Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We investigated the long-term pulmonary sequelae of 38 children surviving 3 to 11.5 years (median 7 years) after high-dose chemotherapy (HDC) and autologous bone marrow transplantation (ABMT) without TBI. This cross-sectional study included patients with neuroblastoma (21), non-Hodgkin's lymphoma (7), Ewing's sarcoma (5), rhabdomyosarcoma (3), medulloblastoma (1) and ALL (1). They were asked and examined for clinical signs and underwent a physical examination with chest X-ray; 33/38 had pulmonary function tests (PFT) performed. No obstructive disease was found. Fifteen out of 32 evaluable PFT (47%) were abnormal with a pulmonary restrictive syndrome in 10, and borderline values in five patients. Four of these 15 patients were symptomatic with exertional dyspnea and two of four had abnormal chest X-rays. The etiology was mainly multifactorial, associating HDC with thoracic radiotherapy +/- scoliosis/kyphosis +/- previous thoracotomy +/- post-ABMT interstitial pneumonitis. Only 3/10 patients with a restrictive syndrome had HDC containing BCNU or busulfan as the only risk factor for lung disease. We conclude that the prevalence of late pulmonary sequelae after ABMT without TBI is moderate and rarely due to HDC alone, since most abnormal PFT can be explained by heavy pretreatment prior to ABMT. As symptoms are scarce even in advanced disease, repeated testing and very long-term follow-up are needed.
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PMID:Long-term pulmonary sequelae after autologous bone marrow transplantation in children without total body irradiation. 875 Feb 68

This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature. Epidemiological studies have evidenced the high incidence of Graves' disease and Hashimoto's thyroiditis in patients with Down syndrome. Documentation of novel risk factors for celiac disease are of use to develop strategies for prevention in the population at-risk. Diagnostic criteria for non-celiac gluten sensitivity have been reported. Negative effect on nervous system development of the supernumerary X chromosome in Klinefelter syndrome has emerged. Improvements have been made in understanding rare diseases such as Rubinstein-Taybi syndrome. Eltrombopag is an effective therapy for immune trombocytopenia. Children with sickle-cell anemia are at risk for nocturnal enuresis. Invasive diseases caused by Streptococcus pyogenes are still common despite of vaccination. No difference in frequency of antibiotic prescriptions for acute otitis media between before the publication of the national guideline and after has been found. The importance of timing of iron administration in low birth weight infants, the effect of probiotics for preventing necrotising enterocolitis and perspectives for managing jaundice and cholestasis in neonates have been highlighted. New strategies have been developed to reduce the risk for relapse in nephrotic syndrome including prednisolone during upper respiratory infection. Insights into the pathophysiology of cerebral palsy, arterial ischemic stroke and acute encephalitis may drive advances in treatment. Recommendations on breastfeeding and complementary feeding have been updated. Novel treatments for rhabdomyosarcoma should be considered for paediatric patients. Control of risk factors for bronchiolitis and administration of pavilizumab for preventing respiratory syncytial virus infection may reduce hospitalization. Identification of risk factors for hospitalization in children with wheezing can improve the management of this disease. Deletions or mutations in genes encoding proteins for surfactant function may cause diffuse lung disease.
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PMID:Progress in pediatrics in 2015: choices in allergy, endocrinology, gastroenterology, genetics, haematology, infectious diseases, neonatology, nephrology, neurology, nutrition, oncology and pulmonology. 2756 21