ʻO ka maʻi diabetes Phropate: nā kumu, nā hōʻailona a me nā mālama

I kēia ao, aneane nā mākua āpau e ʻike i kahi maʻi e like me ka rickets. ʻO nā mea pediatrician āpau mai nā mahina mua o ke ola o kahi pēpē e lūlū i ka pono e pale i kēia ʻano kuʻuna. ʻO nā Rickets e pili ana i nā maʻi i loaʻa ma muli o ka nele o ka cholecalciferol, he mea e ʻike ʻia o ka vitamin D.

Eia nō naʻe, i kekahi mau hihia, ʻike ka ʻōpio o nā keiki i nā hōʻailona o ka patology, ʻoiai ka nānā ʻana o nā hana pale. I kēlā mau hihia, pono e lālū ʻia kahi maʻi e like me ka maʻi phosphate. ʻAʻole e like me rickets, aia kēia maʻi ma nā pathology genetic. No laila, he maʻi koʻikoʻi loa ia a koi aku i ka mālama mau loa.

He aha ka maʻi diabetes?

ʻO kēia ka inoa o kahi hui o nā maʻi āpau e pili ana i ka minerism mineral impaired. Ma kahi ala'ē aʻe, ua kapa ʻia ke pathology me nā huaʻō kūkioki-D-rickets. Ke loaʻa nei ka ʻano nui o ka maʻi diabetes phosphate ma kahi o 1 mai 20,000 mau pēpē hānau hou. ʻO ka maʻi mua ua wehewehe mua ʻia ma ka 1937. Ma hope mai ua hoʻohuli ʻia nā ʻano ʻano ʻē aʻe o ka pathology. ʻO ka maʻi diabetes ka puna ma ka wā ʻōpio. Hoʻowalewale ʻia ka nānā ʻana o nā mākua e ka gait impaired a paʻa i nā iwi.

ʻO kekahi mau ʻano o nā patologi ke hele pū me nā maʻi metabolic ʻē aʻe. I kekahi manawa he asymptomatic maʻi, a hiki ke ʻike ʻia e nā hoʻokolohua hoʻoliʻi. Hoʻokaʻawale ʻia ka maʻi me nā rickets i loaʻa, ka hypoparathyroidism a me nā ala āpau endocrine. Pono ka hānai ʻana mai ka wā ʻōpio.

Nā kumu kumu o ka maʻi

ʻO ke kumu nui no ka hoʻomohala ʻana i ka maʻi phosphate he mau hana hōʻeuʻeu i ka ʻano genetic. Ua hoʻoilina ʻia nā Mutations. Hiki i nā ʻūlani o nā gen pathological ke lilo i wahine a me nā kāne. Hōʻike ka mutation i ka chomosome X-pili, kahi mea nui. I nā pilikia liʻiliʻi, hiki ke loaʻa ka maʻi diabetes phosphate. Hoʻoulu ia e kū'ē i kahiʻano o nā hina o ka kiko mesenchymal, ka mea i kūkulu ai i ka manawa prenatal. I kēia hihia, ua kapa ʻia ke alahele "he forcogenic rickets."

Aia ka maʻi i ka hui o tubulopathies - hana i ʻole ke āpau. Ma muli o ka hiki ʻana o ka reabsorption o nā minima i loko o ka tubula proximal, ua ulu ka hala o ka phosphorus i ke kino. Eia kekahi, ua kīpī ka hemahema o ka ʻōpū. A ke hopena, ʻike ʻia nā loli o ka hana o ka mineral mineral o ka iwi o ka iwi. ʻO ka pinepine he halaʻole i ka hana o osteoblasts. Huli wale nā ​​kāne i ke ʻano pathologically i kā lākou mau kaikamāhine, a me nā wāhine - i nā keiki o nā kāne like ʻole. E like me ke kānāwai, ʻoi aku ka nui o ka maʻi o ka maʻi ma mua o nā kaikamahine.

Pilikū o Phabetate Diabetes i nā Keiki

Nui kekahi ʻano like o ka maʻi i ʻokoʻa i ka genetic a me nā tapa kulekele. ʻO ka hapa pinepine, ua ʻike ʻia nā kani umauma X-pilina e loaʻa ana i kahi ʻano hoʻoilina. Eia nō naʻe, aia kahi ʻano ʻē aʻe o ka pathology. Penei ka papa o nā ʻano like ʻole o ka maʻi.

