• Maxillofacial Plastic and Reconstructive Surgery
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• v.18 no.1
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• pp.61-68
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• 1996

In Fibrous dysplasia(FD) of the jaws, the majority of cases can await the cessation of growth before surgical intervention, and it seems prudent to delay surgery whenever possible until growth has ceased. In craniofacial FD, however, the dangers of dystopia, dystopia and loss of vision may require early surgery to prevent or control cranio-orbital complications. Delaying surgery in those circumstances may be significantly detrimental to such patients. Conservative surgical management of FD is widely practised and we advocate an extension to this conservative treatment by combining surgical recontouring with appropriate osteotomies if indicated, to achieve an optimal esthetic and functional results in craniofacial FD. One case will be presented to illustrate the feasiblility of such combined treatment, to report the uneventful healing of osteotomies in the FD of the jaws, and to demonstrate the use of titanium miniplate fixation in dysplastic bone. The other case had expansile disease of the left facial and fronto-temporal bones and osteolytic change left mandible. This patient complained of severe spontaneous bleeding of left mandibular premolar area and it was suspected as central hemangioma of the left mandible and craniofacial FD. Angiogram disclosed generalized dilation of the external carotid artery and its branches, especially terminal branches of the left facial and inferior alveolar arteries. But no specific abnormalities, such as A-V shunt, venous lake, or early venous drainage, was seen. So it was diagnosed craniofacial FD with hypercellularity and generalized bony recontouring was performed via coronal and transoral approaches.

• Journal of Genetic Medicine
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• v.8 no.2
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• pp.125-129
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• 2011

Stickler syndrome is a very rare connective tissue disorder. The authors of the present study describe an 11-month-old girl with high myopia, retinal abnormalities, flat nose, cleft palate, retrognathia, micrognathia, short stature and arthrogryposis. Radiological evaluation also showed irregularity of the epiphysis of the femur and tibia and spondyloepiphyseal dysplasia. Genetic analysis using a peripheral blood sample revealed a novel variant c.787G>A (p.Gly246Asp) mutation of the COL2A1 gene. This is the first Korean case with Stickler syndrome confirmed by genetic testing.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.711-715
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• 2005

Purpose : With the recent improved survival of extremely low birth weight infants(ELBWI), enteral feeding has become a major issue. This study investigates the effects of early enteral feeding in ELBWI on their morbidity, duration of hospitalization, and mortality. Methods : ELBWI admitted to the neonatal intensive care unit at Samsung Medical Center from November 1994 to April 2004 who survived more than 14 days were enrolled. ELBWI were divided into two groups : an early feeding group(EF), in which enteral feeding was started within 3 days after birth; and a late feeding group(LF), in which enteral feeding was started beyond 3 days after birth. 80 ELBWI came under EF, and 131 ELBWI under LF. Results : Birth weight and gestational age did not differ between the two groups. In EF, the time to achieve full enteral feeding and the duration of parenteral nutrition were significantly shorter than in LF. The incidence of bronchopulmonary dysplasia was significantly lower in EF, but the incidences of sepsis, necrotizing enterocolitis, and cholestasis were not different between the two groups. There was no difference in the survival rate between the two groups, but the duration of hospitalization was significantly shorter in EF. Conclusion : Early enteral feeding in ELBWI did not increase the incidence of necrotizing enterocolitis and sepsis, but rather decreased the incidence of bronchopulmonary dysplasia and shortened the duration of hospitalization.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.882-890
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• 2007

Purpose : Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental sequale in perterm VLBW. Methods : Subjects were 26 preterm infants whose birth weight are less than 1,500 g and delivered under the intrauterine period 34 weeks. Autologous umbilical mononuclear cells (about $5.87{\times}10^7/kg$) were injected to neonate via the umbilical vein on the postnatal 24-48 hour. The therapeutic efficacy was assessed by numbers of nucleated RBC, urinary uric acid/creatinine ratio, concentration of neuron specific enolase (NSE), interleukin 6 (IL6), interleukin-$1{\beta}$ ($IL-1{\beta}$), and glial cell derived neurotrophic factor (GDNF) in serum and cerebrospinal fluid on day 1 and 7. Results : There were no significant differences in the numbers of the nucleated RBC, urinary uric acid/creatinine ratio, concentration of creatine kinase between the transplanted infants and controls. But the nucleated RBC is more likely to be rapidly discharged in the transplanted group. In the transplanted group, the concentrations of IL6, $IL-1{\beta}$, and GDNF were no significant difference between day 1 and 7, although GDNF seemed to be elevated. Serum NSE concentration was significantly elevated after transplantation, but not in CSF. Conclusion : It is suggested that autologous umbilical cord blood transplantation in preterm very low birth weight infant is safe to apply clinical practice. Long term follow up study should be needed to evaluate the potential therapeutic effect of umbilical cord blood transplantation for neuroprotection.

