bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics of juvenile dermatomyositis in Japan
Authors: KOBAYASHI SHINICHI , HIGUCHI KAORU , TAMAKI HISASHI , WADA YASUYUKI , WADA NORIYUKI , KUBO MASAKATSU , KOIKE YUICHI , NAGATA MASATO , TATSUZAWA OSAMU , FUJIKAWA SATOSHI 
Published: January 2011

AbstractQuestionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker‐type JDM, Brunsting‐type and fulminant‐type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting‐type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker‐type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant‐type JDM.
Non‐furosemide‐related renal calcifications in premature infants with bronchopulmonary dysplasia
Authors: TOFFOLO ANTONELLA , TREVISANUTO DANIELE , MENEGHETTI STEFANO , TALENTI ENRICO , ZACCHELLO GRAZIELLA , ZANARDO VINCENZO 
Published: January 2011

AbstractRenal calcification is a known complication of long‐term furosemide therapy in infants with bronchopulmonary dysplasia (BPD). In a prospective study the clinical course and long‐term renal sequelae of renal calcifications of 19 consecutive premature neonates (birthweight < 1250 g) with bronchopulmonary dysplasia who did not receive furosemide were examined. Infants were divided into two different groups on the basis of ultrasound evidence of renal calcifications (RC group) or absence of renal calcifications (NRC group). Serial examinations, performed at the age of 1, 2, 3, 6, 9 and 12 months, showed that 12 infants at the mean age of 68.5 ± 12.8 days of life had renal calcifications (63%), and 3 of them had nephrolithiasis; 8 had bilateral renal calcifications. Among the 9 survivors, 2 had chronic renal calcifications at the age of 9 months; however, all normalized at the age of 12 months. Twelve infants received hydrochlorothiazide and spironolactone (63%), 17 had prolonged courses of xanthines and dexamethasone (89.5%), while furosemide was not part of the routine pharmacological administration. Statistical analysis showed that birthweight, gestational age, Apgar score and length of parenteral nutrition were comparable in the RC and NRC group infants. Mean serum creatinine, creatinine clearance, fractional sodium excretion and urinary calcium excretion values during the 12‐month study period were comparable in the RC and NRC groups. Mechanical ventilation and hospital stay length were instead associated with renal calcification occurrence. The strongest indicator of renal calcification risk for this high‐risk population is the severity of the unresolved acute lung disease, where different facets of respiratory management, other than the addition of furosemide, represent sufficient stimuli and renal injury to potentiate stone formation.
Interleukin 8 and granulocyte elastase in the tracheobronchial aspirate of infants without respiratory distress syndrome or intrauterine infection and development of chronic lung disease
Authors: TAKASAKI JIRO , OGAWA YUNOSUKE 
Published: January 2011

AbstractTo elucidate the mechanism of the development of chronic lung disease (CLD) in infants without respiratory distress syndrome or intra‐uterine infection, we serially measured the concentrations of interleukin 8 (IL‐8) and granulocyte elastase α1 proteinase inhibitor complex (E‐α1 PI) and elastase activity in the tracheobronchial aspirate of very low birth weight infants without respiratory distress syndrome or intra‐uterine infection until day 28. IL‐8 concentration and elastase activity between day 21 and 28 in infants who developed CLD later were significantly higher compared with those in infants who did not develop CLD. E‐α1 PI concentration between day 25 and 28 in infants who developed CLD later was significantly higher compared with those in infants who did not develop CLD. The area under the curve of the IL‐8 and E‐α1 PI concentrations and elastase activity between day 1 and day 28 in infants with CLD was significantly higher than those in infants without CLD. These data suggest that the lung tissue injury caused by the enzymes from neutrophils accumulated and activated by IL‐8 also play an important role in the development of this type of CLD.
Platelet activation in congenital heart diseases
Authors: OLGUN NUR , UYSAL KAMER MUTAFOǦLU , IRKEN GüLERSU , üNAL NURETTIN , üNDAR BüLENT , AKKOÇ NURULLAH , AKÇORAL ADNAN , SARIALIOǦLU FAIK , ÇEVIK NAMIK 
Published: January 2011

