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目的探讨儿童非惊厥性癫痫持续状态(NCSE)的临床表现、脑电图(EEG)特征、治疗及预后,提高诊疗水平。方法对2005年1月至2011年4月山东大学附属省立医院小儿神经科病房收治的12例NCSE患儿临床及EEG资料进行分析,并随访治疗疗效及预后。结果 12例中男8例、女4例,平均起病年龄(6.22±3.72)岁,发作持续时间平均为35h,失神癫痫持续状态(ASE)3例,精神运动性癫痫持续状态(PSE)9例。其中意识障碍12例,语言表达障碍9例,精神行为异常8例,复杂自动症5例,幻觉及饮食增多、烦渴各2例,自主神经症状1例。发作期EEG背景节律活动均变慢,3例为全导持续性棘慢综合波、慢波发放;9例为周期性或持续性限局性癫痫性放电,以θ波或δ波为主5例,以棘(尖)慢波为主4例,单纯颞区放电3例,颞外区放电2例,颞区合并颞外区放电4例。静注安定后癫痫性放电控制率在ASE组和PSE组差异无统计学意义(P>0.05)。6例患儿出现认知障碍。结论意识、语言障碍及精神行为异常为NCSE主要表现,发作期EEG是诊断的主要依据,及时有效地控制发作可减少对认知功能的损害。     

Nonconvulsive status epilepticus in pediatric populations: diagnosis and management

The diagnosis and management of nonconvulsive status epilepticus (NCSE) pose special challenges in childhood. NCSE can be defined as a condition of ongoing or intermittent clinical epileptic activity without convulsions, for at least 30 minutes, with electroencephalographic evidence of seizures. NCSE accounts for approximately one-quarter of all cases of status epilepticus. NCSE should be suspected in children with epilepsy who undergo an otherwise inexplicable change in behavior and in children with recent onset change in speech, memory, school performance. NCSE can be observed in acute neurological injuries, specific childhood epilepsy syndromes, individuals with learning difficulties and other neurological conditions. NCSE is not uncommon in pediatric patients with an altered state of consciousness. NCSE in children is thought to be under-recognized. It is a highly heterogeneous clinical condition that is understudied in the pediatric population. Prompt recognition and treatment may be necessary to improve neurological outcome. Based on clinical semiology alone, diagnosis is difficult and requires emergent EEG. Management is often difficult, with highly variable response to treatment. Here I describe the diagnosis and management of NCSE in children.     

Vitamin D replacement therapy and renal function. Calcitriol v dihydrotachysterol

We treated 24 patients who had chronic renal insufficiency and renal osteodystrophy with either calcitriol (1,25-dihydroxyvitamin D3) or dihydrotachysterol. Renal function was evaluated before and during treatment to determine if these vitamin D analogues caused an accelerated rate of renal function deterioration. An accelerated rate of increase in the serum creatinine level was found in three of 12 patients in each treatment group after therapy was started, but the mean rate of increase during treatment did not differ significantly from the rate during the pretreatment control period in either group. The occurrence of hypercalcemia or an excessive serum calcium x phosphorus-product did not correlate with the rate of change in renal function during treatment with either drug. We concluded that children receiving calcitriol are not at greater risk for an accelerated rate of renal function deterioration than are children treated with dihydrotachysterol. Furthermore, neither vitamin D analogue could be directly implicated as a cause of an accelerated rate of renal function deterioration when episodes of hypercalcemia were transient and occurred infrequently.     

Treatment of acute,episodic asthma in preschool children using intermittent high dose inhaled steroids at home.

In a double blind, controlled trial, the effect of high dose beclomethasone dipropionate (750 micrograms three times daily for five days) administered by metered dose inhaler and valved spacer, was compared with placebo, during 70 paired episodes of acute asthma in 24 preschool children. Treatment commenced at home at the first sign of an attack. Parents'' blind preference for active treatment was significant. Data from 17 pairs of treatment, however, were affected by interventions such as hospital admission or oral corticosteroid treatment. These events occurred similarly in active and control periods. An intrasubject comparison was made of diary scores from the 18 pairs of episodes in which no intervention occurred in either the active or placebo treatment. Both daytime and night symptoms over the first week of the attack were significantly reduced by active treatment. Intermittent high dose inhaled beclomethasone dipropionate is beneficial in modifying the severity of acute episodic asthma in preschool children able to use a spacer device.     

