bluuebunny/crossref_metadata_2025 huggingface dataset:
Treatment of Acute Lymphoblastic Leukemia in the Tokyo Children's Cancer Study Group—Preliminary Results of L84‐11 Protocol
Authors: Tsuchida Masahiro , Akatsuka Jun‐ichi , Bessho Fumio , Chihara Hiroshi , Yasuhide Hayashi M.D. , Hoshi Yasutaka , Hosoya Ryota , Furukawa Toshiharu , Ikuta Koichiro , Inana Ichiro , Ishikawa Akira , Ishimoto Koichi , Ito Koreatsu , Kaneko Masafumi , Kaneko Takashi , Kato Shun‐ichi , Komiyama Jun , Matsuyama Shusuke , Nagao Takeshi , Nakazawa Shinpei , Nishihara Koichi , Ohira Mutsuro , Okimoto Yuri , Ohkawa Yoji , Ohtsuki Hiroshi , Sato Takeyuki , Shibuya Atsushi , Shitara Toshiji , Sugita Ken‐ichi , Taguchi Nobuyuki , Torigoe Katsumi , Tsukada Masashige , Tsukimoto Ichiro , Tsunematsu Yukiko , Wada Emiko , Yamada Kaneo , Yamada Koichiro , Yamamoto Keiko , Yamamoto Masao , Yata Jun‐ichi , Nishimura Kozo , Saito Tomohiro
Published: October 2007

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 SI, S2 for a standard risk group (SRG), HI and H2 for a high risk group (HRG) and HEX for a extremely high risk group of patients. We added intermediate‐dose methotrexate as early intensification therapy in the S2 and H2 groups to reduce extramedullar 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.
Allogeneic Bone Marrow Transplantation in Childhood Leukemia
Authors: Kato Shunichi , Yabe Miharu , Yabe Hiromasa , Kubota Chidori , Hinohara Tomoyuki , Hattori Kinya , Shinohara Osamu
Published: October 2007

Allogeneic bone marrow transplantation was performed in 94 patients with hematologic malignancies or other various diseases during the period between March 1982 and November 1990 at Tokai University Hospital. Projected disease‐free survival rates of HLA genotypically identical marrow recipients were 88.9% for chronic myeloid leukemia transplanted in the first chronic phase (N = 9), 90.9% for acute leukemia in the first complete remission (N = 15), 54.5% for acute leukemia in later remissions (N = 14), 62.5% for solid tumors (N = 8) and 0% for patients transplanted in relapse (N = 7). The rate for HI A‐mismatched marrow recipients with leukemia was 27.8% (N = 16). For patients with non‐neoplastic diseases it was 100% regardless of HLA‐compatibility (N = 26). The quality of life in long‐term surviving pediatric marrow recipients has been acceptable. Common abnormalities among survivors are long‐lasting hypogonadism due to radiation and subclinical impairment of lung function in the first year post‐BMT. About two‐thirds of children experienced a transient decrease in growth velocity in the immediate posttransplant period.
Principles of Total Care for Children with Leukemia —Psychosocial Interventions —
Authors: Hosoya Ryota , Nishimura Kozo
Published: October 2007

Today about 70% of children with leukemia are cured by intensive chemotherapy. Therefore how to deal with the children and their families before, during, and after the treatment has become a major concern for caregivers. And it is a fact that there are still a few patients who relapse and are destined to die. We are now taking care of two groups of patients. One group is truly cured and one group is dying. We have been trying to establish a good total care system that fits our society. We have formed medical teams which consist of doctors, nurses, and care workers. From our experience, we think the most important principle for the caregivers is frankness. Sometimes this is very difficult. Other important points of total care, especially from psychosocial aspects, are mentioned.
Study to Establish Normal Values for Portal Vein Blood Flow in Children Using a Duplex Ultrasound System
Authors: Kagimoto Seiichi , Fujitsuka Satoshi , Kinoshita Keiji , Onoue Masahiro , Fujisawa Tomoo , Yoshioka Shigetake
Published: October 2007

Portal vein blood flow in healthy children was measured using a duplex ultrasound system consisting of a B mode linear scanner and a single Doppler transducer. Portal blood flow volume (PBFV) correlated with age, height, weight and body surface area. Increases in PBFV with age were greater in boys than girls. A correlation was noted between the diameter of the portal vein and PBFV (r=0.89). However, the maximum portal blood flow velocity (Vmax) did not correlate with age, height, weight or body surface area. Increases in PBFV in accordance with physical development was thought to depend mainly on widening of the portal vein and not on acceleration of portal blood flow velocity. The formula for calculating PBFV by multivaliate statistical analysis is as follows:

PBFV(ml/min)=30.1×age(yrs)‐1.07×height(cm)=3.31×weight(kg).

