Table 1 Indication and follow-up of GSDIa and GSDIb liver transplantation

Indications

Hepatic adenomas or liver abnormalities (mostly focal nodular hyperplasia)

29 [6–22]

Poor metabolic control

27 [6, 8–10, 14–16, 19, 21, 23–27]

21 [13, 28–34]

Growth retardation

13 [10, 14–16, 23, 24, 27]

3 [32, 33, 35]

Recurrent infections

10 [28, 33, 34]

Renal failure

5 [36]

-Of which also kidney transplant

3 [17, 26, 37]

Bleeding complications (anemia)

1 [27]

Pancreatitis

1 [24]

Anal and oral ulcera

1 [30]

Immunosuppressive regime

Cyclosporine

12 [6, 7, 9, 10, 12, 14, 15, 17],[21, 26]

1 [28]

Cyclosporine + azathioprine

17 [16, 23, 25, 38, 39]

-

Tacrolimus

9 [16, 18, 21, 36]

16 [30, 31, 33–35]

Not reported

22

6

Outcome and complications GSDIa

Short-term (≤1 year)

Long-term (>1 year)

Normalization liver function

58 (all)

-

Catch-up growth

-

13 [10, 13, 14, 23, 25, 39]

Sexual maturation

-

2 [25]

Re-OLT

2 [16, 23]

1 [20]

Death

1 (pancreatitis and sepsis) [25]

3 (rejection related liver failure, suicide, metastatic hepatocellular carcinoma) [19], ^PC

Renal failure

6 [21]

4 [20]

-Of which already pre-transplant

1 [39]

-Requiring dialysis

1 [12] (temporary)

2 [27], ^PC (permanent)

-Gouty arthritis (due to renal failure?)

-

1 [14]

Metastatic hepatocellular carcinoma

1^PC

Transplantation associated

-Hepatic artery thrombosis

2 [16, 23]

-

-Portal vein thrombosis

-

2 [6, 16]

-Hepatic vein obstruction

2 [25]

-

-Prolonged drainage

2 [25, 39]

-

-Rejection

5 [14, 20, 21, 38, 39]

3 [6, 9]

-Never functioning liver after transplantation

2 [23, 24]

Therapy (immune suppression) associated

-Hypertension

4 [25, 39]

-

-Insulin-dependent diabetes (reversible)

3 [8, 9, 21]

-

-Infections (CMV, hepatitis)

7 [21, 25, 39]

1 [14]

Outcome and complications GSDIb

Short-term (≤1 year)

Long-term (>1 year)

Normalization liver function

23 (all)

Catch-up growth

2 [32]

Death

1 (systemic candidiasis) [40]

Neutropenia

-

-Improved

-

14 [31, 34, 35]

-Persisted

7 [13, 29, 30, 32, 33]

Thrombocytopenia

1 [28]

Transplantation associated

-Infection

6 [34]

-

-Anemia

1 [33]

-

-Rejection

-

1 [13]

Therapy (immune suppression) associated

-Infection

1 [30]

1 [26]

-Tacrolimus encephalopathy

1 [35]