Kodi mukudziwa za ROHHAD Syndrome? Kodi mwana wanu ali ndi zizindikiro izi?

Kodi mwana wanu wayamba kukula mwadzidzidzi? Kapena mwaona kuti akuvutika kupuma akamagona? Nthawi zina izi zitha kukhala zizindikiro za matenda osowa kwambiri otchedwa ROHHAD Syndrome. Tiyeni tikambirane za izi mwatsatanetsatane, chifukwa ndikofunikira kwambiri kudziwa zinthu ngati izi.

Kodi ROHHAD Syndrome ndi chiyani?

Mwachidule, ROHHAD Syndrome ndi matenda osowa kwambiri komanso oopsa omwe amakhudza ana aang'ono. Amakhudza kwambiri dongosolo la endocrine, dongosolo la mitsempha lodziyimira pawokha, komanso kupuma. Ana omwe ali ndi vutoli amatha kulemera kwambiri pakapita nthawi yochepa kwambiri . Amathanso kusintha momwe amapumira, khalidwe, komanso momwe amamvera akamagona komanso nthawi zina akamadzuka.

Matenda a ROHHAD ndi matenda atsopano. Anayamba kuzindikirika ndi kufotokozedwa mu 1965. Taganizirani izi, pakhala milandu yosakwana 200 ya matendawa padziko lonse lapansi. Ndiye mutha kuganiza momwe zimakhalira zachilendo. Akatswiri azachipatala akufufuzabe vutoli kuti apeze chomwe chimayambitsa matendawa ndikupeza mankhwala ochiritsira. Pakadali pano, palibe mayeso kapena chithandizo chapadera cha matenda a ROHHAD. Chifukwa chake, madokotala amachiritsa mwana aliyense payekhapayekha kutengera zizindikiro zake.

Kodi zizindikiro za matenda a ROHHAD ndi ziti?

Ana omwe ali ndi matenda a ROHHAD nthawi zambiri amakhala athanzi ndipo amakula bwino zizindikiro zisanawonekere. Ngakhale kuti zizindikiro zimatha kuonekera ali ndi zaka 9, ana ambiri amasonyeza zizindikiro zawo zoyambirira ali ndi zaka 2 mpaka 4.

Dzina lakuti 'ROHHAD' limachokera ku zilembo zoyambirira za zizindikiro zinayi zazikulu za matendawa. Tiyeni tiwone zomwe zili:

• (RO) Kunenepa Kwambiri: Apa ndi pamene chilakolako cha mwana chimawonjezeka mwadzidzidzi, zomwe zimapangitsa kuti awonjezere kulemera kwa makilogalamu 20-30 (makilogalamu 9-13) mkati mwa nthawi yochepa, nthawi zambiri pakati pa miyezi itatu ndi khumi ndi iwiri. Ichi nthawi zambiri chimakhala chizindikiro choyamba cha kunenepa kwambiri. Taganizirani izi, sikuti ndi kuwonjezeka thupi kokha, ndi kusintha kwachangu komanso koonekera.

• (H) Kusokonezeka kwa Hypothalamus: Hypothalamus ndi gawo la ubongo wanu lomwe limayang'anira pituitary gland. Imayang'aniranso zinthu zambiri monga kukula kwa thupi, kulemera, chilakolako cha chakudya, kutha msinkhu, malingaliro, ndi khalidwe. Choncho, ngati pali vuto lililonse pakugwira ntchito kwake, mwanayoZizindikiro monga kunenepa kwambiri, kutalika kochepa, khunyu, kuchedwa kukula, kukodza kwambiri kapena kuchepa, komanso kutha msinkhu msanga kapena kuchedwa kungachitike. Kuphatikiza apo, matenda monga hypothyroidism ndi matenda a shuga amathanso kuchitika.

• (H) Kupuma Mofulumira: Ana omwe ali ndi matenda a ROHHAD sangathe kulamulira kupuma kwawo bwino. Izi zikutanthauza kuti mpweya woipa ukachepa m'magazi kapena mpweya woipa ukakwera, thupi silipuma mozama kapena mwachangu mokwanira kuti libwezeretse mpweyawo. Uwu ndi vuto loopsa kwambiri. Nthawi zina, limatha kuchitika mwadzidzidzi, monga matenda ang'onoang'ono opumira, monga chimfine, kapena pambuyo pa mankhwala oletsa kupweteka.

