Matenda a Phosphate > Matenda a shuga

Matenda a shuga a Phosphate ndi matenda obadwa nawo omwe samagwirizanitsidwa ndi kuphwanya kwa insulini mu kapamba ndi kuchuluka kwa shuga m'magazi. Matendawa amayambitsidwa ndi kagayidwe kolakwika ka vitamini D, komanso ma phosphates. Pozindikira matenda a chifuwa chachikulu cha phosphate, palibe kusintha komwe kumayamwa zinthu izi m'matumbo aimpso, ndipo minyewa ya mafupa imadziwika ndi kapangidwe kolakwika ka mankhwala.

Kodi matenda a shuga a phosphate amawonekera bwanji mwa ana?

Hypophosphatemic rickets amayamba kuwonekera mwa ana adakali aang'ono. Nthawi zambiri, zizindikiro zoyambirira zimachitika mchaka choyamba kapena chaka chotsatira, mwana akangoyamba kuyenda pawokha. Matenda a shuga a Phosphate samakhudza mkhalidwe wamunthu wamba.

Pali kukula wobwerera.
Miyendo yokhotakhota.
Maondo ndi bondo ndi opindika.
Mafupa omwe amapezeka m'dera la malo olumikizirana mafupa amakhala otakata.
Kutsitsa kamvekedwe ka minofu.
Panthawi yamkamwa, kupweteka kumbuyo ndi mafupa kumamveka. Zowawa kwambiri zitha kupangitsa kuti mwanayo asiye kuyima payekha pamapazi ake.
Nthawi zina, zolakwika za enamel pamano, ma rickets a msana kapena mafupa a pelvic amawoneka.
Spasmophilia, yokhala ndi mtundu wa rickets wokhala ndi vitamini D, itha kukhala.
Miyendo ya wakhanda imafupikitsidwa (nthawi zambiri mosasokoneza).
Ndi zaka, wodwalayo amakula osteomalacia.
Zithunzi za X-ray zikuwonetsa kukula kwa mafupa, mafupa amapangika mochedwa.
Khansa yokhala ndi calcium yambiri m'mafupa.
Mapangidwe a ma elekitirodi, amino acid, creatinine, CBS sasintha.

Mwana amafunika kuchuluka kwazinthu zofunikira komanso zopatsa thanzi, kuphatikizapo phosphorous ndi calcium, kuti akule bwino ndi kukula. Kuperewera kwa zinthuzi m'zaka zoyambirira za moyo kumafotokozera ukulu wa matendawa.

Zosiyanasiyana mawonetsedwe a matendawa

Matenda a shuga a Phosphate, kutengera momwe angayambitsire vitamini D amawerengedwa motere:

Amadziwika ndi kuchuluka kwa mapangidwe anachilengedwe m'magazi, omwe adawoneka chifukwa chakuwonjezeka kwa reabsorption mu aimpso tubules.
Amadziwika ndi kuwonjezereka kwa phosphates m'matumbo ndi impso.
Reabsorption imakhala yowonjezereka m'matumbo okha.
Matenda a shuga a Phosphate amakhala ndi chidwi chokwanira cha vitamini D. Ngakhale kukula kwa zinthu izi kungayambitse kuledzera.

Zolemba zaukadaulo wazachipatala

Matenda a hepateral phosphate a shuga ndi gulu lotengera matenda obadwa nawo a phosphates ndi vitamini D. Hypophosphatemic rickets ndi matenda omwe amadziwika ndi hypophosphatemia, kuchepa kwa calcium komanso ma osteomalacia, osagwirizana ndi vitamini D. Zizindikiro zimaphatikizira kupweteka kwa mafupa, mafupa kukula. Kuzindikiritsa kumadalira kutsimikiza kwa serum phosphate, alkaline phosphatase ndi milingo 1,3-dihydroxyvitamin D3. Chithandizo chimaphatikizanso kumeza phosphates ndi calcitriol.

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Amayambitsa ndi pathogenesis a matenda a shuga a phosphate

Family hypophosphatemic rickets ndi cholowa ndi mtundu wothandizira wa X. Milandu ya sporadic yomwe yapezeka mwa hypophosphatemic rickets nthawi zina imalumikizidwa ndi ma benign mesenchymal tumors (oncogenic rickets).

