ʻO ka insipidus diabetes nephrogenic

ʻO ka nephrogenic diabetes insipidus (NDI) kahi maʻi kahi maʻi kīnā ma nā paipu liʻiliʻi (tubules) i nā puʻupaʻa e hele ai ke kanaka i ka nui o ka mimi a lilo ka nui o ka wai.

ʻO ka maʻamau, ʻae nā puʻupaʻa puʻupaʻa i ka nui o ka wai i ke koko e kānana a hoʻihoʻi ʻia i ke koko.

Hana ʻia ʻo NDI ke pane ʻole ka tubula kōpū i kahi hormone i loko o ke kino i kapa ʻia he antidiuretic hormone (ADH), i kapa ʻia hoʻi ʻo vasopressin Hana pinepine ʻo ADH i nā puʻupaʻa e hoʻonui i ka mimi.

Ma muli o ka pane ʻole ʻana i ka hōʻailona ADH, hoʻokuʻu nui nā puʻupaʻa i ka wai i ka mimi. Hoʻokumu kēia i ke kino e hana i kahi nui nui o ka mimi dilute loa.

Kākaʻikahi loa ʻo NDI. Aia ka pēpē nephrogenic diabetes insipidus i ka wā hānau. He hopena ia o kahi kīnā i hoʻoili ʻia i waena o nā ʻohana. Hoʻopili pinepine ʻia nā kāne, ʻoiai hiki i nā wahine ke hoʻoili i kēia ʻāpana i kā lākou keiki.

ʻO ka maʻamau, hoʻomohala ʻo NDI ma muli o nā kumu ʻē aʻe. Kapa ʻia kēia he maʻi loaʻa. Nā kumu i hiki ke hoʻoulu i ke ʻano i loaʻa o kēia ʻano me:

• ʻO ka ālai ʻana i ka maʻi urinary
• Nā pae calcium kiʻekiʻe
• Nā pae potassium haʻahaʻa
• Hoʻohana o kekahi mau lāʻau (lithium, demeclocycline, amphotericin B)

Loaʻa paha ʻoe i ka make wai ikaika a i ʻole kaohi ʻole, a ʻiʻini i ka wai hau.

E hoʻopuka ʻoe i ka nui o ka mimi, ma mua o 3 liters, a hiki i 15 liters i kēlā me kēia lā. Pelu loa ka mimi a ʻaneʻane like me ka wai. Pono paha ʻoe e mimi i kēlā me kēia hola a ʻoi aku paha, ʻoiai i ka pō ke ʻai ʻole ʻoe a inu ʻole paha.

Inā ʻaʻole ʻoe e inu lawa i nā wai, hiki i ka make wai ke hopena. Pākuʻi ʻia paha nā ʻōuli:

• Nā membrane mucous maloʻo
• ʻIli maloʻo
• ʻIke ʻia e nā maka
• Sunken fontanelles (kahi palupalu) i nā pēpē
• Hoʻololi i ka hoʻomanaʻo a me ke kaulike

Nā ʻōuli ʻē aʻe i hiki ke hana ʻia ma muli o ka nele o nā wai, e hoʻomaka ai ka make wai, e hoʻopili pū me:

• Ka luhi, nāwaliwali
• ʻEha poʻo
• Huhū
• Mahana haʻahaʻa kino
• ʻEha ʻeha
• Helu puʻuwai wikiwiki
• Kaumaha emi
• Hoʻololi i ka makaʻala, a me ke koma nō hoʻi

E nānā ka mea mālama ola iā ʻoe a nīnau e pili ana i nā hōʻailona o kāu keiki a kāu keiki paha.

