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Notes: [Auszug] The characteristic phenotype in individuals affected with HPRC is shown in Fig. 1. The intial genome scan performed with families 150, 152 and 153 suggested linkage of HPRC to loci on chromosome 7q. The linkage analysis was repeated when DNA samples from families 160 and 162 became available. The ...

Notes: Abstract Pheochromocytoma is a catecholamine-producing tumor of the sympathetic nervous system. Signs and symptoms are generally related to catecholamine excess; these include hypertension, sweating, palpitatione, headaches, and anxiety attacks. Abdominal imaging and 24-h urine collection for catecholamines are usually be sufficient for diagnosis. Catecholamine blockade with phenoxybenzamine and metyrosine generally ameliorates symptoms and is necessary to prevent hypertensive crisis during surgery. Standard treatment is laparoscopic adrenalectomy, although partial adrenalectomy is gaining enthusiastic support in familial forms of pheochromocytoma.

Notes: Abstract Adrenoleukodystrophy is an X-linked disorder characterized by loss of adrenal function and demyelinating nervous disease. A slowly progressive form, adrenomyeloneuropathy, has been described that can mimic multiple sclerosis. Two brothers are presented whose contrasting urodynamic findings are compatible with upper and mixed (upper and lower) motor neuron disease, respectively.

Notes: Abstract Suramin has been shown to have an effect on bone resorption in in vitro models. It is not clear if a similar effect is seen in patients treated with suramin. The clinical effect of suramin treatment on total serum calcium was examined in two groups of patients with hormone-refractory prostate cancer. In all, 28 patients in group 1 were examined within 2 weeks before and 2 weeks after suramin treatment and 72 patients in group 2 were examined within 2 weeks before, during, and after treatment with suramin. In addition, calcium controls spiked with suramin were run in three different commercially available assays for evaluation of the effect of suramin dose on calcium determination. Group 1 patients showed a decrease in serum calcium after treatment with suramin. The mean uncorrected serum calcium level was 2.29 ± 0.025 mmol/l before treatment and 2.09 ± 0.025 mmol/l after treatment (P 〈 0.0001, paired Wilcoxon test). The mean serum calcium value corrected for albumin was 2.33 ± 0.02 mmol/l before treatment and 2.24 ± 0.02 mmol/l after treatment (P=0.0022, paired Wilcoxon test). Group 2 patients also displayed a decrease in serum calcium after treatment with suramin. The mean baseline value was 2.23 mmol/l (median 2.26 mmol/l, range 1.20–2.54 mmol/l). The mean level of serum calcium corrected for albumin as determined at the end of treatment was 2.14 mmol/l (median 2.16 mmol/l, range 0.98–2.46 mmol/l). In all, 48 patients for whom pre- and posttreatment values were available for analysis displayed a median calcium decrease of 0.09 mmol/l (P=0.0005, Wilcoxon signed-rank test for the null hypothesis of no change). For 68 patients in group 2, data on serial serum calcium measurements during treatment were available for analysis. A projected median decrease in serum calcium of 0.06 mmol/l (range –0.43 to 0.72 mmol/l) over an 8-week interval of suramin therapy was found. Overall, 47 of the 68 slopes were negative (P=0.0022, Wilcoxon signed-rank test). Nine patients were treated with suramin for less than 6 weeks. These patients' calcium levels were significantly higher than those of 50 patients treated for longer periods (median value 2.24 versus 2.16 mmol/l, P=0.035, Wilcoxon rank-sum test). No correlation was found between suramin dose and calcium level using the Kodak Ektachem, Hitachi 914, or Synchron Clinical System CX3 method. In conclusion, suramin treatment was consistently associated with decreases in serum calcium in two groups of patients with hormone-refractory cancer. Suramin placed in calcium controls did not affect calcium determination using three commercially available methods.