A Case of Concurrent Non-Intestinal Type Adenocarcinoma and Inverted Papilloma in a Maxillary Sinus / 대한이비인후과학회지

Inverted papilloma is a benign epithelial tumor that arises from the sinonasal epithelium and occurs in 0.5–4% of all sinonasal tumors. Although benign, it is associated with malignant transformation in 2–27% of the cases, with the most commonly accompanying malignant tumor being squamous cell carcinoma. The malignant transformation of inverted papilloma into adenocarcinoma is extremely rare, with two cases reported worldwide to date. Here, along with a literature review, we report a recent case of a 53-year-old man with non-intestinal type adenocarcinoma associated with a sinonasal inverted papilloma. This case shows the possibility of a malignant transformation of inverted papilloma into non-intestinal type adenocarcinoma, which may be associated with human papilloma virus and thus requires further investigation.

A Case of Concurrent Non-Intestinal Type Adenocarcinoma and Inverted Papilloma in a Maxillary Sinus / 대한이비인후과학회지

Inverted papilloma is a benign epithelial tumor that arises from the sinonasal epithelium and occurs in 0.5–4% of all sinonasal tumors. Although benign, it is associated with malignant transformation in 2–27% of the cases, with the most commonly accompanying malignant tumor being squamous cell carcinoma. The malignant transformation of inverted papilloma into adenocarcinoma is extremely rare, with two cases reported worldwide to date. Here, along with a literature review, we report a recent case of a 53-year-old man with non-intestinal type adenocarcinoma associated with a sinonasal inverted papilloma. This case shows the possibility of a malignant transformation of inverted papilloma into non-intestinal type adenocarcinoma, which may be associated with human papilloma virus and thus requires further investigation.

Clinical Characteristics of Acute Pediatric Neck Infection and Predictive Factors of Abscess Formation / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Abscess of neck is a life-threatening disease in children. Detection of abscess is important because it is essential for the determination of surgical drainage. However, clinical diagnosis is difficult, because children are seldom able to verbalize their symptoms or cooperate with physical examination. This study aims to review the clinical characteristics of 157 pediatric patients with neck inflammation and investigate the relative risk factors for abscess. SUBJECTS AND METHOD: Pediatric patients who were admitted to Dongguk University Hospital from January 2005 to July 2014 with acute neck inflammation were reviewed. All 157 pediatric patients were divided into two groups, based on radiologic findings with and without neck abscess. RESULTS: Of 157 patients, 53 children were diagnosed with neck abscess, and peritonsillar abscess was the most common type of neck abscess followed by submandibular abscess, retro·parapharyngeal abscess and posterior triangle & other abscess. The dominant pathogens, staphylococcal infection (≤2 yr) and streptococcal infection (>3 yr), was different for the different age group. The abscess group except for peritonsillar abscess was characterized by younger age, higher heart rate and WBC count, and longer hospital days than those without abscess (p=0.026, 0.026, <0.001, 0.007 respectively). Multivariate analysis revealed younger age (≤24 mo) and higher heart rate were independent predictors for abscess formation (odds ratio: 3.022, 2.923). CONCLUSION: Pediatric patients with high heart rate & younger age are at risk for abscess formation; meticulous care and early imaging work up are required in younger children with deep neck infection, and especially with higher heart rate.

Distribution of Disease Severity and Group A Rotavirus Genotypes (VP7 & VP4) in Children with Acute Rotavirus Gastroenteritis / 대한소아소화기영양학회지

PURPOSE: We aimed to study the distribution of rotavirus genotypes (VP7 and VP4) and disease severity of rotavirus gastroenteritis prevalent in our community. METHODS: Stool samples were collected from 156 children who were hospitalized with rotavirus gastroenteritis from December 2007 to June 2008. The disease severity of all patients was scored using the Vesikari scale. After extraction of ds-RNA of the rotavirus, cDNA synthesis using reverse transcription and polymerase chain reaction (RT-PCR) and multiplex PCR was performed. Following this, the final identification of genotypes was performed. RESULTS: Of the 156 samples, VP7(G) and VP4(P) genotypes were identified in 147 (94.2%) and 140 (89.7%) samples, respectively. G1 (116 of 147 samples; 78.9%) and P[8] (137 of 140 samples; 97.9%) were the most prevalent, respectively. Of the 138 samples identified of combination types of VP7 and VP4, G1P[8] (111 samples; 80.4%) was the most prevalent. Other combination types varied with very low distribution rates. 9.4% of genotypes were not included in the new vaccines. The disease severity score was 11.8+/-3.3 (mean+/-2SD). The distribution of disease severity was mild or moderate in 37.8% and severe in 62.2% of patients. CONCLUSION: The most prevalent genotype combination of rotavirus was G1P[8] and genotypes not included in the vaccines represented 9.4% in our community. Disease severity distribution of hospitalized children with rotavirus gastroenteritis was higher in the severe than in the mild and moderate categories.

