Giant exomphalos--conservative or operative treatment?

The rate of survival for infants with intact giant exomphatos has much improved during the last 20 years; this is partly due to better respiratory and nutritional support. The use of a staged operative closure using a sialon prosthesis has been advocated for 12 years, but our data do not show this to be superior to nonoperative management.     

Cell death in normal and abnormal development

Research over the past 50 years has consistently documented that cell death is an integral part of both normal development and the etiology of birth defects; however, the significance of this cell death has been, until recently, unclear. Research published during the past 15 years has now shown that programmed cell death (PCD) and teratogen-induced cell death are genetically controlled processes (apoptosis) that play important roles in both normal and abnormal development. Therefore, the purpose of this review is to highlight what is known about PCD and teratogen-induced cell death and their relationships to the mechanisms of apoptosis and abnormal development.     

Paralytic poliomyelitis 1976-1988: report from a Sentinel Centre

Kalawati Saran Children's Hospital, New Delhi, is the Sentinel Centre for poliomyelitis in the Northern region and 52-76% of cases of acute poliomyelitis from the Union Territory of Delhi are registered here. In this paper, data from this hospital for the last 13 years is presented. A detailed analysis of 1874 cases seen during the year 1987 is also presented. Inspite of large scale vaccination being carried out, the total number of cases have not shown a corresponding decline. The incidence of acute polio in Delhi has not declined over the years: it was 14.7/100,000 population in 1976 and 15.25 100,000 in 1988. There has been no change in the epidemiology of the disease over the years, except that the disease among the fully vaccinated children has increased from 1.3% in 1979 to 13.9% in 1988. Inspite of a decade of use of the oral polio vaccine there has been no significant reduction in the incidence of the disease, and urgent alternative strategies are needed.     

Late recurrence in primary region of parosteal osteosarcoma: a case report

Recurrence in patients with osteosarcoma after 5 years is a rare condition and has been infrequently reported. Here, the authors present a case of conventional parosteal osteosarcoma that had relapsed 17 years after diagnosis. To their knowledge, this is one of the latest recurrence times in the primary region of osteosarcoma.     

Changing epidemiology of Reye syndrome in the United States

The average annual incidence of Reye syndrome reported through national surveillance was lower during 1981 to 1984 than during the previous five surveillance years. This is accounted for by a decrease in cases among children younger than 10 years of age; the number of cases in 10- to 19-year-old persons remained relatively stable during this period. The overall decline in incidence and the differing age-specific incidence trends are apparent for both varicella-associated cases and for nonvaricella-associated cases. During 1985, the incidence has been much lower than during any previous year since surveillance was initiated; this most recent decrease includes children 10 to 19 years of age. Independently conducted surveys suggest that the prevalence of salicylate use for viral illnesses has decreased among children in recent years, particularly among children younger than 10 years of age. The changing epidemiology of Reye syndrome may reflect, in part, the declining use of salicylates among children and teenagers in the United States.     

Precocious puberty associated with Silver's syndrome

Precocious puberty has been reported as an important feature of the Silver-Russell syndrome. The present case refers to a boy entered puberty at the chronological age of 11 years and the bone age of 6 years 6/12. Criteria for precocious puberty in this syndrome are discussed by comparison with previously published cases, and it is concluded that abnormalities in clinical pubertal development are quite unusual in this syndrome.     

Mesenchymal hamartoma of the liver demonstrating peripheral calcification in a 12-year-old boy

Mesenchymal hamartoma of the liver is a rare benign hepatic tumor that typically presents in the first 2 years of life. Approximately 85% of affected children present before the age of 3 years and less than 5% present after the age of 5 years. Although spontaneous regression has been reported in this benign tumor, most mesenchymal hamartomas gradually increase in size and can reach enormous proportions. We present an atypical case of mesenchymal hamartoma in a 12-year-old boy. The boy's age and presence of calcifications on CT demonstrate a unique presentation of this rare entity.     

Gastric stromal tumors in children

Gastrointestinal stromal tumors (GIST) in children are rare and their behavior has been regarded as difficult to predict on pathological criteria. We report our experience with two gastric GISTs in children aged 10 and 11 years. Both remain alive and free of disease at 5 years and 2 years respectively. Comparison of the pathological features in the resected specimens with a recently proposed guidelines for predicting outcome in this group of tumors is reported.     

