Unoziva nezve ROHHAD Syndrome here? Mwana wako ane zviratidzo izvi here?

Mwana wako mudiki akatanga kukura zvakanyanya here? Kana kuti wakaona here kuti anonetseka kufema panguva yekurara? Dzimwe nguva izvi zvinogona kuva zviratidzo zvechirwere chisingawanzoitiki chinonzi ROHHAD Syndrome. Ngatitaurei nezvazvo zvakadzama, nekuti zvakakosha kuziva zvinhu zvakaita seizvi.

Chii chinonzi ROHHAD Syndrome?

Zvichitaurwa zviri nyore, ROHHAD Syndrome chirwere chisingawanzoitiki, chinoisa upenyu panjodzi chinokanganisa vana vadiki. Chinonyanya kukanganisa endocrine system, autonomic nervous system, uye kufema. Vana vane chirwere ichi vanogona kuwedzera uremu munguva pfupi . Vanogonawo kuona shanduko mumafambiro ekufema, maitiro, uye kurongeka kwemanzwiro pavanenge vakarara uye dzimwe nguva vakamuka.

Chirwere cheROHHAD chirwere chitsva chekurapa. Chakatanga kuzivikanwa nekutsanangurwa muna 1965. Kuti ufunge nezvazvo, kwave nevanhu vashoma pane mazana maviri vane chirwere ichi pasi rose. Saka unogona kufungidzira kuti hachiwanzoitiki sei. Nyanzvi dzezvekurapa dzichiri kutsvaga chirwere ichi kuti dziwane chikonzero chaicho uye dziwane mushonga usingaperi. Parizvino, hapana bvunzo chaiyo kana kurapwa kwechirwere cheROHHAD. Naizvozvo, vanachiremba vanorapa mwana wega wega zvichienderana nezviratidzo zvake.

Ndezvipi zviratidzo zvechirwere cheROHHAD?

Vana vane ROHHAD syndrome vanowanzova neutano hwakanaka uye vanokura zvakanaka vasati vaona zviratidzo. Kunyange zvazvo zviratidzo zvichigona kuonekwa pazera remakore mapfumbamwe, vana vazhinji vanoratidza zviratidzo zvavo zvekutanga pakati pemakore maviri nemana.

Zita rekuti 'ROHHAD' rinobva pamavara ekutanga ezviratidzo zvina zvikuru zvechirwere ichi. Ngationei kuti chii:

• (RO) Kufutisa Kukurumidza: Apa ndipo apo chido chemwana chinowedzera nekukurumidza, zvichikonzera kuwedzera uremu kwakakura kwemapaundi 20-30 (9-13 kg) mukati menguva pfupi, kazhinji pakati pemwedzi mitatu kusvika gumi nemiviri. Ichi chinowanzova chiratidzo chekutanga chekufutisa. Funga nezvazvo, hakusi kuwedzera uremu chete, asi kuchinja kunokurumidza uye kunoonekwa.

• (H) Kusashanda zvakanaka kwemuviri: Hypothalamus chikamu cheuropi hwako chinodzora gland repituitary. Inodzorawo zvinhu zvakawanda zvakaita sekukura kwemuviri, huremu, kuda kudya, kuyaruka, manzwiro, uye maitiro. Saka, kana paine dambudziko mukushanda kwayo, mwanaZviratidzo zvakaita sekufuta, kupfupika kwemuviri, pfari, kunonoka kukura, kuwedzera kana kudzikira kwekuita weti, uye kutanga kwekuyaruka kana kunonoka zvinogona kuitika. Pamusoro pezvo, zvirwere zvakaita sehypothyroidism uye chirwere cheshuga zvinogonawo kuitika.

• (H) Kufema Neropa Zvishoma: Vana vane ROHHAD syndrome havagone kudzora kufema kwavo nemazvo. Izvi zvinoreva kuti kana mwero weokisijeni muropa waderera kana mwero wecarbon dioxide ukawedzera, muviri haufeme zvakadzika kana nekukurumidza zvakakwana kuti udzore. Ichi chirwere chine njodzi zvikuru. Dzimwe nguva, chinogona kuitika kamwe kamwe, senge mushure mehutachiona hudiki hwekufema, senge dzihwa, kana mushure mekunzwa kuneta.

