甲泼尼龙联合顺尔宁治疗重症毛细支气管炎的临床和诱导痰细胞学研究

目的 研究甲泼尼龙(MP)联合顺尔宁治疗重症毛细支气管炎(毛支)的疗效和治疗前后诱导痰细胞学的变化.方法 将64例重症毛细支气管炎患儿随机分为两组,治疗组32例联用MP和顺尔宁治疗,对照组32例用地塞米松治疗.分别观察两组的喘憋消失时间、肺部体征消失时间和住院天数.同时分析治疗组用MP和顺尔宁联合治疗前后的诱导痰细胞学变化.结果 与对照组相比,治疗组的喘憋消失时间、肺部体征消失时间和住院天数均明显缩短(P均＜0.01).经MP和顺尔宁联合治疗后,治疗组的嗜酸性粒细胞和肥大细胞的比例明显下降(P均＜0.001).结论 MP和顺尔宁联合治疗重症毛支的疗效优于常规治疗方法,同时对其诱导痰细胞学有显著影响.     

急性脑部炎症合并视觉障碍患儿22例临床特征及预后

目的 分析急性脑部炎症合并视觉障碍患儿临床特征及预后。方法 对2003年1月至2014年6月中国医科大学附属盛京医院小儿神经内科22例临床诊断为急性脑部炎症合并视觉障碍患儿的临床症状、实验室检查、视觉诱发电位、神经影像学特点等临床资料进行回顾性分析。结果 发病＜1周就诊入院的10例、1～2周8例、＞2～4周2例、＞4周2例。以视觉障碍首发就诊的7例。确诊为病毒性脑炎合并视神经炎9例,病毒性脑炎合并视神经乳头炎1例,病毒性脑炎及化脓性脑膜炎合并视觉障碍性质待定各1例,播散性脑脊髓炎合并视神经炎的2例,脑干脑炎合并暴露性眼炎的1例,病毒性脑炎合并右眼晶体点状混浊、双眼视网膜发育不良的1例,视神经脊髓炎1例,病毒性脑炎合并皮质盲1例,播散性脑脊髓炎合并视乳头炎、疑似小柳原田综合征1例,多发性硬化症可能性大3例。全部患儿均进行了视觉诱发电位检查,其中视觉传导通路异常18例。10例行眼底照相,结果异常7例。均行头磁共振（MR）平扫检查,结果异常10例,其中6例脑内病变范围广泛。18例行丙种球蛋白联合激素冲击治疗,其中出院时14例视觉障碍症状好转。出院后随访6个月至8年,20例预后较好、2例预后较差。结论 急性脑部炎症合并视觉障碍病因多样,除考虑常见病,如视神经炎、视神经乳头炎、视神经脊髓炎、脑血管病外,还应考虑到少见疾病,如皮质盲、小柳原田综合征等。早期行视觉诱发电位、眼底照相检查对于疾病诊断与鉴别有意义,积极治疗对改善预后有重要作用。     

血液净化治疗非肾脏疾病危重症患儿的临床分析

目的 总结血液净化(blood purification,BP)治疗非肾脏疾病危重症患儿的经验.方法 回顾性分析2009年1月至12月在我院PICU因非肾脏疾病而行BP治疗的10例危重症患儿的临床资料.10例患儿中,5例为急性肝能衰竭;2例自身免疫性疾病,其中1例吉兰-巴雷综合征,1例幼年特发性关节炎(全身型)并发MAS;2例重症脓毒症;1例由于代谢病出现持续性的严重代谢性酸中毒.结果 10例非肾脏疾病危重患儿中,实施CVVH联合PE治疗4例次,单纯CVVH治疗3例,单纯PE治疗3例.临床痊愈7例,好转1例,家长放弃2例.结论 BP作为一种脏器功能替代及支持的新疗法对PICU中肝功能衰竭及自身免疫性疾病等非肾脏病危重症患儿有较好的疗效.     

