bluuebunny/crossref_metadata_2025 huggingface dataset:
Combined Esophageal and Pyloric Atresia without Tracheoesophageal Fistula in Down's Syndrome
Authors: Matsuo Yasutaka , Nukina Sadayuki , Hasegawa Kou , Yoshida Naoko , Doi Yasuo , Sawada Tadashi , Tomioka Megumi
Published: October 2007

This is the first report of a newborn infant with Down's syndrome associated with combined congenital pyloric and esophageal atresias without a tracheoesophageal fistula (Gross A type).

A mother was noted to have marked abdominal distension and hydramios was suspected at 28 weeks gestation. Ultrasonographic findings of her abdomen demonstrated that the fetus was suspected of having congenital duodenal and esophageal atresia, from the large doubleecho‐free spaces occupying the greater part of the abdomen. Chromosome analysis of the amniotic fluid showed a karyotype of 21 trisomy. The newborn baby had clinical characteristics of Down's syndrome and severe abdominal distension after birth.

At 19 days after birth, autopsy revealed pyloric and esophageal atresias without trache oesophageal fistula.

We conclude that ultrasonographic examination is useful for the prenatal diagnosis of a patient with gastrointestinal obstruction combined with esophageal atresias, without trache oesophageal fistula.
Histopathological Study of the Biopsied Muscles from Juvenile Patients with Congenital Myotonic Dystrophy
Authors: Wakai Shuji , Kameda Keiji , Okabe Minoru , Nagaoka Masato , Minami Ryoji , Tachi Nobutada
Published: October 2007

Histopathology and histochemistry were studied in biopsied muscles from eight patients with the congenital form of myotonic dystrophy (congenital MyD) and one patient with the adult form (adult MyD). In the muscle pathology of the four patients aged between 5 and 11 years with congenital MyD, there was no immaturity of the fibers and the histological alterations were minimal. The pathological findings of the adult patient with congenital MyD resembled those of adult MyD. The immature condition of the musculature observed during the early infantile period, therefore, may once improve with motor development during childhood and, after that, the muscle fibers may degenerate in a similar manner to that seen in adult MyD.

Two patients with marked talipes equinovarus displayed grouped atrophy. Small angular fibers and pyknotic nuclear clumps were observed in five patients. These findings suggest that some neurogenic factor might be involved in the muscular changes in this disorder.
Scanning Electron Microscopic Studies on Streptococcal Aerosol Infection
Authors: Yoshinaga Masao , Hokonohara Masashi , Haraguchi Tsutomu , Inoue Hiroyuki , Miyata Koichiro
Published: October 2007

The initial focus of streptococcal invasion and histological changes of rabbit tonsils infected by aerosolinduced streptococci was investigated by scanning electron microscopic studies of the free and fractured surface of the tonsils.

Group A type 12 streptococci were suspended in saline at a concentration of 108 to 109 colony forming units per ml. Rabbits were exposed to the aerosolized suspension for about 120 minutes and sacrificed within 48 hours after the exposure.

The tonsilar epithelium was organized in two different types. The micro‐pores of the crypts were divided into three types. Some interesting findings were observed in the micropores covered by cobblestone‐like cells with micro‐villi or cillia (the third type). That is, the micropore had a trace left by something that had passed. Also, the infiltrating cells appeared numerously after the exposure and continuously from the parenchyma of the tonsils.

These data suggested that the third type micropores play an important role in the initial focus of invasion and in the defense or immunological process.
Effects of Maternal Phenylalanine or Tyrosine Hydroxylase Inhibition on Postnatal Maturation of Catecholamine and Amino Acid Metabolism in Rats
Authors: Sato Tamotsu , Imura Eiichi , Murata Akisato , Minami Satoshi , Okabe Takashi , Ohno Ichiroh
Published: October 2007

Effects of the inhibition of maternal phenylalanine (Phe) or tyrosine (Tyr) hydroxylase on the postnatal maturation of catecholamine, thyroxine and amino acid metabolisms were examined in young rats. The intraperitoneal injections of 100 mg/kg p‐chlorophenylalanine three times during the second gestational week together with 0.2% Phe solution as drinking water produced marked reduction in birth weight, body weight gain, brain weight, cerebral Tyr hydroxylase activity, adrenal epinephrine, renal norepinephrine and free amino acids in plasma and brain as well as delayed maturation of thermoregulation in the offspring. In contrast, the administration of 100 mg/kg α‐methyl‐p‐tyrosine with 0.2% Tyr supplementation did not affect general growth, but only decreased cerebral Tyr hydroxylase and serum thyroxine level, while cerebral dopamine was increased. These results indicate that maternal Phe hydroxylase inhibition induces more severe growth failure including catecholamine and amino acid metabolism than Try hydroxylase inhibition in postnatal development.
Benign Childhood Epilepsy with Centro‐Temporal Spikes: Evolution of Seizure Types
Authors: Ishikawa Tatsuya , Nakazato Mikiko , Awaya Atsuko , Kanayarna Manabu
Published: October 2007

Evolution of the seizure types according to age in 20 outpatients with benign childhood epilepsy with centro‐temporal spikes (BECT) was studied. BECT satisfied the following criteria: 1) normal intelligence, 2) central and/or mid‐temporal spikes with normal background activities on the interictal EEG recordings, and 3) partial and/or generalized seizures in sleep. The patients were divided into two groups according to the clinical course; P (n = 10): patients with only (n = 6) or predominantly (n = 4) partial seizures, G (n = 10): those with only (n = 7) or predominantly (n = 3) generalized seizures. No difference in the age at onset was noted between the two groups. There were also no indications that the seizures tend to generalize at a younger age or remain partial at an older age. Therefore, age is not important in the evolution of partial to generalized seizures in BECT.
Ultrasonographic Findings in Periventricular Leukomalacia in the Newborn: Two Cases Associated with Early Onset Group B Streptococcal Sepsis
Authors: Ogino Takatoshi , Kanda Yasushi , Kawakita Akira , Yamagrchi Nobuyuki , Futamura Masahide , Goto Haruo
Published: October 2007

