Kawasaki syndrome hospitalizations among children in Hawaii and Connecticut

OBJECTIVES: To estimate the incidence and describe recent trends of Kawasaki syndrome (KS) in 2 different areas of the United States. METHODS: Retrospective analysis of Hawaii and Connecticut State KS hospital discharge records for children younger than 5 years. RESULTS: In Hawaii, 175 KS hospitalizations for children younger than 5 years were reported during 1994 through 1997; the annual hospitalization rate per 100,000 children was 47.7. The rate for Hawaiian children younger than 1 year (83.2) was greater than that for 1- to 4-year-old children (39.0), and most hospitalizations occurred prior to age 2 years (median age, 17 months). In Connecticut, 171 KS hospitalizations for children younger than 5 years were reported during 1993 through 1996; the annual hospitalization rate per 100,000 children was 18.8, and the median age at hospitalization was 28 months. For both states, most hospitalizations were for boys. Although no clear seasonality was apparent, monthly peaks occurred in some of the years from December through March. CONCLUSIONS: Kawasaki syndrome seems to remain an endemic disease in the United States. A high KS annual hospitalization rate was seen in Hawaii, especially in children younger than 1 year, whereas in Connecticut, the KS rate was more consistent with those previously reported in the continental United States. Arch Pediatr Adolesc Med. 2000;154:804-808     

Kawasaki syndrome hospitalizations in Ireland, 1996 through 2000

OBJECTIVE: To describe the epidemiologic characteristics and estimate the incidence of Kawasaki syndrome (KS) among children in Ireland. METHODS: Hospital discharge records with a KS diagnosis among patients <18 years of age were examined using Ireland's Hospital In-Patient Enquiry database for 1996 through 2000. RESULTS: During the study period 265 hospitalizations associated with KS among children <18 years of age were recorded in Ireland. Of those, 194 (73%) occurred among children <5 years of age. The median age of patients at admission was 2 years. The average annual KS hospitalization rate for children <5 years of age was 15.2 per 100 000 children, and among that group the hospitalization rate was higher for infants <1 year of age than for children 1 to 4 years of age (19.7 and 16.0 per 100 000 children, respectively). Most KS hospitalizations occurred among children <5 years of age and among boys. The highest monthly number of hospitalizations occurred during the months of November through January. No deaths associated with KS were reported among hospitalized children. CONCLUSION: Hospital discharge data provide useful information on the epidemiology of KS in Ireland. The hospitalization rate for KS in Ireland is similar to rates in the United States and may be higher than those in other European countries, although the European studies differ in methodologies and time periods.     

Kawasaki syndrome in Denmark

OBJECTIVE: To describe the epidemiologic characteristics of Kawasaki syndrome (KS) and to estimate national KS incidence rates among children in Denmark. METHODS: A retrospective population-based study using hospital discharge records with a KS diagnosis for children younger than 15 years selected from the Danish National Hospital Register for 1981-2004. Incidence rates were calculated using the number of KS patients and corresponding census data. RESULTS: During 1981-2004, 360 children younger than 15 years were hospitalized with KS in Denmark, with 73% younger than 5 years. In this age group, the average annual incidence of KS gradually increased from 1981 to 1999 and thereafter stabilized at 4.5 to 5.0 per 100,000 person-years. The incidence was greater for boys than for girls (RR = 1.6, 95% CI = 1.2-2.0) and was highest among infants younger than 1 year (4.5), declining with increasing age (P = 0.03). However, the age-specific decline in incidence was only observed for boys, whereas the incidence for girls remained unchanged by age. The median length of hospital stay was 12 days, and the incidence peaked in the winter months. CONCLUSIONS: Major epidemiologic characteristics identified among Danish childhood KS are consistent with those described in previous studies, such as highest incidence among young children and winter-seasonality. The KS incidence rate among children younger than 5 years in Denmark increased steadily during the early study period (coinciding with global recognition of KS) and seems to have stabilized from 1998-1999 onwards. Although the incidence among Danish children was lower than that reported for several other European countries, differences in methodology challenge definite comparisons.     

Kawasaki syndrome among American Indian and Alaska Native children, 1980 through 1995.

