Congenital nephrotic lefu

Congenital nephrotic syndrome ke lefu le fetisoang ke malapa ao ho lona lesea le hlahisang protheine morong le ho ruruha ha 'mele.

Congenital nephrotic syndrome ke boloetse bo fetelletseng ba liphatsa tsa lefutso. Sena se bolela hore motsoali e mong le e mong o tlameha ho fetisa kopi ea lefutso le nang le sekoli e le hore ngoana a ka ba le lefu lena.

Leha congenital e bolela ho ba teng ho tloha tsoalong, ka congenital nephrotic syndrome, matšoao a lefu lena a hlaha likhoeling tse tharo tsa pele tsa bophelo.

Congenital nephrotic syndrome ke mofuta o sa tloaelehang haholo oa lefu la nephrotic.

Nephrotic syndrome ke sehlopha sa matšoao a kenyeletsang:

• Liprotheine morotong
• Liprotheine tse tlase tsa mali maling
• Lik'holeseterole tse phahameng
• Maemo a phahameng a triglyceride
• Ho ruruha

Bana ba nang le bothata bona ba na le mofuta o sa tloaelehang oa protheine e bitsoang nephrin. Metlhotlo ea liphio (glomeruli) e hloka protheine ena hore e sebetse ka mokhoa o tloaelehileng.

Matšoao a lefu la nephrotic a kenyelletsa:

• Khohlela
• Ho fokotsa tlhahiso ea moroto
• Ponahalo e bobebe ya moroto
• Boima ba 'mele bo tlaase
• Takatso e fosahetseng ea lijo
• Ho ruruha (mmele o felletseng)

Ultrasound e entsoeng ho mme ea moimana e ka bonts'a placenta e kholo ho feta e tloaelehileng. Placenta ke setho se hlahang nakong ea bokhachane ho fepa lesea le ntseng le hola.

Bo-'mè ba nang le bakhachane ba ka 'na ba etsoa tlhahlobo ea tlhahlobisiso nakong ea bokhachane ho hlahloba boemo bona. Teko e sheba litekanyo tse phahameng ho feta tse tloaelehileng tsa alpha-fetoprotein ka sampole ea amniotic fluid. Joale liteko tsa liphatsa tsa lefutso li sebelisoa ho netefatsa tlhahlobo ea tlhahlobo haeba tlhahlobo ea tlhahlobo ea 'mele e le ntle.

Kamora ho hlaha, lesea le tla bontša matšoao a ho boloka metsi a mangata le ho ruruha. Mofani oa tlhokomelo ea bophelo o tla utloa melumo e sa tloaelehang ha a mametse pelo le matšoafo a lesea ka stethoscope. Khatello ea mali e ka ba holimo. Ho ka ba le matšoao a khaello ea phepo e nepahetseng.

Ho hlahlojoa ha metsi ho senola mafura le liprotheine tse ngata morotong. Kakaretso ea protheine maling e ka ba tlase.

Ho hlokahala kalafo ea pele le e mabifi ho laola bothata bona.

Kalafo e ka kenyelletsa:

• Lithibela-mafu ho laola tšoaetso
• Meriana ea khatello ea mali e bitsoang angiotensin-converting enzyme (ACE) inhibitors le angiotensin receptor blockers (ARBs) ho fokotsa palo ea protheine e dutlang ka morong
• Diuretics ("lipilisi tsa metsi") ho tlosa mokelikeli o feteletseng
• Li-NSAID, joalo ka indomethacin, ho fokotsa palo ea protheine e tsoang ka har'a moroto

Maro a ka fokotsoa ho thusa ho laola ho ruruha.

Mofani oa thepa a ka khothaletsa ho tlosa liphio ho emisa tahlehelo ea protheine. Sena se ka lateloa ke dialysis kapa ho kenyetsoa liphio.

Hangata lefu lena le lebisa ho tšoaetso, khaello ea phepo e nepahetseng le ho hloleha ha liphio. E ka lebisa lefung ka lilemo tse 5, mme bana ba bangata ba shoa pele ho selemo. Congenital nephrotic syndrome e ka laoloa maemong a mang ka kalafo ea pele le e mabifi, ho kenyelletsa le ho kenella liphio kapele.

Mathata a boemo bona a kenyelletsa:

• Ho hloleha ho matla ha liphio
• Ho koala mali
• Ho hloleha ha liphio ho sa foleng
• Boloetse ba liphio qetellong
• Khafetsa, tšoaetso e matla
• Khaello ea phepo e nepahetseng le mafu a amanang le ona

Bitsa mofani oa hau haeba ngoana oa hau a e-na le matšoao a congenital nephrotic syndrome.

Nephrotic syndrome, - tsoalo

• Mosali pampitšana ea mosese
• Mokhoa oa banna oa ho ntša metsi

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Schlöndorff J, Pollak Monghali. Mathata a futsitsoeng a glomerulus. Ka: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, li-eds. Brenner le Rector ea Liphio. 11th ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 43.

Vogt BA, Springel T. Liphio le mosese oa lesea le sa tsoa hlaha. Ka: Martin RJ, Fanaroff AA, Walsh MC, bangoli. Bongaka ba Fanaroff le Martin's Neonatal-Perinatal Medicine: Maloetse a lesea le ka lesea. 11th ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 93.