Developmental outcomes of children with congenital diaphragmatic hernia: A multicenter prospective study

Purpose

To determine developmental outcomes and associated factors in patients with congenital diaphragmatic hernia (CDH) at 2 years of age.

Methods

This is a multicenter prospective study of a CDH birth cohort. Clinical and socioeconomic data were collected. Bayley Scales of Infant Development (BSID-III) and Vineland Adaptive Behavior Scales (VABS-II) were performed at 2 years of age.

Results

BSID-III and VABS-II assessments were completed on 48 and 49 children, respectively. The BSID-III mean cognitive, language, and motor scores were significantly below the norm mean with average scores of 93 ± 15, 95 ± 16, and 95 ± 11. Ten percent (5/47) scored more than 2 standard deviations below the norm on one or more domains. VABS-II scores were similar to BSID-III scores with mean communication, daily living skills, social, motor, adaptive behavior scores of 97 ± 14, 94 ± 16, 93 ± 13, 97 ± 10, and 94 ± 14. For the BSID-III, supplemental oxygen at 28 days, a prenatal diagnosis, need for extracorporeal membrane oxygenation (ECMO) and exclusive tube feeds at time of discharge were associated with lower scores. At 2 years of age, history of hospital readmission and need for tube feeds were associated with lower scores. Lower socioeconomic status correlated with lower developmental scores when adjusted for significant health factors.

Conclusion

CDH patients on average have lower developmental scores at 2 years of age compared to the norm. A need for ECMO, oxygen at 28 days of life, ongoing health issues and lower socioeconomic status are factors associated with developmental delays.     

Dual-energy X-ray absorptiometry and biochemical markers of bone turnover after autologous stem cell transplantation in myeloma

Objectives: To evaluate the effect of high‐dose chemotherapy (HDT) followed by autologous stem cell transplantation (ASCT) on bone turnover and bone mineral density in a cohort of 39 consecutive patients with multiple myeloma (MM). Methods: Phosphorus and calcium parameters, bone turnover markers, and bone mineral density were studied. Timepoints were diagnosis (T1), just before ASCT (T2), 6 months (T3) after ASCT, and 1 yr (T4) after ASCT. Results: No bone mineral loss was shown on dual‐energy X‐ray absorptiometry (DXA) at T1 (lumbar Z‐score ?0.02, femoral neck Z‐score 0.77) or during follow‐up. Chronic vitamin D deficiency (25OHD3 11.7 ± 7.7 ng/mL at T1) and relative hyperparathyroidism from T2 to T4 were observed. In spite of this moderate hyperparathyroidism, serum C‐telopeptide of type I collagen (CTX) decreased significantly between T1 and T4. Bone alkaline phosphatase levels were low at diagnosis and showed no significant change after ASCT, unlike DKK1 levels that were high at diagnosis and decreased 6 months after ASCT in patients not previously treated with bisphosphonates. Conclusion: Bone demineralization is moderate in multiple myeloma. ASCT induces a decrease in bone resorption but no changes in bone formation, remaining low despite the decrease in DKK1. Bone mineral loss, evaluated by DXA, is moderate in multiple myeloma. High‐dose chemotherapy followed by ASCT leads to decreased bone resorption but osteoblastic bone formation remains low, in spite of reduced circulating DKK1.     

山东省缺碘地区中的“适碘”地区调查

目的 调查山东省缺碘地区中“适碘”地区分布,为碘缺乏地区的重新划定及实施科学补碘提供依据.方法 在山东省105个缺碘县(市、区),以村为单位,采集1～3份饮用人数最多的水源样品,水碘含量检测采用砷-铈催化分光光度法.地区划分标准:水碘含量＜ 10 μg/L为低水碘地区;水碘含量10～150μg/L为水碘适宜(适碘)地区;水碘含量＞ 150μg/L为高水碘地区.结果 本次调查涉及了14个市、105个县(市、区)、1337个乡(镇),收集水样65 716份,非空白水样65 572份,回收样品有效率达到99.8％,水碘中位数为5.57 μg/L.被调查的1337个乡(镇)中,有82.05％(1097/1337)的乡(镇)水碘中位数＜10 μg/L;有17.43％(233/1337)的乡(镇)水碘中位数介于10 ～ 150 μg/L;有0.52％(7/1337)的乡(镇)水碘中位数＞150 μg/L.结论 山东省“适碘”地区散在存在,且占有相当比例.在缺碘地区还存在高水碘地区,应重新划定缺碘地区,重视“适碘”地区居民碘营养状况,开展科学补碘.     

