前矢状入路直肠肛门成形术治疗女婴无肛前庭瘘的疗效观察

目的评价前矢状入路直肠肛门成形术治疗女婴无肛前庭瘘的疗效。方法自会阴前矢状入路游离瘘口,游离直肠侧壁及直肠后壁,保留直肠末端瘘口不受损伤,保证瘘口内括约肌结构完整,使直肠末端置于外括约肌中心无张力重建会阴体,恢复女童正常会阴外观。结果本组86例一期手术获成功,经近远期随访,会阴体外观及肛门功能均达到满意的效果。结论前矢状入路直肠肛门成形术治疗女婴无肛前庭瘘,直视下重建肛门直肠,完整保留了直肠瘘口及直肠盲袋,获得了满意的排便功能。     

前会阴入路手术在女孩肛门直肠疾病中的应用

目的　讨论及评估前会阴入路治疗先天性无肛前庭瘘、后天性直肠前庭瘘及会阴Ⅲ°裂伤的手术方法及效果。方法　对 2 14例前会阴入路手术的临床资料进行总结分析。其中先天性无肛前庭瘘组 4 1例 ,后天性直肠前庭瘘组 16 6例 ,会阴Ⅲ°裂伤组 7例。前会阴入路先天性无肛前庭瘘手术方法 :完整游离瘘口及充分游离直肠 ,在电刺激仪引导下 ,将游离之直肠置于横纹肌复合体中心 ;利用瘘口和两侧的耻尾肌分别成形肛门和重建会阴体。后天性直肠前庭瘘及会阴Ⅲ°裂伤前会阴入路手术仅需游离直肠前壁及两侧壁 ,前者切除瘘管和在无张力情况下修补直肠前壁缺损 ;后者在电刺激仪引导下确定外括约肌断端并原位修复外括约肌。结果　先天性无肛前庭瘘组 4 1例及会阴裂伤组 7例患儿会阴部切口均Ⅰ期愈合。术后 3个月及半年常规复诊 ,患儿会阴及肛门外观正常 ;采用肛门功能临床评分标准评估其排便功能 ,两组总评分均为优。后天性直肠前庭瘘组 16 6例 ,术后痊愈15 6例 (94 % ) ;瘘管复发患儿经 3%硼酸液坐浴后 ,6 0 %瘘口自行愈合。结论　前会阴入路手术治疗先天性无肛前庭瘘、后天性直肠前庭瘘及会阴Ⅲ°裂伤是一种合理、可靠的手术方法 ,效果满意。     

女婴无肛前庭瘘手术方法探讨

目的探讨女婴无肛并前庭瘘的手术治疗方法。方法2005—2011年作者收治年龄4个月至1岁的先天性肛门闭锁并直肠前庭瘘患儿21例,均通过保留瘘管肛门成形术治疗,分析其临床疗效及排便功能。结果一期愈合19例,2例出现术后切口感染,经应用抗生素及局部理疗后痊愈。21例均获随访,时间6个月至2年,全部患儿排便功能良好,会阴外观正常,无一例复发。结论保留瘘管肛门成形术治疗女婴无肛前庭瘘简便易行,是一种合理、可靠的手术方法。     

前矢状入路直肠肛门成形术治疗女婴无肛并前庭瘘20例

目的探讨女婴无肛并前庭瘘的手术方式。方法对2000~2004年入院的20例先天性肛门闭锁并直肠舟状窝瘘患儿均采用前矢状入路直肠肛门成形术(ASARP)治疗,对手术后效果及排便功能进行分析。结果18例切口一期愈合;2例术后切口感染,于12周后再次手术,治愈;20例患儿术后随访6~12个月,肛门排便临床评分均为优。结论ASARP可作为先天性肛门闭锁并直肠舟状窝瘘的首选手术方法。     

无肛术后残留直肠尿道瘘手术方法的选择和治疗效果

目的 总结治疗无肛术后残留直肠尿道瘘手术方法 的选择和临床效果.方法 对28例无肛术后残留直肠尿道瘘,按肛门外观和功能不同,采取两种手术方法 :肛门外观和功能良好的22例采用肛门前会阴矢状入路直肠尿道瘘修补、肛门成形术,肛门外观和功能较差(直肠回复严重5例及肛门开口前移1例)的6例采用后矢状入路直肠尿道瘘修补、肛门成形术,修补直肠尿道瘘,同时肛门成形.结果 无肛术后残留直肠尿道瘘存在明显的瘘管样结构,管长约(0.8±0.3)cm,瘘管的尿道开口大都位于尿道膜部.27例尿道瘘修补Ⅰ期愈合.25例获得随访,尿道无狭窄,无憩室;肛门功能临床评分21例优,4例良,与术前比较意义有差异统计学意义(P＜0.05).两种手术的手术时间分别为(72.8±11.2)min, (105.6±14.6)min(P＜0.05). 结论 通过肛门前会阴矢状入路和后矢状入路修补无肛术后残留的直肠尿道瘘,成功率高,肛门功能明显提高.肛门前会阴矢状入路尿道瘘修补术术野清楚,操作方便.     

