-
Pediatrics International 06/2011; 53(3):399-402. · 0.63 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Adenine phosphoribosyltransferase deficiency is a disorder in which 2,8-dihydroxyadenine (2,8-DHA) crystalluria is caused by a congenital deficiency in the enzyme adenine phosphoribosyltransferase (APRT). In most cases, APRT deficiency is caused by autosomal recessive inheritance of a homozygote of the mutant gene APRT*Q0 or APRT*J, but there are also some cases in which the disorder is caused by the compound heterozygote APRT*Q0 and APRT*J. In the patients described here, brown round crystals were found in their urinary sediment. Crystalluria was the first sign of APRT deficiency, thereafter confirmed by genetic screening for APRT*/Q0 and APRT*. We performed genetic screening for APRT*Q0 and APRT*J in two families and diagnosed three cases of APRT*Q0 /APRT*J compound heterozygote-type APRT deficiency. Genetic screening for APRT*Q0 and APRT*J of family members is effective for early diagnosis and early treatment for family members.
Pediatric Nephrology 06/2010; 25(6):1173-6. · 2.52 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Reported herein is a case of medulloblastoma with myogenic differentiation in a 3-year-old girl who died 1 year after appearance of clinical signs. Magnetic resonance imaging indicated a mass lesion in the cerebellar vermis. She underwent total resection of the tumor, followed by chemotherapy and radiotherapy in the brain and spinal cord. The resected specimen mainly consisted of densely packed cells with round-to-oval highly chromatic nuclei surrounded by scanty cytoplasm and focally of long spindle-shaped cells with elongated nuclei and eosinophilic cytoplasm showing discernible cross-striations. Immunohistochemistry indicated partial expression of synaptophysin in the former area and focal expression of desmin in the latter area. The diagnosis was medulloblastoma with myogenic differentiation, also known as medullomyoblastoma. Autopsy indicated disseminated proliferation of immature neuroglial cells with highly chromatic nuclei and scanty cytoplasm showing partial expression of synaptophysin, neurofilaments, and GFAP, and focal proliferation of round-to-oval immature cells showing immunoreactivity of myoglobin. The tumor cells had large nuclei, frequent mitoses, apoptoses, nuclear molding, and cell wrapping, indicating moderate anaplasia. Their Ki-67 labeling index was 54%. In addition, some tumor cells had double immunopositivity for synaptophysin or neurofilament and myoglobin, suggesting that the neuroectodermal cells may undergo differentiation into rhabdomyoblasts.
Pathology International 05/2009; 59(4):255-60. · 1.62 Impact Factor
-
Takashi Kusaka,
Masaki Ueno,
Takanori Miki,
Toru Kuboi,
Shinji Nakamura,
Kosuke Koyano,
Sonoko Ijichi,
Saneyuki Yasuda,
Kensuke Okubo,
Kou Kawada,
Masanori Namba,
Tomoko Nishida, Tadashi Imai,
Kenichi Isobe,
Susumu Itoh
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study was to evaluate the hypothesis that cerebral hemoglobin (Hb) oxygenation is related to phosphorylation potential during primary and secondary cerebral energy failure in newborn infants who have experienced birth asphyxia. We subjected newborn piglets to severe transient cerebral hypoxic-ischemia followed by resuscitation and examined cerebral energy metabolism by 31P-magnetic resonance spectroscopy and evaluated changes in cerebral Hb oxygen saturation (ScO2) using full-spectrum near-infrared spectroscopy before, during, and up to 54 h after the hypoxic-ischemic insult. ScO2 was significantly decreased during the hypoxic-ischemic insult compared with baseline values. During secondary energy failure, piglets were separated based on the relationship between the ratio of phosphocreatine to inorganic phosphate and ScO2; those with a negative correlation were less injured than those with a positive correlation. These results indicate that changes in ScO2 as measured by near-infrared spectroscopy are related to phosphorylation potential during secondary energy failure in asphyxiated infants.
