bluuebunny/crossref_metadata_2025 huggingface dataset:
Prevention of Neonatal HBV Infection with the Combination of HBIG and HBV Vaccine and Its Long‐Term Efficacy in Infants Born to HBeAg Positive HBV Carrier Mothers
Authors: Tajiri Hitoshi , Nose Osamu , Shimizu Kazuo , Ida Shinobu , Miki Kazunori , Kimura Saburo , Yabuuchi Hyakuji
Published: October 2007

Seventy‐three infants born to HBeAg positive HBV carrier mothers were protected from neonatal HBV infection with our standard prevention schedule consisting of two doses of HBIC (0, 2 mo) and three doses of HBV vaccine (2, 3, 5 mo). In 62 infants who successfully responded to HBV vaccine with a titer of anti‐HBs greater than 23, anti‐HBs titer was monitored for as long as 48 months (25.6 ±11.0 mo) and found to decrease as follows: 5.1 ± 1.7 at 12 mo., 4.5 ± 1.8 at 18 mo., 4.2 ± 1.8 at 24 mo., 4.0± 1.6 at 30 mo., 3.7 ± 1.7 at 36 mo., 32 ± 2.0 at 48 mo. During the follow‐up period, eight HBV events (11.9%) were demonstrated: one case showed an increase of anti‐HBs, three showed a reappearance of anti‐HBc alone, three showed a reappearance of anti‐HBc with increase of anti‐HBs, and one became a chronic HBV carrier. All infants were further divided into three groups by their maximal response of anti‐HBs to HBV vaccine: Group I (26), Group II (23‐25), and Group III (22). Group I sustained a higher titer from 12 to 30 months of age and had less HBV events than (3‐II and G‐III. Our study suggests that acquisition of a high titer of anti‐HBs is important in long‐term prevention of HBV infection as well as in the neonatal period in infants born to HBeAg positive HBV carrier mothers.
National Project on the Prevention of Mother‐to‐Infant Infection by Hepatitis B Virus in Japan
Authors: Eto Takashi , Shiraki Kazuo
Published: October 2007

In Japan, a nationwide prevention program against mother‐to‐infant infection by hepatitis B virus (HBV) started in 1985. This program consists of double screenings of pregnant women and prophylactic treatment to the infants born to both hepatitis B surface antigen (HBsAg) and hepatitis B e antigen (HBeAg) positive mothers. These infants are treated with two injections of hepatitis B immune globulin (HBIG) and at least three injections of plasma derived hepatitis B vaccine. We sent questionnaires about the numbers of each procedure or examination during nine months of investigation period to each local government in 1986 and 1987. 93.4% pregnant women had the chance to be examined for HBsAg, and the positive rate was 1.4 to 1.5%. The HBeAg positive rate in HBsAg positive was 23 to 26%. The HBsAg positive rate in neonates and in infants before two months were 3% and 2% respectively. Some problems may arise, because 27 to 30% of infants need the fourth vaccination in some restricted areas.
Teratogenic Effects of Combined Use of Anti‐Kidney Serum and E‐64 in the Rat
Authors: Chen Shen Fang
Published: October 2007

The teratogenic effects of rabbit anti‐rat‐kidney semm (AKS) combined with E‐64 (a thiol protease inhibitor) were examined. Wistar rats were injected with 10 or 20 mg/kg of E‐64 on days 9 and 10 of gestation, and with a subteratogenic dose (1 ml/kg) of AKS on day 9 or day 10. The most common malformations were hydronephrosis, hydrocephalus, ureteral dilatation, microphthalmia, anophthalmia, and heart defects. When AKS was given on day 9 or 10, the incidence of malformations was about 7% (10 mg/kg group) and 24% (20 mg/kg group). This indicated that the types of malformation are similar to those produced by AKS and E‐64, and there is a synergistic effect between AKS and E‐64.
Syndrome of Generalized (Peripheral Tissue and Pituitary) Resistance to Thyroid Hormone
Authors: Aritaki Sekoiya , Shirnazaki Tetsuya , Ogihara Masaaki , Matsuno Tetsuhiko , Honda Teruo , Izumizawa Akira
Published: October 2007

Generalized resistance to thyroid hormone (GRTH), or Refetoff syndrome, is a disease in which peripheral tissues show resistance to thyroid hormone. Three patients with this disease were investigated. Cases 1 and 2 involved identical 7‐year‐old female twins and case 3, a 5‐year‐old girl. All three patients had goiters, and cases 1 and 2 had sensorineural deafness. In all three, the blood levels of T4, free T4, and T3 were high, while the blood levels of TSH were normal or slightly elevated. The responses shown by blood levels of the thyroid hormone and TSH to administration of propylthiouracil and T3 suggest that the regulating mechanism in the hypothalamic‐pituitary‐thyroid system was functional. Upon administration of T3, no sign of hyperthyroidism was observed.
Bam HI Restriction Fragment Length Polymorphisms for Hypoxanthine‐Guanine Phosphoribosyltransferase (HPRT) Gene of Carriers and Controls of HPRT Deficiency in Japan
Authors: Igarashi Takashi , Ikegami Hirohiko , Yamazaki Hiroko , Minami Mutsuhiko
Published: October 2007

Three‐allele restriction fragment length polymorphisms (RFLPs) for the restriction endonuclease Bam HI are known at the hypoxanthine‐guanine phosphoribosyltransferase (HPRT, E.C.2.4.2.8.) gene locus. The alleles are expressed phenotypically on Southern blots as three distinct pairs of fragments that hybridize to HPRT cDNA: i)a 22‐kilobase (kb) / 25‐kb pair, ii) a 12‐kb / 25‐kb pair, and iii) a 22‐kb / 18‐kb pair. Allele frequencies in 119 unrelated Japanese people were 0.38 for the 22‐kb / 25‐kb allele, 0.43 for the 12‐kb / 25‐kb allele, and 0.19 for the 22‐kb / 18‐kb allele, an average heterozygosity of 66% in Japanese females, a higher rate than in Caucasian females. Five out of nine carriers of partial or complete HPRT deficiency showed heterozygous patterns for Bam HI RFLPs.
Pancreatic Hormone Changes in Infantile Obstructive jaundice
Authors: Hata Yoshinobu , Sasaki Fumiaki , Igarashi Motomu , Namieno Tsutomu , Takahashi Hiromasa , Uchino Junichi
Published: October 2007

