A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma

Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.

Simultaneous elevation of serum parathyroid hormone(PTH) and parathyroid hormone-related protein(PTHrP) in a case of lung cancer with hypercalcemia / 결핵

The parathyroid hormone related protein(PTHrP) is the most common causative peptide of humoral hypercalcemia of malignancy. In contrast, the serum level of parathyroid hormone(PTH) is low to undetectable in the majority of patients with malignancy associated hypercalcemia. Few cases exist in which the production and secretion of PTH by malignant nonparathyroid tumors have been authenticated. To our knowledge, there is very rare case in which a nonparathyroid tumor expressed simultaneously both the PTH and PTHrP. We report a case of squamous cell carcinoma of the lung with hypercalcemia which presented with simultaneous elevation of serum PTH and PTHrP. Severe hypercalcemia (serum calcium, 7.5mEq/L) was found in a 65-year-old man who had a squamous cell carcinoma of the lung without any body metastasis and detectable parathyroid abnormalities on isotope scintigraphy. The serum level of intact parathyroid hormone (PTH) concentration was markedly elevated as measured in two site radioimmunoreactive PTH assays (intact PTH 150pg/mL ; normal 9~55). The serum level of a PTHrP was also increased as measured in C-terminal region specific radioimmunoassay (PTHrP 99.1 pmol/L ; normal 13.8~55.3). There are no evidences of coincidental primary hyperparathyroidism in parathyroid MIBI scan and other imaging studies including neck ultrasonography and computed tomography. These results suggest that simultaneous elevation of serum PTH and PTHrP in this patient can be caused by production of both PTHrP and PTH in other nonparathyroid lesions such as squamous cell carcinoma.

A Case of Pulmonary Thromboembolism due to Congenital Antithrombin III Deficiency / 결핵

We report a case of congenital and familial antithrombin III deficiency developing massive pulmonary thromboembolism. A 44-year-old man was admitted to our hospital because of sudden chest pain and severe dyspnea. Five years ago, he was operated due to a mesenteric vein thrombosis of unknown cause. On admission, radioisotopic venogram showed deep vein thrombosis and lung scintigram showed multiple segmental perfusion defects. His plasma antithrombin III level was 10.5 mg/dL which was less than 50% of normal and those of a son and two daughters were also decreased. After treatment with tissue plasminogen activator, heparin and coumadin, his symptom and lung scintigram were significantly improved. As far as we reviewed, there were very rare reports with congenital antithrombin III deficiency presenting as pulmonary thromboembolism in Korea.

M-mode Ultrasound Assessment of Diaphagmatic Excursions in Chronic Obstructive Pulmonary Disease: Relation to Pulmonary Function Test and Mouth Pressure / 결핵

BACKGROUND: Respiratory muscle interaction is further profoundly affected by a number of pathologic conditions. Hyperinflation may be particularly severe in chronic obstructive pulmonary disease(COPD) patients, in whom the functional residual capacity(FRC) often exceeds predicted total lung capacity(TLC). Hyperinflation reduces the diaphragmatic effectiveness as a pressure generator and reduces diaphragmatio contribution to chest wall motion Ultrasonography has recently been shown to be a sensitive and reproducible method of assessing diaphragmatic excursion. This study was performed to evaluate how differences of diaphragmatic excursion measured by ultrasonography associate with normal subjects and COPD patients. METHODS: We measured diaphragmatic excursions with ultrasonography on 28 healthy subjects(16 medical studentz 12 age-matched control) and 17 COPD patientc Ultrasonographic measurements were performed during tidal breathing and maxima] respiratory efforts approximating vital capacity breathing using Aloka KEC-620 with 3.5 MHz transducen Measurements were taken in the supine posture. The ultrasonograpbic probe was positioned transversely in the midclavicular line below the right subcostal margin After detecting the right hetnidiaphragm in the B-mode the ultrasound beam was then positioned so that it was approximately parallel to the movement of middle or posterior third of right diaphragm. Recordings in the M-inodc at this position were made throughout the test Measurements of diaphragmatio excursion on M-mode tmcing were calculated by the average gap in 3 times-respiration cycle. Pulmonary functicn test(SensorMedics 2800), maximal inspiratory(Plmax) and expiratory mouth pressure(PEmax, Vitalopower KH-101, Chest) were measured in the seated posture. RESULTS: During the tidal breathing, diaphragmatic excursions were recorded 1.5 +/-0.5cm, 1.7+/-0.5cm and 1.5 +/- 0.6cm in medical students, age-matched control group and COPD patients, respectively. Diaphragm excursions during maximal respiratory efforts were significantly decreased in COPD patients (3.7+/-1.3cm) when compared with medical students, age-matched control group(6.7+/-1.3cm, 5.8+/-1.2cm, p<0.05). During maximal respiratory efforts in control subjects, diaphragm excursions were cowelatal with FEV1, FEV1/FVC, PEF, PIF, and height. In COPD patients, diaphragm excursions during maximal respiratory efforts were correlated with PEmax(maxinIal expiratory pressure), age, and %FVC. In multiple regression analysis, The combination of PEmax and age was an independent marker of diaplngnt excursions during maximal respiratory efforts with COPD patients. CONCLUISON: COPD subjects had smaller diaplragmatic excursions during maximal respintoty efforts than control subjects. During maximal respiratory efforts in COPD patients, diaphragm excursions were well correlated with PEmax. Those results suggest that diaphragm excursions during rnaximel respiratory efforts with COPD patients may be valuable at predicting the pulnionmy function.

