IgA is an immunoglogulin which is considered to have a responsibility in local immunity and is excreted into the bile. We measured the IgA level in the serum in postoperative patients with biliary atresia, which was extremely low from the birth to 6 months of age, and then increased rapidly from 7 months to 11 months of age. In our experiences, postoperative ascending cholangitis was most frequently occured in the period between 4 months to 6 months of age and was not occured from 7 months to 11 months of age. Furthermore secretory IgA increased proportionally in bile as serum SIgA increases. These results suggest that secretory IgA in bile plays an important role to protect the ascending cholangitis in postoperative biliary atresia patients.
Cytotoxic T lymphocytes (CTL) against syngeneic A/J mouse neuroblastoma (C-1300) were induced in vitro primary or secondary culture. Its specificity as CTL was characterized by treatment with monoclonal anti-Thy 1.2 or anti-Lyt 2.2 antibody and complement. CTL specific for syngeneic neuroblastoma C-1300 cells definitely cross reacted with human neuroblastoma cells (NB 1, NBGOTO) across a species barrier. In next experiment, using mixed lymphocyte tumor cell culture by 51^Cr release assays for 14 patients with various types of pediatric malignant solid tumor, the following results were obtaind. In peripheral blood lymphocytes of post operative neuroblastoma patients, CTL were induced in vitro against allogeneic neuroblastoma cell (NBGOTO), and in some of them, specific cytotoxic antivity for C-1300 were noticed. But no CTL could be detected from patients of preoperative, during therapy periods and with other malignant tumors. Surprisingly, in patients with Stage IV-S neuroblastoma, high level of CTL was appeared in each time, even in preoperative period. This result indicates that Stage IV-S neuroblastoma is associated with high levels of immunological surveillance. The conclusion is that a clearly cross activity exists in T cell mediated cytotoxic reaction between the mouse and human neuroblastoma without recognizing both MHC determinants.
The effect of myocardial protective method on the post operative hemodynamic was studied in two groups of patients with Tetralogy of Fallot (TOF) who had a radical operation. Twenty Four TOF patients were divided into two groups based on Crystalloid Cardioplegia (group A) or non Crystalloid Cardioplegia (group B). The Crystalloid Cardioplegic Solution was made of 500ml of 5% glucose to which we added 10 mEq of sodium bicarbonate and 10 mEq of potassium chlorid. This solution was kept at 4 to 6℃. The pH of this solution was 8.322, and the milliosmolarity was 327. At 3 hrs after operation, LVWI showed higher Cardiac Pump-Function in the group A than group B. It was not different for the postoperative pleural effusion between the two groups in those cases with a patch size of 10cm^2/m^2 or larger, however the incidence of postoperative pleural effusion was somewhat smaller for those cases with a patch size of less than 10cm^2/m^2, particularly so in the cardioplegic solution group. The mortality rate in A group was 7.6%, and in B group 18.1%.
Since 1978, 125 children of post-operative intestinal obstruction have been treated in our institute. Of these 125, 106 patients have initially undergone hyperbalic therapy. Eighty-five patients recovered but 21 required operation. In the operated patients, 13 were with stangulated, twisted or intussuscepted intestinal obstruction, but all were not severe. The recovery of obstruction was seeen in 80% of patients by hyperbalic therapy. 19 of 125 cases was very mild or extremely ill and with possible strangulation at arrival. They were not indicated for hyperbalic therapy. The reccurence of intestinal obstruction was seen in 26% by conservative therapy and 36% by operative therapy. It is possible that an operation for intestinal obstruction will provide another intestinal obstruction. By this reason, post-operative intestinal obstruction should be initially treated by a conservative therapy as much as possible. A hyperbalic therapy is helpful for conservative therapy.
