bluuebunny/crossref_metadata_2025_split huggingface dataset:
Urinary pregnanetriol‐3‐glucuronide in children: Age‐related change and application to the management of 21‐hydroxylase deficiency
Authors: YANG YANLING , SAISHO SUMITAKA , TOYOURA TAKIO , SHIMOZAWA KAZUHIKO , YATA JUNICHI 
Published: January 2011

AbstractUrinary concentrations of pregnanetriol‐3‐glucuronide (PT‐3‐G) were determined in 485 normal Japanese subjects (277 males and 208 females), aged 5 days to 20 years, using an enzyme‐linked immunosorbent assay (ELISA). The usefulness of urinary PT‐3‐G concentrations before giving the morning dose of medications in monitoring the adequacy of glucocorticoid treatment was assessed in eight patients with 21‐hydroxylase deficiency (21‐OHD). The ratio of PT‐3‐G to excreted creatinine (PT‐3‐G/Cre ratio) increased significantly during the first month and did not change from age 1 month to 1 year of life. The ratio decreased to a nadir at age 3 or 4 years followed by continuous, significant increase until late adolescence. In the subjects treated with corticosteroids for 21‐OHD, PT‐3‐G/Cre ratios at the 50th percentile or below suggested a risk of excessive treatment, as judged by the patients' growth. Measurement of the PT‐3‐G/Cre ratio enabled recognition of corticosteroid overtreatment, which was not demonstrated by determining the serum concentrations of 17α‐hydroxyprogesterone (17‐OHP) On the other hand, ratios at the upper 95–99% tolerance limits seemed to be required for optimal control. The present study revealed the normal age‐related changes in urinary excretion of PT‐3‐G and showed it to be a reliable marker for evaluating glucocorticoid treatment in young children with 21‐OHD.
Serum Cortisol and prolactin levels in childhood paroxysmal disorders
Authors: DİRİK ERAY , ŞEN ALP , ANAL ÖZDEN , ÇEVIK NECLA T 
Published: January 2011

AbstractPostictal serum prolactin and Cortisol levels were measured in 37 children having either epilepsy, febrile seizure or syncopal attack and in 37 normal controls. Blood samples were obtained within 1.5 h following the seizure episode. All serum levels were compared between each group and the control groups. Significantly higher (P < 0.005) prolactin levels (56.64 ± 34.78 ng/mL) were found in the epileptic group, compared to the levels in children with febrile seizures (21.72 ± 12.92 ng/mL), syncope attacks (13.88 ± 5.27 ng/mL) and the control group (14.32 ± 5.05 ng/mL). In contrast, serum Cortisol levels were non‐specifically elevated in children with epilepsy, febrile seizures and syncopal attacks. Cortisol secretion appears to be non‐specifically elevated in all stressful events. Elevated prolactin levels may be helpful in differentiating epilepsy from febrile seizures and syncope.
IgG subclasses in children with recurrent respiratory tract infections in an allergy practice
Authors: ŞEKEREL BÜLENT ENİS , SARAÇLAR YILDIZ , SANAL ÖZDEN , ERSOY FÜGEN , ADALIOǦLU GÖNÜL , TUNCER AYFER 
Published: January 2011

AbstractIsolated or combined deficiencies of immunoglobulin G (IgG) subclasses have been recognized in children with recurrent infections. In our allergy practice, there are a subset of children with recurrent respiratory tract infections. To investigate the presence of immunoglobulin G subclass deficiency (IgGSD), 60 children with atopy and 14 children without atopy suffering from recurrent respiratory tract infections were studied in an attempt to determine whether atopy is associated with a certain IgG subclass pattern. Ten atopic children were found to have isolated or combined IgG subclass deficiencies: one with IgG1, two with IgG2, four with IgG3 and three children had IgG2–IgG3. Neither IgG subclass concentration nor the frequency of children with high or low IgG subclasses showed any difference between atopic and non‐atopic groups. Except for a week correlation with IgG3, no correlation existed between IgE and other IgG subclasses. It was concluded that childhood respiratory diseases complicated by recurrent respiratory tract infections may be associated with IgG subclass deficiencies. Although there have been reports noting some IgG subclass patterns in atopic disorders, in the present study, no distinctive feature between atopics and non‐atopics with respect to IgG subclass concentrations and patterns was observed.
Hepatitis B virus‐associated nephropathy: 17 year progression from onset to end‐stage renal failure
Authors: TOMONAGA KAORI , IITAKA KIKUO , NAKAMURA SHINYA , MORIYA SHUNSUKE , HOJO MIDORI , SAKAI TADASU 
Published: January 2011

