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.     

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.     

Fungal lung disease

Fungal lung disease in the paediatric population occurs with distinct features in the immunocompetent, in immunocompromised patients and in people with cystic fibrosis. Pulmonary mycoses are the least prevalent in immunocompetent children, with the most common diseases being the endemic mycoses and Aspergillomas. Filamentous fungi such as Aspergillus and Scedosporium have been isolated with increased frequency in recent years from the respiratory secretions of individuals with cystic fibrosis. Undoubtedly, fungal respiratory infections are encountered with increased frequency and severity in patients with impaired immune systems, such as patients with malignancies, solid organ or bone marrow transplants and immunodeficiencies [1].     

IL-31 significantly correlates with disease activity and Th2 cytokine levels in children with atopic dermatitis

Recently, we could show that IL-31 serum levels are significantly increased in adult patients with atopic dermatitis compared with skin healthy controls. However, the regulation of IL-31 in children with atopic dermatitis so far is not clear. Thus, we analyzed IL-31 serum levels together with IL-4, IL-13, ECP, and total IgE levels in 60 children with extrinsic, in five children with intrinsic atopic dermatitis, and 20 non-atopic healthy children. Further, we determined the SCORAD score, sleeplessness, and pruritus severity in all children with atopic dermatitis. IL-31 was significantly increased in children with the intrinsic and extrinsic type of atopic dermatitis compared with non-atopic healthy children (p?相似文献   

VISUAL PATHWAY TUMORS AND HYDROCEPHALUS

The authors evaluated the impact of hydrocephalus on the clinical picture of children with visua pathway tumor (VPT) with or without neurofibromatosis (NF).Charts of children with VPT treated in the authors' center since 1985 were retrospectively reviewed, and those with hydrocephalus were selected and summarized. Thirty-five children with VPT were found, of whom 20 had NF.Hydrocephalus was found in 4 children with NF (20% ) and in 5 without NF (33.3% ). In 6 ofthechildren, ventricular dilatation with signs of acute increased intracranial pressure already existed at the time of diagnosis and the hydrocephalus was shunted at this time. In the other 3 children, all with NF,the hydrocephalus resulted from slowly developing aqueductal stenosis, leading in 2 to severe visual acuity deterioration. The results suggest that in children with VPT and NF, hydrocephalus, and especially hydrocephalus resulting from aqueductal stenosis, is more frequent than in the general population of NF patients, and less frequent than in VPT patients without NF. The possibility of the indolent development of hydrocephalus should be borne in mind while following children with NF. The optic nerve, when already involved with a glioma, is more vulnerable to increased pressure. Thus, in children with VPT and NF, any ventricular dilatation should lead to a consideration of early shunting.     

Is adrenomedullin involved in the pathophysiology of persistent pulmonary hypertension of the newborn?

Although adrenomedullin (ADM) is a potent vasodilating peptide reported to play a possible role in the mechanisms of fetal lung differentiation and maturation, the ADM blood level in fetuses and in neonates with persistent pulmonary hypertension (PPHN) and pulmonary hypoplasia is not known. Therefore, we examined 15 patients with PPHN: 10 with congenital diaphragmatic hernia, four with congenital cystic adenomatoid malformation of the lung, and one with misalignment of pulmonary vessels with alveolar capillary dysplasia. Eight surgical patients with neonatal conditions such as intestinal atresia served as controls. Blood samples were drawn from the umbilical artery and vein at birth, and arterial blood was drawn from patients with PPHN on the 3rd and 6th days after birth. Plasma levels of ADM were measured by radiometric assay. Plasma levels of ADM in the umbilical artery and vein were elevated in patients with PPHN compared with controls, and in all groups the levels in the umbilical vein were higher than those in the umbilical artery. The arterial levels in patients with poor prognoses were elevated on the 3rd and 6th days after birth compared with those in survivors. These results indicate that ADM may be involved in the pathophysiology of PPHN and in the mechanisms of lung differentiation and/or maturation.     

