Serum and urinary amino acid patterns in biliary atresia were studied. The patients were divided into 4 groups as follows. Group 1. Nine cases of biliary atresia, before operation. Group 2. Seven cases of biliary atresia, 1 to 3 months after successful operation. Group 3. Seven cases of biliary atresia, 2 to 5 years after operation without jaundice. Group 4. Seven cases of biliary atresia, more than 5 years after operation. Following results were obtained. 1) In biliary atresia (Group 1), glutamic acid, phenylalanine, aspartic acid and histidine significantly increased, while threonine, valine, tryptophan, alanine and cystine decreased. 2) In comparison of patterns of serum amino acids in each group, glutamic acid, proline, threonine, tyrosine, lysin, isoleucine showed the tendency of decreasing as time relapsed after operation, while arginine and tryptophan showed the tendency of increasing. There was no relation between the patterns of amino acids of serum and those of urine. 3) In Group 4, abnormal increase in arginine and glycine, and abnormal decrease in proline were observed. 4) The ratio of branched chain amino acids to aromatic amino acids (normal : 2.9) was 2.16 in Group 1, 1.97 in Group 2, 2.4 in Group 3, and 2.7 in Group 4. These results suggested that the regulation mechanism of the amino acids in the liver in biliary atresia gradually recoveres after successful operation.
The liver plays a main role in the metabolism and storage of nutrients. A deficiency of nutrients, therefore, is often associated with liver diseases and may be caused by decreased storaged, abnormalities in metabolism or increased requirements for nutrients. It should be corrected by means of parenteral nutrition when enteral feeding is not available, especially in young infants who have minimal storage and maximal requirements for nutrients. For the purpose to establish an optimal parenteral nutrition in young infants with hepatic disorders, experimental studies have been performed. Ten obstructive jaundiced weanling rats with mild hepatic dysfunction have been fed intravenously for seven days. The two I. V. feeding regimens used in the series consisted of glucose and commercially available L-synthetic amino acids PROTEAMIN (E/N : 1.0, FAO/WHO : , 1963) or newly deviced L-synthetic amino acids FAH-01, made for the subjects. These two regimens provided 350 Cal/kg/day that was considered as "full-strength" by the preliminary studies using ten weanling rats with normal liver function. Although positive nitrogen balances were achieved in both groups of rats, all of the rats fed PROTEAMIN regimen markedly lost their weight and fairly well weight gains were shown in the other group of rats fed with FAH-01 regimen. The serum aminograms of the rats fed with PROTEAMIN regimen indicated marked elevation of His., Thr., and Pro., whereas the minimal abnormalities were detected in the serum aminograms in the rats fed with FAH-01 regimen. These results suggested that the composition of the amino acid solutions is very important to perform the optimal parenteral nutrition for the young infants with mild hepatic dysfunction.
The purpose of this paper is to describe the congenital abnormal junction of the common bile and pancreatic ducts which caused the infantile obstructive cholangiopathy. Studies were done on both normal junction of the common bile and pancreatic ducts and the influence of the pancreatic juice refluxed into the biliary system. A histopathological investigation on the junction of the common bile and pancreatic ducts was performed in 67 autopsy cases, all of these had no hepato-biliary and pancreatic lesion. The normal junction is existing within or/and under the mucosa of the papilla of Vater and the average length of the common channel of the common bile and pancreatic ducts is 1.06 mm. It seems that the abnormal junction of the common bile and pancreatic ducts is existing at exterior to the tunica muscularis propria of the duodenum. In addition, the length of the common channel is extremely long. If there is the anatomical abnormality, namely the abnormal junction of the common bile and pancreatic ducts, the pancreatic juice refluxes easily into the biliary system and then if the bile stasis by the stenosis of the common bile duct exists, the refluxed pancreatic juice causes a histopathological changes in the biliary system. This study suggests that the existence of the stenosis of the biliary system and of the common bile and pancreatic ducts is essential for the origin of the dilatation of common bile duct.
Since 1969 to December 1979, we have treated 69 cases of biliary atresia. Sixteen cases of them have lived over 2 years of age. The analysis by inquirement for mental and physical developments were done in these 16 and one who died at six years of age at home. The percutaneous transhepatic cholangiography (PTC), serum biochemical examination for liver function test and ICG clearance test were performed in fifteen cases at two to nine years of age. From these results, it was found that cases survived more than two years had no retardation of their mental and physical developments. And, ICG clearance test was better parameter for evaluation of liver function when compared to that of serum biochemical examination. On cholangiographic studies, in all 9 cases in whom PTC was successful, partial narrowing, dilatation of the intrahepatic bile duct and the type B cholangiogram, i. e., proliferated bile duct bundle type as classified by us in prevenous report, were observed. Two cases showed partial segmental atrophy of the liver. Therefore, we conclude that cases with biliary atresia having survived long period still show unusual features of the intrahepatic bile duct indicating that continuous efforts for preventing complications such as infection should be made.