This study was designed & purposed to examine whether the mechanism of bile secretion is normal or not after hepatic portoenterostomy for biliary atresia. Seven patients who have undergone a Suruga II portoenterostomy were studied. In five of six patients, bile volume (Y) showed a correlation with bile salt excretion (X) (Y=0.03-0.167x+0.01-0.08, R=0.648-0.892), and Y intercrept were always positive (0.01-0.08 ml/min.). This evidence suggested a presence of both bile salt dependant and independant bile secretion machanism. Bilirubin excretion showed no correlation with bile salt excretion (R=0.357), but cholesterol exretion (Y) showed a correlative relationship with bile salt excretion (X). (Y=0.014x+0.07, n=49, R=0.801) Phospholipid (Y) had a correlation with bile salt excretion (X). (Y=0.092x+0.012, n=48, R=0.952) The results suggest that the mechanism of bile secretion is almost normal after hepatic portoenterostomy for biliary atresia.
Postoperative course of twenty-six patients with biliary atresia were investigated to analyse the cause of prolonged jaundice. The following results were obtained. 1) Four types of clinical course after surgery were observed. In type 1, bile excretion is good and the amount of excreted bilirubin is usually exceeded that of bilirubin production. Serum bilirubin level quickly falls down to the normal level. In type 2, comparatively slow recovery of serum bilirubin is observed. In spite of the small amount of bilious fluid, the concentration of bilirubin is usually high. Bilirubin excretion gradually increases in this group. In type 3, the amount of excreted bilious fluid is adequate, but bilirubin concentration is very low. Serum bilirubin level does not decrease to normal level. In type 4, the amount of bile excretion from fistula is very low, even after re-operation. It is suggested that prolonged jaundice is related to the amount of the excreted bilirubin. 2) Life span of the red blood cell was short in two patients with prolonged jaundice althogh there was a good excretion of bile and bilirubin. It is assumed that the function of red blood cell is related to prolonged jaundice. 3) Proper use of cholagogues is recommended for the purpose to reduce the number of patients with prolonged jaundice.
Histopathological study of the intrahepatic bile ducts (IHBD) was performed, using the wedge liver biopsy taken at hepatic portoenterostomy in 60 patients of biliary atresia. First, total area of all intrahepatic bile ductuli (B), and total area of all fibrotic area (F) in one field were measured by image processing system. B/F ratio was lower in patients with good postoperative bile flow (≧100ml/day), compared to patients with poor bile flow (<100ml/day), (p<0.05). B/F ratio became higher in patients with mild fibrosis (p<O.05). Secondly, patients were divided into three groups, according to the degree of degenerative changes of IHBDs. In group I (13 cases), degenerative change was minimal and no bile plug was identified. In group II (27 cases), degeneration was moderate and bile plugs were found in less than 10% of the total bile ducts. In group III (20 cases), degeneration was severe, and bile plugs were found in more than 10% of the total bile ducts. Degenerative change of the IHBDs shows parallel correlation to the age at operation (p<0.01) and the degree of liver fibrosis (p<0.05). Whereas, grading of degenerative changes of the IHBDs shows no correlation to the diameters of extrahepatic bile ducts at the porta hepatis. The present study suggested that the degenerative change of IHBDs is one of the most important factors, concerning the prognosis in biliary atresia patients.
In type B esophageal atresia, presence of proximal pouch fistula is sometimes difficult to to diagnose in the earliest stage of life. Wide gap between upper and lower segment presents the main therapeutic problem. This paper describes successful treatment of a case by delayed primary anastomosis after bougienage of the upper and lower pouch and closure of tracheoesophageal fistula. A male newborn with a birth weight of 2,335 g was diagnosed to have esophageal atresia. Proximal fistula was not diagnosed by Dionosil swallow performed on admission. Following gastrostomy which was done on the second day of life, proximal and distal esophageal bougienage was commenced. The patient had been well until two and a half months when there was sudden onset of cyanosis. Proximal pouch fistula was revealed at the level of the first thoracic vertebra by Dionosil swallow. The gap between proximal and distal pouch of the esophagus showed a length of six and a half vertebrae. The upper tracheoesophageal fistula was closed transcervically. The esophageal bougienage was continued untill four and a half months when both ends met together. Finally, esophageal anastimosis with circular myotomy was performed through extrapleural approach. Postoperative course was uneventful.
