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Biomedical subjects

Tasuku Miyajima

Publications and source records attributed to Tasuku Miyajima.

16 recordsLinked to original sources

Usefulness of indomethacin for patent ductus arteriosus in full-term infants.

The aim of this retrospective study was to evaluate the effectiveness of indomethacin therapy for patent ductus arteriosus (PDA) in full-term infants. The patients were 41 full-term infants with a PDA birth weight (BW) > or =2500 g and a gestational age (GA) > or =37 weeks. The echocardiographic evaluation and medical management of PDA in these infants was similar to that for PDA in low-birth-weight infants. Indomethacin (0.2-0.25 mg/kg/dose) was given intravenously at 12-24-hour intervals within 23 days of birth. Of the 41 infants, 12 showed complete closure, and 13 showed improvement of clinical symptoms. These 25 infants were classified as the responder group (61%). The other 16 infants, who did not show improvement in clinical symptoms, were classified as the nonresponder group. Statistical analysis revealed no difference between the two groups regarding GA, BW, Apgar score at 1 minute, minimum diameter of the DA before treatment, the average age at the initiation of treatment, and DA flow pattern. No severe adverse reactions were observed in any infant. Indomethacin therapy appears to be an effective medical treatment option for PDA in full-term symptomatic infants prior to considering surgical treatment.

Cyclooxygenase Inhibitors↗

Diagnostic and predictive value of CSF d-ROM level in influenza virus-associated encephalopathy.

The aim of this study was to assess the validity of serum and CSF oxidative status of patients with IE in their initial stage through the d-ROM (Diacron-Reactive Oxygen Metabolites, Italy) test, compared to those with other neurological diseases. The study was conducted on the following four groups: (1) influenza virus-associated encephalopathy (IE, n = 8), including four patients showing neurological sequelae or mortal; (2) influenza virus-associated febrile seizures (IFS, n = 11); (3) febrile convulsion (FC, n = 10): (4) enterovirus-associated encephalopathy (EE, n = 4), including one patient with neurological sequelae. The CSF d-ROM levels in the IE group were significantly higher than those in the IFS and the FC groups but not in the EE group. In addition, general laboratory findings such as leukocytes, platelets, C-reactive protein, aspartate aminotransferase, creatinine, creatinine kinase and LDH, including interleukin-6 (IL-6), were analyzed in each group. The CSF d-ROM levels in the IE group were significantly higher than those in the IFS and FC groups but not in the EE group. As for the serum d-ROM levels and general laboratory findings, with the exception of CSF IL-6 levels in IE, no significant differences were detected compared with the other groups. In patients with IE, the CSF d-ROM levels could be a valid predictive biomarker of the severity, and oxidative stress may be related to the pathogenesis of IE.

Biomarkers↗

Effects of rapid rewarming on cerebral nitric oxide production and cerebral hemodynamics after hypothermia therapy for kainic acid-induced seizures in immature rabbits.

