bluuebunny/crossref_metadata_2025 huggingface dataset:
The Measles Outbreak in Chikuhou District, Fukuoka, Japan, 1990: Correlation between Herd Immunity Level and Outbreak Size
Authors: Ohga Shouichi , Okada Kenji , Miyazaki Chiaki , Akazawa Kouhei , Ueda Kohji
Published: October 2007

A measles outbreak occurred in the Chikuhou district of Fukuoka, Japan from May to October 1990, during which 71 patients were cared for at the Itoda Public Hospital. Hospital records revealed a large outbreak in the adjacent town of Kanada. In order to characterize the outbreak, questionnaires were sent to all preschool‐age children in Itoda (73% effective response) and in Kanada (76% effective response) requesting information about their vaccination and/or history of measles. The number of patients was 22 (4%) in Itoda and 63 (14%) in Kanada, most of these being preschoolers, while the vaccination rate was 61% and 44%, respectively. The herd immunity levels in age‐specific groups were compared between the two towns. Before the epidemic, the immunity level of 1 year old children in Kanada, who showed the higher attack rate, was lower (18%) than that in Itoda (39%), while after the epidemic it rose above 60% in both towns. When we studied the correlation between the attack rate and the vaccination rate, or the number of children susceptible to measles (susceptibility rate) in each preschool, the attack rate correlated negatively with the vaccination rate (correlation coefficient [CC] = ‐ 0.818; P < 0.01), and positively with the susceptibility rate (CC 0.860; P < 0.01). The regressed equation on the correlation indicated that the immunity level should be more than 70% to keep the attack rate under 5% in preschoois. After the epidemic, the immunity levels of all preschoolers reached above 70%. Early vaccination should be given to at least 70% of young preschoolers in order to prevent a large outbreak of measles under the present vaccination program in Japan.
Persistently High Antibody Titers and Deficient Specific Cellular Immunity to Varicella‐Zoster Virus in a Retarded Patient after Varicella Infection
Authors: Terada Kihei , Kawano Shoji , Yoshihiro Kiyomi , Morita Tetsuro
Published: October 2007

We report on a 19 month old female who has been retarded developmentally after a severe varicella infection contracted from her mother 4 months after delivery. Her titer of varicella‐zoster virus (VZV) IgG antibody has been remarkably high for 4 years after the infection. Natural killer activity and the specific cellular immunity to VZV, as tested by delayed type hypersensitivity skin test and lymphocyte‐proliferation assay, were impaired. She had an improvement of ataxia and then progressed developmentally after administration of an anti‐viral agent. Administration of oral corticosteroids and methylprednisolone transiently decreased titers of VZV antibody and, contrary to previous reports, returned natural killer activity to normal levels. We suggest that this case may be a persistent VZV infection similar to congenital cytomegalovirus infection due to selective defects in cellular immunity including NK cells. Whether this specific deficient cellular immunity is genetically determined or secondary to the viral infection is speculative. This is the first known report of such a case.
A Case of Ulcerative Colitis Induced by Oral Ferrous Sulfate
Authors: Kawai Masahiko , Sumimoto Shinichi , Kasajima Yoshiki , Hamamoto Torata
Published: October 2007

We report a case of ulcerative colitis (UC) in a 15 year old female undergoing treatment for anemia with oral ferrous sulfate. We suggest that the oral ferrous sulfate initiated the typical symptoms of UC in this case. This case is the first clinical report to our knowledge supporting the ‘iron‐catalysed oxidant‐mediated ischemic injury theory’ of UC.
Nosocomial meningitis due to Campylobacter fetus subspecies fetus in a neonatal intensive care unit
Authors: Morooka Tatsuya , Takeo Hiromi , Yasumoto Sawa , Mimatsu Takaharu , Yukitake Koh , Oda Teiichi
Published: October 2007

We experienced a nosocomial outbreak of Campylobacter fetus subspecies fetus (C. fetus) meningitis in a neonatal intensive care unit. A cluster of three infants developed meningitis approximately 1–1.5 months after the index patient was admitted. Two asymptomatic intestinal carriers of C. fetus were detected during the outbreak. Our experience indicates that C. fetus can cause nosocomial meningitis in neonates, and asymptomatic carriers may play a role in the transmission of the organism.
A Mosaic Case of Isodicentric Chromosome 18
Authors: Fujiwara Masataka , Kamasaki Hotaka , Morita Yasuyuki , Kamada Makoto
Published: October 2007

A case of mosaicism of isodicentric chromosome 18 is reported. Dicentric chromosome 18 occurs rarely and only five cases of isodicentric chromosome 18 have been documented. A high resolution banding method revealed that the karyotype of the patient was mos 46,XX/46,XX idic(18)(pter→q21.3::q21.3→pter), and the ratio of normal and abnormal clones was 1:1. The clinical manifestations, resembling those of trisomy 18 syndrome, were affected by both partial trisomy 18pter→q21.3 and partial monosomy 18q21.3→qter.
Myelodysplastic Syndrome with Partial Deletion of the Long Arm of Chromosome 5: First Report of a Case in a Child
Authors: Shikano Takaaki , Ishikawa Yorikazu , Anakura Michiya
Published: October 2007

A childhood case of myelodysplastic syndrome (MDS) with a deletion of the long arm of chromosome 5 (5q‐) is reported. The patient was an 8 year old boy who has recurrent angina. Laboratory evaluation revealed the following: hemoglobin 8.1 gm/dl, white blood cell count 4.9 × 103/l with 3% atypical lymphocytes, and platelet count 17.7 × 104/l. A bone marrow aspirate revealed 20% blast cells and dysmyelopoietic changes involving all three marrow cell lines. Karyotype analysis of marrow cells revealed 46,XY,5q‐ in 100% of the metaphases.

