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Background: The aim of the present study was to evaluate the role of interleukin (IL)-6-634 polymorphism in neonatal disorders such as bronchopulmonary dysplasia (BPD) and periventricular leukomalacia (PVL) in very low-birthweight (VLBW) infants. Methods: This prospective cohort study included 202 infants (gestational age at birth, 23–34 weeks; birthweight, 500–1499 g). Genotypic analysis (polymerase chain reaction–restriction fragment length polymorphism) was performed with DNA extracted from whole-blood samples. Results: Genotype distribution (66.8% CC, 28.2% CG, 5.0% GG) was similar to that in the adult Japanese population. BPD occurred in 85 infants (42.1%) among 202 VLBW infants. The duration of O2 therapy in infants with CG/GG genotypes was significantly longer than that in infants with the CC genotype (CG/GG vs CC: 40.3 ± 52.2 days vs 28.4 ± 32.6 days, P < 0.05), but the prevalence of BPD was not associated with the CG/GG genotype (CG/GG, 40.0%; CC, 46.3%, P= 0.24). Infants with CG/GG genotypes were more likely to have received postnatal corticosteroid therapy for BPD than those with the CC genotype (CG/GG vs CC: 20.9% vs 11.1%, P= 0.05). PVL occurred in six infants (3.0%). There was no significant difference in the prevalence of PVL among IL-6-634 polymorphisms (CG/GG, 3.0%; CC, 3.0%, P= 0.65). Conclusions: IL-6-634 polymorphism is associated with duration of oxygen therapy in VLBW infants. This suggests that the IL-6-634 polymorphism G allele is an aggravating factor of BPD. IL-6-634 polymorphism is not associated with PVL.

Background: Children with a history of low birthweight (LBW) are often hospitalized with plural episodes of pneumonia after discharge from the neonatal intensive care unit. The aim of this study was to clarify the multiple factors predisposing them to developing three or more hospitalizations with pneumonia and whether the factors are related to their own prematurity. We also aimed to determine a predictable numerical formula for three or more episodes. Methods: Fourteen patients with two hospitalizations with pneumonia were grouped into group A. Fourteen patients with at least three episodes during the same investigation period were grouped into group B. The quantification theory type III was employed to investigate the similarities among the items and the gravity of each attribution in the two groups. To evaluate the items of discrimination of both groups, six items were analyzed by the quantification theory type II. Results: The dominant order of items contributing to the grouping was as follows: methicillin-resistant staphylococcus aureus detection (partial correlation coefficient = 0.5284), asthmatic attack (partial correlation coefficient = 0.4138), severe motor and intellectual disability, Haemophilus influenzae, accompanying diseases and chronic lung disease. A predicting numerical formula was attained from these results. The success rate of discrimination was 85.7%. The six items seemed to be related to the patients' own prematurity. Conclusions: The authors emphasize that plural hospitalizations with pneumonia in the patients with LBW might be caused by the combined influence of six clinical factors as well as their own prematurity.

Background— Electrical storms (ESs) in patients with Brugada syndrome (BrS) are rare though potentially lethal. Methods and Results— We studied 22 men with BrS and ES, defined as ≥3 episodes/d of ventricular fibrillation (VF) and compared their characteristics with those of 110 age-matched, control men with BrS without ES. BrS was diagnosed by a spontaneous or drug-induced type 1 pattern on the ECG in the absence of structural heart disease. Early repolarization (ER) was diagnosed by J waves, ie, >0.1 mV notches or slurs of the terminal portion of the QRS complex. The BrS ECG pattern was provoked with pilsicainide. A spontaneous type I ECG pattern, J waves, and horizontal/descending ST elevation were found, respectively, in 77%, 36%, and 88% of patients with ES, versus 28% ( P P =0.003), and 60% ( P =0.06) of controls. The J-wave amplitude was significantly higher in patients with than without ES ( P =0.03). VF occurred during undisturbed sinus rhythm in 14 of 19 patients (74%), and ES were controlled by isoproterenol administration. All patients with ES received an implantable cardioverter defibrillator and over a 6.0±5.4 years follow-up, the prognosis of patients with ES was significantly worse than that of patients without ES. Bepridil was effective in preventing VF in 6 patients. Conclusions— A high prevalence of ER was found in a subgroup of patients with BrS associated with ES. ES appeared to be suppressed by isoproterenol or quinidine, whereas bepridil and quinidine were effective in the long-term prevention of VF in the highest-risk patients.

