Pseudohypoparathyroidism

Pseudohypoparathyroidism (PHP) ndi matenda amtundu womwe thupi limalephera kuyankha mahomoni amanjenje.

Matenda ena oterewa ndi hypoparathyroidism, momwe thupi limapangira mahomoni osakwanira.

Matenda a parathyroid amapanga mahomoni otchedwa parathyroid (PTH). PTH imathandiza kuchepetsa calcium, phosphorus, ndi mavitamini D m'magazi ndipo ndizofunikira pa thanzi la mafupa.

Ngati muli ndi PHP, thupi lanu limapanga kuchuluka kwa PTH, koma "sikulimbana" ndi zotsatira zake. Izi zimayambitsa kuchepa kwa calcium m'magazi komanso kuchuluka kwa phosphate yamagazi.

PHP imayambitsidwa ndi majini achilendo. Pali mitundu yosiyanasiyana ya PHP. Mitundu yonse ndiyosowa ndipo imapezeka ali mwana.

• Type 1a imalandiridwa mwanjira yodziwika kwambiri ya autosomal. Izi zikutanthauza kuti kholo limodzi lokha liyenera kukupatsirani jini yolakwika kuti mukhale ndi vutoli. Amatchedwanso Albright cholowa cha osteodystrophy. Vutoli limayambitsa kukula kwakanthawi, nkhope yozungulira, kunenepa kwambiri, kuchedwa kwakukula, ndi mafupa amfupi. Zizindikiro zimadalira ngati mumalandira cholowa kuchokera kwa amayi kapena abambo anu.
• Mtundu 1b umaphatikizapo kukana PTH kokha mu impso. Zochepa ndizodziwika pamtundu wa 1b kuposa mtundu 1a. Kashiamu m'magazi ndi otsika, koma palibe zina mwazomwe Albright cholowa cha osteodystrophy.
• Mtundu wachiwiri umaphatikizaponso kashiamu wotsika kwambiri wamagazi komanso ma phosphate ambiri. Anthu omwe ali ndi mawonekedwewa alibe mikhalidwe yodziwika kwa anthu omwe ali ndi Type 1a. Zovuta zachibadwa zomwe zimayambitsa sizidziwika. Ndizosiyana ndi mtundu 1b momwe impso zimayankhira pamitengo yayikulu ya PTH.

Zizindikiro zimakhudzana ndi kuchepa kwa calcium ndipo zimaphatikizapo:

• Kupunduka
• Mavuto amano
• Kunjenjemera
• Kugwidwa
• Tetany (zizindikiro zingapo kuphatikizapo kupindika kwa minofu ndi kukokana kwa manja ndi miyendo ndi kupindika kwa minofu)

Anthu omwe ali ndi Albright cholowa cha osteodystrophy atha kukhala ndi izi:

• Kalasi imayikidwa pansi pa khungu
• Zotupa zomwe zingalowe m'malo mwa zala zazala zakhudzidwa
• Nkhope yozungulira ndi khosi lalifupi
• Mafupa amfupi, makamaka fupa pansi pa chala chachinayi
• Kutalika kwakanthawi

Kuyezetsa magazi kudzachitika kuti muwone kuchuluka kwa calcium, phosphorus, ndi PTH. Mwinanso mungafunike kuyesa mkodzo.

Mayesero ena atha kuphatikizira:

• Kuyesedwa kwachibadwa
• Mutu wa MRI kapena CT waubongo

Wothandizira zaumoyo wanu amalangiza calcium ndi vitamini D zowonjezerapo kuti mukhale ndi calcium yokwanira. Ngati mulingo wa phosphate uli wokwera, mungafunikire kutsatira zakudya zopanda phosphorous kapena kumwa mankhwala otchedwa phosphate binders (monga calcium carbonate kapena calcium acetate). Chithandizo nthawi zambiri chimakhala chamoyo wonse.

Kashiamu wamagazi ochepa mu PHP nthawi zambiri amakhala ocheperako kuposa mitundu ina ya hypoparathyroidism, koma kuuma kwa zizindikilo kumatha kukhala kosiyana pakati pa anthu osiyanasiyana.

Anthu omwe ali ndi mtundu wa 1a PHP amakhala ndi zovuta zina zamagetsi (monga hypothyroidism ndi hypogonadism).

PHP ikhoza kulumikizidwa ndi mavuto ena a mahomoni, zomwe zingayambitse:

• Kuyendetsa kotsika
• Kukula pang'onopang'ono pakukula
• Mphamvu zochepa
• Kulemera

Lumikizanani ndi omwe amakupatsani ngati inu kapena mwana wanu muli ndi vuto lililonse la calcium kapena pseudohypoparathyroidism.

Albright cholowa cha osteodystrophy; Mitundu 1A ndi 1B pseudohypoparathyroidism; Pulogalamu ya PHP

• Matenda a Endocrine
• Matenda a Parathyroid

Bastepe M, Juppner H. Pseudohypoparathyroidism, Albright's cholowa osteodystrophy, ndi osseous heteroplasia: zovuta zomwe zimayambitsidwa chifukwa chotsegula kusintha kwa GNAS. Mu: Jameson JL, De Groot LJ, de Kretser DM, et al, olemba. Endocrinology: Akuluakulu ndi Ana. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier Saunders; 2016: chap 66.

Doyle DA. Pseudohypoparathyroidism (Albright cholowa osteodystrophy). Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 590.

Thakker RV. Matenda a parathyroid, hypercalcemia ndi hypocalcemia. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 232.