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  • 1
    Electronic Resource
    Electronic Resource
    Late onset of renal and hepatic cysts in Pkd1 -targeted heterozygotes (1999)
    [s.l.] : Nature America Inc.
    Nature genetics 21 (1999), S. 160-161 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Autosomal dominant polycystic kidney disease (ADPKD) due to PKD1 mutations is characterized by the progressive appearance of renal, hepatic and pancreatic cysts in adults. We previously reported that targeted deletion of exon 34 in Pkd1, the mouse homologue of PKD1 , results in renal cysts and ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/5944
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  • 2
    Electronic Resource
    Electronic Resource
    Prenatal detection of a Wilms' tumor (1999)
    Springer
    Pediatric radiology 29 (1999), S. 64-67 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Kidney tumors occur very rarely in utero but when present, they are most often congenital mesoblastic nephroma. A newborn boy was transferred to our hospital with a history of fetal renal mass which proved to be a Wilms' tumor. The clinical history, imaging results, and differential diagnosis are presented with a discussion of neonatal Wilms' tumor. While imaging may not specify the exact diagnosis, it provides staging and anatomic information for the surgeon and the oncologist.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002470050538
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  • 3
    Electronic Resource
    Electronic Resource
    Prenatal detection of a solitary liver adenoma (1999)
    Springer
    Pediatric radiology 29 (1999), S. 92-94 
    Details
    ISSN: 1432-1998
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Solitary liver adenomas are rare in children, especially in neonates, and can be difficult to distinguish from other more common liver tumors of newborns and infants. An otherwise healthy male neonate with a prenatal diagnosis of a liver mass underwent ultrasound and MRI followed by resection of the mass. The final histopathological diagnosis was hepatic adenoma. A discussion of the imaging evaluation, differential diagnosis, and literature review is presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s002470050548
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  • 4
    Electronic Resource
    Electronic Resource
    Autoimmune interstitial nephritis and hepatitis in polyglandular autoimmune syndrome (1996)
    Springer
    Pediatric nephrology 10 (1996), S. 511-514 
    Details
    ISSN: 1432-198X
    Keywords: Key words: Polyendocrinopathy ; Chronic renal failure ; Chronic active hepatitis ; Tubular basement membrane ; Autoantibodies
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. A 6-year-old female with polyglandular autoimmune syndrome type I, chronic active hepatitis, and renal failure is described. The renal biopsy demonstrated advanced tubulointerstitial disease with antibodies directed against tubular basement membranes. The patient’s serum contained circulating antibodies directed against both renal and hepatic parenchyma. Renal disease culminating in renal failure and anti-tubular basement membrane disease have not been previously reported in association with polyglandular autoimmune disease. We describe for the first time a patient with polyglandular autoimmune syndrome, chronic active hepatitis, circulating antibodies directed against both renal and hepatic parenchyma, and primary tubulointerstitial disease culminating in renal failure.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004670050153
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  • 5
    Electronic Resource
    Electronic Resource
    Bone marrow transplantation (BMT) for the syndrome of pigmentary dilution and lymphohistiocytosis (Griscelli's syndrome) (1990)
    Springer
    Journal of clinical immunology 10 (1990), S. 146-153 
    Details
    ISSN: 1573-2592
    Keywords: Pigmentary dilution ; partial albinism ; lymphohistiocytosis ; Griscelli's syndrome ; bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the successful treatment of the syndrome of pigmentary dilution and immunodeficiency (Griscelli's syndrome) with allogeneic bone marrow transplantation. Griscelli's syndrome includes silvery hair, recurrent infections, hepatosplenomegaly, progressive neurologic deterioration, and lymphohistiocytosis and is uniformly fatal. We present a family in which four of seven children of consanguinous parents were affected. The affected children were all born with silvery hair. Microscopic examination of the hair showed large clumps of pigment in the hair shaft. Skin biopsy revealed an accumulation of melanosomes in the melanocytes. Three of the affected children were deceased before 20 months of age. Pathology of liver and lung in two of the siblings showed an infiltration of lymphocytes and histiocytes. Immunologic studies in one of these cases were notable for a decreased number of T cells and poor T-cell mitogen stimulation with concanavalin A (Con A) and pokeweed mitogen (PWM). Recently, a fourth sibling with silvery gray hair was referred to us at 3 months of age for evaluation. T-cell function studies were normal and she appeared in overall good health. Because of the expected fatal outcome, allogeneic bone marrow transplantation was performed at 4 months of age. Two years later the patient remains in excellent health. This suggests that cells of hematopoietic origin are responsible for the fatal outcome in Griscelli's syndrome and that bone marrow transplantation early in the course of the disease is an important modality for treatment of this syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00917914
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  • 6
    Electronic Resource
    Electronic Resource
    Cystic Neuroblastoma: Emphasis on Gene Expression, Morphology, and Pathogenesis (1998)
    Springer
    Pediatric and developmental pathology 1 (1998), S. 17-28 
    Details
    ISSN: 1615-5742
    Keywords: Key words: congenital neuroblastoma, cystic neuroblastoma, adrenal cyst, newborn, infant
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: ABSTRACT Cystic neuroblastoma (CN) is an unusual variant of neuroblastoma characterized by a grossly visible cyst(s) and almost always distinctive microcysts on light microscopy. Rarely, CN will appear solid grossly, but microcystification will be present. We examined the clinical, pathologic, and biologic features of 17 cases of CN. The majority of CN had been detected by prenatal ultrasound. The tumors were favorable stage, stroma-poor, but with low or intermediate mitotic-karyorhectic indices and had favorable biologic markers reflected by aneuploidy and by an absence of N-myc amplification and chromosome 1p deletions. However, the high trk expression typically identified in good risk tumors was absent. Although the complete natural history of CN is not fully defined, our experience suggests that some tumors progress in size, whereas others may spontaneously regress or mature. The clinical outcome is excellent, as is expected in localized and stage 4S neuroblastoma in infancy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s100249900001
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