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

BACKGROUND: As single donor platelets (SDP) has been increasingly used, the quality of SDP, especially apheresis-induced platelet activation, has become a major issue. This study evaluated the activation of SDP platelets prepared with three different cell separators that are currently being used at the Korean Red Cross. METHODS: CD62p, CD63 and CD42 were measured in 35 units of SDP prepared with Amicus (Baxter, Deerfield, IL, USA), MCS+ (Haemonetics, Braintree, MA, USA), or Trima (Gambro BCT, Lakewood, USA) using flow cytometry. RESULTS: Expression of CD62p gradually increased with storage time, but no difference in expression was noted between cell separators. Expression of CD63 also increased with storage time and platelets prepared with the Amicus displayed significantly higher CD63 expression 72 and 120 hours after collection compared to those prepared with MCS+ and Trima. Expression of CD42b tended to decrease with storage time, but this was only significant for Amicus 120 hours after collection. No difference in CD42b expression was noted between cell separators. CONCLUSIONS: Platelet activation increased with storage time, and platelet activation was more pronounced in the platelets prepared with the Amicus. However, because in vitro results of platelet activation does not necessarily reflect in vivo platelet function and survival, additional studies are needed to clarify clinical effectiveness of activated platelets.
Blood Platelets* ; Flow Cytometry ; Humans ; Platelet Activation* ; Plateletpheresis* ; Red Cross ; Tissue Donors

No abstract available.

BACKGROUND: Acute renal failure (ARF) due to acute pyelonephritis (APN) in patients in the absence of obstructive uropathy, diabetes, or chronic renal failure was reported rarely (12 cases in the last 25 yrs in Clin Inf Dis, 1992; 243-6 by SR Jones)2). Acute renal failure is a rare complication of acute pyelonephritis in patients who do not have urinary obstruction. Although urinary tract infections are common in adults, pyelonephritis is rarely considered in the differential diagnosis of acute renal failure, and when clinicians weigh the possible consequences of bacteriuria, renal failure is not considered to be a reasonable possibility. METHODS: We observed 6 patients of acute pyelonephritis associated with acute renal failure as an initial manifestation on admission in the past 6 months. The analysis of clinical features of these 6 patients (APN+) compared to 7 patients of APN without ARF (APN-) revealed following data. RESULTS: All were female in both groups. The mean age was 331.7 years in APN+ and 48+6 years in APN-, respectively (p, ns). All in both APN- and APN+ were caused by E. coli. But, none in both groups except 1 in APN- had bacteremia. The previous history of UTI was present in 5 in APN-, but none in APN+. Duration of clinical symptoms before admission (8.4+/-0.5 vs. 4.4+/-1.0 days, P<0.05) and admission days (24.2+/-1.8 vs. 11.1+/-1.5 days, p=0.000) were significantly longer in APN+. On admission, HUN and serum creatinine was 45+/-2.7mg/dL and 3.5+/-0.2mg/dL in APN+, respectively. No one in APN+ required dialysis, and subsequently recovered renal function with prompt antibiotic therapy resulting in no significant difference compared to that of APN on discharge (serum creatinine, 1.0+/-0.1 vs. 0.9+/-0.1mg/dL). No differences in leukocytosis and serum electrolyte levels on admission were present. However, significant anemia was noted in APN+ (hemoglobin, 9.0+/-0.7 vs. 11.9+/-0.2gm/dL). The days of fever and leukocytosis before disappearance were not different in both groups, but those of flank pain (12.8+/-1.5 vs. 5.7+/-0.7 days, P=0.001) and pyuria (16+/-1.9 vs. 6.4+/-0.6 days, P=0.000) significantly longer in APN+. Upon ultrasonography, all in APN+ showed enhanced echogenicity, but I in APN . (P=P value, NS=not significant) CONCLUSION: Upon these data, we concluded that ARF associated with APN as an initial manifestation was accompanied by several distinct clinical characteristics, which could be used for the early recognition of its unusual occurrence and subsequent appropriate management including antibiotics leading to a favorable outcome.
Acute Kidney Injury* ; Adult ; Anemia ; Anti-Bacterial Agents ; Bacteremia ; Bacteriuria ; Creatinine ; Diagnosis, Differential ; Dialysis ; Female ; Fever ; Flank Pain ; Humans ; Kidney Failure, Chronic ; Leukocytosis ; Pyelonephritis* ; Pyuria ; Renal Insufficiency ; Ultrasonography ; Urinary Tract Infections

