We measured counts of peripheral leukocyte, lymphocyte, T-cell and B-cell, peripheral T and B cell fractions, values of lymphocyte blastoformation by PHA and Con A, and levels of serum IgG, IgA, IgM, C_3, C_4 and CH50 in 8 patients with neuroblastoma, 6 children with SSPE (subacute sclerosing panencephalitis), 11 mothers of the patients with neuroblastoma and 3 mothers of the patients with other diseases. Patients with neuroblastoma have higher counts of lymphocytes, T cells and B cells, higher response of lymphocyte blastogenesis to Con A and lower levels of some immunoglobulin than their mothers and SSPE patients. These results were thought to be age-dependent and not neuroblastoma-specific. We also discussed the development and growth of neuroblastoma, based on the results of these levels of non-specific immunocompetence.
For the purpose of clarifying a developmental maturation of the lower esophageal sphincteric mechanism, which is a representative of antireflux competence, 47 normal newborn and early infants were examined by 24-hour pH-monitoring in the disital esophagus, and 37 regurgitated infants without organic disturbance and infants and children with gastroesophageal reflux (GER) caused by organic disturbances were also invesitgated. And following results were obtained. 1) It was revealed that physiological maturation of the competence of lower esophageal sphincter would be completed within 3 weeks of life. 2) No significant difference of pH-score calculated from 4 components of pH-data was noted between the position of supine and semi-sitting, resulting from examinations in each position. 3) From comparing normal infants and those with a regurgitation or vomiting, it was demonstrated that ph-monitoring was beneficial to make the precise evaluation of GER. 4) Infants with regurgitation or vomiting were divided into 3 groups according to pH-scoring system, i. e.; A group had no necessity for surgical treatment, C group is having a high need to surgery, and B group consists of infants who need careful investigation because they have a possibility of moving forward C group. 5) For taking the objective decision of surgery in infants with GER, the age factor seemed to be important in addition to pH-score system and symptoms.
Oliguric conditions accompanying renal failure associated with severe diseases treated by pediatric surgery were investigated in 24 cases. During the period from admission up to between three and seven days after surgery, renal function was evaluated through the following parameters: (1) excreted urin volume, (2) S-cr and BUN, (3) urine osmotic pressure and U/P Osm and (4) urine concentration of Na and FENa. Changes in PR A and A-II were also observed. Standard values of PRA and A-II had been determind. PRA and A-II were highly correlated, with maximal values in the neonatal period, which thereafter showed an age-dependent decrease. Parameters for evaluating renal function were reliable, S-cr and FENa being especially useful. With regard to oliguric conditions on admission, a neonatal death group represented acute nephric renal failure, and a surviving group represented the transition from prenephric renal failure to nephric renal failure, while corresponding infant and child groups represented prenephric renal failure. On the other hand, a surviving neonatal group represented the pre-symptoms of nephric renal failure for two or three days after surgery even if diuresis was obtained. Activated and/or increased PRA and A-II were considered to contribute to the occurrence of oliguria leading to nephric renal failure. Dopamine, combined with fluid transfusion, was useful for oliguria on admission, being especially effective for prenephric renal failure. Inhibition of increase in the renin-angiotensin system for neonates with presymptoms of nephric renal failure was necessary even if diuresis was found, suggesting that early usage of captopril was desirable.
To make respiratory management in neonates and infants after cardiovascular surgery easier and more objective, measurement of lung compliance (C_L) and respiratory resistence (R_L) using esophageal balloon technique was examined. Basic investigations of the method and clinical measurement in 16 cases revealed; (1) Esophageal balloon technique provides reasonably precise values of C_L and R_L in neonates and infants in supine position, if properties of the esophageal bolloon and the measuring units are frequently checked, and if the balloon is placed in the esophagus accurately with the formula based on body weight. (2) Clinical investigation was done with 16 neonates and and infants after cardiovascular surgery, of which 9 cases (group 1) were difficult to manage, though 7 (group 2) had little difficulty. Both C_L and R_L showed good relation to other parameters and general condition. Measurement of C_L and R_L should be useful for their post-operative management, assessing their condition more accurately. (3) There were several problems in this trial. Measurement in neonates and infants under mechanical ventilation was impossible because of the leakage from endotracheal tube caused by positive pressure ventilation. It should be recommended, to estimate the condition of the infants not only from C_L and R_L, but also from other informations which are easy to get.
