bluuebunny/crossref_metadata_2025_split huggingface dataset:
Transient Neonatal Hyperphenylalaninemia due to Immature Development of 7, 8‐Dihydrobiopterin Synthesis
Authors: Sawada Yoshitomo , Shintaku Haruo , Isshiki Gen , Hase Yutaka , Tsuruhara Tsuneo , Oura Toshiaki 
Published: October 2007

SummaryWe describe a case with transient neonatal hyperphenylalaninemia, possibly due to immature biosynthesis of 7, 8‐dihydrobiopterin (BH2). In the neonatal period his blood phenylalanine levels were high and the patterns of urinary pterins were similar to those found in defective synthesis of BH2, which results in a type of hyperphenylalaninemia. But the laboratory data became almost normal after three months. Transient hyperphenylalaninemia has generally been thought to be caused by the delayed maturation of phenylalanine hydroxylase. But it is suggested that some cases with transient neonatal hyperphenylalaninemia are due to immaturity of BH2 cofactor biosynthesis rather than that of phenylalanine hydroxylase.
Interstitial Deletion of the Long Arm of Chromosome 13 and Retinoblastoma
Authors: Ohdo Shozo , Sonoda Tohru , Madokoro Harumichi , Hayakawa Kunio 
Published: October 2007

AbstractA female infant is reported in whom bilateral retinoblastoma developed due to interstitial delection of the long arm of chromosome 13. Her chromosome karyotype examined with peripheral lymphocytes proved to be 46, XX, del (13) (q12.3 q21.2). Parental chromosome karyotypes were normal.Clinical symptoms were bilateral retinoblastoma, retardation of growth and development, and mild microcephaly. No other marked dysmorphic features were noted.
Metabolism: Therapeutic Bone Marrow Transplantation in Niemann‐Pick Mice
Authors: Sakiyama Takeshi , Tsuda Masahiko , Owada Misao , Nakabayashi Hiroki , Miyawaki Shigeki , Kitagawa Teruo 
Published: October 2007

AbstractMice with Niemann‐Pick disease were treated with bone marrow transplantation. The loss of Purkinje cells remained unchanged, but the accumulation of sphingomy elin and cholesterol was reduced in the visceral organs, especially in the spleen, three weeks after marrow graft. The activity of acid sphingomyelinase was increased in the grafted liver of mice, and in the spleen this activity was elevated to normal levels.
Increase of Immunoglobulin‐ Bearing Peripheral Blood Lymphocytes in Children with Kawasaki Disease
Authors: Kuno‐Sakai Harumi 
Published: October 2007

AbstractImmunoglobulin‐bearing peripheral blood lymphocytes were measured serially in 15 patients with Kawasaki disease. IgG‐bearing lymphocytes and IgMbearing lymphocytes were increased significantly during the acute phase of the disease (p<O.O5). No such increase was observed during the convalescent phase (28th–45th day). Further investigation is needed to reveal the mechanisms of the increase of immunoglobulin‐bearing cells in the peripheral blood of children with Kawasaki disease.
Unusual Lack of the Epitope Recognized by OKT4 on the Helper/Inducer T Lymphocytes
Authors: Fukunaga Yoshitaka , Ogura Yuichi , Jinbo Osamu , Murakami Mutsumi , Yamamoto Masao , Ueda Yutaka , Good Robert A. 
Published: October 2007

AbstractLack of the epitope recognized by OKT4 monoclonal antibody on the helper/inducer T lymphocytes in a 14‐year‐old boy with IgA nephritis is described.The lymphocytes reacted normally with OKT3 /Leu4 and OKT8/αLeu2a monoclonal antibodies but not with OKT4 monoclonal antibody. Studies with other monoclonal antibodies (αLeu3a, OKT4A, OKT4B, OKT4C, OKT4D) which also identify the helper/inducer T lymphocyte subset revealed that cells of this population were present in normal numbers among the lymphocytes of the peripheral blood. Staining with OKT4 plus αLeu3a in normal persons indicated that T4 antigen is present on a small population of lymphocytes which lack Leu3a antigen. Further, the intensity of staining of the majority of cells in the subpopulation is increased when these two fluorescienated antibodies are used together. In this patient neither this small OKT4+ Leu 3a‐ population nor the cells bearing the Leu3a antigen showed OKT4 staining. The findings in the surface marker analysis of E+ OKT8‐ peripheral lymphocytes which were achieved by panning of the patient's peripheral cells indicated the existence of a population of E+OKT8‐ peripheral lymphocytes which were achieved by panning of the patient's periphera cells indicated the existence of a population of E+ (4A+4B+4C4D+)αLeu3al+ ly mphocytes in this patient. Lymphocyte responses to PHA, ConA and PWM, however, were all within normal range. Further, this patient had normal serum immunoglobulin levels and exhibited no symptoms or signs of immunodeficiency.These findings indicate that the patient under study has functionally normal helper/inducer T lymphocytes which lack the epitope recognized by OKT4 monoclonal antibody.
SCREENING SYSTEM: School Physical Examination and Mass Screening for Tuberculosis
Authors: Yamato Jungo 
Published: October 2007

