bluuebunny/crossref_metadata_2025 huggingface dataset:
Effect of calcium antagonist, nicardipine, on cerebral blood flow in postasphyxial newborn piglets
Authors: IMAI TADASHI , KONDO MASATOSHI , KUSAKA TAKASHI , SUGIHARA SATORU , ISOBE KENICHI , ITOH SUSUMU , ONISHI SHOJU
Published: January 2011

An experiment was carried out in nine piglets within 24 h after birth (control group: four, nicardipine group: five) for the purpose of evaluating the effects of a calcium antagonist, nicardipine, on cerebral blood flow changes induced by asphyxia neonatorum. Under respiratory control with a mechanical ventilator, the animals were exposed to hypoxia. The inspiratory oxygen level was lowered at 15 min intervals from 0.08 to 0.06 and then to 0.05. When bradycardia (heart rate: 60/min or less) was observed, 100% oxygen, adrenaline, and sodium bicarbonate were administered for resuscitation. Nicardipine was administered at a dosage of 10μg/kg via bolus injection 30 min after the resuscitation. It was administered thereafter at a rate of 10μg/kg per h. The cerebral blood flow was measured using a laser Doppler velocimeter. The cerebral blood flow, electroencephalograph (EEG), blood pressure, and heart rate were continuously measured for 120min after the resuscitation. In the control group, the mean arterial pressure 35 min after the resuscitation was 60 mmHg or more. However, the cerebral blood flow was lower than the prehypoxia value in the animals with a mean arterial pressure of 75mmHg or less. In the nicardipine group, the mean arterial pressure was lower, but the cerebral blood flow was higher than the prehypoxia value and cerebral ischemia was not induced. The mean arterial pressure 120 min after the resuscitation was 72.0 ± 8.2 mmHg in the control group, while it was 56.7 ± 7.5 mmHg in the nicardipine group. It was significantly lower in the latter. The cerebral blood flow as compared to that before the initiation of exposure to hypoxia (this being considered 100) was 83.1 ± 22.0% in the control group, while it was 125.1 ± 26.2% in the nicardipine group. It was significantly higher in the latter. A suppression burst‐like abnormal EEG finding occurred in two of four animals from the control group and was noted in one of five animals from the nicardipine group.

In the nicardipine group, the mean arterial pressure was lowered, but a decrease in the cerebral blood flow after asphyxia was totally prevented and the incidence of EEG abnormality was low. It seems possible that this drug protects the brain from asphyxia‐induced changes.
Abnormal cardiac histology in severe intrauterine growth retardation infants
Authors: TAKAHASHI NAOTO , NISHIDA HIROSHI , ARAI TOSHIHIKO , KANEDA YOSHIO
Published: January 2011

Four infants with severe intrauterine growth retardation (IUGR) weighing less than 1000 g at birth developed heart failure and died in our unit, where heart failure of IUGR infants is the main reason of death in extremely low birth‐weight infants. The causes of their heart failure are one of the main themes in current neonatal medicine. The subjects of this study were four small for gestational age infants; all died due to heart failure 5 to 10 days after birth. Microscopic specimens of hearts from autopsies were evaluated with respect to the following characteristics: thickness of myocardial fibers, maturation of nuclei, presence of dysgenesis or necrosis in myocardium, and amount of glycogen in the heart. Neither dysgenesis nor infarction of the heart was found but hypoplasia in myocardial fibers and decreased glycogen levels were observed. Maturation delay in myocytes' nuclei did not appear to be severe. We conclude that these infants' hearts failed to adapt to postnatal hemodynamic changes because of inadequate myocardial function and inadequate glycogen reserves.
Acute rheumatic fever and poststreptococcal acute glomerulonephritis caused by T serotype 12 Streptococcus
Authors: IMANAKA HIROYUKI , ETO SUGURU , TAKEI SYUJI , YOSHINAGA MASAO , HOKONOHARA MASASHI , MIYATA KOICHIRO
Published: January 2011

We present a rare case of a 10 year old Japanese boy with acute rheumatic fever accompanied with poststreptococcal acute glomerulonephritis. We isolated group A Streptococcus serotype T 12, a strain that was thought to be nephritogenic but not rheumatogenic, from throat culture. Although rare, physicians should be aware that acute renal disease may accompany rheumatic fever.
Cardiac sequelae of Kawasaki disease in Japan over 10 years
Authors: HIROSE KENJI , NAKAMURA YOSIKAZU , YANAGAWA HIROSHI
Published: January 2011

To observe the secular trend of a proportion of Kawasaki disease patients with cardiac sequelae in Japan, we analyzed patients with Kawasaki disease reported to nationwide surveys of the disease during 10.5 years from July 1982 to December 1992. Of 69 382 patients reported to the surveys, 10 596 (15.3%) were reported to have cardiac sequelae such as dilatation or stenosis of coronary arteries, myocardial infarction or valvar lesions, 1 month or more after onset. The percentage of cardiac sequelae was particularly high in males, infants younger than 1 year and children older than 5 years of age. The overall prevalence declined steadily over the observed period. However, the percentage for children older than 5 years of age did not decrease, whether treated with intravenous gamma globulin or untreated. As a consequence of the increased number of patients treated with intravenous gamma globulin, the proportion of Kawasaki disease patients with cardiac sequelae decreased annually. However, the proportion of children older than 5 years of age did not decrease.
Optimal dosage and differences in therapeutic efficacy of IGIV in Kawasaki disease
Authors: ONOUCHI ZENSHIRO , YANAGISAWA MASAYOSHI , HIRAYAMA TSUNEO , KIYOSAWA NOBUYUKI , MATSUDA HIROSHI , NAKASHIMA MITSUYOSHI
Published: January 2011

In an initial study, three groups of patients with Kawasaki disease received either aspirin alone or alkylated immunoglobulin G intravenous preparation (IGIV) 200 mg/kg daily x3 days + aspirin, or 400 mg/kg alkylated IGIV daily x3 days + aspirin. In a second study, three groups of patients were treated with either 100, 200 or 400 mg/kg of native IGIV in combination with aspirin daily for 5 days.