Rotavirus in infant-toddler day care centers: epidemiology relevant to disease control strategies

A 15-month prospective longitudinal study of diarrhea and rotavirus (RV) infection was conducted concurrently in infants and toddlers in day care centers (DCCs) and in a large pediatric clinic in Houston. The mean number of children in the DCCs was 223; the diarrhea rate during the first 12 months was 2.62 episodes per child-year. Rotavirus accounted for approximately 10% of the total episodes of diarrhea in the pediatric clinic and DCC populations, but 50% during the winter months. The occurrence of RV in the DCCs paralleled that seen in the pediatric clinic. The annual rate of RV infection in DCCs was 0.55 episodes per child-year, with diarrhea occurring in only 40% of the episodes (0.22 episodes per child-year). There were 45 diarrhea outbreaks in DCCs, for a mean of 3.8 per center per year; nine of these outbreaks were associated with RV. Polyacrylamide gel electrophoresis of RNA genome patterns of RV strains from eight of these outbreaks showed that in seven outbreaks a single strain was identified in children in that DCC, whereas multiple strains were identified simultaneously in the community. The age distributions of symptomatic and asymptomatic RV infections in DCC study children were not significantly different. In symptomatic RV-infected children in DCCs, 42% had RV identified in stool specimens within 2 days before diarrhea occurred. Thirty-eight DCC children had more than one episode of RV infection, but only five had two symptomatic RV infections. Diarrhea caused by RV is common in children in DCCs, often occurs in outbreaks due to the same strain, and parallels disease in the community; asymptomatic RV infection is also common in children in DCCs.     

Acute gastroenteritis in children attending day-care centres with special reference to rotavirus infections. II. Clinical manifestations

In a prospective study of a cohort of 214 children (aged 6 months-7 years) attending day-care centres, a total of 197 episodes of acute gastroenteritis (GE) occurred in 109 children (i.e. 51% of the participants) during a 12-month observation period. Rotavirus, pathogenic bacteria and Giardia lamblia caused GE in 24%, 6% and 2% of the cases, respectively. The aetiology of the remaining 68% was not discovered. Generally, the symptoms of GE were light and only two episodes led to hospitalization. Thirty-two rotavirus infections were asymptomatic. Two rotavirus GE reinfections occurred. They showed less severe symptoms than the primary infections. The older children (greater than 1.5 years) with rotavirus GE had lighter symptoms than the younger ones (less than or equal to 1.5 years). Compared with children with non-rotavirus GE, those with rotavirus GE showed the following clinical features: (1) Age between 6 months and 2 years. (2) Occurrence of rotavirus GE almost exclusively during the rotavirus season, i.e. January to April (winter). (3) High frequency of vomiting, the onset of which often preceded that of diarrhoea. However, these signs did not form a safe basis for the clinical diagnosis of rotavirus GE. One or more upper respiratory manifestations (URM) were observed in 39% of the children with rotavirus GE and in 36% of those with non-rotavirus GE. The occurrence of URM was age-related being highest in children less than 2 years. Consequently, the existence of a rotavirus syndrome is questioned. It is argued that URM in children with rotavirus GE may be due to a co-infection of the upper respiratory tract by a different micro-organism.     

Oesophageal replacement in the management of corrosive strictures: when is surgery indicated?

