ABSTRACT
Multiple miliary osteoma cutis (MMOC) is a rare variant of osteoma, characterized by multiple eruptive hard nodules on the face. A 70-year-old female presented with multiple solid skin-colored papules on both cheeks, unresponsive to conventional medical treatments. She reported receiving an injection of an unknown cosmetic filler substance into her face by an unlicensed medical practitioner 20 years ago. Facial computed tomography showed multiple small calcifications immediately adjacent to foreign material assumed to be the filler substance in the dermis. Histological examination revealed osteoclasts, osteocytes, and eosinophilic bony tissue in the dermis, suggestive of osteoma cutis. Although the pathogenesis remains unclear, inflammation caused by injected foreign material may induce metaplastic transformation of multipotent mesenchymal cells into osteoblasts, resulting in heterotopic ossification. Dermatologists should be aware that MMOC may occur following injection of foreign material by unlicensed practitioners. Performing a detailed history and clinical evaluation may aid in the diagnosis of such recalcitrant skin lesions.
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Background@#Oxidative stress is generally accepted as one of the principal pathogenesis of vitiligo, and keratinocyte-melanocyte interactions are also thought to play critical roles. It is well-known that antioxidant response and autophagy protect cells against oxidative damage, but the details and the compensatory relationship between the two mechanisms in the keratinocytes of vitiligo lesions remain unclear. @*Objective@#To evaluate the antioxidant response and autophagy status of patients with vitiligo and to explore the interactions between these two mechanisms. @*Methods@#Ten patients with clinicopathologically proven vitiligo and 10 normal controls were enrolled in our Department of Dermatology, Soonchunhyang University Hospital, Bucheon, Korea. Tissue samples of vitiligo lesions in the patient group and normal skin in the control group were immunohistochemically stained for nuclear factor erythroid 2-related factor 2 (Nrf2), Beclin-1, microtubule-associated protein light chain 3 (LC3)-II, and p62. The immunopositivity of epidermal keratinocytes was evaluated. @*Results@#Keratinocytes in vitiligo lesions had a significantly lower expression of Nrf2 (p=0.002) than that in the cells of normal controls. The levels of autophagy markers did not differ significantly between the two groups, but decreases in the Beclin-1 and LC3-II levels, and an increase in the p62 level in the patient group may indicate a small decrease in autophagy of patients with vitiligo. @*Conclusion@#Decreased antioxidant response and reduced autophagy may trigger melanocyte apoptosis in vitiligo lesions.
ABSTRACT
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.
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BACKGROUND@#Tissue engineering, including 3D bioprinting, holds great promise as a therapeutic tool for repairing cartilage defects. Mesenchymal stem cells have the potential to treat various fields due to their ability to differentiate into different cell types. The biomimetic substrate, such as scaffolds and hydrogels, is a crucial factor that affects cell behavior, and the mechanical properties of the substrate have been shown to impact differentiation during incubation. In this study, we examine the effect of the mechanical properties of the 3D printed scaffolds, made using different concentrations of cross-linker, on hMSCs differentiation towards chondrogenesis. @*METHODS@#The 3D scaffold was fabricated using 3D bioprinting technology with gelatin/hyaluronic acid (HyA) biomaterial ink. Crosslinking was achieved by using different concentrations of 4-(4,6-dimethoxy-1,3,5-triazin-2-yl)-4-methlymorpholinium chloride n-hydrate (DMTMM), allowing for control of the scaffold’s mechanical properties. The printability and stability were also evaluated based on the concentration of DMTMM used. The effects of the gelatin/HyA scaffold on chondrogenic differentiation was analyzed by utilizing various concentrations of DMTMM. @*RESULTS@#The addition of HyA was found to improve the printability and stability of 3D printed gelatin/HyA scaffolds.The mechanical properties of the 3D gelatin/HyA scaffold could be regulated through the use of different concentrations of DMTMM cross-linker. In particular, the use of 0.25 mM DMTMM for crosslinking the 3D gelatin/HyA scaffold resulted in enhanced chondrocyte differentiation. @*CONCLUSION@#The mechanical properties of 3D printed gelatin/HyA scaffolds cross-linked using various concentrations of DMTMM can influence the differentiation of hMSCs into chondrocytes.