The ultrasonographic findings in periventricular leukomalacia (PVL) in the newborn are described, and the relationship between PVL and group B streptococcal (GBS) infection is discussed. Two newborn infants (one preterm and one term) suffered from early onset GBS sepsis with shock; they showed increased echogenicity in the periventricular regions; one of them developed cystic changes. These findings might be due to decreased perfusion of the periventricular end arterial zone. It is suggested that serial ultrasonography should be performed in neonates who suffer hypoxic‐ischemic brain injury.
Outcome of Therapy of Hereditary Tyrosinemia
Authors: Halvorsen Sverre , Kvittingen Eli‐Anne , Flatmark Audun
Published: October 2007

We diagnosed 21 patients with hereditary tyrosinemia in Norway; 14 of them had the diagnosis confirmed by enzyme studies. Five died in infancy before dietary treatment was introduced. Six developed hepatoma, between the ages of 3 ½‐ 20 years, three of them while on dietary treatment. Three died from causes other than hepatoma. Of the seven still alive at the time of writing, two received liver transplants, and one a kidney transplant, three are receiving dietary treatment and one is being managed without a special diet.

Dietary treatment may be life‐saving in acute cases. It improves the general condition in the chronic forms, improves tubular dysfunction and growth and may postpone develop ment of hepatoma. Liver transplantation is the only curative treatment, but difficulty remains in deciding the optimum time for transplantation; it should ideally be performed before hepatoma develops.
Influence of Fatty Diet on Neonatal Jaundice in Breast‐Fed Infants
Authors: Amato M. , Berthet G. , Muralt G. Von
Published: October 2007

We studied milk samples from mothers of breast‐fed full‐term infants with “physiologic jaundice” and compared them with milk samples of healthy breast‐fed control babies to investigate if the percentage of cream or “Creamatocrit” could be related to neonatal hyper‐bilirubinemia. The results of this study confirm our hypothesis: a statistically significant difference was found between the two groups of patients (χ2 ‐test, p < 0.002).
Ring Chromosome 7: Report of a Case
Authors: Kohyama Jun , Watanabe Seiichi , Nakajima Masaki , Suzumura Hirosi , Ishikawa Toshihiko , Ishikawa Kiyoto , Saito Fumiko , Fukuda Chikao.
Published: October 2007

A 5‐month‐old girl with a ring chromosome 7 [46, XX, r(7) (p22q36)I is presented. She was the fifth and youngest patient with this chromosomal anomaly, and the first instance whose chromosomal breakpoints were determined by high resolution banding analysis, Numerous café‐au‐lait‐like pigmentation spots and hypoplasia of the thenar creases were observed.
Phosphorus Deficiency Syndrome in Two Very Low Birthweight Infants
Authors: Tsukahara Hirokazu , Sakaguchi Tadahiko , Mitsuyoshi Izuru , Fukuhara Hitoo , Hayashi Shuhei , Nakamura Katsuji
Published: October 2007

Biochemical and radiographic abnormalities linked with phosphorus deficiency syndrome (PDS) developed in two very low birthweight (VLBW) infants fed exclusively with human milk. By increasing phosphorus (P) and calcium (Ca) intakes with the introduction of a specialized premature formula, osteopenia or rickets, as well as hypophosphatemia, hypo‐phosphaturia and increased serum alkaline phosphatase levels improved in both patients. We speculated that all VLBW infants who are being fed exclusively with human milk should be monitored for PDS and that if PDS develops, supplementation of human milk with both P and Ca appears to be necessary for its treatment.
Bone Evaluation by Microdensitometry in Patients with Juvenile Reumatoid Arthritis
Authors: Takei Syuji , Hiwatari Mihoko , Imanaka Hiroyuki , Hokonohara Masashi , Miyata Koichiro
Published: October 2007

To evaluate the hand bones of patients with juvenile rheumatoid arthritis (JRA) objectively, 30 patients with JRA were examined by microdensitometric analysis (MD). The results were as follows: 1) Among the nine indices of MD, %σGS/D, reflecting the bone mineral content, correlated well with the stage of JRA determined by roentgenological findings of the hand bones. 2) %σGS/D showed remarkable improvement after the remission of JRA. 3) Rheumatoid factor (RF) positive JRA showed statistically low %σGS/D compared with RF negative JRA, and kept a low %σGS/D for a long period from the onset in contrast to RF negative JRA. 4) %σGS/D in the patients with JRA treated with steroid decreased more rapidly according to the dosage of steroid than in the patients with SLE. Based on these results it is suggested that MD seems to be useful in evaluating the bone objectively, and not only steroid but also RF seems to be a factor leading to bone destruction in patients with JRA.
Mothers' Attitudes towards Children's Three‐Year Health Examination
Authors: Nishi Motoi , Akatsuka Tomohiro , Inoue Yuko , Kamasaki Hotaka , Kamibayashi Yoshihito , Nakamachi Satoko , Hamaoka Shuji , Hayashi Eri , Miyake Hirotsugu , Takasugi Nobuo , Sato Yasumasa , Hanai Junji , Nagasaka Mina , Saeki Yoshito , Takeda Takeo
Published: October 2007

In the two health centers of Sapporo City, 112 mothers whose children participated in a three‐year health examination, and 107 mothers whose children did not, were interviewed about their children's health.

