[Diabetes of idiopathic haemochromatosis and common diabetes mellitus. Results of a prospective study of 97 families with idiopathic haemochromatosis (author's transl)]. 1976

M Simon, and J L Alexandre, and C Scordia, and J P Hespel, and M Bourel

The purpose of this study was to investigate through an epidemiological approach two controversial aspects of the pathogenesis of the diabetes mellitus of idiopathic haemochromatosis (I.H.) : the possible inheritance of the gene(s) for common diabetes mellitus (C.D.), and the diabetogenic role of iron overload. More than 80% of the living first degree relatives of 97 patients with I.H. were examined, while data were collected by inquiry concerning first degree relatives who had refused investigations or had died. Data on the more distant family members were also collected by inquiry. Physical examination and estimation of serum iron level and unsaturated-iron-binding capacity were systematically performed. When an anomaly had been thus detected further investigation for iron overload was carried out by mean of a deferoxamine test and eventually by liver biopsy. Evaluation of carbohydrate metabolism included testing for post-prandial glycosuria, estimation of post-prandial blood sugar, and eventually an oral glucose tolerance test. The results were compared to those of an inquiry for family history of diabetes in 100 patients with C.D. successively admitted to our department. Among the first degree relatives of patients with C.D. the prevalence of overt diabetes was 33 of 612 (5.4 %); whereas in the I.H. group it was 8 of 735 (1.1 %). The differences between the C.D. and I.H. groups were significant, whether the total I.H. group (p less than 10(-5)) or only I.H. proposite having overt diabetes (p less than 2 X 10(-2)) were considered. With respect to the more distant relatives the number of affected families was significantly higher in the C.D. group (31 of 100) than in the total I.H. group (5 of 97 ; p less than 10(-5)) or in the I.H. sub-group diabetic proposite (3 of 36 ; p less than 10(-2)). The frequency of carbohydrate intolerance in relatives bore no relation to the carbohydrate pattern of propositi. Carbohydrate intolerance was frequently found in relatives with iron overload (17 of 72). However, no correlation was observed between blood sugar and serum iron level or unsaturated-iron-binding-capacity, relatively gross parameters. Thus, the pathogenesis of diabetes mellitus associated with I.H. remains uncertain, but the inheritance of gene(s) for common diabetes is unlikely to play a determinant role.

UI MeSH Term Description Entries
D007501 Iron A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN. Iron-56,Iron 56
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D003920 Diabetes Mellitus A heterogeneous group of disorders characterized by HYPERGLYCEMIA and GLUCOSE INTOLERANCE.
D005260 Female Females
D005602 France A country in western Europe bordered by the Atlantic Ocean, the English Channel, the Mediterranean Sea, and the countries of Belgium, Germany, Italy, Spain, Switzerland, the principalities of Andorra and Monaco, and by the duchy of Luxembourg. Its capital is Paris. Corsica,Saint Pierre and Miquelon,Miquelon and Saint Pierre,Miquelon and St. Pierre,St. Pierre and Miquelon
D005947 Glucose A primary source of energy for living organisms. It is naturally occurring and is found in fruits and other parts of plants in its free state. It is used therapeutically in fluid and nutrient replacement. Dextrose,Anhydrous Dextrose,D-Glucose,Glucose Monohydrate,Glucose, (DL)-Isomer,Glucose, (alpha-D)-Isomer,Glucose, (beta-D)-Isomer,D Glucose,Dextrose, Anhydrous,Monohydrate, Glucose
D006432 Hemochromatosis A disorder of iron metabolism characterized by a triad of HEMOSIDEROSIS; LIVER CIRRHOSIS; and DIABETES MELLITUS. It is caused by massive iron deposits in parenchymal cells that may develop after a prolonged increase of iron absorption. (Jablonski's Dictionary of Syndromes & Eponymic Diseases, 2d ed) Diabetes, Bronze,Bronze Diabetes,Bronzed Cirrhosis,Familial Hemochromatosis,Genetic Hemochromatosis,Haemochromatosis,Hemochromatoses,Iron Storage Disorder,Pigmentary Cirrhosis,Primary Hemochromatosis,Troisier-Hanot-Chauffard Syndrome,Von Recklenhausen-Applebaum Disease,Bronzed Cirrhoses,Cirrhoses, Bronzed,Cirrhoses, Pigmentary,Cirrhosis, Bronzed,Cirrhosis, Pigmentary,Disease, Von Recklenhausen-Applebaum,Diseases, Von Recklenhausen-Applebaum,Disorder, Iron Storage,Disorders, Iron Storage,Familial Hemochromatoses,Genetic Hemochromatoses,Haemochromatoses,Hemochromatose,Hemochromatoses, Familial,Hemochromatoses, Genetic,Hemochromatosis, Familial,Hemochromatosis, Genetic,Iron Storage Disorders,Pigmentary Cirrhoses,Recklenhausen-Applebaum Disease, Von,Recklenhausen-Applebaum Diseases, Von,Storage Disorder, Iron,Storage Disorders, Iron,Syndrome, Troisier-Hanot-Chauffard,Syndromes, Troisier-Hanot-Chauffard,Troisier Hanot Chauffard Syndrome,Troisier-Hanot-Chauffard Syndromes,Von Recklenhausen Applebaum Disease,Von Recklenhausen-Applebaum Diseases
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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