Spectrum and presentation of pediatric malignancies in the HIV era: experience from Blantyre, Malawi, 1998-2003

BACKGROUND: Data on childhood cancers in Africa are sparse, particularly since the spread of HIV. We aimed to document the frequency of pediatric cancers presenting to a large central hospital in Malawi, detailing the presenting features, initial investigations, and HIV status of these children. PROCEDURE: A retrospective audit of the spectrum and clinical presentation of cancers among children (<16 years) seen at Queen Elizabeth's Central Hospital (QECH), between 1998 and 2003. RESULTS: Seven hundred seven children with cancer were seen, the number of cases per year increased over the time period; 50% (351) had Burkitt lymphoma, 13% (89) had retinoblastoma, and 9% (61) had Kaposi sarcoma, with a variety of other tumors comprising the remainder. Kaposi sarcoma markedly increased in frequency over time. Histological verification of diagnosis was available for 49% (348). The proportion of children with cancer who were tested for HIV increased over time, but varied by cancer type. Amongst those tested, the seroprevalence was 93% (52/56) for children with Kaposi sarcoma, 4% (11/289) for those with Burkitt lymphoma, 31% (8/26) for those with other non-Hodgkin lymphomas, 7% (1/15) for those with Hodgkin disease, and 5% (5/103) for those with other cancers. CONCLUSIONS: The number of cases seen per year has increased over the study period for almost all cancers, but in particular for Kaposi sarcoma. Burkitt lymphoma remains the commonest pediatric tumor in Malawi. In the case of Burkitt lymphoma, non-Hodgkin lymphoma, and Kaposi sarcoma there is a significant difference in the presentation of HIV-seropositive and -seronegative children.     

MRSA at an English children's hospital from 1998 to 2003.

AIMS: To investigate the epidemiological and clinical aspects of MRSA among inpatients and outpatients presenting to hospital. METHODS: Analysis of demographic, epidemiological, and clinical data collected on 385 children first identified as having MRSA between January 1998 and December 2003 in a 250 bed English children's hospital. RESULTS: There were 267 inpatients and 118 outpatients. The number of new cases of MRSA declined from 72 in 1998 to 52 in 2003, whereas hospital activity increased. Ninety nine (37.1%) inpatients acquired MRSA outside the hospital; a further 90 occurred among 31 clusters of cases. One hundred and seventy eight (66.7%) inpatients were aged <2 years; cardiac services and paediatric & neonatal surgery accounted for 59.6% of cases. Dermatology and A&E accounted for 51.7% of outpatients; 73.8% of outpatients had recently previously attended the hospital. A total of 13.9% of inpatients with MRSA developed bacteraemia; MRSA accounted for 15% of Staphylococcus aureus bacteraemias. The risk of MRSA bacteraemia in colonised patients, and the proportion of S aureus bacteraemias that were MRSA, varied between specialties. Intravascular devices were the most common source of MRSA bacteraemia (63.4% of cases). The mortality rate was 7.3%. CONCLUSIONS: Enhanced surveillance of MRSA can identify at-risk patient groups, thus facilitating targeting of control measures. The absence of a link between numbers of cases of acquisition of MRSA and bacteraemia suggests that the rise in MRSA bacteraemia may not solely reflect an increase in MRSA prevalence in children in the UK. The need for larger epidemiological studies is emphasised.     

Host and environmental factors associated with Hib in England, 1998-2002.

BACKGROUND: Declining effectiveness of the UK's Hib vaccine programme was observed between 1998 and 2002. OBJECTIVE: To provide insight into non-vaccine factors contributing to ongoing Hib disease in England after immunisation. DESIGN: Postal questionnaire study, matched case-control design. SETTING: Health Protection Agency Centre for Infections, England. Patients: Cases were children born after 1 January 1993 presenting with confirmed Hib infection in England between the start of 1998 and end of 2002, regardless of vaccination status. Controls were matched by date of birth and region. MAIN OUTCOME MEASURES: Odds ratios were calculated to assess the impact of host and environmental variables on disease risk. RESULTS: Increased disease risk was noted among children with frequent antibiotic use (adjusted OR (AOR) (trend) 1.51 (95% CI 1.06 to 2.13); p = 0.02) and from sole-parent households (AOR 2.56 (95% CI 1.24 to 5.29); p = 0.01). These two risk factors were further related to each other, consistent with previously reported associations between infection and social deprivation. In fully immunised children, receipt of all three doses of the primary course as an acellular pertussis-containing combination vaccine (DTaP-Hib) increased the risk of vaccine failure (OR 2.88 (95% CI 0.99 to 8.37), p = 0.01). Day care attendance between 2 and 5 years of age was linked with a dose-dependent reduction in risk (AOR (trend) 0.79 (95% CI 0.66 to 0.93); p = 0.01), possibly because of natural boosting of immunity. CONCLUSIONS: The association noted between invasive infection and social deprivation in this and other studies is concerning and merits further investigation. The importance of ongoing surveillance of vaccine-preventable diseases to allow nested studies of this kind was reinforced.     

