巻号一覧
181 巻 (1997)
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181 巻, 1 号
January
選択された号の論文の27件中1～27を表示しています
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6th International Sendai Symposium on Biliary Atresia
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Nobuyoshi Shiojiri, Tohru Koike1997 年 181 巻 1 号 p. 1-8
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーDifferentiation of biliary epithelial cells from hepatic endodermal cells of the mouse embryo was examined with a special attention to the role of the connective tissue. When the whole liver primordium of the 9.5-day mouse embryo was cultured in vitro for 5 days, the endodermal cells differentiated into mature hepatocytes expressing carbamoylphosphate synthetase I (CPSI) and accumulating glycogen. Intrahepatic bile duct cells and connective tissue were poorly developed in this culture. However, when the hepatic endoderm was recombined with the 4-day embryonic chick lung mesenchyme and cultured in vitro, the endodermal cells differentiated into many ductal epithelial cells as well as mature hepatocytes with abundant connective tissue development. These results suggest that the ducts might be bile ducts, and that connective tissue is very important for bile duct development. In addition, this in vitro culture system might be useful for the study of mechanisms of bile duct differentiation and congenital biliary atresia.抄録全体を表示PDF形式でダウンロード (4056K) -
Yumi Kono, Maki Fukunaga, Kazuo Shiraki, Hideo Akiyoshi1997 年 181 巻 1 号 p. 9-18
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe effects of cytochalasin B and colchicine on the structure and function of bile canaliculi were studied in neonatal rat hepatocytes in primary culture. Cellular contacts of neonatal hepatocytes were not as tight as those of adult hepatocytes. There was no remarkable difference in the ultrastructure of bile canaliculi between neonatal and adult hepatocytes. Neonatal hepatocytes treated with cytochalasin B were round in shape and aggregated in groups of several cells. Actin filaments stained by rhodamine-phalloidin were disrupted and condensed at the cell periphery or around dilated bile canaliculi. Markedly-dilated bile canaliculi with less microvilli were observed by transmission electron microscopy while the secretory function of horseradish peroxidase, which was used as a marker for uptake, transport and secretion into bile canaliculi, were maintained. The lumen of dilated bile canaliculi was found close to the undersurfaces of hepatocytes by scanning electron microscopy after turning over the cultured cells. By colchicine treatment, the filamentous structure of microtubules in neonatal hepatocytes disappeared. The ultrastructure of the bile canaliculi was not affected by the treatment, but transport and secretion of horseradish peroxidase into bile canaliculi were inhibited. The development of strict cellular polarity in neonatal hepatocytes may be suppressed in neonatal hepatocytes; however, cholestatic agents which rearrange the cytoskeleton caused the same morphological or functional changes of bile canaliculi as in adult hepatocytes.抄録全体を表示PDF形式でダウンロード (4578K) -
Tadashi Terada, Yukisato Kitamura, Yasuni Nakanuma1997 年 181 巻 1 号 p. 19-32
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーMorphology and immunohistochemical features of the developmental process of the human intrahepatic biliary system (IBS) are reviewed. Human IBS arises from the ductal plate, a double-layered cylindrical structure located at the interface between portal mesenchyme and primitive hepatocytes. The ductal plate first appears from primitive hepatocytes (hepatoblasts) around 8 gestational weeks (GW), and its formation proceeds from the hepatic hilum to the periphery. The ductal plate gradually undergoes remodeling from 12 GW; some parts of the ductal plate disappear and other parts migrate into the portal mesenchyme. Around 20 GW, the migrated duct cells transform into immature bile ducts and peribiliary glands. Some immature peribiliary glands transform into pancreatic acinar cells around postnatal 3 months. The immature biliary elements express cytokeratins no. 7, 8, 18 and 19. Several growth factors (TGF-α, HGF) and their receptors (EGFR, MET, ERBB2) were expressed in the primitive IBS cells. Some extracellular matrix proteins including type IV collagen, laminin and tenascin are expressed in the mesenchyme around the primitive IBS. During IBS remodeling, apoptosis and cell proliferation occur with appropriate expression of apoptosis-related proteins (bcl-2, Fas, c-myc, Lewisy). Some pancreatic digestive enzymes (α-amylase, trypsinogen, lipase), cathepsin B, and matrix metalloproteinases (MMP-1, 2, 3, 9) and their inhibitors (TIMP-1, 2) are expressed in the remodeling IBS cells. Glycoconjugate residues of glycoproteins gradually appear during IBS development. The appropriate expression of these immunophenotypes may play an important role in the normal development of IBS.抄録全体を表示PDF形式でダウンロード (2435K) -
