cystic fibrosis...in ummi view...

Assalammualaikum wbt...

hari ni hr 1st ummi as PRHO (pre reistered houseman) kt wad paed...best, excited but penat sikit sbb dh almost 4 month just bt kje2 lab...ssm...zaman 2 dh berlalu dh, so, skrg fokus...

sbb hr ni ad mcm2 perkara kene bt, dgn orientasinya, jd ummi just sempat clerk 1 case...

12 y/o, malay boy, k/c/o cystic fibrosis with bronciectasis since 9 years old...electively admitted for intensive physiotx and iv antibiotic...hx taken from his mother...

oklah, xnk cite byk psl pt ni sbb xhbs clerk lg sbb dia g bt physio...so, nk cite basic about cystic fibrosis biar sume org faham n ummi pun sng je revise melalui pembacaan blog sendiri...

cystic fibrosis is a lethal autosomic recessive ds. it is a ds of exocrine gland that involve multiple organ.

usually the main complain is chronic resp ix (90%), pancreatic enzyme insufficiency (PI), and its cx...usually the cause of death is end stage lung ds..

actually the pathophysio is like this....

there is defect in CFTR (cystic fibrosis tranmembrance conductance regulator). its encode for a protein that fx as a chloride channel n is reg by cAMP...abN of CFTR will result in reduction of cl secretion and increase reabsorption of na and water across the epi cell....will later result in decrease hydration of mucus---and stickier mucus for bacteria---result in ix n inflammation...

it can be at lung, intestine, oancreas and liver...all start from CFTR defect...so, the result come about d same...later on we discuss about d cx n mx ok....

cukup smpai sini dl utk hari ni...

salam sayang,

ummuhumaira'