Significance of glomerular activation of the alternative pathway and lectin pathway in lupus nephritis.

The objective of the present study was to elucidate the association between glomerular complement depositions belonging to the alternative (AP) and lectin (LP) pathways, and clinical findings of lupus nephritis (LN). Immunofluorescence (IF) was performed on 17 LN patients using antibodies against factor B, factor H, properdin, mannose-binding lectin (MBL) and L-ficolin. Compared with factor B/factor H negative patients (n = 9), positive patients (n = 8) showed longer duration of LN (p < 0.05) and more severe interstitial fibrosis (p < 0.05). Eleven patients had properdin deposition in glomeruli, and in three of them, with a duration of LN of less than 1 month, factor B was undetectable. Compared with properdin negative patients (n = 6), positive patients (n = 11) showed significantly higher urinary protein excretion (p < 0.01). MBL/L-ficolin positive patients (n = 11) also had significantly higher urinary protein excretion (p < 0.05) compared with negative patients (n = 6). An independent association was found between glomerular deposition of properdin and that of MBL/L-ficolin (p < 0.01) in addition to factor B/factor H. Traces of glomerular activation of AP and LP reflected the clinical status of LN. It appears that glomerular deposition of each complement component, especially properdin, may be an index of the histological activity of LN.

Cylindrical dilatation of the choledochus: a special type of congenital bile duct dilatation.

Cylindrical dilatation of the choledochus develops in 20% of patients with congenital bile duct dilatation and usually has acute-angled unions of the pancreatobiliary ductal system. Symptoms generally develop in patients over 1 year of age. The patients frequently complain of abdominal pain, vomiting, and fever as in those with acute pancreatitis. Ultrasonography and infusion cholangiography are the most useful tools in making a correct diagnosis. A high amylase level in the bile caused by the refluxing of pancreatic juice through anomalous ductal unions is commonly observed. This is responsible for biliary perforation in infancy and possibly carcinoma arising in the bile duct. The amylase concentration in the serum at the time of epigastric pain often is high, which leads to the diagnosis of acute pancreatitis. However, evidence of pancreatic inflammation is seldom noted. Accordingly, amylase in the bile may enter the circulating blood through the denuded epithelium or sinusoids of the liver. Excision of the whole extrahepatic duct along with hepaticoenterostomy would be essential for the treatment of cylindrical dilatation of the bile duct, especially when an anomalous ductal union is present.

Congenital choledochal cyst with intrahepatic involvement.

We reviewed congenital choledochal cyst with intrahepatic involvement in 38 patients aged 52 days to 29 years. Dilatations were classified into the following three forms, based on the shape of the extrahepatic and intrahepatic bile duct: cystic-cystic, cystic-cylindrical, and cylindrical-cylindrical. Stenosis of the hepatic duct near the hilum probably does not cause dilatation of the intrahepatic duct. The anomalous arrangement of the pancreatobiliary union seems to determine only the form of extrahepatic dilatation, and cystic dilatation of the intrahepatic duct may be caused by some other factors. Total excision of the extrahepatic bile duct with the creation of a wide anastomotic stoma apparently is the treatment of choice. Hepatectomy would be recommended for some patients with the cystic-cystic form.

Hepaticoduodenostomy at the hepatic hilum after excision of choledochal cyst.

During the past 11 years, 30 patients, aged 27 days to 25 years, underwent excision of choledochal cyst. Reconstruction of the biliary tract was performed by two techniques: hepaticoduodenostomy in 19 patients and Roux-Y hepaticojejunostomy in 11. The technique of hepaticoduodenostomy consisted of transection of the common hepatic duct at the hilum with an incision extending approximately 5 mm along the lateral wall of both the hepatic ducts to permit a wide anastomotic stoma. There was no mortality. Postoperative cholangitis occurred in five patients, four of whom were in the hepaticoduodenostomy group. All episodes except one responded to antibiotics and have resulted in no demonstrable hepatic dysfunction. Intestinal bleeding occurred in one Roux-Y patient and postoperative intestinal obstruction in another. There was no significant difference in the results of these two procedures in the follow-up period (average length 4 years, 3 months). The hepaticoduodenostomy with a wide stoma at the hilum is advocated because (1) it has significant capability of preventing cholangitis, which has been thought to be the primary objection so far, (2) it creates a better physiologic state, and (3) it may be associated with fewer postoperative complications.

Effects of enteral fat emulsion on fat absorption in obstructive jaundiced rats.

