672例儿童声嘶的病因分析及对策

目的探讨儿童声嘶的常见病因及预防措施。方法回顾性分析672例声嘶患儿临床资料，对声嘶原因及诊治结果进行分析。结果本组672例中，慢性喉炎303例，占45．08％；声带小结188例，占27．97％；喉软骨软化症89例，占13．24％；声带瘫痪11例，占1．63％；喉乳头状瘤38例，占5．6％；喉异物12例，占1．78％，先天性喉室带发音困难6例，占0．89％；先天性喉蹼8例，占1．19％；会厌囊肿4例，占0．59％；声带肿块3例，占0．44％；声带息肉6例，占0．89％；喉真菌感染1例，占0．14％；喉结核3例，占0．45％。全组依据不同病因采取相应治疗，疗效满意，好转及治愈率为92．26％。结论儿童声嘶病因复杂，应采取针对性治疗措施，慢性喉炎、声带小结与喉软骨软化症是引起儿童声嘶的主要病因。     

显微外科为主的综合治疗对小儿髓母细胞瘤预后的影响

目的 分析显微手术辅以放疗、化疗对小儿髓线细胞瘤治疗效果及其预后的影响。方法 将髓母细胞瘤患儿分为二组。手术全切除组和手术未全切除组。前才术后只接受全神经轴放射治疗，后者术后辅以放疗和化疗，观察二组治疗效果和预后的差异。结果 手术全切除组术后肿瘤复发率２９．３％，１、２、５年生存率分别为１００％、９４．１％、８２．４％；手术未全切出生率且术后肿瘤复发率８５．７％、１、２、５年的生存率分别为７１．４     

综合疗法治疗复发性儿童喉乳头状瘤

目的 探讨手术切除联合局部注射麻疹疫苗及肌肉注射α—干扰素的综合治疗方法对儿童复发性喉乳头状瘤（JRLP）的疗效。方法将46例喉乳头状瘤患儿分为两组，其中综合治疗组26例，均在支撑喉内镜下采用喉旋切技术治疗，并在肿瘤基底部注射麻疹疫苗（1支麻疹疫苗用注射用水1 mL溶解，0.3 mL/次），自术后第15天开始肌肉注射α—干扰素，每次100万单位，隔日1次，疗程3个月。对照组20例，均经手术切除肿瘤。结果对所有患儿随访1年，综合治疗组26例中，有效20例；对照组20例中，有效8例。结论手术切除联合局部注射麻疹疫苗及肌肉注射αα—干扰素，是儿童复发性喉乳头状瘤的有效治疗方法，较单纯手术治疗疗效更好。     

Meningococcal disease in Dallas County, Texas: results of a six-year population-based study

OBJECTIVE: Neisseria meningitidis is an important cause of serious bacterial infection in children and adults in the US. From 1992 to 1997 invasive disease caused by N. meningitidis was studied among 1.9 million residents of Dallas County, TX METHODS: The demographic characteristics and diagnoses of 151 patients were identified through active, population-based surveillance and review of medical records. Serogroups were determined for strains infecting 129 (85%) patients. RESULTS: The average annualized incidence rate was 1.3 cases per 100,000 person years and was highest for children <1 year (13 cases/100,000 person years). Older patients (50+ years old) were more likely to present with pneumonia and less likely to present with meningitis than younger patients. Neither the fatality rate nor the duration of hospitalization for surviving patients was associated with age. Among patients with a known serogroup, serogroup C disease was found in 35% of cases <1 year old, 64% of those 1 to 49 years old and 44% of those 50+ years old. Serogroup B strains were isolated from 26% of patients <1 year, 17% of patients 1 to 49 years old and none of the patients 50+ years old. Serogroup Y disease increased from 22% to 35% of cases between 1992 and 1997 (P = 0.03). This serogroup was identified in 26% of patients <1 year old, 17% of patients 1 to 49 years old and in 50% of patients 50+ years old. Serogroup C and Y accounted for 61% of cases in children <1 year old and for 79% of cases in all age groups. CONCLUSION: The results underscore the importance of conjugate vaccines for serogroups C and Y.     

