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。     

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.     

50例冠心病某些生化基础观测的临床诊断意义

对５０例冠心病患者进行某些生化基础观测，发现冠心病肾阴虚与糖耐量减低有关，肾阳虚患者尿１７－羟低於正常；而冠心病标证有其生化基础，与甘油三酯增高有密切相关。     

生长激素治疗对GHD患儿免疫功能的影响

对生长激素缺乏症（GHD）患儿生长激素（GH）治疗前后的免疫功能改变进行了观察。结果显示：（1）GHD患儿NK细胞活性明显降低，经GH治疗3个月后恢复到正常水平；（2）GHD患儿治疗前IL－1a和IL－2活性偏低，治疗后两者有逐渐增高的趋势；（3）治疗前后CD细胞亚群、sIL－2R和LPS诱生的TNFa含量均无明显变化。认为GH缺乏症患儿存在一定的免疫功能缺陷，而GH有调节其免疫功能的作用。     

7～12岁儿童铁缺乏及补充对身体做功能力的影响

以１１６０名７～１２岁儿童为研究对象，共筛查出ＩＤＡ儿童２６９人，贫血患病率为２３．１９％。抽取６０名ＩＤＡ儿童分为二组（补铁组、安慰剂组），另选３０名正常儿童为对照组。补铁前后测Ｈｂ、ＦＥＰ、ＰＷＣ１７０、ＲＰＷＣ１７０、ＶＯ２ｍａｘ及血乳酸浓度值。补铁前，ＩＤＡ儿童反映ＰＷＣ大小的各项指标值与正常儿童之间存在显著性差异；经三个月补铁后，补铁组以上各指标值恢复正常水平。结果提示：轻度ＩＤＡ对７～１２岁儿童ＰＷＣ有明显影响，并能通过铁剂治疗得以矫正。     

铁强化食盐预防缺铁性贫血效果的观察

本文试以食盐为载体,使在食盐中每日为学龄前儿童增加元素铁5～10mg,对象为5岁的幼儿园儿童165名,分别在城镇与农村观察一年。初步的结果是:实验组与对照组在开始时的血红蛋白水平一致,但一年后,实验组由原来的平均值11.78±0.57增加至12.72±0.72,比对照组有明显的改善,这是解决缺铁性贫血的一种实际方法。     

A controlled trial of dapsone versus pyrimethamine-sulfadoxine for primary prophylaxis of Pneumocystis carinii pneumonia and toxoplasmosis in patients with AIDS

Pneumocystis carinii pneumonia (PCP) is the most common opportunistic human immunodeficiency virus (HIV)-related infection, occurring in 85 % of HIV infected patients without prophylaxis. Preventive treatment is required when CD₄ cell count falls below 200 cells per cubic millimeter. Cotrimoxazole has been shown to be highly effective but alternative drug regimens are often necessary because of the frequent drug hypersensitivity exhibited by HIV infected patients. The aim of this prospective, open, randomized, one-site study, involving HIV-infected patients with a CD₄ cell count below 200/mm³, or a percentage under 20%, randomly assigned to receive either dapsone 50 mg daily or Fansidar^® one tablet weekly, was to compare the efficacy and safety of these drugs in the primary prophylaxis of PCP. Both dapsone and Fansidar^® appear to be safe and effective alternative agents for the prevention of PCP. Their role in Toxoplasma gondii prophylaxis requires further evaluation.     

Validation of a self-administered diet history questionnaire using multiple diet records

The validity of a self-administered diet history questionnaire has been estimated using as the reference data the mean of three 4-day diet records collected over the year prior to the administration of the questionnaire, in 1985–1986. Subjects were women ages 45–70 years, participants in the Women's Health Trial Feasibility Study, a multi-center clinical trial in which some women were randomized to be taught to adopt and maintain a low-fat diet, while others maintained their usual diet. The questionnaire produced group mean nutrient estimates closely approximating the values obtained by three 4-day records, e.g. in the usual-diet group, 37.7% of calories from fat by both food records and by questionnaire, and in the low-fat, group, 21.3% of calories from fat by food records and 23.7% of calories from fat by questionnaire. Correlations between questionnaire and diet records for per cent of calories from fat were 0.67 and 0.65 respectively in the two groups; most correlations were in the 0.5–0.6 range, and were similar to those achievable by a single 4-day food record.     

A randomized, blinded trial of uncooked cornstarch to diminish nocturnal hypoglycemia at Diabetes Camp

Objective: To determine if uncooked cornstarch, as part of the evening snack, can avert nocturnal hypoglycemia in type 1 diabetes. Research Design and Methods: Fifty-one campers and counselors at the American Diabetes Association Camp in San Bernardino, CA were randomly assigned to receive 5 g of uncooked cornstarch as part of the 21:00 evening snack vs. a standard snack of equivalent carbohydrate content. Each snack was given for five nights and the participants and medical personnel were blinded as to assignment. Midnight and 07:00 finger stick blood glucose levels were compared with values <60 mg/dl defined as hypoglycemia and values >250 mg/dl defined as hyperglycemia. Results: There were 218 midnight and 222 07:00 values for comparison. There were six episodes of hypoglycemia at midnight and nine episodes of hypoglycemia at 07:00 for the cornstarch snack nights vs. 30 hypoglycemia episodes at midnight and 21 at 07:00 for the standard snack nights (P < 0.001 and < 0.05, respectively). There was no difference in the number of hyperglycemic events at midnight or 07:00 for the cornstarch vs. standard snack nights. At midnight, 12% of campers had hypoglycemia after the cornstarch snack vs. 46% after the standard snack (P < 0.001), and at 07:00, 16% had hypoglycemia after cornstarch vs. 26% after the standard snack (P = 0.327). Conclusions: These data suggest that uncooked cornstarch, as part of the evening snack, can diminish the nighttime and morning hypoglycemia associated with type 1 diabetes, without causing hyperglycemia.     

Comparison of phosphoprotein isolated from mature and immature human tooth roots

Mature (average patient age = 29.5 yr, closed apical foramen) and immature (average patient age = 17.5 yr, open apical foramen) root shards were placed in dialysis tubing and demineralized to completion using either 10% disodium EDTA plus protease inhibitors or 0.6 N HCl. The demineralized shards were re-extracted (five times) with 0.05 M tris-HCl, 1.0 M NaCl and then collagenase digested. No major differences were observed in chromatograms of extracts, re-extracts or collagenase digests from root shards demineralized in either way. In contrast, chromatograms of immature and mature roots showed qualitative differences. Chromatograms of mature roots demineralized in either way showed broader protein peaks and less organic phosphorus than those from immature tooth roots. A distinct band amid degraded phosphoprotein (150 K) was found in SDS-PAGE gels (7.5%) from EDTA-extracted immature tooth roots but not from mature tooth roots. Electroelution of this band revealed a typical phosphoprotein amino-acid profile containing increased aspartic acid and serine residues. Comparison of the total phosphoprotein and amino acid composition of extracts, re-extracts and collagenase digests revealed that phosphoprotein, serine and to a lesser extent aspartic acid were recovered in greater quantities from immature roots than mature tooth roots. These data suggest that the degree of maturation is crucial to the isolation of an intact phosphoprotein and provides additional evidence that human dentine phosphoprotein undergoes amino acid compositional changes during maturation.