• Journal of the Korean Society of Radiology
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• v.85 no.1
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• pp.109-123
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• 2024

Xanthogranulomatous (XG) inflammatory disease is a rare benign disease involving various organs, including the gallbladder, bile duct, pancreas, spleen, stomach, small bowel, colon, appendix, kidney, adrenal gland, urachus, urinary bladder, retroperitoneum, and female genital organs. The imaging features of XG inflammatory disease are nonspecific, usually presenting as a heterogeneous solid or cystic mass. The disease may also extend to adjacent structures. Due to its aggressive nature, it is occasionally misdiagnosed as a malignant neoplasm. Herein, we review the radiological features and clinical manifestations of XG inflammatory diseases in various organs of the abdomen and pelvis.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.237-244
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• 2005

Membranoproliferative glomerulonephritis (MPGN) is a progressive primary glomerulonephritis characterized by mesangial proliferation with increased mesangial matrix, subendothelial immune deposits, mesangial interposition and a double contour feature of the glomerular basement membrane. The glomerular involvement in MPGN is usually diffuse, however, cases of focal or segmental MPGN have been reported by several authors. We report a case of focal segmental MPGN with prolonged hypocomplementemia for ,3 years in a 5 years old girl. (J Korean Soc Pediatr Nephrol 2005;9:237-244)

• Tuberculosis and Respiratory Diseases
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• v.53 no.4
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• pp.463-469
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• 2002

A 49-year old man who had been treated for five months at a private clinic due to chronic paranasal sinusitis was admitted to our hospital because of recently aggravated nasal stuffiness, headache, and cough. The X-ray film of paranasal sinuses and facial CT scan showed marked mucosal thickening of the nasal cavity and paranasal sinuses. The plain chest film and chest CT scan showed multiple, vatiable sized, pulmonary nodules in both lungs. The level of c-ANCA was elevated and urinalysis revealed proteinuria and hematuria. Percutaneous lung and kidney biopsies were performed for confirmative diagnosis. Histologic examination of the lung nodule demonstrated extensive necrosis and poorly-formed granulomatous inflammation. The histologic finding of the kidney showed focal necrotizing glomerulonephritis. A diagnosis of Wegener's granulomatosis involving the paranasal sinuses, lung and kidney was made, and treatment was successfully performed with cyclophosphamide and prednisone.

• Journal of Hospice and Palliative Care
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• v.17 no.4
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• pp.207-215
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• 2014

Multiple bone metastases are common manifestation of many malignant tumors such as lung cancer, breast cancer, prostate cancer and renal cell carcinoma. Bone metastasis is secondary cancer in the bone, and it can lead to bone pain, fracture, and instability of the weight bearing bones, all of which may profoundly reduce physical activity and life quality. Treatment for bone metastasis is determined by multiple factors including pathology, performance status, involved site, and neurologic status. Treatment strategies for bone metastasis are analgesics, surgery, chemotherapy and radiotherapy. External beam radiotherapy has traditionally been an effective palliative treatment for localized painful bone metastasis. However, in some cases such as multiple bone metastases, especially osteoblastic bone metastasis originated from breast or prostate cancer, the radiopharmaceutical therapy using $^{89}Sr$, $^{186}Re$, $^{188}Re$, $^{153}Sm$ and $^{117m}Sn$ are also useful treatment option because of administrative simplicity (injection), few side effects, low risk of radiation exposure and high response rate. This article offers a concise explanation of the radiopharmaceutical therapy for multiple bone metastases.

• Clinical and Experimental Pediatrics
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• v.52 no.3
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• pp.389-391
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• 2009

Antiepileptic drug hypersensitivity syndrome (AHS), a delayed immunological reaction, is a relatively rare side effect of antiepileptic drugs and is usually overlooked. An array of symptoms can occur one to eight weeks after treatment with an antiepileptic drug. Symptoms may be as simple as a fever, skin rash, or lymphadenopathy, but may eventually involve internal organs and cause fatal outcomes. Additionally, because the symptoms resemble the features of various arrays of diseases and the reported mortality rate is approximately 10%, the importance of early diagnosis and ability to differentiate AHS from other diseases cannot be overemphasized. We report a case of a 14-year-old girl with AHS caused by lamotrigine, which mimicked atypical Kawasaki disease and infectious mononucleosis.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.110-115
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• 2008

Malperfusion of a major organ with aortic dissection has various clinical features according branch. The morbidity and mortality rate can increase without suspicion especially postoperative period. Surgical outcomes and prognosis are influenced by early expeditious diagnostic and therapeutic measures are mandatory for successful treatment. The authors report four successful cases of acute aortic dissection with mal perfusion of various organs, such as the brain, kidney, and the lower extremities.