Peter W. Dillon, Deborah Belchis, Kathleen Minnick, Thomas Tracy1997 年 181 巻 1 号 p. 33-40
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーAberrant expression on biliary epithelial cells of the major histocompatibility complex (MHC) antigens in association with adhesion molecule intercellular adhesion molecule-1 (ICAM-1) may be crucial to the immunopathogenesis of biliary atresia. The patterns of MHC class I and II expression in relation to ICAM-1 expression as well as the associated lymphocyte subpopulations were studied in frozen section liver biopsies from six infants with biliary atresia. Intense ICAM-1 expression was found on all ductal epithelial cells in association with MHC I. No ductal epithelial cells demonstrated MHC II expression. Lymphocyte populations within the portal tracts all expressed LFA-1 and were predominantly CD4 positive (>70%). CD8 positive cells accounted for less than 30%. The expression of ICAM-1 appears to be important in the pathogenesis of biliary atresia but is not linked to the expression of MHC II determinants. This result suggests that different regulatory mechanisms govern the expression of these important immunological receptors on biliary epithelial cells.抄録全体を表示PDF形式でダウンロード (685K) -
Masahiro Nakada, Koonosuke Nakada, Fumio Kawaguchi, Munechika Wakisaka ...1997 年 181 巻 1 号 p. 41-47
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe characteristic histopathological features seen in the livers of patients with biliary atresia (BA) are very similar to those of primary biliary cirrhosis, which is an autoimmune disease. To clarify whether BA liver possesses an immunological response similar to that in primary biliary cirrhosis, we studied HLA-DR expression in liver tissue of BA patients, using a HLA-DR staining method, and determined the frequency of HLA types in BA patients and their families. HLA-DR was expressed by the bile duct epithelium in 11 of 16 liver specimens obtained from 13 BA patients. By contrast, HLA-DR was not expressed in liver specimens from 6 patients with congenital biliary dilatation. Among the HLA types seen in BA patients and their families, HLA-A33, -B44 and -DR6 were frequently expressed in blood. These results suggest that certain immunological factors and disease-susceptible genes might be involved in the etiology of BA.抄録全体を表示PDF形式でダウンロード (2652K) -
Antonio Dessanti, Giovannino Massarelli, Maria T. Piga, Alberto Porcu, ...1997 年 181 巻 1 号 p. 49-55
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーA unique case of newborn biliary atresia associated with esophageal atresia and tracheoesophageal fistula, ano-rectal atresia, Reovirus type 3 infection and an early switch of fetal into adult hemoglobin is reported. At birth, the infant, who had only one umbilical artery, was operated on by primary anastomosis of the esophagous, and descending colostomy. At six weeks of age the baby underwent a “Kasai hepatic portoenterostomy-Type I” for a EHBA Type III, Subtype C2, Subgroup O (“aplasia” of all extrahepatic biliary ducts, including the gallbladder). The absence of an artery branch for the left lobe of the liver was observed. Histologically, the liver showed a hyperplasia of the intrahepatic bile ducts due to persistence of an excess of embryologic bile ducts in “ductal plate malformation” (DPM). Specific Reovirus type 3 antibodies were found in both the mother's and baby's sera. In the postoperative period the infant developed rapid and severe liver failure and underwent a successful liver transplantation. Although in most cases EHBA appears to be a perinatal event due to a necroinflammatory process of unknown etiology, cases associated with complex extrahepatic anomalies, may be due to different pathogenetic mechanisms supported by different causative agents operating very early in the fetal period. Viral infection seems to be the most reliable etiology.抄録全体を表示PDF形式でダウンロード (1712K) -
Daiji Iwami, Ryoji Ohi, Masaki Nio, Satoru Shimaoka, Nobuyuki Sano, Hi ...1997 年 181 巻 1 号 p. 57-65
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーWe investigated changes in the pattern of hepatic innervation in liver specimens from 15 infants with biliary atresia and 4 age-matched controls by immunohistochemical methods. In the control, nerve fibers identified by immunoreactivity for neural cell adhesion molecule (NCAM) and S100 protein were present around the branches of hepatic arteries, portal veins and bile ducts in the portal areas and the hepatic lobules. In biliary atresia, NCAM and S100 positive nerve fibers were increased in the vicinity of the hepatic arteries and the portal veins in the enlarged portal areas, while no nerve fibers were observed around bile ducts and periportal ductules which became NCAM positive. No innervation in the lobules was seen in any cases regardless of the histological alteration. These findings may suggest that the abnormal innervation in the liver with biliary atresia does not occur as a result of structural changes in liver architecture caused by portal fibrosis and inflammation, but is associated with immaturity or malformation of hepatic innervation in the patients.抄録全体を表示PDF形式でダウンロード (3124K) -
