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Biomedical subjects

J Fukushige

Publications and source records attributed to J Fukushige.

At least 19 recordsLinked to original sources
PubMed1998

Prevention of hypoglycaemia in a patient with type Ib glycogen storage disease by an amylase (alpha-glucosidase) inhibitor.

Patients with type Ib glycogen storage disease (GSD Ib) are susceptible to hypoglycaemic episodes. To determine whether an amylase (alpha-glucosidase) inhibitor, voglibose, can be useful in the control of hypoglycaemia, we tried it in a 14-y-old male with GSD Ib. Oral administration of voglibose prolonged the duration of normoglycaemia and reduced the incidence of hypoglycaemia attacks. These findings indicate that voglibose may be useful for preventing hypoglycaemia in GSD Ib patients.

Adolescent↗
PubMed1998

Perinatal management of congenital complete atrioventricular block: report of nine cases.

BACKGROUND: The addition of fetal ultrasonography has allowed the prenatal diagnosis and observation of congenital complete atrioventricular block (CCAVB). Thus, the management of the affected fetuses should be modified accordingly. METHODS: The medical records were reviewed to identify patients with CCAVB, and clinical and laboratory data were collected. RESULTS: Nine patients with CCAVB, diagnosed prenatally, were identified. The gestational age at the time of diagnosis ranged from 18 to 38 weeks (median, 25 weeks). The gestational age and birthweight ranged from 29 to 41 weeks and from 1420 to 4075 g, respectively. An accompanying congenital cardiac anomaly was noted in three cases, including one with polysplenia syndrome. The heart rate at birth ranged from 30 to 80 bpm. In three neonates, isoproterenol was given for bradycardia. One fetus with hydrops fetalis associated with complex heart disease was treated with maternally administered digoxin, with resolution of the fluid accumulation. A permanent pacemaker was implanted in six cases: two within 1 day of birth; two during the neonatal period; one at 51 days; and the other at 2 years and 11 months of age. There were no deaths or major complications and all have remained well beyond their infancy. CONCLUSIONS: With the improvement of diagnostic methodology, more cases with CCAVB, including those with complex heart disease, could be diagnosed during fetal life. Their outcome has been steadily improving with the advances in perinatal management, pacemakers and implant techniques, irrespective of associated structural cardiac defects.

Abnormalities, Multiple↗
PubMed1998

Diagnosis and management of partial anomalous pulmonary venous connection. A review of 28 pediatric cases.

Medical records, angiograms and operative records of 28 patients with partial anomalous pulmonary venous connection (PAPVC) were reviewed. Twenty patients had one anomalous pulmonary vein (APV), and 8 had more than two APVs. Twenty-five patients (89%) had APVs originating from the right lung, 2 (7%) from the left lung and 1 (4%) from both lungs. In the 25 patients with APVs originating from the right lung, 9 had APVs draining into the superior vena cava (SVC), 13 into the right atrium (RA), 1 into the inferior vena cava (IVC) and 2 into both the SVC and RA. In the 2 patients with APVs originating from the left lung, 1 had APVs draining into the RA, and the other had APVs draining into the innominate vein. The patient with APVs originating from both lungs had connection to the IVC. Twenty-three patients (82%) had additional cardiovascular defects. Surgery was performed in 13 patients who had pulmonary/systemic flow ratios greater than 2.0. The patients have done well after surgery. In 7 patients, we were unable to accurately determine the number or sites of drainage of APVs prior to surgery. We conclude that selective pulmonary angiography is indispensable for the accurate diagnosis of PAPVC.

Adolescent↗
PubMed1997

An asymptomatic infant with isolated 3-methylcrotonyl-coenzyme: a carboxylase deficiency detected by newborn screening for maple syrup urine disease.

