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Biomedical subjects

C Lawrence

Publications and source records attributed to C Lawrence.

At least 163 records · Page 9Linked to original sources

An objective sign in painful crisis in sickle cell anemia: the concomitant reduction of high density red cells.

The etiopathologic basis of painful crisis in sickle cell anemia is largely unknown, and no objective criteria for its diagnosis and follow-up exist at present. We have studied 11 patients through 14 painful crises and observed a significant decrease of the densest fraction of red cells in 12 of the 14 crises as determined by isopycnic Percoll-Stractan continuous density gradients. If the first observation is normalized to 100%, the average decrease in dense cells was 77% with a range of 36% to 94%. The time needed for the percentage of dense cells to return to the steady-state level varied from seven to more than 30 days. These findings were in sharp contrast to the stability of the density pattern observed in another group of sickle cell patients, who were studied during crisis-free periods. The mechanism of the disappearance of dense cells could involve selective destruction by the reticuloendothelial (RE) system, selective sequestration in the areas of vasoocclusion, or a combination of both factors.

Adult↗

Inhibition of cold insolubility of an IgA cryoglobulin by decanedicarboxylic acid and related compounds.

Cold insolubility of a serum IgA cryoimmunoglobulin was found to be inhibited by the addition of 1.5 mM sodium decanedicarboxylate in vitro. The patient with the cryoglobulin had advanced multiple myeloma complicated by severe hyperviscosity that caused lethargy and episodic loss of consciousness. Decanedicarboxylic acid administered orally resulted in transient relief of symptoms and the loss of cryoprecipitability of the paraprotein. Further in vitro studies revealed that sodium salts of long-chain monocarboxylic acids with a minimum of eight carbons, and dicarboxylic acids with a minimum of 12 carbons inhibited cryoprecipitation. Salts of short-chain carboxylic acids, by contrast, enhanced cryoprecipitation. Sodium phenolate and sodium salts of benzoic acid, 2,4-DNP, phenylpropionic acid, and salicylic acid were also inhibitory. These latter compounds, which have a ring structure, did not cause precipitation at any concentration. It was demonstrated that the presence of a free carboxylic group was required for these activities; conversion of carboxylic acid to amide resulted in the loss of both the inhibitory and cryoprecipitation-enhancing effects. Normal plasma, or plasma from five other patients who had IgG, IgM, or mixed-type cryoglobulinemia, were not affected by any of these compounds. It is suggested that in selected cases of hyperviscosity syndrome associated with cryoglobulinemia, some of these compounds, especially monocarboxylic acids with appropriate chain lengths, or those with a ring structure, may have therapeutic applications.

Acetamides↗

A noninvasive comparison of left ventricular performance in sickle cell anemia and chronic aortic regurgitation.

Abnormalities of the cardiovascular system are common in patients with sickle cell anemia (SS). Noninvasive testing to document left ventricular dysfunction has yielded conflicting results. Left ventricular performance was evaluated in 27 patients with SS by M-mode and 2-D echocardiography, and systolic time intervals. Comparisons were made to 25 normal controls, and to 22 patients with chronic aortic regurgitation. Left ventricular diastolic diameter (LVDD) and cardiac index (CI) were significantly greater in the patients with SS than in controls (LVDD 5.3 +/- 0.4 vs. 4.7 +/- 0.5 cm; CI 4.2 +/- 1.3 vs. 3.1 +/- 0.8 liters/min/m2; both p less than 0.001). Left ventricular ejection fraction (EF) was slightly, but significantly less (62.9 +/- 7.3 vs. 67.0 +/- 5.4; p less than 0.05). In comparison to the patients with chronic aortic regurgitation, the LVDD in the patients with SS was slightly, but significantly lower (LVDD 5.3 +/- 0.4 vs. 5.9 +/- 0.6 cm; p less than 0.05). There was no significant difference between the patients in EF or CI (EF 62.9 +/- 7.3 vs. 63.3 +/- 4.4; CI 4.2 +/- 1.3 vs. 5.0 +/- 1.0 liters/min/m2; NS). Left ventricular EF was below 55 in three patients who also had hypertension at the time of examination. We conclude that patients with SS have resting left ventricular performance consistent with a high output state. Significant left ventricular dysfunction related to sickle cell disease alone was not demonstrated in this population, although the addition of hypertension appears to deleteriously affect resting left ventricular performance.

Adult↗

Progressive cardiac conduction defect and cardiomyopathy in siblings with syncope.

Three siblings evaluated for syncope were found to have left bundle-branch block. Progression to complete heart block occurred in all. Pacemaker implantation eliminated syncopal epidoses in each case. Echocardiographic manifestations of cardiomyopathy were present in each child despite normal roentgenographic heart size. Careful family study identified no other affected members with conduction defects. These children area believed to represent a form of familial cardiomyopathy in which clinical manifestations of cardiac conduction system disease predominate.

Bundle-Branch Block↗

A comparative study of four methods of evaluating velopharyngeal adequacy.

Forty-eight subjects with repaired palatal clefts were divided into three major groups on the basis of speech symptoms and were examined for velopharyngeal valving integrity by means of the R-D nasal manometer, pressure-flow techniques, the Hunter oral manometer, and videofluoroscopy. Videofluoroscopy appeared to provide data that most nearly agreed with predictions of valving made from speech. The Hunter oral manometer was the least useful of the four techniques.

Adolescent↗

Narcotics addiction treatment: behavioral methods concurrent with methadone maintenance.

Methadone maintenance patients were assigned among four treatment cells involving contingency management, emotionally-based behavior therapy, a combination of these, or a control condition receiving counseling but no behavioral treatment. With 60 patients who remained in treatment at least 6 months, treatment outcomes were compared using objective measures: hours employed, urinalysis results, and counseling participation. No significant differences were found between modalities. Case studies suggested that under appropriate conditions, contingency management might reduce illicit drug use. It is suggested that focused experimental studies may be more productive than broad clinical outcome evaluations.

Adult↗