Short- and long-term evolution of body composition in 1389 obese outpatients following a very low calorie diet (Pro'gram18 VLCD).

One thousand three-hundred and eighty-nine obese outpatients were followed by 28 practitioners. They were enrolled in a multidisciplinary weight control program for at least 1 year. The major components of the program include a commercial very low calorie diet (Pro'gram18 VLCD), behavior modification, and exercise. There was a significant decrease in body weight compared with baseline of approximately 12.3+/-5.3 kg at the end of the maintenance period; the weight loss was achieved essentially at the expense of fatty mass, -10.3+/-5.5 kg at 90 days while fat-free mass loss was -2.0+/-2.5 kg at 90 days. Mean serum total cholesterol and triglycerides were also lowered and systolic and diastolic blood pressure and fasting blood glucose values were normalized at the end of the weight-loss phase. Obese outpatients lost substantial amounts of weight using VLCD, reduced the risk factors associated with obesity, and had encouraging long-term results, with weight loss maintained at 2-year follow-up.

[The cardiomyopathy of idiopathic hemochromatosis]. / La myocardiopathie de l'hémochromatose idiopathique.

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.

Optimum duration of antithyroid drug treatment determined by assay of thyroid stimulating antibody in patients with Graves' disease.

OBJECTIVE: To determine the optimal duration of antithyroid drug treatment by monitoring serum thyroid stimulating antibody values in patients with Graves' disease. DESIGN: Prospective longitudinal trial of patients with Graves' disease followed up for 24 months after withdrawal of treatment. SETTING: Tertiary referral centre. PATIENTS: A total of 64 consecutive patients with untreated Graves' disease, eight of whom were subsequently excluded. Fifty six patients completed the study. INTERVENTIONS: All patients were treated initially with carbimazole 40 mg, then with decreasing doses that maintained a euthyroid state. Treatment was scheduled to continue for 18 months but was withdrawn earlier if serum thyroid stimulating antibody became undetectable. END POINT: Serum values of thyroid stimulating antibody (assayed by stimulation of human thyroid cells in vitro) and thyroid hormones and thyroid state every three months during treatment and afterwards every six months for 24 months. MEASUREMENTS AND MAIN RESULTS: In 44 patients serum thyroid stimulating antibody became undetectable during treatment and treatment was withdrawn (median duration of treatment nine months, range 3-18 months). In 12 patients the antibody could be detected during 18 months of treatment. Among the first group of 44 patients initial values of the antibody before treatment were significantly lower than in the second group of 12 patients (median 225% (range 138-1236%) v 570% (250-1480%), p less than 0.001); the incidence of relapse was also lower (41% v 92%, p less than 0.001); and among those who did relapse the disease free interval after treatment was longer (median 12 months v 1 month, p less than 0.001). Moreover, the initial median serum values of thyroid stimulating antibodies were not related to the occurrence of relapse or remission as these did not differ between patients who did and did not have a relapse (median 267% (range 139-1480%) v 220% (range 138-1236%). CONCLUSION: Monitoring of serum thyroid stimulating antibody was a good guide to the duration of treatment as it allowed the treatment period to be considerably shortened in a large group of patients with no loss of efficiency.

[A severely disturbed lipid profile]. / Un bilan lipidique très perturbé

We report here the case of a 30-year-old woman presenting a disturbed lipid profile since her childhood. The rarity of this pathology (Anderson' disease or chylomicron retention disease) and its recent recognition explain its late detection in this case. The description of the biochemical profile is interesting especially the low level of vitamin A and E which explains the severity of the disease. A vitamin enriched-diet may be useful.

[Pregnancy and adrenal insufficiency. Apropos of a case and reminder of management procedures]. / Grossesse et insuffisance surrénale. A propos d'une observation, rappel de la conduite à tenir.

The authors report a case of pregnancy which proceeded normally in a patient who had Addison's disease. This is not a common condition for obstetricians to meet. Thanks to replacement hormone therapy a patient with Addison's disease can become fertile and pregnancy usually starts in a patient whose chronic adrenal insufficiency (Addison's disease) is known and has been treated. The consequences of this association are reviewed: In the first trimester of pregnancy the hormone state may become more disturbed because of intestinal troubles and can become acutely decompensated because of the "stress" of delivery and its immediate consequences. There are no maternal or fetal repercussions so long as hormone replacement is adequate. The delivery can be by the vaginal route unless there are obstetric contra-indications. These are the particular precautions that have to be undertaken, together with regular supervision of the mother to maintain her hormonal equilibrium: Looking for, prevention and treatment of any condition that may lead to adrenal decompensation: these are particularly intestinal upsets in the first trimester and urinary tract infections. At the end of pregnancy the patient should be hospitalised with resuscitation carried out during and after delivery. These may need to be performed in addition to any surgical procedure that is carried out. The infant does not have any particular treatment and can breast-feed.

[Peripheral distribution of lymphocyte subpopulations in Basedow's disease. Decrease in suppressor T-lymphocytes]. / Répartition périphérique des sous-populations lymphocytaires dans la maladie de Basedow. Diminution des lymphocytes T suppresseurs.

