儿科加强医疗病房全身炎症反应综合征和多器官功能不全综合征的临床分析

目的应用新概念———全身炎症反应综合征（ＳＩＲＳ）和多器官功能不全综合征（ＭＯＤＳ）认识多器官功能衰竭（ＭＯＦ）。方法回顾性总结分析１９９５年１月～１９９７年１２月我院儿科加强监护病房收治的危重患儿，根据Ｈａｙｄｅｎ修订的ＳＩＲＳ诊断标准和全国小儿急诊学组拟定的ＭＯＦ诊断标准进行临床分析。结果全组３０４例患儿，符合ＳＩＲＳ标准２５２例，占８２．９％．原发病有感染性疾病和非感染性疾病。在ＳＩＲＳ和ＭＯＤＳ病程发展过程中部分患儿出现一过性肝、肾、胃肠道和脑等器官功能受损的表现，最终进展为ＭＯＤＳ共６５例，占２５．８％。ＭＯＤＳ的病死率为４３．１％（２８／６５）。发生ＭＯＤＳ的高危因素为入院危重评分＜７５分，明显高代谢反应和合并Ｃ反应蛋白，而与年龄无明显关系。结论ＳＩＲＳ和ＭＯＤＳ是小儿加强医疗病房中常见病症，病死率较高。ＳＩＲＳ和急诊学组制定的ＭＯＦ诊断标准简单实用，但应根据美国胸科协会和危重病医学会倡议将ＭＯＦ更名为ＭＯＤＳ。ＳＩＲＳ和ＭＯＤＳ进展至器官功能衰竭前存在器官功能受损的表现，应予高度重视并早期干预。     

Thrombomodulin serum levels in ventilated preterm babies with respiratory distress syndrome

A soluble form of thrombomodulin (TM), an anticoagulant proteoglycan of the endothelial cell membrane, considered a marker of vascular endothelial damage, was measured in plasma of preterm infants with respiratory distress syndrome (RDS). In these patients, lung immaturity leads to endothelial leak of plasma proteins and to surfactant inhibition. In 18 babies with RDS, plasma TM concentration was significantly elevated compared with values of a matched group of babies without pulmonary disease (276.1 ng/ml vs 141.3 ng/ml) (P<0.05). Furthermore, TM levels of mechanical ventilated babies (IPPV) with severe RDS were higher than those of babies with moderate RDS and treated with nasal CPAP (340.9 ng/ml vs 174.2 ng/ml) (P<0.05). Conclusion These data show that TM can be used as marker of pulmonary endothelial damage in preterm babies treated with mechanical ventilation for RDS and suggest early intervention with exogenous surfactant to limit alveolar protein leakage and surfactant inactivation. Received: 20 February 1997 and in revised form: 7 July 1997 / Accepted: 8 July 1997     

Surgical Treatment of Aortic Root Aneurysm Related to Marfan Syndrome in Early Childhood

The prognosis of Marfan syndrome in both adult and pediatric patients is primarily related to the cardiovascular complications. In infantile Marfan syndrome, although involvement of the mitral valve is the most frequently encountered cardiovascular lesion, the aortic root can be more worrisome because of its excessive dilatation, leading to aortic insufficiency or dissection. If the role of elective surgery is relatively well defined for adult patients, it is still debated during childhood. We report two patients, aged 22 months and 5 years, each presenting an aortic root aneurysm related to Marfan syndrome, and each treated with the Bentall procedure without specific age-related mortality or morbidity. These two patients experienced normal growth and were free of any complication for a follow-up period of 8 and 2 years, respectively. More than an absolute value of the aortic root dimension, it is the conjunction of the rate of progression of the aortic root dilatation, the degree and the duration of the aortic valve regurgitation, and its resulting left ventricular dysfunction that must be taken into consideration in choosing the surgical option.     

Median nerve compression in Proteus syndrome

Proteus syndrome is a multi–organ disorder, a prime feature of which is localized gigantism, usually clinically obvious. Symptoms secondary to hypertrophy of nerves has not been previously recognized as a part of the syndrome. Accepted: 16 May 1997     

盆腔静脉瘀血症患者瘀血静脉超微结构观察

为探讨盆腔静脉瘀血症患者瘀血静脉形态学改变,本文对11例瘀血症患者和6例结扎术后无任何症状和体征的妇女盆腔静脉进行了扫描及透射电镜观察.发现瘀血静脉壁间渗出增多;内皮细胞数量增多、体积变小且外形肿胀;细胞表面粗糙,突起增粗,小孔及凹陷增多、变大;细胞质致密并含大量饮液泡,线粒体肿胀而数量增多;染色质呈块状分布于核的周边;内皮细胞间嵌合连接和紧密连接正常存在.这些变化说明缺氧是引起本症盆腔静脉超微结构改变的主要原因,提示本症是一种功能性疾病.     