Background: Children with orthostatic dysregulation (OD) appear to have hypodynamia, as well as the symptoms described in the OD criteira. Hypodynamia, which is greatly influenced by motivation, volition and concentration, is unexceptionally recognized in their everyday life. It has been suggested that the symptoms and hypodynamia aggravate considerably the quality of life (QOL) of children with OD. The purpose of this study was to distinguish the characteristics of contingent negative variation (CNV) and post imperative negative variation, which may reflect the level of attention and motivation in children with OD. Methods: Twelve patients with OD aged 10–15 years and 23 age-matched healthy children were included. The CNV was recorded from Fz, Cz and Pz linked to earlobes during 30 trials consisting of a warning stimulus and an imperative stimulus with an interstimulus interval (ISI) of 2 s and an intertrial interval (ITI) of 10 s. The imperative stimulus of each trial required a button to be pressed. Results: The untreated children with OD did not have a significantly smaller CNV amplitude than healthy children. Children with OD treated with midodrine and autonomic training had a significantly larger CNV amplitude than the untreated children, in the area of early, late and total CNV at the three sites. Conclusion: The present study confirms that children with OD have diminished motivation and deterioration of concentration, which cause hypodynamia in everyday life. Treatment for OD improves the symptoms, diminished motivation and deterioration of concentration, consequently restoring dynamia. Treatment for OD should be recommended to ameliorate QOL of children with OD.

Background: Emission from rice straw burning (ERSB) is observed everywhere after harvest of rice in Niigata Prefecture every year. Pediatricians and many guardians in this district have had the impression that ERSB may induce asthma attack. Recent studies have suggested that particulate air pollution plays a role in the exacerbation of asthma. The authors investigated relationship of ERSB to asthma attack in children. Methods: A questionnaire on rice straw burning (RSB) was circulated to guardians and pediatric institutions. Change in the monthly number of children with asthma attack (CAA) for 5 years from January 1994 to December 1998 was investigated. In addition, change in the number of CAA from the meteorologic conditions and RSB was investigated from the fourth week of August to the third week of September in 1996, 1997 and 1998. Challenge test exposure to ERSB was tried on a volunteer adult with chronic asthma. The situation of air pollution was examined by measuring suspended particulate matter (PM10). The relationship between PM10 and the number of CAA was studied. Results: A majority of the guardians had the impression that ERSB induces asthma attack. Pediatricians replied similarly to the questionnaire. The number of CAA visiting our emergency room and admitted to our ward increased in the season of RSB. The PM10 had a significant correlation with the number of CAA. It was suggested that the increase in CAA may be not due to the meteorologic conditions, but to the influence of ERSB. Conclusion: The ERSB has made an issue of air pollution. Furthermore, the possibility that ERSB induces or exacerbates asthma attack has become clear in the present study. Therefore, it is recommended that RSB should be abolished for the health of inhabitants, especially children with asthma.

The aim of the present study was to evaluate the role of interleukin (IL)-6-634 polymorphism in neonatal disorders such as bronchopulmonary dysplasia (BPD) and periventricular leukomalacia (PVL) in very low-birthweight (VLBW) infants.This prospective cohort study included 202 infants (gestational age at birth, 23-34 weeks; birthweight, 500-1499 g). Genotypic analysis (polymerase chain reaction-restriction fragment length polymorphism) was performed with DNA extracted from whole-blood samples.Genotype distribution (66.8% CC, 28.2% CG, 5.0% GG) was similar to that in the adult Japanese population. BPD occurred in 85 infants (42.1%) among 202 VLBW infants. The duration of O(2) therapy in infants with CG/GG genotypes was significantly longer than that in infants with the CC genotype (CG/GG vs CC: 40.3 ± 52.2 days vs 28.4 ± 32.6 days, P0.05), but the prevalence of BPD was not associated with the CG/GG genotype (CG/GG, 40.0%; CC, 46.3%, P= 0.24). Infants with CG/GG genotypes were more likely to have received postnatal corticosteroid therapy for BPD than those with the CC genotype (CG/GG vs CC: 20.9% vs 11.1%, P = 0.05). PVL occurred in six infants (3.0%). There was no significant difference in the prevalence of PVL among IL-6-634 polymorphisms (CG/GG, 3.0%; CC, 3.0%, P = 0.65).IL-6-634 polymorphism is associated with duration of oxygen therapy in VLBW infants. This suggests that the IL-6-634 polymorphism G allele is an aggravating factor of BPD. IL-6-634 polymorphism is not associated with PVL.