The distribution of B- and T-lymphocyte subpopulations was studied in peripheral blood of 45 patients with untreated Graves' disease and 45 sex- and age-matched healthy controls. Blood samples were taken at the same hour in all subjects. The following tests were performed: HTLA and E (AET) for relative T-lymphocyte count, complement receptor (EAC) and surface immunoglobulins (IgS, IgG, IgM, IgA, kappa, lambda) for relative B-lymphocyte count. In 23 subjects of each group the subpopulations of T-lymphocytes were defined by their reactivity with monoclonal antibodies OKT8 (T-suppressor cells) and OKT4 (T-helper cells). Compared with the control group, patients with Graves' disease showed a decrease in the number of T-lymphocytes (HTLA: P less than 0.05: EAET: P less than 0.001) a decrease in T-suppressor cells (P less than 0.01), and no significant difference in B-lymphocytes and T-helper cells. Thus, the main lymphocyte characteristic in Graves' disease is a decrease in the relative value of T-cells, specifically affecting T-suppressor cells.

Hodgkin's disease and hyperthyroidism.

In recent years we have had the occasion to observe hyperthyroidism in 6 patients with Hodgkin's disease. All patients had received Mantlefield irradiation and were disease-free when hyperthyroidism appeared. Hyperthyroidism allows three different pictures to be distinguished: 1 case report of Graves' disease without ophthalmopathy, 1 case report of Hashimoto's thyroiditis corresponding to a particular form called hashitoxicosis, and 4 case reports of atypical silent thyroiditis. Reports concerning case studies of postirradiation Graves' disease or Hashimoto's thyroiditis during Hodgkin's disease are only to be found exceptionally. Atypical silent thyroiditis was recently individualized, but no postirradiation case studies have been reported. It is suggested that these 6 cases represent a radiation-induced immune thyroid disease: physiopathology and predisposing factors are discussed.

[Diabetes of idiopathic haemochromatosis and common diabetes mellitus. Results of a prospective study of 97 families with idiopathic haemochromatosis (author's transl)]. / Diatete de l'hémochromatose idiopathique et diabéte commun. Résultats d'une étude prospective de 97 familles d'hémochromatose idiopathique

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.

Serum ferritin as a possible marker of the hemochromatosis allele.

To determine whether a correlation exists between the biochemical expression of hemochromatosis and the HLA genotype, we studied 174 family members of 32 persons with the disease. Persons who shared both HLA haplotypes with the proband (and presumably having two hemochromatosis alleles) differed significantly from those who shared only one haplotype (and presumably having one hemochromatosis allele) in terms of serum iron (P less than 0.001 for both sexes), unsaturated iron-binding capacity (P less than 0.01 for female and P less than 0.0001 for male subjects) and serum ferritin (P less than 0.0001 for female and P less than 0.00001 for male subjects). The only significant difference between relatives having one hemochromatosis allele and age and sex-matched controls was related to serum ferritin values in male subjects (P less than 0.05, despite considerable overlap). In our hands, serum ferritin was the best indicator of disordered iron metabolism and was elevated among most homozygous but among few heterozygous family members.

[Demonstration by iron overloading study and HLA genotyping of recessive transmission of idiopathic haemochromatosis in two pseudodominant pedigrees (author's transl)]. / Hérédité récessive de l'hémochromatose idiopathique: deux observations de transmission pseudo-dominante reconnue comme récessive par l'étude de la surcharge en fer et des génotypes HLA dan les familles.

We studied iron overloading and HLA genotype in two families with overt forms of idiopathic haemochromatosis in two successive generations. In each family the spouse of the patient with overt haemochromatosis in the first generation had clinical and laboratory signs of moderate iron overload and a HLA haplotype A3, B14 and A3, B7 respectively--which is frequently associated with the haemochromatosis gene. This specific HLA haplotype had been transmitted to the second generation patient with overt disease, which thus could be considered as having received a haemochromatosis gene from each parent. Although the finding of cases of overt disease in successive generation firstly suggests a dominant transmission the genetical analysis of these families lead to further strong argument in favour of recessive inheritance of idiopathic haemochromatosis.

Idiopathic hemochromatosis: a study of biochemical expression in 247 heterozygous members of 63 families: evidence for a single major HLA-linked gene.

The hypothesis has been advanced that the two genes on chromosome 6 determining idiopathic hemochromatosis are not identical alleles and therefore that the disease is not recessively inherited, but rather that two different genes are involved. A study of 63 families points to: (a) the rarity with which a single hemochromatosis gene finds biochemical expression (in only 1 of 5 cases), as revealed through determinations of serum iron, serum ferritin and the desferrioxamine test; (b) no difference in HLA-antigen marking between genes with and those without biochemical expression: (c) no difference other than that produced by chance in the biochemical expression of the two genes in families; and (d) the finding in one highly informative family of identical expression of the two genes. It is concluded that idiopathic hemochromatosis is determined by two homologous alleles in accordance with the classical mode of recessive inheritance.