Recent molecular studies have revealed that a 22q11 deletion is frequently detected in DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and conotruncal anomaly face syndrome (CTAFS). As one of the major clinical manifestations in these three syndrome is conotruncal cardiac malformation, we prospectively studied the frequency of a 22q11 deletion in a group of patients with conotruncal cardiac malformation. Fluorescence in situ hybridization (FISH) analyses using N25 (D22S75) DiGeorge Chromosome Region probe were performed on 64 patients with conotruncal cardiac malformation, who visited our clinic from October 1993 to January 1994. Of the 64 patients studied, a 22q11 deletion was detected in 5 patients (7.8%): 3 out of 30 patients with tetralogy of Fallot, one of three with interruption of the aortic arch, and one hemitruncus patient. No deletion was found in 16 patients with complete transposition of the great arteries, 8 with double outlet right ventricle and 2 with aortopulmonary window. In these five patients with 22q11 deletion, patient 1 was clinically diagnosed as having DGS, patients 2 and 3 had CTAFS, and patient 4 had VCFS. Patient 5 could not be dysmorphologically evaluated. It was noteworthy that all patients with a 22q11 deletion, except a non-evaluated patient, had some symptoms DGS, CTAFS or VCFS, and that we failed to identify a non-syndromic 22q11 deletion positive patients in the present series' of 64 patients. Conclusion. This study suggests that it is advisable to bear 22q11 deletion in mind when a patient with conotruncal cardiac anomalies has some other features of DGS, VCFS or CTAFS.

This study was conducted to determine the incidence and clinical significance of human herpesvirus-6 (HHV-6) infection in renal allografts. A total of 105 biopsy specimens from 72 recipients were immunohistochemically examined for the presence of HHV-6 antigen, which localized in the distal tubular epithelial cells and in a few lymphocytes infiltrating into the interstitium. HHV-6 antigen in the tubular epithelia was detected in 63 (61.2%) specimens. Categorically, a higher incidence of the antigen was noted in specimens of accelerated rejection (3/4, 75.0%), acute rejection (28/3, 73.7%), and cyclosporin nephropathy (8/11, 72.7%). The antigen was present and absent an almost equal number of times in the categories of chronic rejection, intraoperative and routine protocol biopsies. Repeated biopsies were performed in six cases showing HHV-6 antigen, only one of which underwent transplant nephrectomy due to severe chronic rejection. Single or multinucleated giant cells in distal tubuli occurred in 10 (9.5%) specimens in a scattered manner. All of them were diagnosed as acute or chronic rejection. The giant cells showed no immunoreactivity for HHV-6, cytomegalovirus, or herpes simplex virus. These results indicate overall that HHV-6 infection is common in renal allografts and might be reactivated in acute rejection or cyclosporin nephropathy. The presence of HHV-6 antigen, however, does not necessarily correlate with a poor prognosis for the renal graft nor with the occurrence of giant cells in distal tubuli.

