Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adenocarcinoma arising in Barrett's esophagus has recently been described in two children aged 11 and 14 years. The long-term follow-up of Barrett's esophagus in children is not well described. We evaluated 16 cases of Barrett's esophagus in children treated at this institution during the last 16 years. Ages ranged from 1.2 to 16 years (mean, 10.3 years). There were 11 boys and 5 girls. Barrett's esophagus was documented by endoscopy in 14 instances and at autopsy in 2 patients with secretory diarrhea and tetralogy of Fallot who died of sepsis. Two children had cancer (neuroblastoma, leukemia) and died of their malignant disease. Five patients had cerebral palsy, 1 esophageal atresia, 1 Fanconi's anemia, and 5 were otherwise normal children. Six were treated medically. Eight patients underwent Nissen fundoplication for complications of gastroesophageal reflux (GER). Five patients were available for follow-up endoscopy (mean, 2 years; range, 1.1 to 5.4 years). Endoscopy was performed on a yearly basis, obtaining biopsy specimens from multiple levels of the esophagus. Four children had satisfactory clinical response to an antireflux procedure including the resolution of a stricture in one case. However, in all 5 cases persistent metaplastic epithelium was documented and showed no evidence of regression. Although there has been speculation that Barrett's esophagus in children may be more likely to revert to normal squamous epithelium than in the adult, there has been only one case of regression in 180 cases of Barrett's esophagus occurring in children described in 37 reports in the literature.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Persistence of Barrett's esophagus in children after antireflux surgery: influence on follow-up care. 156 27

To document the incidence and severity of symptomatic hepatomegaly in patients with Stage IV-S neuroblastoma, we reviewed the charts of children with neuroblastoma seen at Children's Hospital of Pittsburgh between 1951 and 1985. Sixteen met the criteria for IV-S disease, and 11 of these (69%) had massive hepatomegaly. Five children had symptoms referable to their liver size including respiratory distress, gastroesophageal reflux, or decreased urine output. Liver function appeared to be normal or only mildly abnormal in the 9 patients where data were available. Because therapy was so variable, it was not possible to correlate treatment regimen with outcome. However, 3 symptomatic patients who received less than or equal to 600 rad without chemotherapy had prompt subjective responses. Follow-up was available on 10 children 6 months-18 years (median 18 years) from diagnosis. Eight were alive with resolved or resolving hepatomegaly. High dose (greater than or equal to 3,300 rad) radiation-related side effects included multiple rib chondromas, chest- and pelvic-wall hypoplasia in one patient, and radiation nephritis with hepatic fibrosis resulting in death of a second patient. Our results support prior recommendations that for symptomatic hepatomegaly, low doses of radiation be considered.
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PMID:Results of therapy in stage IV-S neuroblastoma with massive hepatomegaly. 362 25

The term "Western diseases" refers to those conditions that are rare or absent in underdeveloped areas of the Third World and increase in frequency with adoptions of Western customs. In adults, they include such common conditions as coronary artery disease, essential hypertension, appendicitis, cholesterol gall stones, and colon cancer. The best examples of Western diseases in the pediatric population are asthma, allergies, appendicitis, and inflammatory bowel disease. Limited data from sub-Saharan Africa suggest other pediatric surgical conditions may fall into this category, including hypertrophic pyloric stenosis, gastroesophageal reflux, perirectal abscess, anal fissure, gastroschesis, and neuroblastoma. Existing theories for the origins of Western diseases have postulated a role for decreased dietary fiber, improved hygiene, fetal programming, and a protective effect of tropical enteropathy. How these factors might relate to the rise of appendicitis, inflammatory bowel disease, and possibly other common pediatric surgical diseases in industrialized societies remains poorly understood. Further research is needed to better define geographical differences in common pediatric surgical conditions and to investigate how genetic and environmental factors interact to modify risk of disease. Understanding the molecular mechanisms that give rise to Western diseases could lead to new therapeutic and prevention strategies for some of the most common pediatric surgical conditions in industrialized countries.
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PMID:Western diseases: current concepts and implications for pediatric surgery research and practice. 1808 4

Chronic abdominal pain is a very common complaint in the population of children and adolescents. In most cases, the usual cause are functional gastrointestinal disorders. However, in a few percent of children, the reason for persistent chronic stomach pain are organic diseases occurring in the gastrointestinal tract, as well as parenteral diseases, including uro-genital tract abnormalities, inflammation of the lower respiratory tract and cancer processes. Among organic causes, in addition to those commonly encountered, such as: intolerances and food allergies, gastroesophageal reflux disease, chronic gastritis or duodenitis, or urinary tract infections, the diagnosis should also include very rare causes, for example, neoplastic diseases, among them tumors of the abdominal cavity. In the case described in the present article, a 6-year-old girl with chronic abdominal pain, symptoms of gastro-oesophageal reflux and constipation, and previously diagnosed food allergy and lactose intolerance, was referred for widening the diagnosics due to the occurrence of alarm symptoms. The nodule revealed in the chest X-ray, in CT scan, turned out to be a paravertebral tumor with the specific features of neuroblastoma. After a macroscopically complete tumor resection based on the result of histopathological examination, the diagnosis of ganglineuroblastoma was established. The presence of alarm symptoms in anamnesis and physical examination in children with abdominal pain suggests a higher probability of the organic origin of the disease and should always lead to extended diagnostics. Ganglioneuroblastoma is a very rare disease, in most cases is located primarily in the abdominal cavity, and the most common associated symptom is abdominal pain.
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PMID:[Ganglioneuroblastoma in a child with chronic abdominal pain - a case report]. 3063 34