Metabolic disturbances of pancreatic hormones in obstructive jaundice in infancy were evaluated experimentally and clinically. In our experimental study, using young rats, the level of plasma insulin (IRI) gradually increased after ligation of the common bile duct. These levels were a little lower than those in the non‐treated controls. The level of plasma glucagon (IRG) incerased remarkably 4 weeks after ligation of the common bile duct. Clinically, there were no significant differnces in the levels of IRI and IRG among normal controls and cases of neonatal hepatitis and congenital biliary atresia (CBA). In CBA patients, these levels can be correlated with the progression of hepatic fibrosis; an increase in IRG and a decrease in teh IRI/IRG mol ratio was noticed in patients with grade III of hepatic fibrosis. These indicate that, in obstructive jaundice in infancy, the more severe the hepatic damage due to obstructive jaundice, the higher the level of plasma glucagon concentration will rise.
The Third Nationwide Survey in Japan of Vitamin K Deficiency in Infancy
Authors: Hanawa Yoshiyuki , Maki Masahiro , Matsuyama Eikichi , Tada Hiroshi , Urayama Tsutomu , Yamada Kaneo , Mori Hiroko , Nagao Takeshi , Terao Toshihiko , Mikami Sadaaki , Shiraki Kazuo , Onishi Shoju , Shirahata Akira , Tsuji Yoshiro , Motohara Kunihiko , Tsukimoto Ichiro , Sawada Ken
Published: October 2007

The occurrence of hemorrhagic disease due to vitamin K (VK) deficiency beyond the neonatal period has come under investigation in Japan. In 1980 the 1st nationwide survey was conducted in Japan by Nakayama and others [1], and was followed by the 2nd nationwide survey in 1985 by Hanawa [2]. The present survey was designed to further monitor the incidence of this disease in Japan during the 3‐year period from July 1985 to June 1988. Questionnaires were sent to 1,315 hospitals having more than 200 beds, located throughout Japan. Responses were received from 775 hospitals, for an answer rate of 58.9%. The total number of reported cases was 175, including 129 idiopathic type, 28 secondary type and 18 near‐miss type. In this survey it was revealed that the incidence rate of the idiopathic type of vitamin K deficiency in infancy (VKDI) has decreased remarkably, to about one‐fourth that reported in the first survey. The declining incidence rate of VK deficiency in Japan is considered to be the result of ever more widespread prophylactic administration of VK during the neonatal period, as most occurrences of VK deficiency in infancy are preventable by prophylactic administration of VK from the neonatal period. However, in 16 cases of the idiopathic type of VK deficiency found in the present survey, VK had been administered at least once during or after the neonatal period. This shows the heterogeneity of this condition.
A Rubella Epidemic in Sasebo, Japan in 1957, with Various Complications
Authors: Moriuchi Hiroyuki , Yamasaki Shiro , Mori Koichi , Sakai Masayoshi , Tsuji Yoshiro
Published: October 2007

Though the rubella vaccination programme for adolescent girls was introduced in Japan in 1977, rubella epidemics have occurred repeatedly. Also in Sasebo, Japan in 1987, we experienced various complications as follows: encephalitis (five cases), meningitis (three), thrombocytopenic purpura (four), vascular purpura (four), hemolytic anemia (two), pneumonia (eight), protein‐losing gastroenteropathy (one), multiple organ disorder with encephalitis, purpura, myocarditis, hepatic and renal dysfunction (one), and congenital rubella syndrome (CRS: three). Disorders ranging over multiple organs seem to occur in acquired as well as congenital rubella infection. The incidence of encephalitis was estimated to be 1: 1600 cases of rubella and two of five cases were apparently serious. Though the strategy for preventing rubella has been directed only against CRS, we should note the various and severe complications with acquired rubella infection, and should adopt two‐stepped protection: vaccination of young children of both sexes and of adolescent girls.
Japanese Encephalitis in a 7‐Month‐Old Japanese Boy
Authors: Tamada Koichi , Kasai Hideaki , Hara Mitsuhiro , Aoyama Tatsuo , Takeuchi Yoshinao , Yamashita Ryoko
Published: October 2007

A 7‐month‐old Japanese boy was diagnosed as having meningo‐encephalitis with anterior horn cell damage. The complement fixation (CF) and hemagglutination inhibition (HI) tests showed increased antibody titers against Japanese encephalitis (JE) virus,

but HI antibody in the mother's serum was not increased. It appears that the lack of JE‐antibodies from the mother may have resulted in increased JE viral infectivity in the patient. A decrease in the incidence of antibodies against various viruses in women of childbearing age may lead to increased rates of severe viral infection in infants.
Histiocytosis X: Successful Treatment with Recombinant Interferon‐αA
Authors: Sato Yuichi , Ikeda Yasuhiko , Ito Etsuro , Miyano Takakazu , Kawauchi Kyoichi , Yokoyama Masaru , Kamata Yoshimasa
Published: October 2007

Two patients with disseminated histiocytosis X were treated with intramuscular injections of recombinant interferon αLA. The bone, skin and visceral lesions of these patients diminished rapidly. Significant sclerosis and recalcification of bone were observed. From our limited but positive experience, it would be justified to consider the use of interferon in patients with disseminated histiocytosis X.
Bronchial Asthma in Singapore Children
Authors: Chay O. M.
Published: October 2007

