膀胱副神经节瘤:单中心7例报告

目的归纳膀胱副神经节瘤的临床一般特征、诊断及治疗。方法收集兰州大学第二医院2013年3月—2020年8月所有膀胱肿瘤患者术后病理结果,对术后病理证实为副神经节瘤的7例患者的临床资料及随访结果进行回顾性分析。结果7例中术前明确诊断2例。7例均成功完成手术治疗。经尿道膀胱肿瘤电切术4例[2例术程平稳;2例术中出现血压剧烈波动,经过积极降压1例最终完成手术、另1例取活检后终止手术(院外积极酚苄明准备,3月后再次入院行腹腔镜下膀胱部分切除术)];1例行经尿道膀胱肿瘤激光剜除术,术程平稳;腹腔镜下膀胱部分切除3例(包括上述因术中血压无法有效控制中止手术者1例),平均手术时间130(30~180)min,平均术中出血30(20~50)mL。术后无相关并发症发生,术后住院3~5 d后恢复良好出院。1例发现肿瘤远处转移建议转外院继续治疗。7例均获随访,全身转移1例于外院治疗效果不佳,其余6例症状均有所改善,影像学检查未发现复发征象。远期结果仍有待进一步随访。结论膀胱副神经节瘤术前明确诊断困难,往往误诊为膀胱恶性肿瘤。手术为一线治疗方式,腹腔镜下膀胱部分切除术体现出微创优势,术后恢复快,部分选择性病例采用经尿道膀胱肿瘤电切术,同样可取得良好临床结果。     

Programmed Cell Death-One Inhibition Therapy in Classical Hodgkin Lymphoma

The majority of patients with classical Hodgkin lymphoma (cHL) may be cured, but for patients with relapsed or refractory (R/R) cHL, the prognosis is unfavorable. Immune dysfunction is a significant contributor of relapse and a hallmark of cHL; in particular, the immune system is unable to eradicate lymphoma cells that overexpress immune checkpoint proteins. The blocking of this mechanism used by lymphoma cells to evade the immune system has resulted in clinical benefits. Use of checkpoint inhibitors (CPIs) in R/R cHL is associated with high response rates and an acceptable adverse effects profile. There is growing interest in combining chemotherapy with CPIs in frontline therapy of cHL treatment to improve relapse rates without significant additive toxicity. In this review, we discuss the current evidence supporting CPI use in R/R cHL and maintenance therapy. We present emerging CPI data in frontline adult cHL and assess its role in the elderly. In addition, we discuss critical immune-related toxicities and their management, and elaborate on the challenges of monitoring response and minimal residual disease as tools for maximizing efficacy by limiting toxicity.     

Typhoid fever complicated by multiple organ involvement: report of two cases

Typhoid fever complicated by multiple organ involvement has been rarely mentioned in the literature. We reported two cases of typhoid fever with several unusual manifestations, including acute renal failure, acute hepatitis, acute pancreatitis, disseminated intravascular coagulation, and lower gastrointestinal bleeding. A renal biopsy in the first case showed no pathological change. Bone marrow biopsy showed focal necrosis of matrix, which might have been due to severe illness. A liver biopsy in the second case showed a predominantly histiocytic proliferation with occasional neutrophilic infiltration in the portal areas and hepatic sinusoids. Focal necrosis, bile duct injury, and multiple eosinophilic bodies were also noted. After appropriate antimicrobial therapy, both patients recovered without any sequelae. The potential of multiple organ involvement is highlighted in typhoid fever, which, on rare occasions, may occur simultaneously in the same patient.     

Mitochondrial and Nuclear Genes Suggest that Stony Corals Are Monophyletic but Most Families of Stony Corals Are Not (Order Scleractinia, Class Anthozoa, Phylum Cnidaria)

Modern hard corals (Class Hexacorallia; Order Scleractinia) are widely studied because of their fundamental role in reef building and their superb fossil record extending back to the Triassic. Nevertheless, interpretations of their evolutionary relationships have been in flux for over a decade. Recent analyses undermine the legitimacy of traditional suborders, families and genera, and suggest that a non-skeletal sister clade (Order Corallimorpharia) might be imbedded within the stony corals. However, these studies either sampled a relatively limited array of taxa or assembled trees from heterogeneous data sets. Here we provide a more comprehensive analysis of Scleractinia (127 species, 75 genera, 17 families) and various outgroups, based on two mitochondrial genes (cytochrome oxidase I, cytochrome b), with analyses of nuclear genes (ß-tubulin, ribosomal DNA) of a subset of taxa to test unexpected relationships. Eleven of 16 families were found to be polyphyletic. Strikingly, over one third of all families as conventionally defined contain representatives from the highly divergent “robust” and “complex” clades. However, the recent suggestion that corallimorpharians are true corals that have lost their skeletons was not upheld. Relationships were supported not only by mitochondrial and nuclear genes, but also often by morphological characters which had been ignored or never noted previously. The concordance of molecular characters and more carefully examined morphological characters suggests a future of greater taxonomic stability, as well as the potential to trace the evolutionary history of this ecologically important group using fossils.     

