Pediatric and MCH training in Japan: JICA training program in the National Children's Hospital

The experience of the training program for overseas doctors in the National Children's Hospital during 8 years is described. The program was supported by the Japan International Cooperation Agency (JICA) and the objectives of the training course are to provide doctors from developing countries with a better understanding of diagnosis and treatment as specialized pediatricians and pediatric surgeons and to introduce recent medical techniques and equipment for child care. From 1984 to 1993, 37 doctors from 22 developing countries of Asia, Middle and South America and Africa have attended this program. There were 22 pediatricians, 14 pediatric surgeons and one dentist. The outline of this group training program and problems with the course are discussed.     

The joint JMA-JICA project in Nepal

Nepal is among the 20 countries with the lowest human development index, according to the criteria of the United Nations Development Program (UNDP), and has a very high under-five mortality rate, according to the United Nations Children's Fund (UNICEF).¹ In December 1992, the Japan Medical Association (JMA) and the Japan International Cooperation Agency (JICA) began jointly carrying out a maternal and child health (MCH) project. Its beneficiaries are the population of Kavre District, the District Public Health Office in Kavre, the Central Region Health Directorate, and the Ministry of Health of Nepal. The MCH project stems from a request to Japan made by His Majesty's Government of Nepal to implement and evaluate a new health system policy in a model area, the Kavre District. The project's main goal is to improve the general health and to reduce the maternal, infant, and under-five child mortality rates by providing basic primary health care services and means of disease prevention. It will improve the government's medical care delivery system, better educate the population and promote community action in questions related to health and hygiene. This is the first cooperative project organized by JICA that involves the participation of a nongovernmental organization, in this case, the JMA. The JMA will construct the primary Health Care (PHC) Center, equip it with a dormitory and safe water supply system, and provide sufficient drugs for 2 years. The Japan International Cooperation Agency will dispatch MCH experts and provide medical equipment and supplies. The government of Nepal will dispatch a medical staff of 12 members, including a medical doctor, nurses, midwives, and laboratory technician, and other assistants as counterparts, and will be supported and monitored by the National Children's Hospital Medical Research Center of Japan. This joint project and new health policy will be evaluated after 2 years to decide whether the new PHC center system should be expanded.     

Carers' perception of childhood asthma and its management in a selected Pakistani community

Methods: Subjects were children''s parents/guardians visiting the asthma clinic, Children''s Hospital, Islamabad. The questionnaire included items on general understanding of asthma, its triggers, and management. Results: Two hundred carers of asthmatic patients participated. Thirty six per cent thought that asthma is a communicable disease. Rice and oily foods were blamed for asthmatic exacerbations in up to 57% of cases; 82% felt that inhalation therapy is effective in controlling asthma symptoms. Conclusions: Asthma awareness is inadequate. The majority of the carers unnecessarily blamed and withheld many nutritious foods. Social stigmata can undermine the self esteem of growing asthmatics. Lack of awareness is not significantly related to the socioeconomic or educational background. Awareness raising strategies are needed in the community.     

Hodgkin's disease and non-Hodgkin's lymphoma in children and young adults: A clinicopathologic study of 127 cases

One hundred twenty-seven cases of non-Hodgkin's lymphoma and Hodgkin's disease in children and young adults at the University of Wisconsin Hospital between 1969 and 1980 have been reviewed. Nodular sclerosing was the most frequent histologic type in patients with Hodgkin's disease. Malignant lymphoblastic lymphoma (MLLB) was the most common type of non-Hodgkin's lymphoma. The relationship of the histological pattern to age and sex as well as clinical behavior and survival are discussed.     

A new therapy schedule for pediatric non-Hodgkin lymphoma toxicity and preliminary results

A pilot study of the toxicity and efficacy of a new treatment schedule for childhood non-Hodgkin's lymphoma was conducted by members of the Children's Cancer Study Group (CCSG) prior to its use in a randomized phase III trial. Chemotherapeutic agents used were cyclophosphamide (CPM), vincristine (VCR), and prednisone, together with intravenous (IV) and intrathecal methotrexate (IT MTX). Radiation therapy was also employed. From September 1976 to April 1977, 27 eligible, newly diagnosed patients with non-Hodgkin's lymphoma were entered onto this pilot study. Toxicity was acceptable with minor adjustments in dosage and timing of the myelosuppressive agents. Fourteen of the 22 patients entered onto maintenance remain entirely disease-free, and all have completed the prescribed course of chemotherapy. None of the 12 patients characterized as having a “favorable” prognosis has relapsed, with a median follow-up of 27 months from on study.     

