bluuebunny/crossref_metadata_2025 huggingface dataset:
Home mechanical ventilation in the aftermath of the Hanshin‐Awaji earthquake disaster
Authors: SHIMADA SEIICHI , FUNATO MASAHISA
Published: January 2011

Children who were dependent upon home mechanical ventilation (HMV), suffered in various ways from the disastrous Hanshin‐Awaji earthquake disaster. The earthquake abruptly cut the supplies of water, gas and electricity, causing intense anxiety for those families. Through loss of the respirator function, some of them experienced an unexpected catastrophe.

In the disaster area, there were children who were dependent upon HMV (19 cases) and children who were preparing for HMV in hospitals (nine cases). Information was gathered from questionnaires about the disaster, communication and correspondence with families.

None of the 28 cases died or were injured. Nineteen cases had a variety of problems. In eight cases, respiratory support problems were acute. Nevertheless, all of them survived the crisis successfully even in the midst of such a catastrophic situation. An organization of HMV children's families, called the Baku‐Baku Club, helped families with HMV problems by supplying water, food, oxygen and compressed air cylinders among other things.

Additional outside batteries for portable respirators are essential equipment for HMV, especially for emergencies. A manual for clarifying the system for support in the Baku‐Baku Club and a registration system for public medical service should be established in preparation for such a crisis.
Childhood obesity: Pathophysiology and treatment
Authors: KLISH WILLIAM J.
Published: January 2011

Childhood obesity is among the most difficult problems which pediatricians treat. It is frequently ignored by the pediatrician or viewed as a form of social deviancy, and blame for treatment failure placed on the patients or their families. The definition of obesity is difficult. Using total body electrical conductivity (TOBEC) technology, total body fat ranges between 12% and 30% of total body weight in normal children and adolescents. This is influenced not only by age, but also by physical fitness. Anthropometry is the easiest way to define obesity. Children whose weight exceeds 120% of that expected for their height are considered overweight. Skinfold thickness and body mass index are indices of obesity that are more difficult to apply to the child.

Childhood obesity is associated with obese parents, a higher socioeconomic status, increased parental education, small family size and a sedentary lifestyle. Genetics also clearly plays a role. Studies have demonstrated that obese and non‐obese individuals have similar energy intakes implying that obesity results from very small imbalances of energy intake and expenditure. An excess intake of only 418 kJ per day can result in about 4.5 kg of excess weight gain per year. Small differences in basal metabolic rate or the thermic effects of food may also account for the difference in energy balance between the obese and non‐obese. In the Prader Willi Syndrome, there appears to be a link between appetite and body fatness. When placed on growth hormone, lean body mass increases, body fat decreases, sometimes to normal, and appetite becomes more normal.

Our weight control program is very intensive and combines behavior modification with dietary instruction and exercise. Patients are screened both physically and psychologically. Abnormalities found are treated before the child is allowed to enter the weight loss program. Depression is frequently encountered. If treatment success is defined as either weight reduction or maintenance of weight as linear growth increases, then 88% of the children who completed the program were successful. The dropout rate however was 65%.
Helicobacter pylori colonization in children with peptic ulcer disease III. Diagnostic value of the 13C‐urea breath test to detect gastric H. pylori colonization
Authors: YAMASHIRO YUICHIRO , OGUCHI SATOSHI , OTSUKA YOSHIKAZU , NAGATA SATORU , SHIOYA TAKEHIRO , SHIMIZU TOSHIAKI
Published: January 2011