Type 1 diabetes: a disease of developmental origins

The incidence of type 1 diabetes globally has increased dramatically over the last 50 years. Proposed environmental reasons for this increase mirror the modern lifestyle. Type 1 diabetes can be viewed as part of the non‐ communicable disease epidemic in our modern society. Meanwhile rapidly evolving new technologies are advancing our understanding of how human microbial communities interface with the immune system and metabolism, and how the modern pro‐inflammatory environment is changing these communities and contributing to the rapid rise of non‐communicable disease. The majority of children who present with clinical type 1 diabetes are of school age; however 80% of children who develop type 1 diabetes by 18 years of age will have detectable islet autoantibodies by 3 years of age. The evolving concept that type 1 diabetes in many children has developmental origins has directed research questions in search of prevention back to pregnancy and early life. To this end the world's first pregnancy to early childhood cohort study in at‐risk children has commenced.     

Pinching, electrocution, ravens' beaks, and positive pressure ventilation: a brief history of neonatal resuscitation

Since ancient times many different methods have been used to revive newborns. Although subject to the vagaries of fashion for 2000 years, artificial respiration has been accepted as the mainstay of neonatal resuscitation for about the last 40. Formal teaching programmes have evolved over the last 20 years. The last 10 years have seen international collaboration, which has resulted in careful evaluation of the available evidence and publication of recommendations for clinical practice. There is, however, little evidence to support current recommendations, which are largely based on expert opinion. The challenge for neonatologists today is to gather robust evidence to support or refute these recommendations, thereby refining this common and important intervention.     

A boy with psychosocial short stature followed up from infancy to adulthood

A boy with psychosocial short stature who has been followed up from the age of 11 months to adulthood is described. The boy was the product of an unwanted pregnancy. The emaciated short boy gained weight and height markedly during a short-term stay at hospital, but lost weight and experienced minimal height gain at home. On the fourth hospital admission at the age of 6 years 3 months the boy weighed 10 kg and measured 85.7 cm, he was malnourished and exhibited strange behavior and had a voracious appetite. He was examined endocrinologically and provocative tests performed early after admission showed insufficient growth hormone secretion, although this recovered later at a time of catch-up growth. The boy was reared in an orphanage from the age of 6 years 5 months until the age of 15 years 3 months. His growth rapidly caught up to a normal rate, his abnormal behavior disappeared, and he demonstrated an increased IQ. He attained 169.5 cm at the age of 17.5 years and possessed normal secondary sexual characteristics. After graduating from senior high school the patient has been living happily by himself without intervention from his mother, and is working in a Chinese restaurant. The impaired relationship between the boy and mother has never been restored. The record of growth and development described in this case is the longest ever reported.     

Cancer in older adolescents and young adults: epidemiology, diagnosis, treatment, survival, and importance of clinical trials

Cancer in adolescents and young adults has unique features in addition to the special medical, physical, psychological, and social needs of patients in this age group. The spectrum of malignant diseases is different from that in any other period in life, and it is strikingly different from the pattern in older persons. More people 15-25 years of age are diagnosed to have cancer than during the first 15 years of life. During the last 25 years, the incidence of cancer in this age range has increased faster while the increase in their cancer survival rates has been significantly lower than in younger or older patients, especially in comparison to results of the national pediatric cooperative cancer groups. Thus the 5-year outcome in 15-19 year olds with leukemias and sarcomas is not only worse than in younger patients, but also lower in this population at large than in patients of the same age treated at Children's Cancer Group institutions. In the United States, only approximately 5% of 15-25 year olds with cancer are entered onto clinical trials, in contrast to 60-65% of younger patients. Thus, cancer during adolescence and early adult life is an underestimated challenge that merits specific resources, solutions, and an international focus.     

Commentary

Cancer in adolescents and young adults has unique features in addition to the special medical, physical, psychological, and social needs of patients in this age group. The spectrum of malignant diseases is different from that in any other period in life, and it is strikingly different from the pattern in older persons. More people 15–25 years of age are diagnosed to have cancer than during the first 15 years of life. During the last 25 years, the incidence of cancer in this age range has increased faster while the increase in their cancer survival rates has been significantly lower than in younger or older patients, especially in comparison to results of the national pediatric cooperative cancer groups. Thus the 5‐year outcome in 15–19 year olds with leukemias and sarcomas is not only worse than in younger patients, but also lower in this population at large than in patients of the same age treated at Children's Cancer Group institutions. In the United States, only ～5% of 15–25 year olds with cancer are entered onto clinical trials, in contrast to 60–65% of younger patients. Thus, cancer during adolescence and early adult life is an underestimated challenge that merits specific resources, solutions, and an international focus. Med Pediatr Oncol 2002;38:1–10. © 2002 Wiley‐Liss, Inc.     

