bluuebunny/crossref_metadata_2025 huggingface dataset:
Effect of varicella zoster virus antigen‐antibody complexes on hydrogen peroxide generation by human polymorphonuclear leukocytes
Authors: FUJIWARA TAKASHI , IHARA TOSHIAKI , YAMAWAKI KATSUHIRO , ITO MASAHIRO , KAMIYA HITOSHI , SAKURAI MINORU
Published: January 2011

Hydrogen peroxide (H2O2) generation by human polymorphonuclear leukocytes (PMN) incubated with varicella zoster virus (VZV) antigen was studied by cytofluorography. Hydrogen peroxide generation was detected in the presence of VZV‐seropositive sera. When seropositive sera were heat‐inactivated, H2O2 generation was reduced. When PMN were pre‐incubated with Leu‐1 1b, a monoclonal antibody to the Fc receptor on PMN, H2O2 generation was also reduced. These results suggest that VZV antigen‐antibody‐complement complexes induce H2O2 generation by PMN after these complexes attach to Fc receptors on PMN.
A multicenter, randomized, controlled trial of intravenous gamma globulin therapy in children with acute Kawasaki disease
Authors: MORIKAWA YOSHIYUKI , OHASHI YASUO , HARADA KENSUKE , ASAI TOSHIO , OKAWA SUMIO , NAGASHIMA MASAMI , KATOH TOSHIYUKI , BABA KUNIZO , FURUSHO KENSHI , OKUNI MASAHIKO , OSANO MITSURU
Published: January 2011

We studied the effect of intravenous, polyethyleneglycol‐treated, human immunoglobulin, administered at 200 mg/kg per day (group A: n = 147; male 86, female 61; age < 1 year, 50) or 400 mg/kg per day (group B: n = 152; male 87, female 65; age < l year, 52) for five consecutive days and compared it with freeze‐dried, sulfonated human immunoglobulin [group C: n = 152; male 87, female 65; age < 1 year, 51), administered at 200 mg/kg per day for five consecutive days, on the prevention of coronary artery abnormalities in Kawasaki disease. Echocardiograms were interpreted blindly and independently. Proportions of 87.1%, 95.4%, and 82.3% in groups A, B, and C, respectively, had no coronary artery abnormalities. The confidence limits of difference between the proportions of groups A and C, groups B and C, and groups B and A were −4.4% and 10.4%, 7.8% and 15.9%, and 4.0% and 10.8%, respectively. Duration of fever and serum immunoglobulin G (IgG) levels were correlated with the prevalence of coronary artery abnormalities. We concluded that intravenous, polyethyleneglycol‐treated, human immunoglobulin and freeze‐dried, sulfonated human immunoglobulin had clinically equivalent effects on coronary artery abnormalities, and that five daily doses of 400 mg/kg of intravenous, polyethyleneglycol‐treated, human immunoglobulin is more effective than that of 200 mg/kg gamma globulin.
Aberrant distribution of tyrosine hydroxylase and substance P in infants with brain‐stem infarction
Authors: NISHIMURA ATUSHI , TAKASHIMA SACHIO , MITO TAKASHI , BECKER LAURENCE E.
Published: January 2011

The distribution of tyrosine hydroxylase (TH) and substance P (SP) was examined in the brain‐stem of 4 infants with respiratory abnormalities associated with remote brain‐stem or cerebellar infarction utilizing immunohistochemical methods. TH‐immunoreactive cells and SP‐immunoreactive fibers were found in and around the area of the infarction in the tegmentum, in amounts and sites different from that seen in controls. The aberrant localization of SP and TH may represent an altered repair process associated with resolution of the infarction and may be related to abnormal respiratory control or sudden death.
Defective natural killer cell activity and deficient production of interferon‐γ in children with acute lymphoblastic leukemia
Authors: WAKIGUCHI HIROSHI , KUBOTA HARUO , HISAKAWA HIROAKI , FUJIEDA MIKIYA , KURASHIGE TAKANOBU
Published: January 2011

Natural killer (NK) cell activity, OK‐432‐augmented‐NK cell activity, concentrations of interferon‐γ (IFN‐γ) in the culture supernatants of lymphocytes stimulated with OK‐432, and subsets of NK cells and memory T cells were analyzed in 42 children with acute lymphoblastic leukemia (ALL) receiving maintenance chemotherapy. Natural killer and augmented‐NK cell activities, and concentrations of IFN‐γ in the supernatants of cultured lymphocytes, were significantly lower in the patients with ALL than in age‐matched control children. Among the NK cell subsets, proportions of CD57+ cells in the patients with ALL were significantly higher than in the controls, and proportions of a memory T cell subset (CD4+ CD29+ T cells) in the patients were also significantly higher than in the controls.

These results suggest that the function of NK cells and memory T cells that are considered as IFN‐γ producing cells, may be defective in ALL, and that CD57+ cells and CD4+ CD29+ cells may be resistant to or recover rapidly from suppression by cytotoxic chemotherapy.
Risks of antipyretics in young children with fever due to infectious disease
Authors: SUGIMURA TETSU , FUJIMOTO TAMOTSU , MOTOYAMA HIROKI , MARUOKA TAKANORI , KOREMATU SEIGOU , ASAKUNO YUKI , HAYAKAWA HIROSHI
Published: January 2011