While the regimen of 200 mg/kg native IGIV daily x 5 days was found to be effective, the incidence of coronary artery lesions (CAL) was even less on a regimen of 400 mg/kg daily x 5 days. It is therefore suggested that a better therapeutic effect can be achieved with a 400 mg/kg dose of native IGIV.

Based on the results from these two studies, it is assumed that native IGIV is more effective in inhibiting CAL formation and persistence than the chemically modified preparation in which the biological activity of the Fc region in the immunoglobulin G molecule is altered.
A case report of Stevens‐Johnson syndrome with Mycoplasma pneumoniae infection
Authors: SAITOH AKIHIKO , OHYA TATSUO , YOSHIDA SHIGEHIKO , HOSOYA RYOHTA , NISHIMURA KOZO
Published: January 2011

We encountered an 8 year old boy who suffered from Stevens‐Johnson syndrome with Mycoplasma pneumoniae infection. He had multiple erythema with vesicles in oral mucosa, and on his palms and feet, trunk and genital regions. We treated him with prednisolone (1 mg/kg per day) and antibiotics. His skin lesions improved dramatically, and a persistent fever and toxic general condition also showed dramatic improvement.

Although the use of corticosteroids for Stevens‐Johnson syndrome has recently been controversial, we thought that administration of corticosteroids was an effective treatment for some selective cases of Stevens‐Johnson syndrome. The patient reported in this study had many beneficial effects in response to corticosteroid treatment.
Serum cholesterol levels in school‐aged Japanese children: The Hisayama study
Authors: FUKUSHIGE JUNICHIRO , IGARASHI HISAJI , UEDA KOHJI , AKAZAWA KOUHEI , NOSE YOSHIAKI
Published: January 2011

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.
Insertion of a pacing lead via the tricuspid valve does not affect cardiac function and tricuspid valve regurgitation in young dogs
Authors: KIKUCHI YUTAKA , SHIRAISHI HIROHIKO , IGARASHI HIROSHI , YANAGISAWA MASAYOSHI
Published: January 2011

A preliminary experimental study in dogs was conducted to evaluate the feasibility of transvenous cardiac pacing in the fetus with complete heart block associated with hydrops. Four young mongrel dogs were anesthetized with intravenous administration of sodium pentobarbital and mechanically ventilated, and a pacing lead was inserted via the tricuspid valve. The right ventricular cardiac output, aortic pressure and central venous pressure were measured, and the tricuspid valve regurgitation was measured semi‐quantitatively using echo‐Doppler color flow imaging. The relationship between the location of the pacing lead and the tricuspid valve regurgitation and cardiac function was examined.

The mean right ventricular cardiac output when the pacing lead was inserted into the superior vena cava (126 ± 54 mL/min per kg) was not significantly different from that when it was inserted into the right ventricle (110 ± 43 mL/min per kg). The aortic pressure was 66 ± 7.7 mmHg and 67 ± 6.6 mmHg, respectively, and the central venous pressure 5.9 ± 1.7 mmHg and 5.7 ± 1.6 mmHg, respectively, under the two conditions (not significantly different). The ratio of demonstrating significant tricuspid valve regurgitation was 4/13 into the superior vena cava and 5/13 into the right ventricle, respectively (not significantly different).

The location of the pacing lead did not change the cardiac function or the amount of the tricuspid valve regurgitation in our experimental study. It was therefore concluded that the transvenous cardiac pacing technique has potential application in intrauterine transvenous cardiac pacing in the fetus with complete heart block.
Psychosocial problems of children and adolescents with a chronic disease: Coping strategies
Authors: MILOUSHEVA JULIETTE , KOBAYASHI NOBORU , MATSUI ICHIRO
Published: January 2011

The purpose of this pilot study was to identify the coping strategies used by children, adolescents and youths with insulin dependent diabetes mellitus (IDDM) attending a camp for IDDM patients near Ryosen town, Fukushima Prefecture, Japan.

Forty‐three IDDM patients (24 females and 19 males) were studied, divided into two age groups. The first group included children (seven males and 10 females). The second group included adolescents and youths (12 males and 14 females). For the child group a projective drawing method was used for study and for the older group, an open questionnaire was used.

Ryan‐Wenger's taxonomy of children's coping strategies and Band's coding systems and classification were used for the content analysis.

‘Instrumental action’, ‘Emotional expression’ and ‘Catastrophizing thinking’ were the coping strategies, represented in the child group.

Gender differences in coping strategies were found in the group of adolescents and youths. The most often represented and most important coping category for the male subgroup was ‘Behavioral avoidance’. Next in frequency of representation for the male subgroup were ‘Cognitive distraction’ and ‘Behavioral distraction’. The coping categories ‘Seeking social support’ and ‘Behavioral distraction’ were represented with equal frequency in the female subgroup and the next was ‘Aggressive activities’. The most important coping strategy for the female group was ‘Talking to peers’ from the seeking social support category.

