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P Töndury

Publications and source records attributed to P Töndury.

4 recordsLinked to original sources

Liver iron and fibrosis during long-term treatment with deferiprone in Swiss thalassaemic patients.

Serum ferritin levels, hepatic histology and iron concentration were studied in a 'veteran' group of seven Swiss beta-thalassaemic patients after 93-99 months of treatment with the oral iron chelator deferiprone (L1), and another four patients who had received 54-82 months of L1 therapy. Despite continuous compliance, unexplained resurgence of serum ferritin levels occurred in 4/7 patients of the 'veteran' group after 4-5 years on L1. In three of these a concomitant increase of liver iron was also observed. Hepatic histology revealed significantly higher degrees of fibrosis in 6/11 hepatitis C (HC)-positive patients (fibrosis scores 1-5, mean 3.0) than in the HC-negative group (fibrosis score 0-2, mean 0.8). Two HC-negative patients had no detectable fibrosis after 98 and 93 months on deferiprone. Therefore the hepatic pathology in these patients cannot definitely be attributed as a side-effect of deferiprone. Chronic active hepatitis C and the accumulation of iron are the major causative factors to be considered.

Adolescent↗

L1 (1,2-dimethyl-3-hydroxypyrid-4-one) for oral iron chelation in patients with beta-thalassaemia major.

L1 was given to eight patients with beta-thalassaemia major who had previously been treated with deferoxamine (DF) for 4-10 years. The patients' ages ranged from 11 to 27 years. Serum ferritin values ranged from 1.3 to 11.5 x 10(3) micrograms/l. L1 was given twice daily at a daily dose of 55-80 mg/kg body weight and was continued for 10 months in two patients, 9 months in three, 7 months in two patients and 4 months in one patient. As previously observed with DF, each patient's urinary iron excretion (UIE) varied greatly from day to day. The mean UIE of the eight patients ranged from 11 to 49 mg/d (0.2-0.87 mmol/d) on subcutaneous DF and from 16 to 53 mg/d (0.28-0.95 mmol/d) on L1. Two patients excreted significantly more and one patient significantly less iron while on L1. If the UIE was calculated as mmol Fe/mmol creatinine there was no statistically significant difference. Serum ferritin values fluctuated widely in all, with a consistent downward trend in three, no change in four and an increase in one of two non-splenectomized patients. This patient's splenomegaly and need for transfusions continued to increase while on L1. No toxicities attributable to the drug were detected during the period of study and tolerance of the drug was excellent.

Adolescent↗

[Effect of two years of deferoxamine therapy on iron balance, ferritin, liver and heart in patients with thalassemia major].

The iron balance, the urinary excretion of iron between transfusions, the serum ferritin, the liver density, the size of the heart, the ECG and the echocardiogram of 14 children with thalassaemia major were assessed before and during or after 2 years of deferoxamine therapy (DF, 1 or 2 g/kg body weight/day according to age, by subcutaneous infusion on 5 days per week, 11 months per year). The mean iron balance decreased significantly (p less than 0.001) from 15.4 +/- 4 mg/kg body weight/month before DF to -7.1 +/- 9.4 mg/kg body weight/month in the first year of DF and increased to -2.5 +/- 6.5 mg/kg body weight/month during the second year of DF therapy. Despite administration of a constant dose of DF the urinary excretion of iron during the last days before transfusion was twice as high as during the first days after transfusion. The mean serum ferritin level fell from 6380 +/- 2600 ng/ml before DF to 5074 +/- 1600 ng/ml during the first and 4346 +/- 1900 ng/ml during the second year of DF therapy (p less than 0.05). There was no significant change in liver density or cardiac parameters.

Adolescent↗