PURPOSE: The aim of this study is to compare the clinical results and radiologic changes of closing-wedge high tibial osteotomy (CW HTO) and opening-wedge high tibial osteotomy (OW HTO). MATERIALS AND METHODS: Seventy five knees were treated with a CW HTO and 41 with an OW HTO. For each patient the hip-knee-ankle (H-K-A) axis was evaluated and the degree of the medial compartmental arthrosis was measured by Ahlback-type radiological classification. The function of the knee was evaluated by the Tegner activity score, the Lysholm knee scoring scale and the Western Ontario and McMaster University index. Lateral radiographs were taken to assess the patellar height and the posterior tibial inclination. RESULTS: In both groups significant improvement of the visual analogue scale and range of motion was achieved. The frontal plane H-K-A axis was corrected significantly from varus to the range of physiological valgus and the arthrosis of the medial compartment of the knee progressed gradually. The body mass index was significantly influential to the progression of arthrosis. The functions of the knee were improved significantly in all cases. In the closing-wedge group, the patella height was increased at the postoperative period, while it was decreased in the opening-wedge group. There was a tendency of a decrease of the tibial inclination in the CW HTO group and a statistically significant increase of the tibial inclination in the OW HTO group. Recurrence of varus occurred in sixteen cases. CONCLUSION: In both groups, improvement of the function of the knee was achieved, but there was no statistical difference. However, the opposite result was found in the patella height and the tibial inclination.
Axis, Cervical Vertebra ; Body Mass Index ; Humans ; Knee ; Ontario ; Osteotomy ; Patella ; Postoperative Period ; Range of Motion, Articular ; Recurrence

Fetus-in-fetu (FIF) is a rare congenital condition in which a fetiform mass is detected in the host abdomen and also in other sites such as the intracranium, thorax, head, and neck. This condition has been rarely reported in the literature. Herein, we report the case of a fetus presenting with abdominal cystic mass and ascites and prenatally diagnosed as meconium pseudocyst. Explorative laparotomy revealed an irregular fetiform mass in the retroperitoneum within a fluid-filled cyst. The mass contained intestinal tract, liver, pancreas, and finger. Fetal abdominal cystic mass has been identified in a broad spectrum of diseases. However, as in our case, FIF is often overlooked during differential diagnosis. FIF should also be differentiated from other conditions associated with fetal abdominal masses.
Abdomen ; Ascites ; Diagnosis, Differential ; Diagnostic Errors ; Fetus ; Fingers ; Head ; Laparotomy ; Liver ; Meconium ; Neck ; Pancreas ; Peritonitis ; Thorax

PURPOSE: The aim of this study is to compare the characteristics of tumor and prognosis, depending on the status of resection margin involvement, on the frozen section diagnosis in gastric cancer. MATERIALS AND METHODS: This study was conducted retrospectively, in 83 margin-positive patients on the frozen section diagnosis, who underwent gastrectomy from July 1995 to September 2006. The control group was selected by matching the age, gender, TNM stage and status of adjuvant chemoradiotherapy, among those who had shown clear resection margins. The characteristics of tumor and patient survival are investigated, and they were analyzed between the two groups. RESULTS: The tumor size was significantly larger in the study group than that of the control group (P=0.037). There was significant difference between the two groups in location of the tumors (P=0.003). Multivariate analysis indicated that only the location and Lauren's classification are independent factors, which affected the resection margin involvement. Median survival was 41.0+/-11.5 months in the study group and 93.0+/-30.3 months in the control group (P=0.049). In the survival analysis, it was investigated that TNM stage and the resection margin involvement of the frozen section diagnosis were the critical variables. CONCLUSIONS: When the tumor is located at the middle or the upper third, or the Lauren's indeterminate type, they are highly likely to show the resection margin involvement on the frozen section diagnosis, and it can, therefore, have negative effects on the prognosis. It is considered as good to perform more extensive resection as possible, during the initial resection.
Chemoradiotherapy, Adjuvant ; Frozen Sections ; Gastrectomy ; Humans ; Multivariate Analysis ; Prognosis ; Retrospective Studies ; Stomach Neoplasms