Naomi Ohnuma, Hideyo Takahashi, Masahiro Tanabe, Hideo Yoshida, Jun Iw ...1997 年 181 巻 1 号 p. 67-74
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーWe performed a total of 75 examinations with endoscopic retrograde chlangiopancreatography (ERCP) in 73 infants aged from 8 days to 300 days (mean 71 days) between 1977 and 1995. ERCP examination was performed with a prototype duodenoscope and was successful in 47 of 52 examinations in biliary atresia, 9 of 11 in neonatal hepatitis, all 4 in paucity of intra-hepatic bile duct, 4 of 5 in congenital biliary dilatation, 1 of 2 in duodenal stricture, and 1 case of in postoperative jaundice of hepatoblastoma. In 46 infants with biliary atresia, excluding one in whom the findings could not be evaluated due to poor x-ray image quality, we distinguished the following four patterns of ERCP findings: Pattern 1, only the pancreatic duct could be demonstrated and no bile duct was visualized (76%); Pattern 2, only a part of the distal common bile duct with the pancreatic duct was visualized (2%); Pattern 3, the entire length of the common bile duct with the pancreatic duct was visualized without the gallbladder and the common hepatic duct (4%); and Pattern 4, the common bile duct and the gallbladder with the pancreatic duct were visualized without the common hepatic duct (18%). In all of these 46 patients, laparotomy, an operative cholangiogram, and histological evaluation of the biliary duct were performed. In 9 neonates with neonatal hepatitis, the biliary tract was opacified and biliary atresia was excluded. Laparotomy was thus avoided in these neonates. There was no complication caused by either ERCP or by anesthesia.抄録全体を表示PDF形式でダウンロード (2147K) -
Edward R. Howard, Mark Davenport1997 年 181 巻 1 号 p. 75-83
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーEuropean studies of biliary atresia have suggested that the aetiology is heterogeneous. Histological studies of the liver and biliary remnants excised at portoenterostomy have failed to identify any prognostic features except for the size of bile ductules in the porta hepatis. Most of the major series have confirmed that there is a relationship between age at portoenterostomy and clearance of jaundice which has been achieved in more than 67% of infants under 10 weeks of age. Cholangitis reduced survival and bleeding from esophageal varices has occurred in more than 19% of long-term survivors. The 5-year jaundice-free survival rate after portoenterstomy is 37% and the 10 year rate is 18%. It is predicted that these survival rates will improve. Orthotopic transplantation now results in long-term survival in 70% of patients who fail the portoenterostomy operation and long-term survival is now achieved in a majority of children born with biliary atresia.抄録全体を表示PDF形式でダウンロード (509K) -
Mohamed Ibrahim, Takeshi Miyano, Ryoji Ohi, Morihiro Saeki, Kazuo Shir ...1997 年 181 巻 1 号 p. 85-95
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe Japanese Biliary Atresia Society founded in 1980 for the aim of investigations of all aspects of biliary atresia (BA), started a nationwide registry of BA patients in 1989. A total of 626 cases were registered from 1989 to 1994. The male to female ratio was 0.58. Corrective surgery was performed in 603 patients. Regarding the type of obstruction, 63 cases were Type I, atresia of the common bile duct, 9 were Type II, atresia of the hepatic ducts, and 543 were Type III, atresia of the porta hepatis. As initial corrective procedures, original Roux-en Y, Suruga II and Roux-en Y with intestinal valve were mainly employed. Jaundice cleared in 346 patients (57%) and decreased in 131, while it persisted in 120. The 5-year-follow-up showed that 34 patients, 49% of the patients who were followed up, were alive without jaundice, while 28 (41%) are dead. Thirty five, 33% of the patients who were entered to the Registry, were lost to follow-up.抄録全体を表示PDF形式でダウンロード (518K) -
Frédéric Gauthier, Jean-Luc Luciani, Christophe Chardot, ...1997 年 181 巻 1 号 p. 97-107
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe aim of this work is to determine the influence of age, extrahepatic biliary lesions pattern (EHBP) and association to polysplenia syndrome (PS) on 10 years outcome of 164 patients with biliary atresia (BA) treated from 1984 to 1992 by initial Kasai operation (KO) and secondary liver transplantation (LT) when necessary. Actuarial crude survival without or after LT(CS), actuarial survival with native liver (NLS) and jaundice-free actuarial survival with native liver (JFS) were calculated from 1 to 10 years versus age (under/over 45 days), EHBP (favorable/unfavorable) and PS (no/yes). Overall 10-year CS is 70%, overall 10-year NLS and JFS are 14%. In univariate analysis, age at KO under 46 days, favorable EHBP (BA with patent gallbladder, and/or cystic dilatation of extrahepatic bile duct, or BA restricted to choledocus), and absence of PS are significant determinants of a better outcome regarding CS, NLS and JFS. EHBP is more discriminant than age. Influence of PS in this series is redundant with that of EHBP since 11/11 patients with PS had unfavorable EHBP.抄録全体を表示PDF形式でダウンロード (615K) -
