Management of Rh-isoimmunised Pregnancies : Our Experience

Background

The aim of this study was to assess the role of middle cerebral artery peak systolic velocity (MCA-PSV), as measured by doppler ultrasound, in detecting foetal anaemia in Rh- isoimmunised pregnancies. Intra-uterine foetal blood transfusion was performed in such anaemic foetuses to tide over the crisis of foetal immaturity till considered fit for extra-uterine survival.

Methods

Rh-isoimmunised pregnancies reporting to a tertiary institute from 2003 to 2005, were screened by doppler ultrasound to estimate MCA-PSV to detect foetal anaemia. If the foetus developed MCA-PSV of more than 1.5 multiple of median (MoM) for the gestational age, foetal blood sampling through cordocentesis was performed to confirm foetal anaemia, followed by intrauterine foetal blood transfusion to all anaemic foetuses at the same sitting. Neonatal outcome was evaluated by recording gestational age at the time of delivery, duration of gestational time gained and need for blood transfusion in the neonatal period.

Results

A total of thirteen isoimmunised pregnancies were evaluated. Three pregnancies did not require in-utero foetal blood transfusion. Twenty-one intrauterine foetal blood transfusions were performed in the remaining ten patients. Five received blood transfusion in the neonatal period. Intra uterine foetal death occurred in one grossly hydropic foetus and favourable neonatal outcome was recorded in the rest.

Conclusion

The clinical outcome of these pregnancies justifies the use of doppler studies of MCA-PSV in detecting foetal anaemia and intra uterine foetal blood transfusion is the only hope of prolonging pregnancy and salvaging such foetuses.Key Words: Rh-isoimmunisation, Middle cerebral artery peak systolic velocity, Foetal anaemia, Foetal blood transfusion     

Genotype-phenotype correlations to aid in the prognosis of individuals with uncommon 20q13.33 subtelomere deletions: a collaborative study on behalf of the 'association des Cytogénéticiens de langue Française'

The identification of subtelomeric rearrangements as a cause of mental retardation has made a considerable contribution to diagnosing patients with mental retardation. It is remarkable that for certain subtelomeric regions, deletions have hardly ever been reported so far. All the laboratories from the 'Association des Cytogénéticiens de Langue Fran?aise' were surveyed for cases where an abnormality of the subtelomere FISH analysis had been ascertained. Among 1511 cases referred owing to unexplained mental retardation, 115 (7.6%) patients showed a clinically significant subtelomeric abnormality. We report the clinical features and the molecular cytogenetic delineation of isolated de novo deletions on 20q13.33 in two cases. Detailed mapping was performed by micro-array CGH in one patient and confirmed by FISH in the two patients. We compare our data with the only three patients reported in the literature. Both patients shared a deleted region of approximately 1.33 Mb including 40 genes, with a 324 kb difference between the two patients. Haploinsufficiency for CHRNA4 and ARFGAP1 may have contributed towards a severe phenotype. In addition, the data in all patients suggest that haploinsufficiency for SOX18 may not cause the hypotrichosis-lymphedema-telangiectasia syndrome, or causes milder disease. Our study gives important information by defining the size of imbalance and better predicting the phenotype. Two clinically distinct phenotypes may be drawn, a mild mental retardation or a more complex and severe phenotype, according to the presence or absence of the CHRNA4 and ARFGAP1 genes respectively.     

