Health reform for children in Japan's aging society: A paradigm shift for pediatricians*

Japan confronts a dilemma in reshaping its health care for children: the low birth rate is reducing the proportion of children in the overall population, while the health issues for children are becoming increasingly complex and connected to social behavior. The present paper reviews Japan's changing demographics and the declining proportion of children in the population structure. This change has important implications for how the government sets priorities in the health sector. Next, the paper considers how 'problems' are defined for health policy in society and how the agenda for health reform is determined. Attention is directed to the political dimensions of these two processes of problem definition and agenda setting. Two examples of health reform for children, in other countries, are discussed to show how these two processes have worked in practice. In the next section, the paper explores new health issues for children in Japan and the challenges to Japanese pediatricians in shaping the agenda for health reform.     

Situational analysis of maternal and child health services for foreign residents in Japan

BACKGROUND: Since the 1980s, the number of foreign residents in Japan has continuously been on the rise. In order to improve the foreign resident support services infrastructure and effectiveness, a survey was conducted at a national level from February to August 2002. The survey was done via a mailed questionnaire to all municipalities in Japan. METHODS: For the purpose of the study, municipalities were divided into four groups based on level of urbanization and numbers of foreign residents in the area. The situation of the foreign maternal and child support programs were compared and the municipal administrations were evaluated. In addition, the attribution analysis and evaluation of the foreigner support program was conducted in each municipality group. RESULTS: The evaluation of the current service support program for foreigner was not judged positively in the majority (95%) of the municipalities. In the non-urbanized municipalities with a low composition of foreigners, the foreigner mother and child support program were not functional compared with other regions. Additionally, various factors were highlighted based on attribution analysis among each group. Although most of the municipalities recognized the importance of the foreign resident support program, the evaluations showed a wide gap between intention and reality. CONCLUSION: It is recommended that the barriers as identified in the research results are rectified, and the current situation improved based on municipality characteristics, local demands and the needs of the population. The efficient use of limited fiscal and human resources is also advocated by strengthening of cooperation with other official bodies and also employing foreign residents to work in public offices for facilitating the foreign residents support programs.     

Incidence of Kawasaki disease in Japan: the nationwide surveys of 1999–2002

BACKGROUND: The purpose of the present study was to describe the results of nationwide epidemiologic surveys of Kawasaki disease for the 4 year period 1999-2002. METHODS: The design is a retrospective incidence survey. The patients reported in these two surveys are all new patients who were reported during the two study periods (1999-2000 and 2001-2002), although the data were collected retrospectively. A questionnaire was sent to all pediatric departments of hospitals with 100 beds or more throughout Japan, requesting data on patients with Kawasaki disease, such as sex, age, date of first hospital visit, recurrence, and cardiac lesions. RESULTS: The total number of patients reported in the 4 year period 1999-2002 was 32 266 (18 604 male, 13 662 female), with an average annual incidence of 137.7 per 100 000 children younger than 5 years old. The male/female ratio was 1.30. The incidence peaked at 9-11 months of age, and the proportion of patients under 1 year of age was 26%. The monthly distribution had a high peak in January and a gradual increase in summer. Geographically, the high-incidence areas were limited to certain prefectures and moved from year to year. The cardiac lesions at acute stage and cardiac sequelae occurred more in children under 1 year and older than 4 years. Among the principal symptoms, fever persisting >or=5 days occurred most commonly, followed by conjunctival congestion, changes in lips and oral cavity, polymorphous exanthema, and changes of extremities. Cervical lymphadenopathy occurred less. CONCLUSION: More than 32 000 patients with Kawasaki disease during the 4 year period 1999-2002 were reported to the nationwide incidence surveys. The number of patients is steadily increasing despite the decrease of children. The seasonal variation, geographical distribution, and age-specific distribution support the infection theory for the etiology of Kawasaki disease.     

