Ọrịa shuga Phosphate > Ọrịa mamịrị

Ọrịa shuga Phosphate bụ usoro ọmụmụ nke na - esiteghị na imebi insulin na pancreas na oke glucose dị n'ọbara. Ihe kpatara ọrịa a bụ metabolism nke vitamin D, yana phosphates. Site na nchọpụta nke ọrịa shuga phosphate, a dịghị ewepu ihe ndị a na tubules akụrụ, a na-ejikwa usoro kemịkal na-ezighi ezi mara njiri anụ ahụ.

Kedu ka esi gosiputa ọrịa shuga phosphate n'ime ụmụaka?

Hypophosphatemic rickets na-amalite igosipụta n’ebe ụmụaka nọ n’oge nwata. Ọtụtụ mgbe, ihe ịrịba ama na-eme na afọ mbụ ma ọ bụ n'afọ na - esote, mgbe nwatakịrị ahụ na - amalite ije n'enweghị nsogbu. Ọrịa shuga phosphate anaghị emetụta ọnọdụ mmadụ niile.

Enwere nlọghachi azụ na-eto.
Twkwụ gbagọrọ agbagọ.
Nkwonkwo ikpere na nkwonkwo ụkwụ na-emebi emebi.
Ọkpụkpụ dị na mpaghara nke nkwonkwo aka aka.
Uda olu.
N'oge palpalica, a na-enwe mgbu azụ na ọkpụkpụ. Ọkpụkpụ siri ike nwere ike ime ka nwatakịrị kwụsị ịgagharị onwe ya n'ụkwụ ya.
N'ọnọdụ ndị na-adịghị ahụkebe, ntụpọ enamel na ezé, rickets na spain ma ọ bụ ọkpụkpụ pelvic na-ahụ.
Spasmophilia, njiri mara rickets nwere ụkọ vitamin D, nwere ike ịmalite.
Ọ nwere ike ga - aka mbọ ụkwụ aka nwa amụrụ ọhụrụ (a na -echekarị ya).
Na ọgbọ, onye ọrịa na-etolite osteomalacia.
Ihe onyonyo X-ray na egosiputa osteoporosis, a na –ezube ọkpụkpụ n’aho.
Calcium dị elu n’ọkpụkpụ.
Ngwakọta nke electrolytes, amino acids ọbara, creatinine, CBS agbanweghị.

Nwatakịrị choro otutu ihe bara uru ma na-edozi ahụ, gụnyere phosphorus na calcium, maka mmepe na uto nkịtị. Enweghị ihe ndị a na-eme n'afọ mbụ nke ndụ na-akọwa ịdị njọ nke ọrịa ahụ.

Iche-iche nke ngosipụta ọrịa

Phosphate ọrịa shuga, dabere na mmeghachi omume na ntinye nke vitamin D ka edepụtara dị ka ndị a:

Ejiri amamịghe nke ihe ana - eme phosphates inorganic n’ime ọbara, nke pụtara n’ihi mmụba nke reabsorption n’ime akwara ndị gbasara aha.
Amara ya site na ịbawanye n’iyi nke phosphates n’ime eriri afọ ya.
Reabsorption na-agbasiwanye ike naanị na eriri afọ.
Ọrịa shuga phosphate nwere mmetụta na-abawanye na vitamin D. Ọbụna obere usoro nke ihe a nwere ike ime ka egbu egbu.

Isiokwu ndị ọkachamara n'ihe banyere ọgwụ

Ọrịa shuga na-akpata ọrịa bụ ihe a na-ahụkarị n'ọrịa ndị a na-eketa eketa na-arịa metabolism nke phosphates na vitamin D. Ọrịa hypophosphatemic ọrịa bụ hypophosphatemia, mmịpụta calcium na rickets ma ọ bụ osteomalacia, na-adịghị emetụta vitamin D. Ihe mgbaàmà gụnyere mgbu ọkpụkpụ, mgbaji na ọgba aghara. uto. Nchoputa a dabere na mkpebi nke serum phosphate, alkaline phosphatase na ọkwa 1,25-dihydroxyvitamin D3. Ọgwụgwọ gụnyere nnabata nke phosphates na calcitriol.

