Incontinentia pigmenti

Incontinentia pigmenti (IP) ndichikhalidwe chosowa khungu chomwe chimadutsa m'mabanja. Zimakhudza khungu, tsitsi, maso, mano, ndi dongosolo lamanjenje.

IP imayambitsidwa ndi vuto lomwe limalumikizidwa ndi X lomwe limapezeka pa jini lotchedwa IKBKG.

Chifukwa chakuti vuto la jini limapezeka pa X chromosome, vutoli limawoneka mwa akazi. Ikapezeka mwa amuna, nthawi zambiri imakhala yoopsa mwa mwana wosabadwayo ndipo imabweretsa padera.

Ndi zizindikiro za khungu, pali magawo anayi. Makanda omwe ali ndi IP amabadwa ndi malo owopsa, ophulika. Gawo lachiwiri, maderawo akamachira, amasanduka mabampu. Gawo lachitatu, ziphuphu zimatha, koma zimasiya khungu lakuda, lotchedwa hyperpigmentation. Pambuyo pa zaka zingapo, khungu limabwerera mwakale. Pa gawo 4, pakhoza kukhala madera a khungu lowala kwambiri (hypopigmentation) lomwe ndi locheperako.

IP imagwirizanitsidwa ndi mavuto apakati amanjenje, kuphatikiza:

• Kukula kwakuchedwa
• Kutaya kuyenda (ziwalo)
• Kulemala kwamaluso
• Kupweteka kwa minofu
• Kugwidwa

Anthu omwe ali ndi IP amathanso kukhala ndi mano osazolowereka, tsitsi, komanso mavuto amaso.

Wothandizira zaumoyo adzayesa thupi, kuyang'ana maso, ndikuyesa kuyenda kwa minofu.

Pakhoza kukhala njira zosazolowereka ndi zotupa pakhungu, komanso zovuta zamfupa. Kuyezetsa maso kumatha kuwonetsa khungu, strabismus (maso owoloka), kapena mavuto ena.

Kuti mutsimikizire matendawa, mayesowa atha kuchitika:

• Kuyesa magazi
• Khungu lakhungu
• Kujambula kwa CT kapena MRI kwa ubongo

Palibe mankhwala enieni a IP. Chithandizo chake ndichachidziwikire. Mwachitsanzo, pamafunika magalasi kuti muwone bwino. Mankhwala atha kulembedwa kuti athandizire kuthana ndi kukomoka kapena kupweteka kwa minofu.

Izi zitha kupereka zambiri za IP:

• Incontinentia Pigmenti International Foundation - www.ipif.org
• National Organisation for Rare Disways - rarediseases.org/rare-diseases/incontinentia-pigmenti

Momwe munthu amachitila bwino zimadalira kuuma kwa magwiridwe antchito amkati amanjenje komanso mavuto amaso.

Itanani omwe akukuthandizani ngati:

• Muli ndi mbiri ya banja la IP ndipo mukuganiza zokhala ndi ana
• Mwana wanu ali ndi zizindikiro za matendawa

Upangiri wamtundu ungakhale wothandiza kwa iwo omwe ali ndi mbiri yabanja ya IP omwe akuganiza zokhala ndi ana.

Matenda a Bloch-Sulzberger; Matenda a Bloch-Siemens

• Incontinentia pigmenti pamiyendo
• Incontinentia pigmenti pamiyendo

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James WD, Elston DM, Chitani JR, Rosenbach MA, Neuhaus IM. Genodermatoses ndi zovuta zobadwa nazo. James WD, Elston DM, Chitani JR, Rosenbach MA, Neuhaus IM, eds. Matenda a Andrews a Khungu: Clinical Dermatology. Wolemba 13. Philadelphia, PA: Elsevier; 2020: chap 27.

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