bluuebunny/crossref_metadata_2025 huggingface dataset:
High intensity in the globus pallidus on proton and T2‐weighted MRI in a case of dentato‐ruburo‐pallido‐luysian atrophy of myoclonus epilepsy type
Authors: IMAMURA ATSUSHI , SUGAI KENJI , WATANABE SEIJI , HAMADA FUMIHIKO , KURASHIGE TAKANOBU , TAKASHIMA SACHIO
Published: January 2011

Although marked atrophy of the tegmentum of the mid‐brain is a known computed tomography (CT) and magnetic resonance imaging (MRI) feature in patients with dentato‐ruburo‐pallido‐luysian atrophy (DRPLA), this is the first report of marked high signal intensity of the globus pallidus on proton and T2‐weighted MR images in a patient in the early stage of DRPLA of the myoclonus epilepsy type. We suggest that the globus pallidus may be involved in the early stage of DRPLA of this type. Magnetic resonance imaging is considered to be useful for the early detection of involvement of the globus pallidus and in the clinical diagnosis of DRPLA.
Mucosal immunity and strategies for novel microbial vaccines
Authors: MESTECKY JIRI , MOLDOVEANU ZINA , NOVAK MIROSLAV , COMPANS RICHARD W.
Published: January 2011

Infectious diseases continue to be the leading cause of morbidity and mortality worldwide. Increased awareness of the fact that mucosal membranes are the most frequent portals of entry of pathogenic microorganisms has prompted studies aimed at the development of vaccination protocols and antigen delivery systems that would lead to an increased protection of mucosae. Although systemic and strictly local immunizations are of limited effectiveness in the induction of mucosal protection, ingestion or inhalation of antigens results in a generalized immune response manifested by the appearance of specific antibodies of the secretory immunoglobulin (Ig) isotype in external secretions due to the dissemination of IgA precursor cells from IgA‐inductive lymphoid tissues. Furthermore, additional inductive sites strategically positioned at the opening of the respiratory and digestive tracts may also be suitable targets for induction of immune responses at desired effector sites. To prevent degradation and the increase of ingested antigens absorption, novel strategies including enclosure of antigens into biodegradable microspheres, liposomes or their expression in viral and bacterial vectors and plants are currently being considered. Forthcoming technological advances in antigen preparation and routes of delivery will undoubtedly have a profound impact on immunization practices in the future.
Food sensitive enteropathy: Overview and update
Authors: WALKER‐SMITH JOHN
Published: January 2011

There are two types of food sensitive enteropathy; permanent and temporary. Celiac disease belongs to the former, the temporary food sensitive enteropathies of early childhood to the latter. A food sensitive enteropathy is characterized by an abnormal small intestinal mucosa while having the offending food in the diet; the abnormality is reversed by an elimination diet, only to recur once more on challenge with the relevant food. These disorders are temporary and may follow gastroenteritis. Cow's milk sensitive enteropathy is the most frequent and best known example but soy protein, egg, fish, chicken meat, ground rice and probably gluten may also temporarily damage the small intestinal mucosa in infancy. Treatment is with an elimination diet and protein hydrolysates as a cow's milk substitute. The reason why these enteropathies are temporary has not yet been established.
Early infant feeding and micro‐ecology of the gut
Authors: ZETTERSTRÖM ROLF , BENNET RUTGER , NORD KARL‐ERIK
Published: January 2011

Newborn infants are rapidly colonized by both aerobic and and anaerobic bacteria, initially with about 50% of each type. Several factors related both to the infant and its environment influence the composition of the intestinal microflora quantitatively as well as qualitatively. Major ecological disturbances are observed in newborn infants treated with antimicrobial agents. One way of minimizing the ecological disturbances, which may be seen in infants treated in neonatal intensive care units, is to provide them with fresh breast milk from their mothers and to use antimicrobial therapy only under strict clinical indications.
Absence of anti‐neutrophil cytoplasmic antibody (ANCA) in Henoch‐Schönlein purpura and immunoglobulin A nephropathy
Authors: KANEKO KAZUNARI , SUZUKI YOSHIHARU , YABUTA KEIJIRO
Published: January 2011

We have studied the presence of anti‐neutrophil cytoplasmic antibodies (ANCA) in 16 patients with Henoch‐Schönlein purpura (HSP) and 10 patients with immunoglobulin A nephropathy (IgAN).

An indirect immunofluorescence test using ethanol‐fixed neutrophils as a source of antigen and an enzyme‐linked immunosorbent assay using purified proteinase‐3 and myeloperoxidase as antigens were used. Neither immunoglobulin G nor immunoglobulin A (IgA) ANCA were found in any of the patients studied. It was shown that fluorescein‐isothiocyanate conjugated anti‐human IgA antibodies bound to ethanol‐fixed normal human neutrophils non‐specifically, suggesting the possibility that false positive staining was responsible for the previous reports.

We conclude that ANCA does not play an important role in the pathogenesis of HSP and IgAN.
Urinary excretion of acid‐soluble peptides in children with Duchenne muscular dystrophy
Authors: HIRANO KUMIKO , SAKAMOTO YOSHIMASA
Published: January 2011

In order to investigate the validity of the hypothesis that acid‐soluble peptides (ASP) in urinary excreta can be applied as an index of the protein catabolism of the whole body, we measured the urinary excretion of ASP in 46 normal children and in 18 children with Duchenne muscular dystrophy (DMD), in which continuous breakdown of skeletal muscle protein is presumed. The mean value of ASP in the children with DMD was significantly higher than that in normal controls.

The concentration of ASP was correlated with that of 3‐methylhistidine (3MH), which has been proposed as an index of muscle breakdown. This finding indicates that urinary ASP reflects the catabolism of body proteins. No correlation was observed between the concentration of ASP and that of 1‐methylhistidine (1MH), which is used as an objective index of meat and fish ingestion.

