Management of colorectal carcinoma in children and young adults

Colorectal Carcinoma (CRC) is rare in patients less than 20 years of age. Although presenting symptoms are similar to adults, this diagnosis is rarely considered in the initial differential diagnosis of young patients. We will review what is published about the incidence, epidemiology, and clinical presentation of CRC in children, adolescents and young adults. Because of its rarity in this age group, few pediatric oncologists will have experience with CRC, and clinical trials will rarely be available. The treatment of CRC in adults is evolving rapidly and consultation with medical oncologists experience in treating adults with CRC is essential to develop the best treatment plan for a young patient diagnosed with CRC.     

Teratomas in children and young adults.

A review of all teratomas seen at the University of Wisconsin Hospital between 1965 and 1977 revealed that sacrococcygeal and presacral teratomas were most common. In these cases survival was best in infants less than one year old, with the exception of two cases of malignant medulloepithelioma. Testicular teratomas were predominant in the young adult male, and survival was poor. Pathology and treatment of teratomas are discussed, with an accompanying discussion of congenital anomalies associated with teratomas.     

Equestrian injuries in children and young adults.

We reviewed the English language scientific literature about equestrian injuries among children and young adults. All studies showed that more females than males were injured, with falls from horses being the most common cause of injury. Fractures were common, and head injuries were associated with the vast majority of deaths (72% to 78%) and hospitalizations (55% to 100%). Although the overall injury rate was low, equestrian athletes are at risk for serious injuries. Pediatricians should know the medical contraindications for participation in equestrian sports and encourage riders to obtain horse safety training and use protective headgear (helmets) approved by the American Society for Testing Materials when riding or working around horses. Pediatricians can play an active role in increasing public awareness of equestrian injuries and in reducing risk of injury.     

WT1 peptide immunotherapy for cancer in children and young adults

Wilms tumor gene (WT1) can be overexpressed in childhood cancers. We evaluated the efficacy of WT1 vaccination for five children with solid cancer or acute leukemia. WT1 vaccine was administered to HLA‐A*2402‐positive patients with WT1‐overexpressing residual tumor despite prior conventional treatment. One patient showed complete response and one patient showed stable disease according to the Response Evaluation Criteria in Solid Tumors; the remaining three showed progressive disease. Treatment‐related adverse effects were limited to local injection site erythema. These results suggest that WT1 vaccination has therapeutic potential, but any beneficial effect may be insufficient in the presence of gross residual disease. Pediatr Blood Cancer. 2010;55:352–355. © 2010 Wiley–Liss, Inc.     

Family history of cancer among children with brain tumors: a critical review

The occurrence of brain tumors in children has been anecdotally associated with an increased cancer incidence among relatives. This study rigorously reviewed the epidemiologic literature regarding family history of cancer in children with brain tumors. Six case-control and 10 cohort studies remained after applying stringent inclusion criteria. Most studies found no significant increase in cancer risk among relatives of childhood brain tumor patients. Those associations that were detected were often of borderline significance or demonstrated wide confidence intervals. There is limited evidence that a family history of cancer is more common among families of childhood brain tumor patients.     

Teratomas in children and young adults

A review of all teratomas seen at the University of Wisconsin Hospital between 1965 and 1977 revealed that sacrococcygeal and presacral teratomas were most common. In these cases survival was best in infants less than one year old, with the exception of two cases of malignant medulloepithelioma. Testicular teratomas were predominant in the young adult male, and survival was poor. Pathology and treatment of teratomas are discussed, with an accompanying discussion of congenital anomalies associated with teratomas.     

Certified cause of death in children and young adults with cerebral palsy.