Bullous Erythema Multiforme and Stevens-Johnson Syndrome Caused by Mycoplasma pneumoniae Infection / 대한피부과학회지

In adults, bullous erythema multiforme is most often caused by herpes simplex virus infection, while Stevens-Johnson syndrome is most often associated with drug hypersensitivity. Mycoplasma pneumoniae infection is highly known to be a cause of bullous erythema multiforme and Stevens-Johnson syndrome in children and young adults. To our knowledge, there are only a few reports of bullous erythema multiforme or Stevens-Johnson syndrome induced by M. pneumoniae infection in the Korean literature. We encountered two cases of female children diagnosed as having bullous erythema multiforme associated with M. pneumoniae infection. We also describe an unusual case of Stevens-Johnson syndrome associated with M. pneumoniae infection in a 65-year-old woman.

Recent Advances in Echocardiography

Echocardiography is a very useful, safe, and noninvasive method for the diagnosis and management of cardiac diseases. Echocardiographic studies, which use ultrasound, provide anatomic diagnosis, as well as functional information. Over the past twenty years, important advances have occurred in echocardiographic assessment of myocardial function. Tissue Doppler imaging and three dimensional echocardiography can provide potential benefits to analyze hemodynamic information of heart in pediatric patients. This paper reviews some of these major advances in pediatric echocardiography, as well as conventional methods of pediatric echocardiography.

Association of Leukotriene C4 Synthase Gene Polymorphism with Clinical Response to Montelukast in Childhood Asthma / 소아과

PURPOSE: Cysteinyl leukotrienes are important inflammatory mediators in the pathogenesis of asthma; therefore interruption of cysteinyl leukotrienes by leukotriene receptor antagonists improves clinical symptoms in the management of patients with mild to moderate asthma. We evaluated whether clinical response to montelukast, a leukotriene receptor antagonist, in childhood asthma was predicted by genotypes of leukotriene C4 synthase (LTC4S) promoter gene polymorphism. METHODS: An 8-week prospective, open trial of montelukast was carried out in 161 children with mild to moderate asthma. Genotyping of LTC4S gene polymorphism was determined by restriction fragment length polymorphism. RESULTS: The distribution of the LTC4S genotypes AA, AC, and CC was 70.8 percent, 23.6 percent, and 5.6 percent, respectively in asthma group and 74.0 percent, 22.6 percent, and 3.4 percent, respectively in control group. A statistically significant difference in the distribution of LTC4S genotype was not observed between the asthma and the control groups, and there was no significant difference between the LTC4S genotype and asthma severity. The responders to montelukast were significantly prevalent in the mild asthma group (P< 0.05). There was no significant difference in the distribution of the responders compared to non-responders within genotype in the total asthma group or the moderate asthma group. However, the responsiveness for montelukast was significant difference within genotype for both AA and AC/CC in the mild asthma group: The AA genotype was more included in the responder group (P< 0.05). CONCLUSION: In the mild persistent asthma group, the A allele of LTC4S polymorphism may be regarded as a predictable factor for clinical response to montelukast. However, LTC4S polymorphism was not significantly associated with the clinical response to montelukast in asthmatic children.

Polymorphism of Matrix Metalloproteinase-3 Promoter Gene as a Risk Factor for Coronary Artery Lesions in Kawasaki Disease

Kawasaki disease (KD) is a major cause of acquired coronary artery diseases in childhood. The serum levels of matrix metalloproteinase (MMP)-3 and MMP-9 in KD have been reported to be significantly higher than other diseases. Several studies have demonstrated that MMP-3 5A/6A polymorphism and MMP-9 C-1562T polymorphism modify each transcriptional activity in allele specific manner. We hypothesized that these polymorphisms may play a role as a risk factor for development of coronary artery lesions (CAL) in KD. Eighty-three patients, diagnosed with KD in Cheju National University Hospital from January 2000 to February 2004, were divided into two groups according to the presence of CAL. Genotyping of MMP-3 and MMP-9 gene polymorphisms were determined by restriction fragment length polymorphism. With regard to MMP-3 gene polymorphism, the KD with CAL group had a higher frequency of 6A/6A genotype than control group (p=0.0127) and the KD without CAL group (p=0.0036). However, no significant differences in the allele and genotype distributions of the MMP-9 polymorphism were observed. These findings suggest that MMP-3 6A/6A genotype may be an independent risk factor for CAL formation in KD.