UNLABELLED: We describe an asymptomatic male infant with isolated 3-methylcrotonyl-coenzyme A carboxylase deficiency who came to medical attention by newborn mass screening due to elevated blood leucine. The diagnosis was made by abnormal urinary organic acids at 20 days of age and was confirmed by assay of the carboxylase activities in cultured skin fibroblasts. CONCLUSION: More attention should be paid to slight elevations of leucine levels in newborn mass screening. Urinary organic acid analysis should be performed in conspicuous cases.

Carbon-Carbon Ligases↗
PubMed1997

Blood pressure measurement in preschool children: the Hisayama Study.

OBJECTIVE: To establish the blood pressure (BP) measurement protocol for Japanese preschool children, systolic BP (K1) and diastolic BP (K4, K5) were measured along with anthropometric values. METHODOLOGY: Commercially available mercury sphygmomanometers were used. In the first group of children (group A), BP was measured in 79 boys and 85 girls using a cuff 9 cm wide and 23 cm long. In the second group (group B), the length and circumference of the upper right arm of 147 boys and 139 girls were measured to select cuffs appropriate for their sizes. Blood pressure measurements were performed twice on the right arm of the children in the seated position at a mean interval of 5 min. RESULTS: There were no significant differences in the anthropometric values (height, bodyweight, body mass index [BMI], length and circumference of the upper arm) between groups A and B. The BP values at K1, K4 and K5 in the first measurement of group B were 91 +/- 9, 54 +/- 8, and 48 +/- 10 mmHg (boys) and 90 +/- 9, 54 +/- 8, and 48 +/- 12 mmHg (girls), respectively. There were no significant differences between the first and second measurements in both groups, however, there were significant differences in the first and second measurements of K4 between groups A and B. Multiple regression analysis by the stepwise method revealed a strong correlation between K1 and the length of the upper arm in the boys and the bodyweight in the girls: between K4 and the bodyweight in the boys and the BMI in the girls, and between K5 and the height in the boys and the upper arm circumference in the girls. CONCLUSIONS: From these results it would appear that a single measurement is sufficient under appropriate measurement conditions such as rest before measurement and the choice of the cuff size according to the upper arm circumference, and that BP is closely correlated with the anthropometric values in preschool children.

Anthropometry↗
PubMed1997

Anthracycline-induced cardiotoxicity in children with malignancies.

The anthracyclines (ATC) have been used in the treatment of many pediatric patients with malignancies. Their use, however, has been limited by a serious and potentially irreversible cardiotoxicity. The medical records of pediatric patients with malignancies who received ATC at the Department of Pediatrics, Kyushu University, from January 1985 to December 1994 were reviewed. Among the 120 children with malignancies who received ATC, six patients (5%) developed congestive heart failure, 33 (28%) had subclinical cardiac dysfunction, whereas 81 (66%) showed no evidence of cardiac dysfunction. The incidence of ATC cardiotoxicity increased at higher cumulative doses. The earliest cardiotoxicity, however, was observed in a patient who received 180 mg/m2 unassociated with other risk factors, showing the marked individual variations in its development. Children younger than 4 years had a higher incidence of cardiotoxicity compared with those older than 4 years (P < 0.01) and are therefore more vulnerable to ATC cardiotoxicity. Because serial myocardial biopsies and stress testing are not practical in younger children, and radionuclide angiogram is rather costly and not appropriate for frequent evaluation, non-invasive tests such as echocardiography and electrocardiography (ECG) still play the main role for serial cardiac monitoring in these age groups. Radionuclide angiogram should be added as the confirmatory test modality.

Antibiotics, Antineoplastic↗
PubMed1997

Life expectancy and social adaptation in individuals with Down syndrome with and without surgery for congenital heart disease.

Life expectancy and social adaptation in 373 children with Down syndrome with and without congenital heart disease (CHD) were assessed retrospectively. Survival at age 24 years was 92.2% for patients without CHD (n=200), and 74.6% for those with CHD (n=173). Survival for those who underwent operation for cardiovascular lesions (n=95) was 87.8%, and for those not operated on despite hemodynamically significant cardiovascular lesions (n=39), it was 41.4%. Cardiac functional capacity was better in the children without congenital heart disease and in the group operated on, where most patients also attained good social adaptation. We conclude that children with Down syndrome with congenital heart disease should undergo early cardiac evaluation and surgery if indicated.

