Primary amyloidosis

Primary amyloidosis chirwere chisingawanzoitika umo mapuroteni asina kujairika anovaka mumatumbu uye nhengo. Clumps emapuroteni asina kujairika anonzi maamyloid deposits.

Chikonzero chekutanga amyloidosis hachina kunyatsonzwisiswa. Majini anogona kutora chinzvimbo.

Mamiriro acho ane hukama nekusajairika uye kuwedzeredza kugadzirwa kwemapuroteni. Mapundu emapuroteni asina kujairika anovakwa mune dzimwe nhengo. Izvi zvinoita kuti zviomere nhengo kuti dzishande nemazvo.

Primary amyloidosis inogona kutungamira kumamiriro anosanganisira:

• Carpal tunnel chirwere
• Kukuvara kwemhasuru yemwoyo (cardiomyopathy) kunotungamira kukundikana kwemoyo kukundikana
• Utumbo malabsorption
• Chiropa kuzvimba uye kusashanda
• Itsvo kutadza
• Nephrotic syndrome (boka rezviratidzo zvinosanganisira mapuroteni mumurini, mapuroteni eropa mashoma muropa, cholesterol yakawanda, mwero we triglyceride, uye kuzvimba mumuviri wese)
• Matambudziko etsinga (neuropathy)
• Orthostatic hypotension (donha muropa kana iwe uchisimuka)

Zviratidzo zvinoenderana nenhengo dzakabatwa. Chirwere ichi chinogona kukanganisa mazhinji mitezo uye zvinyama, zvinosanganisira rurimi, ura, marangwanda uye akatsetseka tsandanyama, tsinga, ganda, mitsipa, moyo, chiropa, chirwatata, neitsvo.

Zviratidzo zvinogona kusanganisira chero zvinotevera:

• Abnormal moyo mutinhimira
• Kuneta
• Kuvezwa kwemaoko kana tsoka
• Kufema kwekufema
• Ganda rinochinja
• Kumedza matambudziko
• Kuzvimba mumaoko nemakumbo
• Rurimi rwakazvimba
• Ruoko rwakasimba
• Kurera huremu kana kuwedzera huremu

Zvimwe zviratidzo zvinogona kuitika nechirwere ichi:

• Yakaderera weti kubuda
• Manyoka
• Hoarseness kana kuchinja izwi
• Joint kurwadziwa
• Kushaya simba

Anokuchengetedza hutano achakuongorora. Iwe uchazobvunzwa nezve yako nhoroondo yezvokurapa uye zviratidzo. Kuongorora kwemuviri kunogona kuratidza kuti une chiropa chakazvimba kana chirwatata, kana zviratidzo zvekukuvara kwetsinga.

Nhanho yekutanga yekuongorora amyloidosis inofanirwa kuve yeropa uye weti bvunzo kutsvaga mapuroteni asina kujairika.

Mimwe bvunzo inoenderana nezviratidzo zvako uye nhengo iyo inogona kukanganisika. Mimwe bvunzo inosanganisira:

• Abdominal ultrasound kutarisa chiropa uye spleen
• Miedzo yemoyo, senge ECG, kana echocardiogram, kana MRI
• Itsvo basa rekuyedza kutarisa zviratidzo zveitsvo kutadza (nephrotic syndrome)

Miedzo inogona kubatsira kusimbisa kuongororwa kunosanganisira:

• Abdominal fat pad kuda
• Bone marora bhiopsy
• Mwoyo mhasuru biopsy
• Rectal mucosa biopsy

Kurapa kunogona kusanganisira:

• Chemotherapy
• Stem cell kuisirwa
• Kuisa muviri

Kana mamiriro acho achikonzerwa nechimwe chirwere, chirwere ichocho chinofanirwa kurapwa zvine hukasha. Izvi zvinogona kuvandudza zviratidzo kana kudzora chirwere kubva pakuwedzera. Zvinetso zvakaita sekutadza kwemoyo, itsvo kutadza, uye mamwe matambudziko dzimwe nguva anogona kurapwa, pazvinodiwa.

Kunyatsoita kwako kunoenderana nenhengo dzipi dzinokanganiswa. Kubatanidzwa kwemoyo neitsvo kunogona kutungamira kukundikana kwemuviri uye kufa. Muviri-wakapamhama (systemic) amyloidosis inogona kutungamira kurufu mukati memakore maviri.

Fonera mupiki wako kana iwe uine zviratidzo zvechirwere ichi. Zvakare fona kana iwe waonekwa uine chirwere ichi uye uine:

• Kuderedza weti
• Kunetseka kufema
• Kuzvimba kwemakumbo kana zvimwe zvikamu zvemuviri zvisingaendi

Iko hakuna kuzivikanwa kudzivirira kwekutanga amyloidosis.

Amyloidosis - chepuraimari; Immunoglobulin mwenje cheni amyloidosis; Primary systemic amyloidosis

• Amyloidosis yeminwe
• Amyloidosis yechiso

Gertz MA, Buadi FK, Lacy MQ, Hayman SR. Immunoglobulin mwenje-cheni amyloidosis (primary amyloidosis). Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chits. 88.

Hawkins PN. Amyloidosis.Mu: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019: chitsiko 177.