PubMed Health⌕ Search

Biomedical subjects

Y Sera

Publications and source records attributed to Y Sera.

At least 55 records · Page 3Linked to original sources

Stratification of treatment of stage 4 neuroblastoma patients based on N-myc amplification status. Study Group of Japan for Treatment of Advanced Neuroblastoma, Tokyo, Japan.

BACKGROUND: It has been shown that children aged more than 12 months with stage 3 and 4 neuroblastoma with N-myc amplification do worse than those without amplification. Development of an innovative chemotherapeutic protocol for patients in such an extremely poor-risk group was the purpose of this study. PROCEDURE: Since March 1991 a new protocol for the treatment of advanced neuroblastoma was started. When N-myc was amplified more than 10-fold, patients received regimen A3, in which cyclophosphamide 1,200 mg/m2 was given on days 1 and 2; hence the dose of cytotoxic drugs was doubled. Patients with fewer than 10 copies of N-myc received regimen new A1, which is very similar to regimen A1 that had been used until March 1991 for all patients with advanced neuroblastoma with/without N-myc amplification. The efficacy of regimen A3 was evaluated. RESULTS: The relapse-free survival rate at 1 and 2 years for stage 4 patients older than 12 months of age with N-myc amplification of more than 10-fold was 43% and 29%, respectively, with regimen A1 and that for the same subgroup of patients treated with regimen A3 since March 1991 was 79% and 49%, respectively; the difference is statistically significant. On the other hand, there were no differences in the relapse-free survival rate at 1 year and 2 years for stage 4 patients with fewer than 10 copies of N-myc between those treated with regimen A1 and those treated with new A1 since March 1991. CONCLUSIONS: Stratification based on N-myc amplification into new A1 and A3 treatment protocols is of significant clinical importance. Regimen A3 was well tolerated and showed an improvement in clinical results in stage 4 patients with N-myc amplified more than 10-fold.

Antineoplastic Combined Chemotherapy Protocols↗

No deterioration in insulin sensitivity, but impairment of both pancreatic beta-cell function and glucose sensitivity, in Japanese women with former gestational diabetes mellitus.

To identify the primary pathogenic factors involved in the development of Type 2 diabetes mellitus (DM), we studied Japanese women with former gestational diabetes mellitus (GDM) who are at risk for the later development of Type 2 DM. We used the minimal model analysis derived from frequently sampled intravenous glucose tolerance test (FSIGT). The subjects consisted of eight non-obese women with a history of GDM and eight non-obese normal women as control subjects. The 75 g oral glucose tolerance test (75 g OGTT) performed within 6 months of delivery confirmed that all the subjects with former GDM had a normal glucose tolerance. Insulin sensitivity (SI) derived from the minimal model analysis was not different between the two groups. Glucose effectiveness at zero insulin (GEZI), reflecting tissue glucose sensitivity, was significantly lower in former GDM patients than in control subjects (1.18+/-0.34 vs 2.26+/-0.29 x 10(-2) min(-1), p < 0.05). The early phase insulin secretion found in FSIGT was markedly reduced to 56% of that observed in control subjects (1250+/-87.4 vs 2223+/-304.3 pmol l(-1) min, p < 0.01). Our results indicate that in former GDM patients, who are japanese and non-obese, impairment of the acute insulin response to glucose and a decrease in tissue glucose sensitivity rather than insulin sensitivity are the primary pathogenic factors involved.

Blood Glucose↗

Gallbladder contraction in biliary atresia: a pitfall of ultrasound diagnosis.

In 3 (9 %) of 34 children with biliary atresia, US revealed gallbladder contraction following an oral feed, given on admission, but not with subsequent feeds. Surgery revealed a Kasai type IIIa biliary atresia with a patent communication between the gallbladder and duodenum. We propose that the bile ducts may initially have been patent, but then gradually became obliterated secondary to inflammation. These cases may explain the development of one type of biliary atresia.

Biliary Atresia↗

Transiliac catheterization of the inferior vena cava for long-term venous access in children.