The accidental ingestion of corrosive agents is a major cause of oesophageal strictures in children. The mainstay of treatment is repeated dilatations. Despite this, a significant number of patients eventually require oesophageal bypass. We reviewed the records of all cases managed with this condition at the University of Cape Town teaching hospitals between 1976 and 1994. Dilatation therapy alone was successful in 14 out of 39 patients (41%). Morbidity of failed dilatation therapy included repeated hospital admissions over an average 11.5 months and 17 dilatations each. Oesophageal perforations occurred in 7 cases (18%). Early factors predictive of failure of conservative treatment were: delay in presentation of more than 1 month; severe pharyngo-oesophageal burns requiring a tracheostomy; oesophageal perforation; and a stricture longer than 5 cm on radiological assessment. The size of dilators accepted during early bougienage also correlated with eventual outcome. These criteria may be useful in predicting which patients will not respond to repeated dilatations. Early surgical intervention in such cases will prevent fruitless dilatations and related complications. Accepted: 12 September 1997     

Chloride deficiency as a presentation or complication of cystic fibrosis

Thirteen children with cystic fibrosis (CF), aged 1.5 months–15 years, had 18 episodes of hypochloraemia and metabolic alkalosis over the period 1983–1991. Five patients were not known to have CF prior to developing these electrolyte disturbances. There were two distinct clinical presentations: 5 patients had an acute isolated picture of heat exhaustion while 8 patients (all infants) had a more chronic course associated with failure to thrive. Many episodes were not associated with particularly high environmental temperatures, although most occurred during the summer and early autumn months. Serum electrolytes should be assessed regularly in children with CF, and this diagnosis should be considered in any infant presenting with unexplained hypochloraemic metabolic alkalosis.     

Sleep related upper airway obstruction in a cohort with Down's syndrome.

The prevalence of sleep related upper airway obstruction (UAO) was studied in a cohort of 34 children with Down''s syndrome from a geographically defined area. Thirty two (94%) of the children, ranging in age between 0.1 and 4.9 years (median 1.4), underwent full clinical assessment for UAO including parental questionnaires and overnight tape recordings of chest wall movements and arterial oxygen saturation (SaO2). Compared with controls, children with Down''s syndrome had (a) an increased incidence of stridor and chest wall recession during sleep, (b) an increased frequency of a pattern on inspiration indicating increased upper airway resistance, (c) a reduced baseline oxygen saturation (having excluded recordings on four children with potential for right to left intracardiac shunting), and (d) an increased number of episodes with SaO2 less than or equal to 90% despite continued chest wall movements. At their initial assessment seven children (22%) had evidence of UAO. The 18 youngest children (less than or equal to 1.7 years) underwent repeated recordings and clinical assessment until they had all reached 2 years of age. A further three were found to have developed UAO. Sleep related UAO is a common problem in children with Down''s syndrome, occurring in 10 of 32 (31%) of this population based sample.     

Cerebral blood flow during vasovagal syncope induced by active standing or head up tilt.

BACKGROUND: Vasovagal syncope is usually associated with a sudden drop of blood pressure and/or heart rate. However, occasionally the symptoms of syncope induced by orthostatic stress testing are not associated with obvious haemodynamic changes. The mechanisms of syncope in these patients are not clear. AIM: To evaluate changes in cerebral blood flow velocities during orthostatic stress testing in children and adolescents with vasovagal syncope. METHODS: Electrocardiogram, instantaneous arterial blood pressure, and right middle cerebral artery blood flow velocity were recorded at rest, during active standing, and 80 degrees head up tilt. 32 children and adolescents aged between 7 and 18 years with a history of repeated vasovagal syncope and 23 healthy control subjects were studied. RESULTS: Presyncope occurred in 10 patients during standing, and 13 patients during head up tilt. None of the controls had symptoms during the test. The transcranial Doppler study showed that the symptoms were associated with significant decreases of diastolic cerebral blood flow velocity and an increase of pulsatility. There was no significant change of the systolic cerebral blood flow velocity. The changes of cerebral blood flow velocities occurred in all episodes of presyncope, including those not associated with severe drop of blood pressure or heart rate. CONCLUSIONS: Diastolic cerebral blood flow velocity decreased significantly during episodes of presyncope induced by orthostatic stress. Impairment of autoregulation of cerebral blood flow might play an important role in the pathophysiology of syncope.     