Pharmacologic Therapy / 소아과

No abstract available.

Telelaryngoscopic Findings in the Unilateral Vocal Fold Paralysis with Diplophonia / 대한이비인후과학회지

OBJECTIVES: Diplophonia is the voice of two separate tones through quasi-periodic variations in the vocal cord vibration when an imbalance in the tension and the level applied to the vocal cords. The purpose of this study is to investigate the relationship between the occurrence of the diplophonia and the endoscopic findings in the unilateral vocal cord paralysis. MATERIALS AND METHOD: A retrospective review was employed using video recorded images of larynx with unilateral vocal cord paralysis. A total 104 patients selected for this study complained of voice change due to unilaterally paralyzed vocal cord. Video-recordings were obtained using a laryngeal telescope. The paralyzed positions, bowing, shapes of the paralyzed arytenoids and level differences between two vocal folds were evaluated according to whether diplophonia. existed or not. RESULTS: A large number of patients of paramedian paralysis showed diplophonia when the bowing of paralyzed vocal fold was shown. However, diplophonia was shown in a small number of patients with median and intermediate paralysis. Diplophonia also seems to occur when the vertical mismatch was shown. CONCLUSION: Occurene of diplophonia depends largely on the paralyzed position, adequate glottal gap such as paramedian position, with the presence of bowing of paralyzed vocal cord.

A Case of Ileal Atresia with Hypertrophic Pyloric Stenosis

Ileal atresia, a subtype of intestinal atresia, is one of the well-recognized causes of bowel obstruction in newborns. Prenatal diagnosis of intestinal atresia is very important in its management and outcome. Unfortunately, there are few cases of ileal atresia diagnosed prenatally, so more appropriate diagnoses and management plans are needed. As an associated gastrointestinal malformation with ileal atresia, hypertrophic pyloric stenosis is rarely reported. We report one case of postnatally diagnosed ileal atresia associated with hypertrophic pyloric stenosis which was complicated initially by bowel perforation and later by vomiting due to pyloric obstruction. Vomiting in the postoperative period is a common problem. But, if vomiting continues after the operation for ileal atresia, hypertrophic pyloric stenosis should be considered as a possible cause of medically retractable non-bilious vomiting.

Congenital Ocular Motor Apraxia with Cerebellar Vermian Hypoplasia

Congenital ocular motor apraxia is characterized by impaired voluntary saccades and abnormal head thrusts to induce a fixation. We report a case of a 7-year-old boy who shows typical finding of congenital ocular motor apraxia. He had a history of spasmus nutans. His developmental milestones were delayed. Brain MRI demonstrated cerebellar vermian hypoplasia, especially in the inferior portion. We report on a case of congenital ocular motor apraxia associated with cerebellar vermian hypoplasia.

Congenital Ocular Motor Apraxia with Cerebellar Vermian Hypoplasia

Congenital ocular motor apraxia is characterized by impaired voluntary saccades and abnormal head thrusts to induce a fixation. We report a case of a 7-year-old boy who shows typical finding of congenital ocular motor apraxia. He had a history of spasmus nutans. His developmental milestones were delayed. Brain MRI demonstrated cerebellar vermian hypoplasia, especially in the inferior portion. We report on a case of congenital ocular motor apraxia associated with cerebellar vermian hypoplasia.

Identification of Fastidious Mycobacteria other than M. tuberculosis ( MOTT ) by Comparative Sequence Analysis of rpoB and 16S rDNA

Conventional tests for the identification of mycobacteria may frequently result in erroneous identification and underestimate the diversity within the genus Mycobacterium. However, this problem can be overcome by molecular approach like as 16S rRNA gene (rDNA) or RNA polymerase gene (rpoB) sequence analysis. In this study, a molecular approach analyzing partial sequence of 16S rDNA and rpoB gene was applied to mycobacteria other than M tuberculosis (MOTT) isolates that had not been definitely identified by conventional physical and biochemical tests. Among the eighteen isolates included in this study, twelve isolates could be identified to the species level and six were identified to the complex level. Compared with the results by 16S rDNA analysis, the rpoB analysis could di6erentiate some of the strains into the subspecies level.