不同基因型地中海贫血患儿铁代谢和红细胞系造血状况研究

目的：探讨不同基因型地中海贫血（地贫）患儿体内铁代谢和红细胞系造血状况。方法：对158例确诊地贫患儿进行血清铁蛋白（SF）、血清转铁蛋白受体（sTfR）、促红细胞生成素（EPO）检测,比较不同基因型地贫患儿之间的差异,并分析其与血红蛋白水平的相关性。结果：158例地贫患儿中,轻型α地贫52例（32.9％）,血红蛋白H病（HbH病）27例（17.1％）,轻型β地贫59例（37.4％）,重型β地贫13例（8.2％）,α复合β地贫7例（4.4％）。HbH病及重型β地贫患儿的SF水平较其他各组明显升高,差异有统计学意义（P＜0.01）;重型β地贫患儿sTfR水平较其他各组升高,差异有统计学意义（P<0.05）。重型β地贫患儿EPO水平较其他各组明显升高,差异有统计学意义（P＜0.01）;HbH病和重型β地贫患儿Hb水平和EPO水平呈负相关,分别为γ=-0.656（P＜0.01）和γ=-0.641（P＜0.05）。结论：不同基因型地贫患儿体内铁代谢和红细胞系造血状况不同,联合SF、sTfR和EPO检测可反映造血状况,指导临床治疗。［中国当代儿科杂志,2010,12（8）：602-604］     

Mast cells and eosinophils in the jejunal mucosa of patients with intestinal cow's milk allergy and celiac disease of childhood

We counted the number of granulated mast cells with high iron diamine staining, and the number of eosinophils with hematoxyline-eosin staining, in the lamina propria of the jejunum in 12 untreated patients with intestinal cow's milk allergy (CMA), 47 with celiac disease (CD), and 14 controls. A decreased number of mast cells and an increased number of eosinophils were found in 58% of patients with CMA, and in 60% of those with CD. The number of mast cells showed a significant positive correlation with the villous height, and the number of eosinophils a negative correlation with both the villous height, and the number of mast cells. Appropriate dietary treatment resulted in a rise in the number of granulated mast cells and a decrease in the number of eosinophils in both patient groups.     

Yeast opsonisation in children with chronic diarrhoeal states.

Four patients with defective yeast opsonisation and protracted diarrhoea are reported. Plasma infusions improved the opsonising function in all 4 and the diarrhoea in 3. This immunological abnormality was assessed in 100 sequential patients with chronic diarrhoea associated with various gastrointestinal disorders; 52 with protracted diarrhoea and failure to thrive of undetermined cause, 26 with ''toddler diarrhoea'', 8 with coeliac disease, 5 with chronic inflammatory bowel disease, and 9 with miscellaneous disorders. 23% of the patients with protracted diarrhoea of undetermined cause had defective opsonisation, a greater proportion (P less than 0.05) than that in ''toddler diarrhoea'' or the remaining patients, in whom the frequency (4%) was similar to that (5%) in healthy populations. We suggest that yeast opsonisation be tested in children with protracted diarrhoea, as plasma infusions can be an effective form of treatment.     

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.     

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.     

The prognostic value of serum lysozyme activity in acute myelogenous leukemia

Serum lysozyme activity was measured in samples from adult patients with acute leukemia, malignant tumors, and in normal adults. Twenty-eight adult patients with acute myelogenous leukemia (AML) had significantly elevated levels of lysozyme at diagnosis, and none of the adults fell within the normal range. Thirty-two patients with AML in complete remission had lysozyme levels comparable to normal adults, whereas patients with AML in relapse (eight cases) also had abnormally high levels of lysozyme activity. Ten patients with AML in remission and off therapy also had normal lysozyme levels. Three patients with acute lymphatic leukemia had normal lysozyme levels, while one child with monomyelocytic leukemia had substantially elevated lysozyme levels before treatment. It seems that in patients in remission and with normal blood values, the serum lysozyme activity is valuable for monitoring the remission.     

Correlation of the C677T MTHFR genotype with homocysteine levels in children with sickle cell disease.

