Pseudohypoparathyroidism

Pseudohypoparathyroidism (PHP) ke lefu la lefutso leo 'mele o sitang ho arabela ho hormone ea parathyroid.

Boemo bo amanang le sena ke hypoparathyroidism, eo 'mele o sa etseng hormone e lekaneng ea parathyroid.

Litšoelesa tsa parathyroid li hlahisa hormone ea parathyroid (PTH). PTH e thusa ho laola maemo a calcium, phosphorus, le vithamine D maling mme e bohlokoa bakeng sa bophelo bo botle ba masapo.

Haeba u na le PHP, 'mele oa hau o hlahisa palo e nepahetseng ea PTH, empa e "hanela" phello ea ona. Sena se baka maemo a tlase a mali a calcium le phosphate e phahameng ea mali.

PHP e bakoa ke liphatsa tsa lefutso tse sa tloaelehang. Ho na le mefuta e fapaneng ea PHP. Mefuta eohle ha e fumanehe hangata 'me hangata e fumanoa bongoaneng.

• Mofuta oa 1a o futsitsoe ka mokhoa o ka sehloohong oa likoloi. Seo se bolela hore ke motsoali a le mong feela ea hlokang ho u fetisetsa liphatsa tsa lefutso tse fosahetseng hore u be le boemo boo. E boetse e bitsoa Albright lefutso osteodystrophy. Boemo bona bo baka seemo se sekhuts'oane, sefahleho se chitja, botenya, tieho ea kholo, le masapo a matsoho a makhuts'oane. Matšoao a ipapisitse le hore na o futsitse 'm'ao kapa ntate oa hau.
• Mofuta oa 1b o kenyelletsa ho hanyetsa PTH feela liphio. Ho tsejoa hanyane ka mofuta oa 1b ho feta mofuta oa 1a. K'halsiamo maling e tlase, empa ha ho na litšobotsi tse ling tsa Albright lefutso la lefu la masapo.
• Mofuta oa 2 o boetse o kenyelletsa khalsiamo e tlase ea mali le maemo a phahameng a phosphate ea mali. Batho ba nang le foromo ena ha ba na litšobotsi tse tloaelehileng ho batho ba nang le Mofuta oa 1a. Ho tsebahala ho sa tloaelehang hoa lefutso ho e bakang ha ho tsejoe. E fapane le Mofuta oa 1b ka moo liphio li arabelang maemong a phahameng a PTH.

Matšoao a amana le khalsiamo e tlase mme a kenyelletsa:

• Likotsi
• Mathata a meno
• Boikitlaetso
• Ho oa
• Tetany (pokello ea matšoao a kenyeletsang ho sotha ha mesifa le likerempe tsa matsoho le maoto le mesifa)

Batho ba nang le lefu la osteodystrophy ea Albright ba ka ba le matšoao a latelang:

• Li-calcium li beha tlas'a letlalo
• Li-dimples tse ka nkelang li-knuckle menoaneng e amehileng
• Sefahleho se chitja le molala o mokhutšoane
• Masapo a matsoho a makhuts'oane, haholo lesapo le ka tlasa monoana oa bone
• Bolelele bo bolelele

Ho tla etsoa liteko tsa mali ho lekola calcium, phosphorus le PTH. U kanna ua hloka liteko tsa moroto.

Liteko tse ling li ka kenyelletsa:

• Ho etsa liteko tsa lefutso
• Hlooho ea MRI kapa CT ea boko

Mofani oa hao oa tlhokomelo ea bophelo o tla khothaletsa calcium le vithamine D ho tlatselletsa ho boloka boemo bo nepahetseng ba calcium. Haeba boemo ba phosphate ea mali bo phahame, o kanna oa hloka ho latela phepo e tlase ea phosphorus kapa ho noa meriana e bitsoang phosphate binders (joalo ka calcium carbonate kapa calcium acetate). Kalafo hangata e nka bophelo bohle.

K'halsiamo e tlase ea mali ho PHP hangata e bobebe ho feta mefuta e meng ea hypoparathyroidism, empa ho tiea ha matšoao ho ka fapana pakeng tsa batho ba fapaneng.

Batho ba nang le mofuta oa 1a PHP ba na le monyetla oa ho ba le mathata a mang a endocrine system (joalo ka hypothyroidism le hypogonadism).

PHP e kanna ea hokahanngoa le mathata a mang a li-hormone, e ka baka:

• Koloi e tlase ea thobalano
• Khōlo e potlakileng ea thobalano
• Maemo a tlase a matla
• Boima ba 'mele

Ikopanye le mofani oa hau haeba uena kapa ngoana oa hau le na le matšoao a boemo bo tlase ba calcium kapa pseudohypoparathyroidism.

Albright lefutso osteodystrophy; Mefuta ea 1A le 1B pseudohypoparathyroidism; PHP

• Litšoelesa tsa endocrine
• Litšoelesa tsa Parathyroid

Bastepe M, Juppner H. Pseudohypoparathyroidism, lefu la lefu la lefu la Albright, le lefu la heteroplasia e tsoelang pele: mathata a bakoang ke ho thibela liphetoho tsa GNAS. Ka: Jameson JL, De Groot LJ, oa Kretser DM, et al, eds. Endocrinology: Batho ba baholo le bana. La 7th. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 66.

DA ea Doyle. Pseudohypoparathyroidism (Albright lefutso osteodystrophy). Ka: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 590.

Thakker RV. Litšoelesa tsa parathyroid, hypercalcemia le hypocalcemia. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 232.