Trisomy 18q-. Trisomy mapping of chromosome 18 revisited

Two patients with trisomy for 18p and the proximal segment of 18q (trisomy 18q –) are reported and compared with similar cases from the literature. The phenotype of trisomy 18q– resembles that of full trisomy 18 but differs mainly in the birdlike face, small mouth, more pronounced microretrognathia, minor skeletal dysplasia and inner organ malformations, and less severe vital prognosis.     

Experience with intracoronary streptokinase in 36 patients with acute evolving myocardial infarction

Acute angiography was performed in 36 consecutive patients withevolving myocardial infarction admitted within 3 h after onsetof symptoms. No fatal complication occurred. Angiography revealeda total occlusion in 32 patients (89%), a subtotal stenosisin three (8%), and a 90% stenosis in one patient (3%). Anteriorinfarction was exclusively related to left anterior descending,and inferior infarction to right coronary or circumflex obstruction.After identification of the ‘infarct-vessel’, nifedipine10 mg was administered sublingually. In no patient was anterogradeflow affected with this treatment. In 35 patients an attempt to lyse clot was made with intracoronarystreptokinase; an infusion of 2000–4000 U/min, precededby a bolus of 10 000–20 000 U was infused into the ‘infarct-vessel’. In 26 patients (74%) reperfusion was achieved, two combinedwith guidewire perforation. The mean duration of onset of symptomsto reperfusion was 3.6 h (range 1.8–5.6). The mean durationof lysis was 1.2 h (range 0.3–3), and the mean dosageof streptokinase was 200 000 U (50 000–400 000 U]. In25 out of 26 patients (96%) a high degree of obstruction remainedimmediately after lysis and at repeat angiography 6–8weeks after the acute event. Despite treatment with aspirin200 mg daily and nifedipine 30 mg daily four re-occlusions occurred.Coronary bypass surgery was performed electively in five patients. Thus, we conclude that in patients with evolving myocardialinfarction, the infarct-vessel can be recanalized in 74% ofpatients by intracoronary streptokinase. The true benefit ofthis treatment must await a controlled study.     

Light stability of molsidomine in infusion fluids

Abstract— The influence of artificial light, daylight or stimulated sunlight on the stability of molsidomine was investigated. In static experiments an 80 μg mL^?1 solution of molsidomine in saline was stored in an unprotected infusion bag. During dynamic experiments the molsidomine solution (80 μg mL^?1) was dropped at 12·5 mL h^?1 from an unprotected infusion bag, from an infusion bag covered with aluminium-foil, or from an infusion bag protected with a UV-cover. Either unprotected infusion tubing or infusion tubing with a UV-filter were connected to the infusion bags. Static as well as dynamic experiments showed a half-life of about 20 min for the unprotected molsidomine solutions, when placed behind a window during a sunny day. Protection from light of the infusion bag but not of the infusion tubing had only a minor influence on the drug half-life. Protection of the infusion bag and the infusion tubing with a UV-filter increased the half-life to several days. These results confirm that both the infusion bag and the infusion tubing need adequate light protection during molsidomine administration.     

Extracorporeal photochemotherapy induces apoptosis of infiltrating lymphoid cells in patients with mycosis fungoides in early stages. A quantitative histological study

Extracorporeal photochemotherapy (ExP) is a well-tolerated new form of chemoimmunotherapy, which is considered to be effective for cutaneous T-cell lymphoma (CTCL) and the treatment of choice for Sézary syndrome. Improvements have also been seen in patients with non-erythrodermic mycosis fungoides (MF) in the early stages, even when tumour cells are not detectable in the peripheral blood. In this study, we used ExP as a monotherapy in seven patients who had early stage (Ib) MF, and who were no longer responsive to or had contraindications for other therapies. We observed a clinical improvement in the disease after 12 months of treatment: one patient showed a complete response, five a partial response, and one remained stable. In each patient we compared skin biopsies of large plaque lesions before and after the treatment. We undertook a histological evaluation of the infiltrate. The lymphoid cell proliferation and death rates were quantified using the following parameters: lymphoid cell density (LCD), Ki67+ lymphoid cell nuclei percentage (Ki67+ Lcn percentage), and apoptotic index (AI). Significant decreases in the lymphoid cell infiltrate and in cell proliferation, and a significant increase in AI were observed after therapy. The mean LCD decreased from 187 ± 33 to 34 ± 17·7, Ki67+ Lcn mean percentage decreased from 16·9 ± 3·9 to 4·9 ± 2·4, and the AI mean value increased from 0·05 ± 0·03 to 2·41 ± 1·54. Our results suggest a role for apoptosis in the improvement of the skin lesions and are in line with some reports on the mode of action of ExP. Although the way in which ExP works needs to be clarified further, it does seem to stimulate a CD8+ cell-mediated anticlonotypic activity against circulating pathogenic clones. Furthermore, a release of tumour necrosis factor α (TNF-α) by circulating monocytes has been demonstrated after ExP. Both are known to induce cell death by apoptosis.