Maziko a matendawa ndi kuchepa kwa kubwezeretsanso kwa ma phosphates mu proximal tubules, yomwe imatsogolera ku hypophosphatemia. Kusokonezeka kumeneku kumayamba chifukwa cha kufalikira kwa zinthu ndipo kumalumikizidwa ndi matenda oyamba mu ntchito ya osteoblast. Palinso kuchepa kwamatumbo oyamwa a calcium ndi phosphate. Kuchepa kwa minofu m'mitsempha kumachitika chifukwa chakuchepa kwambiri kwa phosphate komanso kusowa kwa mafupa a mafupa kuposa chifukwa chokhala ndi calcium yambiri komanso kuchuluka kwa mahomoni amtundu wa parathyroid pama ricores osowa calcium. Popeza mulingo wa 1,25-dihydroxycholecalciferol (1,25-dihydroxyvitamin D) ndi wabwinobwino kapena wochepetsedwa, cholakwika pakupanga mitundu ya Vitamini D chitha kulingaliridwa, mwachizolowezi hypophosphatemia iyenera kukulitsa kuchuluka kwa 1,25-dihydroxyvitamin D.

Hypophosphatemic rickets (phosphate shuga) imayamba chifukwa kuchepa kwa kubwezeretsa kwa phosphate mu proximal tubules. Kuwonongeka kwa tubular kumeneku kumawonedwa pakudzipatula, mtundu wa cholowa ndiwambiri, wolumikizidwa ndi X chromosome. Kuphatikiza apo, matenda a shuga a phosphate ndi imodzi mwazinthu za Fanconi syndrome.

Matenda a shuga a paraneoplastic phosphate amayamba chifukwa chopanga ma cell a parathyroid.

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Zizindikiro za Phosphate shuga

Hypophosphatemic rickets imawonekera ngati zovuta zingapo, kuchokera ku asymptomatic hypophosphatemia mpaka kuchepetsedwa kukula kwakuthupi komanso kukula kochepa mpaka ku chipatala cha ricores kapena osteomalacia. Mawonekedwe mu ana nthawi zambiri amasintha atayamba kuyenda, amakhala ndi mawonekedwe opindika a miyendo ndi kufowoka kwina kwa mafupa, mafinya a pseudo, kupweteka kwa mafupa komanso kufupika. Kukula kwa mafupa pamalo ophatikizika ndi minofu kumachepetsa kuyenda. Ndi ma hypophosphatemic rickets, ma reseches a msana kapena mafupa a m'chifuwa, zolakwika mu enamel ya mano ndi spasmophilia, omwe amapezeka ndi mavitamini osakwanira a Vitamin D, samadziwika.

Odwala ayenera kudziwa kuchuluka kwa calcium, phosphates, alkaline phosphatase ndi 1,25-dihydroxyvitamin D ndi HPT mu seramu, komanso urine phosphate excretion. Ndi ma hypophosphatemic rickets, mulingo wa ma phosphates mu seramu ya magazi amachepetsedwa, koma kutulutsa kwawo mkodzo ndikokwera. Serum calcium ndi PTH misinkhu yachilendo, ndipo alkaline phosphatase nthawi zambiri imakwezedwa. Ndi mataxi osakwanira calcium, hypocalcemia imadziwika, palibe hypophosphatemia kapena yofatsa, yowonetsa phosphates mu mkodzo siinakulidwe.

Hypophosphatemia wapezeka kale mwa wakhanda. Mu chaka cha 1-2 chaumoyo, zizindikiro za matendawa zimayamba: kukula kwakumapeto, kupunduka kwakukulu kwa malekezero apansi. Zofooka za minofu ndizofatsa kapena kulibe. Manja miyendo yochepa mosiyanasiyana. Akuluakulu, osteomalacia imayamba kukula.

Mpaka pano, mitundu inayi ya zovuta zobadwa nazo mu hypophosphatemic rickets zafotokozedwa.

Type I - X yolumikizana ndi hypophosphatemia - mavitamini D osagwirizana (hypophosphatemic tubulopathy, hypophosphatemia, cholowa m'magazi a impso, matenda a impso a phosphate, matenda abwinobwino a phosphate, matenda a renal tubular, matenda a Albert Blairt) chifukwa kuchepa kwa phosphate reabsorption mu proximal renal tubule ndikuwonetsedwa ndi hyperphosphaturia, hypophosphatemia ndi chitukuko cha ma protein ooneka ngati osagwirizana ndi mavitamini wamba D.

Amaganiziridwa kuti ndi ma X yogwirizanitsa ndi hypophosphatemic, kutsata kwa ntchito ya 1-a-hydroxylase yokhala ndi phosphate kumayipa, zomwe zimawonetsa kulephera mu kapangidwe ka vitamini D metabolite 1.25 (OH) 2D3. The kuchuluka kwa l, 25 (OH) 2D3 odwala amachepetsedwa moyenera kuchuluka kwa hypophosphatemia.

