Matenda a Peutz-Jeghers

Matenda a Peutz-Jeghers (PJS) ndimatenda achilendo pomwe zophukira zimapangidwa m'matumbo. Munthu amene ali ndi PJS ali pachiwopsezo chachikulu chotenga khansa.

Sizikudziwika kuti ndi anthu angati omwe akukhudzidwa ndi PJS. Komabe, National Institutes of Health imaganiza kuti zimakhudza pafupifupi 1 mwa 25,000 mpaka 300,000 obadwa.

PJS imayambitsidwa ndi kusintha kwa jini lotchedwa STK11 (kale limadziwika kuti LKB1). Pali njira ziwiri zomwe PJS ingalandire:

• PJS yodziwika bwino imabadwa kudzera m'mabanja ngati chikhalidwe chodziyimira payokha. Izi zikutanthauza kuti ngati m'modzi mwa makolo anu ali ndi PJS yamtunduwu, muli ndi mwayi wokhala ndi jini ndikukhala ndi matendawa.
• PJS yokhazikika sikutengera kwa kholo. Kusintha kwa majini kumachitika paokha. Munthu wina akatenga kusintha kwa majini, ana awo amakhala ndi mwayi wolandira cholowa mwa 50%.

Zizindikiro za PJS ndi izi:

• Mawanga a bulauni kapena otuwa pamilomo, m'kamwa, mkatikati mwa kamwa, ndi khungu
• Anadula zala kapena zala
• Kupweteka kwam'mimba
• Mdima wandiweyani pamilomo ya mwana komanso mozungulira
• Magazi pamalopo omwe amatha kuwoneka ndi maso (nthawi zina)
• Kusanza

Ma polyps amakula makamaka m'matumbo ang'onoang'ono, komanso m'matumbo akulu (colon). Kuyesedwa kwa colon komwe kumatchedwa colonoscopy kukuwonetsa ma polyp polyps. Matumbo aang'ono amayesedwa m'njira ziwiri. Imodzi ndi barium x-ray (matumbo ang'onoang'ono). Yina ndi kapisozi kotchedwa endoscopy, momwe kamera yaying'ono imamezedwa kenako ndikujambula zithunzi zambiri ikamadutsa m'matumbo ang'onoang'ono.

Mayeso owonjezera atha kuwonetsa:

• Gawo lina la m'matumbo limadzipukusa lokha (intussusception)
• Zotupa za Benign (noncancerous) m'mphuno, mpweya, ureters, kapena chikhodzodzo

Mayeso a labotale atha kuphatikiza:

• Kuwerengera kwathunthu kwa magazi (CBC) - kumatha kuwulula kuchepa kwa magazi
• Kuyesedwa kwachibadwa
• Chopondapo guaiac, kuyang'ana magazi mu chopondapo
• Kuchuluka kwazitsulo (TIBC) - kumatha kulumikizidwa ndi kuchepa kwa magazi m'thupi

Kuchita opaleshoni kungafunike kuchotsa ma polyp omwe amabweretsa mavuto kwakanthawi. Mavitamini a iron amathandizira kuthana ndi kutayika kwa magazi.

Anthu omwe ali ndi vutoli ayenera kuyang'aniridwa ndi othandizira azaumoyo ndikuwunika pafupipafupi ngati ali ndi khansa itasinthidwa.

Zida zotsatirazi zitha kupereka zambiri pa PJS:

• National Organisation for Rare Disways (NORD) - rarediseases.org/rare-diseases/peutz-jeghers-syndrome
• Buku Lofotokozera la NIH / NLM Genetics - ghr.nlm.nih.gov/condition/peutz-jeghers-syndrome

Pakhoza kukhala chiopsezo chachikulu kuti tizilombo ting'onoting'ono timakhala khansa. Kafukufuku wina amalumikiza PJS ndi khansa yam'mimba, m'mapapo, m'mawere, pachiberekero, ndi m'mimba mwake.

Zovuta zingaphatikizepo:

• Kusokoneza maganizo
• Mitundu yambiri yomwe imayambitsa khansa
• Ziphuphu zamchiberekero
• Mtundu wa zotupa zamchiberekero zotchedwa zotupa zogonana

Itanani kuti mudzakumane ndi omwe amakupatsani ngati inu kapena mwana wanu muli ndi zodwala. Kupweteka kwam'mimba kwambiri kumatha kukhala chisonyezo chazidzidzidzi monga kukhumudwa.

Upangiri wa chibadwa umalimbikitsidwa ngati mukukonzekera kukhala ndi ana ndikukhala ndi mbiri yabanja ya vutoli.

PJS

• Zakudya zam'mimba ziwalo

McGarrity TJ, Amosi CI, Baker MJ. Matenda a Peutz-Jeghers. Mu: Adam MP, Ardinger HH, Pagon RA, et al, eds.Zowonjezera. Seattle, WA: Yunivesite ya Washington. www.ncbi.nlm.nih.gov/books/NBK1266. Idasinthidwa pa Julayi 14, 2016. Idapezeka Novembala 5, 2019.

Wendel D, Murray KF. Zotupa za m'mimba. 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 372.