A case of esophagitis and mediastinitis developed in an infant with an esophageal duplication cyst

An esophageal duplication cyst (EDC) is a congenital anomaly of the foregut. EDCs are often asymptomatic, so they are found incidentally on chest radiographs. In symptomatic patients, EDCs present with cough, dyspnea, vomiting, dysphagia, and chest pain. Potential complications of EDCs include perforation, obstruction, bleeding, and infection. The rarity of this condition may hinder timely diagnosis, which may lead to life-threatening complications. This article describes a case of esophagitis and mediastinitis developed in a 12-month-old infant with an EDC. We emphasize that although rare, EDCs should be considered in the differential diagnosis of non-specific respiratory signs such as cough, dyspnea, and stridor.

A case of food-induced acute pancreatitis in a child with egg white allergy

Acute pancreatitis (AP) rarely occurs in association with food allergy (FA), and the mechanism is not clearly explored. We herein report a rare case of egg protein-induced AP. A 5-year-old girl was brought to the hospital because of tongue pruritus and an increased level of egg white-specific immunoglobulin E. During an oral food challenge, she developed hives, abdominal pain and vomiting after ingestion of a cumulative of 610 mg of boiled egg white (70 mg of protein). After management with intramuscular injection of epinephrine and orally administered prednisolone and levocetirizine, her skin symptoms improved. The next day, blood tests showed elevated levels of amylase and lipase, and computed tomography revealed a swollen pancreas with a low-attenuated necrotic portion. She was diagnosed with AP and treated with food restriction, intravenous methylprednisolone and gabexate mesilate. The serum levels of amylase and lipase were reduced to normal levels on day 5 and her symptoms completely resolved on day 10. A suspicion of food-induced AP is required for correct diagnosis and proper management in children with FA when they manifest severe abdominal pain.

A case of ROHHAD (rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation) syndrome in an 11-year-old girl

Rapid-onset obesity with hypoventilation, hypothalamic, and autonomic dysregulation (ROHHAD) syndrome is a rare disease characterized by rapid progression of obesity and central hypoventilation with autonomic and endocrine dysregulation. There is no gold-standard diagnostic method for ROHHAD syndrome; it is diagnosed based on a years-long clinical course. For this reason, diagnosis of ROHHAD syndrome is often delayed. In particular, ROHHAD has a high mortality rate due to cardiopulmonary arrest when quick diagnosis and appropriate intervention of central sleep apnea are not timely. We report a case in which an 11-year-old girl with central sleep apnea was diagnosed with ROHHAD syndrome: the clinical course with early breathing intervention using noninvasive positive pressure ventilation. We emphasize the importance of respiratory interventions in the clinical course of ROHHAD syndrome.

Successful Sirolimus Treatment for Korean Patients with Activated Phosphoinositide 3-kinase δ Syndrome 1: the First Case Series in Korea

Activated phosphoinositide 3-kinase δ syndrome (APDS)1 is caused by gain-of-function mutations in PIK3CD, which encodes the catalytic p110δ subunit of phosphoinositide 3 kinase. We describe three patients with APDS1, the first thereof in Korea. Therein, we investigated clinical manifestations of APDS1 and collected data on the efficacy and safety profile of sirolimus, a mammalian target of rapamycin inhibitor and pathway-specific targeted medicine. The same heterozygous PIK3CD mutation was detected in all three patients (E1021K). After genetic diagnosis, all patients received sirolimus and experienced an excellent response, including amelioration of lymphoproliferation and improvement of nodular mucosal lymphoid hyperplasia in the gastrointestinal tract. The median trough level of sirolimus was 5.5 ng/mL (range, 2.8–7.5) at a dose of 2.6–3.6 mg/m2. Two patients who needed highdose, short-interval, immunoglobulin-replacement treatment (IGRT) had a reduced requirement for IGRT after initiating sirolimus, and the dosing interval was extended from 2 and 3 weeks to 4 weeks. The IgG trough level after sirolimus treatment (median, 594 mg/dL; range, 332–799 mg/dL) was significantly higher than that before sirolimus treatment (median, 290 mg/dL; range, 163–346 mg/dL) (p<0.001). One episode of elevated serum creatinine with a surge of sirolimus (Patient 2) and episodes of neutropenia and oral stomatitis (Patient 1) were observed. We diagnosed the first three patients with APDS1 in Korea. Low-dose sirolimus may alleviate clinical manifestations thereof, including hypogammaglobulinemia.