Atypical pulmonary artery sling with diffuse-type pulmonary arteriovenous fistula

The case of a cyanotic infant with a rare combination of atypical pulmonary artery sling, imperforate anus, absence of the left kidney, interruption of the inferior vena cava, left side hemihypertrophy and diffuse-type pulmonary arteriovenous fistula is described. The clinical features were confusing, because of compounding abnormalities involving the respiratory tract and pulmonary circulation. The diagnostic approach to the etiology of cyanosis is discussed and the embryonic origin of pulmonary artery sling is reviewed.

Follow-up Study after Atrial Switch Operation for Complete Transposition of the Great Arteries

BACKGROUND: Since the introduction of atrial switch operation, the outlook for patients with complete transposition of the great arteries (TGA) has improved dramatically. However, many survivors are afflicted with postoperative complications and continue to demand medical attention. We therefore performed the study for the evaluation of the clinical course in the patients with complete TGA after atrial switch operation. METHODS: We analyzed the data of 51 patients who underwent the atrial switch operation for complete TGA at Seoul National University Hospital between January 1981 and June 1993 retrospectively. RESULTS: The patients were composed of 36 males and 15 females (mean age at operation, 15+/-30 months, range 2 months-18 years). Senning operation was undertaken in 43 and Mustard operation in 8. Among these, 27 had simple and 24 complex complete TGA. The early mortality rate within 1 month postoperatively was 39% and the late mortality rate 16%. The actuarial survival rate after 10 years was 78%. Of these, 17 cases have been followed up so far. Although the significant tricuspid regurgitation was noted in 9, right ventricular systolic function was maintained in all of them. Preoperative sinus node dysfunction was noted in 1 case. Postoperatively, premature ventricular contractions were found in 8 cases, atrial flutter in 4, and sinus node dysfunction in 7. Subclinical pulmonary venous obstruction was found in 2 and systemic venous obstruction in 1. At present, one takes digoxin for prevention of atrial flutter and 7 take digoxin, furosemide, and/or angiotensin-converting enzyme inhibitors for prevention of ventricular functional deterioration. Twelve cases were in New York Heart Association functional class I at the latest follow-up and 5 were in class II. CONCLUSION: This study revealed that morbidity and mortality were not low after atrial switch operation. However, considering the good long-term functional status in the survivors, atrial switch operation could be an alternative to arterial switch operation in complicated cases of TGA.

Follow-up Study after Atrial Switch Operation for Complete Transposition of the Great Arteries

BACKGROUND: Since the introduction of atrial switch operation, the outlook for patients with complete transposition of the great arteries (TGA) has improved dramatically. However, many survivors are afflicted with postoperative complications and continue to demand medical attention. We therefore performed the study for the evaluation of the clinical course in the patients with complete TGA after atrial switch operation. METHODS: We analyzed the data of 51 patients who underwent the atrial switch operation for complete TGA at Seoul National University Hospital between January 1981 and June 1993 retrospectively. RESULTS: The patients were composed of 36 males and 15 females (mean age at operation, 15+/-30 months, range 2 months-18 years). Senning operation was undertaken in 43 and Mustard operation in 8. Among these, 27 had simple and 24 complex complete TGA. The early mortality rate within 1 month postoperatively was 39% and the late mortality rate 16%. The actuarial survival rate after 10 years was 78%. Of these, 17 cases have been followed up so far. Although the significant tricuspid regurgitation was noted in 9, right ventricular systolic function was maintained in all of them. Preoperative sinus node dysfunction was noted in 1 case. Postoperatively, premature ventricular contractions were found in 8 cases, atrial flutter in 4, and sinus node dysfunction in 7. Subclinical pulmonary venous obstruction was found in 2 and systemic venous obstruction in 1. At present, one takes digoxin for prevention of atrial flutter and 7 take digoxin, furosemide, and/or angiotensin-converting enzyme inhibitors for prevention of ventricular functional deterioration. Twelve cases were in New York Heart Association functional class I at the latest follow-up and 5 were in class II. CONCLUSION: This study revealed that morbidity and mortality were not low after atrial switch operation. However, considering the good long-term functional status in the survivors, atrial switch operation could be an alternative to arterial switch operation in complicated cases of TGA.

