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Biomedical subjects

E Rosenthal

Publications and source records attributed to E Rosenthal.

At least 19 recordsLinked to original sources

Translocation (2;14)(p13;q32) in CD10+ ;CD13+ acute lymphatic leukemia.

The rare t(2;14)(p13;q32) was previously described in the three pediatric patients with acute lymphatic leukemia. In these cases this abnormality was found at diagnosis, manifested the sole chromosomal abnormality, and was associated with a favorable prognosis. We here describe three cases of leukemia where such translocations were found at relapse, were associated in two of the cases with additional known characteristic chromosomal aberration, and were associated with a grave prognosis. Interestingly enough, the malignant cells of all three patients shared the same surface antigens: CD34, HLA DR, CD10, CD20, and the myeloid marker CD13. The leukemic clone exhibiting t(2;14) probably evolved from a t(1;19)6q- pre-B acute lymphatic leukemia in one of the cases, and from a chronic phase Ph1 chromosome in another. The significance of the translocation and the coexistence of CD10 and CD13 on the same cell are discussed.

Adolescent

[Bacillary epithelioid angiomatosis in AIDS. Two cases].

Bacillary angiomatosis is a newly recognized multisystem opportunistic infection seen in the human immunodeficiency virus infection. The disease is marked by papular and nodular vascular skin lesions that clinically resemble Kaposi's sarcoma. Histologically, the lesions are different and show clusters of bacteria showing the structure of Gram negative bacilli staining with Warthin-Starry stain. Transmission electron microscopy shows that the organisms (1 to 2 microns) have a trilamellar wall structure. Treatment with oral erythromycin (2 to 3 g a day) for 2 to 4 weeks rapidly leads to resolution.

AIDS-Related Opportunistic Infections

Determinants of hemodynamic results of balloon dilation of aortic recoarctation.

Between October 1982 and May 1990, balloon dilation of aortic recoarctation was attempted in 27 patients. In 1 case dilation was not performed owing to suspected aortic perforation. The remaining 26 patients had 30 procedures. The age at the first dilation ranged between 2.6 months and 18.3 years. After dilation, systolic gradient decreased from 49 +/- 17 mm Hg to 20 +/- 17 mm Hg (p less than 0.001). A reduction of gradient to less than or equal to 20 mm Hg occurred after the first dilation in 17 of 26 (65%) patients. Residual gradients between 25 and 80 mm Hg were present in the remaining 9 patients. During follow-up of 2 months to 6.7 years, 5 of 17 patients with a good initial result developed further recoarctation (2 had successful redilation, 2 had reoperation, and 1 is awaiting repeat dilation). Of 9 patients with gradients greater than 20 mm Hg after the first dilation, 1 had successful redilation and 3 had reoperation. The remaining 5 patients are being managed conservatively. 2 patients developed aneurysms after dilation (1 immediately and the other at 2 months). In all, 15 patients (58%) had a good, and 11 (42%) a poor late hemodynamic result. Aortic diameters at different levels of the aortic arch and at the reconstructed isthmus (normalized to the aorta at the level of the diaphragm) were significantly higher in the group with a good late result than in that with a poor one. Balloon/aortic diameter ratio at diaphragm level also had a significant influence on the late results.

Adolescent

Laser-assisted tricuspid valve balloon dilation for acquired tricuspid valve atresia.

Acquired pulmonary valve atresia is a well-recognized but uncommon complication of surgical systemic-to-pulmonary artery shunts in patients with tetralogy of Fallot. Acquired atresia of the tricuspid valve, however, has not been reported previously. This complication developed in a 3-year-old girl, with pulmonary atresia and an intact ventricular septum, after a Blalock-Taussig shunt and right ventricular outflow tract reconstruction. Percutaneous transcatheter laser-assisted balloon dilation re-established antegrade flow across the tricuspid valve.

Catheterization

The binding and functional properties of voltage dependent calcium channel receptors in pediatric normal and myelodysplastic bladders.

The present study was designed to compare the binding and functional properties of calcium channel receptors in normal and myelodysplastic bladders. Normal bladders were obtained from children with vesicoureteral reflux undergoing ureteral reimplantation. Myelodysplastic bladder specimens were obtained from patients undergoing bladder augmentation. The functional studies included agonist (calcium chloride) dose response experiments and the determination of apparent antagonist dissociation constants for various calcium channel antagonists. The receptor binding studies were performed using the ligand (+)-3H-PN200-110 (specific activity 86.6 Ci./mmol.). The mean maximal response of myelodysplastic bladders to calcium ions was 31% less than normal bladders (p greater than 0.05). The mean EC50 for calcium mediated isometric tension and the mean -log antagonist dissociation constant values of nifedipine, diltiazem and verapamil were similar in normal and myelodysplastic bladders. The radioligand receptor binding studies demonstrated that the equilibrium dissociation constant of (+)-3H-PN200-110 in myelodysplastic bladders was 4-fold greater than in normal bladders. The density of dihydropyridine binding sites in myelodysplastic and normal bladders was similar. Our study demonstrated that the pathophysiology of the poorly compliant hyperreflexic bladder is not related to up regulation of dihydropyridine calcium channel receptors or alterations in the response of detrusor muscle to calcium ions. The relative abundance of calcium channel receptors in the normal and myelodysplastic bladders, and the regulation of detrusor contraction by calcium ions suggest that calcium channel receptors have a meaningful role in detrusor function.

