Bile Duct Involvement Research Articles

Biliary complications of pancreatic necrosis.

Involvement of the biliary tract in pancreatic necrosis is rare. The authors are presenting six patients with this unusual complication. Retrospective analysis of a case series. The necrotic process involved the bile duct in four patients (bile duct alone in two and bile duct with duodenum in two) and the gall bladder in two patients. It was not possible to make a preoperative diagnosis of biliary tract involvement in any of these patients. The lesions in the biliary tract were caused by the direct erosion by the necrotic tissue in five patients, and in one patient with gangrene of the gall bladder, it was a "remote" complication of the necrotizing process. All patients underwent surgery. Necrosectomy and cholecystectomy were performed in patients with gall bladder lesions, and proximal biliary drainage was the method of choice in patients with erosion of the bile duct. One patient died postoperatively. During follow-up, another patient who had bile-duct involvement developed a stricture in the damaged part of the bile duct and needed hepaticojejunostomy Necrotizing pancreatitis can involve the biliary tract, both by direct extension and by its secondary effects. Although cholecystectomy is the treatment of choice in the presence of gallbladder involvement, proximal biliary diversion may be indicated in patients with erosion of the bile duct.

Hepatocellular Carcinoma with Bile Duct Involvement: Computed Tomographic (CT) Findings

Purpose : To describe the radiologic features of computed tomography(CT) in hepatocelluar carcinoma(HCC) with bile duct involvement. Materials and Methods : We retrospectively analyzed the two phase spiral CT findings of 31 patients in whom HCC with bile duct invasion (n=28) or compression (n=3), was diagnosed. Eight of these underwent follow-up CT after transarterial chemoembolization. We analyzed the size, type, location, enhancement pattern, and lipiodol retention of parenchymal and intraductal masses, as well as their and lymphadenopathy. Results : In all patients with bile duct invasion, single or multiple masses were demonstrated in the bile ducts. Intraductal masses showed the same enhancement characteristics as the parenchymal mass (kappa 0.550, p

Cholangiopathy and intrahepatic stones in sickle cell disease: coincidence or ischemic cholangiopathy?

Hepatic and bile duct involvement are common in sickle cell disease. This article reports the case of a young woman with cholangiopathy, homozygous sickle cell disease, and protracted anemia. We suggest that sickle cell disease was directly responsible for ischemic cholangiopathy through anoxia.

Recognition of intrabiliary hepatic metastases from colorectal adenocarcinoma.

Intrinsic involvement of bile ducts, by metastatic colorectal adenocarcinoma growing from within or invading the lumen of bile ducts, is not a well recognized pattern of tumor growth. Clinical, radiographic, operative, and histopathologic aspects of 15 patients with intrabiliary colorectal metastases were described. Fourteen patients were explored for possible hepatic resection. Two had jaundice, two radiographic evidence of an intrabiliary filling defect, 10 intraoperative evidence of intrabiliary tumor, and six microscopic evidence of intrabiliariy tumor. Eleven patients underwent hepatic resection. Five of the resected patients developed hepatic recurrence. Four patients were explored for possible repeat resection. One had jaundice, one radiographic evidence of an intrabiliary filling defect, all had intraoperative evidence of intrabiliary tumor, and three microscopic evidence of intrabiliary tumor. Three patients underwent repeat hepatic resection. All patients with preoperative jaundice and radiographic evidence of an intrabiliary filling defect were unresectable. Overall, actuarial five-year survival is 33% for those patients resected versus 0% for those not resected. Intraoperative recognition of intrabiliary tumor at exploration for hepatic resection was more common than clinical, radiographic, or histopathologic recognition. More diligent examination of resected liver tissue by the surgeon and pathologist may increase identification of bile duct involvement and aid in achieving adequate tumor clearance.

Chronic pancreatitis and inflammatory bowel disease: true or coincidental association?