The objective of this study was to determine whether paracetamol (acetaminophen) affects the outcome of children with fever due to bacterial infectious disease. A total of 208 outpatients aged 6 months to 15 years with pyrexia due to bacterial infection who had been examined at the Fujimoto Children's Hospital from March 1992 to May 1992. The number of antipyretic doses of paracetamol (10 mg/kg) a day received within 3 days of illness in the patients with acute fever (≥ = 38°C) was investigated. In this study, the patients were divided into two groups: (i) the pneumonia group, which consisted of 101 patients who were subsequently diagnosed as having pneumonia during their illness and (ii) the control group, which consisted of 107 patients who were subsequently diagnosed as having illness with fever that did not progress to pneumonia. The mean number of daily doses was significantly higher for the pneumonia group (2.52 ± 0.80) than for the control group (1.37 ± 0.72, P < 0.001). There was no significant difference between the pneumonia group and the control group in body temperature during acute fever (38.7 ± 0.65 vs 38.8 ± 0.54°C). The data suggest that frequent administration of antipyretics to children with infectious disease may lead to a worsening of their illness.
Rapid dipstick test for diagnosis of urinary tract infection
Authors: HIRAOKA MASAHIRO , HIDA YUKIO , HORI CHIKAHIDE , TUCHIDA SHINYA , KURODA MITSUHIKO , SUDO MASAKATSU
Published: January 2011

The rapid dipstick test of urine leukocyte esterase (LE) activity and nitrite has not been studied fully in pediatric clinical situations. We investigated the usefulness of the dipstick LE and nitrite test in the screening of urinary tract infection (UTI) in pediatric patients. Ninety‐two fresh urine samples were obtained from children suspected of having UTI. Leukocyte esterase activity and nitrite were measured in the urine specimens read by a photometer. Leukocytes were also counted on a disposable slide. Urine samples were examined for bacteriuria by the standard culture method. The results of the urine dipstick test of LE showed a close relationship with leukocyte counts on a counting chamber. Leukocyte esterase (‐) indicated leukocyte counts of less than 10/uL with a probability of 97% (58/60). Of the 22 urine samples with significant bacteriuria diagnosed by standard urine culture, the nitrite test did not detect bacteriuria in 10. While the sensitivities of the dipstick tests of nitrite (+) and LE ± or more for the diagnosis of significant bacteriuria were 55% (12/22) and 86% (19/22), respectively, the sensitivity and negative predictive value of the combined test were 100%. These results suggest that use of the dipstick test of LE and nitrite can avoid a large part of the cost incurred by urine culture and is useful for screening UTI in children.
Neuropathology and immunohistochemistry of the brain‐stem in neonates with congenital hydrocephalus: Comparative studies between aqueductal stenosis and Arnold‐Chiari malformation
Authors: FUKUMIZU MICHIO , TAKASHIMA SACHIO , BECKER LAURENCE E.
Published: January 2011

Neuropathological and immunohistochemical studies were done on the brain‐stem of neonates who had congenital hydrocephalus with aqueductal stenosis or Arnold‐Chiari malformation (ACM). The infants with aqueductal stenosis showed heterogeneity in their clinicopathological findings while the infants with ACM were relatively similar in neuropathological findings. There were prominent astrogliosis, decreased immunoreactivity with antisera to tyrosine hydroxylase and myelin basic protein in the periaqueductal area, and an increased reactivity with antiserum to substance P in the tegmentum of most patients with aqueductal stenosis and other malformations. In ACM, there was little gliosis in the tegmentum and periaqueductal area and minimal immunoreactivity of tyrosine hydroxylase, myelin basic protein and substance P. In both groups of cases, the cells in the periaqueductal region differ in neurotransmitter/neuromodulator immunoreactivity and degree of myelination reflecting a difference possibly in their maldevelopment.
The fluctuations of neuron‐specific enolase (NSE) levels of cerebrospinal fluid during bacterial meningitis: The relationship between the fluctuations of NSE levels and neurological complications or outcome
Authors: INOUE SHIGEAKI , TAKAHASHI HIROSHI , KANEKO KEN‐ICHIRO
Published: January 2011

Neuron‐specific enolase (NSE) is one of the glycolytic enzymes distributed exclusively in neurons. It was measured serially in the cerebrospinal fluid (CSF) of 10 children with bacterial meningitis during the illness using radio‐immunoassay.

The relationship between CSF‐NSE levels and neurological complications or outcome was examined. CSF‐NSE levels were significantly higher in the patients with bacterial meningitis than in the patients with the other central nervous system (CNS) infectious diseases, suggesting that CNS damage in those patients with bacterial meningitis was exacerbated. As CSF‐NSE levels increased to above 25 ng/mL in the acute phase, all patients except one had subdural effusion. In those patients whose CSF‐NSE level rose again during the illness, CNS complications or sequelae occurred. CSF‐NSE may be a useful prognostic factor for predicting CNS damage in childhood bacterial meningitis.
Increased levels of antibodies to food proteins in Down syndrome
Authors: REICHELT KARL L , LINDBACK TORE , SCOTT HELGE
Published: January 2011

An increased level of immunoglobulin A (IgA) antibodies to food antigens has been found in Down syndrome. Biopsy did not show any anomaly of the gut. Increased IgA levels against specific proteins reflect increased transmucosal transport of antigens. The antigens presented may be immunogenic peptides or intact antigens. In this paper, we wished to exclude an institutional cause such as infections and also age differences.
What are parents of obese children concerned about in their children?
Authors: YOSHINAGA MASAO , YUASA YOSHIHIRO , KITAHARA TOMOKO , SHIMAGO ATSUSHI , MIYATA KOICHIRO , SAMESHIMA KOJI , IMAMURA MASATO , ARIMA KATSURA , MATSUOKA KATSUMI
Published: January 2011

We retrospectively examined the issues that concern parents of obese children to determine the most effective means of motivating them to seek treatment for obesity in their children.

Children with an obesity index > 40%, aged six to 12 years, were screened in Kagoshima City in 1992. Parents were notified if their children needed an evaluation that included a family history and measurements of the blood pressure, total cholesterol, high density lipoprotein (HDL)‐cholesterol, atherogenic index (ASI), triglycerides, aspartate aminotransferase (AST) and alanine aminotransferase (ALT). Parents were informed of the results of the evaluation and invited to attend a lecture on the treatment of obesity in children.

