[Family study of lymphocyte populations in common variable immunodeficiency].
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Biomedical subjects
Publications and source records attributed to J Glaser.
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The two complexes formed in the aqueous vanadooxalate system, V(Ox)- and V(Ox)2(3-), have been characterized using 51V, 13C and 17O NMR. For the V(Ox)2(3-) complex, two peaks are observed in 13C NMR and four in 17O NMR. This leads to the conclusion that each oxalate ligand has two different distances to the VO2 group. This fact, together with the peak integrals and the chemical shifts, indicates strongly that the hexacoordinate complex [VO2(C2O4)2]3- found in single-crystal X-ray structure determinations persists in aqueous solution. The dependence of the 13C NMR linewidths upon temperature reveals two types of dynamic processes: (1) a rearrangement in which the two different V-Oox switch places and (2) an exchange of the oxalate ligands in the [VO2(C2O4)2]3- complex with free oxalate, probably through a dissociative process. Rate constants and activation parameters for the two dynamic processes involving [VO2(C2O4)2]3- have been calculated from the shape of the 13C NMR signals. For the V(Ox)- complex, only one relatively narrow peak is obtained in 13C NMR and three peaks in 17O. This fact, as well as the relative positions of these peaks, is in accordance with a pentacoordinate complex [VO2(C2O4)H2O]-, where the two V-O distances to the oxalate ligand are equal. We also show that, in the pH range 0.8-6.6, there is no protonation of the studied complexes, in agreement with previous potentiometric results.
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Metaphyseal abnormalities are present in greater than 90% of infants with symptomatic congenital syphilis. The incidence of these lesions in asymptomatic newborns in the present epidemic is not known. To determine the incidence of bone lesions at birth in asymptomatic congenital syphilis, long-bone films were obtained for all babies born during a 9-month period with a positive perinatal serology. Of 2544 newborns, 61 had a positive maternal serology and 40 also had a positive cord serology. Two symptomatic babies had abnormal radiographs and 12 of 59 asymptomatic newborns had metaphyseal changes consistent with congenital syphilis. It is concluded that long-bone radiographs are abnormal in approximately 20% of asymptomatic newborns with positive perinatal treponemal serology. With the increasing incidence of congenital syphilis, radiologic studies should be included in the assessment of all newborns with a positive serology. Indeed, those patients with any sign of active disease should be carefully followed because even treated infants remain at some risk for developing the late sequelae of congenital syphilis.
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We present our experience with 54 episodes of Pneumocystis carinii pneumonia in 50 young children with AIDS, all but one representing congenitally acquired infection. Findings at history and physical examination are not helpful in suggesting the diagnosis. The diagnosis is suggested by marked hypoxemia, diffuse disease on chest radiograph, and elevated serum LDH level. Because important aspects of the history may be withheld, a high index of suspicion may be necessary for the correct diagnosis. The mortality rate for ventilated patients was 50%.
The concentrations of noradrenaline (NA), dopamine (DA), serotonin (5-HT), and their metabolites were determined in 5 brain areas of non-pregnant, 15 and 20 day pregnant and 4 day post-partum rats. Striatal 5-HT content was significantly lower in 15 and 20 day pregnant rats than in estrous controls. A significant decrease in striatal and frontal cortex 5-hydroxyindole-3-acetic acid (5-HIAA) concentration was observed in 15 day pregnant rats. Significant increases in hypothalamic and hippocampal NA levels were observed at 4 days post-partum. Frontal cortex serotonin S2 receptor KD was reduced in 4 day post-partum rats. There was no significant change in S2 receptor Bmax during pregnancy. Levels of progesterone were negatively correlated with striatal DA, homovanillic acid (HVA), 5-HT, and 5-HIAA levels, hypothalamic DA, hippocampal 5-HT, and frontal cortex 5-HIAA values as well as striatal HVA to DA, and HVA to 3,4-dihydroxyphenylacetic acid (DOPAC) ratios and amygdaloid HVA to DOPAC ratios. The limbic neurotransmitter changes might possibly contribute to mood changes which occur during pregnancy and post-partum.
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A case of aggressive abdominal fibromatosis is reported. Ultrasonography revealed multiple inhomogenous, hypoechoic tumours within the mesenterium and the anterior abdominal wall. Some of these lesions had a hyperechogenic margin.
Cor triatriatum (CTT) sinistrum is a rare, congenital left ventricular inflow anomaly. Previous reports have used cardiac angiography, two-dimensional echocardiography, transesophageal echocardiography and magnetic resonance imaging to establish the diagnosis prior to surgery. We report a case of CTT diagnosed by echocardiography whose hemodynamic significance was established by the use of pulsed wave and color Doppler techniques. Immediate surgery was recommended based on anatomical and hemodynamic parameters.
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Extracorporeal treatment of acute systemic lupus erythematous (SLE) by plasmapheresis has been reported for more than 10 years. Risks of plasmapheresis include unselective procedure, an immunoadsorbent plasma perfusion by IM-P column. Eight of 10 patients evaluated improved through 3 treatments of a 2-liter perfusion. Analysis of proteins removed during plasma perfusion showed a semiselectivity of the IM-P column for circulating immune complexes and anti-dsDNA antibodies. After perfusion of 2 liters of plasma, these substances were reduced by about 35-40%, whereas whole protein was lowered by less than 10% and immunoglobulins by about 18%. Our results in severe SLE indicate that immunoadsorption having the advantage of lowering side effects by dispensing wih foreign protein substitution.
In 73 patients with pneumonia treated empirically with antibiotics or antimycotics, a bronchoscopy with bronchoalveolar lavage was performed in an attempt to identify the pathogens involved. Patients in whom it proved possible to detect a specific pathogen showed a lower mortality rate than did those in whom it was not possible to clarify the aetiology of the pneumonia (24 per cent and 34 per cent, respectively). In patients receiving empirical treatment over a long term, pneumonia persisted longer, and the mortality rate was higher, than in patients who were submitted to invasive examination early on.
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A baby girl, with a birth weight of 2540 g, developed myocarditis with gross, permanent myocardial calcification. The clinical course was progressively downhill, and she died at 14 months of age. Neutralisation antibody to Coxsackie virus group B1 rose from 1:80 after delivery to 1:1280 at age 5 months. The protracted course of this infant's disease represents a new clinical form of fatal neonatal Coxsackie virus group B infection.
The interventricular septal defect in patients with tetralogy of Fallot is, in most cases, a nonrestrictive malalignment type of defect. Rare examples of a restrictive defect have been described. We report a case of tetralogy of Fallot with pulmonic atresia in which the interventricular septal defect was found to be nonrestrictive at birth. The obstruction of the defect developed gradually, until, at the age of 6 years, a gradient of 55 mm Hg across the defect was found at repeat cardiac catheterization. Echocardiography proved that the acquired obstruction was due to excessive tricuspid valve tissue, while Doppler studies suggested a similar gradient between the right ventricle and the ascending aorta. This rare case should alert the clinician to the possibility of an acquired obstruction of the ventricular septal defect, with suprasystemic right ventricular pressure, in cases of tetralogy of Fallot, even if an early study suggested a nonobstructive defect. The diagnosis of this situation is of importance because of the poor prognosis which it carries.