bluuebunny/crossref_metadata_2025_split huggingface dataset:
Phimosis of infants and young children in Japan
Authors: IMAMURA EIICHI 
Published: January 2011

AbstractThe prepuce and glans was examined in 4521 healthy infants and young children with a birthweight over 2600 g. There were 3238 infants aged 1–12 months and 1283 children aged 3 years. There is no custom of circumcision in Japan. The term phimosis implies the adhesion of the prepuce and glans, which cannot be separated by manipulation. Phimosis was found in 88.5% of infants aged 1–3 months, and the corresponding figures at the ages of 4–6 months, 7–9 months, 10–12 months and 3 years were: 74.4, 63.9, 58.0 and 35.0%, respectively. However, cases where the prepuce could be retracted by gentle manipulation were found in 3.0% of infants aged 1–3 months, 19.9% of those aged 10–12 months and increased to 38.4% of children aged 3 years, which exceeded the rate of phimosis. The complete adhesion of prepuce and glans was found in many infants, and a small space between the prepuce and glans was observed in some cases of 3‐year‐old boys. The smegma was notable in only 16 cases (0.5%) of infants and in 5 cases (0.4%) of the 3‐year‐olds. Balanopothitis was found in only one case of the 3‐year‐olds. It is not recommended to separate the foreskin by manipulation, which sometimes leads to bleeding or paraphimosis. And it is not necessary to surgically correct phimosis in infancy and early childhood except in the case of accompanying urological disturbance.
Comparison of specificity between IgG, IgE and T cells to three casein components: Implication for the role of circulating allergenspecific T cells in food allergy
Authors: SHIMOJO NAOKI , KOHNO YOICHI , KATSUKI TOSHIYUKI , HONMA KIRI , YAMAGUCHI KEN‐ICHI , TSUNOO HAJIME , TAKAHASHI TAKESHI , KAMINOGAWA SHUICHI , NIIMI HIROO 
Published: January 2011

AbstractIn order to investigate the role of food antigen‐specific T cells circulating in the blood of patients with food allergy, we compared T cell response to three casein components (αs‐, β‐ and, K‐casein) with specificities of IgG and IgE binding to the casein components in four milk‐allergic patients (P1‐4) with atopic dermatitis. In all patients, the binding activities of IgG antibodies to αs‐casein were most dominant, followed by those to β‐ and to K‐casein. The major component of casein bound by IgE antibodies was αs‐casein in P1 and P3, K‐casein in P2, and αs‐casein as well as K‐casein in P4; the order of casein components bound by IgE antibodies was different from that by IgG antibodies. Proliferative responses of peripheral blood mononuclear cells (PBMC) to casein components were so low that the dominance of casein recognition could not be clearly demonstrated. However, short‐term T cell lines that specifically respond to casein were successfully established from PBMC of the four patients and the proliferative responses of the T cell lines to the three components of casein were in accord with the IgE antibody specificity to casein components but not with that of IgG antibody specificity. When taken together, these results indicate that casein‐specific T cells circulating in the blood are involved in or reflect an allergic reaction against casein.
Serum fibronectin in neonatal sepsis: Is it valuable in early diagnosis and outcome prediction?
Authors: KOÇAK üLKER , EZER üSTüN , VIDINLISAN SADI 
Published: January 2011

AbstractThe value of the serum fibronectin level in early diagnosis of neonatal sepsis and as a prognostic indicator was investigated. The serum fibronectin levels and Töllner's sepsis scores of 45 neonates who were hospitalized for the suspicion of infection and of 20 healthy neonates as controls were evaluated. Depending on the findings it was concluded that serum fibronectin level varies according to the gestational age, and that the serum fibronectin level is a useful acute phase reactant in the early diagnosis of neonatal sepsis. It can also be used as a prognostic indicator in neonatal sepsis.
X‐linked severe combined immunodeficiency with γδT cells
Authors: JUNG EUN‐YOUNG , HEIKE TOSHIO , KATAMURA KENJI , KIMATA HAJIME , OHMORI KATSUYUKI , MORIKAWA YOSHIRO , ISHII NAOTO , MAYUMI MITSUFUMI 
Published: January 2011

