Cutaneous manifestations associated with gamma heavy chain disease. Report of an unusual case and review of literature.
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Biomedical subjects
Publications and source records attributed to C Picard.
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The authors report the case of a child followed up from the age of 4 months to the age of 15 for asymptomatic congenital heart disease. Initial clinical examination showed the presence of a systolic murmur and a diastolic rumble in the xiphoid area with signs of right atrial dilatation-hypertrophy. During follow-up, an apical diastolic rumble was detected with left atrial dilatation from the age of 3. Doppler echocardiography carried out recently showed double mitral and tricuspid valve stenosis with two-dimensional appearances of doming of both valves and moderate transvalvular pressure gradients with slight elevation of pulmonary artery pressures on the Doppler study. The very early detection of the malformation and the continuous follow-up of this patient indicate the congenital and not rheumatic origin of this double valve stenosis.
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The binding of [125I]-recombinant basic FGF (rec bFGF) to rat hepatic plasma membranes was investigated. [125I] rec bFGF bound to an apparent single class of high affinity binding sites (KD = 69 pM; Bmax = 9.61 fmoles/mg proteins). The absence of low affinity sites was confirmed by the inability of sulphated polysaccharides and heparinase to interfere with FGF binding. A good correlation existed between the ability of bovine pituitary-derived bFGF, rec bFGF and bovine brain-derived aFGF to displace [125I]rec bFGF from these binding sites and their in vitro potency on bovine aortic endothelial cell proliferation.
Lower pulmonary tract cell populations collected by bronchoalveolar lavages (BAL) were evaluated in three groups of immunocompromised patients: HIV infected patients with Pneumocystis carinii (PC) pneumonitis (n = 22), or pneumonitis not related to PC (n = 29), and non-HIV-infected, immunocompromised patients with a PC pneumonitis (n = 18). In AIDS patients with PC pneumonitis, the cell populations were 59.3 +/- 4.5 percent alveolar macrophages (AM), 19.6 +/- 2.5 percent lymphocytes, 14.6 +/- 4.4 percent polymorphonuclear cells (PMN), and 10.3 +/- 3.6 percent eosinophils. In HIV-infected patients without PC pneumonitis, they were 76.5 +/- 3.3 percent AM, 13 +/- 2.1 percent lymphocytes, 9.2 +/- 0.3 percent PMN, and 0.6 +/- 0.2 percent eosinophils, and in non-HIV-infected, immunocompromised patients with PC pneumonitis, they were 43.9 +/- 5.7 percent AM, 30.2 +/- 4.3 percent lymphocytes, 20.4 +/- 4.7 percent PMN, and 0.9 +/- 0.4 percent eosinophils. The most striking finding was a marked BAL eosinophilia in AIDS patients with PC pneumonitis. The significance of this particular cellular pulmonary response to PC is not clear, and its consequences on the lung structures and/or PC require evaluation.
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The authors report two cases of glucagonoma, a rare endocrine tumor of the pancreas, and describe the data currently found in literature. Glucagonoma is a single and usually large tumor, which develops in the alpha cells of the islets of Langerhans and evolves slowly. The combination of characteristic skin lesions, diabetes and weight loss should lead to searching for hyperglucagonemia and for the pancreatic tumor. The diagnosis is usually made rather late, average evolution is five years before diagnosis when it is detected. Imaging, in particular ultrasound and computed tomography (CT), proves to be necessary for the positive diagnosis of glucagonoma as it localizes the pancreatic mass and plays a role in local assessment, thus providing guidance for surgery. The role of imaging is also fundamental for the detection of metastases, which are the only sign of malignancy as no criterion of benignity is found for this tumor.
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In eleven patients with AIDS and suspected Pneumocystis carinii pneumonia we measured the pulmonary clearance and half-life of aerosolized 99m Tc-DTPA (diethylenetriamine pentaacetate). We correlated the half-life with gallium scans and bronchoalveolar lavages. In all nine patients with Pneumocystis, this half-life was shorter than normal value, documenting a significant increase of lung epithelial permeability (p less than 0.001). Six of these 9 patients had abnormal gallium scans. However, in two patients with normal chest X-rays, PaO2 and gallium scans, only the reduced half-life of 99mTc-DTPA evidenced acute lung disease. After treatment, the results of the DTPA half-life correlated with the presence or absence of Pneumocystis. This method is, therefore, a sensitive initial diagnostic test in patients with suspected Pneumocystis pneumonia. A short half-life should prompt bronchoalveolar lavage even when PaO2 and/or chest X-rays and gallium scans are normal.
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