倒V形外展截骨术治疗先天性髋内翻

目的　评价倒V形外展截骨术治疗先天性髋内翻的临床应用价值。方法　 2 7例先天性髋内翻的患儿 ,年龄 3～ 1 4岁 ,均采用倒V形外展截骨术治疗 ,并对临床效果及X线片进行评价。结果　本组共有 2 2例 (2 8髋 )获得随访 ,平均随访 5 .4年 ,参考史氏标准 ,临床评价优良率为 82 .1 % ;术后及最后随访时的NS角、HE角和ATD值均较术前有显著性变化 (P <0 .0 1 )。结论　倒V形外展截骨术操作简单 ,内固定牢固 ,矫正充分 ,临床效果好 ,不易复发 ,是治疗先天性髋内翻较满意的方法     

Ⅰ期Westin骨盆截骨和股骨短缩截骨治疗年长儿童发育性髋关节脱位

目的介绍一种适于治疗年长儿童发育性髋脱位的手术方法,重点评价其重建髋关节稳定性的效果。方法从1993~1998年,应用Westin骨盆截骨和股骨短缩截骨,治疗年长儿童先天性髋脱位32例40个髋,患儿年龄平均9.5岁(6.6~13岁)。术前股骨头平均向上移位3.8cm(2.6~5.5cm),术前髋臼指数平均42°(38°~50°)。沿用Mckay的临床评定标准和Severin的X线评定标准,对手术结果做出评价。结果31例39个髋术后获得3.4年至7年(平均3.5年)的随访。术后髋臼指数平均下降至22°,CE角平均增加至34°。临床评定结果:优20髋(51.3%),良14髋(35.9%),可2髋(5.1%),差3髋(7.7%);X线评定结果:优19髋(48.7%),良13髋(33.3%),可4髋(10.3%),差3髋(7.7%)。结论Westin骨盆联合截骨是治疗①年龄>6岁;②髋臼浅和外侧壁缺损、髋臼指数>45°;③股骨头与髋臼的大小相匹配者的理想方法。采取Ⅰ期股骨短缩截骨,而术前不做骨骼牵引,能有效的减少股骨头缺血性坏死的发生率。但是,髋臼后外侧严重的缺损、股骨头明显大于髋臼的头臼不匹配者,应视为本手术的禁忌证。     

外展推移截骨术治疗先天性髋内翻

目的：分析转子间外展推移截骨术治疗先天性髋内翻的远期疗效。方法：用转子间外展推移截骨术治疗先天性髋内翻２０例（２７髋），平均随访１４年。结果：所有股骨头骺板早期闭合，大转子上移，大部分髋臼发育不良，股骨头球形适应改变，肢体短缩。结论：手术可以使股骨头骺板处在正常位置上，但不能使股骨头骺板内部异常生长得到控制。股骨头骺板的早期闭合是骺板损伤后修复的结局，它维持头干角的稳定并引发周围形态的畸形     

改良Pauwels'股骨转子间截骨术治疗儿童创伤后髋内翻

目的 评估改良Pauwels'股骨转子间截骨术在儿童创伤性髋内翻治疗中的作用.方法 作者近年来收治10例儿童创伤性髋内翻患儿,其中男7例、女3例,平均年龄11.2岁,采用改良Pauwels'股骨转子间截骨解剖钢板内固定术,比较术前术后颈干角、双侧肢体长度的变化.结果 10例患儿均获随访,平均随访时间3.5(3~5)年,...     

Dega截骨术治疗发育性髋关节脱位

目的探讨Dega截骨术治疗发育性髋关节脱位的治疗效果。方法采用Dega截骨术治疗发育性髋关节脱位36例40个髋,女29例,男7例;左髋16例,右髋16例,双髋4例。术前股骨头脱位高度平均2．0cm,髋臼指数平均38．5°。手术年龄平均5．3岁。结果术后随诊时间平均2年,根据McKay的临床评定标准,32个髋关节为优,6个为良,1个为可,1个为差。X线片结果显示CE角平均26°,2个髋关节出现Ⅰ型股骨头缺血性坏死。根据Severin的评定标准,29个髋关节为优,7个为良,4个为可。结论Dega截骨术是治疗发育性髋关节脱位的有效术式之一。     