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Ihe na - akpata ya na pathogenesis nke ọrịa shuga phosphate

A na - enweta ụdị hypophosphatemic rickets site na ụdị X jikọtara. A na-ejikọ ọnọdụ nke sporadic hypophosphatemic rickets mgbe ụfọdụ na etuto benen mesenchymal (oncogenic rickets).

Ndabere nke oria bu mbelata ihe eji emeghari mmiri n’osimiri proximal, nke n’eduba na hypophosphatemia. Ọrịa a na - etolite n'ihi mgbasa ozi ihe yana ejikọtara ya na nsogbu ndị bụ isi na ọrụ osteoblast. Enwekwara mbelata nke mkpanaka calcium na phosphate. Ọkpụkpụ mejupụtara ọkpụkpụ bụ ihe kachasị n'ihi ọkwa phosphate na dysfunction nke osteoblasts karịa n'ihi ọkwa ọkwa calcium dị ala na ụba nke homonụ parathyroid na rickets na-adịghị ike. Ebe ọ bụ na ọkwa 1,25-dihydroxycholecalciferol (1,25-dihydroxyvitamin D) dị nkịtị ma ọ bụ belata ntakịrị, enwere ike ịmalite ntụpọ na usoro vitamin D dị ike, hypophosphatemia kwesịrị ime ka mmụba nke ọkwa 1,25-dihydroxyvitamin D.

Hypophosphatemic rickets (ọrịa shuga phosphate) na-etolite n'ihi mbelata phosphate reabsorption na proximal tubules. A na-ahụ ọnya ụdọ nke a dịpụrụ iche, ụdị ihe nketa a bụ nke ukwuu, jikọtara ya na X chromosome. Na mgbakwunye, ọrịa shuga phosphate bụ otu n'ime ihe ndị metụtara Fanconi syndrome.

Ọrịa paraneoplastic phosphate na-ebute site na mmepụta nke homonụ parathyroid-dịka nke mkpụrụ ndụ tumo.

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Ọrịa shuga nke Phosphate

Hypophosphatemic rickets gosipụtara onwe ya dị ka usoro nke ọgba aghara, site na hypophosphatemia asymptomatic ruo oge anụ ahụ na-egbu oge na obere ala ruo n'ụlọ ọgwụ nke rickets ma ọ bụ osteomalacia siri ike. Nkpugheputa umuaka na - adi iche mgbe ha bidochara ije, ha na - etolite Ighari-ukwu nke ikpere aka na nrụrụ ọkpụkpụ ndị ọzọ, na - emegharị ụkwụ, mgbu ọkpụkpụ na obere mkpụmkpụ. Otito uto na saịtị njikọta akwara nwere ike ịkwụsị mmegharị. Site na rickets hypophosphatemic, rickets nke spain ma ọ bụ ọkpụkpụ pelvic, mmerụ enamel ezé na spasmophilia, nke na-etolite na vitamin r-adịghị ụkọ, adịghị ahụkebe.

Ndị ọrịa kwesịrị ikpebi ọkwa nke calcium, phosphates, alkaline phosphatase na 1,25-dihydroxyvitamin D na HPT na ọbara, yana urinary phosphate excretion. Site na rickets hypophosphatemic, ogo phosphates na ọbara ọbara na-ebelata, mana ntụpọ ha na mmamịrị dị elu. Ọkwa calcium na PTH bụ ihe dị mma, alkaline phosphatase na-ebukarị elu. Site na rickets calcium na-adịghị ike, achọpụtala ihu abụọ, enweghị hypophosphatemia ma ọ bụ na ọ dị nro, mwepụ nke phosphates na mmamịrị adịghị abawanye.

