bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cloning of Human Thymic Subcapsular Cortex Epithelial Cells with SV40 Ori‐Gene
Authors: Mizutani Shuki , Watt Suzanne M. , Robertson David , Hussein Shabir , Healey Lyn E. , Furley Andrew J.W. , Greaves Melvyn F. 
Published: October 2007

AbstractThe thymic microenvironment involves complex cell interactions among different types of epithelial cells, macrophages, tissue histiocytes and immature and maturing T cells. We describe the isolation of a subset of thymic epithelial cells by selective primary culture followed by SV 40 ori‐and psV‐2 neo co‐transfection. The cloned cells were characterized as subcapsular cortex epithelial cells having hemopoietic growth factor activity, CSF‐1 and LFA3 associated binding sites for T cells. Thus cotransfection with the selectable genes and the gene which gives growth advantage to the transformant enabled us to investigate the rare type of cells which has been difficult to isolate by ordinary tissue culture technique because of mixed cellularity and fibloblast overgrowth.
Cytotoxicity of Mixed Human Monoclonal Antibodies Reacting to Tumor Antigens
Authors: Hagiwara Hideaki , Aotsuka Yasuyuki 
Published: October 2007

AbstractTwo kinds of human monoclonal antibodies, CLN‐IgG and SLN‐IgG, were obtained from human x human hybridomas, termed CLNH11 and SLNF10, respectively. These antibodies recognized different molecules which were expressed simultaneously on the cell surface of tumor cells. When both antibodies were added into culture medium in the presence of complement, a remarkable synergistic cytotoxic‐effect against glioblastoma cells was observed.
The New Pediatrics in Japan
Authors: Tokumaru Minoru 
Published: October 2007

AbstractPediatrics has arrived at a turning point. When diseases in children and the trend of demand for health care in Japan are observed, new fields are expanding which differ from traditional pediatrics, which set its sights on reduction of the death rate and control of infectious diseases; the new pediatrics aims at functional recovery and optimal growth and development, and involves what has to be done mainly in outpatient clinics. Pediatricians who work in these institutions must make their efforts in the ambulatory field and establish a form of ambulatory pediatrics which meets the needs of our “softening” society. Our efforts are necessary for the well‐being of our juniors. I present a framework for the new ambulatory pediatrics and enumerate its various tasks.
Intravenous Gamma Globulin for Prolonged Passive Immune Thrombocytopenia
Authors: Hara Toshiro , Kukita Johji , Yarnashita Hironori , Mizuno Yumi , Ueda Kohji , Shimokawa Hiroshi , Nakano Hitoo , Kishida Kunio 
Published: October 2007

AbstractThe effectiveness of intravenous gamma globulin was evaluated in prolonged thrombocyto penia in two infants of mothers with autoimmune thrombocytopenic purpura. Case 1, de spite unresponsiveness to steroid therapy, showed a slight response to intravenous gamma globulin infused at 15–19 days of age. A second administration of intravenous gamma globu lin at 70–74 days of age completely reversed the thrombocytopenia. In case 2, passive immune thrombocytopenia persisting over one month was reversed by the administration of intravenous gamma globulin at 37–39 days of age. A comparison of the effects of intra venous gamma globulin on the increase in platelet counts in the newborn infant (case 1) and in his mother showed that passive immune thrombocytopenia in neonates can be more resistant to therapy than autoimmune thrombocytopenic purpura in the mother. Adverse effects were not observed in either case. The effectiveness of and indications for intravenous gamma globulin in passive immune neonatal thrombocytopenia are discussed.
Studies on the Effect of Long‐Term Use of Low Dose Aspirin in Kawasaki Disease
Authors: Hamasaki Yuhei , Ichimaru Tomohiro , Tasaki Hakaru , Miyazaki Sumio 
Published: October 2007