The efficiency of the 13C‐Urea Breath Test (13C‐UBT) for the detection of Helicobacter pylori colonization in gastric mucosa was evaluated. The 13C‐UBT was performed in five pediatric and six adult subjects who had had upper gastrointestinal endoscopy within 2 weeks. H. pylori colonization was confirmed in two pediatric and three adult subjects with peptic ulcer combined with antral gastritis, by histological examination of antral biopsy specimens. When an individual with H. pylori colonization ingested a solution containing 13C‐urea, a significant amount of 13CO2 appeared in the respiratory CO2 within 10 min. The mean cumulative percentage dose of 13C recovered in the breath over 30 min in the cases with H. pylori colonization was significantly higher than that in those who were not colonized (4.91 vs 0.41, P <0.001). In addition, the effect of antibiotic on the eradication of H. pylori from gastric mucosa was monitored by 13C‐UBT in two cases. The values of cumulative percentage dose of 13C over 30 min fell to the same levels as those observed in H. pylori negative subjects after just 2 weeks treatment with amoxicillin; however, positive results were obtained again 1 month after the withdrawal of amoxicillin. In summary, 13C‐UBT is a simple, reliable, non‐invasive method in the diagnosis of gastric H. pylori colonization especially for pediatric patients.
Vertical transmission of HTLV‐I from a seronegative mother using the PA method
Authors: UMEMOTO MASAKAZU , TAKE HIROMICHI , KURAYA KAZUE
Published: January 2011

The child was born when his mother was 23 years old. Her serum was negative for human T cell lymphotropic virus type I (HTLV‐I) using the gelatin particle agglutination (PA) method when she was 26.5 years. Three years later, she became seropositive for HTLV‐I using both the PA method and ELISA. She had breast‐fed her child for 3 years. The child was positive for anti‐HTLV‐I antibody at the age of 4.5 years. Vertical transmission of HTLV‐I can occur in women with negative results from the PA method.
Successful α‐interferon therapy in a child with chronic refractory idiopathic thrombocytopenic purpura: A case report
Authors: ASAMI TADASHI , SAKURAI MAMORU
Published: January 2011

We treated a sixteen month old male with chronic refractory idiopathic thrombocytopenic purpura (ITP) in whom α‐interferon (IFN) therapy was effective. He developed ITP which did not respond to various treatments. Six months after admission, we began to treat him with IFN. The patient's platelet count rapidly responded to the therapy and rose above normal range. Serum levels of platelet associated immunoglobulin G (PA‐IgG) showed a tendency to decrease with the administration of IFN. After stopping the IFN therapy for a duration of 3 months, the platelet count remained normal. No serious adverse side effects, except transient fever, were observed. From the experience of this case we propose that IFN is one of the therapeutic options for treatment of refractory ITP not only in adults but also in children.
Continuous muscle fiber activity syndrome
Authors: ÇALIŞKAN MINE , TÜRKDOGAN DILŞAD , ÖZMEN MERAL , CANTEZ TALAT , BASLO AYNUR , APAK SELÇUK
Published: January 2011

A 5.5 year old Turkish boy who suffered from progressive muscle stiffness was diagnosed as having continuous muscle fiber activity syndrome. Electromyography showed continuous motor neuron activity at rest and following intravenous injection of diazepam. Peripheral nerve block, spinal and general anesthesia diminished the spontaneous activities. The clinical and electrophysiological findings of our case were compared to the previously reported cases with emphasis on the heterogeneity of the syndrome.
Age‐related occurrence of inhibitory antibodies to streptococcal pyrogenic superantigens
Authors: KUWAHATA MASATO , IMANAKA HIROYUKI , TAKEI SYUJI , MASUDA KIMINORI
Published: January 2011

Several bacteria, such as staphylococci and streptococci, can produce superantigens (SA) that induce the activation of T cells in humans. Although these organisms are the major causes of infection in children, the evidence that T cells are vigorously activated by SA produced by such organisms has not been reported except for toxic shock syndrome. In a previous paper, we demonstrated that inhibitory IgG antibodies (Ab) to SA in humans may protect against SA stimulation. In the present study, we investigated the occurrence of these inhibitory Ab to SA in 94 healthy children by the enzyme linked immunosorbent assay technique and the suppressive effect on T cell stimulation by SA.