More time and expense were necessary for the latter group to come to the health centers. Some children did not attend because of apparent indifference of their mothers to health matters. In both groups there was a tendency for attendance for vaccinations and health examinations to decrease as the child's age advanced, especially after four months.

For the purpose of increasing the number of children who receive a three‐year health examination, provision of appointments extending over several days with extra examinations in places outside the usual health centers, in addition to making available detailed information on the examination, seem to be effective.

A considerable proportion of mothers accepted re‐screening for neuroblastoma, for which most could afford 1,000 yen.
Virological, Serological and Molecular Analysis of a Case with Congenital Cytomegalovirus Infection
Authors: Eizuru Yoshito , Takahashi Hirohiko , Ueno Ichiro , Minamishima Yoichi
Published: October 2007

Cytomegalovirus (CMV) was isolated concurrently from an infant and her mother on the 2nd day after delivery. Restriction endonuclease cleavage analysis of viral DNAs revealed that these two CMVs were identical. Sera from the infant and mother were both positive for IgM specific to CMV and antibody against early antigen (EA). Thus, virological, serological and molecular analysis indicated the transmission of CMV from a mother to her fetus in utero.
Isolated Tricuspid Valve Endocarditis in a Child with a Normal Heart
Authors: Matsuoka Yuji , Yamami Nobuo , Nishiguchi Toshihiro , Hayakawa Kunio
Published: October 2007

Infective endocarditis localized in the tricuspid valve was observed in a previously completely healthy 10‐yeardd male. The pathogen was identified as Staphylococcus uureus. Twodimensional and Doppler echocardiography revealed rupture of the chordae tendineae and prolapse of the tricuspid septal leaflet with tricuspid regurgitation. Isolated infective tricuspid valve endocarditis is mostly confined to drug addicts, and, to out knowledge, the literature contains only four cases of this disease in children without heart disease. The present case is considered to represent an extremely rare condition.
Effects of Aldosterone and Betamethasone on Na‐K‐ATPase Activity in the Juxtamedullary Proximal Convoluted Tubules of Mice
Authors: Furuse Akio
Published: October 2007

An attempt was made to determine whether or not the steroid hormones, aldosterone and betamethasone, increased the Na‐K‐ATPase activity and the surface density of baso‐lateral cell membranes in the juxtamedullary proximal convoluted tubules of young and adult mice. The results showed that aldosterone and betamethasone increase the enzyme activity in young mice but not in adult mice. However, the surface density of the baso‐lateral cell membranes did not increase either in the young or in the adult mice. Further, the induction ratio for the Na‐K‐ATPase activity did not differ between the two hormone‐treated groups in young mice. The results suggest that a difference in hormone sensitivity exists between the proximal convoluted tubular cells of young mice and those of adult mice.
Exercise‐Induced Ventricular Arrhythmia after Repair of Tetralogy of Fallot
Authors: Fukushige Junichiro , Shimomura Kunihisa , Harada Tatsuo , Fukazawa Mitsuru , Ueda Kohji , Tokunaga Kouichi
Published: October 2007

Treadmill exercise electrocardiography (TE) was recorded in 50 patients to evaluate the incidence of exercise‐induced ventricular arrhythmias (VAs) in a group of patients who underwent corrective surgery of tetralogy of Fallot (TF) more than four years previously. The results of rhythm evaluation were correlated with cardiac catheterization data as well as clinical information such as the age at surgery and the interval from surgery. Of the 50 patients tested, ventricular premature contraction (VPC) was confirmed in 12 patients (24%) on TE, while five patients (10%) demonstrated VPC on the standard ECG. The interval from surgery was significantly longer in those patients with VA (Group 1) than those without VA (Group 11) on TE (p<0.05). There was no significant difference of the age at corrective surgery between the two groups. In group I, the preoperative hemoglobin level was higher (p<0.05) and the right ventricular ejection fraction was lower (p<0.01). Exercise‐induced VAs are closely related to the length of period after surgery and the depressed right ventricular function.
Inherited Deficiency of the Seventh Component of Complement: Studies of C7‐Consuming Activity
Authors: Koitabashi Yasushi , Ikoma Masaaki , Miyahira Tsuneko , Shibawaka Tsuguo , Yamaguchi Yoshiya , Baba Akiko
Published: October 2007

Neither the hemolytic activity nor the protein level of the seventh component of serum complement (C7) was detectable in an 8‐year‐old girl with nephritis, but in her parents and her brother, they were about half of the normal level. The patient was a homozygote type with a complete deficiency of C7 while her parents and brother were all heterozygote type with a partial deficiency of C7. C7consuming activity was demonstrated in the native serum of the patient with complete C7 deficiency, and it was found that large amounts of C56 were readily generated upon incubation of the patient's serum with zymosan. It is proposed that the C7consuming activity in the native serum of this patient is due to small amounts of C56 generated during the activation of serum complement by some kind of infection such as a common cold.
Modulation of Immunological Abnormalities of Growth Hormone‐Deficient Children by Growth Hormone Treatment
Authors: Matsuura Mikio , Kikkawa Yumio , Kitagawa Teruo , Tanaka Shingo
Published: October 2007

We studied the immunomodulatory role of growth hormone (GH) treatment in GH‐deficient children. CH potentiated natural killer (NK)cell activity and the PHA and Con‐A lymphocytoproliferative response. Two‐color fluorescence using monoclonal antibodies (CLM, 2H4, CD8 and CD11) showed a moderate reduction of the helper T cells and NK cells, but no changes of suppressor T cells or cytotoxic T‐cells during GH therapy. Cancer and slow viral infection in GH‐deficient children have been watched for carefully before and after GH therapy.
Transcutaneous Bilirubinometry in Normal Japanese Infants
Authors: Yamauchi Yoshitada , Yamanouchi Ltsuro
Published: October 2007

