Sideropenic Dysphagia综合征膜性蹼26例分析

目的 :研究SideropenicDysphagla (S -D)综合征膜性蹼产生的机制。方法 :回顾分析S -D综合征2 6例的临床资料。结果 :影像学检查显示膜性蹼在颈段食道前壁呈 2mm深的模样陷凹。血液学检查为缺铁性低血红蛋白性贫血改变。结论 :缺铁性贫血是S -D综合征的原因 ,铁剂治疗有效     

Neuro-toxic interaction in alcohol-treated,thiamine-deficient mice

Summary Groups of adult male mice were either fed a thiamine-deficient diet for 10 weeks and thereafter treated with ethanol by making them inhale vapourized cane spirit for 10 weeks, or given both treatments simultaneously. The brains of these mice were then searched for degeneration using both light and electron microscopy. No degenerating nerve cells were observed in any animal in the cerebral cortex, hippocampus, cerebellum, olfactory bulbs, midbrain or hindbrain. However, axon terminal degeneration was seen in the olfactory bulbs and deep cerebellar nuclei in mice given the combined treatment. No cerebellar degeneration was found and only little degeneration was present in the olfactory bulbs of mice given the two treatments at different times. Thus, the combined treatment of alcohol and thiamine deficiency produced more brain damage than the sum of that produced by the two treatments given separately. This represents the first experimental in vivo demonstration of a biochemical interaction between these two factors in alcohol-related brain damage. The findings of long-term animal treatment with models using thiamine antagonists are compared.Supported by the special Research Fund Programme of Monash University (Post-Doctoral Fellowship)     

昆明城区、大理农村7月～7岁儿童铁缺乏调查分析

目的 :了解昆明城区及大理农村 7月～ 7岁儿童目前的铁缺乏水平 ,进一步展开对儿童铁缺乏症的防治 ,降低儿童营养性缺铁性贫血的患病率 .方法 :采用抽样调查的方法对两地 7月～ 7岁儿童共 96 9名进行血清铁蛋白 (SF)、锌原卟啉 (ZPP)、血红蛋白 (Hb)实验室检查 .结果 :7月～ 7岁儿童的总铁缺乏检出率为 2 5 1% ,铁减少检出率为 19 0 % ,贫血患病率为 6 1% ;男女童患病率在 1岁以前差别较大 ,1岁以后差别不明显 ;各年龄组患病率以 7月～ 1岁组最高 ;早产儿、低出生体重儿童患病率相对较高 ;铁缺乏的儿童营养不良的患病率高于正常儿童 .结论 :儿童缺铁性贫血仍要引起重视 .在防治中要抓重点人群 ,除改变膳食结构和饮食习惯 ,对目标人群要适当补充铁剂预防     

Pregnancy outcome in Factor XI deficiency: incidence of miscarriage, antenatal and postnatal haemorrhage in 33 women with Factor XI deficiency

Pregnancy complications in women with Factor XI deficiency were assessed in this retrospective analysis. All nonnulliparous women registered with Factor XI deficiency in the East Midlands region were included. Each woman was classified into 'bleeder' or 'nonbleeder'. Rates of antenatal and postnatal bleeding and miscarriage rate were recorded. A total of 33 women had 105 pregnancies. Pregnancy and delivery was uneventful in 70% of the cases. Postpartum haemorrhage (PPH) appears increased in women with a 'bleeding' phenotype with a highly significant difference between 'bleeders' and 'nonbleeders' (relative risk [RR] 7.2; CI 1.99–25.9). Miscarriage rate appeared unchanged. We conclude that PPH is increased in a subgroup with a bleeding phenotype. Larger studies are needed to define the underlying factors.     

王静安治脾肾两虚脑瘫的比较研究

小儿脑瘫在中医学中属于五迟、五软的范畴,儿科鼻祖钱乙在治疗本病上重视培补先天,并创立了著名的"六味地黄丸"。身为中华医学会儿科专委会名誉主任委员的王静安,认为先天虚损是本病的重要病因之一,但是后天失养,亦可导致先天愈加亏虚,所以在治疗时重视顾护中州生升之气。对王老与钱乙在诊治脑瘫足软行迟方面的比较,另又比较了两例不同类型的脑瘫。     

