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At least 1,603 records · Page 89Linked to original sources
PubMed1993

Fate of iron stores in thalassaemia after bone-marrow transplantation.

After successful bone-marrow transplantation (BMT) in thalassaemia, the individual acquires the pattern of globin synthesis of the donor. We call such an individual "ex-thalassaemic after BMT", a term that underscores the cure of the genetic defect but maintenance of residual signs of organ damage due to iron overload and dysfunction acquired during the pretransplant years. We have analysed the extent and fate of tissue iron overload in 151 ex-thalassaemic patients after BMT, according to the risk factors of hepatomegaly, hepatic portal fibrosis, and inadequate chelation therapy. Serum ferritin concentrations decreased and unbound iron binding capacity (UIBC) increased slowly during the years after the transplant. When analysed according to risk group (assigned at the time of the transplant), ferritin and UIBC returned within the normal ranges in only the low-risk group (without hepatomegaly or portal fibrosis, and with adequate chelation pre-BMT). Ferritin and UIBC were still abnormal 7 years after the transplant in the moderate-risk group (those with one or two risk factors) and highly abnormal in the high-risk group (all three risk factors) indicating persistence of, respectively, moderate and severe iron overload at the time of transplant. In ex-thalassaemic patients who were studied before and yearly after the transplant the extent of haemosiderosis, as judged by staining of liver biopsy samples, decreased during the years after transplant. The degree of iron deposition and rate of post-BMT linear growth seem to influence rate of post-BMT decrease in tissue iron overload in different risk groups at the time of BMT.

Adolescent↗
PubMed1995

Trophoblast basement membrane haemosiderosis in the placental lesion of fetal artery thrombosis: a marker for disturbance of maternofetal transfer?

The placental lesion of fetal artery thromboses is characterized by collapse and obliteration of chorionic vasculature, an increase in stromal connective tissue and syncytial knots, with a thickening of trophoblast basement membrane. An additional feature, not previously described in association with the lesion, is linear trophoblast basement membrane haemosiderosis. Thirty-five such lesions were examined for this feature which was identified in 32. Random tissue sections of placentae from cases of intrauterine death showed a similar basement membrane haemosiderosis and were used as positive controls. None of 20 normal control cases examined demonstrated the feature. Electron microscopy demonstrated electron-dense bodies within the basement membrane. Spectrographic analyses confirmed the presence of iron within these deposits. The significance of this finding lies not so much in the fact that it is an additional finding in fetal artery thrombosis but rather in the underlying pathophysiology.

Basement Membrane↗
PubMed1996

[Intrapulmonary hemorrhages associated with Basedow disease].

We report the second case of intraalveolar haemorrhage associated with an autoimmune thyrotoxicosis. While intraalveolar haemorrhage was not recurrent since 7 years with corticosteroid therapy, a relapse occurred when we began the treatment for an autoimmune thyrotoxicosis. We discuss the link between intraalveolar haemorrhage and autoimmune thyrotoxicosis or its treatment.

Adult↗
PubMed1996

Subchronic toxicity of thiabendazole (TBZ) in ICR mice.

The subchronic toxic effects of thiabendazole (TBZ) administered in the diet at levels of 0 (control), 0.8 and 1.6% for 13 wk to male and female ICR mice were investigated. Mean body weights of male mice fed 0.8 or 1.6% TBZ showed a significant decrease compared with controls, except for wk 3 and 8 for mice fed 0.8% TBZ. Red blood cell parameters in male mice of treated groups were significantly lower than controls. Biochemistry showed increased concentrations of GOT and GPT in male and female mice of the 1.6% TBZ groups. Relative spleen or liver weights were significantly increased in male and female mice of treated groups. Relative kidney weights of treated mice tended to be increased in comparison with controls. Histological findings showed a marked haemosiderosis and extramedullary haematopoiesis in the spleen of treated mice. In the liver, sinusoidal dilatation and enlargement of liver cells were found in treated mice. In the kidney, atrophy of tubules with peritubular fibrosis, cell infiltration and some tubular necrosis were found in treated mice. Slight hyperplasia was found in the urinary bladder of treated mice. The findings in the present study indicate that TBZ caused a slight anaemia and liver or kidney injury at both levels tested, under these conditions.