Binding Sites

Laser thermal angioplasty probe ("hot tip") temperature: effects of flow.

The temperature developed by the laser thermal ("hot tip") probe during arterial recanalisation is primarily dependent on the rate of energy delivery and the rate of dissipation to the surrounding medium. While higher probe tip temperatures enhance the efficacy of atheroma ablation, so too is the incidence of adverse effects increased. We studied the temperature developed in the probe tip in an artificial circulation using both saline and blood. In saline the peak probe temperatures were limited to 100 degrees C (boiling point), falling with each increment in flow. Small discrepancies in probes at different times and may be due to malalignment of the optical fibre-metal cap coupling, temperature measurement inaccuracy, tip insulation, or generator output instability. In blood, charring and clot formation insulated the tip raising the temperature (up to 700 degrees C within 5 seconds at 10 W) but also retarded dissipation of heat to the surroundings. The degree of clot and char formation was critical in determining subsequent thermal responses in any particular probe. The unknown rate and quantity of char buildup and changing blood flow during in vivo angioplasty are likely to be important obstacles to developing a reliable thermal feedback control system.

Angioplasty, Balloon

Characterization of 1,4, dihydropyridine calcium channel binding sites in the human prostate.

The binding and functional properties of calcium channel receptors have not been previously characterized in the normal or hyperplastic prostate. Dihydropyridine (DHP) binding sites have been characterized in other tissues using the ligands 3H-nitrendipene and (+)3H-PN200-110. Saturation experiments were performed on homogenates obtained from five human prostate adenomas using these ligands. The binding of 3H-nitrendipine and (+)3H-PN200-110 in the prostate was saturable and of high affinity. The mean Kd of 3H-nitrendipine and (+)3H-PN200-110 was 0.92 +/- 0.11 nM and 0.14 +/- 0.02 nM, respectively. The mean Bmax of 3H-nitrendipine and (+)3H-PN200-110 was 0.57 +/- 0.06 and 0.19 +/- 0.02 fmol/mg. wet wt., respectively. The percent specific binding of 3H-nitrendipene and (+)3H-PN200-110 was 18 +/- 1% and 38 +/- 4%, respectively. The pharmacology of (+)3H-PN200-110 binding sites was further characterized using competition displacement experiments. The IC50 corrected values for Bay K 8644, nifedipine, verapamil, and diltiazem in the human prostate and other tissues are of the same order of magnitude. The prostate contains an abundance of high affinity DHP binding sites. The physiologic significance of the DHP binding sites in the prostate requires further investigation.

Binding Sites

Normal and stenotic human aortic valve opening: in vitro assessment of orifice area changes with flow.

The ability to measure aortic valve area clinically has emphasized the need to understand the changes in aortic valve orifice area during flow. To compare the performance of normal and stenotic human aortic valves we used a pulsatile flow model that simulated in vivo flow conditions. Five normal autopsy specimens and 15 stenotic valves removed at operation were mounted into the model. Valve function was assessed by analysis of video recordings of valve leaflet motion during flow. Over the flow rates tested normal valves demonstrated a linear increase in orifice area. There was no resistance to leaflet opening and valve closure was rapid. The majority of stenotic valves demonstrated an increase in orifice area at low flow rates. No valve showed any increase in maximal area beyond flow rates of 3 l min-1. Increased leaflet resistance of these abnormal valves resulted in notably slower opening and closing rates. In patients with a high cardiac output and severe stenosis, overestimation of the anatomic orifice area derived by the Gorlin equation can result. This is not related to variability in maximal orifice area.

Aortic Valve

In vitro measurement of stenotic human aortic valve orifice area in a pulsatile flow model. Validation of the continuity equation.

Aortic valve orifice area estimation in patients with aortic stenosis may be obtained non-invasively using several Doppler echocardiographic methods. Their validity has been established by correlation with catheterization data using the Gorlin formula, with its inherent limitations, and small discrepancies between the methods are present. To evaluate these differences further, 15 patients with severe aortic stenosis (mean transvalvular gradient 70, range 40-130 mmHg) had aortic valve area estimations by Doppler echocardiography using two variations of the continuity equation. The intact valves removed at valve replacement surgery were then mounted in a pulsatile model and the anatomical area was measured (mean 0.67 +/- 0.17 cm-2) from video recordings during flow at 5.4 l min-1. Aortic valve area calculated using the integrals of the velocity-time curves measured at the left ventricular outflow tract and aortic jet (mean 0.65 +/- 0.17 cm2) correlated best with the anatomical area (r = 0.87, P less than 0.001). The area derived by using the ratio of maximum velocities from the left ventricular outflow tract and aortic jet (mean 0.69 +/- 0.18 cm2) also correlated well with the anatomical area (r = 0.79, P less than 0.001). The index between the left ventricular outflow tract and aortic jet maximum velocities was less than or equal to 0.25 in all. In patients with severe aortic stenosis the aortic valve area can be reliably estimated using Doppler echocardiography.

Adult