Trends in diabetes mellitus among privately insured children, 1998-2002.

OBJECTIVE: To evaluate trends in health care administrative claims for childhood diabetes mellitus. METHODS: We conducted a serial cross-sectional study of a national sample of privately insured children < or = 18 years old. The number of subjects ranged from 306991 in 1998 to 974407 in 2002. We classified diabetes type by 2 schemes: one based on encounter claims only, and the other based on both encounter and pharmacy claims. The prevalence of diabetes was determined after adjusting for demographic changes in the study population, including age, proportion enrolled in managed care, and urban residence. RESULTS: The adjusted prevalence of diabetes overall increased from 183 (95% confidence interval [CI], 169-198) cases per 100,000 enrollees in 1998 to 218 (95% CI, 208-228) cases per 100,000 enrollees in 2002, primarily because of an increase in type 1 diabetes (based on both encounter and pharmacy claims, 135 [95% CI, 123-148] cases per 100000 enrollees in 1998 to 167 [95% CI, 158-176] cases per 100,000 enrollees in 2002). CONCLUSIONS: Our estimate of the overall prevalence of diabetes is consistent with national data. However, our finding that rising prevalence of type 1 diabetes appear to account for most of the increase in diabetes claims is surprising. Future research will be needed to validate these results.     

Pediatric skull base surgery. 2. Experience and outcomes in 55 patients

In the second of these two articles, we will discuss our clinical experience with skull base surgery in the pediatric population. We present a retrospective analysis of 55 patients less than 16 years of age who underwent skull base surgical approaches at the Primary Children's Medical Center or the University of Utah Medical Center between January 1992 and April 1999. There were 30 boys and 25 girls (mean age 9.8 years). Patient follow-up averaged 58 months. Most patients had pathology that required either an anterior or anterolateral approach; 6 patients underwent a far-lateral or a transpetrosal exposure. Thirty-five procedures were performed by a neurosurgeon, a pediatric otolaryngologist performed 11 procedures, and 10 procedures were performed by both services together. Ninety-six percent of patients (n = 53) had a Glasgow Outcome Score of 4 or 5. Complications included 4 sustained cranial nerve palsies and 2 hemipareses. There were no CSF leaks, infections or deaths. Patients with sellar region pathology had a disproportionately higher incidence of postoperative morbidity. We conclude that in selected pediatric cases, skull base surgical techniques can be performed effectively and safely with the use of multidisciplinary teams. To implement these techniques, knowledge of their limitations and of the anatomical differences between the adult and pediatric cranial base is essential.     

Investigation of unexplained infant deaths in Jerusalem, Israel 1996-2003.

BACKGROUND: Sudden infant death syndrome (SIDS) is a diagnosis of exclusion that may be assigned only after investigations including a forensic autopsy are performed to exclude possible organic and environmental causes of death. Israeli society is influenced by the Jewish and Islamic faiths, which permit autopsy only under selected circumstances. Against this background, we carried out a study to determine what examinations are performed to investigate unexplained infant deaths in Jerusalem, Israel. METHODS: We examined hospital, Ministry of Health and Ministry of Interior records of unexplained infant deaths in the Jerusalem district from the years 1996-2003. RESULTS: Ninety six cases were identified from all sources. Forty nine (51%) infants were brought to a hospital at or near the time of death. Studies to determine the cause of death were performed in 54% of cases for which medical records were available for review. These studies included bacterial cultures (44%), skeletal surveys (12%), computerised tomography (3%) and metabolic studies (3%). Only one forensic autopsy was performed, and in no instance was the death site examined by medical personnel. There was a high rate of retrospective review by district health physicians. The most frequently assigned cause of death was SIDS. CONCLUSIONS: : The capacity of public health officials and forensic pathologists to investigate unexplained infant deaths is strongly affected by the legal, religious and political milieu in which they work. Efforts should be made to develop socially acceptable methods of improving the quality of infant death investigations in Jerusalem.     

Kawasaki disease in Korea, 2003-2005

PURPOSE: The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for the 3-year-period, 2003-2005. METHODS: The questionnaire for an epidemiologic survey on Kawasaki disease was distributed to all 102 Korean hospitals that conduct pediatric residency programs, and obtained data were analyzed upon receipt. RESULTS: The 9662 patients of Kawasaki disease from 85 hospitals that responded (response rate, 83.3%) consisted of 5877 males and 3785 females (male:female ratio, 1.55:1). The incidence rate per 100,000 children <5-year-old was 104.2 in 2003, 106.4 in 2004, and 104.6 in 2005 (average rate, 105.0). Their mean age of onset was 33.3 months, and the proportions of sibling cases and recurrent cases were 0.29% and 2.0%, respectively. Coronary arterial abnormalities were detected at follow-up by echocardiogram in 18.8% of all such cases including dilatations of 18.0% and aneurysms of 2.5%. CONCLUSION: The average annual rate of incidence, 105.0/100,000 in children <5-year-old is the second highest reported rate in the world.     