These findings led to a diagnosis of MDS with 5q‐, which is most commonly found in adult MDS. This case seems to represent an exceedingly rare childhood case of MDS with 5q‐.
Case Report of an Insulin‐dependent Diabetes Multiplex Family with a Pair of Identical Twins
Authors: Tsuchida Shinya , Kaji Masayuki , Nakata Youhei , Noguchi Chieko , Miura Takamasa , Sudo Masakatsu
Published: October 2007

The following is a case of a family with a pair of identical twins and a family history of insulin‐dependent diabetes mellitus (IDDM). A 2 year old identical twin was first admitted to our hospital and diagnosed as IDDM based on diabetic ketoacidosis. His father has been treated with insulin since the diagnosis of IDDM at the age of 17. All family members had the HLA‐DR4 and DQA1*0301 alleles, which are strongly associated with IDDM. The DR‐DQ haplotypes of the father and both twins were DR4‐DQW8 (DQB1*0302), which increases susceptibility to IDDM. Islet cell antibodies were positive only in the index twin at the time of diagnosis. The co‐twin was considered to have β‐cell dysfunction based on the result of an intravenous glucose tolerance test.
Familial Occurrence of Growth Hormone Deficiency and Primary Hypothyroidism
Authors: Matsubara Kousaku , Suzuki Kei , Lin Ying Wei , Yamamoto Toshiyuki , Ohta Shigeru
Published: October 2007

We examined endocrine function in three male siblings with growth hormone (GH) deficiency and primary hypothyroidism. Low GH responses to various provocative tests were revealed in all three. Exaggerated thyrotropin (TSH) responses to thyrotropin‐releasing hormone increased basal TSH levels, and low free thyroxine levels were also found in all three. Additionally, a low testosterone response to human chorionic gonadotropin and low gonadotropin responses to luteinizing hormone‐releasing hormone were noted in the youngest sibling. Skull X‐ray films, computerized tomography and magnetic resonance imaging revealed no abnormalities of the pituitary gland or sella turcica. The GH gene was intact in all of the children and their parents. We diagnosed all siblings as having GH deficiency and primary hypothyroidism. This is the first report of familial occurrence of GH deficiency and primary hypothyroidism.
Intra‐operative Total Enteroscopy for the Management of Peutz‐Jegher's Syndrome
Authors: Takehara Hiroo , Okada Akira , Nishi Masaharu , Masamune Katsuhiro
Published: October 2007

We present a case of Peutz‐Jegher's syndrome in an 18 year old female who was followed for gastrointestinal polyps for 13 years from the age of 5 years. The patient was treated four times with surgical or endoscopic polypectomy for gastrointestinal polyps. At the age of 14 years, a combined surgical and endoscopic approach for the management of Peutz‐Jegher's syndrome was carried out. A large polyp of the ileum required enterotomy for its removal, and another smaller polyp of the upper jejunum was identified and removed by intra‐operative total enteroscopy via the anus. Intra‐operative enteroscopy allows one to identify polyps that would previously have been missed. A more complete polypectomy can be performed using this technique, allowing the patient with Peutz‐Jegher's syndrome a longer interval between laparotomies and a reduction in symptoms attributed to polyps.
Treatment of Kawasaki Disease using the Intravenous Aspirin Anti‐inflammatory Effect of Salicylate
Authors: Umezawa Tetsurow , Matsuo Norio , Saji Tsutomu
Published: October 2007

Serum salicylate concentrations were measured in 60 patients with acute phase Kawasaki disease (KD), who were treated with intravenous aspirin (IVASP), to evaluate its antiinflammatory effect in the treatment of KD. Patients with serum salicylate concentrations ± 150μg/ml showed shorter durations of fever (7.1 ± 2.0 vs 10.4 ± 6.6 days; P < 0.05), shorter durations of positive serum C‐reactive protein (14.6 ± 4.5 vs 22.3 ± 10.6 days; P < 0.01) and lower incidences of coronary arterial involvements (0/10 vs 6/24; P < 0.05) than did patients with serum salicylate concentrations < 150μg/ml. Significant linear correlations were recognized between daily IVASP dosage and serum salicylate concentrations (r= 0.73; P < 0.01), and between serum salicylate concentrations and serum free salicylate concentrations (r= 0.82; P < 0.01). These correlations did not differ between the presence and absence of coronary arterial involvements. Based on these findings we concluded that a beneficial anti‐inflammatory effect in the treatment of KD is achieved when the serum salicylate concentration is 150μg/ml, and that such concentrations could be achieved by increasing the daily IVASP dosage to 100 mg/kg per day or more.
Successful Collection of Peripheral Blood Stem Cells from an Infant with Acute Lymphoblastic Leukemia using the Haemonetics V50.
Authors: Suzuki Nobuhiro , Katoh Shizue , Kudoh Tooru , Yohtoh Yukoh , Chiba Shunzo
Published: October 2007

Peripheral blood stem cells (PBSC) were collected using the Haemonetics V50 from an 8 month old infant weighing 7.8 kg suffering from acute lymphoblastic leukemia in the first complete remission. Leukapheresis was performed according to an exchange transfusion procedure by the two arm method using only a single lumen Broviac catheter. No problem occurred in the patient during this procedure except for a reduction (by half) of the initial platelet count. This method enables one to collect PBSC very safely, even from infants, in a manner that is painless for patients.
Two Children with Bromate Intoxication due to Ingestion of the Second Preparation for Permanent Hair Waving
Authors: Watanabe Tohru , Abe Tokinari , Satoh Masahisa , Oda Yoshihiko , Takada Tsuneo , Yanagihara Toshio
Published: October 2007

