bluuebunny/crossref_metadata_2025_split huggingface dataset:
Masked ureteral duplication with ectopic ureter detected by magnetic resonance imaging
Authors: KANEKO KAZUNARI , OHTSUKA YOSHIKAZU , SUZUKI YOSHIHARU , YABUTA KEIJIRO , YAMATAKA ATSUYUKI , MIYANO TAKESHI 
Published: January 2011

AbstractThe usefulness of magnetic resonance imaging (MRI) in the detection of a masked duplex kidney system is described. A 9 year old girl was admitted to our hospital for urinary incontinence. She was found to possess an ectopic ureter associated with complete ureteral duplication. She had been evaluated for incontinence at several hospitals, but neither ultrasound sonography nor intravenous pyelography detected any abnormalities. Only cystoscopic examination and MRI detected the anomalies. Partial nephroureterectomy resolved the patient's incontinence. MRI is useful for the detection of ureteral duplication and should be used on cases in which this condition is suspected, prior to performing invasive procedures.
Frequency of 985A‐to‐G mutation in medium‐chain acyl‐CoA dehydrogenase gene among patients with sudden infant death syndrome, Reye syndrome, severe motor and intellectual disabilities and healthy newborns in Japan
Authors: NAGAO MASAYOSHI 
Published: January 2011

AbstractThe prevalence of the 985A‐to‐G mutation in the medium‐chain acyl‐CoA dehydrogenase (MCAD) gene among Japanese patients with sudden infant death syndrome, Reye syndrome, unknown fatty acid oxidation disorders and severe motor and intellectual disabilities was studied using the PCR/Nco‐I method for molecular diagnosis. A frequency study of this common mutation was also conducted on blood samples and left over Guthrie cards from 329 healthy newborns in Japan. Neither heterozygotes nor homozygotes for the 985A‐to‐G mutation were identified among both patients and controls. The result of the present study accord with previous reports that MCAD deficiency is a common disorder in Caucasians, but quite rare among Japanese. Therefore, newborn mass‐screening for MCAD deficiency using this method will not be practical in Japan. However, it still seems necessary to investigate a child with fatty acid oxidation disorder for the presence of MCAD deficiency, using both biochemical and molecular genetic methods.
Ventricular arrhythmia and possible myocardial ischemia in late stage Kawasaki disease: Patient with a normal coronary arteriogram
Authors: NAKADA TOSHIMASA 
Published: January 2011

AbstractRecent research reveals that some patients with a normal coronary arteriogram present with myocardial ischemia in the late stages of Kawasaki disease (KD). The present paper reports a patient who developed ventricular arrhythmia and possible myocardial ischemia in the late stages of KD but whose coronary arteriogram was normal. The onset of KD was at 2 years of age but cardiac involvement including coronary arterial lesion was not detected during the acute stage. At 11 years of age, the Holter electrocardiogram showed both frequent ventricular premature contractions and ventricular tachycardia. The single photon emission computed tomogram demonstrated possible myocardial ischemia. Possible myocardial ischemia was considered to be one of the causes of the ventricular arrhythmia. Although the prognosis for the majority of patients without apparent coronary arterial lesion is excellent, regular follow up must be performed on patients with a history of KD.
SF‐1: A key regulator of development and function in the mammalian reproductive system
Authors: IKEDA YAYOI 
Published: January 2011

AbstractThe orphan nuclear receptor steroidogenic factor 1 (SF‐1) was isolated as a transcription factor expressed specifically in the mouse primary steroidogenic tissues. SF‐1 expression occurs at the earliest stages of adrenal and gonadal development and the expression pattern is sexually dimorphic in gonads during sexual differentiation. The two hormones required for male differentiation, testosterone and Müllerian‐inhibiting substance, are regulated by SF‐1. Analyses of knockout mice lacking SF‐1 by gene targeting disruption demonstrated that the SF‐1‐disrupted mice lack adrenal glands and gonads, supporting the suggestion that SF‐1 is an essential regulator of the endocrine development and differentiation. Additionally, SF‐1 is expressed in the pituitary gonadotropes and the ventrolateral hypothalamic nucleus, which are higher levels of the reproductive regulatory axis, of both adults and embryos. These tissues are also affected in SF‐1 knockout mice, indicating that SF‐1 plays extended roles at all levels of the reproductive axis, by regulating more genes involved in reproductive function and development.
Interleukin 8 and granulocyte elastase α1 proteinase inhibitor complex in the tracheobronchial aspirate of infants with chronic lung disease following respiratory distress syndrome
Authors: TAKASAKI JIRO , OGAWA YUNOSUKE 
Published: January 2011

