bluuebunny/crossref_metadata_2025 huggingface dataset:
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome XL Immunoelectron Microscopic Observation of Protoplast‐like “Spherical Bodies” Detected in Peripheral Blood of MCLS Patients
Authors: Akiyama Takehisa , Tamauchi Hidekazu , Nakazato Kazumi , Yashiro Kimio , Itoh Yoshiko
Published: October 2007

Protoplast‐like “spherical bodies” averaging 0.5‐1.5mU m in diameter and devoid of cell walls were first detected by Ueno et al, in the buffy coat of heparinized venous blood from patients with mucocutaneous lymph node syndrome (MCLS). But the nature of the “bodies” has yet to be clarified because of the absence of convincing evidence pointing to their antigenic characteristics. The present investigations were designed solely to provide a serological identification of the “bodies” by the use of immunoelectron microscopy, with the following results. First, “spherical bodies” bearing a striking resemblance to those observed by the above‐mentioned authors were detected in biopsy specimens from challenge sites in mice infected with Streptococcus pyogenes as well as in the buffy coat of peripheral blood from MCLS patients. Second, the “bodies” detected were stained distinctly in both cases by an immunohistochemical technique using, as the primary antibody, a rabbit antiserum raised toward S. pyogenes‐derhed protoplasts, which was then absorbed with protoplasts from Staphylococcus aureus and Escherichia coti. Third, the absorbed sera were proved to be not faultless, because complete specificity toward protoplasts from S. pyogenes was not attained due to the presence of a large amount of cross‐reactive antigens between protoplasts from the immunizing and absorbing strains of bacteria. The implications of these findings are discussed, particularly in relation to the evaluation of the present serological test for the “spherical bodies.”
C3 Deposition in Serially Biopsied Children with IgA Nephropathy
Authors: Hara Masanori , Yoshida Reiko , Inaba Susumu , Higuchi Akira , Suzuki Yoshifumi , Okada Toshio , Tanizawa Takakuni
Published: October 2007

Twenty‐five children with IgA nephropathy were studied by serial renal biopsy to investigate C3 deposition. The children were classified into three groups according to the immunofluorescent (IF) course of C3 deposition: group I (N = 9): unchanged or slightly decreased; group II (N = 4): changed to segmental deposition; and group III (N = 12): changed to negative deposition. Histological changes were scored semiquantitatively as an activity index (cellular proliferation, necrosis, interstitial cell infiltration and cellular crescents) and a chronicity index (mesangial sclerosis, segmental and global glomerular sclerosis, adhesion, fibrous crescents and tubulo‐interstitial change). The IF findings were scored semiquantitatively and laboratory data were also studied. The following results were obtained: 1. Normal urinalysis was often observed in group III; 2. The IF scores of IgA and IgG were decreased at the second biopsy in all groups, most notably in group III; 3. The activity indices at second biopsies were decreased in all groups, most notably in groups II and III, consistently with our previous study which showed that C3 deposition increases in accordance with histological activity; 4. The chronicity index was unchanged in all groups and C3 deposition did not reflect histological chronicity. Thus, this study indicates that C3 deposition occurs as the disease progresses and reflects histological activity.
Infant Botulism with Down Syndrome
Authors: Toyoguchi Sho , Tsugu Hitoshi , Nariai Akiyoshi , Kaburagi Yoichi , Asahina Yoshiko , Ambo Kazutoshi , Katou Kazuo
Published: October 2007

A 4‐month‐old boy with Down syndrome and infant botulism is reported. He was admitted to our hospital with stridor and developed respiratory failure and generalized paralysis. Laboratory examination revealed botulinal toxin type A in his blood and feces. Clostridium botulinum organisms were found in the feces and in the honey which he had been fed. Anti‐botulinal toxin was administered on the 10th day of illness. Artifical ventilation was given for 87 days and he survived. This is the 11th known case of infant botulism in Japan. It is possible that other cases remain undiagnosed.
Bias in Evaluating CNS Complications Following Pertussis Immunization
Authors: Ehrengut Wolfgang
Published: October 2007

Bias in the evaluation of CNS complications following pertussis immunization are the following: 1) Notifications of postimmunization adverse events, 2) Publications by vaccine producers on the frequency of adverse reactions, 3) Comparison of permanent brain damage after DPT and DT immunization, 4) Pro‐immunization, 5) Immunization associated viral encephalitis, 6) Accuracy of statistics, 7) Personal. A review of these points indicates an underestimation of CNS complications after pertussis immunization.
Evaluation of Lumbar Bone Mineral Density by Dual Energy X‐Ray Absorptiometry
Authors: Tsukahara Hirokazu , Nakashima Tetsuo , Yoshimoto Masahiro , Kuriyama Masanori , Shigematsu Yosuke , Hayashi Shuhei , Haruki Shinichi , Ishii Yasushi , Sudo Masakatsu
Published: October 2007

Using dual‐energy x‐ray absorptiometry (DEXA), the lumbar spinal bone mineral density (BMD) in 49 Japanese children with or without metabolic bone disease (MBD) was determined. The following results were obtained: (a) The normal data for healthy Caucasians (J Clin Endocrinol Metab 1990; 70: 1330‐1333) appear to be applicable to Japanese children; (b) BMD was normal in patients with congenital hydronephrosis with normal renal function; (c) One patient with congenital renal failure and one with Lowe syndrome had low BMD, but the MBD in the former improved markedly with peritoneal dialysis; (d) A reduced BMD was found in patients treated with long‐term steroids, probably because of decreased turnover of bone; (e) A reduction in BMD was pronounced in preterm infants during the first few months of life. In conclusion, DEXA is a useful method of bone densitometry in MBD in children.
Extramedullary Relapse in Childhood Leukemia
Authors: Tsukimoto Ichiro
Published: October 2007