OBJECTIVE: Several cases of pancreatitis have been described during the course of Crohn’s disease (CD) or ulcerative colitis (UC), but many of them were related to either biliary lithiasis or drug intake. We tried to evaluate the clinical and morphological features of so-called idiopathic pancreatitis associated with inflammatory bowel disease and to define their pathological characteristics. METHODS: Chronic idiopathic pancreatitis was diagnosed on the basis of abnormal pancreatograms suggestive of chronic pancreatitis associated with or without impaired exocrine pancreatic function, or pathological examination in patients undergoing pancreatic resection. We found 6 patients presenting with features of chronic idiopathic pancreatitis and UC and 2 patients with CD seen between 1981 and 1996 in three hospital centers of the south of France. A review of the literature has identified 6 cases of pancreatitis associated with UC and 14 cases of pancreatitis associated with CD based on the above criteria. RESULTS: Hyperamylasemia was not a sensitive test since it was present in 44% and 64% of patients with UC or CD. In UC, pancreatitis was a prior manifestation in 58% of patients. In contrast, the pancreatitis appeared after the onset of CD in 56% of the cases. In patients with UC, pancreatitis were associated with severe disease revealed by pancolitis (42%) and subsequent surgery. Bile duct involvement was more frequent in patients with UC than with CD (58% vs 12%) mostly in the absence of sclerosing cholangitis (16% vs 6%). Weight loss and pancreatic duct stenosis were also more frequent in UC than in CD (41% vs 12% and 50% vs 23%, respectively). Pathological specimens were analyzed in 5 patients and demonstrated the presence of inter- and intralobular fibrosis with marked acinar regression in 3 and the presence of granulomas in 2 patients, both with CD. CONCLUSIONS: Pancreatitis is a rare extraintestinal manifestation of inflammatory bowel disease. Chronic pancreatitis associated with UC differs from that observed in CD by the presence of more frequent bile duct involvement, weight loss, and pancreatic duct stenosis, possibly giving a pseudo-tumor pattern.

Bile duct cysts in adults: a multi-institutional retrospective study. French Associations for Surgical Research.

To review the features of adult patients undergoing surgery for bile duct cysts, focusing on the anatomy of the biliary tree as well as the long-term outcome. Bile duct cysts (BDCs) are uncommon in Western countries, and the majority of reported cases originate from Asia. Japanese authors have emphasized the frequent association of extra- and intrahepatic bile duct dilatations, but grading of patients based on Todani's classification is often hindered by the absence of an accurate definition of types IC and IVA cysts. Moreover, despite the increasing use of extrahepatic cyst excision, little is known about the long-term outcome in patients with intrahepatic bile duct involvement. Forty-two adult patients with BDC were treated between 1980 and 1992 in 17 institutions of the French Associations for Surgical Research. Clinical presentation, radiologic presurgical evaluation, and surgical procedures were analyzed. The long-term postsurgical outcome was derived from patient charts, attending physicians, or direct patient contact. Twelve patients (30%) had recurrent abdominal pain or jaundice from childhood. Seven (17%) had undergone prior cystenterostomy. Twenty-one (50%) had a Todani-type IVA cyst with extra- and intrahepatic bile duct involvement. Of these, nine had segmental, exclusively left-sided intrahepatic bile duct dilatation. Biliary carcinoma was encountered in five patients (12%). Extrahepatic cyst excision with a Roux-en-Y hepaticojejunostomy was performed in 34 patients with type I or IV cysts. The overall operative mortality rate was 2.4%. Long-term results were clearly correlated with cyst type: during a mean follow-up of 8.4 years, 11 of 12 patients (92%) treated by cyst excision for type I cyst remained free of symptoms, whereas 31% of patients who underwent surgery for type IV cyst had episodic or severe cholangitis with intrahepatic stones. In patients with BDC, particular attention must be given to the associated intrahepatic bile duct dilatations. We propose a modification of Todani's classification to distinguish cystic, segmental, and fusiform dilatations of the intrahepatic biliary tree in type IV cysts. In patients with segmental left intrahepatic cystic dilatations, combined left liver lobectomy and extrahepatic cyst excision is suggested to decrease late postsurgical biliary complications.

