Guthrie spots for DNA-based carrier testing in cystic fibrosis.
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Cystic Fibrosis↗
Biomedical subjects
L Weber
Publications and source records attributed to L Weber.
Long-term analysis of organelle translocation in isolated axoplasm of Myxicola infundibulum.
Moving intra-axonal organelles demonstrate frequent variations in speed when viewed over several seconds. To evaluate these and other motion variations, a long-term analysis of organelle motion in isolated axoplasm of Myxicola infundibulum was carried out using differential interference contrast optics and analog and digital image enhancement techniques. Motion characteristics of individual organelles were analyzed for periods of up to 58 minutes. Three principle observations on organelle motion were made: 1) Classes of organelles of the same size demonstrated a 5- to 25-fold variation of speed, with the slowest speeds occurring most frequently; 2) organelle speeds over individual translocations (motion without stopping) are inversely proportional to their size, but the speeds calculated for the long-term analysis of organelle motion (total distance travelled/total observation time, including pauses) did not reflect this observation; and 3) organelles displayed variable trip lengths, durations, mean speeds, and pause durations, and the relationships between these variations showed no repetitive patterns. In contrast to reported observations of uniform velocities of organelles moving on isolated microtubule preparations, these observations suggest that a variety of factors must play a role in organelle translocation in Myxicola axoplasm.
Animals↗
[Sterile eosinophilic pustulosis (Ofuji)].
In recent years there has been an increase in the information available on "eosinophilic pustular folliculitis (Ofuji)". A significant feature of this disease is extensive tissue eosinophilia in association with follicular, intraepidermal or subcorneal pustules containing eosinophils. A review of the current literature reveals that the skin lesions vary widely. Some patients present with pustules on an erythematous base; in others, annular or circinate papulonodular lesions predominate, which may become crusted. Even vesicular lesions have been reported. The extensive tissue eosinophilia itself suggests that the condition is not caused by an exogenous infectious agent. Most data implicate an immunopathological event that is still not well understood.
Biopsy↗
[Yersinia exanthema].
Yersinia is an enterobacterium that causes acute enterocolitis, pseudoappendicitis and sepsis. Some patients suffer from post-infectious immunopathological complications, such as erythema nodosum and yersinia arthritis, which are well known. Less information exists concerning the erythema multiforme-like yersinia exanthema, which is a distinctive dermatological disorder with target lesions localized predominantly on the neck, shoulders and arms. These lesions, which may be smaller but often are larger than the iris lesions in conventional erythema multiforme, tend to coalesce into plaques and reveal a papulovesicular component at their periphery. In our cases the exanthema was associated with a conjunctivitis, especially of the nasal part of the conjunctiva. This exanthema can be induced by yersinia enterocolitica, serotype O-3 and type O-9, at least in Europe, whereas serotype O-8 prevails in North America. The most helpful diagnostic criteria are serological data. However, it is crucial to remember that Widal's agglutination reaction gives rise to high titres, whereas the results of the complement-fixation test are often not reliable.
Adult↗
[Multicenter skin tumor of the interdigitating dendritic cell].
This report concerns a multicentric skin tumor of dendritic cells. Skin-colored to reddish-brown papules and nodules erupted on the right arm of a 68-year-old man and, during the following years, spread to the upper extremities, shoulders, legs and face. Immunohistochemically the large, histiocyte-like tumor cells are Leu-6 (CD1), Leu-3a (CD4), HLA-DR/DQ and S-100 protein positive, but on thorough ultrastructural investigation were found to lack Birbeck granules. Thus, this disease represents a skin tumor of the interdigitating dendritic cell, an accessory cell which, in this case, was associated with a high content of T-lymphocytes within the cutaneous cellular infiltrate. The clinical course is characterized by periodic progression with intermittent, spontaneous regression. During a 7-year follow-up it was not possible to ascertain involvement of either lymph nodes or internal organs.
Aged↗
[Clinicopathologic conference. Spontaneous esophageal rupture (Boerhaave syndrome) in a 41-year-old male].
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Adult↗
Skin tumor of T accessory cells (interdigitating reticulum cells) with high content of T lymphocytes.
