PURPOSE: This study was designed to define normal values for a nongeometric MPI in children and evaluate the utility of m%cardial performance index(MPI) in congenital heart disease with distorted ventricular geometry. METHODS: The study population consisted of 44 normal patients and 28 patients with atrial septal defect(ASD) with dilated right ventricle and paradoxical septal motion. Right ventricular(RV) and left ventricular(LV) isovolumic contraction time, isovolumic relaxation time, ejection time, ejection fraction and pre-ejection period/ejection time(PEP/ET) were measured using conventional echo- Doppler methods. The MPI measures the ratio of total time spent in isovolumic activity(isovolumic contraction time and isovolumic relaxation time) to ejection time. RESULTS: In normal children, the RV MPI was 0.33+/-0.09 and the LV MPI was 0.36+/-0.04. In the ASD group, LV function seemed grossly normal but LV EF was significantly lower than normal children(57+/-3% vs 67+/-4%, P<0.05), but both LV and RV MPI had no statistically significant difference. After correction of ASD, RV MPI was increased and LV EF(57+/-3% vs 64+/-3%, P<0.05) as wll as the RV PEP/ET(0.27+/-0.05 vs 0.38+/-0.06, P<0.05) were significantly greater than the normal or the preoperation group. CONCLUSION: Components of the MPI are easily measured with conventional Doppler technique using standard echocardiographic examination. These results suggest that the MPI is useful as a means of quantitative assessment of ventricular performance in patients with complex ventricular geometry, particularly RV.
Child ; Echocardiography ; Heart Defects, Congenital ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Reference Values ; Relaxation ; Ventricular Function*

BACKGROUND: Complex regional pain syndrome (CRPS) is categorized into sympathetically maintained pain (SMP) and sympathetically independent pain (SIP). Spinal cord stimulation (SCS) is a promising approach in the treatment of severely disabling CRPS. Patients with good responses to sympathetic block before SCS are more likely to have positive responses to SCS than those with negative responses. This study compared the effects of SCS in patients with CRPS, of SMP and SIP categories. METHODS: This was a retrospective study of 16 patients (SMP 8, SIP 8) with CRPS who had undergone trials of SCS. Eleven of the patients had permanent SCS device implants, and the pain relief levels at 1 and 6 months were recorded. RESULTS: Sixteen patients with severe, incapacitating, and therapy-resistant CRPS underwent SCS trials. Five patients (SMP 3, SIP 2) had poor pain relief during the trial despite adequate coverage. The remaining 11 patients (SMP 5, SIP 6) had permanent electrode implantation performed under local anesthesia and experienced good pain relief. The difference in VAS reduction was not significant between the two groups at the 1-month follow-up (P = 0.325) and the 6-month follow-up (P = 0.779). CONCLUSIONS: There were no statistically significant differences in VAS pain scores between the two groups. The favorable outcome in all 11 patients with only minor remaining symptoms or without remaining symptoms or severe recurrences suggests that SCS is an efficient treatment in SMP and SIP.
Anesthesia, Local ; Electrodes ; Follow-Up Studies ; Humans ; Recurrence ; Retrospective Studies ; Spinal Cord ; Spinal Cord Stimulation

BACKGROUND: Cardiac anesthesia with cardiopulmonary bypass is associated with an increased risk of awareness. A new approach to intraoperative EEG procession, the bispectral index (BIS), may allow more reliable quantitative assessment of the level of consciousness during anesthesia. We evaluated the relationships between BIS and commonly used indices of depth of anesthesia in children. METHODS: In the isoflurane group, anesthesia was maintained using isoflurane 0.5 1.0 vol% inhalation, whereas the fentanyl group received high dose fentanyl-midazolam infusion. BIS, mean arterial blood pressure, and heart rate were recorded at baseline, at postinduction (Tbaseline), skin incision (Tincis), sternotomy (Tsterno), aortic cannulation (Tcannul), nadir temperature (Tnadir), immediate post-CPB (TpostCPB), sternal wire (Twire), and at skin closure (Tclose). RESULTS: BIS showed a significant change over time (P < 0.001), with significant differences between groups (P = 0.04). BIS increased significantly during the immediate post-CPB phase (TpostCPB versus Tbaseline, Tnadir, P < 0.01). No correlations were found between BIS and changes in heart rate or mean arterial pressure during surgery. BIS was higher in the fentanyl group than in the isoflurane group at Tincis, Tcannul, and Twire (P < 0.05). CONCLUSIONS: Even though BIS was relatively lower in the isoflurane group compared to the fentanyl group, we were still unable to demonstrate a relationship between the BIS and hemodynamic indices depth for any group. While the increase in BIS during the rewarming phase could reflect an increase in conscious level, larger prospective studies with postoperative memory test for awareness are necessary.
Anesthesia ; Anesthetics* ; Arterial Pressure ; Cardiopulmonary Bypass ; Catheterization ; Child* ; Consciousness ; Electroencephalography ; Fentanyl ; Heart Rate ; Heart* ; Hemodynamics* ; Humans ; Inhalation ; Isoflurane ; Memory ; Rewarming ; Skin ; Sternotomy ; Thoracic Surgery*

