Acute intermittent porphyria is a rare genetic condition in which disrupted haem synthesis causes overproduction of porphyrin precursors. Occasionally, it is associated with posterior reversible encephalopathy syndrome (PRES), presenting with headache, confusion, seizures and visual disturbance. We describe a patient with acute intermittent porphyria who presented with seizures and PRES, and who had previous unexplained severe abdominal pain. Acute intermittent porphyria should be considered as a possible cause of PRES, especially in those with unexplained abdominal pain, since delays in its diagnosis can result in permanent complications.
Background: AVA by the continuity equation (AVA CE =0.785x(LVOTd) 2 xLVOT VTI /AV VTI) is a primary determinant of aortic stenosis (AS) severity. LVOTd underestimation by 2D TTE is common with resultant erroneously small AVA CE. "LVOT" shape differs between BAVs/TAVs and varies in size by gender and body surface area (BSA). 3D techniques overcome geometrical limitations of 2D measurements but remain underutilized. Further "optimization" of the 2D approach is necessary. Purpose: Developing a phenotype-and gender-specific predicted LVOTd equation to help recognize and avoid significant under sizing during 2D TTE imaging Methods: BAV and TAV TTEs with 3D datasets were included. 3D assessments at the aortic valve annulus level were performed to determine the optimal LVOTd (circumference/area/averaged minimum-maximum diameters) for AVA CE calculations. LVOT VTI /AV VTI were traced from spectral Doppler waveforms. AVA by 3D TTE planimetry (AVA 3D) was the reference standard. Results: 70 BAV and 70 TAV TTEs were included. Mean AVA 3D-BAV and AVA 3D-TAV were 3.4060.95cm 2 and 3.4960.77cm 2. Best LVOTd for AVA CE calculation in BAVs was derived from 3D circumference (Mean AVA CE-BAV 3.3061.06cm 2 ; ICC 0.969) and TAVs by 3D area (Mean AVA CE-TAV 3.3460.77cm 2 ; ICC 0.983). Males have larger LVOTd than females (Mean LVOTd BAV Males vs Females: 2.9560.31cm vs 2.5560.27cm, P,0.005; Mean LVOTd TAV Males vs Females: 2.4760.20cm vs 2.2260.16cm, P,0.005). Linear regression modelling demonstrated that the LVOTd can be predicted using LVOTd BAV =[1.45+(0.75xBSA)-0.25 {Females} ; r 2 =0.60, AIC=-7.51] and LVOTd TAV = [1.52+(0.50xBSA)-0.16 {Females} ; r 2 =0.46, AIC=-48.83]. Conclusions: Predicted LVOTd values will allow early recognition of incorrect 2D TTE LVOTd measurements, prompting sonographers to further optimize images, and preventing cardiologists from reporting inconsistent hemodynamic parameters and misclassifying AS severity.
Abstract. We study matrix forms whose determinants are easily computable. We give various forms of square matrices whose determinants are products of linear factors related with their entries.
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