Improvement of diagnostic criteria in growth hormone insensitivity syndrome: solutions and pitfalls

A survey to identify children and adolescents with primary growth hormone insensitivity syndrome (GHIS) yielded 38 patients who were positively identified using a scoring system that included five criteria: height, basal growth hormone (GH), GH binding protein, basal insulin-like growth factor 1 (1GF-I) and the increase of IGF-I after 4 days of GH administration (IGF generation test). Because of an overlap of the accepted and excluded groups with respect to points scored, an attempt was made to improve the scoring system. The new criteria were: height below –3 SDS, basal GH 4 mU/I or above, GH binding below 10%, basal IGF-I and basal IGF binding protein-3 (IGFBP-3) below the 0.1 centile for age, an increase of IGF-I in the IGF generation test less than 15 μg/1, and the increase of IGFBP-3 less than 0.4 mg/1. With this scoring system, a clear separation between the accepted and the excluded groups was obtained. IGFBP-3 was included to give the GH-dependent parameters of the IGF system more weight and because the accuracy of IGFBP-3 in the IGF generation tests was greater than the accuracy of IGF-I, when the group of patients with GHIS was compared with a group of patients with GH deficiency. Unexpectedly, the IGF generation test was unable to segregate both cohorts completely. In the GHIS-positive group, a significant correlation was found between basal IGF-I or IGFBP-3 levels corrected for age (SDS) and height SDS ( r = 0.49, p < 0.002 and r = 0.61, p < 0.0001, respectively). There was also a significant correlation between the changes of IGF-I or IGFBP-3 in the IGF generation test and height SDS. That is, the patients with a slight response to GH were those with the least growth retardation, suggesting the existence of partial GH insensitivity.     

Massive symptomatic subependymoma of the lateral ventricles: case report and review of the literature

Subependymomas are benign intraventricular tumors with an indolent growth pattern, which are usually asymptomatic, and most commonly occur in the fourth and lateral ventricles. When symptomatic, subependymomas often obstruct critical portions of the cerebrospinal fluid (CSF) pathway, causing hydrocephalus, and range from 3 cm to 5 cm in size. We report a case of an unusually massive subependymoma of the lateral ventricles treated with subtotal resection, ventriculoperitoneal shunt, and post-surgical radiation. The clinical course, radiographic and pathologic characteristics of this massive intraventricular subependymoma are discussed, as well as the differential diagnosis of lateral ventricular masses and a review of the literature concerning subependymomas.     

一阶导数光谱法测定复方红甲凝胶中乳糖酸红霉素的含量

建立测定复方红甲凝胶中乳糖酸红霉素的含量测定方法。方法：以一阶导数光谱的谷一零位值法测定乳糖酸红霉素的含量,测定波长λ谷=490±lnm。结果：回收率100.6％,RSD为3.1％,线性范围40～120μg／ml。结论：方法简便,结果准确,重现性好,适合于该制剂的含量测定。     

几种伪随机序列应用于诱发电位检测的性能比较

伪随机序列技术作为一种快速检测诱发电位的技术已得到重视。本文主要对几种常用序列Ｍ序列、巴克序列和格雷互补序列等应用于诱发电位检测时的性能进行研究，分别给出了它们的解卷积算法及用于诱发电位检测时相对于平均法的信噪比改善倍数。理论及实验表明，Ｍ序列在检测信噪比改善方面较另两种序列好。     

肾移植术后乙型肝炎病毒拉米夫定耐药的治疗

目的:观察肾移植术后乙型肝炎病毒患者发生拉米夫定耐药后,应用新一代核苷类抗乙型肝炎病毒药物阿德福韦和恩替卡韦的治疗效果。方法:选择2001-05/2006-09于南方医科大学南方医院器官移植科就诊的乙型肝炎病毒感染并发生病毒多聚酶催化域YMDD基序变异,对拉米夫定耐药的肾移植患者4例,患者均知情同意。定期复查血清丙氨酸转氨酶、天冬氨酸转氨酶、总胆红素、乙型肝炎病毒DNA定量及乙型肝炎病毒YMDD变异情况,先后采用拉米夫定、阿德福韦及恩替卡韦进行治疗,并观察其治疗效果。结果:①4例患者分别随访16,26,63,67个月,移植肾功能良好。②4例患者分别在服用拉米夫定14,24,51和62个月(术后12个月)时出现乙型肝炎病毒YMDD变异,乙型肝炎病毒DNA升高至105～109拷贝/mL,肝功能异常。2例加用阿德福韦3个月后撤除拉米夫定,单用阿德福韦治疗,乙型肝炎病毒DNA均降为104拷贝/mL,肝功能正常,其中1例单用阿德福韦9个月后乙型肝炎病毒YMDD变异转阴,另1例单用阿德福韦2个月,乙型肝炎病毒YMDD变异仍为阳性;2例停用拉米夫定,直接换为恩替卡韦,其中1例2个月后肝功能正常,乙型肝炎病毒DNA阴性,乙型肝炎病毒YMDD变异阴性,另1例服药2个月后肝功能正常,乙型肝炎病毒DNA降为104拷贝/mL,乙型肝炎病毒YMDD变异仍为阳性。结论:新型抗乙型肝炎病毒药物阿德福韦和恩替卡韦可有效抑制拉米夫定耐药的乙型肝炎病毒复制,有利于乙型肝炎病毒阳性肾移植患者长期存活。     

