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Biomedical subjects

K Ueda

Publications and source records attributed to K Ueda.

At least 163 records · Page 9Linked to original sources

Moebius syndrome with central hypoventilation and brainstem calcification: a case report.

Moebius syndrome (MS) is described in an infant with central hypoventilation and brainstem calcification. The patient had limb defects and bilateral paralysis of the 6th, 7th, 9th, 10th, and 12th cranial nerves. Mechanical ventilation was continued from birth because of shallow spontaneous respiration. Computed tomography revealed brainstem atrophy and four small calcifications restricted to the dorsal portion of the pons and medulla. Prenatal brainstem injury such as ischaemia may have caused MS and central hypoventilation.

Abducens Nerve

Detection of multidrug-resistant protein, P-glycoprotein in childhood leukaemia and lymphoma.

Flow cytometric detection of surface P-glycoprotein, a multidrug-resistant gene product, with a monoclonal antibody, MRK 16, was performed on cells obtained from 18 children with leukaemia and lymphoma. Of 18 patients examined, 1 with malignant lymphoma at relapse showed a significant increase in P-glycoprotein-positive cells and a strong resistance to chemotherapy. Overexpression of P-glycoprotein in a case with B-cell type malignant lymphoma was confirmed by immuno-precipitation and Northern hybridization analysis. The present study suggests that an increased expression of surface P-glycoprotein might be involved in multidrug resistance at least in a certain case of childhood leukaemia and lymphoma.

ATP Binding Cassette Transporter, Subfamily B, Mem

Tumor necrosis factor in the cerebrospinal fluid of children with central nervous system leukemia.

To clarify the role of cytokines in cerebrospinal fluid (CSF) in the pathogenesis of central nervous system (CNS) leukemia, three cytokine activities, interleukin 1 (IL-1)-beta, tumor necrosis factor (TNF)-alpha, and interferon (IFN)-gamma, and their correlations with other laboratory studies of the CSF were analysed in 23 children with acute leukemia. These patients were classified into three groups: group A (n = 8)--patients with overt CNS leukemia, group B (n = 5)--patients with CNS leukemia in remission, group C (n = 10)--patients without CNS disease. IFN-gamma in the CSF was undetectable in these 23 patients. There was no difference in IL-1-beta levels among the three groups. However, TNF-alpha levels were significantly higher in group A than in group B, and higher in group B than in group C. By Kendall's rank sum test, high TNF levels in CSF correlated with high CSF leukemic cell counts and low sugar levels. In two patients with overt CNS leukemia, the TNF level in the CSF decreased gradually with intrathecal chemotherapy. These results indicate that TNF released from stimulated cells in the cerebrospinal space may induce CNS leukemia-related symptoms or alter laboratory parameters measured in the CSF. TNF levels in CSF may also prove useful in diagnosing early CNS involvement in children with acute leukemia.

Acute Disease

The late waves of brainstem auditory evoked potentials in children with cerebrovascular diseases.

We examined brainstem auditory evoked potentials (BAEPs) in 28 children with cerebrovascular diseases and 38 normal control subjects and investigated the neuroanatomic correlations of the late waves (VI and VII) of the BAEPs. The patients included 19 patients with moyamoya disease and 9 with other cerebrovascular accidents. Wave VI was more consistently seen (only missing in 2 cases, 5%-2 ears, 3%) than wave VII (missing in 26 cases, 68%-40 ears, 53%) in the control group. Absent, depressed or prolonged wave VI correlated well with cerebral lesions involving internal capsule and basal ganglia, while wave VII had a less significant correlation with any cerebral lesions in children with cerebrovascular diseases.

Adolescent

Cytogenetic and cellular characteristics of a human embryonal rhabdomyosarcoma cell line, RMS-YM.

A human tumour cell line, designated RMS-YM, was established from a childhood rhabdomyosarcoma. The monolayer cells were polygonal, round or spindle-shaped. The cells became multilayered and formed many focal piles when confluent. RMS-YM became stable with a doubling time of about 30 h and has been maintained for 104 passages to date. Tumourigenicity of the cells was confirmed by heterotransplantation into nude mice. Morphological features were similar to those of the primary tumour, and myofibrils were found by electron microscopy. The expression of desmin and human myoglobin, and high levels of striated muscle system specific enzymes were recognised. Chromosomal analysis revealed possible gene amplification in the form of homogeneously staining regions. Oncogene analysis was performed on the primary tumour and the cell line, but neither N-myc nor N-ras genes were amplified, nor were Ki-ras, Ha-ras or N-ras genes mutated at the 12th, 13th and 61st codons. The RMS-YM cell line may provide a system to identify novel genes which are amplified in rhabdomyosarcoma.

Animals

Well-differentiated hepatocellular carcinoma showing intrahepatic neural invasion: autopsied case.

