bluuebunny/crossref_metadata_2025 huggingface dataset:
The Rate of Clonal Transformation of B‐Lymphocytes by Epstein‐Barr Virus in Childhood
Authors: Furukawa Toshiharu , Kaiga Kazuhiro , Takahashi Hiroshi , Sugita Kenichi , Eguchi Mitsuoki
Published: October 2007

The rate of clonal transformation of the B‐lymphocyte by Epstein‐Barr virus (EBV) in children was studied in semisolid agar culture. A remarkable influence of the cell donor's age on the rate was found: In the order of age, the rate was highest in cord blood, lower in infants, lowest in young children, somewhat higher in school children and high in adults. The rate of EBV‐associated nuclear antigen (EBNA)‐positive cells 24 h after infection showed little age‐dependent difference. The only significant difference was found between cord blood and young children. From these results, the age‐dependent difference in the rate of clonal transformation seemed to be mainly in the process after EBNA expression through transformed cell colony formation.

In the children with trisomy 21 or ataxia‐telangiectasia, who have a high risk of leukemia or malignant lymphoma, the rate of clonal transformation was not higher than in age‐matched control groups.
“How Small Is Too Small?” in Japan 1983
Authors: Nishida Hiroshi , Sakanoue Masamichi , Ishizuka Yuugo
Published: October 2007

Along with the advancement of neonatal medicine, “how small is too small?” for the neonate to survive and for the physician to intervene becomes important. On the basis of recent data on very small neonates in Japan, we present our approach to this subject. The overall mortality rate of neonates weighing less than 1,000 gm was 55% and their incidence of major neurological handicaps was 6.6%. There was a clear difference between mortality rates of neonates weighing above and below 700 gm, which were 34% and 80% respectively, but their morbidity rates were more or less the same. The current record of the smallest neonate to survive in Japan is 444 gm. Therefore “how small is too small?” in the 1980's in Japan will be around 700–1,000 gr for generalized socio‐medical agreement for active intervention and around 400–500 gm for individualized decision‐making for possible survival.
Efficacy of HBIG and Hepatitis B Vaccine for the Prevention of HBV Transmission
Authors: Kobayashi Masakazu , Koike Michio , Kashii Kensaku , Sumiyama Keiichiro , Kusumoto Sachiko
Published: October 2007

Abstracts

The efficacy of HBIG and HB vaccine is summarized for the prophylaxis of HBV infection. Cases were divided into a “vertical” group of 130 neonates, consisting of 90 infants who received HBIG twice and then Hb vaccine from the second month after birth and HB vaccine beginning on the 5th day after birth (vertical II), and a “horizontal” group consisting of 108 infant who were given HB vaccine three times.

In each case, excellent results were obtained and no adverse reaction was noted; however the number of cases who were not immunized was significantly higher and the member of chronic HBs‐Ag carriers was higher in the vertical II group. the continuity of the immune state was fairly good for at least three years in the vertical I and horizontal groups.

We think that in the case of the vertical group, the course of HB vaccine should be started in the second month after birth with the second inoculation of HBIG.
Membranous Glomerulonephritis in WiskottAldrich syndrome
Authors: Ariga Tadashi , Takahashi Yutaka , Watanabe Tohru , Tsuneta Hiromi , Takekoshi Yasuo , Sakiyama Yukio , Matsumoto Shuzo , Ishii Toshiaki
Published: October 2007

15‐year‐old boy with Wiskott‐aldrich syndrome (WAS) associated with nephrotic syndrome was reported. Histological findings on a renal biopsy specimen by light, fluorescence and electron mictroscopies disclosed pathologic changes typical of mambranous glomerulonephritis.

He had an abnormalityin cellular and humoral immunity, i.e. decreased delayed type hypersensitivity, low level of IgM and high level of IgE, and was suspected to have a persistent Epstein Barr virus (EBV) infection. Since membranous glomerulonephritis is one of the immune complex types if nephritis, we attempted to demonstrate EBV in the renal tissue but could not confirm its presence.

Nephritis is not common;y associated wioth WAS. However, the prolonged survival through improved medical management may allow the more frequent development of renal diseases. Attention should therefore be paid to the appearance of renal maniofestation in patients with WAS.
A Case of Thanatophoric Dwarfism With Renal Hypoplasia
Authors: Tohya Toshimitsu , Miura Kazunori , Nagata Noriyuki
Published: October 2007

An autopsy case of thanatophoric dwarfism with renal hypoplasia is described. Histologically, the characteristic findings were disorganization of endochondral ossification at the epiphyseal plate of each femu, and this findings were useful for different diagnosis. Hypogenesis of the right kidney is rare in this disorder, and is suggestive of the vaiety of this disorder.
Hepatitis B Virus Infection in Infants and Children in Japan
Authors: Koike Michio , Kobayashi Masakazu , Sumiyama Keiichiro
Published: October 2007

An epidemiological study done with 4,537 of so‐called “hospital normal” children between January 1979 and April 1982 is reviewed [2, 3].

The number of HBsAg positive carriers is 35 (0.77%) and anti‐HBs positive rate is 1.7%. The average HBeAg positive rate of 81 HBV carriers is 67%, which represents 100% in 0‐year‐old babies and rapidly decreases to 50% in 10‐year‐old children.

