Trisomy 2 in proliferative fasciitis.
We report trisomy 2 as the sole clonal karyotypic abnormality in a case of proliferative fasciitis. To our knowledge, this is the first cytogenetic report of proliferative fasciitis.
Aged↗
Biomedical subjects
C Berger
Publications and source records attributed to C Berger.
Isolation of a yeast artificial chromosome clone that spans the (12;16) translocation breakpoint characteristic of myxoid liposarcoma.
Cytogenetic analysis of liposarcomas has demonstrated that translocation (12;16) (q13.3;p11.2) is characteristic of the myxoid subtype of this adipose tissue tumor. Our previous results suggested that the GLI gene is close to the translocation breakpoint on chromosome 12. We now describe a yeast artificial chromosome (YAC) that contains GLI and spans the chromosome 12 region involved in the t(12;16) breakpoint. This clone will permit rapid definition of the genetic region surrounding the breakpoint and allow isolation of the gene presumably affected by the translocation.
Chromosomes, Fungal↗
Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy.
BACKGROUND: This original cohort of patients with erythrodermic cutaneous T-cell lymphoma (CTCL) was reported to have clinical improvement with photopheresis during the 12 months of the original study. No long-term follow-up data have been available to examine the impact of this therapy on the disease. OBJECTIVE: Our purpose was to provide long-term follow-up on the original 29 erythrodermic CTCL patients treated with photopheresis and to compare these results with historical controls. METHODS: Files of patients from the original photopheresis study centers were reviewed and their current status was documented. RESULTS: The median survival of the treated patients was 60.33 months from the date of diagnosis and 47.9 months from the date of the start of photopheresis therapy. A complete remission has been maintained in four of the six patients who achieved complete responses in the original study. The best responses were seen in patients with a lower CD4/CD8 ratio in the peripheral blood at the start of therapy. CONCLUSION: Photopheresis can influence the natural history of erythrodermic CTCL by inducing remissions and prolonging survival with minimal toxicity.
CD4-CD8 Ratio↗
Lymphocyte xenoantigens recognized by preformed antibodies.
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Animals↗
Viral-specific immunization in AIDS-related complex by photopheresis.
The potential for therapeutic intervention in 7 patients with AIDS-related complex (ARC) was evaluated through the use of photopheresis. The rationale for the study was based on: 1. the demonstration that psoralen and UVA could inactivate HIV/virus in vitro; 2. CD4 cells are the primary target population effected by HIV and photopheresis; and 3. reinfusion of inactivated virus and cell-associated virus might serve to engender an immune response. Preliminary results in 7 patients with ARC over 6 to 18 months revealed a virus-specific response with an elevation of HIV antibodies, while EBV and CMV titers remained unchanged. The immunologic results revealed an increase in the CD8 lymphocyte population, stable activation markers (B2 microglobulin neopterin), a decrease in p24 antigen titers and inability to culture HIV virus in 3 patients. All of these results were in the context of a stable or increasing CD4+ percent. Six patients did not reveal a generalized inhibition of other immune responses as demonstrated by recovery of DTH. In addition, the resolution of lymphadenopathy, night sweats, fever and weight loss, paralleled the immunologic response.
AIDS-Related Complex↗
Anti-IgM antibodies down modulate mu-enhancer activity and OTF2 levels in LPS-stimulated mouse splenic B-cells.
Stimulation of small, resting, splenic B cells with bacterial lipopolysaccharide (LPS) induces proliferation, differentiation to plasma cell formation, and the expression of immunoglobulin heavy chain (IgH). When this is combined with agents which crosslink surface Ig, differentiation and the induction of surface immunoglobulin are suppressed even though proliferation proceeds. We find that anti-mu antibodies suppresses Ig gene expression of transfected mu constructs, even if either the membrane or secretory segments have been deleted. We examined the effects of anti-mu treatment on the IgH enhancer (IgHE) attached to a heterologous test gene (CAT). Indeed the IgH enhancer alone was subject to anti-mu suppression, while the SV40 enhancer was insensitive. To determine what was responsible for suppression of enhancer function by anti-mu we examined nuclear extracts from stimulated splenic B cells for the presence of sequence-specific DNA binding activities to various sites within the enhancer. We found two specific differences--an induction in mu E5 binding activity, and a reduction in octamer transcription factor 2 (OTF2) binding activity, after anti-mu treatment. Analysis of these cells by in situ immunofluorescence with anti-OTF2 antibodies suggests that the nuclear localization of OTF2 in anti-mu treated cells may change, as well as its absolute level.