Age-specific characteristics of brain death in children

Clinical and neuropathologic characteristics of 45 children who met criteria for brain death were analyzed. Children between 2 months and 1 year of age were compared with children older than 1 year and children older than 5 years. The observation period to fulfill brain death criteria was not different between the age groups. Deep tendon and spinal reflexes were preserved significantly less frequently in children younger than 1 year old. Diabetes insipidus and the necessity of inotropic support were significantly more frequent in children older than 5 years. Fifty-eight percent (26/45) of patients had no cerebral perfusion pressure before death. However, 18% (8/45) of patients never had a cerebral perfusion pressure below 40 mm Hg. No relationships could be shown between the clinical or physiologic factors and neuropathologic findings. We found no support for using different brain-death criteria for children between 2 months and 1 year of age.     

声门下囊肿5例

目的:总结儿童声门下囊肿的临床特征及诊疗经验。方法:对2018年4月至2019年6月山东大学齐鲁儿童医院确诊的5例声门下囊肿患儿的临床资料进行回顾性分析。结果:5例患儿中2例为早产儿,3例为足月儿,均有气管插管史。1例一直无症状,其余4例有喉鸣,其中2例伴声嘶、呼吸困难,曾被误诊为喉软化症、喉炎等。支气管镜检查见声门下...     

Chronic duodenal ulcer in children: clinical observation and response to treatment

Chronic duodenal ulcer is diagnosed more frequently in children since recent advances in panendoscopy techniques. From 1976 to 1986, chronic duodenal ulcer was diagnosed in 33 children in this hospital. There was a marked male preponderance (male: female = 4.5:1). Family history for peptic ulcer was positive in 36% of the patients. The important element of emotional stress was identified in 39% of the patients. Thirty patients received medical treatment, and 26 were observed for more than 1 year (1-10 years). In 11 children, medication was changed because of symptom recurrence and change in the mode of treatment. The patients were divided into three groups with different treatment regimens: antacid (12 cases), cytoprotective agent (sucralfate, 17 cases), and histamine H2-receptor antagonist (cimetidine, eight cases). The response to short-term (eight-week) therapy was similarly good (83-94%) in the three treatment groups. However, sucralfate is superior to antacid in the ongoing maintenance therapy of chronic duodenal ulcer in childhood. Because chronic duodenal ulcer recurred frequently, it is crucial to give long-term maintenance therapy.     

Disorders of phonation in children

Vocal disturbances in children are surprisingly common. Typical pathologic conditions differ, however, from those that are seen commonly in adults. Disturbances in the vibratory characteristic of the vocal folds cause dysphonia. The most common causes for dysphonia are infectious, anatomic, congenital, inflammatory, neoplastic, neurologic, or iatrogenic in nature. A child who presents with hoarseness demands a rapid and thorough assessment. Fine points during the history may provide the clinician with clues as to the correct etiologic category. Proper treatment requires an accurate diagnosis. Although some conditions are similar in the adult larynx, treatment of the pediatric larynx often differs. An understanding of the changing and growing pediatric larynx is necessary for treatment and satisfactory results.     

儿童Rathke囊肿的临床特点及显微外科治疗

目的分析、探讨儿童Rathke囊肿的临床特点及其显微外科治疗。方法回顾性分析了我院1993年6月至2004年3月间经手术及病理证实的20例儿童Rathke囊肿病例。患儿的主要临床表现为内分泌异常、视觉障碍、头痛。所有患儿均接受了显微外科手术治疗，其中经蝶手术3例，经额开颅17例；全切8例，近全切5例，大部切除7例。结果本组患儿术后随访5个月至8年，绝大部分患儿症状消失或改善，且均无复发。其中，内分泌异常表现明显减轻或消失者14例，占(14／18)77．8％；头痛症状消失者10例，占(10／12)83．3％；视觉障碍患儿的视力均得到改善。结论儿童Rathke囊肿术前确诊困难，影像学检查无特异性。对于儿童症状性Rathke囊肿，显微手术可明确诊断、改善症状，患儿预后均良好。显微外科手术是治疗本病的有效方法。     

