Acta paediatrica Japonica; Overseas edition (Acta Paediatr Jpn )

Publisher: Nihon Shōnika Gakkai


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    Acta paediatrica Japonica. Overseas edition
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Publications in this journal

  • [Show abstract] [Hide abstract]
    ABSTRACT: Juvenile chronic arthritis (JCA) is the commonest autoimmune rheumatic disease in childhood and presents different clinical subtypes. Juvenile chronic arthritis is considered to be of a polygenic nature and its genetic background is still under investigation. The clinical profile of JCA in the Greek population has not been studied completely. This study retrospectively analyzed the clinical and immunological features of JCA in Greek children presented between 1989 and 1994. Human leukocyte antigen (HLA)-positive or -negative associations in the different clinical subtypes were also detected. The findings of this study were correlated with those reported from other populations. Antinuclear antibodies (ANA) anti-ds DNA and anti-extractable nuclear antigen antibodies were estimated by immunofluorescent and ELISA assays. Human leukocyte antigen typing was performed by microlymphocytotoxicity, using immunobeads. The peak ages of JCA onset were between 2 and 5 years and also between 9 and 12 years. There was a high female predominance in pauciarticular and polyarticular groups. The most common disease was pauciarticular (58.7%) followed by systemic (25%) arthritis. The incidence of eye involvement was 12.5% and presented only in the pauciarticular group. Overall, ANA positivity was 53.7%, increasing to 90% in pauciarticular cases associated with chronic uveitis. In the early onset (EOPA) pauciarticular subtype, positive-HLA associations with alleles DR11 and DR8 were shown. In the late onset pauciarticular (LOPA) group only B27 allele was increased. The results of this retrospective study did not reveal major differences between JCA in Greek children compared with other Caucasian series.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):558-63.
  • Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):638-40.
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    ABSTRACT: The patient is a 12-year-old boy with acute mixed lineage leukemia (AMLL) and with a rare karyotype of trisomy 6. He was referred to our hospital with gingival swelling, bleeding at the conjunctiva and huge hepatosplenomegaly. Complete blood count revealed leukocytosis with 79% blasts, anemia and thrombocytopenia. Bone marrow examination revealed 82.5% blasts which were morphologically judged as M1 according to the French-American-British classification. Immunophenotyping of leukemic cells showed the presence of CD2, CD7, CD19 and CD13 antigens, suggesting the diagnosis of AMLL. Cytogenetic analysis revealed a single abnormal karyotype of 47,XY,+6,add(15)(q22) which was successfully detected by fluorescence in situ hybridization (FISH) with the probe mapping at the alpha-satellite region of chromosome 6. Although the patient was treated with several chemotherapy regimens, he could not achieve complete remission and he died of progressive disease 11 months after admission. Fluorescence in situ hybridization analysis was very informative in assessing the residual leukemic cells in interphase during his clinical course.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):616-20.
  • Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):624-5.
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    ABSTRACT: We report the first case of the syndrome of periodic adrenocorticotropin (ACTH) and vasopressin (ADH) discharge associated with focal glomerulosclerosis. Approximately 30 cases of this syndrome have so far been reported in Japan, but no cases associated with renal dysfunction have yet been reported. The patient, a 10-year-old Japanese boy, was referred to our hospital because of recurrent attacks of vomiting. He was diagnosed as having this syndrome from clinical and laboratory findings. While various drugs were tried to manage his vomiting attacks, only valproic acid appeared to be effective in reducing the frequency of the attacks. Chronic nephritis was manifested when the patient was 12 years old, which required treatment with continuous ambulatory peritoneal dialysis. Valproic acid was proved to be effective in reducing the number of attacks over 4 months.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):600-3.
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    ABSTRACT: To progress the clinical treatment of neonates, especially in the management of respiration, we have to be able to measure their pulmonary function appropriately. Various methods have been developed, but little is known about the pulmonary function of very low birthweight infants (VLBWI) because of the difficulty in taking their measurements with existing equipment. We have developed a very low dead space pneumotachograph to measure lung function in VLBWI. We used our pneumotachograph on 30 infants each weighing less than 1500 g at birth. The infants were intubated with endotracheal tubes of 2.5 or 2.0 mm diameter to measure tidal volume and minute ventilation in the prone and supine position. The tidal volume in the supine position was 6.99 +/- 0.42 mL/kg and 7.58 +/- 0.38 mL/kg in the prone position (mean +/- SE). The tidal volume was significantly larger in the prone than the supine position (P < 0.05). However, no significant difference was observed in minute ventilation and respiratory rates. The tidal volume significantly increased in the prone position in VBLWI, confirming the previous observation of larger healthy infants is also applicable to the very low birthweight infants.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):555-7.
