bluuebunny/crossref_metadata_2025_split huggingface dataset:
Testicular morphological changes in children with acute lymphoblastic leukemia following chemotherapy
Authors: KOBAYASHI H. , URASHIMA M. , HOSHI Y. , UCHIYAMA H. , FUJISAWA K. , AKATSUKA J. , MAEKAWA K. , HURUSATO M. 
Published: January 2011

AbstractMorphological changes in the testis induced by chemotherapy given according to the Tokyo Children's Cancer Study Group (TCCSG) regimens were studied in children with acute lymphoblastic leukemia (ALL). After informed consent, testicular biopsies were performed 14 times in 12 patients at the end of treatment. The testicular morphology in all cases had sustained a degree of damage. The tubular fertility index (TFI), calculated as the percentage of seminiferous tubules containing identifiable spermatogonia, was from 0 to 42.8% (mean 33.4%) below the normal value. Infiltration of leukemic cells was the most significant factor contributing to the decrease in TFI. There were no‐differences in the TFI among the TCCSG protocols. Formation of sperm was recognized in six cases, whose ages were 7, 8, 9, 10, 15 and 19 years. In two children, testicular biopsy was performed twice. In the second biopsy, TFI was elevated and sperm formation with the maturation of Leydig cells was observed. A number of other pathological changes were observed: modification of spermatogonia, Sertoli cells and inclusion bodies in spermatogonia, abnormal maturation of Leydig cells, evidence of interstitial fibrosis and thickening of the basement membrane. These results suggest that recent strong chemotherapy for the treatment of ALL might cause severe but not fatal damage to children's testicular tissue. As chemotherapy escalates, more investigation of testicular function will be necessary.
Listener and context dependency in the perception of emotional aspects of infant voice
Authors: SHIMURA YOKO , IMAIZUMI SATOSHI 
Published: January 2011

AbstractDevelopmental aspects of an infant's ability to express emotions through vocalizations were studied based on perceptual rating experiments against 12 vocalization‐ and emotion‐related reference words. Three groups of listeners, students, mothers with infants, and nursery governesses, rated 28 voice samples recorded from a male infant at 6, 9, 12 and 17 months of age, under a positive or negative context. Among three factors extracted by a factor analysis, one representing the emotional contrast of frightened/angry versus happy was found to be independent of listener group, infant age and context. The other two, one representing contrast between pleased/happy versus demanding/sad and the other seeking affection and rejecting/angry were dependent on infant age and context. These results may indicate that infants even at 6 months of age can express the emotional contrast of ‘pleasure’ versus ‘discomfort’ through vocalization, which listeners perceive independently in context.
Gas chromatographic‐mass spectrometric metabolic profiling of patients with fatal infantile mitochondrial myopathy with de Toni‐Fanconi‐Debré syndrome
Authors: NING CONG , KUHARA TOMIKO , INOUE YOSHITO , ZHANG CHUN‐HUA , MATSUMOTO MASAHIRO , SHINKA TOSHIHIRO , FURUMOTO TADASHI , YOKOTA KAZUKO , MATSUMOTO ISAMU 
Published: January 2011

AbstractThe metabolic profiles of three patients with fatal infantile mitochondrial myopathy with de Toni‐Fanconi‐Debré syndrome were studied by simultaneous analysis, after urease treatment of urinary organic acids, carbohydrates, polyols and amino acids using gas chromatography/mass spectrometry (GC/MS). All three patients persistently showed lactic aciduria, phosphaturia, glucosuria and generalized amino aciduria. This abnormal urinary metabolic profile was observed before the onset of any clinical symptoms, indicating that chemical diagnosis may be done presymptomatically. In one patient, the concentration of lactate increased in parallel with the severity of the clinical condition, whereas the urinary levels of 3‐hydroxybutyrate, amino acids and glucose fluctuated and showed only a general tendency to increase with the clinical course. The above results suggest that simultaneous GC/MS analyses, without fractionation, of urinary metabolites facilitate not only the early chemical diagnosis either before or after the first onset, but also follow‐up studies, providing an important index for the evaluation of the severity and clinical course in patients with this disorder.
Systemic air embolism in an extremely low birthweight infant treated with high‐frequency oscillatory ventilation
Authors: MARUYAMA KENICHI , KOIZUMI TAKENOBU 
Published: January 2011

