bluuebunny/crossref_metadata_2025 huggingface dataset:
Effects of Endocrine Disrupting Compounds in Animal Feed on Reproductive Health in Farm Animals*Euroconference Wageningen, The Netherlands, 20–22nd November 1998
Authors: Boerjan M.L. , Fischer B.
Published: October 2007

This Euroconference was a follow‐up to the Euroconference held in Halle, April 1997. The programme of the conference in Halle had an exploratory character with respect to accumulation of endocrine disrupting compounds (EDCs) in the environment and in animal tissues and their effects on animal physiology and reproduction. Many questions arose from the first Euroconference, such as: What are biologically meaningful levels of environmental contamination? Is there a risk for farm animals’ health and reproduction? What assays are available and which are the most appropriate to be used? These questions formed the basis for the programme of the second Euroconference held in Wageningen in November 1998.

Taking these questions into consideration, the programme of the Wageningen Euroconference was divided into four main topics. In the first session an overview was given on sources and levels of EDCs in the environment. The second session addressed chemical nature and metabolism of EDCs including phytoestrogens. In the third session the effects of EDCs on ovarian function and embryo development were discussed and in the last session the advantages and disadvantages of different in vivo as well as in vitro assays were debated.
Changes in Lipid Classes and Fatty Acid Compositions in Purified Myelin of EAE Guinea Pig Brain
Authors: Iwaki Nobuhiro
Published: October 2007

Purified myelin fractions were prepared from brains of control and EAE sensitized guinea pigs on the 5th, 9th and 15th day after injection of antigen. Lipid classes were quantified and expressed as per cent of micromol contents of phospholipids and galactolipids, and fatty acid compositions each of them were analyzed.

Cerebroside was markedly reduced with progress of the day, keeping Sulf/Osd ratio in normal constant (0.31–0.36). This was a characteristic finding in this study. Phosphatidyl ethanolamine, ethanolamine plasmalogen and sphingomyelin were also decreasing when compared among phospholipids whereas phosphatidyl choline was increasing. Fatty acid composition showed little change.

Anionic lipids like sulfatide are said to play an important role in binding non‐ionic and zwitterionic lipids tightly to the myelin protein. From many reports on electron microscopic observations, it could be considered that the mode of insults to the myelin “lipid” would be rather secondary in demyelination process.

The significance of the findings in this study and informations above mentioned were discussed, and a mechanism of demyelination was speculated. In brief, some factors might cause the ionic derangement in the basic amino group of myelin protein, the target of immune reaction, and anionic lipids might therefore be separated from it, being accompanied by such other lipids as cerebroside.
Studies Alkaline Phosphatase during Development Part II: Changes in alkaline phosphatase activity and isozyme patterns in the serum of healthy children and of children with various diseases
Authors: Mimura Tomohiko
Published: October 2007

In order to clarify the organ origins of high serum alkaline phosphatase in children during development and in children with various diseases, the serum alkaline phosphatase activity and isozyme patterns of children, umbilical cord blood and pregnant woman were investigated by the agar gel electrophoresis and heat stability test. In two infant autopsy cases, isozyme patterns were also investigated in several organs.

1) Serum alkaline phosphatase activity of children was about 2.5 to 3 times as high as that of adults. In the group below 1 year of age and the group between 11 and 15 years of age, the levels were higher than in other groups.

2) Alkaline phosphatase isozyme pattern in normal serum was showed as a single band (band III) around the α2‐globulin fraction in all groups.

3) The alkaline phosphatase isozyme patterns in normal serum were divided into a pattern with non‐shifting peak after heat treatment at 56°c for 10 minutes and another with shifting peak to α1‐globulin side. In younger groups, the pattern with non‐shifting peak was more frequently seen and with growing the pattern with shifting peak increased. In adults, the peak shifted in all cases after heat treatment.

4) The decreasing rate of alkaline phosphatase activity of normal serum by heat treatment was within the range of about 70–80% in each age group without remarkable differences. No calculation was possible in adult group, because the activity was markedly low.

5) Serum alkaline phosphatase activity of pregnant women showed slightly higher than umbilical cord blood, about 3 times higher than in normal adults.

The isozyme patterns were different between sera of pregnant women and umbilical cord blood.

The decreasing rate of alkaline phosphatase activity after heat treatment (56°c for 10 minutes) was less in serum of pregnant women than in other sera, showing greater heat stability.

6) Serum alkaline phosphatase was always high in diseases of liver and bone.

Five bands of alkaline phosphatase activity were noted in the sera of normal subjects, pregnant women and children with various diseases.

In liver diseases without marked obstruction and in bone diseases, the alkaline phosphatase isozyme pattern appeared in α2‐globulin band (band III). In liver diseases with marked obstruction, α1‐globulin band (band II originated from the liver) and β‐globulin band (band V probably from intestine) appeared. In one case, an Albumin‐α1 globulin band (band I) appeared.

7) The alkaline phosphatase isozyme patterns in liver, bone, small intestine and kidney showed different electrophoretic migration rates indicating organ specificity.

8) To sum up, serum alkaline phosphatase of the healthy children appeared to be originated from liver and bone. During childhood the alkaline phosphatase predominantly consists of bone origin, while in adults, major activity appeared to be hepatic origin.
Mucocutaneous lymph node syndrome presenting as acute cholecystitis
Authors: Tanaka Kazuhiko , Onouchi Zenshiro , Fushiki Shinji , Tomisawa Munehiko , Kusunoki Tomoichi
Published: October 2007

Mucocutaneous lymph node syndrome (MLNS) is not uncommon in Japan and it is well recognized that the dominant lesion is located in the coronary artery. We experienced recently a particular case which was diagnosed at first as acute cholecystitis, but a definite diagnosis of MLNS was later made from clinical manifestations and laboratory findings, especially ECG tracings, selective coronary angioram and selective celiac angiogram. We discussed the significant relationship between acute cholecystitis and MLNS.