AbstractIn order to elucidate the role of interleukin 8 (IL‐8) on the development of chronic lung disease (CLD) in neonates following an episode of respiratory distress syndrome (RDS), serial and simultaneous measurements of the concentration of IL‐8 and granulocyte elastase α1 proteinase inhibitor complex (E‐α1 PI) in the tracheobronchial aspirate of very low birthweight infants with RDS were conducted. The concentration of IL‐8 and E‐α1 PI in infants with CLD was low in the first 48 h of life, but dramatically increased after 48 h. The concentration of IL‐8 between 48 h of life and day 5 was significantly correlated to the fraction of inspired oxygen concentration (F1o2) within 48 h of age, but not to the mean airway pressure. Interleukin 8 seemed to stimulate neutrophils to release granulocyte elastase which, in turn, caused lung tissue injury, resulting in the development of CLD. It is suggested that high F1o2 is an important factor causing IL‐8 production in the lung.
Calorie intake in sick versus respiratory stable very low birthweight babies
Authors: LEE JIMMY KF , YU VICTOR YH 
Published: January 2011

AbstractA comparison between the calorie intake and energy source of sick versus respiratory stable very low birthweight (VLBW, less than 1500 g) babies was made to ascertain the time taken for them to achieve adequate daily calorie intake. It was an observational study of 23 consecutive VLBW babies in which sick respiratory unstable babies were defined as those who required ventilation beyond 72 h of life. Data were collected on the daily fluid and calorie intake for 30 days of life, and beyond if necessary until the babies achieved full enteral feeding and calorie intake of more than 100 kcal/kg per day. Growth parameters at the time of transfer or discharge were also analyzed. In the study, there were 14 sick VLBW and 9 respiratory stable babies with a mean birthweight of 1027 g and 1212 g, respectively. Their mean gestational age (28.7 weeks vs 31.2 weeks), mean age when calorie intake of 100 kcal/kg per day was achieved (19.8 days vs 7.0 days), mean duration of parenteral nutrition (17.1 days vs 2.7 days), mean age when enteral feeds commenced (8.9 days vs 1.7 days) and mean age when full enteral feeding was established (20.6 days vs 7.3 days) were statistically different for the two groups. For the respiratory unstable babies, parenteral nutrition provided more energy than milk until 15 days of life. The average daily energy intake of 100 kcal/kg per day was only achieved by 30 days of life in this group. In the respiratory stable group, milk provided more than 100 kcal/kg per day from 10 days of life. There were no significant differences in somatic growth with regard to bodyweight, length and head circumference for these two groups of babies at the time of transfer or discharge. The daily calorie intake of sick VLBW babies was suboptimal even with the use of parenteral nutrition. The respiratory stable babies, through enteral feeding, easily achieved the recommended daily calorie intake.
Thyroid functions in mild and severe forms of sickle cell anemia
Authors: EVLİYAOǦLU NURDAN , KILINÇ YURDANUR , SARGIN ÖZOǦUL 
Published: January 2011

AbstractIn the present study, the weight, height, bone age and growth indices of 24 children with homozygote sickle cell anemia were measured and the relationship of these parameters to thyroid function was evaluated and compared with 14 healthy controls in the same age group. The patients consisted of two groups with either mild (n = 12) or severe (n = 12) clinical courses. There was no difference between both patient groups or with the control group with respect to weight (P > 0.05). However, the difference between the mean height percentiles of the homozygote‐severe group and the control group was found to be statistically significant (P < 0.05). The bone age remained 41.6% behind normal for age in all homozygote sickle cell anemia patients. The serum T3 and T4 levels of all patients showed no significant differences from those of the control group (P > 0.05). These results show that patients with severe clinical courses may have short stature but their thyroid hormones are within normal limits during the first decade of life.
Possible role for a polysaccharide antigen shared between Streptococcus pyogenes and S. mutans in the pathogenesis of poststreptococcal glomerulonephritis
Authors: OKADA KANAME , HIROTA KATSUHIKO , ZHANG RUI JIN , YASUTOMO KOJI , KUHARA TAKASHI , OTA FUSAO , KAGAMI SHOJI , KURODA YASUHIRO 
Published: January 2011