Analysis of the Change in Seropositive Rate of the Epstein-Barr Virus in Koreans: A Single-Center Study

Purpose@#Epstein-Barr virus (EBV) infection is related to infectious mononucleosis or nasopharyngeal cancer, and its epidemiology may change according to the socioeconomic development of communities. This study aimed to evaluate the recent epidemiology of EBV seropositive rate in Korea. @*Methods@#We retrospectively reviewed EBV serology test results obtained from a part of clinical care at Samsung Medical Center, Seoul, South Korea, from January 2000 to December 2017. @*Results@#The EBV seropositive rate in 26,527 subjects during the study period was 81.0% (21,485/26,527): 44.4% (2,716/6,122) in subjects aged 0–9 years, 75.8% (2,077/2,739) in those aged 10–19 years, and 94.5% (16,692/17,666) in those aged ≥20 years. The EBV seropositive rate decreased from 89.4% (8,592/9,616) in 2000–2008 to 76.2% (12,893/16,911) in 2009– 2017 (P<0.001). Especially, the EBV seropositive rate in subjects aged 0–19 years significantly decreased from 2000–2008 to 2009–2017 (0–9 years, 62.8% [1,172/1,866] in 2000–2008 and 36.3% [1,544/4,256] in 2009–2017; 10–19 years, 83.8% [745/858] in 2000–2008 and 70.8% (1,332/1,881) in 2009–2017) (P<0.001). @*Conclusions@#The EBV seropositive rate in children has decreased in the last 20 years. As the age of patients with primary EBV infection increased, there is a need for interest in clinical manifestation, such as infectious mononucleosis, in adolescents and young adults.

Communicating Bronchopulmonary Foregut Malformation Type III with Pulmonary Sequestration Diagnosed in a Newborn: A Case Report

Communicating bronchopulmonary foregut malformation (CBPFM) is a communication between the respiratory and gastrointestinal tracts that can be difficult to differentiate from pulmonary sequestration or H-type tracheoesophageal fistula (TEF) because of the similarities in clinical features. A female neonate born at full term had been experiencing respiratory difficulty during feeding from the third day of life. The esophagography performed to rule out H-type TEF revealed that the esophageal bronchus directly communicated with the left lower lobe (LLL) of the lung. Lobectomy of the LLL, fistulectomy of the esophagobronchial fistula, and primary repair of the esophagus were performed. Finally, CBPFM type III with pulmonary sequestration was confirmed on the basis of the postoperative histopathological finding. We report the first newborn case of CBPFM type III with pulmonary sequestration in Korea.

Factors related to Endocrine Disruptors Exposing Behaviors in Mothers of Infants

PURPOSE: The purpose of this study was to examine factors associated with behaviors of mothers that expose their infants to endocrine disruptors. The PROCEED model was used as a theoretical basis for the study. METHODS: This correlational study included 120 mothers with infants between the ages of 1 month to 36 months. Participants were recruited through the maternal community web-site of mothers of infants in W city. RESULTS: There was a significant negative correlation among experience of mothers who had participated in environmentally friendly activities, the health status of the infants perceived by the mother, and behaviors of mothers that exposed infants to endocrine disruptors. The factors based on PROCEED model explained 15.3% of the behaviors by mothers that exposed infants to endocrine disruptors. CONCLUSION: It is necessary to develop and provide educational programs that include detailed information on relationship of endocrine disruptors to children's health.

The Relationships of Chewing Frequency, Eating Rates, and Food Consumption with the Body Mass Index and Gender of College Students

In this study, we examined chewing frequency, eating rates, and food consumption volume according to the body mass index of male and female college students. Twenty-five male college students were divided into a normal weight male group ( or =23 body mass index, n=11). Twenty-five female college students were divided into a normal weight female group ( or =23 body mass index, n=4). Subjects were provided with abundant fried rice and allowed to consume it at their own pace. Chewing frequency, total mealtime, rate of eating, consumption volume, calorie intake, and the amount of one bite were measured. There were no significant differences in chewing frequency, total mealtime, rate of eating, consumption volume, calorie intake, and the amount of one bite between the normal and obese male groups. There were also no significant differences in chewing frequency, consumption volume, calorie intake, and the amount of one bite between the normal and obese female groups. However, the obese female group had a significantly shorter mealtime and significantly higher eating rate compared with the normal group. Thus, our study suggests that females who eat a meal for a longer time may reduce their food intake, consequently inducing body weight loss.

