• IMMUNE NETWORK
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• v.12 no.1
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• pp.8-17
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• 2012

Background: Respiratory syncytial virus (RSV) is a major cause of severe lower respiratory tract diseases in infancy and early childhood. Despite its importance as a pathogen, there is no licensed vaccine against RSV yet. The attachment glycoprotein (G) of RSV is a potentially important target for protective antiviral immune responses. Recombinant baculovirus has been recently emerged as a new vaccine vector, since it has intrinsic immunostimulatory properties and good bio-safety profile. Methods: We have constructed a recombinant baculovirus-based RSV vaccine, Bac-RSV/G, displaying G glycoprotein, and evaluated immunogenicity and protective efficacy by intranasal immunization of BALB/c mice with Bac-RSV/G. Results: Bac-RSV/G efficiently provides protective immunity against RSV challenge. Strong serum IgG and mucosal IgA responses were induced by intranasal immunization with Bac-RSV/G. In addition to humoral immunity, G-specific Th17- as well as Th1-type T-cell responses were detected in the lungs of Bac-RSV/G-immune mice upon RSV challenge. Neither lung eosinophilia nor vaccine-induced weight loss was observed upon Bac-RSV/G immunization and subsequent RSV infection. Conclusion: Our data demonstrate that intranasal administration of baculovirus-based Bac-RSV/G vaccine is efficient for the induction of protection against RSV and represents a promising prophylactic vaccination regimen.

• Journal of Dental Anesthesia and Pain Medicine
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• v.16 no.4
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• pp.309-312
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• 2016

Patients with cleft lip and palate (CLP) must undergo corrective surgeries during infancy and early childhood. Many patients with CLP undergo orthognathic surgery during their childhood for correction of skeletal asymmetries or pharyngoplasty with a pharyngeal flap to improve the quality of speech and velopharyngeal function. During orthognathic surgeries, nasotracheal intubation is performed under general anesthesia. In our case report, the patient had undergone palatoplasty and pharygoplasty previously. During the orthognathic surgery, a flexible fiberoptic bronchoscope-guided nasotracheal tube was inserted through the pharyngeal flap ostium; however, active bleeding occurred in the nasopharynx. Bleeding occurred because the flap was torn. After achieving hemostasis, the surgery was completed successfully. Thus, if a patient may show the potential for velopharyngeal port obstruction, nasotracheal intubation should be performed with utmost care.

• Childhood Kidney Diseases
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• v.19 no.2
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• pp.118-124
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• 2015

Purpose: The symptoms and signs of urinary tract infection (UTI) in early infancy are non-specific. Prompt diagnosis of UTI is important, as untreated UTI results in renal damage. Especially, febrile UTI in young infants coexist with other serious bacterial infections. The purpose this study was to propose modified Rochester criteria to differentiate viral infection from urinary tract infection. Methods: We carried out a retrospective investigation of 168 infants less than three months old with a tympanic temperature $>38^{\circ}C$ who were admitted to Chung-Ang University Hospital between 2011 and 2014. We compared the symptoms, physical examination results, and laboratory data between viral infection and UTI groups. A modified Rochester criterion was composed of statistically significant factors. Results: A total of 76 and 92 infants with UTI and a viral infection, respectively, were included. Statistically significant differences in gender, previous admission history, neutrophil ratio, and urine WBC count were found between the two study groups. Using a cut off value of 3 points, the sensitivity and specificity of the modified Rochester criteria were 71.28% and 78.57%, respectively. Conclusion: The modified Rochester criteria may give an outline for identifying young infants with UTI.

• Journal of Chest Surgery
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• v.24 no.2
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• pp.115-122
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• 1991

From April 1986 to December 1989, 25 infants under the age of 12 months with tetralogy of Fallot were operated on. Age ranged from 3 to 12 months[mean 8.9$\pm$4.9 months] and mean body weight was 7.8$\pm$ 2.6kg. All the patients were deeply cyanotic, 12 of them experienced anoxic spell. Transannular patch was laid down in 19 patients, in 7 of them monocuspid patch was utilized. Postrepair P RV/LV was measured at operation room in 17 patients[mean 0.48$\pm$0. 14]. Hospital mortality was 20Yo. Causes of deaths include right ventricular failure and low cardiac output. The mortality was closely related with patient`s age and body surface area at operation. Also higher mortality was noticed in patients having major associated anomaly or previous palliative operation, preoperative management with propranolol and transannular repair. 18 patients were followed up for 12 to 50 months with a mean follow-up time of 24 months after operation. There were no late deaths and late ventricular arrhythmia or congestive heart failure was not detected as yet. Redo operation was performed in one case because of residual pulmonic stenosis. Considering several advantages of early primary repair, primary repair of symptomatic infants with tetralogy of Fallot should be encouraged despite somewhat high mortality rate as yet and better results could be anticipated along with improvement of myocardial protection method and postoperative care.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.2
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• pp.180-187
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• 2020

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

• Journal of Chest Surgery
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• v.28 no.5
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• pp.491-494
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• 1995

A fibroma arising in the right ventricle outflow tract of a 14 month-old infant was successfully removed. The patient was first seen because of shortness of breath and tachycardia. Pertinent clinical and laboratory findings included a grade II/VI systolic murmur, blood pressure of 120/60 mmHg, slight cardiomegaly on chest X-ray, a mass obstructing the outflow tract of the right ventricle on echocardiography and magnetic resonance imaging. On october 30,1992, under cardiopulmonary bypass, a 4cm x 3cm x 3cm tumor was resected from the right ventricular outflow tract, together with a portion of the ventricular wall. Histologically, it was diagnosis as a fibroma. The patient was sent home on the 6th postoperative day following an uneventful recovery form the operation. Although cardiac fibroma is the second most common cardiac tumor in infancy and childhood, it is usually found in the left ventricle and one arising in the right ventricle is considered rare. Although it is a benign tumor, it could produce a severe cardiac dysfunction and even sudden death, depending on its size and location. With the advance in diagnostic techniques and operative management, there is a renewed interest in the early detection and operative removal of these tumors. The case herein presented is the first such case successfully managed and reported in the Korean literature.

