Recipient Profile

 Name FATIMA TU ZAHRA

 MR # GOP-18
 Age 4 years
 Gender FEMALE
 Body weight 12 kg
 Height 89 cm
 BMI 15.1
 Blood group B+ve
Patient’s History

 Patient was well till age of 3 months then develop jaundice that was progressively inc
in nature ass with vomiting on off , pruritus,
 She was treated on opd bases and on liver supportive medications
 She had 1 episode of malena and epistaxis and her endoscopy was done on 2 feb
2021,THERE IS 1 COLUMN OF GRADE 3 VARICES,1 band ligation done
On examination:
 Vitals: HR 113 b/min, BP 100/60 mm Hg, Afebrile, RR 20 b/min
 FTT, Jaundiced, clubbed,

 Abdomen: soft, non-tender liver span 3CM, spleen 2CM BCM no ascites

 Chest: unremarkable

 CVS: S1 and S2 audible

 Genitourinary: unremarkable

 CNS: Intact
 Child pugh score 10C (29-5-2021)
Hematology (29-05-21) Biochemistry
WBC 10 Bilirubin 14.2mg/dl
Hb 10.3 AST/ALT 164/73U/L
Platelets 152 Albumin 2.4g/dl
ESR 20 Alkaline Phos 389
CRP 13.81mg/l GGT 32 U/L
Coagulation ELECTROLYTES
Alkaline Phos 389 S.K 4.0mmol/L vit D 17.3ng/ml
PT 19SEC S.Ca 8.2mg/dl Fibrinogen levels 228mg/dl
INR 1.5 S.Mg 1.9mg/dl G6PD Normal
APTT 36SEC S.Na 137mmol/L Creati nine 0.4mg/dl
Tumor Marker
AFP 12ng/ml

(31-12-20)
ECG Normal
Echo(EF 62%) Normal
Cardiac Fit for surgery
 LIVER BIOPSY: (18-07-2017)
 LIVER TISSUE CORE SHOWING MARKED CHOLESTASIS. SEPTAL FIBROSIS (GRADE
III) AND FOCAL MINIMAL PERIPORTAL INFLAMMATION(GRADE I)
DIAGNOSIS:

 Chronic liver disease d/t PFIC 4(TJP2 MUTATION)

Thank You