bluuebunny/crossref_metadata_2025_split huggingface dataset:
Retrospective analysis of clonality and detection of residual disease in myeloid leukemia by FISH on long‐term stored bone marrow smears
Authors: Hyakuna Nobuyuki , Naritomi Kenji , Ito Etsuo 
Published: October 2007

Abstract
Background.
Fluorescence in situ hybridization (FISH) has allowed the detection of numerical chromosomal aberrations in interphase nuclei on fresh or frozen smears of leukemia.
Methods.
To analyze clonality and residual disease in myeloid leukemia retrospectively, we applied FISH to bone marrow smears stored at ambient temperature for up to 9 years.
Results:
When hybridization efficiency was investigated on stored control smears from patients without hematological malignancy, more than 96% of nuclei showed the expected number of signals using DNA probes specific for chromosome 7, X or Y. In combination with cell morphology, we observed much higher hybridization efficiency in blasts and granulomonocytic cells compared with lymphoid and erythroid cells. On the basis of good hybridization efficiency for old smear specimens, we applied FISH to stored bone marrow smears of myeloid leukemias, in which either loss of chromosome 7 or loss of sex chromosomes had been verified previously by conventional cytogenetics (one patient with chronic myelomonocytic leukemia (CMML) and four with acute myeloid leukemia (AML; three M2 and one M7)). As a result, the loss of chromosome was detected in blasts from all patients and was observed in mature granulocytes, except in M7. In the CMML patient and one AML (M2) patient with t(8;21), lymphoid and erythroid cells also showed the loss of chromosomes, suggesting that it should occur at stem‐cell level. A high amount of residual disease was detected in the morphological remission samples in one AML (M2) patient after induction therapy. The patient eventually succumbed to relapse.
Conclusion
Thus, the present FISH technique is useful to analyze the clinical significance of clonality and the residual disease in myeloid leukemia, retrospectively.
Mutation in the gene encoding the fibroblast growth factor receptor‐3 in Korean children with achondroplasia
Authors: Yang Sei Won , Kitoh Hiroshi , Yamada Yasukazu , Goto Haruko , Ogasawara Nobuaki 
Published: October 2007

Abstract
Background.
Achondroplasia (ACH) is the most common form of osteochondrodysplasia, and is mostly associated with a point mutation in the gene on the transmembrane domain of fibroblast growth factor receptor‐3 (FGFR‐3) on chromosome 4p.
Methods:
We investigated the mutations in the gene encoding FGFR‐3 in 15 Korean children with ACH, using polymerase chain reaction (PCR) coupled with direct sequencing.
Results:
In this study, all children with ACH showed the same mutation as those reported in France, USA and Japan; a G→A transition at position 1138 of the coding sequence, resulting in the substitution of arginine for glycine at position 380 of the mature protein.
Conclusions:
This consistent point mutation of Korean children with ACH indicates there is no significant racial difference in the pathogenesis of ACH, compared with data from Caucasian and Japanese children with ACH.
An evaluation of infants' growth in the Kingdom of Nepal
Authors: Hosono Shigeharu , Okazaki Minoru , Kagimoto Seiichi , Ogawa Kiyoshi , Matunaga Kohei , Oishi Tutomu , Ohno Tutomu , Yamaguchi Syuichi , Joh Kosuke , Akashi Syunji , Yamamoto Keiko , Kohno Saburo , Honma Takeshi , Shakya Kashyap Narsingh 
Published: October 2007

Abstract
Background
: The His Majesty's Government/Japan International Cooperation Agency Primary Health Care Project began in April 1993 in collaboration with the Saitama Prefectural Government, for the purpose of improving the health status of the people in model districts of the Kingdom of Nepal. Growth monitoring is one of the basic methods that defines the health and nutritional status of children.
Methods
: Anthropometric indices were measured in 759 children in the Bhaktapur district. We used the World Health Organization prototype growth chart and national growth standard for Japanese children (1990) to analyze the growth data.
Results
: We found that the average bodyweight growth curve of children up to 4 months of age followed the 50th percentile reference curve. For children of5–12 months of age, there was a delay in bodyweight gain and the growth curve reached the 3rd percentile curve. For children more than 1 year old, the growth curve moved below the third percentile curve. Catch‐up growth did not occur before the children reached 5 years of age. The main causes of catch‐up growth being hampered were chronic undernutrition and inadequate nutritional balance.
Conclusions
: As this was the first opportunity to evaluate infant growth in this district, the first important consequence of the results was to analyze the causes of growth faltering and failure‐to‐thrive in Nepalese children. Even more important, was the need to give appropriate counseling on improving feeding and other health‐related practices, and the most important consequence of all was to instruct Nepalese health workers that utilizing the growth charts is an integral part of health care.
Isolated deficiency of glucocorticoids presenting with cholestasis
Authors: BerberoǦLu Merih , YiǦIt Sevket , ÖCal GÖNÜL , Kansu Aydan , Tarcan Aylin , Girgin Nurten , Suskan Emine 
Published: October 2007

