Dr.Ghaniya KC , Dr. C Jayakumar
Amrita Institute of medical sciences
Kochi
Fourteen year old female child developmentally normal, immunized for age presented with moderate grade intermittent fever (Tmax-100 F) since five days ,
Two episodes of non-projectile, non-bilious vomiting and yellowish discoloration of sclera , bilateral upper and lower limbs since 2 days.
H/o outside food intake present
H/o consumption of ayurvedic medicine six months ago is also noted
No h/o cough/abdominal pain / rashes / bleeding
On Examination : Child is sick looking
Restless and irritable
icterus +
No pallor , cyanosis, clubbing, lymphadenopathy, edema
Systemic examination:
P/A : soft, Hepatic tenderness + , BS +
RS : Normal
CVS. : S1 S2 + no muffling ,no murmur or cardiac enlargement present
Differential diagnosis
1Acute infective hepatitis
2 Drug induced hepatitis
3 Other bacterial cause of hepatitis
4CLD
Investigations :
TC – 10.7 K, N/L – 63/21 %, Hb- 5.59 , Plt – 176 K/ul, CRP – 9 mg/dL
LFT – TB – 9.98 / DB – 6.40 / AST – 784/ ALT – 2225 , INR – 7 , Ammonia – 86.5
Dengue panel – negative
IgM HAV and HEV – negative
Pre op serology – negative
Blood and urine culture – sterile
PS : Microscopic hypochromic anemia with neutrophilic leukocytosis
CT brain – No intraparenchymal / extra axial bleed
CT abdomen – Liver shows diffuse fatty Gastric mucosal hyperenhancement.
GB wall edema with marked mucosal hyper enhancement.
Splenomegaly. Mild ascites.
Course : Child was admitted in gastro ICU and acute liver failure protocol started .
Initial acute liver failure treatment started , in view of encephalopathy and high INR , pediatric gastro consult was given and advised Continuous renal replacement therapy (CRRT) and plasma exchange ( PLEX) , Nephrology consult was given in view of worsening ammonia levels and encephalopathy, she was started on CRRT and PLEX , unfortunately both failed and parents were informed the need for emergency transplant and donor evaluation was conveyed. Child underwent liver transplant with mother being her donor
Pediatric Acute Liver Failure (PALF)
Clinical presentation : abdominal discomfort and malaise with or without fever, jaundice and encephalopathy and hepatomegaly are common.
Diagnosis : increased liver enzymes increased bilirubin , increased PT INR
Causes :
1. Acetaminophen : acute chronic overdose and is potentially treatable if rapidly diagnosed. antiseizure medication, including valproate are the most common drugs associated with PALF especially in patients with underlying mitochondrial disease
2. infectious : HAV.HSV can occur in all age group, specially young infants.
3. Autoimmune hepatitis is most common among adolescents, but is responsible for more than 5% of PALF in younger age groups
4. In newborn infants, gestational, alloimmune liver disease, HSV, inborn errors of metabolism, enclosing tyrosinemia and galactosemia and mitochondrial hepatopathies
5. Wilson’s disease is the most common metabolic condition associated with PALF in children over five years of age DCT negative hemolytic, anemia, hypo anemia, low cell plasm, low, alkaline phosphate
6. 30 to 50% cause not discovered categorized as indeterminate