ʻO ka maʻi hôpika Phropate kekahi o nā maʻi i loaʻa ʻole i ka hoʻomaʻamaʻa ʻana i ka lāʻau.
He maʻi maʻamau kēia o ka maʻi, nā like pū me nā lāʻau like ʻole, kahi e kū mai nei i kahi kāʻei o nā mea hou aku i ka phosphate reabsorption i loko o ka renal tubules.
Kēia mau loli e alakaʻi i ka ulu ʻana o ka hypophosphatemia, hoʻopio ʻole o ka calcium a me ka phosphorus i ka ʻōpū o ka ʻōpū a me ka hopena, ʻo ka lawelawe ʻana i ka iwi kūpono ʻole.
E like me kāu e ʻike ai, ʻo ka mea nui e ʻike nui ai ka iwi iwi i ka calcium, kahi mea e loaʻa mai i kekahi kanaka mai ke kaiʻai a me ka meaʻai i ʻono ʻia. Mālama ʻia ka hoʻoliʻi ʻana o kēia ʻano mea ʻenemi e nā pūnaehana fosfor.
Pili nā pathogenesis o ka diabetes phosphate i kahi ʻano genation mutation e alakaʻi ana i nā haunaele i nā hana o ka phosphate mai nā renal tubules i loko o ke kahe wai, ma ia manawa ua hoʻonāukiuki i nā ʻano o nā pilikia pathological e pili ana i ka demineralization iwi, ko lākou deforme a hoʻonui i ka fragility.
No ke aha i kū ai ka maʻi?
ʻO ka maʻi diabetes Phosphate i nā keiki he hopena o ka mutation i loko o kekahi o nā ʻano i loaʻa ma ka X chromosome.
Ua hoʻoili ʻia ka maʻi e like me ke ʻano nui, ʻo ia hoʻi, mai kahi makua maʻi i nā kaikamahine āpau, a mai kahi makuahine maʻi a 50% o nā keikikāne a 25% o nā kaikamahine (i hāʻawi ʻia ka wahine ʻoi ʻia ka maʻi like).
I ka hoʻomaʻamaʻa keiki, ʻike pū kekahi ʻo nā hihia o ka hypophosphatemic rickets, e loaʻa ana me nā maʻi o ka maʻi o ka pūpū, nā maʻi.
Ma ka lāʻau lapaʻau, hoʻokaʻawale pū ʻia nā maʻi diabetes paraneoplastic, ka mea i loaʻa ma muli o ka hoʻonui nui o nā ʻōnaehana hormone parathyroid e nā cell tumor. Eia kekahi, ʻokoʻa paha kēia maʻi genetic i loko o nā ʻōnaehana ʻōnaehana, i ka maʻi Fanconi maʻamau.
Pehea e hōʻike ʻia ai ka maʻi?
I ka lāʻau lapaʻau o kēia wā, he hana maʻamau e hoʻākāka i nā ʻano nui he nui o ka mauʻu hypophosphatemic, kahi i hōʻike ʻia i loko o nā makahiki mua o ke ola o ka mea maʻi liʻiliʻi a loaʻa iā lākou nā hiʻohiʻona.
ʻO ka maʻi diabetes phosphate type a i ʻole hypophosphatemia i hoʻopili ʻia me ka chromosome X he maʻi hoʻoilina, ke kumu nui o ka hoʻopiʻi ʻana i ka puna o ka phosphorus i loko o nā pūhaka renal, e alakaʻi ana i ka hoʻoliʻiliʻi o ka phosphates i ke koko, ka hoʻomohala ʻana i ka phosphaturia a me ka nānā ʻana o nā ʻano like ʻole e like me nā loli o ka iwi i kū ʻole i ka hoʻokipa lawa pono o ka vitamina D.
Hōʻike ka maʻi i nā makahiki ʻelua o ke ola o ke pēpē a hōʻike ʻia e nā ʻano o nā hōʻailona aʻe:
- ulu ʻana i ka ʻala ma ka kua ʻana o ka ikaika ʻoi aku ikaika;
- hana ʻole a hoʻopau ʻole i ka enamel ma nā niho.