大龄儿童发育性髋关节脱位的手术治疗

目的 探讨大龄儿童发育性髋关节脱位的手术治疗方法.方法 手术治疗6岁及以上大龄儿童发育性髋关节脱位39例(48髋),平均手术年龄8.5岁,有8例(11髋)为手术后再脱位的患儿.手术方式有Salter骨盆截骨术4例(6髋),Pemberton髋周截骨术19例(22髋),Dega截骨术12例(16髋);Westin截骨术4例(4髋).其中髋臼软骨有明显缺损,软骨下松质骨部分裸露23例(28髋),取自体股骨近端游离骨膜移植修复.全部病例均行股骨粗隆下去旋转短缩截骨,短缩2～4.5 cm(平均2.8 cm);去旋转25°～45°(平均32°),保留股骨颈前倾角10°～15°,股骨截骨处以鹅头钉或四孔钢板固定.结果 术后获得随访36例(45髋).随访时间5～10年(平均7.2年).随访结果按Mckay临床疗效标准,优17髋(37.8%)、良18髋(40.0%)、可6髋(13.3%)、差4髋(8.9%),优良率达77.8%;按Severin X线评定标准,优19髋(42.2%)、良17髋(37.8%)、可7髋(15.6%)、差2髋(4.4%),优良率达80.0%.术后髋臼指数平均降至18°;CE角平均30°;髋臼覆盖率达平均95%.术后半脱位2例(4.4%),股骨头缺血坏死4例(8.9%),髋关节功能障碍(屈曲＜60°)6例(13.3%).结论 大龄儿童DDH病理改变复杂,术前应根据X线片、CT等检查予以全面评估,制定个性化手术方案;术中松解内收肌和髂腰肌,联合股骨短缩去旋转截骨术,力争达到头臼中心性复位,并在此基础上重建髋臼;对关节软骨面缺损明显者,可移植自体游离骨膜予以修复;术后早期不负重功能锻炼、持续被动活动等,可以显著降低术后再脱位、关节僵硬、股骨头坏死等并发症,获得较满意的疗效.     

骨盆股骨短缩去旋转内翻截骨治疗先天性髋脱位

目的 探讨骨盆截骨结合股骨短缩去旋转内翻截骨治疗先天性髋关节脱位的疗效。方法 手术治疗先天性髋关节脱位15例16髋，全部均为女性，年龄平均4岁6个月(3岁～6岁6个月)，采用髋关节切开复位、骨盆截骨及股骨短缩去旋转内翻截骨术。结果 优13个髋，良3个髋，没有再脱位。X线片结果优12个髋，良4个髋，没有发生股骨头缺血性坏死。结论 骨盆截骨结合股骨短缩去旋转内翻截骨术治疗先天性髋关节脱位能够获得满意的结果。     

Tavares改良骨盆截骨术治疗儿童重度发育性髋关节脱位

目的 介绍、应用Tavares改良骨盆截骨术治疗儿童重度发育性髋关节脱位。方法 2000年1月-2005年6月用Tavares改良骨盆截骨术治疗儿童（2岁4个月～5岁10月）重度发育性髋关节脱位23例，术中作以下改良：①自髂前下棘上方至坐骨切迹前方数毫米处作与髋关节囊平行的弧形截骨，向外、下翻转截骨远端并造成坐骨切迹处青枝骨折；②截骨远端翻转后留下的空隙用自体股骨或（和）同种冻干皮质骨加自体髂骨植入。术后随访13～36个月。结果 23例中术后15例出现患侧闭孔较健侧变小，股骨头复位成功率100％。术前髋臼指数35°～50°，平均38°，术后髋臼指数10°～25°，平均18°，最后随访时的髋臼指数较术后最大增加3°。术后CE角20°～48°，平均32°。植入同种冻干皮质骨形态1年后X线片上基本消失。按Mckay标准评定关节功能：优18髋，良4髋，可1髋。按Severia标准评定X线表现：优16髋，良5髋，可2髋。结论 Tavares改良骨盆截骨术兼有Pemberton关节囊周围髋臼成形术和Salter髂骨截骨术的特征，是治疗年龄小于6岁儿童重度发育性髋关节脱位较理想的术式。同种冻干皮质骨植入增加了植骨的质和量，使已矫正的髋臼指向和髋臼指数得到很好的维持。     

楔形冻干同种髂骨块在Salter、Pemberton手术中的应用

目的评价楔形冻干同种髂骨块在两种骨盆截骨手术中的应用效果。方法回顾性测量1998年7月～2000年9月期间Salter和Pemberton骨盆截骨手术前和手术后不同时期的髋臼指数变化,平均观察植骨块在不同时期的成骨融合情况。结果30例患儿34髋术后平均随访3年,采用Salter手术、楔形冻干同种髂骨块移植、克氏针内固定治疗18髋,随访末期髋臼指数平均矫正19°,植骨块完全融合时间平均1.2年;采用Pemberton手术、楔形冻干同种髂骨块移植治疗16髋,随访末期髋臼指数平均矫正24°,植骨块完全融合时间平均1.3年。结论在年幼儿童先天性髋关节脱位的Salter、Pem鄄berton手术中应用冻干同种髂骨块,既能弥补单纯自体髂骨植骨块厚度和强度的不足,又能够有效防止手术纠正后的髋臼指数的丢失。     

大年龄儿童发育性髋脱位术后再脱位的治疗

目的 探讨大年龄儿童发育性髋脱位术后再脱位的治疗方法及效果.方法 2001年12月至2008年6月,本院经手术治疗大年龄儿童发育性髋脱位术后再脱位11例（11个髋）.平均手术年龄8（6～13）岁.均采用再次切开复位手术,其中实施Dega骨盆截骨术7例,Staheli髋臼延伸术3例,Chiari截骨术1例.8例实施股骨近端缩短、旋转截骨.结果 髋关节功能根据MeKay的临床评定标准,6个髋关节为优,3个髋关节为良,2个髋关节为可.x线片结果采用Severin的评定标准,5个髋关节为优,4个髋关节为良,2个髋关节为可.结论 大年龄儿童发育性髋脱位术后再脱位的治疗应根据髋关节的病理变化,选择切开复位、个性化髋臼手术和股骨近端缩短、旋转截骨,以实现髋关节中心性复位.     

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.