胆道闭锁治疗的新进展

当今世界上对胆道闭锁的治疗有两种方式：肝门肠吻合（Ｋａｓａｉ手术）或肝移植。自从１９５９年日本Ｋａｓａｉ对首例胆道闭锁行肝门空肠吻合术以来，经过近４０年来的临床实践确实有一部分患儿得以长期生存，其中不乏生活质量近乎正常儿童的患儿。也有一部分出现了各种晚期并发症。当肝移植技术日趋成熟，加之环孢霉素Ａ问世以后，胆道闭锁已成为小儿肝移植的主要对象。这两种治疗方法如何选择，多数学者认为肝门肠吻合和肝移植这两种治疗方法是相辅相成的，必须根据当地医疗条件、医疗技术水平以及患儿的具体情况来决定。一般认为①患儿…     

腹腔镜扩大肝门埋入空肠吻合术治疗上端开口过小胆总管囊肿的可行性和有效性探讨

目的通过动物实验和病例观察两方面的结果探讨腹腔镜扩大肝门埋入空肠吻合术预防吻合口狭窄的可行性和有效性。方法动物实验部分遵循相关的实验设计原则,构建巴马小型猪模型,并将其随机分为2组接受不同的干预方案。两组胆总管直径均6 mm。A组(n=12)行胆总管套入式吻合,模拟肝门包入空肠吻合术; B组(n=12)行胆总管空肠吻合,模拟经典肝管空肠吻合术;比较两组吻合口局部组织学特点。病例观察部分选取2012年1月至2018年1月于华中科技大学同济医学院附属协和医院小儿外科接受腹腔镜肝门扩大埋入空肠吻合术的肝门胆管狭窄胆总管囊肿患儿47例并进行随访,初步总结手术治疗经验。结果 A组实验动物术后无一例发生吻合口狭窄,B组中有5只小型猪出现狭窄; B组狭窄吻合口组织表现为炎性细胞浸润严重、粗大的胶原纤维排列紊乱以及遍布吻合口全层,促纤维化因子表达水平较A组显著增高(P 0. 05)。47例患儿腹腔镜肝门扩大埋入空肠吻合手术过程均顺利,手术时长(165±15) min,住院时间(9±2) d。随访(31±18)个月,无一例并发胆管炎、胆管结石及吻合口狭窄。结论对于肝门胆管直径6 mm的胆总管囊肿,腹腔镜肝门扩大埋入空肠吻合术能有效地预防术后吻合口狭窄的发生。     

先天性胆总管囊肿囊肿切除空肠间置与肝总管空肠吻合的远期随访与评价

目的 研究先天性胆这囊肿囊肿切除空肠间置与肝总管空肠Ｒｏｕｘ－Ｙ吻合重建胆管的远期随访结果与疗效评价。方法 对２种常用的胆管重建术：空肠间置术（２０例）和肝总管空肠Ｒｏｕｘ－Ｙ吻合术（３０例）进行３￣１０年随访。结果 获得随访４４例,其中短段空肠间置伴抗掺流瓣术１４例,肝总管空肠Ｒｏｕｘ－Ｙ吻合短段桥袢伴抗反流瓣术１８例均无反流性胆管炎,钡餐检查剂反流,而短段空肠间置不伴抗反流瓣４例中１例,肝总管     

I型胆管闭锁的外科治疗

目的探讨I型胆管闭锁的外科治疗以及临床意义。方法2003—2011年,作者收治胆道闭锁患儿98例,其中伴有胆总管闭锁的I型胆管闭锁患儿5例,男3例,女2例,年龄62～127d。行胆囊-空肠吻合术2例,肝管-空肠吻合术3例。结果2例胆囊-空肠吻合术患儿退黄时间分别为术后10d和术后17d（退黄标准为总胆红素〈20μmol／L）;3例肝管-空肠吻合术患儿退黄时间分别为术后20d、1个月和2个月（退黄标准同前）。术后随访时间1—5年;2例胆囊-空肠吻合术患儿术后未见黄疸反复。3例肝管-空肠胆道重建手术患儿中,1例术后未见黄疸反复,1例术后5个月出现黄疸,诊断为胆管炎,经抗炎治疗后好转;1例反复发作胆管炎最终选择肝移植。结论术中胆道造影是诊断胆道闭锁的金标准;如果术中造影证实为胆总管闭锁,且胆囊与左、右肝管通畅,主张采取胆囊-空肠吻合术。肝管-空肠吻合容易造成吻合口狭窄;过度解剖肝门对于术后恢复不利。     

