小儿胰腺实性-假乳头状瘤(附2例报告)

目的 提高对小儿胰腺实性-假乳头状瘤的认识。方法通过介绍2例小儿胰腺实性-假乳头状瘤的诊治经验，结合复习有关本病的文献报道加以总结。结果本组2例患儿，均行手术治疗，效果满意。病理证实为胰腺实性-假乳头状瘤。通过有关文献复习，提高对本病的认识。结论胰腺实性-假乳头状瘤多见于青少年和年轻女性，小儿约占1／3，较罕见。临床主要表现左上腹隐痛，偶有急性胰腺炎史。B超和CT扫描可发现胰腺囊实性占位性病变。手术依据肿瘤大小、部位与周围浸润程度，可选择肿瘤局部切除；胰体、尾切除；胰十二指肠切除。可达到满意的治疗效果。SPT具有独特的临床病理学特点，目前认为是一种原始胰腺上皮性肿瘤，有低度恶性倾向，预后良好。     

小儿胰腺实性假乳头状瘤的诊断与外科治疗

目的 总结小儿胰腺实性假乳头状瘤(solid pseudo-papillary tumors of pancreas,SPTP)的诊断及治疗经验.方法 回顾性分析2002年1月至2011年12月9例胰腺实性假乳头状瘤患儿的临床资料.其中,男1例,女8例;中位年龄11岁5个月.结果 小儿SPTP无特异性临床表现,主要临床表现为腹痛或腹部不适,CT检查均提示胰腺囊实性或实性占位.手术证实肿瘤位于胰头、颈部4例,胰体部2例,胰尾部3例.肿瘤直径2.8～16.4 cm,无腹腔或肝脏转移.行胰十二指肠切除术(Child术)2例,肿瘤局部切除术4例,胰腺节段切除、胰肠吻合术1例、胰尾联合脾脏切除术1例,保留脾脏的胰体尾切除1例.9例肿瘤标本在显微镜下均具有SPTP的典型病理学表现.随访3个月～8年,均未发现肿瘤复发及转移.结论 CT是小儿SPTP重要的诊断方法,手术为其主要治疗方式,手术术式的选择应依据术前CT和术中对肿瘤部位、包膜是否完整和是否侵及周围组织的探查来决定.     

小儿胰腺囊实性乳头状瘤的超声分析:附4例报告

胰腺囊实性乳头状瘤为一种少见的胰腺肿瘤,近年来被逐步认识。为提高对小儿胰腺囊实性乳头状瘤的诊断水平,现将经手术病理证实的4例资料回顾性分析如下。     

小儿胰腺恶性肿瘤11例诊治分析

目的探讨小儿胰腺恶性肿瘤的临床特征和诊治方法。方法选取1992年7月-2004年5月收治的11例小儿胰腺恶性肿瘤。男5例,女6例;年龄3～14岁。7例患儿表现为腹痛,5例表现为腹部包块;其他症状包括黄疸3例,呕吐、厌食和发热各2例。11例患儿中,5例行胰十二指肠切除术,3例行脾及胰体尾切除术,2例行肿瘤局部切除术,1例仅行病理活检术,并对3例患儿分别给予化疗或放疗。结果11例患儿病灶术前至少一项影像学检查定位于胰腺,3例术前B超误诊为后腹膜肿块;术中探查可见5例患儿肿瘤位于胰体部,3位于胰头体部,2例位于胰尾部,1例位于胰头部;瘤体直径4～12cm,平均长径约6.5cm;肿瘤主要侵犯十二指肠和脾脏,均无远处转移。肿瘤类型包括囊实性乳头状肿瘤4例,胰母细胞瘤3例,无功能胰腺内分泌肿瘤2例,腺泡细胞癌及不典型胰腺类癌瘤各1例。术后随访2个月～9a,4例死亡,7例健在,其中1例复发后行胰头部和十二指肠切除处理。结论儿童胰腺恶性肿瘤少见,类型多样,完整切除后能长期生存,预后较成人好,但存在复发的可能,同时化疗和放疗的效果还不确定。     

