Intramuscular Injection of Autologous Serum in Adolescent and Adult Patients with Atopic Dermatitis: A Preliminary Randomized Clinical Trial

Purpose@#The favorable clinical efficacies of intramuscular injection of autologous blood in patients with atopic dermatitis (AD) and intramuscular injection of autologous serum in patients with chronic urticaria have been demonstrated by randomized clinical trials. In this study, we assessed the clinical effectiveness and safety of the intramuscular injection of autologous serum in patients with AD. @*Materials and Methods@#In this randomized, placebo-controlled, and double-blind trial, 23 adolescent and adult patients with moderate-to-severe AD were enrolled. The patients were randomized to receive eight intramuscular injections of 5 mL of autologous serum (n=11) or saline (n=12) over 4 weeks, and were followed up until week 8. Changes in the clinical severity scores of AD assessed by SCORing Atopic Dermatitis (SCORAD), patient-reported Dermatology Life Quality Index (DLQI) score, and incidence of adverse events were assessed from baseline to week 8. @*Results@#One patient in the treatment group and two patients in the placebo group were lost to follow-up before week 8. The intramuscular administration of autologous serum, compared with saline, decreased the SCORAD clinical severity score (-14.8% vs. 10.7%, p=0.006) and improved the DLQI score (-32.6% vs. 19.5%, p=0.01) from baseline to week 8. Serious adverse events were not observed. @*Conclusion@#Intramuscular injection of autologous serum may be effective in treating AD. Further studies are needed to evaluate the clinical usefulness of this intervention for AD (KCT0001969).

Delayed diagnosis of 22q11 deletion syndrome due to late onset hypocalcemia in a 11-year-old girl with imperforated anus

Neonatal hypocalcemia and congenital heart defects has been known as the first clinical manifestation of the chromosome 22q11.2 deletion syndrome (22q11DS). However, because of its wide clinical spectrum, diagnosis of 22q11DS can be delayed in children without classic symptoms. We report the case of a girl with the history of imperforate anus but without neonatal hypocalcemia or major cardiac anomaly, who was diagnosed for 22q11DS at the age of 11 after the onset of overt hypocalcemia. She was born uneventfully from phenotypically normal Korean parents. Imperforate anus and partial cleft palate were found at birth, which were surgically repaired thereafter. There was no history of neonatal hypocalcemia, and karyotyping by GTG banding was normal. At the age of 11, hypocalcemia (serum calcium, 5.0 mg/dL) and decreased parathyroid hormone level (10.8 pg/mL) was noted when she visited our Emergency Department for fever and vomiting. The 22q11DS was suspected because of her mild mental retardation and velopharyngeal insufficiency, and a microdeletion on chromosome 22q11.2 was confirmed by fluorescence in situ hybridization. The 22q11DS should be considered in the differential diagnosis of hypocalcemia at any age because of its wide clinical spectrum.

The triglyceride-to-high density lipoprotein cholesterol ratio in overweight Korean children and adolescents

PURPOSE: The triglyceride-to-high-density lipoprotein cholesterol (TG/HDL-C) ratio has recently been reported as a biomarker of cardiometabolic risk in obese children and adolescents. The purpose of this study is to describe the TG/HDL-C ratio and related factors in overweight and normal weight Korean children and to evaluate whether the high TG/HDL-C ratio is associated with insulin resistance in overweight children and adolescents. METHODS: Data from 255 overweight (aged 8.7±2.0 years) and 514 normal weight (aged 8.9±1.8 years) children and adolescents were evaluated. Glucose, insulin, total cholesterol (TC), HDL-C and TG levels were measured after overnight fasting, and the TG/HDL-C ratio, non–HDL-C and the homeostasis model assessment of insulin resistance (HOMA-IR) were calculated. RESULTS: The TG/HDL-C ratio was higher in overweight group compared to normal weight group (P < 0.001). Among overweight children and adolescents, alanine aminotransferase (P=0.018), non–HDL-C (P < 0.001), and HOMA-IR (P=0.004) were different between the TG/HDL-C ratio tertile groups. The prevalence of elevated HOMA-IR was increased with increasing TG/HDL-C ratio tertiles (P for trend=0.003). On regression analysis adjusted for age and sex, the BMI (β=0.402, P=0.001) and TG/HDL-C ratio (β=0.251, P=0.014) were independently associated with HOMA-IR (adjusted R2=0.324). The TG/HDL-C ratio of 2.0 or more showed higher sensitivity (55.6%) and specificity (72.9%), when compared to TC (≥200 mg/dL), non–HDL-C (≥145 mg/dL), and LDL-C (≥130 mg/dL) for identifying overweight children with elevated HOMA-IR. CONCLUSION: The TG/HDL-C ratio is independently associated with insulin resistance in overweight children and adolescents, and it can be useful in identifying those at higher cardiometabolic risk.

Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma

Multiple endocrine neoplasia (MEN) mutation is an autosomal dominant disorder characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. The incidence of insulinoma in MEN is relatively uncommon, and there have been a few cases of MEN manifested with insulinoma as the first symptom in children. We experienced a 9-year-old girl having a familial MEN1 mutation. She complained of dizziness, occasional palpitation, weakness, hunger, sweating, and generalized tonic-clonic seizure that lasted for 5 minutes early in the morning. At first, she was only diagnosed with insulinoma by abdominal magnetic resonance images of a 1.3 × 1.5 cm mass in the pancreas and high insulin levels in blood of the hepatic vein, but after her father was diagnosed with MEN1. We found she had familial MEN1 mutation, and she recovered hyperinsulinemic hypoglycemia after enucleation of the mass. Therefore, the early genetic identification of MEN1 mutation is considerable for children with at least one manifestation.

The changes of subtypes in pediatric diabetes and their clinical and laboratory characteristics over the last 20 years

PURPOSE: We studied the changes in subtypes of diabetes mellitus (DM) in children and evaluated the characteristics of each group over the past 20 years. In addition, we also examined the correlation between the glycated hemoglobin (HbA1c) values at the time of diagnosis and lipid profiles. METHODS: The patients were divided into 2 groups: there were a total of 190 patients under 20 years of age firstly diagnosed with DM in Ajou University Hospital. The patients in groups I and II were diagnosed from September 1995 to December 2004 and from January 2005 to April 2014, respectively. RESULTS: The characteristics were compared between the 2 groups of patients. The result showed an increase in percentage of type 2 diabetes and maturity onset diabetes of the young (MODY) patients between the 2 groups. HbA1c and total cholesterol level had statistical significances to explain increasing the low-density lipoprotein cholesterol level among age, HbA1c, total cholesterol level, and z-scores of weight and body mass index (BMI) in type 2 diabetes. R-square was 0.074. However, z-score of BMI and total cholesterol level, not HbA1c, had statistical significances in type 1 diabetic patients. R-square was 0.323. CONCLUSION: The increase in the proportions of both type 2 diabetes and MODY in the last 10 years needed to be reminded when diagnosing the subtypes of DM, and the dyslipidemia should be attended more as a common problem of pediatric diabetic patients.

The changes of subtypes in pediatric diabetes and their clinical and laboratory characteristics over the last 20 years

PURPOSE: We studied the changes in subtypes of diabetes mellitus (DM) in children and evaluated the characteristics of each group over the past 20 years. In addition, we also examined the correlation between the glycated hemoglobin (HbA1c) values at the time of diagnosis and lipid profiles. METHODS: The patients were divided into 2 groups: there were a total of 190 patients under 20 years of age firstly diagnosed with DM in Ajou University Hospital. The patients in groups I and II were diagnosed from September 1995 to December 2004 and from January 2005 to April 2014, respectively. RESULTS: The characteristics were compared between the 2 groups of patients. The result showed an increase in percentage of type 2 diabetes and maturity onset diabetes of the young (MODY) patients between the 2 groups. HbA1c and total cholesterol level had statistical significances to explain increasing the low-density lipoprotein cholesterol level among age, HbA1c, total cholesterol level, and z-scores of weight and body mass index (BMI) in type 2 diabetes. R-square was 0.074. However, z-score of BMI and total cholesterol level, not HbA1c, had statistical significances in type 1 diabetic patients. R-square was 0.323. CONCLUSION: The increase in the proportions of both type 2 diabetes and MODY in the last 10 years needed to be reminded when diagnosing the subtypes of DM, and the dyslipidemia should be attended more as a common problem of pediatric diabetic patients.

