bluuebunny/crossref_metadata_2025 huggingface dataset:
Neonatal Pulmonary Hypertension: Pathophysiologic‐Based Therapy
Authors: Gersony Welton M.
Published: October 2007

Rational management of pulmonary artery hypertension (PAH) in the newborn infant must be based on an understanding of underlying causes. When a specific diagnosis can be made, empirical treatment gives way to specific therapy. For every pathophysiologic abnormality potentially resulting in PAH, clinical entities can be identified and key therapeutic interactions can be considered.

1. Pulmonary Venous Hypertension: PAH is secondary to obstruction of blood flow through the left heart. If based on anatomic abnormality (mitral or left atrial), surgical relief of obstruction is required. If PAH is the result or functional abnormality of the left ventricle (transient left ventricular ischemia), medical treatment of left ventricular failure is most important.

2. Functional Obstruction of Pulmonary Vascular Bed: Hyperviscosity of blood (polycythemia ‐ HCT > 65) results in increased pulmonary vascular resistance. Treatment consists of reduction of red blood cell volume.

3. Pulmonary Vascular Constriction: Hypoxemia with or without pulmonary parenchymal disease (primary or secondary persistent fetal circulation [PFC] syndrome). Therapy is directed at improvement of oxygenation and dilatation of pulmonary arteriolar bed (e.g. O2 hyperventilation and/or pulmonary vasodilator therapy).

4. Decreased Pulmonary Vascular Bed: If primary pulmonary hypoplasia is present, no treatment is available. If secondary form (diaphragmatic hernia), surgical repair and pulmonary vasodilatation of contralateral lung is indicated. Extracorporeal membrane oxygenation (ECMO) can be used in severe cases.

5. Increased pulmonary blood flow: Causes in the neonate include complex heart disease without pulmonary stenosis; and peripheral A—V fistula. Treatment: Surgical limitation of pulmonary blood flow may be required.

Even when PAH is the result of combined etiologies, a prompt well‐organized clinical management plan can follow from understanding of pathologic principles.
A New Interpretation of Child Ecology for the Growth and Development of Children
Authors: Kobayashi Noboru
Published: October 2007

A scientific basis to child ecology based on system theory and behavioral science is introduced. Every pediatrician wishes for the health of the body and the mind of children every time and everywhere. It can be said that child ecology is one of the most important approaches to our common goal. It is hoped that this paper may give pediatricians some insight into new interpretations of child ecology and the growth and development of children.
Echocardiographic and Pathoanatomic Assessment of Growth, Function and Chamber Volumes of the Normal Human Fetal Heart
Authors: Ninomiya Keiko , Onoda Yoshiaki , Ishikawa Tatsuo , Saito Masatoshi , Teshirogi Tadashi
Published: October 2007

To assess the growth pattern, relative left ventricular (LV) and right ventricular (RV) sizes, function and volumes, 168 echocardiograms and 14 postmortem hearts of normal human fetuses were studied. LV and RV diameters, atrioventricular valve diameters, wall thicknesses and aortic diameter increased linearly with gestational age from 20 to 39 weeks, but percent changes in ventricular diameters and wall thickness were constant and similar between LV and RV in echocardiograms. The ratios of LV and RV diameters, wall thicknesses and mitral and tricuspid valve diameters were constant and were approximately 1.0 throughout the period studied in echocardiograms and postmortem hearts. LV and RV volumes in postmortem hearts were also similar. LV volumes of postmortem hearts were best approximately by an ellipsoid model, and clinical application of this model to echocardiograms showed a linear increase of LV volume with age and constant ejection fraction and LV output per body weight. These results suggest a linear growth pattern and constant systolic function of the fetal heart, and do not support the concept of RV dominance.
Growth of Japanese Fetuses ‐Analysis from the Viewpoint of Population and Biological Growth Curves‐
Authors: Yamada Takako , Nishida Hiroshi , Yamaguchi Kiyoko , Sakamoto Shoichi , Sakanoue Masamichi
Published: October 2007

In 1984, Peter Dunn postulated that infants from 28 weeks ‘gestation till 18 weeks’ postnatal age who grow normally have almost identical potential growth velocities, regardless of racial and geographic differences. He suggested that the straight line growth curve in this period could be used as a perinatal growth chart for international reference. We composed biological growth curves for Japanese fetuses by eliminating data with risk factors for fetal growth, and compared them with the curves of Dunn. Our conclusions are as follows:

1) Japanese population fetal growth curves correspond with the curve of Dunn between 28 and 38 weeks' gestation.

2) Even in ideal conditions, flattening of fetal growth after 38 weeks' gestation always occurs.

3) In the early postnatal period, infants grow with the same straight line velocity as during fetal growth between 28–38 weeks' gestation. Infants from 28 weeks' gestation to 18 weeks' postnatal age have an almost identical growth potential in spite of some degree of growth constraint in utero after 38 weeks' gestation.
Multiple Insulin Injections in Adolescent Diabetics Using a Pen‐Type Syringe
Authors: Sasaki Nozomu , Miyamoto Shigeki , Nishioka Tadashi , Niimi Hiroo , Nakajima Hironori
Published: October 2007

Multiple insulin injections using the NovoPen, a new device for insulin administration, were given for 12 weeks to seven adolescent patients with insulin‐dependent diabetes mellitus (ages: 13–18 years; duration of diabetes: 4–14 years). Human Actrapid insulin was injected before every meal using the NovoPen, and human Monotard insulin was injected at bed‐time with a conventional syringe.