Administration, Oral↗
PubMed1980

Nature of iron deposits on the cardiac walls in beta-thalassemia by Mössbauer spectroscopy.

An identification of the nature and an estimation of the particle size distribution of the iron deposits on thalassemic heart tissue is carried out by variable temperature Mössbauer spectroscopy. Comparison of Mössbauer spectra obtained for the thalassemic heart tissue (I) with those of normal heart tissue (II) and of horse spleen ferritin (III) identifies the iron deposits to be small, superparamagnetic particles of ferritin and/or hemosiderin, two closely related iron storage proteins containing an iron core of (FeOOH)8(FeO x OPO3H2). The dependence of the superparamagnetic relaxation time, tau, of magnetically ordered fine particles on their volume V via the magnetic anisotropy constant K of the material and the condition tau > tauL, the Larmor precession time of the nuclear magnetic moment of 57Fe about an effective magnetic field, for observation of hyperfine structure are used in analyzing the Mössbauer data to yield the particle size distribution. Particle diameters are estimated to be 74 +/- 12 A.

Adolescent↗
PubMed1996

The effects of iron loading and iron deficiency on the tissue uptake of 64Cu during development in the rat.

This study examined the uptake of 64Cu by the brain, liver and other organs during development in rats aged 15, 21 and 63 days fed low, normal and high iron diets, using either a solution of 64CuCl2 chelated with nitrilo-triacetic acid (NTA) or 64Cu-ceruloplasmin (64Cu-Cp). 64Cu-NTA uptake was higher in the brain, spleen, kidneys, femurs and red cells at 15 days than at the later ages, while the liver took up most of the 64Cu in 63-day-old rats over the 2 h of the study. The brain had similar levels of 64Cu-NTA uptake at 15 and 21 days, even though liver uptake significantly increased, suggesting that Cu-NTA uptake by the brain increases from 15 to 21 days. The brain took up a greater percent of the injected dose of 64Cu-Cp than 64Cu-NTA yet, in either case, brain uptake was lower than that of the other organs. Iron loaded rats had significantly higher uptake of non-ceruloplasmin-bound 64Cu in all the organs examined, for at least one of the three ages, when compared with control rats. However, iron deficiency produced little change. Iron loading has a greater effect on 64Cu-Cp uptake than 64Cu-NTA, decreasing 64Cu uptake in the brain, liver, kidneys and femurs. Iron deficiency only increased 64Cu-Cp uptake in the liver. These results suggest that the mechanism of copper uptake by the liver is still maturing during suckling in the rat, and that ceruloplasmin receptor numbers are down regulated by iron loading, thus providing evidence of a new link between iron and copper metabolism.

Animals↗
PubMedDate not supplied

Femoral head avascular necrosis in sickle cell anemia: MR characteristics.

Since considerable expansion of hematopoietic marrow occurs in patients with sickle cell anemia (SCA), magnetic resonance images of 20 hips in 10 patients with known homozygous SCA were reviewed to determine a) if low signal hematopoietic marrow extended into the femoral capital epiphysis and b) if the MR characteristics of avascular necrosis (AVN) differed depending on the type of epiphyseal marrow. Our results revealed variable epiphyseal marrow type; mixed (fatty and hematopoietic) marrow (42%), fatty marrow (32%), hematopoietic marrow (16%) and hemosiderotic marrow (10%). AVN occurred irrespective of the underlying marrow. Segmental areas of low signal intensity in variable shapes (ring, band, crescent or large homogeneous area) was the most consistent MR manifestation of AVN in SCA. A low signal intensity peripheral rim surrounding a central zone, isointense with epiphyseal marrow on T1 and T2 weighted images, was most frequently observed similar to that described in patients without hemoglobinopathy. The notable difference, however, was of segmental areas within the same femoral head that demonstrated variable central zone signal on T2 weighted images. Further, while an increase in hip joint fluid is commonly seen with both early and advanced AVN in patients without hemoglobinopathy; it was increased in only one hip in patients with SCA. The observed differences in MR characteristics may be due to different pathophysiology of AVN in patients with SCA.