The incidence of bronchial asthma admissions to Singapor's major Paediatric Units has increased over the years. The diagnosis of asthma was usually simple but patients with atypical presentations required the demonstration of bronchial lability to confirm the diagnosis. National statistics revealed a small but constant number of deaths from asthma in childhood. Underestimation of severity and delay in treatment were important contributing factors to deaths from asthma.
Immunological Aspects of Asthma (Prophylaxis)
Authors: Ito Setsuko , Mikawa Haruki
Published: October 2007

A long follow‐up study revealed that convalescent stage children still have the potency to have an immediate type hypersensitivity reaction on exposure to mite antigen, with a high titer of mite specific IgE in their sera, but they are free from asthmatic attacks because of the reduction in the amount of mite antigen in the body, as shown by the reduction in the amount of mite specific IgG. We also made a prospective study to see whether early elimination of the allergen has any beneficial effect on the cessation of the development of allergic diathesis thereafter. Our study suggested that early elimination of food allergen in infancy not only improved clinical manifestations but also had a protective effect on the progression of the allergic march so far as production of reaginic antibodies was concerned: production of total IgE and mite specific IgE was kept to a minimum.
Urinary Arginine Vasopressin in Asthma: Consideration of Fluid Therapy
Authors: Shimura Naoto , Arisaka Osamu
Published: October 2007

To elucidate the role of antidiuretic hormone (ADH) on water and electrolyte balance in patients with asthmatic attacks, urinary arginine vasopressin (AVP) was assayed in 28 asthmatic patients. In a 3‐year‐old girl with status asthmaticus who developed a grand mal seizure in association with hyponatremia, urinary AVP levels remained high and fluctuated before convulsion; the cause of the convulsion was considered to be water intoxication due to inappropriate ADH secretion. In 19 of 28 patients with moderately severe asthmatic attacks, increases in urinary AVP levels occurred before treatment (300 ± 80 pg/ml vs. 40 ± 24 pg/ml (normal controls), p < 0.01); elvated AVP levels tended to fall in response to intravenous fluid therapy (appropriate ADH secretion) in 2 of 6 patients, but did not fall (inappropriate ADH secretion) in the remaining patients. It is concluded that inappropriate ADH secretion may occur in asthmatic attacks, and that in such a condition there seems to be a potential risk of water intoxication during fluid therapy, as demonstrated in the present patient.
Special Therapy in an Institutional Hospital
Authors: Nishimuta Toshiyuki
Published: October 2007

I would like to introduce institutional therapy, a special therapeutic method used in our country for severe bronchial asthmatic children. Institutional therapy is mainly being used in national and municipal medical facilities. Approximately 2,300 patients receive this treatment yearly. Of the 158 patients who received this treatment in our institution in the past 4 years, 56.3% of them are leading normal lives after hospital discharge. Comparing those with a good prognosis to those with a bad prognosis, the time from onset of the serious stage to hospitalization is shorter and EIA levels are increased in the former group. Adequate amounts of physical training are effective in improving the patients' functional abilities and promoting confidence; therefore this therapy is highly effective.
Death from Asthma in Children
Authors: Matsui Takehiko , Baba Minoru
Published: October 2007

We have recorded 29 cases of death from asthma in children who had visited the Department of Pediatrics of Doai Memorial Hospital between 1965 and 1987. These cases have been analyzed. They were 18 males and 11 females. The mean age at death was 12.4 years and we found that in later childhood and adolescence the age at death had been increasing since 1973. The duration from final attack to death was very short: less than 1 hour: 21% and less than 2 hours: 50%. About half of the cases could not reach the hospital. Ten cases died within one year after cessation of long‐term systemic use of steroid. Severe attacks of asthma were particularly likely to occur during the reducing period or following cessation of these drugs. The importance of adequate and vigorous initial therapy in severe asthmatic attacks should be emphasized. The causes of death are as follows: asphyxia: 23 cases (one complicated by aspiration, one withdrawal syndrome of steroid, one subendocardial haemorrhage and two pneumothorax cases), anaphylactic shock to ACTH: 1 case, shock to stimulant: 1 case, following emergency operation: 1 case and unknown: 3 cases. Asthma death in children in Japan is reviewed.
Pernicious Anemia in a Patient with Hypogammaglobulinemia
Authors: Moriuchi Hiroyuki , Takayanagi Toshimitsu , Yamasaki Shiro , Yasui Makoto , Mori Koichi , Yanai Masanori , Tsuji Yoshiro
Published: October 2007

A 19‐year‐old male with pernicious anemia and hypogammaglobulinemia (common variable immunodeficiency: CVID) is reported in comparison with classical pernicious anemia. This case was characterized by an earlier onset of anemia, the absence of autoantibodies to intrinsic factor or gastric parietal cells and involvement of the pyloric antrum as well as the gastric corpus. It is suggested that dysregulation of cellular immunity produces the autoimmune lesion in the gastric mucosa, including the pyloric antrum, in a patient with CVID, and that some of such cases develop pernicious anemia.
Epidemiologic Survey of Children with End‐Stage Renal Disease
Authors: Hisano Satoshi , Tsuru Noboru , Itoh Yuhei , Hattori Shinzaburo , Uchiyama Makoto , Tamanaha Koichiroh , Ninomiya Makoto , Furuse Akio , Yamagishi Minoru , Hohjoh Minoru , Nagano Kiyoaki , Hashiguchi Kanehide , Yanase Tetsuro , Ohbu Keizo , Mukuno Kumiko , Kuriya Norikazu , Yamashita Fumio
Published: October 2007