AbstractA patient with X‐linked severe combined immunodeficiency (X‐SCID) was found to have a deletion mutation of a four base pair in the transmembrane domain of the IL‐2 receptor γ chain gene, a subunit shared by the receptors for IL‐4, IL‐7, IL‐9, and IL‐15 (common γ chain; γc). He had very few αβT cells but had a considerable number of γδT cells in his peripheral blood. Fluorescence in situ hybridization (FISH) analysis showed that the γδT cells in his peripheral blood were not of maternal origin. He had received a Bacillus Calmette‐Guerin (BCG) vaccination before recognition of the disease, and the BCG infection remained quiescent with no reaction for 19 months. After successful bone marrow transplantation, the site of the BCG vaccination showed a reaction, and live BCG were detected. It is useful to consider the relationship between the existence of γδT cells and BCG in this case, and it is suggested that γδT cells may be, in a given situation, less dependent on the γc chain than are αβT cells.
Sialic acid concentration in different stages of malignant lymphoma and leukemia in children
Authors: TOMASZEWSKA RENATA , SONTA‐JAKIMCZYK DANUTA , DYDUCH ANTONI , OLEJNIK IGOR , MAZUR BOGDAN 
Published: January 2011

AbstractSialic acid concentration in the blood serum of 104 children suffering from leukemia and malignant lymphomas was determined. A significant higher concentration of sialic acid was found at the onset of the disease, compared to the controls. In the group of children suffering from acute lymphoblastic leukemia a dependence was noted between the stage of the disease and the concentration of sialic acid in the serum. The high concentration of sialic acid at the onset of the disease, although decreasing during treatment, rose again during relapses of the disease. It seems that determination of sialic acid concentration may be useful in monitoring treatment.
Suspected acute encephalopathy with symmetrical abnormal signal areas in the basal ganglia, thalamus, midbrain and pons diagnosed by magnetic resonance imaging
Authors: MATSUSHITA EIJI , TAKITA KOSAKU , SHIMADA AKIRA 
Published: January 2011

AbstractA 4‐year‐old boy was admitted with disturbed consciousness following a convulsion. He developed bilateral pyramidal tract signs and showed a decerebrate posture. Laboratory findings revealed severe liver dysfunction and disseminated intravascular coagulation. On the eighth day eight in hospital he developed parkinsonism. However, 5 months from onset he had recovered almost completely. Brain CT on admission showed low density areas in the basal ganglia, thalamus, midbrain and pons. A T2‐weighted scan in magnetic resonance imaging (MRI) showed almost symmetrical high signal intensities in both basal ganglia (including putamen, caudate nucleus, globus pallidus), external capsule, internal capsule, thalamus, midbrain, pons and white matter of the peribasal ganglia; but a T1‐weighted scan showed low signal intensities in the same regions during all phases. Therefore hemorrhagic lesions or the presence of thalamic methemoglobin were excluded. It was considered that the pathogenesis may be cytotoxic cellular edema due to cytotoxic agents or acute metabolic factors. Clinical presentation, laboratory findings and radiological findings were most suggestive of acute necrotizing encephalopathy. As differential diagnoses, acute disseminated encephalomyelitis and brainstem encephalitis were considered.
Hypoparathyroidism and pseudohypoparathyroidism
Authors: YASUDA TOSHIYUKI , NIIMI HIROO 
Published: January 2011

AbstractHypoparathyroidism is a clinical disorder characterized by hypocalcemia and hyperphosphatemia in the absence of renal failure and hypomagnesemia. The causes of hypoparathyroidism can be classified as two groups: (i) insufficient parathyroid hormone (PTH) secretion in relation to the serum calcium level (hypoparathyroidism); and (ii) impaired PTH action (pseudohypoparathyroidism). The main emphasis in this report is to distinguish subgroups based on the etiology and pathophysiology of the various aspects of the disease.
Medical management and complications of X‐linked hypophosphatemic vitamin D resistant rickets
Authors: ONO TAEKO , SEINO YOSHIKI 
Published: January 2011