AbstractHepatitis B virus‐associated nephropathy (HB nephropathy) was first described in 1971. There have been few reports on the long‐term prognosis in children with HB nephropathy. A case is reported here of a child who presented with symptoms of acute glomerulonephritis at 12 years of age and progressed to end‐stage renal failure 17 years after the clinical onset, in spite of the seroconversion of HB virus by formation of HBe antibody.
Gigantic aneurysm in the thoraco‐abdominal aorta of an infant
Authors: TSUJI ATSUTOSHI , YANAI JUN , OHTA MASANORI , SUGIYAMA HISASHI , UENO AKIRA , HASHIMOTO RYOICHI , YOSHIDA YOJI 
Published: January 2011

AbstractWe report the case of a boy with a gigantic aneurysm in the thoraco‐abdominal region which was detected by a chest X‐ray taken prior to surgical correction of ptosis of the eyelids at 11 months of age. At 18 months, he successfully underwent aneurysm exclusion and bypass grafting. A biopsy from the thoracic aorta revealed medial degeneration with conspicuous smooth muscle cell involvement. Laboratory examination showed altered elastase activity in the granulocytes and whole blood. The present case may represent a unique form of aneurysm in infancy.
A case of dysembryoplastic neuroepithelial tumor of the parietal lobe with characteristic magnetic resonance imaging
Authors: KIMURA SEIJI , KOBAYASHI TAKUYA , HARA MASAMICHI 
Published: January 2011

AbstractThe case of a 4 year old boy with a dysembryoplastic neuroepithelial tumor (DNT) of the left parietal lobe is reported. The DNT was located mainly in the cortex and showed no mass effect on magnetic resonance imaging (MRI). T1‐weighted images of the DNT showed the characteristic findings of lesion hypointensity, but with a well preserved gyrus‐like configuration. This lesion was isointense to the normal cortex on proton density imaging and hyperintense on T2‐weighted imaging. The characteristic features on T1‐weighted and proton density imaging in this patient were useful in differentiating DNT from other types of tumors. Histologic findings in DNTs, which include the presence of both glial and neuronal cells without atypia and no definite transitional zone between the adjacent disorganized cortical cell layers, suggest that DNT is not a true neoplasm but rather a dysplastic lesion. It is clinically important to differentiate this tumor both from other benign tumors and malignant tumors which have different prognoses and therapies. The distinctive MRI findings, as well as the histologic features of DNT, support the diagnosis in the clinical setting.
Focal nodular hyperplasia in an adolescent with glycogen storage disease type I with mesocaval shunt operation in childhood: a case report and review of the literature
Authors: SAKATOKU HIROYUKI , HIROKAWA YOSHIHUMI , INOUE MASAKAZU , KOJIMA MASAMITSU , YABANA TADASHI , SAKURAI MINORU 
Published: January 2011

AbstractAs patients with glycogen storage disease type I survive longer, cases with hepatic tumor have been increasingly documented. A 16 year old boy with glycogen storage disease type I was evaluated for multiple liver tumors. He was diagnosed on clinical features at 9 months of age and underwent a mesocaval shunt operation at 5 years of age. The biopsy of one of the masses showed focal nodular hyperplasia. This is uncommon in patients with glycogen storage disease type I, compared to those with adenoma or malignant hepatic tumor. The association of a portacaval shunt with focal nodular hyperplasia is significant compared to other tumors. An environment of high estrogen concentration or sex hormone binding globulin accompanied by shunt operation may cause focal nodular hyperplasia to develop in the liver of patients with glycogen storage disease type I.
Adenovirus vector technology: An efficient method for constructing recombinant adenovirus and on/off switching of gene expression
Authors: KANEGAE YUMI , MIYAKE SANAE , SATO YUMI , LEE GWANG , SAITO IZUMU 
Published: January 2011

