nedzimwe mamiriro e "hypogonadotropic hypogonadism". Izvi zvinoreva kuti chirwere ichi chingangove chinokonzerwa nemajini anopfuura rimwe chete. Paaya, majini akati wandei akawanikwa aine hukama hwakasimba nechirwere ichi:

• `KAL1 (ANOS1)`
• `CHD7`
• `FGFR1`
• `GNRHR`
• `IL17RD`
• `PROK2`
• `SOX10`
• ``TACR3''

Kuchinja kwemajini uku kunoitika panguva yemwana achiri mudumbu raamai vake. Dzimwe nguva kuchinja uku kunogona kuitika zvisina tsarukano, pasina chikonzero. Zvisinei, kazhinji, kuchinja uku kunogona kugara nhaka kubva kuvabereki kuenda kuvana.

Kune nzira dzakasiyana-siyana dzinoshandiswa pakupa shanduko idzi dzemajini kuvana. Tinodaidza nzira idzi kuti:

• Kugara nhaka yeAutosomal recessive: Muchiitiko ichi, vabereki vese vanotakura kusiyana kwemajini (zvichireva kuti havana zviratidzo, asi vane jini rakakanganisika). Kuti mwana ave nechirwere ichi, vabereki vese vanofanira kugara nhaka makopi ese ejini rakakanganisika.
• Kugara nhaka yeAutosomal dominant: Dambudziko iri rinogona kuitika kunyangwe mwana akagara nhaka kopi imwe chete yejini risina kunaka kubva kune mumwe mubereki (angave amai kana baba).
• Kugara nhaka kweX-linked: Muchiitiko ichi, jini risina kunaka riri paX chromosome. Izvi zvinogona kukanganisa varume zvakanyanya.

Kunyangwe izvi zviri chokwadi chakadzama chekurapa, ndinofunga kuti zvakakosha kuti tinzwisise kuti chirwere ichi chingapfuudzwa sei kubva kuchizvarwa kuenda kune chimwe kuburikidza nemajini.

Ndezvipi zvinetso zvinogona kukonzera izvi?

Kuyaruka kwemwana chinhu chakakosha zvikuru mukukura kwake mumuviri nepfungwa. Kallmann Syndrome inogona kudzivirira mwana kusvika padanho iroro sezvaitarisirwa. Izvi zvinoreva kuti kukura kwemwana pabonde kunogona kunonoka kana zvichienzaniswa nevamwe vana vezera rimwe chete, kana kutomira zvachose.

Fungidzira, kana mwana mudiki aine vamwe shamwari dzezera rake, zvinova zvakaoma sei kuona kuti miviri yeshamwari dzake iri kuchinja (sekukura murefu, kuchinja inzwi rake, kukura ndebvu, kukura mazamu), asi yavo isiri kuchinja? Vanganzwa kunyara uye kusakosha nezvechitarisiko chavo. Vanganzwa sekunge vakasiyana uye vakaparadzaniswa nevamwe. Nekuda kwezvinhu zvakaita seizvi, mwana anogona kunge aine matambudziko epfungwa akadai sekuora mwoyo kana kuzvidya mwoyo zvakanyanya . Zvingave zvakaoma kutaurirana nevamwe kuchikoro uye munharaunda.

Saka, zvakakosha zvikuru kupa kurapa kwemuviri kumwana ane chirwere ichi, pamwe nekutarisira hutano hwepfungwa dzake, uye kupa mazano kana zvichidikanwa . Rutsigiro rwevabereki nevadzidzisi runokoshawo pano.

Vanachiremba vanozviwana sei izvi?

Kazhinji, kana muine zvinokunetsai nezvekukura kwemwana wenyu, semuenzaniso, kana asati atanga kuratidza zviratidzo zvekuyaruka pazera remakore 13-14, munotanga maenda kunoona chiremba wevana. Neimwe nzira, munogonawo kuona chiremba we endocrinologist.

