Replacement of the retrohepatic inferior vena cava (IVC) after concurrent resection of IVC and tumor-bearing liver is regarded as a feasible living donor liver transplantation (LDLT) technique to cope with tumors around the IVC. This method can make the resection extent of LDLT comparable to that of deceased-donor liver transplantation. We present one pediatric LDLT case with IVC replacement using an enlarged iliac vein conduit to treat advanced hepatoblastoma. The patient was a 33-monthold and 12 kg-weighing girl suffering from large multiple hepatoblastomas invading the retrohepatic IVC. At 2-month waiting after deciding LDLT, we obtained a coldstored iliac vein graft and LDLT was performed with the father’s left lateral section graft. A 1.3 cm-wide and 10 cm-long iliac vein was transformed to be a 2 cm-wide and 5 cm-long vein graft through a double-barrel unification venoplasty. The left lateral section graft was implanted along the standard procedure of LDLT. The patient recovered uneventfully and is undergoing scheduled adjuvant chemotherapy. IVC replacement with vein homograft is a feasible option for LDLT in pediatric patients with advanced liver malignancy.

Purpose: To compare the efficacy and safety of radiofrequency ablation (RFA) and ethanol ablation (EA) followed by RFA in treating mixed cystic and solid thyroid nodules. Materials and Methods: We included 243 nodules from 243 patients who underwent RFA for mixed cystic and solid benign nodules. The nodules were divided into two groups (RFA alone and EA + RFA). We evaluated volume reduction rate (VRR), therapeutic success rate, improvement in symptomatic and cosmetic issues, complications, and adverse effects. Results: The RFA group included 204 patients, and the EA + RFA group included 39 patients.The long-term success rates in the RFA only and EA + RFA groups were 90.2% and 97.4%, respectively. The mean VRR at the last follow-up in the RFA and EA + RFA groups were 81.6% and 87.2%, respectively. Therapeutic results were similar in both groups at the last followup. Cosmetic and symptomatic problems markedly improved in both groups. No major complications were observed. Conclusion Both RFA alone and EA + RA are safe and effective methods for treating mixed cystic and solid thyroid nodules, although EA + RFA is slightly more effective.

Purpose: To compare the efficacy and safety of radiofrequency ablation (RFA) and ethanol ablation (EA) followed by RFA in treating mixed cystic and solid thyroid nodules. Materials and Methods: We included 243 nodules from 243 patients who underwent RFA for mixed cystic and solid benign nodules. The nodules were divided into two groups (RFA alone and EA + RFA). We evaluated volume reduction rate (VRR), therapeutic success rate, improvement in symptomatic and cosmetic issues, complications, and adverse effects. Results: The RFA group included 204 patients, and the EA + RFA group included 39 patients.The long-term success rates in the RFA only and EA + RFA groups were 90.2% and 97.4%, respectively. The mean VRR at the last follow-up in the RFA and EA + RFA groups were 81.6% and 87.2%, respectively. Therapeutic results were similar in both groups at the last followup. Cosmetic and symptomatic problems markedly improved in both groups. No major complications were observed. Conclusion Both RFA alone and EA + RA are safe and effective methods for treating mixed cystic and solid thyroid nodules, although EA + RFA is slightly more effective.

Purpose: To compare the efficacy and safety of radiofrequency ablation (RFA) and ethanol ablation (EA) followed by RFA in treating mixed cystic and solid thyroid nodules. Materials and Methods: We included 243 nodules from 243 patients who underwent RFA for mixed cystic and solid benign nodules. The nodules were divided into two groups (RFA alone and EA + RFA). We evaluated volume reduction rate (VRR), therapeutic success rate, improvement in symptomatic and cosmetic issues, complications, and adverse effects. Results: The RFA group included 204 patients, and the EA + RFA group included 39 patients.The long-term success rates in the RFA only and EA + RFA groups were 90.2% and 97.4%, respectively. The mean VRR at the last follow-up in the RFA and EA + RFA groups were 81.6% and 87.2%, respectively. Therapeutic results were similar in both groups at the last followup. Cosmetic and symptomatic problems markedly improved in both groups. No major complications were observed. Conclusion Both RFA alone and EA + RA are safe and effective methods for treating mixed cystic and solid thyroid nodules, although EA + RFA is slightly more effective.

Colonic interposition is the main procedure used in esophageal reconstruction. We report a rare case of simultaneous treatment of an anastomotic site stricture and a neoplasm in the interpositioned colon. A 69-year-old female visited our outpatient clinic with symptoms of progressive dysphagia for 1 year. At the age of 30 years, the patient underwent esophagectomy with retrosternal colonic interposition because of severe esophageal burns after chemical ingestion. Upper gastrointestinal endoscopy revealed stricture at the anastomosis site and a 10-mm flat elevated high-grade dysplasia in the interpositioned colon. First, through-the-scope balloon dilatation was performed for strictures. However, stenosis was observed during the second upper gastrointestinal endoscopy session.Therefore, a second session of through-the-scope balloon dilatation was performed, and simultaneously, endoscopic submucosal dissection was also successfully performed. After 2 months of follow-up, stenosis persisted; consequently, balloon dilatation was performed. No recurrence of neoplasm was confirmed endoscopically. Through-the-scope balloon dilatation of the stricture site and simultaneous endoscopic submucosal dissection of the neoplasm in the interpositioned colon were successfully performed.

