Campomelic syndrome. Difficulties in early differential diagnosis from other syndromes involving deformation of the long bones using echography

One case of "campomélique" dysplasia discovered by echography at 17 weeks is reported. The details of the syndrome are recalled. In this particular case, only the histology enabled the exact diagnosis to be made. The distinction between "syndrome campomélique" and other syndromes involving deformation of long bones (imperfect osteogenesis of the foetal type and isolated curvature of the tibia) is not easy during early pregnancy using echography alone. At a later stage, the signs and symptoms become more distinct and an exact diagnosis is probably easier.     

Increased norepinephrine spillover into the jugular veins in essential hypertension.

In essential hypertension sympathetic nerve firing is commonly increased. A central nervous system origin has been presumed but not tested directly. To estimate cerebral norepinephrine release in essential hypertension, spillover of norepinephrine into the cerebrovascular circulation was measured by isotope dilution, with high internal jugular venous sampling. Norepinephrine was released into the cerebrovascular circulation in both hypertensive patients and healthy volunteers and was present after administration of the ganglion blocker trimethaphan and in patients with sympathetic nervous failure, indicating that brain neurons and not cerebrovascular sympathetic nerves were the probable source. Although differing among hypertensive patients, norepinephrine spillover on average was higher in the hypertensive patients (153 +/- 41 pmol/min) than in healthy subjects (59 +/- 12 pmol/min; p less than 0.05), and was elevated in six of 17 patients, in whom the accompanying whole body norepinephrine spillover rate was higher than in the remaining 11 patients (p less than 0.01). To test for a possible link between brain norepinephrine release and human sympathetic nervous function, the effect of the tricyclic antidepressant desipramine (0.3 mg/kg i.v.) on both brain and whole body norepinephrine spillover was measured in healthy volunteers. Desipramine lowered the cerebrovascular spillover of norepinephrine, its precursor dihydroxyphenylalanine, and its metabolite dihydroxyphenylglycol by 50-80% and produced a mean fall of 35% in whole body norepinephrine spillover. One interpretation of these results is that human sympathetic nerve firing is dependent on norepinephrine release within the brain and that increased cerebral norepinephrine release may possibly be present in some patients with essential hypertension, underlying their higher sympathetic nerve firing rates.     

Short-Chain Fatty Acids Induce Cytoskeletal and Extracellular Protein Modifications Associated with Modulation of Proliferation on Primary Culture of Rat Intestinal Smooth Muscle Cells

Short-chain fatty acids are the main end products of bacterial fermentation of carbohydrates. Their role on the metabolism and biology of colonocytes is now well characterized. However, the functional consequences of their presence on intestinal smooth muscle cells remain poorly studied. We aimed to assess the effect of different short-chain fatty acids on ileal and colonic smooth muscle cells in primary culture and on A7R5 line. Butyrate (above 0.1 mM) inhibited A7R5 cell proliferation, while at low concentration (0.05 to 0.5 mM) butyrate significantly stimulated the proliferation of ileal and colonic myocytes in primary culture. An inhibition was observed at higher concentrations. Collagenous and noncollagenous protein synthesis was stimulated by butyrate. Moreover, butyrate stimulated actin and myosin expression. Thus, butyrate, which is produced by dietary fiber fermentation, may affect intestinal muscles by directly acting at the molecular level on myocytes.     

Macro-optical trapping for sample confinement in light sheet microscopy

Light sheet microscopy is a powerful approach to construct three-dimensional images of large specimens with minimal photo-damage and photo-bleaching. To date, the specimens are usually mounted in agents such as agarose, potentially restricting the development of live samples, and also highly mobile specimens need to be anaesthetized before imaging. To overcome these problems, here we demonstrate an integrated light sheet microscope which solely uses optical forces to trap and hold the sample using a counter-propagating laser beam geometry. Specifically, tobacco plant cells and living Spirobranchus lamarcki larvae were successfully trapped and sectional images acquired. This novel approach has the potential to significantly expand the range of applications for light sheet imaging.OCIS codes: (110.0180) Microscopy, (110.0110) Imaging systems, (180.6900) Three-dimensional microscopy, (350.4855) Optical tweezers or optical manipulation     

The "tako-tsubo" syndrome: report of two cases

The "tako-tsubo" syndrome, initially described by Japanese authors is an apical akinesis of the left ventricule without significant coronary artery disease, with a moderate elevation of troponine I. We report 2 cases of women (with an average age of 68 years). They were refered for suspected acute coronary syndrome including on admission chest pain and ST segment elevation. Cardiac ultrasonography shew an apical akinesis. Coronary angiography didn't show significant coronary lesion. A moderate elevation of troponine was noted but no significant elevation of CPK. The prognosis has been excellent for these 2 patients. We observed a ventricular tachycardia due to QT lengthening in 1 case treated anteriorly by Fleca?n which spontaneously resolved. The first month, the electrocardiogram and cardiac ultrasonography anomalies disappeared totally. The treatment is based on beta-blockers, aspirin, statines, ACE. In the two cases, we didn't find arguments for myocarditis, recanalized thrombosis, coronary spasm, pheochromocytomas, but a risk factor is inboth: emotional stress. Maybe the diagnosis could be easier with the submillimeter cardiac computed tomography.     

Hepatitis and mononucleosis syndrome related to the ingestion of salazosulfapyridine

The authors report the case of a patient treated by salazosulfapyridine and presenting with hepatitis and mononucleosis. The clinical pattern was similar to infectious mononucleosis including pharyngitis and lymphadenopathy but infection by Epstein Barr virus or cytomegalovirus has been ruled out by serological tests. Responsibility of salazosulfapyridine was highly suggested by the following facts: hepatocellular necrosis was sharply centrolobular, plasmocytosis was the main finding in the white blood cells count and all abnormalities rapidly improved after treatment withdrawal. The authors point out that salazosulfapyridine intolerance could mimic infectious mononucleosis and viral hepatitis. In such cases the drug must be rapidly withdrawn to avoid massive hepatic necrosis.