Achọpụtala hypophosphatemia n'ime nwa amụrụ ọhụrụ. N'ime afọ 1-2 nke ndụ, akara ngosipụta nke ọrịa ahụ na - ebulite: nlọghachi azụ nke ibu, nrụrụ siri ike nke nsọtụ ala. Akwara isi dị nro ma ọ bụ anọghị ya. Otu aka dị mkpụmkpụ bụ ihe e ji mara ya. N'ime ndị okenye, osteomalacia ji nwayọọ nwayọọ.

Ka ọ dị ugbu a, akọwagola ụdị ọrịa 4 ekerede na hypophosphatemic rickets.

Idị nke m - hypophosphatemia ejikọtara ya - rickets vitamin D na-egbochi (hypophosphatemic tubulopathy, hypophosphatemia ezinụlọ, hepatorial phosphate renal, ọrịa shuga phosphate na-arịa, ọrịa shuga phosphate na-adịgide adịgide, ọrịa akwara renubu tubular rickets, ọrịa Albert Blairt) n'ihi mbelata phosphate reabsorption na proximal renal tubule ma gosipụtara site na hyperphosphaturia, hypophosphatemia na mmepe nke rickets-dị ka mgbanwe ndị na-eguzogide ọgwụ ị na-egbochi vitamin. D.

A na-eche na ya na hypophosphatemic rickets nwere njikọ X, a na-agbaghazi usoro ọrụ 1-a-hydroxylase na phosphate, nke na-egosi ntụpọ na njikọta vitamin D metabolite 1.25 (OH) 2D3. Ntinye uche nke l, 25 (OH) 2D3 n'ime ndị ọrịa belatara nke ọma maka ogo hypophosphatemia dị adị.

Ọrịa ahụ gosipụtara onwe ya ruo afọ 2 nke ndụ. Njirimara kachasị ama:

uto nke ukwu, squat, ike akwara dị ukwuu, enweghi hypoplasia nke enamel nke ezé na-adịgide adịgide, mana enwere oghere nke oghere pulp, alopecia,
hypophosphatemia na hyperphosphaturia nwere calcium ọbara nkịtị wee mee ka ọrụ alkaline phosphatase mụbaa,
nrụrụ ụkwụ siri ike (na mmalite ije),
Rickets-X-ray mgbanwe dị n’ọkpụkpụ - diaphysis sara mbara nke nwere okpukpu abụọ nke cortical, usoro siri ike nke trabeculae, osteoporosis, nrụrụ vagal nke akụkụ ala, igbu oge n’ọkpụkpụ akpụ, ngụkọta mkpụrụ nke calcium na ọkpụkpụ na-abawanye.

Ntughari ozo nke phosphates n’ime akụrụ na-agbadata ruo 20-30% ma ọ bụ ihe na-erughị, mwepu nke phosphorus na mmamịrị na-abawanye ruo 5 g / ụbọchị, ọrụ nke alkaline phosphatase na-abawanye (ugboro 2-4 ma e jiri ya tụnyere usoro). Hyperaminoaciduria na glucosuria bụ ndị a na-amachaghị. A naghị agbanwe ntụgharị Calcium.

Enwere ụdị ọgwụ 4 na ọgwụ kemịkal dị iche iche nke ọrịa shuga phosphate dị ka mmeghachi omume na ntinye nke vitamin D. Na mgbanwe mbu, mmụba na ọdịnaya nke inorganic phosphates na ọbara n'oge ọgwụgwọ na-esonye na mmụba nke reabsorption ha na tubules renal, na nke abụọ, phosphate reabsorption na akụrụ na eriri afọ na-abawanye, - ịbawanye ụba na-emegharị naanị na eriri afọ, na nke anọ, a na-amụbasi ike na vitamin D, nke mere na ọbụna obere doses nke vitamin D na-akpata ihe ọ signsụ ofụ na-egbu egbu.

