The distribution of glial fibrillary acidic (GFA) protein- and S-100 protein-like immunoreactivity was studied in the colons obtained from 24 Japanese children with Hirschsprung's disease. In the enteric nervous system all supportive elements were immunoreactive to S-100 protein, and the activity was localized in the myenteric and submucous ganglia as well as in the intrinsic and extrinsic nerve fibres. On the other hand, GFA protein was present only in the subpopulation of supportive cells within extrinsic nerve fibres which were considered to be parasympathetic preganglionic fibres. We concluded that immunohistochemistry for GFA protein can be of excellent histological value in diagnosis of Hirschsprung's disease.
Plasma catecholamine levels were determined pre- and postoperatively in 70 children. Plasma epinephrine and norepinephrine levels were significantly elevated within 24 hrs postoperatively. Only plasma norepinephrine levels were correlated with surgical stress scores. Plasma epinephrine levels were higher in patients undergoing reoperation than those at the first operation. Plasma norepinephrine levels were reduced in patients receiving analgesics. The results were not associated with age. The duration of high catecholamine levels following operation was 3 to 4 days in children which was longer than that of adults. The changes of urinary catecholamine levels were similar to plasma catecholamine but did not correlate with surgical stress scores. Hair cuticles were located in parallel and there was a tendency of making mosaic formation after operation. Hair cuticle score was graded according to the postoperative changes of hair cuticle examined by scanning electron microscopy. Hair cuticle scores correlated with plasma norepinephrine and surgical stress scores. The examination of hair cuticle of rats in stress showed similar results. In conclusion, plasma catecholamine levels and hair cuticle scores can be an indicator of surgical stress in children.
For the purpose to investigate the influence of interruption of the lymphatic drainage from the liver, we designed experimental study by ligating the lymph vessels in the porta hepatis of mongrel dogs and observed its effects on reticuloendotherial system (RES) function and hepatic microcirculation. The follwing results were obtained. 1) Two weeks following ligature of the lymph vessels there was a significant depression in Kupffer cell phagocytic and bacteriocidal function. Additional common bile duct ligation markedly depressed this function even further. 2) When lipopolysaccharide of E. coli was injected into the bile duct of mongrel dogs with thoracic duct drainage, endotoxin shock was not induced at an intrabiliary pressure of less than 25cmH_2O but was induced at an intrabiliary pressure of 35cmH_2O or more. When the lymph vessels were ligated, endotoxin shock could be induced at an intrabiliary pressure of less than 25cmH_2O. 3) To study the role of hepatic lymphatic system as RES, lipopolysaccharide was injected into the hepatic lymph vessel and thoracic duct lymph sample was collected. It was found that 60 to 70 per cent of injected lipoplysaccaride was cleared from lymph. It was suggested that ligation of the lymph vessels in the porta hepatis would depress RES function, induces the spill over phenomenon from the hepatic lymphatic system, and impair the local host defense of the liver.
A statistical analysis concerning the types of Hirschsprung's disease, operative methods, age of initial treatment, etc. was performed on 79 cases of Hirschsprung's disease, which were treated either at the Gifu University Hospital or at the Nagara National Hospital during the past 18 years period. Relationship between findings of manometric study and postoperative bowel functions was also examined in 32 postoperative patients over 4 years of age. Following results were obtained. 1) Recently, the diagnosis of Hirschsprung's disease was made earlier (within infancy) and radical operation was performed at younger ages. 2) Postoperative bowel functions significantly improved with an increase in age. This improvement continued even over 13 years of age. 3) Satisfactory bowel functions were noted more frequently in the cases of showing higher anal canal pressures and longer anal canal lengths, as evaluated by manometry. The anal canal pressure in the "fair" bowel function group was significantly lower than that in the "excellent" bowel function group. The cases with satisfactory bowel functions did not always show positive rectosphincteric reflex postoperatively, while the cases showing positive reflex postoperatively tended to have satisfactory bowel functions. 4) It was found that "the Scoring of the Meeting of Anorectal Malformation" was useful as a common scale for the evaluation of postoperative bowel functions of Hirschsprung's disease patients.
Questinnaires were sent to the councilors of the Japanese Society of Pediatric Surgeons requesting data on Pseudo-Hirschsprung's disease. Two definitions of Pseudo-Hirschsprung's disease were given: 1) congenital, chronic, nonmechanical obstruction of the intestine, and 2) the presence of intramural ganglion cells in the terminal rectum. A total of 93 replies was received, reporting 148 cases of Pseudo-Hirschsprung's disease of which 77 were abnormal in the intramural ganglia and 42 were normal, and 29 were unknoun. In the abnormal group of the intramural ganglia, there were immaturity of ganglia or oligoganglionosis (54), neuronal intestinal displasia (15), and segmental anomaly (8). In the normal group of myenteric plexus, 22 were chronic idiopathic intestinal pseudoobstruction syndorome (CIIPS), 12 CIIPS suspected, and 8 megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS). The most of oligoganglionosis (or hypoganglionosis) or immaturity of ganglia showed negative rectoanal reflex and normal Ach-E activity, while the most of the normal group of intramural ganglia revealed positive rectoanal reflex and normal Ach-E activity. The prognosis of oligoganglionosis (or hypoganglionosis), CIIPS, and MMIHS were very poor, considering that these are benign diseases. Pseudo-Hirschsprung's disease was suggested to be truely one of serious diseases in children.
