Tizilombo toyambitsa matenda tizilombo toyambitsa matenda

Hereditary spherocytic anemia ndikosowa kwazomwe zimachitika pakhungu lanthaka yamagazi. Zimatsogolera ku maselo ofiira ofiira omwe amapangidwa ngati magawo, ndi kuwonongeka msanga kwa maselo ofiira (hemolytic anemia).

Matendawa amayamba chifukwa cha jini lopunduka. Vutoli limabweretsa khungu lachilendo lofiira. Maselo omwe akhudzidwa amakhala ndi malo ocheperako kuposa kuchuluka kwa magazi ofiira, ndipo amatha kutseguka mosavuta.

Kuchepa kwa magazi kumatha kusiyanasiyana kuchokera pakuchepa kufikira koopsa. Pazovuta kwambiri vutoli limatha kupezeka adakali ana. Nthawi zochepa zimatha kuzindikirika mpaka munthu wamkulu.

Matendawa amapezeka kwambiri kwa anthu ochokera kumadera akumpoto kwa Europe, koma amapezeka m'mitundu yonse.

Makanda atha kukhala achikasu pakhungu ndi maso (jaundice) ndi utoto wotumbululuka (pallor).

Zizindikiro zina zitha kuphatikiza:

• Kutopa
• Kukwiya
• Kupuma pang'ono
• Kufooka

Nthawi zambiri, ndulu imakulitsidwa.

Mayeso a labotale atha kuthandiza kuzindikira izi. Mayeso atha kuphatikiza:

• Magazi opaka magazi kuti asonyeze maselo opangidwa modabwitsa
• Mulingo wa Bilirubin
• Kuwerengera magazi kwathunthu kuti muwone kuchepa kwa magazi
• Mayeso a Coombs
• Mulingo wa LDH
• Kufooka kwa Osmotic kapena kuyesa kwapadera kuti muwone ngati vuto la khungu lofiira la magazi
• Kuwerengera kwa reticulocyte

Opaleshoni yochotsa ndulu (splenectomy) imachiritsa kuchepa kwa magazi koma siyikonza mawonekedwe achilendo a khungu.

Mabanja omwe ali ndi mbiri ya spherocytosis amayenera kuti ana awo awone vutoli.

Ana ayenera kudikirira mpaka zaka 5 kuti akhale ndi splenectomy chifukwa cha chiopsezo chotenga kachilomboka. Pazochepa zomwe zimapezeka mwa akulu, mwina sikungakhale kofunikira kuchotsa ndulu.

Ana ndi akulu ayenera kupatsidwa katemera wa pneumococcal musanachite opaleshoni yochotsa ndulu. Ayeneranso kulandira zowonjezera zowonjezera folic acid. Katemera wowonjezera angafunike kutengera mbiri ya munthuyo.

Zinthu zotsatirazi zitha kupereka chidziwitso chambiri chokhudzana ndi kuchepa kwa magazi m'thupi:

• Chidziwitso cha Matenda a Chibadwa ndi Rare - rarediseases.info.nih.gov/diseases/6639/redited-spherocytosis
• National Organisation for Rare Disways - rarediseases.org/rare-diseases/anemia-hereditary-spherocytic-hemolytic

Zotsatira zake nthawi zambiri zimakhala zabwino ndi chithandizo. Ndulu ikachotsedwa, nthawi yamoyo yamaselo ofiira imabwerera mwakale.

Zovuta zingaphatikizepo:

• Miyala
• Kuchuluka kwa maselo ofiira am'magazi (aplastic crisis) omwe amayamba chifukwa cha ma virus, omwe amatha kupangitsa kuchepa kwa magazi m'thupi

Imbani wothandizira zaumoyo wanu ngati:

• Zizindikiro zanu zimaipiraipira.
• Zizindikiro zanu sizikusintha ndikalandira chithandizo chatsopano.
• Mumakhala ndi zizindikilo zatsopano.

Ili ndi vuto lobadwa nalo ndipo mwina silingalephereke. Kudziwa chiopsezo chanu, monga mbiri ya banja la vutoli, kungakuthandizeni kuti mupezeke ndikuchiritsidwa msanga.

Kobadwa nako spherocytic hemolytic magazi m`thupi; Spherocytosis; Hemolytic magazi m'thupi - spherocytic

• Maselo ofiira ofiira - abwinobwino
• Maselo ofiira ofiira - spherocytosis
• Maselo amwazi

Gallagher PG. Matenda ofiira a khungu la magazi. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 45.

Merguerian MD, Gallagher PG. Cholowa spherocytosis. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 485.