IIdị nke II - ụdị hypophosphatemic rickets - bụ ihe na - achịkwa onwe ya, ejikọghị ya na ọrịa chromosome. Ihe eji mara oria a:

mmalite nke ọrịa mgbe ọ dị afọ 1-2,
ịkpụ ụkwụ ya na mmalite ije, ma na-enweghị ịgbanwee elu, anụ ahụ siri ike, nrụrụ ọkpụkpụ.
hypophosphatemia na hyperphosphaturia nwere ọkwa calcium nkịtị yana mmụba na-arụ ọrụ alkaline phosphatase dị ala,
X-ray: ihe nrịba ama nke rickets, mana osteomalacia siri ike.

Onweghi mgbanwe n’edeputa electrolytes, CBS, nmeghari nke homonụ parathyroid, ihe mejupụtara amino acids ọbara, ọkwa nke creatinine, na nitrogen residual na serum. Mgbanwe mmamịrị bụ ihe anaghị arụ ọrụ.

IIIdị nke III - ịdabere na mmanu nri sitere na vitamin D (agarị agbụrụ, osteomalacia, hypophosphatemic vitamin D-rickets na aminoaciduria). Ihe kpatara ọrịa ahụ bụ imebi usoro nke 1.25 (OH) 2D3 n’ime akụrụ, nke na-eduga n’inwe nrịsị nke calcium na eriri afọ na nsonaazụ vitamin D na-anabata ndị na-anabata ọkpụkpụ, ihu abụọ, hyperaminoaciduria, hyperparathyroidism nke abụọ, ọrịa phosphorus reabsorption na hypophosphatemia.

Mmalite nke ọrịa ahụ na-ezo aka afọ 6 ọnwa. rue afọ abụọ Kachasị mara akara:

mgbaghara, hypotension, ihe ọdịdọ,
ihu abụọ, hypophosphatemia, hyperphosphaturia na ụba ọrụ nke alkaline phosphatase n'ime ọbara. Ọ na-abawanye site na hormone plasma parathyroid na-agbatị, a na-ahụkwa aminoaciduria na ntụpọ, mgbe ụfọdụ ntụpọ urinary acidification,
Ọgwụ mmalite nke ije ije, ịsụ ngọngọ, mmebi ngwa ngwa, akwara ike, enamel hypoplasia, nsogbu ezé,
X-ray kpughere rickets siri ike na mpaghara etolite ogologo ọdụ ụfụ, ụrọ nke cortical layer, ọchịchọ nke osteoporosis. Enweghị mgbanwe na CBS, ọdịnaya nke nitrogen residual, mana itinye uche nke l, 25 (OH) 2D3 n'ime ọbara na-ebelata nke ọma.

IVdị nke anọ - vitamin D3 - na-eketa n'ụkpụrụ nnwekọrịta akpaaka ma ọ bụ na-eme ya na mberede, ụmụ nwanyị na-arịakarị ọrịa. The mmalite nke ọrịa a chọpụtara na nwata, ya mara:

Ọkpụkpụ ụkwụ, nrụrụ ọkpụkpụ, akpụ akpụ,
alopecia ugboro ugboro na oge ụfọdụ mgbe ezighi ike.
X-ray kpughere rickets nke ogo dị iche iche.

Nchọpụta ọrịa shuga phosphate

Otu n'ime akara eji eche na ọrịa shuga phosphate bụ enweghị ike nke etu vitamin vitamin (2000-5000 IU / ụbọchị) dị na nwatakịrị na-arịa Rickets. Agbanyeghị, okwu ahụ bụ “vitamin Rick-Rickets na-eguzogide ọgwụ vitamin”, nke a na-akpọbubu ọrịa shuga phosphate, ezighi ezi.

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Ọrịa dị iche iche banyere ọrịa shuga phosphate

Ọ dị mkpa iji ọdịiche dị n'ọrịa shuga na-akpata ọrịa shuga na vitamin D, na-enye onwe ya nke ọma na ọgwụgwọ siri ike, ọrịa de Toni-Debre-Fanconi, osteopathy na ọdịda akụrụ.