After the administration of bestatin, an inhibitor of leucine aminopeptidase, for 9 months, the urinary ASP concentration of children with DMD increased markedly. This increase is thought to have been directly caused by the bestatin itself.

Urinary ASP is therefore apparently a more conveniently applied index of protein catabolism than is urinary 3MH, which requires the application of several restrictions. However, it should not be applied when the effect of bestatin administration is evident.
Efficacy of captopril treatment in children with steroid‐resistant nephrotic syndrome
Authors: SÖZÜER DT , EMRE S. , TANMAN F. , SIRIN A. , NAYIR A. , UYSAL V.
Published: January 2011

We studied the efficacy of captopril, an angiotensin‐converting enzyme inhibitor in treating persistent moderate or severe proteinuria in children with various glomerular diseases other than minimal‐change nephrotic syndrome. Captopril was administered for 3 months to 15 normotensive and nonazotemic or mildly azotemic patients (12 boys, 3 girls) in whom corticosteroid and cytotoxic treatment had failed to induce remission. Urinary protein excretion decreased from 2873.14±1937.50 (mean ± s.e.m.) to 1684.71 ± 1463.13 mg/day (P < 0.05). The reduction in proteinuria was not related to a significant fall in systemic blood pressure or a change in renal function. Serum albumin did not rise and side effects due to captopril were not observed. We concluded that, in the short term, captopril can be used safely and effectively for decreasing the proteinuria of nephrotic children unresponsive to conventional therapy.
Exercise performance in children with hyperthyroidism
Authors: OTSUKA HIROKO , MURATA MITSUNORI , WAKASUGI KUNIYO , HARA MISUZU , IKETANI YUKO , TATARA KATSUNORI
Published: January 2011

An attempt was made to define exercise performance in children with untreated hyperthyroidism using treadmill stress testing. Data were obtained for five female patients and the results were compared with those obtained for 16 normal female subjects. There were no significant differences at rest between the hyperthyroidism group and the control group in oxygen uptake, minute ventilation and respiratory rate. On the other hand, heart rate in the hyperthyroidism group was significantly higher than that in the control group. During exercise, there were significant differences between the two groups in oxygen uptake, heart rate, minute ventilation and respiratory rate. Hyperthyroid patients did not show an abrupt increase in heart rate during the first 30s of exercise. Exercise stress testing can therefore reveal cardiopulmonary abnormalities that are not evident at rest in children with hyperthyroidism.
A case of common variable immunodeficiency associated with cyclic thrombocytopenia
Authors: KUBOTA MASARU , NAKAMURA KENJI , WATANABE KENSHIRO , KIMATA HAJIME , MIKAWA HARUKI
Published: January 2011

A 12 year old boy was found to be deficient in immunoglobulins (Ig) A, G2 and G4, and common variable immunodeficiency was diagnosed. He also had cyclic thrombocytopenia at intervals of approximately 28–30 days. His bone marrow revealed normocellular with slightly decreased megakaryocytes. In vitro colony assays showed markedly imparied megakaryocytopoiesis, erythropoiesis and granulopoiesis. Platelet‐associated IgG was elevated at his thrombocytopenic phase. Direct Coombs' test was repeatedly positive. Although not defined at present, we suggest the autoimmune nature of the disease.
Bone marrow transplantation for erythroleukemia: A case report
Authors: KOGAWA KAZUHIRO , SEKINE ISAO , MASUDA TOMOO , TAKADA YOSHINOBU , YOSHIOKA SHIGETAKE , HIROTA TAKAHISA , EGUCHI MITSUOKI
Published: January 2011

A 2 year old girl was diagnosed as having erythroleukemia (EL; M6 according to the French‐American‐British classification). After one course of low‐dose cytosine arabinoside (Ara‐C), complete remission was obtained. After three courses of low‐dose Ara‐C for consolidation, allogeneic bone marrow transplantation was performed from HLA‐identical sibling. The course of post‐transplantation was uneventful. Two years after transplantation, she continues to have durable engraftment and remission. In children with EL, conventional chemotherapy appears to be inadequate for producing durable long‐term disease‐free survival. Bone marrow transplantation should be considered in children with EL, in cases where suitable donors are available.
Consecutive cerebral MRI findings of acute relapsing disseminated encephalomyelitis
Authors: MIZUTANI KEN‐ICHI , ATSUTA JUN , SHIBATA TAKEO , AZUMA EIICHI , ITO MASAHIRO , SAKURAI MINORU
Published: January 2011

A seven year old girl with acute relapsing disseminated encephalomyelitis (ARDEM) was observed with serial magnetic resonance imaging (MRI) examination.

She had the first encephalomyelitis episode at the age of 2 years 9 months. She entered remission with steroid therapy. She suffered a recurrence at the age of 7.

Steroid therapy was instituted and consecutive magnetic resonance imaging (MRI) was performed. In spite of rapid improvement of neurological abnormality with steroid and steroid pulse therapy, disseminated high intensity lesions in T2‐weighted imaging which were considered as demyelination remained for a long time. Magnetic resonance imaging seems to be most suitable for evaluating the clinical condition and long‐term follow‐up of ARDEM.
Idiopathic infantile hypercalcemia discovered in the newborn period
Authors: SHIMIZU HIROKO , KODAMA SOICHI , TAKEUCHI ATSUKO , MATUI TADATAKA , NAKAO HIDETO , SAKURAI TAKASI , KOBAYASHI TADASHI
Published: January 2011