Gallbladder cancer: can ultrasonography evaluate extent of disease?

This study reviews the spectrum of sonographic findings in patients with gallbladder cancer, attempts to determine if sonography can identify patients with potentially resectable disease, and emphasizes the limitations of ultrasonography in the evaluation of -gallbladder cancer. Thirty-five consecutive patients with histologically proven gallbladder carcinoma who had preoperative abdominal ultrasonography and surgery were identified. Involvement of the gallbladder and gallbladder fossa, metastases, bile ducts, portal vein, and adjacent lymph nodes was assessed sonographically. The extent of disease and staging as revealed by sonography was compared to operative and surgical pathologic findings. Masses in the gallbladder or gallbladder fossa were present at surgery in 26 patients; 22 (85%) of these masses were shown by sonography. Sonography identified six (67%) of nine cases of pathologically confirmed liver metastases, 11 (79%) of 14 cases of bile duct involvement, and two (67%) of three cases of portal venous involvement by tumor. Sonography revealed lymph node metastases in only five (36%) of 14 patients. None of the 12 cases with peritoneal metastases was identified sonographically. By surgical staging 16 (46%) patients had potentially resectable disease (stage III or less), and 19 (54%) patients had unresectable stage IV disease. Sonography correctly identified 15 (94%) of 16 patients with potentially resectable disease and seven (37%) of 19 patients with advanced disease. Twelve patients with advanced disease were under-staged: nine had peritoneal metastases, two had liver metastases, and one had celiac adenopathy, which was not shown by sonography. In conclusion, sonography is reliable in the detection of a primary gallbladder mass or of local extension of tumor into the liver. However, sonographic findings do not accurately reflect the full extent of disease, and sonography is particularly limited in the diagnoses of metastases to the peritoneum and lymph nodes.

The intrahepatic cholangiopathies.

The intrahepatic biliary epithelial cells or cholangiocytes are the primary focus of injury in many congenital and acquired liver diseases of childhood. Although cholangiocytes account for only 3 to 5% of the liver cell population, injury and progressive loss of intrahepatic bile ducts can result in considerable morbidity and mortality. Table 1 provides an overview of the various disorders that affect the intrahepatic biliary tree. The more common disorders are discussed in detail below. One of the most important cholangiopathies, biliary atresia, characterized by complete destruction of the extrahepatic biliary tree, with variable involvement of the intrahepatic bile ducts, is discussed elsewhere in this series of articles. There has been considerable progress in our understanding of the embryology and physiology of the intrahepatic biliary system. These topics are also selectively reviewed, with an emphasis on advances that aid in the understanding of the pathophysiology of the disorders which affect the biliary tract in children.

External radiation therapy and transcatheter iridium in the treatment of extrahepatic bile duct carcinoma

