bluuebunny/crossref_metadata_2025 huggingface dataset:
Modelado de hábitat potencial del Cuclillo pico amarillo Coccyzus americanus en Baja California Sur
Authors: García de la Puente Orozco José Emer , Albores Barajas Yuri Vladimir , Serrano Hernández David , Ramírez Pérez Jorge Saúl , Ojeda Ruiz Miguel Angel , Hernández Plata Exal , Montes Barrios Natalia
Published: February 2025

El Cuclillo pico amarillo (Coccyzus americanus) es un ave migratoria emblemática en la cultura del noroeste de México. Su población occidental se encuentra catalogada como amenazada a nivel internacional, debido a la disminución y fragmentación de su hábitat. Por ese motivo este estudio tiene como objetivo mejorar el conocimiento sobre la ecología y distribución de esta especie en la región de Los Cabos en el Estado de Baja California Sur. Para lograr este objetivo, se utilizó el programa MaxEnt incorporando variables ambientales como NDVI, temperatura, pendiente, uso del suelo y velocidad del viento, así como registros históricos de presencia de la especie y datos obtenidos en un monitoreo realizado en 2020 y 2021. El modelo generó dos mapas detallados: un mapa de distribución y un modelo de hábitat potencial de la especie, permitiendo identificar las zonas con mayor probabilidad de presencia. Estas áreas se concentran principalmente dentro de una de las Áreas Naturales Protegidas de la región, la Reserva de la Biosfera Sierra La Laguna, donde se identificaron áreas clave para su conservación que cuentan con hábitat ribereño con condiciones adecuadas para la reproducción y migración del Cuco. Este trabajo aporta información esencial para la conservación y manejo de los hábitats ribereños en la región, lo que podría contribuir a mitigar las amenazas que enfrenta esta especie.
Knowledge of Physical Medicine and Rehabilitation among Medical Graduates in Kolkata, India: A Cross-sectional Study
Authors: Sen Mausumi , Mukherjee Shuvankar , Majumdar Sukanta , Naskar Tanmoy , Mishra Swapan
Published: February 2025

Introduction: With the changing demographics and increasing burden of chronic diseases and their consequences, there is increasing need for rehabilitation care. But unfortunately there is lack of knowledge and awareness about Physical Medicine and Rehabilitation (PMR) among healthcare professionals including Indian Medical Graduates (IMG) and medical students.

Aim: To evaluate the basic knowledge and perception regarding PMR among IMGs at a Medical College in Kolkata, India.

Materials and Methods: This cross-sectional study was conducted among interns posted at the Department of PMR, Calcutta National Medical College, Kolkata, West Bengal, India from May 2023 to April 2024. Altogether 165 out of total 200 interns of the current batch filled up an anonymous, pretested, predesigned, structured questionnaire. Prior ethical clearance was obtained from Institutional Ethics Committee. The questionnaire had 20 items, of which 15 were about knowledge pertaining to different aspects of PMR and two questions on the participant’s opinion regarding inclusion of PMR in undergraduate medical curriculum and PMR as a good option for pursuing a postgraduate course in future. The remaining three items are on participant’s age, sex and sources of knowledge regarding PMR. Data were analysed using Statistical Package for the Social Sciences (SPSS) version 20.0. Chi-square test was used to compare between male and female participants regarding their opinion about inclusion of PMR in undergraduate medical curriculum and their willingness to pursue PMR as a postgraduate course in future, at 5% significance level.

Results: The study showed that 75.2% (124/165) of the participants had correct knowledge about the full form of “PMR”. But only 27.9% (46/165) could correctly identify “physiatrist” as the team leader of the rehabilitation team. Eighty three percent (137/165) of them were knowledgeable about the level of prevention, the term “rehabilitation” represented. However, only 30.3% (50/165) and 31.5% (52/165) knew the meaning of “disability and “handicap” respectively. It was observed that 34.5% (57/165) were aware about when to start rehabilitation for any clinical condition. About 33.9% (56/165) had the correct idea that PMR interventions can improve Quality of Life (QOL). Majority was found to be unsure about inclusion of PMR in undergraduate curriculum and whether to pursue PMR in post postgraduate course.

Conclusion: There was gross lack of knowledge regarding PMR among the participating interns.
The first university of Russia: its changes and continuity through the centuries
Authors: Andreev Andrey
Published: February 2025

In the year of the 270-th anniversary of Lomonosov Moscow State University, the article is an overview of its history with an emphasis on the changes that its organizational structure, composition of professors and students have experienced. The changing role of the university in Russian society and the state is shown, together with its overcoming of a number of difficulties in its history, culminating in the acquisition of the modern status of the largest domestic higher education institution.
Touch and see. Living matter in a nanomicroscope
Authors: Yaminsky Igor
Published: February 2025

In 1986, the Nobel Prize in Physics was awarded for the invention of the scanning tunneling microscope. Its subsequent modifications - atomic force, scanning capillary and other microscopes became the leading tools for studying various objects, including living matter at the level of atoms and molecules. Observations became possible both in air and in liquid media. The images obtained in the microscope became three-dimensional.
Suitability Assessment of SRTM and ASTER GDEM2 Digital Elevation Model for Terrain Analysis in Aba North: A Case Study of Abia State Polytechnic Aba
Authors: U.E Ahuchaogu, , U.U Duru , G.O Ikwuemesi, , A.C Okoroji, , O. Ugwu, , E.O Franklin,
Published: February 2025