The positivity of Ab to streptococcal pyrogenic exotoxin (SPE)‐A, SPE‐C and staphylococcal enterotoxin B (SEB) increased with age. The age at which more than 50% of children exhibited Ab to SA was 1 year for SEB, 6 years for SPE‐C and 11 years for SPE‐A. Sera from these children were inhibitory to T cell proliferation elicited by SA in proportion to the concentration of IgG Ab to each SA. Sera supplemented with IgG Ab to SA by gamma‐globulin therapy became inhibitory to T cell proliferation by SA. We conclude that, as children grow, they can develop Ab to SA that may play a role in protecting them against vigorous T cell activation by SA.
Evaluation of the therapy for streptococcal pharyngitis using Abbott Test Pack® Strep A
Authors: KAYABA HIROYUKI , TAMURA HIROMI , FUJIWARA YOSHIYUKI
Published: January 2011

A total of 953 children (511 boys and 442 girls) with streptococcal pharyngitis diagnosed with Abbott

Test Pack® Strep A (ATPSA) or throat cultures were analyzed. ATPSA specimens were repeatedly obtained until ATPSA turned negative during or after the treatment. The percentage of positive ATPSA specimens reached the lowest value (9.2%) on the fourth day of the course of the treatment, which indicates the acquisition from an infected individual is probably uncommon after the initial treatment. Bacteriological treatment failure (positive ATPSA after 14 days of treatment) occurred in 4.1% of the children. Out of 953 subjects studied, 216 (22.7%) had recurrent infections. More than 30% of the recurrent infections occurred within 2 months after initial infection. ATPSA is useful for establishing a rapid diagnosis and confirming the bacteriological success of the treatment.
Bacterial changes in neonatal intensive care unit
Authors: ENDO AKIHIKO , MASUNAGA KEN , MASAKI RYOUSUKE , SHIMADA MASAMI , MINATO MICHIYOSHI , TAKADA MASAAKI , TAKAHASHI SHIGERU , HARADA KENSUKE
Published: January 2011

Organisms routinely cultured from throat swabs and infectious agents of sepsis and/or meningitis were reviewed. During the last 12 years, Klebsiella pneumoniae and Escherichia coli have been replaced by Staphylococcus aureus and Pseudomonas aeruginosa as the predominant isolates from throat swabs after admission. These change in the etiologic pattern of infectious agents of sepsis and/or meningitis, i.e., K. pneumoniae, E. coli, S. aureus, P. aeruginosa and staphylococcus epidermidis, were in agreement with the organisms isolated from the throat swabs after admission. The S. aureus isolated from throat swabs after admission showed a decrease in the bacterial activity of cloxacillin, cephazolin and cefotaxime since 1978.
Follow‐up study of auditory brainstem responses in hyperbilirubinemic newborns treated with exchange transfusion
Authors: FUNATO MASAHISA , TERAOKA SATORI , TAMAI HIROSHI , SHIMIDA SEIICHI
Published: January 2011

Changes in auditory brainstem responses (ABR) were studied and followed in hyperbilirubinemic newborns before and after exchange transfusion (ET), in order to check their usefulness in the early detection of acute and chronic bilirubin encephalopathy.

ABR were measured in 10 newborns with marked hyperbilirubinemia (total bilirubin concentration [TBC] ≧ 20 mg/dL, direct bilirubin concentration < 2 mg/dL) before and after ET. The means of birthweight, gestational age, and day of life on admission were 3267 g, 38.2 weeks and 3.4 days, respectively. The ABR measurements were performed before ET and 6.0 days (the mean) after the ET. The follow‐up of ABR was performed at 3 months of life.

In comparison with the control values, the mean latencies of ABR were significantly prolonged (I, I‐III, and I‐V: P < 0.05) and the mean amplitudes were significantly decreased (I, III and V: P < 0.001) before ET. Significant improvement of ABR was noticed after the ET, especially in the shortening of the latency of wave I (P < 0.02) and in increasing the amplitudes of wave III and V (P < 0.02 and (P < 0.01, respectively), though the recovery of the latency of I‐V (P < 0.02) and the amplitudes of I, III and V wave (P < 0.001, P < 0.001 and P < 0.01, respectively) were delayed in comparison to the control. The follow‐up data of ABR showed that, in two of nine infants (one was lost from the follow‐up), there were still abnormal findings at 3 months of age. Only one of these, who prolonged the recovery of ABR until 5 years of age, developed a border intelligence.

