
barter syndrome

barter syndrome (bii. ezz) ennathu henleyude looppinte kattiyulla aarohana avayavathile oru vaikalyathaal savisheshathayulla oru apoorva paarambarya rogamaanu, ithu pottasyam alavu (hypocalemia), rakthathile piechu (alcalosis) vardhippikkuka, rakthasammardham saadhaarana nilayilekku kuraykkuka ennivaykku kaaranamaakunnu. barter syndrome rund tharamunda neonattal, classic. adutha bandhamullah oru rogamaaya gitalmaan syndrome, barter syndrome enna rund upavibhaagangalekkaalum soumyamaanu.
adayaalangalum lakshanangalum.
90 sathamaanam kesukalilum, garbhaavasthayude 24 muthal 30 aazhchakalkkidayil adikamaaya amneotic draavakam (polyhydramnios) ulla neonattal barter syndrome kaanapedunnu. jananashesham, sisuvinu amithamaayi moothramozhikkunnathum kudikkunnathum kaanapedunnu (polyoria, polydipsia enniva yathaakramam). kunjinu mathiyaya draavakam labhichillengil jeevan apakadappeduthunna nirjjaleekaranam undakam. ekadesham 85 sathamaanam shishukkalum moothrathilum (hypercalsuria) vrukkakalilum (nephrocalcinosis) adhika alavil kaalsyam samskarikkunnu, ithu vrukkayile kallilekku nayichekkaam. apoorva sandarbhangalil, kunju vrukka thakaraarilekku neengiyekkaam.
classic barter syndrome ulla rogikalkku jeevithathinte aadya rund varshangalil lakshanagal undakamenkilum avaa saadhaaranayaayi school praayatthilo athinusheshamo roganirnayam nadathappedunnu. navjata shishukkaleppole, classic barter syndrome ulla rogikalkkum polyuriya, polydipsia, nirjjaleekarana pravanatha ennivayundu, ennaal vrukkayile kallukal undakanulla pravanatha illaathe moothrathile kaalsyam visarjanam saadhaaranamo cheruthaayi vardhikkukayo cheiyunnu. eee rogikalkku chharddiyum valarcha mandagathiyumundu. rogam chikilsichaal vrukkayude pravartthanavum saadhaaranamaanu, pakshe idaykkide rogikal vrukkayude avasana ghattathilekku neengunnu.
rakthathile kuranja alavil pottasyam, alcalosis, saadhaarana muthal kuranja rakthasammardham, uyarnna plasma renin, aaldostiron enniva barter syndromil ulppedunnu. eee syndrominte niravadhi kaaranagal undaayekkaam. serem moolyangal kuravaanengilum uyarnna moothra pottasyam, chloride enniva diagnostic pooyintarukalil ulppedunnu, vardhicha plasma renin, vrukka biopsy jextaglomerular upakaranathinte hyperplasia, moothravisarjjana durupayogam shradhaapoorvvam ozhivakkal enniva ulppedunnu. vrukkakal prostaglandinukalu amita uthppaadanam palappozhum kaanapedunnu. magneeshyam paazhakkunnathum sambhavikkam. homocygus rogikalkku kadinamaaya hypercalsury nephrocalcinosis anubhavappedunnu.
pathophysiology.
henleyude aarohana lupe ennum vilikkappedunna nephroninte kattiyulla aarohana avayavathil vrukka koshangaliludaneelam ionukal kondupookunna jeenukalude encoding proteinukalude mutationukal moolamaanu barter syndrome undaakunnath. prathyekichum, naa-keey-2cl cottrensporter neritto allaatheyo ulppedunna mutationukal pradhaanamaan. tubulinte appikkal membraniludaneelam oru soodiyam, oru pottasyam, rund chloride ionukal ennivayude electronutral gathaagathathil naa-keey-2cl cottrensporter ulppedunnu. sajeevamaakumbol eee transporterinte pravartthanam kuraykkunnathinulla kazhivu basolaattaral kaalsyam sensing resepterinu undu. tuble cellukalilekku athichukazhinjaal, soodiyam ionukal sajeevamaayi baasolaattaral membraniloode Na +/k +-attfesukaliloode kondupookunnu, chloride ionukal basolaattaral chloride chaanalukaliloode sugamamaya vyaapanathiloode kadannupokunnu. ennirunnaalum, pottasyam appikkal pottasyam chaanalukaliloode tuble lumenilekk veendum vyaapikkukayum lumenilekk oru nettu positteev charge thirike nalkukayum lumen intersteeshyal spays thammil oru positteev volteju sthaapikkukayum cheiyunnu. kaalsyam, magneeshyam ayonukalude paracellular reabsorption eee charge gradient nirbandhamaanu.