In infants and children requiring prolonged, multiple central venous (CV) catheterizations, the superior (SVC) and inferior vena cava may become thrombosed or stenotic, making CV access a difficult problem. Use of the iliac vein may be an acceptable alternative. We report a patient with thrombosis of the SVC in whom the external iliac vein was accessed through a retroperitoneal approach for placement of an implantable port. This technique is easy to perform, and there are no special materials or patient positioning required.

Catheterization, Central Venous↗

Mass screening for neuroblastoma at 6 months of age: difficult to justify.

BACKGROUND/PURPOSE: A statistical analysis of the mass screening for neuroblastoma in Japan based on a population study rarely has been reported. This study aims to evaluate retrospectively the effectiveness of mass screening at 6 months of age using the available population data. METHODS: The data on the neuroblastoma cases registered by the Committee for Pediatric Solid Malignant Tumors in the Kyushu area were analyzed based on both screened and unscreened populations in the Kyushu area. RESULTS: From 1988 to 1992, the cumulative incidence of neuroblastoma in children less than 5 years of age was 82 in 484,599 for screened children, and 11 in 92,966 for unscreened children, respectively. Fourteen of the 82 screened patients had negative findings at 6 months of age (MS-negative cases). No significant difference was observed in the cumulative mortality rates from neuroblastoma in children younger than 5 years of age between the screened children and the unscreened children. Six of seven patients who died among the screened children were MS-negative cases with stage III or IV disease. In addition, no significant difference was found in the cumulative mortality rates from the neuroblastoma cases in patients less than 5 years of age between the children screened from 1988 to 1992 (7 of 484,599) and all children from 1980 to 1984 (14 of 668,084). CONCLUSIONS: These findings suggests that the majority of the patients detected by mass screening had a favorable prognosis, and, mass screening in Japan for children less than 6 months of age was not observed to reduce the incidence and mortality from neuroblastoma. Therefore, mass screening at 6 months of age was not found to improve substantially the prognosis of patients with unfavorable neuroblastoma identified over 1 year of age, which is the primary purpose of such mass screening for neuroblastoma.

Evaluation Studies as Topic↗

Single-shot MR cholangiopancreatography of neonates, infants, and young children.

OBJECTIVE: This study was undertaken to assess the usefulness of MR cholangiopancreatography (MRCP) with a half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequence as a noninvasive imaging technique to evaluate biliary or pancreatic disease in young children. SUBJECTS AND METHODS: We prospectively examined 45 children (mean age, 3 years) with MRCP using HASTE on a 1.5-T MR imaging unit. MRCP images were obtained in 1.4 sec without breath-hold. A body phased array coil and fat saturation technique were used for data collection. On the basis of the surgical (n = 19) and ERCP (n = 7) findings, clinical data, and follow-up observation, 21 children were deemed to have no significant abnormality. The other 24 children were found to have pancreaticobiliary disease, including congenital biliary atresia in five neonates, choledochal cyst in six infants, and anomalous connections between the bile and pancreatic ducts in four infants and nine young children. In children without abnormality, the MRCP images were graded for the depiction of normal structures. The findings obtained by MRCP were correlated with the surgical or ERCP results. RESULTS: HASTE MRCP showed the first branch of the intrahepatic duct, the common hepatic duct, the gallbladder, and the common bile duct in most children. In the 21 children with no abnormalities, the main pancreatic duct was visible in two of six neonates, in four of five infants, and in all 10 young children. The diagnostic accuracy of MRCP was 100% in patients with choledochal cysts, 100% in those with congenital biliary atresia, and 69% in those with anomalous connections between the bile and pancreatic ducts. CONCLUSION: MRCP using HASTE with a phased array coil is a noninvasive technique for revealing the pancreaticobiliary tract in young children.

Biliary Atresia↗

Laparoscopic diagnosis of appendicovesical fistula in a pediatric patient.

Appendicovesical fistula is a rare complication of acute appendicitis in children that is difficult to diagnose preoperatively. A 21-month-old boy with signs and symptoms of recurrent urinary tract infections underwent abdominal ultrasonography, computed tomography (CT), barium enema examination, and cystography, none of which was sufficient to establish a diagnosis; an appendicovesical fistula was demonstrated by laparoscopy. The patient underwent open appendectomy, with resection of the fistula through a small incision, and has recovered without complications.

