Pseudohypoparathyroidism

Pseudohypoparathyroidism (PHP) cuta ce ta kwayar halitta wacce jiki ya kasa amsawa ga kwayar parathyroid.

Halin da yake da alaƙa shine hypoparathyroidism, wanda cikin jiki baya yin isasshen hormone parathyroid.

Glandan parathyroid suna samar da parathyroid hormone (PTH). PTH yana taimakawa sarrafa alli, phosphorus, da bitamin D a cikin jini kuma yana da mahimmanci ga lafiyar ƙashi.

Idan kana da PHP, jikinka yana samar da adadin PTH daidai, amma yana "jurewa" ga tasirin sa. Wannan yana haifar da ƙananan ƙwayoyin calcium da matakan jini mai yawa.

PHP yana faruwa ne ta hanyar ƙwayoyin cuta marasa kyau. Akwai nau'ikan PHP daban-daban. Duk nau'ikan ba safai ba kuma galibi akan gano su tun suna yara.

• Nau'in 1a ana gadon shi ta hanyar mamaye ta atomatik. Wannan yana nufin iyaye ɗaya ne ke buƙatar wuce muku lalatacciyar hanyar don ku sami yanayin. Haka kuma ana kiranta Albright hereditary osteodystrophy. Yanayin yana haifar da gajarta, zagaye fuska, kiba, jinkirin girma, da gajerun hannuwa. Kwayar cutar ta dogara ne akan ko zaka gaji kwayar halitta daga mahaifinka ko mahaifinka.
• Nau'in 1b ya ƙunshi juriya ga PTH kawai a cikin kodan. Kadan aka sani game da nau'in 1b fiye da nau'in 1a. Calcium a cikin jini ƙanƙan ne, amma babu ɗayan sauran sifofin halayyar Albright wanda aka gada osteodystrophy.
• Nau'in na 2 kuma ya ƙunshi ƙananan ƙwayar calcium da matakan jini mai yawa. Mutanen da ke da wannan nau'in ba su da halaye na zahiri ga mutanen da ke da Type 1a. Ba a san rashin lafiyar kwayar halitta da ke haifar da ita ba. Ya bambanta da Nau'in 1b na yadda koda ke amsawa ga matakan PTH masu girma.

Kwayar cututtukan suna da alaƙa da ƙananan matakin alli kuma sun haɗa da:

• Ciwon ido
• Matsalar hakori
• Numfashi
• Kamawa
• Tetany (tarin cututtukan ciki har da jijiyoyin jijiyoyi da ciwon hannu da ƙafa da jijiyoyin tsoka)

Mutanen da ke da cututtukan osteocystrophy na Albright na iya samun alamun bayyanar masu zuwa:

• Adadin Calcium ƙarƙashin fata
• Dimples da ke iya maye gurbin ƙusoshin hannu a kan yatsun da abin ya shafa
• Zagaye fuska da gajeriyar wuya
• Gajeren ƙasusuwa, musamman ƙashin da ke ƙasa da yatsa na 4
• Gajeren gajere

Za ayi gwajin jini don a duba alli, phosphorus, da matakan PTH. Hakanan zaka iya buƙatar gwajin fitsari.

Sauran gwaje-gwaje na iya haɗawa da:

• Gwajin kwayoyin halitta
• Shugaban MRI ko CT scan na kwakwalwa

Mai ba ku kiwon lafiya zai ba da shawarar alli da bitamin D kari don kula da matakin alli mai dacewa. Idan matakin phosphate na jini ya yi yawa, maiyuwa kana bukatar ka bi tsarin cin abinci mai saurin-phosphorus ko kuma shan magunguna da ake kira masu dauke da sinadarin phosphate (kamar su sinadarin calcium carbonate ko calcium acetate). Jiyya yawanci tsawon rai ne.

Calciumarancin alli a cikin PHP yawanci yana da sauki fiye da sauran nau'ikan hypoparathyroidism, amma tsananin alamun zai iya bambanta tsakanin mutane daban-daban.

Mutanen da ke da nau'in 1a PHP suna iya samun wasu matsalolin tsarin endocrin (kamar su hypothyroidism da hypogonadism).

PHP na iya haɗawa da wasu matsalolin hormone, wanda ya haifar da:

• Sexarfin jima'i
• Ci gaban jima'i a hankali
• Energyananan matakan makamashi
• Karuwar nauyi

Tuntuɓi mai ba da sabis idan kai ko yaronka suna da alamun alamun ƙarancin alli ko ƙeta.

Albright na gado osteodystrophy; Nau'o'in 1A da 1B pseudohypoparathyroidism; PHP

• Endocrine gland
• Parathyroid gland

Bastepe M, Juppner H. Pseudohypoparathyroidism, Albod's osteodystrophy na gado, da kuma ci gaba mai girma heteroplasia: rikicewar da ke haifar da rashin maye gurbin GNAS. A cikin: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Manya da Yara. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016: babi na 66.

Doyle DA. Pseudohypoparathyroidism (Albright wanda ya gaji osteodystrophy). A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi 590.

Thakker RV. Kwayoyin parathyroid, hypercalcemia da hypocalcemia. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 232.