We report two children who suffered from sodium bromate intoxication due to ingestion of the second preparation for permanent hair waving (the second permanent preparation). One child suffered from gastrointestinal symptoms only. The other exhibited slight acute renal insufficiency. Results of the histological examination of the kidney in the sick child with acute renal insufficiency showed sporadic epithelial separation of the proximal tubuli under light microscopy. In addition, we could demonstrate more clearly epithelial separation and unbroken tubular basement membranes under electron microscopy (EM). To our knowledge, this is the first report of EM findings in this disease. The mechanism of epithelial injuries by bromate is not clear.
Survival Rate of Extremely Low Birthweight Infants and its Effect on the Amendment of the Eugenic Protection Act in Japan
Authors: Nishida Hiroshi , Ishizuka Yugo
Published: October 2007

Because of the increasing survival rate of extremely low birthweight infants in recent years, the viability limit in the Eugenic Protection Act in Japan was amended from 24 to 22 completed weeks of gestation. The Japan Pediatric Society's survey on the outcome of infants born in 1990 focused on infants born before 24 weeks gestation and less than 500 g. The survival rates of infants born in 23, 22 and before 22 weeks gestation overall at 511 hospitals throughout Japan were 43/118 (36%), 3/36 (8%) and 0/8 (0%), respectively. Of 16 infants, none weighing less than 400 g at birth survived but 16 (12%) of 50 infants between 400 and 499 g survived. Up‐to‐date statistical data is essential to the amendment of the concept of viability and subsequent ethical decision‐making on extremely low birthweight infants.
Epidemiology of Neonatal Chronic Lung Disease in Japan
Authors: Ogawa Yunosuke , Fujimura Masanori , Goto Akiko , Kawano Toshio , Kondo Tsutomu , Nakae Nobuyuki , Nishida Akira , Ohno Tsutomu , Takeuchi Yutaka , Togari Hajime , Maeta Haruo , Oguchi Kouki
Published: October 2007

A nationwide survey on the epidemiology of chronic lung disease (CLD) of the newborn was conducted. Questionnaires were sent to 391 level II and III neonatal centers in Japan and the registration of infants born in 1990 with chronic lung disease was requested. CLD was defined as an oxygen requirement greater than that obtainable in room air at 28 days after birth, with symptoms of persistent respiratory distress and a hazy or emphysematous and fibrous appearance on chest X‐ray. A total of 301 neonatal centers (77.0%) responded and 50,290 infants at these centers were registered. Of these, 97% survived the first month and 1,135 of 48,762 neonatal survivors developed CLD. The mortality of infants with CLD was 6.2%. Survival rates at 28 days of age increased consistently with birthweight. Survival at 28 days of age in infants below 1,000 g at birth was 73.7%, but the rate was 93.9% in infants weighing 1,000–1,499 g. The incidence of CLD was inversely proportional to birthweight. Approximately one quarter of neonatal survivors with a birthweight below 1,500 g and approximately half of extremely small infants (<1,000 g) developed CLD. The analysis of CLD infants showed that 28.2% of them had a history of respiratory distress syndrome (RDS) and a typical fibrous appearance on chest X‐ray (Type I), while 29.3% also had a history of RDS but had an atypical X‐ray appearance (Type II). Approximately 13% of CLD infants showed evidence of intra‐uterine infection and typical X‐ray findings (Type III), 11.8% showed a typical X‐ray appearance but no preceding diseases (Type IV), and another 11.5% showed atypical chest X‐ray appearance and no preceding diseases (Type V). Only 5.8% of CLD infants could not be classified into any of these five types, and were grouped as Type VI. Ninety‐two per cent of CLD infants were discharged, 6.2% died in hospital and 1.8% were still in hospital at the time of the survey.
HIV neuropathogenesis and therapeutic strategies
Authors: Epstein Leon G.
Published: October 2007

Abstract Human immunodeficiency virus (HIV)‐l neuropathogenesis can be divided into three important components: (i) virus entry into the nervous system; (ii) the role of viral proteins and/or cellular products in neural tissue damage; and (iii) the mechanisms of neuronal injury/death. Both blood derived macrophages or trafficking HIV‐1 infected T‐lymphocytes have been implicated in viral entry to the central nervous system (CNS). The major cell type harboring productive HIV‐1 infection in the nervous system is the perivascular macrophage/ microglia. The HIV‐1 infection of brain astrocytes, restricted to the expression of regulatory gene products, may cause astrocyte dysfunction and contribute to neuronal injury or to disruption of the blood‐brain barrier (BBB). Studies of cerebrospinal fluid and postmortem tissues reveal chronic inflammation/immune activation in the nervous system during the later stages of HIV‐1 infection associated with disruption of BBB integrity. Blood‐brain barrier damage may underlie the white matter pallor described in HIV‐1 infection and could result in further entry into the CNS of toxic viral or cellular products, or additional HIV‐1 infected cells. The HIV infected and activated macrophages/microglia produce excessive amounts of pro‐inflammatory cytokines, including tumor necrosis factor alpha, and platelet activating factor. These products are directly toxic to human neurons in vitro. The HIV‐1 envelope glycoprotein, gp 120 may stimulate the release of toxic factors from brain macrophages. Blocking N‐methyl‐D‐aspartate (NMDA; or AMPA) glutamate receptors can antagonize candidate toxins of both viral and cellular origin. It has been postulated that (weak) excitotoxicity leads to oxidative stress in neurons and ultimately to apoptosis. Neuronal apoptosis occurs in the brains of both children and adults with HIV‐1 infection. This understanding of HIV neuropathogenesis implies that therapeutic strategies should include: (i) anti‐retroviral medications to decrease systemic and CNS virus load, and possibly to prevent perinatal transmission of HIV; (ii) anti‐inflammatory compounds to decrease the chronic immune activation in microglia and allow the restoration of BBB integrity; and (iii) neuroprotective compounds to reduce neuronal injury and apoptotic death.
Incidence of thrombocytopenia in infants born to mothers with idiopathic thrombocytopenic purpura
Authors: Asano Takeshi , Sawa Rintaro , Arakf Tsutomu , Yamamoto Masao
Published: October 2007