Review survival, prognostic factors, and patterns of failure in patients with extrahepatic bile duct (EHBD) carcinoma treated with external beam irradiation (EBRT) and transcatheter iridium. The charts of 24 patients with EHBD cancer treated with EBRT and transcatheter boost were reviewed. All patients had transhepatic biliary tubes or endoprostheses placed. Two patients underwent hemihepatectomy with hepaticojejunostomy formation but had residual disease. Two patients had biopsy proven adenopathy. Five patients had Grade 1 adenocarcinoma, nine Grade 2, six Grade 3, and one Grade 4 disease. Median EBRT dose was 50.4 Gy delivered in 1.8 Gy/day fractions. Median transcatheter boost at 1 cm radius was 20 Gy. Nine patients received concomitant 5-Fluorouracil (5-FU) during EBRT. Median survival was 12.8 months (range 7.5 months to 9 years). Overall 2- and 5-year survival rates were 18.8 and 14.1%, respectively (three disease-free survivors > or =5 years). One patient is still alive without relapse 10 years from diagnosis and 5 years after liver transplantation for liver failure (no cancer in specimen, underlying sclerosing cholangitis). Two additional long-term survivors had no evidence of relapse 6.9 and 8.2 years after diagnosis. Histologic grade, lymph node status, cystic, hepatic, common hepatic or common bile duct involvement, surgical resection, radiation therapy dose, and chemotherapy did not significantly effect survival due to the number of patients analyzed. There was a trend towards improved survival with the addition of 5-FU chemotherapy (5-year survival in two of nine patients, or 22%). Eight of 24 patients (33%) demonstrated radiographic evidence of local recurrence. Distant metastases developed in 6 of 24 (25%) patients. The most common complications were tube related cholangitis (50%) and gastric/duodenal ulceration or bleeding (42%). External beam irradiation combined with a transcatheter boost can result in long-term survival of patients with EHBD cancer. Both distant metastases and local recurrence develop in 25-30% of patients despite irradiation. Survival may be improved by using chemotherapy in combination with EBRT to impact disease relapse (local and distant). Because there may be a dose response with irradiation, survival may also be improved by increasing the dose of radiation delivered by transcatheter boost. A Phase II trial is being developed using a combination of 45-50 Gy EBRT with concomitant 5-FU delivered by protracted venous infusion followed by a 25-30 Gy transcatheter boost.

Cholangiocarcinoma at the hepatic hilus: sonographic findings.

This study was performed to characterize sonographic findings in patients with cholangiocarcinoma at the hepatic hilus and to compare those sonographic findings with surgical and pathologic findings. Thirty-nine consecutive patients with hilar cholangiocarcinoma (Klatskin tumor) had preoperative color and spectral Doppler sonography and had surgical-pathologic correlation. Biliary drainage catheters were present in 24 patients (62%). In all patients, we evaluated presence of bile duct mass, level of bile duct involvement, patency of portal veins, and hepatic mass lesions. Ductal masses were revealed by sonography in 34 patients (87%). Masses were isoechoic in 22 patients (65%), hypoechoic in seven (21%), and hyperechoic in five (15%). The masses included nodular mural thickening in 19 patients (56%), infiltrative lesions in nine (26%), and intraductal polypoid masses in six (18%). The extent of bile duct involvement was revealed sonographically in 34 cases (87%) by the distribution of bile duct obstruction, the location of a ductal mass, or both. Portal vein involvement by tumor was shown sonographically in 20 patients (51%); 13 patients had occluded portal veins, and seven had encased portal veins without occlusion. Twenty-one portal veins in 16 patients were found to be involved at surgery; sonography showed 18 (86%) of 21 involved portal veins. Hepatic masses were present at surgery in six patients; four of these masses were malignant and two were benign. Sonography revealed five of the six masses and failed to reveal metastases in one patient who had pneumobilia from a biliary drainage catheter. Although Klatskin tumors are usually isoechoic, they can be revealed by sonography, and their morphology can be characterized. The extent of bile duct involvement may be shown on sonograms by the location of tumor and the distribution of bile duct obstruction. Portal vein involvement is frequent, and hepatic metastases are uncommon.

Is there a role for radical surgery in advanced gallbladder carcinoma?

Forty-four patients with advanced gallbladder carcinoma (18 with stage pT3 and 26 with stage pT4 of the Union Internacional Contra la Cancrum classification) were aggressively managed by extended heptatic resection in 33 patients, bile duct resection in 28, pancreaticoduodenectomy in seven, gastrointestinal resection in eleven and portal vein resection and reconstruction in seven. Adjacent organ involvement was classified as follows: type I, hepatic involvement with or without gastrointestinal invasion (Ia, Ib); type II, bile duct involvement with or without gastrointestinal invasion (IIa, IIb); type III, hepatic and bile duct involvement with or without gastrointestinal invasion (IIIa, IIIb); type IV, gastrointestinal involvement without hepatic or bile duct invasion. Fourteen of 15 patients with type I tumours had a curative resection compared with seven of 26 with type III lesions (P< 0.0001). The surgical mortality rate was two of 15 patients with type I tumours, seven of 26 with type III tumours and nine of 44 for the whole group. The long-term survival rate after curative resection was four and two of 23 at 3 and 5 years respectively, significantly better than two and none of 21 at I and 2 years after non-curative resection (P<0.01). The survival rate after curative resection for patients with type I tumours was four and two of 14 at 3 and 5 years respectively, significantly better than for other types (P<0.05). This classification of advanced gallbladder carcinoma according to involvement of adjacent organs might be helpful in planning surgery for this condition; in particular, type I tumours should be treated by a radical surgical procedure to achieve a favourable outcome.

