• Childhood Kidney Diseases
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• v.11 no.1
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• pp.118-125
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• 2007

Wegener's granulomatosis(WG) is a necrotizing granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys. The disease usually manifests in adults between 25 and 50 years of age, but it can also rarely occur-in childhood with some features different from those of adults. WG may be easily overlooked in young patients by misinterpretion of the symptoms as caused by an infectious disease of the respiratory tract. Delayed diagnosis and treatment of the disease may cause more rapid progression of the glomerulonephritis to end stage renal disease. We report a boy who was diagnosed with WG with involvement of multiple organs at 13 years of age.

• Clinical and Experimental Pediatrics
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• v.61 no.10
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• pp.327-331
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• 2018

Purpose: The incidence of inflammatory bowel disease (IBD) is rapidly increasing, and several reports have described the renal complications of IBD. We sought to evaluate the clinical manifestations of renal complications in children with IBD in order to enable early detection and prompt treatment of the complications. Methods: We retrospectively reviewed the medical records of 456 children and adolescents aged <20 years who had been diagnosed with IBD since 2000. We analyzed patient age, sex, medication use, IBD disease activity, and clinical manifestations of renal symptoms. Results: Our study comprising 456 children with IBD included 299 boys (65.6%) and 157 girls (34.4%). The study included 346 children with Crohn disease and 110 children with ulcerative colitis. The incidence of kidney-related symptoms was 14.7%, which was significantly higher than that in normal children. We observed 26 children (38.8%) with isolated hematuria, 30 children (44.8%) with isolated proteinuria, and 11 children (16.4%) with hematuria and concomitant proteinuria. A renal biopsy was performed in 7 children. Histopathological examination revealed immunoglobulin A nephropathy in 5 children (71.4%). All children presented with mild disease and well-controlled disease activity of IBD. Conclusion: Children with IBD are more likely to show kidney-related symptoms than healthy children and adolescents are. Therefore, regular screening of urine and evaluation of renal function in such children are necessary for early detection of renal complications.

• Childhood Kidney Diseases
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• v.12 no.1
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• pp.88-92
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• 2008

Infection of Epstein-Barr virus(EBV) gives rise to a broad spectrum of clinical manifestations in children. Although renal involvement is rare, diverse renal manifestations are known from hematuria to acute renal failure. Secondary membranous nephropathy(MN) associated with systemic EBV infection is an uncommon renal pathology and only two cases have been reported. We are adding another case of MN associated with EBV infection in a child. An 8-year-old girl was admitted for renal biopsy. She had been followed up for microscopic hematuria and intermittent proteinuria for 5 months. There had been no specific findings in serology and radiology. Tonsil biopsy had been done due to exudative tonsillar hypertrophy and enlarged multiple cervical lymph nodes. And it showed EBV-associated lymphoproliferative findings. Serologic tests for EBV showed positive evidence of recent infection; viral capsid antigen(VCA) IgM was borderline positive, VCA IgG and early antigen IgG were positive, and EB nuclear antigen IgG was negative. In Situ Hybridization of tonsil for EBV mRNA was positive. Because her proteinuria and hematuria were aggravated at that time(protein 3 +, RBC >60/HPF), renal biopsy was done. Renal biopsy showed the findings of MN, characterized by thickened capillary walls with epimembranous spikes on light microscopy and subepithelial, mesangial and subendothelial electron dense deposits on electron microscopy. On immunofluorescence microscopy, IgG, C1q, kappa and lambda chains were positive. After steroid administration, proteinuria and hematuria resolved gradually within 6 months.

• Tuberculosis and Respiratory Diseases
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• v.66 no.2
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• pp.116-121
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• 2009

Background: Tsutsugamushi, leptospirosis and hemorrhagic fever with renal syndrome (HFRS) are the prevalent diseases among the acute febrile illnesses in Korea. Pulmonary involvement in the patients with these diseases remains poorly recognized in endemic regions, and this is despite reports of recent outbreaks and epidemic episodes. Pulmonary involvement and a higher CRP level as clinical manifestations show a more severe form of infection. The aim of this study is to analyze the correlation of pulmonary involvement and the CRP level in patients with acute febrile illnesses. Methods: We retrospectively reviewed the clinical records of 105 patients who were diagnosed with tsutsugamushi, HFRS and leptospirosis from January 2002 to May 2008 in Chuncheon Sacred Heart Hospital. The radiographic images were retrospectively analyzed by two radiologists. We analyzed the pulmonary complications of the patients with these febrile diseases and we checked the CRP level at admission. Results: The study included 105 patients who were diagnosed with febrile diseases. Of these patients, 32 patients had hantaan, 10 patients had leptospirosis and 63 patients had tsutsugamushi disease. 42 (40%) patients had pulmonary complications, 20 patients had pulmonary edema, 20 patients had pleural effusion and 2 patients had interstitial pneumonitis. The patients with pulmonary involvement showed a more severe form of infection and a higher CRP level than that of those patients without pulmonary involvement (p=0.0073). Conclusion: Pulmonary involvement in patients with acute febrile diseases might be correlated with a higher CRP level. Identification of this factor on admission might provide useful selection criteria for the patients who need early intensive care.