Estimation of the route of transmission is done in index cases of HBV carriers after thorough examination of their family members. The ratio of horizontal to vertical transmission is 50:50, suggesting the rapid decrease of the incidence of the horizontal route since 1980. The course of HBV carriers is followed and it is found that carriers derived from vertical transmission give rise to a more troublesome prognosis. Other Japanese literature is also reviewed.
Causes of Failure in Prevention of Vertical Infection by Hepatitis B Virus
Authors: Shiraki Kazuo , Tanimoto Kaname , Yamada Kazuhito , Okada Takayoshi
Published: October 2007

In order to prevent the hepatitis B virus carrier state, 190 infants born to hepatitis‐Be antigen positive hepatitis‐B surface antigen carrier mothers were treated with hepatitis‐B hyperimmune globulin(HBIG) and hepatitis‐B vaccine after birth; however, 8 infants(4.2%) ultimately became carriers.

There were three different causes for the failure, namely 1) prenatal infection, 2) insufficiency of HBIG to neutralize the virus transmitted, and 3) poor responsiveness of the infant to the vaccine. The present study suggests that early vaccination in the neonatal period increases the rate of poor responders as compared to later vaccination.
IgM Antibody to Core Antigen of Hepatitis B Virus in Vertical Transmission
Authors: Yamanaka Tatsuru , Tsuzuki Akiko , Takayanagi Naoki , Chiba Shunzo , Chiba Yasuo , Nakao Tooru
Published: October 2007

Transmission of hepatitis B virus (HBV) from a mother to the child is mostly due to infection during delivery. It has been suggested, however, that intrauterine HBV infection can occur in a small number of subjects. In the present study, we examined IgM antibody to core antigen of HBV (IgM‐HBcAB) in cord blood to see whether intrauterine infection could be distinguished from neonatal transmission of HBV. Infants with possible intrauterine infection, however, did not have IgM‐HBcAb in cord blood. It is concluded, therefore, that examination of this antibody is not useful for such purposes. IgM‐HBcAb may not be produced during the period when immune tolerance to HBV is maintained, since it was detected only in association with occurrence of liver cell injury.
Appearance Rat of HBs Antibody and One‐Year Change in Administration of HB Vaccine
Authors: Oda Yoshihiko , Satoh Masahisa , Ishizuka Toshie , Yamazaki Akira , Miyakawa Kimiko
Published: October 2007

In order to prevent vertical infection between mother and infant with HBV, a method of giving passive immunity by anti‐HB human immune globulin just after birth and then active immunity by means of HB vaccine has been initiated. However, the sustained effect of antibody acquired through HB vaccine is not ascertained.

In this study, we examined the appearance rate of anti‐HBs titer and its sustained effect by administering HB vaccine three times.

Anti‐HBs appeared in all 13 cases by five months after birth, after the injection of HB vaccine three times. The titer was from 64 to 1,024. However, nine months after birth, there was a reduction of 1–2 tubes, and antibody titer was 64 to 512. Twelve months after birth a further reduction of 1–2 tubes was observed. In those cases in which anti‐HBs titer was under 256 at six months, by 12 months it decreased to 16–32. In such cases, a booster injection 12 months after birth was considered necessary.

GOT and GPT showed changes within the normal range, and no local reaction at the injection site was found.
Effects of Short‐Term Prednisolone Therapy (So‐Called Steroid Rebound Therapy) in Children With HBeAg‐Positive Chronic Hepatitis B
Authors: Tanaka Hiroshi , Yasuda Masanori
Published: October 2007

We have studied the effect of short‐term prednisolone therapy on seroconversion from HBeAg to anti‐HBe, transaminase levels and hepatitis B virus markers in twelve children with HBeAg‐positive chronic hepatitis B. They were followed up for more than two years after the discontinuation of prednisolone. On discontinuation, 11 of the children (91.6%) showed disappearance of HBeAg. In eight of them (66.6%), seroconversion from HBeAg to anti‐HBe occurred, concurrently with a gradual fall in serum transaminase level. The seronegative reaction occurred within two to 11 months, and seroconversion occurred within five to 15 months after withdrawal of prednisolone. Transaminase activities fell to normal and have remained normal during two to three years in all 11 patients, with disappearance of HBeAg. Hepatitis B virus‐DNA polymerase activities were markedly elevated during prednisolone treatment, and then gradually declined along with or before seroconversion from HBeAg to anti‐HBe. The patients seroconverted from HBeAg to anti‐HBe showed DNA polymerase negativity throughtout the period of observation. It is thought that rapid withdrawal of prednisolone in children with HBsAg‐ and HBeAg‐positive chronic hepatitis will result in a reduction or elimination of HB virus. In our follow‐up study, HBsAg disappeared in only one patient. No severe symptoms were encountered during the period of this short‐term steroid therapy.
Treatment of Cystinosis: Decrease in Content of Free Cystine in Leukocytes and Alleviation of Progressive Renal Failure by Treatment with Riboflavin
Authors: Hayasaka Kiyoshi , Narisawa Kuniaki , Saitoh Takashi , Numakura Hirohiko , Fukuda Kaoru , Kato Seiichi , Tayama Toshiyuki , Tada Keiya
Published: October 2007

Two patients with cystinosis were treated with riboflavin, resulting in a decrease in the content of free cystine and taurine in leukocytes. Riboflavin therapy was not sufficient to improve the renal function; however, the progression of renal failure was alleviated.
Analysis of Thyrotropin‐Thyroxine Secretory Dynamics in Infantile Hyperthyrotropinemia: An Aid for Early Differential Diagnosis
Authors: Sato Tamotu , Ishiguro Kazumasa , Kubo Minoru , Wada Naoki
Published: October 2007

