Biliary hamartomas are tumor-like malformations of the liver. Biliary hamartomas are a type of fibrocystic disorder originating from ductal plate malformation and are typically considered benign, but with the risk of malignant transformation. In this case report, we present a rare occurrence of intrahepatic cholangiocarcinoma (ICC) that developed from biliary hamartomas, along with a literature review. A 76-year-old man with a diagnosis of biliary hamartomas had a history of recurrent cholangitis for 12 years, necessitating cholecystectomy, ERCP, and repeated antibiotic treatments. During his last episode, imaging studies revealed a hypervascular infiltrative mass in the right posterior liver segment. A liver biopsy confirmed adenocarcinoma and subsequent surgical pathology revealed ICC originating from biliary hamartomas. Chronic inflammation in the bile duct associated with biliary hamartomas may serve as a potential trigger for malignant transformation, as observed in this case. Therefore, close surveillance is essential for patients with biliary hamartomas presenting with infectious complications.

BACKGROUND/OBJECTIVES: Hydrolyzed formula is often fed to infants with gastrointestinal or immune issues, such as malabsorption or cow's milk allergy, because enzymatic treatment has rendered it more digestible and less allergenic than standard cow's milk formula (SF). Partially hydrolyzed formula (PHF) should be considered for those infants who are intolerant to extensively hydrolyzed formula. However, there are concerns about the nutritional insufficiencies of PHF. We aimed to evaluate the effects of PHF on the growth and health indicators in infants who were at high-risk of allergic disease and potential candidates for consuming PHF. SUBJECTS/METHODS: A total of 83 infants aged 0–2 mon with a family history of allergies were assigned to consume either PHF or SF until 24 weeks of age. Anthropometric measures were obtained at baseline, 12 weeks, and 24 weeks; blood samples were drawn and evaluated at the end of the study. RESULTS: No significant differences were observed in weight, height, and weight-for-height at any time point in each sex between the PHF and SF groups. At 24 weeks of age, the weightfor-age and height-for-age z-scores of the SF group were higher than those of the PHF group, but there was no significant difference in the weight-for-height z-score. There were no significant differences in levels of white blood cells, hemoglobin, ferritin, protein, albumin, aspartate aminotransferase, alanine aminotransferase, eosinophil cationic protein, and immunoglobulin E. CONCLUSIONS In this study, there were no differences in growth and blood panels between the infants consuming PHF or SF. Therefore, infants who are unable to tolerate SF can be fed PHF without nutritional concerns about growth.

Background: Atopic dermatitis (AD) is a heterogeneous disease with different age of onset, disease course, clinical symptoms, severity, and risk of comorbidity. The characteristics of children with AD also vary by age or country. However, little is known about the clinical characteristics of AD in Korean school-aged children and adolescents. Furthermore, there are few studies on phenotypic differences according to onset age. This study aimed to explore the clinical characteristics and phenotypes according to onset age and severity of AD in children and adolescents in Korea. Methods: AD patients aged 6–18 years who presented to 18 hospitals nationwide were surveyed.The patients were examined for disease severity by pediatric allergy specialists, and data on history of other allergic diseases, familial allergy history, onset age, trigger factors, lesion sites,treatment history and quality of life were collected. The results of the patient’s allergy test were also analyzed. The patients were classified into infancy-onset (< 2 years of age), preschoolonset (2–5 years of age), and childhood-onset (≥ 6 years of age) groups. Study population was analyzed for clinical features according to onset-age groups and severity groups. Results: A total of 258 patients with a mean age of 10.62 ± 3.18 years were included in the study. Infancy-onset group accounted for about 60% of all patients and presented significantly more other allergic diseases, such as allergic rhinitis and asthma (P = 0.002 and P = 0.001, respectively). Food allergy symptoms and diagnoses were highly relevant to both earlier onset and more severe group. Inhalant allergen sensitization was significantly associated with both infancy-onset group and severe group (P = 0.012 and P = 0.024, respectively). A family history of food allergies was significantly associated with infancyonset group (P = 0.036). Severe group was significantly associated with a family history of AD, especially a paternal history of AD (P = 0.048 and P = 0.004, respectively). Facial (periorbital, ear, and cheek) lesions, periauricular fissures, hand/foot eczema, and xerosis were associated with infancy-onset group. The earlier the onset of AD, the poorer the quality of life (P = 0.038). Systemic immunosuppressants were used in only 9.6% of the patients in the severe group. Conclusion This study analyzed the clinical features of AD in Korean children and adolescents through a multicenter nationwide study and demonstrated the phenotypic differences according to onset age and severity. Considering the findings that the early-onset group is more severe and accompanied by more systemic allergic diseases, early management should be emphasized in young children and infants.

