bluuebunny/crossref_metadata_2025_split huggingface dataset:
A new protocol for the perinatal management of patients with congenital diaphragmatic hernia with severe hypoplastic lungs and its clinical application
Authors: SENZAKI HIDEAKI , TAMURA MASANORI , SAKAKIHARA YOICHI , HISHI TOSHIO 
Published: January 2011

AbstractIt is prerequisite for the pre‐operative management of patients with congenital diaphragmatic hernia (CDH) to avoid the factors that increase pulmonary vascular resistance, because such patients easily fall into the state of persistent fetal circulation (PFC). In this paper, a new protocol is proposed for the perinatal management of CDH patients to prevent the PFC which is facilitated by the enlargement of hernia and the deviation of mediastinum caused by spontaneous breathing just after birth. We also describe the usefulness of this protocol based on our experience in which we successfully treated a patient with CDH with severe hypoplastic lungs that were diagnosed antenatally by ultrasonographic examination. In treating the CDH patient with severe hypoplastic lungs, it is effective for the respiratory control and the prevention of PFC to administer morphine and pancuronium to the neonate through the umbilical vein before the resection of the umbilical cord. In the case of Cesarean section, in addition to the direct administration of morphine to the patient, administration of morphine to the patient's mother just before the delivery is more effective to prevent PFC, which can be easily induced by the initial resuscitation at birth.
A comparison of left ventricular volumes and regurgitant fraction by Doppler echocardiography and angiography in children
Authors: TAVLI VEDIDE , AL‐KHATIB YASSER , ISABEL‐JONES JOSEPHINE , TAVLI TALAT , WONG AH LIN , WILLIAMS ROBERTA 
Published: January 2011

AbstractRegurgitant fraction calculations by echocardiography were compared to angiocardiography in 11 patients with mitral regurgitation. Although there was a very good correlation when compared with angiocardiography (r = 0.99, SEE: 6.26, P< 0.001), two‐dimensional echocardiography underestimated left ventricular end‐diastolic volumes (P < 0.05). The mean regurgitant fraction was 36.2 ± 13.5% by echocardiography and 43.6 ± 15.7% (P< 0.05) by angiography. Most of the variability in measuring the regurgitant fraction is attributed to the left ventricular volume measurements. In conclusion, regurgitant fraction calculations of mitral regurgitation by echocardiography compares favorably to angiography. Since the severity of mitral regurgitation is an important determinant of prognosis, quantification of the regurgitant fraction may be useful in the long term follow‐up of those patients.
Interchange trisomy 9 due to maternal t(6; 9) translocation
Authors: NINOMIYA SHINSUKE , NARAHARA KOUJI , YOKOYAMA YUJI , TSUJI KAZUSHIRO , ITO SATOKO , AKAHORI WAITCHIRO , AKAHORI TAIITCHIRO , SEINO YOSHIKI 
Published: January 2011

AbstractThe occurrence of interchange trisomy due to a 3:1 malsegregation has been documented in only a few cases with trisomy 21. We describe the first case of interchange trisomy 9 due to a maternal t(6; 9) translocation. The patient, a boy neonate who died immediately after birth, had intra‐uterine growth retardation, specific craniofacial features including microcephaly with a high forehead, low‐set ears, upslanting short palpebral fissures, microphthalmia, bulbous nose and micrognathia, cryptorchidism, cystic kidney and various skeletal anomalies. His phenotype was consistent with that of the trisomy 9 syndrome. Cytogenetic analysis showed his karyotype of 47,XY,‐6, + der(6), + der(9)t(6; 9)(q27;q21.1)mat. The present report indicates that a very rare interchange mode of a 3:1 segregation can give rise to a live birth with full trisomy 9 in female carriers with reciprocal translocations involving the proximal long arm of chromosome 9.
High intensity in the globus pallidus on proton and T2‐weighted MRI in a case of dentato‐ruburo‐pallido‐luysian atrophy of myoclonus epilepsy type
Authors: IMAMURA ATSUSHI , SUGAI KENJI , WATANABE SEIJI , HAMADA FUMIHIKO , KURASHIGE TAKANOBU , TAKASHIMA SACHIO 
Published: January 2011

AbstractAlthough marked atrophy of the tegmentum of the mid‐brain is a known computed tomography (CT) and magnetic resonance imaging (MRI) feature in patients with dentato‐ruburo‐pallido‐luysian atrophy (DRPLA), this is the first report of marked high signal intensity of the globus pallidus on proton and T2‐weighted MR images in a patient in the early stage of DRPLA of the myoclonus epilepsy type. We suggest that the globus pallidus may be involved in the early stage of DRPLA of this type. Magnetic resonance imaging is considered to be useful for the early detection of involvement of the globus pallidus and in the clinical diagnosis of DRPLA.
Mucosal immunity and strategies for novel microbial vaccines
Authors: MESTECKY JIRI , MOLDOVEANU ZINA , NOVAK MIROSLAV , COMPANS RICHARD W. 
Published: January 2011