Heart distension after the open heart surgery was treated by emergent sternum separating splint and secondary sternal closure in 2 patients of tetralogy of Fallot. Total correction of the anomaly was done using surfacecooling deep hypothermia and cardiopulmonary bypass. A technique of secondary sternal closure is described. Case 1 was weaned off cardiopulmonary bypass without difficulty. Eight hours after operation, the patient became hypotensive and cyanotic. In emergency, sternum was opened and the cardiac function recovered. The sternum remained open by splint with use of the plastic chest drain and steel wire. Eighty days after the operation, sternal splint was removed and the sternum was closed successfuly. Case 2 was complicated by refractory hypotension due to heart distension during radical operation. Therefore, the sternum remained to be separated and only the skin was sutured. Stable circulation was maintained by administration of noradrenalin. However, thirty-six hours after operation, the patient's condition was deteriorated. Sternum was splinted open using the dental resin plate. After that, patient recovered well. Thirty-eight days after the operation, the resin splint was removed and the sternum was closed successfuly. Two patients survived after delayed sternal closure for heart distension. Delayed sternal closure was useful for distension of heart and maintain a good circulation.
A case of volvulus without malrotation seen in a 2-day-old female baby was reported. Her birth weight was 2,884g. An intestinal perforation was suspected preoperatively and it was proved at laparotomy. A perforation of the ileum and volvulus without malrotation were recognised. Sixteen cases of volvulus without malrotation were collected from Japanese literatures. A total number of 23 cases have been reported in the world literature.
A physically retarded eight-year and three-old boy presented with vomiting and diarrhea. He had complained of anorexia since his infancy. Administration of thyroid hormone for hypothyroidism, gastrostomy for feeding and fundplication for gastroesophageal reflux had been performed. A protruding tumor of the anterior wall of the stomach, at the site of former gastrostomy, was discoverd unexpectedly by upper GI series. Endoscopic biopsy of the tumor revealed gastric cancer. Partial gastrectomy with complete removal of group I lymphnodes were performed. The operative finding showed neither peritoneal dissemination nor hepatic metastasis. The skin of the abdominal wall around the gastrostomy was excised additionally. Histological study of the specimen demonstrated moderately differentiated tubular adenocarcinoma with limited invasion into the mucosa. The surgical margin was carcinoma-free. Two years postoperatively the boy is alive and well without any evidence of recurrence. Gastric cancer in children under 15 years of age is extremely rare. Sixty-four cases were collected among Japanese literatures. All 64 cases had advanced cancer and died of cancer. This is an exceptional case with early cancer and survival.
A 3-year-old boy with adrenal cortical carcinoma is reported. Chief complaints were obesity and hypertrichosis. Endocrinological studies and selective renal angiography revealed a left adrenal tumor. Left adrenectomy, weighing 13 g, was performed through an upper transverse incision. Local invasion and distant metastasis were not found. Histologically, the tumor showed adrenal cortical carcinoma with capsular invasion and vascular tumor-thrombi. Vincristin and cyclophosphamide were administered 5 times every other week, and 5-furuolouracil was given for 6 months, postoperatively. No radiation therapy was done. Ten years after the operation, he is doing well without any evidence of disease. Forty seven children under 15 years of age with adrenal cortical carcinoma were reviewed from the Japanese literature. Non-functioning tumor was found in only 9%, and the incidence of non-functioning tumor was obviously lower than that adult patients. Only 8 patients have survived for more than 8 years. There was a close relationship between the tumor-weight and survival: In survivor, the mean tumor-weight was 187 grams, whereas it was 1,006 grams in non-survivor.