Htut Saing, Sheung Tat Fan, Kwong Leung Chan, William Wei, George Hlai ...1997 年 181 巻 1 号 p. 109-116
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーNinety three out of 96 patients with biliary atresia (BA) underwent Kasai 1 portoenterostomy and 11 children subsequently underwent 12 liver transplantation (LTX) procedures which included 8 living related liver transplants (LRLT), 3 reduced-size liver transplants (RSLT) and 1 orthotopic liver transplantation (OLT). During a follow-up period of 3-206 months (mean, 73 months) after portoenterostomy and 3-63 months after LTX, 50% of 96 patients are alive and well with total clearance of jaundice while 12% are mildly jaundiced, 10% are deeply jaundiced and 28% have died. Two of the 3 patients who did not undergo portoenterostomy and 25 from the portoenterostomy group have died. Of the latter group, 22 deaths occurred before, and 3 after the introduction of LTX therapy respectively. Of the 68 long-term survivors, 32 are less than 5, and 36 are 6-17 years old. Complete clearance of jaundice was achieved in 55% of patients irrespective of whether portoenterostomy was initially performed at <10 or between 10 and 12 weeks. Portoenterostomy performed beyond 13 weeks was associated with declining results. We conclude that (1) portoenterostomy combined with liver transplantation, when indicated, has given patients with BA a much better prognosis and (2) pediatric LTX in our institute is a well established procedure with 100% patient and 88% primary graft survival.抄録全体を表示PDF形式でダウンロード (493K) -
Sanjay Nagral, Paolo Muiesan, Hector Vilca-Melendez, Giorgina Mieli-Ve ...1997 年 181 巻 1 号 p. 117-127
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーKasai portoenterostomy has transformed the prognosis for children with Extra Hepatic Biliary Atresia (EHBA). However, for children developing end stage liver disease following portoenterostomy, liver transplantation (OLT) is the treatment of choice. Between February 1989 and March 1996, 64 children with EHBA underwent 79 transplants (26 males, 38 females; median age 2.2 years, range 5 months-17 years; median weight 11.4 kg, range 5-65 kg). Of these, 58 (85%) had undergone previous portoenterostomy. Nineteen patients (30%) had gastrointestinal bleeding prior to OLT assessment. Mean serum bilirubin was 229 μmol/ liter (range 11-801 μml/liter). Four children had associated polysplenia syndrome. Of the 79 transplants, 30 received whole and 41 reduced-size cadaveric grafts and 9 living related grafts. Eleven patients (17%) died, nine within one month of surgery. Thirteen patients were retransplanted once and one twice. There were 16 vascular complications (10 hepatic artery thrombosis, 3 portal vein thrombosis, 3 venous outflow obstruction) and 10 biliary complications (4 anastomotic leaks, 6 strictures). Ten patients (16%) had bowel perforation following the transplant. The 5 year actuarial patient and graft survival for this group is 84% and 69% respectively with normal physical and mental development in the majority. OLT provides satisfactory treatment for children with EHBA with end stage liver disease with long term survival in the majority.抄録全体を表示PDF形式でダウンロード (718K) -
Frédéric Gauthier, Christophe Chardot, Sophie Brancherea ...1997 年 181 巻 1 号 p. 129-138
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーAccording to French rules for cadaver organ sharing, children with biliary atresia (BA) complicated with acute necrosis (ALN) can be registered on the waiting list for liver transplantation (LT) in a special intermediate grade urgent code. Over a 7 years period, 100 children have been submitted to elective LT for BA and 15 to urgent LT. Urgent procedures accounted for 25% of LT for BA in patients aged 0-2 years and 67% (8/12) in patients under 1 year of age. Children actuarial survival at 1, 12 and 48 months was respectively 66%, 60% and 60% versus 92%, 86% and 85%, deaths occuring earlier in the urgent group. Graft actuarial survival at 1, 12 and 48 months were 60%, 53% and 53% versus 85%, 77% and 76% (p<0.05), respectively. Outcome of children and grafts after LT is not significantly different in BA cases and in other urgent indications, excluding retransplantations. In a LT program based on cadaver organ donation, allocation of in an urgent registration code to children with BA and ALN offers them more than 50% chance to escape death and does not result in wasting of grafts.抄録全体を表示PDF形式でダウンロード (458K) -
Hirotaka Katoh, Nobuhiro Ohkohchi, Susumu Satomi, Satoru Shimaoka, Ryo ...1997 年 181 巻 1 号 p. 139-144