AbstractAn extremely low birthweight infant in whom systemic air embolism occurred during high‐frequency oscillatory ventilation is reported. He was delivered at 25 weeks and 6 days gestation weighing 600 g. He was diagnosed as having respiratory distress syndrome and had intermittent mandatory ventilation. At 2 days of age, there was pulmonary hemorrhage and high‐frequency oscillatory ventilation was begun. The maximum mean airway pressure during high‐frequency oscillatory ventilation was 22 cmH2O. At 9 days of age, X‐ray film revealed air in the intracardiac cavity and major systemic vessels. Systemic air embolism can occur in an extremely low birthweight infant having high‐frequency oscillatory ventilation with a mean airway pressure of 22 cmH2O or higher.
Congenital chylothorax in a patient with 21 trisomy syndrome
Authors: YAMAMOTO TOSHIYUKI , KOEDA TATSUYA , TAMURA AKIKO , SAWADA HIROSHI , NAGATA IKUO , NAGATA NAOKI , ITO TAKASHI , MIO YASUYUKI 
Published: January 2011

AbstractA female infant with 21 trisomy syndrome associated with congenital chylothorax was reported. She was born at a gestational age of 34 weeks by Cesarean section because of fetal hydrothorax and hydrops fetus, confirmed by ultrasonography at 32 weeks. Emergent resuscitation and immediate thoracentesis were performed soon after birth. After beginning breast feeding, the serous pleural fluid became opalescent and a diagnosis of congenital chylothorax was made. Feeding was changed to medium‐chain triglyceride (MCT) feeding and the production of pleural effusion disappeared after thoracentesis was performed several times. Accumulating evidence suggested that MCT feeding and intermittent thoracentesis under echo guide were effective. Some reports on patients, including this one, suggest that there may be more patients with 21 trisomy associated with congenital hydrothorax. Therefore, congenital hydrothorax might be listed as a complication of 21 trisomy.
Apparently increased trough levels of tacrolimus caused by acute infantile diarrhea in two infants with biliary atresia after liver transplantation
Authors: MATSUI AKIRA , ARAKAWA YOICHI , MOMOYA TAKAYUKI , SASAKI NOBUHIKO , KAWASAKI SEIJI , TANAKA KOICHI 
Published: January 2011

AbstractTwo infants with biliary atresia who exhibited three‐fold increased trough levels of tacrolimus and required reduced doses during episodes of acute infantile diarrhea within 5 months of liver transplantation are described. The cause of the increase was not explained simply by hemoconcentration as a result of significant loss of extracellular fluid during these episodes. It does highlight an important issue: that of the continuing need to carefully monitor the trough levels of tacrolimus in such infants.
A simple and rapid polymerase chain reaction‐based method for detecting a prevalent mutation (R413P) in Japanese phenylketonuria patients
Authors: NAGAO MASAYOSHI 
Published: January 2011

AbstractA convenient molecular method for the detection of R413P (1238G←C) mutation in exon 12 of the phenylalanine hydroxylase gene, one of the prevalent mutations among Japanese patients with classical phenylketonuria (PKU) is described. The mutation was previously detected by polymerase chain reaction (PCR)‐direct sequencing or allele specific oligonucleotide hybridization. However, these methods were cumbersome and only a few laboratories could provide such a diagnostic service. An improved version of the method has been developed here, involving 30 cycles of PCR following restriction enzyme digestion. In the upstream primer encompassing G‐1207 to C‐1237, two substitutions are artificially introduced, so that a Bam‐HI site involving C‐1238 is introduced in the copies of the mutant allele. With the use of this method, R413P‐homozygote and ‐heterozygote can be readily and unequivocally distinguished from normal using genomic DNA extracted from peripheral blood leukocytes. Among 10 Japanese PKU patients investigated, three were homozygous and three were heterozygous for the R413P allele, whereas four did not carry this mutant allele, indicating that the prevalence of the mutant allele is 45%. The result suggests that it is technically feasible to develop a program for carrier detection of the mutation in the Japanese population.
Chronic urticaria associated with aseptic meningitis: An atypical urticarial vasculitis?
Authors: TANAKA HIROSHI , WAGA SHINOBU , KAKIZAKI YOSHIKI , SUGIMOTO KAZUHIKO , NOMURA KAZUO , YOKOYAMA MASARU 
Published: January 2011

