Early developmental outcome after the Norwood procedure for hypoplastic left heart syndrome

OBJECTIVE: To assess intellect and adaptive behavior in children with hypoplastic left heart syndrome (HLHS) who had undergone at least two surgical stages of the Norwood procedure. METHODS: Fourteen children with HLHS >3 years of age participated in the study. The patients underwent intelligence quotient (IQ) testing, and their parents were interviewed regarding their children's adaptive behavior. Results were compared with those of 10 family controls. Outcomes were studied for possible correlation with perioperative variables. RESULTS: Among the HLHS patients, the median scores for full scale IQ and adaptive behavior were 88 and 91, respectively (normal = 100 +/- 15). One child met criteria for mental retardation. Family controls scored generally higher than did HLHS patients, but only differences in adaptive behavior were statistically significant. A negative correlation was found between stage I circulatory arrest time and full scale IQ. CONCLUSIONS: Children with HLHS most often function in the low-normal range of intelligence and adaptive behavior. A prolonged circulatory arrest time may result in decreased intellectual function.     

白蛋白置换液替代血浆置换治疗难治性肝炎高胆红素血症

我们曾应用血浆置换治疗难治性病毒性肝炎高胆红素血症，取得了一定的疗效。但血浆作为置换液可能会引起丙型或乙型等肝炎病毒的感染。自１９９６年２月至１９９８年３月，我们探索以白蛋白替代血浆置换治疗２９例难治性病毒性肝炎高胆红素血症的病人，现将结果报告如下。...     

11Effect of an immune stress on prolactin secretion in early pregnancy

Problem:  Stress in early pregnancy has been linked to increased abortion rates. Immune stress in early pregnancy inhibits progesterone secretion in mice. As prolactin both mediates implantation and drives progesterone secretion, we hypothesised that stress would decrease prolactin secretion in early pregnancy.
Material and Methods:  Lipopolysaccharide (LPS; 12.5μg intraperitoneally) as an immune stress or vehicle were administered to day 5.5 early pregnant and virgin c57/Bl6J mice. They were killed by decapitation 60, 120 or 240 min later and trunk blood was collected and analysed for prolactin concentration (ELISA); corticosterone was also analysed (RIA).
Results:  LPS significantly decreased prolactin concentration in early pregnancy (P<0.001, 2-way ANOVA); however, there was no significant difference in the virgin groups. In contrast, LPS significantly elevated corticosterone concentration in all groups (P<0.001, 2-way ANOVA), confirming activation of the hypothalamo-pituitary-adrenal stress axis.
Conclusions:  Stress decreased prolactin secretion during early pregnancy, and might explain stress-disrupted implantation and progesterone secretion.     

Unusual deletions within the immunoglobulin heavy-chain locus in acute leukemias

We have investigated the structure of the Ig heavy (IGH) chain locus in 309 cases of acute leukemia. Seventy-one cases of B-cell precursor (BCP) acute lymphoblastic leukemia (ALL) were analyzed: in six cases deletion of joining (JH) segments in the presence of cytogenetically normal chromosome 14 was observed. Similar deletions were seen in 1 out of 8 cases of biphenotypic acute leukemia analyzed: this case exhibited t(9:22)(q34;q11) and coexpressed both myeloid and B cell differentiation antigens. Five of the 7 cases analyzed had deleted the JH segments from both chromosomes. Because these deletions may have contributed to the pathogenesis of the disease we have attempted to define their boundaries. Using probes that map both 5' and 3' of JH, the 3' (centromeric) boundary of the deletions was mapped to an approximately 30-kb central region of the 60 kb between C delta and C gamma 3 in 10 of the 12 deleted chromosomes. In the remaining two chromosomes, the 3' boundary mapped to S mu. The 5' (telomeric) boundary could not be defined. However, three cases with biallelic deletion of JH showed biallelic deletion of the most proximal variable (VH) (VH6 and VH5-B2) genes, indicating that the deletions spanned over 500 kb. VH5-B1 and VH5-B3 were retained in germline configuration and no gross deletions were observed using a VH3 subgroup-specific probe, indicating that the 5' boundary mapped within the VH locus. Unusual deletions of the portion of the IgH locus including JH segments and the C mu and C delta genes may occur in acute leukemias with immunophenotypic evidence of commitment to the B cell differentiation pathway. The possible consequences of the deletions remain to be determined. However, the clustering of the centromeric boundary of the deletions to S mu and to a region between the C delta-C gamma 3 genes, a known "hot spot" for recombination, may indicate the operation of a distinct pathogenic mechanism.