AbstractThe inhibitory action of long‐term low dose aspirin (1–2 mg/kg/day for over 10 months) on the cyclooxygenase pathway in platelets and vascular endothe‐lium was evaluated in 10 patients with Kawasaki disease. The results were compared with those obtained after taking aspirin at 5–10 mg/kg/day during the acute phase of the illness. Platelet aggregations induced by adenosinedi‐phosphate (ADP), epinephrine and collagen were inhibited by aspirin doses of 1–2 and 5–10 mg/kg/day, when compared with those of controls (p < 0.05). Platelet synthesis of thromboxane B2 (TXB2) under doses of 1–2 and 5–10 mg/kg/day was 0.57 ± 0.07 and 0.72 ± 0.09 ng/ml platelet‐rich plasma (PRP) /105 platelets, respectively (p > 0.1). These values were significantly lower than those of the control group (22.88± 3.42 ng/ml PRP/105 platelets) (p < 0.05). No differences were found in platelet aggregation and TXB2 productivity between the two aspirin doses. Levels of 6 keto‐prostaglandin F1α (6k‐PGF1α) in platelet‐poor plasma (PPP) did not differ significantly in these 3 sets of data. The results indicate that long‐term administration of low dose aspirin (1–2 mg/kg/day) inhibits platelet aggregation by inhibiting synthesis of thromboxane A2 (TXA2), without interfering with prostacyclin production probably in the endothelium of blood vessels.
Cohen's Syndrome with Diabetes Mellitus
Authors: Nambu Mitsuhiko , Oshima Yusei , Kakiuchi Toshitaka , Hayakawa Yasushi , Ito Tadashi , Hasegawa Tomoko , Fujita Hiroko 
Published: October 2007

AbstractWe report a case of 12‐year‐old girl with Cohen's syndrome (CS) associated with diabetes mellitus (DM). She had the characteristic facies, with a short philtrum, an open‐mouth appearance, prominent maxillary central incisors, high palate, mild maxillary hypoplasia and micrognathia. Other features included truncal obesity, short stature, mental deficiency, small hands and feet, and accentuated thoracic kyphosis. She had been hypotonic during the neonatal and early infantile periods. Laboratory examinations showed hyperglycemia and glucosuria. HbAl and HbA1 c levels were raised. A 75‐g oral glucose tolerance test (OGTT) showed glucose intolerance consistent with DM, but there was a normal level of urinary C‐peptide. There were no islet cell antibodies (ICAs) and no history of ketoacidosis. This is the first reported case of CS with non‐insulin‐dependent diabetes mellitus (NIDDM).
Outcome of the Patients Detected by Newborn Screening in Japan
Authors: Aoki Kikumaro , Wada Yoshiro 
Published: October 2007

AbstractA follow‐up study of the cases detected by a newborn mass‐screening program has been performed during 1977–1985 by a collaborative study group.The number of hyperphenylalaninemic patients detected was 148, yielding a calculated incidence of one in 82,000. Of these patients, 102 cases were confired as classical PKU, 37 cases as hyperphenylalaninemia, and 9 cases as biopterine deficiency. The ratio of biopterine deficiency to PKU was about 9%. The incidence of maple syrup urine disease was estimated as one in 580,000, and the incidence of homocystimuria and galactosemia was found to be markedly low. Our survey has accumulated 1,362 cases of histidinemia establishing an incidence of one in 9,000. We have evaluated a large‐scale base to study the cad relation of histidinemia to mental development. It seems that low DQ or IQ scores of some histidinemic patients do not correspond to the blood histidine level.
Cognitive Development in Children with Inborn Errors of Urea Synthesis
Authors: Msall Michael , Monahan Peggy S. , Chapanis Natalia , Batshaw Mark L. 
Published: October 2007

AbstractChildren with congenital urea cycle disorders are at a high risk for cerebral damage, mental retardation and other developmental disabilities. Intellectual outcome appears to be correlated with the severity of the underlying cerebral damage and the duration of hyperammonemic coma. Thus, if a good outcome is to Le possible, early diagnosis and treatment is essential. However, even prospective treatment of affected children may not prevent cognitive impairment and even asymptomatic hyperammonemia may have subtle effects on intellect.
Acute and Chronic‐Intermittent Isovaleric Acidemia: Diagnosis and Glycine Therapy
Authors: Elsas Louis J. , Naglak Mary 
Published: October 2007

