CASE REPORT

HEPATOLOGY DIVISION

MEISY GRANIA
Child Health Departement , Faculty of Medicine,
Hasanuddin University /
RSUP dr. Wahidin Sudirohusodo, Makassar
 Cholestasis : clinical syndrome caused by
decrease bile flow and by conjugated bilirubin
accumulation due to impaired hepatobilier
function, either intrahepatic or extrahepatic.

Characterized by jaundice, acholic faeces, dark

(tea-colored) urine, other manifestation : liver
enlargement, spleen enlargement, bleeding
manifestation and itchy in chronic onset
cholestasis
 The most hepatobiliary disease, incidence of 1:2500
birth

Biliary atresia being the major cause.

Intrahepatic cholestasis = hepatocellular cholestasis is

a clinical syndrome caused by the obstacle of
secretion and/or the flow of bile that occurs in the
liver, usually occurs within the first 3 months of life.
 Carolis disease and carolis syndrome are
rare congenital disorders of the
intrahepatic bile ducts
characterized by dilatation of the
intrahepatic biliary tree
The term carolis disease is applied if the
disease is limited to segmental dilatation
of the larger intra-hepatic ducts
 KM, a girl, age 1
year 1 month.
Referred from
Konawe Hospital was
admitted to RSWS
pediatric emergency
department on 20st
Sept 2015 with
diagnosis of
obstructive icteric
 Liver :not enlarged,
parenchymal echo is within
normal limit. Intrahepatic
and ekstrahepatic bile duct
appear to be dilated. No
mass / cyst are visible.

GB : wall doesnt appear to

be thickened, no stone are
visible.

Conclusion :Caroli disease

appearence
Follow Up
 Follow Up day 1 (September 20th,th2015)
Follow Up day 1 (September 20 , 2015)
General Condition :
Fever. Pale stools (+).
Complete blood count:
BP 90/60 mmHg, PR 120 x/minute, RR 40
Hb 6,1 gr/dl, erythrocyte: 2,18x106/uL,
x/minute, T 37,80C.
white blood cell 15.100/uL, platelet
Sclera and skin were ikteric.
256000/uL, hematocryte 18,3%, MCV
abdominal examination: Palpable liver 6 84 fL, MCH 28,1 pg, MCHC 33,4 g/dL
cm below arcus costae, supple in .
consistency, smooth surface, and sharp
Blood chemistry
edge. Spleen was palpable at schufner 1
total Bilirubine 11,51 mg/dl, direct
Treatment bilirubine 9,01mg/dl, indirect bilirubine
2,3 mg/dl, albumine 2,3 gr/dl, GDS 60
Ursodeoxicolate acid 10 mg/kg bw/day = 15
mg/dl, SGOT 531 U/L, SGPT 321 U/L, PT
mg/12 hours/oral. 18,9 detik. APTT 50 detik,
Ampicillin 150 mg/6 jam/intravena
Gentamicin 15 mg/12 jam/intravena
Paracetamol 60 mg/8 jam/intravena
Transfusion of packed red cell Blood smear
Transfusion of albumin Conclusion : Leucocyte with
infection sign
Nutritional marasmus management
I II III
 Follow Up day 3 (September 22th,th2015)
Follow Up day 3 (September 22 , 2015)