自体骨髓来源内皮祖细胞移植对移植静脉桥血管再内皮化及内膜增生的影响

目的:已有实验研究证明,移植骨髓来源内皮祖细胞可以促进球囊损伤后血管内皮的修复及抑制内膜的增生.那么移植骨髓来源内皮祖细胞对自体静脉移植术后静脉血管内皮修复和内膜增生是否起同样的作用?观察自体骨髓来源内皮祖细胞移植对自体静脉移植术后静脉桥血管再内皮化及内膜增生的作用。方法:实验于2007-01/08在北华大学附属医院内科实验室完成。实验室级别:P2级。①实验材料:6～8月龄雄性新西兰大白兔由解放军第二军医大学实验动物中心提供.体质量(2.5±0.5)kg,清洁级,实验过程中对动物处置符合动物伦理学标准。②实验方法:抽取成年兔骨髓分离制备骨髓单个核细胞,体外扩增法培养出骨髓来源内皮祖细胞.在培养第7天通过Ac-Dil-LDL、FITC-BS-1双染色及流式细胞仪检测CD34、CD133、FIK-1表达进行细胞鉴定,应用DAPI荧光染料体外标记培养7 d的骨髓来源内皮祖细胞、将成年新西兰大白兔23只随机分为细胞移植组(n=13)和对照组(n=10)进行自体左颈外静脉、颈总动脉移植术,在建模后第3天将DAPI标记的骨髓来源内皮祖细胞悬液经耳缘静脉植入细胞移植组动物体内,埘照组植入等量的PBS液③实验评估:细胞移植后4周.观察兔静脉移植段血管性及内膜厚度。结果:23只免均进入结果分析,无脱落:①通过体外扩增法培养的骨髓来源内皮组细胞,培养至7 d可见AC-Dil-LDI及FITC-BS-1双染色阳性细胞并表达CD34、CD133及FIK-1。②在呈绿荧光染色的血管内皮中可见有部分不均匀的散发蓝色荧光(DAPI标记细胞的细胞核)。③细胞移植组兔静脉血管内皮有DAPI标记的细胞.且内膜厚度明显低于对照组(P<0.05)结论:自体骨髓来源的内皮祖细胞移植可以促进损伤部位血管内皮的修复.并抑制内膜的过度增生。     

Structuring a safer donor-replacement program

BACKGROUND: Replacement donors are more likely than volunteer donors to have positive or abnormal tests for transfusion-transmissible disease. In an effort to increase the donor pool, workers sought to identify a safer replacement-donor subgroup that may be acceptable for routine donations. STUDY DESIGN AND METHODS: In a retrospective review and cohort study, the replacement-donor effect was separated from the new- donor effect. The relative effect the replacement donor has on the risk of transfusion-transmissible diseases, donor retention, and frequency of returning donations was then quantified by comparison against the effect of repeat volunteer donors. RESULTS: The replacement donor had 3.1 times the risk and 0.72 times the donor retention rate and made 0.81 times as many returning donations as the repeat volunteer donor. The figures for the new-donor effect were similar. The two risks were additive, making a new replacement donor particularly hazardous. If replacement donations only from repeat replacement donors were considered, the donor risk and the number of donations per returning donor were made comparable to those for the general (combined) volunteer donor. CONCLUSION: The negative effect of the replacement donor is similar in magnitude to that of the new volunteer donor. A replacement-donation program targeting repeat replacement donors has an acceptable risk profile and may be a valuable adjunct to the collection of blood from general volunteer donors.     

Hypoceruloplasminemia and ultrastructural changes resembling Wilson's disease in non-alcoholic liver steatosis

ABSTRACT— We report five cases of asymptomatic patients with persistently abnormal liver function tests in whom copper metabolism abnormalities resulted in a misleading suspicion of Wilson's disease. Ceruloplasmin levels assessed by nephelometric immunoassay, single radial immunodiffusion and the enzymatic method averaged 53.8, 61 and 52.8% of the mean value obtained in age- and sex-matched controls (p<0.001). Twenty-four-hour urinary copper excretion was higher than the normal range in three instances. Four patients exhibited hypertriglyceridemia. Liver histology showed fatty change with or without sinusoidal fibrosis. Electron microscopic examination unexpectedly disclosed mitochondrial and lysosomal changes identical to those described in Wilson's disease. The present observations indicate that biochemical and ultrastructural changes suggestive for Wilson's disease may be observed in the absence of increased liver copper content. Whether such cases represent isolated cases of heterozygosity for the Wilson's disease gene remains to be elucidated.     

