Late-onset focal dermal elastosis: clinical and histological features

We describe two patients with lesions clinically resembling pseudoxanthoma elasticum and histologically exhibiting focal elastosis. with normal-appearing elastic fibres in the mid- and deep dermis. We consider that these skin lesions represent a previously undescribed entity, whose pathogenesis appears to be related to the ageing process.     

Time-dependent Effects of Klebsiella pneumoniae Endotoxin on Hepatic Drug-metabolizing Enzyme Activity in Rats

The time-dependent effects of Klebsiella pneumoniae endotoxin on hepatic cytochrome P450-dependent drug-metabolizing capacity (cytochrome P450 and b₅ content, activity of aminopyrine N-demethylase, p-nitroanisole O-demethylase, aniline hydroxylase and benzphetamine N-demethylase) and on the pharmacokinetics of antipyrine have been determined in rats. Measurement of enzyme activity and antipyrine (after intravenous injection of 20 mgkg^?1) were performed 2, 24 and 96 h after a single intraperitoneal injection of endotoxin (1 mgkg^?1) and after repeated doses (once daily for 4 days). The contribution of tumour necrosis factor α (TNFα) to the endotoxin-induced changes was also examined in rats pretreated with granulocyte colony-stimulating factor (G-CSF). The systemic clearance of antipyrine and the activity of hepatic cytochrome P450-dependent drug-metabolizing enzymes were dramatically reduced 24 h after a single injection of endotoxin, but had returned to control levels by 96 h. The magnitudes of these decreases in these measurements after repeated doses of endotoxin were similar to those seen 24 h after the single dose. The systemic clearance of antipyrine correlated significantly with cytochrome P450 content and aminopyrine N-demethylase activity. In histopathological experiments, moderate hypertrophy of Kupffer cells was observed, with no evidence of severe liver-tissue damage. G-CSF pretreatment suppressed the increased plasma concentrations of TNFα produced 2 h after single endotoxin injection, but did not eliminate the endotoxin-induced decrease in the systemic clearance of antipyrine, suggesting that TNFα is not the sole component responsible for the reduction of cytochrome P450-mediated drug-metabolizing enzyme activity. These results provide evidence that a single intraperitoneal injection of 1·0 mgkg^?1 K. pneumoniae endotoxin in rats reduces hepatic P450 and b₅ levels, and reduces the activity of various cytochrome P450-mediated drug-metabolizing enzymes without causing severe liver-tissue damage. This suggests that the effect of endotoxin on hepatic cytochrome P450-mediated drug-metabolizing isozymes is non-selective.     

T cell receptor (TCR) V gene segment use in HLA-typed Japanese healthy subjects

The expression of 13 different α and β V gene segments of the T cell receptor for antigen (TCR) was examined, using V gene-specific MoAbs, on human peripheral blood T lymphocytes from 32 healthy Japanese subjects. In addition, to examine associations between TCR V gene products and HLA alleles, the HLA class I and class II types of all subjects were serologically determined. The reactivities of the anti-TCR V-specific MoAbs were, with some significant exceptions, similar to those previously described in healthy Caucasian subjects. We found a non-random V gene usage as well as a statistically significant bias of the expression of eight Vβ gene products towards the CD4⁺ subpopulation, and a significant skewness in the usage of Vα12 towards the CD8⁺ population. Some subjects showed increased reactivities (above 10%) of certain MoAbs, mainly in the CD8⁺ subpopulation. We found no distinct correlation between any certain HLA class I or II allele and TCR V gene usage in the CD8⁺ or CD4⁺ subpopulations, respectively. In conclusion, the pattern of anti-TCR V-specific MoAb reactivities found in CD4⁺ and CD8⁺ subsets of peripheral blood lymphocytes of healthy Japanese subjects was in general found to match that previously described in healthy Caucasian subjects.     

Aqueous fraction of Sauropus androgynus might be responsible for bronchiolitis obliterans

Background and objective: Bronchiolitis obliterans (BO) has been reported to develop following ingestion of Sauropus androgynus (SA), a leafy shrub distributed in Southeast Asia. Little is known about direct effects of SA on airway resident cells or haematopoietic cells in vitro. Identification of the SA component responsible for the development of BO would be an important key to elucidate its mechanism. We sought to elucidate the direct effects of SA on airway resident cells or haematopoietic cells and identify the SA element responsible for the pathogenesis of BO. Methods: SA dry powder was partitioned into fractions by solvent extraction. Human and murine monocytic cells, epithelial cells and endothelial cells were cultured with SA solution or fractions eluted from SA. We also investigated the effect of SA in vivo using a murine BO syndrome (BOS) model. Results: The aqueous fraction of SA induced significant increases of inflammatory cytokine and chemokine production from monocytic lineage cells. This fraction also induced significant apoptosis of endothelial cells and enhanced intraluminal obstructive fibrosis in allogeneic trachea allograft in the murine BOS model. We found individual differences in tumour necrosis factor α (TNF‐α) production from monocytes of healthy controls stimulated by this aqueous fraction of SA, whereas it induced high‐level TNF‐α production from monocytes of patients with SA‐induced BO. Conclusions: These results suggest that an aqueous fraction of SA may be responsible for the pathogenesis of BO.     