Animals↗
Nonradioactive in situ hybridization. A rapid approach for the identification of marker chromosomes: study of a case of acute leukemia with a Yq specific DNA probe.
Nonradioactive in situ hybridization provides a rapid method for detecting specific nucleic acid sequences. In this study of a patient with acute leukemia, we applied in situ hybridization for identification of a marker chromosome and determination of the number of copies of this marker in interphase nuclei using a biotinylated Yq-specific DNA probe (pY3.4). We show that nonradioactive interphase in situ hybridization can be a useful method for karyotypic analysis in addition to routine cytogenetic techniques in neoplastic disorders.
Acute Disease↗
Essential fatty acid composition of human colostrum triglycerides: its relationship with adipose tissue composition.
The relationships between essential fatty acid (EFA) composition of colostrum and white adipose tissue (WAT) were examined on day 5 after delivery in 69 healthy women. Fatty acid composition was assessed by capillary gas chromatography, and 33 fatty acids were detected in colostrum and in WAT. Total polyunsaturated fatty acid (PUFA) content was similar in colostrum and in WAT (15.7 +/- 3.1% and 16.1 +/- 3.8%, respectively), but long-chain PUFA content was higher in colostrum than in WAT (2.9 +/- 0.6% and 1 +/- 0.2%, respectively; P less than 0.001). The concentrations of linoleic acid were significantly correlated between colostrum and WAT (r = 0.77, P less than 0.0001). No correlation was found for alpha-linolenic acid. The relationships between long-chain PUFA composition of colostrum and WAT suggested that individual factors along with tissue specificity of the mammary gland are involved in either the capacity of desaturating and chain-elongating pathways and/or incorporation of long-chain PUFAs into colostrum.
Adipose Tissue↗
Maternal-infant separation at birth among substance using pregnant women: implications for attachment.
The potential for substance use to interfere with initial maternal-infant contact and attachment was examined. Data were collected from confidential interviews with pregnant women and reviews of the medical charts of these women and their infants. Substance users (N = 60) were found to experience significantly (p = .03) more maternal-infant separation at birth than nonusers (N = 22), most often due to health concerns for mother and/or infant. Substance using mothers scored negatively more frequently than nonusing mothers on variables of attachment. Reasons for these differences are explored and some strategies for dealing with this population in the hospital milieu are suggested.
Female↗
Serum concentrations of immunoglobulins and of antibody isotypes in bone marrow transplant recipients treated with high doses of polyspecific immunoglobulin or with cytomegalovirus hyperimmune globulin.
The kinetics of immunoglobulins (Ig) and antibodies were followed in 10 bone marrow transplant recipients who received either high doses (0.5 g/kg body weight) of polyspecific intravenous Ig (HD-IVIG) weekly or cytomegalovirus hyper-Ig (CMV-IVIG, 0.1 g/kg body weight) every 3 weeks. In the HD-IVIG group, the mean total IgG concentration more than tripled and similar significant increases were seen for IgG1 and IgG2. IgG antibodies to CMV showed a marked increase in the HD-IVIG and a less pronounced rise in the CMV-IVIG group. IgM antibodies to CMV were present initially or became detectable in five patients, unrelated to the IVIG preparation. HD-IVIG induced a significant increase of IgG antibodies to streptococcal group A carbohydrate (A-CHO) and to smooth strain lipopolysaccharides (LPS) but not of antibodies against lipid-A. When the Ig treatment was discontinued, levels of total IgG and of IgG antibody to CMV decreased with an apparent half-life of 30 days. Both IVIG preparations were well tolerated and had no negative feedback on total Ig and on specific antibody production or other antimicrobial defence mechanisms. In patient nos. 4 and 10 who developed severe graft-versus-host-disease, transient serum Ig peaks including several Ig isotypes appeared after day 14. In patient no. 10 this peak contained an IgG antibody to H. influenzae type b (Hib), and IgM antibodies to CMV, Hib, A-CHO and LPS. This study clearly shows that serum concentrations of Ig isotypes, subtypes and specific antibodies, depend on at least four factors: total amount and composition of Ig infused, consumption, catabolism and endogenous production.
Adult↗
Current status of whole-body counting as a means to detect and quantify previous exposures to radioactive materials. Whole-body Counting Working Group.
