La deficiencia de la descarboxilasa de aminoácidos aromáticos (AADCD) es una enfermedad neurometabólica minoritaria de herencia autosómica recesiva que causa un defecto grave de dopamina, serotonina, epinefrina y norepinefrina. El inicio de la enfermedad es precoz, y sus síntomas principales incluyen hipotonía, trastornos del movimiento (crisis oculógiras, distonía e hipocinesia), retraso del desarrollo y signos disautonómicos.
It is not unusual for patients with AADCD to need anesthesia for (minor) interventions such as gastrostomy. Only two reports described anesthesia management in two different AADCD patients [29, 30]. It should be noted that intravenous phenylephrine and dopamine may lead to an exaggerated hemodynamic response in these patients. In case of hypotension during intervention, dopamine should be initiated at low dose (e.g. 1-2 ug/kg/min) . Monitoring of hemodynamic function, temperature and glucose levels is important, especially in prolonged interventions.
We thank all our patients and their parents for teaching us about this disease. We thank Ron Wevers and Russell Dale for being external reviewers. In addition we thank Stefan Wenzel for his input from patient perspective.
On your personal Human Design chart this information is displayed in a column of red numbers on the left, and black numbers on the right. This information is sometimes also overlaid on a mandala to create the wheel with planets and hexagrams around the outside, as shown above in the blank rave mandala.
The Line Companion is an extensive commentary on the Gates and Lines as listed in the Complete Rave I'ching. In this commentary, Ra describes each Gate and Line in much more depth and detail. All quotes from the Complete Rave I'Ching are color coated for easy reference. Appendex includes 16 pages of relevant information (Hexagram Line structure, outer planet transits, Sun/Earth Line Fixations...). 2b1af7f3a8