Generalized Lichen Nitidus, with Perioral and Perinasal Accentuation, in Association with Down Syndrome

Abstract:   Generalized cases of lichen nitidus are rare, but have been infrequently reported in association with Down syndrome. We report the third such case, with unusual perioral and perinasal accentuation.     

The contryphans,a d-trvptophan-containing family of Conus peptides: interconversion between conformers

We previously characterized contryphan-R, a d -tryptophan-containing octapeptide from the venom of Conus radiatus. In this study, we present evidence that the contryphan family of peptides is widely distributed in venoms of the fish-hunting cone snails. We purified, synthesized and characterized contryphan-Sm from Conus stercusmuscarum venom, and obtained molecular evidence for the existence of a third peptide, contryphan-P from Conus purpurascens venom ducts. The sequences of these three contryphans showed identity in seven of eight amino acids and a conserved pattern of post-translational modification. We also demonstrate that contryphan-Sm equilibrates between two distinct conformational states.     

Novel Analysis of Clonal Diversification in Blood B Cell and Bone Marrow Plasma Cell Clones in Immunoglobulin Light Chain Amyloidosis

Immunoglobulin light chain amyloidosis (AL) is characterized by a limited clonal expansion of plasma cells and amyloid formation. Here, we report restriction in the diversity of VL gene usage with a dominance of clonally related B cells in the peripheral blood (PB) isotype-specific repertoire of AL patients. A rigorous quantification of lineage trees reveals presence of intraclonal variations in the PB clones compared to the bone marrow (BM) clones, which suggests a common precursor that is still subject to somatic mutation. When compared to normal BM and PB B cells, AL clones showed significant but incomplete impairment of antigenic selection, which could not be detected by conventional R and S mutation analysis. Therefore, graphical analysis of B cell lineage trees and mathematical quantification of tree properties provide novel insights into the process of B cell clonal evolution in AL.     

Central apnoeas have significant effects on blood pressure and heart rate in children

Brief central apnoeas (CAs) during sleep are common in children and are not usually considered clinically significant unless associated with oxygen desaturation. CAs can occur spontaneously or following a movement or sigh. The aim of this study was to investigate acute cardiovascular changes associated with CAs in children. Beat‐by‐beat mean arterial pressure (MAP) and heart rate (HR) were analysed across CAs, and spontaneous and movement‐induced events were compared using two‐way analysis of variance with post hoc analyses. Fifty‐three children (28 male/25 female) aged 7–12 years referred for investigation of sleep‐disordered breathing (SDB) and 21 age‐matched healthy controls (8 male/13 female) were studied. Children underwent routine clinical polysomnography with continuous blood pressure (BP) recordings. Movement‐induced, but not spontaneous, CAs were more frequent in children with mild or moderate/severe obstructive sleep apnoea (OSA) compared with healthy controls (P < 0.05 for both). Movement‐induced CAs were associated with significantly larger MAP and HR changes across the event compared with spontaneous CAs. The percentage changes in MAP and HR between late‐event and post‐event were significantly greater for movement‐induced compared with spontaneous CAs (MAP 20.6 ± 2.3 versus 12.2 ± 1.8%, P < 0.01; HR 28.2 ± 2.6 versus 14.7 ± 2.5%, P < 0.001). This study demonstrates that movement‐induced CAs are more common in children with OSA, and are associated with significantly greater changes in HR and BP compared with spontaneous CAs. These data suggest that movement‐induced CAs should be considered when assessing the cardiovascular impact of SDB.     

Transcatheter Endocardial Ablation: Acute Effects of Direct Current Countershock on Regional Myocardial Blood Flow

