CALFAN (Low γ-Glutamyl Transpeptidase (GGT) Cholestasis, Acute Liver Failure, and Neurodegeneration) Syndrome: A Case Report with 3-Year Follow-Up after Liver Transplantation in Early Adulthood

Table 1

Summary of clinical findings from the patients of CALFAN syndrome with liver transplantation.

Patient	Our case	Lenz et al. [1]	McNiven et al. [4] patient #1	McNiven et al. [4] patient #2
Sex	Male	Female	Male	Male
Age of onset	9 months	6 months	5 months	4 months
Episode of ALF	3	4	4	1
Triggering events of ALF	Fever	Fever, diarrhea	Febrile upper respiratory infection	Lung infection
Hepatosplenomegaly	Yes	Yes	Yes	Yes
Low or normal GGT	Yes (20 U/L)	Yes (38–97 U/L)	Mildly elevated (58–83 U/L)	Yes (32 U/L)
Liver biopsy before transplantation	At age 4 years: bridging fibrosis	At age 6 months: cholestasis, hepatocyte degeneration, and giant cell transformation	At age 5 months: cholestasis, hepatocyte feathery degeneration, and bridging septa	At age 5 months: cholestasis
		At age 13 months: suggesting cirrhosis, hepatocellular injury, and nonspecific cholangitis
		At age 23 month: stage 3-4 bridging fibrosis with focal nodularity
Motor/sensory dysfunction, developmental delay, skeletal abnormality	Yes	Yes	Yes	Yes
Age at final diagnosis	14 years	Not recorded	13 years	9 years
Age at transplantation	20 years	23 months	21 months	7 months
Follow up (years) after transplantation and course	3 years; overall excellent; no episode of rejection; postop biliary stricture- recovered	8 years; satisfactory (no other descriptions)	11 years; no episode of rejection; no postop complication	9 years; no episode of rejection; postop incisional hernia and CMV colitis