[A Suspected Case of Hemorrhagic Hereditary Telangiectasia Presented with Cerebral Hemorrhage in Infancy].

Hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasias in multiple organs. We experienced an infant with cerebral hemorrhaging and suspected HHT based on his family history of HHT. Computed tomography angiography confirmed a cerebral arteriovenous fistula. The onset of cerebral arteriovenous fistulas associated with HHT is relatively early, and the incidence of bleeding is relatively common. When HHT is suspected based on a family history, early imaging screening is recommended to improve the neurological prognosis, even in asymptomatic cases. (Received April 7, 2020; Accepted May 7, 2020; Published August 1, 2020).

Lack of an association between E-selectin gene polymorphisms and risk of Kawasaki disease.

BACKGROUND: Coronary artery lesions (CAL) are a serious complication of Kawasaki disease (KD). The increased serum E-selectin level during the acute phase of KD and the association of E-selectin gene (SELE) polymorphisms with the prevalence of coronary artery disease in adults suggest a possible association between SELE polymorphisms and the development of CAL in KD patients. METHODS: The subjects consisted of 177 KD patients, including 59 with and 118 without CAL, and 305 healthy controls. Two single nucleotide polymorphisms (SNP) of SELE, 98G>T (rs1805193) and Ser128Arg (rs5361), were genotyped by direct sequencing and the high-resolution melting curve method, respectively. The allele distributions were assessed using the chi-squared test. RESULTS: There were no significant differences in the T allele frequency at 98G>T between KD patients and controls (1.4% vs 1.0%, P = 0.55) or between KD patients with and without CAL (1.7% vs 1.3%, P = 0.77). Similarly, there were no differences in the distribution of the C allele (128Arg) at Ser128Arg between KD patients and controls (4.5% vs 3.4%, P = 0.40) or between KD patients with and without CAL (4.2% vs 4.7%, P = 0.86). CONCLUSION: Although no association was detected between these SELE polymorphisms and the prevalence of KD or the development of CAL, this may have been due to the study limitations, including a low frequency of the minor alleles and a small sample size. A larger-scale association study is needed in order for a definitive conclusion to be made as to whether these SNP are associated with susceptibility to KD or not.

100-Gbps CMOS transceiver for multilane optical backplane system with a 1.3 cm2 footprint.

A compact 4 × 25 Gbps optical transceiver has been fabricated for an optical backplane system, which consists of a 4 × 25 Gbps DFB-LD array, a 4 × 25 Gbps PIN-PD array, and a CMOS transceiver chip. These are directly mounted on 9 × 14 mm(2) multi-layer ceramic package with an electromagnetic shield structure to suppress inner-channel crosstalk effectively. The transceiver includes an analog front-end as well as an electrical interface function to interface with the switch LSI or CPU. Power consumption was as low as 20 mW/Gbps, and a transmission experiment was successfully conducted at 25 Gbps.

Expression of CD180, a toll-like receptor homologue, is up-regulated in children with Kawasaki disease.

Kawasaki disease (KD) is an acute febrile illness in childhood characterized by the formation of aneurysms in coronary arteries. It is believed that KD is caused by infectious agents because of its epidemic waves and high incidence of familial occurrence. Because an increase in the levels and dysfunction of B cells in peripheral blood was reported in KD, we investigated the expression of cluster of differentiation 180 (CD180), a toll-like receptor homologue, in the B cells of children with KD, and in those with bacterial or viral infections. The percentages of CD180 positive B cells were significantly higher in children with KD or viral infections than in those with bacterial infections or in healthy controls. When the expression levels of CD180 were compared by using the mean fluorescent intensity ratio of patients to healthy controls, the level of CD180 expression was also significantly up-regulated in children with KD or viral infections. To clarify the effect of viral infection on the expression of CD180, B cells were stimulated with poly inosinic-cytidyric acid [poly(IC)], a synthetic double-stranded RNA. Poly(IC) clearly enhanced CD180 expression in B cells in vitro, both at the protein and messenger RNA levels. These results suggest that similar mechanisms may be involved in the up-regulation of B cell CD180 expression in patients with either KD or viral infections.

A polymorphism in the promoter of the CD14 gene (CD14/-159) is associated with the development of coronary artery lesions in patients with Kawasaki disease.

OBJECTIVE: To investigate whether a polymorphism in the CD14 gene is associated with Kawasaki disease (KD). STUDY DESIGN: We extracted DNA from the whole blood of 69 control children and 67 patients with KD. We determined a polymorphism in the CD14 gene at position -159 upstream from the major transcription site (CD14/-159) by restriction fragment assay. We then investigated the association between CD14/-159 and the onset of KD and development of coronary artery lesion (CAL). RESULTS: The genomic and allelic frequencies of the polymorphism were not different between normal children and KD patients. The KD patients with TT genotypes at CD14/-159 had more CAL complications than those with CT and CC (OR, 4.05; 95% CI, 1.34-12.22). The frequencies of the T allele was significantly higher than that of the C allele in KD patients with CAL (OR, 2.20; 95% CI, 1.23-3.94). Their data were confirmed in the patients whether the patients were treated with intravenous gamma-globulin. KD patients with TT genotypes had significantly higher levels of C-reactive protein and vascular endothelial growth factor, which had previously been reported as risk factors for CAL, than those with CC genotypes. CONCLUSION: These results indicate that the T allele and TT genotype at CD14/-159 are risk factors for CAL in KD, and that the development of CAL in KD may be related to the magnitude of CD14 toll-like receptor response.

Thromboxane synthesis is increased by upregulation of cytosolic phospholipase A2 and cyclooxygenase-2 in peripheral polymorphonuclear leukocytes during bacterial infection in childhood.

