1.Successful diagnosis and treatment of recurrent atypical hemolytic uremic syndrome posttransplantation caused by the heterozygous deletion of CFH in a patient with end-stage kidney disease of uncertain etiology
Haeun LEE ; Hoon Seok KIM ; Hanbi LEE ; Sang Hun EUM ; In O SUN ; Jaehoon SHIN ; Yeong Jin CHOI ; Chul Woo YANG ; Myungshin KIM ; Byung Ha CHUNG
Kidney Research and Clinical Practice 2024;43(1):125-129
2.Self-screening questionnaire for perianal fistulizing disease in patients with Crohn’s disease
O Seong KWEON ; Ben KANG ; Yoo Jin LEE ; Eun Soo KIM ; Sung Kook KIM ; Hyun Seok LEE ; Yun Jin CHUNG ; Kyeong Ok KIM ; Byung Ik JANG ;
The Korean Journal of Internal Medicine 2024;39(3):430-438
Background/Aims:
A poor prognostic factor for Crohn’s disease (CD) includes perianal fistulizing disease, including perianal fistula and/or perianal abscess. Currently, a tool to assess perianal symptoms in patients with CD remains nonexistent. This study aimed to develop a perianal fistulizing disease self-screening questionnaire for patients with CD.
Methods:
This prospective pilot study was conducted at three tertiary referral centers between January 2019 and May 2020. We formulated questions on perianal symptoms, including tenesmus, anal discharge, bleeding, pain, and heat. A 4-point Likert scale was used to rate each question. Patients with CD completed a questionnaire and underwent pelvic magnetic resonance imaging (MRI).
Results:
Overall, 93 patients were enrolled, with 51 (54.8%) diagnosed with perianal fistulizing disease, as determined by pelvic MRI. The Spearman correlation findings demonstrated that anal pain (p = 0.450, p < 0.001) and anal discharge (p = 0.556, p < 0.001) were the symptoms that most significantly correlated with perianal disease. For anal pain and discharge, the area under the receiver operating characteristic curve of the scores was significantly higher than that of the combined score for all five symptoms (0.855 vs. 0.794, DeLong’s test p = 0.04). For the two symptoms combined, the sensitivity, specificity, and positive predictive and negative predictive values were 88.2, 73.8, 80.4, and 83.8%, respectively, with 81.7% accuracy for detecting perianal fistulizing disease.
Conclusions
This study indicates that simple questions regarding anal pain and discharge can help accurately identify the presence of perianal fistulizing disease in patients with CD.
3.The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Survey About Specific Clinical Scenarios (Version 2023.1)
Min-Sung KIM ; Se-Il GO ; Chan Woo WEE ; Min Ho LEE ; Seok-Gu KANG ; Kyeong-O GO ; Sae Min KWON ; Woohyun KIM ; Yun-Sik DHO ; Sung-Hye PARK ; Youngbeom SEO ; Sang Woo SONG ; Stephen AHN ; Hyuk-Jin OH ; Hong In YOON ; Sea-Won LEE ; Joo Ho LEE ; Kyung Rae CHO ; Jung Won CHOI ; Je Beom HONG ; Kihwan HWANG ; Chul-Kee PARK ; Do Hoon LIM ;
Brain Tumor Research and Treatment 2023;11(2):133-139
Background:
During the coronavirus disease 2019 (COVID-19) pandemic, there was a shortage of medical resources and the need for proper treatment guidelines for brain tumor patients became more pressing. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. As part II of the guideline, this consensus survey is to suggest management options in specific clinical scenarios during the crisis period.
Methods:
The KSNO Guideline Working Group consisted of 22 multidisciplinary experts on neuro-oncology in Korea. In order to confirm a consensus reached by the experts, opinions on 5 specific clinical scenarios about the management of brain tumor patients during the crisis period were devised and asked. To build-up the consensus process, Delphi method was employed.
