Ọrịa Sickle cell

Ọrịa Sickle cell bụ ọgba aghara sitere n'ezinụlọ. Mkpụrụ ndụ ọbara uhie nke na-adịkarị ka diski na-ewere sickle ma ọ bụ mpekere onwa. Mkpụrụ ndụ ọbara ọbara na-ebu oxygen n’ahụ niile.

Ọrịa Sickle cell bụ ụdị ụdị haemoglobin a na - akpọ haemoglobin S. Hemoglobin bụ protein na - adị n’ime mkpụrụ ndụ ọbara uhie nke na - eburu oxygen.

• Hemoglobin S na-agbanwe mkpụrụ ndụ ọbara uhie. Mkpụrụ ndụ ọbara uhie na-emebi emebi ma yie ka ọnwa ma ọ bụ sickles.
• Mkpụrụ ndụ ndị na-adịghị ahụkebe na-ebunye obere oxygen na anụ ahụ.
• Ha nwekwara ike ịrapagidesi ike n’ime obere arịa ọbara ma gbawasịa iberibe. Nke a nwere ike ịkwụsị ọgbụgba ọbara dị mma ma belata karịa karịa ọnụọgụ oxygen na-erute na anụ ahụ.

Ọrịa Sickle cell ketara n'aka nne na nna. Ọ bụrụ na ịnwe mkpụrụ ndụ sickle cell site n'aka naanị nne ma ọ bụ nna, ị ga-enwe njirimara sickle cell. Ndị mmadụ na-arịa ọrịa sickle cell enweghị akara nke ọrịa sickle cell.

Ọrịa Sickle cell na-adịkarịkarị na ndị agbụrụ Africa na Mediterranean. A na-ahụkwa ya na ndị sitere na South na Central America, Caribbean, na Middle East.

Mgbaàmà anaghị emekarị ruo mgbe ọnwa 4 gachara.

Ihe fọrọ nke nta ka ọ bụrụ ndị niile nwere ọrịa sickle cell nwere oge mgbu na-akpọ ọdụdọ. Ndị a nwere ike ịdịru ọtụtụ awa ruo ụbọchị. Ọgba aghara nwere ike ịkpata ihe mgbu na azụ ala, ụkwụ, nkwonkwo, na obi.

Fọdụ ndị nwere otu akụkọ kwa afọ ole na ole. Ndị ọzọ nwere ọtụtụ akụkọ kwa afọ. Ọgba aghara a nwere ike ịdị njọ nke na ịchọrọ ụlọ ọgwụ.

Mgbe anaemia na-akawanye njọ, ihe mgbaàmà nwere ike ịgụnye:

• Ike ọgwụgwụ
• Mbibi
• Otutu obi
• Iku ume ọkụ ọkụ
• Yellowing nke anya na anụ ahụ (jaundice)

Mụaka ndị nwere obere sickle cell nwere mwakpo nke mgbu afọ.

Ihe mgbaàmà ndị a nwere ike ịpụta n'ihi na obere akwara na - egbochi mkpụrụ ndụ ndị na - adịghịzi arụ ọrụ:

• Ogwu na-egbu mgbu na ogologo oge (priapism)
• Ọhụhụ ụzọ ma ọ bụ ìsì na-adịghị mma
• Nsogbu nke iche echiche ma ọ bụ ọgba aghara nke obere ọrịa strok kpatara
• Ọnya na ụkwụ ala (na-eto eto na ndị okenye)

Ka oge na-aga, splin ahụ na-akwụsị ịrụ ọrụ. N'ihi ya, ndị mmadụ nwere ọrịa sickle cell nwere ike ịnwe mgbaàmà nke ọrịa dị ka:

• Ọkpụkpụ ọrịa (osteomyelitis)
• Gallbladder ọrịa (cholecystitis)
• Lung ọrịa (oyi baa)
• Urinary tract ọrịa

Ihe ịrịba ama ndị ọzọ gụnyere:

• Igbu oge na ntorobịa
• Nkwonkwo na-egbu mgbu nke ọrịa ogbu na nkwonkwo kpatara
• Obi ma ọ bụ imeju imeju n'ihi oke iron (site na mmịnye ọbara)

Nnwale a na-eme iji chọpụta ma nyochaa ndị nwere ọrịa sickle cell gụnyere:

• Bilirubin
• Nnukwu ikuku oxygen
• Ọnụọgụ ọbara zuru ezu (CBC)
• Hemoglobin electrophoresis
• Ọbara creatinine
• Ọbara potassium
• Nnwale Sickle cell

Ebumnuche nke ọgwụgwọ bụ ijikwa ma chịkwaa mgbaàmà, yana belata ọnụ ọgụgụ nke ọgba aghara. Ndị nwere ọrịa sickle cell chọrọ ọgwụgwọ na-aga n'ihu, ọbụlagodi mgbe ha enweghị nsogbu.

Ndị mmadụ nwere ụdịrị ọrịa a kwesịrị ị takeụ folic acid. Folic acid na-enyere aka ime ka ọbara uhie uhie ọhụrụ.