We performed an epidemiologic study on the basis of a questionnaire survey of 162 children with end‐stage renal disease (ESRD). Sixty‐nine (43%) of our 162 children, including 25 detected at mass screening of urine, were found by chance hematuria and/or proteinuria. The three major causes of ESRD in our children were chronic glomerulonephritis (CGN) in 56, congenital anomalies of the urinary tract in 30, and nephrotic syndrome (NS) in 27. The renal pathology in 39 children with CGN or NS was focal glomerular sclerosis in 15, diffuse mesangial GN in 7, IgA GN in 5, membranoproliferative GN in 3, membranous GN in 3, and unclassified in 6. Forms of dialysis initiated were hemodialysis in 91 children, continuous ambulatory peritoneal dialysis (PD) in 66, and intermittent PD in 5. Renal transplantation was performed on 38 children, and the graft and the patient survival rates were 76% and 89%, respectively. The survival rate of our 162 children for a mean follow‐up of 8.1 years was 77%. In conclusion, an integrated program of maintenance dialysis and transplantation provides a favorable life for children with ESRD.
Adverse Effects on EEG and Clinical Condition after Immunizing Children with Convulsive Disorders
Authors: Nouno Shin , Togawa Keiko , Yamatogi Yasuko , Kodani Nobuyuki , Ikeda Masanori , Kunitomi Taiji
Published: October 2007

116 immunizations were given to 61 children with febrile convulsion or epilepsy who had not had a seizure for 1 year since the last attack. In 92 of the 116 immunizations the electroencephalogram (EEG) was examined before and after immunization. No adverse effects on the EEG were observed in 19 immunizations with Japanese encephalitis, measles, mumps or rubella vaccines. Epileptic spikes reappeared after 10 immunizations and epileptic spikes increased after 10 immunizations among 73 given for diphtheria, acellular pertussis and tetanus (DPT), diphtheria and tetanus (DT), or Bacillus Calmette‐Guerin (BCG). A convulsion was observed once in one child 7 days after immunization with BCG. A follow‐up EEG examination is necessary after children with convulsive disorders are immunized.
Immune Response of Subjects at High Risk of Hepatitis B to a New Genetically Engineered Hepatitis B Vaccine Administered in Low Doses by the Intradermal Route
Authors: Leonardi S , Leggio T , Barone P , Sciacca A , Musumeci S
Published: October 2007

Fifty‐eight subjects at high risk for hepatitis B were vaccinated with hepatitis B vaccine prepared by a DNA recombinant technique (Engerix B‐ Smith‐Kline Biologicals) by the intradermal route with low doses (5 microgr) at 0, 15, 30 and 45 days on the volar surface of the arms. A positive immune response (100%) was found in all children (5 affected by thalassemia, 5 affected by sickle cell anaemia, 9 affected by neurological handicaps) and in all healthy medical staff. None showed side‐effects and the small pigmented macula following vaccination disappeared after a few months. Since the World Health Organization proposed a program of mass vaccination in highly endemic areas for hepatitis B, the authors suggest that the use of the new engineered vaccine in low doses by the intradermal route could represent an effective strategy to realize this project.
Acute Leukemia with Normal Platelet Count at Diagnosis
Authors: Hara Toshiro , Mizuno Yumi , Ikuno Yoshiko , Okamura Jun , Nagata Mari , Ishii Eiichi , Yamada Shinichi , Tasaka Hideko , Ueda Kohji
Published: October 2007

Thirty‐six (17.8%) of 202 children with acute lymphoblastic leukemia (ALL) and 2 (3.7%) of 54 children with acute nonlymphoblastic leukemia (ANLL) had a platelet count over 150 times 109/1 at diagnosis. Children with ALL and a platelet count over 150 times 109/1 were analysed in detail. The ALL patients without thrombocytopenia tended to be male predominant and had less frequent bleeding manifestations (p < 0.01).These patients without thrombocytopenia had also significantly less marked leukocytosis (p < 0.01), less severe anemia (p < 0.05) and lower percentages of bone marrow blasts (p < 0.05) than those with thrombocytopenia. In addition, ALL patients without thrombocytopenia had a significantly higher probability of continuous complete remission than those with thrombocytopenia (p < 0.01).
Successful Bone Marrow Transplant and Re‐Growth of Hair in a Patient with Posthepatic Aplastic Anemia Complicated by Alopecia Totalis
Authors: Shibuya Atsushi , Shinozawa Takashi , Danya Naohiro , Maeda Kazuichi
Published: October 2007

Bone marrow transplant was performed in a child with posthepatic aplastic anemia complicated by alopecia totalis. The procedure was successful, as the graft was apparently “taken” by the recipient and hair started to grow on the head. The hair has not been lost since, and is still growing as of the date of this report. Although nonA nonB hepatitis virus was found to be the cause of hepatitis in this patient, the reason for the alopecia totalis which developed 7 years previously is unclear. The postoperative growth of the hair was attributed to restoration of normal immunologic function through bone marrow transplant.
Interstitial Deletion of Long Arm of Chromosome 2(q3 lq33)
Authors: Hiroyama Yoshifumi , Hatanaka Hiroyuki , Ikenoue Tsuyomu , Ishihara Yoshimori
Published: October 2007

We describe the case of a 4‐month‐old girl with interstitial deletion of the long arm of chromosome 2(46,XX,del(2) (q31 q33)). Clinical features included intrauterine growth retardation, psychomotor delay, antimongoloid slanting of the palpebral fissures, hypertelorism, low set ears, cleft palate, micrognathia, luxatio coxae and pes varus. It is suggested that the gene for soluble isocitrate dehydrogenase (IDH1) is located on 2q33.3. The activity of serum IDH1 was in the normal range in this patient.
Neonatal Tuberous Sclerosis with Heart and Brain Tumors
Authors: Hayashi Yoshihiro , Yoshimura Kazue , Nakae Youichiro , Nara Takahiro , Hamada Rousei , Maekawa Kihei
Published: October 2007