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    ABSTRACT: Simple and efficient method for the selection of transduced cells would greatly facilitate the clinical utilization of retrovirus vectors. We developed a therapeutic bicistronic retrovirus vector for Gaucher disease, MFG-GC-GFP, which contains the human glucocerebrosidase (GC) gene and the green fluorescent protein (GFP) gene of the jellyfish Aequorea victoria as a vital selection marker, and investigated its applicability as gene therapy for Gaucher disease. A packaging cell line, GP + envAM12, was transfected with MFG-GC-GFP and, thus, produced a high titer recombinant virus (1.0 x 10(6) c.f.u./mL) in the culture supernatant. The expression level of GFP was correlated with the virus production in cells. The recombinant virus infected skin fibroblasts from a Gaucher patient and a sorted fraction of the cells expressing GFP by flow cytometry exhibited almost a six-fold higher activity of GC than normal fibroblasts. These data indicate that MFG-GC-GFP enables the one-step purification of a transduced fraction of target cells and is, therefore, considered to be a useful therapeutic vector for the experimental gene therapy of Gaucher disease.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):586-92.
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    ABSTRACT: Infantile hemangioendothelioma of the thymus is a rare disease. We describe a patient who developed a large anterior mediastinal mass, severe thrombocytopenia and massive pleural effusion at 1 month of age. Glucocorticosteroid and irradiation therapy had no effect on either the tumor size or clinical symptoms and the tumor was resected subtotally. Three months after the subtotal resection, the remaining tumor had almost disappeared and the symptoms had resolved. The patient has now been well for 1 year after surgery without evidence of recurrence. The tumor tissue was characterized by prominent vascular endothelial proliferation intermixed with a normal thymic structure, producing a picture consistent with that of an infantile hemangioendothelioma in the thymus. Immunohistochemically, the tumor cells showed positive staining for vimentin, factor VIII and CD34. The DNA stemline and proliferative activity were examined by flow cytometry, which revealed a diploid stemline with a low growth fraction. DNA content and cell cycle analyses of the tumor tissue may be useful for predicting the biological behavior of the tumor.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):604-7.
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    ABSTRACT: The levels of proliferating cell nuclear antigen (PCNA) are almost negligible in long-term quiescent cells and increase dramatically during the cell cycle. Recently, the monoclonal antibodies to PCNA have been used to demonstrate the proliferative component of paraffin-embedded tumor tissues. It has been shown to be available as a simple histological marker of proliferative activity and the PCNA labeling index has been correlated with the prognosis of several malignant neoplasms. Formalin fixed, paraffin embedded tissue specimens of 29 primary pediatric rhabdomyosarcomas were immunostained by using an anti-PCNA monoclonal antibody (DAKO PCNA PC10). The relationship between the PCNA index and prognosis, clinicopathological features and survival were assessed retrospectively. The mean PCNA index for the whole series was 54%. There was no correlation between PCNA index and any of the clinicopathological characteristics. However, patients having tumors with a high (> 54%) PCNA index demonstrated significantly lower survival rates than tumors with a low (< 54%) PCNA index (P = 0.01). Moreover, there were significantly more patients with relapse or progressive disease in the high PCNA index group (P = 0.005). The PCNA labeling index can be a useful prognostic factor and a good indicator of recurrence and/or survival in patients with rhabdomyosarcoma.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):573-9.
  • Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):644-6.
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    ABSTRACT: To determine the usefulness of a few types of photic stimulation in patients with photo-convulsive response. A clinical electroencephalographic study was conducted in nine children who developed convulsions and other symptoms while they watched the TV animation and were subsequently treated at the hospital. Photic stimulation was applied with their eyes open or closed. Another photic stimulation was applied with the video tape of the animation. A photoparoxysmal response (PPR) was never seen in the electroencephalographs (EEG) in any of these patients when photic stimulation was applied with their eyes closed, while PPR was seen in three of seven patients when photic stimulation was applied with their eyes open. Two patients (siblings) showed no specific abnormality when photic stimulation was applied with their eyes either open or closed. However, myoclonus and PPR appeared when the EEG was conducted while they watched the video tape of the animation under supervised conditions. In a society where people are exposed to abundant TV games, TV animation or videos, self-restraint or regulations of frequent use of flickering scenes is thought to be necessary. However, it is also thought to be necessary to conduct EEG by applying photic stimulation, not only with the eyes closed, but also with the eyes opened or by applying flickering red light stimulation, which is emitted from a strong light source, or a combination of two colors, such as red and blue or red and green, in patients with suspected photosensitivity.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):544-9.