几种胆道重建术治疗先天性胆总管囊肿的疗效评价

目的　评价先天性胆总管囊肿 (CCC)几种胆道重建术疗效。方法　CCC 38例均行囊肿切除 ,其中行肝总管十二指肠吻合术 6例 ,肝总管空肠Roux en Y吻合术 2 0例 ,空肠间置肝总管十二肠吻合术 9例 ,阑尾间置肝总管十二肠吻合术 3例。术后随访 6个月～ 6年。结果　获得随访 34例 ,其中肝总管十二指肠吻合术 5例 ,并胆管炎、钡餐造影钡剂反流各 2例 ;肝总管空肠Roux en Y吻合术 19例 ,钡餐造影钡剂反流 1例 ;空肠间置肝总管十二指肠吻合术 8例 ,吻合口狭窄伴肝内胆管扩张、胆瘘各 1例 ;阑尾代胆道 2例 ,并胆管炎 1例。结论　CCC术后远期疗效与术式选择、抗反流机制的建立密切相关 ,肝总管空肠Roux en Y吻合术伴抗反流术式 ,是囊肿切除术后重建胆道的理想术式     

先天性胆总管囊肿手术治疗的探讨

先天性胆总管囊肿目前公认的首选术式是囊肿切除、肝管空肠Ｒｏｕｘ－Ｙ吻合术,但有少数患儿术后经常发生腹痛、胆管炎等并发症。１９８０年１月～１９９７年７月,我们行胆总管囊肿切除术３０例,其中囊肿切除后行肝管切开肝肠高位大口吻合术１０例,由于注重解除了肝门...     

胆总管囊肿切除后胆道并发症

胆总管囊肿切除后胆道并发症报告９７例胆总管囊肿切除，Ⅰ型４５例，Ⅳ型５２例，囊肿无恶变，年龄２７天至３７天。早期２２例中行总肝管远端－十二指肠吻合９例，Ｒｏｕｘ－Ｙ空肠吻合１３例，该组９例术后数年因吻合口狭窄、肝内胆管结石伴反复胆管炎而再手术。其后７...     

Budd—Chiari综合征10例

小儿Ｂｕｄｄ－Ｃｈｉａｒｉ综合征（ＢＣＳ）尚属少见，１９８８～１９９８年我院治疗小儿ＢＣＳ １０例，现报告如下。 资料与方法 一、一般资料本组１０例中，男女各５例，年龄２．５～１５ ａ，平均年龄 ９．７ ａ。病程２个月～６ ａ。其中肝大 １０例，伴轻度脾大、腹水有牌亢血象者，肝功能均正常，ＨＢｓＡｇ阳性１例，均经Ｂ超和Ｘ线造影检查确诊，排除肝前型门静脉高压。９例作上消化道钡餐检查，其中４例食道静脉曲张。均经下腔静脉造影和经皮肝穿刺肝静脉造影。显示下腔静脉膜状阻塞（ＭＯＶＣ）、下腔静脉（ＩＶＣ）中度狭窄者…     