Abstract Neonatal thrombocytopenia related to maternal idiopathic thrombocytopenic purpura (ITP) is reportedly uncommon but may have severe complications. The present report reviews records of 15 infants born to mothers with ITP during a 10‐year period, and the incidence of neonatal thrombocytopenia and the risk of hematological complications is examined. Severe thrombocytopenia (platelets < 50 000/μL) was seen in three infants despite successful therapy with high‐dose gamma globulin prior to delivery, which elevated maternal platelet counts. Although the platelet counts of these three infants fell to < 10 000/μL, none had severe complications. Moreover, no infants required treatment such as adrenocorticosteroids, platelets transfusion, or high doses of gamma globulin. No maternal markers predicted the degree of neonatal thrombocytopenia. The risk of complications arising from neonatal thrombocytopenia is low, but careful observation is required for the thrombocytopenic newborn of ITP mothers even when the infant has no bleeding complications at delivery.
Central diabetes insipidus as a complication of neonatal pathology: Report of three cases
Authors: Krebs Vera LÚCia Jornada , Damiani Durval , DE Albuquerque Diniz Edna Maria , Ceccon Maria Esther , Yoshimoto Cristina , Aguiar Iara FlÁVia , Setian Nuvarte , Ramos JosÉ Lauro Araujo , Vaz FlÁVio Adolfo Costa
Published: October 2007

Abstract Three patients. II. 17 and 41 days old with various degrees of central nervous system (CNS) lesions developed central diabetes insipidus as a complication of hypothalamic damage. Two of the children had congenital CNS malformations including meningomyelocele, hydrocephalus, and prosencephaly, while the third child presented Streptococcus agalactiae meningitis, complicated with CNS hemorrhage and hypertensive dilatation of the lateral ventricles. All of them fulfilled the criteria for central diabetes insipidus, reaching high levels of serum sodium and osmolality, along with hypotonic urine. The responses to intranasal arginine‐vasopressin were prompt, normalizing the serum levels of sodium and increasing urinary osmolality, allowing a better metabolic balance, avoiding continuing damage to the already compromised CNS. The neonatologist must be aware of the possibility of this kind of complication even in a normal child with CNS infection. Imaging studies showing hemorrhage in the region of the posterior hypothalamus must be a sign that this type of complication is able to occur.
Neonatal intrahepatic cholestasis with hepatic siderosis and steatosis
Authors: Tazawa Yusaku , Nishinomiya Fujihiko , Abukawa Daiki , Aikawa Junichiro , Ohura Toshihiro , Tohma Masahiko , Watanabe Arata , Suzuki Takashi , Takada Goro , Konno Tasuke
Published: October 2007

Abstract Neonatal intrahepatic cholestasis is a heterogeneous disease of undetermined cause. There is an unreported subset of idiopathic neonatal intrahepatic cholestasis with an unusual histological combination of hepatic siderosis and macrovesicular steatosis. The patients were a 34‐day‐old female and a 39‐day‐old male with normal birth weights. Their mothers had received oral iron supplement4–6 weeks before delivery. The patients had obstructive jaundice noticed at the well‐baby clinic at 1 month of life. They had high levels of serum galactose and tyrosine, hyperferritinemia. Urinary organic acid and bile acid analyses were negative, and galactose‐1‐phosphate uridyltransferase activity in red cells was normal. Liver biopsies showed diffuse iron deposits and macrovesicular fat. By substituting formula milk with lactose‐free milk, the patients responded, and had normal biochemical tests within 5 months of life. Follow‐up biopsies, at the age of 12 months, showed mild residual fibrosis without iron or fat deposits. They are both well at 3 and 6 years of age, respectively, without biochemical liver dysfunction and neurologic impairment. Prenatal iron‐overload might contribute to the pathogenesis of the disease, but further studies are needed to confirm the assumption.
Child rearing in Japan: Current trends and problems
Authors: Nishimura Kozo
Published: October 2007

Abstract Loyalty to one's extended family, a well‐known Japanese tradition, has broken down following World War II. Child rearing in Japan, therefore, has been changing gradually and clearly. Traditionally, child rearing was taught to young mothers by the grandmothers. However, recently young couples are no longer living with their parents. Therefore, there are no advisers nor consultants available at home for their child rearing. Commercialism has certainly invaded the field of child rearing, including too many guidebooks and even baby‐sitting companies. Children's lives have become much more competitive, busy and unnatural in comparison with those of20–30 years ago. This might be one cause of the increased incidence of bullying, school phobia and psychological disorders in children.
Is early surgery always necessary in patients with aortic valve prolapse complicating an Eisenmenger‐type ventricular septal defect?
Authors: Tomita Hideshi , Fuse Shigeto , Chiba Shunzo
Published: October 2007

Abstract
Background.
The report presents three patients who showed a typically prolapsed aortic cusp with or without aortic regurgitation associated with a malalignment‐type peri membranous defect, the so called Eisenmenger‐type ventricular septal defect.

Results.
Each patient developed spontaneous complete or near closure of the ventricular septal defect without worsening of the aortic regurgitation.