BACKGROUND AND OBJECTIVES: Arrhythmia is known to be a major cause of death in acute myocardial infarction (AMI). Reperfusion arrhythmias (RA) may also occur during angioplasty or thrombolysis. As yet, the clinical significances of RA and angiographic characteristics of the patients who develop RA during primary angioplasty and stenting are not clearly defined. METHODS: The study group consisted of 60 patients treated with primary angioplasty or stenting for AMI (angioplasty 13, stenting 47 patients). The patients were classified into 2 groups according to RA [RA (-) N=36/RA(+) N=24]: demographic and angiographic characteristics including time to reperfusion and incidence of pre-infarct angina were analyzed. RESULTS: The RA occurred in 40% of patients undergoing primary angioplasty or stenting (24/60 patients). The minor arrhythmias were more common after reperfusion (transient bradycardia 14, accelerated idioventricular rhythm 11, premature ventricular contraction 4 cases): major arrhythmias were uncommon (ventricular tachycardia/fibrillation 5, asystole 1 case). In the two groups, baseline clinical characteristics were similar except for pain to reperfusion time [RA (-): RA (+)=490.8+/-291.7: 252.9+/-109.2 minutes, P=0.001]. There was a trend toward a greater incidence of RA in the right coronary infarct-related artery [RA (-): RA (+)=16.7: 41.7%, P=NS]. The RA occurred in totally occluded artery (TIMI 0) with a giant thrombus and first ballooning in 19/24 patients (79.2%). The RA disappeared with conservative managements including pacemaker insertion and cardiopulmonary resuscitation and there were no differences in major adverse cardiac events in the two groups during follow-up. CONCLUSIONS: These findings suggest that the RA are frequent events during primary angioplasty but unrelated to clinical and angiographic characteristics except for reperfusion time and do not influence short-term prognosis in AMI.
Accelerated Idioventricular Rhythm ; Angioplasty* ; Arrhythmias, Cardiac* ; Arteries ; Bradycardia ; Cardiopulmonary Resuscitation ; Cause of Death ; Follow-Up Studies ; Heart Arrest ; Humans ; Incidence ; Myocardial Infarction* ; Prognosis ; Prospective Studies* ; Reperfusion* ; Stents ; Thrombosis ; Ventricular Premature Complexes

Objective: This study evaluated the clinical features of acute Phytolacca poisoning and investigated the prognostic factors associated with severe poisoning. Methods: This is a retrospective observational study using the data of patients presenting with acute Phytolacca poisoning. Demographic data, toxicological data, vital signs, laboratory data, and electrocardiographic data were collected. Study patients were divided into mild and severe poisoning patients. After a univariate analysis, binary logistic regression analysis, which used ‘severe poisoning’ as a dependent variable, was performed to figure out the independent variables. In addition, the area under the curve and the cut-off value of independent variables were suggested by using receiver operating characteristics analysis. Results: Most poisonings (80.5%) occurred in winter and spring. Most patients (98.4%) ingested the root of Phytolacca. It took 2 hours from ingestion to the beginning of the symptoms (interquartile range, 1.0-3.0) which included nausea/vomiting (98.4%), abdominal pain (58.6%), or diarrhea (53.1%). Severe poisoning developed in 21 patients (16.4%). For prediction of severe poisoning, the adjusted odds ratio of time from ingestion to the onset of symptoms was 0.18 (95% confidence interval [CI], 0.05-0.61) and that of the amount of ingestion was 1.42 (95% CI, 0.99-2.03). The area under the curve of time from ingestion to the onset of symptoms (≤1 hour) was 0.81 (95% CI, 0.73-0.88) and that of the amount of ingestion (>1.5 knuckles) was 0.75 (95% CI, 0.65-0.83). Conclusion Acute Phytolacca poisoning has clinical features of acute enterocolitis. Severe poisoning could develop especially in patients with a rapid onset of symptoms (≤1 hour) and ingesting over 1.5 knuckles.

Objective: This study evaluates the general clinico-toxicological characteristics, and determines whether they are varied with toxin source, in patients admitted to the hospital and diagnosed with grayanotoxin (GTX)/mad honey poisoning. Methods: Patients diagnosed with GTX/mad honey poisoning at the University Teaching Hospital emergency department between January 2001 and December 2015 were included in this retrospective study. The clinico-toxicological characteristics were compared by classifying patients into two groups, according to the toxin source: group A, poisoned by the Himalayan mad honey, and group B, poisoned by biologic materials containing GTX other than Himalayan mad honey. Results: Totally, 26 patients were identified with symptomatic grayanotoxin/mad honey poisoning. There were no statistical differences in the clinico-toxicological characteristics, except systolic blood pressure (SBP). At presentation, the SBP was significantly decreased in group B (P=0.013). Although dizziness and blurred vision were statistically not significant symptoms, there was a trend of significance (P<0.1) in group B. Notably, 5 of the 8 patients who consumed Rhododendron brachycarpum complained of blurred vision, and had a relatively low mean SBP (68.6±15.6 mmHg). Conclusion The general clinico-toxicological characteristics were similar, subsequent to ingestion of Himalayan mad honey and Rhododendron species. However, since blurred vision and hemodynamic instability were relatively more common in poisoning by R. brachycarpum than other Rhododendron species, emergency physicians need to be aware that the symptoms or severity of poisoning may vary according to the Rhododendron species ingested.

