Purpose: To report the findings of a perception survey on intestinal malrotation conducted by the Korean Association of Pediatric Surgeons (KAPS) in 2021. Methods: The perceptions on intestinal malrotation regarding clinical decision making of the KAPS members were collected through web-based survey. Results: A total of 22 surgeons were answered for this study. The results were presented and discussed at the 37th annual meeting of KAPS, which was held in Seoul on June 18, 2021. Conclusion This study provides the clinical decisions of the KAPS members on the intestinal malrotation. The study is expected to be an important reference for improving pediatric surgeons’ understanding and treatment of intestinal malrotation.

Purpose: This study aimed to stratify risk factors and vein levels for postoperative deep vein thrombosis (DVT) after lower-extremity orthopedic surgery. Methods: Ninety-nine patients who underwent Doppler ultrasonography after lower-extremity orthopedic surgery were enrolled. Medical records were reviewed for anesthesia duration, type of surgery, body weight, height, and cardiovascular risk factors (including history of smoking, diabetes mellitus or hypertension, blood pressure, and total cholesterol and high-density lipoprotein [HDL] cholesterol levels), and the DVT treatment. Ultrasound diagnosis of DVT was made according to a routine protocol. The relationships between selected factors and the presence of DVT were assessed using univariate and multivariate regression analyses. Results: Thirty-three (33%) patients were found to have calf DVT. The mean age, weight, and height of the non-DVT and postoperative DVT patients were 55.1 years versus 65.4 years, 70.5 kg versus 61.2 kg, and 163.3 cm versus 157.0 cm, respectively. Total cholesterol/HDL levels in the non-DVT and DVT patients were 70.6/20.7 mg/dL and 90.8/26.0 mg/dL, retrospectively. Systolic and diastolic blood pressure in the non-DVT and DVT patients were 133.6/80.2 mm Hg and 132.2/78.1 mmHg, respectively. The mean duration of anesthesia was 173.9 versus 199.9 minutes, and the operative time was 136.4 minutes versus 161.0 minutes. Older age (P=0.005) and lower body weight (P=0.002) were significantly associated with postoperative DVT. No other significant between-group differences were found (P>0.05). The patients with ultrasound-identified DVT received antithrombotic treatment. None of them had distant thromboembolism. Conclusion After lower-extremity orthopedic surgery, the calf veins in elderly patients with low body weight are susceptible to thrombosis; they would most likely benefit from postoperative ultrasonography.

Purpose: Our purpose in the current meta-analysis was to compare the functional outcomes in patients who have received single-radius (SR) or multi-radius (MR) femoral components in randomized controlled trials (RCTs) for primary total knee arthroplasty (TKA). The hypothesis was that there would be no statistically significant difference between two groups in terms of functional outcomes. Materials and methods: We searched the international electronic databases PubMed, Embase, and the Cochrane Central Register of Controlled Trials up to February 2020 for RCTs that compared functional outcomes of SR and MR femoral component designs after primary TKA. We performed a meta-analysis of nine RCTs using the Knee Society Score for the knee (KSS-knee), KSS-function, Knee Injury and Osteoarthritis Outcome Score (KOOS), Oxford Knee Score (OKS), degree of knee flexion, extension, and complications, including postoperative infection and revision surgery. Results: The meta-analysis revealed no statistically significant differences in all the analyzed variables, including KSSknee, KSS-function, KOOS, OKS, knee flexion, and knee extension. For postoperative complications, no statistically significant differences were detected for femoral component designs in postoperative infection or incidence of revision surgery between the two groups. Conclusions The current meta-analysis of RCTs did not show any statistically significant differences between SR and MR femoral component designs in terms of postoperative functional outcomes. Evaluated outcomes included functional outcome scores, degree of knee flexion, extension, and complications. However, because of the limited clinical evidence of this study owing to the heterogeneity between the included RCTs, a careful approach should be made in order not to arrive at definite conclusions.

Purpose: Our purpose in the current meta-analysis was to compare the functional outcomes in patients who have received single-radius (SR) or multi-radius (MR) femoral components in randomized controlled trials (RCTs) for primary total knee arthroplasty (TKA). The hypothesis was that there would be no statistically significant difference between two groups in terms of functional outcomes. Materials and methods: We searched the international electronic databases PubMed, Embase, and the Cochrane Central Register of Controlled Trials up to February 2020 for RCTs that compared functional outcomes of SR and MR femoral component designs after primary TKA. We performed a meta-analysis of nine RCTs using the Knee Society Score for the knee (KSS-knee), KSS-function, Knee Injury and Osteoarthritis Outcome Score (KOOS), Oxford Knee Score (OKS), degree of knee flexion, extension, and complications, including postoperative infection and revision surgery. Results: The meta-analysis revealed no statistically significant differences in all the analyzed variables, including KSSknee, KSS-function, KOOS, OKS, knee flexion, and knee extension. For postoperative complications, no statistically significant differences were detected for femoral component designs in postoperative infection or incidence of revision surgery between the two groups. Conclusions The current meta-analysis of RCTs did not show any statistically significant differences between SR and MR femoral component designs in terms of postoperative functional outcomes. Evaluated outcomes included functional outcome scores, degree of knee flexion, extension, and complications. However, because of the limited clinical evidence of this study owing to the heterogeneity between the included RCTs, a careful approach should be made in order not to arrive at definite conclusions.