The mean total daily insulin doses at the beginning and end of the study were 1.3 and 1.2 U/kg body weight, respectively. Monotard insulin was increased during the study. Blood HbAl values declined from 16% to 13% in two patients. The incidence of hypoglycemia did not increase and severe hypoglycemia did not develop. No patient reported difficulty in using the NovoPen. Some, however, had problems in administering insulin at school, but they preferred to continue using the device.

The results show that, for adolescent patients,.the NovoPen offers advantages in management and may improve diabetic control.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome. Ill Electron Microscopic Observations on Group A Hemolytic Streptococci Inoculated into Mice
Authors: Akiyama Takehisa , Osawa Nobutaka , Nakasato Kazumi , Yamashina Shohei , Yamamoto Noboru , Tamaki Hideaki , Yashiro Kimio
Published: October 2007

Because of various circumstantial evidence, we have been asserting the streptococcal etiology of mucocutaneous lymph node syndrome (MCLS), although there has been no recovery of Streptococcus pyogenes from the patients at any site.

The present study was designed to provide a satisfactory explanation for the failure to isolate these bacteria from patients with MCLS, by electron microscopic observation of group A ftera‐hemolytic streptococci which had been inoculated intramuscularly into mice.

“Spherical bodies” were detected in biopsy specimens obtained from the infection sites from 10 – 14 days onward after infection; these were spherical, 0.5–1.5 yua in diameter, devoid of cell walls, perhaps uncultivable on any culture medium, and undistinguishable from those discovered in the buffy coat from peripheral blood of MCLS patients and described as ruined etiological microorganisms by Ueno et al.

The implications of these findings, particularly in terms of the etiology of MCLS, were discussed.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome. IV Immunological Tolerance Following Exposure of Neonatal Mice to Live Attenuated Strains of Streptococcus pyogenes
Authors: Akiyama Takehisa , Osawa Nobutaka , Yashiro Kimio
Published: October 2007

The absence of seroconversion to streptolysin O in convalescent patients with mucocutaneous lymph node syndrome has been a perplexing problem for investigators who are in favor of a streptococcal etiology of the disease. In the present studies, mice subjected in the newborn period to an infection with a strain of Streptococcus pyogenes, which fails to confer cellular immuno‐responsiveness even in adult mice, and then reinfected four to six weeks later with another strain of the same species, which has an intrinsic ability to render mature murine hosts sensitive to streptococcal antigens, expressed an enhanced cell‐mediated immunity coupled with a lack of humoral responsiveness to the same antigens. Such immunological characters are an exact counterpart of those of MCLS patients. The involvement of these immunological characters in the pathological process of the disease is discussed.
Specific Responses to Staphylococcus aureus in Patients with Hyper‐lgE Syndrome
Authors: Yanagi Tadamichi , Yasui Makoto , Tsuji Yoshiro
Published: October 2007

Specific responses to Staphylococcus aureus (S.aureus) were assessed to prove a decrease or defect of specific protection against S.aureus in patients with hyper‐IgE syndrome (HIES). Interleukin‐1 (IL‐1) production, interleukin‐2 (IL‐2) production and response to IL‐2 were studied in three groups (A —patients with HIES, B — control patients with S.aureus infections, C — healthy controls). When lymphocytes or monocytes (Mo) were stimulated by lectins, there was no difference in the three groups. In the experiments concerning IL‐1 derived from Mo activated by S.aureus, the proliferation of cultured fibroblasts showed higher levels of DNA synthesis in the A or B group than in the C group. The IL‐2 derived from peripheral blood lymphocytes (PBL) activated by S.aureus showed higher levels of activity in the B group than in the A or C group. The response to IL‐2 in the A group was decreased as compared with the C group, and a control patient aged 7 years showed the highest level of all subjects. When PBL were activated by S.aureus as a specific antigen, both IL‐2 production and response to IL‐2 showed lower levels of activity in the A group than in the B group.

These results indicated that there was a defect or decrease of specific protection against S.aureus in patients with HIES.
Pathogenetic Mechanisms of Exercise Induced Asthma and the Refractory Period
Authors: Belcher Nick G. , O'Hickey Steve , Arm Jonathan P. , Lee Tak H.
Published: October 2007

Exercise is a powerful stimulus to the development of asthma. In most asthmatic subjects the airways obstruction recovers spontaneously within 60 minutes, but in some subjects there is more prolonged airflow obstruction which requires bronchodilator treatment. Approximately 40–50% of subjects with EIA will show a refractory period of 2–4 hours after an initial exercise task, during which time an identical exercise task will evoke significantly less (%lt;50%) bronchoconstriction. In some patients, particularly children, EIA will be followed 3 to 9 hours later by a further episode of bronchospasm, termed the late asthmatic response. There remains considerable debate about the pathogenesis of EIA the refractory period and the late asthmatic response.
Study on Rapid Turnover Proteins in Neonates
Authors: Yen Ko‐Wen , Matsuo Masafumi , Nakamura Hajime , Matsuo Tamotsu
Published: October 2007

Prealbumin is a so‐called rapid turnover protein which has a relatively short half‐life of two days. It has been shown to be a useful indicator of nutritional adequacy in premature babies. Retinol‐binding protein (RBP) and transferrin are two other rapid turnover proteins. The purpose of this study was to determine whether serum retinol‐binding protein and transferrin can be used for evaluating protein‐calorie adequacy in neonates, by correlating serial measurements of their concentrations with serum prealbumin concentrations. Prealbumin, transferrin and RBP concentrations were measured by a single radial immunodiffusion method. Our study showed that there is a significant difference in mean concentration of serum prealbumin between full‐term AFD (appro‐priate‐for‐dates) and SFD (small‐for‐dates) babies (p < 0.05), but not in preterm babies. In normal birth‐weight infants, the concentration of RBP correlates positively with prealbumin (r=0.71), but this correlation was not observed in low birth‐weight infants (r = 0.58). We speculate that the concentration of RBP is an indicator of conditions other than protein nutrition. The serum concentration of transferrin does not correlate with prealbumin. Transferrin is a poor indicator of protein‐calorie adequacy in neonates.
(2) Inspiratory Muscle Hyperreactivity in Children with Severe Asthma
Authors: Obata Toshihiko , Likura Yoji , Homma Ikuo
Published: October 2007

