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Child with cholestasis and
massive hepatomegaly
Marija Dimitrovska-Ivanova
Faculty of Medical Sciences - Stip
University Children’s clinic-Skopje
Republic of Macedonia
Medical history
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Patient: B. Lj. 2.5 years, female
Third child from third normal pregnancy.
Born normaly in the 41 gestational week with born
weight 3150g, born length 50 cm and APGAR SCORE
9/10.
The baby was breast feed just for a month and than
was introduced formula milk until one year. Other non
milk food was administrated in 4 months of age. Poor
weight gain during infancy.
Because of frequent respiratory infections child was
treated by a family doctor with wide spectrum
antibiotics per os.
In the last 5 months with frequent stools, decreased
appetite and poor weight gain.
Clinical status at admission
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Child was taken to the family pediatrician who noticed
jaundice, large abdomen and massive hepatomegaly.
She was first hospitalized in General hospital in Ohrid
and after excluding viral hepatitis was transferred to
the University Children’s Clinic – Skopje
At admission girl was in malnutrition and in bad general
condition, she looked seriously ill, prostrated, febrile,
tachicardic, icteric . Her weight was under 3% il
(BW = 9 kg ) and her height on the 3 % il for age.
Auscultation of lungs - vesicular breathing with
crackles.
Abdomen - Above the chest, distended and hard on
palpation. Liver was palpable 9 cm under right cost
arch and fulfilled the left hypochondrium
Laboratory evaluation
Hgb = 97…88…72…149…128 g/l
 Er = 3,04…2,38…2,48…5,27…4,41 x 10^12/l
 Le = 13,7…12,3…13,1…9,3…9,1 x 10^9/l
 Sed rate = 67…44 CRP= 38,8…42,2 mg/l
 Fe serum = 6,6 µmol/l.
K= 2,9…3,6…4,3
 ABS - Ph = 7,49 HCO3 = 33,1 mmol/l, pO2 = 7,77
kPa (hypoxemia), Pco2 = 5,82 kPa
 Total proteins = 46...54...65…54g/l
Аlbumen=23…30…22…26
 IEPh, alfa – fetoprotein, alfa – 1- antitripsin,
ceruloplazmine and antibodies for autoimmune
hepatitis type 1 and type 2 were normal
 Serum creatinin, urea and amino acid screening in
plasma and urine were normal
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Laboratory evaluation
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Liver tests – Tot. bil. = 40...52...94…38, Conj. bil.=
26...38....19...18
AST= 93…68…317…139 U/L
ALT= 41…43…60…52 U/L, GGT= 123…200 U/L,
ALKP= 109 U/L
The protrombin time was 51s (normal <13 s) and
PTT was 71 s (normal <33 s).
TTG test – negative
Sweat test = 110 mmol/l
Genotype – F 508 del/ F 508 del
Liver biopsy – steatosis of liver with poor chronic
inflamatory infiltrate
Ultrasound of abdomen-hepatomegaly,
the liver is extremely hiperechogenuous
CT of abdomen - Liver with homogeneous
structure with hypodensity on parenchyma and
present hepatomegaly that fulfills the left
hemiabdomen
Chest x ray
Consolidation in
projection of the
lingula of the
left lung lobe
On tracheal
aspirate was
isolated
Staphylococcus
aureus and
Pseudomonas
aeruginosa
Treatment
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Parenteral antibiotic therapy according to antibiogram
(S. aureus and P. aeruginosa) Amp. Meronem i.v and
per os trimetoprim sulfotetoxasole.
I.V Albumin was administrated in 4 times, Er
transfusion, Vit. K, ursodeoxycolic acid and
hepatoprotectiv therapy.
Pancreatic enzymes, oral nutrition energy
supplementation, supplementation of liposoluble
vitamins A, D, E, K and beta – caroten.
After one month child was discharged in general stable
condition with better appetite, normalization of stools,
progressive weight gain, without respiratory symptoms.
Follow up treatment - eradication therapy for P.
aeruginosa (Colomycin for 3 months), Pancretic
enzymes, Vit. ADEK, hyper caloric meals, oral
nutrition energy supplementation and physical therapy.
Current nutritional status
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Child is follow CF outpatient clinic.
She was no hospitalized during 3 years.
The child is making a good weight gain BW= 20,3
kg (75 %il) Body height= 105 cm(10 %il) and
BMI= 18,4.
On the last control with gradual reduction of
hepatomegaly, but with present nodular cirrhosis.
Without exacerbation on lung desease.
Conclusion
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Delayed diagnosis of Cystic fibrosis
Early begining of liver disease with signs of liver
insufiency.
Steatosis and nodular cirrhosis of liver.