For the purpose of differentiating neonatal hyperthyrotropinemia, the interrelation between serum thyrotropin (TSH) and thyroxine (T4) was examined in 10 infants with primary hypothyroidism during 12 months of T4 replacement therapy. A significant inverse correlation was obtained between elevated TSH (Y) (above 12 μU/ml) and T4 (X) levels; Y = 324 ‐ 22 X (r = ‐0.801, p < 0.001). One patient had an abnormally high threshold of TSH‐inhibition by T4. Based on this regression line, a scattergram of TSH‐T4 correlation and changes in the time course was analyzed in 13 infants with neonatal hyperthyrotropinemia, including eight with transient hyperthyrotropinemia (t‐TSH), three with compensated hypothyroidism (c‐Hypo) and two with transient hypothyroidism (t‐Hypo). All plots from t‐TSH were distributed under the regression line and shifted downward with concomitant rise in T4 and free T4 levels. In contrast, plots from c‐Hypo and t‐Hypo showed a vertical upward shift above the line during 3–4 months of observation without treatment. These results indicate that (1) using the TSH‐T4 regression line, differentiation of c‐Hypo from t‐TSH is possible by 3 months of age, (2) the cause of t‐TSH is not an elevated threshold for TSH release, but rather the maturational delay in responsiveness of the thyroid to TSH, and (3) suppression of TSH alone should not be used as the index of adequate T4 dose in infantile hypothyroidism, because of the existence of patients with a high threshold of TSH‐inhibition by T4.
Incidence of Minor Physical Anomalies in Patients With Early Infantile Autism
Authors: Ohdo Shozo , Madokoro Harumichi , Sonoda Tohru , Hayakawa Kunio
Published: October 2007

The incidence of 28 minor physical anomalies was examined in 118 patients with early infantile autism, 132 patients with 21 trisomy, and 237 normal subjects.

Although the incidence of minor physical anomalies in children with early infantile autism was slightly higher than in the group of normal subjects, there was no significant difference between the groups. It was suggested from these findings that there is little possibility of the onset of early infantile autism being due to environmental insults at the embryonic period when minor physical anomalies are formed, and that there is no possibility of minor physical anomalies giving a clue as to the time of onset of early infantile autism. The incidence of minor physical anomalies in patients with 21 trisomy was significantly higher than in the group of normal subjects and of children with early infantile autism (p < 0.00l).

Fra (X) was noted in two patients among 40 examined. These patients were not included among the subjects in the present study.
The West Syndrome: Developmental Aspects
Authors: Ohtahara Shunsuke , Ohtsuka Yoko , Yamatogi Yasuko
Published: October 2007

The electro‐clinical characteristics of childhood epilepsy are known to appear during the development of the brain and to change their features with age. Accordingly, developmental viewpoints are indispensable to researches on childhood epilepsy.

Age‐dependent epileptic encephalopathy has the most remarkable developmental characteristics among childhood epilepsies.

Early‐infantile epileptic encephalopathy with suppression‐burst (EIEE), the West syndrome and the Lennox‐Gastaut syndrome, which constitute age‐dependent epileptic encephalopathy, have many common features. Each of these three syndromes, however, has its own distinctive clinical and electro‐encephalographic specificities, and shows mutual transition with age.

Results of developmental studies on the West syndrome are described, confirming it as one type of age‐dependent epileptic encephalopathy.

1) On the basis of the long‐term follow‐up study of 14 cases of EIEE, sequential transition from EIEE to the West, and further to the Lennox‐Gastaut syndrome was observed, with reference to critical changing period, transitional patterns and prognoses.

2) Analytical follow‐up study of 83 cases of the West syndrome, divided into idiopathic and symptomatic groups showed its evolution into the Lennox‐Gastaut syndrome to be important for its prognosis, with reference to the various factors relating to this transition.
The Incidence of Erythrocyte Pi Antigen Retention in Children With Urinary Tract Infection
Authors: Takano Kenichiro , Sakai Kaoru
Published: October 2007

The erythrocyte P, antigen was observed in 91.7, 70.6, 56.7 and 62.5%, respectively, of children with upper urinary tract infection, symptomatic and asymptomatic urinary tract infections and cases responsive to initial therapy in which the abnormal urinary findings disappeared with the administration of antibiotics given despite the absence of bacteriuria. These values were significantly high (P<0.001, 0.001, 0.01, 0.001, respectively) compared with healthy controls (29.0%).

In cases where initial antibiotic therapy was effective despite no significant bacteriuria, the presence of Gal cd– 4 Gal receptor in large quantity on the surface of the mucosa of the urinary tract was suggested, just as in the typical case of urinary tract infection with bacteria in the urine.

No significant difference was observed in the incidence of P1 antigen in the peripheral blood of sick children according to the clinical symptoms and urinary findings in typical cases of urinary tract infection and cases responsive to initial antibiotic therapy.
Clinical Application of Neonatal Instantaneous Heart Rate Monitoring—Heart Rate Response to Apnea —
Authors: Nishida Hiroshi , Sakanoue Masamichi
Published: October 2007

Changing patterns of instantaneous heart rate associated with apnea were analysed in 22 neonates; ten sleepy infants due to analgesic agents used during delivery, five preterm infants with favourable outcome and seven sick neonates with major medical complications. Patterns of heart rate change were classified into five types: type I deceleration, which is a sharp drop of heart rate synchronized with apnea; type II deceleration, which is a gradual decrease of heart rate during apnea; type III decerelation, which is a bradycardic spell without consistent relation with apnea; acceleration, which is a rapid increase of heart rate associated with respiration change; and a non‐reactive type, in which there is no appreciable heart rate change during apnea. Type II deceleration, type III deceleration and the absence of acceleration were strongly related with poor clinical conditions. Type I deceleration was frequently observed in most neonates with apnea and was not necessarily regarded as an ominous finding. The non‐reactive type was seen in both cases, with favourable and unfavourable outcome. Application of the knowledge of fetal heart rate monitoring to neonatal monitoring is possible and is rather promising.
The Effects of Bifidobacterium breve Administration on Campylobacter Enteritis
Authors: Tojo Masahiro , Oikawa Tadao , Morikawa Yoshiyuki , Yamashita Naoya , Iwata Satoshi , Satoh Yoshitake , Hanada Juhei , Tanaka Ryuichiro
Published: October 2007