Anomalous arrangement of pancreaticobiliary ductal system associated with cholangiocellular carcinoma

A 66-year-old man with congenital cystic dilatation of the common bile duct (CDB; Alonso-Lej Type I) and anomalous arrangement of the pancreaticobiliary ductal system (AAPB) associated with intrahepatic bile duct cancer (cholangiocellular carcinoma; CCC) underwent an extended right hepatic lobectomy. In the resected specimen, the CCC was located in Couinaud’s segment V, with invasion to segment IV and the right hepatic duct and right portal vein. However, there was no cancer involvement of the dilated extrahepatic bile ducts, except for histologic findings of chronic inflammatory cellular infiltration and intestinal metaplasia. It is presumed that the probable mechanisms underlying carcinogenesis in CCC developing from the epithelium of intrahepatic bile ducts are the same mechanisms as those operating in carcinoma of the extrahepatic bile duct in patients with AAPB, although AAPB associated with CCC is uncommon. AAPB appeared to be related to the development of the CCC.

Defining the criteria for local resection of ampullary neoplasms.

To delineate factors determined preoperatively, which predict successful local resection of ampullary neoplasms. Retrospective review of case series of the author's experience from 1988 through 1995. The median follow-up of patients with malignancies was 29 months. Tertiary care university teaching hospital. Twenty-seven patients underwent surgery. The decision to perform either an ampullectomy or pancreaticoduodenectomy (PD) was based on the size of the lesion, the presence of a "field defect" (ie, familial polyposis), depth of invasion determined by preoperative endoscopic ultrasound, and extent of pancreatic and bile duct involvement seen on endoscopic retrograde cholangiopancreatography. Fourteen patients underwent ampullectomy, 12 patients underwent PD, and one patient had a retroperitoneal node biopsy performed without resection of the primary tumor. Resectability, morbidity, and mortality. Depth of invasion was accurately determined in nine of 12 patients studied by preoperative endoscopic ultrasound. Preoperative endoscopic biopsy specimens were obtained in 21 patients and were inaccurate in seven of 21 cases. The length of stay following local resection was 10.5 +/- 3.7 days vs 15.4 +/- 5.8 days following PD (P=.02). One patient died following PD, and there were no deaths following ampullectomy. Six of 12 patients undergoing PD had postoperative complications vs two of 14 patients undergoing local resection. Ampullectomy is the procedure of choice for resecting benign lesions smaller than 3 cm, small neuroendocrine tumors, and T1 carcinomas of the ampulla. While endoscopic ultrasonography is helpful in identifying stage T1 lesions suitable for local resection, no preoperative test proved accurate enough to substitute for clinical judgment and intraoperative pathological confirmation.

Clinico-pathological findings of asymptomatic intrahepatic cholangiocellular carcinoma: report of two cases and review of the literature

The prognosis of intrahepatic bile duct carcinoma (cholangiocellular carcinoma: CCC) is extremely poor unless detected at an early and curable stage. However, little is known about the clinico-pathological features of CCC resected at a symptom-free stage. We herein report the clinico-pathological findings in two asymptomatic patients who underwent resection. Their liver function tests showed elevated levels of gamma-glutamyl transpeptidase. An intrahepatic mass was first detected by ultrasonography, and subsequent direct cholangiography and cytological examination revealed the presence of carcinoma in both patients. Left or right hepatic lobectomy with caudate lobectomy and lymph node dissection with or without resection of the extrahepatic bile duct was carried out. The lesions were 2.5 and 2.8 cm in maximal size, originating from the intrahepatic bile duct of segment IV and I, respectively. Both were free from extrahepatic bile duct involvement, intrahepatic and lymph node metastases. However, even in a symptom-free stage, perineural invasion, vascular or lymphatic involvements were histologically proved in both cases, which may be an indication of poor prognosis after surgery alone.

