腹腔镜联合尿道镜微创保胆取石术治疗小儿胆囊结石

目的探讨腹腔镜联合尿道镜微创保胆取石术治疗小儿胆囊结石的方法及临床应用价值。方法回顾性分析本院2009年8月至2013年8月采用腹腔镜联合尿道镜微创保胆取石术治疗的20例胆囊结石患儿临床资料,男11例,女9例,年龄1岁1月至15岁,胆囊多发并泥沙样结石13例,单发结石3例,遗传性球形红细胞增多症并胆囊结石4例。结果 20例均顺利完成手术,手术时间30~90 min,出血量5~20 mL,无术中输血。4例遗传性球形红细胞增多症并胆囊结石患儿联合行腹腔镜下脾切除术。术后患儿临床症状基本消失,单纯胆囊结石患儿术后3 d复查腹部彩超见结石无残留。随访6个月至4年,17例未发现胆囊结石复发;1例失访;1例术前大量泥沙样结石术后彩超可见少量结石残留,约0.1 cm大小,无临床症状;1例术前提示胆囊萎缩,胆囊多发结石合并胆囊炎,术后1周患儿仍腹痛,彩超发现胆囊少量泥沙样结石,予胆囊造瘘术,术后1个月拔除造瘘管,随访半年未见结石复发。结论腹腔镜联合尿道镜保胆取石术治疗小儿胆囊结石可保留胆囊功能,操作简单,术后恢复快、并发症少,复发率低,是治疗小儿胆囊结石的可行性方法。     

小儿慢性胰腺炎12例

目的总结小儿慢性胰腺炎的临床经验,探讨其诊断与手术治疗方法。方法回顾性分析12例慢性胰腺炎患儿的临床资料,其中男性9例,女性3例。平均年龄11.4岁,病程17.1个月。均因反复发作上腹痛入院。B超、CT或MRCP显示存在胰腺钙化2例,胰管扩张4例,胰管结石4例,胰腺假性囊肿5例。6例1期患儿予保守治疗;6例2期患儿予手术治疗,其中胰管切开取石、胰管空肠侧侧吻合术3例,胰体尾、脾切除术2例,腹腔引流术1例。结果 6例保守治疗患儿症状完全缓解后出院。6例手术治疗患儿中,除1例胰头部结石嵌顿未能取尽外,其余均恢复良好。12例患儿均获得6个月至5年随访,平均随访时间2.4年,其中3例复发腹痛,其余无临床症状。结论小儿慢性胰腺炎多依靠影像学检查确诊。合并胰管结石者首选胰管切开取石、大口径胰管空肠吻合术,可有效缓解疼痛,创伤小,并发症少。     

小儿腹腔镜下胆囊切除术11例

目的总结小儿腹腔镜下胆囊切除术(Laparoscopic Cholecystectomy,LC)的临床经验。方法2002年12月～2007年5月,我院对10例胆囊结石、1例胆囊隆起性病变患儿施行腹腔镜下胆囊切除术。结果11例均顺利切除胆囊,无1例中转开腹,其中1例同期切除病变阑尾。平均手术时间为34min(15～65min)。剖开胆囊后见结石2～18枚,结石直径2～11mm,9例为胆固醇结石,1例为胆色素结石。病理检查证实为急、慢性胆囊炎10例,胆囊胆固醇沉积症合并急性化脓性阑尾炎1例。术后无1例出血及胆漏,全组住院时间2～7d,均痊愈出院。随访1个月～4.5年,1例于术后半年出现轻度腹泻,均无胆管损伤性狭窄及胆囊管、胆总管残余结石或复发,患儿生长发育良好。结论选择性B超检查有助于减少小儿胆囊结石的误诊,腹腔镜下胆囊切除术治疗胆囊结石快捷、安全、有效。     

小儿胆石症的微创治疗

近年来,随着膳食结构与习惯的改变,胆囊炎胆石症发病年龄明显提前,加之超声检查的进步与普及,过去认为少见的小儿胆囊炎胆石症发病率呈明显上升趋势,但是否均需手术干预目前存在分歧,有不少病例虽术前诊断胆囊结石,但切除的胆囊内无结石,儿童期胆囊结石是否均需切除胆囊值得商讨.不少病例经对症处理症状消失,手术应慎重选择.我科2006年10月至2011年7月共收治25例胆囊结石患儿,现总结报告如下.     

三聚氰胺泌尿系结石并急性肾衰竭婴幼儿的预后与转归

目的探讨三聚氰胺泌尿系结石(MUS)并急性肾衰竭(ARF)婴幼儿的预后与转归。方法对2008年7-10月在本院住院治疗的21例MUS并ARF婴幼儿[ARF组,年龄(10.5±5.7)个月]进行20个月随访。随访内容包括泌尿系超声、尿常规、肾功能检查,并记录其身高、体质量、临床症状及并发症情况。选取同期住院治疗的30例单纯MUS无ARF患儿(MUS组)及50例无三聚氰胺污染奶粉喂养史的健康儿童(健康对照组)作为对照。比较三组间相关参数异同。结果成功随访17例MUS并ARF患儿;经过保守治疗(其中12例患儿行血液透析),患儿恢复顺利,无临床症状及相关并发症,尿常规和肾功能检查未见明显异常。12例带结石出院患儿中结石消失7例、变小4例、无明显变化1例。8例出院时仍有肾积水的患儿中,5例消失;2例减轻,1例无明显变化。ARF组患儿出院带石率、结石消失率、出院积水率及积水消失率与MUS组比较差异均无统计学意义(Pa>0.05);AFR组和MUS组患儿及健康对照组儿童身高及体质量比较差异均无统计学意义(Pa>0.05)。结论 MUS并AFR患儿经过住院保守治疗(包括血液透析),ARF得以纠正,症状缓解,20个月随访显示预后与转归较好,生长发育无明显影响,未见泌尿系肿瘤形成。     