AbstractInfectious diseases continue to be the leading cause of morbidity and mortality worldwide. Increased awareness of the fact that mucosal membranes are the most frequent portals of entry of pathogenic microorganisms has prompted studies aimed at the development of vaccination protocols and antigen delivery systems that would lead to an increased protection of mucosae. Although systemic and strictly local immunizations are of limited effectiveness in the induction of mucosal protection, ingestion or inhalation of antigens results in a generalized immune response manifested by the appearance of specific antibodies of the secretory immunoglobulin (Ig) isotype in external secretions due to the dissemination of IgA precursor cells from IgA‐inductive lymphoid tissues. Furthermore, additional inductive sites strategically positioned at the opening of the respiratory and digestive tracts may also be suitable targets for induction of immune responses at desired effector sites. To prevent degradation and the increase of ingested antigens absorption, novel strategies including enclosure of antigens into biodegradable microspheres, liposomes or their expression in viral and bacterial vectors and plants are currently being considered. Forthcoming technological advances in antigen preparation and routes of delivery will undoubtedly have a profound impact on immunization practices in the future.
Food sensitive enteropathy: Overview and update
Authors: WALKER‐SMITH JOHN 
Published: January 2011

AbstractThere are two types of food sensitive enteropathy; permanent and temporary. Celiac disease belongs to the former, the temporary food sensitive enteropathies of early childhood to the latter. A food sensitive enteropathy is characterized by an abnormal small intestinal mucosa while having the offending food in the diet; the abnormality is reversed by an elimination diet, only to recur once more on challenge with the relevant food. These disorders are temporary and may follow gastroenteritis. Cow's milk sensitive enteropathy is the most frequent and best known example but soy protein, egg, fish, chicken meat, ground rice and probably gluten may also temporarily damage the small intestinal mucosa in infancy. Treatment is with an elimination diet and protein hydrolysates as a cow's milk substitute. The reason why these enteropathies are temporary has not yet been established.
Early infant feeding and micro‐ecology of the gut
Authors: ZETTERSTRÖM ROLF , BENNET RUTGER , NORD KARL‐ERIK 
Published: January 2011

AbstractNewborn infants are rapidly colonized by both aerobic and and anaerobic bacteria, initially with about 50% of each type. Several factors related both to the infant and its environment influence the composition of the intestinal microflora quantitatively as well as qualitatively. Major ecological disturbances are observed in newborn infants treated with antimicrobial agents. One way of minimizing the ecological disturbances, which may be seen in infants treated in neonatal intensive care units, is to provide them with fresh breast milk from their mothers and to use antimicrobial therapy only under strict clinical indications.
Absence of anti‐neutrophil cytoplasmic antibody (ANCA) in Henoch‐Schönlein purpura and immunoglobulin A nephropathy
Authors: KANEKO KAZUNARI , SUZUKI YOSHIHARU , YABUTA KEIJIRO 
Published: January 2011

AbstractWe have studied the presence of anti‐neutrophil cytoplasmic antibodies (ANCA) in 16 patients with Henoch‐Schönlein purpura (HSP) and 10 patients with immunoglobulin A nephropathy (IgAN).An indirect immunofluorescence test using ethanol‐fixed neutrophils as a source of antigen and an enzyme‐linked immunosorbent assay using purified proteinase‐3 and myeloperoxidase as antigens were used. Neither immunoglobulin G nor immunoglobulin A (IgA) ANCA were found in any of the patients studied. It was shown that fluorescein‐isothiocyanate conjugated anti‐human IgA antibodies bound to ethanol‐fixed normal human neutrophils non‐specifically, suggesting the possibility that false positive staining was responsible for the previous reports.We conclude that ANCA does not play an important role in the pathogenesis of HSP and IgAN.
Urinary excretion of acid‐soluble peptides in children with Duchenne muscular dystrophy
Authors: HIRANO KUMIKO , SAKAMOTO YOSHIMASA 
Published: January 2011

