Toxic epidermal necrolysis; 15 years'' experience in a Dutch burns centre

BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe and potentially fatal drug reaction characterized by an extensive skin rash with blisters and exfoliation, frequently accompanied by mucositis. The wounds caused by TEN are similar to second-degree burns and severe cases may involve large areas of skin loss. OBJECTIVES: Analysis of our results in patients with TEN and evaluation of the variety of therapeutic interventions that has been studied and suggested in TEN. PATIENTS/METHODS: Retrospective analysis of 19 consecutive patients with TEN treated in our burns centre between 1989 and 2004. RESULTS: Immediate withdrawal of any potentially fatal drug, maximum supportive care, and a restricted and tailored antibiotic, medical and surgical treatment regimen confined mortality to 21%, whereas prognosis scores like APACHE II and SCORTEN predicted mortality of 22 and 30%, respectively. A positive contribution of selective digestive decontamination is suggested but has yet to be established. CONCLUSIONS: Because of a potentially fatal outcome, fast referral of a patient suspected of TEN to a specialized centre (mostly a burns unit or specialized dermatology centre) for expert wound management and tailored comprehensive care is strongly advised and contributes to survival.     

女性慢性下腹痛的干预性治疗

1背景 育龄妇女常见慢性下腹痛，可造成身体损害、情绪忧伤及导致巨大的健康服务费用。美国在这方面的花费超过8亿8千万美元（Mathias 1996）。英国全国数据库的一般性诊治资料显示，慢性下腹痛发病率及流行率与偏头痛、背部痛、哮喘发病率相似（Zondervan 1999）。     

Splicing of the mitochondrial group-II intron rl1: conserved intron-exon interactions diminish splicing efficiency

The mitochondrial intron rI1 is a self-splicing group-II intron of algal mitochondria that can be transferred into chloroplasts from the green alga Chlamydomonas reinhardtii for in vivo investigations (Herdenberger et al. 1994). Thus, rI1 is a suitable system to compare in vitro and in vivo RNA processing. Interestingly, rI1 shows correct RNA splicing, although typical cis-acting exon-sequences (IBS2, δ) of group-II introns are lacking. In order to examine the effect of these exon-intron interactions on splicing, we introduced the endogenous mitochondrial IBS2 sequence in order to produce optimal IBS2-EBS2 base pairing. In addition, the first nucleotide of the 3′exon (δ′) was substituted to create an optimal δ-δ′ interaction. Neither of the two mutations, nor a combination of both, had any effect on the precision of the splice-site selection. Unexpectedly, introduction of IBS2 led to a reduction in the efficiency of the second splicing step in vitro but not in vivo. These findings lead us to conclude that trans-acting factors are present in vivo to optimize splicing efficiency. The possibility is discussed that these factors may, for example, stabilize tertiary intron structures that are a prerequisite for correct RNA processing. Furthermore, our data indicate that similar trans-acting factors promote correct intron splicing in chloroplasts and mitochondria. Received: 18 October / 4 December 1997     

Genetic traits in the area of Bodrogk?z]

In 1984 a late malaria endemic area, called Bodrogk?z was studied. This was a reexamination of the population genetic work performed by Walter, Nemeskéri. In six villages of Bodrogk?z 328 persons were tested for AB0, Rh blood groups, haptoglobins, haemoglobin concentration, haematocrit, erythrocyte amount, the MCV, the MCH and the G-6-PD were analyzed. The quantitative determination of HbF and HbA2, red cell osmotic resistance and thalassemia were measured as well. Thalassemia heterozygote carriers and an increased level of HbF were revealed. The frequency of G-6-PD deficiency was 0.39%. In Bodrogk?z the frequencies of AB0, Rh and haptoglobin types were similar in the present and all previous studies. The background of this similarity might be the genetic similarity between two following generations. On the basis of these facts, the Hb0 Arab and partially DNA work we suggested an alternative hypothesis that these mutant genes got into Bodrogk?z by the rather later migration than with ancient Hungarian people during the period of conquest of Hungary.     

Economics in pediatric type 1 diabetes - results from recently published studies.

OBJECTIVE: Type 1 diabetes in childhood is increasing worldwide. Several therapy components and models of pediatric diabetes care have been introduced. Economic aspects are of increasing interest to decide appropriate strategies in clinical practice. However, knowledge in pediatric diabetes is limited. METHODS: We conducted a literature research to identify and describe systematically recently published analyses on the economics of pediatric diabetes. RESULTS: We substracted ten analyses. Four were cost of illness studies. The main result is that hospitalization, mainly due to metabolic control, education, and acute complications, seems to be a large cost component. Only one study took the perspective of society, including indirect costs. Four of the six studies which evaluated interventions in pediatric diabetes care focussed on ambulatory or home care strategies. Detailed evaluation of new technologies, pharmacotherapy, or screening for late complications is lacking. Except one, all studies performed cost comparison analyses. Evidence is limited, however, diabetes care models may be effective and, possibly, cost saving compared to routine care. CONCLUSIONS: Health economic studies in pediatric diabetes are scarse and limited with respect to topics and quality. Hospitalization has a large impact on health care costs in diabetic children and adolescents. Models in pediatric diabetes care like home intervention may be cost effective compared to more traditional strategies of care. However, further studies are warranted which evaluate clinical and cost effectiveness in pediatric diabetes.     

Reduced secretion of gastric inhibitory polypeptide in turner patients with impaired glucose tolerance

There is a well documented increase in the incidence of abnormal glucose tolerance in patients with Turner syndrome. To elucidate the pathophysiology of this phenomenon, we studied the serum concentrations of gastric inhibitory polypeptide (GIP) — as probably the most important hormonal factor of the entero-insular axis — in relation to impaired glucose tolerance in this syndrome. Oral glucose tolerance tests were performed in 12 Turner patients with simultaneous determination of plasma glucose, insulin and GIP. An impaired glucose tolerance (iGT) was found in four patients with a chronological age between 12.3 and 14.9 years. These patients were compared with found Turner patients of similar age and weight and a normal glucose tolerance (nGT). The highest insulin level occurred 90 min after stimulation in the patients with iGT compared to 30 min in the nGT group. Interestingly, the total areas under the insulin curves were not different. Stimulated plasma GIP concentrations and the areas under the GIP curves wer significantly lower in iGT compared to nGT patients. A disturbed entero-insular axis might contribute to the delayed — rather than diminished — release of insulin in patients with Turner syndrome and impaired glucose tolerance.Deceased February 21, 1987