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーEleven children, 4 males and 7 females, with biliary atresia receiving living related liver graft were studied. The mean age was 1.8 years and the mean body weight was 10.3 kg. The donors were 4 fathers and 7 mothers. The graft was the lateral segment or left lobe. ABO blood group matching was compatible in 9 and incompatible in 2. All patients except one were crossmatch negative. Immunosuppression at induction was triple therapy (cyclosporine, azathioprine and steroid) or FK506 plus steroid. Acute rejection episodes were treated with pulse steroids. When the signs of rejection persisted despite steroid pulse therapy, 15-deoxyspergualin (DSG) was added. The survival rate of the patients was 73%. Three patients died of portal vein thrombosis, hepatic artery thrombosis and sepsis respectively. Other major complications included hyperbilirubinemia, bile duct stenosis, bile leakage and portal vein anastomosis narrowing. Complications of the donor were sepsis in one, and liver dysfunction in two. Although there are some complications related to graft size mismatch and operative procedure, living related partial liver transplantation is an effective therapy in countries where donor source is restricted.抄録全体を表示PDF形式でダウンロード (398K) -
Yorihiro Akamatsu, Nobuhiro Ohkohchi, Kazuhiko Seya, Susumu Satomi1997 年 181 巻 1 号 p. 145-154
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe diagnosis of acute rejection in liver transplantation usually needs hepatic biopsy, but hepatic biopsy sometimes involves severe complications. We analyzed biliary bilirubin fraction after living related liver transplantation by using high performance liquid chromatography (HPLC) and investigated availability for the early diagnosis of acute rejection retrospectively. Nine children with liver cirrhosis due to biliary atresia were inculuded in this study, who underwent living related liver transplantation at The Second Department of Surgery, Tohoku Uninversity School of Medicine. Bile was collected daily from a biliary canulae inserted into the hepatic duct of the graft under aseptic and without exposure to the light. We measured the proportion of bilirubin diglucuronide (BDG), bilirubin monoglucuronide (BMG) and unconjugated bilirubin (UCB) of bile pigments in the bile by HPLC. In three of four patients with acute rejection, BDG+BMG (=Bc) was above 85% and BDG/Bc ratio was below 0.6 at the time of hepatic biopsy. After rejection therapy, BDG/Bc ratio increased in their bile. The remaining one case with acute rejection as well as bile duct injury due to arterial thrombosis of S2, Bc was below 85%, and BDG/Bc ratio was below 0.6. In four of the other five patients who had several severe complications, i.e., arterial or portal vein thrombosis, bile stasis due to cholangitis and sepsis due to necrotizing myofascitis, Bc was below 85% and BDG/Bc ratio was below 0.6. We concluded that analysis of biliary bilirubin fraction after liver transplantation could be reliable as a noninvasive maker and valuable for the early diagnosis of acute rejection.抄録全体を表示PDF形式でダウンロード (560K) -
Toshihiro Muraji, Eiji Nishijima, Yasuyuki Higashimoto, Chikara Tsugaw ...1997 年 181 巻 1 号 p. 155-160
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーBetween 1986 and 1994, 42 patients with BA were treated at the Kobe Children's Hospital. These patients underwent a wider excision of the hilar fibrous remnant with Roux-Y reconstruction (with or without intussuscepted valve) without stoma. Corticosteroids were used postoperatively when the stool was acholic or unsteadily cholic. The daily dose was reduced from 20 mg/day by half down. The patients were divided into two groups; in Group I (n=17, before October 1990), a single course of corticosteroid therapy was employed. In Group II (n=25, from November 1990 on), this regimen was repeated whenever the stool appeared less cholic. The bile flow improved significantly (excellent in 29% and 60%, and poor in 71% and 32% in Groups I and II, respectively.) Corticosteroids were used in 15 Group I patients with good response in 10 and in 21 Group II patients, 15 of whom had multiple courses. Sixteen of the 21 Group II patients had a good response. The incidence of the cholangitis was not significantly different between the 19 patients with valve and the 23 patients without valve. A 5 year survival significantly improved from 70% in Group I to 96% in Group II. In both groups, the survival rate significantly increased, when compared with the survival rate figured out with an assumption of OLT survivors as dead. On the same assumption, the survival rate of Group II is significantly more than that of the Group II. These suggest a positive contribution of liver transplantation and an aggressive corticosteroid therapy on better survival of Group II.抄録全体を表示PDF形式でダウンロード (298K) -
John Ratan, S. Rohatgi, D.K. Gupta, Simmi Ratan1997 年 181 巻 1 号 p. 161-166
