Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to a disturbance in the growth of bone and soft tissue, affecting principally the head, hands, and feet. Recently we experienced a typical Apert syndrome expressed only in one neonate of dizygotic twin.
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Humans ; Infant, Newborn* ; Skull ; Syndactyly ; Twins, Dizygotic*

Diagnosing and selecting an appropriate treatment strategy for left main coronary artery (LMCA) obstruction is very important. Although this disease is not frequently encountered, it can cause severe hemodynamic deterioration resulting in a less favorable prognosis without a suitable management approach. Another aspect of LMCA that we must not overlook is coronary artery spasm, which can be an infrequent but important cause of acute coronary syndrome. Although it is rare, LMCA can cause critical complications. In this study, we report the case of a 35-year-old female who was admitted to the hospital with a diagnosis of acute myocardial infarction with ST-segment elevation in the aVR lead caused by a left main coronary spasm that was examined on intravascular ultrasound.
Acute Coronary Syndrome ; Adult ; Coronary Vasospasm ; Coronary Vessels ; Electrocardiography ; Female ; Hemodynamics ; Humans ; Myocardial Infarction ; Prognosis ; Spasm

Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome involving abrupt or insidious onset of hematuria, proteinuria, and anemia, and rapidly progressive renal failure. Crescentic glomerulonephritis is a histopathological term for RPGN showing extensive extracapillary proliferation, i.e., crescent formation. There are three major immunopathological categories of crescentic glomerulonephritis: anti-glomerular basement membrane (anti-GBM) antibody disease, immune complex-mediated, and pauci-immune (anti-neutrophil cytoplasmic autoantibody [ANCA]-positive). A small minority of all patients with glomerulonephritis develop crescentic glomerulonephritis. Anti-GBM antibodies and ANCA rarely coexist. There have been a few reports of dual positive crescentic glomerulonephritis with anti-GBM antibodies and ANCA in Korea. Here, we describe the case of a 73-year-old woman showing RPGN clinically and crescentic glomerulonephritis pathologically with coexisting anti-GBM antibodies and myeloperoxidase-ANCA.
Aged ; Anemia ; Antibodies ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Basement Membrane ; Cytoplasm ; Female ; Glomerulonephritis ; Hematuria ; Humans ; Immune System Diseases ; Korea ; Proteinuria ; Renal Insufficiency

The goal for the treatment of patients with diabetes has today shifted from merely reducing glucose concentrations to preventing the natural decline in beta-cell function and delay the progression of disease. Pancreatic beta-cell dysfunction and decreased beta-cell mass are crucial in the development of diabetes. The beta-cell defects are the main pathogenesis in patients with type 1 diabetes and are associated with type 2 diabetes as the disease progresses. Recent studies suggest that human pancreatic beta-cells have a capacity for increased proliferation according to increased demands for insulin. In humans, beta-cell mass has been shown to increase in patients showing insulin-resistance states such as obesity or in pregnancy. This capacity might be useful for identifying new therapeutic strategies to reestablish a functional beta-cell mass. In this context, therapeutic approaches designed to increase beta-cell mass might prove a significant way to manage diabetes and prevent its progression. This review describes the various beta-cell defects that appear in patients with diabetes and outline the mechanisms of beta-cell failure. We also review common methods for assessing beta-cell function and mass and methodological limitations in vivo. Finally, we discuss the current therapeutic approaches to improve beta-cell function and increase beta-cell mass.
Glucose ; Humans ; Insulin ; Obesity ; Pregnancy

Drug-eluting stents (DES) dramatically reduce the rate of restenosis and the need for repeat revascularization. Despite these promising results, stent thrombosis seems to occur more frequently with DES and often seems to be associated with premature discontinuation of antiplatelet agents. We experienced a case of recurrent stent thrombosis with separate lesions. Recurrent stent thrombosis is a very rare event, and simultaneous stent thrombosis is also rare. Here, we report a case of recurrently developing stent thrombosis involving separate lesions.
Coronary Thrombosis ; Coronary Vessels ; Drug-Eluting Stents ; Platelet Aggregation Inhibitors ; Stents ; Thrombosis

Paradoxical vocal fold movement (PVFM) is an uncommon upper airway disorder defined as paradoxical adduction of the vocal folds during inspiration. The etiology and treatment of PVFM are unclear. The physician should manage this condition because of the possibility of near complete airway obstruction in severe case of PVFM. We report a case of successful airway management in a patient with PVFM by applying continuous positive airway pressure (CPAP). In this case, PVFM was detected after removing an endotracheal tube from a 67-year-old male who underwent excision of a laryngeal mass. The patient recovered without complications in 1 day with support by CPAP.
Aged ; Airway Extubation* ; Airway Management ; Airway Obstruction ; Continuous Positive Airway Pressure* ; Humans ; Male ; Vocal Cords*