Abstract
Background:
Isolated deficiency of glucocorticoids is characterized by elevated levels of adrenocorticotropin (ACTH) and normal aldosterone production. It is rare for isolated deficiency of glucocorticoids to be associated with liver involvement. A case of an infant with isolated deficiency of glucocorticoids presenting with cholestasis is presented in this article. A male infant on his 39th postnatal day was referred to our hospital for evaluation of prolonged jaundice and convulsion. He had two episodes of hypoglycemic convulsion on postnatal 8th and 39th day, after which he was admitted to our hospital.
Results:
Physical examination revealed systemic jaundice, hyperpigmentation of the skin, hepatomegaly and splenomegaly on admission. He had normal male genitalia with 3.5 cm of penis and bilateral scrotal testes. Laboratory values were as follows: glucose 45 mg/dL, total biluribin 18.14 mg/dL, direct biluribin 6.54 mg/dL. aspartate aminotransferase 378 IU/L. alanine aminotransferase 46IU/L, and alkaline phosphatase (ALP) 1302 IU/L. In abdominal ultrasound and biliary tract scanning, extra‐ and intrahepatic biliary tracts were shown to be normal. Finally, biopsy of the liver revealed cholestasis. An endocrinological evaluation showed high levels of adrenocorticotropin (ACTH. 1000pg/mL), low levels of Cortisol (1 ug/dL) and normal aldosterone levels.
Conclusions:
The diagnosis of cholestasis secondary to isolated glucocorticoid deficiency was suspected with clinical and laboratory findings. Hydrocortisone treatment (30 mg/m2 per day) was initiated after which hyperpigmentation and jaundice decreased and ACTH and ALP levels reduced to 39 pg/mL and 440 IU/1, respectively. We emphasize that cholestasis in infants may be a component of isolated deficiency of glucocorticoids.
Mortality among patients with a history of Kawasaki disease: The third look
Authors: Nakamura Yosikazu , Yanagawa Hiroshi , Kato Hirohisa , Harada Kensuke , Kawasaki Tomisaku ,   THE KAWASAKI DISEASE FOLLOW‐UP GROUP
Published: October 2007

Abstract Background: Long‐term prognosis of Kawasaki disease is still unclear. Methods: In a cohort study, 6576 patients with Kawasaki disease were observed from their first medical encounter because of the disease through the end of 1994, or until death. Standardized mortality ratios (SMR) with 95% confidence intervals (CI) were calculated with vital statistics data of Japan used for the control. Results: Of 6576 patients who met the eligibility criteria, 6550 (99.6%) were followed through either the end of the study or the date of death. Twenty patients (14 male, 6 female subjects) died during the study period; an overall SMR of 1.35 (95% CI 0.82–2.08) was calculated. The SMR was 1.45 (95% CI 0.79–2.44) for male subjects and 1.15 (95% CI 0.42–2.52) for female subjects. During the acute phase of the disease (the first 2 months after the first visit to hospital), the SMR was higher, particularly in male subjects (SMR 10.13, 95% CI 3.72–22.08). After the acute phase, however, both boys and girls had low SMR. Nine of the 20 deaths were caused by Kawasaki disease; there were three deaths as a result of congenital heart diseases and two subjects died of malignant neoplasms of lymphatic or hematopoietic tissues. Conclusions: Although the mortality rate among those with a history of Kawasaki disease was elevated in Japan, many of the deaths that caused the elevation occurred during the acute phase of the disease. The mortality rate was not increased after the acute phase of the disease.
Community‐acquired severe staphylococcal septicemia in children: The relationship with blunt trauma
Authors: GÜLer Nermin , OneŞ ÜLker , YazicioĜLu Mehtap , YalÇIn IŞIk , Salman Nuran , Somer Ayper 
Published: October 2007