Pediatric Research 12/2008; 65(3):317-22. · 2.70 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Using optical topography, changes in the cerebral oxygenation were compared in the parieto-temporal lobe of preterm and term infants of equal postconceptional age in response to verbal stimulation. Eight preterm infants of gestational age 23-34 weeks were studied at postconceptional term age (38-46 weeks). Ten term infants were studied at 2-11 days after birth. Twenty-four-channel near-infrared optical topography (NIOT) was used to measure changes in concentration of oxyhemoglobin ([oxyHb]), deoxyhemoglobin ([deoxyHb]) and total hemoglobin ([totalHb]) in the bilateral temporal cortices. Verbal stimulation was provided by a recording of a Japanese fairy tale. The latency in response to verbal stimulation was significantly shorter in the preterm infants than in the term infants. This time is thought to reflect brain development, particularly the development of the neuro-vascular coupling mechanisms in the cerebral cortex. The present results indicate that the number of days after birth is more closely related to development of auditory system and neuro-vascular coupling than is postconceptional age. Thus, this suggests that early extrauterine environment affects the cortical responses to verbal stimulation in preterm infants.
Neuroscience Letters 09/2008; 443(1):23-6. · 2.11 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Approximately 5% to 10% of intracranial germinomas arise from the basal ganglia or thalamus. Diagnosis is usually made by stereotactic biopsy, and precise location of the biopsy target is crucial because germinoma in these sites is potentially curable. We herein describe a case with germinoma in the basal ganglia that showed nonspecific clinical and radiological findings. The usefulness of MET-PET in locating an optimal biopsy target and monitoring treatment efficacy in this case is presented.
A 9-year-old boy presented with a 4-month history of dystonia in his left upper extremity. Magnetic resonance imaging of the brain showed abnormal signal intensity in the right basal ganglia, internal capsule, and corona radiata without mass formation and enhancement effect. He had been treated as having multiple sclerosis without improvements on clinical and radiological findings. The MET-PET study showed increased tracer uptake in the areas of abnormal signal intensity on the MR images, and the MRI-PET co-registered images exhibited the highest tracer uptake in the anterior limb of the internal capsule. A stereotactic biopsy targeting the highest tracer uptake lesion was performed with histologic verification of germinoma. He was intensively treated with combined chemotherapy and radiotherapy according to the MR images and MET-PET findings. After the treatment, the area of abnormal signal intensity significantly reduced in size on the follow-up MRI and lesional tracer uptake was also decreased on MET-PET images.
The MET-PET study is very useful for locating a precise biopsy target and provides useful information in monitoring treatment efficacy in the basal ganglia germinoma that showed nonspecific radiological findings.
Surgical Neurology 03/2008; 70(4):408-13; discussion 413. · 1.67 Impact Factor
-
Hitoshi Okada,
Tae Abe,
Yoko Etoh,
Shuji Yoshino,
Ikuko Kato,
Takuma Iwaki,
Kensuke Okubo,
Saneyuki Yasuda,
Kou Kawada,
Takashi Kusaka,
Masanori Namba,
Tomoko Nishida, Tadashi Imai,
Kenichi Isobe,
Susumu Itoh
[show abstract]
[hide abstract]
ABSTRACT: The light-emitting diode is used as one of the new light sources for phototherapy. NeoBLUE (Atom Medical, Tokyo, Japan) incorporates blue light-emitting diodes for the treatment of neonatal hyperbilirubinemia. The authors compared the in vitro efficacy of neoBLUE with conventional phototherapy devices.
The three light devices used included neoBLUE and two conventional phototherapy devices with six blue-white (BW) or six green (GR) fluorescent tubes. A bilirubin/human serum albumin solution (15 mg/dL) in 200 x 300 mm elliptical bag was irradiated with each three light device. The average light intensity of neoBLUE, BW and GR was 22.5, 10.2 and 2.6 microW/cm(2) per nm, respectively, for the irradiated area. Bilirubin photoisomers and native bilirubin were measured by high-performance liquid chromatography.