  1. X-pili kikina ʻano nui o ka maʻi phosphate. Hōʻano ʻia ia e nā loli a ka huahelu PHEX, nāna e hoʻopuni nā endopeptidase. Hāpai kēia enzyme no ka lawe ʻana i nā minima like ʻole ma o nā kahiki wai o nā pūpū a me nā ʻāpana liʻiliʻi. Ma muli o ka genation gen, ke hoʻomoe nei kēia kaʻina hana, kahi e hoʻolilo ai i kahi hemahema o nā iʻa phosphate i loko o ke kino.
  2. Xeneleu pilina pili X-pili. Hoʻohālikelike ia me ka hoʻohālikelike ʻana o ka hua CLCN5, ka mea e kāpae i kahi protein e kuleana ai no nā hana o nā kaila wai klorida. Ma muli o ke kuhihewa, ka hoʻopau ʻia ʻana o nā mea āpau ma o ka membrane o nā ʻāpana renal i hoʻopau ʻia. Hiki i nā mea lawe i kēia ʻano papaʻaina ka wahine a me ke kāne. Eia naʻe, hoʻomake ka maʻi i nā keiki kāne.
  3. ʻO Autosomal ka mana nui o ka maʻi phosphate. Hoʻohui ʻia me ka genation mutation ma chromosome 12. Ma muli o ke kīnā ʻole, hūnā ka osteoblasts i kahi protein pathological e hoʻoikaika ana i ka hoʻonui ʻia ʻana o nā phosphates mai ke kino. Hōʻike ʻia ka pathology me kahi papa maʻalahi, me ka hoʻohālikelike ʻia me nā palapala pili X.
  4. Ka ʻano ʻōiwi Autosomal. Heʻilihue kaʻai. Hōʻike ʻia ia e kahi hemahema i ka lā DMP1 i loaʻa ma ka chromosome 4. Hoʻopili i ka protein i kuleana no ka hoʻomohala ʻana o nā iwi a me nā dentin.
  5. Ka maʻi hoihoi autosomal, i hele pū me ka excretion o kalima i ka pā. Hōʻike ʻia ia ma muli o kahi kuhi i kekahi gen ma loko o ke chromosome 9 a me ke kuleana no ka hana ʻana o nā kanaleka kīpē o ka sodium.

Ma kahi o nā papa inoa inoa inoa o ka diabetes Phate, aia kekahi mau ʻano o ka pathology. ʻO nā ʻano like o nā maʻi i aʻo ʻole ʻia.

Nā ʻokoʻa ma waena o ka Rickets a me ka Phosphate Diabetes

ʻAʻole pololei nā mea like ʻole ʻo ka rickets a me ka phosphate i nā keiki. ʻO nā ʻokoʻa ma waena o kēia mau papa i ka etiology a me ke ʻano o ka hoʻolālā ʻana. ʻO nā rickets maʻamau ka loaʻa i kahi maʻi i pili me ka nele o ka cholecalciferol. Kuhi ʻia kēia mea i loko o ka ʻili ma lalo o ka mana o ka lā. He mea maʻamau ia i ka hopena maʻi ma kahi o ka waiola D i kēlā me kēia lā no nā keiki mai 1 mahina a hiki i 3 mau makahiki he nui, ʻaʻole ka manawa o ke kauwela. Ma muli o ka nele o ka cholecalciferol, ke hoʻopau nei ke kaʻina o ka hoʻopili ʻana o ka calcium. ʻO ka hopena, ulu ka ulu ʻana o ka iwi.

ʻO ka ʻokoʻa ma waena o ka maʻi phosphate, ʻo ia nō e pili ana i nā maʻi congenital. Me kēia patula, ʻike ka hoʻopiʻi ʻana o nā minela i loko o nā keiki, ma nā ʻano phosphates. I kekahi mau hihia, ua hoʻomaopopo ʻia ka hemahema o ka calcium. Ma muli o ka halaʻole o ka mineralula o ka kiko o ka iwi, ua like nā hōʻailona o nā ala. Hiki ke hoʻokumu ʻia ka ʻokoʻa ma waena o lākou ma hope o nā diagnostics laboratorium.

ʻO nā maʻi he hypophosphatemic i loko o nā pēpē: hōʻailona o ka maʻi

Hoʻomaopopo ka hōʻailona o nā maʻi i ka makahiki ʻelua o ke ola. I nā hihia pilikia, ʻike ʻia nā rickets hypophosphatemic i nā pēpē. ʻO nā ʻōpuhi e hiki ke hōʻike i ka wā kamaliʻi, ʻo ia ka hypotension a me ke pōkole o ka wāwae. ʻO nā hōʻike hōʻike pilikino nui ka:

  1. ʻO ka hoʻonālikelike o nā hana o nā wāwae.
  2. ʻO ka curvature o O o nā o nā haʻahaʻa haʻahaʻa.
  3. Ke kuʻi nei ka ulu ʻana i nā keiki - e nānā ʻia ma hope o 1 makahiki.
  4. Hoʻoweliweli ana i nā wīwī a me nā kāwili kūloko - "rickety bracelets a me ka rosary."
  5. ʻO ka ʻeha ma nā iwi pelvic a me nā spine.
  6. Hōʻino i ka enamel niho.