We studied the effects of Bifidobacterium breve on Campylobacter enteritis. Patients with Campylobacter enteritis were divided into three groups (I. Erythromycin recipients; II. B. breve recipients; III. control) and studied with regard to the duration of diarrhea and carriage of Campylobacter jejuni in the stools. Additional studies were performed to observe the effects of B. breve on fecal flora. The results showed that there were no significant differences in the duration of diarrhea among the three groups. Comparison of the duration of C. jejuni carriage in the stools was as follows: KIKIII (P<0.01). Microbiological study of fecal flora demonstrated that the number of bifidobacterium was decreased in Campylobacter enteritis compared to the control, and an increase in the number of bifidobacterium could be recognized after the administration of B. breve. B. breve was useful in eradicating C. jejuni from the stools and restoring the normal intestinal flora in Campylobacter enteritis, although if was not effective in shortening the duration of diarrhea.
Growth and Maturation in the Male Genitalia From Birth to Adolescence II. Change of Penile Length
Authors: Fujieda Kenji , Matsuura Nobuo
Published: October 2007

Penile lengths of Japanese males from birth to adolescence were studied in cross‐sectional series. The mean length of the penis in 5 days term newborn was 2.9 ± 0.5 cm (M ± SD). There was a gradual increase in penile length from the age 1 to 11 years. After 12 years of age there was a sudden increase in length. The mean length increased from 5.5 ± 1.5 cm at the age of 12 to 8.3 ± 0.8 cm at 14 years. The largest increment was observed between the age of 13 and 14 years. Mean penile length increased from 4.9 ± 0.8 cm at stage I of pubic hair development to 7.2 ± 1.4 cm at stage II and 9.0 ± 1.0 cm at stages IV‐V. The largest increment was observed between stage I and stage II of pubic hair development. This new reference of penile length can be helpful in those cases in which the children is suspective of having genitals of abnormal size.
Behavioural Deviance in Japanese Children Attending School in England
Authors: Morita Hiroshi
Published: October 2007

The rates of behavioural deviance in Japanese children attending school in England were investigated. A cross‐cultural comparison using the Rutter teachers' questionnaire was made between these Japanese children and some English children, which showed considerable similarity between the two sets. Possible additional ways teachers might identify Japanese children with deviant behaviour were described. These included teachers' ratings of “school failure,”“nurse's room user” and “not being liked by others.” The self‐concept of these Japanese children was also explored using the Piers‐ilarris children's self‐concept scale.
Changes in Renal Excretions of Electrolytes Upon an Orthostatic Postural Change in Chronic Glomerulonephritis
Authors: Asami Tadashi , Hashimoto Kin‐ya , Nakano Tokushi , Sakai Kaoru
Published: October 2007

Changes in renal excretions of sodium (Na), potassium (K), and chloride (CI) upon a postural change from sustained recumbency to standing and walking were examined in 146 children with chance proteinuria and/or hematuria and in 27 children with biopsy‐proved diffuse mesangial proliferative glomerulonephritis (GN) including membranoproliferative GN (MPGN). Renal excretions of Na, K, and CI were inhibited in children with macroscopic hematuria and/or proteinuria and in those with moderate to severe mesangial proliferative GN and MPGN. Reductions in urinary Na/K ratios, reflecting the effect of aldosterone on renal tubular reabsorption, were noted upon a postural change, but there was no significant relation between the changes in urinary Na/K ratios and glomerular lesions. These results suggest that renal excretions of Na, K, and CI are simultaneously decreased upon an orthostatic postural change in children with severe mesangial proliferative GN and MPGN.
Pyogenic Liver Abscess due to Bacteroides fragilis
Authors: Ka Keiki , Ichibangase Makoto , Terashima Itaru , Maeda Kazuichi
Published: October 2007

A 10‐year‐old boy with anaerobic pyogenic liver abscesses due to Bacteroides fragilis is reported. Blunt trauma was considered as the possible cause of the liver abscesses. Imaging methods such as ultrasonography, computed tomography and liver scannings were useful for the diagnosis of the case. The patient was cured after treatment with surgical debridement and drainage followed by a 10‐day course of intravenous Lincomycin.
Clinical Use of a Portable Infusion Pump and Pen‐Type Injector in Type 1 Diabetes Mellitus
Authors: Tsuchiya Yutaka , Cho Hideo , Kotoh Shinobu , Yokoyama Tetsuo , Matsuo Nobutake
Published: October 2007

Intensive insulin therapy was studied in six children with type 1 diabetes mellitus which was refractory to conventional insulin treatment. The methods used were continuous subcutaneous insulin infusion with a portable pump, and pre‐meal pulse infusion of short‐acting insulin with a pen‐type syringe supplemented with a bolus of long‐acting insulin. The former method was used in three patients for two weeks and in one patient for four months, in hospital, and the latter was used in two patients, starting in hospital and continuing for one year at home. Both these methods achieved better metabolic control than conventional therapy. In addition, insulin requirements decreased with continuous subcutaneous infusion but it had to be discontinued after discharge because of costs and the complexity of the equipment. With pre‐meal pulse infusion, insulin requirements increased compared with conventional therapy.
Therapeutic Effects of Dipolar Aprotic Substances on Niemann‐Pick Cells
Authors: Sakuragawa Norio , Sato Mitsuru , Kamo Isao , Arima Masataka
Published: October 2007