A total of 378 obese children were evaluated. However, the parents of only 39 children attended the lecture. Children whose parents attended had higher mean total levels of cholesterol (190 ± 25 vs 175 ± 28, P < 0.01) and ASI values (3.2 ± 0.9 vs 2.7 ± 0.9, P < 0.02) than those whose parents did not attend. There were no significant differences in other factors. Only 4.2% of parents whose children showed no abnormal values, except for obesity, attended the lecture, compared with 20.3% (P< 0.01) or 16.9% (P< 0.05) of parents whose children had abnormal levels of cholesterol or abnormal ASI. Parents may be more concerned about hypercholesterolemia or arteriosclerosis than obesity per se. We should perhaps use the total cholesterol or ASI values, not just the severity of obesity, to motivate parents to enter their children into treatment programs for obesity.
Alagille syndrome with a spontaneous appearance of the interlobular bile ducts
Authors: FUJISAWA TAKUJI , KAGE MASAYOSHI , USHIJIMA KOSUKE , KIMURA AKIHIKO , ONO EIICHIRO , KATO HIROHISA
Published: January 2011

We report a rare case of a 10 year old girl diagnosed as having Alagille syndrome on the basis of a characteristic face, posterior embryotoxon, cholestasis, peripheral pulmonary artery stenosis and the absence of interlobular bile ducts in a liver biopsy at 1.5 years of age. Since 1.5 years old, she had been in good health without medication and the serum biochemical liver function tests indicated no progression of cholestasis. A second liver biopsy at 9.5 years of age showed normal interlobular bile ducts confirmed by anti‐keratin staining at each of the five examined portal areas. Alagille syndrome is usually associated with the progressive disappearance of interlobular bile ducts. The findings of interlobular bile ducts in the second liver biopsy were therefore rare and unique to this case.
Preschool sarcoidosis manifesting as juvenile rheumatoid arthritis: A case report and a review of the literature of Japanese cases
Authors: UKAE SUSUMU , TSUTSUMI HIROYUKI , ADACHI NORIAKI , TAKAHASHI HIROYUKI , KATO FUMIHIRO , CHIBA SHUNZO
Published: January 2011

Nine Japanese cases of sarcoidosis in children of 4 years of age or younger have been reported in the literature, including the case presented here. Clinically, preschool sarcoidosis is distinctly different from that of older children; it is characterized by a triad of skin, joint and eye lesions without pulmonary involvement. It is easily confused with juvenile rheumatoid arthritis which also presents the symptoms of arthritis and uveitis. We report on a patient with preschool sarcoidosis who was initially diagnosed as having juvenile rheumatoid arthritis. We recommend prompt skin biopsy to differentiate between these conditions.
New approaches to the study of human pediatric disease
Authors: SANDERSON IAN R
Published: January 2011

The intestinal epithelium may modulate childhood gastrointestinal disease activity, particularly in chronic inflammatory bowel disease. The epithelium is in direct contact with the intestinal lumen and is ideally placed to act as a communication between the intestinal environment and the immune cells of the mucosa. Interaction is possible through the production of cytokines by enterocytes and the expression of surface molecules on the basolateral membrane. The study of how luminal contents can modulate enterocyte gene expression is central to this process, but the molecular mechanisms by which such interactions occur are not well understood. This review discusses possible molecular pathways between the intestinal lumen and the enterocyte and how they can be examined experimentally. Finally, the importance of enterocyte gene expression in human health and disease is reviewed.
A review: The active peptide of lactoferrin
Authors: TOMITA MAMORU , TAKASE MITSUNORI , BELLAMY WAYNE , SHIMAMURA SEIICHI
Published: January 2011

A potent antimicrobial peptide, ‘lactoferricin’, was found to be generated upon gastric pepsin cleavage of lactoferrin. The active peptide consists mainly of a loop of 18 amino acid residues, derived from the N‐terminal region of the lactoferrin molecule. Like various other antimicrobial peptides that display membrane‐disruptive properties, it contains a high proportion of basic amino acid residues. A physiologically diverse range of micro‐organisms was tested and found to be susceptible to inhibition by this natural peptide including Gram‐negative and Gram‐positive bacteria, yeasts and filamentous fungi. Its antimicrobial effect against sensitive micro‐organisms was lethal. Electron microscopy studies revealed that it induces a profound change in cell ultrastructural features and causes substantial cell damage in bacteria and fungi. These findings suggest the possibility that active peptides of lactoferrin may have a role in the host defense against microbial disease. If produced in substantial quantities in vivo such peptides could have important physiological significance, especially in nursing infants.
Milk proteins, cytokines and intestinal epithelial functions in children
Authors: DESJEUX JEHAN‐FRANÇOIS , HEYMAN MARTINE
Published: January 2011

This paper discusses the relationship between food antigens, lymphocytes and the epithelial properties of the jejunum in children with cow's milk allergy. Experimental results indicate that increased protein permeability is not the primary cause of cow's milk allergy. Rather, results are interpreted as a secondary effect of an abnormal immunological response leading to mucosal inflammation and impairment of the endocytic process by the intestinal epithelial cells. Stimulation by cow's milk proteins caused the lymphocytes from infants with cow's milk allergy to release more tumor necrosis factor‐α TNFα than those from control infants. After appropriate antigenic stimulation, the cytokines released by the activated lymphocytes from these infants perturbed epithelial function, in particular its barrier capacity. Tumor necrosis factor a, together with gamma interferon are involved in these adverse effects. It is thought that bovine β‐lactoglobulin present in the intestinal lumen may be responsible for the secretory diarrhea observed in children with cow's milk allergy, as a consequence of stimulation of electrogenic chloride secretion. In addition, luminal foreign protein may stimulate the submucosal cells. As a consequence, the submucosal release of mediators, including lymphokines, might alter the intestinal epithelial barrier. In conclusion, in physiological conditions, the subepithelial tissue that comprises the immune system and many other systemic systems receive information on the antigenic content within the intestinal lumen via the intestinal epithelium.
Uptake of antigens: Role in gastrointestinal disease
Authors: WALKER W ALLAN
Published: January 2011

