Lymphoid blast crises of chronic myelogenous leukemia represent stages in the development of B-cell precursors

The origin and stage of differentiation of the blast-crisis cells in chronic myelogenous leukemia have remained uncertain. Because immunoglobulin heavy-chain and light-chain genes must undergo a DNA rearrangement during B-cell development but rarely do so in human non-B-cell lineages, we examined these genes in 18 episodes of chronic myelogenous leukemia. In eight of nine episodes of lymphoid blast crisis, heavy-chain genes were rearranged, and in three, rearrangements in light-chain genes were also present. In contrast, cells from chronic myeloid, myeloid blast, and erythroid-like phases retained germ-like immunoglobulin genes. The observed phenotypic markers and gene configurations revealed that most lymphoid blast crises represent stages of development of B-cell precursors. In two separate episodes of lymphoid crisis, cells from a single patient possessed identical heavy-chain but different light-chain-gene configurations. Thus, the precursor cells that monoclonally expand to produce a lymphoid crisis are capable of immunoglobulin-gene rearrangements and represent discrete steps in early B-cell maturation.     

Weight-for-age and weight-for-height references for Hong Kong children from birth to 18 years

Objective : Childhood obesity is an emerging problem in Asia. Sequential monitoring of the growth of an individual can detect a change in body fatness, provided there are ethnically appropriate growth references.
Methodology : A territory wide cross-sectional growth survey of Hong Kong on 24 709 individuals from the age of newborn to 18 years was performed in 1993. Weight-for-age and weight-for-height percentile charts were prepared separately for boys and girls.
Results : There was an average increase of 8.5 kg and 5.1 kg in the 18 year old boys and girls, respectively, compared to those surveyed 30 years ago. The percentile curves between 6 and 18 years were similar to those of Singapore. Weight-for-height percentile curves were close to those of America in the prepubertal years.
Conclusions : These Hong Kong growth standards for weight-for-age and weight-for-height are important tools for the assessment of nutritional status of an individual and for monitoring changes in nutritional status of the population.     

Cell-lineage antigens of the stem cell-megakaryocyte-platelet lineage are associated with the platelet IIb-IIIa glycoprotein complex

The stem cell-platelet lineage is uniquely defined by platelet cell- lineage antigens. These antigens are present on all stem cells measured by the spleen colony assay and become restricted to the platelet cell lineage as differentiation proceeds. In this study, anti-platelet serum (APS) has been used to identify cells in the bone marrow that express platelet cell-lineage antigens and to identify platelet cell surface molecules expressing these antigens. Anti-platelet IgG extensively absorbed with brain, thymus, and peritoneal cells bound selectively to stem cells, megakaryocyte progenitor cells (Mk-CFC), and megakaryocytes in CBA mouse bone marrow and to blood platelets. No other hemopoietic cell type, tissue, cell line, or tumor cell bound significant amounts of antibody against platelet cell-lineage antigens as determined by ability to absorb the anti-stem cell activity in APS. Studies with lactoperoxidase-labeled platelets showed that two major iodinated proteins of Mr = 114,000 and 138,000 were immunoprecipitated with APS and with antiserum that had been extensively absorbed. These proteins correspond to the platelet IIb-IIIa glycoprotein complex, which is known to express receptors for collagen and fibrinogen, molecules known to influence hemopoietic cell proliferation and tumor cell growth. A panel of six monoclonal antibodies against human IIb-IIIa inhibited spleen colony formation by 17% to 100%, J15 and A5.15 also being cytotoxic for granulocyte-macrophage progenitor cells and Mk-CFC. Other platelet monoclonal antibodies did not inhibit spleen colony formation. Although APS inhibited fibrinogen binding to platelets and platelet aggregation, these activities were greatly reduced with absorbed antiserum. Furthermore, fibrinogen treatment of bone marrow did not block the anti-stem cell activity in APS. Thus the evidence is consistent with expression of platelet cell-lineage antigens on the platelet IIb-IIIa glycoprotein complex at a site removed from the fibrinogen binding site.     

Chronic illness perception in adolescence: Implications for the doctor-patient relationship

To describe the inter-relationship of chronic illness severity as perceived by adolescents with both psychosocial well-being and objective measures of illness severity. Additionally to compare the adolescents' perception of illness severity with how their physicians believe that the adolescents perceive their illness severity.

Methods:

The psychological well-being of 48 adolescents with either cystic fibrosis (CF) or insulin-dependent diabetes mellitus (IDDM) was measured by four standardized questionnaires. The adolescents' perception of severity of illness was measured using an original instrument (PSCI), and this measure was compared to their physicians' estimates of how the adolescents perceived the severity of their illness and clinical illness.

Results:

There were 24 patients in both the CF and IDDM groups. Both groups were found to function well psychosocially; although, there were more patients with low self image compared to normative values. Depression and low self image were associated with a greater adolescent perception of illness severity. For both chronic illness groups, physicians' assessment of assumed adolescent perception of disease severity correlated with clinical indices of disease severity and was higher than the perception of illness severity reported by the adolescents. For adolescents with CF, but not with IDDM, perception of severity of chronic illness correlated with clinical indices.

Conclusions:

For adolescents with chronic illness, their perception of illness severity is an important indicator of psychosocial well-being. Physicians do not accurately infer their patients' perception of illness severity.     

Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders

Mutations of the renal-specific chloride channel (CLCN5) gene, which is located on chromosome Xp11.22, are associated with hypercalciuric nephrolithiasis (kidney stones) in the Northern European and Japanese populations. CLCN5 encodes a 746 amino acid channel (CLC-5) that has approximately 12 transmembrane domains, and heterologous expression of wild-type CLC-5 in Xenopus oocytes has yielded outwardly rectifying chloride currents that were markedly reduced or abolished by these mutations. In order to assess further the structural and functional relationships of this recently cloned chloride channel, additional CLCN5 mutations have been identified in five unrelated families with this disorder. Three of these mutations were missense (G57V, G512R and E527D), one was a nonsense (R648Stop) and one was an insertion (30:H insertion). In addition, two of the mutations (30:H insertion and E527D) were demonstrated to be de novo, and the G57V and E527D mutations were identified in families of Afro-American and Indian origin, respectively. The G57V and 30:H insertion mutations represent the first CLCN5 mutations to be identified in the N-terminus region, and the R648Stop mutation, which has been observed previously in an unrelated family, suggests that this codon may be particularly prone to mutations. Heterologous expression of the mutations resulted in a marked reduction or abolition of the chloride currents, thereby establishing their functional importance. These results help to elucidate further the structure-function relationships of this renal chloride channel.      

多囊卵巢综合征代谢异常分析

1 临床资料 2000-10/2005-3我院妇科内分泌门诊的多囊卵巢综合征(PCOS)患者25例,根据体质量指数(BMI)[1]﹥27(肥胖)及﹤24(正常)分为肥胖PCOS组和非肥胖PCOS组.肥胖PCOS组:BMI 27.1±0.8,17例,年龄(24.2±2.9)岁;非肥胖PCOS组:BMI 20.0±0.6,13例,年龄(23.7±3.20)岁.     

Poliomyelitis-like illness after acute asthma

Two children had pain in one arm, followed by weakness and wasting, after an acute exacerbation of bronchial asthma. Similar cases in which a poliomyelitis-like illness was associated with acute asthma have been reported, but the relationship between the two remains conjectural.