Portal vein flowmetry using a duplex ultrasound system may be useful for evaluation of the hepatic circulation in children.
A Case of Small Round Cell Tumor of the Thoracopulmonary Region with Myogenic and Neurogenic Elements
Authors: Goji Junko , Sano Kimihiko , Murakami Ryusuke , Nakamura Hajime , Ninomiya Michindo , Ito Hiroshi
Published: October 2007

We here report a unique case of a young boy with an intrathoracic tumor which consisted of neurogenic and myogenic elements. The initial pathological diagnosis was alveolar rhabdomyosarcoma. The tumor tissue from surgical resection was composed of three parts, each showing a different histological appearance, i.e. a monotonous small cell area, an alveolar area, and an area consisting of pleomorphic rhabdomyoblasts. The small round cells in the monotonous area were immunoreactive with the antibodies for Leu7, neuron‐specific enolase (NSE), neurofilament proteins (NFP), and β2 microglobulin, but not with the antibody for desmin. These cells also had dense core granules. The tumor cells in the alveolar area were immunoreactive with the antibodies for Leu7 and desmin, but not with the antibody for NFP. Pleomorphic rhabdomyoblasts were immunoreactive with the antibody for desmin, but not with the antibodies for Leu7 and NFP. These findings imply that this tumor consisted of neurogenic and myogenic elements and is considered to be a special type of rhabdomyosarcoma.
A Case of Farber Disease
Authors: Fujiwaki Takehisa , Hamanaka Sumiko , Koga Mayumi , Ishihara Tokuhiro , Nishikomori Ryuuta , Kinoshita Eiichi , Furusho Kenshi
Published: October 2007

We report a case of Farber disease (Farber lipogranulomatosis). The main features were a shrill voice, joint swelling, subcutaneous nodules and retarded psychomotor development. Cytological investigation revealed intracytoplasmic inclusion bodies characteristic of Farber disease. Lipid analysis of liver tissue indicated an accumulation of ceramide containing non‐hydroxy fatty acids. It was found that the acid ceramidase activity in the liver was reduced to 31% of the control value. In this patient there were also persistent diarrhea, cholelithiasis, transient proteinuria and increased urinary total sialic acids. These features have not been noted in previously reported cases.
A Case Report of Purulent Pericarditis with Cardiac Tamponade: Echocardiographic Findings
Authors: Abo Ken‐ichi , Agata Youtaro , Hiraishi Satoshi , Yashiro Kimio , Machii Masato , Nakae Seimei
Published: October 2007

A five year old girl with cardiac tamponade due to purulent pericarditis caused by Staphylococcus aureus was treated successfully with a combination therapy of appropriate antibiotics and surgical open drainage. Right atrial collapse was observed during early systole using two‐dimensional echocardiography. This case illustrated the usefulness of echocardiography for early detection and treatment of cardiac tamponade in pediatric patients.
Development of Sound Localization
Authors: Kaga Makiko
Published: October 2007

Perceptual sound localization is determined from differences in time and loudness of signals arriving separately at bilateral ears, with the time difference being the more important one. The author examined the development of sound lateralization using a self‐recording device developed by Sato. It measures the sensitivity of sound lateralization on the basis of time and loudness differences separately. Subjects were 59 children aged 2 to 18 years and 12 adults who were audiologically and neurologically normal. Sound stimulus was 500Hz continuous band noise. (1) The testable rate was increased in children older than the age of 4, and all children over 7 years of age could be tested. (2) Children over 4 years of age could lateralize sound on the basis of time difference. Sensitivity to time differences improved rapidly, showing a steep curve, in children from the ages of 4 to 6, and then increased slowly until adulthood. (3) Sensitivity to loudness differences increased and standard deviation decreased with age. There is a possibility that children aged 4 can discriminate loudness difference almost as well as adults.
Characteristics of Pubertal Growth in Japanese Children from the Standpoint of Skeletal Growth
Authors: Murata Mitsunori
Published: October 2007

Since the end of the 2nd World War, Japan has seen quite rapid socioeconomical development. With this development the physical size of Japanese children has increased, but the final size, especially the stature, is still shorter than that of Americans or Europeans. Bone maturation velocities were compared among Japanese and Chinese children and adolescents aged 7 to 18 (in 1986) and English TW2‐subjects, using the TW2 method. Asian children and adolescents may have a different tempo of skeletal maturation during pubertal growth from that of English children and adolescents. This, probably genetic, difference in the tempo of skeletal maturation, especially that of RUS, between Japanese and English during pubertal growth may be one of the main causes of the final difference in the stature of the two groups.
Sound Spectrographic Studies on the Relation Between Motherese and Pleasure Vocalization in Early Infancy
Authors: Shimura Yoko , Yamanoucho Itsuro
Published: October 2007

The relationship between the vocalizations of early infants and their mothers was investigated by acoustic analysis. The subjects were eight infants aged 40–70 days and their mothers. The infants were sufficiently developed to utter pleasure vocalization. The acoustic characteristics of the voices of the infants and mothers while communicating with each other analyzed in terms of pitch, duration, latency and melody types. The results were as follows: 1) Correlation was significant between the average fundamental frequencies of the infants' voices and those of their mothers. Motherese and infants' vocalizations were similar in pitch. 2) There was little individual difference in the duration of infants' vocalizations, but a considerable individual difference in the duration of motherese. No correlation was observed in the duration of the vocalizations of the infants and mothers. 3) The latencies of infant vocalization and motherese showed large differences among the infant‐mother pairs. There was a tendency for the latency of the motherese to affect the number of utterances by the infant. 4) Significant correlations in the melody types were observed in three mother‐infant pairs. These findings indicate that there are significant correlations in the vocal exchanges between infants and their mothers.
Human Immunodeficiency Virus Infection in Central Nervous System and Myeloid‐Monocytic Lineage
Authors: Ushijima Hiroshi , Abe Toshiaki
Published: October 2007

