Three cases of hepatocellular carcinoma among cyproterone users. Ad hoc Committee on Androcur Users.
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Biomedical subjects
Publications and source records attributed to T Honna.
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Twenty-one children with rhabdomyosarcoma involving the "special pelvic" sites, defined as such occurring in the bladder, prostate, vagina and uterus, were treated with primary surgery between 1969 and 1977, and with primary chemotherapy beginning in 1978. Among 11 patients in the latter group who were placed in Clinical Group III (according to the classification of the US Intergroup Rhabdomyosarcoma Study (IRS), six showed partial response (PR) and underwent tumorectomy by radical surgery an average of 6 months after the start of treatment, and three showed complete response (CR) and were treated with further chemotherapy in the hope that cystectomy could be avoided. However, in the latter group, the tumor recurred (39 months, 35 months, and 27 months later), and all eventually underwent total cystectomy. Seven of the nine long-term survivors underwent total cystectomy and have premanent urinary-cutaneous stomas. Two had tumor-free bladders, but function was impaired in one because of the effect of irradiation. Normal function was preserved in only one patient in the series, whose tumor was located at the dome of the bladder. To preserve bladder function in children with rhabdomyosarcoma in these sites, more effective forms of chemotherapy will be required.
Five cases of intrahepatic biliary cyst or cystic dilatation and one suspicious case were found (6.4%) among 93 survivors of biliary atresia. The age at manifestation of such cysts ranged from 6 months to 12 years. These cysts or cystic dilatations were treated surgically in two cases, by percutaneous transhepatic cholangiodrainage (PTCD) in two, and not treated in one. Four patients are alive; one died of biliary atresia. Twenty-nine well-documented cases of intrahepatic biliary cyst were found in the literature, including five treated by the authors. The modes of dilatation were as follows: type A, noncommunicating cyst; type B, cyst with tiny communication with the intestinal loop; and type C, cystic dilatation. Clinical symptoms were fever, jaundice, acholic stool, and "cholangitis." Nineteen patients (66%) had symptoms within 4 years after the Kasai operation (first group); the modes of dilatation were mostly types A and B. However, in five of six patients with type C dilatation, symptoms developed after 10 years of age (second group). The prognosis for the second group appears poor because the condition of the liver worsens once cystic dilatation develops.
Intraoperative ultrasonography (US) was applied to sacroperineal anorectoplasty for the purpose of making a pull-through tunnel under visual guidance. Intraoperative US images the horizontal plane of the muscle complex and the tip of the forceps, which is placed at the entrance of the tunnel (ie, the space anterior to the puborectalis sling), ready to penetrate the muscle complex. A surgeon can thus penetrate the center of the muscle by confirming the relation between the muscle and forceps on the US screen. Postoperative computed tomography of four patients on whom this technique was applied demonstrated that the rectum was pulled through the exact center of the puborectalis and external sphincter muscles.
A newborn with a patent omphalomesenteric duct is described in this report. This anomaly is a very rare one. Regression of the omphalomesenteric duct occurs as a normal embryonic event in the intrauterine life between the fifth and ninth week of gestation; however, in this case regression occurred spontaneously in the neonatal period and resulted in a Meckel's diverticulum with no connection with the umbilicus. This is the first case to be reported.
A total of 16 cases of tracheal and bronchial lesions such as bronchial tumor (4 cases), aspirated foreign body (5 cases), and tracheo-bronchial stenosis (7 cases) were surgically treated at the National Children's Hospital from 1965 to 1990. A mucoepidermoid tumor was excised via bronchotomy. Pulmonary lobectomy was indicated for another 2 mucoepidermoid tumors which arose from the middle and lower lobe bronchus. Pneumonectomy was indicated for a leiomyoma located at the stem of the left bronchus and showed extensive invasion into the bronchial wall. It is important to accurately diagnose the location and extent of the tumor by performing intraoperative bronchoscopy, bronchotomy, frozen section examination in order to avoid unnecessary pulmonary resection. Three aspirated foreign bodies (2 marking needles and a peanut) were removed via bronchotomy. Right lower lobectomy was performed in a patient who received an accidental injection of barium sulfate into the right lower bronchus. The barium remained for more than one year and caused recurrent pneumonia. A radiolucent toy plate aspirated into the left main bronchus of a 3-year-old girl was overlooked for more than 3 years. Suppurative changes of the left lung necessitated pneumonectomy. Tracheal resection with end-to-end anastomosis was carried out successfully in a 5-year-old patient suffering from segmental fibrogranulous stenosis of the mid-trachea. Extensive congenital stenosis in 6 patients was repaired by costal cartilage autograft, with a 67% mortality rate. Treatment of the associated cardiac anomalies and the possible postoperative complications such as anastomotic leakage, restenosis, exuberant granulation, and tracheomalacia are the major problems associated with this type of tracheoplasty.
