ʻO ka maʻi nephrotic maʻi

ʻO ka maʻi nephrotic maʻi kūpuna kahi maʻi i hoʻoili ʻia i waena o nā ʻohana kahi e hoʻomohala ai kahi pēpē i ka mimi a me ka pehu o ke kino.

ʻO ka maʻi nephrotic Synenital kahi maʻi genes recessive autosomal. Kēia mea pono kēlā me kēia mākua e hoʻoili i kahi kope o ka ʻaoʻao hemahema i mea e loaʻa ai ka maʻi i ke keiki.

ʻOiai ke ʻano o ka hānau hānau mai ka wā hānau, me ka maʻi nephrotic congenital, kū nā ʻōuli o ka maʻi i nā mahina 3 mua o ke ola.

ʻO ka maʻi nephrotic Synenital kahi ʻano liʻiliʻi loa o ka nephrotic syndrome.

ʻO ka maʻi Nephrotic kahi hui o nā ʻōuli e pili pū me:

• Protein i ka mimi
• Nā pae haʻahaʻa protein haʻahaʻa i loko o ke koko
• Nā pae kolesterol kiʻekiʻe
• Nā pae triglyceride kiʻekiʻe
• ʻŌhū

Loaʻa i nā kamaliʻi me kēia maʻi i kahi ʻano maikaʻi ʻole o kahi protein i kapa ʻia nephrin. Pono nā kānana o ka hakuʻala (glomeruli) i kēia protein e hana maʻamau.

ʻO nā ʻōuli o nephrotic syndrome me:

• Cough
• Hoʻoemi i ka puka mimi
• ʻAno ka mimi
• Kaumaha hānau haʻahaʻa
• Makemake maikaʻi ʻole
• Pehu (kino holoʻokoʻa)

Hiki i kahi ultrasound i hana ʻia ma ka makuahine hāpai ke hōʻike i kahi placenta ʻoi aku ka nui ma mua o ka maʻamau. ʻO ka placenta kahi lālā e hoʻomohala i ka wā hāpai e hānai i ka pēpē ulu.

Loaʻa paha i nā makuahine hāpai i kahi hōʻike kānana i hana ʻia i ka wā o ka hāpai ʻana e nānā no kēia ʻano. Nānā ka hōʻike no nā pae kiʻekiʻe ma mua o ka maʻamau o alpha-fetoprotein i kahi laʻana o ka wai amniotic. Hoʻohana ʻia nā hōʻike genetic e hōʻoia i ka hōʻoia inā maikaʻi ka hōʻike kānana.

Ma hope o ka hānau ʻana, e hōʻike ka pēpē i nā hōʻailona o ka paʻa ʻana o ka wai a me ka pehu. E lohe ka mea mālama ola i nā kani maʻamau ke hoʻolohe nei i ka puʻuwai a me nā māmā o ka pēpē me kahi stethoscope. Kiʻekiʻena ke koko. Maliʻa paha he hōʻailona o ka hōʻino kino.

Hōʻike kahi urinalysis i ka momona a me nā nui o nā protein i ka mimi. Hiki i ka huina protein i ke koko ke haʻahaʻa.

Pono ka mālama mua a me ka hakakā e kāohi i kēia maʻi.

Hoʻopili paha ka mālama ʻana:

• Nā lāʻau Antibiotics e kaohi i nā maʻi
• ʻO nā lāʻau kahe koko i kapa ʻia ʻo angiotensin-hoʻololi i ka enzyme (ACE) mea kāohi a me nā angiotensin receptor blockers (ARBs) e hōʻemi i ka nui o ke kahe ʻana o ka protein i ka mimi.
• Diuretics ("wai huaʻai") e hoʻopau i ka nui o ka wai
• Nā NSAID, e like me ka indomethacin, e hōʻemi i ka nui o nā pūmua e hemo i ka mimi

Pākuʻi ʻia paha nā wai e kōkua ai i ka mālama ʻana i ka pehu.

Paipai paha ka mea hāʻawi i ka lawe ʻana i nā puʻupaʻa e hoʻōki i ka nalo ʻana o ka protein. E ukali paha kēia ma dialysis a i ʻole ke kuʻina kōkō.

ʻO ka maʻi e alakaʻi pinepine ai i ka maʻi, malnutrisi, a me nā kōpaʻa ʻole. Hiki iā ia ke alakaʻi i ka make i ka makahiki 5, a make nā keiki he nui i loko o ka makahiki mua. Hiki ke kāohi ʻia ka maʻi nephrotic maʻi i kekahi mau hihia me ka hoʻomaʻamaʻa mua a me ka hakakā, e like me ka transplant kidney mua.

Nā pilikia o kēia ʻano:

• Hewa kōwi koʻikoʻi
• Huʻi koko
• Ka hōʻeha kīnā ʻole
• ʻO ka maʻi hōʻeha hope loa
• ʻO nā maʻi maʻi pinepine
• Malnutrisi a me nā maʻi e pili ana

Kāhea i kāu mea hāʻawi inā loaʻa kāu keiki i nā ʻōuli o ka maʻi nephrotic maʻi.

Nephrotic maʻi pale - hānau hānau

• ʻĀpana mimi wahine
• ʻĀpana mimi kāne

ʻO Erkan E. Nephrotic maʻi maʻi. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 545.

Schlöndorff J, Pollak MR. Nā maʻi i hoʻoili ʻia i ka glomerulus. I: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. ʻO Brenner a me Rector's The Kidney. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 43.

ʻO Vogt BA, ʻo Springel T. ʻO ke kīʻaha a me nā wahi urinary o ka neonate. I: Martin RJ, Fanaroff AA, Walsh MC, hua. ʻO Fanaroff lāua ʻo Martin's Neonatal-Perinatal Medicine: Nā maʻi o ka Fetus a me ka pēpē. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 93.