The status of 732 children suffering from cerebral palsy from the South East Thames region (births from 1970-9) was ascertained at the end of 1989, and copies of death certificates of the 73 children who have died, aged 4 weeks to nearly 16 years, were obtained. Infantile cerebral palsy (ICD Code 343-) was coded as the underlying cause of death in only 16 (22%) cases. On 28 (38%) certificates there was no mention of any form of cerebral palsy, the proportion in which it was not mentioned increasing with age. In 20 (28%) cases the coded underlying cause of death was respiratory, hence in published national statistics the number of deaths from respiratory causes is inflated. A postmortem examination was known to have been performed in 23 cases, but the recorded information was in some cases limited to a ''terminal event''. The importance of good data on the death certificate, and the significance of published national statistics, need to be communicated to all those involved in the certification process if cerebral palsy and other chronic conditions, which raise the relative risk of death, are not to be under-represented.     

Family stress and BMI in young children

Aim: The aim of this study was to investigate if family stress and parental attachment style are associated with body mass index (BMI) in young children, and identify possible explanations. Methods: A cross‐sectional survey with a two‐stage design was used. Parents of 873 children participated. They completed a demographic questionnaire, the Swedish Parenthood Stress Questionnaire (SPSQ), the Relationship Questionnaire (RQ) and reported their children’s television‐viewing habits (as a marker of physical activity). Children’s height, weight and BMI were obtained from a general population‐based register, BASTA. Associations with over‐ and underweight in children were assessed using multiple logistic regression analysis. Results: Family stress indicated by SPSQ‐score was associated with suboptimal BMI. Maternal, but not paternal, SPSQ‐stress score was statistically significantly associated with overweight and underweight, with adjusted odds ratios (and 95% confidence interval) of 4.61 (3.11–6.84; p < 0.001) and 3.08 (1.64–5.81; p < 0.001) respectively. Associations between childhood BMI and parental attachment style were identified, but were not independent of maternal SPSQ‐score. Conclusion: Our findings support a role for family stress in development of both overweight and underweight among young children. This is likely to be attributed to behavioural mechanisms but a more direct metabolic influence of stress could also be involved.     

Sudden death in children and young adults

Congenital cardiac lesions, whether unrepaired or postoperative, are among the most common diagnoses associated with sudden death in the 1- to 20-year-old range. In the general population, myocarditis, hypertrophic cardiomyopathy, and coronary artery anomalies are common causes of sudden cardiac death. Within the subset of pediatric cardiology patients who have not undergone operation, patients at greatest risk of sudden death are those with hypertrophic cardiomyopathy, Eisenmenger syndrome, and tetralogy of Fallot.     

Thyrotropin-receptor autoantibodies in children and young adults with Graves' disease

Thyrotropin (TSH)-receptor autoantibodies were assessed by measurement of 125I-bTSH binding inhibition in 32 patients with juvenile-onset Graves' disease (one of whom was studied twice) and 16 normal control subjects. Thirteen (76.5%) of 17 thyrotoxic patients had significantly elevated TSH binding-inhibitory immunoglobulin (TBII) activity compared with eight (50%) of 16 patients who were in clinical remission and none of the control subjects. Mean TBII activity was significantly greater in thyrotoxic patients than in individuals in remission, except in one unusual patient in whom there was a discordance between TBII activity and in vitro thyroid-stimulatory activity. In eight euthyroid patients who were followed up for at least five months while not receiving treatment, assessment of TBII activity did not predict who would or would not suffer relapse at a later date. Thus, TBIIs are secreted in excess in juvenile Graves' disease, the titer decreasing as the disease remits. The TBII assay cannot be used as the sole predictor of when antithyroid medication can be withdrawn safely, however.     

Cardiac pacing in children and young adults

Pacemakers are being used with increasing frequency in children. New pacemakers are smaller, more sophisticated, can be programmed, have output telemetry, are more reliable, and are longer lasting. Implant techniques have been modified, so that they are less traumatic to the patient and result in better electrical measurements. Transvenous implants seem to be the preferred method in children who weigh 13.5 kg or more. Physiologic dual-chamber pacemakers make the patients feel better. The time between hospitalizations for pacemaker replacements or revisions has increased from 14.7 months in 1974 to 22 months in this study.     