Diagnostic Significance of TNF-alpha in Tuberculous and Non-Tuberculous Pleural Effusion / 결핵

OBJECTIVES: The differentiation of tuberculous effusion from the other causes of exudative pleural effusion remained difficult even with aids of biochemical analyses and pleural biopsy. As the pathophysiology of tuberculous pleural effusion is an enhanced cell mediated immunity, Adenosine deaminase(ADA) and various cytokines including Inteferon-γ, tumor necrosis factor alpha(TNF-α) are considered as useful diagnostic tools in differentiating exudative pleural effusion The author would like to demonstrate the diagnostic usefulness of TNF-α in the differentiation of exudative pleural effusion, and compared the discriminating ability of TNF-α with ADA. METHODS: Pleural fluids obtained from 80 patients (tuberculous : 39, malignant : 31, parapneumonic : 10) with exudate pleural effusions were processed for cell counts and biochemical analysis including ADA and TNF-α RESULTS: Tuberculous pleural fluid showed higher levee of ADA and TNF-α, 48.7α 32.7U/L and 184.1±214.2pg/mL than that of non-tuberculous effusion 26.0α41.3U/L and 44.1α114.2pg/mL, respectively (ADA, TNF-α : p<0.05, p<0.01). Receiver operating characteristics(ROC) curves were generated for ADA and TNF-α, and the best cut-off value for adenosine deaminase and TNF-α were considered as 30U/L and 15pg/ml, respectively. Comparing the area under the ROC curves, there was no significant difference between ADA and TNF-α CONCLUSION: For the differential diagnosis of tuberculous pleural effusion from the other causes of exudative pleural effusions, TNF-α as well as ADA was considered as useful diagnostic method. However adding TNF-α to ADA has no further diagnotic benefit than ADA alone.

A Case of Adenocarinoma of the Lung Associated with Multi-oragn Infarctions / 결핵

The relationship between neoplastic disease and thromboembolic disorders has been recognized since 1865, when Armand Trousseau first reported a high incidence of venous thrombosis in a series of patients with gastric carcinoma. The overall incidence of thromboembolic disease in patients with cancer has been reported to vary 1% to 15%. In a prospective study, Ambrus and associates reported that thrombosis and/or bleeding was the second most common cause of death in hospitalized cancer patients. We report a case who presented as a thromboembolic disease and subsequently confirmed to have an underlying lung malignancy. This 45 years old male patient visited our hospital with abdominal pain and distention of 3 days duration. Abdominal CT scan revealed multiple splenic and renal infarctions. On 20th hospital day, drowsy mental status was developed and hemorrhagic cerebral infarction was noted in brain CT scan. Chest CT scan revealed a 4cm sized spiculated mass on left lung apex and multiple paratracheal lymph adenopathy. With surgical biopsy of left supraclavicular lymph nodes, this patient was confirmed to have adenocarcinoma.

A Case of Ovarian Hyperstimulation Syndrome with Massive Pleural Effusion / 결핵

The ovarian hyperstimulation syndrome is a rare but serious complication of ovulation induction therapy with gonadotropin. The clinical manifestations are generalized edema, ascites with pleural effusion and may become life-threatening in severe cases. The pathophysiology is still unknown therefore, the treatment should be symptomatic and conservative. We report a case of severe OHSS with massive right pleural effusion in excess of ten liters after human menopausal gonadotropin therapy because of secondary infertility. Fluid and electrolyte imbalances were corrected and albumin was administered. A right chest tube was placed for a total of sixteen days, draining eleven liters of pleural effusion totally, resulting a dramatic decrease of pleural effusion and improvement of symptoms.

A Case of Hepatopulmonary Syndrome / 대한내과학회지

Hepatopulmonary syndrome consists of a triad of liver dysfunction, intrapulmonary vascular dilatation, and hypoxemia. This is one of the main causes of arterial hypoxemia in patients with chronic liver disease. The vascular abnormalities are precapillary dilatation, direct arterial-venous communication, and dilated pleural vessels. In this article, we report a case of hepatopulmonary syndrome in a 62-year-old woman who had complained progressively worsening dyspnea, platypnea, and orthodeoxia. She had huge splenomegaly, clubbing fingers and cyanosis of lip and fingers. Arterial blood gas analysis showed refractory arterial hypoxemia and orthodeoxia suggesting right-to-left "shunting". Chest X-ray showed increased interstitial markings on the lower part of right lung, In 99mTc-labeled macroaggregated albumin (MAA) lung perfusion scan, there was no perfusion defect in the lung, but labeled radionuclide were taken up in the intraabdominal organs, kidney, liver and spleen. The amount of shunted radionuclide were about 58 percent. In contrast echocardiography, microbubbles which were injected via cephalic vein were visualized in the left atrium at 4 cardiac cycles after leaving the right ventricle indicating intrapulmonary right-to-left "shunting" rather than intracardiac shunt. Pulmonary angiographic finding revealed diffuse blotchy arterial dilatation on both lung fields, especially lower lobes of both lungs. Current modalities of treatment of hepatopulmonary syndrome are the therapeutic embolization of direct arterial-venous communication for focal vascular dilatations, and TIPSS (Transjugular intrahepatic porto-systemic shunt) or liver transplantation for diffuse intrapulmonary vascular dilatations. Despite our recommendation of TIPSS, she refused the procedure and is under home oxygen therapy.