AbstractThe spread of tuberculosis has been arrested in Japan, and this is especially remarkable in children. Taking as an example the incidence rate of tuberculosis in children of 5 ∼ 9 years of age, there has been a remarkable decrease from 261.3 in 1962 to 4.2 in 1983. In Japan, mass examination for tuberculosis is performed on primary school children in the 1st grade and also on junior high school children in the 1st grade. The detection rate of tuberculosis for these groups is very low. The future direction of examination for tuberculosis in school is as follows:

Records of examinations should be prepared carefully, so as to avoid omitting absentees in examinations.
Emphasis should be placed on examination of children who are in contact with newly detected active tuberculous patients.
Early visits to doctors are recommended when symptoms such as fever of unknown origin or persistent cough appear.
Platelet‐Associated IgG in Children ‐ Values and Evaluation of PAIgG in Various Thrombocytopenias ‐
Authors: Fujisawa Kohji , Akatsuka Jun‐ichi 
Published: October 2007

AbstractPlatelet‐associated IgG (PAIgG) levels were measured in 60 children with ITP (46‐chronic, 14‐acute) using Fab‐anti Fab radioimmunoassay method described by McMillan et al. In some patients platelet binding IgG in serum (PBIgG) was also determined at the same time. Patients with ITP had significantly greater PAIgG levels than 30 normal subjects and 13 non‐immune thrombocytopenic controls. Elevated PAIgG values did not correlate with parameters of platelet size (mean platelet volume; MPV and percentage of large platelet; PLP) and so these data indicated that high levels of PAIgG in ITP were not due to nonspecific adhesion of serum IgG to megathrombocytes usually increased in this disorder, but due to specific immunological reaction. PBIgG IgG values were also elevated in patients with pretreated chronic ITP, but high levels remained even after successful splenectomy.Furthermore, serial determination of PAIgG values were obtained in some patients with chronic ITP who underwent splenectomy and with acute ITP who achieved spontaneous remission. PAIgG returned to normal levels when thrombocytopenia disappeared. PAIgG seems to be the most reproducible indicator which reflects transition of the clinical picture in this disorder.
Partial Trisomy 18q Associated with Terminal Deletion of 5p: 46, XX, +der (5), t(5; 18) (p15.3; q12.2) pat.
Authors: Madokoro Harumichi , Ohdo Shozo , Sonoda Tohru , Togoe Tomoka 
Published: October 2007

AbstractA female infant with a 46,XX,+der95),t(5;18)(p15.3;q12.2) pat karyotype is reported. Her clinical features were milder than those of patients with full trisomy 18. She had no cat‐like cry. This suggests that the deletion of the 5p15.3 to pter is not responsible for the cat‐like cry noted in the patients with the 5p‐syndrome.
Chronic Granulomatous Disease in Japan: A Summary of the Clinical Features of 84 Registered Patients
Authors: Hayakawa Hiroshi , Kobayashi Noboru , Yata Junichi 
Published: October 2007

AbstractEighty‐four patients with chronic granulomatous disease, who were registered in the All Japan Immunodeficiency Registry, were analysed for their clinical features.Family history, presumed mode of inheritance, age of onset, prognosis, major symptoms (particularly infections), complications and some laboratory data were summarized and discussed in comparison with some previous reports. This is the first epidemiological report on chronic granulomatous disease from the Orient.
Impaired Neutrophil Adhesion: A New Patient in a Previously Reported Family
Authors: Fujita Kyoko , Kobayashi Kunihiko , Kajii Tadashi 
Published: October 2007

