• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.31 no.3
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• pp.161-164
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• 2020

Crohn's disease is a chronic inflammatory disease that involves the upper and lower gastrointestinal tracts, including the oral cavity. For the conventional treatment of inflammatory bowel diseases, steroids are usually used as induction therapy, and azathioprine is used as maintenance therapy. Steroids are associated with various side effects. Under rare circumstances, they can even cause psychotic symptoms, and reports on steroid-induced psychosis in the pediatric population are few. Here, we report the first case of steroid-induced psychosis in a 16-year-old female with Crohn's disease administered with steroids as induction therapy in South Korea. She showed psychotic symptoms, including auditory hallucination, loosening of association, and idea of reference, after 2 weeks of administering prednisolone 60 mg/day. One week after steroid discontinuation, the patient gradually recovered and was treated with quetiapine. Therefore, pediatricians should be aware of serious psychiatric adverse effects when administering corticosteroids for various indications.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.

• Childhood Kidney Diseases
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• v.2 no.2
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• pp.110-117
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• 1998

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS. Methods : 37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) fur 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) fur 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. Results : Complete remission rate of nephrotic syndrome was $58.8\%$ in CsA, $57.1\%$ in RFP and $38.4\%$ in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was $88.2\%$ in CsA, $85.7\%$ in RFP and $84.6\%$ in AZA group. Disappearance rate of hematuria was $58.8\%$ in CsA, $57.1\%$ in RFP and $46.2\%$ in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA($29.4\%$), none RFP($0\%$) and 2 AZA group($12.4\%$). Improvement on histologic immune-deposition was seen in 15 CsA($88.2\%$), 6 RFP($85.9\%$) and 4 AZA group($30.8\%$). Conclusion : In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

• Journal of Chest Surgery
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• v.31 no.10
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• pp.1004-1008
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• 1998

We report a case of heart-lung transplantation in a 32 year-old female with Eisenmenger syndrome secondary to patent ductus arteriosus. She has been suffered from congestive heart failure since June 1996 and repeatedly treated at Intensive Care Unit with intravenous inotropic support since July 1997. Preoperative echocardiography showed a patent ductus arteriosus with right to left shunt, severe regurgitation of tricuspid valve and estimated right ventricular systolic pressure of 100mmHg. The brain-dead donor was an 18 year-old male with head trauma from traffic accident 3 days ago. Heart-lung block procurement was performed at another general hospital and was transported to the Seoul National University Hospital by ambulance. Total ischemic time of the transplanted heart and lung were 249 minutes and 270 minutes, respectively. The immunosuppressive therapy was commenced preoperatively with cyclosporine and azathioprine. Corticosteroid was not used until postoperative 3 weeks in order to avoid infection and delayed healing at the tracheal anastomotic site. The patient was discharged at 31st postoperative day, and has been regularly followed up at outpatient clinic without specific complication. The follow-up bronchoscopy, performed 2 weeks and 4 months after surgery, revealed no evidence of cellular rejection.

• Childhood Kidney Diseases
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• v.11 no.1
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• pp.106-111
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• 2007

[ $Henoch-Sch\ddot{o}nlein$ ] Purpura(HSP) is a form of vasculitis that typically affects small arteries in the skin, joints, intestinal tract and kidneys. It usually resolves spontaneously but sometimes can cause serious problems in the kidneys and intestinal tract. A 6-year-old girl with purpura, arthralgia and abdominal pain for 2 weeks was admitted. She also showed gross hematuria, generalized edema and decreased urine output. Blood pressure was in the upper normal range. Initial laboratory findings showed hypoalbuminemia, hyperlipidemia, microhematuria and nephrotic-range proteinuria(27.2 g/day). Initially, she was treated with pulse methylprednisolone, azathioprine, albumin and furosemide. Her renal biopsy revealed diffuse mesangial proliferation with strong IgA deposition. There were no crescents. On the third hospital day, she complained of severe abdominal pain and free peritoneal air was seen on abdominal X-ray. Primary repair of small bowel was performed and two pin-point sized holes were found. One week later, she still showed heavy proteinuria. Therefore, we added an ACE inhibitor and dipyridamole, and changed azathioprine to cyclosporine. One month later, the urine protein/creatinine ratio was decreased to 17.8 from 57, but heavy proteinuria has been still persisted. Here we report a rare case of a patient with HSP who had both severe nephrritc-nephrotic syndrome and small bowel perforation.