ʻO ka pinepine loa, ʻo ka hoʻopiʻi nui a nā mākua he ua hāmeʻe nā wāwae i ka pēpē. Ke holo nei ka maʻi, puka mai ka palupalu o ka iwi - osteomalacia.

ʻO ka hoʻomohala ʻana o ka maʻi maʻamau o ka phosphate a me nā rickets

Ke hoʻomaopopo nei i ka pathology, ke hōʻike ʻia nei kahi hōʻike o ka ʻano biochemical o ke koko. I kēia hihia, ua hoʻomaopopo ʻia ka hypophosphatemia. He maʻa ka pae o ka calcium ma ka hapa o ka nui, i kekahi manawa ua hoʻemi ʻia. I ka wā kiʻekiʻe o nā rickets, kahi kaulana i hoʻonui ʻia i ka phosphatase alkaline. Hōʻike ʻo Radiography i ka osteoporosis a me kahi pale ʻole o nā ʻōpū ulu o nā iwi. Loaʻa i ka urine ka nui o ka phosphate. ʻIke mau ʻia ʻo Calciuria i kekahi manawa.

Ka hoʻomaikaʻi ʻana i nā rickets a me ka maʻi diabetes

Hoʻohana ʻia nā nui o nā cholecalciferol e mālama i ka maʻi diabetes phosphate. I kekahi mau hihia, ua hōʻike ʻia ka hoʻomākaukau ʻana o ka calcium. E noʻonoʻo e hoʻohana i nā lāʻau lapaʻau e pili ana i ka phosphorus. No ka hoʻomaikaʻiʻana i ke kūlana, ua kuhikuhi ʻia nā meaʻala a me nā mineral. Hoʻopili kēia me ka hoʻomākaukau ʻana "Vitrum", "Duovit", "Alphabet", etc.

Me ka curvature koʻikoʻi o nā iwi, hōʻike ka hoʻomaʻamaʻa ʻana i ke kino. Kūkākūkā ʻia me kahi mea lapaʻau orthopedic.

Nā hana kūpono a me ka prognosis

Hoʻokomo ʻia nā hana pale i ka hoʻokolohua ʻana i kahi pediatrician a me nā mea lapaʻau, hele i ka lewa hou, ke koho ʻia o ka vitamin D mai ka mua o ka malama o ke ola. Inā aia nā maʻi maʻi phosphate-diabetes i ka ʻohana, pono kahi hana genetic i ka wā o ke kūkulu ʻana o ka maʻi fetal. Hoʻonui maikaʻi loa ka prognosis me ka mālama manawa.

ʻO nā ʻohana a me nā hoʻoili hoʻoili hoʻoilina: nā mea like a me nā kūʻokoʻa

Me ka maʻi diabetes, e hoʻohui pū i ka maʻi diabetes phosphate nā hōʻailona maʻamau a me ka ʻoiaʻiʻo e wāwahi i ka metabolism. Me nā rickets - ka ʻoiaʻiʻo ke alakaʻi i ka ulu kino o ka iwi, a ʻo kēia ka mea i hoʻonāukiuki i ka hoʻokūkū o ka phosphorus a me calcium. ʻO ka maʻi diabetes Phropate i nā keiki ka like me ka maʻi e loaʻa ana i ka wā i nele ka pono o ka vitamin D i ka meaʻai, i nā pākeke ke ulu pinepine ia a hōʻike ʻia e ka nāwaliwali a me ka hoʻokuʻi o nā iwi - osteomalacia. ʻO kona mau inoa inoa he nui ka huaora D-dependick rickets, hypophosphopenic rickets, familial congenital rickets, type 2 rickets.

He aha kēia mea e kū ai i loko o ke kino me kēia maʻi? I mea e hoʻopili pololei ai ka phosphorus a me ka calcium, a mai kahi e hoʻopili ʻia ai ka iwi ikaika, e pono mua ka vitamina D. I loko o ke kino me ka meaʻai, e hoʻohuli ʻia i loko o nā ʻano lāʻau like-hormone like.

