Poikilocytic hereditary elliptocytosis associated with spectrin Alexandria: an alpha I/50b Kd variant that is caused by a single amino acid deletion

Hereditary elliptocytosis (HE) is a heterogeneous disorder of red blood cells frequently associated with abnormal limited tryptic digestion of the alpha I domain of spectrin and impaired spectrin dimer self- association. We studied two related individuals with poikilocytic hereditary elliptocytosis (HE) of different severity. Limited tryptic digestion of spectrin from these individuals showed the presence of a variant alpha I/50b Kd peptide at the expense of the normal alpha I/80 Kd peptide. Amino acid sequence analysis of the abnormal peptide showed that the proteolytic cleavage occurred after the arginine at position 470 of the alpha spectrin chain. Spectrin from these patients had an impaired ability to undergo self-association, as evidenced by increased amounts of spectrin dimers in 4 degrees C extracts of erythrocyte membrane from affected individuals. The polymerase chain reaction was used to study the DNA sequence of the alpha spectrin gene encoding the region of the alpha spectrin chain surrounding the abnormal proteolytic cleavage site. We detected the in-frame deletion of the trinucleotide CAT, encoding histidine 469, two amino acid residues to the N-terminal side of the abnormal proteolytic cleavage site between residues 470 and 471. Similar to many other defects of spectrin associated with HE, this deletion occurs in helix three of repeat 5 of the proposed triple helical model of spectrin repeats.     

An undifferentiated variant derived from the human acute myelogenous leukemia cell line (KG-1)

A variant subline (KG-1a) of the human acute myelogenous leukemia (AML) cell line (KG-1) has been isolated. The cells retain the same constitutive markers as the parent line, including HLA antigens, isoenzymes, and karyotype. The cells from the subline are morphologically and histochemically undifferentiated blast cells, while the parent cells and several of its clones are at the myeloblast and promyelocyte stages of development. The variant cells do not respond to colony-stimulating factor (CSF), and they do not express the human la antigen, nor a recently characterized AML antigen. The parent KG-1 cells are stimulated to proliferate in the presence of CSF and the cells express the la and AML antigen. Variant AML cell lines, such as KG-1a, will be useful in vitro models for investigating cellular response to CSF and for studying antigen expression in leukemic cells.     

Surface shield: device to reduce personnel radiation exposure

A simple device is described that can reduce personnel exposure from scatter radiation by up to 75%. The device consists of an oblong piece of shielding (0.75-mm lead equivalent) that is taped to the side of the patient during percutaneous renal stone removal and other interventional procedures. Contrary to other shields and barriers, this does not interfere with access to the patient. Scatter exposure data from phantom studies are presented and the rationale for surface shielding discussed.     

Colorectal neoplasms: accuracy of US in demonstrating the depth of invasion

Six normal and 16 neoplastic colorectal specimens were examined with 8.5-MHz ultrasound (US). An articulated system facilitated precise spatial correlation between US and histologic sections. Images were blindly interpreted and then compared with histologic results. All six normal specimen showed five distinct echo layers and were distinguished from neoplastic specimens by all the observers. The central echogenic layer, corresponding to the submucosa, is useful in determining the depth of origin of a neoplasm and the presence of submucosal invasion. US had an accuracy of 92.5% in demonstrating invasion of the submucosa and 77% for invasion of the muscularis externa. For mucosal neoplasms with invasion through the muscularis externa and extension into the subserosal tissues, nearly 90% of US interpretations were correct. High-frequency US may be useful in determining the depth of invasion of mucosal tumors with respect to the submucosa and in differentiating mucosal from extramural masses.     

Urine-compatible polymer for long-term ureteral stenting

Internal double-J ureteral stents were designed from a urine-compatible polymer (C-Flex), and 35 stents were placed in patients. The overall patency rate for the stents was 80%, with most stent failures occurring before 2 months; the follow-up period ranged from 2 to 16 months, with a mean follow-up for all stents of 5.0 months. Stents were considered patent at last follow-up only if they had been in place for at least 2 months. No migration or fracture of the stents occurred. Physical properties of urine-exposed stents were compared with those of virgin tubing and tubing exposed for 1 year to shelf conditions. Stent patency was optimized by increasing urine flow by increasing the patient's voluntary oral intake, administering prophylactic oral antibiotics, and avoiding placement of stents into grossly bloody or infected collecting systems.     