During a period from 1981 to 1983, three infants with intractable hypoglycemia have undergone an extensive pancreatic resection for nesidioblastosis at the Kobe Children's Hospital. In two patients, the initial extensive resection of the pancreas (85-90%) was successful for the management of hypoglycemia. However, one patient required two additional resections of the pancreas for persistent hypoglycemia. At the third operation, total pancreatic tissue was removed, which was successful to stabilize the blood sugar level. The experience in these three patients has provided the following conclusions; (1) An extensive resection of the pancreas (90%) should be carried out at the initial operation. (2) The postoperative cource should be carefully monitored by frequent measurement of blood sugar level. (3) When hypoglycemia is persistent, a total pancreatectomy should be carried out.
A palliative relief of cholestasis was successfully achieved by a continuous PTCD in two patients of biliary atresia who had undergone an unsuccessful Kasai procedure. A temporary impovement in liver functions was observed by decompression of iatrahepatic biliary system. Irrigation of the infected bile ducts was effective for management of cholangitis. Determinations of the antibiotic levels in the bile have provided a fact that a damaged liver does not transfer the systemically given antibiotics into the bile. These two patients finally died of progressive liver damage. However, a continuous PTCD seemed to be useful for preparation of future liver transplantation in such patients.
Two cases of Menkes' kinky hair disease having urologic complications were herein reported. The first case was diagnosed Menkes' kinky hair disease at 6-month old. He noted multiple bladder diverticula, right vesicoureteral reflux and right hydronephrosis at 4-year-9-manth old. The cause of these urologic complications was thought to be a consequence of an increasing disturbance in innervation of the bladder. The other case performed the left nephrectomy at 26-day old at birth, because of massive bleeding from left kidney. He was diagnosed Menkes' kinky hair disease at three months after the operation. The cause of bleeding was thought to be the characteristic disorder of vessels which frequently noticed on the Menkes' kinky hair disease. To authors' knowlege, as this renal complication of Menkes' kinky hair disease, it has not been reported in the literature.
3 years old boy, with a huge mesenteric lipoma is reported. He was well developed and nourished boy with chief complaint of abnominal mass. A smooth surface, elastic soft, movable rounded mass was palpated in the abdomen. His labolatory examination and special tumor markers examination showed no abnormalities. Small intestine was shifted right upper side of abdomen in the abdominal plain film. DIP showed no abnormal findings. Echogram showed a huge mass with homogenous density. CT scan showed an encapsulated, soft tissue density mass in the abdomen, which density was as same as that of fatty tissue. At laparotomy, a huge fatty tumor was found on the 150 cm anal site of mesojejunum from the ligament of treitz. The tumor was well defined and not adhered to the surrounding tissues, and was removed without intestinal resection. The resected tumor showed a smooth, capsulated, elastic soft mass, measuring 23×21×13 cms in size, l,073gms in weight. Histological examination showed a benign mesenteric lipoma. The postoperative course was uneventfull. Mesenteric lipoma is rare. In 1963, Nomura, et al. reported 236 cases of mesenteric tumor in Japan, and found only 5 lipomas. Since then, only scattered case reports have been published in Japan. Mesenteric tumors have been divided into solid and cystic varieties. The mesenteric cysts are all benign. The solid tumors are usually malignant sarcoma. Surgical excision in the treatment of choice, since mesentric lipomas of considerable size may undergo malignant development.
The first reported example of hepatoblastoma developing in a 12-year-old girl with glycogen storage disease type I is presented. The clinical course, the histology of the resected specimen and the results of biochemical analysis of the tumor are described. We also refer to possible mechanism of association of glycogen storage disease type 1 and malignant tumors of the liver.
