• Title/Summary/Keyword: Refractory

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Haploidentical hematopoietic stem cell transplantation in children and adolescents with acquired severe aplastic anemia

  • Im, Ho Joon;Koh, Kyung-Nam;Seo, Jong Jin
    • Clinical and Experimental Pediatrics
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    • v.58 no.6
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    • pp.199-205
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    • 2015
  • Severe aplastic anemia (SAA) is a life-threatening disorder for which allogeneic hematopoietic stem cell transplantation (HSCT) is the current available curative treatment. HSCT from matched sibling donors (MSDs) is the preferred therapy for children with acquired SAA. For patients who lack MSDs, immunosuppressive therapy (IST) is widely accepted as a first-line treatment before considering HCT from an unrelated donor (URD). Given the recent progress in HSCT using URDs for childhood SAA, well-matched URDs became a realistic alternative for pediatric patients who have no suitable related donors and who are refractory to IST. However, it is quite challenging to treat patients with refractory SAA who lack suitable related or URDs. Even though haploidentical HSCT from genetically mismatched family members seemed to be an attractive procedure with the amazing benefit of readily available donors for most patients, early attempts were disappointing because of refractory graft-versus-host disease (GVHD) and excessively high transplant-related mortality. Recent advances with effective ex vivo depletion of T cells or unmanipulated in vivo regulation of T cells, better supportive care, and optimal conditioning regimens have significantly improved the outcome of haploidentical transplant. Besides considerable progress in the treatment of malignant diseases, recent emerging evidences for haploidentical HSCT in SAA has provided additional therapeutic options for patients with refractory diseases. Further improvements to decrease the rates of graft failure, GVHD, and infectious complications will facilitate the emergence of haploidentical HSCT as a front-line therapy for treating acquired SAA in children and adolescents who have no suitably matched donors.

Ascites-decreasing Effect of SB Intraperitoneal Injection to a Refractory Ascites Patient with Synchronous Colorectal Liver Metastasis and Metachronous Peritoneal Carcinomatosis : A Case Report (동시 간전이 직장 구불결장암 환자의 재발성 복수에 대한 SB 복강 주입의 복수 감소 효과 증례)

  • Jeon, Hyung-Joon;Kim, Jong-Min;Cho, Chong-Kwan;Lee, Yeon-Weol;Han, Kyun-In;Yoo, Hwa-Seung
    • The Journal of Internal Korean Medicine
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    • v.34 no.4
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    • pp.466-477
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    • 2013
  • Objectives : To report and demonstrate the effect of decreasing ascites volume by SB intraperitoneal injection to a refractory ascites patient with synchronous colorectal liver metastasis and metachronous peritoneal carcinomatosis. Methods : Two cycles of intraperitoneal and intravenous SB injection were conducted. Each injection cycle was made up of 4 days. Nine vials of SB were injected to the patient every day. To compare the volume of ascites between pret- and post-treatment, follow-up computed tomography was done on June 3, 2013. To observe other therapeutic effects of SB injection, laboratory tests were conducted periodically. Results : On the follow-up computed tomography images, the amount of ascites and pleural effusion had decreased compared to the April 30, 2013 computed tomography images. The levels of aspartate transaminase, alanine aminotransferase and lactate dehydrogenase decreased significantly from May 9, to May 30, 2013. The amount of oral intake increased constantly during hospitalization. The patient's symptoms such as abdominal distension, abdominal pain and dyspnea were improving until discharge. Conclusions : Even if thiese results cannot be applied to every synchronous colorectal cancer liver metastasis patient, we demonstrated that SB intraperitoneal injection has ascites-decreasing effect to refractory ascites patients with synchronous colorectal liver metastasis and metachronous peritoneal carcinomatosis.

Spectrum of the WHO Classification De Novo Myelodysplastic Syndrome: Experience from Southern Pakistan

  • Sultan, Sadia;Irfan, Syed Mohammed;Jawed, Syeda Narisa
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.3
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    • pp.1049-1052
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    • 2016
  • Background: Myelodysplastic syndrome (MDS) is a clonal disorder of hemopoeitic stem cells, characterized by infective hematopoiesis, peripheral cytopenias along with hypercellularity of marrow and marked dysplastic features. Our aim was to study the spectrum of the WHO classification in adult Pakistani patients with MDS at disease presentation. Materials and Methods: This retrospective descriptive study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Patient data were retrieved from the maintained archives. Results: Overall, 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean of $57.6{\pm}17.4years$. The male to female ratio was 1.7:1. According to the WHO classification, 53.3% had refractory cytopenia with multilineage dysplasia, 22.2% had refractory cytopenia with unilineage dysplasia, 4.4% each had refractory anemia with excess of blasts-1 and II and 15.5% had MDS unclassified. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20% of patients. Hemoglobin was <10 g/dl in 41 (91.1%). Pancytopenia and bicytopenia were noted in 18 (40%) and 14 (31.1%) respectively. Conclusions: MDS in our patients presents at a relatively young age. Refractory c ytopenia with multilineage dysplasia was the dominant disease variant in our setting.

