Chronic granulomatous disease (CGD) is one of congenital immunodeficient disease and a rare X-linked or autosomal recessive disease characterized by recurrent life- threatening infections and granuloma formation. We observed clinical features, laboratory findings and genetic subgroups of 33 children who were diagnosed with chronic granulomatous disease in the Department of Pediatrics, Seoul National University Children's Hospital. There were 23 males and 10 females. Activated NBT test of all patients revealed 0% positive cell and mothers of 15 patients had 25%- 75% normal neutrophils in the activated NBT test. According to the result of activated NBT test and family history, the ratio of X-linked and autosomal recessive inheritance was 2:3. There was a significant difference for the age at onset of the first infection in the different genetic subgroups. The X-linked group had the mean onset at 1.98 months of age and autosomal recessive group had a mean onset as late as 3.82 months (p<0.05). The most common type of the first infection was lymphadenopathies (41%) and other infections were skin pustules, fever, perianal abscess, pneumonia and chronic diarrhea. However, the age at diagnosis was not significant in the different genetic subgroups. Lymphadenitis (27%) was the most common infection, and pneumonia, gastrointestinal tract infection, skin infection were also common. The most common infectious agent was Candida sp. (5%) and other microorganisms involved were BCG, coagulase-negative staphylococcus, S. aureus, K/ebsiella pneumoniae, Aspergi/lus sp., and Enterococcus faecium. Chronic condition associated with CGD were hepatomegaly (59%), splenomegaly, and anemia of chronic disease, underweight, and lymphadenopathy. The leukocyte count of patients at diagnosis was within normal limit except in three patients and leukopenia was not observed in any of the patients. The humoral and cellular immunity and complement system were normal, but the level of Ig E in four patients was elevated. Early diagnosis of CGD can be made by suspicion if there are lymphadenitis after BCG vaccination and recurrent pyogenic infections under the first year of age. Though progression in the treatment of CGD, like gene therapy, is concerned, genetic counseling and prenatal diagnosis by carrier detection and molecular genetic analysis is thought to be necessary.
Abscess ; Anemia ; Candida ; Child ; Chronic Disease ; Complement System Proteins ; Diagnosis ; Diarrhea ; Early Diagnosis ; Enterococcus faecium ; Female ; Fever ; Gastrointestinal Tract ; Genetic Counseling ; Genetic Therapy ; Granuloma ; Granulomatous Disease, Chronic* ; Hepatomegaly ; Humans ; Immunity, Cellular ; Korea* ; Leukocyte Count ; Leukopenia ; Lymphadenitis ; Lymphatic Diseases ; Male ; Molecular Biology ; Mothers ; Mycobacterium bovis ; Neutrophils ; Pediatrics ; Pneumonia ; Prenatal Diagnosis ; Seoul ; Skin ; Splenomegaly ; Staphylococcus ; Thinness ; Vaccination ; Wills

BACKGROUND: Glycolic acid has become an important and popular agent for chemical peel. Even though many clinicians perform superficial chemical peel to treat facial acne with glycolic acid, as yet there has been no well-controlled clinical trinls for the comparison of this new peeling agent and Jessner's solution. OBJECTIVE: To compare these two chemical peelI agents, our study was focused on three aspects : 1) treatment effects (acne improvement), 2) side effects (erythema, exfoliation), 3) patients preferences of the treatments. METHOD: Sixteen patients with facial acne vulgaris were treated with 70% glycolic acid and Jessner's solution on each side of the face simultaneously. Treatments were repeated biweekly with acne grading. Patient's subjective preferences were asked after the treatment.
Acne Vulgaris* ; Humans

No abstract available.
Pigmentation*

No abstract available.
Pigmentation*

Obesity is a risk factor for numerous health problems. Behavior therapy is important for obesity treatment. The aim of the present study was to identify the parameters that are associated with successful weight reduction. A database of 123 subjects who underwent weight reduction programs at the Center for Health Promotion, Samsung Medical Center from June 2001 through November 2004 was retrospectively analyzed. The goal of the program was to reduce the body weight by more than 5% during the follow-up period. The study population was divided into 2 categories (the success group and the failure group) based on the outcome of weight reduction. We analyzed the demographic, laboratory and clinical parameters to determine the predictors of successful weight reduction. The prevalence of success group was 36.6% (45/123). Significant correlations for successful weight reduction were found for the follow up period and the frequency of medical visits. Our results suggest that strong motivation was the most important factor for successful weight reduction.

