重型尿道下裂一期重建手术

尿道下裂是常见的泌尿生殖系统畸形,手术是治疗本病的唯一方法,至今已知的术式多达300余种.根据矫正阴茎下弯后尿道口退缩位置,可将尿道下裂分为：远端型（阴茎头型、冠状沟型、阴茎远端1/3）,中段型（阴茎中、近端1/3）,近端型（阴茎阴囊型、阴囊型、会阴型）,其中近端型尿道下裂称为重型尿道下裂.重型尿道下裂由于阴茎下弯重,尿道缺损长,一期手术修复难度大.尤其是背侧包皮发育差、包皮不宽裕的患者,单独应用Duckett术往往较难获得成功,且Duckett段尿道过长,受血管蒂游离的长度限制,常不能满足新尿道的充分覆盖,术后易出现尿瘘、阴茎扭转等并发症;而采用游离组织修复虽可节省局部皮肤组织,但并发症较多且有继发创伤.因此对于重型尿道下裂常需多个皮瓣进行联合修复.目前学术界推崇保留尿道板的术式.以下介绍全剥离并保留尿道板加包皮岛状皮瓣尿道成形术一期修复重型尿道下裂的方法.     

预置"尿道板"的分期手术治疗严重尿道下裂

目的 评价分期手术治疗严重尿道下裂的效果.方法 2001年12月至2009年8月治疗27例严重尿道下裂患儿,年龄8个月至17岁,平均4.3岁.近端型伴重度阴茎下弯23例,既往手术失败组织挛缩4例.Ⅰ期手术:完全脱套包皮,切除阴茎腹侧纤维束带或瘢痕组织,完全伸直阴茎,游离带蒂岛状包皮内板转移至腹侧填补缺损,预置"尿道板",包皮内板缺乏侧取下唇口腔黏膜皮瓣.术后6个月Ⅱ期手术:手术方式采用尿道板纵切卷管尿道成形术.4例皮瓣与尿道板连接处出现瘢痕愈合,予瘢痕切除,口腔黏膜镶嵌后卷管成形尿道.结果 27例患儿中,无尿道狭窄、皮瓣坏死等发生,4例术后尿瘘,6个月后再修补.结论 严重尿道下裂病例采用预置"尿道板"的分期手术,单次的手术操作简化,充分结合了传统尿道板卷管术式的优点,成功率高,整形效果好.     

预置"尿道板"的分期手术治疗严重尿道下裂

目的 评价分期手术治疗严重尿道下裂的效果.方法 2001年12月至2009年8月治疗27例严重尿道下裂患儿,年龄8个月至17岁,平均4.3岁.近端型伴重度阴茎下弯23例,既往手术失败组织挛缩4例.Ⅰ期手术:完全脱套包皮,切除阴茎腹侧纤维束带或瘢痕组织,完全伸直阴茎,游离带蒂岛状包皮内板转移至腹侧填补缺损,预置"尿道板",包皮内板缺乏侧取下唇口腔黏膜皮瓣.术后6个月Ⅱ期手术:手术方式采用尿道板纵切卷管尿道成形术.4例皮瓣与尿道板连接处出现瘢痕愈合,予瘢痕切除,口腔黏膜镶嵌后卷管成形尿道.结果 27例患儿中,无尿道狭窄、皮瓣坏死等发生,4例术后尿瘘,6个月后再修补.结论 严重尿道下裂病例采用预置"尿道板"的分期手术,单次的手术操作简化,充分结合了传统尿道板卷管术式的优点,成功率高,整形效果好.     

预置"尿道板"的分期手术治疗严重尿道下裂

目的 评价分期手术治疗严重尿道下裂的效果.方法 2001年12月至2009年8月治疗27例严重尿道下裂患儿,年龄8个月至17岁,平均4.3岁.近端型伴重度阴茎下弯23例,既往手术失败组织挛缩4例.Ⅰ期手术:完全脱套包皮,切除阴茎腹侧纤维束带或瘢痕组织,完全伸直阴茎,游离带蒂岛状包皮内板转移至腹侧填补缺损,预置"尿道板",包皮内板缺乏侧取下唇口腔黏膜皮瓣.术后6个月Ⅱ期手术:手术方式采用尿道板纵切卷管尿道成形术.4例皮瓣与尿道板连接处出现瘢痕愈合,予瘢痕切除,口腔黏膜镶嵌后卷管成形尿道.结果 27例患儿中,无尿道狭窄、皮瓣坏死等发生,4例术后尿瘘,6个月后再修补.结论 严重尿道下裂病例采用预置"尿道板"的分期手术,单次的手术操作简化,充分结合了传统尿道板卷管术式的优点,成功率高,整形效果好.     