AbstractTo improve the growth failure, bowed legs, and biochemical and radiological abnormalities in patients with X‐linked hypophosphatemic vitamin D resistant rickets (XLH), combined therapy of phosphate and calcitriol is the best therapeutic approach at present. However, the complications involving combined therapy, such as hypercalcemia, nephrocalcinosis and hyperparathyroidism, are not fully solved. To achieve better control, new therapeutic approaches have been reported recently, for example, growth hormone (GH) or new vitamin D analogs. GH improved linear growth, decreased phosphate reabsorption and increased 1‐α‐hydroxylase activity. Furthermore, 24R, 25‐dihydroxyvitamin D3 (24,25) improved the bone lesions in hypophosphatemic (Hyp) mice, and also in XLH, without the adverse effects such as hypercalcemia or hypercalciuria compared with 1,25‐dihydroxyvitamin D3. These new approaches should be considered for the treatment of patients with XLH.
Recent progress in diagnosis and treatment of osteogenesis imperfecta
Authors: MORIWAKE TADASHI , SEINO YOSHIKI 
Published: January 2011

AbstractOsteogenesis imperfecta (OI) is an inheritable disorder characterized by bone fragility with various symptoms of connective tissue disorders. OI is commonly classified by Sillence's classification into four types according to the clinical features. The cardinal symptom is pathologic fracture, which is often recognized before birth, is frequent during infancy and childhood, then decreases at puberty. Bone mineral density is markedly decreased in OI, especially of the lumbar spine. Bone deformities are frequently observed in the long bones of the extremities, and spinal deformities and compression fractures are also common. Growth retardation is extremely severe, especially in type III. Calcitonin has been the most common therapy for OI. Recently, bisphosphonates have been found to be potent drugs that increase bone mass in OI patients. To prevent further fracture or bone deformity, appropriate orthopedic managements, including intramedullary rodding, are critically important. Growth hormone is effective in stimulating bone growth during childhood. The pathogenesis of OI is quantitative or qualitative abnormalities of type I collagen. The clinical features of each type usually correspond to the type of mutation. Several possibilities for gene therapy have been proposed.
Interferon therapy for Japanese hemophiliacs with chronic hepatitis C
Authors: MIURA TAKUMA , MEGURO TAKASHI , TAKAYAMA SHIGENOBU , YAMADA KANEO 
Published: January 2011

AbstractEight Japanese hemophiliacs with chronic hepatitis C (CHC) received interferon (IFN) therapy and four of them (50%) responded completely. Non‐responders included 3 double‐infected patients: I with hepatitis B virus (HBV) and 2 with human immunodeficiency virus‐1 (HIV‐1). In one of the patients with HIV‐1 double infection, the absolute number of CD4* lymphocytes decreased during IFN therapy. These findings suggest that hemophiliac patients with CHC can respond well to IFN therapy, but in patients who are double‐infected with HBV and HIV‐1, the indication of IFN therapy should be considered seriously.
Role of adenosine in the diagnosis and treatment of tachyarrhythmias in pediatric patients
Authors: LENK MUSTAFA , ÇELIKER ALPAY , ALEHAN DURSUN , KOÇAK GüLENDAM , ÖZME ŞENCAN 
Published: January 2011