As long‐term survival of children with leukemia is increasing, the prophylaxis of extramedullary leukemia has become a more important part of treatment. We studied the pattern of occurrence of extramedullary leukemia in a retrospective review. This review included a total of 2317 childhood leukemia patients aged 15 years or less who had been treated at 38 institutes in Japan between 1976 and 1985. Extramedullary leukemia developed in 386 of 1,724 ALL patients (22.4%) and 63 of 544 patients with ANLL (163%). Among the ALL patients, CNS‐L was the most common form and was observed in 315 cases (81.6%), followed by testicular leukemia in 89 (23.0%). In the case of ANLL, the most common form of extramedullary leukemia was CNS‐L (45 cases, 71.4%), followed by cutaneous leukemia in 10 cases (15.9%). In addition, leukemia of the lymph nodes, ovaries, bones, kidneys and eyes was observed in 7, 5, 5, 4 and 4 cases, respectively. The survival rate of ALL patients with CNS‐L was 40.1% for isolated relapse and 2.7% for bone marrow relapse, and no more deaths occurred after 6 years from relapse. The survival rate of patients with testicular leukemia was 40.1% for isolated relapse and 5.9% for complicating bone marrow relapse, and no deaths occurred after 7 years from relapse. Cutaneous leukemia tended to occur late in older children with ALL and early in infants with ANLL, and all these patients died. Infiltration into the kidney was observed in 4 patients, all of whom died. More than 75% of patients died after isolated relapse of leukemia of the bones, ovaries, lymph nodes and eyes. Prophylactic extramedullary leukemia therapy, of CNS‐L in particular, has played an important role in the treatment of childhood leukemia. Careful development of more effective therapy and close observation of late effects are required when monitoring affected children who are still growing.
Immunological Abnormalities and Endothelial Cell Injury in Kawasaki Disease
Authors: Nonoyama Shigeaki
Published: October 2007

Kawasaki disease is an acute febrile disease causing vasculitis, which may lead to as severe a complication as myocardial infarction. This disease is characterized by marked activation of the immune system, including elevation of various cytokines, polyclonal B cell activation, and decrease of CD8+ cells. The high levels of cytokines induce adhesion molecules and a new antigen on endothelial ceils. As a result, the immune cells bind to the endothelium and the activated immune cells produce cytotoxic antibodies against the new antigen on the endothelial cells. Thus, the activated immune system is involved in the endothelial cell injury. IVIG (intravenous immunoglobulin) therapy suppresses the activated immune system and reduces the endothelial cell damage.
Kawasaki Disease in a Father and Son
Authors: Iwata Fujihiko , Hanawa Yoshio , Takashima Hirotada , Shimoura Keichou , Nishibayashi Youhei
Published: October 2007

The pathogenesis and etiology of Kawasaki disease are unknown, but some studies suggest increased genetic susceptibility. The case is presented of an infant with Kawasaki disease whose father suffered from the same illness 21 years previously. The A, B and C loci of the HLA antigens were examined.
Analysis of Blood Spot 17α‐Hydroxyprogesterone Concentration in Premature Infants—Proposal for Cut‐Off Limits in Screening for Congenital Adrenal Hyperplasia—
Authors: Ohkubo Satsuki , Shimozawa Kazuhiko , Matsumoto Masaru , Kitagawa Teruo
Published: October 2007

Blood Spot 17α‐hydroxyprogesterone (17‐OHP) concentrations in neonates, especially in premature babies, were determined in relation to 1) the gestational age at birth, 2) the equivalent age of gestation at blood sampling and 3) the birth weight. The 17‐OHP concentrations were found to be higher with prematurity. Accordingly, the cut‐off limit in screening for congenital adrenal hyperplasia (CAH) in premature infants is proposed as 20 ng/ml. Ideal cut‐off limits were set by the equivalent age of gestation at blood sampling. Cut‐off limits on the basis of gestational age at birth and birth weight are also suggested, where the sampling age is not so advanced. The rate of false positivity in premature infants can be reduced by this method.
Inherited Deficiency of the Ninth Component of Complement Associated with Streptococcal Infection
Authors: Fujita Mitsue , Fujita Teizo , Inai Shinya
Published: October 2007

A 7 year old boy, who presented with streptococcal infection, was found to have a low serum complement level (CH50). The C9 component was undetectable. His CH50 rose to the normal value and remained normal for at least three weeks, but decreased to one‐third of the normal level three months later. Family studies were consistent with a familial C9 deficiency, with autosomal co‐dominant inheritance.
Carrier Detection in Agammaglobulinemia by X Chromosome Inactivation Analysis
Authors: Miyashita Toshiyuki , Iwata Tsutomu , Mizutani Shuki , Kamoshita Shigehiko , Kobayashi Noboru
Published: October 2007