AbstractStreptococcus mutans has been shown to share a polysaccharide (PS) antigen with S. pyogenes strains isolated from patients with acute poststreptococcal glomerulonephritis (PSGN), using a monoclonal antibody f‐77 reactive with the PS.1 To investigate the pathogenetic role of the shared PS in PSNG experimental nephritis was induced in animals. Rats were immunized thrice with heat‐killed cells of S. mutans or S. pyogenes, followed by an intravenous injection of live cells of S. pyogenes. Histologic examination showed that both animal groups had comparable degrees of diffuse proliferative nephritis characterized by immune deposits. The shared PS antigen was detected in glomeruli of all nephritic rats by immunofluorescence using monoclonal antibody f‐77. Furthermore, all nephritic rats had an elevated antibody titer to the shared PS antigen. These results suggest that prior sensitization (infections such as dental caries) to S. mutans modulates immune responses to subsequent S. pyogenes infections and induces immune‐complex disease (PSGN) through the shared PS antigen.
Effects of an acaricide on mite allergen levels in the homes of asthmatic children
Authors: CHEW FOOK‐TIM , GOH DANIEL YAM‐THIAM , LEE BEE‐WAH 
Published: January 2011

AbstractPrevious study by the current authors has shown that treating homes with D'Allergen, an acaricidal agent, can reduce bronchial hyper‐reactivity in asthmatic children with house dust mite allergy. In the present study, the effects of a single D'Allergen treatment on the levels of major dust mite allergens, Der p I and Der f I was evaluated, and the duration of its effectiveness in the environment determined. Twenty randomly selected homes were treated with the acaricide and ten remained untreated. Dust samples were collected from mattresses, upholstered sofas and carpets of these homes before and 1, 2 and 4 months after treatment. The samples were then assayed for Der p I and Der f I allergens using a sandwich enzyme immunoassay. The results showed that D'Allergen was effective in reducing dust mite allergen levels in all three niches by 1.5–22.3 times below baseline values. This effect, however, was only present for 2 months, and the dust mite allergen levels increased to those of the baseline by the fourth month after treatment. These results indicated that repeated applications of the acaricide were required at 2–3 monthly intervals to obtain optimal effectiveness.
Trial of docosahexaenoic acid supplementation on a Japanese patient with a peroxisome biogenesis defect
Authors: SUZUKI YASUYUKI , SHIMOZAWA NOBUYUKI , IMAMURA ATSUSHI , FUKUDA YUKO , ICHIHASHI HIROSHI , ORII TADAO , KONDO NAOMI 
Published: January 2011

AbstractA female Japanese patient diagnosed with peroxisome biogenesis defect (PBD), who had hypotonia and craniofacial dysmorphism, was given supplementation of docosahexaenoic acid (DHA). Accumulation of very long chain fatty acids was revealed, and a diagnosis of PBD was made at 2 months of age because of the absence of peroxisomes, a defect in peroxisomal β‐oxidation enzymes and a decreased level of DHA in the erythrocytes. Supplementation of DHA was introduced at 3 months of age. For the first several months, psychomotor development was fairly good. The patient could laugh, brush off a blanket and play with toys at 6 months of age. However, neurological regression and convulsions occurred after 7 months of age. After recurrent respiratory infections and disturbance of the circadian rhythm, the patient died of liver failure and disseminated intravascular coagulopathy at 20 months of age. DHA may have a favorable effect on the early development of patients with PBD, but neurological deterioration cannot be prevented. Patients with a milder phenotype would be better candidates for DHA supplementation.
Neuraminidase deficiency presenting as a nephrosialidosis: The first case detected in Poland
Authors: TYLKI‐SZYMANSKA ANNA , ŁUGOWSKA AGNIESZKA , CZARTORYSKA BARBARA 
Published: January 2011

AbstractA defect of lysosomal neuraminidase (sialidase N‐acetyl‐neuramine acid hydrolase EC 3.2.1.18) leads to a wide spectrum of phenotypes, the most severe of which is nephrosialidosis. A 4‐year‐old boy of related parents, born at term with hydrops fetalis, is reported. Hydrocephalus was detected at 2 months of age. The child's course over 3 years was characterized by slow growth and psychomotor development. He had mild hepatosplenomegaly, joint restriction, gingival hypertrophy, lens opacities and cherry‐red spot. Coarse facial features and depressed nasal bridge were discreet.At the age of 3.5 years, he developed gradual progressive edema, decreased activity and increased fatigue. A diagnosis of nephrotic syndrome was made because of massive proteinuria. Thin‐layer chromatography of urinary oligosaccharides revealed the presence of several abnormal sialyloligosaccharides. The diagnosis was confirmed by measurement of neuraminidase activity in cultured skin fibroblasts.
Common acute lymphoblastic leukemia preceded by hypercalcemia in an infant
Authors: OHOISHI SATORU , KOSHIBU TATUO , KAZAMA HIROMI , SHINOMIYA NORIAKI , AOKI TUGUTOSHI 
Published: January 2011

