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Biomedical subjects

J Wilson

Publications and source records attributed to J Wilson.

At least 487 records · Page 27Linked to original sources

Successful therapy of metastatic embryonal carcinoma coexisting with gonadoblastoma in a patient with 46,XY pure gonadal dysgenesis (Swyer's syndrome).

A case of gonadoblastoma associated with metastatic embryonal carcinoma occurring in a phenotypic female with 46,XY pure gonadal dysgenesis is presented. Two years after treatment with surgery and combination chemotherapy the patient is disease free. This is the third reported case and first reported cure in the English-language literature over the past 25 years. Current management is reviewed.

Adolescent↗

Free and total lidocaine levels in cardiac surgical patients.

A lidocaine bolus of 2 mg/kg has been shown to decrease the incidence of ventricular fibrillation in cardiac surgical patients during reperfusion following aortic cross-clamp release (CCR). In an effort to extend this effect into the immediate postoperative period, many centers routinely administer a prophylactic lidocaine infusion for the first 12 to 24 hours after surgery. Sequential total serum lidocaine levels were measured over 6 hours in 28 adult cardiac surgical patients without liver, kidney, or respiratory disease, and free serum lidocaine levels were measured in 15 of these patients. In addition, pharmacokinetic data were collected and analyzed in 8 of these patients. Each patient received a lidocaine bolus of 1.5 mg/kg and an infusion at 2 mg/min immediately before CCR. Serum levels were determined just before CCR, and at 10, 20, 30, 60, 120, 240, and 480 minutes after CCR. More than 50% of patients had subtherapeutic total serum lidocaine levels from 20 to 120 minutes after CCR, but free lidocaine levels remained within the therapeutic range. No patient showed malignant ventricular dysrhythmias during the study. The results suggest that (1) it may be misleading to estimate free lidocaine concentration based on total lidocaine levels; and (2) free drug is the moiety responsible for lidocaine's antiarrhythmic effect.

Adrenergic beta-Antagonists↗

The urea cycle in the Rett syndrome.

The in vivo functioning of the urea cycle in the Rett syndrome (RS) was investigated using alanine load test. The test was carried out in girls with RS and their mothers. These results were compared with those on normal females, males and obligate carriers for ornithine carbamoyltransferase deficiency. Post load hyperammonaemia was not seen in any of the RS girls who were not on medication (valproate). Increased orotate excretion was found in some of the RS girls and mothers. The pattern of urinary excretion of orotate in RS girls and their mothers after a standardised alanine load was similar to that found in female carriers of OCT deficiency. This finding can be explained by a mutant OCT, subject to the Lyon effect, and linked to the gene abnormality in RS or by the presence of an abnormal X-linked mitochondrial protein synthesised in cytoplasma and positioned in close proximity to OCT. Such a protein abnormality could also affect mitochondrial functions in other cells, e.g. in the developing brain.

Adult↗

Effects of lovastatin in diabetic patients treated with chlorpropamide.

Patients with non-insulin dependent diabetes mellitus (NIDDM) have a higher risk of atherosclerotic cardiovascular disease than nondiabetic subjects. In seven patients with both hypercholesterolemia and NIDDM controlled by chlorpropamide, lovastatin (20 mg b.i.d. for 6 weeks) lowered low-density lipoprotein cholesterol by 28%, total cholesterol by 24%, and apolipoprotein B by 24%. Lovastatin levels for a 4-hour period (measured as 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitory activity) were similar to those measured previously in nondiabetic patients. Lovastatin did not alter chlorpropamide kinetics or glycemic profiles. No patient had an elevation in serum transaminases or creatinine phosphokinase, and no patient had any other laboratory or clinical drug-related adverse experience during the study. Lovastatin was as effective in reducing low-density lipoprotein cholesterol in patients with NIDDM as in nondiabetic subjects. Diabetic control was unaltered, and no evidence of alteration in lovastatin or chlorpropamide blood levels was noted.