A number of arylbiurets were prepared and evaluated as antiinflammatory and analgesic agents by using the carrageenan paw edema and acetic acid stretching tests. Among them, the antiinflammatory activity of 1,3-dimethyl-5-phenylbiuret (7), 1-ethyl-3-methyl-5-phenylbiuret (11), and 1,1,3-trimethyl-5-phenylbiuret (13) were found to be more potent than phenylbutazone. The analgesic activity of 7 and of 5-(4-chlorophenyl)-1,1,3-trimethylphenylbiuret (16) is higher than that of aminopyrine.
Plasma total renin is a new, useful marker for nephroblastoma, but the conventional method for determining its level is sophisticated and requires large blood samples. To develop a simpler technique, a monoclonal antibody specific for both inactive and active renin (inactive + active = total) was raised, and a radioimmunoassay (RIA) system was established. This monoclonal antibody stains only the juxtaglomerular apparatus; values determined by this RIA did not change before and after activation. So far, the RIA system has been applied to 136 samples from 92 patients. Plasma total renin levels were also determined with the conventional method: samples were activated, then renin activity was assayed by measuring angiotensin I. The coefficient of the data obtained by these two different techniques was 0.921 (P less than .01). Plasma total renin levels in patients with nephroblastoma were significantly increased (546.5 +/- 297.8 pg/mL) over those in patients with neuroblastoma (218.6 +/- 46.5 pg/mL) and in controls (165.8 +/- 67.5 pg/mL, P less than .01). After removal of Wilms' tumors, the levels decreased to normal when sampled every 2 weeks. It was concluded that a newly developed RIA system is more useful in determining plasma total renin levels in patients with nephroblastoma.
A 30-month-old boy was investigated because of a huge abdominal mass in the right upper abdomen. A computed tomography scan and celiac angiography showed that the tumor involved bilateral lobes of the liver. At first, for this disseminated and "unresectable" tumor we did liver biopsy and hepatic arterial catheterization. Through this catheter we started chemotherapy using THP-Adriamycin and cis-platinum. After three courses of chemotherapy, a second-look operation was performed and trisegmentectomy was done to remove the main tumor and metastases, including the left lobe and the right anterior segment. The trisegmentectomy was performed with success, and the boy's serum alpha-fetoprotein (AFP) remains normal 37 months after the start of treatment, indicating a probable permanent cure. Such cases have rarely been reported in the literature. Our experience in treating this patient would seem to encourage aggressive management of advanced hepatoblastoma in other patients.
Gastric volvulus is a rare condition in childhood. Most of the reported cases have been acute and secondary in type with predisposing factors. Between 1965 and 1988, 49 patients were treated at this institution for chronic idiopathic gastric volvulus. Patients were divided further into two groups according to age at admission. The main symptom was vomiting in the neonatal and infantile group, whereas it was abdominal distension, vomiting, weight loss, or constipation in the older age group. This clinical feature was different from that of acute gastric volvulus in which the symptoms resulted from gastric obstruction. Plain film of the abdomen showed no characteristic findings. Contrast study of upper gastrointestinal series showed findings similar to those of the acute volvulus, but the extent or degree was less significant. Conservative treatment was successfully undertaken for the patients in the neonatal and infantile group just by keeping them in prone position. The rationale for this method was demonstrated by the clinical and radiological improvement. Fundic gastropexy was performed in all of 18 patients of the older age group and in one of 31 patients of the neonatal and infantile group. The operative results were satisfactory except for one patient with mental retardation. In Japan, neonates or infants are customarily nursed in the supine position. This is presumed to be a reason why the chronic idiopathic gastric volvulus is frequently noticed.
Hepatoblastoma differs from the adult type of hepatoma in clinical and pathologic features. The ratio of fucosylation of serum alpha-fetoprotein (AFP) was determined in seven patients with hepatoblastoma and in 21 infants and children with otherwise elevated serum AFP, using the improved technique of lentil agglutinin-affinity immunoelectrophoresis. The clinical data for the seven patients with hepatoblastoma were also reviewed. The ratio of fucosylation of AFP was significantly higher in all seven patients with hepatoblastoma, whereas it was minimal in all other cases of benign hepatic conditions such as neonatal hepatitis or biliary atresia, as well as in normal newborns. The ratio of fucosylation in hepatoblastoma, however, definitely decreased with the age of the patient at presentation. This finding suggests a correlation between fucosylation and a rapid rate of tumor growth, because all hepatoblastomas are believed to originate early in fetal life.
A 10-month-old boy was noted to have hepatomegaly, and hepatic angiography showed a huge tumor of the medial segment of the left lobe, located close to the hepatic hilum and involving the anterior segment of the right lobe in contiguity. We considered at first that resection would not be possible, but at operation the anterior segmental branch of the right hepatic artery was successfully ligated and divided, which made left trisegmentectomy possible in this case. Postoperatively, the patient was treated with cisplatin and tetrahydropyranyl Adriamycin he was doing well without any evidence of disease 24 months after the hepatectomy.