We performed TcB readings in healthy full‐term breast‐fed Japanese infants from birth to seven days, and attempted to establish the normal range. TcB readings from Japanese infants were significantly higher over a longer period compared with Caucasian infants. The age of peak TcB readings in Japanese newborn infants was day 6, significantly later than that of Caucasian infants, day 3–4. We also attempted to estimate the total serum bilirubin concentrations using regression line relating TcB readings to serum bilirubin concentrations. Our study demonstrated that estimated total serum bilirubin concentration from forehead TcB readings was 0.56 ± 0.35 mg/dl at birth, thereafter increasing to 6.8 ±0.5 mg/dl on day 1 and reaching a maximum of 12.6 ± 2.5 mg/d on day 6.These values and pattern in Japanese neonatal jaundice were significantly different from those of Caucasian children, but were similar to values and patterns from American Indians, Alaskan Eskimo, and other Asian full‐term newborn infants. Thus TcB measurement may be useful for observation of the course of neonatal jaundice.
Acute Lymphoblastic Leukemia in a Patient with Pituitary Dwarfism under Treatment with Growth Hormone
Authors: Hara Toshihiro , Komiyama Atsushi , Ono Hiroshi , Akabane Taro
Published: October 2007

An 18‐year‐old male with pituitary dwarfism, who had been on replacement of growth hormone (GH) and thyroxine for 3.5 years, developed acute lymphoblastic leukemia (ALL). The GH replacement was discontinued, and he was treated with a conventional protocol for ALL. A complete remission was obtained after 10 weeks. Maintenance chemotherapy was given with reduced doses (1/4 to 1/2) of cytotoxic drugs. The platelet count soon reached 200,000/μL, but the hemoglobin level and white blood cell count improved only slowly, reaching 10.0 g/dL and 1,500/μL, respectively, after five months. He has been in complete remission with a hypocellular bone marrow for nearly 15 months. Since GH can stimulate the proliferation of some normal and leukemic hemato‐lymphoid cells, the slow remission induction and the prolonged anemia and leukocytopenia after remission, may have been related to the absence of GH in this patient.
Effects of Drugs and Fatty Acids Related to Reye Syndrome on Brain Water Content in Rats
Authors: Katafuchi Yukihiko , Yoshida Ichiro , Yamashita Fumio , Sinniah Daven
Published: October 2007

Brain water content was measured by gaschromatography in rats following intraperitoneal injection of drugs and fatty acids related to the etiology of Reye syndrome. A statistically significant increase in brain water content was observed following injection of 5% glucose solution, valproic acid, acetyl salicylic acid, calcium hopantenate, margosa oil, 4‐pentenoic acid, linolenate and arachidonate. Seizures occurred in all animals given valproic acid, margosa oil and 4‐pentenoic acid, and in 25% of those given 5% glucose solution + antidiuretic hormone. The results of these studies may help in the selection of appropriate agents for experimental induction of acute encephalopathy and brain edema in animal models of Reye syndrome. (Acta Paediatr Jpn 1989; 31:115–119)
Analysis of Urinary and Circulating EDP Subfragments and Subunits: Application of the Western‐Blot Technique
Authors: Takahashi Shori , Noguchi Yasuyuki , Kitagawa Teruo
Published: October 2007

We applied the Westem‐blot technique for qualifying fibrin/fibrinogen degradation products (FDP) subfragments and subunits. With this technique we examined urine or serum samples from patients with glomerulonephritis and disseminated intravascular coagulation (DIC), in order to observe how primary or secondary fibrinolysis functioned under these pathological conditions. We also tested the antigenic recognition of several FDP antibodies with this technique. The results obtained were as follows: 1) FDP in serum samples from patients with DIC consisted of both fibrinogenolytic and fibrinolytic products; 2) Fibrinolysis was predominant in the serum from one patient with HenochSchonlein purpura nephritis, and its level increased after fibrinolytic therapy with urokinase; 3) Fibrinogen and fibrin polymers were always the major components of urinary FDP, although fibrinolytic products such as subfragment D‐D dimer were detected in patients with glomerular disease and increased in acute exacerbation; 4) Anti‐FDP D‐D dimer monoclonal antibody (DD 3B6), which is usually considered to react specifically with cross‐linked fibrin derivatives, also seemed to have cross‐reactivity with nonnosslinked fibrin derivatives, (Acta Paediater Jpn 1989; 31: 127–135)
Chemoattractant Receptors of Neonatal Polymor‐phonuclear Leukocytes: Lack of Modulation of the Receptors by Membrane Modifier
Authors: Yasui Kozo
Published: October 2007

To analyze a defect(s) in chemotactic responsiveness of human neonatal polymorphonuclear leukocytes (PMN), PMN functions and their chemoattractant receptor properties were examined using N‐formylmethionyl‐leucyl‐phenylalanine (FMLP). Amphotericin B (AmB) is known to affect human PMN membrane and to alter cellular functions. Although the AmB treatment decreased the FMLP binding and cellular movement in adult PMN, there were no such changes in neonatal PMN. Kinetic studies of 3H‐FMLP binding to PMN, furthermore, indicated the poor internal transportation of the chemoattractant into neonatal PMN. These abnormalities, which are related to the chemoattractant receptors, may explain in part the defective chemotaxis of neonatal PMN. (Acta Paediatr Jpn 1989;31: 150–157)
Restriction Fragment Length Polymorphism Analysis in Healthy Japanese Individuals and Japanese Families with Gaucher Disease
Authors: Masuno Mitsuo , Orii Tadao , Sukegawa Kazuko , Taga Toshiaki
Published: October 2007