The Effect of Nutrition Education and the Diet Rice Bowl on Weight Control

This study examined the effectiveness of nutrition education and program using the diet rice bowl during a four-week diet program. Thirty-eight female college students were randomly assigned to a nutrition education (NE) group or a nutrition education and diet rice bowl (ND) group. The mean energy intake of the NE and ND group during the program was 1,130.8 kcal and 1,287.4 kcal, respectively. The total energy intake of both groups were significantly decreased during the program. In addition, the change of body weight and fat in both groups significantly decreased during the program. The NE group lost 2.8 kg of body weight and 1.4% of body fat, while the ND group lost 2.5 kg of body weight and 1.2% of body fat. The changes in total cholesterol and LDL-cholesterol in both groups also significantly decreased during the program. However, there were no significant differences in the changes of body weight, body fat mass, the total cholesterol and LDL-cholesterol between the two groups. This study could not identify a weight reduction effect of the diet rice bowl, however, this may be due to subjects' infrequent use of the diet rice bowl by their frequent meal skipping and eating-out in our study. Therefore, further studies regarding the compliance to the diet rice bowl should be conducted to examine its effectiveness on weight control.

The General Characteristics of Geriatrics Being Hospitalized in Rehabilitation Hospital for a Stroke

BACKGROUND: We investigated the demographic characteristics of stroke patients over 65 years old and wanted to see the effects of rehabilitation therapies by examining the changing status of patients before and after treatment by comparing with other patients under 65 years old. METHODS: We went through medical records arranged by making a comparative groups of older patients and other younger patients who both admitted within recent 4 years. Accompanied medical problems are categorized by ICD-10, the functional statuses are classified into FIM score at admission, and FIM efficiency at discharge. RESULTS: Between two groups, according to cause of a stroke, the existence of spouse, gender, there is statistical difference and, at admission, functional level of older patients was lower but it was not observed that extent of functional change of the result of rehabilitation therapy with distinction of age. Accompanied diseases were average 4.6 and is cardiovascular disease with the highest rates. CONCLUSION: Since the explosion of an aging population is regarded as serious national problem, people have been trying to find diversified treatments against geriatrics. And the data will be utilized as useful materials for better direction where rehabilitation treatment against a stroke should aim.

Clinical Manifestations of Post-stroke Sexual Lives

OBJECTIVE: To assess the impacts of stroke on sexual activities and functioning of patients with stroke and to study the associations of clinical and psychosocial factors with sexual changes following stroke. METHOD: Ninety-seven patients with stroke were interviewed and completed questionnaire concerning their prestroke and poststroke coital frequency, sexual satisfaction, and sexual functions such as erection, ejaculation, and vaginal lubrication. RESULTS: Sexual activities such as coital frequency and sexual satisfaction were decreased in patients after stroke. Sexual dysfunctions including impotence (68%), a decline in ejaculation (66.7%), and a decline in vaginal lubrication (89.5%) were high. Subjective reasons for decreased sexual life after stroke were denial of spouse, impotence, decreased libido, no opportunity of sexual activity due to hospitalization or no sexual partner, fear of relapse, physical discomfort due to hemiparesis and so on. Comparing sexual group with no sexual group after stroke, there were different in age (p=0.040), brain lesion site (p=0.036), prestroke coital frequency (p=0.048), erectile ability (p=0.005), and ejaculation (p<0.001). CONCLUSION: The decline in sexual activities and functioning was due to interpersonal, physical, psychological factors among patients following stroke.

Standing and Gait of the Paraplegics by Spinal Cord Injury after Discharge

OBJECTIVE: The purpose of this follow-up study was to evaluate the status of standing and gait for the paraplegics at their home after discharge. METHOD: A medical doctor interviewed 93 paraplegics by spinal cord injury, who visited the outpatient clinic of the National Rehabilitation Hospital, or who were admitted for the follow up urologic evaluation. RESULTS: Out of 93 paraplegics, 90 patients had done a standing exercise in hospitals, but 42 patients continued a standing exercise after discharge. Seventy-nine patients had done a gait exercise in hospitals, but only 24 patients continued a gait exercise after discharge. Seventy-five patients were prescribed KAFO (Knee-Ankle-Foot orthosis), but only 11 patients used the KAFO continuously after discharge. The reasons to stop using KAFO were loss of motivation, lack of time, no helper, difficulties with wearing orthoses, and etc. The continuous using rates of other standing or exercise equipments were higher than that of KAFO. CONCLUSION: Many paraplegics had done gait training and had been prescribed KAFO in hospitals. But only 14.7% of them had used KAFO for any purpose after discharge. We believe that more careful consideration and explanation are needed to each patient. We think that standing or exercise equipments are good alternatives.

