Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.

A Case of Hypertrophic Osteoarthropathy Associated with Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma is a rare vascular tumor, which occurs in the lung, liver, bone, and soft tissue. Hypertrophic osteoarthropathy is a syndrome characterized by subperiosteal new bone formation, joint effusion and clubbing, and may be associated with cyanotic heart disease, chronic pulmonary disease, liver disease, and other miscellaneous diseases. The activation of endothelium and platelets has been suggested to be involved in the development of hypertrophic osteoarthropathy. We report a rare case of hypertrophic osteoarthropathy, which developed in association with hepatic epithelioid hemangioendothelioma with pulmonary metastasis. We also discuss the role of vascular endothelial growth factor in its pathogenesis.

Semiotécnica do hipocratismo digital / The semiology of digital clubbing

A interpretação racional dos achados semiológicos é fundamental para a prática médica. O presente artigo aborda a avaliação semiotécnica do hipocratismo digital. Em geral, as técnicas utilizadas para a avaliação e a quantificação desse achado semiológico não são precisas, sendo as formas discretas aquelas que apresentam maior dificuldade para o diagnóstico

Osteoartropatia hipertrófica secundária a fístula arteriovenosa pulmonar: relato de caso / Hypertrophic osteoarthropathy secondary to pulmonary arteriovenous fistula: case report

A osteoartropatia hipertrófica é uma síndrome caracterizada por proliferação periosteal, hipocratismo digital e comprometimento articular. Os autores descrevem o caso de um paciente com diagnóstico de osteoartropatia hipertrófica secundária a fístula arteriovenosa pulmonar, que apresentava concomitantemente quadro de poliartralgia envolvendo pequenas e grandes articulações

Osteoartropatia hipertrófica associada a carcinoma mucoepidermóide da glândula parótida / Hypertrophic osteoarthropathy associated with mycoepidermoid carcinoma of the parotid gland

A osteoartropatia hipertrófica tem sido mais freqüentemente associada a câncer de pulmäo. Raramente tem sido relacionada a doença metastática pulmonar, especialmente em osteossarcomas, sarcomas de partes moles, tumores do nasofaringe e uterinos. Relata-se o primeiro caso de uma síndrome de osteoartropatia relacionada a um carcinoma mucoepidermóide da glândula parótida metastático para pulmäo. A evidência desta síndrome sugeriu a presença de patologia intratorácica. A ressecçäo de múltiplos nódulos pulmonares trouxe alívio transitório dos sintomas