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。     

溃疡散预防消化性溃疡复发临床观察

目的 :观察溃疡散对消化性溃疡的抗复发治疗的临床效果。方法 :随机将 80例患者分成溃疡散治疗组 (4 2例 )和雷尼替丁对照组 (38例 )并同时观察两组患者治疗前后溃疡复发情况。结果 :治疗后治疗组溃疡复发率明显低于对照组 ,P <0 .0 1,两组对比有非常显著性差异。结论 :治疗组能明显的改善患者临床症状和降低消化性溃疡复发率。     

The insulin-like growth factor-II/mannose-6-phosphate receptor: structure, distribution and function in the central nervous system

The insulin-like growth factor-II/mannose-6-phosphate (IGF-II/M6P) receptor is a multifunctional single transmembrane glycoprotein which, along with the cation-dependent M6P (CD-M6P) receptor, mediates the trafficking of M6P-containing lysosomal enzymes from the trans-Golgi network (TGN) to lysosomes. Cell surface IGF-II/M6P receptors also function in the degradation of the non-glycosylated IGF-II polypeptide hormone, as well as in the capture and activation/degradation of extracellular M6P-bearing ligands. In recent years, the multifaceted role of the receptor has become apparent, as several lines of evidence have indicated that in addition to its role in lysosomal enzyme trafficking, clearance and/or activation of a variety of growth factors and endocytosis-mediated degradation of IGF-II, the IGF-II/M6P receptor may also mediate transmembrane signal transduction in response to IGF-II binding under certain conditions. However, very little is known about the physiological significance of the receptor in the function of the central nervous system (CNS). This review aims to delineate what is currently known about IGF-II/M6P receptor structure, its ligand binding properties and role in lysosomal enzyme transport. It also summarizes the recent data regarding the role of the receptor in the CNS, including its distribution, possible importance for normal and activity-dependent functioning as well as its implications in neurodegenerative disorders such as Alzheimer's disease (AD).     

Nasopharyngeal and nasal malignant lymphoma: a clinicopathological study of 54 cases

Forty-one cases of nasopharyngeal and 13 cases of nasal malignant lymphoma have been examined histologically and immunohistochemically. All of the cases were non-Hodgkin's lymphoma; one case was of follicular type and the remaining 53 were of diffuse type. Large cell lymphoma comprised 48% of cases and most of the immunoblastic lymphomas showing pleomorphism occurred in the nose. Twenty-seven cases were of T-cell and 21 of B-cell phenotype. The predominance of T-cell lymphoma was due to an increased incidence of these in the nose, the T:B ratio of 3.33:1 contrasting with a 1:1.05 ratio in the nasopharynx. Nasopharyngeal lymphomas seem to show an intermediate incidence between the T-cell predominance in the nose and a B-cell predominance in the oropharynx. Since the large cell type of lymphoma was predominant, the differential diagnosis from undifferentiated carcinoma is important and is facilitated by the use of immunostaining methods.     

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。