X 染色体连续缺失致慢性肉芽肿病和 Mcleod 综合征 2 例报告并文献复习

目的探讨X染色体连续缺失所致的与X连锁慢性肉芽肿病(X-CGD)相关的Mcleod综合征的临床特征。方法回顾分析2例经基因分析诊断为Mcleod综合征和X连锁慢性肉芽肿病患儿的临床资料。结果两例男性患儿分别为4岁1个月及1岁9个月,均以迁延性肺内感染入院,既往有反复重症感染史,均有卡介苗相关淋巴结炎,因呼吸爆发缺陷和CYBB基因全外显子缺失明确诊断为X连锁慢性肉芽肿病。患儿同时有运动落后,用MLPA方法分别检测到DMD基因外显子1-44及肌肉特异性启动子区及外显子1-2缺失,诊断为Duchenne型肌营养不良(DMD)。例1外周血涂片有明显棘状红细胞,例2 XK基因突变分析为全外显子缺失,诊断为Mcleod综合征。结论 X染色体连续缺失可导致Mcleod综合征及DMD和X-CGD的组合,使病情复杂化。由于缺乏Kx抗原,反复输普通血会产生相关抗体,出现急骤的输血危象。     

Children and the Patient Protection and Affordable Care Act: Opportunities and Challenges in an Evolving System

The Patient Protection and Affordable Care Act (ACA), passed in 2010, focused primarily on the problems of adults, but the changes in payment for and delivery of care it fosters will likely impact the health care of children. The evolving epidemiology of pediatric illness in the United States has resulted in a relatively small population of medically fragile children dispersed through the country and a large population of children with developmental and behavioral health issues who experience wide degrees of health disparities. Review of previous efforts to change the health care system reveals specific innovations in child health delivery that have been designed to address issues of child health. The ACA is complex and contains some language that improves access to care, quality of care, and the particular needs of the pediatric workforce. Most of the payment models and delivery systems proposed in the ACA, however, were not designed with the needs of children in mind and will need to be adapted to address their needs. To assure that the needs of children are met as systems evolve, child health professionals within and outside academe will need to focus their efforts in clinical care, research, education, and advocacy to incorporate child health programs into changing systems and to prevent unintended harm to systems designed to care for children.     

Patterns of disease among children: A simple and versatile measure of child health in communities

Objective : To derive an indicator of community child health that is simple, stable, cost-effective and suitable for small communities.
Methodology : Discharge diagnoses of children have been tabulated for hospitals in Australia and other countries and from an Aboriginal community, for up to 40 years. The differences in ratios between main diagnostic groups relate to the child health status in the community.
Results : As we pass from 'developing' to 'developed' conditions, the proportion of different diagnoses changes with a relative decrease in gastroenteritis and a relative increase in respiratory and other diseases. These changes parallel changes in infant mortality rates and child growth that are independent indicators of community child health.
Conclusions : The ratios of diagnoses of hospital discharges, the 'disease pyramid' is a reliable, valid and cost-effective way of measuring child health in a community while avoiding many of the uncertainties of conventional disease-specific and age-specific discharge rate statistics.     

Chronic granulomatous disease: uptake and intracellular activity of fosfomycin in granulocytes

In chronic granulomatous disease (CGD) polymorphonuclear leukocytes (PMN) are unable to kill phagocytized catalase-positive bacteria. Therefore, patients with CGD are prone to infections and dependent on antimicrobial agents able to penetrate PMN membranes and to act intracellularly. Owing to their good lipid solubility, trimethoprim/sulfamethoxazole and rifampicin passively diffuse the membrane. In contrast, fosfomycin is transported actively into the cell. In normal PMN, it reaches cellular-to-extracellular ratios of 1.83 after 15 min, in CGD-PMN 2.18 after 30 min. At concentrations between 16 and 200 mg/liter, fosfomycin was able to kill staphylococci surviving within CGD-PMN, thus compensating for the bactericidal deficiency in CGD. A combination of low concentrations of fosfomycin (8 mg/liter) plus rifampicin (0.06 mg/liter) was more effective at the intracellular level than either agent alone. Apart from a stimulation of PMN-chemiluminescence of yet unknown significance, the agent did not interfere with other neutrophil functions. Clinical investigations are indicated to study whether fosfomycin can be added to the small number of antibiotics useful in CGD.     