Though ET is effective for improvement of acute bilirubin encephalopathy with impaired ABR, a complete recovery of the ABR might be delayed in marked hyperbilirubinemia. The delay in improvement of ABR abnormalities might be possibly used as an early predictor for following chronic bilirubin encephalopathy.
Hypoxanthine‐guanine phosphoribosyltransferase gene analysis for Japanese patients with Lesch‐Nyhan syndrome
Authors: SHIMIZU NORIKAZU , KONOMI HIROSHI , ARIMA MASATAKA , AOKI TSUGUTOSHI
Published: January 2011

The Lesch‐Nyhan syndrome results as a consequence of a severe deficiency of functional activity of purine salvage enzyme, hypoxanthine phosphoribosyltransferase (HPRT). We performed Southern blot analysis for five patients and their families using full length cDNA of the HPRT gene as a probe. Pst I digested Southern blot analysis revealed a large deletion that included exon 2 in patient 3. The size of this deletion was about 4.4 Kb. The mother of this patient had the same mutated allele and a normal one (heterozygote). This type of mutation from a Lesch‐Nyhan syndrome patient has not been previously reported. The restriction fragment length polymorphism (RFLP) pattern was analyzed by Bam HI digested Southern blot analysis for one family who had no major gene abnormality. We determined from this analysis that the sister of the patient was a Lesch‐Nyhan syndrome carrier and the fetus (brother) was normal for HPRT activity. This study shows RFLP analysis is still useful for carrier detection and prenatal diagnosis of Lesch‐Nyhan syndrome.
Acute disseminated encephalomyelitis associated with Mycoplasma pneumoniae infection
Authors: YAMAMOTO KATSUYA , TAKAYANAGI MASARU , YOSHIHARA YASUSHI , MURATA YUJI , KATO SEIICHI , OTAKE MASATOSHI , NAKAGAWA HIROSHI
Published: January 2011

An 8 year old girl with acute disseminated encephalomyelitis (ADEM) is described. Elevated serum antibody titers suggested recent Mycoplasma pneumoniae infection. T2‐weighted image of magnetic resonance imaging (MRI) disclosed multiple lesions of high signal intensity in bilateral basal ganglia and thalami as well as in the white matter. Postcontrast T1‐weighted image revealed an enhanced lesion in the deep white matter. She showed rapid clinical improvement in response to corticosteroid therapy. The lesions had disappeared completely on MRI performed 10 weeks after the onset. ADEM is believed to be a demyelinating disorder of probable autoimmune etiology. MRI findings in this case may support the hypothesis that the primary pathological event is vascular injury and demyelination occurs only as a secondary phenomenon.
Periventricular leukomalacia associated with hypocarbia
Authors: KUBOTA MASAYA , MATSUDA FUMIKO , HASHIZUME MINORU , NAKAMURA TOSHIHIKO , NISHIDA AKIRA
Published: January 2011

We here report a case of periventricular leukomalacia (PVL) associated with hypocarbia which remained even after extubation. The patient had no risk factors affecting PVL development other than hypocarbia. We consider that the irregular tachypnea which remained after extubation might be attributable to overdriving of ventilation of central neurogenic origin. Our patient's clinical course suggests that sodium bicarbonate drip infusion is a very effective way to alter the set point of respiratory neuronal drive of the patient with central neurogenic hyperventilation.
Interruption of aortic arch type A in two siblings
Authors: NAKADA TOSHIMASA , YONESAKA SUSUMU
Published: January 2011

The recurrence of interruption of aortic arch (IAA) in siblings is rare. According to previous reports concerning siblings with IAA, all cases were IAA type B according to the classification proposed by Celoria and Patton. In this report we present the first cases of brothers with IAA type A. The type of IAA was confirmed by autopsy findings in the elder brother and by operative findings in the younger brother. The etiology of IAA was unclear. Monogenic inheritance was thought unlikely because congenital heart disease was not recognized in other members of the family. It would seem prudent to separate recurrence risks for IAA type A from IAA type B when genetic counselling is provided, but it must be borne in mind that the recurrence of IAA type A can occur among siblings.
Noonan syndrome and its related disorders
Authors: FUKUSHIMA YOSHIMITSU
Published: January 2011