Acyl Coenzyme A↗

Labeling of epsilon-lysine crosslinking sites in proteins with peptide substrates of factor XIIIa and transglutaminase.

Peptides patterned on the N-terminal sequence of fibronectin were synthesized and tested for amine acceptor qualities in reactions with dansylcadaverine catalyzed either by coagulation factor XIIIa or intracellular transglutaminase (protein-glutamine:amine gamma-glutamyltransferase, EC 2.3.2.13). On the basis of inverse half-saturations of the enzymes, the order of acceptor substrate affinity for factor XIIIa was pEAQQIV much greater than Boc-AQQIV greater than Boc-QQIV, and for transglutaminase, Boc-QQIV greater than Boc-AQQIV greater than pEAQQIV (amino acid residues are shown in one-letter code; pE, pyroglutamic acid; Boc, tert-butyloxycarbonyl). Sequence analysis of dansylcadaverine-substituted pEAQQIV indicated that the first of the two adjacent glutamine residues was the target of enzymatic modification. Boc-QIV showed no substrate activity with either enzyme. Crosslinking of crystallins in Ca2(+)-treated rabbit lens homogenate was readily inhibited by Boc-QQIV, Boc-AQQIV, and pEAQQIV, as was the formation of alpha-chain polymers in human fibrin by pEAQQIV in the presence of human factor XIIIa. SDS/PAGE analysis suggested that the inhibitory peptides selectively blocked the electron donor functionalities in these enzymatic crosslinking reactions.

Amino Acid Sequence↗

Peripheral neuropathy in Leigh's disease.

Sural nerves were examined in 3 childhood cases of Leigh's disease (from 2 families), each with electrophysiological documentation of peripheral neuropathy. Postmortem confirmation of Leigh's disease was made in 2 cases; the third had characteristic CT scan appearances. Myelinated fibre densities were within normal limits, with no evidence of degeneration or regeneration. The myelin sheaths, however, appeared thin and this was confirmed quantitatively. In teased fibres there was no demyelination in 2 cases but some in 1 case. The findings suggest that there is hypomyelination of peripheral nerves leading eventually to demyelination.

Brain↗

Preirradiation chemotherapy of supratentorial malignant primary brain tumors with intracarotid cis-platinum (CDDP) and i.v. BCNU. A phase II trial.

Thirty patients with histologically verified malignant supratentorial gliomas were treated with a preirradiation chemotherapy protocol consisting of two courses of intracarotid (i.c.) CDDP, 90 mg/m2, followed by i.v. BCNU, 200 mg/m2. Side effects from therapy were mild and self-limiting; no irreversible retinal or neurologic toxicity could be attributed to i.c. chemotherapy. Of the 27 patients who completed the chemotherapy portion of the protocol, tumor size on postchemotherapy computed tomography (CT) was decreased by greater than 50% in 13% as compared to the postoperative CT scan; in only 4% was the CT scan unequivocally increased in size. Twenty-five (83%) patients completed the entire protocol. Median time to tumor progression and survival in patients who completed the protocol was 53 (range of 13-130+) and 61 (range of 29-130+) weeks, respectively. Twenty-four percent of patients still have not demonstrated tumor progression at intervals greater than 1 year after diagnosis as judged by clinical and radiographic criteria. Tumor recurrences were always contiguous to the original tumor bed. We conclude that preirradiation chemotherapy may be administered safely and with low morbidity. Further study to determine an optimal timing between chemotherapy and radiation therapy is warranted.

Adult↗

Magnetic resonance imaging of Kernohan's notch.

Compression of the cerebral peduncle against the tentorial incisura contralateral to a supratentorial mass, the so-called Kernohan's notch, can be a cause of false localizing motor signs. The authors present a case of Kernohan's notch secondary to a traumatic extradural hematoma. The patient developed an oculomotor palsy and a dense motor deficit ipsilateral to the extra-axial hematoma. Magnetic resonance imaging in the postoperative period clearly showed the midbrain lesion. The motor deficit and 3rd nerve palsy subsequently resolved.