In neoBLUE, BW and GR, the respective production rate of cyclobilirubin was 6.0, 3.7 and 3.9 x 10(-2) mg/dL/min, and the respective (4Z, 15E)-bilirubin/(4Z, 15Z)-bilirubin ratio after irradiation was 0.44, 0.33 and 0.12; the (4Z, 15Z)-bilirubin reduction rate at 20 min after irradiation was 60, 68 and 82%, respectively. The reduction rate of (4Z, 15Z)-bilirubin correlated with the (4Z, 15E)-bilirubin/(4Z, 15Z)-bilirubin ratio.
Phototherapy using the neoBLUE under high level may be clinically more effective than therapy using the conventional light source from the results of the production rate of cyclobilirubin.
Pediatrics International 07/2007; 49(3):318-21. · 0.63 Impact Factor
-
Pediatrics International 05/2006; 48(2):163-5. · 0.63 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Near-infrared spectroscopy has been used for measurement of changes in cerebral Hb concentrations in infants to study cerebral oxygenation and hemodynamics. In this study, measurements by time-resolved spectroscopy (TRS) were performed in 22 neonates to estimate the values of light absorption coefficient and reduced scattering coefficient (mu'(s)), cerebral Hb oxygen saturation (SCO2), cerebral blood volume (CBV), and differential pathlength factor (DPF), and the relationships between postconceptional age and mu'(s), SCO2, CBV, and DPF were investigated. A portable three-wavelength TRS system with a probe attached to the head of the neonate was used. The mean mu'(s) values at 761, 795, and 835 nm in neonates were estimated to be (mean +/- SD) 6.46 +/- 1.21, 5.90 +/- 1.15 and 6.40 +/- 1.16/cm, respectively. There was a significant positive relationship between postconceptional age and mu'(s) at those three wavelengths. The mean SCO2 value was calculated to be 70.0 +/- 4.6%, and postconceptional age and SCO2 showed a negative linear relationship. The mean value of CBV was 2.31 +/- 0.56 mL/100 g. There was a significant positive relationship between postconceptional age and CBV. The mean DPF values at 761, 795, and 835 nm were estimated to be 4.58 +/- 0.41, 4.64 +/- 0.46, and 4.31 +/- 0.42, respectively. There was no relationship between postconceptional age and DPF at those three wavelengths. The results demonstrated that our near-infrared TRS method can be used to monitor mu'(s), SCO2, CBV, and DPF in the neonatal brain at the bedside in an intensive care unit.
Pediatric Research 10/2005; 58(3):568-73. · 2.70 Impact Factor
-
Hitoshi Okada,
Kumiko Masuya,
Saneyuki Yasuda,
Kensuke Okubo,
Kou Kawada,
Takashi Kusaka,
Masanori Namba,
Tomoko Nishida, Tadashi Imai,
Kenichi Isobe,
Susumu Itoh
[show abstract]
[hide abstract]
ABSTRACT: Phototherapy has been a standard treatment for neonatal hyperbilirubinemia for more than 40 years, but it has remained sub-optimal.
To clarify the developmental changes in parameters of (4E, 15Z)-cyclobilirubin ((EZ)-C) elimination in order to obtain basic data for establishing optimal phototherapy.
Blood samples were taken at regular intervals after stopping phototherapy, and bilirubin fractions were analyzed by high-performance liquid chromatography.
The subjects were 46 infants with hyperbilirubinemia who underwent phototherapy. The gestational age and birth weight of the subjects ranged from 25.0 to 41.0 weeks and from 656 to 3810 g, respectively, and the age at cessation of phototherapy was a median of 5 days. A kinetic model of (EZ)-C elimination was established, and the serum half-life of (EZ)-C was calculated on the basis of the determined model. Relationships of the half-life of (EZ)-C with birth weight and gestational age were investigated.