I. Effort has been made to save the spleen in four children, aged 5 to 14 years, with blunt abdominal trauma during the past two years. Three spleens out of the four were affected by a transverse laceration, and the other by a vertical one. All four spleens were well preserved by suture repair of the laceration. The clinical course and the surgical procedure of the splenic repair as of the case with the most extensive splenic laceration were reported. II. Four different types of splenic wounds were created in mongrel dogs to study the effects of suture repair on healing process. Transverse lacerations healed easily within a few postoperative weeks. Even a large vertical laceration, which seemed to be anatomically the most difficult type to heal, could be repaired by a simple suture maneuver. The radioisotope scan showed almost normal uptake of the repaired spleens, and the histological examination revealed satisfactory healing a month postoperatively. As a conclusion, it could be said that the suture repair is an usuful method in preserving the ruptured spleen in the event of an accidental injury, and that every effort should be made to preserve the spleen after traumatic rupture.
During these 12 years, congenital choledochal dilatation has been experienced in 45 children. Clinical analysis was made as related to symptoms such as abdominal pain, palpable mass, jaundice and etc.. In all of the 12 patients examined, an anomalous junction of the pancreaticobiliary ductal system was demonstrated. Results : (1) There were statistical relationships between abdominal pain or vomiting, and elevated serum amylase level. (2) The patients presenting abdominal pain often tended to show high amylase level in bile. These evidences suggest that in these patients, there occurs the reflux of the bile into the pancreatic duct and the pancreatic juice into the bile duct. It is probable that abdominal pain often accompanied with congenital choledochal dilatation is due to pancreatitis.
Content of acetylcholine and muscarinic cholinergic receptor in the aganglionic colon was measured in order to know the pathophysiology of the aganglionic segment in Hirschspruns's disease. Acetylcholine content was measured by pyrolysis gas chromatography, which was found to be a simple, accurate and reliable method. Microwave irradiation on the surgically resected specimens was performed before extraction procedure to abolish acetylcholinesterase and cholineacetyltransferase activity in the tissue. Acetylcholine content in the ganglionic colon was 8.15±3.15 n mol/g (n=15) in contrast to 23.79±14.17 n mol/g (n=19) in the aganglionic segment of Hirschsprung's disease. Muscarinic cholinergic receptor was assayed by measuring specific binding activity of ^3H-quinuclidinyl benzilate (QNB), which is a potent muscarinic antagonist, to receptor site. No significant difference was seen in the amount of muscarinic receptor between aganglionic segment and normal colon. Therefore, it is reasonable to think that the neuro-effector sites exist even in the aganglionic segment biochemically and the elevated acetylcholine may act on the smooth muscle of aganglionic colon.
For the purpose of recognizing the functional development of intestinal motility during foetal life, the change of cholinergic activity was studied biochemically in rabbit foetuses. The esophagus, stomach, small intestine and colon were removed from rabbit foetuses in 3 or 4 days' interval, for measuring the ChAT and AchE activities. The ChAT activity of small intestine was significantly higher in the 28th day comparing with those of the 18th and 21st day. The activity of ChAT and AchE of esophagus, stomach and colon were not significantly increased during foetal development. In comparison of the activity of ChAT and AchE for the location of alimentary tract, the activities of ChAT and AchE were higher in colon than any other location. There was positive correlation between the ChAT and AchE activity. The similarity in the developmental pattern between ChAT and AchE activity suggests the co-existence of Acethylcholine itself in those foetal intestines.
Stricture at the esophageal anastomosis is common in children and usually managed by retrograde dilatation with a bougie. But some stricture may resist dilatation by bouginage. We have performed the cutting method using an endoscopy (fiberscope) for two strictures that did not respond to dilatation. Cutting the stricture site was performed by the electric current of high frequency. The both patients have improved clinically and radiologically. The indication of endoscopic cutting method is restricted in children because of the small size and the thin wall of the esophagus. However with proper technique, the procedure is thought to be usefull for the esophageal stricture which can not be treated by usual dilatation.
Prolapse of the excessive rectal mucosa is one of the most common complications following rectoplasty for anorectal malformation in children. In such cases, a sliding skin graft as described by Carmel has added much to the improvement of results. In annular prolapse of the rectal mucosa, however, the sliding skin graft is either impractical or impossible in order to remove all of the pathologic condition and restore an anatomically correct anus. In six such exceptional patients, we have successfully used a circumferential sliding skin graft without any complication except for occasional scar formation. Use of circumferential sliding skin graft is a simple technique as follows. After removal of all pathologic condition, Whitehead's rectoplasty is done and four Carmel's sliding skin grafts are employed surrounding the restored anus. Because of the sequellae such as scar formation, this operation is, however, not recommeded as routine for annular prolapse of the rectal mucosa. The patients, in whom the mucocutaneous suture line moves into full depth of the anal canal at Whitehead's procedure, may be contraindicated for this operation.
