Biomedical subjects

S Suita

Publications and source records attributed to S Suita.

210 records · Page 12Linked to original sources

PubMedDate not supplied

Treatment results of advanced neuroblastoma with the first Japanese study group protocol. Study Group of Japan for Treatment of Advanced Neuroblastoma.

PURPOSE: To elucidate the efficacy of intensive induction and consolidation chemotherapy regimens (Study Group of Japan for Advanced Neuroblastoma [JANB] 85) for patients with advanced neuroblastoma aged 1 year or older. PATIENT AND METHODS: One hundred fifty-seven patients with newly diagnosed advanced neuroblastoma were entered into this study between January 1985 and December 1990. Eligible patients were 12 months old or older with stage III or IV disease. The patients first received six cyclic courses of intensive induction chemotherapy (designated regimen A1) consisting of cyclophosphamide (1,200 mg/m2), vincristine (1.5 mg/m2), tetrahydro-pyranyl Adriamycin (pirarubicin; 40 mg/m2), and cisplatin (90 mg/m2). The patients were further treated with three different consolidation protocols: 3-[(4-amino-2-methyl-5-pyrimidinyl)methyl]-1-(2-chloroethyl)-1-nitrosour ea, dacarbazine, and bone marrow transplantation. RESULTS: Overall survival rates for patients with stage III disease without reference to the consolidation protocols were 80.8%, 76.9%, and 66.3% at 2, 5, and 10 years, respectively. The overall survival rates for patients with stage IV disease were 58.8%, 34.4%, and 28.9% at 2, 5, and 10 years, respectively. There were no statistically significant differences between the three consolidation treatment groups. Patients who did not achieve complete remission (CR) with induction chemotherapy and surgery all died, suggesting that CR is essential for the cure of advanced neuroblastoma. The overall 5-year survival rate of the 24 patients with N-myc amplified stage III and IV disease was 33.3%, and the longest survival time of a relapse-free patient was 103 months. CONCLUSION: The intensive induction chemotherapy regimen used in this study may be of significant value in increasing the CR rate and survival for patients with N-myc amplified and nonamplified advanced neuroblastoma.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Outcome of fetal cystic hygroma and experience of intrauterine treatment.

OBJECTIVE: To review our cases of fetal cystic hygroma and to examine the prognostic factors with the goal of establishing criteria for the intrauterine treatment for cystic hygroma. PATIENTS AND METHODS: Thirty-one cases of fetal cystic hygroma were managed by us from January 1988 to December 1997, and 21 cases were available for analysis. Three prognostic factors, namely chromosomal abnormality, structural anomaly and hydrops fetalis, were evaluated. We treated 2 cases of cystic hygroma associated with hydrops fetalis in utero using OK-432 injection under ultrasound guidance. RESULTS: The fetuses without any of the prognostic factors listed above showed a good prognosis throughout the fetal and neonatal periods. However, in this group, 2 infants with large tumors died of hemorrhage from the tumor at 6 months and 3 years of age, respectively. Cases with hydrops fetalis without chromosomal abnormalities or structural anomalies (5 cases) resulted in either intrauterine fetal death (IUFD, 2 cases) or early perinatal neonatal death (early PND, 3 cases). The cause of early PND was circulatory failure. Most of the hydrops cases with either a chromosomal abnormality or structural anomaly resulted in IUFD before 22 weeks of gestation. The size of the cyst decreased in 1 of 2 cases treated in utero. CONCLUSIONS: The fetal cases of cystic hygroma showing hydrops fetalis without chromosomal abnormalities or structural anomalies are considered to be possible candidates for intrauterine therapy. Those with very large cystic hygroma without any of the three prognostic factors are also thought to be candidates for fetal treatment. Based on our clinical experience, sclerotherapy using OK-432 is considered to be a treatment option in selected cases with fetal cystic hygroma.

Chromosome Aberrations↗

PubMedDate not supplied

In utero evaluation and the long-term prognosis of living infants with cystic hygroma.

OBJECTIVE: The aim is to evaluate the natural course and the long-term prognosis of cystic hygroma following live birth. METHODS: Of 39 cases, 12 were excluded because of either artificial termination or intrauterine treatment. 27 cases were retrospectively classified into 2 groups based on the outcome, and the clinical profiles were compared. The long-term prognosis was analyzed for live births. RESULTS: 5 (18.5%) and 22 (81.5%) cases resulted in live birth and fetal demise, respectively. Both the frequency of hydrops fetalis and the maximum size of the cyst were more significantly associated with a poor prognosis (p < 0.01). Of 5 live births, 3 cases with chromosomal or structural abnormalities were handicapped. CONCLUSIONS: Hydrops fetalis and cyst size are poor prognostic factors, and fetal chromosome and structural malformations should be taken into account when predicting the long-term prognosis.