Using a recently developed strategy to analyze patterns of X chromosome inactivation in cell populations, we found that two mothers and a sister were carriers in three atypical or sporadic cases of patients with agammaglobulinemia, two of whom were brothers. In this study, a phosphogiycerate kinase 1 (PGK1) gene probe was used to detect patterns of methylation of X‐chromosome genes. A random pattern of X inactivation was observed in isolated peripheral blood granulocytes. In contrast, one of the two X chromosomes was preferentially active in the Epstein‐Barr virus (EBV)‐transformed peripheral B cells of the family members of these patients. The volume of the blood specimen could be significantly reduced using EBV‐transfomed B cell lines which contained multiple clones. The analysis described here can be used to distinguish between X‐linked agammaglobulinemia (XLA) and other forms of a‐ or hypogammaglobulinemia as well as to detect the carrier state.
Oscillatory Variations of Cutaneous Blood Flow in Newborns Using A Laser Doppler Flowmeter
Authors: Takayanagi Toshimitsu , Fukuda Masafumi , Tsuji Yoshiro , Nakazawa Makoto
Published: October 2007

Oscillatory variations of cutaneous blood flow were studied using laser Doppler flowmetry in 20 newborns. Simultaneous monitoring of cardiorespiratory state was also performed. The frequency and amplitude of oscillatory curve varied, even on identical skin regions in each infant. When their respiration and heart rate were irregular, the frequencies of oscillatory curves were significantly higher and with a larger amplitude than when cardiorespiratory function was regular. The frequencies of oscillatory curves at two symmetrical points of an individiual were not different, but a significant difference in the oscillatory cycle was recognized at two asymmetrical points. Distributions of peak‐to‐peak time periods of oscillatory curves were studied in nine healthy and four sick newborns. Seven of nine healthy newborns had more than two peaks in their distributions, while only one peak with a narrowly‐spread distribution was observed in the four sick newborns with almost exclusively periodic breathing throughout monitoring.

In conclusion, we speculate that cardiorespiratory state and some neurogenic control affects the variations observed. In addition, the narrowly‐spread distribution of each time period appeared to be an important indicator of morbidity.
Report of a Japanese Girl with Marfan Syndrome Associated with Insulin‐Dependent Diabetes Mellitus
Authors: Yamamoto Toru , Inoue Fumio , Matsumura Atsuko , Kinugasa Akihiko , Sawada Tadashi , Hayashi Shosei , Hamaoka Kenji
Published: October 2007

A 3 year old girl was admitted to hospital in an emaciated condition and with polydipsia in October 1974. Following the diagnosis of diabetes mellitus, she Received treatment with insulin. On the first admission, a systolic murmur was noted at the apex of the heart. In 1981, the murmur was found to be continuous with a systolic click, and echocardiography demonstrated a mitral valve prolapse. In 1982, electrocardiography revealed left ventricular hypertrophy, and the patient's X‐ray showed vertebral kyphoscoliosis. Ophthalmological examination revealed slightly impaired visual acuity and a mild case of cataracts in 1986. The patient grew to be tall and thin with arachnodactylia of the hands, fingers, feet and toes. These symptoms and findings were compatible with Marfan syndrome, although the ophthalmological findings are not specific for this disease. This patient is the first case in Japan of Marfan syndrome associated with insulin‐dependent diabetes mellitus, although the relation between Marfan syndrome and IDDM remains unclear.
The Very Low Birthweight Neonate With an Interrupted Aortic Arch and Dextrocardia
Authors: Wakazono Yoshihiro , Yamada Itaru , Kagajou Keiichi , Miyazaki Masaaki , Isshiki Masayoshi , Takayama Naomasa , Shinnoka Shunji , Tomino Tetsuo , Furuya Keizou
Published: October 2007

Type 1A interruption of the aortic arch complex accompanied by mirror‐image dextrocardia with situs inversus was diagnosed by counter‐current aortography through the radial artery. This technique is useful for aortography of the very low birthweight neonate.
The Inhibition of Lymphocyte Blastogenesis by Asparaginase: Critical Role of Glutamine in both T and B Lymphocyte Transformation
Authors: Kitoh Toshiyuki , Asai Sadasaburo , Akiyama Yuichi , Kubota Masaru , Mikawa Haruki
Published: October 2007

L‐Asparaginase has long been used in the treatment of acute lymphoblastic leukemia or malignant lymphoma in childhood. To determine cell type specific sensitivity to this drug, the L‐asparaginase‐mediated inhibition of blastogenesis of human peripheral T or B lymphocytes was compared. The rate of incorporation of [3H]‐thymidine into the DNA of either T lymphocytes due to phytohemagglutinin (PHA) or B lymphocytes due to Staphylococcus aureus Cowan I (SAC) was measured by the addition of Escherichia coli L‐asparaginase in the medium. The blastogenic response of either T or B lymphocytes was also determined in medium depleted of exogenous asparagine and/or glutamine, both of which are hydrolyzed by this enzyme. The in vitro blastogenesis of either human T lymphocytes due to PHA or B lymphocytes due to SAC was inhibited by the inclusion of asparaginase in the medium. The deprivation of exogenous asparagine did not have any inhibitory effect on the blastogenic response of both T and B lymphocytes to each mitogen. On the other hand, the glutamine concentration in the culture medium provided a critical influence on the proliferative response of T and B lymphocytes. The rate of incorporation of [3H]‐thymidine into DNA was increased markedly as the concentration of glutamine was increased from 2‐7‐2 mmol/1. It is concluded that the mechanism of inhibition of PHA‐ or SAC‐stimulated lymphocyte blastogenesis by L‐asparaginase is not asparagine deprivation but glutamine deprivation. Glutamine, which is the most abundant amino acid, is thought to have an important role in the immune response of lymphocytes.
Determination of Serum Unbound Bilirubin for Prediction of Kernicterus in Low Birthweight infants
Authors: Nakamura Hajime , Yonetani Masahiko , Uetani Yoshiyuki , Funato Masafumi , Lee Yokei
Published: October 2007