Abstract Background. With the aim of establishing the role of blunt trauma, a trauma without any fracture or obvious wound, as a risk factor in children who were admitted for community‐acquired severe staphylococcal septicemia with various organ involvement, 63 patients were investigated during a seven‐year study period. All children were previously healthy. Results: Blunt trauma was present in 12 (46%) of 26 septicemic patients in whom skeletal infections were diagnosed and the relative risk of trauma was high (odds ratio 15.0; confidence interval 2.9–75.8, P< 0.001) in these children. Patients with multiple organ involvement and with multifocal skeletal infections were more frequent among traumatized patients. Children with skeletal involvement were significantly older than others (P< 0.001). The period between the first symptom of infection and the diagnosis was shorter in traumatized children (P < 0.001). None of the children with staphylococcal pulmonary or soft tissue infection had any history of trauma. We conclude that blunt trauma is one of the important risk factors for skeletal involvement in staphylococcal septicemia. Its immunosuppressive nature, especially in local damage of the skin barrier, may be the cause of seeding and multifocal infection.
Effect of body position on tidal volume and minute ventilation in very low birthweight infants
Authors: Itakura Yukino , Ogawa Yunosuke 
Published: October 2007

Abstract
Background:
To progress the clinical treatment of neonates, especially in the management of respiration, we have to be able to measure their pulmonary function appropriately. Various methods have been developed, but little is known about the pulmonary function of very low birthweight infants (VLBWI) because of the difficulty in taking their measurements with existing equipment. We have developed a very low dead space pneumotachograph to measure lung function in VLBWI.
Methods and results:
We used our pneumotachograph on 30 infants each weighing less than 1500 g at birth. The infants were intubated with endotracheal tubes of 2.5 or 2.0 mm diameter to measure tidal volume and minute ventilation in the prone and supine position. The tidal volume in the supine position was 6.99 ± 0.42 mL/kg and 7.58 ± 0.38 mL/kg in the prone position (mean ± SE). The tidal volume was significantly larger in the prone than the supine position (P < 0.05). However, no significant difference was observed in minute ventilation and respiratory rates.
Conclusion:
The tidal volume significantly increased in the prone position in VBLWI, confirming the previous observation of larger healthy infants is also applicable to the very low birthweight infants.
Effectiveness and safety of intravenous amiodarone in drug‐resistant tachyarrhythmias of children
Authors: ÇEliker Alpay , Ceviz Naci , ÖZme ŞEncan 
Published: October 2007

Abstract Background: Experience with pediatric use of intravenous amiodarone is limited. In this study, our experiences with intravenous amiodarone in children with acute life‐threatening or chronic tachyarrhythmias are reviewed. Methods and Results: Twelve patients, with a mean age of 3.4 ± 3.1 years, range 9 months‐10 years (two with incessant ventricular tachycardia, one with ectopic atrial tachycardia, two with atrioventricular re‐entrant tachycardia (three episodes), four with postoperative or congenital junctional ectopic tachycardia, two with bradycardia‐tachycardia syndrome, one with atrial tachycardia) were treated with intravenous amiodarone during 13 tachycardia episodes. Left ventricular systolic functions were depressed in six patients. In 11 patients, a median of two drugs (range one‐four), including adenosine infusion and in five cases direct current cardioversion were tried without success prior to intravenous amiodarone. The loading dose of amiodarone was 5 mg/kg in all episodes, infused over 1 h. Maintenance infusion was required in 12 episodes. In 10 episodes (77%), amiodarone was considered effective, in one (7.6%) partially effective (junctional ectopic tachycardia) and in two (15.4%) ineffective (sick sinus syndrome, atrial tachycardia). Therapeutic effect was obtained in a median period of 30 h (range1–103 h). The mean effective maintenance dose was 10 ± 4.7μg/kg per min (range5–15 Hg/kg per min). In one patient, mild hypotension, and in three patients cellulitis occurred, but none of them necessitated termination of treatment. Conclusions: Intravenous amiodarone is found to be an effective and safe antiarrhythmic agent for children with acute life‐threatening and chronic tachyarrhythmias and depressed left ventricular systolic functions.
Hepatic damage in gluten sensitive enteropathy
Authors: AltuntaŞ Buket , Kansu Aydan , Girgin Nurten 
Published: October 2007