Matendawa amawonekera mpaka zaka ziwiri za moyo. Zizindikiro zodziwika bwino:

Kukula modabwitsa, squat, mphamvu yayikulu ya minofu, palibe hypoplasia ya enamel ya mano osatha, koma pali zowonjezera za malo a zamkati, alopecia,
Hypophosphatemia ndi hyperphosphaturia ndi magazi abwinobwino ndi kuchuluka kwamchere phosphatase ntchito,
miyendo yolimba (poyambira kuyenda),
Kusintha kwa ma X-ray m'mafupa - kupindika kambiri ndi makulidwe amtundu wa kotekisi, njira yoyipa ya trabeculae, mafupa am'mimba, kuchepa kwa nyini kwam'malo am'munsi, kuchedwa kwa mapangidwe a mafupa.

Kubwezeretsanso kwa phosphates mu impso kumatsika mpaka 20-30% kapena kuchepera, kuchuluka kwa phosphorous mumkodzo kumawonjezeka mpaka 5 g / tsiku, ntchito ya phosphatase yamchere imakulitsidwa (nthawi 2-4 poyerekeza ndi chizolowezi). Hyperaminoaciduria ndi glucosuria ndi zopanda pake. Kuchuluka kwa calcium sakusintha.

Pali mitundu inayi yamankhwala ndi michere yosiyanasiyana ya matenda am'mimba a phosphate malingana ndi momwe amayambira kuyambitsa kwa vitamini D. Pazosintha zoyambirira, kuwonjezeka kwa zomwe zimapangika mu phosphates m'magazi panthawi ya mankhwala zimagwirizanitsidwa ndi kuwonjezeka kwa kuphatikizanso kwa impso tubules, chachiwiri, kupatsidwanso kwa phosphate mu impso ndi matumbo kumalimbikitsidwa. - kuchuluka kubwezeretsa kumachitika m'matumbo okha, ndipo chachinayi, kumva kukoma kwa Vitamini D kumachulukitsidwa kwambiri, kotero kuti, ngakhale Mlingo wocheperako wa vitamini D umayambitsa zizindikiro za kuledzera.

Mtundu Wachiwiri - mawonekedwe a hypophosphatemic rickets - ndiwofatsa kwambiri, osalumikizidwa ndi matenda a X chromosome. Matendawa amadziwika ndi:

kumayambiriro kwa matendawa zaka 1-2,
kupindika kwa miyendo ndikuyamba kuyenda, koma popanda kusintha kutalika, thupi lamphamvu, kufooka kwa mafupa,
Hypophosphatemia ndi hyperphosphaturia yokhala ndi calcium mokwanira komanso kuwonjezeka kwapang'onopang'ono kwa ntchito zamchere za phosphatase,
X-ray: Zizindikiro zofatsa za rickets, koma ndi osteomalacia woopsa.

Palibe kusintha pama kapangidwe ka ma electrolyte, CBS, kuchuluka kwa mahomoni a parathyroid, kapangidwe ka magazi amino acid, kuchuluka kwa creatinine, ndi nitrogen yotsalira mu seramu. Zosintha mu mkodzo ndi zopanda pake.

Mtundu Wachitatu - kudalira kokhazikika kwa mavitamini D (ma hypocalcemic rickets, osteomalacia, hypophosphatemic vitamini D-amadalira ricores aminoaciduria). Zomwe zimayambitsa matendawa ndi kuphwanya mapangidwe a 1.25 (OH) 2D3 mu impso, zomwe zimayambitsa kulowetsedwa kwa calcium m'matumbo ndikuwonetsa mwachindunji Vitamini D pamatchulidwe amkati a mafupa, hypocalcemia, hyperaminoaciduria, yachiwiri hyperparathyroidism, phosphorous reabsorption ndi hypophosphatemia.

Kukhazikika kwa matendawa kumatanthauza zaka 6 zokha. mpaka zaka 2 Zizindikiro zodziwika bwino:

chisangalalo, hypotension, zopweteka,
hypocalcemia, hypophosphatemia, hyperphosphaturia ndi kuchuluka kwa zamchere phosphatase m'magazi. Imachulukitsidwa ndi plasma parathyroid hormone imagwiritsa ntchito, ndipo aminoaciduria ndi chilema chimawonedwanso, nthawi zina vuto la kwamikodzo poyerekeza,
kuyamba mochedwa kuyenda, kudodometsa, kufooka kwakula msanga, kufooka kwa minofu, enamel hypoplasia, kupweteka kwa mano,
X-ray idawululira zolemera zowopsa kumalo osungirako mafupa a tubular ataliitali, kuwonda kwa cortical wosanjikiza, chizolowezi cha mafupa. Palibe kusintha mu CBS, zomwe zimakhala zotsalira za nayitrogeni, koma ndende ya l, 25 (OH) 2D3 m'magazi imachepetsedwa kwambiri.