E like me ke kōkua ʻana o insulin i nā selika e hoʻoheheʻe i ka glucose mai ke koko, e hoʻihoʻi ka huaʻai o vitamin D iā lākou i ka phosphorus a me calcium. A ma ka hopena like me ka mellitus o ka mellitus, ua hoʻoneʻe ʻia ka hana o ka insulin a i ʻole ka hoʻoweliweli ʻana o ka cell i ka mea i kolohe, i loko o ka diabetes phosphate ka hana o ka hoʻouka ʻana i nā mea e pono ai ke kino mai ka huaʻī D ua hūʻai a i ʻole hoʻi ka hoʻowalewale ʻana i ka ʻike o kēia mau mea. ʻAʻole i hoʻopiʻi ʻia e nā iwi, ke waiho ʻia ka calcium ma loko o ke koko, a holoi ʻia ka phosphorus me ka urine.

Hoʻohālikelike ka hopena o ka phosphate diabetes i nā mea e kū ʻole nei me ka mālama ʻana no ka rickets. Ke ulu nei ke keiki, e hoʻomau nā iwi o kāna iwi, a ma nā hihia koʻikoʻi, heʻe ʻo ia i ka hiki ke neʻe kūʻokoʻa.

Hoʻoweliweli ka maʻi hemahema i ka pākeke me kahi sporadic, ʻo ia hoʻi, ʻaʻohe ʻano hoʻoilina o ka maʻi - maʻi pepehū phosphate i ka papa o nā maʻi lalo i hoʻokumu ʻia ai.

Nā ʻlelo a me nā Diagnosis

I nā keiki, ua ʻoi aku ka nui o ka fosfor a me ka calcium ma mua o nā pākeke, no laila ua paʻakikī nā hopena o ka maʻi. E like me nā hōʻailona nui, pili pū ka phosphate diabetes me nā rickets. Ma ke kikoo, keia:

  • kamaika hunoole
  • ka pihi o nā wāwae ʻākau me ka leka O,
  • ma lalo o ka ulu maʻamau
  • curvature o ka pōpō.

I ka wā ʻōpiopio, ʻike pinepine ʻia ka maʻi diabetes phosphate ma hope o ka ʻike ʻana o nā mākua ʻaʻole i loli nui ka pēpē. Poka kekahi poʻe a hana i ka wā e koi ʻia ai lākou e hele - hōʻeha ko lākou mau iwi. Me nā rickets, ua palupalu nā iwi, no laila ʻaʻole i ʻike ʻia ka curvature, hiki ke maʻi i ka maʻi i kēlā mau keiki ke ʻike pinepine ʻia nā maʻi pipi ʻole kūpono ʻole.

Akā aia ka ʻokoʻa ma waena o ka hōʻike o nā ʻano kuʻina a me nā hoʻoilina. Hōʻike nā hika maʻamau i nā mahina mua o ke ola, ka maʻi maʻamau phate - ke hoʻomaka i ka hapa o ka makahiki, a i kekahi manawa i 1.5-2 mau makahiki wale nō ma hope o ka hoʻomaka ʻana o ka pēpē e hele. I kēia hihia, ke kūleʻe nui nei nā wāwae kuʻekuʻe (mau wāwae tubular lōʻihi), nā kuli a me nā ʻūlū kuʻuna e kiko mua.

Hōʻailona i nā hōʻike koko kūpono no ke kōkua ʻana o nā haʻawina biochemistry a me nā x-ray. Nā ʻōnaehana biochemical o ke koko a me ke ʻano o nā kiko o ka iwi ma kahi maʻi ʻohana ʻōpiopio e like ʻole i nā maʻamau maʻamau a me kēlā mau pilikia i ʻike ʻia me nā rickets puʻupuʻu. E like me ke kānāwai, ua manaʻo ʻia ka phosphate i ka maʻi maʻi i kahi keiki, ʻōlelo aku nā kauka i ka hoʻāʻo ʻana i nā mākua a me nā hoahānau pili koke.

Hiki paha ke ho'ōla i nā rīpela congenital ʻohana?

ʻO ke kumu o ka mālama ʻana i nā kuʻikahi a me nā ʻohana hypophosphatemic rickets ka like - ka hoʻomaka ʻana o ka vitamina D. Akā inā pilikia ka hana ʻana o ka ʻohana, ua loaʻa ka nui o ka vitamina i nā manawa mua ma mua o ka hihia mua. I ka manawa like, pono nā kauka e nānā i nā pae o ka lua a me ka calcium ma ke koko a me nā mea ʻē aʻe o nā biochemical ʻē aʻe, e koho ana i ka pāleona kūpono.