The diagnosis of Noonan syndrome still rests on its clinical features. There are several syndromes to consider in the diagnosis of Noonan syndrome. These include cardio‐facial‐cutaneous syndrome, LEOPARD syndrome, neurofibromatosis‐Noonan syndrome and Costello syndrome. The facial appearance and part of the clinical features of these syndromes are very similar to Noonan syndrome. Molecular research likely will elucidate whether these syndromes are variations of Noonan syndrome or etiologically different disorders.
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

Urinary 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

Postictal 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

Isolated 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

Hepatitis 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

We 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

The 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

As 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

An 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

The 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

Several 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

Severe 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

A 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

A 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

Periorbital 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

An 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

A 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

A 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

Reflux 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

Azoospermia 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.
Reverse transcription‐polymerase chain reaction amplification of respiratory syncytial virus genome from neonatal nasal swab samples
Authors: YOSHIO HIROYUKI , YAMADA MASAO , NII SHIRO
Published: January 2011

In order to make a rapid and accurate diagnosis of respiratory syncytial virus (RSV) infection, nasal swabs obtained from 14 neonates suspected of having this disease were examined for the presence of RSV genome by reverse transcription (RT) and nested polymerase chain reaction (PCR) amplification, along with enzyme immunoassay (EIA), serum neutralization testing and virus isolation. The RT‐PCR method was sensitive enough to detect a 0.1 50% tissue culture infectious dose (TCID50) per milliliter by nested PCR. The RSV antigen was detected from the samples at more than 100 TCID50 per milliliter by EIA. Nine patients were positive for the presence of RSV genome by first PCR on the day of admission, and eight were also positive by nested PCR even on the fifth hospital day. Among nine PCR positives, four patients were positive for EIA and five for virus isolation. No cases were serologically diagnosed. The cases that were negative for RT‐PCR were also negative according to the other methods. In the clinical setting, the RT‐PCR assay is more useful for diagnosis of RSV infection than other methods when the suspected cases are negative by EIA assay.
Plasma concentration and acute clinical effects of docarpamine, orally active dopamine prodrug, in infants
Authors: TOMITA HIDESHI , FUSE SHIGETO , CHIBA SHUNZO
Published: January 2011

Currently, there are no data available on the optimal doses and efficacy of docarpamine in infants. In the present study, three doses of docarpamine, 15.0–20.4 (19.0 ± 1.9; mean ± SD) mg/kg per dose every 8 h to 10 infants suffering heart failure. Age and bodyweight were from 1 to 4 (1.4 ± 1) months and 2960–5160 (3350 ± 872) g, respectively. In all infants, plasma concentrations of free dopamine were measured 1, 2 and 3 h after the first administration. Heart rate and systolic blood pressure were examined before and at the same time as the first administration. In seven infants, the 24 h urinary output and urinary excretion of electrolytes and creatinine before and during docarpamine were measured. Peak plasma concentration of free dopamine (ng/mL) was achieved after 1 or 2 h of administration, 0–163.1 (37.9 ± 47.2) and 0–105.0 (37.8 ± 39.3), respectively. The concentration had decreased rapidly by 3 h to 0–34.2 (12.4 ± 11.0). Both heart rate (b.p.m.) and blood pressure (mmHg) tended to increase from 120–154 (140 ± 15) and 56–90 (76 ± 11) to a peak of 124–162 (148 ± 14) and 70–92 (79 ± 8), respectively (P = 0.197, P = 0.289). There were no significant changes in urinary output or excreta. Oral docarpamine of 15–20 mg/kg per dose can achieve plasma free concentrations of dopamine that increase heart rate and systolic blood pressure.
Application of a new perchloric acid treatment method to measure endotoxin in both amniotic fluid and cord blood by an endotoxin‐specific chromogenic Limulus test in intra‐amniotic infection
Authors: SUDA HIDETOSHI , MOROI CHIKAE , INADA KATSUYA , CHIDA SHOICHI , YOSHIDA MASAO
Published: January 2011

