bluuebunny/crossref_metadata_2025 huggingface dataset:
The Effect of Piglet Expulsion in the Sow on Plasma Cortisol, Adrenocorticotropic Hormone and β‐endorphin
Authors: Jarvis S. , Lawrence A.B. , McLean K.A. , Deans L.A. , Chirnside J. , Calvert S.K.
Published: October 2007

Contents

Previous studies have shown an increase in plasma cortisol in gilts over farrowing irrespective of environment suggesting that factor(s) associated with parturition itself cause physiological stress. Factors involved in mediating the hypathalomo–pituitary–adrenal (HPA) axis at parturition are not well understood. This study examines the effect of piglet expulsion on the pituitary–adrenal axis by measurement of plasma cortisol, adrenocorticotropic hormone (ACTH) and β‐endorphin. The effect of farrowing environment in modulating the acute response to piglet expulsion is also investigated. Twelve second parity sows, with indwelling jugular catheters, were moved into either a farrowing crate or a straw‐bedded pen 5 days before their expected parturition date (EPD). Blood samples were taken from each sow during a prefarrowing baseline period and then rapid samples (2.5 mins) were taken for 10 min following the birth of two piglets. No effect of environment was found on any of the hormonal variables which reinforces the hypothesis that the physiological stress seen in parturient pigs is due to some intrinsic factor of parturition. Plasma cortisol, ACTH and β‐endorphin did not change significantly in the period following piglet expulsion suggesting that individual piglet expulsions do not play a major role in ‘parturition stress’. There was however, an increase in plasma cortisol, ACTH and β‐endorphin in response to increasing piglet number which is consistent with previous studies of general farrowing in which cortisol increased as farrowing progressed. Therefore this study reinforces the hypothesis that physiological stress increases with ongoing parturition although this does not appear to be a result of piglet expulsion. The potential role of other factors which may be involved in causing ‘parturition stress’ should be investigated.
Demonstration of 16‐Unsaturated C‐19 Steroids (‘Boar Pheromones’) in Tissues of the Male Camel (Camelus dromedarius)
Authors: Claus R. , Kaufmann B. , Dehnhard M. , Spitzer V.
Published: October 2007

Contents

Investigations into the role of pheromones in reproductive behaviour have been mainly limited to the boar, with 16‐unsaturated C‐19 steroids playing a major role. Similarities in the mating behaviour of the camel and pig suggest that pheromones may also be important in the camel. The aim of this study was to determine the concentrations of 16‐unsaturated steroids in camel testes and other tissues. Samples of testicular tissue were obtained by castration from six male camels (9–15 years) and 16‐unsaturated steroid content was measured by gas–liquid chromatography (GLC). In addition, samples of the glandula occipitalis (‘poll gland’), g. parotis, g. submandibularis and the palatal flap (‘dulaa’) were obtained from a 10‐year‐old camel and also analysed for steroid content. Results were confirmed by GC‐MS (gas‐chromatography‐mass spectrometry) in selected testicular samples. 5α‐androst‐16‐en‐3‐one could be detected in all testicular specimen (range 0.05–1.28 μg/g tissue) as well as in all the other tissues analysed, with highest concentrations in the glandula occipitalis. 5α‐androst‐16‐en‐3β‐ol was present in most tissues, whereas 5α‐androst‐16‐en‐3α‐ol was detectable at 0.75 μg/g only in one testes which also had the highest level of 5α‐androst‐16‐en‐3‐one. The similarities of these findings to the porcine species suggest a role of 16‐unsaturated steroids as pheromones in the camel. Further experimental evidence however, is required to confirm these findings.
Enzymatic Development of the Human Fetus: LDH/Aldolase Ratio in Developing Human Fetus
Authors: Mino Makoto , Takai Toshio
Published: October 2007

Studies on fructose diphosphate aldolase during development of the human fetus were undertaken. Additionally, the relation of the activity of LDH and its isozymes to aldolase in developing tissues including the liver, skeletal muscle, heart and brain were observed.

In the liver, the activity of aldolase was found to be low throughout pre‐ and postnatal life, while in the skeletal muscle, it was found to increase throughout life. In the heart, aldolase activity was relatively high during 4 to 6 months of gestation, and decreased during the remaining fetal period. No remarkable changes were observed during post‐natal development.

The total LDH/aldolase activity ratio was high in the adult liver, while that of the adult muscle was low. During early fetal life these ratios were similar between liver and muscle. With respect to the development of these tissues, the ratio increased in liver and decreased in the muscle. From the finding of changes in this ratio, a critical period in the metabolism during development may be recognized. Such critical periods appeared at the 5th month of gestation in the liver and muscle. Further small changes in the ratio were found to occur only in the muscle at term.