We report a male newborn with typical clinical signs of idiopathic infantile hypercalcemia (IIH); that is, hypercalcemia, hypercalciuria, an elfin face and nephrocalcinosis without giving Vitamin D3 supplementation to the patient. He had been treated with a vitamin D‐free, low calcium milk and rectal administration of exogenous calcitonin (elcatonin). The latter seemed to be more effective as a treatment for IIH. The serum calcium level came within the normal range and the serum 1,25‐dihydroxyvitamin D3 (1,25[OH]2D3) level decreased from 101.5 to 75.6 pg/mL with the treatments mentioned above. These results suggest that a high serum concentration of 1,25(OH)2D3 is part of the pathogenesis of IIH. However, we were not able to clarify the pathogenesis of the high serum concentration of 1,25(OH)2D3.
Kabuki make‐up syndrome associated with chronic idiopathic thrombocytopenic purpura
Authors: WATANABE TOHRU , MIYAKAWA MASAKO , SATOH MASAHISA , ABE TOKINARI , ODA YOSHIHIKO
Published: January 2011

Although susceptibility to infections in Kabuki make‐up syndrome (KMS) has frequently been reported, there have been few immunological studies. We describe a 14 year old girl with KMS exhibiting chronic idiopathic thrombocytopenic purpura (chronic ITP), including immunological studies.

Corticosteroid therapy was not effective therefore splenectomy was performed. The patient's platelet count increased transiently. Immunological studies revealed normal T cell functions and low serum immunoglobulin A (IgA) levels.

Because of the abnormalities of B cell functions in chronic ITP and low serum IgA levels in our patient, we speculate that there may be some abnormalities of humoral immunity in KMS.
Age‐related profile of neuroblastoma: A comparison of tumors detected by mass‐screening with those detected clinically
Authors: TANAKA TAKEO , TANABE MASAHIRO , SUGIMOTO TOHRU , IDA NORIAKI , SAWADA TADASHI
Published: January 2011

Infants with neuroblastoma are known to have a favorable prognosis compared to those over 1 year of age. However, there is little biological information about the age‐related heterogeneity of neuroblastoma. We evaluated the biological profile comparing cases detected by mass screening with those detected clinically.

A total of 238 patients with neuroblastoma were classified into four groups according to their age at diagnosis. Patients in group A were 0–5 months of age (n = 31). Patients in group B were detected clinically and were 6–11 months of age (n = 25). Patients in group C were 6–11 months of age and were detected by mass‐screening (n = 97). Patients in group D were more than 12 months of age (n = 85). The age‐related heterogeneity was evaluated by Kaplan‐Meier survival analysis, several clinical markers (neuron specific enolase, ferritin, vanillylmandelic acid and homovanillic acid) at diagnosis, tumor Ha‐ras p21 expression and tumor N‐myc amplification.

Infant neuroblastoma had unique features in comparison to neuroblastoma diagnosed over 12 months of age. Clinical outcome of the patients in groups A and C was quite favorable. Even patients with stage III or IV disease in group A had a favorable prognosis. However, stage IVs disease in group A was not necessarily associated with a good prognosis and the early death after diagnosis was also characteristic. The biological profile of tumors in group C was similar to that in group A but different from the profile in groups B and D. Tumors in group B had a biological profile intermediate between groups A and D.

Cases detected by mass screening (group C) provided a new clinical entity with a good prognosis. The difference in biological profiles might affect their clinical outcome of respective age group. These analyses confirm the significance of prognostic markers and may help to direct an appropriate modality of treatment for the individual patient.
Opsonic activity in cord blood: Comparison between normal and elevated immunoglobulin M infants
Authors: NISHIDA AKIRA , KIMURA HIROKAZU , NAKANO MINORU
Published: January 2011

In order to clarify the relation between opsonic activity (OA) in newborn infants and their gestational age, we determined OA by Cypiridina luciferin analog‐dependent chemiluminescence (MCLA‐CL). In addition, complement C3 which plays the major role in OA, was determined by the two‐dimensional immunodiffusion method.

Although OA and C3 in newborn infants increased in proportion to their gestational age, the levels were significantly lower in full‐term infants than in adults. In neonates with IgM levels of more than 30 mg/dL, at birth, both OA and C3 levels were significantly higher than in normal neonates, yet these levels were significantly lower than in adults.

These findings suggest that low opsonin activity is an important factor in neonatal susceptibility to infection. When OA is measured by chemiluminescence, MCLA‐CL is the preferred technique to luminol‐dependent chemiluminescence.
Nephrocalcinosis in newborn
Authors: ÜÇSEL RAIF , ÇOBAN ASUMAN , KADIOǦLU ATEŞ , BILGE ILMAY , METIN FAZILET , YÜCER GÜL , CAN GÜLAY
Published: January 2011

Nephrocalcinosis is uncommon in childhood, and almost always develops outside the newborn period. Over the last decade, nephrocalcinosis due to multifactorial pathogenesis has increased in very low birth‐weight infants. In this report a 23 day old full‐term baby with nephrocalcinosis secondary to distal renal tubular acidosis is described.
A case of distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type
Authors: YAMAMATO TOSHIYUKI , ASAI KOHICHI , OHTA SHIGERU , IESHIMA ATSUSHI
Published: January 2011

A male infant with distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type is reported. His clinical features were concordant with those of distal 9q trisomy syndrome. His karyotype was 46,XY, −10, +der (10) t(9;10) (q22.3;q24.3) confirmed by G‐banding and high resolution methods. His father had the balanced translocation t(9;10) (q22.3;924.3). He had a blood type of AB, despite his father's blood type of AB and his mother's blood type of O. The gene of ABO blood type is located at 9q34.1‐q34.2. Therefore, he would have received A and B type alleles from his father. 9q trisomy syndrome should be carefully investigated with ABO blood type.
Recurrent pneumococcal meningitis in a patient with transient IgG subclass deficiency
Authors: OHGA SHOUICHI , OKADA KENJI , ASAHI TAKAHIRO , UEDA KOHJI , SAKIYAMA YUKIO , MATSUMOTO SHUZO
Published: January 2011