The intestine is exposed to a wide variety of macromolecules. Because macromolecules are antigenic, mechanisms have evolved in the gastrointestinal tract to regulate their absorption. Macromolecular uptake can be beneficial in delivering essential factors for growth and in sampling the antigenic milieu of the gastrointestinal tract. Specific transport mechanisms exist to execute this physiological absorption. However, inappropriate and uncontrolled antigen transport may occur in disease states, or as a prelude to disease states in the gastrointestinal tract. Such transport may result in immune responses that are harmful. In this review we examine both physiological transport of macromolecules through epithelia and through M cells. We also discuss uncontrolled transport and its relation to disease states. We conclude by examining the interrelationship between antigen transport and an altered immune system in the establishment of gastrointestinal disease.
Umbilical cord blood as a rich source of immature hematopoietic stem cells
Authors: URASHIMA MITSUYOSHI , HOSHI YASUTAKA , SHISHIKURA AKIHIRO , KAMIJO MAKOTO , KATO YOKO , AKATSUKA JUN‐ICHI , MAEKAWA KIHEI
Published: January 2011

To investigate immaturity of hematopoietic progenitor cells in umbilical cord blood mononuclear cells (CB‐MNC), the formation of macroscopic colonies and mixed‐cell colonies was assayed by methylcellulose culture with various combinations of cytokines (stem cell factor [SCF], interleukin [IL]‐3, IL‐6, granulocyte‐colony stimulating factor [G‐CSF], erythropoietin [EPO]) and compared with bone marrow (BM)‐MNC. Moreover, distribution of the subpopulations divided by CD34, CD38, HLA‐DR and CD33 was compared by flow‐cytometry.

Colonies derived from CB‐MNC were so large that they could be observed with the naked eye and consisted of a variety of types of hematopoietic cells. Mixed‐cell colonies were formed to a much greater extent in CB‐MNC than in BM‐MNC. Addition of EPO, IL‐3, and SCF had rapid effects on the growth of mixed‐cell colonies. The subpopulations of immature hematopoietic progenitor cells (CD34+, CD38−, HLA‐DR−), which are supposed to be able to differentiate into hematopoietic precursors and stromal cells, were significantly higher in CB‐MNC (8.7±6.6%) than in BM‐MNC (0.0±0.1%; P < 0.001). These results suggest that CB is a rich source of immature hematopoietic progenitor cells compared to BM.
Major histocompatibility complex antigens and immune mechanisms in steroid‐responsive nephrotic syndrome
Authors: MIR SEVGI , KUTUKCULER NECIL , KAVAKLI KAAN
Published: January 2011

Although the pathogenesis of steroid‐responsive nephrotic syndrome (SRNS) is obscure, involvement of an immune mechanism is often suggested. Further evidence of an immune basis for this disorder is an increased frequency of specific major histocompatibility complex (MHC) antigens. In the first part of this study, the phenotypic frequency of HLA‐A, ‐B, ‐C, ‐DR antigens were investigated in 30 children with SRNS and in 630 controls. In the second part, total T (CD3+ cells) and B lymphocytes (CD19+ cells) and the lymphocyte subsets (CD4+, CD8+ cells and their ratio) were studied in the same patients and in 30 healthy children. The investigations of all the patients were performed during the acute stage and 14 of 30 during remission stage. Human leukocyte antigens (HLA) were determined by standard microlymphocytotoxicity assay and lymphocytes were analyzed by flow cytometry. Human leukocyte antigens A3, DR4, DR7 and the haplotype HLA‐A2/B12 showed the strongest association with SRNS. In the studies for cellular immune disorder, CD3+ and CD8+ cells were found to be decreased significantly in the acute stage before beginning steroid therapy. No significant difference in lymphocyte subsets was observed in the remission stage without steroid or immunosuppressive therapy.
Effects of an acaricide on asthmatic children with house dust mite allergy
Authors: QUEK S C , CHONG A. , CONNETT G J , LEE B W
Published: January 2011

D'Allergen is a recently available acaricidal and allergen reducing agent. To study any beneficial effects of treating houses with this agent in asthmatic children with dust mite allergy, histamine bronchial responsiveness was measured in 18 children before and 6–8 weeks after their homes were treated with the agent. Comparisons were with nine similar asthmatics whose homes were not treated. The mean provocative concentration of histamine causing a 20% fall in forced expiratory volume in 1s (PC20) increased from 1.02mg/L to 2.07mg/L in all 18 children who were studied (1.01 doubling doses). Bronchial responsiveness was relatively unchanged in a well matched control group of nine children studied over comparable time periods (PC20 pre was 1.25 mg/L, PC20 post was 0.67 mg/L). The results suggest that treating homes with D'Allergen reduces bronchial reactivity in asthmatic residents with house dust mite allergy.
Circadian rhythm of blood pressure in children with reference to normal and diseased children
Authors: WATANABE TOSHIHIKO , NAGASHIMA MASAMI , HOJO YASUO
Published: January 2011

Circadian rhythms of blood pressure in children were investigated using ambulatory blood pressure monitoring (ABPM). The subjects were 58 normal children (26 boys and 32 girls), 10 children with orthostatic dysregulation (OD) consisting of (three boys and seven girls) and 13 children taking prednisolone (nine boys and four girls).

Systolic pressure in the normal group increased from early morning, peaked in the afternoon and gradually dropped thereafter. Blood pressure in the 22:00–05:30 h time range was significantly lower than in other time ranges.