AbstractIn order to investigate the validity of the hypothesis that acid‐soluble peptides (ASP) in urinary excreta can be applied as an index of the protein catabolism of the whole body, we measured the urinary excretion of ASP in 46 normal children and in 18 children with Duchenne muscular dystrophy (DMD), in which continuous breakdown of skeletal muscle protein is presumed. The mean value of ASP in the children with DMD was significantly higher than that in normal controls.The concentration of ASP was correlated with that of 3‐methylhistidine (3MH), which has been proposed as an index of muscle breakdown. This finding indicates that urinary ASP reflects the catabolism of body proteins. No correlation was observed between the concentration of ASP and that of 1‐methylhistidine (1MH), which is used as an objective index of meat and fish ingestion.After the administration of bestatin, an inhibitor of leucine aminopeptidase, for 9 months, the urinary ASP concentration of children with DMD increased markedly. This increase is thought to have been directly caused by the bestatin itself.Urinary ASP is therefore apparently a more conveniently applied index of protein catabolism than is urinary 3MH, which requires the application of several restrictions. However, it should not be applied when the effect of bestatin administration is evident.
Efficacy of captopril treatment in children with steroid‐resistant nephrotic syndrome
Authors: SÖZÜER DT , EMRE S. , TANMAN F. , SIRIN A. , NAYIR A. , UYSAL V. 
Published: January 2011

AbstractWe studied the efficacy of captopril, an angiotensin‐converting enzyme inhibitor in treating persistent moderate or severe proteinuria in children with various glomerular diseases other than minimal‐change nephrotic syndrome. Captopril was administered for 3 months to 15 normotensive and nonazotemic or mildly azotemic patients (12 boys, 3 girls) in whom corticosteroid and cytotoxic treatment had failed to induce remission. Urinary protein excretion decreased from 2873.14±1937.50 (mean ± s.e.m.) to 1684.71 ± 1463.13 mg/day (P < 0.05). The reduction in proteinuria was not related to a significant fall in systemic blood pressure or a change in renal function. Serum albumin did not rise and side effects due to captopril were not observed. We concluded that, in the short term, captopril can be used safely and effectively for decreasing the proteinuria of nephrotic children unresponsive to conventional therapy.
Exercise performance in children with hyperthyroidism
Authors: OTSUKA HIROKO , MURATA MITSUNORI , WAKASUGI KUNIYO , HARA MISUZU , IKETANI YUKO , TATARA KATSUNORI 
Published: January 2011

AbstractAn attempt was made to define exercise performance in children with untreated hyperthyroidism using treadmill stress testing. Data were obtained for five female patients and the results were compared with those obtained for 16 normal female subjects. There were no significant differences at rest between the hyperthyroidism group and the control group in oxygen uptake, minute ventilation and respiratory rate. On the other hand, heart rate in the hyperthyroidism group was significantly higher than that in the control group. During exercise, there were significant differences between the two groups in oxygen uptake, heart rate, minute ventilation and respiratory rate. Hyperthyroid patients did not show an abrupt increase in heart rate during the first 30s of exercise. Exercise stress testing can therefore reveal cardiopulmonary abnormalities that are not evident at rest in children with hyperthyroidism.
A case of common variable immunodeficiency associated with cyclic thrombocytopenia
Authors: KUBOTA MASARU , NAKAMURA KENJI , WATANABE KENSHIRO , KIMATA HAJIME , MIKAWA HARUKI 
Published: January 2011

AbstractA 12 year old boy was found to be deficient in immunoglobulins (Ig) A, G2 and G4, and common variable immunodeficiency was diagnosed. He also had cyclic thrombocytopenia at intervals of approximately 28–30 days. His bone marrow revealed normocellular with slightly decreased megakaryocytes. In vitro colony assays showed markedly imparied megakaryocytopoiesis, erythropoiesis and granulopoiesis. Platelet‐associated IgG was elevated at his thrombocytopenic phase. Direct Coombs' test was repeatedly positive. Although not defined at present, we suggest the autoimmune nature of the disease.
Bone marrow transplantation for erythroleukemia: A case report
Authors: KOGAWA KAZUHIRO , SEKINE ISAO , MASUDA TOMOO , TAKADA YOSHINOBU , YOSHIOKA SHIGETAKE , HIROTA TAKAHISA , EGUCHI MITSUOKI 
Published: January 2011

AbstractA 2 year old girl was diagnosed as having erythroleukemia (EL; M6 according to the French‐American‐British classification). After one course of low‐dose cytosine arabinoside (Ara‐C), complete remission was obtained. After three courses of low‐dose Ara‐C for consolidation, allogeneic bone marrow transplantation was performed from HLA‐identical sibling. The course of post‐transplantation was uneventful. Two years after transplantation, she continues to have durable engraftment and remission. In children with EL, conventional chemotherapy appears to be inadequate for producing durable long‐term disease‐free survival. Bone marrow transplantation should be considered in children with EL, in cases where suitable donors are available.
Consecutive cerebral MRI findings of acute relapsing disseminated encephalomyelitis
Authors: MIZUTANI KEN‐ICHI , ATSUTA JUN , SHIBATA TAKEO , AZUMA EIICHI , ITO MASAHIRO , SAKURAI MINORU 
Published: January 2011