We established persistent infection with a strain of human immunodeficiency type 1, HTLV‐IIIB, in a promyelomonocytic cell line, ML‐1 (CD4 antigen nearly negative and CD4 mRNA negative), and a promonocyte cell line, THP‐1 (CD4 antigen positive). Different reactions of giant cell formation were found after cocultivation of infected and uninfected cells of ML‐1, HL‐60, THP‐1 and U‐937 cell lines with uninfected and infected MOLT4 (a T‐lymphoma cell line). Factors affecting the cellular tropism of human immunodeficiency virus were discussed.
Comparison of Severity of Viremia and Antibody Responses between Infants and Children with Measles
Authors: Suga Sadao , Yoshikawa Tetsushi , Asano Yoshizo , Nakashima Toshihiko , Yazaki Takehiko
Published: October 2007

Severity of viremia and neutralizing antibody responses were compared between nine young infants ( 10 months) with measles and 18 infants and children ( 11 months) with ordinary measles. Peripheral blood mononuclear cell (PBMC)‐associated viremia was detected between the first day of elevation of fever (day 0) and day 5 of the disease in the former group, whereas PBMC‐associated and cell‐free viremia were detected between day 0 and day 14 in the latter group. The number of infected PBMC during the first 7 days of the disease was 3.22 ± 1.07 (log10, mean ± s.d.) per 10 million PBMC in the former group, which was significantly smaller (P= 0.02) than that of the latter group (4.21 ± 1.18). The former group reached the maximum level of antibody earlier than the latter group.
Long Duration of Erythrocyte Hypoplasia after Bone Marrow Transplantation
Authors: Suzuki Nobuhiro , Kudoh Tooru , Katoh Shizue , Yohtoh Yuhkoh , Miura Jun‐ichi , Kamimura Shigeki , Sakuma Yasuhiko , Fujita Shigeru , Chiba Shunzo
Published: October 2007

Bone marrow transplantation was performed on a 15 year old girl with chronic myelogenous leukemia. The bone marrow was obtained from her younger sister, who was human leukocyte antigen haplo‐identical but major ABO incompatible. As a result, the condition of pure red cell aplasia (PRCA) persisted over a long period of time. In order to overcome major ABO incompatibility, erythrocytes were eliminated from the bone marrow graft before transplantation, and methotrexate and cyclosporine (CsA) were used to prevent graft‐versus‐host disease (GVHD). Administration of erythropoietin proved ineffective. B19 parvovirus infection could not be detected during that time. Agglutinin titers decreased to less than fourfold in parallel with the recovery of erythrocytes. Reports on similar PRCA have been limited to cases of transplantation with ABO incompatibility and cases where CsA was administered to prevent GVHD. This suggests that ABO incompatibility and CsA might be related to the development of PRCA.
Gallbladder Changes in Neonatal Hepatitis: Markedly Thickened Wall and Lack of Contractility
Authors: Nakamura Michiru , Matui Akira , Sasaki Nobuhiko , Shinohara Naohiro , Taniguchi Nobuyuki , Itoh Kouichi
Published: October 2007

The gallbladder wall changes were observed on ultrasonography during the course of a patient with neonatal hepatitis. The gallbladder was not detected at 53 days of age, but on the next day its wall was observed to be markedly thickened and without contraction following the administration of cerulein. It had a thinner wall at 57 days of age and reacted to cerulein. The wall thickness and contractility went together with the improvement of jaundice and liver function tests. Histological diagnosis was compatible with neonatal hepatitis.

Ultrasonographic detection of the gallbladder has been helpful to differentiate neonatal hepatitis from biliary atresia. It is reported to be compatible with neonatal hepatitis to detect a normal‐sized gallbladder or its contraction following cerulein administration or feeding. Since this case did not meet these criteria initially, thickened wall of the gallbladder may be an additional finding indicating neonatal hepatitis. The importance of repeated ultrasonography and clinical correlation was stressed.
Relationship between PaO2 and Lung Volume during High Frequency Oscillatory Ventilation
Authors: Suzuki Hiroshi , Papazoglou Kleoniki , Bryan A. Charles
Published: October 2007

The relationship between oxygenation and lung volume during high frequency oscillatory ventilation (HFOV) was studied. We ventilated anesthetized, tracheostomized adult rabbits that were rendered surfactant‐deficient by lung lavage. Lung volume was measured by the ‘disconnection technique’. In the first experiment, HFOV was commenced after conventional mechanical ventilation (CM V) for 1 hr. In the absence of sustained inflation (SI), oxygenation improved with time during HFOV. In the second experiment, HFOV was instituted after CMV for 4hr. In the absence of SI, all animals expired during the experimental period. In the third experiment we ventilated rabbits for 4 hr and then switched to HFOV. We applied SI first and increased mean airway pressure (MAP) by increments of 2 cmH2O every 15 min. However, there was little improvement in PaO2 despite the use of repeated SI and the increase in MAP. We conclude that oxygenation has a linear relationship to lung volume during HFOV, and that secondary lung injury due to long‐term CMV impairs the response to HFOV. Therefore, it is important to minimize the risk of such secondary injuries before instituting HFOV.
Alpha‐ and Beta‐adrenergic Receptors in Children with Essential Hypertension
Authors: Uchiyama Makoto , Hayakawa Hiroshi , Ogawa Teruyuki
Published: October 2007