Differences in dose scheduling as a factor in the etiology of anthracycline-induced cardiotoxicity in Ewing sarcoma patients

Clinical observation suggested a high prevelance of cardiac morbidity and mortality in children with Ewing sarcoma (ES) treated at B.C.'s Children's Hospital. We therefore compared 30 patients treated for Ewing sarcoma between 1978 and 1991 with 26 soft tissue sarcoma (STS) patients treated with similar chemotherapy over the same period of time. All patients were evaluated for cardiac function using echocardiography. Shortening fraction (SF) and left ventricular mass index (Massl) were compared before and after treatment. The role of chest irradiation, dose concentration (DC) of adriamycin (AD), total mean doses of AD, cyclophosphamide (CY) and actinomycin (AC) were analysed. SF for patients with ES and STS postchemotherapy was significantly lower (P ≪ .001 and P = 0.0004, respectively) than pretreatment values. Postchemotherapy SF for ES was lower than STS (P = 0.0097). Massl for each group did not change significantly. Six of the ES patients had postchemotherapy SF of <0.20, with three in congestive failure, two cardiac deaths and one heart transplant. One additional ES patient had sick sinus syndrome and needed a pacemaker. Among the STS patients only one had SF < .20 and none were symptomatic. There were no significant differences in the mean AD, CY and AC doses for ES versus STS. The difference in the DC of AD for ES (mean 744) compared to STS (mean = 362) was significant (P = < 0.001). Regression analysis indicated a trend for decreasing SF with increasing DC (P = 0.017). Chest irradiation did not appear to increase the likelihood of cardiotoxicity. ES patients had a higher prevalence of cardiac dysfunction compared to STS. Studies are required to evaluate the importance of the components of DC, i.e., size of the individual dose and frequency of administration of AD, and to look at other possible factors in the causation of cardiomyopathy in ES. © 1997 Wiley Liss, Inc.     

Novel inpatient surveillance in tertiary paediatric hospitals in New South Wales illustrates impact of first-wave pandemic influenza A H1N1 (2009) and informs future health service planning

Aim: To document the impact of pandemic influenza A H1N1 (2009) in New South Wales (NSW) children's hospitals. Methods: A novel surveillance system, Paediatric Active Enhanced Disease Surveillance (PAEDS), identified hospitalised children <15 years with laboratory‐proven influenza (1 June–30 September 2009) in the three children's hospitals in NSW: Children's Hospital at Westmead (CHW), Sydney Children's Hospital, John Hunter Children's Hospital. Clinical characteristics, management and complications were documented, and at CHW comparison made with 2007 data. Results: The 324 children identified represented 1802 hospital bed‐days and 230 PICU bed‐days. Most (73.1%) children had H1N1, one had an oseltamivir‐resistant isolate. Median age was 2.5 years: 65% were <5 years. Although 80.9% had cough, 8.0% had no respiratory symptoms. Complications occurred in 34.6%, of whom 56% were previously healthy. Only 50% received antivirals. Forty children (12.3%) were admitted to PICU: one child with H1N1 died. At CHW, comparison between 2009 and 2007 showed nearly twice the total number of admissions (226 vs. 122) and PICU admissions (22 vs. 13), but no deaths either year. Vomiting was more frequent in 2009 than 2007 (38.5% vs. 13.1%; P = 0.0001) as were neurological complications (11.4% vs. 2.4%; P = 0.0027) but length of hospital and PICU stay were similar. Conclusions: PAEDS is a valuable surveillance tool that documented the impact of the H1N1 (2009) pandemic in NSW children's hospitals. High numbers of complications, often in previously well children, suggest an important role for early diagnosis, antiviral therapy and influenza vaccination. Observed regional differences identify areas potentially at greater risk in a subsequent wave.     

