PubMed Health⌕ Search

Biomedical subjects

B Ferrand

Publications and source records attributed to B Ferrand.

At least 37 records · Page 2Linked to original sources

Assessment of liver iron content in 271 patients: a reevaluation of direct and indirect methods.

Two direct methods for hepatic iron assessment (liver iron concentration and stainable liver iron--judged by a new histologic grading) and three indirect methods (serum iron and transferrin saturation, deferoxamine-chelation test, and ferritinemia) were reevaluated in 271 patients. These patients consisted of: 103 with idiopathic hemochromatosis, 39 with alcoholic cirrhosis, 54 with noncirrhotic alcoholic liver diseases, 13 with nonalcoholic liver diseases, and 62 with miscellaneous disorders. The results indicate that: (a) liver iron concentration, well correlated with mobilized excess iron (r = 0.88; p < 0.01), is the method of reference and validates the proposed histologic grading; (b) serum ferritin, which is in good correlation with liver iron concentration (r = 0.80; p < 0.01), is a valuable indirect method for hepatic iron evaluation; (c) regarding the other indirect methods a "boundary zone" may be delimited, thus corresponding to liver iron concentration values of 10.7 mumol/100 mg dry liver weight, beyond which values of serum iron less than 28.6 micrometer or transferrin saturation less than 45% or chelatable iron less than 45 mumol/24 h are rare; and (d) using the various indirect methods, there is a marked risk in idiopathic hemochromatosis to underestimate and in alcoholic liver diseases to overestimate hepatic iron content.

Deferoxamine↗

[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)].

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.

Acute Disease↗

[Bone histomorphometry and osteo-articular manifestations of idiopathic hemochromatosis].

An histomorphometric study of the bones was done on 28 patients suffering from an idiopathic hemochromatosis. There was a normocalcinemic hyperparathyroidism in half the cases. It appeared to be closely linked to hypersideremia and to be responsible for the sub-chondral arthropathy. The other lesions demonstrated were an osteoporous one, whose relations with hypogonadism was shown, and osteoidosis variations, which most often decreased rather than increased, and whose causes still seem uncertain.

Adult↗

[The cardiomyopathy of idiopathic hemochromatosis].

A retrospective study of the case histories of 216 patients with idiopathic haemochromatosis has highlighted the frequency of cardiac involvement in this condition (53%). Two forms can be distinguished: a latent one (65%), in which the changes are predominantly electrocardiographic, and a clinical form (35%) with the features of congestive cardiomyopathy, notable for the rapidity of onset after right heart failure, the degree of cardiomegaly, the constant finding of abnormalities of ventricular repolarisation, the relative frequency of latent disorders of supra-His atrio-ventricular conduction, and the finding of elongation of the isovolumic contraction time on the phonomechanocardiogram. A haemodynamic profile is the same as for non-obstructive hypotonic cardiomyopathies, and is usually associated with a slow rise in left ventricular pressure. The cardiomyopathy, which is the most frequent cause of death, determines the prognosis in this condition. It may be found in association with diabetes and gonad failure. The finding of cardiomyopathy indicates basic treatment by veresection, which may be the only means of establishing a favourable outcome.

Adult↗

Cutaneous manifestations of idiopathic hemochromatosis. Study of 100 cases.

Skin manifestations of idiopathic hemochromatosis (IH) are presented in 100 cases with emphasis on the previously unrecognized high frequency of ichthyosis-like states and koilonychia. In 50 cases with treated and nontreated groups, histological siderosis and clinical skin pigmentation were found to decrease postphlebotomy whereas melanosis, histologically, did not. By skin biopsy in 50 cases versus controls, the location of siderosis in eccrine sweat glands seems specific for IH providing a strong basis for a probable diagnosis of the disease. There are correlations between skin manifestations and other signs of the disease.

Adult↗