Hepatitis and cholestasis in infancy: clinical and nutritional aspects

A major complication of cholestasis is fat malabsorption related to decreased intestinal bile acids, which leads to malnutrition and fat-soluble vitamin deficiency. The impaired excretion of bile acids leads to a low intraluminal micellar concentration that causes long-chain triglyceride lipolysis and absorption to be ineffective. Medium-chain triglycerides (MCTs) are more readily absorbed when there are low concentrations of bile acids and therefore are a good source of fat calories; MCTs can be administered as MCT-containing formulas. In those children who are unable to take sufficient calories by mouth, it is important to start nocturnal enteral feeding to improve nutritional status. In infants with cholestasis, the absorption of fat-soluble vitamins (A, D, E and K) that require bile acids is also impaired, and supplementation is mandatory. Vitamin K deficiency may be responsible for hypoprothrombinaemia, which may lead to bleeding diathesis, Vitamin K (phytomenadione) should therefore be promptly administered intravenously, at a dose of 1 mg. Chronic vitamin E (α-tocopherol) deficiency is associated with a progressive neuromuscular syndrome that can cause cerebellar ataxia, areflexia and peripheral neuropathy. Supplements are given orally in doses of 3–5 times the normal requirement if cholestasis is incomplete. In complete cholestasis, supplements must be given intramuscularly at monthly intervals. In infants who fail to thrive, dietary supplements of carbohydrate polymers and MCTs are required.     

后程适形放射治疗Ⅲ期非小细胞肺癌

目的评价三维适形放射治疗对Ⅲ期非小细胞肺癌患者局部控制率和生存率的影响。方法26例Ⅲ期非小细胞肺癌患者先采用6／15MV X线常规外放疗DT38-40Gy，后采用三维适形放疗2．5—3Gy／次．1次／d，累计量70—74GY。结果1、2年生存率分别为69．2％、46．1％，1、2年局部控制率分别为88．5％、76．4％。结论后程三维适形放射治疗可提高Ⅲ期非小细胞肺癌患者近期生存率及局部控制率，并未增加毒副反应。     

肝癌介入治疗和三维适形放疗的临床I／Ⅱ期试验

目的：探讨利用介入治疗和3—DCRT对肝癌进行综合治疗的疗效。方法：建立肝癌的三维适形放疗(3—DCRT)技术，对有病理组织学或细胞学证实的原发性肝癌或单发的转移性肝癌患者进行3—DCRT临床I／Ⅱ期试验，观察其毒、副反应和近期疗效。计划常规分割照射总剂量50Gy至58Gy。3—DCRT之前所有患者进行经导管肝动脉化疗栓塞(TACE)介入治疗。治疗计划中≥90％的等剂量面包绕PTV(planning target volume)，平均肝脏剂量均小于30Gy，V30Gy(接受≥30Gy剂量照射的正常肝百分体积)均小于33％。结果：30例肝癌患者包括21例原发性肝癌入组研究。部分缓解(PR)24例，一年生存率76％，中位生存时间8个月。肝脏急性不良反应反应1级2例，肝脏急性不良反应反应≥2级0例。治疗后随访至今无严重放射性肝损伤发生。结论：系统建立可靠、安全的肝癌的3-DCRT技术，在平均肝脏剂量≤30Gy且接受≥30Gy剂量照射的正常肝百分体积均小于33％的剂量学条件下，常规分割照射剂量58Gy以上是可行的。     

三维适形放疗治疗35例恶性梗阻性黄疸

目的：观察三维适形放射治疗恶性梗阻性黄疸的疗效，探讨其更有效的治疗方法。方法：1998年10月—2000年12月，对35例恶性梗阻性黄疸患者行三维适形放疗，每次3—4Gy，每周5次，总共10—12次。结果：所有病例均完成治疗，病灶完全缓解率(CR)8例，占24％，部份缓解(PR)22例，占63％，无变化5例，占13％，总有效率87％。29例黄疸均有不同程度消退，没有严重的并发症发生。一、二年生存率分别为70％、21％。结论：三维适形放疗对恶性梗阻性黄疸有较好的疗效，绝大多数患者均能耐受，是治疗恶性梗阻性黄疸的一种有效方法。     

Balloon Dilatation of the Pulmonary Valve in a Patient with Pulmonary Atresia and Intact Ventricular Septum Using a Commercially Available Radiofrequency Catheter

Using a commercially available 5F deflectable radiofrequency catheter, we have succeeded in percutaneous valvotomy of an imperforate pulmonary valve and consecutive balloon dilatation in a baby with pulmonary atresia and intact ventricular septum. After the procedure, right ventricular systolic pressure fell from 125 mmHg to 65 mmHg, and right ventriculography demonstrated anterograde blood flow into the pulmonary arteries. There were no major complications. Doppler echocardiography at 1 year after the procedure demonstrated a pressure gradient across the pulmonary valve of 20 mmHg with mild pulmonary and tricuspid regurgitations.     

