ericdamiansean

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Hey, how does G6PD deficiency lead to gallstones? Esp in children?
 

automaton

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probably RBC hemolysis causing pigmented stones
 
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ericdamiansean

ericdamiansean

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Would this be:

G6PD deficiency leads to hemolysis, hem is converted into bilirubin and biliverdin, there is now hyperbilirubinemia, and since bile pigments are made up of bilirubin and biliverdin, there is increase in bile, leading to gallstones?
 

automaton

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yeah i think that's it. increased bilirubin in bile overwhelms gall bladder according to first aid. goljan and brs don't explain really. here's a link that says it's not understood very well but has to do with stasis and increased concentration...

http://www.emedmag.com/html/pre/gic/consults/101502.asp
 

razorback831

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automaton said:
yeah i think that's it. increased bilirubin in bile overwhelms gall bladder according to first aid. goljan and brs don't explain really. here's a link that says it's not understood very well but has to do with stasis and increased concentration...

http://www.emedmag.com/html/pre/gic/consults/101502.asp
In one of goljan's lectures he states that hemolytic states may lead to gall bladder disease in the form on Ca bilirubinate stones....He says it in reference to SCD though
 

automaton

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yeah if you look at his rapid review path, he mentions the ca bilirubinate (by congenital spherocytosis) and it's under the category of black pigment stones
 
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ericdamiansean

ericdamiansean

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So, it's overwhelming of the gall bladder with extra bilirubin/biliverdin from the blood
 

lattimer13

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razorback831 said:
In one of goljan's lectures he states that hemolytic states may lead to gall bladder disease in the form on Ca bilirubinate stones....He says it in reference to SCD though
won't pigment stones occur whenever any type of hemolysis is at play?
 

automaton

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yup
 

dcpark74

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:)

I just got done reviewing my path chapter on liver and gallbladder.

I am such a geek; I love Robbins :oops:

Anyway, everyone above is right about the hemolysis of red cells providing the heme and eventual excreted conjugated bilirubin via hepatocytes. the biliary tree, robbins says, can then deconjugate the conjugated bilirubin (~1%) to yield unconjugated bilirubin which is the only kind of bilirubin that forms pigment stones (calcium salts) along with inorganic calcium salts.
 
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ericdamiansean

ericdamiansean

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dcpark74 said:
don't you mean intravascular? :)
but in G6PD def, the bite cells get destroyed (hemolysed) in the spleen (extravascular) :confused:
 

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ericdamiansean said:
but in G6PD def, the bite cells get destroyed (hemolysed) in the spleen (extravascular) :confused:
With G6PD deficiency, you get both intravascular and extravascular hemolysis. The oxidatively damaged hemoglobin precipitates and forms heinz bodies, which can lead to cell death by themselves. This intravascular hemolysis dumps lots of hemoglobin into the bloodstream. But the inclusions (heinz bodies) also mess up the RBC membranes, which makes them more likely to get caught in the spleen, which leads to the extravascular hemolysis. The end product of extravascular hemolysis is bilirubin. It is this excess bilirubin (from the extravascular hemolysis) that causes the stones.
 

dcpark74

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ericdamiansean said:
but in G6PD def, the bite cells get destroyed (hemolysed) in the spleen (extravascular) :confused:

sorry, my bad...i thought we were talking about autoimmune hemolytic anemia like erythroblastosis fetalis. :)

I am going to review my RBC/WBC chapter in Robbins this week. :thumbup: (the reason i thought intravascular was because Robbins talks about it briefly in the formation of pigment stones.
 

2TIM4:7

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DHMO said:
With G6PD deficiency, you get both intravascular and extravascular hemolysis. The oxidatively damaged hemoglobin precipitates and forms heinz bodies, which can lead to cell death by themselves. This intravascular hemolysis dumps lots of hemoglobin into the bloodstream. But the inclusions (heinz bodies) also mess up the RBC membranes, which makes them more likely to get caught in the spleen, which leads to the extravascular hemolysis. The end product of extravascular hemolysis is bilirubin. It is this excess bilirubin (from the extravascular hemolysis) that causes the stones.
So, i just reviewed RBC chapter in Robbins. Sorry folks, but G6PD deficiency does not cause pigment stones!

It is not a Chronic Hemolytic Anemia. It is self limited (only affects senescent RBC) and therefore is episodic. therefore, there is no splenomegaly or cholelithiasis.