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    ABSTRACT: This study examines JL Conel's data on neuron numbers in 35 human cortical areas for eight age points from 0 (birth) to 72 months, to analyze cortical columns, the presumed functional units of the cortex. For each cortical area at each age point, cortical surface divided by the square root of the area's neuron number gives cross-sectional areas with radii ranging from 180 microns at birth to 250 microns at 72 months. For the prefrontal cortex at birth and 48 months, these radii are approximately 2.10 and 1.19 times the longest radial basal dendrites, suggesting similar dimensions between these two measures of column radius. The logarithm of neuron number per cortical area and age point was examined in relation to the Weber-Fechner law governing the relationship between stimulus intensity and perception. A mechanism for this law consistent with the cortical model of Douglas et al. illustrates the importance of local circuit neurons. The cross-sectional areas of hexagonal columns for prefrontal cortex, using as radius, the longest radial extent of layer 5 pyramidal neuron basal dendrites, ranging from 0.013 mm2 at birth to 0.064 mm2 at 48 months, suggests that functional cortical columns increase cross-sectional area during development. These cross-sectional areas are 55-100-fold larger at birth, and 229-277-fold larger at 48 months, than those computed from somal width in prefrontal, layer 5 pyramidal neurons. Comparison of radial extent of pyramidal basal dendrites to their soma-to-soma distances shows that layer 3 pyramidal basal dendrites reach 1.5 and 4.0 other pyramidal neurons at 15 and 60 months, respectively, while layer 5, extra-large pyramidal basal dendrites reach 1.14 and 1.72 other such neurons at birth and 48 months, respectively. If such a relationship holds for other cortical areas, then the Conel data can be used to estimate basal dendrite extent, for which there currently is a paucity of data.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):530-43.
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    ABSTRACT: The function of CD48, one of the pan leukocyte cell surface antigens, is not yet well understood. CD48 was recently shown to enhance the CD40-mediated activating signal to B lymphocytes. As CD48 is one of the activation antigens of monocytes, neutrophils and lymphocytes, a change of its expression on the cells could be expected in infectious diseases. Leukocytes from 27 healthy controls and 97 patients with various infectious diseases were stained with anti CD48 antibody and analyzed by flow cytometry. On monocytes and neutrophils, the CD48 expression was increased in all of the patients with varicella, measles, rubella, infectious mononucleosis, streptococcus tonsillitis, sepsis and appendicitis. On lymphocytes, a significant increase of CD48 was also detected in the patients with the same diseases, except those with sepsis or appendicitis. The normalization of increased CD48 expression was confirmed on monocytes at the convalescent phase. These data suggest that CD48 expression on leukocytes reflects the disease activity of infectious diseases, especially of viral infections.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):580-5.
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    ABSTRACT: We report a 3-month-old girl with Costello syndrome complicating fatal hypertrophic obstructive cardiomyopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costello syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):608-11.
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    ABSTRACT: We carried out a clinical and electroencephalographic (EEG) study to reveal the pathophysiology of acute symptoms elicited by a TV animation program. Clinical data and EEG were obtained from 20 patients ranging in age from 6 to 30 years. A pattern presentation and an intermittent photic stimulation were performed. Of 20 patients, 13 had a convulsion and seven had other symptoms, mainly nausea and/or vomiting. A photoparoxysmal response (PPR) was confirmed in 12 of 13 patients (92.3%) who had a convulsion. Only one of seven patients (14.3%) without a convulsion showed a PPR. The incidence of PPR was significantly higher in patients with convulsion than those without convulsion. There were no significant differences in the family history of convulsion, gender, parameters associated with TV watching and basic EEG between the two groups. All patients with PPR had a convulsion or consciousness disturbance. Acute symptoms, such as convulsion and impairment of consciousness, are supposed to be based on photosensitivity.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):626-30.