• (AD) Kusagadzikana Kwemuviri: Sisitimu yetsinga yemuviri yedu (ANS) inodzora mabasa ekutanga atisingakwanise kudzora, akadai sekufema, kugaya chikafu, kurova kwemoyo, uye tembiricha yemuviri. Saka, vana vane kukuvara kwesisitimu iyi yetsinga yemuviri vanogona kuona zviratidzo zvakaita sekurova kwemoyo kunonoka zvisina kujairika (Bradycardia), tembiricha yemuviri yakaderera, uye kudzikira kwekunzwisisa kurwadziwa . Fungidzira, kunzwa kurwadziwa kushoma zvinoreva kuti kunyangwe mutsaona yakakomba, mwana anogona kunge asingazvizive.

Ndezvipi zvikonzero zvechirwere cheROHHAD?

Kutaura zvazviri, vanachiremba havasati vawana chikonzero chaicho chechirwere cheROHHAD. Zvisinei, pane dzidziso huru nhatu dziri kutsvakurudzwa pari zvino dzinogona kuchitsanangura.

1. Genetics: Vaongorori vazhinji vanotenda kuti ROHHAD syndrome inokonzerwa nekuchinja kwemajini . Zvisinei, gene chaiyo haisati yazivikanwa. Zvese zviri mumuviri wedu zvinodzorwa nemajini, saka chero shanduko mairi inogona kuikanganisa.

2. Epigenetics: Vamwe vaongorori vanotenda kuti majini anodzora mamwe mabasa mumiviri yedu anogona kuvhurwa kana zvichidikanwa uye kudzimwa kana zvisingadiwi. Izvi zvinonzi epigenetics. Pfungwa iyi yakavakirwa pakudzidza kwakaitwa pamapatya akafanana. Mairi, mwana mumwe chete akatanga chirwere cheROHHAD, nepo mumwe asina. Saka, zvinogona kufungidzirwa kuti kwete majini chete, asiwo kusiyana kwemaitiro emajini iwayo kunogona kukanganisa izvi.

3. Kuzvidzivirira kwemuviri: Imwe tsvakiridzo inoratidza kuti chirwere cheROHHAD chirwere chinokonzerwawo nekuzvidzivirira kwemuviri.Pane hukama huripo pakati pezvirwere zve autoimmune, apo masoja emuviri anorwisa masero awo. Mune imwe ongororo, vana vaviri vane ROHHAD syndrome vakawanikwa vaine ma antibodies anonzi immunoglobulins mu cerebrospinal fluid (CSF) , mvura inotenderedza uropi nemuzongoza. Vachishandisa ruzivo urwu, vaongorori vakagumisa kuti paiva nekuzvimba mu central nervous system (CNS) , uropi nemuzongoza. Izvi zvaireva kuti masoja emuviri airwisa uropi zvisirizvo.

Ndezvipi zvinetso zvechirwere cheROHHAD?

Kuwedzera kune zviratidzo zvikuru zvatakambokurukura, vana vane ROHHAD syndrome vanogona kuwana mamwe matambudziko , kureva mamwe matambudziko ehutano. Zvakakosha kuziva izvi zvakare:

• Matambudziko epfungwa nemaitiro: Vana ava vanogona kusangana nemamiriro ezvinhu akadai sekusagadzikana , hasha, kushingaira zvakanyanya, kuneta nguva dzose, kuzvidya mwoyo, kuora mwoyo, uye kuremara kwepfungwa . Izvi zvinogona kukanganisa hupenyu hwemwana hwezuva nezuva pamwe nemhuri.

• Matambudziko etsinga: Vana vane ROHHAD syndrome vanogona kusangana nematambudziko akadai sepfari, mafambiro emaziso asina kujairika (nystagmus), kushaya hope, kana matambudziko ekukura .