• Neonatal Medicine
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• v.18 no.1
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• pp.42-48
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• 2011

Purpose: Several factors including prolonged inflammatory response are thought to contribute to the pathogenesis of bronchopulmonary dysplasia (BPD). The clinical findings can be explained by an increased production of proinflammatory cytokines such as tumor necrosis factor alpha (TNF-$\alpha$ ). We investigated the relationship between susceptibility to BPD and TNF-$\alpha$ promoter polymorphisms to identify genetic factors of the disease. Methods: Thirty-eight preterm infants who had developed BPD and 55 controlled infants with a birth weight <1,500 g were analyzed for TNF-$\alpha$ genotypes. The alleles of five promoter sites (-1031/-863/-857/-308/-238) of TNF-$\alpha$ gene were determined using $Taqman^{(R)}$-based allelic discrimination assays. Results: Gestational age ($27^{+5}{\pm}2^{+0}$ wk vs. $29^{+2}{\pm}1^{+4}$ wk, P<0.0001) and birth weight (990${\pm}$270 g vs. 1,220${\pm}$230 g, P<0.0001) were lower in the BPD group compared to the control group. The incidence of respiratory distress syndrome (71.1% vs. 49.1%, P=0.035) and patent ductus arteriosus (71.1% vs. 50.9%, P=0.052) was higher in the BPD group compared to the control group. The frequencies of the alleles and genotypes of five promoter sites (-1031/-863/-857/-308/-238) of TNF-$\alpha$ gene did not show differences between the BPD group and the control group. Conclusion: TNF-$\alpha$ promoter polymorphisms are not associated with susceptibility to BPD in Korean preterm infants.

• Pediatric Infection and Vaccine
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• v.18 no.2
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• pp.154-162
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• 2011

Purpose : The aim of this study was to identify the clinical characteristics of lower respiratory tract infection due to respiratory syncytial virus (RSV) in young children and to provide information for an effective guideline for palivizumab administration in Korea. Methods : We reviewed medical charts of 167 patients under 3 years of age who were hospitalized in Dongguk University Ilsan Hospital for lower respiratory tract infection between January 2007 and February 2011. Diagnosis of the virus was made based on the multiplex real time polymerase chain reaction. Results : There were 113 patients who were infected by respiratory syncytial virus. 90 patients were term infants and 23 patients were preterm infants. No difference was shown between term and preterm infants except the days of admission which was 9.0${\pm}$6.0 days and 12.6${\pm}$21.0 days respectively. In the preterm group their mean age at the time of admission was 5.21${\pm}$4.9 months and the mean gestational age was 33.1${\pm}$4.3 weeks, and the mean birth weight was 2,152${\pm}$950 g. Only 4 patients were born under 28 weeks gestational age and were candidates for palivizumab administration. Conclusion : Most of the patients with severe RSV lower respiratory tract infection were term or near term infants who were not candidates for palivizumab prophylaxis. A nationwide study is needed to make a new risk stratified guideline for RSV prophylaxis for our country.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1067-1071
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• 2007