巨尿道口伴完整包皮(MIP)型尿道下裂的认识和处理

目的 探讨巨尿道口伴完整包皮型尿道下裂的认识和处理.方法 回顾性分析2004年7月至2010年2月同一治疗组收治的巨尿道口伴完整包皮型尿道下裂病例.本组13例,年龄4岁5个月至12岁1个月,平均8岁1个月.就诊原因包括尿道口位置异常2例、阴茎外观短小1例、包茎10例.尿道开口于冠状沟3例,开口于阴茎远段10例.10例行尿道板纵切卷管尿道成形术(TIP)尿道成形,3例行Duplay尿道成形.术后随访3～45个月,平均9个月.结果 9例患儿成形尿道0.5～1.3 cm,平均0.9cm.术后尿瘘1例,再次手术矫治满意.其余病例外观满意,呈包皮环切术后外观,尿流尿线正常.结论 MIP型尿道下裂常因包茎手术时发现,阴茎弯曲多不明显,矫治时可保留尿道板,Duplay或TIP手术矫治效果满意.     

先天性尿道下裂68例手术体会

目的总结68例小儿尿道下裂的治疗经验。方法68例尿道下裂患儿,对阴茎头型患儿采用尿道口前移、阴茎头成形法(MAGPI术式);冠状沟型或距冠状沟较近的阴茎体型采用尿道口基底血管皮瓣法(Mathieu术式);尿道口在阴茎体外2/3段伴有阴茎轻度下弯或无下弯者采用尿道板纵切卷管法或加盖岛状皮板法(Snodgrass术式或Onlay术式);有严重阴茎下弯的所有阴茎体型采用横裁或纵裁包皮岛状皮瓣尿道成形(Duckett术式或Hodgson术式);阴囊型或会阴型用阴囊中缝皮管加横裁包皮岛状皮瓣成形尿道(Duplay联合Duckett术式)。结果治愈62例,并发尿道瘘3例,尿道狭窄4例,治愈率为89.8%(61/68)。65例获随访,平均随访时间20.5个月(3～39个月),随访期间患儿排尿正常。结论选择正确的手术方法,重视术中技巧,选用合适的修复材料,能明显提高手术成功率。     

国内医院尿道下裂治疗现状调查

目的 了解国内尿道下裂治疗基本现状,为提高尿道下裂治疗水平提供参考意见.方法 对国内部分治疗尿道下裂医院的主治医师以上级别的医生,通过问卷调查的方法了解尿道下裂治疗情况.调查主要内容包括:尿道下裂患儿手术年龄、术式选择、术前治疗及术后并发症处理方式等.统计不同术式术后并发症发生情况.按年龄及手术量对数据进行分组,每年手术量≤100例的一组(50人),＞100例的一组(32人);手术医生年龄≤50岁一组(59人),＞50岁一组(23人).结果 我们共发出90份问卷,回收90份问卷,有效问卷82份.建议手术年龄6～30个月81份(98.8％).手术方式:对于不合并阴茎下弯的患儿,以尿道口前移阴茎头成型(MAGPI)或尿道板纵切卷管(TIP)为主;对于合并阴茎下弯的患儿,以TIP、Duckett带蒂岛状包皮瓣尿道成形或分期手术为主.手术医生年龄对合并阴茎下弯的阴茎体型尿道下裂手术方法的选择有影响.每年手术量100例以上的医生,对于合并下弯的阴茎阴囊型和阴囊型尿道下裂更多偏向使用Duckett一期修复(75.0％和53.1％).对于阴茎下弯的处理,下弯＜15°,不处理占48.8％(40/82),行阴茎背侧白膜紧缩占41.5％(34/82);下弯15°～35°,行阴茎背侧白膜紧缩占30.5％(25/82),行横断尿道板占59.8％(49/82);下弯＞35°,行横断尿道板占80.5％(66/82).尿道瘘和尿道狭窄是术后主要并发症.结论 目前国内常用的尿道下裂手术方式包括MAGPI、TIP、Duckett及分期手术.医生的年龄(50岁为界)及每年手术量(100例)对无阴茎下弯的尿道下裂手术选择的影响差异无统计学意义.     