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe authors have tried to examine the hepatoprotective and cholerectic action of a new indigenised drug, Livzon (Hind Chemicals Ltd., Kanpur, India) and compared its action to Decholin (casella—Riedel Pharma GmbH, Frankfurt, Germany), a known hepatoprotective and choleretic agent. Albino rats were chosen as the experimental animals. Obstructive jaundice was created by ligating the common bile ducts after taking liver biopsies. The animals were divided into three groups: (i) Control group-no drug was given, (ii) Livzon trial group, (iii) Decholin group. The animals were reoperated, liver biopsies were taken and histologically examined. The study confirmed the hepatoprotective and choleretic actions of Livzon and Decholin. However, Decholin was more of a choleretic, the Livzon was more hepatoprotective.抄録全体を表示PDF形式でダウンロード (423K) -
Hisami Ando, Takahiro Ito, Takahiko Seo, Fujio Ito, Kenitiro Kaneko1997 年 181 巻 1 号 p. 167-174
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーSplenectomy was performed for three patients with biliary atresia because of re-exacerbation of their jaundice following treatment by partial splenic embolization (PSE). The subjects' red blood cell count and hemoglobin, serum level of hepatic enzymes (glutamic oxaloacetic transaminase, glutamic pyruvic transaminase, gamma-glutamyl transpeptidase, alkaline phosphatase, and lactic dehydrogenase), and total bilirubin (TB) were evaluated both before and after splenectomy in order to analyze the effects of splenectomy on these patients. The TB decreased significantly within 3 months after splenectomy in all three patients (13.0±1.6 mg/100 ml to 5.4±0.3 mg/100 ml, p<0.05). The red blood cell count and hemoglobin increased gradually. There was a statistically significant correlation between the TB and the red blood cell count, and/or concentration of hemoglobin. The hepatic enzymes after splenectomy were not significantly different from those before splenectomy. The change in TB following splenectomy was essentially similar to that following PSE. These results suggested that the postoperative improvement in jaundice following splenectomy may not be due to improved hepatic function but merely a reflection of decreased red blood cell turnover. Splenectomy is a useful palliative procedure for jaundice in patients with biliary atresia for whom PSE is no longer effective.抄録全体を表示PDF形式でダウンロード (1098K) -
Hideo Kawarasaki, Mitsuhiro Itoh, Koichi Mizuta, Hideaki Tanaka, Masat ...1997 年 181 巻 1 号 p. 175-183
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThis is a report on ten patients with cystic dilatation of the intrahepatic biliary system (CDIB) after hepatic portoenterostomy. They were five girls and five boys and the diagnosis of CDIB was made at ages 6 months to 11 years (mean age: 2.8±3.3 years). Follow-up ranged from one month to 15 years (mean: 5.5±4.9 years). In order to elucidate the factors which affect the clinical outcome of such patients, the types of CDIB (Type A: non-communicating solitary cyst, Type B: communicating solitary cyst, Type C: multi-cystic dilatation), clinical symptoms at onset of CDIB and the method for the treatment were reviewed in relation to the outcome. For the purpose of understanding pathogenesis of CDIB, immunohistochemical study on hepatobiliary system was done with monoclonal antibody for cytokeratin. Outcome of the patients of Type C was poor, whereas the outcome of patients with type A and B was good. The outcome of preoperatively jaundiced patients was poor, but jaundice-free patients showed good outcome. Method of treatment was not related to the outcome. As epithelium of CDIB was positive for monoclonal antibody of cytokeratin, it was suspected that pathogenesis of CDIB might be related to peribiliary gland which originated from ductal plate.抄録全体を表示PDF形式でダウンロード (2252K) -
Yasuhiro Watanabe, Takuji Todani, Akira Toki, Takuo Noda, Yasuhisa Sat ...1997 年 181 巻 1 号 p. 185-191
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe number of long term survivors who have undergone Kasai operation for biliary atresia is increasing, but some have a hepatic dysfunction likely to require liver transplantation in the near future. Hepatic volume possibly reflects whole liver function, and our objective was to assess the changes of hepatic volume after Kasai operation. Ten patients were studied. Ages ranged from 3 to 27 years. They underwent Kasai operation at ages ranging from 50 to 80 days. Liver areas (cm2) on CT images were measured with an image processing and analysis program (NIH Image 1.57). Hepatic volume (cm3) was calculated by summing up the areas of each image and multiplying by slice thickness (cm). After Kasai operation, the size of the liver increased to 1.7-1.9 times the standard volume, and then reduced to normal size around 5 years of age. In the teens, hepatic volume decreased below the standard volume. Segmental hypertrophy accompanying atrophy of other hepatic segments was observed in 9 out of 10 patients; right lobe hypertrophy: 6, medial segment: 2, and lateral segment: 1. Therefore, progressive hepatic atrophy begins in the teens, but is compensated for by segmental hypertrophy.抄録全体を表示PDF形式でダウンロード (1723K) -