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目的对促肾上腺皮质激素(ACTH)治疗的婴儿痉挛(IS)患儿进行追踪随访,了解其预后并评价影响预后的因素。方法(1)对北京大学第一医院儿科1993年1月至2003年12月住院的53例ACTH治疗的IS患儿的临床随访资料进行回顾性研究。(2)ACTH治疗方法:采用ACTH静滴,25~40IU/d,共4周。结果(1)对53例ACTH治疗患儿随访6个月至10年,ACTH治疗发作完全缓解的31例,随访2年以上,共有16例复发,复发率为51·6%,其中于停用ACTH后2~3个月复发者占50·0%(8/16),复发形式以肌阵挛发作、痉挛发作、部分运动性发作为主;(2)临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关(病程≤2个月者、ACTH近期疗效好的临床发作预后好),与发病年龄、病因无关;(3)智力预后与ACTH开始治疗时的病程、病因、发作预后有关(病程≤2个月者、隐原性者、发作完全缓解者智力预后好),与发病年龄、ACTH近期疗效无关。结论(1)IS患儿临床发作预后与开始用ACTH治疗时的病程、ACTH的近期疗效有关。(2)智力预后与ACTH开始治疗时的病程、病因、发作预后有关。     

Severity of disease correlated with fever reduction in febrile infants

A prospective study of the effects of fever reduction on the clinical appearance of infants at risk for occult bacteremia was undertaken to study the hypothesis that infants with bacteremic illness fail to improve clinically following defervescence compared with infants with benign viral illness. A total of 154 children were enrolled in the study, including 19 with bacteremia: 13 with occult Streptococcus pneumoniae bacteremia, two with occult Haemophilus influenzae, type b bacteremia, and four with Haemophilus meningitis and bacteremia. There were no differences in degree of temperature reduction with acetaminophen between the bacteremic and nonbacteremic groups of infants. Among infants with bacteremia but without meningitis, differences from nonbacteremic children were detected in clinical appearance prior to fever reduction but not following defervescence. All patients with meningitis appeared seriously ill before and after defervescence. It was concluded that clinical improvement with defervescence is not a reliable indicator of the presence of occult bacteremia. Lack of clinical improvement with defervescence may be a reliable indicator for the presence of meningitis. Because there were differences in clinical appearance prior to fever reduction, routine administration of acetaminophen may interfere with the clinical evaluation by the physician.     

Relation between pancreatic lipase activity and gastric emptying rate in children with cystic fibrosis

OBJECTIVE: To determine whether abnormal gastric emptying is responsible for the inability of pancreatic enzyme replacement therapy (PERT) to normalize fat digestion in patients with cystic fibrosis (CF) who are pancreatic-insufficient.Study design Gastric emptying of a solid meal and pancreatic lipase function were assessed in 10 children with CF and 12 healthy control subjects with noninvasive breath tests using (13)C-octanoic acid and (13)C-mixed triglyceride, respectively. Lipase function was assessed in the subjects with CF with and without PERT. RESULTS: Without PERT, the lipase activity for the patients was less than that for the control subjects (P<.001); however, with PERT, 40% of the patients had a normalized lipase function. There were no differences between the mean gastric emptying rates of the patients with CF and the control subjects (P>.05), but there was a negative correlation between gastric half emptying time and percentage improvement in (13)C-mixed triglyceride results of the patients with CF with pancreatic enzymes compared with placebo (P<.05), with patients with slow gastric emptying having less improvement with PERT. CONCLUSIONS: The success of PERT in improving pancreatic lipase activity is reduced in patients with slow gastric emptying, which could explain the variations in improvement of fat digestion with enzyme supplementation.     

神经系统疾病儿童免疫接种策略

儿童神经系统疾病种类繁多、病情复杂,神经系统疾病儿童的免疫接种困难问题突出.文章从神经系统疾病儿童免疫接种现状、免疫接种相关的神经系统反应免疫接种风险及预后、免疫接种的禁忌证以及不同神经系统疾病儿童免疫接种策略五方面进行阐述,强调指出处于稳定期的神经系统疾病儿童可以接种各类疫苗,疫苗相关的神经系统反应极其罕见.     