肛门正常的直肠前庭瘘病因及治疗方法多中心回顾性分析

目的评估肛门正常的直肠前庭瘘(RVFNA)的治疗方法,探讨RVFNA的发病原因。方法回顾性分析2006年1月-2012年1月收治的206例RVFNA患儿的临床资料。年龄3个月～15岁。其中23例患儿曾于外院接受手术治疗失败。181例患儿出生3个月内有明确会阴部感染史,之后在排气或排稀便时前庭部有气体或少量粪便漏出。77例患儿会阴部感染之前曾有腹泻。198例前庭有1个瘘口,8例具有2个瘘口,瘘口之间有皮桥相连。患儿内口均在齿状线以上。173例外口直径<5 mm。本组102例行经肛门直肠前庭瘘修补术,87例行经会阴直肠前庭瘘修补术,17例行会阴成形术,均未出现术后会阴体开裂。结果 29例术后4～10 d前庭瘘复发,其中12例通过每日3次硼酸溶液坐浴治疗自愈,另17例再次手术修补。电话或门诊随访2个月～3 a,患儿排便功能均正常。结论 RVFNA大多是因后天感染获得而不是先天性疾病。采用经肛门或经会阴前庭瘘修补术疗效较为满意。复杂的会阴修补术和肠造瘘术对多数RVFNA患儿是不必要的。     

无肛舟状窝瘘术式改良及疗效评价

先天性无肛舟状窝瘘的治疗目前国内外常用的几种术式操作复杂且仍有少数患儿发生直肠回缩、瘘复发、肛门狭窄、污粪等并发症。自 1993年 3月～1998年 3月间我们设计了“直肠末端旋转式瘘修补肛门成形术”(以下简称直肠末端旋转术 ) ,疗效满意 ,报告如下。临床资料先天性无肛舟状窝瘘 15 0例 ,年龄1个月～ 1岁 3个月。直肠末端旋转术10 2例 ,其中高位无肛 14例、中位无肛5 7例、低位无肛 31例。随机完成对照组 48例 ,其中高位无肛 6例、中位无肛2 7例、低位无肛 15例 ,行骶会阴、尾路肛门成形术 2 0例 ,瘘后移肛门成形术2 8例。随访 3个月～ …     

直肠前庭瘘15例

我院自１９９０～１９９９年共收治直肠前庭瘘１５例,术后疗 效均满意,现报道如下。资料与方法 一、一般资料本组均为女性,年龄５个月～１５ ａ;手术年龄：～１ａ ３例,～２ ａ ２例,７ ａ ４例,８ ａ ３例,９ ａ ２例,１５ ａ１例。其中先天性肛门闭锁并直肠前庭瘘５例,直肠前庭瘘９例,低位复杂性前庭瘘１例。 二、治疗以手术治疗为主,手术时机取决于瘘口的大小及对排便的影响。少数患儿瘘口较大,无排便困难,不必早期手术。注意防止和治疗感染。６个月后再作瘘管切除或瘘管后移肛门成形术或骶会阴肛门成形术。瘘管后移肛…     

前矢状入路手术治疗无肛前庭瘘

无肛前庭瘘是女孩最常见的肛门直肠畸形[1 ] ,以往多采用后切或骶会阴肛门成形术治疗 ,因不能保证瘘口及内括约肌的完整性 ,外观或排便功能不满意[2 ] 。自 1 997年 7月以来 ,我们经前矢状入路手术治疗无肛前庭瘘 39例 ,效果满意。现将手术要点及体会总结如下 :资料与方法一、一般资料本组 39例 ,最大 1 6岁 ,最小 3个月 ,平均 1 1个月。患儿多以大便排出困难就诊 ,瘘口大多位于前庭部舟状窝处。术前口服 3d灭滴灵片和庆大霉素口服液 ,并于手术日前晚和当日晨各清洁洗肠 1次。二、手术方法经瘘口向直肠腔内填塞无菌绷带以阻止肠内容物外溢干…     

保留肛门内括约肌的无肛前庭及会阴瘘的手术治疗

肛门直肠畸形是小儿最常见的消化道先天性畸形，在我国的发生率是2．81万，约2／3是合并瘘口的低位无肛。治疗以往多采用后切或骶会阴肛门成形术。因不能保证位于瘘口近端的肛门内括约肌的完整性和保留位于瘘口近端的齿状线区域分布的高度特化的神经终末组织，因而外观或控便功能不理想。自1997年7月～2004年3月，我们采用保留肛门内括约肌的肛门成形术手术治疗无肛前庭及会阴瘘54例，效果满意。现结合文献加以讨论。     

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     

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.     

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.     

MAINTENANCE OF DIFFERENTIATED FUNCTION OF THE SURFACTANT SYSTEM IN HUMAN FETAL LUNG TYPE II EPITHELIAL CELLS CULTURED ON PLASTIC

We report a simplified culture system for human fetal lung type II cells that maintains surfactant expression. Type II cells isolated from explant cultures of hormone-treated lungs (18-22 wk gestation) by collagenase + trypsin digestion were cultured on plastic for 4 days in serum-free medium containing dexamethasone (Dex, 10 nM) + 8-bromo-cAMP (0.1 mM) + isobutylmethylxanthine (0.1 mM) or were untreated (control). Surfactant protein (SP) mRNAs decreased markedly in control cells between days 1 and 4 of culture, but mRNA levels were high in treated cells on day 4 (SP-A, SP-B, SP-C, SP-D; 600%, 100%, 85%, 130% of day 0 content, respectively) . Dex or cAMP alone increased SP-B, SP-C, and SP-D mRNAs and together had additive effects. The greatest increase in SP-A mRNA occurred with cAMP alone. Treated cells processed pro-SP-B and pro-SP-C proteins to mature forms and had a higher rate of phosphatidylcholine (PC) synthesis (2-fold) and higher saturation of PC (~34% versus 27%) than controls. Only treated cells maintained secretagogue-responsive phospholipid synthesis. By electron microscopy, the treated cells retained lamellar bodies and extensive microvilli. We conclude that Dex and cAMP additively stimulate expression of surfactant components in isolated fetal type II cells, providing a simplified culture system for investigation of surfactant-related, and perhaps other, type II cell functions.