AbstractIsovaleric acidemia is caused by an inherited defect in isovaleryl‐CoA dehydrogenase (E.C. 1.3.99.10). L‐leucine restriction and glycine supplementation are used to decrease the toxic accumulation of isovaleric acid (IVA). Supplemental glycine augments conversion of IVA to isovalerylglycine gVG) through the alternate pathway glycine‐N‐acylase (E.C. 2.3.1.13). Two clinical phenotypes are described, acute and chronic‐intermittent, and are differentiated by enzyme activity in cultured fibroblasts. In this study we determined the optimum therapeutic glycine supplement for treating isovaleric acidemia under stable conditions of restricted leucine intake as compared to oral leucine loading. Under stable clinical conditions and on a restricted leucine intake of 55 mg/kg/day a nine year old with chronic intermittent IVA excreted 12.3 ± 5.8 of IVG which rose to 33.6 ± 14.2 mmoles IVG/gm creatinine as glycine supplements were increased from 0 to 50 mg/kg/day at weekly intervals. Excretion of IVC plateaued between glycine intakes of 50–150 mg/kg, but unexpectedly fell to 13.8 ± 0.5 and 16.3 ± 0.9 mmoles/gm creatinine when glycine supplements were further increased to 300 and 600 mglkglday. IVG excretion increased in a two year old with acute IVA while ingesting 45 mg leucine/kg/day from 5.5 ± 2.8 on no glycine supplement to 10.6 ± 0.8 mmoleslgm creatinine on 600 mg/kg glycine supplements. IVG production was compared in the older patient with chronic intermittent IVA. During acute leucine loads, glycine supplements of 190 or 600 mg/kg/day, resulted in urinary IVG of 194 and 419 mmoles/gm creatinine, respectively.These studies demonstrate that: acute and chronic‐intermittent isovaleric acidemia are differentiated with [1–14C] and [2–14C]‐leucine decarboxylation assays by intact fibroblasts; that glycine supplements of 150 to 250 mg/kg/day are optimum in both forms of the disease when patients are clinically stable but additional supplements will increase IVG production during acute leucine load conditions. However increases in glycine supplementation above 150 mg/kg/day may inhibit IVG production in patients with the chronic intermittent form of isovaleric acidemia, when intracellular IVA accumulation is minimized by diet, or if glycine‐N‐acylase is less competent.
Clinical Course of Hereditary Fructose Intolerance in 56 Patients
Authors: Endres Wolf , Sierck Tanja , Shin Yoon S. 
Published: October 2007

AbstractThe clinical course of hereditary fructose intolerance (HFI) was investigated retrospectively in 56 patients. The most frequently observed features were hepatomegaly, vomiting, diarrhea and failure to thrive. Fructose containing formulae given in the neonatal period lead in about one third of patients to cirrhosis of the liver — regardless of the amount ingested. Among patients receiving small amounts of fructose a mild clinical course was observed five times as often as a severe course. Two out of 12 patients who had developed cirrhosis of the liver, died soon after diagnosis in the early months of life. This stresses the danger of infants receiving fructose very early. Good dietary control brought serum transaminase activity to normal in half the time required with poor control. There was no correlation between dietary control and the duration of hepatomegaly.
Prediction of Prognosis in Patients with Hemolytic‐Uremic Syndrome
Authors: Matsurnoto Tohru , Ito Yuhei , Nakano Mitsurou , Yarnashita Yuji , Kinoshita Shouhei , Shindou Shizuo , Ph.D Norikazu Kuriya , Yamashita Furnio 
Published: October 2007

AbstractHemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia and acute renal insufficiency. Recently we experienced 6 HUS patients who had different clinical modes of onset, different clinical courses and a poor prognosis compared with the patients with the above typical clinical pictures. Thirteen HUS patients were evaluated in two aspects: mode of onset (acute or gradual type) and clinical corn (non‐recurrent or recurrent type).During 4.9 ± 3.1 (mean ± sd) years' of observation, renal insufficiency was more frequently observed in the gradual onset and recurrent course types respectively than in the acute onset and recurrent types (p < 0.05, p < 0.01). Also, the mean age was significantly lower and platelet counts were much higher in the gradual onset type than in the acute onset type (p < 0.05, p < 0.01). However, clinical findings except renal failure revealed no differences between non‐recurrent and recurrent HUS. For the prediction of HUS, we emphasize the importance of classifying HUS by the mode of onset (acute or gradual) and/or clinical course (non‐recurrent or recurrent).
Deletion of the Distal Long Arm of Chromosome 1: An Analytical Approach May Contribute to the Diagnosis
Authors: Yamamoto Yoshifumi , Okamoto Noriko , Yanagisawa Masayoshi , Nishimoto Hiroshi , Kann Zuishyo , Niitsu Naoki 
Published: October 2007

AbstractThe clinical findings in a female infant with a terminal deletion (1) (pter→q42.3:) are described and compared with 18 other cases displaying a similar chromosomal abnormality. bed on an analysis of the 19 cases, postnatal growth retardation, microcephalus, severe mental and motor retardation, muscle hypotonia, high‐pitched shrill cry, and hypoplasia of the external genitalia in males were almost invariably observed. The facial features appear to be insufficiently peculiar to a blink diagnosis, but analytical demonstration of round face, sparse hair, broad nose, smooth philtrum, prominent cupid's bow and downturned comers of the mouth may contribute to the diagnosis. The frequencies of convulsions and CNS anomalies, especially of agenesis of the corpus callosum were relatively high.
Prenatal Diagnosis in High Risk Pregnancies for Zellweger Syndrome
Authors: Aikawa Junichiro , Sato Koji , Miyabayashi Shigeaki , Tada Keiya , Narisawa Kuniaki , Chida Nobukazu , Hashimoto Takashi 
Published: October 2007

