• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.sup2
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• pp.35-43
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• 2008

Jaundice is common in breast-fed infants. Any infant noted to be jaundiced at 2 weeks of age need to be evaluated for cholestasis with measurement of total and direct serum bilirubin. The most common causes of cholestatic jaundice in infants are biliary atresia and neonatal hepatitis. Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and idiopathic neonatal hepatitis is decreasing. Cholestasis should be investigated using a structured protocol. Early detection and timely, accurate diagnosis is important for successful treatment and a favorable prognosis. In particular, a Kasai portoenterostomy for biliary atresia has the best outcome if performed before the infant is 8 weeks of age. The management of cholestasis is mainly supportive, including nutritional support and alleviation of symptoms to improve the quality of life. Specific treatments are available for some causes of neonatal hepatitis syndrome and should be started as soon as possible. For decompensated liver disease, liver transplantation yields a better outcome.

• Neonatal Medicine
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• v.17 no.1
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• pp.123-126
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• 2010

Hepatitis A virus (HAV) infection is a common cause of acute hepatitis, but is rarely responsible for neonatal hepatitis. HAV infection is usually transmitted by the fecal-oral route, but during the neonatal period can be transmitted by the intrauterine vertical route or postnatal horizontal route. HAV infection is usually self-limited, but it can be potentially life-threatening in adults. The clinical course and symptoms are mild and the infection can be asymptomatic in newborns. Recently, we experienced a case of symptomatic neonatal hepatitis A. To our knowledge, this is the first case of neonatal hepatitis with HAV infection in Korea.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.242-248
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• 2006

Purpose: Neonatal hepatitis is the major cause of neonatal cholestasis and may be divided into infectious, metabolic, genetic, and idiopathic neonatal hepatitis. Non-familial, non-metabolic, and non-A, B, C viral neonatal hepatitis is known to have made satisfactory progress, but little is known about its chronic clinical features. Methods: Clinical and histological assessments were carried out in 34 cases with chronic neonatal hepatitis [elevated serum alanine aminotrasferase (ALT) level for more than 6 months] except for A, B, C viral hepatitis, metabolic, or genetic neonatal hepatitis, who were admitted to the Department of Pediatrics, Pusan National University Hospital, from January 1998 to January 2004. Results: Males were more common (70%). Jaundice (100%) and hepatomegaly (44%) were frequent manifestations. Peak serum ALT levels were most commonly below 300 IU/L in 41.2% of patients and peak serum direct bilirubin levels were most commonly between 1.0~5.0 mg/dL in 50% of patients. Ten cases (34%) of 29 patients had positive serum cytomegalovirus (CMV) IgM or urine CMV polymerase chain reaction. Serum ALT level was normalized within 1 year in 11 (37.9%) of 29 cases, and within 2 years in 9 (69.2%) of 13 cases. Serum ALT level was elevated persistently over 2 years in four (30.7%) of 13 cases. Histologic findings such as portal or periportal activity, lobular necrosis, portal or periportal fibrosis were more severe in patients with persistent ALT elevation over 2 years than in those showing normalization of ALT within 2 years (p>0.05). Conclusion: When the elevation of ALT level sustains over 1 year in non-familiar, non-metabolic, non-A, B, C viral neonatal hepatitis, an assessment of the severity of liver injury and a careful monitoring about chronic liver disease may be required.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.177-193
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• 2005

Purpose: To identify genes specifically expressed in biliary atresia, we compared the patterns of gene expression between biliary atresia and neonatal hepatitis syndrome using cDNA microarray analysis. Methods: Liver tissues were taken from livers of 11 patients (7 patients with biliary atresia and four with neonatal hepatitis) with neonatal cholestasis by needle biopsy. Normal control could be obtained from donor liver tissue during living-related liver transplantation. Total RNA was extracted from each samples and reversely transcribed to make cDNA. Then fluorescent cDNA were pooled and hybridized to the clones on the microarray. Fluorescence intensities at the immobilized targets were measured. Utilizing cDNA arrays of 4.7 K human genes, gene expression profiles were analyzed. Results: Among 4,700 microarray clones, 17 cDNA clones were significantly over-expressed in all 11 patients with neonatal cholestasis, while 20 clones were significantly decreased. Genome-wide expression analysis was carried out in livers obtained at the time of diagnosis. We could identify 49 genes, in which there showed differential expression between biliary atresia and neonatal hepatitis syndrome. Conclusion: This study shows the pattern of differentially expressed genes in biliary atresia and neonatal hepatitis syndrome. We believe that this study can contribute to the understanding of pathogenesis of neonatal cholestasis.

• The Korean Journal of Nuclear Medicine
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• v.28 no.3
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• pp.357-363
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• 1994