Endotoxin in both amniotic fluid and cord blood was measured to detect intra‐amniotic fetal infection. Both amniotic fluid and cord blood plasma were pretreated by a perchloric acid treatment, and the endotoxin level was measured by Endospecy test. Cut off values for endotoxin in amniotic fluid and cord blood were 8.5 pg/mL and 7.6 pg/mL, respectively. Escherichia coli intra‐amniotic infection caused respiratory distress syndrome (RDS)‐mimicking pneumonia. Abnormally high values of endotoxin in both amniotic fluid and cord blood were detected. Intra‐amniotic infection caused by Gram‐positive bacteria (group B streptococci, Enterococcus fecalis) was shown to be endotoxin negative in both amniotic fluid and cord blood. In cases of negative amniotic fluid culture, measurement of the value of endotoxin in the amniotic fluid is useful in identifying intra‐amniotic fetal infection.
Serum transferrin receptor levels in the evaluation of iron deficiency in the neonate
Authors: RUSIA USHA , FLOWERS CAROL , MADAN NISHI , AGARWAL NEERA , SOOD SK , SIKKAI MEERA
Published: January 2011

Iron deficiency anemia (IDA) is a major global problem. Early onset of iron deficiency in developing countries makes it imperative to identify iron deficiency in neonates. Most conventional laboratory parameters of iron status fail to distinguish neonates with iron deficient erythropoiesis. Serum transferrin receptor (STFR) levels are a recent sensitive measure of iron deficiency and the present study was carried out to evaluate the usefulness of cord serum transferrin receptors in identifying iron deficient erythropoiesis in neonates. A complete hemogram, red cell indices, iron profile: serum iron (SI), percent transferrin saturation (TS%) and serum ferritin (SF) was carried out in 100 full‐term neonates and their mothers at parturition. Cord and maternal STFR levels were estimated using a sensitive enzyme‐linked immunosorbent assay (ELISA) technique. Anemic women had a significantly lower SI, their TS% and high STFR levels suggesting that iron deficiency was responsible for the anemia. In the neonates of iron deficient mothers, cord SI, TS% and cord ferritin were not significantly different from those of neonates born to non‐anemic mothers. Cord STFR level correlated well with hemoglobin (Hb) and laboratory parameters of iron status, and its level was significantly higher in neonates born to anemic mothers than in those bom to non‐anemic mothers. It was the only laboratory parameter to differentiate between neonates bom to anemic and non‐anemic mothers. Therefore, STFR is a sensitive index of iron status in neonates and identifies neonates with iron deficient erythropoiesis.
Transcapillary ultrafiltration and peritoneal equilibration test in pediatric patients
Authors: KAKU YOSHITSUGU , HISANO SATOSHI , KANEMITSU SATOMI , NOHARA KAORU , HATAE KEN , UEDA KOHJI
Published: January 2011

The usefulness of the peritoneal equilibration test (PET) in children is unknown. The relationship between transcapillary ultrafiltration and PET was investigated in order to evaluate the usefulness of PET in children. PET was performed on 14 patients undergoing peritoneal dialysis. Their age and bodyweight ranged from 3.8 to 23.6 years and 10.2 to 55.8 kg, respectively. The patients were divided into two groups according to bodyweight; group A patients weighed ≤ 40 kg (n = 7) and group B patients weighed > 40 kg (n = 7).

There was no significant difference in the mean infusion volume per bodyweight between the two groups, but the mean infusion volume per body surface area was smaller in group A than in group B. Group A showed a more rapid equilibration of dialysate glucose and creatinine than group B. Higher normalized mass transfer area coefficients were evident in group A. In spite of the lower effective glucose gradient in group A, the transcapillary ultrafiltration capacity (TUFC) showed no difference between the two groups. When the slope indices of the regression equations between the two groups were compared, the slopes of the regression in the relationship between TUFC and dialysate (D) ratios D/D0 glucose or D/P creatinine in group A were steeper than those in group B. Results of the present study indicate that the larger peritoneal area to infusion volume in patients with smaller body size results in both a rapid equilibration of solutes and sufficient transcapillary ultrafiltration.