Hepatocellular adenoma is an uncommon, benign liver tumor usually occurring in patients using estrogen or anabolic androgens and in those with a genetic disease, including glycogen storage disease. Hepatocellular adenomas can sometimes induce pain. However, it is usually asymptomatic. Moreover, few studies have reported cases of hepatocellular adenomas presenting with iron deficiency anemia. Herein, we report a pediatric case of a large hepatocellular adenoma, presenting with iron therapy-refractory iron deficiency anemia. A 14-year-old boy was diagnosed with hepatocellular adenoma during an anemia work-up. Improvement in iron deficiency anemia was observed after tumor resection.

Purpose: Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea. Materials and Methods: Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval. Results: Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in < 3 ng/mL, 23.8% in > 3 ng/mL; p < 0.001). Conclusion In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

BACKGROUND: Scar revision is a fundamental technique in the field of plastic and reconstructive surgery. Methods using local flaps, such as a Z-plasty, W-plasty, or geometric broken-line closure, have been used for scar revision. Widening of scars is a frustrating event that most plastic surgeons have encountered. Several therapeutic modalities have been proposed, but frequently, the result is disappointing. We describe our experience with the use of a dermal splinting technique for scar revision. METHODS: We propose a technique by which tension on the wound is applied by tough scar tissue instead of the suture line, thereby reducing the incidence of postoperative widening. This technique was used to treat 21 nonburn scars that had widened:5 scars were facial (3 patients), 8 were on the extremities (6 patients), and 8 were on the torso (5 patients). All scars were at least 4 months old. The operations were performed between January 2003 and December 2012; follow-up was 9 to 24 months. RESULTS: Only one scar widened during the follow-up period. Overall, satisfaction with scar appearance and surgery was assessed with a visual analog scale (VAS). Mean patient satisfaction was 8.1 +/- 0.5. CONCLUSIONS: We recommend this technique in dealing with widened scars in highly tensile areas during revision surgery.
Cicatrix* ; Cosmetic Techniques ; Extremities ; Follow-Up Studies ; Incidence ; Patient Satisfaction ; Splints* ; Sutures ; Torso ; Visual Analog Scale ; Wounds and Injuries

BACKGROUND: In 2004, we reported on 110 consecutive Asian rhinoplasty patients who were treated with the addition of a footplate incision to obtain a greater aesthetic satisfaction. We continue to perform the reported technique with several modifications, and we still think that this method contributes to Asian rhinoplasty. METHODS: A footplate incision was made along the caudal border of the footplate of the medial crura onto the floor of the nasal vestibule. This incision can be made alone or in combination with either endonasal or open rhinoplasty. It enables surgeons to achieve a further tip projection since the pressure of the skin flap is reduced on the tip. In this study, we emphasize a couple of recent changes that we made to our procedure after publication of the prior article. First, we excised the dog-ear that appeared at the caudal end of the extended footplate incision. After making the new tip, a dog ear can be seen at the posterior end of the footplate incision. Second, an inferior columellar dissection was also extended to achieve an additional tip projection and to improve the columello-labial angle. RESULTS: This study included 85 consecutive patients who underwent an aesthetic rhinoplasty using footplate incision techniques between August of 2010 and May of 2013. A total of 43 patients had an adequate follow-up time of over 12 months. The majority of the patients (40/43 cases) were satisfied with the results. CONCLUSIONS: The authors believe that a footplate incision in aesthetic rhinoplasty is safe and can reliably achieve better results for Asian patients.
Animals ; Asian Continental Ancestry Group ; Dogs ; Ear ; Follow-Up Studies ; Humans ; Publications ; Rhinoplasty* ; Skin