A 6‐day‐old neonate with tuberous sclerosis having brain and cardiac tumors is reported. The patient presented with a seizure 20 minutes after birth. Cranial ultrasonography and computed tomography on admission showed a large and round high‐density tumor lesion in the left frontal lobe. Echocardiography also showed a large tumor lesion beside the left ventricle wall, and a diagnosis of tuberous sclerosis was promptly made. Two months later, a periventricular calcified lesion was found on the demonstrated portion, and the tumor lesion initially observed in the brain had disappeared. These findings suggest that the initial tumor lesion found in the brain developed into a typical calcified lesion.
K1 Antigen, Serotype and Antibiotic Susceptibility of Escherichia coli Isolated from Cerebrospinal Fluid, Blood and Other Specimens from Japanese Infants
Authors: Fujita Kozo , Yoshioka Hajime , Sakata Hiroshi , Murono Koichi , Kakehashi Hitoshi , Kaeriyama Masato , Tsukamoto Teizo
Published: October 2007

K1 antigens, serotypes and antibiotic susceptibilities of Escherichia coli isolates from neonates and infants were investigated. The presence of K1 antigen was tested by the K1‐specific phage method. The number of K1 positive strains was 27 (84%) of 32 isolates from cerebrospinal fluid, 11 (25%) of 44 from blood and 4 (22%) of 18 from other specimens. Fourteen (33%) of the K1 positive strains were serotyped as O16:H6, and 8, 7 and 5 were serotyped as O18ac:H7, O1:H7 and O7:H‐, respectively. One of 5 of the K1 negative strains were distributed into 30 different combinations of O and H antigens. The ampicillin resistance rates were 19% in K1 positive strains and 45% in K1 negative ones. The incidence of chloramphenicol resistance was the same in K1 positive and negative strains (21%). Ampicillin resistance was not noted in O16: H6 strains, but the incidence of antibiotic resistance was high (65% to ampicillin and 53% to chloramphenicol) in the rough‐type strains.
HLA A Class II (DR, DQ) in Japanese Patients with Type 1 Diabetes Mellitus
Authors: Tanaka Masahiro , Abe Jun , Ito Junko , Horikawa Reiko , Miki Yuko , Nakayama Masayukio , Kohsaka Takao , Tanae Ayako , Hibi Itsuro
Published: October 2007

DNA restriction fragment length polymorphism (RFLP) typing of HLA‐DR and DQ alleles of 60 Japanese type 1 (insulin dependent) diabetic patients and 115 controls was performed. RFLP typing of DRB1 showed increased frequency of DR9 and decreased frequencies of DR2 and DRW6 among patients compared to controls. In the RFLP typing of BamHI‐ digested DNA to DQ β probe (BamHI‐DQB1), the incidence of the 10.26 kb fragment, which represents either DQW4, DQW8 or DQW9, was markedly elevated in the patients, whereas the incidence of DQW6 was reduced. The predicted DR‐DQ haplotype study revealed that DR4‐DQW4 or DQW8, DRW8‐DQW4 or DQW8 and DR9‐DQW9 may contribute to susceptibility to type 1 diabetes. When serological typing of the 13 DRW8 patients was performed, all the 11 DRW8 patients carrying DQW4 or DQW8 (BamHI‐10.26 kb) were positive for DQW3.

These results indicated that the HLA‐DQ locus may play an important role in the development of type 1 diabetes in the Japanese as well as other ethnic groups and that the DRW8‐ DQW8 haplotype may predispose to the disease in Japan.
A Case of I‐Cell Disease
Authors: Yuasa Yoshihiro , Yoshinaga Masao , Kitahara Takuma , Miyata Koichiro , Shimozono Isato , Yoshida Hiroki
Published: October 2007

A case of I‐cell disease is reported. The patient suffered from several episodes of pneumonia, and died of pneumonia at 12 months of age. Tissue specimens obtained at autopsy were stained with colloidal iron to demonstrate acid mucopolysaccharides. Characteristic foamy changes were observed in organs such as the heart, kidneys, liver, spleen and brain. An interesting finding in this case was that not only the interstitial cells but the alveolar epithelium in the lung showed the same foamy changes. The major causes of death of patients with I‐cell disease are congestive heart failure and recurrent respiratory infections. However, there have been few reports on the histological changes in the lungs, and none have described the changes in the alveolar epithelium. Further cases must be investigated to examine the pathological relation between the histological changes in the lungs and the cause of death, because recurrent respiratory infections are the major contributor to death in patients with I‐cell disease.
Screening for Hypercalciuria
Authors: Akashi Shunji , Motizuki Hiroshi
Published: October 2007

Reference values for the urinary calcium/creatinine ratio (Ca/Cr ratio) in the first morning urine were established in 361 healthy children aged 5 to 15 years, on unrestricted diets. The urinary Ca/Cr ratio in the urine upon arising was independent of sex but dependent upon age. The measurement of the urinary Ca/Cr ratio in the urine upon arising while on unrestricted diets may be a reasonable screening test for elevated calcium excretion. On the basis of the urinary Ca/Cr ratios in the urine upon arising during unrestricted diets and the calciuric response to calcium restricted diets and the oral calcium loading test, idiopathic hypercalciuria (IH) was subclassified into three groups: (1) absorptive hypercalciuria; (2) renal hypercalciuria; (3) dietary hypercalciuria. The pathogenesis of IH is controversial. Our data suggest that disordered 1,25 (OH)2 vitamin D metabolism with excessive urinary phosphate excretion occurs in absorptive hypercalciuria.
Potential Effects of Recombinant Human Growth Hormone (r‐hGH) on Somatic Growth in Uremic Rats
Authors: Kawaguchi Hiroshi , Komatsu Yasuhiro , Hattori Motoshi , Ito Katsumi , Hizuka Naomi , Takano Kazue
Published: October 2007

