淤胆型肝病致VitE缺乏与周围神经损害的动物实验研究

目的：探讨淤胆型肝病时血清VitE浓度与周围神经损伤的关系。方法：2周龄Wistar幼鼠随机分为对照组、肝病组和VitE治疗组(n=10),建立改良α萘异硫氰酸酯(ANIT)淤胆型肝病模型。观察6周(至8周龄),检测血清VitE浓度、血清总胆红素(TB)、结合胆红素(DB)和丙氨酸氨基转移酶(ALT)含量;观察肝脏、坐骨神经组织病理学改变;采用生物医学图像分析系统分析肝组织病变面积。结果：肝病组和治疗组血清DB、TB值较对照组增高倍数分别为62.4、30.0和21.1、10.1;肝病组肝组织病变面积明显大于治疗组(P<0.001);血清中VitE含量明显低于对照组(P=0.004)和治疗组(P=0.016);坐骨神经有明显原纤维轴索变性和髓鞘脱失等病理改变,异常半薄切片数明显高于治疗组(P=0.02)。结论：体内VitE不足是导致淤胆型肝病周围神经受损的重要原因;补充VitE能有效减轻周围神经损害,同时可明显降低血清TB、DB浓度,有效抑制ANIT所致肝损伤和肝纤维化。     

Language skills at age 6 years in Swedish children screened for language delay at 2(1/2) years of age

This study concerns language outcome in 6-y-old children who participated in a longitudinal community-based study of 105 children screened for language delay (LD) at 2(1/2) y of age. The purpose was to investigate (1) whether results from the 2(1/2)-y screening were persistent at 6 y of age, and (2) what language domains at age 6 were difficult for (a) children with LD at age 2(1/2) y and (b) children with normal language (LN) at the same age. Significant differences between LD and LN at age 2(1/2) y were persistent at age 6. The vulnerability that was identified at 2(1/2) y of age, such as problems with going from single-word utterances to multi-word utterances, seems to persist as delayed development at different language levels and across language domains. CONCLUSION: This study has shown that children who failed the 2(1/2)-y screening are at high risk of having persistent language problems at age 6 y.     

A prospective study of acute diarrhoea in Finnish children from birth to 2 1/2 years of age

To determine the incidence, clinical significance and etiology of acute diarrhoea in early childhood, a cohort of 336 children were followed from birth to the age of 24-32 (mean 26) months. More than half (55%) of the children had no diarrhoea, 26% had one episode and 19% had two or more episodes of diarrhoea during follow-up; altogether 248 episodes of diarrhoea were detected. Rotavirus was by far the most common (26%) identified pathogen; adenoviruses were detected in 4% and bacterial pathogens (EPEC, Salmonellae, Yersiniae) in 4% of the cases. Two thirds of the episodes remained etiologically unresolved. Rotavirus diarrhoea was significantly more severe than diarrhoea due to other causes; 75% of severe episodes of diarrhoea were associated with rotavirus. About two thirds of the infants were breast-fed over 6 months; breast-feeding for less than 6 months was associated with a higher incidence of rotavirus diarrhoea between 7-12 months of age but not thereafter. About three quarters of the children were cared for at home beyond 12 months of age; those at home had a lower rate of rotavirus diarrhoea than those at day-care centers.     

Acoustic quality of cry in very-low-birth-weight infants at the age of 1 1/2 years

BACKGROUND: Infant cry characteristics reflect the integrity of the central nervous system. Previous studies have shown that preterm infants and infants with neurological conditions have different cry characteristics such as fundamental frequency compared to healthy full-term infants. Cry characteristics of preterm infants after the first year of life have not been studied. AIMS: The aim of this study was to assess the quality of cry in 1 1/2-year-old very-low-birth-weight infants (VLBWI, < or =1500 g at birth). STUDY SUBJECTS AND DESIGN: Study groups included 21 VLBWI and 25 healthy full-term controls. Thirty seconds of pain cry after vaccination was recorded at well-baby clinics. The first cry utterance was acoustically analyzed using Praat software. The quality of cry was compared between the groups. In addition, the association of cry quality to patient characteristics, to developmental outcome, and to findings in brain imaging studies of the VLBWI was studied. RESULTS: The cry response was elicited in 20 of the 21 VLBWI and in 20 out of 25 full-term infants. VLBWI had higher minimum fundamental frequency and fourth formant values. Patient characteristics that were associated with cry quality were 5-min Apgar scores, the occurrence of bronchopulmonary dysplasia, Bayley Psychomotor Index scores at 12 months, and current weight and head circumference. CONCLUSIONS: Differences found between the study groups were not explained primarily by brain pathology or by patient characteristics, so it seems that prematurity has an impact on cry quality still at the age of 1 1/2 years.     