AbstractA seven year old girl with acute relapsing disseminated encephalomyelitis (ARDEM) was observed with serial magnetic resonance imaging (MRI) examination.She had the first encephalomyelitis episode at the age of 2 years 9 months. She entered remission with steroid therapy. She suffered a recurrence at the age of 7.Steroid therapy was instituted and consecutive magnetic resonance imaging (MRI) was performed. In spite of rapid improvement of neurological abnormality with steroid and steroid pulse therapy, disseminated high intensity lesions in T2‐weighted imaging which were considered as demyelination remained for a long time. Magnetic resonance imaging seems to be most suitable for evaluating the clinical condition and long‐term follow‐up of ARDEM.
Idiopathic infantile hypercalcemia discovered in the newborn period
Authors: SHIMIZU HIROKO , KODAMA SOICHI , TAKEUCHI ATSUKO , MATUI TADATAKA , NAKAO HIDETO , SAKURAI TAKASI , KOBAYASHI TADASHI 
Published: January 2011

AbstractWe report a male newborn with typical clinical signs of idiopathic infantile hypercalcemia (IIH); that is, hypercalcemia, hypercalciuria, an elfin face and nephrocalcinosis without giving Vitamin D3 supplementation to the patient. He had been treated with a vitamin D‐free, low calcium milk and rectal administration of exogenous calcitonin (elcatonin). The latter seemed to be more effective as a treatment for IIH. The serum calcium level came within the normal range and the serum 1,25‐dihydroxyvitamin D3 (1,25[OH]2D3) level decreased from 101.5 to 75.6 pg/mL with the treatments mentioned above. These results suggest that a high serum concentration of 1,25(OH)2D3 is part of the pathogenesis of IIH. However, we were not able to clarify the pathogenesis of the high serum concentration of 1,25(OH)2D3.
Kabuki make‐up syndrome associated with chronic idiopathic thrombocytopenic purpura
Authors: WATANABE TOHRU , MIYAKAWA MASAKO , SATOH MASAHISA , ABE TOKINARI , ODA YOSHIHIKO 
Published: January 2011

AbstractAlthough susceptibility to infections in Kabuki make‐up syndrome (KMS) has frequently been reported, there have been few immunological studies. We describe a 14 year old girl with KMS exhibiting chronic idiopathic thrombocytopenic purpura (chronic ITP), including immunological studies.Corticosteroid therapy was not effective therefore splenectomy was performed. The patient's platelet count increased transiently. Immunological studies revealed normal T cell functions and low serum immunoglobulin A (IgA) levels.Because of the abnormalities of B cell functions in chronic ITP and low serum IgA levels in our patient, we speculate that there may be some abnormalities of humoral immunity in KMS.
Age‐related profile of neuroblastoma: A comparison of tumors detected by mass‐screening with those detected clinically
Authors: TANAKA TAKEO , TANABE MASAHIRO , SUGIMOTO TOHRU , IDA NORIAKI , SAWADA TADASHI 
Published: January 2011

AbstractInfants with neuroblastoma are known to have a favorable prognosis compared to those over 1 year of age. However, there is little biological information about the age‐related heterogeneity of neuroblastoma. We evaluated the biological profile comparing cases detected by mass screening with those detected clinically.A total of 238 patients with neuroblastoma were classified into four groups according to their age at diagnosis. Patients in group A were 0–5 months of age (n = 31). Patients in group B were detected clinically and were 6–11 months of age (n = 25). Patients in group C were 6–11 months of age and were detected by mass‐screening (n = 97). Patients in group D were more than 12 months of age (n = 85). The age‐related heterogeneity was evaluated by Kaplan‐Meier survival analysis, several clinical markers (neuron specific enolase, ferritin, vanillylmandelic acid and homovanillic acid) at diagnosis, tumor Ha‐ras p21 expression and tumor N‐myc amplification.Infant neuroblastoma had unique features in comparison to neuroblastoma diagnosed over 12 months of age. Clinical outcome of the patients in groups A and C was quite favorable. Even patients with stage III or IV disease in group A had a favorable prognosis. However, stage IVs disease in group A was not necessarily associated with a good prognosis and the early death after diagnosis was also characteristic. The biological profile of tumors in group C was similar to that in group A but different from the profile in groups B and D. Tumors in group B had a biological profile intermediate between groups A and D.Cases detected by mass screening (group C) provided a new clinical entity with a good prognosis. The difference in biological profiles might affect their clinical outcome of respective age group. These analyses confirm the significance of prognostic markers and may help to direct an appropriate modality of treatment for the individual patient.
Opsonic activity in cord blood: Comparison between normal and elevated immunoglobulin M infants
Authors: NISHIDA AKIRA , KIMURA HIROKAZU , NAKANO MINORU 
Published: January 2011