Psychosomatic disorders in children: An emerging challenge to health care in Japan

BACKGROUND: Japan has been witnessing a considerable rise in the number of children presenting with psychosomatic disorders. Thus, there is a mounting demand for pediatricians to be trained in the caring of children with psychosomatic disorders. METHOD: A questionnaire based-survey was conducted to investigate the average number of working hours for the first year (Postgraduate Year 1 (PGY1)) pediatric residents at Keio University Hospital. The same survey was conducted retrospectively with doctors with at least 10 years of experience in child health care. Another survey was conducted at our 27 affiliated hospitals with respect to the number of outpatients and patients presenting with psychosomatic disorders. RESULTS: The survey showed that, in the ward for preschool and school-age children, PGY1 residents spent on average 78.9 h per week. Time required for care of psychosomatic disorders was more than 30% of the total amount of time allotted for information acquisition, interviews and consultations, as well as conferences and discussions. There was virtually no time spent on the care for psychosomatic disorders in our pediatric residency program 10 years ago. It is an implication of the survey that the apparent number of patients with psychosomatic problems is not dependent on the size or location of the hospital. CONCLUSION: General pediatricians will be spending more time than ever before in the caring of children with psychosomatic disorders. Moreover, pediatricians trained for child health care for psychosomatic disorders are short of supply or non-existent, even in the major affiliated hospitals.     

Chronic granulomatous disease: A different pattern in Hong Kong?

From July 1988 to December 1989, six boys with chronic granulomatous disease were diagnosed in our institutions. Their clinical features were reviewed in order to delineate the pattern of infections which seems to have both similarities and differences when compared with published reports of Caucasian patients. The most striking differences was the lack of skin sepsis and chronic lymphadenitis in our six patients. Gram-negative organisms were the commonest pathogens while Staphylococci sp. were not isolated. Clinical features which should alert one to the diagnosis were also highlighted. Prophylactic co-trimoxazole was effective in reducing the frequency of bacterial infections. Early diagnosis is not only essential for optimal patient management but also for genetic counselling for the extended family.     

Chronic granulomatous disease: Clinical,molecular, and therapeutic aspects

Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by defects in the genes encoding any of the NADPH oxidase components responsible for the respiratory burst of phagocytic leukocytes. CGD is a genetically heterogeneous disease with an X‐linked recessive (XR‐CGD) form caused by mutations in the CYBB gene encoding the gp91^phox protein, and an autosomal recessive (AR‐CGD) form caused by mutations in the CYBA, NCF1, NCF2, or NCF4 genes encoding p22^phox, p47^phox, p67^phox, and p40^phox, respectively. Patients suffering from this disease are susceptible to severe life‐threatening bacterial and fungal infections and excessive inflammation characterized by granuloma formation in any organ, for instance, the gastrointestinal and genitourinary tract. An early diagnosis of and the prompt treatment for these conditions are crucial for an optimal outcome of affected patients. To prevent infections, CGD patients should receive lifelong antibiotics and antifungal prophylaxis. These two measures, as well as newer more effective antimicrobials, have significantly modified the natural history of CGD, resulting in a remarkable change in overall survival, which is now around 90%, reaching well into adulthood. At present, hematopoietic stem cell transplantation (HSCT) is the only definitive treatment that can cure CGD and reverse organ dysfunction. Timing, donor selection, and conditioning regimens remain the key points of this therapy. In recent years, gene therapy (GT) for XR‐CGD has been proposed as an alternative to HSCT for CGD patients without a matched donor. After the failure of the first trials performed with retroviral vectors, some groups have proposed the use of regulated SIN‐lentiviral vectors targeting gp91^phox expression in myeloid cells to increase the safety and efficacy of the GT protocols.     

Chronic granulomatous disease in Latin American patients: clinical spectrum and molecular genetics

BACKGROUND: Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by early onset of recurrent and severe infections. The molecular defects causing CGD are heterogeneous and lead to absence, low expression, or malfunctioning of one of the phagocyte NADPH oxidase components. The aim of this study was to analyze the clinical features and to investigate the molecular genetic defects of Latin American patients with CGD. PROCEDURES: The study included 14 patients. The diagnosis was based on a history of recurrent severe infections, impaired respiratory burst, and the demonstration of an underlying mutation by single strand conformation polymorphism (SSCP) or RT-PCR analysis, followed by genomic DNA or cDNA sequencing. RESULTS: Seven unrelated patients were found to have the X-linked form of CGD (X-CGD). Heterogeneous mutations affected the CYBB gene: two insertions, one substitution, and four splice site defects; two of them are novel. Seven patients presented with one of the autosomal recessive forms of CGD (A47-CGD); all had the most common mutation, a DeltaGT deletion in exon 2 of the NCF1 gene. Pneumonia was the most frequent clinical feature, followed by pyoderma, sinusitis, otitis, and liver abscess. Patients with X-CGD were more likely to have initial infections before age 2 years and to have inflammatory obstructive granulomas later. None of the patients had severe adverse reactions to BCG immunization. CONCLUSIONS: X-CGD patients from Latin America showed a high degree of molecular heterogeneity, including two novel mutations. Their clinical characteristics included early onset of infections and eventual obstructive granulomas. A47-CGD represented 50% of the reported cases, a higher prevalence than reported in other series.     

