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1.

Background

Lenticulostriate vasculopathy (LSV) is a hyperechogenicity of the lenticulostriate branches of the basal ganglia and/or thalamus' middle cerebral arteries and is frequently seen in neonatology. Our study primarily describes the perinatal data and long-term follow-up of newborns with lenticulostriate vessel hyperechoic degeneration. Secondly, it describes the cerebral imaging data as a function of perinatal factors and neurodevelopmental follow-up of these newborns.

Methods

This retrospective study assesses the outcome of newborns with LSV hyperechogenicity on cerebral ultrasound (two grades). These children were born between January 2008 and September 2015 and were treated in a large level III neonatal intensive care unit. Thirty-four term-equivalent age children underwent MRIs using a standardized protocol of T2, T1 3D, diffusion and spectro-MRI sequences. The MRIs retrospectively measured the white matter and basal ganglia apparent diffusion coefficients (ADC).

Results

Fifty-eight neonates, ranging from 25 to 42 weeks gestational age (GA), were diagnosed with LSV. There was a significantly increased high-grade LSV when accompanied by fetal heart rate abnormalities (p = 0.03) and the neonate's need for respiratory support at birth (P = 0.002). The mean ADC score was substantially superior in the high-grade versus the low-grade LSVs (p = 0.023). There were no noteworthy outcome differences between a high and low grade LSV. The mean ADC for basal ganglions was appreciably higher in children with a severe prognoses (death or developmental disorder) as compared to children with no abnormalities (p < 0.01).

Conclusion

From the results of our study, it appears that a low-grade LSV could be considered as a normal variant. There are no unifying diagnostic criteria for LSV on cerebral ultrasound. With a cerebral MRI, the use of ADC values of basal ganglia may well underscore the importance of such data in predicting long-term outcomes.  相似文献   

2.

Background and purpose

Safety and efficacy of intravenous (IV) thrombolysis and endovascular therapy in children with acute ischemic stroke (AIS) are unknown to date. We aimed to review and synthesize currently available evidence on these acute recanalization therapies in pediatric stroke patients.

Methods

We performed a systematic review and meta-analysis of all available data on safety and efficacy of acute treatment including thrombolysis and endovascular therapy in pediatric AIS patients aged <18 years. We searched the electronic databases Medline and Cochrane Library for eligible studies published from the earliest date available until August 31, 2016. Safety outcomes included intracerebral hemorrhage (ICH) post-treatment and in-hospital mortality. Efficacy outcomes included functional outcome 3–6 months after index stroke.

Results

We identified 222 records, of which 3 studies with a total of 16,987 pediatric stroke patients met our eligibility criteria of whom 181 received IV thrombolysis. No data exists from randomized trials and no data is available on endovascular thrombectomy. Risk of any ICH was increased in children receiving thrombolysis (risk ratio = 3.48, 95%CI: 1.66–7.29; p = 0.001) compared with controls, with no evidence of heterogeneity (I2 = 0%). None of the included studies reported complete data on symptomatic ICH. In-hospital mortality was similar between pediatric stroke patients treated with thrombolysis and controls (risk ratio = 1.44, 95%CI: 0.39–5.40; p = 0.586), with evidence of heterogeneity (I2 = 62%). Efficacy of revascularization therapies could not be analyzed due to lack of outcome data.

Conclusions

Our analyses demonstrate a substantial lack of data on efficacy and safety of acute recanalization therapies in children with AIS.

PROSPERO Registration Information

URL: http://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42016047140.  相似文献   

3.

Background

The aim of this study was to assess the incidence rate and prevalence of autoimmune myasthenia gravis (MG) among children in Norway.

Methods

This retrospective population-based study was performed in Norway from January 2012 to December 2013. Cases of juvenile MG (JMG) with onset < 18 years were identified through searches in coding systems of electronic patient records at the 15 main hospitals in Norway from 1989 to 2013. In addition, the acetylcholine receptor antibody database at Haukeland University Hospital and the clinical nationwide MG database at Oslo University Hospital were searched for cases of JMG. Diagnosis and age at onset were verified through medical records. Incidence and prevalence rates were calculated using the Norwegian population as reference.

