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. Author manuscript; available in PMC: 2010 Nov 5.
Published in final edited form as: Eur J Pediatr. 1993;152(Suppl 1):S71–S76. doi: 10.1007/bf02072093

Table 1.

Clinical data of patients with type IV glycogen storage disease undergoing liver transplantation

Patient Age (months) at transplantation Skin fibroblast branching enzyme activityc (µmoles/min/mg) Survival
1a 31 0.10 73 months
2 11 0.07 Died 7 days after LTx
3b 36 0.11 Died 36 days after LTx
4 46 0.11 58 months
5b 22 0.08 35 months
6a 20 0.06 18 months
7 34 0.09 16 months
a

Siblings

b

Siblings

c

Normal branching enzyme activity in skin fibroblasts: 1 to 2 µmoles/min/mg protein [2, 4]