• Matambudziko emetabolism: Matambudziko anowanzo itika mumetabolism pakati pevana ava iDiabetes Mellitus, insulin resistance, Impaired glucose gallness, uye Steatotic (fatty liver disease). Aya ndiwo mamiriro ezvinhu anogona kuva nemigumisiro yehutano kwenguva refu.

• Matumbu etsinga: Pakati pe40% ne56% yevana vane ROHHAD syndrome vanokura matumbu etsinga. Muenzaniso mumwe ndewerudzi rwebundu rinonzi ganglioneuroma . Matumbu mazhinji aya haana njodzi , asi dzimwe nguva anogona kuva akaipa . Saka, vanachiremba vanogara vachitsvaga izvi.

• Kumira kwemwoyo nekufema: Iri ndiro dambudziko rine njodzi huru. Kumira kufema kamwe kamwe uye kushanda kwemoyo. Izvi zvinogona kuisa hupenyu panjodzi.

Chirwere cheROHHAD chinowanikwa sei? (Kuongororwa)

Parizvino, chirwere cheROHHAD chinonziHapana bvunzo imwe chete inogona kusimbisa chirwere ichi. Pane kudaro, timu yenyanzvi dzezvekurapa inoshanda pamwe chete kuti iongorore chirwere ichi zvichibva pakusanganiswa kwezviratidzo zvemwana zvakasiyana . Zvakafanana nemupurisa, achiunganidza humbowo uye achisvika pamhedziso.

Kuti mwana aonekwe kuti ane ROHHAD syndrome, anofanira kunge achizadzisa zvinotevera:

• Zviratidzo zvekutanga kamwe kamwe kwekufuta zvakanyanya uye kushaya mweya unobuda panguva yekurara zvinofanira kunge zviripo.
• Zviratidzo zvekushaya simba kwemuviri mumuviri (semuenzaniso, kuwedzera uremu nekukurumidza, hypothyroidism, kana kuchinja kwekuyaruka - pakutanga kana kunonoka) zvinofanira kunge zviripo.
• Zvakakosha kusimbisa kuti hapana shanduko (gene variant) mujini `PHOX2B`. Izvi zvakakosha kuti tisiyanise kubva ku`CCHS (Congenital Central Hypoventilation Syndrome),` chimwe chirwere chine zvimwe zviratidzo zvakafanana neROHHAD syndrome (semuenzaniso, kudzikira kwekufema panguva yekurara).
• Zviratidzo izvi zvinogona kusaonekwa mumakore ekutanga ehupenyu. Zvinowanzotanga pave paya.

Nekuti chirwere cheROHHAD chirwere chisingawanzoitiki, vana vanogona kuongororwa zvisizvo pakutanga. Saka, kana uine zviratidzo izvi, zvakakosha kutsvaga mazano akakodzera kubva kuchikwata chevanachiremba vane ruzivo.

Chirwere cheROHHAD chinorapwa sei? (Kurapwa)

Kurapwa kwechirwere cheROHHAD kunosiyana kubva pamwana kuenda kumwana, zvichienderana nezviratidzo. Haasi mwana wese anogona kupihwa chirongwa chimwe chete chekurapa, nekuti mamiriro emunhu wese akasiyana. Zvisinei, chinhu chimwe chete chakafanana kune vese. Kana mwana ane chirwere cheROHHAD akaratidza kusakwana kwekufema panguva yekurara, achafanira kushandisa muchina unonzi `CPAP` kana `BiPAP` kuti umubatsire kufema. Nekufamba kwenguva, angangoda kushandisa `ventilator` kuti atsigire zvizere maitiro ekufema. Iyi inzira inoponesa hupenyu.