Conclusions: These cases suggest that prolapsed aortic cusp complicating a malalignment perimembranous ventricular septal defect is not always an absolute indication for early surgery to prevent progressive aortic regurgitation.
Short‐term effects of budesonide, nedocromil sodium and salmeterol on bronchial hyperresponsiveness in childhood asthma
Authors: Baki Ali , KaragÜZel GÜLay
Published: October 2007

Abstract
Background:
The effects of budesonide, nedocromil sodium and salmeterol on bronchial hyperresponsiveness were determined over a period of 3 weeks.

Methods:
Forty‐three asymptomatic children (22 male, 21 female, aged7–17 years) with mild‐to‐moderate asthma were evaluated. The study was placebo‐controlled and double‐blind. At the beginning the forced expiratory volume in 1 second (FEV1) was measured and a methacholine challenge was performed to determine PC20 (provocative concentration of inhaled methacholine required to reduce FEVt by 20%). The patients in group I (n = 12), group II (n = 10), group III (n = 11), and group IV (n= 10) inhaled 200 μg of budesonide, 2 mg of nedocromil sodium, 25 μg of salmeterol and a placebo, respectively, twice a day over the period of 3 weeks. Then the methacholine PC20 values of all patients were measured again and the results were compared statistically with their previous values.

Results:
The statistical data revealed that the methacholine doses in PC20 before and after treatment were different in group I (P < 0.01). However, these differences were not statistically significant in the other groups (P > 0.05).

Conclusions:
The short term usage of budesonide decreases bronchial hyperresponsiveness, but nedocromil sodium and salmeterol in the given doses do not affect bronchial hyperresponsiveness.
Seasonal benzathine penicillin G prophylaxis for recurrent streptococcal pharyngitis in children
Authors: AkŞÍT Sadik , ÇAglayan Suat , Dokucu GÜZÍDe
Published: October 2007

Abstract
Background.
To assess the efficacy of benzathine penicillin G (BPG) prophylaxis in recurrent streptococcal pharyngitis in children.

Methods: One hundred and sixty children, aged4–11 years, who experienced at least two episodes of group A β‐hemolytic streptococcal (GABHS) pharyngitis during a 4‐month observation period between September and December 1995 were randomly divided into two groups. During the following 4‐month period between January and April 1996. 80 children received BPG prophylaxis every 3 weeks as a single intramuscular injection of 1.2 million units for a body weight greater than 27 kg and a half dose for 27 kg or less. Eighty children were accepted as a control group and were not given BPG prophylaxis.

Results: The children in the BPG group experienced significantly less GABHS pharyngitis than those in the control group during the second 4‐month period (16 vs 244 episodes, respectively, P < 0.001). BPG prophylaxis decreased streptococcal pharyngitis by 92% in the children in the study group, while the frequency of GABHS pharyngitis was unchanged in those in the control group during the second 4‐month period compared with the first 4‐month period.

Conclusions: The data in the present study demonstrated that intramuscular BPG prophylaxis is very effective in preventing GABHS pharyngitis in children. It is recommended that it is used every 3 weeks in at least the fall‐winter seasons in children susceptible to frequent GABHS pharyngitis.
Clinical and radiological variability of influenza‐related encephalopathy or encephalitis
Authors: Kimura Seiji , Ohtuki Noriyuki , Nezu Atsuo , Tanaka Miyabi , Takeshita Saoko
Published: October 2007

Abstract Background: Influenza‐related encephalopathy or encephalitis is not rare in children. However, it is not well understood why the brain lesion develops from influenza infection. The purpose of this study was to clarify its pathogenesis by analyzing the clinical and neuroradiological findings in patients having influenza‐related brain lesions.

Methods
: The clinical findings in 10 children with influenza‐related brain lesions were analyzed. Eight patients had findings consistent with the diagnosis of acute encephalopathy and two had postinfectious focal encephalitis.

Results
: The results from magnetic resonance imaging (MRI) or computed tomography were divided into five categories', normal (category 1); diffuse involvement of the cerebral cortex (category 2); diffuse brain edema (category 3); symmetrical involvement of the thalamus (category 4); and postinfectious focal encephalitis (category 5). Patients in categories2–4 had symmetrical or diffuse brain lesions without leukocytosis in their cerebrospinal fluid, and there were no inflammatory reactions in the brains of two autopsied patients. Furthermore, serum concentrations of GOT were high in all, and disseminated intervascular coagulation was present in 4 of 5.

Conclusions
: These findings suggested that the pathogenesis of the brain lesions in these cases was toxic or metabolic damage due to multisystemic organ diseases induced by the influenza virus. The MRI findings in patients in category 2 suggested diffuse cortical cell necrosis. On the other hand, the thalamic lesions in category 4 patients reflected hyperpermeability of the blood vessels followed by severe edema with or without microhemorrhages. The pathogenesis of lesions of category 5 included allergic angitis of a self‐limiting nature. These findings indicated that the pathogenesis of brain damage induced by influenza infection was very variable.
Modification of the MTT method for the study of bilirubin cytotoxicity
Authors: Ngai Kin‐Chun , Yeung Chap‐Yung , Karlberg Johan
Published: October 2007

Abstract
Background
: We propose a modification of the MTT [3‐(4,5‐dimethylthiazol‐2‐yl)‐2,5‐diphenyltetrazolium bromide] method to study the cytotoxicity of bilirubin. The original method involves reading the intensity of a purplish blue color resulting from the conversion of MTT to formazan crystals by the mitochondria of viable cells. We have found that when the method is applied to study the effect of bilirubin on growing cells, precipitation of the yellow bilirubin pigment interferes with the colorimetric reading.