Mgbe ihe mgbaàmà nke ọrịa shuga phosphate pụtara na nke mbụ okenye, a ga-ewere oncogenic hypophosphatemic osteomalacia. A na-ahụ ụdị mgbanwe a nke ọrịa paraneoplastic n'ọtụtụ etuto ahụ, gụnyere anụ ahụ (ọtụtụ dysplastic nevi).

Ihe na nsonaazụ na nsogbu enwere ike

Phosphate ọrịa shuga bụ nnukwu ọrịa nke a na-agaghị eleghara anya. Ma ọ bụghị ya, achọghị ya, yana nsogbu ndị ọzọ dị ize ndụ nwere ike ịmalite.

Ndị a gụnyere:

Ọkpụkpụ na-agbagha, ọnya ahụ nwere ike ịrụ ọrụ ma ọ bụrụ na mmadụ nwere ọrịa phosphate ọrịa shuga n’oge ọ bụ nwata.
Nwatakịrị nwere oria yiri ya na-alakarị azụ na mmepe (ma n'uche ma anụ ahụ).
Ọrịa nwere ike nwee nkwarụ n'ihi ọrịa articular na nrụrụ ọkpụkpụ ma ọ bụrụ na ọgwụgwọ adịghị.
A na-emebi okwu na usoro ezé ezé nwa a na-emebi.
A na - ekpughere usoro ọmụmụ nke enamel.
Ndị ọrịa nwere ike ịnwe ịnụ ntị n'ihi mmepe nke ọkpụkpụ ntị nke etiti.
E nwere ihe ize ndụ nke nephrocalcinosis. Ihe kpatara oria a bu itinye ahihia nnu n’ime akuru, bu nke n’enye ya ike ibute oria.
Ọrịa shuga Phosphate, nke a chọpụtara n’ime ụmụ nwanyị, nwere ike ịmebi usoro ọmụmụ nwa ma mekwaa akụkụ akwara.

Nsonaazụ nke ọrịa a n’enweghi ọgwụgwọ zuru oke ka dị ndụ. N ’ihu, ubara nke phosphate na-egosi na-eto eto site n’ọ̀dị ala na agị ụkwụ.

Mgbochi Ọrịa

Ọrịa shuga phosphate bụ ọrịa a na-ekesa n'ihe fọrọ nke nta ka ọ bụrụ n'ọnọdụ niile site n'aka ndị nne na nna na-arịa ọrịa na ụmụaka. Ọ bụrụ na ọdịdị ya sitere n’ọrịa sitere n’ọmụmụ, mgbe ahụ mmadụ ma ọ bụ dọkịta ọ bụla ruru eru agaghị enwe ike ime ka mmepe ya pụọ ma wezuga ihe ize ndụ nke ọrịa.

Mgbochi nke usoro ogwu a bu iji gbochie omume nke nsonaazụ na ibelata ihe ize ndụ nke nrụrụ ọkpụkpụ na ndị ọrịa nke afọ dị iche iche.

Ihe ndị a gụnyere ndị a:

Ọ dị mkpa ka ndị nne na nna ghara ileghara ihe mgbaàmà mbụ nke ọrịa ahụ anya. Ọ dị mkpa ịkpọtụrụ onye ọkachamara ozugbo maka enyemaka iji chọpụta ọrịa shuga phosphate n'oge mmalite nke mmepe wee malite usoro ọgwụgwọ kwesịrị ekwesị.
Mgbe nile lelee ụmụaka nwere ọrịa dị otú ahụ na endocrinologist na pediatrician.
Nyochaa mkpụrụ ndụ ihe ọmụmụ wee mee nyocha dị mkpa n'ọkwa atụmatụ ime ime maka ezinaụlọ ọ bụla nke ezigbo ndị ikwu nwere ụdị ọrịa a na nwata. Nke a ga - eme ka ndị nne na nna mata ihe ọghọm na nsogbu ndị nwa a na - amụbeghị amụ nwere ike iji nwee ike ịmalite ịgwọ ọrịa n’oge a.