Serum (EZ)-C elimination followed a first-order kinetic model in 43 infants and a zero-order kinetic model in three extremely low birth weight infants. The half-life of (EZ)-C calculated on the basis of a first-order elimination model in serum ranged from 68 to 274 min and showed weak negative correlations with birth weight and gestational age.
Serum (EZ)-C excretion followed a first-order kinetic model in most of the neonates. The half-life of (EZ)-C becomes more prolonged in the very low birth weight infant and early gestational age.
Early Human Development 08/2005; 81(7):619-22. · 2.05 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Estimation of hemodynamics is important for critically ill infants. Pulse dye densitometry (PDD) using indocyanine green (ICG), which enables measurements of circulating blood volume at the bedside, has recently been developed for adults.
We conducted a basic investigation to determine whether this method can be applied to infants and measured circulating blood volume in 25 infants whose gestational ages ranged from 24 to 40 weeks (median, 32 weeks). At first, to validate the accuracy of measurements, arterial ICG concentrations determined by blood sample measurements were compared using a spectrophotometer ([ICG blood]) and by noninvasive measurement using PDD ([ICG pdd]) in seven infants. Next, blood volumes in 25 infants were estimated by the PDD method.
There was a positive relationships between [ICG blood] and [ICG pdd] (r = 0.913, P < 0.0001). Using Bland Altman analysis, the bias between the two methods was 0.24 +/- 0.30 mg.l(-1) (95% confidence interval: 0.39-0.09 mg.l(-1)) and the limits of agreement (2 sd) were -0.36 and 0.84 mg.l(-1), respectively. Mean (sd) blood volume was 94.9 ml.kg(-1) (24.3). The values obtained by this study are almost the same as previously reported values obtained by using other methods.
PDD using ICG can be used to monitor of hemodynamics in infants.
Pediatric Anesthesia 03/2005; 15(2):125-30. · 2.10 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The developmental changes of the umbilical cortisol levels in neonates at gestational age of 23-41 weeks were studied and the effect of antenatal steroid administration on the umbilical cortisol levels were examined.
Cortisol levels in the umbilical vein (UV) and the umbilical artery (UA) were studied in 35 neonates at the gestational age (GA) of 23-41 weeks with or without antenatal administration of corticosteroids. Serum cortisol concentrations were measured by the high performance liquid chromatography method.
The correlation between cortisol levels in UV and birthweight (BW) was weak and negative in premature infants. UV cortisol levels in the neonates with antenatal corticosteroid were lower than those in the neonates without antenatal corticosteroid, but the relation was not significant. The developmental changes of UV cortisol levels were the same as those in Murphy's study (spontaneous-onset labor). The cortisol levels in UV and UA had a significantly positive correlation and both had almost equal concentrations. There were no correlations between cortisol levels in UV and placental weight, Apgar Score at 1 and 5 min.
In the neonates whose birthweight was less than 2000 g without antenatal corticosteroid, there was a negative correlation between cortisol levels in UV and BW but there was no correlation between cortisol levels in UV and GA. That the neonates with antenatal corticosteroid would have a suppressed adrenocortical function after birth could not be proved.
Pediatrics International 03/2005; 47(1):60-3. · 0.63 Impact Factor
-
Hitoshi Okada,
Kumiko Masuya,
Yuki Kurono,
Keiko Nagano,
Kensuke Okubo,
Saneyuki Yasuda,
Ayako Kawasaki,
Kou Kawada,
Takashi Kusaka,
Masanori Namba,
Tomoko Nishida, Tadashi Imai,
Kenichi Isobe,
Susumu Itoh
[show abstract]
[hide abstract]
ABSTRACT: The clinical effect of phototherapy for neonatal hyperbilirubinemia is based on the production and elimination of cyclobilirubin. Generally, the clinical effect of light sources is estimated by the reduction in the total serum bilirubin level. One procedure with less invasiveness than blood collecting is urine collection. Whether the effectiveness of light sources used for phototherapy could be assessed using measurements of bilirubin photoisomers in urine was studied.