Recently, a mild to moderate elevation in the plasma homocysteine (Hcy) level has been found to be an important risk factor for stroke. Homozygosity for a common mutation (C677T) in the gene encoding for the enzyme methylenetetrahydrofolate reductase (MTHFR) involved in Hcy metabolism has been associated with increased levels of Hcy. To determine the role of hyperhomocysteinemia in the pathogenesis of stroke in children with sickle cell disease (SCD), Hcy levels and C677T MTHFR genotype were determined in 40 patients homozygous for hemoglobin SS and compared with 197 healthy children. Eleven of 40 patients with SCD had a history of stroke. The prevalence of homozygosity for the C677T MTHFR variant was 5% in the patients with SCD. The median Hcy level was 5.8 micromol/L in the patients versus 5.4 micromol/L in the controls (Fisher's, P > 0.05). There was no correlation of Hcy levels with the MTHFR genotype in patients with SCD. In patients with SCD and stroke, the median Hcy level was 4.8 micromol/L versus 6.0 micromol/L in those without stroke (P = 0.44, Mann-Whitney rank sum test). There was no difference in the proportion of patients with SCD with or without stroke who were homozygous for the C677T MTHFR mutation (0/11 versus 2/29; Fisher's, P = 1.000). In conclusion, this study failed to demonstrate an elevation in plasma Hcy levels in children with SCD compared with normal controls. Furthermore, hyperhomocysteinemia did not seem to be a significant factor in the pathogenesis of stroke in children with SCD.     

儿童先天性肝纤维化临床分型及特征

目的 比较不同临床分型先天性肝纤维化(CHF)患儿的临床特征,总结儿童CHF 的特点.方法 2002 年1 月至2015 年6 月期间确诊为CHF 的60 例患儿中,门脉高压型26 例,胆管炎型3 例,混合型30 例,隐匿型1 例.采集26 例门脉高压型和30 例混合型患儿的性别、年龄、临床表现、体征、实验室检查、影像特点等临床资料进行回顾性研究.结果 发热、黄疸和肝肿大在混合型患儿中的发生率高于门脉高压型患儿(PP>0.05);混合型患儿凝血酶原活动度、白细胞计数、血小板计数、血小板平均体积、丙氨酸转氨酶、天冬氨酸转氨酶、碱性磷酸酶、ν谷氨酰转肽酶、亮氨酸氨基肽酶和总胆汁酸水平高于门脉高压型患儿,国际标准化比值和白蛋白水平低于门脉高压型患儿(P结论 儿童CHF 中以门脉高压型和混合型常见,门脉高压型和混合型均以肝硬化、肝脾肿大等门脉高压表现明显,但混合型常伴肝损伤.     

不同血糖水平对缺氧缺血新生大鼠脑病理改变的影响

目的探讨不同葡萄糖水平对缺氧缺血(HI)新生大鼠脑病理改变的影响。方法对7日龄SD新生大鼠通过调节葡萄糖注射液的注射次数及时间或禁食12 h,分别建立单纯低血糖组、HI组、HI前低血糖组、HI后低血糖组、HI前轻度高血糖组、HI后轻度高血糖组、HI前重度高血糖组及HI后重度高血糖组;另设正常组和假手术组。各组新生大鼠分别均于HI后2、24、487、2 h及7 d断头处死,光镜下行脑病理检查并做病理评分。结果HI后2、244、87、2 h和7 d各时段中,以HI前低血糖组的改变最为明显,HI后7 d的病理评分高达280分。HI后低血糖组的病变程度仅次于HI前低血糖组(180分)。各组中以HI前重度高血糖组的病变程度最轻(9.2分)。余各组病变程度与HI组相似(100分左右)。脑病理改变以坏死为主,在坏死灶边缘可见散在变性神经元。除HI前高血糖组的病变主要累及皮质外,各组在皮质及海马两个部位的受累几率基本均等。结论HI前低血糖可进一步加剧新生大鼠脑缺氧缺血性损害,HI前高血糖则可明显改善新生大鼠脑缺氧缺血性损伤。     

Recurrence of brain tumours in patients treated with growth hormone: analysis of KIGS (Pfizer International Growth Database)