Dimethylsulfoxide (Me2SO) increased acid sphingomyelinase activity in cultured skin fibroblasts (Sakuragawa N et al., Biochem Biophy Res Comm 1985; 126: 756–762). Various drugs were examined to find related compounds showing the same phenomenon as Me2 SO, including other dipolar aprotic substances, various inhibitors of DNA and RNA synthesis, phorbol esters, vitamin E and its derivatives and other miscellaneous agents. Several dipolar aprotic substances, such as Me2SO, dimethylacetamide, tetramethylurea and hexa‐methylene‐bisacetamide, increased the acid and Mg‐independent neutral sphingomyelinase activity in human cultured skin fibroblasts. These dipolar aprotic substances also increased acid and Mg‐independent neutral sphingomyelinase activities in Niemann‐Pick cells, type C, to 300 to 700% of those of untreated controls, repairing their enzyme deficiency. Other lysosomal hydrolases increased to a slightly lesser extent than sphingomyelinase. Dipolar aprotic substances could be used as therapeutic drugs for Niemann‐Pick disease, type C.
The Progeny of Individuals Cured From Acute Lymphoblastic Leukaemia
Authors: Derulska Danuta , Rokicka‐Milewska Roma
Published: October 2007

Five former patients cured from acute lymphoblastic leukaemia (ALL), four women and one man, have six healthy children: three girls and three boys. The children were born in the period from 13 to 24 years since the beginning of the disease, and five to 16 years after therapy cessation. The treatment of the patients lasted eight to nine years.
Hemolytic Uremic Syndrome Associated with Common Bile Duct Obstruction
Authors: Hata Daisuke , Miyanomae Takeshi , Sensaki Shigeki , Okuma Minoru , Mikawa Haruki
Published: October 2007

A four‐year‐old female with hemolytic uremic syndrome (HUS) had reduced plasma prostaglandin I2 regenerating activity (PGI2‐RA). The patient had a cholecystectomy with T‐tube drainage of the common bile duct, because she developed common bile duct obstruction due to cholelithiasis. This is the first report of this syndrome in which there was associated common bile duct obstruction due to cholelithiasis.
A Case With the Infantile Type of Glycerol Kinase Deficiency
Authors: Kakinuma Hiroaki , Nakamura Fumiko , Murayama Shjgeo , Goto Jun , Nakano Imaharu , Saito Fumiko , Ohtake Akira , Takayanagi Masaki , Nakajima Hironori
Published: October 2007

A male infant with the infantile type of glycerol kinase deficiency is described. At six years of age, he showed proximal dominant muscle atrophy and weakness, addisonian pigmentation and mental retardation. Laboratory investigations revealed muscular dystrophy, adrenal insufficiency and glycerol kinase deficiency. He has a small deletion in a band (Xp21) of the X chromosome. The clinical, biochemical and genetic findings in this patient are reported.
Chemical Mediator in Exercise‐Induced Asthma and Exercise‐Induced Late Asthmatic Responses
Authors: Likura Yoji , Nagakura Toshikazu , Kondo Tomomi , Odajima Yasuhei , Walsh Garry M. , Masaki Takuro , Obata Toshihiko
Published: October 2007

In this paper, we describe chemical mediator fluctuation in exercise‐induced asthma (EIA) and exercise‐induced late asthmatic responses (EILA).

The moderate, severe patients who showed EIA, had statistically significant increased plasma histamine levels after exercise. Leukotriene (LT) release from polymorphonuclear leukocytes (PMN) showed two different patterns.

In the EIA negative group, generation of LTs from PMN showed a significant increase after an exercise task. However, the EIA positive group showed statistically significant decreases at 15 minutes after exercise.

In EILA, patients showed biphasic decreases in FEVi.o following exercise, which was accompanied by a parallel increase in neutrophil chemotactic activity (NCA).
Chronic Rhinitis in Children
Authors: Pearlman David S.
Published: October 2007

As pediatricians have long known, children are not small adults. Although there are many features of chronic rhinitis in children similar to adults, there also can be significant differences in etiologic factors, manner of presentation, and therapeutic implications. The fact that children are growing and developing also has special therapeutic implications.
Neurological Outcome in Full‐Term Infants with Intraventricular Hemorrhage
Authors: Sugimoto Tateo , Woo Man , Okazaki Hitoshi , Sonoda Noriaki , Ono Atsushi , Kinoshita Yo , Matsusaki Shuji , Iwase Suiko , Kobayashi Yohnosuke
Published: October 2007

Eight survivors among 12 full‐term infants with intraventricular hemorrhage (IVH) were investigated for neurological handicap. Five patients developed epilepsy during the follow‐up period of 4.5 years (range 2.0—6.8 years); four patients had an electroencephalogram (EEG) showing unilateral paroxysmal spikes, mainly in the central region, suggesting ventricular dilatation. The seizures were either partial, evolving to generalized seizures, or were generalized tonic‐clonic seizures. Patients with either moderate ventricular dilation and/or grade III IVH with severe asphyxia had a poor developmental and neurological outcome.
MELAS, Myoclonus, Ataxia and Deficiencies of Complexes I and IV in Muscle Mitochondria
Authors: Yamanaka Reiko , Nomura Yoshiko , Segawa Masaya , Nonaka Ikuya
Published: October 2007

