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

Continuous supraventricular tachycardia was induced in 13 fetal sheep for 72 to 216 hours. The PaO2 decreased from 18.1 +/- 1.2 (SEM) to 15.4 +/- 0.9 mm Hg and the PaCO2 increased from 41.5 +/- 1.2 (SEM) to 46.0 +/- 1.0 (SEM) mm Hg with pacing. The hematocrit, total protein, albumin, serum [Na+] and [K+], and osmolality remained unchanged throughout the study. All study fetuses showed signs of ascites (mean = 88 +/- 67.5 [SD] ml), and one was grossly hydropic. Six fetuses, all of which had greater than or equal to 50 ml of ascites, died as the results of pacing. Gross pathologic findings in 13 fetuses included: cardiomegaly in seven, cyanotic myocardium in two, hepatomegaly in seven, pulmonary congestion in two, generalized edema in three, and massive edema (hydrops) in one. None of these conditions was found in the 14 control animals. There was no correlation of the severity of effects upon the fetus and the induced heart rate, the duration of tachycardia, or the site of implantation of the pacemaker. The conclusion was that organomegaly, generalized edema, and hydrops fetalis were the direct result of supraventricular tachycardia in utero; the exact mechanism of production and the reasons for the variable manifestations of tachycardia remain unclear.
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PMID:Supraventricular tachycardia with edema, ascites, and hydrops in fetal sheep. 706 22

Emergencies in pediatric cardiology are heart failure, cyanosis and rhythm disturbances. The signs of heart failure are tachycardia, tachypnea and hepatomegaly. The therapy consists of oxygen, diuretics and digoxin. Occasionally, intubation with mechanical ventilation and intravenous catecholamines are needed. Cyanosis is often the only sign of a severe heart malformation, and prompt hospitalization is mandatory. Oxygen and warm environment is important during transport, correction of a possible metabolic acidosis and prostaglandin infusion are done in the hospital. Beyond the newborn period, so-called cyanotic spells are seen, particularly in tetralogy of Fallot. In supraventricular tachycardia, vagal manoeuvres can be tried first, if not successful, intravenous adenosine or electroconversion will restore sinus rhythm. In the older child, intravenous isoptin can be given. Slow heart rates from total AV block or sinus node affection are treated with atrophic, isuprel or electrical pacing.
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PMID:[Pediatric cardiological emergencies]. 797 84

Propofol (2, 6-diisopropylphenol) is a potent intravenous hypnotic agent that is widely used in adults and children for sedation and the induction and maintenance of anaesthesia. Propofol has gained popularity for its rapid onset and rapid recovery even after prolonged use, and for the neuroprotection conferred. However, a review of the literature reveals multiple instances in which prolonged propofol administration (>48 hours) at high doses (>4 mg/kg/h) may cause a rare, but frequently fatal complication known as propofol infusion syndrome (PRIS). PRIS is characterized by metabolic acidosis, rhabdomyolysis of both skeletal and cardiac muscle, arrhythmias (bradycardia, atrial fibrillation, ventricular and supraventricular tachycardia, bundle branch block and asystole), myocardial failure, renal failure, hepatomegaly and death. PRIS has been described as an 'all or none' syndrome with sudden onset and probable death. The literature does not provide evidence of degrees of symptoms, nor of mildness or severity of signs in the clinical course of the syndrome. Recently, a fatal case of PRIS at a low infusion rate (1.9-2.6 mg/kg/h) has been reported. Common laboratory and instrumental findings in PRIS are myoglobinuria, downsloping ST-segment elevation, an increase in plasma creatine kinase, troponin I, potassium, creatinine, azotaemia, malonylcarnitine and C5-acylcarnitine, whereas in the mitochondrial respiratory electron transport chain, the activity of complex IV and cytochrome oxidase ratio is reduced. Propofol should be used with caution for sedation in critically ill children and adults, as well as for long-term anesthesia in otherwise healthy patients, and doses exceeding 4-5 mg/kg/h for long periods (>48 h) should be avoided. If PRIS is suspected, propofol must be stopped immediately and cardiocirculatory stabilization and correction of metabolic acidosis initiated. So, PRIS must be kept in mind as a rare, but highly lethal, complication of propofol use, not necessarily confined to its prolonged use. Furthermore, the safe dosage of propofol may need re-evaluation, and new studies are needed.
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PMID:Propofol infusion syndrome: an overview of a perplexing disease. 2002 84