Blood pressure in the OD group was generally lower than in the normal group, while that in the steroid group tended to be higher.

Systolic mid‐line estimating statistic of rhythm (MESOR) was significantly higher in boys than girls in both 10–12 year olds and 13–15 year olds according to Cosinor analysis. However, no increase in MESOR was observed in either boys or girls between the 10–12 year and 13–15 year age groups.

The OD group showed lower diastolic MESOR than the normal group and the steroid group showed higher systolic MESOR.

Seventy‐two per cent of those in the normal group manifested significant circadian rhythm. Only 50% of 10 12 year olds manifested circadian rhythm, whereas the figure for 13–15 year olds increased to 80–90%, suggesting that a pacemaker regulating circadian rhythm comes into effect at puberty.

The percentage of those who manifested circadian rhythm in the OD group and steroid group was about 60%, lower than in the normal group.
Minocycline‐induced hemolytic anemia
Authors: KUDOH TOORU , NAGATA NOBUO , SUZUKI NOBUHIRO , NAKATA SHUJI , CHIBA SHUNZO , TAKAHASHI TOMOYA
Published: January 2011

A case of drug‐induced immune hemolytic anemia is described. A 2 year old boy exhibited sudden anemia and hemoglobinuria after administration of minocycline (MINO). The specific immunoglobulin G antibody against MINO was demonstrated in the patient's serum by western blotting. This is a rare example where anti‐minocycline immune complex‐mediated hemolysis was responsible for an intravascular hemolytic process.
Twins with moyamoya disease
Authors: SARENUR TÜTÜNCÜOGLU , MEHMET KANTAR , VESILE ÖZTÜRK , NILGÜN YÜNTEN , YUSUF DUMAN , SABAHATTIN KULA , TUGRUL ÖZGÜR
Published: January 2011

Moyamoya disease is a progressive disease which involves the internal carotid arteries and its branches bilaterally. The disease is reported both in adults and in children. Moyamoya disease is frequently seen in Japanese patients having certain human leucocyte antigen (HLA) haplotypes including HLA‐Aw24, Bw46 and Bw54. Twin cases are rarely reported in the literature. We hereby present the first Turkish monozygotic twins with moyamoya disease whose HLA haplotypes are A2, A9, B21, Bw22, Bw4, Bw6, Cw3, and DR2, DR4, DRw52, DRw53, Dq7. The patients with advanced disease were given nifedipine and intravenous immunoglobulin (400mg/kg/d for 5 days). During the 11 months of follow‐up, the patients were attack free.
Kawasaki disease with a concomitant primary Epstein‐Barr virus infection
Authors: KANEGANE HIROKAZU , TSUJI TAKAO , SEKI HIDETOSHI , YACHIE AKIHIRO , YOKOI TOHRU , MIYAWAKI TOSHIO , TANIGUCHI NOBORU
Published: January 2011

A two year old boy exhibited not only clinical manifestations which suggested a recurrence of Kawasaki disease (KD) but also evidence of a primary infection by Epstein‐Barr virus (EBV) including tonsillitis, splenomegaly and atypical lymphocytosis in the peripheral blood. An inverted CD4/CD8 ratio in lymphocyte subsets suggested the presence of infectious mononucleosis (IM). Epstein‐Barr virus titers (viral capsid antigen‐immunoglobulin G 1:20; Epstein‐Barr virus‐associated nuclear antigen < 1:10) showed an acute EBV infection and the presence of EBV genome in the blood was determined by the polymerase chain reaction technique. In Japan, the peak incidence of KD and IM is in children under 4 years of age. From the investigation of EBV titers, it has been reported that some patients with KD develop an associated, unusual primary EBV infection. Kawasaki disease concurrent with a primary EBV infection as in this case, suggests the possibility of an etiologic agent related to the KD rather than to the EBV infection itself.
A case of tetrasomy 9p
Authors: ANDOU RURIKO , MIMAKI TAKASHI , OGIHARA TOHRU , TAMAI HIROSHI , MINO MAKOTO
Published: January 2011

A case of mosaic 9p tetrasomy (46,XX/47,XX, + dic[9] [q21]) is reported. Clinical manifestations of the patient were generalized hypotonia, severe mental retardation and characteristic dysmorphic features of 9p tetrasomy. A brief review of the literature is also included.
Analysis of seven pedigrees of childhood Wilson's disease characterized by abdominal symptoms
Authors: IKEI NOBUHIRO , ABE JUN , SHIN SADAHITO , KOHSAKA TAKAO
Published: January 2011

In a survey of childhood Wilson's disease (WD) characterized by abdominal symptoms, three patients with high levels of immunologically detectable ceruloplasmin (CP) in serum were found. These three cases were compared with typical cases of WD in which serum CP level was low. In order to clarify the cause of WD, serum CP levels were quantified by two methods, an immunological protein assay and an oxidase activity assay. Using the results of these two assays, WD cases were classified into three groups on the basis of CP content; the first group consisted of patients with low enzyme activity and low CP protein content, the second group consisted of patients with low enzyme activity and normal CP protein content, and the third group, those patients with normal enzyme activity and normal CP protein content. No significant difference in symptoms was observed between these three groups. Since relatively high levels of CP were detected in some WD patients, genetic variation in CP in WD patients was examined by restriction enzyme fragment length polymorphism analysis using CP cDNA. However, no large deletion in the CP gene was detected. Using four types of gene probes for chromosome 13 known to be related to WD, the DNA of WD patients was examined in a similar fashion, but no significant difference was observed between the groups.
Unique form of rickets with low serum 25‐hydroxyvitamin D in two normally nourished children
Authors: ASAMI TADASHI , KAWASAKI TAKUYA , UCHIYAMA MAKOTO
Published: January 2011