Typical absence epilepsy presenting prior to age of 3 years: An uncommon form of idiopathic generalized epilepsy

PURPOSE: An attempt to allocate patients with the clinical features and electroencephalography (EEG) abnormalities of typical absence epilepsy presenting before the age of 3 years, similar to childhood and juvenile absence epilepsy (JAE) and delineate the clinical manifestations, EEG abnormalities, therapy and outcome of such an epileptic disorder by conducting a nationwide survey. RESULTS: Overall, eight infants, six males and two females, abided by the inclusion criteria of typical absence epilepsy: They were born after an unremarkable pregnancy and labor presenting at the age of 12-34 months (mean: 19.6 months) with frequent absences time-linked with an EEG demonstrating generalized occasionally irregular epileptiform discharges of 3-4 Hz spike/wave and normal background activity along with an electrographic photosensitive response in one patient. Neurological examination was intact in all infants. All eight infants were initially treated with valproic acid, of whom seven immediately responded and one had increase in frequency and duration of absences completely aborted with treatment of lamotrigine. Three relapsed after termination of therapy of whom two again presented with recurrent absences and another one with generalized tonic-clonic seizures and as such these children had virtually transformed into a later form of idiopathic generalized epilepsy (IGE) during childhood. All eight patients are seizure-free, seven still on therapy; seven children within a follow-up period of 2-7 years and the most recently diagnosed infant for 6 months. Cognitive skills were found normal in all children within the low normal range in three children with short attention and concentration spans. CONCLUSION: The data presented here delineate a very rare form of idiopathic benign generalized epilepsy presenting with typical absences before age of 3 years and a favorable outcome, similar to childhood and JAE, recognized as distinct IGE syndromes by the International League Against Epilepsy (ILAE) classification.     

Visual-motor function of very low birth weight and full-term children at 3 1/2 to 4 years of age

Improvements in perinatal and neonatal management have not only led to a higher survival rate of very low birth weight infants (VLBW; < or = 1,500 g or < 32 weeks gestational age), but also to a better outcome of these children. However the percentage of VLBW children who need special education because of later school problems remains high even in children considered neurologically normal during infancy. We assessed 40 VLBW children and 83 healthy full-term children at age 3 to 4 years by means of a simple and short test for visual-motor deficits. The test included the copying and cutting-out of geometric shapes, the building of models, the recognition of colours and the observation of the concentration and cooperation during the test. All VLBW children had had a good perinatal outcome and had been considered neurologically normal at one year of age. Most VLBW children scored within 1 standard deviation (S.D.) of the test mean, but on average the VLBW children scored significantly lower than the full-term infants in the copying of figures, the cutting-out of geometric forms, the building of models and in the overall concentration and cooperation during the test. Children who attended a nursery school achieved significantly better test results. Girls tended to have better results, but this was not statistically significant. Social factors and age had a significantly greater impact on results than perinatal factors. In summary, VLBW children scored significantly less in almost every test item compared to their term peers. Our test battery could serve as a short introductory test to screen for deficits in visual-motor skills, especially in VLBW children.     

Externalizing behavior in early childhood and body mass index from age 2 to 12 years: longitudinal analyses of a prospective cohort study

Background  

Some evidence suggests that obesity and behavior problems are related in children, but studies have been conflicting and have rarely included children under age 4. An association between behavior problems in early childhood and risk for obesity could suggest that a common set of factors contribute to both. Our research objectives were to determine the extent to which externalizing behavior in early childhood is related to body mass index (BMI) in early childhood and through age 12, and to evaluate whether these associations differ by sex and race.     