Maternal return to work and breastfeeding: A population-based cohort study

Background

In recent decades there has been a marked rise in the participation of women with infants in the labour market, while there has been a decline in the prevalence rate of breastfeeding.

Objective

To explore the relationship between maternal return to work and breastfeeding.

Design

An on-going prospective longitudinal study.

Setting and participants

Multistage stratified systematic sampling was designed to recruit 24,200 pairs, postpartum women and newborns, from the Taiwan national birth register in 2005. Participating women underwent two home interviews at 6 and 18 months after giving birth, following structured questionnaires. A total of 21,248 and 20,172 women were interviewed, and the completed interview rate was thus 87.8% and 83.4% at 6 and 18 months, respectively. All study participants provided informed consent as approved by the Ethics Review Board of the National Taiwan College of Public Health.

Results

The overall prevalence of initial breastfeeding was 83.7%. Postpartum women returning to work less than or equal to 1 month had the lowest initiation of breastfeeding rate (77.5%), but had a higher prevalence of breastfeeding duration less than or equal to 1 month (34.9%) than the overall population (26.8%). Overall 67.9%, 39.4%, 25.4%, and 12.7% mothers who started breastfeeding still breastfed their infants at the age of 1, 3, 6 and 12 months, respectively. Women with maternal leave of less than or equal to 6 months ceased breastfeeding earlier than those with maternal leave beyond 6 months and those who did not return to work up to 18 months after birth. After adjustment for potential confounders, odds ratios of initial breastfeeding seemed no different, except those for postpartum women who returned to work less than or equal to 1 month and those who did not return to work. Mothers returning to work within 1 year after giving birth were significantly earlier in weaning than those without return to work.

Conclusion

In our study, an early maternal return to work, especial within 6 months after giving birth, was a barrier to the initiation and continuation of breastfeeding. Thus, a comprehensive strategy is required to encourage the practice of breastfeeding in working women from pregnancy to the return to work, and nurses should work to promote breastfeeding in the different occasion.     

Molecular and biochemical basis of intermediate maple syrup urine disease. Occurrence of homozygous G245R and F364C mutations at the E1 alpha locus of Hispanic-Mexican patients.

Maple syrup urine disease (MSUD) is caused by a deficiency of the mitochondrial branched-chain alpha-keta acid dehydrogenase (BCKAD) complex. The multienzyme complex comprises five enzyme components, including the E1 decarboxylase with a heterotetrameric (alpha 2 beta 2) structure. Four unrelated Hispanic-Mexican MSUD patients with the intermediate clinical phenotype were diagnosed 7 to 22 mo after birth during evaluation for developmental delay. Three of the four patients were found homozygous for G to A transition at base 895 (exon 7) of the E1 alpha locus, which changes Gly-245 to Arg (G245R) in that subunit. The remaining patient was homozygous for T to G transversion at base 1,253 in the E1 alpha gene, which converts Phe-364 to Cys (F364C) in the gene product. Transfection studies in E1 alpha-deficient lymphoblasts indicate that both G245R and F364C mutant E1 alpha subunits were unable to significantly reconstitute BCKAD activity. Western blotting showed that both mutant E1 alpha subunits in transfected cells failed to efficiently rescue the normal E1 beta through assembly. The putative assembly defect was confirmed by pulse-chase labeling of E1 subunits in a chaperone-augmented bacterial overexpression system. The kinetics of initial assembly of the G245R E1 alpha subunit with the normal E1 beta was shown to be slower than the normal E1 alpha. No detectable assembly of the F364C E1 alpha with normal E1 beta was observed during the 2 h chase. Small amounts of recombinant mutant E1 proteins were produced after 15 h induction with isopropyl thiogalactoside and exhibited very low or no E1 activity. Our study establishes that G245R and F364C mutations in the E1 alpha subunit disrupt both the E1 heterotetrameric assembly and function of the BCKAD complex. Moreover, the results suggest that the G245R mutant E1 alpha allele may be important in the Hispanic-Mexican population.