We analysed the restriction fragment length polymorphism (RFLP) of 30 unrelated, healthy Japanese individuals and the members of seven Japanese families with Caucher disease (5 Type I, 2 Type III) with the cDNA for human glucocerebrosidase, to investigate the frequency of the RFLP among healthy Japanese individuals and to determine the feasibility of the use of this probe DNA for carrier detection and prenatal diagnosis in Japanese families with Caucher disease. In the Pvu II polymorphism, the common allele reported by Sorge et al [1] was the rare allele for healthy Japanese individuals. The frequency of the common allele (+) in Japanese was 0.75 and the rare allele (‐) was 0.25. The RFLP study was informative for carrier detection in the family in which both parents were heterozygous for the Pvu II polymorphism. Even in the family in which only one parent was heterozygous, carrier detection was feasible by combining the clinical history and the RFLP, although prenatal diagnosis was not feasible. The Kpn I polymorphism was not detected. It is necessary for precise carrier detection and prenatal diagnosis to investigate other restriction endonucleases which reveal the RFLP. (Acta Paediatr Jpn 1989; 158 ‐ 162)
Mother's Attitudes towards the Mass Screening of Neuroblastoma
Authors: Nishi Motoi , Miyake Hirotsugu , Takasugi Nobuo , Sato Yasumasa , Hanai Junji , Takeda Takeo
Published: October 2007

Two hundred and two mothers whose, infants participated in mass screening of neuroblastoma in Sapporo City, and 200 mothers whose infants did not, were interviewed about their attitudes towards the mass screening. Only a few nonparticipants (3.5%) thought the mass screening was unnecessary. More than half of non‐participations (56.0%) were due to mothers' forgetfulness or to loss of the “Neuroblastoma Screening Examination Set” during the two months' interval between the delivery of the set and the sample submission. Mailing of the set at six months for the purpose of eliminating this interval would greatly reduce such neglect of the mass screening. On the other hand, not many infants would participate in re‐screening for detection of false negative cases, since the percentage of mothers who were willing to make their children take part in rescreening for a fee of 1,000 yen (about 7 U.S. dollars, which would be administratively necessary) was less than 60%. (Acta Paediatr Jpn 1989; 31: 166–170)
Prevention of Vertical Transmission of Hepatitis B Virus by Yeast Recombinant Hepatitis B Vaccine
Authors: Murata Ryosuke , Isshiki Gen , Yoshioka Hajime , Chiba Yasuo , Tada Hiroshi , Koike Michio , Kimura Mikio
Published: October 2007

In order to prevent vertical transmission of hepatitis B virus (HBV), yeast recombinant HB vaccine at a dose of either 5 mcg or 10 mcg was administered to 185 infants born to mothers who were positive for HBs antigen (HBsAg). All of them developed antibody to HBsAg (anti‐HBs) after three vaccinations. Generally, the cutaff index (COI) of anti‐HBs by radioimmunoassay (RIA) was higher in the 10 mcg dosage group than in the 5 mcg dosage group. The geometric mean titer (GMT) of anti‐HBs, as measured by quantitative RIA, in the former group was 2.4 times that in the latter one month after the third vaccination. The incidence of clinical reactions was only 4% in a total of 561 injections, and none of the reactiom were severe. It is conduded that recombinant HB vaccine is safe and has excellent immunogenicity for infants requiring prevention of HBV vertical transmission. It is also suggested that 10 mcg doses of the recombinant HB vaccine can provide more solid protection to high‐risk infants without serious adverse reactions. (Acta Paediutr Jpn 1989; 31: 180–185)
Five‐Year Clinical Course of Aortic Regurgitation Without Coronary Aneurysm Following Kawasaki Disease
Authors: Yarnasaki Shirou , Arnarnoto Yuuhei , Irnarnura Hajirne , Tsuji Yoshirou
Published: October 2007

Kawasaki disease has recently been considered as a major cause of acquired valvular diseases in infants and children. But there have been few reports on the clinical course of the lesions caused by this disease, particularly aortic regurgitation. In February 1982, we examined a six‐month‐old male infant who had Sellers third degree aortic regurgitation (AR) and Sellers first degree mitral regurgitation (MR) without discernible dilatation, aneurysm formation or stenosis of the coronary arteries, and in January 1987 we performed a second cardiac catheterization in order to estimate the progress of these lesions. The results were: 1) His AR showed no apparent worsening; 2) His MR had completely recovered; 3) His volume and pressure studies were almost normal. The catheterization data were compatible with those of other clinical examinations. He no longer had cardiac enlargement as seen on a chest roentgenogram, and there were no signs of left ventricular hypertrophy on an electrocardiogram (ECG). (Acta Paediatr Jpn 1989; 31: 198 ‐ 204)
Two Cases of Cystic Fibrosis in Japanese Children: Studies on the Essential Fatty Acid and Prostaglandin Metabolism
Authors: Igarashi Noboru , Hashimoto Hiroyuki , Hiratani Michio , Taniguchi Masashi , Kasahara Yoshiro , Sato Tamotsu , Taniguchi Noboru , Funabashi Takashi , Okuda Norihiko , Yamashiro Yuichiro
Published: October 2007

