脊髓拴系综合征术后结肠传输时间测定及意义

目的探讨脊髓拴系综合征术后远期结肠动力改变情况。方法对15例脊髓拴系综合征术后患儿进行临床评分和结肠传输时间的测定。结果应用6分法评分标准,将脊髓拴系综合征术后患儿分为排便功能优良组和劣组。与对照组相比,优良组各节段结肠传输时间均无明显差异,劣组TCTT、LCTT、RSTT明显延长,二组相比分别为(59.9±25.0)h比(28.7±7.7)h,P<0.05;(13.9±8.1)h比(6.5±3.8)h,P<0.05;(34.7±22.0)h比(13.4±5.6)h,P<0.05。与优良组相比,劣组TCTT、LCTT、RSTT明显延长,二组相比分别为(59.9±25.0)h比(25.9±3.2)h,P<0.05;(13.9±8.1)hvs(6.2±1.6)h,P<0.05;(34.7±22.0)h比(13.3±2.6)h,P<0.05。结论脊髓拴系综合征术后患儿排便功能障碍主要表现为结肠传输时间正常型和混合型。     

慢传输型便秘的诊断及治疗

目的　评价检测结肠传输时间对诊断小儿慢传输型便秘的价值 ,探讨聚乙二醇 4 0 0 0 (PEG 4 0 0 0 )治疗慢传输型便秘的疗效及安全性。方法　便秘组 4 6例 ,健康对照组 32例。应用简化的不透X线标志物法 ,测定两组结肠传输时间 (CTT)和分段结肠传输时间 ,包括右半结肠传输时间 (RCTT)、左半结肠传输时间(LCTT)和直肠乙状结肠传输时间 (RSTT)。给予全CTT延长的便秘患儿早晚 2次口服PEG 4 0 0 0 (0 .2 5～ 0 .5g/kg ,2次 /d) ,治疗 2周 ,记录排便次数和大便性状 ;停药 1周后复查CTT ,比较服药前后CTT变化。 结果　便秘组CTT、RCTT、LCTT和RSTT较对照组明显延长 (6 9.9± 13.4 )hvs (15 .2± 4 .6 )h (2 3.7± 5 .4 )hvs (7.4±2 .7)h ,(15 .4± 3.7)hvs (3.8± 2 .4 )h ,(30 .8± 6 .7)hvs (4 .0± 2 .8)h(P均 <0 .0 1)。结肠慢传输型便秘 2 4例 ,治疗前后CTT、RCTT、LCTT和RSTT分别为 (6 4 .3± 8.4 )hvs (16 .3± 3.2 )h ,(2 1.9± 4 .2 )hvs (7.8± 3.1)h ,(14 .8± 3.9)hvs (4 .4± 2 .3)h ,(2 7.6± 5 .1)hvs (4 .1± 2 .3)h ,经统计学处理有极显著差异 (P均 <0 .0 0 1)。每周排便次数由 (2 .2± 0 .4 )次增加至 (10 .7± 1.8)次 (P <0 .0 1)。 83.3%大便性状恢复正常 ,总有效率 95 .8% ,不良反应发生     

经肛巨结肠根治术后肛肠功能评价

目的探讨经肛门巨结肠根治术后排便模式、结肠功能和肛门括约肌功能。方法对58例经肛门巨结肠根治术后半年以上儿童进行随访,并进行排便功能问卷调查、钡灌肠、结肠传输时间和肛门直肠测压检查,对其术后肛肠功能进行综合评价。结果58例接受随访的患儿中,4例(4/58)稀便、便频,9例(9/58)污便,5例(5/58)便秘,3例(3/58)小肠结肠炎;钡灌肠结果:结肠形态恢复良好,乙状结肠迂曲减少或消失,与切除肠段长短相关;全部病例术后直肠肛管角(°)较对照组显著开大(123.3±15.1vs79.0±11.6,P<0.01),有症状组又较无症状组显著开大(135.6±15.9vs111.0±14.3,P<0.05)。结肠传输时间(h):与对照组比较,有症状组显著缩短(25.2±5.6vs28.7±7.7,P<0.05。直肠肛管反射3例阳性,2例弱阳性;肛管最大压力(mmHg):便秘组的最大收缩压明显高于无症状组和对照组(211±36vs200±65,P<0.05;211±36vs190±38,P<0.05);向量容积(cm×cmHg2)和对称指数:污便组向量容积和对称指数较术前及对照组显著降低(381±109vs662±31,P<0.05;0.69±0.32vs0.70±0.07,P<0.05);便秘组对称指数与术前无显著差异,但显著高于对照组(0.74±0.02vs0.70±0.07,P<0.05)。结论经肛门巨结肠根治术后排便功能、结肠功能和括约肌功能多数良好。少数病例排便功能障碍可能与术后乙状结肠曲减少或消失、“新直肠”储便功能代偿不全和拖出结肠致直肠肛管角开大、肛门括约肌痉挛失迟缓有关。     