Platelet α2‐ and lymphocyte β‐adrenergic receptors were examined in eight children aged 10–16 years with essential hypertension, and in 11 age‐matched normotensive children. Both α2‐ and β‐adrenergic receptor concentrations showed no significant differences between children with essential hypertension and normotensive children. According to other studies, physiological fluctuations in catecholamines determine lymphocyte β‐adrenergic receptor concentrations, although down‐regulation in platelet α2‐adrenergic receptors has not been observed. The present findings suggest that there may be no abnormalities in α2‐ and β‐adrenergic receptors in essential hypertension in childhood, although a hyperadrenergic state has been reported to be of some relevance to the pathogenesis of essential hypertension in young adolescents.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome. XV. Potential Utility of Streptococcal Pyrogenic Exotoxin Toxoid for the Prophylaxis and Treatment of MCLS
Authors: Akiyama Takehisa , Shimanuki Kaoru , Yashiro Kimio
Published: October 2007

Mice made tolerant to streptococcal pyrogenic exotoxin (SPE) by neonatal inoculation with SPE emulsified in incomplete Freund's adjuvant demonstrated early thrombocytopenia followed by thrombocytosis. This state is the perfect counterpart of patients with mucocutaneous lymph node syndrome (MCLS). We have hypothesized that by inducing tolerance to SPE, the biological activities of the toxin might play leading roles in the pathogenesis of MCLS. In the present investigations, the efficacy of SPE on the prophylaxis and treatment of diseases caused by Streptococcus pyogenes (including MCLS) were monitored using the murine model system accompanied with a platelet‐counting technique. The mice, rendered tolerant due to neonatal SPE inoculation and followed by immunization with SPE toxoid about 1 month prior to the provocative injections with SPE, demonstrated an almost complete lack of response to the provocation, keeping platelet counts within the normal range of values (except for a marginally significant thrombocytosis 7 days postprovocation). Moreover, anti‐SPE titers of the sera from the mice sacrificed on day 35, at which point the observation was terminated, were proved to be markedly elevated when compared with controls. These findings seem to suggest that immunization with the toxoid could overcome tolerance, resulting in the production of an antitoxin. In a second experiment that examined the effect of administration with rabbit antiserum raised against the toxoid, the antiserum‐treated mice demonstrated a transitory thrombocytosis on 7 days postprovocation with SPE, followed by an abrupt decrease in the number of platelets from day 10 onward. Such a finding was in complete agreement with that observed in tolerant mice administered with antiserum specific for SPE, suggesting a strong neutralizing activity against SPE of the antitoxoid serum. A third experiment evaluated the effect of F(ab')2 fragments of the rabbit antiserum to the toxoid on platelet activity. The tolerant mice passively administered i.v. with the F(ab')2 fractions demonstrated a complete lack of responsiveness, except for a small thrombocytosis on days 7 and 10.
Atelosteogenesis Type 3: The First Patient in Japan and a Survivor for more than 1 year
Authors: Kuwashima Shigeko , Nishimura Gen , Kikushima Hidemaru , Tanaka Goro , Furukawa Toshiharu , Fujioka Mutuhisa
Published: October 2007

We report the first patient of atelosteogenesis type 3 (AO3) in Japan. The patient had multiple craniofacial abnormalities at birth, including ocular hypertelorism, a flat nasal bridge, micrognathia and a cleft palate. There was rhizomelic shortness of the limbs and a club‐foot. The infant had short broad thumbs in the hands similar to those observed in the feet. There were no chromosomal abnormalities. Radiological examination demonstrated striking hypoplasia of the humerus with proximal rounding and distal tapering giving a ‘drumstick’ appearance, ‘S’‐shape configuration of the cervical spine, scoliosis and coronal cleft in the thoracolumbar vertebral bodies. The infant experienced recurrent apnea and persistent severe tracheomalacia, which necessitated tracheostomy at 5 months of age. Despite his multiple skeletal deformities and respiratory problems, this patient survived more than 1 year with motorneuronal developmental delay.
Disodium Cromoglycate in the Treatment of Bronchopulmonary Dysplasia
Authors: Yamamoto Chihiro , Kojima Takatsugu , Sasai Misa , Matsuzaki Sheuji , Kobayashi Yohnosuke
Published: October 2007

