• Journal of Chest Surgery
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• 제17권1호
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• pp.118-124
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• 1984

Congenital Cystic Adenomatiod Malformation (C.C.A.M.) is rare, but one of the most common congenital pulmonary anomalies that cause acute respiratory distress in the newborn infants. It is characterized and differentiated from the diffuse pulmonary cystic disease pathologically, i.e. adenomatoid appearance due to marked proliferation of the terminal respiratory components. An 2/12 year old male patient was suffered from respiratory distress and cyanosis on crying since birth, but no specific therapy was given. With progression of symptoms, he came to Korea University Hospital for further evaluation and then transfered to Dept. of Chest Surgery for operative correction under the impression of Congenital Obstructive Emphysema suggested by a pediatrician. On gestational and family history, there was nothing to be concerned such as congenital anomaly. Physical examinations showed; moderate nourishment and development (Wt. 5.5kg), cyanosis on crying, both intercostal and lower sternal retraction on inspiration, Lt. chest building with tympany, Rt. shifting of cardiac dullness, decreased breathing sound with expiratory wheezing on entire Lt. lung field, decreased breathing sound on Rt. upper lung filed, and tachycardia. The remainders were nonspecific. Laboratory findings were normal except WBC $14000/mm^3$ (lymphocyte 70%), Hgb 9.8m%, Hct 28%, negative Mantaux test, and sinus tachycardia and counter-clockwise rotation on EKG. Preoperative simple Chest PA revealed marked hyperlucent entire Lt. lung, herniation of Lt. upper lobe to Rt., collapsed Rt. upper lobe, tracheal deviation and mediastinal shifting to Rt., and no pleural reaction. At operation, after Lt. posterolateral thoracotomy, 4th rib was resected. Operative findings were severe emphysematous changes limited to both lingular segmentectomy was done. The resected specimen showed slight solidity, measuring $8{\times}4.5{\times}2cm$ in size, and small multiple cystic spaces filled with air. Microscopically, entire tissue structures were glandular in appearance, cyst were lined by ciliated columnar epithelium, and occasional cartilages were noted around the cystic spaces. Bronchial elements were dilated but normal pattern on histologically. The patient had a good postoperative courses clinically and radiologically, and discharged on POD 10th without event. The authors report a case of Cogenital Cystic Adenomatoid Malformation (C.C.A.M.)

• Tuberculosis and Respiratory Diseases
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• 제52권2호
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• pp.179-185
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• 2002

원발성 쇼그렌 증후군 (Primary Sj$\ddot{o}$gren's syndrome) 환자에서 폐에 발생하는 Bronchus-associated lymphoid tissue(BALT) 림프종은 드문 질환이다. 본 환자는 49세 여자 환자로 5년 전부터 안구 건조증 및 구강 건조증이 점차 진행하였으나 그냥 지내던 중 2년 전부터 운동시 호흡곤란 및 전신 무력감이 계속 진행하여 본원에 입원하였다. 흉부청진상 호흡음이 거칠게 들렸으며 양측 폐야 전체에 걸쳐 흡기시 악 설음이 관찰되었다. 형광항핵항체시험 (FANA)이 양성으로 "speckled pattern"을 보였으며 anti-SSA(Ro) Ab 및 anti-SSB(La) Ab는 강양성 소견을 보였다. 단순 흉부 방사선 사진 상 양측 폐에서 미만성의 망상 결절성 음영 증가가 관찰되었으며 고해상도 흉부 컴퓨터 단층촬영상에서 양측 폐에 걸쳐 미만성 분포를 보이는 모자이크 양상의 비균질성 감쇠 (mosaic pattern of inhomogeneous attenuation) 소견이 관찰되었다. 경기관지 폐 조직 생검상 림프구의 침윤이 관찰되었고 개흉 폐 생검술을 이용하여 얻은 폐조직의 병리 소견에서 'low grade marginal zone B cell lymphoma of BALT(bronchus associated lymphoid tissue) type'에 합당한 소견을 얻어 원발성 쇼그렌 증후군 (Primary Sj$\ddot{o}$gren's syndrome) 환자에서 폐에 발생한 Bronchus-associated lymphaid tissue(BALT) 림프종으로 확진되었다.