This study was a retrospective analysis of 38 term infants with hyperbilirubinemia who underwent phototherapy. Bilirubin fractions in serum and urine before and 24 h after the phototherapy were measured by high-performance liquid chromatography. The light sources used for the phototherapy were blue-white light (n = 11), Biliblanket plus high output (n = 13) or green light (n = 14). The relationships between serum and urine bilirubin photoisomers after phototherapy and whether the levels of urine bilirubin photoisomer are affected by the light sources with different wavelength characteristic were analyzed.
There was no correlation between serum (ZE)-bilirubin and urine configurational isomers, but a weak positive correlation between serum (EZ)-cyclobilirubin and urine structural isomers after phototherapy. Although serum (ZE)-bilirubin levels depended on the wavelength characteristic of each light source during phototherapy, the urine configurational isomer levels did not depend on it. The increase in serum (EZ)-cyclobilirubin levels and the urine structural isomer levels were mostly in agreement.
The urine bilirubin structural isomers may be used to estimate the serum (EZ)-cyclobilirubin levels and to evaluate the clinical effects of light sources.
Pediatrics International 01/2005; 46(6):640-4. · 0.63 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: During the developmental stage, the brain undergoes anatomic, functional, and metabolic changes necessary to support the complex adaptive behavior of a mature individual. Estimation of developmental changes occurring in different regions of the brain would provide a means of relating various behavioral phenomena to maturation-specific brain structures, thereby providing useful information on structure-function relationships in both normal and disease states. We used multichannel near-infrared spectroscopy (MNIRS), a new noninvasive imaging technique for revealing the course of neural activity in selected brain regions, to monitor the activities of the visual cortex as mirrored by hemodynamic responses in infants subjected to photostimulation during natural sleep. In the infants, oxyhemoglobin and total hemoglobin decreased and deoxyhemoglobin increased in the visual cortex with photostimulation. This pattern of responses was different from the response pattern in adults reported previously. The different patterns of responses to photostimulation in the visual cortices of infants and adults might reflect developmental and behavioral differences. It may reflect a different functional organization of the visual cortex in infants or ongoing retinal development. Our results demonstrated that regional hemodynamic change could be detected in a small area around the visual cortex. MNIRS offers considerable potential for research and noninvasive clinical applications.
Human Brain Mapping 07/2004; 22(2):122-32. · 5.88 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The aim of this study was to determine the validity of the hypothesis that excitatory amino acids are related to phosphorylation potential during primary and secondary cerebral energy failure observed in asphyxiated infants. We report here the results of experiments using newborn piglets subjected to severe transient cerebral hypoxia-ischemia followed by resuscitation. We examined cerebral energy metabolism by phosphorus nuclear magnetic resonance spectroscopy and changes in levels of amino acid neurotransmitters in the cortex by microdialysis before, during, and up to 24 h after the hypoxic-ischemic insult. The concentrations of aspartate, glutamate, taurine, and gamma-aminobutyric acid were significantly elevated during the hypoxic-ischemic insult compared with prebaseline values. Shortly after resuscitation, glutamate, taurine, and gamma-aminobutyric acid concentrations decreased but then began to increase again. These secondary elevations were greater than the primary elevations. A negative linear correlation was found between primary interstitial levels of glutamate and taurine and minimum values of phosphocreatine/inorganic phosphate during the secondary energy failure. The cerebral energy state depended on the time course of changes in excitatory amino acids, suggesting that amino acids play distinct roles during the early and delayed phases of injury.
