IGlanzmann thrombasthenia

IGlanzmann thrombasthenia yisifo esingajwayelekile samaplatelets egazi. Ama-platelet ayingxenye yegazi elisiza ekunqandeni igazi.

I-Glanzmann thrombasthenia ibangelwa ukungabikho kweprotheni evame ukuba ngaphezulu kwama-platelet. Le nto iyadingeka kuma-platelet ukuze ahlangane ndawonye enze amahlule egazi.

Isimo singesokuzalwa, okusho ukuthi sikhona kusukela ekuzalweni. Kunokukhubazeka okuningana kwezakhi zofuzo okungadala isimo.

Izimpawu zingafaka noma yikuphi okulandelayo:

• Ukopha kakhulu ngesikhathi nangemuva kokuhlinzwa
• Izinsini zokopha
• Ukulimaza kalula
• Ukopha okukhulu kokuya esikhathini
• Ukopha okungapheli kalula
• Ukopha isikhathi eside ngokulimala okuncane

Ukuhlolwa okulandelayo kungasetshenziselwa ukuxilonga lesi simo:

• Qedela ukubalwa kwegazi (CBC)
• Ukuhlolwa kokuhlanganiswa kwePlatelet
• Ukuhlaziywa komsebenzi wePlatelet (PFA)
• Isikhathi se-Prothrombin (PT) nesikhathi esinqunyelwe se-thromboplastin (PTT)

Okunye ukuhlolwa kungadingeka. Amalungu omndeni nawo angadinga ukuhlolwa.

Akukho ukwelashwa okuqondile kwalesi sifo. Ukumpontshelwa kweplatelet kunganikezwa kubantu abaphuma kakhulu egazini.

Lezi zinhlangano ezilandelayo ziyizinsizakusebenza ezinhle zokwaziswa ngeGlanzmann thrombasthenia:

• Isikhungo Sokwaziswa Sezifo Zofuzo kanye Nesifo Esingajwayelekile (GARD) - rarediseases.info.nih.gov/diseases/2478/glanzmann-thrombasthenia
• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile (NORD) - rarediseases.org/rare-diseases/glanzmann-thrombasthenia

IGlanzmann thrombasthenia iyisimo sempilo yonke, futhi alikho ikhambi. Kufanele uthathe izinyathelo ezikhethekile ukuzama ukugwema ukopha uma unalesi simo.

Noma ngubani onenkinga yokopha kufanele agweme ukuthatha i-aspirin nezinye izidakamizwa ezingezona ukuvuvukala (ama-NSAID) njenge-ibuprofen ne-naproxen. Le mithi ingangezelela izikhathi zokopha ngokuvimbela ama-platelet ekuqunjaneni.

Izinkinga zingafaka:

• Ukopha kakhulu
• Ukushoda kwe-iron anemia kubantu besifazane abasesikhathini ngenxa yokopha ngokweqile ngokungajwayelekile

Shayela umhlinzeki wakho wezempilo uma:

• Unegazi noma ukulinyazwa kwesizathu esingaziwa
• Ukopha akuyeki ngemuva kokwelashwa okujwayelekile

IGlanzmann thrombasthenia yisimo esizuzwe njengefa. Akukho ukuvimbela okwaziwayo.

Isifo sikaGlanzmann; I-Thrombasthenia - uGlanzmann

UBhatt MD, Ho K, uKhan AKC. Ukuphazamiseka kwe-coagulation ku-neonate. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 150.

I-Nichols WL. Isifo se-Von Willebrand nokungajwayelekile kokopha kweplatelet nokusebenza kwemithambo yegazi. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 173.