• Clinical and Experimental Pediatrics
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• 제59권2호
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• pp.80-90
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• 2016

Purpose: Studies have been conducted to identify predictive factors of resistance to intravenous immunoglobulin (IVIG) for Kawasaki disease (KD). However, the results are conflicting. This study aimed to identify laboratory factors predictive of resistance to high-dose IVIG for KD by performing meta-analysis of available studies using statistical techniques. Methods: All relevant scientific publications from 2006 to 2014 were identified through PubMed searches. For studies in English on KD and IVIG resistance, predictive factors were included. A meta-analysis was performed that calculated the effect size of various laboratory parameters as predictive factors for IVIG-resistant KD. Results: Twelve studies comprising 2,745 patients were included. Meta-analysis demonstrated significant effect sizes for several laboratory parameters: polymorphonuclear leukocytes (PMNs) 0.698 (95% confidence interval [CI], 0.469-0.926), C-reactive protein (CRP) 0.375 (95% CI, 0.086-0.663), pro-brain natriuretic peptide (pro-BNP) 0.561 (95% CI, 0.261-0.861), total bilirubin 0.859 (95% CI, 0.582-1.136), alanine aminotransferase (AST) 0.503 (95% CI, 0.313-0.693), aspartate aminotransferase (ALT) 0.436 (95% CI, 0.275-0.597), albumin 0.427 (95% CI, -0.657 to -0.198), and sodium 0.604 (95% CI, -0.839 to -0.370). Particularly, total bilirubin, PMN, sodium, pro-BNP, and AST, in descending numerical order, demonstrated more than a medium effect size. Conclusion: Based on the results of this study, laboratory predictive factors for IVIG-resistant KD included higher total bilirubin, PMN, pro-BNP, AST, ALT, and CRP, and lower sodium and albumin. The presence of several of these predictive factors should alert clinicians to the increased likelihood that the patient may not respond adequately to initial IVIG therapy.

• Clinical and Experimental Pediatrics
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• 제51권2호
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• pp.204-208
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• 2008

목 적 : 본 연구는 소아 급성 ITP에서 IVIG 치료 후 호중구 감소의 빈도와 정도 및 경과 등을 알아보기 위하여 시행되었다. 방 법 : 2001년 1월부터 2006년 6월까지 부산대학교병원에서 급성 ITP로 진단받은 소아 환자 총 54례를 대상으로 하였다. 이중 IVIG를 투여한 군이 42례, Anti-D Ig을 투여한 군이 12례이었으며, 각각의 투여 후에 백혈구 및 호중구 수를 비교하였다. 또한 IVIG의 투여 방법 및 투여 횟수에 따른 백혈구 및 호중구 수도 비교하였다. 결 과 : IVIG 투여 군과 Anti-D Ig 투여 군에서 투여 직전의 백혈구 및 호중구 수의 차이는 없었으나, 투여 후에는 IVIG 투여군에서 42례 중 32례(76.2%)가 투여 전에 비해 호중구 수가 50% 이상 감소하였고, 투여 종료 후 1일째에 호중구수가 최저로 감소하였다. 한편 Anti-D Ig 투여군에서는 12례 중 2례(16.7 %)만이 투여 전 호중구 수에 비해 50% 이상으로 감소하였다. 첫 번째 IVIG 투여군(42례)과 재투여군(7례)에서 호중구 수의 감소는 통계학적인 차이가 없었다. IVIG 투여 방법에 따른 호중구 수의 감소는 5일간 투여군과 2일간 투여군 사이에 통계학적인 차이가 없었다. 면역글로불린 투여 후에 발생한 호중구 감소는 평균 투여 종료 7일 후에 39례 중 38례(97%)에서 자연적으로 회복되었다. 결 론 : 소아 ITP 환자에서 IVIG 투여 후에 호중구 감소가 비교적 흔하게 발생되며, 이는 일시적인 현상으로 대부분 자연적으로 회복되는 것으로 생각된다.

• Clinical and Experimental Pediatrics
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• 제59권8호
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• pp.328-334
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• 2016

Purpose: Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods: We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results: A significant difference was found in the total duration of fever between the groups ($13.0{\pm}4.04days$ in group 1 vs. $8.87{\pm}2.30days$ in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4%). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion: No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.

• Kidney Research and Clinical Practice
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• 제37권4호
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• pp.414-417
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• 2018

Disseminated adenovirus infection can result in high mortality and morbidity in immunocompromised patients. Here, we report the case of a 10-year-old renal allograft recipient who presented with hematuria and dysuria. Adenovirus was isolated from his urine. His urinary symptoms decreased after intravenous hydration and reduction of immunosuppressants. However, 2 weeks later he presented with general weakness and laboratory tests indicated renal failure necessitating emergency hemodialysis. Adenovirus was detected in his sputum; therefore, intravenous ganciclovir and immunoglobulin therapy were initiated. Renal biopsy revealed diffuse necrotizing granulomatous tubulointerstitial nephritis compatible with renal involvement of the viral infection. Adenovirus was detected in his serum. Despite cidofovir administration for 2 weeks, adenovirus was also detected in the cerebrospinal fluid, resulting in generalized tonic-clonic seizure. The patient died 7 weeks after the onset of urinary symptoms. Adenovirus should be considered in screening tests for post-renal transplantation patients who present with hemorrhagic cystitis.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.57-60
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• 2004

The sixty two-year-old woman was admitted with facial diplegia and ataxic gait. Neurological examination revealed areflexia and sensory ataxia with decreased sensation of position and vibration in both lower extremities. Electrophysiologic study suggest motor dominant demyelinating polyneuropathy and bilateral facial neuropathy. CSF study revealed no cells and increased proteins. After intravenous immunoglobulin therapy, sensory ataxia and electrophysiological study had markedly improved for 3 months.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.43-47
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• 2004

We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache, diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealed tachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showed ptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increased serum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middle mediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biopsy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticosteroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite the chemotherapy for LMS.

• Clinical and Experimental Pediatrics
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• 제51권5호
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• pp.457-461
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• 2008

Kawasaki disease (KD) was first described by Dr. Tomisaku Kawasaki in his 1975 study, published in Pediatrics. Its pathogenesis is still not clearly understood. Early diagnosis and treatment are very important to preventing concomitant coronary artery complications. Most KD patients respond well to the standard treatment of aspirin and intravenous immunoglobulin; however, some of them are refractory to the standard treatment, and so adjuvant therapies with corticosteroids and anti-tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) antibody are necessary. In this article, the author reviews and summarizes the most recent literature on the treatment of refractory KD.

• Annals of Clinical Neurophysiology
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• 제3권2호
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• pp.172-175
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• 2001

Guillain-$Barr{\acute{e}}$ syndrome(GBS) is a common demyelinating disease of the peripheral nervous system. But recently, the axonal types are also reported. Acute transverse myelitis(ATM) is also a common inflammatory disease of the spinal cord. Generally, it is difficult to identify the etiology of GBS and ATM. I guess the occurrence of the 2 diseases at once is hard to take the place. A 63-year-old woman showed an acute motor axonal GBS and a cervical-upper thoracic ATM occurring at the same time. She was treated by intravenous immunoglobulin and solumedrol therapy. Her sensory symptoms were improved rapidly but motor symptoms showed only mild improvement.

• Journal of Yeungnam Medical Science
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• 제38권2호
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• pp.165-168
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• 2021

We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/µL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/µL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/µL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.161-164
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• 2016

Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-$Barr{\acute{e}}$ syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.