Adult↗

A simple method for the release of islets by controlled collagenase digestion of the human pancreas.

A simple technique for the controlled collagenase digestion of the human pancreas is described. The pancreas is distended with collagenase, and a biopsy taken and divided into 5 pieces that are placed in Universals containing minimal essential medium and dithizone at 39 degrees C. The pancreas itself is incubated in MEM at 39 degrees C. Starting at 5 min and at intervals thereafter, a Universal is removed from the water bath, shaken for 30 sec, and the contents examined by microscopy. As soon as free cleaved islets are seen, the pancreas is placed into one compartment of a kidney-bowl divided in half by a 1-mm mesh. The pancreas is gently teased apart and fluid digest in the empty half of the bowl aspirated and passed through a 500-micron mesh into ice-cold MEM containing 20% newborn calf serum. This process is repeated until the digestion process has ceased. Using this technique on 20 consecutive pancreata, median wt. (range) 53.9 (45.2-72.9) g, we have counted 131,672 (43,516-400,000) islets in the digest, equivalent to 2394 (715-8000) islets/g pancreas. The volume of islet tissue in the digest was 299 (26-1341) mm3 equivalent to 5.81 (0.36-26.81) mm3/g pancreas. In conclusion, we have found this simple technique to be an effective method for the controlled collagenase digestion of the human pancreas.

Adolescent↗

Cardiovascular responses to upright tilting in hypertensive patients, with and without renal impairment and before and following nisoldipine treatment.

1. Cardiovascular responses to upright tilting were determined before and after treatment with the calcium antagonist, nisoldipine, in patients with essential hypertension and in patients of similar ages and similar degrees of hypertension complicated by renal impairment. 2. Cardiac output was determined by a single breath respiratory method and heart rate and blood pressure by an automatic sphygmomanometer. 3. During placebo treatment, the supine values of heart rate and blood pressure were similar in the two groups, but the renal patients had significantly higher cardiac outputs. Following nisoldipine treatment blood pressure was lower in both groups due to decreased vascular resistance; cardiac output was unchanged. 4. Upright tilting lowered cardiac output in both groups, but the fall in the patients with renal disease was greater. There were similar increases in heart rate, diastolic blood pressure and vascular resistance in both groups. After nisoldipine, in the renal patients the fall in cardiac output was again greater and there was also a fall in systolic blood pressure. 5. These results indicate that patients with hypertension complicated by renal impairment may have reduced orthostatic tolerance particularly after treatment with nisoldipine.

Adult↗

Gonococcal arthritis complicated by acute pericarditis and pericardial effusion.

A woman of 23 presented with a painful, swollen left knee. A thick yellow fluid aspirated from the joint cavity grew Neisseria gonorrhoeae. Central pleuritic chest pain and ST segment elevation developed 48 hours after presentation. These signs and symptoms of pericarditis had settled two months after the acute illness.

Adult↗

Non-invasive determination of cardiac output by Doppler echocardiography and electrical bioimpedance.

Cardiac output measured by thermodilution in 25 patients within 24 hours of acute myocardial infarction was compared with cardiac output measured by Doppler echocardiography (24 patients) and electrical bioimpedance (25 patients). The mean (range) cardiac outputs measured by Doppler (4.03 (2.2-6.0) 1/min) and electrical bioimpedance (3.79 (1.1-6.2) 1/min) were similar to the mean thermodilution value (3.95 (2.1-6.2) 1/min). Both non-invasive techniques agreed closely with thermodilution in most patients. None the less, three results with each method disagreed with thermodilution by more than 1 1/min. Both non-invasive techniques were reproducible and accurate in most patients with acute myocardial infarction. Doppler echocardiography was time consuming and technically demanding. Electrical bioimpedance was simple to use and had the additional advantage of allowing continuous monitoring of the cardiac output.

Aged↗

Myofibroblasts and subepithelial fibrosis in bronchial asthma.