益生菌对功能性便秘的防治进展

随着饮食结构的改变及精神和社会压力的增大,便秘已成为困扰现代人的主要问题之一,严重影响着人们的生活质量.功能性便秘是指非全身疾病或肠道疾病引起的原发性持续性便秘,在不同人群中其发病率在2.0%-37.5%.传统的治疗功能性便秘的方式虽然安全可靠,且泻药能很快缓解便秘症状,但不能解决根本问题.因此,找寻一种安全有效的饮食或保健干预措施是非常必要的.便秘患者肠道微生态不同于健康人群,补充益生菌可改变这一现象.本文从介绍功能性便秘的发病现状入手,引入益生菌的概念,论述了益生菌防治便秘的机制、常用效应评价指标及目前的研究进展.     

胃大部切除术后3年胃癌患者生活质量的EORTC QLQ-C30与QLQ-STO22联合评价

目的:采用欧洲癌症研究与治疗组织(EORTC)生活质量核心30问卷调查(QLQ-C30)和QLQ-STO22评价胃癌术后3年患者的生活质量(QOL).方法:对96名胃大部切除术后3年的胃癌患者和90名年龄和性别相匹配的门诊体检健康者进行研究.胃癌患者和健康者平均年龄分别为53.2岁±8.1岁和54.8岁±7.9岁.88.5%患者为I期胃癌术后.患者自行完成QLQ-C30量表和QLQ-STO22量表,对各评分进行统计分析.结果:胃癌患者和健康者EORTC QLQ-C30整体健康状况和QOL评分无显著差异.胃癌患者的认知功能、情绪功能和疲劳要优于对照健康者.但胃癌患者恶心和呕吐评分、经济困难、反流、进食受限和体型评分较健康者差.其他评分未见显著差异.结论:胃癌术后3年,患者的QOL整体评分基本恢复至正常水平,但在某些方面,特别是与上消化道相关的症状(如恶心呕吐、反流、进食受限等)与健康者存在差距.有必要基于这些差异采取针对性的措施进行治疗和护理.     

Health-related quality of life and psychological well-being in elderly patients with haemophilia

Many persons with severe haemophilia reach seniority thanks to effective treatment. There is no information on health-related quality of life (HRQoL) of these patients, who had lived for many years when regular replacement therapy was unavailable. Italian patients with severe haemophilia aged ≥65 years born in the 1940s or earlier were compared with men without bleeding disorders matched for age and geography. HRQoL was assessed via generic and disease-specific questionnaires. Potential associations with concomitant illnesses, orthopaedic status, physical functioning, cognitive status and depression were evaluated. In addition, the newly adapted HRQoL questionnaire specific for elderly persons with haemophilia (Haem-A-QoL(Eldlery)) was psychometrically tested and validated. Thirty-nine patients, aged 65-78 years, were investigated, 33 with haemophilia A and six with haemophilia B, and compared to 43 controls, aged 65-79 years. Chronic blood borne viral infections, hypertension and arthropathy were more frequent in patients, whereas hypercholesterolemia and cardiovascular diseases were more frequent in controls. Psychometric characteristics of Haem-A-QoL(Elderly) showed good to excellent values for reliability and validity. HRQoL was worse in patients at EQ-VAS, WHOQOL-BREF and WHOQOL-Old. The highest impairments were found in patients by means of the haemophilia-specific Haem-A-QoL(Elderly) in such dimensions as 'physical activity & leisure', 'physical health' and 'view'. A poor orthopaedic status was negatively associated with HRQoL. Compared to age-matched controls elderly patients with haemophilia had an impaired HRQoL in association with their health status. The newly developed Haem-A-QoL(Elderly) proved to be a reliable and valid instrument for HRQoL assessment in elderly haemophilia patients.     

Why should we care about quality of life in persons with haemophilia?

The very high cost of haemophilia care, including the increase in use of factor prophylaxis in both children and adults requires that funders of clotting factor concentrates require objective measures of health, such as joint status and quality of life (QOL). Many clinical trials, especially those for licensing of new products, are including QOL instruments in their protocols to evaluate the patients' perspective of wellbeing before and during therapy. This article gives a perspective on QOL the importance of QOL measurement in the field of haemophilia and its impact on patient outcome.     