We report the case of a 3 year old boy who exhibited recurrent serious infections with a transient imbalance of IgG subclass in the second year of life. He suffered from pneumococcal meningitis at 3 months, hepatitis at 9 months, and purulent arthritis at 11 months of age. The second episode of pneumococcal meningitis occurred at 14 months. Serum IgG level was normal for age. Low level of IgG2, undetectable level of IgG4 and negligible level of pneumococcus‐specific IgG1‐G2 antibodies were found. No other primary immunodeficiency was apparent. Serum IgG2‐G4 levels but not pneumococcus‐specific IgG1‐G2 titers increased by the age of 30 months. At that time, he was inoculated with a polyvalent pneumococcal vaccine along with acellular diphtheria‐pertussis‐tetanus vaccine. He acquired the immunity against these agents, and had no episodic infections in the following 2 years. This observation stresses the existence of transient IgG subclass deficiency associated with delayed development of the anti‐polysaccharide antibody response.
β‐Thalassemia with the IVS‐l‐1 (G → T) mutation in a Japanese girl
Authors: OKADA TAISUKE , TOMODA TAKASHI , MORITA HIDEO , WAKIGUCHI HIROSHI , KURASHIGE TAKANOBU
Published: January 2011

We analyzed the hemoglobins of a Japanese girl with β‐thalassemia and those of her immediate family. DNA sequencing of the cloned β‐globin gene from this patient revealed a point mutation at the IVS‐I position 1 (G → T). This rare point mutation has been found in Asian Indians, but this is the first reported Japanese case.
Fatal cytomegalovirus myocarditis in a seronegative ALL patient
Authors: ADACHI NAOTO , KIWAKI KOHJI , TSUCHIYA HIROYUKI , MIGITA MASAHIRO , YOSHIMOTO TOSHIMI , MATSUDA ICHIRO
Published: January 2011

Fatal cytomegalovirus (CMV) myocarditis occurred in a 2 year old boy with acute lymphoblastic leukemia (ALL) in remission. The patient showed mild hepatic dysfunction and a rapid progress of pancytopenia after complete remission had been achieved. At the fifth week of complete remission, he presented signs of heart failure such as tachycardia, S4 gallop on auscultation and decreased ejection fraction on echocardiography. However, no significant electrocardiographic changes were recognized. In addition to the cardiac dysfunction, the patient presented a marked tachypnea and dyspnea associated with hypoxemia. These were dramatically improved by methylprednisolone pulse therapy (30 mg/kg per day, for 3 days) and CMV high titer immunoglobulin (400 mg/kg per day, for 3 days). On the sixth day after signs of respiratory failure were improved, the patient suddenly presented a paroxysmal atrial tachycardia followed by a fatal ventricular fibrillation. Although we could detect neither a specific IgM antibody, a significant increase of IgG antibody, nor CMV genome by DNA hybridization techniques during the course of the illness, microscopic examination of necropsy specimens of the heart showed a marked disruption and disintegration of muscle bands associated with cytomegalic inclusion bodies. Polymerase chain reaction (PCR) yielded a 305 bp amplification product in the heart and lung tissues, supporting the view that myocarditis was caused by CMV.
Interferon‐α potentiates priming‐dependent FMLP‐induced neutrophil superoxide generation in a patient with chronic myeloid leukemia
Authors: TOMODA TAKASHI , OKADA TAISUKE , HISAKAWA HIROAKI , WAKIGUCHI HIROSHI , KURASHIGE TAKANOBU
Published: January 2011

13 year old girl diagnosed as having chronic myeloid leukemia (CML) was treated with interferon‐α (IFN‐α) alone and 4 months later hematological remission was obtained. In the course of the IFN‐α treatment there was neither infectious sign nor side effects. In this study we have examined the effect of IFN‐α on superoxide O2− generation by polymorphonuclear neutrophils (PMN). The PMN of the patient generated less O2− than PMN from normal controls. When patient PMN were cultured in the presence of 1000 U/mL IFN‐α, enhancement of the formyl‐methionyl‐leucyl‐phenylalanine induced O2−generation following priming with tissue necrosis factor‐α was observed. Over the course of the IFN‐α therapy, such O2− generation was gradually restored. It is suggested that CML PMN are in the resting condition in terms of their ability to generate O2− and that IFN‐α is effective in inducing O2− generation by CML PMN.
Clinical features of Bardet‐Biedl syndrome
Authors: ÖZER GÜLER , YÜKSEL BİLGIN , SÜLEYMANOVA DİLARA , ALHAN EMRE , DEMİRCAN NİHAL , ÖNENLİ NESLİHAN
Published: January 2011

Six patients with Bardet‐Biedl syndrome who have been followed in our clinics for the last 5 years are reported in this study. Of the five classic features of this syndrome; obesity and mental retardation were present in all cases, retinal disturbances were present in five, Polydactyly in three and hypogenitalism was observed in all four male patients. Renal involvement, often suggested as a cardinal feature of this syndrome, was described in two patients. Iron deficiency anemia occurred in three patients, two patients were of short stature, one patient presented with an empty sella, and in two patients clinodactyly was detected. The results are compared to previously published literature and discussed.
Child abuse viewed through the hot‐line in Osaka, Japan
Authors: KOBAYASHI MICHIKO , NAYA YASUKO , SUZUKI ATSUKO , KATO YOKO , HIRATA YOSHIKO , GOUGH DAVID
Published: January 2011

