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Neonatal T y Hepatitis
Neonatal T y Hepatitis
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Kalliopi Patsiaoura
Hippokration General Hospital, Thessaloniki
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Original article
tory and factors associated with hepatobiliary disease in compliance. Regular follow-up was finally achieved for
infancy and the long-term prognosis of the non-familial 33 infants (twenty-three boys and 10 girls).
type of INH.
Liver biopsy was performed percutaneously by sin-
gle use Mengini needle (Hepafix 1,2 B Braun Melsun-
PATIENTS AND METHODS
gen AG) on all patients and considered successful only
One hundred thirty-nine children with moderate to when at least 5 portal spaces were included in the tissue
severe neonatal hepatitis were admitted to the 1st and 3rd obtained. All biopsies were done in the surgical depart-
Department of Pediatrics at Hippokration University ment with the patient fully anesthetized by a skilled an-
Hospital of Thessaloniki, from February 1984 to June esthetist. The obtained tissue was placed in a 10% for-
1997. An extensive clinical and laboratory evaluation was mol solution and sent to the pathology laboratory. Eight
performed (Table 1) to exclude all possible causes of patients had a second liver biopsy due to the persistence
neonatal hepatitis. More especially, the LFT tests includ- of clinical and laboratory findings (persistence of jaun-
ed total bilirubin, serum aminotransferases, serum albu- dice, hepatomegaly and transaminasaemia). All these
mins, alkaline phosphatase and serum gamma-glutamyl children are still being followed up.
transpeptidase estimated by routine methods (Depart-
The observation period for every child ranged from
ment of Biochemistry, Hippokration Hospital, Thessal-
9 months to 12 years with 3-6 month follow-ups for clin-
oniki, Greece). The normal values of LFT tests were as
ical and laboratory evaluation. The patients were dis-
follow: total bilirubin 0.1-1.1 mg/dl, alanine aminotrans-
charged from the study after being on clinical remission
ferase 7-33 iu/L, aspartate aminotransferase 10-34 iu/L,
with a normal biochemical profile for at least 6 months.
serum gamma-glutamyl transpeptidase 10-49 iu/L, alka-
line phosphatase 40-600 iu/L and total serum albumins
6.0-8.7 g/dl. RESULTS
We found forty-one infants with features of idiopathic Mode of presentation: Jaundice developed during
neonatal hepatitis (INH). The features for the diagnosis the first two weeks of life in twenty and up to the 3rd month
of INH included: a) the presence of conjugated hyperli- of life in twelve patients. In all cases total bilirubin was
birubinaemia ³ 3mg% lasting for more than 2 weeks in elevated over 12mg% (mean±SD, 13.35±2.45). One
the first 4 months of life, b) biochemical and/or histolog- patient was not clinically jaundiced. Associated features
ical features of hepatocellular damage and c) exclusion were history of prematurity, perinatal asphyxia, septi-
of all other causes of conjugated hyperbilirubinaemia. cemia, respiratory distress syndrome, and hypoglycemia
During the study eight patients dropped out die to no (Table 2). The liver was palpable in all patients at least
Table 1. Protocol for the study of Idiopathic Neonatal Hepatitis in early infancy
1. Detailed history
2. Physical examination
3. Daily stool color obsevation
4. Urine tests: Routine
Bacterial culture
CMV culture
5. Blood tests for the following:
Liver function tests: alanine and aspartate aminotransferase, alkaline phosphate, ã-glutamyl-transpeptidase, and serumpro-
teins
Clotting studies: prothrombin and partial thromboplastine time
Viral studies: heaptitis B, Rubella, toxoplasma, cytomegalovirus and Ebstein Barr viruses
Metabolic screen: plasma and urine aminoacids, urine reducing substances, serum a1-antithrypsin levels
a1-antithrypsin phenotype estimation
6. Imaging studies: abdominal ultrasound scan, scintigraphy scan
7. Liver biopsy: percutaneous liver biopsy
8. Maternal antiodies for CMV, toxoplasma, rubella, herpes simplex
114 K. SPIROGLOU, et al
slight elevation of aminotransferases (AST 40-60 iu/ml). jaundice in the first 2 weeks of life with the other half
becoming apparent up to the 4th month of life.8 In our
study, there was a significant predominence of males,
DISCUSSION
(male to female ratio 2.3:1) and 60% of our patients de-
Neonatal hepatitis syndrome is a term given to a non- veloped jaundice during the first 2 weeks after birth. A
specific hepatic inflammation, which develops second- history of prematurity was also present in 14 infants.
ary to many different etiologies.,8 INH, also known as These data are in accordance with the international lit-
giant cell hepatitis and cryptogenic hepatitis, is respon- erature.3,8
sible for 50-70% of the NH cases.3 By definition, no caus-
The overall prognosis in INH is difficult to estimate,
ative agent can be found in a baby with INH.1,4 It is now
because of the variability of the clinical course and the
considered a dinstict clinical entity with biochemical and
unclear pathogenesis.14,15 Nevertheless the prognosis is
histological features3,8 of cholestasis.
generally good, although different studies have shown
INH is more common in males, small for dates and different mortality rates. Danks et al mentioned a 30%
premature neonates.1,8 In half of the cases it presents with overall mortality in the 1st year of life10 comparer to 55%
116 K. SPIROGLOU, et al
mortality described by Henriksen et al in 1981.4 Other ally quite severe with acholic stools, hepatosplenomega-
studies showed a better outcome with a significantly lower ly amanifestations (e.g. hypoglacaemia, prematurity etc)
mortality rate ranging from 6.5%6 to 12.9%.11 A positive the overall mortality seems to be low apart from the fa-
family history is associated with an underlying bile acid milial cases. Associated problems such as developmen-
metabolism disorder and carries a bad prognosis.16 Oth- tal delay should be recognized and addressed promptly.
er predictors of poor prognosis include prolonged severe
jaundice (beyond six months of age), acholic stools, per- REFERENCES
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