Neurovesical dysfunction in children with anorectal malformations

Chronic renal failure remain the most significant cause of morbidity and mortality in patients with anorectal malformation. The urological anomalies associated with anorectal malformations are not only anatomical, but also functional, the latter being related to congenital neurovesical dysfunction. The neurovesical dysfunction found in children with anorectal malformations results from a possible association with spinal dysraphism.The authors carried out urodynamic evaluation on 6 patients operated on for anorectal malformation by posterior sagittal anorectoplasty. 3 children had normal bladder function, but 3 had neurovesical dysfunction (1 unstable bladder, 2 neuropathic bladder). It is concluded that in patients with anorectal malformations urodynamic investigations should be performed as a routine investigation of the urinary tract. Consequently, patients with lower urinary tract dysfunction should receive prompt treatment, including clean intermittent catheterization, to prevent or reduce secondary urologic morbidity, especially loss of renal function.     

Antegrade continence enemas in the management of intractable faecal incontinence.

The introduction of the Malone procedure has improved the outlook for children with severe faecal incontinence. Phosphate and saline enemas are administered through the exteriorized appendix in antegrade fashion to achieve evacuation and ensure cleanliness. The appendix functions as a non-refluxing catheterizable channel: If it is not available for use, a tubularized caecal flap is a safe alternative. We have constructed Malone stomas using the appendix in 20 patients and another seven patients have undergone the caecal flap modification. The mean age was 8.6 years. Eleven of the patients were boys and 16 (59%) were girls. Six children required dilatation or revision of their stomas for stenosis. One developed small bowel obstruction and another has stopped using the stoma. The results of the continence enemas were considered to be very good by the vast majority of patients and their carers. Our recent experience suggests that bisacodyl may be a valuable adjunct to the antegrade enemas of phosphate and saline. We believe that this procedure may be extended with benefit to adults with serious faecal incontinence in whom standard measures have failed.     

Outcome of infants born with nuchal cords.

BACKGROUND. The effect of a nuchal cord on the outcome of delivery is controversial. The purposes of this study were to investigate the frequency of nuchal cords and determine the effect that nuchal cords have on the neonate. METHODS. In a retrospective, case-control study, 706 consecutive infant deliveries in a community hospital were evaluated. Sixteen deliveries that were complicated by the umbilical cord entangled around an extremity or by a prolapsed cord were excluded from further analysis. The study group consisted of the 167 deliveries (23.7%) complicated by a nuchal cord. The remaining 523 deliveries were used as the control group. RESULTS. There were no significant differences found in maternal age, race, parity, prepregnancy weight, or amount of weight gain between the mothers of the infants in the two groups. Fetal bradycardia and variable decelerations in fetal heart rate occurred almost twice as often in the nuchal cord group (18.6% as compared with 9.6%, P less than .01). Despite this finding, there was no significant difference in the number of operative deliveries or in Apgar scores at 1 and 5 minutes between the two groups. There were no perinatal deaths associated with nuchal cords. Infants born with nuchal cords weighed less than those in the control group (3345 g compared with 3468 g, P less than .01). There were also significantly fewer large-for-gestational-age and macrosomic infants born in the nuchal cord group. Complications such as jaundice, hypoglycemia, sepsis, and respiratory problems were not increased in the postnatal period because of a nuchal cord. CONCLUSIONS. This study suggests that nuchal cords are common and are rarely associated with significant morbidity or mortality in neonates.     

先天性肛门直肠畸形儿童生存质量的研究

目的了解先天性肛门直肠畸形(anorectal malformations,ARM)儿童术后生存质量的状况,为临床制定有效的干预措施提供依据,以提高患儿的生存质量。方法采用PedsQLTM4.0生存质量量表中文版对100例ARM术后2年和术后5年的儿童进行随访调查,比较ARM患儿与健康儿童生存质量的差异。结果 1)2岁~ARM患儿情感功能生存质量得分高于同龄健康儿童相应维度得分(P0.05);5岁~ARM患儿生存质量各维度得分及总分均高于同龄健康儿童得分(P0.05),其中情感功能生存质量得分差异最大。2)5岁~ARM儿童自评生存质量得分高于家长报告得分(P0.05);5~岁健康儿童自评生存质量各维度得分高于家长报告得分(P0.05)。3)2岁~和5岁~两组伴有排便障碍的ARM患儿生存质量相应维度得分低于不伴有排便障碍的患儿(P0.05)。结论 1)生存质量可以通过减少心理压力,给予更多的关爱与照护得到提高,尤其在情感功能方面,家长给予了特别关注。2)排便障碍对2岁~和5岁~ARM患儿的生存质量有着不可忽视的负面影响。     

A search for faecal viruses in new-born and other infants.

