bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Studies of Patients with Rubella Syndrome Occurring in a High Incidence in the Ryukyu Islands in 1965: On the Diagnostic Significance of Clinical Manifestations
Authors: Ueda Kohji , Nishida Yukiaki , Kano Masaaki , Oshima Kenji , Takabayashi Kazuaki , Kato Hirohisa , Nagafuchi Masaaki , Takesue Masayoshi , Yoshizawa Yoshiaki , Nagayama Tokuro 
Published: October 2007

AbstractThe 620 cases with a history of maternal rubella and/or with one or more of cataract, CHD and deafness born in the Ryukyu Islands in 1965 were subjected to clinical studies and 360 cases were diagnosed as rubella syndrome. Rubella retinopathy makes a reliable diagnostic basis for rubella syndrome. As for the typical combination of clinical manifestations, the cases with cataract have CHD, deafness and retinopathy; the cases with CHD not accompanied by cataract have deafness and retinopathy; and the cases with deafness not accompanied by cataract and CHD either have or have not retinopathy. The cases with cataract alone or with CHD can extremely rarely be denned as having rubella syndrome. There existed the close relationship between the combination of clinical manifestations and the time of maternal rubella infection.The main clinical manifestations of rubella syndrome are cataract, congenital heart disease (CHD), and deafness appearing singly or two or more together1)2). Etiology of the congenital cataract, CHD, or deafness is mostly not explained; and only a small portion of the incidence is due to intrauterine rubella infection. For the future of the patients in the above conditions and for their parents, clarification as to whether they are or are not due to rubella is very important. Current general practice for the diagnosis of rubella syndrome is laboratory diagnosis that measures rubella hemagglutination inhibition (HI) antibodies3)4). As the infants grows in age, the above mentioned technique may not assure an absolute judgment5)6). This makes it necessary to arrive at diagnosis from the clinical findings. A prompt, direct diagnosis from the clinical findings alone will be very convenient. In the Ryukyu Islands where there was a high incidence of rubella syndrome in infants in the second half of 1965, we analyzed clinical findings of the affected children with cataract, CHD or deafness on the basis of serological diagnosis. The diagnostic significance of the clinical findings was so studied. Hence our report in this paper.
Effect of Excess Phenylalanine on Utilization of Glucose in Rat Brain
Authors: Kuroda Yasuhiro , Sumitomo Misako , Miyao Masuhide 
Published: October 2007

SummaryThis study was undertaken to determine the effect of excess phenylalanine on utilization of glucose in rat brain. 1) Carbon dioxide formation from glucose in the homogenates of fetal and adult brains was progressively inhibited as the concentration of phenylalanine was increased in the reaction mixture. On the other hand, carbon dioxide formation from pyruvate was little affected in either the fetal or adult brain when the reaction mixture was supplemented with 5 mM phenylalanine. These results indicate that the high concentration of phenylalanine does not inhibit the tricarboxylic acid cycle activity, but the glycolytic activity in the rat brain. 2) The lactate and glucose concentrations in rat brain were measured at brief intervals after the production of complete ischemia by decapitation, in order to investigate the inhibitory effect of phenylalanine on the glycolytic activity in rat brain in vivo. Lactate production during 3 minutes after decapitation was not significantly inhibited, when the phenylalanine concentration in rat brain was elevated to 0.8 to 1.0 mM.
Epidemiology of Kawasaki Disease in Japan
Authors: Yanagawa Hiroshi , Shigematsu Itsuzo , Kusakawa Sanji , Kawasaki Tomisaku 
Published: October 2007

AbstractThree nation‐wide epidemiological surveys, three case control studies and a few other epidemioligical studies on mucocutaneous lymph node syndrome have been conducted in Japan since 1970.The principal epidemiological features of the disease are (1) total numbers reported as of June 1974 being 6,537, (2) prevailing throughout the country without north‐south or urban‐rural differences, (3) steady increases after 1968 without distinct seasonal variation, (4) peak age at 6–11 months in male and 12–17 months in female, (5) male/female ratio in incidence: 1. 5/1, that of fatal cases 4/1, (6) family and area clustering, (7) previous history of pharyngitis and allergic reactions, (8) no specific characteristics in weight at birth, birth order, immunization history, artificial feeding, (9) temporal curves of incidence consistent with the production of certain medicines.
Neonatal Mass‐Screening for Inborn Errors of Metabolism in Japan
Authors: Kitagawa Teruo 
Published: October 2007