BACKGROUND: Kawasaki syndrome (KS) is a leading cause of acquired heart disease among US children, but the epidemiologic features of KS among American Indian and Alaska Native (AI/AN) children have not been described. METHODS: We examined Indian Health Service computerized records of hospital discharges for AI/AN children <18 years of age with KS during 1980 through 1995. RESULTS: During 1980 through 1995, 85 AI/AN children were reported with a hospitalization for KS; 10 of the children had an additional KS hospitalization record within 5 months. The average annual KS hospitalization rate for children <5 years of age, based on first KS hospitalization only, was 4.3 cases per 100000 children; the rate for children age <1 year (n = 21) was 8.6 per 100000 and for children ages 1 to 4 years was 3.6 per 100000. The annual rates for children < 5 years of age ranged from 0 to 8.5 per 100000 children. KS hospitalizations for children peaked in January and February; 50.6% of the children were hospitalized during January through April. The overall median length of hospital stay was 4 days (range, 1 to 29 days); the median duration decreased from 8 days from 1980 through 1982 to 4 days from 1993 through 1995. CONCLUSIONS: The overall annual hospitalization rate of KS among AI/AN children <5 years of age was slightly lower than rates for several majority white populations in the United States. (4.6 to 15.2 cases per 100000) and much lower than rates for blacks and Asians/Pacific Islanders.     

Routine laboratory testing data for surveillance of rotavirus hospitalizations to evaluate the impact of vaccination

OBJECTIVE: The recent implementation of a rotavirus vaccination program in the United States makes it imperative to assess the impact of immunization on the incidence of severe rotavirus disease leading to hospitalization. Active surveillance for laboratory-confirmed rotavirus hospitalizations is the ideal approach for surveillance, but requires substantial resources to implement. We examined laboratory and hospital discharge data for 2 tertiary care pediatric hospitals to assess the utility of routine laboratory testing data for surveillance of rotavirus gastroenteritis and to estimate rotavirus disease burden. DESIGN: We obtained all discharge records of hospitalizations for acute gastroenteritis among children <5 years of age at Children's Mercy Hospital (CMH), Kansas City, from July 2000 to June 2005 and at Children's Hospital of Philadelphia (CHOP) from July 2004 to June 2006. We linked these discharge records to laboratory results of rotavirus testing to evaluate epidemiologic differences in children who were tested and not tested for rotavirus and to estimate overall rotavirus burden by extrapolating clinical testing results to the untested group. RESULTS: At CMH, of the 3702 children with acute gastroenteritis, 69% (n = 2552) were discharged during the winter (January through May) months, when rotavirus is most common. Similarly, at CHOP, 62% (n = 779) of the 1261 gastroenteritis discharges occurred during the winter months. During these months, 47% (n = 1197 of 2552) of the discharges at CMH and 56% (n = 438 of 779) of the discharges at CHOP were tested for rotavirus and of those tested, 71% (n = 853 of 1197) and 55% (n = 242 of 438) were positive, respectively. At both hospitals, children with and without rotavirus testing had similar gender and race/ethnicity, but the rate of testing differed by age at CHOP and by month of admission at CMH. After adjusting for these differences, we estimate that 56%-70% of winter and 34%-48% of year-round gastroenteritis in children <5 years can be attributable to rotavirus. Overall, 3%-5% of all hospitalizations in children <5 years of age were caused by rotavirus. CONCLUSIONS: Sentinel hospitals where a large proportion of children hospitalized for gastroenteritis are routinely tested for rotavirus could provide a useful and cost-efficient platform to complement ongoing active surveillance efforts to evaluate the impact of rotavirus vaccination. The data reaffirm the substantial burden of rotavirus hospitalizations in US children and the potential health benefits of vaccination.     

Immunization status of hospitalized preschool-age children. The need for hospital-based immunization programs

The records of 102 hospitalized preschool-age children were reviewed to determine their immunization status. Nineteen children (19%) had a documented delay in immunizations. None of these children were immunized prior to hospital discharge, although no contraindications to immunization had been identified. Two to five months after hospital discharge, ten patients (53%) were still deficient. Compliance with recommended immunization schedules is not adequately addressed during hospital admissions.     