Faecal specimens were collected at weekly intervals over the winter months from 141 new-born infants without diarrhoea. Contrary to the findings in other studies, no viruses were detected by electron micriscopy or culture in any of these specimens. Over the same period faecal specimens were collected from 84 infants up to four years of age admitted to hospital. Rotaviruses or adenoviruses were found in 48% of infants with gastroenteritis. Enteroviruses and other small round virus-like particles were found in infants both with and without gastroenteritis. No viruses or pathogenic bacteria could be found in 34% of specimens from infants with gastroenteritis.     

No evidence for congenital malformations or prenatal death in infants born to women with a high dietary intake of fish contaminated with persistent organochlorines

Objectives: The aim of the present study was to assess whether a high dietary intake of fatty fish from the Baltic Sea, contaminated with persistent organochlorine compounds, might increase the risk for congenital malformations and perinatal death. Methods: A cohort of fishermen's wives from the Swedish east coast (at the Baltic Sea) were linked to the Swedish Medical Birth Register (MBR), resulting in the identification of 1501 infants born in the period 1973–1991. A further linkage with the Swedish Registry of Congenital Malformations was performed. Similar linkages were made for a comparison group of fishermen's wives from the Swedish west coast, who gave birth to 3553 infants during the study period. The reproductive end points studied included congenital malformations, stillbirths, and early neonatal deaths. The expected numbers of these end points were calculated from the MBR data for the regional populations. Results: In the east coast cohort, 3.3% of the infants had some malformation diagnosis as compared with 5.0% of the west coast cohort. As compared with the general population, somewhat fewer malformations than expected were diagnosed in the east coast cohort (risk ratio 0.78, 95% CI 0.58–1.04). No specific malformation was overrepresented in the east coast cohort. The risk ratios for stillbirths and early neonatal deaths did not significantly differ from unity in any of the cohorts. Conclusions: The present results exclude in an unequivocal way an association between exposure to persistent organochlorines from fatty Baltic Sea fish and an increased risk for all congenital malformations and perinatal death. The limited power of the study design, however, does not allow the exclusion of slight risk excesses for some specific types of malformation. Received: 28 May 1998 / Accepted: 22 October 1998     

Long-term follow-up of a vaccination program for infants born to HBsAg-positive mothers in Stockholm County,Sweden

We investigated the long-term antibody response to hepatitis B virus (HBV) vaccination in babies born to chronically infected mothers. They received one dose of monovalent HBV vaccination at birth and one month of age, followed by 3 doses of hexavalent vaccine including an HBV component at ages 3, 5, and 12 months, respectively, with a very high percentage of protective anti-HBs levels at 13 months. At the age of 8–12 years, 56 out of 68 children (82%) had protective levels of anti-HBs, two had signs of anti-HBc seroconversion without any history of clinical disease and none had ongoing infection. A small subgroup was retested after one booster dose, in all resulting in increase in anti-HBs from below 10 IU/L to levels corresponding to protective immunity. We conclude that this vaccination strategy is effective throughout the first decade of life in avoiding chronic infection and in maintaining a good serological response.     

Hydranencephaly. (Severe cerebral malformations in three newborn infants)

Three neonatal cases of hydranencephaly are reported. Clinical and radiological characteristics of this disease are also described. Factors which can have any role in the development of hydranencephaly are reviewed. The importance of intrauterine ultrasonography and the quick, early diagnosis is emphasized. Therapeutic approaches are also discussed.     

Massage interventions and developmental skills in infants born with low birth weight

This study aimed at evaluating possible effects of massage interventions on developmental progress in the infants born with low birth weight (LBW). Forty infants (17 boys, 23 girls) who were born in St. Petersburg in 2000 through 2002 and met a conventional definition of LBW (<2500 g at birth) entered the study. Of these, 36 (17 boys, 19 girls) were born both light and pre‐term (gestational age ≤36 weeks), and 4 (2 boys, 2 girls) were born light but at term. The control group consisted of an equal number of infants matched to cases for gender, gestational age, weight at birth, date of birth and geographical distribution. The infants from the case group were assigned to the massage intervention therapy at the age of two months. Infant Development Inventory (IDI) was used to check infant’s developmental progress in five areas: social, self‐help, gross motor, fine motor and language skills. The findings were that LBW infants who received massage intervention had advanced skills in all five areas at the age three through eight months. Revealed associations between massage and certain features of advanced behavioural outcomes remained significant after adjustment was made for major potential confounders. Massage can be undertaken to stimulate development in the infants born with LBW.     