During development of the brain, the ratio of total LDH/aldolase decreased, while it increased in the developing heart. Critical periods appear at the 5th month of gestation in the brain and at terminal gestation in the heart.

Similar changes were observed in the activity ratios of main component of LDH isozymes to aldolase in all tissues examined.
Studies on the Lactic Dehydrogenase (LDH) during Development
Authors: Matsuoka Satoru
Published: October 2007

The serum LDH activity and the isozyme patterns in cord blood and the healthy individuals in different age were investigated during the development and the following results were obtained.

1) LDH activity

The mean values in each group were as follows: 490.30 u/ml in cord blood, 623.60 u/ml in early newborns (1–3 days), 575.45 u/ml in newborns (4–7 days), 395.00 u/ml in infants (1–6 m), 371.00 u/ml in infants (6–12 m), 342.00 u/ml in young children (1–6 y), 298.24 u/ml in school children (6–15 y) and 212.00 u/ml in adults.

Marked elevations of the activity were proved in the newborn group, especially 1–3 days after birth.

After the newborn period, the activity gradually decreased to the adult level.

No significant differences were obtained between the male and the female in each group.

2) LDH isozyme

The distribution dominance of the LDH isozymes in all groups were indicated in the following order: LDH‐2>LDH‐1>LDH‐3>LDH‐4>LDH‐5.

The LDH isozyme pattern in cord blood showed the specific pattern considerably different from those in other groups.

The LDH isozyme patterns in newborn serum showed the statistically significant increase of LDH‐3, LDH‐4 and LDH‐5 as compared with those in other age groups and the alteration was marked in the early period of the newborn, 1–3 days after birth.

3) The possible mechanisms of the changes of the serum LDH activity and the alterations of the isozyme patterns were discussed.
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

The 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.
Hyperglycemic Response to Epinephrine in Asthmatic Children Part I: Hyperglycemic Response to Epinephrine in Asthmatic Children with Varying Severity and at Attacks
Authors: Yamashiro Yuichiro
Published: October 2007

In 1948 Ahlquest initially proposed the existence of alpha and beta receptors in smooth muscle to explain the different effects of various adrenergic drugs.1) The bronchi of patients with bronchial asthma are remarkably sensitive to various stimuli, for example, to pharmacological mediators of anaphylaxis, e.g. histamine, serotonine, acetylcholine and bradykinin.2)3)4)
Effect of Excess Phenylalanine on Utilization of Glucose in Rat Brain
Authors: Kuroda Yasuhiro , Sumitomo Misako , Miyao Masuhide
Published: October 2007

This 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.
Studies with Lysosomal Enzyme: The Enzymatic Study of Hepatic Phosphorylase Kinase Deficiency
Authors: Ohya Noriaki , Mizukawa Kiminao , Kinugasa Akihiko , Furukawa Noriaki , Sawada Tadashi , Kusunoki Tomoichi , Soyama Kokichi
Published: October 2007

Studies with Lysosomal Enzyme: The Enzymatic Study of Hepatic Phosphorylase Kinase Deficiency
Epidemiology of Kawasaki Disease in Japan
Authors: Yanagawa Hiroshi , Shigematsu Itsuzo , Kusakawa Sanji , Kawasaki Tomisaku
Published: October 2007

Three 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

This 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

In 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

For 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

As 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

Five 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

Children 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.
Functional Analyses of Cord IgM: Cytotoxic Antibody Against Common Killer T Cells
Authors: Miyagawa Yukiaki , Komiyama Atsushi , Akabane Taro
Published: October 2007

We have analysed cytotoxic antibody against T lymphocytes (TLFA) in cord IgM. TLFA IgM bound to T lymphocytes as determined by the radio immuno assay. TLFA IgM inhibited lymphocyte blastogenesis induced by Con A and PHA but did not inhibit that induced by PWM in the presence of complement. Based on these results, further studies were done to determine which function(S) of Con A‐ and PHA‐induced lymphocytes could be inhibited by TLFA IgM. TLFA IgM inhibited Con A‐induced T cell activity to suppress PWM‐induced antibody formation and autologous mixed lymphocyte culture. In addition, TLFA IgM markedly inhibited PHA‐mitogen factor induced killer T cell colony formation and their activity as determined by the colony assay and lymphocyte‐mediated cytolysis using K562 and Daudi cell lines respectively. TLFA appears to play a role in protecting a fetus from immunologic rejection by the mother, by killing maternal killer cells which may trasfer across the placenta and harm the fetal tissues.
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

The 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

We 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

A 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

Galactosialidosis 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

A 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

The 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

The 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

The 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

A 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

In 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

A 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.
Association of Hirschsprung Disease and Bilateral Preaxial Polydactyly
Authors: Madokoro Harumichi , Ohdo Shozo , Sonoda Tohru , Hayakawa Kunio
Published: October 2007

Two cases with association of Hirschsprung disease and preaxial polydactyly occurring in siblings were reported.

