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.

Hypertrophic osteoarthropathy associated to lymphoepithelioma of the nasopharynx: a case report / Osteoartropatia hipertrófica associada a linfoepitelioma de nasofaringe: relato de caso

Malignant neoplasms of the nasopharynx are very rare and has two peaks of incidence: below the age of 30, and between the 4th and 5th decade of life. It is, however, uncommon after the 60 years of age. In rare cases, some patients may present, in the form of paraneoplastic syndrome, hypertrophic osteoarthropathy (HOA). In this case report, we describe a case of HOA from lymphoepithelioma of the nasopharynx in a 77-year-old male patient, representing an extremely unusual condition.

Neoplasias malignas de nasofaringe são muito raras e apresentam dois picos de incidência: antes dos 30 anos e entre a 4ª e 5ª década de vida. No entanto, é incomum após os 60 anos de idade. Em raros casos, alguns pacientes apresentam na forma de síndrome paraneoplásica a Osteoartropia Hipertrófica (OAH). Este relato de caso, nós descrevemos um caso de OAH associada a linfoepitelioma de nasofaringe em um paciente de 77 anos, representando um condição extremamente incomum.

Osteopatia hipertrófica em sete cães / Hypertrophic osteopathy in seven dogs

Os achados clínico-patológicos de sete casos de osteopatia hipertrófica em cães foram estudados. Os cães afetados apresentavam sinais clínicos indicativos de envolvimento ósseo e lesões macroscópicas principalmente nos ossos longos dos membros. As lesões consistiram de neoformação óssea periosteal detectada em exame radiográfico, na inspeção óssea durante a necropsia e, com grande nível de detalhamento, em espécimes ósseos macerados. A proliferação óssea observada era parcialmente circunferencial e ocorreu principalmente na diáfise dos ossos longos. Era constituída por trabéculas ósseas de tamanho e espessura irregulares que estavam dispostas de forma perpendicular ao córtex ósseo original. Em todos os casos as lesões de osteopatia hipertrófica foram associadas a neoplasmas pulmonares (primários ou metastáticos).

Clinical and pathological aspects of hypertrophic osteopathy in seven dogs were studied. Affected dogs had clinical signs of bone involvement and lesions mainly in the long bones of the limbs. The lesions consisted of periosteal new bone formation detected on radiographic examination, inspection of bones during the necropsy, and with great detail, in macerated bone specimens. The bone proliferation observed was partially circumferential and occurred mainly in the diaphysis of long bones. It consisted of bone trabeculae of irregular size and thickness that were arranged perpendicularly to the original bone cortex. In all cases the lesions were associated with lung neoplasms (primary or metastatic).

Metastatic Carcinoma Manifesting as Clubbed Finger in a Patient with Lung Cancer / 대한피부과학회지

Metastatic carcinoma on the hand occurs in 0.1% of all metastasis and the diagnosis is difficult since it frequently mimics other conditions such as infection, osteomyelitis, gout, and pseudoclubbing. A 70-year-old woman with lung carcinoma was consulted for the evaluation of a painful swelling patch on the tip of her right 2nd finger. She was initially treated with antibiotics due to the misdiagnosis of the condition as an infection. Her pain persisted for a month. The skin biopsy revealed a metastatic adenocarcinoma with papillary growth pattern. We report a rare and an interesting metastatic carcinoma manifesting as clubbed finger.

Regressão do hipocratismo digital em pacientes com câncer de pulmão tratados cirurgicamente / Reversal of digital clubbing in surgically treated lung cancer patients