Serum unbound bilirubin concentrations (UBC) and serum total bilirubin concentrations (TBC) were measured serially in 138 low birthweight (LBW) infants treated with phototherapy for non‐hemolytic hyperbilirubinemia. We attempted to assign the suitable critical UBC levels for predicting bilirubin encephalopathy into two different birthweight groups: a very low birthweight (VLBW) group (birthweight < 1,500 g) and an LBW group (birthweight between 1,500 g and 2,499 g). Twelve infants were diagnosed as ‘at risk’ for kernicterus, of whom 11 had signs of acute bilirubin encephalopathy and exchange transfusion. One VLBW infant had neurological sequelae at a 3 year follow‐up, although exchange transfusion was not carried out because of low TBC.

Sensitivity and specificity for predicting kernicterus were calculated at different UBC levels between 0.6μg/dl and 1.5μg/dl and TBC levels between 8 mg/dl and 26 mg/dl. The receiver‐operating characteristic (ROC) curves plotted for UBC as a predictor of kernicterus were clearly shifted up and to the left compared with the curves for TBC in the VLBW and LBW groups. Thus, the UBC measurement may well provide a more rational basis for evaluating the risk of kernicterus in LBW infants. The optimal cut‐off points were derived from these curves. In the VLBW group, the sensitivity was 100% and the specificity was 96% for a UBC of 0.8μg/dl, and 80% and 64% for a TBC of 11 mg/dl. In the LBW group, the sensitivity was 100% and the specificity was 98% for a UBC of 1.0μg/dl and 71% and 78% for a TBC of 16 mg/dl. These results suggest that UBC determination is more suitable for predicting kernicterus than TBC in LBW infants with non‐hemolytic hyperbilirubinemia.
Prevention of Perinatal Transmission of Hepatitis B Virus Carrier State
Authors: Tada Hiroshi , Uga Naoki , Fuse Yozen , Shimizu Mitsumasa , Nemoto Yuko , Wakae Eriko , Okamoto Yurino , Mishina Jun , Baba Kiyoshi
Published: October 2007

By means of passive and active immunization with hepatitis B immunoglobulin and hepatitis B vaccine, 396 of 407 babies born to hepatitis B antigen‐positive carrier mothers, were protected from establishing the hepatitis B virus (HBV) carrier state during a follow‐up period of 12 months or longer. Four infants developed the HBV carrier state before the completion of the immunoprophylaxis schedule, and another seven developed the state after the completion of the schedule. Seroconversion of anti‐HBc was observed in 26.8% of the successfully protected infants. In Japan a nationwide program to prevent the vertical transmission of HBV with these procedures was established in 1986, and so liver diseases due to HBV are expected to be eliminated in the near future.
Helicobacter pylori ‐ dependent intragastric urea biodegradation in children: Diagnostic and pathogenetic importance
Authors: Nijevitch Alexander A.
Published: October 2007

Abstract The objective of the present work was to study the relationship between intragastric urea hydrolysis generated by Helicobacter pylori urease and acid‐peptic disease in childhood. Intragastric urease activity was examined by urea and ammonia concentration measurement in gastric juice in 91 children with upper abdominal complaints. Helicobacter pylori infection was detected from 70 (76.9%) of 91 patients, including all of the 15 subjects with peptic ulcer disease. Helicobacter pylori‐related gastritis in children was associated with a decrease of urea and an increase of ammonia in gastric juice (P < 0.001) in comparison with H. pylori‐negative children. The gastritis score was correlated with the concentrations of urea and ammonia in the gastric juice of patients infected with H. pylori. There was a significant correlation between the histologically detected dissemination of organisms and gastric ammonia levels. Similar results were obtained concerning correlation between gastric juice ammonia and anti‐H. pylori specific immunoglobulin G versus highly purified antigen of H. pylori containing urease. Present findings prove that H. pylori plays an essential role in the pathogenesis of gastritis and that ammonia is one of the main pathogenic factors of acid‐peptic disease.
Acute bronchodilatory effect of salmeterol on methacholine‐induced bronchoconstriction in childhood asthma
Authors: Baki Ali , Karaguzel GÜLay
Published: October 2007