A case of T-accessory cell tumor of the skin in a 67-year-old man is reported. The limbs, shoulders, and face were affected, but no visceral involvement is evident 6 years after onset. Tumor cells are nonphagocytic mononuclear cells with folded irregular nuclei. Immunologically, cells were positive for S100 protein, HLA-DR, Ki-M1, Leu 3a (CD4), Leu 6 (CD1); that is, they are identical to the phenotype of Langerhans or interdigitating reticulum cells (IDCs). Birbeck granules were absent. The clinical course appears to be less aggressive than that of the reported IDC sarcomas in other anatomical sites. The similarity of our case to some cases of so-called "non-X histiocytosis" of the skin is discussed. It is suspected that the "non-X histiocytosis of the skin" reported in the literature might have included T-accessory cell tumors, especially those of IDC origin. More immunological studies on the histiocytic disorders of the skin are necessary to clarify their cytogenesis.
Aged↗
Selective induction of human heat shock gene transcription by the adenovirus E1A gene products, including the 12S E1A product.
We have previously shown that the human 70-kilodalton heat shock protein gene (hsp70) is induced by the adenovirus E1A gene product and during the S-G2 phase of the cell cycle. In this study, we investigated the effect of E1A on the expression of other human hsp genes. A gene encoding one form of the hsp89 protein (hsp89 alpha) was activated during an adenovirus infection with kinetics similar to those of activation of hsp70. The induction required a functional E1A gene. However, the hsp89 transcript was not cell cycle regulated. Genes encoding another form of hsp89 and the hsp27 protein were not induced by E1A or during the cell cycle. Further examination of hsp70 expression revealed a greater complexity than previously seen. S1 nuclease analysis using an hsp70 cDNA as well as a distinct hsp70 genomic clone demonstrated three related hsp70 transcripts; two were induced by E1A, and one was not. Both of the E1A-inducible genes were regulated during the cell cycle. All three were induced by heat shock. These results suggest common aspects of control among certain members of this family of cellular genes distinct from heat shock control. Finally, using viruses that express the individual E1A proteins, we found that the hsp70 gene is induced by the 12S and the 13S E1A products. The efficiency of induction by the 12S product was somewhat less than that by the 13S product but only by a factor of less than 2. This is in contrast to the induction of early viral genes, for which the 13S product is considerably more efficient than the 12S product.
Adenovirus Early Proteins↗
Hemodynamic effects of intravenous isosorbide dinitrate and nitroglycerine in acute myocardial infarction and elevated pulmonary artery wedge pressure.
We compared in a randomized fashion the hemodynamic effects of intravenous (IV) isosorbide dinitrate (ISDN) and nitroglycerine (NTG) in 45 patients with acute myocardial infarction and elevated pulmonary artery wedge pressure (Paw). Titration of ISDN dose to lower Paw greater than or equal to 25 percent resulted in a fall of this parameter from 32 +/- 8 to 24 +/- 5 mm Hg and was associated with a fall in mean blood pressure (96 +/- 15 to 90 +/- 14 mm Hg, p less than 0.05), systemic vascular resistance (1715 +/- 572 to 1548 +/- 414 dynes X s X cm-5, (p less than 0.05), pulmonary vascular resistance (182 +/- 106 to 154 +/- 78 dynes X s X cm-5, p less than 0.05) and mean right atrial pressure (11 +/- 4 to 7 +/- 4 mm Hg, p less than 0.05). In addition, ISDN significantly (p less than 0.05) increased cardiac index from 2.37 +/- 0.54 to 2.54 +/- 0.59 L/min/m2, stroke volume index from 28 +/- 8 to 31 +/- 8 ml/m2, and stroke work index from 28 +/- 11 to 31 +/- 12 g X m/m2. The ISDN dose ranged from 50 to 533 micrograms/min (mean +/- SD 326 +/- 176 micrograms/min) and could not be predicted from baseline hemodynamic values. A comparison between the effect of ISDN and NTG in doses producing comparable reduction in Paw showed similar hemodynamic changes. It was concluded that IV ISDN in patients with elevated mean pulmonary artery wedge pressure due to acute myocardial infarction results in a decrease in right and left ventricular preload and afterload and improvement of cardiac output and cardiac work. The effective dose ranges from 50 to 533 micrograms/min and cannot be predicted from baseline hemodynamic values. In doses producing comparable reduction in Paw, ISDN and NTG had similar hemodynamic effects.