Clinical Efficacy of Subcutaneous Allergen Immunotherapy in Patients with Atopic Dermatitis

PURPOSE: The clinical usefulness of subcutaneous allergen immunotherapy (SCIT) in the treatment of atopic dermatitis (AD) is still controversial. We analyzed the clinical efficacy of SCIT in patients with AD and the clinical characteristics of patients showing a favorable clinical response to the treatment. MATERIALS AND METHODS: Two hundred and fifty one patients with AD sensitized to house dust mite (HDM) were treated by SCIT using HDM extract. The clinical severity of AD was measured using the standardized clinical severity scoring system for AD (SCORAD) at baseline and 12 months. A favorable clinical response to SCIT was defined as a decrease in SCORAD value at 12 months greater than 50% compared to baseline value. Severe AD was defined as a baseline SCORAD value above 50. RESULTS: A favorable clinical response to SCIT was observed in 73.6% of patients. The proportion of patients showing a favorable clinical response to SCIT was significantly higher in patients with severe AD (90.6%) than patients with mild to moderated AD (63.7%) (p<0.001). Patients with severe AD showing a favorable clinical response had a significantly shorter duration of AD (12.3±8.5 years; mean±SD) than patients with severe AD showing no significant clinical response (20.6±10.9 years) (p<0.05) at baseline. CONCLUSION: SCIT could be a clinically useful therapeutic option for patients with severe AD sensitized to HDM. Early initiation of SCIT might provide a favorable clinical outcome in patients with severe AD sensitized to HDM.

Clinical Efficacy of Subcutaneous Allergen Immunotherapy in Patients with Atopic Dermatitis

PURPOSE: The clinical usefulness of subcutaneous allergen immunotherapy (SCIT) in the treatment of atopic dermatitis (AD) is still controversial. We analyzed the clinical efficacy of SCIT in patients with AD and the clinical characteristics of patients showing a favorable clinical response to the treatment. MATERIALS AND METHODS: Two hundred and fifty one patients with AD sensitized to house dust mite (HDM) were treated by SCIT using HDM extract. The clinical severity of AD was measured using the standardized clinical severity scoring system for AD (SCORAD) at baseline and 12 months. A favorable clinical response to SCIT was defined as a decrease in SCORAD value at 12 months greater than 50% compared to baseline value. Severe AD was defined as a baseline SCORAD value above 50. RESULTS: A favorable clinical response to SCIT was observed in 73.6% of patients. The proportion of patients showing a favorable clinical response to SCIT was significantly higher in patients with severe AD (90.6%) than patients with mild to moderated AD (63.7%) (p<0.001). Patients with severe AD showing a favorable clinical response had a significantly shorter duration of AD (12.3±8.5 years; mean±SD) than patients with severe AD showing no significant clinical response (20.6±10.9 years) (p<0.05) at baseline. CONCLUSION: SCIT could be a clinically useful therapeutic option for patients with severe AD sensitized to HDM. Early initiation of SCIT might provide a favorable clinical outcome in patients with severe AD sensitized to HDM.

The relationship between serum adiponectin and inflammatory cytokines in obese Korean juveniles / 소아과

PURPOSE: Obesity is related to systemic inflammatory processes causing cardiovascular complications. Intercellular adhesion molecule-1 (ICAM-1), CD40 ligand (CD40L), P-selectin are newly described mediators of inflammation and have a significant effect in atherosclerosis. Adiponectin has shown anti-inflammatory effects in adults. The aim of this study was to evaluate the relationship between adiponectin and inflammatory mediators in children and adolescents. METHODS: Fifty children or adolescents, twenty two with a body mass index (BMI) over 95th percentile, and twenty eight with a BMI below 75th percentile were included in the study. Serum soluble ICAM-1 (sICAM-1), P-selectin, CD40L, lipid profiles, aspartate aminotransferase, alanine aminotransferase, glucose and insulin were measured to evaluate associations with adiponectin. Comparison of these variables was performed between the obese and the nonobese group. RESULTS: We found a adiponectin to be significant lower and sICAM-1 significant higher in the obese group compared to the nonobese group, but there were no significant differences in P-selectin and soluble CD40L. Adiponectin was negatively associated with ICAM-1 and P-selectin in the obese group. CONCLUSION: Negative associations of adiponectin with ICAM-1 and P-selectin in obese children and adolescents suggest that serum adiponectin level may represent the inflammatory status.