Prostaglandins (PGs) and thromboxane (TX) are important mediators of inflammation. Recent studies revealed that PG and TX synthesis is controlled by the regulation of PG- and TX-synthesizing enzymes. In this study, we examined the TX synthesis and the expression of TX-synthesizing enzymes in activated peripheral polymorphonuclear leukocytes (PMNs) obtained from children with bacterial infection. Blood samples were obtained from controls and patients with bacterial infection. A23187-stimulated production of TXB(2), a stable metabolite of TXA(2) in PMNs, was measured by a specific radioimmunoassay. The mRNA expression of cytosolic phospholipase A(2) (cPLA(2)), cyclooxygenase (COX)-1, COX-2, and TXA(2) synthase was determined by RT-PCR. The synthesis of TXB(2) in PMNs was significantly increased in the patients [925.0 (550.0-1100.0) pg/10(6) cells], compared with the controls [550.0 (450.0-775.0) pg/10(6) cells]. The mRNA expression for cPLA(2) and COX-2 in PMNs was also enhanced in the patients. The results indicate that TX production in PMNs is significantly increased through possible transcriptional mechanisms of cPLA(2) and COX-2 during bacterial infection in children. The upregulation of TXA(2) synthesis may contribute to the process of acute inflammatory reaction caused by bacterial infection.

[Changes of histamine release rate from basophils measured by HRT Shionogi kit in immunotherapy for cedar pollinosis].

We studied changes in histamine release from basophil measured by HRT Shionogi kit as laboratory procedure to evaluate immunotherapy of allergic rhinitis. Nine cedar pollinosis patients (ages 12-64 yrs, 3 male and 6 female) that started immunotherapy with standardized cedar pollen extract (Torii Co) in 2001 were evaluated. Histamine release rate was measured before immunotherapy and at 6 months after starting immunotherapy. As a result, the histamine release rate after immunotherapy was suppressed in all cases. The average of histamine release rate was significantly decreased by the stimulation at 3, 30 and 300 ng/ml with cedar pollen antigen (p<0.05). These data suggested that the measurement of histamine release rate was usable for evaluating the efficacy of immunotherapy, and it has a possibility for monitoring in immunotherapy. In addition, a new different mechanism that T lymphocyte or serum does not concern can be considerable, because the procedure by HRT Shionogi kit is measured from basophils at the condition without serum.

The Role of the Human Dry Dock in Smoking Cessation in Japan.

The human dry dock, a Japanese system of detailed health check-ups for middle-aged and elderly people was originally set up for the purpose of secondary prevention, but it is now expected to increasingly play a role in primary prevention. A series of our studies of smoking cessation in the human dry dock setting showed that the abstinence rate increased from 5-6% for non-advised smokers to 9-10% for those who were advised. Thoracic CT screening participants were found to be more likely to quit smoking. It can be estimated that an additional seventy thousand male smokers would quit smoking every year if advice on smoking cessation was routinely given in every dock in Japan.

The screening test for chemotherapeutic agents for leprosy

In view of the fact that there is no generally-accepted, uniform method for screening therapeutic agents for leprosy using murine leprosy, the methods used differing with each investigator so that the results are inconsistent, the need for a standard method was felt. Consequently, detailed basic studies were designed for the selection of a standard strain of murine leprosy for the purpose, the most suitable site of inoculation and quantity of inoculum to be used, the simplification and constancy of evaluation of results, and the selection of the best mouse strain. On the basis of the results of these studies a screening method, using the subcutaneous inoculation method, which might serve as a standard was devised.

Studies on the immunology of murine leprosy effects of vaccinations and previous infection

1- Injection of a saline vaccine of heat-killed murine leprosy bacilli some time prior to inoculation of live bacilli, causes inhibition of onset (prophylaxis) in a relatively high degree, although not totally. When the vaccine is given simulataneously with inoculation of live bacilli, there is a distinct but lesser degree of inhibition of onset. 2- Injection of a vaccine prepared by adding liquid paraffin to killed murine leprosy bacilli did not completely prevent infection by a challenge inoculation of live bacilli, but onset was strongly inhibited. The effect of this vaccine was stronger than that of the saline vaccine. 3- Administration of a liquid-paraffin vaccine of nonpathogenic acid-fast bacilli in place of murine leprosy bacilli resulted in a slight degree of immunity. 4- When secondary infection was attempted in rats already infected with murine leprosy, resistance greater than that induced by injectionnof the liquid paraffin vaccine was observed. When the challenge inoculum was a 0.5 cc. dose of a 10-4 leproma suspension, complete prevention was observed. 5- An acute reaction of the skin over the site of the reinoculation dose, resembling the Koch phenomenon in tuberculosis, was not observed. The size of the primary lesion did not influence the progress of the primary lesion. It is indicated that a certain degree of immunity can be attained by injection of killed or live murine leprosy bacilli in rats, and that it is also possible to obtain such an effect nonspecifically but in lesser degrees by injection of vaccines of other acid-fast bacilli.

Intracutaneous injection of acidomycin (Satanis method) in the treatment of leprosy

1- Acidomycin, a new antibiotic prepared in the Takata Laboratory, has been employed in the treatment of leprosy. 2- In this experiment the antibiotic was injected superficially into the leprous skin lesions, because when injected subcutaneously or intravenously acidomycin has shown no therapeutic effect in either tuberculosis or leprosy, although it is very active against the tubercle bacillus in vitro. 3- With intracutaneous application, on the contrary, there has been demonstrated a very interesting action. Where it was effective there was fading or disappearance of the macules and improvement or recovery of sensitivity. In certain cases there was decrease or disappearance of bacilli in the faded macules. 4- In several cases, lesions at a distance from those that were injected also showed improvement. In one of them a nodular auricularis magnus nerve became much reduced.