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2.
Am J Pediatr Hematol Oncol ; 13(1): 77-83, 1991.
Article in English | MEDLINE | ID: mdl-2029083

ABSTRACT

Thrombocytopenia and absent radii (TAR) syndrome is a congenital defect with osseous abnormalities and thrombocytopenia. It is inherited as an autosomal recessive trait, but the mechanism of thrombocytopenia in this disorder is not clear. We have had the opportunity to study the mechanism of thrombocytopenia in an infant with TAR syndrome. The infant had normal levels of thrombopoietin and megakaryocyte colony-stimulating activity in spite of marked thrombocytopenia. However, the megakaryocyte progenitor cells in the bone marrow produced abnormal colonies with increased numbers of megakaryocytes per colony and small megakaryocytes similar to the small megakaryocyte seen in vivo. These findings suggest that the TAR syndrome in this infant is due to a failure in the production of thrombopoietin or to an abnormal progenitor cell with a maturational defect.


Subject(s)
Blood Platelets/cytology , Hematopoiesis/physiology , Megakaryocytes/cytology , Radius/abnormalities , Thrombocytopenia/physiopathology , Animals , Child, Preschool , Colony-Forming Units Assay , Colony-Stimulating Factors/analysis , Female , Humans , Male , Mice , Mice, Inbred C3H , Platelet Count , Ploidies , Syndrome , Thrombopoietin/analysis
5.
Am J Clin Pathol ; 81(3): 388-9, 1984 Mar.
Article in English | MEDLINE | ID: mdl-6702733

ABSTRACT

A woman with premature rupture of membranes and chorioamnionitis gave birth to a 0.73-kg infant at 28 weeks' gestation. The infant died of fulminant septicemia caused by Hemophilus parainfluenzae. This organism should be recognized as a potential cause of chorioamnionitis and neonatal septicemia.


Subject(s)
Haemophilus Infections , Infant, Premature, Diseases/microbiology , Sepsis/etiology , Female , Fetal Membranes, Premature Rupture/complications , Haemophilus/isolation & purification , Humans , Infant, Newborn , Male , Maternal-Fetal Exchange , Pregnancy , Sepsis/microbiology
6.
Arch Dermatol ; 116(9): 1054-8, 1980 Sep.
Article in English | MEDLINE | ID: mdl-7416760

ABSTRACT

In 1967, Mehregan et al described a new and distinct clinicopathologic entity that they called reactive perforating collagenosis (RPC). This rare disease is classified with a group of disorders that involve the transepithelial elimination of histochemically altered dermal tissue. In RPC, minor trauma such as an insect bite, scratch, or pilosebaceous infection alters the collagen fibers in the papillary dermis. The histochemically altered but ultrastructurally intact collagen elicits both a dermal and epithelial response. For the case presented, it is our purpose to demonstrate transepithelial elimination of collagen fibers by ultrastructural study, thereby supporting the recognized histochemical observations.


Subject(s)
Collagen Diseases/pathology , Skin/ultrastructure , Adult , Biological Transport , Bites and Stings/complications , Collagen/analysis , Collagen Diseases/etiology , Epidermis/pathology , Epidermis/ultrastructure , Epithelium/pathology , Epithelium/ultrastructure , Female , Humans , Skin/pathology , Skin Diseases/pathology , Wounds and Injuries/complications
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