Unusual Waveform Detection Algorithm in Arrhythmia ECG Signal (부정맥 심전도 신호에서 특이 파형 검출)

  • Park, Kil-Houm;Kim, Jin-Sub;Ryu, Chunha;Choi, Byung-Jae;Kim, Jungjoon
    • Journal of the Korean Institute of Intelligent Systems
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    • v.23 no.4
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    • pp.292-297
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    • 2013
  • In this paper, unusual waveform detection algorithm based on Refractory Period in arrhythmia ECG signal is proposed. Most of arrhythmia ECG signals consist of unusual waveforms with average 10% rate. Thus tremendous benefit can be obtained in terms of time and cost by providing unusual waveform samples reduced more than 90% to medical staffs who have to monitor and analyze for a long time. The proposed algorithm detects the R-peak using the features of R wave and variable refractory period. For the detected R-peak, unusual waveforms are found using means and standard deviation of electric potential and kurtosis of the R-peaks which are not included in unusual waveform. The proposed algorithm was applied to all records of the MIT-BIH arrhythmia database and showed more than average 90% of compression ratio.

Treatment of refractory IgA vasculitis with dapsone: a systematic review

  • Lee, Keum Hwa;Hong, Sung Hwi;Jun, Jinhae;Jo, Youngheun;Jo, Woogyeong;Choi, Dayeon;Joo, Jeongho;Jung, Guhyun;Ahn, Sunghee;Kronbichler, Andreas;Eisenhut, Michael;Shin, Jae Il
    • Clinical and Experimental Pediatrics
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    • v.63 no.5
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    • pp.158-163
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    • 2020
  • IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

A Case of Refractory Ascites Due To Hepatocellular Carcinoma Treated with Sasang Constitutional Medicine (간세포암에 동반된 난치성 복수를 호전시킨 소양인 환자 치험1례)

  • Jung, Yee-Hong;Kim, Eun-Hee;Seo, Young-Kwang;Choi, Won-Cheol;Lee, Soo-Kyung;Eo, Wan-Kyu
    • Journal of Sasang Constitutional Medicine
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    • v.20 no.2
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    • pp.164-170
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    • 2008
  • 1. Objectives This is a case of refractory ascites due to hepatocellular carcinoma treated with Sasang Constitutional Medication. Refractory ascites, which did not respond to diuretics, was improved with Jeoryeongchajeonja-tang. 2. Methods We recorded the patient's abdominal circumference, body weight and urine output daily. We also investigated the TTKG(transtubular potassium gradient) values 3 times during the treatment course. 3. Results the TTKG value of the patient was siginificantly decreased. TTKG values before administration of Jeotyeongchajeonja-tang were 8.41, 10.82, respectively and 4.25 after administration of Jeotyeongchajeonja-tang. 4. Conclusions We guess that Jeoryeongchajeonja-tang functions as an aldosterone antagonist or increases spironolactone activity through interaction between Jeotyeongchajeonja-tang and spironolactone. But the mechanism of Jeotyeongchajeonja-tang is not clear, so we need more clinical study of refractory ascites improved with Jeotyeongchajeonja-tang and biological study of interaction between Jeotyeongchajeonja-tang and spironolactone.

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An R-wave Detection method in ECG Signal Using Refractory Period (ECG 신호에서 불응기를 이용한 R-파 검출 방법)

  • Kim, Jin-Sub;Kim, Jea-Soo;Kim, Jeong-Hong
    • Journal of the Korea Society of Computer and Information
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    • v.18 no.1
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    • pp.93-101
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    • 2013
  • The accurate detection of R-wave is important for other feature extraction in ECG, and R-wave has a lot of medical information about heart. Numerous R-wave detection algorithms have been studied on the ECG signal shape analysis, but it was difficult to find accurate R-wave when the shape of R-wave is similar to the shape of P-wave. This paper presents an R-wave detection method based on the refractory period that is the period of depolarization and repolarization of the cell membrane after excitation. And we also use the shape of kurtosis in the refractory period. The proposed method is validated using the ECG records of the MIT-BIH arrhythmia database. Experimental results show that the proposed method significantly outperforms other method in case of 105 and 108 record that have R-wave similar to P-wave, as well as other records.

Pathophysiology of Potassium-competitive Acid Blocker-refractory Gastroesophageal Reflux and the Potential of Potassium-competitive Acid Blocker Test

