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Results for “HEMOSIDEROSIS”

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At least 1,711 records · Page 95Linked to original sources
PubMedDate not supplied

[SQUID-biosusceptometry in iron overloaded patients with hematologic diseases].

BACKGROUND: For the long-term survival of iron-loaded patients, early and well adjusted treatment with iron chelators is of crucial importance, especially in children. Basis of the adequate treatment are appropriate diagnostic parameters which are capable to monitor the range of the individual iron burden. PATIENTS: In the time period between 1989 and 2001, the status of iron loading was investigated in 1112 patients with post transfusion siderosis. METHODS: The iron concentration in liver and spleen was quantified by SQUID-biosusceptometry. Using these values, the whole body iron stores were calculated. RESULTS AND DISCUSSION: Based on a large number of patients with secondary siderosis, the benefit of SQUID-biosusceptometry for non-invasive liver iron quantification was evaluated retrospectively. In patients under treatment with deferoxamin, a new therapeutic DFO-index was defined which respects liver iron concentration instead of serum ferritin. This results in a more reliable information about DFO overdosing in a given patient.

Adolescent↗
PubMed2004

[Bull's-Eye Maculopathy with Deferoxamine Treatment].

BACKGROUND: A bull's-eye maculopathy can be provoked by drugs. CASE REPORT: A 67-year-old man observed transient green-yellow spots with both eyes. The visual acuity was 1.0 in the right eye and 0.9 in the left. Tiny pigmentary irregularities of the macula were observed by ophthalmoscopy. Fluorescein angiography revealed a cockade formation of the macula. The multifocal ERG showed normal latencies in the periphery, however, in the centre the amplitudes were reduced to approximately half of the normal values. An acute myeloid leukaemia was diagnosed in 1997 which arose from a myelodysplasia, after performance of a peripheral allogenic blood stem cell transplantation. Because of a serious anaemia, the patient received numerous multiple transfusions with packed blood red cells from April 1999 to April 2000 (a total of more than 90 transfusions). In consequence, a haemosiderosis occurred with a ferritin value of 4390 ng/ml (November 1999) which made treatment with deferoxamine necessary. Multiple injections of deferoxamine (one to two grams were given from August 2000 until February 2001, afterwards an intermittent application until November 2002 was carried out) which resulted in the maculopathy. CONCLUSION: Retinal changes, in particular, a maculopathy, should be considered even if visual acuity and visual fields are not remarkably changed. Dependent on the extent of the disease, deferoxamine treatment should be discontinued in case of distinct visual deterioration.

Aged↗
PubMed1987

The euglycemic clamp in patients with thalassaemia intermedia.

In order to evaluate the influence of haemosiderosis on the glucose metabolism we studied tissue sensitivity to insulin and the metabolic clearance rate (M.C.R.) of this hormone by means of euglycemic clamp technique using an artificial endocrine pancreas in 8 patients with thalassaemia intermedia and 8 control subjects. During the steady-state of euglycemic-hyperinsulinemic clamp (40 mU/m2/min) plasma insulin values were significantly lower and the insulin M.C.R. was significantly higher in thalassaemic patients compared to the controls. To achieve a comparable steady-state insulin concentration to the controls, we performed for a second time the euglycemic clamp in the thalassaemic patients increasing the insulin infusion rate to 80/mU/m2/min. The insulin M.C.R., the M index and the M/IRIs-s ratio were significantly higher in the thalassaemic patients compared to the controls. These results are indicative of an increased tissue peripheral sensitivity to insulin as well as the metabolic clearance rate of this hormone.

Adult↗
PubMedDate not supplied

[Cardiac dysfunction in children with thalassemia major].

Evaluation of the cardiac function of seven patients with beta-thalassemia major was performed by continuous electrocardiography recording during a 24 hr period and during a short term exercise program. A reduction in left ventricular function was assessed by echocardiography and radiocardiography during resting and during exercise. Serious arrhythmias of various forms in the oldest patients (17 and 20 years) were observed during these tests. The younger patients were asymptomatic and their cardiac function tests showed no abnormality. In order to prevent transfusion related cardiac iron toxicity it would seem mandatory to motivate patients for better compliance while on chelation therapy.

