• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.3
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• pp.372-380
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• 2004

Cleidocranial dysplasia(CCD) is a congenital disorder of skeletal and dental abnormality, which is mesodermal dysfunction influencing many tissues and organs. Skeletal abnormalities in CCD are delayed closure of cranial suture and fontanelle, presence of wormian bone and clavicle aplasia. And CCD has an effect on the long bones, phalanges, spine, pelvis, muscles, and central nervous system. Dental manifestations include retention of deciduous teeth, multiple supernumerary teeth, delayed or absent eruption of permanent teeth and formation of cysts around nonerupted teeth. But as a result in common with a lack of medical and physical disability patient may have no substantive complaint, there are many masticatory and psychological problem by absent eruption of permanent teeth after exfoliation of deciduous teeth. For this reason CCD is necessary fo early diagnosis and must be improvement of the patient's appearance as well as provision of a functioning masticatory mechanism by treatment of surgical removal of supernumerary teeth, followed orthodontically eruption of the natural permanent teeth at adequate time.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.3
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• pp.456-463
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• 2009

Cleidocranial dysplasia(CCD) is a congenital disorder of skeletal and dental abnormality, which is a mesodermal dysfunction influencing many tisssues and organs. Skeletal abnormalities in CCD are delayed closure of cranial suture and fontanelle, presence of wormian bone and clavicle aplasia. CCD also has an effect on long bones, phalanges, spine, pelvis, muscles, and central nervous system. Dental manifestations include retention of deciduous teeth, multiple supernumerary teeth, delayed eruption or impaction of permanent teeth and formation of cysts around nonerupted teeth. However, due to lack of any substansive medical or physical disability, diagnosis is often late, thereby causing masticatory and psychological problems caused by delayed eruption of permanent teeth after exfoliation of deciduous teeth. For this reason, CCD requires early diagnosis, and the patient's appearance must be improved. Also, provision for a functional masticatiory mechanism by treatment of surgical removal of supernumerary teeth followed by orthodontic eruption of the natural permanent teeth at an adequate time is necessary.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.703-708
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• 2005

Cleidocranial Dysplasia(CCD) is a congenital disorder of skeletal and dental anomaly with an autosomal dominant mode of inheritance. CCD Shows a generalized defect in intramembranous bones, such as the skull, clavicles, and endochondral bones, such as the long bones and the remainder of the skeleton. The specific clinical feature of CCD is an aplasia & hypoplasia of one or both clavicles, frontal & parietal bone bossing, incomplete fontanels and sutures closure of cranial bone. Generally, relative mandibular prognathism is seen, because maxillar is underdeveloped. Dental anomalies of CCD are prolonged primary teeth, delayed eruption of the permanent teeth and multiple supernumerary teeth. Almost patients of CCD can not recognize their dental abnormality until the permanent teeth eruption was begining. So it is difficult to decide the proper timing of the treatment of patients of CCD. Pedodontists should understand the development of the dentition in CCD patient and start the treatment of CCD patient in proper time.

• Tuberculosis and Respiratory Diseases
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• v.56 no.4
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• pp.420-425
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• 2004

Fistula between coronary artery and pulmonary artery is a type of coronary artery anomalies. It can cause atypical chest pain and fatigue, angina pectoris, endocarditis, finally myocardial steal can result in heart failure and myocardial infarction. But only 0.1-0.2% of coronary angiographic studies reveal the communications between coronary artery and other spaces. (heart chamber, pulmonary artery etc.) It is frequently congenital, but acquired types are increasing because chest and heart manipulations such as opertion of tetralogy of Fallot, endomyocardial biopsy, radiation therapy, or penetrating blunt trauma are increasing. There are reports about repair of fistula using thrombogenic tips, coil embolization and surgical intervention. We report a connection between coronary artery and pulmonary artery in 79 years old female. She was 30 pack-years smoker and suffered from dyspnea several years with chronic obstructive pulmonary disease. She presented with atypical chest pain and palpitation after admission. Electrocardiography showed ST-T wave abnormality. Emergency coronary angiography and chest CT scan revealed coronary-pulmonary artery fistula. Transcatheter embolization was performed and she was relieved from discomforts.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.14 no.1
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• pp.123-127
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• 2003

Cornelia de Lange syndrome is a dysmorphogenic disorder characterized by multiple congenital abnormalities, mental retardation, growth retardation and neurodevelopmental abnormalities. Diagnosis for the Cornelia de Lange syndrome is dependent on the clinical observation because neither definite biological marker nor definite chromosomal abnormality have been investigated. Clinical observation is important for the diagnosis, so we report a case of Corenelia de Lange syndrome with mental retardation and autistic disorder. The patient is a 6-year old girl. Her motor development and language development have been delayed. She could say no meaningful word and understood simple command partially. She showed poor eye contact and poor emotional interaction. Social interaction was impaired and she Showed stereotypic behaviors. Thus we diagnosed her as mental retardation with autistic disorder. She had vesicoureteral reflux, frequent upper respiratory infection and pneumonia. She had experienced febrile convulsions 4 times. She had short stature, confluent eyebrows, long eyelashes, and upturned nose with anteverted nostrils. She also showed low hairline and hypertrichosis in body and extremities. Her finger was short. In this case, we diagnosed Cornelia de Lange syndrome by her characteristic face, hypertrichosis and medical and behavioral problems that were frequently showed in this syndrome.

• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.490-494
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• 2003

Purpose : We'd like to determine the incidence of congenital heart disease and arrhythmia in elementary school children in Busan, and to provide adequate prevention and treatment. Methods : A total of 23,802(male 12,909, female 10,893) 1st grade elementary school children living in Busan were studied. All children were 7-8 years old. We obtained their medical history by questionnaire and checked elecrocardiography(ECG). Subsequent screening tests including a 2nd ECG, chest X-ray, phonocardiogram and CBC for the students who had abnormal findings at the first screening test. The third screening test was done for students who had cardiac murmurs or abnormal ECG findings in the second screening test by echocardiogram, treadmill test and 24-hour Holter monitoring. Results : Among 23,802 children participants, 605(2.54%) had abnormal ECG findings at the first screening test. Q wave abnormality(0.58%) was observed most frequently, and complete right bundle branch block(RBBB)(0.26%), sinus tachycardia(0.24%), right axis deviation(0.22%) and ventricular premature contraction(VPC)(0.21%) followed in order. Four hundred and twenty four children participated in the second ECG screening test. Two hundred and two children(47.6%) had an abnormality such as sinus tachycardia(18.8%), VPC(17.8%), or complete RBBB(17.3%). After completing the third examination including echocardiogram, we couldn't find the students with ventricular tachycardia (VT) or SVT at the third arrhythmia screening test. Conclusion : A high incidence of arrhythmia was found in the 1st grade elementary school children in Busan despite their healthy appearances, although fatal heart diseases were not noted. Early diagnosis, adequate preventative measures and treatment will prevent and decrease the frequency of emergent situations like syncope and sudden death.

• Journal of Veterinary Clinics
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• v.32 no.5
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• pp.469-472
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• 2015

A 10-year-old, castrated poodle dog presented with a cough for 2 weeks, and the cough initially developed since very young age. On radiographs, pneumothorax was noticed by characteristics of radiolucent area without pulmonary markings along the thoracic wall and diaphragm, retracted lung lobes from the thoracic wall and severely decreased volume of the left cranial lung lobe with disconnected bronchus. Computed tomography (CT) findings identified several pulmonary air-filled cysts and collapsed lung with abnormal shape and non-tapered end of bronchus, bronchioles at the accessory lobe and left cranial lobe. Also, pneumothorax, pneumomediastinum and subcutaneous emphysema were found. Imaging diagnosis was the spontaneous pneumothorax caused by ruptured emphysematous bullae associated with congenital bronchial cartilage abnormality or bronchial tree malformation. On surgery, hypoplasia of the left cranial lobe, right middle lobe, and accessory lobe with a bulla where air was leaking was identified. The accessory lobe was partially resected and bronchial cartilage hypoplasia was confirmed by histopathologic examination.

• Development and Reproduction
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• v.10 no.3
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• pp.185-192
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• 2006

It is known that maternal diabetes induces a premature delivery and a congenital malformation. Although it was suggested that hyperglycemia is a main cause, little has been known. The effects of subtotal pancreactomized animal as a diabetic model on the embryo and the offspring were investigated. 1) Although survival rate of offsprings from the 90% pancreactomized female was lower than that of control, body weight of the survived offsprings was similar to that of control until 6 weeks after birth. When the liver was investigated, abnormal tissue was observed in both female and male offsprings from the pancreactomized mother. The level of major urinary protein(MUP) that is synthesized in the liver and secrets into urine was decreased in both female and male, supporting the liver abnormality. 2) In the pancreactomized female, size of the Langhan's island was increased and the liver also was observed abnormal. However, blood glucose level of the pancreactomized mother was not different from that of control. The low body weight and survival rate of offsprings from the $inhibin-{\alpha}$ overexpressed mice was very similar to those of the offsprings from diabetic mother. Thus when the mother itself was investigated after DNA injection, blood glucose level in the $inhibin-{\alpha}$ overexpressed mice was not different from that of control although size of the Langhan's island was decreased. Taken together, subtotal pancreactomy affected the offspring and embryo and influenced deeply the mother itself. However glucose level of the mother was not changed during investigation. It suggested that the relationship between the diabetic mother and embryo through offspring is very complex.