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）。     

The use of magnetic resonance imaging and spectroscopy in the assessment of patients with head and neck and other superficial human malignancies

The proper demarcation of diseased tissue is important for radiation therapy planning and treatment. The volume to be irradiated is usually identified on radiographs or on x-ray computed tomography (CT) sections. Magnetic resonance (MR)-derived images of the proton T2 relaxation times in small pixel elements, typically 0.5 mm2 or less, provide significantly sharper differentiation between normal and diseased tissue. The T2 values in tissue depend on the tissue composition, histologic condition, and physiologic environment within the tumor. Furthermore, for many tumors the histogram of T2 values has a clear biphasic distribution suggesting that T2 maps may be useful for the identification of necrotic or hypoxic regions within tumors. The distribution of T2 values within the tumor bed shows the general pattern that the T2 values are elevated with a range greater than that seen in normal muscle. Elevated T2 values are not by themselves diagnostic of malignancy; however, they demonstrate the heterogeneity of the microenvironment present within a tumor. The spatial distribution of T2 values is being explored as a method for computer assistance in the delineation of the target volume for treatment planning. In addition, MR P-31 spectroscopic examinations were performed on 30 patients with squamous cell carcinomas of the head and neck. Although hampered by muscle contamination in some P-31 spectra obtained with surface coil profile localization techniques, significant trends can still be appreciated in our data. These trends include the following: (1) the P-31 spectra from malignant tissue have well-resolved spectral lines in the upfield region that correspond to Pi, phosphomonoester (PME), and phosphodiester (PDE) not usually seen in normal muscle; (2) the PDE/B-ATP and PME/B-ATP ratios are greater than unity in all cases; and (3) most of the tumors have higher PME peaks than PDE peaks. The P-31 spectra from patients treated with ionizing radiation changed during and after therapy. Some of the changes could be associated with alteration of the tumor metabolic activity or synthesis and breakdown of lipoproteins. These studies suggest that magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) studies may be useful for both radiotherapy treatment planning and the noninvasive monitoring of patients both before and during treatment.     

Central nodal necrosis and extracapsular neoplastic spread in cervical lymph nodes: MR imaging versus CT.

Computed tomographic (CT) scans and magnetic resonance (MR) images obtained in 24 patients with cervical lymphadenopathy were retrospectively and blindly evaluated by two readers for the presence of central nodal necrosis (CNN) and extracapsular nodal spread (ENS). The CT studies were all enhanced, and the MR images were obtained with short repetition time (TR)/echo time (TE), long TR/double echo, and enhanced short TR/TE fat-suppressed sequences. Each MR imaging sequence was interpreted separately and then collectively. Sixty lymph nodes were identified with CT. Sensitivity for CNN was 16%-67% with the unenhanced MR pulse sequences, 50% with enhanced sequences, and 83%-100% with CT. The most accurate reading of MR images for CNN was with the unenhanced T1-weighted and T2-weighted images (86%-87%); the accuracy of CT was 91%-96%. The accuracy of MR imaging for detecting ENS was maximal with T1-weighted images (78%-90%). Gadolinium-enhanced, fat-suppressed images did not improve accuracy in evaluating CNN or ENS. CT is currently more accurate than unenhanced or enhanced MR imaging in detecting CNN or ENS.     

Regulation of synoviocyte proliferation, apoptosis, and invasion by the p53 tumor suppressor gene.

Recent studies show that 1) the p53 tumor suppressor protein is overexpressed by rheumatoid arthritis (RA) synovium and fibroblast-like synoviocytes (FLS) and 2) somatic mutations previously identified in human tumors are present in RA synovium and FLS. We have hypothesized that abnormalities in p53 can contribute to chronic destructive RA synovitis. To understand the functional consequences of p53 abnormalities in FLS, RA and normal FLS expressing wild-type p53 were transduced with a retroviral vector encoding the human papilloma virus 18 E6 gene, which inactivates endogenous p53 protein. Three RA and one normal FLS lines were infected with recombinant retrovirus encoding the neomycin resistance gene (neo) or E6+neo. FLS proliferation, apoptosis, and invasion was studied in E6, neo, and uninfected parental strains (PS). The growth rate for E6 was significantly increased with a sixfold increase in cell number after 7 days compared with a twofold to threefold increase in neo and PS. When FLS were treated with cytokines, proliferative response of E6, neo, and PS to interleukin-1 and transforming growth factor-beta were similar. However, response to platelet-derived growth factor was significantly greater in E6 FLS compared with neo or PS. Apoptosis was studied by incubating FLS with sodium nitroprusside as a source of nitric oxide or hydrogen peroxide for 8 hours and examining DNA fragmentation and E6 cells were significantly less susceptible to cell death. In addition, E6 FLS were more invasive into cartilage extracts than neo or PS using an in vitro cell invasion assay. These data suggest that p53 is a critical regulator of FLS proliferation, apoptosis, and invasiveness. Abnormalities of p53 function might contribute to synovial lining expansion and joint destruction in RA.     

The Chiari-I malformation

We have described the important clinical features and aspects of the Chiari-I malformations, with particular emphasis on Chiari-I malformation. Previously thought to be a rare finding with only minor significance, Chiari-I malformation is an important cause of a variety of symptoms, and will be diagnosed even more frequently as the use of MRI increases. The clinician must consider Chiari-I malformation in any patient with unexplained sensorineural hearing loss, headache, vertigo, ataxia, dysequilibrium, dysphagia or other cranial nerve symptom, especially if accompanied by more classic symptoms of this disorder, such as cervical pain or weakness.     

Erythrokeratodermia Variabilis Present at Birth: Case Report and Review of the Literature

Abstract: A healthy boy had the distinctive lesions of erythrokeratodermia variabilis (EKV) at birth. Twenty-eight patients described in the literature had EKV that presented in childhood. Nine of the 28 were said to have had a rash since birth, but none were distinctive of EKV. To our knowledge this is the first well-documented case describing a child born with the skin manifestations of EKV. We conclude that patients with EKV are infrequently born with a rash, and that only very rarely when the rash is present is it suggestive of the disorder.