Isifo se-nephrotic syndrome

I-congenital nephrotic syndrome sisifo esidluliswa kwiintsapho apho umntwana avelisa khona iprotein kumchamo kunye nokudumba komzimba.

Isifo se-congenital nephrotic syndrome sisifo se-autosomal recessive genetic disorder. Oku kuthetha ukuba umzali ngamnye kufuneka adlulise ikopi yesifo esingafezekanga ukuze umntwana abe nesifo.

Nangona ukuzalwa kuthetha ukubakho kwasekuzalweni, kunye nephrotic yesifo sokuzalwa, iimpawu zesi sifo zenzeka kwiinyanga ezintathu zokuqala zobomi.

Isifo se-congenital nephrotic syndrome luhlobo olunqabileyo lwe-nephrotic syndrome.

I-Nephrotic syndrome liqela leempawu ezibandakanya:

• Iiprotheni kumchamo
• Amanqanaba ephrotheni egazi asezantsi egazini
• Amanqanaba aphezulu e-cholesterol
• Amanqanaba aphezulu e-triglyceride
• Ukudumba

Abantwana abanale ngxaki banefomathi engaqhelekanga ebizwa ngokuba yi-nephrin. Iifilitha zezintso (glomeruli) zidinga ukuba le protein isebenze ngesiqhelo.

Iimpawu ze-nephrotic syndrome zibandakanya:

• Khohlela
• Ukuncipha kwemveliso yomchamo
• Ukubonakala kwegwebu komchamo
• Ubunzima bokuzalwa obuphantsi
• Ukutya okungafanelekanga
• Ukudumba (umzimba uphela)

I-ultrasound eyenziwe kumama okhulelweyo ingabonisa i-placenta enkulu kuneyona eqhelekileyo. I-placenta lilungu elikhula ngexesha lokukhulelwa ukondla umntwana okhulayo.

Oomama abakhulelweyo banokuvavanywa ukuhlolwa ngexesha lokukhulelwa ukujonga le meko. Uvavanyo lujonga amanqanaba aphezulu-kunesiqhelo e-alpha-fetoprotein kwisampulu ye-amniotic fluid. Iimvavanyo ze-Genetic zisetyenziselwa ukuqinisekisa ukuxilongwa ukuba ngaba uvavanyo lovavanyo lukhona.

Emva kokuzalwa, usana luya kubonisa iimpawu zokugcinwa kwamanzi kunye nokuvuvukala. Umboneleli wezempilo uya kuva izandi ezingaqhelekanga xa emamele intliziyo yomntwana kunye nemiphunga ene-stethoscope. Uxinzelelo lwegazi lunokuba phezulu. Kusenokubakho iimpawu zokungondleki.

Uvavanyo lokuchama lutyhila amanqatha kunye nezixa ezikhulu zeprotheyini kumchamo. Iiproteni zizonke egazini zinokuba sezantsi.

Unyango kwangoko nangolunya luyafuneka ukulawula oku kuphazamiseka.

Unyango lunokubandakanya:

• Amayeza okubulala iintsholongwane ukulawula izifo
• Amayeza oxinzelelo lwegazi abizwa ngokuba yi-angiotensin-converting enzyme (ACE) inhibitors kunye ne-angiotensin receptor blockers (ARBs) ukunciphisa isixa seprotein evuzayo kumchamo
• I-diuretics ("iipilisi zamanzi") ukususa ulwelo oluninzi
• Ii-NSAID, ezinjenge-indomethacin, ukunciphisa isixa seprotein evuzayo kumchamo

Ulwelo lunokulinganiselwa ukunceda ukulawula ukudumba.

Umboneleli unokucebisa ukuba asuse izintso ukuze ayeke ukulahleka kweprotein. Oku kunokulandelwa yi-dialysis okanye ukufakelwa kwezintso.

Esi sifo sihlala sikhokelela kusulelo, ukungondleki kunye nokusilela kwezintso. Ingakhokelela ekufeni xa uneminyaka emi-5, kwaye uninzi lwabantwana lusweleka kunyaka wokuqala. I-congenital nephrotic syndrome inokulawulwa kwezinye iimeko ngonyango lwangoko nangoko, kubandakanya nokufakelwa kwezintso kwangoko.

Iingxaki zale meko zibandakanya:

• Ukungaphumeleli kwezintso
• Amahlwili egazi
• Ukusilela kwezintso okungapheliyo
• Isifo sokugqibela sezintso
• Rhoqo, izifo ezosulelayo
• Ukungondleki kunye nezifo ezinxulumene noko

Fowunela umnikezeli wakho ukuba umntwana wakho uneempawu zesifo sokuzalwa esinganyangekiyo.

Nephrotic syndrome - lokuzalwa

• Iphecana lomchamo labasetyhini
• Indoda yokuchama

Isifo sika-Erkan E. Nephrotic. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahlulo 545.

USchlöndorff J, uPollak MR. Ukuphazamiseka okwabangelwa lilifa le-glomerulus. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ii-eds. Brenner kunye noMphathi weZintso. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 43.

IVogt BA, Springel T.Izintso kunye nomchamo womchamo we-neonate. Ku: UMartin RJ, uFanaroff AA, uWalsh MC, ii-eds. UFanaroff kunye noMartin's Neonatal-Perinatal Medicine: Izifo ze-Fetus kunye neentsana. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2020: isahl. 93.