Intercostal EMG's (EMGic) and diaphragmatic EMG's (EMGdi) were studied to evaluate the role of the inspiratory muscles in compensation for an increased load during provocation tests with methacholine. The EMG's were obtained with surface electrodes. Methacholine provocation tests were performed using an Astograph which demonstrated the dose response curve to respiratory resistance (Rrs) on an X‐Y recorder. The moving averages were analyzed in terms of the rates of rise (Xp/Ti) and peak activities (Xp). Experiments were performed on 15 asthmatics, aged 11 to 17 yrs, (6 mild and 9 severe asthmatics) and three normal subjects, aged 8 to 13 yrs. Pulmonary function values before provocation tests, except for mid‐maximal expiratory flow (MMEF), showed no significant differences among these three groups.

Changes in Xp and Xp/Ti were measured at the degree of 1.5 and 2 times the initial respiratory resistance level. In severe asthmatics, the changes in Xp and Xp/Ti were significantly larger than those of mild asthmatics, especially in intercostal Xp/Ti.

From our results, the severe asthmatics had respiratory muscle hyper‐reactivity compared with mild asthmatics, especially in the intercostal muscles.
Analysis of Killer Cell Activities in Epstein‐Barr Virus Infections
Authors: Yanagisawa Mitsuhiko
Published: October 2007

Using spontaneously established autologous lymphoblastoid B cell lines (LCL), killer cell activities were studied in children with severe infectious mononucleosis (IM), chronic IM, and acute IM, and compared with those in EBV‐seropositive normal controls. Natural killer (NK) cell activity of fresh peripheral blood mononuclear cells (PBMC) was normal in acute IM patients, but it was low in four of six patients with severe or chronic IM. Recombinant inter‐leukin 2 (rIL‐2)‐activated PBMC from normal controls showed lymphokine‐activated killer (LAK) cell activity against the respective autologous LCL. The levels of LCL lysis by LAK cells were significantly higher in acute IM patients, lower in chronic IM patients, and much lower in severe IM patients. In contrast to the fact that PBMC stimulated in vitro with autologous LCL (IVS cells) from normal controls and acute IM patients showed potent killing of autologous LCL, IVS cells from severe or chronic IM patients showed lower levels of LCL lysis, which were markedly augmented in three patients by rIL‐2 addition to the cultures. These killer cell dysfunctions appear to be responsible for the severe or chronic course of EBV infection.
DIDMOAD Syndrome (Wolfram Syndrome) in Four Male Siblings
Authors: Kumaki Satoru , Mori Hideki , Suzuki Hitoshi
Published: October 2007

Four male siblings including two typical cases of DIDMOAD syndrome are described. The typical cases were aged 16 and 15, with diabetes insipidus, insulin‐dependent diabetes mellitus (IDDM), optic atrophy, perceptive hearing loss, dilatation of the urinary tract and disturbed EEC One of the others was aged 10, with bilateral concentric limitation of the peripheral visual fields, retinal pigment degeneration and disturbed EEG, and the other was aged 6, with IDDM and perceptive hearing loss. The family history revealed complicated parental consanguinity, with some members suffering from diabetes mellitus and deafness. In our cases hereditary transmission seemed to be autosomal recessive, as previously described, and we could not find any association of this syndrome with HLA.
A Premature Infant with a Bilateral Thalamostriatal Hemorrhage: Brain Imaging and Pathology
Authors: Hokazono Yoshimi , Yokochi Kenji , Ohtani Yoshiaki , Inukai Kazuhisa , Takashima Sachio
Published: October 2007

Hemorrhagic areas were seen on ultrasonography and computed tomography in both thalamostriatal regions in a preterm female infant with perinatal asphyxia due to abruptio placentae. At autopsy, marked perivascular bleeding in the thalamus and putamen and eosinophilic neuronal changes in the thalamus and pontine tegmentum were seen. These thalamostriatal and brain stem lesions are thought to have been caused by an acute process causing total asphyxia.
Non‐Radioactive cDNA Probes for in situ Localization of mRNA
Authors: Nakane Paul K. , Moriuchi Tetsuya , Koji Takehiko , Tanno Masashi , Abe Kaoru
Published: October 2007

DNA labeled with non‐radioactive markers has been utilized for detection of specific DNA or RNA either on filters or in cells and tissues. The presence of protruding markers on the probe DNA has been considered to be a cause for loss of sensitivity and specificity of hybridization. As a non‐protruding marker, we introduced the use of T‐T dimer, which can be generated easily and is a potent hapten. As a marker for DNA, T‐T dimer in DNA was generated by UV irradiation. The T‐T dimerized DNA (T‐T DNA) was then hybridized with complementary DNA or mRNA either on a nitrocellulose filter or in cells. Then the hybridized T‐T DNA was detected immunohistochemically using rabbit anti‐T‐T DNA and peroxidase‐labeled goat anti‐rabbit IgG. The use of T‐T as marker offers several advantages over other markers; it appears not to interfere with hybridization efficiency, is simple to make and can be detected with high sensitivity.
Maternal Inheritance and Study of Human Mitochondrial Genome
Authors: Takeshita Kenzo , Kamimura Naohisa , Ishii Shogo
Published: October 2007