Bronchopulmonary dysplasia (BPD) has become the most common form of chronic lung disease in the neonate. Recently, we have experienced a severe case of BPD and examined the effect of disodium cromoglycate (DSCG) on BPD. The gestational age and birth weight of the patient were 27 weeks and 1,000 g, respectively. Although RDS subsided after surfactant replacement therapy, the arterial‐alveolar oxygen tension ratio (a/APO2) gradually decreased and FiO2 increased with age, respectively, and pure oxygen supplementation was eventually required after 67 days of life. The DSCG treatment was commenced at 80 days of life. After 6 days of the inhalation therapy, a/APO2 gradually increased. After 10 days of the treatment, the baby was extubated. While the baby was intubated, intratracheal lavage fluid samples were obtained. Eosinophilic cationic protein (ECP) and polymorphonuclear (PMN) elastase concentrations were determined. ECP and PMN elastase concentrations of intratracheal lavage fluids gradually decreased with the DSCG treatment. These results may indicate that DSCG has led to an improvement of pulmonary function and facilitated weaning from mechanical ventilation in an infant with BPD.
The 11q‐ Syndrome with Mosaic Partial Deletion of 11q
Authors: Ishida Yasushi , Watanabe Noriyoshi , Ishihara Yoshinori , Matsuda Hiroshi
Published: October 2007

A female child with mosaic partial deletion of 1 lq is reported. At 1 month of age she was presented with congenital glaucoma, trigonocephaly and multiple minor anomalies. She exhibited growth retardation and the typical phenotype of llq‐ syndrome. G‐banding analysis failed to show any abnormality, although subsequent high resolution banding revealed the abnormal karyotype mos 46,XX,del(11)(q23.3 q24.2)/46,XX,del(11)(q23.3 q25). This case is a second case of mosaic llq‐ syndrome and her karyotype suggests that the region of 11q23.3–11q24.2 is critical in 11q‐ syndrome. Congenital glaucoma has never been reported as a complication of llq‐ syndrome.
High Frequency Oscillation
Authors: Kawano Toshio
Published: October 2007

The current state of high frequency oscillation (HFO) in Japan is reviewed. The discussion is focused on the following: (i) the repeated application of short duration SI (sustained inflation) is effective in recruiting lung volume and increasing PaO2; (ii) HFO can prevent the formation of granulocytes related to lung injury; and (iii) HFO does not increase the risk of severe complications such as air leaks, bronchopulmonary dysplasia or intraventricular hemorrhage.
Ionized magnesium level in whole blood of healthy Japanese children
Authors: Hoshino Kenji , Ogawa Kiyoshi , Kitazawa Reiko , Nakamura Yoshihiro , Uehara Ritei
Published: October 2007

Abstract Magnesium (Mg) catalyzes or activates more than 300 enzymes in the body, and it plays a pivotal role in the metabolism of carbohydrates, fats and proteins. Until recently Mg has only been measured as a total substance concentration; however, it is the free ionized form of magnesium (iMg2 +) that is physiologically active. In 1992, a novel ion selective electrode (ISE) utilizing a neutral carrier‐based membrane for assessment of iMg2 + in whole blood, plasma or serum was designed and it was used to measure the iMg2+ level in whole blood of 160 healthy Japanese children. There was a significant positive correlation between serum total magnesium (SMg) and iMg2 +. Percent ionized magnesium (iMg2 +/SMg) was 58.3 ± 4.1 % and increased with growth (age and bodyweight). The level of iMg2 + was 1.29 ± 0.08 mg/dL (range, 1.09–1.51) {0.535 ± 0.033 mmol/L (range. 0.452–0.627)} and was constant irrespective of growth. On the other hand. SMg, serum total calcium (SCa) and ionized calcium (iCa2+) decreased with growth. Thus only iMg2 + level was constant irrespective of growth. It is suspected that the buffer action of protein binding Mg may keep the iMg2 + level constant, because iMg2 + plays an important part in the activation of many enzymes.
Spectrophotometric determination of neutrophil cytochrome b558 of chronic granulomatous disease
Authors: Kiryu Chika , Kaneda Mizuho , Shiraishi Takuo , Tsuda Masahiko , Inana Ichiro , Sakiyama Takeshi , Fujinaga Toru , Nishida Akira , Kakinuma Katsuko
Published: October 2007

Abstract Background: Chronic granulomatous disease (CGD) is an inherited disease characterized clinically by severe recurrent bacterial infections from infancy. This disease is a disorder of the formation of superoxide (O2‐) by the neutrophil NADPH oxidase system, mostly due to defects in cytochrome b558 (cyt b558), which is one ol the oxidase components. Diagnosis of CGD has been performed by the assay of the O2‐ forming activity. immunological determination of defects in the oxidase components, and or spectrophotometry of cyt b558. However, spectrophotometric analysis of the b‐type heme is difficult with small amounts of blood from infant CGD patients, as the limited amounts of neutrophils are contaminated with a relatively high ratio of hemoglobin (Hb) that interferes with the heme spectrum of cyt b558. This report presents an accurate method foi the spectrophotometric analysis of cyt b558 in a small amount of CGD neutrophils that were treated with CO gas in a safe procedure instead of the previously reported CO‐bubbling method.

Methods and Results: The difference of the reduced minus oxidized cyt b558 spectrum was measured under no interference from oxy Hb at the α and β bands and differentiated as d[ΔA]/dΛ, (Λ= wavelength) to obtain further evidence for the defects of the cyt b558 heme spectrum. The interference from CO‐insensitive met Hb was eliminated by subtracting the absorption peak at the Soret (γ) band of the contaminating met Hb, which was estimated from the CO‐treated and untreated spectra of the same, hemolyzed sample.