Mucocutaneous lymph node syndrome (MLNS) is an acute febrile and eruptive disorder described by Kawasaki1)2) for the first time in 1967. According to increasing reports, it has become gradually clear that the dominant lesion is located in the coronary artery and the symptoms result from the generalized vasculitis in small arteries and arterioles. Its etiology, however, remains unknown.

The case, presented here was diagnosed at first as acute cholecystitis and treated with antibiotics, but a definite diagnosis of MLNS was later made by ECG and coronary angiograms.
Clinical Evaluation of the Pathogenesis of Neonatal Asphyxia
Authors: Tada Hiroshi
Published: October 2007

The perinatal mortality rate in Japan decreased from 16.8 per 1000 live‐births in 1964 to 4.5. in 1980, and the early neonatal mortality rate from 8.1 in 1964 to 1.1 in 1980. These decreases are mainly due to the improvement in prognosis of premature infants. The incidence of neonatal asphyxia in mature infants also decreased from 8.8% in 1967–68 to 5.1% in 1979–80. Fetal distress, breech delivery and Caesarean section were the major causes of neonatal asphyxia. Early detection of fetal distress and appropriate care decreased both the incidence of neonatal asphyxia and the neurological signs even in severely asphyxiated infants.
Neonatal Mass Screening of Hereditary Metabolic Diseases in S. Paulo‐Brasil
Authors: Schmidt Benjamin J. , Diament Aron J. , Krynski Stanislau , Kamei Maria E. , Rodrigues Maria M. C. , Takata Sumika
Published: October 2007

The authors report their experience in neonatal mass screening of hereditary metabolic disease in S. Paulo regarding hyperphenylalaninemia (by own programmation), others aminoacidophaties, congenital hypothyroidism, carbohydrates, mucopolysaccharides and heterozygotes for GM2‐gangliosidosis type I (Tay‐Sachs disease). The importance of the performing of such populational screening tests, even in developing countries is stressed.
Japanese Policies and Measures to Prevent Physical and Mental Disabilities
Authors: Fukuwatari Yasushi
Published: October 2007

The prevention of physical and mental disabilities is one of the major objectives of The Maternal and Child Health Services in Japan. The policies and measures to achieve these goals include the following:

1. Health services (preventive and health promotive measures)

2. Medical care services (curative measures)

3. Arrangements to ensure the above mentioned activities

4. Research, evaluation and future policy development

These should be integrated and coordinated so that their maximum effectiveness and efficiency can be brought into full play. The Ministry of Health and Welfare has been, and will continue to be, providing the highest quality services to all Japanese people in order to enrich their lives and prevent disabilities.
A Genetic Study of Hypohidrotic Ectodermal Dysplasia
Authors: Ohdo Shozo , Ikeda Takuya , Hayakawa Kunio
Published: October 2007

The heredity of hypohidrotic ectodermal dysplasia (HED) has been considered to be X‐linked recessive, autosomal recessive or autosomal dominant. In this report, a genetic analysis was carried out on a total of 57 patients with HED including 6 patients recently examined by the present authors and 51 patients reported in Japan before May, 1980.

The morbidity varied depending on sexes, being significantly higher in men than in women (p < 0.001). Consanguinity was positive in 12.8% of the patients. The segregation ratio calculated, however, suggested that an autosomal recessive trait did not account for all the cases. The pedigree analysis and the segregation ratios revealed that HED was probably not autosomal dominant. It is finally pointed out that the heredity of all the patients with HED reported in Japan might be X‐linked recessive.
Tubuloreticular structures and parallel tubular arrays in the lymphocytes of children with aplastic anemia and acute febrile mucocutaneous lymph node syndrome: Electron microscopic observations
Authors: Bessho Fumio , Kobayashi Noboru
Published: October 2007

Tubuloreticular structure was found in 3 and 30% of the lymphoid cells from two consecutive cases of MCLS and in 10 and 15% of the lymphoid cells from two consecutive cases of aplastic anemia. Parallel tubular array was found in 8 and 24% of the lymphoid cells from the cases of MCLS and in 3% of the lymphoid cells from the cases of aplastic anemia. The possibility of the viral etiology of the both diseases was discussed.

eases was discussed.
Angiospermic Changes of Coronary Aneurysms in Kawasaki Disease
Authors: Kawachi Kanji , Kitamura Soichiro , Oyama Choken , Koh Shigeo , Morita Ryuichi , Yamada Yoshiki , Kawashima Yasunaru
Published: October 2007

The fate of coronary aneurysms in Kawasaki disease or MCLS was examined by follow‐up studies on 9 patients with multiple bilateral aneurysms in the coronary arteries demonstrated by coronary arteriography. Their ages at the time if first examination ranged from 0.3 to 11 years (mean, 4.1 years). The mean interval between the onset of acute illness and the initial examination was 15.8 months, and the mean interval between the first and second examination was 17.9 months. Eight of the nine patients received anticoagulants between examinations. The second coronary arteriogram showed morphologic changes of the aneueysm from the time of the first examination: changes were seen in the right coronary artery area in 8 patients (89%) and in the left coronary artery area in 7 patients. These morphologuic changes seemed to be mainly secondary to thrombus formation, calcification, stenosis and/or obstruction of the aneuryms. Thus, coronary aneuryms in Kawasaki disease were transformed despite the use of anticoagulants.

