Ọrịa nephrotic congenital

Ọrịa a na-akpọ 'nephrotic syndrome' bụ ọgbaghara na-agbasa site n'ezinaụlọ nke nwa na-etolite protein na mmamịrị yana ọzịza nke ahụ.

Ọrịa nephrotic nke congenital bụ autosomal recessive mkpụrụ ndụ ihe nketa. Nke a pụtara na nne ma ọ bụ nna ọ bụla ga-ebufe otu mkpụrụ ndụ ihe na-emezighị emezi ka nwa ahụ nwee ọrịa ahụ.

Agbanyeghi na congenital putara ugbua site na nwa, ya na oria nephrotic syndrome, congenital nephrotic syndrome, ihe mgbaàmà nke oria na adi na onwa mbu nke 3 nke ndu

Ọrịa na-arịa ọrịa nephrotic bụ ụdị ọrịa nephrotic na-adịghị ahụkebe.

Ọrịa Nephrotic bụ otu mgbaàmà nke gụnyere:

• Protein na mmamịrị
• Ogologo protein dị n'ọbara
• Akwa cholesterol
• Nnukwu triglyceride etoju
• Ọzịza

Childrenmụaka nwere nsogbu a nwere nsogbu dị iche iche nke protein a na-akpọ nephrin. Ihe nzacha akụrụ (glomeruli) chọrọ protein a ka ọ rụọ ọrụ nke ọma.

Mgbaàmà nke ọrịa nephrotic gụnyere:

• .Kwara
• Mbelata mmepụta mmamịrị
• Amfụfụ ọdịdị nke mmamịrị
• Obere ọmụmụ nwa
• Agụụ na-adịghị mma
• Ọzịza (ngụkọta ahụ)

Ihe ultrasound emere na nne dị ime nwere ike igosi placenta buru ibu karịa nke nkịtị. Plasenta bụ akụkụ na - amalite n’oge afọ ime iji zụọ nwa na - eto eto.

Ndị nne dị ime nwere ike ịnwale ule nyocha n'oge ime ime iji lelee ọnọdụ a. Nnwale ahụ na-achọ ọkwa dị elu karịa nke alpha-fetoprotein na mmiri nke mmiri ọmụmụ. A na-ejizi mkpụrụ ndụ ihe nketa iji gosipụta nchoputa ahụ ma ọ bụrụ na ule nyocha ahụ dị mma.

Mgbe amuchara nwa, nwa a ga - egosi ihe nlere anya nke mmiri mmiri na - agha ma na ọzịza. Onye nlekọta ahụike ga-anụ ụda na-adịghị mma mgbe ị na-ege obi na ngụgụ nwa ahụ ntị na stethoscope. Ọbara mgbali elu. Enwere ike inwe ihe ịrịba ama nke erighị ihe na-edozi ahụ.

Otu mmamịrị na-egosi abụba na nnukwu protein dị na mmamịrị ahụ. Mkpokọta protein n'ime ọbara nwere ike ịdị obere.

Ọ dị mkpa iji gbochie ọgwụgwọ na-eme ihe ike iji chịkwaa nsogbu a.

Ọgwụgwọ nwere ike ịgụnye:

• Ọgwụ nje iji chịkwaa ọrịa
• Ọbara mgbali elu a na - akpọ ndị na - emechi angiotensin na - - enzyme (ACE) na ndị na - egbochi ihe nnabata angiotensin (ARBs) iji belata oke protein na - abanye n’ime mmamịrị.
• Diuretics ("ọgwụ ọgwụ") iji wepu oke mmiri mmiri
• NSAIDs, dị ka indomethacin, iji belata oke protein na-abanye n'ime mamịrị

Fluids nwere ike ịbụ oke iji nyere aka ịchịkwa ọzịza.

Onye na-enye ọrụ ahụ nwere ike ịkwado iwepu akụrụ iji kwụsị protin. Nke a nwere ike soro dialysis ma ọ bụ transplant akụrụ.

Nsogbu a na-ebutekarị ọrịa, erighị ihe na-edozi ahụ, na akụrụ akụrụ. O nwere ike ibute ọnwụ site na afọ 5, ọtụtụ ụmụaka na-anwụkwa n'ime afọ mbụ. Congenital nephrotic syndrome nwere ike na-achịkwa ụfọdụ ụfọdụ na n'oge na ike ike ọgwụgwọ, gụnyere ihe n'oge akụrụ transplant.

Nsogbu nke ọnọdụ a gụnyere:

• Nnukwu akụrụ akụrụ
• Ọbara ọbara
• Adịghị ala ala akụrụ
• Ọgwụgwụ akụrụ
• Ugboro, ajọ ọrịa
• Erighị ihe na-edozi ahụ na ọrịa ndị yiri ya

Kpọọ onye na-ahụ maka ọrụ gị ma ọ bụrụ na nwa gị nwere mgbaàmà nke ọrịa nephrotic syndrome.

Ọrịa Nephrotic - congenital

• Nwanyi urinary tract
• Nwoke urinary tract

Ọrịa Erkan E. Nephrotic. Na: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Akwụkwọ ọgụgụ nke Ọrịa edimụaka. 21nke. Philadelphia, PA: Elsevier; 2020: isi 545.

Schlöndorff J, Pollak MR. Nsogbu eketa nke glomerulus. Na: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. Brenner na Rector nke akụrụ. Nke 11. Philadelphia, PA: Elsevier; 2020: isi nke 43.

Vogt BA, Springel T. Akụrụ na urinary tract nke neonate. Na: Martin RJ, Fanaroff AA, Walsh MC, ụmụ. Fanaroff na Martin's Neonatal-Perinatal Medicine: Ọrịa nke Nwa Fetus na Nwa Ọhụrụ. Nke 11. Philadelphia, PA: Elsevier; 2020: isi nke 93.