Case 1: 

Patient A:

Patient A: 15-year-old girl who experienced uncharacteristic muscle pain, soreness, and fatigue during exercise. AlkPhos 114 (40-150 U/L), AST 385 (0-35 U/L), ALT 460 (0-50 U/L), IgG 7340 (695-1620 mg/dL), F-Actin IgG 95 (>31 units = positive), Smith SSA (Ro) >8.0 (0-0.9 AI). Negative for Scleroderma Scl-70 ENA, SSB (La), Smooth Muscle Antibody (SMA), RNP Antibody IgG, Jo 1 Antibody IgG, LKM Antibody, Hepatitis B and C.

 

• Case 01 - A01-H&E

• Case 01 - A02-PAS

• Case 01 - A03-Mum1

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Patient B: same process as patient "A" :

13 year old girl with a painless left sided abdominal mass for 3 months prior to presentation with left sided pain and thrombocytopenia. Imaging showed splenomegaly and a diagnosis of immune thrombocytopenia was made. Subsequent lab studies showed: Abnormal liver function tests: AlkPhos 232, AST 617, ALT 712, IgG 3502, SMA 93 (0-19), and negative ANA.

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• Case 01 - B01-H&E

• Case 01 - B02-PAS

• Case 01 - B03-Mum1

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Case 2: 

16 year old girl without prior medical problems presents in renal failure requiring hemodialysis. She was found to have hemolytic anemia, AlkPhos 19 (40-150 U\L), ALT =33 (0-50 U\L), Total Bilirubin 17.3 (0.2-1.3 mg/dL). Within 3 days of admission, ammonia was 97 (10-50 umol/L) and an emergent liver transplant was done.

 

• Case 02 - H&E

• Case 02 - Trichrome

 

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Case 3: 

6 month old boy born at 23 weeks gestation with a history of necrotizing enterocolitis and a bowel perforation in the first 2 weeks of life; reanastomosed at 4 months of life. He has generalized bowel dysmotility and a rectal biopsy for Hirschsprung's disease at 5 months of age was normal. A liver biopsy was done during a laparotomy for lysis of adhesions (causing bowel obstruction) because the surgeon thought the liver looked abnormal. Albumin 2.2 (2.6-4.2 g/dL), Total bilirubin 23.4 (0.2-1.3 mg/dL), Direct Bilirubin 18.7 (0-0.2 mg/dL), AlkPhos 493 (110-320 U/L), ALT 118 (0-50 U/L), AST 281 (20-65 U/L), GGT 179 (0-65 U/L).

 

• Case 03 - H&E

• Case 03 - Trichrome

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Case 4: 

Patient A:

8 month old boy presenting with hepatomegaly. AlkPhos 212 (110-320 U/L); ALT 548 (0-50 U/L), AST 708 (20-65 U/L); Triglycerides 745 (<75 mg/dL); Normoglycemic.

 

• Case 04 - A01-H&E

• Case 04 - A02-PAD

• Case 04 - A03-PAS diastase

• Case 04 - A04-Oil Red O

• Case 04 - A05-Trichrome

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Patient B:

18 year old female with elevated liver enzymes, type 1 diabetes mellitus and Crohn's disease.

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• Case 04 - B01-H&E

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Case 5: 

9 year old boy, BMI at 91st percentile for age, elevated AST 153 (0-50 U/L), and ultrasound consistent with steatosis.

 

• Case 05 - H&E

• Case 05 - PAS

• Case 05 - PAS diastase

• Case 05 - Reticulin

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Case 6: 

6 year old boy presents with liver mass and pulmonary nodules. Alpha Fetoprotein 288,067 (0-8 ug/L)

 

• Case 06 - 01-H&E

• Case 06 - 02-Cytokeratin(AE1/AE3)

• Case 06 - 03-Vimentin

• Case 06 - 04-beta Catenin

• Case 06 - 05-Ini-1

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peritoneal implant 1 year later

• Case 06 - 06-H&E