General Condition :
No fever. Pale stools (+).
Complete blood count:
Hb 9,8 gr/dl, erythrocyte: 3,62x106/uL, white
BP 90/60 mmHg, PR 120 x/minute, RR 40
blood cell 17930/uL, platelet 199000/uL,
x/minute, T 37,00C.
hematocryte 30,2%, MCV 83,4 fL, MCH 27,1
Sclera and skin were ikteric. pg, MCHC 32,5 g/dL.
abdominal examination: Palpable liver 6 cm below Albumin 2,5 gr/dl, Ferritine 23,57
arcus costae, supple in consistency, smooth
surface, and sharp edge. Spleen was palpable at
Anti toxoplasma Ig M 0,03 (normal <0,65),
schufner 1
anti CMV Ig G 159 (normal <6), CMV Ig M
Treatment 0,9 (normal <0,9), anti rubella Ig G 11
(<14), anti rubella Ig M 0,30 (normal <1,2)
Ursodeoxicolate acid 10 mg/kg bw/day = 15 mg/12
hours/oral.
Ampicillin 150 mg/6 jam/intravena Fecal analysis
Gentamicin 15 mg/12 jam/intravena Consistency: soft , Color : pale yellow
MCT formula milk
Blood smear
Nutritional marasmus management Conclusion : Leucocyte with
infection sign
Advice
Consult to social pediatric, infection, hematology
division.
 Follow Up day 4 (September 24, 2015)
Follow Up day 4 (September 24, 2015)
General Condition :
No fever. Pale stools (+).
BP 90/60 mmHg, PR 124 x/minute, RR 40
x/minute, T 36,70C. Consults result :
Sclera and skin were ikteric.
abdominal examination: Palpable liver 6 Social Pediatric division : immunization
cm below arcus costae, supple in not complete
consistency, smooth surface, and sharp
edge. Spleen was palpable at schufner 1 Infection division : plan to mothers
Treatment torch laboratory
Ursodeoxicolate acid 10 mg/kg bw/day = 15
Hematology division : anemia by
mg/12 hours/oral. chronic disease, observation of anoxia
Ampicillin 150 mg/6 jam/intravena sign and overcome of underline
disease
Gentamicin 15 mg/12 jam/intravena
MCT formula milk
Kolesteramin 2 gr/8 jam/ NGt
Nutritional marasmus management
 Follow Up day 8 (September 28, 2015)
Follow Up day 8 (September 28, 2015)
General Condition :
No fever. Pale stools (+).
BP 90/60 mmHg, PR 120 x/minute, RR 38
x/minute, T 36,70C.
Sclera and skin were ikteric.
abdominal examination: Palpable liver 6
cm below arcus costae, supple in
consistency, smooth surface, and sharp
edge. Spleen was palpable at schufner 1 USG abdomen : suspect of carolis
Treatment
disease
CT scan abdomen : carolis
Ursodeoxicolate acid 15 mg/12 hours/oral. disease appearence
Kolesteramin 2 gr/8 jam/ NGt
MCT formula milk
Nutritional marasmus management
 Follow Up day 10 (September 30, 2015)
Follow Up day 10 (September 30, 2015)

General Condition :
No fever. Pale stools (+).
BP 90/60 mmHg, PR 124
Treatment
x/minute, RR 38 x/minute, T
36,60C. Ursodeoxicolate acid 15 mg/12
Sclera and skin were ikteric.
hours/oral.
abdominal examination:
Palpable liver 6 cm below arcus MCT formula milk
costae, supple in consistency, Nutritional marasmus
smooth surface, and sharp
edge. Spleen was palpable at management
schufner 1
 Cholestasis due to carolis disease
Nutritional marasmus
Anemic by chronic disease differential
diagnosis iron deficiency anemic
 Qua ad vitam : dubia
Qua ad sanationem : dubia
Discussion
accumulation, retention & regurgitation of bilirubin, bile acid,
also cholesterol into the plasm & damage the liver cells in
many levels of clinical features
 Carolis syndrome is a developmental anomaly. It
is related to ductal plate malformation at different
levels of the intrahepatic biliary tree
On a genetic level, unbalanced translocation
between chromosome 3 and 8 or the structural
rearrangement of genes located
 history taking : jaundice since two weeks of age, dark
colored urine and pale stooles

physical examination : icterus and enlarge liver

laboratory result :increased of direct

hyperbilirubinemia, normal of transaminase enzyme,
and positive IgG anti-CMV

USG :carolis disease appearance

CT scan abdomen : carolis disease appearance
 Direct bilirubin is increased if its serum level is > 1
mg/dl when total bilirubin < 5 mg/dl or direct
bilirubin serum level 20 % when total bilirubin serum
level > 5 mg/dl
in this case : total bilirubin 11,51mg/dl, direct
bilirubin 8,41 mg/dl, indirect bilirubin 3,1 mg/dl
Bilirubin is also found in the urine.
in this case : bilirubin urin +
serum aminotransferase also increase 2-4x above
normal level
in this case : SGOT and SGPT are normal
Albumin level is usually normal in the beginning of the
disease
Positive IgG anti CMV.
 to improve bile flow, by treating cholestasis etiology
using medicine in hepatocellular cholestasis,
stimulating bile flow with ursodeoxycholic acid

To improve patients nutritional state

addressing complications such as hyperlipidaemia

/xantelasma is using kolestipol and in liver failure and
uncontrolled pruritus is by liver transplant

to give psychological support as well as to provide

family education.
 the long-term prognosis is determined mainly by
the frequency and the gravity of the episodes of
cholangitis that can lead to sepsis and death or
creation of hepatic abscesses.
Hepatic insufficiency can develop and
transplantation of the liver may be required
Thank you