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  • 1
    Online Resource
    Online Resource
    SAGE Publications ; 2003
    In:  Pediatric and Developmental Pathology Vol. 6, No. 4 ( 2003-07), p. 284-298
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. 6, No. 4 ( 2003-07), p. 284-298
    Abstract: Recent work is providing new insights into molecular mechanisms of digestive system development and their alteration in clinically significant disorders. An understanding of these mechanisms has largely been gained through the use of animal models, because many of the basic processes required in embryogenesis are functionally conserved among species. Such conserved factors include cell–cell signaling pathways and the regulation of gene expression. Disruption of these pathways have been implicated in several congenital disorders of the digestive system, including Hirschsprung disease, malrotation, altered sphincter development, Meckel diverticulum, biliary atresia, Alagille syndrome, pancreatic heterotopias, and pancreatic agenesis. In this review, we highlight recent studies in digestive system development, which elucidate mechanisms underlying congenital disorders of the human digestive system.
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2003
    detail.hit.zdb_id: 1480654-X
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  • 2
    Online Resource
    Online Resource
    SAGE Publications ; 2006
    In:  Pediatric and Developmental Pathology Vol. preprint, No. 2007 ( 2006), p. 1-
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. preprint, No. 2007 ( 2006), p. 1-
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2006
    detail.hit.zdb_id: 1480654-X
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  • 3
    Online Resource
    Online Resource
    SAGE Publications ; 2009
    In:  Pediatric and Developmental Pathology Vol. 12, No. 6 ( 2009-12), p. 475-480
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. 12, No. 6 ( 2009-12), p. 475-480
    Abstract: Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress. Despite its dramatic clinical presentation, the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of comorbid conditions. In this report, we describe changes in successive lung biopsies in a neonate who presented with respiratory failure and pulmonary hypertension. Diagnostic lung biopsy at 10 days of age exhibited classic histologic and ultrastructural findings of PIG with diffuse expansion of the alveolar interstitium by glycogenated mesenchymal cells. Subsequent to the patient's clinical improvement, a repeat biopsy at 49 days of age showed significant resolution of the disorder. Colocalization of vimentin-immunopositive cells with both phospho-histone H3 and cleaved caspase-3 demonstrated prominent attenuation of mesenchymal cell proliferation and apoptosis in the second biopsy. Although the self-limited nature of PIG has been described clinically, it has never been documented histologically. We present this case to illustrate the clinical and pathologic resolution of the disorder and speculate that the lesional mesenchymal cells may have transient proliferative capacity.
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2009
    detail.hit.zdb_id: 1480654-X
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  • 4
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. 9, No. 3 ( 2006-05), p. 173-180
    Abstract: This is the first of a series on pediatric pulmonary disease that will appear as Perspectives in Pediatric Pathology over the coming months. The series will include practical issues, such as this protocol for handling lung biopsies and another on bronchoalveolar lavage in childhood, as well as reviews of advances in various areas in pediatric pulmonary pathology. It has been 11 years since the last Perspectives on pulmonary disease. Much has happened since then in this area, and this collection will highlight some emerging and rapidly advancing areas in pediatric lung disease. These will include a review of molecular mechanisms of lung development, and another of mechanisms of pulmonary vascular development. The surfactant system and its disorders, as well as recent advances in the biology of the pulmonary neuroendocrine system and mechanisms of respiratory viral disease, will be addressed. Articles on pulmonary hypertension, pulmonary neoplasia, and pediatric lung transplantation, with their implications for the pediatric pathologist, are also planned. The contributors to this series are a diverse group with special interests and expertise in these areas. As Dr. William Thurlbeck noted in his foreword to the previous volume, Pulmonary Disease, volume 18 of Perspectives in Pediatric Pathology, pediatric pathology had been largely concerned with phenomenology, rather than with mechanisms, model systems, and experimental investigation. I think he would have been pleased to see the changes that have occurred over the past 10 years in pediatric lung biology and pathology in particular, because these were particularly favored interests of his later years.
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2006
    detail.hit.zdb_id: 1480654-X
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  • 5
    Online Resource
    Online Resource
    SAGE Publications ; 2006
    In:  Pediatric and Developmental Pathology Vol. preprint, No. 2008 ( 2006), p. 1-
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. preprint, No. 2008 ( 2006), p. 1-
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2006
    detail.hit.zdb_id: 1480654-X
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  • 6
    Online Resource
    Online Resource
    SAGE Publications ; 2007
    In:  Pediatric and Developmental Pathology Vol. 10, No. 5 ( 2007-09), p. 335-347
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. 10, No. 5 ( 2007-09), p. 335-347
    Abstract: Lung morphogenesis requires the integration of multiple regulatory factors, which results in a functional air-blood interface required for gas exchange at birth. The respiratory tract is composed of endodermally derived epithelium surrounded by cells of mesodermal origin. Inductive signaling between these 2 tissue compartments plays a critical role in formation and differentiation of the lung, which is mediated by evolutionarily conserved signaling families used reiteratively during lung formation, including the fibroblast growth factor, hedgehog, retinoic acid, bone morphogenetic protein, and Wnt signaling pathways. Cells coordinate their response to these signaling proteins largely through transcription factors, which determine respiratory cell fate and pattern formation via the activation and repression of downstream target genes. Gain- and loss-of-function studies in null mutant and transgenic mice models have greatly facilitated the identification and hierarchical classification of these molecular programs. In this review, we highlight select molecular events that drive key phases of pulmonary development, including specification of a lung cell fate, primary lung bud formation, tracheoesophageal septation, branching morphogenesis, and proximal-distal epithelial patterning. Understanding the genetic pathways that regulate respiratory tract development is essential to provide insight into the pathogenesis of congenital anomalies and to develop innovative strategies to treat inherited and acquired lung disease.
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2007
    detail.hit.zdb_id: 1480654-X
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  • 7
    Online Resource
    Online Resource
    SAGE Publications ; 2008
    In:  Pediatric and Developmental Pathology Vol. 11, No. 3 ( 2008-05), p. 169-177
    In: Pediatric and Developmental Pathology, SAGE Publications, Vol. 11, No. 3 ( 2008-05), p. 169-177
    Abstract: This review of pediatric pulmonary arterial hypertension provides a framework within which to view pulmonary hypertension in children. Classification schemes, including the latest recommendations from the World Health Organization, are discussed, and the histopathology of severe pulmonary hypertension is reviewed. New information is provided regarding idiopathic and familial forms of the disease. Specific childhood etiologies, including persistent pulmonary hypertension of the newborn and congenital heart disease, are reviewed. Additionally, we examine the role of collagen vascular diseases, portal hypertension, and viruses in the pathogenesis of severe pulmonary arterial hypertension.
    Type of Medium: Online Resource
    ISSN: 1093-5266 , 1615-5742
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2008
    detail.hit.zdb_id: 1480654-X
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