I-Pseudohypoparathyroidism

I-Pseudohypoparathyroidism (PHP) sisifo esingofuzo apho umzimba usilela ukuphendula kwihomoni yeparadroid.

Imeko enxulumene noko yi-hypoparathyroidism, apho umzimba ungenzi ngokwaneleyo i-hormone yeparathyroid.

Amadlala e-parathyroid avelisa i-hormone ye-parathyroid (PTH). I-PTH inceda ukulawula i-calcium, i-phosphorus, kunye ne-vitamin D kumanqanaba egazini kwaye kubalulekile kwimpilo yethambo.

Ukuba unayo i-PHP, umzimba wakho uvelisa isixa esifanelekileyo se-PTH, kodwa "uyala" kwisiphumo sayo. Oku kubangela amanqanaba e-calcium ephantsi kunye namazinga aphezulu e-phosphate.

I-PHP ibangelwa yimfuza engaqhelekanga. Kukho iintlobo ezahlukeneyo ze-PHP. Zonke iifom zinqabile kwaye zihlala zifunyaniswa ebuntwaneni.

• Uhlobo lwe-1a lufunyenwe njengelifa ngendlela ebalaseleyo ye-autosomal. Oko kuthetha ukuba ngumzali omnye kuphela ofuna ukukudlulisela kofuzo olungalunganga ukuze ubenayo imeko. Ikwabizwa ngokuba yiAlbright i-osteodystrophy yelifa. Imeko ibangela ubude obufutshane, ubuso obujikelezileyo, ukutyeba, ukulibaziseka kophuhliso, kunye namathambo esandla amafutshane. Iimpawu zixhomekeke ekubeni ungalifumana njengelifa na uhlobo kumama okanye kutata wakho.
• Uhlobo lwe-1b lubandakanya ukunganyangeki kwi-PTH kuphela kwizintso. Okuncinci kuyaziwa ngohlobo 1b kunohlobo 1a. I-calcium egazini isezantsi, kodwa azikho ezinye zeempawu zeAlbright ye-osteodystrophy yelifa.
• Uhlobo lwe-2 lukwabandakanya i-calcium esezantsi kunye nenqanaba eliphezulu le-phosphate yegazi. Abantu abanale fomu abanazo iimpawu zomzimba eziqhelekileyo kubantu abanohlobo 1a. Ukungaqheleki kwemfuza okubangela ukuba kungaziwa. Kwahlukile kuhlobo 1b kwindlela izintso eziphendula ngayo kumanqanaba aphezulu e-PTH.

Iimpawu zihambelana nenqanaba eliphantsi le-calcium kwaye zibandakanya:

• Iimpawu
• Iingxaki zamazinyo
• Ubumdaka
• Ukuxhuzula
• I-Tetany (ingqokelela yeempawu kubandakanya ukujija kwemisipha kunye neenyawo kunye neenyawo zeenyawo kunye nokuqaqamba kwezihlunu)

Abantu abaneAlbright ye-osteodystrophy banokuba nezi mpawu zilandelayo:

• I-calcium ibeka phantsi kwesikhumba
• Iimpazamo ezinokuthi zithathe indawo yamaqhina kwiminwe echaphazelekayo
• Ubuso obujikelezayo kunye nentamo emfutshane
• Amathambo esandla amafutshane, ngakumbi ithambo elingaphantsi komnwe we-4
• Ukuphakama okufutshane

Uvavanyo lwegazi luya kwenziwa ukukhangela i-calcium, i-phosphorus, kunye namanqanaba e-PTH. Unokufuna iimvavanyo zomchamo.

Olunye uvavanyo lunokubandakanya:

• Uvavanyo lwemfuzo
• Intloko ye-MRI okanye i-CT yokuskena kwengqondo

Umboneleli wakho wezempilo uya kuncoma i-calcium kunye no-vitamin D wokuncedisa ukugcina inqanaba elifanelekileyo le-calcium. Ukuba inqanaba le-phosphate yegazi liphezulu, kungafuneka ulandele ukutya okuphantsi kwe-phosphorus okanye uthathe amayeza abizwa ngokuba yi-phosphate binders (njenge calcium carbonate okanye calcium acetate). Unyango luhlala luhlala ixesha elide.

I-calcium esezantsi yegazi kwi-PHP ihlala ibuthathaka kunezinye iindlela zehypoparathyroidism, kodwa ubukhulu beempawu zokwahluka phakathi kwabantu abohlukeneyo.

Abantu abane-1a PHP banethuba lokuba nezinye iingxaki zenkqubo ye-endocrine (enje nge-hypothyroidism kunye ne-hypogonadism).

I-PHP inokuqhagamshelwa kwezinye iingxaki zehomoni, zikhokelela kwi:

• Ukuqhuba ngesondo okuphantsi
• Ukukhula kancinci kwezesondo
• Amanqanaba amandla aphantsi
• Ukutyeba kwakho

Nxibelelana nomboneleli wakho ukuba wena okanye umntwana wakho unazo naziphi na iimpawu zenqanaba eliphantsi le-calcium okanye i-pseudohypoparathyroidism.

Albright ilifa osteodystrophy; Iindidi 1A kunye 1B pseudohypoparathyroidism; I-PHP

• Amadlala endocrine
• Izibilini zeParathyroid

I-Bastepe M, iJuppner H. Pseudohypoparathyroidism, i-Albright ye-osteodystrophy yelifa, kunye ne-heteroplasia e-osseous eqhubekayo: iingxaki ezibangelwa kukungasebenzi kweenguqu ze-GNAS. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, ii-eds. Endocrinology: Abantu abadala kunye nabantwana. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 66.

UDoyle DA. I-Pseudohypoparathyroidism (Albright ifa le-osteodystrophy). Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 590.

I-Thakker RV. Amadlala parathyroid, hypercalcemia kunye hypocalcemia. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl 232.