先天性肠闭锁40例临床分析

目的总结新生儿肠闭锁的诊断和治疗。方法对40例先天性肠闭锁的患儿进行回顾性分析，其中十二指肠闭锁8例，空肠闭锁8例，回肠闭锁24例；Ⅰ型15例，Ⅱ型6例，Ⅲa型17例．nlb型（Apple—peel）1例，Ⅳ型1例；行隔膜切除纵切横缝术5例，行肠侧侧菱形吻合术6例，行肠切除端端或端斜吻合术28例，肠管裁剪端端吻合（Tapering整形术）1例。结果术后吻合口梗阻5例．吻合口瘘3例．切口裂开2例。术后死亡3例，弃医1例。治愈36例，治愈率90％。结论早期诊断和选择合理的术式是提高肠闭锁治愈率，减少并发症的关键因素，静脉营养的应用促进肠功能的恢复。     

45例腹腔镜辅助胆总管囊肿根治术

目的本研究对腹腔镜先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术进行探讨。方法从2001年6月至2003年9月，共收治先天性胆总管囊肿患儿45例，年龄2个月到12岩。其中42例为囊肿型，平均囊肿直径3.8cm（2-18cm）；其余3例为梭形，直径分别为1．5、2．0和2．2cm。本组45例患儿均经腹腔镜行先天性胆总管囊肿切除，肝管空肠Roux-Y吻合术。结果本组45例患儿手术全部成功，手术时间平均为4.3h（3．5～7．6h），术中出血量约5～10ml。本组8例患儿合并肝管狭窄，术中同时行腹腔镜胆总管囊肿切除及肝管成形；6例患儿合并共同管内蛋白栓，术中通过腹腔镜导入肠道或插管冲洗清除。其中1例患儿于术后第1d发生胆漏，术后第26d时自愈；其余44例患儿术后恢复顺利，住院时间3～6d。术后随访3～30个月，无肠粘连梗阻和吻合口狭窄等术后并发症发生。结论经腹腔镜先天性胆总管囊肿切除，肝管空肠吻合术是一种安全可靠的方法，术中胆道造影全面了解胆道的结构，清晰的肝门暴露，准确的囊肿分离和熟练的缝合技术是手术成功的关键。     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.     

Whole-body mineral measurements in Swedish adolescents at 17 years compared to 15 years of age

Aim: To provide reference data for bone mineral variables in 15- and 17-y-old adolescents and to analyse the relationships between these variables and measures of bone and body size, gender, puberty, growth, various lifestyle and environmental factors and socioeconomic background.

Methods: In the same 321 randomly selected adolescents (147 boys and 174 girls) living in two different regions of Sweden, the total bone mineral content (TBMC), bone area (BA) and total bone mineral density (TBMD) were assessed by dual-energy X-ray absorptiometry at ages 15 and 17 y. The effects of bone and body size, gender, growth, sexual maturity, physical activity, region of domicile, social conditions, food habits, smoking and alcohol intake on TBMC and TBMD were examined in multivariate analyses.

Results: In the 15-y-old adolescents, BA, height, gender, physical activity, maturity and weight explained 91% and 48%, of the variance in TBMC and TBMD, respectively. In similar analyses in the 17-y-olds, the corresponding figures were 92% and 62%, respectively, when BA, height, growth, physical activity, gender and region emerged as significant in the model. In all these analyses, BA explained most of the variance in TBMC and TBMD. No significant reduction of variance was found when different measures of social conditions, smoking, food habits, alcohol or dietary intakes of energy, calcium or vitamin D were included in the models. The reason why region of domicile had a significant impact on TBMC in the 17-y-olds is not known. The fact that the normal fluoride concentration in drinking water (1.1 mg/L) is 10 times higher in the region where TBMC was higher than in the other region is an interesting observation.

Conclusion: Almost 90% of the variance in TBMC and 50% of that in TBMD was explained by measures of bone and body size and only a few percent by gender, physical activity, Tanner stage, growth and region of domicile.     