AbstractThis report describes the results of neonatal mass screening in Japan for PKU, maple syrup urine disease, histidinemia, homocystinuria, galactosemia, inborn errors of urea cycle metabolism and congenital hypothyroidism. The incidence of PKU is low but the incidence of histidinemia is somewhat high compared to that in the United States. Some genetic variants of each of these inborn errors of metabolism such as malignant hyperphenylalaninemia or mild form of MSUD have been detected in the neonatal screening. Clinical values of the screening for each of these in born errors of metabolism are discussed.
Secretin Secretion in Infancy and Childhood. Part 1. Plasma Secretin Levels in Infants and Children
Authors: Yamamoto Koichi 
Published: October 2007

AbstractIn order to elucidate the development of secretin secreting ability in children, plasma secretin levels of children (361 cases), pregnant women (11 cases) and umbilical cord blood samples (35 samples) were measured by double‐antibody radioimmunoassay method using 125I‐[Tyr1] ‐secretin as tracer.

Maternal plasma secretin level immediately before delivery was significantly higher than those of cord plasma and there was no correlation between the two types of plasma levels. Accordingly, it was thought that secretin can not transfer through the placenta from mother to fetus.
Between cord arterial and venous plasma secretin levels, there was a positive correlation (p < 0.005, r = 0.5863, n = 19) and the difference was not significant.
Plasma secretin levels increased gradually for a week after birth and the plasma secretin level on the seventh day was significantly higher than that on the first day (p < 0.04).
Plasma secretin levels in infants lowered a little up to the fourth month but rose again from the fifth month. It was considered that this rise was due to the influence of the initiation of weaning.
In children and school‐children, plasma secretin levels increased rapidly at five years of age and thereafter they showed near adult values or a little higher. It was thought that secretin secreting ability matures at approximately 5 years of age.
The mean plasma secretin levels in childhood appeared paralleling fairly well the growth of gastric acid secreting ability.
Some Problems of Neonatal Infections in Japan
Authors: Fujii Ryochi 
Published: October 2007

AbstractFor the prosperity of Japan in the 21st century, we pediatricians play a very important role in securing the health of future adults. Although our infantile death rate has become one of the worlds lowest recently, the number of neonatal deaths can further be reduced by preventing infections and accidents. Because of the huge consumption of antibiotics in Japan for past decades, the drug resistance of pathogens has increased remarkably and we are forced to choose potent new antibiotics for saving neonatal lives. Some unsolved problems associated with GBS infection in Japan are mentioned and the necessity of development of immunopotentiators as well as chemotherapeutics and vaccines in treating neonatal infections is discussed.
Developmental Maturations of B Cell Differentiation Ability and T Cell Regulatory Function in Man1
Authors: Miyawaki T. , Moriya N. , Nagaoka T. , Yachie A. , Taniguchi N. 
Published: October 2007

AbstractAs assessed in Nocardia mitogen system, B cell differentiation ability and T cell help for IgM‐production appeared to be nearly maturated at birth, but those for IgG‐ and IgA‐production were still deficient in cord blood and required much more time for their maturation. Deficit in concanavalin A‐inducible humoral suppressor activity and marked excess in pokeweed mitogen‐inducible one were peculiar properties of T cells in the early developmental period of life. The T cell subset in cord blood defined by OKT4 monoclonal antisera was much more suppressive on B cell differentiation than did another OKT8+ subset, suggesting functional aberration of cord T cell subsets from corresponding adult ones.
Primary Immunodeficiency Diseases in the Neonates—Analysis of the Registered Cases in Japan
Authors: Hayakawa Hiroshi 
Published: October 2007

AbstractFive hundred and forty three cases were collected in a nation‐wide survey and registration of the primary immunodeficiency diseases in Japan.Among them nineteen cases (3.5%) were diagnosed during the first omit month of life, including ten cases of thymic hypoplasia, four of severe combined immunodeficiency, two of Wiskott‐Aldrich syndrome, one of chronic granulomatous disease and two of Chediak‐Higashi disease.Thymic hypoplasia was diagnosed in the neonatal period so frequently that among all 26 reported cases ten (38.5%) the diagnosis was made by one month of age from characteristic complications such as tetany and congenital anomaly of the heart and great vessels.
IgG Subclass Defficiency and Its Relation to IgA Deficiency
Authors: Oxelius Vivi‐Anne , Lindquist Bertil 
Published: October 2007