Conclusions: This spectrophotometric method is feasible for the determination of abnormality and heme content of cyt b558 with a small amount of CGD neutrophils in10–20 mL of blood even in the presence ol contaminating Hb.
Coronary risk factors in acute Kawasaki disease: Correlation of serum immunoglobulin levels with coronary complications
Authors: Sawaji Yoichi , Haneda Noriyuki , Yamaguchi Seiji , Kajino Yasuhisa , Kishida Kenji , Seto Shiro , Konishi Nakao , Waki Kenji , Baba Kiyoshi , Arisawa Kokichi , Kamiya Tetsuro , Mori Chuzo
Published: October 2007

Abstract Background To determine the usefulness of the IgG z‐score (age and sex‐standardized serum IgG level) before intravenous gamma globulin therapy (1VGG) in predicting the occurrence or severity of coronary complications in Kawasaki disease (KD).

Methods A case‐control study of clinical and laboratory findings with 88 children in the early stage of acute KD who received IVGG (100 or 200 mg/kg for2–5 days) therapy. Of these, 20 cases had persistent coronary arterial lesions (small aneurysm, moderate aneurysm or large aneurysm persisting more than 1 month). The controls comprised 68 children with no coronary aneurysms or transient small aneurysm only observed within 1 month after the onset of KD. The association between serum levels of immunoglobulin G (IgG), IgM, IgA as well as other coronary risk factors previously reported and the occurrence of the coronary arterial lesions was evaluated using logistic regression analysis.

Results: After adjustment for age, gender, total IVGG dose before the 9th illness day and other traditional coronary risk factors, the odds ratio for the persistent coronary aneurysm associated with lower serum IgG r‐score (<‐0.7485 v.v & ‐0.7485). was 30.3 (95% confidence interval, 3.8–243.2). Furthermore, the serum IgG z‐score was inversely correlated with the severity of the coronary arterial lesion.

Conclusions: The IgG z‐score before IVGG therapy in the early stage of KD provides useful information on the risk factors for persistent coronary aneurysm and is a novel, additional indicator for therapy to prevent the coronary complications in acute KD.
Successful pregnancy after coronary artery bypass grafting for Kawasaki disease
Authors: Hayakawa Hideji , Katoh Toshiyuki
Published: October 2007

Abstract A 13‐year‐old girl with a history of Kawasaki disease underwent coronary artery bypass grafting because of angina pectoris due to a giant coronary artery aneurysm on the left main trunk artery. Nine years after the operation, the patient had an uneventful pregnancy followed by a normal vaginal delivery. This is the first case of a successful pregnancy after coronary artery bypass grafting for Kawasaki coronary artery disease.
Role of excitatory aminoacids in neonatal hypoglycemia
Authors: Aral Yusuf Ziya , GÜCÜYener KİVİLcİM , Atalay YİLdİZ , HasanoǦLu Alev , TÜRkyilmaz Canan , Sayal Ahmet , BiberoǦLu GÜRsel
Published: October 2007

Abstract
Background:
In many neurological disorders, injury to neurons may be due in part to overstimulation of the receptors for the excitatory amino acids glutamate and aspartate. The same excitotoxic mechanism and high aspartate levels in experimental studies led to this study of the concentrations of glutamate and aspartate and zinc, copper, and magnesium levels in the cerebrospinal fluid (CSF) of hypoglycemic newborns.

Methods:
Aspartate and glutamate were determined by high‐performance liquid chromatography, and magnesium, zinc and copper by atomic absorption spectrophotometer.

Results:
The CSF levels of aspartate (3.98 ± 1.77 μmol/L) and glutamate (1.7 ± 1.05 μmol/L) in 20 hypoglycemic newborns were significantly higher when compared with the values of aspartate (2.19 ± 0.6 μmol/L) and glutamate (0.77 ± 0.34 μmol/L) of 10 control newborns. In the hypoglycemic patients, the concentration of zinc (0.57 ±0.13 μg/mL), but not copper (0.39 ± 0.40 μg/mL) was significantly lower when compared with the control values. There was no difference in the magnesium levels between the two groups.

Conclusions:
The higher levels of excitatory amino acids found in the CSF of hypoglycemic infants than in controls were consistent with previous animal studies, which may indicate the role of excitatory amino acids in the late biochemical effects of hypoglycemia in newborn brain metabolism.
Bacterial croup caused by Pasteurella haemolytica
Authors: Watanabe Toru , Sato Masahisa , Abe Tokinari , Oda Yoshohiko
Published: October 2007

Abstract We report a case of bacterial croup caused by Pasteurella haemolytica. A nine‐month‐old girl was admitted to our hosptial because of abrupt onset dyspnea and unconsciousness. From clinical and laboratory findings, she was diagnosed as having bacterial croup. Pasteurella haemolytica was recovered from her tracheal secretion on admission. To our knowledge, this is the first report of bacterial croup caused by P. haemolytica.
Celiac disease in Turkish short‐statured children and the value of antigliadin antibody in diagnosis
Authors: AltuntaŞ Buket , Kansu Aydan , Ensari Arzu , GİRgİN Nurten
Published: October 2007

Abstract
Background.
It is generally accepted that celiac disease (CD) must always be taken into consideration when dealing with children manifesting growth failure. It is, therefore, important to have laboratory tests capable of detecting patients who should undergo intestinal biopsy. In this study, we have prospectively evaluated clinical characteristics, gliadin antibody measurements and duodenal biopsies in 47 children with short stature and without gastrointestinal symptoms, in order to determine the incidence of CD and the diagnostic value of immunoglobulin (Ig)A and IgG antigliadin antibodies (AGA) for CD.

