Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

No abstract available.
Mucous Membrane* ; Pigmentation*

BACKGROUND AND OBJECTIVES: Primary percutaneous coronary intervention (PCI) has been reported to be effective reperfusion therapy for acute myocardial infarction (AMI). In the very thrombotic environment of AMI, primary PCI, with heparin-coated stents, has been known to reduce the early reocclusion of the stented vessel by preventing thrombosis. However, little data exist regarding the long-term clinical outcomes. The aim of our study was to evaluate the safety, feasibility and long-term efficacy of heparin-coated stents in AMI. SUBJECTS AND METHODS: Between January 1998 and July 2002, primary PCI with heparin-coated stents was performed in 132 consecutive patients (98 males, with a mean age of 56.3+/-0.7 years) admitted with the diagnosis of AMI within 12 hours from the onset of the chest pain. Major adverse cardiac events (MACE), including death, MI, TLR (target lesion revascularization) and CABG, were recorded during hospitalization and the follow-up period. Angiograms were obtained at the baseline, after stent implantation and at 6 months following implantation. RESULTS: The angiographic and procedure success rate was 96.2%. During hospitalization, there was no evidence of reocclusion of stented vessel, but 1 patient underwent a repeat PCI due to dissection. There were no bleeding complications. A six-month angiographic follow-up was completed in 47.2% of eligible patients and binary restenosis was present in 20.1%. During the long-term clinical follow-up (mean follow-up period 37.2+/-7.2 months), there were 12 deaths, 1 myocardial infarction and 18 TLR. The MACE free survival rate was 76.5%. CONCLUSION: Primary PCI, with heparin-coated stents, shows favorable long-term clinical outcomes.
Chest Pain ; Diagnosis ; Follow-Up Studies ; Hemorrhage ; Hospitalization ; Humans ; Male ; Myocardial Infarction* ; Percutaneous Coronary Intervention ; Reperfusion ; Stents* ; Survival Rate ; Thrombosis

Spontaneous rupture of the bile duct is an exceedingly rare and usually recognized fatal cause of peritonitis and intraabdominal sepsis. Several reports of isolated cases have demonstrated its rarity in the absence of operative trauma or severe injury. Perforation of the biliary system occurs most frequently in the gall bladder, which perforation of the hepatic duct is less common, being described mainly in infants, but extremely rarely in adults. The diagnosis of the spontaneous rupture of the bile duct depends on meticulous clinical examination and imaging investigation. The basic principles of treatment are to stop the bile leakage, evacuate the extravasated bile, apply decortication of the encapsulated fluid collection and provide adequate drainage. We report on a patient with spontaneous perforation of the right hepatic duct who presented with abdominal pain, jaundice and fever. Exploratory surgery showed proximal common bile duct obstruction with right hepatic duct perforation.
Abdominal Pain ; Adult ; Bile ; Bile Ducts ; Biliary Tract ; Common Bile Duct ; Diagnosis ; Drainage ; Fever ; Hepatic Duct, Common* ; Humans ; Infant ; Jaundice ; Peritonitis ; Rupture, Spontaneous* ; Sepsis ; Urinary Bladder

PURPOSE: A transurethral resection of the prostate (TURP) is the gold standard surgical treatment for men with benign prostate hyperplasia (BPH), but 10% of patients shows no improvement in voiding symptom following the procedure. We attempted to find if the prostatic calcification and inflammatory cell infiltration could affect the result of TURP in BPH. MATERIALS AND METHODS: 117 BPH patients who had undergone a TURP, performed by one urologist between March 1999 and February 2001, were evaluated. The prostatic volume, and the size and location of the prostatic calcifications were checked preoperatively. When the calcifications were located around the verumontanum or periurethral area they were classified as central, whereas, when they were located peripherally they were classified as peripheral. The IPSS, Qmax and residual urine were measured preoperatively, and at 6 months after the TURP. The tissues obtained from the TURP were evaluated histologically. Slides of the hematoxylin and eosin-stained tissue of each patient were examined from 5 to 15 chips with a light microscope. They were divided into three groups depending on the level of lymphocyte infiltration; mild: <100/mm2, moderate: 100-499/mm2 and severe: gt;or=500/mm2. RESULTS: In all groups, there were significant pre- and post-operative differences in the IPSS, Qmax and residual urine (p<0.05, t-test). The prostate weights were significantly decreased in the 'central' group preoperatively, and the Qmax was decreased in the 'central' group 6 months postoperatively (p<0.05, ANOVA). CONCLUSIONS: We found no influence of prostatic lymphocyte infiltration on the outcome of TURP. Although the preoperative prostate weight of the 'central' group was smaller than in the 'peripheral' group, if the prostatic calcifications were located around the verumontanum or periurethral area, the Qmax was poorer 6 months postoperatively. Therefore, the evaluation of the size and location of prostatic calcifications from TRUS may be useful in predicting the outcome of TURP.
Hematoxylin ; Humans ; Hyperplasia ; Lymphocytes* ; Male ; Prostate ; Prostatic Hyperplasia* ; Transurethral Resection of Prostate* ; Weights and Measures