In thirty cases, re-closure of VSD was done at our clinic. The purpose of this report is to evaluate the re-closure of VSD for improvement of the cardiac status with minor surgical risk. Re-closure of VSD was undertaken in the period from 5 hours to 18 years after the first operation (mean 4 years and 7 months). The patients were 17 males and 13 females. According to the classification of VSD by Kirklin, the original type of residual VSD were 7 in type 1, 17 in type 11 and 4 in type 111. One showed complete conus defect and another one had multiple VSD. The operative finding at the secondary closure of VSD showed that the primary cause of the development of leakage in type 1 was due to tissure disruption at the site of suture closure without patch. In type 11 VSD, the main location of disruption of the patch was the annulus of the aortic valve in 5 and at the inferior margin of VSD in 5. In residual VSDs in type 111, the inferior margin was identified at the major point of leakage. Operative procedure for re-closure of VSD was closure of VSD with a patch in 15 and suture closure without a patch in 13. One case needed concomitant tricuspid valve replacement with the Hancock prothesis and permanent pacemaker implantation and another one tricuspid annuloplasty beside re-closure of VSD using the Carpentier's ring to control a significant tricuspid regurgitation. The operative results showed that there were one table death (mortality rate 3.3%) and 25 cases in satisfactory with complete closure of VSD (83.3%). However, two cases showed minor residual VSD even after re-closure of VSD. One remained minor aortic regurgitation and the other needed strict medical treatment for chronic congestive heart failure due to large left to right shunt. Postoperative EKG findings in 27 of 30 cases were with complete RBBB, three of which had LAD as well. These results suggested that re-closure of VSD can be done with minor surgical risk in order to improve to patient condition.
In a 25-year period from 1954-1979, there were 53 cases of choledochal cyst operated on at the Tokyo University Hospital. Of those cases, 47 patients underwent pathological examination and hepatocholangiography, and were classified into six types Type I : Cystic dilatation of extra-hepatic duct with normal intrahepatic duct 12 cases ; Type II : Cystic dilatation both of extra and intra-hepatic ducts 10 cases ; Type III : Cystic dilatation of extrahepatic duct with fusiform intrahepatic duct 3 cases ; Type IV : Fusiform dilatation of extrahepatic duct with normal intrahepatic duct 1 cases ; Type V : Fusiform dilatation of extrahepatic duct with cystic intrahepatic duct none : Type VI : Fusiform dilatation both of extra-and intra-hepatic ducts 4 cases and Miscellany : Cystic dilatation of extrahepatic duct with unclarified intrahepatic ducts 17 cases. Anomalous arangement of the pancreaticobiliary ductal system was seen in 19 cases including 6 with elevated amylase level of the cystic content. Biopsy specimen of the liver showed marked fibrosis in 13 cases.
Eight neuroblastomas and 1 ganglioneuroma of human subjects, 1 serial transplanted neuroblastoma in nude mice and C-1300 mouse neuroblastoma cell line were studied on its cell kinetics by flow-microfluorometry (FMF). The specimens resected from stage IV neuroblastoma patients and nude mouse were freezen in liquid nitrogen. The tumor of about 1 cm^3 block was minced and tumor cell suspensions were prepared by treatment with 0.25% Trypsin solution. Following fixation by 50% ethanol, Propidium Iodide staining was carried and DNA histogram was obtained by FMF (4800A biophisics U.S.A.). DNA histograms were divided into three different types ; unimodal, bimodal and intermediate types. Furthermore, cell population ratio of near 2C compartment were calculated. About 4×10^3 to 3×10^4 of cells were measured. Control cells were chicken red blood cells and human lymphocytes which showed 2C unimodal pattern. Five of 8 cases showed near 2C unimodal pattern. The other neuroblastomas and a ganglioneuroma were with intermediate pattern. Serial transplanted tumor in nude mice was with bimodal pattern. C-1300 mouse neuroblastoma cell line was with multimodal pattern. The specimens excised after high dose cyclophosphamide therapy showed relatively low population of 2C compartment cells compared with non-treated cases. It is still not clear how cell cycle of neuroblastoma changes by various therapeutic methods. This cytofluorometric analysis is expected to make therapeutic schedules more reasonable and effective.