Purpose : The aim of this study was to determine whether improved survival of extremely low birth weight infants (ELBWI) was associated with decreased neurodevelopmental disability later in life, and also to identify the factors influencing this disability. Methods : ELBWI admitted to the neonatal intensive care unit of Samsung Medical Center, survived, and followed up until the corrected age of 18 months were enrolled. They were divided into two groups according to admission time: period I (1994-1999, n=36) and period II (2000-2004, n=98). Clinical data were collected retrospectively from the medical records. Results : Survival rates increased from 60.0% to 74.7%, cerebral palsy rates decreased from 22.2% to 8.2% and catch-up growth rate increased from 25.0% to 51.0% during period I and II. Despite less gestational age and birth weight, ELBWI during period II had less periventricular leukomalacia (PVL), sepsis and bronchopulmonary dysplasia compared to period I. The highest risk factors for cerebral palsy were intraventricular hemorrhage (IVH) (${\geq}$Grade III), failure of catch-up growth and PVL. Conclusion : In summary, improved viability was associated with decreased neurodevelopmental disability in ELBWI. Improved neonatal care with resultant decrease in PVL and IVH, and better nutritional support seem to be primarily responsible for this improved outcome.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.2
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• pp.270-274
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• 2009

Dentigerous cysts generally encompass the crown of an unerupted tooth. These cysts are usually solitary. They are the second most common odontogenic type of cysts following radicular cysts, and are frequently associated with impacted mandibular third molars or maxillary canines. Most multiple cysts found in the jaw are odontogenic keratocysts associated with the nevoid basal cell carcinoma syndrome, mucopolysaccharidoses and cleidocranial dysplasia. Although a single dentigerous cyst is well documented in the medical literature, including the prevalence, treatment and prognosis, multiple dentigerous cysts without any systemic symptoms is unusual. Furthermore, cases involving both the maxilla and mandible are especially rare. We present the case of an 11-year-old boy with nonsyndromic multiple dentigerous cysts associated with a mandibular second premolar and a maxillary canine. The treatment was conservative and included marsupialization and eruption guidance. Further follow up is planned to rule out additional problems and the possible identification of a syndrome.

• Journal of Chest Surgery
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• v.32 no.8
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• pp.702-708
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• 1999

Background: Surgical closure of the PDA in premature infants with complications or contraindications to indomethacin use, or recurrence of symptomatic PDA is a safe and effective procedure with low operative risk and minimal complications. Material and Method: From April 1996 to August 1998, 11 premature infants with body weight under 1.5 kg at operation underwent operation for a symptomatic PDA (male:5, female: 6). Associated dise ases were congenital heart disease(7), hyaline membrane disease(6), intraventricular hemor rhage(4), pneumonia(4), pneumothorax(3), hyperbilirubinemia(2), necrotizing enterocolitis(2), renal failure(1), epilepsy(1), and hydrocephalus(1). Surgical techniques are hemoclipping(8) and ligation(3). The size of PDA was 3~6 mm (5.0$\pm$1.2). Result: Systolic and diastolic blood pressure rised and heart rates decreased after PDA closure. ABGA improved postoperatively. There were no surgical complications. Six infants with improved ABGA data were weaned from mechanical ventilatory support. The follow-up durations after discharge were 3 month to 12 month. Five deaths were not related to operation. The causes of death were hyaline membrane disease(2), bronchopulmonary dysplasia with pneumonia(1), sepsis(1), and con gestive heart failure with respiratory distress syndrome(1). Conclusion: Early operative closure is the treatment of choice in most premature infants with a hemodynamically significant shunt(PDA), recurrence of symptomatic PDA, complications of Indomethacin, or contraindi cations to Indomethacin.

• Journal of Korean Orthopaedic Sports Medicine
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• v.9 no.2
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• pp.85-90
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• 2010

Purpose: To evaluate the results of percutaneous lateral release and arthroscopic medial plication for patients with recurrent patella dislocation. Materials and Methods: This study includes the thirty-one cases of patients who have had a surgical operation from March 2001 to March 2008. All the patients had recurrent patella dislocation after trauma history. The average age was 24.2 years old and the average follow up was 47.4 months (minimum 24 months). Results: The preoperative congruence angle, lateral patellofemoral angle, Lysholm score was $24.8^{\circ}$, $-6.2^{\circ}$, 75 points on average respectively. However, the postoperative congruence angle, lateral patellofemoral angle, Lysholm score was improved to normal range: $-2.8^{\circ}$, $10.2^{\circ}$, 95 points on average. Five cases showed the recurrent dislocation after operation. Among them, three cases showed femoral trochlear dysplasia. Conclusion: Percutaneous lateral release and arthroscopic medial plication showed satisfactory results for recurrent patella dislocation with low complication rate. However, in cases with femoral trochlear dysplasia, another surgical treatment may be considered.