Isifo sokuzalwa esine-nephrotic

I-Congenital nephrotic syndrome yisifo esidluliswa ngemindeni lapho ingane iba namaprotheni emchameni nasekuvuvukeni komzimba.

I-Congenital nephrotic syndrome yisifo se-autosomal recessive genetic disorder. Lokhu kusho ukuthi umzali ngamunye kufanele adlulise ikhophi yesakhi sofuzo esingalungile ukuze ingane ithole lesi sifo.

Yize izindlela zokuzalwa ezizalwa zikhona kusukela ekuzalweni, ezine-nephrotic syndrome ezelwe, izimpawu zalesi sifo zenzeka ezinyangeni ezintathu zokuqala zokuphila.

I-Congenital nephrotic syndrome iyindlela engavamile kakhulu ye-nephrotic syndrome.

I-Nephrotic syndrome yiqembu lezimpawu ezibandakanya:

• Amaprotheni emchameni
• Amaphrotheni egazi aphansi egazini
• Amazinga aphezulu e-cholesterol
• Amazinga aphezulu we-triglyceride
• Ukuvuvukala

Izingane ezinalesi sifo zinefomu elingavamile leprotheyini elibizwa nge-nephrin. Izihlungi zezinso (i-glomeruli) zidinga ukuthi le protein isebenze ngokujwayelekile.

Izimpawu ze-nephrotic syndrome zifaka:

• Khwehlela
• Ukwehla kokukhishwa komchamo
• Ukubukeka kwegwebu lomchamo
• Isisindo sokuzalwa esiphansi
• Ukudla okungekuhle
• Ukuvuvukala (umzimba ophelele)

I-ultrasound eyenziwe kumama okhulelwe ingabonisa i-placenta enkulu kunokujwayelekile. I-placenta isitho esikhula ngesikhathi sokukhulelwa ukondla ingane ekhulayo.

Omama abakhulelwe bangase bahlolwe ukuhlolwa ngesikhathi sokukhulelwa ukuhlola lesi simo. Isivivinyo sibheka amazinga aphezulu kunejwayelekile we-alpha-fetoprotein kusampula ye-amniotic fluid. Ukuhlolwa kofuzo kuyabe sekusetshenziswa ukuqinisekisa ukuxilongwa uma isivivinyo sokuhlola silungile.

Ngemuva kokuzalwa, usana luzokhombisa izimpawu zokugcina uketshezi olunzima nokuvuvukala. Umhlinzeki wezokunakekelwa kwezempilo uzozwa imisindo engajwayelekile lapho elalele inhliziyo namaphaphu engane nge-stethoscope. Umfutho wegazi ungaba phezulu. Kungaba nezimpawu zokungondleki.

Ukuhlolwa komchamo kuveza inani elikhulu lamafutha namaprotheni emchameni. Ingqikithi yamaprotheni egazini ingaba ngaphansi.

Ukwelashwa kwasekuqaleni futhi okunolaka kuyadingeka ukulawula lesi sifo.

Ukwelashwa kungafaka:

• Ama-antibiotic ukulawula izifo
• Imithi yengcindezi yegazi ebizwa nge-angiotensin-converting enzyme (ACE) inhibitors kanye ne-angiotensin receptor blockers (ARBs) ukunciphisa inani lamaprotheni avuza emchameni
• Izidakamizwa ("amaphilisi amanzi") ukususa uketshezi oluningi
• Ama-NSAID, njenge-indomethacin, ukunciphisa inani lamaprotheni avuza emchameni

Uketshezi lungakhawulelwa ukusiza ukuvuvukala.

Umhlinzeki angancoma ukususa izinso ukumisa ukulahleka kwamaprotheni. Lokhu kungalandelwa yi-dialysis noma ukufakelwa izinso.

Lesi sifo sivame ukuholela ekutheleleni, ukungondleki kahle, kanye nokwehluleka kwezinso. Kungaholela ekufeni lapho uneminyaka emihlanu, futhi izingane eziningi zifa kungakapheli unyaka wokuqala. I-Congenital nephrotic syndrome ingahle ilawulwe kwezinye izimo ngokwelashwa kokuqala nangolaka, kufaka phakathi ukufakelwa kwezinso zakuqala.

Izinkinga zalesi simo zifaka:

• Ukungasebenzi kahle kwezinso
• Amahlule egazi
• Ukwehluleka kwezinso okungamahlalakhona
• Isifo sezinso sokugcina
• Njalo, izifo ezinzima
• Ukungondleki kanye nezifo ezihlobene nazo

Shayela umhlinzeki wakho uma ingane yakho inezimpawu ze-congenital nephrotic syndrome.

I-Nephrotic syndrome - izalwa naso

• Umgudu womchamo wabesifazane
• Umgudu womchamo wabesilisa

Isifo i-Erkan E. Nephrotic syndrome. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 545.

USchlöndorff J, uPollak MR. Ukuphazamiseka okuzuzwe njengefa kwe-glomerulus. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ama-eds. IBrenner neRector's The Kidney. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 43.

IVogt BA, Springel T. Izinso nomgudu womchamo wengane. Ku: Martin RJ, Fanaroff AA, Walsh MC, ama-eds. UFanaroff noMartin's Neonatal-Perinatal Medicine: Izifo Zengane Nengane. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 93.