Clinical characteristics of primary spontaneous pneumothorax in adolescents: factors for recurrence

PURPOSE: The aim of this study was to investigate the clinical characteristics of primary spontaneous pneumothorax (PSP) in adolescents and identify risk factors for the recurrence of PSP. METHODS: A total of 292 patients diagnosed with PSP from January 1998 to December 2011 were retrospectively studied. Clinical data on demographics, diagnostic imaging, therapies, and risk factors of recurrence were collected and analyzed. RESULTS: The sex ratio of 292 patients was 19.8:1 (male:female), and the average age of the patients was 17.0 years. The average body mass index of the patients was 18.8 kg/m2. The most common presenting symptom was chest pain. There was no seasonal variation in the incidence of PSP. Thirty patients (10.2%) had a history of smoking. The most common location of PSP was the left side. Out of 249 patients, 169 (67.9%) had cysts (blebs/bullae). Fifty-four patients (18.5%) received oxygen therapy, 3 patients (1%) needle aspiration, 119 patients (40.8%) closed tube drainage, and 116 patients (39.7%) surgery. The recurrence rate was 38.6%. Smoking was associated with the size of pneumothorax (P=0.002). Also, the size of pneumothorax and surgery was associated with recurrence (P=0.040 and P=0.004). However, previously reported risk factors for recurrence were not identified in our patients. CONCLUSION: Pediatric PSP occurred mainly in males in late adolescence with normal body mass index. No significant risk factors were related to recurrence of PSP in our study.

Prevalence and comorbidity of allergic diseases in preschool children / 소아과

PURPOSE: Allergic disease and its comorbidities significantly influence the quality of life. Although the comorbidities of allergic diseases are well described in adult populations, little is known about them in preschool children. In the present study, we aimed to assess the prevalence and comorbidity of allergic diseases in Korean preschool children. METHODS: We conducted a cross-sectional study comprising 615 Korean children (age, 3 to 6 years). Symptoms of allergic diseases were assessed using the Korean version of the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire that was modified for preschool children. Comorbidities of allergic diseases were assessed by 'In the last 12 months, has your child had symptoms?'. RESULTS: The prevalence of symptoms of asthma, allergic rhinitis, and atopic dermatitis as recorded using the ISAAC questionnaire, within the last 12 months was 13.8%, 40.7%, and 20.8%, respectively. The symptom rates of allergic conjunctivitis, food allergy, and drug allergy were 14.8%, 10.4%, and 0.8%, respectively. The prevalence of allergic rhinitis in children with asthma was 64.3% and that of asthma in children with allergic rhinitis was 21.6%. The prevalence of rhinitis in children with conjunctivitis was 64.8% and that of conjunctivitis in children with rhinitis was 23.6%. CONCLUSION: The prevalence of current rhinitis in our preschool children is shown to be higher than that previously reported. Allergic conjunctivitis is closely associated with asthma and allergic rhinitis. However, further studies are warranted to determine the prevalence and effects of these comorbidities on health outcomes in preschool children.

Relationship between the asthma and rhinitis in asthmatic children: comparison of allergic rhinitis and nonallergic rhinitis

PURPOSE: We aimed to determine the prevalence of allergic rhinitis and nonallergic rhinitis, difference in symptoms between allergic rhinitis and nonallergic rhinitis, and the association between lung function and the degree of asthma control in children with asthma. METHODS: One hundred seventy patients who were followed-up for asthma treatment at the department of pediatrics of CHA Bundang Medical Center were enrolled in this study. We conducted the questionnaire regarding coexistence of rhinitis, childhood asthma control test (C-ACT), and the basic lung function test. The patients were classified as allergic rhinitis group and nonallergic rhinitis group according to the response to 11 common inhalation and food allergens, and assessed the degree of asthma control and the severity of rhinitis. RESULTS: One hundred thirty patients (73%) were found to have rhinitis. Of these, 79 patients (53%) had allergic rhinitis and 34 patients (20%) had nonallergic rhinitis. The allergic rhinitis group was older than the nonallergic rhinitis group or the nonrhinitis group (7.73+/-2.85 vs. 5.97+/-2.48 vs. 6.12+/-2.70, P<0.001). Nasal itching sense was more prevalent in the allergic-rhinitis group than in the nonallergic rhinitis group (3.23+/-1.90 vs. 2.44+/-1.56, P=0.036). There was an inverse correlation between the rhinitis and C-ACT (r= -0.329, P<0.05). Of note, nasal obstruction symptom was highly correlated with C-ACT (r=-0.334, P<0.001). CONCLUSION: Allergic rhinitis and nonallergic rhinitis were highly prevalent in the pediatric patients with asthma and both of them had a significantly adverse impact on asthma control by rhinitis-itself. Therefore, regardless of atopic status, clinicians should focus on relieving rhinitis symptoms.