Kawasaki syndrome in Hawaii

OBJECTIVE: To describe the incidence and epidemiology of Kawasaki syndrome (KS) in Hawaii. METHODS: Retrospective analysis of the State Inpatient Database for Hawaii residents hospitalized with KS during 1996 through 2001. RESULTS: During 1996 through 2001, 267 persons younger than 18 years of age living in Hawaii were hospitalized with KS; 226 (84.6%) were younger than 5 years of age. The average annual incidence for KS was 45.2 per 100,000 children younger than 5 years of age. The incidence was higher for children younger than 1 year of age than for those 1-4 years of age (74.3 and 37.5 per 100,000). The KS incidence for Asian and Pacific Islander children and for White children was 70.9 and 35.3 per 100,000, respectively. Incidence was highest among Japanese American children living in Hawaii (197.7 per 100,000). Honolulu County had the most KS patients (85.0%) and the highest incidence (53.1 per 100,000) among Hawaii counties. For children younger than 5 years of age hospitalized with KS, the median length of stay was 2 days, and the median hospital charge was $9379. CONCLUSION: During 1996 through 2001, the annual incidence rate for KS among children younger than 5 years of age in Hawaii was the highest in the United States. The incidence among Japanese American children in Hawaii was higher than that among other racial groups in the state and when compared with children living in Japan.     

Disease burden and risk factors for hospitalizations associated with rotavirus infection among children in New York State, 1989 through 2000

OBJECTIVES: To examine trends in hospitalizations for pediatric diarrhea, ascertain the disease burden and risk factors for hospitalizations associated with rotavirus and assess the accuracy of coding for rotavirus hospitalizations in New York State. METHODS: For 1989 through 2000, data were obtained for all diarrhea-associated hospitalizations in New York State among children 1 month through 4 years of age. Characteristics of patients hospitalized with rotavirus were compared with those for hospitalizations with diarrhea from other causes. Medical charts coded as rotavirus diarrhea were reviewed for patients who were discharged during 1997 to determine whether these diagnoses were supported with laboratory results. RESULTS: Diarrhea was reported as a discharge diagnosis in approximately 13% of all hospitalizations for an annual incidence of 83 per 10 000 children. Viruses were the most common etiologic agents reported. Since 1993, when a rotavirus-specific code was introduced, rotavirus infection was coded for 8.7% of all diarrhea-associated hospitalizations. A total of 136 patients with diarrhea died during their hospitalization (hospital fatality rate, 1.6 per 1000), and the 12 deaths among patients with rotavirus had a distinct winter pattern. During 1997 only 46% of the hospitals reporting diarrhea in children used the specific code for rotavirus, and 12% of hospitals reported rotavirus in >30% of all diarrhea-associated hospitalizations. Infants <4 months of age were more likely than older children to be nosocomially infected with rotavirus and had a higher proportion of congenital malformations. CONCLUSION: In New York State diarrhea is a common hospital discharge diagnosis and contributes approximately 13% of all hospitalizations among children <5 years of age. When hospitals with maximum recording were used as a reference point, >30% of diarrhea hospitalizations were recorded as likely the result of rotavirus.     

Variation in hospitalizations and hospital length of stay in children with vaso-occlusive crises in sickle cell disease

OBJECTIVE: As a measure of morbidity of vaso-occlusive crises (VOC) in sickle cell disease, we used a national hospital discharge database, the Kids' Inpatient Database (KID), to determine the number of hospitalizations and hospital length of stay (LOS) by age. PROCEDURE: Nationally weighted hospital discharges for VOC in children with sickle cell disease were analyzed using data from 2,500 hospitals in the Healthcare Cost and Utilization Project (HCUP) KID. Number of discharges and hospital LOS by age group were analyzed. Multiple linear regression was performed to analyze the effect of age on hospital LOS. RESULTS: There were 20,271 hospital discharges for children with sickle cell disease and VOC. Mean age (SD) at time of admission was 10.6 (5.38) years with 49% being males. The overall average LOS was 4.4 days. There were differences in the number of hospital discharges by age group (P < 0.001). In addition, there was a difference in LOS by age group (P < 0.0001). After controlling for potential confounders, older age was associated with a longer LOS (P < 0.0001). CONCLUSIONS: Older children with sickle cell disease and VOC have increased hospitalizations and longer LOS. This age effect should be considered when measuring the effect of an intervention on hospital utilization in these children.     