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BACKGROUND@#Cryopreservation can cause mechanical and chemical stress, ultimately leading to the formation of reactive oxygen species (ROS) and oxidative stress. ROS inhibits the expression of antioxidant enzymes in cells, resulting in increased DNA fragmentation and apoptosis. In this paper, we used a vitrification method that has the advantage of producing less ice crystal formation, cost-effectiveness, and time efficiency during cryopreservation. The objective of this paper is to evaluate the degree of protection of ovarian tissue against oxidative stress when N-acetylcysteine (NAC) and Klotho proteins are treated in the vitrification process of ovarian tissue. @*METHODS@#The control group and the cryopreservation groups were randomly assigned, and treated NAC, Klotho, or the combination (NAC ? Klotho). The cell morphological change, DNA damage, senescence, and apoptosis of each group after the freeze–thaw process were compared using transmission electron microscopy, immunohistochemistry, and western blot analysis. @*RESULTS@#Both NAC and Klotho were found to be more effective at protecting against DNA damage than the control;however, DNA damage was greater in the NAC ? Klotho group than in the group treated with NAC and Klotho, respectively. DNA damage and cellular senescence were also reduced during the vitrification process when cells were treated with NAC, Klotho, or the combination (NAC ? Klotho). NAC increased apoptosis during cryopreservation, whereas Klotho inhibited apoptosis and NAC-induced apoptosis. @*CONCLUSION@#This study highlights Klotho’s benefits in inhibiting DNA damage, cell senescence, and apoptosis, including NAC-induced apoptosis, despite its unclear role in vitrification.
ABSTRACT
Benign cephalic histiocytosis (BCH) is an uncommon subtype of non-Langerhans cell histiocytosis. A 5-month-old boy presented with multiple yellowish facial papules and plaques, which later spread to his trunk and both extremities. Laboratory tests, including lipid profile, were normal. Histological examination revealed non-foamy histiocytes, lymphocytes, and some eosinophils in the dermis. Immunohistochemical staining was positive for CD68and factor XIIIa, but negative for CD1a and S-100. Additionally, the patient developed multiple café-au-lait spots with axillary and inguinal freckling. Next-generation sequencing identified a pathogenic variant of the neurofibromatosis type 1 (NF1) gene. Herein, we report a rare case of BCH in a patient with NF1. Although many cases of NF1 accompanied by juvenile xanthogranuloma have been reported, the association between BCH and NF1 has not been elucidated. However, considering that BCH may be a clinicopathological variant of juvenile xanthogranuloma, an association between the two diseases can be considered.
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Objective@#To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children. @*Materials and Methods@#Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher’s exact test. @*Results@#Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever (p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells (p = 0.004), C-reactive protein (p < 0.001), and serum amylase (p = 0.002), and low levels of albumin (p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites (p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites (p = 0.001), increase in the amount of ascites in a short time (p < 0.001), complex ascites (p < 0.001), and perihepatic pseudocysts (p < 0.001) were more common in the perforated group than in the non-perforated group. @*Conclusion@#Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings.
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A dermatophyte is a zoonotic infection that causes infection on the skin, hair, and nails and is transmitted through contact. The species of Microsporum, Trichophyton, and Epidermophyton are the major dermatophytes that infect humans. Tinea capitis is a dermatophyte infection in the scalp, and it may progress to Kerion celsi, including severe redness, swelling, and pus formation. Kerion celsi is sometimes misdiagnosed as a bacterial infection or a tumor. Trichophyton verrucosum is a zoophilic dermatophyte that mainly causes infection in cattle. It can be spread to dairy farmers or ranchers who have frequent contact with infected cattle. We report a pediatric case who received scalp tumor excision and a split-thickness skin graft for extensive and severe inflammatory scalp tumors that occurred after contact with cattle with ringworm. Finally, the patient was diagnosed with Kerion celsi caused by T. verrucosum infection. This was based on the patient’s medical history, clinical manifestations, and histopathologic findings.