AbstractAn efficient method of constructing recombinant adenoviruses (Ad) has been established. The expression unit to be introduced into recombinant Ad was first inserted into the unique SwaI site of the full‐length Ad genome cloned in a cassette cosmid. The cassette bearing the expression unit was then cotransfected to 293 cells together with the Ad DNA‐terminal protein complex digested at several sites with EcoT22I. The use of the parent Ad DNA‐terminal protein complex instead of the deproteinized Ad genome DNA allowed very efficient recovery of the desired recombinant Ad, and the restriction digestion drastically reduced regeneration of the parent virus. This method may facilitate the application of recombinant Ad and should be useful for further improvement of Ad vectors. Also a recombinant adenovirus expressing Cre recombinase derived from bacteriophage P1 was constructed. To assay the Cre activity in mammalian cells, another recombinant Ad bearing an on/off switching reporter unit, where a LacZ‐expression unit can be activated by the Cre‐mediated excisional deletion of an interposed stuffer DNA, was also constructed. Co‐infection experiments together with the Cre‐expressing and the reporter recombinant Ad showed that the Cre‐mediated switching of gene expression was detected in nearly 100% of cultured CV1 cells. These results suggest that the recombinant Ad efficiently expressed functional Cre and offers a basis for establishing a powerful on/off switching strategy of gene expression in cultured mammalian cells and presumably in future gene therapy.
Adverse events associated with MMR vaccines in Japan
Authors: KIMURA MIKIO , KUNO‐SAKAI HARUMI , YAMAZAKI SHUDO , YAMADA AKIO , HISHIYAMA MICHIKO , KAMIYA HITOSHI , UEDA KOJI , MURASE TOSHIRO , HIRAYAMA MUNEHIRO , OYA AKIRA , NOZAKI SADAHIKO , MURATA RYOSUKE 
Published: January 2011

AbstractThe largest nationwide active surveillance of four Measles‐Mumps‐Rubella (MMR) vaccines was conducted in Japan. A total of 1255 pediatricians actively participated in the study, which comprised 8.6% of all members of the Japanese Pediatric Society. The total number of registered recipients of MMR vaccines was 38 203. They were arbitrarily given one of the MMR vaccines produced by three makers (Takeda, Osaka city, Kitasato Minato‐ku, Tokyo and Biken Suita city, Japan) or the standard MMR vaccine made of designated strains (Kitasato's measles‐AIK‐C, Biken's mumps‐Urabe Am9 and Takeda's rubella‐To336) produced by Takeda, Kitasato and Biken and were observed for 35 days.The rates of virologically confirmed aseptic meningitis per 10 000 recipients were 16.6, 11.6, 3.2 and 0 for the standard MMR, Takeda MMR, Kitasato MMR and Biken MMR vaccines, respectively. The incidence of convulsions between 15 and 35 days was the highest with the standard MMR vaccine and the incidence of fever associated with vomiting occurring between 15 and 35 days (symptoms relevant to aseptic meningitis) were also the highest with the standard MMR vaccine. The incidence of parotid swelling was the lowest with Takeda MMR vaccine.This surveillance revealed that incidences of aseptic meningitis after administration of the standard MMR vaccine and of Biken MMR vaccine were different. This posed questions about the manufacturing consistency of the Urabe Am9 mumps virus vaccines. On the other hand, the National Institute of Health found that the biological characteristics of the Urabe Am9 mumps virus contained in the standard MMR vaccine and in the Biken MMR vaccine were different. The Biken Company reported that the mumps vaccine in the standard MMR vaccine was a mixture of two Urabe Am9 mumps vaccine bulks; one identical to that contained in the Biken MMR vaccine and the other produced by a different manufacturing process.
Ultrasonography for the detection of ureteric reflux in infants with urinary infection
Authors: HIRAOKA MASAHIRO , HASHIMOTO GOTARO , HAYASHI SHUHEI , HORI CHIKAHIDE , TSUCHIDA SHINYA , TSUKAHARA HIROKAZU , KONISHI YUKUO , SUDO MASAKATSU 
Published: January 2011

AbstractSeveral less harmful methods than voiding cysto‐urethrography for detecting significant ureteric reflux have been proposed. The present prospective study investigated whether ultrasonography was effective in identifying ureteric reflux in infants with their first febrile urinary infection. The subjects were 27 infants (24 boys and 3 girls) aged from 0 to 8 months. The urinary tract was scanned when the bladder was full, and before and during induced voiding. Infants with abnormal ultrasound findings underwent voiding cysto‐urethrography. The other infants were followed and those who had a recurrence of urinary infection underwent voiding cystography.Ten children underwent cysto‐urethrography, with eight refluxing ureters identified in six boys. Ultrasound revealed transient dilatation of the renal pelvis on voiding in five kidneys, transient dilatation of distal ureters in 12 and hydro‐ureteronephrosis in two. Each of the five kidneys with pelvic dilatation on voiding was associated with ureteric reflux grades III or IV. Of the 17 children who did not undergo cysto‐urethrography, only one had recurrence of urinary infection and was diagnosed with ureteric reflux. This girl was one of the three babies who were not scanned during voiding. More than half of the infants with febrile urinary infection were excluded from invasive examination without having recurrence of urinary infection. Thus, ultrasound scanning during voiding was effective for screening infants with their first urinary infection to detect significant ureteric reflux.
Growth hormone deficiency in Dubowitz syndrome
Authors: HIRANO TAKEKI , IZUMI ISHOU , TAMURA KAZUYOSHI 
Published: January 2011