The Collagen Binding Protein Cnm Contributes to Oral Colonization and Cariogenicity of Streptococcus mutans OMZ175

Streptococcus mutans is the etiological agent of dental caries and one of the many bacterial species implicated in infective endocarditis. The expression of the collagen-binding protein Cnm by S. mutans has been associated with extraoral infections, but its relevance for dental caries has only been theorized to date. Due to the collagenous composition of dentinal and root tissues, we hypothesized that Cnm may facilitate the colonization of these surfaces, thereby enhancing the pathogenic potential of S. mutans in advancing carious lesions. As shown for extraoral endothelial cell lines, Cnm mediates the invasion of oral keratinocytes and fibroblasts by S. mutans. In this study, we show that in the Cnm⁺ native strain, OMZ175, Cnm mediates stringent adhesion to dentinal and root tissues as well as collagen-coated surfaces and promotes both cariogenicity and carriage in vivo. In vitro, ex vivo, and in vivo experiments revealed that while Cnm is not universally required for S. mutans cariogenicity, it contributes to (i) the invasion of the oral epithelium, (ii) enhanced binding on collagenous surfaces, (iii) implantation of oral biofilms, and (IV) the severity of caries due to a native Cnm⁺ isolate. Taken together, our findings reveal that Cnm is a colonization factor that contributes to the pathogenicity of certain S. mutans strains in their native habitat, the oral cavity.     

Acute esophageal necrosis and low-flow state.

Acute esophageal necrosis (AEN), also called black esophagus, is quite exceptional. Endoscopic findings show circumferential black discolouration of the esophagus with or without exudates. The etiology of AEN is presently unknown and is assumed to be multifactorial. Distal esophageal involvement with proximal extension ending sharply at the gastroesophageal junction is the most common presentation. The present case report describes the clinical and endoscopic evolution of black esophagus observed in a patient with significant peripheral vascular disease, who was presented to the intensive care unit at the Hopital Saint-Francois d'Assise (Quebec City, Quebec). Through an extensive review of the literature, common underlying clinical conditions of patients diagnosed with AEN have been identified.     

The metabolite 1 alpha,25-dihydroxyvitamin D3 enhances lymphokine- mediated activation of the oxidative metabolism of the U937 cell line and phosphorylation of a 48-kD respiratory burst-associated protein

U937 cells respond to a variety of stimuli with increased differentiation as manifested by reduced growth, increased adherence, increased expression of several surface receptors, and increased capacity for phagocytosis and formation of reactive oxygen intermediates. In the present study the effects of lymphocyte conditioned media, recombinant interferon-gamma (IFN-gamma), and 1 alpha,25-dihydroxyvitamin D3 (1,25(OH)2D3) on the ability to form reactive oxygen intermediates by U937 cells were measured by using the luminol-dependent luminescence (LDL) assay. Neither 1,25(OH)2D3 alone nor IFN-gamma alone enhanced competence for phorbol myristate acetate- stimulated LDL. Cells were capable of moderate LDL after exposure to lymphocyte conditioned media, and this was enhanced by 1,25(OH)2D3 (10(- 8) mol/L) and other vitamin D metabolites at higher concentrations. This effect was not secondary to accelerated production of myeloperoxidase, which is important in the LDL assay. Enhanced phorbol myristate acetate-stimulated phosphorylation of a 48-kd substrate was observed in 32P-labeled intact cells treated with 1,25(OH)2D3 alone or in combination with IFN-gamma. Treatment of cells with IFN-gamma or lymphocyte conditioned media did not alter phosphorylation. These results support the concept that 1,25(OH)2D3 plays a role in phagocyte differentiation and activation beyond the effects of lymphokines. Protein kinase C-mediated phosphorylation reactions may be necessary for the ability of U937 cells to reduce O2 and required for maximal activity under some conditions of incubation.