Major anatomical hepatic resection with regional lymph node dissection for liver metastases from colorectal cancer

Sixty-four patients with liver metastases from colorectal cancer were studied to clarify the characteristics of the regional spread of liver metastases (secondary invasive factors) and the effects of major anatomical hepatic resection with lymph node dissection on reducing liver recurrence. No secondary invasive factors, i.e., lymph node metastasis, portal or hepatic vein involvement, bile duct involvement, micrometastasis, and direct invasion, were observed in patients with liver metastases less than 3 cm in diameter (5-year survival rate; 100%). Secondary invasive factors were seen in 19.2% of the patients with liver metastases from 3 cm to less than 6 cm (5-year survival rate; 28.7%), and in 45.2% of those with liver metastases 6 cm and over (5-year survival rate; 14.6%). Secondary invasive factors were noted in 45% of the patients with recurrence in the remmant liver. Although 31% of all 64 patients exhibited secondary invasive factors, major anatomical hepatic resection with lymph node dissection achieved a low liver recurrence rate of 31.3%. In conclusion, considering the risks attributed to secondary invasive factors, major anatomical hepatic resection with lymph node dissection is an appropriate surgical procedure for patients with liver metastases exceeding 3 cm in diameter.

Bile duct involvement in hepatocellular carcinoma: MR demonstration.

To determine the magnetic resonance (MR) features of hepatocellular carcinoma (HCC) with associated bile duct involvement. MR examinations of six patients (mean age, 62 years) demonstrating bile duct involvement due to HCC were retrospectively reviewed and compared to surgical and pathologic findings. Three of the tumors were solitary, and three were multifocal. In two patients, MR showed direct biliary duct invasion by tumor. On T1-weighted MR images, four tumors were hypointense compared to the liver and two were isointense. On T2-weighted MR images, four tumors were hyperintense, and two were isointense. The two tumors studied with dynamic T1-weighted MR images obtained after intravenous administration of a gadolinium chelate, displayed enhancement similar to that of the liver. There was no evidence of a tumor capsule on either unenhanced or enhanced MR images. Intrahepatic bile duct dilatation was seen in five patients. The extrahepatic bile duct was normal in all cases. Although rare, HCC should be included when considering the etiology of intrahepatic bile duct obstruction. Imaging features suggestive of the diagnosis by MR include intrabiliary tumor or bile duct obstruction with an associated hepatic mass.

Oriental cholangiohepatitis: correlation between portal vein occlusion and hepatic atrophy.

The angiographic features of the hepatic vasculature in patients with oriental cholangiohepatitis and their relationship to liver atrophy remain unclear. We studied 11 patients with oriental cholangiohepatitis to define the spectrum of portal vein, bile duct, and parenchymal involvement by correlating findings on cholangiography, sonography, CT, and angiography. The portal veins appeared normal in five cases, pruned in four, and completely obstructed in two. This spectrum of appearances was found to correlate with the severity of liver atrophy. No massive arterioportal shunting was found in any patients in this series. The two patients with complete obstruction of the portal veins showed no evidence of malignancy at histologic examination of the resected specimen. It is concluded that the degree of portal vein obstruction correlates well with the degree of liver atrophy in patients with oriental cholangiohepatitis and that findings of complete central portal obstruction do not necessarily indicate associated malignancy.