Epidemiology of cat-scratch disease hospitalizations among children in the United States

BACKGROUND: Cat-scratch disease (CSD), caused by infection with Bartonella henselae, affects both children and adults but is principally a pediatric disease. Typical CSD is generally benign and self-limited and is characterized by regional lymphadenopathy with fever. Infections can, however, be accompanied by focal or diffuse inflammatory responses (atypical CSD) involving neurologic, organ (liver/spleen), lymphatic or skeletal systems. METHODS: Pediatric hospitalizations with CSD listed as a diagnosis were examined using the Kids' Inpatient Database for the year 2000. National estimates of CSD-associated hospitalizations, hospitalization rates and various hospitalization statistics were examined for patients younger than 18 years of age. RESULTS: During 2000, an estimated 437 (SE 43) pediatric hospitalizations associated with CSD occurred among children younger than 18 years of age in the United States. The national CSD-associated hospitalization rate was 0.60/100,000 children younger than 18 years of age (95% confidence interval, 0.49-0.72) and 0.86/100,000 children younger than 5 years of age (95% CI 0.64-1.07). Accompanying diagnoses included neurologic complications (12%), organ (liver/spleen) involvement (7%) and "other" (5%). Atypical CSD accounted for approximately 24% of the CSD-associated hospitalizations. The median charge for a CSD-associated hospitalization was 6140 dollars with total annual hospital charges of approximately 3.5 million dollars among children in the United States. CONCLUSIONS: The CSD-associated hospitalization rate among children during 2000 appeared similar to those estimated for the 1980s in the United States, despite significant increases in cat ownership in the intervening time. Early serologic and molecular testing for CSD in children is suggested to minimize unnecessary interventions and promote optimally effective care when supportive measures are required.     

How commonly are children hospitalized for dehydration eligible for care in alternative settings?

BACKGROUND: Avoiding unnecessary hospitalization has long been a goal of child health care providers. Managed care practice environments increasingly pressure the practicing pediatrician to avoid hospitalization. OBJECTIVES: To estimate the proportion of childhood dehydration hospitalizations eligible for care in alternative settings (eg, short-stay treatment and triage units, home nursing) and to assess the type and duration of services that might be required for alternative setting care of children with these illness episodes. DESIGN: All dehydration hospitalizations for the 198 593 children (aged > 1 month and < 19 years) dwelling in Rochester, NY (Monroe County), between 1991 and 1995 were identified in county-wide hospital discharge computer files. Medical records were reviewed for a random sample of 380 of the hospitalizations. Children with major underlying conditions were excluded from analysis because of higher risk for deterioration, and greater complexity of medical care might render alternative settings inappropriate. Measures included a 4-item score estimating level of dehydration, serum bicarbonate level at presentation, and time to rehydration. Rehydration was defined as a drop in urine-specific gravity to 1.010 or less or reduction of fluid administration to the maintenance rate. RESULTS: Altogether, 1121 dehydration hospitalizations occurred during the study period. Based on medical record review for a random sample of 380 of these 1121, major underlying problems were present in 27.4% (104) of hospitalizations sampled. Simple, acute gastroenteritis accounted for 75.4% (208) of 276 hospitalizations remaining in the sample. Levels of dehydration for these children were estimated as at least 5% for 51.0% (106) and at least 10% for 16.3% (34) of hospital admissions, and serum bicarbonate levels were 12 mmol/L or less for 26.0% (54). Time from hospital admission to rehydration was no greater than 12 hours for 79.3% (165) and no greater than 24 hours for 94.7% (197). However, hospital stay was generally substantially longer. The time hospitalized following rehydration represented 85.8% of the average inpatient stay. Hospital discharge was heavily concentrated in daytime hours, although the children achieved rehydration at all hours of the day. No deterioration occurred during hospitalizations studied. CONCLUSION: Nearly all children hospitalized for simple, acute gastroenteritis in Rochester might be eligible for care in alternative settings designed to provide hospital-level care for short periods.     

