PURPOSE: To describe the associated conditions and clinical significance of lenticulostriate uasculopathy (LSV) as demonstrated by cranial sonography. MATERIALS AND METHODS: We retrospectively studied 77 LSV cases who between January 1994 and January 1996 had undergone cranial sonography for neonatal asphyxia, seizure, or bulging of anterior fontanel. Cranial sonography was performed with 7 MHz real-time linear and sector transducers using an Acuson computed sonography unit ; examinations were performed in sagittal and coronal planes. Twenty-six LSV patients underwent color Doppler studies, and in 24 of these, color signal and arterial pulse spectral wave were detected. LSV was grouped as one of three types, according to echogenicity : type I (less echogenic than sylvian fissure), type II (similar to sylvian fissure), and type III (more echogenic than sylvian fissures). We retrospectively evaluated associated sonographic abnormalities of the brain, and reviewed medical records for associated conditions and neurologic sequelae. Follow-up sonographic examinations were performed in 23 patients. RESULTS: There were 56 type I cases (73 %), 17 of type II (22 %) and 4 of type III (5 %). Cranial sonographic results were normal in 36 cases (47 %) and abnormal in 41(53 %). Forty-eight cases of nonspecific causes and 29 cases of perinatal and acquired causes accounted for associated conditions. The echogenicity of LSV had not changed in 21 of 23 follow-up cases. Neurologic examinations were performed in 18 cases and in 14 of these (78 %) there was no neurologic sequela. CONCLUSION: LSV in basal ganglia may be associated with nonspecific neonatal conditions, in addition to well known perinatal causes.
Asphyxia ; Basal Ganglia ; Basal Ganglia Cerebrovascular Disease* ; Brain* ; Cranial Fontanelles ; Follow-Up Studies ; Humans ; Medical Records ; Neurologic Examination ; Retrospective Studies ; Seizures ; Transducers ; Ultrasonography*

Bednar tumor was described by Bednar in 1957. The histologic pattern of this tumor shows similar to dermatofibrosarcoma protuberans (DFSP) but melanin pigments are scattered within the tumor, It's rare neoplasm accounting for approximately 1-5% of all case of DFSP. Clinically, this tumor is considered to be intermediated malignancy, because of slow growth and frequent local recurrence and lack of distant metastasis. The majority are located on the trunk and the upper and lower extremities, but extremely rate in the head and neck area. Microscopically, this tumor is characterized by tight storiform spindle cells and long slender cells that admixed with a small population of melanin containing dendritic cells. This dendritic cells are the primary features distinguising this lesion from conventional DFSP. Complete surgical excision and close follow-up case are necessany for this neoplasm because of probable intermediate malignancy. A patient was admitted to Our Department of Oral and Maxillofacial Surgery due to swelling on right parotid area and numbness of the right lower lip on September, 1994, By clinical examinations and C-T finding, we dignosed tentatively as myxoma or pleomorphic adenoma. Surgical excision of this tumor was performed with parotidectomy and mandibular osteotomy under the frozen biopsy. By final microscopic and electromicroscopic examination and immunohistochemical study, this tumor was diagnosed as Bednar tumor. So, we report a case of pigmented DFSP with review of literatures.
Adenoma, Pleomorphic ; Biopsy ; Dendritic Cells ; Dermatofibrosarcoma* ; Follow-Up Studies ; Head ; Humans ; Hypesthesia ; Lip ; Lower Extremity ; Mandibular Osteotomy ; Melanins ; Myxoma ; Neck ; Neoplasm Metastasis ; Recurrence ; Surgery, Oral

The new onset of acanthosis nigricans in an adult-especially when the lesions appear on mucosal membranes such as the lips, periocular areas, and anus-may be indicative of an underlying tumor. Adenocarcinoma of the gastrointestinal tract is the most common malignancy-most often cancer of the stomach. A 72 years old female had disseminated, symmetrically distributed, brownish black pigmentation and papillary hypertrophy on the intertriginous and flexural areas for 12 months, and gastric adenocarcinoma was confirmed during the evaluation of internal malignancy. The cutaneous finding of acanthosis nigricans was a significant cutaneous marker of internal malignancy in this case.
Acanthosis Nigricans* ; Adenocarcinoma* ; Aged ; Female ; Gastrointestinal Tract ; Humans ; Hypertrophy ; Lip ; Membranes ; Pigmentation ; Stomach Neoplasms

No abstract available.
Humans ; Referral and Consultation*

Benzbromarone is a uricosuric agent for hyperuricemia and gout. Some of its well-known side effects include hypersensitivity, renal calculi, and gastrointestinal problems. Although the drug was withdrawn from U.S. market due to severe hepatotoxicity, it is still available in some countries including Korea. We describe a 19-year-old male who was admitted with general weakness and azotemia after use of benzbromarone. A kidney biopsy revealed acute tubular necrosis without an evidence of urate nephropathy. After discontinuation of benzbromarone, the renal function returned to baseline. This is the first case of acute tubular necrosis associated with benzbromarone use.
Azotemia ; Benzbromarone ; Biopsy ; Gout ; Humans ; Hypersensitivity ; Hyperuricemia ; Kidney ; Kidney Calculi ; Korea ; Male ; Necrosis ; Transplants ; Uric Acid ; Young Adult