Abstract A review of the literature highlights the need for research, particularly on the acute bronchodilatory effect of salmeterol on bronchoconstriction in the pediatric age group. The present study attempted to evaluate the acute bronchodilatory effect of salmeterol on methacholine‐induced bronchoconstriction in childhood asthma and to compare it with the effect of salbutamol. Forty‐four asymptomatic children with mild‐to‐moderate asthma (23 boys and 21 girls; aged7–17 years) were studied. At the beginning, the baseline forced expiratory volume in 1 s (FEV1) was measured, and the methacholine challenge was performed by doubling the dose to determine PC20 (provocative concentration of inhaled methacholine required to reduce FEV! by 20%). At the same time, the transcutaneous arterial oxygen saturation (Sao2) was also measured. Each subject inhaled a single dose of 25 μ salmeterol (n: 23, group I) or 100 μg salbutamol (n: 21, group II) following the Sp2 measurement. The same measurements (FEV1, Sao2) were repeated 5 and 20 min after the inhalation. After inhalation of salmeterol or salbutamol, the differences between the values of FEV1 and SaO2 after 5 and 20 min were insignificant in both group I and group II (P > 0.05), although there was a significant improvement in both FEV1 and SaO2 after 5 and 20 min (P < 0.005). From these findings it was concluded that salmeterol can be considered as effective as salbutamol on methacholine‐induced bronchoconstriction.
Hypokalemic paralysis in association with acute gastroenteritis: A report of a sporadic case
Authors: Kinik Sibel Tulgar , SeÇMeer GÜLten , Kanra Guler , Ceyhan Mehmet , Halit Zafer Ecevit Khalil , ÖKsÜZ Nuray
Published: October 2007

Abstract Hypokalemic periodic paralysis is a condition, characterized by episodic weakness of the skeletal muscle. It may be presented as familial or sporadic (secondary). In sporadic cases, the patient may present with paralysis without any previous self or familial history. It is known that these patients are more susceptible to hypokalemia than normal individuals. In low serum K+ concentrations that the normal population can tolerate, these patients may easily suffer from paralysis. A sporadic case of hypokalemic quadriparesis associated with acute gastroenteritis without any self or family history is reported. The patient had experienced two episodes of quadriparesis within a short interval and responded dramatically to potassium replacement therapy.
Inhaled nitric oxide therapy via nasopharyngeal tube in an infant with end‐stage pulmonary hypertension
Authors: Kakuya Fujio , Takase Masashi , Ishii Noriyuki , Kajino Mayumi , Hayashi Tokitsugi , Miyamoto Kazutoshi , Muraki Senichi , Iwamoto Jun , Okuno Akimasa
Published: October 2007

Abstract The delivery of nitric oxide (NO) via a nasopharyngeal tube is an alternative to endotracheal intubation. A male infant with end‐stage pulmonary hypertension (PH) due to a severe hypoplastic lung developed a PH crisis on day 145 and received NO inhalation via a nasopharyngeal tube. Clinical improvement was maintained for 7 days with18–22ppm NO inhalation. The patient remained in close physical contact with his parents without the use of sedation. Blood methemoglobin levels remained below 1%. The environmental NO levels were less than 0.06 ppm and NO2 less than 0.3 ppm throughout the treatment, well within the safety margin. On day 152, the patient succumbed to hypoxemia and heart failure. The use of a nasopharyngeal NO delivery system without sedation, as an alternative to endotracheal intubation with sedation, was a practical method in treating a patient with PH while maintaining a certain quality of life for the patient and the family.
Uncommon multisystemic involvement in a case of Henoch‐Schönlein purpura
Authors: Kano Kenichi , Ozawa Takebumi , Kuwashima Shigeko , Ito Sachio
Published: October 2007

Abstract A case of Henoch‐Schönlein purpura (HSP) characterized by several unusual complications is reported. A 10‐year‐old boy was hospitalized with acute abdomen and developed purpura on the lower extremities after 4 days of hospitalization. He had protein‐losing enteropathy, diagnosed by an elevated fecal α‐1‐antitrypsin clearance. The colicky abdominal pain and protein‐losing enteropathy subsided after methylprednisolone pulse therapy was administered. He had left hydronephrosis and gall‐bladder abnormalities detected by ultrasonography, and purpura nephritis. However, after improvement of these abnormalities, he showed steroid‐induced epidural lipomatosis, detected by magnetic resonance imaging, which resolved with steroid reduction. Ultrasonography and magnetic resonance imaging were useful for detecting these uncommon multisystemic involvements in HSP.
Perinatal outcome and the social contract: Interrelationships between health and society
Authors: Gorski Peter A.
Published: October 2007

Abstract Rates of infant mortality and prematurity or low birthweight serve as indirect measures of the health of a nation. This paper presents current population data documenting the still serious problem of perinatal outcome in the USA as well as in other economically developed countries. International comparisons suggest that nations which have the greatest inequality of income and social opportunity also have the most adverse perinatal, child and adult health outcomes. Furthermore, the data assert that these effects are independent of average national wealth or gross national economic productivity. Health status differs by social class and race, even among the most affluent sectors of the population. All social classes, even the wealthiest, suffer the health consequences of social inequalities. An explanatory socio‐psychological theory of causality is proposed.
National survey of periventricular leukomalacia in Japan
Authors: Fujimoto Shinji , Togari Hajime , Takashima Sachio , Funato Masahisa , Yoshioka Hiroshi , Ibara Satoshi , Tatsuno Masaru
Published: October 2007

Abstract Background: Clarification of the present status of periventricular leukomalacia (PVL) in Japan.

Methods: Questionnaires were mailed to the leading neonatal intensive care units (NICU) and rehabilitation centers for children.

Results: The incidence of PVL in the group of surviving preterm infants of gestational ages less than 33 weeks was 4.8–4.9% on ultrasound and 7.7–7.9% on magnetic resonance imaging and/or computed tomography. The incidence of PVL did not decrease between 1990 and 1994. The incidence of PVL in the NICU varied from 0 to 47.1%. Infants from multiple pregnancies had a higher incidence (9.1%) of PVL than those from single pregnancies (6.2%). The proportion of PVL in all patients in rehabilitation centers with cerebral palsy (CP) has increased recently.