Methods:
Anthropometric parameters and IgA‐ and IgG AGA were evaluated in 47 children with short stature. Antigliadin antibodies were measured by enzyme‐linked immunosorbent assay (Euroimmun kit). Endoscopic intestinal biopsies were taken from all children.

Results:
On the basis of intestinal biopsy, 26 (55.3%) patients were found to be probable CD. Sensitivity, specificity, positive predictive (PPV) and negative predictive value (NPV) for IgA AGA was found to be 23, 90, 75 and 48%, respectively. Sensitivity, specificity and PPV for IgG AGA was 100, 0 and 55%, respectively. The NPV for IgG AGA was not determined.

Conclusions:
The results of our study demonstrated that because of their incomplete sensitivity, specificity, PPV and NPV, intestinal biopsy can not be replaced by these tests.
Pseudosarcomatous myofibroblastic tumor of the urinary bladder with massive intraperitoneal hemorrhage in a child
Authors: Okada Kaname , Fujii Yoshiyuki , Uema Kenzo , Yoshimoto Tsutomu , Nakatsu. Tadanori , Yoshida Tetsuya , Hasegawa Tadashi
Published: October 2007

Abstract A 3‐year‐old boy presented with persistent abdominal pain, hematuria and facial pallor with progressive anemia. A computed tomography scan showed a massive intraperitoneal hemorrhage and tumor mass in the bladder. Histological examinations of the resected tumor revealed findings of pseudosarcomatous myofibroblastic tumor (PMT). The clinico‐pathological features of this case warn us that PMT can cause acute abdomen syndrome with massive intraperitoneal hemorrhage.
Pediatric liver transplantation
Authors: Dhawan Anil , Muiesan Paolo
Published: October 2007

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, Iymphoproliferative disease and viral infections, continue to be a matter of great concern. Most children with good graft function enjoy near normal quality of life.
Prognostic value of proliferating cell nuclear antigen immunostaining in pediatric rhabdomyosarcomas
Authors: TokuÇ GÜLnur , DoǦAn ÖNer , Ayan İNcİ , Kebudİ RejİN , DoǦAn Sema , DemİRyont MİSten , ÇAmlica Hakan
Published: October 2007

Background
: 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.

Methods
: Formalin fixed, paraffin embedded tissue specimens of 29 primary pediatric rhabdomyosarcomas were immunostained by using an anti‐PCNA monoclonal antibody (DAKO PCNA PC 10). The relationship between the PCNA index and prognosis, clinicopathological features and survival were assessed retrospectively.

Results
: 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).

Conclusion
: The PCNA labeling index can be a useful prognostic factor and a good indicator of recurrence and/or survival in patients with rhabdomyosarcoma.
Infantile hemangioendothelioma of the thymus with massive pleural effusion and Kasabach‐Merritt syndrome: Histopathological, flow cytometrical analysis of the tumor
Authors: Hiraiwa Hisayuki , Hamazaki Minoru , Tsuruta Satoru , Hattori Hiroyoshi , Mimaya Jun‐Ichi , Hasegawa Shirou , Kohno Sumio , Aoki Katsuhiko
Published: October 2007

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, lmmunohistochemically, 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.
The Industrial Organization of Financial Markets in Thailand
Authors: Mori Nobuhiro , Tsutsui Yoshiro
Published: October 2007

The purpose of this paper is to investigate the industrial organizational features of the financial markets in Thailand which may play an important role in promoting the recent high economic growth. Specifically, we examine whether or not the market‐structure‐performance hypothesis is valid by estimating profit and cost functions. The main results are as follows:

1) market concentration in Thailand is higher than in Japan;

2) expense rate of Thai commercial banks is considerably higher than Japanese banks;

3) economies of scale are not found;

4) the more concentrated the market is, the larger are the profits and costs of the commercial banks.
Measuring Human Development
Authors: Smith Peter
Published: October 2007

The uneven distribution of resources, income and wealth in the world makes it desirable to devise a method for evaluating the relative levels of human development in different countries, but a satisfactory approach needs to go beyond calibration of output per head. This paper explores key issues concerning the measurement of human development and examines the UNDP's modified Human Development Index, assessing its potential usefulness and limitations in enabling comparisons across countries. The extent to which the approach can provide the basis for a profile of an individual country is also analysed, using Singapore as a case study.
Enterprise Reform and Fiscal Reform in China: Overlapping Webs of Interests
Authors: Takahara Akio
Published: October 2007

This paper investigates the interrelationship between enterprise reform and fiscal reform in China, and illuminates the web of interests which constrains the deepening of system reforms. By analysing the policy processes of the formulation and implementation of ‘management contracting’ in 1987 and ‘fiscal contracting’ in the following year, a division is revealed between the financialists and the productionists among the reformers in the central government. While the financialists aim at full marketisation, the productionists, in alliance with the localities, regard that the majority of state enterprises still need protection and guidance of the administrative agencies.
Determinants of R and D Activity in Japan
Authors: Cortes Bienvenido
Published: October 2007

Numerous studies by Schumpeter (1950), Comanor (1965, 1967), Mansfield (1988), and Scherer (1992), among others, have identified and analysed various causes and motivations for firms’innovative activity including firm size, profitability, market concentration, ‘returns’to R and D investment, and interfirm rivalry.1 Recent international trade studies indicate that comparative advantage results partly from investment in R and D.