Zellweger syndrome is a lethal disorder. At present, no effective therapies are known for the patients of Zellweger syndrome. Recently a typical case of Zellweger syndrome in Japan was observed. In spite of intensive care, the patient died at the age of 3 months. Following this, the parents requested prenatal diagnosis for their following two pregnancies.We investigated levels of very long chain fatty acids (VLCFA), levels of bile acids in amniotic fluid and immunoblotting of peroxisomal β‐oxidation enzymes in cultured amniocytes. We report that immunoblotting using cultured amniocytes is an effective method for prenatal diagnosis of Zellweger syndrome. Furthermore, if we use immunoblotting for prenatal diagnosis, we can discriminate pseudoZellweger syndrome from pseudoneonatal adrenoleucodystrophy.Following prenatal diagnosis, two healthy babies were delivered. After birth, no abnormal levels of VLCFA in either serum or red blood cell membranes were confirmed. In this paper, we report that we can diagnose a healthy fetus in a high risk pregnancy for Zellweger syndrome.
Disseminated Intravascular Coagulation Syndrome in Anorexia Nervosa
Authors: Katamura Kenji , Ishimoto Fuminori , Yamasaki Masue , Yoshimura Masaki , Kisa Toshirou 
Published: October 2007

A 15 year old boy with anorexia nervosa developed disseminated intravascular coagulation syndrome (DIC). Because of severe cachexia he had been admitted to the Shimane Prefectural Central Hospital. During his hospitalization he developed generalized massive ecchymosis. Laboratory data revealed not only DIC but also multiple organ complications. The patient was treated intravenously with FOY®(gabexate mesilate, a protease inhibitor), heparin, a transfusion of fresh frozen plasma, antithrombin III concentrates and platelets. Intravenous hyperalimentation was also administered. The laboratory data, the general condition and the emotional state of the patient improved remarkably.We emphasize the importance of keeping in mind coagulopathy as a complication in anorexia nervosa.
Immature Gastric Teratoma in an Infant
Authors: Mufioz Nestor A. , Takehara Hiroo , Komi Nobuhiko , Hizawa Kazuo 
Published: October 2007

A 45 day old boy presented with progressive abdominal distension, tarry stools and anemia. A plain roentgenogram of the abdomen showed irregular, coarse calcifications in the left upper quadrant. The ultrasonography and computerized tomography of the abdomen revealed a large heterogeneous tumor with calcified parts in the left hemiabdomen. At operation, a 12 cm × 11 cm × 10 cm, multilobular, exogastric and endogastric tumor, including a portion of the anterior wall of the stomach, was extirpated. The pathological examination revealed a gastric teratoma with immature neural elements. Eight years after the total excision of the tumor there has not been any recurrence.This study includes a review of 88 cases (including the present case) of gastric teratoma reported in the English and Japanese literature.
Assessment of D‐glucose transport kinetics in the perfused human placenta: An in vitro study
Authors: Nandakumaran Moorkath , Dev Bhaskar R. , Makhseed Ma'Asoumah , Sugathan Thattaruparambil N. 
Published: October 2007

AbstractBackground: There have been no reports to date on glucose transport kinetics and the effect of graded hyperglycemia in the human placenta in non‐steady‐state conditions.Methods: The transport kinetics of D‐glucose in the human placenta was investigated in non‐steady state conditionsin vitrousing perfusion of isolated placental lobules. National Cancer Tissue Culture (NCTC) 135 culture medium, diluted with Earle's buffered salt solution was used as the perfusate.14C‐Labeled D‐glucose and water as reference were injected as a single bolus into the maternal arterial perfusate. Perfusate samples were collected and analyzed from the maternal and fetal venous outflows.Results: In four successful perfusions, differential transport rates of glucose in the maternal‐fetal direction averaged 1.03, 1.02, 1.09, 1.04 and 1.03 times those of corresponding tritiated water transport rates for 10, 25, 50, 75 and 90% of efflux fractions, respectively. Glucose transport fraction, expressed as percentage of injected maternal dose averaged 84 ± 3.1% of water transport fraction in the four perfusions. Glucose kinetic parameters expressed as area under the curve, elimination constant (Kel), clearance, time for maximum response, absorption rate and elimination rate averaged 0.25, 0.29, 3.96, 1.02, 0.25 and 0.18 times that of the corresponding tritiated water value, respectively. Neither the different kinetic parameters nor the transport fraction indices differed significantly when glucose concentrations in the same perfusions were raised successively from 5.56 to 27.80 and then to 55.6 mmol/L.Conclusions: We speculate that within physiological limits, hyperglycemiaper seplays no significant part in altering glucose transport kinetics across the human placenta in the maternal‐fetal direction.
Serum levels of C3 and Factors I and B in minimal change disease
Authors: Patiroglu Turkan , Melikoglu Ayse , Dusunsel Ruhan 
Published: October 2007