Adolescent↗
PubMed1995

CT and MRI of diffuse liver disease.

CT and MRI contribute important information to the clinical evaluation of diffuse liver disease. In some cases, these modalities can establish a diagnosis that was not ascertained histologically, which is often the case when sampling errors prevent a definitive tissue diagnosis. Characteristic alterations of liver attenuation on CT, signal changes on MRI, and morphological changes appreciated with both modalities can be used to diagnose fatty infiltration, some parenchymal deposition diseases, and cirrhosis. Furthermore, hepatocellular disease can be confirmed in the setting of indeterminate clinical and laboratory findings. Significant overlap in the imaging findings of this wide range of disorders continues to limit specificity; however, at a minimum, these techniques provide a rapid means to a noninvasive evaluation that often guides clinical decisions. Faster scanning techniques available with CT and MRI may provide additional information by assessing contrast dynamics. This review of CT and MRI in diffuse liver disease considers the diagnostic utility and clinical implications of these modalities. Pathological findings relevant to imaging considerations are discussed.

Fatty Liver↗
PubMed1987

Hepatic fibrosis and cirrhosis in tropical countries (including portal hypertension).

The spectrum of diseases seen in patients from certain 'tropical areas' requires that a physician be aware of some of the syndromes discussed here. A high index of suspicion for less usual causes of cirrhosis is imperative when evaluating such patients presenting with hepatocellular disease. The differential must be expanded and the work-up complete. Liver biopsy should be performed as early in the course of disease as is feasible and will often provide valuable diagnostic information. It will not only facilitate treatment but allow for needed clinical trials and may help to increase our understanding of the various disease processes.

Anemia, Hemolytic↗
PubMed2004

Diffuse alveolar hemorrhage.

Diffuse alveolar hemorrhage represents a medical emergency, and clinicians must have an expedient approach to its identification. There are many causes of diffuse alveolar hemorrhage, including vasculitides, immunologic conditions such as Goodpasture's syndrome, collagen vascular disease, and idiopathic conditions. Careful attention to the medical history, physical examination, and targeted laboratory evaluation often suggests the underlying cause. Patients in whom the diagnosis of diffuse alveolar hemorrhage remains uncertain should undergo diagnostic bronchoscopy. In patients with evidence of diffuse alveolar hemorrhage and renal involvement, kidney biopsy should be considered to identify the underlying cause and help direct therapy.

Anti-Glomerular Basement Membrane Disease↗
PubMed2004

Attributes of Stachybotrys chartarum and its association with human disease.

Mold contamination and toxicities are not limited to crops and animals; they are also a concern in human health. Molds occur in outdoor and indoor environments, and water-damaged buildings harbor and provide substrate for several mold species. Of these, Stachybotrys chartarum poses a particular threat to occupants. Patients with building-related symptoms and infant idiopathic pulmonary hemorrhage often have histories of living in moldy, water-damaged buildings. Although a causal connection is far from being unequivocally proven, S. chartarum has been associated with such clinical conditions. These illnesses could be attributed in part to mycotoxins released by S. chartarum. Recently, a hemolysin released by this mold was found to be hemolytic in vitro and in vivo. In addition, allergenic proteins have been characterized from S. chartarum. The exact mechanism of S. chartarum pathogenesis has not yet been defined. Moreover, a causality-effect relation is not yet established. This review summarizes available information on the pathogenic attributes of S. chartarum and calls for well-controlled objective studies.

Animals↗