- lauoho lauoho
- huaʻili valgus deformity o nā kahe haʻahaʻa, e hele pū ana me nā ala mua o ke keiki;
- nā hōʻailona o ka ʻalani e hoʻoheheʻe ʻia nei, e hele ana me nā ʻōhua maʻamau o ka vitamin D;
- ke emi ʻana o ka pae o ka phosphates i ke koko a me ka hoʻonui i ka urine;
- lohi ana i ka ʻōnaehana o ka musculoskeletal e like me ke ʻano o ka makahiki.
ʻO ka maʻi Diabetes Type II he ʻano hoʻohālikelike o ka hypophosphatemia, ʻo ia ka mea mana autosomal a ʻaʻole i pili ʻia me ka chromosome X sex.
Hoʻohālikelike ʻia ka maʻi diabetes Ph II II e nā hōʻailona like ʻole:
- mai nā maʻi mua loa o ka maʻi mai ka makahiki 12;
- nui curvature o nā ʻoi loa a me ka hoʻololi ʻana o ka huila;
- ka nele o ka ulu ʻana a me ka hoʻoikaika kino;
- nā hōʻailona o ka radiological o nā rickets mālie a me ka osteomalacia o ka iwi iwi;
- i nā hoʻokolohua hōʻoia, ua hōʻemi ʻia nā manaʻo o nā phosphates i loko o ke koko a hoʻonui i nā nui i ka urine i ka pae o ka calcium maʻamau.
ʻO ka maʻi diabetes diabetes III a i ʻole nā hypocalcemic rickets kahi maʻi genetic i hoʻoilina i ka hoʻoiho autosomal a me ke hilinaʻi nui ʻana i ka vitamin D. Nā hōʻailona mua o ka maʻi a hiki i ka wā kamaliʻi.
Ke hoʻokolohua nei i kahi mea maʻi liʻiliʻi, hiki ke hoʻoholo i ke alo o kekahi mau ʻōmaʻomaʻo, a ma waena o ia:
- huhū, waimaka, palaka;
- ua hōʻemi i ka leo kani;
- kūloko;
- ka ulu haʻahaʻa, ulu wikiwiki i ka deformations;
- nā uʻi niho maikaʻi;
- hiki ke hoʻomaka o ka hele ʻana;
- Hoʻoholo ka hypophosphatemia i loko o ke koko, a me ka hyperphosphaturia i loko o ka urine;
- nā loli paʻakikī e like me nā rickets ma nā wahi ulu, a me nā hōʻailona no ka nui o osteoporosis o ke kiko o ka axial i hoʻopaʻa ʻia ma ka radiologically.
ʻO ka maʻi diabetes Phropate ka maʻi a i ʻole ka waiʻaole D3 he maʻi hoʻohemo i lawe ʻia i nā keiki mai nā mākua ma kahi ʻano hoihoi autosomal a puka ʻia mai kahi wā ʻōpio loa.ʻO ka hōʻailona aʻe ka hiʻohiʻona o ka maʻi diabetes IV IV:
- valgus deformity o nā kahe haʻahaʻa, curvature o nā ʻāpana like o ka ʻūhā;
- nahoa
- baldness a me ka paʻakikī paʻakikī o ka enamel niho;
- Hoʻomaopopo nā X-hihi i nā hōʻailona maʻamau o nā makaʻi ʻano.
Pehea e hoʻoholo ai i ka maʻi?
Nā loiloi haukaʻi no ka maʻi maʻi phosphate e pili ana i ka nānā ʻana i ke kaʻina hana no ka hoʻomakeʻa ʻana me kekahi o nā hoʻokolohua e pili ana i ka hoʻoholo ʻana i nā pilikia o ka hoʻohālikelike o ka phosphate reabsorption i loko o nā renal tubules.
I waena o nā kaʻina hana diagnostic:
- ka hōʻiliʻili ʻana o ka ʻike anamnestic mai nā ʻōlelo a nā mākua o ka keiki i ʻike i ke ʻano o nā deformities skeletal, curvature o nā wāwae, ulu nui a me nā mea like;
- ka nānāʻana i ke kanaka maʻi liʻiliʻi me ke koho ʻana i nā syndromes alakaʻi;
- noiʻi genetic no nā anomalies o ka chromosome X a me kahi predisposition hoʻoilina i ka hoʻomohala ʻana o ka hypophosphatemia;
- urinalysis, kahi nui o nā pūlea phosphate i huna ʻia;
- ʻO ka nānāʻana X-ray o nā iwi o ka ʻōpū o nā keiki me ka wehewehe ʻana i nā wahi o osteomalacia, osteoporosis, nā maʻi maʻamau i nā ulu ulu;
- ka hoʻokaʻawale ʻana o ke koko ʻōlapa no ka hoʻohaʻahaʻa phosphate i ka nānā ʻana o ka calcium.