AbstractTachyarrhythmias are common rhythm disturbances in infants and children. Despite the availability of diagnostic criteria arrhythmias are sometimes commonly misdiagnosed. Recent reports suggest that an endogenous purine nucleoside, adenosine, has a diagnostic effect in narrow QRS complex tachycardias, in addition to terminating supraventricular tachycardia involving the atrioventricular node. This report reviews the authors' experience with the use of adenosine for diagnosis of narrow and wide complex tachyarrhythmias in children. Adenosine was administered to 43 patients with several types of tachyarrhythmias (mean age, 8.3 ± 5.24 years). Nineteen patients had structural or acquired heart disease. Of the 43 patients there were 28 (65%) several different types of narrow QRS complex tachycardia and 14 (33%) ventricular arrhythmias. One patient (2%) had long QT. Adenosine terminated supraventricular tachycardia, in 11 of 12 patients (92%), ventricular tachycardia in five of eight patients (63%), and transiently terminated premature ventricular contractions in two of six patients (33%). The diagnostic ability of adenosine was perfect in eight supraventricular tachycardia. In these eight cases the tachycardia mechanism was unclear before the administration of adenosine, which demonstrated three cases of sinus tachycardia, three of atrial flutter, one of atrial fibrillation and one of atrial fibrilloflutter. Confirmation of the primary diagnosis by adenosine was perfect in five tachyarrhythmias including three cases of atrial flutter, one of atrial fibrillation and one of ectopic atrial tachycardia. The average effective dose of adenosine was 212 μg/kg (range, 100–400 μg/kg). There were no serious side‐effects except transient dyspnea, chest pain and flushing. These findings demonstrate adenosine to be helpful and safe in the diagnosis of tachyarrhythmias.
Comparison of synchronized and conventional intermittent mandatory ventilation in neonates
Authors: CHEN JIA‐YUH , LING UNG‐PAW , CHEN JEUN‐HORNG 
Published: January 2011

AbstractBetween October 1993 and April 1995, a total of 77 neonates requiring mechanical ventilation were enrolled in this study and were randomly divided into two groups. Group A consisted of 31 premature infants (mean birthweight 1.36 ± 0.29 kg) with respiratory distress syndrome (RDS) and seven neonates (mean birthweight 3.2 ± 0.5 kg) with meconium aspiration syndrome (MAS). Group B consisted of 31 premature infants (mean birthweight 1.31 ± 0.3 kg) with RDS and eight neonates (mean birthweight 3.3 ± 0.5 kg) with MAS. Infants in group A received synchronized intermittent mandatory ventilation (SIMV) and infants in group B received conventional intermittent mandatory ventilation (CIMV) therapy. In premature infants with RDS, our data showed: (i) the duration of ventilation was significantly shorter (P < 0.05) in the synchronized group (156 ± 122 h) compared to the conventional group (242 ± 175 h); (ii) significantly fewer (P <0.05) patients required reintubation in the synchronized group than in the conventional group (three vs 11 patients); (iii) incidence of severe intraventricular hemorrhage (grades 3 and 4) was significantly lower (P < 0.05) in the synchronized group compared to the conventional group (one vs seven patients); (iv) incidence of bronchopulmonary dysplasia was significantly lower (P < 0.05) in the synchronized group than in the control group (one vs seven patients). In neonates with MAS, our data showed no significant difference (P > 0.05) on duration of ventilation, incidence of reintubation, incidence of pneumothorax or mortality rate between synchronized and control groups.
Correlation between deletion patterns of SMN and NAIP genes and the clinical features of spinal muscular atrophy in Japanese patients
Authors: SAITOH MAKIKO , SAKAKIHARA YOICHI , KOBAYASHI SHIGETOSHI , HAYASHI YASUHIDE , YANAGISAWA MASAYOSHI 
Published: January 2011