Role of a children's hospital safety centre in injury control

Over the past 13 years, child safety centres have been established at several Australian Children's hospitals. In a short time, they have developed an important role in community injury control programmes. These centres provide an efficient conduit for transferring up-to-date injury surveillance and prevention information to families, industry, health and education professionals, and government. The activities of the Child Safety Centre at the Royal Children's Hospital in Melbourne are described, together with ways in which a degree of self-funding can be achieved.     

Hirschsprung's Disease: a Clinical and Pathologic Study in Iranian Constipated Children

Objective

Hirschsprung''s disease (HD) is a complex disorder resulting from absence of ganglion cells in the bowel wall leading to functional obstruction and bowel dilatation proximal to the affected segment. The aim of our study was to evaluate rectal biopsies from constipated children in different age groups to see in which age it is more likely to encounter HD to avoid unnecessary rectal biopsy.

Methods

Records of all children with chronic constipation undergoing a rectal biopsy to exclude HD were obtained from the files of Children''s Medical Center in Tehran, Iran. A detailed retrospective demographic review, including age of beginning of signs and symptoms was made of all cases.

Findings

Totally, 172 biopsies were taken from 168 children in a five year period, of which 127 cases (75%) had HD. The mean age of constipated patients at biopsy was 39 months and the mean age of patients with proven HD was 18 months. Males were affected more than females. Congenital anomalies associated with HD were found in 9.6%. In 85 (91%) cases constipation had begun in neonatal period.

Conclusion

Our data supports previous studies that if constipation begins after the neonatal period, the child is unlikely to have HD. In neonates delay in meconium passage is the most important clinical sign of HD.     

Homer's syndrome: An unusual presentation of Hodgkin's disease

Horner's Syndrome (ptosis, anisocoria, and anhydrosis) developed in a 22-year-old woman. A mediastinal mass was noted on chest x-ray. Further evaluation led to a diagnosis of Hodgkin's disease, nodular sclerosis type. Horner's syndrome is an unusual initial manifestation of Hodgkin's disease, and, in this case, it was due to oculosympathetic damage from mediastinal compression. Because the potential for cure is high in Hodgkin's disease, this diagnosis should be considered in patients presenting with Horner's syndrome.     

CUSHING'S SYNDROME IN A THREE MONTH OLD GIRL

This paper describes the rare occurrence of Cushing's syndrome in a female infant aged eleven weeks. An adrenal adenoma was found to be the cause, and its removal was followed by a return to a normal appearance. This patient is the first with this condition seen in this age group at the Royal Children's Hospital since 1948. The Hospital's experience with adrenal tumours since that time is also presented, together with a review of the recent literature.     

Non-urgent use of the Emergency Department of the Bambino Gesù Hospital: a cross sectional survey of parents' motivations

The objective of the study was to identify the reasons why families choose to present to a paediatric Emergency Department (ED), in particular the Bambino Gesù Children's Hospital, with non-urgent conditions, and to discern their relationship with paediatric family practice (primary care). For this, a cross-sectional survey was made of families of children and adolescents aged between 0 and 14 years who presented to the ED of the Bambino Gesù Children's Hospital during a 1-month period. Questionnaires were returned concerning 310 patients with a non-urgent triage code priority. The predominant reason for paediatric ED consultation was the perception of need for urgent care (28%), followed by verbal referral from primary care (26%). The majority of the population surveyed (70%) was self-referred. The most common clinical reason for presenting to the ED was fever (27%) or respiratory symptoms (respiratory distress 12%, cough 14%). While 95% of respondents claimed to have a primary care physician as their children's healthcare referrent, 51% also referred to private practice and hospital-based care when necessary. Furthermore, 80% had been to the ED more than once, 10% more than five times, and 9% more than 10 times; 41% had been admitted at least once. Most parents claimed to be very (37%) or moderately (52%) worried. The majority chose the Bambino Gesù Hospital because they considered that their problem would be dealt with more rapidly there (65%); others referred to the absence of paediatric emergency care in their own district (20%). Most parents considered that they had sufficient health education to deal with the most common problems presented by their children (69%). In conclusion, the majority of non-urgent presentations to the Bambino Gesù Hospital ED were self-referrals. The hospital was chosen because it was considered to be reliable and efficient in solving children's problems more quickly. Parents' anxiety and difficulties accessing primary healthcare emerged in this study, which suggests a gap in the public service that is being inadequately filled by the ED.     