Several diseases with non‐mendelian maternal inheritance patterns have been described, some of which appeared to be associated with abnormal mitochondrial structure or function and might result from the inheritance of an abnormal mitochondrial genome. New techniques in molecular genetics should clarify the molecular basis of these maternally inherited developmental disorders. From families with maternally inherited mitochondrial disorders we conducted a search for an alteration in nucleotide analysis of the mitochondrial DNA obtained from the lymphocytes of the patients. Although it was possible to identify a single substitution (CTT/ATT) in one of five clones in which were transcribed the gene of cytochrome C oxidase subunit II, it was impossible to define the mismatched site by the method of RNA/DNA mapping (Myers, 1985) using a labelled RNA probe containing a cytochrome C oxidase gene which was obtained from normal placenta cells. A congenital myotonic dystrophy is known to be inherited through an autosomal dominant gene, and yet the age of onset and the severity of the disease are influenced by non‐mendelian maternal inheritance. We previously proposed that an abnormality of bile acid metabolism could affect the fetus, resulting perhaps in delayed development. A possible model for this effect might involve the interaction of mitochondrial function with an autosomal gene product, leading to erroneous replication with a mutant form of the polymerase subunit.
A Screening Method for Adrenoleukodystrophy Using a Dried Blood Spot on Filter Paper
Authors: Nishio Hisahide , Kodama Soichi , Nakajima Toshihiro , Matsuo Tamotsu , Mio Takaya , Sumino Kimiaki
Published: October 2007

The authors have devised a new, simple method for the diagnosis of adrenoleukodystrophy (ALD) using a dried blood spot sample. Fatty acid from the dried blood spot was extracted and methylated with HCl‐methanol. Fatty acid methyl esters were analyzed by gas chromatography‐mass spectrometry. Fatty acid composition of the blood spot from five patients with ALD and from five healthy controls was determined from the mass chromatogram of the m/z 143 ion, [(CH2) 6 COOCH3]±. The ratios of tetracosanoic acid to docosanoic acid (C24:0/C22:0) and of hexacosanoic acid to docosanoic acid (C26:0/C22:0) were significantly greater in ALD patients than in controls. The C24:0/C22:0 ratios of ALD patients and of controls were 2.08 ± 0.33 and 1.47 ± 0.16 (mean ± SD), respectively. The C26:0/C22:0 ratios of ALD patients and of controls were 0.31 ± 0.024 and 0.092 ± 0.041, respectively. The fatty acid composition of the dried blood spot did not change at room temperature within a week. Since the specimens can be sent by mail, this method could be applied to the screening of other “peroxisomal” diseases, such as neonatal ALD and Zellweger syndrome, as well.
Clinical Use of Oka Live Varicella Vaccine
Authors: Kamiya Hitoshi , Sakurai Minoru , Ihara Toshiaki , Ito Masahiro , Torigoe Sadayoshi , Ota Yutaka , Kamiya Toshiya , Horiuchi Kiyoshi , Asano Yoshizo , Baba Koichi , Takahashi Michiaki
Published: October 2007

A live varicella vaccine (Oka strain) developed in 1974 was successfully administered to children with various diseases and to normal children. Leukemic children could be immunized safely and effectively using our immunization criteria. All immunized subjects had a good immunoresponse, and the titer of antibodies in 14 children were well maintained over 8 years. The Japan Varicella Vaccine Study Group (JVVSG) found that reactions developed in 46 out of 251 (18.3%) leukemic children in remission and in 38 out of 79 (48.1%) children immunized as an emergency procedure, respectively. Some severe reactions were observed in the latter group. Healthy children were also vaccinated safely and the serocon‐, version rate was satisfactory. The varicella skin reaction became positive after varicella immunization in most children. The incidence of herpes zoster in immunized children with acute leukemia was less than in natural infection. These results indicate that live varicella vaccine is useful to prevent varicella in high‐risk children as well as in normal children.
Concentrations of Zinc, Copper, Manganese and Selenium in Blood and Urine of Patients with Prolactinoma
Authors: Nishi Yoshikazu , Aihara Katsuaki , Hatano Shuichi , Kihara Mikio , Ohta Masahiro , Sakoda Katsuaki , Uozumi Tohru
Published: October 2007

This study was designed to evaluate trace element metabolism in patients with prolactinoma. The mean concentrations of zinc in plasma, erythrocytes and urine, copper in plasma and erythrocytes and selenium in plasma were within normal limits before and after surgery. Urinary copper excretion was significantly lower than in controls, before surgery (<0.05) and rose towards normal after treatment. Erythrocyte manganese content was significantly lower than in controls, in patients with prolactinoma before surgery P<0.05) and rose towards normal after treatment.
Early Treatment of Inborn Errors of Biopterin Metabolism
Authors: Hase Yutaka , Okano Yoshiyuki , Sawada Yoshitomo , Shintaku Haruo , Yamamoto Hiroko , Oura Toshiaki
Published: October 2007

Neurological symptoms in 6‐pyruvoyltetrahydropterin synthase (PTPS) deficiency (formerly called dihydrobiopterin synthetase deficiency) are caused by lack of neurotransmitters in turn due to deficiency of tetrahydrobiopterin (BH4) in the brain. The response to treatment in three patients with PTPS deficiency was variable. Trial of BH4 monotherapy in two patients, after three months and one year, respectively, was unsuccessful. We believe that BH4 monotherapy is unsafe even in the absence of symptoms. The levels of pterins and neurotransmitter metabolites, and the Ne/B ratio, in the cerebrospinal fluid are more reliable indicators of disease activity than in urine and serum. The prognoses of some patients with PTPS deficiency are poor, and brain damage may develop in utero. BH4 is transported from mother to fetus in guinea pigs when a large amount of BH4 is administered. If this is true in humans, prenatal treatment of PTPS deficiency might be feasible.
Long‐Term Follow‐Up of Infants from Birth for Rotavirus Antigen and Antibody in the Feces
Authors: Nishio Osamu , Ishihara Yuichi , Isomura Shin , Inoue Hiromasa , Inouye Sakae
Published: October 2007

