• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.219-223
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• 2011

McKusick-Kaufman syndrome (MKS) is an autosomal recessive multiple malformation syndrome characterized by hydrometrocolpos (HMC) and postaxial polydactyly (PAP). We report a case of a female child with MKS who was transferred to the neonatal intensive care unit of Seoul National University Children's Hospital on her 15th day of life for further evaluation and management of an abdominal cystic mass. She underwent abdominal sonography, magnetic resonance imaging, genitography and cystoscopy which confirmed HMC with a transverse vaginal septum. X-rays of the hand and foot showed bony fusion of the left third and fourth metacarpal bones, right fourth dysplastic metacarpal bone and phalanx, right PAP and hypoplastic left foot with left fourth and fifth dysplastic metatarsal bones. In addition, she had soft palate cleft, mild hydronephroses of both kidneys, hypoplastic right kidney with ectopic location and mild rotation, uterine didelphys with transverse vaginal septum and low-type imperforated anus. She was temporarily treated with ultrasound-guided transurethral aspiration of the HMC. Our patient with HMC and PAP was diagnosed with MKS because she has two typical abnormality of MKS and she has no definite complications of retinal disease, learning disability, obesity and renal failure that develop in Bardet-Biedl syndrome, but not in MKS until 33 months of age. Here, we describe a case of a Korean patient with MKS.

• Journal of the Korean Society for Precision Engineering
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• v.24 no.10
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• pp.123-130
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• 2007

Hypoplastic left heart syndrome is currently the most lethal cardiac malformation of the newborn infant. Survival following a Norwood operation depends on the balance between systemic and pulmonary blood flow, which is highly dependent on the fluid dynamics through the interposition shunt between the two circulations. The purpose of this study is an optimization of the systemic-to-pulmonary artery shunt. In this study, We used computational fluid dynamic(CFD) models to determine the velocity profile in a systemic-to-pulmonary artery shunt and suggested a simplified method of calculating the blood flow in the shunt based on Ultrasound systems. We analyzed the flow characteristic variations and oscillatory shear index(OSI) due to the anastomosis angle and shunt diameter changing. Four different CFD models were constructed with the shunt sizes ranging from 3 to 3.5mm. The angle between the brachiocephalic trunk(BCT) and the shunt were $30^{\circ}$ and $45^{\circ}$, respectively. When the diameter is 3.0 mm, the oscillatory shear index decreased by 1.2% at $30^{\circ}$ as opposed to at $45^{\circ}$. When the diameter is 3.5 mm, it increased by 18% more at $30^{\circ}$ as opposed to at $45^{\circ}$. When the joint angle is $30^{\circ}$ and the diameter is 3.0 mm, the oscillatory shear index decreased by 4.1% in comparison with the 3.5 mm diameter. When the angle is $45^{\circ}$ and the diameter is 3.0 mm, the index increased by 14.6% in comparison with the 3.5 mm diameter.

• Archives of Plastic Surgery
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• v.35 no.6
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• pp.739-742
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• 2008

Purpose: Breast augmentation is one of the common procedures in plastic surgery today. The shape, size and insertion plane of the implant is decided preoperatively by physical examination of the breast. Pectus excavatum is one of the most common anomalies of chest wall, characterized by a depression of the anterior chest wall. For patients with a mild type of pectus excavatum, the main purpose of the treatment is aesthetic rather than functional improvement. Two most surgical treatment options for skeletal deformity are the Ravitch technique and minimally invasive Nuss repair. Other options for soft tissue repair are implant insertion and autologous soft tissue augmentation. We performed a surgical operation with Nuss procedures and breast augmentation for a patient with mild pectus excavatum and hypoplastic breast. Methods: A 32 year-old female was presented with hypoplastic breast. Preoperative chest CT was performed, showing pectus excavatum. After Nuss procedure, we inserted saline implant(275 cc textured round breast implant, moderate profile) submuscularly to restore adequately projected breast. Results: Patient's postoperative course was uneventful without any complication. After 6 months of follow-up period, the patient had an excellent result, with high patient satisfaction and no complications. Conclusion: For patients with a mild type of pectus excavatum, who do not have cardiopulmonary symptoms and requires for aesthetic improvement, this simple approach with Nuss procedure and breast augmentation achieves excellent aesthetic correction with low complication rate and high patient satisfaction.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.547-551
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• 2001

The effects of deep hypothermia and circulatory arrest during aortic arch reconstruction are associated with potential neurologic and myocardial injury. We describe a surgical technique that two patients underwent a modified Norwood procedure without circulatory arrest and myocardial ischemia. One was 13-day-old female patient, weighing 3.1kg, having a variant of hypoplastic left heart syndrome and another was 38-day-old male patient, weighing 3.4 kg, diagnosed Taussig-Bing anomaly with severe aortic arch hypoplasia, coarctation of the aorta, and subaortic stenosis. The arterial cannula was inserted in innominate artery directly. During Norwood reconstruction, regional high-flow perfusion into the inominate artery and coronary perfusion were maintained and there were no neurologic, cardiac, and renal complications in two patients. This technique may help protect the brain and myocardium from ischemic injury in patients with hypoplastic left heart syndrome or other arch anomalies including coarctation or interruption.