Results

In total 63 unique JMG cases were identified. This corresponds to an average annual incidence rate of 1.6 per million. Incidence rate was stable over the study period. Prevalence of JMG was 3.6–13.8 per million. Females constituted the majority of JMG cases (55 vs 8 males). The risk of JMG was higher among females both in the postpubertal and prepubertal group (p < 0.001 and p = 0.02, respectively).

Conclusion

This study confirms the rarity of JMG in Norway, especially among males, and shows a stable incidence rate over the last 25 years.  相似文献   

4.

Introduction

Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM).

Methods

We retrospectively reviewed the medical records of patients seen at the Balance and Vestibular Program at Boston Children's Hospital between January 2012 and December 2016 who were diagnosed with BPTI, BPVC, and/or VM.

Results

Fourteen patients were diagnosed with BPTI, 39 with BPVC, and 100 with VM. Abnormal rotary chair testing was associated with progression from BPTI to BPVC (n = 8, p = 0.045). Eight (57.1%) patients with BPTI and 11 (28.2%) with BPVC had motor delay. Eleven (78.6%) patients with BPTI and 21 (53.8%) with BPVC had balance impairment. Six BPTI patients developed BPVC (42.9%), six BPVC patients developed VM (15.4%), and two patients progressed through all three disorders (2%). One BPTI patient progressed directly to VM.

Discussion

Most patients with BPTI will experience complete resolution in early childhood, but some will progress to BPVC, and similarly many patients with BPVC will progress to VM. Parents of children with these disorders should be made aware of this phenomenon, which we refer to as “the vestibular march.” Children with BPTI and BPVC should also be screened for hearing loss, otitis media, and motor delay.  相似文献   

5.

Background

Data on paediatric deep brain stimulation (DBS) is limited, especially for long-term outcomes, because of small numbers in single center series and lack of systematic multi-center trials.

Objectives

We seek to systematically evaluate the clinical outcome of paediatric patients undergoing DBS.

Methods

A German registry on paediatric DBS (GEPESTIM) was created to collect data of patients with dystonia undergoing DBS up to the age of 18 years. Patients were divided into three groups according to etiology (group 1 inherited, group 2 acquired, and group 3 idiopathic dystonia).

Results

Data of 44 patients with a mean age of 12.8 years at time of operation provided by 6 German centers could be documented in the registry so far (group 1 n = 18, group 2 n = 16, group 3 n = 10). Average absolute improvement after implantation was 15.5 ± 18.0 for 27 patients with pre- and postoperative Burke-Fahn-Marsden Dystonia Rating scale movement scores available (p < 0.001) (group 1: 19.6 ± 19.7, n = 12; group 2: 7.0 ± 8.9, n = 8; group 3: 19.2 ± 20.7, n = 7). Infection was the main reason for hardware removal (n = 6). 20 IPG replacements due to battery expiry were necessary in 15 patients at 3.7 ± 1.8 years after last implantation.

Discussion

Pre- and postoperative data on paediatric DBS are very heterogeneous and incomplete but corroborate the positive effects of DBS on inherited and acquired dystonia. Adverse events including relatively frequent IPG replacements due to battery expiry seem to be a prominent feature of children with dystonia undergoing DBS. The registry enables collaborative research on DBS treatment in the paediatric population and to create standardized management algorithms in the future.  相似文献   

6.

Objective

Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors.

Methods

We retrospectively included patients with ESES syndrome treated in our center. Treatment effect was analyzed on sleep EEG spike wave index (SWI) and cognitive functioning.