Hoʻopili ʻia ka lāʻau lapaʻau me ka hoʻomākaukau ʻana o ka fosfor (calcium glycerophosphate) a me kahi pāʻina, a hilinaʻi ʻia ma muli o ka makahiki, ua kau ʻia nā pēpē vitamina complexes, no ka laʻana, ʻo Oxidevit, a me ka meaʻai momona o ka phosphorus ua hoʻohui ʻia i kā lākou papaʻai.

No ka like me ka maʻi diabetes phosphate e like me nā rickets o ka ʻano 1, a i kekahi manawa ʻaʻole hiki ke hoʻonā ʻia kahi maʻi maʻi koke, aʻo nā kauka i nā mākua i nā keiki ma lalo o ʻelua a ʻekolu paha mau makahiki e hoʻomaʻamaʻa piha i kēlā me kēia manawa. I kekahi mau manawa, ʻaʻole lawa ka loaʻa ʻana o ka lāʻau lapaʻau, a laila hoʻoponopono ʻia nā iwi kūloko e nā ala o ka maʻi. Akā e pau ʻē paha ka maʻi ma hope o ka mālama ʻana?

E like me ka maʻi diabetes 1 hiki ʻole ke hoʻihoʻi hou i ka hana o kou iho i hiki i ka insulin, hiki ʻole ke "hoʻoponopono" i ka maʻi ʻaila ma ka maʻi mellitus. Akā i nā pākeke, ʻo ka nui o ka calcium a me ka phosphorus e hoʻopili ʻia ana e ke kino e lawa.

No laila, ma hope o nā wā'ōpiopio, e ulu nui ana ka maʻi ma waena o nā manawa nui o ka pono no ka calcium a me ka phosphorus - i nā wahine i ka wā hāpai a me ka lactation.

Eia nō naʻe ʻo nā huaʻi "ʻaʻole i kahakaha" no nā rickets - ke kūlana pōkole, ka curvature o nā wāwae - ke noho mau nei nā kāne a me nā kāne no ke ola. ʻO nā wahine ma muli o ka hoʻololi ʻana o nā iwi pelvic i kekahi manawa e hana i kahi ʻāpana cesarean.

Hoʻomaopopo o ka Phosphate Diabetes

  • ʻO kēia maʻi ka hopena o kahi mutation i ka chromosome X, ke hoʻopau nei i ka hoʻopio ʻana o nā phosphates i nā tubula renal, a laila hoʻihoʻi mai i kahi mana o nā kaʻina pathological e hoʻopili nei i nā kinipona maʻamau o ka calcium ma nā iwi iwi.
  • ʻO nā kāne e halihali ana i ke aʻa maikaʻiʻole i hāʻawi i kā lākou mau kaikamahine.
  • Ke hoʻouna nei ka wahine i lawe ʻia i ka lua i nā maʻi i nā keiki o nā kāne a me nā kāne a me nā kaikamahine.

E kōkua ka lāʻau lapaʻau lāʻau i ka mālama ʻana o nā maʻi

Hoʻomaʻamaʻa i ka maʻi phropate

  • Ke koho nei i nā kaumaha he nui o ka vitamina D (i kekahi manawa no ke ola).
  • Manaʻo, kalumo, nā huaora A a me E.
  • Ma kahi o nā nāwaliwali o ka palupalu (a i ʻole nā ​​palena) o ke ʻālaʻi, ua hoʻākāka ʻia ka mālama orthopedic (no ka laʻana, ka hoʻoponopono ʻana i ka curvature o ka mākeke me ka hoʻohana ʻana i nā corsets orthopedic kūikawā).
  • Pono pono ka hoʻomaʻamaʻa ʻana me ka deformations koʻikoʻi o ka pāpaʻi. Mālama ʻia ma hope o ka pau o ka manawa ulu.

Nā hopena a me nā hopena

  • ʻO ka hōʻino ʻana i ka posture a me ka hoʻololi ʻana o ka ʻūhā ma hope o ka maʻi diabetes phosphate i mālama ʻia i ke wā kamaliʻi e mālama ʻia no ke ola.
  • Loaʻa ke keiki i ka noʻonoʻo ʻana a me ke kūkulu kino ʻana.
  • ʻO ka holomua o ka iwi a me ka de artities i ka ʻike ʻole o ka hana kūpono e hiki ke alakaʻi i ka pilikia.
  • Haki i ka ulu ʻana o nā niho (ke alapine o ke ʻano o ka enamel, ka hoʻonāukiuki ʻana o ka manawa a me ka papa o ka mea, me nā mea āpau).
  • Ma muli o ka nalowale ʻana (me ka hopena o ka hoʻomōhala ʻana mai o ka ossicles auditory o ka pepeiao waena.
  • Hiki i ka hopena o ka maʻi ke nephrocalcinosis (ka waiho ʻia ʻana o nā paʻakai kaleka ma loko o nā pūpū), a ma ke ʻano e hiki ke hele i ka hemahema.
  • ʻO kahi maʻi i loaʻa i ke kaikamahine i ka wā kamaliʻi e hiki ke hana i ka deformity o ka iwi pelvic, ma muli o kahi hana paʻakikī paha i hana ʻia. Ma ka hoʻomaka ʻana o ka hoʻolālā hāpai ʻana, pono i nā wahine i loaʻa i ka maʻi diabetes phosphate i ka wā kamaliʻi e kūkākūkā me ka mea loea e pili ana i ka ʻāpana cesarean.