AbstractThe research presented here investigated platelet activation in cyanotic and acyanotic congenital heart diseases (CHD). Children with cyanotic CHD are prone to both thrombosis and hemorrhage. However, patients with acyanotic CHD may also have a mild bleeding disorder. The platelet activation in CHD was investigated in support of a hypothesis that platelet activation may play a role in the hemostatic abnormalities reported in these patients. Platelet activation was determined by using flow cytometry with anti‐CD62 monoclonal antibody (mAb), which has been shown to be a specific marker of platelet activation. Thirteen children with cyanotic CHD, 33 children with acyanotic CHD and 17 healthy children serving as controls were studied. Platelet activation was significantly higher in the cyanotic group and also in the acyanotic group compared with the healthy children (P = 0.0000 and P = 0.019, respectively). In the cyanotic group, platelet activation showed a direct correlation with arterial O2 saturation (SaO2) (P = 0.014). There was no correlation between platelet activation and erythrocyte related parameters in either group. Platelet activation occurs in CHD, particularly in patients with cyanotic CHD (even in patients with no evidence of clinical thrombosis) and it may play a role in the pathogenesis of thrombotic disorders seen in these patients.
Extrahepatic portal venous obstruction in childhood: Etiology, clinical and laboratory findings and prognosis of 34 patients
Authors: GüRAKAN FIGEN , KOĈAK NURTEN , YüCE AYSEL , ÖZEN HASAN 
Published: January 2011

AbstractExtrahepatic obstruction of the portal vein is a well known cause of portal hypertension in childhood, that causes severe morbidity. We evaluated 34 children (24 boys, 10 girls, age 4.5 months to 12 years, mean 5.5 ± 3.8 years) with this diagnosis, to define the clinical picture, laboratory changes, diagnostic tools and therapeutic modalities.Gastrointestinal bleeding was the commonest mode of presentation (64.7%), with the second being splenomegaly. The cause of the obstruction could be determined in 38.2% (13/34) of the subjects. At the beginning of the study the main diagnostic procedure was splenoportography although in more recent years pulsed duplex Doppler ultrasonography has been used. The follow up period was a median of 5 years (range 1–11 years). The mean number of bleeding episodes was 4.7 ± 5.9 (range 1–26), while nine patients never bled. There was no mortality. Ten patients underwent surgery, while sclerotherapy was performed on 10. Twenty‐one patients received beta‐blocker drugs. No difference was found among these therapeutic modalities.It is well established that the major risk for children with extrahepatic portal vein obstruction is gastrointestinal bleeding which is tolerated quite well. Surgery should be indicated only in children where bleeding cannot be controlled by medical means including sclerotherapy.
Genetic analysis of isolated persistent hypermethioninemia with dominant inheritance
Authors: NAGAO MASAYOSHI , OYANAGI KAZUHIKO 
Published: January 2011

AbstractWe describe a type of mild hypermethioninemia due to a point mutation in the MATA1 gene, which was inherited dominantly in a family. Three patients coming from the same family pedigree were detected by the presence of isolated hypermethioninemia on a mass‐screening program. The measurement of methionine adenosyltransferase (MAT) activity in a patient's liver revealed a partial deficiency of hepatic MAT with a reduction in the Km for methionine. Single strand conformation polymorphism (SSCP) analysis and direct sequencing of the patients' genomic DNA revealed a G to A mutation at nucleotide 791 that converts Arg‐264 to His (R264H) in one allele of MATA1 gene. The other allele was normal in all the patients examined. Gene tracking in the family revealed that the hypermethioninemia is associated with heterozygosity for the R264H mutation in the MATA1 gene.
Transient erythrophagocytosis in Diamond‐Blackfan anemia
Authors: SEKINE ISAO , MASUDA TOMOO , KAWAMURA YOUICHI , DOBASHI HIROSHI , KOGAWA KAZUHIRO , YOSHIOKA SHIGETAKE 
Published: January 2011

AbstractWe report on a 4‐month‐old Japanese infant girl with Diamond‐Blackfan anemia (DBA) as shown by congenital macrocytic pure red cell hypoplasia with marked reduction of erythroid precursors in bone marrow, reticulocytopenia, increased fetal hemoglobin, and elevated adenosine deaminase activity in peripheral blood. She responded poorly to conventional doses of corticosteroids, however, with high‐dose corticosteroids she responded with reticulocytosis and an elevation of hemoglobin level above 12 g/dL. Erythrophagocytosis was noted during the tapering period of prednisone when her hemoglobin level declined to 7.6 g/dL and reticulocyte level to 0.4%. At that time, the erythrophagocytosis was noted in about 60% of marrow histiocytes. These findings were not observed prior to or during the high dose prednisone therapy. We speculate that one of the causes of pure red cell aplasia and reticulocytopenia in DBA is mediated by erythrophagocytosis.
A case of X‐linked α‐thalassemia/mental retardation syndrome: Analysis of hemoglobin by an automated glycated hemoglobin analyzer
Authors: KUNO TATEO , IDEGUCHI HIROSHI , YOSHIDA NOBUYUKI , MASUYAMA TATSUO , OHTA MITSUHIRO , NISHIMURA SHINJI , TASAKI HAKARU , MIYAZAKI SUMIO , HARA HIROMICHI , MATSUMOTO KAZUHIRO 
Published: January 2011