AbstractA case of common acute lymphoblastic leukemia (ALL) preceded by hypercalcemia was observed in an infant. A high blood level of parathyroid hormone related protein (PTH‐rP) was detected during the first examination at Ohashi Hospital. Treatment with antineoplastic agents led to complete remission. The blood PTH‐rP level decreased as a result of treatment. PTH‐rP produced by leukemic cells was thought to be the cause of the hypercalcemia.
Distribution of human herpesvirus 6 and varicella‐zoster virus in organs of a fatal case with exanthem subitum and varicella
Authors: UEDA TAKESHI , MIYAKE YOKO , IMOTO KOJI , HATTORI SHIGENORI , MIYAKE SUSUMU , ISHIZAKI TORU , YAMADA AKIRA , KURATA TAKESHI , NAGAI TAKAO , SUGA SADAO , ASANO YOSHIZO 
Published: January 2011

AbstractThe distribution of human herpesvirus 6 (HHV‐6) and varicella‐zoster virus (VZV) was examined in autopsy samples from a fatal case with both virus infections. A 9‐month‐old boy developed convulsive seizures followed by macular skin rashes, rapidly progressed to brain death, and died 15 days after the onset, when signs of varicella were noted. An isolation of HHV‐6 from blood and evaluation of antibody activities to various viral agents including HHV‐6 were performed before his death. Postmortem examinations included: (i) isolation of HHV‐6 and VZV from tissues or organs; (ii) detection of both virus antigens in tissues or organs by an indirect immunofluorescent assay using monoclonal antibodies to both viruses; (iii) amplification of both viruses and human herpesvirus 7 DNA sequences by a nested polymerase chain reaction assay; and (iv) endonuclease digestion of amplified products of HHV‐6 DNA for differentation of variants A and B.Human herpesvirus 6 DNA was detected in peripheral blood mononuclear cells (PBMC) and plasma obtained at the eruptive stage but present only in PBMC 15 days after, indicating the primary infection with HHV‐6, although the virus was not isolated from the same blood sample and a significant rise in the antibody titers to HHV‐6 was not observed. Both virus antigens and DNA were detected in various tissues or organs obtained at autopsy, but only VZV was isolated from these samples, suggesting disseminated infection with both viruses in an infant. All the amplified products of HHV‐6 DNA were variant B. Among the findings for the distribution of virus antigens, it was noteworthy that HHV‐6 antigen was demonstrated in the endothelial cells of small vessels in the frontal lobe of the brain. There was no evidence of HHV‐7 infection. These data indicate that the primary HHV‐6 infection closely followed by the primary VZV infection had the potential hazard of an unexpected and apparently life‐threatening event, in which disseminated infections with both viruses were noted in multiple tissues or organs including the brain.
White blood cell count, C‐reactive protein and erythrocyte sedimentation rate in respiratory syncytial virus infection of the lower respiratory tract
Authors: SAIJO MASAYUKI , ISHII TAKUMA , KOKUBO MASAYO , MURONO KOICHI , TAKIMOTO MASATOSHI , FUJITA KOZO 
Published: January 2011