Psychological functioning in boys of short stature: effects of different levels of growth hormone secretion

Aim: To examine the relationship between growth hormone (GH) and psychological functioning, especially self-perception and well-being, in 60 prepubertal boys of short stature with a wide range of GH levels. Methods: A comparison was made of the well-being and self-perception of children with GH insufficiency, children with idiopathic short stature (ISS), a normative sample and healthy boys with normal stature. Results: Children with GH insufficiency had a more negative perception of their own physical appearance than the normative sample. They perceived themselves as more alert but also more inhibited than both the children with ISS and the healthy boys with normal stature. In comparison with the healthy boys with normal stature they perceived themselves as having more stability. The parents of the boys with GH insufficiency also perceived their children as being more stable compared with how the parents of boys with ISS perceived their children. To elucidate the effects of GH on psychological functioning a multiple regression analysis was performed.

Conclusion: The lower the levels of GH the more inhibited were the boys of short stature, as perceived both by themselves and by their parents. The boys with GH insufficiency had a more negative perception of their physical appearance than the normative sample.     

血液净化治疗非肾脏疾病危重症患儿的临床分析

目的 总结血液净化(blood purification,BP)治疗非肾脏疾病危重症患儿的经验.方法 回顾性分析2009年1月至12月在我院PICU因非肾脏疾病而行BP治疗的10例危重症患儿的临床资料.10例患儿中,5例为急性肝能衰竭;2例自身免疫性疾病,其中1例吉兰-巴雷综合征,1例幼年特发性关节炎(全身型)并发MAS;2例重症脓毒症;1例由于代谢病出现持续性的严重代谢性酸中毒.结果 10例非肾脏疾病危重患儿中,实施CVVH联合PE治疗4例次,单纯CVVH治疗3例,单纯PE治疗3例.临床痊愈7例,好转1例,家长放弃2例.结论 BP作为一种脏器功能替代及支持的新疗法对PICU中肝功能衰竭及自身免疫性疾病等非肾脏病危重症患儿有较好的疗效.     

Serum levels of 25‐hydroxyvitamin D in mothers of Swedish and of Somali origin who have children with and without autism

Aim: To analyse serum levels of 25‐hydroxyvitamin D in mothers of Somali origin and those of Swedish origin who have children with and without autism as there is a growing evidence that low vitamin D impacts adversely on brain development. Method: Four groups of mothers were invited to participate; 20 with Somali origin with at least one child with autism, 20 with Somali origin without a child with autism, 20 of Swedish origin with at least one child with autism and 20 with Swedish origin without a child with autism. Two blood samples were collected from each individual; during autumn and spring. Results: Between 12 and 17 mothers from the different groups accepted to participate, both groups of mothers of Somali origin had significantly lower values of 25‐hydroxyvitamin D compared with Swedish mothers. The difference of 25‐hydroxyvitamin D between mothers of Somali origin with and without a child with autism was not significant. Conclusion: Our findings of low vitamin D levels in Somali women entail considerable consequences in a public health perspective. The observed tendency, i.e. the lowest values in mothers of Somali origin with a child with autism was in the predicted direction, supporting the need for further research of vitamin D levels in larger samples of Somali mothers of children with and without autism.     

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??Abstracts?? Objective To analyze the clinical characteristics and prognosis of acute encephalitis with visual impairment in children. Methods The clinical features?? including clinical manifestations??laboratory tests??Flash visual evoked potential and cranial MRI?? in 22 patients diagnosed with acute encephalitis with visual impairment in children were retrospectively analyzed. Results The disease course was less than one week in 10 cases. The disease course of eight cases was between one week and two weeks?? two cases were between two weeks and four weeks?? two cases were over four weeks. Seven cases had visual impairment as the first symptom. One case was with the diagnosis of viral encephalitis with cortical blindness. Nine cases were with the diagnosis of viral encephalitis with optic neuritis. One case was with the diagnosis of encephalitis with neuropapillitis. One case was with the diagnosis of disseminated encephalomyelitis with optic neuritis. Two cases were with the diagnosis of disseminated encephalomyelitis with optic neuritis. One case was with the diagnosis of brainstem encephalitis with exposure keratitis. One case was with the diagnosis of encephalitis with lens opacity and retinal dysplasia. One case was with the diagnosis of ophthalmoneuromyelitis. One case was likely with the diagnosis of Vogt-Koyanagi-Harada syndrome. In all the cases of Flash visual evoked potential??eighteen were abnormal. In the ten cases of fundus photography??seven were abnormal. In all the cases of cranial MRI??ten were abnormal and six suffered from extensive pathological changes in the brain. In the eighteen cases of hormone therapy??fourteen had good effects. The time of post hospitalization follow-up ranged from 6 months to 8 years. The prognosis of 20 patients was good. Two patients’ prognosis was bad. Conclusions There are different kinds of etiology and diseases in children who are diagnosed with acute encephalitis with visual impairment. Flash visual evoked potential and fundus photography are of important significance in the early stages. High doses of IVIG corticosteroids and hyperbaric oxygen therapy play a key role in improving the prognosis of disease.     

Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism

OBJECTIVES: To identify infants with hyperinsulinism caused by defects of the beta-cell adenosine triphosphate-dependent potassium channel complex and to distinguish focal and diffuse forms of hyperinsulinism caused by these mutations. STUDY DESIGN: The acute insulin response to intravenous calcium stimulation (CaAIR) was determined in 9 patients <20 years with diffuse hyperinsulinism caused by defective beta-cell sulfonylurea receptor (SUR1(-/-)), 3 patients with focal congenital hyperinsulinism (6 weeks to 18 months), a 10-year-old with insulinoma, 5 with hyperinsulinism/hyperammonemia syndrome caused by defective glutamate dehydrogenase (6 months to 28 years), 4 SUR1(+/-) heterozygotes with no symptoms, and 9 normal adults. Three infants with congenital focal disease, 1 with diffuse hyperinsulinism, and the child with insulinoma underwent selective pancreatic intra-arterial calcium stimulation with hepatic venous sampling. RESULTS: Children with diffuse SUR1(-/-) disease and infants with congenital focal hyperinsulinism responded to CaAIR, whereas the normal control group, patients with hyperinsulinism/hyperammonemia syndrome, and SUR1(+/-) carriers did not. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling revealed selective, significant step-ups in insulin secretion that correlated anatomically with the location of solitary lesions confirmed surgically in 2 of 3 infants with congenital focal disease and in the child with insulinoma. Selective arterial calcium stimulation of the pancreas with hepatic venous sampling demonstrated markedly elevated baseline insulin levels throughout the pancreas of the infant with diffuse hyperinsulinism. CONCLUSIONS: The intravenous CaAIR is a safe and simple test for identifying infants with diffuse SUR1(-/-) hyperinsulinism or with focal congenital hyperinsulinism. Preoperative selective arterial calcium stimulation of the pancreas with hepatic venous sampling can localize focal lesions causing hyperinsulinism in children. The combination of these calcium stimulation tests may help distinguish focal lesions suitable for cure by local surgical resection.     

Anaerobic bacteremia in a neonatal intensive care unit: an eighteen-year experience

A review of anaerobic bacteremia in the Neonatal Intensive Care Unit identified 29 episodes of clinically significant bacteremia occurring over the past 18 years. This experience suggested that certain clinical settings were associated with specific anaerobic infections. Although Gram-positive and Gram-negative anaerobes were isolated with similar frequency, 8 of 12 infants bacteremic within the first 48 hours of life were infected with Gram-positive, penicillin G-susceptible organisms whereas 11 of 17 infants older than 2 days were bacteremic with Gram-negative, penicillin G-resistant anaerobes. Eleven of 17 infants with anaerobic bacteremia associated with necrotizing enterocolitis were bacteremic with Gram-negative anaerobes. Five of 6 infants with anaerobic bacteremia associated with chorioamnionitis were bacteremic with Gram-positive anaerobes. These observations should be considered in making decisions regarding empiric therapy for the newborn at highest risk for anaerobic bacteremia.     

