Impact of activated protein C resistance on general vascular surgical patients

Purpose: The prevalence of activated protein C resistance (APCR) and associated thrombotic morbidity among patients who undergo arterial reconstruction were investigated.Methods: Preoperative assays for functional APCR and factor V (Leiden) mutation were performed on 262 patients who underwent arterial reconstructions that consisted of cerebrovascular surgery (109), aortic or iliofemoral procedures (76), or infrainguinal bypass procedures (77). Patients were monitored for thrombotic complications during the postoperative period.Results: Depending on the stringency of the definition used, functional APCR was detected in 10.6% to 22.0% of patients tested. Factor V (Leiden) was found in 5.3% of patients. Thrombotic morbidity consisting of myocardial infarction, cerebrovascular event, or graft thrombosis occurred in 9.9% of patients, who were followed-up for a mean of 4.8 months. No significant overall correlations were found between APCR and thrombotic morbidity. Subgroup analysis revealed significant associations between functional APCR and total early postoperative thrombotic complications and early graft failure, and between factor V (Leiden) and early cerebrovascular events and late graft thrombosis (p < 0.03).Conclusions: Functional APCR is somewhat more prevalent among general vascular surgical patients than in the general population, but factor V (Leiden) is no more prevalent. APCR is not a prominent cause of thrombotic morbidity in contemporary vascular surgery. Nonetheless, it is a sufficiently important potential contributor to morbidity among some subgroups to warrant selective testing and directed therapy pending further study. (J Vasc Surg 1997;25:1054-60.)     

Psychological and family functioning and quality of life in adolescents with cystic fibrosis.

BACKGROUND: The life expectancy of individuals with CF has increased to 33 years. Thus, issues such as quality of life and psychological well-being, previously thought to be of lesser importance than physical well-being, are now recognised as significant factors. This study examined the interrelationships between quality of life, family functioning, individual psychopathology and optimism of adolescents with CF. METHODS: Adolescents attending the CF clinic completed a number of questionnaires. Quality of Life was measured using the Cystic Fibrosis Questionnaire, family functioning by the Family Environment Scale (3rd edition), general psychopathology with the Symptom Checklist-90-Revised and optimism for the future by the Hunter Opinions and Personal Expectations Scale. Disease severity was assessed using the Shwachman score and spirometry at the time of questionnaire completion. RESULTS: The level of psychopathology (12.5% of those 13 years and over) in the group was lower than that reported for young people in Australia (15-20%). The results indicated that young people with a delayed diagnosis and those who are alienated from their families may be in need of additional psychosocial support. The group was hopeful and positive about their future and these attributes were independent of clinical measures of disease severity. In general, these young people scored relatively highly on the quality of life scale. For example the mean standardised score for physical functioning was 70 points, for respiratory symptoms was 63 points and for emotional state was 78 points. Increased levels of psychopathology and lack of hope for the future were however associated with lower ratings on a number of quality of life measures. Family cohesiveness, expressiveness and organization were associated with better psychological functioning in the young people. CONCLUSIONS: Adolescents with CF appear to be a psychologically well functioning and well-adjusted group. These findings support the importance of a more sophisticated model of well-being for adolescents with CF, which explores the young person's views on their quality of life and wider support frameworks rather than relying solely on measures of physical health to gauge well-being.     

CADASIL or CADVaSIL?

In the present study, morphological examination of patients from two unrelated Polish families with CADASIL was performed. Using light microscopy, there were evident changes characteristic to the disease. On electron microscopy, deposits of granular osmiophillic material (GOM) were found not only in cerebral arteries and veins but also in cerebral capillaries and vessels of the internal organs. These findings indicate that pathological process in CADASIL is generalized and involves also small vessels devoid of smooth muscle cells. Therefore, we propose to consider a replacement for the name CADASIL that better reflects the morphological picture of the disease like, for example, cerebral autosomal dominant vasculopathy with subcortical infarcts and leukoencephalopathy (CADVaSIL) or, to preserve the commonly known acronym, cerebral autosomal dominant angiopathy with subcortical infarcts and leukoencephalopathy.     