Results:
The summary of the final consensus from each scenario are as follows. For patients with newly diagnosed astrocytoma with isocitrate dehydrogenase (IDH)-mutant and oligodendroglioma with IDH-mutant/1p19q codeleted, observation was preferred for patients with low-risk, World Health Organization (WHO) grade 2, and Karnofsky Performance Scale (KPS) ≥60, while adjuvant radiotherapy alone was preferred for patients with high-risk, WHO grade 2, and KPS ≥60. For newly diagnosed patients with glioblastoma, the most preferred adjuvant treatment strategy after surgery was radiotherapy plus temozolomide except for patients aged ≥70 years with KPS of 60 and unmethylated MGMT promoters. In patients with symptomatic brain metastasis, the preferred treatment differed according to the number of brain metastasis and performance status. For patients with newly diagnosed atypical meningioma, adjuvant radiation was deferred in patients with older age, poor performance status, complete resection, or low mitotic count.
Conclusion
It is imperative that proper medical care for brain tumor patients be sustained and provided, even during the crisis period. The findings of this consensus survey will be a useful reference in determining appropriate treatment options for brain tumor patients in the specific clinical scenarios covered by the survey during the future crisis.
4.The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Recommendation Using the Delphi Method (Version 2023.1)
Min-Sung KIM ; Se-Il GO ; Chan Woo WEE ; Min Ho LEE ; Seok-Gu KANG ; Kyeong-O GO ; Sae Min KWON ; Woohyun KIM ; Yun-Sik DHO ; Sung-Hye PARK ; Youngbeom SEO ; Sang Woo SONG ; Stephen AHN ; Hyuk-Jin OH ; Hong In YOON ; Sea-Won LEE ; Joo Ho LEE ; Kyung Rae CHO ; Jung Won CHOI ; Je Beom HONG ; Kihwan HWANG ; Chul-Kee PARK ; Do Hoon LIM ;
Brain Tumor Research and Treatment 2023;11(2):123-132
Background:
During the coronavirus disease 2019 (COVID-19) pandemic, the need for appropriate treatment guidelines for patients with brain tumors was indispensable due to the lack and limitations of medical resources. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future.
Methods:
The KSNO Guideline Working Group was composed of 22 multidisciplinary experts on neuro-oncology in Korea. In order to reach consensus among the experts, the Delphi method was used to build up the final recommendations.
Results:
All participating experts completed the series of surveys, and the results of final survey were used to draft the current consensus recommendations. Priority levels of surgery and radiotherapy during crises were proposed using appropriate time window-based criteria for management outcome. The highest priority for surgery is assigned to patients who are life-threatening or have a risk of significant impact on a patient’s prognosis unless immediate intervention is given within 24–48 hours. As for the radiotherapy, patients who are at risk of compromising their overall survival or neurological status within 4–6 weeks are assigned to the highest priority. Curative-intent chemotherapy has the highest priority, followed by neoadjuvant/adjuvant and palliative chemotherapy during a crisis period. Telemedicine should be actively considered as a management tool for brain tumor patients during the mass infection crises such as the COVID-19 pandemic.
Conclusion
It is crucial that adequate medical care for patients with brain tumors is maintained and provided, even during times of crisis. This guideline will serve as a valuable resource, assisting in the delivery of treatment to brain tumor patients in the event of any future crisis.
5.The efficacy of EGFR-tyrosine kinase inhibitor in non-small cell lung cancer patients with synchronous brain metastasis: a real-world study
Jin-Hyuk CHOI ; Yong Won CHOI ; Hyun Woo LEE ; Seok Yun KANG ; Geum Sook JEONG ; Mi Sun AHN ; Young-Taek OH ; O kyu NOH ; Se-Hyuk KIM ; Tae Hoon ROH ; Seung Soo SHEEN
The Korean Journal of Internal Medicine 2022;37(2):434-443
Background/Aims:
The optimal treatment (Tx) for epidermal growth factor receptor (EGFR)-mutant non-small cell lung cancer (NSCLC) patients with brain metastasis (BM) remains to be determined.
Methods:
A retrospective review was conducted on 77 NSCLC patients with synchronous BM who underwent first-line EGFR-tyrosine kinase inhibitor (TKI) Tx. The outcomes of patients were analyzed according to the clinicopathological characteristics including local Tx modalities.