Results

47 patients had 147 (43 steroid and 104 non-steroid) treatments. Cognitive improvement was reported after 36% of treatments at first follow-up and 45% of treatments at last follow-up. The median SWI change for treatments resulting in subjective cognitive improvement was ?44%, and 0% for those not resulting in subjective cognitive improvement at first follow-up (p = 0.008) and ?50% vs. +5% at last follow-up (p = 0.002). No clear association between subjective cognitive improvement and IQ change, and between SWI and IQ change was found. By means of logistic regression we found that steroid treatment, as compared to non-steroid treatment, was associated with cognitive improvement at first follow-up (multivariate OR after multiple imputation 2.5, 95% CI 1.1–5.7), while at last follow-up, higher age at diagnosis was related to cognitive improvement only in univariate analysis (OR 1.02, 95% CI 1.01–1.04).

Conclusions

We found that in children with ESES, cognitive improvement after treatment was strongly associated with SWI decrease, while it was not reflected by a significant IQ increase. Steroid treatment was most successful in improving cognitive performance.  相似文献   

7.

Background

In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort.

Aim

To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions.

Methods

Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m–4y 3m). All eligible patients (n = 24, years 2009–2014) were included.

Results

Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m–19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1–9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes.

Interpretation

SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR.  相似文献   

8.

Aim

We aimed to identify early predictors of intractable epilepsy, intellectual disability (ID) and autism spectrum disorders (ASD) in the cohort of TSC patients initially diagnosed with cardiac rhabdomyomas (CR).

Method

Over the period of twelve years we prospectively obtained clinical, neuropsychological, electrophysiological and neuroimaging data in a group of 22 TSC patients (9 females, 13 males) with the pre/perinatal diagnosis of CR, included to the study at the time of diagnosis. Afterwards, we statistically determined variables associated with ID, ASD and intractable epilepsy.

Results

Development of ID was predicted by severe epilepsy (a higher number of anti-epileptic drugs used), a higher number of dysplastic lesions on MRI, and abnormal background activity on EEG (p < 0.05). Predictors of ASD included early developmental delay, abnormal background activity on EEG at the end of follow-up and a higher number of areas with dysplastic features on MRI (p < 0.05). Intractable epilepsy was associated with a higher number of areas with dysplastic features on MRI, ID and with TSC2 genotype.

Conclusion

Adverse mental and clinical outcome was associated with intractable epilepsy and the severe anatomical brain involvement; therefore, our centre developed a tailored protocol for early identification of TSC patients at a higher risk of developing intractable epilepsy with its deleterious effect on cognitive outcome.  相似文献   

9.

Objectives

The aim of this systematic review was to examine the incidence and prevalence of comorbidity between Cerebral Palsy (CP), Autism spectrum disorders (ASDs) and Attention-Deficit/Hyperactivity Disorder (ADHD).

Methods

We searched for articles indexed in PubMed, EBSCOhost, Scopus, Web of Science and other potentially relevant internet sources using a combination of expressions including “cerebral palsy” AND “autism” OR “ASD” OR “pervasive development disorder” AND “Attention Deficit Hyperactivity Disorder” OR “ADHD”.

Results

We identified 2542 studies on CP and ASD and 998 studies on CP and ADHD. After screening titles and abstracts and removing duplicated studies, 47 full papers (CP and ASD n = 28; CP and ADHD n = 19) were downloaded and screened for eligibility. Twenty-eight (CP and ASD n = 16; CP and ADHD n = 12) studies were identified in the peer-review literature. Based on this systematic review, ASD and ADHD seem to be more common in people with CP than in the general population, yet the gold standard methods for diagnosing ASD or ADHD are not suitable for children with motor problems.

Conclusions

Assessing the occurrence of ASD and ADHD would improve the significant cost of healthcare, therapies, and overall daily living for families with children affected by CP. However, psychometric studies are needed in the future to promote development of measures suitable for individuals with CP. In addition, this review highlights the paucity of peer-reviewed studies investigating the occurrence of ASD and ADHD in children with different CP subtypes or functional abilities, and there are still some open questions about pathogenic mechanisms common to CP, ASD and ADHD.  相似文献   

10.

Background

The use of complementary and alternative medicine (CAM) has been associated with adverse effects and self-imposed dietary restrictions. The prevalence of its use in Asian children with inflammatory bowel disease (IBD) is unknown. We aimed to determine the prevalence, types, and factors associated with the use of CAM among children with IBD from Singapore and Malaysia, and to ascertain if dietary restriction was prevalent in patients who used CAM.

