So wunim ROHHAD Syndrome ho asɛm? So wo ba no wɔ saa sɛnkyerɛnne ahorow yi?

So wo ba ketewa no afi ase reyɛ kɛse ankasa mpofirim? Anaasɛ woahyɛ no nsow sɛ ɛyɛ den ma wɔn sɛ wɔbɛhome bere a wɔada no? Ɛtɔ mmere bi a eyinom betumi ayɛ tebea bi a ɛntaa nsi koraa a wɔfrɛ no ROHHAD Syndrome ho sɛnkyerɛnne. Momma yɛnka eyi ho asɛm nkɔ akyiri kakra, efisɛ ɛho hia yiye sɛ yɛma yɛn ani da hɔ wɔ nneɛma a ɛte sɛɛ ho.

Dɛn ne ROHHAD Syndrome?

Sɛ yɛbɛka no tiawa a, ROHHAD Syndrome yɛ tebea a ɛntaa nsi na ɛde nkwa to asiane mu a ɛka mmofra nkumaa. Ɛka endocrine system, autonomic nervous system, ne ɔhome titiriw. Mmofra a wɔwɔ saa tebea yi betumi ayɛ kɛse wɔ bere tiaa bi mu . Wobetumi nso ahu nsakrae wɔ ɔhome, nneyɛe, ne nkate mu nhyehyɛe mu bere a wɔada ne ɛtɔ mmere bi a wɔda.

ROHHAD syndrome yɛ aduruyɛ mu tebea foforo koraa. Wodii kan huu no na wɔkaa ho asɛm wɔ 1965. Sɛ yesusuw ho a, nnipa a wɔanya saa tebea yi a wɔabɔ ho amanneɛ wɔ wiase nyinaa no nnu 200. Enti wubetumi asusuw sɛnea ɛho yɛ na no ho. Aduruyɛ ho abenfo da so ara reyɛ tebea no mu nhwehwɛmu de ahu nea ɛde ba ankasa na wɔanya aduru a ɛbɛtra hɔ daa. Mprempren, wonni nhwehwɛmu pɔtee biara anaa ayaresa biara a wɔde bedi dwuma wɔ ROHHAD yare no ho. Enti, nnuruyɛfo gyina ne yare no ho sɛnkyerɛnne so sa abofra biara yare mmiako mmiako.

Dɛn ne ROHHAD yare no ho sɛnkyerɛnne?

Mmofra a wɔwɔ ROHHAD yare no taa nya apɔwmuden na wonyin sɛnea ɛsɛ ansa na sɛnkyerɛnne ahorow no ada adi. Ɛwom sɛ yare no ho sɛnkyerɛnne betumi ada adi ntɛm ara wɔ mfe 9 mu de, nanso mmofra dodow no ara da wɔn sɛnkyerɛnne a edi kan adi wɔ mfe 2 kosi 4 ntam.

Wɔnyaa edin ‘ROHHAD’ no firii nkyerɛwdeɛ a ɛdi kan wɔ saa yareɛ yi ho nsɛnkyerɛnneɛ titire anan no mu. Momma yɛnhwɛ nea wɔyɛ:

• (RO) Kɛse mmoroso a efi ase ntɛmntɛm: Eyi ne bere a abofra akɔnnɔ kɔ soro kɛse mpofirim , na ɛma ne mu duru yɛ kɛse nkaribo 20-30 (kilogram 9-13) wɔ bere tiaa bi mu, mpɛn pii no wɔ asram abiɛsa kosi dumien ntam. Eyi taa yɛ kɛseyɛ mmoroso ho sɛnkyerɛnne a edi kan. Susuw ho hwɛ, ɛnyɛ mu duru a ɛkɔ soro ara kwa, ɛyɛ nsakrae a ɛba ntɛmntɛm yiye, a wotumi hu.