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Objectives@#This study aimed to investigate the efficacy of postoperative submucosal injection of hyaluronidase (HUD) for reducing sequelae andquality of life (QOL) after mandibular third molar (M3M) surgery. @*Materials and Methods@#Participants with bilateral impacted M3M underwent surgical extraction with a split-mouth randomized controlled studydesign. M3M were removed by the same surgeon in 2 sessions, one a control and the other experimental. Submucosal injection of HUD was performedin the experimental session and submucosal injection of saline in the control session. Mouth opening, facial swelling, and pain intensity were measured before surgery, and then 2 and 7 days after surgery. The QOL of participants following surgery was evaluated by means of a patient-centered outcome questionnaire (PCOQ). @*Results@#A total of 36 patients was included in the final data analysis. There was a significant reduction in the maximal mouth opening and postoperative pain in the experimental side at the 2 and 7 days after surgery (P<0.05), and a remarkable difference in facial swelling was reported on the experimental side 7 days after surgery (P<0.05). The PCOQ demonstrated that participants reported less pain and swelling on the experimental side. @*Conclusion@#The present study provides clinical evidence that submucosal administration of HUD immediately after M3M surgery reduced postop-erative discomfort and improved patients’ QOL.
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The survival rate of cancer patients is increasing owing to the early diagnosis and treatment methods. Radiotherapy and chemotherapy may cause serious complications, such as ovarian failure and infertility. In particular, preservation of fertility in women of reproductive age with cancer could improve their quality of life as well as reduce social and psychological pain.Current Concepts: Embryo or oocyte cryopreservation is a method of fertility preservation; however, it cannot be utilized by all women with cancer because of the complications of the condition and treatment method. Ovarian tissue cryopreservation and transplantation enables fertility preservation in those needing immediate cancer treatment, such as chemotherapy or radiotherapy, or those unqualified for ovarian stimulation. A recent review reported that frozen-thawed ovarian transplantation led to approximately 130 live births with a conception rate of approximately 30%. Endocrine function recovery occurred in 92.9% of the patients between 3.5 and 6.5 months after transplantation.Discussion and Conclusion: In this study, we introduced various methods and strategies for improving the outcomes of ovarian tissue cryopreservation and transplantation. These results could serve as a reference for patients and clinicians to choose the best options for fertility preservation based on the patient’s current situation and condition.
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Genetic causes of developmental and epileptic encephalopathy (DEE) have been rapidly uncovered from mid-2010s. The mutations of gene enconding calcium channel, voltage-dependent, P/Q type, alpha 1A subunit (CACNA1A) are recently detected in DEE, which gene is already known well in familial hemiplegic migrine type 1 or episodic ataxia type 2. Ketogenic diet therapy (KDT) is effective in some DEE, which data is short in CACNA1A encephalopathy. A 3-month-old male with global developmental delay and multidrug-resistant focal seizures was diagnosed as epilepsy of infancy with migrating focal seizures (EIMFS). Brain magnetic resonance imaging and metabolic screening were all normal. Whole exome sequencing revealed two variants of CACNA1A : c.899A>C, and c.2808del that is from his mother. His seizures disappeared within 3 days whenever on KDT, which recurred without it. To our knowledge, this rare case of EIMFS with novel mutations of CACNA1A, is the first report in CACNA1A encephalopathy becoming seizure-free on KDT.
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Background and Objectives@#Mesenchymal stem cells (MSCs) elicit therapeutic effects against liver fibrosis in animal models. Human liver stem cells (HLSCs) are cells isolated from human liver tissue that have mesenchymal morphology and express MSC markers. HLSCs also possess intrahepatic stem cell properties. We introduce a rat model of liver fibrosis and trans-portal transplantation of HLSC to demonstrate alleviation of liver fibrosis. @*Methods@#and Results: Liver fibrosis was induced by intraperitoneal injection of Carbon tetrachloride (CCl 4 ). Sprague Dawley rats underwent simultaneous partial hepatectomy of the left hepatic lobe and HLSC transplantation via the portal vein. Gross appearance of the liver observed following CCl 4 injection showed cholestasis and surface nodularity. Sirius red staining revealed deposition of collagen fibers in the extracellular matrix (ECM). Following HLSC transplantation, human albumin secreting cells were detected by immunohistochemistry in liver specimens. Quantitative measurements of fibrosis area stained by Sirius red were compared between baseline and post-HLSC transplant (1×10 7 cells) following 10 weeks of CCl 4treatment liver specimens. Fibrosis area (p＜0.05), serum markers of liver inflammation and fibrosis (AST, ALT levels and APRI, p＜0.05) significantly decreased from baseline after HLSC transplantation. RNA expression in liver tissues revealed significant decrease in tissue inhibitor of matrix metalloproteinase 1 (TIMP1), TIMP2 expression and increase in hepatocyte growth factor expression following HLSC transplantation (p＜0.05). @*Conclusions@#HLSC transplantation effectively reduced the area of liver fibrosis with increased expression of factors promoting ECM degradation. These findings suggest the potential therapeutic role of HLSCs in various liver diseases presenting with liver fibrosis.