AbstractThe patient was a 7‐year‐old girl with early onset urticarial cutaneous lesions and was later complicated with aseptic meningitis. Her skin lesions occurred in the infantile period and were diagnosed as urticaria, but did not disappear with antihistamines and were recurrent and persistent. In addition, she had experienced an episode of headache about once a month since 1991, when she was 4 years old, and was diagnosed as aseptic meningitis. All studies including skin biopsy for urticarial vasculitis (UV) and systemic lupus erythematosus (SLE) were negative except for the data from non‐specific inflammations. A systemic corticosteroid therapy dramatically reduced her symptoms. An unusual clinical course for this patient is described. It might suggest that this case is a presentation of the disease entity of UV, chronic urticaria and possibly SLE. To our knowledge, a similar case has not been previously reported.
Preschool sarcoidosis mimicking juvenile rheumatoid arthritis: The significance of gallium scintigraphy and skin biopsy in the differential diagnosis
Authors: SAKURAI YOSHIHIKO , NAKAJIMA MITSURU , KAMISUE SEIKI , NISHIMURA YAEKO , UEDA TAKU , MIYAGAWA SACHIKO , HARA TOKUKO , YOSHIOKA AKIRA 
Published: January 2011

AbstractPreschool sarcoidosis occurring in children less than 6 years old is rare and characterized by the triad of skin, joint and eye manifestations without any pulmonary lesion. Because of similar clinical manifestations, the diagnosis of preschool sarcoidosis and juvenile rheumatoid arthritis (JRA) is confusing. A girl with preschool sarcoidosis, initially diagnosed and treated as having JRA, is reported here. Ophthalmologic examinations revealed posterior involvement of the eye. A gallium scintigram of the head showed panda appearance. Biopsy of the cutaneous lesion demonstrated non‐caseating granuloma. Gallium scanning may be an important clue to correct diagnosis.
Isolated intracranial metastasis of neuroblastoma 2 years after completion of therapy
Authors: LYDAKI EVAGELIA , BOLONAKI IRENE , STIAKAKI EFTICHIA , DINI GIORGIO , KALMANTI MARIA 
Published: January 2011

AbstractMetastatic neuroblastoma in the brain without evidence of intracranial or extracranial disease elsewhere, is a very rare event. The pattern of spread is believed to be via the hematogenous route or cerebrospinal fluid. A child with a cystic neuroblastoma mass in the right temporal lobe 23 months following completion of therapy for the initial disease is described here. Chemotherapy with the protocol ‘eight drugs in 1 day’ was initially effective for this patient.
A case of dyshormonogenetic goiter complicated with growth hormone deficiency
Authors: KAJI MASAYUKI , TERASAKI ATSUKO , NAKATO HIDEKAZU , HATA IKUE , YOKOTA TAKASHI , FUKUMOTO MANABU , HAMASAKI SHUJI 
Published: January 2011

AbstractA 17‐year‐old male patient with dyshormonogenetic goiter complicated with follicular adenoma and growth hormone deficiency is described. He had short stature (‐2.3 SD), diffuse goiter and a particularly large nodule in the right lobe of the thyroid gland. The endocrinological studies revealed slight hypothyroidism. He underwent surgical removal of the tumor, diagnosed histopathologically as follicular adenoma, embryonal type; and the diffuse goiter was diagnosed as dyshormonogenetic goiter. It was speculated that long‐term hypersecretion of thyroid stimulating hormone (TSH) caused adenoma in the thyroid gland. Although the goiter disappeared after oral thyroxine replacement therapy, his height gain remained poor. Then, he was diagnosed partial growth hormone deficiency, and growth hormone therapy improved his height gain. It has been reported that a high percentage of patients with congenital hypothyroidism have additional anomalies. This is, however, the first reported case of dyshormonogenetic goiter complicated with growth hormone deficiency.
The safety of a nitric oxide inhalation system with high frequency oscillatory ventilation
Authors: SHIBATA YOSHIHIRO , OKAMOTO KAZUFUMI , KUKITA ICHIRO , KIKUTA KOICHI , SATO TOSHIHIDE 
Published: January 2011

AbstractNitric oxide (NO) inhalation and high frequency oscillatory ventilation (HFOV) has been indicated in infants with severe respiratory failure. The purpose of the present study was to evaluate the safety of an NO inhalation system with HFOV in terms of nitrogen dioxide (NO2) production. The NO inhalation system consisted of a high frequency oscillatory ventilator, a neonatal circuit and a test lung. The NO concentration was changed from 0 to 19 p.p.m. At each level of NO, the oxygen (O2) concentration was changed from 21 to 100%. The NO and NO2 concentrations were measured with a chemiluminescence analyzer using a molybdenum converter. The NO2 concentration was increased when either the O2 or the NO concentration was increased. The interposition of the endotracheal tubes increased NO2 concentrations at 4 p.p.m. NO. The high stroke volume and high mean airway pressure produced a significant increase in NO2 production at 4 p.p.m. NO. The increase in NO2 production was prevented by placing a one‐way valve at the joint of the NO gas line to the inspired limb. It was concluded that the NO inhalation system with HFOV can be safely used when a one‐way valve is placed at the joint of the NO gas line to the inspired limb and when inhaled NO is at a relatively low concentration.
Anthracycline‐induced cardiotoxicity in children with malignancies
Authors: GODOY LILIAN YS , FUKUSHIGE JUNICHIRO , IGARASHI HISAJI , MATSUZAKI AKINOBU , UEDA KOHJI 
Published: January 2011