• Clinical and Experimental Pediatrics
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• v.48 no.4
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• pp.448-452
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• 2005

Neurofibromatosis(NF) is an autosomal dominant disorder, probably of neural crest origin that affects all three germinal layers. It is a group of heterogeneous multisystemic neurocutaneous disorders involving both neuroectodermal and mesenchymal derivatives. Type 1(von Recklinghausen disease) is the most common neurocutaneous disorder among the eight subtypes. Previous reports showed various involvements in the renal organ. Renovascular hypertension is the most common major manifestation of renal involvement in this disease. However, we experienced a case of ectopic kidney concurrent with neurofibromatosis type 1. The diagnosis of neurofibromatosis had been made by typical skin manifestation on physical examination, and ectopic kidney was discovered accidentally during routine abdominal sonography. The etiological basis of this association is not clear. We report a rare case of coexisting neurofibromatosis and ectopic kidney in a 7-year-old girl with a brief review.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.36 no.2
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• pp.67-72
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• 2014

Necrotizing fasciitis (NF) is defined as rapidly progressive necrosis of subcutaneous fat and fascia. Although NF of the face is rare, its mortality rate is nearly 30%. It usually originates from dental infection and can lead to involvement of the neck, mediastinum, and chest wall. Complications resulting from pre-existing systemic diseases can increase the mortality rate. Known complication factors for NF include diabetes, malnutrition, advanced age, peripheral vascular disease, renal failure, and obesity. Here, we report a case of NF originating from dental infection in an 88-year-old woman already diagnosed with hypertension, thoracic aortic aneurysm, and renal diseases. Such conditions limited adequate surgical and antibiotic treatment. However, interdisciplinary treatment involving multiple departments was implemented with good results.

• Tuberculosis and Respiratory Diseases
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• v.38 no.4
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• pp.389-395
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• 1991

Pulmonary hemorrhage is a feature of several immune and idiopathic disorders. The specific etiology of this disease is determined by clinical, serological and renal pathology. Microscopic polyarteritis nodosa is a vasculitis of small vessels which is characterized by involvement of the lung, kidney and skin in young individuals. Its clinical manifestations differ from classical polyarteritis nodosa. It should be considered in differentiation of recurrent alveolar hemorrhage and hematuria. We reported a case of microscopic polyarteritis nodosa which was confirmed by renal biopsy and positive serum antineutrophil cytoplasmic antibody, associated with recurrent alveolar hemorrhage and hematuria.

• Journal of Korean Neurosurgical Society
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• v.30 no.8
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• pp.1023-1027
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• 2001

Hemangioblastomas are rare benign tumor of the central nervous system that commonly occur in the posterior fossa around the 4th ventricle. In case of von Hippel-Lindau disease, hemangioblastomas involve multiple regions such as cerebellum, spinal cord and brainstem but, rarely show simultaneous involvement of cerebellum and spinal cord. We have experienced a case of multiple hemangioblastomas that were located at the cerebellum, cervical cord and conus medullaris and also had multiple lesions that a part of von Hippel-Lindau disease ; retinal angioma, syringomyelia, multiple cyst on kidney and pancreas, renal cell carcinoma on left kidney. Hemangioblastomas on cerebellum and spinal cord were removed totally, retinal angioma was treated with laser photocoagulation and renal cell carcinoma was also totally excised. The authors report a case of von Hippel-Lindau disease had multiple located hemangioblastomas on cerebellum, cervical cord and conus medullaris with review of literature.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.89-94
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• 1992

A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

• Childhood Kidney Diseases
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• v.17 no.1
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• pp.6-12
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• 2013

Thrombotic microangiopathy (TMA) is a microvascular thrombotic lesion caused by endothelial injury and subsequent formation of platelet rich thrombus. TMA is first described as a classical pathologic feature of HUS/TTP. Renal biopsy finding of TMA represents kidney involvement of HUS/TTP as well as other diseases such as malignant hypertension, drug toxicity, eclampsia, pre-eclampsia, and several systemic infections. Autoimmune diseases and transplant kidney sometime also have TMA. It is needed to consider a complete autoimmune work-up of patients presenting with TMA including tests for ANA, ANCA, and ADAMTS13 inhibitory antibodies, because there are several reports of association with TMA in patients of SLE, anti-phospholipid syndrome, and ANCA-associated vasculitis.