Seizure management at Auckland City Hospital Emergency Department between July and December 2009: time for a change?

Aims: To assess the management of epileptic seizures and status epilepticus in adult patients at Auckland City Hospital emergency department. This information will form the basis of future seizure management protocols and further research on the management of status epilepticus. Methods: The prehospital and acute hospital management of all adult seizure patients seen between 1 July 2009 and 31 December 2009 was reviewed with respect to seizure type, presence of first seizure, pre‐existing epilepsy diagnosis and disposition from the emergency department. Results: Two hundred and fifty‐five seizure events were identified in 227 patients. Nineteen patients presented twice during the study period and three patients presented three or more times. Generalised seizures were much more common than focal seizures. There were 75 presentations with first seizure (29.4%). Thirty‐seven patients (49.3%) with a first seizure received treatment with an anti‐epileptic drug. Status epilepticus occurred on 12 occasions (4.7%) with only three patients receiving lorazepam as treatment. The majority of seizure patients were managed by emergency department staff (58.4%) while general medicine (17.6%) and neurology (11.8%) teams managed fewer patients. Phenytoin was used in 56 patients (22%) with the majority (n= 43) receiving intravenous phenytoin. Many of the patients who received intravenous phenytoin were not subsequently discharged on that medication (46%). Conclusions: More patients than would be expected received treatment after their first seizure. Phenytoin was a widely used anti‐epileptic drug. There was a wide variability in the management of status epilepticus, and intravenous lorazepam was underutilised.     

成年大鼠接受高脂或热量限制饮食对其衰老后身体构成、糖脂代谢及胰岛内β、α细胞分泌功能影响的比较研究

目的通过对成年大鼠给予高脂饮食（HFD）或能量限制饮食（CRD）干预至老年期,比较糖脂代谢指标及胰岛细胞内胰岛素,胰高糖素分泌功能的变化,探讨热量摄入在衰老伴胰岛素抵抗中的作用机制。方法14～16月龄雄性SD大鼠45只分别给予高脂饮食（热量为5．86kcal／kg,以脂肪供能为主,占总热量的43．27％）,正常饮食（热量为3．2kcal／kg）及限制能量摄入（热量为1．6kcal／kg）,分别在干预0,8,16周时检测大鼠的体质量,腹腔脂肪,体脂比,空腹血糖（FBG）、甘油三脂（TG）、胆固醇（TC）、血浆游离脂肪酸（FFA）及血浆胰岛素水平,并根据FBG和空腹胰岛素计算稳态胰岛素抵抗指数（HOMA—IR）、HOMA-β和胰岛素敏感性（ISI）。应用免疫组化方法检测胰岛内胰岛素及胰高血糖素分泌及表达量的变化。结果（1）成年大鼠衰老过程中,体质量、腹腔脂肪、体脂比、FFA、TG、FBG及胰岛素均随年龄的增加而升高;由此计算的HOMA—IR升高,ISI和HOMA-β降低,但差异无统计学意义。胰岛细胞内胰岛素／胰高血糖素的水平和胰岛D凤的面积未见明显变化。（2）HFD组大鼠16周时,HOMA—IR明显上升,ISI明显降低,体质量、腹腔脂肪、体脂比显著增加,FFA显著升高,血胰岛素、胰岛内胰岛素和胰高血糖素分泌量增多,差异均有统计学意义（P〈0．05）。（3）CRD干预时HOMA。IR显著降低,ISI和HOMA—p显著升高;体质量、腹腔脂肪显著降低（P〈0．05）,空腹血糖及胰岛素降低、血浆FFA,TG,TC水平降低;胰岛内胰岛素和胰高血糖素的表达水平亦显著性降低。结论SD大鼠出现胰岛素抵抗、胰岛素敏感性和胰岛13细胞分泌功能下降等增龄变化,因自成年期开始HFD干预而进一步恶化,因CRD干预显著改善;FFA是增龄过程中受HFD或CRD的影响早且变化最持久的因子;HFD以胰岛素分泌增高为主,作用靶点可能主要在胰岛D细胞,而CRD以降低胰高血糖素为主,作用靶点侧重于α细胞。