We followed up five infants from birth to five years of age for the shedding of rotavirus and the appearance of IgA antibody in the feces, in order to know the relation of rotavirus infections with diarrheal symptoms and the host's responses in fecal IgA antibody production. We collected fecal samples basically once a week from the infants, and evaluated the contents of rotayirus antigen and antibody and total IgA in all the samples by enzyme‐linked immunosorbent assays. We also detected virus particles in some concentrated samples by electron microscopy. We found that all the infants shed a large amount of rotavirus in the winter seasons up to two years of age, but two infants showed no symptoms of diarrhea.

After primary infection, the specific IgA index, defined as the specific IgA antibody titer divided by the total IgA concentration of each feces, rose and fluctuated from time to time. However, we observed occasional eight fold or greater rises in the index between two consecutive fecal samples, suggesting anamnestic responses in fecal IgA antibody production. Some of the rises were accompanied with mild diarrhea and the presence of a low amount of virus in the feces detected by electron microscopy. Probably, repeated mild reinfections with rotavirus are occurring in infants at all seasons.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome VII. A Weak Response to Epstein‐Barr Viral Capsid Antigen in Mice Infected Neonatally with S. pyogenes
Authors: Akiyama Takehisa , Osawa Nobutaka , Yamaura Noboru , Yashiro Kimio , Watanabe Mitsuru
Published: October 2007

A negligible, if not entirely negative, production of antibody to Epstein‐Bart virus(EBV)‐associated antigens in patients with mucocutaneous lymph node syndrome(MCLS) has been a perplexing problem for investigators who are in favor of a streptococcal etiology of the disease.

In the present investigations, antibody formation to EB viral capsid antigen (VCA) was assessed by the use of the indirect immunofluorescence technique in mice which had been subjected to neonatal infection with an attenuated strain of Streprococcus pyogenes or inoculation with either heat‐killed streptococci or streptolysin‐O, both of which were emulsified in Freund's complete adjuvant (FCA).

Each mouse of these groups was inoculated 30 days or so later with EBV dilution in FCA, blood was collected about three weeks after virus infection, and antibody titers were assessed individually.

According to the results of these experiments, no apparent difference was detected among the mice, irrespective of the methods of their neonatal treatment concerning their refractoriness to EB‐VCA; the majority of the animals showed titers of less than 1:8, no case having a titer above 1:30. In contrast, all sera from control mice yielded positive responses showing antibody titers of 1:250 and over.

Such an immunological character is an exact counterpart of that of MCLS patients. Accordingly, the recent research focus on EBV as a possible etiological agent of MCLS seems to be too restrictive.
Neonatal Hypothyroid Screening Using Enzymeimmunoassay of Thyrotropin — Comparison with Radioimmunoassay
Authors: Kusuda Satoshi , Tsuruhara Tsuneo , Hase Yutaka , Miyagi Tomiko , Oura Toshiaki , Ishibashi Kaichiro , Kawashima Minoru , Ichihara Kiyoshi , Miyai Kiyoshi
Published: October 2007

In order to evaluate a new enzymeimmunoassay (EIA) of thyrotropin(TSH) for neonatal hypothyroidism, 6,100 blood samples on fdter paper, obtained through a neonatal hypothyroid screening program, were examined using a one‐step sandwich EI A and a radioimmunoassay(RIA). Measured TSH values, numbers of cases and false‐positives detected, and recall rates, were compared between the two methods. During this study two cases of congenital hypothyroidism were found by both EIA and RIA. However, a patient with hyperthyrotropinemia was detected only by the EIA. The reproducibility of the EIA was slightly better than that of the RIA. These results suggest that the EIA has same or higher sensitivity for detecting patients compared with the RIA. On the other hand, three cases with falsepositive elevation of TSH by EIA were found. High TSH values by the EIA were likely to be due to transplacental factors, probably immunoglobulin G. Because of high reproducibility of the EIA and false‐positive cases found with it, recall rates of the screening program were slightly higher for the EIA than for the RIA. The recall rate of the EIA could have been decreased by setting a cut‐off point at a higher percentile. Since the EIA is simple to use, this method is suitable for screening for congenital hypothyroidism.
Blood Ammonium Level in Low Birth Weight Infants in Relation to Arginine Intake
Authors: Nakamura Shinji , Kondo Yuuichi , Ogata Takayuki , Iribe Kaneshige , Kudo Yumiko , Ikeda Tetsuo , Matsuda Ichiro
Published: October 2007

The blood ammonium level was monitored in low birth weight infants (<2,500 g at birth, N=117). There was no significant correlation of the blood ammonium level with gestational age, body weight at birth or the Apgar score. When the blood ammonium level was compared in infants fed with sufficient amounts of arginine‐supplemented (N=29) and non‐supplemented formulas (N=12) in addition to the mothers' milk, the blood ammonium level was found to be significantly lower in the former group than in the latter group (50.1 ± 12.4 μmol/L vs 40.0±12.2 μmol/L; p<0.02). There was no difference in the urinary excretion of orotic acid. This observation indicated the benefit of arginine supplementation, especially in the feeding of low birth wight infants.
Hereditary Nephrogenic Diabetes Insipidus Type‐2
Authors: Matsumoto Tohru , Ito Yuhei , Yukizane Shigenori , Ichikawa Kotaro , Yamashita Fumio
Published: October 2007

