RANTES -28G delays and DC-SIGN - 139C enhances AIDS progression in HIV type 1-infected Japanese hemophiliacs

The relationships between host immune factors and HIV-1 disease progression are still in dispute. Unlike CCR5Delta32, which has been found to delay disease progression of HIV-1, there still remain several factors whose effect on the clinical course is unconfirmed. To clarify the relationships, we selected seven single-nucleotide polymorphisms (SNPs) out of the previously reported factors, namely, RANTES promoter -28G/-403A, RANTES In1.1C, SDF-1 3'A, IL-4 promoter -589T, and DC-SIGN promoter -139C/-336C, and examined these in Japanese HIV-1-infected hemophiliacs (n = 102). The genotypes were examined by the direct sequencing method, and the distributions of genotype and allelic frequencies were compared between two groups, slow progressors (n = 54) who did not develop AIDS more than 10 years after intravenous infection and others (progressors) (n = 48). The allelic frequency of RANTES -28G was significantly higher in slow progressors (0.185) than in the progressor group (0.074) [p = 0.023, OR = 0.35, 95% CI (0.142, 0.880)]. DC-SIGN promoter -139C, and appeared in progressors with significantly higher allelic frequency (0.333) than slow progressors [0.204, p = 0.040, OR = 1.95, 95% CI (1.039, 3.677)]. With RANTES -403A, RANTES In1.1C, SDF-1 3' A, IL-4 -589T, and DC-SIGN -336C, no significant difference was observed in allelic frequencies between the two groups. These results suggest that RANTES -28G was associated with delayed AIDS progression, while DC-SIGN -139C was associated with accelerated AIDS progression in HIV-1-infected Japanese hemophiliacs.     

Oligoclonal Immunoglobulin Gene Rearrangements in Philadelphia Chromosome-positive Common Acute Lymphoblastic Leukemia

The occurrence of more than two rearranged bands of immunoglobulin heavy chain (IgH) genes in B precursor acute lymphoblastic leukemia (ALL) has recently been documented. To elucidate the nature of such leukemias, we studied 30 patients with common ALL, including 6 patients with Philadelphia chromosome (Ph¹)-positive ALL, by immunophenotyping and genotyping. In 10 of the 30, Southern blotting showed oligoclonal patterns of IgH gene arrangements, which were frequently detected in Ph¹-positive ALL. In one patient of the 10, three rearranged bands of Ig k chain genes were detected. Ph¹ abnormality and co-expression of myeloid associated antigens were found in 5 and 5 of the 10, respectively. Detection of multiple fragments of IgH genes would be suggestive of multipotent progenitor origin of these ALL.     

铁过剩时铁在肝小叶内区域性沉积的机理

用Ｗｉｓｔａｒ大鼠通过词以缺铁词料或腹腔内反复注射铁。氨三乙酸络合物４个月制成铁缺乏或铁过剩大鼠模型。用ＡＢＣ法及铁染色法观察、分析铁缺乏（Ａ组）、铁过剩（Ｂ组）与对照（Ｃ组）大鼠肝脏转铁蛋白（Ｔｆ）及其受体（ＴｆＲ）的表达与铁沉积的关系。与Ｃ组相比，ＴｆＲ的表达在Ａ组增强、Ｂ组减弱。三组动物ＴｆＲ在肝小叶第１区表达最强，第２区次之，第３区最弱。三组动物Ｔｆ表达差异与ＴｆＲ类似。Ｂ组肝小叶内可见铁沉积。以１区分布最多，３区最少；与ＴｆＲ表达在肝小叶分布一致。作者认为体内肝细胞摄取铁主要经ＴｆＲ与Ｔｆ介导，ＴｆＲ和Ｔｆ的区域性分布可导致铁过剩时肝小叶内铁从第１区至第３区的顺序递减型区域性沉积。     

Effect of a factor VIII concentrate on type IIB von Willebrand's disease-associated thrombocytopenia presenting during pregnancy in identical twin mothers

Marked thrombocytopenia developed during pregnancy in both identical twins mothers who had systemic lupus erythematosus (SLE) and also type IIB von Willebrand's disease (vWD). The proband's platelet count decreased in the third trimester of pregnancy. Large-dose gamma-globulin and prednisolone treatments were performed because of the suspicion of immune thrombocytopenic reaction associated with SLE. These treatments were not effective. Her platelet count returned to the normal range immediately after delivery. Postpartum examinations revealed the decreased ristocetin cofactor activity and the deficiency of large von Willebrand factor (vWF) multimers in preserved plasma samples from the third trimester. These abnormal findings improved after delivery. Investigation of family members revealed that the proband had inherited type IIB vWD from her mother. The other twin, who was also under treatment for SLE, became pregnant about 1 year after delivery in the proband and followed almost the same course as that observed in the proband. As bleeding tendency was observed a few days before delivery, a factor VIII concentrate (Haemate P) was administered to compete with her variant vWF. This concentrate could prevent the further decrease in her platelet count, thereby correcting the hemorrhagic tendency. It seems evident that factor VII concentrate would be effective in treating thrombocytopenia associated with type IIB vWD.     

Mammoplasty using liposuction and the periareolar incision

The major dilemma of reduction mammoplasty and mastopexy has been the difficult choice between a procedure that yields an ideal shape of the breast versus the size of the scar. With our technique, selected breasts can now be reduced through liposuction and the mastopexy performed through a periareolar incision, resulting in virtually imperceptible scarring.     

The relationship between social capital and self-rated health in a Japanese population: a multilevel analysis

Objective  

The aim of this study was to use a multilevel analysis to examine whether cognitive and structural dimensions of regional social capital were associated with individual health outcomes after adjusting for compositional factors.     

ESHAP therapy effective in a patient with Langerhans cell sarcoma

We describe the rare case of a 53-year-old woman with systemic involvement of Langerhans cell sarcoma (LCS) who had undergone living-related liver transplantation. We chose the CHOP regimen as first-line chemotherapy, and clinical improvement of LCS was obtained. Intensive care was necessary due to the systemic involvement of LCS and severe infectious diseases. After the third cycle of CHOP therapy, however, disease progression was observed, and we administrated a modified ESHAP regimen (etoposide, carboplatin, cytarabine, methylprednisolone) as second-line therapy. A marked response was obtained after four cycles of this combination chemotherapy. Modified ESHAP may be a very effective combination chemotherapy regimen for LCS.     

Pulmonary varix clearly demonstrated by 3D-CT before pulmonary angiography

Pulmonary varix is a rare and usually asymptomatic localized dilation of a pulmonary vein. This disease should be distinguished from other pulmonary and mediastinal diseases, particularly pulmonary arteriovenous malformations. Herein, we encountered a case of pulmonary varix clearly demonstrated by 3-dimensional reconstructed computed tomography (3D-CT) which proved useful in its diagnosis. The 3D-CT enabled easy understanding of the vascular connections and confirmation of the absence of an inflow pulmonary artery. We also performed angiography which showed findings consistent with those obtained by the 3D-CT, thus confirming the diagnosis of pulmonary varix. After the diagnosis, the patient was followed up for several years without any treatment and she remained asymptomatic. On follow-up CT, the lesion remained unchanged.