kattiyulla aarohana avayavathil saadhaarana ayoon reabsorption cheiyunnathinu eee traansporttarukalude sariyaaya pravartthanam aavasyamaanu, koodaathe eathenkilum ghadakam nashtappedunnathu sisttathinte mothatthilulla pravarthanaparamaaya nishkriyathvathinu kaaranamaakukayum barter syndrome avatharippikkunnathilekku nayikkukayum cheyyum. eee reabsorption sisttathinte pravartthanam nashtappedunnathu kattiyulla aarohana avayavathile soodiyam, pottasyam, chloride ennivayude reabsorption kurayunnathinum lumen-positteev volteju nirthalaakkunnathinum kaaranamaakunnu, ithu kaalsyam, magneeshyam ennivayude reabsorption kuraykkunnathinu kaaranamaakunnu. ivide soodiyam veendum agiranam cheyyappedunnathu vrukkasambandhamaaya medulayude hypertonity nirthalaakkunnathinte abhikaamyamallaatha phalavum nalkunnu, ithu pinneedu disttal nephronilum shekharana daktt systethilum vellam veendum agiranam cheyyaanulla kazhivine saaramaayi thadassappeduthunnu, ithu ganyamaaya diurisilekkum vooliyam kurayaanulla saadhyathayilekkum nayikkunnu. avasaanamaayi, distal nephronilekku vardhicha soodiyam looad kompansettari reabsorption samvidaanangal purappeduvikkunnu, ennirunnaalum pradhana koshangal visarjjikkunnathiloodey hypocalemiayude falamaayum pottasyam nashtappedunnu. eee vardhicha pottasyam visarjanam hydrajan ayonukalude chelavil α-intercolated koshangal bhaagikamaayi nikathunnu, ithu metaboolik alcalosisilekku nayikkunnu.
barter, gitalmaan syndrome ennivaye ulppedunna jeenukale adisthaanamaakki vyathyastha upavibhagangalaayi tirikkaam
roganirnayam.
barter syndrome ulla aalukal furosemide polulla lupe diuretixilulla rogikalude lakshanangalkk samaanamaan, kaaranam lupe diuretics syndromil thakaraarulla krithyamaaya transport proteine lakshyamidunnu (kuranjathu type 1 barter syndrome). syndrominte mattu upavibhagangalil mattu transporterukalil mutationukal ulppedunnu, ithu taargett transporterinte pravarthanaparamaaya nashtatthinu kaaranamaakunnu. uppuvellamulla bhakshanangalkk vyakthiparamaaya munganana nalkunnuvennu rogikal palappozhum sammathikkunnu.
hypocalemia, metaboolik alcalosis, saadhaarana rakthasammardham muthal thaazhnna rakthasammardham ennivayaanu barter syndrominte klinikkal kandethalukal. eee kandethalukal aashayakkuzhappamundaakkunna mattu avasthakal moolavum undakam. barter syndrome nirnnayikkumbol, rogalakshanathinte saadhyamaaya kaaranangalayi inipparayunna avasthakal thallikkalayanam
barter syndrome ulla rogikalkku renin, aaldostiron ennivayude alavu uyarnnekkaam.
prasavapoorva barter syndrome polyhydramniosumaayi bandhappettirikkam.
bandhapetta vyavasthakal.
ennirunnaalum, barter syndrominte savisheshathakal uyarnna renin, uyarnna aaldostiron, hypercalciuria, henleyude looppinte kattiyulla aarohana avayavathile asaadhaaranamaaya naa +-keey +-2cl-transporter ennivayum aanu, athesamayam gitalmaan syndrome hypocalciuriaykku kaaranamaakunnu, ithu vidoora vibhaagathile asaadhaaranamaaya tayaside sensitiivu transporter moolamaanu.
"barter syndrome poleyulla samanamaaya oru syndrome aanu "suedo-barter syndrome", ennaal athinte savisheshamaaya janithaka vaikalyangalonnumilla. sistik fibrosisilum athupole thanne poshakangalude amithamaya upayogathilum suedo-barter syndrome kaanappettittund.
chikitsa.
vaidyashaasthraparamaayi melnottam vahikkunna soodiyam, chloride, pottasyam supplementation aavasyamaanu, koodaathe pottasyam nashtam kuraykkunnathinu spironolactone upayogikkaam. rogikalkku uchithamaaya daaham prathikaranamullathinal nirjjaleekaranam thadayaan soujanyavum yogyathayillathathumaaya vellam aavasyamaanu. supplementation maathram bio kemikkal homeostasis nilanirthaan kazhiyaatha gurutharamaaya kesukalil, glomerular filtration kuraykkaan nonstiroyidal aanti-inflammatory marunnukal (enn. ezz. eiii. aii. dii. ezz) upayogikkaam, ithu valare upayogapradamaanu, ennirunnaalum ithu gastric prakopippikkunnathinum vayarile assid adichamarthal chikilsakalkkoppam nalkunnathinum kaaranamakum. glomerular filtration nirakk kuraykkunnathinu angiotensin-converting ensime (easu) inhibittarukal upayogikkaam. cheria kunjungalilum kuttikalilum, rogaavasthayude kaalaghattangalil draavaka upabhogathil vittuveezhcha cheiyunna serem electrolyteukal parishodhikkunnathin kuranja paridhi aavasyamaanu.
moothrathinte eakaagrathayude budhimuttu kooduthal vardhippikkunna oru saadhaarana sankeernathayaaya nephrocalcinosis vikasikkunnath nireekshikkaan nireekshana vrukka ultraasoundu upayogikkanam.
pravachanam.
classic barter syndrome ulla sisukkaludeyum kochukuttikaludeyum neratheyulla roganirnayavum uchithamaaya chikitsa valarchayum orupakshe boudhika vikasanavum mechappeduthumennu labhyamaya parimithamaaya roganirnaya vivarangal soochippikkunnu. maruvashathu, susthiramaaya hypocalemia hyperreninemy purogamana tubulointistil nephraittisin kaaranamakum, ithu avasana ghatta vrukkarogathinu (vrukka takaraar) kaaranamaakunnu. electrolyte asanthulithaavasthayude aadyakaala chikilsayiloode, classic barter syndrome ulla rogikalkku roganirnayam nallathaanu.
charithram.
doow. frederic barter, addehathodoppam doow. 1960-ll aadyamaayi vivaricha pasitta pronov, 1962-ll kooduthal rogikalil ithu vivarichu.