Diagnostic utility of hepatobiliary scintigraphy with 99mTc-DISIDA in neonatal cholestasis

We retrospectively evaluated the utility of hepatobiliary scintigraphy and various clinical factors in differentiating intrahepatic cholestasis from biliary atresia in 28 consecutive infants with neonatal cholestasis. One millicurie of technetium-labeled diisopropyliminodiacetic acid (DISIDA) was administered intravenously, and images were obtained for up to 24 hours or until gastrointestinal excretion was noted. Nine separate studies in seven infants with biliary atresia were correctly interpreted as showing no gastrointestinal excretion of radionuclide. Of the 21 patients with intrahepatic cholestasis, only nine had gastrointestinal excretion on the first study; in eight without excretion, a second study was done, and five of these showed gut excretion. All infants with either neonatal hepatitis (six) or inspissated bile syndrome (three) had demonstrable gastrointestinal excretion either on the first or second DISIDA study. However, five of six infants with paucity of intrahepatic bile ducts, two of six infants with cholestasis secondary to total parenteral nutrition, and one infant with cholangiolitis did not show evidence of gastrointestinal excretion. The mean birth weight, mean gestational age, and mean weight at study were significantly greater (P less than 0.005) for infants with biliary atresia without excretion than for infants with intrahepatic cholestasis without excretion. The mean direct bilirubin concentration was 6.0 mg/dL for both infants with biliary atresia and infants with intrahepatic cholestasis without excretion; however, infants with excretion had a significantly lower (P less than 0.02) mean direct bilirubin value of 3.4 mg/dL. Excretion was noted in four infants with total bilirubin values greater than 10.0 mg/dL. The absence of gut excretion on the first DISIDA study was 100% sensitive but only 43% specific for biliary atresia. In infants without gut excretion of DISIDA, birth weight greater than 2200 g was 100% sensitive and 92% specific for biliary atresia. We conclude that DISIDA scanning, together with clinical data, is useful in differentiating extrahepatic from intrahepatic cholestasis. The absence of gut excretion on the first DISIDA study does not necessarily indicate extrahepatic obstruction; the study should be repeated if the diagnosis is not clear.     

Unconjugated, Glycine-conjugated, Taurine-conjugated Bile Acid Nonsulfates and Sulfates in Urine of Young Infants with Cholestasis

ABSTRACT. A direct assay system for conjugated bile acids using an enzymatic procedure and high-performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra-hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3β-hydroxy-5-cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63±2.83 vs. 2.50±1.25, p <0.05, 15.8±9.9 vs. 34.5±9.9%, p < 0.005). The proportion of taurine-conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7±7.5 vs. 22.7±7.8 %, p < 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine-conjugated chenodeoxycholate sulfate excretion.     

Clinical aspects on neonatal cholestasis based on observations at a Swedish tertiary referral centre

The aim of the study was to investigate the clinical aspects of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. A majority of the patients were referred from other parts of the country. The most common diagnoses were extrahepatic biliary atresia (n = 30 patients), alpha₁-antitrypsin deficiency (n = 11) and progressive familial intrahepatic cholestasis (n = 11). On presentation, the biliary atresia group had higher mean serum values of bilirubin, G-GT and cholesterol than the patients with intrahepatic cholestasis, with no significant differences noticed for any other biochemical parameter. A lack of excretion on hepatobiliary scintigraphy was noticed in all investigated patients with biliary atresia, but also in 9 of 34 patients with intrahepatic neonatal cholestasis. There was no statistical correlation between the age at portoenterostomy and the outcome in patients with biliary atresia. However, both the detection of a partial flow on perioperative cholangiogram and the establishment of a non-icteric phase within 6 mo after the portoenterostomy correlated to a good outcome. Eight of 11 patients with progressive familial intrahepatic cholestasis were treated with a biliary diversion procedure, five of eight experienced a sustained cholestatic remission. Conclusions: Progressive familial intrahepatic cholestasis may be a more common cause of neonatal cholestasis in Sweden than reported elsewhere and that the experience with biliary diversion is positive. While early referral in patients with extrahepatic biliary atresia remains important, a portoenterostomy should be attempted also in patients referred after 3 mo of age.     