Evaluation of Treatment Begun in First Three Months of Life in 184 Cases of Phenylketonuria

Data on 184 patients (of whom 15 were treated at Alder Hey Children''s Hospital), who began treatment for phenylketonuria (PKU) in the first three months of life, are presented and reviewed. The intelligence and physique of these patients are slightly below average, but the majority do not show evidence of severe mental subnormality such as usually occurs in untreated PKU.     

Children's Oncology Group's 2013 blueprint for research: Acute myeloid leukemia

For the 365 children diagnosed with acute myeloid leukemia in the US annually, 5‐year survival for patients on COG trials with low, intermediate, and high risk disease is 83%, 62%, and 23%, respectively. Recent advances include improved therapeutic stratification, improved survival with dose intensification, and further elucidation of the heterogeneity specific to childhood AML. These discoveries now guide current strategy incorporating targeted agents to pathways specific to childhood AML as well as evaluating methods to increase the sensitivity of the leukemic stem cell, first in Phase II feasibility trials followed by Phase III efficacy trials of the most promising agents. Acute myeloid leukemia in children, though with similar subgroups to adults, remains uniquely different based upon quite different prevalence of subtypes as well as overall response to therapy. The Children's Oncology Group's research agenda builds upon earlier efforts to better elucidate the leukemogenic steps distinct to childhood AML in order to more scientifically develop and test novel therapeutic approaches to the treatment and ultimate cure for children with this disorder. Pediatr Blood Cancer 2013; 60: 964–971. © 2012 Wiley Periodicals, Inc.     

Obituary: Yehuda Matoth (1913-1986)

Like pediatricians elsewhere, Austrian pediatricians have always dealt with hematological and oncological problems. However, up to the 1970s in Austria only case reports and a few single institutional reviews with generally unsatisfactory therapy results have been published.^1-3 With the issue of the first cooperative and prospective treatment protocol for acute lymphoblastic leukemia (ALL) in 1974, a fruitful nationwide cooperation in the field of hematology/oncology began.⁴ This date is considered the birthday of the Austrian Leukemia Study Group. In 1978, the Austrian Pediatric Oncology Group was established as a result of the group's intention to encompass all pediatric malignancies in its collective work. The main objective of the group was the development of standardized therapy protocols with the aim of improving the results nationwide. The achievement of this goal has to be largely attributed to the work of Paul Krepler, MD, the former head of the St. Anna Children's Hospital in Vienna. Since 1974, six consecutive national ALL studies have been performed. Three of them were essentially derived from the Memphis protocols, the latter from modifications of the Berlin-Frankfurt-Münster (BFM) strategy.^5,6 Furthermore, national cooperations in the treatment of Wilms’ tumor,⁷ neuroblastoma,⁸ non-Hodgkin's lymphoma,⁹ soft-tissue sarcoma,¹⁰ acute myeloid leukemia,¹¹ and histiocytosis syndromes were initiated, in part with international cooperation.     

Higher protein and energy intake is associated with increased weight gain in pre‐term infants

Aim: To characterise and compare the nutritional management and growth in infants <33 weeks' gestation in two tertiary centres. Methods: An audit of daily intake and growth from birth to discharge home was undertaken in two neonatal units: The KK Women's and Children's Hospital Singapore and the Adelaide Women's and Children's Hospital, South Australia. Mixed models were used to model intake and daily weight (g/day) accounting for repeated day per subject. Results: The clinical characteristics of the two cohorts were similar. The Adelaide cohort had a higher initial energy intake in the first 5 days compared with the Singapore cohort, and a significantly greater weekly increase of 21.0 kcal/week (95% CI 7.7–34.3; P = 0.002). The Adelaide cohort also had a higher initial protein intake and a significantly greater weekly increase of 0.88 g/week (95% CI 0.5, 1.3), P < 0.001) compared with the Singapore cohort. The weight gain of the Adelaide cohort was 9 g/day more than the Singapore cohort (95% CI 7.3, 10.7; P < 0.001). Post‐natal growth failure was evident in 32% (n = 64) of the Adelaide cohort and 64% (n = 94) of the Singapore cohort. Conclusions: The two centres showed distinct differences in nutritional management. A higher energy and protein intake was associated with improved growth yet growth in both cohorts was still below current recommendations.     

