• Clinical and Experimental Pediatrics
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• 제61권1호
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• pp.30-34
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• 2018

Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS sequencing revealed a somatic mutation in Codon12 ($GGT{\rightarrow}GAT$), suggesting the development of a mucinous adenocarcinoma in the background of mucinous metaplasia. Mucinous adenocarcinoma is the most common lung tumor associated with CPAM, but it generally occurs in older children and adults. Further, all cases in the literature are of type I CPAM. This case in a neonate indicates that malignant transformation can occur very early in type II CPAM.

• Journal of Chest Surgery
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• 제13권3호
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• pp.292-297
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• 1980

The congenital cystic adenomatoid malformation of the lung consists of an enlarged, meaty, multicystic lobe with smooth-walled cysts of varying sizes, which can communicate with major bronchi through malformed air passages that usually lacks cartilage. This abnormality is usually symptomatic in infancy with signs of respiratory distress such as tachypnea, substernal retraction and cyanosis. Prompt surgical resection is choice of treatment in life-threatening respiratory distress patients. We recently experienced a case of congenital cystic adenomatoid malformation of the lung in a patient with situs inversus totalis. The patient was 40 days old female who showed severe respiratory difficulty. Emergency left middle lobectomy was undergone successfully. Her postoperative course was uneventful. She was discharged from hospital on the postoperative eighth day in good condition.

• Tuberculosis and Respiratory Diseases
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• 제55권1호
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• pp.107-112
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• 2003

A congenital cystic adenoid malformation of the lung(CCAM) is characterized by an anomalous fetal development of the terminal respiratory structures, resulting in the adenomatoid proliferation of the bronchiolar elements and cystic formation. CCAM has been detected on the fetus, premature babies and stillborn as well as infants and children. An adult presentation of CCAM is extremely rare. When cystic lesions occur with a repeated infection, an evaluation of the cystic lesions requires a differential diagnosis of CCAM, sequestration, a lung abscess, a pneumatocele and a bronchogenic cyst. The definite treatment of CCAM is the surgical removal of the involved lobe. We report a case of a CCAM in a 24-year-old female with a brief review of the relevant literature.

• Clinical and Experimental Pediatrics
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• 제49권7호
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• pp.796-799
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• 2006

편측성 다낭성 신 이형성증과 폐의 선천성 낭성 선종양 기형은 각각 독립된 질환으로 배아 시기에 발생 과정의 문제로 생긴다고 알려져 있다. 폐의 선천성 낭성 선종양 기형에서 폐 외의 기형으로 신장 기형이 동반될 수 있으나 생존 가능성이 적어 사산아로 출생되는 경우가 많다. 저자들은 위장관염으로 입원한 21개월 남아에서 우연히 편측성 다낭성 신 이형성증을 발견했고, 이 과정에서 폐의 선천성 낭성 선종양 기형도 발견하였기에 보고하는 바이다.

• 한국동물생명공학회지
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• 제34권3호
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• pp.253-258
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• 2019

A female English bulldog was gave birth two neonates by cesarean section on the sixty one days after mating, but both neonates were died soon after birth. The bodies of neonates were diagnosed using radiography, ultrasonography, computed tomography and necropsy immediately after death. Both neonates had caudal regression syndrome, butterfly vertebra, hydrocephalus, umbilical hernia, cleft palate and bow-legged hind-limb. At necropsy, neonates had mild fetal anasarca, cleft lip and the skull was remained non-union. At thoracic cavity, only three ribs and thoracic spines were existed and patent ductus arteriosus was found. At abnormal cavity, the renal ectopia was found with abnormal morphology. In the present case, those English bulldog neonates with multiple congenital malformation syndromes seriously suffered vertebral column anomalies and that may induced by neural tube defects in during embryonic period. To prevent congenital malformation occurring in English bulldog, further in depth studies are needed for the breed specific genetic diversity and for the reason of behind genetic abnormality in these breed.

• Journal of Chest Surgery
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• 제30권2호
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• pp.231-235
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• 1997

선천성 낭종성 선종양기 형은 말초 기관지가 심하게 분화하여 선종(adenomatoid)의 형태를 취하는 질환으로 발생 빈도는 매우 낮으나 신생아에서 급성 호흡부전을 일으키는 선천성 폐기 형중 가장 빈도가 높다. 대부분적 선천성 낭종성 선종양기 형은 단일 폐염을 침범하며 다른 선천성 질환을 동반하는 경우는 드물다. 저자들은 빈번한 호흡기 감염을 주소로 내원하여 치료를 받던중 누두흉을 동반한 선천성 낭종성 선종양기 형으로 진단된 3세의 여아를 양폐엽절제술과 변형 흉골거상술을 동시에 시행하여 양호한 성적을 얻었기에 문헌고찰과 더불어 보고하고저 한다.

• Journal of Chest Surgery
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• 제39권2호
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• pp.171-175
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• 2006

최소 침습성 흉부수술은 최근에 가장 발전되어온 중요한 수술의 한 분야이다. 선천성 낭종성 선종양 기형은 비교적 드물게 발생하는 폐질환으로 누두흉, 선천성 심장 및 폐혈관 질환 같은 여러 가지 선천성 기형 등이 동반될 수 있다. 저자들은 우하엽 폐에 선천성 낭종성 선종양 기형이 있으며 누두흉이 동반된 5세 된 남아 환자를 최소 침습적인 방법으로 치료 경험하였다. 저자들은 흉강경을 이용하여 우하엽 폐절제술을 실시하였고, 동시에 누두흉에 대해서는 흉골 하 금속막대를 이용한 너스 술식으로 교정하였다. 이와 같이 이러한 질환에 대한 최소 침습적인 수술방법은 유용하고 미용적으로 우수하다.

• Journal of Chest Surgery
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• 제26권8호
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• pp.650-653
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• 1993

Congenital cystic adenomatoid malformation [ C.C.A.M.] is rare, but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced an infant with C.C.A.M. Type II. The patient was a 7-month-old male, and had been suffering from coughing and fever since 3 months ago. The symptom were not improved with medical treatment and then, exploratory thoracotomy was performed under the impression of infected C.C.A.M. or other congenital cystic disease.The left lower lobectomy was performed, and the postoperative course was uneventful.

• Journal of Chest Surgery
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• 제14권1호
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• pp.87-90
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• 1981

Genetic and environmental factors are the two areas which have received attention in the etiology of congenital cardiac malformation. Genetic factor in many types of congenital heart disease have not been clearly delineated. Congenital heart diseases are a heterogenous category of developmental anomalies, representing in most cases the multifactorial inheritance of threshold characters, the expression of which is the product of a genetic - environmental interaction. Recently we experienced three pairs of congenital heart disease in siblings including ventricular septal defects in twin.

• Tuberculosis and Respiratory Diseases
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• 제74권1호
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• pp.28-31
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• 2013

We present a case of congenital cystic adenomatoid malformation (CCAM) in a 25-year-old male who was presented with chronic cough. Chest radiography revealed an abnormal mass-like shadow in the right lower pulmonary zone. A contrast enhanced computed tomography showed an 11 cm solid, cystic mixed mass on the right lower lobe. A right lower lobectomy was performed by video-assisted thoracoscopic surgery without complications. The gross specimen showed a massive cavitation with multiloculated cysts of varying size, consistent with CCAM, along with noticeable granulomatous inflammation. Non-tuberculosis mycobacteria were isolated from a bronchial wash specimen, and the resected tissue homogenates were positive for Mycobacterium avium-intracellulare complex by polymerase chain reaction.