AbstractThe anthracyclines (ATC) have been used in the treatment of many pediatric patients with malignancies. Their use, however, has been limited by a serious and potentially irreversible cardiotoxicity. The medical records of pediatric patients with malignancies who received ATC at the Department of Pediatrics, Kyushu University, from January 1985 to December 1994 were reviewed. Among the 120 children with malignancies who received ATC, six patients (5%) developed congestive heart failure, 33 (28%) had subclinical cardiac dysfunction, whereas 81 (66%) showed no evidence of cardiac dysfunction. The incidence of ATC cardiotoxicity increased at higher cumulative doses. The earliest cardiotoxicity, however, was observed in a patient who received 180 mg/m2 unassociated with other risk factors, showing the marked individual variations in its development. Children younger than 4 years had a higher incidence of cardiotoxicity compared with those older than 4 years (P < 0.01) and are therefore more vulnerable to ATC cardiotoxicity. Because serial myocardial biopsies and stress testing are not practical in younger children, and radionuclide angiogram is rather costly and not appropriate for frequent evaluation, non‐invasive tests such as echocardiography and electrocardiography (ECG) still play the main role for serial cardiac monitoring in these age groups. Radionuclide angiogram should be added as the confirmatory test modality.
Bronchial hyper‐responsiveness in atopic dermatitis
Authors: MIYABAYASHI YOKO 
Published: January 2011

AbstractMost patients with atopic dermatitis (AD) have symptoms of asthma at some point in their lives, but some do not have any clinical evidence of asthma. Thirty‐two patients with AD who had never suffered from bronchial asthma were investigated by the methacholine inhalation test for the presence of bronchial hyper‐responsiveness (BHR). Serum immunoglobulin E (IgE) levels, mite specific IgE antibody, peripheral eosinophil counts, serum levels of eosinophil cationic proteins (ECP), and serum levels of major basic proteins (MBP) were determined and a skin test to acetylcholine and adrenaline was performed. The percentage of cases who showed BHR (minimum dose; Dmin < 50 units) was 31.3% in AD patients and 19.0% in 21 healthy controls. In those cases, Dmin of methacholine in AD was significantly lower than that in the controls (P <0.01). Serum MBP levels were significantly higher in AD with BHR than in those without it (P < 0.05). These results indicate that some AD patients without asthma have BHR, and it may be related to serum MBP levels.
Evaluation of sponging and antipyretic medication to reduce body temperature in febrile children
Authors: AKSOYLAR SERAP , AKŞİT SADIK , ÇAǦLAYAN SUAT , YAPRAK IŞIN , BAKILER RAHMI , CETIN FUNDA 
Published: January 2011

AbstractTwo hundred and twenty‐four children aged 6 months to 5 years, with rectal temperatures greater than or equal to 39°C (104°F), were randomly treated with sponging alone or with medication including a single oral dose of aspirin 15 mg/kg, or paracetamol 15 mg/kg, or ibuprofen 8 mg/kg. Twenty‐three children were excluded from the final analysis because they did not complete the study. Demographic characteristics of the patients were found to be comparable in all groups. Rectal temperatures were recorded every 30 min for a 3 h period. During the first 30 min of intervention, sponging was found to be more effective than all of the three medications. After 60 min, the effects of each medication became superior to sponging with tepid water in reducing body temperature. Twenty‐three children were excluded from the final analysis because they did not complete the study. Comparing the effect of the three different medications, it was seen that the antipyretic efficacy of aspirin and ibuprofen were significantly more than paracetamol 3 h after intervention (P < 0.05). For the management of fever over 39°C, it is therefore recommended to give children an antipyretic drug, preferably ibuprofen, and at the same time to begin sponging to provide a rapid and sustained antipyresis.
Atypical Epstein‐Barr virus infection during and after intermittent FK506 therapy
Authors: AZUMA HIROSHI , SASAKI KAZUHIRO , MIYAMOTO KAZUTOSHI , TANAKA KOUICHI , KATO HIRONORI , UEMOTO SHINJI , OKUNO AKIMASA 
Published: January 2011

