Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia

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Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia
Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia

Video: Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia

Video: Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia
Video: Difference between Hematuria and Hemoglobinuria 2024, Kaum ib hlis
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Qhov Tseem Ceeb Qhov Sib Txawv - Mob Ntshav Qab Zib Ntshav Qab Zib vs Thalassemia

Thalassemia yog ib pawg ntawm cov kab mob sib txawv uas tshwm sim los ntawm kev hloov pauv hloov pauv uas txo cov synthesis ntawm α -globin lossis β-globin chains. Sickle cell anemia yog ib hom kab mob loj heev ntawm cov kab mob ntshav qab zib uas muaj qhov hloov pauv ntawm hemoglobin cuam tshuam cov qe ntshav liab mus rau hauv cov duab crescent ntawm qis oxygen. Qhov sib txawv tseem ceeb ntawm mob ntshav qab zib thiab thalassemia yog tias nyob rau hauv thalassemia, ob qho tib si α thiab β globin chains tuaj yeem cuam tshuam tab sis nyob rau hauv mob qog noj ntshav tsuas yog cov β globin chains cuam tshuam.

Thalassemia yog dab tsi?

Thalassemia yog ib pawg ntawm cov kab mob sib txawv uas tshwm sim los ntawm kev hloov pauv hloov pauv uas txo qis kev sib txuas ntawm alpha lossis beta globin chains uas ua rau cov neeg laus hemoglobin HbA ua rau cov ntshav tsis txaus, cov ntaub so ntswg hypoxia thiab cov cell liab hemolysis cuam tshuam rau qhov tsis sib xws hauv globin. chain synthesis”.

Thalassemia tuaj yeem faib dav rau hauv ob pawg loj.

  1. Alpha Thalassemia
  2. YBeta Thalassemia.

Alpha Thalassemia

Nyob rau hauv alpha thalassemia, qee cov noob lub luag haujlwm rau kev coding ntawm alpha globin chains raug tshem tawm. Feem ntau, alpha globin noob muaj plaub daim ntawv luam. Qhov hnyav ntawm tus kab mob nyob ntawm tus lej ntawm cov ntawv luam uas ploj lawm.

1. Hydrops Fetalis Y

Thaum tag nrho plaub daim ntawv luam ntawm alpha globin noob ploj lawm, synthesis ntawm alpha globin chains raug txwv. Txij li thaum alpha globin chains yuav tsum tau rau kev sib txuas ntawm ob qho tib si fetal thiab cov neeg laus hemoglobin, tus mob no tsis haum rau lub neej thiab yog li nws ua rau tuag hauv utero.

2. Kab mob HbH

Qhov tsis tuaj ntawm peb daim ntawv theej ntawm alpha globin noob ua rau muaj mob hnyav rau hypochromic microcytic anemia nrog txuam nrog splenomegaly.

3. Cov yam ntxwv ntawm Alpha Thalassemia

Cov no yog vim tsis muaj lossis tsis ua haujlwm ntawm ib lossis ob daim ntawv theej ntawm alpha globin noob. Txawm hais tias alpha thalassemia yam ntxwv tsis ua rau mob ntshav qab zib, lawv tuaj yeem txo qhov nruab nrab lub cev ntim thiab txhais tau tias lub cev hemoglobin qib thaum nce cov qe ntshav liab ntau dua 5.51012/ L.

Qhov txawv tseem ceeb - Sickle Cell Anemia vs Thalassemia
Qhov txawv tseem ceeb - Sickle Cell Anemia vs Thalassemia

Daim duab 01: Keeb kwm ntawm Alpha Thalassemia

YBeta Thalassemia Syndromes

1. Beta Thalassemia Major

Yog tias ob tus niam txiv muaj tus kabmob beta thalassemia, txoj hauv kev uas tus menyuam tau txais beta thalassemia loj yog 25%. Nyob rau hauv cov xwm txheej no, kev tsim cov beta globin chains yog kiag li suppressed los yog drastic txo. Txij li thaum tsis muaj beta globin chains txaus rau lawv los ua ke nrog, ntau tshaj alpha globin chains tau tso rau hauv ob qho tib si paub tab thiab tsis paub qab hau liab. Qhov no ua rau txoj hauv kev rau hemolysis ntxov ntxov ntawm cov hlwb liab thiab tsis muaj txiaj ntsig erythropoiesis.