Nephrogenic diabetes insipidus (NDI) in a fetus in utero was suspected because of the family history, and evidence of increased amniotic fluid by echosonography. The mother's karyo‐type was 46 XX/47 XXX. After birth, the patient was diagnosed as NDI type‐2 which is partially resistant to antidiuretic hormone.
Defective Immune Response to Epstein‐Barr Virus in Patients with Acute Lymphocytic Leukemia
Authors: Wakiguchi Hiroshi , Fujieda Mikiya , Matsumoto Kenji , Ohara Yuji , Wakiguchi Akiko , Kurashige Takanobu
Published: October 2007

Thirteen children with acute lymphocytic leukemia (ALL) receiving maintenance chemotherapy were tested for anti‐Epstein‐Barr virus (EBV) antibodies, EBV specific cytotoxic T lymphocyte (EBVCTL) activity and spontaneous cytotoxicity against Raji cells in order to define the defective cellular immunity in ALL children during complete remission. Among 10 seropositive patients, anti‐virus capsid antigen (VCA) antibody titer vaned from 1:20 to 1:320 and anti‐EBV nuclear antigen (EBNA) antibody was not detectable in four. No patients were positive for antiearly antigen (EA) antibody. EBVCTL activity and inter‐leukin 2 (IL‐2) or interferon a (IFNα) augmented EBVCTL activity in eight seropositive patients was significantly lower than that in the seropositive age matched control group. IFNα, OK432 or IL‐2 augmented spontaneous cytotoxicity were also significantly lower in the patients compared to those in the control group. These defective killer cell activities may allow EBV‐infections to enter a severe, fulminant or persistently active state in the patients with ALL receiving aggressive maintenance chemotherapy. (Acta Paediatr Jpn 1989; 31: 144–149)
Promoting Breast‐Feeding—A Successful Regional Project in Japan
Authors: Kobayashi Michiko
Published: October 2007

According to Health and Welfare Ministry figures (1985), 95% of pregnant women wished to breast‐feed their baby, and the number of breast‐fed newborns is increasing annually. Nevertheless, the Ministry reports that only 49.5% of one‐month‐old babies are still being breast‐fed. In 1977, Ina Health Center established Mother‐Baby Clinics, offering education about breast‐feeding. The Center also started research in 1983, with the cooperation of the Kami‐Ina Public Health Nurses' Association, into infant feeding styles. Since then, the local breast‐feeding rate has risen well above the national average. This paper presents practical guidance toward encouraging successful breast‐feeding. First, timely advice must be offered to pregnant women, to establish a correct understanding of the importance of breast‐feeding. The mother's strong intention is a major factor, as is support from people around her ‐family, employer, health care professionals. The most important period for establishing breast‐feeding is the first three months. Adequate milk secretion, good breast condition, and proper sucking are also crucial. Education and further research are still needed.
Human Milk for Low Birthweight Infants: Immunology, Nutrition and Newer Practical Technologies
Authors: Narayanan Indira
Published: October 2007

Human milk has a number of unique properties which can be of benefit to high risk low birth‐weight and preterm infants. Immunological features are of particular importance. Growth is better with the use of the mother's own milk than with pooled bank milk. The quality of the latter can be improved by various measures noted in the text. Growth can be further improved by enriching the milk with human milk factors, powder formula or protein supplements. As breast‐feeding is best for infants, the maternal milk supply should be maintained during the hospital stay of infants by frequent expression, galactogogues used where necessary under supervision, encouraging maternal contact with the infant, promotion of suckling initially on the “empty” breast after expression of milk, and subsequently institution of direct breast‐feeding. With proper interventions and motivation of both staff and mothers, even very low birthweight infants below 1,500 gm can go on, in time, to exclusive breastfeeding.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome VIII. Immunological Tolerance to S. pyogenes‐Associated Antigens Seems Essential to Induction of MCLS
Authors: Osawa Nobutaka , Yarnaura Noboru , Ikewaki Nobunao , Yashiro Kimio , Akiyarna Takehisa
Published: October 2007

In our previous studies, we attempted to show that new‐born mice infected with an attenuated strain of Streptococcus pyogenes, and exposed to streptococcal antigens approximately one month later, failed to develop a humoral response to those antigens but were able to respond normally to other unrelated antigens. Since such immunological characteristics are identical to those of patients with the mucacutaneous lymph node syndrome (MCLS), the present studies were performed with the aim of assessing the antibody‐forming activities of peripheral blood lymphocytes collected from MCLS patients. These lymphocytes were cultured in the presence of pokeweed mitogen, streptolysin‐O and streptococcal C‐polysaccha‐ride, leading to their refractoriness to S. pyogenes‐associated antigens coupled with normal responsiveness to unrelated antigens. The implication of such immunological responsiveness elicited from neonatal exposure to streptococcal antigens is discussed, particularly with reference to the induction of MCLS.
Syndrome of Resistance to Thyroid Hormone in an Infant with Congenital Cytomegalovirus Infection
Authors: Hirano Takeki , Jogamoto Mitsuto , Chin Ishou
Published: October 2007

