Proximal femoral focal deficiency: radiologic analysis of 49 cases

Proximal femoral focal deficiency, an uncommon congenital anomaly, necessitates early radiologic classification for surgical planning and treatment. Objective radiographic criteria, including femoral length index, acetabular depth index, acetabular angle index, and shape of the proximal femur were determined in 49 patients before cartilaginous ossification of the femoral capital epiphysis; final classification was based on follow-up radiographs or findings at arthrography or surgery. These parameters were analyzed to determine the accuracy and contributions of each in classification. Correct classification into one of three groups was possible in 86% of cases with use of three of the parameters: femoral length index, acetabular depth index, and shape of the proximal femur. The acetabular angle was found to contribute insignificantly to classification. Magnetic resonance imaging, used in only one case, depicted the nonossified cartilaginous femoral capital epiphysis, thus obviating the need for invasive diagnostic procedures and facilitating early classification.     

Mammary fibroblast influence on normal mouse mammary epithelial cell responses to estrogen in vitro

Estrogen-dependent stimulation of progesterone receptor (PgR) concentration or cell proliferation of normal mammary epithelial cells in vitro has been shown to be associated with the presence of mammary fibroblasts. To investigate further the nature of fibroblast influence on epithelial cells, Percoll-purified epithelial cells from collagenase-dissociated mammary glands of mid-pregnant BALB/c mice were co-cultured with mammary fibroblasts that were either untreated, irradiated, or glutaraldehyde-killed or with fibroblast-conditioned medium. Epithelial cells were then assayed for either estrogen-dependent stimulation of PgR by measuring specific [3H]R5020 binding or for estrogen-dependent stimulation of DNA synthesis by [3H]thymidine autoradiography. The results demonstrate that stimulation of PgR does not require the presence of live fibroblasts; either glutaraldehyde-killed fibroblasts or conditioned medium was effective. Pretreatment of culture dishes with type I collagen was equally effective, indicating that fibroblasts may promote the PgR response via a substratum effect. In distinct contrast, estrogen-dependent stimulation of DNA synthesis occurred only when live fibroblasts were present in high numbers and/or in direct contact with epithelial cells. Furthermore, under these latter conditions, epithelial cells also promoted estrogen-dependent stimulation of fibroblast DNA synthesis. Differences in both epithelial and fibroblast cell morphologies were also observed under co-culture conditions, which suggested that cell-cell communication or another interactive phenomenon takes place and is bidirectional. Thus there appear to be at least two different mechanisms by which fibroblasts can influence two specific responses of epithelial cells to estrogen. The present results demonstrate that the specific nature of epithelial-stromal interactions can determine and modulate epithelial cell responses to estrogen and may reflect in vivo regulatory processes affecting normal and neoplastic mammary cells.     

Lipoprotein macroaggregates in bronchoalveolar lavage fluid from patients with diffuse interstitial lung disease: comparison with idiopathic alveolar lipoproteinosis.

Lipoprotein macroaggregates were present in cytocentrifuge preparations of bronchoalveolar lavage fluid from four patients with diffuse lung diseases other than idiopathic alveolar lipoproteinosis. In three patients the primary diagnosis was cryptogenic fibrosing alveolitis and in one sarcoidosis. We confirmed the presence of large multilamellar aggregates of lipoprotein by ultrastructural examination in patients with both interstitial lung disease and idiopathic alveolar lipoproteinosis. The small lamellar bodies and amorphous debris found in idiopathic alveolar lipoproteinosis were rare in the patients with interstitial lung disease. The lavage fluid from patient with interstitial lung disease did not show the substantial alterations in phospholipid composition that were seen in lavage fluid in idiopathic alveolar lipoproteinosis. These ultrastructural and biochemical features may help to distinguish idiopathic from other causes of alveolar lipoproteinosis, particularly at an early stage, when differential diagnosis may be difficult.     

Circulating immune complexes in patients with cryptogenic fibrosing alveolitis.

Increased Clq binding levels have been obtained in serum from twenty-one (50%) of forty-two patients with cryptogenic fibrosing alveolitis (CFA) suggesting the presence of circulating immune complexes. There was a low frequency of positive results using a number of other tests for circulating immune complexes. The increased Clq binding levels were observed in six (35%) out of seventeen patients with lone lung involvement and in fifteen (60%) out of twenty-five patients with extrapulmonary connective tissue disorders. There was an especially close correlation between arthritis and elevated Clq binding. A strong correlation between Clq binding levels and levels of circulating rheumatoid factor (RF) and IgG, and enhancement in macrophage radiobioassay tests using RF-containing sera, suggested that RF might be involved in the circulating immune complexes in these patients. DNAase pre-treatment of sera did not influence the findings, and there was no correlation between Clq binding and levels of immunofluorescent ANA, C-reactive protein levels, or platelet counts. A weak correlation between Clq binding and erythrocyte sedimentation rates, and slightly lower binding levels in treated than untreated patients with 'lone' CFA suggested that binding levels may give some indication of disease activity and may in some instances be influenced by treatment.     

Life expectancy in British Marfan syndrome populations

A total of 206 patients with Marfan syndrome were ascertained throughout genetic clinics in Wales and Scotland during the period 1970–1990. There were 45 deaths representing 22% of the cohort. Mean age at death was 45.3 ± 16.5 years. 50% median cumulative survival in the total cohort (n = 206) was 53 years for males and 72 years for females. Multivariate analysis confirmed severity as the best independent indicator of survival. These findings and survival curves will assist in the counselling of British families and individuals with Marfan syndrome.