Abstract
Background:
Relapses are an important problem in minimal change disease, which accounts for most of the cases of childhood nephrotic syndrome. Because of defects in the humoral immune system, patients are predisposed to infection in nephrotic syndrome and infection is the most important complication that determines mortality and morbidity.
Methods:
In this study, serum levels of Factors I and B and C3 were studied to evaluate the relationships between nephrotic syndrome and infection in 17 children with nephrotic syndrome (24–96 months of age) and 10 healthy children (27–84 months of age).
Results:
Serum levels of Factors I and B were found to be lowered in the active disease group compared with the control group. These values were lowest for the infection group. Although it was observed that these values increased with steroid treatment, they did not reach normal levels. The parameters in remission were not different from the parameters in the control subjects. The serum level of C3 was found to be high during the active disease state and returned to normal levels during remission.
Conclusions:
The patients with active minimal change disease had infections such as peritonitis, septicemia and urinary tract infection because of low concentrations of Factors I and B in their sera.
Renal functional reserve in insulin dependent diabetic children
Authors: Semiz Serap , Bircan Iffet , Yilmaz GÜLsÜN GÜLay , KarayalÇIn Binnur , GÜVen Ayfer GÜR 
Published: October 2007

Abstract Background: Microalbuminuria has been shown to be predictive for clinical diabetic nephropathy. Renal functional reserve (RFR), as a response to protein loading in a short period of time, is a parameter to assess the ability of kidneys to increase the glomerular filtration rate (GFR). The aim of this study was to predict the early phase of diabetic nephropathy by measuring urinary albumin level and RFR capacity in patients with insulin‐dependent diabetes mellitus (IDDM). Methods: Twenty‐two patients with IDDM were studied: 11 with a disease duration of less than 5 years (group 1) and 11 with a disease duration of more than 5 years (group 2). As the control group, 15 healthy children (group 3) were included in the study. At the beginning of the study, glucose was measured and the urinary albumin/creatinine ratio was calculated. Average glycosylated hemoglobin (HbA1c) over 1 year was determined. After protein loading (red meat containing 2 g/kg of protein), the creatinine clearance was calculated at each hour for a duration of 4 h. The RFR was accepted as the peak percentage increase in GFR over the baseline value. Results: Although metabolic control in group 2 was better, the RFR in group 2 was significantly lower than in group 1 (P < 0.05). Urinary microalbumin levels between the groups did not differ (P < 0.05). In two patients in whom microalbuminuria was detected, the RFR was much lower. Conclusions: Detecting lower RFR levels in patients with normal urinary albumin excretion, as well as in patients with microalbuminuria, may support the idea that the RFR capacity is more sensitive than microalbuminuria in assessing the early phase of diabetic nephropathy.
Stretched minimal diameter of the ductus and coil occlusion
Authors: Tomita Hideshi , Fuse Shigeto , Chiba Shunzo 
Published: October 2007

Abstract
Background:
Stretching of the ductus was supposed to determine the size mismatch of the coil with the ductus in coil occlusion that results in residual leak or migration.
Methods:
We measured the minimal diameter (D) and the stretched minimal diameter (S) of the ductus in 12 patients with patent ductus arteriosus (PDA). The stretch index (SI) was calculated as S divided by D. We calculated ratios of the loop diameter of the first implanted coil to D (C,/D) and S (C/S) and those of the sum of all the loop diameters of all implanted coils for complete closure to D (total C/D) and to S (total CIS).
Results:
Stretched minimal diameter divided by D was 1.8 ± 0.3. Ratios of the loop diameter of the first implanted coil to D (C1/D), and S (C1/S) were 3.7 ± 1.8 and 2.1 ± 0.9, respectively. Ratios of the sum of all the loop diameters of all coils to D (total C/D) and S (total C/S) were 5.2 ± 1.6 and 2.9 ± 0.9, respectively. Standard deviations of C1/S and total C/S are significantly smaller than those of C1/D and total C/D, respectively.
Conclusions:
The narrowest segment of the ductus could be stretched to twice the size of the minimal diameter. The stretched minimal diameter may be a more reliable parameter to select the loop diameter of coils than the angiographic minimal diameter.