VARICELLA ZOSTER VIRUS INFECTIONS IN THE PEDIATRIC STEM CELL TRANSPLANT PATIENT

Varicella zoster virus (VZV), a member of the human herpesvirus family, causes the clinical syndromes of chickenpox during primary infection and shingles on later reactivation. In immunocompromised patients, including those undergoing hematopoietic stem cell transplantation, VZV can produce life-threatening infections. The most serious forms of VZV infection involve hematogenous dissemination of the virus to vital organs, such as the lung, brain, and liver. Advances in immunoprophylaxis, antiviral chemotherapy, and vaccine development have provided effective tools to limit the morbidity and mortality previously associated with VZV infection in hematopoietic stem cell transplant patients. In this review, we discuss virologic aspects of VZV, pathogenesis of VZV infection, methods of viral diagnosis, clinical manifestations of infection in both normal and immunocompromised patients, and available preventative and therapeutic measures.     

The potential role of rapamycin in pediatric transplantation as observed from adult studies

Although pediatric patient and renal graft survival rates have shown marked improvements during the past decade, the persistent toxicities of immunosuppressive drugs and chronic allograft attrition remain major obstacles in transplant therapy. Results in adult patients suggest that complete steroid withdrawal is possible in the majority of recipients under treatment with a cyclosporin A-rapamycin (CsA RAPA) regimen. Furthermore, preliminary studies suggest that a marked reduction in the dose of CsA may be possible under the umbrella of RAPA coverage. The gain in immunosuppressive efficacy afforded by RAPA has not only been obtained without an increased morbidity owing to infectious or neoplastic causes, but also with the potential for reducing the incidence and/or progression of chronic rejection.     

Génétique des schizophrénies : mise en perspective des schizophrénies à début précoce et autres pathologies du développement

Schizophrenia (SCZ) is a severe brain disorder characterized by hallucinations, delusions, flat and/or inappropriate affect and cognitive impairment. The lifetime risk is about 0.5% with heritability of 65–85%. The prevalence of early-onset schizophrenia (defined here as before 15 years of age) has not been well studied, but is likely to be 5–10% of all cases. The rarity of early-onset SCZ has made it difficult to study. We focus on genetic studies of adults with schizophrenia, highlighting results for early-onset schizophrenia where available. Prior to the past 5 years, studies failed to find replicable association or linkage between SCZ and specific genes when appropriate statistical corrections for multiple testing were used. Many false positive results were probably reported using the candidate gene approach. Recently, the development of single nucleotide polymorphism (SNP) “chips” has permitted large genome-wide association study (GWAS) analyses that suggest that across all age groups, a proportion of genetic risk can be attributed to a large number of common SNP, each with a very small effect on risk (odds ratios of 1.1 or less). The greatest known genetic effect is conferred by the 1.5–3 Mb 22q.11.2 deletions, which occurs in ∼ 1/4000–1/6000 births with SCZ developing in 20–30% of carriers. Large SNP and aCGH microarray studies have now identified associations between SCZ and other rare, large copy number variations (CNV, insertions and deletions) with high odds ratios (5–10), including deletions of 1q21, 2p16.3 (neurexin-1 gene), 3q29 and 15q13.3, and duplications of 16p11.2. Some of these CNV are also associated with autism or other developmental disorders as well as epilepsy or intellectual deficiency, suggesting some overlap in the mechanisms that contribute to risks of these disorders. Based on preliminary data from larger-scale analyses in progress, approximately 1–2% of cases carry a CNV that has been clearly associated with SCZ (ORs 4–12). Whole exome and genome sequencing studies of large adult samples will be the next steps to identify rarer SCZ-associated mutations, including point mutations and smaller as well as rarer CNV. Genetic findings are beginning to contribute to an understanding of biological mechanisms of SCZ risk and may lead to new approaches to treatment.     

