Ho haelloa ke lefu la congenital fibrinogen

Ho haelloa ke lefu la congenital fibrinogen ke lefu le sa tloaelehang haholo, le futsitsoeng la mali leo ho lona mali a sa koaleng hantle. E ama protheine e bitsoang fibrinogen. Protheine ena ea hlokahala hore mali a hoama.

Lefu lena le bakoa ke liphatsa tsa lefutso tse sa tloaelehang. Fibrinogen ea ameha ho latela hore na liphatsa tsa lefutso li futsitsoe joang:

• Ha lefutso le sa tloaelehang le fetisoa ho tsoa ho batsoali ka bobeli, motho o tla ba le khaello e felletseng ea fibrinogen (afibrinogenemia).
• Ha liphatsa tsa lefutso tse sa tloaelehang li fetisoa ho tsoa ho motsoali a le mong, motho o tla ba le boemo bo fokotsehileng ba fibrinogen (hypofibrinogenemia) kapa bothata ba tšebetso ea fibrinogen (dysfibrinogenemia). Ka linako tse ling, mathata ana a mabeli a fibrinogen a ka hlaha ho motho a le mong.

Batho ba nang le khaello e felletseng ea fibrinogen ba ka ba le matšoao a latelang a ho tsoa mali:

• Ho rohakana habonolo
• Ho tsoa mali mokokotlong hang feela kamora ho hlaha
• Ho tsoa mali ka lera la mucous
• Ho tsoa mali bokong (ho sa tloaelehang haholo)
• Ho tsoa mali manonyeletso
• Ho tsoa mali haholo kamora ho tsoa kotsi kapa ho buuoa
• Madi a nkoang madi a sa emeng habonolo

Batho ba nang le boemo bo fokotsehileng ba fibrinogen ba tsoa mali khafetsa mme ho tsoa mali ha ho matla joalo. Ba nang le bothata ba tšebetso ea fibrinogen hangata ha ba na matšoao.

Haeba mofani oa tlhokomelo ea bophelo a belaella bothata bona, o tla ba le liteko tsa laboraka ho netefatsa mofuta le ho tiea ha lefu lena.

Liteko li kenyelletsa:

• Nako ea ho tsoa mali
• Teko ea Fibrinogen le nako ea ho pheta-pheta ho lekola boemo ba fibrin le boleng
• Nako ea karoloana ea thromboplastin (PTT)
• Nako ea Prothrombin (PT)
• Nako ea Thrombin

Litlhare tse latelang li ka sebelisoa bakeng sa liketsahalo tse tsoang mali kapa ho itokisetsa ho buuoa:

• Cryoprecipitate (sehlahisoa sa mali se nang le fibrinogen e kopaneng le lintho tse ling tse hoamisa)
• Fibrinogen (RiaSTAP)
• Plasma (karolo e mokelikeli ea mali e nang le lintho tse hoamisang)

Batho ba nang le boemo bona ba lokela ho fumana vaksine ea hepatitis B. Ho ba le litšelo tse ngata ho phahamisa kotsi ea ho tšoaroa ke lefu la sebete.

Ho tsoa mali a mangata ho tloaelehile ka boemo bona. Likarolo tsena li ka ba mpe, kapa tsa bolaea. Ho tsoa mali bokong ke sesosa se ka sehloohong sa lefu ho batho ba nang le bothata bona.

Mathata a ka kenyelletsa:

• Ho koala mali ka kalafo
• Nts'etsopele ea lithibela-mafu (inhibitors) ho fibrinogen ka kalafo
• Ho tsoa mali ka mpeng
• Ho nyopa
• Ho phatloha ha spleen
• Ho fola butle ha maqeba

Bitsa mofani oa hau kapa u batle tlhokomelo ea tšohanyetso haeba u tsoa mali a mangata.

Bolella ngaka ea hao e buoang pele u etsoa opereishene haeba u tseba kapa u belaella hore u na le lefu la ho tsoa mali.

Ena ke boemo bo futsitsoeng. Ha ho na thibelo e tsebahalang.

Afibrinogenemia; Hypofibrinogenemia; Dysfibrinogenemia; Bothata ba ntlha ea I

Gailani D, Wheeler AP, Neff AT. Mathata a sa tloaelehang a li-coagulation. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 137.

Ragni MV. Mathata a hemorrhagic: ho haelloa ke lebaka la coagulation. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 174.