We evaluated the diagnostic accuracy of $^{99m}Tc$-DISIDA scintigrauhy as a mean of differentiating biliary atresia from neonatal hepatitis. $^{99m}Tc$-DISIDA scintigraphy was visually interpreted by assessing the presence or absence of radioactivity in the intestine or gall bladder. In patients without intestinal radioactivity, we measured the hepatic retention index and the hepatic uptake index. The hepatic retention Index was expressed as the amount of change of liver activity from 5 minutes to 30 minutes postinjection. The hepatic uptake Index was graded visually with 5 minute images using the following scoring scheme : grade 0(normal hepatic uptake), grade 1(decreased hepatic uptake), grade 2(hepatic uptake equal to cardiac uptake), and grade 3(hepatic uptake less than cardiac uptake). Age, total bilirubin, and hepatic uptake index were compared between the biliary atresia and the neonatal hepatitis group, between neonatal hepatitis patients with and without intestinal radioactivity, and between the biliary atresia and neonatal hepatitis patients with absent intestinal radioactivity. The results were as follows ; 1) None of the 30 biliary atresia patients showed intestinal radioactivity, while 31/40 neonatal hepatitis patients showed intestinal radioactivity. The sensitivity, specificity, and accuracy of the presence of intestinal radioactivity ?or the diagnosis of biliary atresia was 100%, 78%, and 87%, respectively. 2) In patients with absent intestinal radioactivity the mean hepatic retention index was $1.5{\pm}0.6$ in the 16 biliary atresia patients, and $1.1{\pm}0.2$ in the 7 neonatal hepatitis patients(p<0.01). All 7 patients with hepatic retention index over 1.5 had biliary atresia. But there were 9 patients with biliary atresia below 1.5. 3) No significant differences were found in age, total bilirubin, or hepatic uptake Index between biliary atresia and neonatal hepatitis patients. However there were differences in age, total bilirubin, and hepatic uptake index between neonatal hepatitis patients with and without intestinal radioactivity. The hepatic uptake index was significantly lower, age was old, and total bilirubin was low in the group with intestinal radioactivity compared the group without intestinal radioactivity(p<0.05). Relation between total bilirubin and the hepatic uptake index was that total bilirubin was relatively low at normal hepatic uptake index in biliary atresia and neonatal hepatitis patients. 4) When hepatic uptake index and hepatic retention index were high it suggest that biliary atresia is more likely, considered relation between hepatic uptake Index and the hepatic retention index. Thus, we conclude that $^{99m}Tc$-DISIDA scintigraphy is accurate in the differential diagnosis of biliary atresia and neonatal hepatitis. In patients without intestinal radioactivity, the hepatic retention index and hepatic uptake index, along with the patient's age and total bilirubin level may supplement diagnosis and improve diagnostic accuracy.

• The Korean Journal of Nuclear Medicine
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• v.29 no.4
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• pp.492-496
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• 1995

To evaluate the usefulness of hepatic uptake in neonatal jaundice, Tc-99m-DISIDA cholescintigraphy was reviewed for 13 infants with prolonged mired jaundice and no demonstrable excretion into bowel,even after 24hr. Five patients proved to have biliary atresia. The remainder had neonatal hepatitis. There was no distinct differentiation of the hepatic uptake of tracer at 5 and 10 minutes between biliary atresia and neonatal hepatitis. The consideration of hepatic uptake rate of the tracer is not useful in differentiating neonatal hepatitis from biliary atresia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.1
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• pp.46-58
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• 1999

Purpose: The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis. Method: We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed. Result: Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis. Conclusion: This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

• Neonatal Medicine
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• v.25 no.2
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• pp.72-77
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• 2018

Purpose: Glucose has been recommended as an analgesic for mild to moderately painful procedures in neonates. The goal of this study was to assess the optimal dextrose concentration for pain control in newborns. Methods: This prospective, randomized, blinded clinical trial included 116 healthy full-term newborns. The neonates were randomly assigned to the following four groups by drawing straws: groups receiving sterile water or a 10%, 20%, or 40% dextrose solution orally. Each group was treated with the assigned solution prior to hepatitis B vaccination. The Neonatal Facial Coding System (NFCS) and the Neonatal Infant Pain Scale (NIPS) scores were evaluated before, immediately after, and 2 minutes after the injection in all neonates. Premature Infant Pain Profile (PIPP) scores were evaluated during the injection. All procedures were video-recorded, and pain scores were assessed by two independent observers who were not involved in the care of the newborns studied. The pain scores were compared among the four groups. Results: The 40% dextrose solution significantly reduced the NFCS (P=0.002) and the PIPP scores (P=0.001) compared with sterile water. No hyperglycemic events were noted in the study subjects 2 hours after the injection. Conclusion: The 40% dextrose solution effectively relieved pain due to intramuscular injection in full-term newborns without causing hyperglycemic events. However, the 10% and 20% dextrose solutions did not affect neonatal pain scores.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.2
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• pp.122-129
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• 2011

The persistence of jaundice beyond the first 2 weeks of life require further investigation and this can be determined if the conjugated bilirubin levels are greater than 1.5 mg/dL or greater than 20% of the total bilirubin level. There is a diverse differential diagnosis for the cause of neonatal cholestasis due to hepatobiliary disease including biliary atresia, which eventually leads to liver cirrhosis if uncorrected before 60~80 days of life. Long-established initial studies include abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy, but better diagnostic methods are needed. Promising new options are described including MRCP (magnetic resonance cholangiography), ERCP (endoscopic retrograde cholangiography), and PCC (percutaneous cholecysto-cholangiography). Though no single test can differentiate biliary atresia from other neonatal cholestasis with confidence, a combination of diagnostic methods is usually consistently beneficial. By excluding biliary atresia as early as possible, the risk of unnecessary explolaparotomy with intraoperative cholangiography is decreased. Further evaluation would be required for the diagnosis of neonatal cholestasis after excluding biliary atresia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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• pp.1-11
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• 2016

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology. Recent literature shows that optimal nutritional and medical support also plays an integral role in the management of pediatric patients with chronic cholestasis. This review will provide a broad overview of the pathophysiology, diagnostic approach, and management of cholestasis beyond the neonatal and infancy periods.