Tshuaj Kho Mob
  1. mob ntshav qab zib hnyav uas tshwm sim thaum 3- 6 lub hlis tom qab yug menyuam.
  2. Splenomegaly thiab hepatomegaly
  3. thalassemic facies
Kev kuaj mob

Cov txheej txheem tseem ceeb ua haujlwm hauv kev kuaj mob hematological niaj hnub no yog cov kua chromatography (HPLC). Hauv beta thalassemia loj, HPLC qhia tau tias muaj qhov txo qis ntawm HbA ntau ntau nrog cov qib HbF tsis zoo. Cov ntshav suav tag nrho yuav qhia tau tias muaj cov kab mob hypochromic microcytic anemia thaum kev kuaj ntshav cov yeeb yaj kiab yuav qhia pom tias muaj cov reticulocytes ntxiv nrog rau basophilic stippling thiab lub hom phiaj hlwb.

Kev kho mob
  • Txhob muab ntshav
  • Folic acid yog muab yog tias kev noj haus ntawm folic acid tsis txaus siab.
  • Iron chelation therapy
  • Qee zaum splenectomy kuj ua tiav txhawm rau txo qis ntshav.

2. Beta Thalassemia Trait/Me

Beta thalassemia me yog ib yam mob uas tsis muaj tsos mob feem ntau. Txawm hais tias cov yam ntxwv zoo ib yam li alpha thalassemia, beta thalassemia yog qhov hnyav dua li nws tus khub. Kev kuaj mob ntawm beta thalassemia me me yog tsim yog tias HbA 2 qib siab dua 3.5%.

3. Thalassemia Intermedia

Cov neeg mob thalassemia ntawm qhov hnyav me ntsis uas tsis tas yuav hloov pauv li niaj zaus hu ua thalassemia intermedia.

Sickle Cell Anemia yog dab tsi?

Sickle cell anemia yog ib hom kab mob loj heev ntawm kev mob ntshav qab zib uas muaj qhov hloov pauv ntawm hemoglobin cuam tshuam cov qe ntshav liab mus rau hauv cov duab crescent ntawm cov pa oxygen tsawg.

Daim ntawv ntawm hemoglobin tsim los ntawm kev hloov caj ces hauv homozygous sickle cell anemia hu ua HbSS (sickle hemoglobin). Thaum cov pa oxygen poob qis dua ib qho tseem ceeb, cov kab mob hemoglobin tsim cov muaju los ntawm cov txheej txheem ntawm polymerization, tsim cov fibers ntev, txhua tus muaj xya intertwined ob strands nrog kev sib txuas. Nws yog qhov no polymerization uas distorts cov qe ntshav liab mus rau hauv ib tug crescent duab. Txij li thaum cov hlwb liab distorted tau poob lawv txoj kev ua raws, lawv tsis muaj peev xwm dhau los ntawm cov hlab ntsha me me; Hauv cov hlab ntsha me me no, cov kab mob kis tau thaiv cov lumen, cuam tshuam cov ntshav mus rau cov kabmob uas ua rau muaj ntau yam mob.

Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia
Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia

Daim duab 02: Sickle Cell Anemia

Tshuaj Kho Mob Ntawm Sickle Cell Anemia

Hmoov hemolytic anemia punctuated los ntawm kev kub ntxhov tuaj yeem pom.

1. Vaso Occlusive Crises

Yam xws li kab mob, lub cev qhuav dej, acidosis, thiab deoxygenation predispose rau vaso occlusive crises. Tus neeg mob yws ntawm qhov mob hnyav hauv qhov extremities. Qhov no yog vim cov pob txha me me ntawm cov ceg.

2. Visceral Sequestration Crises

Cov no yog vim muaj cov ntshav thiab mob, ua rau hauv lub cev. Qhov teeb meem loj tshaj plaws ntawm qhov no yog mob mob hauv siab mob uas tus neeg mob tuaj yeem ua rau mob plab thiab mob hauv siab. Pulmonary infiltrates tuaj yeem pom ntawm lub hauv siab x-ray.

