Jiyya na cututtuka na Polycystic Koda

Magunguna masu zuwa da za su iya samun magani ga PKD

Kwayar cutar koda ( PKD ) wani cututtukan kwayoyin halitta ne da ke gaban ci gaba da cigaba da cigaban kyakoki a kodan. Sabanin abin da ake kira tsararru mai sauki , PKD ba cuta bane ne kuma babban ɓangaren marasa lafiya na PKD suna fuskantar hadarin koda, yana buƙatar dialysis ko kaya a cikin koda.

Lokacin da mai haƙuri ya koyi game da ganewar su na PKD, tambaya ta farko da ta fito shine ko yana da kyau.

Kafin mu iya fahimtar abin da magani zai iya yi don rage jinkirin cutar, taƙaitacciyar taƙaitaccen ɓangaren aikin hormone da ake kira ADH, ko kuma hormone anti-diuretic (wanda aka sani da vasopressin) ya zama dole.

Matsayin ADH a PKD

ADH ya taimaki rayayye daga teku zuwa kasa, eons da suka wuce. Idan ba don ADH ba, yawancin halittu masu rai ba zasu iya tsayayya da mummunan tasiri na tashar ƙasa mai zafi a karkashin hasken rana!

Sanya wani ɓangare na kwakwalwa da ake kira "hypothalamus," ADH shine hormone wanda ke aiki a koda kuma ya sa ya riƙe da kiyaye ruwa. Abin da ya sa urine ta dubi duhu da kuma mayar da hankali lokacin da ba ka da isasshen ruwa don sha ko ciyar da rana a waje a rana mai zafi. Saboda haka, yana iya rinjayar yadda ruwa yake buƙatar cirewa da kuma yadda za a "sake yin amfani da shi" don saduwa da bukatunmu (dangane da wasu dalilai, ciki har da samar da ruwa da kuma yanayin zafi).

Yaya ADH ya dace cikin tattaunawa akan CKD? Nazarin ya nuna cewa ADH yana daya daga cikin manyan masu tallafawa ci gaban karkara (dalilin rashin cin nasara koda) a cikin PKD. Watau ma'ana, idan zaka iya yin adadin ADH, ko toshe aikinsa a kan cysts, zai yiwu a rage jinkirin girma da rashin ci gaban PKD.

Maganin Jiyya na yau da kullum

Ƙin fahimtar muhimmancin da ADH ke taimakawa wajen fahimtar hanyoyin da za a iya samun magani da kuma dalilin da ya sa za su iya aiki, daga karuwar yawan ruwa zuwa ga magunguna.

Magani Tsarin Zaɓuɓɓuka

Mu fahimtar matsayin da ADH ke yi na ƙaddamar da PKD ya haifar da kyakkyawan bincike wanda zai iya samar da ƙarin maganin magance matsalolin bayan ayyukan "taimakon agaji" da aka bayyana a sama.

Binciken na yau da kullum yana mayar da hankali ga gano kwayoyi wanda zai iya toshe aikin ADH don haka ya hana karfin girma daga girma (tun lokacin karuwar karuwar karuwa ya zama mummunar rashin cin nasara koda a cikin marasa lafiya PKD).

Ga wasu misalai:

  1. Tolvaptan: Wannan magani ne wanda aka yarda da shi don magance ƙananan sodium da ayyukan ta hanyar katange shafin (wanda ake kira V2 receptor) wanda ADH zai haɗawa a cikin koda (tunani na mai karɓar V2 a matsayin "keyhole" zuwa wanda ADH ya buƙaci haɗi, yayin da tolvaptan shine "maɓallin kuskure" cewa lokacin da yanzu zai hana wannan daga faruwa).

    Gwajin TEMPO da aka yi daɗaɗɗa ya nuna wani aikace-aikacen ƙwaƙwalwar ajiya ga tolvaptan a rage jinkirin ƙi aikin aikin koda a cikin PKD. Hanyar yana nuna jinkirin karuwar ƙwayar kodan, wadda take haifar da rashin aikin ƙwayar koda ya wuce shekaru uku. Duk da haka, Tolvaptan bai riga ya sami kyaututtuka na FDA a Amurka don maganin PKD ba, saboda damuwa game da illa akan hanta. An riga an yarda da shi don kula da PKD a wasu sassa na duniya).
  1. Octreotide: Wannan wani abu ne mai sassaucin tsinkaye na hormone da ake kira somatostatin. Wani gwaji a shekarar 2005 ya ruwaito cewa wata sanarwa na watanni shida tare da somatostatin na iya rage karfin girma. Kodayake mun san cewa raguwar aikin koda a cikin PKD ya bi girma, karuwar binciken ya dakatar da furtawa cewa jinkirta girma cikin karuwar, a wannan yanayin, zai fassara cikin kariya ta asibiti.

    Sa'an nan, a 2013 mun ga sakamakon binciken ALADIN da aka buga a Lancet. Wannan binciken yana da tsawon lokaci na biyo baya fiye da binciken da ya gabata kuma ya nuna ƙaramin ƙwayar koda a cikin marasa lafiya da aka bi da octreotide a shekara guda, amma ba a shekaru uku ba.

    Bada bayanin da muke da shi yanzu, yana nuna cewa octreotide zai iya samun rawar da zai taka wajen kula da PKD. Ga wasu dalili, yana nuna cewa octreotide yana jinkirin girma a cikin ƙwayar koda a shekara guda, amma sakamakon ya zama maras muhimmanci a tsawon lokaci. A bayyane yake, ana buƙatar ƙarin nazarin karatun neman bayanai mai tsanani.

Kodayake waɗannan jami'ai sun nuna alƙawari har zuwa yanzu (banda wasu masu gwagwarmaya kamar masu maganin magunguna da sauran kwayoyi a gwaji na asibiti), farashi babbar damuwa ne. Duk sauran abubuwa daidai, octreotide zai iya kasancewa mai rahusa fiye da tolvaptan don abin da zai iya zama magani na rayuwa. A shekara ta 2017, an sayar da kwayoyi masu asibiti (15 MG) na tsawon kwanaki 30 a $ 11,000 zuwa $ 12,000 a Amurka, yayin da 90 amps na octreotide (100 mcg injections) gudu $ 300 zuwa $ 400!

> Sources:

> Nagao S, Kazuhiro N, Makoto K, et al. Ƙara Ruwa Ruwa na Ruwa Rage Rage Ci gaban Polycystic koda cuta a cikin PCK Rat. J Am Soc Nephrol. 2006 Aug 17 (8): 2220-7. Afrilu 2006 Yuni 28.

> Higashihara E, Nutahara K, Tanbo M, et al. Shin karuwar yawan ruwa ya hana ci gaba da cutar a cikin autosomal rinjaye polycystic koda cuta? Nassin Nassin Na'urar Juyin Halitta. 2014 Satumba; 29 (9): 1710-9.

> Torres V, Chapman A, Devuyst O, et al. Tolvaptan a cikin marasa lafiya tare da Autosomal Dominant Polycystic koda cuta. N Engl J Med 2012; 367: 2407-2418, 2012. DOI: 10.1056 / NEJMoa1205511

> Caroli A, Perico N, Perna A, et al. Hanyoyin maganganun somatostatin masu aiki akan ƙwayar koda da kuma karuwar kwayoyin cutar a cikin autosomal mamaye cutar cututtuka na polycystic (ALADIN): wani gwagwarmaya, wuri-sarrafawa, gwaji da yawa. Lancet. 2013 Nov 2; 382 (9903): 1485-95. Doi: 10.1016 / S0140-6736 (13) 61407-5.