AbstractA 5‐year‐old male patient with X‐linked α‐thalassemia/mental retardation syndrome is reported. He showed multiple minor anomalies including characteristic facial abnormalities, α‐thalassemia, severe mental retardation, and hypogonadism. Analysis of his hemoglobin by high performance liquid chromatography using an automated glycated hemoglobin analyzer revealed an abnormal peak. Identification of an abnormal peak by an automated glycated hemoglobin analyzer will aid in the diagnosis of patients with X‐linked α‐thalassemia/mental retardation syndrome.
Imipenem for treatment of relapsing Salmonella meningitis in a newborn infant
Authors: KOĈ ESIN , TURKYILMAZ CANAN , ATALAY YILDIZ , SEN EMEL 
Published: January 2011

AbstractSalmonella meningitis is a rare clinical entity that occurs mainly during early infancy. Treatment of Salmonella infections may be complicated by the bacteria's growing resistance to clinically important antimicrobial agents, especially third‐generation cephalosporins. A report is presented of a newborn infant with Salmonella meningitis who relapsed after 4 weeks of cefotaxime treatment and was cured completely with imipenem cilastatin therapy.
Familial atrial septal defect with prolonged atrioventricular conduction
Authors: GüNAL NAZLIHAN , GüL SEHER , KAHRAMANYOL ÖZ 
Published: January 2011

AbstractA family is described in which a mother, her two children and another two relatives all had atrial septal defect of the ostium secundum type. The index case and her mother also had prolonged atrioventricular conduction. It is considered that the defect is the familial type and inherited as a mendelian dominant trait.
Linkage analysis and identification of deletion in Alagille syndrome gene
Authors: YUAN ZENG‐RONG , KOHSAKA TAKAO , KOBAYASHI NOBORU 
Published: January 2011

AbstractAlagille syndrome (AGS) is a genetic disease and the responsible gene has already been mapped at 20p12. To more accurately detect the region of the AGS gene on the linkage map of chromosome 20p, 14 yeast artificial chromosome (YAC) clones were screened to construct a YAC contig in the candidate region and 13 locus markers and 2 sequence‐tagged sites (STS) were ordered. Combining all of the analyses, a 1.3 Mb critical region from D20S507 to D20S61 for the AGS gene was identified. As the human Jagged 1 gene (JAGI) lies just in this region and is responsible for the AGS disease, the genomic DNA in an AGS family without a visible deletion were analyzed by single‐strand conformational polymorphism (SSCP) and direct DNA sequencing, and a 2‐bp (CT) deletion mutation at exon 26 of the JAGI was identified.
Role of provocation poliomyelitis in vaccine‐associated poliomyelitis
Authors: EHRENGUT WOLFGANG 
Published: January 2011

AbstractThe impact of different vaccine administrations in the Federal Republic of Germany, in relation to vaccine‐associated paralytic poliomyelitis (VAPP) was studied. (This only means that on the basis of the temporal relation of vaccination and onset, the vaccine cannot be ruled out as possible cause.) Oral polio vaccine (OPV) was given between 1963 and 1977 only and recommended simultaneously with inactivated vaccines (DT/DPT) between 1980 and 1985. In the first period 10 VAPP cases occurred among 9.96 million estimated vaccinees aged under 2 years. In the second period 6 cases of provocation poliomyelitis (PRP) were found among 2.9 million estimated OPV/DT/DPT vaccinees of the same age group. Statistically the VAPP/PRP frequencies in the two periods studied did not achieve significance.
High incidence of breakthrough varicella observed in healthy Japanese children immunized with live attenuated varicella vaccine (Oka strain)
Authors: TAKAYAMA NAOHIDE , MINAMITANI MIKIO , TAKAYAMA MICHIKO 
Published: January 2011