PURPOSE: We performed the study to evaluate the risk factors associated with cardiac complication and the outcome of coronary lesions after high-dose (2g/kg, 1dose) gammaglobulin (IVIG) treatment in patients with Kawasaki disease (KD). METHODS: Retrospective studies were performed on 338 cases of KD treated with high-dose IVIG at this hospital from May 1994 to March 1999. RESULTS: Among 338 patients, fever was persistent for 8.09+/-3.45 days in all patients and after IVIG infusion, fever subsided in 26.5+/-26.4 hours. Forty-five patients (13.3%) with abnormal baseline echocardiogram remained febrile for 9.09+/-4.09 days, significantly longer than normal baseline echocardiogram group. There was no significant differences in age, sex and other clinical findings. In 7 patients (2.1%) usage of medications above 12 months were needed. Eleven cases(3.3%) with persistent fever after their first course of IVIG therapy became afebrile post-second course of IVIG in 5 of the 11 cases with cardiac complication and in 1 case with recurrence. Twelve (male 11, female 1) of the 338 cases recurred after 8.45+/-4.39 months. Three of those 12 cases had cardiac complication. CONCLUSION: The overall prevalence of persistent coronary abnormalities in KD patients treated with high-dose IVIG and aspirin remains low. Even if patients had cardiac complication, they improved later.
Aspirin ; Female ; Fever ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Prevalence ; Recurrence ; Retrospective Studies ; Risk Factors

Remote ischemic preconditioning (IP), brief tolerating cycles of ischemia and reperfusion in remote non-vital organs, can reduce ischemic injury of the heart. IP induces cardiac protection by down-regulating iNOS or up-regulating eNOS. In addition, Akt has been known to protect myocardium against ischemia-reperfusion injury. This study was undertaken to observe the expression of iNOS, eNOS, Akt and phospho-Akt (p-Akt) in the rat myocardium after IP. Thirty-five weeks-old male Sprague-Dawley rats were divided into control and IP groups. The IP group was further subdivided into 3 groups based on the number of cycles of IP. For IP, left commom iliac artery was occluded 3, 6 and 10 cycles for 5 min of ischemia alternating with 5 min of reperfusion. The rat were sacrificed at 0, 3, 6, 24 and 72 hours of IP and the heart was removed. The expression of iNOS, eNOS, Akt and p-Akt in the rat myocardium was examined by immunohistochemical staining and Western blot analysis. The expression of iNOS was increased by IP and was higher in 10IP groups than 3IP and 6IP group. The expression of eNOS was increased or decreased by IP and was showed no difference with increasing episode of IP. The expression of Akt was decreased by IP at 24 and 72 hours after reperfusion, and showed no differences with increasing episode of IP. The expression of p-Akt was increased by IP and showed no difference with increasing episode of IP. These results suggest that hind limb ischemic preconditioning provides cardiac protection through up-regulation of eNOS and phosphorylation of Akt, however excessive episodes of remote preconditioning may induce the myocardial ischemic injury through overexpression of iNOS.
Animals ; Blotting, Western ; Extremities ; Heart ; Humans ; Iliac Artery ; Ischemia ; Ischemic Preconditioning ; Male ; Myocardium ; Phosphorylation ; Rats ; Rats, Sprague-Dawley ; Reperfusion ; Reperfusion Injury ; Up-Regulation

Castleman's disease was first described in 1956 in a group of patients with localized mediastinal lymph node enlargement characterized by hyperplasia of lymphoid follicles and marked capillary proliferation with endothelial hyperplasia. They have been divided into 2 histologic types: the hyaline-vascular type, which was more common and usually asymptomatic, was characterized by small hyaline-vascular follicles and interfollicular capillary proliferation; the plasma-cell type was characterized by large follicles with intervening sheets of plasma cells. Systemic manifestations, such as fever, anemia and hyperglobulinemia, were frequently associated with the plasma cell type. Localized lesions have behaved in a benign fashion, and complete surgical excision has been curative. But recent years, reports have described a multicentric variety with severe systemic manifestations, exorable clinical course and poor outcome. Although Castleman's disease has been described at all ages, the disease is rare in childhood. This paper describes a case of plasma cell type Castleman's disease in a 12-year-old boy and review of the literature. We conclude that the Castleman's disease must be considered in childhood lymphadenopathy and the clinicians should be mindful of the malignant potential of the disease and their possible multicentricity. Appropriate treatment plan, close follow-up and periodic surveillance are necessary.
Anemia ; Capillaries ; Child ; Fever ; Follow-Up Studies ; Giant Lymph Node Hyperplasia* ; Humans ; Hyperplasia ; Lymph Nodes ; Lymphatic Diseases ; Male ; Plasma Cells* ; Plasma*

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a distinct subset of cerebral amyloid angiopathy characterized by the auto-inflammatory response to amyloid-laden small arteries of cerebral cortex and leptomeninges. Clinical features include cognitive-behavioral change, headache, focal neurologic deficits and seizure. Because anti-inflammatory treatments can rapidly relieve neurologic symptoms, early diagnosis is critical. Herein, we report a CAA-RI case with distinct laboratory findings of a decreased cerebrospinal fluid amyloid beta 1-42 level and relatively reduced florbetaben uptake in the focal inflammatory lesion during the acute phase of CAA-RI.