Objective: Atopic dermatitis (AD) is a chronic inflammatory disease of the skin that is diagnosed by clinical characteristics including itchiness, eczema, dry skin, etc. High levels of indoor air pollutants may exacerbate atopic diseases, along with various allergic respiratory diseases, especially for those who spend most of their lives indoors. This study was conducted to evaluate the main components responsible for the aggravation of AD symptoms. Methods: A total of 31 patients with AD aged 6 months and 6 years old were enrolled. The measurement of air quality included particulate matter with a diameter of 2.5 μm or less (PM2.5), temperature, relative humidity, and CO2 in their homes. The symptom severity of AD was assessed by the daily record of symptom scores and the degree of skin hydration. Results: The skin hydration level at the most severe area selected by the patient’s caregiver was decreased by median 1.7% (interquartile range [IQR], 0.4%–3.0%) or median 15% (IQR, 5.3%–24%) with a unit increase of indoor PM2.5 (P=0.0133) or room temperature (P=0.0034). CO2 also showed a potentially negative association with the change of skin hydration level but it was not statistically significant. Conclusion Our study showed that indoor PM2.5 and temperature could impact the aggravation of skin hydration in children. Therefore, further studies including a large number of cases and interventions are necessary.

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

Chronic spontaneous urticaria (CSU) is defined as the occurrence of spontaneous wheals, angioedema, or both for >6 weeks in the absence of specific causes. It is a common condition associated with substantial disease burden both for affected individuals and societies in many countries, including Korea. CSU frequently persists for several years and requires high-intensity treatment; therefore, patients experience deteriorations in quality of life and medication-associated complications. During the last decade, there have been major advances in the pharmacological treatment of CSU and there is an outstanding need for evidence-based guidelines that reflect clinical practice in Korea. The guidelines reported here represent a joint initiative of the Korean Academy of Asthma, Allergy and Clinical Immunology and the Korean Dermatological Association, and aim to provide evidence-based guidance for the management of CSU in Korean adults and children. In Part 1, disease definition, guideline scope and development methodology as well as evidence-based recommendations on the use of antihistamines and corticosteroids are summarized.

Background and Objectives: The nucleotide-binding oligomerization domain-like receptor (NLRP) 3 is known as a member of the NLR family, and it has been confirmed that the NLRP3 inflammasome is associated with various diseases such as asthma, inflammatory bowel disease, metabolic disorders and multiple sclerosis, as well as other auto-immune and auto-inflammatory diseases. However, the role of NLRP3 in chronic rhinosinusitis with nasal polyps (CRSwNP) has not yet been explored.Subjects and Method Forty-four specimens of nasal polyps and 25 specimens of uncinate processes were collected from patients with chronic rhinosinusitis with nasal polyps, and 25 specimens of uncinate tissues were collected from patients who underwent other rhino-surgeries. The western blot assay was employed to analyze the expression of NLRP3; interleukin (IL)-1β and IL-17A were detected using immunohistochemistry and real-time polymerase chain reaction. The production of lipopolysaccharide (LPS) induced IL-1β and IL-17A with or without the NLRP3 inflammasome inhibitor (MCC950) was measured using an enzyme linked immunosorbent assay in cultured dispersed nasal polyp cells. Results: NLRP3 showed a high level of expression in nasal polyps than in the control group (p<0.01). The expression of IL-1β and IL-17A was significantly higher in nasal polyps in the CRSwNP group than in the control group (p<0.05). LPS-induced production of IL-1β was significantly suppressed by treatment with the NLRP3 inflammasome inhibitor (p<0.05). Conclusion The NLRP3 inflammasome plays an essential role in the pathogenesis of CRSwNP, and thus MCC950 can be considered a prospective therapeutic for NLRP3 inflammasome-mediated inflammation in nasal polyps. Our data provide new evidence that IL-17A is involved in inflammasome-associated inflammation in nasal polyps.