This paper summarizes our experience on patient with traumatic hematoma who was suc-cessfully treated by nonoperative management. This 7-year-old female sustained a blunt injury in the upper abdomen with a hundle-bar of a bicycle. On admission 50 minutes following injury, physical examination disclosed a marked tenderness in the epigastrium with blood pressure of 90/40mmHg. Laboratory data showed Hgb: 12.2mg/dl, GOT: 354u, GPT: 395u, LDH: 700u, s-amylase : 105IU, Because of her stable hemodynamic condition, nonoperative management was attempted. Her blood pressure decreased to 78mmHg systolic 3 hours after injury. As her BP was restored with a rapid infusion of lactated Ringer solution, the nonoperative management was continued. She received 400ml of blood transfusion because of a decresed hemoglobin, 9.2mg/dl 16 hours after injury. An abdominal CT scan disclosed a low density area in the medial segment of the left lobe of the liver, suggesting intrahepatic hematoma. She recovered well without sequelae and was discharged 3 weeks after injury. A CT scan was repeated 2 months after injury, showing a reduced "low density area" in its intensity as well as its area.
Familial occurrence of pheochromocytoma reported initially by Hyman in 1943 is rare and forty-seven cases of pheochromcytoma in nineteen pedigrees have previously reported in Japan. A nine-year-old girl with pheochromocytoma complained of headache, hypertension and visual disturbance. Her father who died at the age of 32, had been diagnosed as pheochromocytoma and other three persons in her family had been strongly suspected of this lesion with the history of severe hypertension and retinal hemorrhage. Analysis of previous Japanese literature indicated that the incidence of bilateral pheochromocytoma in case of familial occurrence was higher than in others and from the urinary catecholamine assay, the NA/AD ratio in familial pheochromocytoma was very high in comparison with Sipple's Syndrome.
An one-month-old infant was admitted because of dyspnea, tachycardia and systolic heart murmur. The chest X-p film demonstrated a remarkable cardiomegaly and a pulmonary congestion. The ECG showed a depression of ST-T in V5-6 leads. Cardiac catheterization and angiography were performed. The peak systolic pressure gradient across the aortic valve 62mmHg and the aortic valve area was stenotic. An open aortic valvular commissurotomy was performed in emergency with a cardiopulmonary bypass and a profund hypothermia. He recovered well and discharged at 55 days after operation and ST-T change at ECG was diminished. In Japan, congenital aortic stenosis in infancy is rare and there are a few reports of successful surgical treatment. We discussed clinical feature of congenital aortic valvular stenosis in infancy and its surgical treatment.
A 14-year-old girl was found to have right ovarian tumor of 12×6×3cm, which is not associated with any apparent metastasis at her first laparotomy. Although right oophorectomy was performed successfully, tumors were found disseminated into the abdominal cavity 3 months later, accompanied with the increased serum alpha-feto protein(AFP)level. In spite of the vigorous chemotherapy using vincristine, actinomycin-D, and cyclophosphamide, tumor growth continued. Repeated tumor resections were performed to eliminate tumors. After the final resection serum AFP level decreased to normal and no tumor regrowth has been detected. Microscopic examination of the resected tumors demonstrated that the primary tumor was composed of the tridermal immature tissues containin some embryo id bodies. Metastatic tumors were mostly found as the peritoneal dissemination but a few lymph node metastases were also found. All metastatic tumors were also composed of mature and immature tissues of the tridermal origin, but they did not contain any tissue suggesting malignancy or embryoid bodies. Immunoperoxidase techniques revealed that AFP was located at the yolk sac tissue in the embryoid body, at the liver tissue-like cell clusters, and at the tubular structures of immature endodermal origin. Embryoid body may be the structure which suggests the presence of the totipotent cells, which can metastasize and differentiate into any embryonic or adult tissues. These totipotential cells may not necessarrily be of malignant nature. AFP can be found in the benign teratoma when it contains any tissne that can produce AFP in the course of fetal development.