Chromosome Aberrations↗

PubMedDate not supplied

How antenatal ultrasound diagnosis of congenital malformations has contributed to fetal outcome: a 22-year review.

We herein reviewed 630 malformed fetuses delivered from 24 weeks of gestation onwards in our institute over the past 22 years. These fetuses were divided into 2 groups: 210 from 1970 to 1982 (group 1) and 420 from 1983 to 1991 (group 2). Twenty-two varieties of congenital malformations were diagnosed antenatally in group 1, whereas 55 additional malformations became diagnosable in group 2. Thirty-four varieties of congenital malformations still remained undiagnosed throughout the 22-year study period. Cases receiving 'fetal therapy' and 'close obstetric care' increased, with statistical significance, from 1.8 to 15.7%, and from 22.9 to 55.7% in groups 1 and 2, respectively. A significant increase was also noted in the survival rate from group 1 (63.3%: 105/166) to group 2 (75.5%: 259/343). This study has revealed that the steady advancement in antenatal diagnosis of congenital malformations, along with great efforts in terms of intensive care, has contributed to improved fetal outcome.

Apgar Score↗

PubMedDate not supplied

Antenatal diagnosis and surgical management of congenital cystic adenomatoid malformation of the lung.

We experienced 12 cases of congenital cystic adenomatoid malformation of the lung (CCAM) including 6 cases diagnosed antenatally. They were classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 3), group B presented with respiratory distress symptoms after birth (n = 6), and group C showed no respiratory symptoms (n = 3). All cases of group A were lost because of hydrops and respiratory failure due to pulmonary hypoplasia. Because a compression of the mass is thought to be a cause of hydrops, this group is considered to be a good indication for fetal treatment. All cases of group B showed progressive respiratory symptoms a few days after birth which were successfully treated surgically. In 2 of 3 cases of group C, the lesions decreased in size both antenatally and postnatally. We conclude that serial sonographic evaluations for fetal CCAM are important. If the fetus develops hydrops, fetal surgery is to be considered. If not, however, fetal surgery should not be done, because some lesions can shrink in size, or even disappear, while others can be treated successfully after birth by lobectomy or even segmentectomy.

Cystic Adenomatoid Malformation of Lung, Congenita↗

PubMedDate not supplied

Clinical characteristics and natural history of antenatally diagnosed fetal uropathy. An analysis of 55 cases.

The clinical characteristics and natural history of 55 cases with antenatally diagnosed fetal uropathy were investigated. Percutaneous aspiration of the fetal pelvic or vesical urine was performed to decompress progressive unilateral hydronephrosis in 2 cases and to evaluate renal function in another 2 cases of bilateral hydronephrosis. As the postnatal diagnosis, upper urinary tract dilatation (hydronephrosis or hydronephroureter, 33 cases) and renal dysplasia (15 cases) made up 87% of all cases. A combination of hydronephrosis in one kidney and renal dysplasia in the other was also found in another 2 cases. Among 35 cases with upper urinary tract dilatation, 27 cases demonstrated pelviureteric junction stenosis and surgical intervention was necessary in 15 cases. In 17 cases with renal dysplasia, spontaneous regression was observed in only 3 cases and surgical intervention by means of percutaneous nephrostomy and nephrectomy was performed in 4 and 6 cases, respectively.

Constriction, Pathologic↗

PubMedDate not supplied

Manganese intoxication during intermittent parenteral nutrition: report of two cases.

BACKGROUND AND METHODS: The administration of trace elements is thought to be needed in patients receiving long-term parenteral nutrition. Recently, manganese intoxication or deposition was documented in such patients. We report two cases of manganese intoxication during intermittent parenteral nutrition including manganese. Manganese had been administered for 4 years at a frequency of one or two times per week in one case and for 5 years at a frequency of one or two times per month in the other case. Both cases showed mild symptoms with headache and dizziness. One case had mild hepatic dysfunction and the other did not. The whole-blood manganese level increased in one case, but not in the other case. T1-weighted magnetic resonance images revealed symmetrical high-intensity areas in basal ganglia and thalamus in both cases. After the administration of manganese was stopped, these symptoms all disappeared and the magnetic resonance images abnormalities gradually improved in both patients. Mild long-term manganese intoxication is thus considered to occur regardless of the frequency of using a manganese supplement. CONCLUSIONS: Patients should be carefully monitored when receiving long-term parenteral nutrition including manganese, even when the manganese dose is small and the frequency of receiving a manganese supplement is low.

Adolescent↗

PubMedDate not supplied

Cholelithiasis in infants: association with parenteral nutrition.

