LDN therapy appears effective and safe in subjects with active Crohn's disease. Further studies are needed to explore the use of this compound.

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessively inherited disorder consisting of the triad of oculocutaneous tyrosinase-positive albinism, prolonged bleeding time secondary to platelet storage pool defect and ceroid depositions within the reticuloendothelial system. Some patients also reportedly have gastrointestinal (GI) complications related to chronic granulomatous colitis, enterocolitis and extensive granulomatous perianal disease, the later previously unreported in the literature. These observations suggest that the GI complications of HPS are due to the development of classical Crohn's disease. The implications for disease pathogenesis and surgical management are discussed.

The case history of a child with autoimmune hemolytic anemia with complement activating warm antibodies of the pdl type is presented. This child also had giant cell hepatitis. Although plasma exchange is not curative in such a patient, the removal of antibodies and bilirubin by means of plasma exchange has alleviated the patient's condition on two occasions (at age 19 and 33 months respectively), and has permitted the institution of effective immunosuppression. A technique for plasma exchange is described, which by use of plasma filters and the usual hemodialysis equipment allows to safely perform the procedure in any pediatric hemodialysis unit. Its use in this patient stresses the technical practicability even in very young children. It is suggested that plasma exchange, if indicated, should be considered more often as part of a therapeutic concept.

130 patients with Crohn’s disease were colonoscopied in a multicenter trial. The obtained data were analyzed with respect to gathering information on the inflammation pattern, as well as on the importance and prognostic value of special lesions in Crohn’s colitis. In 52 patients a second endoscopy was performed at the end of the 2-year study period. Ulcerations and aphthous lesions were the most common lesions, followed by pseudopolyps, cobblestone lesions and stenosis. In general, there was an increasing, distal gradient in the frequency of severe lesions. Patients with Crohn’s colitis alone had more signs of inflammation than patients with additional involvement of the small intestine. A segmental pattern was the most common form of inflammation. The group of patients (14%) with a continuous pattern did not deviate from the whole collective in clinical activity. In patients with previous resections, inflammation near the anastomosis was accompanied more often than not by stenosis. Patients with ulcerations had a rather short time since confirmation of the diagnosis. Cobblestone lesions and pseudopolyps correlated with short symptomatology. During the follow-up of the study, patients taking steroids or a combination with prednisolone and sulfasalazine seem to have better results than those under placebo or sulfasalazine alone, as regards the more severe symptoms.