Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 14-month-old baby weighing 4300 g was a giant infant with macroglossia. Exomphalos was not present, but diastasis recti abdominis was observed. The patient was therefore diagnosed as having Beckwith-Wiedemann syndrome (EMG syndrome). Other characteristic signs such as neonatal hypoglycemia, hemihypertrophy, and a small ventricular septal defect were also recognized, but nephromegaly or hepatomegaly was not present. Tongue reduction by wedge resection was performed under general anesthesia. Some of the problems associated with anesthetic management in this syndrome are hypoglycemia, airway obstruction and cardiovascular status. After induction with increasing concentration of halothane (0.5-4.0%) and 66% nitrous oxide in oxygen, a nasotracheal tube was inserted. Endotracheal intubation was easy without using a neuromuscular blocking agent. Anesthetic maintenance was accomplished with nitrous oxide 66% in oxygen and halothane 0.5-1.0% and no neuromuscular blocking agent was used. The plasma glucose level was kept within normal ranges during and after the operation by infusion of acetate Ringer's solution with 5% glucose. The postoperative progress was uneventful.
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PMID:[Anesthetic management for partial tongue resection in a patient with Beckwith-Wiedemann syndrome]. 160 68

A female infant is described with a complete ectopia cordis and a single atrium who presented four hours after birth. There was complete deficiency of the sternum with the absence of pericardium over the heart. There was an associated omphalocele containing an enlarged liver. The infant died 45 hours after birth following an attempt to provide tissue covering. Additional intracardiac anomalies included a ventricular septal defect overriding aorta and total anomalous venous drainage.
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PMID:Ectopia cordis in a Nigerian child. 819 68

A 7-year-old boy was admitted for RVOT restenosis. The patient was born following an unremarkable pregnancy and delivery. He was carried out Jatene's procedure for TGA at 23rd day after birth. At 19 month of age, he was removed pulmonary stenosis using patch angioplasty with mono cusp. When he was 6 year of age, the onset of edem, ascites and hepatomegaly were pointed out. At 7 year of age, he was admitted for edema, hypoproteinemia and protein loosing enteropatchy. After he was treated medical therapy, cardiac catheterization showed markedly right atrial and right ventricular hypertension and ventricular septal defect (VSD). Cine angiography suggested the right ventricular outflow tract stenosis, pulmonary valve and tricuspid valve insufficiency. On August 1, 1996, the patient underwent surgical repair: direct closure of VSD, aortic homograft (20mm) replacement for RVOT stenosis and tricuspid valve annuloplasty by DeVega's procedure. Postoperative course was uneventful. At 23rd postoperative day, cardiac catheterization and cine angiography showed no problems. He was not treated with anticoagulation.
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PMID:[A case report: right ventricular outflow tract (RVOT) reconstruction using homograft for RVOT restenosis after Jatene's procedure of transposition of the great arteries (TGA)]. 952 29

A 3.4-kg, 6-day-old infant presented to the pediatric intensive care unit with a 2-day history of poor feeding and tachypnea. Care at an outside hospital included endotracheal intubation, the administration of isotonic fluid (20 mL/kg), and antibiotics (ampicillin and gentamicin) for presumed sepsis. After arrival at our institution, physical examination revealed absent femoral pulses and hepatomegaly. Cerebral oximetry revealed a right-sided reading of 51% and a left-sided reading of 15%. Given the diminished femoral pulses and the disparity in the cerebral oximetry values, a tentative diagnosis of congenital heart disease with an obstructive left-sided lesion was entertained, and a prostaglandin E1 infusion was started at 0.05 microg/kg/min. The diagnosis of a type C interrupted aortic arch and a ventricular septal defect was confirmed by echocardiography. After stabilization and correction of metabolic abnormalities, the infant was taken to the operating room for repair of the interrupted aortic arch and placement of a pulmonary artery band.
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PMID:Cerebral oximetry using near-infrared spectroscopy aids in the diagnosis of interrupted aortic arch. 1870 27

A 6-year-old male India-origin Rhesus macaque (Macaca mulatta) presented with thin body condition and muscular atrophy. Thoracic auscultation revealed a grade VI/VI pansystolic murmur bilaterally. Radiographs showed cardiomegaly with significant left atrial and biventricular enlargement, a dilated pulmonary artery, and hepatomegaly. Electrocardiogram revealed a normal sinus rhythm interspersed with ventricular bigeminy. Hematology showed mild polycythemia and prerenal azotemia. Necropsy demonstrated double-outlet right ventricle with a large subaortic ventricular septal defect, subpulmonary stenosis, small atrial septal defect, and right ventricular hypertrophy. The major histological finding was severe chronic passive hepatic congestion. Double-outlet right ventricle is a rare congenital heart disease, both in human beings and animals.
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PMID:Double-outlet right ventricle and double septal defects in a Rhesus macaque (Macaca mulatta). 2236 52