AbstractWe conducted molecular analysis of two candidate genes for spinal muscular atrophy (SMA), the survival motor neuron gene (SMN) and the neuronal apoptosis inhibitory protein gene (NAIP), in 16 Japanese patients with SMA and compared the phenotypic features of SMA in these patients with the corresponding genotypes. Exons 7 and/or 8 of SMN were homozygously deleted in 11 SMA type I (Werdnig‐Hoffmann disease) patients, two SMA type II patients and one SMA type III patient. Exons 5 and 6 of NAIP were homozygously deleted in six SMA type I patients. No patient had a deletion in NAIP without a deletion in SMN. Mechanical ventilation was required during the first 7 months of life in the SMA type I patients who had a deletion in both SMN and NAIP. Ventilatory support was initiated within 2 years after birth in patients who had a deletion in SMN but not in NAIP. We detected homozygous deletion of exon 5 of NAIP in the unaffected mothers of two SMA type I patients. In these families, the patients exhibited a deletion in both SMN and NAIP. The parents and unaffected siblings of these patients did not have a deletion in SMN. The present findings support the hypothesis that SMN deletion plays an important role in the development of SMA and suggest that combined deletion of both SMN and NAIP may be relevant for determining the disease severity.
Magnetic resonance imaging of the cauda equina in two patients with Guillain‐Barré syndrome
Authors: FUCHIGAMI TATSUO , IWATA FUJIHIKO , NOGUCHI YUKIO , KOHIRA RYUTARO , YAMAZAKI HIROTAKA , OKUBO OSAMI , UTSUMI YASUFUMI , HARADA KENSUKE 
Published: January 2011

AbstractThe results are presented of magnetic resonance imaging (MRI) of the spine in two cases of childhood Guillain‐Barré syndrome. After injection of gadolinium‐diethylenetriamine pentaacetic acid, MRI showed enhancement of the cauda equina in these patients. These MRI observations may help confirm the diagnosis of Guillain‐Barré syndrome. The nerve root enhancement resolved as the clinical symptoms improved. Serial imaging may be useful in monitoring the response to therapy and assessing new treatment regimens. It may also yield a better understanding of the disease process.
Outcomes of infants whose mothers are positive for human herpesvirus‐6 DNA within the genital tract in early gestation
Authors: MAEDA TARO , OKUNO TOSHIOMI , HAYASHI KOZABURO , NAGATA MITSUO , UEDA MASAMI , TERASHIMA KAZUHIRO , KAWASHIMA TADAHISA , MIYAMOTO HAJIME , MORI TOSHIYUKI , YAMADA YOSHIYASU 
Published: January 2011

AbstractIt has been reported that HHV‐6 (human herpesvirus‐6) DNA has been identified within the female genital tract. However, the clinical significance of this finding has been unclear. The clinical outcome of the presence of HHV‐6 DNA in the genital tract of pregnant women on their infants was evaluated in the present study. One hundred and ten pregnant women were enrolled. Vaginal swabs were collected between 4 and 8 weeks of gestation and the presence or absence of HHV‐6 DNA was evaluated by nested polymerase chain reaction (nPCR). The swabs were cultured to isolate the virus. The women were divided into two groups: HHV‐6 DNA‐positive, and negative. The outcome variables of the infants of these two groups were statistically estimated at birth and at 1 month of age. Saliva and blood cells were collected from the infants at birth and at 1 month of age and were also evaluated by nPCR. HHV‐6 DNA was detected in the vaginal swabs of 28 pregnant women (25.5%), but was not detected in any other samples, including saliva and blood cells from their infants. Virus could not be isolated from any vaginal samples. Any outcome variables were not significantly different between the two groups. The presence of HHV‐6 DNA within the genital tract of pregnant women did not affect the health of their infants. It is suggested that HHV‐6 transmission to infants through the genital tract of their mothers during pregnancy does not occur, or only very rarely.
Interstitial pneumonitis probably induced by cyclophosphamide in nephrosis
Authors: HARIGAYA HIDEKAZU , MATSUBARA KOUSAKU , NIGAMI HIROYUKI , BABA KUNIZOU 
Published: January 2011