It is considered that multiple large coronary aneurysms will not healed or disappear, but are simply transformed as a result of their thrombosis and/or calcification.
An Immunological Study on Mucocutaneous Lymph Node Syndrome: Activation of Tg Suppressor Cells
Authors: Matsuoka Yoshiko , Yata Junichi
Published: October 2007

We analyzed the lymphocyte function of MCLS in pokeweed mitogen‐induced immunoglobulin production. T cell suppressor activity was activated in the acute phase adn normalized in convalescence. T cells showing suppressor activity in this system were identified to be Tg cells, and they seemed to be activated as suppressor by a certain factor in the serum. the sera of patients whose T cells showed suppressor activity could induce the suppresoor activity of normal control Tg cells. The suppressor inducing factor in sera is likely to be circulating immune complexes, since Tg cells usually show suppressor activity when they interact with immune complexes. The induction of the suppressor activity of Tg cells by immune complexes may provide a kind of negative feedback suppression mechanism feedback mechanism on the antibody formation and it may be related to the self‐limiting characteristics of MCLS. MCLS is different from collagen diseases such as rheumatoid arthritis in that Tg cells do not usually work as suppressor cells in the later diseases.
Correlation between saliva and serum valproic acid concentrations in epileptic children
Authors: Tokugawa Ken , Ueda Kohji
Published: October 2007

Thirty‐five epileptic children (13 boys, 22 girls; aged 5 6/12 years to 17 8/12 years) were studied for their saliva and setrum levels of valproic acid (VPA). No obvious correlation between the VPA saliva and serum levels was observed.
Histamine suppression of the non‐immunological release of slow reacting substances from neutrophils of asthmatics
Authors: Torii Shimpei , Kishimoto Machiko , Katagiri Masanori , Yamada Masanori
Published: October 2007

Slow reacting substance is released from cytochalasin B treated human polymorphonuclear leukocytes by incubation with zymosan and fresh autologous serum.

It is reversed by FPL 55712, a specific endorgan antagonist of SRS‐A and the process is confirmed to involve LTC4, LTD4, LTE4 by High‐Performance Liquid Chromatography.

The levels of SRS from PMN by the non‐immunological stimuli are significantly higher in asthma group than in control group.

SRS is inhibited with pretreatment of theophylline and histamine, and the suppressed release of SRS is recovered by adding cimetidine, H2‐brocker.

Histamine appears to inhibit SRS release through H2‐receptor.

The role of neutrophils in IgE mediated hypersensitivity was discussed.
Comparison of New Sustained‐Release Theophylline (E‐0686) and Theo‐Dur
Authors: Masaki Takuro , Nagakura Toshikazu , Inui Hiroyuki , Umesato Yoshihiro , Obata Toshihiko , Likura Yoji
Published: October 2007

New sustained‐release theophylline (E‐0686) was administered to asthmatic children to study changes in serum theophylline level and its reproducibility. Results are as follows:

t‐Max. of E‐0686 was shorter than that of Theo‐Dur, however, E‐0686 could maintain a satisfactory serum theophylline level at 12 hours on a single dose.

A steady state was reached after three days on a multiple dose of E‐0686. P‐T difference of E‐06 was 5.17 μg/ml, which could be sufficient for sustained‐release theophylline.

Changes in serum theophylline level of E‐0686 was highly reproducible.
A Survey of HBs Antigenmia among Healthy Inhabitants in Kyungpook Province, Korea
Authors: Dong Hak Shin , Jung Ju Kim , Young Choon Park , Jung Ju Yoon , Rac Kyun Ro , Jung Han Park
Published: October 2007

The prevalence of hepatitis B surface antigen (HBs Ag) in various regions of the world is estimated to range from 0.1% to 15%. The area with high prevalence is the west Pacific region. The carrier rate of hepatitis B surface antigen among 1,310 healthy residents in Taegu city, Korea ws investigated in November 1982. Another sample of 729 pregant women and 43 neonates born to HBs Ag‐positive mothers was tested to survey the vertical transmission of HBs Ag between mother and infant from February 1982 to May 1982.

Overall prevalence of HBs Ag was 5.6% in 2,039 residents composed of 1,310 inhabitants and 729 pregnant women. The occurrence of HBs Ag was higher in males than females. The correlation between HBs Ag positivity and the socio‐economic status of samples classified into residence, family numbers per room and education level of fathers indicated little of significance but the difference of the HBs AG carrier rate in the group with a surgical history was significantly higher than the group without a surgical history. Acupuncture showed no influence on the HBs Ag carrier rate.

43 of 729 pregnant womenad HBs Ag in their sera but none of their neonates were carriers of HBs Ag. Follow‐up tests in 35 infants at 3 months of age and in 32 infants at 6 months of age showed seroconversion to HBs Ag positivity in one infant in each test ‐ but it was the same infant in each case. 31 husbands of HBs Ag ‐ positive women and 20 controls whose wives were HBs Ag ‐ negative were examined. Only one in the control group was a carrier of HBs Ag. These data suggest that close person to person contact transmission of HBV via sexual contact and kissing, etc., are the most probable transmitting modes of HBV if one has susceptibility.
Congenital Heart Disease in a Case with Fetal Alcohol Syndrome: Clinical And Postmortem Findings
Authors: Sonada Tohru , Ohdo Shozo , Sennari Eiichi , Madokoro Harumichi , Kunio
Published: October 2007

Clinical and autopsy findings of a male patient with fetal alcohol syndrome, centering on congenital heart disease, are reported.

Major clinical findings included developmental retardation, irritability, short palpebral fissures, thinned upper‐lip vermillion, hypoplastic philtrum, anteverted nostrils, short fifth fingers, palmar transverse creases and congenital heart disease. In addition, autopsy revealed that congenital heart disease was manifested as mitral regugitation, aortic stenosis and pulmonary stenosis
Right Pulmonary Artery Measurements in Term and Preterm Neonates during the Early Newborn Period: Hemodynamic Correlation and Clinical Significance
Authors: Ortiz Edgardo E. , Oguchi Kouki , Misawa Hitoshi , Agata Youtaro , Hiraishi Satoshi , Nishida Hiroshi , Yashiro Kimio
Published: October 2007

Right pulmonary artery (rPa) diameter was measured during the first hour of life, then during the next 23 hours, at 72 hours and at 120 hours in 62 normal term and 14 normal preterm neonates by M‐mode echocardiography via the suprasternal notch. Left atrium (LA) and aortic (Ao) diameters were likewise determined from the standard parasternal view. Comparison was also made with rPA values of 34 patients with patent ductus arteriosus (26), persistent fetal circulation (6) and cyanotic congenital heart disease (2).