Aged↗
The hand that rocks the cradle.
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Contraception↗
Effects of nifedipine on hemodynamics and cardiac function in patients with normal left ventricular ejection fraction already treated with propranolol.
The interaction between nifedipine and propranolol on cardiac hemodynamics and function was investigated in 9 patients with normal left ventricular (LV) function who were undergoing cardiac catheterization for complaints of chest pain. Only 2 patients had angiographic evidence of significant coronary artery disease but no patient had clinical evidence of ischemia during the study. All patients were pre-treated with propranolol, 30 to 320 mg/day (mean +/- standard deviation 210 +/- 122); the propranolol serum level ranged from 43 to 246 ng/ml (mean 203 +/- 62). The administration of nifedipine resulted in a decrease in blood pressure (from 94 +/- 11 to 85 +/- 13 mm Hg, p less than 0.05), increase in heart rate (from 59 +/- 6 to 65 +/- 7 beats/min, p less than 0.05), and an increase in both mean right atrial and mean pulmonary artery wedge pressures (from 8 +/- 3 to 9 +/- 3 mm Hg and from 13 +/- 3 to 14 +/- 4 mm Hg, respectively, both p less than 0.05). Cardiac index increased (from 2.3 +/- 0.3 to 2.7 +/- 0.2 liters/min/m2, p less than 0.01). Stroke volume index also increased significantly (from 39 +/- 5 to 43 +/- 6 ml/m2) and systemic vascular resistance decreased (from 1,715 +/- 369 to 1,255 +/- 271 dynes s cm-5, p less than 0.01). No significant change was noted in pulmonary vascular resistance (148 +/- 94 vs 140 +/- 62 dynes s cm-5), LV stroke work index (44 +/- 9 vs 42 +/- 10 g-m/m2), LV end-diastolic pressure (15 +/- 2 vs 16 +/- 2 mm Hg).(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Adjuvant chemotherapy in advanced stomach cancer].
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Adult↗
Cell culture studies on neurofibromatosis (von Recklinghausen's disease). IV. Collagen synthesis.
Comparative measurements of collagen synthesis as a portion of total protein synthesis were performed with fragments of peripheral neurofibromas, of skin adjacent to the tumors, and of unaffected skin from patients with neurofibromatosis (NF). No significant difference was found among these various samples. Collagen synthesis was also measured in cell cultures derived from peripheral neurofibromas of eight NF patients and from skin of ten healthy donors. No differences observed in the following respective parameters: dependence on the concentration of fetal calf serum; dependence on cell population density; portion of synthesized collagens in the culture medium and the cell layer. The ratios of synthesized collagen types III to type I, determined in five pairs of NF and control strains, vary within the same range.
Cell Count↗
Neurohumoral consequences of vasodilator therapy with hydralazine and nifedipine in severe congestive heart failure.
We evaluated the effects of intravenous hydralazine (5 to 30 mg) and oral nifedipine (20 to 80 mg) on plasma catecholamines, renin, and aldosterone in 18 patients with severe chronic heart failure. Both drugs resulted in a significant decrease in systemic vascular resistance and mean systemic blood pressure, and led to an increase in cardiac output. Baseline plasma norepinephrine concentration was elevated in most patients; however, augmentation of cardiac output with both drugs did not decrease the values of this hormone (from 870 +/- 128 to 946 +/- 161 pg/ml with hydralazine and from 1088 +/- 260 to 1106 +/- 187 pg/ml with nifedipine). Plasma epinephrine level was also elevated at baseline and did not change significantly following nifedipine therapy (164 +/- 44 vs 199 +/- 54 pg/ml), but increased in most patients following the administration of hydralazine (from 105 +/- 45 to 153 +/- 27 pg/ml, p less than 0.01). The renin-aldosterone system was activated in our patients and also demonstrated a different response to both drugs. Hydralazine therapy did not change either the plasma renin concentration (30 +/- 7 vs 28 +/- 7 ng/ml/hr) or the aldosterone level (24 +/- 7 vs 22 +/- 5 ng/dl). In contrast, nifedipine increased the plasma renin concentration (22 +/- 7 to 29 +/- 8 ng/ml/hr, p less than 0.05). This change did not correlate with changes in systemic blood pressure (r = 0.03) and was probably the result of previously shown calcium blockade-mediated stimulation of renin release from the juxtaglomerular cells of the kidney.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Thalidomide and lymphocyte function.
