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At least 1,549 records · Page 86Linked to original sources
PubMed1995

MR marrow signs of iron overload in transfusion-dependent patients with sickle cell disease.

Magnetic resonance (MR) marrow signal in the axial and appendicular skeleton of 13 transfusion-dependent and chelated pediatric patients with sickle cell anemia (SSD) was compared with marrow signal in six non-transfusion-dependent patients with SSD. Hepatic, pancreatic, and renal MR signal were also evaluated. Indication for hypertransfusion therapy was primarily prior history of stroke. Transfusion-dependent patients had evidence of iron deposition throughout the imaged marrow and the liver, despite deferoxamine chelation therapy. Non-transfusion-dependent patients did not demonstrate grossly apparent signs of iron overload. Red marrow restoration was present in the spine, pelvis, and long bones and, in some patients, within the epiphyses. Marrow edema secondary to vaso-occlusive crises was evident in the metaphyses and diaphyses of long bones in areas of both red and fatty marrow and was best seen using fat-saturated T2-weighted imaging techniques.

Adolescent↗
PubMed1990

Treatment of iron overload in children and adolescents on chronic haemodialysis.

Treatment of secondary haemosiderosis with desferrioxamine (DFO) was evaluated in children and adolescents treated by chronic haemodialysis. Ten patients were treated with intravenous DFO (25 mg/kg body weight). Three patients received DFO throughout dialysis, four were treated only during the last hour of each dialysis session, and three other patients were successively treated with DFO according to both regimens. A significant decrease and normalisation of serum ferritin was achieved only with DFO administered during the last hour of dialysis. In some patients blood haemoglobin (Hb) increased during treatment. Although serum ferritin levels remained normal, Hb levels returned to pretreatment values following discontinuation of DFO treatment. Visual and auditory neurotoxicity was not noticed but increased systemic infections were observed.

Child↗
PubMed1994

Liposteroid against refractory pulmonary haemorrhage in idiopathic pulmonary haemosiderosis.

We describe two Japanese children with idiopathic pulmonary haemosiderosis (IPH), whose refractory haemorrhages were treated with an intravenous lipid emulsion containing dexamethasone (liposteroid). A 22-month-old boy and a 14-month-old girl have been observed with similar symptoms; periodic bouts of anaemia, reticulocytosis, diffuse infiltrates on chest X-ray and the finding of siderophages in sputum or gastric lavage fluid. The MRI of the lung was useful for the diagnosis. Methylprednisolone pulse therapy was successful in treating acute massive bleeding. Subsequent oral prednisolone could not prevent chronic recurrent haemorrhages. However, the intermittent administration of liposteroid (0.05 mg/kg/dose IV) led to a cessation of bleeding; the haemoglobin concentration rose to normal levels. This observation emphasizes the usefulness of liposteroid in the management of refractory IPH.

Dexamethasone↗
PubMed1991

Superficial siderosis of the central nervous system. A 37-year follow-up of a case and review of the literature.

The course of a patient suffering from superficial siderosis of the central nervous system for 37 years is presented and diagnostic and therapeutic approaches are evaluated. The syndrome is clinically defined by slowly progressing deafness, cerebellar ataxia, myelopathy and neuropsychological deficits in combination with recurrent xanthochromia of the cerebrospinal fluid with siderophages. The diagnosis may be confirmed by computed tomography, which shows degeneration of the cerebellar vermis, and by magnetic resonance imaging, demonstrating iron deposits on the surface of brain, brain stem and spinal cord. Therapy should seek to identify and remove the source of bleeding, since pharmacotherapy with iron-depleting drugs is of limited effectiveness.

Brain Diseases↗
PubMed1994

[Benign giant cell tumor of the tendon sheath. A differential diagnosis of cutaneous tumors near the joint].

