monet
Forum Newbie
Topics: 14
Posts: 17
|
A pt who has mild fasing hypoglycemia and enlarged heart. What's the defect?
|
| namf
Forum Elite
Topics: 80
Posts: 312
|
Could it be steroid use (diabetogenic and stimulate hypertrophy of heart muscle)????
Thanks!
|
| dariush
Forum Elite
Topics: 20
Posts: 356
|
but steroids cause Hyperglycemia not Hypo!!! that`s why body in the long starvation releases Cortisol.
how about "Chronic Alchoholism" with Chron.Panc.+Fatty heart ??
___________________
Allah (God) is watching over us...
|
| mash
Forum Fanatic
Topics: 147
Posts: 1,326
|
pompes ds(def of 1,4 glucosidase)
___________________
I hear and I forget. I see and I remember. I do and I understand.
--Confucius
|
| tonguo
Forum Senior
Topics: 29
Posts: 125
|
pompe's dis. due to lysosoal enzyme alpha 1,4-glucosidase deficiency. glycogen stored in liver and striated muscles, cardiomegaly is significant and leads to death.
|
| BumbleB
Forum Junior
Topics: 2
Posts: 83
|
Another clue that'll you'll always see with Pompe's is that the pt. is an infant b/c kids with Pompe's usually don't survive pat their 2nd year... 
|
| Malaysian
Forum Guru
Topics: 28
Posts: 778
|
I agree with Pompe's disease
|
| vrach
Forum Junior
Topics: 3
Posts: 79
|
I don't think there is any hypoglycemia involved in Pompe's disease. Why would a lysosomal alpha glucosidase cause hypoglycemia. The only three glycogen storage diseases in which there is hypoglycemia involved are Von Gierke, Hers, and Cori. If there is hypoglycemia in a glycogen storage disease, shouldn't there be deficency of an enzyme involved in the glycogenolysis or gluconeogenesis in the Liver?
|
| dxtxpx
Forum Guru
Topics: 259
Posts: 1,233
|
maybe hyperosmolar nonketotic coma can present with hypoglycemia and cardiomegaly in severely dehydrated pt.
For sure there is no hypoglycemia in Pompe's dis. the alpha-1,3-glucosidase is a lalysosomal enz and is not part of normal glycogen synthase and phosphorylase cycle. Glycogen accumulates in lysosomal vacuoles, potentially causing problems in many tissues. Cardiomegaly is most life-threatening.
AR dis that requires inheritance of 2 defective copies of gene encoding acid alpha glucosidase (GAA) which is on band 17q23.
http://www.emedicine.com/PED/topic1866.htm
|
|
| |
| | | | | | | | | |