This paper describes a rare case of intussusception in a three-day-old female with melena and bilous vomiting. The abdomen was slightly distended. A mass was palpable in the left abdomen. The abdominal plain film demonstrated dilatated loops of bowel at the center of the abdomen. The barium enema examination revealed a filling defect ranging from the descending colon to the rectum. No barium passed into the transverse colon. The operative finding presented an ileo-colic intussusception. An attempt of manual reduction resulted in perforation of the transverse and sigmoid colon. At last a right hemicolectomy and an end-to-end anastomosis were performed. A tube-sigmoidostomy was done. The pathological examination of the surgical specimen revealed a 2 cm saccular duplication of the terminal ileum. On the 1st postoperative day, the infant became septic, but she recovered by the exchange transfusion and the other intensive medical therapy. The following course was uneventful.
A 7-year-old girl with an immature teratoma of the right ovary combined with a mature teratoma of the left ovary, whose serum alpha-fetoprotein (AFP) was negative, underwent salpingo-oophorectomy for the right tumor and enucleation of the left. Four months later her serum AFP was positive, without apparent clinical symptoms and signs. Nine months after initial operation, a large retroperitoneal tumor developed with elevated serum AFP of 1,600 ng/ml. A subtotal resection of the recurrent tumor was performed, followed by VACA (VCR, AMD, CPM, ADM) chemotherapy, with decrease and disapearance of serum AFP. A microscopic reexamination of the primary and recurrent tumors revealed almost the same findings composed of moderate quantities of immature neural tissue, moderately differentiated endodermal tissue, and small foci of immature endodermal components, combined with localized coagulation necrosis. Tumor cells show the atypicality and mitotic activity corresponding to Grade 2 (Thurlbeck & Scully) immature teratoma. Components of yolk sac tumor or embryonal carcinoma could not be found. AFP was identified by an immunoperqxidase method, which was strongly positive in the cytoplasma of the cells of the immature and moderately differentiated endodermal components, and faintly positive in the immature neural tissue. Despite of the same appearance of the AFP localization and intensity in both specimens, it is unclear why serum AFP was negative in the primary tumor, whereas the serum AFP level was high in the recurrent tumor. Now, 2 years and 10 months after reoperation, she is in a state of disease free.
Chronic granulomatous disease (CGD) is an inhelited illness in which phagocytic cells are unable to kill ingested bacteria. Hepatic abscess is the most commonly reported gastrointestinal complication in these patients. Experience with 4 hepatic abcesses developed in 3 patients with CGD was reviewed. In each case, the diagnosis was confirmed by a negative nitro blue tetrazolium test and by total failure of chemiluminescence after phagocytosis. Operative hepatic debridement and external drainage combined with intravenous antibiotics were curative in every case, but operative morbidity was frequent and severe. Three major postoperative complications included wound disruption with delayed healing, prolonged febrile course and massive hepatic bleeding. One patient received granulocyte transfusions for over 10 days in the pre- and post-operative periods. By the 12th day of therapy, she had become afebrile. For hepatic abscess in CGD patients, drainage must be carried out in an excisional fashion. If the abscess is at periphery of the lobe, partial hepatic lobectomy is the treatment of choice. If placed more centrally within the parenchyma of the liver, operative thepapy should consist of hepatotomy, evacuation of purulent exudate and total granulomatous material and external drainage. Granulocyte transfusion as adjunctive therapy seems to have a significant benefit in CGD patients with serious infection.
A 12-year-old boy received a blunt force in the right upper abdomen with a mop resulting in abdominal pain and vomiting. The upper gastrointestinal series showed an obstruction at the second portion of the duodenum, suggesting traumatic intramural duodenal hematoma. He was treated by continuous nasogastric suction and intravenous fluids since there was no associated bowel perforation nor other intraabdominal damage. On the twenty forth day after injury, the second upper gastrointestinal series showed the slow passage of the barium on the left side upper most, and therefore the oral feeding was started. Unevenfully he was discharged on the thirty fifth day after injury. A review 77 cases described in the literature was presented. This condition is most prevalent in 6 or 7-year-old boys. Onset of symptoms varied from one hour to 14 days, the average being 2 days. Unless clinical signs demand surgical intervention, a conservative approach is instituted, and a surgical intervention is not required in the majority of patients. The average interval of conservative theraphy was 9 days.