AbstractSevere short stature as a result of intra‐uterine growth retardation is one of the characteristics of Dubowitz syndrome. There have been few reports elaborating growth hormone secretory status in this syndrome. A child with Dubowitz syndrome, who was found to have complete growth hormone (GH) deficiency and who responded to growth hormone therapy, is described. This appears to be the first documentation of GH deficiency in this syndrome.
Acute anterior uveitis in a child with HLA‐B60 after Salmonella enteritis associated with the transient appearance of auto‐antibody
Authors: AIHARA YUKOH , SHIMIZU CHISATO , FUJIWARA YOSHITO , YOKOTA SHUMPEI 
Published: January 2011

AbstractA Japanese girl with HLA‐B60, but not B27, who developed acute anterior uveitis after Salmonella enteritis is described. There was no evidence of arthritis or urethritis during this episode. This is the first report that acute anterior uveitis after Salmonella enteritis was associated with the transient appearance of auto‐antibody in the serum at an early period.
Analysis of Met235 to Thr variant of the angiotensinogen gene in relation to the blood pressure and family history of essential hypertension in Japanese children
Authors: KATAOKA SATOSHI , HASHIMOTO NAOSHI , KAKIHARA TOSHIO , TANAKA ATSUSHI , KAWASAKI TAKUYA , KIKUCHI TORU , TAKAHASHI HIDEO , UCHIYAMA MAKOTO 
Published: January 2011

AbstractA recent study reported a significant relationship between a T704→C (Met235→Thr) variant in exon 2 of the angiotensinogen gene in adults and essential hypertension. In the present study, this variant was detected in 131 Japanese children using a polymerase chain reaction. The allele frequency of the variant was 0.76. The genotype frequency of the homozygote for the allele was 0.59, and children who were homozygous had higher systolic blood pressure than those with the other two genotypes. No relationship was found between children's polymorphism and a family history of essential hypertension. These findings suggest that this molecular variant of the angiotensinogen gene may play some role in the regulation of blood pressure in Japanese children.
Periorbital cellulitis: A comparison of different treatment regimens
Authors: KANRA GÜLER , SEÇMEER GÜLTEN , GÖNÇ E. NAZLI , CEYHAN MEHMET , ECEVIT ZAFER 
Published: January 2011

AbstractPeriorbital cellulitis was retrospectively investigated in a cohort of 69 children, 1.5–16 years of age, who were admitted to Hacettepe University Children's Hospital. The aim of the study was to define the most important cause of the disease and to choose the most appropriate antimicrobial regimen.Sinusitis (43%) was found to be the most frequent disease associated with periorbital cellulitis. Trauma (25%) and odontogenic infections (6%) were the next most common predisposing causes. Staphylococcus aureus was isolated from 14 (74%) of 19 cultures. Two antibiotic combinations, penicillin plus chloramphenicol and sulbactam‐ampicillin (SAM) with or without ornidazole was used in 30 (43%) and 39 (57%) of 69 cases, respectively. The duration of treatment with these two antibiotic combinations was generally between 7 and 10 days. No statistical difference was found between the two antibiotic combinations in the cure and recurrence rates but five (17%) of the 30 cases using penicillin plus chloramphenicol, and one (3%) of the 39 cases using SAM with or without ornidazole had recurrent periorbital cellulitis. It was concluded that SAM can be the first line of drug treatment for periorbital cellulitis as it is easily used in every age group and etiology (trauma, sinusitis, etc.). It provides total recovery and has less side effects and risk of recurrence. It also has a broad antimicrobial spectrum.
Adrenoleukodystrophy indicated by abnormal electroencephalography
Authors: WADA NORIKO , TAKEUCHI YOSHIHIRO , FUJIWARA FUMIHIRO , ODANI ISAO , SAWADA TADASHI 
Published: January 2011

