Huntseatchick
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Maybe I'm tired; maybe this is a dumb question -- what produces this substance? I know PMNs produce elastase, but I'm stuck. Thanks!!!
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| asmi
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its antiprotease..may be from alveloar macrophages
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| asmi
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its a protein present in blood.
The serine proteases are a group of closely related proteolytic enzymes, with serine in their active site, which play a key role in coagulation and fibrinolysis and in kinin and complement activation. The activities of these enzymes are controlled at least in part by specific inhibitors known collectively as serine protease inhibitors, or serpins. The serine protease inhibitor found in highest concentration in plasma is alpha 1-antitrypsin, a 52-kDa glycoprotein, which accounts for 90% of the total alpha-1-globulin in plasma. Despite its name, the major function of alpha-1-antitrypsin is to inhibit the activity of elastase generated by neutrophils in the lung. The major phenotype of alpha-1-antitrypsin deficiency is destruction of pulmonary alveoli resulting in ehronie obstructive pulmonary disease or emphysema.
http://www.copd-international.com/Library/Alpha1....
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| peekay
Forum Guru
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there is nice diagrame in lippin bichem.it shows elastase and alph 1 AT interaction
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| mjl1717
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So the answer is mostly liver, monocytes and as asmi said alveolar macrophages
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| asmi
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mjl...iam not sure about alveolar macrophages...if you can find it out,i would be greatful .
I am also a big dumb huntseatchick 
I was just able to find out that it is a protein present in blood .
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| usmle2004
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Alpha-1-antitrypsin is a liver protein that blocks the destructive action of neutrophil elastase on lung elastin fibres. Imbalance of lung proteases and antiproteases probably plays a role in the development of emphysema.
Individuals with a homozygous deficiency who also smoke have a greatly increased risk of developing severe panlobular emphysema before the age of 35. Such a homozygous deficiency accounts for less than 1% of COPD.
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| asmi
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name the disease in which there is excess production of alpha 1 antitrypsin ?
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| maxxicum
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name the disease in which there is excess production of alpha 1 antitrypsin?
Seems there is no such disease described. Tried to search medline for excess of alpha 1 antitrypsine and there is no significant output for word "excess" combined with alpha-1-antitrypsin search.
I suppose that even if excess of a-1-a is possible, it wasn't yet noted that it will be associated with any kind of disorder.
Probably your question is not significant.
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| asmi
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kids with excess alpha1 antitrypsin----> deveolp HCC .
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| maxxicum
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Did I get your idea right?
If a kid has increased a1at level, he has greater risk developing HCC?
I would think HCC induces increased production of a1at (mean it is a consequence)
Haven't found any info about it, so it's just my guess...
And... sorry for gettin into too much details.
Regards.
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| asmi
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Iam so sorry i made a big mistake..in HCC pts there will be alpha-1 trypsin deficiency .I apologise :oops: ..."this is what is known as we learn my our mistakes"
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| asmi
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Yes i got the answer.....now 101% confident about it.
Alpha -1 antrypsin accumulates in liver ( in kids with this autosomal recessive condition) and it cannot be released into blood .Therefore i was correct that in cases of kids with HCC alpha-1 levels are increased in liver .
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| Malaysian
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I would assume that A1AT deficiency leads to cirrhosis and from there the development of HCC.I've never come across any material stating excess HCC causing A1AT.A1AT is reperesented by the S and Z gene.
Normal people have the PiZZ gene whereas those with A1AT have PiSS(if its the hereditary A1AT deficiency).There is heterogenicity as PiSZ which is sub-clinical
Asmi could you kindly quote your source??Thanx.
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| mdwannabe
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Malaysian, you probably made an atypo.
PiZZ phenotype is actually the one with deficiency. ABNORMAL PEOPLE that is.
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| Malaysian
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I just double checked.....apparently both S and Z are abnormal.S is slow variant and Z is very slow variant.In normal people its M variant which is moderate.PiMM is normal.
Thanks for pointing it out MDwannabe
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| mdwannabe
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:-)
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| asmi
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I read in goljan's patho review
:idea: and iam sure about my answer .Its (inc levels of AAT)only seen in kids with HCC
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| Idiopathic
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Heres the deal: 2 types of alpha-1 AT deficiency exist.
In one type, the liver doesnt make enough AAT, and the adult individuals develop severe panacinar emphysema due to the neutrophil elastase degradation of the lung parynchema,
In the other type, the liver makes a defective form of AAT, that cannot get out of the liver. It causes liver failure in children. In my opinion, for the boards, ANY child in liver failure should be assumed to have: a) AAT deficiency, b) Reye's syndrome, or c) Acetaminophen overdose
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| Gul
Forum Elite
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i remember reading in Kaplan , alpha 1 AT deficiency as one of most common causes requiring liver transplantation.
In hepatocellular carcinoma, 2 tumor markers: AAT N AFP
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| sujju
Forum Elite
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a a t deficiency leads to micro nodular cirrosis &panacinarempysema. normal variant is pim .pizz is severe reduced variant.
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| sujju
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a a t deficiency leads to micro nodular cirrosis &panacinarempysema. normal variant is pim .pizz is severe reduced variant.
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