OBJETIVO: Estudar, por meio de avaliações objetivas, a ocorrência de regressão do hipocratismo digital (HD) em pacientes com câncer de pulmão, tratados cirurgicamente, e revisar a literatura sobre o assunto. MÉTODOS: Sessenta e um pacientes com câncer de pulmão não-pequenas células-40 com e 21 sem HD-foram tratados por cirurgia de ressecção pulmonar. Onze deles (18 por cento) também receberam radioterapia pós-operatória. No período pré-operatório e no 7º, no 18º e no 90º dia pós-operatório, o ângulo hiponiquial (AH) e a relação entre as espessuras falangeana distal e interfalangeana (EFD/EIF) foram determinados sobre imagens da sombra dos dedos indicadores em perfil. Uma revisão da literatura sobre regressão do HD (1954-2007) também foi efetuada. RESULTADOS: Do período pré-operatório ao 90º dia pós-operatório, o AH diminuiu de 200,5 ± 5,0º para 193,3 ± 6,8º (p < 0,001), e a relação EFD/EIF, de 1,014 ± 0,051 mm para 0,956 ± 0,045 mm (p < 0,001) no grupo de 40 pacientes com HD. Em 33 (82,5 por cento), o AH e a relação EFD/EIF diminuíram, mas, em 7 (1,7 por cento), 6 com evolução desfavorável, esses valores não se reduziram. Nos 21 pacientes sem HD, tanto o AH (184,5 ± 5,5º) como a relação EFD/EIF (0,937 ± 0,046 mm) permaneceram inalterados após a cirurgia. Na literatura (1954-2007) foram encontrados 52 casos em que a regressão do HD, observada em diversas condições clínicas, foi explicitamente referida, 5 dos quais eram casos de câncer de pulmão. CONCLUSÕES: O HD em pacientes com câncer de pulmão regride na maioria dos casos após tratamento cirúrgico efetivo do tumor, o que pode também ocorrer em pacientes com outras condições.

OBJECTIVE: To objectively evaluate the reversal of digital clubbing (DC) in a series of surgically treated lung cancer patients, and to review the literature on the subject. METHODS: Sixty-one patients with non-small cell lung cancer-40 with and 21 without DC-were treated by pulmonary resection. Eleven (18 percent) received additional postoperative radiation therapy. Preoperatively, as well as on postoperative days 7, 18, and 90, the hyponychial angle (HA) and the distal phalangeal depth/interphalangeal depth (DPD/IPD) ratio were determined on profile shadow projections of the index fingers. A review of the literature on reversal of DC (1954-2007) was also performed. RESULTS: From the preoperative period to postoperative day 90, HA decreased from 200.5 ± 5.0º to 193.3 ± 6.8º (p < 0.001), and the DPD/IPD ratio decreased from 1.014 ± 0.051 mm to 0.956 ± 0.045 mm (p < 0.001) in the group of 40 patients with DC. The HA and the DPD/IPD ratio decreased in 33 (82.5 percent) but remained the same in 7 (1.7 percent), 6 with unfavorable evolution. In the 21 patients without DC, HA (184.5 ± 5.5º) and the DPD/IPD ratio (0.937 ± 0.046 mm) remained unchanged after surgery. In the literature (1954-2007), we found 52 cases, 5 of which were lung cancer cases, in which reversal of DC, observed in several clinical conditions, was explicitly reported. CONCLUSION: In most lung cancer patients, DC resolves after effective surgical treatment of the tumor, as can occur in patients with other conditions.

unusual side effect of interferon alfa 2a: digital clubbing

Interferon Alfa has been widely used to treat chronic hepatitis C virus infection. In this report, we present a case series of two patients referred to Sarwar Zuberi Liver Centre, Civil Hospital Karachi, who suffered from chronic hepatitis C. After getting detailed clinical examination and baseline work up prior to starting treatment, these patients were offered therapy with usual recommended dose of 3 million units of alpha-interferon subcutaneously thrice weekly. Both these patients developed clubbing of fingers during the course of treatment, one developing it during the 2[nd] month while the other during the 4[th] month. It was of grade II in one patient and of grade III in another and was bilateral in both the cases. Clubbing was not presented prior to start of treatment and no other secondary cause of clubbing was found in any of the case. These patients were not on any other drug that is known to interfere with interferon or can be associated with clubbing. No national or international data regarding such unusual side effect is available. Whether this effect is idiosyncratic or dose related and whether it is reversible or not after completion of treatment is yet to be established

Síndromes reumáticas paraneoplásicas / Paraneoplastic rheumatic syndromes

Este artigo faz uma revisão da literatura sobre síndromes reumáticasparaneoplásicas. Doenças reumáticas podem, algumas vezes, termanifestações associadas com o desenvolvimento de processostumorais, sendo, assim, consideradas paraneoplásicas. Certasmanifestações têm estudos que confi rmam a forte associação commalignidade; entre essas manifestações estão a oteoartropatiahipertrófi ca, a poliartrite paraneoplásica, a dermatomiosite e avasculite. Por outro lado, o tratamento oncológico com quimioterapiae com radioterapia pode resultar em complicações reumatológicas.