INACTIVATION OF PULMONARY SURFACTANT AND THE TREATMENT OF ACUTE LUNG INJURIES

Inactivation of pulmonary surfactant may be important in acute lung injury and acute respiratory distress syndrome. Treatment of surfactant dysfunction by instilling exogenous surfactants may improve gas exchange and pulmonary mechanics. Surfactants used for treatment vary in their attributes and effects, so when various surfactants are considered for therapy, resistance to inactivation is an important consideration. Animal models of acute lung injury exist in which the relative merits of surfactants can be compared. We hypothesize that the surfactants most resistant to inactivation in vitro will be the ones that are most effective in treatment of animal models of acute lung injury. Surfactants with higher concentrations of surfactant proteins (specifically A, B, and C) are more resistant to inactivation. Nonionic polymers mimic surfactant proteins in preventing surfactant inactivation under some conditions. Adding nonionic polymers to surfactant containing minimal amounts of SP-B and SP-C markedly improves lung function of animals with lung injury. Making surfactants more "inactivation-proof" may improve surfactant therapy of acute lung injuries.     

Plasma Proinsulin and C-Peptide Concentrations in Children with Hyperinsulinaemic Hypoglycaemia

ABSTRACT. Plasma concentrations of proinsulin and C-peptide were measured in five children presenting with svere hypoglycaemia associated with elevated plasma levels of immunoreactive insulin (IRI) in order to determine whether the profile of circulating B-cell products related to the underlying pathophysiology of the pancreas. Results were compared with data from 13 normal infants. Four children, three neonates and a nine year old girl, were subjected to partial or total pancreatectomy. The neonates had nesidioblastosis, nesidioblastosis with a microadenoma, and a functional abnormality without histological derangement respectively; the older child had a localised adenoma. The remaining child, a neonate, had transient hypoglycaemia and elevated IRI levels associated with hyperlactataemia and hyperalaninae-mia. All the children had markedly elevated plasma proinsulin concentrations; the highest levels were seen in the child with an isolated adenoma and in the neonate with nesidioblastosis and a microadenoma. Both of these children also had substantially elevated plasma C-peptide concentrations. The remaining three neonates had plasma C-peptide levels, which although in the normal range for normoglycaemia were inappropriately elevated during hypoglycaemia. It is concluded that elevated proinsulin and C-peptide concentrations are seen in children with hypoglycaemia associated with increased plasma IRI levels and that the profile of the concentrations does not provide a reliable marker for the nature of the underlying pancreatic abnormality.     

Growth tracks in early childhood

Aim: Child growth is modulated by numerous factors and, particularly in infancy and early childhood, often tends to follow apparently irregular patterns, with many centiles crossed before the later growth channels are reached. The aim of this study was to visualize the diversity of individual growth. Design: The study investigated 333 girls and 329 boys without chronic illnesses from four paediatric practices in Kiel, Germany. The children were measured on natural     

Residual pulmonary hypertension in children after treatment with inhaled nitric oxide: a follow-up study regarding cardiopulmonary and neurological symptoms

Inhaled nitric oxide is a potent vasodilator in acute severe pulmonary hypertension and is increasingly used as rescue treatment in intensive care algorithms aiming at reducing severe hypoxaemia in neonates and children. Although the immediate effects may seem impressive, longterm outcome regarding residual pulmonary hypertension and other sequelae has been studied in only a very few patients. The aim of the present study was to evaluate residual pulmonary hypertension, cardiopulmonary or neurological symptoms in children after treatment with inhaled nitric oxide in severely hypoxaemic and/or pulmonary hypertensive mechanically ventilated children. The study was performed in four paediatric intensive care units in university hospitals in Sweden, Norway and Australia. Patients who had received inhaled nitric oxide as part of their intensive care treatment for severe hypoxaemia and/or pulmonary hypertension, and in whom 6 mo had elapsed since treatment, were included for evaluation. Thus 36 paediatric or neonatal patients were examined for circulatory, respiratory or neurological disorders with clinical examination, echocardiography, chest X-ray and a capillary blood sample. Four patients with congenital heart disease had residual pulmonary hypertension. Nine patients were receiving bronchodilators. Sixteen patients had minor (n = 15) or moderate (n = 1) changes on a chest X-ray. One patient had a possible delay in psychomotor development. Conclusions: In spite of the severity of their primary illness, we found that the overwhelming majority of the surviving children were asymptomatic and doing well. The few residual circulatory and respiratory symptoms could be related to the initial condition.