ABSTRACT
Systemic Lupus erythematosus (SLE) is a chronic inflammatory disease, caused by a fault of the immune regulation. The etiology of the SLE is still unknown, a possible virus infection is discussed. Libman Sacks endokarditis is the most important cardiac manifestation of this illness. Diagnosis, therapy and clinical course of a 7 years old so far healthy girl, which suffered from an acute Libman-Sacks-Endocarditis, are presented.
Subject(s)
Endocarditis/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Child , Diagnosis, Differential , Echocardiography, Transesophageal , Endocarditis/drug therapy , Female , Hemodynamics/drug effects , Hemodynamics/physiology , Humans , Long-Term Care , Lupus Erythematosus, Systemic/drug therapy , Mitral Valve Insufficiency/diagnosis , Mitral Valve Insufficiency/drug therapy , Prednisolone/therapeutic useABSTRACT
We report on a 9-month-old female patient with pre- and postnatal growth retardation, hypertelorism, bilateral cleft lip and palate, and a peripheral pulmonary stenosis. High resolution banding and fluorescent in situ hybridization (FISH) revealed a de novo partial trisomy 22q13-qter. We compare the clinical findings to published patients with this rare chromosomal aberration and discuss the chromosomal differential diagnosis. Facial features at first sight suggestive of Wolf-Hirschhorn syndrome may be an additional, previously undescribed clinical sign in some patients with partial trisomy 22q.
Subject(s)
Abnormalities, Multiple/genetics , Chromosome Aberrations , Chromosomes, Human, Pair 22/genetics , Craniofacial Abnormalities/genetics , Female , Fetal Growth Retardation/genetics , Growth Disorders/genetics , Humans , Hypertelorism/genetics , In Situ Hybridization, Fluorescence , Infant , Mouth Abnormalities/genetics , SyndromeABSTRACT
AQ-A 39 is a new agent with specific bradycardic action on the sinus node. In this study the electrophysiologic and hemodynamic effects of this compound were investigated in 24 patients with various cardiac diseases. The results show: AQ-A 39 causes a significant decrease in enhanced sinus rate. The bradycardic action is more profound at higher sinus rates. In sinus rhythm of normal frequency or, in sinus bradycardia, sinus rate remains unchanged or increases even in the case of sick sinus node syndrome. Conductivity of the AV-node is significantly improved. QT- and QTc-interval increase dose dependently. Other cardiac conduction times and refractory intervals are not significantly changed. In exercise-induced myocardial ischemia increased left ventricular filling pressures are diminished. The extent of this decrease parallels the reduction in heart rate. AQ-A 39 appears to be suitable to reduce inadaequate increases in sinus rate, and seems to be of particular interest in surgical cases or intensive care emergencies as well as in acute ischemic heart disease.