Conclusions: It was roughly calculated that about 750 cases of CP with PVL occurred annually in Japan, thus accounting for about one‐third of the total number of cases of CP.
An outbreak of hepatitis A in a day‐care center: Immunoprophylaxis with human immunoglobulin
Authors: Hatazawa Takako , Abo Wataru , Sakai Yoshiyuki , Seki Kimihira , Doi Toshiaki , Tachibana Naoki , Chiba Shunzo
Published: October 2007

Abstract Background: An outbreak of seven cases of hepatitis A (HA) occurred in a day‐care center. Five of the cases were children attending the center, one was a nurse and one was the mother of a child. It is probable that the first case with HA was a male child infected by an unknown source.

Methods and Results:
Human immunoglobulin (HIG) was administered to both children and staff at the center following which there were no new cases of infection among in‐center contacts. However, a new case of HA among household contacts developed 3 weeks following the treatment of in‐center contacts.

Conclusions
: The outbreak may have been prevented if the sibling (case 2) of the source case of infection (case 1) had been given HIG as soon as infection had been confirmed. Additionally, the data suggest that HIG for prevention of HA should be given not only to children but also to their parents.
Using polymerase chain reaction for rapid sex‐determination of a case of sirenomielia
Authors: Jin Yuko , Endo Akihiko , Shimada Masami , Minato Michiyoshi , Takada Masaaki , Takahashi Shigeru , Harada Kensuke , Hayakawa Satoshi , Sato Kazuo
Published: October 2007

Abstract A rare case of sirenomelia with its autopsy findings is reported. The 1188 g infant was delivered by cesarean section at the 36th week of gestation, and died at 4 h of life. Physical and pathologic findings included sirenomelia (a single lower extremity mass), lung hypoplasia, bilateral renal agenesis, blind‐ending colon, severe deformity of the bony pelvis, an imperforate anus, absence of the bladder, and a single umbilical artery. Because of the difficulty in determing the sex of the infant, PCR directed against the SRY gene was performed. The PCR‐based sex determination is a rapid technique in patients with ambiguous genitalia.
Modern management of childhood diabetes: A role for computerized devices?
Authors: Chiarelli Francesco , DI Ricco Laura , Catino Mariangela , Sabatino Giuseppe , Verrotti Alberto
Published: October 2007

Abstract Technology is increasingly relevant in everyday life and it can be very interesting to apply it also in the field of diabetes as it can be one way of ensuring better care. In the Diabetes Control and Complications Trial, the intensively treated patients were seen fortnightly in hospital and contacted even more frequently by telephone during the day as well as at night. For these reasons, health reforms are undergoing a radical change in an attempt to reduce the spiraling costs of health care provision. Therefore, it can be useful to use information technology (IT) in diabetes care. The European Federation for Medical Informatics recently established some Special Issues to advance IT initiatives that may be able to address the problem of adjusting the insulin dose and controlling blood glucose (BG) levels, as well as assisting in the provision of modern‐day diabetes care. The Special Issues can be classified under the following headings: databases, algorithms, decision support, models and education. It can be useful to develop a prototype computer system that can be applied to different areas of clinical diabetes care: computerized out‐patients' clinical databases that store patients' clinical and biochemical data; statistical and graphical analysis programs that help the clinician; dietary analysis programs which examine food composition and dietary exchanges and help devise meal plans; hand‐held insulin dosage adjustment computers that advise patients on a day‐by‐day or even meal‐by‐meal basis; expert systems and question and answer programs for patients' education, and finally games for children. These systems have the potential to be useful tools in many aspects of diabetes care but their utilization by the vast majority of the health care community has been extremely limited. Nonetheless, computers cannot substitute for the pediatric diabetes team, which remains the major determinant for better care of diabetes in children and adolescents.
Retrospective analysis of clonality and detection of residual disease in myeloid leukemia by FISH on long‐term stored bone marrow smears
Authors: Hyakuna Nobuyuki , Naritomi Kenji , Ito Etsuo
Published: October 2007

Abstract
Background.
Fluorescence in situ hybridization (FISH) has allowed the detection of numerical chromosomal aberrations in interphase nuclei on fresh or frozen smears of leukemia.

Methods.
To analyze clonality and residual disease in myeloid leukemia retrospectively, we applied FISH to bone marrow smears stored at ambient temperature for up to 9 years.

Results:
When hybridization efficiency was investigated on stored control smears from patients without hematological malignancy, more than 96% of nuclei showed the expected number of signals using DNA probes specific for chromosome 7, X or Y. In combination with cell morphology, we observed much higher hybridization efficiency in blasts and granulomonocytic cells compared with lymphoid and erythroid cells. On the basis of good hybridization efficiency for old smear specimens, we applied FISH to stored bone marrow smears of myeloid leukemias, in which either loss of chromosome 7 or loss of sex chromosomes had been verified previously by conventional cytogenetics (one patient with chronic myelomonocytic leukemia (CMML) and four with acute myeloid leukemia (AML; three M2 and one M7)). As a result, the loss of chromosome was detected in blasts from all patients and was observed in mature granulocytes, except in M7. In the CMML patient and one AML (M2) patient with t(8;21), lymphoid and erythroid cells also showed the loss of chromosomes, suggesting that it should occur at stem‐cell level. A high amount of residual disease was detected in the morphological remission samples in one AML (M2) patient after induction therapy. The patient eventually succumbed to relapse.