Accurate geospatial data is fundernental to reliable decision making for sustainable physical development on earth surface. Open-source Digital Elevation Models (DEMs) are imperative to many earths’ surface process analysis therefore it is necessary to subject them to accuracy assessment to evaluate their suitability for different geographical applications. In this study, the elevation quality of DEMs acquired by Surface Radar Topographic Mission of 30m resolution (SRTM-30) and Advanced Spaceborne Thermal Emission Radiometer-Global Digital Elevation Model version2 (ASTER GDEM2) has been evaluated using ground control point captured with digital level and Differential Global Positioning System (DGPS). Two main approaches used to compare the elevation products are determination of the accuracy of the elevation values of the products using 96 ground elevation point (absolute accuracy) and determination of the accuracy of terrain derivatives of the products (relative accuracy). Statistics used to assess absolute accuracy are mean error, root mean square error, correlation coefficient, difference in maximum height, and difference in minimum height. The relative accuracies were assessed by considering similarities between terrain derivatives generated from the DEMS and their equivalence from ground reference.  Results showed that ASTER-GDEM2 has a lower Root Mean Square Error (RMSE = 3. 10m) than SRTM-30m (RMSE = 3.69 m). Further investigation in terms of relative accuracy also revealed that ASTER GDEM2 performed better than SRTM-30 for the study area. Both datasets featured a much better absolute vertical accuracies than the absolute vertical accuracies of 17m (ASTER GDEM2) and 16m (SRTM-30) published in the specification. Although the results of this study may be site-specific, it is an important information that users of the DEMs within Aba north should be aware of, and must be considered into decisions regarding practical applications of these Spatial products. It is also important that the results be considered for the improvement of the next open source GDEM version.
The Role of School Leadership on Implementing School Re-entry Policy for Adolescent Mothers in Tanzania: A Systematic Review
Authors: Manyengo Patrick Renatus
Published: February 2025

Aims: Adolescent motherhood continues to be a major impediment to girls' education in Tanzania, with thousands of them interrupting their schooling each year because of pregnancy.  This interruption often leads to permanent dropout, preventing these young women from completing their education and achieving their full potential. This study aimed to identify the role of school leadership practices that either support or impede the successful reintegration of adolescent mothers into the education system in Tanzania.
Study Design:  A systematic Review.
Duration of Study: The systematic review took place from 15th November 2024 to 2nd February 2025
Methodology: This systematic review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. English-language records were searched in Google Scholar using the phrase "school re-entry policy." Studies focusing on school re-entry policy were eligible for inclusion. A total of 66 records were retrieved, of which 18 met the inclusion criteria after full-text screening.
Results: The findings reveal that school leadership perspectives and practices encompass the provision of support and resources. However, they also highlight a lack of awareness regarding relevant policies and guidelines, negative attitudes towards the policy, and the absence of clear implementation guidelines.
Conclusion: The successful implementation of adolescent mother re-entry policies centers on key factors related to school leadership. School leaders must provide support and resources to foster a welcoming and encouraging environment for these students. Furthermore, awareness of the policy and positive attitudes among school heads, coupled with clear implementation guidelines, are crucial for effective and consistent re-entry programmes.
Competence and Academic Performance of Student Parents in Higher Education: A Descriptive-Correlational Study
Authors: Tabigne Paul Niño S.
Published: February 2025

Learning competence is the ability to effectively acquire and apply knowledge and skills. This study assessed the role of competence in pursuing higher education on the academic performance of student parents in selected state higher education institutions. A total of 101 student parents participated in the study, which employed a descriptive-correlational design and proportionate stratified random sampling. Data were analyzed using frequency, percentages, mean, standard deviation, T-test, one-way ANOVA, Duncan test for post hoc comparison, Pearson correlation, and multiple regression analysis. The results revealed that most student parents were aged 18-24, predominantly female, living with a partner, had a first child aged 0-4, had an income below the poverty threshold, and had parents who attended college without completing a degree. Respondents demonstrated a moderate level of competence but possessed a superior level of academic performance. Interestingly, a significant negative relationship was found between competence and academic performance. This counterintuitive finding suggests that academic performance in this context may rely more on adaptability, support systems, or external academic resources rather than solely on individual competence. The study recommends that policymakers, school administrators, and educators implement assessment systems, mentoring programs, and activities that bridge academic knowledge with practical applications to foster both competence and academic excellence.
Comparative Evaluation of Different Baffle Plates in Light Traps to Lure Major Phototactic Insect Pests of Rabi Season
Authors: Pravin  , Sharma Amit Kumar , Das S. B. , Kumar Neeraj , Singh Ravi
Published: February 2025

The present investigation was carried out at the BSP (Breeder Seed Production) Farm, Adhartal, JNKVV, Jabalpur (MP) during rabi (15th November 2023 to 15th April 2024) season of 2023-2024. Two light trap designs were used in study viz. Electrical light trap with acrylic transparent sheet baffle plates (T2) and Electrical light trap with galvanized iron sheet baffle plates (T3). Light traps were operated every evening and insect pest collection was observed every morning for the duration of the investigation for 12 species viz., Helicoverpa armigera, Agrotis ipsilon, Creatonotus gengis, Spodoptera litura, Gryllus bamaculatus, Gryllotalpa orientalis, Nezara viridula, Amata cyssea, Asota carica Perina nuda, Thysanoplusia orichalcea and Theretra oldenlandiae. Analysis of data revealed that electrical light trap with acrylic transparent baffle sheet plates outperformed the galvanized iron baffle sheet plates in terms of trapping efficacy for most of the pest species tested. The acrylic transparent plates with larger illumination area resulted in a higher capture rate, making them a superior design of light trap for insect collection.
Study of the Malformation of Ductal Plate of the Liver in Meckel Syndrome and Review of Other Syndromes Presenting with this Anomaly
Authors: Sergi Consolato , Adam Sven , Kahl Philip , Otto Herwart F.
Published: July 2002