Lysogeny and lysosensitivity inShigella dysenteriae Group of bacteria

Shigella dysenteriae 7 (D7) strain was extracted by using the Westphal water/phenol method and the extract obtained (Aq/Ph 68?C) was found to have the receptor activity against both T2 and T4 phages. It was shown by the receptor neutralization test that the examined substance had receptor sites distinctive for T2 and T4 phages. The receptor sites for phage T2 had common antigen specificity with receptor sites of this phage localized inE. coli B in the lipoprotein fraction; the receptor sites of phage T4 had common antigen specificity with receptor sites to phage T4, localized inE. coli B in Aq/Ph 68? C extract. It is suggested that in spite of the different localization, the receptors for the same phage in the different bacterial species examined have common serological specificity.     

Lymph Node Mesenchymal and Endothelial Stromal Cells Cooperate via the RANK-RANKL Cytokine Axis to Shape the Sinusoidal Macrophage Niche

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Lysogeny and lysosensitivity inShigella dysenteriae group of bacteria

The temperate phage receptor substances were tested from two serologically related bacterial strains,Sh. dysenteriae 2 andSh. boydii 15. It was shown that the protein component of somatic antigen was responsible for phage receptor activity and that this component possessed common antigen properties.     

Subcutaneous estradiol pellets for endometrial preparation in donor oocyte recipients with a poor endometrial response

Purpose: The purpose of this study was to evaluate the clinical effectiveness of subcutaneous estradiol pellets in donor oocyte recipients with an inadequate endometrial response. Methods: The subjects were 13 women with ovarian failure and a maximal endometrial thickness <10 mm on standard estrogen regimens, as demonstrated during mock and/or prior oocyte donation cycles. They underwent pellet implantation (100–250 mg of estradiol) 6–13 weeks before oocyte donation. Results: Maximal (mean ± SD) endometrial thickness was 8.7±1.5 mm on standard regimens, in contrast to 11.7± 1.8 mm on pellets, while estradiol levels were 674±844 and 815±706 pg/ml, respectively. The estradiol:estrone ratio on pellets was >1. There was 1 pregnancy with early loss during 10 cycles on other estrogen regimens and 12 pregnancies during 19 cycles on pellets. The pregnancy and implantation rates were, respectively, 63 and 27% on pellets and 41 and 14% on standard regimens in historical controls. Conclusions: We conclude that estradiol pellets after a single administration provide constant estradiol levels extending into the first trimester of pregnancy, a physiologic estradiol:estrone ratio, and a better endometrial response than standard estrogen regimens. Implantation and pregnancy rates are higher. This approach may be especially suitable for recipients with a poor endometrial response. Presented at the IXth World Congress on In Vitro Fertilization and Assisted Reproduction, Vienna, Austria, April 3, 1995, and the 51st Annual Meeting of the American Society for Reproductive Medicine, Seattle, Washington, October 7–12, 1995.     

Molecular ontogeny of donor-derived follicular lymphomas occurring after hematopoietic cell transplantation

The relative timing of genetic alterations that contribute to follicular lymphoma remains unknown. We analyzed a donor-recipient pair who both developed grade 2/3A follicular lymphoma 7 years after allogeneic transplantation and donor lymphocyte infusions. Both patients harbored identical BCL2/IGH rearrangements also present in 1 in 2,000 cells in the donor lymphocyte infusion, and the same V(D)J rearrangement, which underwent somatic hypermutation both before and after clonal divergence. Exome sequencing of both follicular lymphomas identified 15 shared mutations, of which 14 (including alterations in EP300 and KLHL6) were recovered from the donor lymphocyte infusion by ultra-deep sequencing (average read coverage, 361,723), indicating acquisition at least 7 years before clinical presentation. Six additional mutations were present in only one follicular lymphoma and not the donor lymphocyte infusion, including an ARID1A premature stop, indicating later acquisition during clonal divergence. Thus, ultrasensitive sequencing can map clonal evolution within rare subpopulations during human lymphomagenesis in vivo. SIGNIFICANCE: For the first time, we define the molecular ontogeny of follicular lymphoma during clonal evolution in vivo. By using ultrasensitive mutation detection, we mapped the time-course of somatic alterations after passage of a malignant ancestor by hematopoietic cell transplantation.     

Hodgkin Lymphoma after Disseminated Mycobacterium genavense Infection,Germany

Mycobacterium genavense infection, a rare nontuberculous mycobacteria infection, occurs in heavily immunocompromised patients (i.e., those with advanced HIV disease, genetic disorders, or acquired immunologic disorders and those undergoing immunosuppressive therapy). We report a case of disseminated M. genavense infection preceding Hodgkin lymphoma in a patient without obvious risk factors for this infection.