A patient with congenital lipoid adrenal hyperplasia evaluated by serial abdominal ultrasonography

Adrenal enlargement was followed by serial ultrasonography in an infant with congenital lipoid adrenal hyperplasia (lipoid CAH) from day 12 until 2 years and 4 months of age, when they were no longer detectable. Contrary to other types of CAH in which the configuration changes soon after replacement therapy, this infant with lipoid CAH showed persistent adrenal cortex enlargement due to massive accumulation of lipids and cholesterol resulting in a damaged glandular cyto-architecture. Conclusion ultrasonographically persistent enlargement of the adrenals after replacement therapy is suggestive of the lipoid form of CAH. Received: 3 June 1997 / Accepted in revised form: 19 December 1997     

Expression of Clara cell 10-kDa protein (CC10) in congenital diaphragmatic hernia

Clara cell 10 kDa protein (CC10) has been thought to be fairly specific to Clara cells and a major secretory protein that is both synthesized and released from Clara cells. In the present study, morphometric analyses of the immunohistochemical expression of CC10 were carried out on the bronchioles of human neonates with congenital diaphragmatic hernia (CDH) and then compared with morphometric analyses from a gestationally and postnatally age-matched control group in order to clarify the immaturity of Clara cells in CDH lungs. No difference was found in CC10 expression between the affected side and the unaffected side of the lungs in the CDH group. However, compared with the lungs of the control group, the CDH group showed a significant decrease in CC10 expression, namely, the ratio of CC10-positive cells per bronchiole, per unit perimeter of bronchiole, and per unit bronchiolar surface area. These results suggest that in the lungs of CDH cases, a possible delay in either functional maturation or the development of CC10 synthesis by the bronchioles may exist, and this retardation of functional maturation of the airway is also considered to play a role in the postnatal respiratory insufficiency observed in CDH patients. Accepted: 25 February 1998     

静脉全麻复合骶管阻滞在婴幼儿先天性心脏病介人封堵治疗的研究

目的比较静脉全麻复合单次骶管阻滞麻醉与单纯静脉全麻用于婴幼儿先天性心脏病介入封堵治疗的效果。方法616例婴幼儿先天性心脏病介入封堵治疗患者随机分成两组:骶管阻滞加静脉全麻(I组);另一组用全凭静脉麻醉(II组)。结果全麻药的用量,II组比I组明显增多(P<0.05);I组苏醒时间短,分泌物少,手术后呕吐发生率少(P<0.05)。结论静脉全麻复合小儿单次骶管阻滞麻醉比全凭静脉麻醉用于婴幼儿先天性心脏病介入封堵治疗麻醉费用少,生命体征平稳,麻醉并发症少,值得推广。     

先天性甲状腺功能低下症57例随访观察

目的 了解早期治疗对先天性甲状腺功能低下症预后的影响及新生儿筛查工作的意义.方法 对在湖南省妇幼保健院治疗的57例甲低患儿进行数年的追踪观察,并评价其体格发育和智力水平.结果 3个月以前开始治疗者体格发育及智力可达到同龄儿童正常水平,治疗越早,预后越好.结论 早期治疗可明显改善预后,减少残疾儿童的发生,同时提示新生儿筛查工作对提高人口素质有重要意义.     

A rare case of isolated accessory mitral tissue in an asymptomatic adult female

Accessory mitral valve tissue (AMVT) is a rare congenital cardiac anomaly, which is associated with other congenital heart diseases. It is diagnosed in neonates or childhood and rarely in adulthood. Nevertheless, AMVT is an incidental finding or described as isolated. Echocardiography, especially three-dimensional echocardiography is considered as an optimal imaging technique for AMVT diagnosis. We herein presented an asymptomatic adult AMVT cases with significant left ventricular outflow tract obstruction and surgical excision was recommended.     

斯坦曼针引导股骨粗隆下外展截骨术治疗先天性髋内翻

斯坦曼针引导股骨粗隆下外展截骨术治疗先天性髋内翻@白德明$山西省儿童医院小儿外科!030013 @王代全$山西省儿童医院小儿外科!030013 @高书林$山西省儿童医院小儿外科!030013 @龙江涛$山西省儿童医院小儿外科!030013~~     

Pregnancy outcome after gestational exposure to the new macrolides: a prospective multi-center observational study

Objectives

To determine whether the use of the new macrolides (azithromycin, clarithromycin, roxithromycin) during the first trimester of pregnancy is associated with an increased risk of major malformations.

Study design

In a prospective multi-center study, pregnancy outcome was compared between pregnant women exposed to one of the new macrolides during the first trimester of pregnancy and two comparison groups one exposed to other antibiotics and the other to other non-teratogenic medications. All women enrolled in the study called one of the three participating teratogen information services (TIS). Group 1 macrolides (n = 161), group 2 other antibiotics (n = 213) and group 3 non-teratogens (n = 740).

Results

A total of 161 women exposed to the new macrolides (118 were exposed in the first trimester of pregnancy) and 953 from a comparison groups were followed up. The rate of major malformations in the study group was 4.1% compared to 2.1% in the other antibiotics exposed group (OR = 1.41, 95% CI 0.47–4.23) and 3.0% in the non-teratogens exposed group. The rate of elective terminations of pregnancy was significantly higher in the exposed group in compare to both comparison groups.

Conclusion

Our study, although relatively small sized, suggests that the use of the new macrolides during the first trimester of pregnancy does not represent an increased risk for congenital malformations strong enough for an induced abortion after such an exposure. Elective terminations of pregnancy because of early exposure to these medications should be reconsidered.