BACKGROUND AND PURPOSE: 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome. Epilepsy and other neuropsychiatric (NP) manifestations of this genetic syndrome are not uncommon, but they are also not well-understood. We sought to identify the characteristics of epilepsy and other associated NP manifestations in patients with 22q11.2DS. METHODS: We retrospectively analyzed the medical records of 145 child and adolescent patients (72 males and 73 females) with genetically diagnosed 22q11.2DS. The clinical data included seizures, growth chart, psychological reports, development characteristics, school performance, other clinical manifestations, and laboratory findings. RESULTS: Of the 145 patients with 22q11.2DS, 22 (15.2%) had epileptic seizures, 15 (10.3%) had developmental delay, and 5 (3.4%) had a psychiatric illness. Twelve patients with epilepsy were classified as genetic epilepsy whereas the remaining were classified as structural, including three with malformations of cortical development. Patients with epilepsy were more likely to display developmental delay (odds ratio=3.98; 95% confidence interval=1.5-10.5; p=0.005), and developmental delay was more common in patients with structural epilepsy than in those with genetic epilepsy. CONCLUSIONS: Patients with 22q11.2DS have a high risk of epilepsy, which in these cases is closely related to other NP manifestations. This implies that this specific genetic locus is critically linked to neurodevelopment and epileptogenesis.
Adolescent* ; Child* ; DiGeorge Syndrome* ; Epilepsy* ; Genetic Loci ; Growth Charts ; Humans ; Male ; Malformations of Cortical Development ; Medical Records ; Mental Disorders ; Neurologic Manifestations ; Retrospective Studies ; Seizures

PURPOSE: Noonan syndrome (NS) is characterized by short stature, heart anomalies, developmental delays, dysmorphic features, cryptorchidism, and coagulation defects. Several studies reported the short-term effects of recombinant human growth hormone (rhGH) treatment on the improvement of height. This study was performed to evaluate the long-term efficacy of rhGH in children with NS in Korea. METHODS: This study included 15 prepubertal NS children who received rhGH subcutaneously at a dose of 50-75 µg/kg/day for 6 days a week for at least >3 years. Preand posttreatment data, such as height, weight, bone age, insulin-like growth factor 1 (IGF-1), and IGF binding protein 3 (IGFBP-3) levels, were collected every 6 months. RESULTS: Chronologic age and bone age at the start of treatment were 7.97±1.81 and 5.09±2.12 years, respectively. Height standard deviation score (SDS) was increased from -2.64±0.64 to -1.54±1.24 years after 3 years (P<0.001). Serum IGF-1 SDS levels were elevated from -1.28±1.03 to -0.10±0.94 (P<0.001). Height SDS was more increased in subjects without PTPN11 mutations compared to those with mutations after 3 years (P=0.012). However, the other parameters, including bone age, IGF-1 SDS, and IGFBP-3 SDS, were not significantly different between patients with and without PTPN11 mutations. CONCLUSION: Although this study included a relatively small number of patients, long-term rhGH therapy in NS patients was safe and effective at improving height, growth velocity, and serum IGF-1 levels, in accordance with previous studies. However, the meticulous monitoring of potential adverse events is still needed because of high dose of rhGH and preexisting hyperactivity of RAS-MAPK pathway. Patients with PTPN11 mutations demonstrated a decreased response to rhGH therapy compared to those without mutations.
Child ; Cryptorchidism ; Growth Hormone ; Heart ; Human Growth Hormone* ; Humans* ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Korea ; Male ; Noonan Syndrome*