A 13‐year‐old Japanese girl developed convulsions, periodic attacks of coma with hyperpyrexia, visual disturbance, aphasia, myoclonus, cerebellar symptoms and dementia. A muscle biopsy four years previously had shown ragged‐red fibers. Biochemical studies showed NADH CoQ reductase and cytochrome c oxidase deficiencies in biopsied muscle at 13 years of age, although normal activity of cytochrome c oxidase had been histochemically detected at 9 years of age. A therapeutic trial of citrate administration produced transient improvement in the clinical features and in the hyperlactic and pyruvic acidemia.
Periodic ACTH ADH Discharge Syndrome: Relationship to Inappropriate ADH Secretion
Authors: Ogihara Masaaki , Aritaki Sekoiya
Published: October 2007

The case of a two‐year‐old Japanese girl is presented who had attacks of vomiting, hypertension and somnolence in a regular cycle. During the attacks, she showed increases in serum ACTH, ADH and Cortisol, decreases in plasma osmolality and sodium concentration, and increases in the urinary excretion of 17‐OHCS and 17‐KS. During attack‐free periods, all these findings became normal. Although this case is similar to cases of periodic ACTH ADH discharge syndrome described by Sato and associates [1, 2], certain differences exist:

(1) Chlorpromazine, reserpine and alpha‐methyldopa were effective in Sato's cases but not in ours.

(2) The vomiting attacks occurred in a circadiseptan rhythm (T= 14 days).

(3) A disturbance in ADH‐inhibition was found by an intravenous water loading test performed during an attack‐free period. After administration of pheny‐toin, the vomiting attacks disappeared. Since phenytoin is an inhibitor of ADH, and has been used for treating inappropriate secretion of ADH, it appeared that the vomiting attacks were due to this mechanism. The cyclic vomiting may have been due to an abnormality in the hypothalmus, which governs biological rhythms.

This case suggests that cyclic ACTH ADH discharge has at least two different pathophysiologies.
Accelerated Ventricular Rhythm in a 13‐Year‐Old Male
Authors: Yokota Yoshifumi , Wada Masafumi , Yorifuji Tohru
Published: October 2007

A 13‐year‐old male was found to have accelerated ventricular rhythm, preceded by coryza and pleurisy. Initially, this arrythmia was present about half the time at rest and gradually decreased in frequency over a year. At 15 years of age the patient was asymptomatic.
The Production of Cardiovascular Malformations with Combined Use of Trypan Blue and Anti‐Heart Serum in the Rat
Authors: Ono Seigo , Miyata Koichiro , Ninomiya Makoto , Yamamoto Eiji , Tachikura Junko
Published: October 2007

Small doses of trypan blue (TB) and anti‐heart serum (AHS) were intraperi‐toneally injected into pregnant Wistar rats to produce in the offspring cardiovascular malformations, which in our previous studies had been induced by the combined use of anti‐sera to two tissues. The results showed that TB could produce a high incidence of malformations, though a dose of 10 mg/kg was subteratogenic in rats. AHS alone was not teratogenic even if 9 ml/kg were administered. Combined use of 10 mg/kg of TB (on day 9 of gestation) and 9 ml/kg of AHS (on day 10) produced a statistically significant increase of malformations (p<0.001). The total incidence of malformations and the incidence of those of the cardiovascular system were 91.5% and 83.1%, respectively. Ventricular septal defects and aortic arch anomalies were the main cardiovascular malformations. Localization of TB in both the yolk sac and the developing embryo was comfirmed, though deposition of AHS was not detected. In this study, the incidence of cardiovascular malformations was higher than that achieved in our previous study, which utilized a combination of small doses of anti‐kidney serum and AHS.
A Study of Taurine Transport and Absorption in Placenta and Neonatal Intestine and Kidney
Authors: Moriyama Ikuko S. , Lioka Hideaki , Kato Yumiko , Nabuchi Yoshino , Ninomiya Yuko , Akasaki Masayuki , Ichijo Motohiko
Published: October 2007

Serum taurine concentration in the human fetus (umbilical blood) and neonate was measured from the 24th to 40th week of pregnancy. The concentration was considerably higher than in maternal serum with a peak at the 24th week of pregnancy and then a gradual decrease until it approached the adult level on the 9th day after birth.

To clarify the underlying mechanism of the human findings, the distribution of 14C‐taurine was investigated after intravenous injection to maternal rats on the 20th day of pregnancy. The taurine administered to the maternal side became concentrated in the placenta and reached the fetus. It was confirmed that taurine accumulated in the human placenta was present in a higher concentration than any other free animo acid.

Placental transport of taurine was studied in isolated brush border microvillous plasma membrane vesicles by a rapid filtration technique. The membrane vesicles exhibited uptake of H3‐labeled traurine into an osmotically reactive intravesicular space. Taurine uptake by vesicles was stimulated specifically by an inward sodium gradient, and replacement of NaCl in the transport medium by KCl, LiCl or choline chloride had no effect on the transport activity of the vesicles. Taurine transport was inhibited competivively by the presence of β‐alanine and GABA. The initial rate of transport followed saturation kinetics with respect to taurine concentration: an apparent Km of 0.22 mM and Vmax of 67 pmol/mg protein were calculated in 20 seconds. These results indicate that transport of taurine across the placental brush border membrane is sodium‐dependent and carrier‐mediated.

The taurine transport system in human fetal small intestine, especially in the jejunum, is a passive transport system quite unlike that of other amino acids.