PURPOSE: Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is rare lysosomal storage disorder caused by N-acetylgalactosamine-6-sulfatase (GALNS) deficiency. Only a few MPS IVA cases have been reported in the Korean literature; there is a paucity of research about clinical or radiologic findings for this disorder. Therefore, we studied clinical findings, radiological features, and genetic data of Korean MPS IVA patients for determining factors that may allow early diagnosis and that may thus improve the patients' quality of life. METHODS: MPS IVA was confirmed via assay for enzymatic activity of leukocytes in 10 patients. The GALNS gene was analyzed. Patients' charts were retrospectively reviewed for obtaining clinical features and evaluated for radiological skeletal surveys, echocardiography, pulmonary function test, and ophthalmologic test results. RESULTS: Nine patients had severe clinical phenotype, and 1 had an intermediate phenotype, on the basis of clinical phenotype criteria. Radiologic findings indicated skeletal abnormalities in all patients, especially in the hips and extremities. Eight patients had an odontoid hypoplasia, and 1 showed mild atlantoaxial subluxation and cord myelopathy. Genetic analysis indicated 10 different GALNS mutations. Two mutations, c.451C>A and c.1000C>T, account for 37.5% (6/16) and 25% (4/16) of all mutations in this samples, respectively. CONCLUSION: An understanding of the clinical and radiological features involved in MPS IVA may allow early diagnosis of MPS IVA. Adequate evaluations and therapy in the early stages may improve the quality of life of patients suffering from skeletal abnormalities and may reduce life-threatening effects of atlantoaxial subluxation.
Early Diagnosis ; Echocardiography ; Extremities ; Hip ; Humans ; Leukocytes ; Mucopolysaccharidoses ; Mucopolysaccharidosis IV ; Phenotype ; Quality of Life ; Respiratory Function Tests ; Retrospective Studies ; Spinal Cord Diseases ; Stress, Psychological

This erratum is being published to correct of page 435 and Table 4.

OBJECTIVE: Small bowel series (SBS) may be a useful test in identifying the causes of feeding intolerance in preterm infants. Unfortunately, little is known about the safety and effectiveness of SBS in preterm infants. By scrutinizing our recent experiences in performing SBS in preterm infants, we aim to provide basic data concerning the use of SBS in preterm infants with feeding intolerance. METHODS: Between January 2005 and October 2008, SBS was done in 46 preterm infants with feeding intolerance, who were admitted to the neonatal intensive care unit (NICU) of Seoul national university children's hospital. We conducted a retrospective analysis of these preterm infants. RESULTS: The average gestational age and birth weight of the 46 infants were, 30(+1)+/-3(+5) weeks, 1,435+/-623 g, respectively. The mean postconceptional age when SBS was done, was 37(+2)+/-4(+2) weeks, 46.8+/-27.3days. SBS proved that 29 infants did not have mechanical obstructions and 22 of these infants had delayed bowel transit time. Eventually, 35 infants attained full feeding except those who underwent operation. SBS was done at their bedside in 15 out of 27 infants in the group less than 1.5 kg. History of NEC, meconium related ileus, and gastrointestinal operation were more frequent in the group less than 1.5 kg. The adverse effects of SBS in preterm infants included vomiting, delayed transit of the contrast media, all of which resolved in the end and there were no reports of aspiration or sepsis. CONCLUSION: Actively performing SBS test in preterm infants with feeding intolerance will promote feeding advancement by discovering the cause of feeding intolerance and subsequent management of the specific problem.
Birth Weight ; Contrast Media ; Gestational Age ; Humans ; Ileus ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Meconium ; Retrospective Studies ; Sepsis ; Vomiting

PURPOSE: Lung injury from mechanical ventilation is one of the major pathogenetic factors of bronchopulmonary dysplasia. Permissive hypercapnia (PH) is one of the strategies for reducing lung injury. However, PH is frequently infeasible in very low birth weight infants (VLBWI) due to their immature renal compensation for respiratory acidosis. The purpose of this study was to identify time when metabolic compensation for hypercapnia begin to occur in VLBWIs. METHODS: Data were retrospectively collected from 82 VLBWI who were admitted to Seoul National University Bundang Hospital from January 2011 to December 2012. The postnatal day when the difference between actual bicarbonate and expected bicarbonate levels became less than 2.0 mmol/L consistently for the first time under hypercapnea (>40 mmHg) was defined as the time when metabolic compensation for hypercapnea occurred. RESULTS: Metabolic compensation for hypercapnea occurred on 9.1+/-3.9 postnatal day. The younger the gestational age (GA) was and the smaller the birth weight was, the later metabolic compensation for hypercapnea occurred. Late metabolic compensators (> or =9 days) were significantly younger in GA (P=0.001), lighter at birth (P=0.041), intubated longer (P=0.002), and less frequently afflicted with respiratory distress syndrome (P=0.036) compared to early metabolic compensators (<9 days). However, logistic regression analysis revealed only young GA was associated with late metabolic compensation with marginal significance (P=0.068). CONCLUSION: Metabolic compensation for hypercapnea occurred 9 days after birth on average. PH strategy for reducing lung injury should be considered after renal metabolic compensation for hypercapnea occurs in VLBWI.
Acidosis, Respiratory ; Birth Weight ; Bronchopulmonary Dysplasia ; Compensation and Redress ; Gestational Age ; Humans ; Hydrogen-Ion Concentration ; Hypercapnia ; Infant ; Infant, Newborn ; Infant, Very Low Birth Weight ; Logistic Models ; Lung Injury ; Parturition ; Respiration, Artificial ; Retrospective Studies