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    ABSTRACT: Chronic pancreatitis is a rare disease in children and is usually secondary to underlying diseases such as hereditary pancreatitis, cystic fibrosis, hyperlipidemia, prolonged malnutrition, gallstones or anomalies of the biliary-pancreatic duct system. Hereditary pancreatitis is a common cause of chronic pancreatitis in children but is often unrecognized until months or years later. We report here a family with hereditary pancreatitis in which four members are affected.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):612-5.
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    ABSTRACT: Experience with pediatric use of intravenous amiodarone is limited. In this study, our experiences with intravenous amiodarone in children with acute life-threatening or chronic tachyarrhythmias are reviewed. Twelve patients, with a mean age of 3.4 +/- 3.1 years, range 9 months-10 years (two with incessant ventricular tachycardia, one with ectopic atrial tachycardia, two with atrioventricular re-entrant tachycardia (three episodes), four with postoperative or congenital junctional ectopic tachycardia, two with bradycardia-tachycardia syndrome, one with atrial tachycardia) were treated with intravenous amiodarone during 13 tachycardia episodes. Left ventricular systolic functions were depressed in six patients. In 11 patients, a median of two drugs (range one-four), including adenosine infusion and in five cases direct current cardioversion were tried without success prior to intravenous amiodarone. The loading dose of amiodarone was 5 mg/kg in all episodes, infused over 1 h. Maintenance infusion was required in 12 episodes. In 10 episodes (77%), amiodarone was considered effective, in one (7.6%) partially effective (junctional ectopic tachycardia) and in two (15.4%) ineffective (sick sinus syndrome, atrial tachycardia). Therapeutic effect was obtained in a median period of 30 h (range 1-103 h). The mean effective maintenance dose was 10 +/- 4.7 micrograms/kg per min (range 5-15 micrograms/kg per min). In one patient, mild hypotension, and in three patients cellulitis occurred, but none of them necessitated termination of treatment. Intravenous amiodarone is found to be an effective and safe antiarrhythmic agent for children with acute life-threatening and chronic tachyarrhythmias and depressed left ventricular systolic functions.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):567-72.
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    ABSTRACT: An 8-year-old girl with hypergammaglobulinemia showed an abnormal 67gallium accumulation in the orbits and parotid glands. Although she did not have any subjective siccant complaints, reported typical histopathological and sialographic changes suggesting Sjögren's syndrome (SjS) were observed in the salivary glands. Gallium scintigram might be a valuable and non-invasive diagnostic tool in the diagnosis of children with SjS without sicca symptoms.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):621-3.
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    ABSTRACT: Liver transplantation in children has become a treatment of choice for end-stage liver disease. Both graft and patient survival has significantly improved over the last two decades. The major factor contributing towards improved survival is availability of cyclosporin. Surgical innovations like living related donation has not only increased the donor pool, but has also helped patients in countries where cadaveric donation is not available. Auxiliary liver transplantation has made a significant change to the management of children with liver-based metabolic disorders and, in selected cases of acute liver failure, immunosuppression can be safely withdrawn once the native liver recovers. However, the complications associated with immunosuppression, such as nephrotoxicity, lymphoproliferative disease and viral infections, continue to be a matter of great concern. Most children with good graft function enjoy near normal quality of life.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):525-9.
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    ABSTRACT: The wheezing infant is a common but difficult patient to approach diagnostically. The prevalence of immunoglobulin (Ig) G subclass deficiency in wheezing infants is still controversial. We studied the serum concentration of IgG subclasses in 38 wheezing infants (aged 6-24 months) who had not received systemic steroids before investigation and in 30 healthy age matched controls (aged 6-24 months). The prevalence of one or more IgG subclass deficiencies was 31.6% in wheezing infants and 26.7% in controls. There was no significant difference in prevalence of IgG subclass deficiency between patients and controls (P > 0.05). The mean concentration of IgG subclasses in patients were compared with controls. There was no significant difference in mean serum concentration of IgG1, G2 and G3 subclasses. However, there was a trend towards higher concentrations of IgG4 in wheezing infants and this difference for IgG4 was significant (P < 0.01). Immunoglobulin G subclass deficiency was found in 25 and 36.4% of wheezing infants who had experienced from two to four and five or more wheezing episodes in 2 years, respectively (P > 0.05). Our findings suggest that wheezing in infancy is not associated with IgG subclass deficiency, and in wheezing infants low IgG subclasses levels do not increase the frequency of wheezing. However, there is a relationship between recurrent wheezing and serum IgG4 subclass concentration.
    Acta paediatrica Japonica; Overseas edition 01/1999; 40(6):564-6.