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Biomedical subjects

E Christensen

Publications and source records attributed to E Christensen.

At least 253 records · Page 14Linked to original sources

Glutaric aciduria in progressive choreo-athetosis.

The clinical symptoms in a 10-year-old girl with progressive dystonic cerebral palsy are described. The biochemical findings were dominated by large amounts of glutaric acid in the urine. The disorder is caused by impairment of the degradation of glutaryl-CoA. A survey is given of the clinical and biochemical symptoms, based on the five cases reported so far. It is concluded that patients with progressive dystonic palsy should be examined for disorders in the metabolism of organic acids.

Acyl Coenzyme A↗

Treatment of gastric ulcer. The randomized clinical trials from 1964 to 1974 and their impact.

Twenty-nine randomized clinical trials (RCTs) from the decade 1964-74 evaluating treatments of gastric ulcer have been analyzed. None of them ful-filled all criteria for an ideal RCT. The most frequent shortcomings were: short treatment or follow-up periods, incomplete description of the patients included, small patient samples, suboptimal experimental design, lack of double-blind testing, high number of drop-outs, less precise or less relevant types of evaluation of the treatment effect, uncontrolled ancillary treatment, or lack of statistical evaluation of the results. The most frequently tested drug was carbenoxolone, which has been shown to be clearly effective. Judged by the effect on the recommendations in standard medical textbooks the impact of the best RCTs have been small. This situation emphasizes the need for more well-planned and performed RCTs on treatments of gastric ulcer.

Adult↗

Calcium levulinate medication. A pitfall in the diagnosis of organic acidurias.

Five children, who received calcium levulinate (calcium 4-oxopentanoate) intravenously in pharmacological doses excreted in the following 24-h period both 4-oxopentanoic acid (3.5--11.0 mg/24 h) and 4-hydroxypentanoic acid (4.5--10.4 mg/24 h). Attention is drawn to the fact that in gas chromatographic-mass spectrometric systems these compounds closely resemble the two acids found in children with beta-ketothiolase deficiency.

Acetyl-CoA C-Acyltransferase↗

The epidemiology of the gastrointestinal randomized clinical trial.

Has the randomized clinical trial (RCT), generally accepted as the method of choice for evaluation of most treatments, obtained a footing in gastroenterology? Among 35,228 citations on gastroenterologic therapy indexed in MEDLARS 1964-1974 306 (0.9 per cent) were RCT's. During the decade their frequency rose significantly (P less than 0.05) from 0.3 per cent in 1964 to 1.7 per cent in 1973. The "typical" RCT was a double-blind two-group comparison of a new and an established drug on the symptoms of peptic ulcer; 50 patients were followed for six weeks, and the number of dropouts was unknown. The new drug was found to be more effective. It is postulated that the RCT's in gastroenterology are quantitatively and qualitatively insufficient and that co-ordinated planning of RCT's and uninhibited publication of statistically proved negative results may help to ensure that the patient with gastrointestinal disease receives the best available treatment.

Europe↗

Glutaric aciduria: clinical and laboratory findings in two brothers.

In two siblings with dystonic cerebral palsy the urinary metabolic profiles of organic acids were dominated by glutaric acid, a metabolite not normally present in urine. The exretion of glutaric acid amounted to several grams per day. The urinary excretion of beta-OH-glutaric acid and glutaconic acid was also enhanced. Imparied metabolism of glutaryl-CoA by leukocytes indicates that the patients suffer from an inborn error of lysine, tryptophan, and hydroxylysine metabolism. A defective oxidation of glutaryl-CoA to crotonyl-CoA, probably due to a deficiency of glutaryl-CoA dehydrogenase, is consistent with these findings.

Amino Acid Metabolism, Inborn Errors↗

Arterial blood pressure in patients with oral lichen planus.

The arterial blood pressure of 121 patients with oral lichen planus was determined using the auscultatory method. These values were then compared with those previously found in a general adult population. No significant difference was found betwween the values of the lichen planus patients and those of the general population. The present investigation does not support the hypothesis of a relationship between lichen planus and hypertension.

Adult↗

Glucose tolerance in patients with oral lichen planus.

Of 123 patients with oral lichen planus two were known diabetics. The remainder were tested for fasting glucosuria, 113 for fasting blood glucose and 103 also with an oral glucose tolerance test. According to the criteria of WHO, 18 of the 123 patients were diabetics. In most of these patients the oral glucose tolerance test was only slightly abnormal. No decrease of the glucose tolerance with increasing duration of lichen planus was demonstrable. Comparison with two general population studies did not reveal any significant difference between the glucose tolerance of the lichen planus patients and that of the general population samples.

Adult↗

Serum zinc and urinary zinc excretion after renal allotransplantation.

In nine kidney-transplanted patients, receiving prednisone as part of the immunosuppression, serum zinc and urinary zinc excretion were measured for a period of 5-7 weeks after the transplantation. Serum zinc decreased significantly from normal pretransplant values to subnormal vaues 1-5 days after the transplantation. Thereafter, the serum zinc rose again, and after 2-3 weeks, before start of reduction of prednisone, the serum zinc had risen to a level that was not significantly lower than the pretransplant level. Urinary zinc excretion was generally elevated, especially just after the transplantation. In patients with initially good kidney function function the zinc clearance to creatinine clearance ratio was significantly higher during the first week after the transplantation than in the rest of the observation period. Similar findings have been reported after major surgery, and no correlation between serum zinc and the corticosteroid dose was demonstrable. Therefore, in renal allotransplantation, no special supplementation of zinc seems necessary beyond what may be justified by the effect of the surgical trauma.

Adult↗

Clinical listeriosis in renal allotransplantation.

Two cases of Listeria monocytogens meningitis among 212 kideney transplanted patients (total of 339 patient years of observation) under immunosuppression with azathioprine and prednisone are presented. Both cases developed shortly after an increase of the immunosuppression. The first case appeared in a 27-year-old man 5 days after a minor increase of the azathioprine dose from 75 to 100 mg/day. The course was relatively mild, and the patient was cured by tetracycline. The second case appeared in a 52-year-old woman 5 days after a massive increment of the steroid dose and adminsteration of a moderate azathioprine dose, carried out to revert a rejection of the graft. This case had a fulminant course and was complicated by Listeria sepsis with hemolysis, pronounced oliguria and thrombocytopenia leading to fatal internal bleedings, primarily in the brain. Considering the poor prognosis of this complication it is suggested that cytotoxic drugs are temporarily discontinued and the steroid dose reduced at the height of the infection.

Adult↗

Involution of polycystic kidneys during active treatment of terminal uremia.

Following institution of chronic dialysis and/or renal transplantation for terminal uremia, four consecutive patients observed for 15-48 months with their polycystic kidneys in situ invariably showed a rapid relief of symptoms (pain, hematuria) followed by a gradual, but steady, involution of the kidneys. The mechanism and the practical clinical implications are discussed.

Adult↗