Meckel syndrome (MIM 249.000) is an autosomal recessive disorder with a variable spectrum of anomalies. Since the first reports of this syndrome, very broad diagnostic criteria have been proposed, but the process of defining them continues. It is probable that at least two of three manifestations, including cystic kidney dysplasia, occipital encephalocele or other anomaly of the central nervous system, and postaxial polydactyly occur in most cases. Arrest of the development of intrahepatic bile ducts at the stage of the bilaminar plate formation or ductal plate malformation is considered of high diagnostic value in Meckel syndrome, but there is no complete agreement in the literature about its occurrence. The aims of this investigation were to study the prevalence and morphologic patterns of ductal plate malformation of the liver in Meckel syndrome by evaluating the dilatation of primitive biliary structures and the increase in connective tissue of the portal tract. Archival data files from four German centers (Berlin, Freiburg, Heidelberg, Mainz) were reviewed. Liver sections of 30 well-studied fetuses with Meckel syndrome were immunostained with antibodies against cytokeratins (intermediate filaments of the cytoskeleton) and factor VIII (an endothelial cell marker) and were evaluated both qualitatively and quantitatively. Cystic kidney dysplasia, occipital encephalocele, and postaxial polydactyly were found in 100%, 90%, and 83.3% of the fetuses, respectively. Ductal plate malformation of the liver was a constant anomaly in Meckel syndrome, seen as frequently as renal lesions. We observed essentially two kinds of hepatic lesions: 23 cases showed mainly a cystic dilatation of primitive biliary structures with little portal fibrosis, while 7 cases showed mainly rings of interrupted curved lumina around a central fibrovascular axis and pronounced portal fibrosis. In these seven cases an abnormal pattern of the portal vein, with many small and closely spaced branches of the portal vein (the so-called pollard willow pattern), was also seen. With respect to other fetal developmental anomalies, no difference between the two types of lesions was found. We also provide a potentially useful comprehensive review of other genetic syndromes in which ductal plate malformations may occur.
Sarcoid Reaction in Primary Neuroblastoma: Case Report
Authors: Hojo Hiroshi , Suzuki Sayuri , Kikuta Atushi , Ito Masaki , Abe Masafumi
Published: July 2002

We present a unique case of a 9-month-old infant with a left adrenal neuroblastoma with sarcoid reaction, detected by mass screening. There was no clinical evidence indicating systemic sarcoidosis or pulmonary mycobacterial infection. Histological examination of the resected adrenal tumor revealed many noncaseating epithelioid granulomas with lymphocytic infiltrate, composed of epithelioid cells and few giant cells, arising in tumor parenchyma and fibrovascular stroma. Most of the lymphocytes in the granulomas were CD3- or CD45RO-positive T cells, with fewer being CD20-positive B cells. The lymphocytes in the epithelioid granulomas expressed CD4 or CD8, but not CD56 and CD57. CD4-positive cells were observed more within the granulomas (internal area) than in the surrounding area (external area) of the same granulomas, while most of the CD8-positive cells were seen consistently at the outer margin of the granulomas (marginal zone). CD45RA-positive T cells were observed predominantly in the external area. The results of immunostaining demonstrated that lymphocytes in granulomas of this case showed the same distribution pattern as that seen in systemic sarcoidosis. Although the sarcoid reaction is a phenomenon known to be associated with the region of cancer, granuloma within the primary neuroblastoma is extremely rare. The sarcoid reaction in the present case of neuroblastoma may be associated with a delayed-type hypersensitivity reaction, and its significance and relevance still remain obscure.
Morbidity, Mortality, and Placental Pathology in Excessively Long Umbilical Cords: Retrospective Study
Authors: Baergen Rebecca N. , Malicki Denise , Behling Cynthia , Benirschke Kurt
Published: July 2002

The purpose of this study was to compare specific fetal, maternal, and placental factors, including neonatal morbidity and mortality, in infants with umbilical cords (UCs) of normal length to the same factors in infants with excessively long umbilical cords (ELUCs). We performed an 18-year retrospective chart review of the medical records of mothers and infants with ELUCs (926 cases) and normal-length UCs (200 cases) and recorded maternal factors, fetal factors, and neonatal outcomes. Corresponding placental pathologic reports and slides were reviewed. Statistical analysis comparing the two groups included univariate and multivariate analyses.