Background: and Objective Endoscopic airway dilation is the primary treatment for pediatric subglottic stenosis (SGS) due to its feasibility and non-invasiveness. The aim of this study is to evaluate the risk factors for the failure of endoscopic airway dilation in pediatric patients with SGS.
Materials and Methods: This study reviewed medical records of 38 pediatric patients had endoscopic dilation from a single and tertiary referral center, retrospectively. The success of the endoscopic dilation procedure was defined as no dyspneic symptom without tracheostomy or laryngotracheal reconstruction. Demographic profiles, underlying disease, and Myer-Cotton SGS severity grade were recorded. Success rates and risk factors for the failure of treatment were analyzed.
Results: The SGS patients with severity grade I was most common. After mean 1.8 numbers of procedures, there were 23 patients (60.5%) in the success group and 15 patients (39.5%) in the failure group. Age, sex, underlying diseases, and SGS severity grade were not significantly different between two groups. In patients who had multiple endoscopic procedures, the failure group showed SGS deteriorated after procedures in 66.7%, compared to 11.1% of the success group. In multivariable analysis, a long-term intubation (≥1 month) was identified as an independent risk factor for failure of endoscopic dilation procedure.
Conclusion Although endoscopic dilation procedure is safe and effective for the management, repetitive endoscopic dilation may not give clinical benefit in patient with long-term intubation. Other airway procedures must be considered in those group of patients.

Objective: Atopic dermatitis (AD) is a chronic inflammatory disease of the skin that is diagnosed by clinical characteristics including itchiness, eczema, dry skin, etc. High levels of indoor air pollutants may exacerbate atopic diseases, along with various allergic respiratory diseases, especially for those who spend most of their lives indoors. This study was conducted to evaluate the main components responsible for the aggravation of AD symptoms. Methods: A total of 31 patients with AD aged 6 months and 6 years old were enrolled. The measurement of air quality included particulate matter with a diameter of 2.5 μm or less (PM2.5), temperature, relative humidity, and CO2 in their homes. The symptom severity of AD was assessed by the daily record of symptom scores and the degree of skin hydration. Results: The skin hydration level at the most severe area selected by the patient’s caregiver was decreased by median 1.7% (interquartile range [IQR], 0.4%–3.0%) or median 15% (IQR, 5.3%–24%) with a unit increase of indoor PM2.5 (P=0.0133) or room temperature (P=0.0034). CO2 also showed a potentially negative association with the change of skin hydration level but it was not statistically significant. Conclusion Our study showed that indoor PM2.5 and temperature could impact the aggravation of skin hydration in children. Therefore, further studies including a large number of cases and interventions are necessary.

With increasing need to prevent serious food allergy reactions, Korean food allergen labeling regulation has been revised repeatedly. This paper aims to summarize current statuses of food allergen labeling in Korea and foreign countries and to analyze the issue of food allergen labeling regulation. Korean food labeling regulation currently requires 19 items and 22 foods to be reported on labels (eggs, milk, buckwheat, peanut, soybean, wheat, mackerel, crab, shrimp, pork, peach, tomato, sulfite, walnut, chicken, beef, squid, shellfish, and pine nut). However, some common food triggers (for example, almond, cashew nut, and kiwi fruit) are not included in the current labeling regulation. Another issue is that the Korean labeling regulation has not yet been fully implemented for nonprepacked foods; thus, consumers still have difficulty in correctly identifying allergenic ingredients in food. It should be assessed whether warning statements for cross-contamination are reasonable. To prevent the occurrence of serious reactions from accidental ingestion, efforts must be made to solve recently raised issues including the items required to be listed on food labels, the system of standards for labeling and display methods.
Anacardium ; Arachis ; Chickens ; Decapodiformes ; Eating ; Fagopyrum ; Food Hypersensitivity ; Food Labeling ; Hypersensitivity ; Juglans ; Korea ; Lycopersicon esculentum ; Milk ; Nuts ; Perciformes ; Prunus dulcis ; Prunus persica ; Red Meat ; Shellfish ; Soybeans ; Triticum