No abstract available.
Bile Duct Neoplasms* ; Bile Ducts* ; Bile*

Essential thrombocythemia is one type of the related chronic myeloproliferative disorders that also include poly-cythemia vera, chronic myelogenous leukemia, and idiopathic myelofibrosis. It is a rare disorder of unknown origin characterized by thrombocytosis, excessive megakaryocytes, hemorrhage, and thrombotic complication. Several cases of ischemic stroke in essential thrombocythemia have been reported, but cerebral infarction combined with cerebral hemorrhage has been very rare and has not been reported in Korea. We report a case of cerebral infarction and chronic subdural hematoma in a pateint with essential thrombocythemia. A 59-year-old woman with essential thrombocythemia was admitted with mild left hemiparesis that developed 3 days prior. She had a history of minor trauma 15 days prior. A brain MRI showed an infarction in the right temporal lobe and a chronic subdural hematoma in the right frontoparietal area. A cerebral angiography revealed an occlusion of the M2 portion of the right middle cerebral artery.
Brain ; Cerebral Angiography ; Cerebral Hemorrhage ; Cerebral Infarction* ; Female ; Hematoma, Subdural, Chronic* ; Hemorrhage ; Humans ; Infarction ; Korea ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Magnetic Resonance Imaging ; Megakaryocytes ; Middle Aged ; Middle Cerebral Artery ; Myeloproliferative Disorders ; Paresis ; Primary Myelofibrosis ; Stroke ; Temporal Lobe ; Thrombocythemia, Essential* ; Thrombocytosis

Sebaceous carcinoma (SC) is a rare malignant tumor, with an estimated incidence of approximately 1 to 2 per 1,000,000 per year. Approximately 75% of SCs are reported to occur in eyelids. Most of these tumors are diagnosed at age 40 or over and exhibit a wide variety of patterns in addition to the general appearance previously reported. SC is difficult to diagnose clinically, but can be diagnosed by accompanying biopsy. In cases of SC, additional examinations, such as endoscopy and computed tomography, may be necessary because of its association with Muir-Torre Syndrome. We present the case of a 22-year-old Asian man who complained of a rapidly growing chin tumor. The tumor was treated by surgical resection and adjuvant radiation therapy.

Sebaceous carcinoma (SC) is a rare malignant tumor, with an estimated incidence of approximately 1 to 2 per 1,000,000 per year. Approximately 75% of SCs are reported to occur in eyelids. Most of these tumors are diagnosed at age 40 or over and exhibit a wide variety of patterns in addition to the general appearance previously reported. SC is difficult to diagnose clinically, but can be diagnosed by accompanying biopsy. In cases of SC, additional examinations, such as endoscopy and computed tomography, may be necessary because of its association with Muir-Torre Syndrome. We present the case of a 22-year-old Asian man who complained of a rapidly growing chin tumor. The tumor was treated by surgical resection and adjuvant radiation therapy.

Hidradenitis suppurativa (HS) is a chronic inflammatory condition with an unclear etiopathogenesis that is considered to be a follicular occlusive disease. We present a case of HS that was suspected to have developed as a complication of subdermal excision. A 19-year-old man who had undergone subdermal excision due to osmidrosis presented 7 months after surgery with a persistent painful mass in his left axilla. Despite medical treatment, incision, and drainage, a painful enlarged abscess recurred in the left axilla and was cured completely by deroofing surgery. However, 15 months after subdermal excision, he revisited the hospital because of a painful mass in the right axilla. The patient’s condition met the diagnostic criteria of HS. After several recurrences, a cure was achieved by radical wide excision. Mechanical stress like that associated with subdermal excision is considered to be a possible etiological factor of HS. In addition, pathological changes at the sebofollicular junction allow rupture and leakage of folliculopilosebaceous units upon exposure to mechanical stress, which may result in the aggressive subcutaneous extension of inflammation. We suggest that HS should be considered in patients with a recurrent abscess after subdermal excision, and recommend surgical treatment as a possible option if conservative treatment is clinically ineffective.

The prevalence of right-sided infective endocarditis is increasing with a growing number of intravenous drug abusers. A case of right-sided endocarditis is reported in a 18-year-old female heroin addict. Tricuspid valve vegetation, septic pulmonary emboli and staphyloccus aureus in blood cultures are noted. During antimicrobial therapy, she developed remittent fever and skin rash due to vancomycin. After a change of the antibiotic to teicoplanin, she was freed from fever and discharged in an improved condition.
Adolescent ; Drug Users ; Endocarditis* ; Exanthema ; Female ; Fever ; Heroin ; Humans ; Malaria ; Prevalence ; Pulmonary Embolism* ; Teicoplanin ; Tricuspid Valve* ; Vancomycin

