小儿先天性心脏病手术中残余机血洗涤回输对凝血功能的影响

目的了解小儿先天性心脏病手术中残余机血洗涤回输对凝血功能的影响。方法选择2011年11月至2012年5月作者收治的63例先天性心脏病体外循环手术患儿,对其残余机血洗涤回输,输血前后进行血栓弹力图（thromboela-stogram,TEG）检测。结果、所有患儿残余机血回输前血红蛋白（Hb）较转流前显著减少（P〈0．01）,回输后显著提高（P〈0．01）。纤维蛋白原（Fib）在残余机血输注前后均较转流前明显下降（P〈0．01）,输注后有所上升（P〈0．05）。经肝素酶杯与高岭土杯血栓弹力图（thromboela-stogram,TEG）检查,患儿回输洗涤残余机血前后各有3例存在肝素残留问题,2例回输前无肝素残留,输残余机血后出现肝素残留,残留率为4．8％。与回输前相比,输入后K值、α-Angle、MA值、CI值均无显著差异。结论小儿先天性心脏病术中残余机血洗涤回输对凝血功能无明显影响,并可以明显提高术后血红蛋白水平,是血液保护有效、简便的手段。     

小儿体外循环术中血浆游离血红蛋白的检测

目的：探讨机器剩余血(机余血)回输安全性指征,以有效利用机余血,减少体外循环手术用血量。方法：对机余血回输组43例、对照组43例、紫绀患儿7例采用邻甲联苯胺 过氧化氢法测定围术期不同时间点：①术前,②预充机血,③转流3 min,④转流30 min,⑤转流60 min,⑥转流90 min,⑦术后3 h,⑧术后24 h血的血浆游离血红蛋白(PF-Hb)。结果：非紫绀患儿PF-Hb随体外循环时间延长而增高,最高达 200 mg/L,机余血回输后24 h恢复正常。紫绀患儿也表现相同的趋势,只是相应值均较高,其PF-Hb在术后24 h也基本恢复正常(86.5 mg/L)。机余血回输后25例肾功能检查均正常,与凝血有关的血液有形成分术后3 h即恢复到正常水平。机余血回输组手术用血量为(400±20) ml,与对照组用血量(800±50) ml相比,差异有显著性(P<0.01)。结论：在小儿体外循环中,实行机余血回输可以有效地减少库血用量。对游离血红蛋白的监测,可以较好地反映出红细胞的破坏程度,并可作为是否将机余血回输参考指征之一。紫绀患儿自身红细胞在氧合情况下较易受到破坏,机余血回输应谨慎。     

换血疗法治疗重症新生儿溶血病

我们自１９９７年５月～１９９８年５月经脐静脉插管对重症新生儿溶血病进行换血治疗，疗效满意，报告如下。资料与方法一、一般资料　１５例重症血型不合溶血病病人，均符合换血指征［１］。男７例，女８例；换血前血总胆红素为４３９．４６±１１２．８７μｍｏｌ／Ｌ；ＡＢＯ溶血病９例，Ｒｈ溶血病６例。换血时间：生后２４ｈ内４例，７２ｈ内３例，６ｄ内６例，８ｄ内２例。二、方法　血源选择：选择Ｒｈ阳性重组血１２例（ＡＢＯ溶血病９例，Ｒｈ溶血病３例），选择Ｒｈ阴性的ＡＢＯ同型血或重组血３例；换血速度为３ｍｌ／（ｋｇ·ｍ…     

急性中枢性尿崩症临床经过和治疗评估

目的 调查分析急性中枢性尿崩症的临床过程和疗效。方法 回顾性总结１９９７年５月～１９９９年１２月我院ＰＩＣＵ的１６例急性中枢性尿崩症患儿临床资料。结果 所有病例均继发于脑损伤和应用垂体加压素治疗，其剂量为０．０００３～０．０００６５Ｕ／（ｋｇ．ｍｉｎ），治疗时间为５小时～１８天，平均２８．５小时。治疗目标达到：尿量２～３ｍｌ（ｋｇ．ｈ），尿比重１．０１０～１．０２０，血钠１４０～１４５ｍｍｏｌ／Ｌ     

自体输血在小儿心脏外科的应用

自体输血在小儿心脏外科的应用上海医科大学儿科医院心血管中心（２０００３２）张善通先天性心脏病是儿科常见病，多数心血管畸形均需在体外循环心内直视手术矫治。早期一般１例心内直视手术，在术中及术后需要血量为４００～６００ｍｌ，需２～３个献血员供血。因库血储...     