This report discusses the principles, techniques, and application of whole-body counting with respect to previous radiation exposure. Whole-body counting facilities are located nationwide and have a wide range of capabilities. A listing of these facilities is provided in Appendix A. However, only a few facilities are truly state-of-the-art and have the sophisticated capabilities required to attempt detection of low-level activity in vivo. Measurements made many years after exposure can be extremely difficult to interpret. The precision and accuracy of resulting dose estimates are functions of such factors as the assumptions made concerning intake, time since intake, radionuclide metabolism, and level of intake. The indiscriminate application of metabolic models to current body contents or minimum detectable amounts of radionuclides with relatively short effective half-lives (such as 137Cs) can lead to absurd results when used as a basis for calculating intakes 25 and 40 y ago. Skull counting for 90Sr-90Y and 239,240Pu can set upper limits on possible uptakes and radiation doses, but in the case of 239,240Pu, the limits are rather high. In both cases, the accuracy of the limits depends on the metabolic models used in the calculations. These models (ICRP 1979) were developed to set safety standards for the intakes of radionuclides by workers and are not intended to be used to back-calculate uptakes and radiation doses from measurements made long after the uptake. There are, therefore, large uncertainties in any conclusions derived from these calculations. The experience gained over the years with whole- and partial-body counting has consistently shown that they are of little use in determining body contents of radionuclides resulting from exposure to weapons debris decades earlier. The development of new detectors such as an array of lithium-drifted silicon devices offers some hope of lowering the minimum detectable amount (MDA) for Pu and Am, but such detectors are still several years from routine application and do not represent current state-of-the-art. Furthermore, it is doubtful that such improvements will be sufficient to meet the need of assessing radiation exposures that occurred decades earlier.
Accidents↗
[Spontaneous rupture of the membranes before 28 weeks of amenorrhea. Obstetrical and perinatal outcome. Apropos of 28 cases].
The authors studied the outcome of pregnancies after spontaneous rupture of the membranes before 28 weeks of amenorrhoea in a series of 28 women. The mean time of the rupture was at 25 1/2 weeks. The duration of the time of rupture was a mean of 10 days and deliveries occurred between the 19th and the 32nd weeks. In 5 cases with an average term of 22 1/2 weeks the pregnancy was terminated. Caesarean operation was carried out in 4 cases and spontaneous vaginal delivery occurred between the 26th and 30th weeks of amenorrhoea in 19 cases. Chorio-amnionitis was the most common maternal complication (32.1%). Maternal morbidity was high (82%) without important sequelae. Thirty infants were born out of these 28 pregnancies (two sets of twins). 33% of the infants were stillborn, 46.6% died in the neonatal period, all of these from respiratory complications. 20% of the children are still alive, and all of these weighed at least 1,000 grams and were born after 28 weeks. A child that was born at 27 weeks amenorrhoea has serious neurological deficits. Having studied the bibliography on the subject and their own personal experience the authors suggest a routine to carry out in these cases. Although the prognosis is poor, the totality of these factors could encourage obstetrical teams to be conservative so long as they watch very carefully under well defined conditions.
Adolescent↗
Extracorporeal photopheresis in the treatment of AIDS-related complex: a pilot study.
OBJECTIVE: To determine side effects of extracorporeal photopheresis in the treatment of patients with the acquired immunodeficiency syndrome (AIDS)-related complex, and to gain early evidence of efficacy of the treatment. DESIGN: Uncontrolled trial. SETTING: Tertiary referral center. PATIENTS: Five patients with AIDS-related complex: three homosexual men and one man and one woman with histories of intravenous drug abuse. One patient, a homosexual man, withdrew from therapy after 5 months but returned for monthly clinical and laboratory evaluations. INTERVENTION: Monthly treatments with extracorporeal photopheresis. MEASUREMENTS AND MAIN RESULTS: Symptoms resolved in four patients. Lymphadenopathy disappeared in all five. Four patients had delayed-hypersensitivity reactions to skin testing (as defined by the Walter Reed staging classification). All showed increases in p24 and gp120 antibody levels. The CD4-cell percentage increased in four patients and declined in one after 6 months of therapy, but the absolute CD4 count decreased in two patients. At 15 months, the CD4 percentage remained at or increased over the baseline value in three patients still in the study but decreased in one. Levels of Beta 2-microglobulin decreased or remained stable in four patients. All patients were culture positive for the human immunodeficiency virus (HIV) before treatment. One patient had a negative viral culture after 5 months of treatment with confirmation. Two other patients became HIV culture negative, one at 14 and one at 15 months: The former patient became positive at 15 months and the latter patient remained negative at 16 months. CONCLUSIONS: The preliminary results suggest that extracorporeal photopheresis deserves further evaluation as therapy for AIDS-related complex.