We describe an infant with congenital cytomegalovirus infection who developed the syndrome of resistance to thyroid hormone at three weeks of age, presenting with elevated thyroxine levels and non‐suppressible thyroid‐hormone‐stimulating hormone secretions without any features of thyrotoxicosis. The resistance was present at the peripheral and pituitary levels and resolved spontaneously by 18 months of age. Cytomegalovirus infee tion was confmed by the positive urine culture of this virus. This case demonstrates for the fit time a possible association of thyroid hormone resistance with congenital cytomegalovirus infection.
Possible Role of Streptococcus pyogenes in Mucocutaneous Lymph Node Syndrome IX. Quantitation by ELISA of Streptococcal Pyrogenic Exotoxin in the Serum of MCLS Patients
Authors: Kawai Mayumi , Osawa Nobutaka , Yamaura Noboru , Ikewaki Nobunao , Yashiro Kimio , Hiraishi Satoshi , Watanabe Mitsuru , Akiyama Takehisa
Published: October 2007

In the present paper we describe the use of an enzyme‐linked immunosorbent assay reinforced with an introduction of monoclonal antibody, for the detection and quantitation of streptococcal pyrogenic exotoxin (SPE) in the serum of patients with mucocutaneous lymph node syndrome (MCLS). The amount of SPE was usually at a high level, and its 100% incidence in patients' sera was proved whenever the assay was made on the day of admission, thereby showing a marked contrast to carefully matched control sera which failed to mediate any positive result. As for the change in detected amount of the toxin, a clear dichotomy was observed between the serum of gammaglobulin‐treated patients and that of infants given aspirin; in the former the positive result turned to negative rapidly following the initiation of treatment coupled with a defervescence, while in the latter the reduction of SPE levels was scarcely monitored for as long as 17 days after the onset of illness. Quantitation of SPE might be an auxiliary test for the diagnosis of MCLS, because a considerable amount of SPE was assessed in a patient who developed characteristic huge coronary artery aneurysms following an illness which did not fulfill the diagnostic criteria. These findings support our speculation in relation to the certain role of S. pyogenes as an etiological agent for MCLS. The possible mechanisms of gammaglobulin treatment in reducing the prevalence of cardiovascular lesions and the duration of systemic inflammation are discussed.
Acromesomelic Dysplasia in a Father and Son: Autosomal Dominant Inheritance
Authors: Ohba Ken‐ichi , Ohdo Shozo , Sonoda Tohru , Madokoro Harumichi
Published: October 2007

A father and son with typical acromesomelic dysplasia are reported, with the father more severely affected than the son. The disease was thus apparently transmitted in an autosomal dominant fashion. This is at variance with the autosomal recessive mode of inheritance assumed from hitherto reported families.
Chemical Mediators in Hypersensitivity Reactions
Authors: Nagakura Toshikazu , Ebisawa Motohiro , Reason Donald C. , Likura Yoji
Published: October 2007

The study of chemical mediators is essential in clarifying the mechanism of hypersensitivity reactions. Recent advances in this field have been made particularly with the arachidonic acid metabolites, several of which strongly contract smooth muscle, attract granulocytes to inflammatory foci and lead to increased vascular permeability. These functions play a crucial role in allergic reactions. Furthermore these mediators might work to modulate the intracellular netwodc which mediates the complicated inflammatory process of hypersensitivity reactions.
Relation between Blood Pressure, Left Ventricular Mass and Pulsed Doppler Parameters in Healthy School Children
Authors: Watanabe Koji , Kishida Kenji , Hayashi Yurika , Ozasa Hiroshi , Masuno Hideko , Haneda Noriyuki , Nishio Toshikazu , Mori Chuzo
Published: October 2007

We analyzed the relations between blood pressure in sitting and supine positions, left ventricular mass (LVM) and Doppler aortic, pulmonary and mitral flow velocity measurements in 163 healthy school children. Systolic blood pressure in a supine position correlated significantly with aortic acceleration (ATc) and ejection time)ETc), corrected by the square of R‐R interval, pulmonary AT and peak flow velocities. Moreover, the systolic blood pressure in a sitting position correlated with pulmonary AT and LVM. LMV correlated with pulmonary ATc, the ratio of AT to ET andaverage acceleration, and aortic ETc and peak flow velocity. These data suggest the following: (1) the posture influences the relation between blood pressure and flow velocity, (2) pulmonary hemodynamics are influenced by systemic blood pressure in healthy children and (3) the development of LVM may be dependentnot only on blood pressure but also on cardiac work in childhood.
The presence of Hypodense Eosinophilic Granulocytes in Allergic Children.
Authors: Kemal Wishwadewa Wirya , Yamada Tsubara , Shike Shoichiro
Published: October 2007

To evaluate hypodense eosinophils known very little in children, peripheral blood from asthmatic, allergic‐non‐asthmatic and non‐allergic children was evaluated by density Percoll gradient techniques. A significantly higher number of eosinophils were hypodense in asthmatic children (n = 24) compared with allergic non‐asthmatic (n = 10) and non‐allergic children (n = 13), namely 484 ± 348 cells/40 μl versus 113 ± 109 and 39 ± 61 (p <0.001) respectively. We also observed by light and electron microscopy that the hypodense eosinophils of asthmatic children were swollen and their granules were dispersed, but the normodense eosinophils of the same patient were small and compact. In three cases of severe asthmatic attack, hypodense eosinophils found on admission decreased in number after intravenous aminophylline therapy and relief of symptoms. Moreover, a decreased number of hypodense eosinophils were found in seven cases of allergic children (p < 0.05) after 2 weeks of antiallergic drug treatment associated with relieved symptoms. From these data we concluded that the presence of hypodense eosinophils in the peripheral blood might be related to the development of allergic symptoms and might participate in the pathophysiology of allergic diseases in children.
Possible Role of Streptococcus Pyogenes in Mucocutaneous Lymph Node Syndrome. XII. Variable Responses of Platelets in MCLS Seem to Be Explainable by Streptococcal Pyrogenic Exotoxin
Authors: Akiyama Takehisa , Osawa Nobutaka , Shimanuki Kaoru , Yashiro Kimio , Oyake Tadashi
Published: October 2007