AbstractThis paper describes a male infant, the younger brother of a girl previously reported to suffer from recurrent skin infections, with an abnormality of neutrophil adhesion to a surface and a lack of two neutrophil membrane proteins, gp 110 and gp 115. The male infant, now seven months old, suffers from a disease identical with that of his sister. Their parents, both healthy, have reduced levels of the neutrophil glycoproteins.Scanning electron microscopy of the infant's neutrophils attached to a glass surface revealed a considerable reduction of pseudopods compared to a normal control. His neutrophils suspended in PBS, however, were morphologically normal. When stimulated in suspension by N‐formyl‐methionyl‐leucyl‐phenylalanine, the neutrophils also showed normal morphological changes. These findings indicate that the function of the cytoskeleton of the neutrophils is not impaired, but the function of the cell surface membrane is defective.Neutrophil adhesion assay using adhesion rate to nylon fibers as an indicator revealed both siblings and their parents as having a reduced adhesion rate. This finding, combined with the fact that the disease occurred in a sister and brother, lends further support to an autosomal recessive mode of inheritance of the disease.
Serum Lipid and Lipoproteins Concentrations in Obese Children as Atherogenic Risk Factors
Authors: Shigenaga Hirotoshi , Yoshii Osamu , Togoe Tomoko , Hayakawa Kunio , Hamada Keisuke , Kida Nobuaki , Ohtaki Sachiya 
Published: October 2007

AbstractNine hundred and ninety‐eight obese children aged 6 to 15 years participated in the present study. Fasting serum lipid and lipoprotein cholesterol values were measured and the relationship between obesity and abnormalities of serum lipids and lipoproteins was assessed. The results were as follows: 1. Obese children were more likely than non‐obese children to have elevated serum total cholesterol (TC), triglyceride (TG) and LDL‐C(low density lipoprotein cholesterol) levels and reduced HDL‐C (high density lipoprotein cholestrol) levels. 2. The more the relative body weight index increased, the worse the abnormalities of serum lipids and lipoproteins became. This tendency was marked in junior high‐school boys. 3. Hypercholesterolemia in obese children was mostly accounted for by LDL‐C only and elevated HDL‐C could not be detected.
Sequential Computed Tomography in Severely Asphyxiated Infants
Authors: Okada Masato , Yokochi Kenji , Suzuki Koichi 
Published: October 2007

AbstractSequential CT scans were evaluated in four term infants with severe asphyxia, who subsequently developed neurodevelopmental sequelae. The initial CT scans performed within the first week of life showed poor visualization of the ventricles, diffuse but mild low density in the cerebral hemispheres and the falx image. In two of them, remarkably low density was seen in the basal ganglia and thalami in the second CT scans. The last CT scans demonstrated multicystic encephalomalasia in one case and brain atrophy with thalamic high density due to postichemic hypervascularity in the other. Of the other two cases, one showed low density in the fronto‐temporal region in the initial CT scan and brain atrophy in the sequential scans. The other showed only transient periventricular low density. In conclusion, the morphologic changes seen in sequential CT scans continue until at least two months after birth and neonatal scans alone may not be useful for predicting the outcome.
Changing pattern in Reserve Capacity of Gonatropin Secretion
Authors: Hoshina Kiyoshi , Yoshida Yasuko , Kambara Misuzu , Tsukada Kazuko 
Published: October 2007

AbstractReserve capacity of Lh and FSH secretion was examined by standarized LHRH loading rest in 30 endocrinologically normal children, whose age ranged from 3 to 13 years.These sunjects were divided by sex. Both male and female subjects were divided into three groups by their chronological age, that is, Group A: under 6 years, Group B: 7 to 10 years and Group C: over 11 years of age. In the male the peakl values of LH were 10.86 ± 1.9 (mean ± SD) for Group a, 23,3 ± 7.3 for Group B, and 73.13 ± 6.4 mIU/ml for Group C. Corresponding values in the female were 8.58 ± 1.3, 20.56 ± 3, and 60.38 ± 8.5 mIU/ml, respectively. These intergroup differences in the peak LH values weer statistically significant (p<0.01)in both sexes. Regarding peak FSH values, no difference was seen among groups of either male or female cases.These findings indicate that even in prepuberty the reserve capacity of LH secretion begins to increase around the age of about 7 years.
Combined Passive and Active Immunization for Preventing Perinatal Transmission of Hepatitis B Virus Carrier State
Authors: Tada Hiroshi , Yanagida Masahiko , Mishina Jun , Sato Noriko , Aihara Shinobu , Baba Kiyoshi , Mayumi Makoto 
Published: October 2007

AbstractWith the intravenous and intramuscular injection of HBIG followed by repeated vaccinations, of 223 infants born to HBeAg‐positive HBV carrier mothers, 215 infants were prevented from developing HBV carrier state during a follow‐up period of longer than 12 months. The protective efficacy rate was 95.6%.During this study, four infants developed HBs antigenemia before the completion of the immunoprophylaxis schedule, and five after the completion of the schedule. Possible preventive methods for these infants for the ultimate prevention of persistent HBV infection are discussed.
Hepatitis B Virus Infection in the Infants Born to HBe Antigen Negative Carrier Mothers: A Preliminary Report
Authors: Shiraki Kazuo , Tanimoto Kaname , Yamada Kazuhito , Okada Takayoshi 
Published: October 2007