Children with a history of low birthweight (LBW) are often hospitalized with plural episodes of pneumonia after discharge from the neonatal intensive care unit. The aim of this study was to clarify the multiple factors predisposing them to developing three or more hospitalizations with pneumonia and whether the factors are related to their own prematurity. We also aimed to determine a predictable numerical formula for three or more episodes.Fourteen patients with two hospitalizations with pneumonia were grouped into group A. Fourteen patients with at least three episodes during the same investigation period were grouped into group B. The quantification theory type III was employed to investigate the similarities among the items and the gravity of each attribution in the two groups. To evaluate the items of discrimination of both groups, six items were analyzed by the quantification theory type II.The dominant order of items contributing to the grouping was as follows: methicillin-resistant staphylococcus aureus detection (partial correlation coefficient = 0.5284), asthmatic attack (partial correlation coefficient = 0.4138), severe motor and intellectual disability, Haemophilus influenzae, accompanying diseases and chronic lung disease. A predicting numerical formula was attained from these results. The success rate of discrimination was 85.7%. The six items seemed to be related to the patients' own prematurity.The authors emphasize that plural hospitalizations with pneumonia in the patients with LBW might be caused by the combined influence of six clinical factors as well as their own prematurity.

The Tokyo Children's Cancer Study Group (TCCSG) has performed prospective randomized controlled studies since 1984 for childhood acute lymphoblastic leukemia (ALL). Four hundred and ninety-eight newly diagnosed patients were treated with 5 different regimens designated as S1, S2 for a standard risk group (SRG), H1 and H2 for a high risk group (HRG) and HEX for an extremely high risk group of patients. We added intermediate-dose methotrexate as early intensification therapy in the S2 and H2 groups to reduce extramedullary and medullary relapses. Event-free survival of all patients at 4 years 6 months from the start of this regimen (median follow-up period 32 months) was 67.5%. The CNS relapse rate was significantly decreased to 2.2% compared to previously reported studies and our own experience. There were some unexpected complications of the CNS such as myelopathy and/or leukoencephalopathy. For the SRG early ID-MTX seemed to be useful to improve the prognosis, but triple intrathecal injections (TIT) should be limited in number. The 24Gy cranial irradiation (CRX) was effective but possibly excessive for the HRG. As evidenced by the success of the HEX group regimen, more intensive multi-drug consolidation in the early post-remission phase might be necessary to improve further the prognosis and to make it possible to shorten the treatment periods.

Background: Although elective cesarean sections are often performed after the 37th week of gestation without any complicating factor that may influence the timing of delivery, there is a possibility that infants born in the 37th week of gestation, especially early in the 37th week of gestation, do not obtain a satisfactory clinical outcome due to premature birth. Methods: The authors analyzed the clinical course during the neonatal period in 96 infants born in the 37th (n = 81) and 38th (n = 15) week of gestation by an elective cesarean section. Subjects were retrospectively divided into two groups: infants born in the first half of the 37th week of gestation (37 + 0 -37 + 3 ) (n = 48), and infants born from the latter half of the 37th week of gestation (37 + 4 -37 + 6 ) through the 38th week of gestation (n = 48). Twin pregnancies, pregnancy with placenta previa. and pregnancy of women who had a diversity of medical complications were excluded from the present study, because of the possibility that these conditions may have affected the infants' status. The incidence of infants who showed clinical symptoms during the neonatal period and who needed medical care was compared between the two groups. Results: Of the 96 subjects, 25 infants (26.0%) had significant clinical symptoms. The incidence of breathing difficulty was significantly higher in the infant group born in the first half of the 37th week of gestation than in the latter group. Conclusions: An elective cesarean section before the onset of labor early in the 37th week of gestation should not be routinely undertaken.

We report a 6-month-old Japanese infant with a malignant form of Brugada syndrome, who had frequent episodes of ventricular fibrillation (VF) and nonsustained polymorphic ventricular tachycardia (VT). To the best of our knowledge, this infant is the youngest patient reported to have Brugada syndrome. Continuous infusion of a beta-adrenergic agonist and intravenous injection of a parasympathetic antagonist suppressed the electrical storm of polymorphic VT and VF. Combined oral administration of a beta1-adrenergic agonist, a parasympathetic antagonist, and quinidine has successfully suppressed recurrences of VT or VF for 6 months, and the combination may have the potential to decrease the incidence of VT or VF as an adjunctive therapy with prophylactic placement of an implantable cardioverter defibrillator.