In normal term babies, the mean rPA size changed significantly during the 4 periods of examination with diameters of 4.6 mm, 5.5mm, 5.2mm and 4.7 mm respectively. Among preterm babies, mean rPA diameter at 24 hours was 3.8 mm, at 72 hours, 3.8 mm, and at 120 hours, 3.3 mm. unlike those in term babies, the changes in preterm babies were not significant. LA dimensions and LA/Ao ratio were more variable. Furthermore, rPA measurements reliably differentiated clinical conditions with increased or decreased pulmonary blood flow. Thus, our data confirm the significance of rPA measurement as it closely correlates with the hemodynamic changes in the early neonatal period in normal and abnormal term and preterm babies.
Incidence and Etiology of Mental Retardation in Patients with Congenital Heart Disease
Authors: Ohdo Shozo , Madokoro Harumichi , Sonoda Tohru , Matsuoka Yuji , Sennari Eiichi , Okishima Takahiro , Suzumiya Hiroko , Nishiguchi Toshihiro , Hayakawa Kunio
Published: October 2007

Of 1,235 patients (609 male; 626 female) with congenital heart disease for whom types, etiology and associated congenital anomalies were determinable, 129 patients (10.4%) had mental retardatin. Patients with congenital heart disease complicated by mental retardation included 70 (54.3%) with chromosome aberrations, three(2.3%) with single gene disorders, two (1.6%) caused by environmental insult, and two (1.6%) with other recognized syndromes. Among the remaining 52 patients, asphyxia at birt was noted in 16, including 12 complicated by multiple malformations, and 4 in whom mental retardedation was presumed to be due to the asphyxia. In the remaining 36 patients, the incidence of the complication of mental retardation in cyanotic congenital heart disese was significantly higher than that in acyanotic congenital heart disease was signicantly higher than that in acyanotic congenital heart disease. Patients with congenital heart disease of unknown etiology associated with mental retardation included those from two families considered to have new pedigree syndromes.
Leukemic Cell Lysis by Autologous Lymphocytes in Childhood ALL: Generation of Cytolysis by Lymphocytes Activated With Cultured Non T Non B Leukemic Cell Lines
Authors: Yamamoto Masuji
Published: October 2007

The lysis of leukemic cells by autologous lymphocytes was studied in the common type of acute lymphoblastic leukemia (ALL) in childhood using the 10‐hour51Cr release assay. Peripheral blood lymphocytes (PBL) from common ALL patients lysed autologous leukemic cells (% lysis: 19–45%), when co‐cultivated in vitro with cultured non T non B leukemic cell lines (NALM 16, Reh and NALL 1). The effector cells were cytotoxic T lymphocytes (CTL) possessing Leu‐1 and Leu‐2a antigens. The effector CTL were cytotoxic not only for autologous leukemic cells but for cultured non T non B leukemic cell lines, whereas they did not lyse human T cell (CCRF‐CEM), B cell (Daudi) and natural killer cell lines (SPI‐802). Cold target competition assays demonstrated that leukemic cells from childhood common ALL and several non T non B leukemic cell lines shared an antigenic determinant(s) recognized by CTL.
Childhood Leukemia and Lymphoma: Long‐Term Sequelae in Visual, Auditory and Vestibular Function
Authors: Hara Toshiro , Ishii Eiichi , Ueda Kohji , Nishida Yukiaki , Kiyosawa Reiko , Sanui Hiroki , Inomata Hajime , Miyazaki Sumio
Published: October 2007

Twenty‐six children in continuous complete remission from leukemia or lymphoma and no longer receiving chemotherapy were studied to determine the long‐term sequelae in visual, auditory and vestibular function. Ophthalmologic examination revealed cataracts in five patients and an abnormality in the retina in one patient. These abnormalities were not associated with any disturbance of visual acuity. By otorhinolaryngologic examination, slight hearing loss was observed in five patients and an abnoormal righting reflex in one patient. The etiologies of these abnormal findings in visual, auditory and vestibular function were discussed.
Natural History of Hepatitis B Virus Carriers Born to Hepatitis B Virus Carrier Mothers
Authors: Eto Takashi , Shiraki Kazuo
Published: October 2007

Ninety‐five infants born to hepatitis B virus (HBV) carrier women were followed without hepatitis B immune globulin injections over five months. Twenty‐one infants (22%) became HBV carriers. These 21 HBV carrier children were followed and the mean follow‐up period is six years and nine months. Eighteen mothers (85.7%) of these HBV carrier children were HBeAg positive in perinatal period. One was both HBeAg and anti‐HBe negative and the status of the other two was unknown. The mean appearance time of HBsAg is 2.0 ± 1.2 months. Nine carrier children (42.9%) became HBeAg negative in the observation period. In seven cases (33.3%), seroconversion from HBeAg to anti‐HBe was observed. In six of seven seroconverted cases, liver dysfunction was observed from the HBeAg positive phase and the liver function normalized within one year after the appearance of anti‐HBe except in one case. The mean values of AST (Aspartate aminotransferase or SGOT) and ALT (Alanine aminotransferase or SGPT) of the seroconverted group during the whole observation period were significantly higher than those of the persistent HBeAg positive group. The HBeAg positive rate decreases year by year and inversely the anti‐HBe positive rate increases. At 8 years old, the former rate is 55.6%, and the latter rate is 33.3%. The mean annual disappearance rate of HBeAg under eight years is 10.1 ± 5.8% and the mean annual appearance rate of anti‐HBe under eight years is 6.1 ± 5.8%. The higher the mean annual disappearance rate of HBeAg, the lower the positive rate of HBeAg in pregnant women. This may contribute to the decrease in the appearance of new HBV carriers.
Pre‐B Cells and B Lymphocytes in Human Cord Blood and Adult Peripheral Blood
Authors: Okino Fumiko
Published: October 2007

The frequencies of pre‐B cells and B lymphocytes subpopulations were studied in 43 cord blood and 21 adult peripheral blood samples using a direct immunofluorescence double staining method.