Efficacy and safety of intravenous amiodarone for short-term treatment of paroxysmal supraventricular tachycardia in children

The use of intravenous amiodarone was assessed during 23 episodes of paroxysmal supraventricular tachycardia in 15 children aged 9 days to 11 years. Five of the fifteen patients had congenital structural heart disease, and three had Wolf-Parkinson-White syndrome. Tachyarrhythmias were returned to sinus rhythm during 20 of the 23 episodes (87%). No major adverse effects occurred. Recurrence of tachycardia was not observed during short-term follow-up. In conclusion, intravenous amiodarone is an effective, safe antiarrhythmic drug for short-term treatment of supraventricular tachycardia in children.     

Neural dysfunction during hypoglycaemia.

There is controversy over the definition of hypoglycaemia in neonates and children and over its significance when ''asymptomatic''. We measured sensory evoked potentials in relation to blood glucose concentration in 17 children: 13 were fasted or given insulin to investigate endocrine or metabolic abnormalities and four had spontaneous episodes of hypoglycaemia. Abnormal evoked potentials were recorded in 10 of the 11 children whose blood glucose concentration fell below 2.6 mmol/l; five of these 10 children were ''asymptomatic''. No change in evoked potentials was recorded in the six children whose blood glucose concentration remained above 2.6 mmol/l. Our findings suggest that the blood glucose concentration should be maintained above 2.6 mmol/l to ensure normal neural function in children irrespective of the presence or absence of abnormal clinical signs.     

VIRAL INFECTIONS OF THE RESPIRATORY TRACT IN HOSPITALIZED CHILDREN A Study from Oslo during a 90 Months'Period

ABSTRACT. A diagnosis of 979 respiratory viral infections was made in hospitalized children. Respiratory syncytial virus greatly outnumbered the other viruses: it caused 58% of the total virus infections and occurred in winter epidemics. Influenza A and B virus occurred during late winter and spring, rhinovirus had a seasonal distribution towards spring and autumn, whereas adenovirus types 1, 2 and 5 had no distinct seasonal distribution. Whereas respiratory syncytial virus were mainly associated with bronchiolitis and adenovirus type 7 with pneumonia, rhinovirus infections were most often found in children with episodes of acute bronchial asthma. The influenza A and B and adenovirus types 1, 2 and 5 infections often occurred with extrarespiratory symptoms, especially febrile convulsions.     

Clinical profile of children with nephrotic syndrome not on glucorticoid therapy, but presenting with infection

OBJECTIVE: This is a prospective, hospital based study over a period of one year, to examine the clinical profile of nephrotic children not on glucocorticoid therapy, but presenting with infection. METHODS: Sixty-eight children with 76 episodes of nephrotic syndrome were enrolled during the study year. They were examined for evidence of infection using clinical and investigative criteria. The children were monitored daily for proteinuria and improvement of infection. After the infection was controlled, prednisolone therapy was started in those who were not already in remission. RESULTS: Of the 76 episodes, eight were excluded from analysis as they had developed infection while they were on glucocorticoid therapy. Of the remaining 68 nephrotic episodes in 60 children, there was evidence of infection in 57 episodes (83.8%). Upper respiratory infection was the most common (28.0%) followed by urinary tract infection (22.8%), peritonitis (15.8%), pneumonia (14.0%), acute invasive diarrhoea (10.5%) and empyema (5.3%). Children with infection had significantly lower serum albumin and higher serum cholesterol compared to non-infected children. Of the 57 episodes with infection, remission occurred with control of infection alone in nine episodes (15.8%) and glucocorticoid therapy was not required. CONCLUSION: Infection is widely prevalent among Indian children presenting with episodes of nephrotic syndrome, even when they are not on glucocorticoid therapy. Some children may go into remission by control of infection alone. Among the infections, upper respiratory and urinary tract infections are the most common.     