• (H) Hypothalamic Dysregulation: Hypothalamus yɛ w’amemene no fã bi a ɛhwɛ pituitary gland no so. Ɛsan nso hwɛ nneɛma pii te sɛ nipadua nyin, mu duru, aduan ho akɔnnɔ, mmabunbere, nkate, ne nneyɛe so. Enti, sɛ ɔhaw bi wɔ ne dwumadi mu a, abofra noNsɛnkyerɛnne te sɛ kɛseyɛ mmoroso, ne tenten tiaa, akisikuru, nyin a ɛkyɛ, nsu a ɛkɔ soro anaasɛ ɛso tew, ne mmerantebere ne mmabaabere a ɛba ntɛm anaasɛ ɛkyɛ betumi aba. Nea ɛka ho no, tebea horow te sɛ hypothyroidism ne asikreyare nso betumi aba.

• (H) Hypoventilation: Mmofra a wɔwɔ ROHHAD syndrome ntumi nni wɔn home so yiye. Eyi kyerɛ sɛ, sɛ oxygen dodow a ɛwɔ mogya no mu so tew anaasɛ carbon dioxide dodow kɔ soro a, nipadua no nhome nkɔ akyiri anaasɛ ɛnhome ntɛmntɛm sɛnea ɛbɛyɛ a ebetumi atua ho ka. Eyi yɛ tebea a ɛyɛ hu paa. Ɛtɔ da bi a, ebetumi aba mpofirim, te sɛ bere a obi anya ɔhome mu yare ketewaa bi, te sɛ atiridiinini, anaasɛ bere a wɔde aduru a ɛma obi dwensɔ akyi.

• (AD) Autonomic Dysregulation: Yɛn nipadua no autonomic nervous system (ANS) no na ɛhwɛ dwumadi atitiriw a yentumi nni so, te sɛ ahome, aduan a wɔyam, koma a ɛbɔ, ne nipadua mu hyew so. Enti, mmofra a ntini a ɛma ɛyɛ adwuma yi asɛe no betumi anya sɛnkyerɛnne ahorow te sɛ koma a ɛbɔ brɛoo wɔ ɔkwan a ɛnteɛ so (Bradycardia), nipadua mu hyew a ɛba fam, ne ɛyaw a wɔte nka a ɛso tew . Fa no sɛ, sɛ abofra no nte ɛyaw kakraa bi a, ɛkyerɛ sɛ wɔ akwanhyia a emu yɛ den mpo mu no, ebia abofra no renhu.

Dɛn ne nneɛma a ɛde ROHHAD yare no ba?

Nokwarem no, nnuruyɛfo nnya nhuu nea ɛde ROHHAD yare no ba ankasa. Nanso, nsusuwii atitiriw abiɛsa na wɔreyɛ ho nhwehwɛmu mprempren a ebetumi akyerɛkyerɛ mu.

1. Awosu mu nneɛma: Nhwehwɛmufo pii gye di sɛ awosu mu nsakrae bi na ɛde ROHHAD yare no ba . Nanso, wonnya nhuu awosu mu abɔde pɔtee no. Awosu mu nneɛma na ɛhwɛ biribiara a ɛwɔ yɛn nipadua mu so, enti nsakrae biara a ɛbɛba mu no betumi aka no.

2. Epigenetics: Nhwehwɛmufo binom gye di sɛ wobetumi asɔ awosu mu nkwaadɔm a ɛhwɛ dwumadi ahorow bi so wɔ yɛn nipadua mu bere a ɛho hia na wɔadum bere a ɛho nhia. Wɔfrɛ eyi sɛ epigenetics. Saa adwene yi gyina nhwehwɛmu bi a wɔyɛe wɔ mmanoma a wɔyɛ pɛ ho so. Wɔ mu no, abofra biako nyaa ROHHAD yare, bere a ɔfoforo no annya bi. Enti, wobetumi asusuw sɛ ɛnyɛ awosu mu nkwaadɔm nko, na mmom nsonsonoe a ɛwɔ ɔkwan a saa awosu mu nkwaadɔm no fa so yɛ adwuma mu nso betumi aka eyi.