PURPOSE: Liver cell damage after ischemia and reperfusion injury has been a major cause of death after liver surgery. Yet there have been no exact and practical guidelines for assessing liver cell damage after ischemia and reperfusion injury. The aim of this study was to estimate the liver cell viability after ischemia and reperfusion injury. METHODS: A 70% partial liver occlusion model with employing Spraque Dawley Rats was used. The ATP content of the liver tissue, the palmitic acid metabolic rate and the histologic change (H/E, TUNEL stain) were all measured at 30 minute intervals to assess liver cell viability during 120 minutes of ischemia. At 24 hours reperfusion after 30, 60 and 120 minutes ischemia, the same parameters and the AST/ALT level in the blood were measured. RESULTS: The ATP content was decreased below 20% compared to normal liver after ischemia, but there were no significant changes in the histology and the palmitic acid metabolic rate during 120 minutes ischemia. At 24 hours reperfusion after 30, 60 and 120 minutes ischemia, the ATP content was decreased to around 50% in all the groups and the palmitic acid metabolic rate was decreased 90.9+/-2.4%, 80.0+/-5.3% and 79.1+/-7.7%, respectively, compared to the control liver. But histologic change was not as great as the change in the ATP content and the palmitic acid metabolic rate. CONCLUSION: Judging by these results, liver has relatively good tolerance during ischemia, but after reperfusion, the liver showed damage depending on the duration of ischemia. This study might be very helpful as a guide line of liver damage after ischemia and reperfusion in both clinical practice and basic research.
Adenosine Triphosphate ; Animals ; Cause of Death ; Cell Survival* ; In Situ Nick-End Labeling ; Ischemia* ; Liver* ; Palmitic Acid ; Rats* ; Reperfusion Injury* ; Reperfusion*

Hereditory Sensory Autonomic Neuropathy(HSAN) is variable rare disorder. So the classification of HSAN could be somewhat unsettled. There are intermingled overlap variants of HSAN in view of clinical manifestations and pathologic findings. Five types of HSAN have been described by Dyck(1993). Type I is dominantly inherited and affects both myelinated(MFs) and unmyetinated fibers(Ufs). Type II is recessively inherited and nerve biopsies show total absence of MFs but presence of Ufs. Type III is a recessive inherited dysautonomia. Type IV is characterized by insensitive to pain anhidrosis, and mild mental retardation with virtually absence of Ufs. Type V affects small MFs. We report a 5 year-old girl who presented with congenital insensitivity to pain, anhidrosis with mild mental retardation. In sural nerve biopsy, Ufs were virtually absent by electron microscopy. We reconfirmed previous pathologic findings in sural nerve of HSAN IV.
Biopsy ; Child, Preschool ; Classification ; Female ; Hereditary Sensory and Autonomic Neuropathies ; Humans ; Hypohidrosis* ; Intellectual Disability ; Microscopy, Electron ; Pain Insensitivity, Congenital* ; Primary Dysautonomias ; Sural Nerve

Idiopathic torsion dystonia is a syndrome of sustained muscle contraction, frequently causing twisting and repetitive movement, or abnormal posture, which is both hereditary or sporadic. It is also classified by age of onset(childhood, adult, juveni1e) and distribution(generalized, focal, multifocal, segmental or hemidystonic). Its anatomical and biochemical pathophysiology are not exactly known yet, but high dosage anticholinergics are known to reduce the dystonic movement and posture in majority. We present a case of idiopathic torsion dystonia who has a family history of same syndrome, with review of literature.
Adult ; Cholinergic Antagonists ; Dystonia Musculorum Deformans ; Humans ; Muscle Contraction ; Posture

Despite the potential importance of the human regulator of calcineurin 1 (RCAN-1) gene in the modulation of cell survival under stress, little is known about its role in death-inducing signal pathways. In this study, we addressed the effects of RCAN1.4 knockdown on cellular susceptibility to apoptosis and the activation of death pathway proteins. Transfection of siRNAs against RCAN1.4 resulted in enhanced Fas- and etoposide-induced apoptosis, which was associated with increased expression and translocation of Bax to mitochondria. Our results suggest that enhanced expression and activation of p53 was responsible for the upregulation of Bax and the increased sensitivity to apoptosis, which could be reversed by p53 knockdown. To explain the observed upregulation of p53, we propose a downregulation of the ubiquitin ligase HDM2, probably translationally. These findings show the importance of appropriate RCAN1.4 expression in the modulation of cell survival and reveal a link between RCAN1.4 and p53.
Aluminum Hydroxide ; Apoptosis ; Calcineurin ; Carbonates ; Cell Survival ; Down-Regulation ; Humans ; Mitochondria ; Proteins ; RNA, Small Interfering ; Signal Transduction ; Transfection ; Ubiquitin ; Up-Regulation