QT离散度对急危重病人预后的评估

目的　研究QT离散度对急危重病人预后的预测价值。方法　死亡组 4 7例、疾病组 4 0例和正常组 4 3例。记录 12导联心电图 ,人工测量各导联R -R间期和QT间期 ,计算R -R、QTc、QTd和QTcd。结果　死亡组的R -R、QTc、QTd和QTcd分别为 0 5 6 2± 0 2 2 0ms、0 35 6± 0 0 6 9ms、0 0 5 6± 0 0 33ms和 0 0 79± 0 0 4 6ms ;疾病组的R -R、QTc、QTd和QTcd分别为0 80± 0 134ms、0 4 16± 0 0 3ms、0 0 34± 0 0 14ms和 0 0 39± 0 0 16ms;正常组的R -R、QTc、QTd和QTcd分别为 0 82 5± 0 0 88ms、0 4 0 2± 0 0 3ms、0 0 2 7± 0 0 15ms和 0 0 31± 0 0 15ms。比较死亡组、疾病组和正常组发现 ,死亡组和疾病组的R -R、QTc、QTd和QTcd存在显著差异 (P分别 <0 0 0 1、<0 0 0 2 5、<0 0 0 1和 <0 0 0 1)。死亡组和正常组的R -R、QTc、QTd和QTcd存在显著差异 (P分别 <0 0 0 1、<0 0 0 2 5、<0 0 0 1和 <0 0 0 1)。结论　QTd对急危重病人的预后有一定的预测价值 ;但其它临床应用价值存在局限性 ,有待进一步探索。     

原发性肉碱缺乏致脂质沉积性肌病的临床与病理特点

目的 分析原发性肉碱缺乏致脂质沉积性肌病（LSM）的临床与病理特点。方法 回顾性分析4例可能LSM患者的临床资料。结果 本组患者为亚急性或慢性起病，主要表现为近端肌无力，疲劳不能耐受；血清肌酶有不同程度的升高；肌电图示肌源性损害；病理检查示肌纤维内可见大量细小空泡和裂隙形成；MGT染色无破碎红纤维，油红O染色显示空泡为大量脂滴充填；受累纤维以Ⅰ型纤维为主。电镜证实肌纤维内脂滴堆积，可伴有线粒体的轻度增多。改善能量和糖皮质激素治疗有效。结论原发性肉碱缺乏致LSM是一种以易疲劳和肌无力为主要临床表现的脂质代谢障碍性肌病，病理改变以肌纤维内脂滴堆积为主，一般不伴有线粒体结构的明显异常。糖皮质激素治疗可获得良好疗效。     

Functional Symptoms and Somatoform Disorders in Children and Adolescents: The Role of Standardised Measures in Assessment

Functional or unexplained medical symptoms (physical symptoms that are not adequately explained by organic factors and where a major role for psychological factors is assumed) are common amongst children in the general population but can also be an expression of somatisation and somatoform disorders. Co-morbid psychopathology is common. We describe measures mostly used in research into problems related to somatisation in children and adolescents that may be helpful to clinical researchers. Some address the nature and severity of physical symptoms, others document illness attitudes, beliefs and functional impairment, and a third group assesses emotional symptoms. Questionnaires can be helpful for clinicians in quantifying (i) the nature and severity of somatic symptoms and associated functional impairment, (ii) contributory health attitudes and illness beliefs and (iii) co-morbid or primary anxiety and depressive disorders. Together with pain and activity diaries and careful documentation of school attendance, these measures may also be helpful in monitoring treatment response.     

Changes with growth hormone treatment in growth hormone deficient children

A total of 54 previously untreated patients (15 girls, 39 boys) with poor growth due to idiopathic growth hormone deficiency (IGHD) were treated with human growth hormone (hGH), continuously up to 4 years. All of the patients had a peak hGH level which was below 10 ng/mL after at least two pharmacological tests and/or blunted physiologic hGH secretion, and their height was below ?2.5 s.d. for age and gender. After the 1st year of therapy, height velocity (HV) increased significantly when compared with baseline (from 3.18 ±0.76 cm/year to 9.17±1.03 cm/year; P <0.001), declined during the 2nd year and then remained significantly higher than pretreatment HV. When considering improvement in height expressed by height standard deviation score (SDS), during the therapy all of the patients showed a significant gain ± 1.72±1.09 (from ?4.11±0.61 to ?2.21±0.48). The height values were significantly higher than pretreatment, but remained below ?2 s.d. after 4 years of hGH therapy in our patients. Increased height velocity has been sustained, but height improvement after therapy was inversely correlated to height SDS for chronological age of patients at the start of therapy. In conclusion post-treatment height has been shown to be related to height deficit at the beginning of therapy. Therapy was well tolerated with no local or systemic adverse effects or acceleration of bone age.