Biomedical subjects
Publications and source records attributed to J W Steffelaar.
The case history is presented of a 10-year-old boy with a fatal combination of acute appendicitis and infectious mononucleosis, in the literature a particularly rare combination. The boy died of a perforative peritonitis. His appendicitis appeared not to be a complication of the infectious mononucleosis.
An 18-year-old man visited the outpatient cardiology clinic because of a cardiac murmur found at a medical examination for military service. An Ebstein's anomaly was diagnosed, with tricuspid valve insufficiency. Exposure to lithium in utero was established. Literature reports indicate an association between exposure to lithium in utero and cardiac anomalies, notably Ebstein's anomaly.
Three stillborn fetuses are reported in which an abdominal wall defect was associated with defects in the urogenital and anal region. Autopsy of these fetuses provided clues indicative of how and where the embryonic development went wrong. The malformation involved a disturbance of the cell deposition process, occurring in the caudal part of the embryo. During the cell deposition process, which takes place in the neural crest and the body wall placode, ectodermal cells are added to the mesodermal compartment of the embryo, thus contributing to the anlagen of several structures, including the ventral body wall. In addition, a change in the shape of the embryo is generated. The sequence of events resulting from a disturbance of the cell deposition process is explained.
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A case is reported of pulmonary lymphangiomyomatosis in 44-year-old woman presenting with progressive exertional dyspnoea, unproductive cough and haemoptysis. The patient showed no symptoms or signs of tuberous sclerosis, a condition often found in association with pulmonary lymphangiomyomatosis. A pulmonary biopsy specimen showed disruption of alveolar septa and fibrosis. Subpleural lymph vessels and pulmonary venules showed cuffs of spindle-shaped cells. The leiomyomatous nature of these cells was evident from their appearance and staining reactions with light microscopy, and the presence of smooth muscle antigens as demonstrated by immunofluorescence technique. Since the lesion is not restricted to lymphatics, but can also be present in blood vessels, the condition may more appropriately be designated as pulmonary angiomyomatosis.
Polymorphonuclear leucocytes (PMN) from patients with systemic lupus erythematosus (SLE) were isolated from defibrinated and heparinized blood. In addition, PMN from a healthy donor were incubated with sera from SLE patients and with sera containing artificially prepared immune complexes of hepatitis B surface antigen (HBsAg) and human anti-HBsAg immunoglobulin (anti-HBs) with well defined variations of the antigen/antibody ratio. To one group of blood samples, 5 mM monoiodine acetic acid (MIAA) was added to block in vitro phagocytosis. The Pmn were examined for the presence of IgG, IgM, and HBsAg by the immunofluorescence technique. PMN from defibrinated blood of SLE patients showed in up to 80% immunoglobulin (Ig)-inclusions. However, addition of 5 mM MIAA reduced the number of Ig-containing PMN to at most 40%, which levels were equal to numbers found in specimens from heparinized blood. Addition of 5 mM MIAA to heparinized blood did not reduce the number of PMN with Ig inclusions. Normal donor PMN isolated from defibrinated, heparinized, and EDT blood showed equal amounts of Ig inclusions after incubation with SLE sera, but none when MIAA had been added. In PMN incubated with HBsAg-anti HBs immune complexes with an antigen antibody ratio between 5 and 0-2, both HBsAg and IgG could be detected. It is concluded that Ig inclusions in PMN from heparinized blood from SLE patients are due to in vivo phagocytosis, presumably of circulating immune complexes. In vitro phagocytosis of Ig from SLE sera by normal donor PMN also suggests the presence of immune complexes. Dependent on the antigen-antibody ratio, artificial HBsAg/anti-HBs immune complexes can be detected by in vitro phagocytosis by PM.
A 70-year-old man had an obstruction of the aortic bifurcation by a primary aortic sarcoma. The tumor was not fibrosarcomatous, as are the majority of primary aortic tumors. To our knowledge, this is the third occurrence of this type of primary aortic malignant neoplasm to be reported in the literature. A possible endotheliomatous origin is suggested.