Renal transplantation: Experience in Australia

Objective : Outcome of renal transplantation in children under the age of 15 years, who received a renal allograft at The New Children’s Hospital between January 1983 to June 1997 was studied.Methods : Retrospective review of patients records and access to data collected from Renal Registry of the Hospital. 64 renal transplants were undertaken in 57 children during this period. Prednisone and Azathioprine were the mainstay of the immunosuppressive regimen up to 1983, then Cyclosporine A was introduced. Median age of first grafts was 10 years (range 1 month –14 years). There were 41 living related and 23 cadaveric grafts. 37 (64%) children had a congenital disorder as the cause of renal failure. Among them 14(37%) had congenital renal dysplasia/ hypoplasia, 20(36%) had primary glomerular disorder as the cause of renal failure.Results : Survival analysis at 12 month, 5 yr and 10 yr showed functioning grafts in 85%, 67% and 64% case respectively. Longest surviving transplant was 14 years. 6 children had died. Cumulative patient survival was 92.9% at 1 year, 90% at 5 year and 87% at 10 year. Sixteen primary grafts were lost with most common cause being chronic rejection accounting for 68% of all transplant lost. Recurrence of primary disease was the second most common cause of graft failure. There was one malignancy in this series. 25 of the 29 children of school age with functioning transplant attended school full time in a class appropriate for their age and nine of 13 years older patients were working full time, two worked part time.Conclusion : Renal transplantation is a successful treatment of end stage renal failure in children with high survival and normally functioning life. Chronic rejection remains a major cause of graft loss.     

先天性血管环99例病例系列报告

目的 探讨先天性血管环（CVR）的临床表现、诊断及预后的相关因素。方法 回顾性收集2006年1月至2015年12月重庆医科大学附属儿童医院确诊CVR病例,从病史中截取CVR分型、临床表现、影像学检查和治疗信息;根据出我院时的预后分为存活组和死亡或放弃治疗组,行预后不良的危险因素分析。结果 99例CVR患儿进入本文分析。男55例,女44例,中位年龄7.3月龄。肺动脉吊带（PAS）51例,双主动脉弓（DAA）32例,右主动脉弓+左主动脉导管/韧带15例,左主动脉弓+右迷走锁骨下动脉1例。入院时仅呼吸系统表现51例（51.5%）,仅心血管系统表现8例（8.1%）,仅消化系统表现2例（2.0%）,呼吸+心血管系统表现33例（33.3%）, 5例无临床症状。CVR+心内畸形64例（64.6%）,其中单纯房间隔缺损(ASD) 31例、室间隔缺损(VSD) 2例,ASD+VSD 9例,CVR+其他复杂心脏畸形22例。气管狭窄69例,食管狭窄14例,喉软骨发育不良10例,先天性支气管肺发育不良9例。超声心动图诊断CVR 54/99例（57.4%）,CT血管造影诊断CVR 87/95例（91.6%）,超声心动图或CT血管造影未诊断病例均通过CT血管造影或超声心动图诊断;26例行纤维支气管镜检查均发现气道狭窄;14/17例（82.3%）行钡剂食管造影检查示食管受压扭曲、狭窄。手术治疗57例,其中死亡4例,术后脱机困难放弃治疗6例;内科治疗42例,1例死亡,10例脱机困难放弃治疗。死亡或放弃治疗患儿的PAS比例(17/21 vs 34/78)、年龄(6.2 vs 15.5月龄)及合并肺发育不良（6/21 vs 3/78）显著高于存活组。结论 超声心动图联合CTA检查可提高CVR诊断率;PAS、年龄、合并肺发育不良是CVR患儿预后不良的危险因素。     

Measuring developmental deficit in children born at gestational age less than 26 weeks using a parent-completed developmental questionnaire

AIM: To assess developmental deficit in children born at gestational age (GA) < 26 wk using a parental questionnaire and to use regression analysis to study a cohort born in 1999-2003. PATIENTS AND METHODS: Three groups were studied: group 1, GA < 26 wk; group 2, GA 26-27 wk; group 3, children born at term. The Ages & Stages Questionnaire (ASQ) was used. The parents of each child were mailed an age-specific questionnaire between November 2004 and April 2005. The term children were used as a reference to calculate a standard deviation score (ASQ-SDS) for each child in the two preterm groups. RESULTS: Seventy-five per cent of the questionnaires were returned (group 1: n=61; group 2: n=57; group 3: n=72). The age at scoring ranged from 12 to 60 mo (mean 32.8 mo). After correction for parental education, 22% of the children born at GA < 26 wk and 13% of those at GA 26-27 wk had an ASQ-SDS below -2. Chronic lung disease of prematurity was associated with developmental deficit (mean difference -1.1 ASQ-SDS, p=0.004). CONCLUSION: The ASQ identified a significant developmental deficit in the children born extremely preterm. The rate of 22%, however, in children born at GA < 26 wk is reassuring.     