• (AD) Kusokonezeka kwa Magazi Odziyimira Pawokha: Dongosolo lathu la mitsempha lodziyimira pawokha (ANS) limalamulira ntchito zoyambira zomwe sitingathe kuzilamulira, monga kupuma, kugaya chakudya, kugunda kwa mtima, ndi kutentha kwa thupi. Chifukwa chake, ana omwe ali ndi vuto la dongosololi la mitsempha lodziyimira pawokha amatha kukhala ndi zizindikiro monga kugunda kwa mtima pang'onopang'ono (Bradycardia), kutentha thupi kochepa, komanso kuchepa kwa kumva ululu . Tangoganizani, kumva ululu wochepa kumatanthauza kuti ngakhale pangozi yayikulu, mwanayo sangadziwe za izi.

Kodi zifukwa za matenda a ROHHAD ndi ziti?

Ndipotu, madokotala sanapeze chifukwa chenicheni cha matenda a ROHHAD. Komabe, pali mfundo zitatu zazikulu zomwe zikufufuzidwa zomwe zingathandize kumvetsa chifukwa chake.

1. Genetics: Ofufuza ambiri amakhulupirira kuti ROHHAD syndrome imayamba chifukwa cha kusintha kwa majini . Komabe, jini yeniyeniyo sinadziwikebe. Chilichonse m'thupi lathu chimayendetsedwa ndi majini, kotero kusintha kulikonse komwe kungakhudze.

2. Epigenetics: Ofufuza ena amakhulupirira kuti majini omwe amalamulira ntchito zina m'thupi lathu amatha kuyatsidwa pakafunika kutero ndikuzimitsidwa pakafunika kutero. Izi zimatchedwa epigenetics. Lingaliroli likuchokera pa kafukufuku wochitidwa pa mapasa ofanana. Mmenemo, mwana mmodzi adadwala matenda a ROHHAD, pomwe winayo sanatero. Chifukwa chake, tingaganize kuti si majini okha, komanso kusiyana kwa momwe majini amenewo amagwirira ntchito kungakhudzire izi.

3. Kudziteteza ku matenda a autoimmune: Kafukufuku wina akusonyeza kuti matenda a ROHHAD ndi matenda omwe amayambitsanso chitetezo chamthupi.Pakhala pali mgwirizano pakati pa matenda a autoimmune, momwe chitetezo chamthupi chimaukira maselo ake. Mu kafukufuku wina, ana awiri omwe ali ndi ROHHAD syndrome adapezeka kuti ali ndi ma antibodies otchedwa immunoglobulins mu cerebrospinal fluid (CSF) , madzi omwe amazungulira ubongo ndi msana. Pogwiritsa ntchito izi, ofufuzawo adatsimikiza kuti panali kutupa mu central nervous system (CNS) , ubongo ndi msana. Izi zikutanthauza kuti chitetezo chamthupi chimaukira ubongo molakwika.

Kodi mavuto a ROHHAD syndrome ndi ati?

Kuwonjezera pa zizindikiro zazikulu zomwe takambirana kale, ana omwe ali ndi matenda a ROHHAD amatha kukhala ndi mavuto ena, kutanthauza mavuto ena azaumoyo. Ndikofunikira kudziwa izi:

• Mavuto a m'maganizo ndi m'makhalidwe: Ana awa angakumane ndi mavuto monga kusakhazikika, kukwiya, kuchita zinthu mopitirira muyeso, kutopa kosalekeza, nkhawa, kuvutika maganizo, komanso kulephera kuganiza bwino . Izi zingakhudze moyo wa tsiku ndi tsiku wa mwana komanso banja lake.

• Mavuto a mitsempha: Ana omwe ali ndi matenda a ROHHAD amatha kukhala ndi matenda monga khunyu, mayendedwe osazolowereka a maso (nystagmus), kupuma movutikira, kapena matenda a kakulidwe .