AIDS-Related Complex↗
Coronary vasomotor tone in large epicardial coronary arteries with special emphasis on beta-adrenergic vasomotion, effects of beta-blockade.
Changes in coronary vasomotor tone of large epicardial coronary arteries today can be assessed quite accurately by exact measurements of coronary diameters applying computer assisted systems. The effect of various vasodilators (nitrates, calcium antagonists, EDRF-dependent compounds) was tested in this way. It appears that normal coronary artery segments reach a maximum of dilator reserve with an increase of luminal diameter of approximately 30-40%; different patterns of kinetics were, however, encountered. beta-Blocking agents, both non-selective (propranolol) and selective (atenolol), were found to lead to a gradual vasoconstriction, i.e., a decrease in diameter by approximately 20-25% over 20 min, an effect which is overcome by nitrates. New beta-blocking compounds with vasodilator properties, such as celiprolol, show no constriction. The vasoconstrictor effect of propranolol and atenolol may not only be due to the decrease of flow following the drop in myocardial oxygen consumption, but could also reflect an unopposed alpha-adrenergic tone. The clinical aspects of this observation are discussed.
Adrenergic beta-Antagonists↗
Chromosomes 17 and 22 involved in marker formation in neurofibrosarcoma in von Recklinghausen disease. A cytogenetic and in situ hybridization study.
We describe the cytogenetic findings in a recurrent neurofibrosarcoma in a patient with nonfamilial von Recklinghausen disease. The composite karyotype was: 40,Y,-X,+dic r(X;20)(:Xp22.2----q26::20p13----q13:), -1, +der(1)t(1;3) (p21;p24),-3,-4,-5,+der(5) t(5;?)(q31;?),-9,-9,+der(9)t(3;9)(q21 or q13;p24 or p22), -11,+der(11)t(11;?)(q22.2;?), -17,+der(17)t(17; 22;?)(q21;q13.1;?), -20, -21, -22, -22, +der(22)t(17; 22;?)(q21;q13.1;?),t(2;10)(q37;q22). The derivative chromosomes were demonstrated at the 500 band level. Chromosomes 17 and 22 were shown to be involved in an unbalanced three-way translocation: t(17;22;?)(q21;q13.1;?). This event was confirmed by in situ hybridization, using two probes mapped to chromosome 17. Hill H is a probe derived from the novel oncogene TRE and is located at 17q12-22. The second probe, derived from the granulocyte colony-stimulating factor (G-CSF), is located at 17q11-q21. The rearrangement between chromosomes 17 and 22 showed breakpoints similar or close to the gene loci for neurofibromatosis 1 (NF-1) and NF-2. Based on our observations we recommend that genetic studies on NF-1 tumors include both gene sites (NF-1 and NF-2) rather than focus on one gene locus.
Adult↗
Phototoxic liposomes coupled to an antibody that alone cannot modulate its cell-surface antigen kill selected target cells.
Molecules such as antibodies that bind to cell surfaces can be used to deliver cytotoxic drugs to selected cells. To be effective the drug must usually be taken into the cells by endocytosis. In this study a T-cell line (CCRF-CEM) was effectively killed by liposomes carrying a photosensitizer and bearing the antibody OKT4 (anti-CD4). The unconjugated antibody does not induce antigenic modulation in the target cells, an indication of the absence of endocytosis, and would therefore not normally have been selected as an agent for drug delivery. It cannot, however, be concluded with certainty that the conjugates act at the cell surface and several alternative explanations of their efficacy are offered.
Animals↗
Photopheresis for the treatment of cutaneous T cell lymphoma.
We investigated the use of extracorporeal chemotherapy (photopheresis) in eight patients with cutaneous T cell lymphoma. Initially described by Edelson et al. for the treatment of erythrodermic cutaneous T cell lymphoma, we have expanded the treatment to include patients with extensive patch/plaque disease as well as tumor-stage disease. Four of five patients with erythrodermic stage disease had either a complete or a partial clinical remission with photopheresis alone. One patient with extensive patch/plaque disease continued to have a partial clinical remission of 7 months' duration with photopheresis alone. Of the two patients with tumor-stage disease, one remained without evidence of clinical disease at 10 months with photopheresis alone, whereas the second patient had a partial clinical remission of 5 months with a combination of local radiation therapy followed by monthly photopheresis. The skin biopsy specimen obtained from the patient with tumor-stage disease in complete clinical remission did not show cutaneous T cell lymphoma. We conclude that photopheresis is an effective modality alone or in combination with adjunctive therapy for erythroderma, extensive patch/plaque disease, and some tumor-stage disease.
Adult↗