Unconjugated, glycine-conjugated, taurine-conjugated bile acid nonsulfates and sulfates in urine of young infants with cholestasis

A direct assay system for conjugated bile acids using an enzymatic procedure and high-performance liquid chromatography was used for the analysis of urinary bile acid profiles in young infants with intrahepatic cholestasis (idiopathic neonatal hepatitis syndrome) or extra-hepatic biliary atresia. The major urinary bile acids were cholate and chenodeoxycholate conjugates, but a small amount of deoxycholate and 3 beta-hydroxy-5-cholenate conjugates were detected. Although there was no significant difference in total bile acid excretion between patients with intrahepatic cholestasis and extrahepatic biliary atresia, mean ratios of cholate to chenodeoxycholate and sulfated to total urinary bile acids were different between the two groups examined (5.63 +/- 2.83 vs. 2.50 +/- 1.25, p less than 0.05, 15.8 +/- 9.9 vs. 34.5 +/- 9.9%, p less than 0.005). The proportion of taurine-conjugated chenodeoxycholate in the sulfate fraction to the total bile acid was lower in intrahepatic cholestasis, compared with that in biliary atresia (7.7 +/- 7.5 vs 22.7% +/- 7.8%, p less than 0.005). The greater ratio of cholate to chenodeoxycholate and the reduced excretion of sulfated urinary bile acids in intrahepatic cholestasis was due to decreased taurine-conjugated chenodeoxycholate sulfate excretion.     

Bile acids in serum and bile of infants with cholestatic syndromes

The concentration of individual bile acids in serum was measured in 18 neonates and infants with various cholestatic conditions (extrahepatic biliary atresia, neonatal hepatitis syndrome, chronic intrahepatic cholestasis and posthemolytic cholestasis). The cholate/chenodeoxycholate ratio in serum was smaller than one in all patients with neonatal hepatitis syndrome or extrahepatic biliary atresia, cholestatic conditions which were accompanied by signs of liver cell injury. It was greater than one in the patients with chronic intrahepatic cholestasis. Administration of cholestyramine to patients with patent extrahepatic bile ducts decreased the total concentration bile acids in serum and elevated the cholate/chenodeoxycholate ratio. Thus, cholestyramine administration may be of diagnostic value for evaluation of bile duct patency in cholestasis of infancy. Differences between the bile acid pattern in serum and bile were observed. Thus, the cholate/chenodeoxycholate ratio was always higher in bile than in serum. 3beta-hydroxy-5-cholenoic acid found in serum was not detectable in bile. This finding suggests that impairment of biliary excretion rather than increased hepatic synthesis is responsible for elevation of this monohydroxy bile acid in serum.     

Ganciclovir treatment in infants with cytomegalovirus infection and cholestasis

BACKGROUND: The authors have previously described an association between cytomegalovirus (CMV) infection and intrahepatic and extrahepatic forms of neonatal cholestasis. Pediatric use of the antiviral drug ganciclovir to treat patients with CMV infection has increased. In this study, infants with CMV infection and cholestasis were treated with ganciclovir. METHODS: Six infants with cholestasis (age, 3-16 weeks) and with signs of ongoing CMV infection were treated with intravenous ganciclovir for 3 to 7 weeks and observed for 4 to 31 months after treatment. Two patients had biliary atresia, one had suspected septo-optic dysplasia and three had no obvious cause for intrahepatic cholestasis other than ongoing CMV infection. RESULTS: Four patients, including one with biliary atresia, responded to the treatment, whereas two patients, including the one with septo-optic dysplasia did not. The latter patient had episodes of symptomatic hypoglycemia during the treatment, which was subsequently stopped. Liver function at the end of follow-up was good in four patients, intermediate in one, and poor in one. CONCLUSION: Ganciclovir treatment may be beneficial in infants with CMV-associated intrahepatic cholestasis, but controlled studies are needed. Because of the possible side effect of hypoglycemia, infants with cholestasis who have increased risk for such episodes should not be treated.     