尿道板斜切延长纵切卷管尿道成形术在尿道下裂中的应用CSCD

目的 总结尿道板斜切延长纵切卷管尿道成形术治疗尿道下裂的经验。方法 回顾性分析2015年6月至2015年11月收治的21例行尿道板斜切延长纵切卷管尿道成形术的尿道下裂患儿的临床资料。本组年龄1岁6个月～4岁11个月,平均2.8岁。阴茎体型17例,阴茎阴囊型4例。所有病例均为首次手术病例。手术方法：保留尿道板,将阴茎皮肤脱套至阴茎根部,充分伸直阴茎。于尿道板正中纵切增宽尿道板,于阴茎弯曲最明显处斜切延长尿道板,留置导尿管,卷管成形尿道。结果 本组手术时间65～100 min,平均78 min。术后近期发生尿道瘘3例（14.3%）,尿道狭窄4例（19.0%）,均在1～3次尿道扩张后缓解。术后3个月发生尿道瘘2例,无尿道狭窄、尿道憩室患儿,3个月并发症发生率为9.5%。阴茎伸直效果满意。结论 尿道板斜切延长纵切卷管尿道成形术治疗尿道下裂近期效果满意,术后并发症发生率较低,且容易在基层医院开展。     

TIP术治疗小儿尿道下裂及尿瘘

目的评价尿道板切开成管尿道成形术(TIP术)治疗小儿尿道下裂及尿瘘的疗效。方法采用TIP术治疗年龄2～10岁男童尿道下裂及尿瘘共35例，其中巨尿道1例，阴茎型14例、阴茎阴囊型7例，阴囊型1例，尿道下裂Ⅰ期伸直术后7例，尿瘘5例。结果阴茎型尿道下裂成功13例，瘘1例；阴茎阴囊型成功6例，瘘1例；Ⅰ期伸直术后成功6例，瘘1例；尿瘘成功2例，瘘复发3例，阴囊型尿道下裂及巨尿道各1例均成功。结论TIP术治疗小儿尿道下裂，其手术方法操作相对简便，取材合理，术后效果好，值得推广。     

下唇黏膜代尿道在尿道下裂治疗中的应用

目的 分析评估下唇黏膜代尿道在复杂性尿道下裂手术中的应用价值.方法 2006年3月至2009年10月共对24例复杂性尿道下裂患儿施行了下唇黏膜代尿道手术.年龄2岁3个月至14岁,平均7.2岁.其中以往手术失败14例,表现为阴茎周围残留大量不规则瘢痕,或膀胱黏膜代尿道后严重尿道狭窄和憩室.或严重阴茎海绵体弯曲残留,或尿道大段缺损伴膀胱造瘘术后等,尿道周围无其他组织如尿道板、皮肤等可用替代尿道.手术方法为先行阴茎整形,包括包皮和阴茎头,完全矫正海绵体弯曲,6个月后行下唇黏膜代尿道手术.会阴型尿道下裂10例,方法为先完全矫正阴茎弯曲和阴茎阴囊转位,同时利用原始尿道板将尿道外口从会阴延长至阴茎根部,6个月后行下唇黏膜代尿道.结果 所有病例均获满意效果,包括阴茎外观,即阴茎勃起时无明显弯曲、尿道开口位于阴茎头顶端、无包皮不规则瘢痕堆积、无排尿困难等.术后9例出现尿瘘,其中2例术后自愈,7例经一次修补后治愈.出现尿道狭窄6例,均经尿道扩张后痊愈.下唇取黏膜处均无术后出血.结论 下唇黏膜代尿道可作为尿道下裂治疗中最后选择的方法之一,具有黏膜切取方便,易存活,新尿道组织黏膜接近正常组织的优点,但并发症发生率仍较高.     

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.     

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   

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.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.