小儿喉乳头状瘤HPV-DNA及体液免疫检测

目的：探讨小儿喉乳头状瘤（ＪＬＰ）人乳头瘤病毒（ＨＰＶ）感染途径及发病机理。方法：采用ＰＣＲ及ＰＣＲ产物斑点杂交技术检测ＪＬＰ组织ＨＰＶ－ＤＮＡ;散射免疫比浊法测定血清Ｉｇ及补体Ｃ３。结果：ＪＬＰ组织ＨＰＶ总感染率为９５％（１９／２０）,其中ＨＰＶ。型为５５％（１１／２０）,ＨＰＶ１１为３０％（６／２０）,ＨＰＶ６＋１１型为１０％（２／２０）;ＪＬＰ患者血清ＩｇＧ、ＩｇＡ、ＩｇＭ、Ｃ３值正常,对照     

Munchausen syndrome by proxy and factitious illness: Symptomatology,parent-child interaction,and psychopathology of the parents

The term Munchausen syndrome by proxy is used to diagnose children presenting symptoms of an organic disorder resulting from manipulations initiated by their caretakers. Even in early infancy it happens that injuries are induced, and that drugs, poisons or medicine are administered in order to provoke and feign clinical symptoms of severe diseases. Exact data on prevalence are not available but it is obvious that Munchausen syndrome by proxy is a rare psychiatric disorder. There is a body of evidence that Munchausen syndrome by proxy is nothing but the extreme of a broader clinical entity for which the term factitious illness has been introduced. In this group children are included whose mothers invent a history of disease in order to produce symptoms without actually damaging their children. It is not well established whether such a distinction is necessary and whether there are differences in long-term outcome. Onset of symptoms is as early as three weeks up to twelve years, and mean age of diagnosis according to a more comprehensive study is 3 1/4 year. The estimated mortality rate of children with Munchausen syndrome by proxy is 9 percent. In three of the four cases of children reported here clinical presentations were dominated by symptoms of central nervous disorders. All mothers showed unsure and inconsistent parental behaviour and inefficient coping. None of them received support from their partners, if present. In interaction the children always wanted to dominate their mothers. The high amount of personality disorders observed in the caretakers might be the reason for the often reported failure of psychotherapeutic interventions.

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Zusammenfassung Die Diagnose Münchhausen Stellvertreter Syndrom wird bei Kindern gestellt, die Symptome einer organischen Erkrankung zeigen, die durch Manipulationen von Eltern oder anderen Sorgeberechtigten hervorgerufen werden. Vielfach werden bereits im frühen Kindesalter den Kindern Verletzungen beigebracht, Drogen, Gifte oder Medikamente verabreicht, um die klinischen Merkmale schwerer Erkrankungen vorzutäuschen. Obwohl genaue Angaben zur Häufigkeit fehlen, kann man insgesamt von einer seltenen Störung ausgehen. Es gibt zahlreiche Hinweise dafür, daß es sich beim Münchhausen Stellvertreter Syndrom um die seltene, aber sehr schwere Ausprägungsform in einer größeren Gruppe von Störungen gleichartigen klinischen Bildes handelt, für die man den Begriff der vorgetäuschte Störungen geprägt hat. In dieser Gruppe werden auch die Kinder erfaßt, deren Mütter ausschließlich Symptome und eine zugehörige Krankheitsgeschichte erfinden, die aber keine körperlichen Eingriffe (einschließlich Drogen- und Medikamentenverabreichung) vornehmen, um organische Symptome zu simulieren. Unklar ist, ob eine solche Unterscheidung notwendig und hilfreich ist. Bisher fehlen Hinweise auf Unterschiede in der Prognose im Langzeitverlauf zwischen den Untergruppen. Die Symptomatik eines Münchhausen Stellvertreter Syndroms konnte bereits bei Kindern im Alter von 3 Wochen beobachtet werden und wurde noch bei 12jährigen gefunden. In einer größeren Studie wurde ein mittleres Alter von 3 3/4Jahren für den Zeitpunkt der Diagnosestellung ermittelt. Die Sterblichkeitsrate wird auf 9 Prozent geschätzt. Bei den vier Kindern, über deren Symptomatik hier berichtet wird, standen bei drei zentralnervöse Störungen im Vordergrund. Drei Mütter gaben zusätzlich den Verdacht des sexuellen Mißbrauchs ihrer Kinder an. Die motivationalen Aspekte lassen deudiche Unterschiede erkennen. Bei drei Müttern bestand der Wunsch nach Schutz des Kindes vor realem oder vermeintlichem sexuellem Mißbrauch des Kindes. Alle Mütter hatten mindestens eine gescheiterte eheliche Beziehung. Im Erziehungsverhalten wirkten sie unsicher, inkompetent und inkonsistent; von ihren Partnern, sofern vorhanden, erhielten sie keine erzieherische Unterstützung. In der Mutter-Kind Interaktion vermochten die Kinder über die Mütter zu dominieren. Der hohe Anteil an Persönlichkeitsstörungen bei den hier beschriebenen Sorgeberechtigten könnte der Grund für das häufig berichtete Scheitern psychotherapeutischer Interventionen sein.