ELUCs were significantly associated with certain maternal factors (systemic diseases, delivery complications, increased maternal age), fetal factors (non-reassuring fetal status, respiratory distress, vertex presentation, cord entanglement, fetal anomalies, male sex, increased birth weight), gross placental features (increased placental weight, right-twisted cords, markedly twisted cords, true knots, congestion), and microscopic placental features (nucleated red blood cells, chorangiosis, vascular thrombi, vascular cushions, meconium, increased syncytial knots, single umbilical artery). Some of these histopathologic features have previously been associated with fetal hypoxia and/or altered blood flow in the placenta. Infants with ELUCs were found to be at a significantly increased risk of brain imaging abnormalities and/or abnormal neurological follow-up. In addition, mothers with a history of an ELUC are at increased risk of a second long cord.
Morphological and Phenotypic Features in Pediatric Large Cell Lymphoma and Their Correlation with ALK Expression and the t(2;5)(p23;q35) Translocation
Authors: Sherman C.G. , Zielenska M. , Lorenzana A.N. , Pulford K.A.F. , Mason D.Y. , Hutchison R.E. , Thorner P.S.
Published: July 2002

Anaplastic large cell lymphoma (ALCL) was proposed as a clinicopathologic entity over 14 years ago, but has been somewhat controversial due to the variability of its defining features and variable occurrence in different age-groups. To evaluate this entity in a pediatric population, 36 cases of childhood large cell lymphoma were evaluated for abnormalities of the anaplastic lymphoma kinase (ALK) gene that has been associated with ALCL morphology and immunophenotype. ALK abnormalities were evaluated by assay for the t(2;5)(p23;q35) translocation by RT-PCR and/or expression of NPM-ALK fusion protein by immunohistochemistry. Results showed 17 patients to have evidence of ALK gene expression. All of these children (mean age, 9.3 years) had tumors that were of T-cell phenotype (with the exception of a single case of null phenotype) and that expressed CD30. In contrast, 19 children with no evidence of ALK expression were older (mean, 12.7 years), and the majority (12/19) had tumors of B-cell phenotype. CD30 was also diffusely expressed in 8 of these 19 tumors. The difference in mean age between the two groups was statistically significant ( P = 0.015). In three cases tested for both ALK and the t(2;5), ALK protein was detected in the absence of the t(2;5) translocation but no cases showed the reverse pattern, consistent with ALK fusion to genes other than NPM or activation of the ALK gene by another mechanism. These findings provide further support that ALK-positive ALCL is a distinct pathologic entity among pediatric large cell lymphomas primarily characterized by expression of T-cell markers, CD30, and EMA, and by a younger mean age.
Primitive Neuroectodermal Tumors of the Biliary and Gastrointestinal Tracts: Clinicopathologic and Molecular Diagnostic Study of Two Cases
Authors: Sarangarajan Ranganathan , Hill D. Ashley , Humphrey Peter A. , Hitchcock Michael G. , Dehner Louis P. , Pfeifer John D.
Published: July 2002

Primitive neuroectodermal tumor (PNET) is a prototypic malignant small round cell tumor of childhood that is characterized in most cases by t(11;22) resulting in an EWS-FLI1 gene fusion. Once thought to be uncommon, PNET now accounts for almost 20% of malignant soft tissue tumors in children. Increased recognition of PNET is partly due to advances in immunohistochemistry and molecular diagnostics, which have led to the identification of the tumor in non-classical sites. We report the clinical, histologic, immunohistochemical, and molecular findings of two visceral PNETs of the digestive system—one involving the small intestine and the other involving the hepatic duct. Histologically, each tumor was composed of malignant small cells growing in sheets, nests, and lobules; the tumor cells of both cases showed characteristic immunoreactivity for vimentin and O13 (CD99). Reverse transcription-polymerase chain reaction (RT-PCR) analysis for t(11;22) using nested primers was performed with RNA extracted from paraffin-embedded, formalin-fixed tissue and demonstrated an EWS exon 7 to FLI1 exon 5 fusion in both cases, confirmed by Southern blot hybridization and DNA sequence analysis. These results illustrate the expanded clinicopathologic profile of PNET, and demonstrate that visceral PNETs, despite their unusual sites of presentation, maintain the characteristic immunohistochemical and genetic features of PNETs at more conventional sites.
Prosopo-Thoracopagus Conjoined Twins and Other Cephalopagus-Thoracopagus Intermediates: Case Report and Review of the Literature
Authors: Spencer Rowena , Robichaux William H.
Published: July 2002

Prosopo-thoracopagus twins are united from the face down to the umbilicus, none with union in the brain but all with visceral anomalies intermediate between those of cephalopagus and thoracopagus. In a review of over 1200 cases of conjoined twins reported during the past 100 years, there were 14 that illustrate the continuum between cephalopagus and thoracopagus, including three that were united only from the cervical region to the umbilicus. Classic cephalopagus twins are joined from the top of the head to the umbilicus, sharing a single foregut as well as two relatively normal hearts, the “posterior” one often diminished. Typical thoracopagus, however, are conjoined only from the upper thorax to the umbilicus, each twin with a normal foregut but both sharing a single complex multiventricular heart. The intermediate cases shared either a single very abnormal heart or two hearts united by double aortic arches, and all except one had a single foregut. It is these cases intermediate between cephalopagus and thoracopagus which are the subject of this report.
Adrenal Fusion
Authors: Klatt Edward C. , Pysher Theodore J. , Pavlova Zdena
Published: July 2002

Fusion of the adrenal glands is a rare congenital anomaly. The six cases described here were encountered in 3537 pediatric-perinatal autopsy cases. A fused adrenal was always associated with multiple congenital anomalies, including major central nervous system malformations in four cases, renal agenesis in three cases, anomalies of internal genitalia in three cases, and complex cardiac anomalies in two cases. The fused adrenal had either a horseshoe or butterfly shape. Neither adrenal hypoplasia nor hyperplasia appeared to be present, and the histologic appearance of the fused adrenal was normal in all cases.
Association of Single Umbilical Artery with Congenital Malformations of Vascular Etiology
Authors: Pavlopoulos Petros M. , Konstantinidou Anastasia E. , Agapitos Emmanuel , Christodoulou Constantinos N. , Davaris Panagiotis
Published: July 2002