The study also helped to identify several children and adolescents who might need special psychological support.
Magnetic resonance imaging of clival marow in patients with anorexia nervosa
Authors: KUWASHIMA SHIGEKO , NISHIMURA GEN , YAMATO MINORU , FUJIOKA MUTSUHISA
Published: January 2011

Hematological abnormalities, commonly associated with anorexia nervosa (AN) patients, are thought to be the results of serous atrophy in the bone marrow. Magnetic resonance imaging (MRI) has been utilized to ascertain T1 and T2 prolongation of marrow intensity in the lumbar spine, pelvis and proximal femora. The results correlate well with the severity of hematological abnormalities and body mass index. More importantly, the propensity for peripheral marrow involvement of T2 prolongation contrasts with the axial involvement in other marrow disorders. MRI undertaken in patients with AN to exclude hypothalamic tumor showed that the clival marrow was equivalent to the peripheral marrow. The signal pattern of clival marrow on sagittal T1 weighted MR images was evaluated in four teenage female patients with AN complicated by hematological abnormalities. Although the clival marrow intensity should be uniformly high in teenagers, three patients, two with pancytopenia and one with leukopenia and anemia, exhibited homogenous low intensity. One patient who had leukopenia only and the highest body mass index, showed inhomogeneous low intensity. The signal changes returned to normal in all patients but one, who died before examination after 6–11 months, at which time the others had almost recovered their original weight and normal hemogram. T1 prolongation in the clival marrow represents bone marrow dysfunction and the inhomogeneity of the signal change may imply relative preservation of hematopoiesis and body fat composition. Lack of knowledge of this phenomenon may lead to diagnostic confusion with other marrow disorders on cranial MRI.
Long‐term ventilator‐assisted children in Japan: A national survey
Authors: SAKAKIHARA YOICHI , YAMANAKA TATSUHIRO , KAJII MOTOFUMI , KAMOSHITA SHIGEHIKO
Published: January 2011

Owing to improved technology and care for patients who need mechanical ventilation, the quality of life as well as the prognosis for long‐term ventilator‐assisted patients has improved significantly in recent years. However, the increased number of these patients has raised economic, ethical and medical problems. In order to assess the magnitude of these problems, we conducted the first nationwide survey on the status of long‐term ventilator‐assisted children in Japan.

Questionnaires were mailed to 2524 pediatric departments at hospitals in Japan with more than 100 beds. At the time of the survey, 282 hospitals had 567 patients who had been ventilated for more than a month. Among these patients, 434 were younger than 20 years and had been ventilated for more than 3 months. The most common basic disorders were: various myopathies (n = 65), hypoxicischemic encephalopathy (n = 60), spinal muscular atrophy type 1 (Werdnig‐Hoffmann disease, n = 55), chronic lung disorders of prematurity (n = 21), Ondine's curse (n = 22), drowning (n = 17) and congenital heart diseases (n = 16). Of these 434 patients, only 61 were ventilated at home. Although home care was considered suitable for chronic ventilator patients by many pediatricians who responded to the survey, its realization has been hampered by the lack of a system and regulations to support it. The fact that many pediatricians in Japan have actively prolonged the life of Werdnig‐Hoffmann patients, from whom aggressive life saving measures have been withheld in most Western countries, has raised ethical as well as medical issues.
Gene therapy for metachromatic leukodystrophy
Authors: OHASHI TOYA , WATABE KAZUHIKO , SATO YUMI , SAITO IZUMU , BARRANGER JOHN A , MATALON REUBEN , ETO YOSHIKATSU
Published: January 2011

Metachromatic leukodystrophy (MLD) is an inherited metabolic disease which is characterized by a deficiency of arylsulfatase A (ASA). This deficiency causes progressive accumulation of cerebroside sulfate in oligodendrocytes (OL) in the brain, resulting in dysmyelination. Approaches being developed by the authors to treating MLD are based on direct delivery of ASA genes into the brain. In the present report, it has been shown that the recombinant adenovirus (Adex 1SRLacZ) was able to transduce the OL very efficiently. Moreover, primary fibroblasts from MLD patients were exposed to recombinant adenovirus expressing the ASA gene (Adex1SRASA) and the cells expressed the transgene. The influence of overexpression of ASA on the activity of other sulfatases was also tested in fibroblasts from patients with MLD using a retrovirus vector (MFG‐ASA). It was demonstrated that the overexpression of ASA reduces the activity of various sulfatases by a small amount but does not induce an accumulation of glycosaminoglycan. These results indicate that the influence of ASA overexpression on other sulfatases is different from that of the N‐acetygalactosamine‐4‐sulfatase overexpression in a previous report. It was concluded that the correction of ASA deficiency by a recombinant adenovirus that potentially could be used to transfer the gene to the brain, and gene therapy for MLD based on gene transfer of the ASA gene to mutant cells will be feasible because the overexpression of ASA in cells does not lead to profound deficiency of other sulfatases or result in a new phenotype.
Relationships between clinical and histological profiles of non‐familial idiopathic neonatal hepatitis
Authors: NISHINOMIYA FUJIHIKO , ABUKAWA DAIKI , TAKADA GORO , TAZAWA YUSAKU
Published: January 2011