We describe the fatty acid (FA) and prostaglandin (PG) metabolism in two Japanese cases of cystic fibrosis (CF) with or without pancreatic insufficiency (PI). The diagnosis of CF was based on the elevated sweat chloride concentration by pilocarpine iontophoresis. A 1‐month‐old boy (case 1) showed poor weight gain, steatorrhea and scaly dermatitis, but no respiratory symptoms were noted. He had decreased levels of serum linoleate and arachido‐nate, and increased palmitoleate and oleate levels, indicating essential fatty acid (EFA) deficiency. Supplementation of fat‐emulsion improved his skin lesions and the altered FA pattern within a few months, associated with the definite reduction of the urinary PC Fza levels. Until two years of age, he has been free from respiratory symptoms. A 12‐year‐old girl (case 2) had had recurrent respiratory tract infections due to Pseudomonas ueruginoso and Staphylococcus aureus for several years, and her pancreatic functions were preserved. The FA patterns of her serum lipid were almost within the normal range. These results indicate that 1) the altered FA composition appeared to be a secondary consequence of PI commonly complicating CF and 2) the correction of the altered FA and PC metabolism might have a beneficial effect on the respiratory function of CF patients with EFA deficiency.
Transient Myocardial Thickening in Acute Myocarditis—Serial Study by Two‐Dimensional Echocardiography
Authors: Matsuoka Yuji , Kitamura Kumiko , Nishiguchi Toshihiro , Hayakawa Kunio
Published: October 2007

A 13‐year‐old girl developed viral myocarditis complicated by pulmonary thromboembolism. Marked dilatation and reduction in the contractility of the left ventricle were noted in a two‐dimensional echocardiogram on admiwion. The thicknesses of the left ventricle and the interventricular septum were both 8 mm at this time, but increased after the 9th day of the illness to reach 2 cm by the 15th day, with a concomitant reduction in the left ventricular cavity. The myocardial thickening gradually decreased and the ejection fraction improved on serial echocardiographic evaluations, and the wall thickness and ejection fraction returned to normal by the 32nd day. The myocarditis resolved without sequelae. The implications of this transient myocardial thickening are discussed. (Acta Paediatr Jpn 1989; 31: 216 ‐ 221)
Clinical Study of Epileptic Children with Frequent Seizures
Authors: Horita Hideki , Nozaki Hidetsugu , Nara Takahiro , Maekawa Kihei
Published: October 2007

The subjects were 98 children with an average age of 5.8 years. The frequent seizures were categorized into the following three modes: (1) seizures occur more than five times a day; (2) 1 ‐4 seizures occur every day for one week or more; (3) 1 ‐4 seizures a day occur weekly for one month or more. The incidence of each mode was as follows: (1) 15.3%; (2) 16.3% (3) 173% (1) + (2) 43.9% etc. We observed frequently the cases of tonicclonic seizures in mode (l), infantile spasms (IS) in mode (2), simple partial seizures (SPS) in mode (3), and minor seizures, IS, and Lennox‐Gastaut syndrome (LCS) in mode (1) + (2). Abnormal CT findings were observed in 37.8%. Of all the patients, 33.7% indicated a developmental quotient (DQ) or intelligence quotient (IQ) above 80, and 31.6% showed a DQ or IQ under 30. Of 96 patients followed for the past one year or more, 32.3%, consisting mainly of the cases with absence seizures and IS, had been seizure‐free for at least one year; 60.4% including the cases with LGS, SPS, etc., still had seizures.
The Probable Mechanisms of Brain Damage and Epilepsy in Febrile Convulsions, Singapore‐ Syndrome and Reye's Syndrome
Authors: Aiyathurai Eric J. , Boon Wong Hock
Published: October 2007

Current knowledge suggests integration of cerebral perfusion and metabolism as enabling normal neuronal function, and their pertubations explaining the brain damage of hypoxia, hypoglycaemia, hypoperfusion and status epilepticus. Similar mechanisms appear operative in the viral encephalopathies and cause psychomotor dysfunction and epilepsy. A transient inhibition of plasma membrane glucose transport is central to the understanding of the metabolic abnormalities of these encephalopathies, the ensuing cell energy crisis resulting from neuroglycopoenia being evidenced by electroencephalographic changes, lactic and ketoacidosis, hyperuricaemia and ionic aberrations. Failure of Na+ and Ca2+ pumps cause cerebral oedema and neuronal death respectively, the selective nature of the latter being due to α‐adrenergic vasoconstriction. Management with hyperglycaemia‐producing infusions and the judicious use of lactate and steroids can overcome the transport dysfunction and enable complete recovery. The temporal profile of the metabolic aberrations of febrile convulsions, which are the result of adaptation, provide a template supporting this mode of management of the severe encephalopathies.
Developmental Retardation in Children with Refractory Epilepsy
Authors: Kurokawa Toru , Akazawa Kouhei , Sakamoto Kanji , Hlaeda Yasufumi , Kitamoto Ikuko , Nose Yoshiaki , Ueda Kohji
Published: October 2007

It is controversial whether developmental retardation occurs in children with epilepsy and what causes the retardation if it occurs. The present study aims to reveal the factors associated with the retardation. The patients were 126 children who had been developmentally normal before the onset of epilepsy and had been followed for more than five years. Fortyeight of the 126 children showed mental retardation, and the remaining 78 had normal mental development at the follow‐up. Developmental retardation was observed in children with onset before one year, known etiology, diffuse slow spike‐wave or hypsarrhy‐thmia on EEC, ingestion of six or more drugs and high blood levels of phenobarbital or phenytoin. Late onset of epilepsy, idiopathic or familial etiology, ingestion of five drugs or less and low blood level of phenobarbital or phenytoin were associated with normal development.
Leukotriene C4 in Children with Atopic Asthma II. Plasma Levels in Bronchial Challenge with Specific Allergen
Authors: Iwasaki Eisaku
Published: October 2007