Panniculitis in childhood and adolescence

Background: The purpose of the present paper was to describe the clinical manifestations and treatment of patients with panniculitis. Methods: From January 1983 to December 2002, 4294 patients were treated for pediatric rheumatological diseases at Pediatric Rheumatology Unit, University of São Paulo, Brazil. Of these, 35 children and adolescents (0.8%) presented with panniculitis: erythema nodosum (EN) or Weber–Christian disease (WCD). Clinical characteristics, laboratory exams, biopsy of the lesion, treatment and clinical course were studied. Results: Of the 35 patients, 29 presented with EN and six with WCD, one of these with cytophagic histiocytic panniculitis. Mean age at symptom onset was 85 months (6–204 months) and the mean duration of follow up was 55 months (1–144 months). All the patients presented with inflammatory subcutaneous nodules. The patients with WCD presented with systemic manifestations and cutaneous atrophy. The principal etiologies of EN were streptococcal infection (42%), undetermined (13.5%), pulmonary tuberculosis (10%), and acute rheumatic fever (10%). Biopsy of the nodules indicated septal panniculitis in 14 patients with EN and lobular panniculitis without vasculitis in the patients with WCD, one of which had cytophagic histiocytic panniculitis. There was recurrence in 11 patients (38%) with EN and in all those with WCD. Non‐steroidal anti‐inflammatory drugs were used in 15 patients with EN and corticosteroids and/or immunosuppressive drugs in the six patients with WCD. Three patients died. Conclusions: EN is the most frequent panniculitis, with a benign course and is mainly associated with infections. WCD is a severe disease, with systemic involvement, that proceeds with cutaneous atrophy and requires the use of corticosteroids and or immunosuppressive drugs.     

Successful allogeneic hemopoietic stem cell transplantation in a child who had anhidrotic ectodermal dysplasia with immunodeficiency

Anhidrotic ectodermal dysplasia with immunodeficiency is associated with multiple infections and a poor clinical outcome. Hypomorphic mutations in nuclear factor kappaB essential modulator (NEMO)/IkappaB kinase complex and a hypermorphic mutation in inhibitor alpha of nuclear factor kappaB (IkappaBalpha) both result in impaired nuclear factor kappaB activation and are associated with X-recessive and autosomal-dominant forms of anhidrotic ectodermal dysplasia with immunodeficiency, respectively. Autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency is also associated with a severe T-cell phenotype. It is not known whether hematopoietic stem cell transplantation can cure immune deficiency in children with anhidrotic ectodermal dysplasia with immunodeficiency. A boy with autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency and a severe T-cell immunodeficiency underwent transplantation at 1 year of age with haploidentical T-cell-depleted bone marrow after myeloablative conditioning. Engraftment occurred, with full hematopoietic chimerism. Seven years after transplantation, clinical outcome is favorable, with normal T-cell development. As expected, the developmental features of the anhidrotic ectodermal dysplasia syndrome have appeared and persisted. This is the first report of successful hematopoietic stem cell transplantation in a child with anhidrotic ectodermal dysplasia with immunodeficiency. Hematopoietic stem cell transplantation is well tolerated and efficiently cures the profound immunodeficiency associated with autosomal-dominant anhidrotic ectodermal dysplasia with immunodeficiency.     

Girls with attention deficit disorder: a silent minority? A report on behavioral and cognitive characteristics

A sample of girls and boys with attention deficit disorder (ADD) were examined for within-group sex differences and compared with control boys and girls on historical, behavioral, and cognitive characteristics. Profiles of essential and secondary features of ADD support the Diagnostic and Statistical Manual of Mental Disorders nosology for ADD with and without hyperactivity as clinically distinct subtypes of ADD, with the girls and boys with ADD with hyperactivity exhibiting attentional, behavioral, and cognitive impairments, and the children with ADD without hyperactivity exhibiting deficits along an attentional/cognitive axis. Management problems and antisocial behaviors were correlates of hyperactivity, and increased impulsivity was not associated with attentional deficits in the absence of hyperactivity. Within the ADD with hyperactivity group, the girls demonstrated more severe cognitive impairments, particularly in the area of language function, were younger at the time referral for medical attention, and tended to come from families of lower socioeconomic status. Disruptive, uncontrolled behaviors were more frequent among the boys with ADD with hyperactivity. The girls with ADD without hyperactivity demonstrated poorer self-esteem and were significantly older than the boys with ADD without hyperactivity at referral. Girls in both ADD with and without hyperactivity groups were more likely to suffer peer rejection than their male counterparts. The results of this study suggest that girls with ADD may be underidentified and that cognitive deficits have a more prominent role in the identification of girls, whereas behavioral disturbances increase the likelihood of identification for boys.     