Nā hiʻohiʻona hana
Hoʻokahi mau makahiki i hala aku nei, ua paʻakikī nui ka maʻi diabetes, me ka aneane hiki ʻole ke pale aku i nā deformities iwi o ka palupalu. I kēia mau lā, mahalo i ka hoʻokolohua hou, ua hiki ke hoʻomaikaʻi i nā helu helu, e like me ka hypophosphatemia ua kūleʻa i ka hoʻomaikaʻi ʻana i ka lāʻau lapaʻau i ʻike ʻia ʻana e ʻike koke ʻia ka maʻi a loaʻa ka ʻike hoʻokūkū.
Hoʻokomo ʻia no ka maʻi phosphate, ka:
- ka koho ʻana o nā haʻahaʻa kiʻekiʻe o ka vitamina D (mai 25 tausani a 250 tausani mau ʻāpana / lā);
- ka hoʻohana ʻana o ka calcium, ka phosphorus;
- ka hana ʻana i nā huaora A a me E, hoʻōla citrate.
Pono ka photherapyotherapy o spinal deformities i kekahi mau papa o ka hiki ʻana i ka lawelawe, me ka paʻa ʻana i ke kāʻei ʻana i ke corset kākoʻo, e ʻae ana i ka ʻōpala i ke ʻano i ke kūlana kūpono.
Hiki paha ke pale i ka maʻi?
No ka mea he hoʻoilina ka maʻi, hiki ke pale ʻia ma ka nānā ʻana i kahi ʻohana ʻōpio i loko o ka ʻikepili genetic i ka wā o ka hoʻolālā ʻana o ka wahine hāpai.
Kūkākūkā genetic
Pono e nānā pono i ka pilikia ma luna o nā kāne i kahi i loaʻa ai kekahi o nā hoapili mai kēia maʻi. E wehewehe ka mea hoʻokūkū hoʻokūkū kūpono i ka hiki ʻana o kahi pēpē e ola pono ana a e lūlū e pili ana i nā pilikia o ka hāpai ʻana i kahi pēpē e hoʻoili i ka maʻi o kekahi o kona mau mākua.
ʻO ka pale ʻana o ka maʻi mai ka manawa kūpono e ʻike nei i nā maʻi e hiki ai ke mālama ʻia i nā keiki liʻiliʻi e loaʻa ana nā hōʻailona mua o ka hypophosphatemia.Me kahi pane ʻole pololei i ka pilikia, he nui nā ʻōuli a me nā hopena, ke keiki:
- waiwa ma hope o nā mea pili i ke ola kino a me ka noʻonoʻo;
- ke ʻano o nā deformities koʻikoʻi o ka spine a me nā haʻahaʻa haʻahaʻa;
- kekahuli o nā niho enamel;
- ka wehe ʻana i nā iwi pelvic, ʻīwī o kona mau diam;
- hōʻino ʻia ka hoʻomohala ʻana o nā ossicles auditory i ka pepeiao waena a me ka lohe ʻole ʻana aku;
- urolithiasis a me ka renal maikaʻi i hua ʻia mai ia.
Nā wikiō pili
ʻO Kauka Komarovsky ma luna o nā rickets a me ka liʻiliʻi o ka waiola D i nā keiki:
Ma ka mea maʻamau, ka puna o ka prognosis no ka diabetes phosphate, akā makemake paha kēlā mau keiki i ka hoʻihoʻi lōʻihi a me ka hoʻololi ʻana i ka nohona i ke ola me nā de mau ulu i kūkulu ʻia. Pono nā mea maʻi liʻiliʻi e hoʻopaʻa inoa mau ʻia i ka ʻahikō a me ka manawa e kūkākūkā mau ai i ko lākou kūlana me nā loea kūikawā (endocrinologist, nephrologist).