Reported herein are two cholelithiasis-afflicted infants with a common inability to tolerate enteral feeding necessitating parenteral nutrition. One of the infants received parenteral nutrition for 5 months because of intestinal dysfunction and enterocolitis secondary to extensive aganglionosis, while the second child was premature and placed on parenteral nutrition because of bowel dysfunction after surgery to repair high jejunal atresia and a right diaphragmatic hernia. The relationship between parenteral nutrition and cholelithiasis is discussed.

Cholelithiasis↗

PubMedDate not supplied

Requirement of parenteral fat in infants with biliary atresia.

In a prospective study on infants with biliary atresia (BA) we measured the composition of the plasma fatty acid to determine the requirement of fat emulsion. In 14 infants who underwent initial operation for treatment of BA, a trend toward essential fatty acid (EFA) deficiency was evident before the surgery. Fourteen infants given parenteral nutrition (PN) after surgery were grouped into three according to the dose of fat emulsion, fat free in 4, 10% of the total calories in 5 and 20% of the total calories in 5. After operation, EFA deficiency gradually progressed when on fat free PN. Improvement in EFA deficiency occurred with the administration of fat which corresponded to 20% of the total caloric intake. During PN including fat emulsion, abnormal changes in liver function tests and serum lipid values due to fat emulsion were nil. Infusion of fat which supplies 20% of the total calories is thus recommended for correction of EFA deficiency and is tolerable in infants with obstructive liver disease. However, clinical and biochemical monitoring should be carefully done.

Bile Ducts↗

PubMedDate not supplied

Complications in neonates with short bowel syndrome and long-term parenteral nutrition.

BACKGROUND: Because of advances in parenteral nutrition (PN), the prognosis for patients with short bowel syndrome (SBS) has recently shown great improvement. Even infants with a small bowel measuring only 20 cm either with or without an ileocoecal valve can now survive. However, because of the increased periods of PN, severe complications associated with PN have been observed. PATIENTS AND METHODS: We analyzed 17 patients with SBS treated by long-term PN to investigate the complications of PN, particularly hepatic dysfunction, micronutrient deficiency, and intoxication. RESULTS: Eleven of 17 patients (64.7%) had hepatic dysfunction, mostly cholestasis. The main causes of hepatic dysfunction were loss of enteral feeding and infections. Regarding the complications of the micronutrients, zinc and copper deficiencies have been observed less often because of trace element supplements. However, manganese deposition, especially in the basal ganglia, was recognized in one patient. CONCLUSIONS: We therefore need to pay more attention to these problems when treating this disease in order to improve the overall prognosis significantly.

Cholestasis↗

PubMedDate not supplied

New technique of experimental partial liver transplantation: hemodynamic study during surgery.

Orthotopic liver transplantation of children is often limited by the scarcity of an appropriately size-matched donor organ. The transplantation of a reduced liver was thus conceived to solve this problem. A new technique of partial liver transplantation in mongrel dogs is reported, which partially retains the recipient's liver and uses an Ameroid constrictor to occlude the recipient's portal branch gradually. The donor's right and median lobectomy was performed in situ. The donor's left lobe was perfused in situ and removed as a graft. It was then transplanted orthotopically in the space after a left and median lobectomy of the recipient. An Ameroid constrictor was applied around the recipient's right portal branch for a gradual occlusion of the host hepatic blood supply (the Ameroid group). To compare the hemodynamic stability during surgery with this technique, two other methods were also performed. The mean systemic blood pressure of orthotopic liver transplantation with a passive venovenous bypass during the anhepatic period (the Bypass group, n = 5) was lower than that of the Ameroid group (n = 16). Instead of fitting an Ameroid constrictor, an orthotopic partial liver transplantation with ligation of the right portal branch (the Ligation group) was performed. The mean systemic blood pressure of the Ligation group (n = 10) was lower than that of the Ameroid group (n = 11) after either fitting a constrictor or performing a ligation.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMedDate not supplied

Establishment and characterization of malignant rhabdoid tumor of the kidney.

Malignant rhabdoid tumor of the kidney (MRTK) is a highly aggressive tumor which occurs in childhood and which is histologically characterized by the existence of eosinophilic intracytoplasmic inclusions. We established and characterized a cell line from this tumor with histological, immunohistochemical and cytogenetical analysis. Histologically, the tumor cells demonstrate typical eosinophilic inclusions, while immunohistochemically the cells demonstrate common mesenchymal and epithelial differentiation. Although the conventional karyotyping of this tumor lacked the abnormalities of 22q chromosome, Southern blot analysis and microsatellite analysis verified abnormalities of the BCR gene and of the hSNF5/INI1 gene. Despite the variety of locations, these common genetic abnormalities appear to contribute to distinguish rhabdoid tumor from such other small round cell tumors as primitive neuroectodermal tumor, rhabdomyosarcoma, poorly differentiated synovial sarcoma and desmoplastic small round cell tumor.

Animals↗