Peptide leukotrienes C4, D4 and E4 are considered to be major mediators of hypersensitivity reactions. In this study, immunoreactive leukotriene C4 (iLTC4) in plasma, taken from asthmatic children who had undergone bronchial challenge with a specific allergen, was measured using radioimmunoassay in order to determine whether LTC4 is released in duo during human allergic reactions. With regard to the bronchial challenge of 10 asthmatic children, plasma iLTC4 levels at the postchallenge stage (0.131 ± 0.037 pmol/ml) were significantly elevated compared to the prechallenge stage (0.065 ± 0.016 pmol/ml) (p <0.01). The bronchial response was not directly related to the increased plasma iLTC4 levels, but the strength of the bronchial response did relate to the plasma iLTC4 levels at the pre‐challenge (p<0.05) and postchallenge (p<0.01) states. The prechallenge levels are relevant to bronchial reactivity, and the post‐challenge levels are relevant to bronchocon‐striction caused by exposure to allergens. The in vivo release of LTC4 following exposure to a specific allergen suggests that leukotrienes are important in the pathogenesis of asthma.
Breast‐Feeding from the Viewpoint of Maternal and Child Health Education
Authors: Yanai Ben
Published: October 2007

Seeing the changes in breast‐feeding and the sources of advice on that knowledge, I make reference to the necessity of breast‐feeding education and point out the problems that we must take into account, in the case of carrying forward breast‐feeding education.
Possible Biological Growth Factors in Breast Milk and Postnatal Development of the Gastrointestinal Tract
Authors: Yamashiro Yuichiro , Sato Mitsuyoshi , Shimizu Toshiaki , Oguchi Satoshi , Maruyama Keiko , Kitamura Shigehiro
Published: October 2007

To investigate as to whether or not biological growth factors known to be present in natural milks could influence postnatal development of Bstrointestinal (GI) tract, tests were made to determine the enteric mucasal, protein and DNA contents, alkaline phosphatase and disaccharidase activities in mongrel puppies at birth and after four days of both mother‐reared and artificial reared. Microvilli of the jejunal segment were also investigated histologically by electron microscopy. Similar increases in body weight over the first four days of life were obtained and mucosal protein and DNA contents in the small intestine were greater in the mother‐reared animals than in the newborn animals but neither mucosal protein content nor DNA content of the artificially reared animals was different from that of the newborn animals. Alkaline phosphatase activity was greater in both the mother‐reared and artificially reared animals than that of the newborn animals. The disaccharidase activities were not different among the three groups. The jejunal microvilli of the mother‐reared animals were more elaborately grown in the structure than those of the artificially reared or newborn animals. Therefore, this study demonstrated that the mother rearing over the fmt four days of life resulted in acceleration of the enteric mucosal growth, and the result indicates that breast feeding plays an important role in the development of the GI tract during the neonatal period.
Milk‐Borne Transmission of HTLV‐I as a Major Route in the Endemic Cycle
Authors: Hino Shigeo
Published: October 2007

Mother‐to‐child transmission of HTLV‐I as a major route in the endemic cycle of HTLV‐I was established by epidemiologic evidence that (1) 22% (17=78) of children of HTLV‐I carrier mothers were themselves carriers, in contrast to approximately 1% of the young age population of the same area, (2) more than 95% (23/24) of mothers of carrier children were themselves carriers, and (3) the product of the prevalence of carrier mothers and the incidence of carriers in children born to them corresponded well to the prevalence of carrier children in the same area. Intrauterine infection was not likely, since none of over 200 cord bloods of babies born to carrier mothers showed infection markers, such as IgM antibody or viral antigens. The possibility of milk‐borne transmission was supported by (1) the presence of sufficient numbers of infected T‐cells in the milk of carrier mothers, (2) the fact that a common marmoset was found to be a carrier after oral administration of the milk of carrier mothers, and (3) a retrospective analysis which revealed that none of nine babies fed only compound milk were carriers. An ongoing intervention study showed that none of 47 babies whose mothers refrained from breast feeding had seroconverted at 12 months of age. Compound milk feeding by carrier mothers seems to be an effective measure to reduce dramatically the infection rate of HTLV‐I.
Concurrent Poststreptococcal Acute Glomerulonephritis and Schonlein‐Henoch Purpura
Authors: Onisawa Shin , Morishima Naoya , Lchimura Tohju
Published: October 2007

A 5‐year‐old Japanese boy developed concurrent poststreptococcal acute glomerulonephritk (PSAGN) and Schonlein‐Henoch purpura (SHP). An elevated titer of ASK on admission confiied the preceding streptococcal infection. Arthritis of the left knee and petechiae on admission were regarded as features of SHP. The presence of SHP was further confiied by the pathological fmding of leukocytoclastic vasculitk in the skin. PSACN was strongly suspected due to the sndings of microscopic hematuria and hypocomplementemia in the acute phase. The concwrence of SHP and PSAGN suggests similar underlying pathophys iological processes as poststreptococcal sequelae. At the height of the illness, peripheral blood lymphocyte subset analysis showed a marked increase in the suppressor inducer T subset and a reciprocal decrease in the helper T subset. lhis alteration in T lymphocyte subsets was regarded as indicative of the immunological derangement in this patient.
Congenital Anterior Urethral Diverticulum and Recurrent Urinary Tract Infection
Authors: Igarashi Takashi , Higashihara Eiji
Published: October 2007

A patient with congenital anterior urethral diverticulum is described. He showed recrent lower urinary tract infection, but he did not have severe upper urinary tract abnormalities. One‐stage resection and urethroplasty were successfully performed before the upper urinary tract change developed. Anterior urethral diverticulum should be considered as a cause of recurrent lower urinary tract infection in young children.
Autogenic Training as an Effective Treatment for Reflex Neurovascular Dystrophy: A Case Report
Authors: Kawano Motoko , Matsuoka Motoko , Kurokawa Toru , Tomita Shigeru , Mizuno Yuji , Ueda Kohji
Published: October 2007