血清γ-谷氨酰转肽酶联合直接胆红素诊断婴儿胆道闭锁的价值

目的 探讨血清γ-谷氨酰转肽酶（GGT）联合直接胆红素（DB）对胆道闭锁的诊断价值。方法 选取2010年7月至2018年12月住院治疗的胆汁淤积症患儿667例为研究对象,根据术中胆管造影结果和随访情况,将患儿分为胆道闭锁组（n=234）和胆汁淤积组（n=433）。比较两组的发病年龄、性别,以及血清总胆红素（TB）、DB、丙氨酸氨基转移酶（ALT）、天门冬氨酸氨基转移酶（AST）、总胆汁酸（TBA）、GGT水平。将有统计学意义的指标纳入受试者工作特征曲线（ROC）分析,计算ROC曲线下面积（AUC）和最佳诊断界值。结果 胆道闭锁组患儿发病年龄早于胆汁淤积组（P < 0.001）。两组患儿性别、ALT及AST水平比较差异无统计学意义（P > 0.05）。胆道闭锁组TB、DB、TBA、GGT水平显著高于胆汁淤积组（P < 0.05）。GGT联合DB诊断胆道闭锁的AUC最大,为0.892（95% CI：0.868~0.916）。当GGT取值为324.0 U/L,DB取值为115.1 μmol/L时,GGT与DB联合诊断胆道闭锁的敏感度和特异度分别为79.8%、83.2%。结论 GGT联合DB诊断胆道闭锁的敏感度和特异度较高,可作为诊断胆道闭锁的有效指标之一。     

Role of reovirus type 3 in persistent infantile cholestasis

The relationship between reovirus type 3 and persistent infantile cholestasis was studied by measuring antibody to the virus in the sera of affected and control babies younger than 1 year of age. One hundred sixty-seven infants were divided into four groups: those with extrahepatic biliary atresia, idiopathic neonatal hepatitis, or other cholestatic disorders, and controls. When available, maternal sera obtained simultaneously with infant sera were also studied. The results indicate that 62% of babies with extrahepatic biliary atresia and 52% of infants with idiopathic neonatal hepatitis have reovirus 3 antibodies. In contrast, less than 12% of either normal infants or babies with other cholestatic disorders have antibodies. These observations suggest that perinatal infection with reovirus type 3 may serve as an initiating event in the genesis of two closely related forms of infantile obstructive cholangiopathy: extrahepatic biliary atresia and idiopathic neonatal hepatitis.     

Cholestatic jaundice in infancy. The importance of familial and genetic factors in aetiology and prognosis.

One hundred and twenty-four infants admitted to hospitals in Norway between 1955 and 1974 during the first 3 months of life with cholestatic jaundice were studied retrospectively. Sixty-four infants had had extrahepatic atresia of the biliary tree and 60 had had intrahepatic cholestasis. This gives an incidence of about 1:9000 live births for cholestasis. In 4 of the 64 infants with extra-hepatic atresia a bile duct-to-bowel anastomosis had been performed but this was successful in only 2. Sixty of these infants had died by their 2nd birthday. Twenty-six of the infants with intrahepatic cholestasis had died by 1978 and the most common causes of death were cholestasis complicated by infection, bleeding, or hepatoma. The survivors aged between 4 and 23 years were followed up in 1978. In about two-thirds of them aetiological factors--such as alpha-1-antitrypsin deficiency, arteriohepatic dysplasia, cholestasis with lymphoedema--and other familial or genetic factors, or infections were found. Four of the 34 survivors are known to have cirrhosis. Twenty patients had biochemical abnormalities, and 12 had normal liver function tests. Two patients could not be examined. Of the 19 patients with familial or genetic aetiological factors, 4 had cirrhosis, 14 had biochemical abnormalities, and only 5 had normal liver function tests. Of 11 survivors with idiopathic disease or septicaemia, none had cirrhosis and only 4 had abnormal liver function tests.     