Percutaneous transluminal dilatation of the iliac artery: long-term results

One hundred fifty-four patients with stenosis of the iliac artery underwent percutaneous transluminal angioplasty (PTA). These patients were followed for 1-7 years. The long-term results of the PTAs were analyzed by computer, and life tables were generated for dilatations of the iliac arteries with unimpaired flow and for those with an obstruction in the outflow tract. The accumulative 7-year patency rate was 90%, which agrees with other reports. This study demonstrates that the long-term results of PTA of iliac arterial stenoses are competitive with reconstructive vascular surgery. PTA should be the treatment of choice in patients with iliac arterial stenoses.     

Signaling and induction of enhanced cytoadhesiveness via the hematopoietic progenitor cell surface molecule CD34

The transmembrane glycoprotein CD34 shows a highly restricted expression on a crucial subset of hematopoietic cells. We show here that engagement of particular determinants of CD34 can lead to signal transduction and to enhanced adhesiveness of CD34+ hematopoietic cells. Monoclonal antibodies (MoAbs) directed against O-sialoglycoprotease- sensitive epitopes of CD34 (QBEND10, ICH3, BI.3C5, MY10) but not MoAbs against O-sialoglycoprotease-resistant epitopes (9F2, 8G12) induce actin polymerization in KG-1a and KG-1 cells and strongly enhanced cytoadhesiveness. The capacity to induce adhesion requires cellular energy, divalent cations, and intact cytoskeleton but not de novo protein synthesis. The observed cytoadhesion seems at least in part to be caused by a concomitant activation of the beta 2 integrin cytoadhesion pathway. It can be significantly inhibited with lymphocyte function-associated antigen-1 and intercelluar adhesion molecule-1 antibodies. Protein kinase inhibition analyses suggest that the pathways initiated by engagement of the CD34 molecule with certain CD34 MoAbs involves protein tyrosine kinases but that protein kinase C is not critically involved.     

Generation of CD4+ cytotoxic T-lymphocyte clones from a patient with severe graft-versus-host disease after allogeneic bone marrow transplantation: implications for graft-versus-leukemia reactivity

HLA-identical bone marrow transplantation (BMT) is associated with both graft-versus-host disease (GVHD) and graft-versus-leukemia (GVL) reactivity. Different T-cell subsets from the bone marrow (BM) graft may be responsible for GVHD and GVL reactivity after BMT. In the etiology of GVHD, not only CD8+ but also CD4+ donor T lymphocytes may play an important role. Here we report a patient with chronic myeloid leukemia (CML) who was transplanted with the BM from his HLA- genotypically identical sister. After BMT there was complete engraftment, but the patient died because of acute GVHD grade III-IV in complete remission. Cytotoxic T-lymphocyte (CTL) lines were generated after BMT using the irradiated leukemic cells from the patient as stimulator cells and the donor-originated peripheral blood mononuclear cells, procured from the patient after BMT, as responder cells. The generated CTL lines showed specific lysis of the recipient lymphocytes and leukemic cells in a 51Cr release assay. Two types of CTL clones could be established from these CTL lines, both phenotypically CD4+. Clone type I showed male-specific HLA-DQ5-restricted lysis of the recipient lymphocytes, but not of the circulating relatively mature leukemic cells from the patient. This may be explained by the low HLA- DQ5 expression of the more mature CML cells. Clone type II showed HLA- DR2-restricted minor histocompatibility antigen-specific lysis of the recipient lymphocytes and leukemic cells. Both types of CTL clones showed antigen-specific cell-mediated growth inhibition of the recipient clonogenic leukemic precursor cells. These CD4+ CTL clones produced several activating cytokines including tumor necrosis factor alpha, interferon gamma, granulocyte-macrophage colony-stimulating factor (GM-CSF), and macrophage CSF. Our results illustrate that these CD4+ CTL clones may have induced GVHD directly by cytolysis and indirectly by activating cytokines. Because both types of CTL clones recognized the recipient leukemic progenitor cells, they may also contribute to GVL reactivity after BMT.