Osteoartropatia hipertrófica (OAH) / Hypertrophic osteoarthropathy

La primera descripción que se tiene de esta patología fue hecha por Hipócrates, posteriormente en 1868 se la llamó “hiperostosis del esqueleto” y se confundía con la acromegalia. En 1887 y 1888 se describía asociada a una dermatopatía llamada “cutis verticis girata”. En 1890 se la llamó “osteoartropatía hipertrofiante neumónica” notando desde ese momento la gran asociación con el pulmón apareciendo luego el término de “paquidermoperiostosis”. Curiosamente hay descrita una enfermedad similar en perros.

Two Case of Pulmonary Arteriovenous Fistula Treatment Depending on the Presence of Pulmonary Hypertension / 소아과

Pulmonary arteriovenous fistulas(PAVFs) is a rare disorder that occurs in two to three children per 100,000 population. It is presented as absence of intervening capillary beds between the pulmonary artery and vein with resultant persistent right to left shunt. Other causes include trauma, liver cirrhosis, malignancy and schistosomiasis. It is mostly asymptomatic, but it may present with respiratory difficulty, cyanosis, clubbed fingers induced by right to left shunt or hemoptysis, polycythemia and epistaxis. Major complications, such as brain abscess, brain embolism, paradoxical embolism and subacute infective endocarditis can be devastating, so therapeutic intervention is recommended in all patients. However, removal of low-resistance fistulas can aggrevate pulmonary hypertension, so detection of increased pulmonary pressure is important. We report two patients:One a 42 year-old male with PAVFs treated with coil embolization, and a 42 year-old female who was treated with anticoagulants due to pulmonary hypertension.

Epithelioid hemangioendothelioma: a case with large solitary pulmonary mass and hypertrophic osteoarthropathy

We describe a case of pulmonary epithelioid hemangioendothelioma [PEH], previously known as intravascular bronchoalveolar tumor, in a 48-year-old woman with an initial diagnosis made by CT-guided transthoracic needle biopsy. This is a rare disease, with approximately 50 cases described in the literature. To our knowledge, this has not been previously described in the English-language literature. This tumor can affect multiple organs. PEH is usually multifocal or small sized; hypertrophic osteoarthropathy is uncommon. This patient presented with hypertrophic osteoarthropathy and large solitary pulmonary mass, rare presentations of this uncommon tumor

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.

Osteoarthropathie hypertrophiante revelant un thymome malin chez un enfant. Apport de l'imagerie

Association of hypertrophic osteoarthropathy and malignant thymoma is very rare in children. Authors report a case of a 14 year old girl followed for rheumatic fever since she was 8 years old. She was explored for arthralgia. The exam showed Hippocratic fingers, soft tissues hypertrophy of lower limbs and dyspnoea. Plain radiography had demonstrated symmetrical ossifying periostisis in tubular bones of lower limbs evocating hypertrophic osteoarthropathy diagnosis. Chest radiography has demonstrated bilobate anterior mediastinal mass. Ultrasonography and MRI revealed a voluminous anterior mediastinal mass with cystic and fleshy components. Thymic adenocarcinoma diagnosis was made by surgical biopsy

Unusual Presentation of Thymic Carcinoma: Hypertrophic Osteoarthropathy

Hypertrophic osteoarthropathy is characterized by clubbing and periosteal new bone formation along the shaft of the long bones of the extremities. Although various intrathoracic malignancies have been associated with the development of HOA, it has been extremely rare for HOA to occur in a patient with a thymic carcinoma. Recently, we experienced a 63-year-old woman diagnosed as a thymic carcinoma with hypertrophic osteoarthropathy. She had both digital clubbing and cortical thickening in her lower extremities identified radiologically. We herein describe this case with a review of the literature.