Pakurapa vana vane ROHHAD syndrome, rubatsiro rwevanachiremba vevana muzvikamu zvakasiyana-siyana runodiwa. Semuenzaniso:

• Vanachiremba vemapapu
• Vanachiremba vetsinga
• Vanachiremba veEndocrinologists (vanachiremba vanoongorora mahormone)
• Vanachiremba veOtolaryngologist (Nyanzvi dzeNzeve, Mhino, neHuro)
• Vanachiremba vemwoyo
• Nyanzvi dzehope
• Nyanzvi dzezvekudya
• Vanachiremba veOncology
• Nyanzvi dzepfungwa
• Vanachiremba vepfungwa

Kuburikidza nekubatana kwevanhu ava vese, tinoedza kupa mwana kurapwa kwakanakisisa.

Ramangwana revana vane ROHHAD syndrome rakaita sei? (Outlook)

Kutaura zvazviri, pari zvino hapana mushonga wechirwere cheROHHAD. Chinangwa chikuru chekurapa ndechekugadzirisa zviratidzo zvinoonekwa pamwana wega wega uye kuchengetedza hupenyu hwemwana zvakanakisisa sezvinobvira.

ROHHAD Syndrome uye Kutarisira Kurarama

Nekuti chirwere cheROHHAD chirwere chisingawanzoitiki, zvakaoma kunyatso fungidzira kuti munhu acharamba achirarama kwenguva yakareba sei. Hapana ruzivo rwakakwana pachiri parizvino.

Zvisinei, chikonzero chikuru cherufu nekuda kwechirwere ichi idambudziko rinonzi cardiorespiratory attack, iro riri kumiswa kamwe kamwe kwekushanda kwemoyo nehurongwa hwekufema .

Izvi zvinogona kudzivirirwa here? (Kudzivirira)

Parizvino hapana nzira inozivikanwa yekudzivirira chirwere cheROHHAD - zvirinani kwete parizvino.

Chirwere cheROHHAD chirwere chisingawanzoitiki uye chitsva. Saka tsvakiridzo pamusoro pechirwere ichi ichiri muchikamu chayo chekutanga. Paunofunga nezvehutano hwemwana wako, zuva rega rega rinopera risina mhinduro rinogona kunzwa serisingaperi. Tinozvinzwisisa izvozvo. Saka, iva nechokwadi chekutsvaga rubatsiro kubva kuna chiremba wako. Iye achakwanisa kukupa mazano ekurapa kunodiwa kugadzirisa zviratidzo zvemwana wako, pamwe neruzivo nezvechirwere ichi.

Zvinhu zvakakosha zvikuru zvatinofanira kuyeuka kubva munyaya iyi (Mharidzo Yekuenda Kumba)

Zvakanaka, saka ikozvino wava kunzwisisa nezve ROHHAD Syndrome yatakataura nezvayo. Heano zvimwe zvinhu zvakakosha zvekurangarira:

• Chirwere cheROHHAD chirwere chisingawanzoitiki, asi chakakomba.
• Zviratidzo zvikuru kuwedzera uremu kamwe kamwe, kuoma kufema, uye matambudziko emahormone uye autonomic nervous system.
• Hapana chikonzero chaicho kana mushonga wechigarire weizvi parizvino. Kurapwa kunoenderana nezviratidzo zvinomuka.
• Kana uchifungidzira kuti mwana wako ane zviratidzo izvi, usavhunduka, asi enda kuna chiremba ane hunyanzvi nekukurumidza kuti akupe mazano.
• Nekuti ichi chirwere chisingawanzoitiki, zvinogona kutora nguva kuti chirwere chiongororwe nemazvo. Asi usakanda mapfumo pasi.
• Zvakakosha zvikuru kutevedzera mazano evanachiremba uye kupa mwana rutsigiro rwakakodzera.

Yeuka kuti hausi wega. Zvakakosha kutsvaga rubatsiro kubva kuna vanachiremba, mhuri, uye kana zvichidikanwa, rubatsiro rwekupa mazano paunenge uchitarisana nemamiriro ezvinhu akadai.

` ROHHAD Syndrome, ROHHAD Syndrome, Zvirwere zveVana, Kufutisa Kukuru, Kunetseka Kufema, Hypothalamus, Zvirwere Zvisingawanzoitiki