Matenda a Bassen-Kornzweig

Matenda a Bassen-Kornzweig ndi matenda osowa omwe amapezeka m'mabanja. Munthuyo sangathe kuyamwa mafuta azakudya m'matumbo.

Matenda a Bassen-Kornzweig amayamba chifukwa cha chilema mumtundu womwe umauza thupi kuti lipange lipoprotein (ma molekyulu amafuta pamodzi ndi mapuloteni). Cholakwikacho chimapangitsa kuti zikhale zovuta kuti thupi ligaye bwino mavitamini ndi mavitamini ofunikira.

Zizindikiro zake ndi izi:

• Kusamala ndi kulumikizana
• Kupindika msana
• Kuchepetsa masomphenya omwe amafika poipa pakapita nthawi
• Kuchedwa kwakukula
• Kulephera kukula bwino (ubwana) ukhanda
• Minofu kufooka
• Kusagwirizana kwa minofu komwe kumayamba pambuyo pa zaka 10
• Kutuluka pamimba
• Mawu osalankhula
• Zoyipa zampando, kuphatikiza ndowe zamafuta zomwe zimawoneka zotuwa, zotayira, ndi mipando yonyansa

Pakhoza kukhala kuwonongeka kwa diso la diso (retinitis pigmentosa).

Mayeso omwe angachitike kuti athetse vutoli ndi awa:

• Kuyezetsa magazi kwa Apolipoprotein B
• Mayeso amwazi kufunafuna zoperewera zama vitamini (mavitamini osungunuka mafuta A, D, E, ndi K)
• "Burr-cell" kusokonekera kwa maselo ofiira (acanthocytosis)
• Kuwerengera kwathunthu kwa magazi (CBC)
• Kafukufuku wa cholesterol
• Zojambulajambula
• Kuyezetsa maso
• Kuthamanga kwamitsempha
• Kusanthula kwazitsulo

Kuyesedwa kwa majini kumatha kupezeka pakusintha kwa MTP jini.

Chithandizochi chimaphatikizapo kuchuluka kwa mavitamini okhala ndi mavitamini osungunuka ndi mafuta (vitamini A, vitamini D, vitamini E, ndi vitamini K).

Linoleic acid amathandizanso.

Anthu omwe ali ndi vutoli ayenera kuyankhula ndi katswiri wazakudya. Kusintha kwa zakudya kumafunika kuti tipewe mavuto am'mimba. Izi zitha kuphatikizira kuchepetsa mafuta amtundu wina.

Zowonjezera zama triglycerides apakatikati amatengedwa moyang'aniridwa ndi othandizira azaumoyo. Ziyenera kugwiritsidwa ntchito mosamala, chifukwa zimatha kuwononga chiwindi.

Momwe munthu amachitira bwino zimadalira kuchuluka kwa mavuto am'magazi ndi dongosolo lamanjenje.

Zovuta zingaphatikizepo:

• Khungu
• Kuwonongeka kwamaganizidwe
• Kutaya ntchito kwa mitsempha ya m'mimba, kuyenda kosagwirizana (ataxia)

Itanani omwe amakupatsani ngati mwana wanu wakhanda ali ndi zizindikiro za matendawa. Upangiri wa chibadwa ungathandize mabanja kumvetsetsa vutoli komanso kuopsa kololera, ndikuphunzira momwe angamusamalire.

Kuchuluka kwa mavitamini osungunuka ndi mafuta kumatha kuchepetsa kukula kwa mavuto ena, monga kuwonongeka kwa diso komanso kuchepa kwamaso.

Abetalipoproteinemia; Acanthocytosis; Kuperewera kwa Apolipoprotein B

Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Zofooka za metabolism mu lipids. 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 104.

Shamir R. Zovuta zakusalaborption. 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 364.