The effects of fat emulsion given enterally on fat absorption were studied with obstructive jaundiced rats (J group) as compared with jaundice-free rats (C group). The J and C groups were subdivided into JE and CE groups using emulsified fat for the fat absorption test, and JU and CU groups using unemulsified fat. Rats in all groups were fed for 7 days with regular rat chow. After fasting for 12 hours, 14C-labeled fat emulsion was infused to the JE and CE, and 14C-labeled unemulsified fat to the JU and CU groups through a gastrostomy for the absorption test. The hourly and cumulative output of 14CO2 by respiration, absorption rate of 14C-labeled fat in the intestine, and metabolic oxidation rate of the absorbed fat were determined during an 8-hour period after the gastroenteral administration of emulsified or unemulsified fat. The peak of hourly output was seen after the first 2 hours in the CE, JE, and CU groups, following which a remarkable decline was seen in the CE and CU groups. However, a more gentle descent in the JE, and fluctuation at a low level in the JU group were observed. The cumulative output in the JE was 61% of that in the CE, while the output in the JU was 16% of that in the CU group. The absorption rate in the JE was 81% of that in the CE group, while the rate in the JU was 22% of that in the Cu group.(ABSTRACT TRUNCATED AT 250 WORDS)

Effects of pectin and cellulose on fat absorption after massive small-bowel resection in weanling rats.

The effects of pectin and cellulose on fat absorption after massive small-bowel resection were studied in rats. Weanling Wistar rats underwent 80% resection of the small intestine with gastrostomy, and fat emulsion labeled with 14C-linoleate was given via a gastrostomy tube for the fat absorption test on the 10th postoperative day. They were classified into three groups by diet as follows: E group, a fiber-free, chemically defined diet (CDD); EP group, CDD with 2% pectin; and EC group, CDD with 2% cellulose. The cumulative and hourly output of 14C in the expired air, the absorption rate of 14C in the intestine, and the metabolic utilization rate of 14C were calculated. The cumulative 14CO2 output was significantly lower in the EC group than in the E and EP groups. However, there was no significant difference in the 14CO2 output between the E and EP groups. The highest hourly 14CO2 output in the E and EP groups was observed during the first 2 hours. However, in the EC group, this fluctuated at low levels. No significant difference in the 1-14C-linoleate absorption rate was observed between the E and EP groups, whereas it was significantly lower in the EC group than in the E and EP groups. The metabolic utilization rate of 1-14C-linoleate showed no significant difference among the three groups. These data suggest that an addition of pectin to CDD does not impair small intestinal absorption of 1-14C-linoleate after massive small-bowel resection, and an addition of cellulose decreases absorption of 1-14C-linoleate.

Frantz's tumor: a papillary and cystic tumor of the pancreas in girls.

Four girls with Frantz's tumor, a papillary and cystic tumor of the pancreas, are studied and discussed in comparison with 112 cases in the literature, including 58 Japanese cases. The neoplasms occur predominantly in girls and young women. Up to recently, the tumors have possibly been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma according to microscopic findings and frequently have been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces show characteristically solid and hemorrhagic-necrotic patterns. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low-grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. Immunochemical and electron microscopic studies can differentiate Frantz's tumor from other neoplasms and also suggest that the tumors originate from primordial cells or multipotential stem cells capable of differentiating into both exocrine and endocrine lines.

Involvement of endogenous nitric oxide and c-kit-expressing cells in chronic intestinal pseudo-obstruction.

BACKGROUND/PURPOSE: Chronic intestinal pseudo-obstruction (CIP) in infants and children is a motility disorder without apparent mechanical cause. Nitric oxide (NO), an inhibitory neurotransmitter and c-kit cells, essential for the intestinal pacemaker activity, both play a key role in the intestinal motility function. In the current study, the authors investigated the distributive change in the intestinal nitric oxide synthase (NOS) and c-kit cells of patients with CIP. METHODS: Tissues were obtained from 4 patients undergoing bowel resection or biopsy for CIP at laparotomy. For controls, the intestinal specimens were obtained from 4 age-matched cases of intestinal stricture, intussusception, and autopsy with no evidence of gastrointestinal disease. Immunohistochemical studies were performed on paraffin-embedded tissue cross sections with neuronal NOS and inducible NOS monoclonal antibody as well as a rabbit polyclonal antibody against the human c-kit receptor. RESULTS: Under immunohistochemical staining, a greatly increased density of neuronal NOS immunoreactivity and an evidently increased number of intense NOS immunoreactive nerve fibers were observed in the myenteric plexus and circular muscle layers compared with the control sections. In the submucosal plexus and longitudinal muscle layer, there was no change in NOS immunoreactivity. Inducible NOS immunoreactivity was not detected in the control cases. However, in tissues of CIP, almost all the epithelial cells were positively and strongly labeled for inducible NOS immunoreactivity. For c-kit cells staining, the number of c-kit-positive cells in the myenteric plexus and circular muscle layers were greatly less than that in the controls, especially in the myenteric plexus region. CONCLUSION: These findings suggest that sustained production of NO by an increased NOS activity and a deficiency of c-kit cells in the intestine may be related to the pathogenesis of CIP.

Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum.

A case of acute necrotizing pancreatitis in association with choledochal cyst is presented. Pancreatitis associated with choledochal cyst is probably caused by a biliary reflux into the pancreatic duct via a pancreatobiliary malunion, as the intraductal pressure of the cyst exceeds that of the pancreatic duct. Ampullar stenosis due to gallstones or inflammatory changes may increase the intraductal pressure. Bile with activated pancreatic enzymes refluxes into the pancreatic duct, and possibly results in acute pancreatitis. However, patients with choledochal cyst presenting with recurrent bouts of abdominal pain, vomiting, and fever have often been diagnosed as having acute pancreatitis because of hyperamylasemia, despite no evidence of pancreatitis at the time of surgery. At the time of bouts, they also show a slight elevation of serum bilirubin, and an increase in the degree of the choledochal dilatation that are possibly caused by biliary obstruction, not ampullar obstruction, due to suppurative cholangitis. The term "fictitious pancreatitis" or "pseudopancreatitis" in choledochal cyst appears to be appropriate. This clinical study shows that amylase in the biliary tract has ready access to the blood stream, probably through a sinusoidal pathway by cholangiovenous reflux, and a lymphatic pathway, via the Disse's space and denuded cyst wall, provided the biliary ductal pressure is increased.

The beneficial effect of tamoxifen therapy in patients with resected adenocarcinoma of the pancreas.

BACKGROUND/AIMS: Hormone therapy by tamoxifen was performed on patients with adenocarcinoma of the pancreas and the effect of tamoxifen on their survival rate was evaluated in patients with resected pancreatic adenocarcinoma (n = 65). MATERIAL AND METHODS: Tamoxifen was administered once daily (at 20 mg) in addition to immunochemotherapy (Tegaful, Mitomycin, Krestin or OK-432). RESULTS: A remarkably beneficial effect of tamoxifen was found when compared with non-tamoxifen group (p < 0.01). When the location of the pancreatic carcinoma was considered, the survival rate of the tamoxifen group with adenocarcinoma in the head and in also the body/tail of the pancreas revealed the same statistically significant difference (p < 0.01) individually. Regarding curability by surgery, the beneficial effect of tamoxifen was observed even in the survival rate of patients who had received noncurative surgery. Furthermore, the remarkable effect of tamoxifen was revealed in the male group, whose one-year survival rate for resected carcinoma in the head of the pancreas was 85.6% (n = 16) as when compared to 19.1% of non-tamoxifen group (n = 15) and also in the female group whose one-year survival rate was 53.8% (n = 1.3) compared with 21.4% of non-tamoxifen group (n = 7) (p < 0.01). CONCLUSION: With these data, we can see that the adjuvant therapy of tamoxifen in carcinoma of the pancreas confers a significant benefit to those patients who have received a surgical resection and reduction in the volume of the carcinoma in the pancreas.

Ischemic jejunal stricture developing after diabetic coma in a girl: a case report.

A 15-year-old girl with ischemic stricture of the jejunum, probably due to microangiopathy caused by diabetes mellitus is presented. Laparotomy revealed two circular strictures in the proximal jejunum, and histology showed ischemic enteritis with ulceration, granulation with microvascular proliferation, inflammatory cell infiltration, marked fibrosis, and hemosiderin deposition. Uncontrolled diabetes mellitus often causes ketoacidosis, increased blood viscosity and platelet aggregation, dehydration in addition to hyperglycemia, and occasionally results in occlusive or non-occlusive vascular disease of the intestine. Ischemic stricture of the bowel in the diabetic state is probably related to moderate ischemia resulting in ulceration and scar formation in the intestine.

Carcinoma of the colon in childhood; report of 2 cases expressing p53 without K-ras mutation.

We report on 2 children with colonic carcinoma and also review 62 cases of Japanese children with colonic carcinoma including ours. Although the dismal prognosis in colonic cancer in children is possibly due to the predominance of poorly differentiated carcinoma, there is no significant difference in the 5-year survival rates among well, moderately and poorly differentiated carcinomas in children. Positive staining with p53 in tumor cells was observed in each, but K-ras mutations were not detected in any. Therefore, these carcinomas possibly developed from de-novo carcinoma. The development pathway of colonic carcinoma may relate to the prognosis in children, and be different from that in adults.