Results:
Fifty-nine patients underwent local Tx for BM (gamma knife surgery [GKS], 37; whole brain radiotherapy [WBRT], 18; others, four) concurrently or sequentially with EGFR-TKI. Patients treated with TKI alone showed significantly lower incidence of central nervous system (CNS) symptoms. The median progression-free survival (PFS) and overall survival (OS) after the initiation of EGFR-TKI for all patients were 9 and 19 months, respectively. In 60 patients with follow-up brain imaging, the median time to CNS progression was 15 months. Patients with EGFR exon 19 deletion had a significantly longer median OS than those with other mutations including L858R (23 months vs. 17 months). Other clinical characteristics, including CNS symptoms, number of BM, and the use of local Tx were not associated with OS, as well as PFS. In terms of the local optimal Tx modality, no difference was found between GKS and WBRT in the OS and PFS.
Conclusions
This study suggests that EGFR-TKI may result in a favorable outcome in NSCLC patients with synchronous BM, especially in deletion 19 mutant, regardless of the extent of BM lesions or local Tx modalities. Patients with asymptomatic BM can be treated with EGFR-TKI and careful surveillance.
6.A Comparative Study of an Anti-Thrombotic Small-Diameter Vascular Graft with Commercially Available e-PTFE Graft in a Porcine Carotid Model
Kyo Seon LEE ; Mukhammad KAYUMOV ; Gladys A. EMECHEBE ; Do-Wan KIM ; Hwa-Jin CHO ; Yun-Jin JEONG ; Dong-Weon LEE ; Jun-Kyu PARK ; Chan-Hee PARK ; Cheol-Sang KIM ; Francis O. OBIWELUOZOR ; In-Seok JEONG
Tissue Engineering and Regenerative Medicine 2022;19(3):537-551
BACKGROUND:
We have designed a reinforced drug-loaded vascular graft composed of polycaprolactone (PCL) and polydioxanone (PDO) via a combination of electrospinning/3D printing approaches. To evaluate its potential for clinical application, we compared the in vivo blood compatibility and performance of PCL/PDO ? 10%DY grafts doped with an antithrombotic drug (dipyridamole) with a commercial expanded polytetrafluoroethylene (e-PTFE) graft in a porcine model.
METHODS:
A total of 10 pigs (weight: 25–35 kg) were used in this study. We made a new 5-mm graft with PCL/PDO composite nanofiber via the electrospinning technique. We simultaneously implanted a commercially available e-PTFE graft (n = 5) and our PCL/PDO ? 10%DY graft (n = 5) into the carotid arteries of the pigs. No anticoagulant/antiplatelet agent was administered during the follow-up period, and ultrasonography was performed weekly to confirm the patency of the two grafts in vivo. Four weeks later, we explanted and compared the performance of the two grafts by histological analysis and scanning electron microscopy (SEM).
RESULTS:
No complications, such as sweating on the graft or significant bleeding from the needle hole site, were seen in the PCL/PDO ? 10%DY graft immediately after implantation. Serial ultrasonographic examination and immunohistochemical analysis demonstrated that PCL/PDO ? 10%DY grafts showed normal physiological blood flow and minimal lumen reduction, and pulsed synchronously with the native artery at 4 weeks after implantation. However, all e-PTFE grafts occluded within the study period. The luminal surface of the PCL/PDO ? 10%DY graft in the transitional zone was fully covered with endothelial cells as observed by SEM.
CONCLUSION
The PCL/PDO ? 10%DY graft was well tolerated, and no adverse tissue reaction was observed in porcine carotid models during the short-term follow-up. Colonization of the graft by host endothelial and smooth muscle cells coupled with substantial extracellular matrix production marked the regenerative capability. Thus, this material may be an ideal substitute for vascular reconstruction and bypass surgeries. Long-term observations will be necessary to determine the anti-thrombotic and remodeling potential of this device.