Evaluation of the WHO/UNICEF algorithm for integrated management of childhood illness between the age of two months to five years

OBJECTIVE: To evaluate the utility of the "WHO/UNICEF algorithm for integrated management of childhood illness (IMCI) between the age of 2 months to 5 years. DESIGN: Prospective observational. SETTING: The Outpatient Department and Emergency Room of a medical college hospital. METHODS: 203 children presenting to Outpatient Department (n=101) or Emergency Room (n=102) were assessed and classified as per 'IMCI' algorithm and treatment required was identified. A detailed evaluation with all relevant investigations was also done for these subjects. The final diagnoses made and therapies instituted on this basis served as 'gold standard'. The diagnostic and therapeutic agreements between the 'gold standard' and the IMCI and vertical (on the basis of primary presenting complaint) algorithms were computed. RESULTS: More than one illness was present in 135 (66.5%) of subjects as per 'gold standard'. The mean (SD) numbers of morbidities as per the gold standard and IMCI- low and high malaria risks were 2.1 (1.1), 1.8 (1.0) and 2.2 (1.1), respectively. Subjects having any referral criteria as per IMCI module had a greater co-existence of illnesses (mean 2.6 vs. 1.6 illnesses per child, respectively). The referral criteria proved useful in predicting hospitalization and a combination of hospitalization and observation; their sensitivity and specificity were 81% and 69% and 74% and 85%, respectively. IMCI algorithms covered majority (92%) of the recorded illnesses. A total agreement with IMCI (malaria low risk) was found in 129 (64%) cases while in 43 (22%) cases, there was partial agreement. Corresponding figures for vertical (split IMCI) program were 93 (46%; p<0.001) and 41 (25%). The difference was primarily due to underdiagnoses (30%). Diagnostic discordance of IMCI algorithm and gold standard was evident for the cough category due to underdiagnosis of bronchial asthma and bronchiolitis and an overdiagnosis of pneumonia whereas the discordance for fever was due to an overdiagnosis of malaria. Identical results were found for broad treatment categories. The IMCI algorithm had a provision for preventive services of immunization (16.3% possibility of availing missed opportunities) and feeding advice. CONCLUSIONS: There is a sound scientific basis for adopting the IMCI approach since: (i) co-existence of morbidities is frequent; (ii) severe illness is assessed with good sensitivity and specificity; and (iii) the IMCI algorithm is diagnostically and therapeutically superior to the vertical disease specific algorithms. The generic IMCI algorithm needs adaptation to reflect the regional morbidity profile.     

Vitamin K deficiency in newborns: a case report in alpha-1-antitrypsin deficiency and a review of factors predisposing to hemorrhage

A 4-week-old, breast-fed female infant appeared healthy until signs and symptoms of CNS deterioration suddenly occurred. At presentation the infant was found to have a left-sided parietal intracerebral hematoma, markedly prolonged prothrombin time, and partial thromboplastin time, normal platelet count, and jaundice with a total and direct serum bilirubin level of 5.4 mg/dL and 2.6 mg/dL, respectively. Vitamin K1 and fresh frozen plasma returned the prothrombin time and partial thromboplastin time to normal values within 18 hours, suggesting that the infant had severe vitamin K deficiency complicated by intracerebral hemorrhage. Evaluation of the infant's direct hyperbilirubinemia led to the diagnosis of homozygous (pi-type ZZ [PiZZ] ) alpha-1-antitrypsin deficiency. The clinical circumstances predisposing to vitamin K deficiency in newborns and infants are discussed. Based on our observations in this case, we suggest that cholestatic liver disease should be suspected when unexplained vitamin K deficiency occurs in early infancy. The role of vitamin K in hemostasis and the laboratory diagnosis of vitamin K deficiency are discussed as they apply to the evaluation of hemorrhage in newborns and infants.     

Growth charts for children with Down syndrome: 1 month to 18 years of age

Centile charts for assessment of stature and weight reflecting expected deficient size and growth rate of home-reared children with Down syndrome are presented for two age intervals, 1 to 36 months and 2 to 18 years, based on 4650 observations on 730 children. Data were pooled and used to estimate five centiles which were smoothed using a flexible mathematical function. These data corroborate other studies of growth in children with Down syndrome demonstrating deficient growth rate throughout the growing period, but most marked in infancy and again at adolescence. Children with Down syndrome in the present sample were taller than those from institutionalized samples at all ages throughout the growing period. Children with moderate or severe congenital heart disease on average were 1.5 to 2.0 cm shorter and about 1 kg lighter than those without or with only mild disease. Mean weight and weight divided by stature squared show that children with Down syndrome have a tendency to be overweight beginning in late infancy and throughout the remainder of the growing years.     