As a model system for mucocutaneous lymph node syndrome (MCLS), we have advocated and used mice which had been rendered tolerant to Streptococcus pyogenes‐associated antigens by neonatal infection with group A fteta‐hemolytic streptococci, because these mice have shown a variety of peculiar bioimmunological characteristics bearing a striking resemblance to those of MCLS patients. The results of our current investigations reaffirmed the reliability of the animal model by indicating that mice subjected to neonatal infection with 5. pyogenes, or inoculation with streptococcal pyrogenic exotoxin (SPE) in Freund's adjuvant, were perfect counterparts of patients with MCLS on account of their platelet activation and hyperaggregability in response to provocative treatment, which are familiar findings in this disease.
Epidemiology of Group C Rotavirus Gastroenteritis in Matsuyama, Japan
Authors: Ishimaru Yoshiro , Nakano Shozo , Nakano Hiroko , Oseto Mitsuaki , Yamashita Yasutaka
Published: October 2007

The epidemiology of rotavirus gastroenteritis was studied in the Matsuyama district from January to May 1988. Among strains of rotaviruses detected 46 showed the group C‐specific PAGE pattern of virus RNA. Group C rotavirus gastroenteritis occurred following an epidemic of group A rotavirus infection, primarily in children aged 4–7 years but rarely in those aged 0–1 years. This was seriologically confirmed after the epidemic. Unlike the previous strains detected in Matsuyama in 1985–1986, PAGE of the RNA showed arrangement of the 5th, 6th and 7th segments at similar intervals, suggesting virologic variation. The symptoms of fever, diarrhea and vomiting were milder than in group A rotavirus infection used as a control.
Postnatal Head Growth in Very Premature Infants with Good Outcome
Authors: Fujimoto Shinji , Ishikawa Tatsuya , Togari Hajime , Kanayama Manabu , Okajima Kazuki , Inukai Kazuhisa
Published: October 2007

Postnatal head growth was examined retrospectively in 118 infants (male 60, female 58) born with a gestational age of from 24 to 29 weeks. Infants fulfilled the following criteria: (1) Those with hydrocephalus and small for dates (‐1.5 standard deviation) were excluded. (2) Infants had normal intelligence and no cerebral palsy or epilepsy at 3 years of age or above. They were divided into four groups according to gestational ages: 24–26 wks (n = 21), 27 wks (n = 25), 28 wks (n = 41) and 29 wks (n = 31). The head circumference was measured at least twice monthly. The head growth curves in each group were obtained. After a transient decrease, head growth averaged 1.1‐1.2 mm/day from day 21–30 to day 61–70 in all groups. From day 61–70 to 91–100 head growth averaged 1.2 mm/day at 24–26 wks and 0.8 mm/ day at 27–29 wks, respectively.
1H‐NMR Studies of Urine in Propionic Acidemia and Methylmalonic Acidemia
Authors: Kodama Soichi , Sugiura Makiko , Nakao Hideto , Kobayashi Ken , Miyoshi Mari , Yoshii Katsuhiko , Komatsu Mikio , Sakurai Takashi
Published: October 2007

The present study deals with the usefulness of 1H‐NMR spectroscopy in the chemical diagnosis of propionic acidemia and methylmalonic acidemia. A Varian VXR‐500 spectrometer was used.x H‐NMR spectra of urine from a patient with propionic acidemia show peaks of 3‐hydroxy‐n‐valerate, methylcitrate, propionyl glycine, 3‐hydroxy‐n‐butyrate, lactate, 3‐hydroxypropionate, tiglylglycine and hippurate. The assignment was made possible by the use of 2D COSY. The spectra from a patient with methylmalonic acidemia show methylmalonate and glycine. 1H‐NMR spectroscopy has the advantages of requiring a short time for analysis, relatively small amounts of urine and no preliminary extraction.
Setleis Bitemporal “Forceps Marks” Syndrome and Its Pathogenesis: A Case Report
Authors: Matsumoto Shigetaka , Kuno Tateo , Hamasaki Yuhei , Miyazaki Sumio , Miyabara Shinichi , Narisawa Yutaka
Published: October 2007

Setleis bitemporal “forceps marks” syndrome is characterized by “forceps marks” and a peculiar facies. The syndrome has previously been reported mainly in the Puerto Rican population. We describe here a Japanese boy with the syndrome. In addition, the hypothesis is presented that the Setleis syndrome may result from an insufficient migration of neural crest cells into the frontonasal process and the first branchial arch.
Congenital Myopathies
Authors: Goebel Hans Hilmar
Published: October 2007

About forty different congenital myopathies (CM) are defined by clinical and morphological criteria. Classical types like central core disease, centronuclear myopathy, and nemaline/rod myopathy are now well established and recognized as neuromuscular conditions. Clinical subtypes as infantile, juvenile, and adult forms have been recognized in several CM. Not infrequently, different disease‐specific morphological features may occur in muscle tissue of the same patient combined. Other CM are marked by aggregates of desmin filaments indicating the importance of recent immunohistochemical techniques. Modern myopathological techniques enabled nosological separation of CM, immunohistochemistry, actually, may usher in a new period of research in and understanding of CM. However, application of molecular genetic and molecular biological methods to CM may clarify still unsolved aspects of gene localisation for which the hereditary nature of many CM is particularly conducive, aspects of heterogeneity versus homogeneity of certain CM or clinical variants, of prenatal diagnosis of CM, of pathogenetic and nosological significance of muscle fiber proteins in CM, and of a new nosological classification of CM.