Takayasu arteritis (TA) is a chronic systemic inflammatory disease that most commonly affects the aorta and its major branches. TA-induced renal artery stenosis (TARAS) can result in malignant hypertension, severe renal dysfunction, cardiac decompression and premature death. Surgical management for TARAS has been proved to be effective and safe, especially in the medically or interventionally-intractable cases. We report here on a 39-year-old patient with recently deteriorating hypertension and renal function because of a recurred RAS, for which he underwent "y-shaped" aorto-birenal reconstruction surgery 20 years ago. CT angiography showed bilateral diffuse calcified stenosis in the previous renal graft and there was a partial rupture of a pseudoaneurysm in the suprarenal abdominal aorta segment between the celiac axis and the renal graft. We performed descending thoracic aorto-abdominal aortic bypass together with a graft-renal bypass and exclusion of the pseudoaneurysm. The renal function was normalized and the blood pressure became stable without any antihypertensive medication. He recovered and was discharged without any complications.
Adult ; Aneurysm, False ; Angiography ; Aorta ; Aorta, Abdominal ; Axis, Cervical Vertebra ; Blood Pressure ; Constriction, Pathologic ; Decompression ; Humans ; Hypertension ; Hypertension, Malignant ; Hypertension, Renovascular ; Mortality, Premature ; Renal Artery Obstruction ; Rupture ; Takayasu Arteritis ; Transplants

No abstract available.
Adenocarcinoma* ; Myxoma*

Background: Post-transplant cancer (PTC) is a critical complication after kidney transplantation. However, whether successfully cured PTC affects the long-term graft outcome remains unclear. Methods: We retrospectively reviewed 1,629 kidney transplant recipients from 1995 to 2017 after excluding patients with post-transplant hematologic or advanced non-curable cancers and who underwent allograft nephrectomy because of cancer. Cured PTCs were defined as cancers treated with curative methods and/or adjuvant therapy without recurrence during ≥ 2 years. Propensity score matching was performed to match cured PTC patients with cancer-naïve patients (i.e., non-PTC group). Results: During the median period of 7 years (maximum, 23 years), 70 patients (4.3%) had cured PTCs. The PTC group showed significantly higher risks of death-censored graft failure (adjusted hazard ratio [HR], 2.56 [1.05–6.23]), class II donor-specific antibodies (adjusted HRs, 3.37 [1.30–8.71]), estimated glomerular filtration rate < 30 mL/min/1.73 m2 (adjusted HR, 2.68 [1.43–5.02]) and random urine protein/creatinine ratio > 1 g (adjusted HR, 3.61 [1.92–6.79]) compared to non-PTC group. However, the risk of mortality was not different between the PTC and non-PTC groups. According to the cancer type, only urogenital cancer had a significant association with graft failure (adjusted HR, 4.26 [1.19–15.22]) and the gastrointestinal cancer showed elevated risk of T cell mediated rejection compared to non-PTC (adjusted HR, 20.44 [6.02–69.39]). Conclusion Appropriate monitoring of graft function is necessary in patients with cured PTCs.

Anti-glomerular basement membrane antibody mediated rapidly progressive glomerulonephritis(anti- GBM antibody mediated RPGN) is defined by the clinical picture of renal failure developing over days or weeks and the histological appearance of crescents and linear immune deposits mediated by the circulating autoantibodies. We report a case of anti-GBM antibody mediated RPGN with review of literature. A 59-year-old female patient was admitted to the Chungang Gil Hospital because of fever and acute deterioration of renal function. On admission, hemoglobin was 7.39g/dL, hematocrit was 20.9%, and BUN/Cr were 39.7 and 5.23mg/dL respectively. Urinalysis showed albumin (1+) with many RBCs. Renal biopsy revealed the presence of segmental or circumferential cellular crescents associated with smooth linear staining of glomerular basement membrane with antibody to IgG. High titer of circulating antibody to glomerular basement membrane antigen was demonstrated by the ELISA. High doses of corticosteroid with plasmapheresis were administered, but her renal function was progressively deteriorated.
Autoantibodies ; Basement Membrane* ; Biopsy ; Enzyme-Linked Immunosorbent Assay ; Female ; Fever ; Glomerular Basement Membrane ; Glomerulonephritis* ; Hematocrit ; Humans ; Immunoglobulin G ; Middle Aged ; Plasmapheresis ; Renal Insufficiency ; Urinalysis

Continuous efforts have been made by the organ donation and transplantation community in Korea to increase organ donation by the deceased. The authors detailed trends of organ donation and utilization over the past 10 yr using data provided by the KONOS. The yearly number of deceased donors has grown gradually since 2003. The number and percentage of old donors (> or =50 yr) and donors dying from intracranial hemorrhage has increased continuously. Therefore, the percentage of standard criteria donors (SCD) has been declining significantly, from 94% in 2000 to 79.2% in 2009. The number of organs transplanted per donor (OTPD) has also declined slightly since 2007, from 3.28 in 2007 to 2.95 in 2009. This decline may be attributable to increases in the number and percentage of extended criteria donors (ECD) and donors after cardiac death (DCD), since the OTPD was 2.25 for DCD, 2.5 for ECD, and 3.09 for SCD in 2009. In summary, the makeup of donors has changed significantly. There is an urgent need for establishment of an institutional framework including an independent organ procurement organization and for improvement for the National Transplant Act to increase deceased donor pool and to optimize management of ECD and DCD.
Adult ; Death ; Demography ; Female ; Humans ; Male ; Republic of Korea ; Tissue Donors ; Tissue and Organ Procurement/*trends