A thickened bronchial epithelial basement membrane has long been regarded as a histopathologic characteristic of bronchial asthma. As we had previously demonstrated that this phenomenon is due to the deposition of interstitial collagens and fibronectin, we have now sought to determine the nature of the cell responsible for this process by studying endobronchial biopsies from eight normal and seven asthmatic volunteers by immunohistochemistry and electron microscopy. Biopsies were stained with PR 2D3, a monoclonal antibody to myofibroblasts of the pericrypt sheath of the colon and a monoclonal antibody to alpha-smooth muscle actin. The thickness of the subepithelial collagen and the organelle content of the cells therein were determined by electron microscopy. The subepithelial collagen thickness in the normal subjects ranged from 2.16 to 6.26 microns, while that in the asthmatic subjects ranged from 3.75 to 11.1 microns (Mann-Whitney test; P = 0.05). Elongated cells in the collagen layer were identified by staining with PR 2D3. As this antibody also stains smooth muscle, consecutive frozen sections were stained for alpha-smooth muscle actin and the number of positive cells per millimeter of basement membrane was subtracted from the count for PR 2D3. This yielded a count of 4.9 to 9.4 cells/mm in the normal subjects and 11.9 to 20.6 cells/mm in the asthmatics (P = 0.001). There was a highly significant correlation between the depth of subepithelial collagen and the number of PR 2D3-positive, alpha-smooth muscle actin-positive cells (Spearman rank correlation; r = 0.764 and P = 0.006). Electron microscopy confirmed the myofibroblastic nature of these cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Actins↗

Quality of the residential environment in board-and-care homes for mentally and developmentally disabled persons.

Data from a multistate survey of board-and-care homes were used to compare the quality of the residential environment in homes for mentally disabled and developmentally disabled persons. Staffing levels, services, quality of the physical structure, rapport between residents and staff, number of residents, staff training, and fire and safety precautions were analyzed. The global quality of the residential environment was found to be appreciably better in homes serving the developmentally disabled. Implications of the findings for public policy are discussed, with specific reference to advocacy strategies used in behalf of developmentally and mentally disabled persons.

Adult↗

Selection of admissions to a therapeutic community using a group setting: association with degree and type of psychological distress.

Henderson Hospital is a therapeutic community offering treatment for personality disordered young adults. The community operates a unique system of selection, whereby a group of residents and staff jointly decide upon offering admission to new referrals. This study is a preliminary investigation examining, in part, the basis upon which the selection group makes its decision. A measure of symptomatic psychological distress (SCL-90) was completed by 156 selection candidates. Comparison of those candidates selected with those who were rejected showed no difference between groups in number of symptoms reported nor the degree of distress attributable to those symptoms. However, analysis of symptom sub-scale scores revealed that those subjects not selected scored significantly higher on measures of somatisation, obsessive-compulsive features and phobic anxiety. It could be argued that these three symptoms are indicative of a tendency to deny or avoid feeling emotional distress. We propose that at one level, the selection group may operate by identifying those candidates who are more able to verbalise their distress, i.e. those presumed most likely to benefit from the Therapeutic Community's psychotherapeutic approach.

Adult↗

Spontaneously occurring antibodies to parathyroid hormone.

A 76-yr-old female with acute pancreatitis and a normal/borderline elevated serum calcium level was found to have an elevated immunoreactive circulating PTH concentration using a C-terminal assay. This high PTH concentration misled the attending physicians and resulted, in retrospect, in an unnecessary neck exploration. When the patient's serum was examined it was found to contain a binding component that bound both C-terminal and PTH-(1-84). This binding component was not retained on a Sep-Pak column and was precipitated by antiserum directed against human immunoglobulin M. The presence of circulating anti-PTH immunoglobulin M explains the apparently high PTH concentrations measured by RIA. The antibodies occurred spontaneously. To the best of our knowledge, this phenomenon has not previously been described.

Acute Disease↗