  • Masaoka, Tatsuhiro;Kameyama, Hisako;Yamane, Tsuyoshi;Yamamoto, Yuta;Takeuchi, Hiroya;Suzuki, Hidekazu;Kitagawa, Yuko;Kanai, Takanori
    • Journal of Neurogastroenterology and Motility
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    • v.24 no.4
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    • pp.577-583
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    • 2018
  • Background/Aims Potassium-competitive acid blockers are expected to be the next generation of drugs for the treatment of diseases caused by gastric acid. In 2015, vonoprazan fumarate, a novel potassium-competitive acid blocker, was approved by the Japanese health insurance system. Since its approval, patients refractory to vonoprazan can be encountered in clinical settings. We designed this study to clarify the pathophysiology of gastroesophageal reflux disease refractory to vonoprazan. Methods In this retrospective study, we involved patients who had refractory symptoms after administration of standard-dose proton pump inhibitors or vonoprazan and underwent diagnostic testing with esophageal high-resolution manometry and 24-hour multichannel intraluminal impedance and pH monitoring while using proton pump inhibitors or vonoprazan. Patients were diagnosed based on the Rome IV criteria for functional gastrointestinal disorders and diagnostic test results. Results Twenty-seven patients were analyzed during this study. Gastric pH ${\geq}4$ was sustained for a longer period of time, and the esophageal acid exposure time and number of acid reflux events were shorter in the vonoprazan group than in the proton pump inhibitor group. The percentage of patients diagnosed with acidic gastroesophageal reflux disease in the vonoprazan group was lower than that in the proton pump inhibitor group. Conclusions Intra-gastric pH and acid reflux were strongly suppressed by 20-mg vonoprazan. When patients with gastroesophageal reflux disease present symptoms after administration of 20-mg vonoprazan, the possibility of pathophysiologies other than acid reflux should be considered.

Hybrid Lymphovenous Anastomosis Surgery Guided by Intraoperative Mesenteric Intranodal Lymphangiography for Refractory Nontraumatic Chylous Ascites: A Case Report

  • Soo Jin Woo;Saebeom Hur;Hee Seung Kim;Hak Chang;Ji-Young Kim;Soo Jin Park;Ung Sik Jin
    • Archives of Plastic Surgery
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    • v.51 no.1
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    • pp.130-134
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    • 2024
  • Refractory chylous ascites can cause significant nutritional and immunologic morbidity, but no clear treatment has been established. This article introduces a case of a 22-year-old female patient with an underlying lymphatic anomaly who presented with refractory chylous ascites after laparoscopic adnexectomy for ovarian teratoma which aggravated after thoracic duct embolization. Ascites (>3,000 mL/d) had to be drained via a percutaneous catheter to relieve abdominal distention and consequent dyspnea, leading to significant cachexia and weight loss. Two sessions of hybrid lymphovenous anastomosis (LVA) surgery with intraoperative mesenteric lymphangiography guidance were performed to decompress the lymphatics. The first LVA was done between inferior mesenteric vein and left para-aortic enlarged lymphatics in a side-to-side manner. The daily drainage of chylous ascites significantly decreased to 130 mL/day immediately following surgery but increased 6 days later. An additional LVA was performed between right ovarian vein and enlarged lymphatics in aortocaval area in side-to-side and end-to-side manner. The chylous ascites resolved subsequently without any complications, and the patient was discharged after 2 weeks. The patient regained weight without ascites recurrence after 22 months of follow-up. This case shares a successful experience of treating refractory chylous ascites with lymphatic anomaly through LVA, reversing the patient's life-threatening weight loss. LVA was applied with a multidisciplinary approach using intraoperative mesenteric lipiodol, and results showed the possibility of expanding its use to challenging problems in the intraperitoneal cavity.

Surgical Excision for Refractory Ischiogluteal Bursitis: A Consecutive Case Series of 21 Patients

  • Sun-Ho Lee;Won-Young Jang;Min-Su Lee;Taek-Rim Yoon;Kyung-Soon Park
    • Hip & pelvis
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    • v.35 no.1
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    • pp.24-31
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    • 2023
  • Purpose: A response to conservative treatment is usually obtained in cases of ischiogluteal bursitis. However, the time required to achieve relief of symptoms can vary from days to weeks, and there is a high recurrence rate, thus invasive treatment in addition to conservative treatment can occasionally be effective. Therefore, the aim of this study was to examine surgical excision in cases of refractory ischiogluteal bursitis and to evaluate patients' progression and outcome. Materials and Methods: A review of 21 patients who underwent surgical excision for treatment of ischiogluteal bursitis between February 2009 and July 2020 was conducted. Of these patients, seven patients were male, and 14 patients were female. Injection of steroid and local anesthetic into the ischial bursa was administered at outpatient clinics in all patients, who and they were refractory to conservative treatment, including aspiration and prescription drugs. Therefore, surgery was considered necessary. Excisions were performed by two orthopedic specialists using a direct vertical incision on the ischial area. A review of each patient was performed after excision, and quantification of the outcomes recorded using clinical scoring systems was performed. Results: The results of radiologic evaluation showed that the mean lesion size was 6.2 cm×4.5 cm×3.6 cm. The average disease course after excision was 21.6 days (range, 15-48 days). Measurement of clinical scores, including the visual analog scale and Harris hip scores, was performed during periodic visits, with scores of 0.7 (range, 0-2) and 98.1 (range, 96-100) at one postoperative month, respectively. Conclusion: Surgical excision, with an expectation of favorable results, could be considered for treatment of ischiogluteal bursitis that is refractory to therapeutic injections, aspirations, and medical prescriptions, particularly in moderate-to-severe cases.