儿童急性淋巴细胞性白血病E2A/PBX1融合基因的检测及临床意义

白血病是儿童期最常见的恶性肿瘤,其中急性淋巴细胞性白血病(ALL)约占70%.t(1;19)(q23;p13)是小儿ALL较常见的染色体易位之一,大约可在3%～5%的B细胞ALL患儿中发生,此易位涉及1号染色体上的前B白血病1(pre-B leukemia 1,PBX1)基因和第19号染色体上的免疫球蛋白增强子结合因子(immunoglobulin enhancer binding factor,E2A)基因,在衍生19号染色体上形成E2A/PBX1融合基因.我们利用巢式逆转录-聚合酶链反应(RT-PCR)技术研究我院ALL患儿中此易位的发生率,并初步探讨患儿 的临床特征.     

The prevalence of mental health problems in children 1(1/2) years of age - the Copenhagen Child Cohort 2000

BACKGROUND: The Copenhagen Child Cohort, CCC 2000, was established to investigate developmental psychopathology prospectively from birth in a general population. METHODS: A random sample of 211 children from the CCC 2000 was investigated when the children were 1(1/2) years of age. The prevalence and associates of mental health problems and psychopathology were studied by clinical and standardised strategies, including videotape recordings, parent interviews and the following instruments: The Child Behavior Check List 1(1/2)-5 (CBCL 1(1/2)-5), The Infant Toddler Symptom Check List (ITSCL), Checklist for Autism in Toddlers (CHAT), Bayley Scales of Infant Development II (BSID II), The Parent Child Early Relationship Assessment (PC ERA) and Parent Infant Relationship Global Assessment Scale (PIR-GAS). RESULTS: Mental health problems according to International Classification of Diseases (ICD-10) and Diagnostic Classification Zero to Three (DC 0-3) diagnoses were found in 16-18% of 1(1/2)-year-old children. Most common were disturbances of emotion, behaviour and eating and the DC 0-3 diagnosis of regulatory disorder. Parent-child relationship disturbances were found in 8%. High psychosocial risk was significantly associated with emotional and behavioural disorders (OR 3.1 95% (1.2-8.1)) and disturbed parent-child relationship (OR 5.0 95% (1.6-16.0)). The strongest association of risk was found between relationship disorders and emotional and behavioural disorders (OR 11.6 95% (3.8-37.5)). CONCLUSIONS: The prevalence and distribution of psychopathology in 1(1/2)-year-old children seem to correspond to the distributions among older children. Disturbances in parent-child relationship have a key position in the risk mechanisms in early child psychopathology.     

Breastfeeding, very long polyunsaturated fatty acids (PUFA) and IQ at 6 1/2 years of age

Aim: Breastfeeding seems to be favorable for cognitive development. Could levels of polyunsaturated fatty acids (PUFA) explain this?Methods: Pregnant mothers were recruited consecutively at maternity care centres. PUFA were analysed in colostrum and breast milk at 1 and 3 mo. The product‐precursor ratios of n‐6+n‐3 PUFA were examined as measures of activity in respective steps in the fatty acid metabolic chain. Also, the quotient between DHA and AA was analysed. The children were tested with the full WISC‐III at 6.5 y. Results: First, the influence of length of breastfeeding was analysed by multiple regression together with relevant cofactors (except for PUFA). In the best models, 46% of the variation in total IQ was explained. Length of breastfeeding contributed significantly to total IQ (beta = 0.228, p= 0.021), verbal IQ (beta = 0.204, p= 0.040) and performance IQ (beta = 0.210, p= 0.056). There were no significant single correlations between PUFA and measures of cognitive development. However, in multiple regression analysis of colostrum, significant beta‐coefficients were found for steps 4+5 in the fatty acid metabolic chain (beta = 0.559, p= 0.002). If length of breastfeeding and gestation week were added to steps 4+5, this three‐factor model could explain 67% of the variation of total IQ. Introducing length of breastfeeding and gestation week together with the quotient DHA/AA (beta = 0.510, p > 0.001) yielded a three‐factor model, which explained 76% of the variation in total IQ. Conclusion: Our findings could be interpreted as supporting the importance of high levels of PUFA for cognitive development. However, the sample is small and the results must be interpreted with caution.