Idiopathic neonatal hepatitis (INH) is a syndrome characterized clinically and histologically but there is little information concerning the relationship between the clinical features and histological findings. In the present study, sixty‐two patients clinically diagnosed as non‐familial INH were histologically classified into four groups according to a provisional definition based on predominant lesions and examination of their clinical features. Patients of cholestasis (n = 23) and giant cell hepatitis (GCH, n = 21) were most frequent (37% and 33%, respectively), and patients of fatty liver (n = 10) and hepatitis (n = 8) were less common (16% and 13%). The GCH group showed a dominance of male, low birthweight, older and breast‐fed babies. The cholestasis group demonstrated a dominance of male, low birthweight, younger and bottle‐fed babies. The hepatitis group had the highest frequencies of high‐grade hepatomegaly and splenomegaly. Fifty six cases completely recovered. Two died of hepatic failure in early infancy and four had chronic liver diseases at the age of 12 months. The fatty liver group had the worst outcome. Histological features in non‐familial INH were variable and typical giant cell hepatitis was seen in only one‐third of patients. Characteristic clinical features in each histologically classified group may suggest heterogenous etiologies underlying non‐familial INH.
Heart disease in Friedreich's ataxia: A clinical and echocardiographic study
Authors: GÜNAL NAZLIHAN , SARAÇLAR MUHSIN , ÖZKUTLU SÜHEYLA , ŞENOCAK FILIZ , TOPALOǦLU HALUK , KARAASLAN SEVIM
Published: January 2011

Twelve patients with Friedreich's ataxia (FA) were evaluated clinically and echocardiographically for evidence of heart disease. Electrocardiographic and echocardiographic abnormal findings were discovered in eight (67%) and seven (58%) children, respectively. A high incidence of cardiac involvement is well known in FA cases. Although the patient number in the present study is small, the findings are consistent with those in the literature. The most common pathology was asymmetric septal hypertrophy (ASH), followed by concentric left ventricular hypertrophy (CLVH) and dilated cardiomyopathy (DC).
Ethanol resistive microbubble test: A modification of the stable microbubble test used to predict respiratory distress syndrome
Authors: CHO KAZUTOSHI , CHIDA SHOICHI , SASAKI MIKA , FUJIWARA TETSURO
Published: January 2011

The stable microbubble (SM) test on gastric aspirate obtained at birth proved useful in identifying infants who would develop respiratory distress syndrome (RDS). This test involves only the count of stable microbubbles of ≤ 15 μm in diameter. Larger bubbles (> 15 μm in diameter) are not necessary for the test and may interfere with stable microbubble counting. The aims of the present study were to determine: (i) if larger bubbles could be selectively removed by adding ethanol, a potent bubble breaker; and (ii) if the predictive value of this modified test, the ethanol resistive microbubble (ERM) test, on the development of RDS was similar to that of the SM test. Varying amounts of different concentrations of ethanol‐water solutions were added to the top of the bubble crop generated by the SM test procedure, and the mean counts of stable microbubbles and larger bubbles in five regions were calculated. A volume of 10 μL of 47.5% ethanol was effective in defoaming larger bubbles generated by the SM test procedure without altering the stable microbubble counts. When concurrently performed on 43 samples of gastric aspirate obtained at birth from infants of less than 35 weeks gestation, the RDS predictive value of the ERM test was similar to that of the SM test. It was concluded that the ERM test may serve as an alternative to the SM test.
Relationship of feeding modality to clinical features in Japanese infants with idiopathic neonatal hepatitis of the non‐familial form
Authors: TAZAWA YUSAKU , NISHINOMIYA FUJIHIKO , ABUKAWA DAIKI , TAKADA GORO , KONNO TASUKE
Published: January 2011

To clarify the relationship between idiopathic neonatal hepatitis and feeding type, that is, formula‐milk feeding and breast‐milk feeding, the medical records of 100 patients (68 male and 32 female babies) with idiopathic neonatal hepatitis of non‐familial form referred to the medical centers of Akita University and Tohoku University during the past 18 years were reviewed. The patients were divided into two 9 year periods (1975–83 and 1984–92), and their clinical features were analyzed in terms of feeding type and sex. The number of patients enrolled decreased from 69 in the first half to 31 in the second half. The number of male patients dropped from 53 to 15, although the number of female patients (n = 16) remained the same in both 9 year periods. The frequency of formula‐milk feeding significantly decreased in the second half (42/69 to 6/31, P < 0.01). Compared with the expected numbers of patients in the second half, calculated on the changes in the live birth population and feeding modality between the two halves, the actually enrolled numbers of patients in the second half were less in both the male and the formula‐milk fed groups (× 0.35 and × 0.22), whereas the numbers of female and breast‐milk feeding groups were close to the expected values (× 1.26 and × 1.08). When sex and feeding modality were combined, the formula‐milk fed male group showed the lowest value (× 0.10), and the breast‐milk fed female group showed the highest value (× 2.85). In conclusion, feeding type, especially in combination with gender, might be one causative factor in the occurrence of idiopathic neonatal hepatitis.
The role of SRY in mammalian sex determination
Authors: FECHNER PATRICIA Y
Published: January 2011

