• Journal of Yeungnam Medical Science
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• v.11 no.2
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• pp.221-229
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• 1994

Tuberous sclerosis is reported rarely and is associated with systemic lesions including central nervous system, skin, heart, eye and kidney. Approximately 5-15% of individuals with tuberous sclerosis will develope brain neoplasia, almost invariably subependymal giant-cell astrocytoma (SGCA). We experienced a case of SGCA with tuberous sclerosis operated by the transcallosal approach and report with literature review.

• Journal of Chest Surgery
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• v.30 no.2
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• pp.205-208
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• 1997

Cardiac rhabdomyomas are the moil common primary tumor in infancy and childhood and are frequently associated with tuberous sclerosis. Although spontaneous regression of cardiac rhabdomyoma has been reported, prognosis of this tumor associated with subaortic stenosis is still considered to be poor and surgery continues to be indicated. A 4-day-old female was admitted due to tachypnea and cyanosis. Single rhabdomyoma arising from the interventricular septum associated with severe subaortic stcnosls as partially removed under cardiopulmonary bypass. Excised tumor sizc was 0.7$\times$0.9$\times$0.4cm in dimension. The postoperative course was uneventful and the infant discharged on the 14th postoperative day.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.232-239
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• 2002

Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.

• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1363-1366
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• 2006

Aneurysmal dilatation of the ductus arteriosis has been considered a rare but potentially fatal abnormality. The mechanism of ductal aneurysmal formation remains uncertain. Plain chest radiography has proven helpful in the diagnosis of ductus arteriosus aneurysm (DAA), before the application of transthoracic echocardiography. The transthoracic echocardiography is an important tool for the diagnosis and follow-up of DAA. We present a case of congenital ductus arteriosus aneurysm in a newborn, that was an incidental discovery. The diagnosis was made by echocardiography, three-dimensional surface rendering computed tomography (CT), and spontaneous regression after four weeks of follow-up.

• Journal of Korean Neurosurgical Society
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• v.29 no.4
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• pp.564-568
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• 2000

The authors present a rare case of subependymal giant cell astrocytoma which successfully treated with endoscopic tumor resection. A 15-year-old boy was presented with multiple episode of generalized seizure and intermittent headache for 5 years. The patient had facial adenoma sebaceum, multiple nevi on his back, and mental retardation. Cerebral MRI scans showed a round tumor located at the frontal horn of right ventricle near the foramen monro. The tumor was totally removed with endoscopic procedure and histologically confirmed as a subependymal giant cell astrocytoma. Subependymal giant cell astrocytomas can cause all the clinical problems that are associated with other types of intracranial tumor. However, surgical indication is not well standardized, because of the lack of basic knowledge about the tumor and the likelihood of surgical morbidity. We believe that endoscopic surgery, a minimally invasive technique, can circumvent the surgical disadvantage while achieving the surgical goal.

• Archives of Plastic Surgery
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• v.35 no.3
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• pp.341-344
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• 2008

Purpose: Tuberous sclerosis is an autosomal dominant multisystemic neurocutaneous syndrome characterized by the development of multiple hamartoma distributed through the body, skin, brain, heart, kidney, and lung. The classic triad is seizure, mental retardation, and facial angiofibroma. We experienced a case of a tuberous sclerosis associated with the facial lesion and multiple masses on scalp, forehead, and right lower extremity. Methods: This a 34-year-old male patient had subependymal giant cell astrocytoma in brain and multiple angiomyolipoma in both kidneys. Tangential excision with razor blade and dermabrasion were done on the centrofacial area. We excised other lesions and the mass on scalp was excised and covered with split thickness skin graft. Results: The histopathological finding revealed that the facial lesion was angiofibroma and the others were multiple fibroma. Conclusion: In our case of tuberous sclerosis, we chose the tangential excision to remove the large nodules of angiofibroma, and then dermabrasion was used to smooth the final contour. The patient appeared to have a good results from this treatment modality. But, tuberous sclerosis is an disease that needs long term follow-up to check up the recurrence of skin problem.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.117-124
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• 2001

Purpose : Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulosclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid- dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. Methods : We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy Period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methylprednisolone, often in combination with oral cyclophosphamide for 82 weeks. Results : In all the 10 children with SD-MCNS, complete remission was visible on average of $18{\pm}9$ days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was $2.1{\pm}1.0$ relpases/year, which was reduced to $1.4{\pm}0.9$ relapses/year during MP therapy(P>0.05) and rose to $2.7{\pm}1.0$ relapse/year after MP therapy. Of the 6 children with FSGS, 4 children($67\%$) showed complete remission, of whom 3 children($50\%$) remained in the remission status during the follow up period, $1.2{\pm}0.7$ years, after the end of MP therapy. 2 children($33\%$) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, $1.7{\pm}0.6$ years The only side effect of MP therapy was transient hypertension in 10 children of ail subjects during the intravenous infusion of methylprednisolone. Conclusion : We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome. (J Korean Soc Pediatr Nephrol 2001 ; 5 : 117-24)