• Matenda a kagayidwe kachakudya: Matenda ofala kwambiri a kagayidwe kachakudya pakati pa ana awa ndi matenda a shuga, kukana insulin, kusalolera shuga m'thupi, ndi matenda a chiwindi a Steatotic (mafuta). Izi ndi matenda omwe angayambitse mavuto azaumoyo kwa nthawi yayitali.

• Zotupa za mitsempha: Ana pakati pa 40% ndi 56% omwe ali ndi matenda a ROHHAD amakhala ndi zotupa za mitsempha. Chitsanzo chimodzi ndi mtundu wa chotupa chotchedwa ganglioneuroma . Zotupa zambirizi ndi zabwino , koma nthawi zina zimakhala zoopsa . Chifukwa chake, madokotala nthawi zonse amakhala akuyang'anitsitsa izi.

• Kulephera kupuma bwino: Ichi ndi vuto loopsa kwambiri. Ndi kusiya kupuma mwadzidzidzi komanso kugwira ntchito kwa mtima. Izi zitha kukhala zoopsa kwambiri.

Kodi matenda a ROHHAD amapezeka bwanji? (Kuzindikira)

Pakadali pano, matenda a ROHHAD amatchedwaPalibe mayeso amodzi omwe angatsimikizire matendawa. M'malo mwake, gulu la akatswiri azachipatala limagwirira ntchito limodzi kuti lipeze matenda kutengera kuphatikiza kwapadera kwa zizindikiro za mwanayo. Zili ngati wofufuza milandu, kusonkhanitsa umboni ndikufika pamapeto.

Kuti mwana apezeke ndi matenda a ROHHAD, ayenera kukwaniritsa izi:

• Zizindikiro zonse ziwiri za kuyamba kwadzidzidzi kwa kunenepa kwambiri komanso kupuma pang'ono panthawi yogona ziyenera kukhalapo.
• Zizindikiro za kusagwira bwino ntchito kwa hypothalamus (monga kunenepa kwambiri, hypothyroidism, kapena kusintha kwa msinkhu - msanga kapena mochedwa) ziyenera kukhalapo.
• Ndikofunikira kutsimikizira kuti palibe kusintha kwa majini (gene variant) mu jini ya `PHOX2B`. Izi ndizofunikira kuti tisiyanitse ndi `CCHS (Congenital Central Hypoventilation Syndrome),` vuto lina lomwe lili ndi zizindikiro zina zofanana ndi ROHHAD syndrome (monga kupuma pang'ono pamene mukugona).
• Zizindikiro zimenezi sizingaonekere m'zaka zoyambirira za moyo. Nthawi zambiri zimayamba pambuyo pake.

Popeza matenda a ROHHAD ndi matenda osowa kwambiri, nthawi zambiri ana amatha kudziwitsidwa molakwika poyamba. Chifukwa chake, ngati muli ndi zizindikiro izi, ndikofunikira kwambiri kufunafuna upangiri woyenera kuchokera kwa gulu lachipatala lodziwa bwino ntchito.

Kodi matenda a ROHHAD amachiritsidwa bwanji? (Chithandizo)

Chithandizo cha matenda a ROHHAD chimasiyana malinga ndi mwana, kutengera zizindikiro. Si mwana aliyense amene angapatsidwe njira yofanana yochizira, chifukwa mkhalidwe wa aliyense ndi wosiyana. Komabe, chinthu chimodzi ndi chofanana kwa onse. Ngati mwana yemwe ali ndi matenda a ROHHAD akuwonetsa zovuta zopumira akagona, ayenera kugwiritsa ntchito makina otchedwa `CPAP` kapena `BiPAP` kuti amuthandize kupuma. Pakapita nthawi, angafunike kugwiritsa ntchito `ventilator` kuti athandizire mokwanira njira yopumira. Iyi ndi njira yopulumutsa moyo.

