Conunshin ingin

Incontinentia pigmenti (IP) yanayi ne mai saurin gaske wanda ya shafi iyalai. Yana shafar fata, gashi, idanu, hakora, da tsarin juyayi.

IP yana faruwa ne ta hanyar babban lahani mai nasaba da X wanda ke faruwa akan kwayar halittar da aka sani da IKBKG.

Saboda lalacewar kwayar halitta yana faruwa akan X chromosome, yawancin lokuta ana ganin yanayin a cikin mata. Lokacin da yake faruwa a cikin maza, yawanci yakan mutu ne a cikin tayi kuma yana haifar da zubar da ciki.

Tare da alamun fata, akwai matakai 4. Jarirai masu dauke da IP ana haihuwarsu tare da kwararar wurare, wurare masu lahani. A mataki na 2, lokacin da wuraren suka warke, sai su rikide su zama tsautsayi. A mataki na 3, kumburin ya tafi, amma ya bar fata mai duhu, wanda ake kira hyperpigmentation. Bayan shekaru da yawa, fatar ta koma yadda take. A mataki na 4, za'a iya samun yankunan fata mai launi (hypopigmentation) wanda ya fi siriri.

IP yana haɗuwa da matsalolin tsarin kulawa na tsakiya, gami da:

• Ci gaban da aka jinkirta
• Rashin motsi (inna)
• Rashin hankali
• Magungunan tsoka
• Kamawa

Mutanen da ke da IP na iya samun hakora marasa kyau, asarar gashi, da matsalolin gani.

Mai ba da sabis na kiwon lafiya zai yi gwajin jiki, ya kalli idanu, ya gwada motsi na tsoka.

Zai yiwu a sami wasu alamomi da busauran da ba na yau da kullun ba a kan fata, da kuma rashin daidaituwar kashi. Gwajin ido na iya bayyana ciwon ido, strabismus (ƙetaren idanu), ko wasu matsaloli.

Don tabbatar da ganewar asali, ana iya yin waɗannan gwaje-gwajen:

• Gwajin jini
• Gwajin fata
• CT ko MRI na kwakwalwa

Babu takamaiman magani don IP. Ana amfani da magani akan alamun mutum. Misali, ana iya bukatar tabarau don inganta gani. Za'a iya ba da magani don taimakawa wajen shawo kan kamuwa da cututtukan tsoka.

Waɗannan albarkatun na iya samar da ƙarin bayani game da IP:

• Incontinentia Pigmenti International Foundation - www.ipif.org
• Nationalungiyar forasa don Rare Rashin Lafiya - rarediseases.org/rare-diseases/incontinentia-pigmenti

Ta yaya mutum yake yi ya dogara da tsananin tasirin tsarin kulawa na tsakiya da matsalolin ido.

Kira mai ba da sabis idan:

• Kuna da tarihin iyali na IP kuma kuna la'akari da samun yara
• Childanka yana da alamun wannan cuta

Ba da shawara kan kwayar halitta na iya zama taimako ga waɗanda ke da tarihin iyali na IP waɗanda ke tunanin samun yara.

Bloch-Sulzberger ciwo; Bloch-Siemens ciwo

• Incontinentia pigmenti a kafa
• Incontinentia pigmenti a kafa

Dan Majalisar Islama, Roach ES. Ciwon ƙwayar cuta na Neurocutaneous. A cikin: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology a cikin Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016: babi na 100.

James WD, Elston DM, Kula da JR, Rosenbach MA, Neuhaus IM. Genodermatoses da cututtukan haihuwa. James WD, Elston DM, Kula da JR, Rosenbach MA, Neuhaus IM, eds. Cututtukan Andrews na Fata: Clinical Dermatology. 13th ed. Philadelphia, PA: Elsevier; 2020: babi na 27.

Thiele EA, Korf BR. Phakomatoses da yanayin haɗin gwiwa. A cikin: Swaiman KF, Ashwal S, Ferriero DM, et al, eds. Swaiman's Neurology na Yara: Ka'idoji da Ayyuka. Na 6 ed. Philadelphia, PA: Elsevier; 2017: babi na 45.