Evidence of the ethical appropriateness and clinical benefits of shared decision-making (SDM) are accumulating. This study aimed to not only identify physicians’ perspectives on SDM, and practices related to end-of-life care in particular, but also to gauge the effect of SDM education on physicians in Korea. Methods: A 14-item questionnaire survey using a modified Delphi process was delivered to nephrologists and internal medicine trainees at 17 university hospitals. Results: A total of 309 physicians completed the survey. Although respondents reported that 69.9% of their practical decisions were made using SDM, 59.9% reported that it is not being applied appropriately. Only 12.3% of respondents had received education on SDM as part of their training. The main obstacles to appropriate SDM were identified as lack of time (46.0%), educational materials and tools (29.4%), and education on SDM (24.3%). Although only a few respondents had received training on SDM, the proportion of those who thought they were using SDM appropriately in actual practice was high; the proportion of those who chose lack of time and education as factors that hindered the proper application of SDM was low. Conclusion: The majority of respondents believed that SDM was not being implemented properly in Korea, despite its use in actual practice. To improve the effectiveness of SDM in the Korean medical system, appropriate training programs and supplemental policies that guarantee sufficient application time are required.

The Say–Barber–Biesecker–Young–Simpson variant of Ohdo syndrome (SBBYSS) (Online Mendelian Inheritance in Man #603736) is a rare autosomal dominant disorder and clinically features blepharophimosis with ptosis, a mask-like facial appearance, cryptorchidism, congenital heart defect, long thumbs/great toes, and thyroid dysfunction. The etiology of SBBYSS has been shown to be due to heterozygous KAT6B gene mutation. Here we report a case of a neonate with SBBYSS identified a novel mutation in KAT6B gene. The patient showed typical dysmorphic facies, cryptorchidism with micropenis, overriding fingers, and long thumbs and toes at birth. He had also hypothyroidism, large atrial septal defect, and sensorineural hearing loss. The next generation sequencing identified a heterozygous novel variant, c.5206C>T (p.Gln1736Ter) in KAT6B gene. At the 9 months of age, he underwent patch closure for atrial septal defect. Until the 12-month follow-up, he was under-developed.

Background: Chondroblastomas, which account for approximately 1% of all bone tumors, typically occur in long bones, such as the femur, humerus, and tibia. However, in extremely rare cases, they may also occur in the craniofacial region where the tumor is often found in the squamous portion of the temporomandibular joint (TMJ) and in the temporal bone.Case presentation: This case report describes a large chondroblastoma (diameter, approximately 37 mm) that occurred in the TMJ. The tumor was sufficiently aggressive to destroy the TMJ, mandibular condyle neck, external auditory canal (EAC), mandibular fossa of the temporal bone, and facial nerve. The tumor was completely excised using a pre-auricular approach. The EAC and surgical defect were successfully reconstructed using a temporoparietal fascia flap (TPFF) and an inguinal free fat graft. There was no local tumor recurrence at the 18-month follow-up visits. However, the patient developed sensory neural hearing loss, and his eyebrow paralysis worsened, eventually requiring plastic surgery. Conclusion Large, invasive chondroblastomas of the TMJ can be completely removed through a pre-auricular approach, and the resulting surgical defect can be reconstructed using TPFF and free fat grafts. However, preoperative evaluation of the facial nerve and auditory function is necessary. Therefore, a multidisciplinary approach is essential.

Short stature homeobox-containing gene (SHOX) is a well-known causative gene for the short stature in Turner syndrome. The clinical manifestation of SHOX gene related disorders varies from SHOX haploinsufficiency, presenting with idiopathic short stature, disproportionate short stature, or Leri-Weill dyschondrosteosis (LWD) to recessive form of extreme dwarfism and limb deformity in Langer mesomelic dysplasia. LWD is usually diagnosed upon suspicion based on short stature and skeletal abnormalities, and it is rarely accompanied with respiratory failure in the neonatal period. Here, we report the case of a newborn infant with LWD presenting with severe micrognathia that caused respiratory distress, which was diagnosed using microarray testing. Even when the manifestation of Madelung deformity is not yet apparent, LWD should be considered as one of underlying diseases related to congenital micrognathia.

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibroinflammatory tissue in the periaortic or periiliac retroperitoneum, where it frequently encases ureters. There is emerging evidence that a subset of this disease is part of a spectrum of multisystemic autoimmune diseases collectively referred to as “immunoglobulin G4 (IgG4)-related disease”. METHODS: We retrospectively analyzed 27 idiopathic RPF patients and identified a subset as IgG4-related RPF, which we categorized according to recently published comprehensive diagnostic criteria. We compared clinical and laboratory characteristics and response to treatment between the two groups. RESULTS: Of 27 total patients, 16 (59.3%) were diagnosed as having IgG4-related RPF, and these were predominantly male. They were also significantly older and more likely to have other organ involvement, hydronephrosis, and postrenal acute kidney injury (AKI) compared to those with idiopathic RPF. However, there was no difference in response rate to systemic steroid treatment. CONCLUSION: IgG4-related RPF accounts for a substantial portion of RPF cases previously identified as “idiopathic RPF” in Korea. Clinical and laboratory characteristics of IgG4-related RPF are similar to those of idiopathic RPF except for a striking male predominance, older age, and higher incidence of postrenal AKI in IgG4-related RPF. More comprehensive, prospective studies are needed to clearly distinguish IgG4-related RPF from idiopathic RPF based on clinical manifestation and to further assess treatment response and long-term prognosis.
Acute Kidney Injury ; Autoimmune Diseases ; Humans ; Hydronephrosis ; Immunoglobulins ; Incidence ; Korea ; Male ; Prognosis ; Prospective Studies ; Rare Diseases ; Retroperitoneal Fibrosis ; Retrospective Studies ; Strikes, Employee ; Ureter