Cases of child abuse identified by a hot‐line telephone service and by a survey of health and welfare agencies are reported. Many consultations to the telephone hot‐line were from mothers concerned about maltreating their own children. In such cases, the extent of abuse was often mild to moderate, and many of those mothers were troubled about child care in the relatively segregated context of the nuclear family. In only a few cases did the callers indicate that the children's life was in danger. In the administrative survey, however, deaths occurred in 5% of the cases, and the abused children frequently had physical and psychological symptoms. There were also more frequent and more serious family problems in the administrative survey compared to the telephone consultation cases. The findings of these surveys suggest that many children and parents are suffering from child abuse in Japan, that many cases are currently not being identified by health and welfare agencies, and that a wide range of measures, from protection of severely abused children to prevention of abuse, are necessary.
A boy with psychosocial short stature followed up from infancy to adulthood
Authors: SUWA SEIZO
Published: January 2011

A boy with psychosocial short stature who has been followed up from the age of 11 months to adulthood is described. The boy was the product of an unwanted pregnancy. The emaciated short boy gained weight and height markedly during a short‐term stay at hospital, but lost weight and experienced minimal height gain at home. On the fourth hospital admission at the age of 6 years 3 months the boy weighed 10 kg and measured 85.7 cm, he was malnourished and exhibited strange behavior and had a voracious appetite. He was examined endocrinologically and provocative tests performed early after admission showed insufficient growth hormone secretion, although this recovered later at a time of catch‐up growth. The boy was reared in an orphanage from the age of 6 years 5 months until the age of 15 years 3 months. His growth rapidly caught up to a normal rate, his abnormal behavior disappeared, and he demonstrated an increased IQ. He attained 169.5 cm at the age of 17.5 years and possessed normal secondary sexual characteristics. After graduating from senior high school the patient has been living happily by himself without intervention from his mother, and is working in a Chinese restaurant. The impaired relationship between the boy and mother has never been restored. The record of growth and development described in this case is the longest ever reported.
Interferon‐γ therapy for infection control in chronic granulomatous disease
Authors: OHGA SHOUICHI , OKAMURA JUN , NAKAYAMA HIDEKI , NAGATOSHI YOSHIHISA , UEDA KOHJI
Published: January 2011

Interferon (IFN)‐γ was subcutaneously administered to four patients with chronic granulomatous disease (CGD) in order to evaluate its effects in controlling infection. Patients 1 to 3 were all males, while patient 4 was female. In patients 2 and 4, the length of infection‐associated hospitalization during the year of IFN therapy was significantly shorter than that during the whole observed period prior to IFN therapy. In patients 1 and 2, the length of hospitalization during a year of IFN therapy was shorter than that during 1 year prior to the therapy. Patient 3 exhibited no reduction in terms of the length of infectious disease during IFN therapy, because he suffered from a liver abscess before and during the therapy. As soon as the IFN therapy was stopped, patient 2 developed pneumonia and lymphadenitis, which were promptly relieved by readministering the agent. During 1 year of IFN therapy, patients 1, 2 and 4 showed no significant changes in either the nitroblue tetrazolium test, O2‐ production or the expression of NADPH oxidase components in neutrophils. On the other hand, the O2‐ generating ability of neutrophils from patient 3 slightly increased. Our limited observations suggest that IFN‐γ may be variably beneficial for infection control in CGD‐patients, irrespective of the in vitro phagocyte functions. A longer follow‐up time is needed to confirm the IFN response in CGD‐patients.
Outbreak of pertussis in highly immunized adolescents and its secondary spread to their families
Authors: AOYAMA TATSUO , HARASHIMA MITSUKO , NISHIMURA KUNIYASU , SAITO YUZURU
Published: January 2011

An outbreak of pertussis was recognized in a highly immunized sixth‐grade class of schoolchildren. Among 43 children aged 11–12 years in the class, 38 had been immunized with three doses or more of DTP containing whole‐cell pertussis vaccine, two with two doses of DTP and three children were unimmunized. The last DTP vaccines had been given 6–10 years before the outbreak. A total of eight children with pertussis suffering paroxysmal coughing attacks for 3 weeks or more were identified, seven being fully immunized and one unimmunized. Among the eight cases, two were confirmed by both culture and serology and one by serology alone. The attack rate in fully immunized children was 18.4% (7/38). Secondary spread of pertussis was identified in five of the households from which the eight patients originated. A total of six cases of pertussis from these five households were identified, and two of these were culture‐confirmed. These observations suggest that vaccine‐induced immunity weakens considerably 6–10 years after vaccination, and that booster immunization with DTP instead of DT is therefore recommended for the control of pertussis.
Bronchial hyper‐responsiveness to inhaled histamine in children with congenital heart disease
Authors: TSUBATA SHINICHI , ICHIDA FUKIKO , MIYAZAKI AYUMI , HASHIMOTO IKUO , HAMAMICHI YUJI , TAKAYANAGI MIKI , MURAKAMI GYOKEI , OKADA TOSHIO
Published: January 2011

In order to assess bronchial responsiveness in patients with congestive heart failure secondary to congenital heart disease, we performed a histamine inhalation test while monitoring transcutaneous oxygen tension and compared the respiratory threshold to histamine with that obtained in patients with bronchial asthma. The inhalation test was performed by doubling concentrations of histamine solution for 2 min at 1 min intervals. The respiratory threshold of histamine was defined as the minimal concentration causing a drop in transcutaneous oxygen tension greater than 10% from baseline. Six of 10 patients with congenital heart disease and all of 12 patients with bronchial asthma had bronchial hyper‐responsiveness to histamine. The mean of histamine concentration was 2750μg/mL and 937μg/mL, respectively. During the histamine inhalation test, respiratory resistance gradually increased in congenital heart disease patients. This was measured by the linear slope of transcutaneous oxygen pressure (‐1.08 ± 0.75 mmHg/min), whereas in the bronchial asthma patients it rapidly decreased at the inflection point (‐4.19 ± 1.86 mmHg/min). We conclude that children with congestive heart failure had bronchial hyper‐responsiveness. We suggest bronchial hyper‐responsiveness to inhaled histamine in congestive heart failure was caused by the gradual increased respiratory resistance, which was different from that of bronchial asthma.
Long‐term change of anti‐acetylcholine receptor antibody in patients with myasthenia gravis after thymectomy
Authors: HAYASHI MASATOSHI , MANABE KOHJI , TAKAOKA TOMOHIKO , YAMADA ITARU , KIDA KAICHI , MATSUDA HIROSHI , YOSHINAGA JUNJI
Published: January 2011