Methods

A cross-sectional study was conducted in which parents of children with IBD attending two tertiary pediatric IBD referral centres in Singapore and Malaysia were interviewed. Data about demographics, conventional treatment, complementary therapies and dietary patterns were collected in a questionnaire.

Results

Of 64 children with IBD interviewed, 83% (n = 53) reported the use of CAM (Singapore [90%] vs. Malaysia [76%]; p = 0.152). The median number of CAM agents used was two (range 1–10). The three most common types of CAM used were probiotics (64%), vitamin and mineral supplements (55%), and food-based therapies (36%). Among individual CAM categories, the use of food-based therapies was correlated significantly with nationality (r = 0.497, p < 0.001), history of weight loss due to IBD (r = 0.340, p = 0.013) and avoidance of certain foods to prevent a relapse (r = 0.289, p = 0.036). Parents who rated their child's disease activity as more severe were less likely to use CAM (r = ?0.257, p = 0.041). Fifty-nine percent of CAM users reported physician awareness of their CAM use. The overall self-perceived efficacy of CAM in improving IBD symptoms was 34%. Of the dietary patterns explored, only intake of dairy products was associated with CAM use (r = 0.306, p = 0.019).

Conclusion

Use of CAM is prevalent in children with IBD in Malaysia and Singapore. Further studies to elucidate reasons influencing CAM use, dietary patterns and efficacy of commonly used CAM would be required.  相似文献   

11.

Study objectives

To assess spindle activity as possible markers for neurocognitive consequences in children with mild obstructive sleep apnea.

Methods

Children aged 6–11 years diagnosed with mild OSA (i.e., an apnea hypopnea index <5.0) were recruited and compared with age and gender-matched healthy controls. Polysomnographic recordings were analyzed for sleep microstructure and spindle activity. All children completed also an intelligence test battery (i.e., the Wechsler intelligence test for children, 4th version).

Results

Nineteen children with OSA (13 boys, mean age 7.1 ± 1.4 y), and 14 controls (7 boys, mean age 8.1 ± 1.9 y) were included. Mean IQ was 110 ± 12 for the complete sample, in children with OSA 111 ± 13, and in controls 108 ± 12 (p = 0.602). Controls showed a higher spindle index in N2 stage than children with OSA: 143.0 ± 42.5 vs 89.5 ± 56.9, respectively (p = 0.003). Spindle index in NREM was strongly and significantly correlated with Verbal Comprehension Index (VCI), Working Memory Index (WMI), Processing Speed Index (PSI), and total IQ in children with OSA.

Conclusions

Children with mild OSA demonstrate a different pattern of sleep spindle activity that seems to be linked with neurocognitive performance, especially concerning memory. Sleep spindle activity seems to be involved with mechanisms related with neurocognitive consequences in children with OSA.  相似文献   

12.

Introduction

Many patients with cerebral palsy (CP) suffer chronic pain as one of the most limiting factors in their quality of life. In CP patients, pain mechanisms are not well understood, and pain therapy remains a challenge. Quantitative sensory testing (QST) might provide unique information about the functional status of the somatosensory system and therefore better guide pain treatment.

Objectives

To understand better the underlying pain mechanisms in pediatric CP patients, we aimed to assess clinical and pain parameters, as well as QST profiles, which were matched to the patients' cerebral imaging pathology.

Patients and methods

Thirty CP patients aged 6–20 years old (mean age 12 years) without intellectual impairment underwent standardized assessments of QST. Cerebral imaging was reassessed. QST results were compared to age- and sex-matched controls (multiple linear regression; Fisher's exact test; linear correlation analysis).

Results

CP patients were less sensitive to all mechanical and thermal stimuli than healthy controls but more sensitive to all mechanical pain stimuli (each p < 0.001). Fifty percent of CP patients showed a combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia; 67% of CP patients had periventricular leukomalacia (PVL), which was correlated with mechanic (r = 0.661; p < 0.001) and thermal (r = 0.624; p = 0.001) hypoesthesia.