Giant cell tumour of the tendon sheath, though rather a common connective tissue tumour, is rarely mentioned in the dermatological literature. It is a benign, slow-growing tumour arising from the synovia and it is most frequently located on the joints of the fingers. It becomes manifest clinically as a firm, skin-coloured nodule fixed to the underlying tissue. The histological picture is characteristic and shows osteoclast-like giant cells, histiocytic cells, foam cells and focal haemosiderosis. It probably reflects a reactive process similar to that of fibrous histiocytoma. The therapy of choice is excision, with a small margin of uninvolved tissue to avoid recurrences.

Adult↗
PubMed2004

[Superficial siderosis of the CNS. 2 cases and a review of the literature].

Superficial siderosis of the CNS is a rare disease. The superficial deposition of haemosiderin in the cerebrum, cerebellum and spinal cord is due to chronic and recurrent subarachnoidal haemorrhage (SAH). Known sources of bleeding are vascular CNS-tumours, CSF-cavity lesions, vascular malformations, nerve root lesions and neurosurgical interventions. Detection of the source of bleeding is successful in only about 50% of cases. The clinical syndrome is characterized by sensorineural deafness, cerebellar ataxia and pyramidal signs. CSF-investigation might be indicative for SAH, while ferritin and ionic iron can be elevated in the CSF. CT is unspecific and insensitive but MR imaging of the brain and spinal cord is very sensitive and specific. The elimination of the source of bleeding alone might prevent the progression of the disease, therefore, an early and extensive search for this source is highly recommended.

Central Nervous System Diseases↗
PubMed1997

Advantages of inferior vena caval flow preservation in combined transplantation of the liver and heart.

Only a few cases of combined liver and heart transplantation have been reported in the literature, and no standard surgical procedure has yet been established. We report the successful transplantation of both liver and heart in a 28-year-old patient suffering from homozygous beta-thalassemia. We used Belghiti's technique of inferior vena caval flow preservation for liver transplantation, which avoids inferior vena cava occlusion by a side-to-side caval anastomosis. Applied to combined liver and heart transplantation, preservation of caval flow during liver transplantation may allow early discontinuation of cardiopulmonary bypass and, thus, minimize the general consequences of prolonged bypass.

Adult↗
PubMed1998

Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss.

We present a 50-year-old man who was investigated for sensorineural hearing loss. On MRI of the brain superficial siderosis of the central nervous system was seen, while MRI of the spine revealed an ependymoma of the cauda equina. This case illustrates the importance of performing T2-weighted imaging of the brain and posterior fossa when sensorineural hearing loss is present. Spine imaging is mandatory when superficial siderosis of the brain is diagnosed without identification of a bleeding source in the brain.

Brain↗
PubMed2000

Decontamination of winter flounder (Pleuronectes americanus) following chronic exposure to effluent from a pulp and paper mill.

A study was conducted to determine the influence of decontamination on winter flounder living downstream from a pulp and paper mill. The fish and controls were held 8-26 weeks under contaminant-free conditions in aquaria supplied with ambient sea water. Groups of flounder, for comparison, were also sampled near the paper mill at the time of or following autopsy. No differences were apparent in length/organ-weight relationships between depurated and control groups after 26 weeks at 0-6 degrees C, but condition factor, gonadal and hepatosomatic indices, coincident with elevated levels of detoxifying enzymes, differed from field-derived samples. Pathological changes, including hemosiderin deposits in the liver and spleen, decreased, whereas pericholangitis and clear cell foci were apparent in the depurated fish and in the field samples. In two additional groups decontaminated for 8-16 weeks at 6-16 degrees C, there was no evidence of pericholangitis, but hemosiderin deposits persisted and an increase of fibrosis and clear cell foci occurred in contrast to the field group. These results suggest that some tissue repair in the liver transpired primarily during summer while other lesions, such as preneoplastic clear cell foci, which were probably induced prior to depuration, increased, but hemosiderin concentration remained unchanged. The latter observation is consistent with previous studies that suggest that hemosiderin concentration is not seasonally but age influenced.

Animals↗