Cholestatic jaundice in infancy. The importance of familial and genetic factors in aetiology and prognosis

One hundred and twenty-four infants admitted to hospitals in Norway between 1955 and 1974 during the first 3 months of life with cholestatic jaundice were studied retrospectively. Sixty-four infants had had extrahepatic atresia of the biliary tree and 60 had had intrahepatic cholestasis. This gives an incidence of about 1:9000 live births for cholestasis. In 4 of the 64 infants with extra-hepatic atresia a bile duct-to-bowel anastomosis had been performed but this was successful in only 2. Sixty of these infants had died by their 2nd birthday. Twenty-six of the infants with intrahepatic cholestasis had died by 1978 and the most common causes of death were cholestasis complicated by infection, bleeding, or hepatoma. The survivors aged between 4 and 23 years were followed up in 1978. In about two-thirds of them aetiological factors--such as alpha-1-antitrypsin deficiency, arteriohepatic dysplasia, cholestasis with lymphoedema--and other familial or genetic factors, or infections were found. Four of the 34 survivors are known to have cirrhosis. Twenty patients had biochemical abnormalities, and 12 had normal liver function tests. Two patients could not be examined. Of the 19 patients with familial or genetic aetiological factors, 4 had cirrhosis, 14 had biochemical abnormalities, and only 5 had normal liver function tests. Of 11 survivors with idiopathic disease or septicaemia, none had cirrhosis and only 4 had abnormal liver function tests.     

Neonatal hepatic cholestasis with particular regard for the use of radioisotopes in its diagnosis]

The aim of this study was to assess the diagnostic sensitivity and specificity of hepatobiliary scintigraphy using a 99mTc-HIDA compound to differentiate intrahepatic cholestasis from extrahepatic forms during the first months of life. The tracer used was acid N-(2,6)-diethylacetanylido-iminodiacetic (diethyl-HIDA) with almost exclusively biliary excretion and a high concentration of radioactivity in the bile. Each neonate was injected with 0.5 mg/kg i.v. of the compound marked with a dose of 99mTc equivalent to 80-100 microCi/kg. Scintigraphic recordings were carried out every 10' for the first hour and further controls were performed at 2, 3, 4, 8 and 24 hours. Scintiphotos were obtained using a Polaroid scintillation camera, PHO Gamma V. Fifty-four patients were included in the study (34 males and 20 females) aged between 4 days and 3 months old. All patients were clinically suspected of pathologies involving the hepatobiliary tract. All cases were affected by persistent jaundice (total bilirubin between 1.8 and 39.6 mg%) with predominantly direct bilirubin (range 1.5-26.2 mg%), acholic feces and hyperchromic urine. Hepato-biliary scintigraphy revealed an intestinal excretion of tracer in 31 out of the 54 neonates examined, excluding the presence of an extrahepatic obstruction of the biliary tract. On the other hand, only 13 out of 23 cases in which no enteric excretion of the tracer was observed, was the final diagnosis one extrahepatic cholestasis. Scintigraphic tests therefore showed a 100% sensitivity associated with a specificity of only 56.52%. This demonstrates that the finding of tracer in the intestine is pathognomonic of the permeability of extrahepatic biliary ducts and that biliary atresia can be ruled out. On the contrary, the absence of the intestinal excretion of the tracer is nor constantly associated with biliary atresia. This study has confirmed this finding in 10 cases of intrahepatic cholestasis (4 hypoplasias of the intrahepatic biliary tract, 3 thick bile syndromes, 3 cases of hepatitis due to cytomegalovirus). In conclusion, the Authors state that hepato-biliary scintigraphy represents a straightforward and non-invasive diagnostic method which enables the permeability of the biliary tract to be assessed in subjects with jaundice.     

Analysis of delay in diagnosis of extrahepatic biliary atresia]

Prognosis of extrahepatic biliary atresia depends on an early surgical treatment. This survey studied the delay to surgery of infants treated for biliary atresia and analysed the causes of late diagnosis and referral. MATERIAL AND METHODS: Medical files of 21 infants treated for biliary atresia between 1988 and 1998 were retrospectively analysed. RESULTS: Median age at biliary operation was 57 days and did not change during time. In only 3/21 cases, surgery was performed before 45 days of age. The first clinical or biological sign of cholestasis was noted at a median age of 12 days. In eight cases, the first medical visit for cholestasis (median age of 21 days) was not followed by a blood test. In 11 cases, infants presenting with biological cholestasis were referred to hospital more than seven days later. False diagnosis was noted in seven cases and delayed significantly the operation. CONCLUSION: The delay to surgical treatment is too long and does not decrease whereas a majority of infants visit their physician early. Medical information is mandatory for all physicians taking care of infants.     