Atsushi Takahashi, Shin-Itsu Hatakeyama, Norio Suzuki, Minoru Kuroiwa, ...1997 年 181 巻 1 号 p. 193-202
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーTo evaluate liver function in biliary atresia (BA) patients after the Kasai operation, magnetic resonance imaging (MRI) was carried out 28 times in 19 BA patients. Sixteen of these were divided into three groups on the basis of the serum level of total bilirubin (t-bil), glutamic pyruvic transaminase (GPT) and γ-glutamyltransferase (γ-GTP) in the postoperative follow- up period (2-11 years). In group 1 (n=7) the t-bil was continuously kept under 1 mg/100 ml. In group 2 (n=4) the t-bil was continuously kept under 1 mg/100 ml, but GPT and γ-GTP remained high (GPT> 100 IU/liter, γ-GTP>200 IU/liter) for more than 2 years. In group 3 (n=5) an increase in the t-bil level reappeared (1-2 mg/100 ml; n=4, >2 mg/100 ml; n=1). The differences between MRI finding in the 3 groups, and the correlation between MRI findings and laboratory data (t-bil, cholinesterase; ChE, GPT and γ-GTP), which were taken around the time of MRI examinations, were studied. The results were as follows: (1) All 19 patients had normal or high signl areas of various sizes on T1 weighted images (WI). (2) Eighteen of 19 patients had high signal areas of various sizes on T2 WI in the portal system areas and/or liver parenchyma, and these areas were enhanced by gadolinium-DTPA in about half of the patients. (3) Ten of 16 patients had atrophic change over one liver lobe. (4) MRI findings for group 1 and the other groups were significantly different, and MRI findings, except for atrophic change, were correlated with the increase or decrease in laboratory data taken around the time of MRI examinations (p<0.05). These results indicate that a normal or high signal area on T1 WI shows functional tissue, and that a high signal area on T2 WI shows tissue damaged by inflammation and/or progressive fibrosis. MRI is useful for evaluating liver function, especially in terms of morphological features, in BA patients after the Kasai operation, and will be one method for establishing their prognosis.抄録全体を表示PDF形式でダウンロード (2421K) -
Satoru Shimaoka, Ryoji Ohi, Masaki Nio, Daiji Iwami, Nobuyuki Sano1997 年 181 巻 1 号 p. 203-211
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーThe scintigram using 99mTechnetium-DTPA galactosyl human serum albumin (99mTc-GSA) which binds to asialoglycoprotein receptors on hepatocytes is a good index of hepatocyte function in various liver diseases in adult patients. In 43 patients (4 months to 30 years old) who had undergone Kasai procedure, we performed 53 series of 99mTc-GSA scintigrams and checked the laboratory data of blood draw and the clinical status. The indices for blood clearance and liver accumulation were evaluated on the basis of the dynamic data after 99mTc-GSA injection. HH155 as an index of the blood clearance, and LHL15 as an index of the accumulation of the hepatocytes were calculated and the HH15/LHL15 ratio (H/L15) was examined. 99mTc-GSA scintigram correlated with liver function and clinical status. Our results revealed that 1) The deterioration of the liver functions and clinical status correlates proportionally with H/L15, 2) The results of 99mTechnetium-GSA scintigram correlate with several liver function tests, especially direct bilirubin, albumin and choline esterase, 3) This scintigram is an useful index of clinical status and hepatic function as well as the change of the hepatic parenchymal reserve in BA patients, especially for the evaluation of liver transplantation.抄録全体を表示PDF形式でダウンロード (944K) -
Akira Toki, Takuji Todani, Yasuhiro Watanabe, Yasuhisa Sato, Kaoru Ogu ...1997 年 181 巻 1 号 p. 213-216
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーMetabolism is probably disturbed in patients with abnormal liver function who have undergone a successful Kasai procedure. We examined bone mineral metabolism in patients who have successfully undergone Kasai procedure. Bone mineral metabolism was examined in 8 patients with biliary atresia after a successful Kasai procedure. Five patients were female and 3 were male. The ages at Kasai procedure ranged from 50 to 80 days, and the follow-up periods ranged from 3 to 27 years after the operation. All patients eat a normal oral diet. We examined plasma levels of 25-OH-D3, 1, 25-(OH)2-D3, Ca and phosphorus (P). Bone mineral content of the lumbar spine (L2-L4) was assessed by dual energy x-ray absorptiometry (DEXA), and the data were expressed as a bone mineral density (BMD). Two patients showed abnormal levels on hepatic function tests. Plasma levels of 1, 25-(OH)2-D3, Ca, and P were normal in all patients. The level of 25-OH-D3 was normal in 7 patients. BMD levels were normal in 6 patients, but low in 2 who had undergone partial splenic embolization and splenectomy, respectively, due to hypersplenism. In long-term survivors of Kasai procedure, measurement of BMD may detect bone mineral deficiency earlier than measurements of serum levels of 25-OH-D, 1, 25-(OH)2-D, Ca and P.抄録全体を表示PDF形式でダウンロード (223K) -