7.Coronoidectomy for reduction of superolateral dislocation of mandible condyle
Hyun SEOK ; Seung-O KO ; Jin-A BAEK ; Dae-Ho LEEM
Journal of the Korean Association of Oral and Maxillofacial Surgeons 2022;48(3):182-187
Superolateral dislocation of the condyle is a rare mandibular fracture. The treatment goal is to return the dislocated condyle to its original position to recover normal function. This study reports on superolateral dislocation of the condyle with mandibular body fracture. The mandibular body was completely separated, and the medial pole of the condyle head was fractured. The condyle segment was unstable and easily dislocated after reduction. The temporalis muscle on the condyle segment might have affected the dislocation of the condyle. A coronoidectomy was performed to disrupt the function of the temporalis muscle on the condyle segment in order to successfully reduce the dislocated condyle. Coronoidectomy is a simple procedure with minimal complications. We successfully performed a coronoidectomy to reduce the superolateral displaced condyle to its original position to achieve normal function. Coronoidectomy can be effectively used for reduction of superolaterally displaced condyles combined with severe maxilla-mandibular fractures.
8.Speech-aid prosthesis in velopharyngeal incompetency patient with cleft palate: can speech aids be applicable for adult patient?
Dong-Cheol KANG ; Jung-Ho PARK ; Hyun SEOK ; Jin-A BAEK ; Da-Wa KIM ; Seung-O KO
Maxillofacial Plastic and Reconstructive Surgery 2021;43(1):29-
Background:
Velopharyngeal incompetence (VPI) therapy for cleft palate (speech therapy alone, speech therapy using speech aids, or combined therapy such as speech therapy using a pharyngeal flap), is more effective in younger patients than in adult patients. Speech therapy is known as very difficult for patients who still have VPI as an adult. Because of the possibility of subsequent speech disorders, the timing of surgery for cleft palate is accelerating. Herein, we present a case of an adult with articulation disorder due to VPI who was treated by speech therapy and a speech-aid prosthesis.Case presentation: A woman who underwent cleft palate surgery at 8 years of age still had difficulty with articulation due to VPI as a 24-year-old adult because of a lack of continuous speech therapy. We decided to use a speech-aid application using palatal lift, and a reduction program was conducted four times, along with simultaneous speech therapy, over a period of 1 year and 7 months. During the therapy period, she was able to speak normally within a relatively short period of time, and after implementation of the reduction program, the therapy was completed by completely removing the device. Long-term observations have shown normal speech function without recurrence, even after the device was removed.
Conclusion
As seen in this case, speech therapy using speech aids can show a good result for adult patients with cleft palate who missed the usual timing for the treatment of articulation disorders, depending on the situation. Therefore, it is hereby reported as a therapy option worthy of consideration.
9.Phenotypes of Severe Cutaneous Adverse Reactions Caused by Nonsteroidal Anti-inflammatory Drugs
Suh Young LEE ; Young Hee NAM ; Young Il KOH ; Sae Hoon KIM ; Sujeong KIM ; Hye Ryun KANG ; Min Hye KIM ; Jun Gyu LEE ; Jung Won PARK ; Hye Kyung PARK ; Hyen O LA ; Mi Yeong KIM ; Seong Ju PARK ; Yong Eun KWON ; Jae Woo JUNG ; Sang Hyon KIM ; Cheol Woo KIM ; Min Seok YANG ; Min Gyu KANG ; Jin Yong LEE ; Joo Hee KIM ; Sang Heon KIM ; Gyu Young HUR ; Young Koo JEE ; Hyun Jung JIN ; Chan Sun PARK ; Yi Yeong JEONG ; Young Min YE
Allergy, Asthma & Immunology Research 2019;11(2):212-221
