ʻAʻole anei e pane pono ana kou kino i ka Parathyroid Hormone? E kamaʻilio kākou e pili ana i kēia kūlana ʻē (Pseudohypoparathyroidism)!

Ke manaʻo nei ʻoe i kekahi manawa e hana pono ʻole ana kekahi mea i loko o kou kino? Aia kekahi mau maʻi ʻē, hiki ke hopohopo iki nā inoa. ʻO kekahi maʻi i lohe pinepine ʻole ʻia, akā he mea nui e ʻike e pili ana, ʻo ia ka Pseudohypoparathyroidism (PHP). ʻOiai paha he lōʻihi iki ka inoa, e mālama kākou i ka maʻalahi.

He aha ka pseudohypoparathyroidism?

I ka ʻōlelo maʻalahi, he maʻi genetic kakaikahi kēia. Loaʻa iā mākou nā glands liʻiliʻi i loko o ko mākou kino i kapa ʻia ʻo parathyroid glands. Hoʻopuka kēia i kahi hormone i kapa ʻia ʻo Parathyroid Hormone (PTH). He mea nui loa kēia hormone PTH no ko mākou kino. No ka mea, kōkua kēia hormone i ka mālama ʻana i nā pae kūpono o ka Calcium, Phosphorus, a me ka Vitamin D i loko o ko mākou koko.

I kēia manawa, ua ʻike anei ʻoe i ka mea e hana ʻia i kahi kanaka me ka pseudohypoparathyroidism (PHP)? ʻOiai ʻo kā lākou mau kelepa parathyroid e hana maʻamau i ka hormone PTH, ʻaʻole pane pono nā cell i loko o ko lākou kino iā ia, ʻo ia hoʻi, ʻaʻole hiki iā lākou ke "ʻike" iā ia. Ua like ia me kekahi e kamaʻilio ana me ʻoe akā ʻaʻole hiki iā ʻoe ke lohe iā lākou. Kāhea mākou i kēia he Parathyroid Hormone Resistance .

He aha ka hopena? E emi ana ka nui o ka calcium i loko o ke koko, ʻoiai e piʻi ana ka nui o ka phosphorus. Hiki i kēia kaulike ʻole ke hoʻoulu i ka ʻūlū ʻana o nā ʻiʻo, ka mae ʻana o ka ʻiʻo, ka hopu ʻana i nā ʻiʻo, a me nā pilikia niho.

ʻO ka mea nui, ʻokoʻa kēia kūlana i kapa ʻia ʻo PHP mai kahi kūlana i kapa ʻia ʻo Hypoparathyroidism. ʻAʻole hana ka poʻe me ka hypoparathyroidism i ka hormone PTH lawa i ka hoʻomaka ʻana. Eia nō naʻe, i ka PHP, hana ʻia ka hormone, akā ʻaʻole hoʻohana ke kino.

He aha nā ʻano nui o PHP?

Aia kekahi mau ʻano nui o kēia kūlana PHP. E nānā kākou i ke ʻano o lākou.

ʻAno PHP-1

ʻO kēia ke ʻano maʻamau o PHP . Ma waho aʻe o ke kū'ē ʻana o ka hormone PTH i ʻōlelo mua ʻia, hiki ke ʻike ʻia kekahi mau loli o ka ʻōnaehana iwi. Eia kekahi laʻana:

• Ka poepoe o ka maka
• Ua emi ke kiʻekiʻe (pōkole o ke kino)
• ʻEha ʻāʻī
• Nā haki o nā lima a me nā wāwae

Ua kapa nā kauka i kēia hui pū ʻana o nā hōʻailona ʻo Albright Hereditary Osteodystrophy (AHO) . Loaʻa i ka poʻe me PHP-1 ke kūlana ma muli o kahi ʻano like ʻole o kahi gene i kapa ʻia ʻo `GNAS` a lākou i hoʻoilina ai mai kekahi o ko lākou mau mākua.

PPHP (Pseudoppseudohypoparathyroidism)

He ʻano palena kēia o PHP-1. Loaʻa paha i ka poʻe me PPHP (Pseudopseudohypoparathyroidism) nā hiʻohiʻona kino i ʻike ʻia ma AHO (e like me ka helehelena poepoe a me ke kino pōkole), akā ʻaʻohe o lākou kū'ē i ka hormone PTH . ʻO ia hoʻi, he maʻamau ko lākou pae calcium a me phosphorus.

