The prognosis of neuroblastoma is said to be affected by factors such as age of onset, stage of the disease, histological type, site of origin and peripheral leucocyte count. In this survey, an analysis was carried out on the clinical data of 380 cases of neuroblastoma which was received from the 11 facilities in Japan. The results were compared with those Breslow and McCann performed on the 246 cases of CCSGA in 1971. The survival rate at each stage, patient's age and the frequency of outbreak were compared with those of CCSGA cases. The results was summarized as follows : (1) The survival rate under 1 year of age was lower in comparison to CCSGA, which was considered due to lager proportion of the patients of stage III and IV. (2) The survival rate and frequency of outbreak of stage IV-S group were lower in this survey than CCSGA cases. (3) In the stage I and II groups, the frequency of outbreak at the age over 2 years was greater in this survey. The estimated survival curve was also compared with that of CCSGA cases for each curve with the real figures. The results were as follows : (1) The estimated survival curve in two groups was far different. This suggests that there is a considerable difference from the real figures. (2) The estimated survival curve for each stage might be obtainable by shifting the standard sigmoid curve (the survival curve by the age of the disease) along the axis of ages. (3) A higher correlation seemed to be present between the stage and the age in neuroblastoma.
Experimental studies were performed, for possible untoward effect and its prevention, of intravenous administration of fat emulsion (Intralipid) to low body weight puppies. Results were as follows. 1) Intralipid was given intravenously to mongrel dogs, weighing from 270g to 2, 500g, either by one shot or by drip infusion method, with doses ranging from 1.0 to 12.0g/kg. ST elevation was noted in ECG, but there were no distinct abnormal findings in blood acid-base balanse, EEG, blood pressure, pulse rate and body temperature. 2) It was found that arterial blood samples, which were taken from low body weight dogs, priorly injected with Intralipid, and incubated for 20 to 30 minutes, showed black-brownish discoloration accompanied by decrease in PO_2. This phenomenon was observed in all the experimental dogs below 1, 500 of body weight, regardless the difference of dose or mode of administration. 3) The phenomenon was manifested more markedly when incubation temperature was high (37℃) or blood sugar level was low (about 40mg/dl). When incubation temperature was very low (4℃) or blood sugar level was extremely high (about 400mg/dl), the phenomenon was not remarkable. The mechanism underlying this phenomenon is to be clarified in future, but administration of glucose, together with Intralipid, may be beneficial in treating baby patients.
Recent investigations on the metabolism of bilirubin suggest that free or unbound bilirubin which is not bound to plasma albumin, easily moves into the brain and results in brain damage. The concentration of unbound bilirubin was compared with that of total bilirubin in 19 cases with hyperbilirubinemia. The concentration of unbound bilirubin in the plasma was measured by the modified Jacobsen's method using peroxidase. The following results and conclusions were obtained by this study : 1) There were no close correlations between the concentrations of unbound bilirubin and that of total bilirubin. 2) In mature neonates, although the concentration of total bilirubin in the plasma exceeded 20mg%, that of unbound bilirubin remained at low levels except in one case. 3) On the contrary, in immature neonates, the concentration of unbound bilirubin remained at high levels, while the amount of total bilirubin was under 15mg%. 4) The unbound bilirubin concentration as well as that of the total bilirubin in the plasma was effectively reduced by phototherapy. In the indication of exchange blood transfusion for severe jaundice in neonates in decided on a basis of the measurement of the concentration of unbound bilirubin, the number of early exchange blood transfusion will be reduced.
In 15 patients with various sysmptoms of vomiting, failure to thrive and/or recurrent respiratory disorders, continuous esophageal pH monitoring was carried out for gastroesophageal reflux (GER). The equipment used in this study was designed with the separate type electrodes; sensing and reference probes. The sensing electrode was with a diameter of 2mm, whicn was connected to the flexible line and was easy to be inserted to the esophagus. The position of the probe was 3cm above the esophagogastric junction. In three of these 15 patients, there was remarkable decrease of pH, which lasted for over 30 minutes regardless cough, strain, feeding or vomiting. These findings were considered as the positive GER in this study, which was confirmed by subsequent upper GI X-ray series. These three patients have undergone a combined procedure of incomplete fundplication and anterior fixation of the stomach. Esophageal pH monitoring, repeated again after operation in these patients, showed no GER. This study suggests that continuous esophageal pH monitoring is a reliable diagnostic technique to detect GER, although the number of the cases experienced is small.
The proper level of the anterior rectal wall in modified Duhamel's procedure was studied and discussed. It is more favorable that remaining aganglionic zone should be as small as possible after radical operation for Hirschsprung's disease. But sympathetic and parasympathetic nerve are vulnerable, when an extensive mobilation of the rectum is attempted between the rectum and posterior urethra. Our study suggests that the level of the bladder neck or P-C line is most favorable for the coloproctostomy in the anterior rectal wall. This level is located in 3.5〜4.0cm in infant and 4.0〜5.0cm in child from the anal opening. When coloproctostomy is performed at this level, a part of the rectal ampulla which plays an important rolo to rectal sensation remains, and postoperative temporary stenosis in anastomotic stoma is avoidable.
This study dealt with 39 infants less than 12 months old who underwent operative correction of ventricular septal defect using extracorporeal circulation under normothermia. The "catch-up" growth pattern of these infants after VSD closure is most gratifying. It was strongly suggested that adequate postoperative weight gain was produced by improvement of infantile lobar emphysema. Results of catheterizations that 18 patients underwent more than 1 year after operation were satisfying except 2 patients reoperated on for residual shunt. Residual shunt was detected in 4 patients 2 of whom had no indications for reoperation for 5 years after operation. We considered that residual shunt should be followed up without immediate reoperation, if symptoms improve. Though there was no late mortaliy, RBBB and LAH following temporary complete A-V block appeared on 2 patients. Careful follow-up is necessary for these patients.
Nine cases with entire colon aganglionosis were reported, of which 5 cases haved survived. Most reliable findings on X-ray studies were ileal dilatation and delayed passage of barium. Histological investigation of the intestinal biopsied specimen during the preliminary operation is essential to make a difinite diagnosis, bacause the change in caliber of the involved ileum is not so reliable as it is in a case with classical Hirschsprung's disease. According to English and Japanese literatures, a great number of cases with entire colon aganglionosis have died before radical operation due to malnutrition and due to fluid imbalance caused by short gut or enteritis. An aggresive supportive measures including intravenous hyperalimentation, are mandatory in the period waiting for the radical operation. Although Martin's operation may be effective to restore the digestive ability, it does not seem to be always to correct all of unphysiological status observed in entire colon aganglionosis.