AbstractChildren may suffer from deficiency of one or more of the IgG subclasses‐ although the total level of IgG may be normal‐resulting in recurrent bacterial infections. Subclass deficiency may also be seen in some other well‐defined immuno deficiency states, i. a. selective IgA deficiency. Thus, IgG2 deficiency was found in 5 of 22 such children with symptoms of various diseases. Normal IgG2 levels were found in sera from 11 healthy individuals with selective IgA deficiency. IgGl levels of the whole IgA deficient group were significantly increased as compared to a normal reference group.
Role of the Biliary slgA in the Gastointestinal Tract and the Effect of Malnutrition on the Biliary slgA in the Rat
Authors: Oka T. , Hosoya N. 
Published: October 2007

AbstractThe secretory IgA (slgA) was purified from rat bile, and the role of slgA in the intestine and the effect of protein restriction on biliary slgA were investigated in the rat. The purified biliary slgA contained s‐chain and L‐chain and secretory component (SC), and was about 400,000 of molecular weight, about 11.7S. Biliary slgA itself was not digested by trypsin and intestinal fluid, though monomeric IgA (mlgA) was digested. By the challenge of DNP‐BSA, specific antibody against DNP‐BSA was secreted into bile and excreted into feces. Only DNP‐BSA alone was hydrolyzed by trypsin in the presence of biliary slgA containing specific DNP‐BSA antibody. When rats were raised on a diet restricted in protein the concentration of IgA, particularly slgA, in bile and the biliary immune response decreased markedly. These results suggest that biliary slgA involves an important function in the defense of intestinal absorption of foreign antigens such as organisms of infectious diseases and macromolecules in foods, and that the production or the secretion of biliary slgA is sensitively affected by the nutritional situation.
A Case of Complex de Novo Rearrangements Involving Four Chromosomes
Authors: Ushijima Hiroshi , Uehara Mariko , Kida Mitsushiro 
Published: October 2007

AbstractWe described complex de novo rearrangements involving four chromosomes in a child with congenital malformations. Clinical features of this patient resembled those of cri du chat syndrome. Examinations in electroencephalography, electrocardiography, computed tomography and gastrointestinal tract showed abnormalities. The karyotype of the propositus was complex de novo rearrangements involving four chromosomes and deletion in the long arm of chromosome 1.
Effects Oxandrolone Treatment on the Linear Growth and the Predicted Adult Height
Authors: Nogawa Takayuki , Milstein Mona , Morishima Akira 
Published: October 2007

AbstractA total of 21 patients with linear growth retardation were treated with low doses of oxandrolone for a period of 8 to 41 months. Nine of the patinets had chromosomal disorders. The other 12 had no chromosomal abnormalities.The effect of oxandrolone on the ultimate adult height was assessed by changes in the ratio of the height age and the bone age, (δHA/δBA), and by the adult height prediction (AHP). In the non‐chromosomal group, the ultimate height prediction improved in patients whose initial bone age was greater than 8 1/2 years. In the group with chromosomal disorders, no change in ultimate height prediction occured. In Turner's syndrome, the adolescent growth spurt is much less than in normal children. Therefore, the data were interpreted to signify probable beneficial response to therapy. Evidence suggests that low dose oxandrolone treatment does not compromise ultimate height. Since it may induce an acceleration of growth rate on a short term basis, it can help ameliorate psychological problems due to poor body image in these short patients.
Galactosialidosis (β‐Galactosidase ‐ Neuraminidase Deficiency): A New Hereditary Metabolic Disease with Abnormal Degradation of Enzyme Molecules
Authors: Suzuki Yoshiyuki , Sakuraba Hitoshi , Yamanaka Tatsuhiro , Ko You‐Min , Okamura Yuko 
Published: October 2007

AbstractGalactosialidosis is a recently established heredodegenerative disease occurring in adults, characterized by cerebellar ataxia, myoclonus, cherry‐red spots, angiokeratoma and deficiencies of two lysosomal enzymes, β‐galactosidase and neuraminidase. Results of recent investigations are briefly reviewed and the molecular basis of this new disease is discussed.
Growth Hormone and Adrenocorticotropic Hormone Levels Increased in a Child with Diencephalic Syndrome
Authors: Hisano S. , Kono H. , Kurokawa T. , Goya N. , Take H. 
Published: October 2007