Reduction of absolute lymphocyte count in children with recurrent medulloblastoma

The complete blood cell (CBC) count with differential cell counts of 36 children with medulloblastoma showed a substantial reduction of the absolute lymphocyte count at recurrence. The preoperative value of the CBC count with differential cell counts failed to predict the survival of patients with medulloblastoma. A prospective study with serial measurement of the CBC count with differential cell counts will be needed to determine sensitivity and specificity of the CBC count with differential cell counts to clinical application for early detection of recurrence of medulloblastoma.     

Bone disease in infants and children with hepatobiliary disease

Radiological studies of bone were performed in infants and children with hepatobiliary disease. Rickets was found in 23 out of 39 patients (59%) with surgically unrepaired biliary atresia, in 4 out of 15 (27%) with surgically repaired biliary atresia, in 11 out of 21 (52%) with neonatal hepatitis, and in 2 out of 4 (50%) with intrahepatic cholestasis. Osteoporosis was found in 23 out of 39 (59%) with unrepaired biliary atresia, in 3 out of 15 (20%) with repaired biliary atresia, in 5 out of 21 (24%) with neonatal hepatitis, and in 1 out of 4 (25%) with intrahepatic cholestasis. 2 girls with Byler disease and 1 infant with choledochal cyst showed no radiological evidence of bone disease.In unrepaired biliary atresia comparative studies of biochemical data in the groups with and without bone disease showed the following. Serum calcium levels were reduced in the patients with bone disease compared with those in the group without it. Serum magnesium levels were markedly reduced in the groups with and without bone disease. The product of serum calcium and phosphorus was reduced in the group with osteoporosis compared with that in the group without it. The raised levels of serum alkaline phosphatase were unrelated to the presence or absence of bone disease.     

Cerebrospinal fluid concentrations of interleukin-1 beta, tumour necrosis factor-alpha, and interferon gamma in bacterial meningitis

To investigate the role of the inflammatory cytokines, the cerebrospinal fluid concentrations of interleukin (IL)-1 beta, tumour necrosis factor-alpha (TNF-alpha), and interferon gamma (IFN-gamma) were measured in 11 children with bacterial meningitis and two with mycoplasmic meningoencephalitis and compared with those in 50 children with aseptic meningitis and 15 with non-pleocytotic cerebrospinal fluid. Concentrations of IL-1 beta and TNF-alpha were each significantly higher in the cerebrospinal fluid of patients with bacterial meningitis than in those with aseptic meningitis or those with non-pleocytotic cerebrospinal fluid. IFN-gamma was detected at low concentrations in the cerebrospinal fluid of only 2/11 of those with bacterial meningitis. On the other hand, the IFN-gamma concentration was the highest in the cerebrospinal fluid of patients with aseptic meningitis. These results suggest that the inflammatory cytokines are differently released in the intrathecal space infected with viruses or bacteria.     

Cerebrospinal fluid concentrations of interleukin-1 beta, tumour necrosis factor-alpha, and interferon gamma in bacterial meningitis.

To investigate the role of the inflammatory cytokines, the cerebrospinal fluid concentrations of interleukin (IL)-1 beta, tumour necrosis factor-alpha (TNF-alpha), and interferon gamma (IFN-gamma) were measured in 11 children with bacterial meningitis and two with mycoplasmic meningoencephalitis and compared with those in 50 children with aseptic meningitis and 15 with non-pleocytotic cerebrospinal fluid. Concentrations of IL-1 beta and TNF-alpha were each significantly higher in the cerebrospinal fluid of patients with bacterial meningitis than in those with aseptic meningitis or those with non-pleocytotic cerebrospinal fluid. IFN-gamma was detected at low concentrations in the cerebrospinal fluid of only 2/11 of those with bacterial meningitis. On the other hand, the IFN-gamma concentration was the highest in the cerebrospinal fluid of patients with aseptic meningitis. These results suggest that the inflammatory cytokines are differently released in the intrathecal space infected with viruses or bacteria.