Survival of infants born with Down's syndrome: 1980-96

The improved life expectancy of people with Down's syndrome as a result of the greater availability of surgery and advances in medical care has been widely documented. However, there has been no evaluation of survival in the Australian Down's syndrome population since the 1980s. This study aimed to evaluate the changes in survival from birth in cases of Down's syndrome notified to the Birth Defects Registry in Western Australia. Babies born with Down's syndrome between 1980 and 1996 (inclusive) and registered with the Birth Defects Registry were studied. Survival status was obtained in several ways. Cases were stratified into three cohorts for comparison. Survival curves were constructed using the methods of Kaplan and Meier. For infants born during 1980-96, survival to 1 year is now > 91%, and 85% can expect to survive until the age of 10 years. Although survival in those with heart disease showed improvement over the period studied, overall this was still a strong predictor of mortality. Survival in Aboriginal children with Down's syndrome was significantly poorer than in non-Aboriginal children, mirroring the pattern in the general population. Mortality was greater in females and in those with a low birthweight. There was no statistically significant difference in the survival between those born in metropolitan and in rural areas. There has been a considerable improvement in survival of infants born with Down's syndrome in Western Australia. This improvement is similar to findings in recent international studies. The difference in survival between Aboriginal and non-Aboriginal children is particularly disturbing. These findings are useful for both clinicians and families who need to plan for the long-term care of these children.     

Mortality among infants with congenital malformations,New York State, 1983 to 1988.

OBJECTIVE. The authors examined first-year mortality and risk factors for mortality among infants with major congenital malformations. METHODS. Infants with major congenital malformations born from 1983 to 1988 were identified from a statewide population-based congenital malformations registry. Variables analyzed included year of birth, birth weight, gestational age, infant sex, number of malformations, number of organ systems involved, level of care of the birth hospital, maternal age, maternal education, and maternal ethnicity. RESULTS. Infants with major malformations had a risk of death 6.3 times higher than the general population of live births. The risk declined from 6.5 in 1983 to 5.9 in 1988. Birth weight and number of malformations were the strongest risk factors. The likelihood of survival was similar for white and black infants. CONCLUSIONS. Being born with a malformation outweighs most of the other risks for infant mortality. Children with congenital malformations had higher cause-specific mortality for all causes except injury.     

糖尿病母亲的婴儿血脂分析

目的:探讨糖尿病母亲婴儿血脂情况。方法:对北京大学人民医院2005年1月～2010年12月收治的63例糖尿病母亲婴儿的相关资料进行回顾性分析,比较糖尿病母亲婴儿与正常新生儿血脂情况有无差异,并分析其血脂与出生体重、胎龄、性别及母亲血糖控制情况的相关性。结果:与正常新生儿相比,糖尿病母亲婴儿血胆固醇〔(2.50±0.91)vs(1.76±0.47)mmol/L〕、甘油三酯〔(0.70±0.50)vs(0.38±0.17)mmol/L〕、高密度脂蛋白〔(0.73±0.23)vs(0.51±0.14)mmol/L〕、低密度脂蛋白〔(1.23±0.62)vs(0.78±0.40)mmol/L〕均增高,差异有显著性。与早产糖尿病母亲婴儿相比,足月产糖尿病母亲婴儿血甘油三酯〔(1.04±0.58)vs(0.48±0.34)mmol/L〕增高,高密度脂蛋白〔(0.64±0.17)vs(0.78±0.24)mmol/L〕降低,差异有显著性。与适于胎龄糖尿病母亲婴儿相比,大于胎龄糖尿病母亲婴儿血脂无差异。与母亲血糖控制良好组相比,母亲血糖控制欠佳组婴儿血脂无显著差异。与糖尿病母亲男性婴儿相比,糖尿病母亲女性婴儿血脂亦无统计学差异。糖尿病母亲婴儿血甘油三酯与出生体重、胎龄呈正相关,血高密度脂蛋白与出生体重、胎龄呈负相关,血胆固醇及低密度脂蛋白与出生体重、胎龄无相关性。结论:糖尿病母亲婴儿的血脂水平较正常新生儿增高,其中足月者较早产者血甘油三酯增高,高密度脂蛋白减低。糖尿病母亲婴儿血甘油三酯与体重、胎龄呈正相关,高密度脂蛋白与体重、胎龄呈负相关。     