AbstractIn order to know the rate of occurrence of varicella among vaccinees (breakthrough varicella: BV), questionnaire postcards were sent to 593 healthy children who had received varicella vaccine (Oka strain) from March 1987 to December 1989. The questionnaire survey was repeated once a year until January 1996. The annual attack rate from the 1st to 3rd questionnaire was approximately 12%; however, from the 5th to 8th one it was 1–4%. To February 1996, the cumulative attack rate was 157/459 (34.2%). This rate was comparable to that among vaccinees who had confirmed seroconversion; namely, 51/132 (38.6%). These rates are much higher than those reported by other authors. All BV cases were clinically mild; even subjects who had received the vaccine 7 years prior to the disease showed mild symptoms. The high incidence may be partly explained by the regional epidemiology of varicella. The decrease in annual incidence with time after vaccination may be due to the following reasons: some vaccinees remained free from BV owing to reinforcement of their immunity from subclinical infection of varicella‐zoster virus (VZV) and others from diminution of opportunity for exposure to VZV with increasing age. Varicella vaccine seems to be effective in modifying the symptoms of varicella, but not potent enough in protecting from VZV infection.
Early cardiac failure in a child with Becker muscular dystrophy is due to an abnormally low amount of dystrophin transcript lacking exon 13
Authors: ISHIGAKI CHIEKO , PATRIA SURYONO YUDHA , NISHIO HISAHIDE , YOSHIOKA AKIRA , MATSUO MASAFUMI 
Published: January 2011

AbstractTwo Japanese brothers with Becker muscular dystrophy were shown by polymerase chain reaction (PCR) and cDNA sequence analysis to produce a dystrophin gene transcript lacking a single exon; that is, number 13. Despite having the same deletion mutation, the brothers showed clearly different clinical phenotypes: the younger brother developed cardiac failure at the age of nine, while the elder brother was asymptomatic. As alternative splicing was not responsible for this clinical difference, the amount of dystrophin transcript was examined by using reverse transcription semi‐nested and parallel PCR. The results showed that the amount of the dystrophin transcript in the younger brother was 20% of that of the elder brother. This finding suggested that lesser amount of dystrophin transcript in the younger brother was responsible for the early onset of cardiac failure. This would represent a novel molecular mechanism for dystrophinopathy.
Early detection of prune belly syndrome in utero by ultrasonography
Authors: KOBATA RITSUYO , TSUKAHARA HIROKAZU , TAKEUCHI MOTOHIRO , HORI CHIKAHIDE , HIRAOKA MASAHIRO , UCHIDA TOSHIYUKI , KOTSUJI FUMIKAZU , ITO YASUHIKO , OKADA KENICHIRO , SUDO MASAKATSU 
Published: January 2011

AbstractA report is presented of a male infant with prune belly syndrome (PBS) in whom bladder enlargement was detected by ultrasound (US) as early as 13 weeks of gestation. Subsequent fetal US identified progressive urinary tract dilatation, ascites and oligohydramnios. At 22 weeks, the fetal bladder was drained under US guidance. A gradual resolution of oligohydramnios was detected on US performed after 26 weeks of gestation. Delivery by cesarean section was performed at 34 weeks of gestation. The newborn had typical features of PBS with a vesico‐cutaneous fistula but did not show respiratory distress. Imaging studies showed hypoplastic left kidney, slightly dysplastic but functioning right kidney, megaureter, megacystis, vesicoureteral reflux and dilated prostatic urethra. The early detection of genitourinary system abnormalities and serial US suggest that a distal urethral obstruction may be the underlying mechanism of PBS. Spontaneous (or therapeutic) intra‐uterine decompression of the bladder might ameliorate obstructive nephropathy and oligohydramnios, allowing adequate lung development.
Trends in neonatal exchange transfusions at Yodogawa Christian Hospital
Authors: FUNATO MASAHISA , TAMAI HIROSHI , SHIMADA SEIICHI 
Published: January 2011