Changes in the Predicted Adult Height after Gonadotropin-Releasing Hormone Agonist Treatment in Girls with Idiopathic True Precocious Puberty

PURPOSE: We evaluated the effects of the timing of treatment initiation with gonadotropin-releasing hormone agonist (GnRHa) on the change in predicted adult height (PAH) in girls with idiopathic true precocious puberty (TPP). METHODS: Data for this retrospective study were collected on 104 girls with TPP who were treated with GnRHa for 36 months, between January 2002 and March 2012. RESULTS: The PAH SDS differed before and after treatment in all patients (-1.91 +/- 1.47 vs. -1.37 +/- 1.17 after 1 year of treatment, -1.96 +/- 1.58 vs. -0.48 +/- 1.11 after 3 years of treatment) as well as in Group 1 (-2.15 +/- 1.54 vs. -1.51 +/- 1.20 after 1 year of treatment, -2.09 +/- 1.59 vs. -0.55 +/- 1.19 after 3 years of treatment) and Group 2 (-1.57 +/- 1.34 vs. -1.17 +/- 1.12 after 1 year of treatment, -1.50 +/- 1.55 vs. -0.21 +/- 0.74 after 3 years of treatment). This result could be due to improvement in bone age advancement during the treatment. The difference between mid-parental height SDS and PAH SDS was decreased after GnRHa treatment. However, the means of PAH SDS did not surpass the mid-parental height SDS. CONCLUSION: GnRHa treatment can preserve growth potential by slowing bone age progression, resulting in short adult height, but it cannot alter the genetic growth potential.

Changes in the Predicted Adult Height after Gonadotropin-Releasing Hormone Agonist Treatment in Girls with Idiopathic True Precocious Puberty

PURPOSE: We evaluated the effects of the timing of treatment initiation with gonadotropin-releasing hormone agonist (GnRHa) on the change in predicted adult height (PAH) in girls with idiopathic true precocious puberty (TPP). METHODS: Data for this retrospective study were collected on 104 girls with TPP who were treated with GnRHa for 36 months, between January 2002 and March 2012. RESULTS: The PAH SDS differed before and after treatment in all patients (-1.91 +/- 1.47 vs. -1.37 +/- 1.17 after 1 year of treatment, -1.96 +/- 1.58 vs. -0.48 +/- 1.11 after 3 years of treatment) as well as in Group 1 (-2.15 +/- 1.54 vs. -1.51 +/- 1.20 after 1 year of treatment, -2.09 +/- 1.59 vs. -0.55 +/- 1.19 after 3 years of treatment) and Group 2 (-1.57 +/- 1.34 vs. -1.17 +/- 1.12 after 1 year of treatment, -1.50 +/- 1.55 vs. -0.21 +/- 0.74 after 3 years of treatment). This result could be due to improvement in bone age advancement during the treatment. The difference between mid-parental height SDS and PAH SDS was decreased after GnRHa treatment. However, the means of PAH SDS did not surpass the mid-parental height SDS. CONCLUSION: GnRHa treatment can preserve growth potential by slowing bone age progression, resulting in short adult height, but it cannot alter the genetic growth potential.

Isotype and IgG Subclass Distribution of Autoantibody Response to Alpha-enolase Protein in Adult Patients with Severe Asthma

PURPOSE: A possible involvement of autoimmune mechanism in the pathogenesis of bronchial asthma has been proposed. Recently, alpha-enolase protein was identified as a major autoantigen recognized by circulating IgG autoantibodies in patients with severe asthma. To evaluate a possible pathogenetic significance of these autoantibodies in severe asthma, isotype (IgG, IgA, IgM, and IgE) and IgG subclass (IgG1, IgG2, IgG3, and IgG4) distributions of autoantibodies to recombinant human alpha-enolase protein were analyzed. PATIENTS AND METHODS: We examined serum samples from 10 patients with severe asthma and 7 patients with mild-to-moderate asthma, and 5 healthy controls by immunoblot analysis. Severe asthma was defined as patients having at least 1 severe asthmatic exacerbation requiring an emergency department visit or admission in the last year despite continuous typical therapies. RESULTS: IgG1 was the predominant IgG subclass antibody response to alpha-enolase protein in patients with severe asthma. IgG1 autoantibody to alpha-enolase protein was detected in 7 of 10 patients with severe asthma (70%), 1 of 7 patients with mild-to-moderate asthma (14.3%), and none of 5 healthy controls (0%) (chi-square test; p < 0.05). IgA, IgM, and IgE autoantibodies to alpha-enolase protein could not be detected in patients with severe asthma. CONCLUSION: IgG1 subclass was the predominant type of autoantibody response to alpha-enolase protein in patients with severe asthma, suggests a possibility of IgG1 autoantibody- mediated complement activation in the pathogenesis of severe asthma.