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