Abstract
Background:
Gluten sensitive enteropathy has been reported to occur concomitantly with liver abnormalities, such as primary biliary cirrhosis, chronic active hepatitis and primary sclerosing cholangitis.
Methods:
Duodenal biopsy was performed in nine children (all with short stature, five with chronic diarrhea and three with hepatosplenomegaly of unknown etiology) with a possible diagnosis of gluten sensitive enteropathy. All of the patients had different abnormalities in serum aminotransferase levels. With the help of laboratory investigations, viral, autoimmune, metabolic and toxic etiologies which cause hepatic damage were excluded. Liver biopsy was performed in five of these patients, two showing fibrosis and three non‐specific reactive changes. Gliadin antibodies were measured in six cases.
Results:
Intestinal mucosal histopathology was compatible with gluten sensitive enteropathy in all patients. While immunoglobulin (Ig) G gliadin antibodies were positive in all cases, only three cases were found to have positive IgA gliadin antibodies. After a gluten‐free diet, levels of transaminases fell to normal within 3 months and remained so in seven of these patients. A second intestinal biopsy, which was performed after 1 year of gluten‐free diet revealed normal intestinal mucosa in all patients.
Conclusion:
Gluten sensitive enteropathy should be considered when evaluating a child with elevated levels of serum transaminase and in cases with cryptogenic liver disease.
Hereditary pancreatitis: Report of a family from Turkey
Authors: Kansu Aydan , Girgin Nurten , YurdaydİN Cihan , Cetinkaya HÜLya , Uzunalimoglu ÖZden 
Published: October 2007

AbstractChronic pancreatitis is a rare disease in children and is usually secondary to underlying diseases such as hereditary pancreatitis, cystic fibrosis, hyperlipidemia, prolonged malnutrition, gallstones or anomalies of the biliary‐pancreatic duct system. Hereditary pancreatitis is a common cause of chronic pancreatitis in children but is often unrecognized until months or years later. We report here a family with hereditary pancreatitis in which four members are affected.
Growth and the Subsectoral Pattern of the Manufacturing Sector in Indonesia ‐ A Comparative Study of 16 Countries
Authors: Fukuchi Takio 
Published: October 2007

There is a widely held and optimistic view that, based upon the successful implementation of the product‐cycle theory and the technological ladder hypothesis, ASEAN countries, the so‐called Asian near NICs, will follow the Asian NICs up the ladder and take off as the second tier of NICs in the near future. To realise this ambition, each of the near NICs needs to successfully achieve two take‐offs: first, rapid quantitative expansion based upon existing comparative advantage and, second, the successful transformation of their industrial structures to create dynamic comparative advantages.The Indonesian manufacturing sector recorded an average growth rate of 12% in 1980‐90, but the accompanying structural changes were not great. The ‘rapid growth’but ‘modest structural change’of the Indonesian manufacturing sector raises the important problem of cultivating new leading sectors in the future. Usually the machinery subsector plays a big role in other ASEAN and NIES economies, but it still needs more momentum for development in Indonesia. This implies a strong need for the institutional and physical infrastructures, and supporting industries for the machinery subsector, as well as further diversification of the industrial structure.The purpose of this paper is to describe the features of industrialization in Indonesia in the 1980s, to measure the capacity of the transformation of the Indonesian manufacturing sector, and to discuss some policy issues related to furthering industrialization in the future. Section I contains some introductory remarks. In Section II, we implement the comparative analysis based upon two aggregate indicators. In Section III, we further analyse the structural changes at a more disaggregate level of 16 subsectors. In Section IV we concentrate on the machinery subsector, and analyse the growth potential and the necessary industrial policy in the medium or long run. In Section V we analyse the possible long‐run development of a specialization pattern in the Indonesia manufacturing sector. We present the summary and conclusions with some relevant policy discussions in Section VI.
A Short Term Step in Development of 3D Graphics Standards
Authors: Sabin M.A. 
Published: October 2007