BACKGROUND: The aim of the present study was to investigate whether rapid rewarming after hypothermia therapy during seizures alters the endogenous nitric oxide (NO) production in and around hippocampus, cortical cerebral blood flow (cCBF), and mean arterial blood pressure (MABP) in immature rabbits. METHODS: The hypothermic rabbits (rectal temperatures, 33 degrees C) were given kainic acid (KA; 12 mg/kg, i.v; at 0 min), followed by cooling (33 degrees C) for 60 min (at 60 min), then either rewarming (RW; 33-37 degrees C) was started (KA[+]RW[+] group, n = 7) or cooling was continued (KA[+]RW[-] group, n = 7) for another 60 min (at the end 120 min). In the KA(-)RW(+) group (n = 5), 0.5 mL normal saline was given (at time 0 min), followed by cooling (33 degrees C) for 60 min (at 60 min), then rewarming to 37 degrees C was started with observation for another 60 min (at the end 120 min). NO production in and around hippocampus was continuously measured by an NO-selective electrode, cCBF by laser Doppler flowmetry, cortical electroencephalogram (EEG), rectal and cerebral temperatures, and MABP during the experiment. Comparisons were made of these parameters between the values at 60 min and 120 min after the KA administrations. RESULTS: KA administration induced abnormal discharges in both KA(+)RW(+) and KA(+)RW(-) groups at the same degree. The KA(+)RW(+) group had a significant increase in %NO, and significant decreases in %cCBF and MABP after rapid rewarming, compared with before rewarming. In the KA(+)RW(-) group, there were no significant changes in %NO, %cCBF, and MABP between values at 60 and 120 min. These changes after rapid rewarming in the KA(+)RW(+) group were different from those with only elevation in brain temperature from 33 to 37 degrees C without seizures (KA[-]RW[+] group). CONCLUSIONS: These results suggest that rapid rewarming after hypothermia therapy induces an increase in the NO production in and around hippocampus and the decreases in cCBF and MABP during seizures in immature rabbits.

Animals↗

[Developmental changes in the Collis horizontal reaction analyzed using a three-dimensional video motion analysis system. I. Examination of techniques for inducing Collis horizontal reaction].

We used a three-dimensional video motion analysis system to evaluate techniques for inducing the Collis horizontal reaction in 46 infants (total 217 times) from 1-9 months old. Additionally, we examined the safety of different techniques for inducing the Collis horizontal reaction in 20 infants (total 66 times), from 1-3 months old, 40 times by holding the shoulder joint and 26 times by not holding the shoulder joint. Regardless of who performed the examination, the average height that induced the reaction increased from 24.3 to 34.6 cm and the average speed of reaction increased from 28.2 to 43.4 m/s as the age of the infants increased. Additionally, when the Collis horizontal reaction was induced in infants 1-3 months old by holding the shoulder joint of infants, the average fall angle of the head was 7.291 degrees and was not significantly larger than when the reaction was induced by not holding the shoulder joint, which resulted is an average fall angle of the head was 19.302 degrees. However, although the average speed to support the body increased, the fall angle of the head did not increase when the shoulder joint was held. The Collis horizontal reaction is usually not performed in early infancy before head control is acquired, because of the burden to neck caused by the fall of the head and the excess expansion of shoulder joint capsule. However these results suggest that when performing the Collis horizontal reaction in early infancy before head control is acquired, the Collis horizontal reaction can be safely performed by holding the shoulder joint, regardless of the skill of the diagnostician. Therefore, the Collis horizontal reaction is an excellent method to assess infant development, and can be performed at the usual infant health examinations.

Child Development↗

[Developmental changes in the Collis horizontal reaction analyzed using a three-dimensional video motion analysis system. II. Examination of upper-limb movement during the Collis horizontal reaction].

At four months old, infants of 72% challenged with the Collis horizontal reaction touch the floor with the back of their free hand. As the infants grow older, the ratio of infants that open their free hand and perform a support reaction increases from 0% to 100%. At nine months old, all infants perform this support reaction with their free hand palm-side down. To assess infant development more accurately, we used a three-dimensional video motion analysis system to evaluate upper limb movement in 38 infants (total 151 times) from 4-9 months old during the second and third phases determined by the Collis horizontal reaction. By analyzing the constituent components of upper limb movement during the Collis horizontal reaction, we assessed the usefulness of the reactions as an objective tool to measure developmental changes in infants. During the analysis, we thought to assess the speed of the upper limb reaction was respect to pronating the forearm, supporting the body with the palm down, and location of their free hand. The analysis shows that if development progresses normally and infants pronate the forearm to support the body with the palm down, the central value at which their free hand is located moves to a point under the shoulder. The results suggest that in infants four to nine months old, the Collis horizontal reaction is an excellent method to assess not only the present physical development level, but also future physical development attainment by only evaluating developmental changes of upper limb movement only.