Conclusion
Thus, the present FISH technique is useful to analyze the clinical significance of clonality and the residual disease in myeloid leukemia, retrospectively.
Mutation in the gene encoding the fibroblast growth factor receptor‐3 in Korean children with achondroplasia
Authors: Yang Sei Won , Kitoh Hiroshi , Yamada Yasukazu , Goto Haruko , Ogasawara Nobuaki
Published: October 2007

Abstract
Background.
Achondroplasia (ACH) is the most common form of osteochondrodysplasia, and is mostly associated with a point mutation in the gene on the transmembrane domain of fibroblast growth factor receptor‐3 (FGFR‐3) on chromosome 4p.

Methods:
We investigated the mutations in the gene encoding FGFR‐3 in 15 Korean children with ACH, using polymerase chain reaction (PCR) coupled with direct sequencing.

Results:
In this study, all children with ACH showed the same mutation as those reported in France, USA and Japan; a G→A transition at position 1138 of the coding sequence, resulting in the substitution of arginine for glycine at position 380 of the mature protein.

Conclusions:
This consistent point mutation of Korean children with ACH indicates there is no significant racial difference in the pathogenesis of ACH, compared with data from Caucasian and Japanese children with ACH.
An evaluation of infants' growth in the Kingdom of Nepal
Authors: Hosono Shigeharu , Okazaki Minoru , Kagimoto Seiichi , Ogawa Kiyoshi , Matunaga Kohei , Oishi Tutomu , Ohno Tutomu , Yamaguchi Syuichi , Joh Kosuke , Akashi Syunji , Yamamoto Keiko , Kohno Saburo , Honma Takeshi , Shakya Kashyap Narsingh
Published: October 2007

Abstract
Background
: The His Majesty's Government/Japan International Cooperation Agency Primary Health Care Project began in April 1993 in collaboration with the Saitama Prefectural Government, for the purpose of improving the health status of the people in model districts of the Kingdom of Nepal. Growth monitoring is one of the basic methods that defines the health and nutritional status of children.

Methods
: Anthropometric indices were measured in 759 children in the Bhaktapur district. We used the World Health Organization prototype growth chart and national growth standard for Japanese children (1990) to analyze the growth data.

Results
: We found that the average bodyweight growth curve of children up to 4 months of age followed the 50th percentile reference curve. For children of5–12 months of age, there was a delay in bodyweight gain and the growth curve reached the 3rd percentile curve. For children more than 1 year old, the growth curve moved below the third percentile curve. Catch‐up growth did not occur before the children reached 5 years of age. The main causes of catch‐up growth being hampered were chronic undernutrition and inadequate nutritional balance.

Conclusions
: As this was the first opportunity to evaluate infant growth in this district, the first important consequence of the results was to analyze the causes of growth faltering and failure‐to‐thrive in Nepalese children. Even more important, was the need to give appropriate counseling on improving feeding and other health‐related practices, and the most important consequence of all was to instruct Nepalese health workers that utilizing the growth charts is an integral part of health care.
Isolated deficiency of glucocorticoids presenting with cholestasis
Authors: BerberoǦLu Merih , YiǦIt Sevket , ÖCal GÖNÜL , Kansu Aydan , Tarcan Aylin , Girgin Nurten , Suskan Emine
Published: October 2007

Abstract
Background:
Isolated deficiency of glucocorticoids is characterized by elevated levels of adrenocorticotropin (ACTH) and normal aldosterone production. It is rare for isolated deficiency of glucocorticoids to be associated with liver involvement. A case of an infant with isolated deficiency of glucocorticoids presenting with cholestasis is presented in this article. A male infant on his 39th postnatal day was referred to our hospital for evaluation of prolonged jaundice and convulsion. He had two episodes of hypoglycemic convulsion on postnatal 8th and 39th day, after which he was admitted to our hospital.

Results:
Physical examination revealed systemic jaundice, hyperpigmentation of the skin, hepatomegaly and splenomegaly on admission. He had normal male genitalia with 3.5 cm of penis and bilateral scrotal testes. Laboratory values were as follows: glucose 45 mg/dL, total biluribin 18.14 mg/dL, direct biluribin 6.54 mg/dL. aspartate aminotransferase 378 IU/L. alanine aminotransferase 46IU/L, and alkaline phosphatase (ALP) 1302 IU/L. In abdominal ultrasound and biliary tract scanning, extra‐ and intrahepatic biliary tracts were shown to be normal. Finally, biopsy of the liver revealed cholestasis. An endocrinological evaluation showed high levels of adrenocorticotropin (ACTH. 1000pg/mL), low levels of Cortisol (1 ug/dL) and normal aldosterone levels.

Conclusions:
The diagnosis of cholestasis secondary to isolated glucocorticoid deficiency was suspected with clinical and laboratory findings. Hydrocortisone treatment (30 mg/m2 per day) was initiated after which hyperpigmentation and jaundice decreased and ACTH and ALP levels reduced to 39 pg/mL and 440 IU/1, respectively. We emphasize that cholestasis in infants may be a component of isolated deficiency of glucocorticoids.
Mortality among patients with a history of Kawasaki disease: The third look
Authors: Nakamura Yosikazu , Yanagawa Hiroshi , Kato Hirohisa , Harada Kensuke , Kawasaki Tomisaku ,   THE KAWASAKI DISEASE FOLLOW‐UP GROUP
Published: October 2007

Abstract Background: Long‐term prognosis of Kawasaki disease is still unclear.