The possible association of a single umbilical artery (SUA) with malformations of vascular etiology is investigated in this study. Four hundred twelve fetal and embryonic autopsies showing one or more congenital malformations, collected over 7 years, were reviewed. Microscopic confirmation of a SUA was evident in 20 cases (4.85%). The two subgroups with 2 ( nA = 20) or 3 umbilical vessels ( nB = 392) were compared with each other, in relation to the frequency of malformations per organ system. In the group of fetuses with congenital malformations, no association was observed between SUA and the incidence of CNS, cardiac, pulmonary, or genital malformations. However, there was a significantly higher incidence of atresia of hollow organs ( P = 0.003), renal aplasia ( P = 0.034), and limb reduction defects (LRD) ( P = 0.0383) when only a single umbilical artery was present. This suggests a possible etiopathogenetic association of SUA with congenital malformations of vascular etiology. Furthermore, the findings of our study suggest that prenatal identification of a SUA warrants a thorough search for atresias, renal aplasia, and LRD-type malformations.
Diffuse Leiomyomatosis of the Esophagus: Disorder of Cell-Matrix Interaction?
Authors: Thorner Paul , Heidet Laurence , Merlo Fernando Moreno , Edwards Vern , Antignac Corinne , Gubler Marie-Claire
Published: July 2002

Diffuse leiomyomatosis (DL) is rare condition characterized by proliferation of smooth muscle in the upper gastrointestinal tract. Most cases are associated with X-linked Alport syndrome and have partial deletions in the genes encoding both the α5 and α6 chains of collagen type IV. We studied aspects of cell-matrix interaction of myocytes in an esophagogastrectomy specimen from a 12-year-old patient with DL. Myocytes had central areas of cytoplasmic rarefaction, which were actin positive and desmin poor, with the reverse pattern of staining at the cell periphery. Electron microscopy (EM) showed that the areas of rarefaction consisted of disorganized aggregates of filaments. The basement membranes ranged from thickened to thinned or absent. Immunohistochemical staining for the α1–α4 chains of collagen type IV, the α1, α2, β2, and γ1 chains of laminin, nidogen, type VI collagen, and fibronectin was normal. There was loss of the α5 and α6 chains of collagen type IV and the β1 chain of laminin. Normal staining for α1, α2, α3, α4, α6, α8, and β1 integrins was noted. Staining for α5 integrin varied from normal to reduced or negative in different cells. In DL, a primary abnormality of basement membrane may be associated with disorganization of the contractile apparatus and alterations of certain integrins. This may reflect a disturbance of cell-matrix interactions that play a role in cell differentiation and internal organization.
Immunological Studies of the Placenta in Maternal Connective Tissue Disease
Authors: Ackerman Jeanne , Gonzalez Erick F. , Gilbert-Barness Enid
Published: July 2002

Maternal connective tissue disease is an important cause of second-trimester fetal loss. In order to assess the pathological changes in the placenta in maternal connective tissue disease, we reviewed the clinical histories and performed histologic and immunofluorescence studies on nine placentas: five from mothers with systemic lupus erythematosus (SLE), two from mothers with mixed connective tissue disease (MCTD), one from a mother with rheumatoid arthritis (RA), and one from a mother without prior known connective tissue disease. Excessive intervillous fibrin deposition and infarction were noted in all cases. Immunofluorescent and immunoperoxidase studies showed deposits of fibrinogen, IgG, IgM, IgA, and complement 3 (C3) localized to the trophoblast basement membrane (TBM). Electron microscopy documented thickening of the trophoblast basal lamina in three SLE placentas examined. The use of immunofluorescence may be enhanced further if antitrophoblast antibodies can be linked to placental compromise.
Multiple Vascular and Bowel Ruptures in an Adolescent Male with Sporadic Ehlers-Danlos Syndrome Type IV
Authors: Collins Margaret H. , Schwarze Ulrike , Carpentieri David F. , Kaplan Paige , Nathanson Katherine , Meyer James S. , Byers Peter H.
Published: July 2002

Ehlers-Danlos syndrome (EDS) type IV is a heritable disorder resulting from mutations in the COL3A1 gene that cause deficient production of type III collagen. Clinical manifestations of EDS type IV include hypermobility of small joints, excessive bruisability, thin translucent skin, poor wound healing, bowel rupture, and vascular rupture that is often fatal. A 14-year-old male without a family history of EDS died following multiple bowel and abdominal blood vessel ruptures. Even in areas apart from rupture sites, the bowel wall was thin because of diminished submucosa and muscularis propria. Similarly, the walls of blood vessels in bowel submucosa and elsewhere in the abdomen varied in thickness, and contained frayed and fragmented elastic tissue fibers. Fibroblasts cultured from the patient's skin secreted reduced quantities of type III collagen that was explained by a point mutation in one copy of the COL3A1 gene. EDS type IV should be strongly suspected in any patient with otherwise unexplainable bowel and/or vessel rupture.
Ivemark's Renal-Hepatic-Pancreatic Dysplasia: Analytic Approach to a Perinatal Autopsy
Authors: Bendon Robert W.
Published: July 2002