AbstractLaboratory findings such as white blood cell (WBC) count, C‐reactive protein (CRP) concentration and erythrocyte sedimentation rate (ESR) level in patients with bronchiolitis, bronchopneumonia and lobar pneumonia caused by respiratory syncytial virus (RSV) were studied. The diagnosis of having RSV infection of the lower respiratory tract was made on the presence of RSV antigen in nasopharyngeal specimens by means of enzyme immunoassay, on chest X‐ray appearances and clinical manifestations. The WBC counts in the lobar pneumonia cases (n = 25, 12 288 ± 6296/mm3) were significantly greater than those for the bronchiolitis (n = 52, 9562 ± 2720/mm3) and bronchopneumonia (n = 43, 8369 ± 3714/mm3) cases. The concentrations of CRP in lobar pneumonia cases (n = 25, 6.5 ± 7.3 mg/dL) were significantly greater than those in the bronchiolitis (n = 52, 1.9 ± 2.0 mg/dL) and bronchopneumonia (n = 43, 2.1 ± 2.4 mg/dL) cases. The ESR levels in the lobar pneumonia cases (n = 24, 43.8 ± 29. mm/h) were also significantly higher than those in the bronchiolitis (n = 34, 20.1 ± 12.3 mm/h) and bronchopneumonia (n = 40, 24.7 ± 15.9 mm/h) cases. There were no significant differences in the WBC counts, the CRP concentrations and ESR levels between the bronchiolitis and bronchopneumonia cases. These results suggest that the RSV lobar pneumonia cases are coinfected with some bacterial organisms more heavily than in the RSV bronchiolitis and bronchopneumonia cases.
Survey of family history on allergy in 1‐year‐old and 6‐year‐old children
Authors: MIKAWA HARUKI , FUKUSHIMA YOICHI , BABA MINORU 
Published: January 2011

AbstractA retrospective survey of a family history of allergy employing a special questionnaire for children was performed. The survey included 1‐year‐old (n = 267) and 6‐year‐old children (n = 410) with allergies as well as 1‐year‐old (n = 313) and 6‐year‐old children (n = 329) without allergies. An ‘Allergy Risk Score’ (ARS) for each subject was calculated according to the history of allergy in three family members: father, mother and a sibling. Each family member was scored as 2.0 (overt history of allergies), 1.0 (provable), 0.5 (possible) or 0 (absent), and the ARS of the subjects was calculated as the total of the family members' scores. The ARS of children in the allergy groups was statistically higher than that of the control groups in both age groups. The ARS increased with an increase in the odds ratio (an approximate value of the risk), especially in 6‐year‐old children. An ARS calculated on the basis of family history could be a useful and practical index for estimating the risk of allergy development in children, and may be helpful in predicting and preventing allergies in infants and children.
Pathological improvement of IgA nephropathy and Henoch‐Schönlein purpura nephritis with urokinase therapy
Authors: WATANABE TAKESHI , TAKAHASHI SHORI , NAKAJO SAORI , HAMASAKI MINORU 
Published: January 2011

AbstractThe pathological findings of 13 patients with immunoglobulin A (IgA) nephropathy or Henoch‐Schönlein purpura nephritis before and after urokinase (UK) administration were investigated retrospectively. The mean activity index value decreased significantly at the time of the second biopsy compared with that of the biopsy before UK treatment. The mean chronicity index value, which was considered to reflect the renal outcome, before and after UK treatment did not change significantly, although it improved significantly in six patients. Immunofluorescence microscopy showed that the immune deposition of C3 decreased, but that of IgA and fibrin‐related antigen were unchanged, by UK therapy. These results suggest that UK may prevent the mesangial proliferation associated with IgA nephropathy and Henoch‐Schönlein purpura nephritis not only by its fibrinolytic action, but also by other mechanisms, such as digestion of the mesangial matrices.
Comparison of two fetal growth curves in screening for high‐risk neonates
Authors: SAKATA YASUKO , NISHIDA HIROSHI 
Published: January 2011

AbstractThe International Statistical Classification of Diseases and Related Health Problems (ICD‐10) defines the 10th percentiles on the fetal growth curve as the cutoff point for light‐ and small‐for‐gestational‐age infants, instead of the mean body weight −1.5 standard deviation (SD) on the fetal growth curve, which has been used in Japan since 1983. Data on a 5260 sample of term neonates who were born during the 30‐month period between January 1993 and June 1995 were obtained. The clinical usefulness of the 10th percentiles and mean bodyweight −1.5 SD cutoff on the fetal growth curve as screening criteria for high‐risk neonates of light‐ and small‐for‐gestational‐age infants was evaluated. There was a statistically significant higher morbidity rate among the neonates whose birthweight was below mean bodyweight −1.5 SD than for those whose birthweight was below the 10th percentile cut off. But there was no significant difference between the morbidity rate of neonates whose birthweight was between mean bodyweight −1.5 SD and the 10th percentile cutoff and the morbidity rate of neonates whose birthweight was above the 10th percentile cutoff. Therefore, mean bodyweight −1.5 SD on the fetal growth curve is a more effective means of screening for high‐risk light‐ and small‐for‐gestational‐age infants than the 10th percentile cutoff on the fetal growth curve.