Anti‐acetylcholine receptor antibody (AChR Ab) plays an important role in the pathogenesis of myasthenia gravis (MG). We investigated the change of anti‐AChR Ab titer after thymectomy of 10 MG patients including five patients whose age at onset was younger than 16 years. Anti‐AChR Ab titer was increased in four of six patients with remission and three of four patients without remission. Change of anti‐AChR Ab titer in individual patients showed an increase occurred 1–4 years after thymectomy. It is likely that thymectomy influences immune response and induces autoreactive lymphocytes and autoantibodies.
Measles encephalomyelitis in a patient with a history of vaccination
Authors: MATSUZONO YOSHIHIRO , NARITA MITSUO , SATAKE AKIRA , TOGASHI TAKEHIRO , ITAKURA OSAMU , OZUTSUMI KUNIO , IGUCHI MASAHIRO
Published: January 2011

Secondary vaccine failure (SVF) of measles is generally believed to run a milder course of illness than an ordinary course of infection. Severe complications such as central nervous system involvement have rarely been reported. A 12 year old girl, who had received a live attenuated measles vaccine 10 years earlier, developed an encephalomyelitis in the absence of symptoms indicative of ordinary measles such as Koplik spots. Anti‐measles hemagglutination inhibition (HI) titer and measles IgM and IgG anitbody titers were measured in a commercial laboratory. Measles virus genomic sequence was detected by polymerase chain reaction. Both serum and cerebrospinal fluid (CSF) samples obtained at acute phase already showed extremely high titers of HI (x 8192 in serum and x 1024 in CSF, respectively) and IgG antibody along with the presence of IgM antibody. Polymerase chain reaction detected the measles virus genomic sequence in the acute phase CSF. The patient's definite history of measles vaccination, high titers of HI and IgG antibodies observed at the very early stage of illness and the clinical course indicated that this patient had an encephalomyelitis due to SVF of measles. It is suggested that measles virus can be a pathogen of encephalitis without symptoms indicative of ordinary measles in individuals who received live attenuated measles vaccines.
A boy with atrophic thyroiditis of prepubertal onset, who was positive for TSH‐binding inhibitor immunoglobulins
Authors: NAKAGAWA YUICHI , TOYA KAZUHIKO , NASUDA KAORU , IIJIMA SHIGEO , KUBOTA AKIRA , NATSUME HIROMUNE , TAKEUCHI RIKA , IGARASHI YOSHIO
Published: January 2011

A 12 year old boy was admitted to our hospital because of short stature. From the age of 7, his growth velocity decreased and he manifested intolerance to low temperatures, hoarseness, dry skin, and slowness of thought and physical movement. On admission, his height was 129.8 cm (‐3 s.d.) and his body weight was 43.2kg (‐0.5 s.d.). His clinical features also included relaxation phase of tendon reflexes, periorbital puffiness and cold skin but no struma. His bone age was 9 years. His serum thyroxine (T4), tri‐iodothyronine (T3), free T4 and free T3 were low, while his thyrotropin was high. He was positive for antithyroglobulin antibodies, antimicrosomal antibodies, and TSH‐binding inhibitor immunoglobulins. He was diagnosed as having atrophic thyroiditis. We also determined the HLA haplotypes of his family members. His father's HLA haplotypes were A2, BW61(a) and A24, BW52(b), while his mother's haplotypes were A24, BW52(c) and A30, BW61(d). The HLA haplotypes of both the patient and his younger brother showed a and d, while the patient's elder brother's HLA haplotypes showed b and c. His family members all had normal thyroid function, but his father was positive for antimicrosomal antibodies. In summary, we describe a rare case where the onset of hypothyroidism was prepubertal, where the pathogenesis may have involved TSH‐receptor blocking antibodies, and where the inheritance of the disease may have been from the paternal side of the family.
Acquired cytomegalovirus infection and blood transfusion in preterm infants
Authors: XU DAKANG , YONETANI MASAHIKO , UETANI YOSHIYUKI , NAKAMURA HAJIME
Published: January 2011

