Prevalence of immune disturbances and chronic liver disease in family members of patients with primary biliary cirrhosis.

BACKGROUND AND AIMS: Primary biliary cirrhosis (PBC) has been reported in up to 4-6% of first degree relatives of patients with the disease. In addition, immune abnormalities, including hypergammaglobulinemia, autoantibodies and increased frequency of autoimmune disorders, were reported in family members of PBC patients. The aim of the present study was to investigate the prevalence of PBC in relatives of patients with PBC, and to investigate the occurrence of chronic liver disease (CLD) and immune abnormalities in these subjects. METHODS: One-hundred first degree relatives of 26 patients with PBC were interviewed and submitted to physical examination and determination of liver enzymes, gamma-globulin, bilirubin and auto-antibodies, including antinuclear (ANA), antismooth muscle (SMA), antimitochondrial antibodies (AMA) by indirect immunofluorescence (IIF) and anti-M2 antibody by immunoblotting (IB). RESULTS: Immune disturbances were rarely observed in relatives of PBC patients. Higher gamma-globulin levels, SMA and ANA were detected in four, eight and two family members, respectively. In most subjects, these autoantibodies were either in low titers or associated with concurrent diseases. Only four relatives had extrahepatic autoimmune diseases and another eight exhibited other CLD. Primary biliary cirrhosis was detected in a sister of one patient. Additionally, two other relatives of PBC patients who tested negative for AMA by IIF showed reactivity for anti-M2 by IB. CONCLUSIONS: Immune disturbances, including ANA and SMA, are uncommon in family members of PBC patients. Conversely, anti-M2 antibodies and overt PBC do occur in relatives of PBC patients, even in Brazil where the disease is quite rare.

Tratamento endoscópico da estenose péptica piloroduodenal com baläo dilatador / Endoscopic treatment of peptic pyloric duodenal stenosis by dilator balloon

Foram tratados oito pacientes com estenose péptica pilórica e do bulbo duodenal com baläo dilatador sob visäo endoscópica. A úlcera estenosante do bulbo duodenal apresentava-se em atividade em três casos e cicatizada em dois. A úlcera pilórica em um paciente apresentava-se em fase ativa e em dois, cicatrizada. O baläo dilatador foi posicionado na regiäo da estenose com auxílio de fio-guia em dois pacientes; e nos demais, esse posicionamento foi realizado sem fio-guia e sob visäo endoscópica. A dilataçäo foi hidrostática com injeçäo de água no baläo de 15mm de diâmetro em cinco pacientes e pneumática em três, com baläo de 20mm de diâmetro. Näo houve complicaçöes com o procedimento. Seis (75 por cento) pacientes apresentavam-se assintomáticos, sem recidiva de estenose ou da úlcera e com ganho de peso no período de seguimento de três a 34 meses. O método é seguro e eficaz no tratamento das úlcers pépticas estenosantes do piloro e do bulbo duodenal. Com o advento de medicamentos antiulcerosos potentes e a erradicaçäo do Helicobacter pylori, o tratamento dilatador poderá ser importante alternativa à cirurgia