Successful left hepatectomy for hepatocellular carcinoma in a child after transcatheter arterial chemoembolization: report of a survival.

A 12-year-old boy with large hepatocellular carcinoma is presented. The tumor was successfully resected after reducing the tumor size by transcatheter arterial embolization with Lipiodor containing an antitumor drug. The patient has achieved a 2-year-10-month disease-free survival.

[Cancer arising in choledochal cyst and management].

Biliary cancer develops in 20-30% of the patients with choledochal cyst and pancreatobiliary malunion. Some bile acid fractions and refluxed pancreatic enxymes into the bile duct is probably responsible for carcinogenesis. Cancer often develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic duct. In cystic dilatation, cancer often occurs in the common bile duct, while in diffuse or non-dilated type it occurs in the gallbladder. Cancer usually occurs in younger patients than does biliary cancer in general population, and the average age is in the 40s. The risk of malignancy in cysts with internal drainage is higher than that in primary cysts, and early removal of the retained cyst should be performed as quickly as possible. Although the prognosis of biliary cancer is usually dismal, aggressive procedures are recently gaining better results than that by conventional methods. The prevention of cancer is the procedure of choice by early excision. Removal of the whole extrahepatic bile duct is necessary, even in case of malunion with no biliary dilatation. Cancer rarely arises in the intrahepatic duct after excisional surgery, due to long standing biliary stricture. Wide anastomosis with ductoplasty should be essential. Cancer also occurs in the remnant duct. Excision of the distal duct in the pancreas is also necessary.

[Total parenteral nutrition in surgical treatment of infants: recent progress and problems].

The frequency of TPN in surgical infants was 242/686 (37.8%) in neonates and 260/2693 (9.7%) in older infants for the past 15 years. The frequent indications of TPN were intestinal atresia, Hirschsprung's disease and esophageal atresia in the neonatal period, and Hirschsprung's disease, pyloric stenosis and biliary atresia in infancy. The effectiveness of TPN was impressively indicated by better survival rate in the infants with massive intestinal resection received TPN. An amino acid solution (N1-2) was newly devised, based on the analysis of plasma aminograms in 36 infants received TPN with a commercial amino acid solution (Proteamin), and theoretical considerations. It was more useful solution for TPN in 12 young infants. Urinary phosphorus was a considerable parameter for the administration of Vitamin D in TPN. The incidence of TPN-induced hepatic dysfunction was significantly referred to the amount of amino acid in TPN. Closed infusion system with a soft bag and triple bacterial filters in the line was effective for the prevention of sepsis caused by central venous catheter. The nutritional care was important in the treatment of infants with biliary atresia, because generally they had some nutritional defects such as essential fatty acid deficiency, insufficient amino acid metabolism and zinc deficiency. The nutritional care in infants with advanced neuroblastoma favorably altered the course of the disease.

Effect of fat supplementation on the maintenance of gut integrity in elemental diet-fed rats.

AIMS: The aims of this study were to investigate the mechanism of atrophic change in ileal mucosa supplied with an elemental diet (ED) and to assess the value of supplemented fat emulsion in the prevention of atrophic change. MATERIALS AND METHODS: In experiment 1, 25 male Wistar rats with a body weight of 160-180 g were divided into 3 groups. The first group was fed regular rat chow (Control group, n=5). The second group was given ED containing 0.6% long-chain triglycerides (ED group, n=10). The third group was provided with fat-enriched ED (FED) containing 3.5% long-chain triglycerides (FED group, n=10). Each group received an isocaloric diet (300 kcal/kg/day). 4 weeks later, after euthanization, ileal samples were taken for light and electron microscopic examinations. The morphological changes of the intestinal mucosa and the crypt cell proliferation rate (CCPR) were determined. In experiment 2, to determine the site of fat absorption, 9 rats were fed ED for 1 week. After 24 h of food deprivation, all rats were given 2 ml of FED through a gastric tube. Then 1, 2, and 3 h(s) later, groups of 3 rats each were euthanized, and the total small intestine was obtained from each rat. The proximal and distal jejunum and distal ileum were stained with oil red O. RESULTS: In experiment 1, the samples had almost the same morphological appearance irrespective of the type of feeding. The CCPR was significantly diminished in the ED group compared with the Control group, while there was no statistical difference between the FED and Control groups. In experiment 2, the oil red O stain was positive in the proximal and distal jejunum, but was completely negative in the distal ileum. CONCLUSIONS: The introduction of ED does not soon result in an atrophic morphological change of the ileum but will decrease the CCPR. An additive fat emulsion which was rapidly absorbed by the distal jejunum could play a role in maintaining ileal mucosa integrity through some mechanism independent of absorption.