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Objectives@#We aimed to collect and report data from all patients who have been diagnosed with mucosal malignant melanoma to obtain the epidemiology and principles of current treatments. @*Materials and Methods@#Between January 2008 and December 2018, 20 patients underwent surgery or follow-up observations at Yonsei University Dental Hospital. The patients’ clinical information was reviewed retrospectively. @*Results@#Seventeen of 20 patients had undergone definitive surgery, while only 6 patients received adjuvant radiotherapy or systemic therapy. Eight of 20 patients, including those that had recurrent lesions, were provided immunotherapy. The 3-year survival for all stages was 50%, with a local recurrence rate of 75% and a metastasis rate of 65%. @*Conclusion@#The overall survival of patients receiving surgical treatment was longer than that of patients who did not undergo surgical resection.Eight of 20 patients received immunotherapy as the first-line regimen at our clinic, and those patients exhibited longer overall survival compared to patients in reported keynote studies.
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Background@#In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. @*Methods@#We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries.Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. @*Results@#The number of pediatric surgeries from 2002 to 2017 increased by 124%.Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. @*Conclusion@#The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.
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Purpose@#Short bowel syndrome (SBS) is the most common etiology for intestinal failure (IF) and these patients are at high risk of developing micronutrient deficiencies. This study aimed at assessing the level of vitamins in adult SBS patients at different stages of their disease before the initiation of multidisciplinary intestinal rehabilitation. @*Methods@#Patient data from November 2015 to March 2017 were retrospectively reviewed. Adult patients who underwent extensive bowel resection and were classified as SBS-IF were selected. Clinical data including age, sex, etiology of IF, biochemical data, nutritional status, nutrition support, and outcome of intestinal rehabilitation were analyzed. @*Results@#Nine patients with SBS-IF were included in the analysis. There were 6 male patients and 3 female patients, with a median age of 55.0 years. Vitamin levels were analyzed at 306 days (median) after the development of SBS. At the time of vitamin levels screening, 4 patients were receiving daily intravenous vitamin supplementation. Five patients were not receiving vitamin supplementations, either intravenously or orally. Vitamin B12 was within the normal range in 6 patients and higher than normal in 3 patients. Vitamin D was within the normal range in 3 patients and lower than normal in 6 patients. Vitamin E was within the normal range in 7 patients and higher than normal in 2 patients. Folate was within the normal range in 8 patients (not checked in 1 patient). Ambulatory patients had significantly higher vitamin D levels compared to hospitalized patients (P=0.015). @*Conclusion@#Vitamin D levels had decreased in 67% of patients with SBS in Korea, while vitamin B12, folate, and vitamin E deficiencies were rarely seen.
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Objectives@#We aimed to collect and report data from all patients who have been diagnosed with mucosal malignant melanoma to obtain the epidemiology and principles of current treatments. @*Materials and Methods@#Between January 2008 and December 2018, 20 patients underwent surgery or follow-up observations at Yonsei University Dental Hospital. The patients’ clinical information was reviewed retrospectively. @*Results@#Seventeen of 20 patients had undergone definitive surgery, while only 6 patients received adjuvant radiotherapy or systemic therapy. Eight of 20 patients, including those that had recurrent lesions, were provided immunotherapy. The 3-year survival for all stages was 50%, with a local recurrence rate of 75% and a metastasis rate of 65%. @*Conclusion@#The overall survival of patients receiving surgical treatment was longer than that of patients who did not undergo surgical resection.Eight of 20 patients received immunotherapy as the first-line regimen at our clinic, and those patients exhibited longer overall survival compared to patients in reported keynote studies.
ABSTRACT
Background@#In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. @*Methods@#We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries.Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. @*Results@#The number of pediatric surgeries from 2002 to 2017 increased by 124%.Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. @*Conclusion@#The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.