The gene SRY (sex determining region of the Y), located at the distal region of the short arm of the Y chromosome, is necessary for male sex determination in mammals. SRY initiates the cascade of steps necessary to form a testis from an undifferentiated gonad. The SRY gene encodes an HMG (High Mobility Group) protein which may act as a transcription factor by binding to double stranded DNA and then bending the DNA. Mutations in SRY have been identified in some subjects with 46,XY pure gonadal dysgenesis. However the role for other autosomal and X‐linked genes in testis determination is evident by the presence of a normal SRY gene in the majority of females with 46,XY pure gonadal dysgenesis and the lack of SRY in a minority of males with 46,XY maleness.
Sex determining gene on the X chromosome short arm: Dosage sensitive sex reversal
Authors: OGATA TSUTOMU , MATSUO NOBUTAKE
Published: January 2011

The present review article summarizes current knowledge concerning the sex determining gene on Xp21, termed DSS (dosage sensitive sex reversal). The presence of DSS has been based on the finding that, in the presence of SRY, partial active Xp duplications encompassing the middle part of Xp result in sex reversal, whereas those of the distal or proximal part of Xp permit male sex development. Because Klinefelter patients develop as males, it is believed that DSS is normally subject to X‐inactivation, and that two active copies of DSS override the function of SRY, resulting in gonadal dysgenesis because of meiotic pairing failure. It may be possible that DSS encodes a target sequence for repressing function of SRY or that DSS is involved in an X chromosome‐counting mechanism. Molecular approaches have localized DSS to a 160 kb region and isolated candidate genes such as DAX‐1 and MAGE‐Xp, but there has been no formal evidence equating the candidate gene with DSS. In addition to its clinical importance, the exploration of DSS must provide a useful clue to phylogenetic studies of sex chromosomes and dosage compensation.
Continuous subcutaneous insulin injection for self‐care of young patients with insulin‐dependent diabetes mellitus
Authors: KUNO TATEO , TASAKI HAKARU , MIYAZAKI SUMIO
Published: January 2011

Continuous subcutaneous insulin injection was used for the self‐care of five patients aged 10–18 years with insulin‐dependent diabetes mellitus. After an introduction to the concept and procedures for continuous subcutaneous injection, the patients soon became familiar with self‐care using an insulin pump at home and at school. Three months later, the control of blood glucose improved with smaller doses of insulin in four cases and milder hypoglycemia was observed compared to when using multiple injections. Significantly decreased variations and lowered means of early morning blood glucose values were observed and seemed to explain the reason for better glycemic control. Buffered regular insulin continuously injected by pumps brought a more stable nocturnal blood glucose level compared to isophane insulin injected at bedtime, the absorption of which seemed to vary considerably. On the contrary, unbuffered regular insulin injected by pumps brought frequent nocturnal hypoglycemia, sudden worsening of glycemic control and skin infections and thus, was deemed inadequate for continuous subcutaneous injection.
Clinico‐epidemiological features of infantile hydrocephalus in Japan
Authors: NAKASHIMA SACHIE , WATANABE KAZUYOSHI , NEGORO TAMIKO , AOKI KUNIO , KIKUCHI HARUHIKO
Published: January 2011

In 1988, a nationwide survey was conducted in Japan, in order to examine the prevalence and clinico‐epidemiological features of hydrocephalus that developed before 1 year of age. The study was based on questionnaires that were sent to all hospitals in Japan with more than 200 beds. There were 1435 patients who were eligible for the analysis. They were born before 1987 and diagnosed as having infantile hydrocephalus. Approximately two‐thirds of the patients had primary hydrocephalus. Neural tube defects were the most frequent cause of the primary hydrocephalus and common among full‐term infants. On the other hand, secondary hydrocephalus occurred more often in preterm infants, mainly following intracranial hemorrhage. Intracranial hemorrhage as a cause of hydrocephalus increased over the year, while the incidence of hydrocephalus secondary to intracranial infection decreased during the same period.
Developmental changes in electroencephalogram for term and preterm infants using an autoregressive model
Authors: SAWAGUCHI HIROTO , OGAWA TERUYUKI , TAKANO TOMOYUKI , SATO KEISUKE
Published: January 2011

The purpose of the present study was to clarify the developmental changes of electroencephalogram (EEG) background activity in term and preterm infants that take place with increasing conceptional age (CA) through an autoregressive (AR) model. Polygraphical EEG recordings were obtained from 76 healthy term and preterm infants with a CA ranging between 31 and 40 weeks. Decreases in total power and component power of δ were noted with CA during burst activity (BA) in quiet sleep (QS) and during active sleep (AS). Increases in total power and component power of δ were noted with CA during interburst interval activity (IBIA) of QS. Regression analysis (RA) of the information amount (IA) indicated a negative correlation with increasing CA in AS and in the monopolar EEG tracings Fp1, C3, O1 and O2 in BA, and a positive correlation in O1 in IBIA. Regression analysis of the IA of δ indicated a negative correlation with increasing CA in AS and in Fp1, C3, O1 and O2 in BA, and a positive correlation in O1 in IBIA. Regression analysis of the IA of δ indicated a negative correlation with increasing CA in BA and AS. These results showed that the high voltage slow wave component changed to a low voltage slow one with development in AS and that BA was longer in duration and lower in power with increasing CA, while IBIA was shorter in duration and higher in power. In conclusion, significant developmental changes occur in all derivatives of AS. Even though the EEG of BA and IBIA change separately, they are followed by EEG of QS in a continuous pattern.
Childhood asthma and atmospheric conditions
Authors: YÜKSEL HASAN , TANAÇ REMZİYE , TEZ ECE , DEMIR ESEN , ÇOKER MAHMUT
Published: January 2011