Pochiza ana omwe ali ndi matenda a ROHHAD, thandizo la madokotala a ana m'magawo osiyanasiyana limafunika. Mwachitsanzo:

• Akatswiri a matenda a m'mapapo
• Akatswiri a za ubongo
• Akatswiri a Endocrinologists (madokotala omwe amagwira ntchito yokhudza ma endocrine glands (mahomoni))
• Akatswiri a Matu, Mphuno, ndi Pakhosi (Akatswiri a Makutu, Mphuno, ndi Pakhosi)
• Akatswiri a mtima
• Akatswiri ogona
• Akatswiri a zakudya
• Madokotala a khansa
• Akatswiri a zamaganizo
• Madokotala a zamaganizo

Ndi kudzera mu umodzi wa anthu onsewa kuti timayesetsa kupereka chithandizo chabwino kwambiri kwa mwana.

Kodi tsogolo la ana omwe ali ndi matenda a ROHHAD ndi lotani? (Outlook)

Ndipotu, pakadali pano palibe mankhwala a ROHHAD syndrome. Cholinga chachikulu cha chithandizo ndi kuthana ndi zizindikiro zomwe zimakhala zapadera kwa mwana aliyense ndikusunga moyo wa mwana momwe angathere.

Matenda a ROHHAD ndi Chiyembekezo cha Moyo

Popeza matenda a ROHHAD ndi osowa kwambiri, n'zovuta kuwerengera molondola nthawi yomwe munthu amakhala ndi moyo. Palibe deta yokwanira yokhudza matendawa pakadali pano.

Komabe, chifukwa chachikulu cha imfa chifukwa cha matendawa ndi vuto lotchedwa kulephera kwa mtima ndi kupuma, lomwe ndi kusiya kugwira ntchito kwa mtima ndi dongosolo lopumira mwadzidzidzi .

Kodi izi zingapewedwe? (Kupewa)

Pakadali pano palibe njira yodziwika yopewera matenda a ROHHAD - osachepera pano.

Matenda a ROHHAD ndi matenda osowa komanso atsopano. Choncho kafukufuku wa matendawa akadali pachiyambi. Mukaganizira za thanzi la mwana wanu, tsiku lililonse lomwe limapita popanda mayankho likhoza kumveka ngati losatha. Timamvetsa zimenezo. Choncho, onetsetsani kuti mwapempha thandizo kwa dokotala wanu. Adzatha kukupatsani malangizo pa mankhwala ofunikira kuti muchepetse zizindikiro za mwana wanu, komanso magwero a chidziwitso chokhudza matendawa.

Zinthu zofunika kwambiri zomwe tiyenera kuzitenga kuchokera mu nkhaniyi (Uthenga Wopita Nawo Kunyumba)

Chabwino, tsopano mwamvetsa pang'ono za ROHHAD Syndrome yomwe tidakambirana. Nazi zinthu zofunika kukumbukira:

• Matenda a ROHHAD ndi matenda osowa kwambiri, koma oopsa.
• Zizindikiro zazikulu ndi kunenepa mwadzidzidzi, kuvutika kupuma, komanso mavuto a mahomoni ndi dongosolo lamanjenje lodziyimira palokha.
• Palibe chifukwa chenicheni kapena mankhwala okhazikika a izi pakadali pano. Chithandizo chimadalira zizindikiro zomwe zimabwera.
• Ngati mukukayikira kuti mwana wanu ali ndi zizindikiro izi, musachite mantha, koma pitani kwa dokotala wodziwa bwino ntchito nthawi yomweyo kuti akupatseni upangiri.
• Popeza matenda amenewa ndi osowa, zingatenge nthawi kuti mudziwe matenda molondola. Koma musataye mtima.
• Ndikofunikira kwambiri kutsatira malangizo a madokotala ndikupatsa mwana chithandizo chofunikira.

Kumbukirani kuti simuli nokha. Ndikofunikira kupempha thandizo kwa madokotala, achibale, ndipo, ngati kuli kofunikira, upangiri mukakumana ndi mavuto ngati awa.

Matenda a ROHHAD , Matenda a ROHHAD, Matenda a Ana, Kunenepa Mwadzidzidzi, Kuvutika Kupuma, Hypothalamus, Matenda Osowa