A case of Ivemark's renal-hepatic dysplasia syndrome is presented. The findings are suggestive of a ductal obstructive process anatomically located at the junction of ducts with the parenchyma. The differential diagnosis includes Meckel syndrome, short rib polydactyly syndromes, and glutaric aciduria type 2. The molecular basis for this syndrome remains unknown.
Immunohistochemical Localization of Erythropoietin and Its Receptor in the Developing Human Brain
Authors: Juul Sandra E. , Yachnis Anthony T. , Rojiani Amyn M. , Christensen Robert D.
Published: July 2002

We have previously shown erythropoietin (Epo) and its receptor (Epo-R) to be present in the fetal human central nervous system (CNS), and Epo to be present in the spinal fluid of normal preterm and term infants. To investigate the cellular specificities and developmental patterns of expression of these polypeptides in the human brain—areas that have not been well researched—we designed the following study. Human brains ranging in maturity from 5 weeks post-conception to adult were preserved at the time of elective abortion, surgical removal (tubal pregnancy, or removal for temporal lobe epilepsy), or autopsy. Immunohistochemistry was used to localize Epo and Epo-R reactivity in brains of different stages of development. Astrocytes, neurons, and microglia were identified in sequential tissue sections by specific antibodies. At 5 to 6 weeks post-conception, both Epo and Epo-R localized to cells in the periventricular germinal zone. At 10 weeks post-conception, Epo immunoreactivity was present throughout the cortical wall, with the most intense immunoreactivity present in the ventricular and subventricular zones. Epo-R, in contrast, was localized primarily to the subventricular zone, with little staining evident in the ventricular zone. In late fetal brains, Epo-R reactivity was most prominent in astrocytic cells, although modest reactivity was observed in certain neuron populations. In contrast, Epo staining localized primarily to neurons in fetal brains, although a subpopulation of astrocytes was also immunoreactive. In postnatal brains, both astrocyte and neuron populations were immunoreactive with antibodies to Epo-R and Epo. From these results it is clear that Epo and its receptor are present in the developing human brain as early as 5 weeks post-conception, and each protein shows a specific distribution that changes with development. We speculate that Epo is important in neurodevelopment, and that it also plays a role in brain homeostasis later in life, functioning in an autocrine or paracrine manner.
Cerebral Mantle Thickness: A Measurement Useful in Anatomic Diagnosis of Fetal Ventriculomegaly
Authors: Siebert Joseph R. , Nyberg David A. , Kapur Raj P.
Published: July 2002

The ultrasonographic diagnosis of cerebral ventriculomegaly carries grave implications, in that affected fetuses may suffer abnormal postnatal development or therapeutic abortion. It is important for pathologists to corroborate the clinical diagnosis, but because diagnostic methodologies and criteria differ so radically, this can be problematic. The clinical diagnosis is made primarily by serial ultrasound examinations of the cerebral ventricles, spaces that can be altered postmortem, particularly when the brain is autolysed or deformed artifactually We therefore sought to learn if examination of tissue, rather than spaces, can identify accurately those fetuses diagnosed with cerebral ventriculomegaly by prenatal ultrasound. The thickness of the cerebral mantle was obtained in 100 control fetuses aged 14 to 26 postmenstrual weeks. Statistical analysis revealed significant correlation of cerebral mantle thickness with crown–rump length, foot length, and head circumference. Twenty fetuses diagnosed with ventriculomegaly showed mantle thicknesses that were less than the control mean. In a few cases, mantle thickness fell between the mean and —1 SD; in several others, thickness was diminished by — 1 SD to —2 SD; in one-half of cases, mantle thickness was 2 SDs or more below the expected mean. Head circumference was within 2 SDs of the control mean in most cases, and increased beyond 2 SDs in only two cases. Head circumference is an unreliable indicator of ventriculomegaly in the midgestational fetus. By contrast, cerebral mantle thickness is a simple and useful way of corroborating ultrasonographic diagnoses at autopsy and may also prove useful in clinical settings.
Seckel-like Syndrome in Three Siblings
Authors: Arnold Sonya Rae , Spicer Diane , Kouseff Boris , Lacson Atilano , Gilbert-Barness Enid
Published: July 2002

Seckel syndrome has been described as the prototype of the primordial bird-headed type of dwarfism. Since Seckel originally defined the disorder, less than 60 cases have been reported. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine, and central nervous systems. This pleiotropy has implied genetic heterogeneity and prompted reviews of previously reported cases of Seckel syndrome. As a result, the characteristic diagnostic features of Seckel syndrome have been highly debated. Although deletions in chromosome 2q have been described, to date, no genetic defect has been defined. We report three cases of Seckel-like syndrome in siblings from nonconsanguinous Caucasian parents. In addition to the typical Seckel phenotypic features, all three cases were characterized by severe hydrocephalus. We review the literature and propose that there is a spectrum of Seckel conditions that share some common key features, but also demonstrate a wide range of phenotypic features.
Hepatic Subcapsular Hematomas in Fetuses and Neonatal Infants
Authors: Singer Don B. , Neave Charles , Oyer Calvin E. , Pinar Halit
Published: July 2002

In fetuses and neonates hepatic subcapsular hematomas are relatively common lesions and may be life-threatening. Conditions previously associated with these hematomas include trauma, coagulopathies, hypoxia, sepsis, pneumothorax, maternal diseases, and placental lesions. In this study of 755 perinatal autopsies, hepatic subcapsular hematomas were found in 52 (6.9%) cases, including 31 stillborn fetuses and 21 liveborn infants. The average body weight was 690 g. A comparison group consisted of 52 temporally proximal autopsies of fetuses and neonates without hematomas. Body weights, gender, maternal age, and stillbirth or postnatal survival were matched as closely as possible while evaluating the presence or absence of sepsis, pneumothorax, cerebral germinal matrix hemorrhage, trauma, coagulopathy, placental lesions, and maternal diseases.