Kākoʻo o ka Phosphate Diabetes

  • ʻO ka ʻike mua i ka maʻi. (Ka manawa kuhikuhi i ka poʻe loea ma ka hōʻailona mua o kahi maʻi no ke kumu o ke ʻano mua a me ke koho ʻana i ka mālama manawa kūpono: ʻo ke kumu nui o kēia mau hana he hoʻemi i nā hōʻeha a me nā hopena hopena).
  • ʻO ka mālama pono ʻana a me ka mālama mau ʻana i nā keiki me kahi ʻano like like e ka pediatrician a me ka endocrinologist.
  • ʻO ke kauka ʻana i ka lāʻau lapaʻau a me ka pili ʻana no nā ʻohana me ka maʻi diabetes phosphate (kahi i loaʻa ai kekahi o nā ʻohana i kēia ala ma ka wā kamaliʻi) i ka hoʻolālā ʻana o ka ʻōpū.ʻO ke kumu o kēia hanana ka mea e wehewehe i nā mākua i ka hiki ke loaʻa o kahi keiki maʻi a hoʻomaopopo i ka pili ʻana i nā pōpilikia, nā hopena, nā hopena o kēia maʻi o ke keiki.

Kālai koho

  • Ua ʻike ʻia kekahi o nā mea nui o ka paepae o ka iwi iwi ka calcium. ʻO ke kumu o ka pīpī o ka iwi me ka kalula ʻo ia hoʻi ka fosfor. Pau ke kanaka i kēia mau mea me ka meaʻai.
  • ʻO kahi hapa nui o nā phosphates (nā pūhui fosfor) i loko o ke kino kekahi o nā kūlana e pono ai no ka hoʻopili ʻana i ka kalala e nā iwi iwi.
  • Ma muli o kahi loli ʻana o kekahi o nā huaʻomo chromosome X, ke kīnā ʻole ʻia ka phosphate i nā pā ʻana o ka renal, a ma hope aku ua kaulia kekahi ʻano kaʻina o nā pathological e hoʻoemi i ka hoʻōla ʻana o ka calcium ma nā iwi iwi.

KA HALEOLELO KUPUNAKANI

Pono ke kūkākūkā me ke kauka

Kūpono ma ka biochemistry o KSMA, 2004
Volkov M.V., Dedova V.D. ʻO nā keiki Orthopedics-Kaukaʻaka, 1980

ʻO pathogenesis o ka maʻi

Ma muli o ka hoʻoili ʻia ʻana ma muli o ka hoʻoili ʻia e kahi ʻano kiʻekiʻe, e hilinaʻi nei ma ka kāne. Me ka maʻi, hoʻohālikelike nā kaʻina o ka enzymatic, e hoʻololi ana i ka vitamin D i nā mea ikaika.

Ka hoʻomohala ʻia nā maʻi diabetes Phropate ma muli o kahi hemahema o ka tubal renal, e hoʻopili ʻia i ka reabsorption phosphate. Me ka nui o ka hapa pakeneka o ka excretion fosfor me ka urine, ʻo ia ka nui o ke koko o kahi kanaka e hoʻemi ana, e hoʻoiho ai i nā iwi deformities.

ʻO ke kumu nui o ka hoʻomaka ʻana o ka maʻi ka mutations o ka X chromosome, ka mea e hōʻole i ka hoʻopili ʻana o nā phosphates a hoʻowalewale i ke ʻano o nā kaʻina hololi e hoʻomāhuahua i ke kaʻina o ka hoʻopili ʻana o ka calcium.

ʻO nā ʻōkuhi e hōʻike ana i ka loaʻa ʻana o nā pilikia olakino e hoʻopili ana i ka hoʻomohala ʻana o ka maʻi phosphate penei:

  • ʻO ka hoʻonāʻana i ke kūlana maʻamau o ke keiki.
  • Ke ulu ʻōpala.
  • ʻO ka lūlū o nā haʻahaʻa haʻahaʻa ma ke ʻano o-a.
  • Nā loli i loko o nā kuʻekuʻe a i nā kuli.
  • Nā leo haʻahaʻa o lalo.
  • Ma ke kua, inā palpating, ʻike ʻia ka ʻeha.