• Clinical and Experimental Pediatrics
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• v.54 no.2
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• pp.51-54
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• 2011

Vitamin D is present in two forms, ergocalciferol (vitamin $D_2$) produced by plants and cholecalciferol (vitamin $D_3$) produced by animal tissues or by the action of ultraviolet light on 7-dehydrocholesterol in human skin. Both forms of vitamin D are biologically inactive pro-hormones that must undergo sequential hydroxylations in the liver and the kidney before they can bind to and activate the vitamin D receptor. The hormonally active form of vitamin D, 1,25-dihydroxyvitamin D3 $[1,25(OH)_2D]$, plays an essential role in calcium and phosphate metabolism, bone growth, and cellular differentiation. Renal synthesis of $1,25(OH)_2D$ from its endogenous precursor, 25-hydroxyvitamin D (25OHD), is the rate-limiting and is catalyzed by the $1{\alpha}$-hydroxylase. Vitamin D dependent rickets type I (VDDR-I), also referred to as vitamin D $1{\alpha}$-hydroxylase deficiency or pseudovitamin D deficiency rickets, is an autosomal recessive disorder characterized clinically by hypotonia, muscle weakness, growth failure, hypocalcemic seizures in early infancy, and radiographic findings of rickets. Characteristic laboratory features are hypocalcemia, increased serum concentrations of parathyroid hormone (PTH), and low or undetectable serum concentrations of $1,25(OH)_2D$ despite normal or increased concentrations of 25OHD. Recent advances have showed in the cloning of the human $1{\alpha}$-hydroxylase and revealed mutations in its gene that cause VDDR-I. This review presents the biology of vitamin D, and $1{\alpha}$-hydroxylase mutations with clinical findings.

• Clinical and Experimental Pediatrics
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• v.54 no.4
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• pp.179-182
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• 2011

Transient neonatal diabetes mellitus (TNDM) is a rare form of diabetes mellitus that presents within the first 6 months of life with remission in infancy or early childhood. TNDM is mainly caused by anomalies in the imprinted region on chromosome 6q24; however, recently, mutations in the ABCC8 gene, which encodes sulfonylurea receptor 1 (SUR1), have also been implicated in TNDM. Herein, we present the case of a male child with TNDM whose mutational analysis revealed a heterozygous c.3547C>T substitution in the ABCC8 gene, leading to an Arg1183Trp mutation in the SUR1 protein. The parents were clinically unaffected and did not show a mutation in the ABCC8 gene. This is the first case of a de novo ABCC8 gene mutation in a Korean patient with TNDM. The patient was initially treated with insulin and successfully switched to sulfonylurea therapy at 14 months of age. Remission of diabetes had occurred at the age of 16 months. Currently, the patient is 21 months old and is euglycemic without any insulin or oral hypoglycemic agents. His growth and physical development are normal, and there are no delays in achieving neurological and developmental milestones.

• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1019-1025
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• 2006

Motor delay, when present, is usually the first concern brought by the parents of children with developmental delay. Cerebral palsy that is the most common motor delay, is a nonspecific, descriptive term pertaining to disordered motor function that is evident in early infancy and is characterized by changes in muscle tone, muscle weakness, involuntary movements, ataxia, or a combination of these abnormalities. A wide range of causative disorders and risk factors have been identified for cerebral palsy, and broadly classified into 5 groups; perinatal brain injury, brain injury related to prematurity, developmental abnormalities, prenatal risk factors, and postnatal brain injury. Delay in attaining developmental milestones is the most distinctive presenting complaint in children with cerebral palsy. A detailed history and thorough physical and neurologic examinations are crucial in the diagnostic process. The clinician should be cautious about diagnostic pronouncement unless the findings are unequivocal. Several serial examinations and history review are necessary. All children with cerebral palsy should undergo a neuroimaging study, preferably MRI, because an abnormality is documented on head MRI(89%) and CT(77%). The high incidence rates for mental retardation, epilepsy, ophthalmologic defects, speech and language disorders and hearing impairment make it imperative that all children with cerebral palsy be screened for mental retardation, ophthalmologic and hearing impairments, and speech and language disorders; nutrition, growth, and swallowing also should be closely monitored.

• Clinical and Experimental Pediatrics
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• v.59 no.1
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• pp.1-7
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• 2016

Infants born small for gestational age (SGA) are at increased risk of perinatal morbidity, persistent short stature, and metabolic alterations in later life. Recent studies have focused on the association between birth weight (BW) and later body composition. Some reports suggest that fetal nutrition, as reflected by BW, may have an inverse programing effect on abdominal adiposity later in life. This inverse association between BW and abdominal adiposity in adults may contribute to insulin resistance. Rapid weight gain during infancy in SGA children seemed to be associated with increased fat mass rather than lean mass. Early catch-up growth after SGA birth rather than SGA itself has been noted as a cardiovascular risk factor in later life. Children who are born SGA also have a predisposition to accumulation of fat mass, particularly intra-abdominal fat. It is not yet clear whether this predisposition is due to low BW itself, rapid postnatal catch-up growth, or a combination of both. In this report, we review the published literature on central fat accumulation and metabolic consequences of being SGA, as well as the currently popular research area of SGA, including growth aspects.