新生儿喉喘鸣病因分析16例报告

目的总结新生儿喉喘鸣的发生原因,以减少新生儿喉喘鸣的误诊。方法分析2004年4月至2005年12月我院新生儿科住院的全部喉喘鸣患儿（16例）的临床资料。全部病例均完成了血清总钙及离子钙的测定、胸部X光片、喉部及肺部CT扫描检查,部分病例进行了喉镜检查、上消化道造影及染色体检查等。结果本组16例患儿院外均诊断为先天性单纯性喉喘鸣;人院后16例中5例（31％）经喉部CT扫描诊断为甲状舌管囊肿（其中2例经喉镜检查已疑似为本病）,1例（6％）经染色体检查证实为猫叫综合征,2例（13％）依据临床症状及体征诊断为Pierre—Robin综合征,其余8例（50％）拟诊为先天性单纯性喉喘鸣;院外诊断与人院后诊断相比误诊50％;全部病例血清总钙及离子钙水平均在正常范围内;6例行上消化道造影的患儿,5例诊断为胃食管反流。结论新生儿喉喘呜非常容易误诊,必须排除引起喉喘鸣的其他病理因素（如占位病变,先天性综合征等）方可拟诊先天性单纯性喉喘呜;新生儿甲状舌管囊肿是新生儿喉喘鸣的常见原因之一;建议将喉镜检查作为喉喘鸣患儿的常规筛选手段,有条件者可进行喉部CT扫检查。     

左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。     

Gastroösophagealer Reflux und Atemwegserkrankungen

Gastro-oesophageal reflux is frequently implicated in pulmonary symptoms, but such a causal connection has not been demonstrated except in a subgroup of the paediatric patients affected. For example, children with mental retardation and pharyngeal dysfunction can develop pulmonary symptoms as a result of aspiration. Even otherwise healthy children can present with hoarseness or other laryngeal complaints as a result of gastro-oesophageal reflux. For the substantial group of children with chronic cough and bronchial asthma, in contrast, a connection with gastro- oesophageal reflux is likely only in isolated cases. In these children it is important to check for concomitant intestinal disorders before further investigations are initiated, which can be stressful for the young patients. The operative approach to the treatment of gastro- oesophageal reflux has lost ground to conservative treatment since the introduction of proton pump inhibitors as the first step in therapy.     

Laryngeal foreign bodies: management in children in Senegal]

Inhalation of foreign bodies is a frequent accident in children. It remains severe in the case of laryngeal foreign bodies. PATIENTS AND METHODS: Retrospectively, for a 16-year period, 65 laryngeal foreign bodies have been treated (44.8%), among 145 cases of airway foreign bodies, in the ENT department of Dakar University hospital. Etiological, clinical and therapeutic aspects were reviewed. RESULTS: Average age was 36 months, with a sex-ratio of 2.42 in favour of males. The time lag (time between the accident and admission to the department) was particularly long; 73.33% of the children were admitted more than 24 hours after the event. Eighty-three percent of the patients presented greater or lesser laryngeal dyspnea. Tracheostomy was performed in 55.4% of the patients. Average duration for abiation of the canula was ten days. Three cases of death were recorded (4.16%). DISCUSSION: The frequency of 44.8% for laryngeal localization of foreign bodies appears to be the highest in the literature. If the appropriate treatment for foreign bodies in the respiratory tract is endoscopic removal, the tracheostomy nevertheless occupies a central place in the management of the disease. This procedure may be recommended to all ENT specialists working in similar conditions. In spite of its inherent complications, tracheostomy allows reduction of mortality in relation to laryngeal foreign bodies. Improvement of prognosis requires prevention based on widespread public information and improving technical infrastructures.     

超细电子鼻咽喉镜在诊断婴幼儿咽喉疾病中的应用

目的 探讨电子鼻咽喉镜在婴幼儿咽喉疾病诊断中的应用价值.方法 采用Pentax-1130型超细电子鼻咽喉镜对121例喉喘鸣伴有不同程度吸气性呼吸困难或声音嘶哑的婴幼儿(包括新生儿)进行了检查.结果 121例患儿中喉软化症66例(54.5%),咽喉部先天性囊肿29例(24.0%),(2.5%),咽后肿物、喉纤维瘤、声门型喉蹼、下咽异物和气管狭窄各1例(0.8%).结论 121例伴有不同程度吸气性呼吸困难的喉喘鸣患儿中,除喉软化症以外,有近一半(45.5%)的患儿经过电子鼻咽喉镜检查在咽喉部发现了器质性病变.超细电子鼻咽喉镜在诊断婴幼儿及新生儿咽喉疾病中具有很大优势,随诊随查,不必预约,不需要镇静,可在自然呼吸状态下检查,并发症少,图像清晰,结果可靠,在临床上可推广使用.     