• Journal of the korean academy of Pediatric Dentistry
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• v.41 no.4
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• pp.322-327
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• 2014

Regional odontodysplasia (RO) is a rare and nonhereditary dental malformation. It is a dental alteration of unknown etiology, involving both mesodermal and ectodermal dental components, which is characterized by clinical, radiographic, and histologic features. The maxilla is more often involved than the mandible (especially the left side), and there is no racial predilection, but females are affected twice as often as males. The affected teeth are clinically hypoplastic and hypocalcified, presenting a "ghost-like" appearance radiographically. The present case features a male patient aged 4 years and 6 months who was diagnosed with regional odontodysplasia in the maxilla on the right side, confirmed by clinical and radiographic examination, with a follow up of 5 years. Since teeth affected by RO have a poor prognosis due to the fragile tooth surface and open apices, the long-term treatment strategy depends on periodic clinical and radiological observations.

• Clinical and Experimental Pediatrics
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• v.58 no.3
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• pp.108-111
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• 2015

DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.

• Korean Journal of Animal Reproduction
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• v.23 no.1
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• pp.69-73
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• 1999

A calf with derodidymus was delivered from a 3-year old Korean-native cow with abortion last ime at Jangheong area in Chonnam Province. The anomaly with body weight of 35 kg was born t the normal time of parturition. During the parturition, however, the calf with twin head was onfirmed and the complete amputation between fore and hind limb was inevitably carried out or safety of the dam. The calf had normal skeleton and extremities but had two heads and cervical vertebrae divided from upper thoracic part. At necropsy, there were found totally 3 pairs of ore limbs including two pairs of hypoplastic ones hided in the thoracic limb and also found one air of sternum. One head had incomplete torsion or unilateral hypoplasia of mandible with artly hypoplastic skull. There were marked fusion and torsion from cervical to 3rd thoracic vertebra. No abnormality was found on all organs in the pleural and abdominal cavities except a rectal stricture formed at 5 cm away from the atresia ani.

• Journal of the Korea Concrete Institute
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• v.15 no.3
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• pp.433-442
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• 2003

This paper presents a hypoplastic model for three-dimensional analysis of concrete structures under monotonic, cyclic, proportional and non-proportional loading. The constitutive model is based on the concept of equivalent uniaxial strains that allows the assumed orthotropic model to be described via three equivalent uniaxial stress-strain curves. The characteristics of these curves are obtained from the ultimate strength surface in the principal stress space based on the Willam-Warnke curve. A cap model is added to consider loading along or near the hydrostatic axis. The equivalent uniaxial curve is based on the Popovics and Saenz models. The post-peak behavior is adjusted to account for the effects of confinement and to describe the change in response from brittle to ductile as the lateral confinement increases. Correlation studies with available experimental tests are presented to demonstrate the model performance. Tests with monotonic loading on specimens under constant lateral confinement are considered first, followed by biaxial and triaxial tests with cyclic loads. The triaxial test example considers non-proportional loading.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.3
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• pp.598-603
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• 1998

Amelogenesis imperfecta is a group of hereditary defects of enamel unassociated with any other generalized defects. The prevalence of this condition has been estimated to range from 1 in 14,000 to 1 in 16,000, depending on the population studied. It may be differentiated into three general types : hypoplastic, hypocalcified, and hypomaturation, depending on the clinical presentation of the defects and the likely stage of enamel formation that is primarily affected. The dentin and root form are usually normal, but the enamel may lack the normal prismatic structure, being laminated throughout its thickness or at the periphery, with the result that these teeth are more resistant to decay. This case is that of an six-year-old girl brought to the pediatric dentistry department by her parents for esthetic reasons and also because of slight dental sensitivity. Clinical and radiographic examinations confirmed amelogenesis imperfecta. The author has treated with the crowning of the primary molars, using prefomed NiCr crowns and periodic fluoride application on whole dentition.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.4
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• pp.499-504
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• 2000

Amelogenesis imperfecta is defined as a genetically determined effect affecting enamel formation and may be associated with other ectodermal or systemic disorders. It is entirely an ectodermal disturbance, since the mesodermal components of the teeth are basically normal. The presentation of diverse clinical manifestations in 1:14,000 to 1:16,000. Classification of the AI types considers mode of inheritance and clinical manifestations. The most widely accepted classification system recognize three major groups; i.e., hypoplastic(thin enamel), hypocalcified(primary mineralization defect), hypomaturation(defect in enamel maturation). The treatment is that at first, genetic counselling must be practiced, and in anterior teeth, composite resin veneer or jacket crown for esthetics, and in posterior teeth, stainlees steel crown or gold onlay.