3. Autoimmunity: Nhwehwɛmu bi kyerɛ sɛ ROHHAD syndrome nso yɛ yare a nipadua no tumi ko tia nyarewa.Abusuabɔ bi aba nyarewa a nipadua no ankasa tumi ko tia nyarewa, a nipadua no ankasa nipadua no mu tumi a ɛko tia nyarewa no tow hyɛ n’ankasa nkwammoaa so no ntam. Wɔ nhwehwɛmu bi mu no, wohui sɛ mmofra baanu a wɔwɔ ROHHAD yare no wɔ nnuru a ɛko tia nyarewa a wɔfrɛ no immunoglobulins wɔ amemene mu nsu (CSF) , nsu a atwa amemene ne akyi berɛmo ho ahyia no mu. Nhwehwɛmufo no de saa nsɛm yi dii dwuma de baa awiei sɛ ɔfe wɔ ntini a ɛwɔ nipadua no mu (CNS) , amemene ne akyi berɛmo no mu. Na eyi kyerɛ sɛ nipadua no ankasa nipadua mu tumi a ɛko tia nyarewa no di mfomso reto ahyɛ amemene no so.

Dɛn ne nsɛnnennen a ɛwɔ ROHHAD yare no mu?

Wɔ sɛnkyerɛnne atitiriw a yɛadi kan aka ho asɛm no akyi no, mmofra a wɔwɔ ROHHAD yare no betumi anya nsɛnnennen afoforo , kyerɛ sɛ, akwahosan ho haw afoforo. Ɛho hia sɛ wuhu eyinom nso:

• Adwene ne nneyɛe mu haw ahorow: Mmofra yi betumi anya tebea horow te sɛ ahomegye a wonni, basabasayɛ, nneɛma a wɔyɛ boro so, ɔbrɛ a ɛba bere nyinaa, dadwen, adwenemhaw, ne adwene mu dɛmdi . Eyinom betumi aka abofra no da biara da asetra ne abusua no nso.

• Ntini mu haw ahorow: Mmofra a wɔwɔ ROHHAD yare no betumi anya tebea horow te sɛ akisikuru, aniwa a ɛkeka ne ho wɔ ɔkwan a ɛnteɛ so (nystagmus), nna mu ahomegye, anaa nkɔso mu haw ahorow .

• Nkwammoaa mu yare: Nkwammoaa mu yare a ɛtaa ba mmofra yi mu ne Asikreyare, Insulin a wɔko tia, Glucose a wontumi nnye ntom, ne Steatotic (srade) mmerɛbo mu yare. Eyinom yɛ tebea horow a ebetumi de akwahosan ho nsunsuanso a ɛtra hɔ kyɛ aba.

• Ntini mu akisikuru: Mmofra a wɔwɔ ROHHAD yare no mu 40% kosi 56% nya ntini mu akisikuru. Nhwɛso biako ne akisikuru bi a wɔfrɛ no ganglioneuroma . Saa akisikuru yi mu dodow no ara nyɛ nea asiane biara nni ho , nanso ɛtɔ mmere bi a ebetumi ayɛ nea ɛyɛ hu . Enti, nnuruyɛfo hwɛ eyi yiye bere nyinaa.

• Koma ne ɔhome a ɛyɛ den: Eyi ne ɔhaw a asiane wom sen biara. Ɛyɛ ahome a wogyae mpofirim na koma no yɛ adwuma. Eyi betumi de nkwa ato asiane mu.

Ɔkwan bɛn so na wohu ROHHAD yare no? (Nyare a Wɔahu) .

Mprempren, wɔfrɛ no ROHHAD syndromeSɔhwɛ biako biara nni hɔ a ebetumi asi nea wɔahu no so dua koraa. Mmom no, aduruyɛ ho abenfo kuw bi bom yɛ adwuma de gyina abofra no yare no ho sɛnkyerɛnne soronko a wɔaka abom so hu yare no . Ɛte sɛ detective, a ɔreboaboa adanse ano na ɔreba awiei.