Reabsorption of taurine through the proximal convoluted tubule is by active transport which depends on sodium. This transport system is developed from the 24th to the 32nd gestational week.
Alternating Hyper‐ and Hypothyroidism with Thyroid‐Stimulating and TSH‐Binding Inhibition Immunoglobulins
Authors: Sato Tamotu , Miyagawa Kazuhiko , Igarashi Noboru , Kajiwara Sohei
Published: October 2007

The clinical course of Graves' disease is described in an 8 year old girl with high thyroid‐stimulating immunogloblin (TSI) and thyrotropin‐binding inhibition immunoglobulin (TBII) titers, 6,421% and 87.8%, respectively. She had three cycles of alternating hyper‐ and hypothyroidism during two years of observation with or without antithyroid medication or thyroxine replacement. She also developed a propylthiouracil‐induced lupus‐like syndrome with a positive antinuclear antibody. Although a causal relationship between elevated TSI and TBII titres and hyper‐hypothyroidism was not established, the coexistence of high TSI and TBII titres requires close observation for the proper treatment of this disorder.
Linkage Maps of Human Genes
Authors: White R. , Leppert M. , O'Connell P. , Nakamura Y. , Holm T. , Lathrop G.M. , Lalouel J.‐M.
Published: October 2007

Methodologies and tools for the construction of complete, primary genetic linkage maps of human chromosomes are emerging. Special families, optimal because of family structure for revealing linkage relationships, have been sampled and a number of genotypic determinations gathered. The associated genotypic database and the cell lines are available to the research community through the Centre d'Etude du Polymorphisme Humain in Paris, and form the basis for an international collaboration in the effort to map the entire human genome. Genetic diseases that are known only by their phenotype can be mapped to chromosomes when they co‐segregate with known map markers; localization of a disease gene represents an important step toward its characterization. New markers for hypervariable loci, highly efficient for mapping purposes, are being developed. Primary maps spanning several chromosomes are already in hand.
Genetic Engineering and Medical Ethics
Authors: Mataix Rev. Anselmo
Published: October 2007

In the introduction I express the need of introducing the point of view of Ethics when we are dealing with problems of science, technology, and especially medicine.

In the first part of my presentation I deal with the problem of the value of human life, coming to the conclusion that human life might not be completely absolute, but is extremely important and “ceteris paribus” should be respected, and the problem of when it starts. Some of the main arguments are discussed. In this connection the question of abortion is briefly discussed.

In the second part I deal with some problems related to human reproduction. Most people will be willing to support programmes for treating individuals with genetic disease; but at the same time the dangers of increasing depersonalization of the reproductive process are pointed out.

In the third part I take up the problem of In Vitro Fertilisation. IVF has certainly brought benefits to some couples suffering from infertility, but at the same time it has raised quite a few human and moral problems. Is it permissible to fertilise an egg with a donor sperm, replacing the embryo in the womb? What about fertilising a donor egg with the husband's sperm? Is it acceptable to store or freeze embryos for future use? Is it moral to implant such an embryo in a woman who has no genetic relationship with the embryo? Is it moral to use surrogate mothers? Is it moral for ‘spare’ embryos to be killed or used as tissue for research?

At the end I stress the need of dialogue between life sciences and ethics.
Birth Complications and Psychological Deviancy: A 25‐Year Prospective Inquiry
Authors: Buka Stephen L. , Lipsitt Lewis P. , Tsuang Ming T.
Published: October 2007

Considerable evidence has accumulated during the past 30 years regarding the relationship between pregnancy and delivery complications and a variety of psychiatric and neurologic disorders. Prior studies have suffered from major methodological problems associated with retrospective designs, notably selection and recall biases. We are conducting a 25‐year prospective investigation of perinatal complications and psychiatric outcomes. The study cohort consists of 176 singleton births diagnosed as either “severe toxemia” or “breech delivery” and 176 matched control subjects, all selected from the Providence Center of the National Collaborative Perinatal Project. Extensive obstetrical, neurological, behavioral and socio‐demographic information was collected prospectively for these subjects, from the perinatal period through age seven. Subjects were recontacted (ages 18–24) and admin istered a battery of instruments designed to assess: a) psychiatric diagnosis (using the Diagnostic Interview Schedule); b) cognitive and psychosocial functioning; and c) family history of mental disorders. 85% of the sample have been contacted and 60% successfully interviewed at this time. Preliminary analyses will assess the relative risk associated with each pregnancy complication and the DSM‐III psychiatric diagnoses covered by the Diag nostic Interview Schedule. Further analyses will examine possible etiologic mechanisms which have been proposed to account for such associations.
The Effect of MCT Oil Supplement in Very Low Birth Weight Infants, with Evaluation by the 13C‐Labeled MCT Breath Test
Authors: Yamada Takako , Nishida Hiroshi , Sakamoto Shoichi , Okada Akemi , Sakanoue Masamichi , Suehiro Makiko
Published: October 2007

We studied the efficacy of medium‐chain triglyceride (MCT) as an energy source in premature infants. Infants who were given 3 g/kg/day of MCT oil gained body weight better than the control group in spite of a smaller water intake. This is advantageous to premature infants who need water restriction due to patent ductus arteriosus (PDA), bronchopulmo nary dysplasia (BPD), etc. We also proved that MCT oil is rapidly absorbed and digested, by means of the 13C‐trioctanoin breath test.
The Usefulness of Doppler Echocardiography for the Evaluation of Hemodynamics in Tetralogy of Fallot: Comparison of the Patients with Pre‐ and Post‐Corrective Surgery and Control Healthy Children
Authors: Watari Tohoru , Awa Shoichi , Hishi Toshio , Akagi Michio , Fong Lin I , Nakamura Gen
Published: October 2007

Twodimensional Doppler echocardiographic examinations were performed in 61 patients (39 boys and 22 girls) with tetralogy of Fallot (TOF). The patients were examined at the Department of Pediatrics, University of Tokyo, and at the Department of Pediatrics, Ohta General Hospital, in Koriyama city, Fukushima Prefecture.