Child Development↗

FSHD-like patients without 4q35 deletion.

Facioscapulohumeral muscular dystrophy (FSHD) is characterized by progressive weakness and wasting of facial, shoulder-girdle and upper arm muscles. Despite of the characteristic clinical features, the diagnosis of FSHD is sometimes difficult because clinical symptoms are extremely variable including facial sparing type, limb-girdle type, and distal myopathy type. Most of the FSHD patients have a deletion in the subtelomeric region of chromosome 4q35 (FSHMD1A), however the linkage analysis in some families suggested genetic heterogeneity. In the present study, we identified 40 patients without a deletion in the 4q35 region (non-4q35del) among 200 Japanese patients who were clinically suspected to have FHSD. All non-4q35del patients had shoulder-girdle weakness and 75% also had facial weakness. Eight patients showed clinical features that were indistinguishable from FSHD, but two of them had Becker muscular dystrophy. FSHD is clinically, and most likely genetically, as well, variable. Other forms of muscular dystrophy can also mimic FSHD.

Adolescent↗

A case report of Kabuki make-up syndrome with cytomegalovirus infection.

An 8-month-male infant was admitted to our institute in order to investigate his developmental delay. He had facial features-long palpebral fissures with eversion of the lower lateral eyelids, arched eyebrows with lateral sparseness, depressed nasal tip, large, prominent and cupped ears. From these characteristical features, he was diagnosed as having Kabuki make-up syndrome (KS). When he was 2 months old, he was admitted to our institute because of intractable stridor and liver dysfunction associated with cytomegalovirus (CMV) infection. In KS, increased susceptibility to infection is described. We suspected persistent CMV infection because of an increased susceptibility to infection in KS. Recently acute idiopathic thrombocytopenic purpura (ITP) was diagnosed. According to the correlation between the number of CMV DNA copies and his platelet count, it is speculated that ITP would occur when the number of CMV DNA copies was elevated.

Abnormalities, Multiple↗

Hypothermia during kainic acid-induced seizures reduces hippocampal lesions and cerebral nitric oxide production in immature rabbits.

We investigated (1) whether cerebral hypothermia during kainic acid (KA)-induced seizures was neuroprotective; and (2) whether nitric oxide (NO) production in the brain during seizures was altered by cerebral hypothermia in immature rabbits. Twelve female rabbits, aged 2 weeks, were anesthetized, paralyzed and mechanically ventilated. We continuously measured NO production in the brain by NO-selective electrode, cortical electroencephalogram (EEG), regional cerebral blood flow (rCBF) by laser Doppler flowmetry, rectal and cerebral temperatures and mean arterial blood pressure (MABP) during KA (12 mg/kg, i.v.)-induced seizures in the hypothermic group (n = 6; rectal temperature, 33 degrees C), and in the normothermic group (n = 6; rectal temperature, 37 degrees C). The normothermic group showed a gradual increase in NO generation in the brain, which was significantly inhibited in the hypothermic group. There were no significant differences in the increases in rCBF, MABP, arterial blood gases, blood glucose, or EEG abnormalities between the two groups. Neuronal damages in the hippocampus (CA3) were significantly lower in hypothermia than in normothermia. These results suggest that hypothermia attenuates NO production during drug-induced seizures and decreases hippocampal brain lesions in the immature rabbit brain. These results may help to explain the neuroprotective effects of hypothermia.

Animals↗

Mutations of neuronal voltage-gated Na+ channel alpha 1 subunit gene SCN1A in core severe myoclonic epilepsy in infancy (SMEI) and in borderline SMEI (SMEB).