Bronchial asthma is the most common chronic respiratory illness in childhood. It is characterized by paroxysmal bronchospastic periods. There are many studies giving reasons to explain the bronchospasm periods. One of the reasons, atmospheric conditions, is effective in creating a clinical picture of asthmatic patients. In the present study, the correlation between atmospheric conditions and asthmatic symptoms in children was investigated using peak expiratory flow rate (PEFR) as the respiratory function test. Twenty‐one children with bronchial asthma were monitored in the study. They were followed as outpatients of the Ege University Medical Faculty, Department of Pediatric Allergy and Pneumotology, between November 1993 and June 1994. Atmospheric conditions were recorded from the local meteorology center. Complaints and the PEFR of children were compared with the meteorological data. Asthmatic symptoms were increased by low temperatures in all asthmatic children. An increase was detected in the extrinsic group by relative humidity and ratio of cloud, but in the intrinsic group only by relative humidity.
Autonomic nervous system functions in childhood essential hypertension
Authors: YAKINCI CENGİZ , MÜNGEN BÜLENT , TAYFUN MELTEM , GÜNDÜZ MEHMET , KARABİBER HAMZA
Published: January 2011

The etiopathology of essential hypertension (EH) is still controversial, different pathologic mechanisms have been estimated as a cause of EH. One of these mechanisms is autonomic nervous system dysfunction (ANSD). Although there are different studies showing ANSD in adult EH, limited studies on ANSD in childhood EH are present. The present study was planned to investigate the role of ANSD in childhood EH. The study group (SG) consisted of 24 children with EH and the control group (CG) was 24 healthy children. Non‐invasive autonomic nervous system function tests: the orthostatic test, Valsalva ratio, 30/15 ratio and heart rate responses to deep breathing were performed on members of both groups. The mean differences in systolic blood pressure between supine resting and standing positions in the study and control groups were significant (z = −2.577, P = 0.0051). The mean differences in diastolic blood pressure between supine resting and standing positions in SG and CG were found to be statistically meaningful (z= −2.722, P = 0.0033). The mean differences in heart rate variation during hyperventilation in SG and CG were significant (z = −2.54, P = 0.0055). The mean differences in the Valsalva ratio between SG and CG were not statistically meaningful (z = 1.52, P = 0.0643). The mean differences in the 30/15 ratio in SG and CG were not found to be significant (z= −1.455, P = 0.0735). In the light of these results, both sympathetic and parasympathetic autonomic nervous system dysfunction in childhood essential hypertension were found.
Treatment of aplastic anemia with antithymocyte globulin, cyclosporin A, methylprednisolone, danazole and recombinant human granulocyte‐colony stimulating factor
Authors: SHICHINO HIROYUKI , MUGISHIMA HIDEO , TAKAMURA MAYUMI , SHIMADA TOSHIAKI , SUZUKI TAKASHI , CHIN MOTOAKI , HARADA KENSUKE , RYO SIGEO , KOJIMA SEIJI
Published: January 2011

The main purpose of the present study was to determine the response rate to immunosuppressive therapy combined with recombinant human granulocyte‐colony stimulating factor (rhG‐CSF) and its efficacy for preventing infections in patients with severe aplastic anemia. The treatments included one course of antithymocyte globulin, cyclosporin A, methylprednisolone, danazole and rhG‐CSF. Three patients had very severe aplastic anemia and two had moderate aplastic anemia. One patient relapsed 13 months following the first course of therapy and received a second course. Five patients received six courses of treatment and the response rate at 6 months was 83.3%. All patients achieved an absolute neutrophil count of greater than 1.0 × 109/L within 40 days. All patients with a complete response are transfusion‐free and doing well. All five patients are currently alive and have not had any episode of infection for 17–53 months. The results of the study indicate that this therapy may improve the poor prognosis of young patients with severe aplastic anemia. It has a good response rate and induces a rather rapid increase in the neutrophil count, which protects against life‐threatening bacterial and fungal infections.
Comparison of specific immunities to varicella‐zoster virus following post‐exposure prophylaxis of varicella by oral acyclovir observed in a family
Authors: ASANO YOSHIZO , SUGA SADAO , YOSHIKAWA TETSUSHI
Published: January 2011

An otherwise healthy 3‐year‐old girl developed severe varicella complicated by aseptic meningitis and received intravenous acyclovir (ACV) therapy. Her two siblings who were susceptible to varicella‐zoster virus (VZV) and closely exposed to VZV in the family received oral ACV (45 or 54 mg/kg per day in four divided doses for 7 days) starting 8 days after onset of the index case for post‐exposure prophylaxis of varicella. They showed only five or seven papules over the body without fever 12 days after onset of the index case, while they had one‐third or half the level of antibody titer and equal sized skin reactions to VZV antigen of the index case 2.5 months later.
Serum level and gene polymorphism of angiotensin I converting enzyme in Japanese children
Authors: YAMAMOTO KOHSUKE , KATAOKA SATOSHI , HASHIMOTO NAOSHI , KAKIHARA TOSHIO , TANAKA ATSUSHI , KAWASAKI TAKUYA , KIKUCHI TORU , TAKAHASHI HIDEO , UCHIYAMA MAKOTO
Published: January 2011