BACKGROUND AND OBJECTIVES: Interleukin-21 receptor (IL-21R) gene polymorphism is related with the development of systemic vasculitis. In this study, we investigated the polymorphisms of IL-21R gene in patients with Kawasaki disease (KD). SUBJECTS AND METHODS: We genotyped the promoter region of IL-21R gene (-2500 bp to +1 bp) in 100 patients with KD and 100 healthy controls. All study subjects were Korean. We designed five pairs of primers and performed polymerase chain reaction (PCR) and direct sequencing. We analyzed whole promoter sequences of 200 individuals with comparison to reference sequences of IL-21R gene (NG_012222.1/NC_000016.9). RESULTS: We found five single nucleotide polymorphisms (SNPs) of which minor allele frequency (MAF) >0.01 in the promoter region of IL-21R gene. Those are -1681 G>T (chromosome site 27411802), -379 G>A (27413104), -332 G>C (27413151, rs2214537), -237 A>T (27413246), and -53 G>A (27413430). There is no significant difference in MAF of each SNP between patients with KD and healthy controls except -237 A>T. Twenty five patients with KD had more than 1 SNP in contrast to only seven healthy controls had. The patients with KD have significantly more IL-21R gene polymorphisms than controls (odds ratio: 3.0, 95% confidence interval: 1.6-5.6, p=0.0005). There was no significant correlation between IL-21R gene polymorphisms and the serum level of IL-21. The serum level of total IgE was not significantly correlated with the presence of IL-21R gene polymorphisms. CONCLUSION: Our data suggest that the genetic susceptibility profile for KD may include IL-21R gene.
Gene Frequency ; Genetic Predisposition to Disease ; Humans ; Immunoglobulin E ; Interleukins ; Mucocutaneous Lymph Node Syndrome ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Receptors, Interleukin-21 ; Systemic Vasculitis

PURPOSE: The serum level of immunoglobulin (Ig)E has been reported to be elevated in patients with Kawasaki disease (KD). We investigated whether interleukin (IL)-21, rather than IL-4, could be related to elevated serum levels of IgE in KD. METHODS: Sera from 48 patients with KD and 12 controls with high fever were collected to determine the level of IgE using an immunoassay system and the levels of IL-4 and IL-21 were determined using enzyme-linked immunosorbent assay kits. RESULTS: The median IL-21 level of KD patients was significantly elevated, at 499.5 pg/mL (range: <62.5-1,544 pg/mL), whereas that of controls was <62.5 pg/mL (<62.5-825 pg/mL; P<0.001). The median IL-4 level of KD patients was not elevated (4.0 pg/mL; 2.1-7.6 pg/mL). The median level of total IgE in KD patients was 58.0 IU/mL (5-1,109 IU/mL). No statistically significant correlation was found between IL-21 and total IgE levels (Spearman's R=0.2; P=0.19). CONCLUSIONS: Patients with KD have elevated levels of IL-21 in the serum. IL-21 may play a role in the pathogenesis of KD.
Enzyme-Linked Immunosorbent Assay ; Fever ; Humans ; Immunoassay ; Immunoglobulin E ; Immunoglobulins ; Interleukin-4 ; Interleukins ; Mucocutaneous Lymph Node Syndrome

Cryptococcus is an opportunistic pathogen that mainly affects immunocompromised hosts and, less frequently, immunocompetent hosts. It causes serious morbidity and mortality due to systemic infections such as meningoencephalitis and pulmonary infection. Urinary involvement of Cryptococcus is sometimes reported among cases of disseminated cryptococcosis in AIDS patients, but no such reports have been published in Korea. We report two cases of cryptococcuria that developed in a 71-year old female with diabetes and liver cirrhosis and in a 50-year old male who received a liver transplant due to HBV-associated hepatic failure. The female patient had received prednisolone for 12 days before we detected C. neoformans in urine culture. Even though no antifungal therapy was indicated for cryptococcuria, following urine culture became negative, but still positive for cryptococcal antigen on hospital day 25. Her blood, CSF culture, and antigen tests were negative, and therefore she was diagnosed with isolated cryptococcuria. The male patient had received prednisolone and tacrolimus for 10 days before sputum and urine cultures became positive for C. neoformans. He had ill defined nodules and pleural effusion in both lungs on chest CT. His cryptococcuria was sustained for over 2 months, despite receiving amphotericin B treatment. His cryptococcuria seemed to be a symptom of disseminated cryptococcosis.
Amphotericin B ; Cryptococcosis ; Cryptococcus ; Cryptococcus neoformans ; Female ; Humans ; Immunocompromised Host ; Korea ; Liver ; Liver Cirrhosis ; Liver Failure ; Lung ; Male ; Meningoencephalitis ; Pleural Effusion ; Prednisolone ; Sputum ; Tacrolimus ; Thorax ; Transplants

