Pseudohypoparathyroidism

Pseudohypoparathyroidism (PHP) chirwere chemajini umo muviri unokundikana kupindura kune parathyroid hormone.

A mamiriro ane hukama ndeye hypoparathyroidism, umo muviri hauite yakakwana parathyroid hormone.

Iwo maronda eparathyroid anogadzira parathyroid hormone (PTH). PTH inobatsira kudzora calcium, phosphorus, uye vhitamini D mazinga muropa uye zvakakosha kune hutano hwemabhonzo.

Kana iwe uine PHP, muviri wako unoburitsa huwandu hwakaenzana hwePTH, asi "husingabatike" kune zvainoita. Izvi zvinokonzeresa yakaderera ropa calcium calcium uye yakakwira ropa phosphate mazinga

PHP inokonzerwa nemaitiro asina kujairika. Kune mhando dzakasiyana dzePHP. Ese mafomu haashome uye anowanzo kuongororwa muhudiki.

• Type 1a inogarwa nhaka nenzira ye autosomal ine mukurumbira. Izvi zvinoreva kuti mubereki mumwe chete ndiye anoda kukupfuudza iro geni risiri iro kuti uve nemamiriro acho. Iyo inonzi zvakare Albright nhaka osteodystrophy. Mamiriro acho anokonzeresa kupfupika, kutenderera kumeso, kufutisa, kunonoka kukura, uye mapfupa emaoko mapfupi. Zviratidzo zvinoenderana nekuti iwe unowana nhaka kubva kuna mai vako here kana baba vako.
• Type 1b inosanganisira kuramba PTH chete muitsvo. Chishoma chinozivikanwa nezve mhando 1b pane mhando 1a. Karusiyamu muropa yakaderera, asi hapana chimwe chehumwe hunhu hweAlbright nhaka nhaka osteodystrophy.
• Rudzi rwechipiri zvakare runosanganisira yakaderera ropa calcium uye yakakwira ropa phosphate mazinga. Vanhu vane fomu iyi havana hunhu hwepanyama hwakajairika kune vanhu vane Type 1a. Iko kusarongeka kwemajini kunokonzera hazvizivikanwe. Izvo zvakasiyana neRudzi 1b mune iyo itsvo inopindura sei kumatunhu akakwirira ePTH.

Zviratidzo zvakabatana nehuremu hwepasi hwecalcium uye zvinosanganisira:

• Kurwara
• Matambudziko emazino
• Unhu
• Kufenda
• Tetany (muunganidzwa wezviratidzo zvinosanganisira mhasuru kumonyoroka uye ruoko uye tsoka tsoka uye mhasuru kupora)

Vanhu vane Albright nhaka osteodystrophy vanogona kuve nezviratidzo zvinotevera:

• Calcium inoisa pasi peganda
• Dimples ayo anogona kutsiva knuckles pane zvakakanganiswa minwe
• Kutenderera kumeso uye mutsipa mupfupi
• Mapfupi emaoko mapfupa, kunyanya pfupa pazasi pechina munwe
• Kureba kupfupi

Kuongororwa kweropa kuchaitwa kutarisa calcium, phosphorus, uye PTH mazinga. Iwe unogona zvakare kuda weti bvunzo.

Mimwe bvunzo inogona kusanganisira:

• Kuongorora genetic
• Musoro MRI kana CT scan yehuropi

Mutapi wako wezvehutano anokurudzira calcium uye vhitamini D zvinowedzera kuti uchengetedze yakakwana calcium huwandu. Kana iyo phosphate level iri kumusoro, ungangoda kutevera yakaderera-phosphorus kudya kana kutora mishonga inonzi phosphate binders (senge calcium carbonate kana calcium acetate). Kurapa kunowanzo kuve kwehupenyu-hurefu.

Yakaderera ropa calcium muPHP inowanzo kuve yakapusa kupfuura mune mamwe marudzi e hypoparathyroidism, asi kuomarara kwezviratidzo kunogona kuve kwakasiyana pakati pevanhu vakasiyana.

Vanhu vane mhando 1a PHP vanowanzo kuve nemamwe matambudziko endocrine system (senge hypothyroidism uye hypogonadism).

PHP inogona kunge yakabatana nemamwe matambudziko ehomoni, zvichikonzera:

• Yakadzika pabonde drive
• Kunonoka kukura pabonde
• Low simba mumatunhu
• Kurera huremu

Bata mupi wako kana iwe kana mwana wako muine chero zviratidzo zveiyo yakaderera calcium level kana pseudohypoparathyroidism.

Albright nhaka osteodystrophy; Mhando 1A uye 1B pseudohypoparathyroidism; PHP

• Endocrine masero
• Zvirwere zveparathyroid

Bastepe M, Juppner H. Pseudohypoparathyroidism, Albright's nhaka nhaka osteodystrophy, uye inofambira mberi osseous heteroplasia: kusagadzikana kunokonzerwa nekumisa shanduko yeGNAS Muna: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Vakuru uye Chiremba. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016: chitsamba 66.

Doyle DA. Pseudohypoparathyroidism (Albright nhaka osteodystrophy). Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chitsiko 590.

Thakker RV. Izvo zvipenga zveparathyroid, hypercalcemia uye hypocalcemia. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsiko 232.