Varicella-related deaths in children and adolescents--Germany 2003-2004.

AIM: Although varicella is acknowledged as a rare cause of death in children, there are few comprehensive data with respect to the clinical course leading to death. METHODS: A nationwide, active surveillance was carried out in Germany for children up to age 17 years who were admitted to a paediatric hospital for varicella or associated complications, including deaths. RESULTS: A total of 10 children with varicella-associated death were reported over period of 2 years, yielding a mortality rate of 0.4/1 000 000 children per year. Three deaths occurred in children diagnosed with acute lymphocytic leukaemia and disseminated varicella, two shortly after diagnosis of leukaemia and therefore not preventable, and one during remission with an untypical presentation. Two children died with a congenital varicella syndrome. There was no death in children with neonatal varicella. Four other cases were related to varicella pneumonia or septicaemia and one to myocarditis. CONCLUSION: In a population with no general varicella vaccination programme, varicella accounted for a small but not negligible risk for death in immunocompetent and immunocompromised children. Together these data point to the importance of a thoroughly implemented, general varicella vaccination programme.     

Acute childhood leukemia in Johannesburg. Ethnic differences in incidence, cell type, and survival

Acute childhood leukemia was found to be less common in black children than in white children in the Johannesburg area of South Africa. Of 195 consecutive patients, 78 were black and 117 white. The annual incidence was 0.8/100,000 black children and 3.3/100,000 white. The low incidence in black children was due to the very low incidence of acute lymphoblastic leukemia (ALL), which was 0.41/100,000 black children compared with 2.73/100,000 white children. The incidence of acute nonlymphoblastic leukemia (ANLL) was approximately the same for both ethnic groups; 0.38/100,000 black children and 0.57/100,000 white children. Remission rates for black children with ALL were lower than for white children and the cumulative proportion of black patients surviving at 60 months was only 32% compared with 72% of white patients (p = 0.0001). The most significant poor prognostic factors in ALL were ethnic group and age (p = 0.0006), CNS disease at onset (p = 0.006), FAB L2 and L3 morphology (p = 0.05), and irregular clinic attendance during maintenance therapy (p = 0.05). In ANLL, remission and survival rates were less favorable than in ALL but there were no significant differences between black and white patients. Black patients exhibited certain clinical features rarely seen in white patients, including chloromata, oropharyngeal lesions, and CNS involvement at onset. Karyotypic abnormalities were common. The most significant poor prognostic factors in ANLL were CNS disease at onset (p = 0.03), generalized lymphadenopathy (p = 0.0001), and FAB morphology classification M3-M6 (p = 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)     

儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的诊治分析

目的总结儿童囊性肾瘤和囊性部分分化型肾母细胞瘤的临床、病理特点,探讨合理的治疗方法。方法回顾性分析本院收治的7例儿童囊性肾瘤、6例囊性部分分化型肾母细胞瘤患儿的临床资料,包括年龄、临床表现、影像学及病理检查结果、治疗和预后。结果13例患儿中,男8例,女5例,年龄4个月至4岁,平均1岁7个月。左侧6例,右侧5例,双侧2例。腹部包块9例,B超偶然发现4例,术前均行B超和增强CT检查。5例7侧行肿瘤剜除术,8例行瘤肾切除术。术后随访6个月至7年,未见肿瘤复发,保留肾脏的5例中,7侧残肾功能良好。结论囊性。肾瘤和囊性部分分化型肾母细胞瘤患儿术前无法鉴别,手术完整切除是主要的治疗方法,肿瘤位于肾脏一极或双侧者可行保留肾脏的肿瘤剜除术。囊性肾瘤为良性病变,术后无需化疗,囊性部分分化型。肾母细胞瘤为低度恶性或潜在恶性,Ⅰ期者可单纯手术治疗,Ⅱ期以上需行手术＋化疗。     

广州地区1998～2003年儿童流行性感冒病毒监测结果分析

目的研究1998-2003年广州地区儿童流感病毒(流感)感染的流行情况。方法对流感样症状的儿童取咽分泌物,接种到狗肾细胞(MDCK)上,在33℃温箱培养48-72h。MDCK细胞上清液进行血凝试验。结果3444例中264例分离出流感病毒,流感病毒分离总阳性率7.7%。1998、1999年流感阳性率分别为16.8%、7.4%,H3N2是强势株;2000年流感分离阳性率8.4%,出现H1N1亚型;2001年流感分离阳性率3馏%,未分离到H3N2亚型;2002年流感分离阳性率7.3%,B型流感病毒为强势株;2003年分离阳性率1.7%。1998-2001年广州地区儿童B型流感病毒流行为Yamagata型,2002-2003年流行为Victoria型。1999年发现禽流感H9N2亚型。结论1998年为流感流行较活跃年份,2001和2003年流感活动较平静;1998-2001年广州地区儿童B型流感流行Yamagata型,2002-2003年流行Victoria型。1999年发现儿童感染H9N2个案。