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Résumé On parle de Syndrôme de Munchausen par procuration chez des enfants, qui présentent des troubles organiques résultant de manipulations de leurs responsables éducatifs. Des blessures, l'administration de médicaments, de drogues ou de poison — même chez des enfants très jeunes — ont pour effet de provoquer et simuler un tableau clinique sévère. Il s'agit d'un désordre psychiatrique rare — les chiffres exacts de prévalence sont difficiles à évaluer. Le Syndrome est décrit chez l'enfant à partir de l'âge de trois semaines jusqu'à 12 ans. L'âge moyen est de trois ans et quart. La mortalité chez les enfants porteurs d'un syndrome de Munchausen par procuration est estimé à 9%. Dans trois des quatre cas présentés le tableau clinique est dominé par des symptômes d'ordre neurologique. De manière supplémentaire trois mères rapportent un abus sexuel de leurs enfants. Les mères se caracterisent par leur incompétence et inconséquence éducative; souvent elles sont sans soutien éducatif de leur partenaire. Dans l'interaction mère — enfant les enfants ont un comportement dominant envers leur mère. La non-efficacité des interventions psychothérapeutiques peut être liée a un pourcentage élevé de troubles de la personnalité chez les parents.

     

Videosurgery in oncological pediatrics

As a recent innovation, videosurgery (VS) has enjoyed rapid and widespread development. While colon cancers in adults are quite commonly removed using laparoscopic techniques, children could also benefit from the improved diagnostic and interventional possibilities using VS techniques. From January 1992 to February 1994, we carried out 15 procedures. Eight thoracic operations were performed on 7 patients: diagnostic in 5 cases and metastasis resection in 3. Six patients underwent 7 laparoscopic operations for biopsies (5) or lymph-node harvesting (2). Based on this experience, we discuss the advantages of VS (noninvasive technique for primary or second-look exploration, guided biopsies, and removal of tumors) and its risks (spillage of malignant cells, subjective palpation) in the management of pediatric malignancies.     

Sleep problems and daytime behavior in childhood idiopathic epilepsy

PURPOSE: To evaluate the presence of sleep problems and their association with behavioral and adjustment problems in children with idiopathic epilepsy. METHODS: A parental questionnaire was used to assess sleep problems in 89 children with idiopathic epilepsy for comparisons with 49 siblings and 321 healthy control children, equally distributed for age and sex. Sleep problems were clustered into five factors: parasomnias, parent/child interaction during the night, sleep fragmentation, daytime drowsiness, and bedtime difficulties. Daytime behavior and psychological adjustment were assessed by means of the Child Behavior Checklist. Maternal distress and disturbance was evaluated by the Malaise Inventory. The better to identify factors associated with sleep problems in the children with epilepsy, multiple regression analysis was performed. RESULTS: Children with epilepsy showed significantly more sleep problems than did both siblings and healthy controls. Within the epileptic group, children with current seizures complained more of sleep problems than did the seizure-free children. Moreover, children with epilepsy showed more behavioral problems and maladjustment. Age, paroxysmal activity density, duration of illness, seizure frequency, and behavioral problems were significantly associated with sleep problems in the epileptic group. CONCLUSIONS: The results of this study in a highly selected sample pointed out the presence of sleep problems, and adjustment and behavioral problems in children with idiopathic epilepsy. The presence of epilepsy, although benign, in childhood is associated with adaptive problems of the child. From this point of view, the alteration of some sleep habits may be a sign of emotional maladjustment. Although parents failed to perceive them as a problem, our findings indicate that attention to sleep and behavioral problems is important in clinical management of children with idiopathic epilepsy.     

