不同剂量促肾上腺皮质激素治疗婴儿痉挛的疗效及不良反应

目的:比较不同剂量促肾上腺皮质激素(ACTH)治疗婴儿痉挛(IS)的疗效及不良反应,为临床提供参考。方法:收集2012年1月至2015年12月在我院住院接受ACTH治疗的60例IS患儿的临床资料,采用随机数表法分为小剂量组和大剂量组各30例。小剂量组的治疗方案:ACTH 1 U/(kg·d)加入60 mL 5%葡萄糖溶液中持续泵入6 h,连用2周,治疗有效,再用2周;治疗无效,加量至25 U/d,再用2周,总疗程4周。大剂量组的治疗方案:ACTH 起始剂量25 U/d,连用2周,治疗有效,再用2周;治疗无效,加量至40 U/d,再用2周,总疗程4周。两组患儿均在ACTH治疗结束后复查脑电图(EEG),并改为口服泼尼松,开始剂量为2 mg/kg,移行减量,总疗程为3个月。结果:大剂量组和小剂量组痉挛发作完全控制率分别为53.33%、46.67%,治疗有效率分别为23.33%、26.67%,两组比较差异均无统计学意义(P均>0.05);但小剂量组高血压、肥胖、易激惹、睡眠障碍发生率均低于大剂量组(P均<0.05)。结论:小剂量ACTH治疗IS疗效与大剂量ACTH相当,但不良反应较少。     

健儿止泻颗粒联合蒙脱石散治疗小儿腹泻的临床研究

目的探究健儿止泻颗粒联合蒙脱石散治疗小儿腹泻的临床疗效。方法选取2015年6月—2017年3月新疆医科大学第一附属医院收治的腹泻患儿102例为研究对象,采取随机数表法将患儿分为对照组和治疗组,每组各51例。对照组口服蒙脱石散,年龄1岁:1/3袋/次,年龄1~3岁:1/2袋/次,年龄≥3岁:1袋/次,3次/d。治疗组在对照组基础上开水冲服健儿止泻颗粒,年龄1岁:6 g/次,年龄1~5岁:6~12 g/次,年龄≥5岁:12~18 g/次,3次/d。两组患儿均连续治疗7 d。观察两组的临床疗效,比较两组的临床症状改善时间和免疫功能指标。结果治疗后,对照组和治疗组的总有效率分别为92.16%、100.00%,两组比较差异有统计学意义(P0.05)。治疗后,治疗组止泻时间、止吐时间、腹痛改善时间、退热时间、脱水纠正时间均短于对照组,两组比较差异具有统计学意义(P0.05)。治疗后,两组免疫球蛋白A(Ig A)、免疫球蛋白G(Ig G)、CD~(4+)/CD~(8+)水平均显著升高,同组治疗前后比较差异有统计学意义(P0.05);且治疗组这些观察指标明显高于对照组,两组比较差异具有统计学意义(P0.05)。结论健儿止泻颗粒联合蒙脱石散治疗小儿腹泻具有较好的临床疗效,可改善临床症状,调节免疫指标,安全性较好,具有一定的临床推广应用价值。     

抗菌功能性敷料联合糠酸莫米松乳膏治疗小儿湿疹的疗效观察

目的探讨抗菌功能性敷料联合糠酸莫米松乳膏治疗小儿湿疹的临床疗效。方法选取北京市海淀医院2016年1月—2017年7月接受治疗的92例小儿湿疹患者为研究对象,随机分为对照组和治疗组,每组各46例,对照组患儿涂抹糠酸莫米松乳膏,0.1 mg/次,每晚1次。治疗组患儿在对照组的基础上外敷抗菌功能性敷料,0.1 mg/次,1~3次/d。两组患者均连续治疗14 d。治疗后,观察两组的临床疗效,比较两组治疗前后临床症状、皮损程度评分和不良反应及复发情况。结果治疗后,对照组和治疗组的总有效率分别为84.78%、97.83%,两组总有效率比较差异有统计学意义(P0.05)。治疗后,两组临床症状和皮损程度评分均显著降低,同组治疗前后差异有统计学意义(P0.05);治疗后治疗组临床症状和皮损程度评分均显著低于对照组,两组比较差异有统计学意义(P0.05)。对照组和治疗组的复发率分别为19.57%、4.35%,两组复发率比较差异具有统计学意义(P0.05)。结论抗菌功能性敷料联合糠酸莫米松乳膏治疗小儿湿疹疗效显著,可明显改善患儿临床症状,安全性较高,具有一定的临床推广应用价值。     