Hōʻike kahi hoʻokolohua kino:

• Kaomi haʻahaʻa
• Pākuʻi wikiwiki
• Pāhaʻi
• Nā hōʻailona o ka make wai

Hōʻike ʻia nā hoʻokolohua:

• Osmolality serum kiʻekiʻe
• Hoʻopuka i ka mimi kiʻekiʻe, me ka nānā ʻole i ka wai āu e inu ai
• ʻAʻole noʻonoʻo nā hakuʻala i ka mimi ke hāʻawi ʻia iā ʻoe ADH (ʻo ka maʻa mau ka lāʻau i kapa ʻia desmopressin)
• Osmolality mimi haʻahaʻa
• Nā pae ADH maʻamau a kiʻekiʻe paha

Nā hoʻokolohua ʻē aʻe i hana ʻia e hoʻopili ʻia:

• Hōʻike koko Sodium
• Urine ka leo 24-hola
• Hōʻoia i ka mimi mimiki
• Umekaumaha kikoʻī
• ʻO ka hoʻāʻo hoʻowahāwahā wai mālama ʻia
  

ʻO ka pahuhopu o ka mālama ʻana ka kaohi ʻana i nā pae kahe o ke kino. E hāʻawi ʻia ka nui o nā wai. E like paha ka nui me ka nui o ka wai i nalowale i ka mimi.

Inā pili ke ʻano i kekahi lāʻau, ke kāpae ʻana i ka lāʻau e hoʻomaikaʻi paha i nā ʻōuli. Akā, mai hoʻopau i ka lawe ʻana i nā lāʻau me ka ʻole e kamaʻilio mua i kāu mea hāʻawi.

Hāʻawi ʻia nā lāʻau e hoʻomaikaʻi i nā hōʻailona ma o ka hōʻemi ʻana i ka puka mimi.

Inā inu ke kanaka i ka wai lawa, ʻaʻole e loaʻa kēia hopena i ka hopena o ke kahe a electrolyte paha o ke kino. I kekahi manawa, hala ka nui o ka mimi no ka manawa lōʻihi hiki ke hana i nā pilikia electrolyte ʻē aʻe.

Inā ʻaʻole inu ke kanaka i nā wai i lawa, hiki i ka puka mimi kiʻekiʻe ke kumu o ka make wai a me nā kiʻekiʻe o ka sodium i loko o ke koko.

ʻO NDI e kū nei i ka wā hānau kahi kūlana lōʻihi e koi ana i ka mālama ola āpau.

Hana ʻole ʻia, hiki iā NDI ke hana i kekahi o kēia mau mea:

• Hoʻonui o ka ureter a me ka bladder
• ʻO ke koko kiʻekiʻe sodium (hypernatremia)
• Ka make nui loa ʻana
• Pāhaʻi
• Koma

Kāhea i kāu mea hāʻawi inā loaʻa a maʻi paha kāu keiki o kēia maʻi.

ʻAʻole hiki ke pale ʻia ka NDI kūlohelohe.

ʻO ka mālama ʻana i nā maʻi i hiki ke alakaʻi i ke ʻano i loaʻa i ke ʻano e kāohi iā ia mai ka hoʻomohala ʻana i kekahi mau hihia.

Neipidus diabetes nephrogenic; Loaʻa insipidus diabetes nephrogenic loaʻa; Congenital nephrogenic diabetes insipidus; NDI

• ʻŌnaehana mimi kāne

ʻO Bockenhauer D. Fluid, electrolyte, a me nā maʻi acid-base i nā keiki. 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 73.

ʻO Breault DT, ʻo Majzoub JA. Insipidus diabetes. 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 574.

ʻO Hannon MJ, ʻo Thompson CJ. ʻO Vasopressin, diabetes insipidus, a me ka maʻi o ka antidiuresis kūpono ʻole. I: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Makua a Pediatric. ʻEpekema 7 Piladelapia, PA: Elsevier Saunders; 2016: chap 18.

ʻO Scheinman SJ. Nā maʻi maʻi hōʻoki ʻōiwi. I: Gilbert SJ, Weiner DE, eds. ʻO ka National Primer Foundation ma ka maʻi maʻi maʻi. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 38.