保留脾脏的胰体尾部肿瘤切除术治疗小儿胰腺实性假乳头状瘤

目的探讨保留脾脏的胰体尾肿瘤切除术治疗小儿胰腺实性假乳头状瘤的可行性及临床疗效评估。方法回顾性分析2009年1月-2012年12月在天津市儿童医院收治的位于胰体尾部胰腺实性假乳头状瘤患儿4例,均成功行保留脾脏的胰体尾肿瘤切除术。术后均在门诊随访,内容包括腹部B超和CT检查,肿瘤标记物和胰腺外分泌功能,并由专人测量其生长发育情况。结果患儿手术过程顺利,切除肿瘤及部分胰体尾,全部成功保留脾脏,肿瘤直径介于4．0～12．5em,包膜完整,手术过程中无脾脏动、静脉损伤及脾实质撕裂伤,无术中输血。术后3d肠功能恢复后开始进食,恢复过程顺利,住院时间为6～9d。无围手术期出血、胰瘘等并发症发生。病理检查结果均为胰腺实性假乳头状瘤,Ki-67〈5％。所有患儿获得随访,时间为6～48个月,均健康存活,生长发育正常。腹部B超、CT检查未见肿瘤复发,肿瘤标记物及胰腺外分泌功能检查未见异常。结论发生在胰体尾部的胰腺实性假乳头状瘤虽然肿瘤直径较大,但是包膜完整,与胰腺血管关系疏松,易于将脾脏动静脉分开而成功保留脾脏。     

胰腺囊实性乳头状瘤的临床特点

目的探讨小儿胰腺囊实性乳头状瘤(SCPT)的诊断与治疗的特点。方法回顾分析近5年收治的7例SCPT患儿临床资料。男2例,女5例;平均11.5岁。病例均有诱因腹痛来诊,71.4%为腹部外伤,28.6%为过饱饮食后腹痛,查体以腹部包块为主。7例均经手术治疗,3例胰头肿瘤中2例行胰十二指肠切除术,1例仅行病理活检,4例胰尾肿瘤全部切除。结果7例均一期愈合出院。随访4个月~4.5年,近期效果良好。术后病理确诊。结论SCPT为低度恶性肿瘤,患儿平时无明显症状,有诱因后可引起突然腹痛,腹部可触及肿物为其特点,SCPT应早期手术切除肿瘤,术后近期效果满意。     

小儿胰腺实体囊性乳头状瘤三例报告

目的 提高对小儿胰腺实体囊性乳头状瘤的认识。方法 介绍3例小儿胰腺实体囊性乳头状瘤的诊治经验并复习有关本病的文献报道，加以总结。结果 3例患儿术前经B超及CT检查，术中探查及术后病理检查证实为胰腺实体囊性乳头状瘤。并通过手术治疗，取得了良好的效果。通过复习文献加深了对本病的认识，对提高本病的正确诊断有帮助。结论 小儿胰腺实体囊性乳头状瘤很少见，是低度恶性肿瘤。多见于女性。腹部肿物是首要症状。B超及CT检查可帮助诊断。完全切除肿瘤是本病唯一的治疗选择，可取得满意的效果。     

儿童胰腺肿瘤精准手术切除策略探讨(附15例报告)