ʻAno PHP-2

Loaʻa i ka poʻe me PHP-2 ke kū'ē i ka hormone PTH, ʻo ia hoʻi, he haʻahaʻa ko lākou calcium a he kiʻekiʻe ka phosphorus. Akā, ʻaʻohe o lākou mau hiʻohiʻona kino (e like me ka helehelena poepoe a me ke kino pōkole) e like me ka mea i ʻike ʻia ma AHO. ʻAʻole i ʻike pono nā kānaka noiʻi i ke ʻano o ka hemahema genetic e hoʻokumu ai i kēia ʻano PHP-2.

He aha nā hōʻailona o PHP?

Hiki ke ʻokoʻa nā hōʻailona o PHP mai kēlā kanaka a kēia kanaka . ʻO ke ʻano kēia, ʻaʻole e ʻike nā kānaka a pau i nā hōʻailona like. ʻO kekahi mau hōʻailona maʻamau:

• Nā ʻūmiʻi ʻiʻo
• ʻO ka ʻeha pinepine o nā ʻiʻo : He manaʻo e like me ka huki ʻia ʻana o kahi wāwae.
• ʻO ka mae a i ʻole ke kani ʻana o nā lālā
• Nā hopu ʻana
• Ka lohi o ka ulu ʻana o nā niho
• Ka nāwaliwali o ka enamel niho
• Ke koko haʻahaʻa
• Lauoho palupalu a me nā kui
• Hopohopo
• Kaumaha

Hiki i nā poʻe me PHP-1 ke ʻike i nā hōʻailona hou aʻe e hele pū me ke kūlana Albright hereditary osteodystrophy (AHO) i ʻōlelo mua ʻia. Hiki i kekahi poʻe me PHP ke ʻike i kēia mau hōʻailona:

• Ka poepoe o ka maka
• Kino pōkole (kino pōkole)
• ʻEha ʻāʻī
• Nā iwi ma nā lima a me nā wāwae : ʻO nā puʻupuʻu lima, ʻoiai nā mea o nā manamana lima ʻehā a me ka ʻelima, me he mea lā ua huli ʻia i loko.
• Momona : ʻO ia hoʻi ke kaumaha nui.
• Nā hemahema o ka ʻike : nā mea e like me ke cataracts, ka ʻike pōwehiwehi, a me ka ʻike i ka mālamalama.
• Nā ʻōpū iwi ma lalo o kou ʻili
• Nā lohi o ka ulu ʻana : Hiki i kekahi mau keiki ke loaʻa i kekahi mau lohi o ka ulu ʻana.

He aha nā kumu o PHP?

ʻO ke kumu nui o kēia maʻi, ʻo ia nā ʻokoʻa o kā mākou mau genes, ʻoi aku hoʻi i ka gene i kapa ʻia ʻo 'GNAS' . Hoʻoilina kekahi poʻe mai ko lākou mau mākua mai . ʻO ia hoʻi, he hoʻoilina ia. Akā i kekahi manawa hiki ke hana ʻia kēia ʻano me ka ʻole o ka mōʻaukala ʻohana a ʻaʻohe kumu e loaʻa.

Hoʻomaopopo pinepine ʻia ka PHP i ka wā kamaliʻi a i ʻole ke kamaliʻi ʻana , akā hiki ke ʻike ʻia i kekahi manawa i ka wā makua.

He aha nā pilikia e hiki ke loaʻa mai ka PHP?

Inā loaʻa iā ʻoe ka PHP, ʻoi aku ka nui o kou loaʻa ʻana i nā maʻi ʻē aʻe o ka ʻōnaehana endocrine . Hiki i kēia mau pilikia e pili ana i ka hormone ke hana i nā hōʻailona e like me ka ikehu haʻahaʻa a me ka makemake moekolohe haʻahaʻa.