Background: Growth hormone (GH) has been used successfully in the treatment of short stature secondary to GH deficiency in survivors of childhood brain tumours. There has been concern that GH might increase the risk of recurrence. Aim: To analyse KIGS (Pfizer International Growth Database) with respect to tumour recurrence in patients with brain tumours. Methods: Data for tumour recurrence were analysed retrospectively in 1038 patients with craniopharyngiomas, 655 with medulloblastomas, 113 with ependymomas, 297 with germinomas, and 400 with astrocytomas or gliomas. All patients had received recombinant human GH (Genotropin®, Pfizer Inc.). Results: Recurrence-free survival rates were 63% at a follow-up of 10.3 y in craniopharyngioma, 69% in 9.1 y in the glial tumours, 71% in 7.4 y in germinomas, 92% in 4.6 y in medulloblastomas and 89% in 2.5 y in ependymomas. Dose of GH and treatment modalities did not differ significantly between patients with and without recurrence.
Conclusion: Tumour recurrence rates in surviving patients with brain tumours receiving GH treatment do not appear to be increased compared with published reports. However, longer follow-up regarding recurrences and secondary neoplasms remains essential.     

婴儿先天性心脏病1387例外科治疗结果分析

目的 回顾性分析我院近11年来外科治疗的≤6个月小婴儿先天性心脏病(先心病)1387例,探讨其病种、手术时机和治疗观念的变迁,以期进一步提高小婴儿先心病的就诊和救治率.方法 1997年1月至2007年12月,在我院行手术治疗的≤6个月先心病患儿1387例,主要病种包括:室间隔缺损合并肺动脉高压(VSD/PH)、法洛四联症(TOF)、完全性大动脉转位(TGA)、完全性肺静脉异位引流(TAPVC)、主动脉缩窄或主动脉弓中断合并室间隔缺损[CoA(IAA)/VSD]、右心室双出口(DORV)、合并室间隔缺损的肺动脉闭锁(PA/VSD)、室间隔完整的肺动脉闭锁(PA/IVS)等,根据病情采取相应的手术方法矫治,部分复杂型先心病进行了随访.结果 手术死亡110例,总手术死亡率7.9%.从历年手术治疗分析,手术死亡率1997至2003年为11.5%～14.4%,2004至2005年降至8.6%～&9%,2006至2007年降至3.3%～3.8%.对TGA、TAPVC、TOF、PA/VSD、PA/IVS患儿进行了随访,随访率分别为83.8%(98/117)、87.8%(79/90)、48.2%(68/141)、65%(13/20)和95%(19/20),随访期限为3～86个月.晚期死亡16例.随访中绝大多数患儿无症状,心功能和生长发育正常.结论 绝大部分早期出现症状的危重先心病可以在小婴儿期进行矫治,手术效果接近国际水平.不能进行一期矫治的可以先做姑息手术,改善缺氧、促进肺动脉发育,为以后的根治手术创造条件.     

儿童髓母细胞瘤两年内复发危险因素分析

目的 探讨儿童髓母细胞瘤（MB）两年内复发的危险因素及对无进展生存（PFS）率的影响。方法 对2017年1~12月收治的123例MB患儿进行回顾性研究,根据复发与否分为复发组（n=30）和非复发组（n=93）,分析影响儿童MB两年内复发的危险因素,比较不同因素间的PFS率。结果 大细胞/间变型、M分期,均为儿童MB两年内复发的危险因素。分期为M+者,其复发风险为M0的3.525倍;大细胞/间变型复发风险为经典型的3.358倍,差异有统计学意义（P < 0.05）。生存分析示分期为M+者,中位PFS期为20个月,M+、M0 20个月PFS率分别为50%±11%、81%±5%（P < 0.05）。经典型、促纤维增生/结节型、广泛结节型、大细胞/间变型20个月PFS率分别为80%±5%、65%±10%、86%±13%、36%±20%,不同病理分型之间的PFS率差异有统计学意义（P < 0.05）。结论 复发是影响儿童MB预后的重要因素,分期为M+及大细胞/间变型MB均为复发的危险因素,且PFS率更低。     

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.