转化生长因子-α和myc癌基因蛋白在卵泡发育与黄体形成及退化中的表达

目的：探讨转化生长因子（ＴＧＦ）－α和ｍｙｃ癌基因蛋白（ｍｙｃ蛋白）对人卵巢卵泡发育的局部调控机理。方法：收集３６例月经规则、因不同妇科情况切除的子宫及卵巢标本，采用免疫组织化学方法，研究ＴＧＦ－α和ｍｙｃ蛋白在卵巢组织中的表达。结果：卵母细胞在始基卵泡阶段，ＴＧＦ－α和ｍｙｃ蛋白的表达呈强阳性，随着卵泡的发育与成熟表达逐渐减弱。在颗粒细胞中，ＴＧＦ－α和ｍｙｃ蛋白表达均出现在窦前卵泡阶段，随卵泡的增大与成熟，ＴＧＦ－α表达逐渐增强。闭锁卵泡中ＴＧＦ－α和ｍｙｃ蛋白表达仅限于卵泡膜细胞。在晚期退化的黄体中，ＴＧＦ－α和ｍｙｃ蛋白表达均局限于黄体中央瘢痕周围的黄体膜细胞。结论：ＴＧＦ－α和ｍｙｃ蛋白作为卵巢内局部的调节因子，通过自分泌和旁分泌途径，协同参与人卵母细胞最初的生长、卵泡细胞的增殖分化和黄体细胞凋亡的过程。     

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.     

Stepwise interventional approach in a neonate with pulmonary valve atresia and intact ventricular septum

In neonates with pulmonary atresia and intact ventricular septum the aims of therapy are maintenance of pulmonary blood flow and right ventricular decompression in order to achieve right ventricular support of the pulmonary circulation. Recent developments in interventional heart catheterization with pulmonary radiofrequency-assisted balloon valvuloplasty and ductal stent implantation offer an alternative to the classical surgical approach. We report on a neonate with membranous pulmonary atresia and intact ventricular septum, in whom a large interatrial right-to-left shunt via the foramen ovale persisted after radiofrequency-assisted pulmonary balloon valvuloplasty on the 2nd day of life. The interatrial shunt prevented adequate right ventricular filling and antegrade pulmonary perfusion leading to severe cyanosis (transcutaneous oxygen saturation 40%). In order to increase pulmonary blood flow and raise left atrial pressure, the arterial duct was stented. After ductal stenting, prostaglandin was discontinued and the transcutaneous oxygen saturation remained stable around 89%. At follow up after 7 weeks the foramen ovale had decreased in size and only a small left-to-right shunt was present, documenting the effectiveness of this approach. Conclusion Based on the present case we propose a stepwise interventional approach for the neonate with pulmonary atresia and intact ventricular septum. If cyanosis persists after isolated pulmonary valvuloplasty despite adequate right ventricular function, ductal stent implantation can reduce interatrial shunting and thus improve oxygen saturation. Received: 12 January 1998 / Accepted: 15 February 1998     

A simple and safe method to visualize the inferior pouch in esophageal atresia without fistula

The authors studied the true “dynamic” distance between the esophageal stumps in type I atresia in order to perform the delayed anastomosis at the most favorable time. The position of the inferior pouch was fluoroscopically evaluated in four patients, inserting a Hegar dilator through the gastrostomy. The superior esophageal pouch was delineated by a Replogle tube. No anesthesia was required. In all cases the procedure was simple, safe, fast, and accurate. No complications occurred, and patients could be operated upon at the optimal time. Accepted: 16 May 1997     

Does gut atresia cause polyhydramnios?

Fetal gut atresia is variably associated with polyhydramnios. In order to determine which pregnancies will develop polyhydramnios, the case notes of 80 babies with gut atresia and stenosis were reviewed. Maternal polyhydramnios developed in all cases of pure oesophageal atresia (n=8), all cases of Type III duodenal atresia (DA) with a non-bifid bile duct (n=8), 80% of cases with type I DA (n=10), and 24% of atresias of the small intestine (n=34). Polyhydramnios did not develop in any case where there was not total obstruction except in 1 baby with DA and a bifid bile duct (BBD). These included stenosis of the oesophagus and duodenum (n=17) and DA type III with a BBD (n=3). These results support the role of fetal swallowing and fluid absorption by the fetal gastro-intestinal tract in the regulation of amniotic fluid volume. Accepted: 24 February 1997