PURPOSE: This study aimed to develop and evaluate the effects of an empathy education program for undergraduate nursing students. METHODS: The study employed a non-equivalent control group pretest-posttest design. A total of 46 nursing students were voluntarily recruited by convenience sampling from senior nursing students from the nursing department of K college in I city, Korea. Participants were divided into two groups, an experimental group of 23 and a control group of 23. The experimental intervention (empathy education program) was conducted from April 19 to May 6, 2016 and consisted of 150-minute sessions, twice a week, for 3 weeks for a total of 15 hours. RESULTS: Participants demonstrated improvements in perspective taking in cognitive empathy, improved empathic concern in emotional empathy, and improved communicative empathy. Analysis of reflective writings identified four theme clusters regarding communicative empathy: improvement of empathic expression, experiencing comfort and healing, improvement of interpersonal relationships, and experience of conflict resolution. Improved interpersonal ability and caring were also identified. CONCLUSION: The empathy education program improved cognitive, emotional and communicative empathy, and interpersonal skills and care, which are all necessary qualities for nurses.
Education ; Empathy ; Humans ; Interpersonal Relations ; Korea ; Negotiating ; Nursing ; Social Skills ; Students, Nursing

No abstract available.
Alopecia Areata* ; Alopecia* ; Bone Density*

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.
Adolescent ; Adult ; Aged ; Alleles ; Child ; Child, Preschool ; Coronary Arteriosclerosis/enzymology/etiology/*genetics ; Female ; Gelatinase B/genetics ; Gene Frequency ; Genotype ; Humans ; Infant ; Male ; Middle Aged ; Mucocutaneous Lymph Node Syndrome/*complications ; *Polymorphism, Genetic ; Promoter Regions (Genetics)/*genetics ; Research Support, Non-U.S. Gov't ; Risk Factors ; Stromelysin 1/*genetics

PURPOSE: We studied the value of clinical signs, laboratory findings and (99m)technetium dimercaptosuccinic acid(DMSA) scan in predicting the presence of vesicoureteral reflux(VUR) in children with first febrile urinary tract infection(UTI). METHODS: A retrospective analysis of 84 hospitalized children with first febrile UTI was performed. They underwent DMSA scan and voiding cystourethrography(VCUG) during the acute phase, and were divided into three groups according to the results of the VCUG: absence of VUR, mild(grade I-II) and severe VUR(grade III-V). We studied the relation of VUR to age, gender, fever duration, causative organism, white blood cell count, serum C-reactive protein and result of DMSA scan. RESULTS: Among 84 patients, 6 had mild and 17 had severe VUR. Thirty-eight had abnormal DMSA scan. results Patients with VUR were older than those without VUR(P<0.01). There was a lower probability of infection with Escherichia coli in patients with severe VUR than in those with mild and absent VUR(P<0.01). An abnormal DMSA scan correlated with the presence and severity of VUR(P<0.05). Severe VUR was present in 10.9% of patients with normal DMSA scan. The sensitivity, specificity, positive and negative predictive values of the DMSA scan in predicting the presence of VUR were 69.6%, 63.9%, 42.1%, and 84.8%, respectively. CONCLUSION: An abnormal DMSA scan correlated to the presence and severity of VUR, but the sensitivity, specificity and positive predictive value of the DMSA scan were low. Therefore, patient with an abnormal DMSA scan requires a VCUG. In order to prevent missing the 10.9% of patients with severe VUR but normal DMSA scans, a VCUG should be performed in patient with normal DMSA scan.
C-Reactive Protein ; Child* ; Child, Hospitalized ; Escherichia coli ; Fever ; Humans ; Leukocyte Count ; Retrospective Studies ; Sensitivity and Specificity ; Succimer ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux

An association between primary sclerosing cholangitis and ulcerative colitis is well known. But, primary sclerosing cholangitis with ulerative colitis has been rarely reported in children. The prevalence of primary sclerosing cholangitis among ulcerative colitis patiens is 3% in children. Primary sclerosing cholangitis is characterised by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The diagnosis of primary sclerosing cholangitis based on biochemical, histologic and cholangiographic criteria. A twofold or greater elevation of serum alkaline phosphatase is required to suspect this diagnosis. The definitive diagnosis of primary sclerong cholangitis can usually made by cholangiography. The prognosis varies. No adequate treatment exists although a number of potential treatments have been evaluated. We experienced a case of primary sclerosing cholangitis with ulcerative colitis in a 14 year old girl. She was admitted with a history of intermittent bloody diarrhea and jaundice over a two year period. Hepatosplenomegaly and cholestasis had been noted. Abnormal liver function tests were noted. AST was 117U/l, ALT 179U/l, alkaline phosphatase 603U/l, gamma-GT 366U/l, total bilirubin 5.5mg/dl. An endoscopic retrograde cholecystopancreatography showed evidence of strictures, beading, and irregularities of intra and extrahepatic biliary system. Liver biopsy showed histologic findings compatible with a sclerosing cholangitis and evidence of periductular fibrosis. She sufferred from persistent cholestasis and sign of portal hypertension. She had developed recurrent episodes of variceal hemorrhages which had been successfully managed several times endoscopic variceal ligations.
Adolescent ; Alkaline Phosphatase ; Bile Ducts, Extrahepatic ; Bilirubin ; Biopsy ; Child ; Cholangiography ; Cholangitis ; Cholangitis, Sclerosing* ; Cholestasis ; Colitis ; Colitis, Ulcerative* ; Constriction, Pathologic ; Diagnosis ; Diarrhea ; Female ; Fibrosis ; Hemorrhage ; Humans ; Hypertension, Portal ; Inflammation ; Jaundice ; Ligation ; Liver ; Liver Function Tests ; Prevalence ; Prognosis ; Ulcer*