We found pre‐B cells in 40 out of 43 cord blood samples studied, a rate of 93%, and in 16 of 21 adult blood samples, a rate of 76%. The mean frequency of pre‐B cells in cord blood lymphocytes was 0.7%, while that in adult blood lymphocytes was 0.2%. The pre‐B cells in both groups were all small pre‐B cells.

The mean frequency of B lymphocytes in cord blood was 11.4%, higher than that in adult blood (5.4%). The difference in B lymphocyte frequency between cord blood and adult blood was exclusively due to high frequency of surface IgM‐positive and IgD‐positive B lymphocytes in the cord blood.
The Study of HLA, ICA and Autoantibodies in Japanese Type 1 Diabetes Mellitus
Authors: Kida Kaichi , Mimura Goto , Kobayashi Tetsuro , Nakamura Koichi , Sonoda Shunro , Inoue Hiroo , Tsuji Kimiyoshi
Published: October 2007

HLA, ICA (islet cell antibody) and autoantibodies were studied in 65 Japanese patients with type 1 diabetes mellitus to elucidate the existence of immuno‐genetic heterogeneity. Patients with autoantibodies had increased frequencies of HLA DRw9 antigen and of HLA haplotype of Bw61‐DRw9, and a slow decay of ICA, while patients without autoantibodies had increased frequencies of HLA DR4 antigen and of HLA haplotype of Bw54‐DR4, and a rapid decay of ICA. These findings support the concept of immunogenetic heterogeneity in Japanese type 1 diabetes mellitus.
Release of High Molecular Weight Neutrophil Chemotactic Activity from Human Cultured Basophilic Cells
Authors: Nagakura Toshikazu , Shichijo Kozaburo , Likura Yoji , Kobayashi Miyuki , Saito Hirohisa , Otani Takeshi
Published: October 2007

We have shown that cultured basophilic cells, derived from human cord blood cells, release histamine and high molecular weight neutrophil chemotactic activity (HMW‐NCA), in a dose‐dependent manner following calcium ionophore and anti‐IgE challenge. The HMW‐NCA detected from human cultured basophilic cells had a similar molecular weight to that of human chopped lung challenged by anti‐IgE as determined by gel filtration chromatography. These results suggest that cultured basophilic cells might be employed for research into the chemical mediators involved in immediate hypersensitivity.
Analysis of Proteins in Unconcentrated Urine
Authors: Miyata Hiroshi , Yamamoto Takashi , Murata Ryogo , Kinoshita Tomohiro , Maki Sunao
Published: October 2007

We investigated methods for measurement of β2‐microglobulin, lysozyme, IgG and other globulin fragments such as Fc, F(ab')2, K‐and Δ‐chains in unconcentrated urine. A log‐log correlation was observed between the percent transferrin clearances and the molecular weights of β2‐microglobulin, lysozyme, transferrin and IgG in normal children by quantitative analysis. Glomerular damage was suggested in patients who excreted urinary protein in excess of 1 g/m2/ day. The selectivity patterns showed persistence of glomerular disorders in patients with membranoproliferative glomerulonephritis, crescentric glomerulonephritis, glomerulonephritis due to Henoch‐Schonlein purpura and lupus nephritis, in spite of decrease in urinary protein excretion after treatment.

Scanty globulin fragments were found in urine. The ratio of dimer‐ and monomer‐light chains found varied depending on the pathogenesis.

Electrophoretic figures seen by the Western blot method were able to differentiate urinary proteins using unconcentrated urine.

A combination of the procedures in this report could be useful to evaluate the severity and to monitor the progress of nephropathy.
Anticoagulant Combination Therapy of Gabexate Mesilate and Urokinase in Purpura Fulminans
Authors: Mimura Shunji , Kutsuna Tomoko , Tsukidate Kouichi , Iwase Katsuhiko
Published: October 2007

An attempt was made to combine anticoagulant therapy with gabexate mesilate and urokinase in a case of purpura fulminans developing after varicella infection. One week after the initiation of the treatment, the gangrenous extremities fully recovered without significant tissue necrosis. This is probably the first case of treatment of the disease that brought an immediate result.
Growth Response to Daily Subcutaneous Administration of Growth Hormone
Authors: Kikuchi Kiyoshi , Sudo Masakatsu , Miyarnoto Atsuko , Ohie Takaharu , Mori Chuzo , Mikawa Haruki
Published: October 2007

The effect on growth rate of daily subcutaneous (sc) administration of growth hormone (GH) was studied in seven GH‐deficient children. All patients had been receiving conventional GH treatment (intramuscularly, twice or thrice weekly) for three to six years. The growth‐promoting effect of conventional treatment gradually decreased in these seven patients [4.0 ± 0.3 (mean ± SD) cm%sol;year during the last preceding year of conventional treatment]. Following transfer to daily sc GH treatment without increase in weekly dosage, all seven patients showed an increase in growth rate (7.5 ±1.1 cm%sol;year, P>0.001). During the period of daily sc GH treatment, the pubertal stage did not advance in six of the seven patients. An increase in Δheight age%sol;Δbone age ratio was observed in six patients. None of the patients developed antibodies against GH. Local reactions were not observed at the injection site. Daily sc GH injections were tolerated without complaint. In conclusion, daily sc GH treatment is recommended for patients whose growth response to conventional GH treatment is poor.
Nasal Application of Inactivated Influenza Vaccine
Authors: Kimura Mikio , Kuno‐Sakai Harumi , Takesue Ryozo , Ozaki Kazuyo
Published: October 2007