ABSTRACT
Purpose@#Short bowel syndrome (SBS) is the most common etiology for intestinal failure (IF) and these patients are at high risk of developing micronutrient deficiencies. This study aimed at assessing the level of vitamins in adult SBS patients at different stages of their disease before the initiation of multidisciplinary intestinal rehabilitation. @*Methods@#Patient data from November 2015 to March 2017 were retrospectively reviewed. Adult patients who underwent extensive bowel resection and were classified as SBS-IF were selected. Clinical data including age, sex, etiology of IF, biochemical data, nutritional status, nutrition support, and outcome of intestinal rehabilitation were analyzed. @*Results@#Nine patients with SBS-IF were included in the analysis. There were 6 male patients and 3 female patients, with a median age of 55.0 years. Vitamin levels were analyzed at 306 days (median) after the development of SBS. At the time of vitamin levels screening, 4 patients were receiving daily intravenous vitamin supplementation. Five patients were not receiving vitamin supplementations, either intravenously or orally. Vitamin B12 was within the normal range in 6 patients and higher than normal in 3 patients. Vitamin D was within the normal range in 3 patients and lower than normal in 6 patients. Vitamin E was within the normal range in 7 patients and higher than normal in 2 patients. Folate was within the normal range in 8 patients (not checked in 1 patient). Ambulatory patients had significantly higher vitamin D levels compared to hospitalized patients (P=0.015). @*Conclusion@#Vitamin D levels had decreased in 67% of patients with SBS in Korea, while vitamin B12, folate, and vitamin E deficiencies were rarely seen.
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Objective@#We investigated prenatal sonographic characteristics of esophageal atresia (EA) with advancing gestation. We focused on the degree of polyhydramnios and the stomach shape. @*Methods@#This study included 27 EA cases (EA group) and 81 idiopathic polyhydramnios cases (non-EA group). The non-EA group consisted of cases without any fetal structural anomaly, musculoskeletal disorder, chromosomal abnormality, or maternal diabetes. Both groups included only singleton pregnancies. Amniotic fluid index (AFI) and width/length (W/L) ratio as well as the product of width and length (W×L) of stomach were serially assessed during gestation and compared between the 2 groups. To predict EA using W/L ratio and W×L, receiver operating characteristic curve analysis was performed. @*Results@#Polyhydramnios was evident in 77.8% of EA cases. We observed 25.9% and 22.2% EA cases with an absent stomach and a small visible stomach, respectively. After 28 weeks, the EA group manifested significantly higher AFI than the non-EA group. After 32 weeks, W/L ratio in the EA group tended to be lower than that in the non-EA group (32–36 weeks: 1.36 vs. 1.72, P=0.092; >36 weeks: 1.43 vs. 1.63, P=0.024). To predict EA, the calculated area under the curve for W/L ratio was 0.651 after 32 weeks. The diagnosis of EA using a cut-off value of W/L ratio <1.376 showed sensitivity, specificity, positive likelihood ratio, and negative likelihood ratio to be 84.6%, 52.9%, 1.796, and 0.081, respectively. @*Conclusion@#A low W/L ratio of stomach after 32 weeks with progressive idiopathic polyhydramnios may be used to predict EA.
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Injury of lower cranial nerves (CNs) by skull base fracture after head trauma can occur sometimes. However, selectively different CN damage on either side is extremely rare.A 53-year-old man had difficulty of swallowing, phonation, and articulation after falling off his bicycle. In physical examination, a deviated tongue to the right side was shown.Brain computed tomography showed a skull base fracture involving bilateral jugular foramina and right hypoglossal canal. Left vocal cord palsy was confirmed by laryngoscopy. Electromyography confirmed injury of left superior laryngeal nerve, recurrent laryngeal nerve, and right hypoglossal nerve. Video fluoroscopic swallowing study revealed large amounts of remnant in vallecula and pyriform sinus without opening of upper esophageal sphincter due to dysfunction of cricopharyngeus muscle. After constant rehabilitation for dysphagia, he was allowed to eat a general diet with compensatory techniques at discharge and further recovery after 3 months. Injury of lower CNs after fracture of the skull base can cause severe morbidity. However, the prognosis of such injuries can be favorable with early rehabilitation treatment by identifying the injured CN. A careful and accurate examination of lower CN injury in skull base fracture is essential for planning a treatment strategy.