0000000000524032

AUTHOR

H. Stöss

showing 4 related works from this author

Sedaghatian congenital lethal metaphyseal chondrodysplasia—observations in a second Iranian family and histopathological studies

1987

In 1980, Sedaghatian described in two brothers and one sister a neonatally lethal disorder associated with slight rhizomelic limb shortness, mild platyspondyly, and severe metaphyseal dysplasia. Here data are presented on another Iranian infant with the Sedaghatian syndrome who died on day 4 and was found to have histologic evidence of severe epimetaphyseal dysplasia. The occurrence in children of both sexes in one instance, born to normal parents who were first cousins, and currently apparent confinement of the disorder to Iranians suggests that the Sedaghatian syndrome is an autosomal recessive trait with high gene frequency in Iranians. This may be a more complexly pleiotropic syndrome t…

MaleGeneticsmedicine.medical_specialtyDepressed nasal bridgeMetaphyseal chondrodysplasiabusiness.industryInfant NewbornShort neckConsanguinityIranOsteochondrodysplasiasmedicine.diseaseMetaphyseal dysplasiaDermatologyMicrophthalmiaBone and BonesAutosomal recessive traitCartilageDysplasiaotorhinolaryngologic diseasesmedicineHumansbusinessGenetics (clinical)American Journal of Medical Genetics
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Cell types infected in human cytomegalovirus placentitis identified by immunohistochemical double staining

1993

Chronic villitis is almost always present in intrauterine infection with human cytomegalovirus (HCMV). The inflammatory response to this virus has been described in detail. However, little is known about the types of placental cells that may be infected by HCMV and six cases of HCMV placentitis were thus investigated to identify the vulnerable cell types. Immunohistochemical double staining analyses were performed using antibodies to HCMV immediate early antigens and to specific cellular marker proteins. Fixed connective tissue cells could be demonstrated to be the predominantly infected cell type in each placental tissue. Endothelial cells and macrophages were also found to be infected in …

Human cytomegalovirusPathologymedicine.medical_specialtyPlacenta DiseasesTransplacental transmissionvirusesCongenital cytomegalovirus infectionCytomegalovirusConnective tissuePathology and Forensic MedicineAntigenPregnancymedicineHumansVimentinMacrophageEndotheliumPregnancy Complications InfectiousAntigens ViralMolecular BiologyCytopathic effectbiologyMacrophagesvirus diseasesCell BiologyGeneral Medicinemedicine.diseaseImmunohistochemistryVirologymedicine.anatomical_structureConnective TissueCytomegalovirus Infectionsbiology.proteinFemaleAntibodyVirchows Archiv A Pathological Anatomy and Histopathology
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Heterogeneity of osteogenesis imperfecta. Biochemical and morphological findings in a case of type III according to Sillence.

1986

A male infant with pale-blue sclerae, who died at the age of 6 weeks through the aspiration of food, presented multiple fractures and deformation of the long tubular bones. The clinical and radiological findings and the course indicated osteogenesis imperfecta, type III, according to Sillence's classification. The family history was unremarkable. Light and electron microscopic studies of iliac crest bone obtained postmortem, showed an abrupt interruption of endochondral ossification, with an active periosteal ossification. In the region of the fractures, a mixed desmochondral callus was seen. The endoplasmic reticulum of the osteoblasts was markedly dilated, the mitochondria were swollen. T…

MaleProlineEndoplasmic ReticulumHydroxylationIliac crestHydroxylysineBone and BonesOsteogenesis Imperfecta Type IIIchemistry.chemical_compoundMedicineHumansAmino AcidsEndochondral ossificationSkinOsteoblastsbusiness.industryOsteoidCartilageInfantAnatomyOsteogenesis Imperfectamedicine.diseaseChromatography Ion ExchangeHydroxylysinemedicine.anatomical_structureCartilagechemistryOsteogenesis imperfectaPediatrics Perinatology and Child HealthCollagenbusinessMitochondrial SwellingReticulumEuropean journal of pediatrics
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Osteogenesis imperfecta and hyperplastic callus formation: light- and electron-microscopic findings.

1993

In rare cases of osteogenesis imperfecta, an "overshoot" growth of new bone may occur, which, clinically gives the impression of a tumour. This condition is known as hyperplastic callus formation. Morphology showed an excessive mixed desmal-chondral osteoneogenesis. Atypical collagen fibrils in non-callus tissue represent an indicator for this callus formation in individual patients.

HyperplasiaCallus formationChemistryfungifood and beveragesAnatomyHyperplasiaOsteogenesis Imperfectamusculoskeletal systemmedicine.diseaseOsteochondrodysplasialaw.inventionbody regionsMicroscopy Electronsurgical procedures operativeOsteogenesis imperfectalawCallusmedicineHyperplastic callus formationHumansElectron microscopeBony CallusElectron microscopicGenetics (clinical)American journal of medical genetics
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