BACKGROUND AND OBJECTIVES: Kawasaki disease (KD) is an acute systemic vasculitis in children which causes coronary arterial dilatation (CAD) and gallbladder distension (GBD). There is a dearth of investigating the relationship between the severity of KD and GBD with lipid profiles. SUBJECTS AND METHODS: A total of 80 patients with 'complete KD' who were diagnosed from January 2005 to May 2009 was enrolled in this study. Serum cholesterol {total, high density lipoprotein-cholesterol (HDL-C) and low density lipoprotein-cholesterol (LDL-C)}, triglyceride (TG), complete blood count, inflammation markers {erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)} were measured at the time of admission during febrile period. Echocardiography and abdominal sonogram were performed in all patients to determine CAD and gallbladder size. According to GBD, patients with KD were classified as patients with GBD and patients without GBD. Between two groups, demographic and clinical data were analyzed. RESULTS: The serum level of LDL-C was significantly lower in patients with GBD (p=0.03) compared with patients without GBD or febrile control. There was no significant difference in inflammatory indices between patients with GBD and patients without GBD. GBD was not significant risk factor of CAD in this study (odds ratio=2.0, 95% confidence interval=0.82-5.3, p=0.16). CONCLUSION: This is the first study that highlights the relationship between the GBD and lipid metabolism in patients with KD. This study provides clinical insights about potential mechanism underpinning the relationship between the GBD and lipid metabolism.
Blood Cell Count ; C-Reactive Protein ; Child ; Cholesterol ; Coronary Artery Disease ; Dilatation ; Echocardiography ; Gallbladder ; Gallbladder Diseases ; Humans ; Inflammation ; Lipid Metabolism ; Mucocutaneous Lymph Node Syndrome ; Risk Factors ; Systemic Vasculitis

BACKGROUND: QuantiFERON(R)-TB Gold In Tube (QFT-G IT) has been used for diagnosing latent tuberculosis infection and active tuberculosis (TB) since 2007. However, there has not been enough data on QFT-G IT for universal use in children. In this study, we evaluated the clinical usefulness of the QFT-G IT in pediatric practice. METHODS: We retrospectively reviewed the clinical records of 70 patients younger than 18 years of age who had taken QFT-G IT and had a tuberculin skin test (TST) between July 2007 and July 2009 at Wonju Christian Hospital. The subjects were divided into two groups, asymptomatic TB exposure group and disease group. Four patients who were taking immunosuppressants during the study period were excluded. RESULTS: A total of 66 immunocompetent children were included in this study. Among 27 asymptomatic children who had contact histories of TB, 6 (22.2%) were found to be positive by QFT-G IT. Eleven (40.7%) and 5 (18.5%) children were found to be positive by TST with cutoff values of > or =5 mm and > or =10 mm, respectively. Agreement was fair to good between QFT-G IT and TST (kappa=0.59: cutoff value > or =5 mm, kappa=0.7: cutoff value > or =10 mm). In disease group, 14 patients (35.9%) were diagnosed with active tuberculosis, 8/14 (57.1%) were positive on TST and 9/14 (64.3%) on QFT-G IT. The positive rate of acid-fast bacilli smear, TB-polymerase chain reaction, and culture for tuberculosis was 11% (1/9), 27.3% (3/11) and 33.3% (3/9), respectively. CONCLUSION: Our data support that the QFT-G IT can be used as an additional diagnostic tool for latent and active tuberculosis infection in children.
Child ; Collodion ; Humans ; Immunosuppressive Agents ; Interferon-gamma ; Interferon-gamma Release Tests ; Latent Tuberculosis ; Retrospective Studies ; Skin Tests ; Tuberculin ; Tuberculosis