Sɛ abofra betumi ahu sɛ ɔwɔ ROHHAD yare no a, ɛsɛ sɛ odu nneɛma a edidi so yi ho:

• Ɛsɛ sɛ sɛnkyerɛnne abien no nyinaa a ɛkyerɛ sɛ obi ayɛ kɛse mmoroso mpofirim ne mframa a ɛmma obi ntumi nnya bere a wada no nyinaa wɔ hɔ.
• Ɛsɛ sɛ hypothalamic a ɛnyɛ adwuma yiye ho sɛnkyerɛnne (e.g., mu duru a ɛkɔ soro ntɛmntɛm, hypothyroidism, anaa nsakrae a ɛba wɔ mmerantebere ne mmabaabere mu - ntɛm anaa akyiri) no wɔ hɔ.
• Ɛho hia sɛ yɛsi so dua sɛ mutation (gene variant) biara nni gene `PHOX2B` mu. Eyi ho hia na ama yɛahu nsonsonoe a ɛda `CCHS (Congenital Central Hypoventilation Syndrome),` tebea foforo a ɛwɔ sɛnkyerɛnne ahorow bi a ɛte sɛ ROHHAD syndrome (e.g., ahome a ɛso tew bere a ɔda).
• Ebia saa sɛnkyerɛnne ahorow yi renna adi wɔ nkwa mfe kakraa a edi kan no mu. Wɔtaa fi ase akyiri yi.

Esiane sɛ ROHHAD yare no yɛ tebea a ɛntaa nsi nti, mpɛn pii no wobetumi ahu mmofra wɔ ɔkwan a ɛnteɛ so mfiase no. Enti, sɛ wowɔ saa sɛnkyerɛnne ahorow yi a, ɛho hia yiye sɛ wohwehwɛ afotu a ɛfata fi nnuruyɛfo kuw a wɔn ho akokwaw hɔ.

Ɔkwan bɛn so na wɔsa ROHHAD yare no? (Ayaresa)

Ɛsono sɛnea wɔsa ROHHAD yare no wɔ abofra biara mu, na egyina sɛnkyerɛnne ahorow no so. Ɛnyɛ abofra biara na wobetumi ama no ayaresa nhyehyɛe koro, efisɛ ɛsono obiara tebea. Nanso, ade biako na ɛwɔ hɔ ma obiara. Sɛ abofra a ɔwɔ ROHHAD yareɛ no da ahomegyeɛ a ɛntene adi berɛ a ɔda a, ɛbɛhia sɛ wɔde afiri bi a wɔfrɛ no `CPAP` anaa `BiPAP` di dwuma de boa no ma wɔhome. Bere kɔ so no, ebia ɛho behia sɛ wɔde `ahomegye afiri` di dwuma de boa ɔhome nhyehyɛe no koraa. Eyi yɛ adeyɛ a egye nkwa.

Sɛ wɔresa mmofra a wɔwɔ ROHHAD yare no yare a, mmofra nnuruyɛfo a wɔwɔ nnwuma ahorow mu mmoa ho hia. Nhwɛsoɔ:

• Ahurututu ho animdefo
• Ntini mu yare ho animdefo
• Endocrinologists (nnuruyɛfo a wɔn ho akokwaw wɔ endocrine glands (hormones) mu) .
• Otolaryngologists (Aso, Hwene, Menewa Ho Nnuruyɛfo) .
• Koma ho aduruyɛfo
• Dae ho abenfo
• Aduandi ho abenfo
• Oncologists ho animdefo
• Adwene ne nneyɛe ho animdefo
• Adwene ne nneyɛe ho aduruyɛfo

Ɛnam saa nnipa yi nyinaa biakoyɛ so na yɛbɔ mmɔden sɛ yɛbɛma abofra no ayaresa a eye sen biara.

Dɛn ne mmofra a wɔwɔ ROHHAD yare no daakye? (Nsusuwii) .