We present an unusual type of rickets involving two children: a 2 year old boy and a 15 month old boy, who presented with marked bowing of the lower extremities and bulging of costochondral junctions. Both children had normal growth, with their height and body weight greater than the 50th and 97th percentile for age. Roentgenograms of their extremities showed the typical changes of vitamin D refractory rickets. Serum alkaline phosphatase levels were elevated and serum levels of calcium and phosphate were both within the normal range. No primary cause for the rickets, including nutritional deficiencies, was found in the two patients. Characteristic findings were persistently low serum 25‐hydroxyvitamin D (25‐OH‐D) and normal 1,25‐dihydroxyvitamin D (1,25‐(OH)2‐D). Improvements in clinical and X‐ray findings were observed after either oral administration of 1 α‐(OH)‐D3 (9–15 μg per day) or massive vitamin D2 therapy (600 000 IU single injection). The low serum levels of 25‐OH‐D did not increase unless massive vitamin D2 therapy was also given. These two cases represent a unique form of rickets that does not meet the criteria for any type of previously known rickets.
Mononucleosis‐like illness in an infant associated with human herpesvirus 6 infection
Authors: KANEGANE CHIHARU , KATAYAMA KEITA , KYOUTANI SEIZO , KANEGANE HIROKAZU , SHINTANI NAOHISA , MIYAWAKI TOSHIO , TANIGUCHI NOBORU
Published: January 2011

Illnesses resembling mononucleosis, hematologically characterized by atypical lymphocytosis in the peripheral blood, are caused by other viral infections as well as by a primary Epstein‐Barr virus infection. Human herpesvirus 6, a newly isolated member of the herpesvirus group, can also cause a mononucleosis‐like illness. Illness associated with human herpesvirus 6 infection mostly occurs in immunocompetent adults. We observed a 3 month old infant who presented with marked atypical lymphocytosis and liver dysfunction. We examined serum samples to detect viral antibodies related to mononucleosis‐like illness. Only the titers of antibody against human herpesvirus 6 were elevated. Primary human herpesvirus 6 infection cannot only cause exanthem subitum or present in an inapparent form but can also cause an illness like mononucleosis, even in an infant.
Child abuse and child abuse studies in Japan
Authors: IKEDA YOSHIKO
Published: January 2011

Medical research on child abuse has a short history of only 20 years. However, the social and familial changes that have occurred since the Second World War have resulted in an increasing number of abused children throughout Japan. Reported cases represent only the tip of the iceberg, with many cases being ignored. This article discusses the history, definition, incidence, and social, familial, and psychopathology factors in the etiology of child abuse based on clinical experience and research surveys undertaken during the past 20 years. Suggestions for improving child abuse preventive and reactive services are also made.
Early intervention in case of child abuse: Co‐operation between the hot‐line and local facilities
Authors: SAITO SATORU
Published: January 2011

The Center for Child Abuse Prevention (CCAP), a citizens' organization, was started in May 1991 with such members as pediatricians, psychiatrists, lawyers and people working at child welfare offices. A hotline dealing with child abuse was set up immediately after the establishment of CCAP on 20 May 1991, and in the 2 years it has been in operation this hot‐line has received 3189 calls out of which we picked 30 cases that CCAP was consulted about by helping persons or therapists affiliated with such facilities as hospitals, public health centers, etc. in order to evaluate how CCAP co‐operates with these organizations.

We reviewed the CCAP records of these 30 cases, comparing them with the materials concerning other hot‐line calls, while at the same time interviewing the helping persons and therapists who had called in those cases, ascertaining their motives and the later course of the cases. This follow‐up study was begun in June 1992 so that the longest follow‐up period from the first call was 2 years and 7 months and the shortest 7 months.

A total of 73.3% of these 30 cases required emergency intervention, and 53.4% were extremely severe, life‐threatening cases of child abuse. The age distribution of these 30 victimized children peaked between 4 and 6, and 13 and 15 years. The perpetrator was the biological mother in 55.9% of the cases, the biological father in 29.4% and the stepfather in 8.9%. The abuse was physical in 58.3% of the cases, sexual in 8.9%, emotional in 5.6%, and involved neglect in 22.2%. These percentages differ from the general trend seen in the total hot‐line cases, but were very similar to those reported by the Child Guidance Center. In the majority (56.7%) of the cases CCAP was consulted about, CCAP went beyond its hot‐line service and co‐operated to intervene, organizing network sessions in which the helping persons and therapists involved could discuss how best to deal with the problem at hand in five of these cases. In 22 of these 30 cases, the persons involved are still co‐operating with CCAP after successful intervention through which the victimized children were rescued from critical situations.

It is vitally important to set up networks between the professionals of the various support agencies involved if we are to intervene successfully in cases of child abuse. Indeed, one should only proceed with the intervention once the various facilities involved have agreed on who should take care of therapy and/or care, and on how to proceed and at what pace.
Immunological unresponsiveness and apoptotic cell death of T cells in measles virus infection
Authors: ADDAE MICHAEL MARK , KOMADA YOSHIHIRO , ZHANG XAO‐LI , SAKURAI MINORU
Published: January 2011

The phenotypic alterations associated with T cells during measles virus infection have been demonstrated and an attempt has been made to show programmed cell death (PCD) of T cells activated in vivo. During the acute phase of illness, activated T cells increased rapidly. Memory T cells (CD45RO+), especially CD8+ memory T cells also tend to increase. During the recovery phase, CD8+ T cells declined rapidly, and naive (CD45RA+) T cells increased in numbers. The anti‐CD3 monoclonal antibody‐induced expression of interleukin‐2 receptor (CD25) was suppressed. However, the addition of phorbol 12‐myristate 13‐acetate (PMA) caused the significant recovery of CD25 expression. In addition, PCD of activated T cells from measles patients was induced in culture. After triggering of the T cell receptor‐CD3 complex, cells became more susceptible to PCD. Interestingly, the addition of PMA could inhibit PCD of activated T cells. Taken together, these data suggest unresponsiveness and activation‐induced cell death of T cells during the primary response to measles virus antigens, depending on the activation status of protein kinase C.
Autoantibodies and CD5+ B cells in childhood onset immune thrombocytopenic purpura
Authors: IYORI HIDEAKI , FUJISAWA KOHJI , AKATSUKA JUN‐ICHI
Published: January 2011

