Dr.Shobika Dr.Bhanu vikraman pillai Dr.C Jayakumar
AIMS kochi
Four year old male child who was developmentally normal and immunized presented with decreased appetite and tiredness of 1 week duration.
H/o Yellowish discoloration of eyes and urine of one day duration
History is negative for travel, food from eateries
No past history of relevant illness
No family history of liver disease and nobody in the family underwent liver transplantation
On examination,the child was tired looking,mild pallor,mild icteric
P/A-soft,Liver enlarged 3cm below right costal margin, slightly firm,No splenomegaly ,BS+
Other system normal
No KF ring
No spiders
Labs showed normal counts with negative CRP.
LFT – SGPT- 2223.6iu/l;
SGOT-1330.3iu/l;
ALP-748
TSB-9.94;DSB-6.98,
PT-INR-33.3/14.7/2.36;
APTT-38.9/30.5
Serum paracetamol level quantitative of <5mcg/ml. Normal
USG abdomen- no increased echo texture normal,
Blood and urine culture were sterile
IgG levels were positive for ANA-IFA
Diagnosis of Acute liver failure, underlying Autoimmune liver disease
The child is taken up for USG guided Liver biopsy under anaesthesia after correcting coagulopathy with FFP prior to procedure.
Liver biopsy showed acute cholestatic hepatitis.
The child was treated with
inj.N-acetylcysteine infusion,
Piptaz,
Vitamin K, Steroids & fluidsSerial monitoring of LFT done.
The child discharged with stable vitals.
and transaminitis and Bili came to normalcy
Acute liver failure:characterized acute liver injury with impaired synthetic function and altered mental status in a patient without cirrhosis or preexisting liver disease.
Duration:
Hyperacute- <7 days
Acute -7 to 21 days
Subacute- 21 to 26 days
Causes:paracetamol
Viral hepatitis
Autoimmune hepatitis
Wilson disease
Symptoms:
Lethargy
Fatigue
Nausea
Vomiting
Jaundice
Abdominal distension from ascites
Grading:
Grade 1-changes in behaviour,slurred speech
Grade 2-moderate confusion
Grade 3-marked confusion
Grade 4-coma,unresponsiveness to pain
Investigations:CBC
PT/INR,LFT,bilirubin level, platelet,paracetamol level
Anti hepatitis A,B,C.
Management of acute liver failure: N acetyl cysteine
Loading dose: 150mg/kg iv over 1hr.
Maintenance dose: 50mg/kg over 4hrs then 100mg/kg over 16hrs thn 100mg/kg/day upto 7 days depending on clinical response.
We should monitor ALT levels. Bleeding prevention,treat the underlying cause.
Management of autoimmune hepatitis: Corticosteroids-predisolone40 to 60mg daily for 1st 2 weeks and then taper 5 to 10mg 2 weeks until 20mg reached and then taper 5mg for two weeks target dose of 10mg daily.monitor CBC.
Immunosuppressants-Azathioprine 5 to 10mg per week until a dose of 10mg daily is reached while maintaining an azathioprine dose of 50mg for first 2 months of therapy.
Monitor CBC.
Take home message:
Children with jaundice ,possibility of liver failure should be considered.