Natural history of intrauterine growth retardation: pubertal growth and adult height

The evolution of height and bone age up to complete or near complete achievement of growth has been followed in 13 children born at term with a length of 42 to 46 cm, who after age 2 years had still a growth delay of -2.1 to -4.9 SD. Their mean annual growth velocity has been slightly below the average, excepted in the years preceding and following immediately the onset of puberty. The bone age, largely delayed and close to height age up to approximatively 8 years, afterwards has increased more than growth, so that the final height has always been less than the adult height predicted at age 8 years. The main factor in this difference between final and predicted height has been the fact that puberty has not been delayed. Starting at the usual age, in children whose height deficiency was still important, the pubertal growth spurt has not allowed full catch-up. Thus, in spite of delayed bone age during childhood, the mean adult height in the 13 patients of this series has been -3.43 SD versus -3.16 SD at age 2-5 years, these mean values involving different individual growth curves with absolute deterioration in 6 only of the 13 cases. These data will have to be considered when discussing the final results of therapeutic trials in children with severe and persisting intra-uterine growth retardation.     

Role of haematological,pulmonary and renal complications in the long-term prognosis of patients with lysinuric protein intolerance

Three patients with lysinuric protein intolerance are reported. The first patient displayed severe haemolytic anaemia, bone marrow erythroblastophagocytosis, renal tubular disease and interstitial lung disease. Despite treatment with citrulline and low-protein diet, this child died at the age of 18 months. The second patient is now 24 years old and has chronic interstitial lung disease and focal renal glomerulosclerosis. The third patient, now 5 years old, has severe chronic interstitial lung disease. A 6-month treatment with prednisone was ineffective in the second and third patients.     

Boxing, youth and children

Boxing remains a popular spectator sport, success in which is achieved by the promotion of boxing in the childhood years. Despite the failure to ban boxing generally, there remains the realistic expectation that organised boxing by children below the age of consent (appropriately 16 years) is still achievable. There are two reasons for banning children under the age of 16 years from boxing. The first is that children have little awareness of risk, specifically the risk of chronic encephalopathy, which develops only after a lag period measured in decades or more. The second is that there is no place in contemporary society for a youth sport which has, as its primary goal, the infliction of acute brain damage on an opponent. This paper analyses the medical and ethico-social issues implicit in this subject. Boxing, in historical perspective, has been an altruistic amateur sport for boys and male youths, and has a proud tradition. But in the context of social evolution, and in current perspective to prevent exploitation of underprivileged youths, the time has come for an absolute ban on underage boxing. Such is achievable even if adult boxing remains an Olympic and television sport for some time to come.     

Olfactory Neuroblastoma (Esthesioneuroblastoma) in a 2-Year-Old Boy

A 2-year-old boy presented with sudden blindness and epistaxis resulting from olfactory neuroblastoma with intracranial extension. This disease is uncommon in children, with only 27 cases reported in patients less than 17 years of age. This article reviews the clinical and histological features of this tumor and discusses treatment. Therapy has generally consisted of surgery and radiation. Our child and 2 others treated with radiation and combination chemotherapy (including cyclophosphamide and anthracyclines) have done well with follow-ups of up to 9 1/2 years. Chemotherapy is an important form of adjuvant therapy in this disease.     

Prävention von Pneumokokkeninfektionen

Pneumococcal infections are associated with high morbidity in the first years of life.For several decades, only polysaccharide vaccines that were effective in individuals 2 years of age or older had been available.A new pneumococcal vaccine in which polysaccharide antigens are bound to carrier proteins has been developed, and this vaccine is efficacious in children younger than 2 years of age.Currently, this vaccine is recommended in Germany for certain patients at high risk of systemic pneumococcal infections.Only universal use of the vaccine would influence the epidemiology of those infections.The positive experience with general use of the vaccine in the USA is promising and could support its extended use in Germany soon. As a prerequisite, a broad consensus is required because resulting costs and ethical aspects are still a matter of intense and controversial discussions.     

Is Perceptual Motor Dysfunction a Problem in Anorexia Nervosa?

In recent years much research has been conducted with anorexia nervosa patients. Perceptual-motor performance has been one major area of concern. In this paper the literature on perceptual dysfunction and increased motor activity in anorexia nervosa is reviewed and areas of needed research by therapists are suggested.