We evaluated platelet associated immunoglobulin (PaIg) G, PaIgM, platelet associated autoantibodies to platelet glycoprotein IIb/IIIa (Pa‐GPIIb/IIIa), the percentage of CD5+ B cells and the amount of platelet‐bound anti‐GPIIb/IIIa monoclonal antibody (mAb) in the peripheral blood of 29 patients with childhood onset chronic immune thrombocytopenic purpura (c‐ITP). The percentage of CD5+ B cells ranged from 2 to 8% (4.7 ± 2.0) in control patients and 1 to 18% (6.2 ± 4.2) in the ITP patients. There was no overall significant difference between the two groups, but the percentage of CD5+ B cells in six of the ITP patients was higher than the mean + 2 s.d. of the controls. There was a significant correlation between the percentage of CD5+ B cells and PaIgM (y = 1.73x + 13.4, r = 0.40, P < 0.05). This finding is the basis for the speculation that CD5+ B cells may play an important role in the production of PaIgM in vivo. There was no correlation between the amounts of PaIgG and Pa‐GPIIb/IIIa. This suggests that the amount of PaIgG does not accurately reflect of the amount of Pa‐GPIIb/IIIa. Furthermore, we have demonstrated that autoantibodies to GPIIb/IIIa are directed to more than one epitope.
Association of empty sella and neuroendocrine disorders in childhood
Authors: AKÇURIN SEMA , ÖCAL GÖNUL , BERBEROǦLU MERIH , MEMIOǦLU NIHAL
Published: January 2011

Empty sella syndrome (ESS) is a multicausal entity. The incidence of primary empty sella syndrome (PESS) in children with neuroendocrine dysfunction is not known. In the pediatric age group, frequency seems to have been underestimated. A total of 117 cases of neuroendocrine disorders, including complete growth hormone deficiency, primary hypothyroidism with pituitary resistance to thyroid hormone, obesity, central precocious puberty, hypothalamic hypogonadism and central diabetes insipidus, have been studied with computed tomography and/or magnetic resonance imaging of sellar region for etiologic evaluation. Twenty‐one patients were found to have PESS. We noted a high incidence of PESS in children with neuroendocrine dysfunction (17.9%). Children with neuroendocrine dysfunction should be investigated with respect to PESS, and children with PESS recognized coincidentally should be studied with the particular consideration of subclinical neuroendocrine dysfunction.
Effect of valproate and carbamazepine on visual evoked potentials in epileptic children
Authors: YÜKSEL ADNAN , ŞARSLAN OSMAN , DEVRANOǦLU KAZIM , DİRİCAN AHMET , HATTAT NEŞE , CENANİ ASIM , YALÇIN ERDİNÇ
Published: January 2011

The effects of carbamazepine (CBZ) and sodium valproate (SV) monotherapy on visual evoked potentials (VEP) were studied in 18 epileptic children receiving CBZ and nine epileptic children receiving SV. Pattern reversal VEP were determined before the administration of antiepileptic drugs (AED) and 1 year later during which time the patients had received AED. The VEP amplitude showed no consistent changes after 1 year of CBZ and SV therapy, but VEP P‐100 latencies were significantly prolonged after 1 year of CBZ therapy. We conclude that CBZ causes a slowing down of central impulse conduction and that VEP is useful to evaluate the effects of AED within the central nervous system.
Hemolytic anemia following high‐dose intravenous immunoglobulin administration
Authors: TAMADA KOH‐ICHI , KOHGA MASATOSHI , MASUDA HIDEKO , HATTORI KAZUMI
Published: January 2011

We describe two cases of hemolytic anemia following high‐dose intravenous human immunoglobulin administration in young females with idiopathic thrombocytopenic purpura. Inducement of hemolytic anemia by anti‐A and anti‐B antibodies in these cases necessitates the performance of a minor crossmatching procedure prior to immunoglobulin use to prevent the occurrence of this adverse reaction.
Neonatal alloimmune thrombocytopenia involving HPA‐5b (Bra): A rare Japanese Case
Authors: SUDA HIDETOSHI , OHTO HITOSHI , TOHYAMA YURIKO , MORITA SHOJI , ISHIJIMA AYAKO , ISHIKAWA KEN , CHIDA SHOICHI , MATSUDA ISAO
Published: January 2011

Neonatal alloimmune thrombocytopenia (NAIT) is caused by platelet antigen incompatibility between the mother and fetus. The frequency of NAIT varies among ethnic groups. In Caucasians, HPA‐5b (Bra) is the antigen that is second most frequently implicated. In Japan, NAIT due to anti‐HPA‐5b antibody is quite rare. The present case is the second case of Bra‐NAIT in Japanese and the first case serologically confirmed by monoclonal antibody‐specific immobilization of platelet antigens.
Paroxysmal kinesigenic choreoathetosis associated with prenatal brain damage
Authors: HAMANO SHIN‐ICHIRO , TANAKA YOSHIKO , NARA TAKAHIRO , NAKANISHI YOUKO , SHIMIZU MASAKI
Published: January 2011