Down Syndrome↗
PubMed1996

Interferon-alpha therapy for multiple hemangiomas associated with coarctation of the aorta.

We report a rare combination of multiple hemangiomas and coarctation of the aorta in a 2-month-old female infant who responded to interferon-alpha (IFN-alpha) therapy. The coarctation was repaired successfully with the use of an artificial graft. In patients with symptomatic hemangiomas that do not respond to steroid therapy, IFN-alpha therapy should be considered.

Antineoplastic Agents↗
PubMed1996

Serum cholesterol levels in school-aged Japanese children: the Hisayama study.

Hypercholesterolemia has been known to be an important factor in the development of atherosclelosis. Blood cholesterol screening and related health education in children, however, have not yet been widely practiced in Japan. From 1985 to 1990, blood samples were obtained from 5825 school children aged 6 to 14 years residing in Hisayama, Japan. The mean total cholesterol (TC), triglyceride (TG), high-density lipoprotein cholesterol (HDL-C) and low-density lipoprotein cholesterol (LDL-C) levels were determined. The mean TC levels ranged from 155 to 172 mg/dL for boys and from 156 to 170 mg/dL for girls, peaking at 9 years for both sexes. The TG levels also tended to increase gradually and to peak at 11 years for both sexes. The tendency for TG levels to be higher was much clearer than in US children and adolescents. The HDL-C levels were highest at 9 years of age for both sexes and the LDL-C levels also tended to peak at 9 years of age for boys and at 8 years of age for girls. Atherogenic Indices [(TC-HDL-C)/HDL-C] ranged from 1.7 to 1.9 for boys and 1.8 to 2.0 for girls. As the cholesterol level of Japanese children would be expected to rise steadily reflecting their westernized lifestyle, preventive programes on a nationwide base including health education at school environments should be emphasized.

Adolescent↗
PubMed1996

The mechanism of comparable serum cholesterol lowering effects of pravastatin sodium, a 3-hydroxy-3-methylglutaryl coenzyme A inhibitor, between once- and twice-daily treatment regimens in beagle dogs and rabbits.

In dogs, no significant difference in the reduction of serum cholesterol was observed among three dosing regimens of pravastatin: once in the morning (3 mg/kg), once in the evening (3 mg/kg), and twice-daily (1.5 mg/kg x 2) for 21 days. In rabbits, pravastatin was administered at a dose of 50 mg/kg once-daily given in the evening or 25 mg/kg twice-daily for 14 days; the respective serum and liver cholesterol levels were decreased by 41% and 7% in the once-daily dosing and by 51% and 11% in the twice-daily dosing. The amount of low density lipoprotein (LDL) receptor protein was increased 1.2-1.3-fold (P < 0.05) by both treatments, and no significant difference was noted between these treatment regimens. In addition, there was no significant difference in the extent of up-regulated LDL receptor protein between once-daily dosing in the evening and once-daily dosing in the morning. In the experiments with rabbit hepatocytes, the up-regulated LDL receptor activity induced by preincubation with pravastatin was retained even 24 hr after the removal of pravastatin. These results suggest that the comparable efficacy of pravastatin between once- and twice-daily treatment could be explained by retention of up-regulated LDL receptor activity for more than 24 hr in vitro and in vivo.

Administration, Oral↗
PubMed1995

The mechanism of lack of hypocholesterolemic effects of pravastatin sodium, a 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor, in rats.

