Dɛn ne mogya a ɛtɔ kɔ nipadua mu ne asaabo mu haw ahorow a ɛyɛ nwonwa yi? (Atypical Hemolytic Uremic Syndrome - aHUS) Momma yɛnkasa!

So wubetumi asusuw nea ɛbɛba sɛ yɛn ankasa nipadua a ɛko tia nyarewa, sɛ́ anka ɛbɛbɔ yɛn ho ban afi nyarewa ho no, fi ase tow hyɛɛ yɛn ankasa nipadua so a? Ɛno yɛ tebea biako a ɛyɛ awerɛhow, nanso ɛntaa nsi kakra, a yɛbɛka ho asɛm nnɛ ne aHUS, anaa Atypical Hemolytic Uremic Syndrome . Eyi ne bere a yɛn mogya ntini no sɛe, mogya mu ntini nketenkete sisi, na mogya a ɛkɔ akwaa a ɛho hia te sɛ asaabo mu no so tew. Mpɛn pii no awosu mu nsakrae bi na ɛde tebea yi ba .

Nnuruyɛfo taa hu sɛ wowɔ aHUS bere a woaka tebea abiɛsa yi nyinaa abom no:

• Microangiopathic hemolytic anemia : Eyi kyerɛ sɛ wo mogya mu nkwammoaa kɔkɔɔ no sɛe ntɛmntɛm dodo, enti wo nipadua no ntumi nyɛ nkwammoaa foforo mfa nsi ananmu.
• Thrombocytopenia : Eyi yɛ mogya nkwammoaa dodow a ɛso tew, na ɛboa ma mogya tu.
• Acute kidney injury : Eyi yɛ asaabo a entumi nyɛ adwuma yiye, nanso ɛtɔ mmere bi a wobetumi asan.

Saa tebea abiɛsa yi nyinaa betumi aba bere koro mu, anaasɛ nyarewa afoforo betumi ama ayɛ kɛse.

Nsonsonoe bɛn na ɛda HUS a wɔyɛ no daa ne aHUS yi ntam?

Bere bi a atwam no, na wɔakyekyɛ tebea a wɔfrɛ no HUS (Hemolytic Uremic Syndrome) yi mu ayɛ no ahorow atitiriw abien.

• HUS a ɛtaa ba (HUS a ɛne atiridiinini wɔ abusuabɔ): Mpɛn pii no, akisikuru ka eyi ho. Mmoawa bi a wɔfrɛ no E. coli na ɛde ba. Nnipa binom nso frɛ no STEC-HUS .
• aHUS a yɛreka ho asɛm nnɛ (Atypical HUS): Eyi nyɛ atiridiinini a wohu. Mpɛn pii no, nnipa a wonya aHUS no mu bɛyɛ fã, awosu mu nsakrae, awosu mu nsakrae na ɛde ba . Ɛtɔ mmere bi a nnuruyɛfo frɛ tebea yi sɛ complement-mediated thrombotic microangiopathy (CM-TMA) .

Dɛn ne nneɛma pɔtee a ɛkanyan aHUS?

Sɛ yɛbɛka no tiawa a, ɛnyɛ obiara a ɔwɔ awosu mu nsakrae na obenya aHUS. Mpɛn pii no, akwahosan tebea afoforo na ɛkanyan no, anaasɛ ɛma ɛyɛ kɛse . Susuw nneɛma yi ho hwɛ:

• Nyinsɛn bere
• Kokoram
• Nyarewa ahorow ahorow
• Nnuru bi, titiriw nnuru a wɔde sa yare , nnuru a ɛma nipadua no tumi ko tia nyarewa , nnuru a ɛma mogya yɛ mmerɛw , nnuru a wɔde siw nyinsɛn ano , ne nnuru bi a ekum ɔyare mmoawa .

Dɛn ne aHUS ho sɛnkyerɛnne ahorow?

Afei ma yɛnhwɛ sɛnkyerɛnne ahorow a obi a ɔwɔ aHUS da no adi. Ebia wowɔ eyinom pii, anaasɛ ebia wubenya yare no ho sɛnkyerɛnne ahorow a ɛbɛda adi nkakrankakra.