Meniscal tears of the knee: accuracy of MR imaging

Before surgery, 277 menisci in 144 knees were examined with magnetic resonance (MR) imaging. They were then examined directly with arthroscopy or arthrotomy. Menisci were graded on a scale of 1-3 according to the character of the intrameniscal MR imaging signal. At surgery, 137 of 154 (89%) menisci exhibiting only grade 1 or grade 2 signal were found to be normal. One hundred sixteen of 123 (94%) menisci exhibiting intrameniscal signal communicating with a meniscal articular surface (grade 3 signal) had tears. If only a grade 3 signal is considered consistent with meniscal tears, then MR findings and surgical findings agreed in 91.3% of menisci. MR imaging can separate surgically significant from nonsignificant meniscal lesions and is useful in the noninvasive preoperative screening of suspected meniscal tears.     

商陆多糖Ⅱ体外对小鼠脾细胞增殖及产生集落刺激因子的影响

用[³H]TdR参入法检测小鼠脾细胞增殖能力及产生集落刺激因子(colony stimulating factor. CSF)含量.证明商陆多糖Ⅱ(PAP-Ⅱ)在31～500 μg·m^-1范围内显著促进小鼠稗细胞增殖。PAP-Ⅱ,31～125 μg·ml^-1可剂量依赖性地促进Con A(1,2.8μg·ml^-1),LPS(3,10,30 μg·ml^-1)诱导的淋巴细胞细咆增殖,随着PAP-Ⅱ剂量加大,对丝裂原诱导的淋巴细胞增殖反呈抑制作用。PAP-Ⅱ。10～500μg·ml^-1呈剂量及时间依赖性地促进脾细胞产生CSF,其最适剂量为100 μg·ml^-1。最佳时间为5 d,提示PAP-Ⅱ能增强免疫及促进造血功能。     

Early life and later determinants of adult disease: a 50 year follow-up study of the Newcastle Thousand Families cohort

The relative contribution of socioeconomic, behavioural and biological factors operating in fetal and infant life, childhood and adulthood to risk for cardiovascular disease, respiratory diseases and non-insulin-dependent diabetes in middle age has become an important research issue. All 1142 babies born in Newcastle upon Tyne in May and June 1947 were recruited into a prospective cohort study of child health (the ‘Thousand Families’ study) and followed in great detail to the age of 15 y, with a brief further follow up at age 22 y. Children from poorer families were at greatest risk of severe respiratory tract infection in infancy. Children from professional and managerial families were on average taller and heavier throughout childhood than those from semi- and unskilled manual social classes. Repeated infections in early childhood greatly increased the risk of developing chronic respiratory disease by age 15 y. This paper outlines a new investigation designed to trace surviving members of this cohort and to chart the relationships between their socioeconomic circumstances, lifestyles, experiences and health from birth through to the present day. Existing data on socioeconomic circumstances and infections in infancy and childhood, infant nutrition, birthweight and physical development to age 22 y will be linked to information gained from a new study. This comprises a postal questionnaire survey of study members' adult health, socioeconomic circumstances and lifestyle, and a hospital based clinical examination including heart and lung function, glucose tolerance, blood lipids and anthropometric measurements at age 49–51 y. Out of a target sample of 979 people for whom sufficient data are available on the first year of life, 866 (88%) have been traced and 649 are still resident in the North of England. Those study members who have been traced are highly representative of the original cohort. The Thousand Families cohort provides a unique opportunity for detailed epidemiological study because of the wealth of data available on infant and childhood socioeconomic and family circumstances, all of which was collected prospectively. In addition, there has been comparatively little loss to follow-up since 1948.