Is steroid dependent Crohn's disease a separate entity?

Crohn's disease has been sometimes considered as a syndrome including different entities. In this prospect we tried to assess whether steroid dependent Crohn's disease could be a separate sub-group. Eighty five patients (mean follow-up: 6 years) with documented Crohn's disease were classified into 3 groups: 1--patients never treated with steroids (NS) (N = 37); 2--patients in whom steroids had been given but had been withdrawn (NSD) (N = 37); 3--patients dependent on continuous steroid therapy (SD) (N = 11). Ten variables were considered: age at onset, sex, CDAI, cumulative topography of lesions, extra-intestinal symptoms, albuminemia, ESR, surgical operations, annual frequency of relapses. Monofactorial analysis (analysis of variance and CHI2 test) showed group SD to be significantly different from group NS and in term of age at onset, CDAI, ESR, annual frequency of relapses, extra-intestinal symptoms, surgical operations. In contrast, a multivariate analysis of correspondences applied to the 3 groups and to 9 dichotomous variables showed that group SD is not a separate entity, but the limit of a continuum extending from group NS to group NSD. This was ascertained by a CHI2 test applied to the dichotomous variables. Thus, within the limits of this study Crohn's disease appears to be a homogeneous entity rather than a heterogeneous syndrome.

[Recklinghausen's disease with digestive localizations associated with gastric acid hypersecretion suggesting Zollinger-Ellison syndrome]. / Maladie de Recklinghausen à localisations digestives associée à une hypersécrétion gastrique acide evoquant un syndrome de Zollinger-Ellison.

We report the case of a 49 year old male patient who had Recklinghausen's disease associated with hyperchlorhydria. The principal features of Recklinghausen's disease were cutaneous localizations and countless digestive tumors, found mainly on the small bowel. The ultrastructural aspect of these tumors was neurinoma or schwannoma. Basal acid gastric hypersecretion and a positive secretin test were highly suggestive of a Zollinger-Ellison syndrome. Gastrinoma was not found at laparotomy even though a gastrin gradient had been demonstrated by pancreatic venous sampling. In a patient with Recklinghausen's disease, neuroendocrine tumors (APUDomas) should be looked for systematically.

[Multifocal cryptogenetic stenosing enteritis: an autonomous entity?]. / L'entérite sténosante multifocale cryptogénétique: une entité autonome?

Multifocal idiopathic stenosing enteritis has been described as a relapsing disease affecting the small bowel. The main anatomical characteristics are multiple non-specific inflammatory strictures. Six cases (4 men, 2 women) presenting this disorder were followed up for 3 to 22 years. Five patients were young. Iterative obstruction or chronic diarrhea and loss of weight were the outstanding clinical features. Hyposideremic anemia and protein-losing enteropathy were common. The anatomical substrate was numerous (2-19) short annular strictures situated in the ileum (75 p. 100). These strictures presented shallow ulcerations and underlying fibrosis which never extended beyond the submucosa. The small bowel wall was otherwise normal and did not show mucosal or vascular abnormalities at microscopic examination. In case of relapse, strictures developed distal to or in close contact with previous suture lines. Cultures for pathogenic bacteria were constantly negative. The natural history of these 6 cases and of 7 other previously published cases suggests that this disease is an autonomous disorder which must be differentiated from other inflammatory bowel disease entities such as Crohn's disease or tuberculosis.

[Tropical sprue. Apropos of a case observed in the Paris region]. / La sprue tropicale. A propos d'un cas observé en région parisienne.

A case of tropical sprue in a 73-year old parisian male returning from endemic areas is reported. Clinical manifestations were megaloblastic anemia, malabsorption and protein-losing enteropathy. Biopsy of the jejunal mucosa showed partial villous atrophy with thickening of the basement membrane region; fat droplets were observed inside and close to the epithelial cells on electron microscopy study. Fat intestinal absorption after a test meal was delayed. Clinical, biological and histological improvement was obtained with tetracycline and folic acid therapy. The particular clinical and anatomical features of this case, as well as the difficulties for establishing the diagnosis of tropical sprue in non-endemic areas are analyzed.

[Late recurrence of Horton's disease with sicca syndrome]. / Récidive tardive d'une maladie de Horton avec syndrome sec.

The authors report the case of an eighty-three-year-old woman with giant-cell arteritis who experienced recurrence five years after the withdrawal of corticosteroids which had been given for four consecutive years. This recurrence, demonstrated by arterial biopsy, was accompanied with Sjögren disease proven upon histological examination. With reference to this case-report, the various possible courses of protracted giant-cell arteritis are discussed; relapses occurring more than one year after therapy is discontinued are exceptional.

[Primary hepatic carcinoma disclosed by Budd-Chiari syndrome. Apropos of a case. Review of the literature]. / Carcinome hépatique primitif révélé par un syndrome de Budd Chiari. A propos d'un cas. Revue de la littérature.

Hepatic carcinoma revealed by Budd Chiari syndrome in an eighty-four-year-old female patient is reported. With reference to this observation and to the twenty-two other cases previously reported in the medical literature, the specific pathological and clinical features of Budd Chiari syndrome caused by primary carcinoma of the liver are reviewed. Histological features of the liver tumour are discussed.