• Ɔbrɛ a wɔte nka bere nyinaa (Ɔbrɛ) .
• Wora a ɛyɛ mmerɛw (Pallor) .
• Akisikuru anaa ɔfe
• Nsu a ɛba no so tew
• Mogya a ɛwɔ nsu mu
• Mogya mmoroso (Hypertension) .
• Ɔhome a ɛyɛ den (Dyspnea) .
• Edema a ɛma obi ho yɛ hyew
• Confusion , nkae a wɔhwere
• Huraeɛ

Nnipa dodow no ara nnya saa sɛnkyerɛnne ahorow yi nyinaa prɛko pɛ. Ɛtɔ mmere bi a wɔba nkakrankakra, nkakrankakra. Ebia wobɛte nka sɛ woahu amane wɔ biribi ho bere tiaa bi, nanso wuntumi nhu nea ɛyɛ. Saa ntini mu sɛnkyerɛnne yi, ɛno ne adwene a ɛyɛ basaa a madi kan aka ho asɛm no, ɛnto obiara, ɛnyɛ nea ɛtaa ba kakra.

Dɛn na ɛde aHUS ba ankasa?

Nea ɛde aHUS ba titiriw ne nsakrae a ɛba yɛn DNA mu (awosu mu nsakrae) , anaasɛ autoantibodies a ɛtow hyɛ yɛn ankasa nkwammoaa so tia ‘protein ahorow a ɛboa’ a ɛwɔ yɛn nipadua mu nkwammoaa a ɛko tia nyarewa mu no . Yebetumi anya awosu mu nsakrae yi afi yɛn awofo hɔ, anaasɛ ebetumi aba kwa. Saa awosu mu nsakrae yi ka protein ahorow a wɔfrɛ no ‘complement factors’ dwumadi .

Susuw ‘nneɛma a ɛboa’ yi ho sɛ asraafo nkumaa a wɔboa yɛn nkwammoaa a ɛko tia nyarewa no. Te sɛ aduan a wɔde yɛ aduan a ɛyɛ dɛ no, saa protein ahorow yi hu mmoawa a epira (te sɛ mmoawa ne mmoawa) na wɔhyɛ wɔn agyirae sɛnea ɛbɛyɛ a nkwammoaa afoforo a ɛko tia nyarewa betumi ‘adi’. Nea ɛtaa ka aHUS ne complement factors H, I, 3, ne B.

Sɛ nhwɛso no, adwuma a complement factor H yɛ ne sɛ ɛbɛbɔ nkwammoaa a ɛwɔ apɔwmuden wɔ yɛn nipadua mu ho ban na ama complement factor afoforo anyɛ adwuma bere a ɛho nhia no. Afei susuw nea ɛbɛba sɛ saa complement factor H yi anyɛ adwuma yiye (kyerɛ sɛ, sɛ nkwaadɔm mu nsakrae bi wɔ CFH awosu mu abɔde mu a) ? Yɛn nkwammoaa no nnya ahobammɔ a ehia no. Afei nneɛma a ɛkɔ so ni:

• Yɛn ankasa nipadua mu nkwammoaa a ɛko tia nyarewa no di mfomso tow hyɛ nkwammoaa a ɛwɔ yɛn mogya ntini mu (endothelial cells) no so na ɛsɛe .
• Ntini mu nkwammoaa boaboa ano twa saa ɔsɛe yi ho hyia na ɛyɛ mogya a ɛtɔ. Eyi siw mogya ntini no kwan.
• Eyi ma mogya a ɛkɔ yɛn akwaa ahorow mu no so tew, na ɛma ɛsɛe na wɔn dwumadi so tew. Asaabo na ɛka no kɛse.
• Saa mogya a ɛtwetwe yi sɛe mogya nkwammoaa afoforo a ɛfa mu no, na ɛde tebea bi a wɔfrɛ no hemolytic anemia ba .

Wobɛyɛ dɛn ahu sɛ wowɔ aHUS?

Mpɛn pii no, oduruyɛfo begyina wo sɛnkyerɛnne ahorow ne nea efi nhwehwɛmu ahorow a ɛhwɛ wo mogya dodow ne sɛnea w’akwaa ahorow reyɛ adwuma mu aba so ahu aHUS. Wɔbɛyɛ nhwehwɛmu nso de ahwɛ sɛ wunni nyarewa afoforo a ɛde sɛnkyerɛnne a ɛte saa ara ba (te sɛ STEC-HUS a yɛadi kan aka ho asɛm no).