The urinary excretion of cytomegalovirus (CMV) DNA, amplified by polymerase chain reaction using two pairs of primers for late antigen (LA) and major immediate‐early antigen (MIE), and serum CMV IgM were examined in 85 pre‐term infants (birth‐weight less than 2000 g) on admission and monthly until 6 months after birth. Of these 85 infants, 27 had blood exchange transfusions (BET) and 28 had bolus blood transfusions two to nine times. Fifteen of 27 infants underwent BET with blood that had been filtered through Pall RC100 leukocyte removal filter; the other 12 with unfiltered blood. Neither urinary CMV DNA nor serum CMV‐specific IgM was detected at birth in any of the 85 pre‐term infants; during the first 6 months after birth urinary CMV DNA, for both MIE and LA, appeared in 22 of the 85 infants (25.9%) and CMV IgM was positive in 14 of the 85 (16.5%). Nine of the 12 (75%) infants who received BET of unfiltered blood showed a significantly higher prevalence of urinary CMV DNA compared to the infants in the other three groups (i.e., those who received no blood transfusion, those who had bolus blood transfusions, or those who received BET of filtered blood; P < 0.01 in each instance). In a logistic regression model, CMV, DNA urinary excretion was significantly associated with the mode of blood transfusion (unfiltered BET), and the Odds ratio was 38.9 (95% confidence interval, 9.4–160). There was no significant association with other independent variables such as gender, mother's seropositivity, gestational age, birth‐weight or delivery mode. In addition breast milk feeding was not likely to have influence on the high incidence of CMV infection among the infants who received the BET with unfiltered blood. In regions where the incidence of CMV seropositivity is high, as it is in the Japanese population, reduction of white blood cell and cellular blood components, such as platelets, through filtration is a convenient and effective way to successfully reduce the occurrence of acquired CMV infection in high‐risk neonates.
Different kinetics of antibody responses between IgA and IgG classes in nasopharyngeal secretion in infants and children during primary respiratory syncytial virus infection
Authors: TSUTSUMI HIROYUKI , MATSUDA KAZUKO , YAMAZAKI HIROFUMI , OGRA PEARY L , CHIBA SHUNZO
Published: January 2011

The secretory antibody responses in 34 infants and children (20 days‐17 months old) with lower respiratory tract disease following primary respiratory syncytial virus (RSV) infection were determined using a sensitive tissue culture enzyme linked immunosorbent assay. None of the patients in the acute phase showed IgA antibody responses. In contrast significant IgG antibody responses which were thought to be maternally derived were observed in infants younger than 2 months of age. In the convalescent phase sample, significantly high IgA antibody responses were observed in all patients except one, and there was no significant difference in magnitude of antibody activity between patients younger than 8 months and patients older than 8 months. However, IgG antibody responses in infants younger than 8 months were significantly lower than in subjects 8 to 17 months old. Notably, infants younger than 2 months developed no significant IgG antibody activity in the convalescent phase. These observations suggest that the antibody activity which contributes to recovery from primary infection by RSV in younger infants may be IgA rather than IgG class antibodies. These observations also suggest that the presumptive immunosuppression mediated by maternally derived antibodies may predominantly influence the IgG antibody response rather than the development of local IgA antibody activity.
Interleukin‐2 receptor positive T and B cells in children with acute severe asthmatic attack
Authors: NABETANI MAKOTO , YAMASAKI TAKEMI , KAMEDA AIJU , OKAMOTO OSAMU , KISHIMOTO TAKUMI
Published: January 2011

Subpopulations of interleukin‐2 receptor (IL‐2R)‐positive CD4 and CD8 T cells and IL‐2R+ CD20 B cells in the peripheral blood lymphocytes as well as serum concentrations of soluble IL‐2R (sIL2R) were measured in children aged 1–7 years who suffered acute severe asthmatic attack. Subpopulations of CD4+ IL‐2R+ cells, CD8+ IL‐2R+ cells and CD20+ IL‐2R+ cells in the peripheral blood lymphocytes at acute severe asthmatic attack phase were significantly higher than those at non‐asthmatic attack phase (P < 0.02, P < 0.03 and P < 0.02, respectively).

Subpopulations of CD20+ IL‐2R+ cells in the peripheral blood lymphocytes significantly decreased 5–10 days after acute severe asthmatic attack (at recovery phase, P < 0.02) and were significantly correlated with clinical severity of asthmatic attack (P < 0.05).

These results indicated that activation of both T cells and B cells was important in the pathogenesis of acute asthmatic attack in young children.
Relation between serum hepatitis C virus‐RNA levels and efficacy of interferon‐β therapy
Authors: MINOHARA YUTAKA , KATO TATSUO , KANKI KOUZOU , GOSHIMA TOSHIRO , NAKAJIMA NATSUKI , KAKU HILOHUMI , ARIMOTO YUTAKA , GOSHIMA FUMIE , ISHIKO HIROAKI
Published: January 2011

We performed two courses of interferon‐β (IFN‐β) to a child with chronic hepatitis C. A complete response was not obtained by the first interferon treatment, however, the results of the second treatment differed from those of the first. Hepatitis C virus (HCV)‐RNA remained negative and both aspartate aminotransferase and alanine aminotransferase levels remained normal after completion of the second course. From these results we estimated that HCV‐RNA levels before IFN therapy could be significantly associated with the efficacy of this treatment. The serum level of HCV‐RNA was 106 copies/50 μL before the first treatment, but was 103 copies/50 μL before the second course.

We conclude that IFN therapy to children with hepatitis C should always be directed at providing a cure. Even if the clinical effects of the first course are minimal decreasing quantities of HCV‐RNA still offer hope for cure by subsequent readministration.
Immunogenicity and reactogenicity of the component acellular pertussis vaccine produced by a combination of column purified pertussis toxin and filamentous haemagglutinin
Authors: KIMURA MIKIO , KUNO‐SAKAI HARUMI , KAMIYA HITOSHI , UEDA KOHJI , ISOMURA SHIN , KOIKE MICHIO , KATO TATSUO , OZAKI TAKAO , HIROSE MIZUO , EGAMI TSUNEYOSHI
Published: January 2011

This is the report on a prospective, single blind, comparative study of a component acellular pertussis vaccine produced by a combination of detoxified, column purified pertussis toxin (PT) and filamentous hemagglutinin (FHA) combined with diphtheria and tetanus toxoids (DTcaP) and the traditional acellular pertussis vaccine produced with essentially the same method as described by Sato with DT (DTaP) of the same manufacturer. A total of 616 infants and children received DTcaP and a total of 289 received DTaP. In all age groups for both vaccines values of serum antibodies to PT and FHA after two doses of the vaccines were comparable to those of convalescent sera. Incidences of systemic and local reactions were, in general, not greatly different between DTcaP and DTaP recipients.