In this study, we investigated both the efficacy of growth hormone (GH) in the improvement of growth disturbances in uremia and the effects on the remaining kidney, using uremic rats. Twenty 5/6 nephrectomized uremic rats were divided into two groups. One half of them were injected intraperitoneally with 2IU of human recombinant growth hormone (r‐hGH) twice daily. The others were injected only with the vehicle. Sham operated control rats were also injected with the vehicle. These rats were pairfed and observed for as long as 4 weeks, during which, the growth in stature in uremic rats without r‐hGH was markedly retarded, with a gain of only 15.8 ±3.7 mm of body length. It was significantly less than the 48.0 ± 5.1 mm (p<0.01) gained by control sham‐operated rats. By contrast uremic rats treated with r‐hGH gained 37.8 ±2.9 mm, which was significantly greater than uremic rats without r‐hGH (p<0.01) but less than controls (p<0.01). Increments of body stature paralleled those of skeletal bones. The food utilization rate was significantly improved in uremic rats treated by r‐hGH. Throughout the experiment, serum creatinine and urea nitrogen levels remained constant and there were no intergroup differences in the uremic rats including insulin like growth factor I (IGF‐1) and other chemical parameters except for plasma GH levels. These results suggest that r‐hGH stimulates somatic growth in rats with chronic renal failure through improving food utilization and a probable direct effect on growing cartilage.
Precision and Reliability of Stable Microbubble Test as a Predictor of Respiratory Distress Syndrome
Authors: Chida Shoichi , Fujiwara Tetsuro , Takahashi Akio , Kanehama Seiki , Kaneko Junko
Published: October 2007

The precision and reliability of the stable microbubble test as a predictor of respiratory distress syndrome (RDS) were studied. In blind experiments, the stable microbubble test was performed by three analysts in quadruplicate on six different amniotic fluid samples obtained from pregnant women whose babies developed RDS and on three samples prepared from mature controls. Three‐way analysis of variance revealed that there were statistically significant differential effects of the analyst, observer, and sampling on the stable microbubble values, and also that there were statistically significant interactions between the analyst and observer as well as between the analyst and sampling. However, the magnitude of these effects was not large enough to modify the interpretation of the test results. We conclude that the rapidity, simplicity and reliability of the stable microbubble test allow for its use as a bedside procedure in identifying infants who are likely to develop RDS.
Intracoronary Urokinase in Kawasaki Disease: Treatment and Prevention of Myocardial Infarction
Authors: Kato Hirohisa , Inoue Osamu , Ichinose Eisei , Akagi Teiji , Sato Noboru
Published: October 2007

The main cause of death in Kawasaki disease is myocardial infarction due to thrombotic occlusion of a coronary aneurysm. Intracoronary thrombolytic treatment was performed in 15 patients with Kawasaki disease with giant coronary aneurysms. Three patients had acute myocardial infarction, four demonstrated silent myocardial infarction, three suffered chest pain and five did not show ischemia features but had massive thrombus in the coronary aneurysms. Urokinase was infused into the coronary aneurysms as a bolus of 8,000 to 10,000 units/kg via a catheter over 10 minutes. Partial but significant coronary recanalization was achieved after injection of urokinase in a patient with acute myocardial infarction. Complete resolution of massive intracoronary thrombi was observed in 3 of 15 patients, and partial resolution was recognized in 4 cases. In 7 patients, the size of thrombus did not change. Recurrence of the thrombus was observed in 4 patients by serial two‐dimensional echocardiography. Urokinase was readmmistered and two showed significant reduction in the thrombus. All patients have been followed for more than 2 years with longest 8 years (mean: 3.3 yrs), and none have had a recurrence of myocardial infarction or died. These findings suggest that intracoronary urokinase is useful for the treatment and prevention of myocardial infarction in Kawasaki disease.
A Clinical Study of Upper Gastrointestinal Endoscopy in Japanese Children
Authors: Kato Seiichi , Nakagawa Hiroshi , Harada Yoshinori , Saito Yasutoshi , Watanabe Nobuo , Abe Junichiro , Yamamoto Katsuya , Shibuya Hidenori , Takahashi Kazutoshi , Watanabe Shuichi
Published: October 2007

A total of 370 children who underwent upper gastrointestinal endoscopy (534 procedures) were retrospectively studied. All procedures were performed successfully. Side effects were demonstrated in only one case (0.2%), which was complicated by perioral cyanosis during the procedure. As to preendoscopic medication, it was thought that topical pharyngeal anesthesia alone may well be used for school‐age children over 7 years of age, general anesthesia with endotracheal intubation for neonates, and intravenous sedation for infants and younger children. Of 370 patients studied, 70.8% had endoscopic diagnoses. The most common diagnosis was gastritis (136 cases), followed by peptic ulcer (75), duodenitis (29), and esophagitis (20). Several conclusions for indications were drawn from the present study. Pediatric upper gastrointestinal endoscopy is a safe and useful method for diagnostic and therapeutic approach, if adequate preendoscopic medication is chosen.
Incidence of Herpes Zoster in Infancy in Japan
Authors: Taki Michio , Inamochi Hideki
Published: October 2007

A search of the literature has revealed only 12 reports of herpes zoster during infancy in Japan. Of these, seven cases were thought to be mother‐child infections where the mother had been infected with varicella and transmitted the VZV to the fetus, producing postnatal herpes zoster. The earliest case of maternal varicella infection occurred at 14 weeks of gestation. In the remaining 6 cases infection took place between the 17th and 32nd week of gestation. Postnatal onset of herpes zoster in the infants occurred within three months in two cases and at seven to eight months in the other four cases. In two cases it was unclear whether it was a mother‐child infection. In three cases no clinical manifestations of maternal VZV infection were observed, but in these cases during early infancy the patients might have acquired asymptomatic infection.
Association of Neonatal Thrombocytopenia and Maternal Anti‐HLA Antibodies
Authors: Koyama Norihisa , Ohama Yoichi , Kaneko Koji , Itakura Yukino , Nakamura Toshihiko , Takasaki Jiro , Tanaka Taihei , Eguchi Hideshi , Kawase Atsushi , Kamiya Kenji , Ogawa Yunosuke , Maeda Hiroo , Ohto Hitoshi
Published: October 2007