In order to clarify the reason why pravastatin, a 3-hydroxy-3-methylglutaryl (HMG)-CoA reductase inhibitor, did not show hypocholesterolemic effects in rats, the changes of various parameters affecting the serum cholesterol levels by pravastatin were determined in rats and rabbits, as a comparison. In rabbits, pravastatin administration at 50 mg/kg for 14 days decreased serum and liver cholesterol by 40% and 8%, respectively. The hepatic LDL receptor activity was increased 1.7-fold, and VLDL cholesterol secretion was decreased. Cholesterol 7 alpha-hydroxylase activity was not changed. In contrast, in rats, serum cholesterol was increased by 14% at 50 mg/kg and 27% at 250 mg/kg for 7 days, respectively. At 250 mg/kg, liver cholesterol was significantly increased by 11%. Under these conditions, neither the hepatic LDL receptor activity nor cholesterol 7 alpha-hydroxylase was changed, and VLDL cholesterol secretion was increased. At 250 mg/kg, net cholesterol synthesis in rat liver was increased after 7 days of consecutive administration. These results imply that in rats, stimulated net cholesterol synthesis caused the increase of liver cholesterol followed by the increase of VLDL cholesterol secretion, and resulted in the raise of plasma cholesterol. Although hepatic HMG-CoA reductase was induced almost the same fold in both animals at 50 mg/kg, the induced HMG-CoA reductase activity in rats might overcome the inhibitory capability of pravastatin, resulting in an increase of net cholesterol synthesis, but not in rabbits. This over response to pravastatin in rats might cause the lack of hypocholesterolemic effects of this drug.

Animals↗
PubMed1995

Patent ductus venosus associated with a hyperintense globus pallidum on T1-weighted magnetic resonance imaging and pulmonary hypertension.

UNLABELLED: We report the case of a 13-year-old Japanese boy with a patent ductus venosus. He experienced mild disorientation and hallucination at age 8 years. Hyperammonaemia was discovered at age 12 years. Brain MRI demonstrated multiple intracranial hyperintense lesions, mainly in the globus pallidum, which suggested portosystemic encephalopathy. Patent ductus venosus was demonstrated by abdominal ultrasonography and angiography. Cardiopulmonary investigation revealed pulmonary hypertension. An intracranial hyperintense lesion observed on T1-weighted MRI may be an initial clue for discovering a patent ductus venosus in asymptomatic patients. CONCLUSION: When patent ductus venosus is disclosed, pulmonary hypertension should be sought, as in cases with other portosystemic shunts.

Adolescent↗
PubMed1995

Histiocytic haemophagocytosis in a patient with Kawasaki disease: changes in the hypercytokinaemic state.

UNLABELLED: A 32-month-old Japanese boy exhibited haemophagocytic syndrome (HPS) during the recurrent course of Kawasaki disease. Despite repeated gamma-globulin therapy, he developed cytopenia with marked hepatomegaly and evidence of histiocytic haemophagocytosis in the bone marrow. Serum levels of interferon-gamma and tumour necrosis factor, but not of interleukin-1 beta, increased in parallel with his symptoms. No confirmation was obtained of the association of toxic reactions to the used drugs. No coronary lesions remained as sequelae. CONCLUSION: Cytopenia in Kawasaki disease could herald HPS, and the hypercytokinaemia involved in the two febrile syndromes might be of distinct nature.

Child, Preschool↗
PubMed1995

Occlusion of the right coronary artery as sequelae of Kawasaki disease: the clinical features of 9 cases.

Among the 302 children with Kawasaki disease (KD) who were evaluated by angiography from 1973 through 1992, 9 (3.0%) had either an occlusion (OC) or segmental stenosis (SS) of the right coronary artery. The interval from the onset of KD to the recognition of OC or SS ranged from 0.5 to 7.7 years (median 4.0 years). Left coronary arterial lesions were also present in 8 of 9 patients. In spite of severe sequelae, children or young adolescents with cardiovascular system-related symptoms were unexpectedly rare. Asymptomatic patients, however, are also at risk of developing myocardial infarction since they have been shown to have a high rate of abnormalities on myocardial scintigraphy. A close observation and careful follow-up are thus considered to be indispensable.

Adolescent↗