SummaryFew of the arguments deployed above have been either device dependent or application area dependent. Even if further discussion leads to a resolution of these and future issues differnt from the author's, the case is made that a Transformations chapter could be written which would be naturally included in most application‐oriented 3D graphics standards.
The Development of Programs for the Processing of Parametric Curves
Authors: Koparkar P A , Mudur S P 
Published: October 2007

AbstractThis paper presents the development of a suite of programs for the processing of parametrically defined curves in two and three dimensions. The programs are presented in Dijkstra's non‐deterministic guarded command notation. The development of the proof of correctness for the programs is also described. Three typical curve processing tasks, such as drawing, curve path following and intersection detection, are described.The algorithms used in these programs are all based on the “divide and conquer (subdivision)” paradigm. Deviation from linearity and Euclidean bounds are two curve shape properties that are used in many of the curve processing tasks. We present fast methods of computing these properties from the algebraic representation of the curve. In particular the paper considers the cubic and the rational quadratic forms of representation.
The Systolic Pixel: A Visible Surface Algorithm for VLSI.
Authors: Mills P L 
Published: October 2007

AbstractThe Systolic Pixel or Spixel is a novel architecture for an intelligent pixel‐based graphics database for geometric‐solid models. An algorithm is described which performs visible surface calculations for any complexity of coloured 3‐dimensional (3‐D) surface and which structures geometric‐solid model data in a natural way. The algorithm/architecture of the spixel features a simple set of priority rules acting upon data in nearest neighbour locations and a simple set of movement rules of data to nearest neighbour locations. The spixel is constructed out of identical functional units. These features are attractive for an implementation of the algorithm in Very Large Scale Integration (VLSI).
Oriented geometric Objects in Computer Graphics and Numerical Control
Authors: Barbic J. , Dacar F. , Spegel M. 
Published: October 2007

AbstractConventional natural ‐ language as well as computer ‐ oriented descriptions of geometric constructions by means of geometric relationships (parallelism, tangency, etc.) and attributes (INSIDE, NEAR, MIDDLE, X‐LARGE, Y‐SMALL, etc.) do not offer a clear and efficient method for selecting a specific object out of a number of possible alternatives. This paper describes a systematic solution to the problem based upon the notions of orientation of geometric objects, of smooth transitions from one object to another (referred to as coherent tangency), and upon locality. Using the notions of object orientation and coherent tangency, some tangent constructions in a plane are developed. The method is shown to employ a minimum set of attributes ‐ at most one, characterizing the intrinsic parameters of geometric objects.
3D Icons and Architectural CAD
Authors: Aish Robert 
Published: October 2007

Abstract3D computer input has been a recurring challenge to engineers developing effective CAD systems. The approach adopted in this paper attempts to address a specific type of 3D input which is applicable to architecture and some engineering design tasks. In these processes, the object being designed is often an assembly of defined components. In a conventional graphics based CAD system these components are usually represented by graphical Icons which are displayed on the graphics screen and are arranged by the user. The system described here consists of 3D modelling elements which the user physically assembles to form his design. Each modelling element contains an element processor consisting of a machine readable label, data paths and control logic. The CAD system interrogates the elements. The logic within the element processors and the data paths are then used to interrogate other adjacent elements in the model. This system can therefore be considered as a “user generated”“machine readable” modelling system. In an architectural application this provides the user with a system of 3D Icons with which to model and evaluate the built environment.
Better Understanding through Formal Specification
Authors: Duce D. A. , Fielding E. V. C. 
Published: October 2007

AbstractThe Graphical Kernel System (GKS) is now registered as an ISO International Standard for computer graphics programming. One of the major innovations of the Standard is the bundled specification of aspects, a mechanism which gives the applications programmer the ability to tailor the appearance of a picture independently on each of the workstations on which it is displayed, using the capabilities of the workstations. GKS also incorporates the traditional method of individual specification of aspects in which each workstation does the best it can to represent global aspect values. In this paper a formal specification technique, the Vienna Development Method (VDM), is used to describe aspect specification. The GKS model of aspect specification is progressively constructed from simpler models. Properties of these simpler models are formulated and the specifications are proved to conform to these. The properties are then traced through the more complex models. The paper demonstrates the applicability of formal specification to the design of graphics software and the ability of formal techniques to catalyse the deeper understanding of designs.