In order to evaluate the influence of maternal anti‐HLA antibody on neonatal thrombocytopenia, clinical features and maternal anti‐HLA antibody of three groups of infants (19 thrombocytopenic and low birth weight, 27 nonthrombocytopenic and low birth weight, and 80 healthy full‐term) were investigated. The incidence of positive maternal anti‐HLA antibodies in the three groups was 73.7%, 29.6% and 27.5%, respectively. Thrombocytopenia in small‐for‐gestational‐age (SGA) infants was closely related to the presence of maternal anti‐HLA antibodies. Among 20 SGA infants (11 thrombocytopenic, 9 non‐thrombocytopenic), anti‐HLA antibody was detected in 10 mothers (90.9%) of thrombocytopenic SGA infants, while it was positive in only one mother (11.1%) of nonthrombocytopenic SGA infants. Investigation of the SGA infants revealed that in those whose mothers were sensitized to HLA antigen, not only the platelet count but also the leukocyte and lymphocyte counts in the first week of life were significantly lower than in infants whose mothers were not sensitized. The results suggest that the presence of maternal anti‐HLA antibody is a cause of neonatal thrombocytopenia especially in SGA infants.
Hepatic Cell Necrosis with Congenital Heart Disease in the Newborn
Authors: Ichihashi Kou , Matsui Akira , Yanagisawa Masayoshi , Yamada Shigeki
Published: October 2007

Hepatic cell necrosis is a recognized complication of circulatory failure. Histological examination of the adult liver in such cases reveals centrilobular necrosis and hepatic congestion. Two cases of hepatic cell necrosis in infancy are reported: a four‐day‐old girl with the hypoplastic left heart syndrome, and a nine‐day‐old girl with interruption of the aortic arch. In the former, midzonal hepatic necrosis (and not centrilobular necrosis) was demonstrated at autopsy. Both patients exhibited severe hepatic insufficiency, but hepatic function improved rapidly with treatment of the coexistent heart failure. It is suggested that there is milder form of hepatic cell necrosis than that previously reported in autopsies.
Mixed Blasts Crisis Following Extramedullary Involvement with the Cytogenetic Evidence of Clonal Evolutions Philadelphia Chromosome‐Positive Chronic Myelocytic Leukemia
Authors: Sugita Kenichi , Kashima Hirohisa , Sakakibara Hitoshi , Eguchi Mitsuoki , Furukawa Toshiharu , Nakamura Kozue , Mizutani Shuki
Published: October 2007

We describe a unique case of a young girl with adult type chronic myelocytic leukemia (CML). Clinically she had an extramedullary Mastic crisis (BC) prior to that in peripheral blood and in bone marrow. The blasts were primitive cells and always CD7, CD33, CD34 and HLA DR‐positive. During the course of illness the blasts became negative for CD5 and positive for CD41a and for platelet‐peroxidase. Additional chromosomal appearances and subsequently clonal evolution were seen during the clinical course. Surface antigen modulation and morphological changes, which were observed by microscopic examination and flow cytometry, were induced by in vitro incubation. Surface antigen modulation was more induced in the presence of phytohemagglutinine‐conditioned media (PHA‐CM) but the effects of PHA‐CM on morphological changes were not clear.
Possible Role of Streptococcus Pyogenes in Mucocutaneous Lymph Node Syndrome XIII. No Recovery of β‐Hemolytic Streptococci from Pharynx of MCLS Patients
Authors: Osawa Nobutaka , Hirata Yasuyoshi , Akiyama Takehisa , Hiraishi Satoshi , Yashiro Kimio
Published: October 2007

Lack of recovery of β‐hemolytic streptococci from the throat of 80 patients finally diagnosed as mucocutaneous lymph node syndrome was again confirmed, although α‐hemolytic streptococci were consistently isolated from all patients but one. The implications of these findings were discussed, particularly in terms of the possible role of Streptococcus pyogenes in the pathogenesis of this disease.
Mixed Invasive Aspergillosis and Candidiasis in a Fatal Case of Leukemia
Authors: Fujita Kozo , Oka Toshiaki , Suzuki Yutaka , Shimizu Shigeo , Ito Yoshiya , Kakehashi Hitoshi , Yoshioka Hajime
Published: October 2007

A fatal case of leukemia complicated by mixed invasive aspergillosis and candidiasis is presented. Mixed fungal infections may occur in any immunocompromised patients. We should be careful to look for different morphologies of fungi in diagnostic examinations of lesions in these patients.
Recent Advances in Neuromuscular Disorders in Childhood
Authors: Dubowitz Victor
Published: October 2007

This paper reviews recent advances in the molecular genetics of the muscular dystrophies and their implications for potential treatment. A brief review is also included of recent advances in locating the gene for the spinal muscular atrophies.
Myoblast Transfer Therapy for Duchenne Muscular Dystrophy
Authors: Law Peter K. , Goodwin Tena G. , Fang Qiuwen , Chen Ming , Li Hua‐Ju , Florendo J. Ann , Dana S , Kirby B.S.
Published: October 2007

A randomly selected extensor digitorum brevis (EDB) muscle in each of three Duchenne muscular dystrophy (DMD) boys aged 9 to 10 was injected with approximately 8 times 106 myoblasts. The contralateral EDBs were sham‐injected with carrier solution. Donor myoblasts were derived from cell culture of muscle biopsies from the normal ward or normal brothers of the recipients. Cyclosporine (CsA) treatment began two days before myoblast injection and continued for three months. Three days prior to myoblast injection and three months after, the isometric twitch and maximum voluntary contraction of the left and the right EDBs were measured. Myoblast‐injected EDBs showed increases in tensions whereas sham‐injected EDBs showed reductions. Both immunocytochemical staining and immunoblot revealed dystrophin in the myoblast‐injected EDBs. Dystrophic characteristics such as fiber splitting, central nucleation, phagocytic necrosis, variation in fiber shape and size, and infiltration of fat and connective tissues were less frequently observed in these muscles. Sham‐injected EDBs exhibited significant structural and functional degeneration and no dystrophin. Throughout the study, there was no sign of erythema, swelling or tenderness at the injection sites. Serial laboratory evaluation including electrolytes, creatinine, and urea did not reveal any significant changes before or after myoblast transfer. We conclude that myoblast transfer therapy is a safe and efficacious procedure to improve the biochemistry, structure, and function of degenerative EDB muscles in DMD.
Dystrophin Abnormality in Progressive Muscular Dystrophy ‐A Review Article‐
Authors: Arahata Kiichi
Published: October 2007