In Japan the use of traditional acellular vaccines replaced whole cell vaccines in 1981. Protective antigens of Bordetella pertussis have now been specified and thus component vaccines have become theoretically possible. This is the first component vaccine which has been developed in Japan. Several other component vaccines are now under investigation in the world.
Effect of cyclosporin A on human bone marrow granulocyte‐macrophage progenitors with anti‐cancer agents
Authors: ISHIDA YASUSHI , MATSUDA HIROSHI , KIDA KAICHI
Published: January 2011

Cyclosporin A (CyA) overcomes P‐glycoprotein (P‐gp) associated multidrug resistance (MDR). P‐gp expression is frequently observed among, not only various cancer cells, but also several normal tissues including bone marrow progenitor cells. These findings lead us to examine whether CyA enhances the myelotoxicity of anti‐cancer agents. Bone marrow mononuclear cells were incubated with anti‐cancer agents (vincristine, VCR; doxorubicin, ADM; etoposide, VP‐16; cytarabine, Ara‐C; methotrexate, MTX) and a concentration of CyA (0.5, 5.0 μg/mL). The methylcellulose assay for granulocyte‐macrophage progenitors (CFU‐GM) was conducted using the post‐treated cells. There was no significant toxicity for marrow CFU‐GM formation after 72 h incubation with CyA (84–108% of control). The inhibitory concentration that reduced colonies by 50% (IC50) was 12 nmol/L for VCR, 6 nmol/L for ADM, 220 nmol/L for VP‐16, 15 nmol/L for Ara‐C and 35 nmol/L for MTX, respectively. For VCR, ADM and VP‐16, the number of CFU‐GM was unchanged with the addition of CyA at 0.5 μg/mL concentration. In contrast at 5 μg/mL CyA, the number of CFU‐GM (% of control) was reduced significantly (P < 0.05 or P < 0.01). With MTX and Ara‐C, the number of CFU‐GM was unchanged after addition of CyA, even at 5 μg/mL concentration. We conclude CyA may therefore enhance cytotoxic drug sensitivity in MDR tumor cells at a clinically achievable concentration (0.5 μg/mL) without marrow toxicity.
The relationship between serum transaminase activities and fatty liver in children with simple obesity
Authors: NOGUCHI HIROO , TAZAWA YUSAKU , NISHINOMIYA FUJIHIKO , TAKADA GORO
Published: January 2011

To determine hepatic diseases in obese children, biochemically and histologically, 11 obese patients with abnormal serum transaminase activities were subjected to this study. Fat accumulation in the liver was semiquantitatively graded, and histologically the 11 patients were classified into four groups; fatty liver, fatty hepatitis, fatty fibrosis and fatty cirrhosis. All patients had fat deposition in liver specimens, the grade of which did not significantly correlate with the degree of obesity. The grade of fat deposition in the liver specimens also did not significantly correlate with either serum transaminase activities or GOT/GPT ratio. Five patients were grouped into the fatty liver group, three into the fatty hepatitis group, and the remaining three patients into the fatty fibrosis group. However, no significant differences were found among the three histologically classified groups in terms of serum transaminase activities or GOT/GPT ratio. The usefulness of serum transaminase activities and GOT/GPT ratio was limited in predicting the severity of fat deposition or histological abnormality in pediatric obese patients.
Developmental change in carotid artery blood flow waveform by component analysis in children
Authors: KOJO MASANOBU , OGAWA TERUYUKI , SONODA HIROTOMI , YAMADA KATSUHIKO
Published: January 2011

The carotid artery blood flow waveform (CABFW) is regarded as a summation of cardiac impulse responses. These impulse responses are divided into several components through a two‐dimensional autoregressive modelling approach. Using this approach, we determined the developmental change in CABFW in 94 normal subjects from the neonatal period to adolescence. Our analysis demonstrated that: (i) the total power of impulse response increased significantly with increasing age. The component of impulse response was divided into six groups according to the damping frequency: group I (0 Hz), group II (1–5 Hz), group III (5–8 Hz), group IV (8–13 Hz), group V (13–17 Hz) and group VI (> 17 Hz); (ii) the power‐density and the damping time of group I and II impulse response increased significantly with increasing age; (iii) the power‐density and percent power of group III impulse response and power‐density of group IV impulse response increased significantly with increasing age. Our results indicated that CABFW contained some regular impulses and that group I, II, III and IV, which were influenced by several factors, including cardiac contraction and the compliance and frictional forces of the carotid artery, appeared to be important to the developmental change of CABFW in children.
Cerebral infarction in acute promyelocytic leukemia at initial presentation
Authors: SAITOH EMI , SUGITA KENICHI , KUROSAWA HIDEMITSU , KUROSAKI MOTOYUKI , EGUCHI MITSUOKI , FURUKAWA TOSHIHARU , NAKAJIMA CHIKAKO , KOBAYASHI YASUAKI
Published: January 2011

We report on a 3 year old girl with acute promyelocytic leukemia (APL) with cerebral infarction due to disseminated intravascular coagulation (DIC) at initial presentation. She was hospitalized because of unconsciousness and petechiae on the chest wall and extremities. Cerebral ischemia and infarction were found on computed tomography scan and magnetic resonance imaging. Peripheral bood content was hemoglobin 7.3 g/dL, white blood cells 1.0 × 103cells/μL (31% blasts) and platelet count was 12 × 103cells/μL. Fragmented erythrocytes were frequently observed on May‐Giemsa stained blood smears. Bone marrow aspirates showed normal cellularity, with 60.4% blasts, containing faggot cells. The blasts were positive for peroxidase. Therapy was begun; however, the patient died 1 week after admission.