Birthweight of infants born to Hispanic women employed in agriculture

A prospective study examined the effects of maternal occupation on the birthweight of infants of Hispanic women. The study population consisted of 1,040 pregnant women enrolled for prenatal care in two rural health care centers in California between 1982 and 1984. Birthweights of infants were examined after classifying these women into three groups: (1) 255 mothers who did agricultural work, (2) 134 mothers who worked in nonagricultural jobs, and (3) 651 mothers who did not work during pregnancy. Prenatal interviews and medical records revealed medical, social, and occupational histories of the mothers. Stepwise linear regression analyses, using the backward elimination method, were conducted for some exposure models to determine whether work category influenced birthweight after controlling for confounders. The incidence of low birthweight and mean birthweights did not vary significantly with work status.     

A modified method for the epidemiological analysis of registry data on infants with multiple malformations.

BACKGROUND: Infants with multiple malformations are important in birth defect epidemiology and malformation monitoring because human teratogens have often caused complex malformations. Various methods for the analysis of multimalformed infants have been tried. METHOD: By using data from four large registries of congenital malformations, 5256 infants were identified with two or more among 73 selected malformations. Pairwise associations between malformations were detected by multiple logistic regression analyses, and putative confounders (programme, maternal age, autopsy, etc.) were controlled for. For each significant pairwise association, further analyses were performed in order to find associations with a possible third malformation. RESULTS: The importance of controlling for several confounders was demonstrated. Several well-known associations were found, which supports the technique used. The interpretation of three-way associations was discussed. Results from the present study were compared with those obtained using some other methods. CONCLUSIONS: Different confounders can cause biased associations. The method presented in the paper takes this into consideration and is therefore more likely than previously used techniques to give unbiased information on the clustering of different malformations among multimalformed infants.     

妊娠高血压母亲对新生儿血液细胞学的影响

【目的】研究妊娠高血压对新生儿血液细胞学的影响以及这些变化是否对新生儿预后产生不利影响。【方法】研究242例妊娠高血压母亲出生的新生儿的血液细胞学的变化,与140例无妊娠高血压母亲分娩的新生儿进行对比研究。【结果】妊娠高血压组中早产儿及小于胎龄儿的比例明显增高。妊娠高血压组新生儿血小板计数明显低于对照组(P<0.05),但并没有增加新生儿出血性疾病的发生率(如颅内出血);妊娠高血压组新生儿有核红细胞的数量及平均红细胞体积比正常对照组明显增加;两组新生儿的中性粒细胞计数差异无显著性(P>0.05)。【结论】妊娠高血压组新生儿在血液细胞学的某些方面与对照组新生儿存在明显的差异,但这些差异没有导致新生儿相关疾病(如败血症、颅内出血等)的发生率的明显增加。     

先天性肛门直肠畸形患儿父母生活质量和社会支持研究进展

先天性肛门直肠畸形是一种常见的消化道畸形。手术治疗虽然挽救了患儿生命,但是术后肛门失禁、狭窄和便秘等并发症给患儿的身心健康带来严重影响。父母作为主要照顾者,既承担照顾患儿的繁重任务,又面临着沉重的经济和精神负担,成为亟待关注的群体。本文就先天性肛门直肠畸形患儿父母生活质量和社会支持的研究进展进行综述,以期为改善该人群生活质量提供参考依据。     

Noise exposure during pregnancy and selected structural malformations in infants

The study tested the hypothesis of exposure to noise during pregnancy being teratogenic. It included 1,475 Finnish mothers who had given birth to a malformed child (orofacial cleft or structural defect of the central nervous system, skeleton, or heart and great vessels) and 1,475 reference mothers. A special interview soon after delivery yielded the primary information on exposure. Of the 783 mothers who reported noise exposure in the first trimester, 370 were case mothers and 413 were referents. Hygienic assessment indicated that 102 case mothers and 103 referents had been exposed in the first trimester to a sound level of around 80 dB LAeq(8 h) or higher, the overall odds ratio being 1.0 (95% confidence interval 0.7-1.3). Adjustment for potential confounders by logistic regression methods gave similar results. There was no obvious trend suggesting a hazard when different exposure categories were considered.