超低出生体重儿抢救成功1例

患儿 ,女 ,出生体重仅 6 0 0 ｇ。生后 1ｍｉｎ阿氏评分 5分。立即吸净口、鼻分泌物后吸氧 ,转入ＮＩＣＵ抢救。主要抢救措施 :1.保温 ,头罩吸氧 (3Ｌ/ｍｉｎ)。 2 .生后 4ｈ开始输液 ,ｄ16 0ｍｌ/ (ｋｇ·ｄ) ,后按 10～ 2 0ｍｌ/ (ｋｇ·ｄ)渐增加 ,直至达 2 0 0ｍｌ/ (ｋｇ·ｄ)。生后 6 6、90ｈ分别输 2 0 %脂肪乳、6 .9%复方氨基酸溶液。 3.生后 18ｈ开始肠道喂养 ,采用低出生体重儿配方乳。经鼻十二指肠喂养。生后 2 8ｄ将胃管退到胃内。生后 4 5ｄ自行吸乳 ,停止静脉营养。 4 .生后 4 8ｈ开始出现呼吸暂停 ,给予静滴纳络酮或氨茶碱。…     

心脏直视手术的体外循环管理

本文对１５８例小儿心脏直视手术的体外循环管理进行了总结，其特点为：１．预充量限制在８００～１２００ｍｌ；２．提高预充液的胶体渗透压，晶胶比为０．５～０．７；３．维持血球压积２５％～３０％；４．高流量灌注１００～１６０ｍｌ／（ｋｇ．ｍｉｎ），灌注压５．３～９．３ｋＰａ；５．加强心肌保护。     

哮喘患儿口服长效缓释茶碱代谢动力学及用药方案探讨

研究哮喘患儿口服长效缓释茶碱８．８ｍｇ／ｋｇ的代谢动力学特点及合理用药方案。代谢动力学参数：达峰时（Ｔｍａｘ）为（５．９１±０．３２）ｈ，峰浓度（Ｃｍａｘ）为（１０．４１±０．８８）ｍｇ／Ｌ，血药半衰期（Ｔ１／２）为（６．８４±１．３４）ｈ，总消除率（Ｔｃｌ）为（０．７８８±０．０９５）ｍｌ／（ｍｉｎ·ｋｇ）。比较了患儿相同日剂量而间隔１２小时及间隔８小时服药的经时血茶碱浓度。两种服药方案的血药峰浓度（Ｃｍａｘ）没有显著差异，而间隔８小时服药时的血药谷浓度（Ｃｍｉｎ）较间隔１２小时服药时明显升高，血药波动度明显下降，血茶碱浓度更为平稳。     

窒息新生儿血浆精氨酸加压素与内皮素变化

新生儿窒息缺氧常导致机体一系列的病理生理变化,围生期窒息时精氨酸加压素（ＡＶＰ）和内皮素（ＥＴ）合成和释放增多,且与窒息后器官损害有关［１］,本文测定５０例不同窒息新生儿血浆ＡＶＰ与ＥＴ浓度,并探讨血浆ＡＶＰ和ＥＴ与窒息程度的关系。对象与方法一、对象　均为生后２４ｈ内足月新生儿,胎龄３７～４２ｗｋ,轻度窒息组２１例,１ｍｉｎＡｐｇａｒ评分４～７分;重度窒息组２９例,１ｍｉｎＡｐｇａｒ评分≤３分;对照组２３例,１ｍｉｎＡｐ－ｇａｒ评分８～１０分,血气分析正常。二、方法　１．血浆ＡＶＰ和ＥＴ测定　入…     

婴儿泌尿系结石1例

患儿，男，３ｍｏ１０ｄ，因无尿６４ｈ伴抽搐１次入院。入院前有上呼吸道症状，无肉眼血尿及异常排尿情况，生后母乳喂养，未添加鱼肝油及钙剂，未接种过疫苗，其父有空洞性肺结核。体检：Ｔ３８．３℃，Ｗ４．８ｋｇ，呼吸稍促，急性病容，反应差，口唇发绀，双肺呼吸音清，心率１３２次／ｍｉｎ，节律齐，肝脾不大，神经系统阴性。血Ｈｂ１２．５ｇ／Ｌ，ＷＢＣ２１．６×１０９／Ｌ，Ｎ０．６６，Ｌ０．３０，Ｅ０．０２，Ｍ０．０１，ＢＴ３ｍｉｎ，ＣＴ４ｍｉｎ，血钾６．１ｍｍｏｌ／Ｌ，血钠１３６ｍｍｏｌ／Ｌ，血氯９８ｍｍｏｌ／…     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.