目的 探讨影响小儿胰腺肿瘤手术切除方式的因素及精准手术切除的可行性.方法 回顾性分析2002年10月至2013年7月收治的儿童胰腺肿瘤15例的临床资料.15例中,女10例,男5例;年龄4个月～14岁,平均8岁4个月.肿瘤位于胰头部9例,胰体尾部6例,伴肝转移1例,合并肝、肠系膜病变1例.结果 本组均接受手术治疗.肿瘤位于胰腺头部9例行保留幽门、胰十二指肠切除术7例,行肿瘤切除术2例,此2例肿瘤均来源于胰头钩突部;肿瘤位于胰腺体尾部6例,均行胰体/尾+脾切除术.病理检查报告:胰腺囊实性假乳头状瘤9例,胰腺母细胞瘤3例,浆液性囊腺瘤1例,胰岛细胞癌1例,促纤维增生性小圆细胞瘤1例.15例患儿术后恢复顺利,随访6个月～12年,促纤维增生性小圆细胞瘤1例患儿死亡,余者均存活.结论 手术切除肿瘤是治疗儿童胰腺肿瘤得重要手段,多数患儿仅通过手术切除肿瘤可长期存活.手术原则是尽可能保持胃肠道连续性,精准手术,尽可能多的保留正常的胰腺组织.影响决定小儿胰腺肿瘤术式的主要因素是肿瘤部位、周围重要组织是否受累肿瘤是否累及胆管、主胰管及脾血管.CT、MRCP属非侵袭性检查方法,可多平面分析,且无需注射造影剂,可对胆管及胰管状况肿瘤进行术前评估,为胰腺肿瘤确定精准手术方案提供依据.     

小儿胰腺肿瘤的诊断与治疗(附15例报告)

目的 探讨小儿胰腺肿瘤的诊断和治疗.方法 回顾性分析2002年1月至2010年1月收治的15例胰腺肿瘤,男8例,女7例;年龄45 d至17岁(平均11.3岁).临床表现:腹痛9例,2例合并黄疸;腹部包块6例.B超和CT检查均为胰腺占位性病变.手术方式:腹腔镜胰腺肿瘤切除术6例;胰腺体尾部切除术4例;肿瘤剔除术4例;Whipple术1例.结果 本组患儿无围手术期死亡.病理:胰腺实性乳头状瘤8例,胰腺母细胞瘤4例,胰腺血管瘤1例,胰腺无功能性内分泌癌1例,胰腺癌1例.术后随访1年至8年,患儿全部存活.14例未见复发和转移,1例胰腺癌化疗后2年肝转移,行转移瘤局部切除术后治愈.结论 小儿胰腺肿瘤罕见,临床多见胰腺实性乳头状瘤和胰腺母细胞瘤.腹腔镜和开腹根治性手术均可获得良好的治疗效果.胰腺癌根治性手术后易复发,需长期随访.     

儿童胰腺实性-假乳头状瘤的临床病理分析

目的探讨儿童发生的胰腺实性-假乳头状肿瘤的临床、病理特点及鉴别诊断。方法对我院2005年-2016年确诊的21例临床资料及病理特点进行回顾性分析。结果男5例,女16例,发病年龄平均10.3(5～15.5)岁,临床表现:上腹痛12例(5例伴呕吐,1例伴皮肤巩膜黄染、大便色浅及尿色深;2例因急腹症入院),发现腹部包块9例。影像学(超声=21,CT=20,MRI=9)均提示胰腺囊和(或)实性占位。实验室检查:除3例淀粉酶和白细胞升高外,余未见异常。21例均行胰腺肿瘤切除术(其中3例同时行Whipple手术),1例先行胰腺囊肿内引流术后,再择期行肿瘤切除。肿瘤部位:胰头10例,胰体3例,胰体尾4例,胰尾4例。全部患者经术后病理确诊后,仅密切随访未再行化疗。随访结果:仅1例术后11个月复发,再次手术切除;所有患儿仍在随访中。结论胰腺实性-假乳头状肿瘤属较罕见的低度恶性胰腺外分泌肿瘤。临床表现及影像学检查均无特征性。病理组织学见上皮样细胞形成的实性或假乳头状结构。若瘤体较大,且有明确的包膜、血管及周围神经的浸润,异型性明显及核分裂活性增加,以及肿瘤坏死明显等,则提示恶性的可能。肿瘤的完整切除既是主要的治疗手段,也是预后相关的重要因素。     

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.