Evidence from proton magnetic resonance spectroscopy for a metabolic cascade of neuronal damage in shaken baby syndrome

OBJECTIVE: The purpose of this study was to use proton magnetic resonance spectroscopy (MRS) as a metabolic assay to describe biochemical changes during the evolution of neuronal injury in infants after shaken baby syndrome (SBS), that explain the disparity between apparent physical injury and the neurological deficit after SBS. METHODOLOGY: Three infants [6 months (A), 5 weeks (B), 7 months (C)] with SBS were examined repeatedly using localized quantitative proton MRS. Examinations were performed on days 7 and 13 (A), on days 1, 3, 5, and 12 (B), and on days 7 and 19 (C) posttrauma. Long-term follow-up examinations were performed 5 months posttrauma (A) and 4.6 months posttrauma (B). Data were compared to control data from 52 neurologically normal infants presented in a previous study. RESULTS: Spectra from parietal white matter obtained at approximately the same time after injury (5 to 7 days) showed markedly different patterns of abnormality. Infant A shows near normal levels of the neuronal marker N-acetyl aspartate, creatine, and phosphocreatine, although infant C shows absent N-acetyl aspartate, almost absent creatine and phosphocreatine, and a great excess of lactate/lipid and lipid. Analysis of the time course in infant B appears to connect these variations as markers of the severity of head injury suffered in the abuse, indicating a progression of biochemical abnormality. The principal cerebral metabolites detected by MRS that remain normal up to 24 hours fall precipitately to approximately 40% of normal within 5 to 12 days, with lactate/lipid and lipid levels more than doubling concentration between days 5 and 12. CONCLUSIONS: A strong impression is gained of MRS as a prognostic marker because infant A recovered although infants B and C remained in a state consistent with compromised neurological capacity. Loss of integrity of the proton MR spectrum appears to signal irreversible neurological damage and occurs at a time when clinical and neurological status gives no indication of long-term outcome. These results suggest the value of sequential MRS in the management of SBS.     

Retrospective study of children with hypophosphatasia with reference to dental changes

Abstract— In the present retrospective study different dental and medical parameters have been analyzed in 17 Swedish children with established hypophosphatasia (HP). It was demonstrated that the basis for the establishment of the diagnosis varied among different dentists and physicians, and that the diagnostic parameters studied among the children varied. The most reliable parameters for HP included raised levels of phosphoethanolamine in urine, and clinical and radiologic findings associated with the legs. These findings were found among the children more often than lowered values of alkaline phosphatase in serum. Histologic analysis of an extracted tooth made a valuable diagnostic complement. It is concluded that a better diagnostic uniformity is recommended. In a well functioning collaboration with well defined tasks, both dentists and physicians can contribute to a reliable diagnosis.     

Improvement in transfusion safety using a new blood unit and patient identification system as part of safe transfusion practice

A new patient and blood unit identification system designed to confirm the identity of crossmatched blood products and that of the intended recipient was evaluated. Six hundred seventy-two red cell concentrates were transfused to 312 patients. Participating hospital personnel and patients were interviewed regarding the use and benefit of this unique system, which incorporates a "lock-box" approach to the identification process. The product and procedure were accepted unanimously and enthusiastically, and three potential mistransfusions were avoided by use of the system during the limited period of observation. This type of approach to the identification process affords greater security than conventional practices and minimally burdens staff.     

Young age at first pregnancy does protect against early onset breast cancer in <Emphasis Type="Italic">BRCA1</Emphasis> and <Emphasis Type="Italic">BRCA2</Emphasis> mutation carriers

Purpose

Previous research assessing the impact of pregnancy and age at first pregnancy on breast cancer risk in BRCA1 and BRCA2 mutation carriers has produced conflicting results, with some studies showing an increased risk following early first pregnancy in contrast to the reduced risk in the general population of women. The present study addresses these inconsistencies.

Methods

Female BRCA1 and BRCA2 carriers from North West England were assessed for breast cancer incidence prior to 50 years of age comparing those with an early first full-term pregnancy (< 21 years) to those without a full-term pregnancy. Breast cancer incidence per decade from 20 years and Kaplan–Meier analyses were performed.

Results

2424 female mutation carriers (1278 BRCA1; 1146 BRCA2) developed 990 breast cancers under the age of 50 years. Women who had their first term pregnancy prior to age 21 (n = 441) had a lower cancer incidence especially between age 30–39 years. Kaplan–Meier analysis showed an odds ratio of 0.78 for BRCA1 (p = 0.005) and 0.73 for BRCA2 (p = 0.002).

Conclusions

The present study demonstrates a clear protective effect of early first pregnancy on breast cancer risk in both BRCA1 and BRCA2 mutation carriers.