Effect of DC Shock Ablation on Myocardial Blood Flow. Introduction: High energy direct current ablative shocks delivered through endocardial electrode catheters have been used as a treatment for various cardiac arrhythmias including ventricular tachycardia. Frequently, these shocks result in depression of myocardial function, at least transiently. Methods and Results: This study assessed the effect of a 200-joule direct current shock delivered endocardially to the apex of the heart in ten adult mongrel dogs. Arterial blood pressures were monitored continuously. Myocardial contraction was monitored via two-dimensional echocardiography. Regional myocardial blood flows were measured using radionuclide labeled microspheres before delivery of the shock, 30 seconds after delivery of the shock, and at 5 and 10 minutes after the shock. The results of the study demonstrated that there was a marked drop in arterial blood pressure immediately after the shock that gradually recovered over a 20-minute period. Regional myocardial blood flow at the site of catheter ablation was severely depressed immediately after the shock. The degree of myocardial blood flow depression was inversely related to the distance from the site of ablation. At 5–10 minutes after ablation there was a marked hyperemic response in areas that suffered from depressed flow immediately after the shock. Two- dimensional echocardiography demonstrated depressed contractile function immediately after the shock with gradual recovery over a 10- to 20-minute period. The catheter shock also generated prominent echo densities within the myocardium surrounding the ablative site. In contrast to the intracavitary echo densities that cleared quickly, the intramyocardial densities cleared much more slowly. Conclusion: The results of the study confirm the clinical observation that human left ventricular function is depressed acutely following a high-energy ablative shock delivered to the left ventricular endocardium. Our results also demonstrated for the flrst time the effect of such ablative shocks on regional myocardial blood flow. Such depressions of blood flow may help explain the sudden drop in contractile function of the heart. The persistent intramyocardial echoes seen after the shock probably represented generation of intramyocardial bubbles that could obstruct arterial flow as well as disrupt cellular function. (J Cardiovasc Electrophysiol, Vol. 3, pp. 40–47, February 1992)     

The Effect of Group Counseling on the Frequency of Grief Reported by Infertile Couples

Twenty-nine individuals undergoing infertility evaluation or treatment attended a six-session, group infertility program. At the first and last session, the individuals were asked to complete a questionnaire about grief, self-concept, marriage satisfaction, and sexual satisfaction. The pretest and posttest scores were used to determine if an infertility group counseling program could be effective in reducing the frequency of grief responses. The study confirmed that infertility counseling was effective in reducing the self-reported symptoms of grief experienced by some infertile couples.     

AN ANALYSIS OF THYROTROPHIN RECEPTOR BINDING AND THYROID STIMULATING ACTIVITIES IN A SERIES OF Graves'' SERA

Improved receptor and bioassays have been used to compare TSH receptor binding and thyroid stimulating activities in unextracted sera from 110 patients with Graves' disease. The two parameters showed a significant correlation (r = 0.65; P less than 0.001) although there were some clear discrepancies. Dose-response studies in 17 sera showed that both receptor binding and thyroid stimulating responses always increased with increasing doses of serum. In patients who were in relapse or remission following antithyroid drug treatment, the results of both bio- and receptor assays correlated well with disease activity with only one clear discrepancy which could have been attributable to the coexistence of autoimmune stimulation and destruction of the thyroid.     

Evaluation of the Acute Respiratory Effects of Aerosolized Machining Fluids in Mice

Using a previously developed bioassay, the sensory and pulmonaryirritating properties of a group of 10 aerosolized machiningfluids were evaluated in mice. Single, 3-hr inhalation exposureswere conducted with the fluids at exposure concentrations-rangingfrom 20 to 2000 mg/m³. The results have shown that all 10 werecapable of inducing sensory and pulmonary irritation, with littleor no change in pulmonary histopathology. A concentration-responserelationship was developed for each fluid which revealed that,for the 10 fluids studied here, the synthetic/semisyntheticand soluble fluids were more potent irritants than the straightoils. Also, 3 of the 10 fluids which had been collected fromworkplace operations (i.e., "in use" fluids) were found to besimilar in potency to the same fluids prior to their introductioninto the workplace (i.e., "neat" fluids). From concentration-responserelationships, the RD50 value (i.e., concentration inducinga 50% response) was obtained for each of the 10 fluids. TheRD50 values ranged from 100 to 1000 mg/m³ for all fluids exceptthe straight oils whose RD50 values were over 100,000 mg/m³.Using these values, exposure limits were then suggested forworkers in industry to prevent irritation. This bioassay maybe a good first step in evaluating new machining fluids whoseformulations may change depending upon the current industrialneeds.     

Electrocardiographic Abnormalities,Malignant Ventricular Arrhythmias,and Cardiomyopathy Associated With Loperamide Abuse

A 20‐year‐old man presented with recurrent syncope and abnormal electrocardiogram (ECG). His evaluation revealed a prolonged QT interval >600 milliseconds, witnessed torsades de pointes (TdP), and dilated cardiomyopathy. At his initial admission, an ICD was implanted and atrial pacing at 80 beats per minute suppressed ventricular arrhythmias. The patient was readmitted with device infection and recurrent TdP leading to intubation. This led to the discovery of a hitherto unrevealed loperamide abuse and his cardiac arrhythmias and LV dysfunction were determined to be related to large doses of loperamide. Following abstinence, his ejection fraction and ECG returned to normal.