Intranasally‐ rpplied inactivated influenza vaccines, which have been made for subcutaneous use, induced local production of specific IgA antibodies. There is a good prospect of practical use of inactivated influenza vaccines applied intranasally.
Relation between Urinary Dopamine and Sodium Output in Term Infants
Authors: Igarashi Noboru , Okuda Norihiko , Sato Tamotsu
Published: October 2007

The present study was designed to investigate the relation between endogenous dopamine and natriuresis during the early postnatal days in term infants. Urinary dopamine levels were measured by high‐performance liquid chromatography in the initial urines of 61 full‐term male infants and in serial collected urines at 3‐hour intervals during the first three days in five full‐term male. infants. Dopamine excretion rate (ng/mgCr or ng/hr) correlated significantly with sodium excretion rate in both the initial urines (mEq/mgCr) and serial collected urines (mEq/hr). In the initial urines, dopamine excretion rate (ng/mgCr) did not correlate with aldosterone excretion rate (ng/mgCr), which showed an inverse relation with sodium excretion rate (mEq/mgCr), but with cyclic AMP values (nmol/mgCr). In addition, dopamine excretion rate (ng/hr) significantly correlated with cyclic AMP excretion rate (nmol/hr) in one infant.

Although the significance of the direct relation between urinary dopamine and sodium output needs to be further investigated, it is possible that endo‐genously produced dopamine may play a role in natriuresis, via the cyclic AMP producing system, during the early neonatal period.
The Murphy‐Bowman Protocol in the Treatment of Childhood Non‐Hodgkin's Lymphoma
Authors: Armata Jerzy , Cyklis Romana , Dhiniewska Agnieszka
Published: October 2007

Twenty‐two consecutive previously untreated children with stage III and IV non‐lymphoblastic non‐Hodgkin's lymphoma (NHL) and with B‐acute lymphoblastic leukemia (B‐ALL) were treated according to the Murphy‐Bowman protocol. The remission rate was 82%. The estimated long‐term survival rate was 49% and the event‐free survival 41% (in stage III 57% and 53% and in stage IV and B‐ALL 20% and 0%, respectively). The protocol is effective in stage III but fails in stage IV and B‐ALL, which corresponds to the results obtained by other investigators using this or similar protocols.(Acta Paediatr Jpn 1988; 30: 204–208)
Outcome of the Treatment of Inborn Errors of Metabolism
Authors: Watts R. W. E.
Published: October 2007

The use of specific dietary restrictions, cofactor administration, mobilisation of insoluble substances, environmental modifications, product replacement and selective enzyme inhibition are now established for the treatment of some inborn errors of metabolism. There is no generally accepted application for enzyme administration, cytopharmacology (manipulation of the cytoskeleton) or for cell transplantation except for bone marrow transplantation in disorders where the bone marrow is primarily at fault. The other uses of bone marrow transplantation which have been proposed require further evaluation. Results of recent research suggest that the scope of this approach is gradually being widened. There is also scope for development in the field of organ transplantation taking advantage of recent technical1 and immunological progress. The treatment of inborn errors of metabolism by genetic modification is not yet on a practical clinical level; more laboratory and animal studies are needed before this can be attempted in man. Adenosine deaminase deficiency appears to be the disease in which this will be first attempted using a retroviral vector to insert the gene into the genome of pluripotential bone marrow cells.
PKU and NON‐PKU Hyperphenylalaninemia: Differentiation, Indication for Therapy and Therapeutic Results
Authors: Trefz Friedrich K. , Lichter‐Konecki Uta , Konecki David. S. , Schlotter Magdalena , Bickel Horst
Published: October 2007

The differential diagnosis of phenylalanine hydroxylase deficiency (PAHD) by biochemical methods is difficult. Using standardized oral protein loading or the intravenous deuterated phenylalanine (phe) load in 46 patients with PAHD, three groups could be distinguished: 1) Phenylketonuria (PKU) with plasma phe levels over 20 mg% under the protein load and with residual activities of less than 1% of normal; 2) mild PKU with plasma phe of 10–20 mg% and residual enzyme activities of 1–3%; 3) Non‐PKU hyperphenylalaninemia with plasma phe levels of less than 10 mg% and residual enzyme activities of more than 3%. Psychomotor development in 32 untreated patients with PAHD showed that there is a high risk of brain damage for all patients with in vivo residual activities of less than 3% of normal. Restriction fragment length polymorphism (RFLP) haplotypes at the phenylalanine hydroxylase (PAH) locus in 60 German patients with PAHD showed that 90% of the mutant alleles are confined to four distinct haplotypes. Using an oligonucleotide probe for the splicing mutation associated with mutant haplotype 3 a close association between the mutation and the haplotype 3 has been observed. There is also an association between haplotypes 2 and 3 and PKU patients with residual enzyme activities of less than 1%. However, in only 37% of our patients with PAHD could a direct diagnosis of the mutations in the PAH gene be made. More knowledge of other mutations in the PAH gene is necessary to differentiate patients with PAHD on the DNA level.
Dietary Treatment of PKU Using A Low‐Phenylalanine Peptide Milk
Authors: Owada Misao , Abe Motoko , Tanimoto Masashi , Minato Motoyuki , Kitagawa Teruo , Katayama Sumio
Published: October 2007

