No abstract available.
Anemia*

Heterotopic salivary gland tissue (HSGT) consists of salivary tissue in places where it does not normally exist. Usually, HSGT presents as a draining sinus or nodule of the neck. The pathogenesis of HSGT of the neck remains uncertain. But HSGT in the neck is most widely thought to arise from defective closures and ectodermal hetroplasia of the precervical sinus of His. HSGT in the neck is rare and only a few cases have been reported in Korea. We recently experienced a case of HSGT in the neck presenting as a fistula of the anterior lower neck. We thought this as a 2nd brachial cleft anomaly; however, biopsy revealed HSGT. HSGT in the neck should be considered as one of the causes of a neck mass or fistula in children. Herein, we report this case of HSGT with a literature review.

Right aortic arch with isolation of the left subclavian artery is a rare anomaly. The incidence of bilateral ductus arteriosus is sporadic, and a right aortic arch with isolation of the left subclavian artery in association with bilateral ductus arteriosus is therefore extremely rare. Since the symptoms and signs of isolation of the left subclavian artery can include the absence or underdevelopment of the left arm, subclavian steal syndrome, or pulmonary artery steal syndrome, the proper therapeutic approach is controversial. We report a case in which surgical reconstruction was used to treat isolation of the left subclavian artery with right aortic arch in association with bilateral ductus arteriosus and a ventricular septal defect.
Aorta, Thoracic* ; Arm ; Ductus Arteriosus* ; Embryology ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular* ; Incidence ; Pulmonary Artery ; Subclavian Artery* ; Subclavian Steal Syndrome

Hereditary fructose intolerance (HFI) is an autosomal recessive disorder caused by a mutation in the aldolase B gene. HFI patients exhibit nausea, vomiting, abdominal pain, hypoglycemia, and elevated liver enzymes after dietary fructose exposure. Chronic exposure might lead to failure to thrive, liver failure, renal failure, and, eventually, death. HFI usually manifests in infants when they are being weaned off of breastmilk. Because HFI has an excellent prognosis when patients maintain a strict restrictive diet, some patients remain undiagnosed due to the voluntary avoidance of sweet foods. In the past, HFI was diagnosed using a fructose tolerance test, liver enzyme assays or intestinal biopsy specimens. Currently, HFI is diagnosed through the analysis of aldolase B mutations. Here, HFI was diagnosed in a 41-year-old woman who complained of sweating, nausea, and vomiting after consuming sweets. She had a compound heterozygous mutation in the aldolase B gene; gene analysis revealed pathogenic nonsense (c.178C>T, p.Arg60Ter) and frameshift (c.360_363delCAAA, p.Asn120LysfsTer32) variants. This is the first report of a Korean HFI patient diagnosed in adulthood.

Purpose: To assess the effectiveness of mixed grafts in lumbar posterolateral fusion (PLF) by comparing the bone union rates of an autobone with a bone substitute mixed graft. Materials and Methods: The patients were followed-up for at least two years after PLF and divided into four groups according to the mixed graft retrospectively. Group I was 48 cases using a femoral head allobone. Group II was 38 cases using b-tricalcium phosphate. Group III was 92 cases using biphasic calcium phosphate. Group IV was 38 cases using biphasic calcium phosphate and autologous bone marrow. Union was evaluated by the work up simple radiographs after two years from PLF. Union was defined if the radiographs demonstrated a bilateral continuity in the fusion mass between the cephalad and caudal transverse processes with less than 2° of angular motion and no translation between the vertebrae at the level of fusion on the lateral flexion-extension radiographs. Results: According to simple radiographs after two years from PLF, the rate of union was highest in Group IV using local autobone, biphasic calcium phosphate and autologous bone marrow mixed graft. Conclusion Biphasic calcium phosphate is an osteoconductive bone substitute that increases the bio-absorbability and mechanical strength. Autologous bone marrow has osteoinductive and osteogenic properties. These features can increase the rate of bone union. Therefore, a local autobone, biphasic calcium phosphate and autologous bone marrow mixed graft can be considered an effective bone graft substitute for lumbar PLF instead of an autobone graft.