357例儿童结肠镜检查回顾性研究

目的探讨结肠镜检查在儿科应用的临床价值及安全性,了解不同年龄段儿童结肠镜检查原因及检出肠道疾病分布情况。方法收集2018-01-01—2020-12-31中国医科大学附属盛京医院0～14岁于内镜中心接受肠镜检查的患儿临床资料并行回顾性分析。结果 357例患儿共计进行399次结肠镜检查,94.5%(377/399)能够完成全结肠检查,219例(61.3%)肠镜下有阳性发现,357例结肠镜检查无1例出现严重并发症。<3岁组发现疾病18例,最主要疾病为克罗恩病7例(36.8%); 3～6岁组检出疾病46例(90.2%),占比最大为结肠息肉(56.9%);>6～14岁组儿童共发现疾病155例(54%),以炎症性肠病检出率最高(19.2%)。结论儿童结肠镜检查安全有效、诊断率高,具有重要的临床诊疗价值。腹泻、便血为0～6岁患儿进行结肠镜检查的主要原因,>6～14岁患儿实施结肠镜检查的原因主要依次为腹痛、腹泻;结直肠息肉是3～6岁儿童结肠镜发现主要病变,0～3岁、>6～14岁患儿以炎症性肠病为主要发现疾病。     

Immune deficiencies in chronic intestinal pseudo-obstruction

Aim: Chronic intestinal pseudo-obstruction has been associated with urinary disorders, myopathy, and ophthalmoplegia in adults and cholelithiasis in children. We observed a high percentage of total-parenteral-nutrition-dependent patients with pseudo-obstruction and recurrent infections requiring gammaglobulin infusions. Methods: AH records for 23 children with chronic intestinal pseudo-obstruction (10 females and 13 males, mean age 9.8 y ± 4.9 y, range 4–24 y) referred for a nutritional evaluation from 1992 to 1995 were reviewed. Chronic intestinal pseudo-obstruction was diagnosed by clinical, radiographic findings and antroduodenal manometry. Intestinal full-thickness biopsies were performed in seven children. Results: Hypogammaglobulinemia was diagnosed in 18 patients (78%): 16 patients had various immunoglobulin deficiencies and 2 had selective antibody deficiency. Intravenous gammaglobulin was administered in 14 patients. Other medical conditions affecting the children are summarized as follows: autonomic dysfunction in 10 patients (43%), recurrent hypoglycemia in 9 (39%), asthma in 9 (39%), cholecystitis in 7 (30%), low serum carnitine level in 6 (26%), urinary dysfunction in 6 (26%), pancreatitis in 5 (22%), behavioral problems in 5 (22%), myopathy in 2 (9%), idiopathic thrombocytopenia in 2 (8%), velopharyngeal insufficiency in 1 (4%), oculocutaneous albinism in 1 (4%), Pierre-Robin syndrome in 1 (4%), and protein C deficiency in 1 (4%). Munchausen syndrome was suspected in two patients. Conclusions: Chronic intestinal pseudo-obstruction appears to be associated with immune deficiencies. It is unclear if the immune deficiencies, intestinal pseudo-obstruction, and the other medical conditions have a common underlying etiology. Repeated infections may be due to impaired immune function in children with chronic intestinal pseudo-obstruction. We recommend screening for immune deficiencies in children with chronic intestinal pseudo-obstruction.     

Cardiovocal syndrome in infancy

Chronic hoarseness is a rare presenting or accompanying sign of congestive heart failure in infancy. Two infants had a paralyzed left vocal cord, secondary to traction on their left recurrent laryngeal nerve. Both had significant underlying congenital heart disease (type 2 aortopulmonary window, ventricular septal defect, patent ductus arteriosus; large ventricular septal defect). After surgical repair of their congenital heart defect, both infants had normal vocal cord function. Direct laryngoscopy is necessary to diagnose the cause of persistent hoarseness in infancy. If a paralyzed left vocal cord is found, a comprehensive cardiovascular diagnostic work-up is warranted.