An autopsied case of well-differentiated hepatocellular carcinoma (HCC), showing neural invasion into the portal tract of the liver, a hitherto undescribed lesion, is reported. The patient, a 68-year-old man, had had treatment for HCC over nine years, comprising surgical resection, transcatheter arterial embolization, transportal venous embolization and percutaneous ethanol injection to nodules of the HCC. At autopsy, many HCC nodules (less than 7 cm in diameter) were found in the liver. In one of them, carcinoma cells infiltrated the medium-sized protal tract among the regenerative nodules and some of these carcinoma cells further invaded nerve fibers. Although the majority of the HCC nodules showed ischemic necrosis, the infiltrating carcinoma cells in the portal tract were viable, suggesting the biological behavior and/or blood supply of these infiltrating carcinoma cells to be different.

Aged

The role of intrahepatic portal venous stenosis in the formation and progression of hepatolithiasis: morphological evaluation of autopsy and surgical series.

Recently, it has been suspected in animal models that a decrease in portal blood flow plays a role in the formation and progression of hepatolithiasis. To find whether this hypothesis is applicable to humans, we examined histologically the intrahepatic portal venous and arterial systems in normal livers (n = 13), extrahepatic biliary obstruction (n = 18), intrahepatic biliary sludge and microcalculi (n = 18, most of which were associated with biliary obstruction and might represent pathogenesis of an early developmental stage of hepatolithiasis), and fully developed hepatolithiasis composed of calcium bilirubinate stones (n = 30). A scoring method was employed to quantify portal stenosis, portal phlebosclerosis, arterial stenosis, and parenchymal atrophy. We found that these vascular changes were significantly more severe in hepatolithiasis than in biliary sludge and microcalculi, or in extrahepatic biliary obstruction. There were no significant differences in the vascular changes except for arterial stenosis between the latter two. There is a positive correlation between vascular stenosis and parenchymal atrophy. These findings suggest that portal venous stenosis deteriorates during the progression of hepatolithiasis. We could not find direct evidence that portal venous stenosis is an initial lesion followed by the formation of hepatolithiasis. The vascular changes may be caused by an inflammatory extension of cholangitis and may deteriorate, causing parenchymal atrophy during the progression of hepatolithiasis.

Aged

Selective necrosis of encapsulated malignant lesion within atypical adenomatous hyperplasia of the liver following transarterial embolization. A report of two autopsy cases.

We report here the morphology of two nodules of atypical adenomatous hyperplasia (AH), a preneoplastic or early developmental stage of hepatocellular carcinoma (HCC), with a fibrously encapsulated malignant lesion occurring in two cirrhotic livers. The two patients had been treated for HCC by transarterial embolization. At autopsy, HCC nodules and several AH nodules were found in each case. Microscopically, two of the several AH nodules contained malignant lesions that showed selective coagulative necrosis: the hepatocytes of the nonmalignant parts of the two AH nodules were viable. The malignant lesions within the atypical AH nodules were surrounded with a fibrous capsule, and the majority of HCC nodules were necrotic; AH nodules themselves, except for malignant lesions, were viable. This suggests to us that there are differences in blood supply between the malignant lesions and surrounding tissue of atypical AH: malignant lesions within atypical AH may be supplied mainly by arterial blood, whereas nonmalignant areas of atypical AH may be dually supplied by both arterial and portal blood. Alternatively, it may be that the malignant lesions in atypical AH are more susceptible to hypoxia caused by transarterial embolization.

Adenoma

Carcinoma of the colon in children: a case report and review of 41 Japanese cases.

We report a case of sigmoid colon carcinoma in a 14-year-old girl. She had been suffering from several nonspecific abdominal symptoms for about 1 year before a definite diagnosis was made. While undergoing an operation, the main tumor was found to be unresectable. She died of Candida-induced septicemia on the 26th day after the operation. There have been 40 cases of colon carcinoma reported in the Japanese literature in patients 15 years of age or younger. We reviewed the 40 Japanese cases along with our case, and compared these to other children and adult studies. The prognosis is quite unfavorable in children because of the poor histological character and the delayed diagnosis. Serum carcinoembryonic antigen measurement might be one means for early diagnosis.

Adolescent

Biocompatibility of radiolucent breast implants.