Methods: In a cohort study, 6576 patients with Kawasaki disease were observed from their first medical encounter because of the disease through the end of 1994, or until death. Standardized mortality ratios (SMR) with 95% confidence intervals (CI) were calculated with vital statistics data of Japan used for the control. Results: Of 6576 patients who met the eligibility criteria, 6550 (99.6%) were followed through either the end of the study or the date of death. Twenty patients (14 male, 6 female subjects) died during the study period; an overall SMR of 1.35 (95% CI 0.82–2.08) was calculated. The SMR was 1.45 (95% CI 0.79–2.44) for male subjects and 1.15 (95% CI 0.42–2.52) for female subjects. During the acute phase of the disease (the first 2 months after the first visit to hospital), the SMR was higher, particularly in male subjects (SMR 10.13, 95% CI 3.72–22.08). After the acute phase, however, both boys and girls had low SMR. Nine of the 20 deaths were caused by Kawasaki disease; there were three deaths as a result of congenital heart diseases and two subjects died of malignant neoplasms of lymphatic or hematopoietic tissues.

Conclusions: Although the mortality rate among those with a history of Kawasaki disease was elevated in Japan, many of the deaths that caused the elevation occurred during the acute phase of the disease. The mortality rate was not increased after the acute phase of the disease.
Community‐acquired severe staphylococcal septicemia in children: The relationship with blunt trauma
Authors: GÜLer Nermin , OneŞ ÜLker , YazicioĜLu Mehtap , YalÇIn IŞIk , Salman Nuran , Somer Ayper
Published: October 2007

Abstract Background. With the aim of establishing the role of blunt trauma, a trauma without any fracture or obvious wound, as a risk factor in children who were admitted for community‐acquired severe staphylococcal septicemia with various organ involvement, 63 patients were investigated during a seven‐year study period. All children were previously healthy.

Results: Blunt trauma was present in 12 (46%) of 26 septicemic patients in whom skeletal infections were diagnosed and the relative risk of trauma was high (odds ratio 15.0; confidence interval 2.9–75.8, P< 0.001) in these children. Patients with multiple organ involvement and with multifocal skeletal infections were more frequent among traumatized patients. Children with skeletal involvement were significantly older than others (P< 0.001). The period between the first symptom of infection and the diagnosis was shorter in traumatized children (P < 0.001). None of the children with staphylococcal pulmonary or soft tissue infection had any history of trauma. We conclude that blunt trauma is one of the important risk factors for skeletal involvement in staphylococcal septicemia. Its immunosuppressive nature, especially in local damage of the skin barrier, may be the cause of seeding and multifocal infection.
Human parvovirus B19‐associated thrombocytopenic purpura
Authors: Kido Shinji , Noriito Yoshi , Nishimura Naoko , Kajita Yuji , Ozaki Takao
Published: October 2007

Abstract We report two patients who presented with thrombocytopenic purpura (TP) associated with primary human parvovirus B19 (B19) infection. One patient also had transient Liver dysfunction. In both cases, B19‐DNA was detected in serum and bone marrow by polymerase chain reaction. Six months after the illness in patient 1 and 8 months after the illness in patient 2, B19‐DNA disappeared from the serum. Serum immunoglobulin (Ig)G antibody to B19 remained positive in both cases, but B19 IgM antibody became negative 3 months after the onset in case 1 and 4 months after the onset in case 2. The mechanism of TP by B19 infection is unknown.
Henoch Schönlein purpura and amebiasis
Authors: Demircin GÜLay , ÖNer AyŞE , ErdoǦAn ÖZlem , BÜLbÜL Mehmet , MemiŞ Leyla
Published: October 2007

Abstract The pathogenesis of Henoch Schonlein purpura (HSP) is unknown but is believed to result from an immune complex reaction to various antigenic stimuli, such as infectious agents. However, its association with Entamoeba histolytica has not been reported before. We present an 11 ‐year‐old boy with HSP, confirmed by the demonstration of leukocytoclastic vasculitis from skin and diffuse endocapillary proliferative glomerulonephritis, together with immunoglobulin A and complement component C3 deposition from renal biopsies. Cysts and trophozoites of Entamoeba histolytica were detected from the stool of the patient at the same time and disappeared after the treatment with metranidasole. The temporal association of these two disorders is either coincidental or due to a causal relationship between them.
Effect of body position on tidal volume and minute ventilation in very low birthweight infants
Authors: Itakura Yukino , Ogawa Yunosuke
Published: October 2007

Background:
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.

Methods and results:
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.

Conclusion:
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.
Effectiveness and safety of intravenous amiodarone in drug‐resistant tachyarrhythmias of children
Authors: ÇEliker Alpay , Ceviz Naci , ÖZme ŞEncan
Published: October 2007

Background: 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.

Methods and Results: 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 (range1–103 h). The mean effective maintenance dose was 10 ± 4.7μg/kg per min (range5–15 Hg/kg per min). In one patient, mild hypotension, and in three patients cellulitis occurred, but none of them necessitated termination of treatment.

Conclusions: 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.