We experienced a case of malignant rhabdoid tumor of the left kidney. The case was a 1-year and 2-month-old girl and had the left a 9 X11 cm sized mass at the left upper abdominal region and multiple metastases in the left lower lung field. Left nephrectomy and tumor extirpation was performed, but the metastatic tumor to paraaortic lymph nodes could not be resected. Histological examination showed malignant rhabdoid tumor. She had been treated after operation with radiotherapy and chemotherapy using actinomycin D, vincristine, adriamycin and cis-platinum. However, several bilateral pulmonary metastases and pleural effusion with numerous tumor cells appeared and the patient expired on the 114th day after operation. The prognosis of this tumor is poor and is considered to be a separate group from the usual nephroblastoma. Seven cases of this tumor have ever been reported in the Japanese literature. There is no effective therapy for this tumor and further study is desired particulary in chemotherapic agent.
A 5-month-old baby was admitted to Tohoku University Hospital with complaint of right abdominal tumor. Multiple, various-size cysts were observed by ultrasonogram and computerized axial tomograrn. High blood pressure, 180/108mmHg, microscopic haematuria, and abnormal LDH level were observed in this patient. Right nephrectomy was performed under the diagnosis of multilocular cyst of the kidney. Microscopic examination disclosed that the tumor have blastoma cells in the tumor and diagnosis of cystic partially differentiated nephroblastoma was made.
There have been a few reports that intra-aortic balloon pumping (IABP) was successfully weaned in children with left heart failure. This is a case report that IABP was successfully weaned in a 17-month-old girl with atrial septal defect and severe left heart failure after closure of atrial septal defect, and the patient was discharged the Hospital without any other complication. The balloon of 6ml was inserted into the ascending aorta and IABP was effective to wean from cardiopulmonary hypass. Diastolic augmentation was clearly evident on the arterial pressure curve. IABP was taken out on the 4th postoperative day, and the hemodynamics were well maintained with dopamine and isoproterenl infusion. The chest wall was closed on the 6th postoperative day. Two-dimensional echocardiography showed improvement of the left ventricular contraction before dismissal. There was no any other complication, and the chest X-ray showed that cardio-thoracic ratio was 46 percent at 2 months after surgery.
In 1984, 643 pediatric malignant solid tumors were registered to the Japanese Society of Pediatric Surgeons under the support of the Children's Cancer Association of Japan. Among those, there were 151 neuroblastoma group tumors, 59 malignant renal tumois, 33 liver tumors, 101 teratoma, 37 rhabdomyosarcomas and 262 other tumors. In 151 neuroblastoma group tumors, there were 99 neuroblastomas, 39 ganglioneuroblastomas and 13 ganglioneuromas. Twelve cases were discovered under the nation-wide VMA mass screening system. Primary tumors were resected totally in 76 cases and subtotally in 22 cases. Second look operation was carried out in 10 cases. Among renal tumors, there were 58 Wilms' tumors, 3 adenocarcinomas and 4 mesoblastic nephromas. Chemotherapy was given to all the Wilm's tumors except one. Among the liver tumors, there were 26 hepatoblastomas, one hepatocarcinoma and 2 undifferentiated sarcomas. In macroscopic findings, 19 were massive, 7 were multi-nodular and one were diffuse in type. In the stage distribution, 77% were Stage III or IV among hepatoblastoma. In teratoma group, 65 mature, 11 immature and 23 malignant tumors were included. Two were head and neck tumors, 9 mediastinal, 33 ovarian, 15 testicular and 23 sacrococcygeal tumors. Total resection was carried out in 88%. VAC was the main chemotherapeutic method. In rhabdomyosarcoma, 14 were in pelvis, 3 in thorax, 4 in extremities and 10 in abdomen. In other tumors, 69 malignant lymphomas, 86 brain tumors, 20 retinoblastomas and 69 osteosarcomas were included.