Clinical presentation and outcome of epidemic Kaposi sarcoma in Ugandan children

Background

Kaposi sarcoma (KS) is one of the most common pediatric cancers in sub‐Saharan Africa. Few data are available about the clinical presentation or response to treatment of children with epidemic (HIV‐associated) KS.

Methods

Medical records of all children with KS and HIV infection referred to the Uganda Cancer Institute in Kampala, Uganda from October 2004 to June 2007 were reviewed. Charts were abstracted for age, sex, location of KS lesions at presentation, biopsy results, CD4 T‐cell count and percentage, and KS treatment and outcome.

Results

Seventy‐three children with epidemic KS were identified, 37 males and 36 females. The median age was 10.1 years (range 2–18). KS presented with lymph node (LN) involvement in 60% of cases. The median absolute and percentage CD4 T‐cells at presentation were 210 cells/µl and 7.4%, respectively. Those children with lymphadenopathic KS were younger (mean difference 3.7 years; P = 0.01) and had higher CD4 T‐cell counts (mean difference 242 cells/µl; P = 0.03) than those without LN involvement. Of 32 patients for whom outcome data were available, a complete response to chemotherapy and/or antiretroviral therapy was documented in 20 (62.5%) patients.

Conclusions

In comparison to cutaneous involvement, LN involvement of epidemic KS occurs at younger ages and at higher CD4 levels. This clinical presentation may reflect recent infection with human herpesvirus 8 followed by a rapid progression to malignancy. Favorable response to treatment was observed in the majority of cases, but prospective studies are needed to determine optimal management. Pediatr Blood Cancer 2010;54:670–674. © 2010 Wiley‐Liss, Inc.     

Acute gastro-enteritis in children in France: estimates of disease burden through national hospital discharge data]

OBJECTIVES: To estimate the burden of hospitalized infectious gastroenteritis of children younger than 5 years of age and associated costs. METHODS: We analyzed 1997 hospital discharges with a primary diagnosis of gastroenteritis or a secondary diagnosis of gastroenteritis with gastroenteritis symptoms or complications as primary diagnosis and compared the deaths with those of the national mortality data. RESULTS: Gastroenteritis was associated with 51,125 hospitalizations which accounted for 11.4% of hospitalization discharges for this age group and an annual rate of 1,385 per 100,000 children <5-year-old. Most gastroenteritis (56%) were registered as "probably infectious", 36% as "viral" (43% of which were coded "rotavirus") and 8% as "bacterial" (of which 60% were coded "Salmonella"). The seasonal peak was winter for rotaviral, viral and "probably infectious" gastroenteritis, summer for those related to salmonellosis. Incidence increased inversely with age: 3606/100,000 infants <1-year-old, 257/100,000 4-year-old children. Complications (especially dehydration) were observed in 21% of viral gastroenteritis and 17% of bacterial gastroenteritis. At least, 14 deaths were found in both hospital discharge and mortality data. The mean duration of stay (3.2 days) was significantly higher in infants <1-year-old, viral etiology, association with complications or bronchiolitis. The costs of hospitalization could be estimated to 62 million Euros. DISCUSSION: Our results are similar to those obtained in other developed countries. Despite variations in encoding the discharge reports, data has proven to be effective to describe national trends for this health event. Our study indicates that the public health burden and economic impact of prevention and control measures can be monitored through hospital discharge surveillance.     

