A CASE OF CLD


Dr.Shobika, Dr.Bhanu vikraman pillai ,Dr.Rekha Hari Dr.Anupa Thomas ,DrC Jayakumar

 
Fifty day old female ba, presented with history of pale coloured stools and high coloured urine since one week of age.
Ba was evaluated at outside hospital from 3 weeks of age,serial monitoring of LFT and INR was on increasing trend.
USG done showed mild enlarged liver with normal echotexture,gall bladder – partially contracted.
HIDA scan showed Transient cholestasis/bile plug syndrome.
Ba received one dose of IVIG and referred here due to persistent cholestasis. (reason unknown)
At admission,ba was irritable, pale jaundiced,with few petechiae over abdomen
P/A: distended ,dilated veins ,umbilical hernia+,liver 3cm below RCM ,spleen palpable 3cm below LCM and moderate Ascites 

Labs:
TC:21.58ku/ml
Hb:7.6g/dl
Platelet:196ku/ml
CRP:7.25mg/l
TSB/DSB-23/19mg%
SGOT/PT: 1138/237
S.Albumin:3.4gdl
ALP:473IU/L
GGT:84u/l
INR1.7
Ammonia 147

Provisional Diagnosis considered:
Jaundice with hepatosplenomegaly, ascites 

Possibilities considered:
Chronic Liver Disease with normal GGT
Ruled out Biliary atresia
Sepsis
Intra uterine Infection
Storage disorder
Progressive fibrosing intra hepatic cholestasis

Further investigations done: 
TORCH IgM: CMV IgM positive
Peripheral Smear: Leucocytosis,microcytic hypochromic anemia 
Lymphocyte subset analysis (NBT -DHR ) was sent in view of any underlying immunodeficiency presenting as sepsis
Urine reducing substance was positive
Blood for CMV DNA PCR was positive.

Ba was admitted to PICU and was started on IV antibiotics,IV N Acetyl cystine,Inj.Vitamin K and bowel wash
Oral Valganciclovir was also added.

Feed was initially changed to soya formula as urine reducing substance was positive.

USG abdomen showed multiple disseminated hypoechoic lesions 
Hence a CECT abdomen and chest waa done to r/o infiltrative lesions and tuberculosis which showed Hepatosplenomegaly,small hypodense lesion segment 7 liver.Intrahepatic branches of portal vein are markedly attenuated showing poor contrast opacification with portosystemic collateral noted along anterior abdominal wall.moderate ascites,umbilical hernia with bilateral indirect inguinal hernia.

Coagulopathy was corrected with serial FFPs and ba was taken up for USG guided liver biopsy under anesthesia

Ba was shifted out to ward after stabilization.

Serial monitoring of LFT showing increasing trend along with progressive ascites, hence ba was started on furosemide and spironolactone.
Eye examination No e/o intrauterine infections,storage disorders or posterior embryotoxon.

Genetics consultation was availed and advised to send for WES and TMS GCMS.

TMS-GCMS showed normal galactose levels,ba was restarted on breast feeds

Total carnitine and Acyl carnitine,urine succinyl acetone( in view of tyrosenemia) were negative

Liver Biopsy result showed neonatal giant cell hepatitis with fibrosis.
IHC antibodies: include BSEP and MDR3 -canalicular staining,CD10- reduced canalicular staining,CK7 -bile ducts,ductules and very focal biliary metaplasia.
S/o Progressive Fibrosing Intra Hepatic Cholestasis Type 1 

Diagnosis : Decompensated Liver Disease

Possibilities: PFIC ( in view of conjugated hyperbilirubinemia with normal GGT
Super added CMV Infection ( As congenital CMV Infection alone unlikely to present as decompensated CLD)
Congenital Porto systemic shunt ( in view of hyperammonemia 
Storage disorder

Transplantation surgery consult was sought liver transplantation is planned for the ba.

CHRONIC LIVER DISEASE:
A progressive deterioration of liver functions for more than 6 months (in infants there is no strict adherence of minimum 6months duration to have CLD)which includes synthesis of clotting factors,other proteins,detoxification of harmful products of metabolism and excretion of bile.

Etiology: Can classify into metabolic auto immune etc 
NAFLD (Older children)
Chronic viral hepatitis 
Genetic causes:
Alpha 1 antitrypsin deficiency 
Wilson disease 
Autoimmune causes:
Primary biliary cirrhosis 
Primary sclerosings cholangitis
Drugs: amiodarone,methotrexate

Clinical manifestations:
Features of Portal hypertension 
Features of liver cell failure 

Diagnosis:
Labs -persistently elevated levels of AST/ALT/LFT/PT/APTT/INR
USG abdomen,CT,
Transient Elastography,upper endoscopy,liver biopsy

Staging:
Hepatitis 
Fibrosis
Cirrhosis 
Hepatocellular carcinoma

Prognosis:
Child Pugh score:uses ascites,bilirubin,albumin,PT and encephalopathy 
CLASS A(score 5-6) :well compensated disease 
CLASS B(score 7-9): functional compromise 
CLASS C(score10-15):
Decompensated liver disease

Complications:
Varicella bleeding
Ascites
Hepatopulmonary syndrome
Hepatocellular carcinoma 
Hepatic encephalopathy 

Management:
Supportive treatment
Corticosteroids in autoimmune hepatitis 
Liver transplantation 

Take home message:The child with suspected liver disease should be vaccinated against hep A and hep B,child with CLD may need liver transplantation as a definitive treatment.

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