AbstractAn 8 year old boy with adrenoleukodystrophy and his asymptomatic brother, both with abnormal electro‐encephalography as the initial sign of adrenoleukodystrophy, are reported. After suffering from aseptic meningitis caused by Echo virus 30, the first case was suspected of having a complication of meningitis because of persistent abnormal electro‐encephalography (i.e. slow wave bursts in the occipital region). This patient was finally diagnosed as having adrenoleukodystrophy because of the increased level of very long chain saturated fatty acids, and typical occipital lesions demonstrated by magnetic resonance imaging and computed tomography. Dietary therapy was tried, but the course was progressive. The brother's electroencephalography was also abnormal and so was diagnosed as having asymptomatic adrenoleukodystrophy because of an increased level of very long chain saturated fatty acids, although brain magnetic resonance imaging was normal. Abnormal electro‐encephalography can therefore be an initial sign of adrenoleukodystrophy even if magnetic resonance imaging shows no abnormality in the brain.
Cardiac rhabdomyoma of a neonate: An autopsy case report
Authors: SADI AL MUKTAFI , TODA TAKAYOSHI , KIYUNA MASAYA , TAMAMOTO TOORU , KINA KYOKO , HIRAYASU KYOMI , YARA ASAO 
Published: January 2011

AbstractA primigravida delivered a cyanosed female infant with a very low Apgar score. Cardiac anomaly of the fetus was detected at 32 weeks of gestation by ultrasonography. The baby died on the day of delivery. Autopsy revealed multiple tumor masses in the interventricular septum and ventricular walls. The tumor originating from the interventricular septum was the largest and measured 3.7 × 3 cm. Histologically, the tumor was composed of large polygonal glycogen‐laden cells and ‘spider‐cells’. Eosinophilic giant histiocytic cells were also observed in the spleen. Ultrastructural features of the tumor cells correlated with those of typical cardiac muscle cells.
Diagnosis of renovascular hypertension by echo‐Doppler velocimetry
Authors: FUSE SHIGETO , HORI TSUKASA , YOSHIDA MASAKI , IGARASHI CHIHARU , FUJITA SHIGERU 
Published: January 2011

AbstractA 13 year old boy suffering hypertension was examined for peripheral plasma renin, angiotensin‐I, angiotensin‐II and aldosterone. All data were within the normal range. The Captopril test and renal scintigraphy (both with and without Captopril) also showed normal patterns. Echo‐Doppler velocimetry of the renal artery revealed that left renal arterial peak flow velocity was fast (3.4 m/s). The patient was therefore diagnosed with left renal arterial stenosis. Angiography demonstrated the duplex of the left renal artery and stenosis of the left lower renal artery. Percutaneous transluminal angioplasty was successfully performed.
A case of ureteral reflux identified by ultrasound
Authors: HIRAOKA MASAHIRO , FUJIMOTO IWAO , HORI CHIKAHIDE , TSUKAHARA HIROKAZU , AKINO HIRONOBU , OKADA KENICHIRO , SUDO MASAKATSU 
Published: January 2011

AbstractReflux nephropathy is known to be a major cause of renal failure in children. Vesico‐ureteral reflux is usually diagnosed by voiding cysto‐urethrography (VCG). However, it has been observed that conventional VCG is not always reliable for the diagnosis of ureteral reflux. In the case of a 5 year old girl with recurrent febrile urinary tract infection, VCG showed no ureteral reflux. Urodynamic study revealed a large bladder capacity and significant residual urine. Renal scintigram delineated a right renal scar. Simple ultrasound examination with videotape recording during voiding definitely demonstrated the presence of significant ureteral reflux when she voided, that is, there was marked dilatation of the right distal ureter and ballooning of the right renal pelvis on voiding, and quick refilling of the bladder concomitantly with the disappearance of the pelvic ballooning. Therefore, an ultrasound during voiding may be useful for diagnosing ureteral reflux in patients where a VCG does not reveal reflux.
The azoospermic factor on the Y chromosome
Authors: SHINKA TOSHIKATSU , NAKAHORI YUTAKA 
Published: January 2011

AbstractAzoospermia is the most frequent cause of male infertility. After excluding the obvious urological reasons and the effect of Klinefelter's syndrome, azoospermia may be caused by an abnormality in the crucial gene(s) expressed during male germ cell differentiation. Recently, two candidate genes for azoospermia have been cloned from the azoospermic factor (AZF) locus on the Y chromosome long arm (Yq). One is YRRM (Y chromosome RNA recognition motif) gene, and the other is DAZ (deletion in azoospermia) gene. Both genes encode RNA binding protein and their expression is restricted to the testis. Therefore they are good candidates for AZF, although their function remains unclear. Here, the genes on the Y chromosome possibly involved in spermatogenesis and the role of the Y chromosome in evolution are discussed.