Hōʻike nā haʻawina haukaʻi i nā hōʻailona nui o ka maʻi ma kahi kanaka i pilikia me ke kūkulu ʻana o ka musculoskeletal system a me ka deformities o nā hopena haʻahaʻa e hele mai. Eia kekahi, i ka poʻe me ka maʻi diabetes, ka maʻa pōkole, nā mea hōʻino a me nā mea hana'ē aʻe o ke kāʻei, e hele i ke ala, ke hoʻololi ʻia ka hana o ke au, ʻo ka ʻeha i ka wā o ka neʻe ke ʻike

Hiki i nā kauka ke hana i kahi maʻi ma nā makahiki ʻelua o ke ola, ʻoiai ke hoʻomohala nei ka palaka i ke wā ʻōpiopio ma muli o kahi predisposition genetic. Aia no ka hoʻomohala naʻauao, ma kēia hihia, ʻaʻohe hopena o ka diabetes phosphate, akā pili naʻe i ka kūlana noʻonoʻo a hōʻike mau iā ia iho i ka ʻike ʻana i ke keiki i kona ʻokoʻa ʻana me nā hoa, nā palena pilikino.

Nā ʻōiwi o ka hypophosphatemic rickets (phosphate diabetes)

Hoʻokaʻawale nā ​​kauka i kēia maʻi i 4 mau ʻano nui:

  • Hōʻike ʻia ka ʻano 1 i ka makahiki 2 o ke ola o ke kanaka. Nā hōʻailona nui: ulu ka ulu, ʻaʻohe ʻōpiopio o ka enamel o nā niho mau, nā deformities o nā wāwae, ʻano like me nā loli o ka iwi. Me kēia ʻano maʻi, hoʻemi ka reabsorption phosphate a hoʻomohala like nā loli rickets-like.
  • ʻO ka ʻaoʻao 2 kahi mana autosomal, ʻaʻole i pili ʻia me ka X chromosome. Hōʻike ʻia i ka makahiki mua a ʻelua paha o ke ola. Hōʻike ia iā ia iho i ke ʻano o ka curvature o nā haʻahaʻa haʻahaʻa, e loli i ka ʻūhā. I kēia hihia, ʻaʻole loli ka hana o ka ulu ʻana, ke ʻano ikaika o kahi kanaka. Aia aia he mau hōʻailona hoʻohālikelike.
  • Hōʻike ka ʻano 3 i ke ʻano o ka hoʻopiʻi ʻana o ka calcium, e alakaʻi ana i ka mea i hiki i ke 6 o ke ola o ke ola, loaʻa ke keiki, nā kīkuhi, pōkole, puʻupuʻu pōkole, nāwaliwali o ka ʻōpū a me nā mea holo manu i ka ulu ʻana.
  • Hoʻokomo ka type 4 i ka wahine i ke wā kamaliʻi. Hōʻike ia iā ia iho i ke ʻano o ka curvature o nā haʻahaʻa haʻahaʻa haʻahaʻa, ke ʻano a me nā hōʻino niho ma nā ʻano ʻokoʻa like ʻole.

Nā hopena e kū ana mai ka maʻi

Ma muli o ka hoʻomaʻemaʻe ʻole ʻana, hiki i nā maʻi me kēia maʻi ke loaʻa i kahi hōʻeha i lalo nei.

  • ʻO ka hōʻino ʻana i nā kuʻekuʻe, ma muli o ka hopena - posture.
  • Ka maʻi noʻonoʻo a kino kino paha i ke kūkulu ʻana o ke keiki.
  • Nā kumulāi i puka ʻole ai ka maʻi articular e alakaʻi ana i ka maʻi kīnā.
  • ʻO ke ala o ke kūkuluʻana o ka niho, ka wā pale o ka manawa o kā lākou kūkulu.
  • ʻO nā maʻi o ka hoʻomohala ʻana i ka ossicle auditory.
  • ʻO Neprocalcinosis, kahi i ho'ākāka ʻia e nā waiho ʻana o ka paʻakai o ka paʻakai i loko o kaʻeha.