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Humans↗
Involvement of the 5'-leader sequence in coupling the stability of a human H3 histone mRNA with DNA replication.
Two lines of evidence derived from fusion gene constructs indicate that sequences residing in the 5'-nontranslated region of a cell cycle-dependent human H3 histone mRNA are involved in the selective destabilization that occurs when DNA synthesis is terminated. The experimental approach was to construct chimeric genes in which fragments of the mRNA coding regions of the H3 histone gene were fused with fragments of genes not expressed in a cell cycle-dependent manner. After transfection in HeLa S3 cells with the recombinant plasmids, levels of fusion mRNAs were determined by S1 nuclease analysis prior to and following DNA synthesis inhibition. When the first 20 nucleotides of an H3 histone mRNA leader were replaced with 89 nucleotides of the leader from a Drosophila heat-shock (hsp70) mRNA, the fusion transcript remained stable during inhibition of DNA synthesis, in contrast to the rapid destabilization of the endogenous histone mRNA in these cells. In a reciprocal experiment, a histone-globin fusion gene was constructed that produced a transcript with the initial 20 nucleotides of the H3 histone mRNA substituted for the human beta-globin mRNA leader. In HeLa cells treated with inhibitors of DNA synthesis and/or protein synthesis, cellular levels of this histone-globin fusion mRNA appeared to be regulated in a manner similar to endogenous histone mRNA levels. These results suggest that the first 20 nucleotides of the leader are sufficient to couple histone mRNA stability with DNA replication.
Animals↗
Modulation of collagen type synthesis in organ and cell cultures of fibroblasts.
Fibroblast cultures are widely used to study abnormalities of collagen metabolism in both inborn and acquired diseases. However, there is reason to question the extent to which the experimental information obtained from in vitro culture systems in fact reflects the in vivo situation. In the present study we analyzed the proportions of collagens I and III synthesized by human and mouse skin fibroblasts maintained under various culture conditions. The amount of type III collagen extracted from skin specimens was lower than that which was newly synthesized in organ culture. Cells obtained by enzymatic disintegration of skin specimens synthesized more type III collagen than fibroblasts grown from explants. However, subcultivation of the enzymatically liberated cells resulted in a continuous decline of type III collagen production which eventually reached levels similar to those observed in explant cultures.
Animals↗
Hemodynamic and hormonal effects of high-dose transdermal nitroglycerin in patients with chronic congestive heart failure.
The hemodynamic effect of a large dose of nitroglycerin (NTG) (90 mg) given transdermally using a reservoir system was studied in 10 patients with severe, long-standing congestive heart failure. Serial hemodynamic measurements over 24 hours revealed a mild decrease in mean pulmonary artery wedge pressure. However, the change from baseline was significant only at 2 hours (19 +/- 9 vs 27 +/- 6 mm Hg). Mean right atrial pressure fell 1 hour after initiation of therapy, from 12 +/- 7 to 8 +/- 5 mm Hg. However, the change from control was not statistically significant. No significant changes were noted in heart rate, mean blood pressure, cardiac index, and systemic and pulmonary vascular resistance. Individual analysis of the effect of transdermal NTG on pulmonary artery wedge pressure demonstrated at 20% or greater reduction in 8 of 10 patients. However, persistent effect (longer than 8 hours) was seen in only 4 patients. Removal of NTG patches at 24 hours did not result in hemodynamic rebound. Serum catecholamine levels and renin concentration did not change 2 hours and 24 hours after initiation of NTG therapy or after removal of NTG patches. Thus, a large dose (90 mg) of transdermal NTG using a reservoir system results in mild and mostly statistically insignificant hemodynamic effect in patients with chronic severe congestive heart failure. Although a reduction in pulmonary artery wedge pressure is seen in most patients, rapid attenuation of this response is found in many patients and the effect only rarely lasts for 24 hours.(ABSTRACT TRUNCATED AT 250 WORDS)
Administration, Topical↗