Treatment of status epilepticus in children with epilepsy]

The treatment of status epilepticus (SE) in children with epilepsy depends on the epilepsy syndrome, in order to avoid worsening drugs such as IV barbiturates in severe myoclonic epilepsy in infancy (SMEI) (Dravet's syndrome) or IV benzodiazepam in tonic SE of Lennox-Gastaut syndrome. Intensive care procedures should not be systematical in convulsive SE (CSE) and are not indicated in non-convulsive SE (NCSE). Generalized CSE mostly involve SMEI before 3 years of age, symptomatic generalized epilepsy and partial lesional epilepsy. Treatment is an emergency and relies on IV benzodiazepines and, if necessary, IV phenytoine using plasmatic concentrations for an optimal management. The partial CSE of partial lesional epilepsy can result in focal deficit and need the same treatment as generalized CSE. NCSE consist in absence and/or myoclonic SE and are often unrecognised during a long time until EEG is performed. They mostly involve myoclonic epilepsies and can be controlled by IV benzodiazepines. The frequency of partial NCSE is underestimated, particularly in infants. Diagnosis relies on video EEG and treatment is the same as that used in partial CSE.     

Duration and morbidity of chronic immune thrombocytopenic purpura in children: five-year follow-up of a Nordic cohort

Aim: To describe the clinical course, morbidity and platelet recovery in an unselected Nordic cohort of children with chronic Immune Thrombocytopenic Purpura (ITP). Methods: Prospective 5‐year follow‐up of 96 children with ITP lasting more than 6 months, with reporting of hospital admissions, severity of bleeding episodes and stabilization of platelet counts above 20, 50 and 150 × 10⁹/L. Results: The estimated 5‐year recovery rate was 52%; exclusion of 12 splenectomized children did not change the estimate. Events eliciting admission to hospital occurred in 39 (41%). Major haemorrhages occurred in eight children (8%), including a nonfatal intracranial haemorrhage in one child (1%). The overall admission rate was 0.4/year of thrombocytopenia, decreasing during follow‐up as thrombocytopenia converted to milder degrees. Early recovery within 2 years of diagnosis occurred in 35%, was associated with low morbidity and was more likely in young children with abrupt onset of symptoms. Conclusion: In a Nordic cohort of children with chronic ITP, one half had recovered 5 years after diagnosis, more than half never required hospitalization and <10% experienced serious bleeding episodes, always with a platelet count <20 × 10⁹/L. Aggressive management can be restricted to the minority of children with continuing severe thrombocytopenia and frequent, clinically significant bleeding events.     

Evaluation of Treatment in Typical Absence Seizures: The Roles of Long-term EEG Monitoring and Ethosuximide

ABSTRACT. Eleven children with typical absence seizures were studied clinically and by repeated 24-h EEGs with portable cassette tape recorder before and during anticonvulsant treatment. The history, the observation of seizures and the amount of spike-and-wave activity (episodes >3 sec) were studied in relation to the plasma levels. The EEG revealed spike-and-wave episodes, considered as seizures, in a higher frequency than the number of absences reported by the parents. Twenty-four hour EEG recording was however not superior to long term observation by relatives in establishing freedom from absences. The drug primary used was ethosuximide. Eight of eleven children responded completely on this drug, i.e. no absences observed and EEG was normalized. Of two children in whom the therapy was changed to sodium valproate, one responded completely and one partly. The relation between the ethosuximide dosage given and the plasma concentration level was good. Two cases responded at low plasma levels (<200 μmol/1). Tonic-clonic seizures occurred in 3/11 children.     

Hypoglycemia in pediatric renal allograft recipients

Symptomatic hypoglycemia developed 5 to 45 months after transplantation in nine children who had renal transplants before 6 years of age. During hypoglycemia, serum glucose levels ranged from 14 to 39 mg/dl (0.8 to 2.1 mmol/L). Hypoglycemic episodes occurred between 1.7 and 7.5 years of age. Six patients had generalized seizures; the remaining three had diaphoresis with stupor or lethargy. None of the children had serious infections, diabetes, congenital defects of glucose metabolism, or a history of treatment with insulin or oral hypoglycemic agents. Six patients had hypoglycemic symptoms after a prolonged fast, and at least four had ketosis. Eight of the nine patients were receiving propranolol when hypoglycemia occurred. No differences in the daily prednisone dose, the number of transplant rejection episodes, or the frequency of treatment with medications other than propranolol were noted between hypoglycemic patients and 56 normoglycemic age-matched renal transplant recipients. All hypoglycemic patients were subsequently treated with frequent feedings and discontinuation of propranolol. No further hypoglycemic episodes have occurred in eight of nine patients. Symptomatic hypoglycemia should be recognized as a potentially devastating complication of pediatric renal transplantation.     