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Autosomal recessive polycystic kidney disease is among the most common inherited ciliopathies and is caused by mutations in the polycystic kidney and hepatic disease 1 (PKHD1) gene. Despite its great phenotypic variability, this condition is usually diagnosed during the neonatal and early infantile periods. We report a 37+3 -gestational-week neonate presenting with fatal autosomal recessive polycystic kidney disease who died at 28 hours of life from severe respiratory failure. The familial history is significant because a previous sibling died in utero at 24+2 weeks of gestational age and was diagnosed with polycystic kidney disease based on prenatal ultrasonography and autopsy. Our patient's autopsy revealed findings compatible with polycystic kidney disease. In addition, a PKHD1 gene study of peripheral blood leukocytes identified the compound heterozygote mutation c.274C>T(p.Arg92Trp), as well as the novel heterozygous nonsense mutation c.2770C>T(p.Gln924*).
Autopsy ; Codon, Nonsense ; Gestational Age ; Heterozygote ; Humans ; Infant, Newborn* ; Leukocytes ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Recessive* ; Respiratory Insufficiency ; Siblings ; Ultrasonography, Prenatal

OBJECTIVES: This study was conducted to propose the need of re-establishing the criteria of the body weight classification in the elderly. We compared the Asia-Pacific Region Criteria (APR-C) with Entropy Model Criteria (ENT-C) using Morbidity rate of chronic diseases which correlates significantly with Body Mass Index (BMI). METHODS: Subjects were 886 elderly female participating in the 2007-2009 Korea National Health and Nutrition Examination Survey (KNHANES). We compared APR-C with those of ENT-C using Receiver Operating Characteristics (ROC) curve and logistic regression analysis. RESULTS: In the case of the morbidity of hypertension, the results were as follows: Where it was in the T-off point of APR-C, sensitivity was 67.5%, specificity was 43.1%, and Youden's index was 10.6. While in the cut-off point of ENT-C, it was 56.7%, 56.6%, and 13.3 respectively. In the case of the morbidity of diabetes, the results were as follows: In the cut-off point of APR-C, Youden's index was 14.2. While in the cut-off point of ENT-C, it was 17.2 respectively. The Area Under the ROC Curve (AUC) of the subjects who had more than 2 diseases among hypertension, diabetes, and dyslipidemia was 0.615 (95% CI: 0.578-0.652). Compared to the normal group, the odds ratio of the hypertension group which will belong to the overweight or obesity was 1.79 (95% CI: 1.30-2.47) in the APR-C, and 2.04 (95% CI: 1.49-2.80) in the ENT-C (p > 0.001). CONCLUSIONS: We conclude that the optimal cut-off point of BMI to distinguish between normal weight and overweight was 24 kg/m2 (ENT-C) rather than 23 kg/m2 (APR-C).
Aged* ; Body Mass Index* ; Body Weight ; Chronic Disease* ; Classification ; Dyslipidemias ; Entropy* ; Female ; Humans ; Hypertension ; Korea ; Logistic Models ; Nutrition Surveys ; Obesity ; Odds Ratio ; Overweight ; ROC Curve ; Sensitivity and Specificity

PURPOSE: We evaluated the effect of early chemoradiotherapy on the treatment of patients with limited stage small cell lung cancer (LS-SCLC). MATERIALS AND METHODS: Between January 2006 and December 2011, thirty-one patients with histologically proven LS-SCLC who were treated with two cycles of chemotherapy followed by concurrent chemoradiotherapy and consolidation chemotherapy were retrospectively analyzed. The chemotherapy regimen was composed of etoposide and cisplatin. Thoracic radiotherapy consisted of 50 to 60 Gy (median, 54 Gy) given in 5 to 6.5 weeks. RESULTS: The follow-up period ranged from 5 to 53 months (median, 22 months). After chemoradiotherapy, 35.5% of the patients (11 patients) showed complete response, 61.3% (19 patients) showed partial response, 3.2% (one patient) showed progressive disease, resulting in an overall response rate of 96.8% (30 patients). The 1-, 2-, and 3-year overall survival (OS) rates were 66.5%, 41.0%, and 28.1%, respectively, with a median OS of 21.3 months. The 1-, 2-, and 3-year progression free survival (PFS) rates were 49.8%, 22.8%, and 13.7%, respectively, with median PFS of 12 months. The patterns of failure were: locoregional recurrences in 29.0% (nine patients), distant metastasis in 9.7% (three patients), and both locoregional and distant metastasis in 9.7% (three patients). Grade 3 or 4 toxicities of leukopenia, anemia, and thrombocytopenia were observed in 32.2%, 29.0%, and 25.8%, respectively. Grade 3 radiation esophagitis and radiation pneumonitis were shown in 12.9% and 6.4%, respectively. CONCLUSION: We conclude that early chemoradiotherapy for LS-SCLC provides feasible and acceptable local control and safety.
Anemia ; Chemoradiotherapy* ; Cisplatin ; Consolidation Chemotherapy ; Disease-Free Survival ; Drug Therapy ; Esophagitis ; Etoposide ; Follow-Up Studies ; Humans ; Leukopenia ; Neoplasm Metastasis ; Radiation Pneumonitis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Small Cell Lung Carcinoma* ; Thrombocytopenia