Chinhu chekutanga chinoitwa nachiremba ndechekuongorora mwana zvakakwana . Kureva kuti, vanoongorora hurefu, huremu, uye zviratidzo zvekuyaruka (zvakaita sekukura kwemazamu nekukura kwenhengo dzesikarudzi). Zvadaro vanobvunza mwana nemi (vabereki) nezvekuchinja kwemuviri kunowanzoonekwa nevana pakati pemakore masere negumi nemashanu. Vanogonawo kubvunza kana paine munhu mumhuri akambomira kuyaruka kana kuti asina kusvika pakuyaruka zvachose. Vanobvunzawo kana paine dambudziko nekunzwa kwekunhuhwirira.

Kana chiremba akafungidzira kuti Kallmann Syndrome ane chirwere ichi, anogona kukurudzira bvunzo dzakawanda, dzakadai se:

• Kuongororwa kweropa:Iyi ndeimwe yemiedzo yakakosha. Inotarisa huwandu hwemahormone akasiyana-siyana mumuviri. Zvikuru sei, mahormone epituitary atakataura nezvawo kare, anonzi FSH neLH, uye mahormone epabonde testosterone (muvakomana) uye estrogen (muvasikana). Mahormone aya anowanzo kuve akaderera muKallmann syndrome.
• Miedzo yekutarisa kunhuhwirira: Iyi bvunzo iri nyore. Mwana anopiwa kunhuhwirira kwakasiyana-siyana kwaanoziva (semuenzaniso kofi, sipo, mint) uye anokumbirwa kuti azive kunhuhwirira kwacho.
• Kuongororwa kwemajini: Izvi zvinosanganisira kutsvaga kusiyana kwemajini chaiwo muropa ratakataura nezvaro kare rine chekuita neKallmann syndrome. Zvisinei, bvunzo idzi hadziwanzoitwa uye dzinogona kudhura zvishoma. Dzinongoitwa chete kana zvichidikanwa, mushure meimwe bvunzo.
• Dzimwe nguva uropi hunogona kutorwa neMRI scan kuti vaone kana paine matambudziko ne hypothalamus ne pituitary glands, kana kuti hapana kukura kwe olfactory bulb.

Pane mishonga here?

Nerombo rakanaka, kune mishonga inoshanda yeKallmann Syndrome. Mushonga mukuru ndeyekutsiva mahormone (HRT) . Izvi zvinosanganisira kupa muviri mahormone ausiri kugadzira zvakasikwa kana kuti ari kugadzira zvishoma. Tariro ndeyekuti mishonga iyi ichabatsira kutanga kuyaruka, kukudziridza hunhu hwepabonde hwechipiri (sekunge bvudzi rechiso nekukura kwemazamu), uye kusimbisa mapfupa.

Zvisinei, marapirwo nemarudzi emahormone anopiwa zvinogona kusiyana kubva pamunhu kuenda kumunhu, zvichienderana nekuti mwana mukomana here kana musikana, uye nezera.

Heano mamwe emishonga inonyanya kushandiswa:

• Kuvakomana: Testosterone ndiyo hormone inopihwa. Inogona kupihwa sejekiseni (kamwe chete pamwedzi), semapete eganda, kana semagel eganda anoiswa zuva nezuva .
• Kuvasikana: Estrogen ndiyo inotanga yapihwa. Gare gare, inosanganiswa neprogesterone kuti iite kuti munhu aende kumwedzi. Izvi zvinogona kupihwa semapiritsi kana semavanga eganda.
• Dzimwe nguva, kunyanya kune vakuru vari kuronga kuva nevana, kurapwa neGnRH hormone pump kana jekiseni remahormone akafanana neFSH neLH, akadai seHCG (human chorionic gonadotropin) uye hMG (human menopausal gonadotropin), kunogona kupihwa kukurudzira ovulation (muvakadzi) kana kugadzirwa kwembeu (muvarume).

Mushure mekutanga kurapwa uku, chiremba achagara achiongorora mwana, oongorora huwandu hwemahormone, uye ogadzirisa mwero wekurapwa sezvinodiwa.