Chizuko Shiga, Ryoji Ohi, Toshio Chiba, Masaki Nio, Naobumi Endo, Sets ...1997 年 181 巻 1 号 p. 217-223
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーSome patients of biliary atresia (BA) suffer from chronic hepatic dysfunction and/or persistent jaundice. The adverse effects of chronic liver disease on nutrition and growth should be considered on BA patients. We studied 45 BA patients ranging in age from 0.5 to 38 years and divided them into 2 groups. Group A contains the patients whose total bilirubin ≥2 mg/ 100 ml, and Group B contains the patients whose total bilirubin <2 mg/100 ml. We measured height, weight, triceps skin fold (TSF), midarm circumference (MAC) and midarm muscle area (MAMA). Visceral protein kinetics was evaluated on the basis of serum albumin and prealbumin levels. Caloric and protein intake was calculated by collecting intake data for 3 days. The results of this study were; 1) The mean TSF in Group A (47th percentile) was not significantly different from that in Group B (53th percentile). 2) The mean MAMA was significantly lower (p<0.01) in Group A (16.4th percentile) than in Group B (36.7th percentile) 3) The prealbumin level was significantly lower (p<0.001) in Group A (mean 9.9 mg/100 ml) than in Group B (mean 18.8 mg/100 ml). The authors conclude that the evaluation of MAMA and prealbumin were very useful to characterize the low metabolic status of protein in the damaged liver. And repeated nutritional assessment was necessary to evaluate liver function and provide adequate nutrition in BA patients.抄録全体を表示PDF形式でダウンロード (354K) -
Yasunobu Shimizu, Takashi Hashimoto, Yoshihiro Otobe, Nobuhisa Ueda, Y ...1997 年 181 巻 1 号 p. 225-233
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーRecently, there has been remarkable increase in the survival rate in cases of biliary atresia (BA). However, long-term survivors are as yet a small population. Of the total of 152 patients undergoing surgery for BA during the period from 1969 to 1995 in our institution, 39 of them were operated on more than 20 years ago with follow up for all but one of these, who can no longer be accounted for. Six are still alive, 1 male, and 5 females, two cases being of type I BA, and the other 4 of type III and 1 is unaccounted for. The prognosis of these individuals was clinically evaluated. At the present time, none of them is receiving hospital care, but 3 have experienced sequelae. Two patients required partial splenic embolization (PSE), endoscopic infusion sclerotherapy (EIS), and/or gastroesophageal decongestion and splenectomy (Hassab's operation) (Hassab 1967) for hypersplenism and/or portal hypertension. The other has needed hospital care for recurrent cholangitis. Laboratory investigations revealed a serum total bilirubin (TB) of less than 1.0 mg/100 ml in 3 of the 5 patients for which samples could be obtained, between 1.0 and 2.0 mg/100 ml in 1, and in excess of this in the remaining case. The l-alanine 2 oxoglutarate aminotransferase (ALT) level was within the normal range in only 1, and was mildly to moderately elevated in 4. The white blood cell count (WBC) was less than 3,000/μl and the platelet count was less than 10×104/μl in 1, and within the normal ranges in the other 4 patients. The results thus indicate that occult and progressive liver damage may occur in long-term survivors of BA.抄録全体を表示PDF形式でダウンロード (2512K) -
Masaki Nio, Ryoji Ohi, Satoru Shimaoka, Daiji Iwami, Nobuyuki Sano1997 年 181 巻 1 号 p. 235-244
発行日: 1997年
公開日: 2006/03/22
ジャーナル フリーBetween 1953 and 1995, 300 patients with biliary atresia underwent surgery at Tohoku University Hospital. The 10-year survival of patients who were operated on in or before 1965 was 9%. But the survival rate went up to 61% in patients operated on between 1976 and 1985. Eighty-five patients including 2 who developed liver failure after Kasai operation and underwent liver transplantation have survived more than 10 years. Eleven of them (13%) have recurrent or persistent jaundice. Of the 30 patients who have survived more than 20 years (10 males and 20 females, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepatic portocholecystostomy. None of these patients has undergone liver transplantation. Twenty-two patients have led near-normal lives. The remaining 8 patients have experienced some troubles due to cholangitis, portal hypertension, intrahepatic gallstones and so on. Two of them are considered as candidates for liver transplantation. While the majority of long-term survivors of biliary atresia have good quality of life, close long-term follow-up is essential even in patients with biliary atresia aged 20 years or more.抄録全体を表示PDF形式でダウンロード (526K)
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