4. BIRTH HISTORY : Non significant.
IMMUNIZATION HISTORY :Completely vaccinated according to EPI schedule.
NUTRITION HISTORY : Consumes up to 1200 kcal per day including dairy,
vegetables and meat. No history of fava bean consumption in diet.
DEVELOPMENTAL HISTORY : Developmentally normal
5. PAST MEDICAL HISTORY : No significant history of hospital admissions and IV
medications before this illness.
PAST SURGICAL HISTORY : Non significant.
FAMILY HISTORY : 6th product of consanguineous marriage. All elder siblings
are alive and healthy. Mother had history of hepB which was treated before
her birth.
DRUG AND TRANSFUSION HISTORY : No history of blood transfusions. No
significant history of prolonged use of painkillers , anti epileptics or any other
drug.
6. PERSONAL HISTORY : sleep and eating habits were normal before this illness.
Normal bowel and bladder habits. Drinks unboiled tap water.
SOCIOECONOMIC HISTORY : belongs to middle class family, lives in well
ventilated house.
7. GENERAL PHYSICALEXAMINATION
Young girl lying on bed well oriented with following
vitals:
HR = 84b/m
RR= 24b/m
BP = 100/60mmhg ( 50th centile)
SO2= 96% at room air
Patient has A+, CL+, E+ at the time of admission while
No signs of J-,CY-, LN-,D-
Weight = 30kg (-1.23 SDS)
Height =130cm ( -2.22 SDS)
8. HEADTO TOE EXAMINATION
Clubbing positive
Nail & palmer crease pale
Purplish discoloration of skin at both right lumber
regions
No cervical lymphadenopathy.
No gum bleeding or oral ulcer.
No jaundice ,Palmar erythema, Asterixis,
koilonychias, leukonychia, Spider navi, Caput
medusa ,Telangiectasia
BCG scar present.
All joints normal with no tenderness & swelling
No bruising or bleeding seen With no spider nevi
seen
9.
10. ABDOMINAL EXAMINATION
on inspection abdomen is grossly distended with slit like
centrally placed everted umbilicus ,abdomen is moving with
respiration. Purplish discoloration of both sides of lower abdomen
is present extending to back , cough reflex is -ve
on palpation abdomen is soft distended mildly tender and
abdominal grith is 40cm
visceromegaly not appreciated due to massive ascites
on percussion : fluid thrill and shifting dullness is positive
on auscultation gut sounds audible with no abdominal bruits
perineum and genital exam is unremarkable with no pubic hair
12. DIFERENTIAL DIAGNOSIS :
CHRONIC LIVER DISEASE SEC TO :
METABOLIC LIVER DISEASE ( WILSON )
AUTOIMMUNE HEPATITIS
CHRONIC VIRAL HEPATITIS ( HEP B OR HEP C)
17. Ultrasound
abdomen
Liver small in size with
heterogenous texture
and irregular margins
Portal veins dilated but
patent
Multiple abnormal
vascular channels seen in
epigastric ,
peripancreatic and peri
splenic area.
Massive ascites.
18. T.BILI > 3 3 POINT
S.ALBUMIN 2.0 3 POINTS
PT/INR 1.6 1 POINT
ASCITES MODERATE 2 POINTS
ENCEPHOLPATH
Y
NONE 1 POINT
TOTAL SCORE 10 POINTS
19. Workup for Wilson disease :
Serum ceruloplasmin : 0.29 age 4-12yrs ( 0.25-0.45
)
Urinary copper : 19.08 ug/Day normal : (less than
60ug/day )
24. FINAL DIAGNOSIS :
Decompensated chronic liver disease secondary to
Autoimmune hepatitis ( type 1 ) .
Abdominal wall cellulitis secondary to pressure sores.
25. GASTRO OPINION
Gastroenterology was taken on board , they advised to
continue conservative management .
Add tablet rifaximine and tab Aldactone.
Liver biopsy in plan.
26. MANAGEMENT IN WARD
Admit in unit 3
IV line maintained
IV fluids started
IV antibiotics started
Inj vit K and omeprazole started.
Syp duphalac 30ml HS
Tab Aldactone 25mg 1+1+1
Tab rifixamine 500mg 1 OD
27. Findings are likely of
cirrhosis of liver with
portal hypertension
without portal venous
thrombosis.
29. AUTOIMMUNE HEPATITIS
Autoimmune hepatitis is a chronic disease of unknown cause,
characterized by continuing hepatocellular inflammation and necrosis
and has a tendency to progress to cirrhosis.
Autoimmune hepatitis may present as acute or chronic hepatitis or as
well-established cirrhosis, although in rare cases it presents as
fulminant hepatic failure.
Autoimmune hepatitis can present at any age and in all ethnic groups,
but it occurs predominantly in women. For type 1 autoimmune
hepatitis, the female to male ratio is 4:1, but for type 2 autoimmune
hepatitis, the ratio is 10:1
32. DIAGNOSIS :
Evidence for Autoimmune hepatitis include:
Elevation in transaminases.
Association with hypergammaglobulinemia and the
presence of a rheumatoid factor
Circulating autoantibodies (ie, nuclear, smooth muscle,
thyroid, liver-kidney microsomal, soluble liver antigen,
hepatic lectin)
Hepatic histopathologic lesions composed predominantly
of cytotoxic T cells and plasma cells
Association with other autoimmune diseases
Response to steroid and/or immunosuppressive therapy
39. TREATMENT END-POINTS :
Patient may achieve 1 of 4 end points
Remission: Remission is indicated by the absence of symptoms,
normalization of aminotransferases, and histologic improvement to
normal or minimal inflammatory activity on liver biopsy.
Treatment failure: Treatment failure is defined as deterioration in a
patient's clinical condition, laboratory tests, or histologic features
during therapy.
Incomplete response: Incomplete response is defined as an
improvement that is insufficient to satisfy remission criteria. It is
estimated to occur in 13% of patients.
Drug toxicity: Drug toxicity may occur. Patients must be tapered off
from the culprit medication. Some patients successfully achieve
treatment goals on alternative medications.
40. RELAPSE
Relapse occurs in 50% of patients within 6 months of
treatment withdrawal and in 80% of patients within 3 years
of treatment. Reinstitution of the original treatment
regimen usually induces another remission; however, relapse
commonly recurs after a second attempt at terminating
therapy. The major consequence of relapse and re-treatment
is the development of drug-related complications, which
occurs in 70% of patients
41. PROGNOSIS :
Without treatment, approximately 40% to 50% of the individuals
with severe disease will die within 6 months to 5 years.
Treatment with steroids has dramatically changed the course of
the disease. Most patients respond to therapy and the 10-year
survival rate is approximately 83.8% to 94%.
The long-term outlook after liver transplantation is excellent,
with 10-year survival rates reported as greater than
70%.Positive autoantibodies and hypergammaglobulinemia tend
to disappear within 2 years of transplantation.
Treatment failure, Relapse and need for long term Maintenance
is common in type 2 AIH. So, compared to type 1 AIH it’s
prognosis is poor.