Since April, 1976, advanced neuroblastoma has been treated with combined modality therapy including immunotherapy and intravenous hyperalimentation at our department. Three-drug combination chemotherapy consisting adriamycin, vincristine and cyclophosphamide was given as initial chemotherapy. Refractory cases were treated with high dose cyclophosphamide, DTIC, DDP and papaverine. Immunotherapy with BCG-CWS or Nocardia-CWS has been administered in their maintenance phase. Intravenous hyperalimentation was used during intensive chemotherapy. 10 neuroblastoma children with stage III and IV received this therapy. Meidian survival time of these children was 14 months and this was much longer than 4 months which derived from neuroblastoma children who were mainly treated with James' therapy in addition to surgery and irradiation before April, 1976. Among multiple anticancer drugs, adriamycin, high dose cyclophosphamide and DDP seemed to contribute to improved survival time. Intravenous hyperalimentation was useful for preventing malnutritional state. From these results we are now trying to establish new protocol for initial and maintenance treatment of advanced neuroblastoma.
An asymptomatic 6-year-old boy was admitted to our university hospital with abnormal findings on chest rentgenogram. His past history was noncontributory. There were no abnormal findings on physical and laboratory examinations. Radiographic studies of the chest on admission demonstrated a large mass with a sharply defined lateral margin occuping the lower left hemithorax. The mass extended from the anterior to the middle mediastinum. The computed tomography confirmed the presence of an left anterior mediastinal mass with CT numbers of -81 units. The posibility of lipoma had been investigated preoperatively. Thoracotomy was performed through the left fifth costal bed exposing a fatty tumor located in the left anterior mediastinum. The mass was easily removed as it was adherent to vital structures and was freely dissectable after the mediastinal pleura was opened. The resected tumor weighed 410 grams and measured 16 by 10 by 6.5 cm. It was consisted of the adult adipose tissues and showed no evidence of malignancy. The patient was discharged on the fourteenth day after operation without any significant complication. The mediastinal lipoma is a rare tumor. To our knowledge, 25 cases of mediastinal lipoma in infancy and childen have been reported in Japanese and English literature. In previously reported cases, only few were diagnosed preoperatively. In our case, the computed tomography examination of the mediastinum was the most valuable in providing the preoperative diagnosis.
A newborn who had an unusual combination of esophageal and duodenal atresia without tracheoesophageal fistula is reported. The patient presented with specific clinical and radiographic findings. The patient is a 1, 120 gm female newborn whose pregnancy and delivery were complicated by polyhydramnios and premature labor. On the first day, she developed cyanosis and frequent apneic attacks which needed nasal oxygen. She was transferred to our hospital on the second day of life. Her abdomen was markedly distended and nasogastric tube was not able to be passed into the stomach. No air was seen within the intestine by radiograph of the abdomen. At emergency laparotomy, the stomach was distended with large quantity of slightly muddy fluid that made suspicious of duodenal obstruction. Because of her poor generalized condition, gastrostomy was performed. Postoperatively, plain abdominal film showed "double bubble" sign. On the purpose of jejunal feeding, jejunostomy was performed on the 16th day of life, however she died due to postoperative panperitonitis on the 23rd day of life. This unusual combination of esophageal and duodenal atresia without T-E fistula was the 8th case reported in Japan.
A case of combined congenital defects of the supraumbilical abdominal wall, sterum, diaphragm, pericardiam, and heart is reported. Wide diastasis recti with lateral insertion of the rectus muscles into the lower costal cartilages, combined with an absence of the xyphoid process and the bified lower sternum. The chest roentogenogram showed dextroversion of the heart, and by chest ausculation, III° grade systolic murmur could be audible. Echocardiographically, VSD was suspected. At the age of 14 months, the chest roentogenogram showed the intestinal gas shadow in the mediastinum, so that an operative repair of defects was performed. A large oval defect in the anterior diaphragm with a corresponding defect in the diaphragmatic pericardium was identified. Loops of the transverse colon herniated into the pericardial cavity. A membranous connection between the apex and the right adjasent costal margine, and the abnormal blood supply to the apex from the terminal branch of the right internal mammary artery was present. The transverse colon was redused from the pericardial cavity, and the diaphragmatic pericardial defect was closed, attaching the diaphragmatic membrane to the undersurface of the anterior chest wall. The diastasis recti was repaired by bringing rectus muscles together without the use of relaxing incisions. The postoperative course was uneventful. The clinical features and treatment of the syndrome was discussed.
A 9 years old girl with pancreatic pseudocyst was reported. 27 days after initial trauma, the patient developed a sudden severe abdominal pain and shock duo to the intracystic hemorrhage. After ligation of the bleeding artery, cystjejunostomy with Roux-en-Y anastomosis was done without any complications. 17 cases of hemorrhagic pancreatic pseudocyst have been reported in Japan including this case. The patient was the youngest case in the literature. 66 cases of pancreatic pseudocyst in children were collected from Japanese literature and were reviewed. Trauma is the most common cause of pancreatic pseudocyst in children, though alcoholic pancreatitis is the most common cause in adults. Although the most serious complication was the hemorrhage in the cyst, this case was successfully treated surgically.