小儿结肠传输时间的检测及临床意义

目的　了解儿童结肠传输时间 (CTT)的正常值 ,并探讨促肠动力药对小儿功能性便秘 (FC)结肠传输时间的影响。方法　采用不透X线标记物测定 68例正常儿童及 2 8例FC患儿全CTT和分段结肠传输时间 ,对全CTT延长的便秘患儿 ,给予促肠动力药物西沙必利 0 2mg kg ,每日 2次 ,治疗 2周 ,停药后 1周复查CTT ,比较服药前后CTT的变化。结果　全CTT的正常值上限为 2 4h ,FC组全结肠及各节段结肠传输时间较对照组明显延长 (P <0 0 5 )。 2 8例FC患儿中 16例的全CTT延长 ,经治疗后平均全CTT由 (5 4 0 1± 3 2 3 )h缩短至 (19 63± 1 3 8)h(P <0 0 1) ,各节段结肠传输时间亦缩短。结论　不透X线标记物检查技术简单易行 ,结果可靠。促肠动力药能缩短FC患儿全结肠及各段结肠传输时间 ,可用于FC患儿     

便秘患儿的胃肠传输时间测定及其意义

目的　初步测定正常中国儿童的胃肠传输时间并探讨胃肠传输时间对小儿便秘的病因、诊断和分型的意义。方法　研究对象分为对照组和便秘组。对照组 3 3例 ,男 2 1例 ,女 12例 ,平均年龄 5岁。便秘组 2 5例 ,男 15例 ,女 10例 ,平均年龄 7岁 ,均符合Benninga的便秘诊断标准。应用简化的不透X线标记物追踪法 ,即多次口服标记物一次摄片法测定正常和便秘儿童的全胃肠传输时间 (Totalgastrointestinaltransittime,TGITT)和节段性结肠传输时间 ,包括 :右半结肠传输时间 (Rightcolonictransittime ,RCTT) ;左半结肠传输时间 (Leftcolonictransittime ,LCTT)和直肠乙状结肠传输时间(Rectosigmoidcolonictransittime ,RSTT) ;部分患儿联合应用X线排便造影 ,探讨便秘的诊断和分型 ;结果　正常儿童的TGITT ,RCTT ,LCTT和RSTT分别为 2 8 7± 7 7小时、7 5± 3 2小时、6 5± 3 8小时和 13 4± 5 6小时 ;便秘组的TGITT ,LCTT和RSTT较对照组明显延长 (92 2± 5 5 5小时vs 2 8 7± 7 7小时 ,P <0 0 0 1;16 9± 12 6小时vs 6 5± 3 8小时 ,P <0 0 1;和 61 5± 2 9 0小时vs 13 4± 5 6小时 ,P <0 0 0 1)。RCTT无显著变化。X线排便造影显示直肠前突、会阴下降综合征和耻骨直肠肌痉挛综合征各 1例。结论　首次     

腹腔镜改良Soave巨结肠根治术后排便功能的随访

目的　评价腹腔镜改良Soave根治术后患儿排便控制情况。方法　自 1999年 5月～2 0 0 1年 9月 ,对 6 4例年龄为 10d～ 1岁的先天性巨结肠患儿行腹腔镜改良Soave巨结肠根治术。术后定期随访患儿 ,平均随访时间为 (2 3.8± 1)个月 ,对患儿大便控制能力、便秘及结肠炎的发生率进行评价。结果　获访 4 3例患儿。大便控制评分正常 (10分 ) 2 2例 ,好 (6～ 9分 ) 18例 ,欠佳 (1～ 5分 )3例 ;1例患儿 (2 .3% )有持续便秘 ;5例 (11.6 % )患儿曾出现结肠炎。结论　应用腹腔镜改良Soave巨结肠根治术治疗新生儿及小婴儿先天性巨结肠 ,术后可获得良好的排便控制功能。     