The proband was a female infant examined on the 12th day after birth. Major findings were as follows: growth retardation, mild microcephaly, rightanophthalmia, left exophthalmus associated with double disk and coloboma choroidae, ocular hypotelorism, cleft lip and palate, and bilateral preaxial polydactyly. CT scan of the brain revealed agenesis of the corpus callosum and atrophy of the cerebral cortex.

Autopsy revealed alobar holoprosencephaly and agenesis of the corpus callosum, right optic nerve and olfactory nerve. Aganglionosis of the rectumwas noted at a site 10 cm from the anus.

When her brother was born, bilateral preaxial polydactyly was found. He had Hirschsprung disease as well.
A Comparative Study on the Epidemiology of IDDM Between Japan, Norway, Israel and the United States
Authors: Kitagawa Teruo , Fujita Hidehiro , Hibi Itsuro , Aagenaes ØYstein , Laron Zvi , Laporte Ronald E. , Tajima Noriko , Drash Allan L.
Published: October 2007

Japn, Norway, Israel and the United States have studied have studied the epidemiology of IDDM in children by a similar method of data collection. Afterwards, the data were compared. Available data demonstrated that children in Norway, the United States and Israel were almost 20,15 and 5 times more likely respectively to develop IDDM than children in Japan. Although there was a samll difference in sex ratio, incidence in the smaller age group, and incidence of slowly progressing type IDDM between Japan and the other three countries, the other descriptive epidemiology of IDDM was quite similar, despite the large difference in the incidence and prevalence of IDDM.

Therefore, it seems that the difference in the incidence would not be primarily environmental and could be related to the number of individuals susceptible to diabetes. It was hypothesized that the difference in incidence may be linked to the difference in prevalence of HLA type among the children in those four countries. However, it cannot be fully explained nby HLA difference. Other genetic markers associated with the developing IDDM musrt be evaluated.
Pittsburgh Diabetes Mellitus Study: Studies on the Etiology of Insulin‐Dependent Diabetes Mellitus with Special Reference to Viral Infections
Authors: Drash Allan , Cavender Druie , Atchison Robert , Becker Dorothy , Eberhardt Mark , Laporte Ronald , Kuller Lewis , Orchard Trevor , Rabin Bruce , Wagener Diane
Published: October 2007

The etiology of IDDM remains incompletely understood, with genetic predisposition, autoimmune destruction of the beta cells and viral infections interacting to produce disease. It appear that the underlying genetic defect is an alteration in cellular and/or humorally‐mediated immunological responsiveness. There are at least two (and probably more) genetically determined defects identified by HLA B‐8 (DR3) and B‐ 15 (DR4). A number of viruses have been implicated in IDDM causation, including Coxsackie, mumps, rubella, Epstein‐Barr, hepatitis and influenza viruses, but in only a few cases is viral etiology fully documented. Viral infections may trigger autoimmunity. Alterations in immunoglobulin concentration, particularly IgA deficiency, have been reported by several investigators. Collaborative prospective studies of individuals at high risk for the development of IDDM (siblings with HLA identical to the index case) are essential in the future study of IDDM etiology.
The Role of Excercise in the Metabolic Control of Juvenile Diabetes
Authors: Larsson Yngve
Published: October 2007

Exercise has a beneficial metabolic effect in diabetic children and adolescents only when they are in good metabolic control with optimal insulin administration. In insulin‐deficient patients exercise may seriously aggravate the diabetic state. The physical working capacity of young diabetics is often lower than that of non‐diabetic children. Their physical fitness can be improved by regular training programs.