Appendectomy↗

Resolution of cirrhosis-related pulmonary shunting in two children with a transplanted liver.

We treated two children with hypoxemia caused by intrapulmonary shunting associated with cirrhosis secondary to extrahepatic biliary atresia. Following orthotopic liver transplantation, digital clubbing and intrapulmonary shunting were resolved, as demonstrated by normalization of room air arterial blood gases, reduction in shunt fraction, and perfusion lung scanning with 99mTc-labeled macroaggregates of albumin. We recommend that early liver transplantation be considered for young subjects with a severe hepatopulmonary syndrome.

Biliary Atresia↗

Mass screening for neuroblastoma: quo vadis? A 9-year experience from the Pediatric Oncology Study Group of the Kyushu area in Japan.

Since 1985, a nationwide program of mass screening for neuroblastoma has been available for 6-month-old infants throughout Japan. From 1985 to 1993, the authors studied 285 patients with neuroblastoma among their regional population of 15 million. There was an increase in the total number of patients per year in comparison to the previous 6-year period (1979 to 1984). However, no significant difference was noted in the number of patients older than 1 year or in the incidence of advanced-stage (stages III and IV) unscreened cases. The majority of neuroblastomas in the screened group showed favorable biological factors, even in the advanced stages. However, there was a small group with histologically and/or biologically unfavorable factors; five of 115 had amplified N-myc oncogene, four of 74 showed unfavorable Shimada histological findings, and three of 33 had an unfavorable DNA ploidy pattern. One case from this group with unfavorable factors died of the tumor. 3) Thirty-eight cases were negative at the time of mass screening, but later presented with neuroblastoma. Most of them were diagnosed between 1 and 3 years of age, and 30 of the 38 cases (78.9%) were advanced stage with unfavorable prognostic factors. Thus, the authors conclude that mass screening at 6 months can detect a selected population of infants with neuroblastoma; some of the tumors may represent subclinical masses destined for spontaneous regression. However, some tumors with unfavorable factors have been detected by mass screening before progression and/or dissemination. Infants in this group are considered to benefit most from early diagnosis and treatment.

Adolescent↗

Laparoscopic surgery for neuroblastoma identified by mass screening.

Most neuroblastoma identified by mass screening are clearly different from the clinically diagnosed tumors with respect to biology, epidemiology, and outcome. Because the neuroblastomas detected by mass screening have favorable biological features (97% survival rate at 5 years), aggressive surgery may not be appropriate for such tumors. Laparoscopic adrenalectomy was performed on a 9-month-old boy and two 8-month-old girls who had suitable lesions. The tumors were small (< 20 mm in diameter). With the patients under general anesthesia, five trocars were placed in the abdomen. The abdominal wall traction method was used in addition to pneumoperitoneum. Intraabdominal pressure was maintained at below 4 mm Hg. The adrenal tumors were well encapsulated and completely excised, placed into a plastic bag, and removed through one of the 10-mm trocar sites. No lymphadenopathy was observed. The postoperative course was uneventful. The tumors were of favorable Shimada histology and had no N-myc gene amplification. The patients have survived, with no evidence of recurrence or metastasis, through the follow-up period of 17 to 22 months.

Adrenal Gland Neoplasms↗

Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus.

Three Japanese brothers with progressive deterioration of hepatic function and fatty degeneration of the liver eventually leading to hepatic encephalopathy were recently encountered. Each of them had a congenital intrahepatic portosystemic venous shunt due to a patent ductus venosus. Liver dysfunction and hepatic steatosis reverted to normal with surgical correction of the ductus venosus. Our observations suggest that impairment of liver function occurs first in the presence of malnutrition related to a reduction of blood in the portal vein. Hepatic steatosis is a consequence of a depleted metabolism of hepatocytes. Congenital portosystemic venous shunt due to a patent ductus venosus in this family has a genetic background.

Angiography↗

Malposition of a totally implantable venous access catheter in the azygous vein: report of a case.