Nhwehwɛmu bɛn na wɔde hu aHUS?

Ebia ɛho behia sɛ woyɛ sɔhwɛ ahorow te sɛ eyinom:

• Mogya mu nhwehwɛmu: Mogya dodow a edi mũ (CBC) , asaabo dwumadi mu nhwehwɛmu, ne nea ɛkeka ho.
• Awosu mu nhwehwɛmu : Eyi hwɛ sɛ awosu mu nsakrae bi wɔ hɔ anaa.
• Nsu mu nhwehwɛmu : Hwɛ sɛ nyarewa te sɛ E. coli wɔ hɔ anaa.
• Kidney biopsy : Asaabo no fã ketewaa bi a wɔfa de yɛ nhwehwɛmu.
• Mfoninitwa nhwehwɛmu : te sɛ ultrasound anaa CT scan .

Ɔkwan bɛn so na wɔsa aHUS?

AHUS ayaresa gyina nea ɛde ba ne sɛnea tebea no mu yɛ den so. Sɛ wowɔ aHUS a ɛnyɛ den a, ebia wo duruyɛfo bɛhwɛ wo na wama wo ɔhwɛ a ɛboa , te sɛ mogya a wɔde ma anaa mogya mmoroso nnuru, sɛ ɛho hia a.

Nanso, sɛ wowɔ aHUS a emu yɛ den a, ebia wubehia ayaresa te sɛ eyi:

• Plasma sesa a wɔde sa yare : Wɔ eyi mu no, woyi mogya no fã a ɛwɔ plasma mu no na wɔma mogya foforo.
• Rituximab anaa nnuru afoforo a wɔde sa nyarewa a nipadua no tumi ko tia nyarewa.
• Dialysis : Ɔkwan a wɔfa so tew mogya no ho bere a asaabo no ntumi nyɛ adwuma yiye.
• Asaabo a wɔde si ananmu : Wɔyɛ eyi wɔ tebea horow a emu yɛ den yiye mu nkutoo.

Wɔ wɔn a wɔwɔ ‘complement’ gene mutations a yɛadi kan aka ho asɛm no fam no, nnuruyɛfo taa de aduru bi a wɔfrɛ no eculizumab di dwuma de sa aHUS. Eculizumab yɛ adwuma denam protein ahorow bi a ɛboa no a esiw ano so, na esiw wo nipadua mu ahoɔden a ɛko tia nyarewa no kwan sɛ ɛnsɛe w’ankasa wo mogya ntini.

So ayaresa no mu nsunsuanso bɔne bi wɔ hɔ?

Sɛ wode Eculizumab redi dwuma a, ebia worenbɔ wo ho ban kakra mfi meningococcal ne pneumococcal vaccines ho. Enti, sɛ ɛbɛyɛ yiye a, wo duruyɛfo betumi atu wo fo sɛ fa saa nnuru yi ansa na woafi Eculizumab ase.

Dɛn na daakye bɛyɛ ma obi a ɔwɔ aHUS?

Sɛ wohu saa tebea yi na wɔsa no ntɛm a, wobetumi adi tebea horow te sɛ asaabo mu opira a emu yɛ den a aHUS de ba no ho dwuma yiye. Ebia wubenya bere bi a wo ho bɛtɔ wo a wunnya yare no ho sɛnkyerɛnne biara. Nanso, sɛ biribi foforo kanyan aHUS bio a, ebetumi abɛyɛ nea ɛde nkwa to asiane mu bio.

Bere bi a atwam no, nnipa a wɔwɔ aHUS no mu bɛyɛ fã, anaa 50% nyaa asaabo mu yare a ɛba awiei (ESKD) , a ɛkyerɛ sɛ wɔn asaabo no yɛɛ nea entumi nyɛ adwuma koraa.

Nanso asɛmpa bi wɔ hɔ! Nhwehwɛmu foforo kyerɛ pefee sɛ asiane a ɛwɔ hɔ sɛ wobenya ESKD no so atew kɛse wɔ nnipa a wɔwɔ aHUS mu esiane aduru a wɔde sa Eculizumab nti .

