Long-term Follow-up of Patients with Diffuse Panbronchiolitis after Erythromycin Therapy / 대한내과학회지

BACKGROUND: Diffuse panbronchiolitis(DPB) is a chronic inflammatory disease affecting the respiratory bronchioles which was first described in Japan in 1966. DPB is prevalent in Japan and is known to be very rare in western countries. The first cases of DPB were reported in Korea in 1992 and the number of the patients has been increasing. The prognosis of DPR had been very poor because there had been no effective treatment for the disease. Hut it has been dramatically changed since the introduction of low-dose long-term erythromycin therapy. In Korea, there is rare experience of 1ong-term follow-up of DPH patients and we presents the results of mean 21.6 months of follow-up after erythromycin treatment. METHODS: We analyzed the long-term follow-up data of 25 DPH patients who were diagnosed in Seoul National University Hospital during the period from September 1989 to December 1994 and followed up more than 6 months with erythromycin therapy. We tried erythromycin 250mg b.i.d. on all the patients and analyzed the changes of subjective symptoms, physical signs, pulmonary function tests, and chest X-rays. RESULTS: 1) The mean follow-up period was 21.6 months. 2) Subjective symptoms improved in 96% of the patients within 3 months and 76% of the patients showed no symptom after 18 months of treatment. 3) Crackles and wheezing decreased in all patients within 3 months and completely disappeared in 76% of the patients after 18 months of treatment. 4) Diffuse small nodular lesions on chest X-ray decreased in 56% of the patients within 3 months and chest PA was normal in 32% of the patients after 12months of treatment. 5) FVC and FEV1 increased remarkably during the first 3 months and slowly increased thereafter, reaching normal level after 12 months of treatment. FEV1/FVC was 60.4% before treatment and in- creased slowly reaching 76.1% after 24 months of treatment. 6) Erythromycin therapy could be finished in 7 patients. The mean duration of medication was 26 months and no evidence of recurrence was found in 6 months of follow-up. 7) No patients had experienced the side effect of erythromycin, CONCLUSION: The prognosis of DPR is very goad when treated with erythromycin. And at least 2 years of erythromycin treatment seems to be needed for DPB patients.

A Case of X-linked Agammaglobulinemia / 대한내과학회지

X-linked agammaglobulinemia is attributed to the genetic defect for Bruton's tyrosine kinase at Xq22 region and the developmental arrest of pre-B lymphocytes. The characteristics of the disease are as follows; 1) male sex, 2) onset in infancy or early childhood, 3) severe panhypogammaglobulinemia, 4) normal cell mediated immunity, 5) recurrent, hardly controlled infection. The most common sites of infection are the respiratory tract and the gastrointestinal tract. The disease must be suspected when the recurrent, hardly controlled infection or the unusual, multiple sites of infection are present. Regular intravenous immune globulin approved the preventive effect against severe infection and fatal complication. But the final outcome remains grave in spite of intensive care. We report an adult case, 20 years old male patient, of X-linked agammaglobulinemia. He has been suffered from recurrent pneumonia and other sites of infection including meningitis and cellulitis. Pseudomonas aeruginosa was cultured from blood. X-linked agammaglobulinemia was diagnosed based on clinical history, severe panhypogammagloblinemia, lack of the gamma-fraction on the serum protein electrophoresis and absence of B-lymphocytes in peripheral blood. The clinical course waxed and waned until intravenous infusion of immune globulin, which dramatically improved pneumonia.

Diagnostic Approach to the Solitary Pulmonary Nodule: Reappraisal of the Traditional Clinical Parameters for Differentiating Malignant Nodule from Benign Nodule / 결핵

BACKGROUND: The solitary pulmonary nodule(SPN) presents a diagnostic dilemma to the physician and the patient. Many clinical characteristics(i.e. age, smoking history, prior history of malignancy) and radiological characteristics(i.e. size, calcification, growth rate, several findings of computed tomography) have been proposed to help to determine whether the SPN was benign or malignant. However, most of these diagnostic guidelines are based on the data collected before computed tomography(CT) has been introduced and lung cancer was not as common as these days. Moreover, it is not well established whether these guidelines from western populations could be applicable to Korean patients. METHODS: We had a retrospective analysis of the case records and radiographic findings in 114 patients presenting with SPN from Jan. 1994 to Feb. 1995 in Seoul National University Hospital, a tertiary referral hospital. RESULTS: We observed the following results ; (1) Out of 113 SPNs, the etiology was documented in 94 SPNs. There were 34 benign SPNs and 60 malignant SPNs. Among which, 49 SPNs were primary lung cancers and the most common histologic type was adenocarcinoma. (2) The average age of patients with benign and malignant SPNs was 49.7+/-12.0 and 58.1 +/-10.0 years, respectively(p=0.0004), and the malignant SPNs had a striking linear propensity to increase with age. (3) No significant difference in the history of smoking was noted between the patients with benign SPNs(13.0+/-17.6 pack-year) and those with malignant SPNs(18.6+/-25.1 pack-year) (p=0.2108). (4) 9 out of 10 patients with prior history of malignancy had malignant SPNs. 5 were new primary lung cancers with no relation to prior malignancy. (5) The average size of benign SPNs (3.01+/-1.20cm) and malignant SPNs(2.98+/- 0.97cm) was not significantly different(p=0.8937). (6) The volume doubling time could be calculated in 22 SPNs. 9 SPNs had the volume doubling time longer than 400 days. Out of these, 6 were malignant SPNs. (7) The CT findings suggesting malignancy included the lobulated or spiculated border, air-bronchogram, pleural tail, and lymphadenopathy. In contrast, calcification, central low attenuation, cavity with even thickness, well-marginated border, and perinodular micronodules were more suggestive for benign nodule. (8) The diagnostic yield of percutaneous needle aspiration and biopsy was 57.6%(19/33) of benign SPNs and 81.0%(47/58) of malignant SPNs. The diagnostic value of sputum analysis and bronchoscopic evaluations were relatively very low. (9) 42.3%(ll/26) of SPNs of undetermined etiology preoperatively turned out to be malignant after surgical resection. Overall, 75.4%(46/61) of surgically resected SPNs were malignant. CONCLUSIONS: We conclude that the likelihood of malignant SPN correlates the age of patient, prior history of malignancy, some CT findings including lobulated or spiculated border, air-bronchogram, pleural tail and lymphadenopathy. However, the history of smoking, the size of the nodule, and the volume doubling time are not helpful to determent whether the SPN is benign or malignant, which have been regarded as valuable clinical parameters previously. We suggest that aggressive diagnostic approach including surgical resection is necessary in patient with SPNs.