Uniparental disomy (UPD) is defined as the presence of a chromosome pair that derives from only one parent in a diploid individual. The human TRKA gene on chromosome 1q21-q22 encodes a receptor tyrosine kinase for nerve growth factor and is responsible for an autosomal recessive genetic disorder: congenital insensitivity to pain with anhidrosis (CIPA). We report here the second case of paternal UPD for chromosome 1 in a male patient with CIPA who developed normally at term and did not show overt dysmorphisms or malformations. He had only the usual features of CIPA with a homozygous mutation at the TRKA locus and a normal karyotype with no visible deletions or evidence of monosomy 1. Haplotype analysis of the TRKA locus and allelotype analyses of whole chromosome 1 revealed that the chromosome pair was exclusively derived from his father. Non-maternity was excluded by analyses of autosomes other than chromosome 1. Thus, we have identified a complete paternal isodisomy for chromosome 1 as the cause of reduction to homozygosity of the TRKA gene mutation, leading to CIPA. Our findings further support the idea that there are no paternally imprinted genes on chromosome 1 with a major effect on phenotype. UPD must be considered as a rare but possible cause of autosomal recessive disorders when conducting genetic testing.

Background: Abnormal sympathetic skin response (SSR) has been reported in adult patients with diabetic neuropathy. In addition, other studies have revealed abnormal SSR in diabetic patients not having autonomic symptoms and autonomic dysfunctions. These findings have been only obtained from adult patients. There have been few reports on the autonomic functions in diabetic children. Accordingly, it is not clear whether the autonomic neuropathy occurs in diabetic children. The aim of the present study is to clear autonomic function in children with insulin-dependent diabetes mellitus by SSR. Methods: The SSR was measured in 28 normal healthy children and in eight patients with IDDM not having symptoms of dysautonomia. The SSR was elicited using 10 stimuli on programmed Nihonkoden Neuropack Sigma model machine. Following a single electrical stimulation, four SSR were recorded in both the palms and the soles simultaneously. Results: The SSR were simultaneously obtained in 100% of the two groups. The amplitudes in the palms and soles were not significantly different between the two groups. The mean and shortest latency in the soles were significantly longer in the IDDM group than in the control group (P

Background: It is a matter of concern whether serum eosinophil cationic protein (ECP) can be considered as a disease marker in children with acute asthma being treated without corticosteroids. Methods: Fourteen children (nine male, five female, aged 6–12 years) with acute asthmatic exacerbation, administered the appropriate drugs, with the exception of systemic or inhaled corticosteroids, were examined. They were all free from apparent asthmatic attacks during a follow-up period of 1 month. Serum ECP, eosinophil count and forced expiratory volume in 1 s (FEV1)_ were measured at referral, on the day of discharge, 1 week and 4 weeks after discharge, respectively. Results: The ratio of ECP/eosinophil count (ECP:Eo ratio), expressed as micrograms of ECP (μg/L)/the number of eosinophil (/μL) x 1000, was also evaluated as a marker of eosinophil activation. Compared with the value at referral, FEV1 (% predicted) significantly increased on the day of discharge (P

The intra-abdominal visceral fat to subcutaneous fat ratio (V/S ratio) has been reported to be strongly related to disorders of glucose and lipid metabolism, and hypertension. It is a matter of concern as to whether weight loss causes an improvement of the V/S ratio or not in obese children. Changes in body fat distribution during weight loss in 23 obese children were quantified by weight, bioelectrical impedance analysis (BIA) and computed tomography (CT scan of the abdomen). Twenty-three patients were divided into two groups; six were in the inpatient group and 17 were in the outpatient group. Bodyweight, body fat percentage, subcutaneous fat and visceral fat were significantly higher in the inpatient group than in the outpatient group before weight loss. Whereas the V/S ratio was almost equal between the two groups before weight loss. Bodyweight, body fat percentage, subcutaneous fat and visceral fat were found to decrease significantly during weight loss in the two groups. The V/S ratio of the outpatient group did not change after weight loss. In contrast, the V/S ratio of the inpatient group decreased significantly during weight loss. These preliminary findings suggest that a large amount of body fat and a high obesity rate are not always accompanied by a high V/S ratio in obese children. The fat pattern changes during weight loss with strict dietary therapy and therapeutic exercise. A larger sample of obese children should be studied to test this conjecture.

Sister chromatid exchange (SCE) was examined in cultured lymphocytes of nephrotic children and some other juvenile patients treated with immunosuppressive agents, azathioprine (AZA) and cyclophosphamide (CYC), and CYC in combination with a protein-bound polysaccharide (PS-K). The frequency of SCEs in children treated with CYC showed a highly significant increase as compared with the non-treated control. AZA treatment also resulted in an increased frequency of SCEs, though the mean value was lower than that obtained for CYC. An extremely high frequency of SCEs resulted from a combined treatment with AZA and CYC. After 3–4 months of cessation of CYC medication, the frequency of SCEs returned to the normal level. The SCE frequency decreased significantly when CYC was used in combination with PS-K.