Acute mastoiditis in children: a seventeen-year experience in Dallas, Texas

BACKGROUND: In the preantibiotic era acute mastoiditis was the most common complication of acute otitis media, often resulting in substantial morbidity and mortality. Since 1989 several investigators have documented an increased frequency of acute mastoiditis in children. METHODS: The medical records of all children with a discharge diagnosis of acute mastoiditis, managed at Children's Medical Center, Dallas, TX, from 1983 through 1999 were reviewed. RESULTS: There were 57 cases of acute mastoiditis during the 17-year period of 1983 through 1999 compared with 57 cases in a 25-year period of 1955 through 1979 reported previously at the same institution. The number of cases of acute mastoiditis per 10,000 hospital admissions increased significantly (regression analysis P = 0.003) during the more recent 17 years. From 1993 through 1999 there were 4.5 cases or more per 10,000 admissions each year, whereas from 1983 through 1992, the incidence never exceeded 4.3 cases per 10,000 admissions (P = 0.018). The median age of the patients was 48 months. Twenty-two patients (38.5%) were younger than 24 months; 17 of these were 12 months of age or younger. Twenty-two (38.5%) patients had no history of previous episodes of acute otitis media. Streptococcus pneumoniae was the pathogen most often isolated from the cultures. Complications of mastoiditis occurred in 20 children (35%). CONCLUSIONS: We conclude that acute mastoiditis continues to be a problem in the post antibiotic era. It occurs mainly in young children and can be the first evidence of ear disease.     

Hospitalizations during a measles epidemic in the Netherlands, 1999 to 2000

BACKGROUND: A measles epidemic occurred in the Netherlands in 1999 to 2000. We collected data on hospitalized and deceased cases to inform the public about the risks associated with measles infection. METHODS: From the National Medical Registration we extracted hospital discharge diagnoses for measles-related hospitalizations (International Classification of Diseases, 9th revision, Clinical Modification Code 055) during the measles epidemic (April 1999 to May 2000). We also reviewed hospital records to assess reasons for hospitalization and severity of disease as a result of measles infection during this epidemic. In addition we estimated the total number of hospitalized cases by a capture-recapture approach using two surveillance sources. RESULTS: Measles without mention of complication (39%) and postmeasles pneumonia (33%) were most often registered as main discharge diagnoses for the 130 cases in the National Medical Registration. Forty-seven (96%) of the 49 patients whose hospital charts were reviewed were not vaccinated. Median admission period was 5 (range, 1 to 26) days, 19 (38%) required mechanical ventilation and 14 (29%) had sequelae at the time of discharge but none was permanent. Three patients died as a result of complications. No association was observed between preexisting illness and either reason for admission (P = 0.5) or residual symptoms at discharge (P = 0.5). The estimated total number of hospitalized measles patients was 157 (95% confidence interval, 145 to 179), leading to an estimated 825 admission days during an epidemic in which 3,292 cases were reported. CONCLUSIONS: Measles infection can still run a severe course even in a prosperous country.     

Familial occurrence of Kawasaki syndrome in North America

OBJECTIVE: To describe families with multiple members affected with Kawasaki syndrome (KS) to increase awareness of the familial occurrence of KS among practitioners who care for these patients. DESIGN: Retrospective review of medical records at 2 medical centers and data collection from remote KS families who contacted the KS Research Program at the University of California, San Diego. RESULTS: Eighteen families with multiple affected members were identified. There were 9 families with 2 affected siblings. In San Diego, 3 (0.7%) of 424 KS families had sibling cases. Nine families were identified with KS in 2 generations or in multiple affected members, yielding a total of 24 KS-affected children. No clear pattern of inheritance could be deduced from these pedigrees, and it is likely that multiple polymorphic alleles influence KS susceptibility. CONCLUSION: Physicians should counsel affected families and make them aware of the potential increased risk of KS among family members.     

Potentially avoidable pediatric hospitalizations as defined by the Agency for Healthcare Research and Quality: what do they tell us about disparities in child health?

This study was conducted to determine if the Agency for Healthcare Research and Quality's newly established pediatric quality monitors, which measure potentially avoidable hospitalizations, are useful in detecting disparities in health care delivery. Data for all hospital discharges in Tennessee in 2002 were evaluated for the 5 pediatric discharge monitors identified by the Agency for Healthcare Research and Quality. These diagnoses were asthma, short-term complications of diabetes, gastroenteritis, urinary tract infection, and perforated appendix. Black children were more likely to be discharged with the diagnoses of asthma and short-term diabetes complications. Publicly insured children were more likely than commercially insured children to be discharged with each of the 5 potentially avoidable hospitalizations. The results show that black children and children insured by public programs have significantly different discharge rates for pediatric potentially avoidable hospitalizations than do white children or commercially insured children. This could be the result of less access to high-quality ambulatory care.     