Nokwarem no, mprempren wonni aduru biara a wɔde sa ROHHAD yare no. Botae titiriw a ɛwɔ ayaresa mu ne sɛ wobedi sɛnkyerɛnne ahorow a ɛyɛ soronko wɔ abofra biara ho no ho dwuma na wɔakura abofra no asetra mu yiye sɛnea ɛbɛyɛ yiye biara.

ROHHAD Syndrome ne Nkwa Ntraso

Esiane sɛ ROHHAD yare no yɛ tebea a ɛntaa nsi saa nti, ɛyɛ den sɛ wobebu nkwa nna ho akontaa pɛpɛɛpɛ. Data a ɛdɔɔso nni hɔ a ɛfa ho de besi nnɛ.

Nanso, ade titiriw a ɛde owu a yare yi ba ne ɔhaw bi a wɔfrɛ no koma ne ɔhome a ɛyɛ den, a ɛyɛ koma ne ɔhome nhyehyɛe a egyae adwumayɛ mpofirim .

So wobetumi asiw eyi ano? (Ayaresa a wɔde siw ano) .

Mprempren ɔkwan biara nni hɔ a wɔbɛfa so asiw ROHHAD yare no ano - anyɛ yiye koraa no, wonnya nyɛɛ saa.

ROHHAD syndrome yɛ tebea a ɛntaa nsi na ɛyɛ foforo koraa. Enti eyi ho nhwehwɛmu da so ara wɔ ne mfiase. Sɛ wususuw wo ba no akwahosan ho a, da biara a ɛbɛtwam a mmuae biara nni ho no betumi ayɛ te sɛ nea ɛbɛtra hɔ daa. Yɛte saa asɛm no ase. Enti, hwɛ hu sɛ wobɛhwehwɛ mmoa afi wo duruyɛfo hɔ. Ɔbɛbɔ wo amanneɛ wɔ ayaresa a ɛhia na wode adi wo ba no yareɛ no ho, ne mmeaeɛ a wobɛtumi asua pii afa saa tebea yi ho.

Nneɛma a ɛho hia paa a ɛsɛ sɛ yɛde kɔ fie fi saa asɛm yi mu (Take-Home Message) .

Okay, enti seesei woanya nteaseɛ bi wɔ ROHHAD Syndrome a yɛkaa ho asɛm no ho. Nneɛma a ɛho hia a ɛsɛ sɛ wokae ni:

• ROHHAD syndrome yɛ tebea a ɛntaa nsi koraa, nanso emu yɛ den.
• Nsɛnkyerɛnne titiriw ne mu duru a ɛkɔ soro mpofirim, ɛyɛ den sɛ ɔbɛhome, ne ɔhaw ahorow a ɛba wɔ hormone ne autonomic nervous system no ho.
• Biribiara nni hɔ pɔtee biara a ɛde eyi ba anaasɛ aduru a ɛbɛtra hɔ daa ama eyi de besi nnɛ. Ayaresa no gyina sɛnkyerɛnne ahorow a ɛsɔre so.
• Sɛ wususuw sɛ wo ba no wɔ saa sɛnkyerɛnne ahorow yi a, mma wo ho nnyɛ wo hu, na mmom kɔ oduruyɛfo a ɔfata nkyɛn ntɛm ara kɔhwehwɛ afotu.
• Esiane sɛ eyi yɛ yare a ɛntaa nsi nti, ebia ebegye bere kakra ansa na wɔahu yare no pɛpɛɛpɛ. Nanso mma w’abaw.
• Ɛho hia paa sɛ wudi nnuruyɛfo afotu akyi na wode mmoa a ɛho hia ma abofra no.

Kae sɛ ɛnyɛ wo nko ara. Ɛho hia sɛ wohwehwɛ mmoa fi nnuruyɛfo, abusua, ne sɛ ɛho hia a, afotu adwumayɛbea ahorow hɔ bere a woredi tebea horow a ɛte sɛɛ ho dwuma no.

` ROHHAD Syndrome, ROHHAD Syndrome, Mmofra Nyarewa, Kɛse mmoroso mpofirim, Ɔhome a ɛyɛ den, Hypothalamus, Nyarewa a ɛntaa nsi