Current implants for breast augmentation containing silicone gel, saline, or both are radiopaque on mammographic examination and can totally obscure microcalcifications and soft-tissue masses. The effect of these implants on the detection of early breast cancers in patients who have undergone augmentation mammaplasty remains unproven and controversial. Implants filled with medium-chain triglycerides (peanut oil) are radiolucent on mammographic examination and allow visualization of both soft-tissue masses and microcalcifications. To investigate the biocompatibility of radiolucent implants, 10 cc of sterile, nonpyrogenic peanut oil was injected subcutaneously into rats using silicone gel as a control. Twenty-one rabbits had two 125-cc silicone shell implants inserted on either side of the chest wall. The right-sided shell was filled with 125 cc of sterile saline, and the left-sided shell was filled with 125 cc of sterile, nonpyrogenic peanut oil. Results were determined by both histologic and radiographic examination. Rats injected with peanut oil equivalent to 7 percent of their body weight rapidly absorbed the freely injected oil without detriment. Histologic examination of the lungs, liver, kidneys, and tissues adjacent to the injection sites demonstrated no abnormalities. There was no evidence of allergic, toxic, inflammatory, or neoplastic response. Eighteen of 21 rabbits survived more than 3 months. Radiographs showed the oil-filled implants to be radiolucent, whereas the saline-filled controls obscured the surrounding soft and bony tissues. Histologic examination demonstrated a fibrous capsule surrounding both types of implants. Histologic examination of the lungs, liver, and kidneys showed no significant abnormalities. These and previous studies have shown peanut oil to be biocompatible when freely injected either intramuscularly or subcutaneously. This study demonstrates that a radiolucent, peanut oil-filled implant is biocompatible in animals and that further long-term studies for its use in humans are merited.

Animals

Dupuytren's contracture.

Dupuytren's contracture is a disease of the palmar and digital fascia that results in a flexion deformity of the fingers. Although the first case was reported more than 350 years ago, many questions remain unanswered. With our present state of knowledge, treatment remains empiric and operative intervention is the only successful mode of treatment. Controversies continue over the type of procedure used, as well as its timing. In light of the high rate of recurrence of flexion contractures after apparently successful operations, current research is being directed toward identifying a biochemical or histologic marker to indicate the probability of recurrence.

Dupuytren Contracture

Congenital factor VII abnormality discovered in an infant at a routine checkup.

Congenital coagulation factor VII abnormality in an infant was first discovered because of a decreased normotest value at a routine health checkup at the age of 1 month. No bleeding tendency had been noticed. The normotest value did not respond to an administration of vitamin K. The prothrombin time (PT) was prolonged, and the activated partial thromboplastin time (A-PTT) was in the normal range. Factor VII activity was extremely low, whereas the level of factor VII antigen was relatively low. These data indicate that the propositus is a factor VII reduced variant, a rare variant of congenital factor VII deficiency. The propositus and her brother are homozygotes, and her parents and sister are heterozygotes.

Blood Coagulation Tests

Etiology of rabbit haemorrhagic disease spontaneously occurring in Korea.

Causative agent of rabbit haemorrhagic disease (RHD) was purified by CsCl density gradient centrifugation from the liver homogenate of rabbits infected with RHD virus which originated from Korea. The viral particles were 35-40 nm in diameter, and had hollow depressions on their surface. Protein A-gold immunoelectron microscopy clearly showed that the convalescent antisera of diseased rabbits reacted specifically with the virus particles. SDS-PAGE and Western blot analyses demonstrated that the structural protein of the virus was composed of a single major polypeptide of 63 kD. These findings indicate that the causative agent of RHD, tentatively named as picornavirus in Korea, belongs to calicivirus.

Animals

Efficacy of once weekly erythropoietin therapy in children on continuous ambulatory peritoneal dialysis.

The efficacy of recombinant human erythropoietin (rHuEPO) on 10 anemic children undergoing continuous ambulatory peritoneal dialysis (CAPD) was evaluated. A mean dose of 93 U/kg of rHuEPO was given once a week for 24 weeks, either intravenously to 5 children or subcutaneously to the other 5. Anemia improved in all children by this therapy. The mean hemoglobin and hematocrit levels increased gradually but significantly from 6.9 g/dl and 20.2% to 9.4 g/dl and 30.1%, respectively, at the end of the study. Elevation of blood pressure or other side effects were not noted in any patient. The present study indicates that the once-a-week injection of rHuEPO by the intravenous or subcutaneous route is effective in children undergoing CAPD.

Adolescent

Late effects of childhood acute leukemia and its treatment.

Late effects of childhood acute leukemia and its treatment were studied in 776 patients (684 ALL, 73 ANLL, and 9 others) in Japan who had remained in remission for more than 1 year after their first complete remission. Delayed adverse sequelae involve a wide variety of organs and their functions. Short stature was present in 2.61%, obesity in 3.79%, abnormalities of growth hormone secretion in 1.5%, delayed secondary sex characteristics in 1.5% of males and 0.6% of females, motor disturbances in 1.17%, sensory disturbances in 0.91%, intellectual and learning disabilities in 2.48%, abnormal findings in routine neurologic examinations in 1.31%, EEG abnormalities in 4.30%, brain CT abnormalities in 5.09% and cardiac dysfunction in 1.07%. Various other disorders were seen in 20 patients. Many of these delayed adverse sequelae are caused by or related to central nervous system prophylaxis and systemic combination chemotherapy. The results suggest that it is needed to improve therapeutic methods through the stratification of patients by risk factors and detailed analysis of prognostic factors. Moreover it is important to render medical and psychosocial support to long-term survivors of childhood leukemia through interactions between the patient, parents and medical staff.

Adolescent