According to Takenaka (1991), R and D activity in Japan is primarily led by the private sector, it is becoming more capital‐intensive, and has a higher marginal profitability. He also argues that despite the strong relationship between R‐and‐D‐intensive industries and technology imports (which fuels the criticism that Japan ‘freeloads’on the R and D activity of other countries), basic research as a percentage of R and D spending is higher in Japan than in the US. Mansfield states, however, that Japanese basic research has not been productive and that much of Japan's success is due to its ‘applied R and D’. Another important difference, Mansfield points out, is that R and D spending is more positively related to firm size in Japan than in the US.2

This paper analyses the determinants of R and D spending in Japan. In particular, it looks at the relationships between R and D activity and factors such as firm size, profitability and the degree of competition or rivalry in the market. The study uses cross‐sectional data for a number of major industries in Japan.
Tax Elasticities and the Impact of Discretionary Tax Measures on Government Revenue in Papua New Guinea, 1975–92
Authors: Kusi Newman Kwadwo , Asafu‐Adjaye John
Published: October 2007

This paper evaluates tax elasticities and the impact of discretionary tax measures on government revenue in Papua New Guinea (PNG) using a dynamic macroeconometric model of taxation which captures the interaction between GDP, individual tax systems and individual tax revenues and bases. Our findings show that economic growth and discretionary tax changes have both been effective in mobilizing additional tax revenue. However, the responsiveness of the individual tax system reflected base‐to‐GDP elasticity except personal income tax whose elasticity reflected increased tax rates.
Cointegration Tests of Purchasing Power Parity: the Case of the Thai Baht*
Authors: Hataiseree Rungsun
Published: October 2007

This paper will examine the validity of the purchasing power parity (PPP) hypothesis for the Thai baht vis‐à‐vis the currencies of Thailand's key trading partners under the new exchange rate regime using the cointegration technique. The major conclusions obtained from this empirical analysis may be broadly summarized as follows. First, the empirical evidence, based on the DF and ADF statistics, seems to suggest that the nominal exchange rates and relative prices are well characterized as non‐stationary I(1) processes. Second, the cointegration analysis provides no evidence in support of a long‐run equilibrium relationship between bilateral nominal exchange rates for the Thai baht vis‐à‐vis the currencies of Thailand's major trading partners and the corresponding relative price ratios. This implies rejection of PPP for these countries. If this is the case, considerable care should be taken in assessing the long‐run implications for the real exchange rate, and thus competitiveness against Thailand's key trading partners, of shocks to the nominal exchange rate.
Labour Shortage in Hong Kong: Causes, Consequences and Policies*
Authors: Lam Kit‐Chun , Liu Pak‐Wai
Published: October 2007

This paper addresses the issue of labour shortage in Hong Kong. Labour shortage is a secular phenomenon caused mainly by a stagnation in the growth of the labour force. The major long‐term factors that explain this stagnation are a decline in fertility, a fall in the labour participation rate, a reduced intake of immigrants and a rising outflow of emigrants. Labour imposes a capacity constraint on the growth of the economy. This paper considers policies which may alleviate the supply constraint on the economy, including reducing emigration, importing labour and increasing immigration intake. These policies, which would lead to a faster expansion of the labour force, are evaluated and compared.
The Market Shares of Small and Medium Scale Enterprises in Taiwan Manufacturing*
Authors: Hu Ming‐Wen , Schive Chi
Published: October 2007

Small and medium‐sized enterprises (SEM s) have played an important role in many countries’industrial development. Among these countries, Taiwan is well known for the outstanding performance of its SME s. Limited knowledge about the causes of such performance has been revealed gradually. The main purpose of this study is to explore the determinants of SME s’market shares in Taiwan's manufacturing sector. According to economics literature, economies of scale determine the market share of SME s. The usual explanation given is that economies of scale create barriers against SME s entering the market and competing efficiently with larger firms. It is also said that the strong entrepreneurial drive to have a self‐owned business is a crucial explanation for the creation of new businesses, usually as offshoots from a small one. Finally, such factors such as an even‐handed policy scheme, the availability of technology in relation to R&D, transaction costs, and so forth, are also important for creating a favourable environment for SME s.

Applying cross‐industry pooling data from 1976 to 1986, the market shares of SME s in Taiwan's manufacturing sector were found to be affected by the following factors: (1) SME s’performance related to labour productivity and their willingness to set up new enterprises; (2) institutional factors including low entry barriers, suitable technology, the adoption of a subcontract system, and prosperous export markets. The empirical findings are compared with those from studies of the US and Germany.