小儿胃扭转的诊治分析

目的探讨小儿胃扭转的诊断、鉴别诊断及治疗方法,以提高临床医生对该病的认识。方法回顾性分析本院2002年2月至2012年6月收治的11例胃扭转患儿临床资料,总结其诊断、鉴别诊断及治疗方法。结果11例患儿中,10例采用手术治疗,1例采用保守治疗。1例手术后死亡,1例术后2个月出现胃食管反流,予对症处理。10例获痊愈,随访无一例复发或发生并发症,患儿均生长发育良好。结论小儿胃扭转的诊断主要依靠临床症状,结合上消化道造影,可排除可能的其他疾病。治疗上建议早期手术,避免保守治疗后复发急症而威胁生命。     

儿童姿势性蛋白尿预后的远期随访和治疗分析

目的　探讨姿势性蛋白尿的远期预后、转归及治疗效果。方法　对 116例本病患儿尿蛋白的自然消失时间、转归、治疗效果进行了 5～ 18年的随访。对症状持续存在达 5年以上者进行肾活检检查 ,并分别给予泼尼松 ,泼尼松与环磷酰胺 (CTX)联合治疗。结果　 116例患儿中伴持续性蛋白尿者 17例 (肾功能减退 3例 ) ,间歇性蛋白尿伴发作性血尿 6例。对 2 1例症状未消失者肾活检结果示 :微小病变性肾炎 3例 ,系膜增生性肾炎 3例 ,局灶硬化 1例 ,无明显异常 14例 ;此 2 1例给予药物治疗后随访 2～ 5年 ,症状消失无复发 5例 ,偶有发作 4例 ,尿蛋白较前减少 3例。结论　本病大多数预后良好 ,症状消失数年无复发。但仍有 19 8%患儿发展为持续性蛋白尿、血尿及肾功能损害 ,对此应给予治疗     

14例小儿乙状结肠冗长症手术治疗分析

目的总结小儿乙状结肠冗长症的诊断、治疗、疗效及短期随访经验。方法2005年至2012年我们收治17例乙状结肠冗长症患儿,其中经手术治疗14例,保守治疗3例。回顾性分析14例手术治疗患儿临床资料。结果14例手术治疗患儿中,13例行经肛门结肠拖出术,1例行经腹Soave术。术后随访8例,最短半年,最长4年,均无吻合口瘘、吻合口狭窄及便秘等并发症。3例行药物、灌肠、饮食疗法等保守治疗6个月,症状好转,2~3 d排便一次。结论小儿乙状结肠冗长症的诊断主要依据临床表现及钡灌肠检查,确诊后应积极临床治疗。首选保守治疗,但症状改善缓慢,保守治疗无效或效果不满意者宜转手术治疗,手术首选经肛门结肠拖出术,损伤小,恢复快,无瘢痕,疗效明显。     

小儿消化道钡剂检查160例临床分析

目的：探讨X线钡剂检查对小儿消化道疾病诊断和治疗的作用。方法：采用回顾性分析方法对160例有消化道症状且经X线钡剂检查确诊之患儿的临床表现、检查结果及部分患儿转归进行总结。结果 160例经X线钡剂检查发现阳性征象75例。其中慢性胃炎21例;胃、十二指肠溃疡16例;食管炎9例;先天性肥大性幽门狭窄8例;贲门失弛缓症8例;食道静脉曲张5例;食管烧伤和瘢痕狭窄3例;先天性巨结肠3例;结肠息肉2例。32例腹痛、呕吐患儿钡剂检查无异常发现,未做任何治疗仅经钡剂检查后症状全部消失。随访1～2年无复发。 结论 小儿消化道疾病X线钡剂检查阳性率 47%;钡剂检查对部分无器质性病变但有消化道症状的患儿有治疗作用,其机制有待探讨。［中国当代儿科杂志,2004, 6(4): 315-316］     

小儿原发性胆管结石27例

小儿原发性胆管结石较少见，其疗效不尽满意。本院１９７５～１９９５年收治该病２７例，占同期治疗胆石症病例的０．７７％，报告如下。临床资料：男１３例，女１４例；年龄２～１５岁，平均１２．２岁；病程４天～１２年，平均４．６年。临床症状，腹痛２６例，黄疸１９例，胆汁性肝硬化４例，有肠道及胆追蛔虫史分别为２７例及２５例，胆退手术史１例，无溶血性疾病史。Ｂ超或手术发现结石分布在肝内胆管２０例（合并肝外胆管及胆囊结石分别为１０例及２例），其中左肝６例，右肝８例，左右肝６例；弥漫型（结石几乎充满整个肝内胆管系统…     

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.     

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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     

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.     

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.     

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.     

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.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.