Menene Ciwon Fanconi?

Wadatacce

• Kwayar cutar Fanconi ciwo
• Dalilin cututtukan Fanconi
• Gadon FS
• An samo FS
• Ganewar asali na ciwon Fanconi
• Jarirai da yara tare da FS da suka gada
• An samo FS
• Bayanai na yau da kullun
• Jiyya na ciwon Fanconi
• Maganin Cystinosis
• An samo FS
• Outlook don cutar Fanconi

Bayani

Ciwon Fanconi (FS) cuta ce mai saurin gaske wacce ke shafar bututu mai tacewa (kusan tubules) na koda. Ara koyo game da sassa daban-daban na koda kuma duba zane anan.

A yadda aka saba, tubules na kusa suna sake dawo da ma'adanai da abubuwan gina jiki (metabolites) a cikin jini wanda ya zama dole don aiki mai kyau. A cikin FS, bututun da ke kusanci maimakon su saki adadi mai yawa na wadannan mahimmin abu a cikin fitsari. Wadannan abubuwa masu mahimmanci sun hada da:

• ruwa
• glucose
• phosphate
• masu amfani da bicarbonates
• carnitine
• potassium
• uric acid
• amino acid
• wasu sunadarai

Kodanku suna tace kusan lita 180 (kwatankwacin 190.2) a kowace rana. Fiye da kashi 98 cikin ɗari na wannan ya kamata a sake zama cikin jini. Wannan ba batun FS ba ne. Rashin ingantattun abubuwa masu narkewa na iya haifar da rashin ruwa, nakasar da ƙashi, da rashin cin nasara.

Akwai wadatar magunguna waɗanda zasu iya jinkirta ko dakatar da ci gaban FS.

FS galibi ana cin gado. Amma kuma ana iya samunta daga wasu magunguna, sunadarai, ko cututtuka.

An kira shi ne bayan likitan likitancin Switzerland Guido Fanconi, wanda ya bayyana rashin lafiyar a cikin shekarun 1930. Fanconi kuma ya fara bayanin cutar karancin jini, Fanconi anemia. Wannan kwata-kwata yanayi ne daban da ba shi da alaƙa da FS.

Kwayar cutar Fanconi ciwo

Kwayar cututtukan FS da aka gada ana iya ganin su tun suna yara. Sun hada da:

• yawan ƙishirwa
• yawan yin fitsari
• amai
• rashin cin nasara
• jinkirin girma
• rashin ƙarfi
• rickets
• ƙananan ƙwayar tsoka
• rashin lafiyar jiki
• cutar koda

Kwayar cutar FS da aka samu sun hada da:

• cutar kashi
• rauni na tsoka
• ƙananan ƙwayar phosphate (hypophosphatemia)
• ƙananan matakan potassium (hypokalemia)
• yawan amino acid a fitsari (hyperaminoaciduria)

Dalilin cututtukan Fanconi

Gadon FS

Cystinosis shine mafi yawan dalilin FS. Cuta ce mai saurin gado. A cikin cystinosis, amino acid cystine yana taruwa ko'ina cikin jiki. Wannan yana haifar da jinkirin girma da jerin rikice-rikice, kamar nakasar kashi. Mafi yawan lokuta kuma mafi tsanani (har zuwa kashi 95) na cystinosis yana faruwa a cikin jarirai kuma ya ƙunshi FS.

Binciken shekara ta 2016 ya kiyasta 1 a cikin kowane jarirai 100,000 zuwa 200,000 yana da cystinosis.

Sauran cututtukan cututtukan gado da za a iya haɗawa tare da FS sun haɗa da:

• Ciwon Lowe
• Cutar Wilson
• rashin haƙuri fructose

An samo FS

Sanadin FS da aka samu ya bambanta. Sun hada da:

• daukan hotuna zuwa wasu sankarar magani
• amfani da magungunan rage kaifin cutar
• amfani da magungunan rigakafi

Abubuwan da ke tattare da guba daga magungunan warkewa sune sanadin kowa. Yawancin lokaci ana iya bi da alamun ko juyawa.