A 15‐year‐old girl complained of swelling and shooting pains in the right upper extremity, which had bothered her for seven months. Physical examination revealed swelling, cyanosis, weakness and hyperesthesia over the entire right upper extremity. Serological and biochemical data were within nod limits. She was diagnosed as having reflex neurovascular dystrophy (RND). Psychological problems with school and her family might have contributed to the pathogenesis of the disease. With autogenic training (AT), remission was obtained within eighteen months.
B‐Cell Function in Kawasaki Disease and the Effect of High‐Dose Gamma‐Globulin Therapy
Authors: Kawamori Juzou , Miyake Takeshi , Yoshida Takami
Published: October 2007

We studied in vino B‐cell function in Kawasaki disease (KD). By plaque‐forming assay, IgG‐, IgA‐ and IgM‐secreting cells in the first week of KD were markedly increased, and recovered to a normal level in the second week in many cases. Lymphocyte blast formation with Staphylococcus aureus Cowan I (SAC), a B‐cell‐specific mitogen, was suppressed in the acute phase, and recovered to a nod level in the convalescent phase. By flow cytometry, HLA‐DR‐ and HLA‐DQ‐positive cells were increased in the acute phase of KD. CD3‐and CD4‐positive cells were also decreased. CD8‐positive cells showed no significant change. In five patients, CD4‐positive cells with HLA‐DR positivity neither increased in the acute phase nor changed during the course of illness. From our results, it can be considered that pathogenic microorganisms or toxins activate B cells directly in KD without the association of T cells. We also studied the effect of high‐dose gamma‐globulin therapy on B‐cell function in KD. However, the results indicated that this form of therapy had no significant effect on B‐cell functions.
High‐Dose Immunoglobulin for Juvenile Myasthenia Gravis
Authors: Maruyama Youichirou , Takeshita Seiichirou , Sekine Isao , Yoshioka Shigetake
Published: October 2007

A four‐year‐old girl with the ocular type of myasthenia gravis was effectively treated with intravenous high dose human immunoglobulin. Since conventional anticholine‐esterase drugs failed to induce a satisfactory outcome and the patient continued to suffer from am‐blyopia, highdose immunoglobulin was tried according to the method of Gados. Sub* quently, she became asymptomatic and her anti‐acetylcholine‐receptor antibody titer decreased gradually to normal. Currently, she is six years old and continues to be free from complaints and on no medication.
Intravenous Flunitrazepam for Status Epilepticus
Authors: Sumi Kiyoomi , Nagaura Tomoaki , Sakata Naoki , Nishigaki Toshinori , Akagi Motohiro
Published: October 2007

Flunitrazepam (FZP) was administered intravenously, with success, to two patients with status epilepticus. Case 1 was a patient with a tonicclonic status epilepticus. Intravenous phenytoin had no effect. Case 2 had minor status epilepticus. With the intravenous administration of FZP, diluted 1:10 with distilled water at a dose of 0.03 mg/kg at a slow rate, both patients were relieved of the status epilepticus. Unlike diazepam, FZP neither looks cloudy when diluted with distilled water nor causes angialgia. No disturbances in circulation or respiration occurred. Considering that FZP has a strong anticonvulsant action on status epilepticus, it can be expected to be a useful therapeutic agent for status epilepticus in infants and children.
Serum C‐Reactive Protein in the Early Diagnosis of Neonatal Septicemia and Bacterial Meningitis
Authors: Nakamura Hajime , Uetani Yoshiyuki , Nagata Tadashi , Yarnasaki Takemi
Published: October 2007

The usefulness of serum C‐reactive protein (CRP) in the early detection of neonatal infection was studied using a special laser nephelometric apparatus (CRP‐1), by which CRP concentrations could be quickly determined in the nursery, with only a small amount of serum (20 μL). Initial serum CRP concentrations of samples obtained from 90 infants suspected to have sepsis and/or meningitis were evaluated. Of the 90 infants, 25 showed culture‐proven septicemia and/or bacterial miningitis, while 18 were considered to be infectious based on clinical signs and positive sepsis work‐up even though cultures were negative. 47 infants had negative cultures and sepsis work‐up and showed a favorable clinical courses. Statistical analysis for the evaluation of serum CRP at the level of one mg/dL was performed. False negative CRP was demonstrated in seven of 25 infants with culture‐proven sepsis and/or meningitis (28%) and in 4 of 18 infants with other infections (22%). On the other hand, seven of 47 (15%) non‐infected infants showed false positive results. The specificity and sensitivity of serum CRP determination were 85% and 74%, respectively, for all patients, and 85% and 72%, respectively, for patients with sepsis and/or meningitis. The sensitivity varied with the pathogens. We conclude that, while the initial CRP values alone are unsatisfactory for deciding the need for antibiotic therapy, CRP is useful in the early detection of neonatal infections, and its measurement by this new equipment should available in the nursery.
A Report of a Case with Pure Red Cell Aplasia Induced by Sodium Valproate
Authors: Kawauchi Kyoichi , Miyano Takakazu , Ikeda Yasuhiko , Tateoka Noboru , Kasai Mikio , Sato Yuichi , Yokoyama Masaru
Published: October 2007

A 5‐year‐old girl developed pure red cell aplasia (PRCA) during sodium valproate (VPA) administration. Only four cases of VPA induced PRCA have been reported in the literature. Furthermore no precise report concerning the underlying mechanisms is available. Using a system for the clonal growth of autologous bone marrow committed erythroid stem cells (CFU‐E, BFU‐E) WA itself was not inhibitory for colony formation at a physiological concentration. Patient's serum and peripheral mononuclear cells collected at admission showed no inhibitory effects on erythroid progenitor cell growth. Rapid recovery from PRCA was observed after discontinuation of VPA, without immunosuppressive therapy. Our observation suggests that VPA may induce PRCA through the inhibitory effect beyond the differentiation stage of BFU‐E and CFU‐E in some cases.