Methods
: A human liver cell line was used. The interference of bilirubin deposition on the MTT assay was investigated by comparing the value of optical density of the MTT solution in the presence and absence of bilirubin. The effect of 0.04 mol/L HCL‐isopropanol on the bilirubin precipitate was tested by recovering the amount of bilirubin from the wells after the isopropanol treatment.

Results
: Bilirubin deposition increases MTT reading by10–24%. Hydrochloride‐isopropanol (0.04 mol/L) dissolves MTT formazan only without disturbing the bilirubin precipitates. The bilirubin extracted into the supernatant was less than 5% of the total bilirubin deposited.

Discussion
: This indirect MTT assay, as developed in this study, could eliminate the interference of bilirubin deposits and serve as a good method for the study of bilirubin cytotoxicity.
Serodiagnosis of infectious mononucleosis in children
Authors: Wakiguchi Hiroshi , Hisakawa Hiroaki , Kubota Haruo , Kurashige Takanobu
Published: October 2007

Abstract
Background:
Although anti‐viral capsid antigen (VCA)‐immunoglobin M (IgM) is the most reliable serological marker of primary Epstein‐Barr virus (EBV) infection, it could only be detected in limited cases of infectious mononucleosis in children. We analyzed anti‐EBV antibodies by an enzyme‐linked immunosorbent assay (ELISA), a sensitive method for detecting IgM antibody and compared these results with those obtained by a conventional indirect immunofluorescence (IF) method.

Methods:
Anti‐Epstein‐Barr virus early antigen (EA)‐IgM and nuclear antigen 1 (EBNAl)‐IgG were examined by an ELISA assay in 180 sera from 70 infants and children with infectious mononucleosis, diagnosed serologically by standard IF methods.

Results:
Although by IF, VCA‐IgM was detected in only 37 of 70 (52.9%) of the sera from the acute phase of the disease, by ELISA, EA‐IgM was detected in 65/70 (92.9%) of these sera. Among infants less than 12 months of age, EA‐IgM was positive in 11/13 cases (84.6%) while VCA‐IgM was detected in only 3/13 cases (23.1%). Anti‐Epstein‐Barr virus nuclear antigen 1‐IgG was not detected by ELISA in the sera from the acute phase of infectious mononucleosis. Anti‐EBNA was not detected by IF in about one‐third of the sera during6–8 months after onset of the disease, whereas by ELISA, EBNAl‐IgG was detected in 93.0%. Sera that were positive or negative for both EA‐IgM and EBNAl‐IgG by ELISA were observed in several cases after the patients recovered from the disease.

Conclusions:
Although serodiagnosis by the combination of ELISA for EA‐IgM and EBNAl‐IgG was more sensitive than IF methods, especially in the case of infants and young children, several patients during convalescence and recovery might be judged as seronegative or as being in highly reactivated states.
Perinatal management of congenital complete atrioventricular block: Report of nine cases
Authors: Fukushige Junichiro , Takahashi Noboru , Igarashi Hisaji , Nakayama Hideki , Inoue Kazuhiko , Hijii Takayuki
Published: October 2007

Abstract Background: The addition of fetal ultrasonography has allowed the prenatal diagnosis and observation of congenital complete atrioventricular block (CCAVB). Thus, the management of the affected fetuses should be modified accordingly.

Methods: The medical records were reviewed to identify patients with CCAVB, and clinical and laboratory data were collected.

Results: Nine patients with CCAVB, diagnosed prenatally, were identified. The gestational age at the time of diagnosis ranged from 18 to 38 weeks (median, 25 weeks). The gestational age and birthweight ranged from 29 to 41 weeks and from 1420 to 4075 g, respectively. An accompanying congenital cardiac anomaly was noted in three cases, including one with polysplenia syndrome. The heart rate at birth ranged from 30 to 80 bpm. In three neonates, isoproterenol was given for bradycardia. One fetus with hydrops fetalis associated with complex heart disease was treated with maternally administered digoxin, with resolution of the fluid accumulation. A permanent pacemaker was implanted in six cases: two within 1 day of birth; two during the neonatal period; one at 51 days; and the other at 2 years and 11 months of age. There were no deaths or major complications and all have remained well beyond their infancy.

Conclusions: With the improvement of diagnostic methodology, more cases with CCAVB, including those with complex heart disease, could be diagnosed during fetal life. Their outcome has been steadily improving with the advances in perinatal management, pacemakers and implant techniques, irrespective of associated structural cardiac defects.
The effect of live measles vaccines on serum vitamin A levels in healthy children
Authors: YalÇIn S SongÜL , YurdakÖK Kadriye , ÖZalp İMran , CoŞKun Turgay
Published: October 2007

Abstract
Objective:
Serum retinol levels have been shown to be depressed during measles infection. This study aims to demonstrate whether there is any decrease in serum vitamin A level following immunization with live viral vaccine and its relation with vaccine seroconversion in children with measles. Since many children receive measles vaccine alone or in combination with measles‐mumps‐rubella vaccine, we studied serum vitamin A levels and antibody levels in healthy, well‐nourished children before and after immunization with monovalent and combined live attenuated measles vaccine.

Methods:
The first group included 21 healthy children between the ages of9–11 months who received live measles (Schwarz) vaccine. There were also 21 healthy children (range14–20 months of age) who received measles‐mumps‐rubella Trimovax® (Pasteur Merieux) vaccine. All children were tested for serum vitamin A levels before vaccination, on days9–14 and30–42 following both vaccinations. Measles specific antibody levels were also measured on admission and30–42 days following vaccinations.