AbstractA Japanese male child with diencephalic syndrome due to suprasellar tumor was treated in our department. There were no symptoms suggestive of hyper‐adrenalism despite an increased adrenocorticotropic hormone (ACTH), nor was there an excessive growth even in the presence of high fasting levels of growth hormone (GH). After radiation therapy, both ACTH and GH levels reverted to normal. Thus, the abnormal hypothalamic control following destruction of a certain area of the diencephalon may have led to increases in the levels of ACTH and GH. This may be the first documentation of increased ACTH levels in a patient with diencephalic syndrome.
Epidemiological Features of Kawasaki Disease in Japan
Authors: Yanagawa Hiroshi , Shigematsu Itsuzo 
Published: October 2007

AbstractThe epidemiological features of Kawasaki Disease deduced from nation‐wide epidemiological surveys and other epidemiological studies conducted so far in Japan are summarized as follows: (1) The total number of cases observed in 7 nation‐wide surveys up to June 1982 were 47,300 (27,600 males and 19,700 females, M/F=1.4), of which 276 (0.7%) were fatal, (2) Unusually high incidences of the disease were recognized in early or late spring in 1979 and 1982 throughout Japan. A shift of the epidemic wave from warm to cool geographical areas was observed in 1979, but not in 1982. (3) The incidence was highest in Shikoku and surrounding prefectures in 1979, whereas it was highest in the central part of Japan in 1982. (4) The sex ratio tended to be smaller and the age of incidence tended to be younger in epidemic years. (5) A marked temporal clustering of the cases was seen in both epidemic years, 1979 and 1982. (6) Two percent of the cases reported in1981–82 were siblings. The time interval between the onset of disease in siblings was one week or less in most cases. (7) Close geographical and social relations between cases were demonstrated in a field investigation of an outbreak of 9 cases within a month on a small island located about 2,000 km south‐west of Tokyo. (8) A table summarizing the descriptive epidemiology of the disease is given.
Role of Lipoprotein Metabolism in Kawasaki Disease (Acute Febrile Mucocutaneous Lymph Node Syndrome)
Authors: Okada Tomoo , Harada Kensuke , Okuni Masahiko 
Published: October 2007

AbstractThe role of inflammatory disease int he formation of arteriosclerosis has been the subject of discussion for many years. We considered that the arterioscleriotic changes in Kawasaki disease might be a model of arteriosclerosis resulting from acute inflammation. And judging from observation of the changes in serum cholestrol values and high density lipoprotein cholestrol values of patients with Kawasaki disease, which were ontained during the period from the acute stage to the convalescent stage and also during the first few years after the onset, we estimated that the acceleration of vascular permeability and the lipoprotein cholestrol changes which had been caused from the systemic vasculitis with endotherial cell injury.
Lymphocytes with receptor for Peanut Agglutin in Wiskott‐Aldrich syndrome
Authors: Mizutani Shuki , Sei Akuhi , Iwata Tsutomu , Akagi Kunihiko , Kaneshi Kunio , Bessho Fumio , Kobayashi Noboru 
Published: October 2007

AbstractThe Wiskott‐Aldrich syndrome is a sex‐linked recessive disorder characterized by having defective T‐cell function and poor antibody response.We have investigated the percentage of lymphocytes with receptor for Peanut Agglutinin in four children of Wiskott‐Aldrich syndrome. We discovered an increase in percentage of the lymphocytes with receptors for Peanut Agglutinin in all Wiskott‐Aldrich syndrome except one. Our findings suggested that the increase in percentage of Peanut Agglutinin positive cells in Wiskott‐Aldrich syndrome may reflect degree of immune deficient state, and one of primary or secondary abnormalities of this immunological disorder.
Taurine Concentration in the Perinatal Period
Authors: Zaima Koji 
Published: October 2007

