ʻO Bartter Syndrome

ʻO Bartter Syndrome kahi hui o nā kūlana laha ʻole e hoʻopili i nā puʻupaʻa.

ʻElima mau kīnā ʻili i ʻike ʻia e pili pū me ka maʻi ʻo Bartter. Aia ke ʻano i ka wā hānau (congenital).

Hoʻokumu ʻia ke ʻano e kahi kīnā i ka hiki i nā puʻupaʻa ke hiki hou i ka sodium. ʻO ka poʻe i hoʻopilikia ʻia e Bartter Syndrome lilo ka nui o ka sodium ma o ka mimi. ʻO kēia ke kumu o ka piʻi ʻana o ka pae o ka hormone aldolone, a hoʻoneʻe ka puʻupaʻa i ka nui o ka pāhare potassium mai ke kino. ʻIke ʻia kēia me ka hoʻonele ʻana i ka potassium.

ʻO ke kumu i hopena i kahi kaulike acid acid i loko o ke koko i kapa ʻia ʻo hypokalemic alkalosis, ka mea e nui ai ka calcium i ka mimi.

Kū pinepine kēia maʻi i ka wā kamaliʻi. ʻO nā hōʻailona me:

• Konipōpō
• ʻOi aku ka haʻahaʻa o ka loaʻa o ke kaumaha ma mua o nā keiki ʻē aʻe o ke ʻano o ka makahiki a me ka wahine (ulu ʻole)
• Pono e mimi hou pinepine ma mua o mau (urinary frequency)
• Kaomi haʻahaʻa
• Pohaku hakuʻala
• Hoʻopaʻi i ka mākala a nāwaliwali hoʻi

Manaʻo pinepine ʻia ʻo Bartter Syndrome ke loaʻa kahi hōʻike haʻahaʻa i kahi pae haʻahaʻa o ka potassium i loko o ke koko. ʻAʻole like me nā ʻano maʻi maʻi ʻē aʻe, ʻaʻole kumu kēia kiʻekiʻe i ke koko kiʻekiʻe. Aia kahi maʻamau i ke kahe haʻahaʻa. Hōʻike paha nā hōʻike Laboratory:

• Nā pae kiʻekiʻe o ka potassium, calcium, a me chloride i ka mimi
• Nā pae kiʻekiʻe o nā hormoni, renin a me aldolone, i loko o ke koko
• Klorida koko haʻahaʻa
• Alkalosis Metabolic

Hiki i kēia mau hōʻailona like a me nā ʻōuli ke hana i ka poʻe i lawe i nā diuretics he nui (nā pila wai) a i ʻole nā ​​laxatives. Hiki ke hana i nā hoʻokolohua mimi e kāpae i nā kumu ʻē aʻe.

Hiki ke hana ʻia kahi ultrasound o nā puʻupaʻa.

Mālama ʻia ʻo Bartter Syndrome e ka ʻai ʻana i nā meaʻai i loko o ka potassium a i ʻole ka lawe ʻana i nā pāʻina potassium.

Pono nā poʻe he nui i ka paʻakai paʻakai a me ka magnesium.Pono paha ka lāʻau lapaʻau e ālai i ka hiki i ka hakuʻala e hoʻopau i ka pāhare potassium. Hiki ke hoʻohana ʻia nā lāʻau kiʻekiʻe o nā lāʻau anti-inflammatory nonsteroidal (NSAIDs).

ʻO nā pēpē i loaʻa i ka ulu nui ʻole e ulu maʻamau me ka lāʻau. Ma ka hala ʻana o ka manawa, hoʻomohala kekahi poʻe me ka maʻi

Kāhea i kāu kahu mālama ola inā ʻo kāu keiki:

• Loaʻa i nā puʻupuʻu puʻupuʻu
• Ke ulu maikaʻi ʻole nei
• Urencing pinepine

Lūlū potasiuma; Nephropathy hoʻowahāwahā paʻakai

• ʻO ka hoʻāʻo pae Aldolone

ʻO Dixon BP. I hoʻoili ʻia i nā pono halihali tubular hoʻoilina: Bartter syndrome. 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 549.1

ʻO Guay-Woodford LM. Nā nephropathies hoʻoilina a me nā hanana kūpono ʻole o ke kahe urinary. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 119.

Mauna DB. Nā maʻi o ke kaulike potassium. 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 17.