ʻOi aku ka nui o ka pilikia o kekahi poʻe me PHP i ka hoʻomohala ʻana i nā kūlana e like me:

• ʻO ka maʻi ParkinsonKe hōʻike nei i nā hōʻailona like me ka maʻi o Parkinson.
• Paʻakikī me nā mākau hana maikaʻi : ʻO ke ʻano kēia o ka paʻakikī me nā hana maikaʻi e like me ke pihi ʻana a i ʻole ke kākau ʻana.
• ʻO ka Carpal Tunnel Syndrome : ʻOi loa aku no ka poʻe me PHP-1. He kūlana kēia kahi e ʻomi ʻia ai kahi aʻalolo ma ka pulima lima, e hoʻoulu ai i ka maneʻo a me ka ʻeha ma ka lima.
• ʻO ke kuʻi ʻana o ka iwi kuamoʻo
• ʻAneʻane hiamoe : ʻOi loa aku hoʻi i nā keiki.

Pehea e ʻike ai nā kauka i ka PHP?

Inā kānalua ʻoe he PHP kou, e hana mua kāu kauka i kahi hoʻokolokolo kino . E nīnau pū lākou e pili ana i kou mōʻaukala ʻohana . A laila hiki iā lākou ke hana i kekahi mau hoʻokolohua ʻē aʻe e hōʻoia i ka hōʻailona. ʻO kēia mau mea:

• Nā hoʻāʻo koko : E ana i nā pae hormone, ʻoiai hoʻi ka calcium, phosphorus, a me nā pae PTH.
• Nā hoʻāʻo mimi : E nānā i nā pae o ka calcium a me ka phosphorus i loko o ka mimi.
• Ka hoʻāʻo ʻana i ka hoʻoilina : E nānā i nā loli i loko o ka gene `GNAS`.
• Electrocardiogram (EKG) : Nānā i nā ʻano ʻē i ka puʻuwai.
• Nā kukuna X-ray lima : E nānā i nā iwi haki a me nā loli ʻē aʻe.
• CT Scan (CT - Computed Tomography scan) : No ka ʻike ʻana inā he mau loli i loko o ka lolo.

Ma muli o ka ʻike i loaʻa mai kēia mau hoʻokolohua hiki i ke kauka ke hōʻoia pololei i ke kūlana PHP.

Pehea e mālama ʻia ai ʻo PHP?

ʻAʻohe lāʻau lapaʻau no kēia maʻi i kēia manawa . Akā, mai hopohopo. Me ka mālama pono ʻana a me ka hoʻokele ʻana, hiki iā ʻoe ke kaohi i kāu mau hōʻailona a ola i kahi ola maʻamau.

ʻO ka pahuhopu nui o ka mālama ʻana, ʻo ia ka mālama ʻana i nā pae kūpono o ka calcium a me ka phosphorus i loko o kou koko . ʻO ka mālama ʻana i kēia mau pae hiki ke hōʻemi nui i nā hōʻailona. He lāʻau lapaʻau kēia no ke ola holoʻokoʻa .

Hiki i nā koho lapaʻau ke komo pū me:

• Nā mea hoʻohui kalima a me ka wikamina D : ma ke ʻano pila a wai paha.
• Nā kāpule Calcitriol : He lāʻau lapaʻau kēia e hoʻonui ai i ka nui o ka calcium i loko o ke kino.
• ʻO ka inikini Calcitriol
• ʻO ka lāʻau lapaʻau no ka hormone ulu a i ʻole ka lāʻau hoʻololi no ka hormone thyroid : inā pono.
• ʻAi kiʻekiʻe i ka calcium, haʻahaʻa i ka phosphorus : E like me ka ʻōlelo a ke kauka meaʻai.

ʻO ka mea nui, ʻo ia ke hahai pono i nā ʻōlelo aʻoaʻo a ke kauka a hoʻokolohua i ka manawa.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe ka PHP, he mea nui e ʻike pinepine i kāu kauka e nānā i kāu mau pae calcium a me phosphorus . E kōkua kēia nānā mau ʻana i ka mālama ʻana i nā mea āpau ma lalo o ka mana.

Inā loaʻa iā ʻoe nā hōʻailona koʻikoʻi e like me ka paʻakikī koke o ka hanu ʻana, ka ʻūlū nui o nā ʻiʻo, a i ʻole nā ​​​​​​hōʻailona hopu , e hele koke i ka lumi pilikia haukapila kokoke loa a kāhea aku i ka 1990.

He aha kāu e manaʻo ai i ka wā e noho ai me ke kūlana PHP?