A method of preparation of low phenylalanine (Phe) peptide fomiula for phenyketonuria (PKU) and the long term therapeutic effects of the milk in 6 PKU infants and one adult female patient aged 24 who wanted to become pregnant, are described. Low phenylalanine peptides (LPP) were prepared from whey protein. After hydrolysis by pronase (actinase), the hydrdysate was passed through a charcoal column to remove aromatic amino acids, and the free amino acids and salts were removed by passing through RD membranes to obtain LPP. Together with vegetable oils, lactose, dextrin, vitamins and minerals, as well as a small amount of amino acid mixture, low phenylalanine milk was madexs by freeze‐drying UP. The product (LPP milk), which does not have the bad taste and odor peculiar to amino acids, was administered to seven patients with PKU for one to three years. There were no differences between the LPP formula and conventional formulae collsisting of amino acids formula (AA formula), in reducing serum Phe levels in PKU patients. There were no adverse effects in patients who received LPP formula. UP formula can be used not only as milk, but also as an ingredient to make more palatable foods of low Phe content.
Significance of Fibrin/ Fibrinogen Related Antigen in Glomeruli in Children with IgA Nephropathy
Authors: Kamitsuji Hidekazu , Sakamoto Shinya , Matsunaga Kenji , Taira Kouji , Kawahara Shingo , Nakajima Mitsuru
Published: October 2007

The relationship between the deposition of fibrin/fibrinogen related antigen (FRA) and morphologic findings in glomeruli was investigated in 59 children with IgA nephropathy. After treatment of renal biopsy sections with monochloroacetic acid (MCA) solution, MCA‐insoluble FRA, suggesting cross‐linked fibrin, was observed within the capillary walls, the mesangium and/or the sclerotic area in children with severe proliferative lesions, such as segmental sclerosis, capsular adhesions or crescent formation. Mesangial deposits of FRA were confined to electron‐dense deposits, and the MCA‐soluble FRA did not seem to be closely related to glomerular damage. FRA deposits within crescents may consist of MCA‐soluble FRA.
Influence of Drugs on Albumin and Bilirubin Interaction
Authors: Funato Masahisa , Lee Youkei , Onishi Shoju , Cashore William J.
Published: October 2007

Twenty‐eight drugs, including cephem antibiotics and anti‐inflammatory agents currently used or considered potentially useful in neonatal intensive care nurseries in Japan, were examined to determine their influence on albumin and bilirubin interaction by means of a glucose oxidase ‐ peroxidase method, using an automated analyzer (Arrows) for unbound bilirubin (U.B.). The apparent binding constant for drugs to the high‐affinity site on albumin (KD) was determined. Of cephem. antibiotics, latamoxef sodium (LMOX) and cefazolin sodium (CEZ) were found to displace bilirubin from albumin (KD= 5.9 times 103 M‐1 and 4.5 times 103 M‐1, respectively) as strongly as Na salicylate (KD= 6.8 times 103 M‐1). Mephenamate and indomethacin, which are used for medical closure of patent ductus arteriosus in premature infants, were also found to be stronger bilirubin displacers (Kn = 1.3 times 105 M‐1 and 1.2 times 105 M‐1, respectively) than sulfisoxazole (KD= 1.6 times 104 M‐1). Maximal displacement factors (MDF's) were also estimated in reference to protein binding (%) and effective serum concentration (M) of each drug in human adults. Of these drugs, mephenamate showed a higher risk of bilirubin displacement (MDF = 3.79) than sulfisoxazole (MDF = 2.58) and LMOX had a higher risk of displacement (MDF = 1.97) than Na salicylate (MDF = 1.85). On the other hand, indomethacin and CEZ showed minimal effects on displacement at therapeutic levels (MDF = 1.03 and 1.00, respectively). At therapeutic serum levels, mephenamate and LMOX may possess the potential for displacing bilirubin from albumin and increasing the risk of bilirubin encephalopathy, in a manner similar to sulfisoxazole.
Y Chromosome Specific DNA Probe in the Diagnosis of a Patient with mos 45, X/46, XYnf
Authors: Yorifuji Tohru , Momoi Toru , Sonomura Takako , Yamanaka Chutaro , Kaji Masayuki , Mikawa Haruki , Kitagawa Kyoko , Ozasa Hiroshi , Tai Shunzo , Mori Takahide
Published: October 2007

In a patient with mos 45,X/46,XYnf, the diagnosis was confirmed with a Y chromosome‐specific DNA probe, Y‐190. The patient was a phenotypic female without Turner syndrome stigmata other than short stature. She showed some evidence of virilization and high serum testosterone. Her peripheral blood karyotype was mos 45,X/46X, +mar. Although this marker chromosome resembled a Y chromosome, there was no quinacrine bright region on its long arm. Southern blot analysis of her peripheral blood mononuclear cell DNA with Y‐190 as a probe showed strong hybridization with this probe. Gonadectomy was performed, and bilateral gonadoblastomas were found.
Very Long‐Chain Fatty Acids in Erythrocyte Membrane Phospholipids in Adrenoleukodystrophy
Authors: Tanaka Kazuhiko , Shimada Morimi , Naruto Toshiyuki , Yamamoto Hisashi , Nishizawa Kashirou , Saeki Yukikazu
Published: October 2007

Mass fragmentography was used to analyze the very longchain fatty acid (VLCFA) composition of erythrocyte membrane phospholipids. The VLCFA content decreased in the order sphingomyelin (SM), phosphatidylcholine (PC) and phosphatidylethanolamine (PE). The tetracosanoic acid (C24:0) and hexacosanoic acid (C26:0) content of both SM and PC in patients with adrenoleukodystrophy (ALD) were significantly higher than those in controls. The VLCFA content of PE was too small, in comparison with those of SM, to be accurately determined. (Acta Paediatr Jpn 1989;31: 136–143)
Evaluation and Comparison of the Axis of the Interventricular Septum by Two‐Dimensional Echocardiography among the Patients with Congenital Heart Disease (ASD, VSD, PDA, PS, TOF) and the Normal Subjects
Authors: Lin Yi‐Fong , Awa Shoichi , Hishi Toshio , Akagi Michio , Dodo Hidemi , Ishii Teruyuki , Watari Tohru , Oho Shinichi , Nakamura Gen
Published: October 2007