Wasu lokuta ba a san dalilin sanadin FS da aka samu ba.

Magungunan maganin ciwon daji da ke haɗe da FS sun haɗa da:

• ifosfamide
• cisplatin da carboplatin
• azacitidine
• mercaptopurine
• suramin (kuma ana amfani dashi don magance cututtukan parasitic)

Sauran kwayoyi suna haifar da FS a cikin wasu mutane, gwargwadon sashi da sauran yanayi. Wadannan sun hada da:

• Tare tetracyclines. Rushewar kayayyakin cututtukan rigakafi sun ƙare a cikin dangin tetracycline (anhydrotetracycline da epitetracycline) na iya haifar da alamun FS cikin kwanaki.
• Aminoglycoside maganin rigakafi. Wadannan sun hada da gentamicin, tobramycin, da amikacin. Har zuwa 25 bisa dari na mutanen da aka bi da su tare da waɗannan maganin rigakafin suna haifar da alamun FS, bayanin kula na 2013.
• Anticonvulsants. Valproic acid misali daya ne.
• Kwayar cutar. Wadannan sun hada da didanosine (ddI), cidofovir, da adefovir.
• Fumaric acid. Wannan magani yana magance cutar psoriasis.
• Ranitidine. Wannan magani yana maganin ulcer.
• Boui-ougi-tou. Wannan magani ne na kasar Sin wanda ake amfani dashi don kiba.

Sauran yanayin da ke tattare da alamun FS sun haɗa da:

• na kullum, yawan amfani da giya
• manna masa hanci
• ɗaukar hotuna zuwa ƙananan ƙarfe da sinadaran aiki
• rashin bitamin D
• dashen koda
• myeloma mai yawa
• amyloidosis

Ba a bayyana ainihin hanyar da ke tattare da FS ba.

Ganewar asali na ciwon Fanconi

Jarirai da yara tare da FS da suka gada

Yawancin lokaci alamun FS suna bayyana tun suna yara da yara. Iyaye na iya lura da ƙishirwa da yawa ko kuma jinkiri fiye da yadda aka saba. Yara na iya samun larura ko matsalolin koda.

Likitan yaronku zai ba da umarnin gwajin jini da na fitsari don bincika rashin daidaito, kamar su yawan glucose, phosphates, ko amino acid, da kuma kawar da wasu hanyoyin. Hakanan zasu iya bincika cystinosis ta hanyar kallon ƙwarjin yaro tare da gwajin fitilar tsaguwa. Wannan saboda cystinosis yana shafar idanu.

An samo FS

Likitanku zai nemi tarihin likitanku ko na ɗanku, gami da duk wani ƙwayoyi da ku ko yaranku ke sha, wasu cututtukan da ke nan, ko baje kolin sana’a. Za su kuma ba da umarnin gwajin jini da fitsari.

A cikin FS da aka samo, ƙila ba ku lura da alamun cutar nan take. Kasusuwa da koda suna iya lalacewa ta lokacin da aka gano cutar.

FS da aka saya na iya shafar mutane a kowane zamani.

Bayanai na yau da kullun

Saboda FS irin wannan cuta ce mai saurin faruwa, likitoci na iya zama ba su sani ba. Hakanan FS na iya kasancewa tare da wasu cututtukan cututtukan ƙwayoyin cuta, kamar:

• cystinosis
• Cutar Wilson
• Ciwon hakori
• Ciwon Lowe

Za'a iya danganta alamun cutar ga sanannun cututtuka, gami da ciwon sukari na 1. Sauran rashin fahimtar cutar sun hada da masu zuwa:

• Za'a iya danganta ci gaban da aka samu a cikin cututtukan sikari, rashin abinci mai gina jiki na yau da kullun, ko kuma yawan guguwar thyroid.
• Rickets za'a iya danganta shi da rashi bitamin D ko nau'ikan rickets na gado.
• Ana iya danganta rashin aikin koda ga wata cuta ta mitochondrial ko wasu cututtukan da ba safai ba.