We describe a 15 year old patient with paroxysmal kinesigenic choreoathetosis. Neurological examinations revealed a paresis of the right arm and hand that was similar to ulnar nerve palsy, a right homonymous hemianopsia and an ocular movement disturbance of smooth pursuit to left. Attacks of dystonic spasms began abruptly, usually following running, and lasted less than 5 min. Magnetic resonance imaging displayed a linear area of increased signal in the T2‐weighted images along the lateral margin of the left putamen, atrophies of the frontal and temporal opercula and a large porencephalic cyst in the left parieto‐temporo‐occipital region. A cerebral blood flow study with single photon emission computed tomography showed hypoperfusion of the lenticular nucleus and the regions corresponding to the atrophies and the porencephalic cyst. Electroencephalograms during the attacks could not demonstrate epileptic abnormality. Only the neuronal plasticity of an immature brain could explain the discrepancy between the observed huge lesions of the brain and the minor neurological symptoms present. Attacks of paroxysmal kinesigenic choreoathetosis might occur when the basal ganglia maturate to some extent, even if the lesions in the brain were caused before birth.
Inhaled nitric oxide therapy for persistent pulmonary hypertension of the newborn
Authors: FINEMAN JEFFREY R , ZWASS MAURICE S
Published: January 2011

Increasing evidence suggests that the pulmonary vascular endothelium is an important mediator of resting pulmonary vascular tone through the synthesis and release of a variety of vasoactive substances including nitric oxide (NO). In addition, pulmonary endothelial dysfunction (such as impairment of NO synthesis) is present in lung injury and may contribute to the pathophysiology of pulmonary hypertensive disorders. Recently, exogenously administered NO gas has been utilized to treat infants with persistent pulmonary hypertension of the newborn (PPHN). These preliminary studies suggest that inhaled NO is a promising new therapy for the treatment of infants with PPHN. Controlled clinical trials must now be performed to determine if the use of inhaled NO improves the long‐term outcome of patients with PPHN. Long‐term exposure must be monitored closely for potential toxicity which includes methemoglobinemia and lung injury secondary to peroxynitrite and nitrogen dioxide production.
Long‐term prognosis and prognostic factors of Japanese children with mesangial proliferative glomerulonephritis without IgA deposition
Authors: KAKU YOSHITSUGU , HISANO SATOSHI , YAMANE ICHIRO , HATAE KEN , UEDA KOHJI , SUEISHI KATSUO
Published: January 2011

Mesangial proliferative glomerulonephritis without IgA deposition (non‐IgA MesPGN) is commonly detected in biopsy specimens, but the clinicopathological correlation with the long‐term prognosis still remains obscure. The aim of our study is to elucidate the long‐term prognosis and the clinicopathological prognostic factors in patients with non‐IgA MesPGN. We mailed questionnaires to 122 patients with primary glomerulonephritis who were biopsied between 1963 and 1975. Information was obtained from 109 of these 122 patients and 55 were histologically rediagnosed as having non‐IgA MesPGN. The histological alterations of glomeruli and tubulointerstitium were classified into five grades. The mean period between the biopsy and the questionnaires was 20.5 years. Six of the 55 patients with non‐IgA MesPGN developed end‐stage renal failure and histopathological alterations of renal biopsies from these six patients were classified into grade IV or V. The presence of hypertension, heavy proteinuria of over 2 + or renal insufficiency at the biopsy was related to the severe histological changes, a grade of IV or V and to a poor prognosis. The renal survival rate of all the 55 patients was 88.3% at 20 years after the biopsy, while that of the 12 patients with severe histological changes was 48.6%. Although non‐IgA MesPGN is considered to be a heterogeneous disease, we cannot ignore the incidence of this disease and thus consider it to be one of the important primary glomerulonephritides that occur in childhood.
The first report of a patient with interrupted inferior vena cava, multiple post‐renal veins and azygos‐hemiazygos continuation
Authors: MATSUOKA SUGURU , ANTONIOU EMMANUEL EH , MORI KAZUHIRO , HAYABUCHI YASUNOBU , KURODA YASUHIRO
Published: January 2011

The first case of a patient with interrupted inferior vena cava, four post‐renal veins and an azygoshemiazygos continuation is presented. The complicated anomalies were omphalocele and atrial septal defect. Cine‐magnetic resonance imaging and cardiac catheterization showed an anomalous retroaortic left innominate vein, azygos‐hemiazygos continuation in the prerenal portion, arch formed renal vein in the renal portion and four embryonic vessels in the post‐renal portion.

Combination of these anomalies in the major venous system suggested that the inferior vena cava had failed to form and that the bilateral embryonic venous system, postcardinal and supracardinal veins persisted to be the systemic venous channels.
Advanced atrioventricular block associated with atrial tachycardia caused by Mycoplasma pneumoniae infection
Authors: UMEMOTO MASAKAZU , FUJII ISAO , TAKE HIROMICHI
Published: January 2011

Atrial tachycardia with atrioventricular (AV) block has been recognized as a common manifestation of digitalis toxicity. We describe here an unusual case of transient advanced AV block associated with atrial tachycardia in a 6 year old boy with evidence of Mycoplasma pneumoniae infection.
Application of a new perchloric acid treatment method to measure endotoxin by an endotoxin‐specific chromogenic Limulus test in neonatal septicemia
Authors: SUDA HIDETOSHI , MOROI CHIKAE , INADA KATSUYA , CHIDA SHOICHI , FUJIWARA TETSURO , YOSHIDA MASAO
Published: January 2011

The endotoxin in blood was measured to establish both the cut‐off value and to detect Gram‐negative septicemia. We employed a new perchloric acid treatment method using an endotoxin‐specific chromogenic Limulus test (Endospecy test). The cut‐off value of endotoxin in blood was 11.2 pg/mL. All cases of septicemia (n = 7) showed high values of endotoxin. Three cases were Group B streptococci, and two cases were Escherichia coli. The others were showed to be negative in blood cultures. The paired values of endotoxin titers during a 48 h interval were useful to evaluate the effectiveness of antibiotics.