Nephrotic syndrome in the 1st year of life

Among the various primary conditions which may be associated with a nephrotic syndrome at birth or within the 1st year of life, the best known is the congenital nephrotic syndrome of finnish type (CNF) characterized by irregular pseudocystic dilatation of proximal tubules. This disease, very frequent in Finaland, is often familial with an autosomal recessive mode of inheritance. Patients are steroid resistant, but the cause of death is usually not uraemia but infection or severe diarrhoea with electrolyte imbalance. The second condition is idiopathic nephrosis including minimal change disease, diffuse mesangial proliferation and focal segmental glomerular sclerosis. As opposed to CNF, infants with early onset nephrosis may respond to steroid therapy as older children do and may even recover. However, there are no histopathological criteria which allow the certain differentiation of idiopathic nephrosis from CNF. The third condition is diffuse mesangial sclerosis (DMS), a clinicopathological entity which can occur as an isolated finding or be associated with male pseudohermaphroditism and/or Wilms' tumour (Drash syndrome). From a morphological point of view, DMS is easy to differentiate from CNF because of the characteristic pattern of involvement of the glomeruli. From a clinical point of view, the nephropathy, almost always characterized by a nephrotic syndrome, has two distinct features: it is most often diagnosed in the first 2 years of life and it progresses rapidly to end-stage renal failure, which usually occurs before the age of 3 years. The clinical findings in 36 patients with DMS are presented. The nephropathy was isolated in 22 infants and associated with male pseudohermaphroditism and/or Wilms' tumour in 14. The early onset of the nephropathy, its familial incidence and its association with Drash syndrome suggests an antenatal dysgenetic process. Recent studies indicate a direct role of WT1 (a Wilms' tumour gene) in the aetiology of Drash syndrome, and mutations of this gene have been found in 3 patients herein reported. It remains to be demonstrated whether or not the WT1 gene is also altered in patients with isolated DMS.Presented at the Festschrift for Professor R. H. R. White on March 6, 1992, Birmingham, UK     

An ethological approach to autism: an analysis of visual behaviour and interpersonal contact in a child versus adult interaction

Eighteen children classified into 4 diagnostic categories were compared based on their behaviour in a child-adult interaction. The categories consist of autistic children with full syndrome, autistic children in a residual state, hyperactive children, and mentally retarded children. An ethological analysis of the interpersonal distance, the amount of physical contact, and the direction of the look/gaze was carried out to describe some behavioural aspects of infantile autism. The autistic children with full syndrome were found to be different from the other categories by a relatively close interpersonal distance, a high frequency of physical contact, and a low frequency of "look at face".     

Clinical and cerebral angiographic evolutions of idiopathic progressive occlusive disease of the circle of Willis ("moyamoya" disease) in children

In 33 pediatric cases of idiopathic progressive occlusive disease of the circle of Willis (so-called moyamoya disease), clinical features and CAG findings were studied with emphasis on their evolutionary aspects. The subjects were clinically classified as follows; 23 cases of pure transient ischemic attacks (TIA), one presenting purely with infarct, one mixed TIA and epileptic, two mixed TIA + infarct, and 6 mixed epileptic and infarct. The core symptom of the TIA type is a recurrent unilateral or alternating unilateral paresis persisting for minutes or hours. Frequently hyperpnea provoked a TIA. Follow-up observation of the TIA type patients for an average of 6 yrs 11 mos shows that about half the subjects became completely free of symptoms and the other half mostly improved considerably. In contrast, all patients not presenting purely with TIA ("non-TIA" type) still had persistent neurological deficits after a mean interval of 6 yrs 4 mos from the onset. The CAG findings in most cases belonged to stage III according to Suzuki's classification. Therefore the authors subdivided stage III into 3 substages, IIIa, IIIb and IIIc. The longer the duration of the disease up to the CAG examination, the more advanced was the CAG stage. Repeat CAGs examined after a mean interval of 2 yrs 11 mos demonstrated the same stage in 32%, and progress by one or two stages in 55% and 14% of the sides examined, respectively.     

Thirty‐two Japanese cases of infantile hemangiomas treated with oral propranolol

Infantile hemangiomas undergo rapid growth during early infancy followed by gradual involution. Infantile hemangiomas sometimes impair vital functions or cause disfigurement. Thirty‐two Japanese patients between the ages of 1 and 4 months with proliferating infantile hemangiomas received oral propranolol on an outpatient basis. The success rate (complete or nearly complete resolution) at week 25 was 56% (18/32). Two patients dropped out because of a personal reason and moving out. Recurrence after termination of treatment was seen in six patients. Adverse events occurred in 16 patients. There were no adverse events on day 1 (initiation of treatment at a dose of 1 mg/kg per day) and day 8 (dose increase to 2 mg/kg per day). One patient was hospitalized due to pneumonia, and suspended propranolol for 26 days. Oral propranolol at 2 mg/kg per day is effective and safe in Japanese patients with infantile hemangiomas.     

Infant oral mutilation in East Africa – therapeutic and ritual grounds

This paper reviews the practice and ritual traditions of infant oral mutilation, drawing on a literature search in PubMed and Google Scholar, historical reports, relevant textbooks, NGO materials and personal observations of the authors.     

婴儿眼球震颤综合征的治疗进展

婴儿眼球震颤综合征(infantile nystagmus syndrome,INS)是一种在生后早期出现的病理性眼球震颤,以双眼非自主性共轭眼球运动为临床特征,会破坏双眼视力及视功能,同时是引起弱视、斜视以及斜颈的原因之一.目前尚无治愈INS的方法,但治疗方法有许多进展,如视光学治疗采用框架眼镜、角膜接触镜、三棱镜、低视力助视器及激光,药物治疗采用碳酸酐酶抑制剂、肉毒素眼外肌注射等,生物反馈治疗采用听觉反馈等,以及新的手术方法.