Inā loaʻa iā ʻoe ka PHP, pono ʻoe e hoʻomau i ka hana me kāu Kauka Mālama Ola Kino . No ka mea hiki i ka PHP ke hoʻopilikia i nā ʻāpana like ʻole o ke kino, pono paha ʻoe e ʻike i kekahi mau loea e hoʻokele i kāu mau hōʻailona kikoʻī. Hiki i kāu hui lapaʻau ke komo pū me:

• Endocrinologists : Nā loea i nā hormones.
• Nā kauka ʻoki kino : No nā pilikia e pili ana i ka iwi.
• Nā Kauka Lapaʻau : Inā he keiki.
• Nā Neurologists : No nā pilikia e pili ana i ka ʻōnaehana nerve.
• Nā mea hoʻōla kino
• Nā mea lapaʻau hana
• Nā mea lapaʻau meaʻai
• Nā kauka niho
• Nā kānaka ʻepekema genetics
• Nā loea hoʻokele ʻeha

Pono paha ʻoe e lawe i kēlā mau mea hoʻohui no ke koena o kou ola .

Hiki ke ʻokoʻa loa nā hiki ke ulu a me ka noʻonoʻo ma muli o ke ʻano a me ke koʻikoʻi o ka maʻi. Eia nō naʻe , i ka hapanui o nā hihia, he ola maʻamau ko ka poʻe me PHP i hoʻohālikelike ʻia me ka poʻe me ka ʻole o PHP . Hiki i kāu kauka ke haʻi iā ʻoe i ka mea e manaʻo ai ma muli o kou kūlana.

No ka mea, he maʻi laha ʻole ka PHP, hiki ke paʻakikī ke loaʻa i nā poʻe ʻē aʻe i loaʻa i ke ʻano like me ʻoe . Hiki nō hoʻi ke manaʻo mehameha i kekahi manawa. Inā ʻike ʻia ʻoe a i ʻole kāu keiki me PHP, e kamaʻilio me kāu kauka e pili ana i nā hui kākoʻo a me nā kumuwaiwai hoʻonaʻauao . Hiki i kēia mau mea ke kōkua iā ʻoe e ʻike i nā ala e hoʻomaikaʻi ai i kāu mau hōʻailona a me ke ʻano holoʻokoʻa o ke ola.

Leka Lawe-Home

ʻAe, e nānā kākou i kekahi mau mea e hoʻomanaʻo ai e pili ana i ka pseudohypoparathyroidism (PHP) a mākou i kamaʻilio ai.

• He ʻano laha ʻole ka PHP kahi e pane pono ʻole ai ke kino i ka hormone parathyroid (PTH).
• ʻO kēia ke kumu o ka emi ʻana o ka calcium i loko o ke koko a me ka piʻi ʻana o ka phosphorus.
• Aia kekahi mau ʻano nui : PHP-1, PHP-2, a me PHP-3.ʻAe. Hiki iā PHP-1 ke hana i nā loli i nā iwi i kapa ʻia ʻo AHO.
• Hiki ke ʻike ʻia nā ʻano hōʻailona like ʻole, e like me ka ʻūlū ʻana o nā ʻiʻo, ka maneʻo, ka huʻi ʻana, nā pilikia niho, ke kino pōkole, a me ka helehelena poepoe .
• ʻO ke kumu nui, he mutation i loko o ka gene 'GNAS' .
• ʻOiai ʻaʻohe lāʻau lapaʻau piha, hiki ke hoʻomalu ʻia nā hōʻailona me ka lāʻau lapaʻau no ke ola holoʻokoʻa (calcium, vitamin D, kahi papaʻai kūikawā).
• He mea nui nā hoʻokolohua lapaʻau maʻamau a me ke kākoʻo o nā kauka loea.
• Hiki i ka hapa nui o nā kānaka ke ola i kahi ola maʻamau .
• He mea nui loa ke kōkua mai nā hui kākoʻo no ka noho ikaika ʻana o ka noʻonoʻo.

Mai poina, ʻaʻole ʻoe hoʻokahi. ʻOiai he maʻi kakaikahi kēia, hiki ke mālama maikaʻi ʻia me ka ʻōlelo aʻoaʻo lapaʻau kūpono a me ke kākoʻo. E hoʻomanaʻo i nā kauka, ka ʻohana, a me nā hoaaloha e kū nei no ʻoe ke pono ʻoe i ka ikaika.

ʻO ka Pseudohypoparathyroidism, PHP, ka hormone parathyroid, calcium, phosphorus, nā maʻi genetic, AHO