AbstractA study was made on the concentration of taurine in the cord blood, maternal serum, breast milk, urinary excretion and plasme of the same mothers and newborns. The results showed that the taurine content in maternal serum taken within 24 ours of delivery was 117.3 n mol/ml and that of cord blood was 303.5 n mol/ml giving a ratio of 1:2.6. The taurine in breast milk on day 1 and 2 was low but there was a daily increase and on teh 7th day its average became 381.8 n mol/ml.The urinary excretion of taurine in teh newborn was 6,222 n mol/ml before the initial feeding on the 1st day. A gradual decrease was noted and on the 5th day it was 1/15 of tat of the first day.Plasma taurine of the newborn was 186.7 n mol/ml on the first day decreasing to 85.2 n mol/ml on the 5th day.There was a lesser tendency for taurine in the plasma and urine to decrease in the breast fed as compared with those on formulas.
Angiographical Studies on the Distribution of Coronary Arteries in Children | Part 2. Caliber and Distribution of Coronary Arteries in Congenital Heart Disease
Authors: Ino Toshihiro 
Published: October 2007

AbstractIn order to determine the normal size of various branches of the coronary arteries and to clarify the etiological factors that determine the dominant patterns of coronary arteries (dominance) and the relationship between the growth of the coronary arteries and the ventricles, the caliber and the distribution of major coronary arterial branches were measured by selective coronary arteriography in 41 cases with normal hearts and 49 cases with congential heart disease. In normal hearts, the incidences of right dominant(R), balanced(B) and left dominant(L) types were 78%, 15% adn 7%, respectively. In congenital heart disease, the incidences of R, B and L types in right ventricular loading hearts (RV group) were 94%, 0% and 6%, respectively and those in left ventricular loading hearts (LV group) were 58%, 27% and 15%, respectively. The ratios of left main trunk and proximal right corornary in size (LMT/RCA ratio) in RV group weremuch smaller and LMT/RCA ratios in RV group were much larger than those in normal hearts. these results indicated that in the different types of heart anomalies the loading of pressure and volume to the ventricles varies in grade, and subsequently induced the hypertrophy of the loaded parts. It was thought that the resultant hypertrophy also affected the growth and dominance of the coronary arteries.
Myelofibrosis Developing in Megakaryoblastic Leukemia in a Boy with Down's Syndrome
Authors: Bessho Fumio , Mizutani Shuki , Kinumaki Hiroshi , Kobayashi Noboru , Hayashi Yasuhide , Urano Norifumi 
Published: October 2007

AbstractA 2 1 /2‐year‐old boy with Down's syndrome developed pancytopenia. After spontaneous recovery, leukocytosis with blast cells in peripheral blood and bone marrow developed, and it was associated with thrombocytopenia and splenomegaly. After transient but complete disappearance of all of these findings, they reappeared and a marked fluctuation with 4 subsequent peaks of leukocytosis was observed. Splenomegaly appeared in association with eukocytosis in early phase and persisted after the third peak.Blast cells were identified as megakaryoblasts and had a karyotype of 47, XY, ‐7, ‐1 2, +21, +der(7)t(7p+;?)(p22;?), +der(12)t(6;12p+)(pll;ll).Bone marrow biopsy at the time of recovery from pancytopenia showed hypercellularity with many megakaryocytes and minimal fibrosis. Subsequent serial bone marrow biopsies showed progressively developing fibrosis leading to the final picture of typical myelofibrosis. A histological examination of spleen and an omental tissue showed marked blastic infiltration associated with megakaryocytic infiltration.This case suggests that megakaryoblastic leukemia associated with myelofibrosis is anadditional type of abnormal myelopoiesis seen in Down's syndrome.
A Case of Nephrotic Syndrome With Tetany
Authors: Tanaka Toshiaki , Yano Hisako , Lin Kakoh , Nagai Megumi , Akagi Kunihiko , Yoshiwara Shoji 
Published: October 2007

Abstract A 7‐year‐old girl with nephrotic syndrome had several attacks of tetany during the relapses. The attacks, acccompanied by hyperventilation, responded to re‐breathing with a vinyl bag. However, other episodes of attacks which were not accompanied by hyperventilation, did not respond to re‐breathing but responded to the intravenous administration of calcium gluconate. The serum concentrations of total calcium and ionized calcium were low and no calcium‐binding protein was found in the serum from the patient, using chromatographic study. The serum concentration of 1, 25(OH)2 vitamin D was within the normal range, but showed a tendency for the value to be lower in relapse than in remission. Urinary, Ca/Cr ratio in relation to serum calcium was high in this patient and a low threshold of calcium excretion in the kidney was demonstrated.It is suggested that the impaired vitamin D metabolism and calcium loss in the urine might have contributed to tetany in this patient.Administration of a calcium preparation and vitamin D may be necessary for a frequentrelapser on long‐term glucocorticoid therapy.