Duchenne muscular dystrophy (DMD) is a fatal X‐linked recessive disorder of muscle in children, with an incidence of approximately 1 in 3,300 male births. In about a third of affected boys, the disease is due to a new mutation, and most patients die in their early 20s. Over the last few years, the genetic, biochemical and histopathological basis of DMD has been elucidated greatly. In particular, the discovery of “dystrophin,” the protein product of the DMD gene is truly an epoch‐making success in the history of muscular dystrophy research. Dystrophin is now thought to be a cytoskeletal protein underlying the plasma membrane (known in muscle as the sarcolemma) of normal muscle fiber, and is undetectable or greatly reduced in DMD. In this review article, dystrophin in normal skeletal muscle and various neuromuscular diseases including DMD/BMD (Becker muscular dystrophy), and its carrier is discussed.
Progressive Muscular Dystrophy with Particular Reference to Muscle Regeneration
Authors: Nonaka Ikuya
Published: October 2007

In various progressive muscular dystrophies (PMD), there were a number of fibers with histological and histochemical characteristics of regenerating fibers. With a morphometric analysis in diseased muscles, an identification of type 2C fiber on ATPase stain was the most simple and reliable method for fiber type evaluation. The type 2C fiber comprised 16.5% in Duchenne and 27.5% in Fukuyama type congenital MD, suggesting an active regenerating process taking place in both diseases. Regenerative capacity of muscle fibers after experimental damage to dystrophic chicken and mdx mouse muscles was similar to those seen in nondystrophic control muscles. From the above results, we speculate that a certain environmental factor such as interstitial fibrosis seems to play a major role in delaying regeneration in dystrophic muscles.
Vimentin/desmin Immunoreactivity of Myofibres in Developmental Myopathies
Authors: Sarnat Harvey B.
Published: October 2007

Immunoreactivity for the intermediate filament proteins vimentin and desmin was studied in muscle biopsies of 33 children with neuromuscular diseases and in postmortem muscle of 15 fetuses and neonates at 8–42 weeks gestation. Fetal myotubes exhibited strong reactions for vimentin and desmin; reactivity was still present, though weaker, by 31 weeks and was no longer demonstrable at term. In X‐linked myotubular myopathy (5 cases) myofibres showed strong reactivity for both vimentin and desmin; in myotonic dystrophy desmin but not vimentin had strong reactivity in myofibres of neonates and children. A similar but much weaker pattern of desmin reactivity was seen in nemaline rod disease and in congenital muscle fibre‐type disproportion. The small myofibres in spinal muscular atrophy were reactive for both vimentin and desmin, as were regenerating myofibres in Duchenne muscular dystrophy and dermatomyositis. Acridine orange fluorochrome distinguished vimentin/desmin‐reactive myofibres that were regenerating from those of developmental myopathies because the RNA fluorescence was strong in regenerating myofibres and in fetal myotubes, but was absent from myofibres in developmental disorders of muscle. A failure to regress of fetal cytoskeletal proteins may contribute to the apparent arrest in morphogenesis of myofibres. These stains are useful in studying the muscle biopsies of children with developmental myopathies because they demonstrate an aspect of muscle maturation not detected by standard histochemical methods.
Effect of Glucocorticoids on Bone Gla Protein Values ‐BGP as a Good Marker of Osteoporosis‐
Authors: Iwasaki Tetsumi
Published: October 2007

It is important to prevent corticosteroid(CS)‐induced osteoporosis, particularly in children. One of the mechanisms is a direct inhibitory effect of C S on osteoblasts. Bone Gla protein (BGP) is produced in osteoblasts, and the serum level of BGP reflects the bone formation rate. The aim of this study is to examine the usefulness of BGP as a marker of CS‐induced osteoporosis. In the present study, serum levels of 24 pediatric patients who were given prednisolone (PSL) for long periods were studied in relation to their growth rate. Serum BGP was also determined in 167 healthy children and 16 adults. In healthy children, BGP levels reached a peak at the age of 15 years in boys and 11 years in girls. In patients who were given more than 0.25 mg/kg/day PSL, serum BGP levels were significantly decreased and height growth was remarkably suppressed. In conclusion, the measurement of serum BGP is useful for early detection of CS‐induced osteoporosis.
C‐Peptide/Creatinine Ratio in Early Morning Urine as an Indicator of Residual B‐Cell Function in Insulin‐Dependent Diabetes
Authors: Sasaki Nozomu , Miyamoto Shigeki , Niimi Hiroo , Nakajima Hironori
Published: October 2007

The C‐peptide/creatinine (Cr) ratio in early morning urine was evaluated to assess B‐cell function. The subjects were 12 boys and 36 girls with insulin‐dependent diabetes mellitus (IDDM). The controls were 130 boys and 137 girls aged 4–15 years. There was a significant inverse correlation of this ratio with the duration of insulin therapy (r = ‐0.5807, P<0.01). The daily insulin dose in U/kg was significantly different among the following groups: 1.22 ± 0.31 U/kg in group 1 with undetectable C‐peptide, 0.94 ± 0.37 in group 2 with a decreased ratio and 0.45 ± 0.28 in group 3 with a normal ratio. HbAl levels were 11.3 ±1.6% in group 1 and 9.2 ± 1.1% in group 3. The difference was significant. The result shows that the C‐peptide/ Cr ratio in early morning urine is useful for assessing B‐cell function in diabetic children.