Reliability of the Norwegian Version of the Children's Assessment of Participation and Enjoyment (CAPE) and Preferences for Activities of Children (PAC)

ABSTRACT

This study examined test–retest reliability of the Norwegian version of Children's Assessment of Participation and Enjoyment (CAPE), and Preferences for Activities of Children (PAC) in children with and without disabilities. Totally 141 children, 107 typically developing, mean age 11.1, and 34 with disabilities, mean age 14.2 years participated. A cross-sectional, test–retest design was applied. The participants completed CAPE and PAC twice within mean 19 days. Reliability was examined by Chronbach's alpha, intraclass correlation coefficients (ICC), and Kappa statistics. The alpha values for internal consistency varied between 0.53 and 0.87 for the CAPE and between 0.75 and 0.93 for the PAC. ICC coefficients varied from 0.49 to 0.83 for the CAPE and 0.50 to 0.85 for the PAC. Kappa coefficients varied from 0.30 to 0.66. The Norwegian CAPE and PAC demonstrated sufficient measurement properties of internal consistency and test–retest reliability. The reliability of the CAPE, however, was not entirely satisfactory.     

Maternal and child health in Indonesia

AIIKU-HAN activity was initiated by the Imperial Gift Foundation BOSHI-AIIKU-KAI in Japan in 1936 and has been introduced and accepted to community-based maternal and child health development in Indonesia through the international cooperation project conducted by Japan International Cooperation Agency (JICA) in order to encourage community health activities carried out by Indonesian women volunteers. As AIIKU-HAN activity in Japan and Dasa Wisma health activity in Indonesia have much in common, transferring concepts, methods and experiences met less obstacles when Indonesian community health volunteers learned AIIKU-HAN activity. Experience gained while developed countries were less developed can be transferred by shifting the time-scale of history in developed countries.     

Second malignant tumours in childhood Hodgkin's disease

This study was undertaken to determine the treatment-specific incidence of second malignant tumours (SMT) in childhood Hodgkin's disease. The institutional databases at The Hospital for Sick Children, the Princess Margaret Hospital, and the Toronto-Bayview Regional Cancer Centre were reviewed for the years 1958–1993. Three hundred and forty-three consecutive newly diagnosed children were evaluated. The overall 30 year cumulative SMT incidence was 31%. The 20 year SMT incidence was greater for patients who relapsed (n = 129), 27%, compared with patients who remained relapse free (n = 214), 13%. For patients with stage 1–3B disease who remained relapse free, the 10 year SMT rate was 7% for patients who were surgically staged and treated with extended field radiation treatment (EF RT) (35 G), compared with 3% in clinically staged patients treated with MOPP (six cycles) and EF RT (25–30 G). To date there is no significant difference in the oncogenicity of these treatment protocols. However, EF RT alone was less effective in disease control. For stages 1–3B, 62% of patients relapsed after EF RT alone compared with 18% after bimodal treatment. Therefore treatment intensification due to relapse was more frequent in the former group. The overall 10 year SMT incidence for patients treated with these protocols was 11% and 3%, respectively. The 20 year SMT incidence following EF RT alone was 24%. We conclude that SMTs were a common late complication in childhood Hodgkin's disease and are a limiting factor in the achievement of cure. The incidence of SMTs was increased in children who required retreatment and was minimal in children who remained in a first complete remission. Therefore the initial treatment strategy in childhood Hodgkin's disease must be to minimize the risk of relapse, in order to avoid the morbidity and mortality associated with both relapse and SMT induction, and to achieve this objective with a primary treatment protocol of low oncogenicity. © 1996 Wiley-Liss, Inc.