妊高征患者合体滋养细胞质膜部分生化指标的变化

目的：研究妊高征（ＰＩＨＳ）患者胎盘合体滋养细胞质膜（ＳＴＣＰＭ）中生化指标的变化及原因。方法：ＰＩＨＳ２４例，其中轻度８例、中度７例、重度９例，以正常孕妇１８例作对照，用Ｗｈｉｔｓｅｔ等法制备ＳＴＣＰＭ，以孔雀石绿无机磷法（ＭＧＩｏＰＡ）测定ＡＴＰａｓｅ的活性，用ＤＰＨ荧光探针测定膜的微粘度来推断膜的流动性，用硫代巴比妥酸荧光微量测定法测丙二醛酸（ＭＤＡ）以代表脂质过氧化物（ＬＰＯ）。结果：ＰＩＨＳ组Ｎａ＋、Ｋ＋－ＡＴＰａｓｅ、Ｃａ２＋、Ｍｇ２＋－ＡＴＰａｓｅ活性均显著低于对照组（Ｐ＜０．０５），且随病情加重而降低，膜的流动性亦显著降低（Ｐ＜０．０５），ＭＤＡ则显著增高，且随病情加重而增高；ＭＤＡ与两种ＡＴＰ酸呈负相关关系（Ｐ＜０．０５）。结论：ＰＩＨＳ的ＳＴＣＰＭ生化指标发生变化，其变化的原因之一是脂质过氧化作用增强     

Infantile hemangioendothelioma of the thymus with massive pleural effusion and Kasabach-Merritt syndrome: Histopathological, flow cytometrical analysis of the tumor

Infantile hemangioendothelioma of the thymus is a rare disease. We describe a patient who developed a large anterior mediastinal mass, severe thrombocytopenia and massive pleural effusion at 1 month of age. Glucocorticosteroid and irradiation therapy had no effect on either the tumor size or clinical symptoms and the tumor was resected subtotally. Three months after the subtotal resection, the remaining tumor had almost disappeared and the symptoms had resolved. The patient has now been well for 1 year after surgery without evidence of recurrence. The tumor tissue was characterized by prominent vascular endothelial proliferation intermixed with a normal thymic structure, producing a picture consistent with that of an infantile hemangioendothelioma in the thymus, lmmunohistochemically, the tumor cells showed positive staining for vimentin, factor VIII and CD34. The DNA stemline and proliferative activity were examined by flow cytometry, which revealed a diploid stemline with a low growth fraction. DNA content and cell cycle analyses of the tumor tissue may be useful for predicting the biological behavior of the tumor.     

An infant with Costelio syndrome complicated with fatal hypertrophic obstructive cardiomyopathy

We report a 3-month-old girl with Costelio syndrome complicating fatal hypertrophic obstructive cardio-myopathy. She had typical findings of this syndrome, slight dyspnea and persistent wheezing. Doppler echocardiography revealed asymmetric septal hypertrophy and systolic anterior movement of the anterior mitral leaflet. There was grade 1 mitral regurgitation. Although once her heart failure had been controlled medically, she died suddenly following deterioration of her heart condition. Costelio syndrome can complicate fatal hypertrophic obstructive cardiomyopathy.     

Hypoglycémie hyperinsulinémique persistante du nouveau-né et du nourrisson

Persistent hyperinsulinemic hypoglycaemia of infancy (PHHI) is the most frequent cause of hypoglycaemia in infancy. Clinical presentation is heterogeneous, with variable onset of hypoglycaemia and response to diazoxide, and presence of sporadic or familial forms. Underlying histopathological lesions can be focal or diffuse. Focal lesions are characterised by focal hyperplasia of pancreatic islet-like cells, whereas diffuse lesions implicate the whole pancreas. The distinction between the two forms is important because surgical treatment and genetic counselling are radically different. Focal lesions correspond to somatic defects which are totally cured by limited pancreatic resection, whereas diffuse lesions require a subtotal pancreatectomy exposing to high risk of diabetes mellitus. Diffuse lesions are due to functional abnormalities involving several genes and different transmission forms. Recessively inherited PHHI have been attributed to homozygote mutations for the beta-cell sulfonylurea receptor (SUR1) or the inward-rectifying potassium-channel (Kir6.2) genes. Dominantly inherited PHHI can implicate the glucokinase gene, particularly when PHHI is associated with diabetes, the glutamate dehydrogenase gene when hyperammonaemia is associated, or another locus.     

青州市小儿格林—巴利综合征流行病学特点与临床分析

目的探讨小儿格林-巴利综合征（GBS）病因、发病机制、防治措施。方法对青州市242例GBS患儿进行回顾性流行病学调查、临床观察、免疫学检测及微量元素检测。结果242例GBS患儿52．81％发病年龄为3～6a，75．61％发病时间在7～9月份，98．7％来自农村，年发病数3～40例，52．17％血清抗神经抗体阳性，平均血清锌含量9．12±0．56μmol／L。补锌治疗可促进康复。结论青州市小儿GBS发病年龄、季节、区域、年发病数有明显差异，患儿存在免疫紊乱及微量元素锌缺乏。