AbstractIn order to clarify the relation between opsonic activity (OA) in newborn infants and their gestational age, we determined OA by Cypiridina luciferin analog‐dependent chemiluminescence (MCLA‐CL). In addition, complement C3 which plays the major role in OA, was determined by the two‐dimensional immunodiffusion method.Although OA and C3 in newborn infants increased in proportion to their gestational age, the levels were significantly lower in full‐term infants than in adults. In neonates with IgM levels of more than 30 mg/dL, at birth, both OA and C3 levels were significantly higher than in normal neonates, yet these levels were significantly lower than in adults.These findings suggest that low opsonin activity is an important factor in neonatal susceptibility to infection. When OA is measured by chemiluminescence, MCLA‐CL is the preferred technique to luminol‐dependent chemiluminescence.
Nephrocalcinosis in newborn
Authors: ÜÇSEL RAIF , ÇOBAN ASUMAN , KADIOǦLU ATEŞ , BILGE ILMAY , METIN FAZILET , YÜCER GÜL , CAN GÜLAY 
Published: January 2011

AbstractNephrocalcinosis is uncommon in childhood, and almost always develops outside the newborn period. Over the last decade, nephrocalcinosis due to multifactorial pathogenesis has increased in very low birth‐weight infants. In this report a 23 day old full‐term baby with nephrocalcinosis secondary to distal renal tubular acidosis is described.
A case of distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type
Authors: YAMAMATO TOSHIYUKI , ASAI KOHICHI , OHTA SHIGERU , IESHIMA ATSUSHI 
Published: January 2011

AbstractA male infant with distal 9q trisomy syndrome associated with an unusual inheritance of ABO blood type is reported. His clinical features were concordant with those of distal 9q trisomy syndrome. His karyotype was 46,XY, −10, +der (10) t(9;10) (q22.3;q24.3) confirmed by G‐banding and high resolution methods. His father had the balanced translocation t(9;10) (q22.3;924.3). He had a blood type of AB, despite his father's blood type of AB and his mother's blood type of O. The gene of ABO blood type is located at 9q34.1‐q34.2. Therefore, he would have received A and B type alleles from his father. 9q trisomy syndrome should be carefully investigated with ABO blood type.
Recurrent pneumococcal meningitis in a patient with transient IgG subclass deficiency
Authors: OHGA SHOUICHI , OKADA KENJI , ASAHI TAKAHIRO , UEDA KOHJI , SAKIYAMA YUKIO , MATSUMOTO SHUZO 
Published: January 2011

AbstractWe report the case of a 3 year old boy who exhibited recurrent serious infections with a transient imbalance of IgG subclass in the second year of life. He suffered from pneumococcal meningitis at 3 months, hepatitis at 9 months, and purulent arthritis at 11 months of age. The second episode of pneumococcal meningitis occurred at 14 months. Serum IgG level was normal for age. Low level of IgG2, undetectable level of IgG4 and negligible level of pneumococcus‐specific IgG1‐G2 antibodies were found. No other primary immunodeficiency was apparent. Serum IgG2‐G4 levels but not pneumococcus‐specific IgG1‐G2 titers increased by the age of 30 months. At that time, he was inoculated with a polyvalent pneumococcal vaccine along with acellular diphtheria‐pertussis‐tetanus vaccine. He acquired the immunity against these agents, and had no episodic infections in the following 2 years. This observation stresses the existence of transient IgG subclass deficiency associated with delayed development of the anti‐polysaccharide antibody response.
β‐Thalassemia with the IVS‐l‐1 (G → T) mutation in a Japanese girl
Authors: OKADA TAISUKE , TOMODA TAKASHI , MORITA HIDEO , WAKIGUCHI HIROSHI , KURASHIGE TAKANOBU 
Published: January 2011

AbstractWe analyzed the hemoglobins of a Japanese girl with β‐thalassemia and those of her immediate family. DNA sequencing of the cloned β‐globin gene from this patient revealed a point mutation at the IVS‐I position 1 (G → T). This rare point mutation has been found in Asian Indians, but this is the first reported Japanese case.