PURPOSE: A transurethral resection of the prostate (TURP) is the gold standard surgical treatment for men with benign prostate hyperplasia (BPH), but 10% of patients shows no improvement in voiding symptom following the procedure. We attempted to find if the prostatic calcification and inflammatory cell infiltration could affect the result of TURP in BPH. MATERIALS AND METHODS: 117 BPH patients who had undergone a TURP, performed by one urologist between March 1999 and February 2001, were evaluated. The prostatic volume, and the size and location of the prostatic calcifications were checked preoperatively. When the calcifications were located around the verumontanum or periurethral area they were classified as central, whereas, when they were located peripherally they were classified as peripheral. The IPSS, Qmax and residual urine were measured preoperatively, and at 6 months after the TURP. The tissues obtained from the TURP were evaluated histologically. Slides of the hematoxylin and eosin-stained tissue of each patient were examined from 5 to 15 chips with a light microscope. They were divided into three groups depending on the level of lymphocyte infiltration; mild: <100/mm2, moderate: 100-499/mm2 and severe: gt;or=500/mm2. RESULTS: In all groups, there were significant pre- and post-operative differences in the IPSS, Qmax and residual urine (p<0.05, t-test). The prostate weights were significantly decreased in the 'central' group preoperatively, and the Qmax was decreased in the 'central' group 6 months postoperatively (p<0.05, ANOVA). CONCLUSIONS: We found no influence of prostatic lymphocyte infiltration on the outcome of TURP. Although the preoperative prostate weight of the 'central' group was smaller than in the 'peripheral' group, if the prostatic calcifications were located around the verumontanum or periurethral area, the Qmax was poorer 6 months postoperatively. Therefore, the evaluation of the size and location of prostatic calcifications from TRUS may be useful in predicting the outcome of TURP.
Hematoxylin ; Humans ; Hyperplasia ; Lymphocytes* ; Male ; Prostate ; Prostatic Hyperplasia* ; Transurethral Resection of Prostate* ; Weights and Measures

Granulocyte macrophage-colony stimulating factor (GM-CSF) has immuno-stimulatory effects. We hypothesized that GM-CSF plays an important role both in lipopolysaccharide (LPS)- and hemorrhage-induced acute lung injury (ALI). We also postulated that GM-CSF augments LPS-induced inflammation by priming neutrophils. ALI was induced in GM-CSF-/- or control C57BL mice either by LPS injection or by hemorrhage. Lung inflammation (by lung expression for tumor necrosis factor-alpha (TNF-alpha), macrophage inflammatory protein-2 (MIP-2), interleukin-1beta (IL-1beta), interleukin- 6 (IL-6), and keratinocyte-derived chemokine) and lung injury (by myeloperoxidase and evans blue dye assay) were evaluated after ALI. Incremental doses of LPS (0, 1, 10, and 100 ng/mL) and GM-CSF (0, 1, 10, and 100 ng/mL) were added to bone marrow neutrophils. The expression of TNF-alpha, MIP-2, and IL-1beta was evaluated with enzyme linked immunosorbent assay. The mRNA expression of three cytokines, and the nuclear translocation of nuclear factor kappa B (NF kappa-B) were evaluated by reverse transcriptase-polymerase chain reaction and electropnoretic mobility shift assay, respectively. GM-CSF -/- mice showed decreased neutrophil infiltration, less leakage, and lower expression of cytokines in the lung after LPS or hemorrhage. GM-CSF augmented LPS-induced protein and mRNA expression of TNF-alpha, MIP-2 and IL-1beta, which was mediated by increased intra-nuclear translocation of NF-kappa B. GM-CSF plays an important role in high-dose LPS and hemorrhage-induced ALI, which appears to be mediated by its priming effect on neutrophils.
Animals ; Bone Marrow Cells/cytology ; Chemokine CXCL2/metabolism ; Granulocyte-Macrophage Colony-Stimulating Factor/metabolism/*physiology ; Interleukin-1beta/metabolism ; Lipopolysaccharides/metabolism ; Lung/metabolism/pathology ; *Lung Injury ; Male ; Mice ; Mice, Inbred BALB C ; Mice, Inbred C57BL ; Mice, Transgenic ; Neutrophils/*cytology/metabolism ; Peroxidase/metabolism ; Tumor Necrosis Factor-alpha/metabolism

No abstract available.
Animals ; Epithelium* ; Guinea Pigs* ; Guinea* ; Muscle, Smooth, Vascular* ; Rats*

No abstract available.
Pleural Effusion ; Pleural Effusion, Malignant ; Thoracoscopy ; Thyroid Gland ; Thyroid Neoplasms

BACKGROUND: Rifampicin (RFP) is a key component of the antituberculous short-course chemotherapy and the RFP resistance is a marker of multi-drug resistant (MDR) tuberculosis. RpoB gene encodes the beta-subunit of RNA polymerase of M. tuberculosis which is the target of RFP. And the mutations of rpoB gene have been found in about 96% of rifampicin resistant clinical isolates of M. tuberculosis. So in order to find a rapid and clinically useful diagnostic method in identifying the REP resistance, we compared the PCR-line probe method with PCR-SSCP for the detection of the rpoB gene mutation in cultured M. tuberculosis. METHODS: 45 clinical isoLates were collected from patients who visited Sung Kyun Kwan University Hospital. The REP susceptibility test was referred to the referral laboratory of the Korean Tuberculosis Institute. 33 were rifampicin resistant and 12 were rifampicin susceptible. The susceptibility results were compared with the results of the PCR-SSCF and PCR-line probe method. RESULTS: We could find rpoB mutations in 27/33(81.8%) RFP-resistant strains by PCR-line probe method, and in 23/33(69.7%) by PCR-SSCP and there was no significant difference between two methods. There was no mutation in rifampicinn susceptible strains by both methods.: Comparison of PCR-line probe and PCR-SSCP methods for detection rifampicin resistance CONCLUSION: PCR-Iine probe method would be a rapid, sensitive and specific method for the detection of rifampicin resistant Mycobacterium tuberculosis.
DNA-Directed RNA Polymerases ; Drug Resistance ; Drug Therapy ; Humans ; Mycobacterium tuberculosis* ; Mycobacterium* ; Polymorphism, Single-Stranded Conformational ; Referral and Consultation ; Rifampin* ; Tuberculosis