A Case of Hepatopulmonary Syndrome in a Patient with Child-Pugh Class A Liver Cirrhosis / 결핵및호흡기질환

Hepatopulmonary syndrome (HPS) is characterized by a defect in arterial oxygenation that's induced by pulmonary vascular dilatation in the setting of liver disease. Some studies have shown the relationship between the presence of the HPS and the severity of liver disease, but there are only rare cases of HPS inpatient with Child-Pugh class A liver cirrhosis. We report here on a case of a 58 years-old male who suffered from progressive dyspnea for the previous few years. He was diagnosed with alcoholic liver cirrhosis 5 years previously. There was no significant abnormality on the chest radiograph and transthoracic echocardiography, but the arterial blood gas analysis revealed severe hypoxemia. Contrast-enhanced transesophageal echocardiograpy with agitated saline demonstrated a delayed appearance of microbubbles in the left cardiac chambers. Thus, he was finally diagnosed with HPS. This case suggests that we should consider HPS when a patient with compensated liver cirrhosis has unexplained dyspnea.

A Case of Necrotizing Sarcoid Granulomatosis / 대한내과학회지

Necrotizing sarcoid granulomatosis was first described by Liebow, who included it in the category of pulmonary angiitis and granulomatosis. Wegener's granulomatosis and limited Wegener's granulomatosis are also included in this category. Necrotizing sarcoid granulomatosis is characterized by histologically sarcoid-like granulomata, prominent and granulomatous vasculitis, and varying degrees of necrosis, radiographically pulmonary nodules but no enlarged hilar lymph nodes, clinically benign course. Whether this entity is a variant of necrotizig vasculitis with sarcoid reaction or sarcoidosis with prominent vascultic features and necrosis is not yet, clear, but many authors suggest the relationship with sarcoidosis from the observation of the similarity of histologic features and clinical course between the two entities. Patients are asymptomatic in about one fourth. Even symptomatic patients have vague symptoms such as cough, chest pain. Extrapulmonary involvement is rare. The prognosis is good even without therapy or with steroid alone, if necessary. We report a case that was diagnosed by open lung biopsy as necrotizing sarcoid granulomatosis, and it is assumed to be the first report of this entity in Korea.

A Case of Sarcoidosis Involving Bone Marrow, Skin, Uvea, Joints, Liver / 대한내과학회지

Sarcoidosis is a multisystem granulomatous disorder commonly affecting young adults. Diagnosis is confirmed by evidence of non-caseating granuloma in more than one organ. A case is presented where the diagnosis was made on a bone marrow biopsy, the first case in Korea. A 54-year-housewife was admitted because of multiple variable sized, skin papules and macules, who showed pancytopenia and evidence of liver cirrhosis on ultrasound. We performed skin biopsy and bone marrow biopsy, which showed non-caseating granulomas. The chest CT, opthalmologic examination was performed and showed the involvement of sarcoidosis of mediastinal lymph nodes and uvea. Multiple joints of both hands and feet also seemed to be involved in view of simple X-ray. By this case, we emphasize the significance of bone marrow biopsy in the sarcoidosis with pancytopenia.

Pulmonary Cryptococcosis / 결핵

A previously healthy 59-year old male patient was admitted due to cough and abnormal chest x-ray. Cough started 5 months ago and persisted. Two months before admission, abnormality in chest PA was detected. He had no symptom other than cough. He was nonsmoker and physical examination revealed no abnormal finding. His chest X-ray showed ill-defined 2x1 cm ovoid infiltration in left middle lung field. On chest computed tomography, it was located in the subpleural region of posterobasal segment of left lower lobe. Mediastinal lymphadenopathy was absent. Blood test and sputum examination were not diagnostic. Fluoroscopy-guided percutaneous needle biopsy revealed pulmonary cryptococcosis. After central nervous system involvement was excluded by spinal tap, oral ketoconazole therapy was started. The lesion decreased in size after 8 weeks of therapy and almost disappeared on follow-up chest X-ray 4 months later.

The Role of Open Lung Biopsy in Diagnosis and Treatment of Diffuse Interstitial Lung Disease in High-resolution Computed Tomography Era / 결핵

Objective: Diffuse interstitial lung disease (DILD) is a group of diverse diseases that share common clinical, radiologic, and pulomonary function features. Open lung biopsy (OLB) has been regarded as gold standard in differential diagnosis of DILD. However open lung biopsy is a invasive diagnostic tool not free of its own risk or complications. These days, high-resolution computed tomography (HRCT) has become an important diagnostic tool in DILD through its precise image analysis. In many instances, HRCT could provide specific diagnosis or, at least, provide information on the disease activity of DILD. The authors re-evaluate the role of open lung biopsy in this "HRCT era" by investigating the additional diagnostic gain and impacts on the treatment plan in patients who have undergone high-resoluticm CT. Method: Diagnoses obtained by high-resolution CT and open lung biopsy were compared and changes of treatment plans were evaluated retrospectively in 30 patients who had undergone open lung biopsy for the purpose of diagnosis of diffuse interstitial lung disease from March 1988 to June 1994. Results: High-resolution CT suggeted specific diagnoses in 22 out of 28 patients (78.6%) and the diagnoses were confirmed to be correct by open lung biopy in 20 of those 22 cases (91%). Open lung biopsy could not give specific diagnosis in 5 out of 30 cases (16.7%). In 5 out of 6 cases (83.3%) in whom high reolution CT was not able to suggest specific diagnosis, open lung biopsy gave specific diagnoses. Treatment plan was altered by the result of open lung biopsy in only 2 cases. Conclusion: The above findings suggest that in "HRCT era", when HRCT could suggest specific diagnosis, the need for open lung biopsy should be re-evaluated.

The Role of Open Lung Biopsy in Diagnosis and Treatment of Diffuse Interstitial Lung Disease in High-resolution Computed Tomography Era / 결핵

Objective: Diffuse interstitial lung disease (DILD) is a group of diverse diseases that share common clinical, radiologic, and pulomonary function features. Open lung biopsy (OLB) has been regarded as gold standard in differential diagnosis of DILD. However open lung biopsy is a invasive diagnostic tool not free of its own risk or complications. These days, high-resolution computed tomography (HRCT) has become an important diagnostic tool in DILD through its precise image analysis. In many instances, HRCT could provide specific diagnosis or, at least, provide information on the disease activity of DILD. The authors re-evaluate the role of open lung biopsy in this "HRCT era" by investigating the additional diagnostic gain and impacts on the treatment plan in patients who have undergone high-resoluticm CT. Method: Diagnoses obtained by high-resolution CT and open lung biopsy were compared and changes of treatment plans were evaluated retrospectively in 30 patients who had undergone open lung biopsy for the purpose of diagnosis of diffuse interstitial lung disease from March 1988 to June 1994. Results: High-resolution CT suggeted specific diagnoses in 22 out of 28 patients (78.6%) and the diagnoses were confirmed to be correct by open lung biopy in 20 of those 22 cases (91%). Open lung biopsy could not give specific diagnosis in 5 out of 30 cases (16.7%). In 5 out of 6 cases (83.3%) in whom high reolution CT was not able to suggest specific diagnosis, open lung biopsy gave specific diagnoses. Treatment plan was altered by the result of open lung biopsy in only 2 cases. Conclusion: The above findings suggest that in "HRCT era", when HRCT could suggest specific diagnosis, the need for open lung biopsy should be re-evaluated.