Sepsis was associated with 62% of the cases with hepatic subcapsular hematomas and with 25% of the comparison group ( P = .0001). Group B streptococcus infection was the most common cause of sepsis, but many different organisms were isolated. Cerebral germinal matrix hemorrhages were present in 35% of the cases with hematomas and in 14% of the comparison group ( P = .0001). No other lesions or conditions were statistically different in the study group versus the comparison group. The delicacy of the hepatic capsule and its connections to the collagen along the sinusoids provide insight for the pathogenesis of hematomas in premature fetuses and neonates. We conclude that sepsis is present in most perinatal cases of hepatic subcapsular hematomas and that such patients also frequently have cerebral germinal matrix hemorrhages. Each of these lesions is a greater hazard among very small premature fetuses or neonates than among older fetuses and neonates.
Prenatal Diagnosis of Oculocutaneous Albinism Type I: Review and Personal Experience
Authors: Rosenmann Eliezer , Rosenmann Ada , Ne'eman Zvi , Lewin Aby , Bejarano-Achache Idit , Blumenfeld Anat
Published: July 2002

Oculocutaneous albinism type I (OCA I) comprises autosomal recessive syndromes of hypopigmentation and low vision, caused by the lack of tyrosinase activity. Affected families seek genetic counseling and prenatal diagnosis as preventive measures. Until recently, prenatal diagnosis of OCA I was achieved by histologic and electron microscopic examination of fetal skin biopsies. Lately, a molecular genetic approach has become possible by the identification of the two mutated copies of the TYR gene, coding the tyrosinase, in which over 60 mutations have been identified.

We report here our experience in prenatal diagnosis of OCA I using the two strategies. Thirty-four prenatal tests were performed in fetuses at risk for OCA I. In 31 cases the diagnosis was made in fetal scalp biopsies using the histological approach. The microscopic observations revealed normal melanogenesis in 26 biopsies. Five albino fetuses were diagnosed by the demonstration of arrest of melanogenesis in early stages I and II. In three pregnancies, molecular genetic tests were performed on DNA extracted from amniocytes, using direct mutation analysis (in one), and complemented by linkage analysis (in two). One albino and two normally pigmented fetuses were diagnosed.

The prenatal molecular genetic test can be applied to families when at least one mutation is diagnosed in the albino patient. The histological approach is applicable in all families at risk for OCA I.
Ewing's Family of Tumors Involving Structures Related to the Central Nervous System: A Review
Authors: Hadfield M. Gary , Quezado Martha M. , Williams Robert L. , Luo Vivian Y.
Published: July 2002

This review consolidates information gleaned from several case reports and larger series on Ewing's sarcoma family of tumors (EFT) involving structures related to and found in the central nervous system (CNS). These tumors involve the skull, the spinal column, adjacent soft tissues, the meninges, and the brain. We have separated the cases by skull region and spinal column level, and we discuss the attendant differences in prognosis following treatment by neurosurgery, radiation, and chemotherapy. Light and electron microscopic features can be used to differentiate EFT from other small round blue cell tumors that involve the CNS (central primitive neuroectodermal tumor, lymphoma, etc.). Recent molecular and genetic findings in EFT provide new diagnostic methods. We conclude that EFT involving the CNS and adjacent structures is not so rare as previously stated and that the prognosis is more favorable, as a rule, than for the more common examples arising in the long bones and pelvis.
Clinicopathologic Study of Ectomesenchymomas from Intergroup Rhabdomyosarcoma Study Groups III and IV
Authors: Boué Daniel R. , Parham David M. , Webber Bruce , Crist William M. , Qualman Stephen J. ,   from the Intergroup Rhabdomyosarcoma Study Group (IRSG) representing the Children's Cancer Group, the Pediatric Oncology Group, and the Intergroup Rhabdomyosarcoma Statistical Office, Anderson James R. , Breitfeld Richard J. , Breneman John C. , Bridge Julia , Brown Kenneth , Crist William M. , Donaldson Sarah S. , Grier Holcombe E. , Hawkins Douglas , Houghton Peter J. , Link Michael , Lobe Thom L. , Maurer Harold M. , Meyer William H. , Michalski Jeff , Murphy Sharon , Paidas Charles N. , Pappo Alberto S. , Parham David M. , Qualman Stephen J. , Raney R. Beverly , Robison Leslie , Sandler Eric , Skapek Stephen , Smith Lynn , Sorensen Poul H.B. , Spunt Sheri , Teot Lisa , Triche Timothy , Vietti Teresa J. , Walterhouse David , Wharam Mondy , Wiener Eugene S. , Wolden Suzanne , Womer Richard
Published: July 2002