Coincidence of congenital toxoplasmosis and biliary atresia in an infant.

A 4-week-old infant presenting with neonatal cholestasis was found to have congenital toxoplasmosis and biliary atresia. This is the first patient in which their coincidence is reported. Because biliary atresia can coexist with either congenital infection or inborn errors of metabolism, evaluation for an obstructive etiology of jaundice in infants with a recognized cause of intrahepatic cholestasis is necessary.     

Computerized three-dimensional study of a rotavirus model of biliary atresia: comparison with human biliary atresia

Biliary atresia is a panbiliary disease causing obstructive jaundice in neonates and infants. The clinical spectrum can be broadly categorized into the fetal and perinatal types. A consistent animal model that accurately mimics the whole clinical spectrum of biliary atresia is not yet available. However, rotavirus infection of neonatal mice has been shown to produce atresia in the biliary system. This study investigates the three-dimensional computerized morphology of the murine neonatal model comparing with age-matched control mice. Newborn Balb/c mice were injected intraperitoneally with rhesus rotavirus within 24–48 h after birth. Control mice received 0.9% NaCl. Pups with symptoms of cholestasis were sacrificed from the 5th to the 15th postinjection day, as were age-matched controls. Their hepatobiliary tissues were prepared for three-dimensional computerized image reconstruction. Rotavirus infection caused obliteration of the intrahepatic bile ducts and single to multiple atresias in the extrahepatic bile duct. At 15 days postinjection, intrahepatic ductal proliferation appeared, and the three-dimensional appearances of the intrahepatic biliary structures were similar to the human disease. Cystic duct and gallbladder dilatation was frequently seen in this model, and this feature distinguishes it from the human disease in which the gallbladder is almost always atretic. This rotavirus murine model demonstrates many of the features of human perinatal biliary atresia, and can be used as an investigative tool to further study the pathogenesis of biliary atresia.     

Use of rifampin for severe pruritus in children with chronic cholestasis.

BACKGROUND: Rifampin has been proposed to reduce pruritus in children and adults with chronic cholestasis; however, there is a paucity of published data regarding the use of rifampin in children. METHODS: In an open trial, 24 children were evaluated during a 6-year period. Diagnoses included 13 patients with extrahepatic biliary atresia (54%), six with Alagille's syndrome, three with Byler's disease, and one each with primary sclerosing cholangitis and alpha1-antitrypsin deficiency. All patients had severe pruritus that had not responded adequately to at least 2 months of therapy with ursodeoxycholic acid, diphenhydramine, or phenobarbital and local skin care measures. Treatment was initiated with rifampin, 10 mg/kg per day in two divided doses for 18+/-20 months, and the effect on the severity of pruritus was assessed by a clinical scoring system. RESULTS: Ten patients showed a complete response, 12 a partial response, and 2 no response. Complete response was more common in extrahepatic cholestasis (64% vs. 10%), whereas partial response was more common in intrahepatic cholestasis (80% vs. 29%). Treatment was associated with reduction of gamma-glutamyl transpeptidase. No clinical or biochemical toxicity of rifampin was observed. CONCLUSIONS: We conclude that for more than 90% of children with chronic cholestasis and severe pruritus unresponsive to other treatments, rifampin appears to be a safe and effective therapy.     

Frequency and clinical progression of the vitamin E deficiency neurologic disorder in children with prolonged neonatal cholestasis