Anti HTLV-I antibody was measured in 590 cerebrospinal fluids (CSFs) employing a gelatin particle agglutination (PA) method. Anti HTLV-I antibody was detected in the CSFs from 24 patients (4.1%). The serum from the 24 patients also showed positive results. Out of the 24 patients, 11 cases (45.8%) had HAMs. Of the remaining patients 3 had ATLs with meningeal infiltration. Only 11 cases out of 24 patients were positive for anti HTLV-I antibodies when an immunofluorescence (IF) method was employed. This discrepancy of the results obtained by two methods may be explained by the presence of the patients whose CSFs contain anti HTLV-I antibodies of low titer. It is concluded that PA method is both sensitive and specific for the detection of anti HTLV-I antibodies in CSFs. Further, PA method is more convenient than IF method. So, it will be a very useful tool in the diagnosis of HTLV-I related neurological disorders and asymptomatic carriers.
High frequency ventilation and extracorporeal membrane oxygenation (ECMO) are devices that are expected to save the lives of newborn infants whose pulmonary conditions have deteriorated. A piston-pump-type high-frequency oscillator (HFO), developed by Bryan and Miyasaka called "Hummingbird," is considered to be superior to high frequency "jet" ventilators or those of the flow-interrupter type, and was used successfully in two neonates with congenital diaphragmatic hernia (CDH) in a high-risk group. The first baby was on a conventional ventilator with pharmacologic support for the first 54 hours and then operated on. Postoperative deterioration necessitated the use of HFO for the next eight days. The infant then recovered uneventfully. For the second baby, HFO was necessary both preoperatively and postoperatively. This baby had a major diaphragmatic defect and her case was complicated with pneumothorax. There was a long stormy course on HFO (total, 70 days), but the patient was successfully extubated on the 75th day postoperatively and is now doing well. We believe active long preoperative stabilization with pharmacologic support and preoperative and postoperative hyperventilation with a piston-pump-type HFO may be a new innovative strategy for the management of severe CDH patients.
The importance of determination of serum neuron-specific enolase (NSE) in patients with neuroblastoma has been emphasized by several authors. However, the specificity and sensitivity of NSE have not yet been well studied in tumors of infancy and childhood, nor is the role of serial determination of NSE in monitoring these patients fully understood. Concentrations of serum NSE were determined by a newly developed radioimmunoassay technique in 241 samples from 111 patients. NSE was also assayed in sera of nude mice bearing human pediatric tumors (16 samples), as well as in 30 tumor specimens. Eighty-two serum samples from 19 patients with neuroblastoma all showed NSE values (mean 120.2 ng/mL, range 16.2 to 722.0 ng/mL) elevated beyond the upper border of the normal range (14.6 ng/mL), even though four of the 19 patients had normal urinary excretion of 3-methoxy-4-hydroxymandelic acid (VMA) and 3-methoxy-4-hydroxy-phenylacetic acid (HVA). Twelve of these patients were monitored with serial NSE determinations, and their serum NSE were found to correlate well with the tumor burden, but were transiently modified by chemotherapeutically induced cell death. All 68 samples from nine patients, free of neuroblastoma at assessment, showed NSE values within the normal range. Thirteen of 25 patients with tumors other than neuroblastoma, however, showed serum NSE values mildly elevated beyond the upper border of the normal range (mean of the 25 patients 36.7 ng/mL, range 5.0 to 234.0 ng/mL). Results from our nude mouse study and from NSE analysis of the tumor extracts paralleled the clinical results.(ABSTRACT TRUNCATED AT 250 WORDS)
From 1977 to 1982 ileocecal resection was done in five neonates admitted to our pediatric surgical units. The growth and development of these infants were examined and comparisons were made with age matched controls. Two who had undergone ileocecal resections had a slight diarrhea. Physical examination, hematology and blood chemistry, including serum levels of rapid turnover proteins, were all within the normal ranges. Significant differences between data on these patients and the controls were not noted. Our findings indicate that ileocecal resection with less extensive ileal resection can be done in neonates as an adequate nutrition is physiologically feasible.
Twelve female infants with double termination of the alimentary tract were reported. One patient had a high rectovaginal fistula, but in the other 11 cases the tract opened into the bowel uniformly at the level of the levator ani (anorectal-vestibular fistula). In these patients, diagnosis of the anatomical level of the fistula was made definitely with our radiological technique. Excision of not only the fistulous tract but also the anterior half of the rectum below the fistula is essential to achieve a cure without recurrence. The pathogenesis of this condition is discussed and the pertinent literature reviewed.
This communication reviews the experience with reoperations for correction of biliary atresia on 29 patients at the University of Tokyo Hospital since 1972, and discusses their value in the management of biliary atresia. It also includes a report on a patient who was treated by a repeated hepatic portoenterostomy in 1972 and has survived for more than ten years without jaundice.