Reference values of carotid artery intima-media thickness and association with atherosclerotic risk factors in healthy subjects in Korea / 대한내과학회지

BACKGROUND: Atherosclerosis is an important cause of cardiovascular and cerebrovascular diseases, which are the major cause of death in middle-aged and older adults. However atherosclerotic lesions are not easily detected before they cause symptoms and signs which means that early detection is important. In Korea it has been reported that the carotid artery intima-media thickness (IMT) correlates well with the atherosclerotic risk factors in type 2 diabetes mellitus, but there is little data available on the carotid artery IMT in healthy subjects. Therefore, the aims of this study were to determine the reference values of the carotid artery IMT in healthy subjects and to investigate the associations of the carotid artery IMT with the atherosclerotic risk factors. METHODS: Ultrasound high-resolution B-mode imaging of the carotid arteries was conducted in 757 healthy subjects (385 men, 372 women) in order to determine the mean and maximal carotid artery IMT and the presence of carotid artery atherosclerotic plaques. RESULTS: The mean carotid artery IMT values with respect to gender were 0.69+/-0.18 mm in men and 0.67+/-0.16 mm in women (NS). The mean carotid artery IMT values with respect to age in men and women were 0.57+/-0.09 mm vs 0.57+/-0.10 mm in those aged 30 to 39, 0.61+/-0.11 mm vs 0.60+/-0.10 mm in those aged 40 to 49, 0.72+/-0.21 mm vs 0.67+/-0.12 mm in those aged 50 to 59, 0.77+/-0.15 mm vs 0.71+/-0.13 mm in those aged 60 to 69 and 0.88+/-0.18 mm vs 0.76+/-0.20 mm in those aged 70 to 79, respectively. The mean carotid artery IMT was significantly greater with increasing age (p<0.001), particularly between those aged 40 to 49 and 50 to 59 (p<0.001). The maximal carotid artery IMT values for men (0.72+/-0.22 mm) were significantly greater than women (0.69+/-0.15 mm). The maximal carotid artery IMT values with respect to age in men and women were 0.60+/-0.10 mm vs 0.61+/-0.12 mm in those aged 30 to 39, 0.65+/-0.11 mm vs 0.64+/-0.11 mm in those aged 40 to 49, 0.79+/-0.29 mm vs 0.71+/-0.14 mm in those aged 50 to 59, 0.83+/-0.20 mm vs 0.77+/-0.18 mm in those aged 60 to 69 and 0.97+/-0.29 mm vs 0.80+/-0.22 mm in those aged 70 to 79. The mean carotid artery IMT was significantly greater with increasing age (p<0.001), particularly between those aged 40 to 49 and 50 to 59 (p<0.001). Age, systolic and diastolic BP, body mass index, waist/hip ratio, total cholesterol, LDL cholesterol correlated with the mean and maximal carotid artery IMT, and smoking, serum glucose, triglyceride, and HDL cholesterol levels were not correlated with the mean and maximal carotid artery IMT. Being male correlated with the maximal carotid artery IMT, but not correlated with the mean carotid artery IMT. CONCLUSION: This study suggested reference values for the mean and maximal carotid artery IMT in healthy subjects, which can be an index for the carotid artery IMT of Koreans. Furthermore, there were significant correlations between the carotid artery IMT and the atherosclerotic risk factors in healthy subjects.

Effect of Repeated Monothermal Caloric Stimulation on the Bithermal Caloric Response / 대한이비인후과학회지

We investigated normal response of bithermal caloric stimulation after repeated monothermal caloric stimulation. 12 subjects were selected for cold(30degreesC) water stimulation. 8 subjects were stimulated by warm(44degreesC) water. After the bithermal caloric test, caloric stimuli(cold or warm) was repeated 5 times on both ear with a 10 minute time interval and the next bithermal caloric test was performed. Monothermal caloric stimuli was done at the same temperature as the previous test a day later. The bithermal caloric test was repeated after 4 days. We checked the maximum slow phase eye velocity(SPEV) and calculated the canal paresis and directional preponderance. The SEPV was changed from 26.4(+/-10.9) degrees/sec to 26.7(+/-20.5) degrees/sec in repeated warm stimulation; from 25.0(+/-11.4) degrees/sec to 27.3(+/-11.1) degrees/sec in cold stimulation. Canal paresis was changed from 12.4(+/-8.7)% to 6.3(+/-5.1)% in repeated warm water stimulation; from 9.6(+/-9.9)% to 6.9(+/-3.1)% in cold stimuli. Directional preponderance was changed from 11.9(+/-6.9)% to 8.7(+/-9.9)% in warm stimuli; from 11.5(+/-7.6)% to 10.6(+/-5.5)% in cold stimuli. There was no significant change of caloric response after repeated monothermal caloric stimulation.