To determine the frequency of biochemical vitamin E deficiency and of the clinical signs of the vitamin E deficiency neurologic syndrome in children with prolonged neonatal cholestatic disorders, we studied 46 children (aged 1 month to 17.0 years) with chronic forms of intrahepatic neonatal cholestasis and 47 children (aged 4 months to 8.0 years) with extrahepatic biliary atresia. Based on serum vitamin E concentrations and the ratios of serum vitamin E concentration to total serum lipid concentration, 64% of the intrahepatic and 77% of the extrahepatic cholestasis groups were vitamin E deficient. Prior to age 1 year, neurologic function was normal in all children. Between ages 1 and 3 years, neurologic abnormalities were present in approximately 50% of the vitamin E-deficient children; after age 3 years, neurologic abnormalities were present in all vitamin E-deficient children. Areflexia was the first abnormality to develop between ages 1 and 4 years; truncal and limb ataxia, peripheral neuropathy, and ophthalmoplegia developed between ages 3 and 6 years. Neurologic dysfunction progressed to a disabling combination of findings by ages 8 to 10 years in the majority of vitamin E-deficient children. Neurologic function was normal in the vitamin E-sufficient children. We conclude that vitamin E status should be evaluated in infants in whom cholestasis is diagnosed, and effective therapy should be initiated to prevent or treat vitamin E deficiency at an early age.     

Hepatobiliary scintigraphy with technetium-99m disofenin in the evaluation of neonatal cholestasis

To assess the reliability of technetium-99m disofenin scanning in evaluating neonatal cholestasis, 33 neonates (less than 3 months of age) with direct hyperbilirubinemia were evaluated prospectively by cholescintigraphy. Results of this test were compared to those of standard serum tests of liver function, ultrasonography, and liver biopsy. The diagnosis of biliary atresia was suggested by a serum gamma-glutamyl transpeptidase (gamma-GTP) greater than 300 units/L, absence of the gallbladder on ultrasonography, and a lack of detectable radioisotope in the gastrointestinal and/or extrahepatic biliary tract on cholescintigraphy. Each of these tests lacked sensitivity and/or specificity when compared to liver biopsy. Of the nine neonates with biliary atresia, three had gallbladders identified by ultrasonography and two had gamma-GTP less than 300 units/L. Of the 24 neonates without biliary atresia, eight had cholescintigraphy without detectable radioisotope excretion, four had ultrasonography that failed to visualize the gallbladder, and nine had gamma-GTP greater than 300 units/L. Cholescintigraphy excluded the diagnosis of biliary atresia when gut and/or extrahepatic biliary excretion of isotope was seen. However, cholescintigraphy required more time, 6-8 days, and was less specific than ultrasonography and liver biopsy. We recommend that cholescintigraphy should not be routinely used in evaluating neonatal cholestasis, especially if it delays surgical intervention.     

Bone disease in infants and children with hepatobiliary disease

Radiological studies of bone were performed in infants and children with hepatobiliary disease. Rickets was found in 23 out of 39 patients (59%) with surgically unrepaired biliary atresia, in 4 out of 15 (27%) with surgically repaired biliary atresia, in 11 out of 21 (52%) with neonatal hepatitis, and in 2 out of 4 (50%) with intrahepatic cholestasis. Osteoporosis was found in 23 out of 39 (59%) with unrepaired biliary atresia, in 3 out of 15 (20%) with repaired biliary atresia, in 5 out of 21 (24%) with neonatal hepatitis, and in 1 out of 4 (25%) with intrahepatic cholestasis. 2 girls with Byler disease and 1 infant with choledochal cyst showed no radiological evidence of bone disease.In unrepaired biliary atresia comparative studies of biochemical data in the groups with and without bone disease showed the following. Serum calcium levels were reduced in the patients with bone disease compared with those in the group without it. Serum magnesium levels were markedly reduced in the groups with and without bone disease. The product of serum calcium and phosphorus was reduced in the group with osteoporosis compared with that in the group without it. The raised levels of serum alkaline phosphatase were unrelated to the presence or absence of bone disease.     

Hepatic portoenterostomy for biliary atresia. A comparative study of histology and prognosis after surgery

Specimens of excised tissue from the porta hepatis in 26 infants with extrahepatic biliary atresia undergoing hepatic portoenterostomy were analysed histologically for the presence and size of biliary ductules. No correlation could be found between the establishment of effective biliary drainage and the number or size of biliary ductules. it is suggested that prognosis after surgery may be related to the intrahepatic lesion and age of the child at operation rather than to the histology of the extrahepatic bile duct remnants.     

Aetiological factors in neonatal cholestasis

The aim of the study was to investigate factors of possible importance for the aetiology of neonatal cholestasis. The medical records of 85 cholestatic infants were retrospectively reviewed. The most common diagnoses were extrahepatic biliary atresia (n