Conclusion

The combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia in our CP patients implicates lemniscal and extralemniscal neuron dysfunction in the thalamus region, likely due to PVL. We suspect that extralemniscal tracts are involved in the original of pain in our CP patients, as in adults.  相似文献   

13.

Objective

To investigate health-related quality of life (HrQoL) and adaptive behavior in young people with narcolepsy and stress among their parents.

Methods

In a cross-sectional exploratory quantitative study design, 37 young people with narcolepsy (8–20 years of age) and their parents were recruited. Thirty-one had post-H1N1 vaccination-related narcolepsy (PHV) and six had narcolepsy not related to PHV (nPHV). In addition, 40 age- and gender-matched controls (aged 5–20 years) were recruited.

Results

Thirty-one patients completed the generic HrQoL questionnaire KIDSCREEN and the disease-specific NARQoL-21. HrQoL was found to be significantly diminished in all domains in the PHV group (p = 0.001) and in the School/Concentration domain (p = 0.004) in the nPHV group compared to age- and gender-matched controls. The Adaptive Behavior Assessment System was completed by parents of 32 patients. They rated their children significantly lower in the General adaptive composite (p = 0.026) and the Conceptual (p = 0.050) and Social composite scores (p = 0.001) compared with reference data on healthy Swedish children's and young people's adaptive behavior. Parents of 36 patients filled in the 36-item short form of the Parenting Stress Index questionnaire. They rated significantly higher Total stress, Parent-child dysfunctional interaction, and Difficult child scores compared with parents of controls (p = 0.001, p = 0.005, p < 0.001).

Conclusions

Children with narcolepsy have diminished HrQoL compared with controls. Parents of children with narcolepsy experience impaired adaptive behavior in their children and high levels of parenting stress. Identifying the contributory factors is necessary, and early intervention is crucial in order to improve the HrQoL of these children and their families.  相似文献   

14.

Background

Ataxia telangiectasia (AT) is a neurodegenerative cerebellar disorder, caused by mutations in the ATM gene, involved in DNA repair. Radiosensitivity, progressive ataxia, immune deficiency and malignancies, are well known symptoms, but urological manifestations are scarcely described.

Objective

To characterize urologic manifestations in a large cohort of AT patients.

Methods

Retrospective cross-sectional chart study comprising 52 AT patients followed at a National AT Center.

Results

25% of the cohort (13 patients/8 males) had urologic symptoms, which presented at 11 ± 4.3 years. The most common symptom was secondary enuresis affecting 15% of the patients (8 children/4 males). Incontinence appeared at 8 ± 6.2 years of age, and resolved spontaneously within 15 ± 8.3 months in 6 patients. It preceded loss of ambulatory capacity by 1–2 years in 7 patients. Lumbosacral MRI were normal (4 children) and urine cultures (all) were negative. Urodynamic evaluation that was performed in only one patient revealed overactive bladder. Additional manifestations were macroscopic hematuria due to bladder telangiectasia in a 12-year-old, and renal cell carcinoma in a 22-year-old. Other manifestations unrelated to AT were neprolithiasis, vesico-ureteral reflux and scrotal pain, each in 1 patient.

Discussion

Transient secondary enuresis is a frequent finding in AT patients, heralding loss of ambulatory capacity, tough it's pathophysiological mechanism is largely no understood.  相似文献   

15.

Background

Maternal trauma complicates pregnancy in approximately 7%. Long-term development of children exposed to maternal trauma is unknown.

Aim

To determine neurobehavioural outcome of children (6–18 years) born after maternal trauma in pregnancy compared to a matched control group.

Study design

Case-control study performed at a tertiary medical centre.

Subjects

All consecutive children born after maternal hospitalization for trauma during pregnancy between 1995 and 2005. Controls were children born at the same hospital and period after an uneventful pregnancy.

Outcome measures

Trauma type and severity (Injury Severity Score, ≥9: severe); information from medical files at admission (cases). All mothers filled out two questionnaires about the infant; 1. concerning health, motor development and educational level, 2. concerning behavioural development through the validated Dutch version of the Child Behavior Checklist (CBCL).