Results:
In both vaccination groups, mean serum vitamin A levels reduced significantly on days9–14, but increased slightly on days30–42 in the measles‐mumps‐rubella vaccinated group (P < 0.05). The baseline and follow‐up levels of mean serum vitamin A did not differ between seroconverted and nonseroconverted cases within the measles vaccinated group.

Conclusion:
Serum vitamin A levels are reduced following vaccination with monovalent and combined live attenuated measles vaccines.
Successful polymerase chain reaction‐based diagnosis of fungal meningitis in a patient with chronic granulomatous disease
Authors: Tsuge Ikuya , Makimura Koichi , Natsume Jun , Kubota Toshiko , Hasegawa Shinji , Kawabe Takashi , Nakashima Sachie , Aso Kosaburo , Negoro Tamiko , Watanabe Kazuyoshi
Published: October 2007

Abstract Meningitis is not a common complication of chronic granulomatous disease (CGD). Here, we present details of a 3‐year‐old boy with X‐linked CGD, who suffered from fungal meningitis. While 19 samplings using conventional cerebrospinal fluid (CSF) cultures failed to detect any organisms, fungal DNA was identified in the CSF by a new polymerase chain reaction (PCR)‐based method. The patient recovered without any sequelae after treatment with a combination of antifungal agents, interferon‐φ and granulocyte infusions. This case report demonstrates that fungal meningitis must be included in the differential diagnosis of infections in CGD patients and that the PCR‐based detection of fungal DNA is a powerful tool for diagnosis.
An infant case of bilateral small kidneys with both proximal and distal tubular dysfunction
Authors: Tanaka Hiroshi , Nakahata Toru , Ito Ryosuke , Onodera Norio , Waga Shinobu
Published: October 2007

Abstract A male infant with bilateral small kidneys associated with both proximal and distal tubular dysfunction, who showed chronic renal failure soon after birth, is reported. He was also noted to have both proximal and distal type of renal tubular acidosis. The small kidneys were thought to be due to renal hypodysplasia associated with bilateral severe vesicoureteral reflux, by radiological findings. An alkalization therapy with chemo‐prophylaxis seemed to be of benefit in slowing the progression of renal failure in this case.
Diffuse brain damage caused by acute twin‐twin transfusion during late pregnancy
Authors: Wada Hideo , Nunogami Koji , Wada Taizo , Niida Yo , Yachie Akihiro , Koizumi Shoichi
Published: October 2007

Abstract
Background:
Monoamniotic twinning is a relatively rare event with increased antenatal and perinatal mortality. We desscribe a brain damage detected in a surviving monoamniotic twin after intrauterine death of the co‐twin at 37 weeks of gestation.

Results:
Severe entanglement and knotting of the umbilical cords was apparent at the time of delivery and a portion of the cors to the dead twin was narrowed significantly. It was suggested that transfer of blood occurred across placental a lastomoses from the survivor to the dead fetus, resulting in transient but severe hypovolemia in the survivor. It is difficult to prevent this type of brain damage because the course of acute twin‐twin transfusion is ven rapid and the damage has already occurred by the time the death of the twin is diagnosed.

Conclusions:
We suggest that elective delivery should be considered in cases of monoamniotic twin pregnancies with additional risk factors.
Diffuse splenic and visceral hemangiomas complicated by chronic consumption coagulopathy
Authors: Platokouki Helen , Aronis Sophie , Mitsika Angeliki , Keramidas Demetrios , Harokopos Efstathios
Published: October 2007

Abstract The case of a 7‐year‐old girl with a 2 year history of easy bruising associated with thrombocytopenia is reported. On admission she presented with ecchymoses, abdominal distention and splenomegaly. Hemostasis investigation revealed a consumption coagulopathy. Several radiological studies failed to confirm the diagnosis of diffuse splenic and visceral hemangiomatosis, which was eventually estasblished by an explorative laparotomy. Platelet count and the other coagulation abnormalities progressively returned to normal after splenectomy, although the remaining hemangiomas were extensive.
Values for urinary β‐microglobulin and N‐acetyl‐β‐D‐glucosaminidase in normal healthy infants
Authors: Nishida Masashi , Kawakatsu Hidekazu , Komatsu Hiroshi , Ishiwari Kohhei , Tamai Megumi , Tsunamoto Kentaroh , Kasubuchi Yasuo , Sawada Tadashi
Published: October 2007

Abstract Background: Measuring urinary β2 microglobin (B2M) and N‐acetyl‐β‐D‐glucosaminidase (NAG) excretion is widely used as a valuable clinical tool in assessing renal tubular lesions. However, few data are available on normal values for urinary excretion of B2M and NAG in infancy.

Methods: Urinary B2M and NAG were measured in healthy infants. The logarithmic values of urinary B2M, NAG. B2M/creatinine ratio and NAG/creatinine ratio were distributed almost normally and reference ranges were calculated from the logarithms of the observed values.

Results: The levels of urinary B2M and B2M/creatinine ratio were highest in the 1‐month‐old group, followed by a decrease during the first 3 months. Urinary B2M excretions in the 3‐monfh‐old group showed rather lower levels than those of the 12‐month‐old and 36‐month‐old groups. Although urinary NAG excretions were almost constant throughout all groups, urinary NAG/creatinine ratio decreased gradually until 3 years of age.

Conclusions: We suggest that these reference ranges are of importance in evaluating tubular damage due to a variety of renal diseases in infancy.
Characterization of developmental changes in EEG‐gamma band activity during childhood using the autoregressive model
Authors: Takano Tomoyuki , Ogawa Teruyuki
Published: October 2007

Abstract
Background
: We characterized developmental changes in the EEG‐gamma band during childhood using the autoregressive (AR) model, which was previously developed and described in our laboratory.