A survey of severe eye injuries in children

We reviewed the medical records of 258 children from newborn to 20 years of age who had ocular injuries severe enough to warrant admission to United Hospitals Medical Center, Newark, NJ, over a 3 1/2-year period. In this time there were 1737 pediatric admissions for eye treatment and of these, 258 (14.85%) were directly related to trauma. Only strabismus, with 676 (38.9%) admissions, accounted for more pediatric hospitalizations for eye injuries. Balls, fists, and sticks were the most common causes of injury, and the most common diagnosis was hyphema. The majority of injuries occurred in children between 11 and 15 years of age (31%). Ocular injuries in children above 10 years of age were most commonly sports-related, whereas injuries in the home accounted for the majority of trauma in children younger than 10 years of age.     

肌阵挛失张力癫(癎)的临床和脑电图特点

目的 总结肌阵挛失张力癫(癎)(MAE)的临床和脑电图特点.方法 分析MAE患儿的临床和视频脑电图及同步肌电图资料,并对治疗效果进行随访.结果 收集MAE患儿共47例,其中25例有热性惊厥史,20例有热性惊厥和(或)癫(癎)家族史,发病前智力运动发育均正常.起病年龄1.4～5.8岁,首次发作为无热的全面强直-阵挛发作(GTCS)41例(87.2%),肌阵挛发作4例,失张力发作2例.病程中均有多种发作类型,包括GTCS46例(97.9%)、肌阵挛失张力发作34例(72.3%)、肌阵挛发作47例(100%)、失张力发作32例(68.1%)、不典型失神36例(76.6%)和强直发作3例(6.4%).出现多种类型的发作时脑电图背景均为弥漫性慢波或顶区为主的θ节律,发作间期呈全导1～4 Hz(以2～3 Hz为主)棘慢波、多棘慢波发放.所有患儿均首选抗癫(癎)药物(AEDs)治疗,41例(87.2%)应用AEDs发作控制,其中37例单用或合用丙戊酸,26例联合应用拉莫三嗪.10例(21.3%)发病后出现智力落后.结论 MAE的临床特点包括:发病前发育正常,多数以GTCS起病,病程中具有多种全面性癫(癎)发作类型,肌阵挛失张力发作是其特征性发作类型;脑电图呈全导棘慢波、多棘慢波发放.早期明确诊断并合理选择AEDs是取得良好预后的关键.

Abstract：

Objective To summarize the electroclinical characteristics of myoclonic atonic epilepsy (MAE) in children. Method The clinical data, video electroencephalogram (EEG) and simultaneous electromyography (EMG) of MAE patients were analyzed. The treatment and its effects were followed up.Result In 47 MAE patients, 25 had a history of febrile seizures ( FS), 20 had a family history of FS or epilepsy. All patients had a normal development before the illness. The age of afebrile seizure onset was between 1.4 years to 5.8 years. The first seizure was generalized tonic-clonic seizure (GTCS) in 41 patients (87.2%). All patients had multiple seizure types, including 47 GTCS (97.9%), 34 myoclonic atonic seizures (72. 3% ), 47 myoclonic seizures ( 100% ), 32 atonic seizures (68. 1% ), 36 atypical absences (76. 6% ) and 3 tonic seizures (6. 4% ). EEG backgrounds were slow or parietal θ rhythm, interictal EEG showed 1-4 Hz (predominant 2-3 Hz) generalized spike and wave or poly spike and wave discharges in all cases. Seizures were controlled by antiepileptic drugs (AEDs) in 41 patients (87.2%). Valproate was used in 37. Lamotrigine was used in 26. Mild mental retardation was observed in 10 children after the onset of the illness. Conclusion The clinical features of MAE included the following: the development was normal before the onset of the illness; the onset of seizure type was often GTCS. All patients had multiple generalized seizure types. Myoclonic atonic seizure was its characteristic seizure type. EEG showed generalized discharges. Early diagnosis and rational choice of AEDs are important for getting a better prognosis.