BACKGROUND AND OBJECTIVES: We assessed the ability of portable echocardiography (with contrasts) to clearly delineate the cardiac structure, and evaluated the impact of its use on the diagnosis and management of critically ill patients in Korea. SUBJECTS AND METHODS: We prospectively enrolled 123 patients (mean age 66+/-16 years), who underwent portable transthoracic echocardiography (with contrast) for image enhancement at 12 medical centers. The quality of the global left ventricular (LV) images, the number of the regional LV segments visualized, the ability to visualize the LV apex and the right ventricle (RV), and any changes in the diagnostic procedure and treatment strategy were compared before and after the contrast. RESULTS: Of the 123 patients, 52 (42%) were using mechanical ventilators. The amount of poor or uninterpretable images decreased from 48% to 5% (p<0.001), after the contrast. Before the contrast, 15.6+/-1.1 of 16 LV segments were seen, which improved to 15.9+/-0.6 segments (p=0.001) after the contrast. The ability to visualize the LV apex increased from 47% to 94% (p<0.001), while the inability to clearly visualize the RV decreased from 46% to 19% (p<0.001). Changes in the diagnostic procedure (for example, not requiring other types of imaging studies) were observed in 18% of the patients, and the treatment plan (medication) was altered in 26% of patients after the contrast echocardiography. CONCLUSION: The use of a contrast agent during the portable echocardiography, in intensive care settings, can improve the image quality and impact the diagnostic procedures and treatment for Korean patients.
Critical Illness ; Diagnosis ; Echocardiography* ; Heart Ventricles ; Humans ; Image Enhancement ; Critical Care* ; Korea ; Prospective Studies ; Ventilators, Mechanical

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.
Enzyme Assays ; Fibroblasts ; Humans ; Iduronate Sulfatase ; Leukocytes ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Phenotype ; Plasma ; Sensitivity and Specificity ; Skin

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging from mild to severe. IDS activity, which is measured in leukocyte pellets or fibroblasts, was reported to be related to clinical phenotype by Sukegawa-Hayasaka et al. Measurement of residual plasma IDS activity using a fluorometric assay is simpler than conventional measurements using skin fibroblasts or peripheral blood mononuclear cells. This is the first study to describe the relationship between plasma IDS activity and clinical phenotype of MPS II. METHODS: We hypothesized that residual plasma IDS activity is related to clinical phenotype. We classified 43 Hunter syndrome patients as having attenuated or severe disease types based on clinical characteristics, especially intellectual and cognitive status. There were 27 patients with the severe type and 16 with the attenuated type. Plasma IDS activity was measured by a fluorometric enzyme assay using 4-methylumbelliferyl-alpha-iduronate 2-sulphate. RESULTS: Plasma IDS activity in patients with the severe type was significantly lower than that in patients with the attenuated type (P=0.006). The optimal cut-off value of plasma IDS activity for distinguishing the severe type from the attenuated type was 0.63 nmol.4 hr-1.mL-1. This value had 88.2% sensitivity, 65.4% specificity, and an area under receiver-operator characteristics (ROC) curve of 0.768 (ROC curve analysis; P=0.003). CONCLUSION: These results show that the mild phenotype may be related to residual lysosomal enzyme activity.
Enzyme Assays ; Fibroblasts ; Humans ; Iduronate Sulfatase ; Leukocytes ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Phenotype ; Plasma ; Sensitivity and Specificity ; Skin