solated non-compaction of the ventricular myocardium (INCVM) is a relatively newly defined disorder of the endomyocardium characterized by prominent ventricular trabeculations and deep intertrabecular recesses. 1 Clinical manifestations include a depressed left ventricular (LV) function, ventricular arrhythmia, and systemic embolization. 2,3 INCVM has been reported to be associated with several electrocardiographic changes such as ST depression and flat or negative T waves, bundle branch block, and Wolff-Parkinson-White (WPW) syndrome. [2][3][4] To the best of our knowledge, a long QT interval has not been reported on previously.We report 2 cases of INCVM with long QT syndrome (LQTS). Case Report Patient 1The patient was a boy aged 1 day old, who had no family history of sudden death or arrhythmia, and electrocardiograms (ECGs) of his parents showed no abnormality. He was born normally in a maternity hospital at the gestational age of 38 weeks. The Apgar score was 8 points at 1 min after birth. He was given low-dose oxygen because of slight cyanosis. At 13 h after birth, he showed tachypnea and systemic cyanosis.When he was admitted to our hospital, his condition was severe. His respiratory rate was 60 breaths/min and heart rate was 130 beats/min. His systolic blood pressure was 60 mmHg. His head and face was edematous. No heart murmur was audible. Hepatosplenomegaly was not recognized.Blood gas analysis indicated severe metabolic acidosis; the base excess was -9.2 mmol/L and pH was 7.305. Both AST and CK were elevated. In particular, CK was as high as 2,830 IU/L. Mild hypocalcemia (7.8 mg/dl) was present and other serum electrolytes including magnesium (2.3 mg/dl) were within normal limits. A physical examination and laboratory data showed marked stress during gestation or circulatory shock of an unknown cause. A chest X-ray revealed cardiomegaly, and the cardiothoracic ratio (CTR) was 68%. The ECG on admission showed left bundle branch block (LBBB) and a long QT time (Figure 1). An echocardiography showed that the ventricular myocardium was thickened and that the endocardial trabeculation was prominent at the apex, especially in the right ventricle. There were no congenital heart defects, and the ejection fraction of the left ventricle (LVEF) was 10%.A few minutes after supplemental calcium infusion, polymorphic ventricular tachycardia (VT) with torsades de pointes and sustained VT were detected on an ECG moniter (Figure 1). Lidocaine 1 mg/kg was infused initially, but it was ineffective. Even though cardioversion was performed several times, it had only a transient effect on resolving polymorphic and sustained VT. After 1 mmol/kg MgSO4 was administered, sustained VT was resolved, and MgSO4 was infused at a rate of 0.1 mmol · kg -1 · h -1 to prevent sustained VT with torsades de pointes.After VT was resolved, the LVEF was gradually improved to 60% and the CTR based on a chest X-ray was normalized at the age of 4 days. An ECG showed a narrow QRS pattern, not a bundle branch block, and QTc was still prolonged to...

A humanized monoclonal IgG1 antibody, designated hC4G1, recognizes the fibrinogen receptor glycoprotein (GP)IIb/IIIa on platelets and inhibits platelet aggregation. When the F(ab')2 fragment of hC4G1 (F(ab')2 hC4G1) was administered to cynomolgus monkeys, all the monkeys showed inhibition of platelet aggregation ex vivo. Unexpectedly, a significant decrease in platelet count was observed in 5 of 18 monkeys. Antibodies against F(ab')2 hC4G1 were detected in the plasma of these monkeys by ELISA. Antibody activity in the plasma of these monkeys was significantly correlated with the intensity of platelet decrease (r = 0.84). The natural monkey antibodies to F(ab')2 hC4G1 were directed against the C-terminal region of F(ab')2 fragment common to all human and humanized IgG antibodies. Natural homo-reactive antibodies were also detected in human plasma from 15 of 40 healthy volunteers. Specificity was closely similar to that of the monkey antibodies. Affinity-purified human homoreactive antibodies enhanced phagocytosis of platelets treated with the F(ab')2 hC4G1. Monkey plasma with high homo-reactive antibody activity was confirmed to decrease platelet count when administered together with F(ab')2 hC4G1 to a monkey with low antibody activity. These results suggest that F(ab')2 of humanized and human antibodies causes elimination of the corresponding antigens from the circulation by homo-reactive antibodies.

High-altitude platform stations (HAPSs) are expected to provide ultrawide-coverage areas and disaster-resilient networks from the stratosphere at around 20 km by installing wireless equipment on HAPS. Because their altitude is much lower than that of communications satellites, HAPSs can provide mobile communications services directly to smartphones, which are commonly used in terrestrial networks, such as fourth generation Long Term Evolution. Considering the widespread nature of mobile broadband communications and the importance as a backup line in case of disaster, HAPSs are expected to provide a large capacity in the future. A cellular system with single-cell frequency reuse using multiple cells similar to terrestrial mobile communications should be introduced to achieve such a capacity. The number of cells that a HAPS can accommodate ranges from 1 to more than 100, depending on unmanned aerial vehicle (UAV) ability. By contrast, the optimal cell configuration, which depends on the number of available cells, has not been clarified in previous research. In this paper, we propose an optimization method for the cell configuration for HAPS mobile communications using a genetic algorithm, which can be generally applied regardless of the number of cells and can clarify the optimal cell configuration. Although many cells are required to achieve gigabit-class HAPS mobile communications, the heightened power consumption due to the large number of cells is a critical problem for UAVs. Thus, we also investigate the reduction of the total transmission power and demonstrate the feasibility of energy-efficient gigabit HAPS mobile communications with wide coverage.