A two dimensional echocardiographic method was applied to determine the axis of interventricular septum (IVS) in the horizontal plane. This study comprised 19 subjects with ASD, 15 subjects with VSD(I), 20 subjects with VSD(II), 13 subjects with PDA, 16 subjects with PS, 15 subjects with TOF and 99 normal children. Parasternal left ventricular short axis view was taken, and the IVS was recorded by using the Polaroid prints. The axis of the IVS in the horizontal plane was measured from the recorded Polaroid prints. The value of the angularity of IVS (IVS‐A) expressed as means f one standard deviation (mean ± 1S.D.) is 52.0 ± 13.7° in ASD, 42.2 ± 9.7° in VSD(I), 43.7 ± 9.1° in VSD(II), 41.6 ± 11.4° in PDA, 38.6 ± 10.9° in PS, 61.0 ± 8.5° in TOF and 40.1 ± 8.2° in normal controls. There was no significant difference among VSD(I), VSD(II), PDA, PS and normal controls but a highly significant difference was noted in ASD and TOF as compared to other groups. The correlation coefficient of the IVS‐A with (1) the Qp/Qs ratio, (2) the magnitude of shunt (%), (3) the right ventricular pressure and (4) the hematocrits were evaluated among the patient groups. The IVS‐A of ASD and VSD(II) had a good correlation with the shunt flow (r=0.921 and 0.88 respectively) and/or the Qp/Qs ratio (r=0.782 and 0.955 respectively); while that of VSD(I), PDA, and PS had a good correlation with the right ventricular pressure (r=0.956, 0.953, 0.842 respectively) and that of TOF was mostly concerned with the hematocrits (r=0.911). Besides, the IVS‐A in each severe CHD subgroup was significantly (p < 0.005 or a better value) higher than the normal control group. Thus an alternative method was validated for evaluating the severity of congenital heart disease by using a noninvasive twodimensional echocardiographic technique. (Acta Paediatr Jpn 1989; 31: 186 ‐ 197)
Transient Carnitine‐Responsive Medium‐Chain Dicarboxylic Aciduria in an Infant with Cholestasis, Hypoglycemia and Cardiac Failure
Authors: Matsuo Masafurni , Saiki Kayoko , Mornota Tetsuya , Ishida Akihito , Kanazawa Katsuhiko , Murakarni Ryusuke , Nakarnura Hajirme , Matsuo Tarnotsu
Published: October 2007

In a cholestatic infant showing hypoglycemia and cardiac failure, nonketotic mediumchain dicarboxylic aciduria was disclosed by urinary organic acid analysis. As urinary excretion of long‐chain fatty acids was also increased, a defect in β‐oxidation of longchain fatty acids appeared likely. To try to improve this abnormality, carnitine supplements were given, which led to the complete resolution of clinical and laboratory abnormalities. This is the first reported case of a cholestatic infant who responded to carnitine supplementation. Deficiency of carnitine palmitoyl transferase was suspected as the underlying cause. (Acta Paediatr Jpn 1989; 31: 211 ‐ 215)
Clinical Course and Prognosis of Childhood Epilepsy
Authors: Oka Eiji , Yamatogi Yasuko , Ohtsuka Yoko , Ohtahara Shunsuke
Published: October 2007

Remarkable progress has been accomplished in epileptology in recent years. The remission rate in childhood epilepsy has been improved by the introduction of new technologies and better diagnostic as well as therapeutic methods. Investigation of prognosis is an important field of epileptology, because prognostic improvement reflects its overall progress, A long‐term follow‐up study of childhood epilepsy was undertaken in the Okayama University Hospital. Ten to 15 years of follow‐up was possible in 730 of 1,295 patients who were first diagnosed at ages below 15 years, from 1968 to 1971. The 3‐year remission rate amounted to 82.0% and 5‐year remission was obtained in 79.1%. These high rates of remission indicate the favorable prognosis of childhood epilepsy. On the other hand, cases of intractable epilepsy also amounted to a considerable number. Intractable epilepsy consisted mainly of agedependent epileptic encephalopathy (Ohtahara's syndrome, West syndrome and Len‐nox‐Gastaut syndrome) and severe myoclonic epilepsy in infancy. Development of effective therapy for these intractable epileptic syndromes will be an important subject of future studies.
Improvement of Modem Treatment and Outcome in Childhood Epilepsy in Asia
Authors: Chi Ching‐Shiang
Published: October 2007

Epilepsy and epileptic syndromes are one of the major pediatric neurological diseases in Taiwan, R.O.C. In 1984 we investigated 38 elementary schools in Taichung city, in the middle‐west part of Taiwan. Among 57,944 school‐aged children, 388 had suffered from at least two episodes of afebrile seizures occurring separately over a two‐week period. The period prevalence rate was 0.67%. In our clinic, once seizures are diagnosed, the seizure types are then classified as well as possible, in order to develop a rational approach to treatment. We commonly use carbamazepine for partial seizures and some generalized seizures, low dose ACTH (10 I.U.) for infantile spasms, valproic acid for absence seizures and myoclonic seizures, clonazepam for atonic‐akinetic and myoclonic seizures, and phenobar‐bital for young children with generalized seizures. In the last three years, we have used a ketogenic diet for akinetic‐atonic seizures and Lennox‐Gastaut syndrome; we have also used temporal lokctomy for complex partial seizures since 1981. Owing to the marked increase in the medical understanding of epileptic seizures and syndromes, and models of treatment, there is no question that children with seizure disorders are better off today than they were ten years ago.