Exercise‐induced hypoglycemia is due to suppression of hepatic glucose output in response to an abnormal insulinemia. It can as a rule be prevented by a liberal carbohydrate intake before and during the exercise period. On a long‐term basis regular physical activity improves diabetic control in young diabetics, increases glucose tolerance and normalizes blood lipids. Regular physical activity increases diabetics' sense of well‐being, gives self‐confidence and makes feelings of being handicapped disappear. Exercise may contribute topreventing the development of long‐term diabetic microangiopathy andneuropathy. However, when such complications once have become manifest, the value of exercise is less obvious. It may even be harmful and should therefore, in such situations, be undertaken with great caution and after careful medical evaluation.
Indications for Plasma Exchange and Other Immunosuppressive Theraphy at the Onset of IDDM
Authors: Ludvigsson Johnny
Published: October 2007

Abstract>

Several facts indicate that immunological mechanisms are involved in the etiology of insulin dependent diabetes mellitus. As there usually is an important residual beta cell function at the time of diagnosis, the destructive process should be stopped. Different kinds of immunosuppressive therapy have been tried. A review is made concluding that especially Cyclosporin A‐treatment has given promising preliminary short‐term results. However, the side effects cannot be neglected and the follow‐up is short. It is therefore recommended that the results of ongoing studies are reported before new studies are started. Plasmapheresis has also given some positive results. Both the removal of plasma from the patients and supply of plasma from healthy donors may contribute to the effect. Further studies are needed before any recommendations for clinical use can be given.
Theoretical and Practical Application of Non‐Enzymatic Glycosylationin Diabetes
Authors: Greenberg Robert E.
Published: October 2007

During heating or prolonged storage of food, a brown color results, in the presence of reducing sugars. This browning of foods (i.e. Maillard reaction) results from a direct non‐enzymatic reaction between free amino groups in proteins and reducing sugars, and results in a decrease in nutritional availability of lysine. Non‐enzymatic glycosylation occurs with proteins in vivo, is increased in diabetic subjects, and is dependent on concentrations of both proteins and sugars, turnover rates of proteins and accessibility of proteins to sugars. Functional changes have been demonstrated to occur in an increasing variety of proteins as a consequence of non‐enzymatic glycosylation. Thus, aberrant function has been suggested with serum albumin, neural tubulin, collagen, low density lipoproteins, fibrin, lens crystallins following glucosylation.
Complications of Type‐1 Diabetes Mellitus in Japanese Children—A Nationwide Study
Authors: Hibi Isturo , Tanae Ayako , Kitagawa Teruo , Isshiki Gen , Tsuchiya Hiroshi , Matsuura Nobuo , Maruyama Hiroshi
Published: October 2007

In a nationwide cross‐sectional study of Japanese type‐1 diabetics, whose disease had manifested itself before the age of 18, we found (1) the long‐term outcome of childhood diabetes to be very poor, (2) the prevalence of microvascular complications to be stronly related to the age of the patients and the duration of diabetes, and (3) the prevalence of retinopathy to be dependent on the degree of diabetic control. Of 90 diabetics born before 1955, 7 had died, 71 had retinopathy of which 36 were proliferative, 47 had cataracts, 12 were blind, 44 had nephropathy, 42 had neuropathy, and 18 had hypertension. Retinopathy was present in 1.8% under the age of 12, in 8.3% from the age of 13 to 16, in 22.1% between the age of 17 to 20, and in 52.5% over the age of 20. A comparison between age‐matched subgroups with and without retinopathy showed that the percentages of patients who were cooperative, with good control and without hyperlipidemia were higher in the group having no retinopathy than in the other.
Analysis of Social‐Professional Outocome of Insulin‐Dependent Diabetic Patients
Authors: Francois Rene , Asenjo‐Ramis Sylvia , Kammerman Rrancois , Gillet Peirre , Kressman Jean
Published: October 2007

Among our 1,000 diabetic patients observed between 195 1 and 1983, we followed up 394 diabetic patients, whose disease started before 18 years of age and who at time of study were older than 18 years. Their social‐professional status, classified into six categories, was compared with that of the active population of the Rh6ne‐Alpes region (France). The CHI 2 statistical test was used.

Of the diabetic population, 41.9% are in the highest social‐professional groups (4, 5, 6) against 26%in the reference population; which is a highly significant difference (p < O.OOl), independently of the age at onset of IDDM. The comparison of the social‐professional status of the diabetic population of masculine sex with that of their father and nondiabetic brothers shows a greater number of diabetic persons in the highest categories: 49.75% against 39.63% and 49.75% against 41.95% respectively.

There is a special attraction for medical and para‐medical professions among diabetic people.