Results

Questionnaires were returned by 34 cases and 28 controls. The traumas concerned mainly motor vehicle accidents and falls, and 3/34 had severe injuries. No differences in health, motor development, educational level and CBCL was found between the cases and controls, except for more hospitalization in the cases (p = 0.009).

Conclusion

Long-term follow-up of a limited population of children 6–18 years after exposure of mainly non-severe trauma before birth is similar to a control population except for unexplained more hospitalization in the cases.  相似文献   

16.

Background

Headache on wakening (HoW) or sleep interruption secondary to headache (SIH) has been commonly regarded as a potential sign of raised intracranial pressure and therefore a sign of significant underlying pathology that necessitates further investigation. Current recommendations for neuroimaging in patients with HoW/SIH are neither consistent nor clear across headache guidelines published both nationally and internationally.

Aim

The main aim of this study was to ascertain the relevance of HoW and/or SIH as an indication for routine neuroimaging.

Methods

This study focused on clinically well patients with normal neurological examinations who had experienced HoW or SIH. Demographic and neuroradiological data were collected prospectively and the headache diagnosis was based on the International Classification of Headache Disorders.

Results

102/1065 patients reported either HoW and/or SIH. There were 57/102 (56%) females, 45/102 males (44%), and 33/102 (32%) of ethnic minority. Their age ranged between 5 and 17 years. 79/102 (77%) patients with HoW, 19/102 (19%) with SIH and 4/102 (4%) with both HoW and SIH. Headache diagnosis included migraine (n = 67; 66%), tension type headaches (n = 16; 16%), medication overuse headaches (n = 11; 11%), and sinusitis (n = 1; 1%). Neuroimaging was performed in 101/102 patients; imaging was normal for 97 scanned patients, and showed non-significant abnormality in the remaining 4 patients.

Conclusion

HoW or SIH among clinically well and neurologically normal paediatric patients was most likely to be caused by primary headaches, particularly migraine or tension type headaches. This symptom alone among healthy and clinically well children is not an indication for routine neuroimaging and is unlikely to be caused by sinister aetiologies.  相似文献   

17.

Objective

To evaluate relationships between whole blood (B-Al) and hair aluminum (H-Al) levels in healthy infants and their immunization history and development.

Methods

We conducted a cross-sectional study of 9- to 13-month-old children recruited from an urban primary care center, excluding those with a history of renal disease or receipt of either aluminum-containing pharmaceuticals or parenteral nutrition. Aluminum levels were measured using inductively coupled plasma-mass spectrometry. Correlation with Bayley Scales of Infant and Toddler Development, Third Edition (BSID) and vaccine-related aluminum load was assessed via linear regression models.

Results

The median age of 85 participants was 287 days. B-Al (median, 15.4 ng/mL; range, 0.9–952 ng/mL) and H-Al (median 42,542 ng/g; range, 2758–211,690 ng/g) were weakly correlated (Spearman ρ = 0.26; P = .03). There was no significant correlation between B-Al or H-Al and estimated aluminum load from vaccines. B-Al was not correlated with BSID composite or subscale scores. Although H-Al was not correlated with BSID scores in models including all data (n = 85), it was inversely correlated with motor composite (P < .02; Wald = 5.88) and the gross motor subscale (P = .04; Wald = 4.38) in models that excluded an extreme outlying H-Al value.

Conclusions

Infant B-Al and H-Al varied considerably but did not correlate with their immunization history. Likewise, there was no correlation between B-Al and infant development or between H-Al and language or cognitive development. An inverse correlation between H-Al and BSID motor scores deserves further investigation.  相似文献   

18.

Objective

To establish the local incidence of hearing loss in newborns with Hypoxic Ischaemic Encephalopathy (HIE) and to identify associated risk factors.

Study design

Retrospective Cohort Study. Neonatal Intensive Care Unit (NICU) dual stage hearing screening protocol, including automated otoacoustic emissions (AOAE) and automated auditory brainstem response (AABR) testing.