Clinical features and natural history of physical urticaria in children

Physical triggers are the most common precipitating factors for chronic urticaria in childhood. We investigate the natural history of physical urticaria (PU) and attempt to identify prognostic features. A retrospective study covering a 3 yr period (1999–2002) identified 82 children with PU seen in a tertiary referral centre. Parents of 53 children with PU responded to follow up by questionnaire and were included in the data analysis. The questionnaire ascertained symptom severity, remission status, effects of treatment and prognostic factors in determining resolution of the urticaria. Not all parents answered every question. Fisher’s exact test was used to compare the remission and non‐remission groups and Kaplan–Meir survival analysis was used to derive estimates of time to achieve remission. All 53 children with PU had chronic urticaria, with dermatographic, cholinergic and mixed subtypes of PU forming over 70% of the subtypes. Overall, 13% (6/45) of the children reported respiratory symptoms with the episodes of urticaria, and 67% (35/52) reported associated angioedema. In univariate analysis, a history of other allergic conditions in the child was associated with a greater chance of non‐remission (p = 0.007). No significant difference in the age of onset of urticaria and duration of individual bouts was noted between the remission and non‐remission groups. Episodes of urticaria were significantly less frequent (p = 0.02) in the remission group (monthly/weekly/daily – 70%/30%/0%) than the non‐remission group (38%/34%/28%). Among the 20 children achieving remission, on average this occurred 30 months after onset of symptoms. In the survival analysis, the number of children becoming urticaria free was 11.6% (95% CI: 5.4%–23.9%) at 1 yr post‐onset and 38.4% (95% CI: 25.9%–54.3%) 5 yr post‐onset. In conclusion, based on this selected tertiary population, the prognosis for PU may not be as benign as has previously been reported. A history of allergic conditions and more frequent episodes of urticaria were associated with a poorer prognosis.     

Chronic active Epstein-Barr virus infection in children in Japan

The patients with chronic active Epstein-Barr virus infection (CAEBV) in childhood in Japan are described. Among 39 registered cases, 20 patients were males and 19 were females. Unlike the X-linked lymphoproliferative syndrome, there was no hereditary background. The incidence of hypersensitivity to mosquito bites was high (31.3%) as a past history. Most patients exhibited hepatomegaly (92.3%), splenomegaly (87.2%) and fever (84.6%). The incidence of absent anti-EB virus nuclear antigen titres was unexpectedly low (17.1%). Lymphoreticular disorders and cardiovascular diseases were major complications. Twenty-four (61.5%) patients died 6 months to 8 years after the onset, mainly of hepatic failure (eight cases), cardiac failure (five cases), virus-associated haemophagocytic syndrome (three cases) and haematological malignancies (two cases). This study reveals that CAEBV in Japan has several clinical features and should be informative for the pathogenesis of EB virus.     

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目的总结分析感染洋葱伯克霍尔德菌的X连锁慢性肉芽肿病患儿的临床特点。方法回顾分析2010年1-2月于北京儿童医院住院治疗的2例感染洋葱伯克霍尔德菌的X连锁慢性肉芽肿病患儿的临床资料。结果 2例男性患儿,分别为0.5岁和1.7岁,均经CYBB基因突变分析明确诊断为X连锁慢性肉芽肿病。1例经反复血培养、1例经反复尿培养诊断为洋葱伯克霍尔德菌感染。药敏试验结果提示均为敏感菌株。结论洋葱伯克霍尔德菌是人类机会性病原,常表现为慢性肉芽肿病。常规抗生素治疗可根除感染。预防应用复方磺胺药物对此类患儿具有保护意义。