-In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are both autoimmune cholestatic liver disease and the association of these two conditions in the same patient is very rare. We report the case of a female patient presenting with a cholestatic liver disease and a panel of autoantibodies specific for PBC, including antibodies to mitochondrial E2-pyruvate dehydrogenase, gp-210 and Sp-100. Beside these findings, the liver biopsy revealed concentric fibrosis of small biliary ducts and the magnetic resonance cholangiography presented no abnormal findings. Diagnosis of small duct PSC/PBC overlapping was done. No description of this association was found in the literature. Clinical and serological features of this unusual finding are discussed.

New data concerning the management of autoimmune liver diseases have emerged since the last single-topic meeting sponsored by the Brazilian Society of Hepatology to draw recommendations about the diagnosis and treatment of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), overlap syndromes of AIH, PBC and PSC and specific complications and topics concerning AIH and cholestatic liver diseases. This manuscript updates those previous recommendations according to the best evidence available in the literature up to now. The same panel of experts that took part in the first consensus document reviewed all recommendations, which were subsequently scrutinized by all members of the Brazilian Society of Hepatology using a web-based approach. The new recommendations are presented herein.

Primary cutaneous amyloidosis is defined as the deposition of amyloid in the skin in the absence of systemic involvement. The association between primary cutaneous amyloidosis and other diseases, although rare, has been documented for connective tissue disorders such as systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. We report the case of a 41-year-old woman who developed primary biliary cirrhosis in association with primary cutaneous amyloidosis. This association has not been reported before in the literature.

A BSTRACT Background: Selective dorsal rhizotomy (SDR) is one of the surgical alternatives for treating spasticity, especially in children with spastic diplegia secondary to cerebral palsy (CP). It is becoming increasingly used, and the results of this operation need to be further highlighted. Aim: The main objective of this article was to present the results of such surgical procedure in a cohort of a specialized center, with a particular focus on a quantitative analysis (goniometry). Materials and Methods: Retrospective review of the medical records and gait analyses of a cohort of 34 patients diagnosed with CP submitted to elective SDR at our institution, in a period of 6 years, was carried out. All patients underwent a thorough clinical and neurological assessment, gait analysis at a dedicated laboratory, and magnetic resonance imaging of whole neuro-axis. Statistical Analysis: For continuous quantitative variables (goniometric angles and muscle tone), a t -student test was used. A scatterplot regression analysis was used for the comparison of modified Ashworth scale (mAS) scores and goniometry measurements. Results and Conclusion: In a mean follow-up of 3.2 years, SDR provides a measurable and consistent improvement in the motor function of spastic patients, as per range of motion and tonus scales, with low complication rates. It also allows for patients to reduce their use of muscle relaxants, even though their global mobility does not change significantly. Therefore, it should be considered for CP patients who suffer with the deleterious effects of spasticity.