Objective: This study evaluated the clinical features of acute Phytolacca poisoning and investigated the prognostic factors associated with severe poisoning. Methods: This is a retrospective observational study using the data of patients presenting with acute Phytolacca poisoning. Demographic data, toxicological data, vital signs, laboratory data, and electrocardiographic data were collected. Study patients were divided into mild and severe poisoning patients. After a univariate analysis, binary logistic regression analysis, which used ‘severe poisoning’ as a dependent variable, was performed to figure out the independent variables. In addition, the area under the curve and the cut-off value of independent variables were suggested by using receiver operating characteristics analysis. Results: Most poisonings (80.5%) occurred in winter and spring. Most patients (98.4%) ingested the root of Phytolacca. It took 2 hours from ingestion to the beginning of the symptoms (interquartile range, 1.0-3.0) which included nausea/vomiting (98.4%), abdominal pain (58.6%), or diarrhea (53.1%). Severe poisoning developed in 21 patients (16.4%). For prediction of severe poisoning, the adjusted odds ratio of time from ingestion to the onset of symptoms was 0.18 (95% confidence interval [CI], 0.05-0.61) and that of the amount of ingestion was 1.42 (95% CI, 0.99-2.03). The area under the curve of time from ingestion to the onset of symptoms (≤1 hour) was 0.81 (95% CI, 0.73-0.88) and that of the amount of ingestion (>1.5 knuckles) was 0.75 (95% CI, 0.65-0.83). Conclusion Acute Phytolacca poisoning has clinical features of acute enterocolitis. Severe poisoning could develop especially in patients with a rapid onset of symptoms (≤1 hour) and ingesting over 1.5 knuckles.

Objective: This study evaluates the general clinico-toxicological characteristics, and determines whether they are varied with toxin source, in patients admitted to the hospital and diagnosed with grayanotoxin (GTX)/mad honey poisoning. Methods: Patients diagnosed with GTX/mad honey poisoning at the University Teaching Hospital emergency department between January 2001 and December 2015 were included in this retrospective study. The clinico-toxicological characteristics were compared by classifying patients into two groups, according to the toxin source: group A, poisoned by the Himalayan mad honey, and group B, poisoned by biologic materials containing GTX other than Himalayan mad honey. Results: Totally, 26 patients were identified with symptomatic grayanotoxin/mad honey poisoning. There were no statistical differences in the clinico-toxicological characteristics, except systolic blood pressure (SBP). At presentation, the SBP was significantly decreased in group B (P=0.013). Although dizziness and blurred vision were statistically not significant symptoms, there was a trend of significance (P<0.1) in group B. Notably, 5 of the 8 patients who consumed Rhododendron brachycarpum complained of blurred vision, and had a relatively low mean SBP (68.6±15.6 mmHg). Conclusion The general clinico-toxicological characteristics were similar, subsequent to ingestion of Himalayan mad honey and Rhododendron species. However, since blurred vision and hemodynamic instability were relatively more common in poisoning by R. brachycarpum than other Rhododendron species, emergency physicians need to be aware that the symptoms or severity of poisoning may vary according to the Rhododendron species ingested.

The understanding of main mechanisms that determine the ability of immune privilege related to Sertoli cells (SCs) will provide clues for promoting a local tolerogenic environment. In this study, we evaluated the property of humoral and cellular immune response modulation provided by porcine SCs. Porcine SCs were resistant to human antibody and complement-mediated formation of the membrane attack complex (38.41+/-2.77% vs. 55.02+/-5.44%, p=0.027) and cell lysis (42.95+/-1.75% vs. 87.99 +/-2.25%, p<0.001) compared to immortalized aortic endothelial cells, suggesting that porcine SCs are able to escape cellular lysis associated with complement activation by producing one or more immunoprotective factors that may be capable of inhibiting membrane attack complex formation. On the other hand, porcine SCs and their culture supernatant suppressed the up-regulation of CD40 expression (p<0.05) on DCs in the presence of LPS stimulation. These novel findings, as we know, suggest that immune modulatory effects of porcine SCs in the presence of other antigen can be obtained from the first step of antigen presentation. These might open optimistic perspectives for the use of porcine SCs in tolerance induction eliminating the need for chronic immunosuppressive drugs.
Animals ; Antibodies, Heterophile/immunology ; Antibody Formation/*immunology ; Antigens, CD40/immunology ; Aorta/cytology ; Cell Line, Transformed ; Cell Survival/immunology ; Complement Membrane Attack Complex/immunology ; Complement System Proteins/immunology ; Dendritic Cells/cytology/immunology ; Endothelial Cells/cytology/immunology ; Epitopes/immunology ; Humans ; Immune Tolerance/*immunology ; Immunity, Cellular/*immunology ; Male ; Mice ; Mice, Inbred C57BL ; Sertoli Cells/cytology/*immunology ; Swine ; *Tissue Engineering ; Transplantation, Heterologous