The aim of the present study was to determine the distribution of the insertion/deletion polymorphism of angiotensin I converting enzyme (ACE) gene in Japanese children. In addition, the relationship between this polymorphism and serum ACE levels in the same population were analyzed. Insertion/deletion polymorphism located in intron 16 of the ACE gene was examined by polymerase chain reaction in Japanese children aged 10–15 years. Allele frequencies were 0.64 for the insertion allele and 0.36 for the deletion allele in 122 subjects. No association was found between genotypes in this polymorphism and the level of systolic or diastolic blood pressure. A significant relationship between this polymorphism and serum ACE activity was observed. These results suggest that interindividual variability of serum ACE level is strongly influenced by the ACE genotype as early as in childhood.
Aflatoxin: Is it a neglected threat for formula‐fed infants?
Authors: AKŞIT SADIK , ÇAǦLAYAN SUAT , YAPRAK ISIN , KANSOY SAVAŞ
Published: January 2011

In the present study, the risk of exposure to aflatoxin in infants fed breast milk and formula was investigated. For this purpose, aflatoxin B1 (AFB1) was determined in the serum of both breast‐fed and formula‐fed infants. Serum AFB1 positivity was significantly higher in the formula‐feeding (F) group than the breast‐feeding (B) group (42.8 vs 8.5%, P <0.01). The AFB, concentration in different commercial formulas was also determined. Aflatoxin B1 was found in seven of the eight newly opened packages of different brands of formula. The concentrations showed a statistically significant increase at the 30th day after opening the packages (P <0.01). Although AFB1 concentrations in the formulas were found to be within acceptable limits for most countries, still, its existence must be carefully evaluated because future influences of very small amounts of aflatoxin on the growing organism have not been fully elucidated. Therefore, it was again concluded that for infants, human milk is safer than commercial formulas because of the lower contamination risk of aflatoxin. Also, commercial formulas must be regularly examined by authorities for the possible risk of aflatoxin contamination.
Rapid, sensitive and specific diagnosis of Bordetella pertussis using the polymerase chain reaction
Authors: AOYAMA TATSUO , TAMURA CHIEKO , TAKEUCHI YOSHINAO , KAMIMURA TAKASHI , IMAIZUMI ATSUSHI
Published: January 2011

Use of a repetitive DNA sequence of Bordetella pertussis allowed successful detection of the organism by the polymerase chain reaction (PCR). The method was highly sensitive, being able to detect B. pertussis in specimens containing only a few cells. It was also highly specific, with no amplification of specimens containing other organisms, for example Haemophilus influenzae or Neisseria, being observed. A diagnosis could be made within 1 day. The PCR assay was also evaluated in clinical specimens. Among 47 nasopharyngeal specimens obtained from 24 patients with laboratory‐confirmed pertussis, 27 were positive by PCR and 19 by culture. In particular, all three bronchial aspirates from one patient with pertussis were positive by PCR, but only one showed positive on culture. Eleven specimens from parapertussis patients and 65 specimens from patients without pertussis tested negative. It was concluded that this newly developed PCR method for the diagnosis of pertussis was more rapid and sensitive than the usual culture method. Polymerase chain reaction could have a major impact on the treatment and control of this infection and would be a useful tool for studying the pathogenesis of B. pertussis infection.
2‘,5’‐oligoadenylate synthetase activity and T cell subset in the cerebrospinal fluid and peripheral blood of aseptic meningitis
Authors: SAKATOKU HIROYUKI , INOUE MASAKAZU , KOJIMA MASAMITSU , AZUMA EIICHI , KOMADA YOSHIHIRO , ZHOU YAN‐WEN , ZHANG SHAO‐LI , SAKURAI MINORU
Published: January 2011

2′,5′‐oligoadenylate synthetase activity, which is assumed to be induced by interferon, is reported to be one of the useful markers reflecting interferon activity. The enzyme activity of patients with aseptic meningitis and febrile convulsion were compared in order to evaluate interferon activity as one of the local immuno‐defense mechanisms of aseptic meningitis. The surface antigen of mononuclear cells in cerebrospinal fluid and peripheral blood of some patients with aseptic meningitis was also measured. The enzyme activity of patients with aseptic meningitis was 191.4 pmol/dL in the cerebrospinal fluid and 395.8 pmol/dL in the serum during the acute phase, while that of patients with febrile convulsion was 45.2 pmol/dL in the cerebrospinal fluid and 326.0 pmol/dL in the serum. The enzyme activity of the former patients significantly decreased during the recovery phase in both the cerebrospinal fluid and serum. CD3 positive cells in the peripheral blood were 56.3% of the total mononuclear cells during the acute phase and 65.2% during the recovery phase, whereas in the cerebrospinal fluid mononuclear cells, they were 87.1 and 85.5%, respectively. During the acute phase, CD4 positive cells were the predominant T lymphocyte subset in the cerebrospinal fluid cells, while CD8 positive cells were predominant during the recovery phase. The relative proportions of CD4 positive and CD8 positive cells during the acute and recovery phase in the cerebrospinal fluid mononuclear cells were quite high compared to the recovery phase, although that ratio of peripheral blood mononuclear cells was not changed throughout the course. It was concluded that T lymphocytes and increased 2′,5′‐oligoadenylate synthetase activity in the cerebrospinal fluid may be one of the important components in the local inflammatory process independent of the systemic host defense mechanism in aseptic meningitis.
Detection of the mutation in facioscapulohumeral muscular dystrophy patients
Authors: OHYA KAZUHIRO , TACHI NOBUTADA , KOZUKA NAOKI , KON SHIN‐ICHIRO , KIKUCHI KOKICHI , CHIBA SHUNZO
Published: January 2011

