Consequences of fluid absorption during transurethral resection of the prostate using distilled water or glycine 1.5 per cent

This prospective and randomized study compared the consequences of two irrigating fluids, distilled water and glycine for transurethral prostatectomy. Forty-nine consecutive unselected patients undergoing transurethral resection of the prostate with spinal anaesthesia were investigated. The irrigating fluid was either distilled water (group A, 24 patients) or glycine 1.5 per cent (group B, 25 patients). The absorption of irrigating fluid was measured, all surgical events and any clinical signs of TURP syndrome during and after surgery were recorded. Early signs of TURP syndrome were observed in one patient in group A and in four in group B without further consequence. From all the biological variables, only plasma protein concentration, haematocrit, free plasma haemoglobin and free bilirubin concentrations were found to have changed. Plasma protein concentration and haematocrit decreased significantly during and after surgery in the two groups. Free plasma haemoglobin increased significantly with time: a significantly higher concentration was observed in group A than group B. Free bilirubin concentration increased with time in the two groups and was statistically greater in group A. With the two irrigating fluids, we observed a significant amount of haemolysis and haemodilution without clinical consequences. A low irrigating fluid pressure, a short resection time, and the use of spinal anaesthesia seems to us to be essential. Close observation of patients following transurethral prostatectomy is needed but the choice of the irrigating fluid does not seem to be important. Cette étude prospective et randomisée a comparé les conséquences de deux liquides d’irrigation, l’eau distillée et le glycocolle au cours de la chirurgie prostatique par voie basse. Quarante-neuf patients consécutifs, non sélectionnés, bénéficiant d’une résection transuréthrale de prostate sous rachianesthésie, sont étudiés. Le liquide d’irrigation est soil l’eau distillée (groupe A, 24 patients), soit le glycocolle à 1.5 pour cent (groupe B, 25 patients). L’absorption des liquides d’irrigation, les incidents chirurgicaux et les signes cliniques du syndrome de réabsorption sont systématiquement notés. Des signes caractéristiques du syndrome de réabsorption sont observés dans un cas dans le groupe A et dans quatre cos dans le groupe B sans conséquence ultérieure. Seuls la protidémie, l’hématocrite, l’hémoglobine plasmatique libre et la bilirubine libre changent significativement. L’hémoglobine plasmatique libre augmente significativement dans les deux groupes, avec un taux plasmatique significativement plus élevé dans le groupe A. Avec les deux liquides d’irrigation, on observe une hémolyse et une hémodilution sans conséquence clinique. Un temps de résection court, un faible niveau de pression d’irrigation, l’utilisation de l’anesthésie rachidienne nous semblent trés importants. Une surveillance clinique et biologique attentive pendant et après la résection transuréthrale de prostate sont nécessaires; en revanche, le choix du liquide d’irrigation ne semble pas l’élément essentiel.     

呼出气氢测定试验对飞行人员乳糖酶缺乏症的研究

对66名汉族飞行人员进行乳糖呼出气氢测定试验,乳糖吸收不良的发生率为83.3％,其中乳糖不耐受者占34.6％;与一般汉族人群无明显差别。对10名确定为中度以上乳糖吸收不良的飞行人员进行250ml鲜牛奶的试验结果,有50％呼出气氢含量在正常范围,并无一例出现胃肠道症状。提示较长期食用牛奶未能使乳糖酶缺乏状态发生改变,但每日食用适量牛奶属合理营养。     

SARS冠状病毒N蛋白单克隆抗体的制备及鉴定

目的 制备SARS冠状病毒(SARS-CoV)N蛋白特异性单克隆抗体(McAb)，为SARS的快速诊断及致病机制的研究提供实验材料。方法 用纯化的重组SARS-CoVN蛋白免疫BALB/c小鼠，经细胞融合和亚克隆后获得分泌针对N蛋白的杂交瘤细胞株，用Western blot和间接免疫荧光法检测这些细胞株分泌的单克隆抗体特异性，并将N蛋白分3段表达初步定位单克隆抗体识别表位所在区域。结果 通过细胞融合和3轮克隆化，筛选出分泌抗N蛋白的6个杂交瘤细胞株。Western blot及免疫荧光显示，获得的McAb可与SARS-CoVN蛋白及SARS-CoV发生特异性反应，有4个细胞株分泌的抗体的识别位点位于N蛋白N端，2个位于C端。结论 获得了SARS-CoV特异性单克隆抗体并进行了初步定位，可用于SARS的早期诊断及致病机制研究。     

Baraitser–Winter cerebrofrontofacial syndrome: Report of two adult siblings

Baraitser–Winter cerebrofrontofacial syndrome (BWCS) is a rare, autosomal dominant condition that is characterized by intellectual disability, distinctive craniofacial features, structural brain abnormalities, seizures, microcephaly, hearing loss, and ocular colobomas. The first three cases were described in 1988 by Baraitser and Winter and included two siblings and an unrelated third patient. Subsequently, causative missense variants in the ACTB and ACTG1 genes were identified, with de novo occurrence in patients with the condition. Herein, we describe two adult siblings who were born to unaffected parents and who were diagnosed with BWCS in their fourth and sixth decade of life following exome sequencing performed for intellectual disability. We review the literature reports of adult patients with BWCS to document the clinical features and phenotypic variability that can occur later in life. This is the first molecularly confirmed report of germline mosaicism in BWCS and one of only a few reports to describe two BWCS patients belonging to the same family.     