     

Invasive Haemophilus influenzae type b disease in the Oxford region (1985-91)

For a seven year period (1985-91) clinical and epidemiological data were prospectively collected on children aged < 10 years with microbiologically confirmed invasive Haemophilus influenzae type b infection in the Oxford region to study the epidemiology of the disease and determine the potential impact of early primary immunisation in infants. Computer records of primary immunisations given to these cases were retrospectively analysed and, where necessary, hospital and general practitioner records were searched to determine the immunisation history. Over the seven year period, 416 cases of invasive H influenzae type b disease were reported. Widescale immunisation against H influenzae type b began in 1991 as part of a regional trial. The estimated annual incidence for invasive disease between 1985 and 1990 was 35.5 cases per 100,000 children aged less than 5 years; for H influenzae type b meningitis it was 25.1 per 100,000 children aged less than 5 years. The cumulative risks for invasive disease and meningitis by the fifth birthday were one in 560 and one in 800 respectively. The majority of disease (71%) occurred in children less than 2 years of age with the peak monthly incidences at 6 and 7 months of age. The overall mortality was 4.3% and 50% of these deaths occurred suddenly. Most (91%) of the children had received at least one primary immunisation against diphtheria, tetanus, and pertussis before H influenzae type b infection and there was only one case of parental refusal of immunisation. None had received H influenzae type b immunisation. Given a vaccine uptake of 90% by 5 months of age it is estimated that at least 82% of the H influenzae type b infections could have been prevented. Extrapolated nationally, 1150 cases of infection and 50 deaths could be prevented each year by routine primary immunisation.     

Atypical distribution of Pneumocystis carinii infiltrates during radiation therapy

Bilateral peripheral pulmonary infiltrates caused by Pneumocystis carinii developed in a patient undergoing mediastinal irradiation after chemotherapy for Hodgkin disease. The paramediastinal part of the lung included within the treatment port remained clear during the 2 1/2 weeks of radiation therapy. The distribution of the pneumocystis infiltrates was altered by the radiation, producing a pattern that is the "radiographic negative" of typical post-radiation therapy paramediastinal fibrosis.     

B超引导穿刺的微通道经皮肾镜碎石术治疗肾盏结石

目的 评价B超引导穿刺的微通道( F12F16)经皮肾镜碎石术治疗肾盏结石的安全性和有效性.方法 2009年5月2011年8月共收治ESWL无效及透X线肾盏结石44例(44侧肾结石),其中下盏结石24侧,中盏结石11侧,分支性肾盂上盏合并下盏结石9侧,结石长径(16.14±5.12)mm.采用B超引导穿刺的微通道(F12～F16)经皮肾镜碎石术,气压弹道击碎并清除结石.结果 44例均一期建立通道并碎石,其中单通道36侧肾,双通道9侧肾;经上盏建立通道9个,经中盏11个,经下盏33个.手术时间(49.3±10.9)min,一期手术结石清除率97.73％ (43/44).术后血红蛋白含量较术前下降(9.13±3.7)g/L.围手术期无输血,与手术相关的感染发生率为6.18％ (3/44),主要表现为术后发热,无气胸或腹腔脏器损伤.44例随访6～20个月,平均11.4个月,1例残余结石排出,术后6个月结石复发率为2.27％(1/44),1年结石复发率为6.18％ (3/44).结论 B超引导穿刺的微通道经皮肾镜碎石术治疗肾盏结石有效、安全,是此类结石的首选治疗方法.     

HLA配型与肾移植术后早期急性排斥反应的关系

目的 研究ＨＬＡ配型与尸体肾移植术后早期急性排斥反应的关系。方法 将２６２例尸体肾移植受者按ＨＬＡ配型的误配率（ＭＭ）进行分组,统计各组术后１～２个月内急性排斥反应的发生次数。结果 当ＭＭ〉３时,若接受的器官ＨＬＡ抗原／基因为可接受性,术后急性排斥反应的发生率为１６．４％;若供体器官ＨＬＡ抗原／基因具免疫原性,则急性排斥反应的发生率为３３．１％,两者比较,Ｐ〈０．０５。结论 供受者间ＨＬＡ配型越好     

胰岛细胞及肾联合移植治疗胰岛素依赖型糖尿病合并尿毒症四例

目的　观察成人胰岛细胞及肾联合移植治疗胰岛素依赖型糖尿病合并尿毒症的临床效果。方法　对 4例胰岛素依赖型糖尿病合并尿毒症的患者施行成人胰岛细胞及肾联合移植。肾移植为常规术式 ,在移植肾开放血液循环后 ,将分离、纯化、培养好的胰岛细胞注入门静脉中。移植后监测患者的空腹血糖、基础C 酞、糖化血红蛋白及肾功能的变化 ,并与其术前各项指标比较。结果　 4例患者术后在观察期间内胰岛素的用量均较术前减少超过 2 5 % ,且持续 3个月以上 ;空腹血糖基本保持在正常水平 ,基础C 酞也基本维持在正常水平 ,糖化血红蛋白于术后降至正常水平 ;4例患者的移植肾功能良好 ,3例患者已恢复正常工作。结论　成人尸体胰岛细胞及肾联合移植治疗胰岛素依赖型糖尿病合并尿毒症的临床效果较好 ,具有操作简单、安全等优点