Neurofibromatosis-1

Neurofibromatosis-1 (NF1) cuta ce ta gado wacce ciwukan nama na jijiyoyin jiki (neurofibromas) ke samuwa a cikin:

• Manya da ƙananan fata na fata
• Jijiyoyi daga kwakwalwa (jijiyoyin kwanya) da kashin baya (jijiyoyin jijiyoyi)

NF1 cuta ce ta gado. Idan kowane mahaifa yana da NF1, kowane ɗayansu na da damar kashi 50% na cutar.

NF1 kuma ya bayyana a cikin iyalai ba tare da tarihin yanayin ba. A waɗannan yanayin, sababin canjin halitta ne (maye gurbi) a cikin maniyyi ko ƙwai. NF1 yana haifar da matsaloli tare da kwayar halitta don furotin da ake kira neurofibromin.

NF tana haifar da nama tare da jijiyoyi don girma ba da tsari. Wannan ci gaban na iya sanya matsin lamba akan jijiyoyin da abin ya shafa.

Idan girma ya kasance a cikin fata, za'a iya samun lamuran kwalliya. Idan ci gaban yana cikin wasu jijiyoyi ko sassan jiki, zasu iya haifar da ciwo, lahani mai tsanani na jijiyoyi, da rasa aiki a yankin da jijiyar ta shafi. Matsaloli tare da ji ko motsi na iya faruwa, gwargwadon jijiyoyin da abin ya shafa.

Yanayin na iya banbanta sosai daga mutum zuwa mutum, har ma tsakanin mutane a cikin iyali ɗaya waɗanda suke da irin canjin kwayar halittar ta NF1 iri ɗaya.

"Kofi-da-madara" (cafe au lait) aibobi sune alamun bayyanar NF. Mutane da yawa da ke da lafiya suna da ƙaramar kafe ɗaya ko biyu a au lato. Koyaya, manya waɗanda ke da tabo shida ko fiye waɗanda suka fi girma fiye da 1.5 cm a diamita (0.5 cm a cikin yara) na iya samun NF. A wasu mutane da ke da yanayin, waɗannan aibobi na iya zama kawai alama ce.

Sauran cututtuka na iya haɗawa da:

• Ciwan ido na ido, kamar su glioma
• Kamawa
• Freckles a cikin underarm ko makwancin gwaiwa
• Manya, marurai masu laushi da ake kira neurofibromas plexiform, wanda ƙila yana da launi mai duhu kuma yana iya yaɗuwa ƙarƙashin fuskar fata
• Pain (daga jijiyoyin da abin ya shafa)
• Smallananan, ciwace-ciwace na fata da ake kira nodfibromas

Mai ba da sabis na kiwon lafiya wanda ke kula da NF1 zai gano wannan yanayin. Mai ba da sabis na iya zama:

• Likitan fata
• Ci gaban likitan yara
• Kwayar halittar jini
• Neurologist

Zai yiwu a gano asalin cutar bisa ga alamomi na musamman da alamun NF.

Alamomin sun hada da:

• Masu launi, wuraren da aka ɗaga (Lisch nodules) akan ɓangaren launuka (iris) na ido
• Yin durƙusar da ƙafa a ƙuruciya wanda hakan na iya haifar da ɓarkewa
• Murdawa a cikin ɗakunan ciki, makwancin gwaiwa, ko ƙasan mama a cikin mata
• Manyan ciwace-ciwace a ƙarƙashin fata (plexiform neurofibromas), wanda zai iya shafar bayyanar kuma sanya matsi akan jijiyoyi ko gabobin da ke kusa
• Yawancin kumburi masu laushi akan fata ko zurfin jiki
• Arancin fahimta, raunin rashin kulawa da hankali (ADHD), rikicewar koyo

Gwaje-gwaje na iya haɗawa da:

• Binciken ido ta likitan ido wanda ya saba da NF1
• Gwajin kwayoyin halitta don neman canji (maye gurbi) a cikin kwayar halittar neurofibromin
• MRI na kwakwalwa ko wasu shafuka da abin ya shafa
• Sauran gwaje-gwaje don rikitarwa

Babu takamaiman magani don NF. Za a iya cire kumburin da ke haifar da ciwo ko asarar aiki. Tumoshin da suka girma da sauri ya kamata a cire su da sauri domin suna iya zama masu cutar kansa (mugu). An ba da izinin selumetinib (Koselugo) a kwanan nan don amfani da shi a cikin yara da keɓaɓɓen ciwayi.

Wasu yara da ke fama da matsalar karatu na iya buƙatar makarantar musamman.

Don ƙarin bayani da albarkatu, tuntuɓi Gidauniyar Tumor ta Yara a www.ctf.org.

Idan babu rikitarwa, tsawon rayuwar mutanen da ke da cutar NF kusan kusan al'ada ce. Tare da ingantaccen ilimi, mutanen da ke da NF na iya rayuwa ta yau da kullun.

Kodayake raunin hankali ya kasance mai sauƙin yanayi, NF1 sanannen sanadi ne game da raunin ƙarancin kulawa. Rashin nakasa ilimi matsala ce da ta zama ruwan dare.

Wasu mutane ana basu kulawa daban saboda suna da ɗaruruwan ƙari akan fatarsu.

Mutanen da ke da NF suna da damar haɓaka ƙananan ƙwayoyin cuta. A wasu lokuta ba safai ba, waɗannan na iya gajarta tsawon rayuwar mutum.

Matsaloli na iya haɗawa da:

• Rashin hankali na rashin kulawa da hankali (ADHD)
• Makafin da ƙari ya haifar a jijiyar gani (optic glioma)
• Karya cikin kashin ƙafa wanda baya warkewa da kyau
• Ciwan ƙwanji
• Rashin aiki a cikin jijiyoyin da neurofibroma ya sanya matsin lamba akan lokaci mai tsawo
• Hawan jini saboda pheochromocytoma ko kuma raunin jijiyar koda
• Cigaba da ciwan NF
• Scoliosis, ko karkatar da kashin baya
• Tumosu na fuska, fata, da sauran wuraren da aka fallasa

Kira mai ba da sabis idan:

• Kuna lura da launuka masu launin-kofi-tare da madara akan fatar ɗanku ko wasu alamomin wannan yanayin.
• Kuna da tarihin iyali na NF kuma kuna shirin haihuwar yara, ko kuna son a bincika ɗiyanku.

Ana ba da shawara kan kwayar halitta don duk wanda ke da tarihin iyali na NF.

Ya kamata a gudanar da duba shekara-shekara don:

• Idanu
• Fata
• Baya
• Jijiya
• Kula da hawan jini

NF1; Von Recklinghausen neurofibromatosis

• Neurofibroma
• Neurofibromatosis - katuwar cafe-au-lait tabo

Friedman JM. Neurofibromatosis 1. Tsinkaya ®. [Intanet]. Seattle (WA): Jami'ar Washington, Seattle; 1993-2020. 1998 Oct 2 [An sabunta 2019 Jun 6]. www.ncbi.nlm.nih.gov/books/NBK1109/.

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.

Sahin M, Ullrich N, Srivastava S, Pinto A. Ciwon ƙwayar cuta na Neurocutaneous. A cikin: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Littafin koyar da ilimin yara. 21st ed. Philadelphia, PA: Elsevier; 2020: babi na 614.

Tsao H, Luo S. Neurofibromatosis da ƙwayar tuberous sclerosis. A cikin: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018: babi na 61.