We report herein the case of a 2-year-old girl with short bowel syndrome who developed chest pain 2 weeks after a totally implantable central venous access catheter had been surgically placed to facilitate total parenteral nutrition. A lateral chest X-ray and contrast flow study subsequently revealed that the catheter tip had been inserted into the azygous vein. Although this complication is very rare, it may be encountered more commonly with the increasing use of this type of venous access. Thus, we believe that a lateral chest X-ray should be routinely performed on all patients following catheter insertion to check for possible malposition of the tip in the azygous vein.

Catheters, Indwelling↗

Neuroblastoma in infants aged less than 6 months: is more aggressive treatment necessary? A report from the Pediatric Oncology Study Group of the Kyushu area.

Infants with neuroblastoma are known to have a better prognosis than older children. In Japan in 1985, mass screening for neuroblastoma in infants aged 6 months was introduced. With this policy, there has been an increase in the number of patients seen with neuroblastoma between 6 and 11 months of age. In a previous report the authors described the management and prognosis of infants with disease detected by mass screening, but there is still little information regarding the strategies of management for infants with neuroblastoma aged less than 6 months. The authors analyzed the data regarding 27 patients aged less than 6 months registered in their region (population 15 million) from 1985 to 1992, and compared it with that of the previous 8-year period. In the younger age group, there was a significantly higher rate of advanced disease stages (III and IV). In spite of the variation in treatment related to the choice of individual institutions, infants with stages I, II, and III disease had a good outcome, suggesting that aggressive chemotherapy is not necessary unless poor prognostic factors are present. One patient with stage IV disease died of disseminated disease and one with stage IVs and 22 copies of N-myc oncogene also died of tumor relapse in spite of aggressive chemotherapy. It is therefore concluded that the prognosis in infants with stage IV and IVs neuroblastoma under the age of 6 months is not as good as had previously been believed, and that such patients, therefore, require special consideration.

Antineoplastic Combined Chemotherapy Protocols↗

Gallbladder expansion induced by feeding.

By using ultrasound we were able to observe any increase in the size of human gallbladders during feeding. In 91 of 123 cases (74%), the gallbladder expanded for a short time prior to contraction during feeding. The examination on eight patients after gastrectomy and seven patients after a medically induced vagotomy revealed contraction only in response to feeding. In seven normal adults, 200 ml of air injected rapidly into stomach through a gastric tube was seen to induce gallbladder expansion. These results suggest that the gallbladder expands in response to feeding via a cholinergic vago-vagal reflex.

Adult↗

How to deal with advanced cases of neuroblastoma detected by mass screening: a report from the Pediatric Oncology Study Group of the Kyushu area of Japan.

Since 1985, a nationwide program of mass screening (MS) for neuroblastoma has been underway for 6-month-old infants throughout Japan. As a result, the number of patients with stage I or II disease has obviously increased, and this has resulted in overall improvement of the prognosis for neuroblastoma. Some cases detected by MS were already in an advanced stage and have also had a good prognosis. In such cases, no definitive treatment protocol has been developed. Therefore, the authors investigated (1) the clinical and biological features of the advanced neuroblastoma cases detected by MS and (2) the best way to deal with such cases. The authors analyzed 94 cases of advanced-stage neuroblastoma registered in the Kyushu area (population, 15 million) between 1985 and 1990. Eighteen cases (16 stage III, 2 stage IV) were found by MS, and the others (23 stage III, 53 stage IV) were diagnosed clinically. The following results were obtained: (1) No N-myc amplifications were observed in cases detected by MS, whereas 16 of the 45 examined patients in the non-MS group had high amplifications of N-myc. (2) With regard to Shimada's classification, DNA content, and S-100 protein positivity, most of the advanced tumors found by MS showed characteristics indicating a good prognosis. (3) The 5-year survival rate for the non-MS group is less than 25%, whereas all of the patients whose tumors were detected by MS are alive, even after undergoing mild chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

Laparoscopic cholecystcholangiography in a patient with biliary atresia.

A 24-day-old female newborn with persistent jaundice and acholic stool was diagnosed by laparoscopic cholecystcholangiogram. A catheter was inserted through a small incision at the fundus of the gallbladder. With definitive diagnosis of biliary atresia, laparotomy was performed, and Kasai's operation was carried out successfully.

Angiography↗