Chii chaungatarisira kana uchirarama nechirwere ichi?

Mwana ane chirwere ichi angangoda kutora mushonga wekutsiva mahormone kweupenyu hwake hwese kana kwenguva yakareba . Ndizvo zvinoitika.

Asi mashoko akanaka ndeekuti varume nevakadzi vane Kallmann syndrome vanogona kuwanazve simba rekubereka nekurapwa nemahormone kwakakodzera. Kune mishonga chaiyo yekurapa izvi. Vamwe varume vanogona kuramba vachibereka mbeu mushure mekunge kurapwa kwamira. Vanachiremba vanodana izvi kuti 'reversible Kallmann syndrome' . Asi izvi hazviitiki kune wese munhu.

Kukura nekupinda mukuyaruka inguva ine matambudziko akawanda muhupenyu. Kuva neKallmann Syndrome kunogona kuita kuti dambudziko iroro riwedzere. Izvi zvinogona kukonzera kunonoka kuyaruka, kana kutotadza zvachose kuyaruka. Saka, kana mwana wako aine chirwere ichi, angasasangana nekuchinja kwemuviri sezvinoita vamwe vake. Izvi zvinogona kumuita kuti anzwe kunetseka nezvechitarisiko chake, anzwe kunyara, uye aedze kuzviparadzanisa nevamwe. Vanogona kunzwa sekunge vari kusiiwa kunze, kana kuti vakasiyana.

Kunyangwe pasina zuva kana nguva yakatarwa yekuyaruka, kana muine mibvunzo kana zvinokunetsai nezvekukura kwemwana wenyu, kunyanya kukura pabonde, chinhu chakanakisa chekuita kutaura nachiremba wevana kana nyanzvi yemahormone . Vanogona kuongorora iwe nemwana wenyu nemazvo uye kupa gwara rinodiwa uye kurapwa.

Mharidzo Yekuenda Nayo Kumba

Zvakanaka, kubva pane zvatakurukura zvakadzama, ndinovimba une pfungwa yakanaka uye yakajeka nezveKallmann Syndrome.

Muchidimbu, chirwere cheKallmann chirwere chisingawanzoitiki chemajini chinonyanya kunonoka kuyaruka uye chinowanzokonzera kurasikirwa nekunzwa kwekunhuhwidza.

• Dambudziko guru mune izvi iketani yekugadzira mahormone inotangira muuropi.
• Chirwere ichi chinogona kubata varume nevakadzi , asi chinowanzoitika kuvakomana.
• Zviratidzo zvinogona kusanganisira zvinhu zvakaita sekusaratidza zviratidzo zvekuyaruka pazera rakakodzera, kurasikirwa kana kudzikira kwekunhuhwidza, matambudziko emazino, uye matambudziko ekufamba kwemaziso.
• Kurapa nehomoni ndiko kurapwa kukuru. Kurapa uku kunogona kudiwa muhupenyu hwese.
• Kurapwa kunogona kukonzera kuyaruka uye kudzorera kugona kwekuva nevana .
• Rutsigiro rwepfungwa nekupa mazano zvakakoshawo zvikuru kuvana nevakuru vane chirwere ichi.
• Kana muine kusava nechokwadi nezvekuyaruka kwemwana wenyu, musanonoka kuenda kuna chiremba . Kana zvikaonekwa nekukurumidza, zvinova nyore kutanga kurapwa uye kuderedza matambudziko angangoitika.

Tinovimba nemoyo wese kuti ruzivo urwu rwakubatsira. Yeukai kuti hamusi mega murwendo urwu. Kune vanachiremba nevapi vemazano vane hunyanzvi muSri Lanka kuti vabatsire nekutungamira avo vari kusangana nemamiriro ezvinhu akafanana.

Kallmann Syndrome, kunonoka kuyaruka, kurasikirwa nekunhuhwidza, kushaya simba remuviri, matambudziko emahormone, zvirwere zvemajini, hypogonadotropic hypogonadism