AbstractOne hundred infants born to hepatitis‐Be antigen (HBeAg)‐negative carrier mothers were followed with or without the administration of hepatitis‐B vaccine (HB vaccine) and/or hepatitis‐B hyperimmune globulin (HBIG). Among the infants without treatment, 7.6% of the infants born to the antibody‐to HBeAg (anti‐HBe)‐positive carrier mothers and 14% of those born to the carrier mothers without HBeAg and anti‐HBe developed HBs‐antigenemia, whereas none of the infants treated became positive for HBsAg. The results indicate the necessity for preventive measures for the babies born to HBsAg‐carrier mothers, regardless of their HBeAg state.
Functional Abnormalities of Neutrophils in Childhood Acute Leukemia: Decreased Myeloperoxidase and 13‐Glucuronidase Contents and Their Release
Authors: Hara Yoji 
Published: October 2007

AbstractThe myeloperoxidase and β‐glucuronidase contents of neutrophils as total index (T index) and their release induced by formyl‐methionyl‐leucyl‐phenylalanine (FMLP) as stimulation release index (SR index) and percent enzyme release index (%ER index) were assayed in children with acute leukemia at various stages.T indexes and SR indexes of these enzymes and %ER index of p‐glucuronidase were significantly decreased at onset or relapse (P<O.O5). In complete remission and off therapy, however, these enzyme activities and the enzyme release recovered, although they remained decreased in some patients. On the other hand, %ER index of myeloperoxidase in patients off therapy exceeded the normal range. The myeloperoxidase and β‐glucuronidase contents, and the release of myeloperoxidase (SR index) were significantly lower in patients with recurrent febrile episodes than in those without such complications.These studies indicate that the myeloperoxidase and β‐glucuronidase contents and their release are closely related to the stages of acute leukemia. The decrease of enzyme contents and enzyme release is one of the possible causes of recurrent infection in children with acute leukemia.
Endocrine Changes of the Hypothalamic, Pituitary and Adrenal Systems in Cyclic Vomiting
Authors: Aritaki Sekoiya , Ogihara Masaaki , Kusakawa Isao , Takamiya Hikari , Hori Yoshihiro , Kawashima Naoshi , Miyajima Tasuku , Chiba Tomoyuki , Tsuyuki Kazumitsu , Takekuma Koji , Matsuno Tetsuhiko , Honda Teruo 
Published: October 2007

AbstractChildren with cyclic vomiting had high blood ADH, ACTH, cortisol and plasma renin activity (PRA) during vomiting, and urinary excretion of 170HCS, 17KGS, 17KS, norepinephrine (NE), epinephrine (E), normetanephrine (NMN), metanephrine (MN) and vanillylmandelic acid (VMA) was increased. After disappearance of the vomiting, the levels fell to within the normal range. Variation was not observed in blood prolactin (PRL), Na, osmotic pressure or urine dopamine (DA). Normal findings were obtained in the intravenous water loading test, response of anterior pituitary hormones and cortisol to the insulin, TRH and LH‐RH loading, the metopirone test and the dexamethasone suppression test which were performed 1 week after the vomiting.
Quantitative Analysis of Cerebello‐Vestibular Function in Congenital Hypothyroidism
Authors: Sato Tamotu , Ishiguro Chiyoko , Watanabe Yukio , Mizukoshi Kanemasa 
Published: October 2007

AbstractUsing computed electro‐nystagmography, quantitative assessment of the cerebello‐vestibular function was performed in 13 patients with congenital hypothyroidism, aged 3 to 20 yrs, during adequate long‐term replacement therapy. Thirty‐two age‐matched children and 127 patients with confirmed cerebellar, brain stem or vestibular disorders served as controls. Routine neurological tests for body equilibrium revealed an abnormal finding in only two patients (15.3%). In contrast, the combination of spontaneous and positional nystagmus, caloric test, visual pendular rotation test, eye‐tracking test and optokinetic nystagmus test revealed abnormalities in 76.9% (10/13). Five patients had impairment of peripheral vestibular pattern, whereas 5 showed vestibulo‐central pattern. The critical factors producing the two different patterns appear to be the initial serum thyroxine level and the time of initiation of the therapy. Severe and long‐standing deficiency of thyroid hormone was associated with vestibulo‐cerebellar impairment, while mild hypothyroidism was associated with vestibular abnormalities. These results confirmed a high incidence of cerebello‐vestibular dysfunction as a neurological sequela of congenital hypothyroidism, even with early replacement therapy. The method affords a tool for objective evaluation of ill‐defined ‘clumsiness’ in congenital hypothyroidism.
Congenital Rubella Syndrome in the Ryukyu Islands: Birth‐Weight and Physical Status Before Two Years of Age
Authors: Tokugawa Ken , Ueda Kohji , Fukushige Junichiro 
Published: October 2007