PURPOSE: Severe myoclonic epilepsy in infancy (SMEI) is a distinct epilepsy syndrome. Patients with borderline SMEI (SMEB) are a subgroup with clinical features similar to those of core SMEI but are not necessarily consistent with the accepted diagnostic criteria for core SMEI. The aim of this study was to delineate the genetic correlation between core SMEI and SMEB and to estimate the frequency of mutations in both phenotypes. METHODS: We examined 96 healthy volunteers and 58 unrelated individuals whose clinical features were consistent with either core SMEI (n = 31) or SMEB (n = 27). We screened for genetic abnormalities within exons and their flanking introns of the genes encoding major subunits of the Na+ channels (SCN1A, SCN2A, SCN1B, and SCN2B) by using a direct sequencing method. RESULTS: In both core SMEI and SMEB, various mutations of SCN1A including nonsense and missense mutations were identified, whereas no mutations of SCN2A, SCN1B, and SCN2B were found within the regions examined. All mutations were heterozygous and not found in 192 control chromosomes. Mutations were identified in 26 (44.8%) of the 58 individuals and were more frequent (p < 0.05) in core SMEI (19 of 31) than in SMEB (seven of 27), as assessed by the continuity-adjusted chi2 test. Mutations resulting in a molecular truncation were found only in core SMEI. Among the mutations, two missense mutations were found in both core SMEI and SMEB. CONCLUSIONS: Our findings confirm that SMEB is part of the SMEI spectrum and may expand the recognition of SMEI and suggest other responsible or modifying genes.

Amino Acid Sequence↗

[Retinopathy of prematurity in extremely low birth weight infants: a Tokyo multicenter study].

OBJECTIVE: To investigate how the increase in survival rate in extremely low birth weight (a birth weight of 1,000 g or less) infants had affected the incidence of retinopathy of prematurity (ROP) and the frequency of laser treatment. METHODS: We retrospectively reviewed the medical records of 122 surviving premature infants with birthweights less than 1,000 g to determine the severity of ROP observed at 16 neonatal intensive care units in Tokyo between April and October 2002. RESULTS: The survival rate was 85.6%. The mean gestational age was 26.74 weeks and the mean birth weight was 782.25 g. One-hundred-and-five infants (86.1%) developed ROP, fifty (41.0%) received laser treatment, and six (4.9%) had retinal detachment. The median postmenstrual age (gestational age at birth plus chronological age in weeks, PMA) at the onset of ROP was 32.5 weeks, and the first laser treatment was performed at the median PMA of 35.7 weeks. CONCLUSIONS: In these extremely low birth weight infants, there was an increase in the survival rate and in the incidence of severe ROP that progressed to the stage that required treatment.

Birth Weight↗

A case of Wilson-Mikity syndrome with high serum KL-6 levels.

KL-6 and surfactant protein D (SP-D) serum concentrations are known to rise in adult patients with various types of interstitial pneumonia. We evaluated the time course of serum KL-6, SP-D, and lactate dehydrogenase (LDH) levels in an infant with Wilson-Mikity syndrome. In this case, serum KL-6 levels correlated with clinical symptoms better than serum SP-D and LDH levels. The findings of this case suggest that high serum levels of KL-6 show activity of interstitial lung disease of Wilson-Mikity syndrome.

Antigens↗

Characteristics of 11 neonates with atrial septal defects detected by heart murmurs.

The aim of this prospective study was to investigate, using echocardiography, the relationship between heart murmurs found in the neonatal period and atrial septal defect (ASD). Heart murmurs were detected in 115 of 5124 term neonates within 1 month after birth. The babies were followed by echocardiographic examination with attention to the atrial septal area. Heart murmurs of 11 term neonates (9.6%) were associated with interatrial shunts consistent with ASD. The defect closed spontaneously in 7 out of the 11 neonates by 18 months of age. Two neonates showed evidence of right ventricular overload from early infancy. This study showed that nearly 1 neonate in 10 with a heart murmur up to 1 month after birth had an interatrial shunt associated with ASD. We concluded that the absence of a flap sign (valve-like openings of the defects) affects the development of the heart murmur of ASD found in the neonatal period.

Heart Murmurs↗

Nitric oxide donor increases cerebral blood flow and oxygenation during kainic acid-induced seizures in newborn rabbits.