Costello syndrome: Clinical phenotype,genotype, and management guidelines

Costello syndrome (CS) is a RASopathy caused by activating germline mutations in HRAS. Due to ubiquitous HRAS gene expression, CS affects multiple organ systems and individuals are predisposed to cancer. Individuals with CS may have distinctive craniofacial features, cardiac anomalies, growth and developmental delays, as well as dermatological, orthopedic, ocular, and neurological issues; however, considerable overlap with other RASopathies exists. Medical evaluation requires an understanding of the multifaceted phenotype. Subspecialists may have limited experience in caring for these individuals because of the rarity of CS. Furthermore, the phenotypic presentation may vary with the underlying genotype. These guidelines were developed by an interdisciplinary team of experts in order to encourage timely health care practices and provide medical management guidelines for the primary and specialty care provider, as well as for the families and affected individuals across their lifespan. These guidelines are based on expert opinion and do not represent evidence‐based guidelines due to the lack of data for this rare condition.     

FG syndrome in a premature male

A 30-week premature male infant is presented with dolichocephaly, frontal bossing, down-slanting palpebral fissures, hypertelorism, long philtrum, micrognathia, cleft palate, and imperforate anus. He is the fifth patient to be presented with FG syndrome and sensorineural deafness. The patient's syndromic manifestations became more obvious during an inpatient observation period of 3 months.     

Changes in activity of some mechanisms of specific and nonspecific immunity in vitamin B1 deficiency

The effect of thiamine deficiency on the immune response and activity of certain mechanisms of natural immunity was studied in adult rats. Thiamine deficiency was simulated experimentally by a single injection of hydroxythiamine, a vitamin B₁ antagonist. Administration of hydroxythiamine caused a marked decrease in complement activity, phagocytic activity of the peripheral blood leukocytes, bactericidal activity of the serum, and antibody production in response to immunization with sheep's red blood cells. Conversely, lysozyme activity increased. In vitamin B₁ deficiency the intensity of incorporation of [¹⁴C]leucine into liver protein synthesis was reduced.Department of Pathological Physiology, N. I. Sechenov First Moscow Medical Institute. (Presented by Academician of the Academy of Medical Sciences of the USSR G. V. Vygodchikov.) Translated from Byulleten' Éksperimental'noi Biologii i Meditsiny, Vol. 88, No. 7, pp. 60–62, July, 1979.     

Holt-Oram syndrome associated with the hypoplastic left heart syndrome

We present the first case of Holt-Oram syndrome associated with the lethal congenital heart defect of hypoplastic left heart syndrome. The possible pathophysiological link is explored and the need for careful genetic and cardiologic evaluation in these patients is reiterated.     

Antibody-dependent cellular cytotoxicity (ADCC)-mediated destruction of human immunodeficiency virus (HIV)-coated CD4+ T lymphocytes by acquired immunodeficiency syndrome (AIDS) effector cells

The acquired immunodeficiency syndrome (AIDS) is defined in clinical terms by the development of Kaposi's sarcoma and/or severe opportunistic infections in persons without predisposing conditions. A hallmark of the syndrome has been a decrease in the number of CD4⁺ T helper cells. The reduction in the frequency of the CD4⁺ lymphocytes has been postulated to be primarily the result of human immunodeficiency virus (HIV) tropism and cytophathogenicity for the T-cell subset. Yet only a small percentage of cells is actually infected with HIV. Recently, we provided evidence indicating that AIDS patients' natural killer cells can mediate normal levels of antibody-dependent cellular cytotoxicity (ADCC) despite exhibiting a defect in natural killer (NK) effector function (J Immunol 139:55, 1987). This finding prompted us to investigate whether AIDS patients' effector cells could mediate ADCC against circulating CD4⁺ T cells infected with or expressing HIV antigen. The findings reported herein demonstrate that AIDS effector cells can mediate lysis of CEM (CD4⁺ T-cell line) coated with HIV protein in the presence of HIV-specific antibody. Lysis was specific, as non-HIV-coated CEM or the addition of HIV-negative serum resulted in no lysis. We then examined HIV-coated peripheral blood-derived CD4⁺ T lymphocytes as targets in ADCC. We demonstrate that in the presence of HIV-specific antibody, HIV-coated CD4⁺ T lymphocytes serve as targets for ADCC by AIDS effector cells. The lytic activity obtained with AIDS effector cells was comparable to that obtained with normal effector cells. These results demonstrate that AIDS effector cells can mediate ADCC against HIV-coated CD4⁺ T lymphocytes and suggest that ADCC may play a rolein vivo in the pathogenesis of AIDS.