Ọgwụgwọ ọrịa sickle cell gụnyere:

• Mmịnye ọbara (a pụkwara inye ya mgbe niile iji gbochie ọrịa strok)
• Ọgwụ mgbu
• Ọtụtụ mmiri

Usoro ọgwụgwọ ndị ọzọ maka ọrịa sickle cell nwere ike ịgụnye:

• Hydroxyurea (Hydrea), nke na - enyere aka belata ọnụọgụ mgbu (gụnyere mgbu obi na nsogbu iku ume) na ụfọdụ ndị
• Ọgwụ nje, nke na-enyere aka igbochi ọrịa nje na-adịkarị na ụmụaka nwere ọrịa sickle cell
• Ọgwụ ndị na-ebelata ọnụọgụ igwe dị n’ahụ
• A kwadoro usoro ọgwụgwọ ọhụụ iji belata oge na oke nke nsogbu nsogbu

Ọgwụ ndị nwere ike ịchọ iji jikwaa nsogbu nke ọrịa sickle cell gụnyere:

• Dialysis ma ọ bụ ịkpụgharị akụrụ maka ọrịa akụrụ
• Ndụmọdụ maka nsogbu uche
• Mwepụ nke Gallbladder na ndị nwere ọrịa gallstone
• Mgbanwe hip maka necrosis nke avascular nke hip
• Gerywa ahụ maka nsogbu anya
• Ọgwụgwọ maka ịuseụbiga mmanya ókè ma ọ bụ mmegbu nke ọgwụ mgbu narcotic
• Nlekọta ọnya maka ọnya ụkwụ

Ọkpụkpụ ma ọ bụ mkpochi sel cell nwere ike ịgwọ ọrịa sickle cell, mana ọgwụgwọ a abụghị nhọrọ maka ọtụtụ mmadụ. Ndị mmadụ na-arịa ọrịa sickle cell anaghị ahụkarị ndị nyere onyinye sel sel nke ọma.

Ndị nwere ọrịa sickle cell kwesịrị ịnwe ọgwụ mgbochi ndị a iji belata ihe egwu maka ibute ọrịa:

• Haemophilus influenzae ogwu (Hib)
• Pneumococcal conjugate ogwu (PCV)
• Pneumococcal polysaccharide ogwu (PPV)

Banye na otu nkwado ebe ndị otu na-ekerịta otu okwu nwere ike belata nrụgide nke ọrịa na-adịghị ala ala.

N’oge gara aga, ndị nwere ọrịa sickle cell na-anwụkarị n’agbata afọ 20 na 40. Site na nlekọta nke oge a, ndị mmadụ ugbu a nwere ike ịdị ndụ ruo afọ 50 na karịrị.

Ihe ndị na-akpata ọnwụ gụnyere ịkpụ akụkụ ahụ́ na ọrịa.

Kpọọ onye nlekọta ahụike gị ma ọ bụrụ na ịnwee:

• Ihe mgbaàmà ọ bụla nke ọrịa (ahụ ọkụ, ahụ mgbu, isi ọwụwa, ike ọgwụgwụ)
• Nsogbu nsogbu
• Mgbatị na-egbu mgbu na ogologo oge (n'ime ụmụ nwoke)

Anemia - sickle cell; Hemoglobin SS ọrịa (Hb SS); Ọrịa Sickle cell

• Mkpụrụ ndụ ọbara uhie, sel sickle cell
• Mkpụrụ ndụ ọbara ọbara - nkịtị
• Mkpụrụ ndụ ọbara ọbara - ọtụtụ sel sickle
• Mkpụrụ ndụ ọbara uhie - mkpụrụ ndụ sickle cell
• Mkpụrụ ndụ ọbara ọbara - sickle na Pappenheimer
• Emebe ọbara
• Mkpụrụ ndụ ọbara

Howard J. Ọrịa Sickle cell na hemoglobinopathies ndị ọzọ. Na: Goldman L, Schafer AI, eds. Ọgwụ Goldman-Cecil. Nke 26th. Philadelphia, PA: Elsevier; 2020: isi 154.

Meier ER. Nhọrọ ọgwụgwọ maka ọrịa sickle cell. Pediatr Ọrịa North Am. 2018; 65 (3) 427-443. PMID 29803275 pubmed.ncbi.nlm.nih.gov/29803275/.

National Heart Lung na Ọbara Institute mkpokọta. Nlekọta dabere na akaebe nke ọrịa sickle cell: mkpesa kọmpụta ọkachamara, 2014. www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. Emelitere September 2014. Nabata Jenụwarị 19, 2018.

Saunthararajah Y, Vichinsky EP. Ọrịa Sickle cell: atụmatụ nlekọta na njikwa. Na: Hoffman R, Benz EJ, Silberstein LE, et al, ndị ọzọ. Hematology: ickpụrụ na Omume. Nke 7. Philadelphia, PA: Elsevier; 2018: isi 42.

Smith-Whitley K, Kwiatkowski JL. Hemoglobinopathies. Na: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Akwụkwọ ọgụgụ nke Ọrịa edimụaka. 21nke. Philadelphia, PA: Elsevier; 2020: isi 489.