Jiyya na ciwon Fanconi

Maganin FS ya dogara da tsananin shi, dalilin sa, da kasancewar sauran cututtuka. FS ba za a iya warkewa yawanci ba har yanzu, amma ana iya sarrafa alamun. A farkon ganewar asali da magani, mafi kyawun hangen nesa.

Ga yara masu fama da larurar FS, layin farko na magani shine maye gurbin abubuwa masu mahimmanci waɗanda ƙododin da suka lalace suka kawar da su fiye da kima. Sauyawa daga waɗannan abubuwan na iya zama ta baki ko ta hanyar jiko. Wannan ya hada da maye gurbin:

• lantarki
• masu amfani da bicarbonates
• potassium
• bitamin D
• phosphates
• ruwa (lokacin da yaro ya bushe)
• sauran ma'adanai da na gina jiki

Ana ba da shawarar cin abinci mai yawan kalori don kula da ci gaban da ya dace. Idan ƙasusuwan yaron ba su da kyau, za a iya kiran masu kwantar da hankali na jiki da ƙwararrun ƙashi.

Kasancewar sauran cututtukan kwayoyin halitta na iya buƙatar ƙarin magani. Misali, ana bada shawarar rage cin abinci mai ƙananan jan ƙarfe don mutanen da ke fama da cutar Wilson.

A cikin cystinosis, an warware FS tare da nasarar dashen koda bayan rashin nasarar koda. Wannan ana ɗaukarsa magani ne ga cutar, maimakon magani ga FS.

Maganin Cystinosis

Yana da mahimmanci don fara magani da wuri-wuri don cystinosis. Idan ba a magance FS da cystinosis ba, yaron na iya samun matsalar rashin koda har zuwa shekara 10.

Hukumar Abinci da Magunguna ta Amurka ta amince da wani magani wanda ke rage yawan sinadarin cystine a cikin sel. Cysteamine (Cystagon, Procysbi) za'a iya amfani dashi tare da yara, farawa tare da ƙananan kashi kuma aiki har zuwa kashi na kulawa. Amfani da shi na iya jinkirta buƙatar dashen koda na shekaru 6 zuwa 10. Koyaya, cystinosis cuta ce ta tsari. Yana iya haifar da matsala tare da sauran gabobin.

Sauran jiyya don cystinosis sun hada da:

• ciwon ido na cysteamine don rage yawan cystine a cikin cornea
• maye gurbin girma na hormone
• dashen koda

Don yara da wasu tare da FS, sa ido mai gudana ya zama dole. Hakanan yana da mahimmanci ga mutanen da ke tare da FS su kasance masu daidaito wajen bin tsarin maganin su.

An samo FS

Lokacin da abin da ke haifar da FS ya daina aiki ko kuma aka rage adadin, kodan za su murmure tsawon lokaci. A wasu lokuta, lalacewar koda na iya ci gaba.

Outlook don cutar Fanconi

Hangen nesa ga FS ya fi kyau sosai a yau fiye da shekarun da suka gabata, lokacin da rayuwar mutanen da ke da cutar cystinosis da FS ta fi guntu sosai. Samuwar maganin cysteamine da dashen koda na baiwa mutane da yawa tare da FS da cystinosis damar yin rayuwa daidai da ta tsawan rai.

Ana haɓaka sabuwar fasaha don bincika jarirai da jarirai don cystinosis da FS. Wannan ya sa ya yiwu magani ya fara da wuri. Har ila yau, ana ci gaba da bincike don gano sababbin kuma ingantattun hanyoyin kwantar da hankali, kamar su dashen kwayar halitta.