• Clinical and Experimental Pediatrics
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• v.61 no.1
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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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• v.13 no.3
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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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• v.55 no.1
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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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• v.49 no.7
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• pp.796-799
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• 2006

Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was admitted with the impression of acute infectious gastroenteritis; multicystic dysplastic kidney with congenital cystic adenomatoid malformation of the lung was detected incidentally during evaluation.

• Journal of Animal Reproduction and Biotechnology
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• v.34 no.3
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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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• v.30 no.2
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• pp.231-235
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• 1997

Congenital cystic adenomatoid malformation of the lung is a rare pulmonary malformation, Although it is one of the most common congenital anomalies which cause acute respiratory distress in the newborn infants, characterized by marked proliferation of terminal respiratory structures. We have experienced an unusual case of congenital cystic adenomatoid malformation associated with pectus excavatum. The patient was 3-year-old female who suffered from cough and high fever for 20 days, and antibiotic therapy was given in other hospital before transfer to our hospital. The findings on chest X-ray, chest CT, aortogram, and selective bronchial arteriogram showed cystic lesions in the right upper and middle lobe accompanied but severe pectus excavatum. Right bilobectomy for pulmonary lesion and costosternal elevation for pectus excavatum was performed simultaneously with successful result. The postoperative course was uneventful and the patient was discharged on the twentieth postoperative day.

• Journal of Chest Surgery
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• v.39 no.2 s.259
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• pp.171-175
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• 2006

Minimally invasive thoracic surgery has been one of the most important surgical advances recently. Congenital cystic adenomatoid malformation of the lung is a relatively rare anomaly and is clearly associated with various congenital anomalies such as pectus excavatum, cardiac and pulmonary vascular lesions. We have experienced a case that was treated with minimal invasive methods for congenital cystic adenomatoid malformation involving in the right lower lobe and pectus excavatum in a 5-year-old boy. We simultaneously performed thoracoscopic right lower lobectomy and Nuss procedure of pectus excavatum using a substernal steel bar. Therefore, a minimally invasive surgical treatment for this diseases is feasible and cosmetically excellent.

• Journal of Chest Surgery
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• v.26 no.8
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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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• v.14 no.1
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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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• v.74 no.1
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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.