Asiane bɛn na ɛwɔ owu mu esiane aHUS nti?

AHUS ho nsɛm a emu yɛ den te sɛ asaabo a entumi nyɛ adwuma yiye anaasɛ akwaa afoforo a ɛsɛe no yɛ aniberesɛm ankasa. Wɔ nsɛm a emu yɛ den saa mu no, asiane a ɛwɔ hɔ sɛ obi bewu bɛyɛ 15%.

So wobetumi asiw aHUS ano?

Esiane sɛ mpɛn pii no awosu mu nsakrae na ɛde ba nti, mpɛn pii no wontumi nsiw aHUS ano. Nanso, sɛ asiane wɔ hɔ sɛ wubenya yare a emu yɛ den a, ebia wubetumi akwati nneɛma a ɛkanyan no no bi .

Mɛyɛ dɛn ahwɛ me ho?

Sɛ wɔahu sɛ wowɔ aHUS a, wo ne wo duruyɛfo nkasa mfa akwan a wobɛfa so akwati nneɛma a ebetumi ama tebea no asɛe kɛse ho. Ɛho hia nso sɛ wuhu sɛnkyerɛnne ahorow a ɛkyerɛ sɛ obi anya ɔyare a emu yɛ den.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ woate nka sɛ wo ho ayɛ wo den anaasɛ woabrɛ akyɛ – te sɛ nea wowɔ yare a enni sabea a ɛrenkɔ a – wo ne wo duruyɛfo nkasa. Afei nso, ka sɛnkyerɛnne foforo biara a woanya nnansa yi, te sɛ nsakrae a ɛba wo tiafi mu ho asɛm.

Bere bɛn na ɛsɛ sɛ mekɔ Emergency Treatment Unit (ETU) ?

Sɛ wowɔ saa sɛnkyerɛnne ahorow a emu yɛ den yi a, kɔ baabi a wɔhwɛ wɔn a wɔwɔ yare a egye ntɛmpɛ ntɛm ara:

• Nsu a ɛba no so tew kɛse.
• Adwene a ɛyɛ basaa anaasɛ adwene a ɛsakra.
• Ɔhonhon a emu yɛ den.
• Ɔhome a ɛyɛ den kɛse.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

Ebia ɛbɛyɛ nea mfaso wɔ so sɛ wubebisa wo duruyɛfo nsɛm te sɛ eyinom:

• Me tebea no mu yɛ den dɛn?
• Dɛn na ɛmaa eyi mu yɛɛ den?
• So metumi asiw tebea horow a ɛte sɛɛ ano daakye?
• Nsɛnkyerɛnne bɛn na ɛsɛ sɛ mehwɛ yiye?
• Bere bɛn na ɛsɛ sɛ meba bɛhwɛ wo bio?

Wɔfrɛ tebea yi sɛ aHUS abu so dɛn?

aHUS ho yɛ na yiye.Ayaresa tebea bi. Ɛka nnipa bɛyɛ mpem ahanu biara mu biako. Ɛtaa ba mpanyimfo mu kakra sen mmofra.

Nea etwa to no, nneɛma a ɛsɛ sɛ yɛkae

Sɛ wuhu sɛ wo anaa wo ba wɔ awosu mu yare bi a, ebetumi asakra asetra. aHUS yɛ tebea a emu yɛ den a ebetumi ama mogya ayɛ mmerɛw na asɛe akwaa ahorow. Nanso, aduru bi a wɔfrɛ no eculizumab a wɔayɛ nnansa yi no ama nnipa a wɔwɔ aHUS no nkwa nna atu mpɔn kɛse. Sɛ wɔahu sɛ wowɔ aHUS a, wo ne wo duruyɛfo nkasa mfa akwan a wobɛfa so akwati nneɛma a ɛkanyan wo na woatew asiane a ɛwɔ hɔ sɛ wubenya yare a emu yɛ den so. Obetumi aboa wo ma woate nea ɛsɛ sɛ woyɛ na ama woanya apɔwmuden ase.

` aHUS, asaabo mu yare, mogya a ɛtɔ, nipadua mu ahoɔden a ɛko tia nyarewa, awosu mu nsakrae, mogya a ɛtɔ kɔ nipadua mu, Eculizumab