The gene responsible for facioscapulohumeral muscular dystrophy (FSHD) was mapped to chromosome 4q35 by linkage analyses. Recently, the probe p13E‐11 derived from the cosmid clone 13E, which has been mapped to 4qter, detected a polymorphic EcoRI fragment, usually greater than 28 kb in normal individuals. In sporadic and familial FSHD patients, a specific shorter fragment, usually smaller than 28 kb, was found to cosegregate with FSHD. Two FSHD patients are presented here. Patient 1 is a sporadic case of FSHD with healthy parents. He had a de novo mutation identified by Southern blot analysis using the above‐mentioned probe. Patient 2 is a typical familial FSHD patient clinically and histologically. A rearranged and shortened EcoRI fragment was identified by molecular analysis. Southern blot analysis using the probe p13E‐11 also indicated a rearranged EcoRI fragment in both patients.
Pharmacokinetics of antibiotics in children
Authors: TOYONAGA YOSHIKIYO
Published: January 2011

In designing the dosage regimen of antibiotics including β‐lactams in the field of pediatrics, the choice of adequate drugs, as well as the dosage and the number of administrations have not been performed exactly. This is possibly because of the extended spectrum of antibacterial activity in recent drugs. Even the timing of discontinuation has not been seriously considered. The present report mainly concentrates on several cephalosporins and demonstrates the pharmacokinetic characteristics of the drugs, and further, describes the difference in pharmacokinetic parameters between age groups. On the other hand, it has been suggested that based on the minimum inhibitory concentration for 80% (MIC80) of antibiotics against subject micro‐organisms, the effective area under the concentration‐time curve, time above MIC80 and Cmax/MIC80 should be considered and the suitability of drug choice, the dosage of suitable drugs, and the number of administrations should be decided. The dosage regimen in the present study was not designed to take into account the effects of sub‐MIC and post‐antibiotic effect. However, for monotherapy, the dosage regimen was designed to maintain the concentration of at least MIC, which is sufficient from the viewpoint of saving life.
Adverse effects of antibiotics
Authors: IWATA SATOSHI , AKITA HIRONOBU
Published: January 2011

The adverse effects caused by antibiotics and the interactions between other drugs based on the results of clinical studies on children recently conducted in Japan, as well as the results of previous studies in the literature were reviewed. Adverse effects of β‐lactam, macrolide and azalide antibiotics commonly observed in children included gastrointestinal symptoms such as diarrhea and loose stool, and hypersensitivity such as rash and fever. The incidences were 1–6% and 0.2–1.6%, respectively. Eosinophilia, thrombocytosis, and elevation of serum glutamate oxaloacetate transaminase and serum glutamate pyruvate transaminase were common abnormal laboratory findings. Although many of the antibiotics used in childen are relatively safe, cautions should be given because developing children tend to have adverse effects unique or common in children, in addition to those commonly seen in adults.
Nitrogen dioxide production in a nitric oxide inhalation system using the Servo Ventilator 900C
Authors: SATO TOSHIHIDE , OKAMOTO KAZUFUMI , KUKITA ICHIRO , KIKUTA KOICHI , HAMAGUCHI MASAMICHI , SHIIHARA KAZUHIKO , SHIBATA YOSHIHIRO
Published: January 2011

During nitric oxide (NO) inhalation therapy, toxicity may be produced by the reactive metabolite nitrogen dioxide (NO2). The purpose of the present study was to determine the NO2concentration in a NO inhalation system used for respiratory failure in children at relatively low concentrations of NO (< 20 ppm). The production of NO2in the NO inhalation system using the Servo Ventilator 900C connected to the test lung under each of 30 combinations of NO concentrations (0, 4, 8, 12, 16, and 19 ppm) and inspired oxygen (O2) concentrations (21, 40, 60, 80, and 100%). Pressure controlled ventilation was used with a respiratory rate of 20 breaths/min. NO and NO2measurements were obtained on the inspiratory side of the Y‐piece connected to the test lung. At a given NO level, increases in the concentration of inspired O2resulted in increases in the concentration of NO2produced, as did increases in the amount of NO at a given concentration of O2. The mean NO2concentration at the inspiratory site of the Y‐piece did not exceed 0.05 ppm (the limit of NO2as an outdoor air pollutant in the United States) when the NO concentration did not exceed 8 ppm, regardless of the O2concentration. NO inhalation therapy for children with severe respiratory failure using the Servo Ventilator 900C can be performed safely when the concentration of NO does not exceed 8 ppm.
Agranulocytosis and thrombocytopenic purpura following measles infection in a living‐related orthotopic liver transplantation recipient
Authors: ARAKAWA YOICHI , MATSUI AKIRA , SASAKI NOBUHIKO , NAKAYAMA TETSUO
Published: January 2011

Agranulocytosis and thrombocytopenic purpura occurred simultaneously following measles infection in a 2‐year‐old girl who had received a living‐related orthotopic liver transplantation. Neutrophil and platelet counts rose to more than 0.5 × 103/mm3 and 30 × 103/mm3, respectively, 8 weeks after the onset of neutropenia and thrombocytopenia, and within 2 weeks from the start of subcutaneous injection of granulocyte colony‐stimulating factor. Simultaneous occurrence of measles infection, neutropenia and thrombocytopenia has not been described before in the literature. An autoimmune mechanism was suspected because serum levels of platelet‐associated immunoglobulin G (IgG) and anti‐neutrophil antibody were elevated during the acute phase.