Adolescent↗
PubMed1980

[Pluriglandular insufficiency due to transfusion haemosiderosis in Blackfan-Diamond anaemia (author's transl)].

The article reports on a boy aged 16 1/2 years suffering from transfusion haemosiderosis associated with corticoid-resistant blackfan diamond anaemia. Besides hepatosplenomegalia and cardiomegalia, the course of the disease was mainly determined by the deficient functioning of several endocrine glands. The diagnosis revealed primary hypothyroidism, hypoparathyroidism and partial insufficiency of the pituitary gland.

Adolescent↗
PubMedDate not supplied

[Beta-thalassemia in Germany. Results of cooperative beta-thalassemia study].

At present, about 300 patients with thalassemia major are living in Germany. Starting in 1991, a multicenter study in Germany has concentrated on identifying all patients suffering from thalassemia as well as on establishing a uniform therapy protocol including follow-up diagnostic procedures. After six years of study, the data of 198 patients suffering from thalassaemia major were analysed. The majority of these patients originate from endemic regions around the Mediterranean Sea. The patient's median age is 13.8 years (range 1-37.5 yrs.). At present, about 20% of patients are older than 21 years. Regarding transfusion therapy, a shortening of the average transfusion interval to 3 weeks in most cases occurred. Throughout the entire period, median baseline haemoglobin concentrations of 10.0 g/dl could be observed. The evaluation of serum ferritin levels revealed considerable differences depending on patients age. 60% of patients in the first decade of life showed good therapeutic results with serum ferritin levels below 1800 ng/ml. In contrast, 52% of patients older than ten years presented with ferritin levels above 2500 ng/ml. During the observation, a decreasing number of patients with ferritin levels above 2500 ng/ml was observed in patients aged 15 to 21 years of age. The situation of patients aged 9 to 15 years proved to be more problematic. More than half of all treated patients presented with siderotic complications as cardiac disease in 13%, liver disease in 21%, impaired glucose metabolism in 14%, hypothyroidism in 24% and hypogonadism in 59% of all patients. These values did not change considerably during the observation apart from an increase of cardiac disorders to 20%. Since the situation concerning siderosis and the lack of compliance proved to be particularly difficult in adolescent patients, further efforts has to concentrate on this age group.

Adolescent↗
PubMed1994

[Congenital atransferrinemia].

Congenital atransferrinemia was first diagnosed at the age of 11 months in a now 27-year-old woman. Until she was aged 14 years treatment with human transferrin was irregular and, as it turned out, inadequate. But since then she has regularly received human transferrin (1 g monthly) and deferoxamine (500 mg twice weekly). Despite this she developed haemosiderosis affecting heart, liver, hypophysis, thyroid and the locomotor apparatus. This case report demonstrates the need of early diagnosis and treatment of congenital atransferrinemia to prevent the mentioned complications.

Adult↗
PubMed1986

[Continuous subcutaneous deferoxamine infusions in thalassemia major. Improvement in glucose tolerance].

Exocrine and endocrine pancreatic functions were studied in 30 patients with homozygotic beta-thalassaemia. All were treated with continuous subcutaneous deferoxamine infusions for a mean period of 30 months. Three patients (aged 18-22 years) had insulin-dependent diabetes, two before and one shortly after the onset of deferoxamine administration. There was no improvement during the treatment. An abnormal glucose tolerance test was demonstrated in 14 patients (47%) before and in seven (23%) during deferoxamine infusion. Enzyme activity of alpha-amylase and lipase as an expression of exocrine pancreatic function was normal in all during the observation period. Improvement in endocrine pancreatic function was apparently age-dependent: the younger the patient at the onset of treatment the more likely is normalization of the oral glucose tolerance test.

Adolescent↗