• Tuberculosis and Respiratory Diseases
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• v.55 no.6
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• pp.589-596
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• 2003

Background : The solitary pulmonary nodule(SPN) presents a diagnostic dilemma to the physician and the patients in the our nation with high incidence of tuberculoma. We could not exclude whether the SPN was benign or malignant by the change of the size at chest radiograph and findings of chest CT. Recently, positron emission tomography(PET) have been tried as the differential diagnostic method of SPN. We evaluated the efficacy of PET for differentiating malignant from benign SPN and the relationship between standardized uptake values(SUV) of PET and serum glucose. Method : Between January 2001 and July 2002, sixty-one patients with pulmonary nodule were examined by the chest CT and PET. The SPN has been finally diagnosed by the transthorasic needle aspiration and biopsy, bronchoscopic biopsy, and open lung biopsy. Results : Forty eight patients had a malignant nodule(23 squamous cell lung carcinoma, 16 adenocarcinoma, 9 small cell lung cancer) and thirteen patients had a benign nodule(3 tuberculoma, 9 inflammatory granuloma, 1 cryptococcosis). The mean size of malignant and benign nodule was 40.6 mm and 20.0 mm, respectively. All malignant nodules showed a marked increase in 18 fluorodeoxyglucose (FDG) uptake. Mean SUV of malignant was $9.52{\pm}5.20$ and benign nodule was $1.61{\pm}3.60$. There were false positive cases with an increase in 18-FDG uptake (2 tuberculoma, 1 inflammatory granuloma). The SUV of malignant nodule in diabetes patients has no difference in non diabetes patients($9.10{\pm}4.51$ vs $9.65{\pm}5.46$). The sensitivity and specificity of the PET scan for SPN were 100%, 77%, respectively. The positive and negative predictive values were 94% and 100%. Conclusion : PET scanning showed highly accurate result in differentiating the malignant and benign SPN. There were no significant differences between the SUV and serum glucose in the patients with lung cancer.

• Tuberculosis and Respiratory Diseases
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• v.49 no.2
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• pp.246-252
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• 2000

Lymphangioleiomyomatosis(LAM) is a rare disease of unknown etiology that occurs mainly in woman in her reproductive age. We recently experienced two cases of bilateral diffuse cystic lesion of the lung on chest X-ray and HRCT. The first case, a 26-year-old female, who had been diagnosed with tuberous sclerosis by the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to a recently developed hemoptysis. Chest PA showed diffuse ground-glass opacity with radiolucent cystic lesions of various sizes on both lung fields. HRCT showed innumerable small cystic lesions with suspicious diffuse ground-glass opacity on both lung fields The second case, a 30-year-old fema1e was admitted due to dyspnea and spewing of blood-tinged sputum for 2 weeks, shortly after delivery. Chest PA showed diffuse reticular and ground-glass opacities on both lung field. HRCT showed multiple well-defined and relatively uniform size air cysts with a uniform wall thickness on entire both lung fields, with small amount of right pleural effusion. By thoracoscopic lung biopsy she was diagnosed with pulmonary lymphangioleiomyomatosis. We report these cases with a brief review of the literatures.

• Journal of the Korean Orthopaedic Association
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• v.54 no.2
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• pp.192-196
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• 2019

Iatrogenic calcinosis cutis is due to the intravenous administration of calcium gluconate or calcium chloride to treat hypocalcemia. The arthors report three cases of calcinosis cutis with calcifications involving the upper or lower extremities in neonates following the extravasation of calcium gluconate. Three neonates, a 2-week-old girl, 4-week-old boy, and a 4-week-old girl, were consulted for indurated nodules after the intravenous administration of calcium gluconate at the intensive care unit. Complete remission of palpable nodule and calcification was observed on the radiograph at three weeks, four weeks and six months after the initial presentation in each. All three neonates with iatrogenic calcinosis curtis were resolved spontaneously without functional and cosmetic complications. According to enhancement of the patient's cognition about benign disease, a suitable explanation of the disease and avoiding unnecessary treatment through an early diagnosis of iatrogenic calcinosis cutis will reduce a number of potential medical malpractice disputes.