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)

Phrenic nerve palsy (PNP) is seen in infants and young children usually resulting from operative trauma or birth injury. Spontaneous recovery usually occurs, but occasionally surgical plication is necessary. Twenty-three cases of PNP over a 10-year period were managed surgically. Patient ages ranged from 1 day to 30 months (median, 4 months), 18 were male and five female. Cause was operative trauma in 18 (17 cardiac surgery, one neuroblastoma), birth trauma in two, and idiopathic in three. The right side was involved in 14, the left in eight, and both in one. Indications for plication were inability to wean from the ventilator (group 1, 16 patients), recurrent pneumonia (group 2, four patients), and respiratory distress (group 3, three patients). The 16 patients in group 1 were intubated for a median of 18.5 days from onset of PNP to plication. Postoperatively, three had continuing congestive heart failure (one died at 16 days of age, one was still chronically ventilated at 22 months, one was extubated at nine days); the other 13 were extubated at a median of two days postoperatively. All the patients in groups 2 and 3 were extubated within two days of surgery. Twelve plications were transthoracic and 11 were transabdominal. Postoperative complications included pneumonia (2), wound infection (1), pneumothorax (2), and mucous plug with pulmonary collapse (1). One patient died of cardiac failure at 16 days. One patient in group 3 developed recurrent respiratory distress 4 months postoperatively; he had a recurrent elevated hemidiaphragm requiring a second plication.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Plication of the diaphragm for infants and young children with phrenic nerve palsy. 317 45

Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CPAM and intralobar sequestration (ILS) have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CPAM and ILS, it is best not to wait for complications to occur. We recommend surgery at 3 to 6 months of life at the latest, to allow compensatory lung growth to occur. At this age the postoperative course is usually smooth and long-term follow- up has shown normal respiratory function. The management of small non- communicating extralobar sequestrations is more controversial; it is known that these can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.
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PMID:The management of asymptomatic congenital lung malformations. 1498 Feb 88

Congenital lung malformations are often discovered incidentally on routine prenatal sonography or postnatal imaging. Lesions such as congenital cystic adenomatoid malformations (CCAM), sequestrations, bronchogenic cysts and congenital lobar emphysema may be asymptomatic at birth or at the time of discovery later in life. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases where CCAM and intralobar sequestration have remained asymptomatic throughout life; complications eventually develop in virtually all patients. The most common complication is pneumonia, which may respond poorly to medical treatment. Other complications include the development of malignancies (carcinomas and pleuropulmonary blastomas), pneumothorax and hemoptysis or hemothorax. Since lung resection will be required sooner or later for CCAM, intralobar sequestration and intrapulmonary bronchogenic cysts it is best not to wait for complications to occur. For patients diagnosed prenatally, we recommend surgery at 3 to 6 months of life at the latest, so that compensatory lung growth can occur. At this age the postoperative course is usually smooth and long-term follow-up has shown normal respiratory function. Mediastinal bronchogenic cysts also tend to become symptomatic and elective resection is recommended. On the other hand, asymptomatic congenital lobar emphysema may regress spontaneously and observation is warranted. The management of small noncommunicating extralobar sequestrations is more controversial; it is known that these lesions can remain asymptomatic throughout life but complications may develop and they are sometimes difficult to differentiate from neuroblastoma.
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PMID:Asymptomatic congenital lung malformations. 1577 May 85