AbstractA review was conducted to determine the trends in exchange transfusion (ET) of newborn infants at the Yodogawa Christian Hospital during the past 18 years. At that hospital in 1957, the first ET was performed on a term infant with severe hemolytic jaundice caused by rhesus factor (Rh) incompatibility. By 1989, ET had been performed in more than 1400 newborn infants. These cases of newborns who had had ET were retrospectively reviewed, with a focus on every 3 year period from 1974 to 1992. The total number of infants requiring ET noticeably decreased from 68 cases (14.0% of total admissions) in 1974 to 19 cases (6.1% of total admissions) in 1992. (X2, P < 0.001) There were three major significant changes in ET during those years. The first was a change in the subjects for ET. The incidence of ET for term infants showed a marked decrease, while the incidence of ET for preterm infants, especially for very low birthweight (VLBW) infants (< 1500 g), noticeably increased. The second was a change in indications for ET. There was a marked decrease in the need for ET as a result of hyperbilirubinemia, while the incidence of ET because of other etiologies, such as septicemia and/or disseminated intravascular coagulopathy, noticeably increased. The third was a change in the technical methods of ET. Now at the Yodogawa Christian Hospital, 100% of the infants are given ET with an automated peripheral two‐site method, instead of the Diamond method. Although ET might still be a useful treatment for severe hyperbilirubinemia and other acute problems, the total number of ET noticeably decreased in accord with a decrease in the number of severe hyperbilirubinemia in term newborns. On the other hand, the incidence of ET in preterm infants increased relatively, accompanied by an increase in the survival of VLBW infants. The automated two‐site method is the preferred technique for ET at the Yodogawa Christian Hospital, rather than the Diamond method. Further changes in ET might occur in accord with new alternative measures in future.
Prevalence of asthma, rhinitis and eczema among schoolchildren in Kelantan, Malaysia
Authors: QUAH BAN SENG , RAZIF ABDUL , RAZAK ABDUL , HASHIM MOHAMAD , HASSAN MOHD 
Published: January 2011

AbstractThe prevalence and severity of asthma, rhinitis and eczema in Kelantanese schoolchildren were determined as part of an international study of the epidemiology of asthma and allergic diseases. The international study of asthma and allergies in childhood (ISAAC) written questionnaire was administered to 7055 schoolchildren from February 1995 to August 1995. The respondents were parents or guardians of 5‐ to 7‐year‐old children (n = 3939), and schoolchildren aged 12–14 years (n = 3116). The ISAAC video questionnaire (AVQ3.0) was shown to children aged 12–14 years after the written questionnaire. The overall prevalences of ‘ever wheezed’ and ‘wheezing in last 12 months' were 9.4 and 6.0% respectively. The prevalence of ‘ever diagnosed with asthma’ was 9.4%. Both ‘ever wheezed’ and ‘wheezing in the last 12 months’ were significantly higher in 12‐ to 14‐year‐old children than in 5‐ to 7‐year‐old children, with P values of 0.0006 and 0.014 respectively. No gender differences in the prevalences were observed. For the complete study group, 4.7% of children had sleep disturbed by wheezing but only 1.1% had a severe attack limiting speech in the preceding 12 months. Sleep disturbance was more common in the 12‐ to 14‐year‐old children than in 5‐ to 7‐year‐old children (P = 0.006). There was no difference between the age groups for severe attacks limiting speech. The overall prevalence of rhinitis and eczema symptoms were 27 and 12%, respectively. The prevalence of rhinitis in the 12–14 year age group (38.2%) was significantly higher (P < 0.0001) than in the 5–7 year age group (18.2%). The prevalence of eczema in the 5–7 year age group (13.7%) was significantly higher (P = < 0.0001) than in the 12–14 year age group (9.9%). These prevalence data are comparable with previous reports in Malaysian children, but are considerably lower than those reported for most developed countries
Serum zinc, copper, and magnesium levels in obese children
Authors: YAKINCI GENGİZ , PAÇ AYŞENUR , KüÇüKBAY F ZEHRA , TAYFUN MELTEM , GüL ABDüLKADİR 
Published: January 2011

AbstractSerum zinc, copper and magnesium levels were measured using atomic absorption spectrophotometry in 41 obese and 41 healthy (control) children (between 7 and 11 years of age) who were living in Malatya (Turkey). Serum zinc levels of obese children (mean value 102.40 ± 2.78 μg/dL) were found to be significantly higher than those of the control group (mean value 80.49 ± 2.98 μg/dL; P < 0.01). Serum copper concentrations were also found to be significantly higher in obese children (mean value 132.34 ± 1.79 μg/dL) than the healthy controls (mean value 107.58 ± 1.62 μg/dL; P < 0.01). On the other hand, serum magnesium levels were found to be significantly lower in obese children (mean value 1.78 ± 0.03 mg/dL) than in the healthy children (mean value 2.14 ± 0.04 mg/dL; P < 0.01).