Inā loaʻa ka predisposition o ke keiki i ka holo ʻana o kēia maʻi, a laila hoʻomaka ke hoʻomaka ʻana kāna nānā ʻana mai ka hānau ʻana, i mea e pale ai i nā hoʻopiʻi ma kahi hiki. No ka hana ʻana i kēia, ʻike lākou i ke kiʻekiʻe o ka calcium a me ka fosfor, ka nānā ʻana i ke ʻano o ka ʻūhā, inā pili ka ulu ʻana i nā ʻōnaehana i kūpono i kēia au.

Ke ʻike ʻia nā hōʻailona mua o ka maʻi phosphate, ua kuhikuhi ʻia nā keiki i nā huaora. Inā makemake ʻia, hiki i nā mākua ke hoʻonohonoho i nā pōmaikaʻi no ke keiki e loaʻa i ka lāʻau lapaʻau no ka manuahi, me ka hoʻonohonoho pono i nā huakaʻi huakaʻi ʻole i nā kahua olakino kūikawā.

Nā manaʻo loea haukapila

ʻO ka maʻi diabetes Phropate kahi maʻi maʻi weliweli e ulu ana ma muli o kahi predisposition genetic, ʻike ʻia i ka wā kamaliʻi a hiki ke hōʻeha i nā hoʻopiʻi weliweli.

ʻO nā manaʻo e pili ana i ka pale ʻana o ka maʻi, hiki ke hoʻokaʻawale i ke kūkulu ʻana o kēia maʻi i ka mālama ʻana ʻana o nā ʻohana ʻōpio, inā hiki ke pilikia kēlā ʻano pilikia i ka ʻohana a me ka predisposition genetic.

Ma mua o ka hānai ʻana i kahi keiki, e kuhikuhi ke kauka i kahi loiloi piha, ma hope o ia e hoʻokau ai i ka hopena o kahi keiki olakino e hānau ʻia ana. Hiki iā ʻoe ke hoʻokaʻawale i ka hoʻomohala ʻana i ka maʻi phosphate me ka hele ʻana i ka manawa ʻike loea i kahi loea inā hoʻomaka kona mau hōʻailona i kahi keiki mai ke wā ʻōpiopio.

I mea e pale ai i ka hoʻomohala ʻana o kahi maʻi weliweli i kahi keiki, pono ia e hahai kūpono i nā ʻōlelo aʻoaʻo a nā poʻe loea a me ka hoʻokaʻa iki ʻana i ka hale lapaʻau no ka maʻi maʻi a me ka mālama ʻana. ʻO ka mea mua ke nānā aku ka pākeke i ka neʻe ʻana i ka hoʻomohala maʻamau o ke keiki, ʻoi aku ka nui o ka hiki o ka neʻe o ka hiki ke hoʻopau i nā hoʻopiʻi he nui.

Wahi a nā hōʻike waiwai, ʻoiai, he 52% o ka poʻe noho o ka ʻāina i hōʻike ʻia me ka maʻi maʻi. Akā i kēia manawa, ʻoi aku ka nui o nā poʻe i huli i nā cardiologists a me nā endocrinologists me kēia pilikia.

Hiki i nā maʻi kuʻuna ke alakaʻi i ke ulu ʻana o nā maʻi ʻopa maʻi. Hoʻokahi ala a i ʻole kekahi, ʻo ka hopena i nā hihia āpau he like - he make ka maʻi maʻi maʻi, hakakā me ka ʻeha hōʻeha, a i ʻole ia e lilo i kahi maʻi kīnā maoli, i kākoʻo ʻia me ke kōkua lāʻau lapaʻau wale nō.

E pane wau i ka nīnau me kahi nīnau - he aha ka mea e hiki ke hana i loko o ia kūlana? ʻAʻohe o mākou papahana loea e hakakā me ka maʻi maʻi, inā e kamaʻilio e pili ana ia. A i loko o nā haukapila i kēia manawa ʻaʻole hiki ke loaʻa ka endocrinologist i kekahi manawa, ʻaʻole ia e haʻi i ke ʻimi ʻana i ka endocrinologist kūpono a maʻi maʻi maʻi paha e hāʻawi iā ʻoe i ke kōkua maikaʻi.

Loaʻa mākou i ka hana mua i ka lāʻau lapaʻau mua i hana ʻia ma ke ʻano o ka papahana o ka honua. Hāʻawi ʻia kona ʻokoʻa a hiki iā ʻoe ke lawe lohi i nā lāʻau lapaʻau pono i loko o nā moku koko o ke kino, e komo nei i loko o nā moku koko o ka ʻili. ʻO ka hōʻemi ʻana i loko o ke kahe koko ke hāʻawi i nā mea pono i ka ʻona o ke kao, e alakaʻi ana i ka hoʻoilo o ke kō.

Waiho I Kou ManaʻO HoʻOpuka