Ectomesenchymomas (EM) are rare malignant neoplasms usually consisting of rhabdomyosarcoma (RMS) with a neural component. Only 21 cases have been previously reported. Here we extend the clinicopathologic spectrum of EM by describing our findings in 15 cases. Only 5 patients were infants; 10 were ≤3 years old and 5 were ≥6 years old. No male predilection was observed; 7 were female. The originating institutional diagnoses were; RMS (12), undifferentiated sarcoma (1), or EM (2), suggesting underdiagnosis of this entity. The primary tumor sites included external genital (5), pelvis/abdomen (6), head and neck (3), and extremity (1). The size of the primary neoplasm was usually ≥5 cm at diagnosis but dissemination only occurred in a minority. Local infiltration was not uncommon. These neoplasms were typically multilobate, thinly encapsulated, hemorrhagic, and necrotic. Light microscopic features were highly variable, but embryonal RMS with scattered or clustered ganglion cells, often in lacunae, was characteristic. In some cases, primitive neuroblastic or neuroectodermal areas were found and/or a component of alveolar RMS was seen. Focal anaplasia was occasionally observed. Mitotic activity appears higher than previously appreciated and some necrosis was invariably present. Electron microscopy was performed in 11 cases, which confirmed skeletal muscle ± neural differentiation. Cytogenetic studies performed in five cases revealed no specific abnormality. Monoclonal neuron-specific enolase was the best marker of ganglion cells and primitive neural elements. MIC-2 (CD99) membrane expression was not definitively present in any of the six cases examined. A number of the above parameters appear to be of some prognostic significance, but overall, these neoplasms appear to have a similar outcome as would be predicted for their RMS element alone (exclusive of any neural component), with respect to the RMS subtype, age of the patient, and anatomic location of the neoplasm.
Quenching of singlet oxygen luminescence by fatty acids and lipids
Authors: Krasnovsky A.A. , Kagan V.E. , Minin A.A.
Published: July 2002

By the use of photosensitized luminescence of singlet oxygen (1O2) in CCl4, the rate constants for quenching 1O2 by saturated and unsaturated fatty acids were determined. The experimental data suggest that saturated fatty acids quench 1O2 via a physical mechanism, presumably as a result of energy transfer to the vibrational sublevels of CH‐ and COOH‐groups. Unsaturated fatty acids are predominantly chemical quenchers. The relative contribution of the physical quenching depends on the number of double bonds in fatty acid molecules. It was found that the quenching activity of egg phosphatidylcholine is approximately equal to the sum of quenching activities of the lipid fatty acids. The data obtained may be used for prediction of the efficiency of singlet oxygen quenching by any lipids whose fatty acid composition is known.
The effect of aglycosylation on the binding of mouse IgG to staphylococcal protein A
Authors: Leatherbarrow Robin J. , Dwek Raymond A.
Published: July 2002

Aglycosylated IgG produced by hybridoma cells cultured in the presence of tunicamycin was compared with normal IgG for its ability to bind to staphylococcal protein A. No differences were found in binding or elution profiles. It is concluded that aglycosylation does not produce major structural alterations at the CH2–CH3 interface of the Fc region of IgG.
Collagen of fibrocartilage: a distinctive molecular phenotype in bovine meniscus
Authors: Eyre David R. , Wu J.J.
Published: July 2002

Fibrocartilage of bovine knee meniscus was analyzed for major and minor collagen constituents. The main fraction (∼98%) of pepsin‐solubilized collagen consisted of type I with a small proportion (< 10%) of type III molecules. The minor fraction (1–2%) isolated by salt precipitation could be further resolved into type V collagen that consisted of α1(V) and α2(V) chains and a type II‐like molecule with chains that had all the characteristics of the 3α variant of α1(II) found in hyaline cartilage. The articular surface zone of the meniscus appeared 2–3‐fold enriched in these minor collagens compared with deeper tissue, though qualitatively the same distinctive collagen phenotype was evident throughout.
Superoxide‐dependent formation of hydroxyl radicals in the presence of iron salts
Authors: Flitter W. , Rowley D.A. , Halliwell B.
Published: July 2002

In the presence of physiological concentrations of iron(III) salts, superoxide radical and hydrogen peroxide can interact to form the highly‐damaging hydroxyl radical. No specific added ‘chelator’ of iron salts is necessary for this reaction to occur.
Amino acid sequence of the chymotryptic protease II from the larvae of the hornet, Vespa crabro
Authors: Jany Klaus-Dieter , Haug Harald
Published: July 2002

The covalent structure of the chymotrypsin II from the larvae of the hornet, Vespa crabro, has been determined. The sequence has been deduced from 3 sets of overlapping peptides generated by trypsin after modification of the lysine or arginine residues and by chymotrypsin. The enzyme is a serine endopeptidase and contains 218 residues in a single polypeptide chain cross‐linked by 3 disulfide bonds. Alignment of the sequence of this insect protease with those of chymotrypsin, elastase and trypsin shows about 35% identity with each and a homologous relationship is evident.
6‐Phosphogluconolactonase from Zymomonas mobilis
Authors: Scopes R.K.
Published: July 2002

The enzyme 6‐phosphogluconolactonase (EC 3.1.1.31) is present at high levels in Zymomonas mobilis cells. A simple procedure for its isolation involving dye‐ligand chromatography and gel filtration has resulted in a 500‐fold purification with high recovery. The purified enzyme is a monomer of 26 kDa, and has a high catalytic efficiency with of 9 × 107 M−1 s−1 at 25° C. Two assay procedures for the enzyme are compared, and a simple method of obtaining a solution of 6‐phosphoglucono‐δ‐lactone relatively free of other metabolites is presented.
Identification of the stable free radical tyrosine residue in ribonucleotide reductase
Authors: Sjöberg B.-M. , Eklund H. , Fuchs J.A. , Carlson J. , Standart N.M. , Ruderman J.V. , Bray S.J. , Hunt T.
Published: July 2002

Ribonucleotide reductase
Secondary structure prediction
Sequence comparison
Tyrosine radical