Conservative treatment of neurogenic bladder dysfunction

Summary In neurogenic bladder dysfunction conservative treatment is preferred whenever possible and preservation of renal function remains the main topic. Additionally, a patient-related form of bladder management with decreased intravesical pressure and social dryness is needed. Therefore many strategies are available and after evaluation of the underlying neurogenic bladder dysfunction an individual kind of treatment can be used. Possible options are the clean intermittent catherization, anticholinergic drugs, detrusor triggering, electrical stimulation and bladder emptying by Valsalva's manouvre.      

内耳畸形患儿的人工耳蜗植入

目的：探讨几种内耳畸形患儿的人工耳蜗植入效果。方法：对10例内耳结构异常的感音性耳聋患儿进行了人工耳蜗植入。结果：术后随访0．5～4．0年，10例基本达到了较满意的听觉言语恢复效果。结论：内耳畸形息儿行人工耳蜗植入，应严格进行术前听力学与影像学的评估，严格掌握手术的适应证，可以达到较满意效果。     

全身麻醉对小儿听性脑干反应测试结果的影响

目的　研究应用听性脑干反应 (auditorybrainstemreaction ,ABR)作为监测小儿全身麻醉深度与觉醒的客观指标。方法　选择听力正常的外科择期手术患儿 4 5例 ,按照美国麻醉学家学会表针分为Ⅰ～Ⅱ级 ,分别施行异丙酚静脉麻醉、芬太尼静脉麻醉及异氟醚吸入全身麻醉 ,随机每组 15例 ,应用丹麦Madsen诱发电位反应仪监测并记录麻醉各阶段ABR的Ⅰ、Ⅲ、Ⅴ波潜伏期及Ⅰ Ⅲ、Ⅲ Ⅴ、Ⅰ Ⅴ波间期 ,研究观察潜伏期和波间期随时间推移及麻醉剂量变化之间的关系 ,探讨ABR在异丙酚、芬太尼及异氟醚等不同麻醉中的表现特征和规律。结果　①异丙酚静脉麻醉和异氟醚吸入麻醉与剂量呈良好的正相关 ;②Ⅰ波的潜伏期特性对于控制麻醉深度极为重要 ;③Ⅴ波监测麻醉具有最佳的稳定性及相关性 ;④停用麻醉药一段时间或患儿基本清醒时 ,ABR各波潜伏期和波间期有的仍高于正常值 ,这是滞后 (延迟 )反应 ;⑤ABR对芬太尼术中的觉醒监测不太敏感。结论　ABR各波的潜伏期及波间期变化 ,可判断小儿全身麻醉深度 ,在一定程度上可作为判断觉醒的参考 ,但应考虑有延迟反应的可能     

小儿原发性扩张型心肌病预后因素探讨

目的 探讨小儿原发性扩张型心肌病的临床特点和预后因素。方法 对45例DCM病例进行回顾性分析，探讨影响预后的因素。结果 未显示不同年龄小儿在疗效上有所不同。心电图左室高电压或肥大预后较好，右室占优势或肥大预后欠佳。心律失常与预后无明显关系。左室收缩功能、射血分数越低、PEP／ET越大预后较差。用多普勒超声估计的肺动脉压与临床预后可能有一定的关系。结论 扩张心肌病一旦作出诊断，预后仍极差。     

Malignant fibrous histiocytoma of the lung in a child

We describe a 12-year-old patient with a primary pulmonary mass in the left upper lung. The diagnosis of inflammatory pseudotumour was suspected preoperatively. After pathological examination and complete clinical evaluation, a diagnosis of malignant primary pulmonary fibrous histiocytoma was established. This is a very uncommon primary neoplasm of the lung and to our knoweledge only five paediatric cases have been reported. Because of the rarity of these sarcomas and histological similarities to benign inflammatory pseudotumour, care must be taken to avoid confusion between the two disorders particularly in intra-operative frozen sections. Conclusion Primary malignant fibrous histiocytoma of the lung is an uncommon tumour that should be considered in the differential diagnosis of pulmonary neoplasms of childhood. The histological diagnosis can be difficult due to the similarities with␣inflammatory pseudotumour. Received: 29 May 1996 / Accepted: 21 July 1996