OBJECTIVES: It is important to consider the symptoms of diabetic neuropathy in determining a diagnostic approach. To evaluate diabetic neuropathy, an appropriate questionnaire for Korean diabetic patients is required. In 1994, Feldman et al. proposed the two-step diagnostic approach which included a questionnaire. To adapt a diagnostic approach and questionnaire for Korean diabetics, we initially diagnosed diabetic neuropathy by using Feldman's method and by assessing the efficiency of their method for Korean diabetics as a preliminary study. METHODS: We diagnosed diabetic neuropathy with Feldman's method in 99 NIDDM patients who visited Ajou university hospital from October 1, 1996 to April 30, 1997. The sensitivity and specificity of Feldman's 4 diagnostic procedures were ebaluated. To include the highly specific symptoms in the diagnostic approach, we applied a different scoring system(from 5 to 1) to the questionnaire accroding to specificity and then evaluated the sensitivity and specificity with this new system. If the sensitivity and specificity were 60% or more, the new scoring system was regarded as clinically useful. RESULTS: Using Feldman's method, the sensitivity of MNSIQ and MNSIC was 12.8% and the specificity was 88.3% and 98.3%, respectively. The sensitivity of MDNS and NCV was 100% and the specificity was 68.3% and 61.6%, respectively. The new scoring system consisted of 13 of 15 questions with sensitivity of 46.2% and specificity of 71.7%, omitting 2 questions due to possible relation to vascular symptoms. Among the 4 diagnostic procedures, the strongest correlation existed between MDNS and NCV. None of the procedures had a significant correlatiion with MNSIQ. MNSIC, which shares similar characteristics with MDNS, had significant correlation with MDNS and NCV, but its sensitivity was very low. CONCLUSON: MNSIQ and MNSIC were not useful as screening instruments in the diagnostic approach to diabetic neuropathy using Feldman's method. Thus, a new questionnaire composed of symptoms common in Korean diabetic patients should be designed, and MNSIC could be omitted from the diagnostic approach.
Diabetes Mellitus, Type 2 ; Diabetic Neuropathies* ; Diagnosis ; Humans ; Mass Screening ; Sensitivity and Specificity ; Surveys and Questionnaires

Purpose: Radiofrequency ablation is a curative treatment option for very early-stage or earlystage hepatocellular carcinoma (HCC). However, percutaneous radiofrequency ablation (PRFA) for subphrenic tumors is technically challenging. Laparoscopic radiofrequency ablation (LRFA) has been used to overcome this disadvantage. This study compared the treatment outcomes between LRFA and PRFA for subphrenic HCC. Methods: This retrospective study screened patients who underwent PRFA or LRFA for subphrenic HCC between 2013 and 2018. Therapeutic outcomes, including local tumor progression (LTP), intrahepatic distant recurrence (IDR), extrahepatic metastasis (EM), disease-free survival (DFS), and overall survival (OS), were compared between the two groups. Results: Thirty patients in the PRFA group and 23 patients in the LRFA group were included. LTP was observed in six patients in the PRFA group (20%), but in no patients in the LRFA group. The cumulative LTP rates at 1, 3, and 5 years were 3.7%, 23.4%, and 23.4%, respectively, in the PRFA group and 0.0% in the LRFA group (P=0.015). The IDR, EM, and DFS rates were not significantly different between the two groups (P=0.304, P=0.175, and P=0.075, respectively). The OS rates at 1, 3, and 5 years were 96.6%, 85.7%, and 71.6%, respectively, in the PRFA group and 100%, 95.7%, and 95.7%, respectively, in the LRFA group (P=0.049). Conclusion LRFA demonstrated better therapeutic outcomes than did PRFA for subphrenic tumors in terms of LTP and OS. Therefore, LRFA can be considered as the first-line treatment option for subphrenic HCC.