结肠传输时间测定和直肠肛管测压在儿童便秘中的意义

目的　小儿便秘的病理生理基础目前尚不清楚,该文对结肠传输时间和直肠肛管测压在儿童便秘 中应用的意义进行初步探讨。方法　对28例便秘儿童(便秘组)和43例正常儿童(对照组)进行全胃肠传输时间 (TGITT)、左半结肠传输时间(LCTT)、右半结肠传输时间(RCTT)和直肠乙状结肠传输时间(RSTT)测定和直肠肛 管向量测压(包括肛管压力、向量容积和对称指数)。根据结肠传输时间是否超过对照组均值加两个标准差将28 例便秘儿童分为传输时间正常型便秘组和传输时间延长型便秘组。结果　便秘组儿童TGITT、LCTT和RSTT较对 照组均显著延长(92±56hvs29±8h,17±13hvs7±4h,62±29hvs13±6h)(P均<0.01),肛管最大收缩压显 著升高(216±44mmHgvs190±38mmHg)(P<0.05),对称指数显著降低(0.71±0.06vs0.84±0.08)(P< 0.05),两组RCTT正常。传输时间正常型便秘组和传输时间延长型便秘组儿童肛管最大压力、向量容积和对称指 数差异无显著意义。结论　便秘儿童的结肠传输功能和/或直肠肛管动力存在不同程度异常;无论结肠传输时间 正常与否,所有便秘患儿均应进行直肠肛管测压检查。     

先天性巨结肠Swenson改良术后排便功能与生活质量研究

目的：评估先天性巨结肠Swenson改良术后排便功能和生活质量。方法：随访常见型先天性巨结肠Swenson行改良术后患儿45例（男37例，女8例），手术年龄2．5个月－6岁，平均1．6岁。术后随访8－16年，平均10．9年，评估其排便功能和生活质量。结果：术后并发症有：污染和小肠结肠炎17例（37．8％），便秘3例（6．7％），大便失禁3例（6．7％），小便失禁4例（8．9％）；吻合口狭窄2例（4．4％）和粘连性梗阻1例（2．2％）。23例（51％）表现不同程度的排便障碍，但肛门功能优良率仍达到84．4％（李正评分）和93．4％（Rintala评分），生活质量评分显示，优者18例（40％），良者21例（46．7％），差者6例（13．3％），结论：先天性巨结肠Swenson改良术后，多数患儿远期肛门功能和生活质量优良，排便功能障碍是影响生活质量的主要因素。     

聚乙二醇4000对重型颅脑损伤便秘患儿的疗效

目的　通过检测重型颅脑损伤便秘患儿结肠传输时间 ,评价聚乙二醇 4 0 0 0 (PEG 4 0 0 0 )对此类患儿的疗效。方法　符合便秘诊断标准的重型颅脑损伤患儿 ,男 11例 ,女 7例 ;年龄 3～ 14岁。应用简化不透X线标志物法 ,测定其治疗前后结肠传输时间 (CTT)和分段CTT ,包括右半结肠传输时间 (RCTT)、左半结肠传输时间 (LCTT)和直肠乙状结肠传输时间 (RSTT)。研究对象早晚 2次口服PEG 4 0 0 0 (0 .2 5～ 0 .5 ) /kg ,2次 /d ,治疗3周 ,记录排便次数和大便性状 ,停药 1周后复查CTT ,比较服药前后CTT的变化。结果　该组患儿治疗前后CTT、RCTT、LCTT和RSTT分别为 (76 .5± 10 .2 )hvs (17.8± 5 .1)h ,(31.4± 5 .3)hvs (9.3± 4 .3)h ,(16 .8± 6 .1)hvs (3.8± 3.4 )h ,(2 8.3± 6 .4 )hvs (4 .7± 3.1)h (P均 <0 .0 0 1)。排便次数由 (1.5± 0 .5 )次 /周 ,增加至 (7.3± 0 .8)次 /周 (P <0 .0 1)。 83.3%患儿大便性状恢复正常 ,总有效率为 94 .4 % ,不良反应发生率为5 .5 %。结论　CTT对诊断便秘和选择治疗方案有重要意义。PEG 4 0 0 0治疗重型颅脑损伤便秘患儿安全、有效 ,是治疗儿童便秘的第一选择     

改良Swenson术治疗先天性巨结肠

目的 探讨改良Swenson手术治疗先天性巨结肠的有效性、安全性。方法 对我院自1995年10月-2004年6月采用改良Swenson术治疗的52例（男50例，女2例，48例为常见型，4例为短段型）先天性巨结肠患儿进行回顾性分析。所有患儿均经病理明确诊断，术后常规肛门指检。随访40例、时间1～6年。对顽固性便秘、营养状况、吻合口狭窄、污裤及失禁等情况进行评价。结果 52例患儿顽固性便秘消失，营养改善。近期无吻合口漏，无腹腔及伤口感染，远期随访无便秘复发及直肠回缩，无吻合口狭窄、污裤及失禁。52例中仅2例有小肠结肠炎，占3．8％。结论改良Swenson术适用于常见型、短段型及长段型先天性巨结肠并且疗效满意，具有较高的实用性和安全性。     

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.     

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.     

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.     

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.