Pediatric Research 03/2004; 55(2):273-9. · 2.70 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Agranulocytosis caused by ethosuximide is extremely rare in children. Drug-induced agranulocytosis is an unexpected side effect of a drug, and delay in diagnosis of agranulocytosis can result in a fatal outcome. We experienced a case of a 16-month-old male infant with Down syndrome in whom fever appeared 16 days after the start of administration of ethosuximide and then severe pneumonia developed. Results of a blood test on admission showed a decreased leukocyte count of 1700/microl, and a hemogram showed that there were no granulocytes. The erythrocyte and thrombocyte counts were within normal ranges. The results of a bone marrow aspiration test showed that there was no production of any types of granulocytes. The patient required mechanical ventilation due to deterioration in his pneumonia and complication with disseminated intravascular coagulation, but the neutrophilic leukocytes began to increase from the 8th day after discontinuation of ethosuximide administration and start of treatment with granulocyte colony-stimulating factor, and the patient survived. The mechanism of onset in this case is thought to have been immunologic. Careful attention should be given to this type of agranulocytosis because of its sudden onset at 1-2 weeks after the start of administration of the causal drug. A drug-induced lymphocyte stimulation test was useful for diagnosis in this case, showing a positive reaction only for ethosuximide.
Brain and Development 11/2003; 25(7):522-4. · 2.12 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Near-infrared spectroscopy (NIRS) has been used for measurement of changes in cerebral hemoglobin concentrations in neonates to study cerebral oxygenation and hemodynamics. In this study, measurements by time-resolved reflectance spectroscopy (TRS) were performed in a piglet model with various degrees of cerebral oxygenation to estimate the differential pathlength factor (DPF). A portable three-wavelength TRS system (TRS-10, Hamamatsu Photonics K.K.) with a probe attached to the head of a piglet was used. Eleven newborn piglets were anesthetized and respired by a ventilator to induce stepwise hypoxia loading. The DPF showed positive linear relationship with arterial hemoglobin (Hb) oxygen saturation and sagittal sinus venous Hb oxygen saturation at 761 and 795 nm. The DPF at 835 nm also showed very slight positive linear relationship with arterial hemoglobin oxygen saturation. The DPF values obtained in this study should contribute to a better understanding of noninvasive measurements by NIRS in neonates.
Optical Review 08/2003; 10(5):466-469. · 0.66 Impact Factor
-
Advances in experimental medicine and biology 02/2003; 540:7-11. · 1.09 Impact Factor
-
Advances in experimental medicine and biology 02/2003; 510:255-9. · 1.09 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: In the enzyme reaction for the determination of the unbound (free) bilirubin concentration by glucose oxidase and peroxidase, materials with low affinity for serum protein are reactive. The influence of these materials on the determination of serum unbound bilirubin was investigated. METHODS Serum samples from patients with neonatal hyperbilirubinaemia were analysed by high-performance liquid chromatography for total glucuronosyl bilirubin concentration (TGC) and (EZ)-cyclobilirubin concentration [(EZ)-C]. Based on these measurements, the samples were classified into three groups: group I [13 samples, TGC <2 micromol/L and (EZ)-C < 2.5 micromol/L]; group II [four samples, TGC < 2 micromol/L and (EZ)-C > or = 2.5 micromol/L]; and group III (five samples, TGC > or = 2 micromol/L). The concentrations of total bilirubin and unbound bilirubin were measured in these same samples with a UB-analyser. When the absorbance at 460nm was monitored, the decrease in absorbance was non-linear (concave curve). The degree of concavity was estimated (D15 value) as the deviation from linearity at 15s.
The D15 value was significantly higher in groups II and III than in group I. D15 value correlated significantly with TGC, (EZ)-C and unbound bilirubin concentration, and the unbound bilirubin concentration correlated significantly with TGC and (EZ)-C.
These results indicated that determination of serum unbound bilirubin concentration using the UB-analyser could be positively skewed by high concentrations of TGC and (EZ)-C.
Annals of Clinical Biochemistry 12/2002; 39(Pt 6):583-8. · 2.17 Impact Factor