Reduced hemoglobin values in children and young adults with hemophilia

Mild leukopenia and thrombocytopenia are common in multitransfused hemophiliacs. Because little attention has previously been directed to measurements of erythropoiesis in these patients, we prospectively examined hemoglobin concentration and RBC indices in 94 children and young adults with hemophilia during comprehensive clinic visits. Additional studies performed in many included serum transferrin saturation, ferritin, haptoglobin, and free erythrocyte protoporphyrin measurements. Hemoglobin concentrations were recorded as age-related percentile values. Hemophiliacs of all ages and degrees of severity often had lower than average values for hemoglobin; 31% had values less than the third percentile, 46% less than the tenth percentile, and 83% less than the mean value. Reduced hemoglobin percentile values were unrelated to age, severity of disease, or human immunodeficiency virus antibody status. Only five patients had an obvious cause for anemia. Serum ferritin, transferrin saturation, and erythrocyte protoporphyrin values were usually normal, indicating that iron deficiency and anemia of chronic disease were uncommon. Although serum haptoglobin was reduced in 44% of the patients, reticulocyte count was infrequently increased. We conclude that hemoglobin values are frequently less than the mean normal values for age in hemophiliacs. Although frank anemia is common, it is usually mild and without obvious cause. Hemophiliac individuals with slightly reduced hemoglobin values probably do not routinely require detailed investigation for occult blood loss, iron deficiency, or inflammation.     

Management of pancreatoblastoma in children and young adults

Pancreatoblastoma is a very rare childhood tumor originating from the epithelial exocrine cells of the pancreas. It is the most common malignant pancreatic tumor in young children and has a mean age of diagnosis of 5 years. It is slow growing and its presentation is varied and often non-specific. Tumors tend to be quite large and appropriate cross sectional imaging is very important to assess for extent, metastatic disease, and resectability. Biopsy for tissue diagnosis is essential. Complete surgical resection is the goal of therapy although many patients are unresectable at initial diagnosis and require neoadjuvant chemotherapy. Adjuvant chemotherapy is also recommended and chemotherapeutic regimens involve cisplatin and doxorubicin. Even with curative resections, these lesions have a high recurrence rate and patients must be followed closely. Knowledge of this rare tumor is important for the clinician confronted with a large retroperitoneal mass in a young child.     

Management of melanomas in children and young adults

Melanoma is rare in children and young adults. The incidence is rising yearly in this population. The clinical features of the disease in the pediatric population have been well documented through single-institution experiences and population-based analyses. Still, our understanding of the etiologic factors in the majority of children remains unclear, and diagnosis of melanoma remains challenging in certain cases. Because of its rarity, the staging, management and treatment of melanoma in this population is adopted from adult guidelines. In this review, we provide information on the epidemiology, clinical presentation, staging, prognosis and management of melanoma in children and young adults.     

Adaptation of reaching movements in children and young adults with myelomeningocele

AIM: To investigate the ability to adapt reaching movements to new visuomotor gain conditions in subjects with myelomeningocele (MMC) and subjects without disabilities. In addition, differences in movement error within the MMC group were investigated. METHODS: Thirty subjects with myelomeningocele, 9-19 y, and 30 subjects without disabilities were included in the study. Data were collected with a digitizing tablet linked to a computer. The subjects made reaching movements towards three targets at different distances displayed on the computer screen, and movements were carried out under two different gain conditions-a base condition and an altered condition. Adaptation was quantified from the extent error and expressed in percentage of the target distances. For statistical analysis, ANOVA and t-tests were used. RESULTS: Under the base condition, no statistically significant difference between the groups was found, but after the first gain change, the MMC group evinced larger percentage error compared to the control group (p = 0.005). Analysis showed that the subjects in the MMC group did not fully adapt their movements under the altered condition after several trials of practice, but in the control group movements were rapidly adapted to the new gain. Subjects with early and severe symptoms of brainstem dysfunction showed a dramatic increase in error variability under the altered condition, while subjects who had shunt-treated hydrocephalus did not have larger percentage error compared to the others. CONCLUSION: Motor adaptation of reaching movements was impaired in subjects with MMC, but short-term motor learning did also occur in the MMC group.