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a distinct subset of cerebral amyloid angiopathy characterized by the auto-inflammatory response to amyloid-laden small arteries of cerebral cortex and leptomeninges. Clinical features include cognitive-behavioral change, headache, focal neurologic deficits and seizure. Because anti-inflammatory treatments can rapidly relieve neurologic symptoms, early diagnosis is critical. Herein, we report a CAA-RI case with distinct laboratory findings of a decreased cerebrospinal fluid amyloid beta 1-42 level and relatively reduced florbetaben uptake in the focal inflammatory lesion during the acute phase of CAA-RI.

PURPOSE: AMD3100, an antagonist of the CXCR4 chemokine receptor is soon to be used clinically for the peripheral mobilization of hematopoietic stem cells (HSCs) in patients with multiple myeloma. AMD3100 has been shown to activate a G protein coupled with CXCR4 and thus acts as a partial CXCR4 agonist in vitro. Thus, we explored whether AMD3100 affected the survival and proliferation of myeloma cells in vitro. MATERIALS AND METHODS: The effects of AMD3100 on survival and proliferation of two myeloma cell lines (RPMI8226 and U266) as well as CD138+ cells obtained from several patients with multiple myeloma were analyzed by flow cytometry using annexin V and a colorimetric cell proliferation assay (CCK-8 assay). RESULTS: AMD3100, but not T140, another CXCR4 antagonist, stimulated the proliferation of myeloma cell lines and CD138+ primary human myeloma cells (-2-fold increase) in a dose-dependent manner in serum-free culture for up to 5 days, which was inhibited by pretreating the cells with pertussis toxin. AMD3100 enhanced the proliferation of U266 cells induced by interleukin-6 and partially reversed AG490-mediated growth inhibition and apoptosis induced by serum deprivation in RPMI8226 cells. AMD3100 induced the phosphorylation of Akt and MAPK p44/p42 in U266 cells and MAPK p44/p42 in RPMI8226 cells. In contrast, AMD3100 markedly increased the cell apoptosis and reduced the number of RPMI8226 cells after 5 to 7 days of culture under serum-free conditions. CONCLUSION: AMD3100 exerts dual effects, initially enhancing and subsequently inhibiting the survival and proliferation of myeloma cells, signaling via CXCR4 in vitro.
Annexin A5 ; Apoptosis ; Cell Line ; Cell Proliferation ; Flow Cytometry ; GTP-Binding Proteins ; Hematopoietic Stem Cells ; Heterocyclic Compounds ; Humans ; Interleukin-6 ; Multiple Myeloma ; Oligopeptides ; Pertussis Toxin ; Phosphorylation

BACKGROUND: Bortezomib is widely used for the treatment of multiple myeloma. Bone marrow stromal cells (BMSCs) endow myeloma cells with survival and growth advantages. However, the influence of bortezomib on BMSCs is not well elucidated. We examined the effects of bortezomib on the survival and growth of BMSCs in vitro. METHODS: The effects of bortezomib on the survival and proliferation of the BMSC MS-5 cell line and on BMSCs obtained from healthy individuals (N=4) and newly diagnosed myeloma patients (N=5) were investigated in vitro. Transmembrane cell migration was evaluated using the Transwell system. A short interfering RNA strategy was used to knock down the expression of chemokine (CXC motif) ligand 12 (CXCL12) mRNA. To examine the effects of bortezomib-exposed BMSCs on the migration and localization of myeloma cells, MS-5 monolayers were treated with bortezomib for 24 hr, washed, and then overlaid with human RPMI8226 myeloma cells. RESULTS: Bortezomib inhibited BMSC proliferation in a concentration-dependent manner, and induced cellular apoptosis. Bortezomib decreased CXCL12 production by BMSCs. Knockdown of CXCL12 mRNA in BMSCs revealed that CXCL12 served as an autocrine growth factor. Short-term bortezomib treatment of BMSC monolayers reduced the tendency of myeloma cells to locate to positions under the monolayers. CONCLUSION: Bortezomib inhibits the survival and growth of BMSCs via downregulation of CXCL12, which may contribute to the clinical effects of this agent.
Apoptosis ; Cell Line ; Cell Movement ; Down-Regulation ; Humans ; Mesenchymal Stromal Cells* ; Multiple Myeloma ; RNA, Messenger ; RNA, Small Interfering ; Bortezomib