AbstractAtypical Epstein‐Barr virus (EBV) infection developed in a patient under intermittent administration of FK506 (one dose in 10 days) after living‐related liver transplantation. The clinical course was similar to severe chronic active EBV infection syndrome (SCAEBV), which is characterized by extremely high titers of antibody to EBV antigens. The clinical symptoms improved without graft rejection even after the cessation of FK506; however, the titers of antibody to EBV antigens remained at high levels. It was considered that: (i) even intermittent use of FK506 could influence the immune response, which then induced atypical EBV infection similar to SCAEBV; and (ii) the impaired immune response, especially to EBV antigens, remained after complete cessation of FK506.
Mixed connective tissue disease in childhood: A nationwide retrospective study in Japan
Authors: YOKOTA SHUMPEI , IMAGAWA TOMOYUKI , KATAKURA SIGEKI , ITOH SHUH‐ICHI , MITSUDA TOSHIHIRO , FUJIKAWA SATOSHI , AIHARA YUHKO 
Published: January 2011

AbstractSixty‐six children with mixed connective tissue disease (MCTD) were analyzed by a nationwide prospective study. The diagnostic significance of Raynaud's phenomenon and positive anti‐RNP antibody was confirmed, and additional symtoms including swelling of fingers, facial erythema, and polyarthralgia, and laboratory findings such as positive rheumatoid factor, hypergammaglobulinemia, and increased levels of myogenic enzymes, were variably positive. These clinical and laboratory characteristics of MCTD were critically different from those of systemic lupus erythematosus, indicating that MCTD is an independent entity of disease.
Aortitis syndrome in children: Clinical observation of 35 cases in Japan
Authors: MAEDA MOTOHARU , KOBAYASHI MUNEMITSU , OKAMOTO SHIZUKA , FUSE TOMOKO , MATSUYAMA TSUYOSHI , WATANABE NOBUO , FUJIKAWA SATOSHI 
Published: January 2011

AbstractThe results of clinical observation of 35 patients with aortitis syndrome (AS) in childhood, obtained by a nationwide survey in Japan, are reported. The male to female ratio was 1:2.5, the estimated age of onset averaged 10.2 years, and the duration from the estimated age of onset to the diagnosis averaged 15 months. In HLA examination A24, Bw52, Cw7 and DR2 were relatively common. Arterial lesions tended to extensively involve the aortic arch and its branches. Fever was the most frequently noted clinical symptom, followed by abdomen, joint and muscle pain. The physical findings in order of frequency were impaired circulation of the upper extremities, cardiac and vascular murmurs, hypertension, impaired cerebral circulation, visual disorder and impaired circulation of the pulmonary artery. The murmurs were found not only over the chest wall but also over the cervical area and abdomen. Pulselessness of the upper extremities occurred in 66% of patients. Percutaneous retrograde aortography and/or intravenous digital subtraction angiography to make the final diagnosis was employed except for three cases. There were not any specific abnormal signs in laboratory data. Steroid hormones were administered in 34 cases, and immunosuppressive agents in 8 cases. Five cases had percutaneous transluminal angioplasty to the right renal artery as an interventional treatment. The high frequency of abdominal pain is considered to be one of the characteristics of AS in childhood. The high frequency of pulselessness of the upper extremities and cardiac and vascular murmurs in this report is considered significant for the diagnosis of AS in childhood.
Behçet disease in children: A nationwide retrospective survey in Japan
Authors: FUJIKAWA SATOSHI , SUEMITSU TOMOKO 
Published: January 2011

AbstractThe purpose of the present study was to evaluate the incidence of Behçet disease among Japanese children and to compare their clinical features with adult patients. A first questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan. Fifty‐one cases of Behçet disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the Behçet's Syndrome Research Committee of Japan. Of 31 patients (14 boys and 17 girls), three cases were the complete type, 24 cases were the incomplete type, and four cases were the possible type. As to the specific type of the disease, 10 cases were of the entero‐Behçet type, two were a combination of the entero‐vasculo‐Behçet type, and four were the neuro‐Behçet type. In the first 6 months after onset, oral ulcers were observed in 77% of cases, genital ulcers in 45%, uveitis in 10%, and skin lesions were observed in 39% of cases. During the course of the disease, the frequency of oral ulcers was 100%, genital ulcers, 58%; ocular complications, 29%; and skin lesions, 55%. Gastrointestinal signs and symptoms were more frequent in childhood Behçet disease than in adults, while ocular complications were less frequent. The diagnosis of Behçet disease in children is difficult because of the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. Specific diagnostic criteria are necessary, based on a discussion of the many childhood cases of Behçet disease.