Apolipoprotein E genotype and cerebral palsy

Aim Apolipoprotein E (APOE, protein; [ApoE, gene]) is a lipid transport protein abundantly present in brain cells. We investigated whether the APOE genotype is associated with cerebral palsy (CP) and whether patients with CP with comorbid conditions and more severe neurological deficits are likely to have a particular genotype. Method In a cross‐sectional study, 243 individuals with spastic CP (135 males, 108 females; mean age at data collection 11 year ([SD 6y 7mo], 34% with hemiplegia, 37% with diplegia, 29% with triplegia/tetraplegia; 44% with mild motor involvement), 31% with moderate motor involvement, 25% with severe motor involvement, were compared with healthy individuals matched by age, race, and sex to analyse the association between APOE genotype and the incidence of CP. Associations between the APOE genotype and the incidence of comorbidities and neurological deficits were studied in the group with CP. Results The APOE ε2ε3 genotype was significantly more prevalent in the group with CP (11%) than the comparison group (5%) (odds ratio [OR] 2.8; 95% confidence interval [CI] 1.01–7.66). The presence of the ε2 allele raised the probability of having CP (OR 3.2; 95% CI 1.27–8.27). The presence of ApoE ε4 was not significantly different among groups. No relation was found between APOE genotype and severity of neurological deficit or distribution of motor involvement. Four patients with CP presented the ε4ε4 genotype, and all exhibited epilepsy and microcephaly. Eleven of 12 individuals with CP and macrocephaly carried the ε3ε3 genotype. Interpretation A higher prevalence of the APOE ε2 genotype was found among those with CP. The association of microcephaly and epilepsy with the ε4ε4 genotype and the association of macrocephaly with ε3 demand further investigation.     

Growth hormone deficiency in Dubowitz syndrome

Severe short stature as a result of intra-uterine growth retardation is one of the characteristics of Dubowitz syndrome. There have been few reports elaborating growth hormone secretory status in this syndrome. A child with Dubowitz syndrome, who was found to have complete growth hormone (GH) deficiency and who responded to growth hormone therapy, is described. This appears to be the first documentation of GH deficiency in this syndrome.     

Severe chronic active giftein-Barr virus infection accompanied by virus-associated hemophagocytic syndrome,cerebellar ataxia and encephalitis

We present a rare case of chronic active giftein-Barr virus (EBV) infection showing various clinical outcomes. A 26-year-old man was admitted to our hospital due to persistent fever and dyspnea. Serologic response of the patient to EBV indicated chronic active infection. He showed pleuritis, parotitis, chronic hepatic dysfunction, disseminated intravascular coagulation, virus associated hemophaghocytic syndrome, acute rhabdomyolysis, acute renal failure, acute cerebellar ataxia, encephalitis and multiple brain abscesses. None of acyclovir, gancyclovir, prednisolone or interleukin-2 was effectual to abolish those abnormalities. This is the first report of transient cerebellar ataxia which aggravated to panencephalitis associated with chronic EBV infection.     

Hemophagocytic lymphohistiocytosis in a human immunodeficiency virus-positive homosexual high school student

The spread of human immunodeficiency virus (HIV) infection has exploded over the past two decades and such infections in young people are no longer uncommon. However, the major infection route of pediatric patients remains vertical transmission, and sexual, especially homosexual transmission, is highly unusual. We herein describe the case of a 17-year-old boy who developed hemophagocytic lymphohistiocytosis (HLH). Although HLH was remitted soon with dexamethasone therapy, an HIV infection caused by homosexual transmission was detected.     

APAAP高敏感信号放大技术在乳腺癌HPV DNA原位杂交检测中的应用

目的　探讨原位杂交 APAAP信号放大技术检测福尔马林固定 石蜡包埋乳腺癌组织HPV感染的实用性。方法　利用HPV通用型及亚型特异性探针 ,设定 4种杂交检测程序对 73例进展期乳腺癌和 34例乳腺良性病变进行HPVDNA原位杂交 APAAP信号放大检测。结果　在适宜的杂交程序下 ,39 19%的乳腺癌病例检测到HPVDNA ;良性病变中仅 1例 (2 94 % )纤维腺瘤检测到HPVDNA ,明显低于乳腺癌组 (P <0 0 1)。乳腺的大汗腺样化生区有细胞核阴性 细胞浆着色的假阳性反应 ,假阳性反应不足以被 1mmol L左旋咪唑阻断 ,但能被 0 2mol L盐酸前处理所消除。APAAP信号检测各步骤间的不同缓冲液可影响杂交结果。结论　只要杂交条件得当 ,原位杂交 APAAP检测技术在乳腺组织HPV感染研究中可获得满意结果。乳腺大汗腺样化生区内源性碱性磷酸酶含量较高 ,低浓度盐酸前处理有消除内源性碱性磷酸酶作用。