BACKGROUND: Macrophages are a component of a tumor's microenvironment and have various roles in tumor progression and metastasis. This study evaluated the relationships between tumor-associated macrophage (TAM) density and clinical outcomes in 14 different types of human cancers. METHODS: We investigated TAM density in human tissue microarray sections from 14 different types of human cancers (n = 266) and normal thyroid, lung, and breast tissues (n = 22). The five-year survival rates of each cancer were obtained from the 2011 Korea Central Cancer Registry. RESULTS: Among 13 human cancers, excluding thyroid cancer, pancreas, lung, and gallbladder cancers had the highest density of CD163-positive macrophages (7.0+/-3.5%, 6.9+/-7.4%, and 6.9 +/- 5.5%, respectively). The five-year relative survival rates of these cancers (pancreas, 8.7%; lung, 20.7%; gallbladder, 27.5%) were lower than those of other cancers. The histological subtypes in thyroid cancer exhibited significantly different CD163-positive macrophages densities (papillary, 1.8 +/- 1.6% vs anaplastic, 22.9 +/- 17.1%; p < .001), but no significant difference between histological subtypes was detected in lung and breast cancers. Moreover, there was no significant difference in CD163-positive macrophages densities among the TNM stages in lung, breast, and thyroid cancers. CONCLUSIONS: Cancers with higher TAM densities (pancreas, lung, anaplastic thyroid, and gallbladder) were associated with poor survival rate.
Breast ; Gallbladder ; Gallbladder Neoplasms ; Humans ; Korea ; Lung ; Macrophages* ; Neoplasm Metastasis ; Pancreas ; Prognosis ; Survival Rate* ; Thyroid Gland ; Thyroid Neoplasms

BACKGROUND/OBJECTIVES: Despite the importance of a low-iodine diet (LID) for thyroid cancer patients preparing for radioactive iodine (RAI) therapy, few studies have evaluated dietary intake during LID. This study evaluated the amount of dietary iodine intake and its major food sources during a typical diet and during LID periods for thyroid cancer patients preparing for RAI therapy, and examined how the type of nutrition education of LID affects iodine intake. SUBJECTS/METHODS: A total of 92 differentiated thyroid cancer patients with total thyroidectomy were enrolled from Seoul National University Hospital. All subjects completed three days of dietary records during usual and low-iodine diets before 131I administration. RESULTS: The median iodine intake was 290 µg/day on the usual diet and 63.2 µg/day on the LID. The major food groups during the usual diet were seaweed, salted vegetables, fish, milk, and dairy products and the consumption of these foods decreased significantly during LID. The mean energy intake on the LID was 1,325 kcal, which was 446 kcal lower than on the usual diet (1,771 kcal). By avoiding iodine, the intake of most other nutrients, including sodium, was significantly reduced during LID (P < 0.005). Regarding nutritional education, intensive education was more effective than a simple education at reducing iodine intake. CONCLUSION: Iodine intake for thyroid cancer patients was significantly reduced during LID and was within the recommended amount. However, the intake of most other nutrients and calories was also reduced. Future studies are needed to develop a practical dietary protocol for a LID in Korean patients.
Dairy Products ; Diet Records ; Diet* ; Education ; Energy Intake ; Humans ; Iodine* ; Milk ; Seaweed ; Seoul ; Sodium ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy ; Vegetables

Background: Thyroid cancer screening has contributed to the skyrocketing prevalence of thyroid cancer. However, the true benefit of thyroid cancer screening is not fully understood. This study aimed to evaluate the impact of screening on the clinical outcomes of thyroid cancer by comparing incidental thyroid cancer (ITC) with non-incidental thyroid cancer (NITC) through a meta-analysis. Methods: PubMed and Embase were searched from inception to September 2022. We estimated and compared the prevalence of high-risk features (aggressive histology of thyroid cancer, extrathyroidal extension, metastasis to regional lymph nodes or distant organs, and advanced tumor-node-metastasis [TNM] stage), thyroid cancer-specific death, and recurrence in the ITC and NITC groups. We also calculated pooled risks and 95% confidence intervals (CIs) of the outcomes derived from these two groups. Results: From 1,078 studies screened, 14 were included. In comparison to NITC, the ITC group had a lower incidence of aggressive histology (odds ratio [OR], 0.46; 95% CI, 0.31 to 0.7), smaller tumors (mean difference, −7.9 mm; 95% CI, −10.2 to −5.6), lymph node metastasis (OR, 0.64; 95% CI, 0.48 to 0.86), and distant metastasis (OR, 0.42; 95% CI, 0.23 to 0.77). The risks of recurrence and thyroid cancer-specific mortality were also lower in the ITC group (OR, 0.42; 95% CI, 0.25 to 0.71 and OR, 0.46; 95% CI, 0.28 to 0.74) than in the NITC group. Conclusion Our findings provide important evidence of a survival benefit from the early detection of thyroid cancer compared to symptomatic thyroid cancer.

The indolent nature and favorable outcomes associated with papillary thyroid microcarcinoma have prompted numerous prospective studies on active surveillance (AS) and its adoption as an alternative to immediate surgery in managing low-risk thyroid cancer. This article reviews the current status of AS, as outlined in various international practice guidelines. AS is typically recommended for tumors that measure 1 cm or less in diameter and do not exhibit aggressive subtypes on cytology, extrathyroidal extension, lymph node metastasis, or distant metastasis. To determine the most appropriate candidates for AS, factors such as tumor size, location, multiplicity, and ultrasound findings are considered, along with patient characteristics like medical condition, age, and family history. Moreover, shared decision-making, which includes patient-reported outcomes such as quality of life and cost-effectiveness, is essential. During AS, patients undergo regular ultrasound examinations to monitor for signs of disease progression, including tumor growth, extrathyroidal extension, or lymph node metastasis. In conclusion, while AS is a feasible and reliable approach for managing lowrisk thyroid cancer, it requires careful patient selection, effective communication for shared decision-making, standardized follow-up protocols, and a clear definition of disease progression.