Relationship of Inflammatory Bowel Disease and Primary Sclerosing Cholangitis

There is a strong association between PSC and IBD. PSC is the most common hepatobiliary lesion seen in association with IBD. Whether there are two subsets of PSC, one associated with IBD and one unassociated, is controversial. A lower male to female ratio in patients without IBD supports this view. The demonstration of the haplotype DRw52a in 100% of patients with PSC, irrespective of the absence of IBD, speaks against this view. Patients with isolated PSC tend to present with jaundice, pruritus, and fatigue more frequently than those with combined PSC and IBD. There may also be a difference in bile duct involvement between patients with and without IBD combined with PSC. Apart from usually being a total colitis, either Crohn's colitis or UC, the IBD associated with PSC cannot be distinguished from IBD without PSC with respect to symptoms and clinical course. Patients with combined IBD and PSC may have somewhat worse prognosis than those with isolated PSC. The majority of patients developing BDC have concomitant IBD, suggesting that patients without IBD represent a different subgroup of PSC and run a different clinical course. Most studies have, however, found no differences in epidemiology, pathogenetic factors, clinical findings related to the hepatobiliary disease and prognosis between those who present with PSC alone and those who present with combined PSC and IBD. A major problem when discussing the relationship between IBD and PSC is that the bowel is inadequately examined in many of the studies relating to this question.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnostic Value of Serum γ-Glutamyl Transpeptidase Activity in Liver Diseases in Children

The clinical usefulness of serum gamma-glutamyl transpeptidase (gamma GT) assay for the diagnosis of liver disease in children was assessed retrospectively in 398 children investigated from 1981 to 1986, in whom diagnosis was ascertained according to currently accepted criteria including liver histology in each case. Serum gamma GT activity was within normal limits in 10 controls, in 19 children with portal vein obstruction, and in 10 of 12 children with congenital hepatic fibrosis. Serum gamma GT was raised in all children with biliary atresia, sclerosing cholangitis, paucity of interlobular bile ducts, and alpha 1-antitrypsin deficiency with jaundice. Serum gamma GT was normal in spite of patent clinical signs of cholestasis in 3 patients with benign recurrent intrahepatic cholestasis, 1 infant with post-hemolytic neonatal cholestasis, and in 22 of 28 patients with progressive idiopathic cholestasis akin to Byler disease. In the latter group, children with raised serum gamma GT displayed extensive portal fibrosis and bile duct proliferation on liver histology, while this was not a prominent feature in children with normal serum gamma GT. These results indicate (a) the value and limits of the assay for serum gamma GT activity in children with liver disease, (b) that raised serum gamma GT may be considered a fairly reliable index of bile duct damage, and (c) that serum gamma GT may prove a useful tool in separating two forms of progressive idiopathic cholestasis, with or without bile duct involvement.

Diagnostic Value of Serum γ‐Glutamyl Transpeptidase Activity in Liver Diseases in Children

SummaryThe clinical usefulness of serum γ‐glutamyl transpeptidase (γGT) assay for the diagnosis of liver disease in children was assessed retrospectively in 398 children investigated from 1981 to 1986, in whom diagnosis was ascertained according to currently accepted criteria including liver histology in each case. Serum γGT activity was within normal limits in 10 controls, in 19 children with portal vein obstruction, and in 10 of 12 children with congenital hepatic fibrosis. Serum γGT was raised in all children with biliary atresia, sclerosing cholangitis, paucity of interlobular bile ducts, and α1‐antitrypsin deficiency with jaundice. Serum γGT was normal in spite of patent clinical signs of cholestasis in 3 patients with benign recurrent intrahepatic cholestasis, 1 infant with posthemolytic neonatal cholestasis, and in 22 of 28 patients with progressive idiopathic cholestasis akin to Byler disease. In the latter group, children with raised serum γGT displayed extensive portal fibrosis and bile duct proliferation on liver histology, while this was not a prominent feature in children with normal serum γGT. These results indicate (a) the value and limits of the assay for serum γGT activity in children with liver disease, (b) that raised serum γGT may be considered a fairly reliable index of bile duct damage, and (c) that serum γGT may prove a useful tool in separating two forms of progressive idiopathic cholestasis, with or without bile duct involvement.