AbstractA case of interstitial shadows associated with oral cyclophosphamide therapy in a 32‐month‐old girl with steroid‐resistant nephrotic syndrome, who was admitted to the Nishi‐Kobe Medical Center with systemic edema, is reported. Due to the lack of response to prednisolone, cyclophosphamide was also administered orally at a dose of 3 mg/kg per day, 4 weeks after the start of steroid therapy. Approximately 3 weeks after the combination treatment she developed a fever, dry cough and cyanosis. Radiographic examination showed diffuse ground‐glass shadow in both lungs, presumably indicating that she had interstitial pneumonitis. Her pulmonary signs and symptoms deteriorated despite various antimicrobial treatments. A discontinuation of cyclophosphamide and the administration of high‐dose methylprednisolone yielded a dramatic improvement. These findings suggest that the diffuse pulmonary disease in this case was induced by cyclophosphamide. Since interstitial pneumonitis may be fatal and irreversible, attention should be paid to this rare complication even in patients undergoing low‐dose oral cyclophosphamide treatment.
Pneumoperitoneum complicating mechanical ventilation in congenital myotonic dystrophy
Authors: KOBAYASHI YASUAKI , TSUTSUMI YOSHIYUKI , SASAKI GORO , KAMIMAKI ISAMU 
Published: January 2011

AbstractPneumoperitoneum is known as a disease caused by perforation of the gastrointestinal tract, but it has also been reported as a complication of mechanical ventilation. In a case of congenital myotonic dystrophy (CMyD), mechanical ventilation was performed to treat severe respiratory distress starting at birth, and then pneumomediastinum and pneumoperitoneum developed as complications 5 days after birth. The fact that the pneumoperitoneum improved when the ventilation pressure was reduced and the patient's course was observed, allowed us to rule out gastrointestinal perforation. It appeared that hypoplasia of the diaphragm may have been involved in the progression of pneumomediastinum to pneumoperitoneum in this patient. Mechanical ventilation is performed on many occasions in CMyD, and the development of pneumoperitoneum secondary to pulmonary air leak should always be borne in mind.
Kabuki make‐up syndrome associated with West syndrome
Authors: MITSUDOME AKIHISA , YASUMOTO SAWA , FUKAMI SHIU , OGAWA ATSUSHI 
Published: January 2011

AbstractA Japanese boy with Kabuki make‐up syndrome associated with West syndrome is reported. He developed periodic tonic spasms at 6 months of age while his electro‐encephalogram also revealed hypsarrhythmia. Although only a few previously reported cases of Kabuki make‐up syndrome have been associated with epilepsy, it is likely that epileptic seizures are another primary feature of Kabuki make‐up syndrome.
Is dinitrogen fixation significant in the Levantine Basin, East Mediterranean Sea?
Authors: Yogev Tali , Rahav Eyal , Bar‐Zeev Edo , Man‐Aharonovich Dikla , Stambler Noga , Kress Nurit , Béjà Oded , Mulholland Margaret R. , Herut Barak , Berman‐Frank Ilana 
Published: January 2011

SummaryWe report N2 fixation rates measured from two stations monitored monthly off the Mediterranean coast of Israel during 2006 and 2007, and along a transect from Israel to Crete in September 2008. Analyses of time‐series data revealed expression of nifH genes from diazotrophs in nifH clusters I and II, including cyanobacterial bloom‐formers Trichodesmium and diatom‐Richelia intracellularis associations. However, nifH gene abundance and rates of N2 fixation were very low in all size fractions measured (> 0.7 µm). Volumetric 15N uptake ranged from below detection (∼36% of > 300 samples) to a high of 0.3 nmol N l−1 d−1 and did not vary distinctly with depth or season. Areal N2 fixation averaged ∼1 to 4 µmol N m−2 d−1 and contributed only ∼1% and 2% of new production and ∼0.25% and 0.5% of primary production for the mixed (winter) and stratified (spring–fall) periods respectively. N2 fixation rates along the 2008 east‐west transect were also extremely low (0–0.04 nmol N l−1 d−1, integrated average 2.6 µmol N m−2 d−1) with 37% of samples below detection and no discernable difference between stations. We demonstrate that diazotrophy and N2 fixation contribute only a minor amount of new N to the P impoverished eastern Mediterranean Sea.