One hundred and eighteen healthy children (59 boys and 59 girls) were also examined at the Waseda housing complex in Misato city, Saitama Prefecture.

By short axis view of aorta, samples were collected from both the right ventricular outflow tract below the pulmonary valve and in the main pulmonary artery. On the other hand, the apex four chamber view was used to collect samples at the right and left atrial outflows.

Right ventricular systolic time intervals were measured both at the main pulmonary artery (PA) and at the outflow tract below the pulmonary valve (RVO).

The right ventricular systolic pre‐ejection period (PAPEP) and acceleration time (PAACT), measured at the supra‐valvular site, were shorter before surgery as compared to after surgery and normals. The ratio of right ventricular pre‐ejection period to ejection time (PAPEP/ET) and the ratio of acceleration time to ejection time (PAACT/ET) were both significantly lower before surgery compared to after surgery and controls.

The mean main pulmonary artery peak velocity before surgery was 369 cm/sec and the peak pressure gradient calculated with the modified Bernoulli formula was 55 mmHg.

Before surgery, peak velocity of right ventricular rapid filling (R‐wave) was low, while peak velocity of atrial contraction (A‐wave) was high. After surgery, however, R‐wave increased and A‐wave decreased in velocity. The peak velocity of left ventricular filling R‐wave was also low before surgery and increased after surgery, while peak velocity of left atrial contraction A‐wave was increased before surgery and decreased to almost normal values after surgery. The left ventricular A/R ratio before surgery was also high as a result of low R‐wave velocity. The measured time interval between aortic valve closing sound and left ventricular rapid filling wave (IIA‐R time), and the measured time interval between pulmo nary valve closing sound and ventricular filling wave (IIP‐R time) were both longer before surgery than those after surgery and controls. Consequently, the measured time intervals between rapid ventricular filling flow wave and atrial contraction wave (R‐A time) for both right and left atrial were shorter before surgery than after surgery and controls. A new method for estimation of pulmonary‐to‐systemic blood flow ratio (Qp/Qs) was successfully applied to TOF, and a good correlation was obtained between Qp/Qs determined by this method and that by the Fick method.
Chest Pain in Pediatric Patients
Authors: Fukushige Junichiro , Tsuchihashi Keiko , Harada Tatsuo , Ueda Kohji
Published: October 2007

One hundred and twenty‐three patients (72 males and 51 females) with the chief complaint of chest pain, seen at our department during the five‐year period from January 1982 to December 1986, were studied regarding their past medical history, physical findings, results of diagnostic tests, and prognosis. The ages of the subjects ranged from 3 to 16 years (mean 11.7 years) in boys and 3 to 20 years (mean 10.9 years) in girls, constituting 0.54% of the total number of the mean annual outpatients. Of these 123, 80 patients (65%) had chest pain lasting over one month, and 91 patients (74%) had a short time of duration of pain which improved within a few seconds to five minutes. In nine (7%) of the 123 patients, the pain was considered to be related to cardiovascular lesions and in 82 patients (67%) as being idiopathic chest pain. Chest pain in children is rarely due to serious primary cardiovascular diseases.
Familial Insulin‐Resistant Diabetes Mellitus without Acanthosis Nigricans or Androgenization
Authors: Takumi Toru , Kodama Sochi , Tsubota Toru , Nishio Hisahide , Takahashi Toshikazu , Yokono Koichi , Matsuo Tamotsu
Published: October 2007

A 12‐year‐old Japanese girl and her mother showed insulin‐resistant diabetes mellitus which was confmed by the response to an oral glucose load in both patients and by the small decrease in blood glucose after intravenous insulin in the daughter. Anti‐insulin antibodies and anti‐insulin‐receptor antibodies were not detected in either patient. The circulating erythrocytes were found to have reduced insulin binding capacity, which, to judge from ID50, was probably caused by decreased binding affhty of insulin receptors.

The patients did not have acanthosis nigricans or signs of androgenization such as hirsutism, dysmenorrhea or polycystic ovaries. Endocrinological studies in the daughter showed elevated basal serum levels of luteinizing hormone (LH), dehydroepiandrosterone, testos terone and estrone, and an exaggerated LH response to LH‐RH. It is possible that hyper‐insulinemia caused by insulin resistance contributed to the increased secretion of LH and androgens, but it was unclear why signs of androgenization were absent in spite of increased secretion of LH and androgens.
Gonadotropin Deficiency as a Significant Association of Complex Glycerol Kinase Deficiency: A Case Report with Cytogenetic and Molecular‐Genetic Studies
Authors: Yoshimoto Masaaki , Baba Tsuneyoshi , Fukuda Shinpei , Matsumoto Tadashi , Hayashi Sawako , Niikawa Norio , Matsuda Lchiro , Tsuji Yoshiro
Published: October 2007

A 6‐year‐old Japanese male patient with complex glycerol kinase deficiency (CGKD) is described. In addition to glycerol kinase deficiency, congenital adrenal hypoplasia, Duchenne muscular dystrophy, mental retardation and short stature, gonadotropin deficiency (GTD) was confirmed. The continuous stimulation of LHRH recovered the increment of serum LH and FSH values. Cytogenetic and molecular‐genetic studies revealed a deletion in the Xp21 region which may involve many genes. Although a few cases of CGKD associated with GTD and/or cryptorchidism have been described, the cause of GTD has not been mentioned in the literature. The simultaneous occurrence of CGKD and GTD in these patients including ours may not be coincidental, but suggests the existence of a certain gene in the Xp21 region which may affect the development of hypothalamic LHRH secretory function. Therefore detailed and repeated evaluations of gonadotropin secretion are necessary in patients with CGKD.