Finally there is no more absenteeism among the diabetic population than among the reference population but the unemployed percentage is discreetly higher.
The Effects of Anti‐Thymus Serum on Embryonic Development in the Rat
Authors: Miyata Koichiro , Jitosho Tamotsu , Tachikura Junko , Haraguchi Tsutomu , Suenaga Shinji
Published: October 2007

Pregnant Wistar rats were injected with rabbit anti‐rat‐thymus serum (ATS) alone or in combination with rabbit anti‐rat‐heart serum (AHS). The results showed that the percentage of malformation was low (5.7 to 7.5%) when doses over 0.6 ml/100 g of ATS were administered; resorption was 23.2 to 28.4%. However, the incidence of malformations markedly increased to 28.9% when a dose (0.9 ml/100 g) of ATS was given on day 9 of gestation followed by 0.9 ml/100 g of AHS on day 10. The resorption and cardiovascular malformations were 39.7% and 10.5%, respectively. These results suggest that ATS potentiates teratogenicity in combination with AHS, though hypoplasia of thymus was not observed.
Hypermethioninemia Associated with Hepatic Methionine Adenosyltransferase Deficiency: Report of Two Cases
Authors: Hase Yutaka , Sawada Yoshitomo , Tsuruhara Tsuneo , Kobayashi Yoji , Ohtake Harumi , Miyagi Tomiko , Oura Toshiaki , Suzuki Yoshimi , Wada Toshiro
Published: October 2007

In two patients with persistent hypermethioninemia who were detected in a neonatal mass‐screening program, hepatic methionine adenosyltransferase activity was found to be low, at 22.7% and 8.3% of the normal child control value. They did not have homocystinuria, cystathioninuria, liver dysfunction or myopathy. Electron microscopy of liver specimens showed large and small irregular‐shaped mitochondria, frequent giant mitochondria, increased smooth endoplasmic reticulum and decreased rough endoplasmic reticulum. Their motor and mental development were normal at the ages of 7 and 5 years respectively.
Metabolism: Galactosemia (Transferase Deficiency)
Authors: Donnell George N.
Published: October 2007

Galactose, a hexose Sugar, is a Constituent of many foodstuffs. It is present in most animals milks and represents a major source of calories for infants. Galactose provides a sources of energy through its converstion to glucose‐1‐containing compounds in the body.

Inherited deficiency states have been described for all steps in the major pathway of galactose metabolism. Galatose‐1‐phosphate uridyl transferase (transferse) deficiency is the most commonly encontered. It has an incidence of approximately 1:60,000 and it is inherited as an autosomal recessive trait. The clinical consequence of the biochemical defecst appears early in life and usually is severe. Vomiting, diarrhea, jundice, hepatomegaly, renal tublar abnormalities, cataracts and mental retardation are some of the majour clinical features. Untreated, mortality rate is high.

Treatment is readily accomplished and consists of elimination of galactose(lactose) from the diet. The acute manifestation are reversed; cataracts do not grogress and severs mental retardation prvented. The outcom, however, is not wholly satisfactory, even when treatment is intiated early. Growth and development usually are normal, but intellectual devlopment variable. Many children have visual‐perceptual problem and I.Q.'s below average. School performance generally has been unsatisfactory.

Ovarian failure frequently has been found in female galactosemics. The casuse has not been determined.
Metabolism: Positron Emission Tomography in the Measurement of Regional Cerebral Glucose Metabolism in Newborn Infants
Authors: Sinclair J.C. , Garnett S. , Nahmias C. , Firnau G. , Kenyon D.B. , Nobbs P.T. , Doyle L.W.
Published: October 2007

The new diagnostic technique, positron emission tomography with 18F‐DG, was used to obtain qualitative estimates of regional cerebral glucose metabolism in newborn infants with perinatal asphyxia, cerebral hemorrhage or focal motor seizures. Glucose metabolism of gray and white matter structures in the brain could be differentiated clearly. Decreased glucose metabolism was identified in regions of the brain shown by CT scan to be structurally abnormal. A localized increase in glucose metabolism was demonstrated in the contralateral cerebral hemisphere of a newborn with focal motor seizures. Positron emission tomography is a promising new diagnostic tool for study of newborn infants with supected abnormalities of brain function.
Increased Serum Levels of Anti‐Mumps and Rubella Viruses Antibodies in Children with Kawasaki Disease
Authors: Kuno‐Sakai Harumi
Published: October 2007

Serum IgM and IgG antibodies against mumps virus and rubella virus were quantitated serially by enzyme‐linked immunosorbent assays in 15 children with Kawasaki disease. IgM antibody against mumps virus and IgG antibody against rubella virus were increased significantly in children with Kawasaki disease who had no previous history of mumps virus or rubella virus infection. Booster‐like effect in IgG antibody levels against mumps virus was observed in children with Kawasaki disease who had a previous history of exposure to mumps virus. Since none of the patients manifested clinical symptoms of mumps or rubella, the increased antibody levels against mumps and rubella may represent a part of the nonspecific increase in serum immunoglobulins in children with Kawasaki disease.