AbstractInfants and toddlers with congenital rubella syndrome born in the Ryukyu Islands after the 1965 rubella epidemic were re‐evaluated in regard to birth‐weight and to body length, body weight, and head circumference before two years of age. Cases with cataract (with deafness and with/without congenital heart disease) showed a tendency of lighter birth‐weight (excluding the group with low birth‐weight) and also poorer physical status than the other cases. In general, an overall percentage of 26.0% were observed to show poorer values than the ‐2 standard deviation values of the general population before two years of age (20.6% in boys and 12.5% in girls for body length; 36.7% in boys and 41.9% in girls for body weight; and 27.9% in boys and 10.8% in girls for head circumference).
A Case of 48, XXX, +18 Double Trisomy
Authors: Imai Ikuko , Shimao Satoshi , Suzuki Yoshifumi , Okada Toshio 
Published: October 2007

AbstractWe report a case of double trisomy, 48, XXX, + 18 in a newborn female who had low set ears, prominent occiput, receding chin, overlapping fingers, structural heart disease and other malformations. These deformities are similar to the characteristic manifestations of trisomy 18 syndrome. She died on the 10th day. Autopsy revealed VSD, ASD, PDA, coarctation of the aorta, Meckel's diverticulum and cerebellar hypoplasia.Our case showed unilateral anomalies on the right side. These may help to suggest the diagnosis of double trisomy.
Immunoreactive Polymorphonuclear Leukocyte Elastase in Complex with Alphal‐Antitrypsin in Kawasaki Disease
Authors: Inamo Yasuzi , Harada Kensuke , Okuni Masahiko , Kimoto Kazuyoshi , Takeuchi Shigeo , Sakurabayashi Ikunosuke 
Published: October 2007

AbstractImmunoreactive polymorphonuclear leukocyte elastase (iPMN elastase) was measured by ELISA in 36 patients with Kawasaki disease and in 41 patients with anaphylactoid purpura and other infectious diseases of childhood. The iPMN elastase level was very high in Kawasaki disease compared with other diseases. There was no significant correlation in iPMN elastase levels in Kawasaki disease between the complicated and non‐complicated groups in the acute period. The iPMN elastase in the non‐complicated group fell below 250 4g/1 at 20 days from the onset of the illness. However, iPMN elastase in the complicated group (coronary aneurysm or dilatation) tended to remain high above 500 ligjl at 20 days from the onset. We suggest that increased iPMN elastase levels in Kawasaki disease reflect stronger enhancement and stimulation of PMN than in other diseases.
Clinical Significance of Lymphokine‐Activated Killer Activity in Childhood Acute Lymphoblastic Leukemia
Authors: Kato Masao 
Published: October 2007

AbstractLymphokine‐activated killer (LAK) activity was studied in 29 children with acute lymphoblastic leukemia (ALL). Peripheral blood lymphocytes of the patients and normal volunteers were cultured in the presence of recombinant‐interleukin 2 (R‐IL2) for five days, and assayed for their LAK activities by 51 Cr release assay using Raji cells as a target. At the time of onset, LAK activity was 4.3±2.6% (mean±SD, n=6) compared to the normal value. During remission LAK activity improved to 49.6±30.2% (n=16, p<0.01). After cessation of therapy LAK activity further improved to 97.3±21.2% (n=7, p<0.01). The conventional anti‐leukemic agents appeared to be suppressive for LAK activity in vitro. Of the patients in remission for more than one year, three with depressed LAK activity relapsed one to four months after the assay. Although it still remains undetermined whether the depression of LAK activity is responsible for the subsequent relapse, the application of adoptive immunotherapy using LAK and R‐IL2 is of clinical interest because it can be expected to enhance the activity of LAK. As compared with natural killer cell activity in childhood ALL, LAK activity showed a prompt improvement after remission.
A Case of Pelvic Osteomyelitis
Authors: Matsukura Hiroyoshi , Higuchi Akira , Suzuki Yoshifumi , Okada Toshio 
Published: October 2007

AbstractA patient presenting with osteomyelitis of the pelvis is described. In this case it was difficult to establish a correct diagnosis by use of scintigraphic scanning, in spite of clear roentgenographic evidence of osteomyelitis.