• Journal of Veterinary Clinics
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• v.23 no.2
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• pp.175-179
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• 2006

A 7-year-old, 3.16 kg intact male Yorkshire terrier had the history of abdominal distension, diarrhea, and weight loss. On the basis of history takings, physical examination, laboratory tests, radiography, ultrasonography, exploratory laparotomy, and histopathological examination, the dog was diagnosed definitely as intestinal lymphangiectasia. In this case, signs and results are consistent with those of other reports, but some clinicopathological findings such as hypocalcemia and hypocholesterolemia are not. This means that the findings were not completely in accord with the typical ones of intestinal lymphangiectasia in this dog. Prednisolone was prescribed to treat. However, it was not effective sufficiently. Thus, azathioprine was added to the regimen used in the first trial, and it was quite efficient in inducing remission in intestinal lymphangiectasia. The clinical signs were improved to the combined therapy. This case report demonstrates that the combined therapy for intestinal lymphangiectasia can be used as an alternative to only glucocorticoid therapy.

• Korean Journal of Head & Neck Oncology
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• v.10 no.1
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• pp.7-12
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• 1994

The clinical features and treatment outcomes of 20 patients with Kimura's disease treated from 1981 to 1993 were analyzed to determine proper therapeutic modalities. The mean age was 36.3 yrs old (range l4-53yrs) and the male to female ratio was 1.5:1 (male:female=12:8). Among 20 patients, 13 had multiple lesions and the remaining 7 had single lesion. Almost all lesions were found in the head and neck area(41 lesions) and only 7 in the other sites. The initial treatment modalities were excision, excision with immunotherapy, radiotherapy with immunotherapy, or immunotherapy(steroid, azathioprine). Among 17 patients who could be followed up, a recurrent or persistent disease was found in 13 patients. The retreatment modalities for patients with recurrent lesions were excision, excision with immunotherapy, or immunotherapy. The retreatment outcomes were also disappointing. The proper therapeutic modality of the Kimura's disease is not established yet, but the radiotherapy after excision or immunotherapy seems to be more effective than others.

• Journal of Chest Surgery
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• v.26 no.3
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• pp.224-227
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• 1993

We experienced one case of orthotopic cardiac transplantation in a patient with end stage dilated cardiomyopathy. This 50 year-old female recipient was suffered from NYHA functional class IV cardiac failure and dependent upon intravenous inotropic support for 2 months [recipient category 1]. Her preoperative condition was grave with left ventricular ejection fraction of 20% and estimated systolic pulmonary arterial pressure [from Doppler study] was 50mmHg. The brain-dead donor was 31 year-old male with head trauma. The body sizes [weight, height] of the donor/recipient were 70 Kg, 165 cm / 43 Kg, 160 cm and appropriately overmatched. Preoperatively, identical ABO/Rh blood group [A+] and nonreacting HLA crossmatching were confirmed. On November 11 1992 cardiac transplantation was performed without complication. Multiple organ procurement team and heart transplantation team were organized the operation schedule appropriately to minimize the ischemic time. The pump time was 126 minutes and aortic crossclamping time of recipient heart was 73 minutes and, as a result, total ischemic time of the transplanted heart was 75 minutes. Postoperatively, the vital signs were stable with minimal inotropic support. The immunosuppressive therapy was commenced from preoperatively and cyclosporine, azathioprine, and corticosteroid were used as a combination therapy as scheduled and monitored with blood drug concentration, WBC count, renal function and most importantly regular endomyocardial biopsy.Now, 5 months after transplantation, the patient is in NYHA functional class II with minimal cardiac drug support.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.215-220
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• 2007

We present a case of autoimmune chronic pancreatitis in a previously healthy child without any history of autoimmune disease. A 12-year-old boy was admitted to the hospital with abdominal pain. The serum amylase, lipase, and IgG levels were elevated and autoantibodies (antinuclear antibody, antineutrophil antibody) were detected. An abdominal CT (computed tomographic) scan revealed diffuse enlargement of the pancreas. ERCP (endoscopic retrograde cholangiopancreaticography) demonstrated an irregular stricture of the main pancreatic duct in the pancreas tail. After two years of oral steroid and immunosuppressive drug therapy, the clinical, laboratory and radiological findings were improved. The patient has been symptom-free for 18 months after the discontinuation of medication.

• Journal of Chest Surgery
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• v.33 no.7
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• pp.585-589
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• 2000

Lung transplantation has been successfully employed for variety of obstructive lung disease. Single lung transplantation has become a therapeutic option for end-stage obstructive lung disease. The patient, a 57 year old man with emphysema, suffered from severe dyspnea, which progressively aggravated him for the last three years. A single lung transplantation was performed from a young brain-dead donor on April 7th, 1999 in the department of thoracic surgery, Respiratory Center, Yongdong Severance hospital, yonsei University. The immunosuppressive regimen was based on cyclosporine A and azathioprine from beginning, adding steroid. Single lung transplantation was feasible and beneficial in patients with end-stage emphysema.