PURPOSE: Nonsteroidal anti-inflammatory drugs (NSAIDs) are common cause of severe cutaneous adverse reactions (SCARs). The present study aimed to investigate the characteristics of SCARs induced by NSAIDs in the Korean SCAR registry. METHODS: A retrospective survey of NSAID-induced SCARs recorded between 2010 and 2015 at 27 university hospitals in Korea was conducted. Clinical phenotypes of SCARs were classified into Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), SJS-TEN overlap syndrome and drug reaction with eosinophilia and systemic symptoms (DRESS). Causative NSAIDs were classified into 7 groups according to their chemical properties: acetaminophen, and propionic, acetic, salicylic, fenamic and enolic acids. RESULTS: A total of 170 SCARs, consisting of 85 SJS, 32 TEN, 17 SJS-TEN overlap syndrome and 36 DRESS reactions, were induced by NSAIDs: propionic acids (n=68), acetaminophen (n=38), acetic acids (n=23), salicylic acids (n=16), coxibs (n=8), fenamic acids (n=7), enolic acids (n=5) and unclassified (n=5). Acetic acids (22%) and coxibs (14%) accounted for higher portions of DRESS than other SCARs. The phenotypes of SCARs induced by both propionic and salicylic acids were similar (SJS, TEN and DRESS, in order). Acetaminophen was primarily associated with SJS (27%) and was less involved in TEN (10%). DRESS occurred more readily among subjects experiencing coxib-induced SCARs than other NSAID-induced SCARs (62.5% vs. 19.7%, P = 0.013). The mean time to symptom onset was longer in DRESS than in SJS or TEN (19.1 ± 4.1 vs. 6.8 ±1.5 vs. 12.1 ± 3.8 days). SCARs caused by propionic salicylic acids showed longer latency, whereas acetaminophen- and acetic acid-induced SCARs appeared within shorter intervals. CONCLUSIONS: The present study indicates that the phenotypes of SCARs may differ according to the chemical classifications of NSAIDs. To establish the mechanisms and incidences of NSAID-induced SCARs, further prospective studies are needed.
Acetaminophen
;
Acetates
;
Acetic Acid
;
Anti-Inflammatory Agents, Non-Steroidal
;
Cicatrix
;
Classification
;
Cyclooxygenase 2 Inhibitors
;
Diethylpropion
;
Drug Hypersensitivity
;
Drug Hypersensitivity Syndrome
;
Hospitals, University
;
Incidence
;
Korea
;
Phenotype
;
Propionates
;
Prospective Studies
;
Retrospective Studies
;
Salicylates
;
Salicylic Acid
;
Stevens-Johnson Syndrome
10.Treatment of velopharyngeal insufficiency in a patient with a submucous cleft palate using a speech aid: the more treatment options, the better the treatment results
Yun Ha PARK ; Hyun Jun JO ; In Seok HONG ; Dae Ho LEEM ; Jin A BAEK ; Seung O KO
Maxillofacial Plastic and Reconstructive Surgery 2019;41(1):19-
BACKGROUND:
The submucous cleft palate (SMCP) is a type of cleft palate that may result in velopharyngeal insufficiency (VPI). Palate muscles completely separate oral and nasal cavities by closing off the velopharynx during functional processes such as speech or swallow. Also, hypernasality may arise from anatomical or neurological abnormalities in these functions. Treatments of this issue involve a combination of surgical intervention, speech aid, and speech therapy. This case report demonstrates successfully treated VPI resulted from SMCP without any surgical intervention but solely with speech aid appliance and speech therapy.CASE PRESENTATION: A 13-year-old female patient with a speech disorder from velopharyngeal insufficiency that was caused by a submucous cleft palate visited to our OMFS clinic. In the intraoral examination, the patient had a short soft palate and bifid uvula. And the muscles in the palate did not contract properly during oral speech. She had no surgical history such as primary palatoplasty or pharyngoplasty except for tonsillectomy. And there were no other medical histories. Objective speech assessment using nasometer was performed. We diagnosed that the patient had a SMCP. The patient has shown a decrease in speech intelligibility, which resulted from hypernasality. We decided to treat the patient with speech aid (palatal lift) along with speech therapy. During the 7-month treatment, hypernasality measured by a nasometer decreased and speech intelligibility became normal.
CONCLUSIONS
Surgery remains the first treatment option for patients with velopharyngeal insufficiencies from submucous cleft palates. However, there were few reports about objective speech evaluation pre- or post-operation. Moreover, there has been no report of non-surgical treatment in the recent studies. From this perspective, this report of objective improvement of speech intelligibility of VPI patient with SMCP by non-surgical treatment has a significant meaning. Speech aid can be considered as one of treatment options for management of SMCP.

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