Mtundu IV - kuchepa kwa vitamini D3 - wobadwa mwanjira yopuma yokha kapena kumachitika mwapang'onopang'ono, atsikana amadwala kwambiri. Kukhazikika kwa matendawa kumadziwika paubwana, kumadziwika ndi:

kupindika kwa miyendo, kusinthika kwa mafupa, kukokana,
pafupipafupi alopecia ndipo nthawi zina dzino anomatic,
X-ray idawululira masheya angapo madigiri osiyanasiyana.

Kuzindikira matenda ashuga a phosphate

Chimodzi mwazolemba zomwe zimakayikira matenda a shuga a phosphate ndi kusakwanira kwa mulingo wovomerezeka wa vitamini D (2000-5000 IU / tsiku) mwa mwana yemwe ali ndi ricches. Komabe, liwu loti "Vitamini D loletsa", lomwe kale limagwiritsa ntchito matenda a chifuwa, silabwino kwenikweni.

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Kusiyanitsa mitundu ya matenda a shuga a phosphate

Ndikofunikira kusiyanitsa matenda obadwa nawo a phosphate a shuga ndi mavitamini osakwanira a vitamini D, omwe amadzichiritsa okha ku zovuta zovuta, de Toni-Debre-Fanconi syndrome, osteopathy yolephera aimpso.

Ngati zizindikiro za matenda a shuga a phosphate zikuchitika koyamba mwa munthu wamkulu, oncogenic hypophosphatemic osteomalacia iyenera kuganiziridwa. Kusiyanaku kwa paraneoplastic syndrome kumachitika m'matumbo ambiri, kuphatikiza khungu (multiple dysplastic nevi).

Zotsatira ndi zovuta zina

Matenda a shuga a Phosphate ndi matenda oopsa omwe sangathe kunyalanyazidwa. Kupanda kutero, mavuto osafunikira, komanso oopsa amatha.

Izi zikuphatikiza:

Kuyimitsidwa kumasokonezedwa, ndipo mafupa amatha kupunduka ngati munthu wadwala matenda a phosphate muubwana.
Mwana yemwe ali ndi matenda ofananawo nthawi zambiri amakhala kumbuyo atakula (mu zamaganizidwe ndi thupi).
Wodwala amatha kukhala wolumala chifukwa cha kupita patsogolo kwa kufowoka kwa mafinya komanso mafupa ngati chithandizo chokwanira sichikupezeka.
Mawu ndi kutsatizana kwa kanthu kena kamwana mano kumaphwanyidwa.
Pathology ya kapangidwe ka enamel imawululidwa.
Odwala amatha kutayikira makutu chifukwa cha kusayenda bwino kwa mafupa a khutu apakati.
Pali chiopsezo cha nephrocalcinosis. Matendawa amadziwika ndi kupezeka kwa mchere wamchere mu impso, zomwe zimapangitsa kuti ziwonetsedwe.
Matenda a shuga a phosphate, omwe amapezeka mwa azimayi, amatha kusokoneza mayendedwe a kubadwa ndikupanga gawo la cesarean.

Zotsatira za matendawa popanda kulandira chithandizo chokwanira zimakhala moyo wonse. Kunja, zovuta za matenda a shuga a phosphate zimawonetsedwa ndi kukula pang'ono ndi kupindika kwa miyendo.

Kuteteza Matenda

Matenda a shuga a Phosphate ndi matenda omwe amapatsira pafupifupi makolo onse odwala mpaka ana. Ngati mawonekedwe ake ali chifukwa cha chibadwa chamunthu, ndiye kuti munthu kapena dokotala aliyense woyenera sangathe kuyambitsa chitukuko ndikupatula ngozi ya matenda.

Kupewa kwa matenda amtunduwu makamaka kumathandizira kupewa zomwe zimachitika ndikuchepetsa chiwopsezo cha mapangidwe a mafupa kwa odwala azaka zosiyanasiyana.

Izi ndi monga:

Ndikofunika kuti makolo asaphonye zizindikiro zoyambirira za matendawa. Ndikofunika kulumikizana ndi katswiri kuti athandizidwe kuti adziwe matenda a shuga a phosphate m'mayambiriro a chitukuko ndikuyamba chithandizo choyenera.
Nthawi zonse muziwona ana omwe ali ndi matenda ku endocrinologist ndi dokotala wa ana.
Mufunseni ma genetic ndikukumununkhirani koyenera panthawi yakukonzekera banja lililonse lomwe abale ake apamtima adadwalanso mwana. Izi zipangitsa kuti makolo azindikire zoopsa komanso zovuta zomwe mwana wosabadwa angakhale nazo kuti akhale okonzeka kuyamba kulandira mankhwalawa munthawi yake.