Liver function derangement is one of the most common complications of total parenteral nutrition (TPN) in pediatric patients, especially in neonates. Recent studies suggest the positive relationship between intestinal overgrowth of anaerobic bacteria and liver dysfunction during TPN. Therefore, we investigated the effect of Metronidazole (MNZ) on hepatic dysfunction associated with TPN in neonates. Neonatal patients on TPN for more than 2 weeks were devided into 3 groups. In Group I TPN alone was given, in Group II MNZ 25 mg/kg/day was administered intravenously for the first 2 weeks of TPN, and in Group III 50 mg/kg/ day of MNZ was given for 3 weeks. The maximal levels of liver function tests during the first one month of life were compared among these 3 groups. There was no significant difference between Group I and Group II. Although as to ALP, γGTP, direct bilirubin and total bile acid there was no significant difference between Group I and Group III, transaminase (GOT and GPT) of Group III remained significantly low compared with that of Group I (p<0.05). MNZ prevented, at least, the elevation of transaminase during TPN in neonates, suggesting that the possible involvement of anaerobic bacterial flora of the intestine in the pathogenesis of TPN-associated liver function derangement.
A fifty eight cases of congenital intestinal atresia were analized. There were 40 survivals and the mortality was 31%. Associated anomalies were found frequently in duodenal atresia and high jejunal atresia, and they tended to be multiple and life-threatning. Since these anomalies affect the mortality, their management would be the key point in the treatment or congenital intestinal atresia, which was especially true in duodenal atresia and high jejunal atresia within 20cm distal to ligament of the Treitz.
Acase of nephroblastoma with a high level of serum α.fetprotein (AFP) arising from the right kidney of a 1 year and 10 months old boy was reported. His preoperative level of serum AFP was 670ng/ml,which decresed to normal value immediately after total excision of the tumor without metastasis to liver. Histochmical examination showed an epithelial type of nephroblastoma. Immunohistochemical study revealed a positive localization of AFP in tublar structurs as well as intratublar secretion. No other one similan to this case has been reportd in the world literture.
A case of postoperative graft-versus-host disese (GVHD) was reported. The patient was a 4-year-old male with a ventricular septal defect. He had on symptoms except heart murmur. The open heart radical operation was performed on cardiopulmonary bypass. The postoperative course was uneventul until the 6th postoperative day when he had high fever followed by skin rashes over the whole body on the 10th postoperative day. His condition became worse progressively with leukopenia, thrombocytopenia, jaundice and diarrhea. The diagnosis of GVHD was made with the findings of skin and bone marrow biopsy. In spite of intensive care, he died on the 35th postoperativeday. GVHD tends to occur after a cardiovascular operation due to a large amount of fresh blood transfusion and severe operative stress. There are no effective treatment except to prevent this complication.
Congenital non-union of the spermatic duct was found in two cases during orchidopexy for undescended testis. The first patient was four years old and the other was 23 months old. The left side was undescended on both cases. The testes were located in the inguinal canal but the spermatic ducts were closely approximated to the pubic bone with an abnormal attachment of the gubernaulum. The spermatic ducts were considered to be completely separated congenitally prior to the attachment to the testicles. The proximal and distal ducts were opened and anastomosed using an operating microscope. The inner layer was anastomosed with three 11-0 monofilament Nylon sutures. The external layer was anastomosed with five 9-0 absorbable sutures (Vicryl). The testes were fixed in the scrotum by the orchidopexy procedure. This may be the first attempt of microsurgical anastomosis of congenitally divided spermatic ducts that will allow a chance of fertility.
Case 1; A 5-year-old male, who had complained of abdominal pain, came to our hospital. Echogram demonstrated a tumor in the right hepatic lobe, in which multiple cystic areas were visualized. Celiac arteriogram disclosed tumor vessels with central hypovascularity distal to the right hepatic artery. AFP was negative. He underwent right hepatic lobectomy. Standard VAC therapy was added postoperatively for 12 weeks, followed by cyclophosphamide given orally for another 1 year. He is doing well without recurrence at the time of follow-up of 35 months postoperatively. Case 2; An 8-year-old male was admitted, suffering from severe abdominal pain and distension due to a large hepatic tumor in the right lobe and left medial segment. Hematological data showed liver dysfunction and negative AFP. Emergency laparotomy revealed the tumor invasion to the diaphragm. He underwent right hepatic trisegmentectomy. CCA (cis-platinum, cyclophosphamide, adriamycin) therapy was started on the 10th postoperative day every 3 months. There is no evidence of recurrence 17 months after surgical intervention. Pathological diagnosis was undifferentiated sarcoma of the liver in both cases, with positive Sudan III in case 1, and positive α_1-antitrypsin and α_1-antichymotrypsin in both. We reviewed 19 pediatric and 3 adult cases reported in Japan. Among the 5 pediatric survivors, 4 have survived over 2 years without recurrence. Survival of 17 months without recurrence in case 2 is the longest among 4 cases with tumor ruptured at the primary operation, while tumor-free period is only 2 〜 8 months in the others. This review suggests that CCA therapy is the most effective for the non-resectable undifferentiated sarcoma of the liver.
An 8-month-old girl with stage II neuroblastoma detected by neuroblastoma mass screening who developed bone and bone marrow metastases 12 months after the teatment is presented. This patient had regional lymph nodes involvment when she was operated. It is generally accepted that the presence of involved lymph nodes worsens the prognosis in stage II neuroblastoma. Therefore, stage II neuroblastoma with regional lymph nodes involvement should be treated with caution.