• Tuberculosis and Respiratory Diseases
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• v.43 no.5
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• pp.779-785
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• 1996

Wegener's granulomatosis is characterized by necrotizing granulomatous vasculitis affecting upper and lower respiratory tracts and kidneys. Vascular lesions commonly involve capillaries and small vessels but, less commonly larger vessels. We report a 46-year-old male patient of Wegener's granulomatosis associated with paranasal sinusitis, pulmonary consolidations, glomerulonephritis, skin lesions with obstruction of both anterior tibial and peroneal arteries. Several necrotic lesions of the toes had progressed to gangrene and both transmetatarsal amputations were done. The patient continued to receive cyclophosphamide and prednisolone.

• Tuberculosis and Respiratory Diseases
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• v.64 no.5
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• pp.369-373
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• 2008

Tuberous sclerosis (TS) is an autosomal dominant disorder that is characterized by cutaneous lesions, seizures, mental retardation and hamartomas in various organs including the skin, kidney and brain. Pulmonary involvement is extremely rare, and occurs in approximately 0.1 to 1% of TS cases. Recent reports have indicated multiple micronodular pneumocyte hyperplasia (MMPH) as another rare form of pulmonary involvement of tuberous sclerosis. We report a case of a 35 year-old-male patient who had no pulmonary symptoms but showed multinodular pulmonary shadows on his chest CT scan. The patient was finally diagnosed with TS with MMPH of the lung. MMPH does not appear to have any malignant potential but the clinical significance of MMPH in TS patients is unknown.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.24 no.1
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• pp.17-25
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• 2024

Fabry disease (FD) is an X-linked lysosomal storage disorder. It is caused by mutations in the α-galactosidase A gene, which results in deficient or absent activity of α-galactosidase A (α-Gal A). This leads to a progressive accumulation of globotriaosylceramide (Gb3) in various tissues. Manifestations of Fabry disease include serious and progressive impairment of renal and cardiac function. In addition, patients experience pain, gastrointestinal disturbance, transient ischaemic attacks, and strokes. Additional effects on the skin, eyes, ears, lungs, and bones are often seen. Reduced life expectancy and deadly consequences are being caused by cardiac involvement. Chaperone therapy or enzyme replacement therapy (ERT) are two disease-specific treatments for FD. Thus, early detection of FD is critical for decreasing morbidity and mortality. Globotriaosysphingosine (lyso-Gb3) for identifying atypical FD variants and highly sensitive troponin T (hsTNT) for detecting cardiac involvement are both significant diagnostic indicators. This review aimed to offer a basic resource for the early diagnosis and update on the diagnosis of having FD. We will also provide a general diagnostic algorithm and information on ERT and its accompanying treatments.

• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.884-890
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• 2002

Purpose : Henoch-Schonlein purpura(HSP) nephritis has been reported to vary from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the Insertion/Deletion(I/D) polymorphism of angiotensin converting enzyme(ACE) gene with clinical manifestations, particularly proteinuria in children with HSP nephritis, compared with that in HSP. Methods : ACE gene polymorphism was determined in children with HSP nephritis(n=33) and HSP(n=28) who were diagnosed in Busan Paik hospital from January 1996 to June 2001. The I/D polymorphism of ACE gene was determined by PCR amplication of genomic DNA. Results : The ACE I/D genotype frequency was DD : 25%, ID : 50%, II : 25% in HSP and DD : 24 %, ID : 46%, II : 30% in HSP nephritis, there was no significant difference in the genotype and allele frequencies between two groups. When statistical analysis was done according to the presence of D allele, the amount of 24-hour urinary protein excretion and the incidence of moderate to heavy proteinuria(>$500mg/m^2/day$) at onset and last follow-up were higher in DD/ID genotype than in those in II genotype, but these differences were not statistically significant. Conclusion : We suggest a lack of association between I/D polymorphism of ACE gene and clinical manifestations in children with HSP nephritis. However, further follow-up studies based on a sufficient number of patients and long term follow up periods are necessary to confirm the role of I/D polymorphism of ACE gene in children with HSP nephritis.