Amyloidosis is defined by tissue deposits of amyloid, which cause both functional and structural damage to organs. Three histopathological types of pulmonary amyloidosis have been described: tracheobronchial, nodular parenchymal, and diffuse parenchymal or alveolar septal types. Diffuse alveolar septal involvement of the lung is a rare form of primary pulmonary amyloidosis, with radiological features similar to those of pulmonary edema, pulmonary fibrosis, and lymphangitic carcinomatosis. Therefore, it is always challenging to diagnose alveolar septal involvement of pulmonary amyloidosis. Here, we report a case of alveolar septal involvement of pulmonary amyloidosis mimicking lymphangitic carcinomatosis, which was diagnosed from a transbronchial lung biopsy.
Amyloid ; Amyloidosis* ; Biopsy ; Carcinoma* ; Lung* ; Lymphatic Metastasis ; Multiple Myeloma ; Pulmonary Edema ; Pulmonary Fibrosis

OBJECTIVE: The purpose of this study was to determine whether funisitis is associated with changes in the umbilical cord plasma concentration of interleukin-6 (IL-6) and matrix metalloproteinase-8 (MMP-8), microbial invasion of the amniotic cavity and neonatal outcome. METHODS: The relationship among the presence of funisitis, IL-6 and MMP-8 concentrations in umbilical cord plasma at birth, the results of amniotic fluid culture performed within 5 days of birth was examined in 83 consecutive singleton births (20-35 weeks' gestation). Funisitis was diagnosed in the presence of neutrophil infiltration into the umbilical vessel wall or Wharton's jelly. The IL-6 and MMP-8 concentration was measured with a specific immunoassay. Amniocentesis was performed in 47 patients within 5 days of birth. RESULTS: (1) Funisitis was present in 21.7% of patients. (2) Patients with funisitis had a significant higher cord plasma IL-6 concentration, but had no significant difference in cord plasma MMP-8 concentration. (3) Clinical chorioamnionitis was more common in patients with funisitis than those without funisitis. (4) A cord plasma IL-6 > 6.34 pg/ml had a sensitivity of 77.8% and a specificity of 75.4% in the identification of funisitis. (5) No correlation between cord blood plasma IL-6 concentration and MMP-8 concentration was found. (6) There was no significant correlation between gestational age at birth and cord blood plasma MMP-8 concentrations, but there appeared to be a trend to increase of cord plasma MMP-8 concentrations as gestational ages at birth were increased. (7) Neonates with congenital sepsis had a significantly higher cord plasma IL-6 and MMP-8 concentration than those without congenital sepsis. CONCLUSION: In patient with funisitis, umbilical cord plasma IL-6 concentrations were higher than those without funisitis, but umbilical cord plasma MMP-8 concentrations had no significant difference in each group. The umbilical cord plasma IL-6 and MMP-8 can be useful as a predictor of the occurrence of congenital sepsis in preterm infant.
Amniocentesis ; Amniotic Fluid* ; Chorioamnionitis* ; Female ; Fetal Blood ; Gestational Age ; Humans ; Immunoassay ; Infant, Newborn ; Infant, Premature ; Interleukin-6* ; Matrix Metalloproteinase 8* ; Neutrophil Infiltration ; Parturition ; Plasma* ; Pregnancy ; Sensitivity and Specificity ; Sepsis ; Umbilical Cord* ; Wharton Jelly

College of Medicine, Ulsan University, Seoul, Korea The vascular rings comprise less than 1% of congenital cardiovascular malformations and can be overlooked because they are rare diseases and show nonspecific respiratory symptoms such as wheezing or airway obstruction or apnea. This case is a 2-months old infant who had been repaired patch closure due to ventricular septal defect, but failed to wean from ventilator. Chest CT showed right main stem bronchus lying between right pulmonary arery and right descending aorta, bronchoscopy showed right main bronchus was compressed by extrinsic pulsating mass which were descending aorta and Kommerell's diverticulum . After aortopexy and transfer of subclavian artery, this patient was able to be extubated and is now free of respiratory symptoms. When there is failure to weaning of ventilator and persistent bronchial obstruction and emphysema, we should have more vigorous effort to seek the cause of bronchial obstruction, using bronchoscope and chest CT.
Airway Obstruction ; Aorta, Thoracic* ; Apnea ; Bronchi ; Bronchoscopes ; Bronchoscopy ; Deception ; Diverticulum* ; Emphysema ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Korea ; Rare Diseases ; Respiratory Sounds ; Seoul ; Subclavian Artery ; Tomography, X-Ray Computed ; Ulsan ; Ventilators, Mechanical ; Weaning