Results

57 newborns received therapeutic hypothermia for HIE. Twelve babies (21%) died. Audiology data was incomplete in 3 babies. Complete data was available for 42 babies (male n = 24), 4 (9.5%) of whom had hearing impairment. The development of hearing loss was associated with abnormal blood glucose levels (p = 0.006), low Apgar score at 1 min (p = 0.0219) and evidence of multi organ dysfunction [high creatinine (p = 0.0172 and 0.0198) and raised liver transaminases (aspartate aminotransferase (AST) p = 0.0012, alanine aminotransferase (ALT) p = 0.0037)]. An association with gentamicin was not found.

Conclusion

This study confirms that hearing impairment is common in term infants who have undergone therapeutic hypothermia for moderate/severe HIE. Blood glucose should be monitored carefully in these infants and developmental surveillance should include formal audiology. Further larger studies are needed to clarify the role, if any, of hypothermia per se in causation of hearing loss and to fully identify risk factors for hearing impairment in this population.

What is new

The current study confirms that hearing impairment is common in term infants who have undergone therapeutic hypothermia for moderate/severe HIE.No association between gentamicin use and the development of hearing impairment was found however initial blood glucose outside the normal range was of significance.Other factors associated with hearing impairment were low Apgar scores, greater need for resuscitation and evidence of multi organ dysfunction (renal and liver failure).  相似文献   

19.

Aim

To analyse trends in prevalence and severity of cerebral palsy (CP) in Denmark in birth years 1999–2007 and compare with previous periods.

Method

Data has been collected uniformly in the Danish cerebral palsy national register nationwide since 1995. Rates in the time periods 1999–2001, 2002–2004 and 2005–2007 covering 585,393 births were analysed by gestational age and subtypes.

Results

Total number of CP cases in the period was 1165. The overall prevalence of CP decreased significantly from 2.1 in 1999–2001 to 1.8 in 2005–2007 per 1000 livebirths (p = 0.022). The decline was only significant for children born at term (p = 0.007) but not for the preterm (p = 0.44). The decline in children born at term was based on a decrease in bilateral spastic CP (n = 117 in years 1999–2001 and n = 59 in 2005–2007). Multidisciplinary obstetric skills training with neonatal resuscitation in Denmark was initiated in 2003 and timely associated with the decrease. The prevalence of unilateral spastic CP the prevalence did not change, but in the two last time periods more children had a right-sided than left-sided unilateral spastic CP.

Conclusion

The decline in rate of CP seen in 2005–2007 as compared to 1999–2001 was mainly based on fewer cases of severe spastic CP in term infants. We hypothesize that improved neonatal resuscitation in the delivery room may be partly responsible for the decrease. In premature children the decline was not significant in this time period, but has been dramatically decreasing in the years before the time period here analysed.  相似文献   

20.

Introduction

Shapiro syndrome (SS) is characterized by spontaneous recurrent episodes of hypothermia, hyperhidrosis and corpus callosum (CC) agenesis. Less than 60 cases have been reported to date and the pathogenic mechanism as well as the prognosis of this syndrome are still debated. We describe the clinical features and long-term follow-up of a pediatric cohort of SS patients.

Methods

We collected 13 (10 novel) pediatric cases of SS and report their long-term follow-up and neurological outcome.

Results

All patients experienced recurring hypothermia, with body temperature below 35 °C during the episodes, often accompanied by hyperidrosis. CC agenesis was an inconstant structural feature in the present series (2/13 patients). Seven patients received antiepileptic drugs (AEDs) or other drug therapy for a mean period of 12 months. At long-term follow-up (mean = 61 months, range: 60–96), all individuals were free from episodes of paroxysmal hypothermia independently from previous AED use or other drug therapy.

Conclusion

Paroxysmal hypothermia, the core symptom of SS, behaved as a age-dependent feature in our cohort, supporting a good long-term prognosis for SS. A prompt diagnosis of SS is crucial to avoid unnecessary diagnostic investigations.  相似文献   

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