BACKGROUND: We investigated the hypothesis that sodium nitroprusside (SNP), a nitric oxide (NO) donor, increased the cerebral blood flow and oxygenation during kainic acid (KA)-induced seizures in newborn rabbits. METHODS: After KA administration (i.v. 12 mg/kg)to induce seizures, either 1.2 mg/kg SNP (SNP group, i.v., n = 6) or 1 mL normal saline (vehicle group, i.v., n = 6)was given. Regional cerebral blood flow (rCBF), cerebral oxyhemoglobin(oxy-Hb), deoxyhemoglobin (deoxy-Hb), total hemoglobin (t-Hb), mean arterial blood pressure (MABP), heart rate (HR) and electroencephalography(EEG) were continuously monitored throughout the experiment, lasting at least 60 min after the KA administration. RESULTS: The value for rCBF was greatly increased during seizures in the SNP group than in the vehicle group. The values for oxy-Hb and t-Hb were significantly increased, and deoxy-Hb was significantly decreased. There were ameliorations of cerebral oxygenation in the SNP group during the acute phase of seizures in the neonatal animals, compared with the vehicle group. There were no significant differences in the MABP, HR, arterial blood gases, rectal and brain temperatures, blood hemoglobin concentrations,blood glucose levels, the latencies to first abnormal discharges in EEG, the total sum of the duration of abnormal discharges in EEG and the incidences of subclinical electric status epileptics between the two groups. CONCLUSIONS: These results suggest that the treatment with SNP contributed to the increases in cerebral blood flow and oxygenation, and that EEG abnormalities were unchanged by the treatment with SNP during neonatal seizures.

Animals↗

[Developmental change of Moro reflex studied with a three-dimensional motion analysis system].

We evaluated developmental change of the reaction time of the Moro reflex from birth to three months of age using a three-dimensional motion analysis system (ToMoCo-VM:Tousou system). The reaction time was the shortest in one-month-old babies and became successively prolonged in two and three-month-old babies, showing a U-shaped pattern. The three phase of the Moro reflex, as defined by McGraw, changed with advancing age. The reaction time changed monthly. At the same monthly age, the reaction time was the same irrespective of the phase.

Aging↗

[Clinical and genetical features of Japanese early-onset facioscapulohumeral muscular dystrophy].

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant form of muscular dystrophy characterized by progressive weakness and wasting of the facial, shoulder-girdle and upper arm muscles. The gene locus for FSHD is mapped to the subtelomeric region of chromosome 4q35, in which smaller EcoRI fragments (10 to 33 kb) are detected in most families by Southern blot analysis. The purpose of this study is to clarify the frequency and clinical/genetical features of early-onset FSHD in Japanese patients with 4q35-FSHD. In a series of 231 patients from 145 families with 4q35-FSHD, there were 31 patients (13.4%; male: female = 12:19) of 29 families (20%) with early-onset FSHD, 16 of whom were sporadic. Genetic analysis revealed that they had significantly smaller sized EcoRI fragments (range, 10 to 23 kb; mean 14.1 kb) than the other patients (range, 12 to 33 kb; mean 19.6 kb) (p < 0.001, U-test). All patients with the smallest EcoRI fragments (10 to 11 kb) were sporadic cases with early onset. Mental retardation (10/11) and epilepsy (4/11) was often observed in them, but not in the other patients. In early-onset FSHD, tongue muscle involvement (8/31) and swallowing disturbance (2/31), which has been regarded as exclusion criteria of FSHD, were also present. The onset of gait disturbance was significantly earlier (mean age = 11.9) in early-onset FSHD compared to the other group (mean age = 28.7). All patients with early-onset FSHD showed gait disturbance before 28 years of age. In conclusion, Japanese early-onset FSHD patients tend to have large gene deletions on chromosome 4q35, and show severe and variable phenotypes.

Adolescent↗