3. Aplastic crises

Aplastic crises yog qhov tshwm sim los ntawm kev poob qis hauv hemoglobin feem ntau tom qab tus kab mob parvo. Folate deficiency kuj tseem tuaj yeem ua rau muaj txiaj ntsig.

Lwm yam kev kho mob ntawm Sickle Cell Anemia

  • Ntshav kub hnyiab hauv qis qis
  • Pulmonary hypertension
  • Gallstones

Kev kuaj mob ntawm Sickle Cell Anemia

  • qib hemoglobin feem ntau yog 6-9g / dL.
  • Ntseeg cov mob mob thiab lub hom phiaj hauv cov ntshav zaj duab xis.
  • Kev tshuaj ntsuam xyuas mob rau cov tshuaj xws li dithionate yog qhov zoo thaum cov ntshav deoxygenated.
  • Hauv HPLC, tsis pom HbA.

Kev kho mob Sickle Cell Anemia

  • Kev zam txim rau qhov paub tias ua rau muaj kev kub ntxhov.
  • khoom noj khoom haus zoo thiab huv.
  • YPneumococcal, Haemophilus thiab tshuaj tiv thaiv kab mob meningococcal.
  • Kev kub ntxhov yuav tsum tau kho raws li tus mob, hnub nyoog thiab kev ua raws li cov tshuaj ntawm tus neeg mob.

Dab tsi zoo sib xws ntawm Sickle Cell Anemia thiab Thalassemia?

Ob qho tib si thalassemia thiab mob qog noj ntshav yog kab mob caj ces uas cuam tshuam rau cov qauv thiab kev ua haujlwm ntawm hemoglobin

Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia yog dab tsi?

Sickle Cell Anemia vs Thalassemia

Sickle cell anemia yog ib hom kab mob loj heev ntawm kev mob ntshav qab zib uas muaj qhov hloov pauv ntawm hemoglobin cuam tshuam cov qe ntshav liab mus rau hauv cov duab crescent ntawm cov pa oxygen tsawg. Thalassemia yog ib pab pawg neeg sib txawv ntawm cov kab mob tshwm sim los ntawm kev hloov pauv hloov pauv uas txo cov synthesis ntawm α lossis β globin chains.
Hom Globin Chain cuam tshuam
Nyob rau hauv mob qog ntshav ntshav tsuas yog beta chains cuam tshuam. Ob leeg alpha thiab beta chains tuaj yeem cuam tshuam hauv thalassemia.
Nature of Genetic Mutation
Cov noob caj noob ces ua rau mob qog nqaij hlav ntshav ntshav yog kev hloov cov noob. Thalassemia yog tshwm sim los ntawm kev hloov pauv lossis los ntawm kev tshem tawm cov noob.
Resistance Against Malaria
Cov noob caj noob ces ua rau mob qog ntshav ntshav paub tias muaj kev tiv thaiv kab mob malaria. Txiv kab ntxwv tsis xws luag hauv thalassemia tsis tuaj yeem tiv thaiv malaria.
keeb kwm caj ces
Sickle cell anemia yog ib qho mob autosomal recessive. Thalassemia yog ib qho mob autosomal codominant.

Summary – Sickle Cell Anemia vs Thalassemia

Thalassemia thiab mob mob qog noj ntshav yog ob yam kab mob hematological loj heev uas feem ntau ntsib hauv kev xyaum menyuam yaus. Yog li ntawd, nws yog ib qho tseem ceeb kom nkag siab meej qhov sib txawv ntawm cov kab mob cell anemia thiab thalassemia. Kev ua kom lub zej zog paub txog cov kab mob no yuav pab tau kom txo qis qhov tshwm sim ntawm lawv vim tias kev sib yuav sib koom ua ke ua lub luag haujlwm tseem ceeb hauv kev sib kis ntawm cov kab mob no los ntawm ib tiam neeg mus rau ib tiam.

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Koj tuaj yeem rub tawm PDF version ntawm kab lus no thiab siv nws rau offline hom phiaj raws li cov ntawv sau tseg. Thov download PDF version ntawm no Qhov txawv ntawm Sickle Cell Anemia thiab Thalassemia.

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