Cerebral blood flow during vasovagal syncope induced by active standing or head up tilt

BACKGROUND—Vasovagal syncope is usually associated with a sudden drop of blood pressure and/or heart rate. However, occasionally the symptoms of syncope induced by orthostatic stress testing are not associated with obvious haemodynamic changes. The mechanisms of syncope in these patients are not clear.
AIM—To evaluate changes in cerebral blood flow velocities during orthostatic stress testing in children and adolescents with vasovagal syncope.
METHODS—Electrocardiogram, instantaneous arterial blood pressure, and right middle cerebral artery blood flow velocity were recorded at rest, during active standing, and 80° head up tilt. 32 children and adolescents aged between 7 and 18 years with a history of repeated vasovagal syncope and 23 healthy control subjects were studied.
RESULTS—Presyncope occurred in 10 patients during standing, and 13 patients during head up tilt. None of the controls had symptoms during the test. The transcranial Doppler study showed that the symptoms were associated with significant decreases of diastolic cerebral blood flow velocity and an increase of pulsatility. There was no significant change of the systolic cerebral blood flow velocity. The changes of cerebral blood flow velocities occurred in all episodes of presyncope, including those not associated with severe drop of blood pressure or heart rate.
CONCLUSIONS—Diastolic cerebral blood flow velocity decreased significantly during episodes of presyncope induced by orthostatic stress. Impairment of autoregulation of cerebral blood flow might play an important role in the pathophysiology of syncope.

     

重症病毒性脑炎患儿非惊厥性癫痫持续状态9例临床分析

目的 通过分析重症病毒性脑炎患儿非惊厥性癫痫持续状态（NCSE）的临床特征、诊断和治疗,提高对NCSE的认识。方法 回顾性分析2012年6月至2014年9月广州市妇女儿童医疗中心神经内科诊断为重症病毒性脑炎NCSE的病例。结果 ①9例重症病毒性脑炎NCSE连续病例进入本文分析,男5例。脑炎起病年龄（7.2±3.9）岁;Glasgow评分（8.6±1.9）分;脑炎起始与NCSE起始间隔4~70（19.4±20.9）d;昏迷中癫痫持续状态（SEC）4例,复杂部分发作持续状态4例,不典型失神发作持续状态1例。②病因包括病毒性脑炎8例,病毒性脑炎抗癫痫药物调整1例;9例前驱均有惊厥,其中3例伴惊厥性癫痫持续状态。③9例临床发现主要有口面部或肢体微小抽动、认知障碍、孤独症样行为等。④VEEG发作期主要以背景活动变慢,伴δ、θ、β、棘节律,或棘慢波持续发放为特征。⑤4例SEC患儿予麻醉药联合多种抗癫痫药物治疗,发作平均持续42.8 d;5例非SEC患儿予多种抗癫痫药物治疗,其中3例发作平均持续9 d,1例抗癫痫药物调整所致NSEC发作持续4个月。⑥1例自动出院失访,1例死亡,余7例随访3个月至2.5年;6例VEEG均有不同程度的发作期或发作间期脑电异常,1例正常;1例神经学功能正常,5例有不同程度认知下降伴继发性癫痫,1例持续植物状态伴继发性癫痫。结论 口面部或肢体运动现象、认知及行为改变为重症病毒性脑炎患儿NCSE临床特征;发作期脑电波形变异大,棘节律可能是重症病毒性脑炎患儿NCSE的独特形式;抗癫痫治疗及起效时间与NCSE类型相关。