Clinical Experience of Transcatheter Coil Embolization in Children

BACKGROUND: Transcatheter coil embolization has been described as a method of nonsurgical closure of variable pathologic vascular structures. This study was aimed to evaluate the outcome of transcatheter coil embolization in variable clinical conditions. METHODS AND RESULTS: We collected data from patients' medical record and their cardiac angiography films. From January 1995 to June 1997, coil embolization was attempted in 51 patients who were 38 patients with systemic-pulmonary collaterals (5 patients have venous collaterals, too), six patients with venous collaterals, nine patients with patent ductus arteriosus (PDA), one patient with Blalok-Taussig shunt (BT shunt), one patient with coronary-right atrial fistula and one patient with coronary-right ventricular fistula. In 38 patients with systemic-pulmonary collaterals, 123 coils were inserted to 70 collaterals, therefore mean 1.79+/-0.77 coils were inserted to one collateral. The results were complete occlusions (74%), incomplete occlusions (21%), and partial occlusions (4%). In six patients with venous collaterals, the outcomes were complete occlusions (50%) and incomplete occlusions (50%). In a patient with BT shunt, hemolytic anemia occurred in 1st attempt and in 2nd attempt, shunt was incompletely occluded and one coil was carried away and embolized the peripheral pulmonary artery. In nine patients with PDA, ten cases of transcatheter coil embolization was executed. Mean minimum ductal diameter was 2.1+/-0.85 mm. The results were initial occlusion (30%), occlusion within one month (66%), and occlusion within one year (75%). Left pulmonary artery stenosis owing to coil insertion was not found. In one case of coil malposition, retrieval and reinsertion of coil was successful. In two patients who have coronary artery fistula, coil embolization was successfully executed without any complications. CONCLUSIONS: Transcatheter coil embolization executed in variable clinical conditions without significant complications. It was effective and safe nonsurgical method.

Clinical Experience of Transcatheter Coil Embolization in Children

BACKGROUND: Transcatheter coil embolization has been described as a method of nonsurgical closure of variable pathologic vascular structures. This study was aimed to evaluate the outcome of transcatheter coil embolization in variable clinical conditions. METHODS AND RESULTS: We collected data from patients' medical record and their cardiac angiography films. From January 1995 to June 1997, coil embolization was attempted in 51 patients who were 38 patients with systemic-pulmonary collaterals (5 patients have venous collaterals, too), six patients with venous collaterals, nine patients with patent ductus arteriosus (PDA), one patient with Blalok-Taussig shunt (BT shunt), one patient with coronary-right atrial fistula and one patient with coronary-right ventricular fistula. In 38 patients with systemic-pulmonary collaterals, 123 coils were inserted to 70 collaterals, therefore mean 1.79+/-0.77 coils were inserted to one collateral. The results were complete occlusions (74%), incomplete occlusions (21%), and partial occlusions (4%). In six patients with venous collaterals, the outcomes were complete occlusions (50%) and incomplete occlusions (50%). In a patient with BT shunt, hemolytic anemia occurred in 1st attempt and in 2nd attempt, shunt was incompletely occluded and one coil was carried away and embolized the peripheral pulmonary artery. In nine patients with PDA, ten cases of transcatheter coil embolization was executed. Mean minimum ductal diameter was 2.1+/-0.85 mm. The results were initial occlusion (30%), occlusion within one month (66%), and occlusion within one year (75%). Left pulmonary artery stenosis owing to coil insertion was not found. In one case of coil malposition, retrieval and reinsertion of coil was successful. In two patients who have coronary artery fistula, coil embolization was successfully executed without any complications. CONCLUSIONS: Transcatheter coil embolization executed in variable clinical conditions without significant complications. It was effective and safe nonsurgical method.

Percutaneous transluminal coronary angioplasty in a child with Kawasaki disease

A successful attempt at percutaneous transluminanl coronary angioplasty (PTCA) to relieve stenosis of the mid-portion of the left anterior descending artery was achieved in a 6-year 9-month old boy who had multiple coronary aneurysms and stenosis due to Kawasaki disease. Despite the progression of coronary stenosis he had been well except for the perfusion defect of the anterior wall of myocardium on 99mTc-MIBI SPECT with dipyridamole infusion until PTCA was carried out after 4-year 4-months of the onset of illness. The area of stenosis was 70% before PTCA and 20% after PTCA. No restenosis at the site of PTCA was observed on follow-up angiography at 26 months after PTCA. This successful attempt may indicate that this procedure should be considered early in subclinical stenosis to prevent ischemic cardiac damage.