Methods
: The subjects comprised 707 healthy children ranging in age from 3 to 12 years. Electroencephalographs (EEG) were recorded from Fpl, Fp2. C3. C4, 01 and 02 monopolar leads referenced to the ear during quiet wakefulness (eyes closed). One 10 s artifact‐free segment was selected from each record, digitized, then analyzed by AR and component analysis, which estimated the component wave and power spectrum.

Results
: Component waves obtained from all EEG leads were divided into eight groups, which comprised approximately 2, 5. 10, 17. 25, 30, 40 and 47 Hz peaks. The gamma band (peak wave 40 Hz; range35–45 Hz) was a major EEG component throughout childhood. Different developmental characteristics of the gamma band power were exhibited in each lead. The power increased significantly (P<0.01) between 3 and 4 years of age in all leads reaching a peak at 4–5 years of age, especially in the frontal region.

Conclusions
: These developmental characteristics might be related to the information processing and cognitive function, which is enhanced at4–5 years of age, especially in the frontal region.
Transient extreme spindles in a case of subacute Mycoplasma pneumoniae encephalitis
Authors: Akaboshi Shinjiro , Koeda Tatsuya , Houdou Sadataka
Published: October 2007

Abstract We describe a 4‐year‐old boy with delirium including somnolence, irritability, agitation and visual hallucination, associated with Mycoplasma pneumoniae (MP) encephalitis. The MP encephalitis was diagnosed from increased MP‐antibody (> 1:1280). Electroencephalography during sleep revealed continuous 9 Hz fast wave activity over the bilateral frontocentral regions, which was thought to represent extreme spindles. These extreme spindles were in parallel with the degree of delirium. Transient extreme spindles have not previously been reported in MP or other encephalitis.
Type III D mucopolysaccharidosis (Sanfilippo D): Clinical course and symptoms
Authors: Tylki‐SzymaŃSka Anna , Czartoryska Barbara , GÓRska Danuta , Piesiewicz‐Grzonkowska Ewa
Published: October 2007

Abstract A case of a rare form of Sanfilippo disease, mucopolysaccharidosis type III D is presented. The cause of the disease is a deficit of N‐acetylglycosamine‐6‐sulfate sulfatase. Differences in clinical course and symptoms with type A and B Sanfilippo disease are shown (later presentation of symptoms, milder course, lack of distinct psychomotor regression and differences in characteristic phenotypic traits, such as facial features, joint contracture, tall height). It is suggested that type III D mucopolysaccharidosis be taken into account in the differentiation of mental retardation syndromes with hyperactivity.
Juvenile chronic arthritis profile in Greek children
Authors: Dracou Christine , Constantinidou Nikki , Constantopoulos Andreas
Published: October 2007

Background:
Juvenile chronic arthritis (JCA) is the commonest autoimmune rheumatic disease in childhood and presents different clinical subtypes. Juvenile chronic arthritis is considered to be of a polygenic nature and its genetic background is still under investigation. The clinical profile of JCA in the Greek population has not been studied completely. This study retrospectively analyzed the clinical and immunological features of JCA in Greek children presented between 1989 and 1994. Human leukocyte antigen (HLA)‐positive or ‐negative associations in the different clinical subtypes were also detected. The findings of this study were correlated with those reported from other populations.

Methods and results:
Antinuclear antibodies (ANA) anti‐ds DNA and anti‐extractable nuclear antigen antibodies were estimated by immunofluorescent and ELISA assays. Human leukocyte antigen typing was performed by microlymphocytotoxicity. using immunobeads. The peak ages of JCA onset were between 2 and 5 years and also between 9 and 12 years. There was a high female predominance in pauciarticular and polyarticular groups. The most common disease was pauciarticular (58.7%) followed by systemic (25%) arthritis. The incidence of eye involvement was 12.5% and presented only in the pauciarticular group. Overall, ANA positivity was 53.7%, increasing to 90% in pauciarticular cases associated with chronic uveitis. In the early‐onset (EOPA) pauciarticular subtype, positive‐HLA associations with alleles DR11 and DR8 were shown. In the late onset pauciarticular (LOPA) group only B27 allele was increased.

Conclusions:
The results of this retrospective study did not reveal major differences between JCA in Greek children compared with other Caucasian series.
A simple and efficient purification of transduced cells by using green fluorescent protein gene as a selection marker
Authors: Shimizu Takashi , Ando Kiyoshi , Kimura Minoru , Miyatake Hiroko , Inokuchi Sadaki , Takakura Iwao , Migita Makoto , Shimada Takashi , Kato Shunichi
Published: October 2007

Background
: Simple and efficient method for the selection of transduced cells would greatly facilitate the clinical utilization of retrovirus vectors. We developed a therapeutic bicistronic retrovirus vector for Gaucher disease, MFG‐GC‐GFP, which contains the human glucocerebrosidase (GC) gene and the green fluorescent protein (GFP) gene of the jellyfish Aequorea victoria as a vital selection marker, and investigated its applicability as gene therapy for Gaucher disease.

Methods and Results
: A packaging cell line, GP + envAM12, was transfected with MFG‐GC‐GFP and, thus, produced a high titer recombinant virus (1.0 × 106 c.f.u./mL) in the culture supernatant. The expression level of GFP was correlated with the virus production in cells. The recombinant virus infected skin fibroblasts from a Gaucher patient and a sorted fraction of the cells expressing GFP by flow cytometry exhibited almost a six‐fold higher activity of GC than normal fibroblasts.

Conclusions
: These data indicate that MFG‐GC‐GFP enables the one‐step purification of a transduced fraction of target cells and is, therefore, considered to be a useful therapeutic vector for the experimental gene therapy of Gaucher disease.